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Leiomyosarcoma: computed tomographic findings.

The computed tomographic (CT) findings in 118 patients with the diagnosis of leiomyosarcoma were reviewed. The tumor masses visualized in these patients were often quite large; extensive necrotic or cystic change was a frequent finding. Calcification was not observed in these tumors. The liver was the most common site of metastasis in these patients, with marked necrosis of the liver lesions a common finding. Other manifestations of tumor spread included pulmonary metastases, mesenteric or omental metastases, retroperitoneal lymphadenopathy, soft-tissue metastases, bone metastases, splenic metastases, and ascites. The metastatic lesions were often centrally necrotic. Although the CT appearance of leiomyosarcoma is not specific, these findings, when present, suggest consideration of this diagnosis.

Adolescent↗

Primary leiomyosarcoma of the gallbladder. Case report and review of the literature.

The case of a 64 year old female who was known to have gallstones is presented. She was admitted to the Hospital following an attack of acute cholecystitis. Ten days after vigorous conservative treatment cholecystectomy was performed. The histological examination showed the presence of the gallbladder leiomyosarcoma. Primary sarcomas of the gallbladder are rare, leiomyosarcoma being the most infrequent type, their preoperative diagnosis almost impossible and their prognosis poor.

Acute Disease↗

Gene therapy with E2F-1 up-regulates the protein kinase PKR and inhibits growth of leiomyosarcoma in vivo.

Overexpression of the transcription factor E2F-1 induces apoptosis in a variety of carcinoma cells and inactivates murine double minute protein 2, a factor associated with poor prognosis in soft tissue sarcomas. We have shown previously that the double-stranded RNA-activated protein kinase PKR plays an important role in mediating this apoptotic response in carcinoma cells to E2F-1. We sought to evaluate the potential of E2F-1 gene therapy in soft tissue sarcomas and to study the involvement of PKR in the response to E2F-1 overexpression in mesenchymal cells. A replication-deficient adenovirus carrying the E2F-1 gene (Ad5E2F) was used to induce E2F-1 overexpression in the p53 mutated leiomyosarcoma cell line, SKLMS-1. Western blot analysis confirmed E2F-1 overexpression and up-regulation of the antiapoptotic factor Bcl-2 48 hours following infection with Ad5E2F. Apoptosis in Ad5E2F-treated cells was confirmed by fluorescence-activated cell sorting analysis and by poly(ADP-ribose) polymerase cleavage and DNA fragmentation assays. Vector-dependent up-regulation of PKR correlated with the amount of Ad5E2F-induced apoptosis. In vivo treatment of SKLMS-1 tumor-bearing BALB/c mice with intratumoral injections of Ad5E2F at a dose of 2 x 10(10) viral particles resulted in significant inhibition in tumor growth compared with control-treated animals (P < 0.016). Complete disappearance of all tumors was seen in two of seven mice in the Ad5E2F-treated animals. Immunohistochemical analysis of tumor specimens showed overexpression of E2F-1 and up-regulation of PKR in Ad5E2F-treated tumors. These findings show that adenovirus-mediated overexpression of E2F-1 results in up-regulation of PKR and significant growth suppression of leiomyosarcomas in vivo. Taken together, these data suggest that E2F-1 gene therapy and PKR modulation might be a promising treatment strategy for these tumors that are highly resistant to conventional therapies.

Adenoviridae↗

Intestinal leiomyosarcoma and gastroparesis associated with von Recklinghausen's disease.

The rare association between intestinal leiomyosarcoma, von Recklinghausen's disease (type-1 neurofibromatosis) and gastroparesis is described. A 20-year-old male, diagnosed 12 years earlier as having pelvic von Recklinghausen's disease, presented with nausea and vomiting. A gastric scintigraphy demonstrated an extremely slow gastric emptying time in the absence of obvious causes for gastroparesis. A small ileal leiomyosarcoma was later found and removed by surgery. The latter was followed by a marked improvement in the clinical condition of the patient.

Adult↗

Cystic leiomyosarcoma of the kidney: an unusual clinical presentation.

Primary sarcomas of the kidney are rare, accounting for 1-3% of all renal malignancies. We describe an unusual case of renal leiomyosarcoma in a 41-year-old white woman who presented with a large smooth mass, which was mobile to the overlying structures and which occupied the right hypochondria and flank. Radical nephrectomy was carried out and the patient is well, without symptoms of relapse, 1 year after surgery. Leiomyosarcomas of the kidney have an aggressive and rapidly progressive natural history, with 5-year survival rates of 29-36%. Size <5 cm, low histological grade, absence of lymph node metastases and radical surgical treatment are all associated with a better prognosis. Irradiation and chemotherapy do not appear to alter the clinical course.

Adult↗

Leiomyosarcoma of the bladder in a retinoblastoma patient.

We report a rare case of leiomyosarcoma in the bladder which occurred in an 18-year-old female with a prior history of retinoblastoma (RB). Molecular characterization of this tumor displayed a homozygous RB deletion and a reduced P53 expression. These results suggest that the loss of RB and P53 may have contributed to the initiation and/or progression of the leiomyosarcoma of the bladder in this patient.

Adolescent↗

Simultaneous lung and liver resection for primary pulmonary leiomyosarcoma.

We report on a patient with primary pulmonary leiomyosarcoma and hepatic metastasis who was treated successfully with a single-stage surgical operation. As a cure for primary pulmonary leiomyosarcoma has only been achieved by surgical resection, primary radical treatment should be considered, even in advanced cases having distant metastases which are expected to be resectable.

Humans↗

Sonographic findings of uterine leiomyosarcoma.

We describe herein various sonographic features of uterine leiomyosarcoma. Transabdominal scanning (TAS) revealed an image indistinguishable from benign leiomyoma, with evidence of degeneration. Transvaginal scanning (TVS) clearly showed the thinness of the myometrium, and the possibility of deep myometrial invasion was suspected at intraoperative open direct ultrasonography (ODU). A very high peak systolic velocity and a slightly increased diastolic component at the periphery of the tumor were evident with pulsed Doppler ultrasound. Thus, TAS, TVS, ODU and Doppler ultrasound will yield useful information for the physician attempting to evaluate the extent and vascularity of uterine leiomyosarcoma.

Female↗

Leiomyosarcoma of the trachea. Case report.

Leiomyosarcoma of the trachea is a rare primary tumor of the upper airway. Twelve cases have been reported in the English-language literature. Our experience with a patient with leiomyosarcoma of the cervical trachea is presented. The classic symptoms, diagnosis, and surgical management of this case are contrasted with those of the previously reported cases. The surgical technique used for our patient, primary tumor resection with skin grafting and placement of a Montgomery Laryngeal Stent, is a unique and effective treatment.

Adult↗

Leiomyosarcoma of the larynx. Case report.

Leiomyosarcoma involving the larynx is extremely rare and may be difficult to diagnose. Likewise, because of the rarity of this lesion, little information exists with regard to long-term follow-up or optimal management. Reported here is a review of the literature and a case report of a patient with leiomyosarcoma of the larynx treated by surgery alone with 5 years of follow-up.

Aged↗

Myxoid leiomyosarcoma of the uterus--a case report with light microscopic and ultrastructural appraisal.

A case of an infiltrating myxopolypoid tumor of the uterus is presented. Histologically, the tumor revealed spindle-shaped cells with mitotic counts ranging between 1-4/hpf, myxoid stroma, and multinucleated giant cells mimicking syncitial giant cells of pregnancy trophoblast. Conventional light microscopic methods failed to establish the histogenesis of the tumor. At the ultrastructural level the tumor showed three main cellular components; myoblasts, myofibroblasts and collagen-producing fibroblasts. On the basis of light microscopic and electron microscopic findings, the tumor was labelled myxoid leiomyosarcoma. This variant of leiomyosarcoma is rare. This is the seventh case reported in the international literature.

Female↗

Leiomyosarcoma of the small bowel.

Two cases of leiomyosarcoma of the small bowel are described. Both patients presented a large mass adherent to the intestinal wall, with a central necrotic area communicating with the intestinal lumen. Microscopically, the lesions were identified as grade III leiomyosarcomas.

Aged↗

Leiomyosarcoma of the soft tissues in a patient with nevoid basal-cell carcinoma syndrome.

A 57-year-old man was admitted with complaints of progressive anorexia, weight loss and right flank pain. He had been treated for basal-cell carcinoma of the skin 19 years before. On physical examination, eight moles in the face, back and left thigh were found along with palmar pits. In addition, a painful induration in his right thigh was evident. Biopsy proved that six moles were basal-cell carcinomas and the thigh mass a high-grade leiomyosarcoma. Computed tomographs revealed multiple metastases in the lungs and the liver. The patient was treated with epirubicin, with partial response, and subsequently with ifosfamide. He died 17 months after diagnosis. Whereas the world literature records several cases of soft tissue tumors in patients with nevoid basal-cell carcinoma syndrome, this is the first report of a simultaneous occurrence of leiomyosarcoma and nevoid basal-cell carcinoma syndrome.

Basal Cell Nevus Syndrome↗

Rhabdoid epithelioid leiomyosarcoma of the uterine corpus: a case report and literature review.

A case of epithelioid leiomyosarcoma of the uterus with rhabdoid phenotype and early rhabdomyoblastic differentiation is described. A 72-year-old woman with a 5-week history of increased abdominal girth was found to have a large pelvic mass. The uterus revealed a large intramyometrial and left adnexal necrotic tumor that had spread to the small bowel mesentery and to the anterior abdominal peritoneum. The tumor was an epithelioid leiomyosarcoma with rhabdoid phenotype and focal early rhabdomyoblastic differentiation, as confirmed by immunohistochemical and ultrastructural techniques. Also called composite extrarenal rhabdoid tumor (CERT), this lesion should be differentiated from malignant mixed müllerian tumor, rhabdomyosarcoma, endometrial stromal sarcoma, and pure rhabdoid tumors of the uterus. The recognition of a rhabdoid phenotype is of clinical importance since these tumors are prone to be aggressive.

Aged↗

Primary cutaneous leiomyosarcoma: a clinicopathological and immunohistochemical study of 7 cases.

Primary cutaneous leiomyosarcomas are rare tumors, few series being reported in the current literature. A retrospective study of 7 cases was undertaken to understand the clinicopathological characteristics of these neoplasms and some of their molecular mutations. Histologically, a well-differentiated proliferation of cells of smooth muscle derivation was evident in all cases. The number of mitoses was considered the most important criterion of malignancy (more than 2 for 10 HPF). Smooth muscle actin, desmin, and vimentin were positive in all cases. Immunohistochemical analysis also revealed a positivity for p53 in 3 cases and no reaction for retinoblastoma protein. Research for Epstein-Barr virus was negative in all cases. Three patients developed local recurrences owing to incomplete surgical excision. Recurrent tumors were more atypical and located deeper. No distant metastases were observed. Our results emphasize that cutaneous leiomyosarcomas have an indolent biological course if treated by surgical excision with wide margins. Molecular abnormalities involving tumor suppressor genes are probably involved.

Aged↗

C-kit expression in uterine leiomyosarcomas: an immunocytochemical study of 29 cases of malignant smooth muscle tumors of the uterus.

Uterine malignant stromal tumors are rare neoplasms characterized by fatal prognosis. At the moment no effective systemic treatment is available for metastases or recurrent disease. The drugs employed in advanced neoplasms are iposfamide, doxorubicin or epidoxorubicin, but the clinical response to chemotherapy is poor. Recent studies have shown that cells in gastrointestinal stromal tumors express a growth factor receptor with tyrosine kinase activity termed c-kit. Lately reports of efficacy of a specific anticancer drug with imatinib (ST1571) based on specific molecular abnormalities of proto-oncogene c-kit present in gastrointestinal stromal tumors induced us to identify the c-kit phenotype also in uterine leiomyosarcomas. These data may be useful for treating metastatic uterine leiomyosarcomas with increased c-kit kinase activity.

Adult↗