Bronchopulmonary neuroendocrine tumors (BP-NETs) comprise 20% of all lung cancers. Typical low grade carcinoid tumors (TC) represent the majority of BP-carcinoids. Intermediate grade atypical carcinoid tumors (AC) are less often encountered but more aggressive. Although metastatic dissemination to mediastinal lymph nodes as well as liver, skeleton, central nervous system, skin and mammary glands is well known, the involvement of endocrine glands is really rare.
Case report: A 54 year old man presented with severe headache, left temporal hemianopsia, ptosis of the left eye lid, polyouria and polydipsia. MRI of the brain revealed a large pituitary mass of 3.5 cm in diameter that compressed the optic chiasm and infiltrated the cavernous sinus. Chest x-ray shoewd a mass located by the right hilum area that was confirmed by CT scan of the thorax. CT scan of the abdomen demonstrated an enlargement of the right adrenal measuring 3 cm. Histological examination of lung biopsy revealed the presence of an intermediate grade AC. MIBG scan was performed to exclude the coexistence of a neuroendocrine tumor and an adrenal pheochromocytoma. The latter showed high uptake of the radioactive tracer in pituitary, right lung and right adrenal supporting the diagnosis of a neuroendocrine malignancy metastatic to the endocrine glands.
A bone marrow biopsy, performed due to persistently increased white blood cell counts in the absence of active infection, revealed infiltration byf carcinoid. The patient underwent cranial irradiation followed by chemotherapy using the combination of etoposide and cisplatin. Despite an initial temporary improvement of CNS symptoms after the cranial irradiation, the patient suffered rapidly progressive deterioration of the disease in the lung, adrenal and pituitary accompanied by leptomeningeal dissemination and eventually died.
Prague, Czech Republic
24 - 28 Apr 2010
European Society of Endocrinology