Neuroectodermal Tumors, Primitive

神经外胚层肿瘤,原始
  • 文章类型: Journal Article
    Variation in the management of PNETs exist due to the limited high-level evidence to guide clinical practice. The aim of this work is to generate consensus guidelines with a Delphi process for managing PNETs.
    A panel of experts reviewed the surgical literature and scored a set of clinical case statements using a web-based survey to identify areas of agreement and disagreement. Results of the survey were discussed after each round of review. This cycle was repeated until no further likelihood of reaching consensus existed.
    Twenty-two case statements related to surgical indications, preoperative biopsy, extent of resection, type of surgery, and tumor location were scored. Using a pre-defined definition of consensus, the panel achieved consensus on the following: i) resection is not recommended for <1 cm lesions; ii) resection is recommended for lesions greater than 2 cm; iii) lymph node dissection is recommended for radiographically-suspicious nodes with splenectomy for distal lesions; iv) tumor enucleation and central pancreatectomy are acceptable when technically feasible. No consensus was reached regarding issues of preoperative biopsy or 1-2 cm tumors.
    Using a structured, validated system for identifying consensus, an expert panel identified areas of agreement regarding critical management decisions for patients with PNET. Issues without consensus warrant additional clinical investigation.
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  • 文章类型: Consensus Development Conference
    Management of asymptomatic small well-differentiated (panNET) <2 cm remains controversial. A consensus conference was held on this topic. The impact of attending the conference and participating in the audience response survey on surgeon\'s clinical approach to pancreatic neuroendocrine tumors was assessed.
    Audience members were surveyed using a smartphone real-time response system at the beginning and end of the conference.
    The majority of 75 attendees underwent fellowship training, and 30% had >10 years experience as attending surgeons. Previously published consensus statements on the topic were considered insufficient to guide surgical practice by 82% of attendees, and over 96% desired additional data. After review of the data, consensus statements, and decision-making process, a significant number of participants changed their opinions regarding indications for tissue biopsy (p = 0.001), size thresholds for excision (p = 0.002), and regional lymph node dissection (p = 0.002) independent of whether a consensus was reached by the content-expert panel.
    This represented the first Delphi process consensus on the topic, and the survey confirmed the topic as well-chosen and timely. Attendees changed opinions on management of panNET regardless of whether formal consensus was reached. Therefore, statements of consensus combined with presentation of literature and live discussion served to impact attendees\' approach to this disease.
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  • 文章类型: Consensus Development Conference
    CNS non-cerebellar primitive neuroectodermal tumors (PNET) represent <5% of all childhood brain tumors. Despite similar therapies, these patients have had significantly worse outcomes compared to patients with medulloblastoma. Although these tumors have historically been considered analogous to medulloblastoma, there is growing evidence that they are biologically distinct. Since these tumors are relatively rare, there are few large series in the literature. Here we present the international experience treating these patients with a variety of therapies, including marrow ablative chemotherapy followed by autologous hematopoietic cell rescue.
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