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Non-tumorous Lesions of the Pineal Gland

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Abstract

Most common non-tumorous lesion of the pineal gland is a pineal cyst. Prevalence of pineal cyst in general population is 0.7–1.5%. Most PCs are asymptomatic, and their diagnosis is rather incidental. Surgical treatment in patients with non-specific symptoms is controversial. However, literature suggests improvement in majority of patients. Other non-tumorous lesion of the pineal gland are calcifications, apoplexy and arteriovenous malformation.

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Notes

  1. 1.

    One possible mechanism of PC development might be mild brain hypoxia in the perinatal period [15]. Ozmen et al. reported a higher prevalence of PC in patients with cerebral palsy and periventricular leukomalacia [16]. Some studies suggest that pineal necrosis (apoplexy) may lead to subsequent creation of a glial scar in the form of a cyst [17, 18]. Another proposed explanation is sequestration of the pineal recess of the third ventricle [6, 19].

  2. 2.

    The largest study, published by Al-Holou et al., reviewed 48,417 consecutive adult patients who underwent a MRI examination. Four hundred and seventy-eight PCs larger than 5 mm (i.e., 1.0%) were found with a slight female preponderance (316:162).

  3. 3.

    Al-Holou et al. found peak prevalence of PC 3.7% in a age group 6–12 years followed by steady decline in older age groups reaching 0.1% in an age group 81–90 years [8, 14]. Majovsky et al. found increase in the PC size during the follow-up in 5.5% and decreased in size in 8.2% in their series of 110 patients [20]. Mean age in a group of patients whose PC increased was significantly lower compared to those with PC size decrease.

  4. 4.

    Fakhran et al. analyzed preoperative images of 52 patients with pineocytoma GI [28] and demonstrate that none met MRI criteria of “a typical pineal cyst” postulated by Barboriak et al. [11].

  5. 5.

    Hajnsek et al. did not diagnose hydrocephalus or Parinaud’s syndrome in a series of 56 patients [32], and Majovsky et al. reported only 1 patient in series of 110 patients [20]. On the other hand, Berhouma et al. reported obstructive hydrocephalus in 18 (75%) of 24 operated patients [37].

  6. 6.

    Seifert et al. studied the association between PC and headache. These authors compared 51 patients with PC, and 51 matched controls without PC [34]. Patients with PC had headaches twice as often as the controls, suggesting the existence of causality. The exact mechanism remains unknown, although several hypotheses have been proposed (e.g., a melatonin disturbance or compression of the vein of Galen) [38, 39].

  7. 7.

    The largest surgical series published by Hajnsek et al. demonstrated 56 patients with PC and 38% of them presented with a seizure [32].

  8. 8.

    Eide et al. developed a hypothesis of central venous hypertension caused by a compression of the vein of Galen by PC [38, 39]. The authors showed correlation between the severity of symptoms and certain MRI markers of a central venous hypertension as well as an increased pulsatile intracranial pressure in patients with symptomatic PC.

  9. 9.

    An online survey was conducted to analyze clinical management of patients with PC among neurosurgeons worldwide [42]. Most of respondents have operated on patients with a PC only if they presented with symptoms attributable to a mass effect. Surgery for patients with non-specific complaints is not widely accepted (only 15% respondents do so), although some agree that such surgery may be effective.

  10. 10.

    Large surgical series report the proportion of operated patients to be 16.6–20.9% [20, 31, 43, 44]. Obviously, these numbers are high due to selection bias. The real proportion of patients with PC in the general population who might benefit from the surgery is much smaller.

  11. 11.

    In the largest surgical series, all patients underwent surgery of PC using the SCIT approach in a sitting position. In this patient group, the complication rate was 0% [32].

  12. 12.

    Hemianopia occurs transiently after OTT approach in 16.1–79% of the patients and permanently in 0–4.1% [37, 45, 46]. Berhouma et al. used OTT in 20 patients with PC and achieved radical resection of PCs in 70% with a complication rate of 20% (four patients with transient hemianopia) [37].

  13. 13.

    Majovsky et al. examined melatonin and cortisol secretion profiles in four patients before and after PC resection. In all cases patients experienced a loss of endogenous pineal melatonin production, which equated with pinealectomy. Surprisingly, cortisol secretion substantially increased in patients after surgery [30].

  14. 14.

    Kreth et al. reported the largest case series using stereotactic aspiration of PCs (n = 14 patients). The authors encountered no complications, but clinical improvement was achieved in only 42.9% of the patients. No recurrence was noted in this series, although Mena et al. reported the regrowth of a PC in their series with only one patient treated with stereotactic aspiration [51]. Stern and Ross treated two patients with PC using stereotactic aspiration and one of the PC regrew in 6 years [52].

  15. 15.

    Tirakotai et al. performed an endoscopic fenestration and biopsy in nine patients. No complications occurred in this series but one PC recurred. Regrettably, the authors do not state the clinical outcome of the patients.

  16. 16.

    Eide et al. reported on 21 operated patients: 15 had their PC resected and 6 had their PC microscopically fenestrated. The authors found significantly better results in the resection group [44]. Majovsky et al., in their series of patients with PC resection, spared some pineal tissue (less than half of the PC) to prevent total melatonin secretion loss [20]. Despite this sparing strategy, authors still achieved improvement in 95.2% of patients.

  17. 17.

    Mattogno et al. described two cases of vanishing pineal tumor following apoplexy [13]. This phenomenon has been described in pituitary apoplexy as well. McNeely et al. speculate that bleeding into the pineal gland may promote PC formation [18].

  18. 18.

    Weil et al. reported case of intraparenchymal pineal AVM. Patient presented with intraventricular hemorrhage and hydrocephalus. AVM was fed by posterior choroidal arteries and draining into an ectatic vein of Galen. Diagnosis was confirmed on histopathological examination following successful surgical removal [61].

  19. 19.

    Ko et al. reported case of delayed abscess in patient after transsphenoidal resection of pituitary adenoma associated with cerebrospinal fluid leak [62].

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I would like to acknowledge prof. Vladimir Benes, M.D., Ph.D. for his support and valuable help during the preparation of this chapter.

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Majovsky, M. (2020). Non-tumorous Lesions of the Pineal Gland. In: Hoz, S.S., et al. Pineal Neurosurgery. Springer, Cham. https://doi.org/10.1007/978-3-030-53191-1_6

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