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复发性颈部黏液纤维肉瘤:一例报告。

Recurrent neck myxofibrosarcoma: a case report.

机构信息

Campus Bio-Medico University of Rome, Via Alvaro del Portillo, 200 - 00128, Roma, RM, Italy.

IRCCS Fondazione Don Carlo Gnocchi-Onlus, Florence, Italy.

出版信息

J Med Case Rep. 2021 Oct 1;15(1):484. doi: 10.1186/s13256-021-03053-4.

Abstract

BACKGROUND

Myxofibrosarcoma (MFS) is a rare soft tissue sarcoma with a high recurrence rate and a low risk of distant metastasis. It occurs mainly in the extremities of elderly men. Head and neck MFS is extremely rare. Surgery is the cornerstone of treatment. The role of radiotherapy (RT) and chemotherapy (CHT) on MFS is still debated.

CASE PRESENTATION

A 67-year-old Caucasian man presented to our sarcoma referral center (SRC) with a history of MFS of the neck excised with microscopic positive surgical margins in a non-referral center. Staging imaging exams did not reveal distant metastasis. After a multidisciplinary discussion, preoperative RT was administered with a total dose of 50 Gy followed by wide surgical excision. Histological examination was negative for viable tumor cells. No relapse occurred during the 24-month postoperative follow-up.

CONCLUSIONS

The case described suggests the importance of planned combined treatments with both RT and surgery for high-grade soft tissue sarcoma. RT seems to be promising within this specific histotype. Close follow-up is advisable in all cases. Further studies are needed to confirm if the observed efficacy of combined treatments results in a prolonged time of disease-free survival and overall survival.

摘要

背景

黏液纤维肉瘤(MFS)是一种罕见的软组织肉瘤,具有较高的复发率和较低的远处转移风险。它主要发生在老年男性的四肢。头颈部 MFS 极为罕见。手术是治疗的基石。放疗(RT)和化疗(CHT)在 MFS 中的作用仍存在争议。

病例介绍

一位 67 岁的白人男性因头颈部 MFS 就诊于肉瘤转诊中心(SRC),此前在非转诊中心接受过颈部 MFS 切除术,显微镜下切缘阳性。分期影像学检查未发现远处转移。经过多学科讨论,患者接受了术前 RT,总剂量为 50 Gy,随后进行了广泛的手术切除。组织学检查未见存活肿瘤细胞。术后 24 个月的随访中无复发。

结论

本例提示对于高级别软组织肉瘤,计划联合 RT 和手术治疗的重要性。RT 在这种特定的组织学类型中似乎很有前途。所有病例均应密切随访。需要进一步的研究来证实观察到的联合治疗的疗效是否导致无疾病生存期和总生存期的延长。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d06c/8485472/e78c0d084b97/13256_2021_3053_Fig1_HTML.jpg

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