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A Mullerian adenosarcoma in which the sarcomatous element showed ovarian sex-cord like differentiation, occurred as a polypoid growth in the uterine cervix of a 53-year-old woman. The tumor was composed of an admixture of benign neoplastic glands and a sarcomatous stroma, the latter containing in addition to endometrial stromal sarcoma, nests of lipid-rich cells resembling ovarian sex-cord elements. This is the first report of such differentiation in Mullerian adenosarcoma. Origin from a focus of cervical endometriosis is postulated.

We present a tumor of the uterine cervix that was composed of histologically benign glandular epithelium and a malignant stromal component, justifying the term mullerian adenosarcoma. Ultrastructurally the stromal cells resemble the endometrial stromal cells, the tumor cells of an endolymphatic stromal myosis of the uterine cervix, and the stromal cells of a stromomyoma of the uterus. In addition there is evidence strongly suggesting a differentiation toward smooth muscle. The patient exhibited no evidence of disease one year after surgery.

The author reports two cases of mullerian adenosarcoma (MAS) in the endometrium in patients aged 60 and 73 years. Both were treated by total abdominal hysterectomy and bilateral salpingo-oophorectomy. The 60-year-old patient is alive without signs of recurrence or metastases 3 years after the operation. The other patient, who received postoperative vaginal radium therapy, died of disseminated abdominal metastases 7 months after the operation. Only two cases of MAS leading to death within less than 1 year have been reported so far.

Mullerian adenosarcoma of the cervix with heterologous elements is an extremely rare tumor first described by Roth and colleagues (L. M. Roth, G. L. Pride, and H. M. Sharma, Cancer 37, 1725–1736) in 1976. Since that time there have been only three subsequent reports of these cervical Mullerian adenosarcomas, which seem to occur most often in the postmenarchal age group. Due to the paucity of cases and the unknown biological potential of the tumor, therapy has ranged from simple excision to radical pelvic surgery and vaginectomy combined with both radiotherapy and chemotherapy. We report another case of Mullerian adenosarcoma of the cervix occurring in a teenage woman and make recommendations about diagnostic and therapeutic measures available to the physician.

The clinicopathologic features of five cases of mullerian adenosarcoma of the uterus are presented and are compared with those of previously reported cases. Four tumors were localized in the endometrium; in one case, a cervical or endometrial origin was not clear. The ages of the patients ranged from 53 to 79 years. Two died without clinical evidence of recurrence 19 and 18 years after hysterectomy. One was alive and well 10 years postoperatively and one was lost to follow-up 6 months after therapy. In one case, a first abdominal recurrence was resected 5 years and 9 months after hysterectomy; the patient died of a second abdominal recurrence 18 months later. The tumors were bulky polypoid masses producing metrorrhagia. Histologically, a sarcomatous component was intimately associated with benign epithelium of various mullerian types. The neoplasm was typically organized in papillary fronds and periglandular sarcomatous cuffs. Cystic glands were also a constant feature. Mitoses were generally fewer than 10 per 10 high-power fields, except in one case where they were 44 in a more malignant-appearing area. Adenosarcoma must be differentiated from other mullerian tumors, mainly carcinosarcoma whose prognosis is much less favorable.

Background Uterine adenosarcomas are rare malignant gynaecological tumours. Due to its submucous localization, they can be easily confound with benign tumours like endometrial polyps or submucous myomas. However, the treatment of uterine adenosarcomas requires an oncologic surgical approach. Case presentation In the following case report, we present the minimally invasive treatment of a uterine adenosarcoma by hysteroscopy and laparoscopy in a 37-year-old patient and discuss the special role of hysteroscopy in such cases. Conclusions In case of unknown or suspect intrauterine tumours, a diagnostic and operative hysteroscopy with biopsy could be realized prior to laparoscopic hysterectomy especially when the use of a laparoscopic electric morcellation is planned. Thus, a correct oncologic approach can be guaranteed if an adenosarcoma is diagnosed. Trial registration ISRCTN