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Weekly Chest CasesArchive of Old Cases

Case No : 65 Date 1999-01-23

  • Courtesy of Jeung Sook Kim, M.D., Sam Hyun Yoon, M.D. / Pundang CHA General Hospital, Pochon CHA university
  • Age/Sex 46 / F
  • Chief Complaintincidental chest radiograph abnormality
  • Figure 1
  • Figure 2
  • Figure 3

Diagnosis With Brief Discussion

Diagnosis
Plexiform neurofibromatosis in von Recklinghausen disease
Radiologic Findings
On chest PA, well-defined large mediastinal mass is seen in far posterior mediastinum without obliteration of the aortic arch. On pre-enhanced scan, multiple well-defined ovoid masses are seen in the left paravertebral area, right costochondral area and azygoesophageal recess. The right paravertebral mass shows widening of neural foramen with bony erosion. All of these are not well enhanced and show relatively low-density. And numerous protruding skin lesions are seen in entire chest wall.
Brief Review
Classic neurofibromatosis (or von Recklinghausen’s disease) is a hereditary, autosomal dominant, hamartomatous disorder. Severeal types of neurofibromas occur in von Recklinghausen’s disease 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. 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 thorax. In the thorax, the sympathetic chains are most commonly involved.
Plexiform neurofibromas appear as a diffuse fusiform enlargment of the peripheral nerves and/or as multiple masses along the course of the peripheral nerves. Plexiform neurofibromas have a low attenuation compared with that of muscle tissue, with CT values ranging from 15 to 20 H on non-enhanced scans. The low attenuation of these lesions is related to the presence of the lipidic Schwann cells, the high water content of the mucinous matrix, entrapment of perineural adipose tissue, and cystic degeneration. Peripheral enhancement after administration of IV contrast material and calcifications may occur in neurofibromas and neurofibrosarcomas and are nonspecific findings. And lateral menigocele, interstitial pulmonary fibrosis, thoracic skeletal deformaities (scoliosis with or without kyphosis), optic glioma, astrocytoma, acoustic neuroma and menigiomas are also developed in von Recklinghausen’s disease.
Lateral thoracic meningoceles are protrusion of the spinal meninges through the intervertebral foramina. They are detected in patients between 30 and 60 years of age. Approximately two thirds of cases occur in association with neurofibromatosis. Enlargement of the adjacent intervertebral foramen is an important diagnostic feature. This sign is identical to that seen with dumbbell nerve sheath tumors- a diagnostic problem complicated by the fact that both conditions are frequently associated with neurofibromatosis. CT scan shows the low attenuation value of the mass because much of its bulk consists of CSF.
References
1.Bourgouin PM, Shepard JO, Moore EH, McLoud TC. Plexiform neurofibromatosis of the mediastinum: CT appearance. AJR 1988;151:461-463
2.Aughenbauch GL. Thoracic manifestations of neurocutaneous diseases. RCNA 1984;22:741-756
Keywords
Mediastinum, Chest wall, Benign tumor, Plexiform neurofibromatosis in von Recklinghausen disease

No. of Applicants : 41

▶ Correct Answer : 38/41,  92.7%
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  • - AZ Vesalius Tongeren, Belgium Dr. Rudi Stokmans
  • - Dumfries & Galloway Royal Infirmary, Scotland David Hill
  • - Northwestern Memorial Hospital, Chicago, IL. USA Mitchell J. Kline
  • - SGPGIMS, Lucknow, India Dr. Rajan Jain
▶ Semi-Correct Answer : 3/41,  7.3%
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  • - Sir H N Hospital, Mumbai, India Dr. Bhavin Jankharia
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