J of IMAB. 2019 Jul-Sep;25(3) https://www.journal-imab-bg.org 2671

Original article

UTERINE AND OVARIAN SARCOMAS: CLINICALAND HISTOPATHOLOGICAL CHARACTERIS-TICS

Tihomir Totev1, Desislava Kiprova2, Nadezhda Hinkova2

1) Institute of Science and Research, Medical University - Pleven, Bulgaria.2) St. Marina University Hospital, Medical University - Pleven, Bulgaria.

Journal of IMAB - Annual Proceeding (Scientific Papers). 2019 Jul-Sep;25(3)Journal of IMABISSN: 1312-773Xhttps://www.journal-imab-bg.org

ABSTRACTPurpose: To investigate the clinical and histopatho-

logical characteristics of patients with uterine and ovariansarcomas and analyse surgical operations already per-formed.

Material and Methods: The retrospective study in-cluded 17 patients with uterine and 2 patients with ovar-ian sarcomas, who were diagnosed and operated on for threeyears at St. Marina University Hospital in Pleven, Bulgaria.

Results: In the group of uterine sarcomas, the high-est incidence was that of leiomyosarcomas (53.3%), fol-lowed by endometrial stromal sarcomas (33.3%) and thehomologous carcinosarcomas (13.3%). Most of the patientswere diagnosed in the first clinical stage (73.3%), and themost common surgery performed was total abdominal hys-terectomy with salpingo-oophorectomy, with or withoutomentectomy (53.3%). The two patients with ovarian tu-mours were histologically diagnosed with carcinosarcoma.

Conclusions: Genital sarcomas are a heterogeneousgroup of rare malignant diseases with poor prognoses. Earlydetection, adequate histological diagnosis and staging areof utmost importance for control.

Keywords: uterine sarcoma, ovarian sarcoma

INTRODUCTIONSarcomas account for about 5% of uterine neo-

plasms [1]. The most common histological ones are carci-nosarcoma (50%), leiomyosarcoma (30%) and endometrialstromal sarcoma (10%) [2]. Carcinosarcomas are a variablemixture containing malignant epithelial and malignantmesenchymal components. These malignancies can occurin any part of the genital tract, though the most commonlocation is in the uterus [3]. The epithelial component canbe endometrioid, clear- cell, serous or squamous. The ma-lignant mesenchymal components defined as homologousor heterologous. If the sarcomatous part contains elementsof the Müllerian system (endometrial stromal sarcoma, leio-myosarcoma, and other), it is classified as homologous. The

heterologous ones contain malignant tissues such ascartilaginous, bone, and transversely striated muscle tissue,which are not generally found in the genital system. It issuggested that carcinosarcomas belong to the group of car-cinomas and be treated as such, rather than as sarcomas, asthey are now categorized in the current FIGO classification[4]. Sarcomas account for ≤1% - 4% of all ovarian tumors[5]. The etiology and pathogenesis of these mesenchymalneoplasms remain largely unknown yet, and any subtypeis characterized by a variety of risk factors, specific geneticaberrations, clinical courseð, staging and prognosis.

MATERIALS AND METHODSPatientsThis retrospective study included 17 patients with

uterine sarcomas, and 2 patients with ovarian sarcomas, di-agnosed and operated on between July 2015 and July 2018at St Marina University Hospital in Pleven, Bulgaria. Themean age of the patients with uterine sarcomas was 55.5years (age range 40-76), and ten of them were in meno-pause. They accounted for 6.3% of all patients operatedon for malignant diseases of the uterus at the clinic. Thetwo patients with ovarian sarcomas were 14 and 50 yearsold and were 0.8% of all patients treated for malignant dis-eases of the ovaries. We assigned the stage of uterine sar-comas using the FIGO 2009 system. Ovarian tumors werestaged according to FIGO 2014.

Statistical analysisTo evaluate the results, we applied the descriptive

method.

RESULTSOf the patients with uterine sarcomas, 11 were ad-

mitted for primary surgical treatment, and six because ofrecurrences or need for additional surgery. Two of themwere only diagnosed by testing abrasion/biopsy but werenot operated on at the clinic. The rest of the patients un-derwent surgical operations as follows:

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The mean operating time (incision time - skin clo-sure time) was 125 minutes, range 65-420 minutes. No sig-nificant complications were found during the 30-day post-

Table 1. Major surgical interventions performed.

Cases (n) Percentage (%)

TAH + adnexectomy +/- omentectomy 8 53.2%

Extirpation of recurrent tumor 3 20%

Radical hysterectomy with lymph node dissection 2 13.3%

Extended surgery 2 13.3%

Distribution by stages was as follows:

Fig. 2. Distribution of uterine sarcomas by stage (number of cases and percentage).

Fig. 1. Histological types of uterine sarcomas, distributed by number and percentage.

operative follow-up. The histological type was defined bycomprehensive histopathological and immunohistochemi-cal investigations. The following types were identified:

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Fig. 3. Large uterine sarcoma, occupying the ab-dominal cavity.

Fig. 5. The structures shown above were EMA posi-tive and demonstrated proliferative activity (Ki67>80%).

DISCUSSIONSarcomas and carcinosarcomas of the genital tract

are rare neoplasms, and most studies cover a small numberof cases. A large-scale 10-year study, including 40 ovariancarcinosarcomas, revealed an incidence of 1.3% of suchtumors in the group of the malignant ovarian tumors [6].Most often, the cases diagnosed are in FIGO stage III-IV[7]. The two cases in our study were with carcinosarcomas.The case of the 14-year-old girl, diagnosed shortly aftermenarche is unusual for her age – the disease is generallyseen between the age of 60 and 70 years. The main pur-pose of surgical treatment is to remove all visible tumormasses and full surgical staging. Surgery usually includesperitoneal wash cytology, total hysterectomy with bilateraladnexectomy, omentectomy. Pelvic and para-aortic lymphnode dissection, peritoneal pelvic, paracolic and subdia-phragmatic biopsy are also performed. In advanced cases(FIGO III-IV), an as extensive as possible cytoreduction,sometimes with greater interventions like bowel resectionand splenectomy is needed. Adjuvant chemotherapy is ad-ministered in all cases, usually including platinum prepa-rations and taxanes. In cases of tumor resistance and recur-rence, alternative treatment regimens include Gemcitabine,Topotecan, Etoposide, Ifosfamide, Doxorubicin. The roleof hyperthermic intraperitoneal chemotherapy (HIPEC) forthe treatment of ovarian carcinosarcomas is still subject todebate. Irrespective of the sarcomatous component, the ne-cessity for radiotherapy has not been established. Targetradiotherapy may be indicated in individual patients,e.g.those with bone metastases.

Uterine carcinosarcomas are the most frequentsubtype and usually occur in 50- to 70-year-old patients.Unlike these, leiomyosarcomas are seen at an earlier age(40-55 years) and are usually limited to the uterus (stageI) [2]. Primary surgical treatment for all types of uterinesarcomas includes TAH with bilateral adnexectomy withor without omentectomy and lymph node dissection. Adju-vant chemotherapy depends on the staging, the histologi-cal type, and the degree of differentiation and the pres-

The patients with ovarian sarcomas were admittedfor primary surgery. After informed consent and an ex-pressed request, the 14-year-old patient with stage IIIC un-derwent adnexectomy and additional staging procedures.The other patient with stage IC underwent a standard totalabdominal hysterectomy and omentectomy. There were noserious postoperative complications, and histology re-vealed homologous carcinosarcoma in both cases.

Fig. 4. Ovarian carcinosarcoma. Anastomosing atypi-cal glandules and solid nests, located among edematousatypical stroma (H&E).

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2. Kobayashi H, Uekuri C, AkasakaJ, Ito F, Shigemitsu A, Koike N, et al.The biology of uterine sarcomas: A re-view and update. Mol Clin Oncol.2013 Jul;1(4):599-609. [PubMed][Crossref]

3. Spaziani E, Picchio M, PetrozzaV, Briganti M, Ceci F, Di Filippo A. etal. Carcinosarcoma of the uterus: acase report and review of the literature.Eur J Gynaecol Oncol. 2008; 29(5):531-4. [PubMed]

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ence of lymphovascular invasion. For patients with highergrading, a combination of Ifosfamide and Paclitaxel is rec-ommended [8]. Effective chemotherapy for leiomyosar-coma includes Doxorubicin, Ifosfamide, Gemcitabine andPaclitaxel [9]. Endometrial stromal sarcoma has positivehormonal receptors and treatment with progestins andaromatase inhibitors is often administered. Postoperativeradiotherapy in the early stages of genital sarcomas isaimed to improve local control and includes external pel-vic radiotherapy, followed by intravaginal brachytherapy[2].

The analysis of the histological types of uterine sar-comas in our study showed the highest prevalence of leio-myosarcomas (53.3%), followed by endometrial and ho-mologous carcinosarcomas. The dominant group was that

of tumors in stage I (73.3%). Comparison by staging thedifferent subtypes is not applicable since they have differ-ent stages by FIGO 2009. The surgical operations performedvary from extirpation of recurrence to radical hysterectomyand interventions extended to other abdominal organs. Insome of the cases, multidisciplinary teams were needed. Theprocedure most commonly applied was TAH with bilateraladnexectomy, with or without omentectomy (53.3%).

CONCLUSIONGenital sarcomas are a heterogeneous group of rare

malignant diseases with poor prognoses. Early detection,accurate histological diagnosis and staging are crucial forcontrolling the condition. When treatment is carried outin specialized centers, it is more successful.

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Corresponding Author:Tihomir P. Totev MD, PhDSt. Marina University Hospital, Department of GynecologyBulgarska aviatsia str., Pleven 5800, BulgariaE-mail: t.totev@mail.bg

Please cite this article as: Totev T, Kiprova D, Hinkova N. Uterine and ovarian sarcomas: clinical and histopathologicalcharacteristics. J of IMAB. 2019 Jul-Sep;25(3):2671-2674. DOI: https://doi.org/10.5272/jimab.2019253.2671

Received: 14/02/2019; Published online: 27/08/2019