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* 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 | * 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.<ref>https://www.dovemed.com/diseases-conditions/papillary-intralymphatic-angioendothelioma-pila/</ref> | ||
====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.<ref>29233122</ref> | |||
* 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.<ref>26050262</ref> | |||
* It may recur locally and has the potential to metastasize. Recurrence was found to be in 8/10 cases in some studies. <ref>https://www.jpatholtm.org/upload/pdf/kjp-40-2-142.pdf</ref> | |||
* Diagnostic work up must include biopsy because of heterogeneity of lesions and it must be differentiated from other vascular tumors.<ref>29233122</ref> | |||
* Surgical excision is the treatment of choice although some patients have been treated with interferon and electron beams.<ref>29233122</ref> | |||
===Physiology=== | ===Physiology=== | ||
Revision as of 16:37, 21 September 2018
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hannan Javed, M.D.[2]
Overview
| Vascular Anomalies | ||||
|---|---|---|---|---|
| Vascular Tumors | Vascular Malformations | |||
| Benign
Locally aggressive or Borderline Malignant |
Simple | Combined° | of major named vessels | associated with other anomalies |
| Capillary malformations
Lymphatic malformations Venous malformations Arteriovenous malformations* Arteriovenous fistula* |
Capillary venous malformation , Capillary lymphatic malformation
Lymphatic venous malformation, Capillary lymphatic venous malformation Capillary arteriovenous malformation Capillary lymphatic arteriovenous malformation others |
See details | See list | |
° defined as two or more vascular malformations found in one lesion
* high flow lesions
Classification
Classification of Vascular Malformations
| Vascular malformations | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Simple | Combined | of major named vessels | asscoiated with other anomalies | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Combined vascular malformations*
| Anomalies of major named vessels (also known as "channel type" or "truncal" vascular malformations) | Vascular malformations associated with other anomalies
| |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Capillary malformations | Lymphatic malformations | Venous malformations | Arteriovenous malformations | Arteriovenous fistula | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Nevus simplex / salmon patch, “angel kiss”, “stork bite” | Common (cystic) LM Macrocystic LM Microcystic LM Mixed cystic LM | Common VM | Sporadic | Sporadic | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Cutaneous and/or mucosal CM (also known as “port-wine” stain) Nonsyndromic CM CM with CNS and/or ocular anomalies (Sturge-Weber syndrome) CM with bone and/or soft tissues overgrowth Diffuse CM with overgrowth (DCMO) | Generalized lymphatic anomaly (GLA) Kaposiform lymphangiomatosis (KLA) | Familial VM cutaneo-mucosal (VMCM) | In HHT | In HHT | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Reticulate CM CM of MIC-CAP (microcephaly-capillary malformation) CM of MCAP (megalencephaly-capillary malformation-polymicrogyria) | LM in Gorham-Stout disease | Blue rubber bleb nevus (Bean) syndrome VM | In CM-AVM | In CM-AVM | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| CM of CM-AVM | Channel type LM | Glomuvenous malformation (GVM) | Others | Others | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Cutis marmorata telangiectatica congenita (CMTC) | “Acquired” progressive lymphatic anomaly (so called acquired progressive "lymphangioma") | Cerebral cavernous malformation (CCM) | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Others | Primary lymphedema | Familial intraosseous vascular malformation (VMOS) | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Telangiectasia Hereditary hemorrhagic telangiectasia (HHT) Others | Others | Verrucous venous malformation (formerly verrucous hemangioma) | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Others | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Tables
| Anomalies of major named vessels
(also known as "channel type" or "truncal" vascular malformations) |
|---|
Affect
lymphatics veins arteries Anomalies of origin course number length diameter (aplasia, hypoplasia, stenosis, ectasia / aneurysm) valves communication (AVF) persistence (of embryonal vessel) |
| Combined vascular malformations* | ||
|---|---|---|
| CM + VM | capillary-venous malformation | CVM |
| CM + LM | capillary-lymphatic malformation | CLM |
| CM + AVM | capillary-arteriovenous malformation | CAVM |
| LM + VM | lymphatic-venous malformation | LVM |
| CM + LM + VM | capillary-lymphatic-venous malformation | CLVM |
| CM + LM + AVM | capillary-lymphatic-arteriovenous malformation | CLAVM |
| CM + VM + AVM | capillary-venous-arteriovenous malformation | CVAVM |
| CM + LM + VM + AVM | capillary-lymphatic-venous-arteriovenous m. | CLVAVM |
| Vascular malformations associated with other anomalies | ||
|---|---|---|
| Klippel-Trenaunay syndrome * | CM + VM +/-LM + limb overgrowth | |
| Parkes Weber syndrome | CM + AVF + limb overgrowth | |
| Servelle-Martorell syndrome | limb VM + bone undergrowth | |
| Sturge-Weber syndrome | facial + leptomeningeal CM + eye anomalies
+/-bone and/or soft tissue overgrowth | |
| Limb CM + congenital non-progressive limb overgrowth | ||
| Maffucci syndrome | VM +/-spindle-cell hemangioma + enchondroma | |
| Macrocephaly-CM (M-CM / MCAP) * | ||
| Microcephaly-CM (MICCAP) | ||
| CLOVES syndrome * | LM + VM + CM +/-AVM+ lipomatous overgrowth | |
| Proteus syndrome | CM, VM and/or LM + asymmetrical somatic overgrowth | |
| Bannayan-Riley-Ruvalcaba sd | lower lip CM + face and neck LM + asymmetry and partial/generalized overgrowth | |
Vascular Tumors
Locally aggressive or borderline vascular tumors
Kaposiform hemangioendothelioma
- Locally Aggressive tumor that originates on skin and occurs primarily in childhood.[1] It is characterized by a single or multiple masses with following characteristics:
** 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.[2] 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.[3]
- Invasion of bone, retroperitoneum, and mediastinum has occured in some cases but no case of metastasis has been reported yet. [4]
- 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]
Retiform hemangioendothelioma
- First described in 1994 as a form of low grade angiosarcoma[6], Retiform hemangioendothelioma commonly presents as a slow growing asymptomatic solitary nodule or plaque on distal extremities in 2nd-4th decade of life.
- Must be differentiated from Angiosarcoma.
- High level of local recurrence but very low potential for metastasis.
- Diagnostic work up includes histopathological studies, that shows arborizing blood vessels are arranged in retiform pattern [7], and MRI.
- Surgery is the treatment of choice, though 2/3rd cases recur. Adjuvant radiotherapy and ddjuvant chemotherapy with recombinant interferon alpha and low dose cisplatin have also been reported in selected cases. [8]
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.[9]
- 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.[10]
- 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.[11]
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.[12]
- 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.[13]
- It may recur locally and has the potential to metastasize. Recurrence was found to be in 8/10 cases in some studies. [14]
- Diagnostic work up must include biopsy because of heterogeneity of lesions and it must be differentiated from other vascular tumors.[15]
- Surgical excision is the treatment of choice although some patients have been treated with interferon and electron beams.[16]
Physiology
Medical Therapy
Naural History
Historical Perspective
References
- ↑ C537007
- ↑ D059885
- ↑ 27476652
- ↑ C537007
- ↑ 30054848
- ↑ 25484427
- ↑ 25484427
- ↑ 25484427
- ↑ © 1999 Lippincott Williams & Wilkins, Inc.
- ↑ https://www.dovemed.com/diseases-conditions/papillary-intralymphatic-angioendothelioma-pila/
- ↑ https://www.dovemed.com/diseases-conditions/papillary-intralymphatic-angioendothelioma-pila/
- ↑ 29233122
- ↑ 26050262
- ↑ https://www.jpatholtm.org/upload/pdf/kjp-40-2-142.pdf
- ↑ 29233122
- ↑ 29233122