Diagnosis Of Langerhans Cell Histiocytosis Research Articles

Langerhans cell histiocytosis in an adult patient

Aim: to present a clinical case of a rare dermatosis — Langerhans cell histiocytosis (LCH) in an adult patient.Materials and methods. A clinical and laboratory examination of a 64-year-old woman who had complained of rashes on the skin of the scalp, neck, trunk and lower extremities accompanied by itching was carried out. A histological study of skin biopsy samples from the lesion area, as well as an immunohistochemical study of Langerhans cell markers — langerin and S-100 protein — were performed.Results. Clinical manifestations of the disease, the presence of histiocytic infiltrate in the epidermis and dermis during the histological study and immunohistochemical detection of langerin infiltrate cells and S-100 protein were all consistent with the diagnosis of LCH. The therapy with methotrexate subcutaneously significantly improved the patient’s condition.Conclusion. Verification of the LCH diagnosis requires a histological study of skin biopsy samples and an immunohistochemical study of Langerhans cell markers. The efficacy of methotrexate in the treatment of this disease has been confirmed.

Tullio phenomenon and drop attacks – An unsuspected cause

Tullio phenomenon or loudness intolerance is most commonly associated with superior semicircular canal dehiscence. We describe a patient presenting solely with this phenomenon and found to have an unsuspected cause. A 43-years-old gentleman presented to us complaining of intolerance to loud noise, occasionally leading to falls, for six months. These complaints were insidious in onset and steadily progressing. Interestingly, the intolerance in heavy traffic used to subside on wearing a helmet. There was no history of hearing loss, tinnitus, earache, or ear discharge. Clinical tests of hearing including Rinne, Weber and absolute bone conduction test were within normal limits. The neurologic as well as systemic examination was normal. Pure tone audiometry (PTA) showed mild conductive hearing loss in left ear and brainstem auditory evoked response (BAER) was normal. Patient’s skull radiograph showed multiple large lytic lesions giving the appearance of “geographical skull”. Chest X-ray showed a lytic lesion with pathological fracture involving posterior part of in left sided rib. Computed Tomography of head with bone window also showed multiple lytic lesions in skull (Figure 1,2). Brain parenchyma was normal. Incisional biopsy of one of the skull lesions was performed, which was consistent with a diagnosis of Langerhans cell histiocytosis (LCH) (Figure 3). The patient showed considerable improvement after being initiated on Etoposide and Vinblastine. LCH is a rare cause of Tullio phenomenon. It appears that the loss of skull tissue due to lytic lesions leads to impairment of “modifier” function of intact skull, resulting in “volume conduction” and noise intolerance.

Langerhans Cell Histiocytosis of the Skull

This is a case of a pediatric male who presented with a postauricular mass and pain. Computed tomography and magnetic resonance imaging suggested a diagnosis of Langerhans cell histiocytosis. Characteristic imaging findings and initial workup for Langerhans cell histiocytosis are described.

Hepatic Langerhans Cell Histiocytosis (LCH) Mimicking Antimitochondrial Antibody (AMA)–Negative Primary Biliary Cholangitis (PBC), Presenting as a Harbinger of Multisystem LCH

Abstract Objectives LCH is a rare systemic disorder characterized by an infiltration of CD1a+/Langerin+ histiocytes. LCH commonly involves bone, skin, and lymph nodes in children. Hepatic involvement is rarely observed in multisystem LCH. We describe an exceptional case of hepatic LCH in an adult, mimicking AMA-negative PBC, preceding the diagnosis of multisystem LCH. Methods A 65-year-old man presented with intermittent pruritus, weakness, dyspnea, fever, and chills that have been progressive for 4 years. Physical examination was unremarkable. Laboratory work revealed elevated alkaline phosphatase (471 U/L; ref 40-120 U/L), GGT (271 U/L; ref 0-41 U/L), total bilirubin (2.0 mg/dL; ref 0.0-1.2 mg/dL), and direct bilirubin (1.1 mg/dL; ref 0.0-0.3 mg/dL). Indirect bilirubin, AST, and ALT were within normal limits. Liver biopsy was performed. Results Liver biopsy showed lobular and portal nonnecrotizing granulomas with one “florid duct lesion” with duct injury, suggestive of PBC. Tests for autoimmune diseases including AMA were negative. ERCP was negative for biliary obstruction. One month after the liver biopsy, he developed flaking, red, and burning rash on the right scalp, forehead, and epigastric skin. A skin biopsy at an outside institution revealed dermal and epidermal infiltration of CD1a-positive histiocytes with indented nuclei and pale eosinophilic cytoplasm, consistent with LCH. Subsequent reexamination of the liver biopsy showed that the histiocytes within the florid duct lesion were positive for CD1a and S-100, while the remaining granulomas were negative. The following day, a small focus of LCH was noted in his gastric biopsy performed for gastritis symptoms. Conclusion Hepatic LCH may mimic AMA-negative PBC histologically and clinically, and may present as a harbinger of multisystem LCH. While rendering the diagnosis would be challenging without prior history of LCH and with focal involvement, awareness of such presentation and communication with clinical colleagues may be helpful.

Relevance of Bone Scintigraphy in the Diagnostic Algorithm for Examining Treatment-Naive Pediatric Patients with Langerhans Cell Histiocytosis: a Retrospective Cohort Study

Background. Histiocytosis is a heterogeneous group of rare diseases of unknown etiology. Langerhans cell histiocytosis (LCH) is characterized by an abnormal proliferation of histiocytes (activated dendric cells and macrophages). LCH is the most common form of histiocytosis, it is a potentially fatal diseases. Early detection of LCH plays an important role in its prognosis and outcome. However, the role of advanced methods of nuclear medicine in diagnosis of LCH is still to be researched. We have long-term experience in observing pediatric patients with LCH. Taking into the account the difficulty of diagnostic task for bone scintigraphy in identifying lytic bone destruction, we stated the following objective of the study. Objective. Our aim was to perform retrospective analysis of bone scintigraphy examinations of treatment-naive pediatric patients with LCH and calculate the diagnostic efficacy of bone scintigraphy. Methods. We retrospectively analyzed 55 examinations of treatment-naive pediatric patients (the median age was 5.9 years) with proven case of LCH (2014–2018). Results. During examination we visualized 82 lesions with pathological level of accumulation of radiopharmaceutical in 55 patients. 78 with high level of accumulation (>120%); 3 with slightly increased level of accumulation (100–120%) and 1 with lower than normal level of accumulation (<100%). The median level of accumulation of radiopharmaceutical was 276%: max. level of accumulation was 1422% (patient with subtotal involvement of femoral bone), min. — 60% (patient with lytic destruction in orbital bone). During X-Ray we found out 91 pathological focuses of bone lytic destruction. In 3 patients with polyostotic form we found extra focuses on scintigraphy, which were confirmed with following examination and X-Ray. 8 lesions were not found on scintigraphy in 8 patients. Сonclusion. Bone scintigraphy with 99mTc-MDP in treatment-naïve pediatric patients with proven case of LCH showed sensitivity — 91.0%, specificity — 50.0%, NPV — 11.1%, and PPV — 98.8%.

Langerhans cell histiocytosis of the jaw, a mimicker of osteomyelitis on CT and MR images: A retrospective analysis.

Differential diagnosis of Langerhans Cell Histiocytosis (LCH) in the jaw is essential for early treatment including systemic therapy. Records of 17 patients (6 men and 11 women; mean age, 14 years) with histologically confirmed LCH were reviewed. All the lesions occurred in the mandible. Most of the cases (n=12) were intraosseous type LCH, only 5 patients had alveolar type LCH. Patients complained of facial swelling and pain most likely. In the 14 patients who underwent CT and/or MR imaging, all LCH lesions were osteolytic, with a mean size of 23 mm. LCH presented as expansile lesions with periosteal new bone formation, perilesional sclerosis, fluid attenuation/signal within the lesion, and inflammatory changes in adjacent soft tissues on CT/MR images. Considering the major symptoms of LCH were swelling and pain, the differential diagnosis of LCH from osteomyelitis might be more difficult. The differential diagnosis for osteolytic lesions of the jaw with surrounding inflammatory changes should include LCH, especially in young patients.

High Prevalence of BRAF V600E Mutations in Langerhans Cell Histiocytosis of Head and Neck in Chinese Patients

Langerhans cell histiocytosis (LCH) is characterized by clonal proliferation of Langerhans cells and has been classified as a hematolymphoid tumor. BRAF V600E mutation was found to be frequent in LCH; however, it has also been reported that Asia patients with LCH tend to show a lower rate of BRAF V600E mutation. In this study, we found LCH from the head and neck region often involved bone especially the posterior of the mandible and presented a high prevalence of BRAF V600E mutation in Chinese patients. Our findings also showed immunohistochemical detection correlated very well to DNA sequencing of BRAF alterations, which may be useful in the diagnosis of LCH, especially in cases with a low proportion of Langerhans cells, and BRAF inhibitors might be a treatment option for patients with LCH harboring BRAF V600E mutation.

Cytological diagnosis of Langerhans cell histiocytosis: A series of 47 cases.

Langerhans cell histiocytosis (LCH) is a rare disease affecting predominantly children and young adults but can be found in any age group. Diagnosis of LCH is often difficult and can be delayed because of its rarity. The present study highlights the cytomorphological features in a large cohort of cases. An accurate cytological diagnosis may avoid unnecessary biopsy and guide appropriate management. Fourty seven (47) cases of LCH diagnosed on cytological material & fine-needle aspiration (FNA) over a period of 14 years (2003-2016) were retrieved from the archives. The cytological smears were evaluated and microscopic findings collected by semi-quantitative assessment done by two different pathologists RESULT: The age at the diagnosis of the patients ranged from 9 months to 28 years. The majority of cases were in the age group of 0-5 years. The most common site was head and neck region, which included cervical lymphadenopathy and scalp swelling. Two cases were diagnosed each from inguinal lymph node and bronchio-alveolar lavage (BAL). Cytological smears in the majority of the cases were moderate to highly cellular (58%) and showing abundant Langerhans cell in (72%) of cases. Areas of necrosis were seen in 38%, while 78% of cases showed giant cells. The majority of cases showed mild eosinophilia (61%), sparse lymphocytosis (83%) and mild neutrophilic infiltration (64%). There were 1-2 mitoses per 10 high power field in 12 cases (25.5%). No abnormal mitoses were identified. The presence of cells with features of Langerhans cells associated with the expression of selected immunohistochemical markers allow the diagnosis of LCH on cytological samples, sparing more invasive procedure as a biopsy.

Rare Case of Adult Langerhans Cell Histiocytosis: A Case Report

Background: Langerhans Cell Histiocytosis (LCH) is a disease characterized by proliferation and infiltration of histiocytes in various organs that is caused by local or systemic effects. LCH could affect bones, lungs, central nervous system, liver, thymus, skin, and lymph nodes. The diagnosis of LCH is difficult to enforce and rarely found in adults, namely 1-2 cases per million per year.Case Presentation: In Indonesia, there were very few cases of LCH in adults, one of which was found in women aged 56 years. LCH does not yet have standard treatments. The treatment is given based on location and severity, including intralesional corticosteroid injection, radiotherapy, surgical or excision curettage, and chemotherapy. Prednisone and vinblastine chemotherapy was one of chemotherapy that is given to LCH patients. Prognosis of LCH consists of various factors, one of which is the number of organs involved. This is a case report of LCH in an adult male patient with skin involvement who received chemotherapy for 6 weeks and experienced improvement.Conclusions: LCH is not only a disease of children; it is also reported in an adult. The prognosis is related to the number of organ involved and involvement of the risk organ. LCH can recur easily and accompany malignant tumors, so follow-up and long-term observations are still needed to be done.

Langerhans cell histiocytosis presenting as eosinophilic granuloma of the bilateral forearms in an 8-year-old girl: a case report

BackgroundLangerhans cell histiocytosis previously known as histiocytosis X is a rare disease of children and young adults with a very broad clinical spectrum. In children, its annual incidence is estimated between 0.2–0.5 per 100,000.Case representationAn 8-year-old Moroccan girl with no known personal or family history presented to our institution with painful swelling of both forearms. An X-ray and magnetic resonance imaging were inconclusive. We then performed a biopsy curettage (of her left forearm). Microscopic analysis followed by immunohistochemical analysis disclosed a diagnosis of Langerhans cell histiocytosis. No chemotherapy was necessary. Clinical and radiological improvement was achieved after 6 months.ConclusionThe particularity of this observation is the bilaterality of the lesion on both forearms and it has not previously been reported. Langerhans cell histiocytosis should be included in the differential diagnosis of osteomyelitis and Ewing’s sarcoma.

Pulmonary Langerhans cell histiocytosis with Skin involvement; an amazing case report

Introduction: Langerhans cell histiocytosis (LCH) is a rare disease characterized by infiltration of large mononuclear or dendritic cells in one or more organs. Lung can be involved alone or with other organs in this complication. Case Report: We report a 33-year-old smoker man with LCH in whom both the lung and the skin were involved. He was referred to our department due to malodourous, crusted and scaly erythematous patches, plaques, and pustules involved scalp and flexural area. His medical problem started with pulmonary involvement. He experienced spontaneous pneumothorax, and was suffering progressive dyspnea for years. Cryo-transbronchial lung biopsy previously had been done. Pathology report was pulmonary LCH (PLCH). Weid skin biopsy. Histologic analysis of skin with haemotoxylin and eosin (H&E) staining and immunohistochemistry (IHC) exam with S100 and CDa1 confirmed the diagnosis of LCH. Conclusion: It is important to consider PLCH in the setting of recurrent pneumothorax and progressive dyspnea in middle-aged smoker patient. PLCH may be associated with skin involvement.

Langerhans cell histiocytosis with concurrent papillary thyroid microcarcinoma

Langerhans cell histiocytosis (LCH) is a rare disease, characterised by proliferation of Langerhans cells in various organs. It can present as localised or disseminated disease, with particular predilection for involvement of skin, bone, brain and lungs. Solitary thyroid gland involvement is exceptionally rare, with only a limited number of cases reported in the literature. Here we describe a case of a 45-year-old female with a known history of hypothyroidism, presenting with enlarged multinodular thyroid gland on clinical examination. Ultrasonography showed a 20 mm left lobe thyroid nodule in the lower pole, and pre-operative fine needle aspiration cytology assessment was classified as suspicious for malignancy (Bethesda category 5). The patient underwent left hemi-thyroidectomy with neck dissection. Microscopic examination revealed chronic lymphocytic thyroiditis with two foci of papillary thyroid microcarcinoma (7.5 mm and 0.6 mm) together with adjacent LCH spanning 3.5 mm. Although concomitant papillary thyroid carcinoma and LCH in thyroid is rare, it has recently been increasingly reported. This draws attention to possible association between the aetiology of both disorders, and necessitates thorough histological examination for concurrent papillary thyroid carcinoma and lymphocytic thyroiditis when diagnosis of LCH in thyroid gland is attained.

Langerhans cell histiocytosis in children: clinicopathological and ultrastructural analyses of 345 cases

Objective: To investigate the clinicopathological and ultrastructural characteristics of Langerhans cell histiocytosis (LCH) in children. Methods: A total of 345 cases of LCH from the Department of Pathology, Beijing Children Hospital from January 2012 to March 2016 were investigated by hematoxylin-eosin stain, EnVision immunohistochemistry and transmission electron microscopy. Results: The rate of primary clinical diagnosis of LCH in children was 46.0%(210/457). Among 345 patients of LCH, 213 were male and 132 were female, the male to female ratio was 1.6∶1.0, and the median age was 21 months (range from 2 days after birth to 13.3 years). There were total 597 lesions, including bony lesions (258, 43.2%), skin lesions (206, 34.5%) , followed by lymph node (16, 2.7%), lung (28, 4.7%), liver (25, 4.2%) and head-neck (50, 8.4%). Single organ system LCH (SS-LCH) was seen in 295 cases (85.5%) and 50 cases (14.5%) presented with multiple organ system involvement LCH (MS-LCH). There was no significant difference in age and gender between SS-LCH and MS-LCH groups. Regarding sites, more lesions were seen in bone and skin in SS-LCH group, in contrast lymph node, lung, liver and head-neck involvements were often seen in MS-LCH group. Immunohistochemically, the expression of CD1a and Langerin was seen in 99.7% (341/342) and 98.8% (338/342) of the cases respectively. The diagnostic rates by light and transmission electron microscopy were 98.8% (341/345) and 97.4% (112/115) respectively (P>0.05). Conclusions: LCH of children occurs predominantly in SS-LCH pattern, frequently involving bone, skin, lymph node, lung and liver and other sites with unique histopathological, immunophenotypical and ultrastructural features. Accurate diagnosis relies on the morphology, immunophenotype and ultrastructural features. Further refinement of specimen processing may improve the accuracy of pathological diagnosis.

Initial Symptoms of Langerhans Cell Histiocytosis: A Case Series.

Langerhans cell histiocytosis (LCH) is a rare childhood hematopoietic disease, and hence, there are few reports summarizing the course leading to the diagnosis. We described the initial symptoms and the clinical course of LCH. We carried out a retrospective review of charts from a single medical center, and 21 patients with the diagnosis of LCH were enrolled. The initial symptoms of 16 cases were caused by bone lesions; of these cases, there were 8 instances of soft tissue swelling as the initial symptom (38%) and 8 instances of bone pain without swelling (38%). Among the cases of bone lesion, 4 of 6 cases of skull lesion were painless while all vertebral body lesions and long bone lesions were accompanied by pain. LCH bone lesions caused various symptoms depending on the site of the lesion and this makes the diagnosis difficult. A detailed physical examination and imaging studies are recommended for early diagnosis.

A neonatal pustule:Langerhans cell histiocytosis

Langerhans cell histiocytosis (LCH) is a rare, clinically heterogeneous disease that most commonly occurs in pediatric populations. Congenital self-limited LCH is a benign variant of LCH. It most commonly presents as a diffuse eruption and reports of single lesion cases are infrequent in the literature. Even in the case of congenital self-limited LCH, there is potential for future multisystem relapse, making long-term follow-up important. We present a case of single lesion self-limited LCH in a full-term male infant with interesting morphology. Physical examination revealed a painless, 6 millimeter, well-demarcated, papule encircled by erythema with central hemorrhage. An infectious workup was negative and a punch biopsy was obtained, which showed a dermal infiltrate of histiocytes consistent with a diagnosis of LCH. The lesion healed without intervention within three weeks. Our case highlights the need for dermatologists to consider LCH in the differential diagnosis for lesions of varying morphology in children, as proper identification is necessary to monitor for multisystem recurrence.

Langerhans cell histiocytosis of the hip in children.

Eight year old girl was referred to our consultation for a lameness with a flessum of the left hip with fever (38.5°C). Biology showed an inflammatory syndroma (CRP: 29 mg/l). Pelvic X-ray showed an osteolysis of the roof of the left acetabulum with regular contour (A). Magnetic resonance imaging (MRI) showed tissue thinning and damage to the roof of the left acetabulum (B). A scanno-guided biopsy was performed, anatomopathological study confirmed the diagnosis of Langerhans cell histiocytosis. A discharge, with traction was done. The child has improved and biology has normalized. The radiograph of the hip after 1 year showed a filling of the gap (C). Langerha's cell histiocytosis is a proliferation of mononuclear phagocytic cells. It is also called eosinophilic granuloma. It is often located at the skull and spine. The localization at the hip represents only 8% of the cases it is especially supra-acetabular it's spontaneous evolution is favorable.

Squash cytology of langerhans cell histiocytosis of sellar region

Langerhans cell histiocytosis (LCH) is a systemic disease, which usually involves the skeleton and skin of children with occasional involvement of the central nervous system (hypothalamo–pituitary axis). We report the case of a 16-year-old male with a space-occupying lesion (SOL) in the sellar region and diabetes insipidus. However, no systemic abnormality was noted. A clinical diagnosis of pituitary adenoma was given. However, on intraoperative consultation by squash smears, cells with nuclear grooves and few eosinophils were seen. Hence, a differential diagnosis of LCH was also given, which was confirmed by histopathology and immunohistochemistry. Hence, as a matter of fact, LCH should be considered as one of the rare differential diagnoses of SOL of sellar region.

Diagnostic yield and safety of EUS-FNA biopsy in sub-aortic and para-aortic lymph node stations with the trans-aortic approach: a case series and literature review

ABSTRACTBackground: Sub-aortic (station 5) and para-aortic (station 6) lymph nodes are not easily accessible due to the interposition of the aorta and the left pulmonary artery. Taking a biopsy from those stations could be of value when there are no other mediastinal lymph node stations or when biopsy in other sites failed to reach a diagnosis. Surgery is the gold standard technique in the evaluation of those stations; endoscopic ultrasound-fine needle aspiration (EUS-FNA) has been proposed as a minimally invasive technique through the trans-aortic approach, with an acceptable diagnostic yield and safety profile.Objective: To evaluate diagnostic accuracy and safety of the trans-aortic EUS-FNA in lymph node stations 5 and 6.Methods: We reviewed all patients who underwent trans-aortic EUS-FNA from 2010 to 2017, for mediastinal lymph node enlargement or positron emission tomography/computed tomography (PET/CT) positivity (integrated 2-deoxy-2-fluoro-d-glucose). Demographic characteristics, lesion site and size, needle, final diagnosis, and complications were collected.Results: A total of 11 patients were included, 5 males, mean age 59 years. Samples were inadequate in two cases, a diagnosis of lung cancer was reached in four patients (two adenocarcinoma and two squamous cell carcinoma) and five cases were negative for malignancy (one confirmed by surgery, two were found to be cancer at percutaneous lung biopsy and transbronchial biopsy, one patient received a diagnosis of Langerhans cell histiocytosis by transbronchial lung cryobiopsy, and one patient was lost at follow-up). The sensitivity for malignancy was 57%, and the overall diagnostic accuracy was 45%. No complications occurred.Conclusions: Trans-aortic EUS-FNA could be proposed as a valuable and safe approach for taking biopsy from mediastinal lymph node stations 5 and 6.

A multicenter study of patients with multisystem Langerhans cell histiocytosis who develop secondary hemophagocytic lymphohistiocytosis.

Langerhans cell histiocytosis (LCH) is a rare myeloid neoplasm characterized by the presence of abnormal CD1a-positive (CD1a+ )/CD207+ histiocytes. Hemophagocytic lymphohistiocytosis (HLH) represents a spectrum of hyperinflammatory syndromes typified by the dysregulated activation of the innate and adaptive immune systems. Patients with LCH, particularly those with multisystem (MS) involvement, can develop severe hyperinflammation mimicking that observed in HLH. Nevertheless, to the authors' knowledge, little is known regarding the prevalence, timing, risk factors for development, and outcomes of children and young adults who develop HLH within the context of MS-LCH (hereafter referred to LCH-associated HLH). To gain further insights, the authors conducted a retrospective, multicenter study and collected data regarding all patients diagnosed with MS-LCH between 2000 and 2015. Of 384 patients with MS-LCH, 32 were reported by their primary providers to have met the diagnostic criteria for HLH, yielding an estimated 2-year cumulative incidence of 9.3% ± 1.6%. The majority of patients developed HLH at or after the diagnosis of MS-LCH, and nearly one-third (31%) had evidence of an intercurrent infection. Patient age <2 years at the time of diagnosis of LCH; female sex; LCH involvement of the liver, spleen, and hematopoietic system; and a lack of bone involvement each were found to be independently associated with an increased risk of LCH-associated HLH. Patients with MS-LCH who met the criteria for HLH had significantly poorer 5-year survival compared with patients with MS-LCH who did not meet the criteria for HLH (69% vs 97%; P<.0001). Given its inferior prognosis, further efforts are warranted to enhance the recognition and optimize the treatment of patients with LCH-associated HLH.

Surgical management of persistent pneumothorax in patient with Langerhan’s histiocytosis: case report

Langerhans cell histiocytosis (LCH) is a rare disease that involves multiple organ systems and may cause secondary pneumothorax in children. Management of luteinizing hormone (LH) involves multiple medical disciplines and primarily relies on long-term chemotherapeutic agents. Surgery, namely total pleurectomy, is rarely necessary and is described only in a few case reports, but to our knowledge, has not been described using the thoracoscopic approach in a 1-year old patient. We discuss a 1-year old child who initially presented to an outside hospital with respiratory distress and was found to have pneumothoraces, jaundice, and a rash. Bilateral chest tubes were placed and the diagnosis of LCH was secured by skin biopsy. She was started on weekly vinblastine and prednisone, but had a prolonged hospital course due to a persistent right pneumothorax and a continuous air leak for which she was transferred to our institution. Her treatment course was changed to cytarabine however her air leak persisted. Computed tomography of her chest confirmed extensive, bilateral involvement of both lungs with large cystic spaces throughout the lung parenchyma, and a sizeable pneumothorax, despite chest tube drainage. At 8 weeks of treatment, she underwent a right video-assisted thoracoscopic total pleurectomy. She was extubated on postoperative day 1. She continued to have an air leak and a small pneumothorax for several days after surgery until she achieved pleural apposition and resolution of her air leak at postoperative day 7. We maintained her chest tube to suction for another 7 days and her chest tube was removed successfully after a water seal test. She continues on her cytarabine therapy. This case describes the first report of a thoracoscopic total pleurectomy in a 1-year-old child with LCH.