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Mediastinal tumours
Published in Anju Sahdev, Sarah J. Vinnicombe, Husband & Reznek's Imaging in Oncology, 2020
Lymphangiomas are rare congenital abnormalities of the lymphatic vessels. Typically, they are isolated solitary masses, but they can be more widespread or associated with chromosomal abnormalities (152). These lesions are benign in nature and are found in the cervical region 75% of the time. In 10% of cases, the cysts extend into the mediastinum and are associated with chylothorax and haemangiomas (152). Although these tumours are commonly identified in children before the age of 2 years, when the mass is isolated to the mediastinum, it is often not identified until it has gotten large enough to cause compressive symptoms (153). Such symptoms include chest pain, cough, and dyspnoea. Radiographically, these lesions appear cystic and can be confused with pericardial cysts, although lymphangiomas are more likely to have a lobulated appearance (153). The use of lymphangiographic contrast media combined with CT scanning can also differentiate these lesions (152). Total resection is optimal; however, in cases complicated by chylothorax, there is some evidence suggesting that additional radiotherapy may be of some benefit (154). Lymphangiomatosis seen in young women is typically a more progressive form of disease in which multiple tumours are found and invade multiple organ structures, including the lung, heart, and bone (155).
How much different should the management of lymphangioma among the pediatric/neonatal age group be?
Published in Byung-Boong Lee, Peter Gloviczki, Francine Blei, Jovan N. Markovic, Vascular Malformations, 2019
Cristobal M. Papendieck, Miguel Amore
Lymphangioma is a benign cystic vascular lymphatic malformation that can present as a single or multiple, micro or macro, uni- or multilocular, simple or mixed (hemo-lymphangioma) lesion. If it is multiple, it can be called lymphangiomatosis (Figure 73.1).
Rare forms of interstitial lung disease
Published in Muhunthan Thillai, David R Moller, Keith C Meyer, Clinical Handbook of Interstitial Lung Disease, 2017
Management of diffuse lymphatic diseases in the lung can be difficult due to absence of established treatment of proven efficacy. The clinical course is characterized by slow progression for patients with both disorders (120,123–125). For diffuse pulmonary lymphangiomatosis, sirolimus (130–132), bevacizumab (133), propranolol (134), imatinib (135) and interferon-α (125,136) therapy have been reported to benefit some affected patients. Lung transplantation has been performed for few patients with diffuse pulmonary lymphangiomatosis (137).
A patient with macrodystrophia lipomatosa bilaterally affecting the entire upper extremity: reporting of a rare case and literature review
Published in Case Reports in Plastic Surgery and Hand Surgery, 2021
Kyoko Baba, Shinya Kashiwagi, Mitsuru Nemoto, Akira Takeda, Keizo Fukumoto, Eiju Uchinuma
The differential diagnosis of MDL includes fibrolipomatous hamartoma, lymphangiomatosis, hemangiomatosis, Klippel-Trenaunay-Weber syndrome, and Proteus syndrome [16–20]. Among them, the concurrence of MDL and fibrolipomatous hamartoma in the upper extremities has frequently been reported [21–23]; however, the association thereof remains unclear [6]. Other concurrent disorders include clinodactyly, syndactyly, polydactyly, and symphalangism [4,24]. The some authors consider that MDL as a localized form of Proteus syndrome in reports before 2000 [25–27]. Furthermore, there are some confusion of macrodystrophia lipomatosa and fibrolipoma of nerve [9,28]. Although, the most authors believe that the gross changes have as histopathological substrate the proliferation of mesenchymal elements with an excess of adipose/fibro-adipose tissue in the dermis in MDL [28].
Splenic Hemangiomatosis: An Uncommon Vascular Lesion in an Infant
Published in Fetal and Pediatric Pathology, 2018
Gargi Kapatia, Suvradeep Mitra, Kirti Gupta, Prema Menon, K. L. N. Rao
Characteristic features on histology include endothelial-lined vascular channels many of which were cystically dilated. Littoral cell angiomatosis is an important differential to be considered [5]. However, negativity for CD68 as noted in the index case argues against this possibility. Lymphangioma and lymphangiomatosis are other causes which can result in splenomegaly. The endothelium-lined spaces herein showed negativity for D2-40 while were positive for CD31 and CD34 [6]. Notably, CD31 and CD34 can highlight both vascular and lymphatic endothelium. Nevertheless, negativity for D2-40 (a lymphatic endothelium marker) ruled out a possibility of lymphangiomatosis in the index case.
Chronic interstitial lung diseases in children: diagnosis approaches
Published in Expert Review of Respiratory Medicine, 2018
Nadia Nathan, Laura Berdah, Keren Borensztajn, Annick Clement
Lymphatic disorders can be classified as primary or secondary. Congenital errors of lymphatic development can lead to primary pulmonary lymphatic disorders that include lymphangiomas and lymphangiomatosis, lymphangiectasis, and lymphatic dysplasia syndrome (congenital, yellow nail syndrome). Secondary forms of lung lymphatic disorders result from a variety of processes such as chronic airway inflammation that impair lymph drainage and increase lymph production [52].