Case
The low prevalence of DPL is a barrier to obtaining accurate information about its prevalence, developing protocols, and fully elucidating its pathogenesis. There is strong evidence that hormonal factors play an important role in the development of DPL, as suggested by the predominance of cases among women of reproductive age and the reported reduction in tumor size with aromatase inhibitor therapy. 3 , 9 However, the role of hormonal influence has been questioned in some cases, either due to the absence of hormone receptor expression or the occurrence of DPL in postmenopausal women. 9 , 10
The hypothesis that DPL may have an iatrogenic origin has been discussed based on its association with laparoscopic myomectomies. 11 Power morcellation of fibroids may lead to inadvertent dispersion of smooth muscle tissue within the peritoneal cavity, with subsequent implantation at ectopic sites. 12 More recently, this mechanism has supported the proposal by some authors that these lesions constitute a distinct category of leiomyomas, termed “iatrogenic myomas,” emphasizing the role of surgical manipulation in their pathogenesis and reinforcing the importance of preventive strategies during minimally invasive surgery. 13 , 14
Although the exact mechanism of DPL remains unclear, its development likely results from a complex interaction between individual genetic susceptibility, hormone-mediated cellular metaplasia, and iatrogenic dissemination during myomectomy. 12 – 15 Among the potentially modifiable risk factors, contained (protected) power morcellation has been proposed as a strategy to reduce the risk of DPL development; however, robust evidence regarding its effectiveness is still lacking. 13
Although most uterine leiomyomas grow in a predictable manner, some forms of smooth muscle tumors exhibit unusual growth patterns including intravenous leiomyomatosis, benign metastatic leiomyomatosis, and DPL. 16 DPL can often mimic metastatic carcinoma of the ovaries or peritoneum, which can lead to misdiagnoses and potencially unnecessary radical treatments. 17 , 18
The clinical diagnosis is uncommon, given that the symptoms associated with DPL are nonspecific and include chronic abdominal pain, abdominal distension, changes in bowel habits, changes in the menstrual cycle, and increased urinary frequency. 19 , 20
Detection of DPL on imaging studies can be challenging due to its unusual growth pattern and atypical distribution. DPL has been reported to involve the intestinal serosa, gastric serosa, hepatic surface, omentum, and Douglas pouch. 14 , 21 , 22 From a radiological perspective, the condition may be mistaken for malignant disease because of its appearance and peritoneal dissemination. The lack of specific imaging features contributes to a high rate of preoperative diagnostic error. 23
On imaging, DPL nodules typically demonstrate features similar to uterine leiomyomas on ultrasound and pelvic MRI, appearing as well-defined solid nodules with homogeneous echotexture and regular contours. 24 , 25 The main distinguishing feature is their extrauterine, peritoneal distribution rather than an intrinsic uterine origin. Although signal characteristics on MRI may vary according to tissue composition, these nodules generally show uniform enhancement after contrast administration. 26 , 27
Despite the use of imaging exams, the preoperative diagnosis of DPL remains challenging, with most cases being diagnosed. 24 In the present case, pelvic MRI was performed, but DPL was not suspected preoperatively. After the intraoperative findings, the radiologist was reconsulted and reported that the marked uterine enlargement limited spatial discrimination on preoperative MRI, making it difficult to determine that some nodules were separate from the uterus rather than directly related to it.
The decision to pursue radical resection of DPL nodules should be individualized and is not routinely recommended at the time of initial surgery. It must take into account the complexity and risks of excision, potential long-term surgical morbidity, the likelihood of disease recurrence, and the relatively low risk of malignant transformation. 19 In most cases, a staged approach is preferable, with histopathological confirmation obtained first and more extensive resections reserved for carefully selected situations, such as progressive disease, organ compression, or functional impairment. When radical resection is considered, it should be recognized that involvement of the gastric, hepatic, intestinal, or splenic hilar surfaces has been reported 24 , 28 and may require a multidisciplinary surgical team, highlighting the importance of careful patient selection and timing rather than upfront aggressive surgery.
A series of 14 cases of DPL reported a recurrence rate of 64% and a mean time to recurrence of 31 months. 3 Consequently, even after complete surgical excision of DPL, it is crucial to consider the potential for recurrence and the possible need for additional surgical procedures. Therefore, the choice of radical surgery should be restricted to more severe cases, characterized by a risk of compression and functional impairment of the affected organs. 29
When opting for partial resection of nodules or expectant management, no standardized recommendations for clinical follow-up have been established. It is important to recognize that DPL is a benign disease but carries an estimated 2–5% risk of malignant transformation 19 —higher than that reported for uterine leiomyomas.
Pharmacological treatment as a substitute for surgery or as an adjunct to reduce recurrence has been explored, but available studies show conflicting results. Medications such as ulipristal acetate, GnRH analogs, or aromatase inhibitors have been proposed; however, the evidence remains limited and inconclusive. 9 , 30 While some authors have reported no recurrence with postoperative medical therapy, other reports describe recurrence despite hormonal suppression. 24 , 28
Intro
Uterine fibroids are the most prevalent pelvic tumors in women. 1 In contrast, disseminated peritoneal leiomyomatosis (DPL) is a rare condition characterized by multiple peritoneal and subperitoneal nodules composed of smooth muscle cells. 2 , 3 This entity was first described by Willson and Peale in 1952, being associated with granulosa cell tumors of the ovary. 4
Two main etiopathogenic theories have been proposed for the development of DPL proliferation of submesothelial multipotent cells within the pelvic peritoneum 3 , 5 and iatrogenic dissemination secondary to unprotected power morcellation of fibroids. 6 The second hypothesis becomes more substantial when considering that 75.8% of reported cases of DPL had a history of prior laparoscopic myomectomy. 6
We present the case of a 46-year-old patient, with no history of myomectomy, who was incidentally diagnosed with DPL during an elective hysterectomy due to uterine leiomyomatosis. This case highlights the diagnostic challenges of DPL, discusses its complex etiology beyond iatrogenic spread, and illustrates a balanced laparoscopic surgical approach in the absence of a preoperative diagnosis.
Conclusions
This case underscores that DPL must remain an important differential diagnosis in patients with multiple peritoneal nodules encountered during laparoscopic surgery, regardless of a history of morcellation, and that a minimally invasive approach allows for both diagnostic confirmation and safe, staged management. The reported case of a patient with no prior exposure to power morcellation reinforces the etiological complexity of DPL, suggesting that its origin extends beyond iatrogenesis and may be linked to hormonal factors and peritoneal mesenchymal metaplasia.
The management of DPL, as demonstrated in this case, requires an individualized approach that balances the risks and benefits of complete resection versus conservative or staged management. Laparoscopic resection is feasible, even in cases with multiorgan involvement. Although considered a benign condition, DPL requires close surveillance due to its high recurrence rate and the albeit low risk of malignant transformation. The lack of consensus regarding adjuvant therapy and clinical follow-up highlights the need for further studies and additional case reports to establish clearer management guidelines.