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PubMed1983

Retroperitoneal leiomyosarcoma. A clinicopathologic analysis of 36 cases.

Thirty-six cases of retroperitoneal leiomyosarcoma form the basis for this retrospective clinicopathologic study. This group comprised 24 females (67%) and 12 males (33%), whose ages ranged from 12 to 94 years (median, 60 years.). The clinical presentation, invariably nonspecific, consisted of pain or weight loss, typically associated with a palpable abdominal mass. Of the 30 patients with follow-up data, 23 (77%) died of retroperitoneal leiomyosarcoma. Grossly, the bulky often multinodular tumors, which ranged from 7.5 to 35 cm in maximal dimension (median, 12.8 cm), varied from firm to soft. In addition to the classical microscopic picture of leiomyosarcoma as manifested by interlacing fascicles of slender eosinophilic cells, other less frequently encountered, morphologic variations of malignant smooth-muscle tumors were also observed. Although absolute minimal criteria for a malignant tumor diagnosis could not be established, the findings suggest that a retroperitoneal smooth-muscle tumor that measures at least 7.5 cm in greatest dimension and that has as few as 1 mitosis/10 HPF is capable of metastasis.

Adolescent↗

PubMed1983

Leiomyosarcoma of the broad ligament. A case report and literature review with follow-up.

A case of leiomyosarcoma arising in a preexisting leiomyoma of the broad ligament is presented with ultrastructural and Feulgen microspectrophotometric confirmation. This is the fourth reported case of leiomyosarcoma of the broad ligament and the second reported case to show evidence of a preexisting leiomyoma. Three of these patients died after intervals of 1, 7, and 19 months; one is alive and well with no evidence of disease after 12 years. The definitive evidence of malignant transformation of a leiomyoma has particular relevance to the controversy regarding the relationship of uterine leiomyosarcomas and leiomyomas.

Aged↗

PubMedDate not supplied

Cytology of metastatic leiomyosarcoma. A case report.

BACKGROUND: Metastasis of leiomyosarcoma is not uncommon. Determination of the primary site may be confused with the presenting symptoms and even the known pathology. CASE: A 62-year-old man presented with chest pain, a productive cough and a lung mass on radiography. Fine needle aspiration of the tumor showed pleomorphic tumor cells. The patient began to have abdominal symptoms one year after presentation. Jejunal leiomyosarcoma was proven after an emergency exploratory laparotomy for mechanical ileus. Sputum cytology disclosed tumor cells one month before death. Necropsy of the lung mass demonstrated findings similar to those of the intestinal tumor microscopically. CONCLUSION: Without laparotomy, our patient would have been diagnosed as having a primary pulmonary leiomyosarcoma by the presenting symptoms and known pathology.

Biopsy, Needle↗

PubMed1995

Diagnosis, treatment and prognosis of the leiomyosarcoma of the inferior vena cava. Three cases and summary of published reports.

OBJECTIVE: To describe our experience with three cases of leiomyosarcoma of the inferior vena cava, and summarise current methods of diagnosis and treatment. DESIGN: Descriptive study. SETTING: University hospital, Germany. SUBJECTS: 3 Patients with histologically confirmed leiomyosarcoma of the inferior vena cava. INTERVENTIONS: Resection, with or without vascular reconstruction. MAIN OUTCOME MEASURES: Morbidity and mortality. RESULTS: Case 1 was diagnosed on magnetic resonance imaging, the tumour was resected, and the vena cava replaced with a polytetrafluoroethylene (PTFE) graft. She made an uncomplicated recovery and was well and free of symptoms 10 months later. Case 2 was diagnosed at operation (the differential diagnosis on computed tomography was a retroperitoneal tumour), the tumour was resected, and the defect oversewn. She made an uneventful recovery and was well and free of symptoms 14 months later. Case 3 was diagnosed at operation (a diagnosis of pancreatic cancer had been made preoperatively), the tumour was resected, and the defect oversewn. She was well and free of symptoms 10 months after operation. CONCLUSIONS: Magnetic resonance imaging is superior to computed tomography in the diagnosis of leiomyosarcoma of the inferior vena cava. The treatment of choice is resection; small defects can be closed by suture or PTFE patch, and large defects by PTFE prostheses. Reported resectability is 40% to 60%, but the prognosis is poor; the local recurrence rate is about 36%, most patients are dead within 2.5 years, and the 5-year survival is 30%. Chemotherapy and radiotherapy will give some degree of palliation, but do not affect the outcome.

Aged↗

PubMed1995

Reevaluation of prognostic factors in gastric leiomyosarcoma.

OBJECTIVES: The aim of our study was to reevaluate the prognostic factors in gastric leiomyosarcomas, particularly those under 6 cm, which, because of improved diagnostic techniques, make up a large proportion of this series, in contrast to previously published works. METHODS: The clinicopathological features in 83 patients with gastric leiomyosarcoma were reviewed. Cox's proportional hazards regression analysis was used to determine the prognostic factors. RESULTS: Sixty of 84 leiomyosarcomas (71.4%) were less than 6 cm in diameter. In univariate analysis, the following factors were considered to be unfavorable, with statistical significances: 1) presence of distant metastasis and/or direct invasion of adjacent structures at diagnosis (hazard ratio 36.60), 2) > or = 4 mitoses per 20 high-power fields (25.39), 3) severe nuclear atypia (16.74); 4) presence of ulceration of overlying gastric mucosa (12.57), and 5) diameter > or = 6 cm (5.57). Among these factors, mucosal ulceration has been identified as a new factor associated with poor prognosis. Histologically, the tumors with ulceration showed more severe nuclear atypia (p < 0.05), necrosis (p < 0.01), and a higher incidence of mitosis (p < 0.01), compared with tumors without ulceration. In multivariate analysis, factors 1, 2, and 3 were statistically significant, although neither size nor ulceration was significant. CONCLUSIONS: The presence of distant metastasis and/or direct invasion, high mitotic rate, and severe nuclear atypia were confirmed to be unfavorable prognostic indicators. Mucosal ulceration, indicating high proliferative activity of the tumor, even if small, suggests a poor prognosis, although it was not significant in multivariate analysis.

Combined Modality Therapy↗

PubMed1995

[Leiomyosarcoma of the rectum].

The distinction between leiomyoma and high differentiated leiomyosarcoma can be difficult. The most important distinguishing feature is the number of mitoses. Additionally the tightness of the cells, the number of necroses and cell atypias and the size of the tumor are significant. The most common therapy for large and low differentiated leiomyosarcomas of the rectum are the abdomino-perineal resection or the low anterior resection. In high graded tumors of less than 2.5 cm the radicality of the surgical treatment is questioned. Some authors recommend a wide local excision, but in this case a high local recurrence rate has to be expected. This is the reason for a more radical treatment of all leiomyosarcomas of the rectum. Chemotherapy and radiotherapy are generally not effective. The prognosis is poor.

Adult↗

PubMed1994

[A case of incidental renal leiomyosarcoma].

A 68-year-old man visited his home doctor with the chief complaint of left flank pain. Abdominal ultrasonography revealed left hydronephrosis and space occupying lesion in the right kidney. He was referred to our hospital because of suspected left ureteral stone and for further examination. Drip infusion pyelography revealed a left ureteral stone and retrograde pyelography showed extrinsic compression and deformation of the right pyelogram. A computed tomography showed a heterogeneous solid mass, 7 x 4 cm in size, compressing the pelvicocalyceal system and growing out of the kidney. Right radical nephrectomy was performed under the diagnosis of renal cell carcinoma. The pathological diagnosis was leiomyosarcoma. Seventy-five cases of renal leiomyosarcoma have been reported in the Japanese literature and this is the third case as an incidental tumor. Because of the absence of the characteristic diagnostic signs, this malignant tumor can present diagnostic difficulty. The diagnostic problems and clinical features of renal leiomyosarcoma are discussed.

Aged↗

PubMed1994

Leiomyosarcomas of the gastrointestinal tract.

Nine men and four women (median 69 years, range 38-86 years) with gastrointestinal leiomyosarcomas were treated from 1981 to 1991. Tumours arose from the stomach (5), the small intestine (6) and the rectum (2). Nearly half of all patients were surgical emergencies: one patient had perforation of the stomach and two of the small intestine from tumour ulceration, three cases presented with life-threatening bleeding from leiomyosarcomas of the small intestine. All patients were operated on, but only seven for cure. At follow-up one patient had emigrated and four had died, two from leiomyosarcoma after 13 and 36 months and two from unrelated causes after seven and 48 months. Two patients are still alive 24 and 78 months after surgery without recurrence. Patients and amenable to a curative operation died within nine months.

Adult↗

PubMed1994

Leiomyosarcoma with prominent osteoclast-like giant cells. Analysis of eight cases closely mimicking the so-called giant cell variant of malignant fibrous histiocytoma.

Eight cases of leiomyosarcoma with osteoclast-like giant cells, arising in deep soft tissue, and that mimicked closely the "giant cell variant of malignant fibrous histiocytoma (MFH)," have been studied morphologically and immunohistochemically. The age of the patients ranged from 7 to 88 years (mean, 66.2 years; median, 74 years); five were female patients. Three lesions arose in the lower limbs, two in the buttock, and one each in the shoulder, chest wall, and the floor of the mouth. Follow-up in one case revealed a local recurrence and in two cases systemic metastases. All cases showed, at least focally, interwoven spindle cell fascicles, with the cytologic features of smooth muscle cells, as well as strong positivity for alpha-smooth-muscle actin, muscle actin, and desmin. The morphologically benign osteoclast-like giant cells expressed CD68 but failed to stain with myogenic markers. The association of leiomyosarcoma with prominent osteoclast-like giant cells is not as uncommon as generally believed, being evident in 8.7% of the deep-seated nonvisceral leiomyosarcomas that we have studied. These results provide good evidence for myogenic differentiation in at least a subset of those tumors with morphologic features currently classified as the giant cell variant of MFH. Considering that at least some other reported cases of giant cell MFH appear to be a variant of extraskeletal osteosarcoma, we would suggest that lesions with this distinctive pattern should be more carefully classified according to their apparent line of differentiation.

Aged↗

PubMed1993

[Stomach leiomyosarcoma. Clinico-diagnostic considerations and therapeutic criteria].

An observed case of leiomyosarcoma of the stomach in a 67 year old man leads to an analysis of the diagnostic, clinical and therapeutical aspects of this myoid tumour in the digestive tract representing 50-60% of cases. Symptoms are aspecific and gastric bleeding is the most common sign (50%). leiomyosarcoma always presents considerable problems of diagnosis and represents the most important among the various examined diagnostic investigations but it is maintained that surgery many times plays a primary role in the diagnosis of this tumour. The accuracy of gastrofiberoscopic biopsy is limited by frequent false-negative cases. False negative cases are obtained because the tumour tissues are usually covered by gastric mucosa and so the biopsy with standard forceps is too small and too superficial. The ultrasonographic finding of a gastric leiomyosarcoma is variegated, in fact it can be hyperechoic or hypoechoic. Frequently we can see echo-free spaces due to liquefactive necrosis and in such a case, an echogenic rim is demonstrable. According to our experience ultrasonography is very important in post-operative follow-up. The TC study can be considered superior to the other diagnostic investigations, in fact with it, detailed characteristics and location of the tumour, invasivity, metastatic dissemination, are recognized. It is still controversial the criterium concerning the diagnosis of malignant smooth muscle tumour of the gastrointestinal tract. We believe that number of mitoses (having five or more mitotic figures x 10 high-power fields), cellularity, pleomorphism, atypicality, together with macroscopic aspects (size, bleeding, ulcerations, necrosis, invasivity) are the most reliable indicators of malignancy.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗

PubMed1994

Leiomyosarcoma of the pancreas: report of a case diagnosed by fine needle aspiration biopsy.

We present a case of pancreatic leiomyosarcoma with liver metastasis diagnosed by liver tumor biopsy. A 66-year-old man had a pancreatic tumor and hepatic tumors, which were hypoechoic and mixed echoic on ultrasonography, respectively. They were stained in enhanced computed tomography and angiography. A fine needle aspiration biopsy of the liver tumor was performed, and the pathological examination of the biopsied specimen suggested the tumor cells to have originated from smooth muscle. Under a diagnosis of pancreatic leiomyosarcoma with liver metastasis, the patient was treated with several anticancer agents. The tumor, however, spread to multiple organs and he dies of the disease two years nine months after the start of treatment. The diagnosis of pancreatic leiomyosarcoma was confirmed by autopsy.

Aged↗

PubMed1994

[Leiomyosarcoma of the prostate accompanied by multiple hepatocellular carcinoma: report of a case].

A case of leiomyosarcoma of the prostate accompanied by multiple hepatocellular carcinoma in a 51-year-old man is presented. He visited our hospital with the complaints of feeling of residual urine and pain of the coccygeal bone in November, 1990. A histopathological study of the transrectal needle biopsy specimens revealed leiomyosarcoma of the prostate. Since multiple liver tumors were pointed out, the patient was given combined chemotherapy (CYVADIC: cyclophosphamide, vincristine, adriamycin and DTIC). He died 1 year and 2 months after the initial diagnosis due to hepatic failure. The autopsy findings revealed that the histology of the liver tumors was hepatocellular carcinoma, and that the leiomyosarcoma of the prostate had directly invaded the wall of the urinary bladder and the rectum, but there was no obvious distant metastasis.

Carcinoma, Hepatocellular↗

PubMed1993

Leiomyosarcoma, leiomyomatosis in von Recklinghausen's disease: report of case.

A 48-year-old female patient with von Recklinghausen's disease was admitted to Kaohsiung Medical College Hospital due to massive hematochezia. The emergent mesentery arteriography revealed multiple hypervascular small intestinal lesions but no contrast extravasation. The endoscopic examination revealed a blood-filled small intestine with normal colonic mucosa. Laparotomy was carried out and multiple extraluminal masses on antimesenteric side of the small intestine were found. Segmental resection of the small intestine resulted in resolution of her symptom. The largest mass was diagnosed as leiomyosarcoma, and the others were diagnosed as leiomyoma. Histologically, differential diagnosis between leiomyosarcoma and leiomyoma is not clear cut, because it is based on a number of differentiating features. Surgical resection is the treatment of choice for intestinal leiomyosarcoma. The long-term follow-up of this patient is mandatory.

Diagnosis, Differential↗

PubMedDate not supplied

[Leiomyosarcoma of the gastrointestinal tract].

The records and biopsies of 10 patients with the diagnosis of leiomyosarcomas admitted at the Military Hospital "Dr. Carlos Arvelo" from 1962 to 1991, were reviewed. The sample correspond to 0.47% of all gastro-intestinal tumors. Sixty per cent of tumors were localized in the stomach, 20% in the jejunum, 10% in the duodenum and 10% in the colon. Eighty per cent had pain and 60% had bleeding. Sixty per cent of tumors were low grade leiomyosarcomas and 40% were epithelioid leiomyosarcomas. In eight of nine patients operated, resection was done. The survival range was from six months to 28 years with a median of 12 years. The most important prognostic factor was the extension of the tumor, independently of he histologic type.

Adult↗

PubMed1976

[Cutaneous leiomyosarcoma and osteomedullary plasmocytoma with the demonstration of IgA-kappa paraprotein in serum and skin tumor].

A dermal leiomyosarcoma associated with a still asymptomatic osteomedullary plasmocytoma with IgA paraproteinemia develop-d rapidly in a 78-year-old man. The same paraprotein type IgA kappa was identified, with distinct decrease in concentration, in serum, sarcoma-tissue, and in the tumour-surrounding skin area by m-ans of immunoelectrophoresis, radial immunodiffusion, and Ouchterlony test. The immunochemical identity of the paraprotein, the course of the disease as followed clinically and immunochemically, and several histological criteria are in favour of monoclonal origin of the paraprotein from malignant plasmocytoma cells. The accumulation of paraprotein in the sarcoma tissue is primarily explained by the marked blood congestion of the tumour. To our knowledge no report exists in the medical literature of an association of dermal leiomyosarcoma and early paraproteinemic plasmocytoma. This coincidence may be only of chance but we rather suggest an immunopathological relation of both tumors due to a partial immune insufficiency caused by the preceding plasmocytoma, resulting in a diminished immunological "surveillance" of the organism and favouring the development of a new malignant cell population (i.e. leiomyosarcoma).

Aged↗

PubMed1993

[Leiomyosarcoma of the liver: a case treated by transplantation].

A 68 year old woman with primary hepatic leiomyosarcoma, impairement of general status and right upper quadrant pain was diagnosed with liver tumor by sonography, CT scan and nuclear magnetic resonance imaging. Angiograms showed a hypervascular tumor. The patient was treated by hepatic transplantation but died 15 days after operation. The diagnosis of leiomyosarcoma was established by immunohistochemical and ultrastructural studies; the primary nature was confirmed by necropsy. Diagnostic and therapeutic features of primary hepatic leiomyosarcoma--the 19th case reported in the literature--are discussed.

Aged↗

PubMed1993

[Leiomyosarcoma of the chest wall].

An unusual case of a primary leiomyosarcoma of the chest wall is presented. The patient was a 41-year-old man and had no symptoms. The chest x-ray showed a tumor with mediastinal involvement. A percutaneous needle biopsy indicated that it was histologically a leiomyosarcoma. Wide en bloc excision of the chest wall was done, including much of the third, fourth and fifth ribs and a piece of lung. The defect was closed with GORE-TEX soft tissue patch. There were three courses of chemotherapy added, but the patient had a local recurrence, which was resected again, two years after the first surgery. It may be thought that wide en bloc excision and closely follow-up are important in the management of a leiomyosarcoma of the chest wall because of its high recurrence rate.

Adult↗

PubMed1993

Chromosomal abnormalities in leiomyosarcomas.

Thirty-eight tumors from 30 patients diagnosed as leiomyosarcoma were cytogenetically assessed after short term culture. The specimens were obtained from the retroperitoneum, gastrointestinal tract, and extremities. Chromosomal abnormalities were present in 18 tumors from 13 patients; 15 tumors had clonal changes, whereas 3 tumors had numerous nonclonal changes. Ten tumors from 10 patients had normal karyotypes and no results were obtained in 10 other tumors from 7 patients. Of the tumors with clonal chromosomal aberrations, 4 had near-diploid (3 hypo- and one hyperdiploid) modes, 8 were polyploid, and 3 were bimodal. No specific karyotypic change appeared to characterize the leiomyosarcomas, although involvement of some chromosomes appeared more frequent than others. A comparison of our findings with those reported in the literature revealed certain consistent structural rearrangements involving chromosomes 1, 7, 10, 13, and 14 at bands 1p36, 1p32, 1p13, 1q32, 7p11.1-p21, 7q32, 10q22, 13q14, and 14p11, respectively. Other bands less frequently rearranged were 3p13-p22, 3q21, 4q13-q23, 6q15-q21, 7q11.2-q22, 12q13-q14, 17q12-q25, 19q13.3-q13.4, and 20q12-q13.1. Numerical changes included recurrent loss of chromosomes 4, 9, 14, 15, 16, 18, 21, and 22. Identification of the abnormalities of these chromosomes is important in that it may predict the existence of oncogenes, tumor suppressor genes, and/or growth factor genes at these sites. Subsequent molecular analysis might then lead to the identification of the genes involved and ultimately to a better understanding of the pathogenesis of leiomyosarcomas.

Adult↗