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Pediatric localized amyloidosis in mesopharynx
International Journal of Pediatric Otorhinolaryngology 66 (2002) 181 /184 www.elsevier.com/locate/ijporl
Case report
Pediatric localized amyloidosis in mesopharynx Masahiro Takahashi a,*, Hideki Matsuda a, Keiko Ito b, Takaaki Ito c, Mamoru Tsukuda a a
Department of Otolaryngology, Yokohama City University, 3-9 Fukuura, Kanazawa-ku, Yokohama, Japan b Department of Otolaryngology, Kinugasa Hospital, Yokosuka, Japan c Department of Pathology, Yokohama City University, 3-9 Fukuura, Kanazawa-ku, Yokohama, Japan Received 15 February 2002; received in revised form 25 June 2002; accepted 26 June 2002
Abstract Primary localized amyloidosis of the mesopharynx is rare. We present the case of a 12-year-old girl; as far as we are aware, our case is the third pediatric case in the English literature. Her chief complaint was a pharyngeal mass identified by the health examination at primary school. Physical examination revealed a mass in the faucial tonsil. Histologically, the lesion was composed of deposition of amyloid and chronic inflammatory cell infiltration. Immunohistochemical studies showed that the lesion was positive for amyloid P component (AP) and negative for amyloid L (AL). No treatments were applied, because she had no symptoms. During the observation period of 24 months, the mesopharyngeal mass showed no remarkable change. # 2002 Elsevier Science Ireland Ltd. All rights reserved. Keywords: Localized amyloidosis; Pediatric; Mesopharynx
1. Introduction Amyloidosis, which is characterized by deposition of an acellular proteinous material in tissues, is an uncommon disorder of unknown causes [1]. In 1854, Virchow for the first time used the term ‘amyloid’ to describe the starch-like reaction to eosin and sulfuric acid. Amyloidosis is clinically
* Corresponding author. Present address: Department of Otolaryngology, Toranomon Hospital, 2-2-2 Toranomon, Minato-ku, Tokyo, Japan. Tel.: /81-3-3588-1111; fax: /813-3582-7068 E-mail address: [email protected] (M. Takahashi).
classified into two types: systemic and localized amylodosis. Systemic amyloidosis is a disease with amyloid deposition in several organs, and localized amyloidosis is a disorder with amyloid deposition in one organ. In systemic amyloidosis, amyloid deposition is found in the heart, kidney, or liver. Sites involved in localized amyloidosis of the head and neck region include the eye, salivary gland, nasal mucosa, paranasal sinus, nasopharynx, oral cavity, and larynx [2,3]. Pediatric localized amyloidosis in the head and neck region is very rare [4]. We present a case of pediatric localized amyloidosis in the mesopharynx, and discuss the clinicopathologic and immunohistochemical aspects of this disease.
0165-5876/02/$ - see front matter # 2002 Elsevier Science Ireland Ltd. All rights reserved. PII: S 0 1 6 5 - 5 8 7 6 ( 0 2 ) 0 0 2 1 5 - X
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M. Takahashi et al. / Int. J. Pediatr. Otorhinolaryngol. 66 (2002) 181 /184
2. Case report A 12-year-old girl consulted our clinic on suspicion of a mesopharyngeal tumor after health care examination at the primary school. She had no complaints, such as dysphagia or paresthesia. Physical examination revealed a yellow mass medially to the bilateral faucial tonsils (Fig. 1). The surface of this mass was smooth and soft. No masses were found in other regions. Laboratory evaluation revealed normal findings. The chest radiography, electrocardiography and cardiac ultrasonography were normal. Computed tomography (CT) showed a mass in mesopharyngeal region (Fig. 2). The biopsied specimens were stained with hematoxylin and eosin, and Congo red (Fig. 3a, b). Immunohistochemical study was performed; deparaffinized sections were incubated in rabbit antihuman immunoglobulin (IgG), kappa and lambda light chains, and amyloid A (AA) and amyloid P (AP) for 30 min. They were negative for kappa and lambda chains, and AA. They were positive only for AP (Fig. 4a, b). No treatments had been applied. The amyloid mass showed no remarkable changes for 24 months.
Fig. 2. Axial view of CT shows a mass in the mesopharynx (arrow).
3. Discussion Amyloidosis is a disorder characterized by extracellular deposition of homogenous, eosinophilic, and proteinaneous material [1]. Virchow for the first time used the term of amyloid in 1854.
Fig. 1. A yellow mass is found medially to the bilateral faucial tonsils (arrow). Uvula (arrow head).
Fig. 3. (a) Eosinophilic amorphous materials are seen in the submucosal tissue (arrow). H & E, X50. (b). Staining with Congo red shows orange homogenous material. Congo red, / 50.
M. Takahashi et al. / Int. J. Pediatr. Otorhinolaryngol. 66 (2002) 181 /184
183
Fig. 4. Lesion is positive for AP (a), and negative for AA (b), AL-kappa chain (c) and AL-lambda chain (d).
Amyloidosis is often subclassified as systemic and localized amyloidosis. Systemic amyloidosis includes primary, secondary, and familial amyloidosis. It has been reported that localized amyloidosis is involved in the salivary gland, oral cavity, pharynx, paranasal sinus, larynx, trachea, bronchus, and lung [2,3]. The larynx is the most common site of localized amyloidosis in the head and neck region [3,4]. Our case is localized amyloidosis in the mesopharynx. Pediatric localized amyloidosis is quite rare. As far as we are aware, our case is the third case in the English literature [5,6]. The two cases of pediatric localized amyloidosis reported before were involved in the nasopharynx: thus, our case is the first of pediatric localized amyloidosis found in the mesopharynx.
In recent studies, the relationship between the clinicopathologic features and the biochemical compositions of fibroprotein have been reported [7,8]. There are three kinds of fibroprotein, such as amyloid L (AL), AA and AP. AL consists of kappa or lambda monoclonal light chains. AL is associated with primary systemic amyloidosis, myeloma-associated amyloidosis, and many cases of localized amyloidosis. AA is found in secondary amyloidosis and familial Mediterranean fever, which is one of familial amyloidosis. AP is found in all types of amyloid and has no specificity. The biopsied specimens from our case were positive for AP, but negative for AA and AL. We diagnosed this case as amyloidosis based on these immunohistochemical findings as well as result of Congo red staining. Sometimes it is difficult to diagnose
184
M. Takahashi et al. / Int. J. Pediatr. Otorhinolaryngol. 66 (2002) 181 /184
certainly only with Congo red. In such a case Congo red fluorescene technique, which is more sensitive than Congo red itself, is effective [9]. Clinical examination in our case revealed a mass only in the mesopharynx, and no deposition could be seen in other organs. Therefore, we diagnosed localized amyloidosis although immunohistochemical study showed AP-positive and AL-negative findings. In agreement with our immunohistochemical result, it has been reported that specimens from some cases of localized amyloidosis are negative for AL [10]. The first choice of treatment for localized amyloidosis in the head and neck region is surgical removal [3]. The surgical treatment must be done in case of airway obstruction, bleeding, and other severe symptoms. Operation with CO2-LASER can be used for laryngeal amyloisosis [7,11], and laryngectomy is occassionally required for control of the disease [3]. Our case is the third of pediatric localized amyloidosis in head and neck region; the other two cases of amyloidosis involved in the nasopharynx. In the two cases involving the nasopharynx, surgical treatment was performed because of nasal obstruction and epistaxis. In our case, we have not performed surgical treatment, for the following reasons: (1) the patient has no symptoms, (2) airway obstruction is not found, because the mass is small, (3) localized amyloidosis is clinically benign. In 2 years of follow-up, our case has shown no remarkable change.
References [1] G.G. Glenner, Amyloid deposits and amyloidosis: the bfibrilloses, New Engl. J. Med. 302 (1980) 1283 /1292. [2] M. Toyoda, Y. Ebihara, H. Kato, S. Kita, Tracheobronchial AL Amyloidosis: histologic, immunohistochemical, ultrastructural, and immunoelectron microscopic observations, Hum. Pathol. 24 (9) (1993) 970 /976. [3] J.E. Lewis, K.D. Olsen, P.J. Kurtin, R.A. Kyle, Laryngeal amyloidosis: a clinicopathologic review, Otolaryngol. Head Neck Surg. 106 (1992) 372 /377. [4] N.K. Panda, S.C. Sharma, S.B. Mann, B.D. Radotra, Localized amyloidosis of the nasopharynx, Ear Nose Throat J. 73 (1994) 335 /336. [5] A.A. Mufarrij, N.Y. Busaba, G.M. Zaytoum, G.R. Gallo, H.D. Feiner, Primary localized amyloidosis of the nose and paranasal sinuses. A case report with immunohistochemical observatinon and review of the literature, Am. J. Surg. Pathol. 14 (1990) 379 /383. [6] S. Domingues, P. Wienberg, P. Claros, A. Claros, J. Vila, Primary localized nasopharyngeal amyloisosis. A case report, Int. J. Pediatr. Otorhinolaryngol. 36 (1996) 53 /60. [7] G.T. Simpson, M. Skinner, M.S. Strong, A.S. Cohen, Localized amyloidosis of the head and neck and upper aerodigestive and lower espiratory tracts, Ann. Otol. Rhinol. Laryngol. 93 (1984) 374 /379. [8] M. Beiser, G. Messer, J. Samuel, B. Gross, E. Shanon, Amyloidosis of Waldeyer’s ring. A clinical and ultrastructual report, Acta Otolaryngol. 89 (1980) 562 /569. [9] R.P. Linke, Highly sensitive diagnosis of amyloid and various amyloid syndromes using Congo red fluorescence, Virchows. Arch. 436 (2000) 439 /448. [10] H. Nagata, T. Yoshimura, M. Nomoto, T. Kanda, T. Kaneko, Y. Yaku, Light and electron microscopic studies of localized laryngeal amyloidosis, Arch. Otorhinolaryngol. 244 (1987) 180 /184. [11] G. Galvan-Ogazon, L.A. Teran, J. Fernandez, P. Pasqual, Beta-2 microgloburin as the main component of primary amyloidosis located in the larynx, Laryngoscope 99 (1989) 1072 /1074.
Case report
Pediatric localized amyloidosis in mesopharynx Masahiro Takahashi a,*, Hideki Matsuda a, Keiko Ito b, Takaaki Ito c, Mamoru Tsukuda a a
Department of Otolaryngology, Yokohama City University, 3-9 Fukuura, Kanazawa-ku, Yokohama, Japan b Department of Otolaryngology, Kinugasa Hospital, Yokosuka, Japan c Department of Pathology, Yokohama City University, 3-9 Fukuura, Kanazawa-ku, Yokohama, Japan Received 15 February 2002; received in revised form 25 June 2002; accepted 26 June 2002
Abstract Primary localized amyloidosis of the mesopharynx is rare. We present the case of a 12-year-old girl; as far as we are aware, our case is the third pediatric case in the English literature. Her chief complaint was a pharyngeal mass identified by the health examination at primary school. Physical examination revealed a mass in the faucial tonsil. Histologically, the lesion was composed of deposition of amyloid and chronic inflammatory cell infiltration. Immunohistochemical studies showed that the lesion was positive for amyloid P component (AP) and negative for amyloid L (AL). No treatments were applied, because she had no symptoms. During the observation period of 24 months, the mesopharyngeal mass showed no remarkable change. # 2002 Elsevier Science Ireland Ltd. All rights reserved. Keywords: Localized amyloidosis; Pediatric; Mesopharynx
1. Introduction Amyloidosis, which is characterized by deposition of an acellular proteinous material in tissues, is an uncommon disorder of unknown causes [1]. In 1854, Virchow for the first time used the term ‘amyloid’ to describe the starch-like reaction to eosin and sulfuric acid. Amyloidosis is clinically
* Corresponding author. Present address: Department of Otolaryngology, Toranomon Hospital, 2-2-2 Toranomon, Minato-ku, Tokyo, Japan. Tel.: /81-3-3588-1111; fax: /813-3582-7068 E-mail address: [email protected] (M. Takahashi).
classified into two types: systemic and localized amylodosis. Systemic amyloidosis is a disease with amyloid deposition in several organs, and localized amyloidosis is a disorder with amyloid deposition in one organ. In systemic amyloidosis, amyloid deposition is found in the heart, kidney, or liver. Sites involved in localized amyloidosis of the head and neck region include the eye, salivary gland, nasal mucosa, paranasal sinus, nasopharynx, oral cavity, and larynx [2,3]. Pediatric localized amyloidosis in the head and neck region is very rare [4]. We present a case of pediatric localized amyloidosis in the mesopharynx, and discuss the clinicopathologic and immunohistochemical aspects of this disease.
0165-5876/02/$ - see front matter # 2002 Elsevier Science Ireland Ltd. All rights reserved. PII: S 0 1 6 5 - 5 8 7 6 ( 0 2 ) 0 0 2 1 5 - X
182
M. Takahashi et al. / Int. J. Pediatr. Otorhinolaryngol. 66 (2002) 181 /184
2. Case report A 12-year-old girl consulted our clinic on suspicion of a mesopharyngeal tumor after health care examination at the primary school. She had no complaints, such as dysphagia or paresthesia. Physical examination revealed a yellow mass medially to the bilateral faucial tonsils (Fig. 1). The surface of this mass was smooth and soft. No masses were found in other regions. Laboratory evaluation revealed normal findings. The chest radiography, electrocardiography and cardiac ultrasonography were normal. Computed tomography (CT) showed a mass in mesopharyngeal region (Fig. 2). The biopsied specimens were stained with hematoxylin and eosin, and Congo red (Fig. 3a, b). Immunohistochemical study was performed; deparaffinized sections were incubated in rabbit antihuman immunoglobulin (IgG), kappa and lambda light chains, and amyloid A (AA) and amyloid P (AP) for 30 min. They were negative for kappa and lambda chains, and AA. They were positive only for AP (Fig. 4a, b). No treatments had been applied. The amyloid mass showed no remarkable changes for 24 months.
Fig. 2. Axial view of CT shows a mass in the mesopharynx (arrow).
3. Discussion Amyloidosis is a disorder characterized by extracellular deposition of homogenous, eosinophilic, and proteinaneous material [1]. Virchow for the first time used the term of amyloid in 1854.
Fig. 1. A yellow mass is found medially to the bilateral faucial tonsils (arrow). Uvula (arrow head).
Fig. 3. (a) Eosinophilic amorphous materials are seen in the submucosal tissue (arrow). H & E, X50. (b). Staining with Congo red shows orange homogenous material. Congo red, / 50.
M. Takahashi et al. / Int. J. Pediatr. Otorhinolaryngol. 66 (2002) 181 /184
183
Fig. 4. Lesion is positive for AP (a), and negative for AA (b), AL-kappa chain (c) and AL-lambda chain (d).
Amyloidosis is often subclassified as systemic and localized amyloidosis. Systemic amyloidosis includes primary, secondary, and familial amyloidosis. It has been reported that localized amyloidosis is involved in the salivary gland, oral cavity, pharynx, paranasal sinus, larynx, trachea, bronchus, and lung [2,3]. The larynx is the most common site of localized amyloidosis in the head and neck region [3,4]. Our case is localized amyloidosis in the mesopharynx. Pediatric localized amyloidosis is quite rare. As far as we are aware, our case is the third case in the English literature [5,6]. The two cases of pediatric localized amyloidosis reported before were involved in the nasopharynx: thus, our case is the first of pediatric localized amyloidosis found in the mesopharynx.
In recent studies, the relationship between the clinicopathologic features and the biochemical compositions of fibroprotein have been reported [7,8]. There are three kinds of fibroprotein, such as amyloid L (AL), AA and AP. AL consists of kappa or lambda monoclonal light chains. AL is associated with primary systemic amyloidosis, myeloma-associated amyloidosis, and many cases of localized amyloidosis. AA is found in secondary amyloidosis and familial Mediterranean fever, which is one of familial amyloidosis. AP is found in all types of amyloid and has no specificity. The biopsied specimens from our case were positive for AP, but negative for AA and AL. We diagnosed this case as amyloidosis based on these immunohistochemical findings as well as result of Congo red staining. Sometimes it is difficult to diagnose
184
M. Takahashi et al. / Int. J. Pediatr. Otorhinolaryngol. 66 (2002) 181 /184
certainly only with Congo red. In such a case Congo red fluorescene technique, which is more sensitive than Congo red itself, is effective [9]. Clinical examination in our case revealed a mass only in the mesopharynx, and no deposition could be seen in other organs. Therefore, we diagnosed localized amyloidosis although immunohistochemical study showed AP-positive and AL-negative findings. In agreement with our immunohistochemical result, it has been reported that specimens from some cases of localized amyloidosis are negative for AL [10]. The first choice of treatment for localized amyloidosis in the head and neck region is surgical removal [3]. The surgical treatment must be done in case of airway obstruction, bleeding, and other severe symptoms. Operation with CO2-LASER can be used for laryngeal amyloisosis [7,11], and laryngectomy is occassionally required for control of the disease [3]. Our case is the third of pediatric localized amyloidosis in head and neck region; the other two cases of amyloidosis involved in the nasopharynx. In the two cases involving the nasopharynx, surgical treatment was performed because of nasal obstruction and epistaxis. In our case, we have not performed surgical treatment, for the following reasons: (1) the patient has no symptoms, (2) airway obstruction is not found, because the mass is small, (3) localized amyloidosis is clinically benign. In 2 years of follow-up, our case has shown no remarkable change.
References [1] G.G. Glenner, Amyloid deposits and amyloidosis: the bfibrilloses, New Engl. J. Med. 302 (1980) 1283 /1292. [2] M. Toyoda, Y. Ebihara, H. Kato, S. Kita, Tracheobronchial AL Amyloidosis: histologic, immunohistochemical, ultrastructural, and immunoelectron microscopic observations, Hum. Pathol. 24 (9) (1993) 970 /976. [3] J.E. Lewis, K.D. Olsen, P.J. Kurtin, R.A. Kyle, Laryngeal amyloidosis: a clinicopathologic review, Otolaryngol. Head Neck Surg. 106 (1992) 372 /377. [4] N.K. Panda, S.C. Sharma, S.B. Mann, B.D. Radotra, Localized amyloidosis of the nasopharynx, Ear Nose Throat J. 73 (1994) 335 /336. [5] A.A. Mufarrij, N.Y. Busaba, G.M. Zaytoum, G.R. Gallo, H.D. Feiner, Primary localized amyloidosis of the nose and paranasal sinuses. A case report with immunohistochemical observatinon and review of the literature, Am. J. Surg. Pathol. 14 (1990) 379 /383. [6] S. Domingues, P. Wienberg, P. Claros, A. Claros, J. Vila, Primary localized nasopharyngeal amyloisosis. A case report, Int. J. Pediatr. Otorhinolaryngol. 36 (1996) 53 /60. [7] G.T. Simpson, M. Skinner, M.S. Strong, A.S. Cohen, Localized amyloidosis of the head and neck and upper aerodigestive and lower espiratory tracts, Ann. Otol. Rhinol. Laryngol. 93 (1984) 374 /379. [8] M. Beiser, G. Messer, J. Samuel, B. Gross, E. Shanon, Amyloidosis of Waldeyer’s ring. A clinical and ultrastructual report, Acta Otolaryngol. 89 (1980) 562 /569. [9] R.P. Linke, Highly sensitive diagnosis of amyloid and various amyloid syndromes using Congo red fluorescence, Virchows. Arch. 436 (2000) 439 /448. [10] H. Nagata, T. Yoshimura, M. Nomoto, T. Kanda, T. Kaneko, Y. Yaku, Light and electron microscopic studies of localized laryngeal amyloidosis, Arch. Otorhinolaryngol. 244 (1987) 180 /184. [11] G. Galvan-Ogazon, L.A. Teran, J. Fernandez, P. Pasqual, Beta-2 microgloburin as the main component of primary amyloidosis located in the larynx, Laryngoscope 99 (1989) 1072 /1074.