Jugular foramen chordoid meningioma

Abstract

Chordoid meningioma (CM) is a rare histological variant of meningioma and is classified as an atypical meningioma on pattern alone. Herein is described the first case of CM occurring in the jugular foramen. The patient was a 45-year-old woman with a 2 year history of progressive right hearing loss. Magnetic resonance imaging (MRI) demonstrated a large, dumbbell-shaped, homogenously gadolinium-enhanced mass in the right jugular foramen, extending medially to the cerebellopontine angle and caudally into the upper carotid space. Angiographic findings supported a diagnosis of schwannoma. Intraoperatively, the tumor appeared to involve the right glossopharyngeal nerve completely and the vagus nerve incompletely, and was incompletely resected. Microscopically, the tumor consisted predominantly of cords and nests of medium-sized cells with bland cytological features, surrounded by a pale basophilic mucin. Immunohistochemically, the tumor cells demonstrated reactivity for epithelial membrane antigen (membranous) and vimentin, with negative staining for S-100 protein, cytokeratin, CD34, glial fibrillary acidic protein (GFAP), synaptophysin, and chromogranin A. Based on the chordoid histology, an organoid lobular arrangement of the tumor cells, and the location of the tumor (jugular foramen), the differential diagnosis included not only a chordoma but also a paraganglioma (glomus jugulare tumor). Histological identification of typical meningotheliomatous areas, plus selective immunohistochemical panel, is important to establish the correct diagnosis.

Chordoid meningiomas: Incidence and clinicopathological features of a case series over 18 years

“…Our literature summary concludes that CM has an anatomical preference for the supratentorial region (177/221: 80.1%). The finding is concordant with the work of others . Other CM cases occur in the infratentorial region and unusual sites, notably the ventricular system, the jugular foramen, the orbit, the cervical spine and the pineal region .…”

“…Nine patients were reviewed from 1743 total meningioma patients in a 1995 to 2013 time period at St. Michael's Hospital in Toronto. To the best of our knowledge, 221 cases of CM have been reported in the English literature worldwide in the form of single case reports and/or case series …”

Distinguishing Chordoid Meningiomas From Their Histologic Mimics

“…Meta-analysis results of previously reported immunohistochemical findings in chordoid meningioma 6,17,21,22,24,30,35,36,38,40,42,43,45,47– 49,51–54,61,62,70,75,77,80,82,86 (published in English language) are shown in Figure 7. Additional immunohistochemical staining results in chordoid meningioma reported in the literature (which were not included in Fig.…”

“…35,38,45,52,61 Reported cases of chordoid meningioma have shown no immunohistochemical staining for the following antibodies: carcinoembryonic antigen (n=4), CD34 (n=4), desmin (n=4), smooth muscle actin (n=3), synaptophysin (n=3), chromogranin (n=2), CD56 (n=1), CD99 (n=1), calponin (n=1), 34βE12 (n=1), keratin 14 (n=1), keratin 19 (n=1), factor VIII (n=1), neurofilament (n=1), p63 (n=1), and muscle specific actin (n=1). 21,38,40,48,49,52,54,62,70,80 …”

Clinical features and treatment of intracranial chordoid meningioma: a report of 30 cases

“…Only 120 cases of CM have been reported in the English‐language literature since it was first described in 1988 by Kepes et al . Most of those studies focused only on histopathological, immunohistochemical and cytological aspects, and seldom mentioned its clinical features and recurrence …”