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Recurrent paraganglioma of Meckel's cave: Case report and a review of anatomic origin of paragangliomas.

Prajsnar A, Balak N, Walter GF, Stan AC, Deinsberger W, Tapul L, Bayindir C - Surg Neurol Int (2011)

Bottom Line: A lateral suboccipital approach to the lesion was used under neuronavigational guidance.Five months later, the follow-up MRI showed local regrowth, which required subsequent surgical intervention.Another peculiarity of the case is the local recurrence of the tumor in a relatively short time despite an attempted, almost gross total resection.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Neurosurgery, Klinikum Kassel, Kassel, Germany.

ABSTRACT

Background: Paragangliomas are rare, usually benign tumors of neural crest origin. They account for only 0.6% of all head and neck tumors. In the craniocervical area, they are more common in the carotid body and tympanico-jugular regions. To the authors' knowledge, a case of paraganglioma in Meckel's cave has not yet been reported in the medical literature. The pathogenesis and natural history of paragangliomas are still not well understood. We present a case of recurrent paraganglioma in Meckel's cave.

Case description: A 53-year-old woman was diagnosed with trigeminal neuralgia, dysesthesia and hypoesthesia on the left side of the face, hearing disturbance and a history of chronic, persistent temporal headaches. Magnetic resonance imaging (MRI) showed a lesion located in Meckel's cave on the left side, extending to the posterior cranial fossa and compressing the left cerebral peduncle. The lesion was first thought to be a recurrence of an atypical meningioma, as the pathologist described it in the tissue specimen resected 3 years earlier, and a decision for re-operation was made. A lateral suboccipital approach to the lesion was used under neuronavigational guidance. The tumor was removed, and histological examination proved the lesion to be a paraganglioma. Five months later, the follow-up MRI showed local regrowth, which required subsequent surgical intervention.

Conclusions: A paraganglioma in Meckel's cave is an uncommon tumor in this location. Although ectopic paragangliomas have been described in the literature, a paraganglioma atypically located in Meckel's cave makes a topographic correlation difficult, mainly because paraganglionic cells are usually not found in Meckel's cave. Another peculiarity of the case is the local recurrence of the tumor in a relatively short time despite an attempted, almost gross total resection.

No MeSH data available.


Related in: MedlinePlus

(a) The first manifestation of the tumor. The axial and coronal T1-weighted, contrast-enhanced MR scan shows a strongly enhanced lesion in the Meckel's cave. The large tumor is compressing the prepontine cistern, the pontocerebellar cistern and the pons. A clear border among the tumor, trigeminal nerve and the internal cerebral artery is indistinguishable. (b) Pre-operative axial T1-weighted MRI with contrast media revealing the first reoccurrence of the tumor in the left Meckel's cave. The volume of the tumor in the medial fossa is approximately 2.5 × 1.8 cm. Regressive changes and cystic compartment with liquid mirror image are visible within the lesion. Part of the tumor is located in the posterior fossa; its volume is 1.3 × 1.6 cm. The left trigeminal nerve inside the tumor is no longer distinguishable. Additionally, a compression of the pons as well as the left cerebral peduncle in the ponto-mesencephalic junction can be seen. The part of the tumor in the middle fossa has contact with the C2–C5 segments of the left internal carotid artery
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Figure 1: (a) The first manifestation of the tumor. The axial and coronal T1-weighted, contrast-enhanced MR scan shows a strongly enhanced lesion in the Meckel's cave. The large tumor is compressing the prepontine cistern, the pontocerebellar cistern and the pons. A clear border among the tumor, trigeminal nerve and the internal cerebral artery is indistinguishable. (b) Pre-operative axial T1-weighted MRI with contrast media revealing the first reoccurrence of the tumor in the left Meckel's cave. The volume of the tumor in the medial fossa is approximately 2.5 × 1.8 cm. Regressive changes and cystic compartment with liquid mirror image are visible within the lesion. Part of the tumor is located in the posterior fossa; its volume is 1.3 × 1.6 cm. The left trigeminal nerve inside the tumor is no longer distinguishable. Additionally, a compression of the pons as well as the left cerebral peduncle in the ponto-mesencephalic junction can be seen. The part of the tumor in the middle fossa has contact with the C2–C5 segments of the left internal carotid artery

Mentions: A 53-year-old woman was admitted to the clinic with a 2-month history of trigeminal neuralgia, dysesthesia and hypoesthesia in the left side of the face, combined with a lengthy history of persistent temporal headaches and a progressive hearing problem. An MRI revealed a tumor in the left Meckel's cave, with partial compression of the trigeminal nerve and extension to the posterior cranial fossa [Figure 1a]. A temporobasal craniotomy was performed and the lesion was completely removed. The histological pattern of the specimen was classified initially as an atypical meningioma (WHO grade II) in 2005. Two years later, in 2007, a follow-up MRI showed a contrast-enhanced lesion, a seemingly recurrent meningioma mass in Meckel's cave. A local stereotactic radiotherapy (10 Χ 4 Gy) was applied. However, the patient was re-admitted to the hospital 1 year later, in 2008, because of progressive clinical disturbance and increased volume of the lesion seen in follow-up MRI scans [Figure 1b], and a decision for re-operation was made. A left lateral suboccipital approach was used under neuronavigational guidance. The tumor was almost completely removed. The histopathologic examination of the specimen [Figure 2a–f] showed fragments of well-organized, compacted and nested epitheloid cells of the tumor dispersed in lobules (Zellballen architecture, composed of nests of polygonal chief cells enclosed by trabecula of fibrous and sustentacular elongated cells) which were histologically and immunohistochemically classified as a paraganglioma (WHO grade I). A comparative histopathologic reinvestigation of the tumor specimen surgically removed during the first operation gave the result that the first tumor was a paraganglioma too, not an atypical meningioma. Five months after the second surgical intervention, in 2009, another local recurrence of the tumor was diagnosed in a follow-up MRI [Figure 3]. Consequently, the tumor was removed and again classified as a paraganglioma (WHO grade I).


Recurrent paraganglioma of Meckel's cave: Case report and a review of anatomic origin of paragangliomas.

Prajsnar A, Balak N, Walter GF, Stan AC, Deinsberger W, Tapul L, Bayindir C - Surg Neurol Int (2011)

(a) The first manifestation of the tumor. The axial and coronal T1-weighted, contrast-enhanced MR scan shows a strongly enhanced lesion in the Meckel's cave. The large tumor is compressing the prepontine cistern, the pontocerebellar cistern and the pons. A clear border among the tumor, trigeminal nerve and the internal cerebral artery is indistinguishable. (b) Pre-operative axial T1-weighted MRI with contrast media revealing the first reoccurrence of the tumor in the left Meckel's cave. The volume of the tumor in the medial fossa is approximately 2.5 × 1.8 cm. Regressive changes and cystic compartment with liquid mirror image are visible within the lesion. Part of the tumor is located in the posterior fossa; its volume is 1.3 × 1.6 cm. The left trigeminal nerve inside the tumor is no longer distinguishable. Additionally, a compression of the pons as well as the left cerebral peduncle in the ponto-mesencephalic junction can be seen. The part of the tumor in the middle fossa has contact with the C2–C5 segments of the left internal carotid artery
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3108444&req=5

Figure 1: (a) The first manifestation of the tumor. The axial and coronal T1-weighted, contrast-enhanced MR scan shows a strongly enhanced lesion in the Meckel's cave. The large tumor is compressing the prepontine cistern, the pontocerebellar cistern and the pons. A clear border among the tumor, trigeminal nerve and the internal cerebral artery is indistinguishable. (b) Pre-operative axial T1-weighted MRI with contrast media revealing the first reoccurrence of the tumor in the left Meckel's cave. The volume of the tumor in the medial fossa is approximately 2.5 × 1.8 cm. Regressive changes and cystic compartment with liquid mirror image are visible within the lesion. Part of the tumor is located in the posterior fossa; its volume is 1.3 × 1.6 cm. The left trigeminal nerve inside the tumor is no longer distinguishable. Additionally, a compression of the pons as well as the left cerebral peduncle in the ponto-mesencephalic junction can be seen. The part of the tumor in the middle fossa has contact with the C2–C5 segments of the left internal carotid artery
Mentions: A 53-year-old woman was admitted to the clinic with a 2-month history of trigeminal neuralgia, dysesthesia and hypoesthesia in the left side of the face, combined with a lengthy history of persistent temporal headaches and a progressive hearing problem. An MRI revealed a tumor in the left Meckel's cave, with partial compression of the trigeminal nerve and extension to the posterior cranial fossa [Figure 1a]. A temporobasal craniotomy was performed and the lesion was completely removed. The histological pattern of the specimen was classified initially as an atypical meningioma (WHO grade II) in 2005. Two years later, in 2007, a follow-up MRI showed a contrast-enhanced lesion, a seemingly recurrent meningioma mass in Meckel's cave. A local stereotactic radiotherapy (10 Χ 4 Gy) was applied. However, the patient was re-admitted to the hospital 1 year later, in 2008, because of progressive clinical disturbance and increased volume of the lesion seen in follow-up MRI scans [Figure 1b], and a decision for re-operation was made. A left lateral suboccipital approach was used under neuronavigational guidance. The tumor was almost completely removed. The histopathologic examination of the specimen [Figure 2a–f] showed fragments of well-organized, compacted and nested epitheloid cells of the tumor dispersed in lobules (Zellballen architecture, composed of nests of polygonal chief cells enclosed by trabecula of fibrous and sustentacular elongated cells) which were histologically and immunohistochemically classified as a paraganglioma (WHO grade I). A comparative histopathologic reinvestigation of the tumor specimen surgically removed during the first operation gave the result that the first tumor was a paraganglioma too, not an atypical meningioma. Five months after the second surgical intervention, in 2009, another local recurrence of the tumor was diagnosed in a follow-up MRI [Figure 3]. Consequently, the tumor was removed and again classified as a paraganglioma (WHO grade I).

Bottom Line: A lateral suboccipital approach to the lesion was used under neuronavigational guidance.Five months later, the follow-up MRI showed local regrowth, which required subsequent surgical intervention.Another peculiarity of the case is the local recurrence of the tumor in a relatively short time despite an attempted, almost gross total resection.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Neurosurgery, Klinikum Kassel, Kassel, Germany.

ABSTRACT

Background: Paragangliomas are rare, usually benign tumors of neural crest origin. They account for only 0.6% of all head and neck tumors. In the craniocervical area, they are more common in the carotid body and tympanico-jugular regions. To the authors' knowledge, a case of paraganglioma in Meckel's cave has not yet been reported in the medical literature. The pathogenesis and natural history of paragangliomas are still not well understood. We present a case of recurrent paraganglioma in Meckel's cave.

Case description: A 53-year-old woman was diagnosed with trigeminal neuralgia, dysesthesia and hypoesthesia on the left side of the face, hearing disturbance and a history of chronic, persistent temporal headaches. Magnetic resonance imaging (MRI) showed a lesion located in Meckel's cave on the left side, extending to the posterior cranial fossa and compressing the left cerebral peduncle. The lesion was first thought to be a recurrence of an atypical meningioma, as the pathologist described it in the tissue specimen resected 3 years earlier, and a decision for re-operation was made. A lateral suboccipital approach to the lesion was used under neuronavigational guidance. The tumor was removed, and histological examination proved the lesion to be a paraganglioma. Five months later, the follow-up MRI showed local regrowth, which required subsequent surgical intervention.

Conclusions: A paraganglioma in Meckel's cave is an uncommon tumor in this location. Although ectopic paragangliomas have been described in the literature, a paraganglioma atypically located in Meckel's cave makes a topographic correlation difficult, mainly because paraganglionic cells are usually not found in Meckel's cave. Another peculiarity of the case is the local recurrence of the tumor in a relatively short time despite an attempted, almost gross total resection.

No MeSH data available.


Related in: MedlinePlus