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IgG4-related disease of the paratestis in a patient with Wells syndrome: a case report.

Karashima T, Taniguchi Y, Shimamoto T, Nao T, Nishikawa H, Fukata S, Kamada M, Inoue K, Oko K, Nakajima H, Sano S, Matsumoto M, Kuroda N, Kamei Y, Shuin T - Diagn Pathol (2014)

Bottom Line: Blood testing showed no abnormalities other than a minimal increase in C-reactive protein levels.Urine examination likewise revealed no significant findings.Left radical orchidectomy was performed under a diagnosis of left paratesticular neoplasm suspected as malignant tumor.

View Article: PubMed Central - PubMed

Affiliation: Department of Urology, Kochi Medical School, Kohasu, Oko, Nankoku, Kochi, 783-8505, Japan. karasima@kochi-u.ac.jp.

ABSTRACT

Background: We report a case of a 33-year-old man who presented with immunoglobulin (Ig)G4-related disease (IgG4-RD) forming a pseudotumor in the left paratesticular region during oral administration of corticosteroid for Wells syndrome, which involves cellulitis with eosinophilia.

Case presentation: The patient was introduced to our institution from a private hospital with a 3-month history of asymptomatic left scrotal mass. A 5-cm diameter nodule was palpable in the left scrotum. Tumor lesion in the left paratestis involving the epididymis and spermatic cord was observed on computed tomography and magnetic resonance imaging. Blood testing showed no abnormalities other than a minimal increase in C-reactive protein levels. Urine examination likewise revealed no significant findings. Left radical orchidectomy was performed under a diagnosis of left paratesticular neoplasm suspected as malignant tumor. The tumor was pathologically identified as IgG4-RD of the left paratestis involving the epididymis and spermatic cord.

Conclusions: We present a first description of IgG4-RD in a patient with Wells syndrome and the ninth case of IgG4-RD in a scrotal organ, and discuss this very rare entity with reference to the literature.

Virtual slides: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/13000_2014_225.

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Related in: MedlinePlus

Microscopic findings of surgical specimen. Hematoxylin-eosin staining of the paratesticular mass reveals widespread fibrosis and lymphocyte aggregation from the left epididymis to the spermatic cord (×40;A). Spindle cell proliferation with chronic inflammatory cells mostly comprised plasma cells with neither atypia nor mitosis (×200;B). Myofibroblastic cells with marked fibrosis reveals a storiform pattern (×100;C). Obliterative phlebitis is apparent (×100;D). For immunostaining, sections were stained with anti-alpha-smooth muscle actin (α-SMA) (1A4, 1:800, DAKO, Glostrup, Denmark) and -IgG4 (HP6025, 1:1280; ZYMED Laboratories, CA, USA) antibodies by using automated immunostainer (Ventana Benchmark, Tucson, AZ, USA). Spindle cells are positive for α-SMA, indicating myoepithelial cells (×100;E). More than 10 IgG4-positive plasma cells/HPF are seen on anti-IgG4 immunostaining (×400;F).
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Fig3: Microscopic findings of surgical specimen. Hematoxylin-eosin staining of the paratesticular mass reveals widespread fibrosis and lymphocyte aggregation from the left epididymis to the spermatic cord (×40;A). Spindle cell proliferation with chronic inflammatory cells mostly comprised plasma cells with neither atypia nor mitosis (×200;B). Myofibroblastic cells with marked fibrosis reveals a storiform pattern (×100;C). Obliterative phlebitis is apparent (×100;D). For immunostaining, sections were stained with anti-alpha-smooth muscle actin (α-SMA) (1A4, 1:800, DAKO, Glostrup, Denmark) and -IgG4 (HP6025, 1:1280; ZYMED Laboratories, CA, USA) antibodies by using automated immunostainer (Ventana Benchmark, Tucson, AZ, USA). Spindle cells are positive for α-SMA, indicating myoepithelial cells (×100;E). More than 10 IgG4-positive plasma cells/HPF are seen on anti-IgG4 immunostaining (×400;F).

Mentions: Histopathology of the tumor demonstrated spindle-shaped epithelial and polygonal cell proliferations with storiform fibrosis. Little atypia and few mitoses were identified among spindle-shaped epithelial cells. Plasmacytes, lymphocytes and eosinophils had infiltrated into the tumor. Typical obstructive phlebitis was also observed. Positive immunostaining was obtained for vimentin, α-smooth muscle actin (αSMA) and desmin (focally), but negative results were seen for CD34, S100 protein, p53, anaplastic lymphoma kinase (ALK), cytokeratin (CAM5.2), calretinin, Wilms’ tumor-1, thrombomodulin, epithelial membrane antigen and lymphatic endothelial marker in the spindle cells, identifying the myofibroblastic cells. IgG4-positive cells comprised 50% among the cells staining positively for IgG, and the number of IgG4-positive plasma cells/high-power fields (HPF) were more than 10 (Figure 3). The histological finding of pseudotumor was consistent with IgG4-RD in the left epididymis.Figure 3


IgG4-related disease of the paratestis in a patient with Wells syndrome: a case report.

Karashima T, Taniguchi Y, Shimamoto T, Nao T, Nishikawa H, Fukata S, Kamada M, Inoue K, Oko K, Nakajima H, Sano S, Matsumoto M, Kuroda N, Kamei Y, Shuin T - Diagn Pathol (2014)

Microscopic findings of surgical specimen. Hematoxylin-eosin staining of the paratesticular mass reveals widespread fibrosis and lymphocyte aggregation from the left epididymis to the spermatic cord (×40;A). Spindle cell proliferation with chronic inflammatory cells mostly comprised plasma cells with neither atypia nor mitosis (×200;B). Myofibroblastic cells with marked fibrosis reveals a storiform pattern (×100;C). Obliterative phlebitis is apparent (×100;D). For immunostaining, sections were stained with anti-alpha-smooth muscle actin (α-SMA) (1A4, 1:800, DAKO, Glostrup, Denmark) and -IgG4 (HP6025, 1:1280; ZYMED Laboratories, CA, USA) antibodies by using automated immunostainer (Ventana Benchmark, Tucson, AZ, USA). Spindle cells are positive for α-SMA, indicating myoepithelial cells (×100;E). More than 10 IgG4-positive plasma cells/HPF are seen on anti-IgG4 immunostaining (×400;F).
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4265405&req=5

Fig3: Microscopic findings of surgical specimen. Hematoxylin-eosin staining of the paratesticular mass reveals widespread fibrosis and lymphocyte aggregation from the left epididymis to the spermatic cord (×40;A). Spindle cell proliferation with chronic inflammatory cells mostly comprised plasma cells with neither atypia nor mitosis (×200;B). Myofibroblastic cells with marked fibrosis reveals a storiform pattern (×100;C). Obliterative phlebitis is apparent (×100;D). For immunostaining, sections were stained with anti-alpha-smooth muscle actin (α-SMA) (1A4, 1:800, DAKO, Glostrup, Denmark) and -IgG4 (HP6025, 1:1280; ZYMED Laboratories, CA, USA) antibodies by using automated immunostainer (Ventana Benchmark, Tucson, AZ, USA). Spindle cells are positive for α-SMA, indicating myoepithelial cells (×100;E). More than 10 IgG4-positive plasma cells/HPF are seen on anti-IgG4 immunostaining (×400;F).
Mentions: Histopathology of the tumor demonstrated spindle-shaped epithelial and polygonal cell proliferations with storiform fibrosis. Little atypia and few mitoses were identified among spindle-shaped epithelial cells. Plasmacytes, lymphocytes and eosinophils had infiltrated into the tumor. Typical obstructive phlebitis was also observed. Positive immunostaining was obtained for vimentin, α-smooth muscle actin (αSMA) and desmin (focally), but negative results were seen for CD34, S100 protein, p53, anaplastic lymphoma kinase (ALK), cytokeratin (CAM5.2), calretinin, Wilms’ tumor-1, thrombomodulin, epithelial membrane antigen and lymphatic endothelial marker in the spindle cells, identifying the myofibroblastic cells. IgG4-positive cells comprised 50% among the cells staining positively for IgG, and the number of IgG4-positive plasma cells/high-power fields (HPF) were more than 10 (Figure 3). The histological finding of pseudotumor was consistent with IgG4-RD in the left epididymis.Figure 3

Bottom Line: Blood testing showed no abnormalities other than a minimal increase in C-reactive protein levels.Urine examination likewise revealed no significant findings.Left radical orchidectomy was performed under a diagnosis of left paratesticular neoplasm suspected as malignant tumor.

View Article: PubMed Central - PubMed

Affiliation: Department of Urology, Kochi Medical School, Kohasu, Oko, Nankoku, Kochi, 783-8505, Japan. karasima@kochi-u.ac.jp.

ABSTRACT

Background: We report a case of a 33-year-old man who presented with immunoglobulin (Ig)G4-related disease (IgG4-RD) forming a pseudotumor in the left paratesticular region during oral administration of corticosteroid for Wells syndrome, which involves cellulitis with eosinophilia.

Case presentation: The patient was introduced to our institution from a private hospital with a 3-month history of asymptomatic left scrotal mass. A 5-cm diameter nodule was palpable in the left scrotum. Tumor lesion in the left paratestis involving the epididymis and spermatic cord was observed on computed tomography and magnetic resonance imaging. Blood testing showed no abnormalities other than a minimal increase in C-reactive protein levels. Urine examination likewise revealed no significant findings. Left radical orchidectomy was performed under a diagnosis of left paratesticular neoplasm suspected as malignant tumor. The tumor was pathologically identified as IgG4-RD of the left paratestis involving the epididymis and spermatic cord.

Conclusions: We present a first description of IgG4-RD in a patient with Wells syndrome and the ninth case of IgG4-RD in a scrotal organ, and discuss this very rare entity with reference to the literature.

Virtual slides: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/13000_2014_225.

Show MeSH
Related in: MedlinePlus