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ICAR SINONASAL TUMORS

XXIV SINONASAL NEUROECTODERMAL AND NEUROENDOCRINE CARCINOMAS A Olfactory neuroblastoma Olfactory neuroblastoma, also known as esthesioneurob lastoma (ONB), is a rare malignancy of the anterior skull base. 1332 This tumor demonstrates neuroendocrine differ entiation and is believed to originate from the olfactory neuroepithelium in the upper nasal cavity. 71,1333 ONB falls on the spectrum of neuroendocrine tumors and retains neuroendocrine features including expression of neuroen docrine markers and neurosecretory vesicles. 1333,1334 No known risk factors have been identified for this tumor. ONB has previously been reported to have a bimodal age distribution. 70 However, more recent large studies have demonstrated a true unimodal distribution with a peak between the fourth and sixth decades. 1335 In this section, we performed an evidence-based update to the ICSB 2019 (Section VIII.A) regarding endoscopic skull base surgery and expand on several additional aspects of management related to ONB. 5 Impact of Hyams grade on outcomes Hyams et al. developed the only grading system based on histologic maturation and differentiation that has been shown to be of prognostic value, particularly in comple menting current staging systems. 1336–1339 Multiple studies have shown that Hyams grade allows for the identification of aggressive locoregional disease and subsequent predic tion of poor DFS and may enable stratification for adjuvant therapy. 510,1333,1340,1341 The independent prognostic utility of Hyams grading was demonstrated by Kane et al. and the ability to predict metastasis and OS was further confirmed in a recent meta-analysis by Goshtasbi et al. 1342,1343 Table XXIV.A.1 summarizes evidence surrounding impact of Hyams grade on outcomes of ONB. Impact of Hyams grade on outcomes 1

Harm

Grading may be prone to misinterpretation and requires pathologist expertise. There are no studies investigating the costs of histological grading of ONB. Preponderance of benefits over harms. High grade tumors appear to have more aggressive biological behavior (more prone to recurrence, nodal metastases) and may require more aggressive upfront treatment.

Cost

Benefits–harm assessment

Value

judgments

Policy level Recommendation. Intervention Hyams grading should be routinely assessed when sampling tissue for ONB cases, as knowledge of the grade may impact treatment strategies. 2 Staging systems Historically, the most commonly used prognosticator is the Kadish staging system. 1344 Kadish developed this staging system with the analysis of data from 17 patients and pub lished this work in 1976. Later, Morita et al. performed a retrospective analysis on 49 patients treated at the Mayo Clinic between 1951 and 1990 and proposed the modi fied Kadish staging system. 1345 In 1992, Dulguerov et al. proposed a modified version incorporating radiographic findings. 1346 While the Dulguerov system has been shown to be superior to the Kadish–Morita system in a recent individual patient data meta-analysis of publicly available data, a recent analysis of the NCDB determined that, in general, current clinical staging systems do not adequately predict survival over 10 years. 248,1347 Analyzing the largest reported cohort of over 400 ONB patients, Lechner et al. showed that the Kadish–Morita staging system appeared to be superior to the alternative Dulguerov staging sys tem and that better delineation between stage groups was observed in the former, in comparison with the substantial overlap between Dulguerov T1, T2, and T3. 15 However, in line with the analysis of the SEER database by Joshi et al., they did not observe a statistically significant difference in survival between Kadish A and B tumors. As dural infil tration/invasion was found to be a significant prognostic indicator in their cohort (in line with early findings in cran iofacial surgery and expanding on recent work), a modified staging system was devised, combining the A and B groups and separating the C group into those with and without dural infiltration, termed the Kadish-INSICA (Interna tional Network for Sinonasal Cancer Research) Staging System. 246,1348,1349 Further research to validate this system is required to confirm prognostic value (Table XXIV.A.2).

Aggregate grade of evidence

B (Level 2: three studies; Level 4: five studies)

Benefit

Understanding Hyams grade provides prognostic information that may guide adjuvant therapy and treatment of the neck.

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