Intracranial desmoplastic small round cell tumor presenting as a suprasellar mass
| Autor: | Brian Haylock, Sravan Thondam, Daniel du Plessis, Mohsen Javadpour, Kumar Das, Ian A. MacFarlane, Christina Daousi, Daniel J. Cuthbertson, James Leggate |
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| Rok vydání: | 2014 |
| Předmět: |
Adult
Male Pathology medicine.medical_specialty Desmoplastic small-round-cell tumor medicine.medical_treatment Vision Disorders Sarcoma Ewing Desmoplastic Small Round Cell Tumor Malignancy Wilms Tumor Hypopituitarism Neurosurgical Procedures Fatal Outcome medicine Humans Pituitary Neoplasms business.industry Brain Neoplasms Gene rearrangement Debulking medicine.disease Radiation therapy Mediastinal lymph node Optic Chiasm Visual Field Tests Differential diagnosis business Progressive disease |
| Zdroj: | Journal of neurosurgery. 122(4) |
| ISSN: | 1933-0693 |
| Popis: | Desmoplastic small round cell tumors (DSRCTs) are rare, aggressive neoplasms that typically arise from abdominal and pelvic peritoneum in young adults. Other primary sites are uncommon, and an intracranial origin is exceptionally rare. Here the authors report the first case of a DSRCT presenting as a primary suprasellar tumor causing panhypopituitarism and severe bitemporal hemianopia in a young man. Macroscopic debulking of the tumor was undertaken, and histology revealed features of DSRCT. Reverse transcription polymerase chain reaction confirmed the presence of Ewing's sarcoma–Wilms tumor 1 (EWS-WT1) gene rearrangement specific to DSRCT. Postoperative whole-body imaging showed no primary malignancy elsewhere. The tumor recurred 4 months after surgery, and this was followed by cervical and mediastinal lymph node metastases. The patient died 20 months after initial presentation of rapidly progressive disease. DSRCTs should be included in the differential diagnosis of an unusual suprasellar mass in young adults. Early diagnosis is essential, and once the tumor is identified histologically, gross-total resection and radical postoperative treatment involving radiotherapy, chemotherapy, and close surveillance are required because of the lesion's potential for rapidly progressive malignancy. |
| Databáze: | OpenAIRE |
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