Primary extragenital mixed malignant Mullerian tumour presenting as a painful splenic mass:A case report and review of the literature

• 作者: Allan Mun Fai Kwok
• 来源期刊: 世界妇产科杂志
• 学科: 医学
• 关键词: MIXED MALIGNANT Mullerian TUMOUR CARCINOSARCOMA Extragenital Spleen Metastatic Case report
• 年，卷（期）: 2019,（2）
• 页码范围: 13-24
• 页数: 12页
• 分类号: R73

摘要

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