Intriguing Case: Primitive Pelvic Sarcoma Resembling Clear Cell Sarcoma of Kidney

Clear cell sarcoma of kidney (CCSK) is an aggressive childhood renal tumor of unknown histogenesis that has not been reported to occur outside the kidney. The article describes an extrarenal neoplasm arising in the pelvic soft tissues of a 13-year-old boy that was composed predominantly of uniform mesenchymal cells with optically clear cytoplasm supported by an arborizing network of small blood vessels, which was indistinguishable in appearance from CCSK. The electron microscopic findings, although nonspecific, were essentially identical to those of CCSK, with tumor cells displaying fine chromatin, electron-lucent cytoplasm, and intercellular collagen but no evidence of tissue-specific differentiation. Immunocytochemical studies showed positivity for vimentin but negative results for desmin, myoglobin, cytokeratin, epithelial membrane antigen, S-100 protein, neuron-specific enolase and factor VIII-related antigen. Tumor cells were also nonreactive with Ulex lectin. This unusual pelvic tumor and CCSK may both derive from primitive mesenchymal cells and may represent phenotypic but not necessarily histogenetic analogs.     

Nondifferentiated-Type Small Cell Sarcoma of Kidney in a Young Woman

A case of rapidly fatal small cell sarcoma of kidney in a young woman is described. Histologic, immunohistochemical, and ultrastructural studies demonstrated some features not reported previously in the literature, such as the arrangement of vimentin intermediate filaments in small irregular masses and the presence of various types of cytoplasmic projections. These findings, together with the observation of rare neoplastic elements with immunophenotypic and subcellular aspects of histiocytic and myofibroblastic differentiation, suggest that this tumor should be included in a group of small cell sarcomas of kidney lacking features of differentiation and should be considered a new variant occurring in adulthood.     

6例透明细胞肉瘤的临床病理及免疫组化观察

对６例罕见的透明细胞肉瘤作了光镜和免疫组化观察。光镜下瘤细胞呈多边形，胞界不清，胞浆丰富透明，含ＰＡＳ阳性物，核大，空泡状，核仁大，瘤细胞聚集Ｌｙｓｏｚｙｍｅ阳性和Ｆｉｂｒｏｎｅｃｔｉｎ，Ｖｉｍｅｎｔｉｎ，Ｄｅｓｍｉｎ，Ｃｙｔｏｋｅｒａｔｉｎ，ＣＥＡ阴性。我们的资料支持该瘤起源于神经嵴的观点。     

Ultrastructural Similarities and Differences of Synovial Sarcoma, Epithelioid Sarcoma, and Clear Cell Sarcoma of the Tendons and Aponeuroses

The ultrastructural features of synovial sarcoma, epithelioid sarcoma, and clear cell sarcoma of the tendons and aponeuroses were compared to identify differential markers and similarities. A continuous spectrum of modulation of morphologic features of synovial and epithelioid sarcomas was observed. Biphasic synovial sarcoma with pseudoglandular and stromal components represents one extreme of this spectrum. The gradual disappearance of the pseudoglands and the formation of nests of epithelial-like cells, which are characteristic of epithelioid sarcoma, were observed. The cells of clear cell sarcoma, as well as those of synovial and epithelioid sarcomas, form epithelial-like islands; however, the presence of premelanosomes in the former is a feature of neural crest derivatives.     

Ultrastructural Similarities and Differences of Synovial Sarcoma,Epithelioid Sarcoma,and Clear Cell Sarcoma of the Tendons and Aponeuroses

The ultrastructural features of synovial sarcoma, epithelioid sarcoma, and clear cell sarcoma of the tendons and aponeuroses were compared to identify differential markers and similarities. A continuous spectrum of modulation of morphologic features of synovial and epithelioid sarcomas was observed. Biphasic synovial sarcoma with pseudoglandular and stromal components represents one extreme of this spectrum. The gradual disappearance of the pseudoglands and the formation of nests of epithelial-like cells, which are characteristic of epithelioid sarcoma, were observed. The cells of clear cell sarcoma, as well as those of synovial and epithelioid sarcomas, form epithelial-like islands; however, the presence of premelanosomes in the former is a feature of neural crest derivatives.     

Ultrastructural Study of a Clear Cell Sarcoma Suggesting Schwannian Differentiation

A clear cell sarcoma that developed on the left back of a 19-year-old Japanese female was studied ultrastructurally. The findings included basal laminae, desmosomelike junctions, cytoplasmic inter-digitations, lamellar bodies, and immature melanosomes. These findings suggest schwannian differentiation of this tumor, since they are not usual features of malignant melanoma except for melanosomes. Therefore this tumor seems to be more akin to malignant schwannoma than to malignant melanoma.     

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 ceils 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 immunohisto-chemically 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. Acta Pathol Jpn 39: 321 327, 1989.     

Juxtaglomerular Cell Tumor of the Kidney: A Case Report with Electron Microscopic and Flow Cytometric Investigation

A juxtaglomerular cell tumor (JGCT) was found in the right kidney of a 17-year-old female who had suffered from a headache of 1-year duration and hypertension. CT and US were diagnostic in association with determination of plasma renin activity. Light and electron microscopic, immunohistochemi-cal, and flow cytometric investigation of the resected tumor confirmed the diagnosis of JGCT with demonstration of ultra-structural features and distribution pattern of characteristic secretion granules of various sizes and shapes, coexistence of mast cells within the tumor (JGCT cells/mast cells = 10), and perivascular architecture.     

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.     

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。     

Human Osteogenic Sarcoma: Fine Structural Localization of Adenosine Triphosphatase

The localization of ATPases in 7 osteogenic sarcomas of osteoblastic, chondroblastic and fibroblastic type was investigated at the fine structural level using two types of substrates: one with lead as capturing ion and one with strontium (the latter presumed to reveal sites of Na⁺-K⁺-dependent transport ATPase).

Reaction product with the lead-ATP medium was located on the plasma membrane and the membranes bordering subjacent vesicles and vacuoles in all the various types of osteoblastlike and fibroblastlike cells and also in types 1 and 3 chon-droblastlike cells, and multinucleated giant cells believed to be neoplastic. Furthermore, deposits of reaction product were demonstrated in lysosome-like organelles in all the aforementioned cells. Except in the case of chondroblastlike cells, precipitates marking the localization of enzyme were confined to areas of the plasma membrane where adjacent cells were closely applied (the free surface lacked precipitates). In chondroblastlike cells the reaction product was usually deposited along the whole plasma membrane. Presence of L-Homoarginine or L-Tetramisole in the incubation medium in concentrations that have been shown to completely abolish alkaline phosphatase activity did not affect the occurrence of the reaction product with ATP as substrate indicating that the enzyme hydrolysing ATP was substrate-specific.

Reaction product marking sites of Na⁺-K⁺-dependent ATPase was confined to plasma membranes and lysosomes of cells in vessel walls.

The observations strengthen the notion obtained in studies on the localization of alkaline phosphatase, namely that osteoblastlike, chondroblastlike, and fibroblastlike cells in osteogenic sarcomas are histogenetically related to one another and to those multinucleated giant cells that presumably are of a neoplastic nature.     

Practical Electron Microscopy of Pediatric Renal Tumors

Presented is a short practical review of the key features and principal applications of electron microscopy in the diagnosis of childhood and adult “pediatric” renal tumors.     

Human Osteogenic Sarcoma: Fine Structural Localization of Adenosine Triphosphatase

The localization of ATPases in 7 osteogenic sarcomas of osteoblastic, chondroblastic and fibroblastic type was investigated at the fine structural level using two types of substrates: one with lead as capturing ion and one with strontium (the latter presumed to reveal sites of Na⁺-K⁺-dependent transport ATPase).

Reaction product with the lead-ATP medium was located on the plasma membrane and the membranes bordering subjacent vesicles and vacuoles in all the various types of osteoblastlike and fibroblastlike cells and also in types 1 and 3 chon-droblastlike cells, and multinucleated giant cells believed to be neoplastic. Furthermore, deposits of reaction product were demonstrated in lysosome-like organelles in all the aforementioned cells. Except in the case of chondroblastlike cells, precipitates marking the localization of enzyme were confined to areas of the plasma membrane where adjacent cells were closely applied (the free surface lacked precipitates). In chondroblastlike cells the reaction product was usually deposited along the whole plasma membrane. Presence of L-Homoarginine or L-Tetramisole in the incubation medium in concentrations that have been shown to completely abolish alkaline phosphatase activity did not affect the occurrence of the reaction product with ATP as substrate indicating that the enzyme hydrolysing ATP was substrate-specific.

Reaction product marking sites of Na⁺-K⁺-dependent ATPase was confined to plasma membranes and lysosomes of cells in vessel walls.

The observations strengthen the notion obtained in studies on the localization of alkaline phosphatase, namely that osteoblastlike, chondroblastlike, and fibroblastlike cells in osteogenic sarcomas are histogenetically related to one another and to those multinucleated giant cells that presumably are of a neoplastic nature.     

Glycogen-rich Clear Cell Carcinoma of the Breast An Autopsy Case

An autopsy case of glycogen-rich clear cell carcinoma (GRCCC) which arose in the right breast of a 72 year old woman is reported. Light microscopic examination of the small finger tip-sized tumor revealed solid alveolar proliferation of clear cells containing abundant glycogen. Im-munohistochemically, most of the clear tumor cells were stained for epithelial membrane antigen (EMA) and alpha lactalbumin, whereas a few eosinophilic tumor cells were positive for S 100 protein, EMA and actin. Electron microscopically, aggregates of glycogen particles, numerous empty glycogen lakes, microvilli, tight junctions and basal lamina were identified. Autopsy disclosed marked metastases to the liver, lung, adrenal, skin and lymph nodes. Primary breast cancer was confirmed by exclusion of a primary at any other site. It is suggested that although rare, GRCCC of the breast is as aggressive as usual invasive ductal carcinoma, and is associated with severe nodal and blood borne metastases, followed by death. Acta Pathol Jpn 39: 469 472, 1989.     

Human Osteogenic Sarcoma: Fine Structural Localization of Alkaline Phosphatase

The localization of alkaline phosphatase in eight osteogenic sarcomas of osteoblastic, chondroblastic, and fibroblastic type was investigated at the fine structural level using β-glycerophosphate as substrate and lead as capturing ion. Final product marking localization of alkaline phosphatase was deposited over plasma membranes and associated subplasmalemmal vesicles and vacuoles in various types of osteoblastlike, chondroblastlike, and fibroblastlike cells as well as certain multinucleated giant cells. Presence of L-homoarginine or L-tetramisole in the incubation medium, and incubation at 65°C, prevented the deposition of final product, suggesting that the enzyme studied was “bone specific.” The evidence obtained was compatible with the notion that the different cells showing presence of reaction product were functionally and histogenetically closely related and all were likely to be capable of bone production.     

Clear cell sarcoma (malignant melanoma of soft parts): fine-needle aspiration cytology of a highly pigmented tumor

Diagnosis of clear cell sarcoma by fine-needle aspiration is difficult, as melanin is usually absent. Here we describe a case of a primary clear cell carcinoma with abundant melanin, making diagnosis definitive.     

Clear cell sarcoma of soft tissue in right parapharyngeal region: report of a rare case

Clear cell sarcoma (CCS), initially named malignant melanoma of soft parts, is a rare malignant neoplasm typically involving deep soft tissue of the extremities, in close proximity to tendons and aponeuroses. Here we describe a case of clear cell sarcoma of the right parapharyngeal region in a young female aged 20 years. MRI detected a mass about 4.4 cm×3.4 cm×3.0 cm, located in the right parapharyngeal area and between the external pterygoid and the medial pterygoid. Microscopically, most of the tumor cells were epithelioid with palely eocinophilic cytoplasm arranged in sheets. Pleophorism of tumor cells were not marked. Immunohistochemical analysis shows that the tumor cells were positive for vimentin, S-100, HMB45 and MelanA, and negative for AE1/AE3, actin-sm, desmin, CD117, TFE-3, and P63. Ki67 index was about 5%.