Table 1.
Clinical presentation and the outcome of patients with sinonasal carcinomas (Cohort 1).
| Case number |
Sex | Age | Location | Symptoms at presentation |
Diagnosis |
IDH2 status |
Size (cm) |
T stage |
N stage |
M stage |
Clinical Stage |
Primary treatment (post-recurrence treatment) |
Recurrence | Disease status at last follow- up (months) |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | M | 61 | Ethmoid sinus | Memory loss, personality changes | SNUC | Mutated | NA | cT4a | cN0 | cM0 | IVA | SX+RTCT | No | NED (102) |
| 2 | M | 57 | Ethmoid sinus and nasal cavity | Eye pain, proptosis | SNUC | Mutated | 5.1 | cT4b | cN0 | cM0 | IVB | SX+RTCX | Local recurrence and distant metastasis (bone, liver, abdominal lymph nodes) | DOD (6) |
| 3 | F | 49 | Ethmoid sinus and nasal cavity | Nasal congestion, headache | SNUC | Mutated | NA | cT4a | cN0 | cM0 | IVA | SX+RT | Regional metastasis (lymph nodes) and distant metastasis (liver) | AWD (13) |
| 4 | M | 48 | Nasal cavity | Double vision, swelling and proptosis of the eye, nasal obstruction | SNUC | Mutated | 6.2 | pT4b | cN2a | cM0 | IVB | SX+RTCX | Distant metastasis (bone) and regional metastasis (skin) | DOD (18) |
| 5 | M | 43 | Ethmoid sinus and nasal cavity | Nasal congestion, loss of smell and taste, supra-orbital headaches | SNUC | Mutated | 7 | cT4b | cN2c | cM0 | IVB | RTCX | No | NED (40) |
| 6 | M | 49 | Ethmoid sinus and nasal cavity | Nasal congestion, decrease in taste and smell | SNUC | Mutated | 5 | pT3 | cN0 | cM0 | III | SX+RTCX | No | NED (103) |
| 7 | F | 74 | Sphenoid sinus | Nasal congestion, epistaxis, headache | SNUC | wt | 3 | cT4a | cN0 | cM0 | IVA | NRTCX+SX | No | NED (96) |
| 8 | F | 72 | Nasal cavity | Visual changes, orbital pressure sensation | SNUC | Mutated | 4.5 | cT4b | cN0 | cM0 | IVB | RTCX | No | AWD (30) |
| 9 | M | 53 | Ethmoid sinus and nasal cavity | Nasal congestion | SNUC in situ | Mutated | N/A | pTis | cN0 | cM0 | 0 | SX (RTCX) | Local recurrence (soft palate) | NED (70) |
| 10 | M | 44 | Nasal cavity | Facial pain, swelling | SNUC | Mutated | 5.2 | cT4b | cN2 | cM0 | IVB | CX | Distant metastasis (adrenal gland) | DOD (15) |
| 11 | F | 83 | Ethmoid sinus and nasal cavity | Nasal obstruction, epistaxis | PD non-ITAC | Mutated | 2.5 | cT2 | cN0 | cM0 | II | SX+RT | No | DOUC (69) |
| 12 | M | 39 | Nasal cavity | Nasal obstruction | PDCA with focal glandular/acinar differentiation | Mutated | 5 | pT4a | cN0 | cM0 | IVA | NCX+SX+RTCX | Regional metastasis (lymph nodes) and local recurrence | DOD (33) |
| 13 | F | 65 | Nasal septum | Nasal obstruction | PDCA with focal glandular/acinar differentiation | wt | 2.8 | cT2 | cN0 | cM0 | II | SX+RT (RTCX for local recurrence) | Local recurrence, distant metastasis (bone) | DOD (34) |
| 14 | M | 49 | Maxillary sinus | Headaches, ear obstruction, maxillary and facial pain, diplopia, eye swelling | PDCA with focal glandular/acinar differentiation | wt | NA | cT4b | cN0 | cM0 | IVB | RTCX | No | AWD (14) |
| 15 | M | 47 | Ethmoid sinus | Headaches, blurred vision, facial pain, ear pain, eye swelling | PDCA with focal glandular/acinar differentiation | wt | 4.4 | cT4b | cN0 | cM0 | IVB | SX+RTCX | Local recurrence and distant metastasis (bone, liver, abdominal lymph nodes) | DOD (24) |
| 16 | M | 56 | Nasal cavity | Headache, weakness, poor appetite | PDCA with focal glandular/acinar differentiation | wt | 4.6 | cT4b | cN0 | cM1 | IVC | RTCX | Local recurrence and distant metastasis (liver) | DOD (16) |
| 17 | M | 51 | Sphenoid sinus | Headache, nasal congestion | PDCA with focal glandular/acinar differentiation | wt | 4.5 | pT4a | cN0 | cM0 | IVA | SX+RTCX | Local recurrence and distant metastasis (bone) | DOD (28) |
| 18 | M | 44 | Ethmoid sinus and nasal cavity | Nasal congestion, epistaxis | Non-ITAC, HG | wt | 6.5 | cT4a | cN0 | cM0 | IVA | SX+RTCX | No | NED (10) |
| 19 | F | 30 | Nasal cavity | Nasal congestion, visual changes, sensation deficits | PDCA with focal glandular/acinar differentiation | wt | 4.8 | pT4b | cN0 | cM0 | IVB | NCX+SX+RTCX (RT) | Local recurrence (orbit) and distant metastasis (spine, pleura, adrenal gland) | DOD (30) |
| 20 | M | 45 | Ethmoid sinus | Headache | HGNEC | Mutated | 3.1 | cT4a | pN2b | cM0 | IVA | SX (RTCX for local recurrence) | Local recurrence | NED (60) |
| 21 | F | 62 | Ethmoid sinus and nasal cavity | Sinus congestion, facial pain, occasional double vision | SCNEC | wt | 4.6 | cT4b | cN0 | cM1 | IVC | CX+RT | Local recurrence and distant metastasis (bone) | DOD (7) |
| 22 | F | 62 | Ethmoid sinus and nasal cavity | Nasal congestion, epistaxis | SCNEC | wt | 3.4 | pT4b | cN0 | cM0 | IVB | SX+RTCX | Local recurrence and distant metastasis (lung) | AWD (21) |
| 23 | M | 54 | Ethmoid sinus and nasal cavity | Nasal congestion | SMARCB1-deficient sinonasal carcinoma | wt | 2.6 | cT4a | cN0 | cM0 | IVA | SX (RTCX for local recurrence) | Distant metastasis (bone, brain) | DOD (39) |
| 24 | M | 47 | Ethmoid sinus | Nasal congestion, epistaxis, pain above eye | SMARCB1-deficient sinonasal carcinoma | wt | 4.0 | cT4b | cN0 | cM0 | IVB | NRTCX+SX | Local recurrence | AWD (7) |
| 25 | M | 54 | Ethmoid sinus and nasal cavity | Epistaxis | SMARCB1-deficient sinonasal carcinoma | wt | 4.5 | cT4b | cN0 | cM0 | IVB | NCX + SX | Local recurrence and distant metastasis (bone) | AWD (23) |
| 26 | M | 95 | Ethmoid sinus and nasal cavity | Progressive mental and gait deficit, behavioral changes | SMARCB1-deficient sinonasal carcinoma | wt | NA | cT4b | cN0 | cM0 | IVB | SX | N/A | N/A |
| 27 | F | 77 | Ethmoid sinus | Nasal congestion, obstruction, and purulent drainage | NUT midline carcinoma | wt | NA | cT4b | cN0 | cM0 | IVB | RT | Local recurrence and distant metastasis (bone, lung, mediastinum) | DOD (5) |
| 28 | F | 76 | Nasal cavity | Nasal congestion | MD-ITAC with signet-ring cells and mucinous features | wt | NA | cT4a | cN0 | cM0 | IVA | SX+RT | Local recurrence and regional metastasis (lymph nodes) | DOD (24) |
| 29 | M | 80 | Clivus/skull base | Visual changes, rhinorrhea | MD-ITAC | wt | 5.0 | cT4b | cN0 | cM0 | IVB | RTCX | No | AWD (7) |
| 30 | F | 81 | Nasal cavity | Epistaxis | PDCA with neuroendocrine and glandular differentiation | wt | 5.9 | cT4a | cN0 | cM0 | IVA | SX+RT | No | NED (37) |
Abbreviations: SNUC=sinonasal undifferentiated carcinoma, HGCA=high-grade carcinoma, PDCA=poorly-differentiated carcinoma, HGNEC=high-grade neuroendocrine carcinoma, large cell type, MD-ITAC=moderately-differentiated intestinal type adenocarcinoma, SCNEC=small cell neuroendocrine carcinoma, HG=high-grade,wt=wild type, SX=surgery, RT=radiation therapy, RTCX=chemoradiation, NCX=neoadjuvant chemotherapy, NRTCX=neoadjuvant chemoradiation, DOD=died of disease, AWD=alive with disease, NED=no evidence of disease, DOUC=died of unknown causes.