Hussong J W, Brown M, Perkins S L, Dehner L P, Coffin C M
Department of Pathology, University of Utah Health Sciences Center and Primary Children's Medical Center, Salt Lake City 84113-1100, USA.
Mod Pathol. 1999 Mar;12(3):279-86.
Inflammatory myofibroblastic tumors (IMTs) are uncommon spindle cell proliferations that occur in the viscera and soft tissue of children and young adults. Their biologic potential is indeterminate: 25% of IMTs recur, and rare examples undergo malignant transformation (MT). This study investigates histologic features, DNA ploidy, and expression of apoptotic regulatory and oncogenic proteins in IMTs in an attempt to identify those deviances that might correlate with aggressive behavior or MT. Twenty-four formalin-fixed, paraffin-embedded IMTs for which clinical outcome was known were evaluated for cellularity, cytologic atypia, mitoses, ganglion-like cells, inflammatory infiltrate, DNA ploidy by flow cytometric examination, and bax, bcl-2, p53, and c-myc expression by immunohistochemical analysis. Sixteen (67%) of the IMTs did not recur, 6 (25%) recurred, and 2 (8%) underwent MT. Cellular atypia was observed in 69% of the cases without recurrence, 100% with recurrence, and 100% with MT. Ganglion-like cells were present in 56, 100, and 100% of the IMTs without recurrence, with recurrence, and with MT, respectively. There was no difference in the degree of cellularity, mitoses, or inflammatory infiltrate among the outcome groups. All of the tumors expressed bax, and none expressed c-myc. Two (8%) were immunoreactive for p53, one of which recurred and one of which underwent MT. bcl-2 expression was observed in 9 (37%) of the IMTs, with no difference among the three groups. Two IMTs were aneuploid, one of which underwent MT. Neither morphologic evaluation for cellularity, mitosis, or inflammatory infiltrate nor expression of bax or c-myc were useful for prediction of clinical outcome. The combination of atypia, ganglion-like cells, p53 expression and DNA ploidy analysis, however, might be useful to identify IMTs that might undergo MT or pursue a more aggressive clinical course with recurrences.
炎性肌纤维母细胞瘤(IMTs)是一种罕见的梭形细胞增殖性病变,发生于儿童和青年的内脏及软组织。其生物学潜能尚不确定:25%的IMTs会复发,少数病例会发生恶性转化(MT)。本研究调查IMTs的组织学特征、DNA倍体以及凋亡调节蛋白和致癌蛋白的表达,试图确定那些可能与侵袭性生物学行为或MT相关的偏差。对24例临床结局已知的福尔马林固定、石蜡包埋的IMTs进行评估,包括细胞密度、细胞异型性、核分裂象、神经节样细胞、炎性浸润,通过流式细胞术检测DNA倍体,以及通过免疫组化分析检测bax、bcl-2、p53和c-myc的表达。16例(67%)IMTs未复发,6例(25%)复发,2例(8%)发生MT。细胞异型性在未复发的病例中占69%,复发的病例中占100%,发生MT的病例中占100%。神经节样细胞在未复发、复发和发生MT的IMTs中分别占56%、100%和100%。不同结局组在细胞密度、核分裂象或炎性浸润程度方面无差异。所有肿瘤均表达bax,无一表达c-myc。2例(8%)p53免疫反应阳性,其中1例复发,1例发生MT。9例(37%)IMTs观察到bcl-2表达,三组之间无差异。2例IMTs为非整倍体,其中1例发生MT。细胞密度、核分裂象或炎性浸润的形态学评估以及bax或c-myc的表达均无助于预测临床结局。然而,异型性、神经节样细胞、p53表达和DNA倍体分析的联合应用可能有助于识别可能发生MT或具有更具侵袭性临床病程并伴有复发的IMTs。