scholarly journals Isolated Langerhans Cell Histiocytosis of Orbit: A Case Report and Review of the Literature

2018 ◽  
Vol 2018 ◽  
pp. 1-6
Author(s):  
Mayuresh Naik ◽  
Anuj Mehta ◽  
Neha Mehrotra ◽  
Anil Solanki

A 2-year-old male child presented with a painless progressive mass in the inferolateral aspect of right orbit of three-month duration. Differential leucocyte count revealed raised eosinophil count (13%). On radiological examination, CT scan showed 25 × 27 mm round well-defined smooth-outlined homogenously enhancing extraconal mass arising from the zygomatic bone at the inferotemporal periorbital area of right orbit with bone erosion. Histopathological examination of the incision biopsy revealed characteristic Langerhans cells and immunohistochemical studies were positive for S-100 protein and adenosine deaminase. A diagnosis of Langerhans Cell Histiocytosis (LCH) was made and PET-CT revealed no other foci of uptake anywhere else in the body. The patient received 12 cycles of vinblastine, 0.2 mg/kg body weight, along with oral prednisolone, 1 mg/kg body weight. On completion of three cycles of chemotherapy, a reduction in size of the mass was noticed. A repeat PET scan was done 3 months after completion of chemotherapy did not reveal any activity noted previously.

2013 ◽  
Vol 16 (4) ◽  
pp. 93
Author(s):  
Angela Jordão Camargo ◽  
Mayara Cheade ◽  
Celso Martinelli ◽  
Solange Aparecida Caldeira Monteiro ◽  
Christiano De Oliveira Santos ◽  
...  

<p>This article reports a case of 65 year-old man consultedin a private radiology dental clinic for a panoramicradiography, where was indicated a radiolucent area,extending into the periapical region of the teeth 35 to 43 to the base of the mandible. Radiographydiagnosis was difficult. The immunohistochemical examination showed immunoreactivity for theproteins CD 68, S-100 and CD 1a. Histopathologicaldiagnosis was obtained for Langerhans cell granuloma(histiocytosis X). Langerhans cell histiocytosis iscaused by the abnormal proliferation of histiocytes, previously called Histiocytosis X. It is a rare diseasethat preferentially affects children and young adultswith a predilection for males and can affect differentparts of the body.</p><p>Keywords<br />Adult; Histiocytosis; Langerhans; Mandible.</p>


2010 ◽  
Vol 14 (5) ◽  
pp. 245-248 ◽  
Author(s):  
Iman Hemmati ◽  
W. Alastair McLeod ◽  
Richard I. Crawford

Background: Progressive mucinous histiocytosis (PMH) is a benign, non-Langerhans cell histiocytosis with characteristic ultrastructural features that can be used for diagnosis. Once an important tool in dermatologic diagnosis, electron microscopy has been largely replaced by immunohistochemistry and immunofluorescence techniques today. However, electron microscopy occasionally still plays a crucial role in the diagnosis of dermatologic conditions. We report a case of PMH as an example of a dermatologic disorder that requires electron microscopy for its diagnosis. Methods: A 60-year-old woman presented to our clinic with a history of small, sharply demarcated, skin-colored papules ranging from 2 to 5 mm in diameter distributed over the arms, forearms, and dorsal hands. The results of light microscopy, immunohistochemical studies, and clinical examination were inconclusive. Another biopsy for electron microscopy showed the characteristic features of PMH. Conclusion: This case demonstrates that a dermatopathology service still needs to have access to electron microscopy for diagnostic purposes to successfully diagnose a small number of rare conditions.


2017 ◽  
Vol 26 (2) ◽  
pp. 194-196 ◽  
Author(s):  
Hee Jung Kwon ◽  
Phil Hyun Song ◽  
Mi Jin Gu

Langerhans cell histiocytosis is a rare disease that is characterized by a localized or systemic proliferation of Langerhans dendritic cells and a wide spectrum of clinical presentations. We experienced an unusual case of occult Langerhans cell histiocytosis associated with clear cell renal cell carcinoma. A 62-year-old man underwent a partial nephrectomy for left renal mass. Histologic examination showed nests of clear cells with surrounding thin-walled vessel that were suggestive of clear cell renal cell carcinoma. A well-defined nodule composed of atypical mononuclear cells and plentiful eosinophils presented within clear cell renal cell carcinoma. The mononuclear cells were Langerhans cell histiocytes with S-100 and CD1a immunoreactivity. On follow-up comprehensive physical, laboratory, and radiologic examination, there were no other lesions of Langerhans cell histiocytosis.


2012 ◽  
Vol 02 (04) ◽  
pp. 77-79
Author(s):  
Jayaprakash Shetty K. ◽  
Ajith Kumar ◽  
Shantharam Shetty ◽  
Kishan Prasad H. L. ◽  
Chandrika Rao

AbstractLangerhans cell histiocytosis most commonly occurring in children, demonstrates a broad spectrum of clinical and radiologic features that may mimic those of infection as well as benign and malignant tumors. Osseous involvement is the most common manifestation. Recognition of the skeletal alterations is important so that the disease is considered as differential diagnosis. This disease is of unknown aetiology, non-specific clinical and imaging findings with diagnosis possible only on histopathological examination, will always pose a diagnostic challenge to the orthopaedic surgeon. Here by we are reporting two cases of eosinophilic granuloma that has presented with lytic lesion in the tibia and scapula. The clinico-radiological suspicion was either infection or tumour of the affected bones. Hence, core biopsy was done confirmed as eosinophilic granuloma on histopathology and immunohistochemistry.


2001 ◽  
Vol 5 (6) ◽  
pp. 486-489 ◽  
Author(s):  
Kelli W. Morgan ◽  
Jeffrey P. Callen

Background: Congenital self-healing Langerhans cell histiocytosis (CSHLCH) is a rare condition which may present at birth or during the neonatal period. It is usually characterized by the eruption of multiple, disseminated, red-brown papules and nodules which may increase in size and number during the first few weeks of life. Systemic signs are usually absent except for occasional mild hepatomegaly. Objective: We present a 3.5-kg male infant who presented at birth with numerous diffuse, erythematous, crusted erosions. He was presumed to have congenital herpes simplex virus (HSV) and was started on IV acyclovir. Histopathologic examination revealed a mixed inflammatory infiltrate with numerous histiocytes which were S-100 and peanut agglutin positive consistent with CSHLCH. Further workup did not reveal any signs of systemic involvement. Conclusion: CSHLCH has rarely been reported to present as a papulovesicular eruption at birth. In these cases, a viral etiology is commonly entertained in the differential diagnosis. Despite the spontaneous regression of skin lesions in CSHLCH, close followup is required to evaluate for systemic signs and symptoms associated with latent Letterer–Siwe disease.


2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Roshni Patel ◽  
Shahrukh Kadri ◽  
Priyanshee Gohil ◽  
Shrikalp Deshpande ◽  
Gaurang Shah

Abstract Background Rheumatoid arthritis is the most common cause of disability, affecting 0.3–1% of the adult population worldwide. The latex of Calotropis procera possesses potent anti-inflammatory as well as analgesic properties. In light above facts, the present study was designed to evaluate anti-arthritic activity of Calotropis procera latex in complete Freund's adjuvant (CFA)-induced arthritis in Wistar albino rats. Complete Freund's adjuvant was injected into the left hind paw on day 0, and treatment of prednisolone and Calotropis procera latex was given from day 0 to 28. Various biochemical, hematological and functional parameters as well as radiological and histopathological changes of joint along with body weight and paw volume were measured. Results Calotropis procera treatment significantly lowered paw volume in CFA-induced arthritic rats. Significant improvement was observed in functional, biochemical and hematological parameters in Calotropis procera-treated rats. However, the body weight remained unaffected. Histological and radiographical examination of synovial joints in Calotropis procera-treated animals exhibited less synovial hyperplasia, infiltration and accumulation of inflammatory cell in synovial fluid, cartilage and bone erosion and joint space narrowing. Conclusion Calotropis procera latex possesses anti-arthritic activity, which is facilitated by modulation in the level of inflammatory mediators and oxidative stress. The improvement in hematological as well as biochemical parameters might be reflected on functional, histopathological, radiological changes and thereby disease progression.


Author(s):  
Tuan Nguyen Minh

Histiocytic disorders in children and adults are caused by an abnormal accumulation of cells of the mononuclear phagocytic system. Langerhans cell histiocytosis (LCH) is a myeloid-derived dendritic cell disorder. The disease may affect any organ or system of the body, but those more frequently affected are the skin, bone and lymph nodes. Objectives: To evaluate characteristics of demography, location of lesions and histopathology of LCH. Methods: Cross sectional study of 80 children who have been performed biopsy with pathological diagnosis of LCH at Children’s Hospital 1 from Jan 2015 to Dec 2019. Results: LCH was encountered in children from 1 month to 12 years old in the study population. The mean age is 3.1 ± 2.7 (years) and in both male and female (M: F= 1.7/1). The most frequent sites of lession are skin and soft tissues. Conclusion: Langerhans cell histiocytosis is a malignant disease that may be encountered in clinical practice with different sites of lesions. LCH should be thought of when suspected and diagnosed early by histopathology to help guide treatment promptly and appropriately


2004 ◽  
Vol 31 (12) ◽  
pp. 1023-1026 ◽  
Author(s):  
Aiko Imanaka ◽  
Masahito Tarutani ◽  
Haruyasu Itoh ◽  
Masahiro Kira ◽  
Satoshi Itami

Blood ◽  
2012 ◽  
Vol 120 (12) ◽  
pp. e28-e34 ◽  
Author(s):  
Felix Sahm ◽  
David Capper ◽  
Matthias Preusser ◽  
Jochen Meyer ◽  
Albrecht Stenzinger ◽  
...  

AbstractLangerhans cell histiocytosis (LCH) is a clinically and histologically heterogeneous disorder. Its classification as either reactive inflammatory or neoplastic has been a matter of debate. However, the recent finding of frequent BRAFV600E mutations in LCH argues for the latter. The exact cell type that harbors the mutation and is responsible for proliferation remains to be identified. We here apply a BRAFV600E mutation-specific antibody to detect the BRAF mutant cells in lesions from 89 patients with LCH. We found BRAFV600E mutations in 34 of 89 (38%) lesions. In lesions with the BRAFV600E mutation, the majority of cells coexpressing S-100 and CD1a harbored mutant BRAFV600E protein. These cells also expressed CD14 and CD36, whereas various fractions exhibited CD207. On the other hand, CD80 and CD86 expression was also present on BRAFV600E-positive cells. Thus, cells of variable maturation, exhibiting an immunohistochemical profile compatible either with myeloid cell or with dedifferentiated Langerhans cell antigens, carry the BRAFV600E mutation. In conclusion, we identify and characterize the neoplastic cells in LCH with BRAFV600E mutations by applying a mutation-specific marker and demonstrate feasibility for routine screening.


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