Angiosarcoma arising on the scalp in a Korean patient with xeroderma pigmentosum variant type

Angiosarcoma of the Auricle in a Patient with Xeroderma Pigmentosum Variant

“…A similar report from the Republic of Korea documented 11 cases of cutaneous angiosarcoma complicated by XP [9]. To our knowledge, only 3 cases of angiosarcoma in XP-V patients have been reported (Table 2) [9][10][11]. Most cases occurred on the face and head, as is common for angiosarcoma.…”

“…In most cases, the tumor was <5 cm in size. Wide surgical resection was performed, and 1 patient received preoperative radiation therapy [9]. It remains to be clarified whether radiation, like ultraviolet rays in XP, affects DNA damage repair function.…”

“…A previous study has reported a poor 5-year survival rate in angiosarcoma patients (38%) [ 12 ]. The preferred treatment for angiosarcoma in XP patients is mostly wide surgical resection alone; no cases of local recurrence or distant metastasis have been reported [ 9 ]. This may have been due to the brief follow-up period, although in most cases the tumor was <5 cm in size.…”

Lentigo maligna in a patient with xeroderma pigmentosum, variant type: A case report with dermoscopic findings and review of the literature

“…1,2 XP patients usually develop multiple skin tumors including basal cell carcinoma (BCC), squamous cell carcinoma (SCC), malignant melanoma (MM), and angiosarcoma. 3,4 However, the diagnosis of these tumors is often difficult due to severe actinic damage caused by the UV radiation.…”

Variant subtype of xeroderma pigmentosum with multiple basal cell carcinomas diagnosed in a Chinese woman

“…24 So far, XP-V frequently occurs in combination with skin cancers, including BBC, SCC, melanoma, and angiosarcoma. 25 In managing this disease, prevention is critical and early diagnosis and regular follow-ups are key for the health of patients and their families. Genotyping can be determined through genetic testing, and genetic counseling and prenatal diagnoses can be used to reduce XP mutations in following generations.…”