Pediatric Nonrhabdomyosarcoma Soft Tissue Sarcoma Research Articles

Clinical features and outcomes of young patients with low-grade non-rhabdomyosarcoma soft tissue sarcomas treated with a risk-based strategy: A report from Children's Oncology Group study ARST0332.

In retrospective analyses, the Pediatric Oncology Group [POG) and the Federation National des Centres de Lutte Contre le Cancer (FNCLCC) histologic grade predict outcome in pediatric non-rhabdomyosarcoma soft tissue sarcoma (NRSTS), but prospective data on grading, clinical features, and outcomes of low-grade NRSTS are limited. We analyzed patients less than 30years of age enrolled on Children's Oncology Group (COG) study ARST0332 (NCT00346164) with POG grade 1 or 2 NRSTS. Low-risk patients were treated with surgery alone. Intermediate-/high-risk patients received ifosfamide/doxorubicin and radiotherapy, with definitive resection either before or after 12weeks of chemoradiotherapy. Estimated 5-year event-free and overall survival were 90% and 100% low risk (n=80), 55% and 78% intermediate risk (n=15), and 25% and 25% high risk (n=4). In low-risk patients, only local recurrence was seen in 10%; none with margins greater than 1mm recurred locally. Sixteen of 17 intermediate-/high-risk patients who completed neoadjuvant chemoradiotherapy underwent gross total tumor resection, 80% with negative margins. Intermediate-/high-risk group events included one local and seven metastatic recurrences. Had the FNCLCC grading system been used to direct treatment, 29% of low-risk (surgery alone) patients would have received radiotherapy ± chemotherapy. Most low-risk patients with completely resected POG low-grade NRSTS are successfully treated with surgery alone, and surgical margins greater than 1mm may be sufficient to prevent local recurrence. Patients with intermediate- and high-risk low-grade NRSTS have outcomes similar to patients with high-grade histology, and require more effective therapies. Use of the current FNCLCC grading system may result in overtreatment of low-risk NRSTS curable with surgery alone.

Local treatment in initially unresected non-rhabdomyosarcoma soft-tissue sarcomas of children and adolescents: A retrospective single-center experience.

Pediatric non-rhabdomyosarcoma soft-tissue sarcomas (NRSTS) are a heterogeneous group of aggressive tumors. Patients with locally advanced/initially unresected disease represent a subset of patients with unsatisfactory outcome: limited data are available on the best treatment approach, in particular regarding local therapy. This retrospective analysis concerned 71 patients<21 years old with nonmetastatic, initially unresected adult-type NRSTS, treated at a referral center for pediatric sarcomas from 1990 to 2021. Patients were treated using a multimodal approach, based on the protocols adopted at the time of their diagnosis. The series included a selected group of patients with unfavorable clinical characteristics, i.e., most cases had high-grade and large tumors, arising from axial sites in 61% of cases. All patients received neoadjuvant chemotherapy, 58 (82%) had delayed surgery (R0 in 45 cases), and 50 (70%) had radiotherapy. Partial response to chemotherapy was observed in 46% of cases. With a median follow-up of 152 months (range, 18-233), 5-year event-free survival (EFS) and overall survival (OS) were 39.9% and 56.5%, respectively. Survival was significantly better for patients who responded to chemotherapy, and those who had a delayed R0 resection. Local relapse at 5 years was 7.7% for patients who did not undergo delayed surgery. Our series underscores the unsatisfactory outcome of initially unresected NRSTS patients. Improving the outcome of this patient category requires therapeutic strategies able to combine novel effective systemic therapies with a better-defined local treatment approach to offer patients the best chances to have R0 surgery.

Metastatic adult-type non-rhabdomyosarcoma soft tissue sarcomas in children and adolescents: A cohort study from the European paediatric Soft tissue sarcoma Study Group.

Limited data exist on the clinical behavior of pediatric non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) with distant metastases at onset, and a clear standard of care has not yet been defined. This cohort study reports on pediatric adult-type metastatic NRSTS enrolled in two concurrent prospective European studies, i.e., the randomized BERNIE study and the single-arm MTS 2008 study developed by the European paediatric Soft tissue sarcoma Study Group. Treatment programs were originally designed for patients with metastatic rhabdomyosarcoma, i.e., nine courses of multidrug chemotherapy (with or without bevacizumab in the BERNIE study), followed by 12 cycles of maintenance therapy, whereas radiotherapy and/or surgery (on primary tumor and/or metastases) were delayed until after seven courses of chemotherapy had been administered. The study included 61 patients <21years old treated from July 2008 to December 2016. The lung was the site of metastases in 75% of the cases. All patients received multi-agent chemotherapy, 44% had local therapy to primary tumor, and 18% had treatment of metastases. Median time to progression/relapse was 6months. A high rate of tumor progression was observed during the initial part of the chemotherapy program. With a median follow-up of 41.5months (range, 2-111months), 3-year event-free survival and overall survival were 15.4% (95% confidence interval [CI], 7.6-25.7) and 34.9% (95% CI, 22.7-47.5), respectively. There were no statistically significant differences in outcome depending on the type of treatment administered. The study confirmed the overall poor outcome for patients with metastatic NRSTS, whose treatment remains a challenge. Pediatric non-rhabdomyosarcoma soft tissue sarcomas form a heterogeneous group of rare tumors. Although recent international studies have defined the standard of care for patients with localized disease, limited data are available on the clinical behavior of patients with distant metastases. This study on 61 metastatic cases treated on two prospective European protocols confirms that the chances of survival of such patients are often dismal and a standard treatment is still lacking.

Imaging recommendations for the management of pediatric non-rhabdomyosarcoma soft tissue sarcoma (NRSTS): Consensus statement of the International Soft Tissue Sarcoma Consortium (INSTRuCT)

Imaging recommendations for the management of pediatric non-rhabdomyosarcoma soft tissue sarcoma (NRSTS): Consensus statement of the International Soft Tissue Sarcoma Consortium (INSTRuCT)

Radiotherapy for pediatric non-rhabdomyosarcoma soft tissue sarcomas: a comprehensive review.

For children with non-rhabdomyosarcoma soft tissue sarcomas, a risk-adapted treatment approach is generally used in order to minimize treatment-related morbidity and mortality in low-risk patients and maximize the benefit in high-risk patients. Our aim in this review is to discuss the prognostic factors, riskadapted treatment options and the details of radiotherapy. The publications reached by searching the keywords `pediatric soft tissue sarcoma`, `nonrhabdomyosarcoma soft tissue sarcoma (NRSTS)`, and `radiotherapy` in Pubmed database were evaluated in detail. Today, based on prospective COG-ARST0332 and EpSSG studies, a risk-adapted multimodal treatment approach has become the standard in pediatric NRSTS. According to them, adjuvant chemotherapy/ radiotherapy can be safely omitted in low-risk patients, while adjuvant chemotherapy/radiotherapy or both are recommended in intermediate and high-risk groups. Recent prospective studies for pediatric patients have reported excellent treatment outcomes with smaller radiotherapy fields and lower doses than adult series. The primary goal of surgery is maximal tumor resection with negative margins. In cases that are initially unresectable, neoadjuvant chemotherapy and radiotherapy should be considered. A risk-adapted multimodal treatment approach is the standard in pediatric NRSTS . Surgery alone is sufficient in low-risk patients, and adjuvant therapies may safely be omitted. On the contrary, in intermediateand high-risk patients, adjuvant treatments should be applied to reduce recurrence rates. In unresectable patients, the chance of surgery increases with the neoadjuvant treatment approach and thus treatment results may improve. In the future, outcome might be improved with further clarification of molecular features and targeted therapies in such patients.

Relapsing pediatric non-rhabdomyosarcoma soft tissue sarcomas: The impact of routine imaging surveillance on early detection and post-relapse survival

PurposeThe chances of patients with relapsing pediatric non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) being cured are limited. This retrospective single-institutional study examines the potential role of routine surveillance imaging for detecting recurrent tumor, and its impact on post-relapse survival. MethodsThe analysis concerned 86 patients < 21 years old with relapsing NRSTS treated from 1985 to 2020. Clinical findings, treatment modalities and survival were analyzed, comparing patients whose relapse was first suspected from symptoms (symptomatic group) with those whose relapse was detected by radiological surveillance (imaging group). ResultsTumor relapses were identified from clinical symptoms in 49 cases and on routine imaging in 37. Time to relapse was similar in the two groups. Routine imaging detected 6/32 local relapses and 31/48 distant relapses (and 79% of the cases of lung metastases). Overall survival (OS) at 5 years was 34.3% for the symptomatic group, and 24.0% for the imaging group (p-value 0.270). In patients with lung metastases at relapse, the 5-year OS was statistically better for the imaging group, that is, 25.8% versus 0% for the symptomatic group (p-value 0.044). ConclusionThis is the first study to explore the role of surveillance imaging in pediatric NRSTS. Judging from our findings, the value of routine scanning of primary sites seems limited, while radiological surveillance may help to detect lung metastases, improving survival for this patient category. The potentially negative effects of periodic radiological exams should be considered in deciding the optimal follow-up for patients off therapy.

Pediatric Non-Rhabdomyosarcoma Soft Tissue Sarcomas: Standard of Care and Treatment Recommendations from the European Paediatric Soft Tissue Sarcoma Study Group (EpSSG).

This paper describes the standard of care for patients with non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) and the therapeutic recommendations developed by the European paediatric Soft tissue sarcoma Study Group (EpSSG). NRSTS form a very mixed group of mesenchymal extraskeletal malignancies. Their rarity, heterogeneity, and aggressiveness make the management of children and adolescents with these tumors complex and challenging. The overall cure rate for patients with NRSTS is around 70%, but survival depends on several prognostic variables, such as histotype and tumor grade, extent of disease and stage, tumor size, and tumor site. While surgery remains the mainstay of treatment for most of these tumors, a multimodal therapeutic approach including radiotherapy and chemotherapy is required in many cases. The EpSSG NRSTS 2005 study was the first prospective protocol tailored specifically to NRSTS. Together with the ARST0332 study developed by the North-American Soft Tissue Sarcoma Committee of the Children’s Oncology Group (COG), the EpSSG NRSTS 2005 study currently represents the benchmark for these tumors, establishing risk-adapted standards of care. The EpSSG has developed common treatment recommendations for the large group of adult-type NRSTS (including synovial sarcoma), and specific treatment recommendations for other particular adult-type histologies (ie, alveolar soft-part sarcoma, clear cell sarcoma and dermatofibrosarcoma protuberans); other highly malignant tumors with a biology and clinical behavior differing from those of adult-type NRSTS (ie, rhabdoid tumors and desmoplastic small round cell tumor); and soft tissue tumors of intermediate malignancy (ie desmoid-type fibromatosis, inflammatory myofibroblastic tumors, and infantile fibrosarcoma). New effective drugs are needed for patients whose NRSTS carries the worst prognosis, ie, those with unresectable tumors, metastases at diagnosis, or relapsing disease. Progress in this area relies on our ability to develop international integrated prospective collaborations, both within existing pediatric oncology networks and, importantly, between the communities of specialists treating pediatric and adult sarcoma.

Adult-type non-rhabdomyosarcoma soft tissue sarcomas in pediatric age: Salvage rates and prognostic factors after relapse

PurposeThough the prognosis for patients with pediatric non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) is generally good, the chances of being cured after relapse are limited. This report describes a series of relapsing NRSTS patients treated at a referral center for pediatric sarcoma, investigating the pattern of relapse, salvage rates, and factors correlating with the final outcome. MethodsThe analysis concerned 103 patients <21 years old with relapsing adult-type NRSTS treated from 1985 to 2020. For risk-adapted stratification purposes, the patient outcome was examined using univariable and multivariable analyses based on patients’ clinical features at first diagnosis, first-line treatments, clinical findings at first relapse, and second-line treatments. ResultsThe first relapse occurred within 2–102 months (median 14 months) after patients' first diagnosis and was local in 47%, metastatic in 34%, and both in 19%. Treatment at relapse included chemotherapy in 72 patients, radiotherapy in 38, and surgery in 55. The median overall survival (OS) was 20 months. Post-relapse OS was 56.1%, 25.8%, and 19.1% at 1, 5, and 10 years, respectively. Cox's multivariable regression analysis showed that OS was significantly better for patients with local and late relapses (occurring more than 12 months after their first diagnosis) and for those achieving secondary remission. ConclusionThe outcome of patients with recurrent NRSTS is poor. The above-mentioned variables (type and time of relapse and achievement of secondary remission) were combined in a risk-adapted model to develop a tool for estimating the chance of salvage and deciding the best second-line treatment approach.

Expression of Cytochrome P450 Enzymes in Pediatric Non-Rhabdomyosarcoma Soft Tissue Sarcomas: Possible Role in Carcinogenesis and Treatment Response.

The 5-year relative survival rate estimate of treated patients with non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) is ∼50% since they generally present with tumor progression, relapse, metastasis, and/or chemoresistance. The expression of cytochrome P450 (CYP) enzymes in malignancies can affect the pharmacology of drugs commonly used in chemotherapy or confer susceptibility to development of chemical carcinogenesis; in addition, their specific tumor expression can be used as a therapeutic target. Using qPCR and Western blot assays, the expression of CYP1B1, CYP2E1, CYP3A4, and CYP3A5 were analyzed in a cohort of tumor tissue paired with non-malignant adjacent tissue of patients with NRSTS. The mRNA and protein expression of CYP1B1, CYP2E1, and CYP3A4 were significantly increased in tumor tissue. We propose that the expression of these isoforms is related to carcinogenesis and chemoresistance frequently observed in these neoplasms.

Effect of Peri-Operative Radiotherapy Boost on Local Control in Pediatric Nonrhabdomyosarcoma Soft Tissue Sarcoma (NSTS)

Effect of Peri-Operative Radiotherapy Boost on Local Control in Pediatric Nonrhabdomyosarcoma Soft Tissue Sarcoma (NSTS)

Analysis of mutations in primary and metastatic synovial sarcoma.

Synovial sarcoma is the most common pediatric non-rhabdomyosarcoma soft tissue sarcoma and accounts for about 8–10% of all soft tissue sarcoma in childhood and adolescence. The presence of a chromosomal translocation-associated SS18-SSX-fusion gene is causally linked to development of primary synovial sarcoma. Metastases occur in approximately 50–70% of synovial sarcoma cases with yet unknown mechanisms, which led to about 70–80% mortality rate in five years. To explore the possibilities to investigate metastatic mechanisms of synovial sarcoma, we carried out the first genome-wide search for potential genetic biomarkers and drivers associated with metastasis by comparative mutational profiling of 18 synovial sarcoma samples isolated from four patients carrying the primary tumors and another four patients carrying the metastatic tumors through whole exome sequencing. Selected from the candidates yielded from this effort, we examined the effect of the multiple missense mutations of ADAM17, which were identified solely in metastatic synovial sarcoma. The mutant alleles as well as the wild-type control were expressed in the mammalian cells harboring the SS18-SSX1 fusion gene. The ADAM17-P729H mutation was shown to enhance cell migration, a phenotype associated with metastasis. Therefore, like ADAM17-P729H, other mutations we identified solely in metastatic synovial sarcoma may also have the potential to serve as an entry point for unraveling the metastatic mechanisms of synovial sarcoma.

Pediatric nonrhabdomyosarcoma soft tissue sarcomas arising at visceral sites

Pediatric nonrhabdomyosarcoma soft tissue sarcomas (NRSTS) may rarely occur in visceral tissues, and little is known about their clinical history. The present study retrospectively analyzed a group of patients prospectively registered in Italian pediatric protocols conducted between 1979 and 2004. Inclusion criteria for the study were as follows: a pathological diagnosis of "adult-type NRSTS," arising at visceral sites (lung-pleurae, liver, kidney, and mesentery-bowel); age under 18 years; no previous treatment except for primary surgery; available clinical data; and written consent. Thirty cases with visceral NRSTS were collected and analyzed. Sites of origin were as follows: mesentery-bowel in 12 cases, lung-pleurae in 11, liver in 5, and kidney in 2. According to the Intergroup Rhabdomyosarcoma Study (IRS) surgical grouping system, patients were classified as follows: nine IRS group I, three group II, 12 group III, and six group IV. Patients were treated with a multimodal approach including surgery, radiotherapy, and/or chemotherapy, according to their characteristics. For the series as a whole, the 5-year event-free and overall survival rates were 33.3% and 40.0%, respectively. The IRS group (reflecting the feasibility of initial complete resection) emerged as the main prognostic factor. Survival rates also correlated with tumor size and local invasiveness, histological subtype, and tumor sites (the worst outcome was seen for tumors arising in the lung and pleurae). This study confirmed that visceral NRSTS are aggressive tumors carrying a worse prognosis than pediatric NRSTS arising in soft tissues of the extremities. Local treatment remains the main challenge for these tumors.

Non-rhabdomyosarcoma

Pediatric non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) are a heterogeneous group encompassing more than 50 different histological diagnoses arising from primitive mesenchymal tissue. Together, they comprise about half the soft tissue sarcomas diagnosed in children and young adults. Despite each histologies relative rarity, their management schema is similar among the different NRSTS histologies. Surgical management is an important component of the multimodal treatment strategy of all these tumors. Resection with negative margins, while maintaining function, plays an important role as a primary treatment of these patients as well as diminishing the risks of local and distant recurrence.

Pediatric non-rhabdomyosarcoma soft tissue sarcomas: multimodality approach results and validation of American Children's Oncology Group risk stratifications——a study of single-institutional series

Objective To investigate the clinical characteristics, survival, factors of prognosis and therapeutical strategy of pediatric non-rhabdomyosarcoma soft tissue sarcomas(NRSTS). Methods There were 34 cases of NRSTS treated in Department of Orthopaedic Oncology, Peking University Cancer Hospital during July 2007 and March 2015.The clinical and pathological data and outcome of multimodality approach were retrospectively studied, the survival analysis was performed. Results There were 21 male and 13 female cases, and the median age was 14 years, ranging from 7 to 18 years old.Mean and median size was 5.0 cm and 3.0 cm, ranged 0.5-22.0 cm.Synovial sarcoma was the most common type among the 9 sorts of histologies[29.4%(10/34 cases)], and limb was the most common site [67.6%(23/34 cases), accounting for 50.0% (17/34 cases)by lower limb]. There were 28 cases, 2 cases, 2 cases, 2 cases in Intergroup Rhabdomyosarcoma Study (IRG) groupⅠ, Ⅱ, Ⅲ, Ⅳ, respectively, and 23 cases, 7 cases, 4 cases in Children's Oncology Group (COG) low, moderate and high risk group respectively.The median follow-up was 35 months, ranging from 6 to 96 months.There were 2 cases of local recurrence, 1 case of distant recurrence, 2 cases of metastases of lymph node and distant sites, and 1 case of local and distant recurrence.The 2-year overall survival rate was 89.2%, 100.0%, 70.0% for total group, COG low risk group, COG moderate and high risk group, respectively.For 28 cases of tumors without any nodular or distant metastasis, the 2-year local recurrence-free survival and distant recurrence-free survival rate was 96.4% and 87.6% respectively.IRS group, COG risk group and size of tumor were associated with the prognosis.The groups of completely resected tumor(P=0.007), COG low risk (P=0.014)and tumors no larger than 5.0 cm in diameter(P=0.037) had more favorable overall survivals.There were 14 cases, 10 cases, 4 cases, 2 cases, 2 cases, 2 cases who had surgery alone, surgery plus chemotherapy, surgery plus chemoradiotherapy, surgery plus radiation, chemoradiotherapy alone and chemotherapy alone, respectively, as multimodality approaches.Mesna+ Adriamycin+ Ifosfamide regimen(Pirarubicin 40 mg/m2, Ifosfamide 10 g/m2) was the main(17/18 cases) choice as chemotherapy, with a total and mean cycles of chemotherapy for every case of 2-12 and 4.6 respectively. Conclusions This series of NRSTS showed favorable oncological outcomes after a multimodality approach composed of surgery, radiation and chemotherapy on MAI regimen.The COG risk stratifications were firstly adopted in domestic study of pediatric NRSTS, with prognostic efficacy being validated.Risk-based multimodality approach was also recommended. Key words: Non-rhabdomyosarcoma soft tissue sarcomas; Survival analysis; Risk stratifications; Multimodality approach

Preliminary analysis of the mutational landscape of non-rhabdomyosarcoma soft tissue sarcoma: A Children's Oncology Group study.

10510 Background: Pediatric non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) represent a histologically diverse group of more than 20 tumor subtypes, including synovial sarcoma, liposarcoma, and malignant peripheral nerve sheath tumors. Unlike children with rhabdomyosarcoma, systemic chemotherapy has failed to improve survival for those with NRSTS. Of the nearly 50% with intermediate or high-risk disease, only 50% and 15%, respectively, are expected to survive. Methods: To determine more effective, targeted therapies, we analyzed 55 archived formalin-fixed paraffin-embedded (FFPE) cases of NRSTS using Sequenom’s MassARRAY system to interrogate 296 unique “actionable” mutations (mutations that can be targeted by currently available drugs) in 33 oncogenes and tumor suppressor genes. We also analyzed 15 of the samples for copy-number variation and 74 additional “actionable” mutations in 9 cancer-associated genes using the Affymetrix OncoScan platform. Results: Using MassARRAY, we found 11 mutations in 8 NRS...

Pediatric non-rhabdomyosarcoma soft tissue sarcomas

Keywords Pediatric .Softtissuesarcoma .Non-rhabdomyosarcoma .RadiotherapyBackgroundSofttissuesarcomasencompassadiversegroupofmesenchy-mal tumors, some of which have unique biology and epide-miologywithinthepediatricagegroup.Rhabdomyosarcomasare the best characterized and studied with relatively goodsensitivity to both chemotherapy and radiotherapy. To distin-guish the other broad category of pediatric tumors derivedfrom connective tissue, the cumbersome term, non-rhabdomyosarcoma soft tissue sarcomas (NRSTS), has beencoined. Many, but not all NRSTS, share characteristics withadult-type soft tissue sarcomas (STS) leading to a wealth oftreatment principles that cross the age spectrum. But becauseof developmental toxicities especially from radiotherapy,there are considerable modifications in treatment philosophywiththemanagementofNRSTS.Unlikerhabdomyosarcoma,where well conducted randomized trials have progressivelyrefined the dose, fractionation, and volume of radiotherapy ina risk adapted manner, the evidence for optimal managementof NRSTS is still evolving. Relative to the adult managementof STS, the oncologist needs to be more mindful of the lateeffects of treatment, which include concerns for normalgrowth, fertility, and risk for secondary malignancy. But atwhat age should STS be treated more aggressively with ra-diotherapyisdifficulttoknowasNRSTSclinicaltrialsextendeligibility to young adults. This review will provide an over-view of evolving knowledge regarding NRSTS while focus-ing on the radiotherapeutic aspects.EpidemiologySoft tissue sarcomas represent 6–7 % of all pediatric cancers(defined as those occurring before age 20 years). Of these,40 % are rhabdomyosarcoma and 60 % are NRSTS [1–3].The USA’s Surveillance, Epidemiology, and End Results(SEER) program provides some of the most comprehensivecancer epidemiologic data available [4]. Of the EuropeanTumor Registries, only data published from Germany havesufficient detail to distinguish rhabdomyosarcoma fromNRSTS [5]. Overall, NRSTS comprise approximately 3–3.5 % of all pediatric cancers with 550 to 600 new casesdiagnosed each year in the USA. The incidence rate overtime has remained steady in the range of six to eight casesper million population. In Germany, the incidence rate isslightly less at five cases per million persons. Data fromSEER based on the time period 1975–1995 suggest thatNRSTS has had a propensity to dominate in older pediatricage groups (10–14 years and 15–19 years, specifically) aspart of the incidence curves for STS leading into adulthood.In fact, clinical features for soft tissue sarcomas are similarbetween children and adults, except that survival rates di-minish for patients over age 50 years [6]. See Fig. 1, depict-ing the distributionof STS subtypes, location of disease, andstaging by 10-year age intervals.The survival of patients with NRSTS is dependent on thehistologic subtype to a certain degree. Figure 2 depicts the

Myxoid Malignant Fibrous Histiocytoma Presenting as a Midline Nasal Mass

Myxoid malignant fibrous histiocytoma is a rare type of pediatric non-rhabdomyosarcoma soft-tissue sarcoma. The case of a 5-year-old girl is presented, highlighting the potential for multiple pitfalls and aberrant differential diagnoses that need to be identified for successful treatment of pediatric myxofibrosarcomas. An awareness of these tumors and a call for standardized postsurgical treatment protocols is necessary in order to successfully treat children with this disease.

Resectable Pediatric Nonrhabdomyosarcoma Soft Tissue Sarcoma: Which Patients Benefit from Adjuvant Radiation Therapy and How Much?

It remains unclear which children and adolescents with resected nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) benefit from radiation therapy, as well as the optimal dose, volume, and timing of radiotherapy when used with primary surgical resection. This paper reviews the sparse literature from clinical trials and retrospective studies of resected pediatric NRSTS to discern local recurrence rates in relationship to the use of radiation therapy.

Refractory pediatric nonrhabdomyosarcoma soft tissue sarcoma associated with ectopic production of beta hCG and hypercalcemia induced by PTHrP

A 3-month-old male with a mass on the right side of his back was admitted to our hospital. The tumor was a pathologically high-grade nonrhabdomyosarcoma soft tissue sarcoma (NRSTS). Treatment included subtotal tumor resection followed by chemotherapy. Elevation of serum beta hCG and hypercalcemia with detection of PTHrP was associated with tumor progression. The tumor was refractory to multiagent chemotherapy, and the patient died of the disease at 22 months of age. This case is novel in demonstrating a beta hCG secreting refractory NRSTS.

Clinical Outcomes of Pediatric Soft-tissue Sarcoma in the Extremities

Soft-tissue sarcomas are rare malignancies in children and adolescents, representing only 7.4 % of cancer cases in children &lt;20 years of age. They are categorized into rhabdomyosarcomas (RMS) and non-rhabdomyosarcoma soft-tissue sarcomas (NRSTS). RMS of the extremities is associated with a poorer prognosis than RMS overall. Important negative prognostic factors are higher stage and clinical group, positive regional nodes, larger size, and greater tumor invasiveness. Overall survival for extremity RMS is around 70 % at five years, with significant variability based on clinical group. NRSTS is a heterogeneous group with frequent location in the extremities. Data are more limited in pediatric NRSTS, but general negative prognostic factors are extent of disease, tumor size, quality of resection, and grade. Survival ranges from 90 % for low-grade tumors to 15 % for patients presenting with metastatic disease. Further efforts to improve the outcome for extremity RMS and to research pediatric NRSTS in general are essential for future studies.