Ferrario Luca, Zerbi Pietro, Angiolini Maria Rachele, Agarossi Alberto, Riggio Eliana, Bondurri Andrea, Danelli Piergiorgio
Department of General Surgery, Department of Biomedical and Clinical Sciences "Luigi Sacco", "Luigi Sacco" University Hospital, Università degli Studi di Milano, 20157 Milan, Italy.
Department of Pathology, Department of Biomedical and Clinical Sciences "Luigi Sacco", "Luigi Sacco" University Hospital, Università degli Studi di Milano, 20157 Milan, Italy.
Int J Surg Case Rep. 2018;49:25-29. doi: 10.1016/j.ijscr.2018.06.004. Epub 2018 Jun 18.
Leiomyomatosis peritonealis disseminata (LPD) is an uncommon disease featured by the presence of multiple nodules of smooth muscle cells scattered in the abdominal cavity. To date only about 150 cases have been reported in literature. We report a case of recurrent LPD after laparotomy.
In March 2016 a 36-year-old female, with a history of multiple previous laparoscopic myomectomies, consulted her gynaecologist complaining abdominal pain; a MRI was performed and reported multiple pelvic masses, subsequently excised during laparotomy. The patient refused a total hysterectomy with bilateral salpingo-oopherectomy so a close follow-up was recommended. In November 2017 when a new MRI revealed recurrency of the disease, a second laparotomy is performed and all visible nodules are excised. The histological exam confirms LPD diagnosis. On follow-up after three months the patients is completely asymptomatic.
Differential diagnosis of LPD is challenging due to its similarity to carcinomatosis and to other benign abdominal disorders. Malignant transformation is rare, but it may occur, so a close follow-up is necessary. Even if there is no consensus regarding the treatment, hormonal therapy is probably the best first line approach, while surgery should be the second choice.
LPD is an uncommon but potentially severe disease. In our opinion larger studies are necessary to improve our diagnostic effectiveness and to define the best therapeutic strategy.
播散性腹膜平滑肌瘤病(LPD)是一种罕见疾病,其特征是腹腔内散在多个平滑肌细胞结节。迄今为止,文献中仅报道了约150例病例。我们报告一例剖腹手术后复发性LPD病例。
2016年3月,一名有多次腹腔镜子宫肌瘤切除术史的36岁女性因腹痛咨询妇科医生;进行了磁共振成像(MRI)检查,报告发现多个盆腔肿块,随后在剖腹手术中切除。患者拒绝行全子宫加双侧输卵管卵巢切除术,因此建议密切随访。2017年11月,当新的MRI显示疾病复发时,进行了第二次剖腹手术,切除了所有可见结节。组织学检查确诊为LPD。随访三个月后,患者完全无症状。
LPD的鉴别诊断具有挑战性,因为它与癌病及其他腹部良性疾病相似。恶性转化很少见,但可能发生,因此有必要密切随访。即使对于治疗尚无共识,但激素治疗可能是最佳的一线治疗方法,而手术应作为第二选择。
LPD是一种罕见但可能严重的疾病。我们认为有必要进行更大规模的研究,以提高我们的诊断效率并确定最佳治疗策略。