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Stenting for pulmonary artery stenosis due to a recurrent primary leiomyosarcoma.

PURPOSE: To report stent implantation for a malignant obstruction within the pulmonary artery (PA) caused by a recurrent leiomyosarcoma in the pulmonary trunk. CASE REPORT: A 62-year-old man with a non-metastatic primary leiomyosarcoma of the right PA underwent pneumectomy of the right lung and postoperative radiotherapy in 1994. Six years later, he presented with symptoms of progressive right ventricular dysfunction. Computed tomography (CT) identified a high-grade stenosis of the left PA due to recurrent tumor within the pulmonary trunk extending into the left PA. Transthoracic ultrasound documented severe pulmonary hypertension with a high pressure gradient across the stenosis. A stent was deployed percutaneously, successfully establishing PA patency. Pressure measurements showed a significantly reduced gradient across the stented area. In follow-up, the patient reported subjective improvement of symptoms; CT scans revealed a fully patent stent. His status remained stable 11 months after stent implantation. CONCLUSIONS: PA leiomyosarcoma is a rare and highly malignant tumor. In most cases, surgery can only prolong survival for the short term. Palliative interventional PA stenting performed under local anesthesia can offer improvement in quality of life by reducing excessive pulmonary hypertension.

Constriction, Pathologic↗

Delayed diagnosis of leiomyosarcoma of the common femoral artery after endovascular repair.

PURPOSE: To describe a unique case of misdiagnosed leiomyosarcoma of the common femoral artery presenting with signs and symptoms of high-grade stenosis, which was treated with stent placement. CASE REPORT: A 31-year-old woman with a history of diabetes and hyperlipidemia had recurrent claudication and showed significant in-stent restenosis of the common femoral artery in a postoperative angiogram at 5 months. The patient's clinical stage remained unchanged after repeat percutaneous intervention, and leiomyosarcoma was diagnosed from surgical specimens. The patient underwent resection and repair of the involved artery. She has survived 30 months with no further evidence of local recurrence or systemic metastatic disease. CONCLUSIONS: Endovascular repair may cause a long delay in the diagnosis of an arterial leiomyosarcoma mimicking peripheral occlusive disease.

Adult↗

Leiomyosarcoma of the inferior vena cava--a case report.

This paper presents a patient successfully treated for leiomyosarcoma of the vena cava, with no recurrence or symptoms on an 8-year follow-up. A 56-year-old woman presented with chronic and progressive periumbilical and right upper abdominal pain. Physical examination and laboratory tests revealed no abnormalities. Computed tomography showed a high-density image inside the vena cava. Cavography showed a filling defect with complete occlusion of the vena cava involving the renal veins and greatly developed collateral circulation through lumbar veins. Laparotomy was performed and a large caval mass involving the renal veins was dissected and resected. Venous reconstruction was undertaken using a 19-mm bovine pericardium prosthesis (Labcor, MG-Brazil) interposed as a substitute to the vena cava, and the renal veins were anastomosed to the side of the graft. Pathologic examination confirmed a leiomyosarcoma with free surgical margins. The postoperative course was uneventful and the patient was discharged on postoperative day 8. The venous flow through the inferior vena became normal, as confirmed by later cavography. This paper presents a case of successfully managed leiomyosarcoma of the vena cava with a thorough review of the literature. The treatment of such patients and the use of bovine pericardium are also discussed.

Blood Vessel Prosthesis Implantation↗

Cyclooxygenase-2 expression in uterine leiomyosarcomas.

Many studies have focused on cyclooxygenase-2 (COX-2) alterations as a critical step in the onset and progression of cancer. Moreover, a strong correlation between COX-2 and chemoresistance has been demonstrated in several carcinomas. Recently, COX-2 expression has been observed in uterine carcinosarcoma, osteosarcoma, and rhabdomyosarcoma. We investigated COX-2 expression in chemoresistant uterine leiomyosarcoma in 30 patients who had undergone surgical treatment. COX-2 expression was observed in 13 cases (43.3%). Of the 13 patients with distinct COX-2 positive immunoreactivity uterine leiomyosarcomas, 7 had stage I or II disease and 6 had stage III or IV disease. The expression of COX-2 in uterine stromal malignancies may reveal a therapeutic hypothesis in the context of uterine leiomyosarcoma molecular chemotherapeutic approach.

Adult↗

Molecular cloning of TA16, a transcriptional repressor that may mediate glucocorticoid-induced growth arrest of leiomyosarcoma cells.

The DDT1 MF2 smooth muscle tumor cell line was derived from an estrogen/androgen-induced leiomyosarcoma that arose in the ductus deferens of a Syrian hamster. The growth of this cell line is arrested at the G0/G1 phase of the cell cycle after treatment with glucocorticoids. To identify the putative gene(s) that are potentially involved in this hormone-induced cell growth arrest, we have used a differential screening technique to clone those genes whose expression is induced or up-regulated by glucocorticoids. A number of glucocorticoid response genes were thereby isolated from the leiomyosarcoma cells. One of these clones, termed TA16, was found to be markedly up-regulated by glucocorticoids in DDT1 MF2 cells, but only marginally changed in GR1 cells, a glucocorticoid-resistant variant that was selected from the wild type DDT1 MF2 cell. Isolation and sequencing of its intact cDNA indicated that the TA16 encodes a protein 485 amino acids long, and its sequence is closely homologous to a novel transcriptional repressor that presumably represses the transcription activity of some zinc finger transcriptional factors through a direct interaction. Transfection assays demonstrated that introduction of an antisense TA16 cDNA expression vector, controlled by an MMTV promoter, into the DDT1 MF2 cell significantly relieved the glucocorticoid-induced cell growth arrest. This finding suggests that TA16 might participate in the mediation of glucocorticoid-induced cell cycle arrest in leiomyosarcoma cells.

Amino Acid Sequence↗

Leiomyosarcoma metastatic to the brain: case report and literature review.

An intracerebral hematoma (ICH) developed in a patient during diagnostic evaluation for hemoptysis and guaiac-positive stools. Tissue obtained from colonic polyps, a pregastric mass, and cerebrum had features of poorly differentiated leiomyosarcoma. Only three other cases of pathologically confirmed cerebral metastasis of leiomyosarcoma are reported in the literature. The presence of cerebral metastasis at the time of the initial diagnosis of leiomyosarcoma and the associated ICH are features unique to this case.

Biopsy↗

Primary extradural epithelioid leiomyosarcoma of the cervical spine: case report and literature review.

OBJECTIVE AND IMPORTANCE: No case of primary epithelioid leiomyosarcoma involving the spine has been reported previously. CLINICAL PRESENTATION: A 61-year-old Nigerian woman presented with progressive spastic quadriparesis and acute urinary retention. Her only medical history included a total abdominal hysterectomy for fibroids 10 years earlier in Nigeria. Results of the general examination were normal. Pyramidal spastic quadriparesis (3/5) with a sensory level at C5-C6 was found neurologically. Magnetic resonance imaging scans of the brain and spine revealed extradural cord compression at C3-C5 as the sole abnormality. This was caused by a large, soft tissue mass arising from the erector spinae muscles, which was predominantly isointense on T1-weighted images, of mixed intensity on T2-weighted images, and homogenously enhanced after gadolinium contrast agent administration. There was an associated signal change in the cord at C3-C4. Computed tomography confirmed the predominantly soft tissue involvement, but with bone erosion and infiltration within the posterior elements of C4. Systemic screening for cancer was negative. INTERVENTION: At decompressive laminectomy, urgently undertaken under corticosteroid cover, an excessively vascularized, soft tissue tumor was subtotally excised, after which independent walking and normal sphincter function were regained within 1 week. Four weeks later, a complete macroscopic tumor excision was undertaken, incorporating lateral mass (C3-C6) and C2 pedicle screw stabilization, along with iliac crest bone grafting. The patient's neurological status continued to improve. However, while awaiting radical radiotherapy, the patient declined further treatment and returned to her native Nigeria. Histopathological findings were consistent with an epithelioid leiomyosarcoma. CONCLUSION: This is the first reported case of a primary craniospinal epithelioid leiomyosarcoma.

Actins↗

MR imaging of primary pancreatic leiomyosarcoma.

Primary leiomyosarcoma of the pancreas is a rare neoplasm with only 17 reported cases in the literature. Although the ultrasonic and computed tomographic appearances have been described, the magnetic resonance features of pancreatic leiomyosarcomas have not. In this report, we describe the MR characteristics of a primary pancreatic leiomyosarcoma which was an incidental finding on ultrasound. The unenhanced T1 weighted and T2 weighted images were the most useful in tumour localization whereas the enhanced images actually obscured the tumour within the pancreas. Differentiation from the much more commonly occurring adenocarcinoma was impossible.

Female↗

Oral leiomyosarcoma in a woodchuck (Marmota monax).

We report the case of a 4-year-old female woodchuck (Marmota monax) which presented with a white, firm and discrete mass in the hard palate. The mass extended into the oral cavity but it was well separated from the surrounding tissues. Histology of the tumor showed a malignant mesenchymal tumor with pleomorphic spindle cells varying in degrees of differentiation and density. The neoplastic cells had moderate amounts of granular or fibrillar eosinophilic cytoplasm with indistinct cell margins. Nuclei were oval to elongated and frequently blunt-ended with vesicular chromatin. Immunohistochemical study showed that the neoplastic cells expressed vimentin and alpha-smooth muscle actin but did not express desmin, pan-cytokeratin, and S-100. Therefore, histology and immunohistochemistry revealed that the tumor was oral leiomyosarcoma. Oral cavity is an extremely rare site for leiomyosarcoma and the present case is the first report of spontaneous oral leiomyosarcoma in animals.

Actins↗

Caprine genital leiomyosarcoma.

An aged Saanen doe was euthanized following repeated severe hemorrhage from the vulva. Necropsy examination revealed mural thickening of tubular genitalia with firm, protruding intralumenal masses containing blood-filled cavitations, and effacement of normal cervical architecture. Histologically, uterine and cervical masses comprised a variably dense population of mildly pleomorphic spindle cells forming interlacing streams supported by variably dense collagenous stroma. Immunoperoxidase staining of neoplastic cells was positive for muscle-specific actin, supporting the diagnosis of low-grade leiomyosarcoma. Months later, the doe's twin was likewise euthanized due to persistent bleeding from the vulva associated with a large vulvar mass having histopathologic features similar to those of the previous case. The clinical, gross, and histologic findings are similar to five cases of caprine genital leiomyosarcoma identified in retrospectively analyzed case material. Analysis of caprine tumor accessions over 20 years demonstrated a significantly higher incidence of genital leiomyosarcoma within the Saanen breed.

Animals↗

Human pancreatic leiomyosarcoma (PZX-7) growing as a serially transplantable xenograft in immunosuppressed mice.

BACKGROUND: Several human leiomyosarcoma xenografts have been established, but pancreatic smooth muscle sarcomas have never been serially transplanted and investigated. METHOD: Immunosuppression of CBA/CA mice was achieved by thymectomy, whole-body irradiation, and bone marrow reconstruction. Tumor fragments were subcutaneously implanted from a Grade III pancreatic leiomyosarcoma and serially passaged for more than 24 mo. The xenografted tumors were characterized by morphological, morphometrical, biochemical, and flow cytometric methods. RESULTS: The tumor has retained its characteristic morphology and no further differentiation occurred. The mitotic counts and the amount of the connective tissue all remained constant. The calculated volume doubling time was 11.3 d. Immunohistochemically, the tumor proved to be p53-negative, but the strong expression of the bcl-2 remained as a constant feature throughout successive transplantations. The DNA index and the proliferation indices did not change significantly with the time (mean DI: 1.65, range: 1.561-1.70; mean PI: 17.9%, range: 15.3-20.7%). Lactose dehydrogenase (LDH) isoenzyme electrophoresis evidenced a retained human pattern of the tumor even after 32 mo of transplantations. CONCLUSION: The first human pancreatic leiomyosarcoma xenograft (PZX-7) growing in immuno-suppressed mice is described and characterized.

Animals↗

Preoperative diagnosis of pancreatic leiomyosarcoma.

BACKGROUND: The low incidence of pancreatic leiomyosarcoma is responsible for the small number of cases correctly diagnosed preoperatively, the tumor being frequently confused with benign pancreatic lesions. RESULTS: We describe a symptom free 52-yr-old male bearing an abdominal mass incidentally found at physical examination. Imaging techniques revealed a nonhomogenous large mass at the head of the pancreas that dislodged the portal vein and the superior mesenteric vein. Increased metabolic activity in the tumor area demonstrated by 18F-fluorodeoxyglicose positron emission tomography scan allowed the diagnosis of a malignant lesion. The patient was operated on and a pylorus preserving pancreatoduodenectomy performed. The pathology diagnosis was a low grade leiomyosarcoma. Immunohistochemistry revealed positivity for vimentin and smooth muscle specific actin. The clinical course was uneventful after 2 yr follow-up. CONCLUSION: Pancreatic leiomyosarcoma may be preoperatively diagnosed by image techniques and differentiated from benign lesions by means of fluorodeoxyglicose positron emission tomography scanning (FDGPET).

Actins↗

Ventricular outflow tract obstruction secondary to leiomyosarcoma of the right ventricle.

Primary leiomyosarcomas of the heart, particularly those affecting the right ventricle, are uncommon. We report the case of a 70-year-old Belgian woman presenting with the symptoms of progressive exertional dyspnea and left-sided pleuritic pain. A leiomyosarcoma which originated from the right lateral ventricle wall, causing pulmonary outflow obstruction, was diagnosed. Pathology revealed a neoplasm with a myxoid stroma, high mitotic activity and nuclei expressing atypia. Immunohistochemical staining was positive for vimentine and desmin. Seven months after complete surgical resection the tumor relapsed. This case demonstrates the poor outcome, the high relapse rate and inefficiency of treatment associated with primary cardiac leiomyosarcomas. The current literature regarding the incidence, diagnostic techniques, treatment strategies and survival rates of this rare but terminal disease is reviewed.

Aged↗

Paratesticular leiomyosarcoma treated by enucleation.

Spermatic cord leiomyosarcomas are rare tumors and standard treatment consists of radical orchiectomy and high cord ligation. We report a case of a paratesticular leiomyosarcoma successfully treated by enucleation. A 22-year-old man presented with a 6-months history of inguinal pain. Physical examination revealed a right paratesticular nodule about 0.5 cm in diameter. Inguinal exploration and nodule biopsy were performed. It was thought to be a benign epididymal nodule on a quick section and the tumor was enucleated and sent for paraffin section. Histology and immunohistochemistry were compatible with leiomyosarcoma. The patient was advised to undergo radical orchiectomy with high cord ligation. However, he refused surgery. An alternative approach with clinical, biochemical and radiological follow-up was adopted. The patient has been followed up for thirteen years and shows no evidence of disease.

Adult↗

Cerebral metastasis of a uterine leiomyosarcoma--case report.

A 38-year-old female presented with sudden neurological deterioration 6 years after an operation and chemotherapy for uterine leiomyosarcoma. An extremely rare metastasis of the uterine leiomyosarcoma to the brain was identified and totally resected. Whole brain irradiation (50 Gy) was given. A recurrence of the metastasis was resected 10 weeks later. She ultimately died of a second recurrence. Aggressive surgical management of cerebral metastasis of uterine leiomyosarcoma may achieve an improved outcome.

Adult↗

Metastatic leiomyosarcoma of the brain manifesting as multiple hemorrhages. Case report.

A 74-year-old man presented with a rare metastatic leiomyosarcoma of the brain manifesting as subacute development of multiple cerebral hemorrhages. Cerebral angiography demonstrated no tumor staining or vascular malformation. Whole body computed tomography revealed abnormal masses in the liver, left adrenal gland, and duodenum. Histological examination of an open biopsy specimen identified the lesion as metastatic leiomyosarcoma. Whole brain irradiation controlled the intracranial lesions, but the patient's general condition progressively deteriorated and he died of pneumonia. Metastatic leiomyosarcoma is a very uncommon tumor in the central nervous system with a poor response to existing treatment options. Consequently, new approaches to the treatment of this disease are needed.

Aged↗

Angiography of duodenal leiomyomas and leiomyosarcomas.

Five duodenal smooth muscle neoplasms (two leiomyomas and three leiomyosarcomas) are reported. All occurred in the lower second part of the duodenum, and the angiographic findings were similar to those of myomatous tumors occurring elsewhere in the gastrointestinal tract. The leiomyomas were hypervascular with sharply demarcated margins. The leiomyosarcomas were hypervascular with sharply demarcated margins. The leiomyosarcomas were larger and less vascular than the leiomyomas and had indistinct margins. However, local invasion and metastases were definite signs of malignancy. One of the patients with a leiomyoma was examined because of active bleeding, and the hemorrhage was controlled by selective embolization.

Angiography↗

Primary leiomyosarcoma of bone.

OBJECTIVE: This study describes radiographic and MR imaging features of primary leiomyosarcoma of bone. SUBJECTS AND METHODS: Twelve patients (five men and seven women, 39-79 years old) who were treated at two oncology centers for primary leiomyosarcoma of bone involving the femurs, tibia, ilium, and inferior pubic ramus were studied. None of the patients had preexisting disease or disease elsewhere at the time of diagnosis. Pathologic diagnosis was obtained in all patients. RESULTS: Radiographs of all patients showed a matrix that was exclusively osteolytic. In long bones (seven patients), the tumor had an average length of 11 cm (range, 7-17 cm) and revealed an elongated configuration. In the pelvis (five patients), the average length of the tumor was 10 cm (range, 4-15 cm). MR imaging confirmed an intramedullary lesion in all patients, with extension into the soft tissues in eight patients and no identifiable soft-tissue mass in the remaining four patients. Four of the five pelvic tumors had a prominent soft-tissue mass, whereas only four of the seven long bone lesions revealed a soft-tissue mass that was, in all instances, small. The tumor was hypointense on T1-weighted images and showed heterogeneous signal intensity on T2-weighted conventional and fast spin-echo sequences. We saw low signal intensity (short T2) in eight patients and homogeneous hyperintense signal intensity in one patient. In the remaining three patients, T2-weighted spin-echo sequences obtained with fat saturation showed high signal intensity (long T2) in the tumors. CONCLUSION: Primary leiomyosarcoma of bone is a rare tumor that on radiography reveals no matrix and on MR imaging reveals areas of T2 shortening in relation to fat on conventional and fast spin-echo sequences.

Adult↗