Intravenous leiomyomatosis with intracardiac involvement.

Intravenous leiomyomatosis is considered to be a rare neoplastic disease usually arising from uterine fibromyomata, but its true incidence may be under-recognized. Mortalities from this condition are usually the result of intracardiac involvement. A case report of successful treatment of intravenous leiomyomatosis with intracardiac extension is presented, together with a review of the existing literature concerning this rare condition.

[A case of so-called benign metastasizing leiomyoma responsive to progesterone].

A 47-year-old female, who had undergone hysterectomy and unilateral oophorectomy in 1975, was admitted to our hospital in 1989 because chest X-ray films revealed an increase in size and number of pulmonary nodules for two years. On admission, a left inguinal tumor was found and histologically it consisted of smooth muscle cells with nuclear atypia arranged in interlacing fascicles. An open-lung biopsy was performed. Pulmonary tumors revealed similar histology to the inguinal tumor. They were diagnosed as metastatic low-grade leiomyosarcoma, so-called benign metastasizing leiomyoma (BML), on the basis of location and history, reinforced by mild histologic atypia. The tumor contained a high progesterone receptor level (400 fmol/mg). Therefore, medroxyprogesterone acetate, 600 mg daily, was administered orally. At two years the pulmonary lesions had regressed. BML is a rare condition, considered to be pulmonary metastasis from low-grade leiomyosarcoma of the uterus. Measuring estrogen and progesterone receptors in lung biopy material may help determine the most appropriate therapy.

[Benign metastatic leiomyoma of the lung]. / Gutartige metastasierende Leiomyome der Lunge.

The authors report on the case of a woman of 41 years of age presenting with asymptomatic multiple circular foci in the lung that were primarily suspected of being malignomas. Diagnosis of benign metastasising leiomyomas was confirmed for bilateral multiple circular foci suspected of lung metastasising originating from an unknown tumour of the lung. This diagnosis was achieved by open biopsy. The seemingly contradictory diagnosis of a benign but metastasising tumour is characteristic of a rare disease pattern that, however, is well documented in the literature. The course of the disease is compared with the data given in the literature.

Response of "benign" metastasizing leiomyoma to progestin withdrawal. Case report.

We report a rare case of a benign metastasizing leiomyoma in which progestin therapy permitted or promoted pulmonary metastases, but progestin withdrawal induced a marked tumor regression. The significance of these observations is discussed relative to the estrogen and progesterone receptor positivity of this patient's tumor.

[Intravenous leiomyomatosis of the inferior vena cava and the right heart atrium. Apropos of a case and review of the literature]. / Léiomyomatose intra-veineuse cave inférieure et cardiaque droite. A propos d'un cas et revue de la littérature.

The authors report a case of intravenous leiomyomatosis, a rare uterine tumor, extending to the inferior vena cava and to the right atrium. It seems to be the first case described in France. The wrong diagnosis of massive pulmonary embolism and the distance from a cardiac surgery unit led to emergency tumorectomy without cardiopulmonary bypass. A review of the literature studied the 24 cases with cardiac extension already reported, 19 operated and 5 autopsy reports. Right cardiac failure or syncopes are the most frequent clinical signs. Sometimes histologic examination after hysterectomy leads to the diagnosis. Echocardiography diagnoses an intra-atrial mass. Abdominal ultrasonography and phlebocavography show the iliocaval portion of the tumor. Cardiac angiography and computed tomography are also contributive. Surgical treatment except in extreme conditions should be performed by a cardiac surgical team. If the diagnosis of intra-venoux leiomyomatosis has been made preoperatively a one-stage cardiac and abdominal treatment should be preferred. In the other cases, cardiac surgery should be done first, allowing a precise histologic diagnosis and subsequent treatment of the iliocaval and uterine lesions.

Stromal neoplasms of the rectum and anal canal.

Nineteen mural-based stromal tumors of the rectum and anal canal were reviewed, with the objective of delineating pathologic features discriminative of malignancy in these uncommon neoplasms. Ten locally excised tumors failed to recur during long-term follow-up and were considered benign. All occurred in the submucosa and ranged in size from 1.0 to 7.0 cm (mean, 2.1 cm). Three were sparsely cellular; seven had the appearance of gastric-type cellular leiomyomas. All lacked nuclear atypia and displayed mitotic activity not exceeding 1 mitosis/50 high-power microscopic fields. In contrast, of nine tumors exhibiting malignant behavior, eight (89%) were located in the muscularis propria. Their mean size was 4.5 cm (range, 1.6 to 11 cm). Necrosis was present in six tumors (67%). Seven sarcomas retained a cellular leiomyomatous appearance but exhibited moderate cytologic atypia. Mitotic counts ranged from 5 to 58 mitoses/50 high-power microscopic fields. Three locally excised sarcomas recurred in the rectum at 2, 2, and 7 years. In five patients tumor recurred in the pelvis. Five patients died of disease 0.67, 1.2, 3, 5, and 11 years post-diagnosis. One patient died with sarcoma at 31 years. Three patients are without evidence of recurrent neoplasm 5, 5, and 7 years postresection. Our data indicate that not all anorectal, mural-based stromal neoplasms are a priori malignant. While location within the muscularis propria, size, nuclear atypia, and tumoral necrosis correlate with malignancy, mitotic activity is the cardinal indicator of sarcomatous behavior in stromal neoplasms of the rectum and anal canal.

[Osseous leiomyoma--a first-time description]. / Das ossäre Leiomyom--Eine Erstbeschreibung.

In a 54-year old housewife presenting with left hip pain due to a circumscribed intraosseous lesion of the femoral neck, histological assessment following resection showed a benign intraosseous leiomyoma. Years ago a diagnosis of disseminated peritoneal leiomyomatosis had been made.

Benign metastasizing leiomyoma. A case report and review of the literature.

This is a case report of an asymptomatic, 65-year-old white female who was evaluated for multiple pulmonary nodules. This patient's presentation, clinical course and pathologic specimens are consistent with benign metastasizing leiomyoma. A review of this rare disorder is included in this report with emphasis on past cases, clinical overview and treatment.

Recurrent intravenous leiomyomatosis with cardiac extension.

A case of recurrent intravenous leiomyomatosis with cardiac extension and a temporally extended presentation is described. Complete excision was achieved employing simultaneous sternotomy and laparotomy and deep hypothermia with circulatory arrest. Coronary revascularization was performed concomitantly with complete tumor resection. Diagnostic, operative, and pathologic considerations are reviewed and a preferred surgical approach discussed.

The intriguing nature of gastric tumors in Carney's triad. Ultrastructural and immunohistochemical observations.

The authors describe clinical and pathologic features present in an adolescent girl who had a gastric tumor and mediastinal mass. The latter was shown to be a paraganglioma, and the gastric neoplasm was classified as malignant "leiomyoblastoma," with the use of current histologic criteria. This tumor had metastasized to the liver but not to the lungs. Although the histologic criteria for leiomyoblastoma were fulfilled, no definite evidence of smooth-muscle cell differentiation was present ultrastructurally or by immunostaining methods. Gastric tumors that form part of "Carney's triad" are known to differ clinically and pathologically in important ways from smooth-muscle cell malignant neoplasms that are not part of this syndrome. Some have been classified as gastrointestinal autonomic nerve tumors, but the current study did not confirm this contention. The nature of gastric leiomyoblastomas in Carney's multitumoral association remains undecided.

A case of multiple leiomyomatous lesions of the lung: an analysis of flow cytometry and hormone receptors.

A 36 year old woman was admitted to our department because of a chest X-ray which showed multiple developing shadows. She underwent bilateral exploratory thoracotomies and a total 5 tumors were resected and pathologically diagnosed as benign metastasizing leiomyoma, the largest of which was positive for the progesterone receptor and negative for the estrogen receptor. A histogram of this tumor using a flow cytometer showed a diploid pattern and 4.6 percent of the S phase which was not more than that of a leiomyoma of the uterus from another patient. Two months later, she underwent a hysterectomy and bilateral salpingo-oophorectomy for treatment of the positive progesterone receptor in the pulmonary lesions. The resected uterine myoma and normal myometrium showed positive estrogen and progesterone receptors. For the subsequent 28 months she has been free of any further symptoms. Benign metastasizing leiomyoma of the uterus is a rare disease and very interesting because of its histological benignity and hormonal dependency. However, according to the literature, it is often confused in entity due to the fact that normal lung tissue also possesses hormone receptors. Considering our data on hormone receptors, it is rational to think that multiple leiomyomatous lesions in the lung should only be diagnosed as benign metastasizing leiomyomas when they possess positive estrogen and progesterone receptors.

Fine-needle aspiration of gastric leiomyoblastoma metastatic to the subcutis.

Leiomyoblastomas are rare tumors, and there have been few reports on their fine-needle aspiration (FNA) cytologic morphology. We herein describe the FNA features of a gastric leiomyoblastoma with subcutaneous metastasis. The cells had eosinophilic cytoplasm and oval eccentric nuclei, occasionally with intranuclear cytoplasmic inclusions. An organoid pattern was obvious in cell block sections. The tumor was positive for vimentin but negative for desmin, S-100 protein, and the common epithelial markers. The histogenesis is discussed, as are reasons why this tumor is better termed epithelioid mesenchymal tumor.

Uterine benign-appearing smooth muscle tumor "metastatic" to the lungs: a case report.

A case of pulmonary fibroleiomyomatosis secondary to a uterine "metastasizing" leiomyoma, with aspects of vascular invasion, is reported. Although we cannot exclude the existence of primitive pulmonary forms, which we do not consider to be of a hamartomatous nature, we believe that a careful examination of the uterus for the presence of angioinvasive leiomyoma is mandatory.

[Carney's triad: a new case associated with adrenal cortex adenoma]. / Triade de Carney: un nouveau cas associé à un adénome cortico-surrénalien.

Carney's triad is a combination of at least 2 of 3 tumor sites: multiple gastric leiomyoblastoma, pulmonary chondroma and paraganglioma, most often extra-adrenal and secreting. Thirty-three cases have been published; we report a new case in a 20 year-old woman, with multiple gastric leiomyoblastomas, liver metastasis, and bilateral pulmonary chondroma. The research of paraganglioma, in regard to Carney's disease, led to the discovery of corticoadrenal adenoma.

[Intra-caval leiomyomatosis. Surgical treatment]. / Leiomyomatose intra-cave. Traitement chirurgical.

The authors report a case of intracaval leiomyomatosis diagnosed in a 11-year old female, who had been initially operated for a massive tumor of uterus (35 X 45 X 25) and who had had subtotal hysterectomy performed. The histologic diagnosis at was intravenous leiomyomatosis. Investigation of extent of neoplastic spread included echography, cavography and revealed inferior vena cava involvement. Cavotomy removed a tumor fragment. Intravenous leiomyomatosis has always its source in the uterus and spreads by progressive invasion of the vena cava. The right cardiac cavities and, at times, the pulmonary artery are also involved. Management of cardiac invasion is a determinant factor of the severity of the disease. Indication for surgery is systematic and should result in complete tumor excision.

[Carney's syndrome disclosed by hemoperitoneum]. / Syndrome de Carney révélé par un hémopéritoine.

The authors report a case of Carney's triad in a blood-group O woman who successively sustained a) at age 13, a multicentric gastric leiomyoblastoma revealed by an hemoperitoneum; b) 6 years later, a pulmonary chondroma diagnosed by chest standard roentgenogram and CT scan, associated with leiomyoblastoma recurrence and hepatic metastases; c) 10 years later, a juxta-carotid tumor of unknown (paraganglion?) origin. This patient is presently in good clinical condition, in spite of hepatic metastases known for 5 years. Based on this case and the 33 previously reported cases, the main features of this very unusual entity are analyzed.

[Leiomyoma of the esophagus and pulmonary metastasis]. / Léiomyome de l'oesophage et métastase pulmonaire.

An important and symptomatic esophageal leiomyoma is treated by enucleation. A secondary metastasis, also important and symptomatic, is resected 3 years later. The follow-up is now 2 years, without any sign of relapse. This case allows for discussion of the malignancy of leiomyoma and of its microscopic criteria.

Eighteen-year survival of unresected leiomyoblastoma of the stomach with liver and lymph node metastases.

A case of gastric leiomyoblastoma with liver and lymph node metastases occurring in a 31-year-old man, who has been alive with the tumor but without symptoms for more than 18 years after only an exploratory laparotomy without resection, is reported. On laparotomy, the liver metastasis and paragastric and para-aortic lymphadenopathies made it impossible to resect the stomach with the tumor. A diagnosis of malignant leiomyoblastoma was confirmed by incision of the liver and paragastric lymph nodes. Postoperative chemotherapy with mitomycin C and cyclophosphamide was performed for 6 months. As demonstrated by roentgenography, echography and computed tomography, the size of the tumor has remained almost identical for an 18-year period. Histologically, the low mitotic rate of the tumor in this case is considered primarily responsible for the long survival of the patient. A review of the records has revealed that our patient is the longest survivor to have unresectable gastric leiomyoblastoma with liver and lymph nodes metastases.