仅蒂与肝相连的罕见巨大肝间叶错构瘤1例报道并文献复习_CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY

Authors：

秦旭东 ¹ , 廖昱 ¹ , 赵肖欢 ¹ , 王维鳌 ¹ , 郑泽兵 ¹ , 汤成艳 ¹ , 祝代威 ¹ , 杜青 ¹ , 李红艳 ² , 刘远梅 ¹ ,  金祝 ¹

1. ;
2. ;

Corresponding author：

金祝, Email: 915884700@qq.com

Keywords：

肝间叶错构瘤; 蒂连接; 儿童

DOI：

10.7507/1007-9424.202304050

Video：

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目的探讨小儿肝脏间叶错构瘤（mesenchymal hamartoma of liver，MHL）的诊治方法，提高术前诊断率。方法对2021年11月遵义医科大学附属医院收治的1例5岁女性腹腔占位患儿的临床表现、诊断方法、治疗方案、术后情况及病理结果进行回顾性分析，并进行相关文献复习，总结该疾病的诊治方法。结果本例患儿术前检查无特异性，相关检查均未发现肿瘤与肝脏相连接，术前诊断困难，经手术探查发现肿瘤仅由一条索状蒂与肝脏相连接，手术完整切除肿瘤，手术顺利，术后康复出院，术后病理诊断为MHL，出院后随访6个月无复发及恶变。文献复习共32例患儿，其中男25例，女7例；肿瘤仅累及肝右叶21例，累及右叶及左叶4例，仅累及尾状叶3例，仅累及左叶4例；肿瘤与肝脏相融合26例，带蒂与肝脏相连接6例。术前实验室检查部分患儿AFP（9例）或肝功能（AST、ALT，5例）异常，术后均逐渐下降至正常值；主要辅助检查为彩超、CT及MRI，检查发现肿瘤体积普遍较大，肿瘤长径5～20 cm、平均13.4 cm。32例均行手术切除，术前明确诊断为MHL 5例（其中1例术前行穿刺活检证实），术后病理诊断均为MHL；术后恢复良好，其中14例进行了术后随访，随访时间1个月～10年不等，中位随访时间为12个月，均无复发。结论对于体积较大的腹腔肿瘤或肝脏肿瘤，在无特异性的检验及检查结果时，应考虑为MHL。手术完全切除是治疗MHL的方法，预后较好，由于肿瘤有复发及恶变的可能性，故术后应定期随访复查。

Citation： 秦旭东, 廖昱, 赵肖欢, 王维鳌, 郑泽兵, 汤成艳, 祝代威, 杜青, 李红艳, 刘远梅, 金祝. 仅蒂与肝相连的罕见巨大肝间叶错构瘤1例报道并文献复习. CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY, 2023, 30(9): 1104-1108. doi: 10.7507/1007-9424.202304050 Copy

1.	于啸, 辛涛. 新生儿肝脏间叶性错构瘤1例. 医学影像学杂志, 2018, 28(4): 654, 658.
2.	相一, 芶欣, 周长升, 等. 肝间叶错构瘤恶变为肉瘤1例报告. 贵州医药, 2017, 41(4): 412-413, 封3.
3.	王宇蔚. 儿童肝左叶巨大间叶错构瘤1例. 中国卫生标准管理, 2016, 7(23): 52-54.
4.	侯志彬, 王春祥, 詹江华, 等. 《请您诊断》病例112答案: 小儿肝脏罕见非典型囊性间叶性错构瘤. 放射学实践, 2016, 31(7): 675-676.
5.	司友娇, 冯卫华, 华辉, 等. 肝脏间叶性错构瘤的CT表现(附3例报告并文献复习). 临床放射学杂志, 2014, 33(8): 1286-1288.
6.	冯平勇, 高洁. 肝脏间叶性错构瘤一例. 临床放射学杂志, 2013, 32(8): 1174-1175.
7.	王小鹏, 陈卫霞, 陈军法, 等. 小儿外生性肝脏间叶性错构瘤1例. 华西医学, 2008, 23(6): 1476-1477.
8.	张再重, 王烈, 王瑜, 等. 肝间叶性错构瘤诊断与治疗(附2例报告并文献复习). 临床军医杂志, 2008, 36(1): 156-159.
9.	Le LT, Phan HT, Nguyen TS, et al. Infantile mesenchymal hamartoma of the liver with elevated alpha fetoprotein. BJR Case Rep, 2021, 7(3): 20200196.
10.	Gray SC, Pienaar JA, Sofianos Z, et al. Hepatic mesenchymal hamartoma: an uncommon but important paediatric diagnosis. SA J Radiol, 2020, 24(1): 1891.
11.	Khan MR, Binkovitz LA, Smyrk TC, et al. Mesenchymal hamartoma in children: a diagnostic challenge. Case Rep Pediatr, 2019, 2019: 4132842.
12.	El Demellawy D, Lee JY, McDonell L, et al. Atypical hepatic mesenchymal hamartoma: histologic appearance, immunophenotype, and molecular findings. Pediatr Dev Pathol, 2019, 22(4): 365-369.
13.	Rahadiani N, Stephanie M, Putra J. Recurrent hepatic mesenchymal hamartoma with osseous metaplasia. Liver Int, 2018, 38(10): 1875.
14.	Berte N, Filfilan A, Mainard L, et al. Co-existing infantile hepatic hemangioma and mesenchymal hamartoma in a neonate. J Surg Case Rep, 2018, 2018(1): rjx260.
15.	Liao W, Zhang B, Zhang W, et al. A 4 and a half years old boy with mesenchymal hamartomas in the left lateral lobe of the liver: a case report and literature review. Medicine (Baltimore), 2017, 96(31): e7281.
16.	Sen D, Gulati YS, Majumder A, et al. Hepatic cystic mesenchymal hamartoma. Med J Armed Forces India, 2015, 71(Suppl 2): S574-S577.
17.	Patel SR, Misra V, Verma K, et al. Benign hepatic mesenchymal hamartoma (HMH) -a case report. J Clin Diagn Res, 2014, 8(3): 119-120.
18.	Chandramouleeswari K, Anita S, Shivali B. Mesenchymal hamartoma of the liver: a case report. J Clin Diagn Res, 2012, 6(9): 1552-1554.
19.	Orłowski A, Bręborowicz D. Mesenchymal hamartoma of the liver-case report and short literature overview. Pol J Pathol, 2011, 62(2): 108-112.
20.	Anil G, Fortier M, Low Y. Cystic hepatic mesenchymal hamartoma: the role of radiology in diagnosis and perioperative management. Br J Radiol, 2011, 84(1001): e91-e94.
21.	Kodandapani S, Pai MV, Kumar V, et al. Prenatal diagnosis of congenital mesenchymal hamartoma of liver: a case report. Case Rep Obstet Gynecol, 2011, 2011: 932583.
22.	Shintaku M, Watanabe K. Mesenchymal hamartoma of the liver: a proliferative lesion of possible hepatic stellate cell (Ito cell) origin. Pathol Res Pract, 2010, 206(7): 532-536.
23.	Cornette J, Festen S, van den Hoonaard TL, et al. Mesenchymal hamartoma of the liver: a benign tumor with deceptive prognosis in the perinatal period. Case report and review of the literature. Fetal Diagn Ther, 2009, 25(2): 196-202.
24.	Vázquez-Lima I, Vázquez JL, Gallego M, et al. Torsed pedunculated hepatic hamartoma. Pediatr Radiol, 2009, 39(1): 62-65.
25.	Baboiu OE, Saal H, Collins M. Hepatic mesenchymal hamartoma: cytogenetic analysis of a case and review of the literature. Pediatr Dev Pathol, 2008, 11(4): 295-299.
26.	Steiner MA, Giles HW. Mesenchymal hamartoma of the liver demonstrating peripheral calcification in a 12-year-old boy. Pediatr Radiol, 2008, 38(11): 1232-1234.
27.	Mansour A, Qandeel M, Sughayer M, et al. Solid (stromal predominance) hepatic mesenchymal hamartoma. Pediatr Radiol, 2005, 35(3): 349-350.
28.	Abdulkader I, Fraga M, Pérez-Becerra E, et al. Mesenchymal hamartoma of the liver; Clinicopathological, immunohistochemical and flow cytometric study of two cases. Hepatol Res, 2004, 28(4): 216-219.
29.	Bašković M, Đinkić M, Župančić B, et al. Torsion of a mesenchymal hamartoma of the liver in a 1-year-old boy. Acta Chir Belg, 2017, 117(2): 128-130.
30.	Apellaniz-Ruiz M, Segni M, Kettwig M, et al. Mesenchymal hamartoma of the liver and DICER1 syndrome. N Engl J Med, 2019, 380(19): 1834-1842.
31.	Li J, Cai JZ, Guo QJ, et al. Liver transplantation for a giant mesenchymal hamartoma of the liver in an adult: case report and review of the literature. World J Gastroenterol, 2015, 21(20): 6409-6416.
32.	Yesim G, Gupse T, Zafer U, et al. Mesenchymal hamartoma of the liver in adulthood: immunohistochemical profiles, clinical and histopathological features in two patients. J Hepatobiliary Pancreat Surg, 2005, 12(6): 502-507.
33.	Wada M, Ohashi E, Jin H, et al. Mesenchymal hamartoma of the liver: report of an adult case and review of the literature. Intern Med, 1992, 31(12): 1370-1375.
34.	Robertson JC, Jorcyk CL, Oxford JT. DICER1 syndrome: DICER1 mutations in rare cancers. Cancers (Basel), 2018, 10(5): 143.
35.	Koganti SB, Thumma VM, Nagari B. Mesenchymal hamartoma of the liver: complete excision always necessary. Case Rep Surg, 2017, 2017: 8314102.
36.	Meyers R, Hiyama E, Czauderna P, et al. Liver tumors in pediatric patients. Surg Oncol Clin N Am, 2021, 30(2): 253-274.
37.	Vasireddi AK, Leo ME, Squires JH. Magnetic resonance imaging of pediatric liver tumors. Pediatr Radiol, 2022, 52(2): 177-188.
38.	Ninh TP, Dinh TQ, My TT, et al. Hepatic mesenchymal hamartoma: the role of radiology in diagnosis and management. Radiol Case Rep, 2021, 16(8): 2139-2141.
39.	Habibzadeh P, Ansari Asl M, Foroutan HR, et al. Clinicopathological study of hepatic mesenchymal hamartoma and undifferentiated embryonal sarcoma of the liver: a single center study from Iran. Diagn Pathol, 2021, 16(1): 55.
40.	Kuperman AA, Kornreich L, Feinmesser M, et al. Regression of mesenchymal hamartoma of the liver with sarcoma chemotherapy. J Pediatr Hematol Oncol, 2013, 35(1): e25-e26.
41.	Stringer MD, Alizai NK. Mesenchymal hamartoma of the liver: a systematic review. J Pediatr Surg, 2005, 40(11): 1681-1690.
42.	贺海珍, 汤宏峰, 金梅, 等. 肝间叶性错构瘤9例临床病理及预后观察. 肝胆胰外科杂志, 2012, 24(6): 504-506.
43.	齐广伟, 陈莲, 郑佳, 等. 肝脏间叶性错构瘤的临床和病理特征分析. 浙江医学, 2020, 42(5): 497-499.
44.	曹其伟, 张亚洲, 孙青. 肝脏间叶性错构瘤临床病理特征分析. 诊断病理学杂志, 2022, 29(6): 533-536, 541.

1. 于啸, 辛涛. 新生儿肝脏间叶性错构瘤1例. 医学影像学杂志, 2018, 28(4): 654, 658.
2. 相一, 芶欣, 周长升, 等. 肝间叶错构瘤恶变为肉瘤1例报告. 贵州医药, 2017, 41(4): 412-413, 封3.
3. 王宇蔚. 儿童肝左叶巨大间叶错构瘤1例. 中国卫生标准管理, 2016, 7(23): 52-54.
4. 侯志彬, 王春祥, 詹江华, 等. 《请您诊断》病例112答案: 小儿肝脏罕见非典型囊性间叶性错构瘤. 放射学实践, 2016, 31(7): 675-676.
5. 司友娇, 冯卫华, 华辉, 等. 肝脏间叶性错构瘤的CT表现(附3例报告并文献复习). 临床放射学杂志, 2014, 33(8): 1286-1288.
6. 冯平勇, 高洁. 肝脏间叶性错构瘤一例. 临床放射学杂志, 2013, 32(8): 1174-1175.
7. 王小鹏, 陈卫霞, 陈军法, 等. 小儿外生性肝脏间叶性错构瘤1例. 华西医学, 2008, 23(6): 1476-1477.
8. 张再重, 王烈, 王瑜, 等. 肝间叶性错构瘤诊断与治疗(附2例报告并文献复习). 临床军医杂志, 2008, 36(1): 156-159.
9. Le LT, Phan HT, Nguyen TS, et al. Infantile mesenchymal hamartoma of the liver with elevated alpha fetoprotein. BJR Case Rep, 2021, 7(3): 20200196.
10. Gray SC, Pienaar JA, Sofianos Z, et al. Hepatic mesenchymal hamartoma: an uncommon but important paediatric diagnosis. SA J Radiol, 2020, 24(1): 1891.
11. Khan MR, Binkovitz LA, Smyrk TC, et al. Mesenchymal hamartoma in children: a diagnostic challenge. Case Rep Pediatr, 2019, 2019: 4132842.
12. El Demellawy D, Lee JY, McDonell L, et al. Atypical hepatic mesenchymal hamartoma: histologic appearance, immunophenotype, and molecular findings. Pediatr Dev Pathol, 2019, 22(4): 365-369.
13. Rahadiani N, Stephanie M, Putra J. Recurrent hepatic mesenchymal hamartoma with osseous metaplasia. Liver Int, 2018, 38(10): 1875.
14. Berte N, Filfilan A, Mainard L, et al. Co-existing infantile hepatic hemangioma and mesenchymal hamartoma in a neonate. J Surg Case Rep, 2018, 2018(1): rjx260.
15. Liao W, Zhang B, Zhang W, et al. A 4 and a half years old boy with mesenchymal hamartomas in the left lateral lobe of the liver: a case report and literature review. Medicine (Baltimore), 2017, 96(31): e7281.
16. Sen D, Gulati YS, Majumder A, et al. Hepatic cystic mesenchymal hamartoma. Med J Armed Forces India, 2015, 71(Suppl 2): S574-S577.
17. Patel SR, Misra V, Verma K, et al. Benign hepatic mesenchymal hamartoma (HMH) -a case report. J Clin Diagn Res, 2014, 8(3): 119-120.
18. Chandramouleeswari K, Anita S, Shivali B. Mesenchymal hamartoma of the liver: a case report. J Clin Diagn Res, 2012, 6(9): 1552-1554.
19. Orłowski A, Bręborowicz D. Mesenchymal hamartoma of the liver-case report and short literature overview. Pol J Pathol, 2011, 62(2): 108-112.
20. Anil G, Fortier M, Low Y. Cystic hepatic mesenchymal hamartoma: the role of radiology in diagnosis and perioperative management. Br J Radiol, 2011, 84(1001): e91-e94.
21. Kodandapani S, Pai MV, Kumar V, et al. Prenatal diagnosis of congenital mesenchymal hamartoma of liver: a case report. Case Rep Obstet Gynecol, 2011, 2011: 932583.
22. Shintaku M, Watanabe K. Mesenchymal hamartoma of the liver: a proliferative lesion of possible hepatic stellate cell (Ito cell) origin. Pathol Res Pract, 2010, 206(7): 532-536.
23. Cornette J, Festen S, van den Hoonaard TL, et al. Mesenchymal hamartoma of the liver: a benign tumor with deceptive prognosis in the perinatal period. Case report and review of the literature. Fetal Diagn Ther, 2009, 25(2): 196-202.
24. Vázquez-Lima I, Vázquez JL, Gallego M, et al. Torsed pedunculated hepatic hamartoma. Pediatr Radiol, 2009, 39(1): 62-65.
25. Baboiu OE, Saal H, Collins M. Hepatic mesenchymal hamartoma: cytogenetic analysis of a case and review of the literature. Pediatr Dev Pathol, 2008, 11(4): 295-299.
26. Steiner MA, Giles HW. Mesenchymal hamartoma of the liver demonstrating peripheral calcification in a 12-year-old boy. Pediatr Radiol, 2008, 38(11): 1232-1234.
27. Mansour A, Qandeel M, Sughayer M, et al. Solid (stromal predominance) hepatic mesenchymal hamartoma. Pediatr Radiol, 2005, 35(3): 349-350.
28. Abdulkader I, Fraga M, Pérez-Becerra E, et al. Mesenchymal hamartoma of the liver; Clinicopathological, immunohistochemical and flow cytometric study of two cases. Hepatol Res, 2004, 28(4): 216-219.
29. Bašković M, Đinkić M, Župančić B, et al. Torsion of a mesenchymal hamartoma of the liver in a 1-year-old boy. Acta Chir Belg, 2017, 117(2): 128-130.
30. Apellaniz-Ruiz M, Segni M, Kettwig M, et al. Mesenchymal hamartoma of the liver and DICER1 syndrome. N Engl J Med, 2019, 380(19): 1834-1842.
31. Li J, Cai JZ, Guo QJ, et al. Liver transplantation for a giant mesenchymal hamartoma of the liver in an adult: case report and review of the literature. World J Gastroenterol, 2015, 21(20): 6409-6416.
32. Yesim G, Gupse T, Zafer U, et al. Mesenchymal hamartoma of the liver in adulthood: immunohistochemical profiles, clinical and histopathological features in two patients. J Hepatobiliary Pancreat Surg, 2005, 12(6): 502-507.
33. Wada M, Ohashi E, Jin H, et al. Mesenchymal hamartoma of the liver: report of an adult case and review of the literature. Intern Med, 1992, 31(12): 1370-1375.
34. Robertson JC, Jorcyk CL, Oxford JT. DICER1 syndrome: DICER1 mutations in rare cancers. Cancers (Basel), 2018, 10(5): 143.
35. Koganti SB, Thumma VM, Nagari B. Mesenchymal hamartoma of the liver: complete excision always necessary. Case Rep Surg, 2017, 2017: 8314102.
36. Meyers R, Hiyama E, Czauderna P, et al. Liver tumors in pediatric patients. Surg Oncol Clin N Am, 2021, 30(2): 253-274.
37. Vasireddi AK, Leo ME, Squires JH. Magnetic resonance imaging of pediatric liver tumors. Pediatr Radiol, 2022, 52(2): 177-188.
38. Ninh TP, Dinh TQ, My TT, et al. Hepatic mesenchymal hamartoma: the role of radiology in diagnosis and management. Radiol Case Rep, 2021, 16(8): 2139-2141.
39. Habibzadeh P, Ansari Asl M, Foroutan HR, et al. Clinicopathological study of hepatic mesenchymal hamartoma and undifferentiated embryonal sarcoma of the liver: a single center study from Iran. Diagn Pathol, 2021, 16(1): 55.
40. Kuperman AA, Kornreich L, Feinmesser M, et al. Regression of mesenchymal hamartoma of the liver with sarcoma chemotherapy. J Pediatr Hematol Oncol, 2013, 35(1): e25-e26.
41. Stringer MD, Alizai NK. Mesenchymal hamartoma of the liver: a systematic review. J Pediatr Surg, 2005, 40(11): 1681-1690.
42. 贺海珍, 汤宏峰, 金梅, 等. 肝间叶性错构瘤9例临床病理及预后观察. 肝胆胰外科杂志, 2012, 24(6): 504-506.
43. 齐广伟, 陈莲, 郑佳, 等. 肝脏间叶性错构瘤的临床和病理特征分析. 浙江医学, 2020, 42(5): 497-499.
44. 曹其伟, 张亚洲, 孙青. 肝脏间叶性错构瘤临床病理特征分析. 诊断病理学杂志, 2022, 29(6): 533-536, 541.

CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY

仅蒂与肝相连的罕见巨大肝间叶错构瘤1例报道并文献复习

Abstract Full text Figures/Tables Video References Cited by

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