Rathke's cleft cysts in twins with Type 2C von Hippel-Lindau disease

Report of 2 cases

Wei X. Huff M.D., Ph.D.1, José M. Bonnin M.D.2, and Daniel H. Fulkerson M.D.3
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  • 1 Department of Neurological Surgery,
  • | 2 Department of Pathology and Laboratory Medicine, and
  • | 3 Department of Neurological Surgery, Division of Pediatric Neurosurgery, Goodman Campbell Brain and Spine, Indiana University School of Medicine, Indianapolis, Indiana
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Von Hippel-Lindau disease (VHLD) is characterized by a spectrum of benign and malignant tumors in the CNS and visceral organs. Rathke's cleft cysts are benign, nonneoplastic sellar lesions that are often asymptomatic. The authors report the case of twin sisters with VHLD Type 2C with radiographically similar sellar lesions. One twin required surgery for progressive visual loss. Pathological examination of resected tissue demonstrated Rathke's cleft cyst.

Abbreviations used in this paper:

pVHL = von Hippel-Lindau protein; VHLD = von Hippel-Lindau disease.

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