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Localized amyloid tumor of the nasopharynx
Otolaryngology– Head and Neck Surgery Volume 122 Number 2
PITKÄRANTA and MALMBERG
309
Localized amyloid tumor of the nasopharynx ANNE PITKÄRANTA, MD, and HENRIK MALMBERG, MD, Helsinki, Finland
Amyloidosis can occur in virtually every organ system of the body. However, localized amyloid tumors of the nasopharynx are exceedingly rare, and only a few cases have been reported.1,2 Among young patients amyloid tumors in the nasopharynx are even more rare.2,3 We present 2 patients with a localized amyloid tumor in the nasopharynx. One of the patients, in whom recurrent disease developed, has been followed up for 16 years and underwent a second operation. CASE REPORTS Case 1. A 14-year-old boy was referred to the Department of Otorhinolaryngology, University of Helsinki, in 1982 because of right glue ear. His medical history included insertion of grommet tubes in 1975 and 1978 and adenoidectomy in 1975. During the previous 6 months, he had had rhinorrhea and right ear obstruction. Clinical examination revealed right glue ear. Pure-tone audiometry showed a 25- to 55-dB conductive hearing loss, and the tympanometric impedance curve was flat (type C). When the postnasal space was examined, a large obstructive mass was seen extending from the upper pole of the right palatine tonsil to the nasopharyngeal tonsil and over the midline, reaching both pharyngotympanic eminences. CT showed a nasopharyngeal mass without clear margins. Carotid angiography did not show increased vasculature, which could indicate angiofibroma. Surgery was performed in
From the Department of Otolaryngology, Helsinki University Hospital. Reprint requests: Anne Pitkäranta, MD, Department of Otolaryngology, University of Helsinki, Haartmaninkatu 4 E, FIN-00290 Helsinki, Finland. Otolaryngol Head Neck Surg 2000;122:309-10. Copyright © 2000 by the American Academy of Otolaryngology– Head and Neck Surgery Foundation, Inc. 0194-5998/2000/$12.00 + 0 23/78/99035
1982 through a transpalatine approach. The tumor consisted of a nonbleeding soft mass without clear boundaries. It was totally removed both macroscopically and microscopically. Histologic examination revealed an amyloid tumor. To exclude systemic amyloidosis, the following investigations were done: serum proteins, urinalysis, creatinine, liver enzyme, and rectal biopsy stained with Congo red. No signs of systemic amyloidosis were found. The patient had continued eustachian tube dysfunction and needed recurrent tympanostomies, but otherwise he was healthy. Gradually, the postnasal space narrowed, causing more obstruction. The pharyngotympanic eminences in particular seemed to grow and almost occluded the nasopharynx with resultant nasal stuffiness. The patient underwent a second operation in 1996, during which the occluding part of the tumor was excised with a CO2 laser. Again, histologic examination showed an amyloid tumor when the specimen was stained with Congo red. During 2 years of follow-up, the nasal obstruction worsened again. However, the biopsy specimens from the prominent narrowing lower turbinate and pharyngotympanic eminences showed no amyloid tumor. Case 2. 41-year-old woman reported continuous postnasal drip and stertor for 6 months. She was admitted to the Department of Otorhinolaryngology, University of Helsinki, in 1994. Epipharyngoscopy revealed a 1.5 × 2 cm polyp-like swelling in the nasopharynx. The tumor was excised, and histologic examination showed an amyloid tumor when the specimen was stained with Congo red. The following investigations were carried out to exclude secondary or systemic amyloidosis: serum protein electrophoresis, urinalysis, urea, electrolytes, hemoglobin, blood glucose, erythrocyte sedimentation rate, white and differential cell counts, and fineneedle aspiration biopsy of adipose tissue from the anterior abdominal wall. No signs of systemic amyloidosis were
310
Otolaryngology– Head and Neck Surgery February 2000
PITKÄRANTA and MALMBERG
found. The chest x-ray film and nasopharynx MRI after operation were normal. One and a half years after removal of the tumor, nasal obstruction reappeared, and posterior rhinoscopy revealed a small mass in the region of the middle nasopharynx. The mass was removed with a sharp curette, and mirror inspection of the nasopharynx was used to control for completeness of removal. During the histopathologic analysis, however, no signs of residual or recurrent disease could be found. After 4 years’ follow-up, no residual tumor has been found. DISCUSSION Localized amyloid tumors in the head and neck region are an extremely rare manifestation that usually is not associated with either multiple myeloma or systemic amyloidosis.4 However, careful examination is necessary to make the diagnosis of localized amyloidosis, as distinguished from systemic amyloidosis, because in the systemic forms of amyloidosis, life expectancy is markedly shortened.5 So far, the best therapy of localized amyloidosis is surgery. In our first case the condition has proved difficult to treat. The slow enlargement of amyloid in the nasopharynx after surgical treatment persisted, and the patient’s quality of life decreased because of nasal obstruction, eustachian tube dysfunction, and recurrent need for tympanostomy tubes. The rate of recurrence may decrease with CO2 laser excision, but the great rarity and slow progression of this disease make it difficult to verify.
Our first patient was 14 years old when the amyloid tumor was found in his nasopharynx and surgically treated. Now, 16 years later, after surgical operations his postnasal space is narrowed, occluding nasal respiration, and he still needs the tympanostomy tubes. In the literature we found only 2 reports of this condition in young persons. In the first case, the treatment was not surgical because the patient’s parents refused the removal of the amyloid deposit.3 In the second case, the treatment was surgical and successful during 9 months of follow-up.2 We conclude that amyloidosis should be considered in the differential diagnosis of glue ear, nasal obstruction, epistaxis, and postnasal discharge and that amyloid tumors in the nasopharynx are difficult to treat and can reoccur, thus requiring a long follow-up period. REFERENCES 1. Panda NK, Sharma SC, Mann SBS, et al. Localized amyloidosis of the nasopharynx. Ear Nose Throat J 1994;73:335-6. 2. Dominguez S, Wienberg P, Claros P, et al. Primary localized nasopharyngeal amyloidosis. A case report. Int J Pediatr Otorhinolaryngol 1996;36:61-7. 3. Zakzouk MS. Clinical records. An unusual etiology of unilateral glue ear in a child. J Laryngol Otol 1984;98:519-22. 4. Godbersen GS, Leh JF, Hansmann ML, et al. Organ-limited laryngeal amyloid deposits: clinical, morphological, and immunohistochemical results of five case. Ann Otol Rhinol Laryngol 1992;101:770-5. 5. Kyle RA, Bayrd ED. Amyloidosis: review of 236 case. Medicine (Baltimore) 1975;54:271-99.
5th International Muscle Symposium
The symposium will be held May 19-21, 2000, in Vienna, Austria. For further information, contact Manfred Frey, MD, Division of Plastic and Reconstructive Surgery, Department of Surgery, University of Vienna, Medical School, Währinger Gürtel 18-20, A-1090 Vienna, Austria; phone, 43-1-40400-6986; fax, 43-1-40400-6988; e-mail, [email protected]; URL, http://www.akhwien.ac.at/plastsurg.
PITKÄRANTA and MALMBERG
309
Localized amyloid tumor of the nasopharynx ANNE PITKÄRANTA, MD, and HENRIK MALMBERG, MD, Helsinki, Finland
Amyloidosis can occur in virtually every organ system of the body. However, localized amyloid tumors of the nasopharynx are exceedingly rare, and only a few cases have been reported.1,2 Among young patients amyloid tumors in the nasopharynx are even more rare.2,3 We present 2 patients with a localized amyloid tumor in the nasopharynx. One of the patients, in whom recurrent disease developed, has been followed up for 16 years and underwent a second operation. CASE REPORTS Case 1. A 14-year-old boy was referred to the Department of Otorhinolaryngology, University of Helsinki, in 1982 because of right glue ear. His medical history included insertion of grommet tubes in 1975 and 1978 and adenoidectomy in 1975. During the previous 6 months, he had had rhinorrhea and right ear obstruction. Clinical examination revealed right glue ear. Pure-tone audiometry showed a 25- to 55-dB conductive hearing loss, and the tympanometric impedance curve was flat (type C). When the postnasal space was examined, a large obstructive mass was seen extending from the upper pole of the right palatine tonsil to the nasopharyngeal tonsil and over the midline, reaching both pharyngotympanic eminences. CT showed a nasopharyngeal mass without clear margins. Carotid angiography did not show increased vasculature, which could indicate angiofibroma. Surgery was performed in
From the Department of Otolaryngology, Helsinki University Hospital. Reprint requests: Anne Pitkäranta, MD, Department of Otolaryngology, University of Helsinki, Haartmaninkatu 4 E, FIN-00290 Helsinki, Finland. Otolaryngol Head Neck Surg 2000;122:309-10. Copyright © 2000 by the American Academy of Otolaryngology– Head and Neck Surgery Foundation, Inc. 0194-5998/2000/$12.00 + 0 23/78/99035
1982 through a transpalatine approach. The tumor consisted of a nonbleeding soft mass without clear boundaries. It was totally removed both macroscopically and microscopically. Histologic examination revealed an amyloid tumor. To exclude systemic amyloidosis, the following investigations were done: serum proteins, urinalysis, creatinine, liver enzyme, and rectal biopsy stained with Congo red. No signs of systemic amyloidosis were found. The patient had continued eustachian tube dysfunction and needed recurrent tympanostomies, but otherwise he was healthy. Gradually, the postnasal space narrowed, causing more obstruction. The pharyngotympanic eminences in particular seemed to grow and almost occluded the nasopharynx with resultant nasal stuffiness. The patient underwent a second operation in 1996, during which the occluding part of the tumor was excised with a CO2 laser. Again, histologic examination showed an amyloid tumor when the specimen was stained with Congo red. During 2 years of follow-up, the nasal obstruction worsened again. However, the biopsy specimens from the prominent narrowing lower turbinate and pharyngotympanic eminences showed no amyloid tumor. Case 2. 41-year-old woman reported continuous postnasal drip and stertor for 6 months. She was admitted to the Department of Otorhinolaryngology, University of Helsinki, in 1994. Epipharyngoscopy revealed a 1.5 × 2 cm polyp-like swelling in the nasopharynx. The tumor was excised, and histologic examination showed an amyloid tumor when the specimen was stained with Congo red. The following investigations were carried out to exclude secondary or systemic amyloidosis: serum protein electrophoresis, urinalysis, urea, electrolytes, hemoglobin, blood glucose, erythrocyte sedimentation rate, white and differential cell counts, and fineneedle aspiration biopsy of adipose tissue from the anterior abdominal wall. No signs of systemic amyloidosis were
310
Otolaryngology– Head and Neck Surgery February 2000
PITKÄRANTA and MALMBERG
found. The chest x-ray film and nasopharynx MRI after operation were normal. One and a half years after removal of the tumor, nasal obstruction reappeared, and posterior rhinoscopy revealed a small mass in the region of the middle nasopharynx. The mass was removed with a sharp curette, and mirror inspection of the nasopharynx was used to control for completeness of removal. During the histopathologic analysis, however, no signs of residual or recurrent disease could be found. After 4 years’ follow-up, no residual tumor has been found. DISCUSSION Localized amyloid tumors in the head and neck region are an extremely rare manifestation that usually is not associated with either multiple myeloma or systemic amyloidosis.4 However, careful examination is necessary to make the diagnosis of localized amyloidosis, as distinguished from systemic amyloidosis, because in the systemic forms of amyloidosis, life expectancy is markedly shortened.5 So far, the best therapy of localized amyloidosis is surgery. In our first case the condition has proved difficult to treat. The slow enlargement of amyloid in the nasopharynx after surgical treatment persisted, and the patient’s quality of life decreased because of nasal obstruction, eustachian tube dysfunction, and recurrent need for tympanostomy tubes. The rate of recurrence may decrease with CO2 laser excision, but the great rarity and slow progression of this disease make it difficult to verify.
Our first patient was 14 years old when the amyloid tumor was found in his nasopharynx and surgically treated. Now, 16 years later, after surgical operations his postnasal space is narrowed, occluding nasal respiration, and he still needs the tympanostomy tubes. In the literature we found only 2 reports of this condition in young persons. In the first case, the treatment was not surgical because the patient’s parents refused the removal of the amyloid deposit.3 In the second case, the treatment was surgical and successful during 9 months of follow-up.2 We conclude that amyloidosis should be considered in the differential diagnosis of glue ear, nasal obstruction, epistaxis, and postnasal discharge and that amyloid tumors in the nasopharynx are difficult to treat and can reoccur, thus requiring a long follow-up period. REFERENCES 1. Panda NK, Sharma SC, Mann SBS, et al. Localized amyloidosis of the nasopharynx. Ear Nose Throat J 1994;73:335-6. 2. Dominguez S, Wienberg P, Claros P, et al. Primary localized nasopharyngeal amyloidosis. A case report. Int J Pediatr Otorhinolaryngol 1996;36:61-7. 3. Zakzouk MS. Clinical records. An unusual etiology of unilateral glue ear in a child. J Laryngol Otol 1984;98:519-22. 4. Godbersen GS, Leh JF, Hansmann ML, et al. Organ-limited laryngeal amyloid deposits: clinical, morphological, and immunohistochemical results of five case. Ann Otol Rhinol Laryngol 1992;101:770-5. 5. Kyle RA, Bayrd ED. Amyloidosis: review of 236 case. Medicine (Baltimore) 1975;54:271-99.
5th International Muscle Symposium
The symposium will be held May 19-21, 2000, in Vienna, Austria. For further information, contact Manfred Frey, MD, Division of Plastic and Reconstructive Surgery, Department of Surgery, University of Vienna, Medical School, Währinger Gürtel 18-20, A-1090 Vienna, Austria; phone, 43-1-40400-6986; fax, 43-1-40400-6988; e-mail, [email protected]; URL, http://www.akhwien.ac.at/plastsurg.