Abstract
Oral manifestations may be the first sign of Langerhans Cell Histiocytosis. Due to diversity in the clinical findings of LCH, this disease has been a diagnostic dilemma to the clinicians, dentists and pathologist, as well. The pediatrician plays a crucial role in the diagnosis and multidisciplinary approach. This paper reports a case of LCH involving the mandible in a 2-year-old girl, highlight the difficulties involved in LCH diagnosis and the utmost importance of a multidisciplinary treatment approach involving pediatricians and pediatric dentists. A 2-year-old girl was referred to the Stomatology Department for evaluation of a lesion in her mandible. Her mother reported that when the baby was 7 months old, the mother noticed the presence of a small red nodule in the baby’s mandible. Clinical examination revealed markedly reddened and swollen gingiva around mandibular primary incisors, which was painless to palpation presenting slight bleeding. The child has no other health problem, without allergies and familial history of tumors. Our initial diagnosis hypotheses were Melanotic Neuroectodermal Tumor of infancy and Central Giant Cell Granuloma. The treatment consisted of surgical removal of lesion under general anesthesia. The diagnosis of Langerhans cell histiocytosis was confirmed. The patient is currently under regular review and there has been no evidence of recurrence after 3 years of surgery.
1
Introduction
Langerhans cell histiocytosis, a group of rare histiocytic disorders found in infant and children, is characterized by tissue infiltration with dendritic cells . Despite affecting any age group, the incidence of LCH is higher in children compared to adults, with a peak incidence of 2–4 years with a male predilection, twice that of the females .
Although some authors have stated that adults and older children present single system disease frequently affecting bone, it has been observed that young babies presented multisystem involvement including skin rash, otitis media, fever, organomegaly, anemia, pathological fracture of involved bone, diabetes insipidus and pain .
According to the literature, oral manifestations are highly variable and may be the first sign of langerhans cell histiocytosis . Due to diversity in the clinical findings of LCH, this disease has been a diagnostic dilemma to the pediatricians, dentists and pathologist, as well. The pediatrician plays a crucial role in the diagnosis and multidisciplinary approach. Hence, this paper presents a case and treatment of LCH involving the mandible in a child.
2
Clinical case
A 2-year-old white girl was referred to the Stomatology Department of São Leopoldo Mandic Dental School, Campinas, São Paulo, Brazil for evaluation of a lesion in her mandible. Her mother reported that when the baby was 7 months old, the mother noticed the presence of a small red nodule in the baby’s mandible.
Clinical examination revealed markedly reddened and swollen gingiva around mandibular primary incisors ( Fig. 1 ), which were soft and painless to palpation presenting slight bleeding. The child has no other health problem, without allergies and familial history of tumors. She was born at proper date of birth by normal delivery.
Magnetic Resonance Image (MRI) axial unenhanced T1-weighted image shows an expansive lesion centered on the bone marrow of the mandible, with a wide zone of transition, expansile growth pattern, discontinuity of the anterior aspect of the cortical bone, characterized by homogeneous isosignal in T1 sequence. The set of findings favors moderated aggressiveness ( Fig. 2 ).
Our initial diagnosis hypotheses were Melanotic neuroectodermal tumor of infancy and Central Giant Cell Granuloma which treatment consisted of surgical removal of lesion under general anesthesia. The surgery was performed on a single occasion and due to the child’s low age and little size of the tumor, we performed curettage until an area without neoplasia was obtained. Mandibular arch was preserved and two incisor teeth involved by the tumor were resected ( Fig. 3 ).
The histopathology features revealed infiltration of Langerhans cell histiocytosis with histiocyte-like cells and eosinophils; large cells with characteristically lobulated and indented nuclei. In addition, immunohistochemical analysis of the specimen shows that tumor cells are positive for CD1a ( Fig. 4 ). Based on these findings, the diagnosis of Langerhans cell histiocytosis was at last, confirmed. A bone scintigraphy presented normal features and there were no abnormal lesions in other areas of the body ( Fig. 5 ). The patient is currently under regular review and there has been no evidence of recurrence. The follow-up after 3 years of surgery shows absence of tumor in the clinical ( Fig. 6 ) and imaging exams.
