Giant nonfunctioning adrenocortical carcinoma: a case report and review of the literature

Ahmad Almarzouq, Sami Asfar, Sundus Hussain, Adel Al-Hunayan, Saad Aldousari 1

Affiliations

doi: 10.1186/1756-0500-7-769

Abstract

Background: Adrenocortical carcinoma is a rare and aggressive malignancy. Patients usually present early with manifestation of abnormal hormone secretion. However, adrenocortical carcinoma can also be nonfunctioning, and such patients present late with a mass effect or distant metastases.

Case presentation: We herein report a case of a 30-year-old Sri-Lankan woman who presented with a 3-month history of left flank pain associated with nausea, vomiting, and weight loss. Imaging revealed a large left upper quadrant mass with a 1.8-cm left lung nodule. The differential diagnoses included a left adrenal mass, left upper pole renal mass, and retroperitoneal sarcoma. A functional adrenal work-up revealed no abnormal findings. Surgical excision of the mass was uneventful with no postoperative complications. Pathological analysis revealed a nonfunctioning adrenocortical carcinoma measuring 16 × 14 × 10 cm. To our knowledge, a mass of this size is among the largest nonfunctioning adrenocortical carcinomas reported in the published literature. The investigations and approach to treatment were consistent with those in the published literature.

Conclusion: Large nonfunctioning adrenocortical carcinomas pose a diagnostic and therapeutic challenge, and most are diagnosed at a late stage. Appropriate imaging and functional work-up of the mass are vital before treatment. Surgical excision is safe, even for large adrenocortical carcinomas; excision in patients with advanced disease has been shown to have the best outcomes.

Figures

Figure 1 A coronal section of the abdomen and pelvis illustrating the size of the mass. The mass is shown compressing and displacing the adjacent structures and occupying the whole left upper quadrant.

Figure 1 A coronal section of the abdomen and pelvis illustrating the size of the mass. The mass is shown compressing and displacing the adjacent structures and occupying the whole left upper quadrant.

Figure 2 A cross section of the abdomen showing the large mass crossing the midline.

Figure 2 A cross section of the abdomen showing the large mass crossing the midline.

Figure 3 Illustrates the extracted mass which includes the kidney and the adrenal gland. a) Gross appearance of the specimen showing a well-encapsulated mass with a distinct plane separating it from the kidney. b) En bloc specimen including the left kidney.

Figure 3 Illustrates the extracted mass which includes the kidney and the adrenal gland. a) Gross appearance of the specimen showing a well-encapsulated mass with a distinct plane separating it from the kidney. b) En bloc specimen including the left kidney.

Figure 4 Illustrates the histopathological and immuno-cytochemistry stained slides as described in a, b and c. a) Hematoxylin and eosin stain of the adrenal gland. Right: Tumor. Left: Residual rim of normal adrenal gland (10×). b) Immunohistochemical stain: inhibin (10×). c) Immunohistochemical stain: vimentin (10×).

Figure 4 Illustrates the histopathological and immuno-cytochemistry stained slides as described in a, b and c. a) Hematoxylin and eosin stain of the adrenal gland. Right: Tumor. Left: Residual rim of normal adrenal gland (10×). b) Immunohistochemical stain: inhibin (10×). c) Immunohistochemical stain: vimentin (10×).

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