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PubMed2002

Leiomyosarcoma presenting as a spontaneously ruptured renal tumor-case report.

BACKGROUND: Ruptured renal neoplasms can be a catastrophic clinical presentation. Angiomyolipoma is the commonest renal tumor which presents in this fashion. Renal sarcomas are rare renal neoplasms. Renal leiomyosarcomas are the most common histological subtype of renal sarcomas, accounting for approximately 50-60% of the reported cases. These tumors are usually peripherally located and appear to arise from either the renal capsule or smooth muscle tissue in the renal pelvic wall. CASE PRESENTATION: A 70 years old male, with hypertension and ischemic disease, developed acute left flank pain. The general physician evaluated this using ultrasound, which showed a solid left renal mass. Two weeks later, he presented in the emergency room in a state of shock with a palpable flank mass. CT scan of the abdomen showed a large heterogeneous mass lesion in the left perinephric space with minimal post contrast enhancement. Per-operatively, large retroperitoneal hematoma was found within Gerota's fascia along with spleen plastered to the upper limit of hematoma. Nephrectomy and splenectomy were performed. Postoperative course was uneventful and patient was discharged on the 10th post-operative day. Histopathological evaluation of the specimen showed high-grade leiomyosarcoma CONCLUSIONS: Spontaneous rupture of renal neoplasm is a rare clinical presentation. Angiomyolipoma is the commonest cause of spontaneous rupture of the kidney. Presentation of a leimyosarcoma as a ruptured renal neoplasm has not been previously reported in the English literature.

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PubMed2000

Soft tissue leiomyosarcomas and malignant gastrointestinal stromal tumors: differences in clinical outcome and expression of multidrug resistance proteins.

PURPOSE: Several studies have reported clinical behavior and chemotherapy resistance in leiomyosarcomas, but these studies did not differentiate between soft tissue leiomyosarcomas (LMS) and malignant gastrointestinal stromal tumors (GIST). Multidrug resistance (MDR) has been associated with the expression of P-glycoprotein (P-gp), multidrug resistance protein (MRP(1)), and lung resistance protein (LRP). The aim of the present study was to compare LMS and GIST with respect to clinical outcome and MDR parameters. PATIENTS AND METHODS: Clinical outcome was evaluated in 29 patients with a primary deep-seated LMS and 26 patients with a primary malignant GIST. Paraffin-embedded material, available for 26 patients with LMS and 25 with GIST, was used for immunohistochemical detection of P-gp, MRP(1), LRP, and c-kit. RESULTS: Mean overall survival (OS) was 72 months for LMS patients and 31 months for GIST patients (P: <.05). Metastases occurred in 16 (59%) of 27 assessable LMS patients and in 10 (56%) of 18 assessable GIST patients. LMS predominantly metastasized to the lungs (14 of 16 patients), whereas GIST tended to spread to the liver (five of 10 patients) and the abdominal cavity (three of 10 patients; P: <.001). P-gp and MRP(1) expression was more pronounced in GIST than in LMS (P: <.05): the mean percentage of P-gp expressing cells was 13.4% in patients with LMS and 38.4% in patients with GIST, and the mean percentage MRP(1) expressing cells was 13.3% in patients with LMS and 35.4% in patients with GIST. LRP expression did not differ between LMS and GIST. c-kit was expressed in 5% of the LMS patients and in 68% of the GIST patients. CONCLUSION: LMS patients have a better survival than GIST patients, and the metastatic pattern is different. Expression of MDR proteins in LMS is less pronounced than in GIST.

ATP Binding Cassette Transporter, Subfamily B, Mem↗

PubMed2001

Leiomyosarcoma of the larynx: emergency tracheostomy.

Leiomyosarcoma of the larynx is extremely rare. Histological diagnosis for excluding other tumours remains extremely difficult. A rare case of leiomyosarcoma of the larynx, treated by surgery and radiotherapy, is reported. We discuss the clinical presentation, diagnosis, and treatment of leiomysarcoma with a review of the literature.

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PubMed1998

Leiomyosarcoma of the maxillary sinus: CT and MRI findings.

This report describes the CT and MRI appearances of a leiomyosarcoma of the maxillary sinus. CT showed frank bony destruction, no calcification and a low attenuation area within the mass. MRI showed intermediate intensity on T1 weighted images, intermediate to slightly high signal intensity on T2 weighted images and moderate inhomogeneous enhancement. Precise identification of the extent of the tumour, especially of orbital invasion, is of utmost importance because local recurrence is common after the resection of leiomyosarcomas.

Aged↗

PubMed1991

A case of renin producing leiomyosarcoma originating in the lung.

A 54-year-old woman was referred to our hospital for the treatment of a tumor of the right chest wall. Clinical examination revealed hypertension, hypokalemia, metabolic alkalosis, hyperaldosteronism and hyperreninemia. Computed tomography and an abdominal echogram indicated a tumor in the right phrenic area and two tumors in the retroperitoneum near the pancreas head. After the surgical resection of these tumors, the primary reninism was diminished. The pathological diagnosis of these tumors was leiomyosarcoma. Plasma active and inactive (trypsin-activated) renin activities (PRA) were 85.7 and 38.9 ng angiotensin I/ml/h, respectively. These PRA did not respond to either postural stimulation or suppression by the volume expansion. Active and inactive renin activities in a right phrenic area tumor were 208 and 32 ng angiotensin I/mg protein /h, respectively. Those of an abdominal tumor were 196 and 30 ng angiotensin I/mg protein/h, respectively. Renin mRNA identical in molecular size to that of the human kidney was identified by northern blot analysis. This is the first case report of renin producing leiomyosarcoma derived from the lung, which is characterized by relatively lower plasma prorenin concentrations.

Abdomen↗

PubMed1997

A case of leiomyosarcoma of the sphenoid sinus.

A 43-year-old man with a primary leiomyosarcoma of the left sphenoid sinus is presented. To our knowledge, this is an unusual case of leiomyosarcoma, which has never been reported in the literature. Accurate and safe diagnosis was obtained by an endonasal endoscopic approach with minimal tissue invasion.

Adult↗

PubMedDate not supplied

Myxoid leiomyosarcoma of the gizzard in a broiler chicken.

A leiomyosarcoma was found in the gizzard of a 57-day-old female broiler chicken weighing 1.8 kg. Grossly, the tumor mass, 13.0 x 8.5 x 10.0 cm, enveloped the gizzard and had a gelatinous appearance due to the rich production of mucin. Miliary metastatic tumors were noted in the liver. Histopathologically, there was marked production of mucus throughout the tumor tissue, and densely or loosely arranged long spindle-shaped leiomyosarcoma cells proliferated. The tumor cells had a low rate of mitosis, showed slight cellular atypia, and, immunohistochemically, were positive for actin, alpha-smooth muscle actin, and desmin. Electron microscopically, various amounts of microfibrils with focal densities, dense patches, and basal plates were observed.

Animals↗

PubMedDate not supplied

Gastrointestinal leiomyosarcoma in 14 dogs.

A retrospective review over a 5-year period was performed to identify clinical characteristic features and survival in 14 dogs with intestinal leiomyosarcoma after surgery. Anemia, hypoglycemia, polyuria, and polydipsia were identified as common clinical signs in these dogs. Dogs that survived the immediate postoperative period had a median survival of 21.3 months, (range 0.1-72.5 months). The 1- and 2-year survival rates were 75 and 66%, respectively. Dogs with intestinal leiomyosarcoma that survive the immediate postoperative period can experience long-term survival after surgical excision. Furthermore, dogs with histologically documented metastasis at the time of surgery also appear to have a long survival, with a mean survival of 21.7 months (range 4.2-41.5 months).

Aging↗

PubMed1999

Leiomyosarcoma presenting as a mandibular gingival swelling: a case report.

We report a case of primary low-grade leiomyosarcoma of the mandible in an otherwise healthy young woman. The neoplasm presented as a painful, pericoronal gingival swelling that mimicked an acute periodontal infection. It was managed accordingly, with curettage, debridement, and antibiotics. When the lesion failed to respond to this treatment approach, a biopsy was performed. Microscopy revealed a malignant mesenchymal neoplasm which, on immunohistochemistry analysis, demonstrated reactivity for smooth muscle actin (SMA) and vimentin. This established the diagnosis of leiomyosarcoma; subsequently, an en bloc resection of mandibular bone and overlying soft tissue was performed. Close follow-up for over 10 years has revealed no evidence of recurrent or metastatic disease. Since the patient was taking oral contraceptives prior to the onset of the lesion, a possible link between estrogen and smooth muscle tumors is considered.

Adult↗

PubMed1992

A case of primary leiomyosarcoma of the heart.

A 53-year-old male was hospitalized with complaints of cough, fever and backache. Two-dimensional echo-cardiography showed a pericardial echo-free space and a mass in the right atrium. Based on the MRI findings showing a pericardial mass originating from the atrial tumor, the final diagnosis of leiomyosarcoma was made by a percutaneous pericardial biopsy. Despite various therapies, the patient died after 3 wk. Because of its rareness (to date only 25 case reports), a premortem diagnosis of primary cardiac leiomyosarcoma is generally difficult. However, we feel that MRI and a subsequent biopsy is quite useful for making an early diagnosis of this disease.

Biopsy, Needle↗

PubMed1998

Primary cardiac leiomyosarcoma growing rapidly and causing right ventricular outflow obstruction.

Leiomyosarcomas are extremely rare primary cardiac tumors. We report a rapidly growing primary leiomyosarcoma of the right ventricle, which obstructed the right ventricular outflow tract within one month after symptom onset in a 68-year-old man. Two-dimensional echocardiography was useful in diagnosing the extent and progression of the tumor. The tumor was surgically resected on an emergency basis, and the right ventricle and pulmonary artery were successfully reconstructed. Recurrence of the tumor on the right ventricle was observed, and the patient was overcome by sudden dyspnea and died three months after surgery.

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PubMed2000

Synchronous primary adenocarcinoma of the lung and leiomyosarcoma of the small intestine.

The occurrence of synchronous epithelial cancer of the lung and leiomyosarcoma of the small intestine is rare. We report here the case of a 62-year-old man with adenocarcinoma of the lung in clinical stage IIIB (T4N0M0). After two courses of chemotherapy (cisplatin, 80 mg/m2 and mitomycin C, 8 mg/m2) the patient developed symptoms of a small bowel obstruction. Palliative surgical resection was performed and a leiomyosarcoma of the small intestine was found and defined by an immunohistological study. The resection ameliorated the patient's symptoms. The patient died of disseminated adenocarcinoma 26 months following chemotherapy.

Adenocarcinoma↗

PubMed2003

Rapid progression of primary cardiac leiomyosarcoma with obstruction of the left ventricular outflow tract and mitral stenosis.

We report a 73-year-old woman with primary cardiac leiomyosarcoma in the left atrium and ventricle. The tumor progressed very rapidly for 2 months after initial clinical evaluation. Obstruction of the left ventricular outflow tract and mitral stenosis were induced by the tumor. Urgent surgical resection was performed because she had cardiogenic shock due to paroxysmal atrial fibrillation. We could not resect the tumor completely because of severe invasion. She refused postoperative chemotherapy and radiotherapy, and died suddenly at home 89 days after surgery. To our knowledge, this is the first observation of mitral stenosis concomitant with obstruction of the left ventricular outflow tract in a patient with primary cardiac leiomyosarcoma.

Aged↗

PubMed1994

Metastatic leiomyosarcoma of the skull--case report.

A 65-year-old female presented with a rare intestinal leiomyosarcoma metastasis to the skull manifesting as a mass beneath the scalp. She was free of neurological and physical symptoms on admission. The tumor was totally removed with normal surrounding bone and dura. The histological diagnosis was leiomyosarcoma. Ultrastructural and immunohistochemical studies demonstrated the smooth muscle origin of the tumor. Such patients in good physical condition should be immediately treated surgically to achieve the best chance of survival.

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PubMed1996

Uterine leiomyosarcoma metastasis to the skull--case report.

A 54-year-old female presented with a rare uterine leiomyosarcoma metastasis to the skull appearing as a gross mass beneath her scalp. She had no neurological or other physical symptoms on admission. Computed tomography and magnetic resonance imaging demonstrated an enhanced dumbbell-shaped mass at the mid-frontal region beneath the scalp. The tumor was totally removed with normal surrounding bone and dura. The histological diagnosis was leiomyosarcoma. Her postoperative course was uneventful, and she received adjuvant chemotherapy. However, multiple distant bone metastases developed 1 year later. Immediate and radical resection of such tumors is recommended.

Fatal Outcome↗

PubMed2002

Secondary erythrocytosis associated with high plasma erythropoietin concentrations in a dog with cecal leiomyosarcoma.

A 14-year-old mixed-breed dog was examined because of severe absolute erythrocytosis (PCV, 70%). Plasma erythropoietin (EPO) concentration was consistently high, even though results of arterial blood gas analyses were normal. Radiography, ultrasonography, urinalysis, and serum biochemical analyses did not reveal any cardiac, pulmonary, or renal abnormalities that could cause the erythrocytosis, and erythrocytosis secondary to inappropriate EPO secretion was diagnosed. The PCV was maintained at approximately 60% by means of periodic phlebotomy, and the dog died of acute renal failure 2 years later. At necropsy, a cecal leiomyosarcoma was identified. Immunohistochemical staining of sections of the tumor revealed intracellular vacuoles containing EPO, and EPO mRNA was detected in the tumor by use of a reverse transcriptase-polymerase chain reaction assay These results suggested that ectopic production of EPO by a cecal leiomyosarcoma was the cause of erythrocytosis in this dog.

Animals↗

PubMed1998

Three cases of the giant gastric leiomyosarcomas.

We report three cases of giant gastric leiomyosarcoma. Diagnosis was made prior to surgery using various diagnostic modalities. The patients were two women (77 and 77 years old) and one man (40 years old) whose chief complaints were abdominal pain, anorexia, and tarry stool. All patients presented with a large palpable mass in their upper abdomen at the time of admission. Based on characteristic findings from a gastric barium study, computed tomography (CT), and angiography, the patients were diagnosed as having gastric leiomyosarcomas displaying extramural growth. In the first case, a patient received a total gastrectomy, while local resection was performed in the second case because of pedunculated extragastric development. In the third case, total gastrectomy was combined with splenectomy and resection of the pancreatic tail. Twenty-two months after the original operation, the first case had multiple liver metastases. We present the three cases here, and review the literature.

Adult↗

PubMed1998

A case of leiomyosarcoma of the small bowel mesenterium.

Leiomyosarcoma of the small bowel mesenterium is a rare entity. Approximately 21 cases originating from the small bowel mesenterium have been described in the Japanese literature. Differentiation from its benign counterpart, leiomyoma, and other connective tissue tumors is often difficult, but it is important because each respective tumor type has an entirely different prognosis. The case of a 65 year-old-man in whom an 9 x 6 x 5 cm leiomyosarcoma of the mesenterium was excised surgically is presented.

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