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At least 19 recordsLinked to original sources
PubMed2026

Management of Soft Tissue and Visceral Leiomyosarcomas.

IMPORTANCE: Leiomyosarcoma is a rare and heterogeneous malignant mesenchymal neoplasm associated with substantial morbidity and mortality. Given recent advances in biologic understanding and the complexity of leiomyosarcoma, a consensus-driven approach is needed to harmonize management and address remaining clinical and research gaps. OBJECTIVE: To provide an evidence-based synthesis of current diagnostic and therapeutic approaches for leiomyosarcoma by an international panel of physicians, researchers, and patient advocates, focusing on site-specific management, systemic therapy strategies, and key areas of clinical uncertainty, while identifying unmet needs and research priorities. EVIDENCE REVIEW: This review is based on a comprehensive evaluation of the literature, including clinical trials, observational studies, and international consensus guidelines. Sources were identified through MEDLINE (via PubMed) and Embase database searches and reference screening, then supplemented by multidisciplinary expert consensus. Emphasis was placed on studies informing diagnosis, surgical management, radiotherapy, and systemic therapy in leiomyosarcoma. FINDINGS: The rarity and heterogeneity of leiomyosarcoma poses substantial challenges in its management. In localized disease, complete surgical resection remains the cornerstone of treatment, with evidence supporting the use of site-specific perioperative treatment strategies. Prospective data supporting neoadjuvant or adjuvant chemotherapy are lacking, and the role of radiotherapy differs across anatomic disease sites and institutions. In advanced disease, multiple systemic therapies demonstrate activity, including anthracycline-based and gemcitabine-based combinations, trabectedin, and tyrosine kinase inhibitors, although optimal sequencing after first-line therapy remains undefined. Emerging data suggest potential benefit from treatment continuation strategies and selected use of local therapies in oligometastatic settings. Molecular heterogeneity is increasingly recognized but has not yet translated into routine clinical implementation, and integration of molecular profiling into diagnostic pathways for predictive and therapeutic insights remains an unmet need. CONCLUSIONS AND RELEVANCE: This international consensus addresses the diagnosis and management of leiomyosarcoma. Management requires a multidisciplinary, site-specific approach informed by limited but evolving evidence. Key uncertainties persist, particularly regarding perioperative therapy, optimal sequencing and combination of systemic treatments, and integration of molecular data. Continued international collaboration and leiomyosarcoma-specific clinical trials are needed to refine treatment strategies and improve patient outcomes.

Journal Article↗
PubMed1979

Paratesticular rhabdomyosarcomas and leiomyosarcomas: a clinicopathological review.

One case of embryonal paratesticular sarcoma and 2 cases of leiomyosarcomas are reported. The 13-year-old boy with embryonal sarcoma is well after an orchiectomy and high ligation of the spermatic cord followed by radiotherapy and chemotherapy. A 66-year-old man has been doing well after orchiectomy and hemiscrotectomy for a paratesticular leiomyosarcoma. The third patient had a highly pleomorphic leiomyosarcoma and died 2 months postoperatively. The clinical and histological diagnosis of paratesticular rhabdomyosarcomas and leiomyosarcomas is reviewed and the various therapeutic approaches to these neoplasms are discussed. Retroperitoneal lymph node dissection, radiotherapy and chemotherapy are important adjuncts to orchiectomy in the management of rhabdomyosarcomas. On the contrary, retroperitoneal lymph node dissection and radiotherapy are not indicated in leiomyosarcomas, since these neoplasms tend to metastasize by the hematogenous route and are radioresistant. At the present time we are unable to evaluate chemotherapy in the management of paratesticular leiomyosarcomas.

Aged↗
PubMed1978

[Primary leiomyosarcoma of the liver (author's transl)].

The occurrence of leiomyosarcoma was demonstrated in a tissue cylinder obtained by liver biopsy in a 68-year-old woman with unclear hepatomegaly. The patient died 8 months after she had experienced first signs of illness. Autopsy revealed a primary leiomyosarcoma of the liver with metastases in the lungs, bilaterally in the pleura, in the kidneys, and in the periportal and spleno-pancreatic lymph nodes. The occurrence of a primary tumour in the urogenital system or in the gastrointestinal-tract could be excluded. The diagnosis of leiomyosarcoma was based on the microscopical demonstration of smooth muscle fibres with enlarged red-like and partially atypical cell nuclei and atypical mitoses. Furthermore, we observed extensive necroses and haemorrhagia in the tumour tissue. Preexisting tumours of the liver as, e.g., teratoma or hepatoblastoma were not found. No cirrhosis of the liver could be detected. Obviously, the leiomyosarcoma had its origin in the smooth muscle fibres of the liver vascular system.--According to the literature primary leiomyosarcomas in the liver are only rarely found.

Aged↗
PubMed1979

Primary leiomyosarcoma of the bone and its comparison with fibrosarcoma.

Two cases of primary leiomyosarcoma of the bone are recorded, one in the distal fibula of a 66-year-old man, the other in the proximal tibia of a 61-year-old woman. The cytological, histological, and ultrastructural features of leiomyosarcoma of bone are described and compared with those of fibrosarcoma. These features are sufficiently characteristic to enable a confident diagnostic distinction between leiomyosarcoma and fibrosarcoma. Nevertheless, certain basic similarities exist between these two tumors, manifested at the ultrastructural level by the presence of myofilaments in fibrosarcoma; it would seem that the observed differences relate to the degree of development of the myofilamentous structures. It is postulated that primary leiomyosarcoma of the bone need not necessarily always arise from the media of blood vessels; it might also conceivably develop through advanced myogenic metaplasia of a sarcoma originating from fibroblastic tissue.

Aged↗
PubMed1979

Vascular leiomyosarcoma: the malignant counterpart of vascular leiomyoma.

The clinical and pathological findings of six cases of leiomyosarcoma arising from blood vessels of different caliber are described. The term vascular leiomyosarcoma, having both a topographic and morphologic significance, is proposed for these tumors. The histologic pattern is characterized by a proliferation of atypical smooth muscle cells with a large number of intermingled blood vessels. Mitoses were counted per 10 high power field (hpf) and tumors were divided in three groups I, 10 to 20 mitoses, group II, 20 to 35 mitoses, and group III, more than 35 mitoses per 10 hpf. The mitotic index seems to be the most important pathological feature on which a prognostic evaluation for vascular leiomyosarcoma can be based. Tumors in group I had neither local recurrences nor metastases; the one tumor in group II had one local recurrence, but the patient is free of disease 6 years after surgical treatment; the three tumors in group III developed distant metastases and constitutional symptoms. Vascular leiomyoma, bizarre leiomyoma, and hemangiopericytoma are included in the differential diagnosis of vascular leimyosarcoma. The possibility that vascular leiomyosarcoma arising from small vessels represents the malignant counterpart of vascular leiomyoma is proposed.

Adult↗
PubMed1978

Angiography of abdominal leiomyosarcoma.

The spectrum of angiographic findings of abdominal leiomyosarcoma in 33 cases is presented, and the value of angiography in the diagnosis and management of such patients is discussed. Angiographic features of abdominal leiomyosarcomas are different depending upon the site of origin. Small bowel lesions are hypervascular, and those in the stomach and colon moderately vascular. Well circumscribed masses with enlarged feeding arteries and draining veins form the predominant presentation of small bowel leiomyosarcoma. In the retroperitoneum these neoplasms are usually hypovascular to moderately vascular, and displacement of major vessels, particularly the inferior vena cava, is the most common angiographic finding. Bladder leiomyosarcoma can be either moderately vascular or hypervascular. Vascularity of metastases is usually similar to that of the primary lesion.

Abdominal Neoplasms↗
PubMed1978

Leiomyosarcomas of the extremities: angiography as a diagnostic aid.

Thirteen cases of leiomyosarcomas of the extremities are reported. Most of the cases were operated on without information concerning benignancy or malignancy. Plain radiographs showed amorphous calcification in two cases. Three cases had angiographic study and all revealed malignant neovascularity. In no literature that we reviewed was angiography mentioned in the cases of leiomyosarcomas of the extremities. Venography alone has been done in many reported cases. The leiomyosarcoma of the extremities looks often benign but is notorious for local recurrences and distant metastases.

Adult↗
PubMed1978

Synchronous leiomyosarcoma and ameboma of the rectum: report of a case.

A case of leiomyosarcoma of the rectum associated with rectal amebiasis is presented. This is the first case to be reported. Initially, amebiasis obscured the histologic diagnosis of leiomyosarcoma, but incomplete regression following a course of treatment with metronidazole aroused the suspicion of malignancy, which was confirmed on repeat biopsy. A brief review of the features of leiomyosarcoma was also undertaken.

Aged↗
PubMed1977

Primary leiomyosarcoma of skin: a report and critical appraisal.

A primary leiomyosarcoma of skin was studied by light and electron microscopy and by histochemistry. Systematic evaluation of the entire neoplasm suggested that a single biopsy sample would show little cellular pleomorphism but could vary considerably in number of mitoses per mm2. Electron microscopy revealed a high degree of cytologic differentiation. Strong myosin ATPase activity and negative demonstrations for hydrolytic enzymes suggest a diagnostic profile which will clearly separate this neoplasm from malignant fibrous histiocytoma. High mitoses counts, the conventional criterion for malignancy of non-cutaneous smooth muscle tumors, may not be appropriately applied to primary leiomyosarcomas arising in the dermis. The findings in this case and a critical review of the literature suggest that reliable criteria for diagnosis of primary cutaneous leiomyosarcoma by light microscopy remain to be established.

Adenosine Triphosphatases↗
PubMed1978

Secondary leiomyosarcoma of the right ventricle. A surgical report.

A 48-year-old woman developed symptoms related to a tumour attached to the outflow tract of the right ventricle. Eleven years earlier she had undergone hysterectomy for leiomyosarcoma, and 4 years later a retroperitoneal mass was removed. One year later a further retroperitoneal mass was removed, and after a further 4 years a cutaneous tumour was removed from her back. Histologically these three tumours were identical with the leiomyosarcoma removed with the uterus at the primary operation. A confident preoperative diagnosis of recurrent leiomyosarcoma within the pericardium was made, and it was possible to remove this tumour using cardiopulmonary bypass with relief of symptoms. The patient remains well 15 months after surgery.

Female↗
PubMed1978

[Penile leiomyosarcoma. Ultrastructural study (author's transl)].

A case of leiomyosarcoma of the penis in a farmer aged 70 is described. The patient was treated by excision/biopsy, and six months later three little tumors recurred on the glans and prepuce. A wide local excision and direct suture was carried out. No troubles happened in the next eight months. The ultrastructural study confirmed the histogenesis showing: polymorphic cells, as well as clear and dark ones, reduction of intracytoplasmic myofilaments, anisocytosis and poikilokaryosis and wide infoldings of the nuclear membrane; very vascularized areas with confluent vesicles on the endothelial cells; and intersticial haemorrhages. This tumor is included among the superficial leiomyosarcomas of "low malignancy" of McKenzie Pratt et Ross, for which simple excision that avoid mutilations is suitable. Differential histologic diagnosis includes epithelioid sarcoma of Enzinger. Leiomyosarcoma of the penis is a very rare malinant tumor. Only 15 cases have been described in the literature. A further case is reported and the previous cases are reviewed.

Aged↗
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↗