Endocrine Abstracts

JOINT2585

Introduction: Pheochromocytomas are rare neuroendocrine tumors, typically located in the adrenal medulla, but they can also affect sympathetic paraganglia, where they are referred to as paragangliomas. Rare but serious during pregnancy, pheochromocytoma poses a high risk of maternal and fetal mortality, highlighting the importance of early diagnosis and treatment to improve prognosis.

Observation: A 31-year-old patient was admitted for severe preeclampsia at 31 weeks of gestation, with a history of untreated hypertension prior to pregnancy. Given the suspicion of secondary hypertension and the presence of the Menard triad, an abdominal MRI was requested, which revealed a left adrenal mass measuring 5.8 × 4.7 × 7.6 cm, with a cystic fleshy component in the center (necrosis?), associated with centimeter-sized juxta-lesional lymphadenopathy, suggestive of pheochromocytoma; however, an adrenal corticoid tumor could not be ruled out, necessitating confrontation with clinical, biological, and histological data.

Methoxy derivatives: Normetanephrine 18.45 µmol/24h (0.40-2.10 µmol/24h) Metanephrine 0.17 µmol/24h (0.20-1.00 µmol/24h). After a multidisciplinary team meeting: Decision for fetal extraction via cesarean section followed by left adrenalectomy 10 days later. The newborn died on day 3 due to respiratory distress.

Discussion: The symptoms of pheochromocytoma during pregnancy are often nonspecific and can mimic common conditions such as preeclampsia or gestational hypertension. The biological diagnosis relies on measuring plasma or urinary metanephrines, which remain reliable during pregnancy (Gruber et al., 2022). A significant elevation of these markers confirms the diagnosis. MRI imaging without gadolinium is the preferred examination to locate the tumor during pregnancy, as it is non-radiating and very sensitive. Initial management involves medical treatment aimed at stabilizing blood pressure and preventing hypertensive crises. Alpha-blockers, such as phenoxybenzamine or doxazosin, are used to block the effects of catecholamines. Surgical excision of the tumor is the curative treatment for pheochromocytoma. The timing of the intervention depends on the stage of pregnancy: Before 24 weeks of amenorrhea, laparoscopic surgery is recommended after medical stabilization. After 24 weeks, surgery is generally deferred until after delivery, except in life-threatening situations.

Conclusion: Pheochromocytoma during pregnancy is a rare but potentially fatal condition. Its diagnosis requires heightened vigilance in the face of atypical hypertension or paroxysmal symptoms. Management relies on rigorous medical stabilization, followed by surgical excision at the appropriate time.