title: Primitive neuroectodermal tumor of adrenal: clinical presentation and outcomes.
abstract: Primitive neuroectodermal tumor (PNET) of adrenal is an extremely rare tumor of neural crest origin. A nonfunctional left adrenal mass (14.6 × 10.5 × 10.0 cm) on computed tomography (CT) was detected in a 40-year-old lady with abdominal pain, swelling, and left pleural effusion. She underwent left adrenalectomy and left nephrectomy with retroperitoneal resection. Histopathology revealed sheets and nest of oval tumor cells with hyperchromatic nuclei, prominent nucleoli, scanty cytoplasm, brisk mitotic activity, necrosis, lymphovascular invasion, capsular invasion, and extension to the surrounding muscles; staining positive for Mic-2 (CD-99 antigen), vimentin, synaptophysin, and Melan-A. Thoracocentesis, pleural fluid study, and pleural biopsy did not show metastasis. She responded well to vincristine, adriamycin, and cyclophosphamide followed by ifosfamide and etoposide (IE). This is the first report of adrenal peripheral PNET (pPNET) from India. This report intends to highlight that pPNET should be suspected in a patient presenting with huge nonfunctional adrenal mass which may be confused with adrenocortical carcinoma.
authors: Department of Endocrinology and Metabolism, Institute of Post Graduate Medical Education and Research and Seth Sukhlal Karnani Memorial Hospital, Kolkata, India.
authors: Deep
authors: D
authors: Dutta
authors: K S
authors: KS
authors: Shivaprasad
authors: Ram Narayan
authors: RN
authors: Das
authors: Sujoy
authors: S
authors: Ghosh
authors: Subhankar
authors: S
authors: Chowdhury
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000175:diagnosis* / Q000188:drug therapy / Q000601:surgery; D000311:Adrenal Glands / Q000473:pathology* / Q000601:surgery; D000315:Adrenalectomy; D000328:Adult; D000970:Antineoplastic Agents / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D003520:Cyclophosphamide / Q000627:therapeutic use; D005047:Etoposide / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007069:Ifosfamide / Q000627:therapeutic use; D007194:India; D009392:Nephrectomy; D009432:Neural Crest / Q000473:pathology; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery; D016708:Synaptophysin / Q000627:therapeutic use; D016896:Treatment Outcome; D014746:Vimentin / Q000627:therapeutic use
chemical_list: D000970:Antineoplastic Agents; D016708:Synaptophysin; D014746:Vimentin; D005047:Etoposide; D003520:Cyclophosphamide; D007069:Ifosfamide