Multisystem Langerhans cell histiocytosis coexisting with metastasizing adenocarcinoma of the lung: A case report (original) (raw)
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Langerhans' cell histiocytosis mimicking metastatic carcinoma of the lung.
Langerhans' cell histiocytosis (LCH), previously known as Histiocytosis X, is characterized by abnormal accumulations of large mononuclear cells forming granulomas in various organs, mainly bone, skin and lung. This case report describes a 50-year-old man with a history of left pneumonectomy due to squamous cell carcinoma (SCC). During routine follow up, a CXR showed new parenchymal nodules in the right lung 57 months post treatment. Chest CT scan confirmed the presence of multiple parenchymal nodules. Open lung biopsy from the right upper lobe was performed and LCH was diagnosed. Re-analysis of the initial pneumonectomy specimen revealed no evidence of LCH in the surrounding lung tissue. On diagnosis, the patient stopped smoking and was treated with vinblastine and prednisolone for LCH. The nodules disappeared and have not returned in a further 18 months of follow up. In this patient LCH was diagnosed after SCC, which highlights that smoking-related diseases can be seen concomitantly or sequentially in the same patient.
LCH with Multisystemic Involvement-A Case Report
Langerhans cell histiocytosis (LCH) constitutes a rare group of disorder derived from the macrophages and dendritic cells. The cells in LCH show close similarity to the langerhans cells found in the skin and mucosa and stain positive for CD1a, S100, and CD207 antigens. These cells display the same intracytoplasmic organelles on electron microscopy as seen in Langerhans cells i.e. Birbeck granules. LCH is ten times more prevalent in the paediatric age group as compared to adult population. Females are affected more commonly as compared to the males. LCH can have single-or multi-system types of involvement. Unifocal or multifocal pattern is seen in singlesystem type of disease. The multisystem pattern is often seen in the paediatric population while unisystem pattern is seen in adults. We present case report of a 35 yrs old male patient with multisystemic involvement. A 35yr old male patient presented to our tertiary care hospital with complaints of pain abdomen, jaundice and a large swelling over the head. Plain radiographs of the head, chest and pelvis were done. Multiple punched out lytic calvarial lesions having bevelled margins were seen on skull radiographs. A large craniectomy defect was also noted on the left side of skull on the plain x-ray suggesting post-operative status (Figure 1 A-B). Plain radiograph of the chest showed diffuse reticular opacities in bilateral lungs with sparing of the bilateral costophrenic angles and well-defined lytic lesion with thin sclerotic rim was seen in left iliac bone, another small lytic lesion was seen in inferior part of right iliac bone, close to the right SI joint in the plain X-ray of the hip (Figure 1 C-D). Further, contrast enhanced CT scan of the head, chest and abdomen was done to evaluate complete extent of the disease. Contrast
European Journal of Cancer, 2003
Langerhans cell histiocytosis (LCH), characterised by the infiltration of one or more organs by large mononuclear cells, can develop in persons of any age. Although the features of this disease are well described in children, they remain poorly defined in adults. From January 2000 to June 2001, 274 adults from 13 countries, with biopsy-proven adult LCH, were registered with the International Histiocyte Society Registry. Information was collected about clinical presentation, family history, associated conditions, cigarette smoking and treatment, to assist in future management decisions in patients aged 18 years and older. There were slightly more males than females (143:126), and the mean ages at the onset and diagnosis of disease were 33 years (standard deviation (S.D.) 15 years) and 35 years (S.D. 14 years), respectively. 2 patients had consanguineous parents, and 1 had a family history of LCH; 129 reported smoking (47.1%); 17 (6.2%) had been diagnosed with different types of cancer. Single-system LCH, found in 86 patients (31.4%), included isolated pulmonary involvement in 44 cases; 188 patients (68.6%) had multisystem disease; 81 (29.6%) had diabetes insipidus. Initial treatment consisted of vinblastine administered with or without steroids, to 82 patients (29.9%), including 9 who had received it with etoposide, which was the sole agent given to 19 patients. 236 patients were considered evaluable for survival. At a median follow-up of 28 months from diagnosis, 15 patients (6.4%) had died (death rate, 1.5/100 person years, 95% Confidence Interval (95% CI) 0.9-2.4). The probability of survival at 5 years postdiagnosis was 92.3% (95% CI 85.6-95.9) overall, 100% for patients with single-system disease (n=37), 87.8% (95% CI 54.9-97.2) for isolated pulmonary disease (n=34), and 91.7% (95% CI 83.6-95.9) for multisystem disease (n=163). Survival did not differ significantly among patients with multisystem disease, with or without liver or lung involvement) 5-year survival 93.6% (95% CI 84.7-97.4) versus 87.5% (95% CI 65.5-95.9), respectively; P value 0.1). LCH in adults is most often a multisystem disease with the highest mortality seen in patients with isolated pulmonary involvement. It should be included in the differential diagnosis of disseminated or localised disease of the bone, skin and mucosa, as well as the lung and the endocrine and central nervous system, regardless of the age of the patient. A prospective international therapeutic study is warranted.
Pathohistological aspects of pulmonary Langerhans cell histiocytosis
Materia medica, 2018
Pulmonary Langerhans cell histiocytosis (PLCH) is a rare disease of unknown etiology, which most commonly affects men, smokers, aged from 20 to 40. It is diagnosed by histological analysis of material obtained by lung biopsy, with immunohistochemical proving of Langerhans cells. The aim of this research is to determine pathohistological characteristics of PLCH and analyzing demographic, clinical and radiological parameters. Retrospective analysis of medical data for 13 patients, proven for PLCH at Institute for Pulmonary diseases of Vojvodina in period of fifteen years. PLCH was found at 9 (69.3%) women and at 4 (30.7%) men, average age 34.7 years. Main clinical symptoms were cough (76.9%) and chest pain (61.5%). Out of 13 patients, 11 (84.6%) were smokers. In most cases PLCH histologically corresponded to the cellular phase of the disease (46.1%), proliferative phase was present at 5 (38.4%), and the fibrotic phase at 2 (15.5%) patients. Immunohistochemically, Langerhans' cells were positive for presence of CD1a and S-100 antigens in all 13 of analyzed cases, while CD68 antigen was positive in 6 patients. In 6 patients (46.2%) there was disease regression, and at 7 (53.8%) patients the disease progressed despite the applied therapy. In our research, PHLC was more common in younger females, smokers with cough and chest pain. At most of the patients, histologically disease was in the cellular phase. Langerhans cells were positive to presence of CD1a and S100 antigens in all 13 patients. At more than half of the patients the disease progresses despite the applied therapy.
2013
Langerhans cell histiocytosis (LCH) of the CNS is a rare entity, known to involve primarily the hypothalamicpituitary region, with the clinical hallmark of diabetes insipidus. There have been a few reports of CNS LCH involving the brainstem as intraparenchymal enhancing lesions, but this has never been the presenting complaint of LCH. The authors report on a 7-year-old boy who presented with right cerebellopontine syndrome, in whom a well-defined, solid, enhancing lesion in the brainstem was diagnosed. Clinicoradiological differential diagnosis included glioma and tuberculosis. Biopsy revealed atypical histiocytes positive for CD68, CD1a, and S100 protein; these are the diagnostic features of LCH on histopathological examination. The rapid growth of the lesion was controlled with a chemotherapeutic regimen of cladribine.
Primary Langerhan's cell histiocytosis of the brain - Case report and literature review
Primary Langerhan's cell histiocytosis of the brain is considered to be rare and with good prognosis. However, several cases in the recent literature proved that the disease is not particularly rare and frequently fatal. We are reporting a case of primary Langerhan's cell histiocytosis of the brain, which caused the death of a 39-year-old Saudi male patient. The tumour was excised totally twice and radiotherapy was given but in spite of that the patient's clinical status deteriorated rapidly and death occurred within 6 months. We found 14 other cases in the literature, 5 of the cases ended in death. Therefore, we report this case to draw attention to the fact that this disease is not rare nor benign as previously thought. (p40-44)
Cancer, 2018
Central nervous system Langerhans cell histiocytosis (CNS-LCH) brain involvement may include mass lesions and/or a neurodegenerative disease (LCH-ND) of unknown etiology. The goal of this study was to define the mechanisms of pathogenesis that drive CNS-LCH. Cerebrospinal fluid (CSF) biomarkers including CSF proteins and extracellular BRAFV600E DNA were analyzed in CSF from patients with CNS-LCH lesions compared with patients with brain tumors and other neurodegenerative conditions. Additionally, the presence of BRAFV600E was tested in peripheral mononuclear blood cells (PBMCs) as well as brain biopsies from LCH-ND patients, and the response to BRAF-V600E inhibitor was evaluated in 4 patients with progressive disease. Osteopontin was the only consistently elevated CSF protein in patients with CNS-LCH compared with patients with other brain pathologies. BRAFV600E DNA was detected in CSF of only 2/20 (10%) cases, both with LCH-ND and active lesions outside the CNS. However, BRAFV600EP...
Langerhans cell histiocytosis is a neoplasm and consequently its recurrence is a relapse
Pediatric Blood & Cancer, 2016
Langerhans cell histiocytosis (LCH) remains a poorly understood disorder with heterogeneous clinical presentations characterized by focal or disseminated lesions that contain excessive CD1a+ langerin+ cells with dendritic cell features known as "LCH cells." Two of the major questions investigated over the past century have been (i) the origin of LCH cells and (ii) whether LCH is primarily an immune dysregulatory disorder or a neoplasm. Current opinion is that LCH cells are likely to arise from hematopoietic precursor cells, although the stage of derailment and site of transformation remain unclear and may vary in patients with different extent of disease. Over the years, evidence has provided the view that LCH is a neoplasm. The demonstration of clonality of LCH cells, insufficient evidence alone for neoplasia, is now bolstered by finding driver somatic mutations in BRAF in up to 55% of patients with LCH, and activation of the RAS-RAF-MEK-ERK (where MEK and ERK are mitogen-activated protein kinase and extracellular signal-regulated kinase, respectively) pathway in nearly 100% of patients with LCH. Herein, we review the evidence that recurrent genetic abnormalities characterized by activating oncogenic mutations should satisfy prerequisites for LCH to be called a neoplasm. As a consequence, recurrent episodes of LCH should be considered relapsed disease rather than disease reactivation. Mapping the complete genetic landscape of this intriguing disease will provide additional support for the conclusion that LCH is a neoplasm and is likely to provide more potential opportunities for molecularly targeted therapies.