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Neuroimaging Spectrum of Erdheim-Chester Disease: An Image-based Review.
AJNR Am J Neuroradiol
November 2024
From the (1) Department of Radiology, Tata Memorial Hospital, Parel, Mumbai 400012 (P.R.), (2) Department of Radiology, Mayo Clinic, Rochester, MN (H.J.S, J.C.B, S.A.M, P.J.F, C.M.C, G.B.), and (3) Department of Radiology, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL 55902 (N.S., A.A), and The authors have no relevant disclosures of interest.
Erdheim-Chester Disease (ECD) is a rare, multisystem histiocytic disorder characterized by its variable clinical presentations. Central Nervous System (CNS) involvement is observed in approximately half of ECD patients (up to 76% in some series), and often carries a poorer prognosis. While CNS involvement may remain asymptomatic, others may experience a range of neurological symptoms, including cognitive decline, neuropsychiatric disturbances, motor deficits, cranial or peripheral neuropathies, and endocrine abnormalities.
View Article and Find Full Text PDFManagement of Spinal Langerhans Cell Histiocytosis in Children: A Systematic Review.
Int J Spine Surg
November 2024
Department of Spine Surgery, Apollo Speciality Hospital, Perungudi, Chennai, Tamil Nadu, India.
Background: Spinal Langerhans cell histiocytosis can manifest as solitary site unifocal form or as systemic form in children. The management options for solitary spinal site unifocal form are many. They include spontaneous resolution of the lesion and supervised treatment, steroid injection of the lesion, systemic chemotherapy, radiation therapy and surgery.
View Article and Find Full Text PDFOrbital Langerhans Cell Histiocytosis: A Systematic Review of 228 Cases.
Cureus
October 2024
Department of Surgery, Baghdad Teaching Hospital, Medical City Complex, Baghdad, IRQ.
Orbital Langerhans cell histiocytosis (LCH) is an extremely rare disorder, and widely different manifestations often make it diagnostically challenging. The variability of symptoms - from common presentations, such as eyelid swelling and exophthalmos, to very atypical symptoms, like headaches and diplopia - frequently results in delayed diagnosis and mismanagement. This systematic review aims to describe in detail the clinical presentation, diagnostic approaches, treatment modalities, and outcomes of orbital LCH.
View Article and Find Full Text PDFUnusual presentation of primary central xanthoma of the maxilla associated with impacted canine: An update on immunohistochemistry in the diagnosis.
J Oral Maxillofac Pathol
July 2024
Department of Prosthodontics and Crown and Bridge, JSS Dental College and Hospital, Mysuru, Karnataka, India.
Article Synopsis
- A xanthoma is a rare condition characterized by lipid-rich foam cells and can occur in soft tissues and bones, often linked to hyperlipidemia.
- Primary bone xanthomas are uncommon benign lesions that aren’t associated with lipid issues, and the text discusses a unique case of a maxillary xanthoma in a healthy 23-year-old woman.
- The diagnosis involves distinguishing different types of histiocytes through specific immunohistochemical staining techniques.
Histopathological and Ultrastructural Study of a Canine Langerhans Cell Tumour (Canine Cutaneous Histiocytoma).
Cells
July 2024
School of Medicine and Biomedical Sciences, University of Porto, 4099-002 Porto, Portugal.
Canine cutaneous histiocytoma (CCH) represents a significant proportion of dog skin tumours, often manifesting as the most common neoplastic skin condition in young animals. Predominantly affecting dogs under four, these tumours appear primarily as solitary lesions that may regress spontaneously. This study, conducted over five years at the University of Trás-os-Montes e Alto Douro, involved a detailed histopathological and ultrastructural examination of 93 CCH cases.
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