Pituitary and cerebral metastases - neuroendocrine tumor

Case contributed by Natalie Yang
Diagnosis certain

Presentation

Known metastatic carcinoid with ACTH secretion. Bilateral adrenalectomy.

Patient Data

Age: 30 years
Gender: Male

MRI of the brain demonstrates widespread abnormal enhancement, with multiple cerebral ring enhancing masses, dural thickening and enhancement as well as nodular enhancement of the pituitary and infundibulum. 

The patient went on to have a biopsy of the frontal lesion. 

Histology

MICROSCOPIC DESCRIPTION: The section shows fragments of cerebral tissue containing multiple localized deposits of tumor tissue, which appear to be adjacent to small blood vessels. The tumor deposits vary in size and shape, with the largest ones showing central necrosis. The tumor is cellular, composed of crowded elongate cells with oval nuclei. There are occasional rosettes or tubules surrounded by tumor cells. Tumor cell nuclei show generally granular nucleoplasm and, frequently,  small nucleoli. There are scattered mitotic figures at a rate of 20/50 hpf. The Ki67 index is approximately 30% of tumor cells.

The neuroendocrine nature of the cells is confirmed with positive cytoplasmic staining for chromogranin, synaptophysin  and CD56. Staining for TTF-1 shows a very occasional single tumor  cell nucleus positive. About 30% of tumor cells show moderately strong staining for ACTH. Cells are negative for somatostatin.

The features are those of  metastatic  neuroendocrine tumor, lacking morphologic features of  small cell  carcinoma. Applying  criteria for neuroendocrine  tumors described in the WHO Classification  of Tumors of the Digestive System, Bosman et al,  IARC, Lyon, 2010, this tumor would be categorized in the "neuroendocrine  tumor grade 3" group. In the 2004 WHO categorization of  lung tumors, the  terminology would be "atypical carcinoid". 

FINAL DIAGNOSIS: Left  frontal  tumor -  metastatic neuroendocrine  tumor (G3) showing  evidence of ACTH production.  

Case Discussion

This case illustrates widespread intracranial metastases from a neuroendocrine tumor. 

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