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19p deletion in recurring leiomyosarcoma lesions from the same patient.

Ten leiomyosarcomas (LMS) affecting the same patient over a period of 3 years were cytogenetically studied to detect nonrandom chromosomal changes with a pathogenetic significance. All tumors, likely metastases of a previous LMS presentation, were classified as small, including eight that developed before chemotherapy; the diagnoses were based on standard immunohistochemistry methods for smooth muscle tumors. Scoring of 613 metaphases revealed monosomy of chromosome 22 in six LMS, monosomy of chromosome 19 in three, and deletion of chromosome 19p in all ten. Interphase fluorescence in situ hybridization (FISH) of the 22-alphoid-specific probe allowed loss of the target chromosome to be detected in four tumors at higher frequencies than those detected by cytogenetics. Double-color FISH of the 19p- and 19q-specific YAC performed on one tumor made it possible to distinguish the monosomic and 19p deleted cells, the relative frequencies of which were found to be 10% and 20%, respectively. The deletion breakpoint could be mapped at 19p13 between YAC 957d12 and YAC 947g4. The recurrence of the 19p deletion in a subset of tumor cells from all of the analyzed LMS suggests that this structural aberration is a significant change in the development of leiomyosarcomas.

Adult↗

Primary leiomyosarcoma of the skin. Case report and review of the literature.

Leiomyosarcoma is a rare tumor usually treated by wide local excision. We present a case of primary leiomyosarcoma of the skin on the anterior aspect of the chest wall treated by Mohs micrographic surgery. This is only the second reported case so treated. The patient has remained recurrence-free for 30 months.

Humans↗

[Primary hepatic leiomyosarcoma in a patient with gastric non-Hodgkin lymphoma].

Primary leiomyosarcoma of the liver is a rare entity and fewer than 100 cases have been reported in the medical literature. Even more unusual is the association with another previous tumour. We report the case of a 84-year-old man who had been treated for a gastric non-Hodgkin lymphoma two years previously. He presented with a painful mass in the right upper abdomen. An ultrasound scan showed a large mass in the right lobe of the liver and chest radiography showed pulmonary metastases. Liver biopsy was performed and immunohistochemical analysis revealed a malignant leiomyosarcoma. We discuss this case and review the available literature.

Aged↗

Hyponatremic hypertensive syndrome and massive proteinuria in a patient with renin-producing leiomyosarcoma.

We report a case of renin-producing leiomyosarcoma associated with the hyponatremic hypertensive syndrome and nephrotic-range proteinuria. Extremely high levels of active renin and, to a greater extent, of prorenin were found in plasma and tumor tissue. Immunohistochemical and in situ hybridization studies demonstrated that the neoplastic cells were the source of renin production. The hyponatremic hypertensive syndrome and proteinuria promptly responded to treatment with angiotensin-converting enzyme inhibitors, suggesting an angiotensin II dependency of these disorders. After removal of the leiomyosarcoma, plasma concentration of active renin, but not of prorenin, normalized and the hypertension, proteinuria, and electrolyte abnormalities disappeared. However, 5 months after operation, the patient presented once again with hypertension, hypokalemia, proteinuria, and markedly increased plasma levels of both active renin and prorenin that heralded the relapse of neoplastic disease.

Enzyme Precursors↗

Leiomyosarcoma of mandible in a Sudanese female.

Leiomyosarcomas are extremely rare in the oral region. A case of a malignant tumour of the smooth muscle of the mandible possibly originating from a pre-existent benign counterpart is reported and the literature briefly reviewed. The case presented suggested the possibility that leiomyosarcomas, whenever they originate as a result of malignant transformation in a leiomyoma, may grow to an enormous size and grow slowly.

Adult↗

Unsuspected uterine leiomyosarcoma discovered during treatment with a gonadotropin-releasing hormone analogue: a case report and literature review.

We report a case of a 37-year-old woman who had received five courses of gonadotropin-releasing hormone (GnRH) agonist (Decapeptyl) for presumed uterine leiomyomata associated with episodes of uterine bleeding. Submucous myoma (histologically proven) was partially removed on the first visit. After a period of significant reduction in the tumor size and cessation of uterine bleeding, the symptoms recurred along with rapid re-growth of the uterus. Total abdominal hysterectomy was performed and the pathologic evaluation revealed leiomyosarcoma with a high mitotic rate. This case and the literature review emphasize the problems encountered with the early diagnosis of uterine leiomyosarcoma during GnRH agonist therapy.

Adult↗

Primary cerebral leiomyosarcoma.

A case of a rare primary cerebral leiomyosarcoma in an 8-year-old male is described. The patient presented with a new-onset seizure disorder and was found to have a rapidly expanding left parietal extra-axial lesion, documented by radiological imagings. The patient underwent surgical resection of the leiomyosarcoma, as well as adjuvant radiotherapy and chemotherapy. He is still surviving to date in stable neurological condition. The clinical presentation, surgical procedure, pathological findings and post-operative clinical course will be reported. The possible etiology of this rare extra-axial neoplasm is discussed.

Brain Neoplasms↗

Metastatic potential of gastric leiomyosarcoma.

Metastatic and lethal gastric leiomyosarcoma in 12 of 30 surgically treated cases was found most closely correlated with larger size tumors. A highly statistically significant correlation (p less than 0.0001) between tumor diameter and success or failure of surgical extirpation was demonstrated for the total of 241 gastric leiomyosarcomas in the present series and collected from the literature. Six other gross and microscopic attributes analyzed, including mitotic counts, did not provide equally useful prognostic indications. Inadequate sampling may be responsible for lack of correlation between histological criteria and tumor progression.

Humans↗

Cardiac metastasis from uterine leiomyosarcoma.

The clinical course and echocardiographic and hemodynamic derangements in a case of uterine leiomyosarcoma metastatic to the heart are discussed. The literature on cardiac metastasis in general and cardiac involvement by leiomyosarcoma in particular is reviewed. Although the presentation of cardiac tumors is highly variable and the diagnosis is usually made postmortem, antemortem diagnosis is possible, as in this case, if a high index of suspicion is combined with information from two-dimensional echocardiography and cardiac catheterization.

Adult↗

[Leiomyosarcoma of the broad ligament].

INTRODUCTION: Leiomyosarcoma of the broad ligament is an extremely rare malignant tumor, with only 13 cases reported. OBSERVATION: A 52 year-old woman consulted for pelvic pain and metrorrhagia. Clinical examination revealed a multinodular mass on the right side of the uterus. Pelvic ultrasound revealed a heterogeneous mass. Laparotomy found a malignant tumor arising from the right broad ligament. It was independent from the proximity organs. Treatment consisted in resection and total hysterectomy with bilateral salpingo-oophorectomy. The histologic examination led to the diagnosis of leiomyosarcoma. The patient died three months later. COMMENT: This is an exceptional case. Most authors underline the poor prognosis when tumoral relapse occurs early, as was the case in our patient.

Broad Ligament↗

Unusual feature of jejunal leiomyosarcoma studied with US and CT-enteroclysis: a case report.

Leiomyosarcoma is a mesodermal malignant neoplasm affecting the small intestine. Usually, the clinical signs are not specific and most commonly related to intestinal bleeding or obstruction. We report the case of a 71-year-old woman presenting anemia and melena: there was no evidence of abdominal palpable mass and endoscopic examinations were negative for pathologic findings. We performed sonography and CT-enterclysis. We detected a small jejunal mass presenting a heterogeneous density and echogenicity pattern, with some large peripheral calcifications. Histologic examination after surgery revealed the lesion to be a small bowel leiomyosarcoma.

Aged↗

Primary leiomyosarcoma of the pulmonary artery: a diagnostic dilemma.

Primary leiomyosarcoma of the pulmonary artery is a rare malignancy arising from the multipotential mesenchymal cell of the intima of the pulmonary artery. Due to its rarity and nonspecific clinical symptoms, the correct diagnosis and proper management are often delayed. Furthermore, it is frequently misdiagnosed as pulmonary embolism, mediastinal mass, pulmonary stenosis and lung cancer. Therefore, it is important to consider primary leiomyosarcoma of the pulmonary artery a possibility when a persistent filling defect is present in the pulmonary artery and there is no response to optimal anticoagulation treatment. Radiologic findings such as a unilateral mass continuously filling the pulmonary artery, inhomogenous enhancement, vascular distension, extravascular invasion into adjacent structure or uptake in the area of tumor on the FDG-PET can be helpful when differentiating pulmonary artery sarcoma (PAS) from chronic thromboembolism.

Aged↗

Renin-producing leiomyosarcoma originating in the uterus.

We present a case of uterine leiomyosarcoma in a 60-year-old woman, who had severe hypertension with hypokalaemia and metabolic alkalosis. Investigation revealed an elevated serum renin level; the tumour stained for renin on immunocytochemistry. The serum renin level fell on successful treatment of the primary. We suggest that this is the first reported case of uterine leiomyosarcoma shown to be producing renin.

Female↗

Primary leiomyosarcoma of the anterior mediastinum.

Primary mediastinal leiomyosarcoma are extremely rare tumors, which develop from smooth muscle, usually in the esophagus or main vessels. Very few cases have been reported in the literature and only two of these developed from small vessels in the soft tissue of the anterior mediastinum. We report on a 45-year-old man who presented with a huge anterior mediastinal tumor. The tumor was surgically removed with right thoracotomy and the diagnosis of well-differentiated leiomyosarcoma was established. It probably originated from the anterior mediastinal tissue, so at operation a continuity was not found between the tumor and neighboring structures. This rare and interesting histopathology is discussed and the literature is reviewed.

Adult↗

Asymptomatic right atrial leiomyosarcoma with tricuspid valve obstruction in a young female patient.

Primary cardiac leiomyosarcomas of the heart are rare tumors usually diagnosed post-mortem. Like other sarcomas located in the heart, it causes symptoms by obstruction or occlusion of cardiac cavities, local invasion, embolization or by systemic manifestations. We present an unusual case of a previously healthy young female patient who was accidentally diagnosed with a cardiac tumor of unknown origin during routine physical examination in May 1997. Until a few days prior to elective cardiac surgery for diagnostic purposes in June 1997, no clinical symptoms were present. To our surprise, a primary right atrial leiomyosarcoma was found which almost completely occluded the right atrium and destroyed the tricuspid valve. Despite the combination of surgical removal and adjuvant chemotherapy the patient died 3 weeks after the operation due to progressive tumor disease and development of congestive heart failure and lung embolism.

Adult↗

Therapeutic failure of uterine fibroid embolization caused by underlying leiomyosarcoma.

The authors describe an unusual case in which continued growth of uterine fibroids in a postmenopausal patient after polyvinyl alcohol embolization therapy prompted hysterectomy, which revealed an underlying leiomyosarcoma. The surgery was nearly fatal as a result of venous bleeding, and parasitization of blood from adjacent bowel by the tumor was noted. The difficulty of preoperative diagnosis of leiomyosarcoma and the need for diligent follow-up after uterine fibroid embolization are discussed.

Embolization, Therapeutic↗