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PubMed2003

Gene expression analysis of human soft tissue leiomyosarcomas.

Leiomyosarcoma of the somatic soft tissue is a rare malignant mesenchymal neoplasm that metastasizes to other organs in a subset of cases. Much remains to be learned about the mechanisms underlying the development of aggressive behavior of this tumor. It has been difficult to predict the clinical behavior of leiomyosarcomas using the morphology-based grading system, even though tumor size and histological grade have correlated with biologic behavior in some studies. In this study we analyzed the gene expression patterns of 35 samples of mesenchymal origin, including 11 cases of leiomyosarcomas of different histological grades arising in soft tissue and the retroperitoneum, using the Affymetrix U133a chips, which contain more than 22,000 genes and expression sequence tags (ESTs). We identified a set of genes whose expression was commonly altered in all leiomyosarcoma samples. In addition, we identified specific gene expression patterns in several subsets of the tumor. We used these alterations of gene expression to subclassify the leiomyosarcomas into 3 groups. Interestingly, the grouping of these samples correlated well with tumor differentiation and clinical aggressiveness. The analysis identified 92 genes that distinguish low-grade, well-differentiated leiomyosarcomas from less well-differentiated, high-grade, and metastatic leiomyosarcoma. Thesse alterations of gene expression appear to be correlated with the clinical behavior and histological grade of the tumor. The striking differences in terms of gene expression pattern among leiomyosarcomas of different differentiation status and clinical aggressiveness imply that several genetic abnormalities are responsible for the genesis and progression of this tumor.

Adolescent↗
PubMed2000

Transforming growth factor-alpha, epidermal growth factor receptor, and PCNA immunoexpression in uterine leiomyosarcomas and leiomyomas in B6C3F1 mice.

The role of growth factors in the development of murine uterine mesenchymal tumors is unknown. In this study, immunohistochemical expression of transforming growth factor alpha (TGF-alpha) and its receptor epidermal growth factor (EGF-R) was assessed in spontaneous uterine leiomyomas and leiomyosarcomas in B6C3F1 mice. Cell proliferation, which has been induced by some growth factors, was evaluated by immunohistochemical detection of an endogenous marker of cell proliferation, proliferating cell nuclear antigen (PCNA). PCNA labeling indices were determined and compared to the intensity and distribution of TGF-alpha staining in sequential sections of control myometrium or tumor tissue. Results showed uterine leiomyosarcomas had positive cytoplasmic staining for TGF-alpha; however, all uterine leiomyomas evaluated were negative. Positive EGF-R staining was also observed in the uterine leiomyosarcomas, but not in the leiomyomas. EGF-R immunoexpression was detected primarily within the cytoplasm of the leiomyosarcoma cells, with occasional nuclear immunoreactivity. Immunohistochemical staining for PCNA was more intense and there were increased numbers of positively staining nuclei in the leiomyosarcomas compared to samples of control myometrium or leiomyomas. The mean labeling index for the uterine leiomyosarcomas (7.40%) was significantly (p < 0.01) higher than that of leiomyomas (0.29%) and control uterine myometrium (0.13%). We conclude, that TGF-alpha and its receptor, EGF-R, are expressed more intensely in uterine leiomyosarcomas, compared to leiomyomas in B6C3F1 mice. Immunoexpression of TGF-alpha may be an important biomarker of malignancy in uterine smooth muscle tumors in mice. Futhermore, TGF-alpha may play a critical role in increased proliferation of uterine smooth muscle tumor cells as suggested by increased immunolocalization of PCNA in rodent leiomyosarcomas expressing TGF-alpha, although other factors regulating cell replication can not be ruled out.

Animals↗
PubMed2006

Primary left atrial leiomyosarcoma: literature review and lessons of a case.

Primary cardiac sarcoma is an uncommon disease. In particular, leiomyosarcoma of cardiovascular origin is extremely rare. Half of all cardiac leiomyosarcomas are located in the left atrium. Due to the extreme rarity of left atrial leiomyosarcoma, there is no great experience in its management. This review includes a report of a case of left atrial leiomyosarcoma followed up over 45 months. The literature review examines the distribution of left atrial leiomyosarcoma, the physiological reasons for the tendency of cardiac leiomyosarcoma to be localized to the left atrial cavity, the clinical and physical appearance of this disease, and the key differences between left atrial leiomyosarcoma and the most common left atrial tumor, myxoma. The morphological features, using light and electron microscopy and immunochemical staining, are discussed. Treatment modalities including adjuvant therapy and surgical resection are examined and their effectiveness compared. Opinions regarding the results and optimal treatment of leiomyosarcoma are not always in agreement. This highlights the need for inter-hospital comparison to determine the optimal treatment regimen.

Adult↗
PubMed2001

Expression of angiogenic factors and apoptotic factors in leiomyosarcoma and leiomyoma.

Angiogenesis is essential for tumor growth and metastasis. Some angiogenic factors, such as vascular endothelial growth factor (VEGF), platelet-derived endothelial cell growth factor (PD-ECGF), transforming growth factor-alpha (TGF-alpha) and basic fibroblast growth factor (bFGF) are involved in increased angiogenic activity and disease progression in many carcinomas. However, there is little information regarding the association between angiogenic factors and leiomyosarcoma. Although there are abundant vessels in the sarcoma which enable it to easily receive nutrition and medicinal components, chemotherapy cannot effectively treat leiomyosarcoma. This means the resistance to anticancer drugs in leiomyosarcoma is very strong. However, the resistant mechanism is still unclear. In this study, expressions of VEGF, PD-ECGF, TGF-alpha, bFGF, intratumoral microvessel density (IMVD), and p53, Bcl-2 and Bax were examined by immunohistochemistry in 30 patients with leiomyosarcoma and 21 patients with leiomyoma. With regard to angiogenesis, PD-ECGF and TGF-alpha were closely associated with an increase in IMVD (p=0.012, 0.0196, respectively), and VEGF and PD-ECGF were significantly expressed in leiomyosarcoma compared with leiomyoma (p=0.041, 0.041, respectively). Although p53 expression in leiomyosarcoma was significantly higher than in leiomyoma (p=0.016), the frequency of p53 positivity was not so high (47%). On the other hand, the ratio of Bcl-2/Bax in leiomyosarcoma was significantly higher than that in leiomyoma (p=0.033). The findings of this study suggest that in leiomyosarcoma, angiogenic factors, such as PD-ECGF, VEGF and TGF-alpha expression may be involved in tumor angiogenesis, and the frequently high ratio of Bcl-2/Bax and expression of p53 gene mutation might be related to chemoresistance mechanism.

Angiogenesis Inducing Agents↗
PubMed2006

Potential role of preoperative serum CA125 for the differential diagnosis between uterine leiomyoma and uterine leiomyosarcoma.

PURPOSE: Most comparisons between uterine leiomyoma and uterine leiomyosarcoma have been based on postoperative pathological or molecular analyses. Very few reports have investigated preoperative differentiation between uterine leiomyoma and uterine leiomyosarcoma. METHODS: Between January 1990 and December 2003, 42 consecutive patients with uterine leiomyosarcoma treated at index hospitals were analyzed. Meanwhile, 84 patients with uterine leiomyomas were used as controls. The diagnostic performance of preoperative serum CA125 for the differential diagnosis between uterine leiomyoma and uterine leiomyosarcoma using receiver operating characteristic (ROC) curves was evaluated. Data presentations were categorized into premenopausal and postmenopausal groups. Diagnostic efficiency was calculated as the sensitivity multiplied by the specificity. RESULTS: Values of preoperative serum CA125 were significantly higher in the uterine leiomyosarcoma group than those in the uterine leiomyoma group. There was significant overlapping of preoperative serum CA125 between the uterine leiomyoma group and early-stage uterine leiomyosarcoma. For both the premenopausal and postmenopausal group, there was a significant difference in the distribution of preoperative serum CA125 in early-stage and advanced-stage uterine leiomyosarcoma. The optimal cutoff values of serum CA125 for the premenopausal group and postmenopausal group was 162 U/mL and 75 U/mL, respectively. CONCLUSION: These findings demonstrated that preoperative serum CA125 had a potential role in the differential diagnosis between early-stage and advanced-stage uterine leiomyosarcoma. Further investigation with a larger sample size at adequate power is necessary to verify the current study.

Adult↗
PubMed1974

Cutaneous and subcutaneous leiomyosarcoma. A clinicopathologic study of 47 patients.

A retrospective study of 47 patients with leiomyosarcoma of superficial (skin and subcutaneous) soft tissue is presented. The criteria for the light microscopic diagnosis are given and the differential diagnoses are discussed. Forty patients had a solitary tumour which, in 19 patients, was situated entirely or almost entirely in the corium. There were two distinct growth patterns for the leiomyosarcomas. The cutaneous tumours were poorly delimited and the subcutaneous tumours more well-circumscribed or nodular. Some three quarters of the tumours were located in the extremities, the thigh and hip regions being the predilection sites; the highest frequency was noted in patients in their seventies; the ages ranged between 25 and 89 years. The sex ratio (male to female) was 2 to 1. In 37 patients follow-up information was available, the follow-up period ranging from 1 month to 16 1/2 years; the median time was 6 years. One or more local recurrences developed in 15 patients. Seven out of the 47 patients had multiple leiomyosarcomas; 4 of these patients had already been operated on for a retroperitoneal leiomyosarcoma. Fourteen patients in the whole series died with metastases especially in the lungs. Metastases were seen particularly in patients with subcutaneous and multiple leiomyosarcomas. Our study suggest that the size and the mitotic activity of the tumour appear to have some prognostic value. The initial surgical procedure was found to be the most important factor in influencing the outcome of the disease and it is stressed that leiomyosarcoma in superficial soft tissues should be treated by wide surgical excision. It is recommended that patients with multiple leiomyosarcomas in the superficial soft tissues should be subjected to further examination in order to exclude the possible occurrence of a retroperitoneal tumour. Finally, we consider that the use of a trichrome stain, such as the haematoxylin-van Gieson's stain, is superior to the haematoxylin-eosin stain in diagnosing leiomyosarcoma.

Adult↗
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↗
PubMed1997

Analysis of genetic alterations in uterine leiomyomas and leiomyosarcomas by comparative genomic hybridization.

Uterine leiomyomas are the most prevalent tumor type in women of reproductive age and are the most common reason for hysterectomies. Although uterine leiomyomas are considered to be benign, they are a major public health concern for women. In contrast, leiomyosarcomas are rare but highly malignant uterine tumors. They may arise in uteri with preexisting leiomyomas and histologically sometimes resemble leiomyomas, thus causing controversy about whether leiomyosarcomas arise within leiomyomas. In this study, we used comparative genomic hybridization (CGH) to identify genetic alterations unique to each tumor type and alterations that are common between the two tumors. We analyzed 14 cases of uterine leiomyomas and eight cases of uterine leiomyosarcomas. Only two of the 14 leiomyomas exhibited genetic alterations, and those were restricted to gains on chromosomes 14 and 19 and losses on chromosomes 1 and 4. In addition, 68 leiomyomas were examined for loss of heterozygosity on chromosomes 1 and 4, and only three tumors exhibited any losses. In contrast, all eight leiomyosarcomas showed gains and losses of DNA by CGH, and in many cases multiple changes were observed. The most commonly observed genetic aberration, occurring in five tumors, was gains on both arms of chromosome 1, suggesting that this chromosome contains loci involved in the development of leiomyosarcoma. Our results do not provide evidence for the progression from benign leiomyoma to malignant leiomyosarcoma. Moreover, the large number of random chromosomal alterations in the leiomyosarcomas suggests that increased genetic instability plays a role in the formation of these tumors.

Chromosome Aberrations↗
PubMed1997

Uterine leiomyosarcoma has deregulated cell proliferation, but not increased microvessel density compared with uterine leiomyoma.

OBJECTIVE: To investigate the differences of biological aggressiveness in terms of proliferating cell nuclear antigen (PCNA) expression, cell proliferation, and microvessel density between uterine leiomyosarcoma and leiomyoma. STUDY DESIGN: All patients with uterine leiomyosarcoma undergoing surgery at National Cheng Kung University Hospital were eligible. Forty-four patients with uterine myoma were also studied as the benign counterpart. The paraffin-embedded slides were stained with hematoxylin and eosin to confirm the presence of tumor and to quantitate mitoses, PC 10 for measurement of PCNA expression, MIB 1 for measurement of cell proliferation, and factor VIII for quantitation of microvessel density. The immunohistochemical findings of the slides were correlated with clinocopathologic findings of the patients, and the data were analyzed by either chi2 or unpaired t test. RESULTS: Six patients with uterine leiomyosarcoma and 44 patients with uterine leiomyoma were studied. Statistically significant higher mean levels of PCNA and MIB 1 were observed in uterine leiomyosarcoma compared with those of uterine myoma (for PCNA expression, P = 0.0001; for MIB 1, 11.61 +/- 11.42% vs 0.45 +/- 0.21%, P < 0.0001). No significant difference of microvessel density was observed between these two groups (65.73 +/- 48.62 vs 41.97 +/- 28.20, P = 0.084). Among the six patients with leiomyosarcoma, two patients with a higher percentage of MIB 1-positive tumor cells died of recurrent disease. In contrast, two patients with lower MIB 1 counts were disease-free for 3 years or more. CONCLUSION: Deregulated cell growth in uterine leiomyosarcoma may account for the biological aggressiveness of this tumor. Furthermore, the percentage of MIB 1-positive tumor cells seems to be associated with the prognosis or extent of uterine leiomyosarcoma.

Adult↗
PubMed2007

[Subcutaneous leiomyosarcoma metastatic to the eyelid].

BACKGROUND: Metastatic eyelid lesions are rare. Most metastatic lesions to the eyelid are carcinomas. Leiomyosarcomas are soft tissue sarcomas arising most commonly in the uterus and gastrointestinal tract, whereas dermal leiomyosarcomas are less frequent. Dermal leiomyosarcomas metastatic to the eyelid have not been reported so far. CASE REPORT: A 28-year-old female patient presented with a rapidly growing tumor of her right lower eyelid. A subcutaneous leiomyosarcoma of the neck had been excised 21 months before with subsequent radiotherapy. Seven months later metastases to the lung, liver, and left upper arm were detected. The patient received chemotherapy. The eyelid tumor was completely excised. Histologically the lesion consisted of spindle cells arranged in fascicles with perinuclear vacuoles and myofilaments. There was a strong immunoreactivity for smooth muscle actin, and negative staining for cytokeratin and S100. CONCLUSION: Cutaneous and subcutaneous leiomyosarcomas occur at almost any age, but are most common between the 5th and 7th decades. They are more common in men and usually occur at the extremities. The prognosis of cutaneous/subcutaneous leiomyosarcoma correlates with the depth of the tumor. To our knowledge this is the first presentation of a subcutaneous leiomyosarcoma metastatic to the eyelid. Presumably, parenchymatous metastases of this tumor occur before dermal metastases arise.

Adult↗
PubMed1990

Leiomyosarcoma in a series of hysterectomies performed for presumed uterine leiomyomas.

The incidence of leiomyosarcoma in uterine leiomyomas is estimated to be between 0.13 to 0.29%. However, the exact incidence of leiomyosarcoma in uteri removed with a preoperative diagnosis of benign uterine leiomyomas has not been previously reported. Between 1983 and 1988, a total of 1432 patients in Women's Hospital, a self-referred indigent population, had a hysterectomy planned because of abnormal uterine bleeding or abdominal pain associated with the presence of uterine leiomyomas, or because of a pelvic mass thought to be uterine leiomyoma of sufficient size or character to warrant surgical exploration. The ages of these women ranged from 36 to 62 years and the presence of leiomyosarcoma in the hysterectomy specimens increased steadily from the fourth to seventh decades of age (0.2%, 0.9%, 1.4%, and 1.7%, respectively). Preoperative histologic examination of the endometrium was performed in eight patients. Three of the eight patients had a preoperative tissue diagnosis of leiomyosarcoma that was clinically confined to the uterus. After the hysterectomy in the 1429 patients with presumed benign disease, histologic diagnosis of leiomyosarcoma was made in seven (0.49%). There was no evidence of malignancy in the endometrial sampling of any of these seven patients and the diagnosis was suspected intraoperatively in only three. Preoperative uterine size ranged from 8 to 20 weeks' gestational size and postoperative uterine weight ranged from 120 to 1100 gm. Seven of the 10 patients had symptoms of abnormal uterine bleeding. Between the ages of 40 and 60 years, 1% (8 of 817) of women with presumed uterine leiomyomas producing symptoms that necessitated hysterectomy in this series had leiomyosarcoma diagnosis postoperatively. Such treatments as gonadotropin-releasing hormone agonists, endometrial ablation, myomectomy by hysteroscopy or laparotomy instead of hysterectomy in such women could delay the diagnosis and definitive treatment of leiomyosarcoma.

Adult↗
PubMed1983

Leiomyosarcoma of the gastro-intestinal tract: general pattern of metastasis and recurrence.

Clinically, epithelial cancers (squamous cell carcinoma and adenocarcinomas) metastasize primarily by the lymphatic route, while mesenchymal sarcomas more frequently enter the blood stream directly. Nevertheless metastatic involvement of regional nodes has been seen in 13% of patients with soft tissue sarcomas and in 7% of bone sarcomas at initial presentation (29). About one-third of the leiomyosarcomas of the gastro-intestinal tract developed metastasis and about 90% of the metastasis were intra-abdominal (45). Liver is the most common site of metastasis, followed by peritoneal seeding and local recurrence or extension. Regional nodal metastasis also has been noted both initially and subsequently. The most important criterion of diagnosing leiomyosarcoma appears to be the presence of mitosis. Leiomyosarcoma with 1 mitosis per 2 high power fields or epithelioid leiomyosarcoma with 1 mitosis per 5 fields behaved aggressively. Additional criteria useful for diagnosing and for determining prognosis of malignancy include the presence of necrosis, cellular atypia, and the gross size of the tumor. In general, almost all tumors larger than 5 cm in diameter should be viewed with suspicion. For patients with gastric leiomyosarcomas, a proportion, sometimes as high as 14%, had perigastric lymph node involvement at diagnosis (9), and about 7% of those who relapsed had regional nodal metastasis (Table 2). For patients with leiomyosarcoma of the small intestine, about 5% of the patients had nodal metastasis initially, and 9% subsequently (Table 4). Regional lymphatic metastasis also has been reported in leiomyosarcoma of the colorectum. It is believed that wide resection of normal tissues (at least 10 cm margin on either side of tumor) and adjacent mesentery (with routine omentectomy for gastric lesions) not only may discover a higher frequency of occult metastasis in the regional lymph node, but will also decrease the chance of loco-regional relapse and the occurrence of sarcomatosis. As a result of this review and a previous one (30), the surgeon ought to consider when faced with a smooth muscle tumor of the gastro-intestinal tract a wide segmental resection including the adjacent mesentery and omentum, because (a) the precise histologic nature of the lesion (benign vs. malignant) is hard to define even by permanent sections; (b) the possibility of involvement of draining nodes exists and (c) little or no increase in morbidity is anticipated between a wide, as against a limited wedge or segmental resection of gastrointestinal tract. The last point does not need elaboration, except for the very unusual lesions of the duodenum, distal stomach or lower rectum. When adherence to this principle would require a pancreaticoduodenectomy, near total gastrectomy or abdominoperineal resection, it may be wise to establish a definitive histologic diagnosis of the primary lesion first, and to use a more conservative procedure initially.

Colonic Neoplasms↗
PubMed1995

Hepatic leiomyosarcomas: CT features with pathologic correlation.

The purpose of this study was to determine the computed tomographic (CT) features of hepatic leiomyosarcoma and to correlate them with pathologic findings. CT examinations of 15 patients with pathologically proven leiomyosarcoma of the liver were retrospectively reviewed. Three patients had primary leiomyosarcoma of the liver and 12 patients had 43 distinct hepatic metastases originating from a primary gastrointestinal (n = 8), uterine (n = 2) or retroperitoneal (n = 2) leiomyosarcoma. In the 11 patients who had partial hepatic resection or surgical biopsy of their tumors, a correlation was made between CT imaging and pathologic findings. Primary leiomyosarcomas showed heterogeneous enhancement; two displayed internal and peripheral enhancement, and one showed peripheral enhancement with a pseudocystic pattern (i.e. enhancing thick wall with internal nonenhancing low attenuation area). Leiomyosarcomas metastatic to the liver were homogeneous, showing no or moderate enhancement (10 of 43 metastases, 23%) or heterogeneous and predominantly peripheral enhancement (33 of 43 metastases, 77%). A pseudocystic pattern was seen in 13 of the 43 metastases (30%). Homogeneous tumors were made of smooth fibrous-like tissue without area of necrosis. Heterogeneous tumors contained varying degrees of necrosis and hemorrhage or gelatinous tissue. We found that primary and secondary hepatic leiomyosarcomas of the liver can exhibit different features. A pseudocystic pattern is uncommon. Furthermore, purely cystic tumors were not seen in our series.

Adult↗
PubMed2000

Rhabdoid features in leiomyosarcoma of soft tissue: with special reference to aggressive behavior.

The presence of rhabdoid cells has been reported in various types of malignant neoplasms and has been determined to be a predictor of aggressive behavior of neoplasms regardless of tumor histogenesis. One hundred and thirteen cases of leiomyosarcoma, selected from 1800 soft tissue sarcomas, were reviewed on hematoxylin and eosin sections, and immunohistochemical staining when available, and seven cases with rhabdoid features were retrieved. Clinicopathologic differences were analyzed to compare between cases with rhabdoid features and those without rhabdoid features. In the seven cases with rhabdoid features, two were intra-abdominal, and the others arose in external soft tissues including muscle, subcutis, and cutis. Patient age ranged from 33 to 84 years, three were female, and four were male. Tumor size ranged from 3 to 22 cm. Clinical evidence showed no differences from those cases without rhabdoid features. Histologically, one of the abdominal cases was epithelioid leiomyosarcoma. Two of the 7 cases were better subclassified as pleomorphic leiomyosarcoma, in which rhabdoid cells are diffusely scattered. In cases other than those with pleomorphic leiomyosarcomas, foci of anaplastic areas were observed, and collections of rhabdoid cells were present in those areas. Immunohistochemical examination of the cases confirmed myogenic differentiation, and showed rhabdoid cells being positive for vimentin and desmin in the inclusion bodies, and diffusely so for muscle actin in the cytoplasm. After dividing all the cases of leiomyosarcoma by their location, prognostic analysis was performed. Leiomyosarcoma of external soft tissue with rhabdoid cells showed a tendency for poorer prognoses than cases without rhabdoid features. On the contrary, retroperitoneal cases did not. This study indicates that rhabdoid features are associated with aggressive biological behavior in leiomyosarcoma of the external soft tissue.

Adult↗
PubMed1999

Subcutaneous leiomyosarcoma on the face.

BACKGROUND: Cutaneous leiomyosarcoma is a rare soft tissue sarcoma with a predilection for the lower extremities. Leiomyosarcoma of the face is very rare. Subcutaneous leiomyosarcoma has a higher likelihood of recurrence and metastases than that of the superficial dermal type. OBJECTIVE: The dermatologic surgeon and pathologist should be familiar with the characters of subcutaneous leiomyosarcoma. METHODS: We report a case of subcutaneous leiomyosarcoma on the face with the results of histologic examination and immunohistochemical studies. RESULTS: Leiomyosarcoma of the face is exceedingly rare. The deep subcutaneous type is thought to arise from the smooth muscle of the vascular wall. The neoplasm we report here has deep tumor invasion, high malignancy grade (3B), and large tumor size. Wide excision and postoperative radiotherapy were performed. CONCLUSION: Subcutaneous leiomyosarcoma has a higher likelihood of recurrence and metastases than that of the superficial dermal type. The most effective treatment is wide excision with 3-5 cm lateral margins and a depth that includes subcutaneous tissue and fascia.

Facial Neoplasms↗
PubMed1999

Leiomyosarcoma of soft tissue in children: clinicopathologic analysis of 20 cases.

Leiomyosarcoma in the pediatric age group is uncommon and incompletely characterized. A series of 20 primary leiomyosarcomas of soft tissue occurring in children younger than 16 years is presented. No significant gender predilection was observed (11 girls and 9 boys). Patient age ranged from 4 to 15 years (median, 12 years). Tumor size ranged from 0.5 to 13 cm (median, 2.5 cm); subcutaneous and deep locations were equally represented. Tumors were evenly distributed among the trunk (30%), head and neck (25%), lower limbs (25%), and upper limbs (20%). All lesions showed at least focally typical features of smooth muscle differentiation, principally in the form of fascicles of eosinophilic spindle cells with cigar-shaped nuclei. An unusual whorled growth pattern was seen in two cases. Morphologic variants including inflammatory leiomyosarcoma (one case), granular cell leiomyosarcoma (two cases), giant-cell rich leiomyosarcoma (two cases), and epithelioid leiomyosarcoma (one case) were seen. Dystrophic calcifications were present in two cases. Most lesions (85%) were low grade. Immunohistochemical staining showed positivity for alpha-smooth muscle actin in 89% of the cases, HHF-35 in 87%, and desmin in 61%. Positivity for cytokeratins, observed in 6 (43%) of 14 cases tested, was usually strong and was diffuse in two cases. Follow-up data, available in 15 (75%) patients (median duration, 49 months), showed late local recurrence in only two cases, one with progression to a higher grade lesion, and no metastasis. These results show that, although extremely rare, soft-tissue leiomyosarcomas do occur in children, in whom they usually present as small morphologically low-grade lesions that seem to behave in a relatively indolent fashion, although longer follow-up data are needed. Differential diagnosis in this setting includes infantile myofibromatosis, leiomyoma, monophasic synovial sarcoma, and spindle cell rhabdomyosarcoma.

Adolescent↗
PubMed1999

Spinal manifestation of metastasizing leiomyosarcoma.

STUDY DESIGN: Case report. OBJECTIVE: To provide additional information on possible relations between uterine and spinal manifestations of leiomyosarcoma. SUMMARY OF BACKGROUND DATA: Spinal metastases and primary spinal manifestation of leiomyosarcoma and other malignant smooth muscle tumors are rarely observed. METHODS: Clinical and radiologic follow-up of a patient with a spinal tumor. RESULTS: A 46-year-old women had rapidly progressive paraplegia caused by an extramedullary lesion in the extradural space at T2-T3, with spinal cord compression. After surgical decompression, the resected tumor was histologically classified as a leiomyosarcoma. Diagnostic work-up failed to detect the primary tumor site. Previous medical history had been uneventful except for hysterectomy 3 years earlier for a leiomyoma. Extended necroses and focal metaplasia were already described in the hysterectomy specimen indicating malignant disease; however, it was not definitely diagnosed. Multiple leiomyosarcoma metastases developed 22 months later. CONCLUSIONS: In retrospect, the origin of the leiomyosarcoma that manifested in the thoracic spine of the patient must be reconsidered. Rather than a primary dural leiomyosarcoma, this tumor represented the first evidence of recurrence of a missed diagnosis of early-stage uterine leiomyosarcoma.

Biomarkers, Tumor↗
PubMed2002

Leiomyosarcoma of the lumbar spine: case report.

STUDY DESIGN: A primary leiomyosarcoma in the L2 vertebral body without any neurologic deficit is reported. The radiologic diagnosis was a metastasis, and the histologic diagnosis was confirmed by transpedicular needle biopsy. OBJECTIVE: To emphasize the occurrence of primary leiomyosarcoma in the lumbar spine. SUMMARY OF BACKGROUND DATA: Leiomyosarcoma is a rare malignant neoplasm of the bone, and only one report of the primary spinal tumor exists. In the case of leiomyosarcoma of the bone, metastasis, most commonly of the uterus or gastrointestinal tract, must be carefully ruled out before the diagnosis of primary leiomyosarcoma in the bone is confirmed. METHODS: Leiomyosarcoma of the second lumbar vertebra in a 47-year-old woman with no neurologic deficit is reported. Imaging findings indicated a nonspecific high-grade lesion. A total spondylectomy of L2 with en bloc excision of the lower half of L1 and the upper third of L3 vertebral bodies was performed after preoperative radiation therapy. The defect of the vertebral body was reconstructed by titanium mesh cage, and T11 to S1 vertebrae were instrumented by the pedicle screw and rod system. RESULTS: The surgical margin was marginal. Histologically, the tumor cells were elongated, with abundant cytoplasm and cigar-shaped nuclei, showing proliferation in fascicles and bundles. No sign of local recurrence or metastasis was evident 2 years and 1 month after surgery. CONCLUSIONS: Leiomyosarcoma should be included in the differential diagnosis of spindle cell tumor of the spine, and it should be confirmed or excluded by immunohistochemical and/or ultrastructural studies. If the expected surgical margin is definitive, a total en bloc spondylectomy should be performed.

Diagnosis, Differential↗