대한흉부영상의학회 Weekly Chest Cases

Case No : 711 Date 2011-06-13

Courtesy of Ki-Tae Han, Jin-Hwan Kim, Song-soo Kim. / Chungnam National University Hospital
Age/Sex 57 / M
Chief ComplaintButtock mass, incidental finding

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Figure 1

Diagnosis With Brief Discussion

Diagnosis: Neurofibromatosis type 1(Von Recklinhausen\'s disease)
Radiologic Findings: Chest radiograph and HRCT show the multiple, well-demarcated bullous cystic lesion with predominent distribution of both upper lobes.
Abdomen CT images show multifocal variable manifestation of neurofibromas in Lt. paraspinal complex cystic mass, subcutaneous soft tissue mass of lower back and multiple subcutaneous nodules.
Brief Review: Von Recklinhausen's disease or neurofibromatosis type 1(NF) is an autosomal dominant dysplasia of ectoderm and mesoderm with a variable clinical expression characterized by collections of neurofibromas, caf�-au-lait spots and Lisch nodules.
In NF, the thorax and lungs can be affected in several ways: cutaneous and subcutaneous neurofibromas on the chest wall; kyphoscoliosis; ribbon deformity of the ribs; thoracic neoplasms; and interstitial lung disease(ILD). Rarely, it can be associated with multifocal lung cysts. On chest radiography, bullous lung disease is present, almost always in the upper lobes. Others findings are basilar linear densities and radiographic honeycombing. HRCT revealed emphysema, cyst, ground-glass abnormality, bullae and reticular abnormalities.
References: 1. Cantin L, Bankier AA, Eisenberg RL. Multiple cystlike lung lesions in the adult. AJR Am J Roentgenol 2010;194:W1-W11
2. Zamora AC, Collard HR, Wolters PJ, Webb WR, King TE. Neurofibromatosis-associated lung disease: a case series and literature review. Eur Respir J 2007;29:210-214
3. 신소연, 이영경, 문아림, 성동욱. 자발성 기흉으로 발현된 제1형 신경섬유종증: 증례보고. 대한영상의학회지 2010;63:379-382
Keywords: lung, congenital,

No. of Applicants : 73

▶ Correct Answer : 22/73, 30.1%

- Fukuyama daiichi Hospital , Japan Mototsugu Saeki
- Hangang sacred heart hospital , Korea (South) You Mie Han
- Chaeil Clinic , Korea (South) Sunkyung Lim
- CHA Bundang hospital , Korea (South) Hyerin Kim
- Onomichi municipal hospital , Japan Hirofumi Mifune
- PingTung Christian Hospital ,China Medical University ,Taiwan,R.O.C. , Taiwan Jun Jun Yeh
- Az Osp Santa Maria Terni , Italy angelo carloni
- Chonnam national university hospital , Korea (South) Jang-hyeon Song
- Department of Radiology, Chonnam National University Hospital , Korea (South) Hyungjun Kim
- IRSA La Rochelle France , France Denis Chabassiere
- Okayama University , Japan Akihiro Tada
- Soon Chun Hyang, Seoul , Korea (South) Yoo Na Kim
- Chungnam National University hospital , Korea (South) sunkyoung you
- sunder lal jain hospital , India sanjeev vachher
- Diskapi Yildirim Beyazit Hospital, Ankara , Turkey Meric Tuzun
- yeong gwang general hospital , Korea (South) Mokhee Kim
- HSC , Brazil Diogo Pinheiro
- Istanbul , Turkey Armagan Sarac
- All India Institute of medical sciences , India Justin Moses
- Saga University , Japan Ryoko Egashira
- Gangnam Severance Hospital , Korea (South) Min Jeong Kim
- Pneumologia Universitaria, Policlinico di Bari , Italy Mario Damiani

▶ Correct Answer as Differential Diagnosis : 6/73, 8.2%

- Yokohama-asahi-chuo-general hospital , Japan Kyoko Nagai
- Hopital Louis Pradel LYON , France matthieu ROUSSET
- IRCCS Istituto Oncologico - Bari , Italy Carlo Florio
- Hotel-Dieu Montreal , Canada nicolas gautier
- Mallinckrodt Institute of Radiology , United States Travis Henry
- Severance hospital , Korea (South) Saerom Hong

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 711 Date 2011-06-13

Discussion

No. of Applicants : 73