Round Cell Tumors Of Children Research Articles

Anesthesia and Pain Management for Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy for Desmoplastic Small Round Cell Tumors in Children, Adolescents, and Young Adults

Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive sarcoma. Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) may improve survival. A retrospective review of anesthetic management and postoperative pain control strategies after CRS/HIPEC for DSRCT from 2013 to 2017 was performed. The review analyzed 10 CRS/HIPEC procedures performed for nine DSRCT patients with a median age of 19years (range 10-24years). Six of these patients were Caucasian, and seven were men. The median operative duration was 551min (range 510-725min), and the median anesthesia duration was 621min (range 480-820min). Postoperative mechanical ventilation was necessary in 5 patients for a median duration of 1day (range 0-2days). The median intraoperative intravenous fluid administration was 13ml/kg/h (range 6.3-24.4ml/kg/h), and the colloid administration was 12ml/kg (range 0.0-53.0ml/kg). The median blood loss was 15ml/kg (range 6.3-77.2ml/kg). Nine patients received intraoperative transfusion with a median red blood cell transfusion volume of 14ml/kg (range 10.1-58.5ml/kg). The median intraoperative urine output was 2ml/kg/h (range 0.09-8.40ml/kg/h), and half of the patients received intraoperative diuretics. Cisplatin was used during HIPEC for eight surgeries. Acute kidney injury was observed in two patients, one of whom required short-term dialysis. Epidural infusions were used in eight cases for a median of 4days (range 3-5days). Postoperative intravenous opioid use (morphine equivalent) was 0.67mg/kg/day (range 0.1-9.2mg/kg/day) administered for a median of 11days (range 2-35days). Cytoreduction and HIPEC for DSRCT are associated with significant perioperative fluid requirements and potentially challenging pain management. Renal protective strategies should be considered for reduction of cisplatin-associated nephrotoxicity. Further investigation for a more effective, less systemically toxic HIPEC agent is warranted.

Management of Desmoplastic Small Round-cell Tumors in Children and Young Adults

Desmoplastic small round cell tumor (DSRCT) is a rare type of sarcoma which is primarily abdominal in origin. Less than 200 cases have been reported. DSRCT presents with multifocal tumors believed to begin in the peritoneal surfaces of the abdominal cavity. Up to hundreds of tumors can be found in the abdominal cavity. Adolescent males are primarily affected. Because of the rarity of the tumor and the unusually aggressive presentation, treatment is challenging and has not been standardized. Here we summarize the clinical presentation, diagnosis and management options of this rare tumor.

4121 DNA ploidy and proliferative activity in common round cell tumors in children and their value as prognostic indicators

4121 DNA ploidy and proliferative activity in common round cell tumors in children and their value as prognostic indicators

Hepatoblastoma in a 15-Month-Old Male: Cytomorphology, Electron Microscopy, and Differential Diagnosis

The authors describe a case of hepatoblastoma in a 15-month-old male and discuss the differential diagnosis and electron microscopic features of small round cell tumors. The patient was found to have an enlarged liver and was admitted to the hospital for further investigation. Fine-needle aspiration of the liver revealed small. uniform cells with increased nuclear/cytoplasmic ratio and focal rosette formation. A diagnosis of small blue cell neoplasm favoring hepatoblastoma was made, but neuroblastoma could not be ruled out. Electron microscopic analysis performed on the liver aspirate showed features of hepatic differentiation as well as absence of neuroblastic differentiation. The diagnosis of hepatoblastoma was made. Serum alpha-fetoprotein level of 33,250 mg/L confirmed the diagnosis. Liver biopsy performed subsequently showed tumor cells arranged in nests, acini, and trabeculae with mitotic figures. Electron microscopy showed the same findings as described above. The patient underwent chemotherapy for 4 months and subsequently a partial liver resection was performed. This case illustrates the important role of electron microscopy in evaluating small round cell tumors in children.

Hepatoblastoma in a 15-Month-Old Male: Cytomorphology, Electron Microscopy, and Differential Diagnosis

The authors describe a case of hepatoblastoma in a 15-month-old male and discuss the differential diagnosis and electron microscopic features of small round cell tumors. The patient was found to have an enlarged liver and was admitted to the hospital for further investigation. Fine-needle aspiration of the liver revealed small, uniform cells with increased nuclear/cytoplasmic ratio and focal rosette formation. A diagnosis of small blue cell neoplasm favoring hepatoblastoma was made, but neuroblastoma could not be ruled out. Electron microscopic analysis performed on the liver aspirate showed features of hepatic differentiation as well as absence of neuroblastic differentiation. The diagnosis of hepatoblastoma was made. Serum alpha-fetoprotein level of 33,250 mg/L confirmed the diagnosis. Liver biopsy performed subsequently showed tumor cells arranged in nests, acini, and trabeculae with mitotic figures. Electron microscopy showed the same findings as described above. The patient underwent chemotherapy for 4 months and subsequently a partial liver resection was performed. This case illustrates the important role of electron microscopy in evaluating small round cell tumors in children.

A Multiplex Real-Time PCR Assay for the Detection of Gene Fusions Observed in Solid Tumors

Specific gene fusions observed in solid tumors are extremely useful diagnostic markers. We report the development of a method based on real-time PCR which enables the detection upon identical PCR conditions of the different fusions specifically observed in Ewing tumors (ET), alveolar rhabdomyosarcoma (ARMS), synovial sarcoma (SS), small round cell desmoplastic tumors (SRCDT), extraskeletal myxoid chondrosarcoma, malignant melanoma of soft parts, congenital fibrosarcoma, and anaplastic large cell lymphoma. A simple assay, based on multiplexing of primers and probes, is described for the routine genetic diagnosis of small round cell tumors of children. It enables the detection of the five EWS-ETS, the two PAX-FKHR, the three SYT-SSX, and the EWS-WT1 fusions of ET, ARMS, SS, and SRCDT, respectively. The sensitivity of this test is high enough to detect all fusions, including the large EWS-FLI-1 transcripts, with the equivalent of 100 tumor cells as a starting material. This multiplex fluorescent analysis of chromosome translocations (MFACT) was validated in comparison with conventional RT-PCR on a series of 79 tumors. A major advantage of this method is that it completely abolishes the manipulation of PCR-products. It, therefore, considerably lowers the risk of cross-contamination linked to carry-over of RT-PCR products. It also constitutes an important step toward the complete automation of the detection of cancer-specific gene fusions.

Genetics of Small Round Cell Tumors of Children

Genetics of Small Round Cell Tumors of Children

Detection of Terminal Deoxynucleotidyl Transferase (TdT) in Nonhematopoietic Small Round Cell Tumors of Children

Detection of Terminal Deoxynucleotidyl Transferase (TdT) in Nonhematopoietic Small Round Cell Tumors of Children

Round cell tumors of children

Round cell tumors of children

Detection of Terminal Deoxynucleotidyl Transferase (TdT) in Nonhematopoietic Small Round Cell Tumors of Children

Detection of Terminal Deoxynucleotidyl Transferase (TdT) in Nonhematopoietic Small Round Cell Tumors of Children

Diagnostic Relevance of Clonal Cytogenetic Aberrations in Malignant Soft-Tissue Tumors

Malignant soft-tissue tumors often present substantial diagnostic challenges. Chromosome aberrations that might be diagnostic have been identified in some types of soft-tissue tumors, but the overall frequency and diagnostic relevance of these aberrations have not been established. We attempted to determine the karyotypes of a series of 62 consecutive, unselected malignant spindle-cell or small round-cell soft-tissue tumors (from 46 adults and 16 children) after direct harvesting of cells or short-term culture. All tumors were examined independently by immunohistochemical staining in addition to routine light-microscopical evaluation, and all but two tumors were examined by electron microscopy. Metaphases were obtained from 61 of the 62 tumors, and clonal chromosome aberrations were identified in 55 (89 percent). In the six tumors that yielded metaphases but lacked apparent clonal aberrations, the normal metaphases were found to originate from non-neoplastic stromal elements within the tumor specimens. Thus, all tumors in which karyotyping was successful contained clonal chromosome aberrations. Forty of 62 tumors (65 percent) contained clonal chromosome aberrations that either suggested or confirmed a specific diagnosis; in 15 of these tumors (24 percent of all tumors), the aberrations were important in establishing the final diagnosis. Cytogenetic analyses were particularly informative about small round-cell tumors from children: 8 of 14 round-cell tumors contained diagnostically important chromosome aberrations. Using the combined approaches of light and electron microscopy, immunohistochemistry, and cytogenetics, we established an unambiguous diagnosis for 60 of 62 tumors. Cytogenetic analyses reveal clonal chromosome aberrations in virtually all malignant soft-tissue tumors. These clonal chromosome aberrations, particularly in small round-cell tumors in children, often have diagnostic relevance.

Creatine kinase subunits M and B as markers in the diagnosis of poorly differentiated rhabdomyosarcomas in children

Antibodies against the M and B subunits of creatine kinase were assessed for their usefulness in the diagnosis of poorly differentiated rhabdomyosarcoma. Routinely processed formaldehyde-fixed tissue and the avidin-biotin-peroxidase complex technique were used. The majority of the poorly differentiated and all of the moderately and well-differentiated rhabdomyosarcomas studied showed immunostaining for the M subunit. The rhabdomyoblastic component of malignant "triton" tumors was also positive. Staining, although weak compared with that of the rhabdomyosarcomas, was also observed in a few leiomyosarcomas, hemangioendotheliosarcomas, malignant fibrous histiocytomas, and ganglioneuroblastomas. On the other hand, staining for the B subunit was seen in many types of soft tissue tumors, including rhabdomyosarcoma, Ewing's sarcoma, and (ganglio)neuroblastoma. The results indicate that creatine kinase subunit M is a useful marker for distinguishing poorly differentiated rhabdomyosarcoma from other types of small round cell tumors in children, such as neuroblastoma, Ewing's sarcoma, and malignant lymphoma.

Neuron-specific enolase in the diagnosis of neuroblastoma and other small, round-cell tumors in children

Immunocytochemical staining for neuron-specific enolase (NSE) was performed in 44 round-cell tumors from children by the improved immunoglobulin-enzyme bridge method with antiserum against NSE. The tumors studied consisted of 15 neuroblastomas showing various degrees of histologic differentiation, 13 Ewing's sarcomas, ten soft tissue sarcomas of diverse origin, and six lymphomas of bone and soft tissues. Neuron-specific enolase was detected in all neuroblastomas, irrespective of the degree of histologic differentiation. None of the other round-cell tumors was positive for NSE, except one embryonal rhabdomyosarcoma that contained differentiated myoblasts. The primitive cells of this tumor were negative as well. It is concluded that immunocytochemical staining with antibodies to NSE is a practical and reliable method for distinguishing neuroblastomas from other nonneural round-cell tumors in children. This is true even for the most primitive forms of neuroblastomas, in which morphologic techniques are less reliable. Neuron-specific enolase may also be useful in delineating the neural histogenesis of other ill-defined tumors.