xRead - Nasal Obstruction (September 2024) Full Articles

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ing underwent photon-based RT. One- and 3-year OS and DSS estimates were in the range of 63.3%–100%. Five- and 10-year OS and DSS survival were in the range of 56.4%– 100%, with five of seven studies reporting estimates > 80% at 5 years. RFS was 60.7%–83.7% at 3 years and 42.9%–80.8% at 5 years. Considering the 91 patients with single-patient data available, 1-, 3-, 5-, and 10-year OS estimates were 92.3%, 81.2%, 75.7%, and 73.0%, respectively. The main cause of cancer-specific death was local recurrence or pro gression with compression of vital structures. Only three cases (1.3%) of distant metastases were reported. However, de Souza et al. published a systematic review including publications on chondrosarcomas of the mandible and maxilla in the period 1950–2017 and found distant metasta sis in 29 of 169 (17.2%) cases. 1261 Nodal primary involvement and nodal recurrence were rare. Advanced age, large tumor volume, and need for com bined surgery were associated with worse prognosis. 1257 Proton beam RT was found to significantly increase PFS. 1258 In the analysis of their entire series including also nonsinonasal, skull base chondrosarcomas, Rimmer et al. found that proton beam RT was associated with higher OS compared with photon-based RT. 1259 Sinonasal chondrosarcoma mostly affects patients around their fifth decade of life. Although a predilection for the female gender was found in this review, other studies showed a slight male prevalence. 1261 Maffucci syndrome and other enchondromatosis such as the Ollier disease are predisposing factors. 1262 Conventional chon drosarcoma is the most frequently represented subtype, which is further graded from I to III based on histomor phology. Mesenchymal and myxoid subtype have been reported in the sinonasal tract, whereas other subtypes are extremely rare. Mesenchymal chondrosarcoma, which harbors the typical HEY1::NCOA2 fusion, 1263 is associated with more aggressive behavior, with a higher propensity to recur and metastasize. 1255 However, mesenchymal chon drosarcoma of the craniofacial area shows less aggressive behavior compared with other anatomical sites. 1263 Surgical resection classification is nonhomogenous, with some studies distinguishing GTR versus STR and others reporting on margin status. If one considers the anatomical site, which does not allow for wide-margin resection around tumor surfaces, the frequent need for multibloc (with multiple oriented specimens) resection, the constraints of frozen section on bone, and the propen sity of chondrosarcomas to invade the medullary niches and Haversian system of grossly normal surrounding bones, defining resection based on margin status may be unreliable. Chemotherapy is rarely included in the treatment of sinonasal chondrosarcoma, mostly in the mesenchymal and dedifferentiated subtypes. In contrast, one third of

patients received RT. However, the role of adjuvant RT is debated. 1264 While there is evidence that adjuvant RT improves PFS, its effect on OS is less clear. 1258,1265 Thedis crepancy between PFS and OS may be attributable to the successful treatment of recurrences or slow progression, thus justifying reserving RT for cases that are deemed to be high risk for recurrence (e.g., recurrent tumors, moderate to-high-grade tumors, very extensive disease, multiple neoplastic microscopic foci in the resection bed). Particle therapy is reported to be more advantageous than pho ton RT in sinonasal and skull base chondrosarcoma. 1259 Based on the available data, no definite conclusions on the need for adjuvant RT can be drawn. Thus, postoper ative RT should be discussed in a multidisciplinary setting on a case-by-case basis, taking into consideration factors such as tumor subtype, grade, completeness of resection, and salvageability of recurrence, as well as patients age, comorbidities, and goals of treatment. 2 Osteosarcoma Nineteen articles with sufficient information on sinonasal osteosarcomas have been identified (Table XXIII.B.2). 1266–1283 Overall, 310 patients were included. In 10 studies, osteosarcoma of the superior craniofacial area and mandible were included together. In these papers, data of cases centered on the sinonasal tract could not be extracted and were estimated from the full-series data. Thus, the overall denominator was 551 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 36.7 years. Males were more frequently affected (340, 61.7%). Considering the 13 studies specifying previous exposure to radiation, 130 of 459 (28.3%) patients had secondary or “RT-associated” osteosarcoma. Chondroblastic type was the most fre quent (23.8%), followed by conventional “not otherwise specified” (11.3%), osteoblastic (10.3%), fibroblastic (8.0%), telangiectatic (1.3%), dedifferentiated (1.1%), and other rare subtypes. Histologic subtype was not specified in 39.2% of patients. Tumor grade was classified as high in 41.2%, inter mediate in 4.2%, and low in 12.0% and was not specified in 42.6% cases. The majority of patients received surgery (95.1%). Of these, 5.6% had macroscopic residual disease after surgery (R2). GTR (i.e., non-R2) was achieved in 81.7% patients, with microscopically negative (R0), “close” (with no met ric definition), positive (R1), and not specified margin status in 51.7%, 1.1%, 17.4%, and 11.4% cases, respectively. In 5.8% cases, the resection was classified as “incom plete” with no specification on the microscopic versus