Treatment outcomes in 23 thoracic primitive neuroectodermal tumours: a retrospective study

Sirivella, Srikrishna; Gielchinsky, Isaac

doi:10.1093/icvts/ivt161

Cited by 12 publications

(4 citation statements)

References 15 publications

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“…The median age ranged from 1 (19) to 36 (25) years. A male preponderance was described in 14 studies (8,(12)(13)(14)(15)(16)(17)(20)(21)(22)(23)(24)(25)(26). Nevertheless, there were a comparable 43 males and 46 females in the present study.…”

Section: Discussioncontrasting

confidence: 52%

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Peripheral primitive neuroectodermal tumors: A retrospective analysis of 89�cases and literature review (Review)

et al. 2019

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Peripheral primitive neuroectodermal tumors (pPNETs) are rare, small cell carcinomas with a poor prognosis. The aim of the present study was to describe therapeutic approaches, outcomes and probable prognostic factors. The clinical features, treatments, and outcomes of 89 consecutive patients with pPNET treated at the Peking Union Medical College Hospital from 1999 to 2018 were retrospectively reviewed. A total of 43 males and 46 females were included in the study, with a median age of 25 years (range, 5-73 years). The predominantly affected regions were the abdomen and pelvis, followed by the thoracopulmonary region. The mean primary tumor size was 12.6 cm (range, 1-30 cm). A total of 16 patients (18%) initially presented with metastasis. A total of 46 patients received combined therapy, 35 received mono-therapy, and 8 underwent only biopsy with no further treatment. The period of observation ranged from 1-232 months. The median overall survival (OS) time was 15 months [95% confidence interval (CI), 9-21 months], with 3-and 5-year OS rates of 32 and 25%, respectively. Large tumor size [adjusted hazard ratio (aHR)=3.65; 95% CI, 2.07-6.42; P<0.001), metastasis at initial presentation (aHR=4.34, 95% CI, 2.23-8.42; P<0.001), and combined modality treatment (aHR=0.16, 95% CI, 0.06-0.39; P<0.001) were significantly associated with OS. The prognosis of pPNET is, overall, poor. Large tumor size and metastasis at initial presentation are associated with poorer outcomes. This highly malignant tumor requires an aggressive combination of radical resection, chemotherapy and radiotherapy, when indicated.

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Section: Discussioncontrasting

confidence: 52%

“…Our review of the literature revealed 17 pPNET report series. The clinical features, treatments and outcomes of the reported patients, as well as the 89 in the present study, are summarized in Table III (8,(11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22)(23)(24)(25)(26).…”

Section: Discussionmentioning

confidence: 94%

Peripheral primitive neuroectodermal tumors: A retrospective analysis of 89�cases and literature review (Review)

et al. 2019

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“…1,2 According to the 2013 WHO classification of tumours of soft tissue and bone, both Ewing's sarcoma (ES) and PNET have a similar gene-phenotype, which can strongly express CD99 and exhibit characteristic chromosomal translocation t (11;22) (q24;q12). 3 Hence, they should be viewed as the same tumours, and these tumours have similar clinical appearances, immunohistochemical features, cellular molecular characteristics and prognoses, differing from each other only in their degree of neural differentiation.…”

Section: Introductionmentioning

confidence: 99%

CT and clinical findings of peripheral primitive neuroectodermal tumour in children

Xiao

Bao

Tan

et al. 2016

BJR

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“…The absence of clinical guidelines that establish standardized management contributes to an unfavorable prognosis and a limited survival rate (24) In our patient's case, initial tissue biopsy was followed by neoadjuvant chemotherapy before surgical resection. This approach is supported by Sirivella et al 25), whose report suggests that the implementation of neoadjuvant chemotherapy led to an improvement in the 10-year survival free of disease indicator, observed in up to 84% of patients without metastases. Surgical resection is essential, and neoadjuvant chemotherapy leads to negative margins in 71% of cases, compared to 37% when surgical resection is the initial approach (26).…”

Section: Marinău Et Almentioning

confidence: 78%

Difficulties in the management of an Askin tumor in a pediatric patient with cystic fibrosis: case report and literature review

Marinău,

Csep,

Sava

et al. 2023

Front. Pediatr.

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Treating Ewing's Sarcoma of the thorax (Askin's tumor) with antineoplastic therapy in a malnourished cystic fibrosis patient colonized with Pseudomonas aeruginosa and Staphylococcus aureus may carry a significant potential for complications. We present the case of a known cystic fibrosis patient, diagnosed with Askin's tumor 5 years ago. Despite facing severe neutropenia, exacerbations of cystic fibrosis with Pseudomonas aeruginosa infections, and challenges in maintaining adequate caloric intake during the oncological treatment, the patient's outcome has been favorable. Chemotherapy doses had to be adjusted, and continuous antibiotic treatment was introduced throughout the course of therapy to reduce the frequency and intensity of exacerbations. Approximately 5 years after the cancer diagnosis, with no signs of relapse, the patient was started on CFTR (Cystic fibrosis transmembrane conductance regulator) modulator treatment. This intervention has successfully corrected the weight deficit. The coincidence of Ewing's sarcoma of the chest wall and cystic fibrosis in a single patient is 2.857 × 10−5% and to the best of our knowledge, this scenario has not been documented before.

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Treatment outcomes in 23 thoracic primitive neuroectodermal tumours: a retrospective study

Abstract: The primitive neuroectodermal tumours are aggressive neoplasms with poor prognosis. Early diagnosis and total surgical excision of localized tumours with neoadjuvant or adjuvant chemotherapy and radiation improved disease-free survival.

Cited by 12 publications

References 15 publications

Peripheral primitive neuroectodermal tumors: A retrospective analysis of 89�cases and literature review (Review)

Peripheral primitive neuroectodermal tumors: A retrospective analysis of 89�cases and literature review (Review)

CT and clinical findings of peripheral primitive neuroectodermal tumour in children

Difficulties in the management of an Askin tumor in a pediatric patient with cystic fibrosis: case report and literature review

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