肝脏原发性Rosai-Dorfman病：1例报道并文献复习_CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY

Authors：

陈康权 ¹ , 张森 ² , 刘晓光 ¹ , 袁国佳 ¹ ,  陈念平 ¹

1. ;
2. ;

Corresponding author：

陈念平, Email: npc88@163.com

Keywords：

Rosai-Dorfman病; 肝脏原发性; 病理特征; 诊断; 治疗; 预后

DOI：

10.7507/1007-9424.202312091

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目的总结肝脏原发性Rosai-Dorfman（RD）病的临床及影像学表现、病理特征、鉴别诊断、治疗及预后。方法回顾性收集广东医科大学附属医院收治的1例肝脏原发性RD病的相关资料并结合国内外相关文献报道病例进行分析。结果本例患者为一41岁女性患者，因“反复右上腹疼痛2⁺ 个月”就诊，缺乏特异性体征和症状，术前误诊为肝癌。行手术完整切除肝右叶肿物，约4.0 cm×4.0 cm×3.7 cm大小，质地稍硬，边界清；术后组织病理学检查见肿物有大量淋巴细胞和浆细胞，其中可见胞质嗜酸、胞核稍大的梭形细胞，边界较清；免疫组织化学染色发现，RD病组织中S-100、八聚体结合转录因子-2、cyclinD1、CD68、CD163等标志物呈不同程度的阳性。术后病理检查诊断为肝脏原发性RD病，术后随访7个月肿瘤无复发。复习国内外文献共收集到2例肝脏原发性RD病，术前皆误诊为肝恶性肿瘤，均行根治性手术切除，术后经组织病理学检查才确诊，术后随访均未见复发。结论 RD病是一种罕见的非朗格汉斯细胞组织细胞增多症，原发于肝脏的RD病更加罕见，容易误诊，需与肝细胞癌、肝内胆管细胞癌、肝腺瘤及肝转移瘤鉴别，确诊依赖于术后组织病理学检查，手术治疗效果较好，预后良好，术后需严密随访。

Citation： 陈康权, 张森, 刘晓光, 袁国佳, 陈念平. 肝脏原发性Rosai-Dorfman病：1例报道并文献复习. CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY, 2024, 31(8): 981-985. doi: 10.7507/1007-9424.202312091 Copy

1.	Destombes P. Adenitis with lipid excess, in children or young adults, seen in the Antilles and in Mali. (4 cases). Bull Soc Pathol Exot Filiales. 1965, 58(6): 1169-1175.
2.	Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity. Arch Pathol, 1969, 87(1): 63-70.
3.	Emile JF, Abla O, Fraitag S, et al. Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood, 2016, 127(22): 2672-2681.
4.	Alaggio R, Amador C, Anagnostopoulos I, et al. The 5th edition of the World Health Organization classification of haematolymphoid tumours: lymphoid neoplasms. Leukemia, 2022, 36(7): 1720-1748.
5.	邓斐文, 胡健垣, 张良运, 等. 肝脏原发性Rosai-Doffman病一例并文献复习. 中华肝胆外科杂志, 2015, 21(8): 522.
6.	Gazia C, Giordano L, Diodoro MG, et al. Differential diagnosis in Rosai-Dorfman disease: a rare case of isolated hepatic presentation mimicking a metastatic tumor with positive 18-FDG uptake. Intractable Rare Dis Res, 2022, 11(2): 90-92.
7.	崔哲卿, 濮田, 刘蕾, 等. 累及腮腺、双侧鼻腔及颈部淋巴结Rosai-Dorfman病一例. 中国临床案例成果数据库, 2022, 4(1): E00873-E00873.
8.	Xia Y, Yang Z, Qian Y. Rosai-Dorfman disease of bilateral lateral buccal skin: a case report. J Clin Dermatol, 2015, 44(3): 175-177.
9.	Moore JC, Zhao X, Nelson EL. Concomitant sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) and diffuse large B-cell lymphoma: a case report. J Med Case Rep, 2008, 2: 70. doi: 10.1186/1752-1947-2-70.
10.	Luppi M, Barozzi P, Garber R, et al. Expression of human herpesvirus-6 antigens in benign and malignant lymphoproliferative diseases. Am J Pathol, 1998, 153(3): 815-823.
11.	Foucar E, Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. Semin Diagn Pathol, 1990, 7(1): 19-73.
12.	Al-Daraji W, Anandan A, Klassen-Fischer M, et al. Soft tissue Rosai-Dorfman disease: 29 new lesions in 18 patients, with detection of polyomavirus antigen in 3 abdominal cases. Ann Diagn Pathol, 2010, 14(5): 309-316.
13.	Nathan B, Roomallah B, Salisbury JR, et al. A rare cause of lymphadenopathy—Rosai-Dorfman disease in a HIV-positive Ugandan woman. Int J STD AIDS, 2016, 27(12): 1123-1125.
14.	Menon MP, Evbuomwan MO, Rosai J, et al. A subset of Rosai-Dorfman disease cases show increased IgG4-positive plasma cells: another red herring or a true association with IgG4-related disease?. Histopathology, 2014, 64(3): 455-459.
15.	Fatobene G, Haroche J, Hélias-Rodzwicz Z, et al. BRAF^V600E mutation detected in a case of Rosai-Dorfman disease. Haematologica, 2018, 103(8): e377-e379. doi: 10.3324/haematol.2018.190934.
16.	Garces S, Medeiros LJ, Patel KP, et al. Mutually exclusive recurrent KRAS and MAP2K1 mutations in Rosai-Dorfman disease. Mod Pathol, 2017, 30(10): 1367-1377.
17.	Matter MS, Bihl M, Juskevicius D, et al. Is Rosai-Dorfman disease a reactve process? Detection of a MAP2K1 L115V mutation in a case of Rosai-Dorfman disease. Virchows Arch, 2017, 471(4): 545-547.
18.	Lauwers GY, Perez-Atayde A, Dorfman RF, et al. The digestive system manifestations of Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy): review of 11 cases. Hum Pathol, 2000, 31(3): 380-385.
19.	Mahajan S, Nakajima R, Yabe M, et al. Rosai-Dorfman disease-utility of ¹⁸F-FDG PET/CT for initial evaluation and follow-up. Clin Nucl Med, 2020, 45(6): e260-e266. doi: 10.1097/RLU.0000000000003014.
20.	Dhull VS, Passah A, Rana N, et al. ¹⁸F-FDG PET/CT of widespread Rosai-Dorfman disease. Clin Nucl Med, 2016, 41(1): 57-59.
21.	Halil O, Barrett AJ. Phagocytosis by megakaryocytes in malignant disorders. Br J Haematol, 1980, 46(1): 161. doi: 10.1111/j.1365-2141.1980.tb05951.x.
22.	Dalia S, Sagatys E, Sokol L, et al. Rosai-Dorfman disease: tumor biology, clinical features, pathology, and treatment. Cancer Control, 2014, 21(4): 322-327.
23.	Kreisel FH. Hematolymphoid lesions of the sinonasal tract. Head Neck Pathol, 2016, 10(1): 109-117.
24.	Pulsoni A, Anghel G, Falcucci P, et al. Treatment of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): report of a case and literature review. Am J Hematol, 2002, 69(1): 67-71.
25.	徐夏, 邓文婷, 黄成志, 等. 喉部Rosai-Dorfman病1例报道及文献复习. 南方医科大学学报, 2018, 38(2): 125-129.
26.	Chang L, Qiao B, Cai H, et al. Clinical phenotypes, molecular analysis, and outcomes of patients with Rosai-Dorfman disease. Leukemia, 2023, 37(11): 2297-2300.

1. Destombes P. Adenitis with lipid excess, in children or young adults, seen in the Antilles and in Mali. (4 cases). Bull Soc Pathol Exot Filiales. 1965, 58(6): 1169-1175.
2. Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity. Arch Pathol, 1969, 87(1): 63-70.
3. Emile JF, Abla O, Fraitag S, et al. Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood, 2016, 127(22): 2672-2681.
4. Alaggio R, Amador C, Anagnostopoulos I, et al. The 5th edition of the World Health Organization classification of haematolymphoid tumours: lymphoid neoplasms. Leukemia, 2022, 36(7): 1720-1748.
5. 邓斐文, 胡健垣, 张良运, 等. 肝脏原发性Rosai-Doffman病一例并文献复习. 中华肝胆外科杂志, 2015, 21(8): 522.
6. Gazia C, Giordano L, Diodoro MG, et al. Differential diagnosis in Rosai-Dorfman disease: a rare case of isolated hepatic presentation mimicking a metastatic tumor with positive 18-FDG uptake. Intractable Rare Dis Res, 2022, 11(2): 90-92.
7. 崔哲卿, 濮田, 刘蕾, 等. 累及腮腺、双侧鼻腔及颈部淋巴结Rosai-Dorfman病一例. 中国临床案例成果数据库, 2022, 4(1): E00873-E00873.
8. Xia Y, Yang Z, Qian Y. Rosai-Dorfman disease of bilateral lateral buccal skin: a case report. J Clin Dermatol, 2015, 44(3): 175-177.
9. Moore JC, Zhao X, Nelson EL. Concomitant sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) and diffuse large B-cell lymphoma: a case report. J Med Case Rep, 2008, 2: 70. doi: 10.1186/1752-1947-2-70.
10. Luppi M, Barozzi P, Garber R, et al. Expression of human herpesvirus-6 antigens in benign and malignant lymphoproliferative diseases. Am J Pathol, 1998, 153(3): 815-823.
11. Foucar E, Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. Semin Diagn Pathol, 1990, 7(1): 19-73.
12. Al-Daraji W, Anandan A, Klassen-Fischer M, et al. Soft tissue Rosai-Dorfman disease: 29 new lesions in 18 patients, with detection of polyomavirus antigen in 3 abdominal cases. Ann Diagn Pathol, 2010, 14(5): 309-316.
13. Nathan B, Roomallah B, Salisbury JR, et al. A rare cause of lymphadenopathy—Rosai-Dorfman disease in a HIV-positive Ugandan woman. Int J STD AIDS, 2016, 27(12): 1123-1125.
14. Menon MP, Evbuomwan MO, Rosai J, et al. A subset of Rosai-Dorfman disease cases show increased IgG4-positive plasma cells: another red herring or a true association with IgG4-related disease?. Histopathology, 2014, 64(3): 455-459.
15. Fatobene G, Haroche J, Hélias-Rodzwicz Z, et al. BRAF^V600E mutation detected in a case of Rosai-Dorfman disease. Haematologica, 2018, 103(8): e377-e379. doi: 10.3324/haematol.2018.190934.
16. Garces S, Medeiros LJ, Patel KP, et al. Mutually exclusive recurrent KRAS and MAP2K1 mutations in Rosai-Dorfman disease. Mod Pathol, 2017, 30(10): 1367-1377.
17. Matter MS, Bihl M, Juskevicius D, et al. Is Rosai-Dorfman disease a reactve process? Detection of a MAP2K1 L115V mutation in a case of Rosai-Dorfman disease. Virchows Arch, 2017, 471(4): 545-547.
18. Lauwers GY, Perez-Atayde A, Dorfman RF, et al. The digestive system manifestations of Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy): review of 11 cases. Hum Pathol, 2000, 31(3): 380-385.
19. Mahajan S, Nakajima R, Yabe M, et al. Rosai-Dorfman disease-utility of ¹⁸F-FDG PET/CT for initial evaluation and follow-up. Clin Nucl Med, 2020, 45(6): e260-e266. doi: 10.1097/RLU.0000000000003014.
20. Dhull VS, Passah A, Rana N, et al. ¹⁸F-FDG PET/CT of widespread Rosai-Dorfman disease. Clin Nucl Med, 2016, 41(1): 57-59.
21. Halil O, Barrett AJ. Phagocytosis by megakaryocytes in malignant disorders. Br J Haematol, 1980, 46(1): 161. doi: 10.1111/j.1365-2141.1980.tb05951.x.
22. Dalia S, Sagatys E, Sokol L, et al. Rosai-Dorfman disease: tumor biology, clinical features, pathology, and treatment. Cancer Control, 2014, 21(4): 322-327.
23. Kreisel FH. Hematolymphoid lesions of the sinonasal tract. Head Neck Pathol, 2016, 10(1): 109-117.
24. Pulsoni A, Anghel G, Falcucci P, et al. Treatment of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): report of a case and literature review. Am J Hematol, 2002, 69(1): 67-71.
25. 徐夏, 邓文婷, 黄成志, 等. 喉部Rosai-Dorfman病1例报道及文献复习. 南方医科大学学报, 2018, 38(2): 125-129.
26. Chang L, Qiao B, Cai H, et al. Clinical phenotypes, molecular analysis, and outcomes of patients with Rosai-Dorfman disease. Leukemia, 2023, 37(11): 2297-2300.

CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY

肝脏原发性Rosai-Dorfman病：1例报道并文献复习

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