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一名5个月大婴儿的巨大先天性颈面部硬纤维瘤病样纤维瘤病

Massive congenital cervicofacial desmoid-type fibromatosis in a 5-month-old infant.

作者信息

Albokashy Mohammed S, Halawani Mohammed S, Eshky Anoof T, Alsaad Khalid, Khoja Hatim A, Bawazir Samir M

机构信息

Pediatric Division, Department of Otolaryngology/Head and Neck Surgery, Prince Sultan Military Medical City, Riyadh, Saudi Arabia.

Department of Oral and Maxillofacial Surgery, Prince Sultan Military Medical City, Riyadh, Saudi Arabia.

出版信息

J Surg Case Rep. 2021 May 27;2021(5):rjab206. doi: 10.1093/jscr/rjab206. eCollection 2021 May.

DOI:10.1093/jscr/rjab206
PMID:34055293
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8159267/
Abstract

Desmoid-type fibromatosis (DF) is a borderline tumor of soft tissues that has low malignant potential but described as infiltrative, locally aggressive and rapidly growing. In the pediatric population, it occurs in the head and neck. Presentation varies based on tumor size and location. Despite the high recurrence rate, surgical excision remains the modality of choice with. Here, we report a case of a 5-month-old boy, with extensive head and neck DF that was managed twice with conservative debulking surgery through a combined transoral-transcervical approach. On 2-year follow-up, he was gaining weight with no developmental delay and had no clinical evidence tumor regrowth.

摘要

韧带样型纤维瘤病（DF）是一种软组织交界性肿瘤，恶性潜能低，但具有浸润性、局部侵袭性且生长迅速。在儿童群体中，它发生于头颈部。临床表现因肿瘤大小和位置而异。尽管复发率高，但手术切除仍是首选治疗方式。在此，我们报告一例5个月大的男童，患有广泛的头颈部DF，通过经口-经颈联合入路进行了两次保守性减瘤手术。在2年的随访中，他体重增加，无发育迟缓，且无肿瘤复发的临床证据。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/efbb/8159267/4233b9474478/rjab206f4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/efbb/8159267/c2eceb81a4c7/rjab206f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/efbb/8159267/83b5f6808ee1/rjab206f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/efbb/8159267/e4828f607b8e/rjab206f3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/efbb/8159267/4233b9474478/rjab206f4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/efbb/8159267/c2eceb81a4c7/rjab206f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/efbb/8159267/83b5f6808ee1/rjab206f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/efbb/8159267/e4828f607b8e/rjab206f3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/efbb/8159267/4233b9474478/rjab206f4.jpg

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