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Postepy Dermatologii I Alergologii Feb 2021
PubMed: 34408584
DOI: 10.5114/ada.2021.104292 -
Contemporary Oncology (Poznan, Poland) 2020Juvenile xanthogranuloma (JXG) is a rare non-Langerhans cell tumour usually diagnosed during infancy. The lesion is typically located in the skin; however,...
Juvenile xanthogranuloma (JXG) is a rare non-Langerhans cell tumour usually diagnosed during infancy. The lesion is typically located in the skin; however, extracutaneous lesions have been described. The symptoms vary depending on the location and size of the lesion. Presented here is a case of 13-year-old girl with this type of tumour located within the bronchus. She reported chest discomfort, difficulty breathing, and cough. A month prior to admission she had suffered from a respiratory tract infection. Prolonged surgical treatment was required due to the tumour's atypical location and recurrence of the tumour following initial resection. Isolated pulmonary JXG is an extremely rare finding, which requires accurate diagnosis and careful planning of therapeutic treatment. Severe pulmonary symptoms, inconclusive histopathological examination, malignancy risk, and large tumour size are indications for radical surgical resection of the tumour. Non-surgical treatment with chemotherapy is useful in cases of inoperable tumours.
PubMed: 33235547
DOI: 10.5114/wo.2020.97636 -
JAAD Case Reports Jan 2022
PubMed: 34977310
DOI: 10.1016/j.jdcr.2021.08.038 -
Applied Immunohistochemistry &... Jul 2022There are several activated forms of macrophages: 2 main groups are designated M1 and M2. While M1 macrophages have proinflammatory, bactericidal, and phagocytic...
There are several activated forms of macrophages: 2 main groups are designated M1 and M2. While M1 macrophages have proinflammatory, bactericidal, and phagocytic functions and are the dominant phenotype observed in the early stages of inflammation, M2 macrophages are involved in constructive processes such as tissue repair; they play a role in wound healing and are required for revascularization and re-epithelialization. Juvenile xanthogranuloma (JXG) is the most common non-Langerhans cell histiocytosis. Its pathogenesis is not well understood, but it is not considered a neoplastic entity. JXGs possibly appear as a reaction to a nonspecific injury such as trauma or viral infection, although a genetic predisposition has been suggested in some cases. Tissue damage leads to a histiocytic response. JXGs appear, evolve toward maturation, and then most of them spontaneously regress. Young JXGs are characterized by small macrophages scattered in the dermis, in apposition close to the epidermis. As the lesion matures, the number of foamy macrophages and Touton cells increases and other cell types such as plasma cells, lymphocytes, and polymorphs are observed. Regressing xanthogranulomas will show numerous spindle cells in Significant values are in bold.a storiform distribution, interstitial fibrosis, and few foamy and Touton cells. In this study, we studied the immunophenotypic profile of macrophages found in cutaneous JXGs according to their stage of maturation. We examined the skin biopsies from 25 patients; all were embedded in paraffin and stained with hematoxylin and eosin and for immunohistochemistry. Typically, all JXGs were positive for factor XIIIa and CD4, and were negative for CD1a. The following histiocyte markers were used: CD68, CD204, CD163, MAC387, and HAM56. Images were analyzed by Image J software; data were statistically evaluated by SAS 9.0 software. The cases showed a slight predominance of males and the preference of the JXGs for the axial skin. Lesions occupied the papillary and reticular dermis in 85% of the cases and extended to the subcutaneous fat in the remainder. Compared with mature and regressing JXGs, younger lesions had a higher density of M1 macrophages, stained with MAC387. This antibody labels the histiocytes that have recently arrived in the areas of inflammation. As the lesions matured, there was an overwhelming predominance of M2 macrophages. These cells tended to cluster against the epidermis, except in the 2 cases in phase of regression. This suggests that there is a cross-talk between the epidermis and macrophages and that receptors, cytokines, chemokines, and adhesion molecules may play a role in the development and evolution of JXGs. These results indicate that, for most of their life, JXGs are formed by repairing M2 macrophages and are not just an M1 macrophagic response to a local antigen. The process appears to be influenced by chemical-mediator epidermal-macrophage cross-talking, considering the tendency of these cells to accumulate against the dermoepidermal junction.
Topics: Female; Humans; Immunohistochemistry; Inflammation; Macrophages; Male; Skin; Xanthogranuloma, Juvenile
PubMed: 35435864
DOI: 10.1097/PAI.0000000000001029 -
Frontiers in Oncology 2020The association between acute lymphoblastic leukemia (ALL), non-Langerhans cell histiocytosis (non-LCH), and hemophagocytic lymphohistiocytosis (HLH), to the best of our...
The association between acute lymphoblastic leukemia (ALL), non-Langerhans cell histiocytosis (non-LCH), and hemophagocytic lymphohistiocytosis (HLH), to the best of our knowledge, has not been published to date. Juvenile xanthogranuloma (JXG), as a type of non-LCH, is usually a benign disease limited to the skin. Systemic involvement is rarely reported. The present case report describes a 15-year-old boy diagnosed with disseminated JXG involving skin and bone marrow concurrent with severe symptoms of HLH during ALL therapy. Examination of immunoglobulin heavy chain genes in B-cell precursor leukemic blasts and histiocytes in the skin and bone marrow revealed identical rearrangements, confirming clonal relationship between both diseases. Implementation of corticosteroids, vinblastine, etoposide, cyclosporine, and tocilizumab resulted in partial skin lesion resolution with no improvement of bone marrow function; therefore, hematopoietic stem cell transplantation (HSCT) was eventually performed. The patient's hematological and general status has improved gradually; however, remarkable recovery of skin lesions was observed after empirical antitubercular therapy. spp. infection should be considered as a possible secondary HLH trigger. Triple association of ALL, non-LCH, and HLH highlights heterogeneity of histiocytic disorders and possible common origin of dendritic and lymphoid cells.
PubMed: 32719740
DOI: 10.3389/fonc.2020.00921 -
Histopathology Apr 2024The discovery of somatic genetic alterations established many histiocytic disorders as haematologic neoplasms. We aimed to investigate the demographic characteristics...
AIMS
The discovery of somatic genetic alterations established many histiocytic disorders as haematologic neoplasms. We aimed to investigate the demographic characteristics and additional haematologic cancers of patients diagnosed with histiocytic disorders in The Netherlands.
METHODS AND RESULTS
We retrieved data on histiocytosis patients from the Dutch Nationwide Pathology Databank (Palga). During 1993 to 2022, more than 4000 patients with a pathologist-assigned diagnosis of a histiocytic disorder were registered in Palga. Xanthogranulomas were the most common subtype, challenging the prevailing assumption that Langerhans cell histiocytosis (LCH) is the most common histiocytic disorder. LCH and juvenile xanthogranuloma (JXG) had a peak incidence in the first years of life; males were overrepresented among all histiocytosis subgroups. 118 patients had a histiocytic disorder and an additional haematologic malignancy, including 107 (91%) adults at the time of histiocytosis diagnosis. In 16/118 patients, both entities had been analysed for the same genetic alteration(s). In 11 of these 16 patients, identical genetic alterations had been detected in both haematologic neoplasms. This included two patients with PAX5 p.P80R mutated B cell acute lymphoblastic leukaemia and secondary histiocytic sarcoma, further supporting that PAX5 alterations may predispose (precursor) B cells to differentiate into the myeloid lineage. All 4/11 patients with myeloid neoplasms as their additional haematologic malignancy had shared N/KRAS mutations.
CONCLUSIONS
This population-based study highlights the frequency of xanthogranulomas. Furthermore, our data add to the growing evidence supporting clonal relationships between histiocytic/dendritic cell neoplasms and additional myeloid or lymphoid malignancies. Particularly adult histiocytosis patients should be carefully evaluated for the development of these associated haematologic cancers.
Topics: Adult; Male; Humans; Histiocytosis, Langerhans-Cell; Histiocytes; Hematologic Neoplasms; Dendritic Cells; Demography
PubMed: 38213281
DOI: 10.1111/his.15127 -
International Journal of Surgical... Sep 2020Juvenile xanthogranuloma (JXG) is a benign histiocytic disorder usually affecting the head and trunk region of a child. The isolated occurrence of JXG in the penile...
Juvenile xanthogranuloma (JXG) is a benign histiocytic disorder usually affecting the head and trunk region of a child. The isolated occurrence of JXG in the penile shaft of a young adult is hitherto unreported. This lesion is amenable to surgical resection although systemic and/or internal visceral involvement can occur. The clinical differential of this solid cystic lesion may include other solid cystic lesions of the penile shaft, namely, epidermal inclusion cyst. A typical yellowish color can aid in the diagnosis though it is not a constant feature. In this article, we discuss a case of isolated penile JXG in a young adult with salient clinical and histopathological differentials.
Topics: Adolescent; Humans; Male; Penis; Xanthogranuloma, Juvenile
PubMed: 32338090
DOI: 10.1177/1066896920916242 -
Journal of Stomatology, Oral and... Oct 2022Juvenile xanthogranuloma, a form of non-Langerhans cell histiocytosis can be defined as the proliferation of cells with macrophage like characteristics. It has been...
Juvenile xanthogranuloma, a form of non-Langerhans cell histiocytosis can be defined as the proliferation of cells with macrophage like characteristics. It has been described as a benign, asymptomatic and common self-healing disorder of non-Langerhans cell histiocytosis (LCH), affecting mostly infants, children and rarely adults. We have documented a case of a 40 year old male who presented to us with extensive insidious papulonodulous growth over the face. The facial disfigurement caused was catastrophic. An extensive surgical excision and reconstruction was performed followed by histopathological evaluation. Microscopic study and immune histochemistry revaled Juvenile Xanthogranuloma of adult. The article highlights the presentation, diagnosis and management of this mammoth, rare disease.
Topics: Adult; Humans; Male; Xanthogranuloma, Juvenile
PubMed: 35395418
DOI: 10.1016/j.jormas.2022.03.019 -
Annals of Diagnostic Pathology Oct 2019Multinucleated giant cells (MGC) are commonly seen in an array of neoplastic and non-neoplastic conditions, to include: granulomatous dermatitis, fibrohistiocytic... (Review)
Review
Multinucleated giant cells (MGC) are commonly seen in an array of neoplastic and non-neoplastic conditions, to include: granulomatous dermatitis, fibrohistiocytic lesions such as xanthogranulomas, and soft tissue tumors such as giant cell tumors of soft tissue. In addition, multinucleated giant cells are infrequently seen in melanoma, squamous cell carcinoma, and atypical fibroxanthoma. There are many different types of MGCs and their presence, cytologic, and immunohistochemical features within these pathologic entities vary. Thus, correct identification of the different types of MGCs can aid the practicing pathologist in making the correct diagnosis of the overall pathologic disease. The biologic diversity and variation of MGCs is currently best exemplified in cytologic appearance and immunohistochemical profiles. However, much remains unknown about the origination and evolution. In this review, we i) reflect on the various types of MGCs and the current understanding of their divergent development, ii) describe the histologic, immunohistochemical, and molecular (if previously reported) differentiating features of common skin and superficial soft tissue neoplasms that may present with multinucleated giant cells.
Topics: Giant Cell Tumors; Giant Cells; Humans; Skin Neoplasms; Soft Tissue Neoplasms
PubMed: 31306855
DOI: 10.1016/j.anndiagpath.2019.06.013 -
Taiwan Journal of Ophthalmology 2023We report an unusual presentation of a 10-month-old girl with left eye (LE) redness and watering. Evaluation showed an iris vascular lesion and lens opacity in her LE....
We report an unusual presentation of a 10-month-old girl with left eye (LE) redness and watering. Evaluation showed an iris vascular lesion and lens opacity in her LE. Child underwent USG B-scan and ultrasound biomicroscopy, by which an extensive mass lesion arising from iris and ciliary body with absent calcification was revealed. Following extensive evaluation, child underwent cataract extraction and trans-scleral total excision of the mass lesion. Histopathology proved it as juvenile xanthogranuloma (JXG) with vascular proliferation. JXG is a rare benign self-limiting dermatologic disorder affecting mainly infants and small children. Ocular lesions are the most common extracutaneous manifestation. Cataract in JXG is less frequently reported. This case is reported due to its rarity and as it presented solely as an intraocular lesion with combined diffuse infiltration into ciliary body and cataract which is unusual. Early recognition and systematic approach helped in sight saving and organ salvaging.
PubMed: 37252160
DOI: 10.4103/tjo.tjo_35_21