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.

Radiological features of primitive neuroectodermal tumors in intra-abdominal and retroperitoneal regions: A series of 18 cases

“…Therefore, the radiologist’s role in differentiating lesions, such as PNETs, that do require surgical therapy from those lesions that do not is becoming increasingly important. Moreover, among the few cases that have been reported, neo-adjuvant chemotherapy may play an important role in the treatment of PNETs, especially in advanced patients [13–15]. Therefore, the preoperative non-invasive imaging diagnosis of PNET is even more important.…”

Primary Ewing sarcoma/primitive neuroectodermal tumor in the adrenal gland

“…Other potential differential diagnoses include malignant solitary fibrous tumor, small cell carcinoma, neuroblastoma, lymphoblastic lymphoma, desmoplastic small round cell tumor, and adrenocortical carcinoma, all of which can be ruled out without much difficulty in the present case. To the best of our knowledge, only approximately 20 cases of Ewing sarcoma/PNET of the adrenal gland have been reported in the literature (including one French (12) and one Japanese (21) articles) (3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22); however, this is the first case wherein a comprehensive pathological analysis was performed. The first case was reported by Marina et al in 1989 (3).…”

“…To the best of our knowledge, only approximately 20 cases of Ewing sarcoma/PNET of the adrenal gland have been reported in the literature (including one French (12) and one Japanese (21) articles) ; however, this is the first case wherein a comprehensive pathological analysis was performed. The first case was reported by Marina et al in 1989 .…”

Unusual Presentations of Adrenal Masses

“…These cells have little endoplasm with dark stained nucleus high in nucleoplasm [67]. Among all the markers, Mic-2 (CD-99) is the most sensitive and specific marker (nearly 100%), which is a cell surface glycoprotein coded by genes on X and Y-chromosomes [70]. Other common markers of neural differentiation include neuron-specific enolase (NSE), S-100 protein, neurofilaments, synaptophysin (Syn), chromogranin A (CgA) and vimentin.…”

“…In general, 5-year survival of patients with PNETs is 58-61% with a median survival of 120 months. Local or distant metastasis at the time of diagnosis is associated with poor outcome [70].…”