Idiopathic granulomatous orchitis

Morphology and evaluation of its relationship to IgG4 related disease

Sarah Karram, Chia Sui Kao, Adeboye O. Osunkoya, Thomas Ulbright, Jonathan I. Epstein

Research output: Contribution to journalArticle

4 Citations (Scopus)

Abstract

Idiopathic granulomatous orchitis (IGO) is rare, thought to result from an autoimmune reaction to spermatogenic elements. Its relationship to IgG4-related disease (IgG4-RD) has not been evaluated. Sixteen orchiectomy specimens (1984-2012) with a prominent intratubular granulomatous reaction were reviewed: IGO (n = 6); intratubular germ cell neoplasia unclassified (IGCNU) with a granulomatous reaction and associated seminoma (GS, n = 6); and unclassified intratubular granulomatous orchitis not fitting into a specific entity (UGO, n = 4). Men with IGO were 32 to 86 years old, presenting with a mass suspicious for malignancy. Only one patient had a history of an inflammatory disease. Clinical follow-up was available for 2 patients with IGO, and both had no evidence of systemic IgG4-RD. All IGO cases had an epithelioid granulomatous reaction confined to seminiferous tubules, an extensive interstitial lymphoplasmacytic inflammation, 3 of 6 had prominent interstitial fibrosis, and 3 of 6 cases had plasma cells with an IgG4+/IgG+ ratio >40%. In GS, 10% to 100% of tubules with IGCNU had a granulomatous reaction, which in 3 cases replaced IGCNU cells. In contrast to IGO, GS had more intratubular multinucleated giant cells, more peritubular sclerosis, fewer interstitial plasma cells, and no interstitial fibrosis. Of the 4 UGO cases, most had predominantly interstitial with less intratubular granulomatous inflammation. Only 1 non-IGO case had elevated tissue IgG4 (GS case). It is critical and sometimes difficult to distinguish GS from IGO. IGO shares some features with IgG4-RD, yet current evidence does not support its classification as a localized manifestation of IgG4-RD occurring in the testis.

Original languageEnglish
Pages (from-to)844-850
Number of pages7
JournalHuman Pathology
Volume45
Issue number4
DOIs
StatePublished - 2014

Fingerprint

Orchitis
Immunoglobulin G
Germ Cells
Plasma Cells
Neoplasms
Fibrosis
Inflammation
Seminoma
Seminiferous Tubules
Orchiectomy
Sclerosis
Giant Cells
Testis

Keywords

  • Idiopathic granulomatous orchitis
  • IgG4
  • IgG4-related disease

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

Cite this

Idiopathic granulomatous orchitis : Morphology and evaluation of its relationship to IgG4 related disease. / Karram, Sarah; Kao, Chia Sui; Osunkoya, Adeboye O.; Ulbright, Thomas; Epstein, Jonathan I.

In: Human Pathology, Vol. 45, No. 4, 2014, p. 844-850.

Research output: Contribution to journalArticle

Karram, Sarah ; Kao, Chia Sui ; Osunkoya, Adeboye O. ; Ulbright, Thomas ; Epstein, Jonathan I. / Idiopathic granulomatous orchitis : Morphology and evaluation of its relationship to IgG4 related disease. In: Human Pathology. 2014 ; Vol. 45, No. 4. pp. 844-850.
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