Adrenal lesions in multiple endocrine neoplasia type 1: data from the French Group for the Study of Endocrine Tumors (GTE)

Blandine Gatta; Maud Monsaingeon; Pierre Goudet; Arnaud Murat; Patricia Niccoli-Sire; Alain Calender; Vincent Rohmer; Olivier Chabre; Antoine Tabarin

OC3.2

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Endocrine Abstracts (2007) 14 OC3.2

ECE2007 Oral Communications Endocrine tumors & neoplasia (7 abstracts)

Adrenal lesions in multiple endocrine neoplasia type 1: data from the French Group for the Study of Endocrine Tumors (GTE)

Blandine Gatta ¹ , Maud Monsaingeon ¹ , Pierre Goudet ² , Arnaud Murat ³ , Patricia Niccoli-Sire ⁷ , Alain Calender ⁵ , Vincent Rohmer ⁶ , Olivier Chabre ⁴ & Antoine Tabarin ¹

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¹Dpt Endocrinology, Bordeaux, France; ²Dpt Surgery, Dijon, France; ³Dpt Endocrinology, Nantes, France; ⁴Dpt Endocrinology, Grenoble, France; ⁵Lab Genetics, Lyon, France; ⁶Dpt Endocrinology, Angers, France; ⁷Dpt Endocrinology, Marseille, France.

The characteristics of adrenal involvement in Multiple Endocrine Neoplasia type 1 (MEN1) have been defined from studies involving a limited number of patients. We have assessed retrospectively the prevalence, characteristics and evolution of adrenal involvement from the French group for the study of endocrine tumours (GTE) registry, involving 688 patients with MEN1. In our series, adrenal tumours identified at abdominal imaging occurred in 130 patients (18.9%). The mean age of patients at the discovery of the adrenal lesion was 46.1 yr (range, 2–78 yr). Adrenal lesions were bilateral in 32% of cases and the mean tumor diameter was 27.6 mm (range, 7–70 mm). Hormonal hypersecretion was found in 16% of patients with adrenal involvement (10 cases of Cushing’s syndrome, 7 cases of primary hyperaldosteronism, 2 cases of hyperandrogenism and 1 pheochromocytoma). Among adrenal lesions that were removed, histopathologic examination revealed benign lesions (adenoma and hyperplasia) in 87.5% of cases, adrenal carcinoma in 7.5% and adrenal metastasis in 5%. Overall, malignancy of adrenal lesions was documented in 3.8% of the whole series. Adrenal lesions were associated with enteropancreatic tumors in 66.4% of cases. In patients in whom follow-up imaging was available (mean 6.6 years), 15% demonstrated significant tumoral progression and 13% developed controlateral lesions. No case of adrenal malignancy was found during the follow-up. No correlation was found between genotypic lesions of the menin gene and the presence or the type of adrenal lesion. In our series, adrenal tumours are a less frequent than previously reported. Most of adrenal lesions are small in size benign and not responsible for hormonal hypersecretion. Our series do not support the hypothesis of a physiopathologic link between pancreatic tumours and adrenal lesions in MEN1.