P.037 Disseminated leptomeningeal hemangioblastoma in a case of Von Hippel Lindau

Background: Leptomeningeal dissemination of hemangioblastomas (HB), whether sporadic or associated with Von Hippel Lindau (VHL), are extremely rare. Very scanty literature is available. Methods: A 36 year old female with VHL and stable pancreatic, adrenal and renal lesions was operated upon 4 years ago for a large symptomatic cervicomedullary cystic and solid tumor. 2 years after surgery the tumour recurred and further removal was unsuccessful due to medullary adhesions. Radiation was given to the posterior fossa area and to several small nodules over the cauda equina resulting in severe pain. Serial follow up imaging revealed diffuse leptomeningeal dissemination increasing in size of the suprasellar region, ambient cistern and Sylvian fissures. Clinically, she has been stable with small dose of steroids and VP shunt insertion for papilledema. Results: Review of the literature consists of 2 series of 7 and 21 patients each with leptomeningeal dissemination involving sporadic HB and VHL associated HB. Leptomeningeal dissemination is estimated at about 4.3%. It is postulated that the tumour starts in the Pia and spreads in an extra medullary fashion throughout the subarachnoid spaces. Conclusions: Long term recurrence has been noted raising the question of aggressive treatment with some drug therapy related to angiogenesis is postulated.

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Long-term follow-up and clinical course of a rare case of von Hippel-Lindau disease: A case report and review of the literature

Oncology Letters ◽

10.3892/ol.2016.4387 ◽

2016 ◽

Vol 11 (5) ◽

pp. 3273-3278 ◽

Cited By ~ 2

Author(s):

YU ZOU ◽

JINGJING XU ◽

MINMING ZHANG

Keyword(s):

Case Report ◽

Rare Case ◽

Clinical Course ◽

Review Of The Literature ◽

Von Hippel Lindau ◽

Long Term Follow Up ◽

Von Hippel Lindau Disease

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Long-term stability of fusiform dilatation of the internal carotid artery following surgery adjacent to the circle of Willis: report of 2 cases

Journal of Neurosurgery Pediatrics ◽

10.3171/2020.7.peds20469 ◽

2020 ◽

pp. 1-4

Author(s):

Madeline B. Karsten ◽

R. Michael Scott

Keyword(s):

Carotid Artery ◽

Internal Carotid Artery ◽

Arachnoid Cyst ◽

Pediatric Patients ◽

Internal Carotid ◽

Imaging Finding ◽

Neurological Status ◽

Suprasellar Region

Fusiform dilatation of the internal carotid artery (FDCA) is a known postoperative imaging finding after craniopharyngioma resection. FDCA has also been reported following surgery for other lesions in the suprasellar region in pediatric patients and is thought to be due to trauma to the internal carotid artery (ICA) wall during tumor dissection. Here, the authors report 2 cases of pediatric patients with FDCA. Case 1 is a patient in whom FDCA was visualized on follow-up scans after total resection of a craniopharyngioma; this patient’s subsequent scans and neurological status remained stable throughout a 20-year follow-up period. In case 2, FDCA appeared after resection and fenestration of a giant arachnoid cyst in a 3-year-old child, with 6 years of stable subsequent follow-up, an imaging finding that to the authors’ knowledge has not previously been reported following surgery for arachnoid cyst fenestration. These cases demonstrate that surgery involving dissection adjacent to the carotid artery wall in pediatric patients may lead to the development of FDCA. On very long-term follow-up, this imaging finding rarely changes and virtually all patients remain asymptomatic. Neurointerventional treatment of FDCA in the absence of symptoms or significant late enlargement of the arterial ectasia does not appear to be indicated.

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Microvascular decompression as a surgical management for trigeminal neuralgia: long-term follow-up and review of the literature

Neurosurgical Review ◽

10.1007/s10143-008-0171-3 ◽

2008 ◽

Vol 32 (1) ◽

pp. 87-94 ◽

Cited By ~ 37

Author(s):

Serdar Kabatas ◽

Aykut Karasu ◽

Erdinc Civelek ◽

Akin P. Sabanci ◽

Kemal T. Hepgul ◽

...

Keyword(s):

Trigeminal Neuralgia ◽

Surgical Management ◽

Microvascular Decompression ◽

Review Of The Literature ◽

Long Term Follow Up

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Primary Intrapulmonary Thymomas: Case Report and Review of the Literature

Journal of International Medical Research ◽

10.1177/147323000903700434 ◽

2009 ◽

Vol 37 (4) ◽

pp. 1252-1257 ◽

Cited By ~ 8

Author(s):

L Gong ◽

Y-H Li ◽

X-L He ◽

Q Wang ◽

L Yao ◽

...

Keyword(s):

Epithelial Membrane Antigen ◽

Immunohistochemical Analysis ◽

Lung Tumour ◽

Review Of The Literature ◽

Malignant Tumours ◽

Pathological Characteristics ◽

Immunological Phenotype ◽

Type A Thymoma

Primary intrapulmonary thymomas (PIT), which are intrapulmonary tumours without an associated mediastinal component, are very rare; only 29 cases of PIT have been described in the literature since 1951. This report presents a case of PIT in a 59-year old Chinese woman with a type A thymoma (including its pathogenesis, clinical pathological characteristics, immunological phenotype, treatment and prognosis), in the context of a review of the current literature. The origin of thymomas in this unusual location remains unknown. In this case, immunohistochemical analysis demonstrated that the epithelial component was strongly positive for cytokeratin and focally reactive for epithelial membrane antigen. It is concluded that PIT should be considered when the histopathological appearance of a lung tumour is not typical of other pulmonary neoplasms. Complete resection appears sufficient in non-malignant tumours, while in cases of partial resection or malignancy, adjuvant radiotherapy should be considered. Long-term regular clinical follow-up is also warranted, due to the risk of late local recurrence.

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