Case ReportMullerian Adenosarcoma of the Cervix in a 10-Year-Old Girl: Case Report and Review of the Literature
Introduction
Müllerian adenosarcoma is a rare neoplasm usually found in postmenopausal women and represents less than 8% of uterine sarcoma.1, 2 It usually presents as a polypoid mass within the endometrium.1 This neoplasm has also been found in the myometrium, endocervix, ovary, round ligament, and pelvic wall.1 It is a biphasic tumor, composed of a benign epithelial component and a malignant stromal component.1, 2, 3 To date, müllerian adenosarcoma has been reported in only 16 adolescent girls.1, 3 We present the youngest case ever reported, a 10-year-old female diagnosed with müllerian adenosarcoma arising from the endocervix.
Section snippets
Case Report
A 10-year-old, previously healthy girl, presented to the Emergency Department at the Children's Hospital of Eastern Ontario (CHEO) 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
Discussion
Müllerian adenosarcoma of the endocervix is a very rare pediatric tumor. It is a biphasic tumor, composed of a benign epithelial component and a malignant stromal component.1, 2, 3 The stromal component may include only elements indigenous to uterus (homologous) or show differentiation toward elements not normally found in the uterus (heterologous).3 Adenofibroma (where both epithelial and stromal components are benign) as well as malignant mixed müllerian tumor and carcinosarcoma (where both
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
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Management and survival of patients with Mullerian adenosarcoma of the cervix without sarcomatous overgrowth desiring fertility preservation, a case report and review of the literature
2020, Gynecologic Oncology ReportsCitation Excerpt :There is limited information regarding primary cervical Mullerian adenosarcomas. Prognosis has historically been favorable, with the exception of deeply invasive tumors or those with high-grade sarcomatous overgrowth (Fleming et al., 2009). Twenty-nine cases of women with low grade disease have been collected for review.
Clinical management of uterine cervical mullerian adenosarcoma: A clinicopathological study of six cases and review of the literature
2018, Taiwanese Journal of Obstetrics and GynecologyUterine Adenosarcoma with Sarcomatous Overgrowth: A Case Report of Aggressive Disease in a 16-Year-Old Girl and a Literature Review
2018, Journal of Pediatric and Adolescent GynecologyManagement of Low-Grade Cervical Müllerian Adenosarcoma in a 14-Year-Old Girl
2017, Journal of Pediatric and Adolescent GynecologyCitation Excerpt :In previously reported cases of Müllerian adenosarcoma, management recommendations often included total hysterectomy, with or without chemotherapy and radiation.4–6 However, for patients with low-grade lesions who desire to preserve fertility, local excision might be a reasonable option in the setting of close follow-up, because most reported recurrences are local.3,4,6,7 The absence of sarcomatous overgrowth and heterologous elements noted in this case confers a favorable prognosis; although the propensity for these lesions to locally recur underscores the importance of long-term close monitoring.
Müllerian adenosarcoma of the uterine cervix with sarcomatous overgrowth: A case report of aggressive disease in a young patient
2016, International Journal of Surgery Case ReportsUse of hysteroscope for vaginoscopy or hysteroscopy in adolescents for the diagnosis and therapeutic management of gynecologic disorders: A systematic review
2015, Journal of Pediatric and Adolescent GynecologyCitation Excerpt :The age of the participants was between 10 and 18 years old, which is consistent with the World Health Organization's definition of adolescence.23 Different symptoms were identified in these patients, including dysmenorrhea,4,5,9,10,12,15,16,18,22 abnormal vaginal bleeding,8,9,14,19 irregular heavy vaginal bleeding,17 pelvic/abdominal pain,11,13 protruding vaginal masses,19,21 and agonizing pain and swelling along with difficulty in passing urine and stool.9 The majority of the patients was known to have poor responses or lack of benefit after medical management and required further management, such as vaginoscopic or hysteroscopic procedures.