腹膜后继发性去分化脂肪肉瘤6例临床病理分析
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摘要
目的探讨腹膜后继发性去分化脂肪肉瘤(DDLS)的临床病理学特征。方法收集2015-01—2018-07北京大学国际医院收治的6例腹膜后继发性DDLS的患者资料,包括临床表现、组织学形态、诊断、鉴别诊断、治疗及预后,并复习相关文献。结果 6例腹膜后继发性DDLS初诊时均为高分化脂肪肉瘤(WDLS)。男性5例,女性1例;年龄45～77岁,平均年龄56.7岁。所有肿瘤发生去分化均经过1～3次复发,所需的时间为9～120个月,平均26.8个月。发生去分化时患者的年龄46～78岁,平均年龄60岁。镜下DDLS的高分化区的亚型与首次诊断WDLS的亚型不完全相同。DDLS的去分化区呈多样性。4例肿瘤去分化区均为纤维肉瘤样,且其中1例合并有黏液纤维肉瘤样。另外2例肿瘤去分化区分别为恶性纤维组织细胞瘤样和黏液纤维肉瘤样。免疫组化染色:6例肿瘤细胞均表达CDK4和P16,其中前者往往为弥漫阳性。5例肿瘤细胞均表达MDM2,MDM2多呈散在阳性;6例肿瘤高分化区均不同程度表达S-100;1例肿瘤去分化区呈CD34弱阳性表达。6例患者均行手术切除。结论 DDLS由高分化区和去分化区组成,亦可由WDLS发生去分化而形成。DDLS的高分化区的亚型与首次诊断WDLS的亚型不完全相同,其去分化区以纤维肉瘤样最常见。诊断以形态学为基础,辅以免疫组织化学或基因检测。治疗以手术切除为主,并密切随诊。
Objective To investigate the clinicopathological features of secondary dedifferentiated liposarcoma in retroperitoneum. Methods Six cases of secondary dedifferentiated liposarcoma in retroperitoneum were collected from January 2015 to July 2018 at Peking University International Hospital. The clinical manifestations,histomorphologic changes, diagnosis, differential diagnosis, treatment and follow-up data were analyzed, and relevant literature reviewed. Results Initial diagnosis of six cases of secondary dedifferentiated liposarcoma in retroperitoneum was well-differentiated liposarcoma. Six patients included five males and one female,with age range of 45 to 77 years(mean 56.7 years). All the tumors underwent dedifferentiation after 1 to 3 recurrences, with a mean time of 26.8 months(9 to 120 months). The age of the dedifferentiated patients was 46～78 years, with a mean age of 60 years. Microscopically, the subtypes of DDLS's well-differentiated areas were partially identical to those of WDLS. The DDLS's dedifferentiated areas were diverse. The dedifferentiated areas of four tumors were fibrosarcoma-like, and one of them was combined with myxofibrosarcoma-like. In the other two cases,the dedifferentiated areas were malignant fibrous histiocytoma-like and myxofibrosarcoma-like, respectively. By immunohistochemical staining, the tumor cells of all cases were positive for CDK4 and P16, and the former was typically diffuse positive. MDM2 protein was expressed scatteredly in five cases. S-100 protein expressed in well-differentiated area in all cases but at different levels. The tumor cells of dedifferentiated areas were focally positive for CD34 protein in one case. All cases underwent surgical resection. Conclusions DDLS is composed of well-differentiated areas and dedifferentiated areas, and it can also be present in a background of a preexisting WDLS with dedifferentiation. The subtypes of DDLS's well-differentiated areas are partly identical to those of WDLS. Fibrosarcoma-like is the most common type of the DDLS's dedifferentiated areas. Pathological diagnosis is based on morphology, with supplementary of immunohistochemistry or gene detertion. Surgical resection is the recommended therapy,with close follow-up.
Objective To investigate the clinicopathological features of secondary dedifferentiated liposarcoma in retroperitoneum. Methods Six cases of secondary dedifferentiated liposarcoma in retroperitoneum were collected from January 2015 to July 2018 at Peking University International Hospital. The clinical manifestations,histomorphologic changes, diagnosis, differential diagnosis, treatment and follow-up data were analyzed, and relevant literature reviewed. Results Initial diagnosis of six cases of secondary dedifferentiated liposarcoma in retroperitoneum was well-differentiated liposarcoma. Six patients included five males and one female,with age range of 45 to 77 years(mean 56.7 years). All the tumors underwent dedifferentiation after 1 to 3 recurrences, with a mean time of 26.8 months(9 to 120 months). The age of the dedifferentiated patients was 46～78 years, with a mean age of 60 years. Microscopically, the subtypes of DDLS's well-differentiated areas were partially identical to those of WDLS. The DDLS's dedifferentiated areas were diverse. The dedifferentiated areas of four tumors were fibrosarcoma-like, and one of them was combined with myxofibrosarcoma-like. In the other two cases,the dedifferentiated areas were malignant fibrous histiocytoma-like and myxofibrosarcoma-like, respectively. By immunohistochemical staining, the tumor cells of all cases were positive for CDK4 and P16, and the former was typically diffuse positive. MDM2 protein was expressed scatteredly in five cases. S-100 protein expressed in well-differentiated area in all cases but at different levels. The tumor cells of dedifferentiated areas were focally positive for CD34 protein in one case. All cases underwent surgical resection. Conclusions DDLS is composed of well-differentiated areas and dedifferentiated areas, and it can also be present in a background of a preexisting WDLS with dedifferentiation. The subtypes of DDLS's well-differentiated areas are partly identical to those of WDLS. Fibrosarcoma-like is the most common type of the DDLS's dedifferentiated areas. Pathological diagnosis is based on morphology, with supplementary of immunohistochemistry or gene detertion. Surgical resection is the recommended therapy,with close follow-up.
引文
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[3] Henricks WH,Chu YC,Goldblum JR,et al.Dedifferentiated liposarcoma:a clinicopathological analysis of 155 cases with a proposal for an expanded definition of dedifferentiation[J].Am J Surg Pathol,1997,21(3):271-281.
[4] Ben Salha I,Zaidi S,Noujaim J,et al.Rare Aggressive Behavior of MDM2-Amplified Retroperitoneal Dedifferentiated Liposarcoma,with Brain,Lung and Subcutaneous Metastases [J].Rare Tumors,2016,8(3):6282.
[5] Gronchi A,Collini P,Miceli R,et al.Myogenic differentiation and histologic grading are major prognostic determinants in retroperitoneal liposarcoma[J].Am J Surg Pathol,2015,39(3):383-393.
[6] Evans H L.liposarcoma:a study of 55 cases with reassessment of its classification[J].Am J Surg Pathol,1979,3(6):507-23.
[7] Byun JY,Choi YW,Choi HY,et al.A Case of Dedifferentiated Liposarcoma That Developed in the Dermis[J].Ann Dermatol,2008,20(4):204-208.
[8] 刘权,彭卫军,王坚.腹膜后去分化脂肪肉瘤的CT诊断[J].中华放射学杂志,2004,38(11):1206-1209.
[9] 谢凯圣,张斌,郑绍光,等.腹膜后伴有异源性分化的去分化脂肪肉瘤病例分析及文献复习[J].中国临床新医学,2013,(11):1065-1068.
[10] Shimada S,Ishizawa T,Ishizawa K,et al.Dedifferentiated liposarcoma with rhabdomyoblastic differentiation[J].Virehows Arch,2005,447(5):835-841.
[11] Fletcher CD,Unni K K,Mertens F.World health organization chassification of tumours.Pathology and genetics of tumours of soft tissue and bone[M].Lyon:IARC Press,2002.
[12] Nishio J,Iwasaki H,Ishiguro M,et al.Establishment of a novel human dedifferentiated liposarcoma cell line,FU-DDLS-1:conventional and molecular cytogenetic characterization[J].Int J Oncol,2003,22(3):535-542.
[13] Sirvent N,Forus A,Lescaut W,et al.Characterization of centromere alterations in liposarcomas[J].Genes Chromosomes Cancer,2000,29(2):117-129.
[14] Fletcher CD,Bridge JA,Hogendoom PC,et al.World health organization chassification of tumours of soft tissue and bone[M].Lyon:IARC Press,2013.
[15] Fritz B,Schubert F,Wrobel G,et al.Microarray-based copy number and expression profiling in dedifferentiated and pleomorphic liposarcomal[J].Cancer Res,2002,62(11):2993 -2998.
[16] Antonescu CR,Elahi A,Healey JH,et al.Monoclonality of multifocal myxoid liposarcoma:confirmation by analysis of TLS-CHOP or EWS-CHOP rearrangements [J].Clin Cancer Res,2000,6(7):2788 -2793.
[17] Ricciotti RW,Baraff AJ,Jour G,et al.High amplification levels of MDM2 and CDK4 correlate with poor outcome in patients with dedifferentiated liposarcoma:A cytogenomic microarray analysis of 47 cases[J].Cancer Genet,2017,218-219:69-80.
[18] Sirvent N,Coindre JM,Maire G,et al.Detection of MDM2-CDK4 amplification by fluorescence in situ hybridization in 200 paraffin-embedded tumor samples:utility in diagnosing adipocytic lesions and comparison with immunohistochemistry and real-time PCR[J].Am J Surg Pathol,2007,31(10):1476-1489.
[19] Binh MB,Sastre-Garau X,Guillou L,et al.MDM2 and CDK4 immunostainings are useful adjuncts in diagnosing well-differentiated and dedifferentiated liposarcoma subtypes:a comparative analysis of 559 soft tissue neoplasms with genetic data[J].Am J Surg Pathol,2005,29(10):1340-1347.
[20] Aleixo PB,Hartmann AA,Menezes IC,et al.Can MDM2 and CDK4 make the diagnosis of well differentiated/dedifferentiated liposarcoma?An immunohistochemical study on 129 soft tissue tumours[J].J Clin Pathol,2009,62(12):1127-1135.
[21] Kang Y,Horvai AE.P16 immunohistochemistry is less useful than MDM2 and CDK4 to distinguish dedifferentiated liposarcomas from other retroperitoneal mimics[J].Appl Immunohistochem Mol Morphol[J].2017,25(1):58-63.
[22] Kammerer-Jacquet SF,Thierry S,Cabillic F,et al.Differential diagnosis of atypical lipomatous tumor/well-differentiated liposarcoma and dedifferentiated liposarcoma:utility of p16 in combination with MDM2 and CDK4 immunohistochemistry [J].Hum Pathol,2017,59:34-40.
[23] 张兆祥,韩林.去分化脂肪肉瘤的病理学研究进展[J].临床与实验病理学杂,2010,26(1):97-100.
[2] Nascimento AG.Dedifferentiated liposarcoma[J].Semin Diagn Pathol,2001,18(4):263-266.
[3] Henricks WH,Chu YC,Goldblum JR,et al.Dedifferentiated liposarcoma:a clinicopathological analysis of 155 cases with a proposal for an expanded definition of dedifferentiation[J].Am J Surg Pathol,1997,21(3):271-281.
[4] Ben Salha I,Zaidi S,Noujaim J,et al.Rare Aggressive Behavior of MDM2-Amplified Retroperitoneal Dedifferentiated Liposarcoma,with Brain,Lung and Subcutaneous Metastases [J].Rare Tumors,2016,8(3):6282.
[5] Gronchi A,Collini P,Miceli R,et al.Myogenic differentiation and histologic grading are major prognostic determinants in retroperitoneal liposarcoma[J].Am J Surg Pathol,2015,39(3):383-393.
[6] Evans H L.liposarcoma:a study of 55 cases with reassessment of its classification[J].Am J Surg Pathol,1979,3(6):507-23.
[7] Byun JY,Choi YW,Choi HY,et al.A Case of Dedifferentiated Liposarcoma That Developed in the Dermis[J].Ann Dermatol,2008,20(4):204-208.
[8] 刘权,彭卫军,王坚.腹膜后去分化脂肪肉瘤的CT诊断[J].中华放射学杂志,2004,38(11):1206-1209.
[9] 谢凯圣,张斌,郑绍光,等.腹膜后伴有异源性分化的去分化脂肪肉瘤病例分析及文献复习[J].中国临床新医学,2013,(11):1065-1068.
[10] Shimada S,Ishizawa T,Ishizawa K,et al.Dedifferentiated liposarcoma with rhabdomyoblastic differentiation[J].Virehows Arch,2005,447(5):835-841.
[11] Fletcher CD,Unni K K,Mertens F.World health organization chassification of tumours.Pathology and genetics of tumours of soft tissue and bone[M].Lyon:IARC Press,2002.
[12] Nishio J,Iwasaki H,Ishiguro M,et al.Establishment of a novel human dedifferentiated liposarcoma cell line,FU-DDLS-1:conventional and molecular cytogenetic characterization[J].Int J Oncol,2003,22(3):535-542.
[13] Sirvent N,Forus A,Lescaut W,et al.Characterization of centromere alterations in liposarcomas[J].Genes Chromosomes Cancer,2000,29(2):117-129.
[14] Fletcher CD,Bridge JA,Hogendoom PC,et al.World health organization chassification of tumours of soft tissue and bone[M].Lyon:IARC Press,2013.
[15] Fritz B,Schubert F,Wrobel G,et al.Microarray-based copy number and expression profiling in dedifferentiated and pleomorphic liposarcomal[J].Cancer Res,2002,62(11):2993 -2998.
[16] Antonescu CR,Elahi A,Healey JH,et al.Monoclonality of multifocal myxoid liposarcoma:confirmation by analysis of TLS-CHOP or EWS-CHOP rearrangements [J].Clin Cancer Res,2000,6(7):2788 -2793.
[17] Ricciotti RW,Baraff AJ,Jour G,et al.High amplification levels of MDM2 and CDK4 correlate with poor outcome in patients with dedifferentiated liposarcoma:A cytogenomic microarray analysis of 47 cases[J].Cancer Genet,2017,218-219:69-80.
[18] Sirvent N,Coindre JM,Maire G,et al.Detection of MDM2-CDK4 amplification by fluorescence in situ hybridization in 200 paraffin-embedded tumor samples:utility in diagnosing adipocytic lesions and comparison with immunohistochemistry and real-time PCR[J].Am J Surg Pathol,2007,31(10):1476-1489.
[19] Binh MB,Sastre-Garau X,Guillou L,et al.MDM2 and CDK4 immunostainings are useful adjuncts in diagnosing well-differentiated and dedifferentiated liposarcoma subtypes:a comparative analysis of 559 soft tissue neoplasms with genetic data[J].Am J Surg Pathol,2005,29(10):1340-1347.
[20] Aleixo PB,Hartmann AA,Menezes IC,et al.Can MDM2 and CDK4 make the diagnosis of well differentiated/dedifferentiated liposarcoma?An immunohistochemical study on 129 soft tissue tumours[J].J Clin Pathol,2009,62(12):1127-1135.
[21] Kang Y,Horvai AE.P16 immunohistochemistry is less useful than MDM2 and CDK4 to distinguish dedifferentiated liposarcomas from other retroperitoneal mimics[J].Appl Immunohistochem Mol Morphol[J].2017,25(1):58-63.
[22] Kammerer-Jacquet SF,Thierry S,Cabillic F,et al.Differential diagnosis of atypical lipomatous tumor/well-differentiated liposarcoma and dedifferentiated liposarcoma:utility of p16 in combination with MDM2 and CDK4 immunohistochemistry [J].Hum Pathol,2017,59:34-40.
[23] 张兆祥,韩林.去分化脂肪肉瘤的病理学研究进展[J].临床与实验病理学杂,2010,26(1):97-100.
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