Rare case of extracranial chordoid meningioma adjacent to the carotid sheath: illustrative case

Seung W. Jeong Departments of Neurosurgery and

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Michael Moran Drexel University College of Medicine, Philadelphia, Pennsylvania

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Shahed Elhamdani Departments of Neurosurgery and

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Dorian M. Kusyk Departments of Neurosurgery and

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Chen Xu Departments of Neurosurgery and

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Kymberly Gyure Pathology, Allegheny Health Network, Pittsburgh, Pennsylvania; and

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Richard Williamson Departments of Neurosurgery and

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BACKGROUND

Chordoid meningioma is a rare World Health Organization (WHO) grade 2 variant of meningioma with histological features resembling those of a chordoma. This tumor type is known for having an aggressive clinical course with a propensity for local recurrence. Most cases occur within the cranium, more specifically around the cerebral convexities. Although extracranial meningiomas of various subtypes have been documented, extracranial meningioma with a chordoid subtype is an extremely rare entity.

OBSERVATIONS

The authors herein report the case of a 51-year-old female who presented with a chief complaint of dysphagia and was found to have a neck mass abutting the carotid sheath. The patient underwent resection and final pathology results revealed a WHO grade 2 chordoid meningioma.

LESSONS

This case report demonstrates an atypical case of an extracranial chordoid meningioma adjacent to the carotid sheath. To the authors’ knowledge, this is the first reported case of a chordoid meningioma occurring within the soft tissue of the neck.

ABBREVIATIONS

CNS = central nervous system; CTA = computed tomography angiography; EMA = epithelial membrane antigen; MRI = magnetic resonance imaging; SCM = sternocleidomastoid; WHO = World Health Organization

BACKGROUND

Chordoid meningioma is a rare World Health Organization (WHO) grade 2 variant of meningioma with histological features resembling those of a chordoma. This tumor type is known for having an aggressive clinical course with a propensity for local recurrence. Most cases occur within the cranium, more specifically around the cerebral convexities. Although extracranial meningiomas of various subtypes have been documented, extracranial meningioma with a chordoid subtype is an extremely rare entity.

OBSERVATIONS

The authors herein report the case of a 51-year-old female who presented with a chief complaint of dysphagia and was found to have a neck mass abutting the carotid sheath. The patient underwent resection and final pathology results revealed a WHO grade 2 chordoid meningioma.

LESSONS

This case report demonstrates an atypical case of an extracranial chordoid meningioma adjacent to the carotid sheath. To the authors’ knowledge, this is the first reported case of a chordoid meningioma occurring within the soft tissue of the neck.

ABBREVIATIONS

CNS = central nervous system; CTA = computed tomography angiography; EMA = epithelial membrane antigen; MRI = magnetic resonance imaging; SCM = sternocleidomastoid; WHO = World Health Organization

Meningiomas are the most common benign tumors arising from within the central nervous system (CNS), comprising approximately 15% of intracranial and 25% of spinal tumors.1 In rare cases (∼1%), they may arise outside the CNS,2 and only 0.1% are thought to have metastatic seeding from an intracranial primary tumor.3 The World Health Organization (WHO) has defined 15 histological meningioma subtypes that fall into a 3-tier grading system that stratifies recurrence risk.4 Chordoid meningiomas are a rare grade 2 variant with regional histological patterns similar to chordomas and a high likelihood of recurrence.5

Here we present an unusual case of an extracranial chordoid meningioma adjacent to the carotid sheath in a patient with a history of whole-brain radiation therapy for childhood leukemia and multiple intracranial meningiomas.

Illustrative Case

A 51-year-old female presented to our clinic for evaluation and treatment of a growing neck mass. The mass had been first discovered incidentally on magnetic resonance imaging (MRI) approximately 2 years earlier but had become painful in the past year, prompting antibiotic treatment for presumed lymphadenitis by an outside physician. At the time of her presentation to our clinic, her main complaint was dysphagia. Her physical examination was unremarkable except for a firm and tender mass in the right neck just below the angle of the mandible. Her neurological examination was intact, including lower cranial nerve function. She did have a direct laryngoscopy performed, which demonstrated normal vocal cord function.

Medical History

Her medical history was notable for acute lymphoblastic leukemia treated with chemotherapy and whole-brain radiation approximately 45 years ago, as well as a history of multiple intracranial meningiomas. She had a posterior fossa meningioma that was resected approximately 15 years ago. Two years later a second meningioma emerged in the right temporal fossa with bony penetration and temporalis invasion. Resection of this WHO grade 2 chordoid meningioma and the involved temporal bone and temporalis muscle was performed. Unfortunately, the tumor recurred 2 years later, with extension into the orbital cavity. In order to prevent optic nerve damage, subtotal resection followed by radiosurgery was undertaken. Residual tumor burden in that area gradually expanded as multiple semi-discrete masses, which occupied the masticator space, pterygoids, and angle of the mandible. Simultaneously, 2 additional meningiomas appeared in the posterior peri-sagittal dura and planum sphenoidale. The former was treated with stereotactic radiosurgery and the latter was left untreated.

Imaging

Her neck mass first appeared on MRI performed for neuro-navigation purposes prior to radiosurgery for her temporal and sagittal sinus meningiomas. It was not contiguous with the tumor burden in the masticator space. Instead, it occupied the right parapharyngeal space posterior to the submandibular gland, abutting the carotid sheath. At that time, it was hypothesized to be a lymph node and measured 1.9 × 1.2 × 1.9 cm. This mass gradually enlarged and MRI and computed tomography angiography (CTA) performed just prior to the patient’s neck surgery demonstrated the same mass to be 4.3 × 3.4 × 2.9 cm with medial displacement of the right carotid artery at its bifurcation (Fig. 1).

FIG. 1.
FIG. 1.

Preoperative imaging of the neck mass. Left: Axial T1-weighted MRI with contrast showing a homogeneously enhancing right-sided lesion. Right: Axial CTA showing a right-sided mass displacing the external (thin arrow) and internal (thick arrow) carotid arteries medially.

Operation

The patient underwent resection of the neck mass. An incision was made along the SCM border below the angle of the mandible. Dissection was performed through the platysma and continued along a plane medial to the SCM until the mass was encountered. Using an operative microscope, the borders of the tumor were micro-dissected, separating it from surrounding tissues. The tumor was separated completely from the carotid sheath and removed en bloc. The patient recovered well and was discharged without complication on postoperative day 2.

Histological Report

Although the frozen histological specimens were consistent with paraganglioma, the final histopathological diagnosis was consistent with meningioma. Final analysis demonstrated EMA-positive neoplasm composed largely of nests and cords of cells with prominent vacuolated cytoplasm with focal whorl formation and abundant lymphoid infiltrate (Fig. 2). The Ki-67/MIB1 proliferative index was approximately 2%–5%, and no staining was apparent for chromogranin, synaptophysin, cytokeratin, CAM5.2, or S100. These findings are consistent with those of WHO grade 2 chordoid meningioma.

FIG. 2.
FIG. 2.

Histopathology of the neck mass. The neoplasm was associated with a prominent lymphoplasmacytic inflammatory infiltrate (A) and was composed of cords of cells with prominent vacuolated cytoplasm (B). It infiltrated the adjacent adipose tissue in the neck (C). Hematoxylin and eosin, original magnification ×100 (A and C), ×400 (B).

Discussion

The anatomical complexity of the carotid space allows for a great diversity of pathology. Masses arising at this location may include paragangliomas, nerve sheath tumors, lipomas, reactive lymph nodes, as well as meningiomas. Prior to surgery, known risk factors as well as imaging characteristics can be used to differentiate these masses. Additionally, as these masses enlarge, associated mass effects on neighboring structures can give clues to their cellular origins.6,7 For example, carotid body–derived masses that arise at the medial surface of the bifurcation tend to splay internal and external carotid arteries apart as they grow.8

Meningiomas, on the other hand, despite being the most common extra-axial neoplasm of the CNS, occur infrequently outside of the skull.2 These masses demonstrate significant variability in imaging characteristics, with 60% being hyperattenuating on computed tomography and 20% showing calcifications.8 On MRI, meningiomas are typically iso- to hypointense to gray matter on T1 and iso- to hyperintense on T2.8 They are also highly vascular tumors and tend to enhance homogenously.9 Finally, as these masses do not typically arise in the neck or carotid sheath, the effect of their growth in this region is less predictable, but would not necessarily be expected to cause splaying of internal and external carotids.

Observations

In this case, the neck mass was initially described on MRI as a 1.9 × 1.9–cm enhancing soft tissue mass and hypothesized to be a lymph node. The patient was even treated for lymphadenitis by an outside physician as the mass grew and became tender. Further characterization by CTA demonstrated medial displacement of both internal and external carotid arteries.

Although residual meningioma persisted in relatively close proximity to the neck mass (in the masticator space), the neck mass was clearly a separate entity, making its relationship to the residual tumor unclear. It is notable that the residual tumor burden in the masticator space, which was the product of an incomplete resection of an invasive meningioma that had gained access to the orbit and breached the temporal bone, shares the same histology (WHO grade 2 chordoid type) with the neck mass. There have been suggestions and reports of iatrogenic10,11 and traumatic12 seeding of extracranial meningiomas, which may represent a feasible means of tumor spread here.

Another, albeit less likely, possibility is that this mass metastasized on its own. The carotid sheath is an infrequent metastatic site,3,13–15 and truly metastatic meningiomas frequently display high-risk features,14 including high proliferative indices, which was not the case here (MIB1 2%–5%). Last, chordoid-type meningiomas are not frequently identified in surveys of metastatic meningiomas,3 except for a few instances where they were similarly found to recur in remote locations after surgery.16,17 Interestingly, 1 study reported that the overwhelming majority (93%) of all metastatic meningiomas in their series of 115 cases were found postsurgery.3 Whether this is simply a function of extending life or a consequence of traumatic seeding is not apparent.

It is also seemingly unlikely that this patient’s neck mass represents a primary extracranial meningioma. In 1 case series of 146 primary extracranial meningiomas, none were reported as chordoid type.18 In this study, the predominant subtypes were meningothelial (77.4%), atypical (7.5%), psammomatous (4.1%), and anaplastic (2.7%). These findings are similar to those of a second report on 231 primary extradural meningiomas, which found the meningothelial subtype to be most predominant and did not identify any cases of chordoid-type meningioma.2

The location of this patient’s mass in the carotid sheath, its chordoid subtype, and the emergence of multiple additional intracranial meningiomas are independently rare occurrences that are even more unusual when considered coincidentally. When the patient’s radiation history is taken into consideration, radiation to the cranium when incurred during treatment of childhood leukemia is highly associated with meningioma incidence later in life (∼20% of individuals; average onset, 25 years after exposure).6 Furthermore, the likelihood of multiple meningiomas increases from 0.6%–2.4% to 4.6%–29% or even higher.7 However, the influence of her radiation on the meningioma subtype or its extracranial location remains unclear as no associations have been drawn between radiation therapy and chordoid-type or extracranial meningiomas.

With regard to subtype, it appears that, although radiation-induced meningiomas are less frequently benign (76% vs 92%) and more frequently atypical (19% vs 7.2%),7,19 the overall proportion of major histological variants does not change significantly.7 With regard to tumor location, neither of 2 large case series on extracranial meningiomas reported patients with prior history of radiation,2,18 and studies of radiation-induced meningiomas did not report extracranial masses.6,7

Given the unique nature of this patient’s neck mass, predicting its recurrence risk is challenging. Its histological features carry the worst prognosis, with 1 study indicating a recurrence rate of 38% for chordoid-type meningiomas after 56 months of follow-up.16 Otherwise, the recurrence risk of extracranial meningiomas in 2 large case series was found to be approximately 25% after 36 and 174 months of follow-up.2,18 The influence of location on meningioma recurrence after resection of extradural meningiomas may be independent of histological subtype, however, and instead related to the possibility of complete resection.2 Because the carotid sheath is a relatively accessible location, this would positively influence the patient’s risk of recurrence, although to what extent is unclear.

Lessons

Chordoid meningiomas rarely occur in the neck and are rarely metastatic but have been reported at distant extracranial sites after intracranial surgery. This case report illustrates a rare case of an extracranial chordoid meningioma abutting the carotid artery at its bifurcation in a 51-year-old female who received whole-brain radiation therapy for childhood leukemia and had undergone surgery for multiple intracranial meningiomas. We hereby demonstrate the diagnostic challenge of an uncommon subtype of a relatively common intracranial pathology occurring in an unexpected extracranial location and the importance of awareness of such diagnosis in management of these tumors.

Disclosures

The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

Author Contributions

Conception and design: Jeong, Elhamdani, Williamson. Acquisition of data: Jeong, Xu, Gyure, Williamson. Analysis and interpretation of data: Jeong, Kusyk, Xu, Gyure, Williamson. Drafting of the article: Moran, Jeong. Critically revising the article: Moran, Jeong, Elhamdani, Kusyk, Gyure, Williamson. Reviewed submitted version of the manuscript: all authors. Approved the final version of the manuscript on behalf of all authors: Moran. Administrative/technical/material support: Jeong, Xu, Williamson. Study supervision: Elhamdani, Xu, Williamson.

References

  • 1

    Burger PC, Scheithauer BW. In: Tumors of the Central Nervous System: Atlas of Tumor Pathology. 2nd series. Armed Forces Institute of Pathology; 1993:169190.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 2

    Liu Y, Wang H, Shao H, Wang C. Primary extradural meningiomas in head: a report of 19 cases and review of literature. Int J Clin Exp Pathol. 2015;8(5):56245632.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 3

    Surov A, Gottschling S, Bolz J, et al. Distant metastases in meningioma: an underestimated problem. J Neurooncol. 2013;112(3):323327.

  • 4

    Louis DN, Perry A, Reifenberger G, et al. The 2016 World Health Organization classification of tumors of the central nervous system: a summary. Acta Neuropathol. 2016;131(6):803820.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 5

    Couce ME, Aker FV, Scheithauer BW. Chordoid meningioma: a clinicopathologic study of 42 cases. [published correction appears in Am J Surg Pathol 2000;24(9):1316-7]. Am J Surg Pathol. 2000;24(7):899905.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 6

    Banerjee J, Pääkkö E, Harila M, et al. Radiation-induced meningiomas: a shadow in the success story of childhood leukemia. Neuro Oncol. 2009;11(5):543549.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 7

    Al-Mefty O, Topsakal C, Pravdenkova S, Sawyer JR, Harrison MJ. Radiation-induced meningiomas: clinical, pathological, cytokinetic, and cytogenetic characteristics. J Neurosurg. 2004;100(6):10021013.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 8

    Chengazi HU, Bhatt AA. Pathology of the carotid space. Insights Imaging. 2019;10(1):21.

  • 9

    Watts J, Box G, Galvin A, Brotchie P, Trost N, Sutherland T. Magnetic resonance imaging of meningiomas: a pictorial review. Insights Imaging. 2014;5(1):113122.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 10

    Maddah G, Shabahang H, Zehi V, Sharifi Sistani N, Mashhadi Nejad H. Iatrogenic seeding of tumor cells in thigh soft tissue upon surgical removal of intracranial meningioma. Basic Clin Neurosci. 2016;7(2):159164.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 11

    Kamiya K, Inagawa T, Nagasako R. Malignant intraventricular meningioma with spinal metastasis through the cerebrospinal fluid. Surg Neurol. 1989;32(3):213218.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 12

    Suzuki H, Gilbert EF, Zimmermann B. Primary extracranial meningioma. Arch Pathol. 1967;84(2):202206.

  • 13

    Karasick JL, Mullan SF. A survey of metastatic meningiomas. J Neurosurg. 1974;40(2):206212.

  • 14

    Enam SA, Abdulrauf S, Mehta B, Malik GM, Mahmood A. Metastasis in meningioma. Acta Neurochir (Wien). 1996;138(10):11721178.

  • 15

    Slavin ML. Metastatic malignant meningioma. J Clin Neuroophthalmol. 1989;9(1):5559.

  • 16

    Jee TK, Jo KI, Seol HJ, Kong DS, Lee JI, Shin HJ. Clinical features and treatment outcome of chordoid meningiomas in a single institute. J Korean Neurosurg Soc. 2014;56(3):194199.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 17

    Dalle Ore CL, Magill ST, Yen AJ, et al. Meningioma metastases: incidence and proposed screening paradigm. J Neurosurg. 2019;132(5):14471455.

  • 18

    Rushing EJ, Bouffard JP, McCall S, et al. Primary extracranial meningiomas: an analysis of 146 cases. Head Neck Pathol. 2009;3(2):116130.

  • 19

    Musa BS, Pople IK, Cummins BH. Intracranial meningiomas following irradiation—a growing problem? Br J Neurosurg. 1995;9(5):629637.

  • Collapse
  • Expand
  • FIG. 1.

    Preoperative imaging of the neck mass. Left: Axial T1-weighted MRI with contrast showing a homogeneously enhancing right-sided lesion. Right: Axial CTA showing a right-sided mass displacing the external (thin arrow) and internal (thick arrow) carotid arteries medially.

  • FIG. 2.

    Histopathology of the neck mass. The neoplasm was associated with a prominent lymphoplasmacytic inflammatory infiltrate (A) and was composed of cords of cells with prominent vacuolated cytoplasm (B). It infiltrated the adjacent adipose tissue in the neck (C). Hematoxylin and eosin, original magnification ×100 (A and C), ×400 (B).

  • 1

    Burger PC, Scheithauer BW. In: Tumors of the Central Nervous System: Atlas of Tumor Pathology. 2nd series. Armed Forces Institute of Pathology; 1993:169190.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 2

    Liu Y, Wang H, Shao H, Wang C. Primary extradural meningiomas in head: a report of 19 cases and review of literature. Int J Clin Exp Pathol. 2015;8(5):56245632.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 3

    Surov A, Gottschling S, Bolz J, et al. Distant metastases in meningioma: an underestimated problem. J Neurooncol. 2013;112(3):323327.

  • 4

    Louis DN, Perry A, Reifenberger G, et al. The 2016 World Health Organization classification of tumors of the central nervous system: a summary. Acta Neuropathol. 2016;131(6):803820.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 5

    Couce ME, Aker FV, Scheithauer BW. Chordoid meningioma: a clinicopathologic study of 42 cases. [published correction appears in Am J Surg Pathol 2000;24(9):1316-7]. Am J Surg Pathol. 2000;24(7):899905.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 6

    Banerjee J, Pääkkö E, Harila M, et al. Radiation-induced meningiomas: a shadow in the success story of childhood leukemia. Neuro Oncol. 2009;11(5):543549.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 7

    Al-Mefty O, Topsakal C, Pravdenkova S, Sawyer JR, Harrison MJ. Radiation-induced meningiomas: clinical, pathological, cytokinetic, and cytogenetic characteristics. J Neurosurg. 2004;100(6):10021013.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 8

    Chengazi HU, Bhatt AA. Pathology of the carotid space. Insights Imaging. 2019;10(1):21.

  • 9

    Watts J, Box G, Galvin A, Brotchie P, Trost N, Sutherland T. Magnetic resonance imaging of meningiomas: a pictorial review. Insights Imaging. 2014;5(1):113122.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 10

    Maddah G, Shabahang H, Zehi V, Sharifi Sistani N, Mashhadi Nejad H. Iatrogenic seeding of tumor cells in thigh soft tissue upon surgical removal of intracranial meningioma. Basic Clin Neurosci. 2016;7(2):159164.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 11

    Kamiya K, Inagawa T, Nagasako R. Malignant intraventricular meningioma with spinal metastasis through the cerebrospinal fluid. Surg Neurol. 1989;32(3):213218.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 12

    Suzuki H, Gilbert EF, Zimmermann B. Primary extracranial meningioma. Arch Pathol. 1967;84(2):202206.

  • 13

    Karasick JL, Mullan SF. A survey of metastatic meningiomas. J Neurosurg. 1974;40(2):206212.

  • 14

    Enam SA, Abdulrauf S, Mehta B, Malik GM, Mahmood A. Metastasis in meningioma. Acta Neurochir (Wien). 1996;138(10):11721178.

  • 15

    Slavin ML. Metastatic malignant meningioma. J Clin Neuroophthalmol. 1989;9(1):5559.

  • 16

    Jee TK, Jo KI, Seol HJ, Kong DS, Lee JI, Shin HJ. Clinical features and treatment outcome of chordoid meningiomas in a single institute. J Korean Neurosurg Soc. 2014;56(3):194199.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 17

    Dalle Ore CL, Magill ST, Yen AJ, et al. Meningioma metastases: incidence and proposed screening paradigm. J Neurosurg. 2019;132(5):14471455.

  • 18

    Rushing EJ, Bouffard JP, McCall S, et al. Primary extracranial meningiomas: an analysis of 146 cases. Head Neck Pathol. 2009;3(2):116130.

  • 19

    Musa BS, Pople IK, Cummins BH. Intracranial meningiomas following irradiation—a growing problem? Br J Neurosurg. 1995;9(5):629637.

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