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|LETTER TO EDITOR
|Year : 2018 | Volume
| Issue : 4 | Page : 1183-1185
Primary granulocytic sarcoma presenting as a lumbar plexopathy in a nonleukemic patient
Mishaal Talish1, Boby Varkey Maramattom1, Nanda Kacchare2
1 Department of Neurology, Aster Medcity, Kochi, Kerala, India
2 Department of Pathology, Aster Medcity, Kochi, Kerala, India
|Date of Web Publication||18-Jul-2018|
Dr. Boby Varkey Maramattom
Department of Neurology, Aster Medcity, Kochi, Kerala
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Talish M, Maramattom BV, Kacchare N. Primary granulocytic sarcoma presenting as a lumbar plexopathy in a nonleukemic patient. Neurol India 2018;66:1183-5
A 57-year old male patient presented with progressive pain and weakness in the right lower limb for the last 2 months. He was a known case of psoriatic arthritis on treatment with methotrexate for the past 2 years. Examination revealed weakness of right hip flexion, knee extension, ankle dorsiflexion, and inversion (Grade 2/5). Sensory exam was normal. Right knee jerk was absent. Flexion, adduction, and internal rotation of the right hip (FAIR) test was positive. Computed tomography (CT) of the abdomen and magnetic resonance imaging (MRI) showed diffused enlargement of the entire right iliopsoas muscle [Figure 1]. Ultrasonography (USG)-guided biopsy showed a malignant round cell neoplasm infiltrating the skeletal muscle [Figure 2]. Positron emission tomography (PET)-CT showed focal hypermetabolism in the region of the lesion without any other abnormalities. The bone marrow biopsy was normal. A diagnosis of a primary extramedullary myeloid tumor (primary granulocytic sarcoma [GS]) was made. The patient underwent 10 cycles of chemotherapy, with shrinkage of the tumor and almost complete relief of his symptoms. Granulocytic sarcoma (GS), also known as myeloid sarcoma or chloroma [due to its green color attributed to the enzyme myeloperoxidase (MPO)], is a tumoral lesion of immature granulocytic cells. It represents a rare extramedullary presentation of an underlying hematological malignancy.,, It is most commonly associated with acute myeloid leukemia (AML) with an incidence rate of 3–5%.,,, GS can occur prior to, concurrently with, or as a relapse of AML after stem cell therapy., It may rarely be detected before AML (primary, isolated, or nonleukemic GS). However, it invariably progresses to a myeloproliferative disorder (MPD). Paradoxically, isolated GS has a worse outcome than GS associated with MPD. The interval between its diagnosis and progression to AML is 1–49 months. The median age of presentation of GS is 36 years. Common locations affected by GS include the bone, soft tissue as well as skin. Rarely, the disease also involves the brain and genitourinary (GU) system., The differential diagnoses include non Hodgkin's lymphoma, rhabdomyosarcoma, Ewing's sarcoma, neuroblastoma, and osteosarcoma.
|Figure 1: (a) Contrast enhanced CT (CECT) scan of the abdomen shows diffuse enlargement of the right iliopsoas muscle, having a heterogeneous appearance without necrosis or enhancement. (b and c). T1 MRI coronal sections showing a large homogeneous, mass lesion predominantly involving the right iliopsoas muscle with diffusion restriction and patchy enhancement. (d and e) T2-weighted MRI; lesion exhibits mild hyperintensity. The major bulk of the right psoas muscle is displaced anteriorly by the lesion with the presence of focal infiltration. Medially, the lesion extends till the right paravertebral region with possible compression/infiltration of the right lumbar plexus|
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|Figure 2: Core biopsy of the mass with neoplasm infiltrating the skeletal muscle showing (a) a diffuse sheet of malignant round cells (inset). Immunohistochemistry shows a strong positive staining for leucocytic common antigen (b); CD117 (c), focal myeloperoxidase (d); CD163 (e), CD43 (f), scattered cells for CD68 (g). Tumor cells show a negative staining for CD3 (h), CD20 (i), and CD34 (j) and a high Ki67 proliferation index (k)|
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An underlying immunosuppressive state plays a role in tumorigenesis. Some studies indicate that the onset of GS could occur due to long-term treatment with drugs such as methotrexate. Our patient was on methotrexate for 2 years, suggesting a possible causative factor.
Involvement of the peripheral nervous system is very rare in GS with a few reports of brachial plexopathy or sciatica., The most common symptoms are attributed to compression of the surrounding structures, often associated with pain and/or abnormal bleeding. The computed tomography (CT) and magnetic resonance imaging (MRI) can localize and differentiate the tumor from an abscess or hemorrhage, with MRI being the gold standard. GS appears isointense on T1-weighted MRI sequences and has intermediate signal density on T2-weighted images. The positron emission tomography (PET)-CT is also useful in the localization as well as in the detection of concurrent lesions, and monitoring of the response to treatment.,
Histopathological examination shows medium-to-large sized pleomorphic cells with irregular nuclear outlines, prominent nucleoli, and finely dispersed chromatin with an eosinophilic cytoplasm. There is partial-to-complete obliteration of the surrounding tissue architecture. Immunohistochemistry commonly shows cluster of differentiation (CD)-68 KP1 positivity followed by myeloperoxidase, CD117, CD99, CD68, lysozyme, CD34, terminal deoxynucleotidyl transferase (Tdt), CD56, and CD61 positivity.,,
Treatment for GS employs regimens used in AML remission- induction therapy. At present, systemic chemotherapy is advocated for all patients to decrease the rate of progression to AML and increase the survival rates.,, Surgical removal or decompression is indicated in massive tumors or in spinal cord compression.
Neoplastic lumbar plexopathy occurs in approximately 0.71% of cancer patients. Lumbosacral plexopathy may manifest due to the presence of the primary tumor as well as metastasis, or secondary to radiation therapy. MRI is the imaging modality of choice in GS. In conclusion, we present the first case of GS presenting as a lumbar plexopathy. GS should be considered in the differential diagnosis of mass lesions that result in a compressive plexopathy.
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There are no conflicts of interest.
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