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ICAR SINONASAL TUMORS

TABLE XXIV.B.3 Evidence surrounding SMARCB1 ( INI1 )- and SMARCA4 ( BRG1 )-deficient sinonasal carcinomas.

Clinical endpoints

Study

Year LOE Study design Study groups

Conclusion

Ayyanar

2021 4

Retrospective case series

SMARCB1 : Sx + CRT (3)

Clinicopathologic characteristics

Mixed pattern of cells with a predominance of basaloid morphology

et al. 1436

McLean

2021 4

Retrospective case series

SMARCB1 (6), SMARCA4 (2): Treatment not reported SMARCB1 (17): Treatment not reported SMARCA4 : C(3),

Clinicopathologic characteristics

1. SMARB1 lower grade than SMARCA4 2. Cytomorphology not an accurate predictor 12% alive without disease at 1 year

Holden et al. 1429

Mittal et al. 1434

OS

2021 4

Retrospective case series

Agaimy et al. 1433 2020 4

Retrospective case series

Disease related mortality

66% disease related mortality (median of 3 months)

Sx + RT (1), Sx (1), palliative (1), unknown (4) SMARCB1 : Sx + CRT (2), IC + CRT(4)

Chitguppi et al. 1456

2020 4

Retrospective case series Retrospective case series

1. OS 2. DFS

SMARCB1 significantly worse DFS and OS than SNUC Suspected for high-grade carcinomas with nonkeratinizing squamous or rhabdoid morphology

Allard et al. 1457

2018 4

SMARCB1 (4):

Clinicopathologic characteristics

Treatment not reported

Agaimy et al. 1431 2017 4

OS

1. Median age of 52 years 2. 30% survival at a median follow-up of 2 years

Retrospective case series

SMARCB1 : Sx + CRT (22), Sx (4), CRT (5),

palliative (5), unknown (6)

Lacoet al. 1458

2017 4

Retrospective case series Retrospective case series

SMARCB1 : Sx + RT (3), Sx + CRT(1)

Clinicopathologic characteristics

Higher methylation of RASSF1 gene

Radiographic features

Avid contrast enhancement, intermediate-low T2 signal, FDG avid, calcifications, periosteal reaction 1. Undifferentiated basaloid “blue” appearance 2. Strong expression of pancytokeratin Basaloid/rhabdoid morphology, restricted to the sinonasal tract

2016 4

SMARCB1 (17):

Shatzkes

et al. 1437

Treatment not reported

Agaimy et al. 1431 2015 4

Retrospective case series

SMARCB1 : Sx + RTor CRT(11)

Clinicopathologic characteristics

Bell 1459

Clinicopathologic characteristics

2015 4

Retrospective case series

SMARCB1 : Sx + CRT (3), IC + Sx + CRT (1) SMARCB1 : Sx + RT(5), Sx + CRT (3), Sx (1)

Bishop et al. 1440 First identification and description of pathogenesis Abbreviations: CRT, chemoradiation therapy; DFS, disease-free survival; IC, induction chemotherapy; OS, overall survival; RT, radiation therapy; Sx, surgical therapy. 2014 4 Retrospective case series Clinicopathologic characteristics

while SMARCA4 -deficient carcinoma tends to be higher grade. 1429 Both tumors are positive for pancytokeratin and AE1/AE3, while SMARCB1 and SMARCA4 are diffusely negative for INI1 and BRG1, respectively. 1435 These tumors typically originate in the ethmoid sinus with local invasion into the orbit or anterior cranial fossa. 1437 Both tumors can occur outside the sinonasal tract, including the soft tissues of the neck. 1438 There is moderate diffusion restriction on MRI with low to intermediate signal on T2. 1192,1437 These tumors have calcifications in half of cases and occasionally

have aggressive periosteal reactions, visualized as a “hair on end” appearance. Outcomes for patients with SMARCB1 - and SMARCA4 deficient carcinoma are worse than those for SNUC patients. 1439 SMARCB1 - and SMARCA4 -deficient car cinomas are clinically aggressive, and patients usually present with local invasion. 1435 The vast majority of patients present with advanced disease, and half present with locoregional or distant metastasis. 1431,1434,1440 While reports of long-term outcomes are scarce, studies show a