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Recurrent leiomyomatosis peritonealis disseminata
  1. Emmanuel Sanchez Diaz1,
  2. Gabriel Jaime Rendon Pereira2,
  3. Rene Pareja3,
  4. Oscar German Palacios Rodriguez4,
  5. Carlos Andres Serna Ortíz5,6 and
  6. Claudia Patricia Huertas Duran7,8
  1. 1 Ob Gyn third year resident, Universidad Pontificia Bolivariana, Medellin, Colombia
  2. 2 Gynecologic Oncologist, Clínica Las Américas AUNA, Medellín, Antioquia, Colombia
  3. 3 Gynecologic Oncology, Clinica ASTORGA and Instituto Nacional de Cancerología, Medellín and Bogotá, Colombia
  4. 4 Hepatobiliary surgeon, Clínica ASTORGA and Clínica Las Américas AUNA, Medellín, Colombia
  5. 5 CES University CES Clinic, Medellin, Colombia
  6. 6 Pathology Department, Clinica Las Américas AUNA, Medellín, Colombia
  7. 7 Radiology, The University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA
  8. 8 Department of Radiology, Clinica Las Américas AUNA, Medellín, Colombia
  1. Correspondence to Dr Emmanuel Sanchez Diaz, Obgyn, Universidad Pontificia Bolivariana, Medellin, Antioquia, Carrera 83 C 33 107 050032, Colombia; emmanuel4416{at}

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Clinical Case Overview

A 33-year-old woman with a personal history of uterine leiomyomas, disseminated peritoneal leiomyomatosis, and a borderline peritoneal tumor, presented to the emergency department in November 2022 complaining of acute onset pain located in the left hypochondrium and flank for 48 hours, with no other associated symptoms. Her medical history included three previous surgeries. The first surgery was a laparoscopic myomectomy performed in April 2017, but the surgical description was not available because it was performed by a gynecologist in an external institution (there was no information on whether morcellation was required). The findings and final pathology report were not available, but the patient was informed regarding the benign nature of the disease.

Subsequently, surveillance MRI in July 2018 showed free fluid in the pelvis, peritoneal implants, a 5×3 cm mass in the cul-de-sac, and uterine myomatosis. At that time, she underwent laparoscopy where gross findings included a left ovarian tumor with a coralliform appearance of approximately 5 cm, omentum adhesion to the previous myomectomy bed and to the pelvic peritoneum, normal right ovary, uterus with a right intraligamentary myoma extending through the right pillar of the bladder without other lesions, free fluid in the pelvis, tumor implant in the omentum, right diaphragmatic dome and a mirror lesion in the liver.

In addition, another retroperitoneal solid tumor at the level of the aortic bifurcation, of approximately 10 cm with a myomatous appearance, was described, with myomatosis implants in both fallopian tubes, right pararectal space, right parietocolic gutter, right diaphragmatic dome, round hepatic ligament and lesser omentum in the lesser curvature of the stomach (7 cm), and multiple lesions with the same characteristics at the level of the greater omentum. A frozen section of the left ovarian mass showed a serous borderline peritoneal tumor and thus resection of the left ovarian tumor, unilateral salpingectomy, myomectomy of multiple peritoneal leiomyomas, resection of a retroperitoneal leiomyoma, total omentectomy, and enterorrhaphy were performed, all by a laparoscopic approach, in July 2018. The final pathology confirmed an International Federation of Gynecology and Obstetrics (FIGO) stage IC borderline tumor in the left ovary, non-invasive implants in the peritoneum and omentum, as well as numerous leiomyomata (Figure 1).

Figure 1

Gross findings of laparoscopic surgery in 2018. (A) Left ovarian tumor with a coralliform appearance of approximately 5 cm (arrow), normal right ovary, and retroperitoneal tumor (asterisk). (B) Leiomyoma located in the omentum (arrow). (C) Retroperitoneal tumor of solid myomatous appearance (arrow). (D) Omentum adhesion to the previous myomectomy bed (arrow) and with myomatosis implants in the omentum (asterisk).

Dr Pareja: What Would be the Recommendation for the Borderline Peritoneal Tumor and Non-invasive Implants?

Serous borderline ovarian tumors do not cause destructive stromal invasion but can be associated with extraovarian implants. These can be classified according to their histological appearance, in non-invasive and invasive implants, taking into account the presence of invasion of underlying normal tissue, micropapillary architecture, and solid epithelial nests surrounded by clefts. This classification may determine the prognosis and treatment. Invasive implants are in line with the serous borderline tumor biological behavior and thus were redefined by the 2014 World Health Organization classification as peritoneal low grade serous carcinoma whereas non-invasive implants follow a different biological course.1 2

Regarding the characteristic histological findings of invasive implants, the micropapillary pattern has been associated with higher recurrence rates. However, when it is found alone, it does not behave as an independent prognostic factor. Furthermore, its presence determines the requirement for restaging surgery if the abdominal cavity was not fully evaluated in the initial procedure.2 3

Surgery (including ovarian cancer debulking surgery or fertility sparing surgery) is the primary treatment for borderline epithelial tumors; the presence of invasive peritoneal implants determines the requirement of additional therapies due to the previously mentioned higher rates of relapse (>50%). Borderline disease with invasive implants may be treated as low grade serous disease, with observation for tumor stages IA and IB and systemic therapy with chemotherapy or hormonal therapy for higher stages. For patients with non-invasive implants, observation is the standard of care after surgery.4 5

Based on these recommendations, the patient was offered surgical management for primary cytoreduction, as described. Considering that the pathology reported non-invasive implants, the patient did not require additional therapies, and a periodic follow-up strategy based on clinical and imaging follow-up was established.

Dr Pareja: What are the Criteria for Evaluation of Presumed Leiomyoma When Deciding Open Versus Minimally Invasive Surgery?

Uterine leiomyomas are frequent in women of reproductive age and are diagnosed in approximately 25–30% of women. Laparoscopic surgery remains the standard approach in the management of leiomyomas. There are currently no strict criteria in selecting the surgical approach for a suspected uterine leiomyoma, or for leiomyomatous lesions of extrauterine location. However, a thorough preoperative evaluation should be performed, including a complete medical history, comprehensive physical examination, and imaging, usually transvaginal ultrasound, keeping in mind that leiomyosarcomas have a low incidence of <1 per 1 00 000 women. Therefore, the choice of the imaging technique should be based on clinical suspicion. Currently, there are no reliable diagnostic tools that allow differentiating a classic leiomyoma from a leiomyosarcoma, but MRI has the best performance, especially when diffusion weighted images are obtained, with sensitivity and specificity of 100%. Transvaginal ultrasound has a detection rate for uterine sarcoma of 80.0% and a negative predictive value of 97.6%.6

Also, it is essential to consider other factors, such as the expertise of the surgeon, location, number, and size of the uterine leiomyomas. Indications for consideration of the open approach include lesion size >8–12 cm, diffuse leiomyomatosis, and comorbidities. The minimally invasive approach is increasingly preferred to treat leiomyomas due to the already known advantages in terms of rapid recovery, less bleeding, and postoperative pain, and also because more and more women prefer fertility sparing treatments. However, due to safety concerns of morcellation in relation to the recurrence or potential spread of inadvertent malignant lesions, large lesions represent a challenge for this approach. A laparoscopic radiofrequency ablation technique has recently emerged, but published studies are limited to patients with fewer than six leiomyomas and lesions <10 cm.7

An important factor to consider in this case is the risk of recurrence of leiomyomas in relation to the surgical approach chosen for myomectomy. Despite the fact that no differences have been found in the recurrence rate when comparing the open and laparoscopic techniques, when ≤5 leiomyomas are resected, the recurrence rate is higher with the minimally invasive approach for >5 leiomyomas.8

In November 2018, the patient became pregnant, and in August 2019, an elective cesarean section was scheduled due to the history of uterine scar secondary to the previous myomectomy. Details of the surgery are not available as the patient did not provide a medical history and the procedure was carried out at another institution. According to the patient, during surgery, a uterine leiomyoma was identified and a myomectomy was performed. The surgical findings or final pathology reports were not available. However, according to the patient, the findings were consistent with leiomyomatous tissue. The patient was followed periodically, with transvaginal pelvic ultrasound in 2019 that reported no pathological findings. An MRI of the abdomen and pelvis in 2020 showed a normal result. Since then, follow-up was abandoned due to the COVID-19 pandemic, until 2022 when clinical and imaging follow-up was restarted.

During the initial consultation with our team, the results of the patient's MRI, performed in September 2022, were reviewed, which showed a hypointense nodular lesion in the splenorenal space on T2 and T of 4 cm in the craniocaudal axis, suggestive of intraperitoneal myoma, without other intra-abdominal myomas (Figure 2). Physical examination revealed an Eastern Cooperative Oncology Group (ECOG) performance status of 0 and pain on palpation in the left hypochondrium and flank without signs of peritoneal irritation or palpable abdominal mass. The pelvic examination was normal. A CT scan of the abdomen and pelvis was obtained.

Figure 2

MRI in September 2022. Top row shows simple T2 sequences (A) Coronal section T2 haste sequence, with round, solid, hypointense lesion on T2, without areas of cystic degeneration or calcifications (arrow). (B) T2 fat sat sequence without suppression of fat. Half row shows T1 weighted sequences with a solid, rounded, hypointense lesion in simple phase, with gradual uptake of contrast medium in dynamic sequences (arrow). (C) Pre-contrast. (D) Arterial phase. (E) Portal phase. (F) Late arterial phase. Bottom row shows functional MRI sequences with no obvious restriction (arrow). (G) Diffusion sequence. (H) Apparent diffusion coefficient sequence.

Dr Huertas Duran: Please Describe the Findings on MRI Images

A contrasted tomography of the abdomen and pelvis was requested, which showed a solid 3.5×4.1×4.4 cm heterogeneous mass in the splenorenal space, which did not change its attenuation between the arterial and portal phases. There was adjacent laminar fluid thickening of the lateroconal fascia. This lesion was not present in a previous image (2018). A solid lesion adjacent to the upper pole of the spleen, measuring 1.5×1.7×1.1 cm, which was not observed in the September 2022 MRI, was reported, and another one with a solid density, in the upper pole of the spleen, measuring 1.4×1.5×1.3 cm, which increased in size compared with the 2018 CT scan. Lastly, there were two enlarged lymph nodes, one in the right common iliac chain and one in the left external iliac chain, both measuring 10 mm.

For better characterization of the masses and optimal surgical planning, the next day an abdominopelvic MRI was ordered. This showed an increase in the size of the smooth muscle soft tissue lesion located in the splenorenal space, measuring 5.0 cm in its greatest diameter (September 2022, 3.3 cm). The lesion was associated with inflammatory changes and free fluid, compromising the retroperitoneum in the perirenal space, extending towards the left paracolic gutter and lateroconal fascia. The findings were suggestive of retroperitoneal leiomyoma infarction, and a solid 1.7 cm lesion was identified between the spleen and the left liver lobe, compatible with tumors of fibrous tissue (leiomyomatosis) (Figure 3). The patient had normal levels of carcinoembryonic antigen (CEA) and CA125 (<1.73 U/mL and 16.3 µg/L, respectively).

Figure 3

November 2022. Abdomen and pelvis MRI (A, B). The lesion is located in the splenorenal space (arrow) associated with inflammatory changes and free fluid, compromising the retroperitoneum in the perirenal space, suggestive of retroperitoneal leiomyoma infarction (A) T1 sequence with contrast in the portal phase axial plane. (B) T2 haste sequence, coronal plane. (C, D) Solid appearance lesion located between the spleen and left liver lobe, measuring 17 mm. (C) T1 sequence with contrast in the portal phase axial plane. (D) T2 haste sequence, coronal plane.

Dr Pareja: Based on the Information Provided, What was the Differential Diagnosis and What was the Discussion Regarding Surgical Approach?

In the scenario of a patient with a history of multiple previous myomectomies and imaging findings highly suggestive of smooth muscle lesions, peritoneal leiomyomatosis was considered the most probable diagnosis by the treating team. However, within the differential diagnoses, it is important to consider gastrointestinal stromal tumor, primary peritoneal leiomyosarcoma, or angiolipomas.9 10

During the discussion regarding the surgical approach, several factors were considered, such as the location of the lesions and their anatomical relationships with the liver, spleen, and vascular structures of the upper abdomen, as well as the experience of the surgeons in the minimally invasive technique. There is currently no standard treatment strategy for cases of disseminated peritoneal myomatosis, but some authors have suggested the laparoscopic approach due to its rapid recovery and less possibility of trauma. Given that the lesion had infarction and that the patient was symptomatic, primary surgical management was chosen. It was also agreed that the entire surgical specimen should be extracted to avoid morcellation and to reduce the risk of recurrence due to seeding in the peritoneal cavity.11

The patient underwent a laparoscopic posterior radical antegrade modular pancreatosplenectomy, performed by a gynecologic oncologist and hepatobiliary surgeon in December 2022. The findings revealed involvement of the spleen up to the hilum, the distal pancreas, and the left adrenal gland by two masses, one located between the left hepatic lobe and the spleen of approximately 2 cm and another located inferior to the spleen, measuring 5 cm. Otherwise, there was no free fluid in the pelvic cavity or left adnexa. The right tube and ovary were normal. No lesions were identified in the pelvic peritoneum or elsewhere. Due to the specimen size (approximately 20 cm), it was necessary to perform a Pfannenstiel’s incision for the extraction of the surgical specimen. It was decided to schedule the surgery as a priority considering that the patient was symptomatic secondary to myoma infarction. The procedure was performed on a day when there was no frozen biopsy available, and the length of surgery was approximately 240 min, including the hepatobiliary surgery intervention and the laparotomy for surgical specimen extraction, with an estimated blood loss of 100 mL.

Dr Serna Ortiz: Please Provide Details Describing Pathology

The final pathology showed spindle cell proliferation and hemorrhagic infarction. The resected spleen and pancreas fragments had normal histological characteristics. Immunohistochemistry showed: smooth muscle actin, positive; demine, positive; H caldesmon, positive; S100, negative; SOX 10, negative; CD34, negative; CD117, negative; DOG1, negative; CDK4, negative; MDM2, negative; estrogen receptors, positive; progesterone receptors, positive, which corresponds to a leiomyoma of gynecological origin ( Figure 4 ).

Figure 4

Histopathological findings. (A) Bundles of elongated benign cells (hematoxylin and eosin, ×40 magnification). (B) H caldesmon expression was positive. (C) Estrogen receptor expression was positive. (D) Progesterone receptor expression was positive.

In the immediate postoperative period, the patient required observation in the intensive care unit for 24 hours because the duration of the procedure was longer than expected. Due to the prolonged hospitalization, extent of the surgery, and to provide advice regarding the expansion of the vaccination scheme and necessary precautions for splenectomized patients, a multidisciplinary approach was offered, with assessment by physical therapy and rehabilitation, infectious diseases, and psychology. The patient was discharged 72 hours after surgery. She was subsequently readmitted with pulmonary thromboembolism without ventricular dysfunction or hypoxemia. She required a hospital stay of 48 hours, and outpatient treatment with low molecular weight heparin anticoagulation was started.

Dr Pareja: What was the Recommendation for the Patient, and Provide Details Regarding Surveillance?

The patient was offered a periodic follow-up strategy, with appointments on the first month, at at 3, 6, and 12 months, with an approach based on clinical assessment and MRI. Positron emission tomography is not routinely recommended since disseminated peritoneal myomatosis does not routinely uptake fludeoxyglucose, but it may be of use in the setting of identifying malignant peritoneal disease.10

Closing Summary

Disseminated peritoneal myomatosis is an infrequent condition with a heterogeneous clinical picture that can range from asymptomatic patients to abdominal pain symptoms secondary to associated complications. Within the pathogenesis of this condition are found the morcellation of the surgical specimens of myomectomy or hysterectomy and hormonal influences, which is why these factors should be controlled when possible, avoiding morcellation and selecting a proper surgical approach considering the size of the uterus or the myomas and its location.

For diagnosis, it is essential to carry out an approach with an adequate clinical history, complete physical examination, and imaging, such as MRI, that allows characterization of the lesion, and determination of its nature. Irregular margins, tumor heterogeneity, and high signal intensity are characteristics suggestive of sarcoma, which is an important differential diagnosis.

Histopathologic evaluation is the gold standard, where a wide range of cytologic atypia, from extremely well differentiated to highly anaplastic cells, can be identified as characteristic of leiomyosarcoma while leiomyomas are composed of bundles of smooth muscle cells that resemble the uninvolved myometrium. The distinction from leiomyoma is based on nuclear atypia, mitotic index, and tumor necrosis. Leiomyomas are not prone to malignant transformation, with a global risk of <1%.11

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  • Twitter @EmmanuelSnche18, @RParejaGineOnco

  • Contributors ESD compiled the case and carried out a review of the literature. CASO prepared the pathology images and provided the histopathological findings, as well as advice regarding the histological diagnosis. CPHD provided the diagnostic images as well as the concept regarding the usefulness of MRI. OGPR and GJRP provided the images and surgical details. RP led the discussion of the case regarding clinical and surgical decisions.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial, or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; internally peer reviewed.