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Endocrine Abstracts (2014) 35 P567 | DOI: 10.1530/endoabs.35.P567

First Department of Medicine, University of Szeged, Szeged, Hungary.

The neuroendocrine tumor of thymus (TNET) is an extremely rare disease. It can occur sporadically or as a part of the multiple endocrine neoplasia (MEN1) syndrome. TNET may secret hormons (ectopic ACTH production). It is a potentially malignant tumor which often develops distant metastases. Its prognostic factors are the tumor size, histological grade, Ki67 index, paraneoplastic symptoms, surgical resection and Masaoka staging. The options for treatment are radical surgery, chemotherapy (temozolomide, platinum based agents) and somatostatin analogue therapy (SSA).

First case: investigations of a 38-year-old male patient revealed the presence of primary hyperparathyroidism due to a parathyroid adenoma, a pituitary adenoma, a pancreatic neuroendocrine tumor, neuroendocrine cancer of the thymus, hormonally inactive adrenocortical adenomas, and a non-endocrine tumor (facial angiofibroma). All these components together witnessed for the diagnosis of MEN 1. Parathyroid and thymic tumors were surgically removed. The patient has been kept under continuous SSA therapy for his pancreatic and thymic tumors. The clinical diagnosis was proved by genetic methods.

Second case: diagnostic procedures of a 51-year-old female patient showed an ACTH producing mediastinal tumor; non radical surgical removal was performed due to tumorous infiltration of the blood vessels. Histology revealed well differentiated neuroendocrine carcinoma of thymus. The general condition of the patient became rapidly deteriorated and she died after the surgical intervetion soon at the intensive care unit.

In our presentation two cases of a very rare disease are to be demonstrated. The patient with MEN1 syndrome is still in a symptom and metastasis free period after 3 years of surgical removal of his two distinct neuroendocrine tumors and under a continuous SSA therapy. In the other case the rapid progression of the disease led to the death of the patient soon after the surgical intervention and before any further therapeutic procedure.

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