Saturday 18 April 2015

Cerebellopontine angle and Retromastoid Craniectomy

Retromastoid craniectomy is an important neurosurgical approach for operating lesions of the cerebellopontine angle ( CP angle).
Commonest CP angle tumor is acoustic schwannoma  Other common tumors of CP angle are CP angle meningioma and epidermoid. 
Retrosigmoid region is the part of the cranium situated just behind the sigmoid sinus and below the transverse sinus.
The lateral cerebellomedullary and cerebellopontine cisterns are encountered in this approach.
Three important arteries are encountered bilaterally in this region: 
Posterior inferior cerbellar artery (PICA),
Anterior inferior cerbellar artery (AICA), &
Superior cerebellar artery (SCA)

PICA arises from the vertebral artery and is seen in relation to the lower cranial nerves. Occasionally, it may reach high enough to come in contact with the lower limit of the VII/VIII complex.
AICA arises from the basilar artery and distal part is seen in relation to the VII/VIII complex. This nerve-related segment is divided into premeatal, meatal, and postmeatal parts. The meatal part forms a loop ( meatal loop) which travels along the VII/VIII complex for a variable distance toward the internal acoustic meatus. Several nerve-related branches of AICA exist: the internal auditory artery, recurrent perforating arteries, subarcuate artery , and cerebellosubarcuate artery. The internal auditory artery is the most constant branch of the AICA.
The SCA arises from the basilar artery quadrification courses back along the side of the mid brain and pons , and is seen in the cerebellopontine angle in front of the Vth cranial nerve.
The veins of the posterior fossa can be divided into superficial , deep , and bridging veins. Anterior hemispheric veins , veins of the cerebellopontine fissureand middle cerebellar peduncular veins, lateral medullary and lateral mesencephalic veins, and petrosal bridging veins.
Petrosal veins are divided into superior and inferior veins which drain into superior or inferior petrosal sinus.
The superior petrosal veins are the most prominent bridging veins of the posterior fossa. They drain blood from a large part of the cerebellar hemisphere and brainstem. Superior petrosal veins are divided into a lateral, intermediate or medial group based on their relation to the site of drainage into the superior petrosal sinus and IAM.
Cranial nerves V through XII are seen in this approach. CN V is a stout nerve arising from the upper and outer aspect of the anterior pons. Its motor component arises as a separate rootlet just anterior to the main nerve , and both quickly merge to form a single nerve. Loops of the SCA can be seen in front of the nerve at this level.The nerve courses anteriorly and outward to cross the petrous apex and enter into the Meckel,s cave.
Cranial nerve VI arise medially at the pontomedullary sulcus. It courses up the surface of the clivus exiting the posterior fossa through Dorello’s canal. It enters the posterior cavernous sinus on its way through the middle cranial fossa to the orbit.
The VII/VIII complex, with the nervus intermedius in between, arises from the pontomedullary junction surrounded by the same fascial sheath. They course backward, downward, and outward toward the internal acoustic meatus.

Cranial nerves IX, X, XI arise as a linear series of rootlets from just below the pontomedullary junction in the anterolateral medullary sulcus. After their origin, they converge outward and backward toward the jugular foramen. CN XII lies in close proximity to the vertebral artery-PICA junction. Its many rootlets exit though the hypoglossal canal.

Retromastoid craniectomy can be performed with the patient in sitting, park-bench, three-quarter prone position, lateral or supine position.
The description by Robert L. Martuza for surgery of acoustic neuroma in supine position is straight forward. The patient is positioned supine with one or two folded soft cotton blankets beneath the ipsilateral shoulder and padding beneath the knees and is secured with one or two belts to allow the table to be rolled if necessary during surgery. Too much turning of the body can place the shoulder in a position that interferes with surgery.
The head should be fixed in Mayfield or Sugita frame.
A retroauricular lazy 'S' or linear or C shaped incision is made in the scalp which is paramedian and about 5 mm medial to the mastoid notch.
Dr Robert L. Martuza suggests a linear incision approximately 3 cm behind the insertion of the pinna and going from approximately 1 cm above the tip of the pinna to 1 cm below the ear lobe. The muscles are then divided inferiorly and self retaining retractors are placed.
The sigmoid sinus lies beneath the mastoid groove.
The transverse sinus and sigmoid sinus generally curve around the asterion. Therefore, a burr hole is placed medial to the asterion.
Craniectomy is done after placing a burr hole.  After craniectomy the inferior edge of the transverse sinus and the medial edge of the sigmoid sinus should be defined with the help of rongeurs. The transverse sinus lies above the upper limit of craniectomy and sigmoid sinus is lateral to the craniectomy.typically a K-shaped incision or linear incision about 2 cm medial to the edge of the sigmoid sinus  is made in the dura with the edges being based on the sinus.
After retracting the cerebellar hemisphere medially and superiorly cisterna magna comes into the view. The arachnoid of this cistern is opened and CSF is drained and this maneuver relaxes the cerebellum, obviating the need for further retraction.

Middle neurovascular complex in CP angle includes AICA, pons and middle cerebellar peduncle. The AICA arises at the pontine level and courses in relationship to the abducens, facial & vestibulocochlear nerve to reach the surface of the middle cerebellar peduncle, where it courses along the cerebellopontine fissure and terminates by supplying the petrosal surface of the cerebellum. Common operations in this region are for removal of acoustic schwannomas and for the relief of hemifacial spasm.
Acoustic schwannomas, as they expand, may involve a majority of the cranial nerves, cerebellar arteries, and parts of the brain stem.

Lateral view of skull to show Asterion: Meeting point of Parietomastoid suture, Occipitomastoid suture and lambdoid suture.   

Sources:
1. Chapter: Suboccipital retrosigmoid surgical approach for vestibular schwannoma ( acoustic neuroma) by Robert L. Martuza in Schmidek & Sweet operative neurosurgical techniques- indications, methods & results. Alfredo Quinones- Hinojosa. 6th ed. (Elsevier Saunders) 
2. Rhoton Cranial anatomy and surgical approaches, Neurosurgery, Albert L. Rhoton, Jr  
3. Oerative Neurosurgical Anatomy by Damirez T. Fosset and Anthony J. Caputy ( Thieme publication)
4, Wikipedia

Sunday 5 April 2015

Neurosurgical Management of CNS Tumors: Review of Recent Advances

Due to technological advancements and better understanding of brain tumors, there had been a significant improvement in the clinical outcome of brain tumors. The majority of the brain tumors are now diagnosed at a very early stage. CT scan or MRI of the brain is a very common diagnostic tool and help to diagnose any lesion inside the brain.
All the brain tumors need not be operated. Non invasive single dose radiation therapy like Gamma knife or Cyberknife is a noninvasive way of treating small lesions of the brain.
Stereotactic biopsy helps in minimal invasive way of diagnosing brain tumor.
Stereotactic Radiotherapy ( SRT) is used to treat malignant lesions of the brain. Even multiple metastasis of systemic cancers of the body to the brain can be treated with surgery and SRT.
Meningioma which constitute about 15% of all the primary brain tumors can be excised completely without any recurrence.
So, if somebody is diagnosed as a case of brain tumor, he or she should not lose hope. Unlike other tumors of the body, the brain tumors are not labeled as cancer and non cancer. Rather, there are differerent grades of the tumor ( WHO Grade 1 to IV). Grade 1 tumors are usually benign and do not require chemotherapy or radiotherapy.
Neuro-oncology constitutes the major part of clinical practice of neurosurgeons. Almost every neurosurgeon operates and manages cases of CNS tumours. Many advances have occurred in last few decades and now many options are available for investigating and treating such lesions. Translational research has provided hope for the better clinical outcome in future. MRI with contrast , MR spectroscopy, PET scan are the new investigative modalities to detect neoplasis and metastasis . Immunotherapy, vaccine therapy, molecular based targeted therapy, Bevacizumab, Temozolomide, Brachytherapy, stereotactic radiotherapy, stereotactic biopsy of the lesion, gamma knife are the new treatment options for treating CNS tumors.

Classification of CNS tumors by WHO (World Health Organization) in 2007 identifies 7 broad categories of CNS tumors:
1. tumors of neuroepithelial tissue
2.tumors of cranial and paraspinal nerves
3. tumors of the meninges
4. lymphomas & hematopoietic neoplasms
5.germ cell tumors
6. tumors of the sellar region
7. metastatic tumors

This framework is necessary to have an idea about all CNS tumors.

It is very important to use authentic terms for the cns tumors and have an overall view about all the tumors of the central nervous system. Various nomenclatures and classifications are used for description. Therefore, it is imperative to know what is most authentic and easiest way to remember. 

The pathological classification of cns tumors is the most common and most practical way of nomenclature and classification of such tumors.

CNS tumors, especially gliomas, contrary to the other cancers of the body , cannot be classified like Benign or Malignant or  Cancer and Non cancer or like Benign lesion of the Breast ( Fibroadenoma) and Cancer of the breast ( Ca Breast) , or Benign Prostrate Hyperplasia (BPH) and Cancer of the Prostrate. 
On the contrary, the Gliomas or Astrocytomas are classified on the basis of features of neoplasia- like cellular atypia, nuclear proliferation, vascular proliferation & necrosis. So, there is a grading of neoplasia. 
Cushing and Bailey classification was an earlier effort to classify brain tumors. 
World Health Organization ( WHO) classification of  tumors of central nervous system ( 2007)  is a comprehensive, and  authentic  source of reference.


1. Tumours of neuroepithelial tissue

1.1. Astrocytic tumours
        
        Pilocytic astrocytoma  & Subependymal giant cell astrocytoma (WHO grade I)
       
        Pilomyxoid astrocytoma & Pleomorphic xanthoastrocytoma (WHO grade II)
        
         Diffuse astrocytoma (WHO grade II) 
                        Variants:  protoplasmic,   gemistocytic,   fibrillary,  mixed
       
         Anaplastic astrocytoma (WHO grade III)
        
         Glioblastoma (WHO grade IV)
                                         a. Giant cell glioblastoma
                                         b. Gliosarcoma
    
          Gliomatosis cerebri (WHO grade III)

1.2. Oligodendroglial tumours
                 Oligodendroglioma (WHO grade II)
                 Anaplastic oligodendroglioma (WHO grade III)

1.3. Oligoastrocytic tumours
                 Oligoastrocytoma (WHO grade II)
                 Anaplastic oligoastrocytoma (WHO grade III)

1.4. Ependymal tumours
                 Subependymoma &  Myxopapillary ependymoma (WHO grade I)
                 Ependymoma (WHO grade II)
                 Anaplastic ependymoma (WHO grade III)

1.5. Choroid plexus tumours
                     Choroid plexus papilloma (WHO grade I)
                     Atypical choroid plexus papilloma ( WHO grade II)
                     Choroid plexus carcinoma (WHO grade III)

1.6. Other neuroepithelial tumours
                   Astroblastoma (WHO grade I)
                  Chordoid glioma of the third ventricle (WHO grade II)
                  Angiocentric glioma (WHO grade I)

1.7. Neuronal and mixed neuronal-glial tumours
                    Dysplastic gangliocytoma of cerebellum (Lhermitte-Duclos),
                    Desmoplastic infantile astrocytoma/ganglioglioma,
                    Dysembryoplastic neuroepithelial tumour, 
                    Gangliocytoma ,
                    Ganglioglioma
                    Papillary glioneuronal tumour
                    Rosette-forming glioneuronal tumour of the fourth ventricle, & 
                    Paraganglioma are WHO grade I tumors.
                    Central neurocytoma & Extraventricular neurocytoma , Cerebellar liponeurocytoma   (WHO grade II)
                    Anaplastic ganglioglioma (WHO grade III)
                                 
1.8. Tumours of the pineal region
               Pineocytoma (WHO grade I)
               Pineal parenchymal tumour of intermediate differentiation (WHO grade II, III)
               Pineoblastoma (WHO grade IV)
               Papillary tumors of the pineal region (WHO grade II, III)

1.9. Embryonal tumours
              Medulloblastoma (WHO grade IV)
              Medulloblastoma with extensive nodularity (WHO grade IV)
              Anaplastic medulloblastoma (WHO grade IV)
              CNS Primitive neuroectodermal tumour (WHO grade IV)
              CNS Neuroblastoma (WHO grade IV)
              Atypical teratoid/rhabdoid tumour (WHO grade IV)

2. Tumours of cranial and paraspinal nerves
              Schwannoma (WHO grade I)
              Neurofibroma (WHO grade I)
              Perineurioma (WHO grade I, II, III)
              Malignant peripheral nerve sheath tumour (MPNST) (WHO grade II, III, IV)

3. Tumours of the meninges

3.1 Tumours of meningothelial cells

 Meningioma
                   Variants ( Subtypes ): meningothelial,  fibrous (fibroblastic),  transitional (mixed),  psammomatous,  angiomatous,  microcystic,  secretory,  clear cell,  chordoid,  lymphoplasmacyte-rich, and  metaplastic
 Atypical meningioma (WHO grade II)

 Anaplastic meningioma (WHO grade III)

3.2 Mesenchymal tumours

 Lipoma , Angiolipoma ,Liposarcoma,  Leiomyoma,  Leiomyosarcoma, Solitary fibrous tumour ,  Fibrosarcoma , Rhabdomyoma, Rhabdomyosarcoma,Chondroma , Chondrosarcoma,  Osteoma,Osteosarcoma, Osteochondroma, Angiosarcoma, Kaposi Sarcoma, Ewing Sarcoma - PNET 
 Malignant fibrous histiocytoma
 Hibernoma
 Haemangioma 
 Epithelioid hemangioendothelioma
 Haemangiopericytoma & Anaplastic haemangiopericytoma (WHO grade III)

3.3 Primary melanocytic lesions
                      Diffuse melanocytosis
                      Melanocytoma 
                      Malignant melanoma 
                      Meningeal melanomatosis 

3.4 Other neoplasms related to the meninges
                     Haemangioblastoma (WHO grade I)

4. Tumors of the haematopoietic system
                       Malignant Lymphomas
                        Plasmocytoma
                        Granulocytic sarcoma
5. Germ cell tumours
                             Germinoma
                             Embryonal carcinoma
                             Yolk sac tumour
                             Choriocarcinoma
                             Teratoma
                             Mixed germ cell tumours
6. Tumours of the sellar region
                                Craniopharyngioma (WHO grade I)
                                Granular cell tumour (WHO grade I)
                                Pituicytoma (WHO grade I)
                                Spindle cell oncocytoma of the adenohypophysis (WHO grade I)
7. Metastatic Tumours


I have made an effort to create an outline and curtail the details so that any doctor can have an overview of all the tumors of CNS. The details of all such tumors should  be read in conjunction with the radiological findings which is more interesting and easy.
The most recent classification of CNS is proposed by WHO in 2016.



WHO (2016) Classification of Tumors of Central Nervous System

In year 2016, for the first time, the World Health Organization ( WHO) classification of CNS tumors uses molecular parameters in addition to histology to define many tumor entities. Major restructuring has been done in diffuse gliomas, medulloblastomas and other embryonal tumors. It has added newly recognized neoplasms, and has deleted some entities, variants and patterns that are no longer have diagnostic and/or biological relevance.

Summary of changes in new classification

  • Major restructuring of diffuse gliomas, Medulloblastomas, other embryonal tumors & incorporation of genetically defined entities
  • The term “primitive neuroectodermal tumor” is removed
  • Incorporation of a genetically defined ependymoma variant - RELA fusion positive

  • Addition of
              Newly recognized entities, variants and patterns have been added:
                              1.  IDH-wildtype and IDH-mutant glioblastoma 
                              2. Diffuse midline glioma, H3 K27M–mutant
                              3. Embryonal tumour with multilayered rosettes, C19MC-altered
                              4. Ependymoma, RELA fusion–positive
                              5. Diffuse leptomeningeal glioneuronal tumor
                              6. Anaplastic PXA
                              7. Epithelioid glioblastoma  
                               8. Glioblastoma with primitive neuronal component
                               9. Multinodular and vacuolated pattern of ganglion cell tumor
    Deletion of
                Gliomatosis cerebri
                Protoplasmic and fibrillary astrocytoma variants
                cellular ependymoma variant
                Primitive Neuroectodermal tumor


  • Addition of Brain invasion as a criterion for atypical meningioma
  • Restructuring of solitary fibrous tumor and hemangiopericytoma ( SFT/HPC) as one entity
  • Expansion & clarification of entities included in of Nerve sheath tumors, with addition of hybrid nerve sheath tumors & separation of melanotic schwannoma from other schwanomas
  • Expansion of entities included in hematopoietic/lymphoid tumors of the CNS ( lymphomas & histiocytic tumors)
                         
                                       NEW CLASSIFICATION
    Diffuse astrocyic and oligodendroglial tumors

  • Diffuse astrocytoma, IDH-mutant
                            Gemistocytic astrocytoma, IDH-mutant

  •  Diffuse astrocytoma, IDH-wildtype
  •  Diffuse astrocytoma, NOS
  • Anaplastic astrocytoma, IDH-mutant
  • Anaplasticastrocytoma, IDH-wildtype
  • Anaplastic astrocytoma, NOS

  • Glioblastoma, IDH-wildtype
                         Giant cell glioblastoma
                         Gliosarcoma
                         Epitheloid glioblastoma

  • Glioblastoma, IDH-mutant
  • Glioblastoma, NOS

  • Diffuse midline glioma, H3 K27M-mutant
  • Oligodendroglioma, IDH-mutant and 1p/19q-codeleted
  • Oligodendroglioma, NOS


  • Anaplastic Oligodendroglioma, IDH-mutant and 1p/19q-codeleted
  • Anaplastic oligodendroglioma, NOS


  • Oligoastrocytoma, NOS
  • Anaplastic Oligoastrocytoma, NOS
    Other astrocytic tumor

  • Pilocytic astrocytoma
                     Pilomyxoid astrocytoma

  • Subependymal giant cell astrocytoma
  • Pleomorphic xanthoastrocytoma
  • Anaplastic pleomorphic xanthastrocytoma
    Ependymal tumors

  • Subependymoma
  • Myxopapillary ependymoma
  • Ependymoma
                    Papillary ependymoma
                    Clear cell ependymoma
                    Tanycytic ependymoma

  • Ependymoma, RELA fusion-positive
    Anaplastic ependymoma
    Other gliomas

  • Choroid glioma of the third ventricle
  • Angiocentric glioma
  • Astroblastoma
    Choroid plexus tumors

  • Choroid plexus papilloma
  • Atypical choroid plexus papilloma
  • Choroid plexus carcinoma
    Neuronal & mixed Neuronal-glial tumors

  • Dysembryoplastic neuroepithelial tumor
  • Gangliocytoma
  • Ganglioglioma
  • Anaplastic ganglioglioma
  • Dysplastic cerebellar gangliocytoma ( Lhermitte-Duclos disease)
  • Desmoplastic infantile astrocytoma and ganglioglioma
  • Papillary glioneuronal tumor
  • Rosette forming glioneuronal tumor
  • Diffuse leptomeningeal glioneuronal tumor
  • Central neurocytoma
  • Extraventricular neurocytoma
  • Cerebellar liponeurocytoma
  • Paraganglioma
    Tumor of the Pineal region

  • Pineocytoma
  • Pineal parenchymal tumor of intermediate differentiation
  • Pineoblastoma
  • Papillary tumor of pineal region
    Embryonal tumors
    Medulloblastoma, genetically defined
                           Medulloblastoma, WNT activated
                           Medulloblastoma, SHH-activated and T53-mutant
                           Medulloblastoma, SHH-activated and T53-wildtype
                           Medulloblastoma,  non-WNT/non SHH
                                    Medulloblastoma,  group 3
                                    Medulloblastoma, group 4
    Medulloblastoma, histologically defined
                           Medulloblastoma, classic
                           Medulloblastoma, desmoplastic/nodular
                           Medulloblastoma with extensive nodularity
                           Medulloblastoma, large cell/anaplastic
    Medulloblastoma NOS

  • Embryonal tumor with multilayered rosettes, C19MC-altered
  • Embryonal tumor with multilayered rosettes, NOS
  • Medulloepithelioma
  • CNS Neuroblastoma
  • CNS ganglioneuroblastoma
  • CNS embryonal tumor, NOS
  • Atypical teratoid/ rhabdoid tumor
  • CNS embryonal tumor with rhabdoid features

    Tumors of cranial and paraspinal nerves
    Schwannoma
              Cellular Schwannoma
              Plexiform Schwannoma
              Melanotic Schwannoma
    Neurfibroma
              Atypical neurofibroma
              Plexiform neurofibroma
    Perineurioma
    Hybrid nerve sheath tumors
    Malignat peripheral nerve sheath tumor
                Epitheloid MPNST
                MPNST with perineurial differentiation

    Meningioma

  • Meningioma
  • Meningothelial meningioma
  • Fibrous meningioma
  • Transitional meningioma
  • Psammomatous meningioma
  • Angiomatous meningioma
  • Microcystic meningioma
  • Secretory meningioma
  • Lymphoplasmacyte-rich meningioma
  • Metaplastic meningioma
  • Chordoid meningioma
  • Clear cell meningioma
  • Atypical meningioma
  • Papillary meningioma
  • Rhabdoid meningioma
  • Anaplastic ( malignant) meningioma 

    Mesenchymal, Non-meningothelial tumors

  • Solitary fibrous tumor/hemangiopericytoma
  • Hemangioblastoma
  • Hemangioma
  • Epitheloid hemangioendothelioma
  • Angiosarcoma,
  • Kaposi Sarcoma,
  • Ewing sarcoma/ PNET
  • Lipoma
  • Angiolipoma
  • Hibernoma
  • Liposarcoma
  • Desmoid type fibromatosis
  • Myofibriblastoma

  •  Inflammatory myofibroblastic tumor
  • Benign fibrous histiocytoma
  • Leiomyoma
  • Leiomyosarcoma
  • Rhabdomyoma
  • Rhabdomyosarcoma
  • Chondroma’
  • Chondrosarcoma
  • Osteoma
  • Osteochondroam
  • Osteosarcoma
    Melanocytic tumors

  • Meningeal melaonocytosis
  • Meningeal melanocytoma
  • Meningeal melanoma
  • Meningeal melanomatosis
    Lymphoma

  • Diffuse large B-cell lymphoma of the CNS 
  • Immunodeficiency-associated CNS lymphoma
                              AIDS-related diffuse large B-cell lymphoma
                              EBV-positive diffuse large B-cell lymphoma, NOS
                              Lymphomatoid granulomatosis 

  • Intravascular large B-cell lymphoma 
  • Low-grade B-cell lymphoma of the CNS 
  • T-cell & NK/T cell-lymphoma of the CNS 
  • Anaplastic large cell lymphoma , ALK-positive 
  • Anaplastic large cell lymphoma , ALK-negative 
  • MALT lymphoma of the dura
    Histiocytic tumors

  • Langerhans cell histocytosis
  • Erdheim-Chester disease
  • Rosai-Dorfman disease
  • Juvenile xanthogranuloma
  • Histiocytic sarcoma
    Germ cell tumors

  • Germinoma
  • Embryonal carcinoma
  • Yolk sac tumor
  • Choriocarcinoma
  • Teratoma
                     Mature, Immature

  • Teratoma with malignant transformation
  • Mixed germ cell tumor
    Tumors of the sellar region
    Craniopharyngioma
                 Admantinomatous craniopharyngioma
                 Papillary craniopharyngioma
    Granular cell tumor of sellar region
    Pituicytoma
    Spindle cell oncocytoma
    Metastatic tumors


Neurosurgery is the cornerstone of the management of tumors of the brain and spinal cord. Because glioma annd metastasis constitute majority of tumors, very judicious approach is required for the diagnosis and treatment of such lesion. Many options are available but they may be costly, unnecessary and harmful. A very small low grade glioma be an incidental finding and surgery may not be required. A small neoplastic lesion of less than 3 cm may be treated with gamma knife which is a non surgical treatment and utilizes precise and focussed single dose radiation over a deep seated lesion in the eloquent area of the brain.

About 4 decades ago, whole brain radiotherapy and steroids were the main treatment modality for treating brain metastasis. But now few brain metastatic lesions may be excised safely and stereotactic radiotherapy can be given postoperatively, reducing the morbidity in a patient.

Advances in the skull base approaches have made the neurosurgery relatively safer, and few tumors can be excised completely. This may be true in cases of intracranial meningiomas.





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