MYC-Nonamplified Secondary Lymphatic-Type Angiosarcoma With Prominent Lymphocytic Infiltrate Following Radiation Therapy for Myxoid Liposarcoma.

Abstract

ABSTRACT:

Cutaneous angiosarcomas (AS) are uncommon and morphologically heterogeneous. Recently, a distinctive lymphatic-type AS with prominent lymphocytic infiltrate has been observed. Although conventional AS typically bear poor prognosis, lymphatic-type AS with prominent lymphocytic infiltrate and pseudolymphomatous AS show prolonged survival with rare extracutaneous spread. We describe a unique case of AS in a 55-year-old woman who received surgical resection and radiation therapy for her prior myxoid liposarcoma. She developed a suspected recurrence 15 years later. Microscopically, the lesion showed an infiltration of the reticular dermis by irregular interanastamosing vascular spaces lined by atypical endothelial cells with nuclear "hobnailing" and hyperchromasia. A prominent intratumoral and peritumoral lymphocytic infiltrate obscuring the tumor cells was also present. The tumor cells were diffusely positive for endothelial cell markers, including D2-40. Notably, there was no evidence of MYC gene amplification by FISH. Additional NGS-based molecular analysis demonstrated no significant genetic mutations. The patient is alive with a history of two local recurrences, but no evidence of metastasis. We present this case to raise awareness of MYC-nonamplified secondary lymphatic-type AS with prominent lymphocytic infiltrate (pseudolymphomatous AS) and to discuss its differential diagnosis.

• Authors: Andrea Krajisnik, Bonnie L Balzer, Eric Vail, Earl Brien, Wonwoo Shon
• Journal: The American Journal of dermatopathology (Am J Dermatopathol) Vol. 44 Issue 12 Pg. 955-957 (Dec 01 2022) ISSN: 1533-0311 [Electronic] United States
• PMID: 36395451 (Publication Type: Case Reports, Journal Article)
• Copyright: Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.

Topics

• Female
• Humans
• Adult
• Hemangiosarcoma (genetics, radiotherapy, diagnosis)
• Liposarcoma, Myxoid (genetics, radiotherapy)
• Endothelial Cells (pathology)
• Lymphatic Vessels (pathology)
• Skin Neoplasms (pathology)
• Pseudolymphoma