Humans ; Sarcoma, Clear Cell/pathology* ; Gastrointestinal Neoplasms/pathology* ; Esophagus ; Neuroectodermal Tumors/pathology*

Humans ; Sarcoma, Clear Cell/pathology* ; Melanoma/pathology* ; Skin Neoplasms/pathology* ; Diagnostic Errors ; Soft Tissue Neoplasms ; Melanoma, Cutaneous Malignant

Cytodiagnosis ; Diagnostic Errors ; Humans ; Sarcoma, Clear Cell/pathology* ; Soft Tissue Neoplasms/pathology*

Pediatric renal tumors represent a diverse group, which include Wilms' tumor (WT), renal cell carcinoma (RCC), clear cell sarcoma of the kidney (CCSK), congenital mesoblastic nephroma, malignant rhabdoid tumor of the kidney (MRTK) and primitive neuroectodermal tumor. WT (85%) and RCC (8%) are the most prevalent types. WT predominates among the 1- to 10-year age group, but RCC exceeds WT in children over age 10 years. Pediatric renal tumors are genetically, histologically and clinically heterogeneous. The overall survival for children with localized WT is currently more than 90%, whereas poorer survival rates are observed for anaplastic WT, metastatic WT, metastatic CCSK, MRTK, metastatic RCC and relapsed WT. Therefore risk-stratified treatment is important to minimize treatment morbidity while preserving survival. This review focuses on distinct characteristics of each tumor type and optimal stratified treatment.
Carcinoma, Renal Cell ; Child ; Humans ; Kidney ; Nephroma, Mesoblastic ; Neuroectodermal Tumors, Primitive ; Rhabdoid Tumor ; Sarcoma, Clear Cell ; Survival Rate ; Wilms Tumor

BACKGROUND: Soft tissue clear cell sarcoma is a rare tumor which originates from neural crest cells. Due to its rarity and lack of established treatment, the prognosis of clear cell sarcoma is poor. Here, we reviewed the clinical data and outcome of patients diagnosed with soft tissue clear cell sarcoma in our institution.METHODS: A retrospective study was conducted on pediatric patients who were treated for pathologically confirmed soft tissue clear cell sarcoma at the Seoul National University Hospital, between January 2000 and July 2017.RESULTS: Six patients (3 boys and 3 girls) were diagnosed with soft tissue clear cell sarcoma at a median age of 14 years 4 months (range 11 years 7 months - 19 years 3 months). The median size of the tumor was 5.6 cm (range, 0.6 cm to 7.9 cm). The most frequent symptom was pain (67%), and the most common primary site was the lower limb (67%). Three patients (50%) presented with metastases at diagnosis. Four patients underwent chemotherapy with various therapeutic combinations. Four patients received surgical resection. Only one patient received local radiotherapy. One patient died of primary refractory disease, three patients relapsed, while the remaining two survive event-free.CONCLUSION: Soft tissue clear cell sarcoma is a rare and highly aggressive tumor, for which there is no established treatment. All surviving patients received surgery, indicating that surgery is a key treatment modality. Further genetic studies of soft tissue clear cell sarcoma are needed to find a better treatment strategy.
Diagnosis ; Drug Therapy ; Humans ; Korea ; Lower Extremity ; Neoplasm Metastasis ; Neural Crest ; Pediatrics ; Prognosis ; Radiotherapy ; Retrospective Studies ; Sarcoma, Clear Cell ; Seoul ; Treatment Outcome

Clear cell sarcoma is rare and difficult to diagnose. Herein, we present a case of clear cell sarcoma in the dorsum of the wrist with MRI findings, including diffusion-weighted imaging, and histopathologic correlation, which was initially diagnosed as giant cell tumor of tendon sheath.
Diffusion Magnetic Resonance Imaging ; Giant Cell Tumors ; Magnetic Resonance Imaging* ; Sarcoma, Clear Cell* ; Tendons ; Wrist*

Clear-cell sarcoma (CCS) is a rare soft tissue sarcoma that usually develops in the lower extremities of young adults. CCS of the gastrointestinal tract is extremely rare. We report here the first case of CCS of the stomach in Korea. A 28-year-old female developed a large tumor of the stomach that was initially considered Ewing sarcoma, based on the results of a needle biopsy. She had paraneoplastic syndrome; fever, anemia, and hyperglycemia. Follow-up abdominal computed tomography after four-cycles of chemotherapy with vincristine-doxorubicin-cyclophosphamide or ifosfamide-etoposide showed no tumor shrinkage and revealed a fistula between the tumor and the gastric lumen. The infected tumor prompted debulking surgery, which resulted in improvement of her symptoms. The pathologic findings were consistent with CCS, and fluorescence in situ hybridization for EWS gene rearrangement was positive, confirming the diagnosis. This case highlights the importance of clinical suspicion for CCS in chemotherapy-refractory Ewing sarcoma.
Adult ; Anemia ; Biopsy, Needle ; Diagnosis ; Drug Therapy ; Female ; Fever ; Fistula ; Fluorescence ; Follow-Up Studies ; Gastrointestinal Tract ; Gene Rearrangement ; Humans ; Hyperglycemia ; In Situ Hybridization ; Korea ; Lower Extremity ; Paraneoplastic Syndromes ; Sarcoma* ; Sarcoma, Clear Cell ; Sarcoma, Ewing ; Stomach* ; Young Adult

Chromosomal Proteins, Non-Histone ; metabolism ; DNA-Binding Proteins ; metabolism ; Diagnosis, Differential ; Follow-Up Studies ; Humans ; Infant ; Infant, Newborn ; Keratins ; metabolism ; Male ; Mucin-1 ; metabolism ; Phosphopyruvate Hydratase ; metabolism ; Rhabdoid Tumor ; metabolism ; pathology ; surgery ; Rhabdomyosarcoma ; metabolism ; pathology ; S100 Proteins ; metabolism ; SMARCB1 Protein ; Sarcoma ; metabolism ; pathology ; Sarcoma, Clear Cell ; metabolism ; pathology ; Skin Neoplasms ; metabolism ; pathology ; surgery ; Transcription Factors ; metabolism ; Vimentin ; metabolism

Adolescent ; Carcinoma, Renal Cell ; metabolism ; pathology ; Desmin ; metabolism ; Diagnosis, Differential ; Humans ; Leg ; MART-1 Antigen ; metabolism ; Male ; Melanoma ; metabolism ; pathology ; Melanoma-Specific Antigens ; metabolism ; Perivascular Epithelioid Cell Neoplasms ; metabolism ; pathology ; surgery ; Sarcoma, Clear Cell ; metabolism ; pathology ; Skin Neoplasms ; metabolism ; pathology ; surgery

Chondroblastoma ; pathology ; Chondroma ; pathology ; Chondrosarcoma ; metabolism ; pathology ; surgery ; Cricoid Cartilage ; Diagnosis, Differential ; Humans ; Laryngeal Neoplasms ; metabolism ; pathology ; surgery ; Laryngectomy ; Lymph Node Excision ; Male ; Middle Aged ; Osteoblastoma ; pathology ; Osteosarcoma ; pathology ; S100 Proteins ; metabolism ; Sarcoma, Clear Cell ; metabolism ; pathology ; surgery ; Vimentin ; metabolism