肝脏孤立性纤维瘤临床病理特征及手术疗效并文献复习

doi:10.3877/cma.j.issn.2095-3232.2021.01.019

目的

探讨肝脏孤立性纤维瘤（SFT）的临床病理特点及手术疗效。

方法

回顾性分析2001年1月至2018年12月在西安交通大学第一附属医院接受根治性切除术的2例肝脏SFT患者，以及检索文献纳入49例肝脏SFT患者临床资料。本院2例患者均签署知情同意书，符合医学伦理学规定。分析纳入病例的一般情况、临床表现、组织学类型，采用免疫组化染色检测肿瘤Vim、CD34、CD99、desmin、Bcl-2和STAT6表达，总结其临床病理特征和手术疗效。

结果

共51例经病理确诊且有随访资料的肝脏SFT病例纳入研究。其中男23例，女28例；平均年龄（52±3）岁；临床表现为腹胀11例，低血糖7例，腹痛4例，癫痫1例，28例无明显临床症状。肿瘤均为单发且有完整包膜，血供丰富，质地韧，界限较清楚，剖面多为黄色。肿瘤直径（15.4±2.3） cm。组织学表现为瘤细胞多呈梭形或卵圆形，排列不规则，疏密不均，细胞核呈卵圆形，核仁小，瘤细胞间大量胶原纤维。免疫组化结果示CD34阳性率为94%（48/51），Vim为77%（39/51），Bcl-2为55% （28/51）。本组患者中位生存时间17.4个月，无复发病例报道。

结论

肝脏SFT临床少见，早期无明显症状，病灶较大时引起腹胀、腹痛，可伴有低血糖。病理检查是确诊的金标准，根治性切除是首选治疗方法，手术切除配合规律复查随访疗效确切。

关键词: 肝肿瘤, 孤立性纤维瘤, 病理状态, 体征和症状, 预后

Objective

To investigate the clinicopathological characteristics and surgical efficacy of liver solitary fibrous tumor (SFT).

Methods

Clinical data of 2 patients diagnosed with liver SFT who underwent radical resection in the First Affiliated Hospital of Xi'an Jiaotong University from January 2001 to December 2018 were retrospectively analyzed. In addition, 49 cases with liver SFT were included by literature searching. The informed consents of 2 patients of our hospital were obtained and the local ethical committee approval was received. Baseline data, clinical manifestations and histological types of the patients were analyzed. The expression levels of Vim, CD34, CD99, desmin, Bcl-2 and STAT6 of tumors were detected by immunohistochemical staining. Clinicopathological characteristics and surgical efficacy were summarized.

Results

A total of 51 patients pathologically diagnosed with liver SFT and complete follow-up data were included in this study. 23 cases were male and 28 female, aged (52±3) years on average. The clinical manifestations included abdominal distension in 11 cases, hypoglycemia in 7 cases, abdominal pain in 4 cases, epilepsy in 1 case, and no obvious clinical symptoms were observed in 28 cases. All the tumors were solitary with complete capsule, abundant blood supply, tough texture, clear boundary and yellow cross-section. The tumor diameter was (15.4±2.3) cm. Histological manifestations were spindle-shaped or ovoid tumor cells, irregular arrangement and uneven density, ovoid nucleus, small nucleoli, and a large amount of collagen fibers between tumor cells. Immunohistochemical staining revealed that the positive rate of CD34 was 94% (48/51), 77% (39/51) for Vim and 55% (28/51) for Bcl-2. The median survival time of patients in this group was 17.4 months. No recurrence was reported.

Conclusions

Liver SFT is rarely seen in clinical practice. No evident symptom is noted in the early stage. Large lesions may cause abdominal distension and pain, accompanied by hypoglycemia. Pathological examination is the gold standard for diagnosis. Radical resection is the primary choice of treatment. Surgical resection combined with regular follow-up yield exact clinical efficacy.

Key words: Liver neoplasms, Solitary fibrous tumors, Pathological conditions, signs and symptoms, Prognosis

图1 一例孤立性肝纤维瘤患者肝脏CT图

图2 一例孤立性肝纤维瘤患者肿瘤切除标本

图3 一例孤立性肝纤维瘤细胞HE及免疫组化染色（×200）

[1]	Bishop AJ, Zagars GK, Demicco EG, et al. Soft tissue solitary fibrous tumor: combined surgery and radiation therapy results in excellent local control[J]. Am J Clin Oncol, 2018, 41(1):81-85.
[2]	Davanzo B, Emerson RE, Lisy M, et al. Solitary fibrous tumor[J]. Transl Gastroenterol Hepatol, 2018(3):94.
[3]	Kottke-Marchant K, Hart WR, Broughan T. Localized fibrous tumor (localized fibrous mesothelioma) of the liver[J]. Cancer, 1989, 64(5):1096-1102.
[4]	Levine TS, Rose DS. Solitary fibrous tumour of the liver[J]. Histopathology, 1997, 30(4):396-397.
[5]	Guglielmi A, Frameglia M, Iuzzolino P, et al. Solitary fibrous tumor of the liver with CD34 positivity and hypoglycaemia[J].J Hepatobiliary Pancreat Surg, 1998, 5(2):212-216.
[6]	Lecesne R, Drouillard J, Le Bail B, et al. Localised fibrous tumor of the liver: imaging findings[J]. Eur Radiol, 1998, 8(1):36-38.
[7]	Bejarano PA, Blanco R, Hanto DW. Solitary fibrous tumor of the liver. a case report, river of the literature and differential diagnosis of spindle cell lesions of the liver[J]. Int J Surg Pathol, 1998, 6(1):93-100.
[8]	Moran CA, Ishak KG, Goodman ZD. Solitary fibrous tumor of the liver: a clinicopathologic and immunohistochemical study of nine cases[J]. Ann Diagn Pathol, 1998, 2(1):19-24.
[9]	Yilmaz S, Kirimlioglu V, Ertas E, et al. Giant solitary fibrous tumor of the liver with metastasis to the skeletal system successfully treated with trisegmentectomy[J]. Dig Dis Sci, 2000, 45(1):168-174.
[10]	Lin YT, Lo GH, Lai KH, et al. Solitary fibrous tumor of the liver[J]. Zhonghua Yi Xue Za Zhi (Taipei), 2001, 64(5):305-309.
[11]	Neeff H, Obermaier R, Technau-Ihling K, et al. Solitary fibrous tumour of the liver: case report and review of the literature[J]. Langenbecks Arch Surg, 2004, 389(4):293-298.
[12]	Chithriki M, Jaibaji M, Vandermolen R. Solitary fibrous tumor of the liver with presening symptoms of hypoglycemic coma[J]. Am Surg, 2004, 70(4):291-293.
[13]	Vennarecci G, Ettorre GM, Giovannelli L, et al. Solitary fibrous tumor of the liver[J]. J Hepatobiliary Pancreat Surg, 2005, 12(4):341-344.
[14]	Lehmann C, Mourra N, Tubiana JM, et al. Solitary fibrous tumor of the liver[J]. J Radiol, 2006, 87(2 Pt 1):139-142.
[15]	Nath DS, Rutzick AD, Sielaff TD. Solitary fibrous tumor of the liver[J]. AJR Am J Roentgenol, 2006, 187(2):W187-190.
[16]	Terkivatan T, Kliffen M, Johannes HW, et al. Giant solitary fibrous tumour of the liver[J]. World J Surg Oncol, 2006(4):81.
[17]	Chan G, Horton PJ, Thyssen S, et al. Malignant transformation ofa solitary fibrous tumor of the liver and intractable hypoglycaemia[J]. J Hepatobiliary Pancreat Surg, 2007, 14(6):595-599.
[18]	Obuz F, Secil M, Sagol O, et al. Ultrasonography and magnetic resonance imaging findings of solitary fibrous tumor of the liver[J]. Tumori, 2007, 93(1):100-102.
[19]	Perini MV, Herman P, D'Albuquerque LA, et al. Solitary fibrous tumor of the liver: report of a rare case and review of the literature[J]. Int J Surg, 2008, 6(5):396-399.
[20]	Korkolis DP, Apostolaki K, Aggeli C, et al. Solitary fibrous tumor of the liver expressing CD34 and vimentin: a case report[J]. World J Gastroenterol, 2008, 14(40):6261-6264.
[21]	Chen JJ, Ong SL, Richards C, et al. Inaccuracy of fine-needle biopsy in the diagnosis of solitary fibrous tumour of the liver[J]. Asian J Surg, 2008, 31(4):195-198.
[22]	Novais P, Robles-Medranda C, Pannain VL, et al. Solitary fibrous liver tumor: is surgical approach the best option?[J]. J Gastrointestin Liver Dis, 2010, 19(1):81-84.
[23]	Brochard C, Michalak S, Aubé C, et al. A not so solitary fibrous tumor of the liver[J]. Gastroenterol Clin Biol, 2010, 34(12): 716-720.
[24]	Haddad A, Karras R, Fraiman M, et al. Solitary fibrous tumor of the liver[J]. Am Surg, 2010, 76(7):E78-79.
[25]	Peng L, Liu Y, Ai Y, et al. Skull base metastases from a malignant solitary fibrous tumor of the liver. a case report and literature review[J]. Diagn Pathol, 2011(6):127.
[26]	Sun K, Lu JJ, Teng XD, et al. Solitary fibrous tumor of the liver:a case report[J]. World J Surg Oncol, 2011(9):37.
[27]	Patra S, Vij M, Venugopal K, et al. Hepatic solitary fibrous tumor: a report of a rare case[J]. Indian J Pathol Microbiol, 2012, 55(2):236-238.
[28]	Belga S, Ferreira S, Lemos MM. A rare tumor of the liver with a sudden presentation[J]. Gastroenterology, 2012, 143(3):e14-15.
[29]	Morris R, McIntosh D, Helling T, et al. Solid fibrous tumor of the liver: a case in pregnancy[J]. J Matern Fetal Neonatal Med, 2012, 25(6):866-868.
[30]	Debs T, Kassir R, Amor IB, et al. Solitary fibrous tumor of the liver: report of two cases and review of the literature[J]. Int J Surg, 2014, 12(12):1291-1294.
[31]	Song L, Zhang W, Zhang Y. (18) F-FDG PET/CT imaging of malignant hepatic solitary fibrous tumor[J]. Clin Nucl Med, 2014, 39(7):662-664.
[32]	Teixeira F Jr, de Freitas Perina AL, de Oliveira Mendes G, et al. Fibrous solitary tumour of the liver[J]. J Gastrointest Cancer, 2014, 45(Suppl 1):216-217.
[33]	Du EH, Walshe TM, Buckley AR. Recurring rare liver tumor presenting with hypoglycemia[J]. Gastroenteroloy, 2015, 148(2): e11-13.
[34]	Beltrán MA. Solitary fibrous tumor of the liver: a review of the current knowledge and report of a new case[J]. J Gastrointest Cancer, 2015, 46(4):333-342.
[35]	Feng LH, Dong H, Zhu YY, et al. An update on primary hepatic solitary fibrous tumor: an examination of the clinical and pathological features of four case studies and a literature review[J]. Pathol Res Pract, 2015, 211(12):911-917.
[36]	Silvanto A, Karanjia ND, Bagwan IN. Primary hepatic solitary fibrous tumor with histologically benign and malignant areas[J]. Hepatobiliary Pancreat Dis Int, 2015, 14(6):665-668.
[37]	Maccio L, Bonetti LR, Siopis E, et al. Malignant metastasizing solitary fibrous tumors of the liver: a report of three cases[J]. Pol J Pathol, 2015, 66(1):72-76.
[38]	Makino Y, Miyazaki M, Shigekawa M, et al. Solitary fibrous tumor of the liver from development to resection[J]. Intern Med, 2015, 54(7):765-770.
[39]	Dey B, Gochhait D, Kaushal G, et al. Solitary fibrous tumor of the liver: a rare tumor in a rarer location[J]. Rare Tumors, 2016, 8(3):6403.
[40]	Degnan AJ, Lee KK, Minervini MI, et al. Metastatic extrapleural malignant solitary fibrous tumor presenting with hypoglycemia (Doege-Potter syndrome)[J]. Radiol Case Rep, 2016, 12(1):113-119.
[41]	Mačák J, Buzrla P, Dvořáčková J, et al. Hypoglycemia in a solitary fibrous tumor of the liver[J]. Cesk Patol, 2016, 52(1):41-44.
[42]	Chen N, Slater K. Solitary fibrous tumour of the liver-report on metastasis and local recurrence of a malignant case and review of literature[J]. World J Surg Oncol, 2017, 15(1):27.
[43]	Sun Z, Ding Y, Jiang Y, et al. Ex situ hepatectomy and liver autotransplantation for a treating giant solitary fibrous tumor: a case report[J]. Oncol Lett, 2019, 17(1):1042-1052.
[44]	Yugawa K, Yoshizumi T, Mano Y, et al. Solitary fibrous tumor in the liver: case report and literature review[J]. Surg Case Rep, 2019, 5(1): 68.
[45]	张静, 许敬尧, 盛彩霞. 肝脏巨大孤立性纤维瘤[J]. 临床与实验病理学杂志, 2002, 18(2):213-214.
[46]	冉鹏程, 李进兵, 杨海峰, 等. 肝镰状韧带孤立性纤维瘤一例[J]. 临床误诊误治, 2008, 21(10):81.
[47]	潘卫东, 周静, 徐见亮, 等. 肝脏巨大恶性孤立性纤维瘤伴癫痫一例[J]. 中华肝胆外科杂志, 2012, 18(2):152-153.
[48]	李小祺, 崔培元. 肝脏孤立性纤维性肿瘤1例并文献复习[J]. 蚌埠医学院学报, 2016, 41(11):1492-1495.
[49]	Gholami S, Cassidy MR, Kirane A, et al. Size and location are the most important risk factors for malignant behavior in resected solitary fibrous tumors[J]. Ann Surg Oncol, 2017, 24(13):3865-3871.
[50]	Grech N, Ellul E, Spiteri N, et al. Solitary fibrous tumour a rare cause of acute abdomen[J]. Ann Ital Chir, 2017(6):S2239253X17026767.
[51]	Gomez FD, Robin L, Jakubowicz D, et al. Solitary fibrous tumor of the retroperitoneum with urinary symptoms revealing a Doege-Potter's syndrome[J]. Prog Urol, 2019, 29(3):136-137.
[52]	Liu X, Zhu W, Zhou X, et al. 18F-FDG PET/CT imaging findings of multiple solitary fibrous tumor: a case report[J]. Medicine, 2019, 98(32):e16743.
[53]	Liu Y, Wang Q, Zhang T, et al. MR imaging of intracranial solitary fibrous tumor: a retrospective study of 7 cases[J]. Afr Health Sci, 2018, 18(3):799-806.
[54]	Park HK, Yu DB, Sung M, et al. Molecular changes in solitary fibrous tumor progression[J]. J Mol Med, 2019, 97(10):1413-1425.
[55]	Kayani B, Sharma A, Sewell MD, et al. A review of the surgical management of extrathoracic solitary fibrous tumors[J]. Am J Clin Oncol, 2018, 41(7):687-694.
[56]	Yamada Y, Kohashi K, Kinoshita I, et al. Clinicopathological review of solitary fibrous tumors: dedifferentiation is a major cause of patient death[J]. Virchows Arch, 2019, 475(4):467-477.
[57]	Velayati S, Erinjeri JP, Brody LA, et al. Safety and efficacy of hepatic artery embolization in treating solitary fibrous tumor metastatic to the liver[J]. Sarcoma, 2019:3060658.

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Clinicopathological characteristics and surgical efficacy of liver solitary fibrous tumor and literature review

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Clinicopathological characteristics and surgical efficacy of liver solitary fibrous tumor and literature review