CHEST Guidelines-Anterior-Mediastinal-Bone-Eroding-Mass-With-Dissem

Anterior-Mediastinal-Bone-Eroding-Mass-With-Dissem

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A 4-year-old boy was evaluated for nontender, mobile lymph nodes in his neck, initially treated without success for presumed acute lymphadenitis. Despite having no fever or other systemic symptoms, his history included three pneumonia episodes. Physical examination revealed a heart murmur, and imaging showed significant mediastinal widening and diffuse lung nodules. A detailed CT scan uncovered massive adenopathy in the neck and chest with a thymic mass impacting major veins and eroding bone. The lung was affected with nodular lesions consistent with hematogenous distribution.<br /><br />Histopathological examination of biopsy samples revealed a mixed cellular infiltrate prominently featuring Langerhans cells, identified by immunohistochemical markers CD1a, S100, and others. These cells, along with eosinophils and other inflammatory cells, confirmed the diagnosis of Langerhans cell histiocytosis (LCH), particularly multisystem LCH, a rare disorder characterized by the clonal proliferation of Langerhans cells. The boy's scalloping bone lesions and disseminated lung lesions reinforced this diagnosis, challenging due to its rare bone-eroding anterior mediastinal mass presentation.<br /><br />LCH is a condition more predominant in children, notably males, and can affect diverse body systems, leading to a variety of clinical presentations, from localized lesions to systemic manifestations. Its course varies, ranging from isolated bone lesions, often resolving spontaneously, to complex multisystem involvement, which necessitates intervention due to severe complications. The highlighted case required a combination of chemotherapy agents: vincristine, prednisolone, and 6-mercaptopurine, resulting in marked improvement. Early treatment response is critical for prognosis, particularly in multisystem LCH, which possesses substantial mortality risk. The case underscores the importance of comprehensive diagnostics, utilizing a multidisciplinary approach, for uncommon pediatric conditions such as LCH.

Keywords

Langerhans cell histiocytosis

multisystem LCH

pediatric

lymph nodes

thymic mass

chemotherapy

vincristine

immunohistochemical markers

bone lesions

mediastinal widening