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KUANetal.

TABLE XXV.10 Evidence for the treatment of Low-grade nasopharyngeal papillary adenocarcinoma (LGNPPAc).

Clinical endpoints

Study

Year LOE Study design Study groups

Conclusions

Lai et al. 1722

Recurrence rate 1. All patients were alive without evidence of lymphatic or distant metastases in the follow-up period (range 7 to 121 months) 2. Two patients (7%, 2/28) experienced disease recurrence

2021

4

Retrospective case series

28T1N0M0 patients Endoscopic surgery ( n = 23) Preoperative RT + endoscopic surgery ( n = 3) Endoscopy surgery + postopera tiveRT ( n = 2) Five T1N0M0 patients with LGNPPAc Endoscopic surgery ( n = 3) Endoscopic surgery + postopera LGNPPAc treated with surgery, RT, or surgery + RT LGNPPAc treated with surgery, RT, or surgery + RT 22 patients with tiveRT ( n = 2) 25 patients with

Huang et al. 1721

2019 4

Retrospective case series

Recurrence rate All five patients were followed up for 6 months to 8.8 years, without recurrence or metastasis

Booth et al. 1730

DSS

1. 1-year DSS: 94.4% 2. 5-year DSS: 85.7% 3. 10-year DSS: 71%

2019 4

Database study (SEER)

Kuanet al. 1715

2017 4

Database study (SEER)

1. OS 2. DDS

1. OS: 226.9 months 2. DDS: 444.0 months

Clinical outcome 1. Follow-up period for all patients from the time of the initial diagnosis ranged from 5 to 20years 2. All patients are alive with no evidence of disease. Clinical outcome At 6 years and 9 months of

Pineda-Daboin et al. 1717

2006 4

Retrospective case series

11 patients with LGNPPAc two patients with TL-LGNPPAc

treated with surgery

Wenig et al. 1716

1988 4

Retrospective study

Nine patients with LGNPPAc treated with surgery versus preoperative RT + surgery versus surgery + postoperative RT

follow-up, all patients were alive without recurrence or metastasis

Abbreviations: DSS, disease-specific survival; OS, overall survival; RT, radiation therapy; SEER, Surveillance, Epidemiology, and End Results.

up. 1717 Taken together, the overall 5-year survival rate was 100%. Lai et al. 1722 analyzed the treatment efficacy of EEA for a cohort of 28 LGNPPAc patients. Among 28 patients, 23 patients underwent endoscopic surgery alone, three patients received preoperative RT (to decrease tumor vol ume), and two patients received postoperative RT. All patients were alive without evidence of regional or distant metastases in the follow-up period (range 7–121 months). Two patients (7%, 2/28) experienced local disease recur rence. Huang et al. 1721 reported a case series of five patients

the risk of local recurrence if positive margins are obtained. 1721,1726,1728 Wenig et al. 1716 reported the clinical outcome of nine patients with LGNPPAcs. In nine cases, all tumors were resected via an open transpalatal approach. Three patients received preoperative or postoperative RT. At the median follow-up (6 years and 9 months), all patients were alive without recurrence or metastasis. A similar study by Pineda-Daboin et al. reporting surgical out comes of 13 patients revealed that all patients were alive without recurrence or metastasis at 5–20 years follow