Clinical Review & Education
Clinical Problem Solving | RADIOLOGY
A Rare Sinonasal Entity Pooja H. Doshi, MD; Benjamin Roman, MD; Jessica Lim, MD; Deborah R. Shatzkes, MD
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B
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Figure. A patient with a sinonasal mass (arrowheads). A, Coronal computed tomographic image with bone window. B, Sagittal T1-weighted magnetic resonance image (MRI) without contrast. C, Coronal T2-weighted MRI. D, Coronal contrast-enhanced, fat-saturated, T1-weighted MRI.
A woman in her 70s with a history of radiation therapy for a nasal cavity lymphoma 15 years prior presented with complaints of chronic right nasal obstruction and right epiphora. She denied epistaxis or pain. She described having undergone 2 surgical procedures in the interim for occluded nasolacrimal duct, at 1 year and 8 years after treatment. On nasal endoscopy, a smooth, pink, friable mass was seen abutting the posterior aspect of the right inferior turbinate and extending to fill most of the nasopharynx. This mass was visible through the left choana as well. There was also an erythematous irregular area, smaller than 1 cm, on the right lateral nasal wall, corresponding to the orifice of the nasolacrimal duct. The middle turbinates were
jamaotolaryngology.com
intact, and no other lesions were seen. The oropharynx and oral cavity were clear, and findings from fiber-optic laryngoscopy was normal. Orbital examination revealed right epiphora without additional abnormality. Maxillofacial computed tomographic (CT) imaging along with a magnetic resonance imaging (MRI) of the head were performed. Coronal CT image with bone window (Figure, A), sagittal T1weighted image without contrast (Figure, B), coronal T2-weighted (Figure, C), and contrast-enhanced, fat-saturated, T1-weighted (Figure, D) images are presented for interpretation. The patient was brought to the operating room for biopsy. What is your diagnosis?
JAMA Otolaryngology–Head & Neck Surgery May 2014 Volume 140, Number 5
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archotol.jamanetwork.com/ by a University of Texas at Austin User on 06/07/2015
477
Clinical Review & Education Clinical Problem Solving
Diagnosis Sinonasal amyloidosis
Discussion Amyloidosis is a heterogeneous group of disorders characterized by the idiopathic extracellular deposition of fibrils containing amorphous proteinaceous material in various tissues and organs in the body with variable clinical presentation. Clinically, amyloidosis is classified as systemic or localized, the latter being rare. Systemic amyloidosis can be further characterized as primary or secondary. Secondary amyloidosis is associated with chronic inflammatory diseases like tuberculosis, rheumatoid arthritis, inflammatory bowel disease, or chronic renal failure.1-3 A link has been identified with hematologic disorders, such as plasma dyscrasias and lymphoma.1,2 Amyloidosis most commonly affects individuals 50 to 70 years of age, with a 3:1 or 3:2 male to female predominance. Amyloidosis of the head and neck is infrequently encountered, with about 150 cases documented since 1935. The larynx is most commonly involved (61%), followed by the oropharynx (23%), trachea (9%), orbit (4%), and the nasopharynx (3%). Isolated nasal amyloidosis is extremely rare, with about 23 reported cases to date.2,3 Presentation depends on the specific site of involvement. Sinonasal amyloidosis may present with nasal obstruction, nasal discharge, epistaxis from vessel wall invasion, and postnasal drip. On direct inspection, the mass is seen as yellowish polypoidal lesion.1-3 The patient described herein has a history of lymphoma, which is associated with secondary amyloidosis.4 To date, this patient has no signs of systemic involvement. A coronal CT image with bone window (Figure, A) demonstrates a partially calcified mass in the right inferior meatus. A sagittal T1-weighted image without contrast (Figure, B) demonstrates the mass, seen prolapsing into the nasopharynx, to be slightly hypointense relative to skeletal muscle. The mass remains hypointense on coronal T2-weighted (Figure, C) and contrast-enhanced, fat-saturated T1-weighted (Figure, D) images. The differential diagnosis of calcified sinonasal lesions includes other inflammatory processes such as chronic fungal and nonfungal rhinosinusitis and ARTICLE INFORMATION Author Affiliations: Lenox Hill Hospital, New York, New York (Doshi, Lim, Shatzkes); New York University Hospital, New York (Roman). Corresponding Author: Pooja H. Doshi, MD, Department of Radiology, Lenox Hill Hospital, 50 W 34th St, Apt 4B5, New York, NY 10001 (poojahd85 @gmail.com). Section Editor: C. Douglas Phillips, MD. Published Online: April 10, 2014. doi:10.1001/jamaoto.2014.334. Conflict of Interest Disclosures: None reported. REFERENCES
postsurgical neo-osteogenesis, as well as neoplastic entities such as chondrosarcoma, esthesioneuroblastoma, adenocarcinoma, meningioma, fibro-osseous lesions, and inverted papilloma.5 There is little in the literature to describe the imaging appearance of amyloidosis of the head and neck. Recent reports suggest that CT imaging scans tend to show a “fluffy” appearance with bony hypertrophy adjacent to a soft-tissue mass representing amyloid deposits.1 It is speculated that submucosal amyloid deposits within the nasal cavity and paranasal sinuses incite an osteoblastic reaction within the adjacent bone, causing it to appear fluffy. On MRI scans, signal intensity is similar to that of skeletal muscle on T1- and T2-weighted imaging.1 Typically, the soft-tissue masses do not enhance, although peripheral enhancement has been described. The lack of enhancement may help differentiate this process from cellular tumors.1,6 Amyloidosis must be confirmed by biopsy and pathologic analysis. This is achieved through Congo red staining, which demonstrates a characteristic “apple green” birefringence visible under polarized microscopy.2-5,7 Once a biopsy-proven diagnosis is made, systemic amyloidosis should be ruled out by a comprehensive physical examination, and additional tests such as peripheral blood smear, erythrocyte sedimentation rate, liver function tests, serum electrophoresis, and bone marrow biopsy. Abdominal fat aspiration is low risk and as sensitive as rectal mucosal biopsy (70%80%) to rule out systemic amyloidosis.2,3 There is no successful treatment for systemic amyloidosis. Localized amyloidosis is treated symptomatically with excision as necessary. The recurrence rate after localized excision is as high as 50%. Treatment with corticosteroids, chemotherapy, and radiotherapy have not been successful.3,6,8 In conclusion, amyloidosis must be considered in the differential diagnosis for sinonasal masses in patients at risk as was the case of the presented patient with a history of treated lymphoma. The CT image appearance of fluffy bone, MRI signal similar to that of skeletal muscle, and the presence of peripheral enhancement may help distinguish this diagnosis from those of neoplastic entities occurring in this region.
2. Prasad D, Somayaji GK, Arror, R, Abdulla MN. Primary nasal amyloidosis. Internet J Otorhinolaryngol. 2009; 9(2). http://ispub.com /IJORL/9/2/10505. Accessed October 3, 2012.
6. Simpson GT II, Strong MS, Skinner M, Cohen AS. Localized amyloidosis of the head and neck and upper aerodigestive and lower respiratory tracts. Ann Otol Rhinol Laryngol. 1984;93(4, pt 1):374-379.
3. Tsikoudas A, Martin-Hirsch DP, Woodhead CJ. Primary sinonasal amyloidosis. J Laryngol Otol. 2001;115(1):55-56.
7. Birchall D, Fields JM, Poon CL. Case report: focal amyloidosis of the maxillary antrum: plain film, CT and MR appearances. Clin Radiol. 1997;52(5):392-394.
4. Guvenc IA. Localized amyloidosis of the head and neck. In: Guvenc IA, ed. Amyloidosis: An Insight to Disease of Systems and Novel Therapies. 11th ed. Imzir, Turkey: InTech; 2011:107-117.
8. Paccalin M, Hachulla E, Cazalet C, et al. Localized amyloidosis: a survey of 35 French cases. Amyloid. 2005;12(4):239-245.
5. Johner CH, Widen AH, Sahgal S. Amyloidosis of the head and neck. Trans Am Acad Ophthalmol Otolaryngol. 1972;76(5):1354-1355.
1. Chin SC, Fatterpeckar G, Kao CH, Chen CY, Som PM. Amyloidosis concurrently involving the sinonasal cavities and larynx. AJNR Am J Neuroradiol. 2004;25(4):636-638.
478
JAMA Otolaryngology–Head & Neck Surgery May 2014 Volume 140, Number 5
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archotol.jamanetwork.com/ by a University of Texas at Austin User on 06/07/2015
jamaotolaryngology.com
Clinical Problem Solving | RADIOLOGY
A Rare Sinonasal Entity Pooja H. Doshi, MD; Benjamin Roman, MD; Jessica Lim, MD; Deborah R. Shatzkes, MD
A
B
C
D
Figure. A patient with a sinonasal mass (arrowheads). A, Coronal computed tomographic image with bone window. B, Sagittal T1-weighted magnetic resonance image (MRI) without contrast. C, Coronal T2-weighted MRI. D, Coronal contrast-enhanced, fat-saturated, T1-weighted MRI.
A woman in her 70s with a history of radiation therapy for a nasal cavity lymphoma 15 years prior presented with complaints of chronic right nasal obstruction and right epiphora. She denied epistaxis or pain. She described having undergone 2 surgical procedures in the interim for occluded nasolacrimal duct, at 1 year and 8 years after treatment. On nasal endoscopy, a smooth, pink, friable mass was seen abutting the posterior aspect of the right inferior turbinate and extending to fill most of the nasopharynx. This mass was visible through the left choana as well. There was also an erythematous irregular area, smaller than 1 cm, on the right lateral nasal wall, corresponding to the orifice of the nasolacrimal duct. The middle turbinates were
jamaotolaryngology.com
intact, and no other lesions were seen. The oropharynx and oral cavity were clear, and findings from fiber-optic laryngoscopy was normal. Orbital examination revealed right epiphora without additional abnormality. Maxillofacial computed tomographic (CT) imaging along with a magnetic resonance imaging (MRI) of the head were performed. Coronal CT image with bone window (Figure, A), sagittal T1weighted image without contrast (Figure, B), coronal T2-weighted (Figure, C), and contrast-enhanced, fat-saturated, T1-weighted (Figure, D) images are presented for interpretation. The patient was brought to the operating room for biopsy. What is your diagnosis?
JAMA Otolaryngology–Head & Neck Surgery May 2014 Volume 140, Number 5
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archotol.jamanetwork.com/ by a University of Texas at Austin User on 06/07/2015
477
Clinical Review & Education Clinical Problem Solving
Diagnosis Sinonasal amyloidosis
Discussion Amyloidosis is a heterogeneous group of disorders characterized by the idiopathic extracellular deposition of fibrils containing amorphous proteinaceous material in various tissues and organs in the body with variable clinical presentation. Clinically, amyloidosis is classified as systemic or localized, the latter being rare. Systemic amyloidosis can be further characterized as primary or secondary. Secondary amyloidosis is associated with chronic inflammatory diseases like tuberculosis, rheumatoid arthritis, inflammatory bowel disease, or chronic renal failure.1-3 A link has been identified with hematologic disorders, such as plasma dyscrasias and lymphoma.1,2 Amyloidosis most commonly affects individuals 50 to 70 years of age, with a 3:1 or 3:2 male to female predominance. Amyloidosis of the head and neck is infrequently encountered, with about 150 cases documented since 1935. The larynx is most commonly involved (61%), followed by the oropharynx (23%), trachea (9%), orbit (4%), and the nasopharynx (3%). Isolated nasal amyloidosis is extremely rare, with about 23 reported cases to date.2,3 Presentation depends on the specific site of involvement. Sinonasal amyloidosis may present with nasal obstruction, nasal discharge, epistaxis from vessel wall invasion, and postnasal drip. On direct inspection, the mass is seen as yellowish polypoidal lesion.1-3 The patient described herein has a history of lymphoma, which is associated with secondary amyloidosis.4 To date, this patient has no signs of systemic involvement. A coronal CT image with bone window (Figure, A) demonstrates a partially calcified mass in the right inferior meatus. A sagittal T1-weighted image without contrast (Figure, B) demonstrates the mass, seen prolapsing into the nasopharynx, to be slightly hypointense relative to skeletal muscle. The mass remains hypointense on coronal T2-weighted (Figure, C) and contrast-enhanced, fat-saturated T1-weighted (Figure, D) images. The differential diagnosis of calcified sinonasal lesions includes other inflammatory processes such as chronic fungal and nonfungal rhinosinusitis and ARTICLE INFORMATION Author Affiliations: Lenox Hill Hospital, New York, New York (Doshi, Lim, Shatzkes); New York University Hospital, New York (Roman). Corresponding Author: Pooja H. Doshi, MD, Department of Radiology, Lenox Hill Hospital, 50 W 34th St, Apt 4B5, New York, NY 10001 (poojahd85 @gmail.com). Section Editor: C. Douglas Phillips, MD. Published Online: April 10, 2014. doi:10.1001/jamaoto.2014.334. Conflict of Interest Disclosures: None reported. REFERENCES
postsurgical neo-osteogenesis, as well as neoplastic entities such as chondrosarcoma, esthesioneuroblastoma, adenocarcinoma, meningioma, fibro-osseous lesions, and inverted papilloma.5 There is little in the literature to describe the imaging appearance of amyloidosis of the head and neck. Recent reports suggest that CT imaging scans tend to show a “fluffy” appearance with bony hypertrophy adjacent to a soft-tissue mass representing amyloid deposits.1 It is speculated that submucosal amyloid deposits within the nasal cavity and paranasal sinuses incite an osteoblastic reaction within the adjacent bone, causing it to appear fluffy. On MRI scans, signal intensity is similar to that of skeletal muscle on T1- and T2-weighted imaging.1 Typically, the soft-tissue masses do not enhance, although peripheral enhancement has been described. The lack of enhancement may help differentiate this process from cellular tumors.1,6 Amyloidosis must be confirmed by biopsy and pathologic analysis. This is achieved through Congo red staining, which demonstrates a characteristic “apple green” birefringence visible under polarized microscopy.2-5,7 Once a biopsy-proven diagnosis is made, systemic amyloidosis should be ruled out by a comprehensive physical examination, and additional tests such as peripheral blood smear, erythrocyte sedimentation rate, liver function tests, serum electrophoresis, and bone marrow biopsy. Abdominal fat aspiration is low risk and as sensitive as rectal mucosal biopsy (70%80%) to rule out systemic amyloidosis.2,3 There is no successful treatment for systemic amyloidosis. Localized amyloidosis is treated symptomatically with excision as necessary. The recurrence rate after localized excision is as high as 50%. Treatment with corticosteroids, chemotherapy, and radiotherapy have not been successful.3,6,8 In conclusion, amyloidosis must be considered in the differential diagnosis for sinonasal masses in patients at risk as was the case of the presented patient with a history of treated lymphoma. The CT image appearance of fluffy bone, MRI signal similar to that of skeletal muscle, and the presence of peripheral enhancement may help distinguish this diagnosis from those of neoplastic entities occurring in this region.
2. Prasad D, Somayaji GK, Arror, R, Abdulla MN. Primary nasal amyloidosis. Internet J Otorhinolaryngol. 2009; 9(2). http://ispub.com /IJORL/9/2/10505. Accessed October 3, 2012.
6. Simpson GT II, Strong MS, Skinner M, Cohen AS. Localized amyloidosis of the head and neck and upper aerodigestive and lower respiratory tracts. Ann Otol Rhinol Laryngol. 1984;93(4, pt 1):374-379.
3. Tsikoudas A, Martin-Hirsch DP, Woodhead CJ. Primary sinonasal amyloidosis. J Laryngol Otol. 2001;115(1):55-56.
7. Birchall D, Fields JM, Poon CL. Case report: focal amyloidosis of the maxillary antrum: plain film, CT and MR appearances. Clin Radiol. 1997;52(5):392-394.
4. Guvenc IA. Localized amyloidosis of the head and neck. In: Guvenc IA, ed. Amyloidosis: An Insight to Disease of Systems and Novel Therapies. 11th ed. Imzir, Turkey: InTech; 2011:107-117.
8. Paccalin M, Hachulla E, Cazalet C, et al. Localized amyloidosis: a survey of 35 French cases. Amyloid. 2005;12(4):239-245.
5. Johner CH, Widen AH, Sahgal S. Amyloidosis of the head and neck. Trans Am Acad Ophthalmol Otolaryngol. 1972;76(5):1354-1355.
1. Chin SC, Fatterpeckar G, Kao CH, Chen CY, Som PM. Amyloidosis concurrently involving the sinonasal cavities and larynx. AJNR Am J Neuroradiol. 2004;25(4):636-638.
478
JAMA Otolaryngology–Head & Neck Surgery May 2014 Volume 140, Number 5
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archotol.jamanetwork.com/ by a University of Texas at Austin User on 06/07/2015
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