Adrenocortical Carcinoma: A 20-Year Multi-Hospital Retrospective Analysis. (21st September 2018)

• Record Type: Journal Article
• Authors: Mahabir, Roshan; Khandakar, Binny; Raza, Roshan; Khan, Fahad; Yuan, Songyang
• Is Part Of: American journal of clinical pathology. Volume 150(2018)Supplement 1
• Page Start: S49
• Page End: S49
• Publication Date: 2018-09-21
• Subjects: Diagnosis, Laboratory -- Periodicals; Pathology -- Periodicals; 616.07
• DOI: 10.1093/ajcp/aqy090.120
• Languages: English
• ISSNs: 0002-9173

Abstract

Abstract: Objectives: Adrenocortical carcinoma (ACC) is a rare malignancy without sex preference and has a bimodal pattern of incidence. Metastasis typically involves the liver, lungs, and pleural surfaces and is associated with a poor prognosis. Methods: A 20-year retrospective, semiquantitative study (1997–2017) for adrenocortical carcinomas was conducted at multiple hospitals in urban New York. Clinicopathological parameters at the time of presentation were recorded. Results: Sixteen cases of ACC were identified; 10 of 16 (62.5%) were primary and 6 of 16 (37.5%) were metastatic. Six patients with metastasis presented with nodules to liver (2), pleura (1), bone (1), lung (1), and abdomen (1). There was a female predominance in the primary tumor group (F = 6, M = 4) and an equal sex distribution in the metastatic group. Females presented at an earlier age with primary tumors (F = 54.7 vs M = 66.7) with the opposite in metastatic diseases (F = 65.3 vs M = 59.6). Most patients with primary tumors presented with an abdominal mass (8/10, 80%), whereas one patient presented with a diagnosis of congenital adrenal hyperplasia and one had no history available. Overall tumor showed a predominance to the right adrenal (R = 6, L = 4). Primary tumors were staged pT3 in 5 of 10 (50%) cases, pT4 in 4 of 10 (40%) cases, and pT2 in 1 of 10 (10%) cases. All four pT4 staging cases showed extension of tumor into the periadrenal fat, lymph nodes, inferior vena cava (IVC), and diaphragm. Immunohistochemistry showed variable results. One hundred percent of primary tumors showed positivity for synaptophysin and melan-A and 66% for inhibin-A with a MIB-1 index ranging from 1% to 30%. One hundred percent of metastatic tumors showed positivity for inhibin-A and synaptophysin and 66.7% for melan-A with a MIB-1 index ranging from 1% to 40%. Conclusion: Our study shows a female predominance in primary ACC and an equal sex distribution in metastatic disease. Most common site for metastatic disease was the liver. Synatophysin, inhibin-A, and melan-A showed variable results but were paramount in establishing diagnosis.