A Rare Case of Intramural Müllerian Adenosarcoma Arising from Adenomyosis of the Uterus

Abstract

Müllerian adenosarcomas usually arise as polypoid masses in the endometrium of post-menopausal women. Occasionally, these tumors arise in the cervix, vagina, broad and round ligaments, ovaries and rarely in extragenital sites; these cases are generally associated with endometriosis. We experienced a rare case of extraendometrial, intramural adenosarcoma arising in a patient with adenomyosis. A 40-year-old woman presented with sudden-onset suprapubic pain. The imaging findings suggested leiomyoma with cystic degeneration in the uterine fundus. An ill-defined ovoid tumor with hemorrhagic degeneration, measuring 7.5 cm in diameter, was detected. The microscopic findings showed glandular cells without atypia and a sarcomatous component with pleomorphism and high mitotic rates. There was no evidence of endometrial origin. To recognize that adenosarcoma can, although rarely, arise from adenomyosis is important to avoid overstaging and inappropriate treatment.

Adenomyosis as a Risk Factor for Myometrial or Endometrial Neoplasms—Review

“…There are also several case reports on sarcomas arising in adenomyosis foci described in the literature [ 45 , 46 , 47 ]. Malignant transformation in adenomyosis other than endometroid endometrial cancer has been documented [ 48 ].…”

New Aspects of Sarcomas of Uterine Corpus—A Brief Narrative Review

“…It is stated that approximately 1–10% of adenosarcomas are thought to arise from the malignant transformation of endometriosis [ 74 ]. Cases of adenosarcomas arising due to endometriosis have been reported in medical literature—from adenomyosis of the uterus, bladder adenosarcoma arising from endometriosis, and adenosarcoma arising in an abdominal scar in a patient with a history of endometriosis [ 78 , 79 , 80 ]. Unlike uterine carcinosarcoma, UAS is a mesenchymal neoplasms where clonal genetic alterations are found in the sarcomatous but not in the epithelial components.…”

“…The most significant prognostic factors are: age, sarcomatous overgrowth, myometrial invasion, lymphovascular invasion, and lymph node involvement. Tumor necrosis and cellular atypia (asterisk in the table) could not be independent risk factors, as they are associated with sarcomatous overgrowth, high mitotic rate, myometrial invasion or necrosis [ 74 , 77 , 78 , 79 , 80 , 81 , 82 , 83 , 84 ]. Resection status and rhabdomyosarcoma components are possible prognostic factors [ 74 , 77 , 78 , 79 , 80 , 81 , 82 , 83 , 84 ].…”

Case Report: Uterine Adenosarcoma With Sarcomatous Overgrowth and Malignant Heterologous Elements

“…The morphological characteristics of these neoplasms are distinguished from other biphasic tumors (epithelial and mesenchymal), both benign and malignant. UA are generally neoplasms of low malignant potential, except when accompanied by sarcomatous overgrowth and myometrial invasion (1,4); these tumors can recur locally, and rarely metastasize (6). About 15% of UA can have heterologous elements, such as skeletal muscle, cartilage, fat, and other components.…”