Granulocytic sarcoma

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

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List of terms related to Granulocytic sarcoma

Historical Perspective

• 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 was first recognized bt Dock in 1902 .
• The term granulocytic sarcoma was suggested by Rappaport in 1967 to grant generalisability to it .

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.

Differentiating [disease name] 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

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

Natural History, Complications and Prognosis

• The majority of patients with [disease name] remain asymptomatic for [duration/years].
• Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
• If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
• Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
• Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with [disease name] is approximately [#%].

Diagnosis

Diagnostic Criteria

• The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:

• [criterion 1]
• [criterion 2]
• [criterion 3]
• [criterion 4]

Symptoms

• [Disease name] is usually asymptomatic.
• Symptoms of [disease name] may include the following:

• [symptom 1]
• [symptom 2]
• [symptom 3]
• [symptom 4]
• [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

• There is no treatment for [disease name]; the mainstay of therapy is supportive care.

• The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
• [Medical therapy 1] acts by [mechanism of action 1].
• Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

Surgery

• Surgery is the mainstay of therapy for [disease name].
• [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
• [Surgical procedure] can only be performed for patients with [disease stage] [disease name].

Prevention

• There are no primary preventive measures available for [disease name].

• Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

• Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].