Table 1.

Clinical features, diagnosis, and outcomes of intracranial phosphaturic mesenchymal tumor.

No.	Author/year	Age/sex (years)	Location	Size (cm)	Diagnosis	Symptoms	Osteomalacia	Serum phosphate	Serum calcium	ALP	1,25-(OH) 2D	25 (OH) D	Serum PTH	Serum FGF23	FGF23 expression in tumor	TFOTD (yr)	Treatment	Adjunctive therapy*	Outcome and recurrence (mo)
1	David et al (1996)[7]	60/F	Right frontal Anterior cranial fossa	–	PMT	Right hip pain, change in behavior and personality with increasing apathy, memory disturbance and bilateral anosmia	Yes	↓	Normal	↑	↓	↓	Normal	–	–	1.5	Surgery	No	Subtotal resection Recurrence (36)
2	Compta et al (1998)[8]	69/F	Right ethmoido-frontal extending to the anterior cranial fossa	–	PMT	Diffuse bone pain, progressive difficulty in walking and intracerebral hemorrhage	Yes	–	–	–	–	–	↑	–	–	19	Biopsy	No	Died (12 d)
3	Filho et al (2004)[9]	47/F	Left cavernous sinus	3.0*2.0*2.0	PMT	Muscle pain and weakness, visual disturbances and diplopia	Yes	↓	Normal	↑	–	–	–	–	–	7	Surgery	No	Subtotal resection Improved (48)
4	Kaylie et al (2006)[10]	46/F	Left temporal Posterior cranial fossa	–	PMT	Primary symptom: pulsatile tinnitus of left ear and vertigo Recurrent symptom: fullness of left ear, dizziness and bone pain	Yes	↓	–	↑	Normal		–	–	–	–	Surgery	No	Recurrence (24)
5	Yoshioka et al (2006)[11]	45/M	Left clivus region Posterior cranial fossa	–	PMT	Primary symptom: posterior neck and bone pain, left hypoglossal nerve palsy Recurrent symptom: painful motor impairment and multiple bone fractures	Yes	↓	Normal	↑	↓	–	Normal	Normal	–	2.5	Surgery	GKRS Octreotide therapy	Subtotal resection Recurrence (19)
6	Elston et al (2007)[12]	69/F	Frontal and parietal bones extending into the venous lakes and arachnoid granulation	–	PMT	Hypophosphatemia, bone pain and muscle weakness, wheelchair bound with a thoracic kyphosis and severe proximal myopathy	Yes	↓	–	↑	↓	Normal	↑	↑	Positive	5	Surgery	Octreotide therapy	Cured, NED (7)
7	Kobayashi et al (2011)[13]	53/F	Right temporal bone extending to the middle cranial fossa	–	PMT	Systemic bone pain and muscle weakness, vertebral and pelvis fractures	Yes	↓	Normal	–	–	–	–	↑	Positive	4	Surgery	Preoperative embolization	Cured, NED (13)
8	Uno et al (2011)[14]	53/F	Right temporal bone extending to the middle cranial fossa	2.0	PMT	Progressive body pain and unable to walk	Yes	↓	–	↑	–	–	–	↑	Positive	4	Surgery	Preoperative embolization	Cured, NED (2 wk)
9	Uno et al (2011)[14]	61/M	Left frontal Anterior cranial fossa	2.2	PMT	Primary symptom: body pain, difficulty in walking and intracerebral hemorrhage Progression: bed-ridden because of severe pain	Yes	↓	–	↑	–	–	–	↑	Positive	5	Surgery	No	Cured, NED (6)
10	Syed et al (2011)[15]	71/F	Right temporal bone extending to the middle cranial fossa	–	PMT	Primary symptom: hearing loss in the right ear Recurrent symptom: House–Brackmann grade IV, infranuclear facial nerve palsy on the right side	No	–	–	–	–	–	–	–	–	1	Surgery	No	Recurrence (12)
11	Chokyu et al (2012)[16]	57/M	Left temporal Middle cranial fossa	2.7*1.8*2.0	PMT	Bone pain and multiple bone fractures	Yes	↓	Normal	–	↓	–	–	↑	–	2	Surgery	No	Cured, NED (12)
12	Ling et al (2012)[17]	43/F	Right temporal bone extending to the sinus cavernous	1.7*1.3	PMT	Dffuse and progressive bone pain, muscle weakness, progressive dffculty in walking, esotropia in right eye and horizontal diplopia	Yes	↓	↓	↑	–	–	↑	–	–	4	Surgery	No	Subtotal resection Improved (8 d)
13	Bower et al (2012)[18]	67/F	Left frontal Anterior cranial fossa	7.4*4.3*5.1	PMT	Progressive abulia, apathy, depression, and urinary and fecal incontinence	No	–	–	–	–	–	–	–	Positive	1 mo	Surgery	No	Cured, NED (18)
14	Mathis et al (2013)[19]	28/F	Right frontal Anterior cranial fossa	1.8*1.8*1.0	PMT	Back stiffness upon exertion and proximal lower-extremity weak	Yes	↓	Normal	↑	↓	–	–	–	Positive	3	Surgery	No	Cured, NED (30)
15	Mathis et al (2013)[19]	32/M	Left frontal Anterior cranial fossa	–	PMT	Intracranial hemorrhage, hip and low-back pain	Yes	↓	–	–		–	–	–	Positive	13	Surgery	No	Recurrence (24)
16	Fathalla et al (2015)[20]	49/F	Right frontal Anterior cranial fossa	4.8*4.0*4.7	PMT	Multiple bone fractures	Yes	↓	Normal	–	↓	Normal	Normal	↑	–	3	Surgery	No	Cured, NED (6)
17	Ellis et al (2016)[21]	8/F	Right cerebellar hemisphere Posterior cranial fossa	–	PMT	Headache, unable to walk and ataxia	No	–	–	–	–	–	–	–	–	–	Surgery	No	Cured, NED (42)
18	Basu et al (2016)[22]	53/F	Left occipital Posterior cranial fossa	3.5*2.7	PMT	Bilateral groin pain and difficulty walking.	Yes	↓	–	–	–	–	–	↑	–	2	Surgery	PRRT	Subtotal resection First resection: recurrence (4) Second resection: recurrence (9)
19	Mulani et al (2017)[23]	48/F	Left temporal and occipital Posterior cranial fossa	–	PMT	Bilateral hips and thighs pain and progressive difficulty in walking, tinnitus and heaviness of the left ear	Yes	↓	Normal	↑	–	–	↑	↑	–	2	Surgery	Preoperative embolization	Cured, NED (1)
20	González et al (2017)[24]	42/M	Left nasal fossa extending to the anterior cranial fossa	8.0*4.0	PMT	Weakness and diffuse pain in the extremities, nasal obstruction	Yes	↓	Normal	↑	–	–	↑	↑	–	6	Surgery	No	Cured, NED (6)
21	Hana et al (2017)[25]	38/F	Right frontal, anterior cranial fossa extruding into bilateral ethmoid sinuses	–	PMT	Progressive bone, muscle pain and olfactory disturbance	Yes	↓	Normal	–	Normal	–	–	↑	Positive	7	Surgery (twice)	No	Cured, NED (25)
22	Ding et al (2018)[26]	68/F	Right frontal Anterior cranial fossa	–	PMT	Primary symptom: hypophosphatemia and Osteoporosis, no obvious bone pain Recurrent symptom: diffuse bone pain, frequent fractures	Yes	↓	–	–	–	–	–	–	–	–	Surgery	No	Recurrence (60)
23	Villepele et al (2018)[27]	41/F	Right nasal cavity and ethmoid sinus extending to the anterior cranial fossa	–	PMT	Multiple fractures, major disability and pain, nasal obstruction	Yes	–	Normal	–	–	–	Normal	–	–	–	Surgery	No	Cured, NED (9)
24	Mishra et al (2019)[28]	46/M	Right temporal Posterior cranial fossa	1.9*1.7*2.6	PMT	Progressive difficulty in walking and bilateral femoral fracture	Yes	↓	–	↑	↓	–	–	↑	–	5	Surgery	No	Cured
25	Mishra et al (2019)[28]	52/F	Left occipital Posterior cranial fossa	–	PMT	Bilateral hips and thighs pain and progressive difficulty in walking	Yes	↓	Normal	–	–	–	Normal	↑	–	3	Surgery	No	Cured, NED (3)
26	Walsh et al (2019)[29]	30s/F	Left cerebellopontine angle Posterior cranial fossa	1.5*2.0	PMT	Left tinnitus, distorted hearing, and occa sionalotalgia	No	↓	↓	–	–	–	–	–	Positive	–	Surgery	No	Subtotal resection Improved (1 d)
27	Gunawat et al (2019)[30]	53/F	Left jugular foramen Posterior cranial fossa	–	PMT	Bilateral hip, knee joint pain, and backache, proximal muscle weakness along with walking difficulty	Yes	–	–	–	–	–	–	↑	–	5	Surgery	Preoperative embolization	Cured, NED (6)
28	Gunawat et al (2019)[30]	45/M	Middle cranial fossa	–	PMT	Lower limbs and back pain	Yes	↓	–	–	–	–	–	↑	–	–	Surgery	No	Cured, NED (6)
29	Hadelsberg et al (2019)[31]	58/M	Left frontal Anterior cranial fossa	2.6*1.9	PMT	Osteoporosis, bone pain, muscle weakness, gait disturbances, and seizure	Yes	↓	Normal	–	–	–	–	–	–	6	Surgery	No	Cured, NED (2)
30	Colazo et al (2020)[32]	29/M	Right temporal Middle cranial fossa	1.3*1.1*1.0	PMT	Low-back and hip pain, gait changes, proximal muscle weakness, and multiple stress fractures	Yes	↓	Normal	↑	↓	–	↑	Normal	–	2	Surgery	No	Cured, NED (12)
31	Tang et al (2020)[33]	54/F	Right temporal bone extending to the middle cranial fossa	3.7	PMT	TIO-related symptoms, right tinnitus for and right ear hearing loss	Yes	↓	–	–	–	–	–	–	–	2	Surgery	Preoperative ICA stenting and embolization	Cured, NED (42)
32	Tang et al (2020)[33]	42/M	Right nasal cavity and ethmoid sinus extending to the anterior cranial fossa	3.5	PMT	TIO-related symptoms and hyposmia	Yes	↓	–	–	–	–	–	–	–	8	Surgery	Postoperative local radiotherapy	Subtotal resection First recurrence (24) Second recurrence (12) Died (24)
33	Riminucci et al (2022)[34]	39/F	Right nasal cavity and ethmoid sinus extending to the anterior cranial fossa	2.0	PMT	Muscular weakness, back pain, and fractures of vertebrae and hip, nasal obstruction and headache	Yes	↓	Normal	↑	↓	Normal	Normal	–	Positive	3	Surgery	No	Cured, NED (2)
34	Argersinge et al (2022)[35]	64/M	Right posterolateral frontal sinus extending to the anterior cranial fossa	3.0	PMT	Diffuse bone pain and weight loss	Yes	↓	Normal	↑	–	Normal	↑	↑	–	5	Surgery	No	Cured, NED (24)
35	Argersinge et al (2022)[35]	62/M	Right jugular foramen Posterior cranial fossa	2.4	PMT	Multiple fractures, diffuse bone pain and right-sided hearing loss	Yes	↓	Normal	↑	–	↓	↑	↑	Positive	–	Surgery	No	Cured, NED (30)
36	Kojima et al (2022)[36]	45/M	Right frontal Anterior cranial fossa	–	PMT	Right ankle and hip pain	Yes	↓	Normal	–	–	–	–	↑	Positive	–	Surgery	No	Cured, NED (120)
37	Present case (2024)	56/M	Right temporal and occipital Posterior cranial fossa	5.0*4.0*2.5	PMT	Headache, dizziness, multiple fractures and bone pain	Yes	↓	Normal	↑	–	↓	Normal	↑	–	8	Surgery	No	Subtotal resection Improved (3)

En dash indicates unavailable.

1,25-(OH) 2D = 1,25-dihydroxyvitamin D, 25 (OH) D = 25-hydroxyvitamin D, ALP = alkaline phosphatase, FEBI = focused external-beam irradiation, FGF23 = fibroblast growth factor 23, GKRS = gamma knife radiosurgery, ICA = internal carotid artery, NED = no evidence of disease, PMT = phosphaturic mesenchymal tumor, PRRT = peptide receptor radionuclide therapy, PTH = parathyroid hormone, SRT = stereotactic radiotherapy, TFOTD = time from onset to diagnosis.

^* Oral medication therapy was excluded from adjunctive therapy because most patients took oral medications such as phosphates and vitamin D.