Correlapaedia - a Correlative Encyclopedia of Pediatric Imaging, Surgery, and Pathology
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.
Clinical Differential Diagnosis:
Spinal films and a computed tomography exam demonstrated the extent of the right thoracic mass and its bony involvement.
Images 2 and 3
Imaging Differential Diagnosis:
Probably malignant degeneration of the plexiform neurofibroma due to its rapid increase in size.
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.
Examination of the surgical specimen revealed characteristic findings of malignant schwannoma within a plexiform neurofibroma.
Neurofibroma of Thorax (Malignant)
Follow-up and Prognosis:
The patient had an uncomplicated post-operative course.
Case 27, Case 39
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