Pediatric solid tumors have many similarity among different tumors. These tumors present small round cell types, and cause frequent diagnostic problems in pediatric pathology. An important advance in the investigation of these small round cell tumors has been the identification of consistent chromosomal translocations associated with several types of tumor. Eighteen patients with soft tissue sarcoma were available for review. Seventeen cell lines were also included in this study. The RNA from the specimens were analyzed by reverse transcriptase-polymerase chain reaction (RT-PCR). PAX3-FKHR fusion was present in four of five alveolar rhabdomyosarcoma and PAX7-FKHR fusion was detected in one of five alveolar rhabdomyosarcoma. None of the specimens expressed more than one chimeric transcript. EWS-FLI1 or EWS-ERG fusions were detected in all seven Ewings' sarcoma. No specimens showed EWS-WT1 fusion. These results corresponded well to the histopathologic diagnosis. There were no differences in the histologic appearances of tumors with the more frequent PAX3-FKHR or EWS-FLI1 fusions compared with those containing the variant PAX7-FKHR or EWS-ERG fusions. RT-PCR assay for chimeric transcript is an useful tool for a rapid and objective diagnosis of pediatric solid tumors. Through these tools, we can approach genetically to the differential diagnosis of undifferentiated small round tumors.
Cell Line ; Diagnosis* ; Diagnosis, Differential ; Humans ; Lymphoma ; Pathology ; Rhabdomyosarcoma ; Rhabdomyosarcoma, Alveolar ; RNA ; Sarcoma ; Sarcoma, Ewing ; Translocation, Genetic ; Wilms Tumor

A case of abdominal Burkitt's lymphoma diagnosed through aspiration cytology is described. This 9-year-old boy presented with abdominal pain and distention for three months accompanied by fever and night sweat during the last month. An abdominal sonography and CT scan showed hepatosplenomegaly and an intrahepatic mass with celiac lymph node enlargement, ascites, and pleural fluid. A peripheral blood smear showed a few blast cells. Aspiration of the abdominal mass revealed very cellular aspirates consisting of diffusely scattered small monotonous round cells. The cells had little cytoplasm, along with round nuclei that showed clear-cut nuclear membrane, coarse chromatin pattern, and multiple small prominent nucleoli. Differential diagnoses considered were small round cell sarcomas such as malignant lymphoma, neuroblastoma, Ewing's sarcoma, and rhabdomyosarcoma. Of these, malignant lymphoma of the small noncleaved cell type was most consistent with the results of several studies including immunohistochemical staining, peripheral blood smear, and bone marrow biopsy. The cells were positive for leukocyte common antigen (LCA) and showed finely vacuolated basophilic cytoplasm in both the peripheral blood smear and bone marrow biopsy, characteristic of Burkitt's lymphoma cells.
Biopsy, Needle ; Burkitt Lymphoma/*diagnosis/pathology/radionuclide imaging ; Child ; Diagnosis, Differential ; Humans ; Male ; Neuroblastoma/*diagnosis/pathology ; Rhabdomyosarcoma/*diagnosis/pathology ; Sarcoma, Ewing/*diagnosis/pathology ; Tomography, X-Ray Computed

Although intracranial dural metastasis of Ewing's sarcoma is a very rare finding, its imaging characteristics are similar to those of its primary form in the central nervous system. Thus, this tumor must be considered in the differential diagnosis of extra-axial dural masses.
Adult ; Dura Mater/*pathology ; Female ; Humans ; Magnetic Resonance Imaging ; Sarcoma, Ewing's/diagnosis/*pathology/surgery ; Skull Neoplasms/diagnosis/*secondary/surgery ; Spinal Neoplasms/diagnosis/pathology/surgery ; Tomography, X-Ray Computed

No abstract available.
Antigens, CD/metabolism ; Cell Adhesion Molecules/metabolism ; Female ; Humans ; Immunohistochemistry ; Peritoneum/metabolism/pathology ; Sarcoma, Ewing/*diagnosis/pathology/radiography ; Tomography, X-Ray Computed ; Young Adult

OBJECTIVETo study the clinicopathological features and diagnostic criteria of Ewing's sarcoma/primitive neuroectodermal tumor (Ewing's sarcoma/PNET) in oral and maxillofacial region.

METHODSThere were 15 patients with Ewing's sarcoma/PNET in the last 35 years at our hospital, aged 1-49 years and mean 14.5 years. The most common manifestation was swelling of the affected region. The cases were analyzed and histological and immunohistochemical studies were also conducted to examine CD99 (12E7), Vimentin, NSE, S-100, Syn, CD45 (LCA), desmin.

RESULTS(1) The most common histological pattern of Ewing's sarcoma/PNET was a lobular arrangement of uniform, small, hyperchromatic cells in a fibrous background. Some of these tumors were rich in cytoplasmic glycogen. (2) Of the 7 cases, IHC was positive for CD99 (12E7) and Vimentin and negative for lymphoid (CD45), muscle (desmin) markers. S-100 was positive in 4 cases and NSE negative in 3, Syn positive in 1. NSE, S-100, Syn were all negative in 2 cases.

CONCLUSIONSEwing's sarcoma/PNET is more common in teenagers and young people. Immunohistochemistry is essential to distinguish Ewing's sarcoma/PNET from other small round cell tumors. Immunohistochemistry is useful in the diagnosis.

Adolescent ; Adult ; Child ; Child, Preschool ; Diagnosis, Differential ; Facial Neoplasms ; diagnosis ; pathology ; Female ; Humans ; Infant ; Jaw Neoplasms ; diagnosis ; pathology ; Male ; Middle Aged ; Neuroectodermal Tumors, Primitive, Peripheral ; diagnosis ; pathology ; Sarcoma, Ewing ; diagnosis ; pathology ; Young Adult

Adamantinoma ; diagnostic imaging ; metabolism ; pathology ; surgery ; Adult ; Diagnosis, Differential ; Female ; Femur ; Follow-Up Studies ; Humans ; Humerus ; Ilium ; Keratins ; metabolism ; Male ; Middle Aged ; Mucin-1 ; metabolism ; Retrospective Studies ; Sarcoma, Ewing ; pathology ; Sarcoma, Synovial ; pathology ; Tibia ; Tomography, X-Ray Computed ; Young Adult

Arthritis, Juvenile ; diagnosis ; pathology ; Biomarkers, Tumor ; blood ; Biopsy ; Bone Neoplasms ; diagnosis ; pathology ; Child, Preschool ; Diagnostic Errors ; Female ; Hip Joint ; diagnostic imaging ; pathology ; Humans ; Ilium ; diagnostic imaging ; pathology ; Magnetic Resonance Imaging ; Radiography ; Sarcoma, Ewing ; diagnosis ; pathology

Extraskeletal Ewing's sarcoma (EES) is a branch of neuroectodermal tumor (PNET), which is very rare soft tissue sarcoma. We report a case of EES/PNET arising is the lung of a 67-yr-old man. Computed tomography, bone scintigraphy, and positron emission tomography confirmed the mass to have a primary pulmonary origin. The mass showed positive reactivity in the Periodic Acid Schiff (PAS) stain and MIC-2 immunoreactivity in immunohistochemical stain. Fluorescence in situ hybridization (FISH) was performed, which revealed an EWSR1 (Ewing sarcoma breakpoint region 1) 22q12 rearrangement. The diagnosis was confirmed both pathologically and genetically. The mass lesion was resected, and the patient is currently undergoing chemotherapy.
Aged ; Calmodulin-Binding Proteins/genetics ; Chromosome Breakage ; Chromosomes, Human, Pair 22/genetics ; Diagnosis, Differential ; Humans ; Immunohistochemistry ; In Situ Hybridization, Fluorescence ; Lung Neoplasms/*diagnosis/genetics/metabolism/pathology ; Male ; Neuroectodermal Tumors, Primitive, ; RNA-Binding Proteins/genetics ; Sarcoma, Ewing's/*diagnosis/genetics/metabolism/pathology

Extraskeletal Ewing's sarcoma is rarely found in the head and neck regions. We report an unusual case of extraskeletal Ewing's Sarcoma of the parapharynx region in a 49-year-old man who presented with blindness. MRI examination showed marked enhancement of tumor thrombosis involving the superior sagittal sinus, straight sinus, transverse sinus, sigmoid sinus, and internal jugular vein. The final diagnosis was extraskeletal Ewing's sarcoma after biopsy of the internal jugular vein thrombosis by histopathological evaluation and immunohistochemical assay. In addition, the patient was diagnosed as having adenocarcinoma of the rectum by biopsy of the rectal mass. The patient was treated with systemic chemotherapy and showed improved response with durable remission. The patient's visual acuity, however, did not improve.
Antineoplastic Agents/therapeutic use ; *Blindness ; Cyclophosphamide/therapeutic use ; Doxorubicin/therapeutic use ; Head and Neck Neoplasms/diagnosis/drug therapy/*pathology ; Humans ; Male ; Middle Aged ; Sarcoma, Ewing's/diagnosis/drug therapy/*pathology ; Vincristine/therapeutic use

Extraosseous Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) is an uncommon, aggressive, and malignant tumor with a poor patient outcome. Its occurrence in the lesser sac is a rare event and to the best of our knowledge, has not been previously described. The present case was clinically and radiologically misdiagnosed as a pancreatic tumor/gastrointestinal stromal tumor. Histopathology revealed a tumor with "small round cells" that were positive for CD99, confirming the diagnosis of ES/PNET. This report highlights the importance of considering Ewing's sarcoma in the differential diagnosis of intraabdominal, extraintestinal masses.
Antigens, CD/analysis ; Biopsy ; Cell Adhesion Molecules/analysis ; *Diagnostic Errors ; Female ; Humans ; Immunohistochemistry ; Middle Aged ; Neuroectodermal Tumors, Primitive, Peripheral/*diagnosis/immunology/pathology/therapy ; Pancreatic Neoplasms/*diagnosis ; Peritoneal Neoplasms/*diagnosis/immunology/pathology/therapy ; Predictive Value of Tests ; Sarcoma, Ewing/*diagnosis/immunology/pathology/therapy ; Tomography, X-Ray Computed ; Tumor Markers, Biological/analysis