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Primary leiomyosarcoma of the skin.

Leiomyosarcomas of the skin are frequently misdiagnosed clinically. This tumour however, exhibits typical histological and immunohistochemical features. This collection of seven cases of cutaneous leiomyosarcoma on the trunk and limbs of adults with a mean age of 63 years and a range of 49-78 years demonstrates that the clinical diagnosis in each case, from seven different clinicians, did not include the correct diagnosis. In each case, the special immunohistochemical stains were helpful in making the diagnosis with actin and vimentin being positive and S100 and prekeratin stains being negative.

Adult↗

Incidental computed tomography diagnosis of a giant oesophageal leiomyosarcoma.

Leiomyosarcoma of the oesophagus is a very unusual tumour; only 53 cases have been reported in the English-language literature. A case is reported here of a patient with a giant leiomyosarcoma, without any symptoms of dysphagia. The diagnosis was made incidentally during CT examination of the chest for detection of possible pulmonary metastases from a coexisting carcinoma of the bladder. This was confirmed by a barium swallow, oesophagoscopy and tissue diagnosis. The literature pertaining to this most uncommon tumour is reviewed.

Aged↗

Leiomyosarcoma of the inferior vena cava.

Leiomyosarcoma of the inferior vena cava is a rare primary tumour. We present a case report of a 67-year-old man with a long history of abdominal pain and gastroesophageal reflux, who was found to have a large retroperitoneal mass confirmed to be a leiomyosarcoma. The clinical and imaging features are outlined, and in addition the treatment and prognosis.

Aged↗

Leiomyosarcoma of the great saphenous vein.

Peripheral vascular leiomyosarcomas are rare. A case of leiomyosarcoma of the great saphenous vein diagnosed pre-surgically by MRI and fine-needle aspiration is presented. Characteristics of the tumour and imaging features are discussed.

Biopsy, Fine-Needle↗

Primary leiomyosarcoma arising in the trunk of pulmonary artery: a case report and review of literature.

An autopsy case of a 46-year-old woman with primary leiomyosarcoma arising in the pulmonary artery is presented. This case is the first description in Japan and the 10th in the world literature as leiomyosarcoma in this part of the body. An electronmicroscopic observation of the postmortem material was useful to conform the histologic diagnosis, in addition to some kinds of staining method of histologic section.

Female↗

A comparative electron microscopic study of leiomyosarcoma and leiomyoma of the duodenum.

Comparison of ultrastructural features between leiomyosarcoma and leiomyoma of the duodenum was made. There was no absolute difference between the two, but the following ultrastructural features of leiomyosarcoma were different from leiomyoma: (1) occasional conglomerations of mitochondria surrounded by granular endoplasmic reticulum; (2) quite rare presence of single cilia; (3) frequent occurrence of intranuclear inclusion bodies; (4) variability in nuclear size and shape; and (5) wide interstitial space with scanty collagen fibrils. Although both tumors were considered to be of smooth muscle origin, their ultrastructural features were distinctively different from the normal smooth muscle in some points. In addition to the light microscopic findings, ultrastructural features were considered to be valuable for distinguishing the malignant from the benign smooth muscle tumors in the gastrointestinal tract.

Cell Membrane↗

Prostatic leiomyosarcoma revealing cytoplasmic virus-like particles and intranuclear paracrystalline structures.

This report concerns an ultramicroscopic study of an autopsy case of primary leiomyosarcoma of the prostate in a patient who died of widespread blood-borne metastases after a rapid clinical course. Ultrastructural observation of tumor cells demonstrated some characteristics of leiomyosarcoma and furthermore revealed frequent intranuclear structures in paracrystalline array together with aggregates of interchromatin granules and cytoplasmic virus-like particles.

Adult↗

Leiomyosarcoma of pulmonary truncus.

Insidious and progressive right heart failure of obscure origin warrants differential diagnosis of primary sarcoma of the pulmonary artery in patients of both sexes and 20 years of age and older. We report a case of primary leiomyosarcoma of the pulmonary truncus in a 67 years old woman. She had been well in general until when she started having fluctuating and progressive right heart failure three years ago. Clinical data included systolic murmurs in the pulmonary valve area, right ventricular hypertrophy, diminished x-ray density of the pulmonary vasculature, moderate elevation of serum LDH, alkaline phosphatase, and uric acid, and prolonged or unobtainable circulation time from the arm to lungs. The immediate cause of death was attributable to right heart failure due to pulmonary arterial stenosis caused by a locally invasive leiomyosarcoma apparently arising in the pulmonary truncus. Contributing to this right heart failure were 280 ml of serosanguinous pericardial effusion and fibrinous pericarditis.

Aged↗

Gastric leiomyosarcoma with massive myxoid degeneration. A histochemical and ultrastructural study.

Histochemical and ultrastructural features of a gastric myxoid leiomyosarcoma from a 55-year-old man were examined. At autopsy, the tumor was located mainly in the greater omentum and was directly connected to a coinsized gastric tumor. Multiple hepatic metastases and peritoneal disseminations were noted. Light-microscopically, the tumor was composed of a prominent myxoid stroma and ovoid or rounded tumor cells. The myxoid stroma was stained weakly basophilic with hematoxylin and eosin, and was mainly composed of hyaluronic acid. Tumor cells in the stomach were spindle-shaped and apparently myogenic. Ultrastructurally, the gastric tumor cells showed a loose cohesion with junctional apparatuses, pinocytotic vesicles, basal laminae, and cytoplasmic filaments with focal densities. Tumor cells in the omentum and the liver, however, were poorly differentiated and showed an epithelioid nature in part. This unique leiomyosarcoma is reported with some differential diagnoses from other myxoid sarcomas.

Chondrosarcoma↗

Primary leiomyosarcoma of the inferior vena cava with Budd-Chiari syndrome.

A 44-year-old Japanese woman with leiomyosarcoma of the inferior vena cava is reported. She presented with Budd-Chiari syndrome and died of hepatic failure about 3 months after the onset of symptoms. The tumor arose from the middle segment of the inferior vena cava, occluded the inferior vena cava and projected into the right atrium. A total of 28 cases of Budd-Chiari syndrome due to primary leiomyosarcoma of the inferior vena cava are reviewed.

Adult↗

Two autopsy cases of primary leiomyosarcoma of the liver. Superiority of muscle-specific actin immunoreactivity in diagnosis.

Two autopsy cases of leiomyosarcoma of the liver in a 49-year-old female and 63-year-old male are reported. Both of the liver tumors showed electron microscopically dense patches in the cytoplasm and intermediate junctions between the tumor cells, suggesting a smooth muscle cell origin, irrespective of their different histological features. The nature of both tumors was confirmed by positive immunoreactivity for muscle-specific actin in the tumor cells, whereas desmin immunoreactivity was labile in both cases, showing a higher diagnostic value of the former compared with the latter in these leiomyosarcomas. Both cases, showed extensive distant metastases in spite of an evident difference in their mitotic indices, indicating that this index is not reliable for judging the metastatic potential of these tumors.

Actins↗

Histological and immunohistochemical observations of dedifferentiated leiomyosarcoma of the uterus.

A case of dedifferentiated leiomyosarcoma of the uterus was examined using immunohistochemistry. The tumor arose in the myometrium, and was a whitish large nodule with hemorrhage and necrosis. Histologically it was a well differentiated leiomyosarcoma with foci showing epithelioid pattern, and in part resembling malignant fibrous histiocytoma (MFH) and giant cell tumor (GCT). Additionally, small round neoplastic cells arranged in an alveolar manner, simulating alveolar rhabdomyosarcoma, were seen in some areas. Neoplastic cells in well differentiated areas expressed desmin, muscle-specific actin and LeuM1, whereas those in epithelioid and poorly differentiated areas lacked these antigens. Instead, tumor cells in epithelioid and small round cell areas were positive for keratin. Interestingly, most tumor cells in well differentiated, epithelioid and small round cell areas were also positive for MB1. However, tumor cells in GCT- and MFH-like areas reacted with none of the antibodies used. Ultrastructurally, some tumor cells possessed various amounts of microfilaments with or without dense patches, whereas others lacked them. These findings suggest that the divergent antigen expression was attributable to different levels of differentiation, and that poorly differentiated components had lost their native features.

Actins↗

Pulmonary myxoid leiomyosarcoma.

A primary myxoid leiomyosarcoma arising from the peripheral bronchus of the right middle lobe was removed from a 20 year old man and examined by immunohistochemistry and electron microscopy. The tumor was well circumscribed, yellow-whitish focal polypoid growth into the bronchial lumen and consisted of spindle cells with abundant myxoid substance in the stroma. Intrapulmonary metastasis, invasion to the bronchial wall and a few mitotic figures were found. Immunohistochemically, several, but not all, tumor cells were positive against anti-vimentin, anti-S-100 protein, anti-myosin and anti-muscle specific actin. Ultrastructurally, tumor cells had thin filaments with dense bodies, pinocytic vesicles and discontinuous basal lamina. These findings indicate a myoxoid variant of leiomyosarcoma arising from mesenchyme in the peribronchial area.

Adult↗

Uterine leiomyosarcoma with osteoclast-like giant cells: histopathological and cytological observations.

A 56 year old woman presented with abnormal uterine bleeding. Except for a myomatous uterus, no other abnormalities were noted on physical examination and in radiographic and serologic studies. The hysterectomy specimen revealed an 8 cm uterine fundic tumor composed of two histologically different patterns that merged with one another; one was a well differentiated leiomyosarcoma and the other a mixture of osteoclast-like giant cells (OGC) and plump spindle cells whose cell borders blended, resembling the histology of giant cell tumor of bone. Immunohistochemical studies showed positive staining for muscle actin, alpha-smooth muscle actin, and KP-1 (CD68) in both the spindle cells and OGC. The latter also stained for alpha-1-antitrypsin and alpha-1-antichymotrypsin. These findings suggested that OGC may be formed by the fusion of spindle cells of leiomyosarcoma and also express histiocytic markers.

Female↗

Metastizing low-grade clear cell leiomyosarcoma of the uterus.

An unusual case of uterine clear cell tumor is reported in a 56-year-old woman. The neoplasm was identified as a peculiar low-grade leiomyosarcoma, composed mainly of watery, clear large cells with round, fairly regular nuclei. No appreciable pleomorphism or high mitotic activity was noted. There was no necrosis. The presence of occasional areas of transition between these cells and typical spindle leiomyosarcoma cells, together with the immunohistochemical results, allowed the recognition of a smooth muscle origin of this clear cell tumor. The patient was treated by hysterectomy. Six years later she developed an inguinal metastasis resembling an hibernoma, and then later developed a large retroperitoneal metastasis, which was composed entirely of clear cells and was distinguished from a liposarcoma by immunohistochemistry.

Actins↗

Retroperitoneal leiomyosarcoma of a male centenarian.

An autopsy case of retroperitoneal leiomyosarcoma of a centenarial male is described. He complained of abdominal pain in his left side 1 month before his death. Ultrasonography and computed tomography revealed a large mass in the left abdominal cavity. At autopsy, a circumscribed mass, 15 cm in diameter, was found in the left-upper abdominal cavity, and seemed to be derived from the retroperitoneum in an exophytic manner. Histologically, spindle cells with slight eosinophilic cytoplasm and blunt-ended nuclei showed interwoven fascicular growth, and mitotic figures were easily encountered. Immunohistochemically, the tumor cells were labeled by alpha-smooth muscle actin and vimentin. To our knowledge, this case of leiomyosarcoma has presented in the oldest patient ever reported. The rarity of soft tissue sarcomas in the extremely elderly is also discussed.

Aged↗

Dumbbell-shaped leiomyosarcoma of the inferior vena cava with foci of rhabdoid changes and osteoclast-type giant cells.

An inferior vena cava (IVC) tumor was incidentally found in a 67-year-old Japanese man. The resected tumor was lobulated and multinodular, measuring 14.0 x 6.5 x 7.0 cm, showing a dumbbell-shaped appearance with a central constriction. The tumor showed both intra- and extra-luminal growth. The tumor was primarily composed of well-differentiated leiomyosarcoma. Spindle tumor cells in the well-differentiated area were positive for vimentin, muscle actin, alpha-smooth muscle actin, and desmin. Foci of rhabdoid cells and osteoclast-type multinucleated giant cells were also found. Rhabdoid cells ultrastructurally had paranuclear aggregates or whorls of intermediate filaments that were positive for vimentin, low molecular weight cytokeratin, and desmin. Osteoclast-type multinucleated giant cells were positive for only CD68 antigen, suggesting a reactive histiocytic lineage. To the best of our knowledge, this is the first case of IVC leiomyosarcoma accompanied by both rhabdoid tumor cells and osteoclast-type reactive multinucleated giant cells. These unusual features should be kept in mind in the diagnosis of dumbbell-shaped retroperitoneal tumors that involve the IVC.

Aged↗