Cozzi Salvatore, Finocchi Ghersi Sebastiano, Tava Francesca, Bardoscia Lilia, Najafi Masoumeh, Ruggieri Maria Paola, Serre Anne-Agathe, Roukoz Camille, Gutierrez Miguelez Cristina, Lazrek Amina, Sardaro Angela, Taverna Cecilia
Radiation Oncology Department, Centre Léon Bérard, 69373 Lyon, France.
Radiation Therapy Unit, Azienda USL-IRCCS di Reggio Emilia, 42123 Reggio Emilia, Italy.
J Pers Med. 2024 Aug 14;14(8):859. doi: 10.3390/jpm14080859.
Considering the increasing number of conservative surgeries (quadrantectomies) for primary breast carcinoma, especially in the early stages, often followed by adjuvant radiotherapy, the incidence of radiation-associated angiosarcoma (RAS) is expected to rise in the coming decades, and it will represent a clinical and therapeutic challenge, as limited data are available due to the rarity and heterogeneity of the disease. Though the prognosis of these patients is poor, a number of clinical and pathological factors can be evaluated to better understand the course of RAS. The aim of this systematic review is to explore the available clinical-pathological, therapeutic, and prognostic data regarding RAS to evaluate its occurrence, diagnosis, treatment, and outcomes.
RAS clinical data were identified by a systematic review conducted in five different databases (Medline, Web of Knowledge, Google Scholar, Scopus, and Cochrane). Only RAS works published in English, with access to the full manuscript text, and with clear results, were considered as eligible.
We considered 52 papers comprising 319 RAS cases. The patient age at diagnosis ranged from 37 to 93 years, with most cases occurring from 5 to 10 years after breast irradiation. The most common clinical presentation was an aggressive development of macules, papules, or erythematous-violaceous skin discoloration at the site of previous radiation treatment for primary breast carcinoma. Complete surgical excision appeared to be the first-line treatment of the disease, in some cases followed by adjuvant local and/or systemic therapies. Despite different treatments, local recurrence rates ranged from 40% to 90%, leading to negative outcomes and poor prognosis for patients.
Although the literature is limited and the data are heterogeneous and contentious, our review aims to highlight the importance of early diagnosis, multimodal treatment, and long-term follow-up of RAS in order to limit and prevent the aggressiveness of this neoplasm.
鉴于原发性乳腺癌保乳手术（象限切除术）的数量不断增加，尤其是在早期阶段，且术后常需辅助放疗，预计未来几十年与放疗相关的血管肉瘤（RAS）的发病率将会上升，这将成为一个临床和治疗挑战，因为该疾病罕见且具有异质性，可用数据有限。尽管这些患者的预后较差，但可以评估一些临床和病理因素，以更好地了解RAS的病程。本系统评价的目的是探讨有关RAS的现有临床病理、治疗和预后数据，以评估其发生、诊断、治疗及转归。
通过在五个不同数据库（Medline、Web of Knowledge、谷歌学术、Scopus和Cochrane）中进行系统评价来确定RAS的临床数据。仅将以英文发表、可获取全文且结果明确的RAS研究视为合格。
我们纳入了52篇论文，共319例RAS病例。诊断时患者年龄在37至93岁之间，大多数病例发生在乳腺癌放疗后5至10年。最常见的临床表现是在原发性乳腺癌既往放疗部位出现斑疹、丘疹或红斑 - 紫罗兰色皮肤变色的侵袭性发展。完整手术切除似乎是该病的一线治疗方法，在某些情况下随后进行辅助局部和/或全身治疗。尽管治疗方法不同，但局部复发率在40%至90%之间，导致患者预后不良。
尽管文献有限且数据存在异质性和争议性，但我们的综述旨在强调RAS早期诊断、多模式治疗和长期随访的重要性，以限制和预防这种肿瘤的侵袭性。
