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Pineal tumors
- 2. Case scenerio ⢠An 14-year-old right-handed boy, otherwise healthy, presents to your clinic with a one-week history of progressively increasing headaches. These are worse in the morning and are associated with nausea and vomiting. ⢠There are no other systemic symptoms. ⢠Physical examination is unremarkable except upward gaze palsy.
- 3. ⢠CT was orderd
- 5. Epidemiology ⢠Pineal region Tumors account for <1%of all intracranial tumors in adults and 3-8% in children ⢠Average age at presentation is 13 years. ⢠Adults typically are older than 30 years at presentation.
- 6. Relevant anatomy ďą Anteriorly - Posterior 3rd V ďą Posteriorly - Cerebellar vermis ďą Superiorly - Splenium - Tela choroidea ďą Inferiorly - Quadrigeminal plate / Midbrain tectum
- 7. Etiology and Pathology ⢠Pineal tumors are grouped into Four main categories 1. Germ cell tumors 2. Pineal cell tumors 3. Glial cell tumors 4. Miscellaneous tumors
- 8. Classification Subtypes Pineal Parenchyma â˘Pineoblastoma â˘Pineocytoma Germ cell origin Germinoma (also called Dysgerminoma) Non Germinomatous Germ Cell Tumors (NGGCTs): Also called Non Seminomatous Germ Cell Tumors (NSGCTs): â˘Embryonal â˘Teratoma â˘Endodermal sinus (= yolk sac) â˘Choriocarcinoma Glial â˘Astrocytoma â˘Glioblastoma â˘Oligodendroglioma â˘Ependymoma Other â˘Meningioma â˘Chemodectoma â˘Craniopharyngioma â˘Metastases Non-neoplastic â˘Cysts â˘Dermoid cyst â˘Arachnoid cyst â˘Lipoma
- 9. Benign lesions (teratoma, pineocytoma, meningioma) 30-40% Germ cell tumors 60% Glial tumor 25% Ependymal tumor 15%
- 10. Presentation Generally manifested in THREE WAYS 1. Symptoms of increased ICP(obstructive HCP) 2. Direct brainstem and cerebellar compression 3. Endocrine dysfunction
- 11. Symptoms of increased ICP â Headache (worse in morning) â Nausea and vomiting â On examination papilledema ⢠Parinaud's Syndrome results in several different visual deficits which include â paralysis of upward gaze â reduced pupillary reflexes to light â convergence paresis
- 12. ⢠Gait abnormalities, unsteadiness and frequent falls due to pressure on cerebellar peduncles ⢠endocrine effects can include â Diabetes mellitus â Hypothalamic dysfunction ⢠Other secondary neuroendocrine deficits: â Include hypopituitarism, growth failure, and elevated hCG or LH level. â Precocious puberty
- 13. investigations ⢠CT ⢠MRI ⢠Serum & CSF levels of AFP and βHCG ⢠CSF cytology ⢠Endocrine evaluation ⢠Open biopsy, stereotactic biopsy or endoscopic biopsy
- 14. Radiography ⢠Germinomas are associated with a high incidence of pineal gland calcification. ⢠The tumor does not calcify, but it may engulf a calcified pineal gland. ⢠A calcified pineal gland on plain radiographs in a boy younger than 10 years suggests pineal germinoma.
- 15. Computed Tomography ⢠Nonenhanced CT scans â Typically demonstrate a slightly hyperattenuating mass that engulfs a prominent calcified pineal gland. ⢠Contrast-enhanced CT scanning â demonstrates homogeneous enhancement.
- 16. ⢠More sensitive than plain radiography in depicting pineal calcification. ⢠Small speckles of calcification can be seen in children 6 years and are considered normal ⢠Pineal calcification in children younger than 6 years is suggestive of pineal neoplasm. ⢠Pineal calcifications should be less than 1 cm in diameter in size.
- 17. Germinoma âŚ..MRI findings ⢠T1/T2: ⢠Usually isointense relative to cerebral gray matter ⢠Occasionally ⢠T1:hypointense ⢠T2:hyperintense ⢠I/V injection of gadolinium-based contrast material, ⢠homogeneous and intense enhancement is seen.
- 18. T1-Non Contrast
- 19. T1Contrast
- 20. T1c Coronal and Saggital
- 21. T2
- 24. Managment
- 25. Surgical Approach ⢠Supra cerebellar-infratentorial approach ⢠Occipital transtentorial approach ⢠Transcallosal,interhemispheric approach
- 27. Patient positioning ⢠Sitting position : â Most often used for Supra cerebellar- infratentorial approach
- 28. ⢠Three-quarter prone / Lateral decubitus â Used for occipital trans tentorial approach
- 29. ⢠Prone position â Used for supra tentorial transcallosal, interhemispheric approach
- 30. Supra cerebellar-infratentorial approach ⢠Most preffered ⢠Approach is to the center of the tumor ⢠No normal tissue is violated on route to the tumor.
- 31. M I D L I N E I N F R A T E N T O R I A L S U P R A C E R E B E L L A R A P P R O A C H
- 32. M I D L I N E I N F R A T E N T O R I A L S U P R A C E R E B E L L A R A P P R O A C H
- 33. M I D L I N E I N F R A T E N T O R I A L S U P R A C E R E B E L L A R A P P R O A C H
- 34. M I D L I N E I N F R A T E N T O R I A L S U P R A C E R E B E L L A R A P P R O A C H
- 35. M I D L I N E I N F R A T E N T O R I A L S U P R A C E R E B E L L A R A P P R O A C H
- 36. M I D L I N E I N F R A T E N T O R I A L S U P R A C E R E B E L L A R
- 37. M I D L I N E I N F R A T E N T O R I A L
- 38. M I D L I N E I N F R A T E N T O R I A L S U P R A C E R E B E L L A R A P P R O A C H
- 40. Post operative care ⢠Steroids first few days ⢠CT = Suspicion of any complications ⢠Mobilize and ambulate ⢠Drain â removed/ converted to shunt within 72 hrs ⢠Post OP MRI â 72 hrs ⢠Tumour markers
- 41. Complications of surgery ⢠Sitting position-air embolism, subdural hygromas, pneumocephalus ⢠Hemorrhage â Incomplete resection ⢠HCP â air, debris , blood ⢠Hemiparesis from brain retraction/ sacrifice of bridging vein ⢠Impairment of extraocular movements (up gaze, convergence), pupillary abnormalities ⢠Interhemispheric â sensory or stereognostic deficits ⢠Occipital transtentorial- visual field defects
- 42. prognosis ⢠Prognosis is dependent on histologic diagnosis. ⢠In germinoma, the long-term survival rate is 90% with RT. Poor prognostic indicators included positive CSF cytology for tumor cells and increased β-HCG levels. ⢠In NG-GCT, in a study the 5-year survival rate with RT was 36%. 1 The 2-year survival with chemotherapy was 62%. There was a 10% mortality rate from toxicity of chemotherapeutic agents
- 43. ⢠⢠In pineocytoma, total or subtotal resection has a mean survival rate of 8 years. (Tsumanuma et al) ⢠⢠In pineoblastoma, mean survival rate of 25 months after gross total resection and RT. (Lee et al)
- 44. ⢠Thank you!!
Hinweis der Redaktion
- HCG is present in ECG Embryonal cell Ca Chriocarcinoma germinoma
- A normal pineal gland can become calcified In children younger than 10 years, physiologic calcification of a healthy pineal gland is usually not detectable on plain radiographs. Pineal calcification can be seen in patients with pineal cell tumors.
- Below is a MR of a germinoma arising in the pineal region (#1). #2 points to an area of intraventricular seeding.
- axial (post gad) MR of a pineal non-germinomatous germ cell tumor
- Part I: Midline infratentorial supracerebellar approach Step 0: Position The sitting position is generally preferred Step 1: Skin A midline incision (white line) to bone is used extending 1-2 cm superior to the inion (*) to C1-C2 However, an inverted U-shaped incision can be used too The theoretical trajectory of transverse sinus is showed with the blue line.
- Step 2:Craniotomy Step 2a: bone landmarks Suboccipital muscles are dissected laterally and held in place using cerebellar retractors in a typical fashion to reveal underlying bone The widest part of the exposure should be at the level of the transverse sinus The bone landmarks must be recognized in order to place the craniotomy: external occipital protuberance (*), superior nuchal line (SNL) and inferior nuchal line (INL)
- Step 2b: burr holes and shape A craniotomy is preferred over a craniectomy because it is faster and seems to reduce postoperative discomfort Slotted burr holes are made at the sagittal sinus just above the torcula and at the lateral aspect of the transverse sinus bilaterally. It is not necessary to open the foramen magnum
- Step 3: Dural stage Step 3a: Dural sinuses The main pupose of the craniotomy is to expose, without injuring, the torcula (T), the inferior portion of the superior sagittal sinus (SSS), the occipital sinus (OS) and both transverse sinuses (TS). The trajectoy of the dural sinuses has been drawn Step 3b: Durotomy The dura is opened with a gentle curving incision extending from the lateralmost exposure of the transverse sinus to its contralateral exposure The incisions should extend from each side down to the midline, where the midline venous sinus can be ligated and the small cerebellar falx can be divided.
- Step 4: Cerebellar stage Step 4a: Suboccipital surface After the dura is opened, the bridging veins will be visible. The inferior vermian (IVv) and hemispheric veins (IHv) on both halves of the suboccipital surface ascend and pass below the transverse sinus to empty into the sinuses in the tentorium. These should be cauterized and divided thereby freeing up the cerebellum from the tentorium.
- Step 4b: Tentorial surface and its venous relationships In the infratentorial supracerebellar approach to the pineal region, it may be necessary to divide numerous bridging veins entering the torcula and the tentorial sinuses, including some of the superior and inferior hemispheric and vermian veins, and the vein of the cerebellomesencephalic fissure These veins have commonly been sacrificed without adverse effect to open the quadrigeminal region and the incisura. The posterior group of superior vermian (SVV) and superior hemispheric veins (SHV) arise on the posterior part of the tentorial surface and descend to empty into tentorial sinuses The superior hemispheric veins (SHV), which drain the tentorial surface, are divided into an anterior group, which empties into the Galenic system, and a posterior group, like the veinshown, which empties into the tentorial sinuses. TT: Tentorium; C: cerebellum; SVV: superior vermian vein; SVV: superior vermian vein; TT: tentorium.
- In the infratentorial supracerebellar approach to the pineal region, it may be necessary to divide numerous bridging veins entering the torcula and the tentorial sinuses, including some of the superior and inferior hemispheric and vermian veins, and the vein of the cerebellomesencephalic fissure These veins have commonly been sacrificed without adverse effect to open the quadrigeminal region and the incisura. The posterior group of superior vermian (SVV) and superior hemispheric veins (SHV) arise on the posterior part of the tentorial surface and descend to empty into tentorial sinuses The superior hemispheric veins (SHV), which drain the tentorial surface, are divided into an anterior group, which empties into the Galenic system, and a posterior group, like the vein shown, which empties into the tentorial sinuses. TT: Tentorium; C: cerebellum; SVV: superior vermian vein; SVV: superior vermian vein; TT: tentorium.
- With the microscope in place, the arachnoid (*) overlying the quadrigeminal region is opened sharply. This arachnoid is usually thickened and partially opaque when tumors are present. TT: tentorium.
- FIGURE 37.24 Intraoperative findings in case of a germinoma as presented in Figure 37.8. (A) ď Photograph during the opening of the arachnoid membranes in the pineal region with the tumor in the background (B) ď Appearance of the tumor before resection (C) ď Appearance after complete tumor removal (D) ď Endoscope- assisted approach with the view into the third ventricle presenting the intermediate mass, the choroid plexus at the roof, both fornices, and the anterior commissure
- Complications of Surgery Serious complications of pineal tumor surgery, regardless of the route used, are related to the nature of the tumor and its potential for intra- or postoperative hemorrhage.15 Hemorrhage has played a major role in most of the surgeryrelated deaths and can occur with a delay of as long as several postoperative days. This phenomenon is most prevalent with malignant pineal cell tumors (pineoblastomas), which tend to be soft and highly vascular. Hemorrhage can occur before surgery as a so-called pineal apoplexy or can be associated with stereotactic biopsy. Complications of the sitting position, particularly with the posterior fossa approach, include air embolism, hypotension, and cortical collapse when hydrocephalus of significant degree is relieved by tumor removal. The incidence of cortical collapse can be reduced by preoperative shunting or third ventriculostomy to allow the ventricular system a chance to accommodate over several days before the major operation. This phenomenon can occur in varying degrees and, although striking on the postoperative computed tomography scan, gradually improves without major neurologic complications for the patient. Subdural shunting is rarely required to relieve chronic hygromas resulting from this complication. The complications of the interhemispheric approach are related to retraction of the parietal lobe with transient sensory or stereognostic deficits on the opposite side. These have not been serious or permanent. Unlike the occipital transtentorial approach, the interhemispheric approach has not been associated with visual field defects. Regardless of the operative approach used, various pupil abnormalities, difficulty focusing or accommodating, interocular palsies, and limitation of upward gaze can be expected whenever the tumor is dissected from the quadrigeminal region. These deficits improve gradually but may last for many months or as long as a year before normal function returns. Manipulation of the brain adjacent to the third ventricle can lead to impaired consciousness. The fourth cranial nerve is generally caudal to the tumor and is rarely identified or injured. Ataxia has been minimal and usually transient. The incidence and severity of deficits are increased with prior radiation therapy, presence of symptoms preoperatively, and a high degree of malignant and invasive characteristics. Shunt malfunction or blockage of a ventriculostomy can occur in as many as 20% of patients after surgery