MEDizzy - A study of adults with cerebellar neoplasms reveals that

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Central Nervous System (II)

A study of adults with cerebellar neoplasms reveals that some of them have an autosomal dominant inheritance pattern with von Hippel–Lindau disease. Their cerebellar tumors are cystic with a mural nodule. Molecular analysis of tumor cells shows increased amounts of hypoxia-inducible factor (HIF). The incidence of renal cell carcinomas is increased in these persons. Which of the following paraneoplastic manifestations is most likely to be found in these adults?

ExplanationHemangioblastomas are part of the spectrum of neoplasms seen in association with von Hippel–Lindau disease. The VHL gene encodes for a ubiquitin-ligase component important in the degradation pathway of HIF, and this increases vascular endothelial growth factor (VEGF) expression to drive vascular proliferation. Cushing syndrome with increased corticosteroid production can be related to ectopic ACTH production by a carcinoma. Cushing disease is caused by an ACTH-producing adenohypophyseal adenoma. Hypercalcemia of malignancy often results from parathormone-related peptide production by a carcinoma. Syndrome of inappropriate antidiuretic hormone (SIADH) maybe caused by neuroendocrine tumors such as small cell lung carcinomas