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Chapter 48
Meningotheliomatous Meningioma
DilcanKotan , EsenÇiçekli , andDeryaKaraGenç
48.1 Introduction
Meningiomas are among the most common types of intracranial tumors, with an
annual incidence of approximately 8 cases per 100,000. Although more than half of
the cases have a benign course, meningiomas may become symptomatic due to
mass effect on the adjacent parenchyma or peritumoral edema [1]. Olfactory groove
meningiomas (OGM) account for 8–13% of all intracranial meningiomas. These
space-occupying lesions located in the anterior cranial fossa often arise from arachnoid cells embedded in the ethmoid lamina cribrosa and midline dural covers of the
frontosphenoidal suture. Rarely, it may also arise from meningeal precursor cells
derived from mesoderm and neuronal crest [2]. It is more common in women than
men and its frequency increases in the sixth decade [3].
Due to its benign and slow-progressing nature, OGMs, which do not cause
symptoms until they reach large sizes, are diagnosed quite late. In OGMs, there
are ndings such as personality changes, anosmia, visual impairment, seizures,
intracranial hypertension because of pressure on the frontal lobe, optic nerve, and
optic chiasm, and the most common symptom is headache [2, 4]. The most important treatment approach in OGM is surgical resection, and radiotherapy (RT) can
be performed in selected cases. RT is not as successful as surgery in relieving
tumor- related neurological symptoms. For patients whose tumors cannot be
D. Kotan (*)
Sakarya University, School of Medicine, Department of Neurology, Sakarya, Turkey
E. Çiçekli
Akyazı State Hospital, Neurology Department, Sakarya, Turkey
D. K. Genç
Sakarya University Education and Research Hospital, Neurology Department,
Sakarya, Turkey
Switzerland AG 2026
D. Uludüz et al. (eds.), Rare Causes of Headache Disorders, Headache,
https://doi.org/10.1007/978-3-032-10242-3_48
459© The Author(s), under exclusive license to Springer Nature
460
accessed surgically or are at risk for surgery, RT is frequently used to control local
tumor growth [5].
In this section, a case of olfactory meningioma that presented with complaints of
headache and visual impairment is described. It aims to increase the curiosity of clinicians and researchers in this subject by providing detailed clinical information about
OGMs, which are not frequently considered among the causes of headache in clinical
practice and whose diagnosis can be overlooked until they reach giant proportions.
D. Kotan et al.
48.2 Pathophysiology
Meningothelial meningiomas originate from arachnoidal cap (meningothelial)
cells, which form part of the meninges and regulate cerebrospinal uid absorption.
The most frequent genetic facilitator involves loss-of-function mutations in the NF2
gene on chromosome 22q, leading to inactivation of its protein product, Merlin, a
tumor suppressor that normally restrains multiple proliferative pathways.
Neurobromin 2 (NF2) inactivation results in disrupted cytoskeleton remodeling
and cell–cell junction integrity, permitting unchecked cell growth and chromosomal
instability [6]. Additional genomic alterations (e.g. TNF Receptor Associated Factor
7 (TRAF7), AKT Serine/Threonine Kinase 1 (AKT1), and Smoothened Frizzled
Class Receptor (SMO)) may also play a role, though they are often mutually exclusive to NF2 mutations [7]. Histologically, meningothelial features include syncytial
cell sheets with indistinct borders, whorled patterns, and occasional psammoma
bodies—calcied concentric structures reecting apoptotic debris. These tumors
exhibit low mitotic activity and proliferation index (Ki-67), in line with their benign
WHOGradeI classication and favorable prognosis [6].
48.3 Case Presentation
A 39-year-old female patient applied to the neurology clinic with a complaint of
headache that started a few weeks ago, lasted 5–10minutes, and recurred from time
to time during the day. The headache was responsive to analgesics, was not accompanied by nausea and vomiting, had no photophobia or phonophobia, and was felt
more intensely around the right eye. For the last week, blurry vision in both eyes
was added to his headache. The patient, who was interned for further examination,
had a history of allergic asthma and a family history of migraine in his mother.
There was no regular medication use other than occasional inhaler asthma treatment.
In her neurological examination, her pupils were isochoric, and his light reex
was positive bilaterally. Bilateral eye movements were free in all directions. In
fundus evaluation, optic disc borders were slightly blurred on the right and normal
on the left. On vision examination, the right eye was 2/10 and the left eye was
6/10. On visual eld examination, there was near-total vision loss in all quadrants
in the right eye (Fig.48.1a). There was a visual eld defect in the left eye, more
ab
48 Meningotheliomatous Meningioma
Fig. 48.1 (a) Right eye visual eld test (b) Left eye visual eld test
ab c
461
Fig. 48.2 From left to right (a) Cranial CT (b) T2-weighted MRI (c) Contrast-enhanced
T1-weighted MRI images of the patient
prominent in the upper outer quadrants (Fig.48.1b). There was no facial asymmetry. The patient’s muscle strength examination was bilaterally normal, and his
plantar skin reex was bilateral exor. Vital signs were stable, and laboratory
parameters were normal.
Cranial computed tomography (CT) showed a hyperdense appearance with wellcircumscribed 22mm diameter and minimal calcication in the sellar-suprasellar
region (Fig.48.2a). Contrast-enhanced cranial magnetic resonance imaging (MRI)
showed a well-circumscribed lesion in the sellar region, consistent with CT, isointense and sometimes minimally hyperintense in T2 sections, hypointense in T1
series, and with pathological contrast enhancement (Figs.48.2b, c). When the neurological examination and current neuroimaging ndings were evaluated together, it
was evaluated as olfactory meningioma, which was thought to originate from the
462
ab
Fig. 48.3 From left to right (a) Postoperative right eye visual eld test (b) Postoperative left eye
visual eld test
D. Kotan et al.
olfactory groove, and it was referred for neurosurgical intervention. The patient’s
tumor tissue was resected under elective conditions by the neurosurgery department. For this purpose, the mass was removed through a right frontal incision and
sent for pathological examination. The pathology result was reported as meningotheliomatous meningioma, Grade 1.
At the rst postoperative follow-up examination, visual acuity was recorded as
4/10 on the right and full on the left. In the visual eld examination, although the
defect continued in every quadrant of the visual eld in the right eye, there was an
improvement compared to the pre-operative period (Fig.48.3a). Visual eld examination was normal in the left eye (Fig.48.3b). The patient’s follow-up continues in
the neurosurgery and ophthalmology outpatient clinics.
48.4 Case Discussion
OGMs most commonly present with symptoms of headache, anosmia, or personality changes. In our case, clinical ndings rst presented with headache, followed by
visual impairment a few weeks later. The fact that the headache was located in the
same region and unilateral. The accompanying symptom of visual impairment suggested an underlying organic cause, and the diagnosis was made after the requested
cranial imaging. Imaging methods are used in the diagnosis of OGMs. Although
diagnosis can be made with cranial CT or MRI, in cases where there is a choice,
MRI provides better image quality and localization chance [8].
48 Meningotheliomatous Meningioma
463
Due to its anatomical location, OGM may cause long-term psychiatric symptoms before the onset of signicant neurological decits. Because of these mild
symptoms, olfactory groove meningiomas can grow insidiously and emerge as one
of the largest intracranial tumors. Tumor size was categorized by neuroimaging
assessment. As classication, they are small (0–2cm in diameter), medium (2–4cm
in diameter), large (4–6cm in diameter), and giant (>6 cm in diameter). Visual
symptoms usually occur after an olfactory groove meningioma has reached a signicant size but may also depend on the anatomical site of origin. Because olfactory
groove meningiomas extend posteriorly, this extension may cause compression of
one or both optic nerves or the chiasm. It may occur earlier in smaller tumors arising
from the posterior half of the cribriform plate. Compression injury to the optic
nerves can cause optic nerve head edema and eventually progress to optic atrophy
[9]. The mechanism of damage in OGM is caused by the size of the tumor and creates a downward pressure on the nerve and chiasm from above, whereas in suprasellar meningioma, the chiasm rises, thus the cross bers are stretched and optic
atrophy and bitemporal hemianopsia occur. In our current case, which involved a
medium-sized olfactory groove meningioma measuring 2.2cm in axial diameter,
there were complaints of headaches and visual ndings that were attributed to chiasm involvement due to the suprasellar location.
OGMs must be distinguished from other midline meningiomas of the anterior
cranial fossa, namely planum sphenoidale meningiomas, whose dural attachments
lie behind the frontosphenoidal suture, and tuberculum sellae meningiomas,
which arise from the dura anterior to the chiasmatic sulcus. This distinction is not
only an anatomical application but also has clinical importance. Planum sphenoidale and tuberculum sellae meningiomas are generally diagnosed earlier in their
clinical course due to visual impairment. It is smaller in size and easier to approach
during surgery. Due to their large size, OGMs can sometimes be long and difcult
to approach and require meticulous surgery to minimize tissue damage. Tumor
size and location are also important in determining the surgical approach to be
chosen on a patient-by-patient basis [10]. In addition to the transcranial approach,
the endoscopic endonasal approach is also used in appropriate cases [11].
However, the chance of success after a good resection is quite high and complications can be easily controlled. In our case, resection was performed through a
right frontal incision, which is the point where the tumor can be most easily
reached. High patient age and large tumor size are considered poor prognostic
factors [12]. Our patient’s age was considered early according to the literature.
The fact that the tumor size was detected when it was medium sized also suggests
that our case will have a good prognosis.
Although most meningiomas are Grade 1 and benign tumors, up to 15% may
be atypical and 2% may be anaplastic [13]. As in most cases, the pathology in
our patient was Grade 1. This result suggests that the patient will have a good
prognosis.
464
D. Kotan et al.
48.5 Clinical Characteristics
Headache is the most common presenting symptom in patients with meningothelial
meningiomas. It is typically attributed to a combination of mass effect, irritation of
pain-sensitive dural structures, and increased intracranial pressure (ICP). The headache usually develops insidiously over weeks or months and is often described as a
dull, constant, pressure-like pain rather than a sharp or pulsating sensation. It frequently worsens in the early morning hours, likely due to overnight CO₂ retention
and the supine position increasing cerebral blood ow and ICP.In some cases, the
headache may be exacerbated by Valsalva maneuvers such as coughing or straining,
further supporting the role of raised ICP.These headaches are often poorly responsive to standard analgesics and may be accompanied by additional signs such as
nausea, vomiting, or transient visual disturbances like blurred vision or papilledema.
Signicantly, the location of the headache does not consistently correlate with the
anatomical site of the tumor, reecting the diffuse nature of pressure- related symptoms rather than localized cortical involvement [14, 15].
48.6 Diagnosis ofMeningothelial Meningiomas
The diagnosis of meningothelial meningiomas involves a combination of clinical
evaluation, neuroimaging, and histopathological conrmation. Clinically, patients
may present with non-specic symptoms such as headache, seizures, or focal neurological decits, depending on the tumor’s location and mass effect. Radiologically,
contrast-enhanced MRI is the modality of choice, typically revealing a wellcircumscribed, extra-axial mass that shows homogeneous enhancement and is often
associated with a dural tail sign. These tumors are usually isointense to gray matter
on T1- and T2-weighted images. Denitive diagnosis is made via histopathological
examination following surgical resection. Meningothelial meningiomas are characterized by lobules or whorls of polygonal cells with indistinct cell borders, eosinophilic cytoplasm, and round to oval nuclei. Psammoma bodies may also be present.
Immunohistochemistry is supportive, with tumors typically positive for epithelial
membrane antigen (EMA) and vimentin [8, 15, 16].
48.7 Treatment
The primary treatment of meningothelial meningiomas is surgical resection, aiming
for complete removal of the tumor, including involved dura and any invaded bone.
For WHO Grade I meningiomas, such as the meningothelial subtype, gross total
resection is often curative, and adjuvant therapy is usually not required. However, in
cases of subtotal resection, tumor recurrence, or when surgery is contraindicated
48 Meningotheliomatous Meningioma
465
due to location or patient factors, radiotherapy—particularly stereotactic radiosurgery or fractionated radiotherapy—may be employed as an adjunct or primary treatment. Observation (watchful waiting) is also an option in asymptomatic or incidental
cases, especially when the tumor is small and not causing mass effect. Long-term
follow-up with periodic imaging is essential, as even benign meningiomas may
recur, particularly if resection is incomplete [13, 15, 17].
48.8 Conclusion
The importance of this case report is that when evaluating intracranial areas in
patients presenting with headache, evaluation of the extracranial regions should not
be overlooked. However, it is essential to draw attention to the diagnostic value of
imaging, as well as a good anamnesis and examination, in the differential diagnosis
of visual ndings accompanying headache, which may occur in insidious tumors
such as OGM.It is aimed to raise awareness about OGMs, as they can be successfully treated and prevented from becoming life-threatening by a surgical procedure
performed before the tumor reaches giant sizes. The importance of prioritizing
appropriate imaging in terms of early detection of pathologies in headache and
accompanying vision problems in the young female population is emphasized.
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D. Kotan et al.
Chapter 49
Headache or Facial Pain Attributed
toInammation oftheStylohyoid
Ligament
ElderSarmento
49.1 Introduction
Headache and facial pain are common complaints in neurological practice, with a
wide range of etiologies. Among the less frequent causes, inammation of the stylohyoid ligament emerges as a distinct clinical entity, although it is often underdiagnosed. Previously known as Eagle syndrome, this condition is now classied in the
International Classication of Headache Disorders, third edition (ICHD-3) as 11.8
Headache or facial pain attributed to inammation of the stylohyoid ligament [1].
This chapter aims to provide an in-depth analysis of this condition, exploring its
anatomical bases, pathophysiological mechanisms, clinical manifestations, diagnostic approaches, and treatment options. In doing so, we aim to raise awareness of
this rare cause of craniofacial pain and equip clinicians with the necessary tools for
accurate recognition and management.
E. Sarmento (*)
Sociedade Brasileira de Cefaleia, Barra Mansa, Brazil
UniFOA, Centro Universitário Fundação Oswaldo Aranha, Volta Redonda, Brasil
Switzerland AG 2026
D. Uludüz et al. (eds.), Rare Causes of Headache Disorders, Headache,
https://doi.org/10.1007/978-3-032-10242-3_49
467© The Author(s), under exclusive license to Springer Nature
468
E. Sarmento
49.2 Pathophysiology
The stylohyoid ligament is a brous structure that extends from the styloid process
of the temporal bone to the lesser horn of the hyoid bone. Its primary function is to
suspend and stabilize the hyoid bone, which plays a crucial role in swallowing,
speech, and breathing. Inammation of the stylohyoid ligament can result from a
variety of factors, including trauma, infection, prior surgery (such as tonsillectomy),
or systemic inammatory conditions [2].
The pathophysiology of pain in inammation of the stylohyoid ligament is multifaceted. Direct inammation of the ligament can activate local nociceptors, leading to referred pain in the cervical, facial, or cranial region. Additionally,
inammation can cause compression or irritation of adjacent nerve structures, such
as cranial nerves V, VII, IX, and X, resulting in neuropathic pain. Vascular compression may also play a role, especially in the context of Eagle syndrome, where an
elongated styloid process or a calcied stylohyoid ligament can compress the internal or external carotid artery [3].
49.3 Case Presentation
A 34-year-old patient begins with incipient symptoms: difculty in ingesting solid
foods and odynophagia. Sudden intensication of symptoms includes dyspnea,
homolateral cervicalgia, cervical edema with lymphadenomegaly, intense headache, leading to the rst hospital admission. The evolution of the condition includes
worsening of cervicalgia during neck rotation, otalgia, pain in the temporomandibular joint (TMJ), changes in dental occlusion, dizziness, and changes in facial and
dental arch sensitivity on the homolateral side. Characteristic symptom includes
lancinating pain in the topography of the submandibular gland and region posterior
to the palatine tonsil. The patient relieved the pain by pressing the area with his
nger. During an episode of intense pain, he applied excessive pressure, probably
leading to a fracture of the styloid process, which signicantly aggravated the clinical condition. Multiple hospitalizations occur due to the intensity of the symptoms,
including difculty swallowing, persistent cervical edema, and a foreign body sensation in the pharynx. Initial examinations (electroneuromyography, magnetic resonance imaging [MRI] of the skull and neck, cerebrospinal uid [CSF]) do not reveal
a specic cause. Cervical MRI shows degenerative changes, which were ruled out
as the cause of the symptoms. Panoramic radiography suggests Eagle syndrome,
revealing calcication of the right stylohyoid ligament (Fig.49.1). Cone-beam com-
puted tomography (CT) with three-dimensional (3D) reconstruction conrms calcication and elongation of the right stylohyoid ligament (50 mm) (Fig. 49.2).
Cervical angiotomography shows contact of the styloid process with a branch of the
external carotid artery. Surgery was performed to resect the right styloid process,