Introduction

Leiomyomatosis peritoneal disseminated (LPD) is a rare, benign disease characterized by the proliferation of multiple peritoneal and subperitoneal nodules consisting of smooth muscle cells¹. The differential diagnosis of LPD is challenging due to its clinical similarity to peritoneal carcinomatosis or metastatic lesions. and its histological similarity with benign metastatic leiomyoma². The first reported case in the literature was published in 1952 by Wilson and Peale³. There are less than 200 cases reported in the world literature, given its rarity there are no established guidelines for its treatment⁴. Some risk factors have been identified for LPD, use of oral contraceptives, metaplasia, genetics, pregnancy, iatrogenic and surgical manipulation⁵. We present a case of LPD intervened in our center.

Clinical case

A 29-year-old female with a history of cesarean section and abdominal hysterectomy for uterine myomatosis 4 years ago. He came due to presenting abdominal pain of moderate intensity, located in the hypogastrium with irradiation towards the left pelvic limb, which increased with the passing of days. Ultrasound is performed where multiple images of solid characteristics in the pelvic cavity are identified. In the simple and contrasted abdominopelvic tomography, a left retroperitoneal image of 6.4 x 7.4 x 7.2 cm with a volume of 178.3 cc was observed. displacing the kidney on the same side (Fig. 1), multiple solid images (approximately ten) of different sizes were found in the pelvis, the largest being 7.4 x 8.0 x 7.6 cm with intense enhancement with contrast medium (Fig. 2). The patient underwent elective surgery, during surgery multiple peritoneal and retroperitoneal nodules were found in the right obturator fossa, left iliac muscle, left renal pole, pelvic cavity and right paracolic frame (Fig. 3). They were completely resected carefully by the surgeons, hemostasis was performed with electrocoagulation and sutures (Vicryl 2-0), with satisfactory postoperative evolution.

Figure 1 Computed tomography: heterogeneous mass with well-defined borders that displaces the left kidney. 

Figure 2 Computed tomography: multiple pelvic heterogeneous nodules. 

Figure 3 Intraoperative image showing representative pedunculated nodular formation. 

Histological analysis of the surgical specimens confirmed the diagnosis. The histological structure of the tissue consisted of benign smooth muscle cell tumors, without mitotic figures or cellular atypia, without necrosis (Fig. 4). Immunohistochemistry: overexpression of estrogen (Fig. 5) and progesterone (Fig. 6) receptors. Follow-up was continued for 1 year, being asymptomatic and without evidence of ultrasound disease.

Figure 4 View at 40X, spindle nuclei, fine chromatin, no mitosis, no atypia. 

Figure 5 Immunohistochemistry, estrogen receptors with nuclear intensity 1. 

Figure 6 Immunohistochemistry, nuclear intensity 2 progesterone receptors. 

Discussion

LPD is an extremely rare clinical condition. Two main theories of the etiology and pathophysiology of LPD have been reported: a hormonal theory with mesenchymal stem cell metaplasia and an iatrogenic origin after surgery. According to the hormonal theory, LPD is supposed to result from the metaplastic change of mesenchymal stem cells with exposure to high levels of female steroids⁶. Recent publications have highlighted a link between surgical history such as hysterectomy for myomatosis or the laparoscopic uterine fibroid fragmentation technique and the development of LPD, due to the potential for tumor implantation and dissemination⁷. More specifically, LPD can present years after myomectomy or hysterectomy⁸. In our case, the history of hysterectomy due to myomatosis without adequate containment systems could have played a role in the pathogenesis of LPD.

The preoperative diagnosis of LPD can be challenging due to its clinical manifestations and nonspecific radiological features. Most patients with LPD remain asymptomatic. Symptoms, if any, are nonspecific and include abdominal pain and discomfort, bloating, or abdominal masses that may lead to intestinal obstruction⁹. In the case presented, the patient presented abdominal pain due to compression.

Ultrasound, computerized axial tomography and nuclear magnetic resonance have been described as useful elements in preoperative diagnosis. The typical images are solid nodules with regular contours and variable size scattered on the peritoneal surfaces, which can be confused with peritoneal carcinomatosis or gastrointestinal tumors due to similar image characteristics¹⁰. The definitive diagnosis is histological, and confirms in our case that the nodules are formed mainly by muscle fibers and fibroblasts, without atypia or necrosis. The differential diagnosis includes leiomyosarcoma, mesothelioma, tuberculosis, lymphoma and peritoneal carcinomatosis¹¹. Intraoperatively, LPD presents as multiple round nodules, ranging in size from several millimeters to centimeters, and can be detected on any peritoneal surface or omentum in the abdominal cavity, small or large intestine, mesentery, and retroperitoneum¹².

There are few data on the most appropriate treatment for LPD. Recently, determining therapy according to the patient’s age, symptoms, and desire to have children has been proposed. For women with reproductive desire, hormone therapy with gonadotropin-releasing hormone injection, aromatase inhibitor, or selective progesterone receptor modulator is usually the first-line treatment option. This approach is also preferred in the prevention of postoperative recurrence¹³-¹⁴. For women without reproductive desire, the best alternative may be a more extensive surgical procedure with total abdominal hysterectomy, bilateral salpingo-oophorectomy, omentectomy, myomectomy, and excision of the nodes¹⁵.

In this case, given the size of the nodules, the exacerbation of the symptoms and the patient’s clinical worsening, it was decided to perform surgical treatment.

Conclusion

LPD is a rare clinical condition, it is mainly associated with a history of minimally invasive uterine myomectomy or hysterectomy for myomatosis. The LPD should prefer the differential diagnoses of women with disseminated intra-abdominal or pelvic tumors, especially those with a history of gynecologic surgery. The case presented is of interest because it represents an infrequent entity that can unequivocally simulate peritoneal carcinomatosis. Surgery remains the main therapeutic weapon in symptomatic cases. Surgeons’ knowledge of this rare condition is essential to establish a correct diagnosis and ensure proper treatment.