xRead - Nasal Obstruction (September 2024) Full Articles

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meta-analysis of 184 patients showed that chemother apy improves survival when adverse features such as high-grade tumors, positive margins, and recurrence were present. 1289 On the other hand, Bouaoud et al. did not find any benefit and reported a potential deleterious effect. 1280 Thus, no clear conclusion on the indications for (neo)adjuvant chemotherapy can be drawn based on the available evidence, but most studies are in favor of its use in selected cases. The role of adjuvant RT is less controversial. Patel et al. suggested that adjuvant RT should be considered in case of positive or close margins. 1267 Guadagnolo et al. demonstrated that incorporating RT confers a prognostic advantage for disease control and survival only when sur gical margins are involved or uncertain. 1283 Proton beam RT has been suggested as a potential strategy for local con trol in patients with unresectable or incompletely resected craniofacial osteosarcomas. 1290 C Other sarcomas This section discusses rare primary sinonasal sarco mas, including biphenotypic sinonasal sarcoma (BSNS), fibrosarcoma, angiosarcoma, leiomyosarcoma, malignant peripheral nerve sheath tumor (MPNST), Ewing sarcoma (ES), adamantinoma-like ES (ALES), and synovial sar coma. Literature search did not meet ICAR inclusion cri teria for liposarcoma. Table XXIII.C.1 includes a summary of important findings from this review. Diagnosis of sinonasal sarcoma requires biopsy; if safe, a large specimen should be collected to reduce the high rate of initial misdiagnosis (46%). 1292 Surgical excision is the preferred treatment for resectable lesions and is associated with the highest survival rates. 1293 Tumor size, histologic grade, and margin status impact sur vival in patients with head and neck sarcoma. 1294 Sercarz et al. demonstrated that 63% of patients with sinonasal non-rhabdomyosarcoma with positive surgical margins recurred; 50% were successfully salvaged (follow-up range 2–12 years). 1236 Twelve patients with positive margins received adjuvant RT, 41.7% of whom had no further evi dence of disease. 1236 O’Sullivan et al. report encouraging results using preoperative neoadjuvant RT (50–60 Gy) in head and neck soft tissue sarcoma with 5-year OS ≥ 83% for patients with negative or microscopically posi tive margins. 1295 Treatment with chemotherapy or RT has not demonstrated improved outcomes, although random ized studies are lacking. 1295 Nevertheless, use of RT has increased from 55% between 1973 and 2008 to 64% from 2009 to 2014. 51,1296

71.7% (5 years) 49 > 95% 1301,1302 66%(mean 38months) 1312 40%–60% 1298,1316 44%–86% 51,1317,1323 45% (5 years) 1324 > 49% (5 years) 1329,1331

55 a 49 52 (24–85) 1301,1302 46 (10–87) 1312 47 (13–81) 1316 32 (7–70) 1317 31 (5–55) 1324 62 (38–81) 1329,1331

+ S100, SOX10;

Sex (M:F) 1:1 49 2:3 1301–1303 1:1 1312 3:2 1316 2:3 1317,1318,1323 2:1 1324 1:1 1329

perivascular

accentuation;

-H3K27me3 protein

Sarcoma MPNST

Spindle cells,

t(X;18)(p11;q11) transloca tion; + TLE1

Sx, neoadjuvant/ adjuvant CTX ± RT Sx ± CTX/RT Sx

(monophasic/

biphasic/poorly

differentiated);

Synovial

Spindle cells

Small round blue cells; + CD99, nuclear NKX2.2; EWSR1

gene rearrangement

Biphenotypic Sinonasal Sarcoma Leiomyosarcoma Angiosarcoma Ewing Sarcoma Low-grade spindle cells, herringbone pattern; + S100, smooth muscle actin, muscle-specific actin; PAX3::MAML3 fusion gene Spindle cells; + SMA, desmin, and/or caldesmon; S100 -, RB1 protein loss (75% of cases) Spindled/epithelioid cells with hemorrhage/ necrosis, CD31 and ERG + ;HHV-8– Sx ± RT Sx ± RT Sx ± CTX/RT Sx ± RT; consider preop embolization or storiform pattern

TABLE XXIII.C.1 Other primary sarcomas of sinonasal tract. Fibrosarcoma Age at presentation, years; mean (range) Histologic characteristics Spindle cells in chevrons, herringbone

(follow-up duration)

recommendations Overall survival

Primary treatment

Abbreviations: CTX, chemotherapy; MPNST, malignant peripheral nerve sheath. a Range not reported.