Lymphangiomatosis: a Wide Spectrum of Disease

GC Gan, ML Wastie

Hong Kong J Radiol 2010;13:222-5

Lymphangiomatosis is a systemic disorder that can affect any organ system that contains lymphatics, including bones and soft tissues, resulting in a wide range of clinical and radiological manifestations. This report highlights lymphangiomatosis in a 29-year-old woman with both soft tissue and skeletal involvement. She gave a history of limb fractures but presented with left hypochondrial pain and imaging revealed multicystic lesions in the spleen. She had a splenectomy and its histology revealed lymphangiomatosis. Computed tomography scan revealed lucent lesions in her bones. As the presence of lucent bony lesions and visceral involvement can mimic a malignancy with bony metastases, lymphangiomatosis needs to be considered in the differential diagnosis, especially owing to the presence of lucent bone lesions with visceral involvement and chylous ascites.

中文摘要

淋巴管瘤病：多種不同表現的疾病

GC Gan、ML Wastie

淋巴管瘤病是一種系統性疾病，可發生在身體有淋巴管的任何器官，包括骨和軟組織，形成有多種不同臨床及影像表現。本文報告一名29歲淋巴管瘤病女性患者，病灶涉及其骨和軟組織。患者曾有骨折，病發時出現左季肋部疼痛，放射影像顯示其脾臟出現多囊性病變。患者接受脾切除，組織學檢查顯示淋巴管瘤病。電腦斷層攝影可見其病灶的骨透亮像。由於骨透亮病灶和涉及內臟的情況與有骨轉移的惡性腫瘤很相似，必須把淋巴管瘤病納入鑒別診斷中，尤其是當病灶出現骨透亮像，並涉及內臟和乳糜性腹水。