1 - Infectious Diseases Unit, Department of Pediatric Medical, Hospital Dona Estefânia, Centro Hospitalar de Lisboa Central, Lisbon
2 - Infectious Diseases Unit, Department of Pediatric Medical, Hospital Dona Estefânia, Centro Hospitalar de Lisboa Central, Lisbon
3 - Pathological Anatomy Unit, Hospital São José, Centro Hospitalar de Lisboa Central, Lisbon
4 - Infectious Diseases Unit, Department of Pediatric Medical, Hospital Dona Estefânia, Centro Hospitalar de Lisboa Central, Lisbon
- JSM Pediatrics and Child Health, Clinical Image
- JSM Pediat Child Health 4: 2
Introduction: Most acquired cases of cervical lymphadenopathy have an infectious or reactive origin, but malignancy must be considered. An asymptomatic lesion that appears to be an enlarged lymph node creates a difficult dilemma for the physician.
Case Report: A 4-years-old male from Guinea-Bissau, with a three years’ history of an asymptomatic bilateral cervical mass. No preceding illnesses, fever or constitutional symptoms were recorded. Besides bulky, bilateral cervical, physical exam was otherwise normal. Ultrasonography showed an extensive, lobulated, solid, heterogeneous cervical mass that involved cervical structures without invading them. X-ray and abdominal ultrasonography were normal. Cytomegalovirus, HIV, atypical Mycobacteria and tuberculosis, toxoplasmosis and bartonellosis were excluded. VCA-IgG and EBNA antibodies for Epstein-Barr virus (EBV) were positive and VCA-IgM were negative. Lymph node biopsy demonstrated diffuse proliferation of dendritic histiocytic cells with eosinophilic cytoplasm and irregular elongated nuclei, which were immunoreactive for CD1a and S100protein, establishing the diagnosis of Langerhans cell histiocytosis (LCH). Also, DNA EBV was detected in biopsy by polymerase chain reaction.
Discussion: LCH is a rare disease characterized by a clonal proliferation of histiocytes. It affects mostly children younger than five-years-old. Cervical mass with lymph node involvement in a primary and isolated form, without cutaneous or bone involvement is extremely rare. The pathogenesis of LCH remains unclear. The etiologic association of human herpes viruses was suggested in many reports and remains debated. In our case, the presence of EBV DNA in the lymph node suggest a viral contribution to LCH pathophysiology.
Palavras Chave: cervical mass, Epstein-Barr virus, Langerhans cell histiocytosis