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Peritoneal leiomyosarcomatosis originating from gastrointestinal leiomyosarcomas: CT features.

PURPOSE: To evaluate the computed tomographic (CT) features of peritoneal leiomyosarcomatosis (PL) originating from gastrointestinal leiomyosarcoma and compare the CT features of primary gastrointestinal leiomyosarcomas between patients with and those without PL. MATERIALS AND METHODS: The authors reviewed the medical and surgical-pathology records and the CT scans of 89 patients with gastrointestinal leiomyosarcoma to determine the prevalence of PL. Also, the CT scans of the patients with PL were evaluated for the morphologic appearance of peritoneal lesions. The CT features of the primary tumors were compared between the patients with and those without PL. Student t and Fisher exact tests were performed. RESULTS: Metastases were present in 39 patients. PL was seen in 25 patients. Other metastatic sites included liver in 26 patients, lymph nodes in 11, lungs in three, and bone in one. In the 25 patients with PL, CT findings included multiple discrete peritoneal nodules in 22 patients and massive peritoneal lesions in three. Smudged omental infiltration was combined with other findings in 10 patients. The sites of the peritoneal masses were mesentery in 18 patients, omentum in 11, paracolic gutters in 11, pelvic cavity in eight, perihepatic space in three, and perisplenic space in one. Four patients had ascites. There were statistically significant differences in size of primary tumor (mean diameter, 12.5 cm vs 9.2 cm) (P =.01) and prevalence of exophytic growth of primary tumor (P =.02) between the patients with and those without PL. CONCLUSION: CT findings can indicate PL in the differential diagnosis. The prevalence of PL appears to be higher when it originates from large primary tumors.

Adult↗

Liver resections for metastases from intraabdominal leiomyosarcoma.

This paper discusses liver resection for intraabdominal leiomyosarcoma metastases as a therapy for carefully selected patients. Of the 83 hepatectomies performed from 1992 to 1996, five were resections for liver metastases due to intraabdominal leiomyosarcoma, in 3 patients. The surgical indication was single liver metastases, without any evidence of extrahepatic disease. No mortality occurred during surgery and the longest survival was 38 months. We concluded that liver resection for leiomyosarcoma metastases can be performed, allowing a long term survival in an occasional patient.

Abdominal Neoplasms↗

Primary endobronchial leiomyosarcoma. Diagnosis following expectoration of tumor fragment.

A case is presented with spontaneous expectoration of a small piece of solid tissue. Pathologic examination of the expectorated tissue was found to be consistent with leiomyosarcoma. After further work-up, there was no evidence of another primary site of leiomyosarcoma except for the right lower lobe. Right lower lobectomy was performed. The surgical specimen showed a tumor that was histologically identical to the patient's previous expectorated tissue. To the authors' knowledge, this is the first report of partial expectoration of a primary endobronchial leiomyosarcoma.

Biopsy, Needle↗

Leiomyosarcoma complicating chronic inflammation of the testis.

OBJECTIVE: To report on a case of leiomyosarcoma of the testis that appeared to have arisen from a background of chronic testicular inflammation. CLINICAL PRESENTATION: A 65-year-old man with a 15-year history of diabetes mellitus and low-grade discomfort and swelling in the right testis presented as an emergency with exacerbation of the pain and swelling of the testis. Repeated ultrasound examination of the testis in the past 5 years had suggested a chronic testicular inflammatory disorder. Ultrasound during the current emergency case admission revealed a normal left testis, but a large heterogeneous solid mass with a moderate intratesticular calcification in the right testis and the presence of a moderate hydrocele. Serum alpha-fetoprotein and beta-human chorionic gonadotropin were normal. A right radical orchidectomy was performed. Histopathology and immunohistochemistry revealed primary leiomyosarcoma of the right testis. There was no spermatic cord or venous involvement. One year after orchidectomy there was no sign of metastasis. CONCLUSION: Radical orchidectomy followed by surveillance appears to be the treatment of choice for this testicular leiomyosarcoma, which seemed to have run an indolent course compared to other testicular tumours.

Aged↗

Development of leiomyosarcoma from primary leiomyoma?

Kidney leiomyosarcoma represents a rare variety of malignant kidney tumours. In this paper, we report on a patient with an inoperable leiomyosarcoma. Since this neoplasm is very rare, there is very little information on this type of malignancy. We present the symptoms, radiological findings, diagnostic criteria and differential diagnosis of the tumour. Leiomyosarcoma exhibits aggressive biological behaviour and has a poor prognosis. We have found that the treatment of choice is a radical nephrectomy.

Cell Transformation, Neoplastic↗

'Spontaneous' regression of advanced leiomyosarcoma of the urinary bladder.

A case of advanced leiomyosarcoma of the urinary bladder is reported in a 25-year old man who, in a short time, experienced a complete 'spontaneous' regression of his fatal illness. He first presented with severe haemoperitoneum resulting from an unresectable bleeding tumour of the urinary bladder. Debulking surgery was performed, followed by salvage chemotherapy. Histological and ultrastructural examinations of the tumour confirmed a poorly differentiated leiomyosarcoma. The residual disease failed to respond to salvage chemotherapy, but regressed 'spontaneously' 5 months after cessation of therapy. The patient is alive without evidence of disease 51 months after the diagnosis. This remarkable phenomenon and relatively long survival in a poor-risk leiomyosarcoma of the urinary bladder has never been reported previously.

Adult↗

Subcutaneous leiomyosarcoma developing in a radiation dermatitis.

A case of subcutaneous leiomyosarcoma developing in a skin affected by radiation dermatitis is presented. A 59-year-old patient had been suffering from radiation dermatitis induced by radiotherapy for a mediastinal tumor diagnosed at 24 years of age. Thirty-four years later, slow growing nodules developed in this lesion. Light and electron microscopic studies revealed that the tumor was a subcutaneous leiomyosarcoma. There is no previous report of a subcutaneous leiomyosarcoma developing in radiation dermatitis.

Dermatitis↗

Primary pulmonary leiomyosarcoma. Report of a case diagnosed by fine needle aspiration cytology.

BACKGROUND: Primary pulmonary leiomyosarcoma is a rare but important entity. We report a case diagnosed by fine needle aspiration cytology. CASE: A 73-year-old male presented with an asymptomatic, right, pulmonary, subpleural nodule detected by computed tomography during follow-up for chronic obstructive pulmonary disease. Fine needle aspiration cytology showed cellular smears with numerous single or loosely cohesive groups of spindle-shaped to round cells. The tumor cell nuclei were blunt ended (cigar shaped), with fine to fine-granular chromatin, prominent nucleoli and an irregular nuclear rim. The tumor cells were positive for desmin and negative for cytokeratin and S-100 protein by immunocytochemistry. Right upper lobectomy with lymph node dissection was performed. Pathologic diagnosis after microscopic, immunohistochemical and electron microscopic studies was leiomyosarcoma. CONCLUSION: To our knowledge, this is the first reported case of primary pulmonary leiomyosarcoma arising in the subpleural region diagnosed by fine needle aspiration cytology. Immunocytochemistry was useful in establishing the diagnosis in this case.

Actins↗

Myxoid leiomyosarcoma of the uterus. Report of a case with cytologic findings.

BACKGROUND: Myxoid leiomyosarcoma is a rare variant of uterine sarcoma, exhibiting malignant biologic behavior despite the absence of cytologic atypia and of significant mitotic activity. CASE: A 20-year-old female was referred with a cystic pelvic mass. At laparotomy, the tumor, weighed 2,200 g and originating in the left lateral uterine wall, was removed. Microscopic examination revealed well-differentiated smooth muscle cells without atypia and with a few mitotic figures in the copious myxoid matrix, suggesting myxoid leiomyosarcoma. Three years following laparotomy, an irregular mass around the uterus was noted on sonographic examination, suggesting local recurrence. Two years and six months later, the second operation was performed, and a locally recurrent, multicystic tumor weighing 3,500 g was excised. The histopathology was similar to that of the primary tumor. Cytologic findings on imprint material from the tumor revealed a few isolated or sheet like small cells consisting of spindle and polygonal cells with round and oval nuclei. Cytologic atypia was also minimal. CONCLUSION: Myxoid leiomyosarcoma should be included in the differential diagnosis of smooth muscle neoplasia.

Adult↗

Fine needle aspiration cytodiagnosis of leiomyosarcoma of the breast. A case report.

BACKGROUND: Leiomyosarcoma of the breast is a rare tumor. Here we present a case in an elderly female in which the diagnosis was suggested from an aspirate sample. CASE: An 80-year-old female presented with an irregular, firm mass in the left breast of a few months' duration. In view of the clinical suspicion of a tumor, fine needle aspiration was performed. It showed a large number of dissociated cells and compact sheets of spindly and round cells with pleomorphic, hyperchromatic and anaplastic nuclei; mitoses; nucleoli; and somewhat-vacuolated, eosinophilic cytoplasm. Examination of the cell block, tumor tissue and immunostaining further suggested the cytologic impression of a leiomyosarcoma. CONCLUSION: Although leiomyosarcoma of the breast is very rare, fine needle aspiration cytology may allow the diagnosis to be suggested. Correlation with cell block findings and the application of appropriate immunostains as an adjuvant to standard cytologic and histologic stains may allow a more confident diagnosis.

Aged↗

Fine needle aspiration cytology of gastric epithelioid leiomyosarcoma metastasized to the liver. A case report.

BACKGROUND: There have been only a few reports on fine needle aspiration (FNA) cytology of epithelioid leiomyosarcoma, especially of the stomach, and a summary of the cytologic findings in this tumor is needed. CASE: A case of epithelioid leiomyosarcoma of the stomach metastasized to the liver and was composed cytologically of peculiar binucleated cells. CONCLUSION: Similar findings in most cases are that the cells are round or polygonal, with eccentrically located nuclei. The most variable findings relate to the texture of the cytoplasm, which varies from granular to dense to vacuolar. FNA cytology of epithelioid leiomyosarcoma can show cells that are mononuclear, binucleated or multinucleated, with eccentric nuclei and dense to vacuolar cytoplasm, with the variations probably depending on fixation status.

Biopsy, Needle↗

1H-NMR detectable fatty acyl chain unsaturation in excised leiomyosarcoma correlate with grade and mitotic activity.

We report on the use of 1H-NMR two-dimensional total correlated spectroscopy (2D TOCSY) at 600 MHz for an ex vivo analysis of fatty acyl chain lipid in normal smooth muscle and a series of primary retroperitoneal leiomyosarcomas. These TOCSY spectra were used to identify and quantitate the methylene protons situated between unsaturated site protons (D) to those bordered by only one unsaturated site proton (C). The D/C cross-peak volume ratios determined for oleic (18:1), linoleic (18:2), linolenic (18:3), and arachidonic (20:4) acids were 0.0, 1.3, 2.7, and 4.0, respectively, suggesting that this ratio can be a measure of the degree of unsaturation for fatty acyl chains of lipids. The D/C cross-peak volume ratio was found to be proportional to the mean mitotic activity (r = 0.94) in nine smooth muscle tissues. These results suggest, that for leiomyosarcoma, the degree of fatty acyl unsaturation may be an important determinant of the metastatic potential of these tumors. Furthermore, application of TOCSY for the ex vivo study of smooth muscle tumors would potentially serve as a pathologist-independent and quantitative method for assessment of leiomyosarcoma grade and mitotic activity thereby rendering a more accurate staging of patients.

Amino Acids↗

Leiomyosarcoma of the inferior vena cava: surgical management and clinical results.

Leiomyosarcoma of the inferior vena cava (IVC) is a rare lesion with less than 300 cases reported. Optimal management and long-term outcomes are not well described. From August 1984 to June 2004, eight patients with leiomyosarcoma of the IVC were treated at our institution. Clinical and pathologic data, surgical management, and outcomes were assessed. Eight cases were identified (4 males) with a median age of 52 (range 29-66). Presenting symptoms included abdominal pain (n = 5, 63%), lower extremity edema (n = 2, 25%), and palpable mass (n = 2, 25%). Tumor location was between the renal and iliac veins (low) (n = 4, 50%), between the hepatic and renal veins (middle) (n = 3, 38%), and above the hepatic veins with right atrial extension (high) (n = 1, 12%). Two patients with preoperative IVC occlusion were managed with tumor excision and IVC ligation. Three patients had primary repair of the IVC after tumor excision. A polytetrafluorothylene (PTFE) tube graft was used for IVC reconstruction in three cases. There was no postoperative mortality. Postoperative morbidity included deep venous thrombosis (DVT) (n = 1), lower extremity edema (mild n = 1; moderate n = 1), GI bleed (n = 1), and chronic renal insufficiency (n = 1). One patient is currently receiving adjuvant chemotherapy. Four patients received chemotherapy after recurrence, and one received palliative radiation therapy as well. Median survival to this point was 60 months with a median follow-up of 39 months. The 5-year overall survival and disease-free survival was 31 per cent for both (CI 0.1-1.0). The type of IVC reconstruction had no effect on survival (P = 0.22). Recurrence was discovered in four patients (50%) at a median time of 14 months. Resection of leiomyosarcoma of the IVC should be attempted whenever feasible. The management of the IVC can be managed with primary repair, ligation, or prosthetic graft. Long-term survival is possible if complete resection can be achieved.

Adult↗

Leiomyosarcoma of the middle ear and temporal bone.

Leiomyosarcoma is a malignant tumor of smooth muscle origin. It is rarely encountered in the head and neck area. A patient with leiomyosarcoma of the middle ear and the temporal bone with unusual bone destruction is presented. To our knowledge it is the first report of a middle ear and temporal bone leiomyosarcoma.

Aged↗

Spontaneously occurring leiomyosarcomas of the mouse urinary bladder.

Spontaneous leiomyosarcomas of the mouse urinary bladder have not been reported. Data from 8 chronic toxicity/oncogenicity studies that included 400 male and 400 female mice were reviewed and evaluated to gather information on spontaneously occurring urinary bladder leiomyosarcomas. Three control mice from 3 different studies had leiomyosarcomas in the submucosa of the trigone area of the urinary bladder. These tumors were not connected to the surface epithelium; however, they were connected to and destroyed the smooth muscle layer of the urinary bladder. This communication describes the incidence and histopathological features of these neoplasms.

Animals↗

Leiomyosarcoma of the larynx.

Leiomyosarcoma involving the larynx is extremely rare and may be difficult to diagnose. Because of the rarity of this tumor, little information exists on its long-term follow-up and optimal management. We present a review of the literature and report on a patient with leiomyosarcoma of the larynx treated with surgery and postoperative irradiation. In addition, the diagnosis and treatment of leiomyosarcoma are discussed. At six months' follow-up the patient showed no signs of local recurrence but had developed metastases to both lungs.

Combined Modality Therapy↗

Primary leiomyosarcoma affecting the ankle joint.

In a 50-year-old patient with a malignant tumor of the left distal tibia (stage la, according to Enneking) undermining the cartilage of the ankle joint, an extremely rare primary leiomyosarcoma of bone was diagnosed. The diagnosis of a primary leiomyosarcoma of bone is possible only after a secondary (metastatic) leiomyosarcoma has been excluded. Furthermore, typical histopathological features, including immunohistochemical staining results, are required for the diagnosis. Surgery is the therapy of choice. An R0 resection should always be attempted.

Adolescent↗