PMC5677210_01
(A) Computed tomography scan at diagnosis showing a retroperitoneal mass (145 x 100 mm) (white dotted line). (B) 18F-fluorodeoxyglucose positron emission tomography before mitotane and chemotherapy showing an uptake localized to pulmonary hilar lymph nodes (green arrows) and retroperitoneal mass with a maximum standardized uptake value of 10.2 and an adrenal/liver maximum standardized uptake value ratio of 3.7 (red arrow). (C) Biopsy: alpha-inhibin subunit immunostaining of the retroperitoneal mass (original magnification, x20). (D) alpha-Inhibin subunit immunostaining of adrenal mass after surgery (original magnification, x20).
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The computed tomography showed a retroperitoneal mass (145 x 100 mm) associated with peritoneal and lung metastases and pulmonary hilar lymph nodes [Fig. 1(A)]. The magnetic resonance imaging was in favor of an adrenal mass and showed a venous tumor thrombus in vena cava and in renal vein. 18F-fluorodeoxyglucose positron emission tomography showed an uptake of the retroperitoneal mass (standardized uptake value maximum adrenal/liver ratio of 3.7), the peritoneal metastases, and the pulmonary hilar lymph nodes, suggesting a malignant tumor [Fig. 1(B)]. Labeling of ACC sections with an anti-inhibin alpha-subunit antibody revealed an intense and diffuse immunostaining consistent with the high inhibin B levels [Fig. 1(C)]. The intensity of the alpha-inhibin subunit immunostaining was significantly decreased and showed a different pattern than the one observed in the earlier biopsy [Fig. 1(D)].
GnRH stimulation test: FSH response (red line) and LH response (blue line) at initial evaluation (solid line) and after chemotherapy (dotted line).
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Surprisingly, the follicle stimulating hormone (FSH) levels were suppressed (0.14 IU/L; normal, 1 to 12) and remained blunted during gonadotropin-releasing hormone (GnRH) stimulation, whereas basal (3.7 IU/L; normal, 0.8 to 12) and stimulated luteinizing hormone (LH) levels were normal (Fig. 2). Inhibin B levels remained low (10 pg/mL) and basal and GnRH-stimulated FSH levels were normalized (Fig. 2).