Diffuse Thoracic Lymphangiomatosis: Diagnosis and Treatment

Casero, Estrella; Poce, Roberto Mongil; Sánchez, Rosario; Avecilla, A. Fernández de Rota; Domenech, Adolfo; Bermúdez, J.L. Fernández

doi:10.1016/s1579-2129(06)60381-9

Cited by 9 publications

(5 citation statements)

References 5 publications

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“…Non-surgical measures include medium chain triglyceride diet, 3 alphainterferon, 4 thalidomide 5 and radiotherapy. 6,7 In patients with large symptomatic chylothorax, repeated pleurocentesis, thoracoscopic thoracic duct ligation and pleurodesis have been reported. 3 Other measures include pleurodectomy, 3 pulmonary lymphangiectomy 8 and more recently, bilateral lung transplant.…”

Section: Lymphangioleiomyomatosis (Lam) -Diffuse Lymphatic Proliferatmentioning

confidence: 99%

A Pleural Effusion with an Unusual Cause

Tan

Ong

Takano

2011

Proceedings of Singapore Healthcare

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Background and Aims: Peritoneal dialysis (PD) is a rare cause of pleural effusion. It appears as a complication of PD in approximately 2% patients. Hydrothorax in this situation is called "sweet hydrothorax" as hypertonic glucose solution fills the pleura. Methods: 62-year-old woman with end stage renal disease presented with dyspnea on exertion and chest pain for about 4 weeks after initiation of PD six months back. A chest x-ray was done showed massive unilateral right-sided pleural effusion. She underwent small bore chest drainage for massive pleural effusion.Pleural fluid was crystal clear with high glucose concentration of 735 mg/dl.The pleural fluid protein was 0.2g/dl .Cytological and microbiological examination of the pleural fluid showed no abnormalities .Pleural fluid analysis was consistent with transudate and pleural fluid glucose concentration was higher than patient serum glucose concentration suggestive of sweet hydrothorax.She was switched over to haemodialysis for 4 weeks. After 4 weeks she was started back on peritoneal dialysis with reduced fluid volume and reduced dwell time and increased frequency of fluid change without recurrence for six months under followup. Results: Hydrothorax may develop several weeks or months after starting of PD. Diagnostic pleural tapping and pleural-fluid analysis are often diagnostic, revealing a crystal clear pleural fluid with a low protein and a high glucose concentration. In the patient presented here, glucose concentration in the pleural fluid was much higher than that in the serum drawn concomitantly. Conclusions: It is advisable to keep sweet hydrothorax as a differential diagnosis while evaluating hydrothorax in patients on peritoneal dialysis.

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Section: Lymphangioleiomyomatosis (Lam) -Diffuse Lymphatic Proliferatmentioning

confidence: 99%

A Pleural Effusion with an Unusual Cause

Tan

Ong

Takano

2011

Proceedings of Singapore Healthcare

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“…In contrast, lymphangiomatosis shows diffuse proliferation of lymphatic tissues and is frequently associated with other lymphaticrelated abnormalities, such as lymphangiectasis and lymphangioleiomyomatosis [1,3,5,6,9]. The present case is quite unusual in its late onset age and the lack of other lymphatic-related abnormalities.…”

Section: Discussionmentioning

confidence: 60%

“…Lymphangiomatosis, a diffuse, proliferative form of lymphangioma, is typically benign, but association with serious morbidity is not uncommon [1,3,5,6,9]. Involvement of serosal surfaces and viscera by lymphangiomatosis may result in severe complications such as dyspnea due to compression by the mediastinal or pulmonary mass and pleural or pericardial effusion [1,3,9].…”

Section: Introductionmentioning

confidence: 99%

Mediastinal lymphangiomatosis coexisting with occult thymic carcinoma

et al. 2006

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Mediastinal lymphangiomatosis in a 70-year-old woman was diagnosed on a medical checkup. The tumor was resistant to sclerotherapy with OK432 or bleomycin. The patient continued on a downhill course and died approximately 3 years after the initial diagnosis. Autopsy revealed a large tumor mass occupying the anterior mediastinum and firmly adhered to the pericardium and the pleura. The tumor consisted of two intermingled lesions: dilated vessels lined with D2-40-positive lymphatic endothelium and CD5-positive atypical cell nests with focal keratinization. The former was diagnosed as lymphangiomatosis and the latter as thymic squamous cell carcinoma. Vascular endothelial growth factor (VEGF)-C, a growth factor for lymphatic endothelial cells, was expressed by the carcinoma, and VEGF-C receptor was expressed by the endothelium of lymphangiomatosis. These findings suggested that VEGF-C derived from the thymic carcinoma induced the lymphangiomatosis lesion in a paracrine manner.

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“…Drug therapy included propranolol ( 12 ), glucocorticoids, and bevacizumab ( 13 , 14 ). Surgical treatment included thoracic duct ligation and heart and lung transplantation ( 15 ). There have been reports of no recurrence of the disease after 4 months to 3 years of follow-up following complete surgical resection of the pulmonary lymphangioma ( 16 , 17 , 18 ).…”

Section: Discussionmentioning

confidence: 99%

Diagnosis and treatment of diffuse pulmonary lymphangioma in children: A case report

Huang

et al. 2023

Exp Ther Med

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The present study aimed to investigate the clinical characteristics of diffuse pulmonary lymphangioma (DPL) in children to improve the diagnosis and treatment of this disease. A case of pediatric DPL was observed for its clinical symptoms, imaging features, lung biopsy pathological characteristics and immunohistochemical phenotypes, and relevant literature was also reviewed. The main clinical manifestations of this pediatric patient were a cough, shortness of breath, hemoptysis, bloody chylothorax and pericardial effusion. Chest computed tomography showed a grid-like shadow and markedly thickened interlobular septa. Pathological examination revealed lymphatic vessel hyperplasia and expansion. Immunohistochemistry showed positive staining of lymphatic endothelial cells CD31 and D2-40. The patient's condition improved after combined treatment with methylprednisone, propranolol, sirolimus and somatostatin, whose bloody chylothorax also achieved good therapeutic effect after conservative treatment. Overall, the clinical and imaging appearances of DPL are lack of characterization, and its clinical manifestations include cough, shortness of breath and chylothorax. Computed tomography may show mesh-like shadows of both lungs and thickened interlobular septa. The definite diagnosis of DPL depends on biopsy pathology. In addition to this case, B-ultrasound-guided puncture biopsy is effective and safe, and propranolol-sirolimus treatment has a certain effect, but the clinical effect may be different. Conservative treatment of pleural effusion can result in better curative effect.

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Diffuse Thoracic Lymphangiomatosis: Diagnosis and Treatment

Cited by 9 publications

References 5 publications

A Pleural Effusion with an Unusual Cause

A Pleural Effusion with an Unusual Cause

Mediastinal lymphangiomatosis coexisting with occult thymic carcinoma

Diagnosis and treatment of diffuse pulmonary lymphangioma in children: A case report

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