1/32. Pulmonary lymphangiomyomatosis: three new cases studied with electron microscopy. Three cases of pulmonary lymphangiomyomatosis are described, with emphasis on the ultrastructural changes. The clinicopathologic features corresponded to those previously described. Each patient was a female in the reproductive years; breathlessness and recurrent pneumothoraces were the predominant clinical features. Histologically, the lungs showed a focal interstitial infiltrate of short, spindle-shaped mononuclear cells compatible with primitive smooth muscle, which was associated with irregular emphysema and hemosiderosis. Electron microscopy confirmed the smooth muscle nature of the pulmonary infiltrate and showed the presence of cells intermediate between smooth muscle and fibroblasts. Abnormalities were also noted in the pulmonary connective tissue that are possibly related to the fragility of the lung in this condition. ( info) |
| edwardsiella tarda (E. tarda) has recently become recognized as a pathogen in humans. Here we report a new case of E. tarda bacteraemia complicated by acute pancreatitis and pyomyoma. A 46-year-old female came to our emergency room complaining of sudden onset of left upper quadrant pain and vomiting for the previous few hours after drinking three bottles of wine. An abdominal computed tomography (CT) scan revealed multiple biliary stones, acute pancreatitis with extensive inflammatory change, and a large uterine myoma. fever, watery diarrhoea, and mild suprapubic discomfort with vaginal spotting were noted soon after admission. The patient's blood cultures yielded E. tarda and symptoms subsided after antibiotic therapy. fever and severe suprapubic pain with rebound tenderness developed 12 days later. Repeat abdominal CT scan revealed an enlarged uterine myoma with central necrosis. The patient subsequently underwent anterior total hysterectomy and bilateral salpingo-oophorectomy, revealing a uterine myoma with infarction and abscess formation. The patient recovered uneventfully and was discharged 1 week later. ( info) |
3/32. Pregnancy after embolization of uterine myoma: report of 12 cases. OBJECTIVE: To treat uterine myomas with embolization, to look for pregnancy-induced myoma recurrences, and to assess pregnancy course and outcome after embolization. DESIGN: Observational clinical study. SETTING: University of paris VII hospital.Patient(s): Nine women had embolization for symptomatic myoma, with 12 pregnancies observed. INTERVENTION(s): Embolizations were highly selective and performed bilaterally through the uterine arteries with polyvinyl alcohol. MAIN OUTCOME MEASURE(s): pregnant women were evaluated by physical and sonographic examinations. RESULT(s): Before embolization, the mean uterine volume was 450 cm(3), and in six patients polymyomas were present. The median age at embolization was 40 years; the median delay before pregnancy was 9 months; and the median age at first pregnancy outcome was 41 years. Five early miscarriages occurred. The seven other pregnancies were uneventful, although three premature births and one case of late toxemia occurred unrelated to previous embolization. Three women delivered vaginally and four by cesarean section. Neither myoma recurrence nor abnormality in uterine function was observed. CONCLUSION(s): The results of this first series of 12 pregnancies after myoma embolization are promising. If these preliminary results are confirmed, embolization could be a major breakthrough in the management of myoma and could replace conventional medical and surgical treatments. ( info) |
4/32. One-step hysteroscopic removal of sinking submucous myoma in two infertile patients. OBJECTIVE: To report one-step resectoscopic removal of submucous myomas that were pushed back into the muscular layer by increased intrauterine pressure during hysteroscopic procedures. DESIGN: Case report. SETTING: Kawasaki Municipal Hospital, Kawasaki, japan. PATIENT(S): Two infertile women presenting with menorrhagia in whom submucous myoma with a broad base was diagnosed. INTERVENTION(S): One patient was pretreated with GnRH agonist for 4 months; the other patient did not receive this treatment. Resectoscopic myomectomies were performed under close sonographic monitoring. MAIN OUTCOME MEASURE(S): Clinical symptoms and conception status. RESULT(S): Tumor sinking occurred during the hysteroscopic procedures, but complete resectoscopic removal of the submucous myomas was achieved under sonographic and hysteroscopic visualization. One patient experienced hyponatremia but recovered after conservative treatment. Both patients conceived after myoma removal. CONCLUSION(S): Sinking myomas, which may cause infertility, can be removed with a one-step hysteroscopic procedure. Sinking of submucous myomas during hysteroscopy might be caused by pretreatment with GnRH agonist and by increased intrauterine pressure during hysteroscopy. We recommend that intrauterine pressure be <45 mmHg, equivalent to hanging a bag of fluid under gravity control 70 cm above the patient's uterus, at the beginning of operations for sinking myomas. ( info) |
5/32. sepsis in second trimester of pregnancy due to an infected myoma. A case report and a review of the literature. Considering the high incidence of uterine myomata in women in reproductive age, myomata are only found in 2% of all pregnancies. Although they frequently lead to complications in pregnancy, cases of pyomyomata during pregnancy are rarely reported. A 44-year-old gravida 1 in her 26th week of gestation was admitted to the hospital for septic temperatures of unknown cause. A 12-cm leiomyoma with solid structures of heterogenic sonographic pattern and cystic spaces had been documented on a prior first trimester sonogram. The myoma now appeared with the same size but an increased echogenicity of the liquid parts. Ultrasound guided aspiration of the fluid within the myoma showed an infection with klebsiella pneumoniae. A cesarian section with myo- mectomy confirmed the diagnosis of a pyomyoma. ( info) |
6/32. Acute uterine inversion due to a growing submucous myoma in an elderly woman: case report. A case of acute non-puerperal uterine inversion due to a growing submucous myoma in elderly woman is presented. This is rare and the diagnosis is often difficult. ( info) |
7/32. Perivascular myoma: case report with immunohistochemical and ultrastructural studies. A subcutaneous myopericytoma-type perivascular myoma arising in the elbow of a 61-year-old woman is described. The tumor was well demarcated and consisted microscopically of small ovoid and spindle cells arranged in a concentric fashion, surrounding small to medium-sized vessels and imparting a superficial resemblance to hemangiopericytoma. In some areas, the cellular whorls were separated by myxoid stroma. cells located between cellular whorls appeared immature with scant cytoplasm but did not show distinct nuclear anaplasia, increased mitoses or foci of necrosis. immunohistochemistry showed that constituent cells were positive for alpha-smooth muscle actin and desmin. Electron microscopy disclosed that the immature-looking cells, as well as the ovoid and spindle-shaped cells, possessed focal densities along with thin filaments, subplasmalemmal densities, pinocytotic vesicles and an external lamina. These ultrastructural and immunohistochemical features indicate a myoid nature of pericytic cells and justify this type of neoplasm being categorized as perivascular myoid tumor. ( info) |
8/32. The lungs in lymphangiomyomatosis and in tuberous sclerosis. Two cases of pulmonary lymphangiomyomatosis (PL) are described and 33 other cases from the literature are reviewed. These are compared with one case of tuberous sclerosis with pulmonary involvement (PTS) and 32 other cases from the literature. There are no differences in lung function between these two conditions, both of which show airways obstruction associated with diffuse radiological lung changes. There are, however, both clinical and radiological differences and also differences in the distribution of the lesions and the histological location of the excessive smooth muscle; these indicate that PL and PTS are probably different entities and not polar forms of one condition. Finally, the strictly female incidence of PL suggests a sex-linked disorder, and it is postulated that this may be related to congenital pulmonary lymphangiectases. ( info) |
9/32. Carney's syndrome: complex myxomas. Report of four cases and review of the literature. Cardiac myxomas are rare tumors. They usually appear as a sporadic isolated condition in the left atrium of middle-aged women with no other coincidental pathology. Carney and others have described in young people a special complex group of cardiac myxomas associated to a distinctive complex pathology, giving identity to the "syndrome myxoma" or "Carney's syndrome". Four additional cases of this syndrome, treated from 1977 to 1999 at the Hospital Clinico de la Universidad de chile are presented here with a comprehensive review of the literature, accumulating 100 cases. The main features of our cases include the presence of malignant non cardiac tumors, a familial trend, follow-up of 23 years and an iterative recurrence in the elder case. To date all patients are tumor free. Reviewing the literature, patients with Carney's syndrome were younger, with a mean age of 26 years and female predominance (62%). Cardiac myxomas affected the four chambers of the heart: 64% the left atrium; 44% the right atrium; 14% the left ventricle and 12% the right ventricle. They were multiple tumors in 41% and involved more than one chamber in 31%, being synchronous or metachronous. There was a marked familial trend (52%), a high incidence of recurrence (20%), with more than one occurring in half the cases. Extra-cardiac involvement consisted of: 68% pigmented skin lesions, 40% cutaneous myxomas, 37% adrenal cortical disease, 27% myxoid mammary fibroadenoma and 34% male patients with testes tumors. A low percentage had pituitary adenoma, melanotic schwannomas and thyroid disease. The diagnosis is made when two or more of these criteria are present. In agreement with these findings the four chambers of the heart should be examined at surgery for atypical myxoma locations, right atriotomy and combined superior-transseptal approach improve exposure of the cavities, careful screening of the first degree family members should be conducted, and closed short and long term follow up controls are important. Complex myxoma appears as a multi-systemic disorder, occasionally having an ominous prognosis and malignant potentiality, and is still undergoing investigation for better understanding and identification. ( info) |
10/32. Intravascular myopericytoma. BACKGROUND: Myopericytoma is a benign tumor composed of cells that show apparent differentiation towards putative perivascular myoid cells called myopericytes. It arises most commonly in the dermis or subcutaneous tissue of the extremities in adults. methods: We describe a myopericytoma that was unusual in its intravascular location. RESULTS: A 54-year-old man presented with a 10-year history of a painful slowly growing 1.5-cm nodule in the subcutaneous tissue of the thigh. Histologic examination of the excised lesion showed that is was entirely contained within the lumen of a vein. It was composed of a proliferation of myoid-appearing spindle cells, which were arranged in a striking concentric pattern around numerous blood vessels, in a manner that accentuated the vessel walls. This pattern is characteristic of myopericytoma. In some areas, fascicles of spindle cells, embedded in a myxoid stroma, bulged into the lumina of lesional vessels, reminiscent of myofibroma/myofibromatosis. Lesional spindle cells were diffusely positive for smooth muscle actin, focally positive for CD34 and were negative for desmin, cytokeratin, S100 protein, HMB-45 and CD31. CONCLUSION: This case illustrates that myopericytoma can be entirely intravascular in its location. ( info) |