Journal of Pathology and Translational Medicine

Original Article

A study of pathological characteristics and BRAF V600E status in Langerhans cell histiocytosis of Vietnamese children: Thu Dang Anh Phan, Bao Gia Phung, Tu Thanh Duong, Vu Anh Hoang, Dat Quoc Ngo, Nguyen Dinh The Trinh, Tung Thanh Tran; J Pathol Transl Med. 2021;55(2):112-117. Published online January 27, 2021; DOI: https://doi.org/10.4132/jptm.2020.11.30

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Background
Langerhans cell histiocytosis (LCH) is more common in children than adults and involves many organs. In children, the BRAF V600E mutation is associated with recurrent and high-risk LCH.
Methods
We collected paraffin blocks of 94 pediatric LCH patients to detect BRAF V600E mutation by sequencing. The relationship between BRAF V600E status and clinicopathological parameters were also critically analyzed.
Results
BRAF V600E mutation exon 15 was detected in 45 cases (47.9%). Multiple systems LCH showed a significantly higher BRAF V600E mutation rate than a single system (p=.001). No statistical significance was evident for other clinical characteristics such as age, sex, location, risk organs involvement, and CD1a expression.
Conclusions
In Vietnamese LCH children, the proportion of BRAF V600E mutational status was relatively high and related to multiple systems.

Citations

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Sulfur dots/Au@Ag nanorods array-based polarized ECL sensor for the detection of thyroid cancer biomarker
Zixuan Ding, Peilin Wang, Zhenrun Li, Yupeng Guo, Qiang Ma
Talanta.2023; 265: 124925. CrossRef

Case Study

Gastric crystal-storing histiocytosis with concomitant mucosa-associated lymphoid tissue lymphoma: Mee Joo, Nam-Hoon Kim; J Pathol Transl Med. 2020;54(4):332-335. Published online May 22, 2020; DOI: https://doi.org/10.4132/jptm.2020.04.20

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Abstract PDF

Crystal-storing histiocytosis (CSH) is a rare entity that is characterized by intrahistiocytic accumulation of crystallized immunoglobulins. CSH is not a malignant process per se, but the majority of CSH cases are associated with underlying lymphoproliferative disorder. Although CSH can occur in a variety of organs, gastric CSH is very rare. We present a localized gastric CSH with concomitant mucosaassociated lymphoid tissue (MALT) lymphoma, manifesting as an ulcer bleeding in a 56-year-old man. Histologically, the biopsied gastric mucosa demonstrated expansion of the lamina propria by prominent collections of large eosinophilic mononuclear cells containing fibrillary crystalloid inclusions. Immunohistochemical studies revealed that the crystal-storing cells were histiocytes harboring kappa light chain-restricted immunoglobulin crystals. Within the lesion, atypical centrocyte-like cells forming lymphoepithelial lesions were seen, consistent with MALT lymphoma. Since this entity is rare and unfamiliar, difficulties in diagnosis may arise. Particularly, in this case, the lymphomatous area was obscured by florid CSH, making the diagnosis more challenging.

Citations

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Crystal-storing histiocytosis in the stomach: A case report and review of the literature
Linghong Kong, Liyan Xue, Yanfeng Zhong, Shenglan Wang, Danfeng Zheng, Lining Wang, Yang Jiao, Xinpeng Zhang, Huizhong Xue, Xiaogang Liu
Frontiers in Oncology.2022;[Epub] CrossRef
Lambda-Restricted Crystal-Storing Histiocytosis of Stomach: A Case Report and Review of Literature
Nalini Bansal, Pankaj Puri, Nishant Nagpal, Rahul Naithani, Rahul Gupta
Cureus.2021;[Epub] CrossRef
Immunoglobulin-Storing Histiocytosis: A Case Based Systemic Review
Hanne Wiese-Hansen, Friedemann Leh, Anette Lodvir Hemsing, Håkon Reikvam
Journal of Clinical Medicine.2021; 10(9): 1834. CrossRef

Case Reports

Rosai-Dorfman Disease: Report of a Case Associated with IgG4-Related Sclerotic Lesions: Bong-Hee Park, Da Hye Son, Myung-Hwan Kim, Tae Sun Shim, Hee Jin Lee, Jooryung Huh; Korean J Pathol. 2012;46(6):583-586. Published online December 26, 2012; DOI: https://doi.org/10.4132/KoreanJPathol.2012.46.6.583

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Abstract PDF

We describe a rare case of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) associated with a six-year history of autoimmune pancreatitis, which was controlled by steroid treatment. The patient presented with multiple, cervical and thoracic lymphadenopathy and abnormal, nodular opacities in the lung. Histologically, Rosai-Dorfman disease with numerous IgG4-positive cells was identified in a subcutaneous lymph node in the patient's left forearm. The patient recovered uneventfully with steroid treatment.

Citations

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Lung Involvement in Destombes-Rosai-Dorfman Disease
Quentin Moyon, Samia Boussouar, Philippe Maksud, Jean-François Emile, Frédéric Charlotte, Nathalie Aladjidi, Grégoire Prévot, Jean Donadieu, Zahir Amoura, Philippe Grenier, Julien Haroche, Fleur Cohen Aubart
Chest.2020; 157(2): 323. CrossRef
Rosai-Dorfman Disease: Rare Pulmonary Involvement Mimicking Pulmonary Langerhans Cell Histiocytosis and Review of the Literature
Rashid AL Umairi, Danielle Blunt, Wedad Hana, Matthew Cheung, Anastasia Oikonomou
Case Reports in Radiology.2018; 2018: 1. CrossRef
IgG4‐related skin disease may have distinct systemic manifestations: a systematic review
Adam E. Bennett, Neil A. Fenske, Paul Rodriguez‐Waitkus, Jane L. Messina
International Journal of Dermatology.2016; 55(11): 1184. CrossRef
Nosology and Pathology of Langerhans Cell Histiocytosis
Jennifer Picarsic, Ronald Jaffe
Hematology/Oncology Clinics of North America.2015; 29(5): 799. CrossRef
A subset of Rosai–Dorfman disease cases show increased IgG4‐positive plasma cells: another red herring or a true association with IgG4‐related disease?
Madhu P Menon, Moses O Evbuomwan, Juan Rosai, Elaine S Jaffe, Stefania Pittaluga
Histopathology.2014; 64(3): 455. CrossRef

Rosai-Dorfman Disease in the Breast with Increased IgG4 Expressing Plasma Cells: A Case Report: Yoon Jin Cha, Woo Ick Yang, Se Ho Park, Ja Seung Koo; Korean J Pathol. 2012;46(5):489-493. Published online October 25, 2012; DOI: https://doi.org/10.4132/KoreanJPathol.2012.46.5.489

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Abstract PDF

Rosai-Dorfman disease (RDD) can present in any anatomic site, but breast involvement is rarely reported. Recently, a relationship between RDD and IgG4-related sclerosing disease has been suggested. Here we report another case of RDD with overlapping features of IgG4-related sclerosing disease occurring in a right breast of a 62-year-old female. On microscopic examination, the mass demonstrated a characteristic zonal pattern of proliferation of large polygonal histiocytes and lymphoplasma cells with stromal fibrosis. Emperipolesis was observed in histiocytes with abundant cytoplasm, which showed immunoreactivity for S-100 protein and CD68; the diagnosis of RDD was made. Sheets of plasma cells in the fibrotic stroma demonstrated positive reactions for IgG and IgG4. The mean count of IgG4-positive plasma cells was 100.2/high power field, and the ratio of IgG4/IgG was 56.7%. Additional findings of stromal fibrosis and obliteration of preexisting breast lobules suggested overlapping features with IgG4-related sclerosing disease.

Citations

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Pathology of IgG4-related sclerosing mastitis
Polycarp Erivwo, Gulisa Turashvili
Journal of Clinical Pathology.2021; 74(8): 475. CrossRef
Rosai–Dorfman Disease: Breast Involvement—Case Report and Literature Review
George Iancu, Nicolae Gica, Laura Mihaela Mustata, Anca Maria Panaitescu, Danut Vasile, Gheorghe Peltecu
Medicina.2021; 57(11): 1167. CrossRef
Sonographic features of Rosai‐Dorfman disease in the breast: A case report
Gi W. Shin, Young M. Park, Young J. Heo, Jin W. Baek, Yoo J. Lee, Ji Y. Han, Hayoung Park
Journal of Clinical Ultrasound.2020; 48(2): 108. CrossRef
Rosai-Dorfman Disease of the Breast With Variable IgG4+ Plasma Cells
Jenny C. Hoffmann, Chieh-Yu Lin, Siddhartha Bhattacharyya, Olga K. Weinberg, Karen M. Chisholm, Michael Bayerl, Michael Cascio, Girish Venkataraman, Kimberly Allison, Megan Troxell, Chung-Che Chang, Adam Bagg, Tracy I. George, Dennis O’Malley, Robert S. O
American Journal of Surgical Pathology.2019; 43(12): 1653. CrossRef
Rosai-Dorfman disease presenting as a breast mass
Dai Ding*, Cai Qi, Vohra Nasreen A. , Wong Jan, Therien Zsuzsanna P. , Hewan-Lowe Karlene , Sutton Ann
Archives of Pathology and Clinical Research.2019; 3(1): 008. CrossRef
Erdheim-Chester Disease Involving Lymph Nodes and Liver Clinically Mimicking Lymphoma: A Case Report
Yeoun Eun Sung, Yoon Seo Lee, Jieun Lee, Kyo Young Lee
Journal of Pathology and Translational Medicine.2018; 52(3): 183. CrossRef
Enfermedad de Rosai-Dorfman en mama de paciente masculino: una entidad rara
Paola Iturralde Rosas-Priego, José Daniel Flores-Alatriste, Daniela Stuht López, Javier Gómez Pedroso-Rea, Cecilia Ortiz-de-Iturbide, Jorge Valenzuela-Tamariz, Manuel Ubiergo-García
Revista de Senología y Patología Mamaria.2018; 31(2): 72. CrossRef
Increased Immunoglobulin G4-positive Plasma Cells in Lymphadenoma of the Salivary Gland: An Immunohistochemical Comparison Among Lymphoepithelial Lesions
Jiyoon Kim, Joon Seon Song, Jong-Lyel Roh, Seung-Ho Choi, Soon Yuhl Nam, Sang Yoon Kim, Kyung-Ja Cho
Applied Immunohistochemistry & Molecular Morphology.2018; 26(6): 420. CrossRef
IgG4-Related Sclerosing Disease of the Breast in a Male Patient
Taisia Vitkovski, Galina S. Marder, Dominic A. Filardi, Ekta Gupta, Frank Breuer
International Journal of Surgical Pathology.2017; 25(8): 711. CrossRef
Extranodal manifestation of Rosai-Dorfman disease in the breast tissue
Qiao Zhou, Umer Ansari, Nandan Keshav, Fiona Davis, Maria Cundiff
Radiology Case Reports.2016; 11(3): 125. CrossRef
Rosai-Dorfman Disease with Massive Cutaneous Nodule on the Shoulder and Back
Han Ma, Yue Zheng, Guoxing Zhu, Jie Wu, Chun Lu, Wei Lai
Annals of Dermatology.2015; 27(1): 71. CrossRef
IgG4-related disease of the breast: a systemic disease whose mammary manifestations mimic breast cancer
Takuya Moriya, Hisashi Hirakawa, Maki Nagashima, Mitsuhiko Yasuda, Izo Kimijima
International Cancer Conference Journal.2015; 4(2): 67. CrossRef
Primary Cutaneous Marginal IgG4 Lymphoma and Rosai–Dorfman's Disease Coexisting in Several Lesions of the Same Patient
Salma Machan, Camino Medina, Socorro María Rodríguez-Pinilla, José M. Suárez-Peñaranda, Yolanda Castro, Paula Molés, Celia Requena, Carles Saus, Luis Requena, Carlos Santonja
The American Journal of Dermatopathology.2015; 37(5): 413. CrossRef
A subset of Rosai–Dorfman disease cases show increased IgG4‐positive plasma cells: another red herring or a true association with IgG4‐related disease?
Madhu P Menon, Moses O Evbuomwan, Juan Rosai, Elaine S Jaffe, Stefania Pittaluga
Histopathology.2014; 64(3): 455. CrossRef
Freiburg Neuropathology Case Conference: A Partially Calcified, Dura-based Tumour of the Frontal Lobe
C. A. Taschner, O. Staszewski, R. Jabbarli, A. Keuler, M. Prinz
Clinical Neuroradiology.2013; 23(1): 63. CrossRef

Fine Needle Aspiration Cytology of Langerhans Cell Histiocytosis of Mandible: A Case Report.: Sang Ryung Lee, Jae Hee Suh, Hee Jung Cha, Young Min Kim, Hye Jeong Choi; Korean J Pathol. 2010;44(1):106-109.; DOI: https://doi.org/10.4132/KoreanJPathol.2010.44.1.106

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Abstract PDF

We present a case of mandibular involvement with Langerhans cell histiocytosis (LCH), diagnosed by ultrasound-guided aspiration and subsequently confirmed by incisional biopsy and immunohistochemistry in an eight-year-old boy. The cytologic findings included the presence of characteristic Langerhans cells of both mononucleate and multinucleate form. Diagnostic confirmation was obtained by immunopositivity for S-100 protein and CD1a of Langerhans histiocytes on paraffin-embedded sections obtained during incisional biopsy of the right mandibular area. By reporting a case of childhood LCH, we correlate the cytologic findings with histologic features and discuss the role of aspiration cytologic diagnosis in such a rare and cytomorphologically characteristic case.

Citations

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Bronchial Washing Cytology of Pulmonary Langerhans Cell Histiocytosis: A Case Report
Taeyeong Kim, Hyeong Ju Kwon, Minseob Eom, Sang Wook Kim, Min Hi Sin, Soon-Hee Jung
Journal of Pathology and Translational Medicine.2017; 51(4): 444. CrossRef

Original Articles

Pathological Characteristics of 20 Cases of Langerhans Cell Histiocytosis and Specificity of Immunohistochemical Stain of Langerin (CD207).: Youngjoon Ryu, Hyunjoo Lee, Sangho Lee, Hoiseon Jeong, Bongkyung Shin, Aeree Kim, Hankyeom Kim, Insun Kim; Korean J Pathol. 2009;43(2):113-119.; DOI: https://doi.org/10.4132/KoreanJPathol.2009.43.2.113

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Abstract PDF

BACKGROUND
Langerhans cell histiocytosis (LCH) is a well-known neoplastic disorder of Langerhans cells which has characteristic findings, however, LCH has not been adequately studied in Korea.
METHODS
We analyzed the clinicopathologic features of 20 patients with LCH who were diagnosed between 1997 and 2006 at the Korea University Guro and Anam Hospitals.
RESULTS
The M:F ratio was 3:1 and the age ranged from 2-60 years (mean, 23.8 years [4 in 1st decade, 6 in 2nd decade, 2 in 3rd decade, 5 in 4th decade and 3> or =40 years of age). The cases were classified as unifocal unisystemic in 13 patients, multifocal unisystemic in 4 patients, and multifocal multisystemic in 3 patients. The bone was the most commonly involved organ (14), followed by lymph node (5), lung (2), skin (2) and ureter (1). The Langerhans cells were immunohistochemically stained with Langerin, CD1a, S-100 protein, and CD68. Langerin and CD1a were specific for Langerhans cells.
CONCLUSIONS
The distribution of the involved organs in patients with LCH was similar to the distribution in Western countries, but lymph node involvement was more frequent, whereas lung involvement was less common. Langerin is considered to be a specific marker for Langerhans cells.

Citations

Citations to this article as recorded by

Treatment Outcome of Langerhans Cell Histocytosis
So Hak Chung, Jae Do Kim, Hyun Ik Jo
The Journal of the Korean Bone and Joint Tumor Society.2014; 20(1): 14. CrossRef

Fine Needle Aspiration Cytology of Langerhans' Cell Histiocytosis in the Lymph Node.: Eun Kyung Kim, Jong Eun Joo; Korean J Cytopathol. 1997;8(2):170-173.

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Abstract PDF: Langerhans' cell histiocytosis is a relatively rare disorder of children, characterized by abnormal proliferation of Langerhans' cells. It usually presents as multiple osteolytic lesion with lymphadenopathy or cutaneous manifestations. We experienced a case of Langerhans' cell histiocytosis involving bone and lymph node, diagnosed by fine needle aspiration cytology of the lymph node. The patient was a 10-year old girl with left inguinal lymphadenopathy. Fine needle aspiration cytology from the lymph node disclosed very cellular smear consisted of Langerhans' cells, eosinophils and lymphocytes. The Langerhans' cells had eccentric oval to reniform shape nuclei with grooving and abundant pale cytoplasm. The diagnosis was confirmed later by histologic study of bone lesion.

Case Reports

Fine Needle Aspiration Cytology of Langerhans Cell Histiocytosis in a Lymph Node: A Case Report.: Seung Yeon Ha, Min Jung Kim, Gou Young Kim, Hyun Yee Cho, Dong Hae Chung, Na Rae Kim; Korean J Cytopathol. 2007;18(1):87-91.

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Abstract PDF: Langerhans cell histiocytosis (LCH) is a pleomorphic disease entity characterized by local or disseminated atypical Langerhans cells (LCs) found most commonly in bone, lungs, mucocutaneous structures and endocrine organs. Among different sites, unifocal disease confined to a lymph node (LN) is rare. We report a case of LCH confined to a LN in a 38-year-old male who presented with a 2 cm-sized cervical mass. The fine needle aspiration (FNA) smears of cervical LN showed high cellularity having isolated LCs with contorted nuclei and nuclear grooves mixed in multinucleated giant cells, small lymphocytes and eosinophils. Charcot-Leyden crystals were also seen, as were a few dendritic-like cells and intranuclear inclusions. Confirmation of LCH was made by histopathologic studies, positive reactions for S-100 protein and CD1a immunohistochemical staining and by the demonstration of Birbeck granules on electron microscopy. The differentials to be considered include dermatopathic lymphadenitis, sinus histiocytosis with massive lymphadenopathy, Hodgkin's lymphoma and malignant histiocytosis. The characteristic cytomorphologic pattern of LCH in a LN FNA smear plays an important role in suggesting the diagnosis of LCH.

Juvenile Xanthogranuloma of the Nasal Cavity: A Case Report and Review of the Literature.: Jung Suk An, Sun Hee Han, Ju Han Lee, Eung Seok Lee, Heum Rye Park, Young Sik Kim; Korean J Pathol. 2006;40(3):241-244.

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Abstract PDF: Juvenile xanthogranuloma (JXG) is a benign and self-limiting non-Langerhans-cell histiocytosis that generally occurs during infancy and childhood. It develops frequently in the head and neck but is very rare in the nasal cavity. To date, only five cases of JXG in the nasal cavity have been reported. Here, we report the second case of JXG in the nasal cavity in Korea. A 19-year-old male patient presented with a protruding 1.1 cm mass in the left nasal vestibule. Histologically, a dense dermal infiltrate of histiocytes with Touton giant cells was observed. Immunohistochemically, the histiocytes tested positive for CD68 and the S-100 protein but negative for CD1a. This shows that a S-100-positive histiocytic lesion dose not exclude a diagnosis of JXG.

Original Article

Macrophage/dendritic Cell Marker Staining Characteristics of Langerhans cell Granulomatosis(Histiocytosis X).: Sang Ae Yoon, In Sun Kim; Korean J Pathol. 1992;26(3):310-313.

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Abstract PDF: Histiocytosis X is characterized by aggregates of Langerhans cells with other inflammatory cells. These Langerhans cells are antigen-presenting cells to T lymphocytes and identified by characteristic morphology, ultrastructural demonstration of Birbeck granules and immunologic reactivity with OKT-6 and HLA-DR antibodies. In this report, the tumor arising in a 2-years-old baby was examined byimmunostaining with several macrophage/dendritic cell markers. The main tumor cells showed cytoplasmic and nuclear staining with S-100 protein and ring-like surface and paranuclear staining with PNA. However, they were negative for follicular dendritic cell marker CD21, macrophage markers lysozyme, Mac 387, alpha-1 antitrypsin and CD68, and interdigitating reticulum cell marker ID4 and ID5. These observations demonstrate the usefulness of S-100 protein and PNA for the identification of Langerhans cells in paraffin-embedded tissue.

Case Report

Infantile Solitary Eosinophilic Granuloma of the Lymph Node: A case report.: Sun Hee Sung, Woo Ick Yang, Jae Ok Kim; Korean J Pathol. 1992;26(3):277-282.

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Abstract PDF: Infantile form of histiocytosis X is commonly presented as multiorgan desseminated form such as Letterer-Siwe disease. Lymph node involvement of histiocytosis X is usually accompanied by adjacent bone or skin lesion. Solitary nodal eosinophilic granuloma without evidence of other organ involvement is very rare. A case herein report is a 11 month-old female infant presented with fever and palpable both inguinal lymph nodes. There was neither skin lesion nor hepatosplenomegaly. Laboratory evaluation was within normal range except increased alkaline phosphatase and many neutrophils in urine. Radiologic examination revealed no remarkable bone lesions. And she showed good clinical outcome without evidence of other organ involvements. On microscopic examination of inguinal lymph node it was replaced by infiltration of histiocytes mainly along the sinusoid. Some of histiocytes showed morphologic features of "histiocytosis X cell" having nuclear grooves or multilobulation. Multinulceated giant cells were frequently see. Numerous eosinphils were also infiltrated and showed multifocal microabscess formation. Immunohistochemical staining revealed that majority of histiocytes were postitive for S-100 protein but multinucleated histriocytes, phagocytic histiocytes and those around the abscess were positive for macrophage marker, suck as CD68 and alpha-1-antichymotrypsin. Interestingly some histiocytes showed positivity for both S-100 protein and macrophage marker. These results suggest that histiocytosis X is proliferative disorder of phenotypically heterogenous population of histiocytes in contrast to the theory that it is a proliferative disorder of Langerhans cells.

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