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We report the case of a 26-year-old man with diffuse esophageal leiomyomatosis involving the trachea. The tumor was resected by total esophagectomy and partial resection of the trachea and the left main bronchus. The tracheobronchial defect was repaired with a free forearm skin graft with satisfactory outcome. This approach offers good long-term prospects.

A case of intravenous leiomyomatosis of the uterus with extension into the heart discovered in a 34-year old patient who suffered from dyspnea is reported. The findings suggest that intravenous leiomyomatosis originates from the smooth muscle component of uterine veins. Only 30 cases of intravenous leiomyomatosis with intracardiac extension have been reported. Two-dimensional echocardiography showed an abnormal mass in the right atrium. Pelvic echography showed an abnormal mass in the pelvis. In case of cardiac involvement, spontaneous prognosis remains poor. The primary goal of surgery is the complete removal of the tumor. Hormonotherapy should also be considered in case of unresectable residual tumor. A long-term follow-up of patients after resection of intravenous leiomyomatosis is needed because recurrences up to 17 years after primary resection have been reported.

Purpose: To reassess the clinical and radiologic findings in patients with esophageal leiomyomatosis., Materials and Methods: A search of the authors' radiologic archives revealed six cases of esophageal leiomyomatosis in a 22-year period. The clinical findings and radiologic images were reviewed retrospectively., Results: The average age of the patients was 10.8 years (range, 6-18 years). Five patients presented with slowly progressive dysphagia. Barium studies revealed smooth, tapered narrowing of the distal esophagus in five patients and characteristic defects on the superomedial aspect of the gastric fundus abutting the cardia, presumably due to bulging of this thickened mass of muscle into the stomach, in four patients. In two patients, computed tomography (CT) revealed marked thickening of the distal esophageal wall., Conclusion: Esophageal leiomyomatosis can be suggested in a pediatric patient with long-standing dysphagia in whom smooth, tapered distal esophageal narrowing is seen at barium study and circumferential esophageal wall thickening is seen at CT.

We report two cases where high-resolution computed tomography (HRCT) assisted in the diagnosis of a patient with lymphangioleiomyomatosis and a patient with pulmonary tuberous sclerosis. HRCT was used in both cases where the chest radiographs and conventional computed tomographic scans appeared relatively normal but the conditions were strongly suspected.

Adenomyosis is an underestimated pathology frequently responsible of unknown origin pelvic pain and uterine enlargement. It is hyperestrinic condition with poor specific symptomatology and preoperative diagnostic tools are very few. The most important of them, ultrasound, has improved its diagnostic capacity with the introduction of transvaginal way of examination. However, for the ultrasound resemblance, the common etiopathogenetic origin and the symptomatologic likeness between adenomyosis and uterine leiomyomatosis, a problem still open is the differential diagnosis between these two pathologies especially when both are present in the same subject. In our study where the preoperative ultrasound was followed by surgical finding, we try to give some ultrasonographical guidelines to discern among these two pathologic conditions.

Objective: To study the response of uterine leiomyomata to three daily doses of RU486 (5, 25, and 50 mg)., Design: Prospective nonrandomized trial of women with symptomatic leiomyomata., Setting: Patients from the clinical practice of the authors at the University of California, San Diego Medical Center., Patients: Ten patients with symptomatic leiomyomata previously reported after treatment with 50 mg of RU486 daily for 3 months. Eleven patients treated with 25 mg of RU486 daily and nine patients placed on 5 mg of RU486 daily for 12 weeks., Main Outcome Measures: Changes in leiomyomata volume as measured with vaginal ultrasounds at baseline and monthly thereafter. Frequent blood samples for hematology, chemistry, and hormone levels were obtained. Twenty-four-hour urine collections for free cortisol and creatinine were obtained at baseline and at 12 weeks., Results: All three doses induce ovarian acyclicity. Administration of 50 mg of RU486 decreases leiomyomata volume to 78.1% +/- 4.8% of baseline at 4 weeks, 60.5% +/- 6.6% at 8 weeks, and 51.0% +/- 9.2% after 12 weeks of treatment. Regressive response in patients treated with 25 mg of RU486 daily was 76.3% +/- 5.0% of baseline at 4 weeks, 54.0% +/- 5.1% at 8 weeks, and 44.0% +/- 5.0% after 12 weeks. At 5 mg of RU486 leiomyomata volume was 80.6% +/- 8.3% of baseline after 4 weeks, 63.7% +/- 14.6% after 8 weeks, and 74.4% +/- 19.8% after 12 weeks of therapy., Conclusions: Although acyclicity is seen at all three doses, an effective dose to cause a clinically significant (50%) decrease in leiomyomata volume appears to be 25 mg daily.

Intravenous leiomyomatosis (IVLM) is a condition characterized by intravenous growth of histologically benign smooth muscle tumour originating in the uterus. Rarely, the tumour can extend via the inferior vena cava into the right side of the heart. In only one previously described case has the tumour extended into the pulmonary artery. We present a second such tumour. In this case, tumour calcification could be identified during cine recording moving within the pulmonary outflow tract, a finding not commonly associated with this condition. The radiological investigation of IVLM is briefly discussed.

Objective: To study the complications related to leiomyomatosis in pregnancy by clinical and ultrasonographic assessment., Design: A retrospective study., Subjects: All pregnancies admitted to the 2nd Institute of Gynecology and Obstetrics, Policlinico Umberto I, in the period between January 1992 to December 1993 were surveyed., Results: Gestational age at the time of ultrasonographic neoplasm diagnosis was 25.1 +/- 13.4 weeks, 'we found no correlation between maternal age or parity affecting pregnancy outcome, Leiomyomatosis complicated pregnancy rate was 1.68%. Myomatosis was diagnosed clinically in 25 of 67 cases (37.3%). Regarding the location of the neoplasm, 59% was located in the corpus-uteri, 21% was considered a diffuse neoplasm and the 14% was located in the fundus. Threatened abortion was the most frequent complication (20%), abortion was the second (16.4%). We observed an increased abortion threat rate (p < 0.001) in those cases where the leiomyoma was in relation with the placenta. We had a surgery rate of 76% in pregnancies complicated by myomatosis, and the indication for surgery was given either primarily or exclusively by the presence of myomatous formation in 19 cases (50%)., Conclusions: Our study suggests that location of the leiomyoma in relation to the placenta is a higher risk factor than its size, and that there is a higher risk for threats of abortion and abortion rates in pregnancies complicated by leiomyomatosis. We recommend that every pregnant woman with a suspected myoma should be ultrasonographically scanned.

A case of intravenous leiomyomatosis with extension into the right ventricle is described. A tumor in the inferior vena cava was detected 5 years after a hysterectomy had been performed for a myomatous uterus, but was misdiagnosed as an intracaval thrombus. Three years later this tumor was discovered after it had enlarged and intruded into the right ventricle. The correct diagnosis was made after a complete evaluation. The patient underwent surgery employing simultaneous sternotomy and laparotomy. Radical excision was achieved using cardiopulmonary bypass with hypothermic circulatory arrest. The diagnostic and surgical approaches are reviewed and discussed.

A 30-year-old woman had a history of a uterine fibroid 6 years before admission. She had resection of a right atrial mass diagnosed as a leiomyoma 2 years ago and a second cardiac procedure for recurrent tumor 1 year ago. Pathologic examination at that time indicated that the tumor was a low-grade endometrial stromal sarcoma (stromatosis) with features of benign leiomyoma (intravenous leiomyomatosis). This time she was admitted with facial and lower extremity swelling as well as ascites. Transthoracic and transesophageal echocardiography revealed a large tumor entering the heart from the inferior vena cava and filling the right atrium and ventricle. Lower extremity ischemia from bilateral compartment syndrome due to severe edema developed, and she underwent successful surgical resection of the tumor that filled the right side of the heart, inferior vena cava, and mesenteric and renal veins.

A 6-year-old female Quarter Horse became inappetent, lost weight, was febrile, and had abdominal enlargement and infrequent bouts of mild colic. Palpation per rectum detected a prominent, smooth-walled mass adjacent to the pelvic flexure of the colon. Analyses revealed the peritoneal fluid was apparently normal except for a high protein concentration. By the use of ultrasonography, the mass was observed adjacent to the body wall in many locations throughout the abdomen, appearing as an irregular, multiloculated structure composed of innumerable cysts of various sizes with interspersed, circular hyperechoic nodules. A celiotomy was performed, and the 34-kg mass was removed. On the basis of histologic examination, a diagnosis of disseminated peritoneal leiomyomatosis was made. The horse had an uncomplicated recovery and gained weight. Further problems have not been reported and the horse subsequently gave birth to a healthy foal. A complete examination of the horse 22 months after surgery did not reveal signs of redevelopment of the condition.

The case of a patient with severe fever in the puerperium is presented. Ultrasound, CT, and MRI revealed multiple myomas; all the myomas, even the smallest ones, were hypodense on pre- and postcontrast enhanced CT and presented with high signal intensities on T2-weighted MRI. After hysterectomy, histology demonstrated necrosis of all leiomyomas without hemorrhage or inflammatory reaction. Therefore, fever was caused solely by extensive necrotic degeneration of the fibroids, probably due to hypoperfusion related to delivery or hormonal changes during pregnancy and puerperium.

Leiomyomatosis peritonealis disseminata is a very rare disorder. The number of cases documented in the literature is only 44. Of these cases, only one was in the postmenopausal period. We report a new case of leiomyomatosis peritonealis disseminata in a postmenopausal woman.