Hemangioblastoma in the Lung: Metastatic or Primary Lesions?

Abstract

Hemangioblastoma primarily occurs in the CNS, most commonly in the posterior fossa. Extracranial locations are less common, and metastatic tumor involving the lung is exceedingly rare with only three cases previously reported. Two were autopsy studies in patients who died of complications of the CNS hemangioblastomas in 1943 and 1981, and the third was mentioned in a case report addendum providing follow-up information on hepatic hemangioblastoma in 1991. We report a case of a 48-year-old man who presented with multiple lung nodules treated by surgical excision. Pathological study revealed features classic for hemangioblastoma. The patient had a remote history of hemangioblastomas having been excised from the posterior fossa 7 and 20 years previously. This report details a fourth case of metastatic pulmonary hemangioblastoma. It is the first report on surgically resected hemangioblastomas from the lung of a living patient with histological and immunohistochemical characterization.

Von Hippel Lindau Disease with central nervous system and multiple visceral manifestations: A case report

“…It is also important to know the possible manifestations of VHL disease in the lung which include pulmonary arterial hypertension, lung cancer, pulmonary fibrosis, and ARDS [17] . It is important to consider the possibility of metastasizing renal cell carcinoma, especially with multiple lung nodules [18] . The choice of imaging for screening and follow up however is dependent on multiple factors with CT and MRI both having advantages and disadvantages in different scenarios; however, CT is being considered in most scenarios [19] .…”

Congenital extraneural hemangioblastoma in a lamb

“…27 Also, although the stromal cells in the tumor are usually bland, they often show significant nuclear pleomorphism suggestive of a more malignant nature. 27 In humans, immunohistochemistry is routinely used to assist in the diagnosis in both CNS 4 and extraneural 14 cases, especially where stromal cells show a positive reaction to NSE antibody. 14 In HB in dogs, stromal cells have been reported to be positive for NSE.…”

“…27 In humans, immunohistochemistry is routinely used to assist in the diagnosis in both CNS 4 and extraneural 14 cases, especially where stromal cells show a positive reaction to NSE antibody. 14 In HB in dogs, stromal cells have been reported to be positive for NSE. 2,5,6 In our case, stromal cells were positive for NSE.…”