Table 1.
Clinicopathological features of sinonasal leiomyosarcomas (n = 9)
| No. | Age sex | Site | Treatment | Associated tumors | Previous irradiation or chemotherapy? | Family history | Follow up and outcome |
|---|---|---|---|---|---|---|---|
| 1 | 77 M | Right nasal cavity + maxillary sinus | Excision and radiotherapy |
Prostatic carcinoma at age 73 Sinonasal leiomyosarcoma at age 77 Rectal carcinoma at age 81 |
Anti-androgen therapy for prostate cancer | No |
55 months later, there was resection of multiple lung metastases of both rectal cancer and leiomyosarcoma Currently, alive with disease at 88 months |
| 2 | 48 F | Left nasal cavity + sinuses | Excision and adjuvant radiochemo-therapy | Tubulovillous rectal adenoma, 13 years later | No | No |
Local recurrence middle turbinate (26 months) Two metastases to the right lower lung lobe (13 years) Alive with no evidence of disease at 17 years |
| 3 | 43 F | Right nasal cavity | Excision and radiochemo-therapy | Retinoblastoma of right eye at age 3 | Radiotherapy for retinoblastoma | No |
At 16 months, imaging showed solitary lung metastasis, cerebral metastasis and local recurrence (not verified histologically) Lost to follow-up thereafter |
| 4 | 61 M | Nasal cavity | Surgery | Not known | Not known | Not known | Not known |
| 5 | 28 M | Maxillary sinus, infiltrating dura | Surgery | Not known | Not known | Not known | Multiple recurrences over 16 months, then lost to follow-up |
| 6 | 26 F | Maxillary sinus | Radical maxillect-omy |
Hodgkin Lymphoma (Stage IIIB in 3/2003) Allogenic stem cell transplantation after recurrent Hodgkin (2011) Bad GVHD |
Chemotherapy then stem cell transplant in 2004 | No |
Lung metastases at 20 months, treated with surgery Alive without evidence of disease at 8.2 years |
| 7 | 27 M | Maxillary sinus | Wide excision |
Retinoblastoma (bilateral) at 18 months (bilateral blindness) Macrocytosis Intellectual disability with epilepsy/seizures |
Yes: for retinoblastoma; 2 times: at initial and then at age 2 after second eye developed retinoblastoma | Mother had retinobla-stoma and rhabdo-myosarcoma |
Local recurrence at 2 years which resulted in brain invasion Dead of disease at 2.4 years |
| 8 | 66 F | Nasal cavity | Wide excision | Grade 1, Stage 1A uterine endometrioid adenocarcinoma (1999) | No | No | Alive without evidence of disease at 6.6 years |
| 9 | 66 M | Maxillary sinus | Wide excision | Polyostotic fibrous dysplasia | Yes: irradiation for fibrous dysplasia at a younger age | No | Brain extension resulted in death from disease at 0.5 years |
M male, F female, GVHD graft-versus-host-disease