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Immunohistochemical and immunoblotting detection of cytokeratin in smooth muscle tumors.

Forty-six smooth muscle tumors, including 35 of gastrointestinal origin, were studied immunohistochemically for the localization of cytokeratin using a variety of monoclonal antibodies. In formalin-fixed, paraffin-embedded material, six of 40 leiomyomas, two of five leiomyosarcomas and one leiomyoblastoma were immunoreactive for cytokeratin in a few tumor cells. A proportion of non-neoplastic smooth muscle cells also exhibited a positive reaction. In fresh frozen sections of one gastric leiomyosarcoma, a high percentage of tumor cells were reactive with nine of the eleven anti-cytokeratin monoclonal antibodies examined. Cytokeratin-positive cytoplasmic filaments were further demonstrated by immunoelectron microscopy. By immunoblot analysis, an extract of this immunohistochemically cytokeratin-positive leiomyosarcoma showed a distinct band at the same position as an extract of pancreas, whereas no bands were seen in an extract of a cytokeratin-negative esophageal leiomyosarcoma. These immunohistochemical and immunoblotting findings indicate that a certain subset of smooth muscle neoplasms express genuine cytokeratin filaments.

Humans↗

Mitochondria as a feature of smooth muscle differentiation: a study of 70 smooth muscle tumors.

We report that the presence of numerous mitochondria is an ultrastructural feature of smooth muscle neoplasms which is diagnostically useful as a marker for smooth muscle differentiation. The number of mitochondria, as well as the usual features of smooth muscle differentiation, were studied in 70 smooth muscle neoplasms from a variety of body sites. The tumors were sub-classified according to the number of mitochondria (in the majority of the tumor cells) as sparse, moderate, abundant or packed. Thirty-one percent of the leiomyomas had sparse mitochondria and 69% had a moderate number of mitochondria. Seven percent of the leiomyoblastomas contained a moderate number of mitochondria, 33% contained abundant mitochondria and 60% were packed with mitochondria. Twelve percent of the leiomyosarcomas showed sparse mitochondria, 67% showed a moderate number of mitochondria, and 21% had abundant mitochondria. For the above tumor types, the cells with abundant and packed mitochondria contained few or no visible filaments, and these cells were often round or polygonal. By contrast, adjacent spindle cells often contained numerous filaments including dense bodies. The recognition of mitochondria as a feature of smooth muscle differentiation is diagnostically useful in tumor samples showing numerous mitochondria and a paucity of filaments and dense bodies.

Biomarkers, Tumor↗

Smooth muscle tumor of the pleura. A case report and review of the literature.

Smooth muscle tumors of the serosal membranes are extremely rare and have received little attention in the literature. To the best of our knowledge, only 1 published series of 5 pleural smooth muscle neoplasms has been published to date. We describe a primary pleural neoplasm with smooth muscle differentiation documented by light microscopy, immunohistochemistry, and electron microscopy. This tumor originated in the parietal pleura in a 32-year-old white man and was diagnosed incidentally by chest radiography; the diagnosis was confirmed by magnetic resonance imaging and biopsy. Four years later, the tumor was noted to have increased in size and disseminated into the chest wall as a separate circumscribed mass located in the pectoral muscle. Both masses were resected and diagnosed as smooth muscle tumors. We conclude that smooth muscle tumor of the pleura is a well-defined entity with a low, but definite malignant potential; therefore, we recommend complete resection and long-term follow-up for all patients.

Actins↗

Splenic smooth-muscle tumors in children with acquired immunodeficiency syndrome: report of two cases of this unusual location with evidence of an association with Epstein-Barr virus.

Smooth-muscle neoplasms are rarely located in the spleen. They have been previously reported in five cases of children with human immunodeficiency virus (HIV) infection/acquired immunodeficiency syndrome (AIDS). Two cases of children with HIV infection/AIDS with autopsy and surgical pathology evidence of multiple smooth-muscle neoplasms with splenic involvement are presented. DNA was extracted from histology slides in both cases for analysis for Epstein Barr (EB) virus. In both cases, the presence of EB virus was confirmed. This paper documents two additional cases of the unusual phenomenon of splenic involvement by smooth-muscle neoplasms in the setting of AIDS in childhood and further supports the role of EB virus in the development of these neoplasms.

Acquired Immunodeficiency Syndrome↗

Thick (myosin) filaments in a glomus tumor.

An otherwise classic digital glomus tumor is presented with the unusual ultrastructural finding of cytoplasmic thick (myosin) filaments together with thin (actin) filaments in many of the cells. In places, sarcomere-like orientation was seen. It is little appreciated among diagnostic pathologists, but is well-established, that thick (myosin) filaments occur in smooth muscle type cells. They are present in vivo and can be demonstrated ultrastructurally if rather stringent preparative conditions are met. Whether or not the contractile process in smooth muscle is analogous to skeletal muscle is a debated issue. In the context of diagnostic electron microscopy, it is stressed that thick filaments are not, as often stated, pathognomonic of skeletal muscle neoplasms, and may potentially be found in smooth muscle neoplasms and neoplasms of related cell type (glomus tumors, hemangiopericytomas, tumors of myofibroblasts, etc.).

Actins↗

Comparative analysis of smooth muscle isoactin gene expression in normal and neoplastic tissues.

Monoclonal antibodies derived from the actin multigene family are routinely used as an adjunct to morphologic diagnoses of smooth muscle tumors. Northern blot analysis was performed on 60 surgical resections utilizing isoactin-specific cDNAs. A comparison of this analysis to immunohistochemical studies demonstrated that actin-specific monoclonal antibodies represent reliable markers of the smooth muscle lineage. Smooth muscle neoplasms showed a unique pattern of gamma-smooth muscle isoactin gene expression, providing a potentially valuable molecular adjunct to the morphologic diagnosis of uterine smooth muscle tumors.

Actins↗

Breast leiomyoma.

Smooth muscle neoplasms exist as a spectrum of pathologic processes ranging from benign leiomyoma to anaplastic leiomyosarcoma. Although these tumors typically occur in regions where there is an abundance of smooth muscle, they may also be derived from the muscularis of the gastrointestinal tract or the media of blood vessels. The majority of breast leiomyomas are contiguous with the muscular components of the nipple areolar complex. Strong, in 1913, is credited with the early descriptions of leiomyoma of the mammary gland. There have been relatively few reports of this entity since Strong's initial description. Smooth muscle neoplasms of the breast present unique clinical challenges for which therapeutic decisions are based on criteria extrapolated from the management of similar tumors at other sites. This report high-lights key clinical and pathologic findings in a postmenopausal woman with a discrete breast mass of benign smooth muscle histology.

Breast Neoplasms↗

Adult rhabdomyoma of the extremity: a case report and review of the literature.

The adult rhabdomyoma is a rare, benign skeletal muscle neoplasm that usually occurs in the head and neck. A case report of an adult rhabdomyoma arising in the thigh is presented with a review of the literature. This is the first case of an extremity adult rhabdomyoma to be reported. It is also the largest at 13 centimeters. Distinction from a highly differentiated rhabdomyosarcoma is important. Recent chromosomal studies suggest that the adult rhabdomyoma is a true neoplasm. Total resection is curative but the lesion may recur if incompletely excised.

Aged↗

Piloleiomyoma--a report of five cases.

Piloleiomyomas are uncommon smooth muscle neoplasms of the skin with a few reproted cases in Indian literature (1,2,3,4,5). They are often misdiagnosed clinically. A correct biopsy report is important because patients may have to be managed medically since surgery is associated with a high rate of recurrence. The classical histologic findings, and Masson's stain to confirm the smooth muscle origin aids in the correct diagnosis.

Adolescent↗

Aggressive angiomyxoma of pelvic parts exhibits oestrogen and progesterone receptor positivity.

AIMS: Aggressive angiomyxoma of pelvic parts is a distinctive soft tissue tumour that chiefly involves the vulvar and perineal region of female patients. Several previous reports have demonstrated oestrogen receptor (ER) and/or progesterone receptor (PR) positivity in this neoplasm. The aim of this study was to confirm whether ER and/or PR positivity is present in aggressive angiomyxoma. We also wished to ascertain whether positivity may be found in the stromal cells of normal vulval skin and in other lesions at this site that can cause diagnostic confusion with aggressive angiomyxoma. METHODS: Five aggressive angiomyxomas in female patients and one involving male pelvic soft parts were stained immunohistochemically with antibodies against ER and PR. Other samples studied were normal vulval skin (n = 7), fibroepithelial polyps of vulva (n = 7), vulval smooth muscle neoplasms (n = 5), vulval nerve sheath tumours (n = 2), vaginal angiomyofibroblastoma (n = 1), and pelvic myxoma (n = 1). Nuclear staining was classified as negative, weak, moderate, or strong and the proportion of positively staining cells was categorised as 0, < 10%, 10-50%, or > 50%. RESULTS: All five cases of aggressive angiomyxoma in female patients were positive for ER (two with weak intensity involving < 10% of cells and three with moderate intensity involving 10-50% of cells) and four of five cases were strongly positive for PR in > 50% of cells. The other case was negative for PR. There was no staining with antibodies to ER or PR in the single male patient with aggressive angiomyxoma. Other samples exhibiting positivity of the stromal cells for either ER or PR were normal vulval skin (five of seven, ER; two of seven, PR), fibroepithelial polyps (four of seven, ER; five of seven, PR), smooth muscle neoplasms (three of five, ER; four of five, PR), nerve sheath tumours (one of two, ER; one of two, PR), angiomyofibroblastoma (one of one, ER; one of one, PR), and pelvic myxoma (one of one, PR). CONCLUSIONS: All cases of aggressive angiomyxoma of pelvic soft parts in female patients exhibited positivity for ER and/or PR. Because of its propensity to occur in female patients during the reproductive years, it is possible that aggressive angiomyxoma is a hormonally responsive neoplasm. However, dermal fibroblasts in normal vulval skin and stromal cells in a variety of vulval lesions can also be positive. ER or PR immunoreactivity cannot be used to distinguish aggressive angiomyxoma and its histological mimics.

Biomarkers, Tumor↗

Localization of myoglobin in normal and neoplastic human skeletal muscle cells using an immunoperoxidase method.

Using an immunoperoxidase method, myoglobin is localized in the cytoplasm of normal and neoplastic human skeletal muscle. The staining intensity is variable in individual cells. This can be explained by the variable concentration of myoglobin in the different fiber types of normal muscle or the different degree of differentiation in neoplastic muscle cells, respectively. A potential application of this method in tumor pathology is discussed in connection with myoglobin as a tissue-specific marker for skeletal muscle neoplasms.

Humans↗

Late relapse of adrenocortical carcinoma in Beckwith-Wiedemann syndrome. Clinical, endocrinological and genetic aspects.

UNLABELLED: We report on a girl with an unusual Beckwith-Wiedemann syndrome (BWS) and hemihypertrophy, who developed an adrenocortical carcinoma with atypical clinical behaviour. At 4 y of age the girls was admitted to hospital with cushingoid features, virilization, increased excretion of steroids and low serum ACTH. A right-sided adrenocortical carcinoma was removed. At age 12.5 y the cushingoid features reappeared together with a tumour in the left thigh. A CT scan of the thorax and abdomen revealed pulmonary metastasis only. Corticosteroid excretion was increased and serum ACTH level suppressed. The femoral and the pulmonary metastases were removed and histology showed adrenocortical carcinoma. Excretion of corticosteroids subsequently normalized. Meningeal and pulmonary metastases with similar histologies appeared one year later with normal hormone values. Twenty-two months after the recurrence the girl died of an intracranial metastasis. Southern blot analysis of the LITI transcript in the KvLQT1 gene in the BWS region on chromosome 11p15 revealed hypomethylation of the maternal allele. CONCLUSION: Adrenocortical carcinoma in childhood may recur years after onset and at rare sites and hormonal levels may be an insufficient indicator of small metastases.

Adrenal Cortex Neoplasms↗

A review and update of morphologically bland vulvovaginal mesenchymal lesions.

Vulvovaginal mesenchymal lesions composed of morphologically bland spindle-shaped cells often pose a particular diagnostic problem for the surgical pathologist not only because of the rarity of these lesions but also because of the wide array of entities with overlapping morphologic features. Included in this group of lesions are soft tissue neoplasms that may arise at any site and those that are characteristic of, or relatively specific to, the vulvovaginal region. Lesions that are relatively specific to the vulvovaginal region include well-known neoplasms such as aggressive angiomyxoma and angiomyofibroblastoma as well as more recently described lesions such as cellular angiofibroma and superficial cervicovaginal myofibroblastoma. Fibroepithelial stromal polyp, superficial angiomyxoma, and smooth muscle neoplasms also can occur in, but are not specific to, this site. In this review, the clinicopathologic features of these lesions are described with an emphasis on recent developments. The value of ancillary studies, especially immunohistochemistry, is discussed, although it is stressed that in general these are of limited value and routine morphology remains the mainstay in diagnosis. Morphologically bland spindle cell lesions that are not characteristic of the vulvovaginal region, but which also may occur here, are briefly discussed as are a variety of extremely rare mesenchymal lesions that have recently been described at this site.

Angiofibroma↗