Mullerian adenosarcoma of the cervix in a 10-year-old girl: case report and review of the literature.

UNLABELLED: Müllerian adenosarcoma is a rare neoplasm usually found in postmenopausal women. It usually presents as a polypoid mass within the endometrium. It is a biphasic tumor, composed of a benign epithelial component and a malignant stromal component. To date, this neoplasm has been reported in only 16 adolescent girls. We present a case of a 10-year-old girl who was diagnosed with müllerian adenosarcoma arising from the endocervix, the youngest female ever reported. CASE REPORT: A 10-year-old previously healthy girl presented to the Emergency Department at the Children's Hospital of Eastern Ontario with a painless mass protruding from her vagina. She had experienced mild vaginal bleeding for two weeks prior to her presentation. On physical examination, her vital signs were stable, and pubertal development was Tanner III breast and Tanner II pubic development. Rectoabdominal examination was negative. Two polypoid lesions were seen protruding past the hymenal ring and were removed in the emergency department. On gross examination, they were a dark tan color and had a fleshy appearance with a gelatinous consistency. They measured 5.5 x 1.5 x 1.0 cm and 3.5 x 1.5 x 1.5 cm. The final pathology revealed müllerian adenosarcoma, favoring an endocervical origin. Further investigations, including an abdominal/pelvic ultrasound and MRI and chest radiography, were negative. The patient subsequently underwent examination under anesthesia, vaginoscopy, hysteroscopy, polypectomy, and dilatation and curettage. The vagina appeared normal. At the level of the cervix, there were 3 polypoid gelatinous structures arising from the endocervix and extruding past the exocervix. They measured 0.8 x 0.5 x 0.2 cm up to 1.1 x 0.7 x 0.5 cm. The lesions were removed. Hysteroscopic inspection of the uterine cavity did not find any abnormalities. An endometrial curettage was performed. Pathology confirmed a diagnosis of müllerian adenosarcoma originating from the endocervix. Uterine curettings were negative for malignancy. After a thorough evaluation of the available literature, review with the Regional Tumor Board and extensive discussions with the family, a decision was made to perform a radical hysterectomy, bilateral salpingectomy, bilateral pelvic lymph node dissection, upper vaginectomy and preservation of ovaries. The procedure was uncomplicated. Clinically, there was no evidence of residual disease. The final pathology was negative for malignancy.
CONCLUSION: Müllerian adenosarcoma of the endocervix is a very rare pediatric tumor. Due to the rarity of this tumor in this age group, optimal therapy is uncertain. Most experts recommend hysterectomy. The review of literature reveals a high recurrence rate following conservative surgical management. Chemotherapy and radiation have not been used in the absence of extensive pelvic and/or residual disease. Poor prognostic factors include depth of invasion, sarcomatous overgrowth and high-grade malignant features in the stromal component. If recurrence occurs, it tends to be local and following prior conservative treatments such as cone biopsy or trachelectomy. Recurrences may occur late and thus long term follow-up of these patients is recommended.