Latest revision as of 18:51, 26 October 2018

For information on vascular anomalies, click here.

For information on benign vascular tumors, Click here.

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hannan Javed, M.D.[2], Anmol Pitliya, M.B.B.S. M.D.[3]

Overview

Vascular tumors are growths (benign or malignant) formed from blood vessels; for example, hemangioma, hemangioendothelioma, Kaposi sarcoma, angiosarcoma. International Society for the Study of Vascular Anomalies (ISSVA) has classified vascular tumors into three main categories depending on their clinical and histological behavior. Some lesions related clinically and/or histologically to vascular tumors have been described in provisionally unclassified vascular anomalies and related lesions.

Classification

Vascular tumors

Benign

Locally aggressive or borderline

Malignant

Infantile hemangioma / Hemangioma of infancy

Kaposiform hemangioendothelioma

Angiosarcoma

Congenital hemangioma

Retiform hemangioendothelioma

Epithelioid hemangioendothelioma

Tufted angioma

Papillary intralymphatic angioendothelioma (PILA), Dabska tumor

Others

Spindle-cell hemangioma

Composite hemangioendothelioma

Epithelioid hemangioma

Pseudomyogenic hemangioendothelioma

Pyogenic granuloma (also known as lobular capillary hemangioma)

Polymorphous hemangioendothelioma

Others
• Microvenular hemangioma
• Anastomosing hemangioma
• Glomeruloid hemangioma
• Papillary hemangioma
• Intravascular papillary endothelial hyperplasia
• Cutaneous epithelioid angiomatous nodule
• Acquired elastotic hemangioma
• Littoral cell hemangioma of the spleen

Hemangioendothelioma not otherwise specified

'

Related lesions
• Eccrine angiomatous hamartoma
• Reactive angioendotheliomatosis
• Bacillary angiomatosis

Kaposi sarcoma

Others

^*congenital hemangioma (rapidly involuting type) and tufted angioma may be associated with thrombocytopenia and/or consumptive coagulopathy in some cases. Many experts consider tufted angioma and kaposiform hemangioendothelioma to be part of a spectrum rather than distinct entities
Adapted from International Society for the Study of Vascular Anomalies^[1]

Benign vascular tumors

	Vascular Tumor
Home
Overview
Classification Benign Vascular tumor Locally aggressive or borderline vascular tumors Malignant vascular tumors
Benign Vascular Tumor Home Page

Benign vascular tumors, are benign growths formed from blood vessels; such as, hemangioma, hemangioendothelioma, Kaposi sarcoma. They exhibit a wide range of clinical manifestations, and may occur as isolated lesions or may occur as manifestation of multi-system syndromes and diseases. Their diagnosis and management depends on their clinical manifestations and coexistent anomalies. International Society for the Study of Vascular Anomalies (ISSVA) has classified these lesions into benign vascular tumors and related lesions.

For more information on benign vascular tumors, click here.

Locally aggressive or borderline vascular tumors

Kaposiform hemangioendothelioma

Locally Aggressive tumor that originates on skin and occurs primarily in childhood. It is characterized by a single or multiple masses with following characteristics:^[2]
- Deep reddish-purple color
- Shiny, firm texture
- Warm to the touch
- Swollen and painful
May be complicated by Kasabach-Merritt phenomenon (KMP), characterized by consumption coagulopathy, thrombocytopenia, and hemolytic anemia.^[3] Typical features also include low fibrinogen and elevated d-dimers.
Somatic activating GNA14 c.614A>T (p.Gln205Leu) mutations have been found in some KHE.^[4]
Invasion of bone, retroperitoneum, and mediastinum has occured in some cases but no case of metastasis has been reported yet.^[3]
Diagnostic work up may include blood tests, biopsy, contrast enhanced ultrasound and MRI or CT scan imaging.
Treatment options include steroid, vincristine, interferon alpha, anti-platelet agents, sirolimus-containing therapies, and surgery.^[5]

For more information on Kaposiform hemangioendothelioma, click here.

Retiform hemangioendothelioma

First described in 1994 as a form of low grade angiosarcoma, retiform hemangioendothelioma commonly presents as a slow growing asymptomatic solitary nodule or plaque on distal extremities in 2nd-4th decade of life.^[6]
It must be differentiated from angiosarcoma.
High level of local recurrence but very low potential for metastasis.
Diagnostic work up includes histopathological studies, that shows branching blood vessels are arranged in retiform pattern and MRI.
Surgery is the treatment of choice, though 2/3rd cases recur.
Adjuvant radiotherapy and adjuvant chemotherapy with recombinant interferon alpha and low dose cisplatin have also been reported in selected cases.

Papillary intralymphatic angioendothelioma (PILA), Dabska tumor

First described in 1969 by Dabska, this rare vascular neoplasm generally occurs in soft tissues but can also occur in bone. They usually appear as painless inflammatory irregular or nodular lesions below the skin surface.
The distinctive feature on histopathology is the intravascular growth of well-differentiated endothelial cells presenting as a matchstick columnar configuration.^[7]
They are locally aggressive but rarely metastasize. Locally recurrence after surgery is very common.
Diagnostic studies may include histopathological studies, fine needle aspiration, MRI, and CT scan.^[8]
Wide local excision is the treatment of choice. However any combination of steroids, chemotherapy, radiation therapy, and invasive procedures can be used to treat this tumor.

Composite hemangioendothelioma

A rare vascular neoplasms, characterized by an admixture of benign, low-grade malignant, and malignant vascular components, the ratio of each component can vary. They can occur in any age group.^[9]
They occur predominantly as long-standing lesions in the dermis and subcutis of the extremities, but can also occur at other sites, including the oral cavity and in viscera such as kidney and spleen.^[10]
It may recur locally and has the potential to metastasize. Recurrence was found to be in 8/10 cases in some studies. ^[11]
Diagnostic work up must include biopsy because of heterogeneity of lesions and it must be differentiated from other vascular tumors.^[9]
Surgical excision is the treatment of choice although some patients have been treated with interferons and electron beams.^[9]

Pseudomyogenic hemangioendothelioma

A locally aggressive tumor with endothelial differentiation that usually presents as multiple asymptomatic discontinuous lesions, often at extremities.^[12]^[13]
SERPINE1-FOSB fusions are characteristic that result in over-expression of truncated form of FOSB. FBJ murine osteosarcoma viral oncogene homolog B, also known as Finkel-Biskis-Jinkins murine osteosarcoma viral oncogene homolog B, FOSB or FosB, is a protein that, in humans have been implicated as regulators of cell proliferation, differentiation, and transformation.^[12]
It may mimic epithelioid sarcoma on histology but metastasis is very rare and prognosis is excellent.^[13]
Diagnostic work up includes X-ray, MRI, CT scan and biopsy of the lesion.
Excision is the typical treatment but chemotherapeutic agents including gemcitabine/taxane and mammalian target of rapamycin inhibitor, mTOR inhibitors such as sirolimus, VEGFR1-4/PDGFRA inhibitors such as telatinib have been used with success in various studies.^[14]^[15]^[12]

Polymorphous hemangioendothelioma

A rare vascular neoplasm, polymorphous hemangioendothelioma occurs in lymph nodes, but a few cases have been found in extra-nodal sites such as the mediastinum, spinal cord, and liver. It is a very rare cause of persistent lymphadenopathy. The data on natural history and clinical presentation is limited due to very few number of cases reported.^[16]
Characterized by a polymorphous blend of solid, primitive vascular and angiomatous areas in varied proportions on microscopic examination.^[16]
Diagnotic work up includes histopathological examination, MRI, and CT scan.
Wide local excision has been used for treatment, with radiation therapy in case of recurrence.^[17]^[18]

Kaposi sarcoma

An AIDS-associated vascular malignancy that usually presents as mucocutaneous lesions but can also occur in viscera such as lungs. It can remain confined to skin but widespread visceral involvement may occur.^[19]
There are three known variants:
- One variant occurs spontaneously in Jewish and Italian males in Europe and the United States.
- Another more aggressive variant is endemic in young children is endemic in Africa.
- A third form occurs in about 0.04% of kidney transplant patients. There is also a high incidence in AIDS patients. HHV-8 is the suspected cause.^[20]

For more information on Kaposi sarcoma, Click here

Malignant vascular tumors

Angiosarcoma

Angiosarcoma (AS) is malignancy that presents with a very heterogeneous distribution in the human body with aggressive clinical course, and may appear in multiple locations, from breast to liver or skin.^[21]
Associated with MYC gene amplification and protein overexpression. Myc is a family of regulator genes and proto-oncogenes that code for transcription factors.^[22]
Complete surgical excision and radiotherapy are the main treatments, with a minor role of chemotherapy.^[23]

For more information about angiosarcoma, click here.

Epithelioid hemangioendothelioma

A rare vascular tumor, described for the first time in 1975 by Dail and Liebow, that usually affects lung, liver and bones, although may occur many other sites in body including head and neck, breasts and lymph nodes.^[24]
Usually asymptomatic but patient may present with respiratory symptoms, bone pains or other symptoms depending on the site of the tumor.
Majority are characterized by a reciprocal t(1;3)(p36;q25) translocation. The t(1;3) results in fusion of a gene known as WWTR1 (or TAZ) to CAMTA1. These genes code for transcription factors.^[25]^[26]
Imaging is crucial in forming both diagnosis and management plan. Recognition of the expression of vascular markers (Fli-1 and CD31 are endothelial-specific markers), and the microscopic evidence of vascular differentiation is of primary importance as well.^[24]
Surgery has been used as primary treatment modality depending upon the location of the tumor, with radiotherapy being used in some cases.

References

↑ "Classification | International Society for the Study of Vascular Anomalies".
↑ Hu PA, Zhou ZR (June 2018). "Clinical and imaging features of Kaposiform Hemangioendothelioma". Br J Radiol. 91 (1086): 20170798. doi:10.1259/bjr.20170798. PMID 29536769.
↑ ^3.0 ^3.1 "Letter: Prevention of coronary heart-disease". Lancet. 2 (7980): 313–4. August 1976. PMID 59885.
↑ Lim YH, Bacchiocchi A, Qiu J, Straub R, Bruckner A, Bercovitch L, Narayan D, McNiff J, Ko C, Robinson-Bostom L, Antaya R, Halaban R, Choate KA (August 2016). "GNA14 Somatic Mutation Causes Congenital and Sporadic Vascular Tumors by MAPK Activation". Am. J. Hum. Genet. 99 (2): 443–50. doi:10.1016/j.ajhg.2016.06.010. PMC 4974082. PMID 27476652.
↑ Schmid I, Klenk AK, Sparber-Sauer M, Koscielniak E, Maxwell R, Häberle B (2018). "Kaposiform hemangioendothelioma in children: a benign vascular tumor with multiple treatment options". World J Pediatr. 14 (4): 322–329. doi:10.1007/s12519-018-0171-5. PMID 30054848.
↑ Ranga SM, Kuchangi NC, Shankar VS, Amita K, Haleuoor BB, Belagola SD (November 2014). "Retiform hemangioendothelioma: an uncommon pediatric vascular neoplasm". Indian J Dermatol. 59 (6): 633. doi:10.4103/0019-5154.143583. PMC 4248535. PMID 25484427.
↑ © 1999 Lippincott Williams & Wilkins, Inc.
↑ https://www.dovemed.com/diseases-conditions/papillary-intralymphatic-angioendothelioma-pila/
↑ ^9.0 ^9.1 ^9.2 Rokni GR, Montazer F, Sharifian M, Goldust M (December 2017). "Composite hemangioendothelioma of the forehead and right eye; a case report". BMC Dermatol. 17 (1): 15. doi:10.1186/s12895-017-0067-4. PMC 5727897. PMID 29233122.
↑ Shang Leen SL, Fisher C, Thway K (July 2015). "Composite hemangioendothelioma: clinical and histologic features of an enigmatic entity". Adv Anat Pathol. 22 (4): 254–9. doi:10.1097/PAP.0000000000000079. PMID 26050262.
↑ https://www.jpatholtm.org/upload/pdf/kjp-40-2-142.pdf
↑ ^12.0 ^12.1 ^12.2 van IJzendoorn D, Sleijfer S, Gelderblom H, Eskens F, van Leenders G, Szuhai K, Bovée J (June 2018). "Telatinib Is an Effective Targeted Therapy for Pseudomyogenic Hemangioendothelioma". Clin. Cancer Res. 24 (11): 2678–2687. doi:10.1158/1078-0432.CCR-17-3512. PMID 29511030. Vancouver style error: initials (help)
↑ ^13.0 ^13.1 Raftopoulos E, Royer M, Warren M, Zhao J, Rush W (August 2018). "Pseudomyogenic Hemangioendothelioma: Case Report and Review of the Literature". Am J Dermatopathol. 40 (8): 597–601. doi:10.1097/DAD.0000000000001104. PMID 29406432.
↑ Joseph J, Wang WL, Patnana M, Ramesh N, Benjamin R, Patel S, Ravi V (2015). "Cytotoxic and targeted therapy for treatment of pseudomyogenic hemangioendothelioma". Clin Sarcoma Res. 5: 22. doi:10.1186/s13569-015-0037-8. PMC 4615364. PMID 26500758.
↑ Gabor KM, Sapi Z, Tiszlavicz LG, Fige A, Bereczki C, Bartyik K (February 2018). "Sirolimus therapy in the treatment of pseudomyogenic hemangioendothelioma". Pediatr Blood Cancer. 65 (2). doi:10.1002/pbc.26781. PMID 28843050.
↑ ^16.0 ^16.1 El Hussein S, Omarzai Y (May 2017). "Multifocal Polymorphous Hemangioendothelioma of the Liver: Case Report and Review of Literature". Int. J. Surg. Pathol. 25 (3): 266–270. doi:10.1177/1066896916679517. PMID 27913780.
↑ Tadros M, Rizk SS, Opher E, Thompson LD (June 2003). "Polymorphous hemangioendothelioma of the neck". Ann Diagn Pathol. 7 (3): 165–8. PMID 12808568.
↑ Falleti J, Siano M, De Cecio R, Somma A, Pettinato G, Insabato L (2009). "Nodal and extranodal soft tissue polymorphous hemangioendothelioma: a case report and review of the literature". Tumori. 95 (1): 94–7. PMID 19366064.
↑ Khan S, Guevara J, Barbosa A, Ayuby A, Bien-Aime F, Verda L, Glick N, Mehta V (2018). "Primary pulmonary Kaposi Sarcoma in a newly diagnosed cisgender heterosexual HIV positive patient presenting before cutaneous manifestations". IDCases. 14: e00420. doi:10.1016/j.idcr.2018.e00420. PMC 6125769. PMID 30191128.
↑ Piccolo V, Russo T, Moscarella E, Brancaccio G, Alfano R, Argenziano G (October 2018). "Dermatoscopy of Vascular Lesions". Dermatol Clin. 36 (4): 389–395. doi:10.1016/j.det.2018.05.006. PMID 30201148.
↑ Villaescusa Catalan JM, Martín IG, Cagigal Cobo ML (September 2018). "Popliteal Angiosarcoma After Bypass With Autologous Saphenous Vein". Ann Vasc Surg. doi:10.1016/j.avsg.2018.06.034. PMID 30217704.
↑ Udager AM, Ishikawa MK, Lucas DR, McHugh JB, Patel RM (December 2016). "MYC immunohistochemistry in angiosarcoma and atypical vascular lesions: practical considerations based on a single institutional experience". Pathology. 48 (7): 697–704. doi:10.1016/j.pathol.2016.08.007. PMID 27780597.
↑ Priyakumari T, Chandar R, Jayasree K, Ramachandran K (September 2018). "Pediatric Primary Ovarian Angiosarcoma: From rarity to a realization". J Pediatr Adolesc Gynecol. doi:10.1016/j.jpag.2018.08.008. PMID 30179666.
↑ ^24.0 ^24.1 Sardaro A, Bardoscia L, Petruzzelli MF, Portaluri M (September 2014). "Epithelioid hemangioendothelioma: an overview and update on a rare vascular tumor". Oncol Rev. 8 (2): 259. doi:10.4081/oncol.2014.259. PMC 4419652. PMID 25992243.
↑ Errani C, Zhang L, Sung YS, Hajdu M, Singer S, Maki RG, Healey JH, Antonescu CR (August 2011). "A novel WWTR1-CAMTA1 gene fusion is a consistent abnormality in epithelioid hemangioendothelioma of different anatomic sites". Genes Chromosomes Cancer. 50 (8): 644–53. doi:10.1002/gcc.20886. PMC 3264678. PMID 21584898.
↑ Tanas MR, Sboner A, Oliveira AM, Erickson-Johnson MR, Hespelt J, Hanwright PJ, Flanagan J, Luo Y, Fenwick K, Natrajan R, Mitsopoulos C, Zvelebil M, Hoch BL, Weiss SW, Debiec-Rychter M, Sciot R, West RB, Lazar AJ, Ashworth A, Reis-Filho JS, Lord CJ, Gerstein MB, Rubin MA, Rubin BP (August 2011). "Identification of a disease-defining gene fusion in epithelioid hemangioendothelioma". Sci Transl Med. 3 (98): 98ra82. doi:10.1126/scitranslmed.3002409. PMID 21885404.

Template:WikiDoc Sources

[urlClassification_|_International_Society_for_the_Study_of_Vascular_Anomalies-1] "Classification | International Society for the Study of Vascular Anomalies".

[pmid29536769-2] Hu PA, Zhou ZR (June 2018). "Clinical and imaging features of Kaposiform Hemangioendothelioma". Br J Radiol. 91 (1086): 20170798. doi:10.1259/bjr.20170798. PMID 29536769.

[pmid59885-3] 3.0 ^3.1 "Letter: Prevention of coronary heart-disease". Lancet. 2 (7980): 313–4. August 1976. PMID 59885.

[pmid27476652-4] Lim YH, Bacchiocchi A, Qiu J, Straub R, Bruckner A, Bercovitch L, Narayan D, McNiff J, Ko C, Robinson-Bostom L, Antaya R, Halaban R, Choate KA (August 2016). "GNA14 Somatic Mutation Causes Congenital and Sporadic Vascular Tumors by MAPK Activation". Am. J. Hum. Genet. 99 (2): 443–50. doi:10.1016/j.ajhg.2016.06.010. PMC 4974082. PMID 27476652.

[pmid30054848-5] Schmid I, Klenk AK, Sparber-Sauer M, Koscielniak E, Maxwell R, Häberle B (2018). "Kaposiform hemangioendothelioma in children: a benign vascular tumor with multiple treatment options". World J Pediatr. 14 (4): 322–329. doi:10.1007/s12519-018-0171-5. PMID 30054848.

[pmid25484427-6] Ranga SM, Kuchangi NC, Shankar VS, Amita K, Haleuoor BB, Belagola SD (November 2014). "Retiform hemangioendothelioma: an uncommon pediatric vascular neoplasm". Indian J Dermatol. 59 (6): 633. doi:10.4103/0019-5154.143583. PMC 4248535. PMID 25484427.

[7] © 1999 Lippincott Williams & Wilkins, Inc.

[8] ttps://www.dovemed.com/diseases-conditions/papillary-intralymphatic-angioendothelioma-pila/

[pmid29233122-9] 9.0 ^9.1 ^9.2 Rokni GR, Montazer F, Sharifian M, Goldust M (December 2017). "Composite hemangioendothelioma of the forehead and right eye; a case report". BMC Dermatol. 17 (1): 15. doi:10.1186/s12895-017-0067-4. PMC 5727897. PMID 29233122.

[pmid26050262-10] Shang Leen SL, Fisher C, Thway K (July 2015). "Composite hemangioendothelioma: clinical and histologic features of an enigmatic entity". Adv Anat Pathol. 22 (4): 254–9. doi:10.1097/PAP.0000000000000079. PMID 26050262.

[11] ttps://www.jpatholtm.org/upload/pdf/kjp-40-2-142.pdf

[pmid29511030-12] 12.0 ^12.1 ^12.2 van IJzendoorn D, Sleijfer S, Gelderblom H, Eskens F, van Leenders G, Szuhai K, Bovée J (June 2018). "Telatinib Is an Effective Targeted Therapy for Pseudomyogenic Hemangioendothelioma". Clin. Cancer Res. 24 (11): 2678–2687. doi:10.1158/1078-0432.CCR-17-3512. PMID 29511030. Vancouver style error: initials (help)

[pmid29406432-13] 13.0 ^13.1 Raftopoulos E, Royer M, Warren M, Zhao J, Rush W (August 2018). "Pseudomyogenic Hemangioendothelioma: Case Report and Review of the Literature". Am J Dermatopathol. 40 (8): 597–601. doi:10.1097/DAD.0000000000001104. PMID 29406432.

[pmid26500758-14] Joseph J, Wang WL, Patnana M, Ramesh N, Benjamin R, Patel S, Ravi V (2015). "Cytotoxic and targeted therapy for treatment of pseudomyogenic hemangioendothelioma". Clin Sarcoma Res. 5: 22. doi:10.1186/s13569-015-0037-8. PMC 4615364. PMID 26500758.

[pmid28843050-15] Gabor KM, Sapi Z, Tiszlavicz LG, Fige A, Bereczki C, Bartyik K (February 2018). "Sirolimus therapy in the treatment of pseudomyogenic hemangioendothelioma". Pediatr Blood Cancer. 65 (2). doi:10.1002/pbc.26781. PMID 28843050.

[pmid27913780-16] 16.0 ^16.1 El Hussein S, Omarzai Y (May 2017). "Multifocal Polymorphous Hemangioendothelioma of the Liver: Case Report and Review of Literature". Int. J. Surg. Pathol. 25 (3): 266–270. doi:10.1177/1066896916679517. PMID 27913780.

[pmid12808568-17] Tadros M, Rizk SS, Opher E, Thompson LD (June 2003). "Polymorphous hemangioendothelioma of the neck". Ann Diagn Pathol. 7 (3): 165–8. PMID 12808568.

[pmid19366064-18] Falleti J, Siano M, De Cecio R, Somma A, Pettinato G, Insabato L (2009). "Nodal and extranodal soft tissue polymorphous hemangioendothelioma: a case report and review of the literature". Tumori. 95 (1): 94–7. PMID 19366064.

[pmid30191128-19] Khan S, Guevara J, Barbosa A, Ayuby A, Bien-Aime F, Verda L, Glick N, Mehta V (2018). "Primary pulmonary Kaposi Sarcoma in a newly diagnosed cisgender heterosexual HIV positive patient presenting before cutaneous manifestations". IDCases. 14: e00420. doi:10.1016/j.idcr.2018.e00420. PMC 6125769. PMID 30191128.

[pmid30201148-20] Piccolo V, Russo T, Moscarella E, Brancaccio G, Alfano R, Argenziano G (October 2018). "Dermatoscopy of Vascular Lesions". Dermatol Clin. 36 (4): 389–395. doi:10.1016/j.det.2018.05.006. PMID 30201148.

[pmid30217704-21] Villaescusa Catalan JM, Martín IG, Cagigal Cobo ML (September 2018). "Popliteal Angiosarcoma After Bypass With Autologous Saphenous Vein". Ann Vasc Surg. doi:10.1016/j.avsg.2018.06.034. PMID 30217704.

[pmid27780597-22] Udager AM, Ishikawa MK, Lucas DR, McHugh JB, Patel RM (December 2016). "MYC immunohistochemistry in angiosarcoma and atypical vascular lesions: practical considerations based on a single institutional experience". Pathology. 48 (7): 697–704. doi:10.1016/j.pathol.2016.08.007. PMID 27780597.

[pmid30179666-23] Priyakumari T, Chandar R, Jayasree K, Ramachandran K (September 2018). "Pediatric Primary Ovarian Angiosarcoma: From rarity to a realization". J Pediatr Adolesc Gynecol. doi:10.1016/j.jpag.2018.08.008. PMID 30179666.

[pmid25992243-24] 24.0 ^24.1 Sardaro A, Bardoscia L, Petruzzelli MF, Portaluri M (September 2014). "Epithelioid hemangioendothelioma: an overview and update on a rare vascular tumor". Oncol Rev. 8 (2): 259. doi:10.4081/oncol.2014.259. PMC 4419652. PMID 25992243.

[pmid21584898-25] Errani C, Zhang L, Sung YS, Hajdu M, Singer S, Maki RG, Healey JH, Antonescu CR (August 2011). "A novel WWTR1-CAMTA1 gene fusion is a consistent abnormality in epithelioid hemangioendothelioma of different anatomic sites". Genes Chromosomes Cancer. 50 (8): 644–53. doi:10.1002/gcc.20886. PMC 3264678. PMID 21584898.

[pmid21885404-26] Tanas MR, Sboner A, Oliveira AM, Erickson-Johnson MR, Hespelt J, Hanwright PJ, Flanagan J, Luo Y, Fenwick K, Natrajan R, Mitsopoulos C, Zvelebil M, Hoch BL, Weiss SW, Debiec-Rychter M, Sciot R, West RB, Lazar AJ, Ashworth A, Reis-Filho JS, Lord CJ, Gerstein MB, Rubin MA, Rubin BP (August 2011). "Identification of a disease-defining gene fusion in epithelioid hemangioendothelioma". Sci Transl Med. 3 (98): 98ra82. doi:10.1126/scitranslmed.3002409. PMID 21885404.

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@@ Line 6: / Line 6: @@
 {{Vascular tumor}}
-{{CMG}}; {{AE}} {{HMHJ}}
+{{CMG}}; {{AE}} {{HMHJ}}, {{Anmol}}
 ==Overview==
-'''Vascular tumor''' may mean:
+'''[[Vascular]] [[tumors]]''' are growths ([[tumor#Tumor Types: Malignant vs. benign|benign or malignant]]) formed from [[blood vessel]]s; for example, [[hemangioma]], [[hemangioendothelioma]], [[Kaposi sarcoma]], [[angiosarcoma]]. '''International Society for the Study of Vascular Anomalies (ISSVA)''' has classified [[vascular tumors]] into three main categories depending on their clinical and histological behavior. Some [[lesions]] related clinically and/or histologically to [[vascular]] [[tumors]] have been described in '''[[provisionally unclassified vascular anomalies]]''' and '''[[related lesions]]'''.
+==Classification==
-* '''[[tumor]] of vascular origin''', a growth ([[tumor#Tumor Types: Malignant vs. benign|benign or malignant]]) formed from [[blood vessel]]s; for example, [[hemangioma]], [[hemangioendothelioma]], [[Kaposi sarcoma]], [[angiosarcoma]], etc.
-* '''International Society for the Study of Vascular Anomalies (ISSVA)''' has classified [[vascular tumors]] into three categories depending on their clinical and histological behavior. Some [[lesions]] related clinically and/or histologically to [[vascular]] tumors have been described as '''related lesions'''.
-==Classification==
 {{Family tree/start}}
-{{Family tree | | | | | | | | | | | | | | | A01 | | | | | | | | | | | | | | | | A01= '''Vascular tumors'''}}
+{{Family tree | | | | | | | | | | | | | | | A01 | | | | | | | | | | | | | | | | A01= '''[[Vascular tumors]]'''}}
 {{Family tree | | | | | | | |,|-|-|-|-|-|-|-|+|-|-|-|-|-|-|-|.| | | | | | | | | }}
 {{Family tree | | | | | | | B01 | | | | | | B02 | | | | | | B03 | | | | | | | | B01='''''Benign'''''|B02='''''Locally aggressive or borderline'''''|B03='''''Malignant'''''}}
 {{Family tree | | | | | | |!| | | | | | | |!| | | | | | | |!| | | | | | | | | | }}
-{{Family tree | | | | | | |)| C01 | | | | |)| D01 | | | | |)| E01 | | | | | | | C01=Infantile hemangioma / Hemangioma of infancy|D01=Kaposiform hemangioendothelioma<sup>*</sup>|E01=Angiosarcoma}}
+{{Family tree | | | | | | |)| C01 | | | | |)| D01 | | | | |)| E01 | | | | | | | C01=[[Infantile hemangioma]] / [[Hemangioma of infancy]]|D01=[[Kaposiform hemangioendothelioma]]|E01=[[Angiosarcoma]]}}
 {{Family tree | | | | | | |!| | | | | | | |!| | | | | | | |!| | | | | | | | | | }}
-{{Family tree | | | | | | |)| C02 | | | | |)| D02 | | | | |)| E02 | | | | | | | C02=Congenital hemangioma<sup>*</sup>|D02=Retiform hemangioendothelioma|E02=Epithelioid hemangioendothelioma }}
+{{Family tree | | | | | | |)| C02 | | | | |)| D02 | | | | |)| E02 | | | | | | | C02=[[Congenital hemangioma]]|D02=[[Retiform hemangioendothelioma]]|E02=[[Epithelioid hemangioendothelioma]]}}
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-{{Family tree | | | | | | |)| C03 | | | | |)| D03 | | | | |`| E03 | | | | | | | C03=Tufted angioma<sup>*</sup>|D03=Papillary intralymphatic angioendothelioma (PILA), Dabska tumor|E03=Others}}
+{{Family tree | | | | | | |)| C03 | | | | |)| D03 | | | | |`| E03 | | | | | | | C03=[[Tufted angioma]]|D03=[[Papillary intralymphatic angioendothelioma]] ([[PILA]]), [[Dabska tumor]]|E03=Others}}
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-{{Family tree | | | | | | |)| C04 | | | | |)| D04 | | | | | | | | | | | | | | | C04=Spindle-cell hemangioma|D04=Composite hemangioendothelioma}}
+{{Family tree | | | | | | |)| C04 | | | | |)| D04 | | | | | | | | | | | | | | | C04=[[Spindle-cell hemangioma]]|D04=[[Composite hemangioendothelioma]]}}
 {{Family tree | | | | | | |!| | | | | | | |!| | | | | | | | | | | | | | | | | | }}
-{{Family tree | | | | | | |)| C05 | | | | |)| D05 | | | | | | | | | | | | | | | C05=Epithelioid hemangioma|D05=Pseudomyogenic hemangioendothelioma}}
+{{Family tree | | | | | | |)| C05 | | | | |)| D05 | | | | | | | | | | | | | | | C05=[[Epithelioid hemangioma]]|D05=[[Pseudomyogenic hemangioendothelioma]]}}
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-{{Family tree | | | | | | |)| C06 | | | | |)| D06 | | | | | | | | | | | | | | | C06=Pyogenic granuloma (also known as lobular capillary hemangioma)|D06=Polymorphous hemangioendothelioma}}
+{{Family tree | | | | | | |)| C06 | | | | |)| D06 | | | | | | | | | | | | | | | C06=[[Pyogenic granuloma]] (also known as [[lobular capillary hemangioma]])|D06=[[Polymorphous hemangioendothelioma]]}}
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 {{Family tree | | | | | | |)| C07 | | | | |)| D07 | | | | | | | | | | | | | | | | |C07=<table>
 <tr><td>Others</td></tr>
-<tr><td>• Microvenular hemangioma<td></tr>
+<tr><td>• [[Microvenular hemangioma]]<td></tr>
-<tr><td>• Anastomosing hemangioma<td></tr>
+<tr><td>• [[Anastomosing hemangioma]]<td></tr>
-<tr><td>• Glomeruloid hemangioma<td></tr>
+<tr><td>• [[Glomeruloid hemangioma]]<td></tr>
-<tr><td>• Papillary hemangioma<td></tr>
+<tr><td>• [[Papillary hemangioma]]<td></tr>
-<tr><td>• Intravascular papillary endothelial hyperplasia<td></tr>
+<tr><td>• [[Intravascular papillary endothelial hyperplasia]]<td></tr>
-<tr><td>• Cutaneous epithelioid angiomatous nodule<td></tr>
+<tr><td>• [[Cutaneous epithelioid angiomatous nodule]]<td></tr>
-<tr><td>• Acquired elastotic hemangioma<td></tr>
+<tr><td>• [[Acquired elastotic hemangioma]]<td></tr>
-<tr><td>• Littoral cell hemangioma of the spleen<td></tr>
+<tr><td>• [[Littoral cell hemangioma of the spleen]]<td></tr>
 </table> |D07=Hemangioendothelioma not otherwise specified}}
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 {{Family tree | | | | | | |`| C08 | | | | |)| D08 | | | | | | | | | | | | | | | |C08=<table>
 <tr><td>Related lesions</td></tr>
-<tr><td>• Eccrine angiomatous hamartoma<td></tr>
+<tr><td>• [[Eccrine angiomatous hamartoma]]<td></tr>
-<tr><td>• Reactive angioendotheliomatosis<td></tr>
+<tr><td>• [[Reactive angioendotheliomatosis]]<td></tr>
-<tr><td>• Bacillary angiomatosis<td></tr>'
+<tr><td>• [[Bacillary angiomatosis]]<td></tr>'
-</table> |D08=Kaposi sarcoma}}
+</table> |D08=[[Kaposi sarcoma]]}}
 {{Family tree | | | | | | | | | | | | | | |!| | | | | | | | | | | | | | | | | | }}
 {{Family tree | | | | | | | | | | | | | | |`| D09 | | | | | | | | | | | | | | | D09=Others}}
 {{Family tree/end}}
-<sup>*</sup>congenital hemangioma (rapidly involuting type) and tufted angioma may be associated with thrombocytopenia and/or consumptive coagulopathy in some cases. Many experts consider tufted angioma and kaposiform hemangioendothelioma to be part of a spectrum rather than distinct entities
-'''Adapted from International Society for the Study of Vascular Anomalies'''<ref name="urlClassification | International Society for the Study of Vascular Anomalies">{{cite web |url=http://www.issva.org/classification |title=Classification &#124; International Society for the Study of Vascular Anomalies |format= |work= |accessdate=}}</ref></small>
+<sup>*</sup>[[congenital hemangioma]] (rapidly involuting type) and [[tufted angioma]] may be associated with thrombocytopenia and/or consumptive coagulopathy in some cases. Many experts consider [[tufted angioma]] and [[kaposiform hemangioendothelioma]] to be part of a spectrum rather than distinct entities
+<br>'''Adapted from International Society for the Study of Vascular Anomalies'''<ref name="urlClassification | International Society for the Study of Vascular Anomalies">{{cite web |url=http://www.issva.org/classification |title=Classification &#124; International Society for the Study of Vascular Anomalies |format= |work= |accessdate=}}</ref>
 =Benign vascular tumors=
-For details about benign vascular tumors, [[Benign vascular tumor#Benign vascular tumor|Click here]].
+''' [[Benign]] [[vascular]] [[tumors]]''', are [[benign]] growths formed from [[blood vessel]]s; such as, [[hemangioma]], [[hemangioendothelioma]], [[Kaposi sarcoma]]. They exhibit a wide range of clinical manifestations, and may occur as isolated [[lesions]] or may occur as manifestation of multi-system [[syndromes]] and [[diseases]]. Their [[diagnosis]] and management depends on their clinical manifestations and coexistent [[anomalies]]. '''International Society for the Study of Vascular Anomalies (ISSVA)''' has classified these [[lesions]] into [[benign vascular tumors]] and related [[lesions]].
+For more information on benign vascular tumors, [[Benign vascular tumor#Benign vascular tumor|click here]].
 =Locally aggressive or borderline vascular tumors=
 ===Kaposiform hemangioendothelioma===
-* Locally Aggressive [[tumor]] that originates on [[skin]] and occurs primarily in [[childhood]].<ref name="pmid29536769">{{cite journal |vauthors=Hu PA, Zhou ZR |title=Clinical and imaging features of Kaposiform Hemangioendothelioma |journal=Br J Radiol |volume=91 |issue=1086 |pages=20170798 |date=June 2018 |pmid=29536769 |doi=10.1259/bjr.20170798 |url=}}</ref> It is characterized by a single or multiple masses with following characteristics:
+* Locally Aggressive [[tumor]] that originates on [[skin]] and occurs primarily in [[childhood]]. It is characterized by a single or multiple masses with following characteristics:<ref name="pmid29536769">{{cite journal |vauthors=Hu PA, Zhou ZR |title=Clinical and imaging features of Kaposiform Hemangioendothelioma |journal=Br J Radiol |volume=91 |issue=1086 |pages=20170798 |date=June 2018 |pmid=29536769 |doi=10.1259/bjr.20170798 |url=}}</ref>
 ** Deep reddish-purple color
 ** Shiny, firm texture
 ** Warm to the touch
 ** Swollen and painful
-* May be complicated by [[Kasabach-Merritt phenomenon]](KMP), characterized by consumption [[coagulopathy]], [[thrombocytopenia]], and [[hemolytic anemia]].<ref name="pmid59885">{{cite journal |vauthors= |title=Letter: Prevention of coronary heart-disease |journal=Lancet |volume=2 |issue=7980 |pages=313–4 |date=August 1976 |pmid=59885 |doi= |url=}}</ref> Typical features also include low fibrinogen and elevated D-dimers.
+* May be complicated by Kasabach-Merritt phenomenon (KMP), characterized by consumption [[coagulopathy]], [[thrombocytopenia]], and [[hemolytic anemia]].<ref name="pmid59885">{{cite journal |vauthors= |title=Letter: Prevention of coronary heart-disease |journal=Lancet |volume=2 |issue=7980 |pages=313–4 |date=August 1976 |pmid=59885 |doi= |url=}}</ref> Typical features also include low fibrinogen and elevated d-dimers.
-* [[somatic]] activating GNA14 c.614A>T (p.Gln205Leu) [[mutations]] have been found in some KHE.<ref name="pmid27476652">{{cite journal |vauthors=Lim YH, Bacchiocchi A, Qiu J, Straub R, Bruckner A, Bercovitch L, Narayan D, McNiff J, Ko C, Robinson-Bostom L, Antaya R, Halaban R, Choate KA |title=GNA14 Somatic Mutation Causes Congenital and Sporadic Vascular Tumors by MAPK Activation |journal=Am. J. Hum. Genet. |volume=99 |issue=2 |pages=443–50 |date=August 2016 |pmid=27476652 |pmc=4974082 |doi=10.1016/j.ajhg.2016.06.010 |url=}}</ref>
+* [[Somatic]] activating GNA14 c.614A>T (p.Gln205Leu) [[mutations]] have been found in some KHE.<ref name="pmid27476652">{{cite journal |vauthors=Lim YH, Bacchiocchi A, Qiu J, Straub R, Bruckner A, Bercovitch L, Narayan D, McNiff J, Ko C, Robinson-Bostom L, Antaya R, Halaban R, Choate KA |title=GNA14 Somatic Mutation Causes Congenital and Sporadic Vascular Tumors by MAPK Activation |journal=Am. J. Hum. Genet. |volume=99 |issue=2 |pages=443–50 |date=August 2016 |pmid=27476652 |pmc=4974082 |doi=10.1016/j.ajhg.2016.06.010 |url=}}</ref>
-* Invasion of [[bone]], [[retroperitoneum]], and [[mediastinum]] has occured in some cases but no case of [[metastasis]] has been reported yet. <ref name="pmid59885">{{cite journal |vauthors= |title=Letter: Prevention of coronary heart-disease |journal=Lancet |volume=2 |issue=7980 |pages=313–4 |date=August 1976 |pmid=59885 |doi= |url=}}</ref>
+* Invasion of [[bone]], [[retroperitoneum]], and [[mediastinum]] has occured in some cases but no case of [[metastasis]] has been reported yet.<ref name="pmid59885">{{cite journal |vauthors= |title=Letter: Prevention of coronary heart-disease |journal=Lancet |volume=2 |issue=7980 |pages=313–4 |date=August 1976 |pmid=59885 |doi= |url=}}</ref>
-* [[Diagnostic]] work up may include blood tests, [[biopsy]], [[contrast enhanced ultrasound]] and [[MRI]] or [[CT scan imaging]].
+* [[Diagnostic]] work up may include blood tests, [[biopsy]], [[Ultrasound|contrast enhanced ultrasound]] and [[MRI]] or [[CT scan|CT scan imaging]].
-* Treatment Options include [[steroid]], [[vincristine]], [[interferon alpha]], [[anti-platelet agents]], sirolimus-containing therapies and [[surgery]].<ref name="pmid30054848">{{cite journal| author=Schmid I, Klenk AK, Sparber-Sauer M, Koscielniak E, Maxwell R, Häberle B| title=Kaposiform hemangioendothelioma in children: a benign vascular tumor with multiple treatment options. | journal=World J Pediatr | year= 2018 | volume= 14 | issue= 4 | pages= 322-329 | pmid=30054848 | doi=10.1007/s12519-018-0171-5 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30054848  }} </ref>
+* Treatment options include [[steroid]], [[vincristine]], [[interferon alpha]], anti-platelet agents, [[sirolimus]]-containing therapies, and [[surgery]].<ref name="pmid30054848">{{cite journal| author=Schmid I, Klenk AK, Sparber-Sauer M, Koscielniak E, Maxwell R, Häberle B| title=Kaposiform hemangioendothelioma in children: a benign vascular tumor with multiple treatment options. | journal=World J Pediatr | year= 2018 | volume= 14 | issue= 4 | pages= 322-329 | pmid=30054848 | doi=10.1007/s12519-018-0171-5 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30054848  }} </ref>
 For more information on [[Kaposiform hemangioendothelioma]], [[Kaposiform hemangioendothelioma#Kaposiform hemangioendothelioma|click here]].
 ===Retiform hemangioendothelioma===
-* First described in 1994 as a form of low grade [[angiosarcoma]], Retiform hemangioendothelioma commonly presents as a slow growing [[asymptomatic]] solitary nodule or plaque on distal [[extremities]] in 2nd-4th decade of life.<ref name="pmid25484427">{{cite journal |vauthors=Ranga SM, Kuchangi NC, Shankar VS, Amita K, Haleuoor BB, Belagola SD |title=Retiform hemangioendothelioma: an uncommon pediatric vascular neoplasm |journal=Indian J Dermatol |volume=59 |issue=6 |pages=633 |date=November 2014 |pmid=25484427 |pmc=4248535 |doi=10.4103/0019-5154.143583 |url=}}</ref>
+* First described in 1994 as a form of low grade [[angiosarcoma]], retiform hemangioendothelioma commonly presents as a slow growing [[asymptomatic]] solitary nodule or plaque on distal [[extremities]] in 2nd-4th decade of life.<ref name="pmid25484427">{{cite journal |vauthors=Ranga SM, Kuchangi NC, Shankar VS, Amita K, Haleuoor BB, Belagola SD |title=Retiform hemangioendothelioma: an uncommon pediatric vascular neoplasm |journal=Indian J Dermatol |volume=59 |issue=6 |pages=633 |date=November 2014 |pmid=25484427 |pmc=4248535 |doi=10.4103/0019-5154.143583 |url=}}</ref>
-* Must be differentiated from [[Angiosarcoma]].
+* It must be differentiated from [[angiosarcoma]].
 * High level of local [[recurrence]] but very low potential for [[metastasis]].
-* [[Diagnostic]] work up includes histopathological studies, that shows branching [[blood]] [[vessels]] are arranged in [[retiform]] pattern <ref name="pmid25484427">{{cite journal |vauthors=Ranga SM, Kuchangi NC, Shankar VS, Amita K, Haleuoor BB, Belagola SD |title=Retiform hemangioendothelioma: an uncommon pediatric vascular neoplasm |journal=Indian J Dermatol |volume=59 |issue=6 |pages=633 |date=November 2014 |pmid=25484427 |pmc=4248535 |doi=10.4103/0019-5154.143583 |url=}}</ref>, and MRI.
+* [[Diagnostic]] work up includes histopathological studies, that shows branching [[blood]] [[vessels]] are arranged in retiform pattern and MRI.
-* [[Surgery]] is the treatment of choice, though 2/3rd cases recur. Adjuvant [[radiotherapy]] and adjuvant [[chemotherapy]] with recombinant [[interferon alpha]] and low dose [[cisplatin]] have also been reported in selected cases. <ref name="pmid25484427">{{cite journal |vauthors=Ranga SM, Kuchangi NC, Shankar VS, Amita K, Haleuoor BB, Belagola SD |title=Retiform hemangioendothelioma: an uncommon pediatric vascular neoplasm |journal=Indian J Dermatol |volume=59 |issue=6 |pages=633 |date=November 2014 |pmid=25484427 |pmc=4248535 |doi=10.4103/0019-5154.143583 |url=}}</ref>
+* [[Surgery]] is the treatment of choice, though 2/3rd cases recur.
+* Adjuvant [[radiotherapy]] and adjuvant [[chemotherapy]] with recombinant [[interferon alpha]] and low dose [[cisplatin]] have also been reported in selected cases.
 ===Papillary intralymphatic angioendothelioma (PILA), Dabska tumor===
-* First described in 1969 by Dabska,this rare [[vascular]] [[neoplasm]] generally occurs in soft tissues but can also occur in [[bone]]. They usually appear as painless inflammatory irregular or nodular [[lesions]] below the [[skin]] surface.
+* First described in 1969 by Dabska, this rare [[vascular]] [[neoplasm]] generally occurs in soft tissues but can also occur in [[bone]]. They usually appear as painless inflammatory irregular or nodular [[lesions]] below the [[skin]] surface.
 * The distinctive feature on histopathology is the intravascular growth of well-differentiated [[endothelial cells]] presenting as a matchstick columnar configuration.<ref>© 1999 Lippincott Williams & Wilkins, Inc.</ref>
-* They are locally aggressive but rarely [[metastasize]]. Locally [[recurrence]] after [[surgery]] is very common.
+* They are locally aggressive but rarely [[metastasize]]. Locally recurrence after [[surgery]] is very common.
-* [[Diagnostic]] studies may include histopathological studies, [[fine needle aspiration]], [[MRI]] and [[CT scan]].<ref>https://www.dovemed.com/diseases-conditions/papillary-intralymphatic-angioendothelioma-pila/</ref>
+* [[Diagnostic]] studies may include histopathological studies, [[fine needle aspiration]], [[MRI]], and [[CT scan]].<ref>https://www.dovemed.com/diseases-conditions/papillary-intralymphatic-angioendothelioma-pila/</ref>
 * Wide local excision is the treatment of choice. However any combination of [[steroids]], [[chemotherapy]], [[radiation]] therapy, and invasive procedures can be used to treat this tumor.
@@ Line 97: / Line 100: @@
 ===Pseudomyogenic hemangioendothelioma===
-* A locally aggressive [[tumor]] with endothelial differentiation that usually presents as multiple [[asymptomatic]] discontinuous [[lesions]], often at [[extremities]].<ref name="pmid29511030">{{cite journal |vauthors=van IJzendoorn DGP, Sleijfer S, Gelderblom H, Eskens FALM, van Leenders GJLH, Szuhai K, Bovée JVMG |title=Telatinib Is an Effective Targeted Therapy for Pseudomyogenic Hemangioendothelioma |journal=Clin. Cancer Res. |volume=24 |issue=11 |pages=2678–2687 |date=June 2018 |pmid=29511030 |doi=10.1158/1078-0432.CCR-17-3512 |url=}}</ref><ref name="pmid29406432">{{cite journal |vauthors=Raftopoulos E, Royer M, Warren M, Zhao J, Rush W |title=Pseudomyogenic Hemangioendothelioma: Case Report and Review of the Literature |journal=Am J Dermatopathol |volume=40 |issue=8 |pages=597–601 |date=August 2018 |pmid=29406432 |doi=10.1097/DAD.0000000000001104 |url=}}</ref>
+* A locally aggressive [[tumor]] with [[endothelial]] differentiation that usually presents as multiple [[asymptomatic]] discontinuous [[lesions]], often at [[extremities]].<ref name="pmid29511030">{{cite journal |vauthors=van IJzendoorn DGP, Sleijfer S, Gelderblom H, Eskens FALM, van Leenders GJLH, Szuhai K, Bovée JVMG |title=Telatinib Is an Effective Targeted Therapy for Pseudomyogenic Hemangioendothelioma |journal=Clin. Cancer Res. |volume=24 |issue=11 |pages=2678–2687 |date=June 2018 |pmid=29511030 |doi=10.1158/1078-0432.CCR-17-3512 |url=}}</ref><ref name="pmid29406432">{{cite journal |vauthors=Raftopoulos E, Royer M, Warren M, Zhao J, Rush W |title=Pseudomyogenic Hemangioendothelioma: Case Report and Review of the Literature |journal=Am J Dermatopathol |volume=40 |issue=8 |pages=597–601 |date=August 2018 |pmid=29406432 |doi=10.1097/DAD.0000000000001104 |url=}}</ref>
 * SERPINE1-FOSB fusions are characteristic that result in over-expression of truncated form of FOSB. FBJ murine osteosarcoma viral oncogene homolog B, also known as Finkel-Biskis-Jinkins murine osteosarcoma viral oncogene homolog B, FOSB or FosB, is a [[protein]] that, in humans have been implicated as regulators of [[cell proliferation]], [[differentiation]], and transformation.<ref name="pmid29511030">{{cite journal |vauthors=van IJzendoorn DGP, Sleijfer S, Gelderblom H, Eskens FALM, van Leenders GJLH, Szuhai K, Bovée JVMG |title=Telatinib Is an Effective Targeted Therapy for Pseudomyogenic Hemangioendothelioma |journal=Clin. Cancer Res. |volume=24 |issue=11 |pages=2678–2687 |date=June 2018 |pmid=29511030 |doi=10.1158/1078-0432.CCR-17-3512 |url=}}</ref>
 * It may mimic epithelioid [[sarcoma]] on histology but [[metastasis]] is very rare and [[prognosis]] is excellent.<ref name="pmid29406432">{{cite journal |vauthors=Raftopoulos E, Royer M, Warren M, Zhao J, Rush W |title=Pseudomyogenic Hemangioendothelioma: Case Report and Review of the Literature |journal=Am J Dermatopathol |volume=40 |issue=8 |pages=597–601 |date=August 2018 |pmid=29406432 |doi=10.1097/DAD.0000000000001104 |url=}}</ref>
 * [[Diagnostic]] work up includes [[X-ray]], [[MRI]], [[CT scan]] and [[biopsy]] of the [[lesion]].
-* [[Excision]] is the typical treatment but [[chemotherapeutic]] agents including gemcitabine/taxane and mammalian target of rapamycin inhibitor , [[mTOR]] inhibitors such as [[sirolimus]], VEGFR1-4/PDGFRA inhibitors such as telatinib have been used with success in various studies.<ref name="pmid26500758">{{cite journal |vauthors=Joseph J, Wang WL, Patnana M, Ramesh N, Benjamin R, Patel S, Ravi V |title=Cytotoxic and targeted therapy for treatment of pseudomyogenic hemangioendothelioma |journal=Clin Sarcoma Res |volume=5 |issue= |pages=22 |date=2015 |pmid=26500758 |pmc=4615364 |doi=10.1186/s13569-015-0037-8 |url=}}</ref><ref name="pmid28843050">{{cite journal |vauthors=Gabor KM, Sapi Z, Tiszlavicz LG, Fige A, Bereczki C, Bartyik K |title=Sirolimus therapy in the treatment of pseudomyogenic hemangioendothelioma |journal=Pediatr Blood Cancer |volume=65 |issue=2 |pages= |date=February 2018 |pmid=28843050 |doi=10.1002/pbc.26781 |url=}}</ref><ref name="pmid29511030">{{cite journal |vauthors=van IJzendoorn DGP, Sleijfer S, Gelderblom H, Eskens FALM, van Leenders GJLH, Szuhai K, Bovée JVMG |title=Telatinib Is an Effective Targeted Therapy for Pseudomyogenic Hemangioendothelioma |journal=Clin. Cancer Res. |volume=24 |issue=11 |pages=2678–2687 |date=June 2018 |pmid=29511030 |doi=10.1158/1078-0432.CCR-17-3512 |url=}}</ref>
+* [[Excision]] is the typical treatment but [[chemotherapeutic]] agents including [[gemcitabine]]/taxane and mammalian target of [[rapamycin]] inhibitor, [[mTOR]] inhibitors such as [[sirolimus]], VEGFR1-4/PDGFRA inhibitors such as telatinib have been used with success in various studies.<ref name="pmid26500758">{{cite journal |vauthors=Joseph J, Wang WL, Patnana M, Ramesh N, Benjamin R, Patel S, Ravi V |title=Cytotoxic and targeted therapy for treatment of pseudomyogenic hemangioendothelioma |journal=Clin Sarcoma Res |volume=5 |issue= |pages=22 |date=2015 |pmid=26500758 |pmc=4615364 |doi=10.1186/s13569-015-0037-8 |url=}}</ref><ref name="pmid28843050">{{cite journal |vauthors=Gabor KM, Sapi Z, Tiszlavicz LG, Fige A, Bereczki C, Bartyik K |title=Sirolimus therapy in the treatment of pseudomyogenic hemangioendothelioma |journal=Pediatr Blood Cancer |volume=65 |issue=2 |pages= |date=February 2018 |pmid=28843050 |doi=10.1002/pbc.26781 |url=}}</ref><ref name="pmid29511030">{{cite journal |vauthors=van IJzendoorn DGP, Sleijfer S, Gelderblom H, Eskens FALM, van Leenders GJLH, Szuhai K, Bovée JVMG |title=Telatinib Is an Effective Targeted Therapy for Pseudomyogenic Hemangioendothelioma |journal=Clin. Cancer Res. |volume=24 |issue=11 |pages=2678–2687 |date=June 2018 |pmid=29511030 |doi=10.1158/1078-0432.CCR-17-3512 |url=}}</ref>
 ===Polymorphous hemangioendothelioma===
-* A rare [[vascular]] [[neoplasm]], Polymorphous hemangioendothelioma occurs in [[lymph nodes]], but a few cases have been found in extra-nodal sites such as the [[mediastinum]], [[spinal cord]], and [[liver]]. It is a very rare cause of persistent [[lymphadenopathy]]. The data on [[natural history]] and clinical presentation is limited due to very few number of cases reported. <ref name="pmid27913780">{{cite journal |vauthors=El Hussein S, Omarzai Y |title=Multifocal Polymorphous Hemangioendothelioma of the Liver: Case Report and Review of Literature |journal=Int. J. Surg. Pathol. |volume=25 |issue=3 |pages=266–270 |date=May 2017 |pmid=27913780 |doi=10.1177/1066896916679517 |url=}}</ref>
+* A rare [[vascular]] [[neoplasm]], polymorphous hemangioendothelioma occurs in [[lymph nodes]], but a few cases have been found in extra-nodal sites such as the [[mediastinum]], [[spinal cord]], and [[liver]]. It is a very rare cause of persistent [[lymphadenopathy]]. The data on [[natural history]] and clinical presentation is limited due to very few number of cases reported.<ref name="pmid27913780">{{cite journal |vauthors=El Hussein S, Omarzai Y |title=Multifocal Polymorphous Hemangioendothelioma of the Liver: Case Report and Review of Literature |journal=Int. J. Surg. Pathol. |volume=25 |issue=3 |pages=266–270 |date=May 2017 |pmid=27913780 |doi=10.1177/1066896916679517 |url=}}</ref>
 * Characterized by a polymorphous blend of solid, primitive [[vascular]] and angiomatous areas in varied proportions on microscopic examination.<ref name="pmid27913780">{{cite journal |vauthors=El Hussein S, Omarzai Y |title=Multifocal Polymorphous Hemangioendothelioma of the Liver: Case Report and Review of Literature |journal=Int. J. Surg. Pathol. |volume=25 |issue=3 |pages=266–270 |date=May 2017 |pmid=27913780 |doi=10.1177/1066896916679517 |url=}}</ref>
-* [[Diagnotic]] work up includes histopathological examination, [[MRI]] and [[CT scan]].
+* [[Diagnotic]] work up includes histopathological examination, [[MRI]], and [[CT scan]].
-* Wide local excisio has been used for treatment, with [[radiation]] therapy in case of [[recurrence]].n<ref name="pmid12808568">{{cite journal |vauthors=Tadros M, Rizk SS, Opher E, Thompson LD |title=Polymorphous hemangioendothelioma of the neck |journal=Ann Diagn Pathol |volume=7 |issue=3 |pages=165–8 |date=June 2003 |pmid=12808568 |doi= |url=}}</ref><ref name="pmid19366064">{{cite journal |vauthors=Falleti J, Siano M, De Cecio R, Somma A, Pettinato G, Insabato L |title=Nodal and extranodal soft tissue polymorphous hemangioendothelioma: a case report and review of the literature |journal=Tumori |volume=95 |issue=1 |pages=94–7 |date=2009 |pmid=19366064 |doi= |url=}}</ref>
+* Wide local excision has been used for treatment, with [[radiation]] therapy in case of [[recurrence]].<ref name="pmid12808568">{{cite journal |vauthors=Tadros M, Rizk SS, Opher E, Thompson LD |title=Polymorphous hemangioendothelioma of the neck |journal=Ann Diagn Pathol |volume=7 |issue=3 |pages=165–8 |date=June 2003 |pmid=12808568 |doi= |url=}}</ref><ref name="pmid19366064">{{cite journal |vauthors=Falleti J, Siano M, De Cecio R, Somma A, Pettinato G, Insabato L |title=Nodal and extranodal soft tissue polymorphous hemangioendothelioma: a case report and review of the literature |journal=Tumori |volume=95 |issue=1 |pages=94–7 |date=2009 |pmid=19366064 |doi= |url=}}</ref>
 ===Kaposi sarcoma===
-* An [[AIDS]]-associated [[vascular]] [[malignancy]] that usually presents as [[mucocutaneous]] [[lesions]] <ref name="pmid30191128">{{cite journal |vauthors=Khan S, Guevara J, Barbosa A, Ayuby A, Bien-Aime F, Verda L, Glick N, Mehta V |title=Primary pulmonary Kaposi Sarcoma in a newly diagnosed cisgender heterosexual HIV positive patient presenting before cutaneous manifestations |journal=IDCases |volume=14 |issue= |pages=e00420 |date=2018 |pmid=30191128 |pmc=6125769 |doi=10.1016/j.idcr.2018.e00420 |url=}}</ref> but can also occur in viscera such as lungs. It can remain confined to skin but widespread visceral involvement may occur.
+* An [[AIDS]]-associated [[vascular]] [[malignancy]] that usually presents as [[mucocutaneous]] [[lesions]] but can also occur in viscera such as lungs. It can remain confined to skin but widespread visceral involvement may occur.<ref name="pmid30191128">{{cite journal |vauthors=Khan S, Guevara J, Barbosa A, Ayuby A, Bien-Aime F, Verda L, Glick N, Mehta V |title=Primary pulmonary Kaposi Sarcoma in a newly diagnosed cisgender heterosexual HIV positive patient presenting before cutaneous manifestations |journal=IDCases |volume=14 |issue= |pages=e00420 |date=2018 |pmid=30191128 |pmc=6125769 |doi=10.1016/j.idcr.2018.e00420 |url=}}</ref>
-* There are three known variants
+* There are three known variants:
 ** One variant occurs spontaneously in Jewish and Italian males in Europe and the United States.
 ** Another more aggressive variant is endemic in young children is endemic in Africa.
@@ Line 119: / Line 122: @@
 =Malignant vascular tumors=
 ===Angiosarcoma===
-* [[Angiosarcoma]](AS) is [[malignancy]] that presents with a very heterogeneous distribution in the human [[body]] with aggressive clinical course, and may appear in multiple locations, from [[breast]] to [[liver]] or [[skin]].<ref name="pmid30217704">{{cite journal |vauthors=Villaescusa Catalan JM, Martín IG, Cagigal Cobo ML |title=Popliteal Angiosarcoma After Bypass With Autologous Saphenous Vein |journal=Ann Vasc Surg |volume= |issue= |pages= |date=September 2018 |pmid=30217704 |doi=10.1016/j.avsg.2018.06.034 |url=}}</ref>
+* [[Angiosarcoma]] (AS) is [[malignancy]] that presents with a very heterogeneous distribution in the human [[body]] with aggressive clinical course, and may appear in multiple locations, from [[breast]] to [[liver]] or [[skin]].<ref name="pmid30217704">{{cite journal |vauthors=Villaescusa Catalan JM, Martín IG, Cagigal Cobo ML |title=Popliteal Angiosarcoma After Bypass With Autologous Saphenous Vein |journal=Ann Vasc Surg |volume= |issue= |pages= |date=September 2018 |pmid=30217704 |doi=10.1016/j.avsg.2018.06.034 |url=}}</ref>
 * Associated with MYC gene amplification and [[protein]] overexpression. Myc is a family of regulator genes and [[proto-oncogenes]] that code for [[transcription factors]].<ref name="pmid27780597">{{cite journal |vauthors=Udager AM, Ishikawa MK, Lucas DR, McHugh JB, Patel RM |title=MYC immunohistochemistry in angiosarcoma and atypical vascular lesions: practical considerations based on a single institutional experience |journal=Pathology |volume=48 |issue=7 |pages=697–704 |date=December 2016 |pmid=27780597 |doi=10.1016/j.pathol.2016.08.007 |url=}}</ref>
 * Complete [[surgical excision]] and [[radiotherapy]] are the main treatments, with a minor role of [[chemotherapy]].<ref name="pmid30179666">{{cite journal |vauthors=Priyakumari T, Chandar R, Jayasree K, Ramachandran K |title=Pediatric Primary Ovarian Angiosarcoma: From rarity to a realization |journal=J Pediatr Adolesc Gynecol |volume= |issue= |pages= |date=September 2018 |pmid=30179666 |doi=10.1016/j.jpag.2018.08.008 |url=}}</ref>
-For more information about [[angiosarcoma]], [[Angiosarcoma#Angiosarcoma|Click here]].
+For more information about [[angiosarcoma]], [[Angiosarcoma|click here]].
 ===Epithelioid hemangioendothelioma===
-* A rare [[vascular]] [[tumor]], described for the first time in 1975 by Dail and Liebow,that usually affects [[lung]], [[liver]] and [[bones]], although may occur many other sites in [[body]] including [[head and neck]], [[breasts]] and [[lymph nodes]].<ref name="pmid25992243">{{cite journal |vauthors=Sardaro A, Bardoscia L, Petruzzelli MF, Portaluri M |title=Epithelioid hemangioendothelioma: an overview and update on a rare vascular tumor |journal=Oncol Rev |volume=8 |issue=2 |pages=259 |date=September 2014 |pmid=25992243 |pmc=4419652 |doi=10.4081/oncol.2014.259 |url=}}</ref>
+* A rare [[vascular]] [[tumor]], described for the first time in 1975 by Dail and Liebow, that usually affects [[lung]], [[liver]] and [[bones]], although may occur many other sites in [[body]] including [[head and neck]], [[breasts]] and [[lymph nodes]].<ref name="pmid25992243">{{cite journal |vauthors=Sardaro A, Bardoscia L, Petruzzelli MF, Portaluri M |title=Epithelioid hemangioendothelioma: an overview and update on a rare vascular tumor |journal=Oncol Rev |volume=8 |issue=2 |pages=259 |date=September 2014 |pmid=25992243 |pmc=4419652 |doi=10.4081/oncol.2014.259 |url=}}</ref>
 * Usually [[asymptomatic]] but patient may present with [[respiratory]] symptoms, [[bone]] pains or other symptoms depending on the site of the [[tumor]].
 * Majority are characterized by a reciprocal t(1;3)(p36;q25) [[translocation]]. The t(1;3) results in fusion of a [[gene]] known as WWTR1 (or TAZ) to CAMTA1. These [[genes code]] for [[transcription factors]].<ref name="pmid21584898">{{cite journal |vauthors=Errani C, Zhang L, Sung YS, Hajdu M, Singer S, Maki RG, Healey JH, Antonescu CR |title=A novel WWTR1-CAMTA1 gene fusion is a consistent abnormality in epithelioid hemangioendothelioma of different anatomic sites |journal=Genes Chromosomes Cancer |volume=50 |issue=8 |pages=644–53 |date=August 2011 |pmid=21584898 |pmc=3264678 |doi=10.1002/gcc.20886 |url=}}</ref><ref name="pmid21885404">{{cite journal |vauthors=Tanas MR, Sboner A, Oliveira AM, Erickson-Johnson MR, Hespelt J, Hanwright PJ, Flanagan J, Luo Y, Fenwick K, Natrajan R, Mitsopoulos C, Zvelebil M, Hoch BL, Weiss SW, Debiec-Rychter M, Sciot R, West RB, Lazar AJ, Ashworth A, Reis-Filho JS, Lord CJ, Gerstein MB, Rubin MA, Rubin BP |title=Identification of a disease-defining gene fusion in epithelioid hemangioendothelioma |journal=Sci Transl Med |volume=3 |issue=98 |pages=98ra82 |date=August 2011 |pmid=21885404 |doi=10.1126/scitranslmed.3002409 |url=}}</ref>

Vascular tumor: Difference between revisions

Latest revision as of 18:51, 26 October 2018

Overview

Classification

Benign vascular tumors

Locally aggressive or borderline vascular tumors

Kaposiform hemangioendothelioma

Retiform hemangioendothelioma

Papillary intralymphatic angioendothelioma (PILA), Dabska tumor

Composite hemangioendothelioma

Pseudomyogenic hemangioendothelioma

Polymorphous hemangioendothelioma

Kaposi sarcoma

Malignant vascular tumors

Angiosarcoma

Epithelioid hemangioendothelioma

References

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