Williams Austin D, Heightchew Kimberly, Siripirapu Veeraiah
Department of Surgery, Lankenau Medical Center, 100 E Lancaster Avenue, Wynnewood, PA 19096, USA.
Department of Pathology, Lankenau Medical Center, 100 E Lancaster Avenue, Wynnewood, PA 19096, USA.
Int J Surg Case Rep. 2016;26:150-3. doi: 10.1016/j.ijscr.2016.07.044. Epub 2016 Jul 28.
Intra-abdominal desmoid tumors (DTs) are a rare and anatomically diverse group of locally-aggressive, benign neoplasms. They are often difficult to diagnose, even in patients who possess risk factors for the disease. Even after a diagnosis has been reached, the optimal therapy is often not well-defined.
The case discussed of a 33-year old male with a giant intra-abdominal desmoid is an example of both the diagnostic and therapeutic dilemmas that arise when confronted with a patient with a DT. Initial confusion over diagnosis led to ineffective therapy, but once the correct diagnosis was made, the patient went on to definitive surgical resection.
The differential diagnosis of DTs is broad, and the diagnosis is often delayed due to nonspecific presentations. Immunohistochemistry is crucial in the accurate histological diagnosis, which guides treatment. Chemotherapy and radiation have a role in the management of both primary and recurrent lesions, but surgical resection remains the cornerstone of treatment.
DTs present a clinical challenge in their diagnosis and management, and despite providing standard medical and surgical treatment, recurrence rates are high and continued surveillance is crucial.
腹腔内硬纤维瘤（DTs）是一组罕见的、解剖结构多样的局部侵袭性良性肿瘤。即使对于有该疾病危险因素的患者，它们也常常难以诊断。即使确诊后，最佳治疗方案通常也不明确。
所讨论的一名33岁患有巨大腹腔内硬纤维瘤的男性病例，是面对DT患者时出现的诊断和治疗困境的一个例子。最初诊断上的困惑导致治疗无效，但一旦做出正确诊断，患者接受了确定性手术切除。
DTs的鉴别诊断范围广泛，由于表现不具特异性，诊断常常延迟。免疫组织化学在准确的组织学诊断中至关重要，该诊断指导治疗。化疗和放疗在原发性和复发性病变的管理中发挥作用，但手术切除仍然是治疗的基石。
DTs在诊断和管理方面带来临床挑战，尽管提供了标准的医学和手术治疗，但复发率很高，持续监测至关重要。
