A series of 13 new cases of langerhans cell histiocytosis of the oral cavity: a master of disguise

Abstract

Langerhans cell histiocytosis(LCH) is a disease characterized by clonal proliferation and excessive accumulation of disseminated forms of dendritic Langerhans cells. The aims of thestudy were to describe clinico-pathological presentations of thirteen new cases of LCH of the oral cavity and to present its differential diagnoses. The majority of 61.53% (8/13) of cases occurred in infants and adolescents with female predominance (61.53%). Out of all the lesions,61.53 % occurred intraosseously;with majority 53.82% (7/13) presenting on the posterior mandible and 15.38% (2/13)in the anterior mandible.Five lesions extended to involve more than one site. Radiologically all intraosseous lesions presented as osteolytic lesions with ill-defined or well defined margins. A wide range of clinical differential diagnoses including malignancies have been proposed indicating the aggressive clinical presentations exhibited by some lesions. Histopathologically, all lesions at first glance resembled an infection/chronic inflammatory process and contained mixed inflammatory cells including eosinophils. Deep fungal infections and traumatic ulcerative granuloma with stromal eosinophilia (TUGSE) were the two main histopathological differential diagnoses that were considered for hard tissue and soft tissue lesions respectively. Round mononuclear cells, containing eosinophilic cytoplasms and peripheral indented cerebriform nuclei were only observed under higher magnification. Immunohistochemical investigations with S-100 antibody reveled nuclear and cytoplasmic positivity in mononuclear cells confirming these cells as Langerhans cells. All the patients were treated with complete surgical excision with or without chemo/radiotherapyand are under follow up care. In conclusion, as LCH of oral cavity may have a broad spectrum of clinico-pathological presentations, high degree of clinical awareness is required to arrive at the definitive diagnosis. The histopathological diagnosis is reliable with immunohistochemical confirmation of the Langerhans cell. However, awareness of its differential diagnosis is important to avoid misdiagnosis; resulting in incorrect management.

Langerhans cell histiocytosis in a pediatric patient: A typical presentation with oral manifestations

“…In about 5%–75% of patients, oral manifestations are the first signs and symptoms and sometimes the only symptom of LCH, thus, prompting the patient to seek treatment from a dentist 8 . Eden P et al, reported in a case series ( n = 13) that seven of the cases showed intra‐osseous lesion in the posterior region of the jaw and oral mucosal involvement (tongue, buccal mucosa and gingiva) 9 . Sore throat, halitosis, gingivitis, gingival hypertrophy, unpleasant taste, tooth mobility with alveolar expansion, jaw pain, facial swelling, mental nerve anesthesia, and inability of extracted tooth sockets to heal are some of the oral symptoms.…”

“… 8 Eden P et al, reported in a case series ( n = 13) that seven of the cases showed intra‐osseous lesion in the posterior region of the jaw and oral mucosal involvement (tongue, buccal mucosa and gingiva). 9 Sore throat, halitosis, gingivitis, gingival hypertrophy, unpleasant taste, tooth mobility with alveolar expansion, jaw pain, facial swelling, mental nerve anesthesia, and inability of extracted tooth sockets to heal are some of the oral symptoms. The loss of supporting alveolar bone may be mistaken for advanced periodontitis.…”

Oral Manifestations of Histiocytosis of Langerhans Cells: a Pediatric Case Involving Mandible Bone

“…The oral manifestations frequently intraosseous lesions, affection of posterior area of mandible, anterior mandible, soft tissues of buccal mucosa, gingiva and tongue (9,10). Eden P., et al, reported in a case series (n=13), 7 of the cases showed intra osseous lesion in the posterior region of the jaw and oral mucosal involvement (tongue, buccal mucosa and gingiva) (11).…”