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Epithelioid leiomyosarcoma of the left atrium: immunohistochemical and ultrastructural findings.

Leiomyosarcomas of the heart are rare, with only 12 cases reported in the literature. An example of the epithelioid variant of leiomyosarcoma is described. The tumour cells expressed vimentin, desmin, muscle-specific actin and smooth muscle-specific actin. In addition, they were also labelled by monoclonal and polyclonal antibodies to cytokeratin intermediate filaments and displayed cell junctions at the ultrastructural level, features which highlight the potential pitfalls in the application of immunohistochemistry and electron microscopy in the diagnosis of poorly differentiated tumours.

Female↗

Leiomyosarcoma of the upper extremity.

A case of rapidly fatal, high-grade leiomyosarcoma of the upper extremity is described. The pleomorphic appearance of this sarcoma on light microscopy required the use of immunohistochemistry and electron microscopy to establish the morphologic diagnosis and to exclude a diagnosis of other high-grade sarcoma, such as malignant fibrous histiocytoma or pleomorphic rhabdomyosarcoma. The observed clinical behavior of this leiomyosarcoma of the upper extremity seemed unusually aggressive compared with the biologic behavior predicted by the independent prognostic indicators of location, tumor size, and depth of invasion. The observed clinical behavior did not seem unusual compared with the clinical behavior predicted by the use of a staging scheme for soft tissue sarcomas.

Arm↗

Primary leiomyosarcoma of brain in an adolescent with common variable immunodeficiency syndrome.

A 14-year-old girl with common variable immunodeficiency syndrome was found to have a low-grade malignant neoplasm arising in the left temporal lobe of the brain. Ultrastructural and immunohistochemical studies established a diagnosis of leiomyosarcoma, despite the rarity of this tumor in children. In situ hybridization with the EBER probe revealed essentially all of the neoplastic cells to be infected with Epstein-Barr virus (EBV). Children with the acquired immunodeficiency syndrome (AIDS) are known to exhibit an increased incidence of smooth muscle tumors associated with EBV. Similar tumors have been reported in EBV-infected patients undergoing therapeutic immunosuppression. This appears to be the first reported case of childhood leiomyosarcoma where the cause of the underlying immunodeficiency was a genetic rather than acquired disorder. The authors conclude that electron microscopy, immunohistochemistry, and other ancillary techniques are essential in the evaluation of unusual tumors in immunocompromised children, whether the cause is hereditary or acquired.

Adolescent↗

Epstein-Barr virus-associated dural leiomyosarcoma in a man infected with human immunodeficiency virus. Case report.

A 35-year-old man infected with human immunodeficiency virus presented with cervical myelopathy of 2 months duration. Clinical and radiographic evaluation revealed a discrete, subdural mass at C-6. At surgery, the mass proved to have a dural attachment and thus clinically, radiographically, and grossly, it resembled meningioma. Histopathological analysis revealed a leiomyosarcoma that stained diffusely for muscle-specific actin. Electron microscopy revealed basal lamina surrounding the tumor cells and intracytoplasmic bundles of myofilaments. Epstein-Barr virus (EBV) was demonstrated within tumor cell nuclei by in situ hybridization for EBER1 messenger RNA and immunohistochemical staining for EBNA2 protein. Epstein-Barr virus latent membrane protein (LMP1) was not detected. This is the first documentation of an EBV-associated smooth-muscle tumor of the dura, and the first demonstration that tumors in this location contain EBV in an unusual form of latency not seen in lymphoid cell lines. With increasing numbers of individuals being afflicted with long-term immunosuppression, EBV-associated dural leiomyoma and leiomyosarcoma may be encountered more frequently in the future.

Adult↗

Leiomyosarcoma arising in the great saphenous vein--a case report.

Though leiomyosarcoma usually occurs in the gastrointestinal tract and uterus, it rarely occurs in the wall of large veins and arteries. We present a case of primary leiomyosarcoma arising in the great saphenous vein of the left inner thigh and spreading for some extent along the vein in a 54 year old female. Diagnosis was confirmed by desmin stain and electron microscopy. Postoperative course was fine. This is the first report of this in Korean literature.

Female↗

Myxoid leiomyosarcoma of the uterus: a case report and review of the literature.

A case report of myxoid leiomyosarcoma of the uterus in a 54-year-old woman is presented as a rare variant of uterine sarcoma. Only 14 cases have been described in the literature. These tumors have a striking myxoid appearance and exhibit highly malignant behavior despite their low mitotic index. Although it is difficult to establish the nature of the neoplastic cells in the myxoid areas, both light microscopical and immunohistochemical characteristics showed features of smooth muscle cells in some of the cellular areas. Our patient had a tumor which arose in association with hyalinized benign leiomyomatous lesion. Like other reported cases of myxoid leiomyosarcoma, the tumor behaved aggressively.

Female↗

Retroperitoneal malignant mesenchymoma: a case of mesenchymal mixed tumor with osteosarcoma, leiomyosarcoma, liposarcoma and fibrosarcoma.

Malignant mesenchymoma is an interesting but very rare tumor in which malignant differentiation has occurred twice or more. We report a case of retroperitoneal malignant mesenchymoma consisting of osteosarcoma, leiomyosarcoma, liposarcoma and fibrosarcoma. Abdominal CT showed a large retroperitoneal mass with two separate and distinct parts, namely an area of prominent calcification and one of clearly enhancing solid components. The mass contained histologically distinct tumorous components with no histologic admixure at the interfaces. The densely calcified nodule corresponded to osteosarcoma, and the noncalcified clearly enhancing nodules to leiomyosarcoma, liposarcoma and fibrosarcoma.

Fibrosarcoma↗

Fatal tumor thrombosis due to an inferior vena cava leiomyosarcoma in a patient with antiphospholipid antibody syndrome.

We describe a patient with antiphospholipid antibody syndrome (APS) who died because of relentless inferior vena cava (IVC) tumor thrombosis due to an unsuspected leiomyosarcoma. Laboratory confirmation for APS was provided by functional identification of a lupus anticoagulant and anticardiolipin IgG and anti-beta2-glycoprotein I IgM antibodies. Although sensitive for detecting vascular obstruction, radiocontrast venography and magnetic resonance imaging and angiography detected the IVC thrombosis but failed to distinguish its malignant nature. Concomitant refractory thrombocytopenia prevented further invasive diagnostic and therapeutic maneuvers for progressive, severe IVC thrombosis unresponsive to aggressive treatment of APS. Deep venous thrombosis refractory to anticoagulant and immunomodulatory therapies in a patient with APS may be due to a concomitant underlying malignancy, such as a leiomyosarcoma, causing vascular obstruction.

Angiography↗

Leiomyosarcoma of the esophagus in a patient with chagasic megaesophagus: case report and literature review.

Leiomyosarcoma constitutes approximately 0.5% of the malignant neoplasias of the esophagus and its association with megaesophagus has not been described. We report on a case of a woman with dysphagia that was slowly progressive from the age of 19 due to chagasic megaesophagus. The woman was subjected to cardiomyotomy at the age of 49. She presented a rapid worsening of the dysphagia due to leiomyosarcoma at the age of 61, and was subjected to subtotal esophagectomy with cervical esophagogastroplasty. She developed pulmonary and hepatic metastases 14 months after surgery and died six months later.

Chagas Disease↗

[Gastrointestinal bleeding caused by small bowel leiomyosarcoma].

The small bowel leiomyosarcoma is a infrequent tumour in the clinical practice. We report the case of a 50 year old male admitted to the hospital on two occasions for gastrointestinal bleeding. On the second occasion a push enteroscopy located in jejunum a tumour. The pathologic diagnosis was a low grade jejunal leiomyosarcoma. The patient required a surgical intervention with resection of the affected portion of the jejunum.

Gastrointestinal Hemorrhage↗

Gastric leiomyosarcoma associated with hypoglycemia in a dog.

Hypoglycemia associated with a large, gastric leiomyosarcoma was diagnosed in an 11-year-old, yellow Labrador retriever. The dog had persistent hypoglycemia and low serum insulin levels. Although radiological and ultrasonographical evaluations suggested an abdominal mass involving the liver, a large, nonresectable, gastric mass was found during exploratory laparotomy. Metastasis was not identified on gross inspection of the liver or abdomen, and no metastatic lesions were seen on thoracic radiographs. The dog was euthanized. Histopathological examination of the mass revealed a poorly differentiated, gastric leiomyosarcoma that extended into the mesentery without regional lymph-node involvement.

Animals↗

Concomitant well-differentiated adenocarcinoma and leiomyosarcoma of the uterus.

We describe a case of a concomitant well-differentiated endometrial endometrioid adenocarcinoma and leiomyosarcoma of the uterus in a 66-year-old woman who presented with a 6-month history of vaginal bleeding. The patient underwent total hysterectomy for endometrial carcinoma diagnosed by endometrial biopsy. Gross examination of the specimen revealed an endometrial mass bulging into the endometrial cavity and an underlying well-circumscribed nodule separated from the endometrial mass by a myometrial band. Frozen section performed at the time of the total hysterectomy rendered a diagnosis of malignant mixed-müllerian tumor. Histologic examination of the permanent sections revealed well-differentiated endometrial endometrioid adenocarcinoma clearly separated from a high-grade leiomyosarcoma. Differential diagnosis included malignant mixed-müllerian tumor. However, no admixture of carcinomatous and sarcomatous elements was present. There were no heterologous elements. To the best of our knowledge, no similar case has been described in the English literature.

Aged↗

A parathyroid hormone-related protein-secreting metastatic epithelioid leiomyosarcoma. A case report and review of the literature.

A diagnosis of parathyroid hormone-related protein (PTH-rP)-secreting metastatic uterine epithelioid leiomyosarcoma was made in a 61-year-old woman with humoral hypercalcemia of malignancy. A primary uterine tumor had been removed 10 years previously, which had been associated with a short history of hypercalcemia. The original uterine tumor was diagnosed as a smooth muscle tumor of uncertain malignant potential. To the best of our knowledge, this is the first reported case of a PTH-rP-secreting uterine leiomyosarcoma. We demonstrate the dramatic changes in serum calcium, phosphorus, PTH, and PTH-rP levels after tumor resection. Extensive biochemical analysis and detailed immunohistochemical and ultrastructural characterization demonstrate several features of this tumor.

Calcium↗

[A case of gastrointestinal stromal tumor of rectum, difficult to differentiate from leiomyosarcoma of prostate].

A 70 year-old male was seen at the hospital with the chief complaints of frequent miction and incomplete urinary retention. A hen's egg-sized firm mass was palpable in anterior wall of rectum by digital rectal examination. Intravenous urography showed severe bilateral hydronephroureter. Transrectal ultrasound, CT scan and MRI revealed a mass with 5 cm in diameter between prostate and rectum, and the margin of them were unclear. On needle biopsy of the tumor, leiomyosarcoma of the prostate was suspected. We performed radical cytectomy and created continent urinary reserver. Because the tumor and rectum could not be lysed, part of the rectum was resected. Histological examination showed gastrointestinal stromal tumor (GIST) of rectum. GIST of rectum is a rare entity, and in case of contact with the prostate, it is difficult to differentiate from leiomyosarcoma of prostate.

Aged↗

[A case of leiomyosarcoma of the testis].

A 64-year-old man presented with the painless hard swelling of left scrotal content. Left orchiectomy with high ligation of the spermatic cord was performed with clinical diagnosis of testicular tumor. Histopathologically, it was diagnosed as leiomyosarcoma arising from the spermatic cord. There has been neither local recurrence nor metastasis for 5 months after operation. This is the 20th case of leiomyosarcoma of the spermatic cord in Japan.

Humans↗

[Renal leiomyosarcoma. Apropos a case].

OBJECTIVE: To describe an additional case of leiomyosarcoma of the kidney in a patient that presented with nonspecific abdominal pain and a palpable mass. The clinical features, diagnosis and therapeutic aspects, as well as the prognostic factors of this tumor type are reviewed. METHODS: Patient evaluation included US, CT and MRI. The patient underwent a left radical nephrectomy and received adjuvant external radiotherapy. RESULTS: The patient is asymptomatic and has no local recurrence or metastasis 15 months postoperatively. CONCLUSIONS: Leiomyosarcoma of the kidney is an uncommon malignant mesenchymal tumor. To our knowledge, less than 100 cases have been reported in the literature. Its clinical features and the US, CT and MRI findings are not unlike those of renal adenocarcinoma. The treatment of choice is by radical nephrectomy.

Adult↗

[Rectal leiomyosarcoma. 4 new cases].

We report four cases of leiomyosarcoma of the rectum suspected by endoscopic ultrasonography. Three patients were treated by local excision and one by abdominoperineal resection. An excision of the mass via a Kraske's approach was used. Leiomyosarcoma confined to the rectum wall can be treated by local excision. Endosonography can provide exact estimation of the lesion and is of great value in selecting the appropriate treatment. The treatment is surgical excision with wide margins. The histological stage and the presence or absence of metastases determine the therapeutic. Two patients in our series underwent radiation therapy. Chemotherapeutic agents including doxorubicin have had beneficial effect on recurrence or survival, only for higher grade sarcomas.

Aged↗