Metastatic clear cell sarcoma of the pancreas:A sporadic cancer

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.

Metastatic clear cell sarcoma of the pancreas:A rare case report

BACKGROUND Clear cell sarcoma(CCS)is a rare soft-tissue sarcoma.The most common metastatic sites for CCS are the lungs,bones and brain.CCS is highly invasive and mainly metastasizes to the lung,followed by the bone and brain;however,pancreatic metastasis is relatively rare.CASE SUMMARY We report on a rare case of CCS with pancreatic metastasis in a 47-year-old man.The patient had a relevant medical history 3 years ago,with abdominal pain as the main clinical manifestation.No abnormalities were observed on physical examination and the tumor was found on abdominal computed tomography.Based on the medical history and postoperative pathology,the patient was diagnosed with CCS with pancreatic metastasis.The patient was successfully treated with surgical interventions,including distal pancreatectomy and sple-nectomy.CONCLUSION This report summarizes the available treatment modalities for CCS and the importance of regular postoperative follow-up for patients with CCS.

Metastatic clear cell sarcoma of the pancreas:From diagnosis to treatment

This article presents a comprehensive case report on an uncommon instance of metastatic clear cell sarcoma(CCS)originating from the pancreas.The high mortality rate of pancreatic carcinoma underscores the importance of precise diagnosis and early detection.The authors report a novel case of CCS with pancreatic metastasis,detailing successful surgical intervention through distal pancreatectomy and splenectomy,resulting in favourable outcomes.This study highlights the standard role of surgery in treating advanced CCS and emphasizes preoperative imaging and thorough patient history assessment.This article also underscores the necessity for long-term surveillance due to the potential for recurrence or metastasis.Despite the favourable recovery postsurgery,the absence of subsequent follow-up evaluation prompts consideration of the need for extended monitoring.This article raises questions about the nature of the pancreatic lesion and suggests the possibility of a primary lesion.Further evidence is crucial to establish the correlation between the features related to the development of the patient's primary and metastatic tumours.In conclusion,this study offers valuable insights into metastatic CCS of the pancreas,highlighting the importance of regular postoperative follow-up for improved outcomes through early detection and intervention.

Clear cell sarcoma metastasizing to the pancreas

In this editorial based on a case report,we delve into a seldom-seen occurrence of clear cell sarcoma featuring pancreatic metastasis in a 47-year-old male patient.Recognized for its typical tendency to metastasize to the lungs,bones,and brain,clear cell sarcoma rarely extends its reach to the pancreas.Despite the initial absence of discernible abnormalities during the patient's physical examination,the manifestation of abdominal pain prompted further investigation.Subsequent abdominal computed tomography brought to light the presence of a pancreatic tumor,culminating in the definitive diagnosis of clear cell sarcoma with pancreatic metastasis.The successful management of this atypical presentation involved a series of surgical interventions,including distal pancreatectomy and splenectomy.This report not only sheds light on the infrequent manifestation of clear cell sarcoma within the pancreas but also underscores the pivotal role of vigilant postoperative follow-up in addressing this rare sarcoma.The emphasis on postoperative care serves as a crucial aspect of the broader narrative,acknowledging the need for ongoing monitoring and management to ensure a comprehensive and successful treatment trajectory for patients with this unique presentation of clear cell sarcoma.

Primary clear cell sarcoma of soft tissue in the posterior cervical spine invading the medulla oblongata:A case report

BACKGROUND Clear cell sarcoma(CCS)is a rare and highly malignant soft tissue tumor,usually occurring in the deep soft tissues of the distal tendons and aponeurosis of the extremities,especially the feet and knees.CCS originating in the head and neck is extremely rare.The clinical manifestations of CCS in the head and neck are not typical,and the imaging manifestations have certain characteristics,but the diagnosis still depends on pathological examination and genetic testing.CASE SUMMARY A 33-year-old male patient had paroxysmal headache for more than 4 years,accompanied by nausea and vomiting,which could be relieved after rest.Computed tomography angiography showed a left paraspinal soft tissue mass.Contrast-enhanced imaging showed obvious uneven enhancement with adjacent bone lytic destruction.Magnetic resonance imaging examination showed isosignal on T_(1)-weighted images,slightly high signal on T_(2)-weighted images(T_(2)WI),high signal on Tirm fat suppression sequence,significantly high signal on diffusion weighted imaging,and obvious and uneven enhancement.The lesion invaded the anterior medulla oblongata through the left atlantoaxial foramen and compressed the cervical spinal cord on T_(2)WI.Primary CCS of soft tissue was diagnosed by pathology and genetic examination.CONCLUSION CCS should be considered in the differential diagnosis of soft tissue tumors of the head and neck,and their diagnosis depends on pathological examination and genetic testing.

Paraplegia after transcatheter artery chemoembolization in a child with clear cell sarcoma of the kidney:A case report

BACKGROUND Transcatheter arterial chemoembolization(TACE)is a common treatment for inoperable malignant renal tumors.However,a series of complications may follow the TACE treatment.Spinal cord injury caused by the embolization of intercostal or lumbar arteries is extremely rare.CASE SUMMARY We describe a case with quite uncommon spinal cord injury after TACE in a 3-year-old child with clear cell sarcoma of the kidney.Sensory impairment beneath the T10 dermatomes and paraplegia on the day after TACE were found in this patient.Unfortunately,sustained paraplegia still existed for more than 2 mo after TACE despite the large dose of steroids and supportive therapy.CONCLUSION We should draw attention to an uncommon complication of paraplegia after TACE treatment in malignant renal tumors.Although it is rare,the result is disastrous.

Analysis of Sentinel Lymph Node Biopsy on Clear Cell Sarcoma Treatment

Clear Cell Sarcoma (CCS) is a rare soft tissue sarcoma that corresponds to a less than 1% of all sarcomas and is a high degree neoplasm with propensity to disseminate to regional lymph nodes. Regional lymphadenectomies have a controversial impact on sarcomas treatment and survival, even though those patients have higher local recurrence and lower survival. So, sentinel lymph node biopsy must be one option for better regional staging to STS with higher propensity to lymph node commitment. The authors demonstrate two cases of CCS submitted to surgery through compartmental resection associated with the sentinel lymph node biopsy. A literature review about soft tissue sarcoma and lymph node sentinel biopsy is shown as an initial experience of two cases.

Clear Cell Sarcoma of the Kidney-A Case Report

Clear cell sarcoma of the kidney (CCSK) is a rare and highly malignant tumor which is usually confused with other kidney tumors. We experienced such a patient and present report this. Case Report A 9-year old girl was admitted to a local hospital because of fever and a pain in her right abdomen. The computerized tomography showed:

Clear cell sarcoma of soft tissue in pleural cavity: A case report

BACKGROUND Clear cell sarcoma(CCS)of soft tissue is a rare malignant soft tissue sarcoma usually reported to locate at distal end of extremities and rarely at trunk.Herein,we report a case of CCS in pleural cavity.CASE SUMMARY A 31-year-old male was admitted for an uncertain mass in left pleural cavity in routine physical examination without any symptoms.A VATS surgery was performed to remove the tumor.The pathological finding displayed a cystic mass with 6.5 cm at the longest diameter,dark red in section and cysts could be found locally.A strong expression of S-100,HMB45 and Vimentin was detected in immunohistochemical staining,which was inclined to the diagnosis of the CCS of soft tissue.The patient refused chemotherapy,radiotherapy and targeted therapy because of the personal financial situation.Follow-up computed tomography scans were done at the 90th postoperative day and the 180th postoperative day,and no obvious sign of recurrence was found till now.CONCLUSION CCS of soft tissue also can be found in pleural cavity although in an extremely rare incidence.Radical resection is useful to improve the prognosis.

Clear cell sarcoma in unusual sites mimicking metastatic melanoma

BACKGROUND Clear cell sarcoma is an aggressive rare malignant neoplasm with morphologic and immunohistochemical similarities to malignant melanoma.Both disease entities display melanin pigment and melanocytic markers,making differentiation between the two difficult.Although clear cell sarcoma cases in the literature have mainly involved deep soft tissues of the extremities,trunk or limb girdles,we report here two cases of primary clear cell sarcoma in unusual sites and describe their clinicopathologic findings.CASE SUMMARY The first case involves a 37-year-old female,who presented with jaw pain and a submandibular mass.The second case involves a 33-year-old male,who presented with back pain and a thoracic spine tumor.Both cases showed tumors with diffuse infiltration of neoplastic cells that were positive for melanocytic markers,and in both cases this finding led to an initial diagnosis of metastatic melanoma.However,further analysis by fluorescence in situ hybridization(commonly known as FISH)showed a rearrangement of the EWS RNA binding protein 1(EWSR1)gene on chromosome 22q12 in both patients,confirming the diagnosis of clear cell sarcoma.CONCLUSION Distinction between clear cell sarcoma and malignant melanoma can be made by FISH,particularly in cases of unusual tumor sites.

Clear cell sarcoma of the jejunum—Surgical management in two patients with review of the literature

Clear cell sarcoma is usually described as a malignant melanoma of the soft tissues. The overall prognosis is poor because of delay in diagnosis and vague clinical symptoms. It is rarely involved in the gastrointestinal tract, and its diagnosis is often missed secondary to infrequent occurrence and histological resemblance to melanoma. We present two cases of primary CCS of the jejunum whose clinical presentations were complicated by lymph node involvement. Prompt diagnosis and potential aggressive surgical intervention may improve overall survival.

Clear Cell Pleomorphic Dermal Sarcoma: A Case Report and Literature Review

Introduction: Atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) are one spectrum of rare cutaneous neoplasms that typically arise in sun-exposed skin of older population. AFX/PDS is essentially diagnosis of exclusion requiring Immunohistochemical work-up to exclude other types of tumors. Case Report: We present a case involving an ulcerated solitary lesion on the scalp of an elderly man. Histological examination revealed that the dermal tumor was composed of large pleomorphic, epithelioid, and spindle cells with clear cytoplasm. These cells were negative for cytokeratins, melanocytes and smooth muscle markers, but positive for CD10. These findings are consistent with a diagnosis of clear cell (CC) PDS. Conclusion: PDS is a low-grade malignancy that can recur locally and metastasize, which is distinguished from AFX by its larger size and the presence of aggressive histopathologic features including deeper invasion into the subcutaneous tissue, tumor necrosis, and lymphovascular and/or perineural involvement. Among several histopathologic variants, the CC variant is extremely rare with only two cases of PDS reported in the literature to date.

Sorafenib Acts through VEGFR-2 Inhibition in a Metastatic Clear-Cell Sarcoma of the Kidney

We report here the case of a young patient with metastatic clear-cell sarcoma of the kidney resistant to standard chemotherapy, and with complete response under sorafenib treatment. The remarkable response of her tumor to sorafenib led us to study sorafenib molecular targets in the metastatic tissue. Background: Biomarkers predicting response to anti-angiogenic tyrosine kinase inhibitors remain to be identified. Methods and Findings: In this paper, we studied the molecular targets of sorafenib in the lung metastasis of a kidney clear-cell sarcoma. In a patient with complete response under sorafenib treatment, we showed high VEGFR2 expression by tumor endothelial cells from the lung metastasis. Conclusion: The original mechanistic results that we obtained using immunostainings and quantitative RT-PCR on laser-microdissected tumor endothelial cells have a direct application in daily clinical practice: metastatic tumors with a large angiogenic component should be tested for VEGFRs expression to consider anti-angiogenic tyrosine kinase inhibitor treatments.

Multiple clear-cell sarcomas of small intestine with parotid gland metastasis: A case report

Clear-cell sarcoma is a rare, malignant soft tissue tumor that displays melanocytic differentiation with a distinct molecular profile. It is rarely localized in the gastrointestinal tract. Herein we reported a case of multiple synchronous clear-cell sarcomas of the gastrointestinal tract with parotid gland metastasis. A 51-year-old male patient presented with a growing painless mass under the right ear. A preoperative positron emission tomography/computed tomography showed multiple intestinal masses and a mass in the right parotid with increased glucose uptake, and he underwent operative treatment with resection of three tumors in the jejunum and ileum and then received a right parotidectomy. Postoperative pathological examination showed that cells in the intestinal tumor were consistent with clear-cell sarcoma of the gastrointestinal tract, and the malignant cells in the parotid gland were similar to the intestinal tumor. Immunohistochemical studies revealed positive expression of HMB-45, Melan-A, and S-100. EWSR1 gene fusion transcripts were undetectable by fluorescence in situ hybridization.

儿童肾透明细胞肉瘤CT诊断及误诊分析

目的 分析儿童肾透明细胞肉瘤(CCSK)的影像表现,对本病影像学与临床表现加以总结,探究误诊因素,提高对本病认知度,提升诊断符合率。方法 对河南省儿童医院由病理确诊11名CCSK患者的影像资料展开回顾分析,全部患儿皆行CT扫描,对其影像与临床表现加以总结,并对误诊因素展开分析。结果 肿瘤皆是单侧发病,其中5例为右肾,6例为左肾,11例患儿发病年龄区间为5月9天至10岁,肿瘤多较大,密度混杂,均为单发,10例有囊变;9例肿瘤内见多发细小动脉,实性成分呈延迟渐进性强化,2例伴下腔静脉、同侧肾静脉癌栓。7例肾周血管充盈,骨转移与钙化灶分别为2例,包膜下积液1例。术前误诊10人,其中8人误诊为肾母细胞瘤(WT),1例误诊为先天性中胚叶肾瘤,1例误诊为肾脏横纹肌样瘤。结论 在影像表现上,CCSK和其它儿童肾肿瘤尤其是肾母细胞瘤高度接近,术前误诊几率高,发病年龄小,具不良预后,病程进展快,易发生囊变、坏死,增强肿瘤内多发细小动脉、延迟渐进性强化,还有鱼肉状、虎斑状、条纹状或者云絮状强化,肾周血管充盈多见,有助于与其他肾肿瘤相鉴别。

恶性胃肠道神经外胚层肿瘤临床病理分析

目的:研究恶性胃肠道神经外胚层肿瘤(malignant gastrointestinal neuroectodermal tumor,MGNET)的临床病理学和分子特征、诊断、鉴别诊断和预后,以提高对该病的认识。方法:报道2例我院诊断的恶性胃肠道神经外胚层肿瘤,分析临床和影像学特征、组织形态学、免疫表型、分子遗传学和预后,并回顾相关文献。结果:病例1、2分别为28岁、45岁女性,CT提示盆腔占位及肠壁增厚。大体表现为肠壁肿块,显微镜下短梭形肿瘤细胞排列成片状、巢状、腺泡状和假乳头状,伴有散在的多核巨细胞。2例肿瘤都表达S-100,伴有EWSR1(22q12)易位,诊断为恶性胃肠道神经外胚层肿瘤。病例1术后6个月,CT考虑术后复发。病例2术后16个月,PET-CT提示肝脏腹腔多发肿瘤转移,19个月后去世。结论:恶性胃肠道神经外胚层肿瘤是一种罕见且高侵袭性的软组织肿瘤。在临床工作中应考虑到此类罕见肿瘤,并合理选用免疫组化指标及基因检测。目前手术切除是主要的治疗方法,手术切除后的化疗尚无共识。

儿童肾透明细胞肉瘤的影像学表现

目的探讨分析儿童肾透明细胞肉瘤(clear cell sarcom a of the kidney,CCSK)的影像学特征,以提高对本病的认识。方法选取经手术病理证实的13例CCSK患儿的临床及影像学资料。采用CT、超声检查,将所得图像传入后处理工作站行冠状位及矢状位等平面重建。重点观察病灶的侧别、部位(皮质、髓质)、大小(最大横截面长径)、形态、边缘等。结果13例患儿均单侧发病,肿瘤均位于肾髓质区。肿瘤平均长径10.87 cm,边界清楚,9例(69.2%)轮廓光整,4例(30.8%)呈分叶状。CT平扫肿瘤密度不均,13例病灶内均出现囊变坏死,6例(46.2%)钙化,3例(23.1%)出血,11例(84.6%)肿瘤实性成分呈等低密度,1例呈稍高密度,1例呈等密度。行CT增强扫描,12例(92.3%)中度强化,1例轻度强化,10例(76.9%)增强皮质期肿瘤内可见多发细小肿瘤血管影,肿瘤内无强化的囊变坏死区与肿瘤实性成分相间呈虎斑条纹样改变。肿瘤灰阶超声表现均为肾内不均质肿块,彩色多普勒超声显示肿瘤内部均可见血流信号。9例(69.2%)无转移,4例(30.8%)出现其他部位多处转移。结论儿童CCSK CT及超声表现具有一定的特征性,联合CT和超声检查有助于提高该病的诊断准确率。

儿童肾透明细胞肉瘤CT影像表现特征

目的探讨儿童肾透明细胞肉瘤(CCSK)的CT影像学表现特点。方法回顾性分析2014年8月至2022年4月经手术及病理证实的11例CCSK患者的CT平扫、三期增强扫描图像及病理免疫组化等资料,结合文献复习进行分析总结。结果位于右肾5例,左肾6例,肿瘤最大长径4~18 cm,5例大于10 cm,均呈膨胀性生长,CT平扫肿瘤呈等低混杂密度,肿瘤周边有囊变8例(72.7%),增强扫描动脉期肿瘤内有多发强化细小血管影9例(81.8%),实质期呈“虎斑样”强化6例(54.5%),延迟期肿瘤实性成分渐进强化9例(81.8%)。2例初诊时出现骨转移,2例腹膜后淋巴结转移。结论儿童CCSK的CT影像学表现具有一定的特征,有助于其诊断和鉴别诊断。

腹股沟透明细胞肉瘤1例并文献复习

目的探讨软组织透明细胞肉瘤的诊断治疗。方法分析现有病例临床资料并文献复习,总结软组织透明细胞肉瘤诊断及治疗。结果软组织透明细胞肉瘤采用手术或活检可以确定诊断,采用以手术为主的综合治疗。结论通过软组织透明细胞肉瘤的临床资料的认真分析,该病恶性度高,预后差,宜采用以手术为主的综合治疗。

透明细胞肉瘤临床病理分析

目的探讨透明细胞肉瘤(clear cell sarcoma,CCS)的临床病理特点及诊断与鉴别诊断。方法对9例CCS进行临床病理分析,并行组织学、免疫组化染色和电镜观察。结果9例CCS患者年龄19～62岁,平均38.6岁;肿瘤多数位于肢体末端(6/9)。组织学观察:肿瘤由被纤维组织分隔成巢或束状的胞质透亮或淡伊红染的上皮样细胞和梭形细胞构成,核圆形或卵圆形,较空淡,可见明显核仁。1例见少数多核巨细胞,1例胞质内含色素颗粒,2例间质黏液样变性,1例局部侵及表皮。术后随访6～24个月,6例中2例局部复发伴转移。免疫组化观察:9例CCS均表达vimentin和HMB-45,7/9例S-100蛋白阳性,5/9例NSE阳性,4/9例melan-A阳性,1/9例Syn阳性。电镜观察:胞质内可见不同时期的黑色素小体。结论CCS是好发年轻人肢体末端的软组织肉瘤,预后不良,属高度恶性。诊断应结合临床与病理形态,免疫组化在诊断和鉴别诊断中起重要作用。