Correlapaedia - a Correlative Encyclopedia of Pediatric Imaging, Surgery, and Pathology

Case 31

Clinical History:
The patient was an 11 year old male with a long-standing history of plexiform neurofibromatosis involving his right arm and chest. His past surgical history was remarkable for having multiple resections of benign neurofibromas in the past.

Clinical Physical Exam:
Remarkable for an extensive soft tissue mass involving his right arm and chest.

Image 1

Clinical Labs:
Non-contributory

Clinical Differential Diagnosis:
Neurofibromatosis

Imaging Findings:
Spinal films and a computed tomography exam demonstrated the extent of the right thoracic mass and its bony involvement.

Images 2 and 3

Image 4

Imaging Differential Diagnosis:
Probably malignant degeneration of the plexiform neurofibroma due to its rapid increase in size.

Operative Findings:
The patient underwent elective resection of the large right posterior mediastinal mass. During the thoracotomy, the large mass was seen to originate in the apex of the right chest.

Image 5

Pathological Findings:
Examination of the surgical specimen revealed characteristic findings of malignant schwannoma within a plexiform neurofibroma.

Image 6

Final Diagnosis:
Neurofibroma of Thorax (Malignant)

Follow-up and Prognosis:
The patient had an uncomplicated post-operative course.

Similar Cases:
Case 27, Case 39

References:

• Paediapedia

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