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Neuroectodermal immunophenotype in uterine malignant mullerian tumors (MMT): comparative immunohistochemical analysis with embryonal uterine development.

Multidirectional differentiation of neoplastic cells in uterine MMT is still a subject of controversy. The present study was designed to assess the immunophenotype of 15 uterine MMT paying special attention to the markers of neural (neuroendocrine) differentiation. In addition, the same immunohistochemical study was performed on 20 human fetal specimens in order to establish possible relationships between the immunophenotype of MMT and the expression of the corresponding antigens in the fetal tissues of the embryonal female genital tract. Besides the typical immunohistochemical patterns in three cases the epithelial component showed simultaneous coexpression of vimentin and desmin. EMA and cytokeratin, whereas epithelial markers were coexpressed with vimentin in the sarcomatous component of one adenosarcoma. Moreover, both components were immunoreactive to the markers of neural differentiation (PGP 9.5, GFAP, HNK-1, N-CAM, HBA71). This aberrant expression was not correlated with morphological signs of neural differentiation at either light microscopy or ultrastructural levels. Regarding the analysis of fetal tissues, both epithelial and mesenchymal elements in the fetal genital tract expressed the above-mentioned neural markers at different dates of gestation. The intensity of this expression diminishes as the fetus matures and at the end of antenatal life the immunophenotype characteristic for adult life is established. Taking into consideration the capacity of uterine tissue to reproduce embryonal phenotype during neoplastic transformation, we studied this abnormal immunoprofile and its hypothetic value for the diagnosis and prognosis of MMT.

Aged↗

Pathology of the uterine body.

During the past year, evidence has compiled to suggest that uterine carcinosarcomas are, in fact, metaplastic carcinomas and that mitotic counts are of no value in distinguishing between high- and low-grade endometrial stromal sarcomas in stage I cases. The pathologic and biologic behaviour of uterine müllerian adenosarcomas has been comprehensively reviewed. Prognostic factors have been identified in a large series of surgically staged endometrioid adenocarcinomas of the endometrium, while the sinister nature of the serous papillary and clear cell carcinomas of the endometrium have been confirmed. It has been claimed that a papillary pattern of growth is an independent prognostic factor in endometrial neoplasia and shown that the accuracy of macroscopic estimation of depth of myometrial invasion decreases with increasing tumor grade. The value of peritoneal cytologic examination in early stage endometrial adenocarcinoma has been questioned, as has the value of DNA flow cytometry in endometrial neoplasia. The prognostic significance of steroid receptor status has been confirmed and studies of oncogene amplification are beginning to yield prognostically useful information. Evidence is also beginning to emerge concerning chromosomal abnormalities in many women with endometrial adenocarcinoma.

Adenocarcinoma↗

Extrauterine mixed mesodermal tumors. An immunohistochemical study.

Mixed mesodermal tumors are uncommon outside the uterus. Nine extrauterine mixed mesodermal tumors (eight ovarian and one extragenital) were selected for histochemical and immunoperoxidase study. In eight cases, both epithelial and mesenchymal elements were malignant (chondroid in six, rhabdomyoid in four, and osteoid in two). One ovarian tumor was an adenosarcoma. All cases were stained with periodic acid-Schiff with and without diastase and for alpha 1-antitrypsin, myoglobin, keratin, vimentin, muscle-specific actin, and alpha 1-antichymotrypsin, by using the avidin-biotin-immunoperoxidase method. The periodic acid-Schiff-positive, diastase-resistant droplets in several of the tumors showed peripheral alpha 1-antitrypsin positivity. Keratin delineated epithelial areas well in seven cases, and rhabdomyoid differentiation was confirmed with myoglobin in four cases. However, squamous elements in one tumor were falsely positive for myoglobin. We concluded that despite occasional cross-reactivity, carefully interpreted immunoperoxidase stains can be useful in distinguishing epithelial and mesenchymal elements in these tumors.

Female↗

[Müllerian papillary adeno-fibromata and müllerian adenosarcomata. Mülleromas and mixed malignant müllerian growths contingent with benign epithelium (author's transl)].

The authors present 6 cases of müllerian papillary adeno-fibromata (or mixed mesodermal müllerian benign tumours) of which 4 are anatomo-clinical presentations and 2 are histological presentations, as well as 3 cases of müllerian adenosarcoma (mixed müllerian mesodermal tumour with stroma only sarcomatous). The study of these cases added on to those found in the literature leads to the following conclusions: The first group can be accepted. The second group will be of mild malignancy. All the same, 4 of which the sarcomatous nature is not in doubt at all (malignant from the start) recurred rapidly. Those in which the sarcomatous nature was attenuated or doubtful (15 cases) seemed to have a much longer evolution with some local recurrences. Only 3 patients have been followed up for more than 5 years. It is absolutely essential to carry out further observations before confirming for certain the prognosis of this new type of tumour.

Aged↗

[Primary study of histogenesis and diagnosis of synoviosarcoma].

Fourteen cases of synoviosarcoma were investigated by electron microscopy and immunohistochemical technique (PAP) using keratin and EMA as epithelial markers, vimentin as a mesenchymal marker. Of the 14 cases 9 were biphasic (BS) and 5 were monophasic synoviosarcoma (MS). In BS, epithelial-like cells, glandular, tubular of slit-like structures, junctional complex, microvilli at the luminar surface and basal lamellae at bottom could be easily seen. Spindle cells both in BS and MS were similar. These cells had somewhat similarity with epithelia-like cells, such as slender cell processes or microvilli, the external lamellae and cell junctions. The spindle cells also formed some slit-like structures. Collagenization was mild in matrix. Whether in BS or MS, epithelial or spindle cells, there were positive reactions for keratin, EMA and vimentin. The present study suggests that synoviosarcoma is neither a tumor of synovium origin nor a sarcoma of synovial differentiation, but a carcinosarcoma or adenosarcoma of soft tissues; the monophasic type is not a variant of fibrosarcoma either. It really is a distinct variant of synoviosarcoma. The expressions of both epithelial and mesenchymal features are useful criteria for diagnosis of synoviosarcoma.

Humans↗

[Sarcoma of the uterus. A clinical study apropos of 50 surgically treated cases at the Gustave Roussy Institute. Review of the literature].

From 1970 to 1987 fifty patients with uterine sarcoma who have been operated at the G. Roussy Institute (IGR) were studied. The histological material was reviewed according to the W.H.O. classification and the Hendrickson and Kempson's criteria. Staging was done on surgical and histological findings according to the pTNM classification of the UICC for endometrial carcinoma. There were 22 cases of heterologous malignant mixed müllerian tumor (MMT), 5 cases of homologous MMT, 20 cases of leiomyosarcoma (LS), 1 high grade stromal sarcoma, 1 low grade stromal sarcoma (stromal myosis (SM], 1 adenosarcoma. There were 22 cases of stage T1 T2, 23 cases of stage T3 T4 and 5 TX (first surgery outside IGR). Total hysterectomy with bilateral salpingo-oophorectomy was performed in 86% of the cases. Radiation therapy was performed in 26 cases mostly external pelvic irradiation associated with endobrachytherapy. "Cyvadic" combination chemotherapy was used in 16 cases associated with platinum in few cases. There were 2 postoperative deaths, 11 cases of progression and 37 cases of complete remission (CR). Of the 37 cases of CR, 10 patients are alive with NED 6 months to 10 years after diagnosis in 2 cases of MMT. 27 patients presented recurrence or metastasis. Of the 22 patients with pelvi-abdominal recurrence, 10 had debulking surgery (one total pelvic exenteration (PE), three posterior PE). Among them five are alive 14 to 78 months later (3 LS, 1 AS, 1 SM). Thirty percent of the patients developed lung metastasis. The overall five-year survival was 42 +/- 16% (28 patients) (Kaplan-Meier's method), similar to literature data.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Uterine tumors with mixed epithelial and mesenchymal elements.

The clinical and pathologic features and the differential diagnosis of uterine tumors containing mixed epithelial and mesenchymal elements are reviewed. The malignant mullerian mixed tumor (MMMT) is the most common neoplasm in this group, which also includes the mullerian adenofibroma and adenosarcoma, endometrial stromal neoplasms with focal epithelial differentiation, uterine tumors resembling ovarian sex cord tumors, and adenomyomas, including the atypical polypoid adenomyoma.

Adenofibroma↗

[Endometrial stromal sarcoma of vaginal and para-vaginal localization. Morphology, diagnosis and differential diagnosis].

Endometrial stromal sarcomas constitute a subgroup of malignant mesenchymal uterine neoplasms and comprise biologically somewhat different variants. Their occurrence in extrauterine tissues is possible but extremely rare and therefore they are quite often mistaken for other tumours. Reported in this paper is a stromal sarcoma in vaginal and paravaginal localization (64-years old woman). It was the purpose of our study to present the histological structure in comparison to uterine neoplasms and to discuss differential diagnosis. It could be demonstrated that histological features of the actual case corresponded precisely to the respective uterine tumours originating within the myometrium. In extrauterine position, haemangiopericytomas, leiomyosarcoma, malignant schwannomas, fibrosarcomas and synovial sarcomas as well as Müllerian adenosarcomas and carcinosarcomas should be taken into account. Important histological features facilitating distinction of endometrial stromal sarcomas from the aforementioned malignant tumours are discussed, and some comments are added with regard to histogenetical interpretation.

Diagnosis, Differential↗

Teratogenic and carcinogenic effects of some chemicals during perinatal life in rats, Syrian golden hamsters, and minipigs.

Teratogenic effects of ENU have been observed in the rat, Syrian golden hamster, and minipig. In BD and Wistar rats, as well as in hamsters, ENU is a potent carcinogen when administered prenatally. Other members of the homologous series of alkylnitrosoureas, except n-propylnitrosourea, have been shown to be less active or totally inactive as carcinogens in experiments on prenatal animals. Simultaneous oral administration of L-citrulline and sodium nitrite induced adenosarcomas of the kidney (Wilm's tumors) in 6 of 22 offspring. The importance of prophylactic measures in man during prenatal development is emphasized.

Animals↗

[Mesodermal (Muller) mixed ovarian tumor].

A case of mesodermal (Müller) mixed tumor of the ovary is described. The tumor consisted of the endometrial component with benign epithelial structures, stromal sarcoma, rhabdomyosarcoma, and elements of undifferentiated sarcoma. By its histological structure the tumor may be classified as one of the variants of mesodermal (Müller) mixed tumor of the heterologous adenosarcoma type.

Adult↗

Disseminated adenomyomas of the abdominal and pelvic cavities: a case report.

A case report of disseminated adenomyomas of the abdominal and pelvic cavities is presented. Although the pathogenesis of these benign tumors is still unresolved, the authors postulate that they develop from the differentiation into smooth muscle of a multi-potential cell that arises from endometrial stromal cells in an area of preexisting endometriosis. Based on the authors' experience, when multiple leiomyomas are found in the peritoneal cavity, it is wise to make serial sections to determine whether endometrial tissue is present. The clinician must be alert to the fact that on gross examination adenomyomas can mimic metastatic carcinoma. Differentiation is made only through histologic examination. In addition, since adenosarcoma arising from adenomyoma is a documented occurrence, careful examination of histologic sections of adenomyomas is essential to avoid making the wrong diagnosis.

Abdominal Neoplasms↗

[Granulomatous hepatitis: aetiological study of 107 cases (author's transl)].

In this series, the commonest aetiology was tuberculosis (30 cases, 28%), followed by sarcoidosis (18 cases, 17,7%), mediterranean fever (Olmer's disease) (13 cases, 12,1%), brucellosis (8 cases, 7,4%), typhoid fever (7 cases, 6,6%) and idiopathic forms (8 cases, 7,4%). These were followed by Hodgkin's disease, toxoplasmosis, adenosarcoma, and leprosy. Finally, there were single cases due to infectious mononucleosis, B.C.G. reaction, hypogammaglobulinaemia, coeliac disease and temporal arteritis. Half of the patients had hepatomegaly and an increase, in general moderate, in hepatic enzymes (transaminases, alkaline phosphatase). The highest enzyme levels were seen in cases of brucellosis, hepatic enzymes being normal in patients with sarcoidosis.

Alanine Transaminase↗

Malignant lesions of the female genital tract and peritoneum that may be underdiagnosed.

Female genital tract neoplasms that may have a deceptively benign appearance and selected frankly malignant neoplasms that may be misinterpreted as less aggressive lesions are reviewed. In the uterine cervix, the two major neoplasms in this group are the minimal deviation adenocarcinomas of mucinous and endometrioid types. The latter subtype has only recently been described. Endometrioid adenocarcinomas, usually of the uterine corpus, but occasionally of other sites, may have microglandular patterns that can lead to their misdiagnosis, sometimes as microglandular hyperplasia. Pure squamous cell carcinomas of the uterine corpus frequently are composed of very well differentiated epithelium, so that it is possible to misinterpret them as nonneoplastic, and a similar phenomenon may occur in association with the squamous element in some adenocarcinomas with squamous differentiation. Other uterine entities that may be underdiagnosed are malignant lymphoma of the cervix, placental site trophoblastic tumor, myxoid leiomyosarcoma, endometrial stromal sarcoma with glandular differentiation, and mullerian adenosarcoma. Consideration of a variety of architectural and cytological features should facilitate their interpretation. One recently described variant of adenocarcinoma of the fallopian tube that may be confused with the usually clinically benign female adnexal tumors of probable Wolffian origin is microfollicular endometrioid adenocarcinoma. Ovarian tumors subject to misinterpretation that are reviewed herein include metastatic tumors with deceptively benign foci, endometrioid adenocarcinomas that may be misdiagnosed as sex cord tumors, and cystic granulosa cell tumors that may be misinterpreted as follicle cysts. Finally, rare variants of malignant mesothelioma that may be underdiagnosed are reviewed.

Adenocarcinoma↗

Non-germ-cell or teratomatous malignant tumors showing additional rhabdomyoblastic differentiation, with emphasis on the malignant Triton tumor.

Non-germ-cell or teratomatous malignant tumors showing additional rhabdomyoblastic differentiation can be divided into three groups. Group I consists of tumors with only sarcomatous differentiation. Included in this group are malignant mesenchymoma, dedifferentiated chondrosarcoma, and the dedifferentiated retroperitoneal liposarcoma. Epithelial or Sertoli-Leydig cell elements represent the second component of tumors in group II. The best known tumor in this group is the malignant mullerian mixed tumor, but other well described tumors with rhabdomyosarcomatous differentiation are the carcinosarcoma, mullerian adenosarcoma, Sertoli-Leydig cell tumor, mammary cystosarcoma, and blastomas. Most of the tumors in groups I and II are derived from mesenchymal tissue. A neuroectodermal origin is shared by all tumors in group III. This includes the medulloblastoma, retinoblastoma and, most frequently, the malignant Triton tumor. The tumors in all three groups are clinically malignant to a varying degree.

Humans↗

[Botryoid rhabdomyosarcoma of the cervix. Clinico-pathologic study of a case].

The botryoid rhabdomyosarcoma of the cervix is a rare tumour occurring in young woman or during genital activity. It mainly causes vaginal bleeding or appears as a polypoid grape-like mass with a gelatinous cut-surface. The diagnosis is based on the presence of a submucosal cambium layer and a rhabdomyoblastic differentiation, corresponding to an intracytoplasmic double cross-striation. Metaplastic cartilaginous islands are sometimes observed. The immunostaining ensures the muscular origin of the tumour, characterized by the expression of actin and desmin. Apart from mullerian adenosarcomas, the main differential diagnosis is represented by the benign polypoid formations of the cervix: the genital rhabdomyoma and the fibroblastic lesion, called fibro-epithelial polyp with atypical stroma. The treatment actually includes both chemotherapy and surgery which is often limited to a conization.

Adolescent↗

[Angiosarcoma of the heart and its spontaneous rupture. A rare cause of effusive-constrictive pericarditis. A case report and review of the literature].

The authors present the clinical history of a male 44 year old patient who was hospitalized with the diagnosis of pericardial constriction and effusion and operated on as an emergency because of spontaneous cardiac rupture and was found to have a cardiac adenosarcoma. They review the literature in order to discuss a very rare cause of hemopericardium and constrictive pericarditis with epidemiologic, diagnostic, therapeutic and prognostic commentaries and secondly, to try to establish if this type case does not represent a diagnostic problem for the ecocardiographer since an angiosarcoma that occupies the pericardial space can be confused with a hemopericardium. They also mention other imaging studies that are used to better characterize and diagnose these tumors.

Adult↗

Epidermoid carcinoma complicating chronic osteomyelitis of the femur.

A case of epidermoid carcinoma of the femur that arose in an area affected with chronic osteomyelitis for over 40 years is reported. The incidence of this rare complication has been estimated at 0.23% to 1.6% of all cases of chronic osteomyelitis. Although cases of sarcoma, adenosarcoma, plasmocytoma, and malignant fibrous histiocytoma have been reported, epidermoid carcinoma is the most common cancer type. This tumor develops after 20 to 40 years of chronic infection with a discharging fistula. Epithelialization of the fistulous tract has been demonstrated. The cancer arises in the metaplastic epithelium and can secondarily spread to the bone. Despite the low grade of malignancy of many of these epidermoid carcinomas, pulmonary metastases are not rare. Amputation meeting criteria for oncologic surgery is the only means of ensuring a good prognosis.

Aged↗

Endometrial pathology.

The current literature on endometrial neoplasia deals with proliferative lesions such as endometrial proliferation in early pregnancy, atypical polypoid adenomyofibromata and their possible relationship to carcinoma, and histological grading of endometrial cancer with emphasis on nuclear grading and subtypes of endometrial cancer including the newly described intestinal and hepatoid types. Recent publications dealing with p53 protein, genetic studies, and nucleolar organizer regions are of interest but have provided no striking new insight into endometrial neoplasia. Angiogenesis has appeared for the first time in the literature in connection with endometrial neoplasia. Tamoxifen continues to occupy an important place in the literature, and endometrial adenosarcoma has been identified recently as one of the myriad of lesions which patients treated with tamoxifen are prone to develop.

Antineoplastic Agents, Phytogenic↗