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Case Reports
. 2017 May 9;11(1):130.
doi: 10.1186/s13256-017-1286-3.

Langerhans cell histiocytosis of the maxillae in a child treated only with chemotherapy: a case report

Angela Pia Cazzolla  1 , Giuseppe Troiano  2 , Khrystyna Zhurakivska  3   4 , Eugenio Maiorano  5 , Gianfranco Favia  1 , Maria Grazia Lacaita  1 , Giuseppe Marzo  6 , Franca Dicuonzo  7 , Stefano Andresciani  8 , Lorenzo Lo Muzio  2
Affiliations
Case Reports

Langerhans cell histiocytosis of the maxillae in a child treated only with chemotherapy: a case report

Angela Pia Cazzolla et al. J Med Case Rep. .

Abstract

Background: Langerhans cell histiocytosis is a sporadic disease caused by an uncontrolled pathogenic clonal proliferation of dendritic cells that have Langerhans cell characteristics. New treatment protocols provided by the HISTSOC-LCH-III (NCT00276757) trial show an improvement in the survival of children with langerhans cell histiocytosis.

Case presentation: We report a case of Langerhans cell histiocytosis, which presented as an osteolytic lesion of the left pre-maxillae enclosing the deciduous incisor and canine in a 7-month-old white Italian boy. He was treated with chemotherapy. He achieved complete remission after 7 months and after 24 months no signs of recurrence were observed.

Conclusions: As a result of this treatment, anesthetic sequelae and loss of teeth were avoided; in addition, we prevented a loss of the vertical dimension of occlusion.

Keywords: Case report; Chemotherapy treatment; Children; Langerhans cell histiocytosis.

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Figures

Fig. 1
Fig. 1
a, b Computed tomography scan showing a osteolytic bone lesion with poorly defined borders of the maxilla enclosing the deciduous incisor and canine, which resulted in the swelling of the alveolar cortical bone. c, d The lesion was composed of Langerhans cells with abundant cytoplasm and undefined cell borders, which were admixed with eosinophils and other inflammatory cells (c hematoxylin and eosin stain, original magnification ×10; d hematoxylin and eosin stain, original magnification ×20). e Immunohistochemical stain for Langerhans cell-specific CD1a antigen showing strong positive staining of neoplastic cells (original magnification ×20). f Mild positive staining for CD31 antigen (original magnification ×20)
Fig. 2
Fig. 2
Magnetic resonance imaging performed after chemotherapy, showing no sign of soft tissue involvement with complete regression of the disease
Fig. 3
Fig. 3
Computed tomography performed after chemotherapy, showing no sign of bone involvement with complete regression of the disease and a good position of the teeth involved in the neoplastic lesion
See this image and copyright information in PMC

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