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A blue middle ear mass: Cholesterol granuloma mimicking a glomus tumor and endolymphatic sac tumor
A Blue Middle Ear Mass: Cholesterol Granuloma Mimicking a Glomus Tumor and Endolymphatic Sac Tumor Clara Olcott M.D., Barry Strasnick M.D., FACS PII: DOI: Reference:
S0196-0709(16)30167-3 doi: 10.1016/j.amjoto.2016.09.013 YAJOT 1753
To appear in:
American Journal of Otolaryngology–Head and Neck Medicine and Surgery
Received date:
31 July 2016
Please cite this article as: Olcott Clara, Strasnick Barry, A Blue Middle Ear Mass: Cholesterol Granuloma Mimicking a Glomus Tumor and Endolymphatic Sac Tumor, American Journal of Otolaryngology–Head and Neck Medicine and Surgery (2016), doi: 10.1016/j.amjoto.2016.09.013
This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT Title: A Blue Middle Ear Mass: Cholesterol Granuloma Mimicking a Glomus Tumor and Endolymphatic Sac Tumor Authors: Clara Olcott, M.D., Barry Strasnick, M.D., FACS
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Abstract
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Cholesterol granuloma (CG) is the most common benign lesion of the petrous apex, however, it can grow significantly large and become destructive causing a diagnostic dilemma. This case presents a 25year-old female with 2-year history of left-sided progressive and profound hearing loss, a transient leftsided facial paralysis and cranial nerve 10 palsy who presented with a blue middle ear mass. Her diagnosis did not become apparent until direct visualization intraoperatively. The objective of this case study is to highlight the destructive capabilities of CG and the importance to keep it in the differential diagnosis of a large, erosive, expansile skull base lesion in order to avoid overly aggressive resection or other unnecessary treatment. Case Description
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25-year-old female with 2-year history of progressive left-sided profound hearing loss and left facial paralysis. Her past medical history was significant for hypertension but no family history of hearing loss or other genetic disease.
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Physical exam revealed anacusis of left ear with a reddish-blue lesion in the hypotympanum extending to the posterosuperior quadrant. Right ear was normal. She also had House-Brackmann III/IV left facial paralysis, diminished gag reflex and left true vocal fold paresis. Audiogram showed profound sensorineural hearing loss on left with type B tympanogram. CT temporal bone (Fig. 1) and MRI (Fig. 2-3) below demonstrated a 4-cm skull base lesion centered at the jugular foramen invading the labyrinth, cochlear and endolymphatic sac with extension to the foramen magnum and middle ear (ME) space. This lesion was markedly hyperintense on both T1 and T2 signals without gadolinium enhancement. The internal jugular vein was intimately involved with the mass on MRA/V which suggested the possibility of a glomus jugulare tumor. Differential diagnosis includes cholesterol granuloma, glomus tumor and endolymphatic sac tumor (ELST). Preoperative angiography was considered but it was not performed. Patient was taken to the operating room in conjunction with neurosurgery team for resection of the petrous apex tumor with possible translabyrinthe versus transcochlear versus foramen magnum approach. She underwent a translabyrinthe approach resection of the mass. Intraoperatively, the cystic mass was seen posterior to the posterior semicircular canal, replacing the endolymphatic sac and the entire petrous apex (Fig. 4). Chocolate-brown fluid was aspirated and the lesion was decompressed along with removal of the cyst wall (Fig. 5). A tubed silastic sheet was placed within the cystic cavity to prevent accumulation of the CG. Discussion Cholesterol granuloma (CG) is the most common cystic lesion of the petrous apex1. It is an intraosseous inflammatory cyst with chocolate-brown fluid secondary to blood degradation products from intralesional hemorrhage2. Its pathogenesis remains controversial. The two main hypotheses include the obstruction-vacuum theory and exposed marrow theory3. In the obstruction-vacuum theory, mucosal edema of chronic otitis media and eustachian tube dysfunction result in interruption of the air circulation of the air cells. Resorption of the trapped gas leads to a vacuum seal which in turn causes
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bleeding. However, the degree of pneumatization seen in CG cases and the rarity of CG compared to common obstructive conditions contradict this theory. On the other hand, the exposed-marrow theory describes the replacement of red marrow during the process of pneumatization. When the exposed marrow bleeds and coagulates within the mucosal lining, it causes obstruction and leads to progressive cyst expansion. CG has been described as “blue dome cyst” or “blue dome eardrum” when it is seen within ME or tympanic membrane4. It is hyperintense on both T1 and T2 sequences on MRI.
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Endolymphtic sac tumor (ELST) typically enhances on CT. It is hyperintense on MRI T1 with gadolinium enhancement in the non-cystic portion. It is also more heterogeneous on MRI T2 due to hemorrhage. Glomus tumor is hypointense on MRI T1 and enhances with gadolinium. MRI T2 shows hyperintense, hypervascular and flow voids which gives a salt-and-pepper appearance.
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The large size of this tumor with invasion to multiple structures including endolymphatic sac, labyrinth, cochlear, foramen magnum and ME extension created a diagnostic dilemma. A reddish or blue ME “mass” often suggests a diagnosis of a vascular lesion, such as glomus tympanicum or jugulare. ELST is seen in 11-15% of all Von-Hippel-Landau (VHL) patients while 60% of VHL patients with vestibular symptoms have ELST5.
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Preoperative angiography with possible embolization was considered in this case as in treatment for a highly vascular glomus tumor. More aggressive surgical approaches were planned but were not deemed necessary once the diagnosis was confirmed. Conclusion
References
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Important to keep CG in the differential diagnosis of a large, erosive, expansile skull base lesion to avoid overly aggressive surgery or unnecessary treatment. Simple removal of the cyst wall and drainage with ventilation rather than complete excision is often sufficient. Patient will need to be monitored closely for recurrence.
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1. Muckle RP, De la Cruz A, Lo WM. Petrous apex lesions. Am J Otol. 1998(19):219-25. 2. Isaacson B, Kutz JW, Roland PS. Lesions of the petrous apex: diagnosis and management. Oto Clin N Am. 2007(40):479-519. 3. Jackler RK, Cho M. A new theory to explain the genesis of petrous apex cholesterol granuloma. Otol Neurotol 2003. Jan;24(1)96-106. 4. Jang CH1, Kim JS, Cho YB. Cholesterol granuloma of the tympanic membrane presenting as a blue eardrum. Yonsei Med J. 2009 Aug 31;50(4):585-7. 5. Lonser RR, Kim HJ, Butman JA et-al. Tumors of the endolymphatic sac in von Hippel-Lindau disease. N. Engl. J. Med. 2004;350 (24): 2481-6.
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Fig. 1-3. CT on left. MRI T1 with fat suppression and gadolinium (middle) and T2 (right) showed a 4 cm expansile lesion with bone destruction and hyperintensity on both T1 and T2 sequence.
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Figure 4
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Figure 5
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S0196-0709(16)30167-3 doi: 10.1016/j.amjoto.2016.09.013 YAJOT 1753
To appear in:
American Journal of Otolaryngology–Head and Neck Medicine and Surgery
Received date:
31 July 2016
Please cite this article as: Olcott Clara, Strasnick Barry, A Blue Middle Ear Mass: Cholesterol Granuloma Mimicking a Glomus Tumor and Endolymphatic Sac Tumor, American Journal of Otolaryngology–Head and Neck Medicine and Surgery (2016), doi: 10.1016/j.amjoto.2016.09.013
This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT Title: A Blue Middle Ear Mass: Cholesterol Granuloma Mimicking a Glomus Tumor and Endolymphatic Sac Tumor Authors: Clara Olcott, M.D., Barry Strasnick, M.D., FACS
T
Abstract
NU
SC
RI P
Cholesterol granuloma (CG) is the most common benign lesion of the petrous apex, however, it can grow significantly large and become destructive causing a diagnostic dilemma. This case presents a 25year-old female with 2-year history of left-sided progressive and profound hearing loss, a transient leftsided facial paralysis and cranial nerve 10 palsy who presented with a blue middle ear mass. Her diagnosis did not become apparent until direct visualization intraoperatively. The objective of this case study is to highlight the destructive capabilities of CG and the importance to keep it in the differential diagnosis of a large, erosive, expansile skull base lesion in order to avoid overly aggressive resection or other unnecessary treatment. Case Description
MA
25-year-old female with 2-year history of progressive left-sided profound hearing loss and left facial paralysis. Her past medical history was significant for hypertension but no family history of hearing loss or other genetic disease.
AC
CE
PT
ED
Physical exam revealed anacusis of left ear with a reddish-blue lesion in the hypotympanum extending to the posterosuperior quadrant. Right ear was normal. She also had House-Brackmann III/IV left facial paralysis, diminished gag reflex and left true vocal fold paresis. Audiogram showed profound sensorineural hearing loss on left with type B tympanogram. CT temporal bone (Fig. 1) and MRI (Fig. 2-3) below demonstrated a 4-cm skull base lesion centered at the jugular foramen invading the labyrinth, cochlear and endolymphatic sac with extension to the foramen magnum and middle ear (ME) space. This lesion was markedly hyperintense on both T1 and T2 signals without gadolinium enhancement. The internal jugular vein was intimately involved with the mass on MRA/V which suggested the possibility of a glomus jugulare tumor. Differential diagnosis includes cholesterol granuloma, glomus tumor and endolymphatic sac tumor (ELST). Preoperative angiography was considered but it was not performed. Patient was taken to the operating room in conjunction with neurosurgery team for resection of the petrous apex tumor with possible translabyrinthe versus transcochlear versus foramen magnum approach. She underwent a translabyrinthe approach resection of the mass. Intraoperatively, the cystic mass was seen posterior to the posterior semicircular canal, replacing the endolymphatic sac and the entire petrous apex (Fig. 4). Chocolate-brown fluid was aspirated and the lesion was decompressed along with removal of the cyst wall (Fig. 5). A tubed silastic sheet was placed within the cystic cavity to prevent accumulation of the CG. Discussion Cholesterol granuloma (CG) is the most common cystic lesion of the petrous apex1. It is an intraosseous inflammatory cyst with chocolate-brown fluid secondary to blood degradation products from intralesional hemorrhage2. Its pathogenesis remains controversial. The two main hypotheses include the obstruction-vacuum theory and exposed marrow theory3. In the obstruction-vacuum theory, mucosal edema of chronic otitis media and eustachian tube dysfunction result in interruption of the air circulation of the air cells. Resorption of the trapped gas leads to a vacuum seal which in turn causes
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bleeding. However, the degree of pneumatization seen in CG cases and the rarity of CG compared to common obstructive conditions contradict this theory. On the other hand, the exposed-marrow theory describes the replacement of red marrow during the process of pneumatization. When the exposed marrow bleeds and coagulates within the mucosal lining, it causes obstruction and leads to progressive cyst expansion. CG has been described as “blue dome cyst” or “blue dome eardrum” when it is seen within ME or tympanic membrane4. It is hyperintense on both T1 and T2 sequences on MRI.
SC
Endolymphtic sac tumor (ELST) typically enhances on CT. It is hyperintense on MRI T1 with gadolinium enhancement in the non-cystic portion. It is also more heterogeneous on MRI T2 due to hemorrhage. Glomus tumor is hypointense on MRI T1 and enhances with gadolinium. MRI T2 shows hyperintense, hypervascular and flow voids which gives a salt-and-pepper appearance.
MA
NU
The large size of this tumor with invasion to multiple structures including endolymphatic sac, labyrinth, cochlear, foramen magnum and ME extension created a diagnostic dilemma. A reddish or blue ME “mass” often suggests a diagnosis of a vascular lesion, such as glomus tympanicum or jugulare. ELST is seen in 11-15% of all Von-Hippel-Landau (VHL) patients while 60% of VHL patients with vestibular symptoms have ELST5.
ED
Preoperative angiography with possible embolization was considered in this case as in treatment for a highly vascular glomus tumor. More aggressive surgical approaches were planned but were not deemed necessary once the diagnosis was confirmed. Conclusion
References
CE
PT
Important to keep CG in the differential diagnosis of a large, erosive, expansile skull base lesion to avoid overly aggressive surgery or unnecessary treatment. Simple removal of the cyst wall and drainage with ventilation rather than complete excision is often sufficient. Patient will need to be monitored closely for recurrence.
AC
1. Muckle RP, De la Cruz A, Lo WM. Petrous apex lesions. Am J Otol. 1998(19):219-25. 2. Isaacson B, Kutz JW, Roland PS. Lesions of the petrous apex: diagnosis and management. Oto Clin N Am. 2007(40):479-519. 3. Jackler RK, Cho M. A new theory to explain the genesis of petrous apex cholesterol granuloma. Otol Neurotol 2003. Jan;24(1)96-106. 4. Jang CH1, Kim JS, Cho YB. Cholesterol granuloma of the tympanic membrane presenting as a blue eardrum. Yonsei Med J. 2009 Aug 31;50(4):585-7. 5. Lonser RR, Kim HJ, Butman JA et-al. Tumors of the endolymphatic sac in von Hippel-Lindau disease. N. Engl. J. Med. 2004;350 (24): 2481-6.
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Fig. 1-3. CT on left. MRI T1 with fat suppression and gadolinium (middle) and T2 (right) showed a 4 cm expansile lesion with bone destruction and hyperintensity on both T1 and T2 sequence.
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Figure 4
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Figure 5
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