Pineal mass

By Marcello Cherchi, MD PhD

For patients

The pineal gland is a structure deep in the brain. If the pineal gland greatly enlarges (such as with a cyst or a tumor), then it can cause problems with eye movements, vision, walking and sometimes with hearing, and in such cases, a neurosurgeon may recommend removing the pineal mass. In cases where the pineal gland is affected by a non-malignant cyst that is not very large, then it is unlikely to be causing symptoms, and neurosurgeons will usually not recommend surgery.

For clinicians

Overview

Large pineal masses can have mechanical effects by narrowing or closing the cerebral aqueduct (resulting in obstructive hydrocephalus) and compressing the tectal plate (causing Parinaud syndrome and sometimes auditory symptoms). Non-malignant pineal cysts are frequently encountered as incidental findings on brain imaging. If a pineal mass (of any etiology) is causing mechanical compression, then neurosurgical intervention is indicated. In cases of non-malignant pineal cysts that are not causing mechanical compression, management is less clear; most clinicians will refrain from neurosurgical intervention.

Introduction

Large pineal masses can narrow or close the cerebral aqueduct (resulting in obstructive hydrocephalus) and compress the tectal plate (causing Parinaud syndrome and sometimes auditory symptoms), with corresponding abnormalities on physical examination, ocular motor examination, and audio-vestibular evaluation.

In routine clinical practice, the most commonly encountered pineal mass is a non-malignant pineal cyst found incidentally on brain imaging. Such pineal cysts usually remain stable in size; a small proportion of them (about 2.6% – 5.5%) enlarge. If a non-malignant pineal cyst is large enough to be causing obstructive hydrocephalus or Parinaud syndrome (dorsal midbrain syndrome), then neurosurgical intervention is indicated. If a non-malignant pineal cyst is not that large (and thus causing neither obstructive hydrocephalus nor Parinaud syndrome), then it is difficult to establish a causative relationship between the cyst and any symptoms, so management is less clear.

Many cases of pineal masses have been reported in the literature. Case series (whether prospective or retrospective) are more helpful in understanding the behavior of these lesions. Fortunately, there have been several informative case series of pineal masses (Fernandes et al. 2026; Gregory et al. 2011; Hajnsek et al. 2013; Hankinson et al. 2016; Hoehn et al. 2017; Májovský et al. 2017; Shields et al. 2017).

Epidemiology

Non-malignant pineal cysts are a common incidental radiologic finding.

Fernandes and colleagues (Fernandes et al. 2026) note that literature cites prevalence rates of non-malignant pineal cysts in the general population as ranging widely from 1.4% to 37.5%. Pineal cysts are more common in women. Of all pineal cysts, about 5% increase in size; the remainder are static.

Májovský and colleagues (Májovský et al. 2017) prospective studied 110 patients with pineal cysts, with a mean follow-up time of 79.2 months. They reported that the pineal cyst increased in size in 6 patients (5.5%), decreased in size in 9 patients (8.2%), and were stable (unchanged in size) in the remaining 95 (86.3%).

Al-Holou and colleagues (Al-Holou et al. 2011) studied 48,417 consecutive adults (over 18 years) who underwent brain MRI over a 12-year period. They found pineal cysts measuring 5 mm or larger in greatest dimensions in 478 patients (1.0%), of which 162 (33.9%) were male and 316 (66.1%) were female. Of 151 patients with subsequent brain MRIs (done at a mean interval of 3.4 years from the index study), 4 (2.6%) increased in size, 23 (15.2%) decreased in size, and 124 (82.1%) pineal cysts remained stable.

Al-Holou and colleagues (Al-Holou et al. 2011) then combined MRI data from the same 48,417 consecutive adults (over 18 years) with 14,516 consecutive children (≤8 years) on whom they had previously reported (Al-Holou et al. 2009), and arrived at the age-based histogram of prevalence of pineal cysts shown in the Figure below.

Figure : Histogram showing the prevalence of pineal cysts in 48,417 consecutive adult patients and 14,516 consecutive pediatric patients undergoing MRI. From Al-Holou et al (Al-Holou et al. 2011).

Genetics

Until recently there were no known genetic factors predisposing to the development of non-malignant pineal cysts. With the advent of whole exome sequencing, some research suggests that several hundred genetic variants may be associated with non-malignant pineal cysts (Yan et al. 2021). While this is interesting, there is not yet any practical consequence, so genetic testing currently plays no role in the routine evaluation of non-malignant pineal cysts.

Some malignant pineal masses do have known genetic associations. For example, a malignant pineal mass occurring in a patient with bilateral retinoblastomas (OMIM 180200) is associated with a mutation on chromosome 13q14.2 encoding retinoblastoma transcriptional corepressor 1 (RB1) (OMIM 614041).

Pathophysiological mechanism of disease

Pineal masses (of any etiology) can provoke symptoms when they are large enough to cause the mechanical effects of (1) blocking the quadrigeminal cistern, thereby limiting the flow through the cerebral aqueduct and leading to obstructive hydrocephalus, and/or (2) compressing the tectal plate.

The various etiologies of pineal masses vary in prevalence, and case series report different rates.

For example, Hajnsek and colleagues (Hajnsek et al. 2013) studied a retrospective series of 84 patients who underwent surgery for pineal masses. They reported the following histopathologies:

  • Pineal gland cysts in 80%
  • Pineocytomas in 16%
  • Pineoblastomas in 3%
  • Teratoma in 1%

In contrast, Shields and colleagues (Shields et al. 2017) retrospectively reviewed 40 cases of Parinaud syndrome. Twelve of these cases were due to pineal tumors, and the histopathologies were:

  • Germinoma in 4 (25%)
  • Pineoblastoma in 2 (17%)
  • Meningioma in 2 (17%)
  • Astrocytoma in 1 (8%)
  • Cavernoma in 1 (8%)
  • Choriocarcinoma in 1 (8%)
  • Diffuse B-cell lymphoma in 1 (8%)

Clinical presentation

A non-malignant pineal cyst is a common incidental finding on brain imaging (see the earlier section on epidemiology). Incidentally discovered pineal cysts are rarely the cause of symptoms (Al-Holou et al. 2011; Choy et al. 2011; Di Costanzo et al. 1993; Pu et al. 2007).

When a pineal mass (of any etiology) causes obstructive hydrocephalus, common presenting symptoms are those of hydrocephalus in general, including nausea, vomiting and visual abnormalities (Upadhyayula et al. 2023), including from ocular motor cranial nerve palsies.

Májovský and colleagues (Májovský et al. 2017) prospectively studied 110 patients with pineal cysts who were referred for neurosurgical consultation, and reported the following presenting symptoms:

  • Tension headache in 62.7%
  • Vertigo in 16.4%
  • Migraine in 12.7%
  • Syncope in 10.9%
  • Nausea in 8.2%
  • Diplopia 8.2%

Májovský and colleagues (Májovský et al. 2017) followed these 110 patients over a mean follow-up time of 79.2 months and reported that symptoms worsened in 17 (15.5%), improved in 13 (11.8%) and remained stable in 81 (73.6%).

If a pineal mass compresses the inferior colliculi, then various auditory symptoms may result, including:

  • Hearing loss (Gaspar et al. 2003; Islam et al. 2002; Mizobuchi et al. 2021; Woo et al. 2013)
  • Word deafness (Joswig et al. 2015; Masuda et al. 2000)
  • Tinnitus (Missori et al. 1995)

Physical examination

If a pineal mass is causing hydrocephalus, the ophthalmoscopic examination may show papilledema. Hankinson and colleagues (Hankinson et al. 2016) studied a case series of 29 children and reported that 69% exhibited papilledema.

Ocular motor examination

Pineal masses often manifest with Parinaud syndrome (also known as dorsal midbrain syndrome), whose classic presentation comprises the triad of conjugate upgaze paralysis, convergence-retraction nystagmus and light-near dissociation. However, Shields and colleagues (Shields et al. 2017) report that Parinaud syndrome presents with the full triad of signs in only 65% of cases. Hankinson and colleagues (Hankinson et al. 2016) studied a case series of 29 children and reported that 75% exhibited “partial or complete Parinaud’s syndrome.”

The upgaze paresis and convergence-retraction nystagmus can be elicited by vertical optokinetic stimulation (horizontal stripes moving vertically). This is one of the few instances in which it is helpful to use an optokinetic drum oriented such that the axis of rotation is parallel to the earth’s surface during ocular motor examination.

Less common manifestations in Parinaud syndrome include upper eyelid retraction (Collier’s sign), diplopia, visual field defects and ataxia (Orvis 2024). Gregory and colleagues report impaired downgaze and convergence insufficiency (Gregory et al. 2011). Hoehn and colleagues report exotropia (Hoehn et al. 2017).

Testing: auditory

If a pineal mass compresses the inferior colliculi, then various auditory symptoms may result, such as hearing loss, word deafness and tinnitus.

Gaspar and colleagues (Gaspar et al. 2003) report the case of a 12-year-old boy with a pineal germ cell tumor that was compressing the inferior colliculi. Audiometry showed approximately symmetrical sensorineural hearing loss (greater in the high frequencies than the middle frequencies) and complete word deafness, as shown in the Figure below.

Figure : Case of a 12-year-old boy with pineal germ cell tumor compressing the inferior colliculi. Audiometry shows approximately symmetrical high greater than middle frequency sensorineural hearing loss, and complete word deafness. From Gaspar et al (Gaspar et al. 2003).

Compression of the inferior colliculi by a pineal mass can also cause abnormalities in auditory brainstem evoked responses (ABR). For example, Mizobuchi and colleagues (Mizobuchi et al. 2021) report a case of a patient pineal glial cyst compressing the inferior colliculi, whose auditory brainstem evoked responses showed an absence of wave V, as displayed in the Figure below.

Figure : Auditory brainstem evoked response in a patient with a pineal glial cyst compressing the inferior colliculi. Note the absence of wave V bilaterally. From Mizobuchi et al (Mizobuchi et al. 2021).

Woo and colleagues (Woo et al. 2013) reported the case of a patient with a pineal ganglioglioma compressing the inferior colliculi. In this case wave V was preserved, but the wave III-V interpeak interval was prolonged.

Testing: vestibular

Baloh and colleagues (Baloh et al. 1985) reported a series of patients with Parinaud’s syndrome, of which 5 were cases of pineal tumors.

The Figure below, from Baloh and colleagues (Baloh et al. 1985), shows impairment of vertical saccades in a 15-year-old boy with a pineal dysgerminoma.

Figure : Ocular motor tracings of a 15-year-old boy with pineal dysgerminoma. Note that upward saccades are hypometric by more than 1 standard deviation. From Baloh et al (Baloh et al. 1985).

Eliciting convergence-retraction nystagmus can be done very effectively with a projected optokinetic stimulus, as shown in the Figure below from Garbutt and Harris (Garbutt and Harris 2000).

Figure : Projection of a vertically moving optokinetic stimulus. From Garbutt and Harris (Garbutt and Harris 2000).

Imaging

The Figure below, from Aaroe and colleagues (Aaroe et al. 2021), shows an MRI from a patient whose pineal mass is a primary pineal melanoma.

Figure : MRI of a patient with a primary pineal melanoma. Panel (A) shows a hyperintense pineal region mass. Panel (B) shows enhancement of this mass, as well as enhancement of numerous foci in the subarachnoid space. Panel (C) shows high signal intensity on T1-weighted images. From Aaroe et al (Aaroe et al. 2021).

Histopathology

Pineal masses can result from a range of etiologies, including:

  • Cyst (Bosnjak et al. 2009; Fleck et al. 2022; Gotz Wieckowska et al. 2017; Lukewich et al. 2020; Woodward et al. 2020)
  • Arachnoid cyst (Gonzalez et al. 2007)
  • Epidermoid cyst (Konovalov et al. 1999)
  • Ganglioglioma (Woo et al. 2013)
  • Germinoma (Gaspar et al. 2003; Gurusamy et al. 2022; Islam et al. 2002; Joswig et al. 2015; Nishibayashi et al. 2005; Prasetio et al. 2022; Wong et al. 2023; Yang et al. 2004)
  • Glial cyst (Mizobuchi et al. 2021)
  • Granular cell astrocytoma (El Asri et al. 2015)
  • Hemangioblastoma (Isolan et al. 2007)
  • Melanoma (Aaroe et al. 2021)
  • Metastatic small cell carcinoma (Kashiwagi et al. 1989)
  • Neuroendocrine carcinoma (Hakar et al. 2017)
  • Papillary tumor (Boco et al. 2008; Mobark et al. 2022)
  • Paraganglioma (Reithmeier et al. 2000)
  • Pineoblastoma (Saito et al. 2002; Yiğit and Gökoğlu 2025)
  • Pineocytoma (Allmer and Golis 2001)
  • Rosette-forming glioneuromal tumor (Solis et al. 2011)
  • Sarcoidosis (Adel et al. 2021)
  • Teratoma (Lewis et al. 1963)
  • Trilateral retinoblastoma (Bejjani et al. 1996; Cho et al. 2002)
  • Indeterminate or undifferentiated tissue (Shrateh et al. 2023)

Differential diagnosis

The main differential diagnosis of symptomatic pineal cysts includes other causes of hydrocephalus, and other causes of Parinaud syndrome (such as intrinsic lesions of the tectal plate).

Management

When a pineal mass — including a non-malignant pineal cyst — is large enough to cause obstructive hydrocephalus or Parinaud syndrome, then neurosurgical resection is indicated.

For patients who have pineal cysts that are not causing obstructive hydrocephalus or Parinaud syndrome, yet still have symptoms, it is difficult to establish a causative relationship between the pineal mass and the symptoms with confidence, so management is unclear.

Kalani and colleagues (Kalani et al. 2015) retrospectively described a series of 18 patients (14 female, 4 male; mean age 24 years, range 4 – 47 years) who underwent neurosurgical resection of benign pineal cysts (preoperative cyst diameter 1.5 cm, with a range of 0.9 – 2.2 cm) that were not causing obstructive hydrocephalus or Parinaud syndrome. The presenting symptoms in these 18 patients were:

  • Headache in 17 (94%)
  • Visual disturbances in 10 (56%)
  • Gait instability in 5 (28%)
  • Dizziness in 5 (28%)
  • Recurrent syncope in 2 (11%)
  • Hypersomnolence in 1 (6%)

Kalani and colleagues (Kalani et al. 2015) followed these patients for a mean of 19.1 months (ranging from postoperative to 71 months) and reported that 17 (94%) patients had “resolution of improvement in their presenting symptoms.”

Prognosis

Hoehn and colleagues (Hoehn et al. 2017) described a series of pediatric patients with Parinaud syndrome from pineal tumors. Out of 17 patients who underwent surgery, 7 (41%) exhibited postoperative improvement in the ocular motor abnormalities, but only 2 (12%) exhibited complete resolution.

When a pineal mass causes auditory symptoms due to compression of the inferior colliculi, treatment of the mass may bring about improvement in auditory symptoms (Gaspar et al. 2003; Joswig et al. 2015; Mizobuchi et al. 2021; Woo et al. 2013), as well as in audiometric results such as pure tone audiometry (Gaspar et al. 2003; Mizobuchi et al. 2021; Woo et al. 2013), word recognition (Gaspar et al. 2003; Woo et al. 2013), and auditory brainstem evoked responses (Islam et al. 2002).

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Page first published on May 10, 2026. Page last updated on May 10, 2026

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