Endocrine Abstracts

Introduction

The adrenocortical carcinoma(ACC) is a rare malignant tumor arising from the adrenal cortex, it is usually associated to abnormal hormone secretion, but sometimes it is nonfunctioning, leading to a delayed diagnosis with a locally advanced and/or a metastatic disease.

Observation

We report the case of a 42 year-old woman with no medical history who consulted for chronic epigastralgia. On physical examination, a palpable mass in the right flank was found. Her vital signs were within the normal range and the body mass index was at 25.96 kg/m². An abdominal CT-scan revealed a giant retroperitoneal highly vascular tissue mass, encapsulated, well circumscribed and containing areas of necrosis, measuring 18.4 × 13 × 21.5 cm in diameter. Its origin couldn’t be clearly defined, it pushes the liver up and to the left, lowers the right kidney, comes into contact with the diaphragmatic dome and rolls the inferior vena cava, suggesting a high potential of malignancy. In front of this giant mass, the biological exploration didn’t show any excess of hormone secretion; plasma free normetanephine 0.35 nmol/l < 0.93 mmol/l, plasma free metanephrine < 0.10 nmol/l, cortisol 305 nmol/l, overnight dexamethasone suppression test: cortisol 32 nmol/l < 50 nmol/l, testosterone 0.27 ng/ml, SDHEA 215 µg/dl ( normal level 42–307 µg/dl ). A tumor biopsy was performed and the histo-pathological examination concluded to an adrenocortical carcinoma. The patient was presented to a multidisciplinary team meeting ( endocrinology, urology, oncology ), that decided that the tumor was inoperable due to its intimate contact with the vascular structures. As for mitotane treatement, it is not available in our country.

Conclusion

This case is one of the most rare cases of a giant nonfunctioning adrenocortical carcinoma that was reported in the literature. In fact, ACC is an aggressive tumor with poor prognosis.When doable and in the absence of disseminated metastasis, surgery remains the mainstay treatement and has better outcomes than chemotherapy alone. However adjuvant chemotherapy has shown a good effect on the duration of recurrence-free period and on the overall survival.