Menzie Jack, Kuan Chih C, Ackermann Travis, Tay Yeng Kwang
Department of General Surgery, Monash Health, Melbourne, AUS.
Department of Pathology, Monash Health, Melbourne, AUS.
Cureus. 2024 Sep 9;16(9):e69042. doi: 10.7759/cureus.69042. eCollection 2024 Sep.
Pulmonary lymphangioleiomyomatosis (LAM) is a rare condition characterised by infiltration of the lungs with abnormal smooth muscles and cystic lesions. A rarer form of the condition is extrapulmonary LAM (E-LAM) where the same cystic lesions are found in various organs throughout the body resulting in a variety of symptoms. Given the rarity of E-LAM and the difficulty in diagnosing it, there is little evidence to guide its management both surgically and medically. We describe a case of a 22-year-old female with a nine-month history of abdominal pain found to have a large mesenteric mass during laparoscopy for suspected ovarian torsion. She underwent a laparotomy to exteriorise the mass and dissect it off the mesentery. The mass was removed without compromise to the bowel or mesentery. The patient recovered well without symptoms and was discharged without complications. Histopathology of the mass revealed it to be E-LAM. This case is the first to our knowledge that demonstrates the successful removal of a solitary E-LAM from the mesentery with minimal adverse outcomes and symptomatic relief.
肺淋巴管平滑肌瘤病（LAM）是一种罕见疾病，其特征是肺部被异常平滑肌和囊性病变浸润。该疾病的一种更罕见形式是肺外LAM（E-LAM），在全身各个器官中都能发现相同的囊性病变，从而导致各种症状。鉴于E-LAM的罕见性以及诊断困难，几乎没有证据可指导其手术和药物治疗。我们描述了一例22岁女性病例，该患者有9个月的腹痛病史，在因疑似卵巢扭转进行腹腔镜检查时发现有一个巨大的肠系膜肿块。她接受了剖腹手术以将肿块取出并从肠系膜上剥离。肿块被成功切除，未对肠管或肠系膜造成损伤。患者恢复良好，无症状，无并发症出院。肿块的组织病理学检查显示为E-LAM。据我们所知，该病例是首例成功从肠系膜切除孤立性E-LAM且不良后果最小并缓解症状的病例。
