Nabais Celso, Salústio Raquel, Sousa Francisco V, Porto Eusébio, Cardoso Carlos, Fradique Caldeira
Department of Surgery, Hospital de São José, Centro Hospitalar de Lisboa Central, Lisboa, Portugal.
Ann Med Surg (Lond). 2015 Apr 14;4(2):116-8. doi: 10.1016/j.amsu.2015.04.003. eCollection 2015 Jun.
Primary small bowel malignancy is unusual and accounts for 1-3% of all gastrointestinal tract neoplasms. Adenocarcinoma is one of the most common histologic types, but its frequency decreases with more distal locations. Its clinical presentation is nonspecific and is usually associated with advanced disease, which contributes to delayed diagnosis.
A 66-year-old woman was admitted to the hospital with a 6-day history of progressively worsening abdominal pain localized in the right lower quadrant, nausea, and vomiting. Investigation revealed an inflammatory appendiceal tumor. The patient underwent surgery and an unexpected tumor involving the distal ileal segment and ileocecal appendix was found. Right radical hemicolectomy with en bloc resection of the distal ileum was performed. Histopathological examination revealed adenocarcinoma of the ileum.
This rare entity is associated with a nonspecific clinical presentation that contributes to delayed diagnosis and treatment, and consequently to a worse prognosis. Approximately half of the cases are only diagnosed at surgery. Primary treatment consists of wide resection with locoregional lymphadenectomy. The role of adjuvant chemotherapy has yet to be determined.
This case demonstrates an unusual condition characterized by late and challenging diagnosis. We highlight the importance of an earlier diagnosis and optimal treatment for improved patient outcomes.
原发性小肠恶性肿瘤较为罕见，占所有胃肠道肿瘤的1% - 3%。腺癌是最常见的组织学类型之一，但其发生率随病变部位越靠近远端而降低。其临床表现缺乏特异性，通常与疾病进展相关，这导致诊断延迟。
一名66岁女性因右下腹部进行性加重的腹痛、恶心和呕吐6天入院。检查发现炎性阑尾肿瘤。患者接受手术，术中意外发现累及回肠远端和回盲部阑尾的肿瘤。遂行右半结肠根治性切除术并整块切除回肠远端。组织病理学检查显示为回肠腺癌。
这种罕见疾病的临床表现缺乏特异性，导致诊断和治疗延迟，进而预后较差。大约一半的病例仅在手术时才被诊断出来。主要治疗方法是广泛切除并进行局部区域淋巴结清扫。辅助化疗的作用尚未确定。
本病例显示了一种诊断较晚且具有挑战性的罕见情况。我们强调早期诊断和优化治疗对改善患者预后的重要性。
