TISSUE DIAGNOSIS OF TWO DISTINCT EOSINOPHIL RICH LESIONS WITH SIMILAR HISTOMORPHOLOGY

2021 ◽  
pp. 33-34
Author(s):  
Shreekant Bharti ◽  
Bibhuti Bhushan ◽  
Punam Pd. Bhadani
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Neck Region ◽  
Langerhans Cell ◽  
Angiolymphoid Hyperplasia With Eosinophilia ◽  
Tissue Diagnosis ◽  
Angiolymphoid Hyperplasia ◽  
Radiological Features ◽  
Immune Mediated ◽  
Histological Criteria ◽  
Wide Range

Eosinophil rich lesions in histopathology have a wide range of differential diagnosis including conditions arising from inammatory, reactive, immune mediated, drugs, parasitic infestations to neoplasms of both hematopoietic and non-hematopoietic origin (Nutman, 2007). Some of them are yet to be clearly differentiated by histological criteria, though being genetically different, such as Kimura disease and Angiolymphoid hyperplasia with eosinophilia (ALHE) (Googe et al., 1987). Some close mimics of eosinophil rich reactive and clonal lesions need to be differentiated based on the site of involvement, radiological features and appropriate immunohistochemical evaluation on biopsy tissues. We present here two distinct diagnoses of Langerhans cell histiocytosis (LCH) and ALHE which appear similar in terms of eosinophil rich inammatory inltrate on histological examination but have entirely different clinical course. Summary: Langerhans cell histiocytosis is a locally aggressive slow growing neoplasm while Angiolymphoid hyperplasia with eosinophilia is a reactive lesion. Problem arises sometimes in the tissue diagnosis of these two lesions owing to their similarities in a couple of features. Both have a predilection for head and neck region. Both appear as an inammatory lesion with eosinophils being a striking feature in biopsy tissues. Since, both the lesions have an entirely different clinical behaviour and prognosis, it is important to differentiate between the two based on the histopathology, clinical as well as radiological features. This paper emphasizes upon the same.

Angiolymphoid hyperplasia with eosinophilia

1990 ◽  
Vol 104 (12) ◽  
pp. 977-979 ◽  
Author(s):  
J. F. Sharp ◽  
M. J. C. Rodgers ◽  
F. B. Macgregor ◽  
C. J. Meehan ◽  
K. McLaren
Keyword(s):  
Neck Region ◽  
Rare Condition ◽  
Surgical Removal ◽  
External Ear ◽  
Disease Entity ◽  
Peripheral Eosinophilia ◽  
Head And Neck Region ◽  
Angiolymphoid Hyperplasia With Eosinophilia ◽  
Angiolymphoid Hyperplasia ◽  
Unusual Condition

AbstractAngiolymphoid hyperplasia with eosinophilia is a rare condition and is poorly recognized in the otolaryngological literature. The condition is characterized by the appearance of cutaneous nodules within the head and neck region especially around the external ear. Variable lymphadenopathy and peripheral eosinophilia can occur and the condition can mimic neoplasia. It is important to be aware of this disease entity in order to avoid overtreatment. Surgical removal is the treatment of choice; however, this often multilobulated and poorly delineated lesion often precludes initial wide excision and local recurrence is common.We present three cases of this unusual condition and a brief resumé of the literature.

scholarly journals Laryngeal Langerhans Cell Histiocytosis Presenting with Neck Mass in an Adult Woman

2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Hesam Jahandideh ◽  
Yasser Nasoori ◽  
Sara Rostami ◽  
Mahdi Safdarian
Keyword(s):  
Head And Neck ◽  
Langerhans Cell Histiocytosis ◽  
Pediatric Patients ◽  
Neck Region ◽  
Rare Condition ◽  
Neck Mass ◽  
Langerhans Cell ◽  
Adult Woman ◽  
Head And Neck Region ◽  
Laryngeal Involvement

Langerhans cell histiocytosis (LCH) is a very rare condition that commonly affects the head and neck region. There are very few cases of isolated laryngeal involvement by LCH, mostly reported in pediatric patients. Here, we report a case of laryngeal LCH in a 62-year-old woman presenting with a neck mass several weeks ago. The clinical and histopathological findings are reported with a brief discussion about the disease.

scholarly journals Biological and clinical significance of somatic mutations in Langerhans cell histiocytosis and related histiocytic neoplastic disorders

Hematology ◽  
2015 ◽  
Vol 2015 (1) ◽  
pp. 559-564 ◽  
Author(s):  
Carl E. Allen ◽  
D. Williams Parsons
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Somatic Mutations ◽  
Mapk Pathway ◽  
Clinical Manifestations ◽  
Langerhans Cell ◽  
Braf V600e ◽  
Common Mechanism ◽  
Recurrent Mutations ◽  
Wide Range ◽  
Neoplastic Disorders

AbstractLangerhans cell histiocytosis (LCH), juvenile xanthogranuloma (JXG), and Erdheim–Chester disease (ECD) represent histiocytic disorders with a wide range of clinical manifestations. Until recently, mechanisms of pathogenesis have been speculative and debate has focused on classification of these conditions as reactive versus neoplastic. Genomic studies have been challenged by scarce tissue specimens, as well as heterogeneous nature of the lesions with variable infiltration of pathologic histiocytes. Whole-exome sequencing recently revealed a very low frequency of somatic mutations in LCH, JXG, and ECD compared to other neoplastic disorders. However, at least in the cases of LCH and ECD, there is a very high frequency of activating mutations in MAPK pathway genes, most notably BRAF-V600E, as well as MAP2K1, in LCH and NRAS in ECD. In ECD, recurrent mutations in the PI3K pathway gene PIK3CA have also been described. The heterogeneous clinical manifestations of these disorders may therefore be the cumulative result of activation of MAPK mutations (along with modifying signals from other pathways) at distinct stages of myeloid differentiation. Implications of this model include redefinition of LCH, JXG, and ECD as a group of clinically diverse myeloid neoplastic disorders with a common mechanism of pathogenesis. This model supports refocusing therapeutic strategies for these diseases on a personalized approach based on specific mutations and the cell(s) of origin.

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

2019 ◽  
Vol 27 (8) ◽  
pp. 836-843
Author(s):  
Xiaoxiao Liu ◽  
Ye Zhang ◽  
Chuan-Xiang Zhou
Keyword(s):  
Head And Neck ◽  
Langerhans Cells ◽  
Langerhans Cell Histiocytosis ◽  
Neck Region ◽  
Braf V600e Mutation ◽  
Langerhans Cell ◽  
Braf V600e ◽  
Chinese Patients ◽  
Clonal Proliferation ◽  
High Prevalence

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.

Evolving radiological features of hypothalamo-pituitary lesions in adult patients with Langerhans cell histiocytosis (LCH)

2005 ◽  
Vol 48 (1) ◽  
pp. 37-44 ◽  
Author(s):  
P. Makras ◽  
C. Samara ◽  
M. Antoniou ◽  
A. Zetos ◽  
D. Papadogias ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Adult Patients ◽  
Radiological Features

scholarly journals Clinical characteristics and outcomes of Langerhans cell histiocytosis at a single institution in Thailand: a 20-year retrospective study

2021 ◽  
Vol 15 (4) ◽  
pp. 171-181
Author(s):  
Ponrachet Kitticharoenjit ◽  
Nucharin Supakul ◽  
Piya Rujkijyanont ◽  
Chanchai Traivaree ◽  
Apichat Photia ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Langerhans Cell Histiocytosis ◽  
Clinical Characteristics ◽  
Clinical Manifestations ◽  
Langerhans Cell ◽  
Organ Involvement ◽  
Bone Imaging ◽  
X Ray ◽  
Wide Range ◽  
Conjugated Hyperbilirubinemia

Abstract Background Langerhans cell histiocytosis (LCH) is a rare disease characterized by the various systems involved and clinical manifestations with a wide range of symptoms. Objectives To describe clinical characteristics, imaging, treatment, and outcomes of pediatric LCH at Phramongkutklao Hospital, Bangkok, Thailand. Methods We conducted a 20-year retrospective review of the medical records of patients diagnosed with LCH from birth to 21 years old from January 1, 1997, to December 31, 2016. Results In all, 14 patients with median age of 2.5 years were studied. Six (43%) patients had single-system (SS) LCH. Five patients (63%) with multisystem (MS) LCH (n = 8. 57%) had risk-organ involvement (RO+). All patients had plain X-ray imaging of their skull with 11 (79%) showing abnormal findings. Tc-99m bone imaging and fluorodeoxyglucose F18 (FDG) positron emission tomography (PET)-computed tomography (CT) demonstrated abnormal findings in 8 (89%) and 4 (29%) patients, respectively. The 5-year event-free survival (EFS) for patients with RO+ MS-LCH was less than that for those without risk-organ involvement (RO−) MS-LCH and SS-LCH (20% vs. 100%, P = 0.005). Hematological dysfunction, hypoalbuminemia, and conjugated hyperbilirubinemia may be worse prognostic factors for RO+ MS-LCH. Conclusion FDG-PET-CT might have a greater accuracy to detect LCH disease than conventional plain X-ray and Tc-99m bone imaging. RO+ MS-LCH has been encountered with relapse and poor outcomes. Hematopoietic involvement, hypoalbuminemia, and conjugated hyperbilirubinemia may be worse prognostic factors for RO+ MS-LCH.

EVOLUTION OF TREATMENT OF CHILDREN WITH LANGERHANS CELL HISTIOCYTOSIS

2021 ◽  
Vol 100 (3) ◽  
pp. 107-120
Author(s):  
M. Minkov ◽  
◽  
◽  
Keyword(s):  
Intracellular Signaling ◽  
Langerhans Cell Histiocytosis ◽  
Standard Treatment ◽  
Organs At Risk ◽  
Severe Disease ◽  
Clinical Manifestations ◽  
Maintenance Phase ◽  
Langerhans Cell ◽  
Mitogen Activated Protein Kinase ◽  
Wide Range

Langerhans cell histiocytosis (LCH) is a rare myeloid neoplasm caused by mutations in proteins in the intracellular signaling MAPK (mitogen-activated protein kinase) pathways. Its rarity, wide range of clinical manifestations, and varied course made it difficult to conduct clinical trials. The use of different stratification systems made it even more difficult to compare the published results. The review attempts to summarize approaches to treatment of LCH in children since the 1960s. Materials and methods of research: searches for original papers in PubMed were performed using combinations of the keywords Langerhans cell histiocytosis, X histiocytosis, pediatrics, children, treatment, and therapy. Only full articles published in English and German languages were included in the review. Conclusion: a combination of vinblastine and prednisolone as an intensive initial phase (6–12 weeks) followed by a less intensive maintenance phase of 12 months is the standard treatment of disseminated LCH. Mortality in multisystem LCH is caused by damage to the organs at risk (hematopoiesis, liver and spleen) and is especially high (up to 60–70%) if the patient does not respond to standard treatment. In the remaining patients, mortality is almost negligible, with the main problems being the recurrence of the disease and its irreversible effects in 50% of the survivors. Although systemic therapy has clearly improved survival in patients with the most severe disease, its role in sustaining disease control and prevention of irreversible consequences in patients with low-risk disease is less obvious.

Radiological features of thymic langerhans cell histiocytosis

2013 ◽  
Vol 60 (11) ◽  
pp. E143-E145 ◽  
Author(s):  
Karoly Lakatos ◽  
Heidrun Herbrüggen ◽  
Ulrike Pötschger ◽  
Helmut Prosch ◽  
Milen Minkov
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Radiological Features

Head and Neck Langerhans Cell Histiocytosis

1997 ◽  
Vol 106 (6) ◽  
pp. 526-532 ◽  
Author(s):  
Kenneth O. Devaney ◽  
Alfio Ferlito ◽  
Mathew J. Putzi ◽  
Alessandra Rtnaldo
Keyword(s):  
Head And Neck ◽  
Langerhans Cell Histiocytosis ◽  
Neck Region ◽  
Langerhans Cell ◽  
Skeletal System ◽  
Head And Neck Region ◽  
The Past ◽  
System Disease ◽  
Secondary Infections ◽  
Multifocal Disease

Among the potential sites of involvement by Langerhans cell histiocytosis (LCH), the head and neck region is the most commonly cited. Though principally a pediatric disease, LCH can affect any age group. It can be unifocal (skeletal) or multifocal (skeletal and/or visceral); it appears as though the presence of visceral lesions is more common in the youngest patients, and may be associated in some with a rapidly progressive course resulting in death. Head and neck manifestations may mimic such varied entities as eczema, otitis media, osteomyelitis, and cholesteatoma. Current approaches to therapy are less aggressive than they were in the past, and are particularly intended to monitor for and treat any complicating secondary infections (which may develop in the youngest patients with multifocal disease including visceral involvement). The prognosis is very good for unifocal skeletal system disease, and poor for multifocal disease with involvement of tissues other than bone.

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