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Desmoid-type fibromatoses involving the brachial plexus: treatment options and assessment of c-KIT mutational status.
- Source :
-
Journal of neurosurgery [J Neurosurg] 2006 May; Vol. 104 (5), pp. 749-56. - Publication Year :
- 2006
-
Abstract
- Object: Desmoid-type fibromatoses are a locally invasive soft-tissue lesion that is most commonly encountered in abdominal sites. The tumor also affects head and neck areas, particularly the supraclavicular region, where it may encase and distort the brachial plexus and compromise neurovascular structures. Neurosurgeons may be called on to treat desmoid-type fibromatoses in these sites. The authors describe their experience in treating four patients with desmoid-type fibromatoses involving the brachial plexus and report the results of immunohistochemical analysis of the tumors.<br />Methods: Gross-total excision with nerve sparing was the first-line therapy of choice, although the surgery was challenging. Intraoperative identification of the site of tumor origin from musculoaponeurotic tissues by the neurosurgeon was necessary in two of the four cases to achieve a correct frozen section or final pathological diagnosis. Immunostaining for c-KIT (CD117) was undertaken in all cases in light of a previous report of positive CD117 immunoreactivity in abdominal desmoid-type fibromatoses. All four tumors manifested weak focal immunostaining for c-KIT. One of the patients was given adjuvant imatinib mesylate therapy, with limited success. Subsequent polymerase chain reaction testing revealed that three of the four tumors manifested a single base pair change in exon 10 of the c-KIT gene (A to C in two cases and A to G in one case). There was local recurrence in three patients, despite gross-total excision. With the combination of surgery and radiation therapy, local disease control was achieved in three of the four patients.<br />Conclusions: This represents the first report of c-KIT sequencing in desmoid-type fibromatoses and suggests a possible biological basis for continuing to explore the use of adjuvant imatinib mesylate therapy.
- Subjects :
- Adult
Base Pair Mismatch genetics
Benzamides
Brachial Plexus pathology
Brachial Plexus surgery
Brachial Plexus Neuropathies diagnosis
Brachial Plexus Neuropathies drug therapy
Brachial Plexus Neuropathies genetics
Chemotherapy, Adjuvant
Combined Modality Therapy
Exons genetics
Female
Fibromatosis, Aggressive diagnosis
Fibromatosis, Aggressive drug therapy
Fibromatosis, Aggressive genetics
Humans
Imatinib Mesylate
Male
Middle Aged
Neoplasm Recurrence, Local diagnosis
Neoplasm Recurrence, Local genetics
Neoplasm Recurrence, Local surgery
Peripheral Nervous System Neoplasms diagnosis
Peripheral Nervous System Neoplasms drug therapy
Peripheral Nervous System Neoplasms genetics
Piperazines therapeutic use
Polymerase Chain Reaction
Pyrimidines therapeutic use
Reoperation
Brachial Plexus Neuropathies surgery
DNA Mutational Analysis
Fibromatosis, Aggressive surgery
Peripheral Nervous System Neoplasms surgery
Proto-Oncogene Proteins c-kit genetics
Subjects
Details
- Language :
- English
- ISSN :
- 0022-3085
- Volume :
- 104
- Issue :
- 5
- Database :
- MEDLINE
- Journal :
- Journal of neurosurgery
- Publication Type :
- Academic Journal
- Accession number :
- 16703880
- Full Text :
- https://doi.org/10.3171/jns.2006.104.5.749