1/116. Metastatic endometrial stromal sarcoma masquerading as pulmonary lymphangioleiomyomatosis. A 39 year old female presented with bilateral pneumothoraces and interstitial shadowing on chest x ray. A diagnosis of lymphangioleiomyomatosis was made following an open lung biopsy. Over the next eight years she developed respiratory failure leading to single lung transplantation but she died in the immediate postoperative period. Necropsy examination and review of the previous open lung biopsy revealed multiple pulmonary metastases from a low grade endometrial stromal sarcoma of the uterus. This case high-lights the importance of an accurate diagnosis before transplantation. ( info) |
2/116. Pulmonary lymphangioleiomyomatosis. A study of 69 patients. Groupe d'Etudes et de Recherche sur les Maladies "Orphelines" Pulmonaires (GERM"O"P). Pulmonary lymphangioleiomyomatosis (LAM) is a rare disorder of unknown cause characterized by peribronchial, perivascular, and perilymphatic proliferation of abnormal smooth muscle cells leading to cystic lesions. The hypothesis of hormonal dependence and the effectiveness of hormonal therapy have not yet been demonstrated conclusively, and the prevalence of extrathoracic manifestations and the survival of patients with LAM are somewhat contradictory. A multicentric retrospective study was conducted in an attempt to describe better the initial features, the diagnostic procedures, the associated lesions, and, above all, the management and course of LAM in a large homogeneous series of 69 stringently selected patients, with a majority of cases diagnosed since 1990. The aim of the study, based on a review of the literature, also was to provide a comprehensive view of this uncommon disease. The clinical features were in keeping with previous studies, but we found that exertional dyspnea and pneumothorax were the most common features, and chylous involvement was less frequent. LAM was diagnosed after menopause in about 10% of cases. The onset of LAM occurred during pregnancy in 20% of cases, and a clear exacerbation of LAM was observed in 14% of cases during pregnancy. Pulmonary LAM was diagnosed on lung histopathology in 83% of cases, but renal angiomyolipoma, observed in 32% of our patients, may be a useful diagnostic criterion when associated with typical multiple cysts on chest CT scan or with chylous effusion. Chest CT scan was more informative than chest X-ray (normal in 9% of cases), and may be indicated in spontaneous pneumothorax or renal angiomyolipoma in women of childbearing age. About 40% of the patients had a normal initial spirometry, while an obstructive ventilatory defect (44%), a restrictive ventilatory defect (23%), was observed in other patients. Initial diffusing capacity for carbon monoxide was frequently decreased (82%). Hormonal therapy was administered in 57 patients, but a clear > or = 15% improvement of FEV1 was observed in only 4 evaluable patients, treated with tamoxifen and progestogens (n = 2), progestogen (n = 1), and oophorectomy (n = 1). Probably 1 of the most urgent needs for clinical research in LAM is to test the currently available hormonal treatments in the context of international multicenter prospective controlled studies. pleurodesis was performed in 40 patients. lung transplantation was performed in 13 patients, 7.8 /- 5.2 years after onset of LAM, in whom the mean FEV1 was 0.57 /- 0.15 L. After a follow-up of 2.3 /- 2.2 years, 9 patients were alive. Mean follow-up from onset of disease to either death or closing date was 8.2 /- 6.3 years. overall survival was better than usually reported in LAM, and Kaplan-Meier plot showed survival probabilities of 91% after 5 years, 79% after 10 years, and 71% after 15 years of disease duration. ( info) |
3/116. Regional proliferation of HMB-45-positive clear cells of the lung with lymphangioleiomyomatosislike distribution, replacing the lobes with multiple cysts and a nodule. The authors report a case of a localized lesion of the lung presenting as multiple cysts and as a tumor in the right upper and middle lobes, consisting of a diffuse proliferation of clear cells with intralysosomal glycogen granules and human melanin black (HMB)-45 immunoreactivity. A 33-year-old woman complained of dyspnea because of the enlargement of bullae in the right upper and middle lung fields without stigmata of tuberous sclerosis. Resection showed multiple, various-size air-filled cysts and a tumor. The cysts in the resected lungs were reminiscent of lymphangioleiomyomatosis (LAM), accompanied by the diffuse proliferation of clear cells in the interstitium. The tumor, 1.8 cm in diameter, resembled a clear cell tumor of the lung (CCTL) and showed proliferation of clear cells with sinusoidlike vascular spaces. Both forms of proliferation were continuous spatially, and both constituent cells showed diffuse HMB-45 immunoreactivity. The cells that comprised a nodule revealed ultrastructurally abundant cytoplasmic glycogen, which was in the form of free and membrane-bound glycogen granules. This case may represent a particular pulmonary lesion consisting of CCTL-LAM hybrid cells, which share the cytologic features with CCTL cells on one hand, and the proliferative pattern and potential with LAM cells on the other. ( info) |
4/116. A breathless lady with lumpy kidneys. tuberous sclerosis complex (TSC) is now known to be associated with pulmonary lymphangiomyomatosis (PLAM). patients with either isolated PLAM or pulmonary involvement in TSC suffer from progressive respiratory failure and death within ten years of diagnosis. We report a case of TSC with PLAM, and a short review of recent literature regarding the conditions. ( info) |
5/116. Fine needle aspiration diagnosis of lymphangiomyomatosis. A case report. BACKGROUND: Lymphangiomyomatosis is a rare disease of females, usually of reproductive age. There is a proliferation of lymphatic smooth muscle in mediastinal, retroperitoneal and often pulmonary lymphatics and lymph nodes. CASE: A 45-year-old female presented with a right pleural effusion and increasing retroperitoneal adenopathy with palpable left inguinal adenopathy. Three months previously she had undergone a right salpingo-oophrectomy for an ovarian fibroma with concomitant left ovarian wedge biopsy, myomectomy for leiomyomas and partial omentectomy. Three years previously, at age 42, she had experienced two transient episodes of chylous pleural effusion with no sequelae. She underwent computed tomography-guided fine needle aspiration of a 4-cm inguinal lymph node to rule out lymphoma. CONCLUSION: Fine needle aspiration of lymphangiomyomatosis yields distinctive cytologic morphology. This characteristic morphology, in combination with the appropriate history, permits a minimally invasive, timely and in this particular case, entirely unexpected diagnosis. ( info) |
6/116. Thoracoscopic lung biopsy in a patient with pulmonary lymphangiomyomatosis. PURPOSE: We describe the anesthetic management of a patient with pulmonary lymphangiomyomatosis (LAM) during thoracoscopic lung biopsy (TSLB). CLINICAL FEATURES: LAM is a rare idiopathic disease characterised by progressive deterioration in respiratory function, occurring almost exclusively in women. In establishing the diagnosis, an open lung biopsy (OLB) has been employed in patients with relatively normal lung function. However, TSLB rather than OLB is less invasive. A 38 yr old woman developed a clinical course of cough, shortness of breath and sputum production, Chest X-ray findings, 99mTc-MAA scintigraphy and thin-sliced high resolution CT, typical of LAM, TSLB was scheduled to establish the diagnosis. General anesthesia, employing differential lung ventilation and high frequency jet ventilation combined with epidural anesthesia and continuous intravenous propofol was performed successfully. High frequency ventilation was applied to the non-dependent lung and intermittent positive pressure ventilation (IPPV) to the dependent lung with lower tidal volume and respiratory rate, allowing permissive hypercapnia. In the postoperative period, although synchronized intermittent mandatory ventilation was applied, pressure support ventilation or continuous positive airway pressure (CPAP) would have been a better selection. Postoperative sedation was performed satisfactorily using propofol. CONCLUSIONS: We recommend general anesthesia using differential lung ventilation combined with epidural anesthesia and intravenous propofol during TSLB for LAM. Postoperative ventilation should be pressure support ventilation or CPAP to keep peak inspiratory pressure low and allow permissive hypercapnia. ( info) |
7/116. Improvement of disseminated lymphangiomatosis with recombinant interferon therapy. Disseminated lymphangiomatosis is a rare disorder with a poor prognosis. We present a case involving a 3-year-old boy who presented with pulmonary infiltrates, multiple lytic lesions of the ribcage, and small cystic lesions in the spleen. Open-lung and bone biopsies revealed disseminated lymphangiomatosis. Significant clinical and radiologic improvement were observed and persisted after 28 months of treatment with recombinant interferon alpha-2b (IFN alpha-2b). No significant toxicity has been observed. ( info) |
8/116. Lymphangiomyomatosis: radiological and measured lung function deterioration after contrast-enhanced computed tomography. Pulmonary lymphangiomyomatosis (LAM) is a rare disease of unknown aetiology which occurs exclusively in women, usually of reproductive age. The findings on CT and high-resolution CT (HRCT) are well described and characteristic, and in a young woman they are virtually pathognomonic. A case of symptomatic, radiological and measured lung function deterioration following contrast-enhanced CT in a patient with LAM are reported here. These observations, to the authors' knowledge, have never been reported before. The authors attempt to explain these observations based on the known pathology of LAM. ( info) |
9/116. Pulmonary lymphangiomyomatosis (LAM) developing chylothorax. We describe a case of pulmonary lymphangiomyomatosis (LAM) with chylothorax that developed in a 46-year-old Japanese woman. This patient exhibited clinical symptoms of dyspnea and chest X-ray showed right pleural effusion. Thoracocentesis demonstrated chylous effusion. Chest computed tomography (CT) scan revealed multiple cystic lesions. Subsequent thoracoscopy revealed the chylorrhea from swelled vessels on the diaphragm. The clinical diagnosis, based on histological examinations with biopsy specimens obtained by thoracoscopy, was pulmonary LAM. Although the hormone therapy was not effective, chylous effusion was improved by the pleurodesis. Pulmonary LAM developing chylothorax is rare in japan. ( info) |
10/116. Pseudomembranous aspergillus bronchitis in a double-lung transplanted patient: unusual radiographic and CT features. Pseudomembranous aspergillus bronchitis is considered as an early form of invasive pulmonary aspergillosis, a well-known airway infection in immunocompromised patients. Radiologic features concerning invasive aspergillosis of the airways have been reported. However, we describe here an unusual feature of invasive aspergillus bronchitis, never reported to date, observed in a double-lung transplanted patient. Chest radiograph and CT revealed significant peribronchial thickening without any parenchymal involvement. ( info) |