Developmental venous anomalies and sinus pericranii in the blue rubber-bleb nevus syndrome

2003 ◽  
Vol 99 (2) ◽  
pp. 409-411 ◽  
Author(s):  
Patrik Gabikian ◽  
Richard E. Clatterbuck ◽  
Philippe Gailloud ◽  
Danielle Rigamonti

✓ Blue rubber-bleb nevus syndrome (BRBNS) is a developmental disorder that originally was identified by the presence of distinctive cutaneous and gastrointestinal hemangiomas. More recently it has been recognized that the number of affected organs is larger and that BRBNS includes central nervous system vascular malformations. A 52-year-old woman in whom intracranial vascular malformations had been diagnosed earlier presented for evaluation. At birth, several blue nevi had been noted on her tongue, lips, and neck. Cerebral angiography demonstrated an extensive network of developmental venous anomalies and a left anterior sinus pericranii. The literature on BRBNS and developmental venous anomalies is reviewed.

2003 ◽  
Vol 9 (2) ◽  
pp. 169-176 ◽  
Author(s):  
J.I. Chung ◽  
H. Alvarez ◽  
P. Lasjaunias

We report a sporadic case of probable BRBN (blue rubber bleb nevus syndrome) with multiple CNS (central nervous system) involvement. These features consisted of multiple VMs (venous malformations) and DVAs (developmental venous anomalies) in supratentorial brain, cerebellum, and diencephalon. Since its first description by Bean, there have been many cases of BRBN manifesting with gastrointestinal bleeding with or without associated hemorrhage. Cases with CNS involvement were rarely reported and many of the descriptions were confusing with different terminologies used to describe them such as capillary venous malformation, hemangiomas, and vascular malformations. The lesions illustrated are venous malformations similar to our case. The association of DVA was recognized in some cases; they are likely to be underestimated when revisiting the published case illustrations. Although our case is sporadic, the link with HHT1 is unlikely despite the involvement of the same chromosome (Ch 9).


1982 ◽  
Vol 56 (4) ◽  
pp. 555-564 ◽  
Author(s):  
Wen-qing Huang ◽  
Shi-ju Zheng ◽  
Qing-sheng Tian ◽  
Jian-qing Huang ◽  
Yu-xia Li ◽  
...  

✓ The authors present a statistical survey of the general incidence, age distribution, and preferential sites of 25,122 tumors of the central nervous system (CNS), from 12 centers in China. Of these tumors, 22,457 were intracranial and the rest intraspinal. Of the 22,457 intracranial neoplasms collected, tumors of neuroepithelial tissue comprised 43.85%, meningiomas 16.58%, tumors of nerve sheath cells 9.5%, pituitary adenoma 9.52%, congenital tumors 8.46%, secondary tumors 6.8%, vascular malformations and tumors 3.82%, and primary sarcomas 0.72%. Neuroepithelial and meningeal tumors occurred first and second in all series, but the other tumors varied in frequency. There was a higher incidence of nerve-sheath tumors in southern than in northern regions. The age distribution of Chinese patients with tumors of the CNS was lower than that of Caucasians: nearly two-thirds (64.57%) had the clinical onset of their tumor between the ages of 31 and 40 years, with the peak incidence at 35 years. Nearly 20% of tumors of the CNS occurred before 20 years of age. The male:female ratio was 1.53:1; the only tumor with a definite preponderance of females over males was the meningioma. Intraspinal tumors derived from nerve sheaths comprised 47.13% of all tumors within the spinal canal. Meningiomas were second with an incidence of 14.06%, then followed congenital tumors (12.06%) and neoplasms of neuroepithelial tissue (10.83%). Secondary tumors, vascular malformations and neoplasms, and sarcoma were next in order of frequency with 4.6%, 4.5%, and 4.16%, respectively.


1998 ◽  
Vol 89 (5) ◽  
pp. 728-737 ◽  
Author(s):  
Yutaka Sawamura ◽  
Tsutomu Kato ◽  
Jun Ikeda ◽  
Jun-ichi Murata ◽  
Mitsuhiro Tada ◽  
...  

Object. The optimum clinical management of central nervous system (CNS) teratomas, particularly postsurgical adjuvant therapy, is still unclear, partly as a result of the tumors' low incidence. In this study the authors analyze 34 cases of CNS teratomas so that they may adequately indicate management of these lesions. Methods. The median age of the 34 patients was 13 years. Twenty-seven patients treated between 1970 and 1991 were retrospectively reviewed. Four of these 27 patients died as a result of radical surgery; each of them had a teratoma involving the hypothalamus. After initial treatment, which included radiation therapy, 20 patients (48%) had died. In all seven cases of mature teratomas there was no recurrence. In two cases of immature teratomas in which there was complete surgical resection there was recurrence; however, salvage therapies were effective. Seven of eight patients with highly malignant teratomas died; for these patients salvage therapies, including repeated radiation and chemotherapy, failed. Seven patients who presented with CNS teratomas between 1992 and 1996 received adjuvant chemotherapy and radiation therapy according to a prospective study protocol. All seven patients were free from recurrence with a 70 to 100% Karnofsky Performance Scale score at a median follow-up period of 41 months. Patients with CNS teratomas rarely responded completely to chemotherapy or radiation therapy; an effective adjuvant therapy produced a partial response at best. Conclusions. Because teratomas show various responses to adjuvant therapy, a misdiagnosis of their histological subtype will lead to inadequate therapy. A diverse therapeutic protocol based on histological diagnosis is necessary to plan appropriate management. Treatment recommendations are discussed in detail in the article.


1990 ◽  
Vol 73 (1) ◽  
pp. 147-150 ◽  
Author(s):  
Andrew Mitchell ◽  
Bernd W. Scheithauer ◽  
Patrick J. Kelly ◽  
Glenn S. Forbes ◽  
Jon E. Rosenblatt

✓ The tapeworm Spirometra mansonoides infects man worldwide, particularly in Asian countries. Rarely, the central nervous system is involved; such a case is presented here. In the total of 12 reported cases, including the case described, the worm presented clinically as a mass suspicious for neoplasm or chronic abscess cavity. Surgical removal was invariably curative in each case. Although infrequent, the possibility of tapeworm infection should be entertained in the evaluation of intracranial masses in patients who have visited exotic locales.


1996 ◽  
Vol 84 (6) ◽  
pp. 1060-1061 ◽  
Author(s):  
Akira Miyauchi ◽  
Katsumi Matsumoto ◽  
Eiji Kohmura ◽  
Teruo Doi ◽  
Kazuhiko Hashimoto ◽  
...  

✓ Primary central nervous system germinoma usually presents as an extraaxial intracerebral mass. The authors report the rare occurrence of an intramedullary spinal cord germinoma at the conus medullaris in a 24-year-old man, which was treated by partial removal and radiation therapy. The tumor was highly radiosensitive and the patient remains disease free 15 months posttreatment.


1984 ◽  
Vol 61 (2) ◽  
pp. 375-381 ◽  
Author(s):  
Griffith R. Harsh ◽  
Charles B. Wilson

✓ Local recurrence developed 6 years after the initial resection of an intraspinal meningeal tumor that originally was thought to be an angioblastic meningioma. Histological review of the pathology led to a change of that diagnosis to one of mesenchymal chondrosarcoma. The recurrent vascular tumor was embolized, then totally excised. Because this tumor had malignant features, the patient received irradiation and chemotherapy. No evidence of regrowth has been observed during a period of more than 4 years. Mesenchymal chondrosarcomas of the central nervous system and their treatment are reviewed.


1977 ◽  
Vol 47 (1) ◽  
pp. 12-18 ◽  
Author(s):  
Beniamino Guidetti ◽  
Franco M. Gagliardi

✓ The authors report on the clinical data, operating technique, postoperative complications, and late results in a series of 31 epidermoid and 21 dermoid cysts of the central nervous system.


2002 ◽  
Vol 96 (2) ◽  
pp. 197-205 ◽  
Author(s):  
Allan D. O. Levi ◽  
Hector Dancausse ◽  
Xiuming Li ◽  
Suzanne Duncan ◽  
Laura Horkey ◽  
...  

Object. Partial restoration of hindlimb function in adult rats following spinal cord injury (SCI) has been demonstrated using a variety of transplantation techniques. The purpose of the present study was twofold: 1) to determine whether strategies designed to promote regeneration in the rat can yield similar results in the primate; and 2) to establish whether central nervous system (CNS) regeneration will influence voluntary grasping and locomotor function in the nonhuman primate. Methods. Ten cynomologus monkeys underwent T-11 laminectomy and resection of a 1-cm length of hemispinal cord. Five monkeys received six intercostal nerve autografts and fibrin glue containing acidic fibroblast growth factor (2.1 µg/ml) whereas controls underwent the identical laminectomy procedure but did not receive the nerve grafts. At 4 months postgrafting, the spinal cord—graft site was sectioned and immunostained for peripheral myelin proteins, biotinylated dextran amine, and tyrosine hydroxylase, whereas the midpoint of the graft was analyzed histologically for the total number of myelinated axons within and around the grafts. The animals underwent pre- and postoperative testing for changes in voluntary hindlimb grasping and gait. Conclusions. 1) A reproducible model of SCI in the primate was developed. 2) Spontaneous recovery of the ipsilateral hindlimb function occurred in both graft- and nongraft—treated monkeys over time without evidence of recovering the ability for voluntary tasks. 3) Regeneration of the CNS from proximal spinal axons into the peripheral nerve grafts was observed; however, the grafts did not promote regeneration beyond the lesion site. 4) The grafts significantly enhanced (p < 0.0001) the regeneration of myelinated axons into the region of the hemisected spinal cord compared with the nongrafted animals.


1982 ◽  
Vol 56 (3) ◽  
pp. 430-434 ◽  
Author(s):  
Edwin G. Fischer ◽  
Ana Sotrel ◽  
Keasley Welch

✓ Two patients are reported who had intracerebral mass lesions composed of hemangioma and glial neoplasm. After excision, one recurred as an oligodendroglioma, and the remnant of the other remained static over a 5-year period. These lesions may represent a subgroup of cerebral hemangiomas that have the biological potential for future glial neoplastic growth. Reference is made to experimental work with polyoma virus which can induce cavernous hemangiomas in the central nervous system in mice, and which is a papovavirus. Other papovaviruses can induce ependymomas in hamsters.


1981 ◽  
Vol 55 (4) ◽  
pp. 615-619 ◽  
Author(s):  
Neil A. Martin ◽  
Martha Hales ◽  
Charles B. Wilson

✓ A 31-year-old woman developed a cerebellar metastasis from an invasive prolactin-secreting pituitary adenoma while undergoing treatment with bromocriptine. The metastatic tumor was totally excised. Metastatic spread of pituitary tumors within the central nervous system is reviewed briefly.


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