Supratentorial haemangioblastoma without von Hippel–Lindau syndrome in an adult: A rare tumor with review of literature

Pandey, Sharad; Sharma, Vishal; Pandey, Daya Shankar; Kumar, Vijay; Kumar, Mohan

doi:10.4103/1793-5482.165782

Cited by 17 publications

(24 citation statements)

References 65 publications

(115 reference statements)

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“…Supratentorial hemangioblastoma occurs mainly in the frontal lobe, followed by the parietal lobe and temporal lobe. 6 The 6 cases reported in this article were all sporadic, of which 4 occurred in the frontal lobe and 2 occurred in the parietal lobe, and this result was consistent with the lesion location reported in other studies.…”

Section: Discussionsupporting

confidence: 91%

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Cerebral Hemangioblastoma Without Von Hippel-Lindau Syndrome: A Report of 6 Cases

et al. 2020

Int J Surg Pathol

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Background. Hemangioblastoma occurs mainly in the cerebellum and rarely in the cerebrum. Objective. The present study aimed to analyze the clinical manifestations and radiological and pathological features of cerebral hemangioblastoma, and to improve the recognition of this tumor and avoid misdiagnosis. Methods. The characteristics of 6 patients with cerebral hemangioblastoma were analyzed, and a retrospective review of cerebral hemangioblastoma reported in the literature was performed. Results. All 6 patients were female, aged from 22 to 70 years (55 years on average), and all cases were wild-type sporadic, in which 4 cases occurred in the frontal lobe and 2 cases occurred in the parietal lobe. Imaging revealed a solid tumor in 4 cases, a cystic tumor in 1 case, and a mixed tumor in 1 case. Microscopically, the morphology and immunophenotype of tumor cells were not different from those of classical hemangioblastoma. All 6 patients survived tumor free during the follow-up period. Conclusions. Cerebral hemangioblastoma often simulates the imaging characteristics of meningioma or glioma. Enough attention should be paid to differential diagnosis before the operation, and exact diagnosis relies on the pathological examination.

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Section: Discussionsupporting

confidence: 91%

“…8 VEGF inhibitors for antiangiogenic therapy may be a new choice of treatment. 6 However, due to the rarity of supratentorial hemangioblastoma and insufficient evidence in the existing literature, the best treatment needs to be further explored.…”

Section: Discussionmentioning

confidence: 99%

Cerebral Hemangioblastoma Without Von Hippel-Lindau Syndrome: A Report of 6 Cases

et al. 2020

Int J Surg Pathol

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“…Metastasis was unlikely given negative metastatic workup, clinical features of hemangioblastoma such as a history of von Hippel-Lindau (VHL) and polycythemia were not present, and imaging features associated with hemangiopericytoma, such as lytic bone lesions and lack of calcifications, were not consistent with our patient's MRI findings (47–50). Furthermore, supratentorial hemangioblastoma without VHL is exceedingly rare, with only about 60 total reported cases, and hemangiopericytoma likewise only represents 0.4% of primary intracranial tumors, making meningioma the most likely pathology based on clinical and imaging features (46, 51). Additionally, multiple intracranial meningiomas are known to occur in up to 10% of patients with diagnoses of meningioma, a much higher incidence than both primary hemangioblastoma and hemangiopericytoma (52).…”

Section: Discussionmentioning

confidence: 99%

Possible Abscopal Effect Observed in Frontal Meningioma After Localized IMRT on Posterior Meningioma Resection Cavity Without Adjuvant Immunotherapy

et al. 2019

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Background: Localized radiation therapy (RT) is known to infrequently cause off-target or “abscopal” effects at distant metastatic lesions. The mechanism through which abscopal effects occur remains unknown, but is thought to be caused by a humoral immune response to tumor-specific antigens generated by RT. Combination treatment regimens involving RT and immunotherapy to boost the humoral immune response have demonstrated synergistic effects in promoting and accelerating abscopal effects in metastatic cancer. Nevertheless, abscopal effects, particularly after RT alone, remain exceedingly rare.Case Presentation: We report the case of an 84-year-old man with an atypical meningioma, who demonstrated a radiographically significant response to an untreated second intracranial lesion, likely also a meningioma, after intensity-modulated radiation therapy (IMRT) to a separate, detatched resection cavity. Serial annual MRI imaging starting at 2- to 3.5-year (most recent) post-IMRT follow-up demonstrated a persistent decrease in both tumor size and surrounding edema in the untreated second lesion, suggestive of a possible abscopal effect.Conclusions: We describe here the first report of a potential abscopal effect in meningioma, summarize the limited literature on the topic of abscopal effects in cancer, and detail the existing hypothesis on how this phenomenon may occur and possibly relate to the development of future treatments for patients with metastatic disease.

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“…Hemangioblastomas account for 2% of intracranial tumors and is associated with von Hippel-Lindau syndrome. 77 It commonly occurs in patients aged 20-40 years. The commonest site is the posterior fossa, particularly the cerebellum.…”

Section: Hemangioblastomamentioning

confidence: 99%

“…Patients need long-term follow-up. 77 Table 1 summarizes the features and management of uncommon low-grade tumors. Primary surgery followed by close follow-up remains the standard of care.…”

Section: Hemangioblastomamentioning

confidence: 99%

Uncommon low-grade brain tumors

et al. 2018

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An initial search of PubMed used broad search terms 'brain tumors', 'low-grade', 'radiotherapy, 'chemotherapy', 'surgery' and 'treatment' from January 1990 to March 2018. A subsequent focused search was undertaken using the names of individual histological subtypes of low-grade brain tumors as in the 2016 WHO classification. Only papers published in English were reviewed. The final reference list was generated on the basis of originality and relevance to the broad scope of this review.

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Supratentorial haemangioblastoma without von Hippel–Lindau syndrome in an adult: A rare tumor with review of literature

Cited by 17 publications

References 65 publications

Cerebral Hemangioblastoma Without Von Hippel-Lindau Syndrome: A Report of 6 Cases

Cerebral Hemangioblastoma Without Von Hippel-Lindau Syndrome: A Report of 6 Cases

Possible Abscopal Effect Observed in Frontal Meningioma After Localized IMRT on Posterior Meningioma Resection Cavity Without Adjuvant Immunotherapy

Uncommon low-grade brain tumors

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