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Weekly Chest CasesArchive of Old Cases

Case No : 124 Date 2000-03-11

  • Courtesy of Kyung Soo Lee, M.D. / Sungkyunkwan University School of Medicine, Samsung Medical Center, Seoul, Korea
  • Age/Sex 17 / M
  • Chief ComplaintMultiple skin nodules
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4

Diagnosis With Brief Discussion

Diagnosis
Plexiform Neurofibromatosis
Radiologic Findings
CT scans show round and well-defined masses of longitudinally elongated shape in bilateral mediastinum.
The masses conform to the expected courses of bilateral vagus nerves.
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
Mediastinum, Benign tumor,

No. of Applicants : 59

▶ Correct Answer : 43/59,  72.9%
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  • - pusan100
  • - Baptist Hospital, Pusan Mi-Jeong Shin
  • - Dong-A University Hospital Ki-Nam Lee
  • - Gachon Medical School Gil Medical Center Seo Joon Beom
  • - Stedelijk OLV Ziekenhuis Mechelen, Belgium Ivan Pilate
  • - Pusan National University Hospital, Pusan, Korea Kun-Il Kim
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