Early versus delayed administration of granulocyte-colony stimulating factor following chemotherapy in pediatric patients with Ewing sarcoma.

BACKGROUND: The optimal timing of initiating granulocyte-colony stimulating factor following chemotherapy in pediatric patients has not been clearly defined. This study aimed to compare the administration of granulocyte-colony stimulating factor on day 1 versus day 3 postchemotherapy in pediatric patients with Ewing sarcoma. METHOD: A retrospective study of pediatric patients with Ewing sarcoma who received granulocyte-colony stimulating factor following chemotherapy between January 2016 and September 2018 at a comprehensive cancer center. The institution's chemotherapy protocol for Ewing sarcoma was modified in April 2017 to include granulocyte-colony stimulating factor initiation on day 3 instead of day 1 post-chemotherapy. Febrile neutropenia requiring hospitalization, duration of hospital stay, and chemotherapy delay were compared for patients before and after the protocol change. RESULTS: Over the study period, 250 cycles were evaluated with day 1 granulocyte-colony stimulating factor and 221 cycles with day 3 granulocyte-colony stimulating factor. There were no differences between the day 1 and day 3 groups in the number of cycles associated with Febrile neutropenia requiring hospitalization (34 vs. 19, p = 0.086), and the length of Febrile neutropenia-related hospitalization (mean 4 ± 2.1 vs. 4.6 ± 1.8, p = 0.123). However, delay in chemotherapy due to neutropenia was reported in significantly more cycles in the day 1 group, compared to the day 3 group (37 vs. 16, p = 0.01). CONCLUSIONS: Febrile neutropenia resulting in hospital admission and the length of hospital stay was not different between pediatric patients with Ewing sarcoma who received granulocyte-colony stimulating factor on day 1 or day 3 post-chemotherapy. Chemotherapy delay due to neutropenia was higher in patients who received granulocyte-colony stimulating factor on day 1. Larger studies are required to fully determine the impact of delayed initiation of granulocyte-colony stimulating factor.

Photothermal theragnosis synergistic therapy based on bimetal sulphide nanocrystals rather than nanocomposites.

A new generation of photothermal theranostic agents is developed based on Cu3BiS3 nanocrystals. A computed tomography imaging response and photothermal effect, as well as near-infrared fluorescence emission, can be simultaneously achieved through Cu3BiS3 nanocrystals rather than frequently used nanocomposites. These results provide some insight into the synergistic effect from bimetal sulphide semiconductor compounds for photothermal theragnosis therapy.

La familia de tumores del sarcoma de Ewing / No disponible

La familia de tumores del sarcoma de Ewing (SE) es un conjunto de neoplasias primarias del hueso y/o tejidos de soporte, causado por la proliferación de células madre mesenquimales bloqueadas en su maduración fetal y que adquieren posteriormente las características de células malignas. Este conjunto de enfermedades se caracterizan por una firma molecular que las define: la fusión del gen EWS del cromosoma 22q12 con genes implicados en el control de la replicación celular, creando un gen de fusión anómalo. Los genes de fusión o sus productos se detectan mediante la PCR de RNA o RT-PCR. Virtualmente todos los enfermos con SE tienen enfermedad microscópica subclínica, por ello el tratamiento eficaz del SE es por vía sistémica. La probabilidad de supervivencia libre de enfermedad de los pacientes sin metástasis detectables clínicamente es del 70%; para los pacientes con metástasis es inferior al 30%. La quimioterapia es el tratamiento esencial para el control de la enfermedad microscópica sistémica, pero sola no puede erradicar la enfermedad en forma de grandes masas. El control local de la enfermedad visible debe realizarse mediante cirugía y/o radioterapia. El objetivo fundamental de la cirugía es obtener márgenes de resección amplios. El SE es un tumor radiosensible y dada la sinergia entre la quimioterapia y la radioterapia, los tratamientos suelen ser combinados(AU)

The Ewing family of tumors (SE) is a group of neoplasias arising from developing bone or soft tissues. SE is likely derived from the malignant proliferation of mesenchymal stem cells. This group of diseases has a unique molecular signature: the fusion of the EWS gene on chromosome 22q12 to genes involved in the control of cell proliferation, generating an abnormal fusion gene. The fusion genes and their products are detected in the laboratory by the RTPCR method. Virtually all SE patients have subclinical microscopic disease; therefore effective treatment must always include systemic chemotherapy. The event-free survival for patients without clinically detectable disease is 70% at 5 years from diagnosis; for patients with metastasis at diagnosis is less than 30%. Chemotherapy is essential for the treatment of microscopic metastatic disease, but it cannot eradicate the disease in the form of masses. The local control of the disease is made by surgery and/or radiation therapy. The main objective of surgery is to achieve tumor resection with ample margins. The SE is a radiosensitive tumor and because the synergistic effect with chemotherapy, they are used in combination. In summary: SE is a rare, complex and life-threatening group of diseases that should be managed in comprehensive cancer centers(AU)

Intraoperative microwave inactivation in-situ of malignant tumors in the scapula.

OBJECTIVE: To explore the feasibility and effect of microwave in situ inactivation of malignant primary or metastatic tumors in the scapula. METHODS: Seventeen patients (12 men, 5 women, mean age 48 years [range, 13-59 years]) with malignant primary or metastatic tumors involving the scapula were treated by microwave inactivation between June 1998 and February 2008. There were 12 malignant primary bone and 5 metastatic tumors. In 14 cases Area Sl was involved and in 3 cases both Areas S1 and S2. All 17 cases were by making a dorsal arc- or "∩-" shaped incision to expose the tumor, protecting the surrounding soft tissues with a copper grid, and then heated the tumors locally with 2450 MHz microwave to 50°C for 20 min, after which all or some of the necrotic tumor tissue was removed, preserving the support role of the scapula. RESULTS: The operation time was 60-180 min (mean 120 min) and blood loss was 300-1000 mL (mean 460 mL). No serious intraoperative or postoperative complications occurred in any patient. The patients were followed up for 3 months to 10 years (mean 4.2 years). Three patients with Ewing's sarcoma in the scapula had pulmonary, cerebral and systemic multiple metastases and died 8~24 months after surgery. Three patients with malignant fibrous histiocytoma died of pulmonary and systemic multiple metastases 10~22 months after surgery; one patient had recurrence 6 months after surgery and survived with tumor. Five patients with metastatic tumor in the scapula died of non-scapular metastatic tumor 6~14 months after surgery. The other five patients with primary malignant bone tumors had no recurrence or metastasis during follow-up. Three cases had restricted extension of the shoulder joint with unrestricted protraction and retroflexion after surgery. CONCLUSION: In situ microwave inactivation features simple surgery, reliable effects and patient acceptability, making it an ideal surgical method for malignant tumors in the scapula.

[Changes of 3-tesla 31P-MR spectroscopy of bone and soft tissue tumors].

OBJECTIVE: To study the characteristic changes of 31P-MR spectroscopy of bone and soft tissue tumors. METHODS: 41 patients were examined by phosphorus surface coil of 3 tesla MR machine, including 18 benign tumor foci and 28 malignant foci, and adjacent normal muscles. The areas under the peaks of various metabolites in the spectra were measured, including phosphomonoester (PME), inorganic phosphours (Pi), phosphodiester (PDE), phosphocreatine (Pcr), adenosine triphosphate (ATP) gamma, alpha, beta. The ratios of the metabolites to beta-ATP, NTP and Pcr were calculated. Intracellular pH was calculated according to the chemical shift change of Pi relative to Pcr. RESULTS: The ratios of Pcr/PME and PME/NTP in benign and malignant tumor groups were significantly different from those of the normal group (P<0.05). Between benign and malignant tumor groups, the ratios of PME/beta-ATP and PME/NTP were significantly different (P<0.05). CONCLUSION: Pcr/PME and PME/NTP are potential diagnostic indexes of bone and soft tissue tumors. PME/beta-ATP and PME/NTP are potential indexes of differential diagnosis of bone and soft tissue tumors.

Ewing's sarcoma as second malignant neoplasm after retinoblastoma: a case report.

OBJECTIVES: To report a case of a child with the hereditary form of unilateral retinoblastoma (RB), who developed Ewing's sarcoma of the right fibula 3 years after the enucleation of the right eye. CASE PRESENTATION AND INTERVENTION: The child was diagnosed as a case of RB of the right eye at the age of 9 months. He was fully investigated and found to have locally advanced RB with bone marrow involvement (Reese-Ellsworth stage IVA). Enucleation was recommended to the family, but they refused. The patient received chemotherapy and diode laser thermotherapy in Kuwait and the UK. He had a local relapse after 11 months and subsequently underwent enucleation of the right eye. After 3 years, he was investigated for a small swelling in his right lower leg. After extensive investigations, it was reported as Ewing's sarcoma. He was treated with chemotherapy, surgery (complete excision of the fibula) and high-dose chemotherapy followed by autologous stem cell transplantation. The child is now nearly 2 years after completing the treatment and is disease free. CONCLUSIONS: This case confirms the increased risk of a second malignant neoplasm (SMN) in children with hereditary RB. These children need a very close follow-up for the early diagnosis of SMNs or even subsequent malignancies.

A case report of an uncommon cause of cauda equina symptoms.

OBJECTIVE: This case report discusses a patient who presented with right-sided buttock pain of apparently uncomplicated mechanical origin that was eventually diagnosed as a primary Ewing sarcoma/primitive neuroectodermal tumor of the sacrum. CLINICAL FEATURES: A 32-year-old male full-time student presented for care with right-sided buttock pain. INTERVENTION AND OUTCOME: After examination, the patient was referred to his general practitioner for urgent magnetic resonance imaging, the report revealed no explanation for the presenting symptoms. After further imaging and biopsy, an eventual diagnosis of Ewing sarcoma/primitive neuroectodermal tumor was reached. The patient died 12 months later. CONCLUSION: This case highlights a nondiscal cause for cauda equina symptoms. It emphasizes potential diagnostic complexities that may present due to preconceptions based upon the probability of symptoms being related to a specific disease process.

The first report of extraosseous Ewing's sarcoma in the rectovaginal septum.

AIMS AND BACKGROUND: To report an extremely rare case of Ewing's sarcoma located in the rectovaginal septum. Ewing's sarcoma is a highly malignant neoplasm of bone, which usually occurs during childhood. Common extraosseous localizations of Ewing's sarcoma include the trunk, extremities, uterus, cervix and vagina. METHODS: A 45-year-old woman presented to us with a six-month history of pain in the lower abdomen during intercourse. Pelvic examination was performed and a palpable mass was found. The mass had a size of 9 x 6 cm, a soft tissue consistency, was partially movable and the patient felt the pain during palpation. Examination of the inguinal lymph nodes revealed no signs of inguinal adenopathy. The results of laboratory tests, rectoscopy, chest X-rays, barium enema and bone scan were normal. Computed tomography (CT) showed an inhomogeneous expansive mass in the rectovaginal septum measuring 8.7 x 6.1 cm, without any signs of rectum or bladder invasion. The vascular structures of the pelvis were normal. At laparotomy the process was judged inoperable and only biopsy of the tumor mass was carried out. Histology showed a neoplasm with small, round to oval cells with scarce cytoplasm. Immunohistology with the monoclonal antibody CD99 (MIC-2 gene product, Ewing's sarcoma marker, clone 12E7, DAKO A/S, Glostrup, Denmark) revealed an extraosseous Ewing's sarcoma. The patient was treated with chemotherapy followed by whole-pelvis external beam radiation and intracavitary brachytherapy. RESULTS: A residual mass measuring 3.5 x 2.5 cm was visible on a control CT scan 18 months after treatment; however, the patient was feeling well and refused surgery to remove the residual mass. CONCLUSIONS: To our knowledge this is the first reported case of extraosseous Ewing's sarcoma in the rectovaginal septum.

Osteosarcoma and Ewing's sarcoma after neoadjuvant chemotherapy: value of dynamic MR imaging in detecting viable tumor before surgery.

OBJECTIVE: This study analyzed the value of dynamic contrast-enhanced and subtraction MR images in detecting residual viable tumor before surgery, with emphasis on timing of enhancement, in patients with high-grade osteosarcoma and Ewing's sarcoma after neoadjuvant chemotherapy. SUBJECTS AND METHODS: Twenty-one patients with proved osteosarcoma or Ewing's sarcoma were treated with neoadjuvant chemotherapy followed by surgery. After IV administration of gadopentetate dimeglumine, dynamic enhancement patterns on preoperative MR images were compared with corresponding areas on histologic sections of the resected specimens. On dynamic subtraction images obtained with high temporal resolution (1.5-3 sec), the interval between arrival of the bolus of contrast agent in an artery and start of tumoral enhancement was used to distinguish residual viable tumor. Early enhancing foci (interval artery-tumor < 6 sec) and late or nonenhancing areas seen on MR images were correlated with the histopathologic findings in these areas of the resected specimens. RESULTS: Early and progressively enhancing structures seen on MR images corresponded to feeding arteries, physeal vessels, or residual viable tumor at specific preferential sites on corresponding histologic sections of the resected specimens. Tumor foci as small as 3-5 mm2 could be detected on dynamic MR images. Remnant viable tumor was often located subperiosteally and at the margins of the tumor. Occasionally, active periosteal reaction without presence of viable tumor contributed to early enhancement. Late and gradually enhancing or nonenhancing areas corresponded histopathologically to regions of chemotherapy-induced necrosis, mucomyxoid degeneration, or fibrosis. Alternatively, late or nonenhancing areas were associated with reactive changes such as edema, hemorrhage, or osteomyelitis or with tumor-related extracellular matrices such as abundant osteoid or chondroid. Viable tumor areas with scarce formation of matrix on microscopy, such as small cell osteosarcoma areas or Ewing's sarcoma, showed early enhancement with rapid washout of contrast agent on the dynamic MR images. CONCLUSION: Fast dynamic contrast-enhanced sequences are essential for identifying residual tumor before surgery. A short time interval of less than 6 sec between arterial enhancement and tumoral enhancement strongly correlates with presence of viable tumor. Both therapy-related alterations of tissue and tumor-related matrices must be considered when late or lack of enhancement is noted on dynamic MR images.