oa Disseminated peritoneal leiomyomatosis: A benign condition with diagnostic and therapeutic challenges

Background: Disseminated peritoneal leiomyomatosis (DPL) is a variant of parasitic leiomyomas that is characterized by multiple peritoneal and subperitoneal nodules of proliferating smooth muscle cells that histologically resemble uterine leiomyoma. We report a case of recurrent DPL to highlight its diagnostic and therapeutic challenges at the Federal Medical Centre, Keffi, Nigeria.

Case Report: The patient is a 25-year-old woman with a previous history of myomectomy 3 years before presentation to the hematology unit on account of abdominal lymphoma. Based on the working diagnosis, she was referred to the general surgery unit for an open biopsy and cytoreductive surgery. She was explored, and intraoperative findings were in keeping with multiple well-circumscribed intra-abdominal masses of varying sizes. The multiple and widespread locations of the masses precluded the complete removal of the masses. Four months post-surgery, she presented with similar lesions and had a repeat laparotomy. At the surgery, she had a total abdominal hysterectomy with bilateral salpingophorectomy and excision of the abdominal masses. She was then placed on letrozole, which prevented further tumor growth and abated her symptoms.

Discussion: DPL is often rarely diagnosed preoperatively and thus poses a diagnostic challenge, with many cases asymptomatic and therapeutic challenges due to its tendency to recur. Its management currently lacks consensus and is often determined by many factors, such as the age of the patients, the number of nodules, and the desire to have more children, among others. Surgical excision combined with hormonal therapy is recommended for patients who wish to conceive. For postmenopausal women and those who no longer desire conception, total abdominal hysterectomy with bilateral salpingo-oophorectomy should be considered to prevent recurrence.

Conclusion: DPL is a rare form of multiple extrauterine leiomyomas. We report a case of DPL in a woman that was managed with surgical intervention and hormonal manipulation therapy following the failure of the initial surgical excision alone. We thus suggest a combination of surgical intervention and postoperative hormonal manipulation in its management, as such a multi-modality of therapy was employed in the index case without evidence of recurrence after a year post-surgery.