2000
DOI: 10.1080/13577140500353743
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Angiosarcoma With Malignant Peripheral Nerve Sheath TumourDeveloping in a Patient With Klippel–Trénaunay–Weber Syndrome

Abstract: Purpose: We discuss the coexistence of Klippel-Trénaunay-Weber syndrome with various malignancies, the possible histogenetic pathways and therapeutic implications. Patient: We report on a 46-year-old man presenting with increasing pain and swelling of his right lower leg after fracturing his fibula. Since birth he was known as having the uncommon syndrome of Klippel-Trénaunay-Weber of his right lower leg. Methods: Our patient underwent an above-knee amputation for biopsy-proven malignant vascular tumour, first… Show more

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Cited by 20 publications
(15 citation statements)
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“…Other known risk factors include UV irradiation, given the typical locations of cutaneous angiosarcomas on the head and neck (10)(11)(12) , as well as occupational exposure to vinyl chloride (13) , arsenicals, and use of anabolic steroids (14) . Genetically, angiosarcoma is associated with familial syndromes including Li-Fraumeni syndrome ( TP53 mutations) (15) and Klippel-Trenaunay syndrome ( PIK3CA mutations) (16) . However, these syndromes do not solely cause angiosarcoma (7) , and are not present in the majority of cases.…”
Section: Introductionmentioning
confidence: 99%
“…Other known risk factors include UV irradiation, given the typical locations of cutaneous angiosarcomas on the head and neck (10)(11)(12) , as well as occupational exposure to vinyl chloride (13) , arsenicals, and use of anabolic steroids (14) . Genetically, angiosarcoma is associated with familial syndromes including Li-Fraumeni syndrome ( TP53 mutations) (15) and Klippel-Trenaunay syndrome ( PIK3CA mutations) (16) . However, these syndromes do not solely cause angiosarcoma (7) , and are not present in the majority of cases.…”
Section: Introductionmentioning
confidence: 99%
“…Our patient developed a rapidly enlarging osseous mass with nonaggressive periosteal new bone formation, thus not implying malignancy. However, the true nature of the lesion remained unknown before final excision, as there are reports on tumors detected in patients with KTS, including malignant peripheral nerve sheath tumors, angiosarcomas, astrocytomas, hemangiopericytomas, hemangiomas, and meningiomas [23][24][25][26][27][28]. Moreover, isolated hemihypertrophy, a major clinical manifestation of KTS, is a potential risk factor for developing neoplasms, although the risk of embryonal cancer is reportedly not higher in children with KTS [29][30][31].…”
Section: Discussionmentioning
confidence: 99%
“…The classic presentation of primary pulmonary angiosarcoma is a single lesion spreading locally as a multicentric mass, which presents a diagnostic challenge by imaging alone . Rare reports have described cases of synchronous primary pulmonary angiosarcomas and angiosarcomas of extrapulmonary sites in patients with Maffucci and Klippel‐Trenaunay‐Weber syndromes . In addition, the differential diagnosis of primary lung angiosarcoma includes Kaposi sarcoma and epithelioid hemangioendothelioma.…”
Section: Discussionmentioning
confidence: 99%
“…Mentzel Trenaunay-Weber syndromes. 16,17 In addition, the differential diagnosis of primary lung angiosarcoma includes Kaposi sarcoma and epithelioid hemangioendothelioma. These can be differentiated through immunohistochemistry for HHV-8 and morphology.…”
Section: Discussionmentioning
confidence: 99%