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PubMed1998

Metastatic pulmonary leiomyosarcoma: cytopathologic diagnosis on sputum examination.

Pulmonary metastasis of sarcomas is not uncommon. Rarely, endobronchial involvement may result in exfoliation of diagnostic cells in sputum. This case report is of a 71-yr-old man with a history of lower leg leiomyosarcoma who developed multiple lung metastases. Sputum examination revealed malignant cells with pleomorphic, elongated, and cigar-shaped nuclei and occasional bipolar cytoplasmic processes. Immunoperoxidase studies on the smears using desmin and smooth muscle actin were strongly positive, consistent with leiomyosarcoma. Confirmation of metastatic lung disease by sputum cytology not only has prognostic importance but also obviates the need for further investigations.

Aged↗

PubMed1975

Nonepithelial tumors of the nasal cavity, paranasal sinuses, and nasopharynx: a clinicopathologic study. IV. Smooth muscle tumors (leiomyoma, leiomyosarcoma)

In a review of nonepithelial tumors involving the nasal cavity, paranasal sinuses, and nasopharynx, 256 lesions, 156 benign and 100 malignant, were identified. Of these, 8 were smooth muscle neoplasms, 2 leiomyomas, and 6 leiomyosarcomas. The clinical findings associated with these smooth muscle tumors are described, their histologic features illustrated, results of therapy presented, and clinicopathologic correlations made. The leiomyomas were small lesions found incidentally within polypectomy specimens, and were successfully treated by polypectomy. Leiomyosarcomas could not be eradicated easily by surgery, recurred frequently, did not respond well to radiotherapy, and carried a poor prognosis.

Adolescent↗

PubMed1975

An unusual leiomyosarcoma of the uterus containing osteoclast-like giant cells.

An unusual variant of a poorly differentiated leiomyosarcoma of the uterus, containing osteoclast-like giant cells, is described. Areas of the resected tumor bore a close resemblance to giant cell tumor of bone. It is postulated that these giant cells, as well as the osteoclast-like giant cells reported in a number of tumors of other tissues, originate from the monocyte/histiocyte group of cells. The diagnosis of leiomyosarcoma was possible only after the tumor had been examined in the electron microscope. The ultrastructural features are consistent with a smooth muscle origin of the neoplasm; the final assessment is based on the relative proportions and frequency of such structures as: bundles of myofilaments with focal densities, marginal densities, pinocytotic vesicles, and an external lamina.

Cell Nucleus↗

PubMed1978

A reassessment of uterine neoplasms originally diagnosed as leiomyosarcomas.

Twenty-eight uterine tumors originally diagnosed as leiomyosarcomas were histologically reclassified without prior knowledge of follow-up or clinical data. Thirteen (46%) neoplasms were reinterpreted as cellular or pleomorphic leiomyomas. They had sparse mitotic activity with three or fewer mitotic figures per 10 high power microscopic fields (MF/10 HPF). None recurred or metastasized, and all patients were alive from 6.3 to 23 years after operation (median of 14.2 years). In 15 cases the diagnosis of leiomyosarcoma (LMS) was confirmed. All showed hypercellularity, nuclear atypism and high mitotic activity. Mitosis counts ranged from 6 to more than 50 MF/10 HPF with 93% of LMS having at least 15 MF/10 HPF. These patients all died of LMS after post-operative intervals of 3 months to 7.5 years (median survival of 13 months). No consistent correlation was found between length of survival and the patient's menopausal status or histologic grade of LMS. The degree of mitotic activity is the single most reliable diagnostic criterion of malignant potential, albeit not the only one. Surgery alone is ineffective treatment for LMS and combination therapy with radiation therapy and/or chemotherapy should be considered.

Adult↗

PubMed1979

The triad of gastric epithelioid leiomyosarcoma, functioning extra-adrenal paraganglioma, and pulmonary chondroma.

The triad of gastric epithelioid leiomyosarcoma, functioning extra-adrenal paraganglioma, and pulmonary chondroma was found in two young female Mayo Clinic patients, and two of these tumors occurred in another two. Review of the world literature disclosed nine patients who probably had two or more of the three neoplasms. We are also aware of two other patients with two of the three tumors. The association of gastric epitheloid leiomyosarcoma, functioning extra-adrenal paraganglioma, and pulmonary chondroma may well constitute a syndrome because of 1) the improbability of the coincidental occurrence of this triad of tumors, 2) the multicentricity of the tumors in the organs or system affected, and 3) the development of the tumors at a young age. Because two of the components of the triad are potentially lethal, it is important that patients less than 35 years of age who have any one of the three tumors be examined periodically to search for the others.

Adolescent↗

PubMed1983

Leiomyosarcoma arising in an arteriovenous fistula.

The case of a man who developed a leiomyosarcoma in association with a surgically created arteriovenous fistula is reported. This unusual complication of a dialysis fistula is the first reported case of a leiomyosarcoma occurring in a surgically created fistula.

Aged↗

PubMed1983

Leiomyosarcoma of the colon in an infant. A case report and review of the literature.

A rare case of leiomyosarcoma of the sigmoid colon in a 7-week-old infant presenting with signs of intestinal obstruction followed by colonic perforation and peritonitis is reported, and the literature pertaining to childhood colorectal leiomyosarcoma is reviewed.

Age Factors↗

PubMed1984

Primary leiomyosarcoma of extragnathic bones. Case report and review of literature.

In a 60-year-old man, a swelling anteromedially just below the knee led to the discovery of an intraosseous leiomyosarcoma. It is the 13th documented case of primary leiomyosarcoma of bone outside the facial skeleton. Clinical and pathologic findings, modes of treatment and therapeutic results are reviewed, and theories of histogenesis discussed. As to the latter, ultrastructural features in our case support the pleuripotent mesenchymal rather than the vascular smooth muscle origin.

Adolescent↗

PubMed1986

Epithelioid leiomyosarcoma of the uterus.

Uterine epithelioid leiomyosarcoma is an unusual smooth muscle neoplasm. It is distinguished on cytoarchitectural grounds from the majority of leiomyosarcomas that arise in the uterus. Three cases of this atypical lesion are presented and the pathologic features are discussed. One patient is alive with no evidence of disease at 3.5 years, one patient has persistent disease at 4 years, and the third patient died of disease at 8 months. Fundamental differences in biologic behavior of this subset of uterine smooth muscle tumors cannot be discerned.

Adult↗

PubMed1986

Beta-chain human chorionic gonadotropin-producing leiomyosarcoma of the small intestine.

A 22-year-old patient with primary leiomyosarcoma of the small bowel presented with symptoms including nausea and vomiting, a symptom complex similar to hyperemesis gravidarum in the pregnant female. She was found to have elevated levels of serum beta-HCG which were localized to the leiomyosarcoma cells utilizing an immunoperoxidase staining technique. Thus, this patient presents evidence that beta-HCG may be produced by sarcoma cells and, therefore, that beta-HCG may be used as a tumor marker in some sarcoma patients.

Adult↗

PubMed1987

A clinicopathologic and immunohistochemical study of 16 patients with small intestinal leiomyosarcoma. Limited utility of immunophenotyping.

Clinicopathologic data including immunophenotypic expression of 16 new cases of small intestinal leiomyosarcoma are presented. Patient age ranged from 27 to 87 years (mean, 61 years) with a 2.2:1 male-female ratio. Eighty-eight percent of tumors occurred distally, i.e., in the jejunoileum. Considerable histologic heterogeneity was noted including epithelioid, myxoid, and sclerotic variants. Stratification by histologic grade suggests important clinical subsets. Only in grade I tumors is surgical extirpation alone potentially curative. Grade II tumors are associated with meaningful survival usually measured in years, however, nearly all patients can be expected to die from their tumors. Grade III sarcomas are fully malignant and rapidly progressive: except for one case with limited follow-up, all patients with Grade III sarcomas died of disease with a mean survival of only nine months (median, 2 months). Immunostaining with antibodies directed against vimentin, muscle-specific actin, desmin, and S-100 protein were performed. Only antivimentin was uniformly reactive. Sixty-three percent of cases tested showed actin immunoreactivity, and no tumors revealed immunohistochemically detectable desmin or S-100 protein. Although many small bowel stromal sarcomas appear "poorly differentiated" by immunohistochemical methods presently available, that they are indeed leiomyosarcomas seems most likely from their intramural location and light histologic study. The limited utility of immunophenotyping and the importance of histologic grading are stressed.

Actins↗

PubMed1988

Leiomyosarcoma of the thyroid gland. A case report with a comparative study of five cases of anaplastic carcinoma.

We report on a case of leiomyosarcoma of the thyroid with ultrastructural features and expression of intermediate filament proteins different from that of anaplastic carcinomas. Histologic examination of multiple sections showed that they were leiomyosarcomatous, and neither epithelial features nor giant cell configurations were found. Electron microscopic examination showed microfilament bundles with dense patches and discontinuous basal lamina. Desmin, vimentin, and actin were positive for tumor cells, and cytokeratin, thyroglobulin, and calcitonin were negative. In contrast, the anaplastic carcinoma cases did not contain microfilament bundles with dense patches in their tumor cells. There were no desmin-positive or cytokeratin-negative tumor cells. We concluded that this case was a primary leiomyosarcoma, presumably derived from the smooth muscle cell of the vascular wall.

Actins↗

PubMed1991

Disseminated leiomyosarcoma in a child with acquired immune deficiency syndrome.

A child with disseminated leiomyosarcoma and acquired immune deficiency syndrome (AIDS) is reported. She was originally believed to have peptic ulcer disease by radiographic and endoscopic evaluation but was found subsequently to have hypergastrinemia, hypochlorhydria, and a smooth muscle tumor. Leiomyosarcoma in children and its evolution in AIDS are discussed.

Acquired Immunodeficiency Syndrome↗

PubMed1991

Conservative management of anal leiomyosarcoma.

Leiomyosarcomas of the large intestine are unusual neoplasms, comprising less than 0.1% of all malignancies of the colon and rectum. Six cases of leiomyosarcoma of the anus have been reported. The optimal treatment for this neoplasm is not known. The standard surgical approach is abdominoperineal resection. The authors report the seventh case of this rare neoplasm and outline its treatment using local excision and iridium 192 brachytherapy in an attempt to preserve the anal sphincter. In selected patients, conservative surgery followed by radiation therapy may be an alternative to radical surgery, with the goals of local control of the disease and anal sphincter preservation. However, more experience is needed before this approach could be recommended routinely.

Anus Neoplasms↗

PubMed1992

Soft tissue leiomyosarcoma. A population-based epidemiologic and prognostic study of 48 patients, including cellular DNA content.

BACKGROUND: Leiomyosarcoma of soft tissue is a rare tumor. There are different opinions regarding epidemiology and prognosis. METHODS: Epidemiology and prognosis were analyzed in a consecutive, population-based series of 48 patients with subcutaneous and deep-seated leiomyosarcoma in the extremities and trunk wall with a complete follow-up of a minimum of 3 years. Cutaneous tumors were not included. RESULTS: The annual incidence was 0.13/10(5). The ratio of men to women was 1.2, and the median age was 65 years. The thigh was the most common location. Almost half of the tumors were subcutaneous. The median tumor size was 6 cm (range, 1-25 cm). All patients were treated with surgery, and in 19 cases it was combined with adjuvant radiation therapy or chemotherapy. The cumulative 5-year survival rate was 64%. Multivariate analysis indicated that age of 60 years or greater (relative risk [RR] = 8) and intratumoral vascular invasion (RR = 4) were independent risk factors for death resulting from tumor. DNA aneuploidy (RR = 4) and tumor necrosis (RR = 3) were associated with poor prognosis, but did not reach statistic significance. CONCLUSIONS: Advanced age, vascular invasion, and DNA aneuploidy could be used to identify prognostic subgroups.

Adult↗

PubMed1992

Metastasizing gastric epithelioid leiomyosarcomas (leiomyoblastomas) in young individuals with long-term survival.

RESULTS. Four patients with metastasizing epithelioid leiomyosarcoma of the stomach, three females and one male (15, 20, 22, and 25 years of age, respectively), are reported. Two patients had recurrent tumors in the gastric remnant. Liver metastases occurred in all patients, three of whom had lymph node metastases; two had peritoneal metastases. The patients are alive at 17, 19, 27 and 48 years after the diagnosis was made and 17-27 years after the first demonstration of metastases. One woman had multiple chondromatous hamartomas of the lung. Operations were performed when the patients had symptoms and during periods of no symptoms, as directed by the second-look principle. One patient had 10 operations. None of the patients received adjuvant therapy. All three women have given birth to healthy children after metastases were diagnosed. Three of the primary tumors were large (10-20 cm) and multinodular, features that have been associated with unfavorable prognosis. The four primary tumors had a similar light microscopic appearance, characterized by moderate cell and nuclear pleomorphism and low mitotic activity, 0.03-0.1/mm2. Ultrastructurally, a network of intermediate filaments was found within the cytoplasm of the tumor cells corresponding to the immunohistochemical positivity for vimentin. The immunohistochemical findings (negative immunoreaction for desmin and alpha smooth-muscle actin) and the ultrastructural analysis produced no evidence of the production of smooth-muscle cell myofilaments. However, there were tumor cells with an abundance of mitochondria and a paucity of filaments, features that may be characteristic of epithelioid leiomyomatous tumors. In addition, immunohistochemical negativity for cytokeratins, epithelial membrane antigen, S-100 protein, neuron-specific enolase, and chromogranin militate against an epithelial or neuroectodermal cell differentiation. CONCLUSIONS. An awareness of this type of gastric leiomyosarcoma in children and young adults is of importance in making correct assessments of prognosis and choosing an active therapeutic approach. The biologic background to the clinical behavior of these metastasizing tumors remains an enigma. Additional studies are needed to elucidate the biology of these tumors.

Adolescent↗

PubMed2003

Differential gene expression in leiomyosarcoma.

BACKGROUND: Leiomyosarcomas (LMS) are a common subtype of soft tissue sarcoma. The molecular causes of the disease remain unclear. METHODS: In the current study, gene expression in LMS, leiomyomas, and normal myometrium was examined. RNA was prepared and gene expression was determined using microarray analysis arrays containing approximately 12,000 known genes and 48,000 expressed sequence tags (ESTs). RESULTS: A number of genes were found to be differentially expressed in these sample sets, and six genes including cyclin-dependent kinase inhibitor 2A, diaphanous (Drosophila homolog) 3, doublecortin, calpain 6, interleukin-17B, and proteolipid 1 were found to be overexpressed in LMS compared with normal myometrium and 18 other tissues. Sets of genes were identified whose expression could be used to cluster samples with either LMS, leiomyomas, or normal myometrium. CONCLUSIONS: The authors concluded that differences in gene expression can be detected between LMS and leiomyomas, normal myometrium, and other tissues, and that these changes in gene expression may yield clues with regard to the pathophysiology of leiomyosarcoma.

Adult↗

PubMed1989

The influence of glucocorticoids on the growth of a human leiomyosarcoma cell line SK-LMS-1.

A cell line derived from a human leiomyosarcoma, SK-LMS-I, has cystolic [Bmax 201.6 +/- 39.7, means +/- SD, fm/mg cytosol protein KD 14.6 +/- 7.8 nM (n = 8)] and nuclear [Bmax 189.2 +/- 87.3 fM/mg DNA, KD 4.8 +/- 0.26 nM (n = 5)] glucocorticoid receptor, by Scatchard analysis of tumors grown in male athymic mice. Tumor growth of SK-LMS-I cells in male athymic mice is inhibited by daily s.c. injection of DEX 5 micrograms, DEX 25 micrograms, DEX 5 micrograms with 5 mu RU-486 and 5 micrograms RU-486. In sharp contrast, in vitro, glucocorticoid markedly stimulates the growth (as determined by cell number) of SK-LMS-I cells, principally at higher cell densities (days 10-21 of growth carried on over a 21- to 23-day period), the greatest stimulation being seen with DEX 10(-6) to 10(-8) M, and no stimulation being seen with DEX 10(-9) and 10(-10) M. In vitro, glucocorticoids with higher affinity for the GR stimulate growth, steroids with lower affinity inhibit growth. No alterations in cell-cycle distribution (percent G0/G1, S, or G2/m) could be found by flow cytometric analysis of glucocorticoid-stimulated asynchronously growing cultures. Single, isolated, untreated SK-LMS-I cells form colonies in soft agar with an efficiency of 1.78 +/- 0.10%. Pre-treatment of cells with DEX 10(-7) M increases this to 3.24 +/- 0.17%, while cells pre-treated with both DEX 10(-7) M RU-486 10(7) M form colonies with the same efficiency as untreated cells. Glucocorticoids have inhibitory effects on in vivo growth and stimulatory effects on in vitro growth of a GR-positive human leiomyosarcoma cell line.

Animals↗