Skull base tumors

Dr Nandeesh BN
Department of Neuropathology
NIMHANS
 Introduction

Skull base

▪ Central & complex bone structure of the skull that forms the floor of
the cranial cavity
▪ Anatomical interface
▪ Boundary & bridging function of various tissues of
different histogenetic origin
▪ anterior, middle and posterior compartments
 Introduction
Skull base neoplasms
❑Complex & diversity of tissue types
❑Numerous benign & malignant neoplasms of distinctive differentiation, morphology & behavior
• arise from skull base structures or
• extend into the skull base region from intra or extra cranial lesions

• Diagnostic & Therapeutic Challenge

• Deep location
• Complex anatomy (neurovascular foramina, adjacent structures)
• Close proximity to eloquent structures (brain, orbit, CN’s, vessels)
• Diverse pathology (benign/malignant tumors, infectious, congenital)
• Osseous skull base and pachymeninges (dura mater) - effective barriers, but tumor can spread through skull
base foramina
 Classification
❖I: Benign / Malignant
❖Primary / secondary
❖II: anterior, middle, and the posterior cranial fossae
❖III: Tissue type:
▪Ectopically displaced cells/tissues
❖IV ▪Retained embryonal structures
❖Extra-cranial vs. Intra-cranial
❖Intrinsic / Extrinsic
 adjacent intracranial location / extracranial location
 Direct extension
 Perineural spread

❖Combined
 Classification of Histologic Types of Cranial Base Tumors

Benign Tumors
Meningiomas Intermediate Malignant Tumors /
Low Grade
Schwannomas
Chordomas Highly Malignant Tumors
Pituitary adenomas
Chondrosarcomas Carcinomas (adenoca, Sq cell
Paragangliomas
Adenoid cystic carcinomas Ca, transitional,
Hemangiomas in cavernous undifferentiated)
sinus Low-grade
esthesioneuroblastomas Sarcomas: RMS, EWS, FS,OS,
Juvenile angiofibromas MPNST
Desmoid,,
Fibrous dysplasia Higher-grade
low-grade sarcomas - FS,
Dermoid / Epidermoid cysts esthesioneuroblastomas
SFT
Cholesterol granulomas Lymphomas
Osteoma Myelomas
Metastasis
 Evaluation
• Clinical & Radiologic evaluation
• Systematic approach to narrow the differential diagnosis.
• Anatomic location
• Behaviour : Benign or malignant ?
• Cellularity
• Architecture / pattern
• Cell details – cytoplasm, nuclear, mitosis
• Stroma / matrix
• Differentiation / lineage
• Additional components
• Extension (direct vs. indirect)
 Anterior Cranial Fossa tumors
• Imp tumor examples: –Sinonasal
• Meningioma –Olfactory
• Esthesioneuroblastoma –Orbits
–Meninges
• Sino-nasal (SN) malignancies
–Subfrontal brain
• Giant cell tumor (GCT)
• Hemangiopericytoma
• Multiple myeloma (MM)/plasmacytoma Sinonasal malignancy
• Sarcomas (Osteo. and Rhabdo.) Epithelial: Inverted papilloma,
nasal polyposis,
• Lymphoma nasopharyngeal carcinoma
• Melanoma Nonepithelial: Olfactory
neuroblastoma, juvenile
nasopharyngeal angiofibroma

• MC malignant tumor - Cancers of the paranasal sinuses or nasal

cavity
 Orbital tumors

Primary Orbital Tumors Secondary Orbital Tumors

Primary Malignant ▪Paranasal sinuses
▪ Benign –
▪ Vascular – ▪Lacrimal gland ▪Intracranial
▪ Cavernous hemangiomas ▪ACC, malignant ▪Meningiomas
▪ Nerve sheath ▪Sarcoma ▪Dermal
▪ Bony ▪ Osteosarcoma
▪ Lymphangioma ▪ Rhabdomyosarcoma
▪Metastatic Orbital Tumors
 Skull base
meningioma

A: olfactory groove,
B: para- , suprasellar,
C: petro-clival,
D: sphenoid wing,
E:Foramen magnum.
Meningioma
• Origin - arachnoid cells.
• MC non-glial primary brain tumor - 20% of all intracranial neoplasms
• Peak – 6th – 7th decade; F>M
• a/w neurofibromatosis (multiple tumors)

• Grade I - 75 - 90%
• Grade II - Atypical - 10 - 20%
• Grade III - Anaplastic - 1–3%
• MC - skull vault
• Parasellar region (tuberculum sella, cavernous sinus, planum sphenoidale, diaphragma sellae, clinoid process) - 15–30% of
meningiomas.
• MC after pituitary adenomas; pure sellar – rare (mimic NFPAs)
 Mesenchymal, non-meningothelial tumors

•Lipomatous tumors •Osteoma

•Solitary fibrous tumour •Osteosarcoma

•Osteochondroma
•Fibrosarcoma

•Vascular tumors
•Haemangioma
•Histiocytic tumors
•Epithelioid Haemangio-
•Leiomyoma endothelioma
•Leiomyosarcoma •Haemangiopericytoma – Gr.II/III

•Rhabdomyoma •Angiosarcoma

•Rhabdomyosarcoma
•Chondroma
•Chondrosarcoma
 Paranasal Sinus Lesions

▪ Benign Tumors
▪ Ossifying fibromas
▪ Malignant Tumors
▪ Carcinomas
▪ Esthesioneuroblastoma
▪ Lymphoma
▪ Nasoparyngeal carcinoma
▪ Orbital glioma
▪ Osteogenic sarcomas
▪ Rhabdomyosarcoma
▪ Melanomas
 Neoplasms of the Central Skull Base
• Intrinsic
• Chordoma
• Chondrosarcoma
• Metastasis
• Lymphoma/Leukemia/Myeloma
• Extend from adjacent intracranial lesions
• Meningioma
• Nerve sheath tumor
• Invasive pituitary adenoma
• Extend from extracranial soft tissues
• Direct (JNA, NPC)
• Perineural (SCC)
• Central region: • Para-central/Cavernous Sinus region:
• Pituitary adenoma, • Meningioma,
• meningioma, • schwannoma,
• pseudotumor, • adenoid cystic carcinoma (ACC),
• craniopharyngioma, • NP carcinoma,
• sphenoid sinus carcinoma • GCT, pseudotumor

• Clival region: • Petro-Clival/Lateral region:

• Chordoma, • Meningioma,
• meningioma, • schwannoma,
• paraganglioma, • NP angiofibroma,
• naso-pharyngeal (NP) carcinoma, • ACC,
• schwannoma, • sarcoma,
• chondrosarcoma, • acquired/congenital cholesteatoma,
• MM/plasmacytoma, cholesterol granuloma,
• pseudotumor • pseudotumor
 PITUITARY ADENOMAS
• MC tumor of adenohypophysis
• 30 - 60 years
• F>M
• Endocrine symptoms - hormonal overproduction
• Compression or invasion of adjacent structures: headaches, bitemporal hemianopsia, hypothalamic
dysfunction, cavernous sinus syndrome, hydrocephalus & pituitary hypofunction.
• Pituitary apoplexy - hemorrhagic infarction [1% to 2%]
• 10% to 15% of all intracranial tumors
• 25 % ;  age; M=F

Classification:
• Size:
• Micro / Macro : cut off 10 mm
• Micro >>> Macro
• Functional: functioning & non-functioning
• Histological: Histochemical reaction - acidophilic, basophilic and chromophobic
• Obsolete
• Immunohistochemical: hormone content + addl (transcription factors and keratins).
• Clinicopathological: morphological (Immunophenotype) + clinical features.
• Most effective classification scheme
 Classification / DDs of sellar and parasellar lesions
❑ Neoplastic • Neoplastic
❑ Malformative lesions
❑
• Adeno-hypophyseal origin
Inflammatory
• Pituitary adenoma • Schwannoma / Neurinoma
❑ Vascular lesions • Metastases
• Pituitary carcinoma
❑ Miscellaneous • Lymphoma
• Embryonal / PNET
• Neurohypophyseal origin • Chordoma
• Pituitocytoma • Chondrosarcoma
• Granular cell tumor • Chondroma
• Spindle cell oncocytoma • Langerhans’ cell
(SCO) histiocytosis
• Solitary fibrous tumor
• Plasmacytoma
• Parasellar tumors • Paraganglioma
• Meningioma, • Lipoma
• Craniopharyngioma • Gangliocytoma
• Glioma
• Germ Cell tumor
 Craniopharyngioma
• 2–5% of all primary intracranial neoplasms
• Bimodal age distribution:
• children (5–14 years) & older adults (50–74 years)
• 2/3rd < 20 years.
• Origin - squamous cell rests in the remnant of Rathke’s pouch between the adeno-hypophysis and neuro-hypophysis
• a benign histological appearance but - infiltrative tendency into critical parasellar structures
• Solid-Cystic: Predominantly cystic in 46–64%, Predominantly solid in 18–39% and mixed in 8–36%.
• Calcifications - 45–57% (in 78–100% of children)
• (1) Adamantinomatous (2) Papillary
Craniopharyngioma

• Adamantinomatous
– Palisading, “wet” keratin,
calcification, “machinery oil” fluid
– Children & adults

• Papillary
– Well diff squamous epithelium with
no keratin, palisading or
calcification
– Adults
Craniopharyngioma
– Better prognosis
 Chordoma
• Arises from notochordal remnants
• Locally aggressive
• Proximity to vital structures limits resection

• Location
• 35% skull base
• Dorsum sella, clivus & nasopharynx
• 50% sacrococcygeal
• 15% vertebral body
Chordoma
• Pain , cranial nerve involvement, pituitary dysfunction,
Mass in nose or nasopharynx

• Types:
• Conventional - MC
• Chondroid (5-15%)
• Dedifferentiation or sarcomatous transformation (2-8%)
• Poorly differentiated

❖IHC: CK, EMA, CEA

 Posterior Cranial
Fossa

•Cerebellopontine (CP) angle: •Foramen magnum:

• Schwannoma, • Meningioma,
• meningioma, • schwannoma,
• epidermoid, • chordoma,
• arachnoid cyst, • intra-medullary cord tumor
• cholesterol granuloma,
• endo-lymphatic sac tumor,
• metastasis,

•Jugular foramen:
• Paraganglioma,
• schwannoma,
• meningioma,
http://www.mayfieldclinic.com
• Metastasis
Glomus jugulare tumor
• Paraganglioma – Jugulotympanic
• Pulsating tinnitus

• Chromogranin & synaptophysin (chief cells), S100 (sustentacular cells)

• Reticulin - (stains stroma & delineates nesting pattern,
 Endolymphatic Sac Tumor
• rare non metastasizing adenocarcinoma
• Origin - endolymphatic sac (neuroectodermal origin, lines the Vestibular canal)
• Adults
• Associated with von Hippel –Lindau disease
• Synonyms: Low grade adenocarcinoma of Endolymphatic sac origin, Aggressive middle ear
tumour, Heffner tumor

Microscopic Diagnostic features

• Papillary – glandular (variable)
• Single row of cuboidal cells
• Papillae – vascular
• Thyroid like areas
 CP angle
1. Vestibular Schwannoma (acoustic neuroma). MC

2. Meningioma

3. Epidermoid cyst

4. Trigeminal neuroma

5. Metastases

6. Skull base/temporal bone tumours:eg, glomus

tumors,metastases,cholesterol granuloma

7. Misc – Lipoma, Hemangioma

 Schwannomas of the Cranial Nerve Roots

• 8% of intracranial tumors.
• MC - acoustic nerve

Acoustic schwannoma:
• 40 – 60 yrs
• F>M
• Cerebellopontine angle
• Tinnitus, hearing impairment, dizziness & unsteady gait.
• As tumor grows - cerebellar symptoms, facial, trigeminal & pyramidal signs develop, +
symptoms & signs of raised ICP.
 Skull base Intra – osseous
Malignant Tumors

• Chondrosarcoma
• Chordoma
• Endolymphatic Sac Tumor
• Metastasis
• Plasmacytoma
• Lymphoma
• Nasopharyngeal Carcinoma
• Rhabdomyosarcoma
• Langerhans Cell Histiocytosis
Location
Chondrosarcoma
• petro-occipital synchondrosis (MC)
• sphenoethmoidal junction
• 6.5% involve H+N, 75% of these - skull
• sella turcica base
• Petrous portion of the temporal bone • Matrix Ca2+ in 50%

• Grade I to III
• Conventional, Mesenchymal, clear cell & dedifferentiated.
• No clear cell or dedifferentiated subtypes - intracranially
Summary
• Complex anatomy & diverse pathology

• Systematic approach

• Consider :
• Anatomic localization & extent of disease
• Biologic behavior (benign vs. aggressive)
• Involvement of adjacent eloquent structures
• Thank you