Retroperitoneal lymphangioleiomyoma with lymph node involvement: A pathologic-radiologic correlation of a rare form of myomelanocytic tumor.

Lymphangioleiomyomatosis (LAM) is a rare and slowly progressive disorder that usually arises in the lung, affects exclusively women in their childbearing years, and typically presents with progressive dyspnea on exertion and pneumothorax. Infrequently, extra-pulmonary LAM can occur in the retroperitoneum, uterine wall, mediastinum and intraperitoneal lymph nodes. Histologically, LAM is characterized by a proliferation of perivascular epithelioid cells (PEC) that express markers for both melanocytes and smooth muscle cells. We report a case of a peripancreatic retroperitoneal mass that was incidentally discovered on magnetic resonance image (MRI) scan of a 38-year-old female. The morphologic findings and the immunohistochemical staining were consistent with a lymphangioleiomyoma. The radiologic and pathologic correlation along with differential diagnosis of this rare entity is discussed.

[Case of lymphangioleiomyomatosis (LAM) discovered during cesarean section under spinal anesthesia].

We experienced a case of scheduled cesarean section under spinal anesthesia in a patient with LAM which had been missed in spite of preoperative medical examination and consultation with specialists but discovered because of perioperative hypoxia A 35-year-old woman, Gravida 1 Para 0, with breech presentation was scheduled to undergo cesarean section under spinal anesthesia at 38 weeks of gestation. She had no history of asthma or abnormal findings at annual medical examination. She had suffered from dry cough and nocturnal dyspnea for 7 weeks and an inhaled bronchodilator was administered with diagnosis of inflammatory airway disease by her respiratory physicians. Spinal anesthesia was performed with bupivacaine 12.5 mg. At the beginning of anesthesia SPO2 was 97% in supine position, but it rapidly decreased to less than 90% and 3 l x min(-1) oxygen was supplied with a facial mask. The anesthetic level was thoracal 4 bilaterally and her breathing was stable. The circulatory state, Apgar score and other vital signs were within normal ranges. Postoperative chest X-ray showed bilateral numerous grained spots and computed tomography scans showed multiple thin-walled cysts. The characteristic history and the fluoroscopic data gave her clinical diagnosis of LAM.

Changes in lung function and chylous effusions in patients with lymphangioleiomyomatosis treated with sirolimus.

BACKGROUND: Lymphangioleiomyomatosis (LAM) is a disorder that affects women and is characterized by cystic lung destruction, chylous effusions, lymphangioleiomyomas, and angiomyolipomas. It is caused by proliferation of abnormal smooth muscle-like cells. Sirolimus is a mammalian target of rapamycin inhibitor that has been reported to decrease the size of neoplastic growths in animal models of tuberous sclerosis complex and to reduce the size of angiomyolipomas and stabilize lung function in humans. OBJECTIVE: To assess whether sirolimus therapy is associated with improvement in lung function and a decrease in the size of chylous effusions and lymphangioleiomyomas in patients with LAM. DESIGN: Observational study. SETTING: The National Institutes of Health Clinical Center. PATIENTS: 19 patients with rapidly progressing LAM or chylous effusions. INTERVENTION: Treatment with sirolimus. MEASUREMENTS: Lung function and the size of chylous effusions and lymphangioleiomyomas before and during sirolimus therapy. RESULTS: Over a mean of 2.5 years before beginning sirolimus therapy, the mean (±SE) FEV1 decreased by 2.8%±0.8% predicted and diffusing capacity of the lung for carbon monoxide (Dlco) decreased by 4.8%±0.9% predicted per year. In contrast, over a mean of 2.6 years of sirolimus therapy, the mean (±SE) FEV1 increased by 1.8%±0.5% predicted and Dlco increased by 0.8%±0.5% predicted per year (P<0.001). After beginning sirolimus therapy, 12 patients with chylous effusions and 11 patients with lymphangioleiomyomas experienced almost complete resolution of these conditions. In 2 of the 12 patients, sirolimus therapy enabled discontinuation of pleural fluid drainage. LIMITATIONS: This was an observational study. The resolution of effusions may have affected improvements in lung function. CONCLUSION: Sirolimus therapy is associated with improvement or stabilization of lung function and reduction in the size of chylous effusions and lymphangioleiomyomas in patients with LAM. PRIMARY FUNDING SOURCE: Intramural Research Program, National Heart, Lung, and Blood Institute, National Institutes of Health.

Pulmonary lymphangiomyomatosis.

Lymphangiomyomatosis is an interesting disease with distinctive clinical and histopathologic findings. We report herein two additional cases of lymphangiomyomatosis, including one with clinical improvement after therapy with progesterone. This case is of particular significance in view of the patient's negative sex steroid receptor analysis. These findings open new avenues for future considerations in the therapy of this unusual but interesting disease.

Pulmonary lymphangioleiomyomatosis associated with pulmonary parenchymal, hilar, and mediastinal noncaseating granulomas.

A young nonsmoking woman presented with severe dyspnea, exercise desaturation, and chest discomfort. Pathologic and histochemical findings revealed pulmonary lymphangioleiomyomatosis (LAM) as the primary abnormality. In addition, there were multiple noncaseating granulomas with special stains and cultures negative for organisms. This highly unusual combination of pathologic findings might suggest the presence of coexistent sarcoidosis in our patient with LAM.

Pulmonary lymphangiomyomatosis causing bilateral pneumothorax during pregnancy.

A 32-year-old woman, 12 weeks pregnant, presented with bilateral spontaneous pneumothorax that did not heal with tube thoracostomy. At right and left thoracotomy, lymphangiomyomatosis of the lung was found and treated by pleurodesis. Lymphangiomyomatosis has been infrequently reported in the surgical literature, even though it may befall the thoracic surgeon to establish the diagnosis and aid in treatment.

Lymphangiomyomatosis: a respiratory illness with an endocrinologic therapy.

Lymphangiomyomatosis should be considered when unexplained dyspnea, pneumothorax, pleural effusion or infiltrative radiographic changes occurs in a woman of childbearing age. Radiographic and pulmonary function peculiarities help in the diagnosis and evaluation of results of treatment. Diagnosis is dependent on lung biopsy and preferably identification of hormonal receptors. Endocrinological manipulation, in this study with medroxyprogesterone, decreased disability, morbidity and progression of this otherwise fatal illness. Early diagnosis and institution of treatment would appear to provide the best result.

Diagnostic approach to pulmonary lymphangioleiomyomatosis. Case report.

A diagnosis of lymphangioleiomyomatosis is to be considered in women with pleural chylous effusion when plain chest films show diffuse interstitial changes, possibly associated with cystic-like spaces and overinflation. The CT, showing the presence of a retroperitoneal mass, can ultimately help to outline the diagnosis of this syndrome as in the personal case observed in a postmenopausal woman. A presumptive pre-surgical biopsy diagnosis can be useful for studies of hormone-receptors and may be clinically relevant.

[High resolution CT of interstitial lung diseases]. / High-Resolution-CT bei interstitiellen Lungenerkrankungen.

High-resolution CT images were assessed in 39 patients with confirmed interstitial lung disease. 22 patients with fibrosing alveolitis, 12 patients with lymphangitic spread of tumour and 5 patients with lymphangioleiomyomatosis of the lung were included. In fibrosing alveolitis the dominant features on HR-CT were honeycomb cysts with concomitant fibrosis in a predominantly peripheral subpleural distribution. In contrast to this the patients with LAM showed cystic lesions without any predominance--throughout the lung fields and without any fibrotic changes. In lymphangitic spread of tumour, polygonal lines and septal lines as well as nodules were found along the bronchovascular bundles. The patterns seen on HR-CT of the thorax correlate extremely well with the underlying pathology and make it possible to differentiate between the three disease entities by means of characteristic features.

[Pulmonary involvement in lymphangioleiomyomatosis: studies using high-resolution computed tomography]. / Pulmonale Beteiligung bei Lymphangioleiomyomatose: Untersuchungen mit Hilfe der hochauflösenden Computertomographie.

Three female patients with histories of lymphangiomyomatosis are reported. Each was examined by means of high resolution computerized tomography. All three cases showed homogeneous distribution of cystic lesions in the pulmonary parenchyma. This pattern may be regarded as typical and may even be pathognomonic. In two patients lymphomas of the retroperitoneal space and renal masses were found.

[High-resolution computed tomography of the thorax in lymphangioleiomyomatosis and tuberous sclerosis]. / High-resolution-Computertomographie des Thorax bei Lymphangioleiomyomatose und tuberöser Sklerose.

We present three cases that illustrate the HRCT appearance of pulmonary lymphangioleiomyomatosis and pulmonary involvement in tuberous sclerosis. All patients had multiple cystic lesions diffusely distributed throughout the lungs. The lack of fibrotic changes was a prominent feature in all patients. HRCT correlated more closely than conventional CT with the pathologic findings.

Lymphoedema of the legs as a result of lymphangiomyomatosis. A case report and review of the literature.

Leg lymphoedema is described as an unusual presenting feature of lymphangiomyomatosis. MRI scanning revealed typical lesions. Possible sex hormonal manipulation as a therapy for pulmonary or abdominal involvement is reviewed.

Successful treatment of pulmonary lymphangioleiomyomatosis with oophorectomy and medroxyprogesterone-acetate: report of a case and brief review of the literature.

This report describes the successful hormonal treatment of a young woman with pulmonary lymphangioleiomyomatosis. In addition a brief review of the literature is presented.

Two cases of hamartoangiomyomatosis with characteristic scintigraphic findings.

Hamartoangiomyomatosis is a rather rare pulmonary disease which occurs in young to middle-aged women. The chief complaint of this disease is dyspnea. The chest X-ray findings of this disease are such complicated features as reticular, reticulogranular, miliary and honeycomb-like shadow. A pulmonary perfusion scintigram was prepared with a scinticamera after intravenous injection of 10 mCi of Tc-99m MAA. The anterior image showed a remarkably reduced bilateral pulmonary blood flow, especially in the middle and lower areas of the lungs. In the upper portions, the blood flow remained relatively sufficient. The posterior and lateral images also gave similar findings. In two cases of hamartoangiomyomatosis, pulmonary perfusion scintigraphy was successful in identifying the characteristic findings of the disease.

Pulmonary lymphangiomyomatosis.

Two cases of pulmonary lymphangiomyomatosis are reported and findings of high resolution computed tomography (CT) are described. CT reveals that most lesions appearing reticular or emphysematous on radiographs are actually cysts, and accurately displays the extent and distribution of cystic change of the lung. On high resolution CT, individual cystic walls are much better displayed than on routine 10 mm section CT. Further, it is possible to detect even trivial pleural effusion and mediastinal lymph node swelling by CT.

Early onset of childhood pulmonary lymphangiomyomatosis.

The authors present the first case of lymphangiomyomatosis in a pediatric patient. Radiographic findings, biopsy, computerized tomography, and electron microscopy were useful in detailing the presentation of early onset of pulmonary lymphangiomyomatosis. The unusual presentation of this child includes a description of her disease prior to any pulmonary symptomatology and 4 years of follow-up.

[Sonographic imaging of lymphatic vessels compared to other methods]. / Apport de l'échographie à l'imagerie des vaisseaux lymphatiques par rapport aux autres méthodes.

This paper reviews for the first time the normal and abnormal appearances of lymphatic channels of the skin using ultrasound. After a review of anatomy and histology, the authors present the current imaging modalities available for lymph vessel imaging. The ultrasound examination is presented with a description of the author's technique as well as the technical requirements of the ultrasound unit (12 MHz linear probe with a resolution of 400 microns). They present the ultrasound appearance of normal lymphatic channels and their relationships to the dermis, hypodermis and lymph nodes, and at last the ultrasound appearance of abnormal lymphatic pathways

[The contribution of computerized tomography to the diagnosis of diffuse pulmonary lymphangiomyomatosis. Apropos of a case]. / Apport de la tomodensitométrie au diagnostic de lymphangiomyomatose pulmonaire diffuse. A propos d'un cas.

The authors report a case of lymphangiomatosis (LAM) proven histologically in a 49 year old female. This case benefitted from very detailed CT-scanning (IDM) with characteristic images obtained from the thorax and bilateral renal tumours suggestive of angiomyolipomas. This association seems to be quasi-pathognomic of the disease. The discovery at CT-scanning of cerebral calcification raises a possible connection of LAM with Bourneville's tuberous sclerosis. This CT-scanning study has several points of interest: the early diagnosis of pulmonary lesions which are under evaluated by standard radiography; a complete inventory of the possible localisations of the disease; it enables a possible diagnosis in very frail patients in whom a lung biopsy would be a risk; an ability to assess further progress as there also exists a good correlation between the extent of the disease assessed by TDM and the disturbance in pulmonary function.

[Lymphangiomyoma of the thoracic duct]. / A ductus thoracicus lymphangiomyomája.

From the right superior mediastinum of a 31-year old woman the authors extirpated a lymphangiomyoma originating from the thoracic duct. The clinical feature and pathology of this rare lesions are discussed. This case is the first successfully removed thoracic duct lymphangiomyoma in Hungary.

[A new diagnostic approach in lymphangiomatosis. Apropos of a case]. / Nouvelle approche diagnostique de la lymphangiomyomatose. A propos d'une observation.

Lymphangiomyomatosis is a rare disease affecting exclusively women usually in child-bearing age. It is characterized by proliferation of an atypical smooth muscle involving the pulmonary, mediastinal and retroperitoneal lymph system. The case reported here concerns a 53-year old woman presenting with an interstitial pulmonary lesion giving a characteristic image at CT. The patient also had recurrent chylothorax and a renal angiomyolipoma explored by CT and magnetic resonance imaging. The diagnosis of lymphangiomyomatosis was made on a cluster of arguments, without having recourse to surgical lung biopsy.