Clear cell sarcoma of the kidney--a case report

Clear cell sarcoma of the kidney (CCSK) is a highly malignant childhood tumor, distinguished from classic Wilms' tumor by its propensity to metastasize to the skeletal system. Authors described a case of CCSK from a 3-year-old boy in the right kidney, showing various histologic features, such as classic, epithelioid, trabecular, neurilemmoma-like, cystic and entrapped collecting tubular pattern. Ultrastructurally epithelial differentiation was absent. Immunohistochemically, none of the intrinsic tumor cells showed positive staining with the antibodies against the keratin, S-100 protein, carcinoembryonic antigen, vimentin, desmin and myoglobin, suggesting primitive mesenchymal cell in origin.     

Clear cell sarcoma: a clinicopathological study of 11 cases

The clinicopathologic, ultrastructural and immunohistochemical features of eleven cases of clear cell sarcoma are described. There were 6 males and 5 females with an average age of thirty-six (10-59 years). Tumors were found arising from the tendons, aponeuroses and fascial structures with a predilection for the lower and upper extremities. Follow-up data was available in 8 patients. Five of them are alive. Nevertheless, 3 of the five showed evidence of recurrence or metastasis. The other 3 patients died of tumor with metastasis. Microscopically, the tumors were composed of short fascicles of fusiform cells with a clear to eosinophilic cytoplasm and vesicular nuclei with prominent nucleoli. Melanin was demonstrated in 5 cases and S-100 was known present focally in all cases, but no positive keratin staining was obtained. Electron microscopic studies revealed cell attachments and mature melanosomes. The exact histogenesis remains obscure, but our ultrastructural and immunohistochemical findings support the idea of neural crest origin of this tumor.     

Clear cell sarcoma of kidney: a clinicopathological study of eight cases from Malaysia

Eight cases of clear cell sarcoma of kidney were seen in the Department of Pathology, University Hospital, Kuala Lumpur, Malaysia over the 16-year period from 1973 to 1989. Five of the patients were males. Six patients were Malay, one Chinese and one Indian. The patients' ages ranged from 8 months to 3 years. Clear cell sarcoma was the original diagnosis in two patients while six were diagnosed as blastemal-predominant Wilms' tumours at presentation. Metastases developed in five patients. Metastatic sites included the thoracic vertebra, skull, orbit, humerus, radius, ulna, shoulder, lung and liver. The prolonged survival, of 9 years and 9 months, seen in one patient despite omission of Adriamycin (doxorubicin) from the chemotherapeutic protocol is highlighted. We also emphasise the histological factors which are of help in differentiating clear cell sarcoma from Wilms' tumour.     

Clear cell sarcoma of the kidney. An immunohistochemical study

Three cases of clear cell sarcoma of the kidney (CCSK) and 5 cases of Wilms' tumor were investigated immunohistochemically to examine the expression of tissue-specific intermediate filaments (cytokeratin, vimentin, and desmin) and myoglobin. In CCSK, tumor cells were negative for cytokeratin, except for occasional tubular structures, and vimentin was demonstrated in only one case. In Wilms' tumor, epithelial components were positive for cytokeratin and stromal cells were positive for vimentin, while no staining was found in blastemal cells for either. Both desmin and myoglobin were negative in all tumor cells except for skeletal muscle cells in Wilms' tumor. In the current study, some neoplastic cells in CCSK were revealed to be of mesenchymal nature, but blastemal cells in Wilms' tumor were not.     

Clear cell sarcoma of the penis

The case of a 10-year-old boy in whom clear cell sarcoma occurred at the root of the penis is reported. The tumor cells were positive for S-100 protein, and ultrastructural examination showed the presence of melanosomes.     

Clear cell sarcoma of the stomach

AIMS: To investigate the role of the tumour suppressor gene PTEN in the tumorigenesis and growth of sporadic vestibular schwannomas, and to characterize the cellular distribution of the PTEN protein in relation to the MIB-1 proliferation index in these tumour. METHODS AND RESULTS: Immunoexpression of the PTEN protein was observed within the neoplastic Schwann cells in 21 out of 30 sporadic schwannomas examined (70%). PTEN expression was consistently stronger in Antoni A areas than in Antoni B areas. High levels of PTEN immmunoexpression in schwannomas were associated with an increased MIB-1 labelling index. Occasionally, vascular endothelial cells also showed PTEN immunoreaction. By polymerase chain reaction-single strand conformation polymorphism screening, no mutations were found in the complete protein coding region of the PTEN gene. CONCLUSIONS: The PTEN tumour suppressor gene is expressed in the majority of sporadic schwannomas. The maintained expression of the PTEN protein, together with the lack of detectable mutations in this gene, suggests that the function of the PTEN tumour suppressor gene is not altered in sporadic vestibular schwannomas.     

Clear cell sarcoma of the mediastinum

A 59-year-old woman presented with a large mediastinal mass. At thoracotomy, the mass was found tightly adherent to the esophageal wall and right lower lobe of the lung. Histological examination showed a solid tumor composed of closely packed nests of cells with clear and eosinophilic cytoplasm, which were strongly and diffusely positive for S100 protein but negative for HMB45 and Melan-A. The diagnosis of clear cell sarcoma was supported by demonstrating the presence of an EWS gene rearrangement by fluorescence in situ hybridization. There was no evidence that this lesion represented metastatic disease. To the best of our knowledge, primary mediastinal clear cell sarcoma has not been previously reported in the literature. We present the case and discuss the differential diagnosis.     

Intracranial metastasis from clear cell sarcoma of the kidney--a case report.

Childhood kidney tumors seldom metastasize into the cranial cavity unless it is a special histological variant. We report a 4-year-old boy with multiple intracranial metastases in the left parietotemporal and right cerebellar area from primary clear cell sarcoma of the kidney without evidence of bony metastases. Metastatic tumor revealed nests of uniformly polygonal cells with clear cytoplasm demarcated by delicate fibrovascular arcades. Tumor cells were positive for vimentin and negative for cytokeratin, S-100 protein, desmin, and myoglobin. Cellular proliferation rate measured by PCNA, and Ki-67 was not significantly different between primary tumor mass and metastatic brain lesion. Expression of p53 oncoprotein was not evident in both lesions. These findings suggested that the relapse and metastasis of clear cell sarcoma of the kidney was probably due to regrowth of micro-metastases which were present at an early stage of disease.     

Clear cell sarcoma. An immunohistochemical and ultrastructural study

The histogenesis of clear cell sarcoma was investigated by immunohistochemical examination of five tumors (two melanotic and three amelanotic) and electron microscopic examination of two of these tumors (one melanotic and one amelanotic). Melanin production was observed histologically in two of the tumors. The cytoplasm of cells in both types of tumor contained various numbers of melanosomes. Melanoma-specific antibody (HMB-45), anti-S-100 protein, and anti-vimentin antibodies gave positive reactions in four tumors, while all tumors showed Leu-7 immunoreactivity. No cytokeratin or epithelial membrane antigen (EMA) was detected immunohistochemically in any tumor. The immunoreactivity of this type of tumor with HMB-45 antibody strongly suggests melanocytic differentiation rather than schwannian or synovial differentiation. The reaction of the cells of one tumor with only Leu-7 indicates the existence of undifferentiated clear cell sarcoma of neuroectodermal origin that does not show definite melanocytic differentiation.     

Clear cell soft tissue sarcoma. Clinical, histopathological and prognostic study of 36 cases

Thirty six cases of clear cell sarcoma of soft tissue are reported. The median age was 44 years (5 to 80 years). The principal sites were the foot (11 cases), the hand and wrist (7 cases) and the knee (6 cases). The architecture was fascicular with lobular arrangement of cells delimited by delicate fibrous septa intimately bound to tendons or aponeuroses. Tumoral cells were round or fusiform with abundant clear cytoplasm sometimes epithelioid with round nuclei and prominent nucleoli. The mitotic rate was evaluated to 9/10 HPF. S100 protein was expressed in 33/36 cases and HMB45 marked 29/31 cases, without expression of cytokeratin. Three-year and 5-year survival rate were respectively 72% and 62%. Prognosis factors for global survival were efficiency of initial treatment with distal location and necrosis and FNCLCC grade. The distinction of clear cell sarcoma from metastatic melanoma is important because of the difference of prognosis.     

Clear cell lymphoma: a clinicopathological study of four cases

Four cases of clear cell lymphoma were studied by means of light and electron microscopy, enzyme-histochemistry, and E- and EAC-rosette formation. On light microscopic examination the tumors were seen to be composed mainly of round, oval, or slight irregular cells with water-clear, abundant cytoplasm. The neoplastic cells has round, oval, or convoluted nuclei with fine, evenly dispersed chromatin and one or more small but distinct nucleoli. On electron microscopic examination the clear cell lymphoma were characterized by lymphoid cells with striking electron lucent cytoplasm with few organelles. According to E-rosette test and enzyme-histochemical findings, the investigators proposed that the clear cell lymphoma may be derived from T-cell lineages. Differentiated diagnoses of clear cell lymphoma from B-immunoblastic sarcoma, pleomorphic cell lymphoma, clear cell sarcoma, and clear carcinomas derived from lung, ovary, or kidney were discussed.     

肾透明细胞肉瘤的临床病理及免疫表型特征

目的 探讨肾透明细胞肉瘤（clear cell sarcoma of the kidney,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vimentin、bcl-2、desmin、S-100蛋白、CD99、CD34、CDll7、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vimentin和bcl-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。     

Spinal congenital dermal sinus--experience of 5 cases over a period of 10 years.

Spinal congenital dermal sinus (CDS) is a rare entity which supposedly results from the failure of the neuroectoderm to separate from the cutaneous ectoderm during the process of neurulation. The lesions are most frequent at the lumbosacral followed by the occipital region. CDS of the thoracic region is very rare. The patients with spinal CDS present with meningitis and/or mass effect from the associated inclusion tumor. They are usually dermoid or epidermoid cysts. Teratoma is rarely associated. The authors experienced 5 cases of spinal CDS over a 10 year period. Of the 5 cases, 2 were at thoracic and 3 were at lumbosacral levels. Dermoid cyst, epidermoid cyst and teratoma were associated in one case each. Two cases presented with neurological deficit and meningitis while an additional case presented with neurological deficit and a history of probable meningitis. Pain was present in 2 cases. Magnetic resonance imaging played an important role in the diagnosis of the lesion and planning of surgery. All the cases showed a good response to surgery even though one patient had persistent neurological deficit.     

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。     

具有乳头状结构的透明细胞肾细胞癌临床病理分析

目的探讨具有乳头状结构的透明细胞肾细胞癌的临床病理学特征及诊断与鉴别诊断。方法对12例具有乳头状结构的透明细胞肾癌进行光镜及免疫组织化学观察。结果12例中男性11例,女性1例,平均53岁。10例发生于右侧肾脏,2例发生于左侧肾脏,主要症状为血尿、腰腹痛或无明显症状仅体检发现。12例肿瘤组织形态学上都具有乳头状结构区域,占整体肿瘤的10%～30%,其他区域为经典型透明细胞癌排列区,乳头结构为原发性簇状微乳头、小管乳头或纤维血管轴心乳头及继发性囊性变形成、坏死形成或人工因素形成的乳头;免疫组化:CK(12/12)、CD10(9/12)、vimentin(11/12)、CK7(5/12)、AMACR(3/12)阳性,CD117和TFE3阴性。9例患者随访2至66个月,5例无瘤生存,3例复发,其中2例死亡。结论具有乳头状结构的透明细胞肾细胞癌属于透明细胞肾细胞癌范畴,以形态学上具有乳头状结构为特点,免疫表型与经典型透明细胞肾癌基本一致并具有一定的差异,这些特点对临床病理诊断具有很高的价值。     

颞部软组织透明细胞肉瘤一例

患者女,37岁。因左颞部复发性肿瘤月余,于2003年3月31日就诊。1年半前发现局部肿块,缓慢生长,临床于11个月前行第1次手术切除,术中见肿瘤位于肌间筋膜内,略呈梭形,长径1.5cm,边界清,局部皮肤无色素痣。病理会诊意见为恶性黑色素瘤。遂入住上级医院行扩大切除术,病理报告未见残存肿瘤,术后进行了干扰素治疗和放疗。查体：浅表淋巴结不肿大,系统查体无异常发现。左颞部手术瘢痕旁深部触及肿块,直径约1cm,境界清,不活动,临床诊断恶性肿瘤术后复发,手术切除送检。