Cervical Mullerian adenosarcoma with heterologous sarcomatous overgrowth: a fourth case and review of literature

• Published in: BMC cancer Vol. 11; no. 1; p. 236
• Main Authors: Patrelli, Tito Silvio, Gizzo, Salvatore, Di Gangi, Stefania, Guidi, Giorgia, Rondinelli, Mario, Nardelli, Giovanni Battista
• Format: Journal Article
• Language: English
• Published: England BioMed Central Ltd 11.06.2011; BioMed Central; BMC
• Subjects: Adenosarcoma - drug therapy; Adenosarcoma - pathology; Adenosarcoma - radiotherapy; Adenosarcoma - surgery; Adjuvant treatment; Aged; Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Appendectomy; Cancer; Care and treatment; Case Report; Case studies; Chemotherapy, Adjuvant; Cisplatin - administration & dosage; Diagnosis; Female; Health aspects; Hormone therapy; Humans; Hysterectomy; Ifosfamide - administration & dosage; Lymph Node Excision; Mixed Tumor, Mullerian - drug therapy; Mixed Tumor, Mullerian - pathology; Mixed Tumor, Mullerian - radiotherapy; Mixed Tumor, Mullerian - surgery; Neoplasm Invasiveness; Omentum - pathology; Ovariectomy; Prognosis; Radiotherapy, Adjuvant; Remission Induction; Salpingectomy; Sarcoma; Ultrasound imaging; Uterine Cervical Neoplasms - drug therapy; Uterine Cervical Neoplasms - pathology; Uterine Cervical Neoplasms - radiotherapy; Uterine Cervical Neoplasms - surgery; Italy
• ISSN: 1471-2407; 1471-2407
• DOI: 10.1186/1471-2407-11-236

Uterine sarcomas are relatively rare tumors that account for approximately 1-3% of female genital tract malignancies and between 4-9% of uterine cancers. Less than 8% of all cases are Mullerian adenosarcoma, a distinctive uterine neoplasm characterized by a benign, but occasionally atypical, epithelial and a malignant, usually low-grade, stromal component, both of which should be integral and neoplastic constituents of the tumor. Mullerian adenosarcoma with sarcomatous overgrowth (MASO) is a very aggressive variant, associated with post-operative recurrence, metastases, even when diagnosed in early stage. We present a fourth MASO case derived from uterine cervix in a 72-year-old woman with metrorrhagia and a polypoid mass protruding through the cervical ostium. Total abdominal hysterectomy, bilateral salpingo-oophorectomy, systematic pelvic lymph node dissection, omental biopsy and appendectomy were performed. Surgery treatment was associated with adjuvant whole-pelvis radiation (45 Gy) and adjuvant chemotherapy (cisplatin/ifosfamide). After nine months of follow up, the patient was free of tumor. The rarity of MASO of the cervix involves a management difficult. Most authors recommend total abdominal hysterectomy, usually accompanied by bilateral salpingo-oophorectomy. There is no common agreement on staging by lymphadenectomy during primary surgery and adjuvant chemo-radio therapy.