Postmastectomy lymphangiosarcoma.

Wis Med J

Published: October 1967

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Lower Extremity Angiosarcoma: A Life-Threatening Complication of Lymphedema.

Adv Skin Wound Care

May 2024

At Mayo Clinic, Rochester, Minnesota, USA, Vishal Garimella, MBBS, and Nimay Anand, BA, are Visiting Research Fellows; Elliott H. Campbell, MD, is Dermatology Resident Physician; and Christian L. Baum, MD, and Afsaneh Alavi, MD, are Professors of Dermatology. Acknowledgments: Afsaneh Alavi is on the Board of Directors for the Hidradenitis Suppurativa Foundation and served as a consultant for AbbVie, Almirall, Boehringer Ingelheim, InflaRx, Incyte, Kymera, Novartis, and UCB and investigator for Boehringer Ingelheim, and Processa. The authors have disclosed no other financial relationships related to this article. Submitted January 10, 2023; accepted in revised form June 16, 2023.

Article Synopsis
  • * The symptoms of angiosarcoma can vary widely, presenting as anything from bleeding spots to larger masses, which may eventually lead to serious complications like ulceration.
  • * Treatment requires a comprehensive approach involving surgery, radiation, and chemotherapy, and there is currently limited research on lower limb cases, prompting the need for healthcare providers to consider angiosarcoma in patients with lymphedema-related symptoms.
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Article Synopsis
  • * A male patient with a 10-year history of right leg elephantiasis developed large ulceronodular masses in his leg, raising suspicion of cancer, which was confirmed through a biopsy showing angiosarcoma with sickled red blood cells.
  • * The case highlights a rare occurrence of multifocal angiosarcoma related to Stewart-Treves syndrome in a patient with a history of filariasis and identified sickle cell trait, making this combination very uncommon.
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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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Lymphangiectasia is characterized by vesicular dilation of lymphatic vessels and is generally a complication of radiotherapy or surgery of various malignant tumors such as breast and cervical cancers. Although it is not a precancerous disease, correct diagnosis is important to rule out Stewart-Treves syndrome which is defined as lymphangiosarcoma that develops in long-standing chronic postmastectomy lymphedema. Observation alone or many therapeutic options including cryosurgery, electrocauterization, sclerotherapy, and excision have been widely used in the management.

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