Abstract:
UNASSIGNED: Paratesticular fibrous pseudotumor (PFP) is a rare intrascrotal benign fibrous mass of uncertain aetiology, usually arising between testicular tunica layers and is supposed to be related to inflammatory reactive conditions. Because of morphological similarities to IgG4-related sclerosing fibro-inflammatory lesions, some authors recently postulated that PFP might belong to the IgG4-related disease (IgG4-RD) family. Considering the rarity of this lesion, only few cases have been reported in literature about the correlation between IgG4-RD and PFP. Management of PFP could be extremely challenging: due to the lack of typical clinical signs and the non-specific radiological characteristics, misapprehension does occur in the majority of cases, mainly because these intrascrotal mass may mimic testicular neoplasm, therefore leading to radical orchidectomy rather than a desirable testis-sparing surgery.
UNASSIGNED: Herein we report two cases of young males treated for PFP with histological feature of IgG4-RD. Patients underwent testicular sparing surgery. At 2-year follow-up no evidence of local or distant relapse nor testicular disorder was observed in both patients. An up-to-date review of the literature about the correlation between PFP and the IgG4-RD was carried out.
UNASSIGNED: PFP is an extremely rare condition with uncertain etiology being part of IgG4-RD family. Preoperative imaging mimics malignancy hence diagnosis is usually made by specimen analysis. Intraoperative frozen section is fundamental in order to guarantee conservative treatment that is feasible and safe after mid-term follow-up.
摘要:
■睾丸旁纤维假瘤(PFP)是一种病因不明的罕见的包囊内良性纤维团,通常出现在睾丸膜层之间,被认为与炎症反应性病症有关。由于与IgG4相关的硬化性纤维炎性病变的形态学相似性,一些作者最近推测PFP可能属于IgG4相关疾病(IgG4-RD)家族.考虑到这个病变的稀有性,关于IgG4-RD和PFP之间的相关性的文献报道很少。PFP的管理可能极具挑战性:由于缺乏典型的临床体征和非特异性放射学特征,大多数情况下确实会发生误解,主要是因为这些阴囊内肿块可能模仿睾丸肿瘤,因此导致根治性睾丸切除术,而不是理想的保留睾丸的手术。
■在此,我们报告了2例年轻男性治疗PFP的病例,其组织学特征为IgG4-RD。患者接受保留睾丸手术。在2年的随访中,两名患者均未观察到局部或远处复发或睾丸疾病的证据。对有关PFP和IgG4-RD之间的相关性的文献进行了最新综述。
■PFP是一种极其罕见的疾病,病因不确定,是IgG4-RD家族的一部分。术前成像模拟恶性肿瘤,因此通常通过标本分析进行诊断。术中冰冻切片是保证中期随访后保守治疗可行、安全的基础。