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Extramammary Paget Disease of the Scrotum
Applied Immunohistochemistry & Molecular Morphology ( IF 1.6 ) Pub Date : 2019-07-08 , DOI: 10.1097/pai.0000000000000789
Maryam Shabihkhani 1 , Pallavi Patil 2 , Belkiss Murati Amador 1 , Jose A Plaza 3 , Adeboye O Osunkoya 1, 4 , Kara A Lombardo 1 , Jonathan I Epstein 1, 5, 6 , Andres Matoso 1, 5, 6
Affiliation  

Extramammary Paget disease (EMPD) often involves apocrine gland-bearing locations including vulva and perianal area. EMPD of the scrotum is rare. Twenty patients were identified from the pathology files of 4 institutions between 2000 and 2018. Patients were 63- to 87-year-old (mean: 73 y) with a history of symptoms of between 4 months and 10 years. Two patients had a history of prostate cancer. Follow-up was available in 11 patients for a median of 71 months (range: 8 to 126 mo). Nine of 11 patients (82%) had positive margins, and 73% required reexcisions. Three patients had a focal dermal invasion, 1 of whom reportedly died of another etiology 25 months post diagnosis and 2 were disease-free at 24 and 68 months. No patient had inguinal lymphadenopathy. Two patients were alive with disease. Immunohistochemically, GATA3 and GCDFP15 were expressed in 6/6 cases, CK7 in 8/8 cases, and androgen receptor in 13/13 cases. HER2 was positive in 5/12 cases. PSA was positive in 1 patient who had a history of prostate cancer, whereas other prostate markers (NKX3.1 and prostein) were negative, and CK7 and GCDFP15 were positive, rendering primary EMPD diagnosis. Twelve other cases were negative for PSA and NKX3.1. In conclusion, EMPD of the scrotum has an insidious onset and its nonspecific symptoms can be misdiagnosed as dermatitis or fungal infection. Although localized EMPD has a favorable prognosis, the invasive disease is rare and did not predict metastasis or progression. Margins are frequently positive requiring reexcision. Occasionally, cases can be positive for PSA leading to diagnostic pitfalls.

中文翻译:

阴囊乳腺外佩吉特病

乳腺外佩吉特病 (EMPD) 通常涉及大汗腺部位,包括外阴和肛周区域。阴囊的 EMPD 很少见。从 2000 年至 2018 年 4 家机构的病理档案中确定了 20 名患者。患者年龄为 63 至 87 岁(平均:73 岁),症状史 4 个月至 10 年。两名患者有前列腺癌病史。11 名患者的随访时间中位数为 71 个月(范围:8 至 126 个月)。11 名患者中有 9 名 (82%) 切缘阳性,73% 需要再次切除。3 名患者发生局灶性皮肤浸润,据报道其中 1 人在诊断后 25 个月死于另一种病因,2 人在 24 个月和 68 个月时无病。没有患者出现腹股沟淋巴结肿大。两名患者因疾病而活着。免疫组化,GATA3和GCDFP15在6/6例中表达,CK7在8/8例中表达,雄激素受体在13/13例中表达。5/12 例 HER2 呈阳性。1 例有前列腺癌病史的患者 PSA 呈阳性,而其他前列腺标志物(NKX3.1 和 prostein)呈阴性,CK7 和 GCDFP15 呈阳性,初步诊断为 EMPD。其他 12 例 PSA 和 NKX3.1 均为阴性。总之,阴囊EMPD起病隐匿,其非特异性症状易误诊为皮炎或真菌感染。尽管局部 EMPD 具有良好的预后,但侵袭性疾病很少见,并且不能预测转移或进展。边距通常为正值,需要重新切除。有时,病例可能对 PSA 呈阳性,从而导致诊断错误。5/12 例 HER2 呈阳性。1 例有前列腺癌病史的患者 PSA 呈阳性,而其他前列腺标志物(NKX3.1 和 prostein)呈阴性,CK7 和 GCDFP15 呈阳性,初步诊断为 EMPD。其他 12 例 PSA 和 NKX3.1 均为阴性。总之,阴囊EMPD起病隐匿,其非特异性症状易误诊为皮炎或真菌感染。尽管局部 EMPD 具有良好的预后,但侵袭性疾病很少见,并且不能预测转移或进展。边距通常为正值,需要重新切除。有时,病例可能对 PSA 呈阳性,从而导致诊断错误。5/12 例 HER2 呈阳性。1 例有前列腺癌病史的患者 PSA 呈阳性,而其他前列腺标志物(NKX3.1 和 prostein)呈阴性,CK7 和 GCDFP15 呈阳性,初步诊断为 EMPD。其他 12 例 PSA 和 NKX3.1 均为阴性。总之,阴囊EMPD起病隐匿,其非特异性症状易误诊为皮炎或真菌感染。尽管局部 EMPD 具有良好的预后,但侵袭性疾病很少见,并且不能预测转移或进展。边距通常为正值,需要重新切除。有时,病例可能对 PSA 呈阳性,从而导致诊断错误。CK7 和 GCDFP15 阳性,初步诊断为 EMPD。其他 12 例 PSA 和 NKX3.1 均为阴性。总之,阴囊EMPD起病隐匿,其非特异性症状易误诊为皮炎或真菌感染。尽管局部 EMPD 具有良好的预后,但侵袭性疾病很少见,并且不能预测转移或进展。边距通常为正值,需要重新切除。有时,病例可能对 PSA 呈阳性,从而导致诊断错误。CK7 和 GCDFP15 阳性,初步诊断为 EMPD。其他 12 例 PSA 和 NKX3.1 均为阴性。总之,阴囊EMPD起病隐匿,其非特异性症状易误诊为皮炎或真菌感染。尽管局部 EMPD 具有良好的预后,但侵袭性疾病很少见,并且不能预测转移或进展。边距通常为正值,需要重新切除。有时,病例可能对 PSA 呈阳性,从而导致诊断错误。侵袭性疾病是罕见的并且不能预测转移或进展。边距通常为正值,需要重新切除。有时,病例可能对 PSA 呈阳性,从而导致诊断错误。侵袭性疾病是罕见的并且不能预测转移或进展。边距通常为正值,需要重新切除。有时,病例可能对 PSA 呈阳性,从而导致诊断错误。
更新日期:2019-07-08
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