胃肠道钙化性纤维性肿瘤5例临床病理分析并文献复习

doi:10.16150/j.1671-2870.2026.01.010

摘要/Abstract

摘要：

目的：探讨发生在胃肠道的钙化性纤维性肿瘤（calcifying fibrous tumor，CFT）的病理及免疫组化特征、诊断和鉴别诊断要点。方法：回顾性收集黄山市人民医院2023年10月至2024年11月连续入组的胃肠道CFT病例5例，分析患者的临床及病理资料，并复习相关文献。结果：5例患者中，男性2例，女性3例，年龄为49~68岁，4例单发（位于胃体3例、胃底部1例），1例为胃体、回肠及盆腹壁多发性肿块。镜下特征表现为大量玻璃样变的胶原纤维背景下夹杂少量纤维母细胞及血管，细胞无异型，核分裂象罕见，其中3例伴有砂砾体或营养不良性钙化。免疫组化检测示，肿瘤细胞表达Vimentin（5/5）、SMA（2/5）、CD34（1/5）和CD117（2/5），均不表达DOG-1、Desmin、S-100，Ki-67增殖指数约为1%。5例患者随访6~18个月，均无肿瘤复发和转移。结论：胃肠道CFT是一种相对罕见的良性肿瘤，需要与胃肠道间质瘤、炎症性肌纤维母细胞瘤等其他间叶源性肿瘤相鉴别，特征性的玻璃样变性及营养不良性钙化等组织形态有助于诊断。

关键词: 钙化性纤维性肿瘤, 组织病理, 胃肠道间质瘤, 炎症性肌纤维母细胞瘤

Abstract:

Objective To investigate the pathological and immunohistochemical features, diagnosis, and key points of differential diagnosis of calcifying fibrous tumors (CFT) occurring in the gastrointestinal tract. Methods The clinical and pathological data of 5 consecutive patients with gastrointestinal CFT admitted to Huangshan People's Hospital from October 2023 to November 2024 were retrospectively collected. The clinical and pathological features of gastrointestinal CFT were analyzed by HE staining and immunohistochemical staining, and relevant literature was reviewed. Results Among the 5 patients, there were 2 males and 3 females, aged between 49 and 68 years old. Four cases with single lesion occurred in the gastric body (3 cases) and fundus (1 cases), and one case involved multiple masses in the gastric body, ileum, and pelvic abdominal wall. Microscopically, the tumors exhibited abundant hyalinized collagen fibers mixed with a small number of fibroblasts and blood vessels. The cells showed no atypia, and mitotic figures were rare. Three cases were accompanied by psammoma bodies or dystrophic calcifications. Immunohistochemical staining showed that tumor cells expressed Vimentin (5/5), SMA (2/5), CD34 (1/5), and CD117 (2/5), but no DOG-1, Desmin, and S-100. The Ki-67 proliferation index was approximately 1%. All 5 patients were followed up for 6-18 months, and no tumor recurrence or metastasis was observed. Conclusions Gastrointestinal CFT is a relatively rare benign tumor that needs to be differentiated from other mesenchymal tumors, such as gastrointestinal stromal tumors and inflammatory myofibroblastic tumors. Characteristic histomorphological features, such as hyaline degeneration and dystrophic calcification, are helpful for diagnosis.

Key words: Calcifying fibrous tumor, Histopathology, Gastrointestinal stromal tumors, Inflammatory myofibroblastic tumor

中图分类号:

唐婷, 王笛, 方威. 胃肠道钙化性纤维性肿瘤5例临床病理分析并文献复习[J]. 诊断学理论与实践, 2026, 25(01): 71-77.

TANG Ting, WANG Di, FANG Wei. Clinical and pathological analysis of 5 cases of calcifying fibrous tumor in gastrointestinal tract and literature review[J]. Journal of Diagnostics Concepts & Practice, 2026, 25(01): 71-77.

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参考文献 22

[1]	ROSENTHAL N S, ABDUL-KARIM F W. Childhood fibrous tumor with psammoma bodies. Clinicopathologic features in two cases[J]. Arch Pathol Lab Med, 1988, 112(8):798-800. pmid: 3395217
[2]	FLETCHER C D M, UNNI K, MERTENS F, et al. World Health Organization Classification of tumours. Pathology and genetics of tumours of soft tissue and bone[M]. Lyon France,IARC Press, 2002.
[3]	CHORTI A, PAPAVRAMIDIS T S, MICHALOPOULOS A. Calcifying fibrous tumor: Review of 157 patients reported in international literature[J]. Medicine (Baltimore), 2016, 95(20):e3690. doi: 10.1097/MD.0000000000003690 URL
[4]	张雷, 魏建国, 方三高, 等. 钙化性纤维性肿瘤32例临床病理学分析[J]. 中华病理学杂志, 2020, 49(2):129-133.
	ZHANG L, WEI J G, FANG S G, et al. Calcifying fibrous tumor: A clinicopathological analysis of 32 cases[J]. Chin J Pathol, 2020, 49(2):129-133.
[5]	FETSCH J F, MONTGOMERY E A, MEIS J M. Calcifyi-ng fibrous pseudotumor[J]. Am J Surg Pathol, 1993, 17(5):502-508. doi: 10.1097/00000478-199305000-00010 URL
[6]	NASCIMENTO A F, RUIZ R, HORNICK J L, et al. Calcifying fibrous 'pseudotumor': Clinicopathologic study of 15 cases and analysis of its relationship to inflammatory myofibroblastic tumor[J]. Int J Surg Pathol, 2002, 10(3):189-196. doi: 10.1177/106689690201000304 pmid: 12232572
[7]	LARSON B K, BALZER B, GOLDWASSER J, et al. Calcifying fibrous tumor: an unrecognized IgG4---related disease?[J]. APMIS, 2015, 123(1):72-76. doi: 10.1111/apm.2014.123.issue-1 URL
[8]	VAN DORPE J, ECTORS N, GEBOES K, et al. Is calcif-ying fibrous pseudotumor a late sclerosing stage of inflammatory myofibroblastic tumor?[J]. Am J Surg Pathol, 1999, 23(3):329-335. doi: 10.1097/00000478-199903000-00013 URL
[9]	LEE D, SUH Y L, LEE S K. Calcifying fibrous pseudotumour arising in a gastric inflammatory myofibroblastic tumour[J]. Pathology, 2006, 38(6):588-591. doi: 10.1080/00313020601023997 URL
[10]	PEZHOUH M K, REZAEI M K, SHABIHKHANI M, et al. Clinicopathologic study of calcifying fibrous tumor of the gastrointestinal tract: a case series[J]. Hum Pathol, 2017,62:199-205.
[11]	AGAIMY A, BIHL M P, TORNILLO L, et al. Calcifying fibrous tumor of the stomach: Clinicopathologic and molecular study of seven cases with literature review and reappraisal of histogenesis[J]. Am J Surg Pathol, 2010, 34(2):271-278. doi: 10.1097/PAS.0b013e3181ccb172 pmid: 20090503
[12]	李璐, 王淑妍, 邓茜, 等. 胃钙化性纤维性肿瘤24例临床病理分析[J]. 临床与实验病理学杂志, 2022, 38(2):211-213.
	LI L, WANG S Y, DENG Q, et al. Clinicopathological analysis of 24 cases of calcific fibrous tumor of stomach[J]. J Clin Exp pathol, 2022, 38(2):211-213.
[13]	李红娜, 毛昕, 胡倩岚, 等. 回肠钙化性纤维性肿瘤1例[J]. 诊断病理学杂志, 2022, 29(8):782-783,封3.
	LI H N, MAO X, HU Q L, et al. Calcific fibrous tumor of ileum: a case report[J]. J Diag Pathol, 2022, 29(8):782-783,i3.
[14]	WANG J, AO W, MAO G, et al. Gastric calcifying fibrous tumors: Computed tomography findings and clinical manifestations[J]. Medicine (Baltimore), 2021, 100(5):e23334. doi: 10.1097/MD.0000000000023334 URL
[15]	杨朝武, 王娟. 腹部钙化性纤维性肿瘤CT及MRI诊断价值[J]. 影像研究与医学应用, 2023, 7(9):174-176,180.
	YANG C W, WANG J. Diagnostic value of CT and MRI in calcifying fibrous tumor of abdomen[J]. J Imaging Res Med Appl, 2023, 7(9):174-176,180.
[16]	唐晓婷, 刘振民, 房太勇. 胃钙化性纤维性肿瘤2例并文献复习[J]. 中华灾害救援医学, 2024, 11(11):1258-1262.
	TANG X T, LIU Z M, FANG T Y. Two cases of gastric calcified fibrous tumors with literature review[J]. Chin J Disaster Med, 2024, 11(11):1258-1262.
[17]	杜志华, 余嘉惠, 林晓丰, 等. 胃肠道钙化性纤维性肿瘤17例临床病理分析[J]. 诊断病理学杂志, 2024, 31(3):208-212.
	DU Z H, YU J H, LIN X F, et al. Calcifying fibrous tumor of the gastrointestinal tract:a clinicopathological analysis of 17 cases[J]. J Diag Pathol, 2024, 31(3):208-212.
[18]	高珊, 易慕华, 刘涛, 等. 胃钙化性纤维性肿瘤的诊断过程[J]. 巴楚医学, 2024, 7(2):27-29.
	GAO S, YI M H, LIU T, et al. Diagnosis of gastric calcif-ying fibrous tumor[J]. Bachu Med J, 2024, 7(2):27-29.
[19]	彭金榜, 林佳佳, 叶丽萍, 等. 胃钙化性纤维性假瘤2例并文献复习[J]. 胃肠病学, 2014(11):703-704.
	PENG J B, LIN J J, YE L P, et al. Gastric calcifying fibrous pseudotumor: Report of two cases and review of literature[J]. Chin J Gastroenterol, 2014(11):703-704.
[20]	TURBIVILLE D, ZHANG X. Calcifying fibrous tumor of the gastrointestinal tract: A clinicopathologic review and update[J]. World J Gastroenterol, 2020, 26(37):5597-5605. doi: 10.3748/wjg.v26.i37.5597 URL
[21]	朱常琰, 王修远, 胡玉秋, 等. 胃大弯侧外生性浆膜层下钙化性纤维性肿瘤1例[J]. 消化肿瘤杂志(电子版), 2024, 16(3):385-388.
	ZHU C Y, WANG X Y, HU Y Q, et al. Exogenous subserosal calcifying fibrous tumor in gastric greater curvature:a case report[J]. J Dig Oncol(Electronic Version), 2024, 16(3):385-388.
[22]	周影, 陈百胜, 杨丹琪, 等. 胃钙化性纤维性肿瘤的内镜表现及病理特征[J]. 中国医药指南, 2020, 18(28):4-6.
	ZHOU Y, CHEN B S, YANG D Q, et al. Endoscopic manifestations and pathological features of gastric calcif-ying fibrous tumors[J]. Guide Chin Med, 2020, 18(28):4-6.

Case	Age (Year)	Gender	Location	Clinical manifestations	Gastroscopic findings	Imaging findings	Treatment methods
1	49	Female	Subserosa of gastric body, subserosa of small intestinal wall, abdominopelvic wall and mesentery	Nausea and discomfort	Not performed	CT: Oval soft tissue density mass under the serosa of the gastric fundus, with clear and smooth boundaries. On plain scan, the parenchymal density of the tumor was higher than that of the normal gastric wall, with atypical calcification in the tumor, and mild progressive enhancement on enhanced scan	Laparoscopic resection of gastric lesion, resection of ileal lesion and resection of intra-abdominal mass
2	54	Female	Gastric fundus	Discovered during physical examination	Submucosal protrusion with smooth surface mucosa, accompanied by hyperemia and focal erosion	CT: No abnormality reported	Endoscopic submucosal dissection
3	68	Male	Gastric body	Discovered during physical examination	Submucosal protrusion with smooth surface mucosa	CT: Uniform enhancement after enhanced scan	Endoscopic submucosal dissection
4	53	Male	Gastric body	Discovered during physical examination	Submucosal protrusion with hyperemia, macular appearance and focal erosion	CT: No abnormality reported	Endoscopic submucosal dissection
5	68	Female	Gastric body	Upper abdominal pain accompanied by nausea and vomiting	Submucosal protrusion with smooth surface mucosa	CT: Mild enhancement after enhanced scan	Endoscopic full-thickness resection

Case	Disease location	Solitary / Multiple	Size（cm）	Gross	Pathological morphology	Psammoma bodies	"Cuff-like" structure	Immunophenotype
1	Subserosa of gastric body, subserosa of small intestinal wall, abdominopelvic wall and mesentery	Multiple	1.1-2.0	Well-circumscribed, grayish-white cut surface, firm or hard consistency	Hyaline degeneration of collagen fibers with a small amount of non-atypical fibroblasts	Present	Present	CD34 （partially+），CD117（partially+），DOG-1（-），S-100（-），SMA（partially+），Desmin（-）
2	Submucosa of gastric fundus	Solitary	0.8	Well-circumscribed, grayish-white cut surface, hard consistency	Hyaline degeneration of collagen fibers with a small amount of non-atypical fibroblasts	Absent	Absent	CD34（-），CD117（-），DOG-1（-），S-100 （-），SMA（-），Desmin（-）
3	Submucosa of gastric body	Solitary	1.0	Well-circumscribed, grayish-white cut surface, hard consistency	Hyaline degeneration of collagen fibers with a small amount of non-atypical fibroblasts	Absent	Absent	CD117（-），CD34（-），Desmin（-），DOG-1（-）， SMA（-），S-100（-）
4	Submucosa of gastric body	Solitary	1.7	Well-circumscribed, grayish-white cut surface, moderate consistency	Hyaline degeneration of collagen fibers with a small amount of non-atypical fibroblasts	Present	Present	CD117（-），DOG-1（-），CD34（-），Desmin（-）， SMA（-），S-100（-）
5	Submucosa of gastric body	Solitary	1.7	Well-circumscribed, grayish-white cut surface, moderate consistency	Hyaline degeneration of collagen fibers with a small amount of non-atypical fibroblasts	Present	Present	CD34（-），CD117(scattered+)，DOG-1（-），Desmin（-），S-100（-），SMA（+）