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Weekly Chest CasesArchive of Old Cases

Case No : 377 Date 2005-01-15

  • Courtesy of Ju-Hyun Lee, Tae-Sung Kim / Samsung Medical Cneter, Sungkyunkwan University, Korea
  • Age/Sex 19 / F
  • Chief ComplaintMultiple skin nodules
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5

Diagnosis With Brief Discussion

Diagnosis
Plexiform Neurofibromatosis
Radiologic Findings
CT scans show multiple round and well-defined masses along left paraspinal area, left chest wall and left intercostal nerve with chest wall deformity. And also diffuse low attenuating retroperitoneal mass encasing great vessel branch is seen.
Brief Review
Von Recklinghausen neurofibromatosis is a hamartomatous disorder that primarily involves the ectoderm and mesoderm. Several types of neurofibromas occur in this disorder and are distinguished on the basis of their gross and microscopic appearances. The localized neurofibroma, the most common type, is a focal lesion that typically is located in the dermis and subcutis (1). The diffuse neurofibroma is localized in the subcutis, usually in the head and neck region. Plexiform neurofibromas, which are considered pathognomonic of von Recklinghausen neurofibromatosis, usually occur in the neck, pelvis, and extremities, but they may be seen in virtually any location, including the thorax (1-4). In the thorax, the sympathetic chains are most commonly involved, but plexiform neurofibromas of the vagus and phrenic nerves are rare (5).

Plexiform neurofibromas appear as a diffuse fusiform enlargement of the peripheral nerves and /or as multiple masses along the course of the peripheral nerves (4). On CT scans, plexiform neurofibromas have a low attenuation compared with that of muscle tissue, which CT values ranging from 15 to 20 H on unenhanced scans. The lesions have ill-defined margins with mediastinal fat. Peripheral enhancement after administration of IV contrast material and calcifications can be observed (6), although both findings may occur in neurofibromas and neurofibosarcomas as nonspecific findings (3, 4).
References
1. Enzinger FM, Weiss SW. Benign tumors of peripheral nerves. In: Enzinger FM, Weiss SW, eds. Soft-tissue tumors. St.
Louis: Mosby, 1983: 580-624

2. Biondetti PR, Vigo M, Fiore D, DeFaveri D, Rovasini R, Benedetti L. CT appearance of generalized von Recklinghausen
neurofibromatosis. J Comput Assist Tomogr 1983; 7:866-869

3. Aughenbaugh GL. Thoracic manifestations of neurocutaneous disease. Radiol Clin North Am 1984; 22:741-756

4. Kumar AJ, Kuhajda FP, Maatinez CR, Fishman EK, Jezic DV, Siegelman SS. Computed tomography of extracranial nerve
sheath tumors with pathological correlation. J Comput Assist Tomogr 1983; 7:857-865

5. Strickland B, Wolverson MK. Intrathoracic vagus nerve tumors. Thorax 1974; 29:215-222

6. Bourgouin PM, Shepard JO, Moore EH, McLoud TC. Plexiform neurofibromatosis of the mediastinum: CT appearance. AJR
1988; 151:461-463
Keywords
Chest wall, Benign tumor,

No. of Applicants : 55

▶ Correct Answer : 52/55,  94.5%
  • - Aditya Imaging Centre, Baroda, India Vivek Patel
  • - Al-Mana General Hospital. Saudi Arabia Tamer I.F. Marei
  • - Annecy Hospital, France Gilles Genin
  • - Apollo Firstmed Hospitals, Chennai,India RG Gopinath
  • - Apollo Hospitals, Colombo P.Xavier Rajesh
  • - Asan Medical Center, Korea Eun Jin Chae
  • - ASL BOLOGNA Maggiore Hospital, Bologna, Italy Marcellino Burzi
  • - Busan Veterans Hospital, Korea Suhku Huh
  • - Centre d'imagerie Jacques Callot, Nancy, France Lionel Cannard
  • - Centre Hospitalier Universitaire de Montreal, Qc, Canada Jean-Sebastien Billiard
  • - CHU Grenoble, France Mathieu Rodiere
  • - CHU Nancy-Brabois, France Denis Regent
  • - CIM Saint Dizier, France JC Leclerc
  • - Dong-A University Hospital, Korea Ki-Nam Lee
  • - European Hospital, Paris, France Rached Triki
  • - Ev. Krkhs. Hubertus, Berlin, Germany Michael Weber
  • - Ewha Womans University Mokdong Hospital, Korea Yoo Kyoung Kim
  • - GeojeBaek Hosp., Gyungnam, Korea James Lim
  • - Govt. Medical College, Vadodara , India Vijay Vaidya
  • - Gwangmyoung Sung-Ae Hospital, Korea Jiyong Rhee
  • - Hangang SacredHeart Hospital, Korea Eil Seong Lee
  • - Hansarang Internal Medicine Hospital, Korea Moo Song Kang
  • - Hanyang University Hospital, Seoul, Korea Yo Won Choi
  • - Hhtr.nhs.UK Thomas Joy
  • - Homs National Hospital, Homs, Syria Rami Abou Zalaf
  • - Hospital Cotia Saude - Brazil JLM de Almeida Prado
  • - Hospital Monaldi, Naples, Italy Gaetano Rea
  • - Incheon Sarang Hospital, Korea Jung Hee Kim
  • - Jeil hospital, Jinju, Korea Hyunggon Lee
  • - Jikei University of medicine, Japan Shigeki Misumi
  • - Jikei University, Tokyo, Japan Masuo Ujita
  • - Korea University Hospital, Korea Kyoung-Rae Kim
  • - Kyungpook National University Hospital, Korea Shin Kyung-min
  • - Leeds General Infirmary, UK Girish Raghunathan
  • - Maimonides Medical Center, USA Naomi Twersky
  • - Marien Hospital, Hamm, Germany Davis Chiramel
  • - Max Hospital, Delhi, India Vickrant Malhotra
  • - National Taiwan University Hospital, Taiwan Shen-Hsiang Lin
  • - National Taiwan University Hospital, Taiwan Yi-Ting Wang
  • - Nirman Hi-tech Diagnostic Centre, India Minal Seth
  • - NY, USA Venkatram
  • - Ondokuz Mayis University, Samsun, Turkey Cetin Celenk
  • - Pusan National University Hospital, Korea Soo Jin Kim
  • - Ping Tung Christian Hospital,Taiwan Jun-Jun Yeh
  • - Samsung Medical Center, Korea Sun Young Jeong
  • - Shiga University of Medical Science, Shiga, Japan Yukihiro Nagatani
  • - Social Security Hospital, Ankara, Turkey Meric Tuzun
  • - Soonchunhyang University Cheonan Hospital, Korea Hae Seung Lee
  • - Sweida National Hospital, Syria Marwan Al Chami
  • - University of Colorado, USA Brian Petersen
  • - Voila, France Faites un voeu et Puis
  • - Wagga Medical Imaging, NSW, Australia Rashid Hashmi
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Current Editor : Sung Shine Shim, MD, PhD. Email : sinisim@ewha.ac.kr

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