China
Africa
Explore chapters and articles related to this topic
Histiocytosis and Lipid Storage Diseases
Published in Harold R. Schumacher, William A. Rock, Sanford A. Stass, Handbook of Hematologic Pathology, 2019
Salwa Shabbir Sheikh, David F. Garvin
a. Juvenile Xanthogranuloma. Juvenile xanthogranuloma usually presents during infancy, but lesions may be present at birth or may appear during adulthood. There are widespread skin and mucous membrane nodules (Fig. 1) measuring 0.5–1.0 cm in diameter, which are yellow to red in color and most commonly affect the scalp and face, but may involve mesentery and viscera as well. The disease usually involutes spontaneously.
Histiocytic lesions
Published in Eckart Haneke, Histopathology of the NailOnychopathology, 2017
Histopathology of juvenile xanthogranuloma is characteristic. There is a granuloma-like infiltrate composed of lymphocytes, some eosinophils, foam cells, and giant cells both of foreign and Touton type. The latter typically exhibit a central homogeneously eosinophilic cytoplasm, a wreath of nuclei, and an outer ring of foamy cytoplasm. Cell atypias are not seen. An occasional mitosis may be present.
Iris Juvenile Xanthogranuloma Presenting with Hypopyon
Published in Ocular Immunology and Inflammation, 2022
Mine Esen Baris, Mukaddes Damla Ciftci, Melis Palamar, Suzan Guven Yilmaz
Juvenile xanthogranuloma (JXG) is a benign non-Langerhans histiocytic cutaneous disease that generally affects infants and young children but also is known to affect adults up to 77 years of age.1 Extracutaneous forms of the disease can affect the eye and ocular adnexa, as well as central nervous system, lung, liver, and other sites. JXG is also reported to be associated with systemic disorders and malignancies such as neurofibromatosis type 1 and juvenile myelomonocytic leukemia. Risk factors for ocular involvement include the presence of multiple cutaneous lesions and being under 2 years of age.2 Ocular involvement in JXG varies from 0.24% to 10%.3,4 Thus, although ocular involvement in cutaneous JXG is not common, potential complications that can lead to blindness such as glaucoma and persistent hyphema make ophthalmological examination mandatory for all children with cutaneous JXG.
Multiple subcutaneous xanthogranuloma at juxta-articular sites with bone cystic changes resembling rheumatoid arthritis: A case report
Published in Modern Rheumatology, 2018
Shoichi Kaneshiro, Kenrin Shi, Kosuke Ebina, Masao Yukioka, Hideki Yoshikawa, Norikazu Murata
Xanthogranuloma is usually a benign, asymptomatic, and self-healing nodule of non-Langerhans cell histiocytosis which predominantly occurs in the dermis in small children, but rarely in adults as the disease is also called as “juvenile xanthogranuloma”. The histological manifestation of the disease is accumulation of foamy histiocytes/macrophages with inflammatory cell infiltration and tissue necrosis. A specific type of xanthogranuloma affects a variety of organs such as kidney and gallbladder, but bone involvement is extremely rare [1]. Joint changes associated with infiltration of histiocytes or foam cells within bone tissue manifest various kinds, often with some difficulty in differential diagnosis from other diseases [2]. Here, we report a case of an adult patient with multiple subcutaneous xanthogranuloma at juxta-articular sites with bone cystic changes, manifesting very similar clinical profiles to and had been diagnosed and treated as rheumatoid arthritis (RA).
A chronic eyelid lesion in a child: multi-disciplinary approach to diagnosis, treatment and management of a highly atypical histiocytic lesion
Published in Pediatric Hematology and Oncology, 2022
Archana Ramgopal, Julia Segal, Sabrina Mukhtar, Jenny Yu, Jennifer Picarsic, Jean M. Tersak, Steven W. Allen
Malignant histiocytic neoplasm with histiocytic sarcoma (HS) phenotype is an extraordinarily rare malignant neoplasm arising from myeloid precursor derived hematopoietic tissue, accounting for less than one percent of all hematolymphoid neoplasms. It is distinguished cytologically by cells with phenotypic characteristics of mature tissue histiocytes.1,2 However, there are no defined or well-established criteria for an “atypical” juvenile xanthogranuloma (JXG) family that bears an increased proliferation index and atypia outside of what is expected for a typical benign JXG family lesion. JXG is often identified as a solitary lesion on the head and neck region, seen with characteristic Touton giant cells, and is typically positive for vimentin, CD68, and factor XIIIa, and negative for S-100 and CD1a.3 The Ki-67/MIB1 proliferation index is typically less than 20%; however, one described case that had an elevated Ki-67 (up to 40%) went on to have an aggressive clinical behavior and harbored a BRAF fusion.4,5 We present the case of a teenager presenting with a histiocytic eyelid lesion with a monomorphic infiltrate without Touton giant cells, an unusually high Ki-67 index of > 50%, lack of Factor XIIIa expression, but with a quiescent molecular profile. The differential diagnosis for such a lesion in a child includes a low-grade histiocytic lesion with atypical pathologic features versus a high-grade malignant histiocytic neoplasm with an unusual site and age.6,7 This report highlights the importance of an integrated team approach to the diagnosis of pediatric histiocytic neoplasms, especially those that hover between low-grade and high-grade neoplasms.