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Weekly Chest CasesCases by Disease Category

Case No : 1295 Date 2022-08-16

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  • Courtesy of Won Jae Choi, Jeong Joo Woo / Nowon Eulji University Hospital
  • Age/Sex 47 / M
  • Chief ComplaintDyspnea, skin nodules
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5
  • Figure 6
  • Figure 7

Diagnosis With Brief Discussion

Diagnosis
Thoracic manifestation of neurofibromatosis type 1 / with malignant peripheral nerve sheath tumor
Radiologic Findings
Fig 1-2. Chest PA and lateral radiograph show a huge well-defined mass opacity at left anterior mediastinum and left superior mediastinal widening with negative cervicothoracic sign. Emphysematous lung with the predominance in both upper lobes is noted.
Fig 3-7. CT scans reveal a well-circumscribed large mass with inhomogeneous
enhancement at aortopulmonary window and a left superior mediastinal mass abutting body of the 1st and 2nd throracic spines. Diffuse bullous emphysema mainly in both upper lobes is observed. Note small skin nodule at right anterior chest wall and thoracic cage deformity.

- Malignant peripheral nerve sheath tumor was confirmed by percutaneous biopsy for anterior mediastinal mass.
Brief Review
- Neurofibromatosis-I (NF1) is the most common of the phakomatoses, occurring with an incidence of 1:2000 to 1:3000. Thoracic manifestation of NF1 can be diverse.
- Cutaneous manifestation includes cutaneous neurofibroma, which can be misinterpreted as pulmonary nodules on plain radiograph.
- Skeletal manifestations involve thoracic spine and ribs. Characteristic rib abnormalities include erosions of either inferior or superior aspect of one or more ribs and the separation of adjacent ribs. Scoliosis, posterior scalloping of the vertebral bodies, and enlargement of one or more neural foramina are typical spinal deformities in NF1.
- Pulmonary abnormalities include fibrosis with basal predominance and thin-walled bullae. Bullae, a characteristic feature, are typically thin-walled and occur in the upper lobes.
- Plexiform neurofibromas are considered pathognomic findings of NF1. A characteristic finding is an infiltration by tumor of multiple tissue types. Both focal and plexiform neurofibromas usually have low attenuation on CT and may contain calcification. On T1-weighted MR image, neurofibromas can show variable signal intensity. However, on T2-weighted MR image, the peripheral portion of the lesion is typically hyperintense and central portion is hypointense.
References
1. Fortman BJ, Kuszyk BS, Urban BA, Fishman EKl. Neurofibroatosis type 1 :
A diagnostic mimicker at CT. Radiographics. 2001;21(3):601-612
Keywords
Neurofibromatosis type 1,

No. of Applicants : 80

▶ Correct Answer : 53/80,  66.3%
  • - Wakayama Red Cross Hospital , Japan TOMOAKI OTANI
  • - Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
  • - Jichi Medical University Hospital , Japan JUN KANZAWA
  • - ZIGONG TCM HOSPITAL OF CHINA , China CAO CUN YOU
  • - Showa General Hospital , Japan MASAFUMI KAIUME
  • - Ajou University Hospital , Korea (South) SUNG HYUN AN
  • - Mayo Clinic , United States AKITOSHI INOUE
  • - McGill University Health Center , Canada ALEXANDRE SEMIONOV
  • - Kyoto University Hospital , Japan YUSUKE UTSUNOMIYA
  • - National Center of Neurology and Psychiatry , Japan MOTO NAKAYA
  • - , Italy PAOLO BALDASSARI
  • - Tiger Gate Hospital , Japan SHIN-ICHI CHO
  • - Vita Hospital , Brazil DIOGO LAGO PINHEIRO
  • - Ajou University Hospital , Korea (South) KYUNGMIN LEE
  • - Other , Korea (South) SEONGSU KANG
  • - Scans world , India PHILSON JOSEPH MUKKADA
  • - Chonbuk National University Hospital , Korea (South) JUNGHWAN KIM
  • - Osaka University , Japan AKINORI HATA
  • - Ajou University Hospital , Korea (South) HAEIN LEE
  • - Shimada General Medical Center , Japan HAYATO NOZAWA
  • - , Korea (South) MINJI KIM
  • - Other , Korea (South) HYEON IL CHOI
  • - Ajou University Hospital , Korea (South) HYUNSEUNG LEE
  • - The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
  • - Ajou University Hospital , Korea (South) MINJI KIM
  • - Yamanashi Prefectural Hospital , Japan HIROAKI WATANABE
  • - Inje University Pusan Paik Hospital , Korea (South) JIYEON HAN
  • - Gifu University Hospital , Japan Yo Kaneko
  • - , Japan KENTARO KOTANI
  • - Kyoto University Hospital , Japan YASUHISA KURATA
  • - Ajou University Hospital , Korea (South) YOU NA KIM
  • - Mallinckrodt Institute of Radiology , United States NAGANATHAN MANI
  • - Osaka Metropolitan University Hospital , Japan SHU MATSUSHITA
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey MERIC TUZUN
  • - University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
  • - Avrasya Hospital/ISTANBUL , Turkey MURAT ULUSOY
  • - Eskisehir Sehir Hastanesi , Turkey MURAT SAHIN
  • - , United Arab Emirates NEHAR RANJAN CHAKRABORTY
  • - Chonnam National University Hwasun Hospital , Korea (South) JEONG YEOP LEE
  • - Manipal hospital Hebbal ,Bengaluru , India PRAVIN KUMAR M
  • - Osmania Medical College, Hyderabad , India PURUSHOTHAMA RAO TUMMALA
  • - Other , Korea (South) KYU-CHONG LEE
  • - Kyoto University Hospital , Japan SATOSHI IKEDA
  • - Seoul National University Bundang Hospital , Korea (South) YOONAH DO
  • - Toranomon Hospital , Japan KAORU SUMIDA
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - Nerimahikarigaoka hospital , Japan TAKANA HAYASHI
  • - , Japan YUMI MAEHARA
  • - , Japan YUKI HAYASHI
  • - , Korea (South) HM PARK
  • - the first affiliatited hospital of nanjing medical univercity , China HAI XU
  • - Hanyang University Hospital , Korea (South) SEUNG JIN YOO
  • - Jiangsu province hospital , China WANGJIAN ZHA
▶ Correct Answer as Differential Diagnosis : 4/80,  5.0%
  • - Narayana Multispeciality Hospital Jaipur Rajasthan , India JAINENDRA JAIN
  • - McGill , Canada MUTAZ ADNAN KHAIRO
  • - Osaka metropolitan university Hospital , Japan TATSUSHI OURA
  • - Pusan National University Yangsan Hospital , Korea (South) JI HWAN KIM
▶ Semi-Correct Answer : 8/80,  10.0%
  • - Kinki University Faculty of Medicine, , Japan MITSURU MATSUKI
  • - , Korea (South) JIN YOUNG LEE
  • - Oita University, Faculty of Medicine , Japan FUMITO OKADA
  • - , India JAWED AKHTER
  • - Chungbuk National University Hospital , Korea (South) MIHYEON PARK
  • - Other , Korea (South) SEONG WON JANG
  • - Yonsei University,Severance Hospital , Korea (South) SEO BUM CHO
  • - Diagnose.me (BV) / Royal Perth Hospital , Australia YURANGA WEERAKKODY
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