Department of Pathology, University of Pittsburgh, PA, USA; Department of Pathology, Ohio State University, Columbus, OH, USA.
Department of Pathology, Ohio State University, Columbus, OH, USA.
Pathol Res Pract. 2022 Nov;239:154164. doi: 10.1016/j.prp.2022.154164. Epub 2022 Oct 11.
Osteoclast-type giant cell-rich carcinomas (OGCRCs) of urinary bladder are extremely rare, aggressive tumors that are often diagnosed as undifferentiated carcinomas. The morphology overlaps with other giant cell-rich benign and malignant bladder lesions. Little is known about the pathogenesis and clinical management of this aggressive variant. The aim of this study was to review clinico-pathologic features, and survival characteristics in a series of OGCRCs.
Five cases of OGCRCs of bladder were retrospectively reviewed. Clinical presentation, histomorphology, ancillary tests, treatment and follow-up data were retrieved and analyzed.
All patients were adult males (age range 63-86 years) and presented with painless gross hematuria. All cases showed biphasic morphology with polygonal to epithelioid to spindle mononuclear cells (MCs) and scattered multinucleated osteoclast-like giant cells (OGCs). Background urothelium showed urothelial carcinoma in-situ (CIS) (4/5) and/or invasive urothelial carcinoma (UC) (2/5) and invasive high-grade papillary urothelial carcinoma (PUC) (2/5). MCs showed focal expression of at least one epithelial marker and focal/diffuse expression of urothelial markers. OGCs were positive only for histiocytic markers. Oncomine test showed presence of p53 mutation (p.R282W) in case 3. Pathologic stage was T1 (n = 3), T2b (n = 1) and T3a (n = 1). 2/5 patients died of disease within 3 years of diagnosis.
OGCRC is an extremely rare and potentially aggressive malignant neoplasm of bladder. Most cases have associated conventional in-situ or invasive UC supporting undifferentiated or de-differentiated nature of this neoplasm. Surgery should be considered given the potential for aggressive behavior. However optimal treatment for OGCRCs remains unknown.
膀胱癌中的破骨细胞样巨细胞丰富型癌(OGCRC)是一种极其罕见的侵袭性肿瘤,通常被诊断为未分化癌。其形态与其他富含巨细胞的良性和恶性膀胱病变重叠。对于这种侵袭性变体的发病机制和临床管理知之甚少。本研究的目的是回顾一系列 OGCRC 的临床病理特征和生存特征。
回顾性分析 5 例膀胱 OGCRC 病例。检索并分析了临床表现、组织形态学、辅助检查、治疗和随访资料。
所有患者均为成年男性(年龄 63-86 岁),表现为无痛性肉眼血尿。所有病例均表现为双相形态,有多边形、上皮样至梭形单核细胞(MC)和散在的多核破骨样巨细胞(OGC)。背景尿路上皮显示原位尿路上皮癌(CIS)(4/5)和/或浸润性尿路上皮癌(UC)(2/5)和浸润性高级别乳头状尿路上皮癌(PUC)(2/5)。MC 至少有一种上皮标志物的局灶性表达和尿路上皮标志物的局灶性/弥漫性表达。OGC 仅对组织细胞标志物呈阳性。Oncomine 检测显示病例 3 存在 p53 突变(p.R282W)。病理分期为 T1(n=3)、T2b(n=1)和 T3a(n=1)。5 例中有 2 例在诊断后 3 年内死于疾病。
OGCRC 是一种极其罕见的、具有潜在侵袭性的膀胱恶性肿瘤。大多数病例伴有常规原位或浸润性 UC,支持这种肿瘤的未分化或去分化性质。鉴于其潜在的侵袭性行为,应考虑手术。然而,OGCRC 的最佳治疗方法仍不清楚。