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Weekly Chest CasesArchive of Old Cases

Case No : 1003 Date 2017-01-16

  • Courtesy of So Youn Shin, Dong Wook Sung / Kyung Hee University Hospital
  • Age/Sex 28 / F
  • Chief Complaintcough (onset: 1 month ago)
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5

Diagnosis With Brief Discussion

Diagnosis
Neurofibromatosis type 1
Radiologic Findings
Fig 1. Chest PA shows bilateral mediastinal bulging shadows.
Fig 2-5. On chest CT, there are multiple conglomerated soft tissue lesions in the mediastinum, supraclavicular fossae, axillae, r and the upper abdomen. The masses show homogeneous low attenuation without significant enhancement. Some masses show encasement of surrounding structures without mass effect or invasion. Also, there are other multiple nodules and low attenuated lesions in the subcutaneous fat layer and the muscles of the chest wall.
Brief Review
Neurofibromatosis type 1 (NF1), or von Recklinghausen disease, is the most common of the phakomatoses and has a localized or systemic manifestations involving the thorax, abdomen, pelvis, and extremities.
Typical CT findings in patients with NF1 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 in the thorax. NF1 in the abdomen frequently involves the retroperitoneal, mesenteric, and paraspinal areas. The typical peripheral manifestations of NF1 include limb hemihypertrophy, pseudarthrosis, peripheral nerve neurofibromas, and subcutaneous common and plexiform neurofibromas.
Differential diagnosis for multiple mediastinal lymphadenopathies include sarcoidosis, tuberculosis, lymphoma, metastasis, and so on. Up to 73% of cases of masses in NF1 demonstrate characteristic low attenuation, but they may show soft-tissue attenuation at contrast enhanced CT. Factors responsible for this low attenuation include cystic degeneration, xanthomatous features, confluent areas of hypocellularity, and lipid-rich Schwann cells.
References
1. Fortman BJ, Kuszyk BS, Urban BA, Fishman EK. Neurofibromatosis Type 1: A Diagnostic Mimicker at CT. RadioGraphics 2001; 21:601-612.
Keywords
mediastinum, chest wall,

No. of Applicants : 123

▶ Correct Answer : 30/123,  24.4%
  • - , Turkey ELIF YASEMIN GULUM
  • - Seoul St. Mary , Korea (South) Myeong Im Ahn
  • - Kizawa Memorial Hospital , Japan Tomohiro Ando
  • - Korea University Guro Hospital , Korea (South) Bit Na Park
  • - Ajou university hospital , Korea (South) Pae Sun Suh
  • - Korea University Guro Hospital , Korea (South) PARK SOYEON
  • - The University of Tokyo Hospital , Japan Yusuke Watanabe
  • - Korea University Guro Hospital , Korea (South) GAYOUNG CHOI
  • - KAMC , Saudi Arabia NESREEN HASSAN ABOUROKBAH
  • - Asan Medical Center , Korea (South) HYUN JUNG KOO
  • - Beaulieu clinic Geneva , Switzerland gilles GENIN
  • - The first affiliated Hospital of Xiamen University , China Qing Qiang Guo
  • - Ajou University School of Medicine , Korea (South) YOOLIM BAEK
  • - Qatif Central Hospital , Saudi Arabia HANI MAKKY ALSALAM
  • - Onomichi municipal hospital , Japan Hirofumi Mifune
  • - Asan Medical Center , Korea (South) HONG SANG HYUP
  • - Dong-A University, College of Medicine , Korea (South) KI-NAM LEE
  • - , United Kingdom KRISHNA PRASAD PREMNATH BELLAM
  • - Hallym University Medical Center , Korea (South) HAYEON LEE
  • - University of Tsukuba, Dept of Radiology , Japan Manabu Minami
  • - , Korea (South) HYERIN KIM
  • - Azienda Ospedaliera di Cremona , Italy Pietro Sergio
  • - Seoul National University Hospital , Korea (South) KUM JU CHAE
  • - Ajou University Hospital , Korea (South) SEULGI YOU
  • - Korea University Guro Hospital , Korea (South) SUN HONG
  • - The University of Tokyo Hospital , Japan KAORU SUMIDA
  • - Hallym University Medical Center , Korea (South) HWANG HYE JEON
  • - CNUH , Korea (South) LEE JONG EUN
  • - the first affiliatited hospital of nanjing medical univercity , China HAI XU
  • - Korea University Guro Hospital , Korea (South) Yoonmi Choi
▶ Correct Answer as Differential Diagnosis : 10/123,  8.1%
  • - ZIGONG TCM HOSPITAL OF CHINA , China Cao Cunyou
  • - The University of Tokyo Hospital , Japan Akifumi Hagiwara
  • - Seoul National University Hospital , Korea (South) HAN SEUNGCHUL
  • - Mashhad University of Medical Sciences , Iran BITA ABBASI
  • - tongji medical university affiliated jingzhou central hospital , China HUANG YUAN YI
  • - Asan Medical Center , Korea (South) JIHYE KWON
  • - GHICL , France MANUEL TOLEDANO
  • - Aerospace medical group, Air Force Education and Training Command , Korea (South) Donghwan Kim
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - Diagnose.me (BV) / Royal Perth Hospital , Australia YURANGA WEERAKKODY
▶ Semi-Correct Answer : 1/123,  0.8%
  • - Korea university medical center, guro hospital , Korea (South) Lee kyu chong
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