Hemangioblastoma (HBL) in the pituitary stalk is extremely rare. Only 16 such cases have been reported in the past and 5 cases have been treated with surgical procedure. Here, we report surgical case of HBL in the pituitary stalk diagnosed in a 34-year-old woman. The patient underwent a gross-total resection via the modified lateral supra-orbital approach. No recurrence was observed in two years after surgery. To our knowledge, this is the 17th case of HBL in the pituitary stalk and the 6th surgical case. If the tumor is symptomatic and the volume is over 5 cubic centimeters as in our case, we recommend that the surgical resection of the HBL in the pituitary stalk is a more safe and reasonable than radiotherapy.
Adult ; Cerebellar Neoplasms/pathology/*surgery ; Female ; Hemangioblastoma/pathology/*surgery ; Humans ; Pituitary Gland/*pathology ; Treatment Outcome

Hemangioblastoma ; metabolism ; pathology ; surgery ; Humans ; Male ; Middle Aged ; Optic Nerve ; pathology ; Optic Nerve Neoplasms ; metabolism ; pathology ; surgery ; Phosphopyruvate Hydratase ; metabolism ; Vimentin ; metabolism ; von Willebrand Factor ; metabolism

OBJECTIVETo analyze and summarize the clinical and pathological characteristics of intracranial haemangioblastoma and to improve surgery effect.

METHODSeventy-two patients with intracranial haemangioblastoma who were proven by operation and pathology from 1970 to 1988 were analyzed retrospectively.

RESULTSIntracranial haemangioblastoma tends to occur in the hemisphere of cerebellum (83 tumours, 87%) and the age of them ranged from 20 to 40 years (47 cases, 58.3%) mostly. The ratio of men (46 cases) was higher than women (26 cases). The diagnosis of the disease depends on CT and MR substantive haemangioblastoma. The most effective and reliable treatment of intracranial haemangioblastoma is surgical resection.

CONCLUSIONIntracranial haemangioblastoma is benign tumour which can be, cured by total surgical resection. The key recurrence factors include the young age of initial onset, mistaken exploration and incomplete extirpation of tumour.

Adolescent ; Adult ; Brain Neoplasms ; pathology ; surgery ; Child ; Female ; Hemangioblastoma ; pathology ; surgery ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Treatment Outcome ; Young Adult

Adult ; Carcinoma, Renal Cell ; pathology ; surgery ; Cerebellar Neoplasms ; pathology ; surgery ; Cystadenoma, Papillary ; pathology ; surgery ; Diseases in Twins ; pathology ; surgery ; Epididymis ; pathology ; surgery ; Genital Neoplasms, Male ; pathology ; surgery ; Hemangioblastoma ; pathology ; surgery ; Humans ; Kidney Neoplasms ; pathology ; surgery ; Male ; von Hippel-Lindau Disease ; pathology ; surgery

We report a very rare case of hemangioblastomatosis that developed after surgical removal of a solitary cerebellar hemangioblastoma (HB). A 51-yr-old man presented with back pain 10 yr after undergoing surgery for cerebellar HB. Magnetic resonance imaging showed numerous mass lesions along the entire neuraxis accompanied by prominent leptomeningeal enhancement. Genomic DNA analysis showed no mutation in the von Hippel-Lindau (VHL) genes. A surgical specimen obtained from a lesion in the cauda equina showed pathological findings identical to those of the cerebellar HB that had been resected 10 yr earlier. External beam radiation therapy and radiosurgery were subsequently performed; however, the patient succumbed one year after receiving the diagnosis of hemangioblastomatosis. The reduction of tumor cell spillage during surgery and regular long-term follow-up are recommended for patients with HBs.
Central Nervous System Neoplasms/*diagnosis/pathology/surgery ; Cerebellar Neoplasms/pathology/radiotherapy/surgery ; Hemangioblastoma/*diagnosis/pathology/surgery ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Recurrence ; von Hippel-Lindau Disease/genetics

Diagnosis, Differential ; Ependymoma ; metabolism ; pathology ; Female ; Hemangioblastoma ; metabolism ; pathology ; Humans ; Ki-67 Antigen ; metabolism ; Meningeal Neoplasms ; metabolism ; pathology ; surgery ; Meningioma ; metabolism ; pathology ; surgery ; Middle Aged ; Mucin-1 ; metabolism ; Neoplasm Recurrence, Local ; Vimentin ; metabolism

PURPOSE: Standard treatment of asymptomatic spinal cord hemangioblastoma in von Hippel-Lindau (VHL) disease has yet to be established. The purpose of this study was to propose guidelines for the treatment of asymptomatic spinal cord hemangioblastomas in VHL disease. MATERIALS AND METHODS: VHL disease patients treated for spinal cord hemangioblastomas between 1999 and 2009 were included. All spinal cord hemangioblastomas were divided into three groups: Group 1, asymptomatic tumors at initial diagnosis followed with serial imaging studies; Group 2, asymptomatic tumors at initial diagnosis that were subsequently resected; and Group 3, symptomatic tumors at initial diagnosis, all of which were resected. RESULTS: We identified 24 spinal cord hemangioblastomas in 12 patients. Groups 1, 2 and 3 comprised 13, 4 and 7 tumors, respectively. Group 1 exhibited a smaller tumor volume (257.1 mm3) and syrinx size (0.8 vertebral columns) than those of Group 2 (1304.5 mm3, 3.3 vertebral columns) and Group 3 (1787.4 mm3, 6.1 vertebral columns). No difference in tumor volume or syrinx size was observed between Groups 2 and 3. Five tumors in Group 1 were resected during follow-up because symptoms had developed or the tumor had significantly grown. Finally, among 17 asymptomatic tumors at the initial diagnosis, nine tumors were resected. Only one tumor of these nine tumors resulted in neurological deficits, while five of seven symptomatic tumors caused neurological deficits. CONCLUSION: Selective resection of asymptomatic tumors before they cause neurological deficits might bring about better outcomes.
Adult ; Aged ; Aged, 80 and over ; Female ; Hemangioblastoma/etiology/*pathology/*surgery ; Humans ; Male ; Middle Aged ; Treatment Outcome ; von Hippel-Lindau Disease/*complications

Antigens, CD34 ; metabolism ; Carcinoma, Renal Cell ; metabolism ; pathology ; Central Nervous System Neoplasms ; metabolism ; pathology ; surgery ; ultrastructure ; Diagnosis, Differential ; Ependymoma ; metabolism ; pathology ; Hemangioblastoma ; metabolism ; pathology ; surgery ; ultrastructure ; Humans ; Meningeal Neoplasms ; metabolism ; pathology ; Meningioma ; metabolism ; pathology ; Mutation ; Neoplasm Recurrence, Local ; Prognosis ; Receptor, Epidermal Growth Factor ; metabolism ; Vimentin ; metabolism ; von Hippel-Lindau Disease ; genetics ; metabolism ; pathology ; surgery

OBJECTIVETo study clinicopathologic features, diagnosis, treatment and prognosis of von Hippel-Lindau (VHL) syndrome-related and sporadic hemangioblastomas of the central nervous system (CNS-HB).

METHODSHistopathological, ultrastructural, immunohistochemical (EnVision method) and clinical features of 21 VHL syndrome and 63 sporadic CNS-HB cases were studied with correlation of the available follow-up information.

RESULTSTwenty-one VHL patients accompanied with a total of 87 CNS-HBs, including one patient of developing 12 HBs within 13 years. There were 10 patients presenting other lesions related to VHL, including 6 retinal HBs, 4 pancreatic tumors (endocrine tumor and microcystic cystadenoma), 1 clear renal cell carcinoma, 4 renal cysts and 1 endolymphatic sac tumor. One patient developed 5 different tumors related to VHL within a period of 4 years. In the 63 cases of sporadic CNS-HB (34 male and 29 female), the mean age was 43.0 years. Among the 18 VHL syndrome patients with available follow-up information, 14 were still alive and within them, 4 became disabled and 11 had developed new lesions. The other 4 patients died. Among the 42 patients of sporadic HB with follow-up information, 39 were alive including 3 disabled cases, and the other 3 died. Histologically, the tumors showed large and vacuolated stromal cells. Some tumors showed atypical nuclei. Involvement of the brain tissue was seen in 32 cases, among which, 21 patients with available follow-up information were learnt to be alive. Tumor cells of HB stained positive for vimentin, EGFR, Inhibin alpha and D2-40, but negative for CD34 and CD68. In 3 cases of HB, some stromal cells were positive for GFAP. All cases showed a low expression for Ki-67, except 2 cases with 2% and 1 case with 5% Ki-67 indices.

CONCLUSIONSVHL syndrome is a multisystem disorder with a poor prognosis and a high rate of missed diagnosis. The syndrome is characterized by development of various benign and malignant tumors. The most common tumor is CNS-HB, which occurs predominantly in the cerebellum. Patients with VHL syndrome tend to present at a younger age than patients with sporadic CNS-HBs, and VHL related HB occurs more predominantly in the brain stem and spinal cord. Prognosis of CNS-HB patients is not correlated with the nuclear atypicality, expression for Ki-67 and involvement of the brain tissue. Because new lesions may develop during the patient's lifetime. So that, regular clinical inspection is recommended in order to check up the development of any new lesions.

Adolescent ; Adult ; Carcinoma, Renal Cell ; metabolism ; pathology ; surgery ; Central Nervous System Neoplasms ; metabolism ; pathology ; surgery ; Child ; Female ; Follow-Up Studies ; Glial Fibrillary Acidic Protein ; metabolism ; Hemangioblastoma ; metabolism ; pathology ; surgery ; Humans ; Inhibins ; metabolism ; Ki-67 Antigen ; metabolism ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Pancreatic Neoplasms ; metabolism ; pathology ; surgery ; Receptor, Epidermal Growth Factor ; metabolism ; Retinal Neoplasms ; metabolism ; pathology ; surgery ; Survival Analysis ; Vimentin ; metabolism ; Young Adult ; von Hippel-Lindau Disease ; metabolism ; pathology ; surgery

OBJECTIVETo investigate the clinicopathological characteristics of sporadic renal hemangioblastoma.

METHODSTwo cases of sporadic renal hemangioblastoma were studied by histological and immunohistochemical staining, along with review of the literature.

RESULTSBoth cases were presented with a renal mass during annual physical examination with an age of 40 and 45 years, respectively. The lesions located in the middle portion of the kidney with a sharp tumor border. Histopathologically, the tumors were characterized by two major components: capillary and stromal cells. Immunohistochemically, the stromal cells were positive for vimentin, NSE, S-100 protein and inhibin-α, and negative for CK, HMB45, Melan A, EMA, CD56 and syn. Both tumors showed a very low level of Ki-67 labeling. The endothelial cells were positive for CD34.

CONCLUSIONSporadic renal hemangioblastoma is a rare benign neoplasm, the diagnosis of which is based on the characteristic follicular architecture and an exclusion of other carcinomas of the kidney.

Adult ; Angiomyolipoma ; metabolism ; pathology ; Antigens, CD34 ; metabolism ; Carcinoma, Renal Cell ; metabolism ; pathology ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Hemangioblastoma ; diagnostic imaging ; metabolism ; pathology ; surgery ; Humans ; Immunohistochemistry ; Inhibins ; metabolism ; Kidney Neoplasms ; diagnostic imaging ; metabolism ; pathology ; surgery ; Male ; Middle Aged ; Nephrectomy ; Phosphopyruvate Hydratase ; metabolism ; S100 Proteins ; metabolism ; Tomography, X-Ray Computed ; methods ; Vimentin ; metabolism