Endocrine Abstracts

Introduction

The adrenal cortex is a rare malignant tumor at the expense of the adrenal gland, very aggressive because of its invasion, its metastatic potential and its five (5) year survival at 40% with a high risk of locoregional relapse.

Case presentation

We report the case of a 17-year-old patient with a history of long-term corticosteroid therapy over 3 years and secondary amenorrhea for 2 years, who was admitted in April 2019 for the management of hyper androgenism. On clinical examination, the general condition was preserved with a notion of moderate weight gain, she presented a severe hirsutism rated at 32 on the Ferriman and Gallway score without signs of virilization, signs of hypercorticism with a cunshingoid facies.. The hormonal balance noted a CLU raised to 3 times the normal, i.e. 616 nmol/24 h (30–200), SDHEA > 27 µmol/l (1.3–8), testosterone at 20 nmol/l or 6 times the normal, methoxylated derivatives were normal. The cervico-thoraco-abdomino-pelvic CT scan performed revealed the presence in the right adrenal gland of a hypodense tissue mass with areas of necrosis measuring 6 × 6 × 7 cm, pushing back the IVC and the upper pole of the right kidney, with multiple cervical nodes. A right adrenalectomy with detachment of the adhesions and conservation of the right kidney was performed, the postoperative was complicated by acute adrenal insufficiency for which the patient was substituted. The pathological examination was in favor of a malignant adrenal cortex classified as stage 3 by ENSAT with an intermediate prognostic risk. At 6 months of follow-up there was a clear regression of clinical signs, at 12 months there was a reappearance of hirsutism, the control of the abdomino-pelvic CT noted a hypodense formation of 41 × 36 mm at the expense of the right adrenal gland. in connection with a local recurrence. Iterative adrenalectomy was performed, with confirmation on pathological examination of a recurrence of adrenal cortex associated with fibromyxoid changes.

Discussion and conclusion

The malignant adrenal cortex has a poor prognosis with a high risk of recurrence especially at stage 3 and 4 of ENSAT, wide surgical excision appears to reduce the risk of relapse according to some authors but treatment with mitotane or poly chemotherapy should be considered.