Soft tissue angiosarcoma of the breast in a patient with Klippel-Trenaunay-Weber syndrome

Klippel-Trenaunay syndrome is a rare congenital vascular disease. The pathogenesis of this syndrome is unclear, but it is thought that most cases are the result of somatic mutations that affect genes that play a role in vasculogenesis and angiogenesis. Some patients come with a triad of capillary malformation (hemangioma or port-wine stain), venous varicosities and bony or soft tissue hypertrophy. Clinical presentation of this syndrome can lead to significant morbidities and mortalities due to severe bleeding and emboli. Although the number of cases is low, a doctor must be able to distinguish Klippel-Trenaunay Syndrome from other rare vascular disorders. Parkes Weber syndrome is usually similar to Klippel-Trenaunay syndrome, except in the arterial malformations associated with capillary malformations and soft tissue to skeletal or bone hypertrophy. The diagnosis of Klippel-Trenaunay Syndrome is carried out clinically and is quite difficult to do even with experienced doctors because there is no precise pathognomonic test. There are several options in relation to the management of Klippel-Trenaunay Syndrome and non-invasive procedure is considered to be the most important of therapy modalities. Early diagnoses, progression monitoring, and proper intervention should be carried out for better prognosis and preventing complication.

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Pulmonary thromboembolism in Klippel–Trenaunay–Weber syndrome – should all cases be anticoagulated?

Phlebology The Journal of Venous Disease ◽

10.1258/026835505774964865 ◽

2005 ◽

Vol 20 (4) ◽

pp. 193-195

Author(s):

N B Bagul ◽

R Pathak ◽

N K Garg ◽

A Platts ◽

D M Baker

Keyword(s):

Pulmonary Embolism ◽

Soft Tissue ◽

Pulmonary Thromboembolism ◽

Varicose Veins ◽

Venous Malformation ◽

Congenital Disorder ◽

Superficial Thrombophlebitis ◽

Weber Syndrome ◽

Cutaneous Haemangioma

Klippel–Trenaunay–Weber syndrome (KTWS) is a congenital disorder characterized by a triad of varicose veins and venous malformation involving one or more extremities, cutaneous haemangioma, and hypertrophy of soft tissue and bone. The syndrome is rare and it usually presents as varicose veins or cutaneous naevi. We report a patient with KTWS who developed superficial thrombophlebitis in her varicose veins and died from a pulmonary embolism. To optimize management it is important to recognize the syndrome, and the patient should ideally be referred for multidisciplinary vascular care and all such patients should be anticoagulated in the long term.

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Sturge Weber Syndrome Overlapping with Klippel Trenaunay Syndrome

Journal of Nepal Paediatric Society ◽

10.3126/jnps.v33i2.7704 ◽

2013 ◽

Vol 33 (2) ◽

pp. 147-149

Author(s):

Shatanik Sarkar ◽

Chaitali Patra ◽

Chandrasekhar Dey ◽

Malay Kumar Dasgupta ◽

Tapan Kumar Kundu

Keyword(s):

Soft Tissue ◽

Varicose Veins ◽

Cerebral Calcification ◽

Rare Type ◽

Weber Syndrome ◽

Sturge Weber Syndrome ◽

Klippel Trenaunay Syndrome

Sturge Weber syndrome (SWS) is a mesodermal phakomatosis characterized by meningo-facial angiomas with cerebral calcification. Klippel Trenaunay syndrome (KTS) is another very rare type of phakomatosis with cutaneous angiomas, varicose veins and enlargement of soft tissue or bones. Overlap between SWS & KTS is very rarely encountered. We report a three and half year old girl with overlapping features of both SWS and KTS. DOI: http://dx.doi.org/10.3126/jnps.v33i2.7704 J Nepal Paediatr Soc. 2013; 33(2):147-149

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TROMBOEMBOLISMO PULMONAR EM PACIENTE COM SÍNDROME DE KLIPPEL-TRENAUNAY-WEBER: RELATO DE CASO

Revista de Patologia do Tocantins ◽

10.20873/uft.2446-6492.2018v5n2p55 ◽

2018 ◽

Vol 5 (2) ◽

pp. 55-57

Author(s):

Luis Arthur Brasil Gadelha Farias ◽

Mariana Fátima Cabral De Oliveira ◽

Maycon Fellipe Da Ponte ◽

Aglaerton Silva Pinheiro

Keyword(s):

Pulmonary Embolism ◽

Venous Thromboembolism ◽

Soft Tissue ◽

Rare Disease ◽

Previous History ◽

Lymphatic Malformation ◽

Vascular Disorder ◽

Ultrasonographic Diagnosis ◽

Weber Syndrome ◽

History Of

RESUMO A síndrome de Klippel-Trenaunay-Weber (KTW) é uma doença rara caracterizada por distúrbio vascular com malformação capilar, venosa e linfática, grandes hemangiomas e hipertrofia óssea ou de tecidos moles. Relatamos o caso de uma mulher de 24 anos de idade, obesa, com história prévia de linfedema e hemangioma unilateral da perna direita que evoluiu com tromboembolismo pulmonar. Ela possuia diagnóstico clínico e ultrassonográfico de síndrome de Klippel-Trenaunay-Weber há 5 anos. Após a anticoagulação, a paciente evoluiu assintomática e sem novos eventos tromboembólicos. Palavras-chave: Síndrome de Klippel-Trenaunay-Weber; Embolia Pulmonar; Tromboembolia; Tromboembolia Venosa. ABSTRACT Klippel-Trenaunay-Weber syndrome (KTW) is a rare disease characterized by vascular disorder with capillary, venous and lymphatic malformation, large hemangiomas and bone or soft tissue hypertrophy. Herein we report the case of a 24-years-old women, obese, with previous history of unilateral lymphedema and hemangioma in right leg witch evolves to pulmonar tromboembolism. She had clinical and ultrasonographic diagnosis of Klippel-Trenaunay-Weber Syndrome five years before. After anticoagulation, the patient evolves assymptomatic and without new tromboembolic events. Keywords: Klippel-Trenaunay-Weber Syndrome; Pulmonary Embolism; Thromboembolism; Venous Thromboembolism.

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Klippel-Trenaunay-Weber Syndrome: A Case Report

Bangladesh Journal of Child Health ◽

10.3329/bjch.v37i2.17271 ◽

2013 ◽

Vol 37 (2) ◽

pp. 130-132

Author(s):

Tahsinul Amin ◽

Syed Zahid Hossain ◽

Asim Kumar Saha ◽

Saiful Hasan ◽

M Faizul Islam

Keyword(s):

Soft Tissue ◽

Varicose Veins ◽

Lower Limbs ◽

Port Wine Stain ◽

Port Wine ◽

Internal Organs ◽

Port Wine Stains ◽

Weber Syndrome ◽

Congenital Condition ◽

Male Neonate

Klippel-Trenaunay-weber syndrome is a rare congenital condition usually presenting with port wine stains, excessive growth of bones and soft tissue, and varicose veins. This occurs most commonly in the legs, but it also may affect the arms, face, head, or internal organs. We report a term male neonate presented with port wine stain, varicose veins and excessive growth of soft tissue of lower limbs consistent with Klippel-Trenaunay-Weber syndrome. DOI: http://dx.doi.org/10.3329/bjch.v37i2.17271 BANGLADESH J CHILD HEALTH 2013; VOL 37 (2) : 130-132

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Localization of Gallium-67 in Peritoneal Cells by Electron Microscopic Autoradiography

Proceedings, annual meeting, Electron Microscopy Society of America ◽

10.1017/s0424820100072460 ◽

1973 ◽

Vol 31 ◽

pp. 404-405

Author(s):

D. C. Swartzendruber ◽

Norma L. Idoyaga-Vargas

Keyword(s):

Clinical Trials ◽

Soft Tissue ◽

Tumor Tissue ◽

Soft Tissue Tumors ◽

Clinical Usefulness ◽

Electron Microscopic ◽

Normal Cells ◽

Electron Microscopic Autoradiography ◽

Peritoneal Cells ◽

Gallium 67

The radionuclide gallium-67 (67Ga) localizes preferentially but not specifically in many human and experimental soft-tissue tumors. Because of this localization, 67Ga is used in clinical trials to detect humar. cancers by external scintiscanning methods. However, the fact that 67Ga does not localize specifically in tumors requires for its eventual clinical usefulness a fuller understanding of the mechanisms that control its deposition in both malignant and normal cells. We have previously reported that 67Ga localizes in lysosomal-like bodies, notably, although not exclusively, in macrophages of the spocytaneous AKR thymoma. Further studies on the uptake of 67Ga by macrophages are needed to determine whether there are factors related to malignancy that might alter the localization of 67Ga in these cells and thus provide clues to discovering the mechanism of 67Ga localization in tumor tissue.

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Morphological Examination of a Herpes-type Virus Indigenous for Tree Shrews

Proceedings, annual meeting, Electron Microscopy Society of America ◽

10.1017/s0424820100067790 ◽

1970 ◽

Vol 28 ◽

pp. 160-161

Author(s):

J. P. Brunschwig ◽

R. M. McCombs ◽

R. Mirkovic ◽

M. Benyesh-Melnick

Keyword(s):

Electron Microscopy ◽

Soft Tissue ◽

Chick Embryo ◽

Soft Tissue Sarcoma ◽

Cell Cultures ◽

Tree Shrew ◽

Type Virus ◽

Morphological Examination ◽

Tree Shrews ◽

Embryo Cells

A new virus, established as a member of the herpesvirus group by electron microscopy, was isolated from spontaneously degenerating cell cultures derived from the kidneys and lungs of two normal tree shrews. The virus was found to replicate best in cells derived from the homologous species. The cells used were a tree shrew cell line, T-23, which was derived from a spontaneous soft tissue sarcoma. The virus did not multiply or did so poorly for a limited number of passages in human, monkey, rodent, rabbit or chick embryo cells. In the T-23 cells, the virus behaved as members of the subgroup B of herpesvirus, in that the virus remained primarily cell associated.

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