Tee Sui Wu, Richards Avisha, Tan Yi Han, Jeyabalan Dhinisya, Durairaj Gunaseelan
Department of General and Vascular Surgery, Serdang Hospital, Selangor, 43000 Kajang, Malaysia.
Division of Clinical and Surgical Sciences, University of Edinburgh, Edinburgh EH8 9YL, UK.
J Surg Case Rep. 2022 Dec 25;2022(12):rjac585. doi: 10.1093/jscr/rjac585. eCollection 2022 Dec.
Desmoid fibromatosis is a rare, benign, locally aggressive fibroblastic proliferation that may occur in almost any anatomical location. Due to its rarity and unpredictable clinical course, there has not been a standard guideline of treatment. We encountered a case of desmoid fibromatosis in our centre. A young lady previously fit and well was referred for a symptomatic, rapidly growing left sided abdominal mass. Otherwise, she denied any bowel related symptoms or constitutional manifestation. Imaging demonstrated a large well-defined lobulated solid-cystic mass extending from vertebral level T10 to L5, measuring 10.5 cm × 15 cm × 23 cm. The mass was in close proximity with the left adrenal gland, left kidney, pancreas and spleen. Ultrasound guided biopsy interpreted it as a fibroblastic or myelofibroblastic tumour, favouring desmoid fibromatosis. Surgery was then performed where the mass was removed along with the left adrenal gland and kidney. Post-operative care was complicated with pulmonary embolism, hospital-acquired pneumonia and pancreatitis.
韧带样纤维瘤病是一种罕见的、良性的、具有局部侵袭性的成纤维细胞增殖性疾病,几乎可发生于任何解剖部位。由于其罕见性及不可预测的临床病程,目前尚无标准的治疗指南。我们中心遇到了一例韧带样纤维瘤病患者。一位既往健康的年轻女性因左侧腹部出现有症状的、迅速增大的肿块前来就诊。除此之外,她否认有任何肠道相关症状或全身表现。影像学检查显示一个边界清晰的分叶状巨大实性囊性肿块,从胸10椎体水平延伸至腰5椎体,大小为10.5 cm×15 cm×23 cm。该肿块与左肾上腺、左肾、胰腺和脾脏紧邻。超声引导下活检将其诊断为成纤维细胞或骨髓成纤维细胞肿瘤,倾向于韧带样纤维瘤病。随后进行了手术,切除了肿块以及左肾上腺和肾脏。术后护理出现了肺栓塞、医院获得性肺炎和胰腺炎等并发症。