Leiomyomatosis peritonealis disseminata presenting as acute abdomen: A rare case report.

A 39-year-old woman, para 1 (live birth via vaginal delivery) with one spontaneous abortion at 3 months' gestation, presented to the emergency department with severe lower abdominal pain and vomiting for the past three days. She reported a history of progressive lower abdominal swelling over several months, accompanied by lower back pain, menstrual-associated abdominal pain, weight loss, and easy fatigability. Her menstrual bleeding was regular and normal in frequency, volume, and duration. She had no current pregnancy or fibroid-related diseases. She also had no prior surgical interventions such as cesarean section, myomectomy, or laparoscopy. She has been married to three partners during her lifetime and had a stable sexual relationship at the time of presentation. Regarding hormonal exposure, the patient had intermittently used post-coital hormonal contraceptives but denied long-term or continuous hormonal contraceptive use, such as depot medroxyprogesterone acetate (DMPA), implants, or combined oral contraceptives. She had never undergone hormonal replacement therapy (HRT) or fertility treatments and reported no history of exogenous estrogen or selective estrogen receptor modulator use. She had no occupational or environmental exposure to known endocrine-disturbing chemicals. On examination, her abdomen was distended, equivalent to a 20-week-sized gravid uterus, and was tender with a firm, irregular palpable mass arising from the pelvis. Bimanual pelvic examination revealed a smooth cervix and an irregularly enlarged, tender uterus with positive cervical motion tenderness. Other systemic examinations were unremarkable. All the baseline laboratory investigations were within normal range. An ultrasound imaging was performed, revealing an ill-defined, heterogeneous, irregular myometrial mass at the anterior uterine wall with multiple loculated subserosal masses and adjacent fat stranding on the left side, which may suggest a torsioned pedunculated subserosal leiomyoma. The endometrial lining of the uterus was smooth, and no collection or mass was seen. There was no gross bilateral adnexal mass noticed. With the impression of a huge myomatous uterus with likely pedunculated subserosal leiomyomatous torsion, the patient was counselled for exploratory laparotomy. During surgery, a large, vascularized, hemorrhagic, multiple nodular mass was found involving the peritoneum. Several nodular masses had vascular supply directly from the peritoneum and showed superficial inflammatory adhesions to the omentum. At the moment, leiomyosarcomatous changes of leiomyoma were considered. As a result, a total abdominal hysterectomy with bilateral salpingo-oophorectomy was performed, along with removal of additional detached nodular masses located in the left paracolic gutters and omentum. All visible lesions were excised and submitted to histopathological analysis ( Fig. 1 ). Fig. 1 Hemorrhagic multiple nodular mass originated from the uterus immediately after laparotomy (A). Huge uterus with intramural and multiple hemorrhagic nodular subserosal myomas after hysterectomy and bilateral salpingo-oophorectomy (B). Fig. 1 Hemorrhagic multiple nodular mass originated from the uterus immediately after laparotomy (A). Huge uterus with intramural and multiple hemorrhagic nodular subserosal myomas after hysterectomy and bilateral salpingo-oophorectomy (B). The patient's postoperative course was uneventful, and she was discharged on the third postoperative day with advice for routine follow-up to monitor for recurrence. Histopathological examination confirmed the diagnosis of Leiomyomatosis peritonealis disseminata (LPD). The microscopic examination through the uterine and omental masses showed right-angle intersecting fascicles of bland oval to spindle cells having elongated nuclei with blunted ends and mild eosinophilic cytoplasm. Areas of hemorrhage and variable-sized blood vessels are also noted. No mitosis, necrosis, or nuclear atypia was observed ( Fig. 2 ). Fig. 2 Hematoxylin and eosin images. A–C. 10× magnification displaying right angles intersecting fascicles of bland oval to spindle cells having elongated blunt-ended nuclei and mild eosinophilic cytoplasm. D. 10× showing interspersed variable-sized vasculatures filled with blood. Fig. 2 Hematoxylin and eosin images. A–C. 10× magnification displaying right angles intersecting fascicles of bland oval to spindle cells having elongated blunt-ended nuclei and mild eosinophilic cytoplasm. D. 10× showing interspersed variable-sized vasculatures filled with blood. At the time of this report, the patient is in her fifth postoperative month. Follow-up clinical and ultrasound evaluations, performed twice, revealed no recurrence or abnormal findings. A long-term surveillance plan has been established, which includes clinical and imaging assessment every three months for the first two years, followed by annual follow-up thereafter. She has been advised to report any symptoms suggestive of recurrence. At her most recent follow-up, she reported significant improvements in overall well-being, and she expressed satisfaction with the outcome of the surgery and understanding of the importance of ongoing monitoring. No new complaints or complications have been identified to date. ( Table 1 ) Table 1 Timeline of the clinical course of evaluation and management of the case. Table 1 Time point Event Day 0- Initial presentation at our emergency department Complete evaluation with history and physical examination done. Routine laboratory workups and abdominopelvic ultrasound scan done, and revealed a large irregular intramural and subserosal mass extending to the peritoneum and omental inflammatory features. Day 1 Exploratory laparotomy done with intraoperative findings of vascularized nodular peritoneal mass extended from enlarged and irregular myomatous uterus. The surgical specimen subjected to histopathologic examination Day 3 Uneventful postoperative course; discharged with follow-up instructions. Day 7 No complaints, appetite improved, no sign of surgical site infection. Abdominopelvic ultrasound scan was unremarkable. Day 14 Histopathology report confirmed the diagnosis of Leiomyomatosis peritonealis disseminata. Discussion made with the patient about the diagnosis and course of the disease. 3 months postoperative No complaint from the patient, abdominopelvic ultrasound scan was unremarkable with no signs of recurrence. Patient is doing well and actively engaged in her routine day to day activities. Timeline of the clinical course of evaluation and management of the case.

Written informed consent was obtained from the patient for publication of this case report. A copy of the written consent is available for review by the editor-in-chief of this journal on request.

The study was notified to the university ethics committee, but this is a case report, and it does not need a specific ethical approval.

This study did not receive any grants or funding from either for-profit or non-profit organizations.

Not applicable.

Rarely occurring leiomyomatosis peritonealis disseminata can be difficult to diagnose, especially when it manifests as acute abdominal symptoms. Early detection is crucial because the clinical presentation frequently overlaps with other more prevalent conditions like fibroids and endometriosis. The cornerstone of treatment, especially for patients with symptoms, is surgery. The prognosis is generally good due to its benign nature, but frequent follow-up is essential to check for recurrence.

Leiomyomatosis peritonealis disseminata (LPD) is a rare, benign condition characterized by the presence of multiple smooth muscle nodules distributed along the peritoneal surfaces. It is a rare variant of uterine leiomyoma, mostly affecting women in the premenopausal age group [ 6 ]. While its pathogenesis remains unclear, two primary theories have been proposed: the hormonal and iatrogenic factors [ 3 ]. Iatrogenic theory suggests that LPD is caused by previous uterine leiomyoma surgeries resulting in small leiomyoma fragment remnants disseminated into the peritoneum [ 7 , 8 ]. Cytogenetic studies support this hypothesis by demonstrating that peritoneal lesions are monoclonal and genetically similar to the original uterine leiomyoma [ 9 ]. However, cases of LPD without any prior surgical history, including our case, challenge the universality of this theory and suggest alternative mechanisms may also be at play. The hormonal theory suggests that high levels of estrogen and progesterone cause submesothelial multipotent stem cells to change into smooth muscle cells through a process called metaplastic transformation [ 10 , 11 ]. There is a lot of evidence for this theory, including the fact that LPD is often linked to pregnancy, estrogen-secreting ovarian tumors, and exogenous hormonal therapy. There is also evidence that it gets better after menopause, oophorectomy, or treatment with gonadotropin-releasing hormone (GnRH) analogues [ [12] , [13] , [14] ]. Our patient, interestingly, had never used prolonged hormonal contraceptive use before, which makes this case different from most others that have been reported. In addition to offering insights into potential alternative disease mechanisms, the patient's demographic and gynecologic profile presents an uncommon context for the development of LPD. The 39-year-old African woman did not have any of the most frequently mentioned risk factors for LPD, including a history of laparoscopic uterine surgery, no long-term hormonal therapy, and limited use of contraceptives. Therefore, her presentation implies that endogenous factors—which could be of genetic or epigenetic origin—may be more important in some populations [ 6 ]. It also suggests that pathogenesis may be influenced by regional or ethnic differences in mesenchymal stem cell responsiveness or hormonal sensitivity [ 15 ]. Additionally, the underreporting of LPD in sub-Saharan Africa could be due to a real lower incidence that has not yet been discovered, diagnostic difficulties, or restricted access to sophisticated imaging and histopathology [ 16 ]. In order to fully comprehend the range of LPD pathophysiology, this case emphasizes the necessity of more extensive epidemiological surveillance and molecular research in a variety of populations. LPD is often asymptomatic and found incidentally in a clinical setting. However, in symptomatic cases, patients may have vague symptoms like abdominal discomfort or bloating or, more rarely, may present as an acute abdomen. In our case, the acute presentation was probably caused by hemorrhagic degeneration or inflammatory irritation from the vascularized nodules that affected the peritoneum and omentum [ 14 ]. Imaging such as ultrasound, CT scan, and MRI are important in differentiating leiomyoma from other solid tumors of the pelvis but cannot reliably distinguish LPD from malignant conditions of the pelvis [ 17 ]. In our patient, ultrasound scanning revealed a large heterogeneous uterine mass with extrauterine extensions and omental inflammatory changes, initially raising concern for leiomyosarcoma. This highlights the importance of the intraoperative evaluation and postoperative histopathological confirmation, which are the gold standards for definitive diagnosis [ 18 ]. There is no standard management protocol that exists for LPD. However, most studies categorize the options of management into observation, hormonal, and surgical approaches depending on symptom severity, patient age, fertility desires, lesion size, and extent of disease. Asymptomatic or minimally symptomatic cases may be managed conservatively with close observation and elimination of hormonal stimulation, particularly in premenopausal women [ 19 , 20 ]. If the condition causes significant symptoms or complications, surgical intervention or hormonal therapy might be required [ 21 ]. Treatment options include local lesion excision, total hysterectomy, bilateral salpingo-oophorectomy, hormonal suppressions like GnRH agonists or antagonists, aromatase inhibitors, and estrogen receptor inhibitors [ 22 , 23 ]. In our case, given the intraoperative suspicion of malignancy, hysterectomy, bilateral salpingo-oophorectomy, and omentectomy were performed [ 24 ]. Postoperatively, the prognosis is generally favorable, with most patients experiencing resolution of symptoms and remaining recurrence-free [ 25 ]. However, recurrence has been seen, especially in those with incomplete resection, ongoing hormonal therapy, or widespread disease [ 26 , 27 ]. Malignant transformation of LPD has also been observed, which could occur from months of LPD diagnosis and management to years [ 27 , 28 ]. This emphasizes the necessity of routine clinical and imaging follow-up in order to identify malignant transformation and recurrence. Our patient is in her fifth month following surgery, and both her clinical and ultrasound evaluations have revealed no recurrence. For the first two years, she will continue to be monitored according to a structured follow-up protocol that includes clinical and imaging evaluation for the first two years, then annually thereafter. The geographic and clinical uniqueness of this case make it especially significant. Very few LPD cases have been reported from Africa, and the majority come from high-income settings. In addition to lacking common risk factors like previous surgery or long-term hormonal therapy, our patient is an unusual addition to the epidemiological literature from a sub-Saharan African setting. This emphasizes the necessity of more extensive documentation of LPD in a variety of populations in order to comprehend its causes, manifestations, and consequences.

Leiomyomatosis peritonealis disseminata (LPD) is a rare condition characterized by the presence of multiple smooth muscle tumors originating from uterine myoma throughout the peritoneum, omentum, and mesentery [ 1 ]. It is one of the three rare and unusual growth variants of uterine leiomyoma: intravenous leiomyomatosis (IVL), benign metastasizing leiomyoma (BML), and disseminated peritoneal leiomyomatosis (DPL) [ 2 ]. Despite being benign, these tumors' size, vasculature, and adherence to nearby structures can cause serious morbidity. Although little is known about the pathophysiology of LDP, experts have divided the causes into two theories, including iatrogenic and hormonal factors [ 3 ]. Although rare, LPD predominantly affects premenopausal women, possibly due to their higher hormonal level, and is difficult to diagnose because of its vague symptoms. Infertility, abdominal edema, and persistent pelvic pain are common presenting symptoms that can coexist with other more prevalent gynecologic disorders like fibroids or endometriosis [ 4 ]. Rarely, as in our case, LPD can manifest as an acute abdomen that necessitates surgery. This case report is written according to the SCARE guideline 2025 [ 5 ].

The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.