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Endocrinopathies are frequently the initial presentation of histiocytic neoplasms, which are rare hematologic disorders affecting multiple organ systems. Langerhans cell histiocytosis and Erdheim-Chester disease are 2 such disorders known to infiltrate the hypothalamus and/or pituitary gland, leading to arginine vasopressin deficiency (AVP-D) and anterior pituitary dysfunction (APD) in 20% to 30% of cases, often as the first manifestation. Conversely, histiocytic disorders account for a notable proportion (10-15%) of all pituitary stalk lesions.

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The patient was a 57-year-old man who developed bilateral thigh pain and chest tightness one year ago. Chest CT scan showed reticular shadows, thickened interlobular septa in both lung fields, and pericardial effusion. Three months ago, his symptoms worsened.

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Total Hip Replacement in a Patient with Erdheim-Chester's Disease: A Case Report.

JBJS Case Connect

October 2024

Max Institute of Musculoskeletal Sciences, Max Super Speciality Hospital, Saket, New Delhi, India.

Case: Erdheim-Chester's disease (ECD) is a rare multisystem disorder considered as a neoplasm of non-Langerhans cell histiocytes. We report a case of uncemented total hip arthroplasty in a 74-year-old patient with ECD for femoral neck fracture with pathological involvement of distal metaphysis and diaphysis. We reviewed the literature on bony involvement.

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Objective: To describe the clinical presentations and radiological manifestations of Erdheim-Chester disease (ECD) in the extremities, with particular emphasis on radiologic findings, as radiographs are typically the initial imaging modality used in clinical practice.

Methods: Following the PRISMA guidelines, a comprehensive systematic search was performed across Scopus, PubMed, Web of Science, and Embase databases, covering case reports from inception until August 1, 2024. Included were studies with pathologically confirmed ECD (CD68 positive and CD1a negative) that were evaluated with at least one imaging modality and provided detailed descriptions of radiological findings.

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Foamy Cell Angiosarcoma Resembling a Xanthomatous Histiocytic Infiltrate Without Evidence of Vascular Malignancy.

J Cutan Pathol

December 2024

Department of Medicine, Division of Dermatology, St. Louis School of Medicine, Washington University, St. Louis, Missouri, USA.

Angiosarcoma is a rare and aggressive malignancy of endothelial cells with multiple subtypes. Foamy cell angiosarcoma is a rare variant in which endothelial cells demonstrate "foamy" cytoplasmic change. We present the case of a 59-year-old male who presented with progressive erythema and swelling of the midface and bilateral eyelids.

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