Malignant rhadboid tumor of the peritoneum, mimicking an advanced ovarian cancer: A case report with literature review

Abstract

Objective. Extra-renal malignant rhabdoid tumor is an uncommon neoplasm, more commonly occurring in pediatric aged patients. Despite current treatment options, prognosis and survival rate are poor. At present, there are only 20 published cases of primary malignant rhadboid tumor of the female genital tract and peritoneum and, due to its rarity, there is no standard therapeutic guideline for appropriate treatment. Methods. We report the case of a young female affected by this extremely rare tumor, who was surgically treated in our gynecologic oncology division and performed a systematic review of the English literature present in PubMed, SCOPUS and Web of Science. The systematic search was performed in agreement with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) and was registered in the International Prospective Register of Systematic Reviews (PROSPERO, no.: CRD 42019138911). Results. Given the rarity of this tumor, only case reports' studies were available on this topic. A narrative description of the clinical characteristics, treatment and patients' status at last follow up has been carried out. Conclusions. We believe that the best treatment option for patients with this type of tumor should be an aggressive debulking surgery in addition to prompt adjuvant treatment, especially in advanced cases.