Metastasis of renal cell carcinoma to central nervous system hemangioblastoma in two patients with von Hippel–Lindau disease

Abstract

Here we report tumor-to-tumor metastases identified in two patients with von Hippel-Lindau (VHL) disease. The first patient had bilateral renal carcinomas and multiple cerebellar hemangioblastomas, and the second patient had a renal carcinoma and multiple hemangioblastomas in the retina, cerebellum and spinal cord. A cerebellar lesion from the first patient and a spinal lesion from the second patient contained two distinct components. The inner part of these tumors consisted of a nested mass of polygonal clear cells that expressed cytokeratin and epithelial membrane antigen, while the outer part of the tumors showed proliferation of capillaries and intervening foamy stromal cells that were negative for cytokeratin and epithelial membrane antigen. The tumors were thus considered to be hemangioblastomas complicated by metastatic lesions of renal cell carcinoma of clear cell type. These cases indicate that tumor-to-tumor metastasis should be considered when hemangioblastoma contains a clear cell carcinoma component in the setting of VHL disease, and that immunohistochemical staining for cytokeratin and epithelial membrane antigen is useful for the diagnosis.

Breast Carcinoma Metastasis in Recurrent Myxoid Liposarcoma

“…Abundant vascularization and slow growth of a low grade tumor make it vulnerable to blood borne metastases. Benign and lowgrade tumors generally proliferate slowly and remain unnoticed for a long period, thus become candidates for a focus of metastasis [10]. Rich vascularity and high lipid content of the myxoid liposarcoma might have provided a suitable environment for metastasis in the presented case.…”

“…The metastatic tumor is commonly an aggressive carcinoma and the recipient tumor is a benign or a low-grade mesenchymal neoplasm [4][5][6][7]. Lung, breast, melanoma and renal cell carcinoma are the most common metastatic tumors and the target of metastasis is a tumor like meningioma [8][9][10][11], leiomyoma, fibroma or an adenoma. Renal cell carcinoma may also act as a recipient [12].…”

Metastasis of renal cell carcinoma to a haemangioblastoma of the medulla oblongata in von Hippel–Lindau syndrome

“…3 RCC and meningioma are the most common visceral and intracranial recipients of metastasis, respectively, although the mechanism of tumor-to-tumor metastasis is not well understood. 2,4,5 In theory, both HAB and meningioma are good recipients of RCC because they have abundant vascularization, slow growth and often remain unnoticed for a long time. 2,4,5 Metastatic RCC to HAB of the CNS was first reported in 1992, in a patient with VHL disease.…”

“…2,4,5 In theory, both HAB and meningioma are good recipients of RCC because they have abundant vascularization, slow growth and often remain unnoticed for a long time. 2,4,5 Metastatic RCC to HAB of the CNS was first reported in 1992, in a patient with VHL disease. 6 To our knowledge, only 14 patients with metastatic RCC to HAB have been reported, and all patients had been diagnosed with VHL syndrome.…”

Expression of HIF-1 regulated proteins vascular endothelial growth factor, carbonic anhydrase IX and hypoxia inducible gene 2 in hemangioblastomas

“…The differential diagnosis between metastatic CCRCC and hemangioblastoma is particularly critical in VHL syndrome patients since they are at increased risk of developing both of these tumors. This is illustrated by reports of VHL patients who were found to have metastatic carcinoma within CNS hemangioblastomas [8,14,19]. A few markers have been suggested as helpful in the diagnosis of hemangioblastoma and in distinguishing hemangioblastoma from CCRCC.…”