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

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

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 study the clinicopathological features, imaging characteristics, immunophenotypes and differential diagnosis of giant cell angioblastoma (GCAB).

METHODSa case of GCAB in the left middle-upper tibia and fibula was studied by light microscopy, X-ray and CT imaging, immunohistochemistry.

RESULTSX-ray and CT imaging showed a clearer lesion in the left middle-upper tibia than in the ipsilateral fibula with enlarged ostealleosis and increased inhomogeneously medullary cavity density, irregular thickening of cortical bone, local cortical default at the inner edge, soft tissue swelling around the abnormal bone. Histologically, tumor tissue was located between the bone trabeculae by nodular, linear and plexiform aggregates of oval-to-spindle cells, large mononucleate cells and multinucleate giant cells with prominent nucleoli and abundant granular eosinophilic cytoplasm. Some aggregates had uncentain amount of discernible lumens, either empty or containing few erythrocytes. A concentric arrangement of oval-to-spindle Cells around small-caliber vascular structures together with collagen fiber contributed to a so-called 'onion-skin' arrangement. The background showed a loose mesenchymal stroma formed of some inconspicuous spindle-fibroblast-like cells, stellate-shape mesenchymal cells, a moderate mononuclear inflammatory cell infiltrate and scattered mast cells. Immunophenotype showed the tumor cells and giant cells strongly positive for vimentin. A good many oval-to-spindle cells stained markedly for CD31 and CD34, but weakly for FVIII, while the giant cells are highlighted instead by CD68, occasionally, very few giant cells showed positive focally for FVIII, a-SMA decorated notedly the cells surrounding the endothelium-like cells but weakly positive in some other tumor cells.

CONCLUSIONGCAB is a rare, locally infiltrative but slow growing neoplastic angiogenesis with unique morphological characteristics during infancy, which may occur not only in the skin, mucosa, subcutis and deep soft tissue but also in the bone.

Actins ; metabolism ; Antigens, CD ; metabolism ; Antigens, CD34 ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Bone Neoplasms ; diagnostic imaging ; metabolism ; pathology ; surgery ; Dermatofibrosarcoma ; metabolism ; pathology ; Diagnosis, Differential ; Fibula ; Giant Cell Tumor of Bone ; diagnostic imaging ; metabolism ; pathology ; surgery ; Hemangioblastoma ; diagnostic imaging ; metabolism ; pathology ; surgery ; Hemangioendothelioma ; metabolism ; pathology ; Hemangioendothelioma, Epithelioid ; metabolism ; pathology ; Hemangioma, Cavernous ; metabolism ; pathology ; Humans ; Infant ; Kasabach-Merritt Syndrome ; Male ; Platelet Endothelial Cell Adhesion Molecule-1 ; metabolism ; Sarcoma, Kaposi ; metabolism ; pathology ; Skin Neoplasms ; metabolism ; pathology ; Thrombocytopenia ; metabolism ; pathology ; Tibia ; Tomography, X-Ray Computed ; Vascular Neoplasms ; metabolism ; pathology ; Vimentin ; metabolism

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

OBJECTIVETo investigate the diagnostic method and analyze the result of microneurosurgical treatment for tumors of the fourth cerebral ventricle.

METHODSTumor of the fourth ventricle was clinically diagnosed in 86 patients basing on the preliminary assessment of symptom and CT or MRI findings. Of these 86 patients treated with micro-neurosurgery, the tumors in 62 were totally removed, subtotally in 19, and partially in 5. Forty-two patients received postoperative radiotherapy.

RESULTSThree patients died postoperatively within ten days, and symptoms in 83 were improved after treatment. The average survival period was over 3 years. The pathology included 32 medulloblastomas, 23 ependymoma, 15 astrocytoma, 10 hemangiblastomas, 2 choroid plexus papillomas, and 4 epidermoid cysts.

CONCLUSIONMedulloblastoma, astrocytoma and hemangiblastoma are suggested to be removed totally whenever technically possible according to the site, character and volume of the tumor. For ependymoma, if close to the brain stem, is recommended to be subtotally removed. Postoperative radiotherapy may be beneficial for malignant types.

Adolescent ; Adult ; Aged ; Astrocytoma ; diagnosis ; diagnostic imaging ; surgery ; Cerebral Ventricle Neoplasms ; diagnosis ; radiotherapy ; surgery ; Child ; Child, Preschool ; Combined Modality Therapy ; Ependymoma ; diagnosis ; diagnostic imaging ; surgery ; Female ; Follow-Up Studies ; Fourth Ventricle ; pathology ; radiation effects ; surgery ; Hemangioblastoma ; diagnosis ; diagnostic imaging ; surgery ; Humans ; Magnetic Resonance Imaging ; Male ; Medulloblastoma ; diagnosis ; diagnostic imaging ; surgery ; Microsurgery ; methods ; mortality ; Middle Aged ; Neoplasm Recurrence, Local ; Survival Analysis ; Survival Rate ; Tomography, X-Ray Computed

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