Presentation
HIV positive, lower limb weakness and loss of sensation for 3 weeks.
Patient Data
Distal conus lesion at L3-L4 demonstrating iso to mildly T1W hyperintense signal with peripheral T2W hypointense signal surrounding a central hyperintense signal. The lesion has peripheral rim enhancement and centrally non-enhancing components.
Normal x-ray with no degenerative disease or destructive bone lesion.
Histopathology report
Macroscopy: Specimen labeled spinal tumor consists of a delineated firm fibrous lesion-40 x 28 x 19 mm.
Microscopy: Sections of the lesion show a well-delineated neoplasm thinly pseudo-encapsulated towards the periphery, the latter irregular and the capsule lost in areas with the contents effaced by a lobulated spindled neoplasm with focal necrosis, also confluent in areas. The cytomorphology consists of elongated, spindled vesicular cells with single enlarged nucleoli, irregular nuclear features and ill-defined eosinophilic cytoplasm. A mild sprinkling of lymphocytes is also present. Mitoses are counted at an average of 1 to 10 per high power field. There is no distinct pattern though cellularity varies from slightly more condensed, cellular to more hyalinised and less cellularity with focal tumor nuclei pleomorphism. Prominent apoptosis is also found within areas. Other areas show more rounded nuclei, vesicular and with prominent nucleoli.
Immunohistochemistry:
- Alpha smooth muscle actin/ASMA-diffusely positive
- EMA: positive in the tumor
- Collagen IV: Focal strong positivity
- s100: negative
- Ki67 proliferation index: Average 10%
- EBER-ISH: strongly positive
- Calponin: Diffusely positive
- BCL2, CK7, CK19, Sox 10, CD99 and progesterone are all negative
Pathological Diagnosis: Extra-medullary intra-dural tumor EBV associated smooth muscle tumor with spotty necrosis and increased mitotic activity. Leiomyosarcomatous changes not excluded. Correlate with the immune status of the patient.
Case Discussion
The patient underwent surgical resection with histology demonstrating cytomorphology consisting of elongated spindled vesicular cells with enlarged vesicular cells. The tumor was diffusely positive for alpha smooth muscle actin/ASMA, diffusely positive for calponin and collagen IV and strongly positive for EBER-ISH. The tumor was negative for s100.
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