Fibrosarcoma of the infratemporal fossa in an 8-year-old girl.
The consultants agree that an open biopsy is generally necessary to establish the histology of a pediatric head and neck neoplasm. Although a frozen section may be useful to ascertain whether tumor tissue has been sampled, definitive therapy should be based only on the histopathologic interpretation of the permanent specimen. The consultants also agree that certain studies should be obtained prior to a biopsy. Dr. Grundfast recommends a chest x-ray, liver function tests, complete blood count, and an magnetic resonance scan. Dr. Healy would obtain additional CT cuts and an MRI scan. Dr. Richardson prefers coronal cuts on the CT scan and an MRI. The skull base is regarded as the area that might pose the greatest difficulty in obtaining a surgical margin. In addition, Dr. Healy states that eustachian tube involvement would compromise the resection. Although all consultants agree that the facial nerve should be sacrificed, they disagree as to how to reconstruct this defect. Dr. Grundfast would restore the mandibular profile with a prosthesis and reconstruct the soft tissue defect with a myocutaneous flap. Dr. Healy would use an iliac bone graft for the skull base defect and would replace soft tissue with a rectus free flap or a myocutaneous flap. Dr. Richardson favors a latissimus dorsi free flap. Because the survival rates for poorly differentiated fibrosarcomas of the head and neck are so low, the experts recommend adjunctive chemotherapy. Drs. Grundfast and Richardson would also advise radiotherapy. Dr. Healy feels that the morbidity of radiotherapy is too high and would use it only in cases of positive margins, parameningeal involvement, or perineural invasion.
Grundfast
K
K
Department of Otolaryngology-Head and Neck Surgery, Georgetown University.
Healy
G
G
Richardson
M
M
D001706:Biopsy; D002648:Child; D003131:Combined Modality Therapy; D005260:Female; D005354:Fibrosarcoma / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D006801:Humans; D011379:Prognosis; D012888:Skull Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy