Spontaneous rupture of a functioning adrenocortical carcinoma

Abstract

Adrenocortical carcinoma (ACC) is a rare malignancy with poor prognosis, and it can be classified as either a functional or nonfunctional tumor. Affected patients usually present with abdominal pain or with symptoms related to the mass effect or hormonal activity of the tumor. Several cases of spontaneously ruptured nonfunctional adrenocortical carcinoma have been reported, but no case of a spontaneous rupture of functioning adrenocortical carcinoma has been described. We report a functioning adrenocortical carcinoma that spontaneously ruptured during a work-up.

Fig. 1

(A) Adrenal T1 MRI showed an approximately 12×10.3×9.7 cm, relatively well-marginated left adrenal mass with central high signal intensity, suggesting an internal hemorrhage. (B) Gadolinium injection revealed heterogeneous enhancement on delayed phase.

Fig. 2

Abdominal CT showed a 14×12×10 cm sized, heterogeneously enhancing tumor mass (A) and a hemorrhagic fluid collection around the left kidney (B).

Fig. 3

(A) The tumor cells with eosinophilic cytoplasm were associated with necrotic and hemorrhagic tissue (H&E stain,×100). (B) We also noted neoplastic, pleomorphic cells with high nuclear grade (H&E stain,×400). The tumor cells had immunoreactivity for synaptophysin (C), CD56 (D), alpha-inhibin (E), and melan-A (F).