Oral Langerhans cell histiocytosis

TY - JOUR

T1 - Oral Langerhans cell histiocytosis

AU - Bartnick, Arnd

AU - Friedrich, Reinhard E

AU - Roeser, Kerstin

AU - Schmelzle, Rainer

N1 - Copyright 2002 Published by Elsevier Science Ltd on behalf of European Associaton for Cranio-Maxillofacial Surgery.

PY - 2002/4/1

Y1 - 2002/4/1

N2 - AIM: Our aim is to compare the different methods of treatment available for Langerhans cell histiocytosis (LCH) in the oral and maxillo-facial region. A classification based on clinical and immunohistochemical data is proposed and a grading for the severity of the disease is proposed.PATIENTS AND METHODS: Records and clinical data of 12 patients were evaluated retrospectively. The patients' ages ranged from 20 months to 47 years. Nine had manifestations in the maxillo-facial region, of which six had a single oral lesion only, with the three remaining patients having multiple lesions in this region. Three patients had lesions in the upper thorax in addition to their oral lesions. Eleven patients were treated surgically with one having been treated previously with steroid-therapy. One patient had already been treated with chemotherapy. The longest follow-up was 16 years. Immunohistochemical stains for antigen-CD-1a, antigen-S-100 and antigen-Ki-67 were performed. A proposal for staging the lesions in the oro-maxillo-facial region was made. The intensity of the antigen-Ki-67 stains was evaluated.RESULTS: Eleven patients exhibited no signs of recurrence whilst only one patient had a recurrence implying that surgical treatment was very effective in eradication and cure of the disorder. It is felt that the number of antigen-Ki-67 positive cells is related to the activity of the disease.CONCLUSION: Langerhans cell histiocytosis should be treated surgically. Only in very severe cases should surgical treatment be complemented with either radio-therapy or chemotherapy. Especially in disseminated cases, chemotherapy would appear to improve the outcome. The antigen Ki-67 as proliferation marker is suggested as a grading parameter of this disease.

AB - AIM: Our aim is to compare the different methods of treatment available for Langerhans cell histiocytosis (LCH) in the oral and maxillo-facial region. A classification based on clinical and immunohistochemical data is proposed and a grading for the severity of the disease is proposed.PATIENTS AND METHODS: Records and clinical data of 12 patients were evaluated retrospectively. The patients' ages ranged from 20 months to 47 years. Nine had manifestations in the maxillo-facial region, of which six had a single oral lesion only, with the three remaining patients having multiple lesions in this region. Three patients had lesions in the upper thorax in addition to their oral lesions. Eleven patients were treated surgically with one having been treated previously with steroid-therapy. One patient had already been treated with chemotherapy. The longest follow-up was 16 years. Immunohistochemical stains for antigen-CD-1a, antigen-S-100 and antigen-Ki-67 were performed. A proposal for staging the lesions in the oro-maxillo-facial region was made. The intensity of the antigen-Ki-67 stains was evaluated.RESULTS: Eleven patients exhibited no signs of recurrence whilst only one patient had a recurrence implying that surgical treatment was very effective in eradication and cure of the disorder. It is felt that the number of antigen-Ki-67 positive cells is related to the activity of the disease.CONCLUSION: Langerhans cell histiocytosis should be treated surgically. Only in very severe cases should surgical treatment be complemented with either radio-therapy or chemotherapy. Especially in disseminated cases, chemotherapy would appear to improve the outcome. The antigen Ki-67 as proliferation marker is suggested as a grading parameter of this disease.

KW - Adolescent

KW - Adult

KW - Child

KW - Child, Preschool

KW - Female

KW - Histiocytosis, Langerhans-Cell

KW - Humans

KW - Immunohistochemistry

KW - Infant

KW - Jaw Diseases

KW - Ki-67 Antigen

KW - Male

KW - Middle Aged

KW - Retrospective Studies

U2 - 10.1054/jcms.2001.0271

DO - 10.1054/jcms.2001.0271

M3 - SCORING: Journal article

C2 - 12069511

VL - 30

SP - 91

EP - 96

JO - J CRANIO MAXILL SURG

JF - J CRANIO MAXILL SURG

SN - 1010-5182

IS - 2

ER -