Table 5.
Clinicopathological features of SMARCB1/INI1-deficient sinonasal carcinoma (SDSC) with original diagnosis of SNUC
| S No. |
Author, year ref | Age (years) |
Sex | Clinical presentation | Initial diagnosis | P16 IHC |
Revised diagnosis | Treatment & follow up |
|---|---|---|---|---|---|---|---|---|
| 1 | Bishop JA et.al., 2014 [6] | 33 | M | Proptosis, vision defect, mass in the nasal, ethmoid and frontal sinus | SNUC | ± | SDSC |
Local recurrence and distant metastasis DOD (30 months) |
| 2 | Bishop JA et.al., 2014 [6] | 54 | F | Headache, nasal discharge, mass in the nasal, ethmoid and maxillary sinus | SNUC | − | SDSC |
Local recurrence DOD (15 months) |
| 3 | Bell D et.al., 2015 [7] | 64 | F | Diplopia, mass in the frontal sinus | SNUC | Focal + | SDSC | NED (3 months) |
| 4 | Bell D et.al., 2015 [7] | 51 | F | Sinusitis, mass in the left temporal fossa and sphenoid | SNUC | Focal + | SDSC |
DOD (24 months) Local recurrence and distant metastasis |
| 5 | Zeng M et.al., 2016 [8] | 86 | M | Left maxillary sinus and nasal mass | SNUC | − | SDSC | Died of colorectal cancer (21 months) |
| 6 | Shatzkes DR et.al. 2016 [9] | 62 | M | NA | SNUC | ND | SDSC | NED (3 months) |
| 7 | Wasserman JK et.al.,2017 [10] | 34 | M | Facial swelling, loose dentition, right maxillary mass extending into orbital floor | SNUC | ND | SDSC | Local recurrence and distant metastasis to lung liver and bone. DOD (26 months) |
| 8 | Kakkar A et.al., 2018 [11] | 23 | M | Epistaxis, upper jaw pain, loosening of teeth | SNUC | ND | SDSC | Biopsy followed by 2 NACT (cisplatin + 5FU) followed by radical maxillectomy and PORT 60gy in 30 fractions; achieved CR; NED at 17 months |
| 9 | Kakkar A et.al 2018 [11] | 60 | M | Mass in the left nasal cavity | SNUC | ND | SDSC | Biopsy only; no follow-up information available |
| 10 | Kakkar A et.al., 2018 [11] | 40 | M | Vascular mass in the right nasal cavity | SNUC | ND | SDSC | Biopsy only; no follow-up information available |
| 11 | Fard EV et.al.,2019 [13] | 61 | M | Large sinonasal mass with orbital and brain extension, possible lung metastasis | SNUC | + | SDSC | AWD 17 months, with multiple bone metastasis |
| 12 | Fard EV et.al.,2019 [13] | 61 | M | Large sinonasal mass with brain, liver and lung metastasis | SNUC | − | SDSC | AWD (11 months) |
| 13 | Fard EV et.al.,2019 [13] | 75 | M | Large sinonasal mass with bilateral orbital and skull base involvement | SNUC | + | SDSC | AWD (7 months) |
| 14 | Present study | 61 | M | Swelling in the fronto-ethmoid region | SNUC | − | SDSC |
Underwent surgery followed by chemo & radiotherapy DOD after 2 years of diagnosis |
| 15 | Present study | 35 | F | Left sinonasal mass associated with, bleeding, decreased vision, CT PNS-Mass occupying left sided nasal cavity with extension into sphenoid, posterior ethmoid sinuses | SNUC | − | SDSC | Underwent surgery followed by chemo& radiotherapy. Alive without any recurrence (1 year 8 months duration) |
| 16 | Present study | 35 | F | Right facial pain, epistaxis for 1 year, 6 × 7 cm swelling over right orbit with right eye pushed outward, right sided nasal mass. CEMRI brain and PNS—A large heterogeneously enhancing mass cantered over right ethmoid sinus, nasal cavity with intracranial extension | SNUC | − | SDSC |
Anterior cranio-facial resection of right sided nasal mass and right orbital exenteration followed by chemoradiotherapy. Alive with local recurrence (c/o increase in swelling size) 5 months |
SNUC sinonasal undifferentiated carcinoma, SDSC SMARCB1/INI1 deficient sinonasal carcinoma, + positive, − negative, NA not available, ND not done, DOD died of diseases, NED no evidence of disease, AWD alive with disease