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Anatomy and related vascular structures of pineal region.pathological classification and incidence. Clinical Presentations and different diagnostics modalities. Different surgical approaches for pineal region

Anatomy and related vascular structures of pineal region.pathological classification and incidence. Clinical Presentations and different diagnostics modalities. Different surgical approaches for pineal region


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  1. 1. Medhat Mustafa, MD, Department of Neurosurgery Suez Canal University, Ismailia, Egypt
  3. 3. Objectives Anatomy Vascular relations Pathological types Diagnosis Treatment
  4. 4. Normal anatomy of pineal region
  5. 5. The pineal gland is a small (5-8 mm AP diameter), pine-cone shaped, midline brain structure.
  6. 6. It is located in the quadrigeminal cistern posteriorly to the third ventricle, inferiorly to the splenium of the corpus callosum and the internal cerebral veins and superiorly to the tectal plate of midbrain.
  7. 7. The presence of calcifications with a diameter greater than 1 cm or before the age of 10 is mainly pathologic.
  8. 8. The choroidal branches of the posterior cerebral artery are the feeding vessels of the gland.
  9. 9. ARTERIAL SUPPLY PCA • – P1 • Quadrigeminal a.– superior colliculi – P2 • M P.ch – Pineal body, corpora quadrigemina, tela choroidea ventriculi , thalamus • L Pch – Choroid plexus of lat. Ventricle, LGB, thalamus
  10. 10. ARTERIAL SUPPLY – P3/P4 • Medial occipital artery – Calcarine artery – calcarine sulcus – Parieto-occipital artery – parieto-occipital sulcus • Posterior pericallosal artery – SCA • Inferior colliculi
  11. 11. • Great cerebral vein of Galen – Pineal veins(sup & inf) -- pineal body, trigonum habenulae – Quadrigeminal veins(sup&inf) —corpora quadrigemina – Superior vermian vein – superior vermis – Precentral cerebellar vein – cerebellum, superior cerebellar peduncle – Posterior pericallosal vein – Internal occipital vein
  12. 12. •VEINS OF PINEAL REGION • Internal cerebral veins – Septal veins(ant&post) –septum pellluvidum – Anterior caudate vein – caudate nucleus – Thalamostriate(terminal) vein – Choroidal vein – choroid plexus lat. Ventricle – Medial atrial (trigonal)vein – Trigone – Direct lateral veins
  13. 13. VEINS OF PINEAL REGION • Basal vein of Rosenthal – Vena cerebri media profunda – Venae centrales (striate) inferiores – Vena cerebri anterior – Vena apicis cornua temporalis (hippocampal vein) – Vena atrii lateralis (lateral atrial vein ) – Vena cornus temporalis (atriotemporal vein )
  14. 14. Pathology • Pineal gland tumors
  15. 15. Pathology There is a wide range of pineal region masses due to the multiple different cell types found there.
  16. 16. Pathology • Tumors arising from the pineal gland are mainly classified into germ cells and pineal parenchymal tumors.
  17. 17. Pathology • Germ cell tumors • are the most common, representing more than 50% of pineal tumors.
  18. 18. Pathology Germ cell tumors They are divided into non-germinomatous and germinomatous. Based on the literature, germ cell tumors affecting the pineal region most frequently occur in males, especially non- germinomatous tumors with an incidence of 90%.
  19. 19. Germ cell tumor Germinoma Nongerminoma embryonic extraembryonic Mature Immature choriocarcinomas yolk sac carcinomas Teratoma embryonal carcinoma
  20. 20. Germ cell tumors Germinomas constitute the majority, accounting for 60-80% of all germ cell intracranial neoplasms, 3-5% of intracranial tumors in children and 0.4-1% of intracranial tumors in adults.
  21. 21. Germ cell tumors Non-germinomatous germ cell tumors (teratomas, embryonal carcinomas, choriocarcinomas and yolk sac carcinomas) are rare. Teratomas are the second more common germ cell tumor in the pineal gland.
  22. 22. Pathology Tumors arising from pineal parenchyma account for 14-27% of pineal gland tumors Pineocytoma, pineoblastoma, pineal parenchymal tumor of intermediate differentiation (PPTID) papillary tumor of the pineal region (PTPR) are included.
  23. 23. Pineocytomas are reported to account for up to 30% of pineal tumors. Pineoblastoma is a common malignant tumor detected to the pineal region, with an incidence up to 50%. PPTID are less often, but account for at least 20% of this category of tumor. Pathology Tumors arising from pineal parenchyma
  24. 24. Pathology The remaining 25% of pineal region lesions include pineal cysts masses expanding from adjacent anatomical structures, such as gliomas arising from the adjacent brain parenchyma and meningiomas arising from the tentorium. Lipomas, pineal cysts, metastases and arachnoid cysts are also included.
  25. 25. Germinomas are mainly seen in children and young adults, with a peak at the age of 10-12. Approximately, 90% of the patients are less than 20 years old at the time of diagnosis. Pathology
  26. 26. Germinoma is histologically similar to dysgerminoma of the ovary and seminoma of the testis. It is identified in the pineal region (50-65%), the suprasellar region (30%) and the basal ganglia-thalamus (5-10%). Pathology
  27. 27. Pathology Germinomas are mainly aggressive tumors that often infiltrate the adjacent anatomical structures and spread through the cerebrospinal fluid (CSF) into the spinal canal.
  28. 28. Teratomas are distinguished in mature, immature and those with malignant transformation, with Alpha fetoprotein (AFP) and Human chorionic gonadotropin (HCG) elevated in immature type.
  29. 29. Pathology Teratomas more often occur in children under 10 years of age. The most common location is the pineal region, but they may also occur in the third ventricle and posterior fossa.
  30. 30. Pathology The rest of the non-germinomatous germ cell tumors are highly malignant. AFP and HCG in CSF are elevated in yolk sac carcinoma and choriocarcinoma respectively.
  31. 31. Pathology Pineocytomas (WHO 2016, grade I) are benign tumors, most commonly presented in adults with a mean age of 38 years old. It is extremely rare for them to metastasize and spread into the spinal canal.
  32. 32. Pathology Pineoblastomas (WHO 2016, grade IV) are highly malignant tumors mainly affecting children and young adults under 20 years old. The tumor infiltrates adjacent anatomical structures and often spreads into the spinal canal.
  33. 33. Pathology PPTID (WHO 2016, grade II or III) has histological features between those of pineocytoma and pineoblastoma without an age restriction, although there is a peak age in young adults. It rarely causes drop metastases.
  34. 34. Pathology PTPR (WHO 2016, grade II or III) is a neuro-epithelial neoplasm arising from ependymocytes of the subcommissular organ. It affects a wide range of ages from 5 to 65 years old. Drop metastases arise in 7% of the cases.
  35. 35. Clinical presentation
  36. 36. Clinical presentation The symptoms are related to tumor local invasion and/or compression of adjacent structures, depending on the size and aggressiveness of the mass.
  37. 37. Clinical presentation Compression of the midbrain anteriorly with obstruction of the aqueduct of Sylvius leads to obstructive hydrocephalus and symptoms of increased intracranial pressure.
  38. 38. Clinical presentation Compression of the tectal plate causes visual problems (Parinaud syndrome) Infiltration of the thalamus may lead to loss of sensation and weakness on one side of the body.
  39. 39. Clinical presentation When the tumor expands into the suprasellar region, symptoms related to the hypothalamus may occur. Precocious puberty is a result of elevated HCG.
  40. 40. Imaging
  41. 41. CT and MRI are critical for the evaluation of the location, size and shape of the pineal tumor. The imaging findings are not specific.
  42. 42. The differential diagnosis is difficult and often a biopsy is required to determine the histological type.
  43. 43. However, there are crucial signs that could narrow the differential diagnosis.
  44. 44. In the presence of calcifications, the pattern of involvement is helpful Germinomas tend to engulf the calcifications, in contrast to pineal parenchymal tumors, where calcifications are distributed.
  45. 45. Additionally, some tumors, like pineoblastomas, infiltrate adjacent structures, the third ventricle and the basal cisterns, whereas others like germinoma exhibit edema.
  46. 46. A CT scan of the brain constitutes the first imaging method and can be beneficial for the assessment of the pineal region and the presence of hydrocephalus, calcifications and hemorrhage, although it should be avoided in children due to radiation.
  47. 47. MRI of the brain is considered the gold standard method for the evaluation of the CNS. This method is superior to CT due to the absence of radiation and its high sensitivity, the ability to illustrate anatomical details of the brain and the excellent separation of gray and white matter.
  48. 48. The imaging characteristics of pineal germinoma are not specific and include a well-circumscribed, ovoid or lobulated, relatively homogenous mass, that overwhelms the pineal gland.
  49. 49. The signal on T1 and T2 WI is variable, most commonly slightly hyperintense on both sequences. They may demonstrate cystic or hemorrhagic components and a tendency to invade the adjacent tissues, surrounded by edema.
  50. 50. Markedly enhancement may be seen on MR, due to their cellularity, either homogenous or inhomogeneous with regard to their content
  51. 51. The differential diagnosis between germinoma and pineal parenchymal tumors is not accurate. A helpful sign could be the engulfment of the normal physiologic calcification, while pineal parenchymal tumors appear exploded.
  52. 52. If the imaging findings are present in a young male, the diagnosis is in favor of germinoma.
  53. 53. Additionally, a hypointensity on T2WI may also be helpful in the differential diagnosis of a germinoma from a pineal parenchymal tumor.
  54. 54. Teratoma is composed of all three germ cell lines presenting a heterogeneous appearance. The majority of non-germinomatous germ cell tumors contain fat, calcifications most often clump like, hemorrhage, teeth, hair, cystic and solid components.
  55. 55. Hydrocephalus occurs frequently. Detection of a midline heterogeneous mass in a child should suggest the diagnosis of a teratoma.
  56. 56. Pineoblastoma, a highly aggressive tumor may contain hemorrhagic, necrotic or cystic foci. MRI can identify the tumor and the intrusion of the adjacent tissue but also the presence of leptomeningeal and subependymal metastases. These tumors are ill-defined because of their tendency to infiltrate the surrounding structures.
  57. 57. On T2 WI, the majority is isointense, unless a cystic component produces a high signal. These tumors exhibit restriction on DWI and intense, inhomogeneous enhancement after Gd administration.
  58. 58. Central necrotic elements must be differentiated from cystic by the administration of Gd, whereas the differentiation from a pineal cyst may be difficult.
  59. 59. Pineocytomas are well defined lesions with solid, enhanced components. Cystic or hemorrhagic areas can be demonstrated, thus the signal on T1 and T2 WI is variable. The well defined contours are helpful to the differential diagnosis in order to distinguish them from pineoblastomas
  60. 60. A calcified pineal mass in a female is more likely to represent a pineocytoma.
  61. 61. Regarding PTPR, although the presence of high signal on T1WI is a relatively specific finding, variable signal intensity on T1WI and hyperintensity on T2WI are presented. According to Chang et al the presence of high signal on T1WI may be related to the concentration of protein and glycoprotein content in the cystic regions and this may be a common imaging finding for papillary tumors.
  62. 62. Drop metastasis is a term for intradural extramedullary metastatic lesions of the spine that result from subarachnoid spread of a primary brain tumor.
  63. 63. They arise from pineal tumors, ependymomas, medulloblastomas, primitive neuroectodermal tumors (PNET), glioblastomas multiform, anaplastic astrocytomas and oligodendrogliomas.
  64. 64. Drop metastases occur in 5% to 30% of children, either early, at the time of diagnosis of a brain tumor, or during the follow-up. Medulloblastoma is the most common source, accounting for half of the patients with drop metastases.It is mainly diagnosed in children under 10 years of age, with a second smaller peak between 15 and 35 years old. Glioblastoma multiform is the second most common tumor with an incidence of 1% of cases and in 15% of all patients with drop metastases.
  65. 65. The two most common pineal tumors causing drop metastases are germinomas and pineoblastomas. Leptomeningeal seeding has been reported in 6% to 14% of patients with germinomas and as high as 45% in all patients with pineoblastomas.
  66. 66. Pineal metastasis is uncommon, affecting 5% of patients with pineal tumors who underwent surgery. Lung cancer is the most common cause, however breast, kidney and gastrointestinal tract are also related. Leptomeningeal spreading is common and is present in 67% of patients.
  67. 67. Therapy
  68. 68. The therapeutic plan of pineal tumors depends on the histological type, the size and the presence of drop metastases.
  69. 69. Surgery in the pineal region is anatomically difficult and is not usually possible to completely remove the tumor.
  71. 71. ADJUVANT THERAPY • RADIOTHERAPY – For malignant germ cell/pineal cell tumors: 4000cGy : Ventricular system 1500cGy : Tumor bed Ø180 cGy daily fractions Ø 3500 cGy to spine if spinal seeding radiographically documented. ØRT maybe withheld: for histologically benign pineocytoma /ependymoma completely resected.
  72. 72. ADJUVANT THERAPY • Germinoma with raised b- hCG has less favorable prognosis. • Side effects of – Cognitive deficits. – Hypothalamic/Endocrine dysfunction. – Cerebral necrosis. – De novo tumor formation.
  73. 73. ADJUVANT THERAPY • CHEMOTHERAPY – Indications • Non germinomatous malignant germ cell tumors • Germinoma with syncytiotrophoblastic giant cells • Recurrent /disseminated pineal cell tumors – Cisplatin/carboplatin + Etoposide – Others: vincrisNne/lomusNne/cyclophosphamide
  74. 74. RADIOSURGERY • For tumors less than 3 cm • For tumors that recur locally.
  75. 75. SURGICAL ANATOMY • Most tumors arise from or attached to the undersurface of velum interpositum. • Tumors rarely extend above velum . • Blood supply comes from within velum mainly from M P.ch & L P.ch with anastomoses to pericallosal & quadrigeminal artery. • Most tumors are centered at pineal gland, some extend to For. Monro.
  76. 76. SURGICAL ANATOMY • Mostly, ICV, Galen , Rosenthal & precentral cerebellar veins surround or cap the periphery of these tumors. • Rarely, ICV are ventral to tumor. • Most tumors are not highly vascular except – Pineoblastomas – Hemangioblastomas – Hemangiopericytomas (Angioblastic meningioma)
  77. 77. •MANAGEMENT OF HYDROCEPHALUS • If complete tumor removal is anticipated: EVD at surgery: removal on POD:2/3 • Conversion to VP shunt • ETV preferred : – Gradual reduction of ICP – Eliminates potential complications • Infection • Overshunting • Peritoneal seeding by malignant cells
  78. 78. OPERATIVE BIOPSY • For adjuvant therapy • Metastatic workup • Prognosis & long term follow up.
  79. 79. OPERATIVE • OPEN BIOPSY – More extensive tissue sampling in view of heterogeneity & mixed cell population – Debulking: favorable response to adjuvant therapy – Risk of postop. bleeding in incompletely resected tumor bed.
  80. 80. OPERATIVE BIOPSY • STEREOTACTIC BIOPSY – Suited for patients with multiple lesions, clinical conditions that contraindicate open surgery/ general anesthesia. – Multiple specimens to be obtained. – Side cutting cannula preferred over cup forceps – Hemorrhage: Continuous suction & irrigation x 15 min.
  81. 81. OPERATIVE STEREOTACTIC APPROACHES • INDICATIONS – For biopsy to achieve diagnosis – Aspiration of cystic masses – Radiosurgery for treatment : meningioma, pineocytoma, AVM – As adjuvant management with chemotherapy for germ cell neoplasms & pinealoblastomas.
  82. 82. OPERATIVE STEREOTACTIC APPROACHES • ADVANTAGES – Resection may not be necessary depending on the histopathology – Biopsy may guide effective non-surgical therapies – Radiosurgery for small volume pathologies • DISADVANTAGES – Small biopsy volume : Difficulty in diagnosis – Experienced neuropathology team required – Risk of radiation related damage & deficits
  83. 83. OPERATIVE STEREOTACTIC BIOPSY • ANTEROLATEROSUPERIOR APPROACH: – Low precoronal entry point just behind the hairline & just above the superior temporal line . – Needle trajectory: Through the frontal lobe , underneath the lateral ventricle & lateral & inferior to ICV. • POSEROLATEROSUPERIOR APPROACH: – Entry point near PO junction, for tumors extending laterally or superiorly.
  84. 84. OPERATIVE ENDOSCOPY • BIOPSY : In conjunction with ETV • FLEXIBLE ENDOSCOPE : Limited trajectory to tumor through foramen magnum • RIGID ENDOSCOPE : Low frontal burr hole • LIMITATIONS • Limited tissue sampling • Difficulty in hemostasis
  85. 85. OPERATIVE INFRATENTORIAL SUPRACEREBELLAR • INDICATIONS – Tumor with major bulk in midline – Tumor ventral to velum interpositum & deep venous system • ADVANTAGES – Minimal risk to deep veins – No normal neural tissue violated enroute – Exposure comparable with that of other routes
  86. 86. OPERATIVE INFRATENTORIAL SUPRACEREBELLAR • COMPLICATIONS – Risks of sitting position – Limited upgaze & convergence – Ataxia – Cognitive impairment – Akinetic mutism • More frequent in patients having preop deficits, prev. radiation or invasive tumors
  87. 87. SURGICAL TECHNIQUE • Postion: Sitting , ¾ prone, concorde • Head flexed: tentorium parallel to floor • Midline incision : inion- C3 • Burr holes: – At sagipal sinus above torcula – At lateral aspect of transverse sinus – Suboccipital Midline above for. magnum
  88. 88. SURGICAL TECHNIQUE • Durotomy:gentle curving incision • Microscope with variable objective/275mm • Bridging & precentral cerebellar veins cauterized & divided. • Until arachnoid is opened & cerebellum freed from brainstem, the trajectory is to aimed at Vein of Galen to avoid injury to ICV/Rosenthal V.
  89. 89. SURGICAL TECHNIQUE • Internal debulking/capsule dissection • Most difficult & dangerous part : inferior portion of tumor adherent to dorsal midbrain. • Copious irrigation to remove all clots that can block aqueduct.
  90. 90. LATERAL PARAMEDIAN INFRATENTORIAL • INDICATIONS • Biopsy • Small quadrigeminal area tumor • ADVANTAGE • Minimal damage to neural Nssues • Useful in steep tentorium • Reduced risk of air embolism • DISADVANTAGES • Narrow space • Difficult to reach tumor portion extending to inferoposterior part of 3rd ventricle
  91. 91. LATERAL PARAMEDIAN INFRATENTORIAL • POSITION – On the side: usually right side down – Upper part of trunk raised 30` – Head flexed with neck stretched & rotated 45` face down
  92. 92. LATERAL PARAMEDIAN INFRATENT • SURGICAL TECHNIQUE – S-shaped incision behind mastoid – Oval craniectomy close to sigmoid sinus laterally & transverse sinus superiorly – Durotomy : cruciate – Bridging veins divided, petrosal & precentral cerebellar veins preserved. – Tentorial incisura reached, preserving SCA.
  93. 93. SUPRATENTORIAL APPROACH • INDICATIONS – Tumors extending superiorly – Tumors involving or destroying the posterior aspect of corpus callosum – Tumors deflecting the deep venous system dorsolaterally – Tumors extending laterally to region of trigone – Tumors displacing deep veins in ventral direction ( e.g.. Meningiomas)
  94. 94. OCCIPITAL TRANSTENTORIAL • INDICATIONS • Tumors straddling or lying above the tentorial notch • Vascular lesions : varices of vein of Galen, AVM, P3/4 PCA aneurysms. • ADVANTAGES • Excellent view both above & below the notch • DISADVANTAGES • Damage to occ. Lobe: visual field defects. • Damage to splenium • Difficult to access subtentorial C/L porNon of tumor
  95. 95. OCCIPITAL TRANSTENTORIAL • Surgical technique – Position: semiprone with nondominant side down – Incision: U-shape – Craniotomy : 6 burr holes : 2 on left, 2 on right of sag. Sinus ,1 just rostral to trans. Sinus & 1 parietal. – Durotomy: T- shape & reflected along sinuses – Retractor on inferior surface of occipital lobe
  96. 96. OCCIPITAL TRANSTENTORIAL • Falx retracted medially • Ventricular drain placed in occipital horn • Tentorium cut 1-1.5 cm from & parallel to straight sinus. • Quadrigeminal cistern opened , CSF drained • Veins visualized : Galen vein – right Rosenthal —ICV—precentral cerebellar
  97. 97. OCCIPITAL TRANSTENTORIAL • Cleavage plane found in small tumor • Debulking in large tumor • For hypervascular tumor: feeding arteries identified & coagulated prior to debulking . • To avoid venous injury, total resection is not necessary & should not be apempted. • Immaculate hemostasis, water-tight dura closure.
  98. 98. TRANS -VELUM INTERPOSITUM • INDICATIONS • Huge tumors in pineal region/posterior 3rd ventricle • Tumors extending anterior to adhesio interthalamica • ADVANTAGES • Tumors extending into lateral ventricular can also be managed • DISADVANTAGES • Damage to anterior corpus callosum • Damage to fornix
  99. 99. TRANS -VELUM INTERPOSITUM • Surgical technique – Position : Supine with head elevation 20` in pin – Coronal/Quadrangular skin flap on nondominant side – Quadrangular bone flap , extending to midline & anterior to coronal suture – Right frontal lobe retracted, corpus callosum exposed , split 3-4 cm to enter pars centralis
  100. 100. TRANS -VELUM INTERPOSITUM • Velum interpositum ( choroid plexus + tela choroidea + ICV ) cut just lateral to tenia fornicis & medial to choroid plexus of lateral ventricle • B/L fornices & IJV retracted medially to explore tumor b/w these structures & right thalamus
  101. 101. • Indications – Large tumors > 4.5 cm –Tumor arising from tentorium or extending above & below – Tumor well below plane of cerebellar retraction ( 2cm below sup. Surface of cerebellum) – Very vascular tumors – Tumors encasing imp. Venous structures.
  102. 102. COMBINED SUPRA-INFRATENT. TRANSSINUS • Cerebral angiogram is mandatory to look for venous anatomy, size & communicaNon b/w transverse sinus & deep venous system. • VP Shunt 2-4 weeks prior to planned surgery • Somatosensory evoked potenNals from UL & LL B/L & BAER monitoring during surgery • Position : Semiprone with proposed trans. Sinus section placed inferiorly.
  103. 103. COMBINED SUPRA-INFRATENT. TRANSSINUS • U-shape incision • Burr holes inferior to trans. Sinus (4) & just above for. Magnum (2) – suboccipital craniotomy • Trans. Sinus separated & occipital craniotomy performed on one side followed by other. • Suboccipital dura opened in transverse fashion inferior to trans. Sinus
  104. 104. COMBINED SUPRA-INFRATENT. TRANSSINUS • Occipital dura is then opened parallel to sinuses • 20 G buperfly needle inserted in trans. Sinus just lateral to torcula & medial to temporary clip placed for test occlusion x 5 min. • Nondominant trans. Sinus can be safely sectioned if : venous pressure > 5 mm Hg, no brain swelling, no change in evoked potentials. • Tentorium is then cut parallel to straight sinus.
  105. 105. COMBINED SUPRA-INFRATENT. TRANSSINUS • Deep vein injury : repair with 7-0 prolene/8-0 nylon • Trans. Sinus can be reconstructed with a short vein gray interposed with 6-0 prolene. • Not necessary to suture tentorium • Dural gray to allow watertight closure & expansion of posterior fossa. • If brain swelling + : suboccipital bone kept out.
  106. 106. COMBINED SUPRA-INFRATENT. TRANSSINUS • Postoperative Care – Look for respiratory abnea in tumors compressing brainstem – CT on POD1 to l/f pneumocephalus/clots – Nystagmus/ataxia/oscillopsia – Ventricular drain x 3-4 days – 20 cm above head x 24 hrs– test clamping x 24 hrs with ICP monitoring —CT to l/f ventricle size. – Vision abnormalities
  107. 107. COMPLICATIONS OF SURGERY • Postop. Hemorrhage/apoplexy • Pupillary abn., accomodation abn., ocular palsies, upward gaze paresis, ataxia, impaired consciousness, shunt malfunction, ETV blockage. • Sitting position: air embolism, hypotension, cortical collapse, subdural hygroma • Parietal approaches: sensory/stereognostic deficits. • Occipital : Visual field defects
  108. 108. Thank you