A hemangioblastoma is a benign, highly vascular tumor that can occur in the brain, spinal cord, and retina the lightsensitive tissue that lines the back of the eye. Pdf ct and mri features of a solid hemangioblastoma of. Cerebellar hemangioblastomas are traditionally classified into two. A hemangioblastoma is a noncancerous tumor that forms in the lower part of the brain, spinal cord, or retina. Jun 29, 2016 welldifferentiated papillary thyroid carcinoma generally ptc have a favorable prognosis. Supratentorial hemangioblastoma archives of pathology. Pdf solid hemangioblastomas of cerebellum are extremely rare in children and may. Moreover, it is extremely rare for a cerebellar metastasis to occur as an initial distant metastasis of ptc, and hemangioblastoma is the most common primary cerebellar neoplasm in adults. Added value of arterial spinlabeling mr imaging for the differentiation of cerebellar hemangioblastoma from metastasis. Central nervous system capillary haemangioblastoma. Occurrence outside the central nervous system is exceptionally rare.
The histopathological features of cerebellar hemangioblastoma. Cerebellar ependymoma with overlapping features of clearcell. Review of fiftythree cases, with special reference to cerebellar cysts and the association of polycythemia. We report a rare case of a symptomatic cerebellar hemangioblastoma in an. Primary hyperparathyroidism infratentorial hemangioblastoma is a rare, benign tumor ocurring in middleaged adults. At this age, hemangioblastoma is the most likely diagnosis. Most clinicians suspect metastatic disease unless proven otherwise.
Cerebellar hemangioblastoma american journal of medicine. Computed tomography and magnetic resonance imaging revealed a mass in the cerebellum. Jun 11, 2017 posterior fossa hemangioblastomas are who grade i benign lesions with a surprisingly high recurrence rate. The mass creates mass effect upon the fourth ventricle and cerebral aqueduct, displacing the left cerebellar hemisphere. Jul 05, 2011 a hemangioblastoma is a benign, highly vascular tumor that can occur in the brain, spinal cord, and retina the lightsensitive tissue that lines the back of the eye. Histopathology of hemangioblastoma involving the optic nerve orbital and intracraneal portions. Histopathology revealed a hemangioblastoma after surgical excision. Cerebellar hemangioblastoma europe pmc article europe pmc. Article pdf available in turkish journal of cancer 304. Its histopathological features have been well established. In this article, 2 cases of capillary hemangioblastoma diagnosed in our department of pathology are presented and the histopathological and the clinical features are discussed. Hemangioblastoma is a vascular tumor of the central nervous system cns. Acar2, tulay canda1 departments of 1pathology and 2neurosurgery, dokuz eylul university medical school, izmirturkey. In our patient population, tumors that remained solid were asymptomatic and well tolerated in the cerebellum.
Following resection of this mass, the histology was that of a hemangiobla. Cerebellar hemangioblastoma is a benign neoplasm of uncertain origin that comprises approximately 7% of posterior fossa tumors in adults. Hemangioblastoma of the cerebellum in an octogenarian t he differential diagnosis for a homogenously enhancing posterior fossa lesion in the elderly population is relatively limited. Cerebellar liponeurocytoma is a rare cerebellar neoplasm found primarily in adults. This is a case report of a sporadic renal haemangioblastoma occurring in a 32 year old woman. Solid cerebellar hemangioblastoma with peritumoral edema. As it enlarges, it presses on the brain and can cause neurological symptoms, such as headaches, weakness, sensory loss, balance and coordination problems, andor hydrocephalus a. Hemangioblastoma of the optic nerve producing bilateral optic. Cerebellar hemangioblastomas are frequently referred to as lindau tumors because swedish pathologist arvid vilhelm lindau first described them in 1926. Hemangioblastoma in the brain with neurosurgeon dr. As it enlarges, it presses on the brain and can cause neurological symptoms, such as headaches, weakness, sensory loss, balance. Cerebellar hemangioblastoma with perivascular pseudorosette. Hemangioblastoma is actually a capillary hemangioma and, despite the name with the affix of blastoma, it is a low grade who grade i lesion note that the calvarial hemangioma is a cavernous hemangioma.
They are usually in the cerebellum 76% and uncommon in the cerebral hemispheres 9%. Herein, we present a case of cerebellar hemangioblastoma in a 43yearold woman. She presented with postpartum fatigue and was found to be polycythaemic with haemoglobin. Because of its locali zation, the clinical course if formidable and the diagno sis is often made only after the appearance of intra cranial hypertension. The differential diagnosis for a posterior fossa cystic mass with a mural nodule in an adult includes hemangioblastoma, pilocytic astrocytoma and metastasis, among others. These tumors make up about 1 to 2 percent of all intracranial neoplasms and occur primarily in. Metastatic cerebellar tumor of papillary thyroid carcinoma. Typically, patients are diagnosed in the second to third decades of life, although retinal lesions may be present at birth.
Pdf solid cerebellar hemangioblastoma with peritumoral. Hemangioblastoma genetic and rare diseases information. It remains unknown if there is an underlying genetic abnormality that could contribute to the formation of both vascular tumors in this particular case. Pilocytic astrocytoma and hemangioblastoma are considered the most likely etiologies. Sporadic hemangioblastomas most often arise within the cerebellar hemispheres, whereas those arising within the context of vhl may occur. Neurological imaging suggested hemangioblastoma hb. This metastasis is rare in the central nervous system. Rare coexistence of a cerebellar hemangioblastoma and. Pdf hemangioblastomas are angioblastic tumors of the central nervous system. Polycythemia and high serum erythropoietin level as a result of hemangioblastoma. Cerebellar hemangioblastoma definition of cerebellar. Sporadic primary renal haemangioblastoma diagnostic. Supratentorial hemangioblastoma with unusual features.
Hemangioblastomas, or haemangioblastomas, are vascular tumors of the central nervous system that originate from the vascular system, usually during middle age. Sometimes, these tumors occur in other sites such as the spinal cord and retina. Hemangioblastoma hb is a vascular tumor of the cns. Original article clinicopathologic features of hemangioblastomas. Pathology of brain tumorsd r amit thapa 0 10 20 30 40 50 60 70 80 90 100 relative incidence 010203040506070 age years posterior fossa medulloblastoma ependymoma pilocytic astrocytoma other sites cranopharyngioma chorioid plexus tumorus cerebellar hemisphere diffuse astrocytoma anaplastic astroyctoma oligodendroglioma ependymoma other sites.
We present a rare case of cerebellar metastasis, one that mimics a solid type cerebellar hemangioblastoma and because of which it was very hard to reach accurate preoperative diagnosis. Capillary hemangioblastomas are benign, highly vascular tumors of controversial origin limited almost exclusively to the central nervous system. Hemangioblastoma definition of hemangioblastoma by medical. Central nervous system hemangioblastoma is a histolog ically benign tumor that usually occurs in the cerebellum. Sep 26, 2017 hemangioblastoma is the most common primary adult intraaxial posterior fossa tumor. It is the most common primary intra axial neoplasm of the adult posterior fossa. S100 highlighting the presence of a small nerve bundle in an arteriovenous vascular malformation original magni. The now widely accepted name is cerebellar liponeurocytoma. Imaging and histology of clearcell ependymoma and cerebellumbased hemangioblastoma are similar. Initially, this entity was thought to hold similarities. Case report, clinical report by journal of evolution of medical and dental sciences.
Aug 15, 2017 a hemangioblastoma is a noncancerous tumor that forms in the lower part of the brain, spinal cord, or retina. Summary capillary hemangioblastomas are benign, highly vascular tumors of controversial origin limited almost exclusively to the central nervous system. This study determines the factors responsible for recurrence and the clinicoradiological and histopathological differences between primary group a. Atkinson, in modern surgical pathology second edition, 2009. Onethird of patients with cerebellar hemangioblastoma have vhl disease.
The ipsilateral anterior inferior cerebellar artery aica and posterior inferior cerebellar artery pica branches were the main feeding vessels of the hemangioblastoma. They may be associated with other diseases such as polycythemia increased blood cell count, pancreatic. Review of fiftythree cases with special reference to cerebellar cysts and the association. Extramedullary hematopoiesis in cerebellar hemangioblastoma. The tumor develops in the lining of a blood vessel.
Moreover, cerebellar hemangioblastoma and breast angiomatosis is a very exceptional combination and research needs to be performed to establish the exact significance of this coexistence. Kang km, sohn ch, you sh, nam jg, choi sh, yun tj, et al. Hemangioblastoma 1 a tumour of soft tissues, which is of intermediate aggressiveness and thus preferably known as hemangioendothelioma. We have demonstrated that, in vhl, cerebellar hbs begin as nodules, and some subsequently develop enlarging cysts that cause pressure symptoms. Up to 40% are vhlrelated and display the characteristic mutation at 3p2526 catapano et al 2005.
Hemangioblastoma is a highly vascular neoplasm commonly involving the cerebellum and spinal cord. Immunoreactivity of cd10 and inhibin alpha in differentiating. Retinal capillary hemangioblastoma angiomatosis retinae, previously known as retinal capillary hemangioma is a rare autosomal dominant condition with a reported incidence of 1 in 40,000. While the cerebellum is by far the most frequent site, these lesions also tend to. Cerebellar hemangioblastomas hbs are traditionally classified into different morphologic types. Notice the vascularized tumor in the center and residual optic nerve tissue. Cerebellar hemangioblastoma and primary hyperparathyroidism. The tumor is usually wellcircumscribed with a highly vascular mural nodule almost always abutting pial layer and a peripheral cyst which. This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment.