Diagnosis of Juvenile Angiofibroma by Computed Tomography 1
Computed Tomography
Meredith A. Weinstein, M.D., Howard Levine, M.D., Paul M. Duchesneau, M.D., and Harvey M. Tucker, M.D. Computedtomography (CT)accurately localized juvenile angiofibromata in 3 patients. The expanded pterygopalatine fossaand canal were visualizedby CT in all three cases.Because of the hemorrhagic tendency of thesetumors,a noninvasive modalitysuch as CT is especially valuable in planning therapy. INDEX TERMS: Computed tomography, cranial 1[0].1211 • (Nasopharynx, benign soft-tissue neoplasm, 2[6].360). Nasopharynx, neoplasms
Radiology 126:703-705, March 1978
TOMOGRAPHY (CT) has accurately localized juvenile angiofibromata in 3 patients. With this noninvasive technique, the location, expansion, erosion, and relationship of this tumor to surrounding structures suchas the pterygopalatine fossa and sphenoid sinus can be demonstrated. CT can be useful both in diagnosis and in planning the treatment of juvenile angiofibromata.
C
OMPUTED
MATERIALS AND METHODS
Three patients with subsequently proved juvenile angiofibroma were examined with an Ohio NuclearDelta 50 Fast Scanner. Two 13-mm sections were obtained simultaneously in 40 seconds. The maximumdosage at the back of the head was 16 rads. The dosageto the anterior aspect of the head and face was 4 rads. This represents a cumulative dose from eight adjacent 13~mm sections. Two patients were examined without the administration of contrast material. In one patient, 100 ml of Conray~400 was administered intravenously in a bolus injection. All the scans were obtained at 0 degrees angulation to the orbital meatal line.
Fig. 1. Normal nasal cavity and nasopharynx. Arrows (anterior to posterior) pointto nasalturbinate, pterygopalatine fossa, orifice of eustachiantube. torus tubarius,and fossa of RosenmOller.
vessels usually lack a completemuscular layerwhich may account for the lesion's well-known hemorrhagic tendencies (1). Thetumor becomes more fibrous with time and usually regresses after age 20 to 25 years. However, complete regression does not occur in all cases (3). Juvenile angiofibromata tend to extend along natural foraminaand fissures(8). Thetumor does not invade bone but often erodes it by pressure. It is not uncommon for the tumor to extend from the nasopharynx to the nasal fossa andnaresas well as to the maxillary, sphenoid and ethmoid sinuses. All of these are alr-contalninq structures in which soft-tissue masses can be accurately delineated by CT. Juvenile angiofibromata frequently extend into the pterygopalatinefossa and canal as well (6). Anterior bowing of the posterior wall of the maxillary antrum and posterior bowingof the anteriorwall of the pterygopalatine fossa and canal, well-recognized conventional roentgenographic signs of this lesion, are also well seen on CT (Figs. 2-4). The tumor may continue through the pterygopalatine fossa into the soft tissues of the pterygomaxilIary fossa. It may
DISCUSSION
Juvenile angiofibroma is the most common benign tumor of the nasopharynx (7). The tumor usually arises eccentrically from the roof of the anterior nasopharynx or from the posterior nasal fossa(5). Sixty-nine patients aged 7 to 32 years were studied by Jereb et al. (4); 80% were between 10 and 21 yearsof age (mean, 15 years). In 91% of these patientsnasal obstruction was the most common presenting complaint and was combined with spontaneous epistaxis in 59% . The sex incidence was 14 males and 1 female. Grossly the tumor is most often nodular and dark red. Compression of surrounding normal tissue produces a pseudocapsule (2). Histologically, the tumor is composed of connective tissue with dilatedblood vessels. The blood
1 From the Departments of Radiology(M.A.W., P.M.D.), and Otolaryngology and Communicative Disorders (H.L., H.M.T.), the ClevelandClinic Foundation and the Cleveland Clinic Educational Foundation, Cleveland, Ohio. Accepted for publication in October 1977. shan
703
704
MEREDITH
A.
WEINSTEIN AND OTHERS
March 1978
Fig. 2. A 15-year-old boy with intermittent epistaxis and obstructed right nostril. He was referred after biopsy. A. Tumor is in right side of nasopharynx, right side of posterior nasal fossa, and right maxillary sinus. The mid-portion of the posterior wall of the right maxillary sinus is thinned (arrow). B. Thirteen millimeters superior to A. Posterior wall of right maxillary sinus is displaced anteriorly (anterior arrow). The posterior wall of the pterygopalatine fossa is displaced posteriorly (posterior arrow). The tumor is in the right nasal cavity, right maxillary sinus and in the sphenoid sinus. C. Sphenoid sinus is filled with tumor (arrows).
also extend to the sella turcica and may involve the middle cranial fossa by extradural spread (8). The diagnosis of juvenile angiofibroma by noninvasive means is desirable because a biopsy may result in uncontrolled bleeding (3). Thus, CT recognition of juvenile angiofibroma is particularly useful. The attenuation coefficient of soft tissues, including juvenile angiofibroma, without contrast material is approximately 35 Hounsfield units. The attenuation coefficient of air is -1,000 Hounsfield units and for bone is approximately 1,000 Hounsfield units. This wide difference in the attenuation coefficient of the juvenile angiofibroma and the surrounding air and bone makes it possible to determine the precise anatomic location of the lesion in the nasopharynx and nasal fossa.
+
Soft-tissue masses in the sphenoid, ethmoid, and maxillary sinuses may also be accurately determined with CT. Administration of contrast material increases the attenuation coefficient of this vascular lesion, thus permitting the differentiation of tumor in the paranasal sinuses from increased density of these structures due to blockage. Contrast material is not required to determine the extent of juvenile angiofibroma into the orbits because of the difference in the attenuation coefficient of the fat in the retro-orbital space (-100 Hounsfield units) from the attenuation coefficient of the tumor (approximately 30 Hounsfield units). When juvenile angiofibroma extends through the pterygopalatine fossa into the soft tissue of the pterygomaxillary fossa, the attenuation coefficients of the
Fig. 3. A 15-year-old boy with intermittent epistaxis and obstructed right nostril. A. Entire nasopharynx, right maxlllary sinus, and all but the most anterior portion of the nasal fossa are filled with tumor. Posterior wall of the right maxillary sinus is displaced anteriorly. B.. Thirteen millimeters superior to A. Tumor has expanded the right pterygopalatine fossa and canal (arrows). C. Juvenile angiofibroma has expanded into the right side of the sphenoid sinus (arrow). Tumor did not involve the orbits.
Vol. 126
soft tissues and the tumor are virtually identical without contrast material. It is thus difficult to differentiate this lesion from the soft tissue and from brain tissue when it extends into the sella turcica or into the middle cranial fossa. Contrast material should be administered in these instances to try to differentiate the juvenile angiofibroma from the less vascular surrounding tissue. Differentiation may be difficult in these sites because slight motion will cause artifacts which decrease the accuracy of the CT numbers. One can also prognosticate possible extension of juvenile angiofibroma into the subtemporal fossa, the sella turcica, and the cranium by evaluating bone displacement, bone thinning, and bone destruction. In most instances, juvenile angiofibroma can be differentiated clinically from other benign nasopharyngeal tumors, such as ordinary fibroma and nasal polyp by the age, sex, symptoms, physical findings, and by the location and extent of the tumor. Widening of the pterygopalatine fossa and canal, present in all 3 of our cases, is highly suggestive of juvenile angiofibroma. If necessary, contrast material should aid in the differential diagnosis because of the great vascularity of juvenile angiofibromata. Malignant tumors have a greater tendency for bone destruction in this region rather than bone displacement. In some institutions, smaller juvenile angiofibromata are treated with surgery; larger tumors extending to adjacent structures such as the intracranial space are treated with radiotherapy. In such instances, CT can be used to help decide which treatment is appropriate. If surgery is contemplated, angiography with embolization to decrease intraoperative bleeding is helpful. Very large tumors can be removed in this manner with reduced blood loss. The use of CT has permitted deferring angiography in these cases until just before surgery, thus permitting embolization at optimal time. This will be the subject of a subsequent report. Meredith A. Weinstein, M.D. Department of Radiology Cleveland Clinic 9500 Euclid Avenue Cleveland, Ohio 44106
705
DIAGNOSIS OF JUVENILE ANGIOFIBROMA BY CT
Computed Tomography
Fig. 4. Nine-year-old boy with .progressive nasal obstruction on the left and recurrent nosebleeds. A. Tumor occupies left .sideof the nasopharynx and nasal fossa and extends across mid-line to the right. The posterior nasopharynx is spared. B. Juvenile angiotibroma occupies sphenoid sinus. The pterygopalatine fossa and canal are expanded (arrows).
REFERENCES 1. Briant TOR,FitzpatriCk PJ, Book H: The radiological treatment of juvenile nasopharyngeal angiofibromas. Ann Otol Rhinol Laryngol 79:1108-1113, Dec 1970 2. Fitzpatrick PJ, Rider WD: The radiotherapy of nasopharyngeal angiofibroma. Radiology 109: 171-178, Oct 1973 3. Holman CB, Miller WE: Juvenile nasopharyngeal fibroma; roentgenologic characteristics. Am J Roentgenol 94:292-298, Jun 1965 4. Jereb B, AnggArd A, BAryd I: Juvenile nasopharyngeal angiofibroma; a clinical study of 69 cases. Acta Radiol [Ther] 9:302-310, Aug 1970 5. Martin JS: Nasopharyngeal fibroma and its treatment. J Laryng Otol 68:39-54, Nov 1954 6. Potter GO: The pterygopalatine fossa and canal. Am J RoentgenoI107:520-525, Nov 1969 7. Rosen L, Hanafee W, Nahum A: Nasopharyngeal angiofibroma, an angiographic evaluation. Radiology 86:103-107, Jan 1966 8. Wilson GH, Hanafee WN: Angiographic findings in 16 patients with juvenile nasopharyngeal angiofibroma. Radiology 92:279-284, Feb 1969
Computed Tomography
Meredith A. Weinstein, M.D., Howard Levine, M.D., Paul M. Duchesneau, M.D., and Harvey M. Tucker, M.D. Computedtomography (CT)accurately localized juvenile angiofibromata in 3 patients. The expanded pterygopalatine fossaand canal were visualizedby CT in all three cases.Because of the hemorrhagic tendency of thesetumors,a noninvasive modalitysuch as CT is especially valuable in planning therapy. INDEX TERMS: Computed tomography, cranial 1[0].1211 • (Nasopharynx, benign soft-tissue neoplasm, 2[6].360). Nasopharynx, neoplasms
Radiology 126:703-705, March 1978
TOMOGRAPHY (CT) has accurately localized juvenile angiofibromata in 3 patients. With this noninvasive technique, the location, expansion, erosion, and relationship of this tumor to surrounding structures suchas the pterygopalatine fossa and sphenoid sinus can be demonstrated. CT can be useful both in diagnosis and in planning the treatment of juvenile angiofibromata.
C
OMPUTED
MATERIALS AND METHODS
Three patients with subsequently proved juvenile angiofibroma were examined with an Ohio NuclearDelta 50 Fast Scanner. Two 13-mm sections were obtained simultaneously in 40 seconds. The maximumdosage at the back of the head was 16 rads. The dosageto the anterior aspect of the head and face was 4 rads. This represents a cumulative dose from eight adjacent 13~mm sections. Two patients were examined without the administration of contrast material. In one patient, 100 ml of Conray~400 was administered intravenously in a bolus injection. All the scans were obtained at 0 degrees angulation to the orbital meatal line.
Fig. 1. Normal nasal cavity and nasopharynx. Arrows (anterior to posterior) pointto nasalturbinate, pterygopalatine fossa, orifice of eustachiantube. torus tubarius,and fossa of RosenmOller.
vessels usually lack a completemuscular layerwhich may account for the lesion's well-known hemorrhagic tendencies (1). Thetumor becomes more fibrous with time and usually regresses after age 20 to 25 years. However, complete regression does not occur in all cases (3). Juvenile angiofibromata tend to extend along natural foraminaand fissures(8). Thetumor does not invade bone but often erodes it by pressure. It is not uncommon for the tumor to extend from the nasopharynx to the nasal fossa andnaresas well as to the maxillary, sphenoid and ethmoid sinuses. All of these are alr-contalninq structures in which soft-tissue masses can be accurately delineated by CT. Juvenile angiofibromata frequently extend into the pterygopalatinefossa and canal as well (6). Anterior bowing of the posterior wall of the maxillary antrum and posterior bowingof the anteriorwall of the pterygopalatine fossa and canal, well-recognized conventional roentgenographic signs of this lesion, are also well seen on CT (Figs. 2-4). The tumor may continue through the pterygopalatine fossa into the soft tissues of the pterygomaxilIary fossa. It may
DISCUSSION
Juvenile angiofibroma is the most common benign tumor of the nasopharynx (7). The tumor usually arises eccentrically from the roof of the anterior nasopharynx or from the posterior nasal fossa(5). Sixty-nine patients aged 7 to 32 years were studied by Jereb et al. (4); 80% were between 10 and 21 yearsof age (mean, 15 years). In 91% of these patientsnasal obstruction was the most common presenting complaint and was combined with spontaneous epistaxis in 59% . The sex incidence was 14 males and 1 female. Grossly the tumor is most often nodular and dark red. Compression of surrounding normal tissue produces a pseudocapsule (2). Histologically, the tumor is composed of connective tissue with dilatedblood vessels. The blood
1 From the Departments of Radiology(M.A.W., P.M.D.), and Otolaryngology and Communicative Disorders (H.L., H.M.T.), the ClevelandClinic Foundation and the Cleveland Clinic Educational Foundation, Cleveland, Ohio. Accepted for publication in October 1977. shan
703
704
MEREDITH
A.
WEINSTEIN AND OTHERS
March 1978
Fig. 2. A 15-year-old boy with intermittent epistaxis and obstructed right nostril. He was referred after biopsy. A. Tumor is in right side of nasopharynx, right side of posterior nasal fossa, and right maxillary sinus. The mid-portion of the posterior wall of the right maxillary sinus is thinned (arrow). B. Thirteen millimeters superior to A. Posterior wall of right maxillary sinus is displaced anteriorly (anterior arrow). The posterior wall of the pterygopalatine fossa is displaced posteriorly (posterior arrow). The tumor is in the right nasal cavity, right maxillary sinus and in the sphenoid sinus. C. Sphenoid sinus is filled with tumor (arrows).
also extend to the sella turcica and may involve the middle cranial fossa by extradural spread (8). The diagnosis of juvenile angiofibroma by noninvasive means is desirable because a biopsy may result in uncontrolled bleeding (3). Thus, CT recognition of juvenile angiofibroma is particularly useful. The attenuation coefficient of soft tissues, including juvenile angiofibroma, without contrast material is approximately 35 Hounsfield units. The attenuation coefficient of air is -1,000 Hounsfield units and for bone is approximately 1,000 Hounsfield units. This wide difference in the attenuation coefficient of the juvenile angiofibroma and the surrounding air and bone makes it possible to determine the precise anatomic location of the lesion in the nasopharynx and nasal fossa.
+
Soft-tissue masses in the sphenoid, ethmoid, and maxillary sinuses may also be accurately determined with CT. Administration of contrast material increases the attenuation coefficient of this vascular lesion, thus permitting the differentiation of tumor in the paranasal sinuses from increased density of these structures due to blockage. Contrast material is not required to determine the extent of juvenile angiofibroma into the orbits because of the difference in the attenuation coefficient of the fat in the retro-orbital space (-100 Hounsfield units) from the attenuation coefficient of the tumor (approximately 30 Hounsfield units). When juvenile angiofibroma extends through the pterygopalatine fossa into the soft tissue of the pterygomaxillary fossa, the attenuation coefficients of the
Fig. 3. A 15-year-old boy with intermittent epistaxis and obstructed right nostril. A. Entire nasopharynx, right maxlllary sinus, and all but the most anterior portion of the nasal fossa are filled with tumor. Posterior wall of the right maxillary sinus is displaced anteriorly. B.. Thirteen millimeters superior to A. Tumor has expanded the right pterygopalatine fossa and canal (arrows). C. Juvenile angiofibroma has expanded into the right side of the sphenoid sinus (arrow). Tumor did not involve the orbits.
Vol. 126
soft tissues and the tumor are virtually identical without contrast material. It is thus difficult to differentiate this lesion from the soft tissue and from brain tissue when it extends into the sella turcica or into the middle cranial fossa. Contrast material should be administered in these instances to try to differentiate the juvenile angiofibroma from the less vascular surrounding tissue. Differentiation may be difficult in these sites because slight motion will cause artifacts which decrease the accuracy of the CT numbers. One can also prognosticate possible extension of juvenile angiofibroma into the subtemporal fossa, the sella turcica, and the cranium by evaluating bone displacement, bone thinning, and bone destruction. In most instances, juvenile angiofibroma can be differentiated clinically from other benign nasopharyngeal tumors, such as ordinary fibroma and nasal polyp by the age, sex, symptoms, physical findings, and by the location and extent of the tumor. Widening of the pterygopalatine fossa and canal, present in all 3 of our cases, is highly suggestive of juvenile angiofibroma. If necessary, contrast material should aid in the differential diagnosis because of the great vascularity of juvenile angiofibromata. Malignant tumors have a greater tendency for bone destruction in this region rather than bone displacement. In some institutions, smaller juvenile angiofibromata are treated with surgery; larger tumors extending to adjacent structures such as the intracranial space are treated with radiotherapy. In such instances, CT can be used to help decide which treatment is appropriate. If surgery is contemplated, angiography with embolization to decrease intraoperative bleeding is helpful. Very large tumors can be removed in this manner with reduced blood loss. The use of CT has permitted deferring angiography in these cases until just before surgery, thus permitting embolization at optimal time. This will be the subject of a subsequent report. Meredith A. Weinstein, M.D. Department of Radiology Cleveland Clinic 9500 Euclid Avenue Cleveland, Ohio 44106
705
DIAGNOSIS OF JUVENILE ANGIOFIBROMA BY CT
Computed Tomography
Fig. 4. Nine-year-old boy with .progressive nasal obstruction on the left and recurrent nosebleeds. A. Tumor occupies left .sideof the nasopharynx and nasal fossa and extends across mid-line to the right. The posterior nasopharynx is spared. B. Juvenile angiotibroma occupies sphenoid sinus. The pterygopalatine fossa and canal are expanded (arrows).
REFERENCES 1. Briant TOR,FitzpatriCk PJ, Book H: The radiological treatment of juvenile nasopharyngeal angiofibromas. Ann Otol Rhinol Laryngol 79:1108-1113, Dec 1970 2. Fitzpatrick PJ, Rider WD: The radiotherapy of nasopharyngeal angiofibroma. Radiology 109: 171-178, Oct 1973 3. Holman CB, Miller WE: Juvenile nasopharyngeal fibroma; roentgenologic characteristics. Am J Roentgenol 94:292-298, Jun 1965 4. Jereb B, AnggArd A, BAryd I: Juvenile nasopharyngeal angiofibroma; a clinical study of 69 cases. Acta Radiol [Ther] 9:302-310, Aug 1970 5. Martin JS: Nasopharyngeal fibroma and its treatment. J Laryng Otol 68:39-54, Nov 1954 6. Potter GO: The pterygopalatine fossa and canal. Am J RoentgenoI107:520-525, Nov 1969 7. Rosen L, Hanafee W, Nahum A: Nasopharyngeal angiofibroma, an angiographic evaluation. Radiology 86:103-107, Jan 1966 8. Wilson GH, Hanafee WN: Angiographic findings in 16 patients with juvenile nasopharyngeal angiofibroma. Radiology 92:279-284, Feb 1969
