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Weekly Chest CasesArchive of Old Cases

Case No : 1087 Date 2018-08-28

  • Courtesy of Hyungjin Kim, MD, Soon Ho Yoon, MD / Seoul National University Hospital
  • Age/Sex 48 / M
  • Chief Complaintscreening-detected lung abnormality, never-smoker
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5
  • Figure 6

Diagnosis With Brief Discussion

Diagnosis
Pulmonary lymphangioma
Radiologic Findings
Fig 1. Chest PA shows a cystic opacity in the left lung base.
Fig 2. Axial chest CT images (a, lung window; b, mediastinal window) showed a cystic mass with a thin wall and septa in the left lower lobe. A focal solid portion was noted in the dependent portion of the lesion.
Fig 3. There is no avid FDG uptake in the mass at PET/CT scan.
Fig 4. On the follow-up axial CT images (a, lung window; b, mediastinal window), which was taken four years later, the size of the cystic mass increased and inner air-fluid level was noted. While the patient denied any symptom, surgical resection was performed due to patient’s concerns about this growing lesion.
Fig 5. Surgical specimen exhibits multiple cystic structures filled with jellylike material. No solid mass or necrosis is observed. Multilocular cysts are lined with lymphatic endothelial cells.
Brief Review
Lymphangioma is a benign disease that can occur at any organs in the body. However, pulmonary lymphangioma is an extremely rare disease. Abnormal development and proliferation of lymphatic vessels that do not communicate with normal lymphovascular system lead to the development of pulmonary lymphangioma. In case without having a lymphatic drainage, it may increase as time passes. In 90% of the cases, lymphangioma develops in early childhood up to the age of 2 years.
For the imaging findings, pulmonary lymphangioma may appear as a thin-walled cystic or multi-lobulated mass with a well-defined border and homogeneous density in the localized type. Calcification or contrast enhancement is unusual. At MRI, the cystic component shows iso or low signal intensity to muscle on T1-weighted image and high signal intensity to fat on T2-weighted image. Imaging studies are helpful in delineating the extent of the disease preoperatively but it is difficult to make a proper diagnosis based on the imaging alone as there are no specific imaging features of the lymphangioma.
Pulmonary lymphangioma may remain asymptomatic for many years and only become symptomatic when to the lesion compresses vital structures.
Pulmonary lymphangioma typically requires a surgical excision. Surgery or sclerotherapy are potential treatment options. Incomplete resection, or sclerotherapy can result in the recurrence of the lymphangioma with patient’s symptoms.
References
1. Faul JL, Berry GJ, Colby TV, et al. Thoracic lymphangiomas, lymphangiectasis, lymphangiomatosis, and lymphatic dysplasia syndrome. Am J Respir Crit Care Med. 2000;161:1037-46.
2. Limmer S, Krokowski M, Kujath P. Pulmonary lymphangioma. Ann Thorac Surg. 2008;85:336-9.
3. Nagayasu T, Hayashi T, Ashizawa K, et al. A case of solitary pulmonary lymphangioma. J Clin Pathol. 2003;56:396-8.
4. Raman SP, Pipavath SN, Raghu G, et al. Imaging of thoracic lymphatic diseases. AJR Am J Roentgenol. 2009;193:1504-13.
Keywords
Lung, Developmental,

No. of Applicants : 89

▶ Correct Answer : 1/89,  1.1%
  • - Chonbuk National University Hospital , Korea (South) KUM JU CHAE
▶ Correct Answer as Differential Diagnosis : 1/89,  1.1%
  • - Other , Korea (South) SEONGSU KANG
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Current Editor : Sung Shine Shim, MD, PhD. Email : sinisim@ewha.ac.kr

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