scholarly journals Extra and intradural spinal Hemangioblastoma

2012 ◽  
Vol 11 (3) ◽  
pp. 242-244
Author(s):  
Marcelo Campos Moraes Amato ◽  
Caio César Marconato Simões Matias ◽  
João Alberto Assirati Junior ◽  
Aline Paixão Becker ◽  
Carlos Gilberto Carlotti Junior ◽  
...  
Keyword(s):  
Histopathological Examination ◽  
Correct Diagnosis ◽  
White Male ◽  
Residual Tumor ◽  
Illustrative Case ◽  
Low Grade ◽  
Von Hippel Lindau ◽  
Von Hippel Lindau Disease ◽  
The Central Nervous System ◽  
Spinal Hemangioblastoma

Hemangioblastomas of the central nervous system (CNS) are low-grade highly vascularized tumors that may be sporadic or associated with Von Hippel-Lindau disease. Extradural hemangioblastomas are uncommon and those located extra and intradurally are even rarer. This study uses an illustrative case and literature review to discuss the difficulties to consider the correct diagnosis and to select the best surgical approach. A 57 years-old white male patient presented with myelopathy and right C5 radiculopathy. The images showed a lobulated, hourglass shaped, highly enhanced extra/intradural lesion that occupied the spinal canal and widened the C4-C5 right intervertebral foramen. Total resection of the intradural lesion was achieved through a posterior approach, but the extradural part could only be partially removed. Complete improvement was observed after four months of follow-up and the residual tumor has been followed up clinically and radiologically. Even though the preoperative impression was of a spinal schwannoma, the histopathological examination revealed grade I hemangioblastoma as per WHO. Despite their rarity, current complementary exams allow considering the diagnosis of hemangioblastoma preoperatively. That is essential to a better surgical planning in view of the particular surgical features of this lesion.

Hemangioblastoma diagnosis and surveillance in von Hippel–Lindau disease: a consensus statement

2021 ◽  
pp. 1-6
Author(s):  
Kristin Huntoon ◽  
Matthew J. Shepard ◽  
Rimas V. Lukas ◽  
Ian E. McCutcheon ◽  
Anthony B. Daniels ◽  
...  
Keyword(s):  
Age At Onset ◽  
Benign Tumors ◽  
Expert Committee ◽  
Level Of Evidence ◽  
Von Hippel Lindau ◽  
Assessment Development ◽  
History Of ◽  
Von Hippel Lindau Disease ◽  
The Central Nervous System ◽  
Cancer Network

OBJECTIVE Hemangioblastomas are a frequent underlying cause of neurological morbidity and death in patients with von Hippel–Lindau disease (VHL). Although these benign tumors can cause significant neurological debility when undetected and untreated, unified evidence-based surveillance recommendations for VHL patients have not been established. To develop consensus recommendations, the VHL Alliance established an expert committee, named the International VHL Surveillance Guidelines Consortium, to define surveillance recommendations. METHODS The Central Nervous System (CNS) Hemangioblastoma Subcommittee of the Guidelines Consortium was formed as a multidisciplinary team of experts in the diagnosis and management of hemangioblastomas. Recommendations were formulated using the Grading of Recommendations, Assessment, Development and Evaluation (GRADE) and National Comprehensive Cancer Network Categories of Evidence and Consensus categorization after a comprehensive literature review. RESULTS Published studies (n = 49) that discussed age at onset, MRI frequency, natural history of VHL, and the risks and benefits of surveillance were analyzed. Based on this analysis, the authors recommend that clinical evaluation (yearly) be used as the primary screening tool for hemangioblastomas in VHL. The subcommittee suggests that screening be performed between the ages of 11 and 65 years, or with the onset of symptoms, for synchronicity with other testing regimens in VHL. The subcommittee also recommends that baseline MRI be first performed at the age of 11 years (suggested 2B, level of evidence D) or after identification of neurological symptoms or signs (if earlier) and continue every 2 years (recommended 2A, level of evidence A). CONCLUSIONS The CNS Hemangioblastoma Subcommittee of the International VHL Surveillance Guidelines Consortium here proposes guidelines that aim to increase the early detection of VHL-associated hemangioblastomas to reduce their morbidity and mortality.

scholarly journals A RARE CASE OF SPORADIC SUPRATENTORIAL HEMANGIOBLASTOMA

2020 ◽  
pp. 57-63
Author(s):  
V. E. Kocharyan ◽  
T. G. Sarkisyan ◽  
G. I. Kovalev ◽  
G. G. Muzlaev ◽  
A. I. Bogrov
Keyword(s):  
World Literature ◽  
Rare Case ◽  
Benign Tumor ◽  
Tumor Resection ◽  
Von Hippel Lindau ◽  
Dense Networks ◽  
Rare Benign Tumor ◽  
Von Hippel Lindau Disease ◽  
The Central Nervous System ◽  
The Brain

We report the rare case of 65-year-old male patient with supratentorial hemangioblastoma and gigantic cyst of the left hemisphere of the brain without von Hippel-Lindau disease. Hemangioblastoma is a rare benign tumor classified as grade I by the WHO of the central nervous system of uncertain histogenesis. This neoplasm’s stroma consists of dense networks of thin-walled blood vessels of various calibers. About 140 cases of supratentorial localization of this type of tumor are presented in the world literature. We have given a fairly complete clinical, neuroradiological and histological picture that allows one to differentiate hemangioblastoma from other histological structures. Promising modalities in the treatment of patients with this pathology when it is impossible to perform total tumor resection are considered.

scholarly journals A case of glucose-sensitive insulinoma accompanied by slightly elevated serum insulin levels and persisting convulsions after the surgically removed neuroendocrine tumor

2013 ◽  
Vol 154 (2) ◽  
pp. 69-73
Author(s):  
Zsuzsanna Soós ◽  
Anna Czégeni ◽  
Ferenc Salamon ◽  
Mónika Salamon ◽  
István Kenessey ◽  
...  
Keyword(s):  
Neuroendocrine Tumor ◽  
Pancreatic Beta Cells ◽  
Serum Insulin ◽  
Correct Diagnosis ◽  
Elevated Serum ◽  
Pancreatic Neuroendocrine Tumor ◽  
Residual Tumor ◽  
Surgical Removal ◽  
Von Hippel Lindau ◽  
Insulin Levels

Insul(in)oma is a usually solitary or, in some cases, multifocal tumor of pancreatic beta cells. It may be a component of multiple endocrine neoplasia type 1. or von Hippel-Lindau syndrome. In typical forms the diagnosis – based on the Whipple triad – is simple, however, it may be difficult to recognize in cases with near normal or only slightly elevated serum insulin levels, as well as in patients with known convulsive episodes. With the case presentation of an 81-year-old woman the authors draw attention to the pitfalls of the correct diagnosis. A special feature of the presented case is that convulsions persisted after surgical removal of the pancreatic neuroendocrine tumor verified with functional and imaging methods. Recurrant or residual tumor was not found, and morphological damage of the brain was absent. In the background of the continuing convulsions cerebrovascular alterations as well as the cytotoxic effect of the hypoglycemia-induced excessive glutamate production can be postulated. Orv. Hetil., 2013, 154, 69–73.

scholarly journals Resection of spinal hemangioblastoma

2014 ◽  
Vol 37 (v2supplement) ◽  
pp. Video15
Author(s):  
Giuseppe Lanzino ◽  
Saul F. Morales-Valero ◽  
William E. Krauss
Keyword(s):  
Spinal Cord ◽  
Venous Drainage ◽  
Complete Resection ◽  
Cervical Cord ◽  
Von Hippel Lindau ◽  
Link Type ◽  
Single Piece ◽  
Von Hippel Lindau Disease ◽  
Spinal Hemangioblastoma

Spinal cord hemangioblastomas occur as sporadic lesions or in the setting of Von Hippel-Lindau disease. In this intraoperative video we present a case of sporadic cervical cord hemangioblastoma and illustrate the main surgical steps to achieve safe and complete resection which include: identification and division of the feeding arteries; careful circumferential dissection of the tumor from the surrounding gliotic cord; identification, isolation and division of the main venous drainage and single piece removal of the tumor.The video can be found here: http://youtu.be/I7DxqRrfTxc.

MUCINOUS LOW-GRADE ADENOCARCINOMA ARISING IN AN INTRACRANIAL ENTEROGENOUS CYST

Neurosurgery ◽  
2008 ◽  
Vol 62 (4) ◽  
pp. E972-E973 ◽  
Author(s):  
Marco Gessi ◽  
Federico G. Legnani ◽  
Emanuela Maderna ◽  
Cecilia Casali ◽  
Carlo L. Solero ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Malignant Transformation ◽  
Histopathological Examination ◽  
Present Report ◽  
Cranial Nerves ◽  
Residual Tumor ◽  
Magnetic Resonance Imaging Scan ◽  
Low Grade ◽  
Resonance Imaging

Abstract OBJECTIVE Enterogenous cysts (ECs) of the central nervous system are developmental malformations that occur in the spinal canal, posterior fossa, or cerebral hemispheres. They are usually benign lesions, and malignant transformation is rare. To date, only three cases of malignant transformation have been reported in the literature. We present a case of a cerebellopontine EC showing foci of epithelial dysplasia and malignant transformation into a low-grade papillary mucinous adenocarcinoma. CLINICAL PRESENTATION A 25 year-old man with a 6-year history of hypoacusia presented to our department with facial nerve deficit, visual disturbances, and gait instability. A magnetic resonance imaging scan demonstrated a multiloculated cerebellopontine angle cyst with supratentorial hydrocephalus. INTERVENTION A retrosigmoidal approach was used to achieve cyst removal. This was followed several months later by ventriculoperitoneal shunt placement. The cyst was adherent to the brainstem, cranial nerves, and vessels, and it resembled a thin encapsulated structure filled with mucinous-like substance. No solid component was identified. Histopathological examination revealed an EC with foci of malignant transformation in a mucinous papillary adenocarcinoma. Magnetic resonance imaging was performed 5 months postoperatively due to progressive clinical worsening; this scan revealed lesion recurrence with severe brainstem compression. Emergency surgery was performed, and a large decompression was achieved. Subsequent follow-up computed tomographic scans showed progression of the residual tumor. The patient's neurological condition rapidly worsened, ultimately resulting in death. CONCLUSION The present report suggests that a careful histological examination of all ECs after surgery should be made to exclude dysplastic foci or carcinomatous transformation. Although the clinical behavior of ECs with malignant trasformation is unpredictable, surgery remains the treatment of choice. The use of possible adjuvant chemo- or radiotherapy has not been established.

scholarly journals Propranolol and Somatostatin Agonist Therapy Resulting in Symptom Improvement and Stabilization of von Hippel-Lindau-Associated Hemangioblastoma: A Case Report

2021 ◽  
Author(s):  
Maran Ilanchezhian ◽  
Reinier Alvarez ◽  
Amanda Carbonell ◽  
Brigitte C. Widemann ◽  
Prashant Chittiboina ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Autosomal Dominant ◽  
Symptom Improvement ◽  
Agonist Therapy ◽  
Von Hippel Lindau ◽  
Von Hippel Lindau Disease ◽  
The Central Nervous System ◽  
Tumor Types ◽  
Systemic Therapies

Abstract Von Hippel-Lindau disease is an inherited, autosomal dominant tumor predisposition syndrome which leads to susceptibility for developing hemangioblastomas of the central nervous system and retina, as well as other tumor types. No approved systemic therapies exist for this disease. Here, we present a patient diagnosed with Von Hippel-Lindau disease, with central nervous system and retinal hemangioblastomas, treated with propranolol and subsequently with Lanreotide. On both treatments, the patient showed notable symptom improvement. In addition, since starting Lanreotide, the patients hemangioblastoma has remained stable on imaging. Based on the symptom improvement in this patient and other reported findings in the literature, propranolol and somatostatin agonist therapy may have an anti-tumor effect in Von Hippel-Lindau associated hemangioblastomas; however, further investigation is warranted.

scholarly journals Radiation-associated Pituitary Sarcoma

2018 ◽  
Vol 25 (2) ◽  
pp. 161-165
Author(s):  
Felipe Andres Constanzo ◽  
Jaime Mauricio Pinto ◽  
Thomas Wilhem Schmidt ◽  
Maria Loreto Spencer
Keyword(s):  
Short Term Memory ◽  
Background Radiation ◽  
Histopathological Examination ◽  
Rare Complication ◽  
Memory Loss ◽  
Whole Brain Radiotherapy ◽  
Residual Tumor ◽  
Optic Chiasm ◽  
Low Grade ◽  
Brain Radiotherapy

Background: Radiation therapy is commonly used as primary or adjuvant treatment for pituitary adenomas. Frequent side effects of this modality include hypopituitarism, visual disturbances, short-term memory loss and secondary tumors, sarcomas being among the rarest of the sellar region, with only 55 reported cases to date. Clinical Presentation: A 57-year old man with hypopituitarism and a 20-year history of a non-functioning pituitary macroadenoma subtotally resected four times and undergoing whole brain radiotherapy and bromocriptine, who developed acute visual deterioration due to compression of the optic chiasm by rapid growth of the residual tumor. An endoscopic transplanum approach was performed achieving gross total resection. Histopathological examination was consistent with an undifferentiated low-grade pituitary sarcoma. The patient’s vision improved after surgery, but healthcare-associated complications compromised his clinical outcome, succumbing to a pulmonary embolism a month after surgery. Conclusion: Radiation-induced pituitary sarcoma is a very rare complication of radiotherapy. There is no distinctive feature on imaging that can predict its occurrence. Prognosis is grim, without effective management and a mean survival of 6.5 months after diagnosis.

scholarly journals Pathology of the Nervous System in Von Hippel-Lindau Disease

2015 ◽  
Vol 2 (3) ◽  
pp. 114-129 ◽  
Author(s):  
Alexander O. Vortmeyer ◽  
Ahmed K. Alomari
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Hypoxia Inducible Factors ◽  
Metastatic Renal Cancer ◽  
Vhl Gene ◽  
Von Hippel Lindau ◽  
Von Hippel Lindau Disease ◽  
The Central Nervous System ◽  
Vhl Disease ◽  
Common Manifestation

Von Hippel-Lindau (VHL) disease is a tumor syndrome that frequently involves the central nervous system (CNS). It is caused by germline mutation of the VHL gene. Subsequent VHL inactivation in selected cells is followed by numerous well-characterized molecular consequences, in particular, activation and stabilization of hypoxia-inducible factors HIF1 and HIF2. The link between VHL gene inactivation and tumorigenesis remains poorly understood. Hemangioblastomas are the most common manifestation in the CNS; however, CNS invasion by VHL disease-associated endolymphatic sac tumors or metastatic renal cancer also occur, and their differentiation from primary hemangioblastoma may be challenging. Finally, in this review, we present recent morphologic insights on the developmental concept of VHL tumorigenesis which is best explained by pathologic persistence of temporary embryonic progenitor cells. 

Symptomatic intrasellar hemangioblastoma in a child treated with subtotal resection and adjuvant radiosurgery

1996 ◽  
Vol 84 (6) ◽  
pp. 1046-1050 ◽  
Author(s):  
Paul D. Sawin ◽  
Kenneth A. Follett ◽  
B. Chen Wen ◽  
Edward R. Laws
Keyword(s):  
Residual Tumor ◽  
Subtotal Resection ◽  
Resonance Imaging ◽  
Subsequent Growth ◽  
Conventional Radiotherapy ◽  
Content Type ◽  
Von Hippel Lindau ◽  
Adjuvant Therapies ◽  
Von Hippel Lindau Disease ◽  
Fine Print

✓ The first documented case of a symptomatic intrasellar hemangioblastoma is described, occurring in an 11-year-old girl with stigmata of von Hippel—Lindau disease who presented with headaches, progressive bitemporal hemianopsia, and adenohypophysial dysfunction. A subtotal resection of the lesion was achieved with two separate surgical procedures: a transsphenoidal approach and a subfrontal craniotomy. Subsequent growth of residual tumor was treated with combined conventional radiotherapy and stereotactic radiosurgery. Two years following completion of these adjuvant therapies, no residual tumor was evident on magnetic resonance imaging. Previous experience with hemangioblastoma in this region, as well as the rationale for radiotherapy in the treatment of incompletely resected lesions, is reviewed.

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