Zi Ying, Shi Yuchen, Shi Rongjie
The First Affiliated Hospital of Dali University, Dali, Yunnan, China.
The First Affiliated Hospital of Kunming Medical University, Kunming, Yunnan, China.
Front Med (Lausanne). 2024 Dec 17;11:1420414. doi: 10.3389/fmed.2024.1420414. eCollection 2024.
Lymphangioleiomyomatosis (LAM) is a rare, low-grade malignant condition that typically affects women of childbearing age and primarily involves the lungs. While cases involving males and affecting the gastrointestinal tract are exceedingly uncommon. This report discusses an unusual case of abdominal LAM in a male patient with gastrointestinal hemorrhage. The patient, a 70-year-old man, had been experiencing recurrent abdominal pain, occasional black stools, dizziness, and fatigue for over a month before being admitted to the hospital. Diagnostic electronic gastroscopy identified ulcers in the gastric and duodenal bulb with hemorrhage. An abdominal CT scan revealed multiple cystic foci in the retroperitoneum and mesentery, but no masses were found. Despite receiving medical treatment, the patient continued to have black stools and eventually underwent laparoscopic distal subtotal gastrectomy. The pathological results of the excised distal gastric specimens showed LAM-like lesions in the submucosal layer of the pylorus, mesentery of the side of the lesser curvature of the stomach, and small intestine, leading to a diagnosis of abdominal LAM. However, even after the surgery, the patient still experienced recurrent black stools and developed new symptoms of chest tightness and shortness of breath. A follow-up chest CT revealed bilateral pleural effusion and multiple lung cysts, indicating a worsening condition. The patient was then prescribed oral Sirolimus, which resulted in an improvement in symptoms, including black stool, chest tightness, and shortness of breath. This case report provides a detailed account of the progression of an unusual gastrointestinal LAM case and suggests that a combination of surgery and Sirolimus may be effective in managing the condition.
淋巴管平滑肌瘤病（LAM）是一种罕见的低度恶性疾病，通常影响育龄女性，主要累及肺部。而涉及男性并影响胃肠道的病例极为罕见。本报告讨论了一例男性患者发生胃肠道出血的腹部LAM罕见病例。该患者为一名70岁男性，在入院前一个多月一直反复出现腹痛、偶尔黑便、头晕和疲劳症状。诊断性电子胃镜检查发现胃和十二指肠球部有溃疡并伴有出血。腹部CT扫描显示腹膜后和肠系膜有多个囊性病灶，但未发现肿块。尽管接受了药物治疗，患者仍持续出现黑便，最终接受了腹腔镜远端胃次全切除术。切除的远端胃标本的病理结果显示，幽门黏膜下层、胃小弯侧肠系膜和小肠有LAM样病变，从而确诊为腹部LAM。然而，即使手术后，患者仍反复出现黑便，并出现了胸闷和气短的新症状。随访胸部CT显示双侧胸腔积液和多个肺囊肿，表明病情恶化。随后给患者开了口服西罗莫司，症状包括黑便、胸闷和气短均有所改善。本病例报告详细描述了一例罕见的胃肠道LAM病例的病程，并表明手术和西罗莫司联合使用可能对治疗该病有效。
