There is a natural tendency to assume that any pathology seen in patients with VHL is therefore a result of VHL. This disregards the statistical probability that a patient with VHL can have additional lesions that are either coinherited or acquired. Once these lesions are "in the literature", however, it is difficult to expunge them without careful study of a large population of VHL patients which is generally not worth the effort. Thus, it is likely that some of the lesions discussed below may not be associated with VHL.
A hepatic hemangioblastoma, identical in histology to the patient's cerebellar hemangioblastoma has been reported (98). Multiple "cavernous hemangiomas" and hepatic cysts have been reported in VHL (12,36,75) but there does not appear to be an increased risk relative to the general population for these common lesions based on our experience.
There have been several reports of pulmonary hemangioblastomas in VHL (12,98,108). These lesions are cystic and lined by the same cuboidal epithelium found elsewhere in VHL. It is possible that they represent metastatic foci (since they are usually found in association with advanced stages of VHL) or embryonic rests.
Omental cysts (12), skeletal hemangiomas, ovarian cysts and angiomas, medullary and papillary carcinoma of the thyroid (63), pituitary adenoma, dermal hemangiomas (36) and pigmented nevi (8) have been reported to occur with VHL.
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