Pulmonary Langerhans cell histiocytosis in children: A spectrum of radiologic findings

Bano, Shahina; Chaudhary, Vikas; Narula, MK; Anand, Rama; Bhuvaneswari, V.; Mandal, Shramana; Majumdar, Kaushik

doi:10.1016/j.ejrad.2013.04.044

Cited by 34 publications

(18 citation statements)

References 37 publications

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“…The poor outcome of the PLCH is related with extremes age, prolonged worse conditions, multiorgan involvement, reduced diffusion capacity, extensive cysts and honeycombing on imaging, recurrent pneumothoraces, pulmonary hypertension, and prolonged treatment. 16 Death may occur due to pulmonary hypertension and/or severe respiratory insufficiency. Our patient had most of the poor prognostic factors and died due to severe pulmonary insufficiency and ECMO complications.…”

Section: Discussionmentioning

confidence: 99%

Bilateral Pulmonary Langerhans's Cell Histiocytosis is Surgical Challenge in Children: A Case Report

Soyer

Özyüksel

Boybeyi

et al. 2019

European J Pediatr Surg Rep

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Background Pulmonary Langerhans's cell histiocytosis (PLCH) is a rare cause of interstitial lung disease in children and more than half of the cases are bilateral. Persistent respiratory distress due to spontaneous pneumothorax (SP) in bilateral PLCH may refractory to conservative treatment and posed a great challenge to surgical modalities. A 3-year-old boy with SP due to bilateral PLCH is presented to discuss the surgical options of recurrent and refractory PLCH cases in children. Case Report The patient was admitted to the emergency department with severe respiratory distress and SP. After chest tube insertion, biopsy from neck mass revealed Langerhans's cell histiocytosis. Chemotherapy including vinblastine and prednisone was initiated. Due to persistent respiratory difficulty and air leaks, talc pleurodesis and thoracoscopic bullae excision with pleural decortication were performed. Two months after the admission, due to nosocomial infection and severe respiratory distress, extracorporeal membranous oxygenation (ECMO) support was initiated. The patient was died of ECMO complications on 24th day of ECMO. Conclusion Despite the use of chemotherapy and surgical excision of cystic lesions, bilateral PLCH in children may have lethal outcome. Other treatment options including respiratory support with ECMO and lung transplantation should be considered as last resort of treatment alternative in persistent cases.

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Section: Discussionmentioning

confidence: 99%

Bilateral Pulmonary Langerhans's Cell Histiocytosis is Surgical Challenge in Children: A Case Report

Soyer

Özyüksel

Boybeyi

et al. 2019

European J Pediatr Surg Rep

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“…Such a distribution pattern is atypical for PLCH, whereas it is typical in other cystic lung diseases such as Lymphangioleiomyomatosis (LAM) or Birt-HoggDubé syndrome [7,9,10]. Notably, involvement of costo-phrenic sulci has been reported in children with pulmonary PLCH [8,11]. Hence, we might hypothesize infantile onset of asymptomatic PLCH in the reported case.…”

Section: Discussionmentioning

confidence: 71%

“…HRCT features of PLCH consist of a combination of centrilobular ill-defined nodules (2-10 mm in diameter) and cysts of variable shape and size (usually <10 mm) [7,8]. In advanced stages of PLCH, cysts may enlarge or coalesce resulting into bizarre-shaped cystic lesions [7].…”

Section: Discussionmentioning

confidence: 99%

Unusual Findings of Langerhans Cell Histocytosis in a Young Asymptomatic Patient: Case Report

N¹

2014

J Pulm Respir Med

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Cyst is described on high-resolution computed tomography (HRCT) as focal round area of parenchymal hyperlucency with a well-defined interface with normal lung. A relatively small subset of lung diseases should be suspected when cystic pattern is seen on HRCT. In particular, the differential diagnosis of this pattern relies upon features such as: cranio-caudal distribution, cystic shape, and coexisting pulmonary or extra-pulmonary findings.Pulmonary Langerhans Cell Histocytosis (PLCH) is a smoke-related disease associated with cystic pattern. PLCH is characterized by infiltration of Langerhans cells in pulmonary interstitium with varying pathologic findings and depend on disease stage. The findings usually consist of a heterogeneous combination of lung nodules and cysts with upper lobes predominance. Notably, the HRCT pattern of PLCH is often striking and usually suggests confident diagnosis. Nevertheless, cases with atypical features can be seen.This case report describes the ambiguous imaging findings of a biopsy-proven PLCH. Case ReportA 33 years-old smoker male presented at the emergency department with right abdominal pain. A heterogeneous left renal mass was reported at ultrasonographic examination of the abdomen. The left incidental finding was therefore investigated by both Magnetic Resonance Imaging (MRI) and CT of the abdomen (Figure 1). The MRI and CT highlighted fatty areas within the left renal mass. Besides, the upper CT images revealed the presence of bilateral basal pulmonary cysts.

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“…De façon intéressante, il a été montré que les adolescents qui développent une atteinte pulmonaire au cours de l'évolution d'une HL extra-pulmonaire de l'enfance sont plus fréquemment fumeurs [30]. La TDM-HR est importante pour le diagnostic et permet de préciser l'étendue des lésions, qui ont souvent une prédominance basale chez ces jeunes patients [31,32].…”

Section: Histopathologie Pulmonaireunclassified

Manifestations pulmonaires de l’histiocytose langerhansienne

Obert

Tazi

2015

Revue des Maladies Respiratoires

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Pulmonary Langerhans cell histiocytosis is a rare diffuse cystic interstitial pneumonia of unknown etiology that occurs selectively in young smokers of both genders. The multicenter studies conducted by the reference center have better defined the short and medium terms natural history of the disease and the clinical management of patients. A substantial proportion of patients experience a dramatic decline in their lung function soon after diagnosis. Importantly, smoking cessation is associated with a decreased risk of subsequent deterioration. Cladribine, a purine analogue, chemotherapy may dramatically improve lung function in patients with progressive pulmonary Langerhans cell histiocytosis, but this treatment should be used only in the setting of clinical research. Specific pulmonary hypertension therapies (anti-endothelin receptors, inhibitors of phosphodiesterases) may be used with caution in specialized centres for patients with severe pulmonary hypertension, and seem to be well tolerated. The recent identification of the V600E mutation of the BRAF oncogene in approximately half of the Langerhans cell histiocytosis lesions, including pulmonary granulomas, represents an important step forward in the understanding of the pathogenesis of Langerhans cell histiocytosis. Potentially it opens the way to targeted therapies.

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Pulmonary Langerhans cell histiocytosis in children: A spectrum of radiologic findings

Cited by 34 publications

References 37 publications

Bilateral Pulmonary Langerhans's Cell Histiocytosis is Surgical Challenge in Children: A Case Report

Bilateral Pulmonary Langerhans's Cell Histiocytosis is Surgical Challenge in Children: A Case Report

Unusual Findings of Langerhans Cell Histocytosis in a Young Asymptomatic Patient: Case Report

Manifestations pulmonaires de l’histiocytose langerhansienne

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