CNS metastasis secondary to malignant-mixed Müllerian tumor: case report and review of therapeutics

Abstract

This paper reviews CNS involvement secondary to malignant-mixed Müllerian tumor or uterine carcinosarcoma, a rare aggressive biphasic Müllerian tumor. We report a cerebellar metastasis with epithelial and mesenchymal components, demonstrating heterologous rhabdomyogenic and chondroblastic differentiation. The patient had undergone total abdominal hysterectomy and bilateral salpingo-oophorectomy for palliation of symptomatic chemotherapy-resistant node-positive disease. CNS involvement is rare, and prognostically poor, and suggestively poorer in predominantly sarcomatous metastases. Multimodal therapy is indicated; in solitary metastases, surgical resection or stereotactic radiosurgery is included, followed by whole brain radiotherapy. In unresectable brain metastases, stereotactic radiosurgery and whole brain radiotherapy warrant consideration in up to 2-3 metastases. In multiple metastases, palliative steroid therapy or cranial irradiation may be considered. Combination or platinum-based chemotherapy (i.e., ifosfamide-paclitaxel or carboplatin-paclitaxel) is indicated in all stages, with a role in both disease cure and control-directed management. Targeted therapeutics have thus far not demonstrated significant clinical efficacy.

Brain Metastasis in a Young Patient with Uterine Carcinosarcoma

“…Similar to other uterine and gynecologic malignancies, brain metastases have rarely been described [5]. To our knowledge, eight other cases of MMMT with central nervous system (CNS) involvement have been reported [3]. Compared to those, our patient was the youngest to develop brain metastasis from MMMT at age 41.…”

“…The median interval from presentation to CNS involvement and median survival, thereafter, have been reported to be 4.5 months and 3 months, respectively. Sarcomatous predomination has been linked to shorter survival [3]. Our patient was 41-year-old at diagnosis, brain metastasis occurred seven months after presentation, and she expired in the following month.…”

“…MMMT has both epithelial and mesenchymal elements and was traditionally categorized as a sarcoma. However, a growing body of evidence suggested that the sarcomatous component is, in fact, derived from the epithelium, prompting reclassification as dedifferentiated endometrial carcinoma [3]. While the epithelial component of this metaplastic tumor has been most frequently identified at the metastatic foci, most cases of CNS involvement showed a predominant sarcomatous differentiation [2-3].…”

“…This metaplastic tumor is known to metastasize extensively, however central nervous system involvement is an unusual occurrence. Management options for brain metastasis in UCS can be surgical resection, stereotactic radiosurgery (SRS) and whole brain radiation therapy (WBRT) with corticosteroid use depending on the number and site of lesions [3].…”

Uterine Carcinosarcoma: A Case Report and Literature Review

“…The local recurrence may be treated with radiotherapy or systemic chemotherapy [8]. Single-metastatic brain metastasis is managed with surgical removal of the tumor or stereotactic radiosurgery, after which adjuvant whole-brain radiotherapy is performed [29]. Palliative treatment with steroids and whole-brain radiation are considered for cases with multiple brain metastases [29].…”