Pituitary and cerebral metastases: neuroendocrine tumour

Case contributed by Dr Natalie Yang

Presentation

Known metastatic carcinoid with ACTH secretion. Bilateral adrenalectomy.

Patient Data

Age: 30 years
Gender: Male

MRI brain

Modality: MRI

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 localised deposits of tumour tissue, which appear to be adjacent to small blood vessels. The tumour deposits vary in size and shape, with the largest ones showing central necrosis. The tumour is cellular, composed of crowded elongate cells with oval nuclei. There are occasional rosettes or tubules  surrounded by tumour cells. Tumour 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 tumour 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 tumour  cell nucleus positive. About 30% of tumour cells show moderately strong staining for ACTH. Cells are negative for somatostatin.

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

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

Case Discussion

This case illustrates widespread intracranial metastases from a neuroendocrine tumour. 

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Case Information

rID: 17934
Case created: 26th May 2012
Last edited: 20th Jan 2016
Inclusion in quiz mode: Included

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