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PubMed1976

Leiomyomas and leiomyosarcomas of the kidney in 4-day-old BUF rats given methylazoxymethanol acetate intraperitoneally.

The effect of age and sex on the development of renal tumors was studied in inbred BUF male and female rats 4 days, 5, 8, 12, 24, or 52 weeks old. Methylazoxymethanol (MAM) acetate was injected ip (20 mg/kg body wt) once weekly for 9 weeks. Animals 52 weeks old died from hepatic and/or renal necrosis; however, animals of other ages survived 24-42 weeks. Female rats 4 days old were susceptible to the development of leiomyomas and leiomyosarcomas of the kidney, whereas 4-day-old male rats had a few leiomyomas. Adenomas and carcinomas of the kidney and nephroblastomas were not observed. It was concluded that the aglycone of cycasin, MAM, is important in the induction of leiomyosarcomas of the kidneys in 4-day-old rats.

Age Factors↗

PubMed1996

Treatment of rupture of a liver metastasis from esophageal leiomyosarcoma.

We describe a case of rupture of a liver metastasis from esophageal leiomyosarcoma which was treated successfully by hepatic arterial embolization, thus facilitating hepatectomy. A 59-year-old woman who had previously undergone esophagectomy for leiomyosarcoma was admitted in a state of hypovolemic shock. Ultrasonography revealed multiple tumors in the left lobe of the liver and massive intraperitoneal hemorrhage, confirmed by paracentesis, possibly due to spontaneous rupture. Subsequent hepatic angiography showed extravasation from the tumor, and embolization of the feeding left hepatic artery was performed. After achieving hemostasis, a left hepatic lobectomy was carried out just beneath the reconstructed stomach tube. The patient made an uneventful recovery and remains well after one year. Emergency arterial embolization followed by hepatectomy is an appropriate treatment for patients with spontaneous rupture of liver metastases.

Combined Modality Therapy↗

PubMed1986

Cisplatin as second-line chemotherapy in the treatment of advanced or recurrent leiomyosarcoma of the uterus. A phase II trial of the Gynecologic Oncology Group.

Twenty patients with advanced or recurrent leiomyosarcoma of the uterus who failed prior chemotherapy were placed on study by the Gynecologic Oncology Group. One patient had insufficient data to permit analysis. Of the remaining 19, there was only one objective response to cisplatin 50 mg/m2 intravenously every 3 weeks. Although toxicity was tolerable, there is little evidence of drug activity in patients with leiomyosarcomas of the uterus when the drug is given as second-line chemotherapy in the dose and schedule tested.

Aged↗

PubMed1998

Amonafide in patients with leiomyosarcoma of the uterus: a phase II Gynecologic Oncology Group study.

Twenty-six evaluable patients who had leiomyosarcoma of the uterus were treated with amonafide, 300 mg/m2, for 5 consecutive days every 3 weeks. One partial response (4%) resulted. Hematologic toxicity was substantial, with grade 3 or 4 events occurring as follows: leukopenia, 12 patients (46%); thrombocytopenia, 4 patients (15%); and granulocytopenia, 7 patients (27%). One patient had transient grade 4 renal failure. Considering the poor activity and substantial toxicity that was observed, no further studies are planned by the Gynecologic Oncology Group using amonafide at this dose schedule in leiomyosarcomas.

Adenine↗

PubMed2000

Phase II trial of topotecan in patients with advanced, persistent, or recurrent uterine leiomyosarcomas: a Gynecologic Oncology Group Study.

From October 1995 to March 1997, a phase II trial of topotecan was carried out in chemotherapy-naive women with advanced, persistent, or recurrent uterine leiomyosarcomas. Thirty-six patients were entered. Median age was 53 years. Performance status was 0 (50%) in 18, 1 (36%) in 13, and 2 (14%) in 5. Most patients, 33 (92%), had undergone prior surgery, and 8 (22%) prior radiation therapy. Topotecan, 1.5 mg/m2. was administered intravenously daily for 5 days, every 3 weeks, until progression of disease or adverse affects prohibited further therapy. Patients received 1 to 13 courses with a median of 3 courses. The most frequent grade 4 adverse effects were neutropenia in 28 (78%), leukopenia in 8 (22%), thrombocytopenia in 3 (8%), and anemia in 3 (8%). Complete response was seen in 1 (3%), partial response in 3 (8%), stable disease in 12 (33%), and increasing tumor in 20 (56%). Thus topotecan at this dose and schedule does not appear to have major activity in uterine leiomyosarcomas.

Adult↗

PubMed2002

Retroperitoneal leiomyosarcoma and enlarged epididymis associated with a positive pregnancy test.

A 57-year-old man presented with a 1-month history of progressive abdominal pain and weight loss. A palpable, nonpulsatile, firm abdominal mass was felt below the xiphisternum down to the pelvis. A pregnancy test performed on a urine sample was positive. Testicular examination and testicular ultrasound were normal. Computerized tomography of the abdomen revealed a retroperitoneal mass measuring 30 x 21 x 13 cm. Serum beta-human chorionic gonadotropin (beta-HCG) was serially increased (19.71-22.71 mIU/mL). Results of histopathology tests confirmed the diagnosis of leiomyosarcoma. The level of serum beta-HCG decreased to < 0.2 mlU/mL after chemotherapy. Beta-HCG is usually increased in germ-cell tumors but few reports in the literature describe beta-HCG-secreting leiomyosarcomas. The incidence of increased levels of beta-HCG in sarcomas in general, and its potential role as a tumor marker, is not known. A simple urine pregnancy test may be done in the work-up of abdominal masses.

Abdominal Pain↗

PubMed1979

Primary leiomyosarcoma of the aorta.

A case of primary leiomyosarcoma of the abdominal aorta is reported. The tumor replaced the full thickness of the aortic wall with formation of a large saccular aortic aneurysm filled with an admixture of tumor and mural thrombus. Electron microscopy of the surgical biopsy of the tumor revealed neoplastic smooth muscle cells with myofilaments and a discontinuous basal lamina. To our knowledge, leiomyosarcoma of the aorta has never been reported.

Adult↗

PubMed1999

Leiomyosarcoma of the pulmonary veins.

Primary sarcomas of the great vessels are rare, but the most common site is the inferior vena cava. Herein are reported five new cases arising from the pulmonary veins with clinicopathologic correlation and comparison to previously reported cases. All new cases occurred in women ranging in age from 23 to 64 years at diagnosis (mean, 56 years). They had symptoms suggestive of left heart failure, including three patients with dyspnea, one with hemoptysis, and one with cough. Three cases showed tumor extension along the pulmonary veins into the left atrium. Tumors ranged in size from 2.8 to 7 cm in greatest dimension. Histologically, all were leiomyosarcomas. They were highly cellular tumors. Three cases had predominantly spindle cell morphology and two were predominantly epithelioid; one had foci of calcification. Most showed extensive necrosis. All tumors were reactive with antibodies to actin and desmin. Two cases were reactive with antibodies to MIC-2 (dotlike); two cases showed reactivity to keratin antibodies; and two showed reactivity for estrogen, progesterone receptor protein, or both. None were positive for antibodies to S-100 protein. All cases were treated with surgical excision. Follow-up ranged from 2 months to 21 years (mean, 4.8 years). Two patients were alive and well; two were alive with metastases; and one died of disease. Pulmonary vein sarcomas represent intermediate- to high-grade leiomyosarcoma. Although often lethal, complete surgical excision can lead to long-term survival. They occur predominantly in women and may express hormone receptors. Therefore, hormonal manipulation may offer promise as adjuvant therapy.

Adult↗

PubMed2000

Epstein-Barr virus-associated multicentric leiomyosarcoma in an adult patient after heart transplantation: case report and review of the literature.

Epstein-Barr virus (EBV)-associated smooth muscle tumors following solid organ transplantation are extremely rare, with only 12 cases reported in the literature thus far. The exact pathogenetic role of EBV infection in the oncogenesis of these soft tissue tumors in immunodeficient patients and the biologic behavior of such tumors is still unclear. We report a 26-year-old man in whom multiple smooth muscle tumors developed 36 to 51 months after heart transplantation. All tumors, two synchronous liver nodules, two subsequently occurring paravertebral tumors, and a single tumor in a vein at the left ankle were surgically resected. The tumor tissue was processed for routine histology and immunohistochemical (IHC) stains. Additionally, competitive polymerase-chain-reaction (PCR), reverse-transcriptase PCR (RT-PCR), as well as in situ hybridization (ISH) were used for EBV particle quantification and gene transcription analysis. The histologic features and immunohistochemical profiles were consistent with leiomyosarcoma in all tumor nodules. EBV infection was detected in >95% of tumor cell nuclei by EBER 1/2 ISH. Competitive PCR revealed 3105 EBV particles per milligram of tumor tissue. The EBV gene expression pattern analyzed by RT-PCR and IHC corresponded to the latency type III with specific expression of EBNA1, EBNA2, LMP1, and LMP2A genes. Under continuous antiviral therapy (famcyclovir) the patient currently shows no evidence of disease. Our data indicate that EBV infection plays a causal role in the development of smooth muscle tumors following organ transplantation. A latency type III, identical to EBV-associated posttransplant lymphoproliferative disorders, was identified and suggests a common pathogenetic mechanism in the development of these histogenetically distinct neoplasms. The fact that the patient currently shows no evidence of disease may be the result of the continuous administration of antiviral therapy because the soft tissue recurrences of the leiomyosarcoma occurred while the patient was not receiving antiviral prophylaxis.

Adult↗

PubMed2001

Retroperitoneal leiomyomas: a clinicopathologic and immunohistochemical study of 56 cases with a comparison to retroperitoneal leiomyosarcomas.

Most retroperitoneal smooth muscle tumors are believed to be malignant, and leiomyomas are considered very rare. This study was undertaken to determine the clinicopathologic features and long-term follow-up of 56 tumors diagnosed as retroperitoneal leiomyomas (LM) or smooth muscle tumors with an uncertain malignant potential (SMTUMP) in an effort to correlate their behavior and clinicopathologic features. These tumors were compared with a series of 11 cases of retroperitoneal leiomyosarcomas (excluding gastrointestinal stromal tumors). Histologic slides and immunohistochemistry for SMA, desmin, S-100 protein, HMB45, CD34, C-KIT, estrogen (ER) and progesterone (PR) receptor proteins, and MIB-1 were analyzed. All tumors diagnosed as LM and all but one SMTUMP were well-differentiated smooth muscle tumors that lacked atypia and coagulative necrosis. There was <1 mitosis per 50 high power field (HPF) in 38 tumors; no tumor had >3 mitoses/50 HPF. Most tumors had a striking resemblance to uterine smooth muscle tumors with common hyaline change and trabecular patterns. There were 51 females and 5 males ranging in age from 25 to 79 years (mean 45 years, median 43 years). These tumors were typically large, with a mean size of 16.2 cm and weight of 1600 g. Immunohistochemically, all 35 tumors studied were positive for alpha-SMA, 30 of 35 tumors were positive for desmin, and all were negative for CD117, S100 protein, and HMB45 and all but one for CD34. Steroid receptors were commonly present: ER in 20 of 29 cases and PR in 26 of 31 cases in the tumors of female patients. MIB-1 score was <2% in all of 28 cases. Long-term follow-up (mean 140 months) did not reveal metastases, but two patients had local recurrence; however, neither patient with recurrence demonstrated disease progression in follow-up. By contrast, all 11 leiomyosarcomas had at least mild atypia, and all were ER and PR negative. All cases had MIB-1-positive nuclei, but only four had >10% nuclei positive. Four patients died of disease, four were alive with recurrence, and three had no evidence of disease. A group of benign leiomyomas can be identified among retroperitoneal smooth muscle tumors. Most of these tumors resemble uterine leiomyomas by histology and positive hormone receptors, and they seem to have a good long-term prognosis with a small potential for local recurrence.

Adult↗

PubMed2004

Leiomyosarcoma of the penis: a clinicopathologic study of 14 cases with review of the literature and discussion of the differential diagnosis.

Primary leiomyosarcomas of the penis are very rare. To date, less than 30 have been documented in the English language literature. In this report, we describe the clinical, histopathologic, and immunohistochemical findings in 14 cases retrieved from our files. The patients ranged in age from 43 to 62 years (mean age, 51 years) at the time of initial surgical resection. The tumors involved the prepuce (n = 1), prepuce and distal shaft (n = 1), circumcision scar line (n = 2), circumcision scar line and distal shaft (n = 1), shaft (n = 5), base of the penis (n = 3), and penis, not otherwise specified (n = 1). The lesions ranged in size from 0.5 to 6.0 cm (median size, 1.5 cm) in greatest dimension. Nine tumors were superficially located, two were of indeterminate depth, and three were deep-seated. The superficial tumors were relatively asymptomatic, and seven were reportedly present for 1 year to more than 20 years (median duration, 5 years) before medical attention was sought. In contrast, one deep-seated lesion caused dysuria and difficulty voiding, prompting the patient to seek a clinical opinion within only a few months of the apparent onset. Histologically, all tumors contained smooth muscle cells with both cytologic atypia and mitotic activity. Immunohistochemical studies were available for nine tumors, and immunoreactivity for desmin was present in all instances. All patients were initially treated with a local procedure. Follow-up information is available for 9 of the 14 patients (64%), with a median follow-up interval of 12 years 11 months. Three patients had multiple (two to four) local recurrences. Two of these patients were ultimately treated with a wide local excision or partial penectomy, and both were alive and well at last follow-up. In contrast, one patient, who had four local recurrences and refused a penectomy, developed a distant metastasis 10 months after the fourth recurrence. The best predictors of outcome are tumor depth and tumor size. Superficial leiomyosarcomas of the penis are optimally managed by wide local excision whenever this is technically feasible. Tumors with a deep-seated component may require more aggressive intervention to ensure complete removal.

Adult↗

PubMed1987

Cutaneous leiomyosarcoma.

A cutaneous leiomyosarcoma arose in the upper lip skin of a 22-year-old patient. This unusual tumor was diagnosed clinically as a basal cell carcinoma although histological studies confirmed a leiomyosarcoma. The tumor was excised using Mohs technique and the wound reconstructed with a nasolabial flap and full-thickness skin graft. No recurrence or distant metastasis has appeared in a two-year follow-up.

Adult↗

PubMed1975

Primary pulmonary leiomyosarcoma: case report and review of literature.

A case of primary leiomyosarcoma of the lung is added to the literature. Previously, 66 cases were documented. This is a case of a 55-year-old male, a pulmonary cripple for many years, with a collapsed left lower lobe found by roentgenogram, verified by bronchoscopy and bronchograms. Biopsy of lesion was reported as low grade leiomyosarcoma. Despite pulmonary function studies showing a vital capacity of 37% of predicted normal, a left pneumonectomy was preformed. Postoperatively, the patient had a tachycardia which gradually decreased in the next several months. Two months following operation pulmonary function studies were essentially unchanged from those made preoperatively. Arterial gas studies are presented pre and postoperatively.

Humans↗

PubMed2001

Rare incidence of three consecutive primary tumors in the maxillofacial region: retinoblastoma, leiomyosarcoma, and choriocarcinoma: case report.

Multiple primary tumors occur more commonly in the region of the head and neck than elsewhere in the body. The chance of this is particularly high in patients treated for retinoblastoma, in part because of a genetic predisposition, and in part because of the possibility of irradiation treatment. However, triple tumors occur in only 0.5% of multiple tumors. A rare case of a triple (metachronous) tumor is reported: 12 years after the treatment of bilateral retinoblastoma (enucleation and irradiation), secondary leiomyosarcoma developed in the maxillofacial region, followed 5 years later by choriocarcinoma. Surgery was performed on all three types of tumor. As a result, the female patient (currently 21 years old) is now free of complaints and has married. It is extremely rare for either leiomyosarcoma or choriocarcinoma (CHC) to appear in the maxillofacial region. The long-term, systematic control of such patients is absolutely necessary, for the multiple tumors tend to develop only after a long latency period of 10 to 20 years.

Adolescent↗

PubMed2002

Leiomyosarcoma of the esophagus metastatic to the eyelid: a clinicopathologic report.

PURPOSE: To report a case of gastrointestinal sarcoma with leiomyosarcomatous differentiation metastatic to the eyelid. METHODS: Clinical data including a comprehensive ophthalmologic examination, histologic findings, a bone scan, and hospital records were reviewed. RESULTS: A 56-year-old woman with a history of esophageal cancer had a rapidly growing lesion on her right upper eyelid that was initially treated as a chalazion. A biopsy specimen of the lesion was consistent with a gastrointestinal sarcoma with leiomyosarcomatous differentiation. The esophageal tumor was reclassified after histopathologic evaluation of the eyelid specimen. Bony metastasis developed soon after excision of the eyelid lesion. The patient died 3 months after proper diagnosis of the eyelid lesion. CONCLUSIONS: This is a rare case of a metastatic leiomyosarcoma of the eyelid. To our knowledge, only two other cases of eyelid leiomyosarcoma have been described in the literature. The importance of correct histopathologic diagnosis of eyelid lesions is underscored in this case report.

Biomarkers, Tumor↗

PubMed1988

Delayed accumulation (flip flop) of Tc-99m DTPA in a leiomyosarcoma.

An 85-year-old woman with leiomyosarcoma occupying the abdominopelvic area had a renal dynamic study with Tc-99m DTPA. The tumor area initially showed almost no content of the radiopharmaceutical. At 24 hours, there was retention of activity in the leiomyosarcoma as compared with surrounding tissues (flip flop). It was noted that, based on literature reports, tumors of mesodermal origin (leiomyoma, spindle cell sarcoma, neurofibroma) may have the ability to concentrate Tc-99m DTPA.

Aged↗

PubMed1995

Uterine leiomyosarcoma with clear cell areas.

An unusual case of a uterine leiomyosarcoma with a clear cell component in an 82-year-old woman who presented with vaginal bleeding and a mass protruding through the cervix is reported. Histologic examination of the biopsy sample showed only pleomorphic clear tumor cells and necrosis. Because the patient had a history of a breast carcinoma, and a computed tomography scan of the abdomen showed a renal cyst, these lesions created some problems in the differential diagnosis of the patient's clear cell neoplasm. The patient was treated with a hysterectomy, and examination of the uterus showed an epithelioid leiomyosarcoma with areas of clear cells. Four months after surgery, the patient presented with a vaginal recurrence, which was resected. Twelve months later, the patient had no evidence of recurrence.

Aged↗

PubMed2004

Leiomyosarcoma of the uterus with focal rhabdomyosarcomatous differentiation.

An unusual myometrial tumor was encountered in a 70-year-old female who presented with lower abdominal fullness and symptoms related to pulmonary metastases. Laparotomy revealed a uterine mass that was removed by total abdominal hysterectomy and bilateral salpingo-oophorectomy. Multiple liver metastases also were noted. Pathologic examination of the hysterectomy specimen revealed a 25-cm, well-circumscribed myometrial mass that had a firm, white-to-yellow, focally whorled, sectioned surface with focal hemorrhage and necrosis. Within the main mass was a discrete, 7-cm, soft, gelatinous, reddish-yellow nodule. The main tumor was a well-to-moderately differentiated leiomyosarcoma, whereas the gelatinous nodule was rhabdomyosarcomatous and contained rhabdomyoblasts that exhibited cytoplasmic cross striations and immunoreactivity for myoglobin. No epithelial elements were detected in the neoplasm. At the time of last follow-up, the patient was undergoing chemotherapy. This is only the second well-documented case of a hybrid leiomyosarcoma-rhabdomyosarcoma of the uterus.

Aged↗