Case Report
Copyright ©The Author(s) 2019. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Obstet Gynecol. Dec 27, 2019; 8(2): 13-24
Published online Dec 27, 2019. doi: 10.5317/wjog.v8.i2.13
Primary extragenital mixed malignant Mullerian tumour presenting as a painful splenic mass: A case report and review of the literature
Allan Mun Fai Kwok
Allan Mun Fai Kwok, Department of Surgery, Wollongong Hospital, Wollongong, NSW 2500, Australia
Author contributions: Kwok AMF was involved in the clinical care of the patient’s initial admission, reviewed the literature and drafted and reviewed the manuscript.
Informed consent statement: Consent was obtained directly from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflicts of interest.
CARE Checklist (2016) statement: The authors have read the CARE checklist (2016), and the manuscript was prepared and revised according to the CARE checklist (2016).
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Corresponding author: Allan Mun Fai Kwok, BSc MBBS, FRACS, General Surgeon, Department of Surgery, Wollongong Hospital, Locked Bag 8808, South Coast Mail Centre, Wollongong, NSW 2521, Australia.
Received: August 21, 2019
Peer-review started: August 21, 2019
First decision: November 12, 2019
Revised: November 18, 2019
Accepted: December 14, 2019
Article in press: December 14, 2019
Published online: December 27, 2019

Mixed malignant Mullerian tumours (MMMTs) are highly aggressive cancers that present at a late stage and are associated with a poor long-term prognosis. They are characterised by the presence of both epithelial and sarcomatous tissue types on histological examination and hence are known as biphasic tumours or carcinosarcomas. MMMTs almost always arise in the female genital tract (most commonly the uterus) but extragenital sites are also possible. Treatment options are limited and usually consists of surgery and adjuvant chemotherapy.


A 74-year-old woman presented with a 1-wk history of worsening left upper quadrant abdominal pain and a perisplenic mass on computed tomography (CT) scan. Features on imaging raised the possibility of a malignant process but were not diagnostic. Splenectomy was performed and histology confirmed the presence of a mixed malignant Mullerian tumour. A subsequent pelvic ultrasound identified another heterogenous cystic mass in the Pouch of Douglas without evidence of primary pathology in the uterus or adnexa. A second operation was performed for this with curative intent but previously-unknown widespread metastases were encountered at laparotomy. A biopsy of the pelvic mass confirmed it was also a MMMT with identical histological features to the perisplenic mass. There was no clinical or histological evidence of the MMMT having arisen primarily from the female genital tract. The patient subsequently suffered multi-organ failure and was palliated, succumbing to death on the 19th post-operative day.


Primary extragenital MMMT arising from the spleen or peritoneum represents an atypical form of an already rare gynaecological tumour.

Keywords: Mixed malignant Mullerian tumour, Carcinosarcoma, Extragenital, Spleen, Metastatic, Case report

Core tip: Mixed malignant Mullerian tumours (MMMTs) are rare tumours that normally arise from the female genital tract, with the uterus and cervix being the most common sites. Very rarely they may arise from extragenital sites such as the spleen or the peritoneum. We describe the case of a 74-year-old woman who presented with left-sided upper abdominal pain due to a MMMT involving her spleen. Further investigations revealed additional extensive disease in the pelvis and there was evidence that it specifically did not arise from the genital tract. This case represents a highly atypical presentation of an already rare gynaecological tumour.