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From the Archives of the AFIP¹

Paragangliomas of the Head and Neck: Radiologic-Pathologic Correlation

¹ From the Departments of Radiologic Pathology (A.B.R., K.K.K.) and Otolaryngic and Endocrine Pathology (C.F.A.), Armed Forces Institute of Pathology, 14th St at Alaska Ave, Bldg 54, Rm M-121 Washington, DC 20306-6000 and the Departments of Radiology and Nuclear Medicine (K.K.K.) and Pathology (C.F.A.), Uniformed Services University of the Health Sciences, Bethesda, Md. Received May 5, 1999; revision requested May 20 and received June 21; accepted June 21. Address reprint requests to K.K.K.

View larger version (144K): [in a new window]   Figure 1.   Drawing illustrates the most common locations of paraganglia of the head and neck. The carotid body paraganglion is a discrete mass found at the CCA bifurcation. Other locations include along the path of the vagus nerve, the jugular foramen, and the middle ear. The paraganglia tend to occur along the path of either vessels or nerves. (Reprinted from reference 3.)

View larger version (149K): [in a new window]   Figure 2.   Drawing illustrates the sites of paraganglia of the skull base. Jugulotympanic paraganglia are located near the jugular bulb. The vagal paraganglia may arise at the jugular ganglion, at the nodose ganglion, or along the path of the vagus nerve (within the perineurium, between the nerve fascicles, or within the nerve itself). (Reprinted from reference 3.)

View larger version (125K): [in a new window]   Figure 3.   Diagram shows the major nerves and vessels of the jugular fossa. The inferior tympanic branch, or Jacobson nerve (J), of the glossopharyngeal nerve (IX) arises from and travels anterior to the IJV (JV) toward the middle ear, where it lies against the cochlear promontory. The auricular branch, or Arnold nerve (A), of the vagus nerve (X) follows a path posterior to the IJV on its way to the facial canal. Paraganglia are distributed along the course of these two nerves. Both nerves may give rise to paragangliomas. With current terminology, those residing in the middle ear and mastoid are called glomus tympanicum tumors, whereas those arising from the jugular foramen and adjacent skull base are called glomus jugulare tumors. XI = spinal accessory nerve, VII = facial nerve. (Reprinted, with permission, from reference 20.)

View larger version (161K): [in a new window]   Figure 4a.   Bilateral carotid body tumors in a 40-year-old man with progressive bilateral neck swelling, dysphagia, and dyspnea. (a) Sagittal T1-weighted MR image shows a left-sided neck mass (m) that is isointense relative to muscle at the level of the common carotid bifurcation. The ECA (short arrow) is splayed from the ICA (long arrow). An additional component of the mass (*) extended inferiorly. (b) Different sagittal T1-weighted MR image reveals numerous flow voids (arrowheads) throughout the right-sided neck mass (m). (c) Axial T1-weighted MR image demonstrates bilateral carotid space masses (m). c = right CCA, i = left ICA, v = IJV, arrow = ECA.

View larger version (161K): [in a new window]   Figure 4b.   Bilateral carotid body tumors in a 40-year-old man with progressive bilateral neck swelling, dysphagia, and dyspnea. (a) Sagittal T1-weighted MR image shows a left-sided neck mass (m) that is isointense relative to muscle at the level of the common carotid bifurcation. The ECA (short arrow) is splayed from the ICA (long arrow). An additional component of the mass (*) extended inferiorly. (b) Different sagittal T1-weighted MR image reveals numerous flow voids (arrowheads) throughout the right-sided neck mass (m). (c) Axial T1-weighted MR image demonstrates bilateral carotid space masses (m). c = right CCA, i = left ICA, v = IJV, arrow = ECA.

View larger version (151K): [in a new window]   Figure 4c.   Bilateral carotid body tumors in a 40-year-old man with progressive bilateral neck swelling, dysphagia, and dyspnea. (a) Sagittal T1-weighted MR image shows a left-sided neck mass (m) that is isointense relative to muscle at the level of the common carotid bifurcation. The ECA (short arrow) is splayed from the ICA (long arrow). An additional component of the mass (*) extended inferiorly. (b) Different sagittal T1-weighted MR image reveals numerous flow voids (arrowheads) throughout the right-sided neck mass (m). (c) Axial T1-weighted MR image demonstrates bilateral carotid space masses (m). c = right CCA, i = left ICA, v = IJV, arrow = ECA.

View larger version (121K): [in a new window]   Figure 5.   Bilateral vagal paragangliomas in a 58-year-old man with asymptomatic suprahyoid neck masses. Contrast material-enhanced axial CT scan shows heterogeneous bilateral carotid space masses (m) with displacement of the parapharyngeal fat (arrows) anteriorly. The styloid processes are indicated by arrowheads. Preoperative embolization could not be performed secondary to vasospasm. At surgery, only the left vagal paraganglioma was removed following uneventful ligation of the left ICA.

View larger version (221K): [in a new window]   Figure 6a.   Paraganglioma with typical histologic features. (a) High-power photomicrograph (original magnification, x200; reticulin stain) shows the characteristic zellballen (cell ball) growth pattern. The reticulin stain accentuates the delicate fibrovascular network (primarily collagen and capillaries) that surrounds each "ball" (arrows) of chief cells. (b) High-power photomicrograph (original magnification, x300; hematoxylin-eosin stain) reveals a sea of chief cells with abundant granular cytoplasm. Some degree of nuclear pleomorphism is often present. Two nuclei with different morphology are indicated (arrow and arrowhead). The zellballen pattern is very inconspicuous in a routine hematoxylin-eosin stained section and better demonstrated by immunohistochemical techniques.

View larger version (229K): [in a new window]   Figure 6b.   Paraganglioma with typical histologic features. (a) High-power photomicrograph (original magnification, x200; reticulin stain) shows the characteristic zellballen (cell ball) growth pattern. The reticulin stain accentuates the delicate fibrovascular network (primarily collagen and capillaries) that surrounds each "ball" (arrows) of chief cells. (b) High-power photomicrograph (original magnification, x300; hematoxylin-eosin stain) reveals a sea of chief cells with abundant granular cytoplasm. Some degree of nuclear pleomorphism is often present. Two nuclei with different morphology are indicated (arrow and arrowhead). The zellballen pattern is very inconspicuous in a routine hematoxylin-eosin stained section and better demonstrated by immunohistochemical techniques.

View larger version (213K): [in a new window]   Figure 7a.   Two cell types seen in a paraganglioma. (a) High-power photomicrograph (original magnification, x400; S-100 protein stain) shows sustentacular cells with prominent brownish stain found at the periphery of the cell balls of chief cells. Two such cells are indicated by the arrows. (b) High-power photomicrograph (original magnification, x400; chromogranin stain) reveals brownish stain (arrows) of chromogranin in the cytoplasm of chief cells. The nuclei of the chief cells remain bluish.

View larger version (217K): [in a new window]   Figure 7b.   Two cell types seen in a paraganglioma. (a) High-power photomicrograph (original magnification, x400; S-100 protein stain) shows sustentacular cells with prominent brownish stain found at the periphery of the cell balls of chief cells. Two such cells are indicated by the arrows. (b) High-power photomicrograph (original magnification, x400; chromogranin stain) reveals brownish stain (arrows) of chromogranin in the cytoplasm of chief cells. The nuclei of the chief cells remain bluish.

View larger version (147K): [in a new window]   Figure 8a.   Carotid body tumor in a 67-year-old woman with a slowly growing, left-sided neck mass for several years and recent onset of left ear tinnitus. (a) Contrast-enhanced axial CT image demonstrates an intensely enhancing left carotid space mass (m) that splays the ECA (short arrow) from the ICA (long arrow). (b) Lateral angiographic view obtained after a left CCA injection reveals splaying of the ECA from the ICA by a hypervascular mass (arrows) that extends to the bifurcation. (c) Lateral angiographic view obtained after a selective left ascending pharyngeal artery injection reveals the hypervascular mass (arrows) with primary vascular supply from this artery.

View larger version (149K): [in a new window]   Figure 8b.   Carotid body tumor in a 67-year-old woman with a slowly growing, left-sided neck mass for several years and recent onset of left ear tinnitus. (a) Contrast-enhanced axial CT image demonstrates an intensely enhancing left carotid space mass (m) that splays the ECA (short arrow) from the ICA (long arrow). (b) Lateral angiographic view obtained after a left CCA injection reveals splaying of the ECA from the ICA by a hypervascular mass (arrows) that extends to the bifurcation. (c) Lateral angiographic view obtained after a selective left ascending pharyngeal artery injection reveals the hypervascular mass (arrows) with primary vascular supply from this artery.

View larger version (131K): [in a new window]   Figure 8c.   Carotid body tumor in a 67-year-old woman with a slowly growing, left-sided neck mass for several years and recent onset of left ear tinnitus. (a) Contrast-enhanced axial CT image demonstrates an intensely enhancing left carotid space mass (m) that splays the ECA (short arrow) from the ICA (long arrow). (b) Lateral angiographic view obtained after a left CCA injection reveals splaying of the ECA from the ICA by a hypervascular mass (arrows) that extends to the bifurcation. (c) Lateral angiographic view obtained after a selective left ascending pharyngeal artery injection reveals the hypervascular mass (arrows) with primary vascular supply from this artery.

View larger version (149K): [in a new window]   Figure 9a.   Carotid body tumor in a 63-year-old woman with a slowly enlarging, left-sided neck mass over a 2-year period. (a) Contrast-enhanced axial CT image obtained at the level of the hyoid bone (partially seen secondary to asymmetry of the patient in the scanner) reveals a heterogeneously enhancing mass (m) within the left carotid space. (b) Contrast-enhanced axial CT image of the suprahyoid neck shows the mass (m) extending superiorly within the left carotid space. (c) Photograph of the gross specimen shows the smooth surface and thin capsule of the carotid body tumor. Numerous vessels feed the tumor along the capsular surface.

View larger version (146K): [in a new window]   Figure 9b.   Carotid body tumor in a 63-year-old woman with a slowly enlarging, left-sided neck mass over a 2-year period. (a) Contrast-enhanced axial CT image obtained at the level of the hyoid bone (partially seen secondary to asymmetry of the patient in the scanner) reveals a heterogeneously enhancing mass (m) within the left carotid space. (b) Contrast-enhanced axial CT image of the suprahyoid neck shows the mass (m) extending superiorly within the left carotid space. (c) Photograph of the gross specimen shows the smooth surface and thin capsule of the carotid body tumor. Numerous vessels feed the tumor along the capsular surface.

View larger version (111K): [in a new window]   Figure 9c.   Carotid body tumor in a 63-year-old woman with a slowly enlarging, left-sided neck mass over a 2-year period. (a) Contrast-enhanced axial CT image obtained at the level of the hyoid bone (partially seen secondary to asymmetry of the patient in the scanner) reveals a heterogeneously enhancing mass (m) within the left carotid space. (b) Contrast-enhanced axial CT image of the suprahyoid neck shows the mass (m) extending superiorly within the left carotid space. (c) Photograph of the gross specimen shows the smooth surface and thin capsule of the carotid body tumor. Numerous vessels feed the tumor along the capsular surface.

View larger version (150K): [in a new window]   Figure 10a.   Vagal paraganglioma in a 29-year-old woman with an asymptomatic, painless neck mass for several years that recently enlarged. (a) Contrast-enhanced axial CT image reveals intense enhancement of a left carotid space mass (m). (b) Lateral angiographic view obtained with a left CCA injection demonstrates the hypervascular mass (arrow) displacing both the ECA and ICA anteriorly.

View larger version (133K): [in a new window]   Figure 10b.   Vagal paraganglioma in a 29-year-old woman with an asymptomatic, painless neck mass for several years that recently enlarged. (a) Contrast-enhanced axial CT image reveals intense enhancement of a left carotid space mass (m). (b) Lateral angiographic view obtained with a left CCA injection demonstrates the hypervascular mass (arrow) displacing both the ECA and ICA anteriorly.

View larger version (151K): [in a new window]   Figure 11a.   Vagal paraganglioma in a 30-year-old woman with left-sided neck swelling and a pulsatile mass at physical examination. (a) Contrast-enhanced CT image shows mild heterogeneous enhancement of a large left carotid space mass (m). (b) Contrast-enhanced CT image shows inferior extension of the mass (m) to the level of the hyoid bone.

View larger version (147K): [in a new window]   Figure 11b.   Vagal paraganglioma in a 30-year-old woman with left-sided neck swelling and a pulsatile mass at physical examination. (a) Contrast-enhanced CT image shows mild heterogeneous enhancement of a large left carotid space mass (m). (b) Contrast-enhanced CT image shows inferior extension of the mass (m) to the level of the hyoid bone.

View larger version (147K): [in a new window]   Figure 12a.   Vagal paraganglioma in a 46-year-old man with a nontender, slowly enlarging, right-sided neck mass. (a) Contrast-enhanced axial CT image shows a well-defined right carotid space mass (m) with smooth margins and enhancement. (b) Lateral angiographic view obtained after an ECA injection reveals an enlarged ascending pharyngeal artery as the primary supply to the hypervascular mass (arrow). (c) Another lateral angiographic view from the same injection in the later arterial phase shows an intense blush more prominent in the superior half of the mass (arrow).

View larger version (140K): [in a new window]   Figure 12b.   Vagal paraganglioma in a 46-year-old man with a nontender, slowly enlarging, right-sided neck mass. (a) Contrast-enhanced axial CT image shows a well-defined right carotid space mass (m) with smooth margins and enhancement. (b) Lateral angiographic view obtained after an ECA injection reveals an enlarged ascending pharyngeal artery as the primary supply to the hypervascular mass (arrow). (c) Another lateral angiographic view from the same injection in the later arterial phase shows an intense blush more prominent in the superior half of the mass (arrow).

View larger version (142K): [in a new window]   Figure 12c.   Vagal paraganglioma in a 46-year-old man with a nontender, slowly enlarging, right-sided neck mass. (a) Contrast-enhanced axial CT image shows a well-defined right carotid space mass (m) with smooth margins and enhancement. (b) Lateral angiographic view obtained after an ECA injection reveals an enlarged ascending pharyngeal artery as the primary supply to the hypervascular mass (arrow). (c) Another lateral angiographic view from the same injection in the later arterial phase shows an intense blush more prominent in the superior half of the mass (arrow).

View larger version (154K): [in a new window]   Figure 13a.   Glomus jugulare tumor in a 56-year-old man with positional vertigo, left sensorineural hearing loss, and paresis of the left facial and hypoglossal nerves. (a) Axial CT image (bone window) shows a left jugular foramen mass (m) with mildly irregular margins. (b) Coronal CT image (bone window) reveals the mass (m) with irregularity along the superior rim. (c) Coronal CT image (bone window) shows soft tissue extending into the left middle ear (arrow). (d) Contrast-enhanced axial T1-weighted MR image shows the enhancing mass (arrow) of the left jugular foramen.

View larger version (123K): [in a new window]   Figure 13b.   Glomus jugulare tumor in a 56-year-old man with positional vertigo, left sensorineural hearing loss, and paresis of the left facial and hypoglossal nerves. (a) Axial CT image (bone window) shows a left jugular foramen mass (m) with mildly irregular margins. (b) Coronal CT image (bone window) reveals the mass (m) with irregularity along the superior rim. (c) Coronal CT image (bone window) shows soft tissue extending into the left middle ear (arrow). (d) Contrast-enhanced axial T1-weighted MR image shows the enhancing mass (arrow) of the left jugular foramen.

View larger version (113K): [in a new window]   Figure 13c.   Glomus jugulare tumor in a 56-year-old man with positional vertigo, left sensorineural hearing loss, and paresis of the left facial and hypoglossal nerves. (a) Axial CT image (bone window) shows a left jugular foramen mass (m) with mildly irregular margins. (b) Coronal CT image (bone window) reveals the mass (m) with irregularity along the superior rim. (c) Coronal CT image (bone window) shows soft tissue extending into the left middle ear (arrow). (d) Contrast-enhanced axial T1-weighted MR image shows the enhancing mass (arrow) of the left jugular foramen.

View larger version (152K): [in a new window]   Figure 13d.   Glomus jugulare tumor in a 56-year-old man with positional vertigo, left sensorineural hearing loss, and paresis of the left facial and hypoglossal nerves. (a) Axial CT image (bone window) shows a left jugular foramen mass (m) with mildly irregular margins. (b) Coronal CT image (bone window) reveals the mass (m) with irregularity along the superior rim. (c) Coronal CT image (bone window) shows soft tissue extending into the left middle ear (arrow). (d) Contrast-enhanced axial T1-weighted MR image shows the enhancing mass (arrow) of the left jugular foramen.

View larger version (124K): [in a new window]   Figure 14a.   Glomus jugulare tumor in a 44-year-old woman with headaches, visual loss in the right eye, diplopia, and periorbital pain. (a) Contrast-enhanced coronal CT image shows a large infratemporal fossa mass (m) with soft tissue extending into the right middle ear (arrow). (b) Contrast-enhanced coronal CT image (bone window) demonstrates extensive skull base destruction and extension of the mass into the middle cranial fossa. Numerous calcifications are scattered throughout the mass.

View larger version (141K): [in a new window]   Figure 14b.   Glomus jugulare tumor in a 44-year-old woman with headaches, visual loss in the right eye, diplopia, and periorbital pain. (a) Contrast-enhanced coronal CT image shows a large infratemporal fossa mass (m) with soft tissue extending into the right middle ear (arrow). (b) Contrast-enhanced coronal CT image (bone window) demonstrates extensive skull base destruction and extension of the mass into the middle cranial fossa. Numerous calcifications are scattered throughout the mass.

View larger version (136K): [in a new window]   Figure 15a.   Glomus jugulare tumor in a 75-year-old woman with progressive right hearing loss, tinnitus, and right ear pruritus. Deficits involving cranial nerves VII-XII of the right side were documented at physical examination. (a) Axial CT image (bone window) reveals an expanding mass (m) and lytic changes of the right temporal bone (arrows). Soft tissue extends into the middle ear and external auditory canal (arrowheads). (b) Axial CT image (bone window) shows extensive destruction of the right temporal bone centered in the jugular fossa (arrows). Soft-tissue attenuation is present within the mastoid air cells and middle ear secondary to inflammatory change.

View larger version (136K): [in a new window]   Figure 15b.   Glomus jugulare tumor in a 75-year-old woman with progressive right hearing loss, tinnitus, and right ear pruritus. Deficits involving cranial nerves VII-XII of the right side were documented at physical examination. (a) Axial CT image (bone window) reveals an expanding mass (m) and lytic changes of the right temporal bone (arrows). Soft tissue extends into the middle ear and external auditory canal (arrowheads). (b) Axial CT image (bone window) shows extensive destruction of the right temporal bone centered in the jugular fossa (arrows). Soft-tissue attenuation is present within the mastoid air cells and middle ear secondary to inflammatory change.

View larger version (130K): [in a new window]   Figure 16a.   Glomus jugulotympanicum tumor in a 67-year-old woman with pulsatile tinnitus, episodic dizziness, and left hearing loss. At otoscopic examination, a reddish purple mass was seen filling the left middle ear; conductive hearing loss was also documented. (a) Axial CT image (bone window) shows a soft-tissue mass (arrowheads) filling the hypotympanum of the left middle ear. There is erosion (arrow) of the anterolateral segment of the jugular fossa wall. Soft-tissue attenuation fills the left mastoid air cells secondary to inflammatory change. (b) Axial T1-weighted MR image reveals soft-tissue signal intensity within the middle ear (arrow) and inflammatory change within the left mastoid air spaces. (c) Axial T2-weighted MR image shows hyperintensity of the lesion (arrow) and mastoid air spaces. (d) Coronal T1-weighted MR image demonstrates the soft-tissue mass (arrow). (e) Contrast-enhanced coronal T1-weighted MR image shows intense enhancement of the mass (arrow). (f) Photograph shows resected fragments of the glomus tumor, which enveloped the malleus and incus and was dissected away from the stapes, which was left in situ. A partial ossicular replacement prosthesis was inserted at the end of the operation.

View larger version (139K): [in a new window]   Figure 16b.   Glomus jugulotympanicum tumor in a 67-year-old woman with pulsatile tinnitus, episodic dizziness, and left hearing loss. At otoscopic examination, a reddish purple mass was seen filling the left middle ear; conductive hearing loss was also documented. (a) Axial CT image (bone window) shows a soft-tissue mass (arrowheads) filling the hypotympanum of the left middle ear. There is erosion (arrow) of the anterolateral segment of the jugular fossa wall. Soft-tissue attenuation fills the left mastoid air cells secondary to inflammatory change. (b) Axial T1-weighted MR image reveals soft-tissue signal intensity within the middle ear (arrow) and inflammatory change within the left mastoid air spaces. (c) Axial T2-weighted MR image shows hyperintensity of the lesion (arrow) and mastoid air spaces. (d) Coronal T1-weighted MR image demonstrates the soft-tissue mass (arrow). (e) Contrast-enhanced coronal T1-weighted MR image shows intense enhancement of the mass (arrow). (f) Photograph shows resected fragments of the glomus tumor, which enveloped the malleus and incus and was dissected away from the stapes, which was left in situ. A partial ossicular replacement prosthesis was inserted at the end of the operation.

View larger version (123K): [in a new window]   Figure 16c.   Glomus jugulotympanicum tumor in a 67-year-old woman with pulsatile tinnitus, episodic dizziness, and left hearing loss. At otoscopic examination, a reddish purple mass was seen filling the left middle ear; conductive hearing loss was also documented. (a) Axial CT image (bone window) shows a soft-tissue mass (arrowheads) filling the hypotympanum of the left middle ear. There is erosion (arrow) of the anterolateral segment of the jugular fossa wall. Soft-tissue attenuation fills the left mastoid air cells secondary to inflammatory change. (b) Axial T1-weighted MR image reveals soft-tissue signal intensity within the middle ear (arrow) and inflammatory change within the left mastoid air spaces. (c) Axial T2-weighted MR image shows hyperintensity of the lesion (arrow) and mastoid air spaces. (d) Coronal T1-weighted MR image demonstrates the soft-tissue mass (arrow). (e) Contrast-enhanced coronal T1-weighted MR image shows intense enhancement of the mass (arrow). (f) Photograph shows resected fragments of the glomus tumor, which enveloped the malleus and incus and was dissected away from the stapes, which was left in situ. A partial ossicular replacement prosthesis was inserted at the end of the operation.

View larger version (124K): [in a new window]   Figure 16d.   Glomus jugulotympanicum tumor in a 67-year-old woman with pulsatile tinnitus, episodic dizziness, and left hearing loss. At otoscopic examination, a reddish purple mass was seen filling the left middle ear; conductive hearing loss was also documented. (a) Axial CT image (bone window) shows a soft-tissue mass (arrowheads) filling the hypotympanum of the left middle ear. There is erosion (arrow) of the anterolateral segment of the jugular fossa wall. Soft-tissue attenuation fills the left mastoid air cells secondary to inflammatory change. (b) Axial T1-weighted MR image reveals soft-tissue signal intensity within the middle ear (arrow) and inflammatory change within the left mastoid air spaces. (c) Axial T2-weighted MR image shows hyperintensity of the lesion (arrow) and mastoid air spaces. (d) Coronal T1-weighted MR image demonstrates the soft-tissue mass (arrow). (e) Contrast-enhanced coronal T1-weighted MR image shows intense enhancement of the mass (arrow). (f) Photograph shows resected fragments of the glomus tumor, which enveloped the malleus and incus and was dissected away from the stapes, which was left in situ. A partial ossicular replacement prosthesis was inserted at the end of the operation.

View larger version (133K): [in a new window]   Figure 16e.   Glomus jugulotympanicum tumor in a 67-year-old woman with pulsatile tinnitus, episodic dizziness, and left hearing loss. At otoscopic examination, a reddish purple mass was seen filling the left middle ear; conductive hearing loss was also documented. (a) Axial CT image (bone window) shows a soft-tissue mass (arrowheads) filling the hypotympanum of the left middle ear. There is erosion (arrow) of the anterolateral segment of the jugular fossa wall. Soft-tissue attenuation fills the left mastoid air cells secondary to inflammatory change. (b) Axial T1-weighted MR image reveals soft-tissue signal intensity within the middle ear (arrow) and inflammatory change within the left mastoid air spaces. (c) Axial T2-weighted MR image shows hyperintensity of the lesion (arrow) and mastoid air spaces. (d) Coronal T1-weighted MR image demonstrates the soft-tissue mass (arrow). (e) Contrast-enhanced coronal T1-weighted MR image shows intense enhancement of the mass (arrow). (f) Photograph shows resected fragments of the glomus tumor, which enveloped the malleus and incus and was dissected away from the stapes, which was left in situ. A partial ossicular replacement prosthesis was inserted at the end of the operation.

View larger version (142K): [in a new window]   Figure 16f.   Glomus jugulotympanicum tumor in a 67-year-old woman with pulsatile tinnitus, episodic dizziness, and left hearing loss. At otoscopic examination, a reddish purple mass was seen filling the left middle ear; conductive hearing loss was also documented. (a) Axial CT image (bone window) shows a soft-tissue mass (arrowheads) filling the hypotympanum of the left middle ear. There is erosion (arrow) of the anterolateral segment of the jugular fossa wall. Soft-tissue attenuation fills the left mastoid air cells secondary to inflammatory change. (b) Axial T1-weighted MR image reveals soft-tissue signal intensity within the middle ear (arrow) and inflammatory change within the left mastoid air spaces. (c) Axial T2-weighted MR image shows hyperintensity of the lesion (arrow) and mastoid air spaces. (d) Coronal T1-weighted MR image demonstrates the soft-tissue mass (arrow). (e) Contrast-enhanced coronal T1-weighted MR image shows intense enhancement of the mass (arrow). (f) Photograph shows resected fragments of the glomus tumor, which enveloped the malleus and incus and was dissected away from the stapes, which was left in situ. A partial ossicular replacement prosthesis was inserted at the end of the operation.

View larger version (150K): [in a new window]   Figure 17a.   Vagal paraganglioma in a 48-year-old man with an asymptomatic left-sided neck mass. (a) Sagittal T1-weighted MR image shows a mildly heterogeneous mass (m) with numerous flow voids (arrowheads), indicating its hypervascular nature. It displaces the flow voids of both the ECA and ICA anteriorly. (b) Lateral angiographic view obtained after a left CCA injection demonstrates the hypervascular mass (arrows). (c) Photograph of the resected gross specimen shows the mass with its smooth outer capsule. (d) Photograph of the cut gross specimen reveals multiple hemorrhagic foci within the mass. Scale is in centimeters.

View larger version (147K): [in a new window]   Figure 17b.   Vagal paraganglioma in a 48-year-old man with an asymptomatic left-sided neck mass. (a) Sagittal T1-weighted MR image shows a mildly heterogeneous mass (m) with numerous flow voids (arrowheads), indicating its hypervascular nature. It displaces the flow voids of both the ECA and ICA anteriorly. (b) Lateral angiographic view obtained after a left CCA injection demonstrates the hypervascular mass (arrows). (c) Photograph of the resected gross specimen shows the mass with its smooth outer capsule. (d) Photograph of the cut gross specimen reveals multiple hemorrhagic foci within the mass. Scale is in centimeters.

View larger version (123K): [in a new window]   Figure 17c.   Vagal paraganglioma in a 48-year-old man with an asymptomatic left-sided neck mass. (a) Sagittal T1-weighted MR image shows a mildly heterogeneous mass (m) with numerous flow voids (arrowheads), indicating its hypervascular nature. It displaces the flow voids of both the ECA and ICA anteriorly. (b) Lateral angiographic view obtained after a left CCA injection demonstrates the hypervascular mass (arrows). (c) Photograph of the resected gross specimen shows the mass with its smooth outer capsule. (d) Photograph of the cut gross specimen reveals multiple hemorrhagic foci within the mass. Scale is in centimeters.

View larger version (151K): [in a new window]   Figure 17d.   Vagal paraganglioma in a 48-year-old man with an asymptomatic left-sided neck mass. (a) Sagittal T1-weighted MR image shows a mildly heterogeneous mass (m) with numerous flow voids (arrowheads), indicating its hypervascular nature. It displaces the flow voids of both the ECA and ICA anteriorly. (b) Lateral angiographic view obtained after a left CCA injection demonstrates the hypervascular mass (arrows). (c) Photograph of the resected gross specimen shows the mass with its smooth outer capsule. (d) Photograph of the cut gross specimen reveals multiple hemorrhagic foci within the mass. Scale is in centimeters.

View larger version (143K): [in a new window]   Figure 18a.   Vagal paraganglioma in a 46-year-old woman with vocal cord dysfunction, left hemiparesis, dysphagia, and left-sided neck and shoulder pain. (a) Sagittal T1-weighted MR image reveals a mass (m) isointense relative to muscle extending from the jugular fossa (arrow) to the carotid space inferiorly. The ICA (arrowhead) is displaced anteriorly by the mass. (b) Axial T1-weighted MR image shows the mass (m) extending into the left carotid space.

View larger version (152K): [in a new window]   Figure 18b.   Vagal paraganglioma in a 46-year-old woman with vocal cord dysfunction, left hemiparesis, dysphagia, and left-sided neck and shoulder pain. (a) Sagittal T1-weighted MR image reveals a mass (m) isointense relative to muscle extending from the jugular fossa (arrow) to the carotid space inferiorly. The ICA (arrowhead) is displaced anteriorly by the mass. (b) Axial T1-weighted MR image shows the mass (m) extending into the left carotid space.

View larger version (140K): [in a new window]   Figure 19a.   Vagal paraganglioma in a 35-year-old woman with a left supraclavicular mass, left vocal cord paralysis, and a several year history of voice changes. (a) Coronal T1-weighted MR image shows a homogeneous soft-tissue mass (m) of the left supraclavicular region extending through the thoracic inlet. (b) Axial T1-weighted MR image reveals the mass (m) within the carotid space surrounded by several flow voids (arrowheads), producing a salt-and-pepper appearance. (c) Contrast-enhanced axial CT image demonstrates mild central enhancement within the mass (m).

View larger version (138K): [in a new window]   Figure 19b.   Vagal paraganglioma in a 35-year-old woman with a left supraclavicular mass, left vocal cord paralysis, and a several year history of voice changes. (a) Coronal T1-weighted MR image shows a homogeneous soft-tissue mass (m) of the left supraclavicular region extending through the thoracic inlet. (b) Axial T1-weighted MR image reveals the mass (m) within the carotid space surrounded by several flow voids (arrowheads), producing a salt-and-pepper appearance. (c) Contrast-enhanced axial CT image demonstrates mild central enhancement within the mass (m).

View larger version (103K): [in a new window]   Figure 19c.   Vagal paraganglioma in a 35-year-old woman with a left supraclavicular mass, left vocal cord paralysis, and a several year history of voice changes. (a) Coronal T1-weighted MR image shows a homogeneous soft-tissue mass (m) of the left supraclavicular region extending through the thoracic inlet. (b) Axial T1-weighted MR image reveals the mass (m) within the carotid space surrounded by several flow voids (arrowheads), producing a salt-and-pepper appearance. (c) Contrast-enhanced axial CT image demonstrates mild central enhancement within the mass (m).

View larger version (162K): [in a new window]   Figure 20a.   Glomus jugulare tumor in a 42-year-old woman with hoarseness, right true vocal cord paralysis, and absent gag reflex. (a) Axial T1-weighted MR image shows a soft-tissue mass (m) within the enlarged right jugular fossa. (b) Axial T2-weighted MR image reveals mild hyperintensity of the mass (arrow). (c) Contrast-enhanced axial T1-weighted MR image demonstrates intense enhancement of the lesion (arrow). (d) Contrast-enhanced coronal T1-weighted MR image shows that the lesion (arrow) is confined to the jugular fossa region without extensive spread into the carotid space inferiorly. At surgery, the mass was found to extend into the middle ear and to surround the ICA and cranial nerves X-XII within the jugular fossa.

View larger version (147K): [in a new window]   Figure 20b.   Glomus jugulare tumor in a 42-year-old woman with hoarseness, right true vocal cord paralysis, and absent gag reflex. (a) Axial T1-weighted MR image shows a soft-tissue mass (m) within the enlarged right jugular fossa. (b) Axial T2-weighted MR image reveals mild hyperintensity of the mass (arrow). (c) Contrast-enhanced axial T1-weighted MR image demonstrates intense enhancement of the lesion (arrow). (d) Contrast-enhanced coronal T1-weighted MR image shows that the lesion (arrow) is confined to the jugular fossa region without extensive spread into the carotid space inferiorly. At surgery, the mass was found to extend into the middle ear and to surround the ICA and cranial nerves X-XII within the jugular fossa.

View larger version (158K): [in a new window]   Figure 20c.   Glomus jugulare tumor in a 42-year-old woman with hoarseness, right true vocal cord paralysis, and absent gag reflex. (a) Axial T1-weighted MR image shows a soft-tissue mass (m) within the enlarged right jugular fossa. (b) Axial T2-weighted MR image reveals mild hyperintensity of the mass (arrow). (c) Contrast-enhanced axial T1-weighted MR image demonstrates intense enhancement of the lesion (arrow). (d) Contrast-enhanced coronal T1-weighted MR image shows that the lesion (arrow) is confined to the jugular fossa region without extensive spread into the carotid space inferiorly. At surgery, the mass was found to extend into the middle ear and to surround the ICA and cranial nerves X-XII within the jugular fossa.

View larger version (166K): [in a new window]   Figure 20d.   Glomus jugulare tumor in a 42-year-old woman with hoarseness, right true vocal cord paralysis, and absent gag reflex. (a) Axial T1-weighted MR image shows a soft-tissue mass (m) within the enlarged right jugular fossa. (b) Axial T2-weighted MR image reveals mild hyperintensity of the mass (arrow). (c) Contrast-enhanced axial T1-weighted MR image demonstrates intense enhancement of the lesion (arrow). (d) Contrast-enhanced coronal T1-weighted MR image shows that the lesion (arrow) is confined to the jugular fossa region without extensive spread into the carotid space inferiorly. At surgery, the mass was found to extend into the middle ear and to surround the ICA and cranial nerves X-XII within the jugular fossa.