Corticosurrenaloma metastasized: when the challenge becomes twofold

Fatma Mnif; Arous Mohamed Khairi; Salah Dhoha Ben; Rim Khlif; Kouloud Boujelben; Mouna Elleuch; Kacem Akid Faten Haj; Nadia Charfi; Mouna Mnif; Mohamed Abid; Majdoub Nabila Rekik

doi:10.1530/endoabs.99.EP1158

Introduction: The corticosurrenaloma is a rare malignant adrenal tumor with an unfavorable prognosis. The 5-year survival rate at stage IV, defined by the presence of distant secondary locations, does not exceed 28%. We report the case of a patient followed in our department for stage IV corticosurrenaloma and the challenges encountered during her management

Case report : Ms. H, a 42-year-old patient, is under our care for corticosurrenaloma. Her cancer was discovered 7 years ago due to a general deterioration in health with signs of hyperandrogenism. She underwent total left adrenalectomy with an initial pTNM stage of T2N0M0, complicated by adrenal insufficiency requiring hydrocortisone supplementation. A locoregional recurrence after 3 years necessitated surgical reintervention. Currently, a locoregional recurrence, 4 years after the first recurrence, has been diagnosed, involving the head of the pancreas, compressing the splenic vein, and presenting with giant mesenteric nodules of 20 cm. Imaging also revealed a pulmonary metastasis, while bone scintigraphy was normal. Surgical intervention was deemed impossible, leading us to opt for a systemic treatment with mitotane combined with Doxorubicin and Cisplatin chemotherapy. The initial mitotane dose was 1.5 g/day, with a progressive escalation of 1 g every 3 days to reach 6 g/day. The patient developed acute adrenal insufficiency despite parallel increases in hydrocortisone dose, necessitating a reduction in mitotane to 5 g. Thirty days after initiating mitotane and five days after chemotherapy, the patient presented with progressively worsening ataxia. Initially ruling out cerebral metastasis through imaging, we attributed this complication to mitotane’s neurological toxicity, leading to a dose reduction to 4 g/day with a favorable outcome.

Discussion and conclusion: The management of corticosurrenaloma is complex, primarily due to the rapidly deteriorating potential of this tumor and, secondarily, the limited therapeutic means available to date. Mitotane stands as the principal alternative in the face of stage IV corticosurrenaloma when surgery becomes unfeasible. Its proven efficacy in controlling disease progression and improving survival is well-established, yet it is accompanied by various undesirable effects, particularly of a neurological nature. The ataxia exhibited by our patient is indeed attributed to mitotane, with its onset five days after chemotherapy possibly suggesting an additive effect, implicating the neurotoxicity of doxorubicin and cisplatin, as reported in the literature.

Endocrine Abstracts

EP1158