Abstract
Introduction: Adrenal cortical carcinoma is a rare aggressive tumor with a poor prognosis. Associated systemic amyloidosis is a rare phenomenon with only single case reported in English literature. case report: We report a case of a 50-year-old female harboring a huge adrenal cortical tumor along with systemic amyloidosis who underwent primary curative surgical resection of the tumor. Our patient had a Weiss score of 6 which is indicative of bad prognosis. conclusion: the presence of proteinuria in patients with adrenal cortical carcinoma may be indicative of amyloidosis. Further, the high risk of metastasis in adrenal cortical cancer mandates a close clinical follow-up.
Highlights
Adrenal cortical carcinoma is a rare aggressive tumor with a poor prognosis
Associated systemic amyloidosis is a rare phenomenon with only single case reported in English literature
Case Report: We report a case of a 50-year-old female harboring a huge adrenal cortical tumor along with systemic amyloidosis who underwent primary curative surgical resection of the tumor
Summary
Adrenal cortical carcinoma (ACC) is a rare aggressive endocrine malignancy accounting for 0.2% of all cancers [1]. Representative sections examined from the adrenal mass showed a well-encapsulated tumor with organoid and lobular pattern (Figure 2A). The tumor cells were large with plasmacytoid appearance at places (Figure 2B) showing considerable nuclear pleomorphism, and atypia with moderate to abundant eosinophilic cytoplasm (Figure 2C). Sections from the kidney were free of tumor and showed deposition of homogeneous acellular eosinophilic material in the glomerular tuft, mesangium and the peritubular capillary walls (Figure 3B). Sections from spleen were free of tumor and showed deposition of eosinophilic material in the arteriolar walls with replacement of follicles at places (Figure 3C). The acellular eosinophilic material deposits seen in tumor per se, kidney and spleen showed congophilia and apple green birefringence on polarization (Figure 3D), confirming it to be amyloid. The final histopathological diagnosis was of an adrenal cortical carcinoma with amyloid deposition and amyloidosis kidney and spleen. After surgery the patient was advised for follow-up in the oncology clinic but the patient did not come for follow-up
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