Thoracic Lymphangiomatosis: Report of 3 Patients With Different Presentations

Lymphangiomatosis originates from congenital multiple malformations of the lymphatic vessel system. It is a benign lesion with malignant behavior. No radical treatment is indicated for this disease. The first patient presented initially with multiple bone cysts suggestive of multiple myeloma, the second with pleural effusion, and the third with mild shortness of breath. The lesions in these 3 patients were mainly located in the upper mediastinum, the parietal pleura, and the lung, respectively. Without any further treatment, their symptoms and radiographic images showed no progression of these conditions after a follow-up time of 7 to 42 months. Lymphangiomatosis originates from congenital multiple malformations of the lymphatic vessel system. It is a benign lesion with malignant behavior. No radical treatment is indicated for this disease. The first patient presented initially with multiple bone cysts suggestive of multiple myeloma, the second with pleural effusion, and the third with mild shortness of breath. The lesions in these 3 patients were mainly located in the upper mediastinum, the parietal pleura, and the lung, respectively. Without any further treatment, their symptoms and radiographic images showed no progression of these conditions after a follow-up time of 7 to 42 months. Lymphangioma is a rare disease originating from congenital developmental malformations of the lymphatic vessel system. “Lymphangiomatosis” describes the presence of multiple or multiorgan lymphangiomas. In 75% of cases, multiple organs with a predilection for the thoracic and neck regions are involved, and as many as 75% of patients have bone involvement [1Faul J.L. Berry G.J. Colby T.V. et al.Thoracic lymphangiomas, lymphangiectasis, lymphangiomatosis, and lymphatic dysplasia syndrome.Am J Respir Crit Care Med. 2000; 161: 1037-1046Crossref PubMed Scopus (321) Google Scholar]. We present 3 cases of thoracic lymphangiomatosis with quite different symptoms, clinical courses, and diagnostic processes. A 34-year-old man was admitted for asymptomatic mediastinal mass on computed tomography (CT) of the chest. A CT scan of the skull 1 month earlier had incidentally found multiple cystic bone destruction, clinically suggestive of multiple myeloma or metastatic carcinoma. The patient underwent CT of the thorax, which revealed multiple low-density shadows in the mediastinum (Fig 1). Ultrasonography of the abdomen showed multiple small cysts in the spleen. Positron emission tomography/CT showed diffuse porous bony defects involving the skull, humerus, ilium, pubis, femoral head, sternum, and almost all vertebrae and ribs. Skull biopsy before admission led to a diagnosis of hemangioma. Thoracoscopic exploration found a spongelike soft tissue tumor extending around the surface of the thymus, superior vena cava, trachea, and cupula of pleura. On biopsy, turbid chylous fluid was seen as a continuous discharge from the cut surface of the tumor (Fig 2), which was controlled by suture and cauterization. The patient's postoperative recovery was uneventful. Pathologic examination confirmed the diagnosis as lymphangiomatosis. Pathologic review of the skull biopsy sample corrected the former diagnosis to lymphangiomatosis.Fig 2Intraoperative video clips. (A) Soft tissue tumor around the upper mediastinum and the parietal pleura (arrows) in patient 1. (B) Chylous fluid discharged from the cut surface after biopsy (arrows) in patient 1. (C) Massive bloody chylous hydrothorax and thickened pleura with multiple scattered nodes in patient 2.View Large Image Figure ViewerDownload (PPT) A 21-year-old girl had experienced idiopathic massive left pleural effusion for 1 month. She underwent thoracocentesis several times, and a total of 5,000 mL of bloody pleural fluid was drained. A CT scan showed pleural effusion and a low-density soft tissue shadow in the posterior mediastinum (Fig 1). The spleen had multiple large cysts, the largest being 6 cm in diameter. Thoracoscopic exploration showed that the visceral and parietal pleura were thickened and scattered with multiple small nodules, which were 0.5 to 1.0 cm, soft, and yellowish (Fig 2). After pleural biopsy, the pleural cavity was soaked with interleukin-2 solution for 15 minutes, and fibrin glue was applied over the biopsy site. One month later, the patient underwent splenectomy. Pathologic examination of both the spleen and the thorax lesions showed lymphangiomatosis. The patient was followed up bimonthly for 15 months, and roentgenograms showed no relapse of the pleural effusion. A 53-year-old woman described having mild chest tightness and shortness of breath for 10 years. She had no cough, no sputum, no fever, and no chest pain. Chest CT revealed diffuse thickening of the interlobular septa and bronchovascular bundle in bilateral lungs and enlargement of the mediastinal lymph nodes (Fig 1). The results of bronchoscopy and cytologic examination of lavage fluid were unremarkable. Sarcoidosis was initially suspected. Cervical mediastinoscopy showed dense scarring tissue surrounding the trachea and carina, closely adherent to the superior vena cava. Biopsy of lymph nodes failed because it was hard to get enough tissue. Subsequently, thoracoscopy was carried out. Abnormal fibrous tissue was found on the surface of the pericardium, the great vessels, and around the left hilum. Biopsy showed lymphadenoid tissue. Biopsy of the left lower lobe by wedge resection was also performed, and the diagnosis of diffuse pulmonary lymphangiomatosis was confirmed. The patient's recovery was uneventful. She received no further treatment, but none of her symptoms deteriorated after a follow-up period of 42 months. Recent repeated CT of the chest showed no significant progress of the thoracic lesion. Thoracic lymphangiomatosis is usually part of a systemic disease, with involvement of multiple organs. All compartments of the mediastinum, the pleura, the lung, or a combination of these organs may be affected. We encountered 3 patients with multiorgan lymphangiomatosis and reviewed their radiographic findings and the intraoperative video clips. These 3 patients had thoracic involvement but presented with quite different characteristics. The first patient presented with asymptomatic multiple bone cysts, suggestive of multiple myeloma; the second patient with massive unilateral pleural effusion; and the third with mild shortness of breath for 10 years. Their thoracic lesions were mainly located in the upper mediastinum, the parietal pleura, and the lung, respectively. Although multiple organs were involved, the primary lesions were rather asymptomatic. The clinical manifestations resulted from secondary hydrothorax in patient 2 and from secondary pulmonary interstitial disease in patient 3. Pleural or pericardial effusion is a common complication of thoracic lymphangiomatosis. The pathophysiologic changes causing the effusion have not been elucidated [2Zisis C. Rontogianni D. Charalambous E. Bellenis I. Lymphangiomatous hamartoma: cause or bystander of the isolated chylopericardium?.J Thorac Cardiovasc Surg. 2005; 130: 1201-1202Abstract Full Text Full Text PDF PubMed Scopus (6) Google Scholar]. Zisis and colleagues [3Zisis C. Spiliotopoulos K. Patronis M. et al.Diffuse lymphangiomatosis: are there any clinical or therapeutic standards?.J Thorac Cardiovasc Surg. 2007; 133: 1664-1665Abstract Full Text Full Text PDF Scopus (9) Google Scholar] deemed that the onset of this complication does not seem to depend on the pathologic “burden” of the disease, the extent of involved tissue and organs, or the age of the patient. In our first case, during biopsy, there was a continuous flow of milky colored fluid from the cut surface. This interesting phenomenon was also observed by Shahriari and Odell [4Shahriari A. Odell J.A. Cervical and thoracic components of multiorgan lymphangiomatosis managed surgically.Ann Thorac Surg. 2001; 71: 694-696Abstract Full Text Full Text PDF Scopus (13) Google Scholar]. Given that the cause of lymphangiomatosis is deformation of lymph vessels, we postulate that the thoracic effusion in such patients is due to spontaneous rupture of abnormal lymph vessels in the lesion, similar to the iatrogenic damage in the operation. It is more like bleeding than exuding. This presumption is partly verified in our second patient, whose hydrothorax vanished after thoracoscopic exploration and application of fibrin glue, although the lesions were widely scattered all over the parietal pleura. Differential diagnosis is essential for patients suspected of having lymphangiomatosis because it may mimic other mediastinal or pulmonary lesions. In patients with hydrothorax, thoracentesis and analysis of the aspirated pleural effusion may arouse suspicion. Definite diagnosis should always be established histologically on biopsy samples. No drug or other therapeutics can prevent the development of this disease. Therapy should be aimed at decreasing the symptoms that arise from compressive effects or at the control of chylous effusions. Surgical treatment is indicated for complications or for biopsy [4Shahriari A. Odell J.A. Cervical and thoracic components of multiorgan lymphangiomatosis managed surgically.Ann Thorac Surg. 2001; 71: 694-696Abstract Full Text Full Text PDF Scopus (13) Google Scholar]. Bermejo Casero and associates [5Bermejo Casero E.J. Mongil Poce R. Arrabal Sánchez R. Fernández de Rota Avecilla A. Benítez Doménech A. Fernández Bermúdez J.L. Diffuse thoracic lymphangiomatosis: diagnosis and treatment.Arch Bronconeumol. 2004; 40: 599-601Crossref Google Scholar] reported 2 patients successfully treated with radiotherapy of 18 Gy in 12 sessions. The peribronchovascular pulmonary infiltrate disappeared, the mediastinal neoplasm became smaller, and the clinical symptoms diminished afterward. Patients with a macrocystic form of the disease may benefit from sclerosing treatment of infusion with streptococcus antigen OK-432 [6Banieghbal B. Davies M.R. Guidelines for the successful treatment of lymphangioma with OK-432.Eur J Pediatr Surg. 2003; 13: 103-107Crossref PubMed Scopus (69) Google Scholar]. End-stage pulmonary lymphangioma may induce respiratory failure that requires lung transplantation [7Kinnier C.V. Eu J.P. Davis R.D. Howell D.N. Sheets J. Palmer S.M. Successful bilateral lung transplantation for lymphangiomatosis.Am J Transplant. 2008; 8: 1946-1950Crossref Scopus (21) Google Scholar]. None of our 3 patients received any specific treatment postoperatively. Their symptoms and radiographic images indicated no progress of their condition in the following 7 to 42 months. This indicates that lymphangiomatosis is a rather benign lesion and progresses slowly, although it has malignant behavior.