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Query: UMLS:C0027960 (mole)
 21,279 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Cobb syndrome describes the association of a spinal angioma and a cutaneous angioma such as nevus flammeus or angiokeratoma of the corresponding dermatome. A 23-year-old Korean female showed spiral cord angiomas and segmentally distributed nevus flammeus of corresponding dermatomes (Th5-Th2). Interestingly she had a lymphangioma circumscriptum in the same dermatomes. To the best of our knowledge, there is no report of this constellation. Therefore, we suggest that lymphangioma circumscriptum may be considered as a skin finding of Cobb syndrome.
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PMID:A case of Cobb syndrome associated with lymphangioma circumscriptum. 886 18

A case of phakomatosis pigmentovascularis type IIa in a, short stature, 6-year old girl with nevus flammeus of the face and palate, bilateral ocular melanosis, aberrant mongolian spots and minor dysmorphic traits is reported. The patient has been considered at risk for glaucoma and ocular melanoma and an ophthalmologic follow-up including a tonometry is yearly requested. Phakomatosis Pigmentovascularis (PP) is a rare disease characterized by coexistence of capillary hemangioma and pigment abnormalities. The authors review the literature and discuss clinical features which actually allow the distinction of PP into 4 types. The opportunity of a follow-up specifically planned on the patient's phenotype is emphasized.
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PMID:[Phakomatosis pigmentovascularis ]. 892 60

Encephalotrigeminal angiomatosis (Sturge-Weber syndrome) is a rather uncommon congenital condition characterized by the combination of venous angioma of the leptomeninges over the cerebral cortex with ipsilateral angiomatous lesions of the face and sometimes, the skull, jaws and oral soft tissues. Two patients came to the Kaohsiung Medical College Hospital's dental clinic with complaints of localized gingival enlargement or tumor-like swelling. Based on the presence of facial nevus flammeus, examinations of angiography, radiological evidence of calcific densities, and ipsilaterally intraoral vascular hyperplasia in the lip, cheek and gingiva, encephalotrigeminal angiomatosis was diagnosed. Dental management included plaque control instructions, scaling, root planing and periodontal surgery. Recurrence of gingival enlargement in both cases was noted, so periodontal surgery was performed a second time. Close follow up and complete plaque control have kept the periodontal condition fairly well under control in these two cases. We introduce the oral manifestations and the experience of treatments in these two cases.
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PMID:Periodontal manifestations and treatment of Sturge-Weber syndrome--report of two cases. 909 52

Classification of cutaneous vascular anomalies is difficult because conceptual confusion persists between vascular neoplasms and malformations. However, hemangiomas of the infancy fulfill criteria both for hyperplasia and neoplasm because they result from proliferation of endothelial cells, but often undergo complete regression. Despite these pitfalls we have classified cutaneous vascular anomalies into the following categories: hamartomas, malformations, dilatations of preexisting vessels, hyperplasias, benign neoplasms, and malignant neoplasms. In this first part of our clinicopathologic review of vascular anomalies, hamartomas, malformations, and dilatation of preexisting vessels are covered. Hamartomas include several combined vascular and melanocytic proliferations grouped as phakomatosis pigmentovascularis and the so-called eccrine angiomatous hamartoma that consists of proliferations of both eccrine glands and blood vessels. Vascular malformations result from anomalies of embryologic development, and in some of them the abnormalities of the involved vessels are more functional than anatomic, as is the case of nevus anemicus. In contrast, other cutaneous vascular malformations show striking morphologic abnormalities of the vascular structures. These anatomic vascular malformations are subdivided into the following groups: capillary, venous, arterial, lymphatic, and combined anomalies. Spider angioma, capillary aneurysm-venous lake, and telangiectases are not vascular proliferations at all, but dilations of preexisting vessels. In our opinion, most of the lesions described with the generic term of "angiokeratoma" are not authentic vascular neoplasms, but hyperkeratotic malformations of capillaries and venules or acquired telangiectases of preexisting blood vessels of the papillary dermis. Therefore the first group of these "angiokeratomas" are included in the vascular malformations section, and the second group are covered in the section of dilation of preexisting vessels. Lymphangiectases are considered the lymphatic counterpart of angiokeratomas because they result from ectasia of preexisting lymphatic vessels of the papillary dermis.
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PMID:Cutaneous vascular anomalies. Part I. Hamartomas, malformations, and dilation of preexisting vessels. 934 91

We present two patients with unilateral occipital gyriform calcification and seizures. Gyriform or serpentine calcification as revealed by computed tomography (CT) scan is rare and is a characteristic finding of Sturge-Weber syndrome (SWS) and celiac disease (CD). These patients had neither the facial nevus flammeus or neurological deficits characteristic of SWS, nor the gastrointestinal symptoms characteristic of CD. CD is often accompanied by cerebral occipital calcification indistinguishable from that of SWS. We demonstrate the presence of cerebral leptomeningeal angiomatosis (LA) by Gadolinium-DTPA-enhanced magnetic resonance imaging (MRI) but could not detect LA by either CT scanning or angiography. It has been reported that contrast-enhanced MRI is useful to detect LA in SWS. However, we found no reports of enhanced MRI in patients with SWS without facial angioma. If future studies can demonstrate the absence of cortical enhancement by contrast-enhanced MRI in CD with cerebral calcifications, enhanced MRI would become an important tool for differentiating CD from SWS.
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PMID:Enhanced magnetic resonance imaging of leptomeningeal angiomatosis. 939 Jul 6

Choroidal osteoma is a benign, ossifying tumor, first described by Gass and Williams in 1978, typically found in healthy young females. Usually it is localized in the posterior pole of the eye, near the optic disc and the macula. Its aetiology is unknown: it may be caused by osseous metaplasia of the retinal pigment epithelium, or it may represent a kind of choristoma. The diagnosis of the choroidal osteoma is based mainly on its very characteristic clinical picture, ultrasonography and computed tomography--both demonstrating calcific plaque corresponding to the lesion. The most important complications of the tumor are: subretinal neovascularisation, subretinal and intraretinal haemorrhages, serous and haemorrhagic retinal detachments. Neovascularisation can be successfully treated by laser photocoagulation, which can even lead to decalcification and involution of the tumor. Choroidal osteoma must be differentiated mainly from amelanotic choroidal melanoma, choroidal nevus, choroidal haemangioma, choroidal metastasis, granuloma, organized subretinal haemorrhage, sclerochoroidal calcification and age-related macular degeneration. In this paper we present a case of a choroidal osteoma recognized in an 11-year-old girl. There have been no signs of tumor's growth or signs of subretinal neovascularisation for three years of follow-up. As far as we know, it is the first case of this disease described in Polish literature.
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PMID:[Choroidal osteoma]. 968 99

One hundred and three cases of Spitz nevi were reviewed, 36% of these patients were adults. The lesions in children occurred primarily on the face and secondarily on the trunk followed by the extremities. In adults, they affect the legs in women, and the trunk in men as does malignant melanoma. Clinically they were diagnosed more frequently as Spitz nevi in children and adolescents than in adults. Spitz nevi were most commonly mistaken clinically for "common" melanocytic nevus, hemangioma, verruca, fibrous histiocytoma, molluscum contagiosum, granuloma pyogenicum, keloid, and melanoma. Histologically, there are various expressions of Spitz nevi, but they are usually compound melanocytic nevi with little pigmentation and typically large spindle- and/or epithelioid melanocytes. There are only small histopathological differences between Spitz nevi in childhood and adulthood: one important feature rarely seen in Spitz nevi of adults is multiple mitotic figures at the dermo-epidermal junction but rarely at the base of the melanocytic nevus. Nevus Reed is considered to possibly be a distinctly pigmented variant of Spitz nevus.
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PMID:[Spitz nevus and Reed nevus: simulating melanoma in adults]. 988 3

A 71-year-old male who had previously suffered from chronic alcoholism was admitted to the Internal Medicine Service of Coimbra Hospital Center in January 1996 due to asthenia, loss of weight, icterus and abdominal pain, clinical features that had begun six months before admittance to hospital. A physical examination revealed that, in addition to icterus, the patient presented multiple hemangiomas of 1 to 5 cm in diameter, located in the oral cavity, neck, breast and left axilla. These lesions were bluish, elevated and with a rubber-nipple consistency, and had been developing for about 15 years. Subsequent examination revealed normocytic normochromic anemia, cholestatic icterus and the existence of a gastrointestinal hemangioma located in the esophagus. Excisional biopsy of an element proved that it was cavernous hemangioma. A subsequent angio-scintigraphy indicated other aspects suggestive of deep hemangiomas located in the legs, face and cervical region. The authors had the opportunity of examining other members of the patient's family, who apparently did not exhibit similar lesions. They concluded that it was a case of blue rubber bleb nevus syndrome (BRBNS), probably in its sporadic form. Treatment was essentially conservative and the patient is well.
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PMID:[The blue rubber bleb nevus syndrome or Bean's syndrome. A rare cause of digestive hemorrhage]. 1019 91

Between May 1995 and March 1996 we were able to diagnose and remove glomus tumors of the left nasal cavity from three female patients. Ages of the patients were 9, 36 and 74 years. All patients suffered from a frequent epistaxis and all were extremely sensitive to the slightest nasal touch. One patient reported breathing difficulties due to nasal congestion. Examination revealed a tumor that filled the entire nasal cavity. The method of choice in treatment of these tumors is surgical removal. Hemangiopericytoma, non-chromaffin paraganglioma, hidradenoma, cavernous hemangioma and nevus cell nevi have to be excluded by histology and immunohistochemical techniques. From a clinical perspective the bleeding septal polyp (granuloma telangiectaticum sive pyogenicum sive pediculatum) has to be considered because it often comes from Kieselbachi's plexus, has a mushroom-like appearance and bleeds slightly.
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PMID:[Glomus tumor of the nose]. 1019 80

Hobnail hemangioma, also known as "targetoid hemosiderotic hemangioma", represents a distinctive, benign vascular tumor, characterized histologically by a biphasic growth pattern of dilated vascular structures in the superficial dermis lined by prominent hobnail endothelial cells, and collagen dissecting, rather narrow neoplastic vessels in deeper parts of the lesion. We analyzed the clinicopathologic and immunohistochemical features in a series of 62 cases. Patient age range was 6-72 years (median: 32 years); 34 patients were male and 25 female. Clinically, a broad variation of diagnoses ranging from hemangioma to dermal melanocytic nevus and fibrous histiocytoma was suggested. Nineteen tumors arose in the lower and 13 in the upper extremities, 12 on the back, 8 in the buttock and hip region, and one case on the chest wall. Follow-up information on 35 patients (range from 1 to 4 years; mean: 1.5 years) revealed no local recurrence nor systemic metastasis. All neoplasms were located in the dermis and showed a broad morphologic spectrum in dependence of the age of the lesions. In addition to lesions resembling cavernous lymphangioma or lymphangioma circumscriptum, neoplasms were seen with morphologic features reminiscent to retiform hemangioendothelioma, progressive lymphangioma and so-called Dabska's tumor. Immunohistochemistry performed in 28 cases showed positive staining of tumor cells for CD31 in all cases tested, whereas only 3 out of 28 cases stained completely positive for CD34. In addition 4 out of 8 cases stained positively for vascular endothelial growth factor receptor-3 (VEGFR-3). Neoplastic endothelial cells were surrounded by actin-positive pericytes in only 7 out of 27 cases tested. Hobnail hemangioma occurs more frequently in male patients and arises commonly in the extremities and the trunk. Histologic and immunohistochemcial features suggest a lymphatic line of differentiation for this distinctive vascular neoplasm.
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PMID:Hobnail hemangioma ("targetoid hemosiderotic hemangioma"): clinicopathologic and immunohistochemical analysis of 62 cases. 1047 56


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