Tag: Nasopharyngeal Angiofibroma

Nasopharyngeal Angiofibroma

Nasopharyngeal Angiofibroma


NASOPHARYNGEAL ANGIOFIBROMA

  • It is a histologically benign but locally aggressive vascular tumor that grows in the back of the nasal cavity.
  •  It is the most common benign neoplasm of nasopharynx.
  • It is a highly vascular tumor.It consists of fibrous tissue and blood vessels,
  • Blood supply of the tumor most commonly arises from the internal maxillary artery. 
  • The exact cause is unknown. As the tumour is predominantly seen in adolescent males in the second decade of life, it is thought to be testosterone dependent.
  • Such patients have a hamartomatous nidus of vascular tissue in the nasopharynx and this is activated to form angiofibroma when male sex hormone appears.
  • Most common site is posterior part of nasal cavity close to the margin of sphenopalatine foramen.
  • It may expand into the surrounding areas like nasal cavity,sphenoid sinus,pterygopalatine fossa,maxillary sinus,orbit and cranial cavity.

CLINICAL PRESENTATION OF NASOPHARYNGEAL ANGIOFIBROMA

  • Sex : Seen almost exclusively in males (testosterone dependent).
  • Age : 10-20 years (2nd decade).
  • Clinical features:   – Most common symptom is profuse and recurrent epistaxis Progressive nasal obstruction

Other features depending upon the extension of the tumour:

  • Denasal speech(Rhinolalai clausa)
  • Conductive hearing loss and serous otitis media due to eustachian tube blockage
  • Broadening of nasal bridge
  • Soft palate may bulge due to a large mass in late stages
  • Proptosis
  • Frog-face deformity
  • Swelling of cheek
  • Involvement of cranial nerves II, Ill, IV, VI

INVESTIGATIONS IN A CASE OF NASOPHARYNGEAL ANGIOFIBROMA

  • Radiography:it shows a soft tissue shadow in the nasopharynx.
  • Lateral view shows forward displacement of the posterior wall of maxilla.
  • Angiography and CT Scan  with contrast deomonstrate the extent and vascularity of the tumor.
  • Biopsy should never be attempted as it may lead to profuse bleeding.
  • Instead,Excision biopsy is performed.

TREATMENT OF NASOPHARYNGEAL ANGIOFIBROMA

Surgery

  • Surgical excision is the treatment of choice.
  • Before surgery at least 2-3 liters of blood should be given.
  • Preoperative embolization and estrogen therapy or cryotherapy reduce blood loss in surgery.
  • Hypotensive Anesthesia in nasopharyngeal angiofibroma is/are given by Propofol and Phentolamine.

Approach

  • Wilson’s Transpalatine approach—done for tumor confined to nasopharynx.
  • Lateral rhinotomy approach—done for large tumors involving, nasal cavity, paranasal sinuses and orbit. 

Other approaches:

  • Sardana’s approach – Transpalatine + Sublabial.
  • Extended Lateral Rhinotomy
  • Intracranial combined with Extracranial.
  • Infratemporal fossa approach.

Radiation

  • It is sometimes advised to reduce the vascularity of the tumor.
  • It may be considered in cases with intracranial extension.

Hormonal Therapy

  • It is doubtful if tumour regression occurs with this therapy.
Exam Question
 
  • Nasopharyngeal angiofibromas are most frequently seen in the second decade.
  • Hypotensive Anesthesia in nasopharyngeal angiofibroma is/are given by Propofol and Phentolamine.
  • Nasopharyngeal fibroma is the most common benign tumor of nasopharynx.
  • Clinical features of nasopharyngeal angiofibroma are Adolescent male and Epistaxis and nasal obstruction is the cardinal symptom.
  • A 14 years boy presented with repeated epistaxis, and a swelling in cheek.Diagnosis is nasopharyngeal angiofibroma and Contrast CT scan should be done to see the extent.
  • Surgery is treatment of choice in juvenile Nasopharyngeal Angiofibroma.
  • The most probable diagnosis in a 14-year old boy presenting with history of frequent nasal bleeding and Hb found to be 6.4 g/dL and peripheral smear showing normocytic hypochromic anemia is Juvenile nasopharyngeal angiofibroma.
  • Most probable diagnosis in a  child with unilateral nasal obstruction along with a mass in cheek and profuse and recurrent epistaxis is Nasopharyngeal Angiofibroma.
  • Hormonal etiology in implicated in Nasopharyngeal Angiofibroma.
  • Radiotherapy may be given in Nasopharyngeal Angiofibroma.
Don’t Forget to Solve all the previous Year Question asked on Nasopharyngeal Angiofibroma

Nasopharyngeal Angiofibroma

Nasopharyngeal Angiofibroma

Q. 1

A 15 year old boy presents with unilateral nasal blockage, mass in the cheek and epistaxis; likely diagnosis is:

 A

Nasopharyngeal ca

 B

Angiofibroma

 C

Inverted papilloma

 D

None of the above

Q. 1

A 15 year old boy presents with unilateral nasal blockage, mass in the cheek and epistaxis; likely diagnosis is:

 A

Nasopharyngeal ca

 B

Angiofibroma

 C

Inverted papilloma

 D

None of the above

Ans. B

Explanation:

Q. 2

A 14 year boy presented with unilateral nasal blockade, mass in the cheek and epistaxis. What is the likely diagnosis?

 A

Nasopharyngeal Ca

 B

Inverted papilloma

 C

Angiofibroma

 D

Thrombocytopenia

Q. 2

A 14 year boy presented with unilateral nasal blockade, mass in the cheek and epistaxis. What is the likely diagnosis?

 A

Nasopharyngeal Ca

 B

Inverted papilloma

 C

Angiofibroma

 D

Thrombocytopenia

Ans. C

Explanation:

Q. 3

Nasopharyngeal angiofibromas are most frequently seen –

 A

In the second decade

 B

In the third decade

 C

In the fourth

 D

In the fifth and sixth decades

Q. 3

Nasopharyngeal angiofibromas are most frequently seen –

 A

In the second decade

 B

In the third decade

 C

In the fourth

 D

In the fifth and sixth decades

Ans. A

Explanation:

Ans. is ‘a’ i.e., In the Second decade

o Nasopharyngeal angiofibroma is a highly vascular tumor that occurs almost exclusively in adolescent males.


Q. 4

Hypotensive Anesthesia in nasopharyngeal angiofibroma is/are given by:

 A

Propofol

 B

Phentolamine

 C

Halothane

 D

b and c

Q. 4

Hypotensive Anesthesia in nasopharyngeal angiofibroma is/are given by:

 A

Propofol

 B

Phentolamine

 C

Halothane

 D

b and c

Ans. D

Explanation:

B, C i.e. Phentolamine; Halothane

Hypotensive anesthesia used to facilitate vessel surgery such as clipping of intracranial aneurysm, excision of highly vascular tumor (eg nasopharyngeal angiofibroma)(2 is given by variable combinations of vasodialators (sodium nitroprusside, nitroglycrine), ganglion blocker (phentolamine, trimethaphan), inhalation agent (isoflurane, halothane) and /3-blockersQ.


Q. 5

A child with unilateral nasal obstruction along with a mass in cheek and profuse and recurrent epistaxis. Diagnosis is 

 A

Glomus tumor

 B

Antrochoanal polyp

 C

Juvenile nasal angiofibroma

 D

Rhinolith

Q. 5

A child with unilateral nasal obstruction along with a mass in cheek and profuse and recurrent epistaxis. Diagnosis is 

 A

Glomus tumor

 B

Antrochoanal polyp

 C

Juvenile nasal angiofibroma

 D

Rhinolith

Ans. C

Explanation:

Q. 6

Nasopharyngeal angiofibroma is:

 A

Benign

 B

Malignant

 C

Benign but potentially malignant

 D

None of the above

Q. 6

Nasopharyngeal angiofibroma is:

 A

Benign

 B

Malignant

 C

Benign but potentially malignant

 D

None of the above

Ans. A

Explanation:

 

  • Nasopharyngeal fibroma is the most common benign tumor of nasopharynx.
  • Most common site is posterior part of nasal cavity close to the margin of Sphenopalatine foramen.
  • Though it is a benign tumor, it is locally invasive and destroys the adjoining structures.
  • Juvenine Angiofibroma is uncommon, benign and extremely vascular tumor that arises in the tissues within the sphenopalatine foramen.

Q. 7

A 10 years child has unilateral nasal obstruction epistaxis, swelling over cheek, the diagnosis is:

 A

Nasal polyp

 B

Nasopharyngeal carcinoma

 C

Angiofibroma

 D

Foreign bodies

Q. 7

A 10 years child has unilateral nasal obstruction epistaxis, swelling over cheek, the diagnosis is:

 A

Nasal polyp

 B

Nasopharyngeal carcinoma

 C

Angiofibroma

 D

Foreign bodies

Ans. C

Explanation:

Q. 8

A 15-year-aged boy presents with unilateral nasal blockade mass in the cheek and epistaxis; likely diagnosis is:

 A

Nasopharyngeal Ca

 B

Angiofibroma

 C

Inverted papilloma

 D

None of the above

Q. 8

A 15-year-aged boy presents with unilateral nasal blockade mass in the cheek and epistaxis; likely diagnosis is:

 A

Nasopharyngeal Ca

 B

Angiofibroma

 C

Inverted papilloma

 D

None of the above

Ans. B

Explanation:

This is Typical Presentation of Nasopharyngeal Fibroma/Angiofibroma

  • Nasopharyngeal fibroma is most common benign tumor of nasopharynx.
  • Most common site is posterior part of nasal cavity close to the margin of sphenopalatine foramen.
  • Sex : Seen almost exclusively in males (testosterone dependent).
  • Age : 10-20 years (2nd decade).
  • Clinical features:   – Most common symptom is profuse and recurrent epistaxis Progressive nasal obstruction

Denasal speech

Conductive hearing loss and serous otitis media Mass in nasopharynx

Broadening of nasal bridge

Proptosis

Frog-face deformity

Swelling of cheek

Involvement of cranial nerves II, Ill, IV, VI


Q. 9

Clinical features of nasopharyngeal angiofibroma are:

 A

3rd to 4th decades

 B

Adolescent male

 C

Epistaxis and nasal obstruction is the cardinal symptom

 D

b and c

Q. 9

Clinical features of nasopharyngeal angiofibroma are:

 A

3rd to 4th decades

 B

Adolescent male

 C

Epistaxis and nasal obstruction is the cardinal symptom

 D

b and c

Ans. D

Explanation:

 

Nasopharyngeal Angiofibroma

Most commonly seen in adolescent males 

Most common age of presentation = second decade of life 

Arises from posterior nasal cavity close to sphenopalatine foramen 

Epistaxis and nasal obstruction are the most common presentation. 

Recurrent severe epistaxis accompanied by progressive nasal obstruction are the classical symptoms ofjuvenile angiofibromas at the time of presentation. – Scott-Brown 7th/ed Vol 2 p 2438

TOC is surgical excision 



Q. 10

A 14 years boy presented with repeated epistaxis, and a swelling in cheek. Which of these statements may be correct?

 A

Diagnosis is nasopharyngeal angiofibroma

 B

Contrast CT scan should be done to see the extent

 C

High propensity to spread via lymphatics

 D

a and b 

Q. 10

A 14 years boy presented with repeated epistaxis, and a swelling in cheek. Which of these statements may be correct?

 A

Diagnosis is nasopharyngeal angiofibroma

 B

Contrast CT scan should be done to see the extent

 C

High propensity to spread via lymphatics

 D

a and b 

Ans. D

Explanation:

Q. 11

True about juvenile nasopharyngeal angiofibroma:

 A

Surgery is treatment of choice

 B

It is malignant tumor

 C

Incidence in females

 D

Hormones not used in Rx

Q. 11

True about juvenile nasopharyngeal angiofibroma:

 A

Surgery is treatment of choice

 B

It is malignant tumor

 C

Incidence in females

 D

Hormones not used in Rx

Ans. A

Explanation:

Q. 12

True about nasopharyngeal angiofibroma: 

 A

Commonly seen in girls

 B

Hormonal etiology

 C

Surgery is treatment of choice, Radiotherapy can be given

 D

b and c

Q. 12

True about nasopharyngeal angiofibroma: 

 A

Commonly seen in girls

 B

Hormonal etiology

 C

Surgery is treatment of choice, Radiotherapy can be given

 D

b and c

Ans. D

Explanation:

Q. 13

A 9 years boy presents with nasal obstruction, proptosis, recurrent epistaxis from 3-4 years. Management includes:

 A

Routine radiological investigations

 B

Embolization alone should be done

 C

Surgery is treatment of choice

 D

a and c

Q. 13

A 9 years boy presents with nasal obstruction, proptosis, recurrent epistaxis from 3-4 years. Management includes:

 A

Routine radiological investigations

 B

Embolization alone should be done

 C

Surgery is treatment of choice

 D

a and c

Ans. D

Explanation:

 

  • Surgical excision is the treatment of choice.
  • Before surgery at least 2-3 liters of blood should be given.                                                                              
  • Preoperative embolization and estrogen therapy or cryotherapy reduce blood loss in surgery.

Approach

  • Transpalatine approach—done for tumor confined to nasopharynx.
  • Lateral rhinotomy approach—done for large tumors involving, nasal cavity, paranasal sinuses and orbit. 

Other Approaches

  • Sardana’s approach – Transpalatine + Sublabial.
  • Transhyoid and transmandibular approach.
  • Transzygomatic approach.

Q. 14

A 14-year old boy presents with history of frequent nasal bleeding. His Hb was found to be 6.4 g/dL and peripheral smear showed normocytic hypochromic anemia. The most probable diagnosis is:

 A

Juvenile nasopharyngeal angiofibroma

 B

Hemangioma

 C

Antrochonal polyp

 D

Carcinoma of nasopharynx

Q. 14

A 14-year old boy presents with history of frequent nasal bleeding. His Hb was found to be 6.4 g/dL and peripheral smear showed normocytic hypochromic anemia. The most probable diagnosis is:

 A

Juvenile nasopharyngeal angiofibroma

 B

Hemangioma

 C

Antrochonal polyp

 D

Carcinoma of nasopharynx

Ans. A

Explanation:

A 14-year-old boy presents with history of frequent nasal bleeding. His Hb was found to be 6.4 g/dL and peripheral smear showed normocytic hypochromic anemia. The most probable diagnosis is juvenile nasopharyngeal angiofibroma.

As the age of the patient (14 years), Sex: (male) and presentation (nasal bleeding) all favour it.

In antrochoanal polyps, the presenting symptom is U/L nasal obstruction and not bleeding.

Age of the patient goes against Nasopharyngeal cancer.

As far as hematoma are concerned, a swelling is generally seen.



Q. 15

A child with unilateral nasal obstruction along with a mass in cheek and profuse & recurrent epistaxis:

 A

Juvenile Nasal angiofibroma

 B

Glomus tumour

 C

Antrochoanal polyp

 D

Rhinolith

Q. 15

A child with unilateral nasal obstruction along with a mass in cheek and profuse & recurrent epistaxis:

 A

Juvenile Nasal angiofibroma

 B

Glomus tumour

 C

Antrochoanal polyp

 D

Rhinolith

Ans. A

Explanation:

 

Nasopharyngeal angiofibroma/ Juvenile nasopharyngeal angiofibroma

  • It is a histologically benign but locally aggressive vascular tumor that grows in the back of the nasal cavity.
  • It most commonly affects adolescent males.
  • Patients with nasopharyngeal angiofibroma usually present with one-sided nasal obstruction and recurrent bleeding.

Q. 16

A 15 year aged boy presents with unilateral nasal blockade,mass in the cheek and epistaxis, the  likely diagnosis is ‑

 A

Nasopharyngeal carcinoma

 B

Nasopharyngeal Angiofibroma

 C

Inverted papilloma

 D

None of the above

Q. 16

A 15 year aged boy presents with unilateral nasal blockade,mass in the cheek and epistaxis, the  likely diagnosis is ‑

 A

Nasopharyngeal carcinoma

 B

Nasopharyngeal Angiofibroma

 C

Inverted papilloma

 D

None of the above

Ans. B

Explanation:

 

Recurrent epistaxis, nasal obstruction and swelling over cheek in a 15 years boy suggest the diagnosis of nasopharyngeal angiofibroma.

Juvenile nasopharyngeal angiofibroma (JNA) is a benign, but locally aggresive, tumor of nasopharynx seen in prepubertal and adolescent males. It is the most common benign neoplasm of nasopharynx. It is a highly vascular tumor and blood supply of the tumor most commonly arises from the internal maxillary artery. Juvenile nasopharyngeal angiofibroma (JNA) occurs almost exclusively in males. Female with Juvenile nasopharyngeal angiofibroma (JNA) should undergo genetic testing. Onset is most commonly in the second decades, the range is 7-19 years.

The exact cause is unknown. As the tumour is predominantly seen in adolescent males in the second decade of life, it is thought to be testosterone dependent.

Such patients have a hamartomatous nidus of vascular tissue in the nasopharynx and this is activated to form angiofibroma when male sex hormone appears.



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