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Multifocal soft-tissue metastases of gastric leiomyosarcoma.

Distant soft-tissue metastases in gastric leiomyosarcoma (LMS) are extremely rare. In a 65-year-old woman, a large, low-grade LMS originating from the stomach wall was treated by partial gastrectomy. Eight years later, the patient developed multifocal, soft-tissue, high-grade LMS. These tumors were presumably metastases of the gastric LMS. However, due to the demonstration of mutations in some cases of leiomyosarcoma, the possibility of multifocal sarcoma should be kept in mind.

Aged↗

Leiomyosarcoma of the parotid gland: a case report.

Primary leiomyosarcoma of the parotid gland is an extremely rare neoplasm. We here describe the case of a 78-year-old male patient presenting with such a tumor. Five years after surgical treatment, the patient is alive and recurrence-free. To our knowledge, our case represents just the sixth case of primary leiomyosarcoma of the parotid gland reported in the English literature. The clinical features of these cases are summarized.

Aged↗

Laryngeal leiomyosarcoma: a case report and review of the literature.

Laryngeal leiomyosarcoma (LLM) is a rare malignancy originating from the smooth muscles of blood vessels or from aberrant undifferentiated mesenchymal tissue. Histological diagnosis may be particularly difficult and correct diagnosis is based on immunohistochemical investigations and electron microscopy. A case report of a LLM in a 74-year-old man is presented. Direct laryngoscopy revealed a large glottic lesion causing airway compromise and an emergency tracheotomy was performed. Subsequent total laryngectomy confirmed the diagnosis of leiomyosarcoma. Lung metastases developed 8 months following treatment, despite the absence of local or regional recurrence, and the patient died 3 months later. A review of the English and French literature revealed 30 previous cases of LLM. Clinical presentation, histological diagnosis, and management of this rare malignancy are analyzed aiming to improve our knowledge regarding the best treatment modality.

Aged↗

Poorly differentiated desmin-negative and vimentin-positive leiomyosarcoma of the stomach examined by the immunohistochemical and quick-freezing and deep-etching methods.

A poorly differentiated leiomyosarcoma of the stomach in a 41-year-old woman is reported. The diagnosis was confirmed by the diffuse immunohistochemical reaction to HHF35, and the presence of focal density and caveolas in some of the tumour cells by conventional electron microscopy. Immunohistochemically, most tumour cells had an undifferentiated nature, in which negative immunostaining for desmin, alpha-smooth muscle actin, and type IV collagen, and positive immunostaining for vimentin were observed. By the quick-freezing and deep-etching (QF-DE) method, these tumour cells revealed the loss of bundled actin and myosin filaments, which constitute desmin associated structures (focal densities and dense patchy areas). Their cytoplasm had many mitochondria and other cell organelles. The intermediate filaments (IFs), which were determined to be vimentin by immunohistochemistry, were observed in the inter-organellar spaces, and connected with these cell organelles. Actin filaments formed a meshwork structure and were distributed mainly in subplasmalemmal regions. Although a basal lamina was not detected by conventional electron microscopy, basal lamina-like structures, an association between the extracellular matrices and the cell membrane, were observed. Using the QF-DE method, three dimensional ultrastructural alterations of the cytoskeleton and extracellular matrix of the leiomyosarcoma were observed.

Actins↗

Vascular leiomyosarcoma arising from the inferior vena cava diagnosed by intraluminal biopsy.

A 61-year-old woman developed pain in the right thigh, paraplagia of the lower extremities and lumbago in November 1996. A lumbar spine roentgenogram showed lytic change in L2, and magnetic resonance imaging showed a patchy destructive lesion and compression of the dural sac from the right by a tumour. Computed tomography (CT) myelography showed a motheaten destructive lesion in L2 and projection of the tumour into the spinal canal. Abdominal ultrasound, CT and cavography showed dilatation of the inferior vena cava (IVC) and an intraluminal tumour about 2x2.8x4 cm in size in the IVC. The tumour arose from the IVC just beneath the renal vein and extended to just short of the right atrium. Both vertebral and intraluminal biopsy materials showed the same morphology, in which atypical spindle cells admixed with multinucleated giant cells proliferated in a fascicular growth pattern. Neoplastic cells were strongly positive for alpha-smooth muscle actin. We diagnosed vascular leiomyosarcoma arising from the IVC with metastasis to the lumbar vertebrae. Cases of vascular leiomyosarcoma diagnosed by intraluminal biopsy are rare.

Biopsy↗

Calponin and h-caldesmon expression in atypical fibroxanthoma and superficial leiomyosarcoma.

To evaluate smooth muscle differentiation, myogenic markers [desmin, alpha-smooth muscle actin (SMA), and muscle-specific actin (HHF35)] have been widely used. Calponin and h-caldesmon, which are cytoskeleton-associated actin-binding proteins, have been reported to be more specific myogenic markers, especially since myofibroblasts express a small amount of h-caldesmon. Atypical fibroxanthoma (AFX) occurs in the sun-exposed skin of the elderly and follows a benign clinical course. Histologically, AFX, which is a pleomorphic spindle cell tumor and considered to be a superficial variant of malignant fibrous histiocytoma, also mimics leiomyosarcoma. AFX has been thought to differentiate along pathways with fibrohistiocytic and myofibroblastic phenotypes. AFX ( n=10), superficial leiomyosarcoma (S-LMS) ( n=17) and benign fibrous histiocytoma (BFH) ( n=17) were analyzed for myofibroblastic and smooth muscle differentiation immunohistochemically from the viewpoint of comparison. AFX and BFH showed immunoreactivities respectively for calponin (3/10, 11/17), desmin (3/10, 1/17), SMA (3/10, 13/17), and HHF35 (1/10, 5/17), but failed to express h-caldesmon (0/10, 0/17). S-LMS had a high immunoreactive rate of calponin (17/17), desmin (13/17), SMA (16/17), and HHF35 (16/17), while also expressing caldesmon (11/17). The results reveal that AFX and BFH have immunoreactivities for several myogenic markers, with myofibroblastic differentiation (calponin: +/-, h-caldesmon: -), but without the smooth muscle differentiation seen in S-LMS (calponin:+, h-caldesmon: +/-). In addition, calponin and h-caldesmon are considered to be useful markers for distinguishing AFX from S-LMS.

Adolescent↗

Jejunal carcinoid tumor mimicking leiomyosarcoma: preoperative diagnosis by endoscopic biopsy.

Primary carcinoid tumor of the jejunum is rare, and is an unusual cause of massive gastrointestinal bleeding. A case of primary jejunal carcinoid tumor in a 39-year-old woman who presented with massive hematochezia is described. Both upper and lower gastrointestinal endoscopies showed no abnormalities. An abdominal computed tomographic scan, small-bowel barium contrast studies, and small-bowel endoscopy showed a subserosal mass, of 5 x 4 cm, with a cavity suggesting central necrosis, and a deep mucosal ulceration, located in the proximal jejunum. Although these clinical presentations were strongly suggestive of a leiomyosarcoma, histologic examination of biopsy samples obtained by enteroscopy confirmed the diagnosis of jejunal carcinoid tumor. The patient underwent radical jejunal resection and recovered uneventfully. In spite of the large size of the tumor, there was one solitary lymph node metastasis, but no evidence of liver metastases. This kind of jejunal carcinoid tumor, presenting with massive gastrointestinal bleeding and a subserosal bulky growth mimicking a leiomyosarcoma, has not been reported previously. Moreover, this is a rare case of a jejunal carcinoid which was diagnosed preoperatively by small bowel-endoscopic biopsy.

Adult↗

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

We report a case of leiomyosarcoma of the inferior vena cava, which was successfully treated by surgical en bloc resection and reconstruction of the inferior vena cava, followed by adjuvant radiation therapy. A 39-year-old man presented with nausea, vomiting, epigastric pain, and weight loss. Radiologic examinations showed a mass originating from the inferior vena cava and surgical resection was performed. Histopathological examination of the specimen revealed a moderately differentiated (grade II) leiomyosarcoma arising from the inferior vena cava. We believe that radical resection with clear surgical margins followed by adjuvant radiation therapy is a good curative strategy for achieving any chance of long-term survival.

Adult↗

Rapidly growing primary cardiac leiomyosarcoma: report of a case.

Primary cardiac leiomyosarcomas are very rare. A 19-year-old man was admitted to a local hospital with dyspnea and hemoptysis. He was later transferred to our hospital because of his worsening dyspnea. An enhanced chest computed tomography scan demonstrated a large mass in the left atrium. A transthoracic echocardiogram showed a large mobile mass in the left atrium. The tumor was totally resected. The pathohistological examination showed leiomyosarcoma. The tumor rapidly recurred. and a second and third operation were performed. After the third operation, the patient was treated with radiotherapy. There was no local recurrence but multiple distant metastases were found 2 months after completion of radiation therapy.

Adult↗

Perforated leiomyosarcoma of Meckel's diverticulum: report of a case.

We report herein a case of leiomyosarcoma of Meckel's diverticulum which presented as a rare manifestation of perforation. A previously healthy 63-year-old man was referred to the Tetsujinkai Eniwa hospital following the sudden development of acute abdominal pain. Abdominal computed tomography revealed a solid mass in the pelvic cavity, and an emergency operation was performed under a provisional diagnosis of peritonitis associated with a pelvic tumor. A perforated tumoral mass was found in Meckel's diverticulum. Segmental resection of the ileum, including the tumor-bearing diverticulum, was performed along with regional lymph node dissection. Histologic examination revealed the lesion to be leiomyosarcoma.

Humans↗

Primary hepatic leiomyosarcoma in a woman after renal transplantation: report of a case.

In contrast to malignant lymphomas or skin cancer, smooth muscle tumors including leiomyosarcoma are rarely associated with transplant recipients. We herein present a 33-year-old woman with end-stage renal disease who received a transplant at 27 years of age. Four years after the transplantation, at age 31, she underwent a mastectomy because of primary right breast cancer, which was found to be a 5-mm-sized mucinous carcinoma with no regional lymph node metastasis. Six years after the transplantation, a liver tumor was unexpectedly discovered. An explorative laparotomy revealed a well-encapsulated tumor occupying the posterior portion of the right lobe of the liver. The patient underwent a posterior segmentectomy. Histologically, the tumor possessed intermingling fascicles of spindle cells with eosinophilic cytoplasm and elongated nuclei. Based on an immunohistochemical examination, the tumor cells were positive for the muscle-associated antibody. In addition, RNA probes for Epstein-Barr virus were negative based on in situ hybridization. The histologic, immunohistochemical findings were considered to be diagnostic for leiomyosarcoma, which is a low-grade malignancy. Two years after surgery, the patient is doing well with no recurrence of liver tumors or breast cancer.

Actins↗

Asymptomatic thrombosis as a late complication of a retrohepatic vena caval graft performed for primary leiomyosarcoma of the inferior vena cava: report of a case.

A 63-year-old woman successfully underwent a graft replacement of the retrohepatic inferior vena cava with a ringed polytetrafluoroethylene graft for primary leiomyosarcoma of the inferior vena cava (IVC). Although anticoagulant had been administered, a thrombus was found in the IVC just cranial of the downstream anastomosis 67 months after the operation. The patient remained free of symptoms, and she had no evidence of any tumor recurrence. She underwent a complete resection with a prosthetic reconstruction for leiomyosarcoma of the IVC and has since been able to enjoy a reasonably long-term survival. The occurrence of thrombosis must be kept in mind in the long-term follow-up of such cases.

Blood Vessel Prosthesis Implantation↗

Leiomyosarcoma of the major peripheral arteries: case report and review of the literature.

We describe a case of primary intramural, extraluminal leiomyosarcoma of the common iliac artery. The patient presented with pelvic and leg pain and pheochromocytoma-like symptoms. The diagnosis was made intraoperatively, the tumor was resected, vascular reconstruction was performed, and postoperative radiation therapy was administered. Primary leiomyosarcoma of a major peripheral artery is extremely rare, and this report examines the clinical presentation, treatment, and prognosis of these patients.

Adult↗

Great saphenous vein leiomyosarcoma: a rare malignant tumor of the extremity--two case reports.

Peripheral vascular leiomyosarcomas are rare. Two cases of intramural leiomyosarcomas of the great saphenous vein are described. The first case is considered a high-grade sarcoma, based on the high mitotic index on histologic study. The second case presents only infrequent mitoses on light microscopy. Immunohistochemical analysis for factor VIII helped to establish the diagnosis. Characteristics of the tumor and differential diagnosis are discussed. Treatment consisted of wide local excision. Radiotherapy and chemotherapy were given to the patient with high-grade tumor.

Aged↗

Case report: leiomyosarcoma of the renal pelvis.

We present a new case of leiomyosarcoma, a very rare clinical entity, arising from the renal pelvis. The primary diagnosis, based on asymptomatic gross hematuria and imaging findings, was tumor of the left kidney. After a left radical nephro-ureterectomy, histology confirmed a leiomyosarcoma of the renal pelvis. No adjuvant treatment was provided and the patient remains healthy 3 years after surgery.

Actins↗

Bladder leiomyosarcoma in a boy.

Leiomyosarcoma of the urinary bladder in childhood is uncommon. We reported bladder leiomyosarcoma in a 10-year-old boy. He had underwent the extirpation of the tumor and performed adjuvant chemotherapy in combination with radiotherapy according to the Intergroup Rhabdomyosarcoma Study-4 regimens. He had no evidence of recurrence of disease after 3 years.

Child↗

Cardiac metastasis from uterine leiomyosarcoma.

Cardiac metastases are more frequent than primary heart neoplasias. Nearly any malignant tumour may metastasize to the heart, but the most common are carcinomas rather than sarcomas. We report the case of a patient who presented with heart metastasis 6 years after resection of an uterine leiomyosarcoma. The patient died thirty months after surgical resection without evidence of cardiac recurrence. Although cardiac metastases from uterine leiomyosarcoma are exceptional, they should be suspected in the presence of suggestive symptoms, since they can be associated with long survival after surgical treatment.

Antineoplastic Combined Chemotherapy Protocols↗