An infant with self‐healing cutaneous Langerhans cell histiocytosis followed by isolated thymic relapse

Abstract

Thymic involvement with Langerhans cell histiocytosis (LCH) typically occurs in children as part of multi-system (M-S) LCH. Patients who develop skin-only LCH during infancy may either follow a self-healing course with spontaneous regression or may progress to M-S involvement. We describe a male infant who developed isolated thymic LCH after spontaneous complete regression of isolated cutaneous lesions. His erythrocyte sedimentation rate and C-reactive protein increased temporarily during the skin-only stage of LCH, and increased again considerably during the thymic relapse. Even for patients with skin-only LCH, these laboratory data might indicate possible relapse or late progression of the disease.

Multisystem langerhans cell histiocytosis with thymic involvement diagnosed with anterior mediastinal mass in a 2-month-old boy

“…Thymus gland involvement in LCH has been published in pathology and radiology case reports and in small series. It is usually part of multi-system disease and may be more common than previously recognized 3,4 . However, thymic involvement causing an anterior mediastinal mass is an extremely rare presentation of multisystem LCH 1,3,9 .…”

“…Involvement in MS LCH represents the largest group of thymic LCH. Although many MS LCH cases in the literature do not describe the histologic pattern of thymic LCH involvement, thymic The prognosis of LCH depends on the patient's age, the presence of organ involvement at the time of diagnosis, the presence and severity of organ dysfunction and involvement of high risk organs, (hematopoietic system, liver, spleen and lung) and the response to treatment during the disease course 4,8 . Involvement of the hematopoietic system, liver, spleen and lung is seen predominantly in children <2 years old.…”

“…Mediastinal involvement was almost always associated with multi-systemic disease as in our patient. Hatakeyama et al 4 reported a 2-month-old baby who was diagnosed to have skin-only LCH at the age of 6 months followed without any treatment. His cutaneous LCH gradually regressed but relapsed with isolated thymic involvement when he was 11 months old.…”

“…Although patients may present at any age, median age at presentation is 30 months 2 . Thymus involvement, extremely rare and usually part of multisystem disease, has only been mentioned in few case reports and small series [3][4][5] . Here we present a case of multisystem LCH diagnosed with an anterior mediastinal mass at a very young age who had skin involvement of LCH with spontaneous regression during newborn period.…”

Thymic langerhans cell histiocytosis in children: A case report

“…Based on these observations, the actual contribution of chemotherapy to the treatment of patients with localized LCH may appear questionable. These patients are likely to have a good response to treatment [13,14]; however, one may wonder whether they actually derive a benefit from chemotherapy or whether spontaneous regression can be expected [15].…”