Mullerian adenosarcoma with sarcomatous overgrowth in a premenopausal patient / Philippine Journal of Obstetrics and Gynecology

@#We report a case of a 33-years old, nulligravid, diagnosed with mullerian adenosarcoma with sarcomatous overgrowth (MASO), who presented with vaginal bleeding and recurrent endometrial polyp. MASO is a rare type of uterine sarcomas, it is a variant of adenosarcomas with poor prognosis. The index patient underwent primary surgical management with lymphadenectomy with a final stage of IC. Histologic diagnosis was Mulllerian adenosarcoma with sarcomatous overgrowth. A panel of immunostaining for estrogen receptors, progesterone receptors and CD 10 showed diffused positivity for the hormones with loss of CD 10 which is consistent with MASO. The rarity of MASO has a distinctive histologic features which merits meticulous sectioning as the clinical course and management vary. It has a poor prognosis due to its short and fast course of the disease.

High grade adenosarcoma of endocervix and endometrial cavity with a heterologous component of rhabdomyosarcoma. Case report and review of the literature / Adenosarcoma de alto grado de endocérvix y cavidad endometrial con componente heterólogo de rabdomiosarcoma. reporte de caso y revisión de la literatura

RESUMEN Objetivo: presentar el caso de una paciente con diagnóstico de adenosarcoma de alto grado de endocérvix y de cavidad endometrial, con componente heterólogo, y hacer una revisión de la literatura con especial atención al diagnóstico y manejo terapéutico de esa patología. Materiales y métodos: Se presenta el caso de una paciente de 31 años, que consulta al Hospital Universitario Virgen Macarena de Sevilla, institución regional de tercer nivel, por presentar san grado genital originado por una masa polipoidea endocervical que se llevó a biopsia con resultado de un leiomiosarcoma poco diferenciado de alto grado de endocérvix. Posteriormente fue llevada a histerectomía total abdominal. En el estudio de la pieza quirúrgica el resultado fue: adenosarcoma de endocérvix y cavidad endometrial, con componente heterólogo de rabdomiosarcoma. Con los términos: "adenosarcoma", "endocervical", "cérvix", "uterus", "heterologous", en las bases de datos Medline vía PubMed se realizó una búsqueda de artículos de revisión bibliográfica, reportes y series de casos clínicos que describían aspectos del adenosarcoma cervicouterino y del componente heterólogo de rabdomiosarcoma, en inglés y español, publicados desde 1974. Resultados: se hallaron seis artículos correspondientes a revisiones de la literatura, reportes o series de casos, donde se describen los aspectos más importantes referentes al diagnóstico y tratamiento de esta patología. Conclusiones: esta patología se caracteriza, en ocasiones, por un crecimiento rápido y agresivo, donde es importante el diagnóstico precoz y el tratamiento óptimo, basado en una combinación de cirugía, radioterapia y quimioterapia, aunque dada su baja prevalencia se necesitan aún más estudios para poder confirmar estos datos.

ABSTRACT Objective: To present the case of a patient diagnosed with high grade adenosarcoma of the endocervix and the endometrial cavity, with a heterologous component, and to conduct a review of the literature focusing on the diagnosis and therapeutic management of this disease condition. Materials and methods: We present the case of a 31-year-old female patient who came to Virgen Macarena University Hospital of Seville, a Level III regional institution, complaining of genital bleeding arising from an endocervical polypoid mass. The biopsy of the mass revealed a high grade, poorly differentiated leiomyosarcoma of the endocervix. The patient was taken later to total abdominal hysterectomy. The study of the surgical specimen provided the following result: adenosarcoma of the endocervix and endometrial cavity with a heterologous rhabdomyosarcoma component. A search was conducted in the Medline database via Pubmed using the terms "adenosarcoma," "endocervical," "cervix," "uterus," "heterologous." The search included literature review articles, case reports and clinical case series describing aspects of cervical adenosarcoma and the heterologous rhabdomyosarcoma component, published in English and Spanish since 1974. Results: Six articles corresponding to literature reviews, case reports or case series in which the most relevant aspects of the diagnosis and treatment of this disease condition are described were retrieved. Conclusions: This condition is characterized, on occasions, by rapid and aggressive growth, hence the importance of early diagnosis and optimal treatment based on a combination of surgery, radiation therapy and chemotherapy. However, due to its low prevalence, further studies are needed in order to confirm these data.

Imaging description of extragenital müllerian adenosarcoma: A case report / Descrição da imagem do adenosarcoma mülleriano extragenital: relato de caso

Abstract Müllerian adenosarcoma is a very rare gynecological disease, comprising 5% of uterine sarcomas. Extragenital localizations are even rarer.We report a very interesting case of a 27-year-old woman complaining of pelvic pain, with a subsequent diagnosis of extragenital Müllerian adenosarcoma. This is the first case reported in the literature with a complete and wide imaging description. Even if rare, Müllerian adenosarcoma should be hypothesized in case of young female patients presenting with suspicious pelvic mass.

Resumo O adenosarcoma Mülleriano é uma doença ginecológica muito rara, compreendendo 5% dos sarcomas uterinos. Localizações extragenitais são ainda mais raras. Relatamos um caso muito interessante de uma mulher de 27 anos queixando-se de dor pélvica com diagnóstico subsequente de adenosarcoma Mülleriano extragenital. Este é o primeiro caso relatado na literatura com uma descrição completa e ampla de imagem. Mesmo que raro, o adenosarcoma Mülleriano deve ser hipotetizado no caso de pacientes jovens do sexo feminino com massa pélvica suspeita.

Current Status of Magnetic Resonance Imaging in Patients with Malignant Uterine Neoplasms: A Review

In this study, we summarize the clinical role of magnetic resonance imaging (MRI) in the diagnosis of patients with malignant uterine neoplasms, including leiomyosarcoma, endometrial stromal sarcoma, adenosarcoma, uterine carcinosarcoma, and endometrial cancer, with emphasis on the challenges and disadvantages. MRI plays an essential role in patients with uterine malignancy, for the purpose of tumor detection, primary staging, and treatment planning. MRI has advanced in scope beyond the visualization of the many aspects of anatomical structures, including diffusion-weighted imaging, dynamic contrast enhancement-MRI, and magnetic resonance spectroscopy. Emerging technologies coupled with the use of artificial intelligence in MRI are expected to lead to progressive improvement in case management of malignant uterine neoplasms.

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

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.

Safety of ovarian preservation in premenopausal women with stage I uterine sarcoma / 부인종양

OBJECTIVE: To evaluate the oncologic safety of ovarian preservation (OP) in premenopausal women diagnosed with the International Federation of Gynecology and Obstetrics (FIGO) stage I uterine sarcoma. METHODS: The National Cancer Institute's Surveillance, Epidemiology, and End Results database was accessed and a cohort of women aged ≤50 diagnosed between 1988–2013 with a sarcoma limited to the uterus was drawn. Based on site-specific surgery codes, women who underwent hysterectomy with or without oophorectomy and did not receive radiation therapy were selected for further analysis. Overall (OS) and cancer-specific (CSS) survival were determined following generation of Kaplan-Meier curves; comparisons were made with the log-rank test. A Cox-proportional hazard model was constructed to control for possible confounders. RESULTS: A total of 1,482 women were included in the analysis; 800 (54.0%) were diagnosed with leiomyosarcoma (LMS), 520 (35.1%) with low-grade endometrial stromal sarcoma (LG-ESS), and 162 (10.9%) with adenosarcoma (AS). The OP group included 418 women (28.2%). Differences in the rate of OP were noted based on histology (p=0.014), year of diagnosis (p=0.001), patient age (p<0.001) and race (p=0.012). There was no difference in OS (p=0.220) or CSS (p=0.210) between women who had OP and those who did not. Multivariate analysis confirmed that OP was not associated with a worse mortality. CONCLUSION: In this population-based cohort of women with sarcoma limited to the uterus, OP was not associated with worse oncologic outcomes. OP could be considered for women with LMS, sparing them from the morbidity associated with iatrogenic menopause. No conclusions could be made for those with LG-ESS or AS.

A Case of Solitary Brain Metastasis from Uterine Mullerian Adenosarcoma with Sarcomatous Overgrowth

Uterine adenosarcoma (AS) are rare tumors and have more favorable outcomes than the aggressive uterine carcinosarcomas. Uterine adenosarcoma with sarcomatous overgrowth (ASSO) is a variant form of AS and exhibits aggressive growth of tumor and the prognosis is relatively poor compared with typical AS. Usually patterns of metastasis have been known to behave like endometrial carcinoma and spread through the lymphatics. Brain metastasis from uterine AS is extremely rare. Herein, we report a case of successfully surgically removed solitary brain metastasis without any extracranial recurrence from uterine ASSO after 4 years of primary treatment.

MR Imaging Findings of Tamoxifen-associated Uterine Adenosarcoma: Report of Two Cases

Adenosarcoma of the uterus is a rare biphasic tumor containing benign glandular epithelial and malignant mesenchymal components. The tumor has been reported to be associated with antiestrogen therapy, particularly tamoxifen, but there have been a few case reports with MRI. We present two cases of MRI findings of uterine adenosarcoma after antiestrogen therapy, tamoxifen and toremifene in breast cancer patients. The tumor presents as a large polypoid mass occupying the endometrial cavity, and may protrude into the vagina. On MRI, the tumor typically shows solid components with scattered small cysts and heterogeneous enhancement. These findings are not significantly different from conventional adenosarcoma.

Mullerian Adenosarcoma Arising From Rectal Endometriosis

A Mullerian adenosarcoma is an extremely rare tumor characterized by a stromal component of usually low-grade malignancy and by a benign glandular epithelial component. A Mullerian adenosarcoma occurs mainly in the uterus, but also in extrauterine locations. Extrauterine Mullerian adenosarcomas are thought to arise from endometriotic deposits. A 36-year-old female presented to Daegu Catholic University Medical Center with a symptom of loose stool for several months. The imaging studies revealed a rectal mass, so she underwent a laparoscopic low anterior resection. Although extemporary pathology revealed an inflammatory myofibroblastic tumor, the final histologic diagnosis was a Mullerian adenosarcoma arising from rectal endometriosis. To our knowledge, except a concomitant rectal villotubular adenoma, cases of Mullerian adenosarcomas arising the rectal wall are rare. An adenosarcoma arising from endometriosis should be considered in the differential diagnosis of a pelvic mass, even one appearing in rectal wall, because ectopic endometrial tissue exists everywhere.

Recurrent Müllerian adenofibroma of uterus: a clinicopathologic study of 7 cases / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features and differential diagnosis of recurrent Müllerian adenofibroma (MAF) of the uterus.</p><p><b>METHODS</b>Clinicopathologic information of 7 cases of recurrent MAF of uterus was retrieved from January 1992 to April 2006 and compared with 12 cases of MAF without recurrence and 14 cases of low-grade Müllerian adenosarcoma (MAS). EnVision immunohistochemistry of estrogen receptor (ER), progesterone receptor (PR), smooth muscle actin (SMA), CD10, Ki-67 and p53 were performed in all cases.</p><p><b>RESULTS</b>All cases of recurrent MAF of the uterus were polypoid, lobulated, and broad based mass arising from the corpus or cervix. Microscopically, the tumor consisted of benign epithelial and mesenchymal components with low mitotic activity ( ≤ 1/10 HPF). The clinical and pathologic features of 3 recurrent tumors were similar to their primary tumors, while 4 cases of recurrent tumor presented with focally higher cellularity and mitotic activity, meeting the diagnostic criteria of adenosarcoma. The stromal expression patterns of ER, PR, SMA and p53 in recurrent MAF were similar to those of clinically benign MAF and low-grade MAS. Negative or focally positive stromal cell expression of CD10 was seen infrequently in recurrent MAF (1/7) and clinically benign MAF (1/12). In contrast, a moderate to strong CD10 staining was frequently seen in MAS (9/14, P < 0.05). The difference of Ki-67-labeling index between MAF and MAS did not reach a statistical significance (P > 0.05). Ki-67-labeling index increased in areas of periglandular stromal cuffing as compared with interglandular areas in all MAS cases, but it was not observed in either recurrent MAF or clinically benign MAF cases.</p><p><b>CONCLUSIONS</b>Recurrent MAF may be associated with aggressive behavior. It is difficult to distinguish MAF from low-grade MAS. CD10 and Ki-67 staining pattern in stromal cells may be helpful for the differential diagnosis.</p>

Synchronous occurrence of primary malignant mixed mullerian tumor in ovary and uterus

Malignant mixed mullerian tumor (MMMT) arising from female internal genitalia is rare with the uterine corpus being the most prevalently affected site. It is even more rare when it occurs on both uterus and ovary at the same time. We describe a case of synchronized occurrence of MMMT on ovary and uterine adenosarcoma with review of literature.

Clinicopathological spectrum of 19 adenosarcomas of female genital tract, including uncommon clinical associations and immunohistochemical profile, reviewed at a single institution.

Background: Adenosarcomas of the female genital tract have been rarely documented as case series from our continent. Materials and Methods: Over a seven-year period, 19 adenosarcomas were critically reviewed. Results: Nineteen tumors occurred in the age range of 21-65 years (mean: 43), in the endometrium (8), endometrium and cervix (4), cervix (4), and ovary (3). Four cases displayed coexisting leiomyomas; two, adenomyosis; two on background endometriosis; and one in post-treated cervix carcinoma. Histopathologically, the tumors were low grade (10; 52.6%) and high grade (9; 47.3%), the latter with sarcomatous overgrowth (SO) (7/9 cases). Dedifferentiation (8, 42.1%) and conspicuous decidualization (2) were noted. Immunohistochemically, the tumors focally expressed CD10 (4/6), smooth muscle actin (SMA) (3/8), desmin (8/11); diffuse vimentin (7/7), and estrogen receptor/progesterone receptor (ER/PR) (2/4). Ki-67 (6 cases) varied 5-20%. Seventeen patients underwent surgery and four received adjuvant treatment (3/4 high-grade tumors). Five tumors recurred (4 high-grade tumors with SO) and one metastasized. Among 11 patients, five were alive with disease (AWD) (mean: 29.4 months) and six, free of disease (FOD) (mean: 15 months), the latter mostly with low-grade type tumors (83.3% cases). Conclusions: Diverse clinicopathological spectrum was noted within adenosarcomas. Low-grade tumors were less aggressive than high-grade ones, with SO. Immunohistochemically, lower CD10 and ER/PR positivity was noted in high-grade tumors. Surgery formed the mainstay of treatment. Adjuvant treatment was offered in high-grade subtypes, including in tumors with SO.

Novos conceitos e revisão atualizada sobre sarcomas uterinos / New concepts and update review of uterine sarcomas

Realizou-se uma revisão sistemática das publicações dos últimos dez anos sobre os sarcomas uterinos. Este artigo traz novos conceitos, como a mudança do carcinossarcoma para carcinoma metaplásico. Evidencia-se a necessidade de individualizar o tratamento dos sarcomas, pois existem muitas diferenças de comportamento clínico, padrão metastático e prognóstico entre os diferentes tipos histológicos. Também foram enfocadas as controvérsias existentes quanto a extensão cirúrgica, a necessidade de ooforectomia e de esvaziamento ganglionar e o papel da adjuvância

It was performed a systematic review of publications for the past 10 years about uterine sarcomas. This article introduces new concepts, such as the change of carcinosarcoma to metaplastic carcinoma. This study highlights the need to individualize the treatment of uterine sarcomas, as there are many differences in clinical behavior, prognosis and metastatic pattern between the different histological types. Controversies have also been focused on the surgical extension, the need for lymph node dissection and oophorectomy and the role of adjuvant therapy

Tumores mesenquimáticos del endometrio / Endometrial mesenchymal tumors. Study of 4 cases

Los tumores mesenquimáticos de la mucosa uterina son un grupo raro, heterogéneo y generalmente agresivo de neoplasias que conduce frecuentemente a una diseminación y muerte temprana. Constituyen menos del 3% de todos los tumores malignos del Tracto Genital Femenino y el 2-7% de las neoplasias malignas uterinas. Presentamos 4 casos de tumores mesenquimáticos diagnosticados en un período de 5 años en el Servicio de Anatomía Patológica del Hospital General de Agudos Carlos G. Durand, analizando su incidencia y haciendo una breve revisión de las características histopatológicas de los mismos

Mesenchymal tumors of the uterine lining are a rare and heterogeneous group of neoplasms, with an agressive behavior leading to an early dissemination and death. They represent less than 3% of all malignant tumors of the Female Genital Tract and 2-7% of uterine malignancies. We report 4 mesenchymal tumors diagnosed throughout a 5 years period at the Department of Pathology Hospital General Carlos G. Durand, analyzing their incidence and making a review of the histopathologic features

Mullerian adenosarcoma (heterologous) of the cervix with sarcomatous overgrowth: a case report with review of literature / 부인종양

Mullerian adenosarcoma is a rare biphasic malignant neoplasm of the cervix characterized by an admixture of benign epithelial elements and a malignant sarcomatous stromal component, which may be either homologous or heterologous. An aggressive variant of adenosarcoma, mullerian adenosarcoma with sarcomatous overgrowth (MASO) is extremely rare, with only two such cases being reported in the English literature to date. In this report we present a case of MASO of uterine cervix with heterologous elements in a 15-year-old unmarried girl presenting with foul smelling menstrual bleeding and passage of fleshy masses. Because MASO with heterologous elements seems to appear at the earliest stages of reproductive lifespan in women, and have an uncertain malignant potential, gynecologists and pathologists should be aware and think about the possibility of this tumor.

Endometrial mullerian adenosarcoma after toremifene treatment in breast cancer patients: a case report / 부인종양

Toremifene is an anti-estrogen which has been shown to be effective in the treatment of breast cancer, and is thought to be a less uterotrophic agent than tamoxifen. The risk assessment concerning endometrial cancer has been inconclusive because of its rare use up to the mid-1990s. We report a case of an adenosarcoma, which is a very rare type of uterine malignancy, after toremifene treatment for 5 years in a breast cancer patient. After 1 year of toremifene use, the patient had a benign Mullerian adenofibroma. After an additional 4 years of toremifene treatment, the endometrial polypoid lesion was transformed into a Mullerian adenosarcoma. Although toremifene is a promising anti-estrogenic agent in the treatment of breast cancer patients, clinicians should not neglect the possibility of a uterine malignancy.

Mullerian adenosarcoma of the uterus: A clinicopathologic analysis of 9 cases / 中华肿瘤杂志

<p><b>OBJECTIVE</b>To investigate the clinicopathologic features, diagnosis, treatment and prognosis of uterine mullerian adenosarcoma.</p><p><b>METHODS</b>The clinicopathological data of 9 cases of uterine mullerian adenosarcoma in PUMC hospital from January 2003 to February 2009 were retrospectively analyzed.</p><p><b>RESULTS</b>There were 6 uterine endometrial adenosarcomas and 3 cervical adenosarcomas. The main clinical manifestations were abnormal vaginal bleeding and pelvic pain. Physical examination showed cervical/vaginal mass, enlarged uterus or pelvic mass. The adenosarcoma was characterized by benign or atypical-appearing neoplastic glands within a sarcomatous stroma. This stroma could appear as periglandular cuffs or intraglandular polypoid projections of increased cellular structure. The primary diagnostic rate was 66.7% and the most common clinical stage was stage I (7/9). All patients received surgical treatment and seven had postoperative chemotherapy, radiotherapy or hormone therapy. Conservation of unilateral ovary or bilateral ovaries was performed in 5 cases. Three patients underwent local excision, which resulted in the preservation of reproductive function. During the follow-up, 2 cases of uterine endometrial adenosarcoma recurred. One patient of clinical stage III containing sarcomatous overgrowth died from recurrence 13 months after surgery. The other one recurred 2 years after local excision of the tumor in the uterine cavity and she remained healthy since hysterectomy.</p><p><b>CONCLUSION</b>Uterine mullerian adenosarcoma is a rare tumor without specific clinical symptoms and signs. The diagnosis depends on pathomorphologic examination. The tumors show low malignant potential and the vast majority are at early stage. Surgical excision is the main treatment strategy with a good prognosis in the early stage disease with complete removal of tumors. The prognosis is poor in advanced adenosarcoma with sarcomatous overgrowth. Due to the relatively high rate of recurrence, long-term follow-up is recommended.</p>

Adenosarcoma de ovario asociado con elevación del antígeno sérico Ca-125: informe de caso y revisión de literatura / Ovarian adenosarcoma with elevated CA125 antigen: case report and literature review

BACKGROUND: Adenosarcomas are rare tumors usually derived from the endometrium. About 50 cases of adenosarcomas of the ovary have been reported. The relationship between adenosarcoma and CA125 has not been described. The authors present a case of adenosarcoma with elevated CA125 because of the unusual presentation of this pathology and also because elevation of the CA125 antigen has not been reported in the literature. CLINICAL CASE: A 42-year-old woman presented for consultation for incidental right ovarian tumor and CA125 of 1100 U/mL. Histology revealed a homologous Müllerian adenosarcoma of the right ovary with sarcomatous overgrowth. CA125 decreased to 16 U/mL after surgery. Sixteen months post-surgery, the patient is disease free and with normal CA125. DISCUSSION: Ovarian adenosarcomas are more aggressive than adenosarcomas of the uterus. Because of the embryological origin, ovarian adenosarcomas are able to produce CA125 antigen, especially in the presence of sarcomatous overgrowth. With these facts, CA125 antigen may be useful as a prognostic factor because it may represent an indirect marker of sarcomatous overgrowth. CONCLUSIONS: CA125 may be useful for follow-up of ovarian adenosarcomas. Elevated CA125 antigen in adenosarcomas of the ovary may be indicative of sarcomatous overgrowth and poor prognosis.

MR Findings of Extrauterine Mullerian Adenosarcoma Associated with Deep Pelvic Endometriosis

Extrauterine mullerian adenosarcoma is a very rare tumor and it is characterized by a benign glandular component and a low-grade sarcomatous stromal component. These tumors have been reported to arise from ovarian or extraovarian endometriosis. However, there are scant reports on the MR findings of extrauterine mullerian adenosarcoma arising from deep pelvic endometriosis. We describe here a case of a large infiltrating extrauterine mullerian adenosarcoma arising from recurrent deep pelvic endometriosis and we discuss its MR findings.

Tumores müllerianos mixtos de cuello uterino / Mullerian mixed tumors of the uterine cervix

Objetivo: Presentar 3 casos clínicos de tumores müllerianos mixtos de cuello uterino más revisión de la literatura. Método: Análisis retrospectivo de fichas clínicas desde noviembre de 1997 hasta enero de 2005 y revisión de las placas histológicas. Resultados: El rango de edad de las pacientes fue de 36 a 56 años (promedio: 48,3 años). El principal motivo de consulta fue la genitorragia. Al examen clínico presentaban un cuello uterino tumoral de gran volumen (5 a 7 cm). En dos pacientes se identificó un carcinosarcoma, en la otra paciente se diagnosticó un adenosarcoma. Al momento del diagnóstico no presentaban enfermedad extrapelviana evidente. Se realizó histerectomía radical y radioterapia pelviana más braquiterapia postoperatoria en todas ellas. Dos pacientes fallecen con enfermedad extrapelviana a los 11 y 20 meses de seguimiento. La otra paciente esta libre de enfermedad a los 48 meses de seguimiento. Conclusión: Los tumores müllerianos mixtos de cuello uterino son raros, de mal pronóstico cuyo tratamiento principal es la cirugía radical con radioterapia adyuvante en caso de enfermedad localmente avanzada. En casos confinados al cuello uterino se puede lograr una mayor sobrevida libre de enfermedad.

Objective: 3 clinical cases of mixed müllerian tumors of uterine cervix and a literature review are presented. Method: Retrospective analysis of clinical charts and pathological reports between November 1997 and January 2005. Results: Patients' ages ranged from 36 to 56 years (mean: 48,3 years). Abnormal vaginal bleeding was the most common presenting symptom. Pelvic examination revealed a large cervical mass (5 to 7 cm) in all cases. Two patients presented a carcinosarcoma, the other one an adenosarcoma tumor. At the initial diagnosis there were not evidence of extrapelvic disease. Radical hysterectomy and external beam radiation therapy and brachytherapy was performed in all cases. Two patients died with extrapelvic metasta-ses at 11 and 20 months of follow-up. The other one remains without evidence of recurrence 48 months after her treatment. Conclusion: Cervical mixed müllerian tumors are rare and poor prognosis neoplasms, radical surgery and adjuvant radiation therapy for local bulky disease are the optimal therapy. Long-term survival is possible in cervix confined early stage disease.