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Article Synopsis
  • Composite haemangioendothelioma is a rare vascular tumor that can exhibit low to medium malignant potential and is diagnosed by identifying different vascular components through histopathology.
  • A case study describes a 49-year-old man with chronic lymphoedema who developed this tumor, which showed high-grade angiosarcoma-like features but behaved less aggressively, confusing it with the more severe Stewart-Treves syndrome.
  • Despite needing potentially extensive surgery (hemipelvectomy), the patient opted against it and, after two years of monitoring, experienced no disease progression, indicating the need for accurate diagnosis to avoid unnecessary procedures.
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Aims: To demonstrate clinicopathologic features of Stewart-Treves syndrome (STS) including clinical manifestations, morphology, immunophenotype (especially c-MYC amplification), differential diagnosis, pathogenesis, treatment and prognosis.

Methods And Results: 17 cases of STS were retrospectively archived, involving 6 cases of postmastectomy, 3 cases of postoperative cervical cancer and 8 cases of chronic lymphatic obstruction without history of malignancy. Seven of 9 cancer patients had undergone radiotherapy.

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We herein report the case of a 3-year-old girl with atypical congenital right upper limb lymphedema who developed an angiosarcoma. Only a few cases have been reported following congenital form of lymphedema and only 4 in such a young child. We also summarize all cases of angiosarcoma associated with congenital lymphedema reported in the literature.

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Lymphangioma circumscriptum is a superficially localized variant of lymphangioma. The characteristic clinical presentation is a "frogspawn" grouping of vesicles or papulovesicles on the proximal limb or limb girdle areas. Though most lymphangiomas develop congenitally, the lymphangioma circumscriptum subtype is known to present in adults.

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