J Pathol Transl Med.  2017 Jul;51(4):433-440. 10.4132/jptm.2017.06.11.

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

• Lee SJ
• Park JY

• KMID: 2392586
• DOI: http://doi.org/10.4132/jptm.2017.06.11

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.

Keyword

Uterus; Müllerian adenosarcoma; Adenomyosis

MeSH Terms

Adenomyosis*
Adenosarcoma*
Adult
Cervix Uteri
Endometriosis
Endometrium
Female
Humans
Leiomyoma
Ovary
Round Ligaments
Uterus*
Vagina

Figure

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  Fig. 1. Radiologic findings. (A) Pelvic ultrasonography, showing an enlarged uterus with a 7-cm solid and cystic mass. (B) T2-weighted magnetic resonance imaging, showing an enlarged uterus with a mass, measuring 71×59×72 mm, arising from the uterine fundus.

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  Fig. 2. Macroscopic findings of the hysterectomy specimen. (A) View showing an ill-defined ovoid tumor, 7.5 cm in diameter, together with hemorrhagic degeneration in the uterine fundus. (B) View showing that the cut surface of the lesion was tan-brown in color, multicystic, and solid.

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  Fig. 3. Histologic and immunohistochemical findings. (A, B) Microscopic examination showing a biphasic tumor composed of both dilated glandular elements and abundant, hypercellular stromal elements. (C) The tumor shows expansile growth within the myometrium, with extensive myometrial invasion and focal infiltrates into the subserosa with expansile margins. (D) Proliferation of hypercellular spindle cells growing in a fascicular pattern, around benign endometrial glands. (E) The stromal cells show mild and focal moderate cytological atypia with occasional mitotic figures (arrows). (F–J) Immunohistochemical analysis showing that the glandular and stromal cells in tumor tissue are positive for estrogen receptor (F) and focally positive for p53 (G); that the stromal cells are positive for CD10 (H) and smooth muscle actin (I); and that the Ki-67 proliferation index is higher in the stromal component than in the epithelium (J).

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