Langerhans Cell Histiocytosis in an Adult: A Discussion of Epidemiology and Treatment Options

Abstract

Langerhans cell histiocytosis (LCH) is a rare disorder defined by the abnormal proliferation of Langerhans cells. While LCH can present at any age, it is classically described as a pediatric condition, and is therefore overlooked in the adult patient. Additionally, depending on tumor burden and location, LCH can manifest with a host of oral and systemic symptoms which further confuses the clinical presentation and ultimate diagnosis. The authors present a unique report of an elderly Hispanic male diagnosed with mandibular LCH who sought primary tumor excision after neoadjuvant chemotherapy. In this study, a fibula-free flap was used for subsequent reconstruction. The purpose of the study is 2-fold: to highlight the variability of LCH in both patient symptomatology and demographics, as well as the role of plastic reconstructive surgery in definitive LCH management, particularly in the setting of single system unifocal disease.

Oral manifestation as the only sign of Langerhans cell histiocytosis: A case report

“…17 For severe bone loss, free bone flaps, or microvascular-free flaps are used for jaw reconstruction. 18 As mentioned before, LCH is classified into single/ multisystem and risk organ involvement groups. 5 Based on the report of the first scintigraphy, mild osteoblastic activity was seen in the zygomatic process of the right frontal bone.…”

“…In the meantime, an interim prosthesis might be beneficial if needed 17 . For severe bone loss, free bone flaps, or microvascular‐free flaps are used for jaw reconstruction 18 …”

“… 17 For severe bone loss, free bone flaps, or microvascular‐free flaps are used for jaw reconstruction. 18 …”

Solitary Skull Langerhans Cell Histiocytosis Presenting With a Pus Draining Fistula: An Unusual Presentation and Review of Literature

“…The reported incidence of LCH is 3 to 5 per million for children and 1–2 per million for adults. Children are at risk due to thyroid abnormalities, congenital infections, and parental solvent exposure, whereas smoking appears to be the cause in adults, particularly with lung involvement [ 3 ]. The literature presents conflicting views on the etiology.…”

“…The pathophysiology is also linked with autoimmune or inflammatory processes [ 1 2 ]. It is rare in adults, with an incidence of one-two cases per million involving multiple organs like skin, bone, lung, spleen, liver, lymph nodes, thymus, and central nervous system [ 3 ]. Solitary skull lesion, although rare, is more common in children compared to adults.…”