1.Disseminated Hemangioblastomatosis of the Central Nervous System without von Hippel-Lindau Disease: A Case Report.
Hong Rae KIM ; Yeon Lim SUH ; Jong Won KIM ; Jung Il LEE
Journal of Korean Medical Science 2009;24(4):755-759
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
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Cerebellar Neoplasms/pathology/radiotherapy/surgery
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Hemangioblastoma/*diagnosis/pathology/surgery
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Humans
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Magnetic Resonance Imaging
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Male
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Middle Aged
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Recurrence
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von Hippel-Lindau Disease/genetics
2.Clear cell meningioma: report of a case.
Zhi-yi ZHOU ; Rong-chao SUN ; Shu-dong YANG ; Jia-bei LIANG ; Jun RUI
Chinese Journal of Pathology 2009;38(8):562-563
Diagnosis, Differential
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Ependymoma
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metabolism
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pathology
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Female
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Hemangioblastoma
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metabolism
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pathology
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Humans
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Ki-67 Antigen
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metabolism
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Meningeal Neoplasms
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metabolism
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pathology
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surgery
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Meningioma
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metabolism
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pathology
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surgery
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Middle Aged
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Mucin-1
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metabolism
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Neoplasm Recurrence, Local
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Vimentin
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metabolism
3.Recent advance in von Hippel-Lindau syndrome-related and sporadic hemangioblastomas of central nervous system.
Jing ZHOU ; Nan-yun LI ; Xiao-jun ZHOU
Chinese Journal of Pathology 2010;39(3):212-215
Antigens, CD34
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metabolism
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Carcinoma, Renal Cell
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metabolism
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pathology
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Central Nervous System Neoplasms
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metabolism
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pathology
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surgery
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ultrastructure
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Diagnosis, Differential
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Ependymoma
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metabolism
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pathology
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Hemangioblastoma
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metabolism
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pathology
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surgery
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ultrastructure
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Humans
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Meningeal Neoplasms
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metabolism
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pathology
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Meningioma
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metabolism
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pathology
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Mutation
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Neoplasm Recurrence, Local
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Prognosis
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Receptor, Epidermal Growth Factor
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metabolism
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Vimentin
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metabolism
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von Hippel-Lindau Disease
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genetics
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metabolism
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pathology
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surgery
4.Clinicopathologic characteristics of sporadic renal hemangioblastoma.
Jing GUO ; Liang LI ; Li-hua ZHAO ; Yan LI ; Lin-sheng WANG ; Lin XIAO ; Ren-ya ZHANG ; E-mail: HZZHANG_1964@163.COM.
Chinese Journal of Pathology 2013;42(10):679-682
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
5.Diagnosis and micro-neurosurgery for the fourth cerebral ventricle tumors.
Wei-Wei HU ; Xiu-Jue ZHENG ; Gang SHEN ; Wei-Guo LIU ; Hong SHEN ; Wei-Ming FU ; Jing-Yi ZHOU
Chinese Journal of Oncology 2007;29(2):144-146
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
6.Clinicopathologic study of giant cell angioblastoma.
Rong-Jun MAO ; Qi-Ming LI ; Yue-Ming GUO ; Wei-Qiang LI ; Chang-Shu FAN ; Xiong-Zeng ZHU
Chinese Journal of Pathology 2010;39(11):752-756
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