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Infantile intestinal leiomyosarcoma: surgical resection (without adjuvant therapy) for cure.

A 7-week-old boy presented with a 6-week history of failure to thrive, acute intestinal obstruction, and an apparently irreducible intussusception (noted on contrast enema). He underwent abdominal exploration, during which a cecal mass was identified and resected. The mass proved to be a leiomyosarcoma. Histologically, it was an intermediate-grade malignancy with a predicted 5-year survival rate of 16% to 23% based on data from the adult experience. Three years after resection and without having received adjuvant therapy, he is healthy and free of disease. A review of the literature showed that in infants these tumors are predominantly colonic, compared with the predilection for small intestinal lesions found in the older pediatric and adult populations. Infantile intestinal leiomyosarcomata are rare malignancies that do well if complete surgical excision of the disease can be accomplished. The histological prognostic indicators proposed for intestinal leiomyosarcomas in the adult population cannot be extrapolated to infants because when they occur in infants, they appear to be less aggressive, and these patients do well without adjuvant therapy.

Age of Onset↗

Pediatric intestinal leiomyosarcomas: case report and review of the literature.

Primary intestinal leiomyosarcomas in children are rare tumors. Twenty-seven previous cases are documented in the literature with little information available on the treatment, survival, and prognostic factors surrounding these tumors. The authors report the 28th case of pediatric intestinal leiomyosarcoma and its follow-up, that of a newborn presenting with intestinal obstruction. Follow-up from all previously reported cases is not recorded and is needed. Our patient, like most children with this tumor, underwent complete excision with no evidence of recurrence at 5 years. Visceral metastases from these neoplasms are atypical, and recorded long-term prognosis may be favorable, unlike their counterpart in the adult population.

Humans↗

Bladder leiomyosarcoma following cyclophosphamide therapy for lupus nephritis.

We report 2 cases of leiomyosarcoma of the bladder that occurred after long-term cyclophosphamide chemotherapy for lupus nephritis and rheumatoid arthritis. One patient had a tumor at the end of an 11-year course of chemotherapy and 1 approximately 7 years after completing a 7-year course of chemotherapy. Patient 1 underwent left partial cystectomy and patient 2 underwent cystectomy with ileal conduit urinary diversion. In patient 1 the tumor was a typical leiomyosarcoma and patient 2 had a myxoid variant. Both patients were free of disease at 4 months and 3 years, respectively. Whereas previous reports of the carcinogenic effects of cyclophosphamide have been questioned, since the induced tumors occurred in patients being treated for other neoplasms (lymphoproliferative and myeloproliferative disorders), the disease in both of our patients followed cyclophosphamide therapy for nonneoplastic disorders.

Arthritis, Rheumatoid↗

Primary leiomyosarcoma of renal capsule.

The renal capsule long has been suggested to be the origin of leiomyosarcoma of the kidney but this speculation has never been proved. We describe a case of leiomyosarcoma lying with the renal capsule. The radiographic, as well as the macroscopic and microscopic appearances provide convincing evidence of capsular origin.

Adult↗

Uterine myxoid leiomyosarcoma.

BACKGROUND: Uterine myxoid leiomyosarcoma is rare, has a poor prognosis, and must be distinguished from a uterine myoma with myxomatous change. CASE: A 56-year-old woman with a history of epigastric pain and generalized abdominal swelling had a peritoneal cavity filled with tumor that originated from the posterior uterine wall. Chemotherapy and two operations for recurrence could not prevent fatal metastases to the vascular system. Histopathology showed a typical myxoid leiomyosarcoma. CONCLUSION: Although the patient's prognosis was poor, no mitoses were observed, and the nuclear abnormalities were not sufficient to diagnose the tumor as malignant at the initial surgery. Whenever a uterine myoma with myxomatous change is found, the patient should be monitored carefully.

Fatal Outcome↗

Eosinophilia and uterine leiomyosarcoma.

BACKGROUND: Tumor-associated blood eosinophilia and tumor-associated tissue eosinophilia have been described in association with a spectrum of neoplasms. A case of uterine leiomyosarcoma exhibiting both blood and tissue eosinophilia is presented. CASE: A 67-year-old postmenopausal woman was evaluated for a 6-month history of postmenopausal bleeding. An office-based endometrial biopsy yielded atypical mesenchymal tissue with marked eosinophilic infiltrate suspicious for a uterine sarcoma, and the patient was referred to the gynecologic oncology service in a tertiary care hospital. There was evidence of leukocytosis and eosinophilia in preoperative laboratory assessment. The patient underwent an exploratory laparotomy, a total abdominal hysterectomy, bilateral salpingo-oophorectomy, and pelvic lymph node dissection. Histopathology confirmed a low-grade uterine leiomyosarcoma confined to the uterus. There was extensive infiltration of the tumor with eosinophils. The systemic leukocytosis and the absolute eosinophilia responded to surgical removal of the tumor, with normalization of values in the immediate postoperative period. CONCLUSION: A differential diagnosis of malignancy should be entertained during an evaluation of systemic eosinophilia.

Aged↗

Uterine myxoid leiomyosarcoma within a leiomyoma.

A case of myxoid leiomyosarcoma of the uterus arising in a leiomyoma is reported. Although the tumor showed very low mitotic activity ranging from zero to 2/10 HPF, the presence of infiltrative pattern of growth and a high MIB-1 index (60% of cells positive) established the diagnosis. Myxoid leiomyosarcoma may arise in leiomyoma.

Aged↗

Hepatic leiomyosarcoma: ultrastructural study and review of the differential diagnosis.

A primary leiomyosarcoma of the liver in a 12 year old female is reported. It is the eleventh such tumor to be recorded, the first to be documented in an adolescent and the first to be studied electron microscopically. By light microscopy the well differentiated portions of the tumor were characteristic of leiomyosarcoma. Ultrastructurally a minority of tumor cells contained myofilaments along with cytoplasmic dense bodies and marginal dense plaques, the most distinctive subcellular markers of smooth muscle cells. A brief review of the differential diagnosis is provided.

Cell Nucleus↗

An unusual multifocal leiomyosarcoma of the stomach: a light and electron microscopic study.

A multifocal leiomyosarcoma of the stomach originating from the muscularis mucosae with lymph node and distant metastases is described in a 66 year old man. The electron microscopic features of a representative tumor mass and a metastasis confirmed the smooth muscle histogenesis. The light microscopic appearance consistently suggested malignant fibrous histocytoma. The pathological features of gasttric leiomyosarcomas are reviewed with special emphasis on the problem of practical diagnosis. This case also indicates that not all sarcomas with storiform features are necessarily histiocytic in origin.

Aged↗

Primary omental leiomyosarcoma masquerading as hemorrhagic ascites.

An 85-year-old man with unexplained hemorrhagic pseudoascites of four years' duration was discovered to have a primary omental leiomyosarcoma. Neoplasms arising in the greater omentum are rare. To date, only three primary omental leiomyosarcomas have been described. Clinical and pathologic findings of this case are presented and the topic of primary omental neoplasms is reviewed.

Aged↗

Primary leiomyosarcoma of the seminal vesicle.

A case of leiomyosarcoma of the seminal vesicle is described in a 68-year-old man. Digital rectal examination and pelvic computed tomography (CT) scan disclosed a large pelvic mass in the region of the prostate, whereas magnetic resonance imaging (MRI) suggested that the mass arose from the right seminal vesicle. Biopsy of the mass revealed a high-grade malignancy, thus a radical cystoprostatectomy was performed. Pathologic examination revealed a leiomyosarcoma arising from the right seminal vesicle. The patient is well and free of recurrent disease 13 months following surgery.

Aged↗

Retrospective review of 208 patients with leiomyosarcoma of the uterus: prognostic indicators, surgical management, and adjuvant therapy.

OBJECTIVE: We evaluated the predictive value of several proposed prognostic indicators and the effect of surgical management and adjuvant therapy on clinical outcome associated with leiomyosarcoma (LMS) of the uterus. METHODS: A medical record search of patients treated at Mayo Clinic from 1976 through 1999 was performed using the International Classification of Diseases, Ninth Revision codes for LMS and malignant neoplasm of the uterus. Study inclusion criteria included confirmation of the diagnosis of LMS of the uterus by a pathologist at our institution. Survival curves were generated using the Kaplan-Meier method. Multivariate analysis was performed using the Cox proportional hazards model. A case-control investigation was also performed. RESULTS: A total of 208 patients met study requirements. The median follow-up for survivors was 7.7 years. Multivariate analysis showed that high grade, advanced stage, and oophorectomy were associated with significantly worse disease-specific survival. Case-control investigations suggested that ovarian preservation does not adversely affect survival and that adjuvant pelvic radiation therapy does not significantly improve survival. An LMS risk-assessment index that was generated is highly predictive of survival. CONCLUSION: Tumor grade and stage (using modified criteria for endometrial cancer) appear to be valid prognostic indicators for LMS of the uterus. Ovarian preservation may be considered in premenopausal patients with early-stage leiomyosarcoma of the uterus. Additionally, adjuvant therapy does not appear to significantly affect survival. Finally, our highly predictive LMS risk-assessment index may be useful for counseling patients.

Adult↗

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

Primary leiomyosarcoma of the broad ligament is a very rare, rapidly progressive and highly malignant gynaecological tumor. There are only 11 cases reported so far in the literature. An 87-year-old Caucasian lady with leiomyosarcoma of the broad ligament is presented. Treatment consisted of resection and total abdominal hysterectomy with bilateral salpingo-oopphorectomy. Unusually, she developed large recurrent pelvic disease in the absence of disseminated metastasis following surgery. She died within 8 weeks of primary surgery. This highlights the unknown behavior of this tumor and the need for more research into management.

Aged↗

A case of leiomyosarcoma originating from pulmonary vein, occluding mitral inflow.

Cardiac leiomyosarcoma is a very rare entity that is found in less than 0.2% of all cardiac tumors. Because of its rarity and classification problem, there are few prospective studies about therapy and outcomes. Generally, diagnosis is delayed because of the nonspecific nature of the symptoms and by the time of diagnosis, most cases present with large tumors. Primary treatment of these tumors is still complete surgical resection. In this study we present a myxoid leiomyosarcoma originating from a pulmonary vein and extending into the left atrium, occluding mitral inflow in a woman 49 years of age. The tumor was totally removed. Open heart surgery is the acceptable approach for the management of intracardiac tumors, which may cause obstruction of blood flow through the heart.

Female↗

Cytogenetic abnormalities in primary bronchopulmonary leiomyosarcoma of childhood.

Primary bronchopulmonary leiomyosarcoma (PBLMS) is a rare malignant neoplasm in all age groups and only 10 pediatric cases of PBLMS have been reported. This report presents cytogenetic findings of a PBLMS from an 8-year-old boy. Tumor diagnosis was established by using routine histopathology, immunohistochemistry, and electron microscopy. The karyotype was highly complex, demonstrating consistent structural abnormalities of chromosomes 1, 5, 6, and 7, relative gain of chromosomes 2 and 11, and relative loss of chromosomes 9, 19, 20, and 22, along with the presence of multiple marker chromosomes. Cytogenetic results of previously reported leiomyosarcomas are reviewed and compared with the present case.

Child↗