Table 1 Summary table of systematic review of NCMH cases reported in the literature

(1) McDermot, 1998 [10] USA

1

5 D

M

ND

1. Nasal cavity

1. Nasal Mass

ND

CT

Surgical excision

No recurrence at2 years

2. Respiratory Difficulties

2

3 M

F

ND

1. Nasal cavity

1. Nasal Mass

ND

MRI

Surgical excision

No recurrence at 2 years

2. Ethmoid Sinus

2. Otitis Media

3. Intracranial extension

3

3 M

M

ND

1. Nasal cavity

1. Choanal Mass

ND

ND

Biopsy then surgical excision Subsequent chemotherapy

No recurrence at 4 years

2. Respiratory distress

4

2 M

M

ND

1. Nasal cavity

1. Nasal Mass

ND

ND

Surgical excision

No recurrence at 18 months

2. Intracranial extension

5

12 D

F

ND

1. Nasal cavity

1. Nasal Mass

ND

CT

Surgical excision & further re-excision after 16 months

Unchanged persistent tumour in superior nasal cavity at 12 months

2. Intracranial extension

6

14 D

M

ND

1. Nasal cavity

1. Nasal Mass

ND

CT

Surgical excision VP shunt for hydrocephalus

Residual tumour in anterior cranial fossa at 9 months

2. Ethmoid sinus

2. Hydrocephalus & agenesis of corpus callosum

3. Intracranial extension

7

7 Y

M

ND

1. Nasal cavity

1. Nasal Mass

PPB

ND

Surgical excision

No recurrence at 2 months NB later reported by Priest et al. 2010 [27]- showed with multiple recurrences in first 3 years

2. Sphenoid sinus

2. Nasal Congestion

(2) Chae 1999 Korea [30] *abstract only, Korean paper

8

3 M

F

Right Size: 3.5 × 7.5 × 2.5 cm

1. Nasal cavity

1. Epistaxis

ND

CT

Surgical excision

ND

2. Ethmoid sinus

2. Obstruction

3. Cribriform plate

(3) Kim D 1999 [31] USA

9

3 M

F

Right Size: ND

1. Nasal cavity

1. Nasal mass

None stated

CT MRI

Surgical excision with mid-facial de-gloving and bi-frontal craniotomy

No recurrence at 18 months

2. Intracranial extension

2. Otitis media

3. Ethmoid sinus

(4) Kato, 1999 [17] Japan

10

4 M

M

Left Size: ND

1. Nasal cavity

1. Nasal Mass

None stated

CT

Two stage surgical excision 1st intracranial/sinus lesion, 2nd intranasal lesion

No recurrence at 13 years

2. Intracranial extension

2. Respiratory distress with cyanosis when feeding

3. Extension to left orbit

3. Opthalmoplegia left eye

Radiotherapy post op

(5) Hsueh 2001 [32] Taiwan (2 cases)

11

0 D

M

Left Size: ND

1. Nasal cavity

1. Left facial swelling

None stated

CT MRI

Excision biopsy then subsequent surgical excision with lateral rhinotomy and craniofacial approach

Recurrence after excision biopsy No recurrence at 5 years after second surgery

1. Sphenoid sinus

2. Left nasal mass

3. Ethmoid sinus

3. Respiratory distress & cyanosis when feeding

4. Compression of left orbit

4. Proptosis on recurrence

12

9 M

M

Right ND

1. Nasal cavity

1. Asymmetric face

None stated

CT MRI

Surgical resection

No recurrence at 9 months

2. Maxillary sinus

2. Right opthalmoplegia, enopthalmos and hypotropia

(6) Alrawi 2003 [14] Ireland

13

16 Y

M

Left 1.5 × 1.5 cm

1. Nasal cavity

1. Nasal swelling

None stated

CT MRI

Surgical resection with delayed reconstruction with forehead flap

No recurrence at 8 months

(7) Shet, 2004 [21] India

14

1 Y

M

Left Size: ND

1. Nasal cavity

1. Proptosis of left eye

Non stated

CT

Chemotherapy (VID) as biopsy suggested spindle cell sarcoma- 30 % reduction in tumour size Then Left maxillectomy and surgical excision

Residual tumour at 1.5 years near eye but no further re-growth & stable

2. Extension into left orbit

2. Left facial swelling

3. Ethmoid sinus

4. Sphenoid sinus

(8) Kim B, 2004 [22] Korea

15

5 M

M

Left Size: ND

1. Nasal cavity

1. Left eye ptosis

None stated

CT

Frontal craniotomy & trans-nasal surgical resection

ND

2. Compression of left orbit

3. Defect left ethmoidal bone

4. Defects anterior cranial fossa

(9) Norman, 2004 [15] USA

16

11 Y

M

Left Size: ND

1. Nasal cavity

1. Headaches left sided

None stated

CT

Endoscopic biopsy and anterior craniofacial resection

ND

2. Displacement left orbital wall

(10) Ozolek, 2005 [11] USA (4 cases)

17

11 Y

M

Left Size: ND

1. Nasal cavity

1. Nasal mass

None stated

ND

Surgery and care undertaken in another hospital

ND Surgery and care undertaken in another hospital

2. Ethmoid sinus

3. Extension into left orbit

18

17 Y

F

ND Size: ND

1. Nasal cavity

1. Nasal obstruction

None stated

ND

Surgical excision

ND

2. Facial pain

19

25 Y

M

Bilateral 8 × 5 × 3.5 cm

1. Nasal cavity

1. Respiratory distress from obstructing oropharyngeal tumour requiring emergency tracheostomy

1. Multiple Intracranial vascular aneurysms 2. Longstanding nasopharyngeal tumour- biopsy aged 13 ‘chronic inflamed polyp’

CT

Multiple surgical resections within one year including, tracheostomy and initial surgical resection, further surgical resection of bulbar mass and nasal tumour, then Le-Fort osteotomy and further surgical resection

ND

2. Maxillary sinus

3. Nasopharynx

2. Oropharynx

2. Chronic sinusitis

20

69 Y

F

Right Size: ND

1. Nasal cavity

ND

None stated

ND

Surgical excision

ND

2. Ethmoid sinus

(11) Low 2006 [33] UK

21

11 Y

M

Right Size: ND

1. Nasal cavity

1. Nasal Obstruction

None stated

CT

Surgical excision

No recurrence at 2 months

2. Epistaxis

(12) Johnson, 2007 [29] USA

22

15 Y

F

Bilateral Size: ND

1. Nasal cavity

1. Nasal obstruction

1. PPB

CT

Endoscopic surgical excision

No recurrence at 6 months

2. Nasopharynx

2. Chronic sinusitis

2. Sertoli-Leydig cell Ovarian Tumour

3. Congenital phthisi bulbi

(13) Silkiss, 2007 [18] USA

23

7 M

M

Right 3.2 × 1.4 cm

1. Nasal cavity

1. Ptosis

None stated

CT MRI

Surgical resection- right lateral rhinotomy

No recurrence at 18 months

2. Erosion of cribriform plate

2. Extropia

3. Compression of right orbit

3. Strabismus 4. Stertor

(14) Nakagawa 2008 [34] Japan

24

12 Y

M

Left 1.5 cm

1. Nasal cavity

1. Nasal obstruction

None stated

CT

Endoscopic surgical resection and further endoscopic surgical resection after recurrence

Recurrence at 2 months No recurrence at 5 months post second surgery

2. Sphenoid sinus

3. Ethmoid sinus

4. Maxillary sinus

(15) Finitsis, 2009 [35] Greece

25

12 M

M

Left 4 cm × 4.2 cm

1. Nasal cavity

1. Respiratory distress

None stated

CT MRI

Pre-operative embolization Then Surgical resection with midface de-gloving

ND

2. Compression of left orbit

3. Maxillary sinus compression

4. Nasopharynx

(16) Kim J, 2009 [23] Korea

26

19 M

M

Left 2.7 × 3.5 cm

1. Nasal cavity

1. Watery rhinorrhoea

None stated

CT MRI

Endoscopic surgical resection ×2

Recurrence at one year; 2nd surgery. No recurrence 10 months after second surgery

2. Orbital extension

2. Nasal Obstruction

3. Intracranial extension

(17) Priest, 2010 [27] USA *case previously reported by McDermot et al. 1998 **case previously reported by Johnson et al. 2007 (2 new cases)

-

7 Y *

M

Initially unilateral, then bilateral

1. Sphenoid sinus

1. Nasal Congestion

1. PPB type II–III

Four resections over 3 years

Followed up for 13 years with multiple recurrences in first 3 years

2. Left Nasal cavity

2. Nasal mass

2. Lung cysts

-

15 Y **

F

Bilateral

1. Bilateral nasal cavities

1. Chronic Sinusitis

1. PPB Type II

Surgical resection

No recurrence at 51 months

2. Bony erosion of posterior septum

2. Facial Pain

2. Sertoli-Leydig Cell Ovarian Tumour

2. Extending into nasopharynx

3. Nasal Congestion

3. Congenital phthisi bulbi Stickler syndrome

4. Nasal obstruction

27

10 Y

F

Bilateral Size: ND

1. Bilateral nasal cavities

1. Nasal obstruction

1. PPB Type III

CT

Surgical resection

No recurrence at 21 months

28

11 Y

M

Right Size: ND

1. Nasal cavity

1. Nasal obstruction

1. PPB Type III

ND

Surgical resection

No recurrence at 4 months

2. Extension to anterior skull base

(18) Sarin, 2010 [24] India

29

2.5 Y

M

Right Size: ND

1. Nasal cavity

1. Right eye oculomotor impairment

ND

MRI

Biopsy and then lateral rhinotomy for excision

ND

2. Maxillary, ethmoid and sphenoid sinus

3. Erosion of middle wall of orbit

(19) Eloy 2011 [25] Belgium

30

18 M*

M

Right 0.5 × 0.4 cm

1. Nasal cavity

1. Nasal obstruction

None stated

CT MRI

Endoscopic surgical resection

ND

2. Ethmoid sinus

2. Nasal mass

3. Extension into right orbit

3. Hypertelorism, proptosis, diplopia

4. Intracranial extension

4. Nasal swelling *symptoms 1st noticed at 2 months- delayed referral from Algeria to Brussels

(20) Jeyakumar 2011 [26] USA

31

7 D

F

Right Size: ND

1. Nasal cavity

1. Nasal Mass

None stated

CT MRI

Endoscopic surgical excision

ND

2. Stertor

3. Proptosis right eye

(21) Mattos 2011 [20] USA

32

3 Y

M

Left Size : ND

1. Nasal cavity

1. Eye infections

None stated

CT MRI

Endoscopic excision the further surgical excision

Recurrence at 21 months required further resection

2. Ethmoid sinus

2. Eye congestion

3. Extension into right orbit

3. Nasal obstruction

4. Left maxilla

4. Cheek fullness

5. Intermitted left eye/face pain

(22) Behery, 2012 [36] USA

33

11 Y

M

ND

ND

Nasal Obstruction

1. PPB

Surgical resection

ND

(23) Uzomefuna, 2012 [37] Ireland

34

8 Y

M

ND

1. Sphenoid sinus

1. Frontal Headache

ND

CT MRI

Endoscopic surgical resection

No recurrence at 6 months

2. Ethmoid sinus

(24) Cho, 2013 [4] Korea

35

14 Y

M

Left 5 cm × 5.3 cm × 4 cm

1. Nasal cavity

1. Swelling and pain to left face

None stated

CT

Subtotal maxillectomy, removal or orbital floor, removal of medial nasal mucous membrane. Reconstruction with iliac crest bone block

No recurrence at 4 years

2. Maxillary sinus

2. Tooth mobility

3. Intraoral

4. Orbital floor destruction

(25) Li Y, 2013 [12] China

36

40 Y

F

Bilateral Size: ND

1. Nasal Cavity

1. Nasal Obstruction

None stated

CT MRI

Complete radical resection

Recurrence at 3 months *Malignant transformation seen on histology

2. Maxillary sinus

2. Bloody rhinorrhoea

3. Ethmoid sinus

(26) Li GY 2013 [9] China

37

23 Y

M

Left 3.2 × 2.5 cm

1. Naval cavity

1. Left Lacrimal Sac

None stated

ND

Endoscopic surgical excision

No recurrence at 3 months follow up

2. Extension to lacrimal sac & left orbit

2. Proptosis

3. Ethmoid sinus

3. Lateral displacement of globe

(27) Moon, 2014 [19] Korea

38

9 M

F

Right Size: ND

1. Nasal cavity

1. Incomitant esotropia of right eye (inability to abduct right eye) No nasal symptoms

None stated

CT MRI

Surgery and care undertaken in another hospital

ND Surgery and care undertaken in another hospital

2. Maxillary sinus

3. Erosion of orbital wall

4. Erosion of cribriform plate

(28) Wang T, 2014 [38] China (2 cases)

39

5 Y

M

Right 2.5 × 3.6 × 4.3 cm

1. Nasal cavity

1. Recurrent sinusitis

None stated

CT MRI

Surgical resection

No recurrence at 3 years

2. Ethmoid sinus

2. Nasal Obstruction

3. Intracranial extension

40

6 W

F

Left 2.6 × 3.4 × 3.9 cm

1. Nasal cavity

1. Nasal obstruction

None stated

CT MRI

Surgical resection

No recurrence at 10 months

2. Pressure remodelling of adjacent bones

2. Watery rhinorrhoea

(29) Obidan, 2014 [39] Saudi Arabia

41

14 Y Size: ND

M

Bilateral

1. Bilateral nasal cavities

1. Nasal Obstruction

1. PPB

CT

Surgical endoscopic resection

ND

2. Decreased sense of smell

(30) Stewart, 2014 [13] USA **4 patients previously reported by Priest et al. 2010 [27] (4 new cases)

-

7 Y**

M

Initially unilateral, then bilateral

ND

1. Nasal Congestion

1. PPB

Surgical resection

Multiple recurrences

2. Lung cysts

-

15 Y**

F

Bilateral

ND

1. Chronic Sinusitis

1. PPB

Surgical resection

No recurrence

2. Facial Pain

2. Sertoli-Leydig Cell Ovarian Tumour

3. Nasal Congestion

-

10 Y**

F

Bilateral

ND

1. Nasal congestion

1. PPB

Surgical resection

No recurrence

-

11 Y**

M

Right

ND

1. Nasal Congestion

1. PPB

Surgical resection

No recurrence

42

8 Y

M

ND Size: ND

ND

1. ND

1. PPB

ND

Surgical resection

No recurrence

2. Pulmonary cysts in utero

3. Jejunal Polyps

43

13 Y

F

Bilateral Size: ND

ND

1. ND

1. PPB

ND

Surgical resection

No recurrence

2. Thyroid Papillary carcinoma

3. Sertoli-Leydig tumour

44

8 Y

M

Bilateral Size: ND

ND

1. Chronic Sinusitis

1. PPB

ND

Surgical resection

No recurrence

45

6 Y

F

ND Size: ND

ND

ND

1. PPB

ND

Surgical resection

No recurrence

2. Left cystic nephroma

3. Small bowel loop

46

21 Y

F

Right Size: ND

ND

1. Nasal Congestion

1. PPB

ND

Surgical resection

Recurrence at 4 years

2. Septal deviation

2. Sertoli-Leydig Tumour

3. Nasal Obstruction (at recurrence)

3. Multi-nodular goitre

(31) Chandra 2014 [40] India

47

12 Y

M

Right Size: ND

1. Nasal cavity

1. Nasal Obstruction

None stated

CT MRI

Surgical excision

No recurrence at 5 months

2. Ethmoid sinus

2. Proptosis

3. Extension into right orbit

3. Right facial pain

(32) Mason 2015 UK

48

49 Y

M

Right 0.5 × 2 × 2 cm

1. Nasal cavity

1. Nasal mass

None stated

None

Surgical excision

No recurrence at 4 years