Uterine Adenosarcoma Originating in Adenomyosis: Report of an Extremely Rare Phenomenon and Review of Published Literature.

Müllerian adenosarcoma is an uncommon biphasic malignant tumor most often occurring in the uterine corpus and derived from native surface endometrium. We report a case of intramural uterine adenosarcoma arising in association with adenomyosis, in the absence of tumor involving the surface endometrium. This is an extremely rare phenomenon, with only 8 other published cases of uterine corpus adenosarcoma in the absence of surface endometrial involvement, 5 originating in adenomyosis and 3 in adenomyomas. We review these cases. The current FIGO staging system for uterine adenosarcoma assumes origin from the surface endometrium and does not address the rare occurrence of intramural tumors without a surface endometrial component. Such tumors are problematic to stage and could potentially be overtreated, particularly if there is deep myometrial involvement.

Post-radiation Mullerian adenosarcoma with sarcomatous overgrowth: rare presentation of an uncommon malignancy.

Mullerian adenosarcoma is an uncommon biphasic malignant uterine tumor. It is composed of benign epithelial and malignant stromal elements. We present a case of a 45-year-old woman who presented with post-menopausal bleeding for three months. She had a significant past medical history of pelvic irradiation for squamous carcinoma of cervix 20 years ago. Pathology revealed adenosarcoma with sarcomatous overgrowth. The patient had a recurrence of pure sarcoma three months later and unfortunately succumbed to her disease. The role of radiation in the pathogenesis of adenosarcoma has been uncommonly described compared to its well established role in the development of carcinosarcoma. Our case fulfils the criteria for a radiation induced sarcoma. We review the salient clinical and pathological features of this uncommon lesion highlighting the importance of sarcomatous overgrowth in these lesions and the possible role of radiation in the development of these tumors.

Diagnostic challenges: low-grade adenosarcoma on deep endometriosis.

BACKGROUND: Müllerian adenosarcoma is a rare malignancy. These tumors occur mainly in the uterus, but also in extrauterine locations, usually related to endometriosis. Because of their rarity, there is limited data on optimal management strategies. CASE PRESENTATION: We present a 44-year-old woman with a history of endometriosis who consults for chronic pelvic pain. In the imaging tests, a heterogeneous mass is observed that impresses endometriosis, encompassing the uterus and left appendage. Surgery is performed by finding an extrauterine adenosarcoma that affected the uterus, ovary and bladder wall. CONCLUSION: This is a rare case but should be considered in a patient with atypical clinical characteristics or preoperative pathology, so we show the diagnostic and therapeutic strategies carried out for the resolution of the case.

Ovarian adenosarcoma with extensive deciduoid morphology arising in endometriosis: a case report.

A case of müllerian adenosarcoma arising in ovarian endometriosis is reported in which the whole of the mesenchymal component exhibited striking deciduoid morphology, a phenomenon that has not been previously described. The patient was not taking hormonal preparations. We discuss the differential diagnosis and the possible pathogenesis of the deciduoid stromal alteration.

Extrauterine müllerian adenosarcoma associated with endometriosis and rectal villotubular adenoma: report of a case and review of the literature.

Extrauterine mullerian adenosarcoma is rare and is thought to arise from endometriotic deposits or pluripotent mesothelial and mesenchymal cells of the pelvic cavity. Concomitant colon-rectal neoplasm was reported but the relationship between extrauterine adenosarcoma and colon tumor was not studied. We describe an extrauterine adenosarcoma with a concomitant rectal tubulovillous adenoma. The patient had a long-term history of endometriosis and unopposed estrogen therapy. Immunohistochemical study was performed to investigate the origin and nature of the adenosarcoma. Immunostaining provided the evidence to distinguish between rectal tumor and adenosarcoma. Extrauterine adenosarcoma may arise from endometriosis, and hormone replacement therapy may have some role in the malignant transformation process. Adenosarcoma should be considered in the differential diagnosis of a new pelvic tumor in a patient with a history of endometriosis.

Immunohistochemical expression of CD10 antigen in uterine adenosarcoma.

Uterine adenosarcoma (UAS) is microscopically characterized by a biphasic growth pattern. By definition, the epithelial component is benign, whereas the stromal component typically has the appearance of a low-grade sarcoma, usually an endometrial stromal sarcoma. CD10 acts by reducing cellular response to peptide hormones and is currently regarded as a specific marker for endometrial stromal tumors. In this international multicenter study, we further explored CD10 immunoreactivity in 30 UASs. We encountered CD10 positivity of the sarcomatous component in 18/20 (90%) of UASs, in five of eight (63%) of UASs with sarcomatous overgrowth as well as in both cases of recurrent UAS. The epithelial component stained negative in all cases. These findings suggest that CD10 can be used to differentiate UAS from cellular leiomyoma, or in case endometrial stromal cells exhibit muscle differentiation. Furthermore, CD10 positivity in recurrent UAS might guide the pathologist toward an endometrial stromal origin.

A rare case of extrauterine adenosarcoma arising in endometriosis of the rectovaginal septum.

OBJECTIVE: To present a rare case of endometrial stromal sarcoma arising in endometriosis of the rectovaginal septum. DESIGN: Case report. SETTING: Academic tertiary referral center for endometriosis treatment. PATIENT(S): A 50-year-old woman with a history of endometriosis presented with catamenial rectal pain and deep dyspareunia. Imaging findings suggested new endometriotic lesions in the rectovaginal space. INTERVENTION(S): Total hysterectomy, salpingo-oophorectomy, and excision of the lesion in the rectovaginal septum were performed. Although extemporary pathology confirmed endometriosis, the final histologic diagnosis was extrauterine adenosarcoma in the rectovaginal septum. Two years later, recurrence of the malignancy occurred and was treated by resecting the new perirectal mass. Subsequent radiotherapy and chemotherapy were administered. MAIN OUTCOME MEASURE(S): Imaging findings at follow-up evaluation. RESULT(S): The patient was in good health for 2 years after the initial surgery, when she developed a new lesion at the site of the previous resection. The histologic appearance of the lesion was consistent with recurrence of the tumor. After postoperative therapy, the patient is now without evidence of disease. CONCLUSION(S): Malignant transformation of endometriosis should be considered in the differential diagnosis of a new pelvic lesion in a patient with a history of endometriosis.

Müllerian adenosarcoma of vagina arising in persistent endometriosis: report of a case and review of the literature.

BACKGROUND: Primary adenosarcoma arising in vaginal endometriosis poses a diagnostic challenge, especially in superficial vaginal biopsies. CASE: A 56-year-old woman, with a prior diagnosis of ovarian endometriosis, presented with a rapidly enlarging mass of the vaginal vault. Two prior biopsies were benign and showed endometriosis. The third vaginal biopsy revealed benign endometriotic glands cuffed by a cellular stroma with moderate cytologic atypia, a histological appearance diagnostic of Müllerian adenosarcoma. A 16-cm vaginal mass that had infiltrated the pelvic structures was resected. CONCLUSIONS: Close clinical follow-up of extrauterine endometriosis and clinical-pathologic correlation is necessary. Histological features such as cellular stromal cuffing around benign endometriotic glands are critical in arriving at a timely diagnosis of adenosarcoma in patients with persistent extrauterine endometriosis, even in superficial vaginal biopsies.

Extragenital mullerian adenosarcoma with sarcomatous overgrowth arising in an endometriotic cyst in the pouch of Douglas.

Mullerian adenosarcoma is a neoplasm composed of benign mullerian epithelium and a sarcomatous stroma. This tumor classically occurs in the endometrium of postmenopausal women and less frequently in the extrauterine genital tract. Rare cases of extragenital adenosarcoma have been reported. We present the case of a 23-year-old female who presented with an extragenital adenosarcoma arising in an endometriotic cyst in the pouch of Douglas. The patient was treated with local excision, chemotherapy and radiotherapy. At 2 years follow-up she was disease-free. The literature on extragenital adenosarcoma is reviewed. These tumors are clinically more aggressive than their uterine counterparts. Surgical excision is the initial treatment modality for these patients. Little information is available regarding the efficacy of adjuvant chemotherapy or radiotherapy. This is an area that requires further study.