Meningeal alveolar soft part sarcoma confirmed by characteristic ASPCR1-TFE3 fusion

Neuropathology : Official Journal of the Japanese Society of Neuropathology
Istvan BodiCyril Fisher

Abstract

Sarcoma metastatic to the brain is uncommon and rarely occurs as the initial manifestation of tumor. Alveolar soft part sarcoma (ASPS) is a rare but well-studied subtype of sarcoma. A 39-year-old man presented with seizures due to a left temporal meningeal-enhancing lesion with striking brain edema on MRI. The patient underwent neurosurgical resection for suspected meningioma. Histology showed large tumor cells clustering and forming small nests, in places with pseudoalveolar pattern. Diastase-resistant periodic acid-Schiff revealed very rare granular and rod-like cytoplasmic inclusions. Immunohistochemistry showed convincing positivity only with vimentin and smooth muscle actin. The histological features were strongly suggestive of ASPS. At the molecular level RT-PCR and sequencing analysis demonstrated ASPCR1-TFE3 fusion confirming the histological diagnosis of ASPS. There was no evidence of primary extracranial tumor by physical examination and on chest and abdominal CT scan 11 months after presentation. ASPS typically arise from the soft tissues of the extremities and develop multiple metastatic deposits usually with a long clinical course. This case may represent primary meningeal ASPS although metastatic deposit from an u...Continue Reading

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Apr 11, 2014·Journal of Orthopaedics·Aaron W JamesSarah M Dry
Jun 25, 2013·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·Sook Hyon AhnSeung-Ki Kim
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Apr 30, 2017·World Neurosurgery·Michael J RandazzoAli K Ozturk
Jun 19, 2021·International Journal of Surgical Pathology·Chiara CaporaliniAnna Maria Buccoliero

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