Neurocutaneous vascular hamartomas mimicking Cobb syndrome

2000 ◽  
Vol 93 (1) ◽  
pp. 133-136 ◽  
Author(s):  
Yukihiro Wakabayashi ◽  
Mitsuo Isono ◽  
Tsuyoshi Shimomura ◽  
Atushi Tajima ◽  
Hiroto Terashi ◽  
...  

✓ The authors report the rare case of a patient with neurocutaneous vascular hamartomas mimicking Cobb syndrome. An 8-year-old boy was admitted to the authors' hospital with progressive urinary disturbance and upper back pain. Multiple skin nevi had been noted at the child's birth. Radiological examination revealed multiple cavernous angiomas in the spinal cord in the same metamere in which the skin nevi had been observed and also in the left cerebral hemisphere. His symptoms gradually improved without surgical intervention. Four years later he was readmitted because of a cerebral hemorrhage involving the left cerebral peduncle. Nonsurgical treatment was chosen because his symptoms promptly improved. To the best of the authors' knowledge, this is the first case of multiple cavernous angiomas in the brain and spinal cord associated with skin nevi. The authors discuss this clinical entity and the significance of the disease.

1992 ◽  
Vol 77 (2) ◽  
pp. 302-306 ◽  
Author(s):  
Margaret R. Wacker ◽  
Philip H. Cogen ◽  
Joan E. Etzell ◽  
Laleh Daneshvar ◽  
Richard L. Davis ◽  
...  

✓ Gangliogliomas are tumors composed of neuronal and glial elements that typically grow slowly by expansion only. This report describes a 20-month-old girl with a ganglioglioma that extensively involved the subarachnoid space; microscopic foci of tumor were found in the brain and spinal cord. Despite chemotherapy and radiation therapy, the child died 5 months after diagnosis. Molecular genetic analysis showed loss of chromosome 17p DNA sequences in the tumor tissue.


1995 ◽  
Vol 83 (4) ◽  
pp. 724-728 ◽  
Author(s):  
Robert Chen ◽  
David R. Macdonald ◽  
David A. Ramsay

✓ The authors describe a case of a diffuse primary leptomeningeal oligodendroglioma in a 17-year-old girl who presented with raised intracranial pressure and hydrocephalus. She underwent imaging studies and a left frontotemporal craniotomy that revealed a cystic oligodendroglioma in the suprasellar cistern and spread of neoplastic cells to the spinal leptomeninges. The tumor showed little response to maximum radiotherapy and chemotherapy, and the patient died from complications of high-dose chemotherapy 2 years after diagnosis. Postmortem examination of the brain and spinal cord revealed diffuse meningeal infiltration by neoplastic cells and no evidence of an intraparenchymal origin. Glial heterotopias were noted at several sites along the brain base, adding circumstantial support to the theory that leptomeningeal gliomas are derived from ectopic glial tissue in the subarachnoid space.


1972 ◽  
Vol 37 (5) ◽  
pp. 538-542 ◽  
Author(s):  
George J. Dohrmann

✓ Adult dogs were rendered hydrocephalic by the injection of kaolin into the cisterna magna. One group of dogs was sacrificed 1 month after kaolin administration, and ventriculojugular shunts were performed on the other group. Hydrocephalic dogs with shunts were sacrificed 1 day or 1 week after the shunting procedure. All dogs were perfused with formalin at physiological pressure, and the brain stem and cervical spinal cord were examined by light microscopy. Subarachnoid granulomata encompassed the superior cervical spinal cord and dependent surface of the brain stem. Rarefaction of the posterior white columns and clefts or cavities involving the gray matter posterior to the central canal and/or posterior white columns were present in the spinal cords of both hydrocephalic and shunted hydrocephalic dogs. Predominantly in the dogs with shunts, hemorrhages were noted in the spinal cord in association with the clefts or cavities. A mechanism of ischemia followed by reflow of blood is postulated to explain the hemorrhages in the spinal cords of hydrocephalic dogs with shunts.


1972 ◽  
Vol 37 (5) ◽  
pp. 580-590 ◽  
Author(s):  
Enrica Grossi-Paoletti ◽  
Pietro Paoletti ◽  
Stefano Pezzotta ◽  
Davide Schiffer ◽  
Armando Fabiani

✓ Tumors of the nervous system grew in rats treated at birth with ethylnitrosourea through intracerebral or subcutaneous routes and in fetal rats treated through the mother. In 80% to 85% of the rats, single and multiple tumors developed in the brain and spinal cord regardless of the route of administration. Gasserian neurinomas, oligodendrogliomas, and oligogendroglial foci were the most frequent neoplasms. General morphological aspects and frequency of tumor localizations in relation to drug administration route are discussed. Thymidine incorporation into DNA, and RNA/DNA ratio, were evaluated in order to estimate tumor proliferation rate and growth. Desmosterol, a characteristic sterol of brain tumors, was detected in all the tumors. Regions of the brain and spinal cord of treated rats showed the presence of microscopic pretumoral areas (oligodendroglial foci) which incorporated thymidine into DNA in contrast to the brains of control rats.


2010 ◽  
Vol 67 (11) ◽  
Author(s):  
Hiroya Kuwahara ◽  
Yoshimasa Noguchi ◽  
Yukinobu Saito ◽  
Akira Inaba

1991 ◽  
Vol 74 (5) ◽  
pp. 757-762 ◽  
Author(s):  
W. Craig Clark ◽  
F. Curtis Dohan ◽  
Timothy Moss ◽  
John B. Schweitzer

✓ Neoplastic angioendotheliomatosis is a rare disorder usually characterized by primarily cutaneous or neurological symptoms. Approximately 40 cases of malignant angioendotheliomatosis with primary central nervous system (CNS) symptoms have been reported. Some investigators have postulated a hematopoietic origin for this neoplasm. Most of the literature, however, has perpetuated the idea that the often bizarre symptoms seen with this entity result from neoplastic endothelial cell proliferation within the small vessels of affected organs, including the brain and spinal cord. This report describes the immunohistochemical examination and confirmation of the cell of origin of this neoplasm based on five previously unpublished cases of malignant angioendotheliomatosis with primarily CNS symptoms. It includes the first documentation of a T-cell lymphoma presenting as malignant angioendotheliomatosis. All cases include autopsy findings, and in four cases the diagnosis was made postmortem. One case was proven by stereotactic biopsy, but the patient succumbed as a result of severe intracranial bleeding that occurred at the time of biopsy. Tissues were studied with avidin-biotin peroxidase immunohistochemical techniques using a panel of monoclonal antibodies directed against the leukocyte common antigen, LN-1, LN-2, and anti-Factor VIII, and also using Ulex europaeus agglutinin 1. Based on the results obtained, the authors conclude that the proliferative cells seen within the vessel lumina are of lymphocytic origin and agree that the condition should more properly be designated intravascular lymphomatosis. The therapeutic implications of this conclusion point to the possible administration of chemotherapy and radiotherapy in an effort to achieve remissions in an otherwise relentlessly progressive neurological disorder.


1998 ◽  
Vol 88 (1) ◽  
pp. 126-128 ◽  
Author(s):  
Kiyoshi Nagata ◽  
Yuji Nikaido ◽  
Takashi Yuasa ◽  
Kenta Fujimoto ◽  
Yong Jin Kim ◽  
...  

✓ Germinomas occurring in the thalamus and basal ganglia sometimes cause atrophy of the cerebral hemisphere on the affected side. The authors present the case of a 12-year-old girl with a germinoma that developed in the basal frontal lobe and cerebral basal ganglia. Magnetic resonance imaging showed atrophy not only of the cerebrum but also of the brainstem. A T2-weighted image revealed an area of high intensity that proved to be wallerian degeneration extending from the corona radiata and internal capsule to the brainstem. The authors suggest that this pathological change may be involved in the development of the symptoms and hemiatrophy associated with germinomas in this region of the brain.


2004 ◽  
Vol 100 (5) ◽  
pp. 820-824 ◽  
Author(s):  
Paul House ◽  
Karen L. Salzman ◽  
Anne G. Osborn ◽  
Joel D. MacDonald ◽  
Randy L. Jensen ◽  
...  

Object. Dilations of brain perivascular spaces (PVSs), also known as Virchow—Robin spaces, are routinely identified on magnetic resonance imaging studies of the brain and recognized as benign normal variants. Giant dilations occur only rarely and can be easily misdiagnosed as central nervous system tumors. The relevant surgical literature was reviewed to help establish indications for surgical intervention in these typically benign lesions. Methods. Giant dilations of the PVSs in 12 patients who had undergone surgery for several different indications were identified. Both clinical and radiographic presentations of these patients were reviewed along with the surgical procedures. Conclusions. Dilations of the PVSs can become giant lesions that may necessitate surgical intervention to relieve mass effect or hydrocephalus. The relationship of these lesions to neurological symptoms such as tremor and seizures remains unclear.


2004 ◽  
Vol 101 (1) ◽  
pp. 73-81 ◽  
Author(s):  
Masamitsu Abe ◽  
Kazuo Tabuchi ◽  
Shin Tanaka ◽  
Akira Hodozuka ◽  
Katsuzo Kunishio ◽  
...  

Object. Capillary hemangiomas are benign tumors or tumorlike lesions that originate from blood vessels and have rarely been reported to develop in the brain or spinal cord. The authors summarize the clinical and histological features of capillary hemangiomas of the central nervous system (CNS). Methods. The clinical features, imaging characteristics, and outcomes in 10 patients with CNS capillary hemangiomas were reviewed. Histological studies included immunostaining with CD31, α-smooth muscle actin, vascular endothelial growth factor, and Ki-67 antigen. Three patients with lesions in the brain presented with symptoms of increased intracranial pressure or seizures. Seven patients with lesions in the spinal cord presented with progressive sensorimotor disturbances of the lower limbs. Computerized tomography and magnetic resonance imaging demonstrated well-defined, enhancing lesions associated with marked perifocal edema. Angiography demonstrated hypervascular lesions, which have not recurred after resection. In two cases, multiple satellite lesions resolved after the systemic administration of steroid drugs or interferon-α. Histologically, all lesions were consistent with findings of capillary hemangioma of the skin or soft tissues. The CNS lesions differed significantly from other vascular neoplasms, such as hemangioendotheliomas, hemangiopericytomas, and hemangioblastomas. Conclusions. Capillary hemangiomas of the CNS are benign lesions that can be surgically removed and cured without adjuvant therapy.


1971 ◽  
Vol 34 (3) ◽  
pp. 412-416
Author(s):  
Robert H. Wilkins ◽  
Wang-Kuen Wu

✓ Two cases are reported in which spontaneous rupture of a metastatic brain tumor occurred into the ventricular system, leading to the dissemination of the tumor contents in the cerebrospinal fluid. The site of rupture was demonstrated by ventriculography in the first case and by surgical exposure in the second. These cases support the assumption that metastatic neoplasms within the brain may rupture into the ventricular system and spread via the cerebrospinal fluid pathways.


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