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广泛胸壁硬纤维瘤的复杂外科治疗

Complex Surgical Management of Extensive Chest-Wall Desmoid Fibromatosis.

作者信息

Jang Jeffrey, Cavallo Kathryn, Lee Juliet

机构信息

General Surgery, George Washington University, Washington, DC, USA.

出版信息

Cureus. 2024 Oct 16;16(10):e71670. doi: 10.7759/cureus.71670. eCollection 2024 Oct.

Abstract

Desmoid fibromatosis (DF) is a rare tumor that arises from fibroblasts and myofibroblasts and typically presents on the trunk and limbs. While metastasis of fibromatosis is exceedingly uncommon, DF can spread rapidly to adjacent tissue. Its aggressive nature and frequent recurrence pose challenges for treatment, often requiring a multidisciplinary approach. This case represents the multidisciplinary workup for the identification and management of a rare presentation of desmoid fibromatosis involving the breast. It also discusses the post-surgical follow-up of the patient after a complex resection and reconstruction. We report a case of a patient treated at George Washington University Hospital, Washington, DC. A 26-year-old woman presented with left chest pain. Physical examination revealed an approximately 12-13 cm, ill-defined, immobile mass arising from the left breast. An MRI demonstrated a 9.5 cm spiculated mass involving the left pectoralis major muscle and underlying costal cartilage junctions. The tumor extended deep to the rib margin and along the anterior margin of the pleura. The mass was resected en bloc in a joint case with thoracic and general surgery and reconstructed with a bilateral bi-pedicled, muscle-sparing, transverse rectus abdominis myocutaneous free flap with plastic surgery. Pathology demonstrated desmoid fibromatosis. In conclusion, desmoid tumors of the breast represent a very rare breast neoplasm that can imitate other breast masses, both benign and malignant. Surgery remains an important treatment option and may require coordination and complex surgical planning between various surgical subspecialties.

摘要

韧带样纤维瘤病(DF)是一种罕见的肿瘤,起源于成纤维细胞和平滑肌成纤维细胞,通常出现在躯干和四肢。虽然纤维瘤病的转移极为罕见,但DF可迅速扩散至邻近组织。其侵袭性和频繁复发给治疗带来挑战,通常需要多学科方法。本病例展示了对累及乳腺的罕见韧带样纤维瘤病进行识别和管理的多学科检查。它还讨论了复杂切除和重建术后患者的外科随访情况。我们报告了一例在华盛顿特区乔治·华盛顿大学医院接受治疗的患者。一名26岁女性因左胸痛就诊。体格检查发现左乳有一个约12 - 13厘米、边界不清、固定不动的肿块。磁共振成像(MRI)显示一个9.5厘米的毛刺状肿块,累及左胸大肌和下方的肋软骨连接处。肿瘤深入至肋骨边缘并沿胸膜前缘延伸。该肿块在胸外科和普通外科的联合手术中整块切除,并由整形外科用双侧双蒂、保留肌肉的横行腹直肌肌皮游离皮瓣进行重建。病理显示为韧带样纤维瘤病。总之,乳腺韧带样肿瘤是一种非常罕见的乳腺肿瘤,可模仿其他良性和恶性乳腺肿块。手术仍然是重要的治疗选择,可能需要各外科亚专业之间进行协调和复杂的手术规划。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5cfa/11568368/3dafd910ba78/cureus-0016-00000071670-i01.jpg

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