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__NOTOC__
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{{CMG}}
{{CMG}} {{AE}} [[Parnian Jabbari]]
{{SI}}
{{SI}}
==Overview==
==Overview==
Granulocytic sarcoma (GS, also known as chloroma) was first discovered by Allen Burns, a British physician, in 1811. The term chloroma was first used by King to address the greenish appearance of the tumor due to [[myeloperoxidase]]. The association of the GS with [[Acute myeloid leukemia|acute myeloid leukemia (AML)]] was first recognized by Dock in 1902. GS can be classified into two categories based on its co-occurence with other malignancies. Infiltration of the tumor with [[myeloblasts]] is the main characteristic of the tumor on [[H&E stain]]. GS rises from primitive precursors of [[granulocytes]]. The disease is an extramedullary manifestation of myeloid diseases, however, it can occur as a primary disease. Aggregation of [[myeloblasts]], [[promyelocytes]] and [[Myelocyte|myelocytes]] outside of the bone marrow presents itself as these solid tumors. Tumors can occur at any site and can appear as green, gray, white or brown masses. GS must be differentiated from other diseases that can present as extramedullary solid tumors. All patients with GS must be evaluated for concurrent or future malignancies as GS can occur in the course of or prior to other malignancies. The prevalence of GS is approximately 2 per 1,000,000 individuals worldwide. The most important risk factor for development of GS is genetic mutations and susceptibility. Symptoms of GS may include the following: Symptoms due to mass effect such as [[deafness]], [[ptosis]], altered vision, intestinal obstruction, headache, neck pain, abdominal pain, and constitutional symptoms. [[Chemotherapy]] is the main stain of treatment in patients with GS. Even patients with isolated GS must receive systemic treatment to better the prognosis.
==Historical Perspective==
==Historical Perspective==
*[[File:Screenshot 2019-05-02 Myeloid sarcoma.png|thumb|Plain radiograph of femur in a patient with GS. Courtesy of image: pathology outlines (http://www.pathologyoutlines.com/topic/bonemyeloidsarcoma.html)]]Granulocytic sarcoma (GS, also known as chloroma) was first discovered by Allen Burns, a British physician, in 1811 <ref>{{Cite journal|last=Burns|first=Allen|date=|title=Observations of surgical anatomy, in Head andNeck.|url=|journal=London, England, Royce, 1811|volume=|pages=364-366|via=}}</ref>.
*[[File:Screenshot 2019-05-02 Myeloid sarcoma.png|thumb|Plain radiograph of femur in a patient with GS. Courtesy of image: pathology outlines (http://www.pathologyoutlines.com/topic/bonemyeloidsarcoma.html)]]Granulocytic sarcoma (GS, also known as chloroma) was first discovered by Allen Burns, a British physician, in 1811 <ref>{{Cite journal|last=Burns|first=Allen|date=|title=Observations of surgical anatomy, in Head andNeck.|url=|journal=London, England, Royce, 1811|volume=|pages=364-366|via=}}</ref>.
*The term chloroma was first used by King to address the greenish appearance of the tumor due to myeloperoxidase.
*The term chloroma was first used by King to address the greenish appearance of the tumor due to [[myeloperoxidase]].
*The association of the GS with acute myeloid leukemia (AML) was first recognized bt Dock in 1902 <ref>{{Cite journal|last=Dock G, Warthin AS|first=|date=|title=A new case of chloroma withleukemia.|url=|journal=Trans Assoc Am Phys, 1904|volume=19:64|pages=115|via=}}</ref>.
*The association of the GS with [[Acute myeloid leukemia|acute myeloid leukemia (AML)]] was first recognized by Dock in 1902 <ref>{{Cite journal|last=Dock G, Warthin AS|first=|date=|title=A new case of chloroma withleukemia.|url=|journal=Trans Assoc Am Phys, 1904|volume=19:64|pages=115|via=}}</ref>.
*The term "granulocytic sarcoma" was suggested by Rappaport in 1967 to grant generalisability to it <ref>{{Cite book|title=Tumors of the hematopoietic system, inAtlas of Tumor Pathology, Section III|last=Rappaport H|first=|publisher=Fascicle 8. ArmedForces Institute of Pathology|year=1967|isbn=|location=Washington|pages=241-247}}</ref>.
*The term "granulocytic sarcoma" was suggested by Rappaport in 1967 to grant generalisability to it <ref>{{Cite book|title=Tumors of the hematopoietic system, inAtlas of Tumor Pathology, Section III|last=Rappaport H|first=|publisher=Fascicle 8. ArmedForces Institute of Pathology|year=1967|isbn=|location=Washington|pages=241-247}}</ref>.
==Classification==
==Classification==
:* GS can be classified into two categories based on its co-occurence with other malignancies:
* GS can be classified into two categories based on its co-occurence with other malignancies:
* Infiltration of the tumor with myeloblasts is the main characteristic of the tumor on H&E stain.
* Infiltration of the tumor with [[myeloblasts]] is the main characteristic of the tumor on [[H&E stain]].
* GS rises from primitive precursors of granulocytes.
* GS rises from primitive precursors of [[granulocytes]].
* The disease is an extramedullary manifestation of myeloid diseases, however, it can occur as a primary disease.
* The disease is an extramedullary manifestation of myeloid diseases, however, it can occur as a primary disease.
* Aggregation of myeloblasts, promyelocytes and myelocytes outside of the bone marrow presents itself as these solid tumors.
* Aggregation of [[myeloblasts]], [[Promyelocyte|promyelocytes]] and [[Myelocyte|myelocytes]] outside of the bone marrow presents itself as these solid tumors.
* Tumors can occur at any site and can appear as green, gray, white or brown masses.
* Tumors can occur at any site and can appear as green, gray, white or brown masses.
==Clinical Features==
==Differentiating GS from other Diseases==
==Differentiating GS from other Diseases==
*GS must be differentiated from other diseases that can present as extramedullary solid tumors, such as:
GS must be differentiated from other diseases that can present as extramedullary solid tumors, such as:
:*Large cell lymphoma
* [[Lymphoma|Large cell lymphoma]]
:*Non-Hodgkin lymphoma
* Non-Hodgkin lymphoma
:*Thymoma
* [[Thymoma]]
:*Myeloma
* [[Myeloma]]
:*Esosinophilic sarcoma
* [[Sarcoma|Esosinophilic sarcoma]]
:*Ewing sarcoma
* [[Ewing sarcoma]]
:*Extramedullary sites of hematopoiesis
* Extramedullary sites of [[hematopoiesis]]
:*Burkitt lymphoma
* [[Burkitt lymphoma]]
:*Hypereosinophilic syndrome
* [[Hypereosinophilic syndrome]]
:*Polycythemia vera
* [[Polycythemia vera]]
:All patients with GS must be evaluated for concurrent or future malignancies as GS can occur in the course of or prior to other malignancies.
All patients with GS must be evaluated for concurrent or future malignancies as GS can occur in the course of or prior to other malignancies.
==Epidemiology and Demographics==
==Epidemiology and Demographics==
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===Age===
===Age===
* Patients of all age groups may develop GS.
* Patients of all age groups may develop GS.
* GS associated with [[AML]] occurs more commonly in children.
* GS associated with AML occurs more commonly in children.
===Gender===
===Gender===
*GS affects both men and women.
* GS affects both men and women.
* Due to the rarity of the disease it is not clear whether there is a gender predilection for it.
*Due to the rarity of the disease it is not clear whether there is a gender predilection for it.
===Race===
===Race===
*There is no racial predilection for GS.
* There is no racial predilection for GS.
==Risk Factors==
==Risk Factors==
*Risk factors for GS are usually chromosomal aberrations and include<ref name=":0">{{Cite journal|last=Daniela Dörfel et al.|first=|date=2016|title=Cardiac Myeloid Sarcoma: Multimodality Radiologic Imaging Features and Pathologic Correlation|url=https://www.amjmed.com/article/S0002-9343(16)30356-4/fulltext|journal=The American Journal of Medicine|volume=129|pages=e117-e120|via=}}</ref>:
* Risk factors for GS are usually chromosomal aberrations and include<ref name=":0">{{Cite journal|last=Daniela Dörfel et al.|first=|date=2016|title=Cardiac Myeloid Sarcoma: Multimodality Radiologic Imaging Features and Pathologic Correlation|url=https://www.amjmed.com/article/S0002-9343(16)30356-4/fulltext|journal=The American Journal of Medicine|volume=129|pages=e117-e120|via=}}</ref>:
**Trisomy 8
** [[Trisomy 8]]
**Monosomy 7
** Monosomy 7
**MLL gene rearrangement
** MLL gene rearrangement
**NPM1 mutations
** [[NPM1]] mutations
**FLT3 mutations
** [[FLT3]] mutations
== Natural History, Complications and Prognosis==
== Natural History, Complications and Prognosis==
*How GS evolves over time depends on the co-occurence of the disease with other malignancies.
* GS evolve over time, that depends on the co-occurence of the disease with other malignancies.
*GS may present before evidences of other malignancies manifest or after these malignancies are evident.
* GS may present before evidences of other malignancies manifest or after these malignancies are evident.
*Symptoms of the isolated GS depends on the location and the site of the tumor.
* Symptoms of the isolated GS depends on the location and the site of the tumor.
*Majority of cases are associated with AML or other myeloproliferative/myelodysplastic syndromes.
* Majority of cases are associated with [[AML]] or other [[myeloproliferative]]/[[Myelodysplastic syndrome|myelodysplastic syndromes]].
*Majority of GS tumors are found in the soft tissues such as the peritoneum, lymph nodes, CNS and skin. They are also found in bone and periosteum.
* Majority of GS tumors are found in the soft tissues such as the peritoneum, lymph nodes, CNS and skin. They are also found in bone and [[periosteum]].
*Early clinical features include weight loss, weakness. Other manifestations of the tumor depend on its size and location.
* Early clinical features include weight loss, fatigue. Other manifestations of the tumor depend on its size and location.
*Prognosis of GS depends on its association with other malignacies. In cases of isolated GS the prognosis is good. However, GS associated with myeloproliferative disorders has poor prognosis.
* Prognosis of GS depends on its association with other malignacies. In cases of isolated GS the prognosis is good. However, GS associated with [[myeloproliferative disorders]] has poor prognosis.
*Prognosis of isolated GS with chromosome 8 abnormalities is worse than other cases of isolated GS.
* Prognosis of isolated GS with chromosome 8 abnormalities is worse than other cases of isolated GS.
== Diagnosis ==
== Diagnosis ==
===Diagnostic Criteria===
===Diagnostic Criteria===
*There are no predefined criteria for diagnosis of granulocytic sarcoma.
* There are no predefined criteria for diagnosis of granulocytic sarcoma.
*Granulocytic sarcoma must be suspected in patients with AML or myelodysplatic syndromes. Diagnosis must be confirmed with histopathologic study of the specimen.
* Granulocytic sarcoma must be suspected in patients with [[AML]] or [[Myelodysplastic syndrome|myelodysplastic syndromes]]. Diagnosis must be confirmed with histopathologic study of the specimen.
=== Symptoms ===
=== Symptoms ===
*Symptoms of GS may include the following:
Symptoms of GS may include the following:
:*Symptoms due to mass effect such as deafness, ptosis, altered vision, intestinal obstruction, etc.
* Symptoms due to mass effect such as [[deafness]], [[ptosis]], altered vision, intestinal obstruction, etc.
:*headache, neck pain, abdominal pain,etc. based on the site of the tumor
* [[Headache]], neck pain, abdominal pain,etc. based on the site of the tumor
:*constitutional symptoms such as fever, fatigue, etc.
* Constitutional symptoms such as fever, fatigue, etc.
=== Physical Examination ===
Patients with GS can present with varying presentations.
=== Physical Examination ===
Physical examination may be remarkable for:
*Patients with GS can present with varying presentations.
* [[Lymphadenopathy]] (in cases associated with [[AML]] and other [[Myeloproliferative syndrome|myeloproliferative syndromes]])
*Physical examination may be remarkable for:
* Skin lesions (of varying colors such as green, grey, brown, etc.)
:*Lymph node enlargement (in cases associated with AML and other myeloproliferative syndromes)
* Organ enlargement such as [[hepatosplenomegaly]]
:*skin lesions (of varying colors such as green, grey, brown, etc.)
* [[Petechiae]] in patients with [[thrombocytopenia]]
:*organ enlargement such as hepatosplenomegaly.
* [[Hearing loss]]
:*petechiae in patients with thrombocytopenia
* [[Heart murmurs]] in cases with heart chamber masses
:*hearing loss
* [[Crackles]] on lung auscultation
:*heart murmurs in cases with heart chamber masses
* [[Abdominal distension|Abdominal distention]]/tenderness in cases with intestinal obstruction
:*crackles on lung auscultation
* Limb swelling due to different pathologies such as [[Deep vein thrombosis|deep vein thrombosis (DVT)]]
:*abdominal distention/tenderness in cases with intestinal obstruction
:*limb swelling due to different pathologies such as deep vein thrombosis (DVT).
=== Laboratory Findings ===
=== Laboratory Findings ===
*In cases associated with AML/CML anemia, thrombocytopenia with normal, low or high white blood cells can be present.
* In cases associated with [[AML]]/[[CML]], [[anemia]], [[thrombocytopenia]] with normal, low or high white blood cells can be present.
*In cases associated with polycythemia vera, thrombocytosis and high levels of hemoglobin is present in complete blood count (CBC).
* In cases associated with [[Polycythemia vera CT|polycythemia vera]], [[thrombocytosis]] and high levels of hemoglobin is present in complete blood count (CBC).
*High eosinophil levels can be present in CBC.
* High [[eosinophil]] levels can be present in CBC.
===Imaging Findings===
===Imaging Findings===
*In cases of CNS involvement, magnetic resonance imaging (MRI) or CT scan of the CNS can reveal extra-axial masses.
* In cases of CNS involvement, magnetic resonance imaging (MRI) or CT scan of the CNS can reveal extra-axial masses.
*In cases with soft tissue involvement, sonogram of the tissue can reveal the mass.
* In cases with soft tissue involvement, sonogram of the tissue can reveal the mass.
*GS appears as<ref>{{Cite journal|last=Guermazi et al.|first=|date=2002|title=Granulocytic sarcoma (chloroma): imaging findings in adults and children|url=https://www.ajronline.org/doi/full/10.2214/ajr.178.2.1780319|journal=American Journal of Roentgenology|volume=178|pages=319-25|via=}}</ref>:
GS appears as<ref>{{Cite journal|last=Guermazi et al.|first=|date=2002|title=Granulocytic sarcoma (chloroma): imaging findings in adults and children|url=https://www.ajronline.org/doi/full/10.2214/ajr.178.2.1780319|journal=American Journal of Roentgenology|volume=178|pages=319-25|via=}}</ref>:
:*hyperdense/isodence to brain/muscle in CT scan without enhancement
* Hyperdense/isodence to brain/muscle in CT scan without enhancement
:*isointense/hyperintense on T2-weighted MRI
* Isointense/hyperintense on T2-weighted MRI
:*isointemse/hypointense on T1-weighted MRI
* Isointemse/hypointense on T1-weighted MRI
[[File:Chloroma histopathology micrograph (H&E stain).jpeg|thumb|Histopathologic micrograph of GS. Infiltration with myeloblasts which can be seen in the course of AML. Courtesy of image: Wikipedia (By Nephron - Own work, CC BY-SA 3.0, <nowiki>https://commons.wikimedia.org/w/index.php?curid=15893726</nowiki>)]]
[[File:Chloroma histopathology micrograph (H&E stain).jpeg|thumb|Histopathologic micrograph of GS. Infiltration with myeloblasts which can be seen in the course of AML. Courtesy of image: Wikipedia (By Nephron - Own work, CC BY-SA 3.0, <nowiki>https://commons.wikimedia.org/w/index.php?curid=15893726</nowiki>)]]Abdominal plain radiogram can reveal obstruction, [[intussusception]], etc.
*Abdominal plain radiogram can reveal obstruction, intussusception, etc.
*Echocardiogram may reveal mobile masses in any heart chamber in cases of heart involvement.
Echocardiogram may reveal mobile masses in any heart chamber in cases of heart involvement.
*Chest radiographs can show lymph node enlargement and consolidation.
Chest radiographs can show lymph node enlargement and consolidation.
=== Other Diagnostic Studies ===
=== Other Diagnostic Studies ===
*Peripheral blood smear can reveal circulating blasts.
* Peripheral blood smear can reveal circulating [[Blast|blasts]].
*Flow cytometry can help differentiate AML from acute lymphoblastic leukemia (ALL).
* [[Flow cytometry]] can help differentiate [[AML]] from [[Acute lymphoblastic leukemia|acute lymphoblastic leukemia (ALL)]].
*Histopathologic analysis of biopsy specimen retrieved by excision or fine needle aspiration shows abundant myeloblasts.
* Histopathologic analysis of biopsy specimen retrieved by excision or [[fine needle aspiration]] shows abundant [[myeloblasts]].
*Histopathologic analysis of GS lesions reveals high infiltration with myeloblasts.
* Histopathologic analysis of GS lesions reveals high infiltration with [[myeloblasts]].
== Treatment ==
== Treatment ==
=== Medical Therapy ===
=== Medical Therapy ===
*Chemotherapy is the main stain of treatment in patients with GS. Even patients with isolated GS must receive systemic treatment to better the prognosis.
* [[Chemotherapy]] is the main stain of treatment in patients with GS. Even patients with isolated GS must receive systemic treatment to better the prognosis.
*Patients receiving cytarabine have better prognosis<ref name=":0" />.
* Patients receiving [[cytarabine]] have better prognosis<ref name=":0" />.
*Patients with chemotherpeutic regimens accepted for AML had longer period of progression to AML.
* Patients with chemotherpeutic regimens accepted for [[AML]] had longer period of progression to [[AML]].
*Treatment includes different regimens<ref>{{Cite journal|last=Yilmaz, A. F., Saydam, G., Sahin, F., & Baran, Y.|first=|date=2013|title=Granulocytic sarcoma: a systematic review|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3875275/|journal=American Journal of Blood Research|volume=|pages=|via=}}</ref>:
Treatment includes different regimens<ref>{{Cite journal|last=Yilmaz, A. F., Saydam, G., Sahin, F., & Baran, Y.|first=|date=2013|title=Granulocytic sarcoma: a systematic review|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3875275/|journal=American Journal of Blood Research|volume=|pages=|via=}}</ref>:
:*idarubicine and cytarabine
* Idarubicine and [[cytarabine]]
:*fludarabine and cytarabine
* [[Fludarabine]] and [[cytarabine]]
:*idarubicine and G-CSF
* Idarubicine and [[G-CSF]]
:*cyclophosphamide, cytarabine, topotecan and G-CSF
* [[Cyclophosphamide]], [[cytarabine]], [[topotecan]] and [[G-CSF]]
*All of these therapeutic agents act through DNA damage and interferes with DNA synthesis.
All of these therapeutic agents act through DNA damage and interferes with DNA synthesis.
===Bone marrow transplantation===
===Bone marrow transplantation===
*Hematopoietic stemm cell transplantation can be considered as a treatment option following induction chemotherapy in patients with AML<ref name=":1">{{Cite journal|last=Richard L. Bakst, Martin S. Tallman, Dan Douer and Joachim Yahalom|first=|date=2011|title=How I treat extramedullary acute myeloid leukemia|url=http://www.bloodjournal.org/content/118/14/3785?sso-checked=true|journal=Blood|volume=118|pages=3785-3793|via=}}</ref>.
* [[Hematopoietic stem cell transplantation]] can be considered as a treatment option following induction [[chemotherapy]] in patients with [[AML]].<ref name=":1">{{Cite journal|last=Richard L. Bakst, Martin S. Tallman, Dan Douer and Joachim Yahalom|first=|date=2011|title=How I treat extramedullary acute myeloid leukemia|url=http://www.bloodjournal.org/content/118/14/3785?sso-checked=true|journal=Blood|volume=118|pages=3785-3793|via=}}</ref>
===Radiation===
===Radiation===
*Radiation can be considered as an adjunctive therapy<ref name=":1" />.
* Radiation can be considered as an adjunctive therapy<ref name=":1" />.
*Combination of chemotherapy and radiotherapy can be considered in patients with CNS involvement or when rapid regression of symptoms is required.
* Combination of chemotherapy and radiotherapy can be considered in patients with CNS involvement or when rapid regression of symptoms is required.
=== Surgery ===
=== Surgery ===
*Surgery alone is not a good treatment strategy for GS.
* Surgery alone is not a good treatment strategy for GS.
*Surgery can be considered prior to chemotherapy in patients where debulking can better the prognosis and help with symptom relief<ref>{{Cite journal|last=Antic D et al.|first=|date=2013|title=Is there a "gold" standard treatment for patients with isolated myeloid sarcoma?|url=https://www.sciencedirect.com/science/article/abs/pii/S0753332212001060?via%3Dihub|journal=Biomed Pharmacother|volume=67|pages=72-77|via=}}</ref>.
* Surgery can be considered prior to chemotherapy in patients where debulking can better the prognosis and help with symptom relief.<ref>{{Cite journal|last=Antic D et al.|first=|date=2013|title=Is there a "gold" standard treatment for patients with isolated myeloid sarcoma?|url=https://www.sciencedirect.com/science/article/abs/pii/S0753332212001060?via%3Dihub|journal=Biomed Pharmacother|volume=67|pages=72-77|via=}}</ref>
*Surgery can also have a diagnostic role in cases where excision of the mass provides specimen for histopatjologic diagnosis.
* Surgery can also have a diagnostic role in cases where excision of the mass provides specimen for histopatjologic diagnosis.
=== Prevention ===
=== Prevention ===
*There are no primary preventive measures available for GS.
* There are no primary preventive measures available for GS.
Granulocytic sarcoma (GS, also known as chloroma) was first discovered by Allen Burns, a British physician, in 1811. The term chloroma was first used by King to address the greenish appearance of the tumor due to myeloperoxidase. The association of the GS with acute myeloid leukemia (AML) was first recognized by Dock in 1902. GS can be classified into two categories based on its co-occurence with other malignancies. Infiltration of the tumor with myeloblasts is the main characteristic of the tumor on H&E stain. GS rises from primitive precursors of granulocytes. The disease is an extramedullary manifestation of myeloid diseases, however, it can occur as a primary disease. Aggregation of myeloblasts, promyelocytes and myelocytes outside of the bone marrow presents itself as these solid tumors. Tumors can occur at any site and can appear as green, gray, white or brown masses. GS must be differentiated from other diseases that can present as extramedullary solid tumors. All patients with GS must be evaluated for concurrent or future malignancies as GS can occur in the course of or prior to other malignancies. The prevalence of GS is approximately 2 per 1,000,000 individuals worldwide. The most important risk factor for development of GS is genetic mutations and susceptibility. Symptoms of GS may include the following: Symptoms due to mass effect such as deafness, ptosis, altered vision, intestinal obstruction, headache, neck pain, abdominal pain, and constitutional symptoms. Chemotherapy is the main stain of treatment in patients with GS. Even patients with isolated GS must receive systemic treatment to better the prognosis.
Historical Perspective
Plain radiograph of femur in a patient with GS. Courtesy of image: pathology outlines (http://www.pathologyoutlines.com/topic/bonemyeloidsarcoma.html)Granulocytic sarcoma (GS, also known as chloroma) was first discovered by Allen Burns, a British physician, in 1811 [1].
The term chloroma was first used by King to address the greenish appearance of the tumor due to myeloperoxidase.
Majority of GS tumors are found in the soft tissues such as the peritoneum, lymph nodes, CNS and skin. They are also found in bone and periosteum.
Early clinical features include weight loss, fatigue. Other manifestations of the tumor depend on its size and location.
Prognosis of GS depends on its association with other malignacies. In cases of isolated GS the prognosis is good. However, GS associated with myeloproliferative disorders has poor prognosis.
Prognosis of isolated GS with chromosome 8 abnormalities is worse than other cases of isolated GS.
Diagnosis
Diagnostic Criteria
There are no predefined criteria for diagnosis of granulocytic sarcoma.
Granulocytic sarcoma must be suspected in patients with AML or myelodysplastic syndromes. Diagnosis must be confirmed with histopathologic study of the specimen.
Symptoms
Symptoms of GS may include the following:
Symptoms due to mass effect such as deafness, ptosis, altered vision, intestinal obstruction, etc.
Headache, neck pain, abdominal pain,etc. based on the site of the tumor
Constitutional symptoms such as fever, fatigue, etc.
Physical Examination
Patients with GS can present with varying presentations.
Hyperdense/isodence to brain/muscle in CT scan without enhancement
Isointense/hyperintense on T2-weighted MRI
Isointemse/hypointense on T1-weighted MRI
Histopathologic micrograph of GS. Infiltration with myeloblasts which can be seen in the course of AML. Courtesy of image: Wikipedia (By Nephron - Own work, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=15893726)
Abdominal plain radiogram can reveal obstruction, intussusception, etc.
Echocardiogram may reveal mobile masses in any heart chamber in cases of heart involvement.
Chest radiographs can show lymph node enlargement and consolidation.
Other Diagnostic Studies
Peripheral blood smear can reveal circulating blasts.
Histopathologic analysis of GS lesions reveals high infiltration with myeloblasts.
Treatment
Medical Therapy
Chemotherapy is the main stain of treatment in patients with GS. Even patients with isolated GS must receive systemic treatment to better the prognosis.
Patients receiving cytarabine have better prognosis[4].
Patients with chemotherpeutic regimens accepted for AML had longer period of progression to AML.
Radiation can be considered as an adjunctive therapy[7].
Combination of chemotherapy and radiotherapy can be considered in patients with CNS involvement or when rapid regression of symptoms is required.
Surgery
Surgery alone is not a good treatment strategy for GS.
Surgery can be considered prior to chemotherapy in patients where debulking can better the prognosis and help with symptom relief.[8]
Surgery can also have a diagnostic role in cases where excision of the mass provides specimen for histopatjologic diagnosis.
Prevention
There are no primary preventive measures available for GS.
References
↑Burns, Allen. "Observations of surgical anatomy, in Head andNeck". London, England, Royce, 1811: 364–366.
↑Dock G, Warthin AS. "A new case of chloroma withleukemia". Trans Assoc Am Phys, 1904. 19:64: 115.
↑Rappaport H (1967). Tumors of the hematopoietic system, inAtlas of Tumor Pathology, Section III. Washington: Fascicle 8. ArmedForces Institute of Pathology. pp. 241–247.
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