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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↗
PubMed1976

Gastric epithelioid leiomyoma and leiomyosarcoma (leiomyoblastoma).

A series of 127 surgical specimens of epithelioid leiomyomatous tumors (leiomyoblastomas) of the gastric wall from the files of the Armed Forces Institute of Pathology (AFIP) were studied as to biologic behavior, morphogenesis, and histologic features of value in distinguishing benign and malignant variants. These tumors affect middle-aged men primarily and usually present with upper gastrointestinal bleeding or peptic ulcer-like symptoms. They are composed of a mixture of round epithelioid and spindle cells, many of which have clear cytoplasm. The cells are ensheathed by delicate reticular fibers. The presence of a perithelial or glomoid pattern in some tumors suggests a possible relationship to angiomyoma, glomus tumors, and "pericytoma." The epithelioid leiomyoma, the benign form, often arises in the mid- and distal stomach, especially on the anterior wall. Microscopically, it is recognized by the presence of large epithelioid cells and infrequent mitotic figures. Of 103 epithelioid leiomyomas, only one metastasized and thus was biologically malignant. The epithelioid leiomyosarcoma often arises in the proximal stomach and also distally, especially on the posterior wall. Two histologic types of epithelioid leiomyosarcoma are distinguished from the benign epithelioid leiomyoma by the small size of the cells and occasional higher mitotic counts. One sarcoma variant is a small cell caricature of the leiomyoma. The other is more anaplastic, assoicated with a loss of reticular fibers surrounding the cells and an alveolar arrangement. Epithelioid leiomyosarcomas are the most common type of gastric sarcoma. They are aggressive neoplasms; 63% metastasized, usually within 2 years after diagnosis.

Adolescent↗
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↗
PubMed1976

[Cutaneous leiomyosarcoma and osteomedullary plasmocytoma with the demonstration of IgA-kappa paraprotein in serum and skin tumor].

A dermal leiomyosarcoma associated with a still asymptomatic osteomedullary plasmocytoma with IgA paraproteinemia develop-d rapidly in a 78-year-old man. The same paraprotein type IgA kappa was identified, with distinct decrease in concentration, in serum, sarcoma-tissue, and in the tumour-surrounding skin area by m-ans of immunoelectrophoresis, radial immunodiffusion, and Ouchterlony test. The immunochemical identity of the paraprotein, the course of the disease as followed clinically and immunochemically, and several histological criteria are in favour of monoclonal origin of the paraprotein from malignant plasmocytoma cells. The accumulation of paraprotein in the sarcoma tissue is primarily explained by the marked blood congestion of the tumour. To our knowledge no report exists in the medical literature of an association of dermal leiomyosarcoma and early paraproteinemic plasmocytoma. This coincidence may be only of chance but we rather suggest an immunopathological relation of both tumors due to a partial immune insufficiency caused by the preceding plasmocytoma, resulting in a diminished immunological "surveillance" of the organism and favouring the development of a new malignant cell population (i.e. leiomyosarcoma).

Aged↗
PubMed1977

Leiomyosarcoma of the rectum and prostate.

A 47-year-old man with leiomyosarcoma involving the rectum and prostate was treated by radical abdominoperineal resection. This case appears to be unique in that leiomyosarcoma was present in both the rectum and the prostate. The literature is reviewed, highlighting some interesting clinical features of this condition. The prognosis for leiomyosarcoma of both rectum and prostate is poor.

Humans↗
PubMed1976

Renal vein leiomyosarcoma.

The third reported case of renal vein leiomyosarcoma is presented. Diagnosis was not made until exploratory celiotomy revealed a tumor originating from the left renal vein. The tumor was resected with margins of normal vein, and the patient was alive without recurrence 12 months after operation. Review of 65 cases of leiomyosarcoma originating in other retroperitoneal structures revealed a two-year survival rate of 20%. Therefore, we recommend consideration of postoperative adjunctive chemotherapy for renal vein leiomyosarcomas.

Abdominal Neoplasms↗
PubMed1976

Leiomyosarcomas of the small intestine induced in dogs by N-methul-N'-nitro-N-nitrosoguanidine.

Leiomyosarcomas of the small intestine were found in dogs during experimental induction of gastric carcinoma by oral administration of N-methyl-N'-nitro-N-nitrosoguanidine (MNNG). Leiomyosarcomas were found most frequenly in the duodenum and jejunum, and occasionally in the stomach but never in the ileum, colon, or rectum. The leiomyosarcomas developed in all the dogs given 50 mug/ml of MNNG in deionized water to drink but not in dogs fed on porridge food made from standard pellet diet mashed with MNNG at the same concentration in tap water. Intestinal sarcomas developed in 3 months to 5 years after the end of MNNG administration, and frequently metastasized to the liver and/or the peritoneum.

Administration, Oral↗
PubMed1976

[Cytology and histology of vaginal leiomyosarcoma: a rare tumor (author's transl)].

Leiomyosarcomas as primary tumors are usually found in the corpus uteri or the cervix, they are extremely rare in the vagina. Case report of a 45 yr old woman with primary Leiomyosarcoma of the vagina. The cytological diagnosis was difficult, as a differentiation between Leiomyosarcoma, Melanoma and mesodermal mixed-tumor by means of cytology is not univocal. The histological diagnosis is certain. The tumor is demonstrated in its many histological and cytological aspects. The reaction to radiation is poor. Prognosis depends upon time of diagnosis and treatment and is usually unfavorable.

Diagnosis, Differential↗