Department Of Urology, Armed Forces Hospital, King Abdulaziz Air Base, Dhahran, Kingdom of Saudi Arabia.
Department Of Pathology, Armed Forces Hospital, King Abdulaziz Air Base, Dhahran, Kingdom of Saudi Arabia.
Gulf J Oncolog. 2023 Jan;1(41):100-106.
Desmoid tumors (DTs) account for 3% of all soft tissue tumors. They are benign and have no malignant potential with a favorable prognosis, and predominantly occur in young women. The pathogenesis and clinical behavior of DTs are still uncertain. In addition, most cases of DTs were associated with abdominal trauma (including surgery), while genitourinary involvement seemed to be quite rare. Up to now, there has been only one DT case with urinary bladder involvement reported in the literature. We, Hereby, report a 67-year-old male patient with left lower abdominal pain while micturition. Computed tomography (CT) showed a mass located at the lower aspect of the left rectus muscle with an extension attached to the urinary bladder. Based on the pathological findings of tumor specimen, a diagnosis of benign desmoid tumor (DT) of the abdominal wall was made. Laparotomy with wide local excision was carried out. The patient had a smooth postoperative recovery and was discharged after 10 days. Introduction:Historically, MacFarland first described these tumors in 1832. Etymologically, the word desmoid was first coined by Muller in 1838 and is derived from the Greek word desmos, which means band or tendon-like. Stout first used the term fibromatosis in 1961[1,2,3]. Desmoid tumors (DTs) are a kind of rare neoplasm, which represents 3% of all soft tissue tumors and 0.03% of all neoplasms with an incidence of 5 to 6 per million of the population per annum[4,5,6]. DTs predominantly affect young females with a median age of 30 to 40 years old and is more than twice in female than male patients. However, there is no gender preference in older patients [7,8]. Furthermore, the symptoms of DTs are not typical in general. Symptoms can occasionally occur due to the size and location of the tumor but usually are nonspecific. Because of its rarity and unusual behavior, DT is often associated with diagnostic and therapeutic challenges. Computed tomography (CT) and magnetic resonance imaging (MRI) is beneficial for the diagnosis of this tumor but pathological diagnosis is mandatory. Surgical resection is now considered as the most efficient treatment option for patients with DT, because it offers a good chance of long-term survival. Our case is of unusual presentation and finding of abdominal wall desmoid tumor with an extension to urinary bladder in a male patient who is 67 years old. Keywords: desmoid tumor, fibromatosis, spindle cell tumor, urinary bladder.
腹壁良性纤维瘤病累及膀胱 1 例报告
腹壁硬纤维瘤（desmoid tumors，DTs）占所有软组织肿瘤的 3%。其为良性，无恶性潜能，预后良好，多见于年轻女性。DTs 的发病机制和临床行为仍不确定。此外，大多数 DTs 与腹部创伤（包括手术）有关，而泌尿生殖系统受累似乎很少见。迄今为止，文献中仅报道过一例累及膀胱的 DTs 病例。我们在此报告一例 67 岁男性患者，其表现为左下腹疼痛伴排尿困难。计算机断层扫描（CT）显示左腹直肌下段有一肿块，延伸附着于膀胱。根据肿瘤标本的病理结果，诊断为腹壁良性纤维瘤病（DT）。行剖腹探查并广泛局部切除。患者术后恢复顺利，10 天后出院。
1832 年，MacFarland 首次描述了这些肿瘤。从词源学上讲，“desmoid”一词最早由 Muller 于 1838 年创造，源自希腊语单词 desmos，意思是带或腱样。Stout 于 1961 年首次使用“纤维瘤病”一词[1,2,3]。纤维瘤病是一种罕见的肿瘤，占所有软组织肿瘤的 3%，占所有肿瘤的 0.03%，年发病率为每百万人口 5 至 6 例[4,5,6]。DTs 主要影响年轻女性，中位年龄为 30 至 40 岁，女性患者是男性的两倍以上。然而，在老年患者中无性别偏好[7,8]。此外，DTs 的症状通常不典型。由于肿瘤的大小和位置，偶尔会出现症状，但通常是非特异性的。由于其罕见性和异常行为，DT 常与诊断和治疗挑战相关。计算机断层扫描（CT）和磁共振成像（MRI）有助于诊断该肿瘤，但病理诊断是必需的。手术切除现已被认为是 DT 患者最有效的治疗选择，因为它为长期生存提供了很好的机会。我们的病例是一种不常见的表现和发现，即 67 岁男性患者腹壁纤维瘤病延伸至膀胱。
腹壁硬纤维瘤、纤维瘤病、梭形细胞肿瘤、膀胱
