xRead - Nasal Obstruction (September 2024) Full Articles

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other subsites. 1322 Metastatic disease occurs in 17% of cases. 1317 Published mortality rates range from 14% to 56%. 51,1317,1323 Data on sinonasal ALES are limited, though isolated reports suggest it may be more aggressive than sinonasal ES. 1318 6 Synovial sarcoma Sinonasal synovial sarcoma has approximately 30 reported cases. 1324,1325 SS exhibits calcifications on CT and a charac teristic “triple sign,” possessing hypo-, iso-, and hyperin tense signal on T2-weighted MRI. 1325 Treatment involves endoscopic or open surgery for resectable lesions. Dis ease progression or recurrence within 5 years ranges between 45% and 100%. 1325,1326 Adjuvant chemother apy and/or RT are often used, especially for high grade/advanced lesions; RT dose ranges from 60 to 70 Gy. 1320,1325,1326 Microscopically, this spindle cell sar coma may be monophasic, biphasic (with glandular ele ments), or poorly differentiated. 1325 SS is positive for SS18::SSX fusion-specific antibody (E9 × 9V) and/or an SSX-specific antibody (E5A2C) that show strong diffuse nuclear staining 1327 and variably positive for CD99, B cell lymphoma (BCL)-2, and cytokeratin. 1325 Identifying the pathognomonic t(X;18)(p11;q11) translocation may be diagnostically helpful for SS in uncommon anatomic locations. 1328 Outcomes data are limited, but studies sug gest a recurrence rate of approximately 66% within 2 years and a 5-year DSS of 45% for sinonasal SS. 1324,1325 7 Malignant peripheral nerve sheath tumor MPNSTs arise from peripheral nerves, from precursor benign nerve tumors, or in patients with neurofibromato sis type 1 (NF-1). Patients with NF-1 have a lifetime risk of 10% for development of MPNST, and tumors develop at a younger age. 1329 Microscopic hallmarks include fas cicles of alternating hypo- and hypercellular spindle cells with perivascular accentuation. The presence of heterolo gous elements, especially skeletal muscle (formerly, triton tumor), is seen in more aggressive MPNSTs and may connote a worse prognosis. Limited staining for S100 and SOX10 with complete loss of H3K27me3 expression is compatible with MPNST, but the diagnosis remains challenging. 1330 Prognosis is improved relative to other head and neck subsites. 1329 Definitive surgical therapy is recommended; CRT therapy alone has worse outcomes. 1329 Five-year OS for all head and neck MPNST with rhabdomy oblastic differentiation is 49%, with sinonasal subsite being a favorable prognostic factor. 1329,1331

(elevated mitotic rate, necrosis) demonstrates increased risk of local recurrence or distant metastasis. 1313,1315 Five year DSS for leiomyosarcoma of all head and neck subsites was 52.7% for poorly differentiated lesions and 87.6% for well-differentiated tumors. 1315 OS is 66% at mean follow-up of 38 months; distant metastasis is rare (8.1%). 1312 4 Angiosarcoma Sinonasal angiosarcomas typically present as nodular pur ple/red lesions. Angiography and embolization may be useful in the workup and treatment. 1316 Histologically, these vasoformative neoplasms infiltrate submucosa and bone as ramifying channels lined by spindled or epithelioid cells accompanied by necrosis and/or hemorrhage. 1316 Tumors express endothelial markers CD31 and ERG. 1316 HHV8 is negative, excluding Kaposi sarcoma. Risk of recurrence is reported at 38%, and OS is 40%–60% (mean follow-up: 121 months in one study), a significantly improved prognosis compared with nonsinonasal head and neck soft tissue angiosarcoma. 1298,1316 5 Ewing sarcoma Sinonasal ES has fewer than 100 cases reported. Though skeletal ES originates in long bones and affects chil dren, sinonasal ES affects any age (mean 32 years; range 7–70 years). 1317 Histologically, hypercellular sheets of small round blue cells with monotonous nuclei and occasional rosette formation necessitate broad testing to navigate the microscopic differential diagnosis. 1317 EScon sistently shows diffuse membrane CD99 positivity and nuclear NKX2.2 staining. 1317 ALES is a rare subtype of ES exhibiting basaloid appearance, epithelial differen tiation, and prominent mitoses. ALES is differentiated from classical ES by positive staining for cytokeratin, p63, and p40, an immunoprofile that overlaps with SCC. 1318 EWSR1 gene rearrangement is observed on molecular fluorescence in situ hybridization (FISH) testing in ES and ALES, with molecular confirmation of partners fre quently required. 1317 ES is chemo- and radiosensitive, but surgical excision with neoadjuvant/adjuvant therapy remains the primary treatment modality for resectable dis ease; multimodality therapy including chemotherapy is essential. 1317,1319,1320 Adjuvant RT doses range from 50.4 to 55.8 Gy. 1320 In appropriate candidates, endoscopic resec tion can be used to achieve a negative margin resection. 1321 Advanced tumors may be especially good candidates for neoadjuvant chemotherapy before surgery. ES of the head and neck is associated with improved prognosis versus