Extragenital malignant mixed Mullerian tumor in the incisional hernia - primary carcinosarcoma in the abdominal wall: case report.

INTRODUCTION: This report presents a primary Mullerian carcinosarcoma localized in the incisional hernia i.e. anterior abdominal wall.There is no data in the literature about this localization of extragenital Mullerian carcinosarcoma. CASE OUTLINE: The patient had previous medical history of right-sided ovarian cystadenocarcinoma managed by hysterectomy, bilateral ovariectomy and chemotherapy. An incisional hernia occurred 1 year after the operation and Mullerian carcinosarcoma at the right border of the incisional hernia 16 years later. There was no tumor spreading into the abdominal cavity and pelvis. Full thickness of the abdominal wall resection and coexisting incisional hernia resulted in a large 25x20 cm abdominal wall defect managed by the modified components separation technique and implanting meshes. CONCLUSION: Major abdominal wall resection and abdominal wall reconstruction using the modified components separation technique reinforced with meshes could be one of possible solutions in the surgical treatment of primary malignant mixed Mullerian tumor localized in the abdominal wall.

Primary uterine müllerian mucinous borderline tumor (MMBT) associated with adenomyosis: a case report.

Müllerian mucinous borderline tumors (MMBTs) usually arise from the ovary. The present report is the first case of primary uterine MMBTs associated with adenomyosis. A 51-year-old woman was referred to our hospital for a complex cystic and solid 4×3 cm right adnexal mass. She had a history of a left ovarian endometriotic cyst and had undergone a left oophorectomy 2 yr prior. A laparotomy was performed, and the tumor was found to be originating in the posterior wall of the uterus. She underwent a total abdominal hysterectomy, right salpingo-oophorectomy, and left salpingectomy. Microscopically, the solid portion of the tumor contained papillary proliferations of glands, which were covered by a mucinous epithelium with mild to moderate nuclear atypia, accompanied by stromal infiltration of inflammatory cells. Islands of adenomyosis were also observed around the cyst. These pathologic findings were similar to the features of ovarian MMBT. We diagnosed this tumor as a uterine MMBT, probably arising from adenomyosis.

Eight cases of malignant mixed Müllerian tumor (carcinosarcoma) of the uterus: findings in SurePath™ cervical cytology.

Malignant mixed Müllerian tumor (MMMT)/carcinosarcoma is a rare neoplasm of the female genital tract characterized by a mixture of epithelial and mesenchymal components. There are published reports of conventional cervical smear findings in uterine MMMT, but to the best of our knowledge the cytomorphology of MMMT in SurePath™ liquid-based cytology samples has not been described. We present a series of eight cases of uterine MMMT in SurePath™ cervical samples. The mean age of the women was 65.5 years (range, 60-82 years) and the most common presenting symptom was postmenopausal bleeding. Three out of the eight cases had both epithelial and sarcomatous elements in highly cellular cervical samples. In two cases, abnormal glandular cell clusters were closely associated with the mesenchymal component. The glandular component was usually represented by three-dimensional clusters of malignant glandular cells and a dispersed population of large, round to oval malignant epithelial cells with single prominent nucleoli. Necrosis or tumor diathesis was not seen in any cases. Six out of the eight cases were initially reported as adenocarcinoma, endometrial type, and two cases as normal. Five cases had an atrophic background and tiny atypical glandular clusters that could easily be overlooked. Although cervical sample is not the method of choice for primary uterine carcinosarcoma screening, the possibility of the same should be kept in mind, when reporting a cervical sample from a woman with postmenopausal vaginal bleeding.

Non-puerperal uterine inversion caused by malignant mixed mullerian sarcoma.

Non puerperal uterine inversions resulting from mixed mullerian uterine sarcoma are rare. We present a case of a postmenopausal woman with a large mixed mullerian tumour presenting as a huge abdominopelvic mass. It required a challenging surgical procedure to remove the tumour which is also described along with the review of literature.

Adenosarcoma mülleriano de útero: un tumor infrecuente de difícil diagnóstico / Uterine müllerian adenosarcoma: report of a case and review of the literature

El adenosarcoma de útero es una neoplasia rara y representa aproximadamente el 8% de todos los sarcomas uterinos. Está relacionado con antecedentes de tratamiento con tamoxifeno y con radiación pélvica. La clínica más característica es la de una lesión polipoide recidivante, siendo habitual que la paciente ya haya tenido algunas biopsias previas por ese mismo motivo, en las cuales no haya podido llegarse a un diagnóstico acertado y definitivo. Incluso puede haber llegado a darse un diagnóstico erróneo, de los cuales el más frecuente es el de un pólipo cervical. La lesión consta de un componente glandular benigno creciendo inmerso en un estroma sarcomatoso. Presentamos el caso de una mujer de 53 años con una lesión polipoide uterina. Tras los estudios macroscópico, microscópico e inmunohistoquímico se llegó al diagnóstico de adenosarcoma mülleriano de útero. Se trata de una neoplasia de bajo grado y buen pronóstico, pero que recidiva en un alto porcentaje de casos (25-40%). El tratamiento es la histerectomía total simple con doble anexectomía, seguida o no de radioterapia postoperatoria, aunque en pacientes jóvenes, que no tienen el deseo genésico cumplido, al ser una neoplasia de bajo grado, se han descrito casos en los que se ha optado por un tratamiento conservador. De todas formas, no hay que olvidar que se trata de un tumor maligno y que también se han descrito casos de fallecimiento por esta entidad a causa de metástasis abdominales o de otro tipo, por lo que somos de la opinión de que la mejor y más segura opción terapéutica es la histerectomía (AU)

Uterine adenosarcoma is a rare neoplasm constituting only around 8% of all uterine sarcomas. This tumor is associated with tamoxifen therapy and pelvic radiation. The most characteristic clinical feature is a recurrent cervical polypoid lesion. Patients have often had previous biopsies for the same reason, but without an accurate diagnosis. Furthermore, a mistaken diagnosis may have been made, the most common being cervical polyp. Uterine adenosarcoma consists of neoplastic glands with a benign appearance and a sarcomatous stroma. We report the case of a 53-year-old woman with a uterine polypoid lesion. After macroscopic, microscopic and immunohistochemical studies, the diagnosis was a uterine Müllerian adenosarcoma, which is a low-grade neoplasm with good prognosis, but with a high percentage of recurrences (25%-40%). Treatment is simple hysterectomy with double adnexectomy, with the option of subsequent postoperative radiotherapy. However, because this tumor is a low-grade neoplasm, a more conservative approach has sometimes been adopted in some young patients without children. This tumor is malignant, however, and mortality from abdominal or other types of metastases has been reported. Therefore, we believe that the most appropriate and safest therapeutic option is hysterectomy(AU)

Rotura uterina espontánea y hemoperitoneo como primera manifestación de tumor mixto mülleriano maligno. Informe de un caso de autopsia y revisión de la literatura / Spontaneous rupture of the uterus and hemoperitoneum secondary to a mixed müllerian tumour. A case report and review of the literature

Se presenta el caso de una mujer de 75 años de edad con malestar general y anemia progresiva de origen desconocido, que falleció por choque hipovolémico. Se realizó estudio de autopsia, donde se encontró rotura uterina y hemoperitoneo, a expensas de una neoplasia uterina maligna. En el estudio histopatológico se evidenció un tumor mixto mülleriano maligno heterólogo. Se realiza una revisión de la literatura acerca de la asociación de esta y otras patologías con ruptura uterina espontánea(AU)

A 75 year old female who died from hypovolemic shock after a period of progressive malaise and anaemia of unknown origin was seen on autopsy to have uterine rupture and haemoperitoneum secondary to a heterologous malignant mixed müllerian tumour. The literature on the association of spontaneous uterine rupture with this neoplasm and other conditions is reviewed(AU)

Malignant mixed Mullerian tumor of broad ligament with synchronous ovarian and endometrial carcinoma: a rare association.

Extragenital malignant mixed Mullerian tumor (MMMT) is a rare tumor in females, and it is even more rarely encountered among the multiple genital malignancies. There are some reports of extragenital MMMTs associated with synchronous or metachronous gynecologic tumors of Mullerian duct origin. We recently encountered an MMMT of broad ligament which is associated with papillary serous cystadenocarcinoma of the ovary and endometrioid adenocarcinoma arising in atypical polypoid adenomyoma endometrium in a 76-year-old woman. This case is presented for its rarity and unique presentation. To our knowledge, ours is the first reported case of this unique combination of multiple synchronous genital malignancies.

Primary mixed mullerian tumor of the vagina--a case report with review of the literature.

Malignant mixed mullerian tumor (MMMT) is a rare entity. The commonest site of this tumor in the female genital tract is the uterus followed by cervix. Primary MMMT of vagina is extremely rare. We are reporting this rare entity, with a brief review of the literature, in a 48-year-old perimenopausal female who presented with a history of passage of urine per vagina. On pelvic examination, a polypoidal mass arising from the anterior wall of the vagina was identified. Histopathological examination revealed the biphasic nature of the tumor. Immunohistochemistry confirmed the diagnosis of MMMT of vagina. To conclude, although rare, clinicians, oncologists, and pathologists should identify this malignant tumor for appropriate treatment and management.

Carcinoma of müllerian origin presenting as colorectal cancer: a clinicopathologic study of 13 Cases.

Carcinomas of müllerian origin involving colorectum in women with no concurrent or history of gynecologic malignancies are diagnostically challenging, and its histogenetic origin is uncertain. We reviewed 13 cases of carcinoma of müllerian origin with clinical presentation mimicking primary colorectal carcinoma. The patients' average age was 63.9 years. All except 2 patients presented with mass lesions in rectosigmoid colon or rectovaginal septum. The major presenting symptoms were rectal bleeding (4/13), rectosigmoid mass (6/13), vaginal mass (1/13), and abdominal pain or constipation (2/13). The average size of tumor was 4.2 cm (range, 2.4-15.0 cm). Among the 10 patients who underwent preoperative biopsy, 5 were diagnosed to have moderately and poorly differentiated colorectal carcinoma. All tumors were surgically resected with final diagnoses of moderately differentiated endometrioid carcinoma in 6 cases, mixed serous and endometrioid carcinoma in 4 cases, malignant mixed müllerian tumor in 2 cases, and undifferentiated carcinoma in 1 case. In 9 of 13 cases, foci of endometriosis were identified adjacent to or within the tumor. One case had endosalpingiosis. Immunohistochemical stains showed, after positive results, the following: cytokeratin 7 (CK7; 13/13), estrogen receptor (13/13), progesterone receptor (10/13), cytokeratin 20 (CK20; 0/13), and CDX-2 (0/13). In conclusion, carcinoma of müllerian origin often presents as bulky mass in rectosigmoid or rectovaginal septum clinically mimicking primary colorectal cancer. Endometriosis might be an important etiologic factor. Familiarities of this unusual clinicopathologic entity, careful morphologic evaluation, and immunohistochemical stain with a panel of markers (CK7, CK20, estrogen receptor, progesterone receptor, CDX-2) will be helpful for the correct diagnosis.

Agenesia congénita de endometrio. Una etiologia inusual de amenorrea primaria / Congenital absence of the endometrium: an unusual cause of primary amenorrhea

Se describe el caso de una paciente de 25 años que presenta amenorrea primaria y esterilidad como consecuencia de la agenesia congénita de endometrio, sin otro tipo de alteraciones fenotípicas, cromosómicas o endocrinológicas asociadas. Se discuten aspectos de la fisiopatología, el diagnóstico y las posibilidades terapéuticas en la actualidad (AU)

We report a case of primary amenorrhea and infertility due to congenital absence of the endometrium in a 25 year-old woman. There were no other malformations or endocrinological or chromosomal alterations. The physiopathological features, diagnosis and current therapeutic options in this malformation are discussed (AU)

Tumor mülleriano mixto maligno: la experiencia de un único centro con 43 casos de 1990-2006 / Mixed malignant Mullerian tumours: the experience of a single centre with 43 cases from 1990-2006

Objetivo: Se realiza un estudio descriptivo de las pacientes con diagnóstico de tumor mülleriano mixto maligno (TMMM) en términos de epidemiología, diagnóstico, tratamiento, seguimiento, recurrencias y supervivencia. Material y método: Se revisaron los archivos anatomo-patológicos y las historias médicas de las pacientes tratadas durante el período 1.990-2.006. Las supervivencias se analizan mediante las curvas de Kaplan-Meier. Se emplea un análisis de regresión logística en el estudio uni y multivariable.Resultados43 pacientes son incluidas en esta revisión. El tratamiento inicial fue quirúrgico en el 79% de los casos. El 34,9% de las pacientes se diagnosticaron en estadio I; 16,3% en estadio II; 34,9% en estadio III y 9,3% en estadio IV. Se alcanzó una respuesta completa en el 60,4%. La enfermedad persistió en el 39,6%. La supervivencia libre de enfermedad a los 2, 5 y 10 años fue del 50%, con una mediana de 15 meses (IC 95% 6-32). La tasa de recurrencia fue del 42,3% con un tiempo medio de 8,4 meses. La supervivencia total a los 2, 5 y 10 años fue del 26% con una mediana de 7 meses (IC 95% 1-44). En el análisis univariante el tamaño tumoral, la invasión linfovascular, el estadio y la radioterapia pélvica adyuvante son factores pronósticos. En el análisis multivariante la invasión linfovascular, el estadio y la radioterapia son factores pronósticos independientes. Conclusión: Los TMMM son tumores de comportamiento clínico extremadamente agresivo con un pronóstico pobre. Los factores pronósticos que afectan la supervivencia son el estadio, la invasión linfovascular y la radioterapia pélvica (AU)

Objective: Malignant mixed Müllerian tumours (MMMT) patients were retrospectively evaluated in terms of epidemiology, diagnosis, treatment, follow-up, recurrent disease and survival. Methods: Medical and histopathology records were reviewed during the 17-year period 1990-2006. Survival rates were analysed by means of the Kaplan-Meier technique. The Cox proportional hazards regression model was used in uni- and multivariate analysis. Results: A total of 43 patients were included in this study. First-line treatment was surgery in 79% of cases. Stage I, II, III and IV were identified in 34.9%, 16.3%, 34.9% and 9.3%, respectively. A complete response was achieved in 60.4% of patients. The disease was progressive in 39.6%. Event–free survival at 2, 5 and 10 years was 50% for all, with a median time of 15 months (95% CI,6-32). There was a 42.3% recurrence-rate with a mean time to recurrence of 8.4 months. The 2, 5 and 10-years overall survival was 26% with a median time of 7 months (95% CI, 1-44). In the univariate analysis tumour size, lymphovascular infiltration, stage and pelvic radiotherapy are prognostic factors. In the multivariate analysis lymphovascular infiltration, stage and radiotherapy were found to have an independent influence on overall survival. Conclusions: MMMT are tumours of aggressive clinical behaviour with a poor prognosis. Stage, lymphovascular infiltration and adjuvant radiotherapy are the dominant prognostic factors (AU)

Tumor mülleriano mixto maligno del cérvix uterino. A propósito de un caso y revisión de la literatura / Malignant mixed müllerian tumour of the uterine cervix. Report of a case and review of the literature

Introducción: Los tumores müllerianos mixtosmalignos (TMMM) de cervix o tumores mesodérmicosmixtos malignos son neoplasias infrecuentes con pocomás de 50 casos descritos en la literatura y probablementeconstituyen <3% de todos los TMMM uterinos. Sonmuy agresivos y generalmente se asocian a mal pronóstico.Método: Hemos revisado las características histológicas,clínicas y la literatura médica. Secciones representativashan sido teñidas con hematoxilina-eosina. El estudioinmunohistoquímico se realizó con el método estreptavidina-biotina-peroxidasa para citoqueratina, antígeno carcinoembrionario,CD10, calretinina, vimentina, desminay mioglobina. Resultado: El tumor es bifásico compuestode una mezcla de componente epitelial y mesenquimalhomólogo y heterólogo. Las tinciones inmunohistoquímicasmostraron positividad para citoqueratina y antígenocarcinoembrionario y negatividad para CD10, calretininay vimentina en el componente epitelial. El componenteheterólogo mostró positividad para desmina y mioglobina.Conclusión: Aportamos un nuevo caso de tumormülleriano mixto maligno originado en cérvix uterino yrevisamos la literatura(AU)

Introduction: Malignant mixed müllerian tumors(MMMTs) or malignant mixed mesodermal tumours of thecervix are exceptionally rare, probably accounting for<3% of all uterine MMMTs; fewer than 50 cases havebeen reported to date. They tend to be highly aggressiveand generally have a poor prognosis. Methods:We presentthe clinical and histological features of a case of MMMTof the cervix and review the literature. Representative sectionswere stained with hematoxylin-eosin and immunohistochemistrywas performed using the streptavidin-biotinperoxidase method for cytokeratin, carcinoembryonicantigen, CD10, calretinin, vimentin, desmin and myoglobin.Results: the tumour was biphasic, composed of anadmixture of neoplastic epithelial and homologous andheterologous mesenchymal components. The epithelialcomponent was positive for cytokeratin and carcinoembryonicantigen and negative for CD10, calretinin andvimentin. The heterologous component was positive fordesmin and myoglobin. Conclusions: we report a case ofmalignant mixed müllerian tumour arising in the uterinecervix and review the literature(AU)

Mullerianosis vesical: una entidad infrecuente / Mullerianosis of the urinary bladder: a rare entity

OBJETIVO: Descripción de un nuevo caso de mullerianosis vesical.MÉTODO: Presentamos el caso de una paciente mujer de 30 años con antecedentes de aborto, que refiere molestias miccionales coincidiendo con las menstruaciones. Una ecografía vaginal demostró la existencia de lesión exofítica vesical, confirmada posteriormente por cistoscopia. Se indicó resección transuretral.RESULTADOS: En el estudio histopatológico de los tejidos obtenidos se objetivó un componente glandular mixto de tipo predominantemente tubárico, con elementos endometriales y endocervicales asociados. No evidencia de recidiva endoscópica tras un año de seguimiento.CONCLUSIONES: Aportamos un nuevo caso de mullerianosis vesical. Destacamos el escaso número de casos publicados. Defendemos la opción quirúrgica endoscópica en estas pacientes(AU)

OBJECTIVES: To report a new case of bladder mullerianosis.METHOD: We present the case of a 30 year old female patient with history of miscarriage, who refers voiding dis-turbances with menstruations. Vaginal ultrasound showed an exophytic bladder lesion, which was confirmed by cistoscopy. Endoscopic resection was indicated.RESULTS: The pathological study of tissues obtained showed mixed glandular structures with predominant tubaric-like type, in association with endometrial- and endocervical-like elements. No evidence of endoscopic relapse after one year of follow-up.CONCLUSIONS: We contribute with a new case of bladder mullerianosis. We emphasize the scarcity of its pu-blished reports. We support the option of an endoscopic surgery for these patients(AU)

Feasibility and morbidity of total laparoscopic radical hysterectomy with or without pelvic limphadenectomy in obese women with stage I endometrial cancer.

OBJECTIVE: The aim of this study was to describe the feasibility and morbidity rates associated with total laparoscopic radical hysterectomy (TLRH) with or without pelvic lymphadenectomy for stage I endometrial cancer in obese women. PATIENTS AND METHODS: Obese patients with stage I endometrial cancer who underwent total laparoscopic radical surgery at the Department of Obstetrics and Gynecology of San Gerardo Hospital were compared to nonobese patients. The same group of obese patients was compared with patients who underwent radical laparotomic surgery. Obesity was defined as a body mass index more than 30 kg/m(2). RESULTS: Between September 2003 and September 2007, 75 women underwent TLRH. Median age was 54 years and median body mass index was 28 kg/m(2). Thirty-seven women were obese. There were no differences between nonobese and obese women in operative, time length of parametria and pelvic nodes removed and operative or late complications. Blood loss was significantly higher in obese patients. Comparing retrospectively laparoscopy and laparotomy in obese women treated in our center, laparotomy was associated with decreased operative time, but also with increased blood loss, transfusion rate, duration of hospitalization and frequency of post surgical complications. CONCLUSIONS: Total laparoscopic radical hysterectomy (with pelvic lymphadenectomy) is a safe option in patients with endometrial cancer. Obesity is not a contraindication to perform a TRLH with no differences in surgical parameters between obese and nonobese population. TLRH show a significant decrease of complications compared to laparotomic radical surgery in obese women.

A sarcomatous-type peritoneal malign mixed mullerian tumor implant in association with ovarian adenocarcinoma: a case report.

A rare case of a patient with a histopathological diagnosis of a sarcomatous-type peritoneal malign mixed müllerian tumor implant in association with ovarian adenocarcinoma is reported. A 52-year-old patient was referred to our clinic for an adnexal mass. At pelvic examination, an irregular, fixed, approximately 7-8 cm in size mass was detected in the right adnexal area. At transvaginal ultrasonographic examination, it was observed that there was an 80 x 70 mm sized, irregularly contoured, semisolid mass with hyperechogenous areas inside originating from the ovary in the right adnexal area. At laboratory examination tumor marker CA-125 was 280.4 U/ml (< 35), CA-15-3 was 146.5 U/ml (< 25), whereas other markers were within normal range. The patient was operated on for a right adnexal mass. A staging laparatomy procedure was applied. Postoperative histopathological diagnosis was reported as malignant mixed mullerian tumor of the ovary, with the ovarian component as poorly differentiated adenocarcinoma, and the metastatic foci over serosal surfaces as a sarcomatous component. Postoperatively six courses of adjuvant and consolidation chemotherapy were administered to the patient. Further studies are needed to set a consensus about evaluation of treatment and prognosis for this kind of pathology.

Malignant mixed müllerian tumor of the fallopian tube coincident with a primary serous carcinoma of the ovary. Case report.

Malignant mixed müllerian tumors are very rare neoplasms of the fallopian tube, and treatment is not well defined. A case of malignant mixed müllerian tumor of the tube concomitant with a primary serous carcinoma of the ovary is reported. It was unclear if there were two distinct neoplasms in the same patient, or if it was a single tumor with a sarcomatous fallopian conversion of the serous component, as described for some recurrent ovarian carcinomas. Chemotherapy for ovarian carcinoma with intraperitoneal metastasis was performed, with about a three-year interval-free period of disease, as could be expected for ovarian carcinomas at the same stage. Such coexistence of these two tumors does not afford adequate staging of the malignancy. Therapy for the very rare cases similar to the one reported here needs to be improved.

Paraneoplastic subacute sensory neuronopathy secondary to a malignant mixed mullerian tumor.

BACKGROUND: Paraneoplastic subacute sensory neuronopathy is a rapidly progressive autoimmune disorder commonly associated with small cell cancers. Relentless destruction of dorsal root ganglion cells by cytotoxic T cells leads to a poor prognosis. CASE: A 42-year-old woman developed sensory loss in both lower extremities 10 days after debulking of a uterine malignant mixed müllerian tumor. She progressed to sensory loss over the entire body, with initially preserved strength, severe dysmetria, and truncal ataxia. Her serum was positive for antineuronal nuclear antibody-1 (anti-Hu), confirming the diagnosis of paraneoplastic subacute sensory neuronopathy. Despite treatment with intravenous immunoglobulin, methylprednisolone, and plasmapheresis, she remained severely disabled. CONCLUSION: Because neuronal damage is irreversible, early recognition may be the only means to prevent severe neurologic disability.

Non-puerperal uterine inversion due to uterine sarcoma.

BACKGROUND: Uterine inversion is a very rare pathological condition that usually occurs in puerperium. Non- puerperal uterine inversion is exceptional and to our knowledge only a few cases of uterine inversion due to a uterine sarcoma have been reported. CASE REPORT: A 79-year-old woman, gravida 0, para 0, presented with vaginal bleeding. Pelvic examination under anesthesia revealed a huge mass coming from the cervix filling the vagina to the introitus, and rectal examination could not identify the uterus. Diagnosis of uterine inversion was made and the patient was submitted to total abdominal hysterectomy with bilateral salpingo-oophorectomy and pelvic lymph node sampling. The postoperative course was uneventful and the patient was discharged on the 5th postoperative day. She underwent postoperative pelvic radiation, and no recurrence was found during the 19-month follow-up period. CONCLUSION: Chronic non-puerperal uterine inversion can be considered a rare complication of malignant mixed mullerian tumor of the uterus.