High grade uterine adenosarcoma with sarcomatous overgrowth in a young woman amenable to primary surgical reduction: A case study and literature review

Abstract

Mullerian adenosarcomas are rare and often low-grade mixed tumors that typically respond well to optimal surgical resection. However, adenosarcoma with sarcomatous overgrowth (ASSO) is a high-grade mixed tumor commonly associated with invasion, metastasis, and a poor prognosis. The health care providers herein report a case study of a patient diagnosed with ASSO who has maintained remission status for 19 months following radical surgical resection alone. The patient, a 24-year-old Caucasian female without significant medical history, initially complained of abdominal fullness, pelvic pressure, altered menses, and unintentional weight loss. A necrotic cervical mass was present on the exam; mass biopsy revealed spindle cell sarcoma with rhabdomyosarcomatous differentiation. The patient underwent exploratory laparotomy, total abdominal hysterectomy, bilateral salpingectomy, radical tumor debulking, and pelvic and periaortic lymph node dissection. Histopathological diagnosis of the resected specimen was consistent with ASSO, limited to 0.7 cm out of 2.0 cm of myometrial thickness, with negative lymph node and parametrial tissue, consistent with Stage IB disease. She did not receive adjuvant chemotherapy or radiation and has remained disease-free to date. Because of the rarity of ASSO and lack of abundant case study reports, uniform clinical guidelines for treatment following surgical resection of a high-grade adenosarcoma remain unclear. However, the case study below may suggest that radical surgical debulking of this disease with negative margins in young patients with early-stage disease can be sufficient in treating high-grade ASSO, despite their typical aggressive nature.

Keywords: Adenosarcoma; Heterologous; Rhabdomyosarcomatous differentiation; Sarcomatous overgrowth; Surgical reduction; Uterus.

Fig. 1

A uniform fleshy and partially necrotic mass extending through the cervical canal and into the vagina measuring about 16 cm from the cervical os.

Fig. 2

T2 weighted image of the large ill-defined mass in situ as outlined by the yellow arrows, with the bladder displaced anteriorly noted by the red arrow. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)