Granulocytic sarcoma: Difference between revisions
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| Line 37: | Line 37: | ||
:*Ewing sarcoma | :*Ewing sarcoma | ||
:*Extramedullary sites of hematopoiesis | :*Extramedullary sites of hematopoiesis | ||
:*Burkitt lymphoma | |||
:All patients with granulocytic sarcoma must be evaluated for concurrent or future malignancies as granulocytic sarcoma can occur in the course of or prior to other malignancies. | :All patients with granulocytic sarcoma must be evaluated for concurrent or future malignancies as granulocytic sarcoma can occur in the course of or prior to other malignancies. | ||
==Epidemiology and Demographics== | ==Epidemiology and Demographics== | ||
* The prevalence ofgranulocytic sarcoma is approximately 2 per 1,000,000 individuals worldwide. | * The prevalence ofgranulocytic sarcoma is approximately 2 per 1,000,000 individuals worldwide. | ||
Revision as of 06:08, 1 May 2019
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Historical Perspective
- 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.
- The association of the GS with acute myeloid leukemia was first recognized bt Dock in 1902 [2].
- The term granulocytic sarcoma was suggested by Rappaport in 1967 to grant generalisability to it [3].
Classification
- GS can be classified into two categories based on its co-occurence with other malignancies:
- GS associated with other myeloid diseases:
- acute leukemias (especially acute myeloid leukemia)
- myelodysplastic syndromes
- other myeloproliferative diseases
- Isolated GS
Pathophysiology
- 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.
Clinical Features
Differentiating granulocytic sarcoma from other Diseases
- Granulocytic sarcoma must be differentiated from other diseases that can present as extramedullary solid tumors, such as:
- Large cell lymphoma
- Non-Hodgkin lymphoma
- Thymoma
- Myeloma
- Esosinophilic sarcoma
- Ewing sarcoma
- Extramedullary sites of hematopoiesis
- Burkitt lymphoma
- All patients with granulocytic sarcoma must be evaluated for concurrent or future malignancies as granulocytic sarcoma can occur in the course of or prior to other malignancies.
Epidemiology and Demographics
- The prevalence ofgranulocytic sarcoma is approximately 2 per 1,000,000 individuals worldwide.
- Most of the cases of granulocytic sarcoma are case reports and the disease is extremely rare.
Age
- Patients of all age groups may develop granulocytic sarcoma.
- Granulocytic sarcoma associated with acute myleloid leukemia occurs more commonly in children.
Gender
- Granulocytic sarcoma affects both men and women.
- Due to the rarity of the disease it is not clear whether there is a gender predilection for it.
Race
- There is no racial predilection for granulocytic sarcoma.
Risk Factors
- Risk factors for granulocytic sarcoma are usually chromosomal aberrations and include:
- Trisomy 8
- Monosomy 7
- MLL gene rearrangement
- NPM1 mutations
- FLT3 mutations
Natural History, Complications and Prognosis
- How granulocytic sarcoma evolves over time depends on the co-occurence of the disease with other malignancies.
- Granulocytic sarcoma may present before evidences of other malignancies manifest or after these malignancies are evident.
- Symptoms of the isolated granulocytic sarcoma depends on the location and the site of the tumor.
- Majority of cases are associated with acute myeloid leukemia or other myeloproliferative/myelodysplastic syndromes.
- Majority of granulocytic sarcoma 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.
- Prognosis of granulocytic sarcoma depends on its association with other malignacies. In cases of isolated granulocytic sarcoma the prognosis is good. However, granulocytic sarcoma associated with myeloproliferative disorders has poor prognosis.
- Prognosis of isolated granulocytic sarcoma with chromosome 8 abnormalities is worse than other cases of isolated granulocytic sarcoma.
Diagnosis
Diagnostic Criteria
- 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.
Symptoms
- Symptoms of GS may include the following:
- Symptoms due to pressure effect such as deafness, ptosis
- Lymph node enlargement (in cases associated with AML and other myeloproliferative syndromes)
- anemia (in cases associated with AML and other myeloproliferative syndromes)
- [symptom 5]
- [symptom 6]
Physical Examination
- Patients with [disease name] usually appear [general appearance].
- Physical examination may be remarkable for:
- [finding 1]
- [finding 2]
- [finding 3]
- [finding 4]
- [finding 5]
- [finding 6]
Laboratory Findings
- There are no specific laboratory findings associated with [disease name].
- A [positive/negative] [test name] is diagnostic of [disease name].
- An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
- Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].
Imaging Findings
- There are no [imaging study] findings associated with [disease name].
- [Imaging study 1] is the imaging modality of choice for [disease name].
- On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
- [Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].
Other Diagnostic Studies
- [Disease name] may also be diagnosed using [diagnostic study name].
- Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].
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.
- Patients with chemotherpeutic regimens accepted for AML had longer period of progression to AML.
- Treatment includes different regimens:
- idarubicine and cytarabine
- fludarabine and cytarabine
- idarubicine and G-CSF
- cyclophosphamide, cytarabine, topotecan and G-CSF
- All of these therapeutic agents act through DNA damage and interferes with DNA synthesis.
Radiation
- Radiation can be considered as an adjunctive therapy.
- 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.
- 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.