Oral Tumors,In Oral Cavity

33 

Full text

(1)

Benign and malignant

epithelial tumors

Localization in oral cavity

Benign and malignant

mesenchymal tumors

Localization in oral cavity

Other types tumors (pigment,

nervous system, odontogenic)

Synpsis

 Benign tumors of mesenchymal origin.

 Malignant tumors of mesenchymal origin.

 Benign tumors of epithelial origin – adenoma, papilloma.  Malignant tumors of epithelial origin – carcinoma. Localization in

oral cavity.

 Tumors of melanocyte origin.

 Tumors of the central nervous system.

 Tumors of the peripheral nervous system.

 Benign tumors of connective tissue.

 Benign and malignant tumors of smooth and striated muscles.

 Tumors from vessels’ wall – benign and malignant.

 Malignant tumors of connective tissue origin.

 Osteogenic sarcoma. Malignant synovioma.

(2)

CLASSIFICATION OF

TUMOURS

Behavioural classification

benign or malignant

Histogenetic classification -cell of origin

Epithelial

Mesenchymal

Mixed

Teratomas

Precise classification of individual tumors is

important for planning treatment

Principal characteristics of benign and

malignant tumours

Special features of the

epithelial tumors in

different organs

Epithelial tumors

The most often tumors

 ectoderm -skin

 mesoderm-kidney

 endoderm - GIT

Structure

 the parenchyma

 neoplastic epithelial cells

Benign malignant

(3)

Epithelial tumors

Benign

Papilloma

from the surface epithelium

Adenoma

from the glandular epithelium 

Malignant

carcinomas

 Skin

 Epithelial lining of glands

and ducts  Gastro-intestinal tract

 Oral cavity, esophagus,

stomach, intestine, hepato-biliary system, pancreas  Respiratory passages

 Nasal cavity, larynx,

trachea, bronchi  Urinary tract epithelium  Male and female genital

systems

 Placental epithelium

 Endocrine glands

Tumors of epithelial origin

Tissue of origin Benign Malignant

Special features of epithelial

tumors

Benign and malignant epithelial tumors are the most

common in adults.

Epithelial cells grow as cohesive groups

Malignancy can be diagnosed by invasion through tissue

layers -basement membrane, muscularis mucosae

 intact basement membrane in benign tumors

Carcinomas spread generally

 by lymphatics to lymph nodes,

 later -via the blood stream (liver, pulmo, bones)

Treatment is by surgical resection

 In carcinomas response to radiation and chemotherapy varies with type

Benign epithelial tumors

2 types according to the epithelium

Papilloma – from the surface epithelium

Skin, urinary tract epithelium

(4)

Papillomas

Tumors with finger-like

projections

Macroscopic features

 Exophytic lesions  Rarely endophytic lesions

Histology

 Papillae

 Epithelium lining

 Squamous cell epithelium  Transitional epithelium

 Preserved basement membrane  Connective tissue core

Squamous cell papilloma

Verrucae

 skin  viruses

Squamous cell papilloma

 esophagus

 larynx

children –juvenile papillomatosis trachea

 precancerosis

Condylomata

 genitals, anus

condyloma acuminatum

HPV (human papilloma virus -1,

2, 4, 7 type)

condyloma lata

syphilis

Urothelial papillomas

Transitional epithelium of

ureter, bladder, uretra

Precancerosis

Urothelial carcinoma with

low grade malignancy

Histology

Fragile papillae

Urine -cytology

(5)

Adenomas

 Glandular epithelium

 Endocrine glands

  functional activity – clinical syndromes  Pituitary

 Thyroid gland  Suprarenal glands  Endocrine pancreas

 Exocrine glands

 Skin - oil and sweat glands  Salivary gland  Adenoma gl. Parotis  Breast  Fibroadenoma  Exocrine pancreas  Gastrointestinal tracts  Respiratory tract  Ovarium  Liver  Kidney

Adenomas

Macroscopy

nodules, capsulated

Mucosa surface –

pedunculated and sessile

polyps

Single, multiple

 familial polyposis coli

Various size

 < 1 cm  > 3 cm

Adenomas

Histology

Glands

Various shape and size Preserved basement

membrane

± dysplasia

 low-grade or high-grade

high grade often

classified with carcinoma-in-situ  may develop into malignancy

Uterine cervix Colon polyps

Dysplasia

Normal gland

Mild dysplasia Severe dysplasia

(6)

Adenomas

Histological types

 acinar

 Small glands  lumen, endocrine

glands  trabecular

 Tarbeculae, liver, suprarenal glands

 tubular  tubule, GIT  Villous  Papillae, GIT  Mixed  tubulovilous, GIT  Solid  Nests, bronchi  Cystic

 Papillary cystadenoma – serous,

mucous, ovary

Adenoma рleomorphe glаndulae

parotis

Tumor mixtus

Capsulated,

mucinous cut surface

Histology

 Gland structures  Myoepithelial cells Mucoid substance Basophilic Resemble cartilagous

Fibroadenoma gl. mammae

Female, young age

Capsulated, firm

nodules

Histology

 parenchyma

Gland structures Loose connective tissue

 pericanalicular  intracanalicular

 Fibrous stroma

(7)

Malignant epithelial tumors

= Carcinomas

 Risk factors

 Preneoplastic syndromes –chronic inflammation, hyperplasia, regeneration

 Benign epithelial tumors

 Macroscopy

 Rapid growth – necrosis, haemorrages  Noncapsulated

 Infiltrative

 Exophytic and endophytic growth

 Histology

 invasion through tissue layers -basement

membrane, muscularis mucosae

 differentiation

 Grading - high-, moderate-, poor differentiation

 Metastases  Lymph nodes  Distant metastases  Seeding  TNM staging

Carcinomas

High morbidity and

mortality

may be due to:

pressure on and destruction

of adjacent tissue

metastases

blood loss from ulcerated

surfaces

obstruction of flow

(intestinal obstruction)

paraneoplastic effects weight loss, cachexia

Carcinomas

From surface epithelium

 Squamous cell carcinoma  Basal cell carcinoma  Transitional (urothelial)

carcinoma

From glandular epithelium

 Adenocarcinoma

 Hepatocellular carcinoma  Renal cell carcinoma

 Seminoma

 Choricarcinoma

 Poorly differentiated carcinoma

Malignant tumors of surface

epithelium

Squamous cell carcinoma

 skin  Face  Oral cavity  leukoplakia Metaplasia of stratified squamous non-ketatinized epithelium into keratinized

 Esophagus

 Larynx

 Bronchus

 Squamous cell metaplasia

(8)

Squamous cell carcinoma

Histology

atypical cells at all

levels of the epidermis,

with nuclear crowding

and disorganization

invasions of basement

membrane

variable differentiation

Keratinization  perls

Squamous cell carcinoma

High differentiation

With keratinization

= carcinoma planocellulare keratodes

Moderate differentiation

keratinization

Low differentiation

Without keratinization

= carcinoma planocellulare non-keratodes

WELL?

MODERATE?

POOR?

Grading for Squamous Cell Carcinoma

Basal cell carcinoma

=Ulcus rodens

 Tumor cells resemble the normal epidermal basal cell layer from

which they are derived

 2 patterns: superficial type or nodular lesions

 palisading with separation from the stroma, creating a cleft

(9)

Malignant tumors of glandular

epithelium

= Adenocarcinoma

 Breast  Salivary glands  Gastro-intestinal tract

esopagus – Barrett esophagus stomach – H.pylori gastritis colon– adenomas

 Pancreas

 Endocrine glands  Female genital system

endometrium ovarium

 Male denital system

testis - seminoma

Adenocarcinomas

Histology

Gland structures

Various shape cellular atypia invasion through tissue

layers basement membranes muscularis mucosae

Adenocarcinomas

Histological types

 Mucinous – Mucin production Intra-, extracellular "signet-ring" cells  Papillary carcinoma  Cystadenocarcinoma  Adenoacantoma

+ squamous cell metaplasia

 Adenosquamous carcinoma

+ squamous cell carcinoma

 Mucoepidermoid carcinoma

Mucinous adenosquamous

carcinoma

Adenocarcinoma ventriculi

(10)

Adenocarcinoma uteri

Seminoma Testis

Choriocarcinoma

Хепатоцелуларен карцином

Hepatocellular carcinoma

(11)

Renal cell carcinoma

3 types:

Clear Cell Carcinoma

the most often

Papillary Renal Cell

Carcinoma

Chromophobe Renal

Carcinomas

Poorly differentiated carcinomas

Undifferentiated

carcinomas

2 types

Scirousum

 stroma – firm breast 

medullare

 stroma - soft

Special features of the

mesenchymal tumors in

different organs.

Mesenchymal tumors

Soft tissue tumors

Connective/fibrous tissue

Adipose tissue

Muscle tissue

Vascular tissue

Bone tumors

Joint tumors

A broad group of non-epithelial tumors, deriving from

(12)

SOFT TISSUE TUMORS

Tumors of Adipose Tissue

 Lipomas  Liposarcoma

Tumors and Tumor-like Lesions of

Fibrous Tissue  Fibroma  Nodular fasciitis  Fibromatoses  Fibrosarcoma  Fibrohistiocytic Tumors  Fibrous histiocytoma  Malignant fibrous histiocytoma

Tumors of Skeletal Muscle

 Rhabdomyoma  Rhabdomyosarcoma

Tumors of Smooth Muscle

 Leiomyoma

 Smooth muscle tumors of uncertain

malignant potential  Leiomyosarcoma  Vascular Tumors  Hemangioma  Lymphangioma  Hemangioendothelioma  Hemangiopericytoma  Angiosarcoma  Tumors of Uncertain Histogenesis  Synovial sarcoma  Alveolar soft part sarcoma  Epithelioid sarcoma  Granular cell tumor

Mesenchymal tumors

Benign

by adding “-oma” to cell type, from which tumor

arise

 fibroma, lipoma, chondroma 

Malignant

Sarcomas

 Fibrosarcoma, liposarcoma, chondrosarcoma

Special features of the mesenchymal

tumors

 A great diversity of tumors

 Compared to the epithelial tumors

 Appear at any age

 children  adults

 Risk factors

 Physical –trauma, radiation, thermal burn associations  Genetic

 Chromosome translocations  Part of many syndromes

 No clear distiction between the tumor’s parenchyma and stroma

 Mesenchymal origin of the components  Diffuse growth

 Sarcomas spread generally by via the blood stream to the pulmo, liver  Diagnosis

 Difficult differentiation between some benign and malignant variants

 Tumors of borderline malignancy

 mitosis

 Similar histology – spindle cell type

 Immunohistochemistry –histogenetic markers

 Need of consultation

Chromosomal and Genetic Abnormalities in Soft Tissue Sarcomas

Tumor Cytogenetic Abnormality Genetic Abnormality

Extraosseous Ewing sarcoma and

primitive neuroectodermal tumor t(11:22)(q24;q12) FLI-1-EWS fusion gene t(21:22)(q22;q12) ERG-EWS fusion gene t(7;22)(q22;q12) ETV1-EWS fusion gene Liposarcoma—myxoid and round cell

type t(12:16)(q13;p11) CHOP/TLS fusion gene Synovial sarcoma t(x;18)(p11;q11) SYT-SSX fusion gene Rhabdomyosarcoma—alveolar type t(2;13)(q35;q14) PAX3-FKHR fusion gene t(1;13)(p36;q14) PAX7-FKHR fusion gene Extraskeletal myxoid chondrosarcoma t(9;22)(q22;q12) CHN-EWS fusion gene Desmoplastic small round cell tumor t(11;22)(p13;q12) EWS-WT1 fusion gene Clear cell sarcoma t(12;22)(q13;q12) EWS-ATF1 fusion gene Dermatofibrosarcoma protuberans t(17:22)(q22;q15) COLA1-PDGFB fusion

gene

Alveolar soft part sarcoma t(X;17)(p11.2;q25) TFE3-ASPL fusion gene Congenital fibrosarcoma t(12;15)(p13;q23) ETV6-NTRK3 fusion gene

(13)

SOFT TISSUE TUMORS

Fat (adipose) tissue

Fibrous tissue

Fibrohistiocytic

Skeletal muscle

Smooth muscle

Vascular

Peripheral nerve

Uncertain

 synovial sarcoma, alveolar soft part sarcoma, epitheliod sarcoma

Tumors of Adipose Tissue

Benign

Lipomas

Malignant

Liposarcoma

Lipoma

 Benign tumors of fat

 The most common soft tissue tumors of

adulthood.

 Solitary lesions

 Multiple – in rare autosomal dominant

syndromes.  Localization

 Back, shoulders, thigh  Submucosa of GIT

 Macroscopy

 soft, yellow, well-encapsulated masses

 Histology

 Conventional - mature adipocytes

 Varied in size  Clear empty cytoplasm  Peripheral nucleus

 Angiolipoma

 Numerous capillaries

 Treatment

 complete excision is usually curative

(14)

Liposarcoma

 Malignant neoplasm of adipocytes  Rare tumors,

 Adults, 60-70 y - f > m

 chromosomal translocation - myxoid liposarcomas

 Localization

 deep soft tissues, retroperitoneum  in visceral sites

 Macroscopy

 relatively well-circumscribed lesions , large size  polylobulated

 ± myxoid cut surface

 Histology

 Lipoblasts

 fetal fat cells with cytoplasmic lipid vacuoles

 myxoid liposarcoma

 Mucoid stroma

 Pleomorphic variant

 Atypical cells, inc. multinuclated cells  mitoses

Liposarcoma

Tumors and Tumor-like Lesions

of Fibrous Tissue

Benign

Fibroma

Malignant

Fibrosarcoma

Fibroma

Benign tumor of fibrous

connective tissue

A common tumor of the skin

Macroscopy

 Capsulated nodule

Histology

 Fibrocytes

Spindle cells with sharp edges

nuclei

storiform pattern of growth

 + collagen fibers

hard fibroma -fibroma durum Soft fibroma -fibroma molle

(15)

Fibroma cutis

Fibrosarcomas

 Malignant neoplasms composed of

fibroblasts  tend to grow slowly

 often recur locally after excision (>50% of

cases)

 can metastasize hematogenously (>25% of

cases) - lungs  Adults  Localization

 deep tissues of the thigh, knee, and

retroperitoneal area  Macroscopy

 soft unencapsulated, infiltrative masses  ± areas of hemorrhage and necrosis

 Histology

 all degrees of differentiation  spindled cells growing in a herring bone

fashion  pleomorphism,  mitoses  necrosis

Fbrosarcoma

Tumors of Smooth Muscle

Leiomyoma

Smooth muscle tumors of uncertain

malignant potential

(atypical leiomyomas)

DD leiomyoma/leiomyosarcoma

Leiomyosarcoma - > 10/10 HPF mitoses

(16)

Leiomyoma

 Benign tumor of smooth muscle cells

 Low malignant potential

 Localization

 Myometrium – multiple nodules

Submucosa, intramural, subserosal

 Age – female

 estrogen-dependent

 Macroscopy

 Well circumscribed, grey-white firm nodules, up to 20 cm

 Fascicled cut surface

 Histology

 Spindle cells (smooth muscle), fascicles

Round edges of the nuclei

 ± fibrosis, calcifications, necrosis, cysts

Leiomyoma (HE)

Leiomyosarcoma

 Malignant tumor of smooth muscle

cells

 10% - 20% of sarcomas

 Adult, f> m  Localization

 skin,

 Deep tissues of extremities  Retroperitoneum, uterus

Local invasion, metastasis метастази

 Macroscopy

 Large, firm tumor mass

 Histology

 spindle cells with cigar-shaped nuclei

 arranged in interweaving fascicles

(17)

Tumors of Skeletal Muscle

Rhabdomyoma

Rhabdomyosarcoma

More common

Rhabdomyoma

 Benign tumor of striated musle

cells  Very rare  malformation  Age  Adult type  Fetal type  Localization  Skeletal muscles  Heart Sclerosis tuberans  Histology

 Large round cells with eozinophilic granular cytoplasm

Rhabdomyosarcoma

Malignant tumor of striated muscle cells

 > 50% of sarcomas in children

 Localization

 head, neck, face  extremties  genitourinary tract

sarcoma botryoides -soft, gelatinous, grapelike masses

 Macroscopy

 poorly defined, infiltrating masses

 Histology

 Rhabdomyoblast - round or elongated cells  granular eosinophilic cytoplasm, filaments

Embryonal variant

Small round cells

Alveolar variant

Alveoli, fibrous septa

Pleomorphic variant

Immunohistochemistry

 Desmin, myoglobin, actin, myosin

Vascular tumors

Tumors of blood vessels and lymphatics

Benign

 hamartomas, not even true

neoplasms

Intermediate

 Locally aggressive, rarely metastasize

Malignant

 Frequent and early metastases– lungs

Diagnosis

 “endothelium” lined blood filled spaces

immunohistochemistry - factor VIII

 mitosis

Hemangioma

Lymphangioma

Hemangioendothelioma

(18)

Vascular tumors

Benign

Rare mitosis

Mild, rare atypia

No metastases

Malignant

Common mitosis

Frequent, severe

atypia

Early, frequent

metastases

via bloodstream

Hemangioma

= a generic term for any benign blood vessel

tumor

Capillary (small vascular spaces)

Also called “juvenile”, often called “birth marks”

Usually regress with age

Cavernous (large vascular spaces)

Also called “adult”

Usually do not regress

Smooth-muscle hemangioma

Pyogenic hemangioma

Hemangioma

(19)

Pyogenic granuloma

Oral cavity most common

Regress

Histology

 like capillary hemangioma  Indistinguishable from normal

granulation tissue

Glomus tumor

Most commonly

under nail

Small tumor, 1 cm

Painful

Lymphangiomas

 Benign lymphatic analogue of

hemangiomas.

 Generally rare

 Simple (Capillary) Lymphangioma

 small lymphatic channels

 absence of blood cells

 head, neck, axillary subcutaneous tissues  flat lesions - 1 to 2 cm

 Cavernous Lymphangioma (Cystic Hygroma)

 typically found in the neck or axilla of

children

 common in Turner syndrome

 can be enormous (≤15 cm in diameter), not

encapsulated

 Producing gross deformities - neck

 composed of dilated lymphatic spaces and

connective tissue stroma, Ly aggregates

Malignant vascular tumors

Angiosarcoma

± vessels lumens

 Factor VIII

atypical endothelial cells

 Cellular pleomorphism  Mitoses, atypical 

Lymphangiosarcoma

After mastectomy

Papillary projections of

atypical endothelial cells

(20)

Tumors of Uncertain

Histogenesis

Synovial sarcoma

Alveolar soft part sarcoma

Epithelioid sarcoma

Synovial sarcoma

 Uncertain cellular origin, agressive,

malignant

 The cells are not synoviocytes  metastases –lung, bones, LN

 Localization

 Joints - 10%, knee  extrajoints

 Young adults

 Chromosomal translocations t(X;18)

 SYT gene (transcription factor)

 Histology

 Biphasic variant

 Epithelial-like cells, glands  Spindle cells

 Monophasic variant

 Spindle cells – fascicles

 DD fibrosarcoma – keratin, EMA

Bone tumors

Bone

Cartilage

Fibrous

Other

Ewing’s “sarcoma”

Giant cell tumor

Metastases

Benign

Malignant

Classification of Primary Tumors Involving Bones

Histologic Type Benign Malignant

Hematopoietic (40%) Myeloma

Malignant lymphoma

Chondrogenic (22%) Osteochondroma Chondrosarcoma

Chondroma Dedifferentiated chondrosarcoma

Chondroblastoma Mesenchymal chondrosarcoma

Chondromyxoid fibroma

Osteogenic (19%) Osteoid osteoma Osteosarcoma Osteoblastoma

Unknown origin (10%) Giant cell tumor Ewing’s sarcoma Giant cell tumor Adamantinoma

Histiocytic origin Fibrous histiocytoma Malignant fibrous histiocytoma

Fibrogenic Metaphyseal fibrous defect

(fibroma) Desmoplastic fibroma

Fibrosarcoma

Notochordal Chordoma

Vascular Hemangioma Hemangioendothelioma

Hemangiopericytoma

Lipogenic Lipoma Liposarcoma

(21)

Benign

o

steogenic bone tumors

= Bone Forming, 19%

Osteoma

face, skull; 40-50yrs

Histology - similar to normal bone

Osteoid Osteoma

metaphysis femur, tibia 10-20yrs

Histology – similar to woven bone

Osteoblastoma

vertebral column 10-20yrs

Histology -similar to osteoid osteoma

Osteoma

Solitary tumor

Mean age

Face, skull

Exophytic growth,

attached to the bone

Histology

 similar to normal bone

Frontal sinus

Malignant

osteogenic bone tumors

Osteosarcoma (osteogenic sarcoma)

 Primary

Metaphysis of distal femur, proximal; 10-20 yrs

 secondary - associated with pre-existing disorders such

as benign tumors, Paget disease Femur, humerus, pelvis, > 40 yrs

 Histologic variants

 osteoblastic, chondroblastic, fibroblastic, telangiectatic,

small cell, and giant cell

 Different degree of differentiation

The most common subtype is osteosarcoma that arises in the metaphysis of long bones; solitary, intramedullary, and poorly differentiated; produces a predominantly bony matrix

(22)

Sarcoma osteogenes

Benign cartilagenous bone tumors

= Cartilage forming, 22%

Osteochondroma (exostosis)

 Metaphysis of long bones; 10-30 yrs;  Histology - cartilage and bone tissue

Chondroma

 Small bones of hands and feet; 30-50 yrs; medullary cavity  Histology – hyaline cartilage

Chondroblastoma

 Knees, epiphyses, teenagers, m>>f,  Histology - much less matrix than a chondroma

Chondromyxoid fibroma

Myxoid, atypia

Osteochondroma (exostosis)

Common

Often multiple as a

hereditary syndrome

m>>>f

Pelvis, scapulae, ribs

Metaphysis

Cartilage and bone

present

Chondroma

Hyaline cartilage

Multiple enchondromas

 Ollier’s disease

(23)

Chondroma

cartilagenous bone tumors

Chondrosarcoma

Femur, humerus, pelvis

within medullary cavity

40-60 yrs

Histology – atypical chondroblasts –

abnormal cartilage

conventional hyaline/myxoid Mesenchymal Clear cell dedifferentiated

Chondrosarcoma

Variants -conventional hyaline/myxoid; mesenchymal; clear

Other Bone Tumors

Giant cell tumor

Ewing sarcoma

Metastases

the most common bone tumors

osteoblastic/ lytic

male: prostate female: breast

(24)

Giant cell tumor of bone

Localization

 epiphysis of long bones

 cortical lesions

Young adults -20-40 yrs

Histology

 Macrophages

 Giant cells

Ewing sarcoma (tumor)

PNET (primitive

neuroectodermal tumor)

Localization

 Diaphysis and metaphysis

medullary lesions

Age -10-20 yrs

 Chromosomal translocation -t(11;22)

FLI-EWS gene fusion

Histology - small round

cells

 Resemble lymphoma

Tumors of the central nervous system

General features of the CNS tumors

85 % - intracranial, 15% - intraspinal tumors

 primary tumors and metastatic

20% of all - tumors of childhood.

differ from those in adults both in histologic subtype and location

arise in the posterior fossa ( in adults -mostly supratentorial)

Tumors of the nervous system have unique characteristics

 Histologic distinction between benign/malignant lesions – not clear

low-grade lesions (low mitotic rate, cellular uniformity, and slow

growth) may infiltrate large regions of the brain  The anatomic site of the neoplasm - lethal consequences

irrespective of histologic classification

A benign meningioma in the medulla cardiorespiratory arrest

The ability to resect a lesion may be limited

 The pattern of spread of primary CNS neoplasms differs from that of other tumors -rarely metastasize outside the CNS

(25)

CNS TUMORS

GLIOMAS

 Astrocytoma (I, II,

III,

IV

)

 Oligodendroglioma

 Ependymoma

MENINGIOMAS

NEURONAL

POORLY DIFFERENTIATED

(medulloblastoma) 

LYMPHOMAS

METASTATIC

CNS TUMORS

Symptoms?

Headache

Vomiting

Mental Changes

Motor Problems

Seizures

Increased Intracranial Pressure

any localizing CNS abnormality

CNS TUMORS

History

Physical

Neurologic exam

LP (including cytology)

CT

MRI

Brain angiography

Biopsy

Gliomas

Tumors of the brain parenchyma that

histologically resemble different types

of glial cells

astrocytomas,

oligodendrogliomas

ependymomas

(26)

Astrocytomas

Different categories of astrocytic

tumors

characteristic histologic features

distribution within the brain

age groups

Fibrillary

Pilocytic astrocytomas

Fibrillary

astrocytomas

80% of adult primary

brain tumors

Age – 40 -60 y

Localization

 cerebral hemispheres

seizures, headaches, and

focal neurologic deficits

Macroscopy

 a poorly defined, gray, infiltrative tumor  cut surface - firm, or soft or

gelatinous

 ± cystic degeneration and hemorrhage

Fibrillary

astrocytomas

Microscopy

I –IV grades

cellularity nuclear pleomorphism necrosis mitoses 

Astrocytoma (I, II gr.

Anaplastic astrocytoma (III

gr.)

Glioblastoma- IV grade

vascular or endothelial cell

proliferation and pseudo-palisading nuclei

(27)

Glioblastoma Multiforme

Pilocytic Astrocytoma

 Relatively benign tumors  Age - children and young adults  Localization

 Cerebellum,

 in the floor and walls of the third ventricle

 the optic nerves

 Macroscopy

 well circumscribed, often cystic

with a mural nodule in the wall of the cyst

 Microscopy

 areas with bipolar cells with long, thin "hairlike" processes, GFAP (+)  Rosenthal fibers (eosinophilic granular

bodies)

 Necrosis and mitoses are rare.

Oligodendrogliomas

 Frequency -5-15%  Age – 40-50 y  Localization  cerebral hemispheres  Macroscopy

 infiltrative tumors - gelatinous, gray  ± cysts, focal hemorrhage, and calcifications

 Microscopy

 sheets of regular cells with spherical nuclei containing finely granular chromatin (similar to normal oligodendrocytes) surrounded by a clear halo of cytoplasm

 a delicate network of anastomosing capillaries.

 Calcifications  Mitoses -rare

Except in anaplastic oligodendroglioma

(28)

Ependymoma

 Frequency -5-10%  Age – 10-20 y  Localization  IV –th ventricle  hydrocephalus  Macroscopy

 solid or papillary masses

 Microscopy

 cells with regular, round to oval nuclei with abundant granular chromatin

 perivascular pseudo-rosettes

 Variants

 Anaplastic

increased cell density, high

mitotic rates, necrosis

Ependymoma

Poorly Differentiated Neoplasms

Medulloblastoma

Neuroectodermal cells

Age - children

Localization

 cerebellum (vermis) 

Макроскопски

 well circumscribed, gray,  friable

Histology

 with sheets of anaplastic ("small blue") cells

 with little cytoplasm and

hyperchromatic nuclei

Rossetes of Homer-Wright mitoses – abundant

(29)

Meningiomas

Benign tumors of adults

 from the meningothelial cell of the arachnoid

Localization

 any of the external surfaces of the brain

 ventricular system

from the arachnoid cells of the

choroid plexus

Macroscopy

 well-defined dural-based masses  compress underlying brain

Meningiomas

Microscopy -variants

Fibroblastic - with elongated cells and

abundant collagen deposition

Psammomatous - with numerous

psammoma bodies

Secretory - with PAS-positive

intracytoplasmic droplets

Microcystic - with a loose, spongy

appearance

Atypical meningiomas – mitosis

Anaplastic (malignant) meningiomas

resemble a high-grade sarcoma

Meningeoma

Metastatic brain tumors

Most common brain tumor in

adults.

Common primary sites:

melanoma, lung, breast, GI

tract, kidney.

Most are in cerebrum

 in gray-white junctions due to rich capillarity

Single or multiple.

Discrete, globoid, sharply

demarcated tumors

 amenable to surgical resection.

(30)

Tumors of the peripheral

nervous system

Tumors of the peripheral

nervous system

Arise from cells of the peripheral nerve

Schwann cells,

perineurial cells

Fibroblasts

Schwannoma

Neurofibroma

Malignant Peripheral Nerve Sheath Tumor

Schwannoma

From Schwann cells

Symptoms – due to local compression

Localization

 in the cerebellopontine angle -attached to the vestibular branch of the 8th nerve (vestibular schwannoma)  sensory nerves, large nerve trunks

Macroscopy

 well-circumscribed encapsulated masses that are attached to the nerve

Morphology – 2 growth patterns

Schwannoma

Antoni A - Antoni B

 Antoni A pattern of growth

elongated cells with cytoplasmic

processes - fascicles

Verocay bodies

the "nuclear-free zones" of

processes that lie between the regions of nuclear palisading

 Antoni B pattern of growth

less densely cellular areas microcysts and myxoid changes

Immuhistochemistry

(31)

Neurofibroma

Well differentiated, benign

Form whorls of fibroblasts

Two types:

Classic form

Cutaneous / nerves – solitary collagen matrix, spindle cells,

Plexiform

Neurofibromatosis type 1 Multiple, infiltrative Myxoid stroma

Malignant Peripheral Nerve

Sheath Tumor

Sarcoma

 Highly malignant  multiple recurrence  metastases 

Origin

 De novo  plexiform neurofibroma 

Macroscopy

 poorly defined tumor masses  ±infiltration along the axis of nerve  ± invasion of adjacent soft tissues

Histology

 the tumor cells resemble Schwann cells - elongated nuclei

and prominent bipolar processes, fascicle formation

 Mitoses, necrosis, nuclear anaplasia

Tumors and Tumor-Like

Lesions of Melanocytes

Benign – melanocytic nevus

Malignant - melanoma

Melanocytic nevus

 Benign congenital or acquired

neoplasm of melanocytes

 Numerous types, with varied clinical

appearance

 Macroscopy

 relatively small,  symmetric,

 and uniformly pigmented

 Morphology

 Junctional

more pigmented, more closely associated with

melanoma)‏  Intradermal

(32)

Melanocytic nevus

Junctional nevus

Dermal nevus

MALIGNANT MELANOMA

Malignant proliferations of melanocytes.

Incidence rising,

Related to sun like all other skin cancers

The only primary skin cancer that can

quickly metastasizes

Sporadic

Hereditary -5-10%

Germ-line mutations in the

CDKN2A

gene

(9p21)

MALIGNANT MELANOMA

Difficult to differentiate from

nevus clinically

 often microscopically

Clinical features

 Skin

 a change in the color or size of a

pigmented lesion  less common sites - oral and

anogenital mucosal surfaces, the esophagus, the meninges, eye.

Morphology

 Vertical growth phase  Horizontal growth phase

 Related with prognosis

Breslow, Clark’s staging‏

MALIGNANT MELANOMA

Morphology

Malignant cells with large

nuclei with irregular

contours

chromatin characteristically

clumped at the periphery of the nuclear membrane

prominent eosinophilic

nucleoli -"cherry red"

nests or individual cells at

(33)

MALIGNANT MELANOMA

Teratomas

 =mixed tumors

 originate from totipotential stem cells the capacity to differentiate into any of the cell

types found in the adult body  Ovary, testis

 3 variants  Mature teratomas

contain fully differentiated tissues from one or more germ cell layers

 neural tissue, cartilage, adipose tissue, bone, epithelium in a haphazard array  Immature teratomas

contain immature somatic elements reminiscent of those in developing fetal tissue

mediastinum  Teratomas with malignancies

malignancy in preexisting teratomatous elements squamous cell carcinoma or adenocarcinoma

Mixed germ cell tumors of testis, 40%

combination of teratoma, embryonal carcinoma, and yolk sac tumors.

Figure

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References

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