Sporadic supratentorial hemangioblastoma with meningeal affection: A case report and literature review
- Department of Neurosurgery, Lozano Blesa University Clinical Hospital, Zaragoza, Spain.
Juan Francisco Sánchez-Ortega, Department of Neurosurgery, Lozano Blesa University Clinical Hospital, Zaragoza, Spain.
DOI:10.25259/SNI_441_2021Copyright: © 2021 Surgical Neurology International This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
How to cite this article: Juan Francisco Sánchez-Ortega, Marta Claramonte, Mónica Martín, Juan Calatayud-Pérez. Sporadic supratentorial hemangioblastoma with meningeal affection: A case report and literature review. 09-Aug-2021;12:394
How to cite this URL: Juan Francisco Sánchez-Ortega, Marta Claramonte, Mónica Martín, Juan Calatayud-Pérez. Sporadic supratentorial hemangioblastoma with meningeal affection: A case report and literature review. 09-Aug-2021;12:394. Available from: https://surgicalneurologyint.com/surgicalint-articles/11034/
Background: Hemangioblastomas are vascular tumors, of benign behavior, that originate in the central nervous system. Supratentorial hemangioblastomas are extremely rare and are generally associated with Von Hippel-Lindau disease (VHL). The involvement of structures by contiguity, such as blood vessels or meninges, is something exceptional. There are few references in the literature of supratentorial hemangioblastomas with meningeal involvement and most of them are described in reports or small case series.
Methods: We performed a systematic review of the literature to observe the characteristics of patients with supratentorial hemangioblastomas with meningeal involvement. In addition, we present the case of a 43-year-old male patient with a sporadic supratentorial hemangioblastoma with both, meningeal and vascular involvement that recurred years after treatment.
Results: The patients presented supratentorial tumors with meningeal involvement, we had a 1.2:1 ratio male-female distribution. The mean age was 50 years. Most tumors were located in the cerebral hemispheres, the lobe affected most frequently was the frontal lobe. About 67% of the cases were sporadic and only 21% were related to VHL disease. There were three cases of recurrence after surgery.
Conclusion: Supratentorial hemangioblastomas are extremely rare lesions. It is rare for supratentorial hemangioblastomas to invade adjacent structures such as blood vessels or meninges, however, when this happens, it is recommended a preoperative vascular imaging study, especially in parasagittal hemangioblastomas where superior longitudinal sinus may be involved. In these cases, en bloc surgical resection is difficult and the probability of recurrence is higher. Although clinical trials and studies with a greater casuistry are necessary to establish guidelines for the management of supratentorial hemangioblastomas, nowadays, contribution of new cases is useful for understanding this pathology.
Keywords: Meninges, recurrence, supratentorial hemangioblastoma, Von Hippel-Lindau disease
Hemangioblastomas are benign tumors of vascular origin that develops in the central nervous system. They represent approximately 2% of all intracranial tumors and the most frequent nonmalignant intra-axial tumor in the posterior fossa in adults.[
The present study presents the case of a 43-year-old male patient with a sporadic supratentorial hemangioblastoma, with meningeal involvement, which recurs 10 years after the first surgery. A review of the characteristics of these tumors described in the literature is performed.
A comprehensive systematic review of literature was performed. Articles were identified through PubMed search using the key words “supratentorial hemangioblastoma” and “spontaneous hemangioblastoma” and in combination with the key words “VHL disease” and “meningeal involvement.” All references of these articles were classified to extract data about patients that presented a supratentorial hemangioblastoma and had meningeal involvement whom underwent surgery. A total of 168 articles on supratentorial hemangioblastomas were identified, of which, only 32 had meningeal involvement. All references contained individual patient data or purely supratentorial aggregated data sets of either histologically confirmed tumors. Both patients with sporadic hemangioblastomas and VHL disease-associated hemangioblastoma were included in the study.
Data from individual and aggregated case series were extracted from each article. The epidemiological characteristics of the patients, the location of the tumor, the appearance of the tumor, the association with VHL disease, and postoperative relapses were reviewed.
Finally, we present the case of a 43-year-old male patient who had previously undergone an epididymal cyst 18 years earlier and a right parasagittal parietal hemangioblastoma 12 years ago [
(a) Hematoxylin-eosin (H-E) staining showing the tumor invading meningeal structures. (b) H-E staining showing tumor tissue with numerous blood vessels. (c) H-E staining showing stromal and capillary cells with hemosiderin deposits due to bleeding. (d) Stromal cells showing immunoreactivity for CD34. (e) Vascular endothelial proliferation showing immunoreactivity for podoplanin D2-40. (f) H-E staining showing neoplastic stromal cells.
The literature search yielded a total of 168 references that contained data on patients with supratentorial hemangioblastomas. However, only 32 patients had a supratentorial hemangioblastoma with meningeal involvement.[
Finally, only three cases of supratentorial hemangioblastoma with meningeal involvement presented recurrence 2 years after surgery.
Supratentorial hemangioblastomas are rare tumors and account for 1–2% of all hemangioblastomas.[
Supratentorial hemangioblastomas that develop in adjacent areas or involving meningeal structures are even rarer. According to Rocha et al.[
Most supratentorial hemangioblastomas are located in the cerebral hemispheres. Of these 33 supratentorial hemangioblastomas with meningeal involvement cases described in the literature, 31 (94%) were located in the cerebral hemispheres, with the frontal lobe being the most frequent location; while 2 (6%) were located in the sellar/ parasellar region.
As described previously, supratentorial hemangioblastomas usually appear correlated with VHL disease. In relation to supratentorial hemangioblastomas with meningeal involvement, 22 cases (67%) were not related to VHL disease, 7 cases (21%) presented the disease, and in 4 cases (12%), the VHL gene mutation was not identified, neither personal nor family stigmas were described in relation to the clinical case. Our case, despite having suffered from an epididymis cyst, had no other stigmata of VHL disease and the genetic study was negative. At the moment, it is unknown why some hemangioblastomas invade the meningeal membranes and whether or not there is a genetic mutation that predisposes these tumors to have a certain affinity for invading adjacent tissues. Therefore, in patients with intracranial hemangioblastomas, it is recommended to carry out a genetic and extension study to determine whether or not the patient suffers from this disease.
The pathogenesis of the disease is unknown, although tumor secretion of certain factors, such as vascular endothelial growth factor (VEGF), platelet-derived growth factor, transforming growth factor type alpha (TGD-α), or erythropoietin, is associated with progressive tumor growth and erythrocyte production, respectively. The most accepted hypothesis for this process is a mutation of the VHL gene that encodes the VHL protein, a protein involved in proteasomal degradation of the hypoxia-inducible factor (HIF-1 alpha).[
The most frequent clinical manifestations of patients with supratentorial hemangioblastomas are headache and seizures.
These can be partial or partial with secondary generalization and their expression depends on the location of the lesion. The case we present suffered from partial sensory seizures in the left extremities in relation to a supratentorial hemangioblastoma located in the parietal lobe and with involvement of the right somesthetic cortex. Other symptoms such as focal deficits or cognitive deterioration are less common and are related to tumors with a significant cystic component that causes compression of the underlying brain parenchyma.[
Radiological diagnosis is based on MRI. In most cases, a solid nodule and an adjacent cystic component are identified. In T1-weighted sequences and after gadolinium administration, the nodule appears homogeneous and hyperintense; while in the T2-weighted sequences, the cystic component is hyperintense. Most infratentorial hemangioblastomas are cystic, whereas supratentorial hemangioblastomas are usually solid cystic with a smaller cystic component. This could be because the brain offers less resistance to fluid diffusion than the cerebellum.[
Blood tests can be useful, since the presence of polyglobulia supports the diagnosis of the disease. Likewise, the presence of other lesions, in particular, hemangioblastomas in other locations, renal or pancreatic tumors, cystadenomas of epididymis (men) or broad ligament (women), and/ or pheochromocytomas can guide the diagnosis of VHL disease.[
The differential diagnosis of supratentorial hemangioblastomas includes other tumors such as meningiomas, hemangiopericytomas, and metastases from renal carcinoma. However, supratentorial hemangioblastomas show many radiological similarities with angioplastic meningiomas,[
The definitive diagnosis is made by histological confirmation. Hemangioblastomas are benign tumors (Grade I of the World Health Organization) and highly vascularized.[
The treatment of hemangioblastomas is radical en bloc resection. In cases where the resection of the tumor is not complete, there is an increased risk of recurrence, something that seems more trending in supratentorial hemangioblastomas that invade structures such as SLS. In our case, the patient underwent a subtotal resection of the hemangioblastoma 12 years earlier, with only some remaining tumor attached to the SLS. After 10 years, the patient develops the recurrence that is presented in this work. In the majority of cases of supratentorial hemangioblastomas with meningeal involvement described in the literature, the removal of the tumor was complete and the follow-up time was not more than 5 years. Only the cases described by Rivera et al.[
Treatment with radiosurgery in small focal lesions or in tumor remains after incomplete resection, seems to delay tumor progression, and, in some cases, keeps the course of the disease stable.[
Supratentorial hemangioblastomas are extremely rare lesions. Despite the fact that the majority of hemangioblastomas are sporadic, those that occur in the supratentorial compartment are more related to VHL disease, so it is necessary to carry out a genetic and systemic study to rule out extracranial lesions in these patients. It is rare for supratentorial hemangioblastomas to invade adjacent structures such as blood vessels or meninges, however, when this happens, it is advisable to perform a preoperative vascular imaging study, especially in parasagittal hemangioblastomas where SLS may be involved. In these cases, en bloc surgical resection is difficult and the probability of recurrence is higher. Despite adjuvant treatment with radiotherapy or stereotactic radiosurgery, it is recommended to closely monitor these patients to detect possible tumor recurrences.
All authors have participated in the preparation of the manuscript and concur with the findings and results.
The authors certify that they have obtained all appropriate patient consent.
There are no conflicts of interest.
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