. 2019 Sep 9;8(10):1330–1353. doi: 10.1530/EC-19-0341
This work is licensed under a Table 3.
Summary of literature review.
| Parameter | Value | No. of patients with available data |
|---|---|---|
| Age (years) (mean ± s.d.) | 46 ± 14 | 160 |
| Sex | 81:81 | 162 |
| Location of tumor % (no.) | 163 | |
| Paranasal sinuses | 43.7 (76) | |
| Mandible | 21.5 (34) | |
| Intracranial | 11.8 (19) | |
| Maxilla | 9 (13) | |
| Oral cavity | 6.2 (10) | |
| Skull | 1.2 (2) | |
| Parotid | 1.3 (2) | |
| Posterior neck | 1.3 (2) | |
| Cervical vertebra | 1.3 (2) | |
| Infratemporal fossa | 0.7 (1) | |
| Mastoid antrum | 0.7 (1) | |
| Thyroid | 0.7 (1) | |
| Local symptoms % (no.) | 44.1 (49) | 111 |
| Hypophosphatemic symptoms | ||
| Muscle weakness % (no.) | 77.9 (106) | 136 |
| Fractures % (no.) | 61.2 (68) | 111 |
| Bone pains % (no.) | 100 (142) | 142 |
| Bony deformities % (no.) | 25.7 (27) | 105 |
| Duration of symptoms (months), median (IQR) | 36 (24–72) | 139 |
| Biochemical profile | ||
| S. Calcium (mg %) (mean ± s.d.) | 8.9 ± 0.5 | 87 |
| S. Phosphorus (mg %) (mean ± s.d.) | ||
| Pre-op | 1.4 ± 0.4 | 119 |
| Post-op | 3 ± 0.7 | 62 |
| S. Alkaline phosphatase (U/L) (median (IQR)) | 313 (200–420) | 95 |
| TMP/GFR (median (IQR)) | 0.9 (0.6–1.3) | 39 |
| TRP (median (IQR)) | 61 (46.2–72.2) | 21 |
| PTH (pg/mL) (median (IQR)) | 55.9 (39.3–83.7) | 73 |
| 1,25 (OH)2 vitamin D3 (pg/mL) (median (IQR)) | 18 (8.2–26.2) | 46 |
| FGF-23 (Pre-op) (median (IQR)) | ||
| X ULN | 3.6 (1.8–6.8) | 55 |
| C-terminal (Ru/mL) | 573 (234–1058) | 33 |
| Intact (pg/mL) | 256 (131–393) | 22 |
| FGF-23 (Post-op) | ||
| C-terminal (Ru/mL) | 69.3 (36.5–138) | 18 |
| Intact (pg/mL) | 14 (5.9–50) | 15 |
| Tumor size (cm) (median (IQR)) | 2.5 (1.8–3.2) | 70 |
| Localization imaging % (no.) | 131 | |
| History and PE | 16.7 (22) | |
| X-ray | 2.3 (3) | |
| CT scan | 25.9 (34) | |
| MRI | 10.6 (14) | |
| Octreotide scintigraphy | 20.6 (27) | |
| FDG-PET/CT | 8.4 (11) | |
| Ga-DOTA-based PET/CT | 11.4 (15) | |
| Selective venous sampling of FGF-23 | 3.8 (5) | |
| Primary modality of treatment % (no.) | 160 | |
| Surgery | 97.5 (156) | |
| Radiation therapy | 1.2 (2) | |
| Combined surgery + radiation therapy | 1.2 (2) | |
| Complete response to primary treatment % (no.) | 80.4 (119) | 148 |
| Persistent disease % (no.) | 13.5 (20) | 148 |
| Follow-up (months) | 13 (5.2–36) | 108 |
| Recurrence % (no.) | 7 (9) | 128 |
| Time to recurrence (months) (range) | 2–204 | |
| Site wise persistence/recurrence % (no./no.) | ||
| Paranasal sinuses | 14.4 (7/4) | 76 |
| Mandible | 17.6 (6/0) | 34 |
| Intracranial | 36.8 (4/3) | 19 |
| Maxilla | 7.6 (1/0) | 13 |
| Oral cavity | 33.3 (1/2) | 10 |
| Thyroid | 100 (1) | 1 |
| Secondary modality of treatment % (no.) | 26 | |
| Surgery | 65.4 (17) | |
| RT | 30.8 (8) | |
| Chemotherapy | 7.7 (2) | |
| Cinacalcet | 7.7 (2) | |
| Octreotide | 7.7 (2) | |
| Radiofrequency ablation | 3.8 (1) | |
| PRRT | 3.8 (1) | |
| Others | 3.8 (1) | |
| Metastasis % (no.) | 2.7 (4) | 148 |
| Histopathology % (no.) | 158 | |
| PMTMCT | 48.7 (77) | |
| PMT ossifying fibroma like | 1.3 (2) | |
| PMT mixed epithelial and connective tissue type | 9.5 (15) | |
| Malignant PMTMCT | 3.2 (5) | |
| Hemangiopericytoma | 22.8 (36) | |
| Giant cell tumor | 3.2 (5) | |
| Odontogenic fibroma | 3.2 (5) | |
| Glomangiopericytoma | 2.5 (4) | |
| Malignant schwannoma | 0.6 (1) | |
| Meningioma | 0.6 (1) | |
| Salivary basal cell adenoma | 0.6 (1) | |
| Ameloblastic fibrosarcoma | 0.6 (1) | |
| Primitive mesenchymal tumor | 0.6 (1) | |
| Arteriovenous hemangioma | 0.6 (1) | |
| Spindle cell tumor with PMT features | 0.6 (1) | |
| Cellular non-descript | 0.6 (1) | |
| Chronic inflammatory tissue with fibrosis and epithelial cell rests | 0.6 (1) | |