Angiosarcoma complicating xeroderma pigmentosum

Leake, J.; Sheehan, Mary P.; Rampling, D.; Ramani, Pramila; Atherton, D.J.

doi:10.1111/j.1365-2559.1992.tb00370.x

Cited by 37 publications

(24 citation statements)

References 7 publications

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“…28,32,33 We observed that AS rates rose steadily among whites from 1978 through 2004, although they declined among white men in recent years. AS has been reported in association with chronic lymphoedema, 34 vascular malformations, 35 arteriovenous shunts, 36 environmental exposures, 37 xerodermal pigmentosum, 38 and retinoblastoma. 39 There are some possible explanations for the overall increase in incidence rates of AS.…”

Section: Discussionmentioning

confidence: 99%

Cutaneous soft tissue sarcoma incidence patterns in the U.S.

Rouhani

Fletcher

Devesa

et al. 2008

Cancer

307

228

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BACKGROUND.Cutaneous soft tissue sarcomas (CSTS) are a heterogeneous group of mesenchymal neoplasms. To the authors' knowledge, no prior large, population‐based study has focused on CSTS.METHODS.Surveillance, Epidemiology, and End Results (SEER) Program incidence and relative survival rates of CSTS were analyzed according to race, sex, and histologic type using the 2002 criteria of the World Health Organization classification.RESULTS.Among residents of the 13 SEER registries, 12,114 CSTS were diagnosed from 1992 through 2004. Overall age‐adjusted CSTS incidence rates were highest among blacks (30.8 per 1,000,000 person‐years) followed by whites (25 per 1,000,000 person‐years), and American Indians/Alaska Natives (11.2 per 1,000,000 person‐years) and were lowest among Asian/Pacific Islanders (7.7 per 1,000,000 person‐years). Kaposi sarcoma (KS) accounted for 71.1% of cases, and the rates were similarly ranked. Dermatofibrosarcoma protuberans (DFSP) rates also were highest among blacks, whereas leiomyosarcoma (LS) and angiosarcoma (AS) rates were highest among whites. The rate ratio of men to women was 25.5 for KS, 4.7 for malignant fibrous histiocytoma (MFH), 3.7 for LS, 2.0 for AS, and 0.9 for DFSP. The 5‐year relative survival rates were 99% for patients with DFSP, 89% for patients with MFH, 92% for patients with LS, and 45% for patients with AS. KS rates among men in the original 9 SEER registries increased more than 30‐fold during the 1980s before they peaked around 1991 and subsequently declined rapidly because of human immunodeficiency virus‐associated KS and highly active antiretroviral therapy. This KS pattern was evident not only among those ages 20 to 59 years but also among those ages 60 to 69 years. From 1978 through 2004, LS and AS rates among whites increased exponentially.CONCLUSIONS.CSTS rates varied markedly over time and by race, sex, and histologic type, supporting the notion that these histologic variants of CSTS areetiologically distinct. Cancer 2008. © 2008 American Cancer Society.

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Section: Discussionmentioning

confidence: 99%

Cutaneous soft tissue sarcoma incidence patterns in the U.S.

Rouhani

Fletcher

Devesa

et al. 2008

Cancer

307

228

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“…[1] Angiosarcomas are rare malignant vascular tumors that comprise approximately 2% of all soft tissue sarcomas. Fifty percent of the angiosarcomas occur in the head and neck.…”

Section: Discussionmentioning

confidence: 99%

Angiosarcoma of the scalp associated with Xeroderma pigmentosum

Sharma

Deshmukh

Bal

et al. 2012

Indian J Med Paediatr Oncol

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Xeroderma pigmentosum (XP) is a rare autosomal recessive genodermatosis associated with hypersensitivity to ultraviolet light due to defects in Deoxyribonucleic acid (DNA) repair. These patients have more than a 1000-fold increased risk of developing skin cancers. Although multiple cutaneous malignancies are common, the simultaneous occurrence of angiosarcoma and basal cell carcinoma is a rare phenomenon. We report a case of a 25-year-old male with XP with angiosarcoma scalp and basal cell carcinoma of face and occiput and discuss the treatment of this aggressive neoplasm with a review of the literature pertaining to it.

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“…Although cutaneous angiosarcomas unassociated with lymphedema preferentially target the upper part of the face and scalp (areas of high sunlight exposure) the role of ultraviolet light in the development of these tumors is uncertain. Angiosarcoma has previously been reported in five other patients with Xerdoma pigmentosa (XP)-a rare autosomal recessive defect in endonuclease activity that leads to abnormal DNA repair in response to ultraviolet (UV) radiation-induced damage (Table 1) [3][4][5][6][7]. Patients with XP are very prone to developing UV-associated cutaneous malignancies including basal cell carcinomas, squamous cell carcinomas, and melanomas.…”

Section: Discussionmentioning

confidence: 99%

Angiosarcoma Arising from the Tongue of an 11-Year-Old Girl with Xeroderma Pigmentosum

Olson

Püttgen

Westra

2011

Head and Neck Pathol

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Xeroderma pigmentosum (XP) is a rare autosomal recessive defect in DNA endonuclease activity that is associated with the development of cutaneous malignancies, at sun exposed sites, including basal cell carcinoma, squamous cell carcinoma, and melanoma. Squamous cell carcinomas are also known to target the anterior tongue. Patients sometimes develop angiosarcomas, and these invariably arise from sun-exposed skin. A biopsy was taken from a large mass arising in the anterior tongue of an 11-year-old girl with XP and a history of cutaneous basal cell carcinomas. The histopathologic findings demonstrated a high grade epithelioid neoplasm resembling a poorly differentiated squamous cell carcinoma, but the immunohistochemical profile (AE1/AE3 negative, p63 negative, CD31 positive, CD34 positive) established the diagnosis of angiosarcoma. Angiosarcoma is an XP-related tumor that usually arises in sun-exposed skin but can also arise in the oral cavity. For patients with XP who develop epithelioid neoplasms of the oral cavity, epithelioid angiosarcoma should be considered in the differential diagnosis.

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Angiosarcoma complicating xeroderma pigmentosum

Cited by 37 publications

References 7 publications

Cutaneous soft tissue sarcoma incidence patterns in the U.S.

Cutaneous soft tissue sarcoma incidence patterns in the U.S.

Angiosarcoma of the scalp associated with Xeroderma pigmentosum

Angiosarcoma Arising from the Tongue of an 11-Year-Old Girl with Xeroderma Pigmentosum

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