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KUANetal.

TABLE XXIII.A.6 Evidence surrounding induction chemotherapy for sinonasal rhabdomyosarcoma.

Clinical endpoints

Study

Year LOE Study design Study groups

Conclusions

Stevens et al. 1244 2005 3

1. OS 2. EFS

5-year OS of 59% in cohort treated with induction chemotherapy protocol

Prospective cohort

IVA, CEV, IVE, then evaluation RT if less than CR ( n = 27) in PM-RMS < 3years old Induction IVA × three courses then IVA if PR or DP if less than PR After six courses, if CR, then continued chemo If less than CR, then RT( n = 14) in PM-RMS with high-risk meningeal

Flamant

1998 3

Prospective cohort

1. OS 2. EFS

1. 5-year OS of 50% in cohort treated with an induction chemotherapy protocol 2. Planned randomized study abandoned due to poor compliance

et al. 1251

involvement, < 5 years old

Abbreviations: CEV, carboplatin + epirubicin + vincristine; IVA/VAI, ifosfamide + vincristine + actinomycin D; EFS, event-free survival; IVE, ifosfamide + vin cristine + etoposide; OS, overall survival; RT, radiation therapy.

subjective, with some lesions being clearly centered on structures of the sinonasal tract (e.g., the nasal septum) and others located midway between the sinonasal tract and adjacent anatomical compartments (e.g., the hard palate/alveolar bone). Since from a clinical standpoint chondrosarcoma and osteosarcoma of the craniofacial skeleton exhibit similar challenges irrespective of their origin, the present review includes both these entities. Surgery has an undisputed role in the management of both diseases. Locally uncontrolled disease represents the main cause of cancer-specific mortality for both tumors, which have the propensity to extend through bones, particularly in the medullary part, and this emphasizes the importance of complementing surgery with other modalities. How ever, evidence that clearly delineates the indications to chemotherapy and RT is lacking. Of note, the current section in ICSNT does not cover petroclival chondrosarcoma, which is discussed in ICSB 2019 section IX.B. 5 Chondrosarcoma Based on the search strategy, seven articles with suffi cient information on sinonasal chondrosarcomas were identified (Table XXIII.B.1). 266,1254–1259 Overall, 183 patients affected by sinonasal chondrosarcoma were included in these articles. In two studies, chondrosarcomas of the anterior skull base, nonanterior skull base, and sinonasal tract were included together; data of cases centered on 1

the sinonasal tract could not be extracted and were hence estimated from the full series data. Thus, the overall denominator is 238 subjects. Most relevant data extracted from these publications are reported herein and then discussed in the last section of the paragraph. Mean age at diagnosis was 45.5 years. Women were more frequently affected (132, 59.3%). Conventional chondrosar coma was the most frequent type (182, 76.5%), followed by mesenchymal (24, 10.1%), myxoid (12, 5.0%), dedifferenti ated (3, 1.3%), and clear cell subtype (2, 0.8%). In 15 (6.3%) cases, the histological subtype was not reported. Grade was not systematically reported. Clear cell and dedifferentiated subtypes have been reported to be associated with worse prognosis. 1260 The majority of patients received surgery (94.1%). Among those operated, 2.7% underwent curettage/biopsy, with the remaining treated with surgery aimed at achiev ing a maximal safe resection. The surgical resection was classified heterogeneously: 21.4% patients received resec tion with negative margins, 26.8% GTR with no informa tion on microscopic margin status, 6.7% resection with microscopically involved margins, 20.1% STR with macro scopic residual disease, and 8% a “wide resection” with no details on macroscopic residual disease and margin status. In 14.3% patients, the surgical procedure was not detailed. The method of resection is available for 105 patients, of which 53.3% were operated on endoscopically. The primary treatment included RT and chemotherapy in 35.3% and 3.8% patients, respectively. Thirty-six (15.1%) patients received proton beam RT, whereas the remain-