대한흉부영상의학회 Weekly Chest Cases

Case No : 833 Date 2013-10-14

Courtesy of Shantiranjan Sanyal, Poonam Narang, Satbir Singh / G.B. Pant Hospital, New Delhi, India
Age/Sex 33 / F
Chief ComplaintSlowly progressive shortness of breath, cough with abdominal fullness On clinical examination: features of superior vena cava syndrome with splenomegaly.

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Diagnosis With Brief Discussion

Diagnosis: Thoraco-abdominal lymphangiomatosis
Radiologic Findings: CT scan of thorax at the level of aortic arch shows hypodense lesion of fluid attenuation filling the precarinal and prevascular space with dilated SVC and azygous vein. Inferiorly the lesion is extending into aorto pulmonary window, azygo esophageal recess and encircling all around the ascending and descending aorta.
At the level of left atrium and ventricles shows the same lesion in middle and posterior mediastinum encircling the pulmonary veins and descending aorta without luminal narrowing, involving the pericardium and extending across the midline.
Coronal reformatted images show extensive thoraco-abdominal extension of the non-enhancing lesion involving multiple compartments and spaces without obvious mass effect or deformation of the other visceral organs.
Brief Review: Lymphangiomas are congenital lesions that develop from sequestered lymphatic sacs with histologically three types of capillary,cavernous and cystic. They are most commonly seen as cervical ,axillary masses, while mediastinum and retroperitoneum are uncommon locations. They are developmental abnormalities rather than true neoplasms and are always benign.
Diffuse non-localized proliferation of lymphatic spaces with wide spread involvement of multiple peritoneal compartments and visceral organs is termed as lympahngiomatosis.
The condition tends to affect children and young adults, without predilection for either sex. Mediastinal involvement is common in young adults with coexistence of chylous pleural and pericardial effusions.
Clinical findings in these patients include cough,dyspnea,dysphagia,superior vena cava syndrome,organomegaly ,features related to secondary infection or completely asymptomatic.
Imaging studies reveal the diffuse distribution with an infiltrative cystic mass without any perceptible wall enveloping or conforming to the shape of adjacent mediastinal structures, peritoneal organs without any mass effect. They are developmental anomaly of dilated lymphatic channels is supported by elongated nature of the tumor and crossing multiple compartments in peritoneal cavity without any invasiveness.
The disseminated nature of the disease limits the surgical management to palliative procedures.
References: 1. Charruau L, Parrens M, Jougon J, et al. Mediastinal lymphangioma in adults: CT and MR imaging features. Eur Radiol 2000;10:1310
Keywords: mediastinum, benign tumor,

No. of Applicants : 72

▶ Correct Answer : 9/72, 12.5%

- Niigata City General Hospital , Japan Takao Kiguchi
- University of ottawa , Canada Subramaniyah Ramanathan
- Dept. Radiology, Kyungpook medical school hosipital , Korea (South) Hyejin Cheon
- Onomichi municipal hospital , Japan Hirofumi Mifune
- universidad de chile , Chile aguilera enrique
- Asan medical center , Korea (South) yoonyoung choi
- Kizawa Memorial Hospital , Japan Yo Kaneko
- ASSAM MEDICAL COLLEGE , ASSAM , INDIA , India DEB BORUAH
- Fortis hospital , Mohali , India Shaleen Rana

▶ Correct Answer as Differential Diagnosis : 11/72, 15.3%

- Avrasya Hospital , Turkey Murat Ulusoy
- Asan Medical Center, Ulsan University , Korea (South) Mi Young Kim
- Shiga University of Medical Science , Japan Akitoshi Inoue
- chungbuk national university hospital , Korea (South) jeehye kim
- CBNUH , Korea (South) jihyung lee
- chungbuk national university hospital , Korea (South) soojung lee
- Chungbuk univertisy hospital , Korea (South) Soohyun Lee
- Chungbuk University Hospital , Korea (South) Yunhee Jang
- CLINIQUE STE CLOTILDE , Reunion patrick MASCAREL
- Kyungpook National University Hospital , Korea (South) Jaekwang Lim
- Medicheck health care , Korea (South) Chae Lim

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 833 Date 2013-10-14

Discussion

No. of Applicants : 72