Nonfunctioning Adrenal Cortical Carcinoma Presenting as Flank Pain in a 35-Year-Old Female: A Case Report. (21st September 2018)

• Record Type: Journal Article
• Authors: Newsom, Jonathan; Derakhshandeh, Roshanak; Lazarchick, John
• Is Part Of: American journal of clinical pathology. Volume 150(2018)Supplement 1
• Page Start: S51
• Page End: S51
• Publication Date: 2018-09-21
• Subjects: Diagnosis, Laboratory -- Periodicals; Pathology -- Periodicals; 616.07
• DOI: 10.1093/ajcp/aqy090.126
• Languages: English
• ISSNs: 0002-9173

Abstract

Abstract: Adrenal cortical carcinoma (ACC) is a rare malignant neoplasm (0.5–2 cases/million inhabitants per year) originating in the adrenal cortex with a poor prognosis, slight female predilection, and a bimodal age distribution with peaks in the first and fifth decades of life. However, nonfunctioning ACCs usually affect older patients in the fifth to seventh decades of life and occur in males twice as often as females. Although most cases are sporadic, ACCs are also occasionally associated with a variety of hereditary cancer syndromes. Clinical symptoms are most frequently related to an excess of steroid hormones. We report a 35-year-old previously healthy female who presented with a 1-month history of intermittent left flank pain. A CT scan revealed a 16-cm complex, solid mass with scattered calcifications and circumscription by a thin peripheral capsule. The mass was superior to the upper pole of the left kidney and appeared to be separated from the adjacent organs. On gross examination, the mass measured 16.7 cm in greatest dimension and weighed 993 g. Serial sectioning of the mass revealed solid and cystic cut surfaces with a heterogeneous appearance and areas of extensive necrosis. Microscopically, the mass consisted of sheets of large, pleomorphic cells with a hyperchromatic chromatin pattern, frequent bizarre nuclei, eosinophilic cytoplasm, and high mitotic activity. Extra-adrenal extension into surrounding soft tissue was present. Three regional lymph nodes were negative for metastatic disease. The neoplastic cells were positive for Melan A (weak), steroidogenic factor 1, and synaptophysin. Ki-67 expression was estimated at 75%. Immunohistochemical stains for chromogranin, cytokeratin CAM 5.2, desmin, pan-keratin, PAX-8, S-100, SMA, and SOX10 were negative. Our case is unusual because the patient was a younger female who presented with symptoms related to mass effect rather than the hormonal-related changes more commonly seen as a result of a functional ACC.