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PubMed1996

Ovarian teratomas associated with the insertion of an imprinted transgene.

Ovarian teratomas are tumors that arise from female germ cells and are often a mixture of immature embryonal carcinoma cells and mature embryonic cells. Tissues derived from all three primary embryonic lineages (ectoderm, mesoderm, and endoderm) are typically found in the mature elements of a teratoma. In the case of the transgenic mouse line TG.KD, created with an imprinted transgene construct, malignant ovarian teratomas of a mixed germ cell tumor morphology occur in 15-20% of hemizygous female carriers of the transgene. The tumors frequently metastasize and can result in death of the mouse. Genetic analysis indicates that the tumors are associated with the transgenes integration site. Inbred FVB/N and female mice of other transgenic lines, also created in the inbred FVB/N strain with the same DNA construct as TG.KD, do not develop teratomas. In addition to teratomas, the integration of the transgene on Chromosome (Chr) 8 is associated with a perinatal lethality in homozygous transgenic carriers. The hemizygous genotypes of the teratomas suggest that they arise from early germ cells, prior to the completion of meiosis I.

Animals↗
PubMed1989

Endometrial stromal sarcoma: review of Mayo Clinic experience, 1945-1980.

Uterine sarcomas are uncommon malignancies. We retrospectively examined 24 cases of "high grade" endometrial stromal sarcomas (ESS). The histologic diagnosis was made if there was a high grade sarcoma with a high mitotic index and nuclear anaplasia. Heterologous elements could be present, if focal only. Leiomyosarcomas, mixed mesodermal sarcomas, and carcinosarcomas were excluded. Mitotic index was expressed as the number of mitoses per 10 high-power fields in active areas. Tumor grade was based on nuclear anaplasia. ESS were 5.7% of all uterine sarcomas. Median age was 58.2 years; median duration of symptoms was 67.5 days. Abnormal bleeding was the presenting complaint in 91.7%. Uterine enlargement was seen in 33%; protrusion through the cervical os was seen in 9 cases. Total abdominal hysterectomy and bilateral salpingo-oophorectomy was performed in 95.8%. Twenty patients had myometrial involvement; 5 patients had serosal spread and extrauterine disease; residual disease was seen in 3 patients. Adjuvant radiation was not beneficial. Ten patients had recurrences: 60% in the pelvis, 50% each with subcomponent of abdominal or distant disease. Overall survival was 3.4 years and was significantly associated with extent of disease, size of the primary tumor, and grade.

Adult↗
PubMed2001

True malignant mixed tumor (carcinosarcoma) of parotid gland with unusual mesenchymal component: a case report and review of the literature.

True malignant mixed tumor (carcinosarcoma) of the salivary gland is an extremely rare tumor. By definition, it is composed of both malignant epithelial and malignant mesenchymal elements. The most common type of the former is squamous cell carcinoma or adenocarcinoma and the most common type of the latter is chondrosarcoma, followed in frequency by fibrosarcoma, leiomyosarcoma, osteosarcoma, and in rare instances liposarcoma. We report a case of true malignant mixed tumor of the parotid gland in association with a pleomorphic adenoma in a 47-year-old man that contained a very unusual type of malignant mesenchymal component, rhabdomyosarcoma. Cytologic and histologic features and immunohistochemical results are presented. In addition, the literature is reviewed, and the possible histogenesis and pathogenesis of malignant mixed tumor of the salivary gland are briefly discussed.

Actins↗
PubMed1990

Treatment of uterine sarcomas.

During a 21-year period, 66 patients with uterine sarcomas were treated at California Medical Center. Histological diagnoses were mixed mesodermal sarcoma in 32 patients (48%), leiomyosarcoma in 24 (36%), and endometrial stromal sarcoma in 10 (15%) patients. The majority of patients (73%) had Stage I tumors. The treatment consisted of surgery alone in 27 (41%), surgery in combination with radiation therapy in 36 (55%), and radiation therapy alone in three (4%) patients. The overall 1-, 2-, and 5-year actuarial survival was 74%, 57%, and 38%, respectively. The 1-, 2-, and 5-year actuarial survival for the 27 surgery alone patients was 73%, 50%, and 25%, which compared with 75%, 61%, and 44% for the 36 surgery plus radiation therapy patients (P = 0.12). The disease-free survival was better for the surgery plus radiation therapy patients, as compared with the surgery alone group (38% vs. 18% at 5 years, P = 0.081). The 5-year survival by histology was 70% for the 10 endometrial stromal sarcoma patients, 40% for the 24 leiomyosarcoma patients, and 23% for the 32 mesodermal sarcoma patients (P = 0.064). As expected, survival depended on the stage of disease (P less than 0.0001). Treatment failure was observed in 35 (53%) patients, which included 9 (14%) with failure in the pelvis. There was no difference in the incidence of failure among patients in the three treatment groups and also in the three histologic groups. There was, however, a significant difference in the incidence of pelvic failure between surgery alone and surgery plus radiation therapy patients. In the 27 surgery alone patients, nine (33%) relapsed in the pelvis, whereas none of the 36 surgery plus radiation therapy patients had locoregional failure, P less than 0.0001. Adjuvant radiation therapy is an important treatment in the management of patients with sarcoma of the uterus.

Adult↗
PubMed2003

Severe anemia in a 25-day-old infant due to gastric teratoma with focal neuroblastoma.

Gastric teratoma is an extremely rare embryonic neoplasm containing tissue from all three germ layers (ectoderm, endoderm, and mesoderm). A 25-day-old infant presented with anemia and a palpable abdominal mass. Computed tomography (CT) scanning showed a solid and cystic multiloculated tumor of mixed echogenicity and calcifications. An encapsulated bleeding tumor localized to the posterior wall of the stomach with extension to a bowel segment was promptly excised. Histology revealed a grade III immature teratoma with small foci of neuroblastoma. To the best of our knowledge, this is the first infant with gastric teratoma containing foci of neuroblastoma causing bleeding and anemia as presentation symptoms at neonatal age.

Anemia↗
PubMed1985

[Cytomorphological diagnosis of nonepithelial tumors of the uterus].

Retrospective histocytological correlations are performed of the material from 38 patients with non-epithelial uterine tumours. Cytomorphological features of highly, moderately and poorly differentiated leiomyosarcoma, leiomyoblastoma, endometrial stromal sarcoma, mixed mesodermal tumours and botryoid sarcoma are established.

Biopsy, Needle↗
PubMed2004

Human mesenchymal stem cells suppress induction of cytotoxic response to alloantigens.

Mesenchymal stem cells (MSC) fail to induce allogeneic responses in mixed lymphocyte reaction assays. Because MSC express HLA class I molecules, here we investigated whether they could be recognized as allogeneic targets by cytolytic T lymphocytes (CTL). With this aim, CTL precursor (CTLp) frequencies were measured following stimulation of T cells with either allogeneic mononuclear cells (MNC) or MSC originated from the same human bone marrow donor. Lysis of MSC was measured at day 10 of culture in standard chromium release assays. In addition, allogeneic PHA blast T cells or B-EBV lymphoblastoid cell lines (LCLs) generated from the same donor were used as positive controls of lysis. Our results showed that when allogeneic MNC were used to stimulate T cells, a high CTLp frequency was detected towards MSC targets. However, when MSC were used as stimulators, CTLp frequencies were markedly altered whatever the targets used, i.e.: MSC, PHA blast T cells or EBV-B LCLs. Moreover, when graded concentrations of MSC were added together with MNC upon stimulation of alloreactive T cells, we observed a dose-dependent decrease in CTLp frequencies towards MSC targets. This inhibition of MSC lysis was partially overcome by adding exogenous rh-IL-2 from the beginning of cultures. In addition, this suppressive effect was totally reproduced when, instead of MSC, supernatant harvested from MSC cultures was added to allogeneic MNC, upon stimulation of alloreactive T cells. In conclusion, our results demonstrate that MSC which can be recognized as targets by pre-activated alloreactive CTLs, may be able to suppress differentiation of CTL precursors into CTL effectors through secretion of suppressive factors.

Cell Differentiation↗
PubMed1984

[Uterine sarcoma. Retrospective analysis of a series of 22 cases].

37 uterine tumors originally diagnosed as uterine sarcomas at Fondation Bergonié between 1970 and 1982 were histologically reclassified. 15 patients were excluded from this study because of inadequate data or not confirmed initial diagnosis. The 22 remaining cases were classified as leiomyosarcomas 13, endometrial stromal sarcomas 7 and mixed mesodermal sarcomas 2. The mean age of the patients was 56. The cases were classified according to the FIGO staging system. 5 patients had previously undergone (between 1 and 8 years) a subtotal hysterectomy for uterine leiomyoma. 5 patients had stage 1 sarcoma, 13 patients stage III and 2 patients stage IV. 13 patients are dead, 7 are alive without evidence of disease, 2 are alive with evidence of disease. The 5 year actuarial survival rate was 30%. Tumor extent at diagnosis was not correlated with survival. Analysis of failures revealed : 4 metastatic, 5 metastatic and pelvic and 6 isolated pelvic failures. Prognosis was not significantly different between leiomyosarcomas and endometrial stromal sarcomas. There was no pelvic failure in patients with stage I sarcoma (treated by surgery plus radiotherapy). Survival was not found to be correlated with histopathological grade as described in soft tissue sarcomas. Nevertheless a correlation (non significant) was found between survival and mitosis count. Chemotherapy (Cyvadic) was administered in 8 advanced tumors with partial response (always below 50% of the initial tumor) in 5 patients.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1993

Different types and different prognosis-study of 310 uterine sarcomas.

Three hundred and ten cases of uterine sarcomas, among them, according to pathology: LMS (Leiomyosarcoma)--117, MMS (Mixed mesodermal sarcoma)--62, ESS (Endometrial stromal sarcoma)--56, CS (Carcinosarcoma)--27, RBMS (Rhabdomyosarcoma)--18 and other sarcomas--30, were retrospectively evaluated at the Maria Skłodowska-Curie Memorial Cancer Center between 1950 and 1985. These mesodermal tumors were divided into uterine corpus sarcomas (295) and the rarely occurring uterine cervix sarcomas. As the main modality of treatment 307 patients underwent surgery. Almost all patients received adjuvant radiotherapy but 51 cases of LMS. Overall 5-year survival was 34.5% and in particular subgroups the survivals were as follows: the best, 52% in LMS; lower, 30% in ESS; poor survival about 20% in CS and MMS. In spite of low curability in the majority of subgroups except LMS, the cases of CS with primary site in uterine cervix distinguished itself with better results. No significant survival advantage was found for surgery plus radiotherapy in LMS with low grade mitotic activity, (10 mitoses/10 HPF) compared with surgery alone (63% and 54%). These cases seem to have better prognosis in LMS with high grade mitotic activity (10 mitoses/10 HPF) and do not require adjuvant radiotherapy.

Adult↗
PubMed1977

Mixed mesodermal sarcoma of the ovary.

Three cases of mixed mesodermal sarcoma (MMS) are reported and a review of 90 cases from the English literature is presented. All cases were analyzed with regard to clinical features, prognosis, and therapeutic efficacy. The histogenesis of these rare tumors is discussed and a new classification of ovarian sarcomas is proposed. MMS usually afflicts the postmenopausal women and occurs more often in the nulliparous female. The disease is usually advanced (Stage III) when diagnosed and thus provides a difficult challenge to any postoperative therapy.

Adolescent↗
PubMed1975

[A heterologous mesodermal tumor of the uterus of the pleomorphic rhabdomyosarcoma type].

In the available literature mesodermal tumours of the uterus are described under various names (botryoid sarcoma, carcinocarcoma, chondrosarcoma, rabdomyosarcoma, etc.). For the sake of unification of terminology of these tumours, Kehrer suggested the term "mesodermal mixed tumours", thereby reflecting their origin from cellular elements of the mesoderma. Histologically, benign and malignant variants of tumours are identified; these tumours are characterized by multi-component composition of tissues forming integral parts thereof, the latter may be homologous or heterologous. Clinically, the said tumours are diagnosed in rare cases. Diagnosis is made on the basis of a microscopical investigation of a scrape from the uterine cavity, or investigation of the operation material. Heterologic tumours of the uterus of the type of rabdomyosarcoma have been described by a number of authors. As to their microscopical structure, they may be divided into embryonal, alveolar and pleomorphous. A case of heterologic mesodermal tumour, verified as a pleomorphous rabdomyosarcoma, which could develop in a long existed mesodermal tumour of the uterus in a woman 67 years of age, is described.

Aged↗
PubMed1995

Müllerian adenosarcoma of the cervix: differential diagnosis, histogenesis and review of the literature.

A new case is reported of müllerian adenosarcoma, presenting as a 'benign cervical polyp' protruding through the vulva of a 44 year-old woman admitted with abnormal vaginal bleeding. This report emphasizes the importance of a careful examination of the stroma and special features of the entrapped glands in order to contribute to an earlier and proper diagnosis. The literature is reviewed and the probable histogenesis of this tumor and differential diagnosis with embryonal rhabdomysarcoma (sarcoma botryoides), adenofibroma, malignant mesodermal tumor and carcinosarcoma is discussed.

Adenosarcoma↗
PubMed1987

Metameric capillary hemangioma producing complete myelographic block in an infant. Case report.

A variety of lesions of ectodermal, mesodermal, (rarely) endodermal, or mixed-cell layer origin involve the region of the conus medullaris. Some of these abnormalities produce cord tethering and others, such as lipoma, frequently present as masses. In the present case, an infant was discovered to have a lesion most closely resembling a capillary hemangioma involving the skin of the midline and right buttock, the deep soft tissues of the right buttock, the dura, and the conus medullaris. The origin of this congenital spinal tumor from primitive mesoderm is proposed in light of its metameric pattern of distribution.

Aortography↗
PubMed1984

A clinical and pathologic study of 30 cases of malignant mixed mullerian epithelial and mesenchymal ovarian tumors: a Gynecologic Oncology Group study.

Between November 1971 and June 1980, thirty patients with primary malignant mixed mullerian tumors of the ovary were entered into a Gynecologic Oncology Group registry and treatment protocol. The mean age of the patients is 60.4 years. Six were Stage II, twenty-three were Stage III, and one patient had Stage IV disease. Among the 30 cases, 15 were designated carcinosarcomas (CS) and 15 were mixed mesodermal sarcomas (MMS). Twenty-three of the thirty study patients died from 1 to 16 months following their initial surgery. Two of the seven living patients have persistent cancer at 11 and 54 months. Four patients were alive and well at 6, 11, 22, and 32 months at the close of the study. Of the 12 patients receiving vincristine, dactinomycin, and cyclophosphamide (VAC) with (8 cases) or without (4 cases) radiation therapy (RT), there were two clinical complete responses; both died with disease at 6 and 16 months. One of the six patients receiving adriamycin had a complete response (CR). She is alive with disease at 11 months. There was no apparent difference in the survival or stage distribution among the cases with CS and MMS. Survival was somewhat better for the patients with earlier stage disease or smaller residual tumor burden.

Adenocarcinoma↗
PubMed1996

[Müllerian mixed tumors. Experience of the Rennes Regional University Hospital].

Seventeen cases of mixed Muller tumours (tumours of the uterus with malignant epithelial and mesenchymal components) are presented. These recently described tumours are rare and occur in menopaused women. Bloody discharge is the usual clinical manifestation, together with an increase of the volume of the uterus. Pathology examination of the surgical specimen is required for diagnosis using immunolabeling to distinguish between homologous tumours (the sarcomatous component occurs in the primary mesenchyma) and heterologous tumours (the mesenchymatous component results from a metastasis). Prognosis, usually poor, depends on the stage of the tumour. Overall survival at 5 years is about 30%. Survival in early stage I and state II tumours is no greater than 50% at 5 years. Treatment is based on radiosurgical techniques in less advanced tumours and requires radiochemotherapy in more advanced stage tumours. Recurrence is usually seen within 2 years, involving the pelvis alone in 10% of the cases and metastasis in most of the others. Because of their rapid development and poor prognosis, these tumours should be identified separately as a separate entity within a larger group of mixed mesodermic tumours.

Aged↗
PubMed1992

The role of ifosfamide in gynecologic cancer.

Ifosfamide exhibits promising activity in a variety of gynecologic neoplasms. In carcinoma of the ovary, the drug shows clear-cut activity, effecting responses in patients who have proven clinically resistant to cisplatin-based combination chemotherapy. In carcinoma of the cervix, the drug also appears to be highly active, both as a single agent and in combination with other agents, such as the platinum compounds. The drug is active in uterine sarcomas and appears to be the most active agent studied to date in mixed mesodermal sarcomas of the uterus. The drug's role in combination with other agents in the treatment of all of these neoplasms is under study, but cannot be determined from currently available data on combination therapy. Its activity in other gynecologic tumors, such as endometrial carcinoma and ovarian germ cell tumors, is also indeterminate at present. Although further studies are needed, current evidence supports the contention that ifosfamide is a major new active drug in the management of gynecologic cancers.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1978

Peritoneal mesothelioma in a male pseudohermaphrodite with asymmetrical gonadal differentiation.

A diffuse peritoneal mesothelioma occurring in a patient with male pseudohermaphroditism and asymmetrical gonadal differentiation (mixed gonadal dysgenesis) is described. Malignancies of mesodermal origin, usually derivatives of the urogenital ridge, appear to occur with increased frequency in male pseudohermaphrodites. This appears to be the first reported instance of a mesothelioma in a male pseudohermaphrodite with mixed gonadal dysgenesis. The clinical and pathologic features of this tumor, including the electron microscopic findings, are presented.

Disorders of Sex Development↗