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Unilateral Ear and Temporomandibular Joint Discomfort

Unilateral Ear and Temporomandibular Joint Discomfort Case A woman in her 30s presented with right-sided otalgia, otorrhea, decreased hearing, and worsening right temporomandibular joint (TMJ) pain. Her medical history was unremarkable. Physical examination revealed bilateral TMJ tenderness with crepitus on the right. Examination of the right ear revealed a polyp and granulation tissue in the anterior-superior external auditory canal (EAC) that was mobile with jaw movement and a normal-appearing tympanic membrane. An audiogram showed normal hearing. Computed tomographic (CT) imaging revealed dehiscence of the right glenoid fossa, erosion of the anterior EAC, TMJ, and middle cranial fossa (Figure, A). Magnetic resonance imaging (MRI) revealed a heterogeneous 8.5 × 7-mm mass based in the air cells at the root of zygoma, posterior to the right TMJ, with extension into the osseous external canal. The lesion displayed intermediate signal intensity on T1- and T2-weighting and enhanced with gadolinium administration (Figure, B). Positron emission tomographic (PET)-CT imaging ruled out distant metastases. Figure. View LargeDownload A, Computed tomographic image revealing dehiscence of the right glenoid fossa, erosion of the anterior external auditory canal, temporomandibular joint, and middle cranial fossa. The arrow indicates soft-tissue mass with bony erosion noted anteriorly into glenoid fossa and superiorly approaching the cranial vault. B, Magnetic resonance image (MRI) of the lesion displaying intermediate signal intensity on T1- and T2-weighting and enhanced with gadolinium administration. The arrow indicates coronal T1-weighted MRI postgadolinium with fat saturation revealing avid enhancement of soft-tissue mass superior and posterior to glenoid fossa. C, Multinucleated cells dispersed in a somewhat lobular pattern (hematoxylin-eosin, original magnification ×40). An in-office biopsy of the ear canal tissue was performed, the histologic findings indicated a giant cell granuloma-like lesion with osteogenesis. Adjacent to this were regions of keratin debris and squamous mucosa with an area of cartilage displaying prominent mononuclear morphologic characteristics. A lateral temporal bone resection with en bloc removal of the mass was performed. Gross analysis of the tissue revealed an ill-defined, semitranslucent 0.9 × 0.5 × 0.4-cm mass. Histopathologic findings revealed chondromyxoid proliferation with abundant multinucleated giant cells and prominent admixed mononuclear cells. The mononuclear cells contained central nuclear grooves and were associated with chondroid matrix. The multinucleated cells were dispersed in a somewhat lobular pattern (Figure, C). What is your diagnosis? Read the Discussion. Discussion Diagnosis Chondroblastoma of the temporal bone Discussion Chondroblastoma is a rare, benign neoplasm comprising less than 1% of primary bone tumors. Localization to the temporal bone is similarly infrequent, with most tumors localized to the epiphyses of the long bones and pelvis.1,2 Chondroblastoma of the skull base, particularly the temporal bone, is a rare occurrence, with a 2011 review2 reporting 81 cases. The diagnosis can often be challenging owing to ambiguous clinical findings. The differential diagnosis for chondroblastoma of the temporal bone includes cholesteatoma, giant-cell tumor, cholesterol granuloma, aneurysmal bone cyst, and chondrosarcoma.3 This rare neoplasm presents with a wide range of symptoms. A review of the current literature conducted by Reid et al2 reported that the most common presenting symptoms of chondroblastoma are hearing loss (49%), cranial nerve involvement (43.2%), facial swelling (22.2%), and otalgia (19.8%). Other symptoms include tinnitus, aural fullness, and disturbance of equilibrium. Owing to the ambiguous clinical presentation, complimentary studies are necessary for accurate diagnosis. Classic histologic findings include chondromyxoid proliferation with embedded osteoclast-like giant cells. Cells are often arranged in a lobular fashion and may reveal calcification in a “chicken-wire” fashion dispersed within chondroid and endochondral bone formations.3,4 This was consistent with our biopsy result. Reactive changes may be apparent with portions of aneurysmal bone cyst or reparative granuloma formation. Tumor cells have been reported to exhibit eccentrically localized nuclei often revealing a “grooved” appearance with absence of atypical mitotic figures.3,5 Immunoreactivity to S-100 protein, indicating cartilaginous origin, has been described as a useful adjunct in narrowing the differential between chondroblastoma vs giant-cell tumor, as giant-cell tumor does not produce chondroid matrix.3-5 These factors reinforce the importance of integrating the characteristic radiographic findings when establishing the diagnosis. Histologic findings are supported by CT and MRI studies. The CT studies may reveal a lobulated, expansile soft-tissue mass with foci of calcification and heterogeneous contrast enhancement. Magnetic resonance imaging offers superior visualization to assess for dural or brain tissue involvement and reveal heterogeneous gadolinium enhancement throughout the mass. As such, current recommendations for imaging evaluation recommend both CT and MRI assessment.2,3 One study6 reported a notable finding that 76% of temporal bone chondroblastoma lesions exhibit erosion into the EAC. Dural involvement was less common, with 4 of 21 cases describing dural adhesion. A dural biopsy was performed by the neurosurgery unit in our patient, and the results confirmed that there was no involvement on histologic analysis. Owing to the inability to rule out a malignant process in the initial stages of evaluation, however, imaging with PET-CT was also performed to assess for chondrosarcoma or alternative malignant lesion. PET-CT may be valuable in the initial workup when considering malignant disease, although it may not be necessary once the diagnosis of chondroblastoma, an inherently benign process, has been established. Surgical treatment historically included approaches ranging from simple curettage, irradiation, or en bloc resection.2 Chondroblastoma may display local recurrence rates nearing 20%, and reports of transformation to chondrosarcoma following radiation therapy have led to the recommendation for surgical treatment via en bloc resection without radiotherapy.2,3 Owing to the potential for recurrence, the need for long-term monitoring and follow-up is implicit and establishes the basis for the lateral temporal bone resection with en bloc removal performed in this case. The patient had no morbidity owing to the procedure, and her conductive hearing loss was rehabilitated with a bone conduction hearing device. In sum, chondroblastoma of the temporal bone is a rare neoplastic lesion with ambiguous clinical findings. A strong multidisciplinary approach to include neuroradiologic review and characteristic histopathologic analysis support the diagnosis. Complete en bloc excision without the use of radiotherapy is the recommended surgical intervention and is considered curative, with a necessity for long-term monitoring. Back to top Article Information Corresponding Author: Alexander Lanigan, MD, Captain, Medical Corps, United States Army, San Antonio Military Medical Center, 3551 Roger Brooks Dr, San Antonio, TX 78234 (alexander.e.lanigan.mil@mail.mil) (Alexander.lanigan@usuhs.edu). Published Online: August 14, 2014. doi:10.1001/jamaoto.2014.1472. Conflict of Interest Disclosures: None reported. Additional Contributions: Andrew J. Fishman, MD, of Cadence Neuroscience Institute, provided clinical expertise and editorial support in preparing this case for publication. Section Editor: Edward B. Stelow, MD. References 1. Dahlin DC, Ivins JC. Benign chondroblastoma: a study of 125 cases. Cancer. 1972;30(2):401-413.PubMedGoogle ScholarCrossref 2. Reid LB, Wong DS, Lyons B. Chondroblastoma of the temporal bone: a case series, review, and suggested management strategy. Skull Base Rep. 2011;1(2):71-82.PubMedGoogle ScholarCrossref 3. Bian LG, Sun QF, Zhao WG, Shen JK, Tirakotai W, Bertalanffy H. Temporal bone chondroblastoma: a review. Neuropathology. 2005;25(2):159-164.PubMedGoogle ScholarCrossref 4. Cheng S, Ng T, Gomes L, da Cruz M. Temporal bone chondroblastoma. Otol Neurotol. 2009;30(5):688-689.PubMedGoogle ScholarCrossref 5. Hong SM, Park YK, Ro JY. Chondroblastoma of the temporal bone: a clinicopathologic study of five cases. J Korean Med Sci. 1999;14(5):559-564.PubMedGoogle Scholar 6. Selesnick SH, Levine JM. Chondroblastoma of the temporal bone: consistent middle fossa involvement. Skull Base Surg. 1999;9(4):301-305.PubMedGoogle ScholarCrossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png JAMA Otolaryngology - Head & Neck Surgery American Medical Association

Unilateral Ear and Temporomandibular Joint Discomfort

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Publisher
American Medical Association
Copyright
Copyright © 2014 American Medical Association. All Rights Reserved.
ISSN
2168-6181
eISSN
2168-619X
DOI
10.1001/jamaoto.2014.1472
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Abstract

Case A woman in her 30s presented with right-sided otalgia, otorrhea, decreased hearing, and worsening right temporomandibular joint (TMJ) pain. Her medical history was unremarkable. Physical examination revealed bilateral TMJ tenderness with crepitus on the right. Examination of the right ear revealed a polyp and granulation tissue in the anterior-superior external auditory canal (EAC) that was mobile with jaw movement and a normal-appearing tympanic membrane. An audiogram showed normal hearing. Computed tomographic (CT) imaging revealed dehiscence of the right glenoid fossa, erosion of the anterior EAC, TMJ, and middle cranial fossa (Figure, A). Magnetic resonance imaging (MRI) revealed a heterogeneous 8.5 × 7-mm mass based in the air cells at the root of zygoma, posterior to the right TMJ, with extension into the osseous external canal. The lesion displayed intermediate signal intensity on T1- and T2-weighting and enhanced with gadolinium administration (Figure, B). Positron emission tomographic (PET)-CT imaging ruled out distant metastases. Figure. View LargeDownload A, Computed tomographic image revealing dehiscence of the right glenoid fossa, erosion of the anterior external auditory canal, temporomandibular joint, and middle cranial fossa. The arrow indicates soft-tissue mass with bony erosion noted anteriorly into glenoid fossa and superiorly approaching the cranial vault. B, Magnetic resonance image (MRI) of the lesion displaying intermediate signal intensity on T1- and T2-weighting and enhanced with gadolinium administration. The arrow indicates coronal T1-weighted MRI postgadolinium with fat saturation revealing avid enhancement of soft-tissue mass superior and posterior to glenoid fossa. C, Multinucleated cells dispersed in a somewhat lobular pattern (hematoxylin-eosin, original magnification ×40). An in-office biopsy of the ear canal tissue was performed, the histologic findings indicated a giant cell granuloma-like lesion with osteogenesis. Adjacent to this were regions of keratin debris and squamous mucosa with an area of cartilage displaying prominent mononuclear morphologic characteristics. A lateral temporal bone resection with en bloc removal of the mass was performed. Gross analysis of the tissue revealed an ill-defined, semitranslucent 0.9 × 0.5 × 0.4-cm mass. Histopathologic findings revealed chondromyxoid proliferation with abundant multinucleated giant cells and prominent admixed mononuclear cells. The mononuclear cells contained central nuclear grooves and were associated with chondroid matrix. The multinucleated cells were dispersed in a somewhat lobular pattern (Figure, C). What is your diagnosis? Read the Discussion. Discussion Diagnosis Chondroblastoma of the temporal bone Discussion Chondroblastoma is a rare, benign neoplasm comprising less than 1% of primary bone tumors. Localization to the temporal bone is similarly infrequent, with most tumors localized to the epiphyses of the long bones and pelvis.1,2 Chondroblastoma of the skull base, particularly the temporal bone, is a rare occurrence, with a 2011 review2 reporting 81 cases. The diagnosis can often be challenging owing to ambiguous clinical findings. The differential diagnosis for chondroblastoma of the temporal bone includes cholesteatoma, giant-cell tumor, cholesterol granuloma, aneurysmal bone cyst, and chondrosarcoma.3 This rare neoplasm presents with a wide range of symptoms. A review of the current literature conducted by Reid et al2 reported that the most common presenting symptoms of chondroblastoma are hearing loss (49%), cranial nerve involvement (43.2%), facial swelling (22.2%), and otalgia (19.8%). Other symptoms include tinnitus, aural fullness, and disturbance of equilibrium. Owing to the ambiguous clinical presentation, complimentary studies are necessary for accurate diagnosis. Classic histologic findings include chondromyxoid proliferation with embedded osteoclast-like giant cells. Cells are often arranged in a lobular fashion and may reveal calcification in a “chicken-wire” fashion dispersed within chondroid and endochondral bone formations.3,4 This was consistent with our biopsy result. Reactive changes may be apparent with portions of aneurysmal bone cyst or reparative granuloma formation. Tumor cells have been reported to exhibit eccentrically localized nuclei often revealing a “grooved” appearance with absence of atypical mitotic figures.3,5 Immunoreactivity to S-100 protein, indicating cartilaginous origin, has been described as a useful adjunct in narrowing the differential between chondroblastoma vs giant-cell tumor, as giant-cell tumor does not produce chondroid matrix.3-5 These factors reinforce the importance of integrating the characteristic radiographic findings when establishing the diagnosis. Histologic findings are supported by CT and MRI studies. The CT studies may reveal a lobulated, expansile soft-tissue mass with foci of calcification and heterogeneous contrast enhancement. Magnetic resonance imaging offers superior visualization to assess for dural or brain tissue involvement and reveal heterogeneous gadolinium enhancement throughout the mass. As such, current recommendations for imaging evaluation recommend both CT and MRI assessment.2,3 One study6 reported a notable finding that 76% of temporal bone chondroblastoma lesions exhibit erosion into the EAC. Dural involvement was less common, with 4 of 21 cases describing dural adhesion. A dural biopsy was performed by the neurosurgery unit in our patient, and the results confirmed that there was no involvement on histologic analysis. Owing to the inability to rule out a malignant process in the initial stages of evaluation, however, imaging with PET-CT was also performed to assess for chondrosarcoma or alternative malignant lesion. PET-CT may be valuable in the initial workup when considering malignant disease, although it may not be necessary once the diagnosis of chondroblastoma, an inherently benign process, has been established. Surgical treatment historically included approaches ranging from simple curettage, irradiation, or en bloc resection.2 Chondroblastoma may display local recurrence rates nearing 20%, and reports of transformation to chondrosarcoma following radiation therapy have led to the recommendation for surgical treatment via en bloc resection without radiotherapy.2,3 Owing to the potential for recurrence, the need for long-term monitoring and follow-up is implicit and establishes the basis for the lateral temporal bone resection with en bloc removal performed in this case. The patient had no morbidity owing to the procedure, and her conductive hearing loss was rehabilitated with a bone conduction hearing device. In sum, chondroblastoma of the temporal bone is a rare neoplastic lesion with ambiguous clinical findings. A strong multidisciplinary approach to include neuroradiologic review and characteristic histopathologic analysis support the diagnosis. Complete en bloc excision without the use of radiotherapy is the recommended surgical intervention and is considered curative, with a necessity for long-term monitoring. Back to top Article Information Corresponding Author: Alexander Lanigan, MD, Captain, Medical Corps, United States Army, San Antonio Military Medical Center, 3551 Roger Brooks Dr, San Antonio, TX 78234 (alexander.e.lanigan.mil@mail.mil) (Alexander.lanigan@usuhs.edu). Published Online: August 14, 2014. doi:10.1001/jamaoto.2014.1472. Conflict of Interest Disclosures: None reported. Additional Contributions: Andrew J. Fishman, MD, of Cadence Neuroscience Institute, provided clinical expertise and editorial support in preparing this case for publication. Section Editor: Edward B. Stelow, MD. References 1. Dahlin DC, Ivins JC. Benign chondroblastoma: a study of 125 cases. Cancer. 1972;30(2):401-413.PubMedGoogle ScholarCrossref 2. Reid LB, Wong DS, Lyons B. Chondroblastoma of the temporal bone: a case series, review, and suggested management strategy. Skull Base Rep. 2011;1(2):71-82.PubMedGoogle ScholarCrossref 3. Bian LG, Sun QF, Zhao WG, Shen JK, Tirakotai W, Bertalanffy H. Temporal bone chondroblastoma: a review. Neuropathology. 2005;25(2):159-164.PubMedGoogle ScholarCrossref 4. Cheng S, Ng T, Gomes L, da Cruz M. Temporal bone chondroblastoma. Otol Neurotol. 2009;30(5):688-689.PubMedGoogle ScholarCrossref 5. Hong SM, Park YK, Ro JY. Chondroblastoma of the temporal bone: a clinicopathologic study of five cases. J Korean Med Sci. 1999;14(5):559-564.PubMedGoogle Scholar 6. Selesnick SH, Levine JM. Chondroblastoma of the temporal bone: consistent middle fossa involvement. Skull Base Surg. 1999;9(4):301-305.PubMedGoogle ScholarCrossref

Journal

JAMA Otolaryngology - Head & Neck SurgeryAmerican Medical Association

Published: Sep 1, 2014

Keywords: ear,temporomandibular joint

References