Spontaneous Regression of Pulmonary Involvement after Smoking Reduction and Removal of and Radiation Therapy for Langerhans Cell Histiocytosis of the Sphenoid Bone: Which Comes First, the Chicken or the Egg?

Abstract

Isolated pulmonary Langerhans cell histiocytosis (LCH) in adults is known to regress spontaneously after smoking cessation alone, but little is known about whether this rationale could also apply in cases of multisystem pulmonary LCH. In particular, pediatric patients with multisystem LCH including involvement in "risk organs" such as lungs often benefit from systemic chemotherapy. Here, we present a 37-year-old man with spontaneous regression of pulmonary lesions in multisystem LCH, achieved solely by smoking reduction following local treatment of bone lesions.

Spheno-orbital Lesions—A Major Review of Nonmeningioma Causes

“…The list of our own and published lesions appears in Table 2. 2–99 Published cases of 4 of the listed diagnoses were not reviewed because there is a significant literature on these already (sickle cell crisis with sphenoid infarction, tuberculosis of orbital bone, sphenoid bone dysplasia in NF1, and fibrous dysplasia of bone). During the same period as these new cases were collected from our practices, 97 cases (21 males) of spheno-orbital meningioma were encountered by us.…”

“…We have previously reported 3 cases in young adult men whose lesions were centered on the GWS, 31 and we have also seen a young child and an adolescent with GWS disease (Figure 5). Disease centered on the GWS can be seen in younger children 29,30,[32][33][34] but is far more common in the more anterior superolateral orbit. LCH usually shows extensive bone erosion by an infiltrating soft tissue mass, with soft tissue disease in the orbit and sometimes also the middle cranial fossa and temporal fossa or beyond.…”

A case of adult multisystem Langerhans histiocytosis successfully treated by smoking cessation and radiotherapy for bone lesion

“…Moreover, an international registry study stated that despite no treatment for 31% of adult patients with MS‐LCH, the 5‐year survival rate was 91.7% 7 . Further, there are multiple reports of adult patients with MS‐LCH who have gone into remission by smoking cessation alone or by smoking cessation and local therapy 8–11 . It is controversial whether systemic treatment should be administered even in cases of MS‐LCH.…”

“… 7 Further, there are multiple reports of adult patients with MS‐LCH who have gone into remission by smoking cessation alone or by smoking cessation and local therapy. 8 , 9 , 10 , 11 It is controversial whether systemic treatment should be administered even in cases of MS‐LCH. Radiation therapy has been reported to be highly effective and safe in adult LCH, 12 , 13 , 14 performed mainly in cases with bone lesions and other soft tissue lesions (including unifocal and multifocal) with pain and other symptoms, high surgical risk, and clinical instability.…”

“…7 Further, there are multiple reports of adult patients with MS-LCH who have gone into remission by smoking cessation alone or by smoking cessation and local therapy. [8][9][10][11] It is controversial whether systemic treatment should be administered even in cases of MS-LCH.…”