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PubMedDate not supplied

Aspiration biopsy cytology of smooth muscle tumors. A cytologic approach to the differentiation between leiomyosarcoma and leiomyoma.

From 1970 to 1991, 51 cases of smooth muscle tumors were diagnosed by fine needle aspiration biopsy. All were verified histologically and/or clinically, with no false-positive results. Of the 51 tumors, 41 were leiomyosarcoma and 10 leiomyoma. Among the 41 cases of leiomyosarcoma, 29 were the well-differentiated type; 8, poorly differentiated type; and 4, epithelioid type. Of the 10 cases of leiomyoma, 2 were the epithelioid type. The cytomorphologic features and cytologic patterns of various types of smooth muscle tumors observed in aspirate preparations are presented and compared in order to establish the cytologic criteria for differentiating malignant from benign smooth muscle tumors. Cytomorphologically the various types of smooth muscle tumors were different, and their cytologic features were sufficiently distinctive to distinguish one from the others. It appears possible to differentiate a well-differentiated leiomyosarcoma from a leiomyoma on the basis of cytologic findings observed in aspirate preparations. The recognition of different cytomorphologic features of various types of smooth muscle tumors is important in establishing an accurate cytologic diagnosis, which may be of practical significance to clinical management.

Biopsy, Needle↗

PubMed1996

[Leiomyosarcoma of the stomach--clinical aspects, morphology and therapy].

BACKGROUND: Due to the rarity of gastric leiomyosarcomas, only little is known on the results of diagnostics and therapy of these tumors. According to literature, the problem of suitable criteria in determining malignancy of these neoplasms is unsettled. PATIENTS AND METHOD: 21 patients (10 male, 11 female; aged 13 to 74 [53 +/- 13,9] years) with leiomyosarcomas of the stomach were treated from 1978 to 1993. In a retrospective study, the diagnostic and therapeutic results and the pathologic features of these patients are compared with the results of literature. RESULTS: Diagnosis was rarely confined preoperatively. Potentially curative resection was possible in 13/21 cases. Five year survival was 44% overall (R0-resections: 60%, R1/2-resections 22%) with a wide spread of individual cases. Pathologic examination of the tumors showed only half of the tumors classifiable according to the system of McGrath. Some well differentiated tumors revealed unclear malignancy criteria. Analysis of prognostic factors showed no statistically significant influence of surgical radicality but a significant influence of tumor size, mitotic activity and nuclear grading on survival and recurrence. CONCLUSIONS: Because of their rare incidence, the treatment of gastric leiomyosarcomas remains non-standardized. Surgical radical removal of the tumor seems to promise the largest benefit. The treatment of patients with residual or recurrent tumors remains individual, no adjuvant radio- or chemotherapeutical treatment can be generally recommended.

Adolescent↗

PubMedDate not supplied

[Bladder leiomyosarcoma. Report of 2 cases].

OBJECTIVES: The difficulties encountered in making the differential diagnosis and the highly malignant nature of leiomyosarcoma of the bladder, an uncommon tumor type, have prompted us to report on these two cases. MATERIAL AND METHODS: Two cases of leiomyosarcoma of the bladder are described: a 64-year-old female that was treated sequentially by TUR and chemotherapy and died of metastatic disease after 14 months, and an 81-year-old male with metastasis at the time of diagnosis. The literature is reviewed and the clinical pathological immunohistochemical features and treatment are discussed. CONCLUSIONS: The differential diagnosis of this tumor type is complex and requires immunohistochemical data. The diagnosis of leiomyosarcoma is confirmed by the expression of myogenic antigens. Historically, patient survival has been poor and management has been based upon information obtained from a relatively small number of cases with diverse treatment regimens. Because the efficacy of chemotherapy and radiotherapy remains unclear, the treatment of choice is by radical cystectomy or partial cystectomy when tumor location and size permit.

Aged↗

PubMed1996

[Leiomyosarcoma in jejunal diverticulum. A case report and review of the literature].

The Authors describe a case of double jejunal diverticulum, characterized by the presence of a leiomyosarcoma in one diverticulum and ectopic pancreatic mucosa in the other. The rarity of the occurrence of a leiomyosarcoma in the diverticular area is outlined; only few cases have been reported in Literature, excluding those originating, from Meckel's diverticulum. Barium X-rays of the small intestine allowed the neoplasia to be discovered, while an Echo-Color-Doppler examination suggested the histological type given the high vascularization typical of leiomyosarcomas.

Diverticulum↗

PubMed1996

Primary leiomyosarcoma of bone: report of seven cases and review of the literature.

OBJECTIVE: To report seven cases of intraosseous leiomyosarcoma and compare them with the cases in the literature. DESIGN: Retrospective review of histologic, immunohistochemical, and ultrastructural features. Tumors were examined immunohistochemically for proliferative activity and p53 protein accumulation. SETTING: Mount Sinai Hospital, University of Toronto, Ontario, Canada. RESULTS: The histologic grade of the tumors ranged from I to III. All tumors showed vimentin and muscle-specific actin immunoreactivity and smooth muscle differentiation ultrastructurally. Proliferative activity was demonstrated immunohistochemically in five of the seven cases. Only one tumor showed p53 protein accumulation. CONCLUSION: Primary leiomyosarcoma of bone is an aggressive tumor with light microscopic, ultrastructural, and immunohistochemical features similar to leiomyosarcoma of soft tissue. The proliferative activity of these tumors does not appear to correlate with the histologic grade. p53 protein accumulation as detected by immunostaining is not common in these tumors.

Actins↗

PubMed1996

HMB-45 reactivity in renal leiomyomas and leiomyosarcomas.

Ten renal smooth muscle tumors (seven leiomyomas, three leiomyosarcomas) and an unusual smooth muscle-invested renal cell carcinoma were studied for HMB-45 reactivity. All leiomyomas strongly expressed at least one, and most expressed three, smooth muscle markers (desmin, MSA, SMSA) and were negative for two cytokeratin markers (BDK, AE1). Six of seven leiomyomas and the smooth muscle investment of a renal carcinoma contained a population of cells strongly positive for HMB-45. A total of 15 blocks from the 6 HMB-45-positive leiomyomas were studied, and no adipocytes or abnormal vessels suggestive of angiomyolipoma were identified. The six HMB-45-positive leiomyomas seemed to arise from the renal capsule, whereas a seventh leiomyoma, which was negative for HMB-45, seemed to arise from the renal pelvis. Three leiomyosarcomas were also studied, which strongly expressed at least one smooth muscle marker and contained myofilaments at electron microscopic examination. No cytokeratin reactivity and no HMB-45-positive cells were detected in these leiomyosarcomas nor in the normal renal capsule adjacent to leiomyomas. This study showed that HMB-45-positive cells are detectable in a population of cells in some smooth muscle tumors, particularly in those that appear to arise from the renal capsule.

Actins↗

PubMedDate not supplied

[Leiomyosarcoma originating in the renal vein. A special case].

OBJECTIVES: An additional case of leiomyosarcoma of the renal vein is presented and the literature reviewed. METHODS/RESULTS: The patient had a history of hypertension. He and his brother were the first case of twins with bilateral retinoblastoma reported in the literature. The pathological findings were diagnostic of leiomyosarcoma of the renal vein. The clinical features, diagnosis and treatment are discussed. CONCLUSIONS: Leiomyosarcoma of the renal vein is an uncommon tumor which presents difficulties in making the preoperative diagnosis. A possible relationship may exist between this tumor type and the radiotherapy administered during childhood due to retinoblastoma.

Adult↗

PubMed1977

Leiomyosarcoma of the rectum and prostate.

A 47-year-old man with leiomyosarcoma involving the rectum and prostate was treated by radical abdominoperineal resection. This case appears to be unique in that leiomyosarcoma was present in both the rectum and the prostate. The literature is reviewed, highlighting some interesting clinical features of this condition. The prognosis for leiomyosarcoma of both rectum and prostate is poor.

Humans↗

PubMed1997

Sciatica as the first manifestation of a leiomyosarcoma of the buttock.

We report the case of a 22-year-old woman who was admitted for inflammatory right-sided sciatica with a decline in general health. Laboratory tests showed severe inflammation. Ultrasonography and computed tomography of the buttock and pelvis demonstrated a large tumor in the deep muscles of the right buttock, with an intrapelvic component that displaced the pelvic organs without invading them. Histologic examination of a biopsy specimen taken via the buttock established the diagnosis of leiomyosarcoma. Surgery by a pelvic approach allowed partial resection of the tumor, which was seen to invade the sciatic nerve. Radiation therapy was given. Six months later the residual tumor was considerably larger and the patient's general health had deteriorated further. Leiomyosarcomas are rare tumors whose diagnosis rests on histologic examination of biopsy or surgical specimens. The treatment is surgical. Recurrences are seen in 40 to 60% of cases, and metastases in 27 to 60%. The place of radiation therapy and chemotherapy in the treatment of leiomyosarcoma remains unclear.

Adult↗

PubMed1997

p53, proliferating cell nuclear antigen, and Ki-67 expression in extrauterine leiomyosarcomas.

Extrauterine leiomyosarcomas, because of their relative rarity, are poorly understood in regards to their malignant potential and biologic markers. Many human tumors are characterized by overexpression of p53, a tumor suppressor protein, and by expression of nuclear proteins associated with proliferation, such as proliferating cell nuclear antigen (PCNA) and Ki-67. We examined expression of these markers in 44 extrauterine leiomyosarcomas from 10 men and 25 women who ranged in age from 25 to 82 years (mean, 56 yr). Clinical follow-up was obtained in 34 (97%) of 35 patients, p53 expression was studied with two monoclonal antibodies (1801, D07) by use of an antigen retrieval method. p53 overexpression was present in 19 (43%) of 44 cases, whereas Ki-67 and PCNA staining were seen in 29 (66%) and 36 (82%) of 44 cases, respectively. There was no correlation between overall survival or recurrence and p53 or PCNA and Ki-67 expression. We conclude that p53, Ki-67, and PCNA are expressed in a large number of extrauterine leiomyosarcomas. In this study, the expression of these markers did not predict biologic behavior.

Adolescent↗

PubMed1997

Primary leiomyosarcomas of the lung: a clinicopathologic and immunohistochemical study of 18 cases.

We studied 18 patients with primary malignant smooth muscle tumors of the lung (7 women and 11 men, 5-76 yr old, with a mean age of 50 yr). Lesions varied from 1.7 to 10 cm in greatest diameter. The tumors were classified as low (4 cases), intermediate (2), or high grade (12). Low-grade lesions were characterized by an orderly proliferation of fascicles of spindle cells that intersected at right angles and showed oval-to-spindle cells with cigar-shaped nuclei, minimal pleomorphism, and low mitotic activity, without hemorrhage or necrosis. Intermediate-grade lesions retained the fascicular configuration but showed increased cellularity with atypia and dense chromatin pattern, occasional pleomorphism, and mild increase in mitotic activity. High-grade lesions showed high cellularity, marked pleomorphism and atypia, frequent areas of hemorrhage and necrosis, and high mitotic activity. Immunohistochemical studies in 16 cases showed positive staining of tumor cells with smooth muscle actin in 12, desmin in 5, and coexpression of actin and/or desmin and keratin in 3. Six patients with low- and intermediate-grade lesions were alive and well from 2 to 12 years after diagnosis (mean follow-up time, 6 yr); 8 with high-grade lesions died of their tumors with widespread metastases from 1 to 24 months after diagnosis (median survival time, 5 mo). One patient whose tumor showed features of high-grade leiomyosarcoma was alive and well 12 years after surgery. Three patients with high-grade tumors were lost to follow-up. Our findings suggest that histologic grade may be the most reliable prognostic parameter for predicting clinical behavior in primary leiomyosarcoma of the lung and that smooth muscle actin is the most sensitive immunohistochemical marker for establishing the diagnosis in these tumors. Primary leiomyosarcoma should be considered in the differential diagnosis of pulmonary spindle cell neoplasms; histologic grading may be of value in the planning of therapy and assessment of prognosis for these lesions.

Actins↗

PubMedDate not supplied

Multiple leiomyosarcoma of the stomach.

Leiomyosarcoma is the second most common non epithelial malignant tumor of the stomach. It is almost always a single lesion. Multiple leiomyosarcomas of the stomach are extremely rare. To our knowledge only three cases have been reported so far. We present a 40 year old female with epigastric pain, nausea, diarrhea, weight loss and melena in whom we diagnosed multiple lesions of the stomach. At operation, we found a total of 11 submucosal or subserosal lesions ranging in size from 0.5 to 6 cm in diameter localized throughout the stomach. Histological examination showed leiomyosarcoma in every lesion. Almost all the lymph nodes along curvatures had metastases. Other lymph nodes, peritoneum, liver and other organs were disease free. A total gastrectomy and Roux-en-Y esophagojejunostomy was performed. She had an uneventful recovery and has remained symptom-free so far (nine months).

Adult↗

PubMed1997

[Suspected leiomyosarcoma of the trachea].

A 73-year-old women was admitted to our hospital because of dyspnea, bloody sputum, and an apparent intratracheal tumor. A chest X-ray film from August 1991 showed a sharply circumscribed soft-tissue density in the trachea at the level of the aortic arch. By March 1993 the tumor had grown from 15 mm to 20 mm in diameter. A chest CT scan and fiberoptic bronchoscopy showed an intratracheal tumor that occupied 80% to 90% of the tracheal lumen. The tumor was resected on March 26, 1993. Histopathological study revealed a suspected leiomyosarcoma of the trachea. Only 18 cases of leiomyosarcoma of the trachea have previously been reported worldwide. We know of only 3 previous reports of the case of suspected leiomyosarcoma of the trachea in Japan.

Aged↗

PubMed1997

[Uterine leiomyosarcoma. Nine case reports, review of the literature].

OBJECT OF THE STUDY: To evaluate management and outcome of the treatment of uterine leiomyosarcoma. PATIENTS AND METHODS: Retrospective study of a series of 9 patients treated for uterine leiomyosarcoma in this hospital from 1982 to 1994. RESULTS: The condition is rarely suspected preoperatively, diagnosis is usually made on histological examination of the operative specimen. The outcome is related to the mitotic activity of the tumour and to the infiltration of nearly structures. Surgery is the only effective treatment, chemotherapy is ineffective, adjuvant radiotherapy improves local control of the tumour but has no incidence on survival. CONCLUSION: In the absence of an effective adjuvant treatment uterine leiomyosarcoma bears a poor prognosis with the only exception of small non infiltrating tumours with a low mitotic activity.

Adult↗

PubMed1997

Latent Epstein-Barr virus infection demonstrated in low-grade leiomyosarcomas of adults with acquired immunodeficiency syndrome, but not in adjacent Kaposi's lesion or smooth muscle tumors in immunocompetent patients.

BACKGROUND: Incidence of smooth muscle tumors is increased in patients with human immunodeficiency virus infection and organ transplant recipients. Smooth muscle tumors in immunocompromised patients often occur in unusual locations and exhibit evidence of latent infection by clonal, presumably tumorigenic, Epstein-Barr virus (EBV). OBJECTIVE: To investigate the presence of EBV latent infection in smooth muscle tumors in patients with acquired immunodeficiency syndrome and in immunocompetent patients. DESIGN: Twenty-two extrauterine, extraintestinal smooth muscle and myofibroblastic tumors were reviewed pathologically, and clinical charts were screened. Tumors were malignant (15 patients), benign (6 patients), and borderline (1 patient). Tissue specimens were investigated for latent EBV infection by latent membrane protein immunocytochemistry and EBV-encoded RNA in situ hybridization. SETTING: University Hospital of the University of Nancy, France. PATIENTS: Patients were 18 adults and four children. Two adults had acquired immunodeficiency syndrome. Both had low-grade leiomyosarcomas located in adrenal gland. Moreover, in patient 1, leiomyosarcoma was multifocal in pericardium and lymph node; in lymph node, muscle tumor was adjacent to nodal and skin Kaposi's lesions. RESULTS: In both patients with acquired immunodeficiency syndrome and leiomyosarcoma, latent infection by EBV could be demonstrated in tumor cells, contrasting with absence of detectable EBV infection in adjacent non-neoplastic tissues and nearby Kaposi's lesions. Latent infection by EBV could not be demonstrated in smooth muscle and myofibroblastic tumors in immunocompetent patients. CONCLUSIONS: Latent EBV infection is associated with smooth muscle cell tumors in immunocompromised patients, but not in immunocompetent patients.

AIDS-Related Opportunistic Infections↗

PubMed1996

[Reappraisal of the pathological criteria for uterine leiomyosarcoma].

In order to study the pathologic criteria for uterine Leiomyosarcoma, 42 cases of morphologically malignant or uncertain malignant potential uterine smooth muscle tumors which exhibited recurrences or metastasis, or were followed for a minimum of 2 years clinically, were analysed and reappraised. The results showed that in comparison with the leiomyoma group, active mitosis (mitotic index, MI > or = 10/10HPF), significant cytologic atypia and coagulative tumor cell necrosis (CTCN) were more common in the leiomyosarcoma group (71.4%, 92.9% and 71.4% respectively). Whereas 4 of the 14 leiomyosarcomas had MI < or = 4/10HPF, developed recurrences or metastasis, and 7 of the 28 leiomyomas had MI > or = 5/10HPF, including 2 with MI > 10/10HPF were alive and well 5.5-23.0 years after operation; hence a mitotic count is an unreliable prognostic criterion for uterine smooth muscle tumors. The data suggest that a trivariate rule using MI, the degree of atypia and CTCN best identifies the aggressive potential of uterine smooth muscle tumors.

Adult↗

PubMed1976

Renal vein leiomyosarcoma.

The third reported case of renal vein leiomyosarcoma is presented. Diagnosis was not made until exploratory celiotomy revealed a tumor originating from the left renal vein. The tumor was resected with margins of normal vein, and the patient was alive without recurrence 12 months after operation. Review of 65 cases of leiomyosarcoma originating in other retroperitoneal structures revealed a two-year survival rate of 20%. Therefore, we recommend consideration of postoperative adjunctive chemotherapy for renal vein leiomyosarcomas.

Abdominal Neoplasms↗

PubMedDate not supplied

The effect of extend of caval resection in the treatment of inferior vena cava leiomyosarcoma.

BACKGROUND: A wide and complete surgical resection is the principle modality of therapy in the management of retroperitoneal sarcomas. It is current opinion that, also for inferior vena cava (IVC) leiomyosarcomas, an extended resection of either retroperitoneal tissue and vena cava should be performed. The aim of the study was to investigate the influence of the venous extent of resection on local recurrence and longterm outcome. METHODS: Up to August 1994, 218 patients were enrolled into The International Registry of Inferior Vena Cava (IVC) Leiomyosarcomas. For the purpose of this study we considered 120 patients who underwent a radical resection of the IVC tumor (i.e. removal of all gross disease with microscopic tumor-free margins and no evidence of distant metastases). Resection included an IVC rim in 53 patients and an IVC segment in 67. RESULTS: There were 3 (2.5%) early postoperative deaths and 7 (5.8%) major complications. Postoperative deep venous thrombosis of the lower limbs was diagnosed in 21 (17.5%) patients and was treated by anticoagulant therapy and/or elastic stocking without long-term sequelae. Overall, tumor recurrence was observed in 67 (57.3%) patients at a mean follow-up of 32 +/- 4 months. Seven, 13 and 4 patients who underwent caval wall resection and 9, 29 and 5 patients submitted to a caval segmental resection had either local recurrences, distant metastases or local and distant metastases (p = NS). Survival rates of the two groups were 55% and 37% at 5-year and 42% and 23% at 10-year, respectively (p = NS). CONCLUSION: An extended venous resection in IVC leiomyosarcoma does not influence local recurrence rate nor long-term outcome.

Adolescent↗