Endocrine Abstracts

Introduction: Pheochromocytoma is rarely associated with neurofibromatosis type 1 (NF1). We report 4 observations of hospitalized patients in theIbn Rochd University Hospital endocrinology department in Casablanca.

Observation 1: A 52-year-old male patient was admitted for NF1-associated malignant pheochromocytomawith paroxysmal hypertension, Menard triad, elevated methoxylated urinary derivatives (DMU), and 11 * 8 cm right adrenal mass with hepatic metastasis. A surgical excision of the mass was performed with death of the patient following a hemorrhagic shock.

Observation 2: A 62-year-old patient was admitted for bilateral pheochromocytoma associated with NF1. He had hypertension, Menard triad, and two right (10 * 9 * 8 cm) and left (2 cm) adrenal masses, with no extra-adrenal localization. Bilateral adrenalectomy was performed. Pathological study showed a right pheochromocytoma complete excision with left adrenal hyperplasia. We rated a persistence of a moderate hypertension.

Observation 3: A 26-year-old patient, was operated 4 times for a face shwanoma, was admitted for pheochromocytoma associated with NF1. He had hypertension without menard triad, raised DMU, and left adrenal mass of 3 cm. He died 1 month later by cerebral involvement.

Observation 4: A 22-year-old patient was admitted for pheochromocytoma associated with NF1 discovered by incomplete Menard triad, elevated DMU, right adrenal mass (68×80 mm) including IVC in its inferior vena cava retrohepatic portion. Patient did not receive a surgical excision.

Discussion: Our observations illustrate the need to look for pheochromocytoma in any patient with NF1 because of its serious consequences.