Difficult Diagnostic Cases

Schuch, Gunter; Wit, Maike de; Höltje, J.; Laack, Eckart; Schilling, Georgia; Hossfeld, Dieter K.; Fiedler, Walter; Scigalla, P.; Jacobs, M. S.

doi:10.1200/jco.2005.01.184

Cited by 22 publications

(7 citation statements)

References 9 publications

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“…In cases with unresectable lesions, where alternative treatment options such as radiotherapy and chemotherapy fail to produce significant responses, antiangiogenic treatment has been attempted. [ 85 ] High levels of VEGF in clinical samples provide the basis for the use of specific antiangiogenic treatment of HBL targeting VEGF signaling. Schuch et al .…”

Section: Discussion and Review Of Literaturementioning

confidence: 99%

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

Pandey

Sharma

Pandey³

et al. 2016

Asian J Neurosurg

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Supratentorial hemangioblastomas (HBLs) are rare, benign vascular tumors of the central nervous system neoplasms. Very scarce literature is available regarding supratentorial HBL without von Hippel–Lindau (VHL) syndrome in an adult. We reviewed the literature and PubMed advanced search showed only a few results of supratentorial HBL without VHL syndrome. We reported a rare case of cystic supratentorial HBL in 39-year-old male affecting the parietal lobe without VHL syndrome. Supratentorial HBL is a rare tumor and supratentorial HBL without VHL syndrome are even rarer. Being a rare entity, not much clinical data is currently available regarding supratentorial HBLs, thus necessitating the need for further reporting and review of such cases.

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Section: Discussion and Review Of Literaturementioning

confidence: 99%

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

Pandey

Sharma

Pandey³

et al. 2016

Asian J Neurosurg

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“…Surgical resection combined with radiotherapy is the treatment of choice for most patients with spinal cord tumours, as no significant improvement in survival has been observed with chemotherapy alone in small cohort studies. 1–6 Given the limited efficacy of chemotherapy, rationally designed therapeutics for spinal cord tumours are urgently needed.…”

Section: Introductionmentioning

confidence: 99%

“…5,22 Haemangioblastomas are most commonly treated with radiotherapy or surgery but, owing to their hypervascular nature, the efficacy of angiogenesis inhibitors in these lesions is currently under investigation. 1,6 …”

Section: Introductionmentioning

confidence: 99%

Spinal cord tumours: advances in genetics and their implications for treatment

et al. 2013

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Tumours of the spinal cord, although rare, are associated with high morbidity. Surgical resection remains the primary treatment for patients with this disease, and offers the best chance for cure. Such surgical procedures, however, carry substantial risks such as worsening of neurological deficit, paralysis and death. New therapeutic avenues for spinal cord tumours are needed, but genetic studies of the molecular mechanisms governing tumourigenesis in the spinal cord are limited by the scarcity of high-quality human tumour samples. Many spinal cord tumours have intracranial counterparts that have been extensively studied, but emerging data show that the tumours are genetically and biologically distinct. The differences between brain and spine tumours make extrapolation of data from one to the other difficult. In this Review, we describe the demographics, genetics and current treatment approaches for the most commonly encountered spinal cord tumours—namely, ependymomas, astrocytomas, haemangioblastomas and meningiomas. We highlight advances in understanding of the biological basis of these lesions, and explain how the latest progress in genetics and beyond are being translated to improve patient care.

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“…Another report using SU5416 documented improvement in neurologic symptoms and a stable appearance of a spinal HB for 24 months [10]. In the largest series using SU5416, Madhusudan et al [11] treated six VHL patients with progressive CNS HBs.…”

Section: Discussionmentioning

confidence: 99%

Erlotinib therapy for central nervous system hemangioblastomatosis associated with von Hippel-Lindau disease: a case report

Rogers

LoRusso

Nadler³

et al. 2010

J Neurooncol

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There is a need for effective systemic therapy for central nervous system (CNS) hemangioblastomas (HBs). We report a case of erlotinib therapy for CNS HBs in a patient with von Hippel-Lindau disease, in whom the HBs were associated with diffuse leptomeningeal seeding. We provide the first report of paired serum and cerebrospinal fluid (CSF) levels of erlotinib while on standard dosing. The patient exhibited neurologic and imaging signs of recurrent CNS HBs and progressive leptomeningeal metastasis following surgery, radiation, and stereotactic radiosurgery. The patient was treated with erlotinib 150 mg daily. The patient achieved a minor response to erlotinib therapy, including clinical improvement, reduction in size of two enhancing brain lesions (one of which, however, proved at autopsy to be radiation necrosis) and stabilization of leptomeningeal enhancement. In addition, the CSF white count improved. The duration of response was 9 months. The median plasma and CSF levels of erlotinib while on treatment were 1146.06 and 247.83 ng/ml, respectively (CSF 21.6% of plasma). Erlotinib may have antitumor activity in CNS HBs.

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Difficult Diagnostic Cases

Cited by 22 publications

References 9 publications

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

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

Spinal cord tumours: advances in genetics and their implications for treatment

Erlotinib therapy for central nervous system hemangioblastomatosis associated with von Hippel-Lindau disease: a case report

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