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Case report: a long-term survivor of jejunal leiomyosarcoma with liver metastasis: effective transcathetel arterial embolization for hepatic metastatic foci.

We report a case of jejunal leiomyosarcoma with liver metastases in a 52-year-old Japanese male. An echogram demonstrated multiple cystic liver masses in April 1991. The diagnosis of metastatic leiomyosarcoma was made on the basis of characteristic hepatic angiography images and liver biopsy findings. The jejunal leiomyosarcoma was resected and unresectable liver metastatic foci were treated repeatedly with transcathetel arterial embolization. Transcathetel arterial embolization was considered to be effective in this case as the patient survived 4 years and 9 months after the first treatment.

Catheterization↗

Leiomyosarcoma of the spermatic cord. A case report and a brief review of literature.

A case of leiomyosarcoma of the spermatic cord, occurring in a 79-year-old male patient, is reported. The tumor was resected with high inguinal ligation and orchiectomy. It was located at the scrotal part of the spermatic cord on the left side and was shown to have originated from the spermatic duct using the dissecting method. Histologically, it was identical to leiomyosarcoma showing a typical morphological appearance of smooth muscle cell tumor in the conventional histochemical preparations as well as a focal positive reaction to antidesmin antibody, a marked nuclear pleomorphism, and abundant mitotic figures. The patient had been untroubled for six months after the resection of the tumor. Leiomyosarcoma arising in spermatic cord is quite rare.

Aged↗

An autopsy case of pulmonary artery leiomyosarcoma.

A case of pulmonary artery leiomyosarcoma in a 37 year old Japanese man is reported. The patient complained of chest pain and hemosputum. CT scan and angiography revealed that a tumor arose from the right main branch of the pulmonary artery. The surgical specimen was interpreted as an undifferentiated sarcoma at this time. The tumor recurred 6 months after the resection, and he died of right-sided heart failure. At autopsy, the pulmonary artery truncus was distended by the tumor, which protruded into the lumen. The tumor was composed of pleomorphic cells, giant cells and spindle-shaped cells. Many of the tumor cells were immunohistochemically positive for alpha-smooth muscle actin and vimentin. Electron microscopy revealed microfilaments with dense bodies in the cytoplasm and a discontinuous basement membrane around the cells. Although adenocarcinoma was also observed in the colon, the authors conclude that the present tumor is not a metastasis but a primary leiomyosarcoma arising from the pulmonary artery. Imaging techniques were very useful for differentiating sarcoma from thrombus in this site, and immunohistochemical demonstration for alpha-smooth muscle actin was necessary for making a histological diagnosis of leiomyosarcoma.

Actins↗

Leiomyosarcoma of the superior vena cava producing superior vena cava syndrome and heart tamponade.

An autopsy case of primary leiomyosarcoma arising in the superior vena cava is presented. A 44 year old Japanese man presented with superior vena cava syndrome and eventually died due to heart tamponade and acute renal failure. Autopsy revealed that the superior vena cava was occluded with a tumor that had invaded the pericardium and right thoracic cavity. Primary caval venous leiomyosarcoma is a rare but lethal disease and most cases arise from the inferior vena cava. This case represents a very rare case of leiomyosarcoma with the rare clinical findings of superior vena cava syndrome and heart tamponade.

Adult↗

Primary leiomyosarcoma of the vertebra: Case report and review of the literature.

A case of primary leiomyosarcoma of a thoracic vertebra associated with a compression fracture in a 75-year-old woman was preoperatively thought to be granulation tissue. Surgical decompression was performed and the histological and immunohistochemical studies established the diagnosis of leiomyosarcoma. Based on the clinical and radiological examinations, metastases were ruled out. Primary leiomyosarcoma of the vertebra is extremely rare and in that site it is considered to have a relatively poor prognosis.

Aged↗

Differential alpha-smooth muscle actin expression in a gastric epithelioid leiomyosarcoma in a 14 year old girl.

Leiomyosarcoma in childhood is very rare. This report describes the immunohistochemical study of a gastric epithelioid leiomyosarcoma in a 14 year old girl. Microscopically, the tumor showed two distinct histological patterns within the same tumor: small epithelioid cells forming a lobular pattern and spindle-shaped cells forming interlacing fascicles, but no distinct border divided the two types of cells. Immunohistochemically, vimentin was positive in all tumor cells; alpha-smooth muscle actin, however, was negative in small epithelioid cells and positive in spindle-shaped cells. The data revealed that the tumor had components showing two stages of morphological and biochemical differentiation. This case indicates that leiomyosarcomas might lose smooth muscle actin during malignant transformation; or, alternatively, they might arise from a primitive type of smooth muscle cell and some of them could spontaneously differentiate into mature smooth muscle cells, expressing this actin.

Actins↗

Primary testicular leiomyosarcoma.

We report a case of primary testicular leiomyosarcoma. A 76-year-old man, who complained of left scrotal swelling for 6 months, underwent radical orchiectomy. Microscopic examination revealed intratesticular leiomyosarcoma. The patient received no adjuvant therapy and at 12 months after surgery, there had been no recurrence. Primary leiomyosarcoma of the testis is extremely rare; this is the 9th case recorded worldwide.

Aged↗

A case of leiomyosarcoma of the penis.

A case of leiomyosarcoma of the penis is reported. A 27-year-old-man presented to our department with a mass at the root of the penis. Biopsy of the tumor showed that the tumor was leiomyosarcoma. The tumor was clinically and pathologically categorized into the deep type. Despite total penectomy and adjuvant chemotherapy, the patient died from disseminated disease 14 months after surgery. This is the 45th case of penile leiomyosarcoma.

Adult↗

Primary subcutaneous leiomyosarcoma exhibiting the characteristic immunophenotype positive for vimentin and desmin.

BACKGROUND: Primary subcutaneous leiomyosarcoma is a rare neoplasm composed of plump, elongated spindle cells arranged in interweaving fascicles. OBJECTIVE: Differential diagnosis might be facilitated by the use of immunohistochemistry. METHODS: A case of subcutaneous leiomyosarcoma was investigated histologically on paraffin-embedded tissue and immunohistochemistry was performed following standard procedures. RESULTS: The case reported exhibited light microscopic features and an immunophenotype characteristic of leiomyosarcoma. However, the intermediate filament desmin could only be found on a small number of tumor cells. CONCLUSION: Expression of these markers might vary considerably and immunoperoxidase stainings need to be carefully evaluated. Utilization of several antibodies directed against different desmin epitopes might be advantageous.

Aged↗

Leiomyosarcoma of the broad ligament: a case report and review of literature.

Leiomyosarcoma of the broad ligament is a rare tumor, since only 12 cases have been reported so far in the literature. A 53-year-old patient was diagnosed with leiomyosarcoma of the broad ligament at the Department of Gynecology Oncology of the National Cancer Research Institute in Genoa. The tumor had low mitotic activity and less than ten mitotic figures were found for ten high-power fields. The treatment consisted of total abdominal hysterectomy and bilateral salpingo-oophorectomy with bilateral ureteral neoanastomosis and omentectomy. The patient has not received either radiotherapy or chemotherapy, considering the low grade of malignancy, but she is only followed up on an outpatient basis. No evidence of metastasis has been noted after a follow-up of 13 months. It is concluded that low-grade leiomyosarcoma of the broad ligament should be treated only with surgery.

Broad Ligament↗

Massive myxoid leiomyosarcoma of the uterus.

Uterine myxoid leiomyosarcoma is a very rare and aggressive variant of uterine sarcoma, of which only 26 cases have been described in the literature published in English. A 59-year-old woman, who had been menopausal for 5 years and had a huge abdominal mass underwent laparotomy; histopathology of the bulky tumor revealed the particular characteristics of a myxoid leiomyosarcoma. Immunohistochemically, Ki-67 contributed toward indicating a malignancy, whereas p53 and progesterone and estrogen receptors were negative. The level of serum Ca125 was high at diagnosis, within normal limits after the fifth cycle of chemotherapy, and subsequently increased again at recurrence. Sixteen months after surgery, the patient died of respiratory failure. Immunohistochemistry and Ca125 levels can aid the diagnosis, management, and prognosis of uterine myxoid leiomyosarcoma.

CA-125 Antigen↗

Primary leiomyosarcoma of skin: a report and critical appraisal.

A primary leiomyosarcoma of skin was studied by light and electron microscopy and by histochemistry. Systematic evaluation of the entire neoplasm suggested that a single biopsy sample would show little cellular pleomorphism but could vary considerably in number of mitoses per mm2. Electron microscopy revealed a high degree of cytologic differentiation. Strong myosin ATPase activity and negative demonstrations for hydrolytic enzymes suggest a diagnostic profile which will clearly separate this neoplasm from malignant fibrous histiocytoma. High mitoses counts, the conventional criterion for malignancy of non-cutaneous smooth muscle tumors, may not be appropriately applied to primary leiomyosarcomas arising in the dermis. The findings in this case and a critical review of the literature suggest that reliable criteria for diagnosis of primary cutaneous leiomyosarcoma by light microscopy remain to be established.

Adenosine Triphosphatases↗

Leiomyosarcoma of the inferior vena cava presenting as Budd-Chiari syndrome.

A case of a 72-year-old male with a leiomyosarcoma of the inferior vena cava causing Budd-Chiari syndrome is described. Percutaneous decompression of the portal system was attempted but with no success. At autopsy, a large tumor arising from and completely obstructing the inferior vena cava was found, histologically proven to be a leiomyosarcoma. Over 50 cases of leiomyosarcoma of the inferior vena cava have been reported in the literature, the majority of which occurred in women and 1/3 of which were associated with the Budd-Chiari syndrome. Histologically up to two mitotic figures per 10 high power fields have been scored. This case indicates the unreliability of a low mitotic index as an indicator of benign behavior of smooth muscle neoplasms of the venous system.

Aged↗

Leiomyosarcoma of the right ventricle extending into the pulmonary trunk.

Primary leiomyosarcomas of the heart, particularly those affecting the right ventricle, are uncommon. A 70 year old woman presenting with the symptoms of progressive exertional dyspnoea and left sided pleuritic pain is presented. A leiomyosarcoma was diagnosed that originated in the right lateral ventricle wall, causing pulmonary outflow obstruction. Pathological analysis showed a neoplasm with a myxoid stroma, high mitotic activity, and nuclei expressing atypia. Immunohistochemical staining was found positive for vimentin and desmin. Seven months after complete surgical resection the tumour relapsed. This case illustrates the poor outcome, high relapse rate, and inefficiency of treatment associated with primary cardiac leiomyosarcomas. The current literature regarding incidence, diagnostic techniques, treatment strategies, and survival rates of this rare but terminal disease is reviewed.

Aged↗

Secondary leiomyosarcoma of the right ventricle. A surgical report.

A 48-year-old woman developed symptoms related to a tumour attached to the outflow tract of the right ventricle. Eleven years earlier she had undergone hysterectomy for leiomyosarcoma, and 4 years later a retroperitoneal mass was removed. One year later a further retroperitoneal mass was removed, and after a further 4 years a cutaneous tumour was removed from her back. Histologically these three tumours were identical with the leiomyosarcoma removed with the uterus at the primary operation. A confident preoperative diagnosis of recurrent leiomyosarcoma within the pericardium was made, and it was possible to remove this tumour using cardiopulmonary bypass with relief of symptoms. The patient remains well 15 months after surgery.

Female↗

Primary intratesticular leiomyosarcoma in a mixed race man: a case report.

Primary leiomyosarcoma of the testis is an extremely rare condition with only a few reported cases in the literature. None has been reported in men of Afro-Caribbean descent. This report describes the first case of a primary leiomyosarcoma in a mixed race man. Several different immunostains were used to confirm the morphological suggestion of a primary intratesticular smooth muscle tumour. Scrotal ultrasound and abdominal computed tomography imaging were used to exclude paratesticular and retroperitoneal pathology. Where reported, primary intratesticular leiomyosarcoma is described as an indolent tumour with the potential for distant metastases. Its occurrence in younger men is usually associated with anabolic steroid abuse. Although the number of reported cases is very few, radical orchidectomy followed by surveillance appears to be the treatment of choice.

Adult↗

Soft tissue, pelvic, and urinary bladder leiomyosarcoma as second neoplasm following hereditary retinoblastoma.

This report describes two patients who developed leiomyosarcomas, one involving the subcutaneous tissue of the thigh and the pelvic soft tissues and the other the urinary bladder, following hereditary retinoblastoma 36 and 38 years earlier, respectively. There is an increased risk of the development of sarcoma, most commonly osteosarcoma, as a second malignancy following hereditary retinoblastoma. Leiomyosarcoma developing as a second malignancy has rarely been reported and most have occurred in the field of previous radiotherapy. The literature on leiomyosarcoma occurring as a second neoplasm following retinoblastoma is reviewed.

Adult↗

From the archives of the AFIP. Leiomyosarcoma of the retroperitoneum and inferior vena cava: radiologic-pathologic correlation.

Leiomyosarcoma is the second most common primary retroperitoneal tumor in adults. Retroperitoneal leiomyosarcoma exhibits three major growth patterns: (a) completely extravascular (extraluminal) (62% of cases), (b) completely intravascular (intraluminal) (5% of cases), and (c) extra- and intraluminal (33% of cases). The usual clinical manifestation is a large abdominal mass. Intraluminal leiomyosarcoma may be accompanied by symptoms referable to venous thrombosis. The variable gross features and potential for intravascular extension result in various radiologic appearances, the most common being a large, partially necrotic soft-tissue mass in the retroperitoneum with or without extension into the inferior vena cava. Computed tomography and magnetic resonance imaging typically show a nonfatty, necrotic retroperitoneal mass and a vascular component when it is present. Ultrasound and angiography may also be useful, especially if vascular involvement is suspected from other imaging studies. Treatment of choice is surgical excision, which is frequently impossible due to the invasiveness of the tumor. Prognosis is related to extent of invasion and the adequacy of resection. Long-term prognosis is poor, and most patients die of local recurrence or distant metastasis.

Angiography↗