script #13, bone disorders 5
TRANSCRIPT
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DISORDERS OF BONEThe last lecture about bone disorders .. Be Happy :)
We talked last lecture about Osteosarcoma , Chondrosarcoma and Multiple Myeloma and now we
are gonna talk about Langerhans Cell Histiocytosis
A) Langerhans Cell Histiocytosis--->
Langerhans cells: are antigen presenting cells (APC) present in epidermis, lymph
nodes, mucosa and bone marrow
They are so important for the immune system >> they engulf the particles (
antigens ) and present them using MHC ( Major Histocompatibility Complex ) to the
lymphocytes and then the lymphocytes will induce adaptive immunity which is a
stronger immunity
Langerhans Cell Histiocytosis is a rare disease involving colonal proliferation of the
Langerhans cells ( histocytosis ) >> it's a destructive lesion
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Because they present in the bone marrow they may get monoclonal proliferation
and any thing proliferate monoclonally it is not reactive it is neoplastic , but if any
proliferation was polyclonal it will be reactive not neoplastic
Monoclonal -- > Neoplastic -- > Langerhans cell histocytosis Polyclonal -- > Reactive
Langerhans cells increase in number but they are malignant >> they are not
following the cell cycle control proteins >> so they will proliferate for ever and they will
acquire more mutation >> more aggressive >> more invasive & more malignant
behavior
Langerhans Cell Histiocytosis
Three clinical forms >>
Classified into three forms due to the extension :
1st
form single lesion called solitary , 2nd
form multifocal the bone is involved in addition
to other organs , the 3rd
form the bone is involved in addition to the skin and other
organs
1) Solitary eosinophilic granuloma of bone : called eosinophilic due to present of
eosinophills mixed with langerhans cells
Usually single lesion >> Unifocal >> in the bone , soft tissue , mandible , maxilla orgingiva
2) Multifocal: bone and other organs ( soft tissue )
called Hand Schuller Christian syndrome (triad) where there is involvement of
the orbit and pituitary gland giving skull defects , exophthalmus of the eyes and
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diabetes insipidus ( due to the involvement of the pituitary gland , all the hormones
will be affected or involved leading to DI )
3) Disseminated histiocytosis ( multi-organ disease ) >> not combatable with life >>called Letterer Siewe disease
maybe chronic or acute
this disease is usually Fatal
the multifocal form here usually involves the bone and some other organs but not as
extensive and disseminated as the 3rd form which is >>>> occurs mainly in infants and
childrens under 2 years of age and have a high mortality
Langerhans Cell Histiocytosis
Clinical features
1) It is ill defined , it destroyed the teeth by root resorbtion >
perforate the cortex
2) resorbe the bone >> loosening of teeth
3) Radiographically: floating in air ( important )
4) May simulate a periapical lesion
5) gingival ulceration, or enlargement
Here there is a periapical lesion that doesn't really make sense >> the tooth (1) here is root
canal treated and here (2) is vital having nothing and here (3) is vital too >> so why we have
a big radiolucent area which is ill defined and the teeth maybe loos ?? >> we will think about
neoplastic lesions >> we will take a biopsy and the lesion will turn aout to be langerhans cell
histocytosis
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Langerhans Cell Histiocytosis
Histopathology
How langerhans cells look like ??They have kidney shaped nucleus and abundant cytoplasm ( plenty of cytoplasm )
InLangerhans Cell Histiocytosis Proliferation of Langerhans cells accompanied by:eosinophils, plasma cells and multinucleated giant cells
Electron microscope: Birbek granules
Immunohistochemistry: surface Ag: CD1-a
u know that some cells have antigens on there surfaces that distinguish them from
others like CD3 on the T cells CD20 on the B cells but on the langerhans cells the
antigen that we look for is the CD1-a >> so if u think that this lesion is langerhans cell
hystocytosis u have to ask for a special stain for the CD1-a antigen if it is positive then
it is positive to the disease if not it is not
>> The lesion is focal sometimes
Here the patient has good healthy teeth but
focally there is destroyed periodontium after
radiograph ( look at the radiograph in slide#50
or in the previous page in the lecture ) there was
a radiolucent region , ill-defined , destroying the
bone and resorbing the root >> after biopsy the
lesion turned to be langerhans cell histocytosis
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In the picture above
Arrow1 >> big cell it looks like the macrophage , it is big it has plenty of cytoplasm
(pink) and big nucleus it is oval to kidney shaped ( and may be indented nuclei ) and
the cells are polymorphic >> variation in the size and shape of the nuclei and stain
characteristic
Arrow2 >> eosinophil with pink cytoplasm >> they looks like neutrophil but the
cytoplasm is pink and the neutrophil is having mutilobulated nucleus
The eosinophils are not neaoplastic , the langerhans cells which are neoplastic and
proliferate monoclonally
Langerhans Cell Histiocytosis
Treatment
1) Curettage
2) Radiotherapy
3) Intralesional injection with steroid it may work
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A) Hemangioma of Bone--->
Sometimes is considered as developmental changes not a true neoplasm but
some of the books is still consider hemangioma as benign tumor of blood vessels
Hemangioma may occur in any location where blood vessels are May occur inside the bone , called >> central hemangioma of bone and this type
usually give us >> Multilocular radiolucency bcz it extents within all marrow spaces
and start to expand
Even there is expansion but there still bone trabeculae >> but the trabeculation is
relatively more radiolucent If the lesion is aspirated it will reveal fresh blood and this will confirm the
diagnose
Usually it is cavernous type >>
What is the difference between cavernous type and capillary type ??
The size of the blood vessels ( capillary > small blood vessels , cavernous > big
dilated blood vessels )
This is Multilocular radiolucency extents within all marrow spaces in the body of
the mandible
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This is Multilocular radiolucency or honey cell in the anterior region of the
maxilla >> it's not malignant bcz I don't see root resorption >> the root are pushed
away which means this lesion is slowly growing giving time for root to be displaced
After biopsy u will see bone marrow trabeculation>> (pink)
And in between there are lots of dilated blood
vessels and they are mainly of the cavernous type
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Now we gonna talk about tumors of bone , we finished reactive conditions ,
inflammation and unknown etiology and now we will continue with tumors of bone ..
Ossifying FibromaDr talked about it quickly and nothing new .. every thing is written in the 2
ndlec of
bone but I put her discussion about pictures as revision
This is a general picture for fibro-ossious
lesions so we can't determine it is
ossifying fibroma or any other fibro
osseous lesion
1>> is bone trabeculea and it is vital bone
bcz it contains osteocytes inside lacunae
But when we look at the mass we
will say >> this is ossifying fibroma
bcz it is one piece and encapsulated
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Metastatic TumorsBone metastasis > soft tissue
Mandible>maxilla
Most common primary tumors that metastasize >>1) Carcinoma: breast, bronchus, kidney
2) Prostate, lungClinical features- pain, loose teeth, swelling, parasthesia, asymptomtic
breast and prostate tumors are osteoblasticbut,Most are osteolytic-
I can see in the picture one of the ID
canal borders so I will say >> this lesion
isn't below the ID canal >> it isn't staphne
bone defect >> it has to be biopsied
Now after biopsy it turned to be metastatic
tumor
This island is malignant glandular like
tissue or carcinoma >> metastasizing
from a primary tumor ( from kidney ,
lung etc )
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this is a destructive lesion in the body and
the angle of the mandible
This is the histopathology for the previous
radiograph >> there is a glandular (bcz
there is space in the center ) collections
of malignant cells ( they are dark in
color) >> so this is metastatic carcinoma
How do we know that this is carcinoma??
Epethilial cells usually like to be cohesive
and form islands ( bcz they have
dismosoms and hemidismosoms
This is a Metastatic disease >>
Here we have condensation of bone ..
U may say this is bone scar , u may say this
is metastatic osteoblastic tumor or
condensing ostitis
But later on it will turn to be metastatic
disease
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