01 June 2002
Thymic carcinoid associated with familial multiple endocrine neoplasia type-1
Neerod Kumar Jha , John Stubberfield , Leon Platt Bignold , Kallukudige Jayaprasanna , Bruce McKenzie ThomsonCase Rep Clin Pract Rev 2002; 3(2):68-70 :: ID: 474526
Abstract
Background: Mediastinal or thymic carcinods are rare tumors and arise from the cells of neuro-endocrine origin and known for their unpredictable behavior, tendency to recur, early metastasis and poor response to the radiotherapy. Carcinoids of thymus are often confused clinically with thymoma, which has an entirely different but favorable prognosis. Multiple endocrine neoplasia, type-1 (MEN-1) is associated with carcinoid of thymus
in 25% cases. The familial nature of MEN-1 signifies screening for thymic tumors in the sibling’s mandatory.
Case Report: A 32 year old man is presented herein, who was found to have familial MEN-1 in association with a large antero-superior mediastinal mass on chest x-ray and CT Scan. He underwent successful surgical excision
through median sternotomy approach. On histopathological examination including immunoperoxidase method his tumor was reported to be thymic carcinoid.
Conclusions: This report highlights the clinical features, surgical and pathological findings, therapeutic modalities and the prognosis associated with this rare tumor and need for screening of endocrine glands including thymus gland of patients with MEN-1 and the family members for early detection and the better outcome. CT scan of the chest should be included in the follow-up. Prophylactic thymectomy may be considered during surgery for
parathyroid gland in the patients with MEN-1 to reduce the risk of thymic carcinoid and associated problems in the future.
Keywords: Mediastinum, thymus, carcinoid, Multiple Endocrine Neoplasia
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