Management | n (%) / involved sites | References | ||
---|---|---|---|---|
Unifocal (n = 44) | Multifocal (n = 11) | Multisystem (n = 9) | ||
Total excision | 18 (40.9)/skull, spine, sternum | 2 (18.2)/temporal + intracranial extention + sigmoid sinus, + mandible, Both jaws | – | [8, 16, 18, 20, 22,23,24,25,26,27,28,29,30,31, 40, 42, 52, 57]a |
Total excision + chemotherapy | 2 (4.5)/spine, rib | 1 (11.1)/petrous + SCC + middle cranial base | ||
Subtotal excision + chemotherapy | 1 (2.3)/skull | 1 (9.1)/petrous + inner ear + sphenoid | 1 (11.1)/bilateral mastoid + C2 | |
Subtotal excision + radiotherapy | 1 (2.3)/spine | 1 (9.1)/clivus, sella + sphenoid sinus | ||
Radiosurgery | 1 (2.3)/skull | – | – | [17] |
Radiotherapy | 1 (2.3)/spine | 1 (9.1)/temporal + clivus + occipital + atlantoaxial joint | – | |
Radiotherapy + epidural steroid injection | 1 (2.3)/ [51] | – | – | [51] |
Systemic chemotherapy | 2 (4.5)/clavicle, skull | 4 (36.3) /odontoid process + C2 + femur, bilateral petrous + bilateral labyrinth, both jaws + petrous apex + otic capsule + clivus, sternum + greater trochanter + tibia | 3 (33.3)/bilateral parietal + both jaws, maxillary sinus, Ilium + T6 + 6th rib | |
Chemotherapy + radiotherapy | – | – | 1 (11.1)/temporal with involving dura | [3] |
Analgesics, + intralesional steroid injection + chemotherapy | – | – | 1 (11.1)/both femur | [66] |
Curettage | 9 (20.5)/clavicle, sternum, humerus, acetabulum, metatarsal | – | – | |
Curettage + radiotherapy | 2 (4.5)/sternum, metacarpal | – | – | |
Observation | 4 (9.0)/sternum, clavicle, scapula, ilium | – | – | |
Conservative | 2 (4.5)/scapula, spine | 1 (9.1)/radius, ulna | 1 (11.1)/femur | |
Not determined | – | 1 (9.1)/both jaws | 1 (11.1)/sacral | |
Outcome after initial treatment | n (%) | |||
Complete recovery without complication | 33 (75.0) | 6 (54.5) | 3 (33.3) | |
Complete recovery with permanent complication | 1 (2.3) | 3 (27.3) | 2 (22.2) | |
Recurrence | 1 (2.3) | 1 (9.1) | 1 (11.1) | |
Lost follow- up | 1 (2.3) | 0 (0.0) | 0 (0.0) | |
Not determined | 8 (18.1) | 1 (9.1) | 2 (22.2) | |
Death | 0 (0.0) | 0 (0.0) | 1 (11.1) |