Advances in the Diagnosis and Management of Neonatal Sarcomas

Rashid, Tooba; Noyd, David H.; Iranzad, Natasha; Davis, Jefferson; Deel, Michael D.

doi:10.1016/j.clp.2020.11.007

Cited by 3 publications

(4 citation statements)

References 140 publications

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“…Diagnosis prior to 18 months and absence of MYCN amplification are favorable prognostic factors; cutaneous metastasis is not prognostic [18,25] ] discussed the similarity in dermoscopic characteristics of cutaneous RMS metastasis to other cutaneous malignancies. The highly vascular nature of the tumor can lead to misdiagnosis as vascular tumor or malformation [26]. Diagnosis requires tumor biopsy paired with molecular tissue analysis, imaging, and sentinel lymph node biopsy for staging workup.…”

Section: Cutaneous Neuroblastomamentioning

confidence: 99%

Section: Sarcomasmentioning

confidence: 99%

“…RMS, the most common soft tissue sarcoma in childhood, accounts for 3% of all childhood cancers [16,26,27]. Other nonrhabdomyosarcoma soft tissue sarcomas (NRSTS) include extrarenal rhabdoid tumors [26], Ewing, and synovial sarcomas [27]. This section will focus on RMS as it is far more prevalent and while uncommon, can metastasize to, directly invade, or primarily involve the skin [1,28,29].…”

Section: Sarcomasmentioning

confidence: 99%

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Pearls and updates: cutaneous signs of systemic malignancy

Paquette

Cotter

Huang

2022

Current Opinion in Pediatrics

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Purpose of reviewChildren with systemic malignancies can present with concomitant dermatological manifestations. Cutaneous findings can occur before, during or after diagnosis. Recognizing these features can aid in diagnosis, inform prognosis, and help determine appropriate treatment. Following a review of the literature published over the past two years, we provide an update on cutaneous signs of pediatric systemic malignancies, concentrating on; leukemia cutis, lymphoma cutis, neuroblastoma, sarcomas, Langerhans cell histiocytosis and paraneoplastic syndromes. Recent findingsAuthors highlight the persistently heterogeneous features of cutaneous manifestations of systemic malignancy. Findings are often nonspecific, and a definitive diagnosis requires skin biopsy with immunophenotyping. Several studies describe dermoscopy features, demonstrating this as a useful tool in clinical evaluation. Genetic mutations underlying the pathogenesis of disease continue to be elucidated. Further, advances in medical treatment led to improved prognosis in many systemic malignancies, with early and aggressive treatment heralding better outcomes. SummaryComprehensive cutaneous evaluation alongside thorough clinical history and review of systems remains of paramount importance as dermatological manifestations of systemic malignancy are notoriously variable with a shared feature of often appearing benign but persisting despite usual treatment. Urgent referral to dermatology is recommended when suspicion for any cutaneous presentation of malignancy arises.

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Section: Cutaneous Neuroblastomamentioning

confidence: 99%

Section: Sarcomasmentioning

confidence: 99%

Section: Sarcomasmentioning

confidence: 99%

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Pearls and updates: cutaneous signs of systemic malignancy

Paquette

Cotter

Huang

2022

Current Opinion in Pediatrics

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“…However, with the increased awareness of soft tissue tumors and fibrosarcoma, the diagnosis of adult fibrosarcoma has been tightly defined so that its incidence may be as low as 1% of adult soft tissue sarcomas [ 2 , 3 ]. Unlike the highly malignant adult fibrosarcoma, congenital or infantile fibrosarcoma is intermediate in 2020 WHO classification, with few metastases and a better prognosis [ 4 – 6 ]. Since neurotrophic tropomyosin receptor kinase (NTRK) fusion often occurs in infantile fibrosarcoma, the application of NTRK inhibitors such as larotrectinib is highly efficacious in patients [ 7 – 9 ].…”

Section: Introductionmentioning

confidence: 99%

Score for the Overall Survival Probability Scores of Fibrosarcoma Patients after Surgery: A Novel Nomogram-Based Risk Assessment System

Chen

Chi

Chen

et al. 2021

Journal of Oncology

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Background. The primary purpose of this study was to determine the risk factors affecting overall survival (OS) in patients with fibrosarcoma after surgery and to develop a prognostic nomogram in these patients. Methods. Data were collected from the Surveillance, Epidemiology, and End Results database on 439 postoperative patients with fibrosarcoma who underwent surgical resection from 2004 to 2015. Independent risk factors were identified by performing Cox regression analysis on the training set, and based on this, a prognostic nomogram was created. The accuracy of the prognostic model in terms of survival was demonstrated by the area under the curve (AUC) of the receiver operating characteristic curves. In addition, the prediction consistency and clinical value of the nomogram were validated by calibration curves and decision curve analysis. Results. All included patients were divided into a training set (n = 308) and a validation set (n = 131). Based on univariate and multivariate analyses, we determined that age, race, grade, and historic stage were independent risk factors for overall survival after surgery in patients with fibrosarcoma. The AUC of the receiver operating characteristic curves demonstrated the high predictive accuracy of the prognostic nomogram, while the decision curve analysis revealed the high clinical application of the model. The calibration curves showed good agreement between predicted and observed survival rates. Conclusion. We developed a new nomogram to estimate 1-year, 3-year, and 5-year OS based on the independent risk factors. The model has good discriminatory performance and calibration ability for predicting the prognosis of patients with fibrosarcoma after surgery.

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