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PubMed1998

Stomach leiomyosarcoma: secular trends in incidence and survival in Sweden, 1960-1989.

BACKGROUND: This population-based study was undertaken to investigate the epidemiology of stomach leiomyosarcoma (GIS tumors) and to study the prognosis in affected patients. METHODS: The Swedish Cancer Registry was used to identify all incident cases of stomach leiomyosarcoma from 1960 to 1989, and the Death Registry and the Registry of Population Changes were used for follow-up. RESULTS: The age-standardized incidence in men increased from 1.0 per 10(6 ) per year in 1960-69 to 1.8 per 10(6) per year in 1980-89. The corresponding rates in women were 0.6 per 10(6) and 1.2 per 10(6), respectively. The relative 5-year survival was 39.4% in men and 62.4% in women (P = 0.03). There was no statistically significant improvement in 5-year relative survival during the study period. CONCLUSION: The observed incidence of stomach leiomyosarcoma increased during the study period, most likely due to improved diagnostic accuracy. The survival of patients with this malignant tumor remained unchanged.

Adult↗
PubMed2006

Primary leiomyosarcoma of the oral tongue: magnetic resonance and ultrasonography findings with histopathologic correlation.

Leiomyosarcoma is a malignant smooth muscle tumor that frequently occurs in the gastrointestinal tract and female genital tract. It is aggressive and tends to recur and metastasize. Clinical behavior is unpredictable, mostly influenced by a proper surgical approach. Oral leiomyosarcoma, in particular of the tongue, is extremely rare and poorly documented in the radiology literature. Diagnostic assessment of oral leiomyosarcoma is often challenging, mostly founded on its peculiar immunohistological features. However, imaging evaluation is essential in staging and for preoperative planning. We illustrate the case of a 52-year-old woman with 2-months history of a painless growing mass on the left hemitongue, with magnetic resonance and ultrasonographic features correlated to histopathologic examination.

Female↗
PubMed2002

Primary leiomyosarcomas of the skin.

We report four cases of cutaneous, and one of subcutaneous, leiomyosarcoma with the results of histological examinations and immunohistochemical studies. Subcutaneous leiomyosarcomas have more likelihood of metastases than the cutaneous type. Only one of our cutaneous leiomyosarcomas recurred after 18 months and the patient with a subcutaneous lesion had lung metastases after five years.

Adult↗
PubMed1997

Laparoscopic resection of gastric leiomyosarcoma.

Wedge resection with a normal margin of stomach is generally considered adequate therapy for gastric leiomyosarcoma. We report here four patients with gastric leiomyosarcoma managed laparoscopically. The technique is described. There were no operative complications and all patients have been followed for 7 to 56 months. Three patients are free of disease and one patient with a high grade tumor is alive with liver metastases at 2 yars postoperatively. From this experience we conclude the following: 1) Laparoscopic wedge resection of gastric leiomyosarcoma is straightforward and easily accomplished without violation of standard surgical oncologic principles. 2) Intraoperative endoscopy is helpful in locating and manipulating the tumor, and aids in ensuring lumenal adequacy and staple line integrity. 3) Preoperative localizing studies may be misleading and should not discourage laparoscopic evaluation and treatment.

Aged↗
PubMed1975

Leiomyosarcoma of the long saphenous vein.

An unusual instance of primary leiomyosarcoma of the left long saphenous vein, possibly the tenth recorded case, is presented. Leiomyosarcoma of large veins appears to be a distinct entity, in contrast to the benign counterpart, leiomyoma. The origin of the tumor from the vessel wall, its intraluminal growth, and its ultrastructure are demonstrated. Incidence, sex and age distribution, fate and therapy of patients who have leiomyosarcomas of the large veins gleaned from reported cases in the literature are included.

Biopsy↗
PubMed1989

Primary recurrent leiomyosarcoma of the breast. Case report with ultrastructural and immunohistochemical study and review of the literature.

The authors report a case of recurrent mammary leiomyosarcoma in a 50-year-old woman. The neoplasia, with a recognized clinical evolution of 11 years, was resected on two occasions and had not metastasized. Microscopic examination showed 4 mitoses/10 high-power fields, moderate cytologic atypia, and, ultrastructurally, abundant myofibrils with condensations. Immunoperoxidase stains had positive results for muscle-specific antigen and showed focal reactivity for epithelial membrane antigen and S-100 protein. Analysis of the ten cases (including the present one) reveals that this neoplasm has appeared with greater frequency in women with an average age of 52 years. All neoplasms have been limited to the breast at the time of diagnosis. As a group, they have better prognosis than other sarcomas of the breast, although the possibilities of recurrence or dissemination exist, even many years after the primary extirpation. The size of the tumor and mitotic activity seem to be of little prognostic value. Mammary leiomyosarcoma shares clinical and pathologic similarities with subcutaneous leiomyosarcoma in other anatomic sites.

Actins↗
PubMed1998

Pedunculated esophageal leiomyosarcoma: a case report.

We report a case of esophageal leiomyosarcoma that possibly arose from the muscularis mucosae, thereby showing a particularly unusual appearance. A large polypoid intraluminal lesion in the distal esophagus was found on an endoscopic examination of a 68-year-old man with a 3-month history of dysphagia. Although the histological examination of biopsy specimens clearly revealed leiomyosarcoma, the absence of an exophytic component on computed tomography (CT) scan caused us to suspect that the tumor was carcinosarcoma. The tumor was resected by a subtotal esophagectomy. Microscopic evaluation revealed no involvement in the layer of the muscularis propria and no component of carcinoma. Clinical features of the pedunculated esophageal leiomyosarcoma shown in this case and three additional cases previously reported in the literature are reviewed.

Aged↗
PubMed1995

Sclerotic primary cutaneous leiomyosarcoma.

We describe the case of a painful primary cutaneous leiomyosarcoma that developed on the back of a 54-year-old white male over a 6-year period. The lesion had been sampled by punch technique and had been originally diagnosed as cutaneous sclerosis. Histologic examination of excisional tissue revealed a diffuse spindle cell neoplasm in the dermis that extended into the subcutis. There was extensive sclerosis and sparse cellularity in the deep portion and in several zones throughout the tumor. Immunostaining for desmin was negative, although stains for vimentin and smooth muscle actin were both strongly positive. Sclerotic cutaneous leiomyosarcoma should be recognized as a distinct but unusual variant of leiomyosarcoma that may be difficult to diagnose because of extensive sclerosis. Lesions may be painful and should be considered in the differential diagnosis of painful cutaneous neoplasms of the skin.

Actins↗
PubMed1989

Leiomyosarcoma of the urinary bladder. A clinicopathologic and immunohistochemical study of 15 cases.

We present the clinicopathologic and immunohistochemical features of 15 vesical leiomyosarcomas. The nine male and six female patients ranged in age from 16 to 72 years (median: 49 years). Gross hematuria was the most common complaint. The tumors most often arose in the dome of the bladder (50%) and ranged in size from 1 to 10 cm (median: 5 cm). Five patients underwent total cystectomy, nine had a partial cystectomy or local excision, and one was only biopsied. Based on the highest of five sets of mitotic counts per case, eight tumors had one or fewer mitotic figures per 10 high-power fields (MF/10HPF); none of these patients developed metastases during a follow-up interval of 1 month to 20 years (mean: 62 months). Five tumors showed three to six MF/10HPF; one patient in this group died after 6 years with unresectable metastases, whereas the others are disease free with follow-up of 8 months to 8 years (mean: 62 months). Two tumors contained 10-15 MF/10HPF; one produced widespread metastases and was fatal after 3 months, whereas the other has not recurred or metastasized after 9 months. All tumors had infiltrating margins and invaded the bladder musculature--important features in their distinction from vesical leiomyoma. Nine leiomyosarcomas had zones of myxoid intercellular material; six of these were predominantly or exclusively myxoid. The myxoid foci often closely resembled inflammatory pseudotumor, particularly in superficial portions of the neoplasms. In two cases, an intense superficial plasma cell infiltrate mimicked a plasma cell granuloma. All 12 leiomyosarcomas studied immunohistochemically were negative for epithelial markers (cytokeratins and epithelial membrane antigen); staining for muscle-specific actin and vimentin was uniformly positive, and eight neoplasms also expressed desmin.

Adolescent↗
PubMed2004

Leiomyosarcoma of the kidney: a clinicopathologic study.

Leiomyosarcoma of kidney is a rare lesion for which limited data are available. Cases coded as leiomyosarcoma of the kidney from three institutions were reviewed. These cases comprised 3 men and 7 women, 40 to 75 years of age. Immunohistochemistry was performed where possible and clinical follow-up information was obtained. On immunohistochemical staining, 6 of 6 tumors expressed smooth muscle actin, desmin, calponin, and h-caldesmon, and epithelial membrane antigen was positive in 1 of 5. Tumors were negative for cytokeratin and S-100 protein. Follow-up information was available for 9 patients. Two patients had metastases at diagnosis, four developed metastases, and two had recurrent disease. Five of 9 died of disease. Two patients were alive with no evidence of disease after 19 and 60 months, and 2 patients were alive with disease after 48 months and 56 months. Comparing outcome with tumor grade, the one patient with grade 1 tumor was alive with no evidence of disease; of 5 patients with grade 2 tumor, 2 died of disease, 1 was alive with no evidence of disease, 1 was alive with disease, and 1 was alive with extensive disease; all 3 grade 3 patients died of disease. In summary, the majority of renal leiomyosarcomas are intermediate or high grade with correspondingly poor prognosis.

Adult↗
PubMed1992

Prognostic factors influencing survival in gastrointestinal leiomyosarcomas. Implications for surgical management and staging.

The appropriate surgical therapeutic options for either localized or more advanced disease in patients with gastrointestinal leiomyosarcomas remain unclear. A staging classification for this disease has not been adopted nor risk factors identifying patients at risk for recurrence defined. To address these issues, this study evaluated the influence of various clinicopathologic variables on overall and disease-free survival. In an univariate analysis of overall survival involving 191 patients, the Cox proportional hazards model identified four factors that were associated with a significantly better outcome: complete resection without tumor rupture (p less than 0.001), localized lesions (p less than 0.001), low grade of tumor (p = 0.02), and tumors smaller than 5 cm (p = 0.03). When interactive effects of these factors were taken into account, however, type of resection of the tumor was selected as the only significant prognostic factor in a multivariate analysis. Complete resection without tumor rupture improved overall survival of patients with localized disease (median, 46 months) as well as those with contiguous organ invasion (median, 36 months) or peritoneal implants (median, 36 months). In contrast, patients with incomplete resections survived for a median of 21 months. Patients with tumor rupture, despite removal of all gross disease, behaved similarly to those with incomplete resections; median survival was only 17 months. For disease-free survival, important determinants selected from a multivariate analysis were tumor rupture (p = 0.002), contiguous organ invasion (p = 0.02) and high tumor grade (p = 0.02). A staging classification incorporating these prognostic factors of significance was evaluated using a TGM system: T1 (less than 5 cm), T2 (greater than or equal to 5 cm), T3 (contiguous organ invasion or peritoneal implants), T4 (tumor rupture); G: G1 (low grade), G2 (high grade); M: M0 (no metastases), M1 (metastases present). The corresponding 5-year overall survivals for stages I, II, III, IVA, and IVB were 75%, 52%, 28%, 12%, and 7%. Disease-free survival at 2 years after surgery was 89%, 57%, and 47% for stages I, II, and III, respectively. In conclusion, surgery remains the primary modality of treatment for patients with gastrointestinal leiomyosarcomas, and complete resection of all disease without tumor rupture, even of locally advanced disease, improves overall and disease-free survival. A staging classification appears feasible and is recommended to determine outcome in patients with leiomyosarcomas arising from the gastrointestinal tract.

Actuarial Analysis↗
PubMed1980

Leiomyosarcoma metastatic to the liver: CT appearance.

Leiomyosarcoma is an uncommon malignancy that metastasizes to the liver and lungs. Each of the three cases of leiomyosarcoma metastatic to the liver examined by computed tomography demonstrated similar morphologic and dynamic characteristics. Common features included hepatomegaly and large, circumscribed "cannonball" lesions that distorted normal hempatic architecture. These larger metastases were sharply defined with central necrosis and were accentuated by contrast enhancement of normal surrounding liver. Smaller perfused metastases often became isodense following contrast medium administration. This combination of features and effects should alert one to the possible diagnosis of metastatic leiomyosarcoma and cautions against underestimating the extent of disease by having precontrast control scans for comparison.

Aged↗
PubMedDate not supplied

Leiomyosarcoma of the kidney: CT and MR appearance.

A case of leiomyosarcoma of the kidney is reported here with its CT and MRI manifestations correlated to the pathologic findings. The leiomyosarcoma appeared as a well-defined multinodular mass of varying density and signal intensity on CT and MRI, respectively. The low signal-intensity regions on T2-weighted MRI exhibited delayed enhancement on CT and contained more fibrous tissues than did other sites that had more spindle-shaped muscle cells. Knowledge of the MR and CT characteristics may permit radiologists to refine the diagnosis of renal leiomyosarcoma considerably.

Female↗
PubMed2000

Lumbosacral radiculopathy secondary to metastatic uterine leiomyosarcoma: a case report.

STUDY DESIGN: A retrospective case report. OBJECTIVES: To increase awareness of the fact that very serious and potentially devastating conditions can be associated with lumbosacral radiculopathy. To reinforce the need to have a definitive diagnosis before performing epidural injections in patients with radicular pain who are not responsive to conservative treatment. SUMMARY OF BACKGROUND DATA: To the authors' knowledge, this is the first reported case of uterine leiomyosarcoma presenting with a lumbosacral radiculopathy. METHODS: The authors describe the treatment and the radiologic, surgical, and pathologic findings in this patient. RESULTS: Proper diagnostic work-up led to a diagnosis of metastatic uterine leiomyosarcoma, which was managed with decompressive laminectomy, radiotherapy, and chemotherapy. CONCLUSIONS: This is the first reported case of a uterine leiomyosarcoma presenting with radicular pain. When a patient has not responded to conservative care, a definitive etiology for radiculopathy needs to be established before epidural steroid injection.

Female↗
PubMed2003

Loss of 13q14-q21 and gain of 5p14-pter in the progression of leiomyosarcoma.

Leiomyosarcomas of soft tissues are an aggressive group of tumors with a high incidence of recurrence. Little is known about the molecular genetic changes associated with clinical outcome. Therefore, we studied 28 leiomyosarcoma samples of similar grade using comparative genomic hybridization and DNA flow cytometry and identified a difference in survival time associated with ploidy status and the number of chromosomal aberrations. The average survival time was shown to decrease with increase in chromosomal aberrations identified using comparative genomic hybridization. The average survival time was shorter in the near-tetraploid group than in the diploid and triploid group. Gain of 5p14-pter was significantly more common in near-tetraploid tumors. The survival time of patients with near-tetraploidy together with gain of 5p14-pter was reduced, and 50% died within the 1st year. Furthermore, loss of 13q14-q21 was significantly more frequent in the <5-year than in the >5-year survival group (P =.01). These results suggest that 13q14-q21 loss and 5p14-pter gain at diagnosis could be used to identify patients with leiomyosarcoma who are likely to have a shorter survival time and who might benefit from early treatment intensification.

Adolescent↗
PubMed2005

Subcutaneous leiomyosarcoma of the frenulum.

Leiomyosarcomas of the penis are rare, with only 29 reported cases to date. We record the case of a patient who presented with a 2-year history of a seemingly indolent penile skin lesion. On histopathology of the local resection, a diagnosis of subcutaneous leiomyosarcoma was made. Specifically, leiomyosarcoma of the penile frenulum has not been clearly reported previously. The patient underwent a further excision to ensure an adequate resection margin and has had no disease recurrence at subsequent follow-up. Our case was of a lesion that, although clinically benign, was malignant and this possibility should be borne in mind when assessing patients.

Biomarkers, Tumor↗
PubMed1995

Allelic loss at the tuberous sclerosis (Tsc2) gene locus in spontaneous uterine leiomyosarcomas and pituitary adenomas in the Eker rat model.

Hereditary renal carcinomas (RCs) develop in virtually all Eker rats by the age of one year. Investigation of extra-renal primary tumors co-occurring in Eker rats late in life (at 2 years) additionally revealed enhanced development of hemangiosarcomas of the spleen, uterine leiomyosarcomas and pituitary adenomas, although the demonstrated predilection for these extra-renal tumors was not as complete as with RCs. We identified the germline mutated tuberous sclerosis (Tsc2) gene as the predisposing Eker gene and revealed the tumor suppressor nature of Tsc2 gene function in renal carcinogenesis. In the present study, we examined allelic loss at the Tsc2 gene locus in uterine leiomyosarcomas and pituitary adenomas developing in hybrid F1 rats carrying the Eker mutation as well as in pituitary adenomas from non-carrier rats. We detected loss of heterozygosity in 4 of 11 uterine leiomyosarcomas (36%) and 11 of 31 pituitary adenomas (35%) from Eker rats but in none of 9 pituitary adenomas from non-carrier rats (P < 0.05), suggesting that inactivation of the Tsc2 gene is also a critical event in the pathogenesis of these extra-renal tumors. Our present data indicate that there might be different pathways for tumorigenesis of pituitary adenomas between Eker and

Adenoma↗