Ann Otol 87 :1978
JUVENILE NASOPHARYNGEAL ANGIOFIBROMA HUGH
F.
BILLER,
MD
NEW YORK, NEW YORK
The diagnosis of angiofibroma can be suspected in most cases by clinical examination, supplemented by routine x-rays and tomograms and is confinned by angiography. Adjunctive therapy including embolization, estrogens, cryotherapy and arterial ligation is discussed. Surgical resection is the preferred treatment. Radiotherapy as the primary treatment modality is contraindicated except in select cases.
Juvenile nasopharyngeal angiofibroma, a histologically benign, but at times, a clinically malignant vascular tumor of adolescent males, continues to challenge the head and neck surgeon. The improved surgical curability of this lesion over the past 10 to 15 years has been primarily related to preoperative angiegraphic tumor assessment and the selection of surgical approaches dictated by the extent of the tumor. At the present time there remains controversy related to the following: diagnostic procedures, adjunctive surgical therapy and the role of radiotherapy in the treatment of these tumors. This presentation will be limited to these controversial areas. DIAGNOSIS
The experienced pathologist is able to diagnose angiofibroma accurately, on biopsy, in practically all cases. There are no contra indications to biopsy, provided the surgeon recognizes that the lesion may bleed profusely. This usually requires nasal packing and, occasionally, transfusion. Anticipation of the diagnosis, therefore, requires biopsy in the operating room usually under general anesthesia. The alternative method to biopsy, for diagnosis, is arteriography. The arteriographic picture is diagnostic for angiofibroma. The arteriogram is also necessary to define the vascular supply and the extent of the tumor. The latter can be determined by computerized axial tomography (CAT) but, unfortu-
nately, the blood supply to the tumor is not identified by this method. If arteriography is employed as a diagnostic method then occasionally patients presenting with nasopharyngeal masses which are not angiofibromas may undergo unnecessary arteriography. In the present series of ten cases, three patients were biopsied for diagnosis. These three patients, clinically, had a preoperative diagnosis of angiofibroma. All patients required general anesthesia for biopsy and postoperative packing. One required a transfusion. Four patients with nasopharyngeal masses, suspected of being angiofibroma by the referring physician following physical examination, routine sinus x-rays, and tomograms were thought clinically to have lesions other than angiofibromas. These patients did not have arteriograms but underwent biopsy. The results were antrochoanal polyp in three patients and lymphoma in one patient. It is evident that with accurate clinical assessment supplemented by routine sinus x-rays and, at times, CAT scans and tomograms, the clinical diagnosis of angiofibroma is very accurate. The number of unnecessary arteriograms will be very few. At the present time arteriography in most institutions has less morbidity than biopsy. Therefore, arteriography is used for diagnosis. Biopsy for diagnosis in most institutions should be limited to those lesions which, clinically, are felt not to represent angiofibroma. In those
From the Department of Otolaryngology, Mount Sinal Medical Center, New York, New York. Presented at the meeting of the American Laryngological Association, Palm Beach, Florida, April 22-23, 1978.
630
Downloaded from aor.sagepub.com at Bobst Library, New York University on June 16, 2015
JUVENILE NASOPHARYNGEAL ANGIOFIBROMA
hospitals where arteriography is not performed or the experience is limited, biopsy would be indicated for diagnosis. ADJUNCTS TO SURGICAL REMOVAL
Adjunctive procedures are used to decrease blood loss at the time of surgical resection. Cryosurgery, estrogens, embolization, artery ligation and preoperative radiotherapy have been used as adjunctive measures. Cryotherapy has not been effective in our experience. Only small lesions limited to the nasopharynx can be frozen sufficiently to reduce bleeding. These small tumors can be removed with minimal blood loss without cryotherapy. Estrogen therapy has been advocated to decrease tumor size and vascularity prior to surgery. Schiff- and Walike and Mackay" demonstrated increased collagen tissue and reduced vascularity on comparing preoperative biopsy with postoperative specimens in patients treated with estrogens. Histological evaluation is not a reliable indication of decreased vascularity since sections from the same tumor specimen may show variable histologic vascular patterns. Christianson et al" noticed decreased tumor size, clinically, in five of seven cases treated with estrogens. In three of the five patients, pre and postestrogen arteriograms were reported as demonstrating decreased vascularity. Duckert et al 4 reported evidence of decreased tumor size as measured on CAT scan following estrogen therapy. Not all authors agree that estrogen will demonstrably reduce blood loss at the time of surgery. A recent patient with a recurrent angiofibroma with significant internal carotid artery blood supply received six weeks of diethylstilbestrol (15 mg/ day). Comparison of pre and postestrogen CAT scans showed resolution of thickened inflammatory sinus and nasal mucosa and only slight decrease in the size of the tumor. Pre and postestrogen arteriograms demonstrated no change in the vascular pattern. In our experience, estrogens have had no clinical efficacy in reducing blood loss. Embolization as an adjunct has con-
631
sistently decreased vascularity of the tumors arteriographically and clinically. The size of the tumor does not change significantly. Embolization may be performed a few days prior to surgery or at the time of surgery. Gelfoam® or beads may be used. Experience is required to avoid reflux of emboli into the internal carotid artery when percutaneous catheterization is used. This risk, though small, has deterred the author from using the percutaneous technique. Embolization is reserved for large lesions which have both internal and external carotid blood supply or tumors which have intracranial extension that are deemed resectable. Embolization in these cases is performed at the time of surgery with direct external carotid exposure and proximal external carotid ligation, thereby avoiding internal carotid emboli. Transantral internal maxillary ligation is performed routinely for all other cases. Clinically, the amount of bleeding with embolization is less than with trans antral ligation. The rationale for not performing embolization routinely as described above, is related to the necessity of external carotid artery ligation with resultant inability to perform subsequent arteriography for recurrent tumors. Preoperative radiotherapy will diminish vascularity and frequently tumor size but is not used because of the potential sequelae in later years. RADIATION THERAPY
Briant et a15 advocate radiotherapy as the primary treatment of angiofibroma. This is an aggressive, radical approach for a benign lesion in young patients. Though sufficient time has not elapsed to determine the morbidity related to radiotherapy in the treatment of angiofibroma, there is sufficient evidence from observation of other benign conditions treated by radiotherapy that when alternative treatment is available, radiotherapy is contraindicated for benign tumors in young patients. The rationale for radiotherapy is based on the poor results with surgery. Briant et al state that the results of successful angiofibroma surgery at Princess Margaret
Downloaded from aor.sagepub.com at Bobst Library, New York University on June 16, 2015
632
HUGH F. BILLER
Hospital were 1 case in 19. It is unfortunate that the results of radiotherapy are compared to surgical results of earlier years when transoral avulsion or piecemeal removal of the tumor were the procedures usually employed. It is evident that in the past ten years with improved definition of tumor extent, determination of blood supply and appropriate surgical exposure, the results with surgery for extracranial disease are excellent. Christianson et al" report 79% cure with surgery, Boles and Ded0 6 78%, Biller" 72%, Jafek et al" 90% cure with surgery. In the present series nine of ten patients were cured. With second procedures on those primary failures the percentage is increased to over 90%. It is our feeling that radiotherapy is contraindicated as a primary treatment modality for angiofibromas which do not demonstrate in-
tracranial extension or extensive internal carotid blood supply. In most series intracranial extension represents approximately 5% to 10%. Radiotherapy is indicated in some patients who have intracranial extension. This is true if the intracranial tumor has significant blood flow from the internal carotid artery. Patients who have intracranial extension with minimal or no internal carotid artery supply, can be operated upon. The extracranial portion of the tumor is removed and the intracranial portion is followed postoperatively by CAT scans or arteriography. Postoperatively, if there is evidence of increasing intracranial size, then radiotherapy is indicated. Radiotherapy, therefore, is reserved for only a few select cases. In our experience it has been used in 1 of 12 cases, or 8%.
REFERENCES 1. Schiff M: Juvenile nasopharyngeal angiofibroma. Laryngoscope 69:981-1016, 1959 2. Walike 1. Mackay B: Management of nasopharyngeal angiofibroma. Trans Am Acad Dphthalmol Otolaryngol 76:1346-1353, 1972 3. Christiansen TA, Duvall AJ III, Rosenberg Z, et al: Juvenile nasopharyngeal angiofibroma. Trans Am Acad Ophthalmol Otolaryngol 78:140-147, 1974 4. Duckert L, Carley R, Hilger J: Computerized axial tomography in the preoperative evaluation of an angiofibroma. Laryngoscope 88:613-618, 1978
5. Briant TD, Fitzpatrick PJ, Bock H: The radiological treatment of juvenile nasopharyngeal angiofibromas. Ann Otol Rhinol Laryngol 79:1108-1113, 1970 6. Boles R, Dedo H: Nasopharyngeal angiofibroma. Laryngoscope 86:364-372, 1976 7. Biller HF, Sessions D, Ogura J: Angiofibroma: a treatment approach. Laryngoscope 84:695-706, 1974 8. [afek BW, Nahum AM, Butler RM, et al: Surgical treatment of juvenile nasopharyngeal angiofibroma. Laryngoscope 83:707-729, 1973
REPRINTS Hugh F. Biller, MD, Department of Otolaryngology, The Mount Sinai Medical Center, One Gustave L. Levy Place, New York, NY 10029.
Downloaded from aor.sagepub.com at Bobst Library, New York University on June 16, 2015
JUVENILE NASOPHARYNGEAL ANGIOFIBROMA HUGH
F.
BILLER,
MD
NEW YORK, NEW YORK
The diagnosis of angiofibroma can be suspected in most cases by clinical examination, supplemented by routine x-rays and tomograms and is confinned by angiography. Adjunctive therapy including embolization, estrogens, cryotherapy and arterial ligation is discussed. Surgical resection is the preferred treatment. Radiotherapy as the primary treatment modality is contraindicated except in select cases.
Juvenile nasopharyngeal angiofibroma, a histologically benign, but at times, a clinically malignant vascular tumor of adolescent males, continues to challenge the head and neck surgeon. The improved surgical curability of this lesion over the past 10 to 15 years has been primarily related to preoperative angiegraphic tumor assessment and the selection of surgical approaches dictated by the extent of the tumor. At the present time there remains controversy related to the following: diagnostic procedures, adjunctive surgical therapy and the role of radiotherapy in the treatment of these tumors. This presentation will be limited to these controversial areas. DIAGNOSIS
The experienced pathologist is able to diagnose angiofibroma accurately, on biopsy, in practically all cases. There are no contra indications to biopsy, provided the surgeon recognizes that the lesion may bleed profusely. This usually requires nasal packing and, occasionally, transfusion. Anticipation of the diagnosis, therefore, requires biopsy in the operating room usually under general anesthesia. The alternative method to biopsy, for diagnosis, is arteriography. The arteriographic picture is diagnostic for angiofibroma. The arteriogram is also necessary to define the vascular supply and the extent of the tumor. The latter can be determined by computerized axial tomography (CAT) but, unfortu-
nately, the blood supply to the tumor is not identified by this method. If arteriography is employed as a diagnostic method then occasionally patients presenting with nasopharyngeal masses which are not angiofibromas may undergo unnecessary arteriography. In the present series of ten cases, three patients were biopsied for diagnosis. These three patients, clinically, had a preoperative diagnosis of angiofibroma. All patients required general anesthesia for biopsy and postoperative packing. One required a transfusion. Four patients with nasopharyngeal masses, suspected of being angiofibroma by the referring physician following physical examination, routine sinus x-rays, and tomograms were thought clinically to have lesions other than angiofibromas. These patients did not have arteriograms but underwent biopsy. The results were antrochoanal polyp in three patients and lymphoma in one patient. It is evident that with accurate clinical assessment supplemented by routine sinus x-rays and, at times, CAT scans and tomograms, the clinical diagnosis of angiofibroma is very accurate. The number of unnecessary arteriograms will be very few. At the present time arteriography in most institutions has less morbidity than biopsy. Therefore, arteriography is used for diagnosis. Biopsy for diagnosis in most institutions should be limited to those lesions which, clinically, are felt not to represent angiofibroma. In those
From the Department of Otolaryngology, Mount Sinal Medical Center, New York, New York. Presented at the meeting of the American Laryngological Association, Palm Beach, Florida, April 22-23, 1978.
630
Downloaded from aor.sagepub.com at Bobst Library, New York University on June 16, 2015
JUVENILE NASOPHARYNGEAL ANGIOFIBROMA
hospitals where arteriography is not performed or the experience is limited, biopsy would be indicated for diagnosis. ADJUNCTS TO SURGICAL REMOVAL
Adjunctive procedures are used to decrease blood loss at the time of surgical resection. Cryosurgery, estrogens, embolization, artery ligation and preoperative radiotherapy have been used as adjunctive measures. Cryotherapy has not been effective in our experience. Only small lesions limited to the nasopharynx can be frozen sufficiently to reduce bleeding. These small tumors can be removed with minimal blood loss without cryotherapy. Estrogen therapy has been advocated to decrease tumor size and vascularity prior to surgery. Schiff- and Walike and Mackay" demonstrated increased collagen tissue and reduced vascularity on comparing preoperative biopsy with postoperative specimens in patients treated with estrogens. Histological evaluation is not a reliable indication of decreased vascularity since sections from the same tumor specimen may show variable histologic vascular patterns. Christianson et al" noticed decreased tumor size, clinically, in five of seven cases treated with estrogens. In three of the five patients, pre and postestrogen arteriograms were reported as demonstrating decreased vascularity. Duckert et al 4 reported evidence of decreased tumor size as measured on CAT scan following estrogen therapy. Not all authors agree that estrogen will demonstrably reduce blood loss at the time of surgery. A recent patient with a recurrent angiofibroma with significant internal carotid artery blood supply received six weeks of diethylstilbestrol (15 mg/ day). Comparison of pre and postestrogen CAT scans showed resolution of thickened inflammatory sinus and nasal mucosa and only slight decrease in the size of the tumor. Pre and postestrogen arteriograms demonstrated no change in the vascular pattern. In our experience, estrogens have had no clinical efficacy in reducing blood loss. Embolization as an adjunct has con-
631
sistently decreased vascularity of the tumors arteriographically and clinically. The size of the tumor does not change significantly. Embolization may be performed a few days prior to surgery or at the time of surgery. Gelfoam® or beads may be used. Experience is required to avoid reflux of emboli into the internal carotid artery when percutaneous catheterization is used. This risk, though small, has deterred the author from using the percutaneous technique. Embolization is reserved for large lesions which have both internal and external carotid blood supply or tumors which have intracranial extension that are deemed resectable. Embolization in these cases is performed at the time of surgery with direct external carotid exposure and proximal external carotid ligation, thereby avoiding internal carotid emboli. Transantral internal maxillary ligation is performed routinely for all other cases. Clinically, the amount of bleeding with embolization is less than with trans antral ligation. The rationale for not performing embolization routinely as described above, is related to the necessity of external carotid artery ligation with resultant inability to perform subsequent arteriography for recurrent tumors. Preoperative radiotherapy will diminish vascularity and frequently tumor size but is not used because of the potential sequelae in later years. RADIATION THERAPY
Briant et a15 advocate radiotherapy as the primary treatment of angiofibroma. This is an aggressive, radical approach for a benign lesion in young patients. Though sufficient time has not elapsed to determine the morbidity related to radiotherapy in the treatment of angiofibroma, there is sufficient evidence from observation of other benign conditions treated by radiotherapy that when alternative treatment is available, radiotherapy is contraindicated for benign tumors in young patients. The rationale for radiotherapy is based on the poor results with surgery. Briant et al state that the results of successful angiofibroma surgery at Princess Margaret
Downloaded from aor.sagepub.com at Bobst Library, New York University on June 16, 2015
632
HUGH F. BILLER
Hospital were 1 case in 19. It is unfortunate that the results of radiotherapy are compared to surgical results of earlier years when transoral avulsion or piecemeal removal of the tumor were the procedures usually employed. It is evident that in the past ten years with improved definition of tumor extent, determination of blood supply and appropriate surgical exposure, the results with surgery for extracranial disease are excellent. Christianson et al" report 79% cure with surgery, Boles and Ded0 6 78%, Biller" 72%, Jafek et al" 90% cure with surgery. In the present series nine of ten patients were cured. With second procedures on those primary failures the percentage is increased to over 90%. It is our feeling that radiotherapy is contraindicated as a primary treatment modality for angiofibromas which do not demonstrate in-
tracranial extension or extensive internal carotid blood supply. In most series intracranial extension represents approximately 5% to 10%. Radiotherapy is indicated in some patients who have intracranial extension. This is true if the intracranial tumor has significant blood flow from the internal carotid artery. Patients who have intracranial extension with minimal or no internal carotid artery supply, can be operated upon. The extracranial portion of the tumor is removed and the intracranial portion is followed postoperatively by CAT scans or arteriography. Postoperatively, if there is evidence of increasing intracranial size, then radiotherapy is indicated. Radiotherapy, therefore, is reserved for only a few select cases. In our experience it has been used in 1 of 12 cases, or 8%.
REFERENCES 1. Schiff M: Juvenile nasopharyngeal angiofibroma. Laryngoscope 69:981-1016, 1959 2. Walike 1. Mackay B: Management of nasopharyngeal angiofibroma. Trans Am Acad Dphthalmol Otolaryngol 76:1346-1353, 1972 3. Christiansen TA, Duvall AJ III, Rosenberg Z, et al: Juvenile nasopharyngeal angiofibroma. Trans Am Acad Ophthalmol Otolaryngol 78:140-147, 1974 4. Duckert L, Carley R, Hilger J: Computerized axial tomography in the preoperative evaluation of an angiofibroma. Laryngoscope 88:613-618, 1978
5. Briant TD, Fitzpatrick PJ, Bock H: The radiological treatment of juvenile nasopharyngeal angiofibromas. Ann Otol Rhinol Laryngol 79:1108-1113, 1970 6. Boles R, Dedo H: Nasopharyngeal angiofibroma. Laryngoscope 86:364-372, 1976 7. Biller HF, Sessions D, Ogura J: Angiofibroma: a treatment approach. Laryngoscope 84:695-706, 1974 8. [afek BW, Nahum AM, Butler RM, et al: Surgical treatment of juvenile nasopharyngeal angiofibroma. Laryngoscope 83:707-729, 1973
REPRINTS Hugh F. Biller, MD, Department of Otolaryngology, The Mount Sinai Medical Center, One Gustave L. Levy Place, New York, NY 10029.
Downloaded from aor.sagepub.com at Bobst Library, New York University on June 16, 2015
