
==== Front
Int J Surg Case Rep
Int J Surg Case Rep
International Journal of Surgery Case Reports
2210-2612
Elsevier

S2210-2612(24)00966-0
10.1016/j.ijscr.2024.110185
110185
Case Report
Disseminated peritoneal leiomyomatosis after laparoscopic myomectomy – A case report
Usenko Olexandr a1
Sydiuk Andrii a1
Voitiv Yaroslav a1
Varga Anita b1
Popov Oleh c1
Makarov Vladyslav Vlad111776@gmail.com
a⁎1
a Department of Thoracoabdominal Surgery, Shalimov's National Institute of Surgery and Transplantology, National Academy of Medical Sciences of Ukraine, Kyiv, Ukraine
b Chief medical officer Shalimov's National Institute of Surgery and Transplantology, Kyiv, Ukraine
c Department of Intensive Care and Anaesthesiology, Shalimov's National Institute of Surgery and Transplantology, National Academy of Medical Sciences of Ukraine, Kyiv, Ukraine
⁎ Corresponding author at: Shalimov's National Institute of Surgery and Transplantology, Department of Thoraco-abdominal surgery, Kyiv, Ukraine. Vlad111776@gmail.com
1 Shalimov's National Institute of Surgery and Transplantology» Kyiv, Ukraine.

16 8 2024
9 2024
16 8 2024
122 1101859 7 2024
10 8 2024
14 8 2024
© 2024 The Authors
2024
https://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Introduction and importance

Disseminated peritoneal leiomyomatosis (DPL) is a rare benign disease, characterized by intraperitoneal dissemination of smooth-muscle tumor masses.

Case presentation

The patient, a 40-year-old, female - presented with complaints of periodic back pain. During the initial workup, a tumor mass was visualized in the pelvis on ultrasound. Further clinical workup included computer tomography (CT) scan and ultrasound-guided fine-needle biopsy. A tumor mass in the right adrenal gland was identified on CT. Pathological examination revealed no signs of malignancy. The multidisciplinary team decided on surgical treatment. Tumor masses were removed from the pelvic cavity, the anterior abdominal wall, and the projection of the right adrenal gland with preservation of the uterus and ovaries due to the patient's wishes. Pathological examination with immunohistochemistry of surgical specimen confirmed diagnosis of DPL.

Clinical discussion

Due to the rarity of DPL there are no standard guidelines for diagnostics and treatment. In many cases, on initial workup, DPL may look like a malignant tumor with intraperitoneal spread, which may lead to an inappropriate choice of treatment.

Conclusion

Differential diagnosis of DPL may be a challenge due to its appearance as a disseminated peritoneal malignancy on CT scans and at laparotomy. A multidisciplinary approach is key to choosing the right clinical course for such patients.

Highlights

• This manuscript describes a case of Disseminated peritoneal leiomyomatosis after laparoscopic myomectomy.

• Diagnostic techniques, treatment course, and results are presented in the paper.

• The patient underwent operative treatment with preservation of uterus and ovaries.

Keywords

Peritoneal disease
Disseminated peritoneal leiomyomatosis
Benign leiomyoma of the pelvis
Abdomen
Multidisciplinary approach
==== Body
pmc1 Introduction

Disseminated peritoneal leiomyomatosis was first described by Taubert et.al in 1965 [1]. DPL is a rare benign disorder with a 2–5 % chance of malignant transformation [2]. There are around 200 cases of DPL reported worldwide [3], and this number is increasing. Defined etiology remains unknown. Some case series show a connection between laparoscopic myomectomy and DPL occurrence, due to iatrogenic intraperitoneal tumor cells spread during morcellation [[4], [5], [6], [7]]. Tumor growth may be accelerated by pregnancy and combined oral contraceptive intake [8,9]. To date, there are no standard clinical guidelines for the management of DPL. By presenting our case we hope to add to further research on disseminated peritoneal leiomyomatosis.

This case was presented following the updated consensus Surgical Case Report (SCARE) guidelines [10].

2 Case presentation

A 40-year-old white female patient with a BMI of 23 presented to the outpatient department with periodic back pain. Past medical history includes a cesarian section 10 years ago. Laparoscopic myomectomy 5 years ago. Open myomectomy with excision of fibromyomas of the anterior abdominal wall 1 year ago. The patient doesn't take any drugs and is a non-smoker. On transabdominal ultrasound – a tumor mass of 10 × 9 cm. was identified in the pelvis. For further clinical workup, the patient was admitted to the department of Thoraco-abdominal surgery. At Shalimov's National Institute of Surgery and Transplantology. A contrast-enhanced CT scan of the thoracic and abdominal cavities was obtained, which showed: Multiple tumor masses in the pelvis, with signs of peritoneal dissemination. Tumor in the projection of the right adrenal gland. Left-sided hydronephrosis due to secondary compression of the ureter (Fig. 1).Fig. 1 Contrast-enhanced CT: Multiple solid neoplasms of the pelvic cavity, peritoneum. Tumor mass in the projection of the right adrenal gland.

Fig. 1

There were no elevated levels of serum tumor markers (Table 1).Table 1 Patients' tumor markers. ROMA index was 1.1.

Table 1Tumor marker	Result	Reference range	Units	
CA 19–9	13.7	<37	u/ml	
CA 125	19.9	0–35	u/ml	
HE 4	22	<70	pmol/l	
AFP	2.0	<8,1	ng/ml	
CEA	1.75	<2,5	ng/ml	

The patient underwent clinical blood and urine tests, which showed normal results. To alleviate the patient's hydronephrosis, a stent was placed into the left ureter using video-assisted cystoscopy. Additionally, an ultrasound-guided fine-needle biopsy of a tumor mass in the pelvis was performed. The pathology report indicated that the findings were consistent with DPL, but it was inconclusive regarding the possibility of malignant transformation into leiomyosarcoma due to the small amount of tissue obtained.

The patient was presented to the tumor board of Shalimov's National Institute of Surgery and Transplantation. Operative treatment with the aim of removal of all tumor tissues was recommended. The patient expressed her wishes to retain the ability to have children after the surgery. Open approach was decided upon due to the size of the tumor, and multiple locations of lesions. Pre-operative preparation included antibiotic prophylaxis 60 min before skin incision and low-molecular heparin for the prophylaxis of thrombotic complications. Iodine–impregnated adhesive drapes were used after skin preparation with chlorhexidine solution. Ultrasound-guided TAP block was administered.

During the surgical exploration, tumor masses were found in the anterior abdominal wall, near the right adrenal gland, on fatty appendages of the sigmoid colon, and in the pelvic cavity alongside the uterus. Additionally, a tumor was observed growing from the posterior wall of the uterus into the retroperitoneal space, causing compression of the left ureter (Fig. 2, Fig. 3).Fig. 2 Tumor growth on the fatty appendages of the sigmoid colon.

Fig. 2

Fig. 3 Tumor mass on the posterior wall of the uterus.

Fig. 3

Biopsy results of the tumor in the right adrenal gland area were consistent with the leiomyoma. As a result of a complex surgical procedure, all tumor masses were removed with the use of diathermy and advanced bipolar energy devices (Fig. 4).Fig. 4 Specimen – 4 large nodes 11, 6, 4, and 3 cm in diameter. Multiple smaller nodules from the anterior abdominal wall.

Fig. 4

A pathology review of the specimens showed that the morphological picture, and immunophenotypes, correspond to disseminated peritoneal leiomyomatosis.

An immunohistochemical study of the specimen was performed:

SmoothMuscleActin (1A4) - a pronounced positive reaction in all tumor cells;

Desmin (D33) - pronounced positive reaction in all tumor cells;

EstrogenReceptor (EP) - positive reaction in tumor cells 70 % 2+;

ProgesteroneReceptor (PgR636) - positive reaction in tumor cells 100 % 3+;

CD34 (QBEnd0) - negative reaction in tumor cells, positive reaction in vascular endothelium;

MDM 2 (BSB-64) - moderate positive nuclear reaction in tumor cells;

CD99 (BSB-9) - positive perinuclear cytoplasmic reaction in tumor cells;

CytokeratinPan (AE1/AE3) - negative reaction;

Melan-A (A103) - negative reaction;

S100 (4C4.9) - negative reaction;

CDIO (56C6) - negative reaction,

Ki-67 (MIB-1) - mainly 0–1 %, individual cells up to 4 %.

After the operation, the patient's recovery went smoothly. The patient received standard postoperative care, including intravenous fluids, adequate pain relief, proton pump inhibitors, and resumed enteral nutrition on the first day after the operation. The stent from the left ureter was removed, and the patient was discharged on the fifth day after the operation. Prolonged action gonadotropin-releasing hormone agonist therapy was prescribed for six months after the surgery. Six months later, a transabdominal ultrasound showed no evidence of neoplastic growth.

3 Discussion

Due to its rarity and the challenge of making a differential diagnosis, a multidisciplinary approach is crucial for the diagnosis and treatment of DPL. The clinical picture of DPL is non-specific. Most patients are asymptomatic and complaints arise - often due to a secondary compression of an otherwise healthy organ by a tumor mass.

Clinical imaging of DPL is characterized by multiple nodes spread across the peritoneal surfaces of the abdomen. On ultrasound, these nodes appear as homogeneous, hypoechoic with well-defined contours and smooth surfaces. Contrast-enhanced CT scans show hypodense nodes with significant, uniform contrast accumulation. However, if degenerative changes such as necrosis or endometrial implantation occur within the tumor mass, the enhancement becomes heterogeneous, and the resulting image may resemble disseminated carcinomatosis. [11] On MRI signal intensity of the lesions is similar due to their homology. Due to the origin of the tumor lesions mostly occur on the surfaces of the ovaries, and ovarian parenchyma is not invaded by a tumor, so follicular signals may be observed on MRI, which may help to differentiate between DPL and ovarian cancer. [12]

In our case magnetic – resonance imaging was not obtained, which is a limitation of this study, the available radiological picture was suggestive of malignant disease due to a secondary tumor location in the projection of the right adrenal gland, which could be explained as a hematogenous metastasis.

Several authors conclude that biopsy and pathology review of the specimen is the “gold standard” for diagnosing DPL [5,6,8,13]. Our decision to start with operative treatment was guided by the need to obtain a bigger volume of specimens to exclude other potentially malignant diagnoses. Open approach was chosen due to the size of the lesions, and their location inside the anterior abdominal wall.

The surgical option of removing all tumor masses with a hysterectomy and bilateral salpingo-oophorectomy is widely described in the literature, in the case series Rosati et al. describe the surgical removal of all tumor masses with preservation of uterus and ovaries in 71 % of their cases [14]. Conservative management of DPL is based on a theory that high levels of female gonadal steroids may accelerate myoma growth [15]. Treatments that reduce estrogen levels, such as: gonadotrophin-releasing hormone agonists and aromatase inhibitors may be used as a first line of treatment in the case of localized disease [16].

In the case of patients with DPL receiving assisted reproductive technology In Vitro Fertilization and Embryo Transfer (IVF-ET). Bu et al. in the literature review describes two cases of rapid DPL progression after IVF-ET. And conclude that IVF-ET technology should avoided in this group of patients [17].

To date, there is no available clinical protocol for the diagnostics and treatment of disseminated peritoneal leiomyomatosis.

4 Conclusions

DPL is a rare benign disease that has the potential for malignisation, and as is may present as a disseminated malignancy on imaging. Biopsy and pathological examination is a key to differential diagnosis between DPL and malignant disease with peritoneal spread. While removing all tumor mass with preservation of the uterus and ovaries is acceptable, patients should be followed up after surgery and consulted before planning a pregnancy. There is a significant risk for DPL recurrence and spread following IVF-ET. Therapeutic options may be reviewed in the case of contained disease.

Consent

Written informed consent was obtained from the patient for publication of this report and accompanying images.

Ethical approval

Written informed consent was obtained from the patient for publication and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Funding

This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

Author contribution

Makarov Vladyslav: Drafted the manuscript.

Olexandr Usenko, Andrii Sydiuk, Yaroslav Voytiv, Anita Varga, Oleh Popov: Managed the patient.

Yaroslav Voytiv: Supervised the writing of the manuscript.

Oleksandr Usenko: Approved the final manuscript.

Registration of Research Studies.

This study does not require registration.

Guarantor

Olexandr Usenko ORCID iD 0000-0003-3866-4286 Director, Head of Department, Department of Thoracoabdominal Surgery, Professor, PhD, MD.

Research registration number

This study does not require registration.

Conflict of interest statement

The authors declare no conflicts of interest related to this study.
==== Refs
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