Diagnosis Of Langerhans Cell Histiocytosis Research Articles

Reflectance confocal microscopy imaging of pediatric Langerhans cell histiocytosis lesions: 14 case reports

Objective To assess the reflectance confocal microscopic features of pediatric Langerhans cell histiocytosis (LCH) , and to evaluate the performance of RCM in the early diagnosis of pediatric LCH. Methods RCM was routinely performed to image lesions in 14 children with LCH before biopsy. Then, biopsy and histopathological examination were carried out at the sites determined according to RCM images. A comparison was conducted between the confocal microscopic and pathological findings. All the 14 cases were finally diagnosed based on histopathological and immunohistochemical examination results. Results RCM imaging in these patients showed that the epidermal structure was disorganized with spongiosis, infiltration of moderately refractive inflammatory cells and disappearance of normal honeycomb structures, slightly refractive cavernous or crateriform appearance was observed sometimes in the superficial epidermis and even stratum corneum; normal dermal papillary rings were obscure or absent with infiltration of multiple scattered moderately refractive inflammatory cells; plenty of bright hyper-refractive dendritic cells, which varied in size and shape, gathered into clusters in the dermis; moderately refractive inflammatory cells infiltrated the papillary and reticular dermis with the tangling and dilation of blood vessels giving a spherical or linear appearance in some regions. Histopathological examination and immunohistochemical staining for S100 and CD1a both demonstrated that the bright dendritic cells were activated Langerhans cells. RCM images were highly consistent with histopathological and immunohistochemical findings in these patients. Conclusion RCM, a non-invasive examination method, can facilitate the determination of biopsy sites, and promote the accuracy of pathological diagnosis of LCH. Key words: Histiocytosis, Langerhans-cell; Pathology; Diagnosis; Skin diseases; Child; Reflectance confocal microscopy

Langerhans Cell Histiocytosis with Frontal Bone Indentation by an Adjoining Primary Soft Tissue Lesion in a 17-Month-Old Asian Male Child.

Langerhans cell histiocytosis (LCH) is a rare disease that represents a clonal proliferation of pathologic Langerhans cells [1]. Although its clinical manifestations range from isolated bone lesions to multisystem disease, the pathological finding is uniform irrespective of the disease manifestation. Besides their characteristic morphology, Langerhans cells are further identified histochemically by the presence of the CD1a and S-100 surface markers and electro-microscopically by the presence of Birbeck granules [2]. Pathological findings are obligatory for diagnosis. LCH usually occurs in young children with a peak from 1 to 4 years [3]. Soft tissue involvement is usually the secondary extension of an adjoining primary bone lesion, while the secondary involvement of an adjacent bone by a primary soft tissue LCH lesion is rare [4]. Here, we report the case of a 17-month-old Asian male child with a solitary primary soft tissue LCH lesion in the right forehead area with secondary bone destruction by indentation. This 17-month-old male child was referred to our institution for a forehead mass that rapidly enlarged in three weeks. This child initially presented at an outside clinic, and an ultrasonographic (US) evaluation was performed. The US revealed a soft tissue mass with a diameter of 22 mm and with bone erosion and destruction on the right forehead. LCH was suspected, and the patient was referred to our institution. Initial recognition of the mass was at three weeks before admission. Physical examination revealed a 3.0×3.0 cm tender and fixed mass over the right forehead. Computed tomography (CT), fluorodeoxyglucose positron emission tomography (18F-FDG-PET)/CT, and skull radiography imaging studies were performed before open surgery (Figs. 1, ​,2).2). In December 2011, the mass was extirpated under general anesthesia. On the operative field, we observed an irregularly shaped firm mass measuring 2×3 cm, located in the subcutaneous layer with periosteal inflammation and thinning of the frontal bone without dural exposure. To reconstruct the thinning of the frontal bone, we performed an allograft with a demineralized bone matrix and an osteoconductive bone void filler. Pathologic findings showed that under high magnification (×400), the tumor cells were mostly large monocytes with abundant cytoplasm and occasionally central grooved nuclei, admixed with numerous eosinophils. The presence of Langerhans cells was confirmed by CD1a and S-100 immunohistochemistry (Figs. 3, ​,4).4). To identify whether the tumor origin was the soft tissue and the bone, we conducted a pathologic study of the adjoining periosteum. The histologic study revealed mild chronic inflammation with fibrosis; this result confirms the diagnosis of primary soft tissue LCH. After surgery, this child was followed-up at Samsung Medical Center every 6 months with a bone scan (Fig. 5), and the 1-year follow-ups were conducted at our institution. During the follow-up period of 3 years, no evidence of recurrence was found. LCH, previously known as histiocytosis X, is a histiocytic disorder of unknown etiology that is characterized by the clonal proliferation of dendritic cells in various organs and tissues. LCH has a variable clinical presentation and course, ranging from a solitary, often spontaneously resolving, bone lesion to a fatal multicentric and multiorgan system disease. LCH lesions can involve any organ in the body; however, bones, skin, and lungs are the most common sites of involvement. Soft tissue involvement is usually due to the extension of an adjoining primary bone lesion [4]. In our case, LCH with a soft tissue origin affected the adjoining bone by indentation. Before surgical exploration, the origin of the mass was difficult to identify on the basis of radiologic findings. To identify whether the tumor origin was the soft tissue or the bone, we conducted a pathologic study of the adjoining periosteum. The histologic study revealed mild chronic inflammation with fibrosis; this result confirmed the diagnosis of primary soft tissue LCH. There are multiple treatment modalities for LCH: solitary, easily accessible lesions can be surgically extirpated, and intralesional corticosteroid application and radiotherapy are also possible options [5]. However, clinical guidelines for the treatment of LCH are still under development. In our patient, timely surgical extirpation of the mass was performed and no evidence of recurrence was found during the 3-year follow-up period. Here, we presented the case of a 17-month-old child with a solitary primary soft tissue LCH lesion, which influenced the adjacent bone by indentation. Due to the rarity of LCH that involves both the soft tissue and the bone, it is difficult to identify the tumor origin. In this case, LCH with an ambiguous origin was completely resolved by surgical extirpation, and the primary soft tissue origin of the tumor was confirmed by a histologic study.

Langerhans cell histiocytosis: a comprehensive review

Langerhans cell histiocytosis (LCH) is currently regarded as a myeloid neoplasm, with remarkably broad clinical spectrum, ranging from isolated skin or bone lesions to a disseminated disease that can involve nearly any organ. LCH is generally regarded as a sporadic disease that occurs predominantly in the paediatric population. The diagnosis of LCH is confirmed by immunohistochemistry (IHC) by demonstrating the presence of dendritic cell markers such as S100 protein, in addition to CD1a and langerin. Contrary to previous beliefs, recent literature reveals that the pathogenesis of LCH might involve a clonal process implicating BRAF c.1799T>A (p.Val600Glu) and other mutations [(600DLAT) B-RAF and (T599A) B-RAF, somatic MAP2K1 mutations].Through this review article, we have summarised the latest understanding of the biological and salient histological characteristics of LCH and its potential morphological mimics.

Langerhans cell histiocytosis with multisystem involvement in an infant: A case report

Langerhans cell histiocytosis (LCH) is a proliferative disease of histiocyte-like cells, with a wide range of clinical presentations that vary from a solitary lesion to more severe multifocal or disseminated lesions. The disease can affect any age group; however, the peak incidence rate is in infants aged between 1 and 3 years-old. Diagnosis of LCH should be based on the synthetical analysis of clinical presentations, in addition to features of imaging and histopathology. Although certain cases regress spontaneously, other patients require systemic chemotherapy together with the administration of steroids. The present study reports the case of an infant with LDH with multisystem involvement, including that of the bone, skin, orbit, spleen and lungs. The patient received chemotherapy and obtained rapid improvement in the involved systems. A total of 2.5 years after completion of the therapy, the patient still remains in follow-up and no evidence of active disease has been noted.

Pulmonary Langerhans cell histiocytosis causing spontaneous bilateral pneumothorax in a child

Bilateral pneumothorax is very rare in childhood. Moreover, if it is due to pulmonary involvement of Langerhans cell histiocytosis, it is even rarer in childhood. In our case, a nonsmoker 12-year-old boy presented with bilateral pneumothorax, whose high-resolution computed tomography scan was highly suggestive of pulmonary Langerhans cell histiocytosis. Excision biopsy of a clinically palpable cervical lymph node and histopathological examination and immunohistochemistry positivity for CD1a indicated a diagnosis of Langerhans cell histiocytosis. Clinicians should consider pulmonary Langerhans cell histiocytosis in differential diagnoses in dealing such a case.

Langerhans cell histiocytosis following treatment for testicular cancer. A case report and literature review

Abstract A 35 year old male patient received treatment for testicular cancer of pure seminoma histology. He underwent initially a right inguinal orchiectomy and afterwards he received 3 cycles of BEP chemotherapy, as his imaging studies showed enlarged para-aortic lymph nodes. Five months after completion of chemotherapy treatment, a thoracic CT revealed multiple micronodular lesions in both lungs. The patient was advised about the need of salvage chemotherapy, but he opted to undergo further investigation. A lung lesion biopsy was performed, and histology was compatible with diagnosis of Langerhans cell histiocytosis (LCH). LCH is quite an unusual clinical entity that affects usually -but not solely- the lungs of young adults. The hallmark of the disease, is the presence of the activated Langerhans cells. As its clinical, laboratory and radiological findings are nonspecific, a transbronchial biopsy is sometimes necessary to establish diagnosis. Treatment is generally symptomatic and reserved for patients with multisystem organ involvement. LCH’s relationship with cancer is complex and no certain correlation is evident. More troublesome can be its differential diagnosis from a cancer with metastatic spread to the lungs or other affected organs, and although rare, it has to be kept in mind when suspicious signs arise

Adult onset of multisystem Langerhans cell histiocytosis with skin and lymph node involvement.

Langerhans cell histiocytosis (LCH) is a rare disorder characterized by clonal proliferation of dendritic cells, which comprises a wide range of clinical presentations. The term LCH is used to include a spectrum of disorders previously termed: histiocytosis X, eosinophilic granuloma, Hand-Schuller-Christian disease, Letterer-Siwe disease and Hashimoto-Pritzker disease. The etiology of the lesion is still elusive, and the question of whether LCH is a reactive or neoplastic disorder remains unanswered [1]. The LCH mainly affects children under 10 years of age, but can occur at any age. The estimated incidence in adults is about 1–2 cases per million and is lower than that in children [2]. The LCH may affect any organ, but the most common locations include bone [3–6], mucous membranes [7], skin [8], lymph nodes, lung [9], pituitary [10] and liver [11]. The clinical course and prognosis of LCH is diverse ranging from a spontaneously regressing single lesion to a life-threatening multisystem disease with rapid progression and death. This disorder may involve single or multiple organs and has an unpredictable course. Diagnosis of LCH is difficult and may be delayed due to its rarity and wide clinical manifestation.

Langerhans Cell Histiocytosis Presenting as Endocrine Disorders in an Adult Patient: A Case Report

ABSTRACT Objective: To present a case of Langerhans cell histiocytosis (LCH) involving the pituitary and thyroid that was misdiagnosed as idiopathic diabetes insipidus (DI) and Hashimoto thyroiditis. Methods: The history, clinical findings, laboratory and imaging results, management, and follow-up of the case are presented, and the endocrine involvement is discussed. Results: A 50-year-old female with a 13-year history of DI and a 5-year history of progressive goiter with hypothyroidism had been initially diagnosed with idiopathic DI and Hashimoto thyroiditis. She also had a 10-year history of diabetes mellitus (DM). A partial thyroidectomy was conducted when the goiter caused dyspnea, and the postoperative pathological results suggested LCH. After a thorough examination, the diagnosis of LCH was confirmed, and the patient was treated with chemotherapy and radiotherapy. After the treatment, she went into persistent remission. Conclusion: LCH cases mainly presenting as endocrine disorders are very rare, and the diagnosis tends to be delayed or missed due to atypical clinical manifestations. Lesions or functional disorders of the pituitary and thyroid need to be differentially diagnosed against LCH of these organs. Appropriate therapy and long-term follow-up are necessary for the management of endocrine LCH. Abbreviations: CT computed tomography DI diabetes insipidus DM diabetes mellitus LCH Langerhans cell histiocytosis MRI magnetic resonance imaging TSH thyroid-stimulating hormone

Recognition and Treatment of Concurrent Active and Neurodegenerative Langerhans Cell Histiocytosis

Langerhans cell histiocytosis (LCH) is a disorder of dendritic cell proliferation with subsequent tissue damage often requiring chemotherapy. Neurodegenerative LCH presents with neuromuscular, cognitive, and behavioral alterations typically occurring years after diagnosis of active LCH. We present a male child with a 4-year history of growth arrest, polyuria, polydipsia, recurrent otitis media, and seborrheic dermatitis. Cutaneous biopsies confirmed LCH and chemotherapy was initiated. During treatment for active LCH he developed neuropsychiatric decline. White matter changes on brain MRI were consistent with neurodegenerative LCH. Treatment was changed to cytarabine and intravenous immunoglobulin. After 1 year of therapy the patient experienced neuropsychological improvement.

Langerhans cell histiocytosis in children diagnosed by fine-needle aspiration.

Background:Langerhans cell histiocytosis (LCH) is a rare intricate pediatric neoplasm with varied clinical manifestations and multiple treatment modalities.Aim:To study the cytological features of LCH and the differential diagnoses on fine-needle aspiration (FNA).Materials and Methods:FNA was performed using a 23-gauge needle fitted to a 10 mL syringe mounted on syringe holder. LCH was diagnosed on FNA smears in seven cases confined to the head and neck region, which included three cases of lymphadenopathy, three cases of scalp swelling, and one case of orbital swelling.Results:The age of the patients ranged from 25 days to 11 years and male-to-female ratio was 1:1.3. Clinically, the diagnoses suggested were tuberculosis, inflammatory lesion, abscess, and malignancy. The cytologic findings included high cellularity, isolated Langerhans cells (LCs) with prominent nuclear indentation, grooves and abundant vacuolated cytoplasm, multinucleated giant cells, eosinophils, and lymphocytes. Areas of necrosis were noted in one case. Histopathology, along with positive S-100 immunohistochemistry, confirmed the diagnosis of LCH.Conclusions:LCH is a rare disease occurring predominantly in children and can be diagnosed with ease on FNA cytology by the presence of characteristic Langerhans cells. The S-100 positivity aids in suggesting a diagnosis of LCH.

男児の下顎骨に生じたランゲルハンス細胞組織球症の1例

Langerhans’-cell histiocytosis （LCH） is caused by monoclonal proliferation of Langerhans’ cells, a type of antigen-presenting cell, and affects multiple organs. We report a case of LCH arising in the mandible of a young boy.The patient was an 11-year-old boy who referred to our hospital because of swelling of the right cheek. Computed tomographic examination showed a destructive bony lesion extending from the right mandibular body to the mandibular angle, with resorption of the buccal and lingual cortical plate. Gadolinium-enhanced T1-weighted magnetic resonance imaging showed contrast enhancement in a large portion of the right mandibular body, with soft tissue extension. A biopsy of the lesion was performed with the patient under general anesthesia because the radiographic findings suggested malignancy. Histopathological examination revealed that the lesion was composed of eosinophilic cells and histiocytes, which were positive for S-100 and CD1a immunohistochemically. These findings led to the diagnosis of LCH. PET-CT showed no evidence of lesions in other organs or bone.Patients with unifocal LCH arising in one organ generally have a high chance of spontaneous remission and favorable outcomes. The present case spontaneously remitted 3 months after the biopsy, without additional treatment. Currently, 1 year 9 months after the biopsy, the patient has made favorable progress, with no recurrence.

Highly Elevated IL-18 Is a Reliable Predictor of Progression to Hemophagocytic Syndrome in the Patients with Langerhans Cell Histiocytosis

▪Introduction:Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of epidermal antigen-presenting cells such as dendritic cells (DCs). Historically, three distinct clinical syndromes have been described: eosinophilic granuloma (EG), Hand-Schuller-Christian disease and Letterer-Siwe disease (LSD). The disease has a broad spectrum of clinical behavior, from a mild self-limited form like EG-type LCH to an aggressive form associated with a high mortality rate like LSD-type LCH. Since one of the lethal complications of LCH is hemophagocytic syndrome (HPS), investigating a novel treatment approach for LCH associated HPS (LCH-HPS) is of paramount importance.We have reported the usefulness of cytokine profiles to differentiate HPS from various inflammatory diseases, in which macrophages/monocytes derived cytokines such as interleukin (IL)-18 and neopterin were highly elevated. Since clinical trials using neutralizing antibody to IL-18 in rheumatoid arthritis or plaque psoriasis are now underway, in this study, we examined cytokine profiles over time from six LCH cases and investigated the significance of IL-18 in the pathophysiology of HPS to establish a unique treatment strategy for LCH-HPS.Materials and Methods:The concentrations of serum IL-18 were measured in six patients with LCH, including three patients with EG-type LCH and three with LSD-type LCH. Diagnosis of LCH and HPS were based on clinical and histopathological findings. Serum IL-18 levels were measured by enzyme-linked immunosorbent assay (ELISA). Total RNA was extracted from tumor samples for analysis of IL-18 and LANGERIN mRNA expression. BRAF gene mutation was analyzed using DNA extracted from tumor cells as previously described.Results:Serum IL-18 level of LSD-type LCH was significantly higher than that of EG-type LCH (median 13,600 vs. 449, p<0.05) (Figure 1). In fact, two out of three LSD-type LCH cases developed LCH-HPS, whereas none of the cases with low level of IL-18 developed. When developed to HPS caused by various disorders, serum IL-18 concentrations became markedly increased as shown in Figure 1, suggesting that IL-18 plays a pivotal role in macrophage activation to develop HPS even in LCH. Serum concentration of IL-18 recovered to and maintained normal range after induction chemotherapy. We also observed a dominant increase of soluble tumor necrosis factor alpha-receptor type I (sTNF-RI) and type II (sTNF-RII) in LSD-LCH cases in addition to essential parameters for HPS: IL-18 and neopterin (Figure 2). This unique cytokine profile of LCH-HPS might be the cause of higher mortality rate (20%) compared to HPS caused by various disorders (5-10%).BRAF V600E mutation was detected in one of the three cases in each EG-type LCH and LSD-type LCH, suggesting that existence of BRAF V600E mutation does not correlate to the disease severity as previously reported.As recent studies demonstrated that LCH was originated from DCs, which were close to macrophages/monocytes systems, we hypothesized that LSD-type LCH tumor cells secret IL-18, and then directly initiate macrophage activation to develop HPS. To test this, we assessed IL-18 mRNA expression in tumor cells from LSD-type LCH by RT-PCR. LANGERIN mRNA expression was enhanced from all the LSD-type LCH tumor cells, however IL-18 mRNA did not seem to be derived from tumor cells, suggesting that LSD-type LCH cells themselves would trigger lethal HPS similar to lymphoma associated HPS.Conclusion:In conclusion, though LSD-type LCH tumor cells did not express IL-18 directly, the level of IL-18 was much higher in LSD-type LCH. Paying carful attention should be required to the patients with LSD-type LCH with highly elevated IL-18 to prevent the progression of lethal LCH-HPS. To hamper the macrophage activation, an application of IL-18 antibody may be clinically useful for the disease control of LCH-HPS. [Display omitted] [Display omitted] DisclosuresNo relevant conflicts of interest to declare.

Outcomes of children younger than 24 months with langerhans cell histiocytosis and bone involvement: a report from a single institution.

Langerhans cell histiocytosis (LCH) is a rare disorder that ranges from single-system to disseminated multisystem disease. Patients younger than 24 months of age more commonly present with risk organ (liver, spleen, hematopoietic system, or lung) involvement at diagnosis and have a poor prognosis. Treatment approaches have changed over the last 25 years. Our goal was to describe the course and outcomes of patients younger than 24 months of age at diagnosis and identify the role of bone involvement in outcomes. We conducted a retrospective chart review of patients diagnosed with LCH at Children's Hospital Los Angeles from 1984 to 2010, focusing on 71 patients younger than 24 months of age at diagnosis. Ten patients had single bone lesions at diagnosis and did well irrespective of therapy. The majority of patients (40/71 or 56%) had multiple bone lesions. Of the 37 patients with multisystem disease, 27 children (73%) had risk organ involvement. Fourteen patients with risk organ involvement received ≤ 6 months of initial chemotherapy with prednisone and vinblastine. Six of these patients had reactivation of the disease, and bone was the most frequent site of reactivation. Seven patients with risk organ involvement were treated with at least 12 months of chemotherapy. Only one of these patients had reactivation of the disease, and none died. The majority (7/10) of patients with risk organ involvement who progressed on therapy died despite multiple treatment regimens. Patients younger than 24 months of age at diagnosis are more likely to have multiple bone lesions than older patients, supporting that a radiographic skeletal survey at the time of LCH diagnosis is important to evaluate the extent of bone involvement. Bones were the most common site for reactivation for all patients. As expected, subjects with risk organ involvement had better outcomes when treated with systemic chemotherapy for at least 1 year. As a retrospective review of all cases at our institution over a 26-year period, this article represents level IV evidence.

Clinicopathological analysis of langerhans cell histiocytosis

The clinicopathological data of 13 patients with Langerhans cell histiocytosis (LCH) from January 2004 to January 2014 were retrospectively analyzed.There were 8 males and 5 females with a mean age of 35(6-63)years. For all 13 cases, there were 15 lesions. Histologically, there was a diffuse distribution of Langerhans cells accompanied by a variable number of eosinophils and some other inflammatory cells. Immunohistochemical study showed tumor cells were positive for S-100 protein, CD1a and Langerin. During a follow-up period of 6-101 months, all survived. The diagnosis of LCH was assisted by immunophenotypic analyses for S-100, CD1a and Langerin. Combination therapy was effective. Most patients had an excellent prognosis. Key words: Lymphoproliferative disorders; Langerhans cell

PO-0166 The Langerhans Cell Histiocytosis In Children: Study Of 11 Cases

Background and aims Langerhans cell histiocytosis (LCH) is a rare disease of unknown cause with manifestations ranging from isolated granulomatous lesions to life-threatening multi-system organ involvement. In this study we aimed to evaluate the characteristics, diagnosis, treatment modalities and prognosis of LCH. Methods We conducted a retrospective study of all cases of LCH in paediatrics department of Hedi Chaker University Hospital in Sfax during a period of 16 years (1997–2013) Epidemiologic, clinical, radiological, diagnostic and therapeutic variables were collected. Results We collected 11 cases of LCH. The average age at diagnosis was 3 years 4 months. The patients’ presenting symptoms were: exophtalmia (3 cases), polyuropolydispsic syndrome (3 cases), prolonged fever (2 cases), lymphadenopathy (5 cases). Laboratory tests showed diabetes insipidus (3 cases) and bicytopenia (1 case). The diagnosis was confirmed by histopathologic examination in all cases. Bone was the most frequently affected organ (9 cases) followed by skin (19.2%). Initially, 4 patients had single-system involvement (SS), 3 with mulisystem (MS) disease without risk organ involvement (MS-RO), and 4 multisystem disease with risk organ involvement (MS-RO). Chemotherapy based on vinblastine with corticosteroids was used in 4 patients who had MS-RO form. The outcome was favourable in 6 cases. Conclusions Langerhans cell histiocytosis is a rare and heterogeneous disease. Multisystem disease with risk organ involvement justify the use of many drugs.

English

Langerhans cell histiocytosis (LCH) is a rare proliferative disorder in which pathological Langerhans cells (LCs) accumulate in a variety of organs. This report describes a boy with Langerhans' cell histiocytosis who presented with primarily soft tissue swellings, without pain. A high index of suspicion is required to reach to a diagnosis of langerhans cell histiocytosis to prevent delay in proper management of this disease. INTRODUCTION: The childhood histiocytoses, although individually rare, varies widely in its clinical expression. These disorders are grouped together because they have in common a prominent proliferation or accumulation of cells of the monocyte-macrophage system of bone marrow origin. (1) A systematic classification of the childhood histiocytoses is based on histopathologic findings. The name Langerhans cell histiocytosis (LCH) has been applied to the class I histiocytoses. The normal Langerhans cell is an antigen-presenting cell of the skin. The hallmark of LCH in all forms is the presence of a clonal proliferation of cells of the monocyte lineage containing the characteristic electron microscopic findings of a Langerhans cell. This is the Birbeck granule, a tennis racket-shaped bilamellar granule that, when seen in the cytoplasm of lesional cells in LCH, is diagnostic of the disease. (1) CASE REPORT: A 4 year old male child presented to our pediatric outpatient department of mamata medical college with the chief complaint of multiple swellings over the forehead for the last one year. The child was apparently normal one year back when his parents noticed multiple small swellings which were gradually progressing in size. The child is a product of non-consanguineous marriage, full term, normal vaginal delivery and has a younger sister who is doing well. On examination there were three swellings over the forehead, which were subcutaneous, nontender margins ill-defined and with underlying bony defects in center of the swellings. Bilateral exophthalmos was present There was no organomegaly, generalized lymphadenopathy, skin manifestations or ear discharge.

Clinicopathologic features and prognosis of T lymphoblastic lymphoma associated with Langerhans cell histiocytosis

To study the clinicopathologic features, immunophenotype and molecular genetic changes of T lymphoblastic lymphoma (T-LBL) associated with Langerhans cell histiocytosis (LCH). Three cases of T-LBL associated with LCH were included. The morphologic characteristics were reviewed along with immunohistochemical profiling using EnVision method and TCR gene rearrangement by PCR. A review of composite lymphoma previously reported in the literature was performed. All three patients were male with the mean age of 61.7 years. One was Hans and the other 2 were Uyguers. All presented with superficial lymph node enlargement. Biopsy of lymph node showed two abnormal cell populations: distended sinus by large, pale histiocytes with nuclear grooves, and the interfollicular region containing immature-appearing cells with irregular nuclei slightly larger than that of small lymphocyte, dispersed chromatin, inconspicuous nucleoli, scant cytoplasm, and scattered mitotic figures. These cells presented in aggregates and small sheets interspersed with normal-appearing lymphocyte. The histiocytes were positive for CD1a, S-100 protein and CD68. The lymphoma cells were positive for CD3, CD7, TdT and CD34. TCR-γ gene rearrangement was detected in one case by PCR technology. One case involved bone marrow with double phenotype acute leukemia. Amongst the 8 including 5 reported cases, there were 4 males and 4 females. The mean age of the patients and the median age were 54 years. Lymphoadenopathy was the most common presentation. Bone marrow was involved in 4 cases. The time of follow-up was 2 to 27 months. The median survival was 5.5 months and the one-year survival rate was 33.3%. Diagnosis of T-LBL and LCH should be based on typical morphology, immunophenotype and molecular genetic findings, with differential diagnoses including Langerhans cell hyperplasia originated from dermatopathic lymphadenopathy. When involving lymph node, extensive sampling supplemented by immunohistochemical staining is important to reach a correct diagnosis. Although coexistent T-LBL and LCH is clonally related, the understanding of its pathogenesis requires further investigation.

Analysis of clinical, iconographical and pathological characteristics, prognosis and treatment methods for 35 cases of Langerhans cell histiocytosis

Purpose of this study was to analyse the characteristics of clinical, iconographical, pathological and treatment methods of Langerhans cell histiocytosis (LCH), so as to improve the diagnosis and treatment level of this disease. The clinical data of 35 LCH patients were studied retrospectively. These patients were divided into 2 groups according to age <14 years old and ≥ 14 years old. The clinical symptoms were analysed and the signs, imageology and pathology manifestation and treatment results were evaluated. The results showed that LCH clinical manifestations were diverse and complex. Surgical treatment for patients with single system involvement of LCH was better than that of multi-system involvement of LCH (MS-LCH). For the latter, combined chemotherapy effects was better. After 3-year follow-up, 1-year OS was 94% ± 4%, 2-years OS was 91% ± 5%, 3-year OS was 86% ± 7%. 3 years OS of group <14 years old and ≥ 14 years old was 94% ± 6% and 81% ± 10% respectively. The OS of former was better than that of the later, but because a small number of cases, this difference was not statistically significant. It is concluded that LCH is easy to be misdiagnosed, the pathological biopsy is the gold standard of LCH diagnosis. The PET-CT can be of great help in identifying stages and finding lesion areas of the disease. Pulmonary Langerhans cell histiocytosis (PLCH) is more common in adult. Combined chemotherapy can improve the prognosis of the patients. The treatment methods should be choosed according to the stage and classification of disease.

A rare disorder mimics otitis media: Langerhans cell histiocytosis of the temporal bone in a child with interstitial pulmonary fibrosis

Langerhans cell histiocytosis (LCH) is a rare disease ranging from a benign to a rapidly fatal condition affecting young children predominantly, and is characterized by an abnormal clonal proliferation of Langerhans cells. We report a case of a 3-year-old child presenting with a 1-year history of otorrhea and otorrhagia followed by a 6-month history of postauricular swelling in the right ear. Imaging demonstrated a large mass of organized tissue. A biopsy was conducted, and the diagnosis of LCH was confirmed by histopathological and immunohistochemical examination. The child was treated with a 12-month course of vinblastine chemotherapy with prednisolone. No clinical evidence of recurrence was noticed after 3years of follow-up. This rare case highlights the importance for otolaryngologists to keep LCH in mind for differential diagnosis in very young patients with symptoms and signs suggestive of acute mastoiditis or chronic otitis media.

Preliminary study on the evaluation of Langerhans cell histiocytosis using F-18-fluoro-deoxy-glucose PET/CT

Background Limited number of studies have been reported regarding the utilization of F-18-fluoro-deoxy-glucose (F-18-FDG) positron emission tomography/computed tomography (F-18-FDG PET/CT) in Langerhans cell histiocytosis (LCH). The aim of this study was to assess the role of F-18-FDG PET/CT in the diagnosis and treatment of LCH. Methods Eight newly diagnosed and seven recurrent patients with LCH received F-18-FDG PET/CT scans. The diagnosis of LCH was established by pathology, multi-modality imaging, and clinical follow-up. Results F-18-FDG PET/CT was positive in 14 patients with 13 true positives and one false positive. All 45 LCH lesions were F-18-FDG avid including six small bone lesions &lt;1.0 cm in diameter. The mean maximal standardized uptake value (SUVmax) was 7.13±4.91. F-18-FDG uptake showed no significant difference between newly diagnosed lesions vs recurrent lesions (SUVmax: 6.50±2.97 vs. 7.93±6.60, t=-0.901, P=0.376). Among 45 LCH lesions, 68.9% (31/45) were found in bones and 31.1% (14/45) in soft tissue. The most commonly involved bones were the pelvis and vertebrae. There was no significant difference in F-18-FDG uptake between bone lesions vs. non-bone lesions (SUVmax: 6.30±2.87 vs. 8.97±7.58, t=1.277, P=0.221). In two patients, changes in F-18-FDG uptake on serial PET/CT scans reflected response of lesions to treatment. Conclusions The present study suggests that F-18-FDG PET/CT may be useful for diagnosis and assessing the treatment response of LCH. Because of the small sample size, further research is warranted to confirm our findings.