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PubMed2025

Methylation Signatures Identify Two Distinct Clusters of Uterine Leiomyosarcoma With Unique Histologic and Clinical Behaviors.

Uterine leiomyosarcoma (uLMS) is a rare and deadly gynecologic malignancy. uLMS is histologically heterogeneous and presents with a wide spectrum of tumor differentiation, with a broad range of genomic DNA instability, which can make the diagnosis and prognosis of uLMS challenging. Methylation has emerged as a useful molecular tool in tumor classification and diagnosis in certain neoplasms. We initiated this study to investigate the role of global methylation in the differential diagnosis of uLMS from its mimics in correlation with pathologic characteristics and clinical outcomes. In this study, we performed array-based global methylation profiling analysis in a total of 71 uLMS and compared the methylation signatures of uLMS with several other uterine mesenchymal tumors and soft tissue leiomyosarcoma. We found that uLMS demonstrated distinct methylation patterns differing from all other tumor types. Notably, methylation profiling defines 2 distinct subgroups of uLMS with differing copy number alterations, resulting in unique histologic and clinical behaviors, further emphasized by differences in methylation pathway analysis. This study is the first to report methylation profiling as a useful diagnostic tool in differentiating uLMS from mimics and defines 2 subtypes of uLMS based on methylation signatures.

Humans↗
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↗
PubMed1978

Ultrastructure of gastric leiomyosarcoma.

Samples of gastric leiomyosarcomas from two male patients (69 years and 57 years of age) were studied by electron microscopy. The tumour cells contained abundant 50--90 A thick microfilaments and microtubules. Another cell type, regarded as immature leiomyosarcoma cell, contained numerous profiles of granular endoplasmic reticulum, free ribosomes and a well developed Golgi apparatus. There were intercellular junctions of zonula adherens-type between adjacent tumour cells. It was concluded that electron microscopy offers a valuable aid in the diagnosis of gastric neoplasms of smooth muscle origin.

Aged↗
PubMed1978

Leiomyosarcoma of the rectum: report of three cases.

Three cases of leiomyosarcoma of the rectum are reported. The first patient underwent palliative surgery and died 18 months after diagnosis; local radiotherapy was not effective and chemotherapy failed as well. The second patient underwent radical surgical treatment but monetheless the tumor recurred and the patient died 37 months after diagnosis. The third patient underwent radical surgery for a tumor that was in an early stage, has been followed for several months without evidence of disease. Review of the literature indicates that leiomyosarcoma of the rectum is the most malignant sarcoma of the gastrointestinal tract, and has a 20 per cent five-year survival rate. Even radical surgical treatment yields disappointing results. Following localized operations, recurrences appear in most cases, and this treatment is not recommended. Should an effective chemotherapeutic combination be found, chemotherapy as an adjuvant to surgery will probably be indicated.

Aged↗
PubMed1979

A case report of leiomyosarcoma originating in the ligamentum teres of the liver.

A case of leiomyosarcoma that appeared to have originated in the ligamentum teres hepatis of a 66-year-old female has been presented. The case was suspected of leiomyosarcoma originating in the ligamentum teres of the liver from the findings by preoperative examinations, and definitely diagnosed as such in a laparotomy and by histological examination of the resected tissue specimen.

Female↗
PubMed1979

Uterine leiomyosarcoma.

A series of uterine leiomyosarcomas was reviewed in an attempt to assign prognostic significance to clinical and pathologic features. Extension of the sarcoma at the time of initial diagnosis was associated with a dismal outcome; no patient with disease beyond the confines of the uterus survived. Histologic grade was a useful prognostic feature, although low-grade sarcoma can be associated with metastases. Mitotic count was also useful in prognosis, although it did not correlate as well with clinical outcome as did histologic grade. There was no number of mitoses below which the diagnosis of leiomyosarcoma was excluded.

Adult↗
PubMed1978

Hepatic leiomyosarcoma: ultrastructural study and review of the differential diagnosis.

A primary leiomyosarcoma of the liver in a 12 year old female is reported. It is the eleventh such tumor to be recorded, the first to be documented in an adolescent and the first to be studied electron microscopically. By light microscopy the well differentiated portions of the tumor were characteristic of leiomyosarcoma. Ultrastructurally a minority of tumor cells contained myofilaments along with cytoplasmic dense bodies and marginal dense plaques, the most distinctive subcellular markers of smooth muscle cells. A brief review of the differential diagnosis is provided.

Cell Nucleus↗
PubMed1978

An unusual multifocal leiomyosarcoma of the stomach: a light and electron microscopic study.

A multifocal leiomyosarcoma of the stomach originating from the muscularis mucosae with lymph node and distant metastases is described in a 66 year old man. The electron microscopic features of a representative tumor mass and a metastasis confirmed the smooth muscle histogenesis. The light microscopic appearance consistently suggested malignant fibrous histocytoma. The pathological features of gasttric leiomyosarcomas are reviewed with special emphasis on the problem of practical diagnosis. This case also indicates that not all sarcomas with storiform features are necessarily histiocytic in origin.

Aged↗
PubMed1978

Leiomyosarcoma of paranasal sinuses.

A leiomyosarcoma of the upper respiratory and food passages is a rarity. Two rare cases of leiomyosarcoma of the paranasal sinuses, of which one developed recurrence are reported.

Adult↗
PubMed1979

Primary leiomyosarcoma of the aorta.

A case of primary leiomyosarcoma of the abdominal aorta is reported. The tumor replaced the full thickness of the aortic wall with formation of a large saccular aortic aneurysm filled with an admixture of tumor and mural thrombus. Electron microscopy of the surgical biopsy of the tumor revealed neoplastic smooth muscle cells with myofilaments and a discontinuous basal lamina. To our knowledge, leiomyosarcoma of the aorta has never been reported.

Adult↗
PubMed1977

Primary leiomyosarcoma arising in the trunk of pulmonary artery: a case report and review of literature.

An autopsy case of a 46-year-old woman with primary leiomyosarcoma arising in the pulmonary artery is presented. This case is the first description in Japan and the 10th in the world literature as leiomyosarcoma in this part of the body. An electronmicroscopic observation of the postmortem material was useful to conform the histologic diagnosis, in addition to some kinds of staining method of histologic section.

Female↗
PubMed1977

Leiomyosarcoma of the duodenum: a report of three cases.

Three cases of leiomyosarcoma of the duodenum are reported in this paper. The first patient demonstrated the development of a leiomyosarcoma from a leiomyoma, and also posed diagnostic problems as a Pólya gastrectomy had previously been performed. The second patient presented with acute pancreatitis--a hitherto unreported complication of this condition. The third patient showed that even though the primary tumour was unresectable, the patient remained well for more than a year after diagnosis.

Acute Disease↗
PubMedDate not supplied

Leiomyosarcoma of the lung. Primary cytodiagnosis in two consecutive cases.

Two cases of primary leiomyosarcoma of the lung are described, in which primary cytodiagnosis of the tumor type was established prior to surgery. The cytologic features of the cell samples obtained and correlated histopathologic findings are described. The cytologic features of pulmonary leiomyosarcomas are sufficiently characteristic to allow definitive diagnosis, if representative cell samples are obtained.

Aged↗
PubMed1977

Establishment of a human leiomyosarcoma cell line.

A cell line designated SKN was established from the human uterine leiomyosarcoma of a 52-year-old female. The cell line has grown well and the serial passages were successively carried out 82 times within 12 months. The monolayer cultured cells revealed anaplastic and pleomorphic features, and they multipled rapidly without contact inhibition. Electron microscope studies revealed myoibrils but no virus-like particles, while chromosomal studies showed that all cultured cells were hyperploid, the modal number was 112, and the marker chromosome was present. The cells were transplanted into an immune-depressed hamster cheek pouch and produced a histological leiomyosarcoma resembling the original tumor.

Animals↗
PubMed1977

[Vena cava occlusion-syndrom by leiomyosarcoma. Two case reports (author's transl)].

Two cases of occlusion of the inferior vena cava by leiomyosarcoma are reported. This localisation is extremely rare for mesenchymal tumors. The neoplasm originated from the wall of the vena cava, expanded forward into the lumen of the vessel, in one case up to the right atrium. Collateral circulation, anasarca edema and varices develop by this occlusionsyndrome. The fatal outcome of the disease is usually determined by right heart failure and not by metastases directly. In most cases diagnosis is made by autopsy. In cavography the differential diagnosis of thrombosis might include the rare possibility of leiomyosarcoma of the caval vein. Only early diagnosis makes succesful surgical therapy possible.

Aged↗