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Xanthogranulomatous pyelonephritis (XGP) is an extremely rare, chronic granulomatous inflammatory condition thought to arise secondary to a combination of obstruction, recurrent bacterial infection and an incomplete immune response although the etiology of XGP is more complex. We would like to report a case of XGP occurring in a patient with polycystic kidney disease (PCKD), which has not been previously documented in etiology. A 29-year-old woman presented to our hospital with right upper quadrant pain for 5 days.

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Histological evidence of MAPK pathway activation across subtypes of adult orbital xanthogranulomatous disease irrespective of the detection of oncogenic mutations.

Clin Immunol

August 2024

The Rotterdam Eye Hospital, Rotterdam, the Netherlands; Department of Pathology, Leiden University Medical Center, Leiden, the Netherlands; Department of Pathology, section Ophthalmic Pathology, Erasmus MC University Medical Center Rotterdam, Rotterdam, the Netherlands. Electronic address:

Adult orbital xanthogranulomatous disease (AOXGD) is a spectrum of histiocytoses with four subtypes. Mitogen-activated protein kinase (MAPK) pathway mutations have been detected in various histiocytic neoplasms, little is known about this in AOXGD. Targeted regions of cancer- and histiocytosis-related genes were analyzed and immunohistochemical staining of phosphorylated ERK (pERK), cyclin D1 and PU.

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Introduction And Importance: Xanthomatous inflammation is a rare chronic inflammatory condition typically affecting organs such as the kidney and gallbladder. Its occurrence in the female genital tract, particularly in the ovaries and fallopian tubes, is exceptionally rare and sparsely documented.

Case Presentation: We report a unique case of xanthomatous inflammation involving the fallopian tube and ovary, characterized by the presence of hobnail cells and apocrine metaplasia.

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