Clear cell sarcoma of the kidney in a 62-year-old patient presenting with generalized pruritus

Abstract Background Clear cell sarcoma of the kidney (CCSK) is the second most common renal tumor in children following Wilms’ tumor. CCSK is extremely rare in adults, with only 25 adult cases reported in the medical literature. Case presentation We reported a 62-year-old man with a right renal mass presenting only with generalized pruritus who underwent radical right nephrectomy. With immunostaining, tumor cells were positive for expressed vimentin, neural cell adhesion molecule (NCAM, CD56), and Ki-67 and focally positive for p53, CD10 and Bcl-2. The histopathological diagnosis was CCSK. Two weeks after the operation, the generalized pruritus ended. One month after the operation, the patient started treatment with a regimen combining doxorubicin, vincristine, cyclophosphamide, and etoposide. At the 20-month follow-up visit, there was no evidence of local recurrence or metastases. Conclusions In a patient presenting with generalized pruritus, further evaluation for an underlying malignancy should be considered. It is difficult to distinguish CCSK from undifferentiated renal neoplasms. Immunohistochemistry could help to make exact histopathological diagnoses. The BCL-6 corepressor (BCOR) gene could play a significant role in CCSK tumorigenesis and be a good marker for CCSK diagnosis. Surgery with combination chemotherapy and radiation therapy could be used to treat CCSK in older patients.

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Is clear cell sarcoma a malignant form of psammomatous melanotic schwannoma?

Neurosurgical FOCUS ◽

10.3171/foc.2006.21.6.3 ◽

2006 ◽

Vol 21 (6) ◽

pp. 1-4 ◽

Cited By ~ 3

Author(s):

Ahmet Şengöz ◽

Erol Taşdemiroğlu ◽

Halit Togay

Keyword(s):

Malignant Melanoma ◽

Nerve Root ◽

Clear Cell ◽

Clear Cell Sarcoma ◽

Histopathological Diagnosis ◽

Total Resection ◽

Cell Sarcoma ◽

Melanotic Schwannoma ◽

Psammomatous Melanotic Schwannoma

✓The authors present a case of clear cell sarcoma (CCS) in which the tumor originated in the S-1 nerve root and had been previously diagnosed as psammomatous melanotic schwannoma (PMS). This is the third case of a spinal nerve root origin for CCS reported in the English-language literature. The similar histogenesis of CCS and malignant melanoma supports the hypothesis that biological agents or immunotherapy are potentially important areas of investigation. The patient underwent S1–3 laminectomy and gross-total resection of the mass lesion. The border of the resection was extended 1 cm distal to the tumor margin. The postoperative period was uneventful. The new histopathological diagnosis was CCS (malignant melanoma of soft tissue). Despite total resection, the patient returned with disseminated disease at the 18-month follow-up visit. His follow-up magnetic resonance image of the lumbar spine revealed sacral L5–S3 involvement of the vertebral bodies along with disseminated cauda equina seeding. A CCS originating from peripheral nerves is quite rare. The histopathological and immunohistochemical appearance of CCSs resembles those of PMSs. Surgery should be the first choice of treatment.

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An immunohistochemical study comparing clear cell sarcoma of the kidney and wilms' tumor

Pathology ◽

10.3109/00313029309084780 ◽

1993 ◽

Vol 25 (2) ◽

pp. 106-109 ◽

Cited By ~ 17

Author(s):

Lai-Meng Lool ◽

Phaik-Leng Cheah

Keyword(s):

Clear Cell ◽

Immunohistochemical Study ◽

Wilms Tumor ◽

Clear Cell Sarcoma ◽

Cell Sarcoma

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A long-term survivor of clear cell sarcoma-like tumor of the gastrointestinal tract with liver metastasis: a case report

Surgical Case Reports ◽

10.1186/s40792-020-01028-z ◽

2020 ◽

Vol 6 (1) ◽

Author(s):

Takuhisa Okada ◽

Yasumitsu Hirano ◽

Shintaro Ishikawa ◽

Hiroka Kondo ◽

Toshimasa Ishii ◽

...

Keyword(s):

Small Intestine ◽

Lymph Node ◽

Gastrointestinal Tract ◽

Liver Metastasis ◽

Surgical Resection ◽

Clear Cell ◽

Clear Cell Sarcoma ◽

Cell Sarcoma

Abstract Background Clear cell sarcoma-like tumor of the gastrointestinal tract (CCSLTGT) is extremely rare. It is a mesenchymal neoplasm that usually forms in the small intestine of adolescents and young adults, is prone to local recurrence and metastasis, and has a high mortality rate. We report a patient with CCSLTGT with lymph node- and liver metastases, who continues to survive 6 years after initial surgical resection. Case presentation A 38-year-old woman presented with lightheadedness. Laboratory analysis revealed anemia (hemoglobin, 6.7 g/dL), and enhanced computed tomography (CT) demonstrated a mass in the small intestine, about 6 cm in diameter, with swelling of 2 regional lymph nodes. Double-balloon small intestine endoscopic examination revealed a tumor accompanied by an ulcer; the biopsy findings suggested a primary cancer of the small intestine. She was admitted, and we then performed a laparotomy for partial resection of the small intestine with lymph node dissection. Pathologic examination revealed CCSLTGT with regional lymph node metastases. About 3 years later, follow-up CT revealed a single liver metastasis. Consequently, she underwent a laparoscopic partial liver resection. Histopathologic examination confirmed that the liver metastasis was consistent with CCSLTGT. It has now been 3 years without a recurrence. Conclusion Repeated radical surgical resection with close follow-up may be the only way to achieve long-term survival in patients with CCLSTGT.

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Tubular Entrapment May Be Inconspicuous in Clear Cell Sarcoma of the Kidney in Early Infancy, Compared to Childhood: An In-Depth Case Comparison

American Journal of Clinical Pathology ◽

10.1093/ajcp/aqz113.056 ◽

2019 ◽

Vol 152 (Supplement_1) ◽

pp. S59-S60

Author(s):

Krutika Patel ◽

Sara Avalos Hernandez ◽

S Shawn Liu ◽

J Elliot Carter ◽

Elizabeth Manci

Keyword(s):

Growth Pattern ◽

Clear Cell ◽

Wilms Tumor ◽

Clear Cell Sarcoma ◽

Study Groups ◽

Growth Patterns ◽

Molecular Techniques ◽

Early Infancy ◽

Renal Tumors ◽

Cell Sarcoma

Abstract Introduction Clear cell sarcoma of kidney (CCSK) is a rare malignancy accounting for <0.5% of all primary renal tumors, commonly diagnosed between 2 and 4 years of age and rarely occurring in early infancy. The challenging differentiation between CCSK and blastemal Wilms tumor is important because of the distinct clinical pattern of CCSK to recur and metastasize to bone and brain. The aim of this study is to discern subtle features that could assist pathologists in diagnosing CCSK in infancy. Method In-depth comparison of clinical, histological, and immunohistochemical findings in a case of CCSK diagnosed at 5 months of age with two cases of CCSK diagnosed at 2 and 3 years of age. Results Both groups were male, and each presented with an abdominal mass. Grossly, a single, firm, well-demarcated tumor, morphologically comprising monotonous small primitive round-to-polygonal/spindle cells, was seen in both groups. The major differences between the study groups were growth patterns and stromal reactions. In infancy, the growth pattern was diffusely uniform sheets of malignant cells with no entrapment of tubules and inconspicuous stromal changes. However, in childhood cases, the growth pattern included well-defined tubular entrapment, as well as focal microcyst formation, myxomatous stroma, palisading bodies, and anaplastic and/or rhabdoid histology. In both study groups, the immunohistochemistry showed strong immunoreactivity with cyclin D1 and nonspecific positivity for vimentin, CD99, and BAF47. Conclusion CCSK has notoriously diverse histological heterogeneity and mimics other pediatric renal tumors, making diagnosis treacherous, and commonly erroneous as Wilms tumor with unfavorable histology. Despite the advent of immunohistochemical and molecular techniques, a thorough morphologic analysis remains key in accurately diagnosing CCSK at any age, especially in early infancy. This small in-depth comparison of CCSK by age groups suggests that tubular entrapment and stromal changes may be less conspicuous in CCSK in early infancy than at older ages.

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Hyperthermic Antiblastic Perfusion in the Treatment of Cancer of the Extremities

Tumori Journal ◽

10.1177/030089168507100406 ◽

1985 ◽

Vol 71 (4) ◽

pp. 355-359 ◽

Cited By ~ 23

Author(s):

Maurizio Vaglini ◽

Salvatore Andreola ◽

Angelo Attili ◽

Filiberto Belli ◽

Raffaele Marolda ◽

...

Keyword(s):

Overall Survival ◽

Squamous Cell Carcinoma ◽

Clear Cell ◽

Remission Rate ◽

Osteogenic Sarcoma ◽

Major Complication ◽

Clear Cell Sarcoma ◽

Clinical Results ◽

Cell Sarcoma

From February 1982 to December 1983, 42 patients affected by neoplasms of the limbs were treated at the Istituto Nazionale Tumori of Milan by hyperthermic antiblastic perfusion in extracorporeal circulation at the temperature of 40-41 °C for 1 h. Thirty-two were affected by melanoma, 4 by osteogenic sarcoma, 2 by squamous-cell carcinoma, 1 by liposarcoma, 1 by hemangiopericytoma, 1 by clear-cell sarcoma and 1 by Kaposis's sarcoma. As regards the immediate response, a complete plus partial remission rate of 88 % without any major complication was obtained. The follow-up period is too short for any considerations about overall survival. However, because of these good clinical results we consider this method able to locally control the evolution of neoplasms of the extremities, allowing in many cases a limb salvage.

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Functional and Gene Expression Analysis of the p53 Signaling Pathway in Clear Cell Sarcoma of the Kidney and Congenital Mesoblastic Nephroma

Pediatric and Developmental Pathology ◽

10.1007/s10024-001-0215-y ◽

2002 ◽

Vol 5 (3) ◽

pp. 257-268 ◽

Cited By ~ 3

Author(s):

Noel A. Brownlee ◽

Debra J. Hazen-Martin ◽

A. Julian Garvin ◽

Gian G. Re

Keyword(s):

Tumor Tissue ◽

Clear Cell ◽

Wilms Tumor ◽

Clear Cell Sarcoma ◽

Renal Tumors ◽

Mesoblastic Nephroma ◽

Primary Tumors ◽

Cell Sarcoma ◽

Congenital Mesoblastic Nephroma ◽

Mdr 1

Mutation of p53 has been implicated in progression of classical Wilms tumor (WT) into the anaplastic variant (AWT), drug resistance, and poor prognosis. Because of prognostic similarities, clear cell sarcoma of the kidney (CCSK) has been classified with AWT and other aggressive pediatric renal tumors, apart from congenital mesoblastic nephroma (CMN), which is instead a relatively benign tumor of neonates. Initially, CCSK and CMN were assumed to be ontologically related, but the role of p53 in the pathogenesis of either disease has not been sufficiently evaluated as in AWT. We examined the status of p53 in CMN and CCSK in comparison to AWT by immunohistochemistry and mRNA analysis of p53, the downstream effector p21 WAF-1/CIP-1 ( p21), the multidrug resistance gene MDR-1, a putative target of p53, and the p53-antagonist Mdm-2. Surprisingly, strong p53 nuclear immunoreactivity was found in cultures from two CMN specimens, but not in frozen or fixed tumor tissue from five other CMN specimens, nor in cell lines or tumor tissue from CCSK. Sequence analysis excluded p53 mutations. The size of the p53 mRNA in CMN and CCSK primary tumors excluded gross deletions or rearrangements. Low levels of Mdm-2 mRNA in CCSK and CMN primary tumors and cultures did not support a role for Mdm-2. Absence of MDR-1 mRNA excluded MDR-1 in the drug-resistant phenotype of CCSK. Cisplatin-induced p21 transactivation assays and G1 cell cycle arrest analyses showed that p21 transactivation and G1 arrest occurred in both CCSK and CMN cultures, demonstrating integrity of the p53 signal transduction pathway. Absence of p53 functional abnormalities excluded relationships between CCSK and CMN as in AWT, supporting the association of cellular CMN with congenital fibrosarcomas as more recently proposed.

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Clear cell sarcoma of the kidney: Accuracy of imaging methods for detecting bone metastases. Report from the national Wilms' tumor study

Medical and Pediatric Oncology ◽

10.1002/mpo.2950180311 ◽

1990 ◽

Vol 18 (3) ◽

pp. 225-227 ◽

Cited By ~ 13

Author(s):

James H. Feusner ◽

J. Bruce Beckwith ◽

Giulio J. D'Angio

Keyword(s):

Bone Metastases ◽

Clear Cell ◽

Wilms Tumor ◽

Clear Cell Sarcoma ◽

Cell Sarcoma ◽

Imaging Methods ◽

Tumor Study

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Clear cell sarcoma of the soft parts arising in the rectus abdominis in a child – aspiration cytology of a rare case

CytoJournal ◽

10.1186/1742-6413-4-15 ◽

2007 ◽

Vol 4 ◽

pp. 15 ◽

Cited By ~ 3

Author(s):

Paari Murugan ◽

Debdatta Basu ◽

Surendra Kumar ◽

Sadasivan Jagadish

Keyword(s):

Soft Tissue ◽

Abdominal Wall ◽

Clear Cell ◽

Histopathological Examination ◽

Clear Cell Sarcoma ◽

Female Child ◽

Needle Aspiration ◽

Case Presentation ◽

Cell Sarcoma ◽

High Cellularity

Background Clear cell sarcoma of soft parts is most commonly found associated with the tendons and aponeuroses of distal extremities in young adults with a peak incidence in the third decade. Location in the abdominal wall and in a child is very rare. Case presentation A nine-year-old female child presented with a swelling in the anterior abdominal wall in the suprapubic region. Fine needle aspiration revealed predominantly discrete cells with loose clustering at places. The cytoplasm was abundant, finely granular, and eosinophilic with some cells exhibiting clear vacuolated zones. No pigment was seen. The nuclei were rounded and eccentrically placed with a striking single eosinophilic macro nucleolus present in all the cells. Taking into consideration, the history, age of the patient, location of the tumor and absence of melanin pigment, a diagnosis of soft tissue sarcoma was made, the differential including Clear cell sarcoma. This was subsequently confirmed on histopathological examination and immunohistochemistry Conclusion The atypical presentation of the case made the cytological diagnosis rather challenging. Clear cell sarcoma should be considered when cytology of a soft-tissue tumor shows uncharacteristically high cellularity and relatively uniform cells with macronucleoli.

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