Published online Dec 27, 2019. doi: 10.5317/wjog.v8.i2.13
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
Processing time: 131 Days and 2.5 Hours
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.
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.