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Virtual Pediatric Hospital: Thoracopaedia: An Imaging Encyclopedia of Pediatric Thoracic Disease: Case 68 Thoracopaedia: An Imaging Encyclopedia of Pediatric Thoracic Disease

Case 68

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed


Chief Complaint:
Eleven year old male with a large soft tissue mass involving his right arm and chest.

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.

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Clinical Labs:
Non-contributory

Clinical Differential Diagnosis:
Neurofibromatosis

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

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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.

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

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Final Diagnosis:
Neurofibroma of Thorax

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

Similar Cases:
Case 15, Case 18, Case 27, Case 46, Case 67, Case 69

References:
None

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