Weekly Chest CasesArchive of Old Cases

Case No : 35 Date 1998-06-29

  • Courtesy of KUN-IL KIM, M.D. / Pusan National University Hospital
  • Age/Sex 22 / M
  • Chief ComplaintIncidental chest PA abnormality, skin nodules on thigh, chest and abdomen for several years
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Chest PA

Diagnosis With Brief Discussion

Diagnosis
Mediastinal plexiform neurofibromatosis
Radiologic Findings
Brief Review
Chest radiograph shows mediastinal widening and notching of left 3rd rib. Rib notching in this clinical situation is highly suggestive of mass arising from intercostal nerve although infracostal mass is not definitely seen in this case.
CT reveals multicompartmental mediastinal lesions of low attenuation (less than muscle) and bilateral nipple-like protrusion abutting the heart, which correspond to the course of the phrenic nerves. In the appropriate clinical setting, CT detection of lesions in the distribution of the mediastinal nerves strongly favors the diagnosis of plexiform neurofibromatosis.
References
Bourgouin PM, Shepard JO, Moore EH, McLoud TC. Plexiform neurofibromatosis of the mediastinum: CT appearance. AJR 1988;151:461-463
Keywords
Mediastinum, Chest wall, Benign tumor, Mediastinal plexiform neurofibromatosis

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