[Sarcomas and mixed mesodermal tumors of the uterus]. / Mesenchymale Tumoren des Uterus.

Malignant mesodermal tumors of the uterus are an inhomogenous group of uterine malignancies with different pathogenesis, clinical presentation and prognosis. These rare tumors represent approximately 1 % of all uterine malignancies. The aggressive carcinosarcomas or mixed muellerian tumors are defined by mixed malignant epithelial and malignant mesodermal histopathology and are of the same precursor cell origin like endometrial cancer. Thus, carcinosarcomas were reclassified by the FIGO as an aggressive type of endometrial cancer and treated like type II endometrial cancer. Adenosarcomas are also mixed tumors with benign epithelial proliferation and malignant mesodermal cell growth, have a good prognosis and represent less than 5 % of all mesodermal uterine malignancies. Besides carcinosarcomas, the pure mesodermal leiomyosarcomas are the most common mesodermal malignancies. Patients with leiomyosarcamos are usually perimenopausal, and although more than half of the patients present with symptoms, diagnosis occurs incidentally in most cases in final histopathologic workup of an excised putative myoma or uterus. Adequate anamnesis, gynecologic examination and careful imaging by transvaginal ultrasound in the preoperative setting might hint to correct differential diagnosis in many cases. Overall the prognosis of uterine leiomyomas is poor. Malignancies of the endometrial stroma are very rare and divided in two subgroups, the mostly estrogen receptor positive endometrial stromal sarcoma, which occur preferably in premenopausal women and show a favorable prognosis, and the very aggressive undifferentiated endometrial sarcomas. The more rare undifferentiated endometrial sarcomas occur in postmenopausal women and most patients die in the first two years after diagnosis. Risk stratification of preoperative differential diagnosis requires improvements and the correct histopathologic workup of mesodermal uterine malignancies is still a challenge for pathologists.

A pilot study of cisplatin, ifosfamide and mesna in the treatment of malignant mixed mesodermal tumors of the ovary.

PURPOSE: To evaluate the efficacy and toxicity of cisplatin and ifosfamide in the treatment of patients with malignant mixed mesodermal tumor (MMMT) of the ovary. METHODS: Ten patients with histologically confirmed primary MMMT of the ovary diagnosed between 1993 and 2001 were enrolled in the study. Treatment consisted of cisplatin 75 mg/m2 on day 1, followed by ifosfamide 2.0 g/m2 over 24 h on days 1, 2 and 3. Mesna, 400 mg/m2, was given IV immediately prior to and 4 and 8 h after the start of each ifosfamide infusion. Chemotherapy was repeated on a 28-day cycle if blood counts permitted. Standard response criteria were used. Nine patients were evaluable for response. RESULTS: Eight of the nine patients responded to therapy, with 7 complete responses (78%) and 1 partial response. Seven of the eight responders (87.5%) eventually recurred. The median progression-free survival was 10 months (range 0-94.4 months). The median overall survival was 17.1 months (range 8-125.5 months). One patient remained free of disease 94.4 months after diagnosis, and one patient remained alive with recurrence 125.5 months following diagnosis. There were 13 grade 3 toxicities and 4 grade 4 toxicities. Four patients had grade 4 and three had grade 3 neutropenia, all of which required dose reductions. CONCLUSION: The combination of cisplatin and ifosfamide/mesna demonstrated activity against MMMT of the ovary. Response durations were short, however, and the regimen was associated with significant toxicity. Novel agents with activity against MMMT of the ovary and acceptable toxicity are needed.

Adjuvant ifosfamide and cisplatin in patients with completely resected stage I or II carcinosarcomas (mixed mesodermal tumors) of the uterus: a Gynecologic Oncology Group study.

OBJECTIVES: To determine progression-free survival (PFS) and overall survival (OS) in women with completely resected stage I or II carcinosarcoma of the uterus treated with adjuvant ifosfamide and cisplatin, and to assess the toxicity of this regimen. METHODS: Eligible patients had histologically confirmed carcinosarcoma (mixed mesodermal tumor) and no postoperative radiotherapy following complete resection for clinical stage I or II disease. They were to have adequate renal, hepatic, and hematologic functions and performance status of 2 or less. Study entry was to be within 8 weeks of hysterectomy. Patients with previous chemotherapy, or other noncutaneous malignancies, were ineligible. Ifosfamide was administered 1.5 g/m2 intravenously (IV) over 1 h and cisplatin was given 20 mg/m(2) over 15 min followed by mesna 120 mg/m2 IV bolus, then 1.5 g/m2/24 h as a continuous infusion. Initial doses (daily x 5 every 21 days x 3 cycles) were reduced by 20% (to 4 days) for myelotoxicity. RESULTS: Nine of seventy-six patients enrolled were deemed ineligible and another two who did not receive protocol treatment were inevaluable. Of the 65 evaluable patients, median age was 65 years; 50 patients (77%) were stage I and 15 (23%) were stage II. PFS and OS, respectively, were 69% and 82% at 24 months, and 54% and 52% at 84 months. Overall 5-year survival was 62%. Leukopenia was the most commonly reported, but manageable, toxicity. CONCLUSION: Adjuvant ifosfamide and cisplatin after primary surgery for stage I or II carcinosarcoma of the uterus is tolerable. In the absence of concurrent controls, the impact on PFS and OS is unclear. Pelvic relapse remains problematic.

Mesodermal adenosarcoma of the testis.

Biphasic neoplasms with a benign to atypical epithelial component and a usually low-grade malignant stromal component have been reported in various sites, probably being best known for their occurrence in the uterine corpus (mullerian adenosarcoma). We report a tumor of this type that occurred in the testis of a 76-year-old man and, to our knowledge, is the first mesodermal adenosarcoma reported at this site. The patient had scrotal swelling for many years with a pronounced increase in the swelling over the past 2 years. A large complex solid-cystic testicular tumor was evident on ultrasound, and examination of a radical orchiectomy specimen showed a 6.5-cm mass. On microscopic examination, the neoplasm had a phyllodes-like appearance with bland cuboidal to flattened epithelium covering polypoid fronds, and lining glands and cysts. The stroma varied from cellular, particularly where it condensed around the glands and cysts, to hypocellular and hyalinized. It was immunoreactive for muscle specific actin, CD10, and to a lesser degree, desmin. This case expands the known sites where mesodermal adenosarcoma may occur. The histogenesis is speculative, but possible options are discussed.

[Malignant mixed mesodermal tumor: histo- and cytopathologic correlations]. / Tumora mixta mezodermica maligna. Corelatii cito-histopatologice.

To correlate the cytopathological and the histopathological findings in uterine mixed mesodermal malignant tumor (MMMT) we have examined the cervical smear, endometrial curettage and hysterectomy specimen of a patient diagnosed with uterine tumor. The smear was stained by Papanicolaou staining and the tissue processed by routine technique and stained H&E. The original cytological diagnosis was adenosquamous carcinoma. The histopathological diagnosis was MMMT of heterologous type. A review of the smear revealed features which may orientate the diagnosis: multinucleate cells, isolated cells with cyanophilic cytoplasm, hyperchromatic nuclei and prominent nucleoli, elongated cyanophilic cells of sarcomatous origin. We conclude that the cytopathological diagnosis of the MMMT in cervical smears is very difficult. This may be sustained by the evidence of more cell types and cellular features orientating to a sarcomatous origin. The most important differential diagnosis is adenosquamous carcinoma.

Malignant mixed mesodermal tumor in a young woman with polycystic ovary syndrome. A case report.

BACKGROUND: Malignant mixed mesodermal tumors (MMMTs) are uncommon, highly aggressive tumors of the uterus composed of both carcinomatous and sarcomatous elements, both likely to be derived from the same original stem cell. There is a strong association between endometrial adenocarcinoma and polycystic ovary disease. However, only two cases of MMMT occurring in women with polycystic ovaries have been reported. CASE: A 36-year-old woman with polycystic ovary disease developed an MMMT of the endometrium. CONCLUSION: Some cases of MMMT may be estrogen related.

Pólipo estromal mesodérmico de la vagina: estudio clínico-patológico e inmunohistoquímico de cuatro casos y revisión de la literatura / Mesodermic strumal polyps of the vagina: clinical pathological and immunohistochemical study in four cases and review of the literature

El pólipo estromal mesodérmico de vagina es una lesión benigna, asintomática que se diagnóstica en forma incidental y se presenta a cualquier edad (recién nacidas a 71 años). Se localiza en las paredes laterales en el tercio inferior de la vagina, es generalmente único, de aspecto digitiforme o nodular. Aun cuando su origen no es claro, la presentación en mujeres embarazadas o con manejo hormonal hace suponer que la estimulación hormonal juega un papel relevante en su desarrollo. Se informan cuatro casos estudiados en el Instituto, la edad de presentación osciló entre 19 y 54 años, dos pacientes se encontraban embarazadas y dos con manejo hormonal, el diagnóstico se realizó en todas de forma incidental, el tamaño de las lesiones varió de 1.5 a 5 cm de eje mayor. Se efectuó excisión local en todos los casos, sin presentar recurrencias con un seguimiento de 1 a 40 meses. Histológicamente eran bien circunscritos, revestidos por epitelio escamoso, el estroma incluyó un espectro de lesiones constituidas por tejido fibroconectivo laxo, poco celular sin atipias, hasta lesiones constituidas por estroma fibroconectivo denso, con aumento en la celularidad, presencia de atipias y escasa actividad mitótica . Los receptores hormonales fueron positivos en todos lo casos. Estos resultados apoyan el comportamiento indolente y la influencia de los factores hormonales en el desarrollo de estas lesiones.

Abnormal uterine cavity: differential diagnosis with MR imaging.

The objective of the study was to assess the usefulness of magnetic resonance (MR) imaging in distinguishing malignant from benign conditions in patients with an abnormal uterine cavity. Fifty-four patients that were suspected of having abnormal uterine cavities were retrospectively evaluated by using MR imaging. The diagnosis of an abnormal uterine cavity included a thickened endometrium, and/or a endometrial mass, and/or a submucosal mass. Threshold values to classify the uterine cavity as abnormal on sagittal T2-weighted images were >10 mm for premenopausal women and >5 mm for postmenopausal women. Malignancy was diagnosed when lesions invaded the myometrial/junctional zone, and/or lesion enhancement was lower than that of the adjacent myometrium. The results found that histology confirmed 18 malignant and 37 benign lesions. Twelve of 15 endometrial carcinomas and 3 malignant mixed mesodermal tumors (MMMT) were correctly characterized as malignant on enhanced T1-weighted images; whereas 6 of 15 endometrial carcinomas and 3 MMMT were correctly characterized on T2-weighted images. Thirty-four of 37 benign cases were correctly characterized as not malignant on enhanced T1-weighted images. One of 14 submucosal leiomyomas, one endometrial stromal metaplasia, and one of ten pathologically normal endometria were misdiagnosed on enhanced T1-weighted images but were correctly diagnosed on T2-weighted images. The overall sensitivity, specificity, and accuracy for distinguishing malignant from benign central uterine masses were 83%, 92%, and 89% for enhanced T1-weighted image, and 50%, 97%, and 82% for T2-weighted image, respectively. We came to the conclusion that in diagnosing patients with abnormal uterine cavity, MR imaging may help differentiate malignant from benign disorders.

Malignant mixed mesodermal tumors of the ovary: preoperative diagnosis.

Early diagnosis of malignant mixed mesodermal tumors of the ovary is very difficult because of the rarity and the insidious onset. The purpose of this report is to review the magnetic resonance imaging features of an ovarian malignant mixed mesodermal tumor, which occurred in a 52-year-old woman, aiding in the differential diagnosis.

Malignant mesodermal mixed tumor of the ureter.

Malignant mesodermal mixed tumor comprised of epithelial and nonepithelial components is rarely encountered in the urological field. Only 6 cases of malignant mesodermal mixed tumor of the ureter have previously been reported in the literature. We recently encountered a 7th case of malignant mesodermal mixed tumor of the ureter and describe the case in detail. The etiology of malignant mesodermal mixed tumor is also discussed.

[Malignant mixed mesodermal tumors of the ovary: a clinical analysis of 12 cases].

OBJECTIVE: To explore the clinical characteristics, treatment and prognostic factors of patients with malignant mixed mesodermal tumors of the ovary (MMMTO). METHODS: Clinical data of 12 patients with MMMTO between 1983-1997 were reviewed retrospectively. The median age of the patients was 50.7 years. According to FIGO staging system (1985), there were 4 cases in stage II, 6 in stage III and 1 in stage IV. Staging was unclear in the remaining case. Heterologous and homologous elements were present in the primary ovarian tumors in 4 and 8 of the 12 patients, respectively. All patients received cytoreductive surgery. Eleven of them received postoperative chemotherapy and 3 of these eleven patients received additional radiotherapy of pelvic field. RESULTS: The median survival of this group of 12 patients was 24 months. The 2- and 5-year survival rate was 33.3% (4/12) and 8.33% (1/12), respectively. The 2-year survival rate of stage II and III was 50% and 33.3% respectively. One patent in stage IV died within 1 year. Four of the 8 patients with homologous histology survived over 2 years while all the 4 cases with heterologous histology died within 2 years. Five of the 11 patients (45.5%) received combination therapy died within 1 year while 1 patient treated with surgery alone died within 6 months. CONCLUSIONS: The prognosis of MMMTO is rather poor. Surgery combined with chemotherapy and/or radiotherapy seems to be a better choice of approach. The histological tpyes of tumor and stage of the disease are related to prognosis.

Extragenital malignant mixed mesodermal tumor: case report and review of the literature.

Malignant mixed mesodermal tumors rarely occur in extragenital sites. An 80-year-old patient presented with a malignant mixed mesodermal tumor, consisting of papillary serous adenocarcinoma and chondrosarcomatous components. The patient refused further treatment following surgery and died of disease 2.5 months later. Based on the present case and a review of the literature, primary extragenital malignant mixed mesodermal tumors prove to be rare but highly malignant neoplasms. Further data is yet to be gathered in order to determine the exact origin and behavior of these tumors.

A mixed mesodermal tumor of the cecum: report of a case.

We herein report the case of a 69-year old woman presenting with an abdominal mass, who was found to have a mixed mesodermal tumor (MMT) of the cecum. Imaging studies and endoscopic investigations were consistent with the diagnosis of a nonepithelial malignant tumor of the cecum. On laparotomy, a knuckle-sized firm mass involving the cecum was noticed. As a result, a right hemicolectomy was performed. Pathological examinations, including immunohistochemical staining, resulted in the diagnosis of mesodermal mixed tumor, homologous type. The patient was advised to undergo postoperative chemotherapy but she did not comply. She has been followed up as an outpatient and is still alive 1.5 years after the operation.

Mixed mesodermal sarcoma of the ovary in a young patient.

Mixed mesodermal tumors (MMT) of the ovary are rare and have a poor prognosis. This ovarian malignancy usually occurs in postmenopausal women. We report an unusual ovarian MMT in a young woman given treatment similar to one used for ovarian germ cell malignancies. We believe this is the youngest patient reported with an homologous MMT of the ovary.

Ultrasonographic appearance of fallopian tube carcinoma.

Preoperative diagnosis of tubal carcinoma is difficult and a diagnosis cannot usually be established until the time of operation. However, since prognosis is strictly related to the stage of the neoplasm, it is very important to be familiar with the clinical and imaging characteristics of primary fallopian tube carcinoma in order to make an early and accurate diagnosis. This report presents the ultrasonographic features of three cases of fallopian tube carcinoma and reviews the literature on the subject.

Estrogen and progesterone receptors in malignant mixed mesodermal tumors of the ovary.

Malignant mixed mesodermal tumors of the ovary occur in less than 1% of cases of ovarian cancer. They have a dismal prognosis and the most effective type of therapy is still not known. All cases of malignant mixed mesodermal tumor of the ovary between January 1, 1985 and May 1, 1994 operated on by the gynecologic oncology service are the subject of this report. Data were obtained from the hospital and office records of the patients. Nine patients who had their primary surgery by the gynecologic oncology service were found to have the diagnosis of malignant mixed mesodermal tumor of the ovary. Homologous tumors were found in five patients and heterologous tumors in four. Homologous tumors (mean survival 15.2 months) showed a better survival than heterologous tumors (mean survival 6.5 months; P = 0.001). An elevated estrogen receptor status was shown to correspond to longer survival (P < 0.0001). Six specimens were considered to be estrogen receptor positive and three were receptor negative. The mean survival in those patients who had a positive estrogen receptor status, 13.7 months, with a median of 7.5 months was significantly higher than those who were not positive, 6.7 months (P = 0.019) with a median of 6.25 months. All specimens were progesterone receptor negative. Malignant mixed mesodermal tumors of the ovary have a dismal prognosis, no effective therapy, and controversial prognostic indicators. Increasing estrogen receptor status appeared to correlate with longer mean survival. Larger, multi-institutional studies need to be done to determine the overall significance of these findings.

[Synchronous growth of a mixed mesodermal tumor and an adenocarcinoma of the urinary bladder]. / Synchrones Wachstum eines mesodermalen Mischtumors und eines Adenokarzinoms in der Harnblase.

The case of synchronous growth of a mesodermal mixed tumour and an adenocarcinoma in the bladder is presented. This is a rare phenomenon. Mesodermal mixed tumours are malignancies with both epithelial (carcinoma) and mesenchymal (sarcoma) differentiation. The term carcinosarcoma is frequently used in the literature. In most cases the prognosis of mesodermal mixed tumours, which depends mainly on the high rate of local recurrences, is poor. Therefore, cystectomy with suprapubic urinary diversion is the treatment of choice. However, results reported in the literature show that transurethral resection of the tumour may be adequate when there is only superficial growth of mesodermal mixed tumours. Short follow-up intervals are absolutely essential for patients treated this way.

Sarcoma mesodérmico misto maligno do ovário / Malignant mixed mesodermic sarcoma of the ovary

É descrito um caso raro de sarcoma mesodérmico ou mülleriano misto maligno do ovário. Säo tecidas consideraçöes quanto à incidência, histogênese, anatomia patológica, quadro clínico, vias de propagaçäo e tratamento. O prognóstico é reservado, com baixos índices de sobrevida