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Hyaluronan in intra‐operative edema of NF1‐associated neurofibromas
Author(s) -
Hagel Christian,
Behrens Timo,
Prehm Peter,
Schnabel Claudia,
Glatzel Markus,
Friedrich Reinhard E.
Publication year - 2012
Publication title -
neuropathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.701
H-Index - 61
eISSN - 1440-1789
pISSN - 0919-6544
DOI - 10.1111/j.1440-1789.2011.01276.x
Subject(s) - swelling , neurofibroma , neurofibromatosis , glycosaminoglycan , hyaluronic acid , schwann cell , edema , pathology , medicine , anatomy , surgery
The tumor suppressor disorder neurofibromatosis type 1 (NF1) is associated with development of multiple neurofibromas which may grow intraneurally as plexiform neurofibromas (PNF) or intracutaneously (CNF). Upon surgery neurofibromas may show prominent swelling hindering skin‐edge approximation. To assess whether the water binding glycosaminoglycan hyaluronan is involved in intra‐operative swelling, 51 neurofibromas from 33 NF1‐patients were investigated. Hyaluronan was histologically demonstrated and was quantified by ELISA. Molecular weight of hyaluronan was determined by gel filtration. Further, hyaluronan content was measured in cultivated Schwann cells and fibroblasts. Clinically, 67% of PNF were associated with moderate or severe intra‐operative swelling, whereas only 36% of CNF showed this feature. Significantly higher levels of hyaluronan content were found in PNF compared to CNF ( P < 0.05). Mast cell density did not correlate with any of the parameters. Molecular weight of hyaluronan in PNF and CNF ranged from higher than 10 6 Da to approximately 10 5 Da. Fibroblasts produced less hyaluronan than Schwann cells. The findings support the view that hyaluronan plays an important role in intra‐operative swelling in neurofibroma surgery.