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Weekly Chest CasesArchive of Old Cases

Case No : 973 Date 2016-06-20

  • Courtesy of Mi-Jin, Kang / Inje University, Sanggye Paik Hospital
  • Age/Sex 45 / M
  • Chief ComplaintAbnormal findings on chest radiograph
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4

Diagnosis With Brief Discussion

Diagnosis
Neurofibromatosis type 1 .
Radiologic Findings
Fig 1. Chest PA shows multiple well defined pleural based nodules.
Fig 2-3. Chest CT scans show multiple well-marginated, smooth, round or ovoid shape masses in the in the paravertebral region or intercostal space along the course of intercostals nerves

Fig 4. Bone window setting of chest CT scan shows mild erosion in the inferior aspect of ribs

Brief Review
Neurofibromatosis type 1 (NF1), or von Recklinghausen disease, is a comparatively common hereditary disease in which the skin, nervous system, bones, endocrine glands, and sometimes other organs are the sites of a variety of congenital abnormalities, which often take the form of benign tumors.

Neurofibromas arise from Schwann cells and fibroblasts and may arise in any peripheral nerve. Given the rich distribution of peripheral nerves throughout the thorax, manifestations of NF1 may involve the ribs, chest wall, lungs, and mediastinum.
Classic imaging findings include small, well-defined subcutaneous neurofibromas, focal thoracic scoliosis, posterior vertebral scalloping, enlarged neural foramina, and characteristic rib abnormalities due to bone dysplasia or erosion from adjacent neurofibromas. Neurofibromas typically appear as well-marginated, smooth, round or elliptic masses in the paravertebral regions or along the course of the vagus, phrenic, recurrent laryngeal, or intercostal nerves. Although these tumors may demonstrate soft-tissue attenuation at contrast material–enhanced CT, low attenuation is characteristic and is seen in up to 73% of cases.
References
1. Brian J. Fortman, MD, Brian S. Kuszyk, MD, Bruce A. Urban, MD Elliot K. Fishman, MD, Neurofibromatosis Type 1: A Diagnostic Mimicker at CT, RadioGraphics 2001; 21:601–612
2. Santiago E. Rossi1, Jeremy J. Erasmus, H. Page McAdams, Lane F. Donnelly, Pictorial essay-Thoracic Manifestations of Neurofibromatosis-1, AJR 1999:173:1631-1638
3. Williams VC, Lucas J, Babcock MA et-al. Neurofibromatosis type 1 revisited. Pediatrics. 2009;123 (1): 124-33

Keywords
Chest wall, Paravertebral, Benign tumor,

No. of Applicants : 94

▶ Correct Answer : 52/94,  55.3%
  • - The University of Tokyo Hospital , Japan Akifumi Hagiwara
  • - university of montreal , Canada Andrei Gorgos I
  • - Suncheon Plus Medicine , Korea (South) Park Sang Hyun
  • - Aerospace medical group, Air Force Education and Training Command , Korea (South) Donghwan Kim
  • - Dong-A University Hospital , Korea (South) Ji-yeon Han
  • - The University of Tokyo Hospital , Japan Yusuke Watanabe
  • - Otsu Municipal Hospital , Japan Noriatsu Ichiba
  • - NIMS, HYDERABAD , India bhaskar kakarla
  • - The University of Tokyo Hospital , Japan Toshihiro Furuta
  • - Jangsung public clinic , Korea (South) Yongsub Song
  • - Shanghai Jiao Tong University Affiliated Sixth People萸 , China Jiayin Zhang
  • - Kyoto University , Japan Akihiko Sakata
  • - Red Cross Society Himeji Hospital , Japan Soichiro Okamoto
  • - Shiga University of Medical Science , Japan Akitoshi Inoue
  • - King Abdulaziz University Hospital , Saudi Arabia Amr Ajlan
  • - Ajou University Hospital , Korea (South) Seulgi You
  • - Seoul National University Bundang Hospital , Korea (South) Jee Hye Hur
  • - Seoul National University Bundang Hospital , Korea (South) Youngjune Kim
  • - radiologist, aditya imaging centre , India vivek patel
  • - �stanbul , Turkey Ayhan Yilmaz
  • - Department of Radiology, Seoul National University Bundang Hospital , Korea (South) Sung Hyun Yoon
  • - Okayama University Hospital , Japan Toshiyuki Komaki
  • - University of Tsukuba, Dept of Radiology , Japan Manabu Minami
  • - University of Tsukuba Hospital , Japan Hiroaki Takahashi
  • - Yokohama-asahi-chuo-general hospital , Japan Kyoko Nagai
  • - Policlinico Umberto I , Italy Paolo Baldassari
  • - Ulsan University Hospital , Korea (South) Soyeoun Lim
  • - Gifu University Hospital , Japan Yo Kaneko
  • - Asan Medical Center , Korea (South) Hyung Jung Koo
  • - Sangju Rad Cross Hospital , Korea (South) Dong-Ho Bang
  • - Tong young Red cross hospital , Korea (South)
  • - Asan Medical Center , Korea (South) Chan Ho Park
  • - the first affiliatited hospital of nanjing medical univercity , China Hai Xu
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - Government Medical College, Nagpur, India , India Krishna Prasad Bellam
  • - istituti ospitalieri di cremona , Italy pietro sergio
  • - Niigata University , Japan Atsushi Uehara
  • - Chung buk national university hospital , Korea (South) hong Byun
  • - , Korea (South)
  • - Chungbuk national university hospital , Korea (South) Miran Yeon
  • - IRSA LA ROCHELLE , France, Metropolitan BIGOT
  • - The University of Tokyo Hospital , Japan Kaoru Sumida
  • - Asan Medical Center, Ulsan University , Korea (South) Mi Young Kim
  • - Les cedres St malo , France nicolas gautier
  • - Onomichi municipal hospital , Japan Hirofumi Mifune
  • - Chungbuk national university , Korea (South) Yong Kim
  • - Fortis hospital , Mohali , India Shaleen Rana
  • - NASA SCANS , India RAKESH BHATIA
  • - Kanto Rosai Hospital , Japan Taku Tajima
  • - Medicheck health care , Korea (South) Chae Lim
  • - Hyogo Prefectural Amagasaki General Medical Center , Japan Tomoaki Otani
  • - University of Tsukuba , Japan Toshitaka Ishiguro
▶ Correct Answer as Differential Diagnosis : 7/94,  7.4%
  • - Saitama-Sekishinkai Hosptal , Japan Mihoko Yamazaki
  • - McGill University Health Center , Canada Alexandre Semionov
  • - CNUH , Korea (South) 源€
  • - the First Affiliated Hospital of NanJing Medical University , China Aiping Chen
  • - Centre Imagerie de Fribourg , Switzerland Benoit RIZK
  • - ZIGONG TCM HOSPITAL OF CHINA , China Cao Cunyou
  • - Kyung Hee University Hospital at Gangdong , Korea (South) Han Na Lee
▶ Semi-Correct Answer : 11/94,  11.7%
  • - SAISEIKAI KURIHASHI HOSPITAL , Japan YASUO OOKUBO
  • - Amagasaki General Medical Center , Japan Genki Fukumoto
  • - Pamukkale University, Faculty of Medicine, Dept. of Radiology , Turkey Furkan Ufuk
  • - Kashan University of Medical Sciences, kashan , Iran Ebrahim Razi
  • - Maternity & child hospital , Saudi Arabia Sania ALBlushi
  • - CHU Poitiers , France CHAN paul
  • - Ishikawa Matto Central Hospital , Japan Manabu Akimoto
  • - 異⑸ , Korea (South) Junghwan Kim
  • - Private sector , Greece Vasilios Tzilas
  • - chungbuk university hospital , Korea (South) Hyeonmi Ryu
  • - Himeji Red Cross Hospital , Japan Mayu Uka
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