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Primary leiomyosarcoma of the greater omentum: CT findings.

Three cases of primary leiomyosarcoma arising from the greater omentum are described. On CT the tumors were multilobulated, flat, and pancake-like, with enhancing solid and multicystic densities. The masses in all cases were located in the most anterior part of peritoneal cavity. These CT findings are useful in the preoperative diagnosis of omental leiomyosarcoma.

Adult↗

Vaginal metastasis from uterine leiomyosarcoma. Magnetic resonance imaging features with pathological correlation.

Vaginal metastases, though more frequent than primary vaginal tumors, are rare. Except for isolated reports of metastatic disease arising from extragenital organs, the vagina is typically a site for metastatic disease from genitourinary tract sites, especially cervix, endometrium, and kidney. We present herein a case of a 68-year-old woman presenting with a vaginal metastasis as the first manifestation of a uterine leiomyosarcoma. The magnetic resonance imaging features are described and correlated with the pathological findings. To the best of our knowledge, this is the first case of vaginal metastasis from uterine leiomyosarcoma described in the literature.

Aged↗

Radiologic findings in primary pulmonary leiomyosarcoma.

Leiomyosarcomas are uncommon smooth muscle tumors most commonly encountered in the female genital organs, gastrointestinal tract, and soft tissues. They are rarely encountered as primary pulmonary neoplasms. Herein we describe the conventional radiographic and computed tomographic findings in a patient with a pulmonary leiomyosarcoma.

Adult↗

Cutaneous leiomyosarcoma.

Cutaneous leiomyosarcoma is a rare soft-tissue sarcoma with negligible metastatic potential, but local recurrence rates after surgical excision have ranged from 14 percent to 42 percent. Unlike other sarcomas, guidelines for the optimal surgical excision margin of cutaneous leiomyosarcoma are not clearly defined in the existing literature. A review of local experience with this condition revealed eight patients over 12 years, none of whom developed local recurrence or distant metastases. This is despite poor prognostic factors in seven patients and excision margins ranging from 1 to 27 mm. These findings are compared with previously published data, and conclusions are drawn based on analysis of the collective results. Complete surgical excision with a narrow margin is recommended, and patients should be observed for a minimum of 5 years after surgery.

Adult↗

Leiomyosarcoma of the Cervix Uteri.

We have described a 38-year-old woman with a lesion of the cervix found on routine physical examination. The lesion proved to be a primary leiomyosarcoma of the cervix. Despite radiation therapy, surgery, and chemotherapy, the tumor recurred and grew relentlessly. She is alive with disease at two years seven months after initiation of therapy. The biologic behavior of leiomyosarcoma appears to be quite different from that of squamous cell carcinoma of the cervix.

Adult↗

Exogastric leiomyosarcoma with ultrastructural analysis.

A 50-year-old man was found to have a large exogastric leiomyosarcoma during exploratory laparotomy for a bleeding duodenal ulcer. Although the tumor appeared grossly malignant and was associated with small bowel metastases, its light microscopic appearance was relatively benign. Electron microscopy helped to established the diagnosis of gastric leiomyosarcoma.

Duodenal Ulcer↗

Abdominal leiomyosarcoma: aggressive management.

We retrospectively reviewed 34 cases of intra-abdominal leiomyosarcoma treated between 1967 and 1986. Thirty-three patients had operation, and the primary tumor was removed in all but one. Fifteen patients had peritoneal implants at initial surgical exploration; 14 had tumor recurrence and had at least one reoperation. Five-year survival was 16% overall and 37% for patients without peritoneal implants at the initial procedure. This figure includes five patients in whom distant metastases developed after benign gastric or intestinal leiomyoma was diagnosed based on histologic and gross findings. All patients who have had five-year cures or more than two years of palliation after demonstration of peritoneal metastases had operation after 1978, a finding that probably reflects a more aggressive surgical approach to these tumors. Effective palliation, defined as three months of symptom relief and six-month survival in patients with widespread intraperitoneal tumor, was achieved significantly more often when the tumor causing the symptom was resected (eight of eight patients) than when it was not (five of six patients). These data indicate that an aggressive surgical approach is warranted in attempts to achieve either cure or palliation in patients with intra-abdominal leiomyosarcoma.

Actuarial Analysis↗

Resection of primary hepatic malignant fibrous histiocytoma, fibrosarcoma, and leiomyosarcoma.

Four patients had resection for primary hepatic sarcoma: one with malignant fibrous histiocytoma (MFH), two with poorly differentiated fibrosarcoma, and one with leiomyosarcoma. Age ranged from 40 to 69 years. One patient had a cousin and a grandmother who had died of hepatic tumors. At presentation, all patients had pain; one had tumor rupture, and one had mental changes and hypoglycemia. None had hepatitis or cirrhosis. Results of laboratory evaluation were nonspecific, including normal carcinoembryonic antigen and alpha-fetoprotein levels. Computed tomography showed hypodense masses with enhancement. Angiography showed a hypervascular mass in three patients and an avascular mass in the patient with MFH. Despite large tumors (8 to 32 cm), portal and hepatic veins were not invaded. The pattern of vascularization and lack of venous invasion helps differentiate primary hepatic sarcomas from hepatocellular carcinoma, especially in noncirrhotic patients. All patients had extensive hepatic resections, with one operative death. Immunohistochemical stains of the tumors were positive for vimentin but negative for epithelial markers, differentiating these lesions from other hepatic tumors. The patient with MFH died with recurrence at 10 1/2 months. The patient with the ruptured fibrosarcoma had a second resection and chemotherapy, but died with recurrence at 3 years. The patient with the leiomyosarcoma had a second resection and was disease free at 4 years. Resection of primary hepatic sarcoma is warranted, with potential survival measured in years.

Adult↗

Primary meningeal Epstein-Barr virus-related leiomyosarcoma in a man infected with human immunodeficiency virus: review of literature, emphasizing the differential diagnosis and pathogenesis.

We describe the clinical, radiologic, surgical, and pathologic findings of a 29-year-old Peruvian human immunodeficiency virus-infected man with a primary parasellar meningeal leiomyosarcoma involving the left lesser esphenoidal wing and the cavernous sinus. Over a period of 13 months, he developed headache, vomiting, insomnia, and diplopia. Magnetic resonance imaging revealed a left parasellar extra-axial mass that was isointense in T1, hypointense in T2, and gadolinium-enhanced. The patient underwent subtotal resection of the tumor. The neoplasm was composed of spindle cells with smooth-muscle features. It showed moderate atypia, inconspicuous nucleoli, and scanty mitosis. No tumor necrosis was detected. The immunohistochemistry revealed strong positivity for vimentin, desmin, and smooth-muscle alpha-actin. A low-grade leiomyosarcoma was diagnosed. The in situ hybridization showed positive nuclear reactivity for Epstein-Barr virus-encoded RNA. The immunohistochemistry was negative for Epstein-Barr virus latent membrane protein 1. The main differential diagnosis of primary meningeal smooth-muscle tumors includes meningioma and peripheral nerve sheath tumors. Epstein-Barr virus has been demonstrated in most smooth-muscle tumors associated with acquired immune deficiency syndrome (AIDS). Primary meningeal smooth-muscle tumors, exceedingly rare neoplasms, remarkably affect young adults with AIDS. Comparatively, most AIDS-related visceral (nonmeningeal) smooth-muscle tumors have been reported in children. The permissiveness and tumorigenesis associated with Epstein-Barr virus may depend on the age of human immunodeficiency virus infection.

Adult↗

Imaging of metastatic orbital leiomyosarcoma.

A 74-year-old man with a previous history of lower leg soft tissues leiomyosarcoma and multiple metastasis presented with a progressive painless proptosis of the left eye. Orbital ultrasound, CT, and MRI revealed a large mass in the left medial orbit. The mass was surgically removed and pathologic examination confirmed the diagnosis of a metastatic orbital leiomyosarcoma. The imaging characteristics of this rare tumor are comprehensively detailed, using complementary ultrasound, CT, and MRI, the combination of which allowed planning of total excision of the lesion.

Aged↗

Sinonasal leiomyosarcoma: review of literature and case report.

OBJECTIVES/HYPOTHESIS: To determine prognosis of primary sinonasal leiomyosarcomas after treatment. STUDY DESIGN: Literature review and case report. METHODS: Review of English literature from MEDLINE and independent sources with the addition of our case. RESULTS: Including our case, 63 cases have been reported. Primary treatment includes resection with or without radiation. Chemotherapy has not been reported to be effective. In our case, however, chemotherapy, consisting of etoposide and high-dose ifosfamide, caused the tumor to shrink significantly. On the basis of a review of all reported cases, the overall survival rate at a mean follow-up of 38.24 month is 66%. The minimal overall survival rates at 5 and 10 years are 20% and 6%, respectively. CONCLUSION: The prognosis for primary sinonasal leiomyosarcomas is poor. However, a 10-year survival has been reported in a few patients. Chemotherapy may be a useful adjunct when managing extensive lesions unamenable to curative resection.

Adult↗

Myxoid leiomyosarcoma of the uterus in a patient receiving tamoxifen therapy: a case report.

In this paper, we present a case of myxoid leiomyosarcoma development in a patient receiving tamoxifen for 3 years because of breast cancer. The myxoid leiomyosarcoma should be included in the differential diagnosis of any uterine tumor with a predominantly myxomatous composition. A review of the literature indicates that tamoxifen may increase not only the risk for endometrial cancer but also for uterine sarcoma, suggesting vigilance for uterine cancer in women who are being treated with this drug.

Aged↗

A high-grade uterine leiomyosarcoma with human chorionic gonadotropin production.

Ectopic human chorionic gonadotropin production has been described in a wide variety of non-germ cell tumors, particularly in epithelial tumors, but rarely in sarcomas. In this report, we describe the case of 49-year-old woman with a history of "uterine fibroids," who presented with vaginal bleeding and a positive urine pregnancy test. After pregnancy was ruled out by ultrasound, the patient underwent a laparotomy and hysterectomy for a presumptive diagnosis of "fibroids" and was found to have carcinomatosis at the time of the surgery. Therefore optimal debulking of tumors was performed. Two weeks later, the patient developed a small bowel obstruction, which apparently was due to rapid recurrence of tumors in the abdomen, and soon afterwards she died. Microscopically, the resected pelvic mass was composed of highly atypical and pleomorphic spindle cells admixed with many multinucleated giant cells. The tumor had a high mitotic rate along with areas of hemorrhage and necrosis. Immunohistochemically, the tumor cells were positive for vimentin, desmin, smooth muscle actin, and beta-human chorionic gonadotropin, and were negative for epithelial membrane antigen, keratin AE1/3, S-100, CD31, CD117, Ber-EP4, WT-1, estrogen and progesterone receptors. The majority of cells, including the multinucleated giant cells, were strongly immunoreactive for beta-human chorionic gonadotropin. Only three cases of leiomyosarcomas with beta/human chorionic gonadotropin production have been described in the literature, and all three cases had extrauterine origin. Our case, to the authors' best knowledge, is the first uterine leiomyosarcoma with prominent beta/human chorionic gonadotropin production.

Actins↗

Leiomyosarcoma of the inferior vena cava: experience in 22 cases.

From 1979 to 2004, 22 patients were seen with leiomyosarcomas of the inferior vena cava (IVC). Twenty were treated surgically. Involvement of the IVC included the infrarenal segment in 3 cases, the suprarenal and/or retrohepatic segment in 13, and the suprahepatic segment in 4. Nineteen patients underwent wide tumor resection followed by ligation of the IVC in 5 cases, replacement with a PTFE prosthesis in 13, and cavoplasty in 1. An intracardiac tumor extension was resected during hypothermic circulatory arrest in 1 patient. Vascular exclusion of the liver was used in 5 cases and simple clamping of the IVC in 13 cases. There were 1 intraoperative death due to cardiac failure and 3 postoperative deaths due to multiple organ failure, liver failure, and duodenal fistula after treatment of a bleeding ulcer. Fifteen of the 16 surviving patients underwent adjuvant chemotherapy associated with radiation therapy in 4 cases. One patient was lost from follow-up at 10 months. Four patients including one with metastasis are still alive with a mean follow-up of 18.3 months. Eleven patients died after a mean follow-up period of 43.7 months due to local recurrence and/or distant metastasis in 9 cases and complications of chemotherapy in 2. The 3- and 5-year mean actuarial survival rates in patients who underwent resection were 52.0% and 34.8%, respectively. Leiomyosarcoma of the IVC is a serious disease. Although surgical resection combined with chemotherapy is usually not curative, it can achieve reasonably long-term survival. We recommend aggressive operative management using the latest vascular surgery and oncology techniques.

Adult↗

A case of superficial leiomyosarcoma, and a review of Japanese cases.

We report a case of leiomyosarcoma in a 55-year-old Japanese female. She developed an erythematous nodule, 1.5 cm in diameter, on her right thigh. The totally excised specimen contained tumor cells with variously sized, chromatin-rich nuclei. Gitter staining revealed the so-called encased picture, immunohistopathological staining was positive for smooth muscle actin, and electron microscopy revealed filaments with focal densities. A diagnosis of leiomyosarcoma was made based on these findings, which suggested a muscle cell origin. Normal arrector pili muscles were continuous from the tumor, further suggesting the tumor's origin. The presence of Epstein-Barr virus-encoded RNA (EBER) was examined by in situ hybridization with negative results.

Female↗

Retroperitoneal leiomyosarcoma: a light and electron microscopic study.

Retroperitoneal leiomyosarcoma arising in a spermatic blood vessel is described. In addition to typical leiomyosarcoma, areas of atypical histiocyte-like cells and storiform pattern bearing a close resemblance to malignant fibrous histiocytoma, were seen. Ultrastructurally, cells with features of smooth muscle, partially differentiated cells with marginal densities and basal lamina and histiocyte-like giant cells were present. Phagocytosis of inflammatory cells by tumour cells with marginal densities and an investment of basal lamina was observed. It is suggested that the precursor cell in this lesion was a primitive mesenchymal cell showing varying degrees of smooth muscle differentiation and giant cell formation.

Adult↗

Uterine leiomyoma versus leiomyosarcoma: a new attempt at differential diagnosis based on their cellular characteristics.

AIM: To differentiate uterine leiomyoma (LM) with 'pseudosarcomatous' features from leiomyosarcoma (LMS). We hypothesized that because pseudosarcomatous LM is only a benign variant of conventional LM, it may have some characteristics different from LMS. METHODS AND RESULTS: Fourteen uterine smooth muscle tumours, which had been diagnosed as LMS previously, were reviewed and divided into adverse outcome (true LMS; seven cases) and excellent outcome groups (presumed pseudosarcomatous LM; seven cases) based on their clinical course. The tumours of the adverse outcome group were composed of rather uniform long spindle cells similar to those of leiomyosarcoma arising from the soft tissue. All tumours in this group had coagulative necrosis and all but one case were diagnosed preoperatively as malignant. In contrast, the tumours of the excellent outcome group showed significant histological diversity both between tumours and also in the same tumour and areas indistinguishable from conventional LM were often seen. All the tumours of this group were diagnosed preoperatively as benign. Immunohistochemically, all tumours of the excellent outcome group expressed progesterone receptor, often intensely, and also frequently expressed oestrogen receptor. In contrast, all the tumours of the adverse outcome group lacked them completely. CONCLUSION: Pseudosarcomatous LM has characteristics useful for differentiating it from LMS, which are common in conventional LM.

Actins↗