Hybrid neurofibroma/schwannoma is overrepresented among schwannomatosis and neurofibromatosis patients.
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Hybrid neurofibroma/schwannoma is overrepresented among schwannomatosis and neurofibromatosis patients. / Harder, Anja; Wesemann, Martin; Hagel, Christian; Schittenhelm, Jens; Fischer, Susan; Tatagiba, Marcos; Nagel, Christoph; Jeibmann, Astrid; Bohring, Axel; Mautner, Viktor Felix; Paulus, Werner.
In: AM J SURG PATHOL, Vol. 36, No. 5, 5, 2012, p. 702-709.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - Hybrid neurofibroma/schwannoma is overrepresented among schwannomatosis and neurofibromatosis patients.
AU - Harder, Anja
AU - Wesemann, Martin
AU - Hagel, Christian
AU - Schittenhelm, Jens
AU - Fischer, Susan
AU - Tatagiba, Marcos
AU - Nagel, Christoph
AU - Jeibmann, Astrid
AU - Bohring, Axel
AU - Mautner, Viktor Felix
AU - Paulus, Werner
PY - 2012
Y1 - 2012
N2 - We analyzed the histologic features of peripheral nerve sheath tumors occurring in 14 patients with schwannomatosis. Among a total of 31 tumors, 19 tumors (61%) showed schwannoma-like nodules within a neurofibroma-like tumor, corresponding to hybrid neurofibroma/schwannoma. At least 1 hybrid tumor occurred in 10 of 14 (71%) schwannomatosis patients. We then retrieved cases of hybrid tumors without documented relation to schwannomatosis from our database and identified 41 tumors arising in 23 patients. More than half of these patients (14/23) were reported to suffer from multiple peripheral nerve sheath tumors, favoring a tumor syndrome. Indeed, analysis of clinical records revealed the diagnosis of neurofibromatosis type 2 (NF2) in 26% (6/23), neurofibromatosis type 1 (NF1) in 9% (2/23), definite schwannomatosis in 4% (1/23), and possible schwannomatosis in 13% (3/23) of patients with multiple nerve sheath tumors. Our findings suggest that hybrid neurofibroma/schwannoma represents a common tumor type in schwannomatosis and shows a striking association with neurofibromatoses.
AB - We analyzed the histologic features of peripheral nerve sheath tumors occurring in 14 patients with schwannomatosis. Among a total of 31 tumors, 19 tumors (61%) showed schwannoma-like nodules within a neurofibroma-like tumor, corresponding to hybrid neurofibroma/schwannoma. At least 1 hybrid tumor occurred in 10 of 14 (71%) schwannomatosis patients. We then retrieved cases of hybrid tumors without documented relation to schwannomatosis from our database and identified 41 tumors arising in 23 patients. More than half of these patients (14/23) were reported to suffer from multiple peripheral nerve sheath tumors, favoring a tumor syndrome. Indeed, analysis of clinical records revealed the diagnosis of neurofibromatosis type 2 (NF2) in 26% (6/23), neurofibromatosis type 1 (NF1) in 9% (2/23), definite schwannomatosis in 4% (1/23), and possible schwannomatosis in 13% (3/23) of patients with multiple nerve sheath tumors. Our findings suggest that hybrid neurofibroma/schwannoma represents a common tumor type in schwannomatosis and shows a striking association with neurofibromatoses.
KW - Adult
KW - Humans
KW - Male
KW - Female
KW - Middle Aged
KW - Immunohistochemistry
KW - Retrospective Studies
KW - Tumor Markers, Biological/metabolism
KW - Neurilemmoma/metabolism/pathology
KW - Neurofibroma/metabolism/pathology
KW - Neurofibromatoses/metabolism/pathology
KW - Skin Neoplasms/metabolism/pathology
KW - Adult
KW - Humans
KW - Male
KW - Female
KW - Middle Aged
KW - Immunohistochemistry
KW - Retrospective Studies
KW - Tumor Markers, Biological/metabolism
KW - Neurilemmoma/metabolism/pathology
KW - Neurofibroma/metabolism/pathology
KW - Neurofibromatoses/metabolism/pathology
KW - Skin Neoplasms/metabolism/pathology
M3 - SCORING: Journal article
VL - 36
SP - 702
EP - 709
JO - AM J SURG PATHOL
JF - AM J SURG PATHOL
SN - 0147-5185
IS - 5
M1 - 5
ER -
