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[Retroperitoneal mixed malignant mullerian tumor : exceptional localisation and prognosis]
G El Hachem1, C Jungels1, D Larsimont2
1Clinique d'Oncologie médicale, Institut Jules Bordet, ULB.
Revue Medicale De Bruxelles
|July 3, 2018
Summary
This case study highlights a rare extra-genital retroperitoneal carcinosarcoma. Aggressive surgical treatment and adjuvant chemotherapy led to a record 113-month disease-free survival for this challenging mixed malignant mullerian tumor.
Area of Science:
- Gynecologic Oncology
- Surgical Oncology
- Rare Cancers
Background:
- Primary retroperitoneal carcinosarcoma, a type of mixed malignant mullerian tumor (MMMT), is exceptionally rare.
- These aggressive neoplasms typically originate from the female genital tract, making a primary retroperitoneal location highly unusual.
Observation:
- A 63-year-old female presented with a heterologous, extra-genital, retroperitoneal carcinosarcoma.
- Malignant cells were detected in ascitic fluid, with extra-ovarian metastatic implants observed.
Findings:
- The patient underwent radical surgical resection, including tumor removal, omentectomy, hysterectomy, bilateral salpingo-oophorectomy, and lymphadenectomy.
- Adjuvant chemotherapy with Carboplatin and Paclitaxel resulted in a complete clinical and radiological remission lasting 113 months.
- This represents the longest reported disease-free survival for an extra-genital retroperitoneal MMMT.
Implications:
- The case underscores the critical role of comprehensive surgical management for rare retroperitoneal tumors.
- Current evidence-based guidelines for systemic therapy in such cases are lacking, necessitating further research.
- Long-term remission is achievable with aggressive multimodal treatment strategies.
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