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Endocrine Abstracts (2018) 56 P80 | DOI: 10.1530/endoabs.56.P80

The Endocrinology Department of IBN Rochd University Hospital, Casablanca, Morocco.


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.

Volume 56

20th European Congress of Endocrinology

Barcelona, Spain
19 May 2018 - 22 May 2018

European Society of Endocrinology 

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