DISORDERS OF BONE

The 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

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 or gingiva 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

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
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

5) gingival ulceration, or enlargement

>> 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

Langerhans Cell Histiocytosis Histopathology

How langerhans cells look like ?? They have kidney shaped nucleus and abundant cytoplasm ( plenty of cytoplasm ) In Langerhans 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

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

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

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

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 Fibroma
Dr talked about it quickly and nothing new .. every thing is written in the 2nd lec 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

Metastatic Tumors
Bone metastasis > soft tissue Mandible>maxilla Most common primary tumors that metastasize >> 1) Carcinoma: breast, bronchus, kidney 2) Prostate, lung Clinical features - pain, loose teeth, swelling, parasthesia, asymptomtic - Most are osteolytic , but breast and prostate tumors are osteoblastic

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 )

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

Metastatic in the jaws prefers to go to the bone but it may also go to the soft tissue and I have seen (the Dr) one patient only with a big gingival mass rapidly growing we thought this is biogenic granuloma but later on it turned to be metastatic adinocacinoma of the kidney

Special thx to my real sister Sanaa Maali <3

And to my lovely friends Yara Magableh & Rana 3'bareyah

Thx Rajaa 7adad cz am calling u all the time and keep asking u Qs , thx for ur patience and thx God bcz till now there is people own heart like urs And I will not forget u .. Banan , Muna ,Asal , Yara altebe , Sara kazkaz , 7aneen Mo7sen .. u all my sisters and am proud

Done By ::: HaNaa JadAllah