A case report by Liu et al describes the characteristics of metastatic clear cell sarcoma(CCS)of the pancreas and provides valuable therapeutic insights for this rare malignancy.This case is interesting because of its...A case report by Liu et al describes the characteristics of metastatic clear cell sarcoma(CCS)of the pancreas and provides valuable therapeutic insights for this rare malignancy.This case is interesting because of its rarity,suggesting that the pancreas may be a potential target organ for CCS,either primary or metastatic.At the same time,the authors also emphasize the importance of regular postoperative follow-up for timely detection of recurrent lesions,as CCS is characterized by a high degree of malignancy and a high rate of recurrent metastases.Considering that CCS of the gastrointestinal tract is easily confused with malignant melanoma(MM)of the gastrointestinal tract,here we compare the clinical features,histopathological and immunohistochemical characteristics,diagnosis,treatment,and prognosis of CCS and MM of the gastrointestinal tract,hoping to provide a reference for clinical work.展开更多
Primary or secondary clear cell sarcoma of the pancreas is an exceedingly rare and aggressive disease.In addition to pathology,molecular analysis is pivotal in differential diagnosis,especially with malignant melanoma...Primary or secondary clear cell sarcoma of the pancreas is an exceedingly rare and aggressive disease.In addition to pathology,molecular analysis is pivotal in differential diagnosis,especially with malignant melanoma.A key aspect in identifying clear cell sarcoma is specific genetic alterations,notably the translocation of t(12;22)(q13;q13),a diagnostic hallmark of this sarcoma subtype,which is absent in malignant melanoma.Treatment of primary clear cell sarcoma of the pancreas is the same as that for adenocarcinoma.展开更多
This editorial comments on the study by Liu et al investigating pancreatic metastasis of clear cell sarcoma(CCS)published in the World Journal of Clinical Cases.CCS is a rare and aggressive melanocytic tumor,that typi...This editorial comments on the study by Liu et al investigating pancreatic metastasis of clear cell sarcoma(CCS)published in the World Journal of Clinical Cases.CCS is a rare and aggressive melanocytic tumor,that typically arises from tendons and aponeuroses of the limbs,and metastasizes to the lungs,bones,and brain.However,pancreatic metastasis has rarely been reported,presenting unique diagnostic and therapeutic challenges.Elucidating the clinical characteristics,imaging features,prognostic factors,and treatment outcomes of patients with pancreatic CCS metastasis is crucial.Surgery remains an effective management strategy for CCS.However,the high recurrence rate and low effectiveness of traditional adjuvant treatments necessitate a shift towards more personalized and targeted treatment plans.Research is needed to investigate and validate novel therapeutic approaches specifically tailored to the distinct genetic and molecular characteristics of rare malignancies like CCS.Additionally,the development of late metastases after a long disease-free interval is common in CCS patients.Therefore,routine postoperative surveillance for metastasis using computed tomography,magnetic resonance imaging,bone scans,and positron emission tomography scans is crucial.Moving forward,enhanced collaboration,investigation,and creative thinking among scientists,medical professionals,and legislators are essential to gain a deeper understanding of these rare presentations.展开更多
Clear cell sarcoma of the kidney （CCSK） is a rare and highly malignant tumor which is usually confused with other kidaey tumors We experienced such a patient and present report this.
We experienced a case of small, round-cell malignant neoplasm diagnosed by touch smear cytology and histopathology when an open biopsy was performed in a 50-year-old Japanese woman. She was suspected of having a cervi...We experienced a case of small, round-cell malignant neoplasm diagnosed by touch smear cytology and histopathology when an open biopsy was performed in a 50-year-old Japanese woman. She was suspected of having a cervical spine tumor after surgery for cervical spine foraminal stenosis. After consent, the cervical spine tumor histologically diagnosed by an open biopsy was confirmed to be Ewing sarcoma (EWS) by genetic testing. EWS belongs to a group of small, round-cell tumors that are morphologically similar and often difficult to differentiate. After the open biopsy, the present patient received radiotherapy, and her plasma level of Pro-Gastrin-Releasing-Peptide was decreased (217.2 pg/ml before surgery to 30.3 pg/ml;reference value: 0 - 80 pg/ml). We herein report the process for making the final diagnosis by focusing on the intraoperative cytology, histopathology, and immunohistochemical findings. Our diagnosis was validated by karyotyping and a fluorescence <em>in-situ</em> hybridization analysis.展开更多
目的:探讨儿童肾透明细胞肉瘤(clear cell sarcoma of the kidney,CCSK)的临床病理特点与鉴别诊断。方法:观察1例儿童肾透明细胞肉瘤的病理学形态、特殊染色及免疫表型特征并复习有关文献。结果:片内显示为小细胞肿瘤,呈弥漫性分布,部...目的:探讨儿童肾透明细胞肉瘤(clear cell sarcoma of the kidney,CCSK)的临床病理特点与鉴别诊断。方法:观察1例儿童肾透明细胞肉瘤的病理学形态、特殊染色及免疫表型特征并复习有关文献。结果:片内显示为小细胞肿瘤,呈弥漫性分布,部分细胞丰富,密集,部分区域细胞排列较松散,以分枝状纤维血管间质分隔肿瘤细胞呈不规则索状和小团状为结构特征,细胞呈圆形、多角形或梭形,细胞核呈圆形、卵圆形或棒状,核染色质呈细颗粒状,核膜较薄,核仁不明显。网状纤维染色:网状纤维密布于单个或小团肿瘤细胞之间。Masson三色:肿瘤间质小血管周纤维组织呈绿色。免疫组化染色:Vim、Bcl2和CD99(+),其他(-)。结论:CCSK是少见的小儿恶性肾肿瘤,诊断主要依赖于病理学,熟悉CCSK典型形态学特征以及合理应用特殊染色和免疫组化有助于同肾母细胞瘤、PNET和先天性中胚叶肾瘤等鉴别。展开更多
目的探讨肾透明细胞肉瘤(c lear cell sarcom a of the k idney,CCSK)的临床病理特点、免疫表型特征及鉴别诊断。方法应用HE和免疫组化vim entin、bc l-2、desm in、S-100蛋白、CD99、CD34、CD117、CK、EMA染色,观察2例CCSK的病理组织...目的探讨肾透明细胞肉瘤(c lear cell sarcom a of the k idney,CCSK)的临床病理特点、免疫表型特征及鉴别诊断。方法应用HE和免疫组化vim entin、bc l-2、desm in、S-100蛋白、CD99、CD34、CD117、CK、EMA染色,观察2例CCSK的病理组织学形态,并复习文献。结果镜下见瘤细胞为上皮样或短梭形,被分枝状纤维血管间质分隔成巢团状,部分区域见黏液样变性微囊肿和细胞外胶原玻璃样变类似骨样组织的硬化型等形态变异。免疫组化示:瘤细胞vim entin和bc l-2弥漫阳性,余为阴性。结论CCSK是一种罕见的儿童期恶性肾肿瘤,诊断主要依靠组织病理学和免疫组化,熟悉其形态学变异有利于与其它类似病变如肾母细胞瘤、先天性中胚叶肾瘤、肾恶性横纹肌样瘤、原始神经外胚叶肿瘤等鉴别。展开更多
文摘A case report by Liu et al describes the characteristics of metastatic clear cell sarcoma(CCS)of the pancreas and provides valuable therapeutic insights for this rare malignancy.This case is interesting because of its rarity,suggesting that the pancreas may be a potential target organ for CCS,either primary or metastatic.At the same time,the authors also emphasize the importance of regular postoperative follow-up for timely detection of recurrent lesions,as CCS is characterized by a high degree of malignancy and a high rate of recurrent metastases.Considering that CCS of the gastrointestinal tract is easily confused with malignant melanoma(MM)of the gastrointestinal tract,here we compare the clinical features,histopathological and immunohistochemical characteristics,diagnosis,treatment,and prognosis of CCS and MM of the gastrointestinal tract,hoping to provide a reference for clinical work.
文摘Primary or secondary clear cell sarcoma of the pancreas is an exceedingly rare and aggressive disease.In addition to pathology,molecular analysis is pivotal in differential diagnosis,especially with malignant melanoma.A key aspect in identifying clear cell sarcoma is specific genetic alterations,notably the translocation of t(12;22)(q13;q13),a diagnostic hallmark of this sarcoma subtype,which is absent in malignant melanoma.Treatment of primary clear cell sarcoma of the pancreas is the same as that for adenocarcinoma.
基金Supported by National Research Foundation of Korea,No.NRF-2022R1A2C1003638The Basic Research Lab Program through the NRF of Korea Funded by The Ministry of Science and ICT,No.2022R1A4A1025557.
文摘This editorial comments on the study by Liu et al investigating pancreatic metastasis of clear cell sarcoma(CCS)published in the World Journal of Clinical Cases.CCS is a rare and aggressive melanocytic tumor,that typically arises from tendons and aponeuroses of the limbs,and metastasizes to the lungs,bones,and brain.However,pancreatic metastasis has rarely been reported,presenting unique diagnostic and therapeutic challenges.Elucidating the clinical characteristics,imaging features,prognostic factors,and treatment outcomes of patients with pancreatic CCS metastasis is crucial.Surgery remains an effective management strategy for CCS.However,the high recurrence rate and low effectiveness of traditional adjuvant treatments necessitate a shift towards more personalized and targeted treatment plans.Research is needed to investigate and validate novel therapeutic approaches specifically tailored to the distinct genetic and molecular characteristics of rare malignancies like CCS.Additionally,the development of late metastases after a long disease-free interval is common in CCS patients.Therefore,routine postoperative surveillance for metastasis using computed tomography,magnetic resonance imaging,bone scans,and positron emission tomography scans is crucial.Moving forward,enhanced collaboration,investigation,and creative thinking among scientists,medical professionals,and legislators are essential to gain a deeper understanding of these rare presentations.
文摘Clear cell sarcoma of the kidney （CCSK） is a rare and highly malignant tumor which is usually confused with other kidaey tumors We experienced such a patient and present report this.
文摘We experienced a case of small, round-cell malignant neoplasm diagnosed by touch smear cytology and histopathology when an open biopsy was performed in a 50-year-old Japanese woman. She was suspected of having a cervical spine tumor after surgery for cervical spine foraminal stenosis. After consent, the cervical spine tumor histologically diagnosed by an open biopsy was confirmed to be Ewing sarcoma (EWS) by genetic testing. EWS belongs to a group of small, round-cell tumors that are morphologically similar and often difficult to differentiate. After the open biopsy, the present patient received radiotherapy, and her plasma level of Pro-Gastrin-Releasing-Peptide was decreased (217.2 pg/ml before surgery to 30.3 pg/ml;reference value: 0 - 80 pg/ml). We herein report the process for making the final diagnosis by focusing on the intraoperative cytology, histopathology, and immunohistochemical findings. Our diagnosis was validated by karyotyping and a fluorescence <em>in-situ</em> hybridization analysis.
文摘目的:透明细胞乳头状肾细胞肿瘤(clear cell papillary renal cell tumor,CCPRCT)是一种少见但重要的肾肿瘤类型,与其他肾细胞肿瘤具有类似的形态学特征,易导致误诊。本研究旨在探讨CCPRCT的临床病理特征、诊断及鉴别诊断要点,以提高其病理诊断的准确性。方法:收集15例CCPRCT患者,观察其临床及影像学特点,分析其镜下形态、免疫表型,并复习相关文献。结果:15例患者中,男10例,女5例,年龄为(54±17)岁,10例患者肿瘤位于左肾,5例患者肿瘤位于右肾。组织学上肿瘤均由增厚的纤维囊包裹,并局限于肾实质内;肿瘤细胞排列成乳头状、管状、囊状、腺泡及实性等结构;乳头由小到中等大小、透明细胞质的单层细胞组成,肿瘤均为世界卫生组织/国际泌尿病理学会(World Health Organization/International Society of Urological Pathology,WHO/ISUP)1级或2级;细胞核反极性分布,即核上移,靠近腔面,远离基底,类似鲨鱼牙齿排列。免疫组织化学染色显示:细胞角蛋白7、碳酸酐酶9(carbonic anhydrase 9,CA9)和高分子量细胞角蛋白(34βE12)均为阳性表达;α甲基酰基辅酶A消旋酶、CD117、转录因子E3均为阴性表达;CCPRCT中典型的CA9表现方式是“U型”着色,即腔缘不表达,基底和侧面表达。患者术后随访均未见复发或转移。结论:CCPRCT是一种惰性肾细胞肿瘤,预后好,临床可能存在过诊断,其形态学为特征性的核朝向腔缘的线性排列,特殊的免疫表型CA9呈“U型”阳性,可以与其他肾细胞肿瘤区分开来,但仍然需要积累更多患者来阐释其预后。
文摘目的:探讨儿童肾透明细胞肉瘤(clear cell sarcoma of the kidney,CCSK)的临床病理特点与鉴别诊断。方法:观察1例儿童肾透明细胞肉瘤的病理学形态、特殊染色及免疫表型特征并复习有关文献。结果:片内显示为小细胞肿瘤,呈弥漫性分布,部分细胞丰富,密集,部分区域细胞排列较松散,以分枝状纤维血管间质分隔肿瘤细胞呈不规则索状和小团状为结构特征,细胞呈圆形、多角形或梭形,细胞核呈圆形、卵圆形或棒状,核染色质呈细颗粒状,核膜较薄,核仁不明显。网状纤维染色:网状纤维密布于单个或小团肿瘤细胞之间。Masson三色:肿瘤间质小血管周纤维组织呈绿色。免疫组化染色:Vim、Bcl2和CD99(+),其他(-)。结论:CCSK是少见的小儿恶性肾肿瘤,诊断主要依赖于病理学,熟悉CCSK典型形态学特征以及合理应用特殊染色和免疫组化有助于同肾母细胞瘤、PNET和先天性中胚叶肾瘤等鉴别。
文摘目的探讨肾透明细胞肉瘤(c lear cell sarcom a of the k idney,CCSK)的临床病理特点、免疫表型特征及鉴别诊断。方法应用HE和免疫组化vim entin、bc l-2、desm in、S-100蛋白、CD99、CD34、CD117、CK、EMA染色,观察2例CCSK的病理组织学形态,并复习文献。结果镜下见瘤细胞为上皮样或短梭形,被分枝状纤维血管间质分隔成巢团状,部分区域见黏液样变性微囊肿和细胞外胶原玻璃样变类似骨样组织的硬化型等形态变异。免疫组化示:瘤细胞vim entin和bc l-2弥漫阳性,余为阴性。结论CCSK是一种罕见的儿童期恶性肾肿瘤,诊断主要依靠组织病理学和免疫组化,熟悉其形态学变异有利于与其它类似病变如肾母细胞瘤、先天性中胚叶肾瘤、肾恶性横纹肌样瘤、原始神经外胚叶肿瘤等鉴别。
