Problematic uterine smooth muscle neoplasms.
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Female↗
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Smooth muscle neoplasms of the stomach.
We reviewed the records of 31 patients with smooth muscle tumors of the stomach seen at the First Surgical Department, Medical School, University of Athens, Greece, between the years 1961 and 1981 with special emphasis on the clinical data, diagnosis, and pathology. The majority of patients were symptomatic and were preoperatively diagnosed by radiology and/or endoscopy, but accurate histologic diagnosis was obtained in only three cases. The tumors varied in size, were relatively equally distributed throughout the stomach, and their management required 35 operations, consisting of 18 Billroth II gastrectomies, 15 local excisions, and two total gastrectomies. Histologically, the tumors proved to be leiomyoma in 23 cases, leiomyosarcoma in ten, and leiomyoblastoma in two. The difficulty of histologic classification in the absence of metastasis is clearly shown by the fact that three tumors recurred and were subsequently characterized as leiomyosarcoma one to three years after they were initially classified as leiomyoma.
Adult↗
Creatine kinase isoenzyme patterns in normal smooth muscle and smooth muscle neoplasms.
The CK isoenzyme composition of leiomyoma tissue is predominantly CK-BB and similar to adjacent myometrium tissue, while the leiomyosarcoma revealed a lesser quantity of CK-BB, but a greater quantity of CK-MM. The reasons for the discrepancy between the two types of neoplasms is not clear, but may reflect the changes which occur when smooth muscle becomes malignant.
Ankle↗
[Comments on a better understanding of smooth muscle neoplasms in the uterus].
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Diagnosis, Differential↗
Myocardial Epstein-Barr virus-associated cardiac smooth-muscle neoplasm arising in a cardiac transplant recipient.
Epstein-Barr virus-associated smooth muscle proliferations have been reported in immunosuppressed patients with acquired immunodeficiency syndrome and after organ transplantation. We report here a case of a histologically benign case arising in a 48-year-old male who had received immunosuppressive therapy 4 years earlier, after cardiac transplantation. In the necropsy performed for unrelated reasons, an incidental left intramyocardial tumor was discovered. The presence of Epstein-Barr virus was confirmed by EBER-1 in situ hybridization and polymerase chain reaction. To the best of our knowledge, this is the first case of an Epstein-Barr virus-associated smooth muscle proliferation arising in the heart after cardiac transplantation and should be added to the potential complications of this kind of procedure.
Aortic Aneurysm, Abdominal↗
Smooth muscle neoplasms of the uterus.
Recent investigations, using DNA technology, of the molecular biology of smooth muscle tumours of the uterus have confirmed their monoclonality and have strengthened the view that oestrogen and oestrogen receptors play a major role in the pathogenesis of fibromyomata. In addition, increasing evidence suggests that progesterone, insulin-like growth factors, epidermal growth factors and other proteins are also involved. The mechanisms whereby gonadotrophin-releasing hormone agonists cause shrinkage of fibromyomata remain controversial but both vascular changes and cellular atrophy appear to play a role. A shift of emphasis in the study of fibromyomata has resulted from the demonstration that the myometrium adjacent to fibromyomata is not normal and shows some similarities to the tumours themselves.
Cytogenetics↗
Ophthalmic striated muscle neoplasms.
Rhabdomyosarcoma, the most common primary malignant childhood orbital tumor, is composed of neoplastic striated muscle cells (rhabdomyoblasts) in various stages of differentiation and in patterns suggestive of neoplastic analogs of normal muscle embryogenesis. Orbital rhabdomyosarcoma is most commonly seen in children and adolescents, the average age of onset of symptoms being 7.8 years. The tumor usually presents as a rapidly evolving exophthalmos, often associated with drooping of the upper eyelid. A mass is palapable in only 25% of cases, loss of central vision at the time of presentation is uncommon, and laboratory studies are often of little help in diagnosis. The best diagnostic aid is a high index of suspicion whenever one sees a rapidly progressive exophthalamos in a child. Orbital rhabdomyosarcoma is almost always of the embryonal type, believed to originate in the orbital soft tissues from undifferentiated pluripotential embryonic mesenchyme. In the past, orbital exenteration has been the primary therapy. Review of 162 literature cases of orbital rhabdomyosarcoma, generally treated by unassisted surgery, revealed that only 25% of the patients survived 3 or more years. Recently, it has been shown that radiation therapy, alone or combined with chemotherapy, can be successful. A multidisciplinary approach, utilizing surgery, radiation therapy and chemotherapy has also been advocated. Both approaches appear to offer greater survival than unassisted orbital exenteration. The possibility of primary radiation therapy is extremely promising; if it becomes increasingly effective, a mutilating surgical procedure may become obsolete.
Adolescent↗
AMP-deaminase from human uterine muscle neoplasm (leiomyoma).
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AMP Deaminase↗
Pulmonary metastases (with admixed epithelial elements) from smooth muscle neoplasms. Report of nine cases, including three males.
This study pertains to an entity characterized by the presence of multiple intrapulmonary nodules, which consist of an admixture of bundles of well-differentiated smooth muscle cells and epithelial-lined spaces. These lesions have been frequently interpreted as a variant of hamartomas. However, in this review of the literature, and careful analysis of nine cases of this entity, we concluded that they should be considered metastases from smooth muscle tumors which incorporate some structures of mature lung parenchyma as they slowly expand. We affirm that the designation "fibroeliomyomatous hamartoma" should be discarded. Our cases occurred in six female and three male patients. In all but one female the primary source for lung metastases was uterus, while the male patients had primary lesions in the saphenous vein, diaphragm, and soft tissues. These lung lesions increase in size and number and are potentially fatal, though this may take many years. Even though the smooth muscle cells of the lung nodules appear bland on light microscopy, we were always able to demonstrate mitotic activity; electron microscopy indicated immaturity of the cells. For these reasons, we believe the tumors to represent metastatic leiomyosarcomas.
Adult↗
Smooth muscle neoplasms of the urinary bladder: a clinicopathologic comparison of leiomyoma and leiomyosarcoma.
We report the clinicopathologic, immunohistochemical, and DNA ploidy findings of 18 leiomyosarcomas of the urinary bladder. In addition, we compare these malignant smooth muscle tumors with 10 cases of urinary bladder leiomyoma. The 14 male and four female patients with leiomyosarcoma ranged in age from 25 to 88 years (mean 64 years). The tumors ranged from 3.0 to 15.0 cm (mean 7.1 cm) in greatest dimension and were moderately to highly cellular, consisting of interlacing fascicles of spindled cells with mild to marked nuclear atypia. Coagulative tumor necrosis was identified in 14 cases (78%), and mitotic activity ranged from 1 to 42 mitotic figures (MF) per 10 high power fields (HPF) (mean 12 MF/10 HPF). Tumors were classified as either high-grade (12 cases) or low-grade (six cases) based on nuclear atypia, mitotic activity, and tumor necrosis. Actin positivity was present in 15 tumors (83%), and desmin immunoreactivity was present in seven tumors (39%). All cases were negative for epithelial markers and S-100. Proliferative activity, as assessed by MIB-1 staining, ranged from 0.1% to 51.4% (median 9.1%). Seven (39%) of the leiomyosarcomas were DNA aneuploid, eight (44%) were tetraploid, and three (17%) were diploid. Five patients underwent radical cystoprostatectomy, one radical cystectomy, seven had partial cystectomy, two underwent pelvic exenteration, and three patients had transurethral resection only. Follow-up information was available on all 18 cases and ranged from 2 to 68 months (mean 22 months). Of the 12 patients with high-grade tumors, six (50%) died of disease from 2 to 20 months (mean 7 months) after diagnosis and three patients (25%) are alive with metastatic tumor. Two of the six patients with low-grade leiomyosarcoma died of tumor, 61 and 68 months after diagnosis. There were five male and five female patients with leiomyoma ranging in age from 22 to 78 years (mean 61 years). The tumors ranged from 0.5 to 4.5 cm (mean 1.6 cm) in greatest dimension, were well circumscribed, and had low cellularity. Mitotic activity, necrosis, and cellular atypia were absent, and the tumors were strongly positive for both actin and desmin. MIB-1 staining ranged from 0% to 3.8% (median 0.8%). Seven (87.5%) of the leiomyomas were DNA diploid or near-diploid and one (12.5%) was DNA aneuploid. Six patients were treated with transurethral resection and four with partial cystectomy. All 10 patients were alive at the last follow-up (mean follow-up 75 months), and no tumor recurred or metastasized. Our study shows that low-grade leiomyosarcomas are capable of malignant behavior, and high-grade leiomyosarcomas appear to behave more aggressively than low-grade tumors. In addition, the diagnosis of urinary bladder leiomyoma should be reserved for noninfiltrative smooth muscle tumors lacking mitotic activity, cytologic atypia, and necrosis.
Actins↗
The values of intratumoral mast cell count and Ki-67 immunoreactivity index in differential diagnosis of uterine smooth muscle neoplasms.
In this study, the role of the count of intratumoral mast cells was examined and compared with the proliferative activity exhibited by Ki-67 indices in the differential diagnosis of uterine smooth muscle tumors. Sixteen cases of leiomyosarcoma, nine cases of atypical leiomyoma and 16 cases of ordinary leiomyoma were included. The pathological features of the cases were determined by reviewing the archive materials including the patient records and hematoxylin-eosin-stained sections. Toluidine blue stain was used to highlight the intratumoral mast cells and they were counted in at least 40 high power fields. A standard streptavidin-biotin method was applied to the sections to highlight the Ki-67 immunoreactive tumor cell nuclei. These proliferative cells were counted in at least 10 high-power fields. Atypical leiomyomas tended to have a higher quantity of intratumoral mast cells than leiomyosarcomas and ordinary leiomyomas (P = 0.027 and P = 0.021, respectively). Leiomyosarcomas tended to have higher Ki-67 immunoreactivity rates than atypical leiomyomas, although the difference was not statistically significant (P = 0.82). We concluded that the quantity of intratumoral mast cells is useful in the differential diagnosis between leiomyosarcomas and atypical leiomyomas, while the cell proliferation rate expressed by Ki-67 immunoreactivity has a limited value.
Adult↗
Smooth muscle neoplasms of the uterus other than ordinary leiomyoma. A study of 46 cases, with emphasis on diagnostic criteria and prognostic factors.
Thirty-seven cases of uterine leiomyosarcoma are presented, along with nine cases of leiomyoma variants from which they were distinguished. All patients were followed for a minimum of 10 years. In cases with nuclear pleomorphism, leiomyosarcoma was diagnosed when there were five or more mitotic figures in ten consecutive high-power (X400) fields in the most active area of the tumor, and also when there were fewer mitotic figures but extensive tumor necrosis (there was only one leiomyosarcoma without nuclear pleomorphism, and it had more than 20 mitotic figures in ten high-power fields). Tumor size was the major prognostic factor in the leiomyosarcoma group; five of eight patients with neoplasms measuring less than 5 cm in maximum dimension survived, whereas no patient with a larger tumor did so. Other pathologic and clinical variables, including mitotic rate, tumor necrosis, degree of nuclear pleomorphism, vascular invasion, and patient age, had no significant relationship to survival or tumor behavior in leiomyosarcoma when tumor size was taken into account. The nine cases of leiomyoma variants consisted of three atypical leiomyomas (which had nuclear pleomorphism, one or no mitotic figures per ten high-power fields, and no necrosis), two plexiform leiomyomas, two cases of peritoneal leiomyomatosis, one palisaded leiomyoma, and one case of intravenous leiomyomatosis; all of these patients were tumor-free on follow-up.
Adult↗
Epstein-Barr virus-associated hepatic smooth muscle neoplasm in a cardiac transplant recipient.
Host immunosuppression is increasingly recognized as a significant risk factor for the development of a primary neoplasm. Chronic immunosuppressive therapy, as used in organ transplantation, may perturb the immunosurveillance ability of the host, making the patient more susceptible to virus-associated malignancies. We have taken care of a care of a child who received an orthotopic heart transplant and who then developed both a generalized lymphoproliferative disorder and a leiomyoma of the liver a year later. Epstein-Barr virus DNA was detected in a lymph node initially and the hepatic tumor cells subsequently. The former responded to a reduction in the immunosuppressive medications and the latter responded to surgical resection. This is the first report of a hepatic smooth cell neoplasm occurring following cardiac transplant and the development of two sequential Epstein-Barr virus-associated disorders in an immunosuppressed patient.
Child↗
[Uterine smooth muscle neoplasms: a diagnostic dilemma].
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Female↗
An immunohistochemical study of normal endometrial stroma and endometrial stromal neoplasms. Evidence for smooth muscle differentiation.
To determine the immunohistochemical staining profile of endometrial stromal cells, we analyzed a series of formalin-fixed, paraffin-embedded normal endometrial tissues, stromal nodules, and stromal sarcomas for immunoreactivity with a panel of eight antibodies. Normal proliferative-phase (five cases) and secretory-phase (five cases) endometrial stromal cells showed the following immunopositivity: vimentin 10 of 10, muscle-specific actin (MSA) 10 of 10, alpha-smooth muscle actin (alpha sm) 10 of 10, desmin nine of 10, cytokeratin (AE1/AE3 and CAM 5.2) zero of 10, epithelial membrane antigen (EMA) zero of 10, and S-100 protein zero of 10. Antibodies to vimentin, MSA, and alpha sm stained a greater number of proliferative-phase stromal cells as compared with secretory-phase cells. Only rare stromal cells were immunoreactive for desmin, except for one case in which predecidual cells were diffusely positive. Both endometrial stromal nodules reacted with antibodies to MSA, alpha sm, and desmin, and one was vimentin positive. Each was unreactive for epithelial markers and S-100 protein. The 12 endometrial stromal sarcomas had the following immunopositivity: vimentin 11 of 12, MSA 10 of 12, alpha sm 10 of 12, desmin seven of 12, AE1/AE3 one of 12, CAM 5.2 two of 12, EMA zero of 12, and S-100 protein zero of 12. The antibodies to MSA and alpha sm usually stained a greater number of cells than did the desmin antibody. Three stromal sarcomas had sex cord-like areas, one of which exhibited focal CAM 5.2 positivity. These immunohistochemical findings for normal and neoplastic endometrial stromal cells indicate smooth muscle differentiation and are similar to those of smooth muscle neoplasms and myofibroblastic cells.
Cytoskeletal Proteins↗
Metastasis of malignant neoplasms to skeletal muscle.
Malignant neoplasms were found at 194 of 723 autopsies carried out at the Marqués de Valdecilla National Medical Center in the period 1980-1982, and in 50 of these there was secondary involvement of skeletal muscle. In 16 cases (8.2%) the primary tumour and/or its metastases had invaded skeletal muscle, but in 34 (17.5%) true macroscopic or microscopic metastases were present. It was this latter finding which prompted us to carry out a detailed analysis. Below are given the percentages of malignant neoplasms of each type in which the tumours had metastasized to skeletal muscle and, in brackets, the number of metastases found in skeletal muscle as a percentage of all metastatic tumours. Acute leukaemias 17.2% (14.7%), lymphomas 20.6% (17.6%), carcinomas 17.7% (64.6%): for the various sub-categories of carcinoma the figures were: epidermoid carcinoma 10% (5.8%), adenocarcinoma 29.6% (47%) and anaplastic carcinoma 5.8% (2.9%). The muscles most commonly involved were the diaphragm (67.6%) and the iliopsoas (29.4%). The patients ranged in age from 26 to 84 years, mean age 62.8 years. The male/female ratio was 2.8/1. In advanced cancer an appreciable percentage of metastases occur in skeletal muscle.
Adult↗
Uterine smooth muscle tumors: utility of classification by proliferation, ploidy, and prognostic markers versus traditional histopathology.
CONTEXT: Accurate categorization of uterine smooth muscle neoplasms by light microscopic examination is difficult. Multiple classification schemes have been proposed based on mitotic rate, nuclear atypia, and the presence or absence of necrosis. None of these classification systems has been entirely successful. Multiple ancillary techniques have been tested for their ability to predict behavior of uterine smooth muscle tumors. OBJECTIVE: We assayed 45 smooth muscle neoplasms for a variety of proliferation markers, oncogene protein products, and DNA ploidy level to determine if these markers supplied prognostically useful information over and above that obtained by routine light microscopic assessment. STUDY DESIGN: Forty-five uterine smooth muscle neoplasms were assessed for DNA ploidy; silver-staining nucleolar organizer regions (AgNORs); percent nuclear proliferating cell nuclear antigen (PCNA); expression of p53, Her-2/neu, and MDM-2 protein; mitotic rate; and nuclear grade. These markers were correlated with histologic diagnosis and the occurrence of a clinically adverse event (death, metastasis, or recurrence). RESULTS: Diagnostic category (P <.001), nuclear grade (P <.002), mitotic activity (P <.001), mean AgNORs (P <.001), percent nuclear PCNA (P =.02), and expression of p53 (P =.02) all correlated with clinical outcome. No statistically significant correlation between clinical outcome and the categories MDM-2 expression, Her-2/neu expression, or DNA ploidy was seen. Nuclear grade, p53 expression, mitotic rate, AgNORs, and percent nuclear PCNA correlated with diagnosis. CONCLUSIONS: Diagnostic category, mitotic rate, AgNOR counts, PCNA, and p53 expression dichotomized uterine smooth muscle neoplasms into prognostically favorable and unfavorable groups. Although highly significant, the category AgNORs was no more successful than mitotic rate in dividing uterine smooth muscle neoplasms into prognostically favorable and unfavorable groups. Expression of p53 and percent nuclear PCNA dichotomized uterine smooth muscle neoplasms into prognostic groups, but neither technique reached the level of significance achieved by mitotic rate. Our data indicate that mitotic rate and the classification system of Kempson and Bari are at least as effective as the tested markers in separating uterine smooth muscle neoplasms into prognostic categories.
Aneuploidy↗
Prognostic parameters for survival of patients with malignant mesenchymal tumors of the uterus.
BACKGROUND: Malignant mesenchymal uterine neoplasms are the most aggressive type of primary uterine tumors, with most patients dying within a few years of diagnosis. Thus, it would be very important to define prognostic factors for predicting the malignancy potential of at least some of their subtypes. METHODS: Flow cytometric cell cycle analysis (proliferative activity, DNA ploidy, and DNA index) was performed on archival paraffin embedded blocks from 80 patients with malignant mesenchymal uterine neoplasms (endometrial stromal sarcomas, malignant smooth muscle tumors, and malignant Müllerian mixed tumors). The Cox proportional hazards regression model was used to assess relative effects of the following factors on patient survival: clinical stage, mode of therapy, DNA+proliferative activity, DNA index, histologic type, cellularity, degree of atypia, mitotic activity, and depth of myometrial invasion. RESULTS: There were 9 low grade stromal sarcomas, 17 high grade stromal sarcomas, 8 smooth muscle neoplasms with uncertain malignant potential, 23 leiomyosarcomas, and 16 homologous and 7 heterologous malignant Müllerian mixed tumors. In univariate analysis for stromal sarcomas, statistical significance was found for DNA ploidy+proliferative activity (P < 0.001), histologic type (P = 0.005), and DNA index (P < 0.001). In multivariate analysis, DNA index appeared to be the only significant parameter influencing patient survival (P = 0.005). In univariate analysis for malignant smooth muscle neoplasms, statistical significance was detected for mitotic activity (P = 0.049) and International Federation of Gynecology and Obstetrics classification (P = 0.021), but in multivariate analysis, clinical stage appeared to be the only significant parameter influencing patient survival (P = 0.032). In univariate analysis for malignant Müllerian mixed tumors, statistical significance was found for the depth of myometrial invasion (P = 0.039), DNA index (P = 0.037), and clinical stage (P = 0.013), but in multivariate analysis, only the depth of myometrial invasion (P = 0.036) and clinical stage (P = 0.025) were of statistical significance. CONCLUSIONS: The most powerful prognostic indicator for stromal sarcomas was the DNA index, for malignant smooth muscle neoplasms it was the clinical stage, and for malignant Müllerian mixed tumors it was the depth of myometrial invasion.
Analysis of Variance↗