Limaiem Faten, Gharbi Mohamed Amine, Boujelbene Nadia, Triki Ramy, Romdhane Khaled Ben, Bouzidi Ramzi
University of Tunis El Manar, Tunis Faculty of Medicine, 1007, Tunisia; Pathology Department, Hospital Mongi Slim La Marsa, Tunisia.
University of Tunis El Manar, Tunis Faculty of Medicine, 1007, Tunisia; Department of Orthopedic Surgery, Hospital Mongi Slim La Marsa, Tunisia.
Int J Surg Case Rep. 2024 Dec;125:110508. doi: 10.1016/j.ijscr.2024.110508. Epub 2024 Oct 23.
Desmoid-type fibromatosis is an uncommon tumor characterized by its local invasiveness, with shoulder involvement being notably infrequent. The optimal treatment strategy for this tumor remains a topic of ongoing debate.
A 47-year-old Tunisian woman with a history of hypothyroidism, presented with pain and swelling in her left shoulder for a year. Examination revealed a firm, painful 4 cm mass, and MRI showed a poorly defined intramuscular tumor in the deltoid muscle, initially suspected to be rhabdomyosarcoma. However, surgical biopsy confirmed desmoid-type fibromatosis of the shoulder. The patient underwent surgical wide en-bloc resection of the tumor. The patient's recovery was uneventful, and she received physical therapy. At the three-year follow-up, she reported residual shoulder pain after heavy lifting, improving with analgesics. Examination showed no neurological deficits and a Constant score of 83 out of 100.
Due to their deep-seated nature and infiltrative growth patterns into neighboring subcutaneous tissues or muscles, along with the presence of myxoid or fibrotic components, desmoid-type fibromatosis can present challenges in distinguishing them from malignant soft tissue neoplasms based on imaging characteristics.
While radiologic evaluations may indicate characteristics suggestive of a malignant soft tissue tumor, histological confirmation is imperative prior to initiating surgical intervention. Continued research into the optimal treatment approaches for desmoid-type fibromatosis is essential for improving future patient outcomes and quality of life.
韧带样型纤维瘤病是一种罕见肿瘤，其特点是具有局部侵袭性，累及肩部的情况尤为少见。该肿瘤的最佳治疗策略仍是一个持续争论的话题。
一名47岁的突尼斯女性，有甲状腺功能减退病史，因左肩疼痛和肿胀一年前来就诊。检查发现一个质地坚硬、有压痛的4厘米肿块，磁共振成像（MRI）显示三角肌内有一个边界不清的肌内肿瘤，最初怀疑为横纹肌肉瘤。然而，手术活检证实为肩部韧带样型纤维瘤病。患者接受了肿瘤的手术广泛整块切除。患者恢复顺利，并接受了物理治疗。在三年随访时，她报告说重物提举后仍有肩部疼痛，服用镇痛药后有所改善。检查未发现神经功能缺损，Constant评分为83分（满分100分）。
由于韧带样型纤维瘤病位置较深，呈浸润性生长至邻近皮下组织或肌肉，且存在黏液样或纤维成分，基于影像学特征将其与恶性软组织肿瘤区分开来可能具有挑战性。
虽然影像学评估可能显示提示恶性软组织肿瘤的特征，但在开始手术干预之前，组织学确诊至关重要。持续研究韧带样型纤维瘤病的最佳治疗方法对于改善未来患者的预后和生活质量至关重要。
