Cardiac Output, Venous Return, and Their Regulation
Angiofibroma
1.
2. Introduction
Uncommon, benign and extremely vascular tumour
Up to 0.5% of head and neck tumours
Occurring almost exclusively in males
Average age of onset - 15 years
Intracranial Extension is between 10-20%
Recurrence Rates as high as 50%
3. SITE OF ORIGIN:
Posterior part of the nasal cavity close to the superior margin of
sphenopalatine foramen.
4.
5. Extension:
Locally invasive
Spread – submucosally
Rather than invading surrounding tissue, this tumour displaces
and distorts, relying on pressure necrosis to destroy and
push through its bony confines.
6. Extends into-
Nasal cavity causing nasal obstruction, epistaxis & nasal obstruction.
Paranasal sinuses
Laterally tumor extends from into the pterygomaxillary fossa and hence to
infratemporal fossa and cheek.
Orbits – proptosis & Frog face deformity.
Cranial cavity- into the Anterior cranial fossa & Middle cranial fossa
7.
8. Theory Of Origin:
The most accepted theory is that JNAs originate from sex steroid – stimulated
hamartomatous tissue located in the turbinate cartilage.
The proposed hormonal influence explains why some JNAs involute after
puberty
Patients have hamartomatous nidus of vascular tissue which get activated to
form angiofibroma when male sex hormone is released.
9. Other theories-
Ringertz theory- JNA always arose from the periosteum of the skull base .
Som and Neffson theory- inequalities in the growth of bones forming
skul base resulted in hypertrophy of the underlying periosteum in
response to hormonal influence.
Bensch and Ewing theory- tumour probably arose from embryonic fibro
cartilage between basioccipital and basi-sphenoid.
Brunner theory- suggested origin from conjoined pharyngobasilar and
buccopharyngeal fascia.
10. Marten et al theory-proposed a hormonal theory suggesting that these tumours
resulted from the deficiency of androgen or overactivity of estrogens and that the
hormonal stimulation for angiomatous seen in JNS.
Sternberg- proposed that JNA could be a type of hemangioma like a cutaneous
hemangioma seen in a child which regress with age.
Osborn-it could be due to either a hamartoma or residual fetal erectile
tissue which were subjected to hormonal influence.
11. Tumour blood vessels typically lack smooth muscle and elastic fibers, this is
the reason for sustained bleeding.
Recent immunocytochemical techniques shown that androgen receptors are
present in at least 75 percent of tumours, these receptors are present in both
the vascular and stromal elements.
Angiogenic growth factor (vascular endothelial growth factor (VEGF))
Sporadic juvenile angiofibromas develop 25 times more frequently in patients
with familial adenomatous polyposis (FAP).
AR, VEGF,
beta
catenin ?
12. Macroscopy
Well-defined, spongy lobulated tumours with nodules covered by
nasopharyngeal mucosa (squamous epithelium).
Nodularity increases with age.
Color varies from pink (part seen in nasopharynx) to white or grey
(extrapharyngeal areas).
13. Histology
Fibrous connective tissues interspersed with variable proportion of
endothelium lined blood spaces.
Fibrous tissue increases towards periphery and vascular element tends to be
more central.
14. Clinical features
Profuse, recurrent & spontaneous epistaxis.
Progressive nasal obstruction and hyponasal voice.
Conductive hearing loss and middle ear effusion.
Chronic anemia may be present due to repeated epistaxis.
Complete nasal obstruction may cause stasis of secretions and may also lead
to sepsis.
Patients may have hyposmia or anosmia.
15. Extension of tumor in different directions produces
symptoms like:
facial swelling
Proptosis
Diplopia
Broadening of nasal bridge
Palatal buldge
Cranial nerve palsies.
16. On Anterior rhinoscopy –
Abundant purulent nasal secretions.
Bowing of nasal septum to uninvolved side.
On Posterior rhinoscopy –
Pink or red mass filling the nasopharynx can be seen.
17.
18. Complications:
Recurrence:
most common complication encountered.
reported in up to 25 percent of patients regardless of the method of
treatment.
The more younger the patient, the more likely that future recurrence will
develop.
19. Ocular problems –
Displacement of the globe caused by loss of bony support
Ophthalmoplegia
Visual loss may also be present.
Surgically induced infraorbital nerve sensory deficit.
Complication of mid facial degloving as in Nasal vestibular stenosis
In transpalatal approach fistula of palate at junction of soft and hard palate.
Prolonged Nasal crusting is also common and may develop into ozaena.
20. Late complications that may develop after
radiotherapy:
Growth retardation
Panhypopituitarism
Temporal lobe necrosis
Cataract
Radiation keratopathy
Skin, thyroid and nasopharyngeal malignancies
21. INVESTIGATION:
CT scan of Nose and PNS-
Investigation of choice
Extent / vascularity of tumour
Holman Miller sign ( Anterior bowing of posterior wall of maxillary antrum)
Bone erosion / Widening of sphenopalatine foramen
23. Magnetic resonance imaging (MRI):
to see the extension of the soft tissue tumour into the cranium, orbit and
infra-temporal fossa.
24. ANGIOGRAPHY:
Diagnostic angiography is performed to identify the extend of tumour, its
vascularity , feeder vessels and to embolise it pre-operatively to shrink the
tumour and reduce bleeding during surgery.
Resection of tumour should not be delayed beyond 24-48 h of embolization to
avoid revascularization from contralateral side.
25. It is contraindicated in suspected cases of angiofibroma
because it will cause profuse bleeding (as the muscular
coat of the vessel is absent).
Plain x-rays of the nasopharynx (lateral view) and paranasal sinuses
(occipito-mental view) will show the presence of soft tissues mass.
BIOPSY?
27. Staging system-
Staging is done for prognosis and for therapeutic approaches.
There are four types of staging.
I. Fisch staging
II. Billers staging
III. Andrews staging
IV. Radkowski staging
32. Treatment:
Surgery
Gold standard
Radiotherapy
Reserved for unresectable tumor, intracranial
extension , recurrent cases
Chemotherapy
Recurrent tumors with previous surgery and
radiation
Hormone therapy
To reduce vascularity before surgery
33. Surgical Approaches:
1. Transpalatine
2. Transpalatine + Sublabial (Sardana’s approach)
3. Lateral rhinotomy with medial maxillectomy
(a) Via facial incision
(b) Via degloving approach
4. Endoscopic removal
5. Transmaxillary (Le Fort I) approach
6. Maxillary swing approach or facial translocation
approach, or Wei’s operation
7. Infratemporal fossa approach
8. Intracranial–extracranial approach
34. Preopt. reduction of tumor vascularity:
Embolization of feeding arteries:
24 to 72 hours pre operative
Gelfoam (resorbed in approximately 2 weeks)
Polyvinyl alcohol foam (more permanent )
Estrogen Therapy:
Diethylstilbestrol 2.5 mg PO TDS for 3-6 weeks (Cellular contraction, increase in
collagen and fibroblasts decreases bleeding, reduces size)
Testosterone Receptor blocker
Flutamide
Radiotherapy
Proton stereotactic RT
Cryotherapy
35. Intranasal endoscopic
Approach
Newer technique
Small tumour in nose , PNS , nasopharynx, pterygopalatine fossa
Larger tumours and those extending across or through the skull base are
difficult to remove through this technique.
36.
37. Procedure:
Preoperative embolization is undertaken.
After the induction of anaesthesia, the nose is prepared with a
vasoconstrictor solution (4% cocaine or epinephrine 1:10,000).
The anterior end of the middle turbinate is resected.
38. An anterior ethmoidectomy together with removal of the medial wall of the
maxillary sinus is done.
Access to the posterior wall of the antrum is gained.
This wall is then removed to achieve complete lateral exposure of the
tumour.
39. Dissection is then continued into the sphenoid until its rostrum is reached.
Tumour is peeled off inferiorly.
40. A similar technique can be used to deliver the lateral extension of the tumour
into the operative field.
41. Transpalatal Approach (Wilson):
used for tumours confined to the nasopharynx.
can be extended into Sardana’s approach if the tumour extends laterally.
Wide access to pterygomaxillary fossa can also be obtained by removing
the anterior wall of maxillary sinus along with parts of pyriform aperture
of nose through osteotomies and later reconstruction at the end of
operation with plates.
42. Postoperative Care
Intense humidification to avoid crusting and drying of clots in the nasal cavity.
Oral irrigations are started on the first postoperative day, and the patient is
allowed to take liquids by mouth approximately 48 hours after surgery.
Irrigations are continued until full epithelialization of the mucosa in the raw
areas in the nasopharynx occurs.
Postoperative aesthetic and functional results of this surgical procedure are
excellent with no disability in swallowing, speech, or breathing.
43. Transmaxillary Le Fort I approach:
used to give a wider access to remove tumours which extend into maxillary
and ethmoid sinuses and pterygopalatine fossa.
Facial translocation or Maxillary swing
approach:
For tumours of infratemporal fossa
Here an osteoplastic flap with entire cheek and maxilla is raised as a single
unit, which is later reconstructed.
44. MEDIAL MAXILLECTOMY
APPROACH
Larger angiofibromas extending from the nasal cavity and nasopharynx into
the maxillary, ethmoid, or sphenoid sinuses require a wider exposure through
a modified Weber-Ferguson incision or via a sublabial degloving approach.
(a) Via facial incision
(b) Via degloving approach
46. The incision can be modified with Lynch or subciliary extensions for larger
tumors with significant superior or lateral extension.
47. Midfacial degloving:
A sublabial degloving approach is suitable for larger tumors involving
anteroinferior aspect of the nasal cavity and the infrastructure of the
maxillary sinus, and particularly when access to the posterosuperior part of
the nasal cavity is not satisfactory through other approaches.
Gingivo buccal incision is given.
48. Radiotherapy
It has been used as a primary mode of treatment.
A dose of 3000 to 3500 cGy in 15-18 fractions is delivered in 3 weeks.
Tumour regresses slowly in about a year sometimes even up to 3 years.
Also used for intracranial extension of disease when tumour derives its blood
supply from ICA.
Intensity modulated radiotherapy for recurrent angiofibromas.
Controversial.
49. Chemotherapy
Recurrent and residual lesions have been treated by chemotherapy.
Combination used:
doxorubicin
Vincristine
Dacarbazine
HORMONAL:
Diethylstilboestrol and flutamide have been used as tumour occurs in males at
puberty.
Editor's Notes
It is a rare tumor, though it is the commonest of all
the benign tumors of nasopharynx
The vessels are just endothelium lined spaces
with no muscle coat therefore severe bleeding
may occur on taking biopsy and surgical removal
as these vessels can not contract to stop
bleeding.
