Primary Clear Cell Sarcoma of the Tongue

2013 ◽  
Vol 137 (11) ◽  
pp. 1680-1683 ◽  
Author(s):  
Stefan Kraft ◽  
Cristina R. Antonescu ◽  
Andrew E Rosenberg ◽  
Daniel G. Deschler ◽  
G. Petur Nielsen
Keyword(s):  
Soft Tissues ◽  
Clear Cell ◽  
S100 Protein ◽  
Neck Region ◽  
Clear Cell Sarcoma ◽  
Giant Cells ◽  
Cell Sarcoma ◽  
Node Metastases ◽  
Type Giant

Clear cell sarcoma shares features with melanoma, but frequently shows EWSR1 rearrangements. It is an aggressive tumor typically occurring in the soft tissues of the extremities, with a gastrointestinal variant with less consistent melanocytic differentiation. It is extremely rare in the head and neck region, with no reported cases in the oral cavity. We report a case of an 82-year-old woman with a clear cell sarcoma arising in the tongue, with cervical lymph node metastases. Histologically, the tumor showed some features of gastrointestinal clear cell sarcoma. No osteoclast-type giant cells were present. The tumor cells were positive for S100 protein and negative for other melanocytic markers. Fluorescence in situ hybridization showed rearrangements of EWSR1 and ATF1. This case expands the spectrum of clear cell sarcoma with a gastrointestinal-like variant in a novel site, emphasizing the need to consider it as a differential diagnosis to melanoma in mucosal sites.

scholarly journals Clear cell sarcoma of the nuchal region with metastasis in stomach

2011 ◽  
Vol 19 (3-4) ◽  
pp. 76-78
Author(s):  
Dragana Tegeltija ◽  
Aleksandra Lovrenski ◽  
Milana Panjkovic ◽  
Slavica Knezevic-Usaj ◽  
Zivka Eri ◽  
...  
Keyword(s):  
Malignant Melanoma ◽  
Soft Tissues ◽  
Clear Cell ◽  
Neck Region ◽  
Surgical Excision ◽  
Clear Cell Sarcoma ◽  
Lower Limbs ◽  
Medical Attention ◽  
Cell Sarcoma ◽  
Complete Surgical Excision

Clear cell sarcoma/malignant melanoma of soft parts is a rare malignant tumor that originates from the neural crest. It is most common in young men in the lower limbs, grows slowly in the form of deep localized nodes around the tendons, fascia, and aponeurosis. Prognosis is poor, local recurrences and metastases are common. We present a case of a 53-year-old patient who sought medical attention due to the presence of a tumefaction in the nuchal neck region, followed by pain, heightened sensitivity, and numbness in his right hand. After excision, histological examination, and application of immunohistochemical and histochemical methods, malignant melanoma of soft tissues was diagnosed. Fourteen months after the excision of the neck tumor, a metastatic stomach disease was diagnosed. Larger tumors with necrosis, expressed pleomorphisam, and increased mitotic activity give metastases before local recurrence. Diagnosis is set using immunohistochemical methods after surgical excision of the tumor and the prognosis of the disease depends on the size of tumor and complete surgical excision.

Recurring Translocation (10;17) and Deletion (14q) in Clear Cell Sarcoma of the Kidney

2007 ◽  
Vol 131 (3) ◽  
pp. 446-451 ◽  
Author(s):  
Noel A. Brownlee ◽  
L. Allen Perkins ◽  
Will Stewart ◽  
Beth Jackle ◽  
Mark J. Pettenati ◽  
...  
Keyword(s):  
In Situ Hybridization ◽  
Tumor Suppressor ◽  
Fluorescence In Situ Hybridization ◽  
Clear Cell ◽  
Clear Cell Sarcoma ◽  
Protein Accumulation ◽  
Cytogenetic Abnormalities ◽  
Cell Sarcoma ◽  
Chromosome 17P13

Abstract Context.—Clear cell sarcoma of the kidney (CCSK) is a prognostically unfavorable renal neoplasm of childhood. Previous cytogenetic studies of CCSK have reported balanced translocations t(10;17)(q22;p13) and t(10;17)(q11; p12). Although the tumor suppressor gene p53 is located at the chromosome 17p13 breakpoint, p53 abnormalities are rarely present in these tumors. Objective.—To identify cytogenetic abnormalities in CCSK and correlate these findings with other clinicopathologic parameters. Design.—A retrospective review of CCSK patients from 1990 to 2005 was conducted at our medical center. We performed clinical and histologic review, p53 immunohistochemical and classic cytogenetics (or ploidy analysis), and p53 fluorescence in situ hybridization analyses. Results.—Five male patients (age range, 6 months to 4 years) were identified with cytogenetic abnormalities. Of 3 cytogenetically informative cases, one revealed a clonal balanced translocation t(10;17)(q22;p13) and an interstitial deletion of chromosome 14, del(14)(q24.1q31.1), and the other 2 patients had normal karyotypes. Fluorescence in situ hybridization for p53 in the t(10;17) case revealed no deletion. Immunohistochemical evaluation of p53 demonstrated lack of nuclear protein accumulation in all cases. Conclusions.—Together with the published literature, our results indicate that translocation (10;17) and interstitial deletions of chromosome 14q are recurring cytogenetic lesions in CCSK. To date, 3 cases of CCSK or “sarcomatoid Wilms tumors” have been reported to exhibit t(10;17). One previously reported case of CCSK contained deletion 14q. Results of p53 immunohistochemistry and/or p53 fluorescence in situ hybridization in this report suggest lack of mutations or deletions of this tumor suppressor in these CCSK cases. The t(10;17) breakpoint and deletion of chromosome 14q24 suggest that other genes are involved in tumor pathogenesis.

Clear Cell Sarcoma of Tendons and Aponeuroses: A Review

2007 ◽  
Vol 131 (1) ◽  
pp. 152-156 ◽  
Author(s):  
Daniel C. Dim ◽  
Linda D. Cooley ◽  
Roberto N. Miranda
Keyword(s):  
Clear Cell ◽  
Current Knowledge ◽  
S100 Protein ◽  
Clear Cell Sarcoma ◽  
Prognostic Indicators ◽  
Gene Transcript ◽  
Poor Overall Survival ◽  
Regional Lymph Nodes ◽  
Cell Sarcoma ◽  
Eosinophilic Cytoplasm

Abstract Clear cell sarcoma of tendons and aponeuroses, also referred to as malignant melanoma of soft parts, is a rare malignancy derived from neural crest cells. It usually presents in the distal lower extremities of young adults, frequently attached to tendons or aponeuroses. It behaves like a high-grade soft tissue sarcoma and is associated with poor overall survival. Magnetic resonance imaging studies of the lesion reveal T1 hypointensity, T2 hyperintensity, and gadolinium uptake. Grossly, the tumor is usually circumscribed with a histologic pattern of uniform polygonal to fusiform cells with clear to pale eosinophilic cytoplasm divided into variably sized clusters by fibrous septa. Immunohistochemical studies in most cases show that the neoplastic cells are positive with HMB-45 and react with antibody against S100 protein. Most cases show a reciprocal cytogenetic translocation t(12;22)(q13;q12) that creates a unique chimeric fusion EWSR1/ATF1 gene transcript. Metastasis occurs mainly to regional lymph nodes and lungs. Poor prognostic indicators include a tumor size equal to or more than 5 cm, presence of metastasis, and necrosis. The mainstay of treatment is wide excision of the tumor. The use of sentinel lymph node biopsy may become an important procedure in detecting occult regional metastasis and guiding the extent of surgery. The beneficial effects of adjuvant chemotherapy and radiotherapy have not been fully evaluated. This article provides a short overview of the current knowledge of clear cell sarcoma of tendons and aponeuroses.

Epithelial-Type and Neural-Type Cadherin Expression in Malignant Noncarcinomatous Neoplasms With Epithelioid Features That Involve the Soft Tissues

2002 ◽  
Vol 126 (4) ◽  
pp. 425-431 ◽  
Author(s):  
William B. Laskin ◽  
Markku Miettinen
Keyword(s):  
Malignant Melanoma ◽  
Synovial Sarcoma ◽  
Soft Tissues ◽  
Clear Cell ◽  
Epithelioid Sarcoma ◽  
Clear Cell Sarcoma ◽  
Extraskeletal Myxoid Chondrosarcoma ◽  
Cell Sarcoma ◽  
Myxoid Chondrosarcoma ◽  
Poorly Differentiated

Abstract Context.—Transmembrane adhesion molecules, epithelial-type cadherin (ECAD) and neural-type cadherin (NCAD), help in regulating transformations between epithelial and mesenchymal cells in the developing embryo and in maintaining the epithelioid phenotype. Consequently, the presence of epithelioid cells in certain malignant noncarcinomatous neoplasms raises speculation that the expression of ECAD and NCAD in these neoplasms may have diagnostic significance. Objective.—To investigate the utility of ECAD and NCAD immunoexpression in distinguishing malignant (noncarcinomatous) neoplasms with epithelioid features that involve the soft tissues. Design.—Membranous immunoreactivity of anti-ECAD and anti-NCAD was evaluated on archived cases selected from the files of the Armed Forces Institute of Pathology. Results.—Epithelial-type cadherin was found in biphasic synovial sarcoma (35 of 35 cases), malignant melanoma (13/21), monophasic fibrous synovial sarcoma (13/26), clear cell sarcoma (4/9), poorly differentiated synovial sarcoma (3/13), diffuse mesothelioma (4/20), malignant epithelioid peripheral nerve sheath tumor (1/6), and epithelioid sarcoma (5/62). Neural-type cadherin was observed in chordoma (11/11), biphasic synovial sarcoma (30/35), diffuse mesothelioma (14/20), malignant melanoma (14/25), epithelioid sarcoma (24/63), epithelioid angiosarcoma (1/4), poorly differentiated synovial sarcoma (2/13), clear cell sarcoma (1/10), and monophasic fibrous synovial sarcoma (1/26). Eighteen cases of primary cutaneous squamous cell carcinomas all tested positive for ECAD, whereas NCAD was focally observed in 5 cases. No expression of either molecule was observed in cases of epithelioid hemangioendothelioma (n = 9), alveolar soft part sarcoma (n = 8), and extraskeletal myxoid chondrosarcoma (n = 7). Conclusions.—Epithelial-type and neural-type cadherins are found in a variety of noncarcinomatous neoplasms with epithelioid features that involve the soft tissues and can be utilized, in association with other immunomarkers, in distinguishing chordoma (100% NCAD) from extraskeletal myxoid chondrosarcoma and conventional chondrosarcoma of bone (0% NCAD), squamous cell carcinoma (100% ECAD) from epithelioid sarcoma (8% ECAD), and biphasic synovial sarcoma (100% ECAD) from diffuse mesothelioma (20% ECAD).

CLEAR CELL SARCOMA OF SOFT TISSUES IN CHILDREN AND YOUNG ADULTS: The St. Jude Children's Research Hospital Experience

1999 ◽  
Vol 16 (6) ◽  
pp. 539-544 ◽  
Author(s):  
Sudha Parasuraman ◽  
Bhaskar N. Rao ◽  
Sara Bodner ◽  
Alvida Cain ◽  
Charles B. Pratt ◽  
...  
Keyword(s):  
Young Adults ◽  
Soft Tissues ◽  
Clear Cell ◽  
Clear Cell Sarcoma ◽  
Cell Sarcoma ◽  
Research Hospital ◽  
Hospital Experience ◽  
Children And Young Adults

Clear Cell Sarcoma-like Tumor with Osteoclast-like Giant Cells in the Small Bowel: Further Evidence for a New Tumor Entity

2005 ◽  
Vol 13 (4) ◽  
pp. 313-318 ◽  
Author(s):  
Nicolaus Friedrichs ◽  
Maria Adele Testi ◽  
Luisa Moiraghi ◽  
Piergiorgio Modena ◽  
Ellen Paggen ◽  
...  
Keyword(s):  
Small Bowel ◽  
Clear Cell ◽  
Clear Cell Sarcoma ◽  
Giant Cells ◽  
Cell Sarcoma ◽  
Tumor Entity

Primary Clear Cell Sarcoma of the Ileum: A Case Report With Next-Generation Sequencing Analysis

2021 ◽  
pp. 106689692098531
Author(s):  
Peipei Zhu ◽  
Tingting Zhang ◽  
Ke Bi ◽  
Yunjin Wu ◽  
Xue Chen ◽  
...  
Keyword(s):  
Next Generation Sequencing ◽  
Clear Cell ◽  
S100 Protein ◽  
Clear Cell Sarcoma ◽  
Sequencing Analysis ◽  
Next Generation ◽  
Cell Sarcoma ◽  
Next Generation Sequencing Analysis ◽  
Large Round ◽  
Generation Sequencing

As the concept of clear cell sarcoma–like tumor or malignant gastrointestinal neuroectodermal tumor (CCS-LT/MGNET) has been widely accepted, primary CCS of the gastrointestinal tract (CCS-GI) is becoming a rare entity. In this article, we describe a case of primary CCS-GI that occurred in the ileum of a 65-year-old male to further illustrate its rare occurrence. Similar to CCS of soft tissue (CCS-ST), the tumor was composed of spindled to epithelioid cells displaying fascicular, nested, or pseudopapillary arrangement. The tumor cells had large round to ovoid nuclei with vesicular chromatin and prominent nucleoli, containing eosinophilic to pale cytoplasm. In contrast to CCS-LT/MGNET, immunohistochemical study also showed variable positivity of HMB45, melan A, and MiTF besides the strong and diffuse staining of S100 protein and SOX10. Fluorescence in situ hybridization (FISH) using fusion probes identified EWSR1 and ATF1 genes rearrangement. Next-generation sequencing (NGS) analysis further revealed EWSR1 exons9/8- ATF1 exon4 and ATF1 exon3- EWSR1 exon11 fusion genes. CCS-GI and CCS-LT/MGNET possibly represent 2 related entities of the same spectrum, which differentiate along 2 different pathways.

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