Connective Tissue Neoplasms (1)

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Connective tissue neoplasms

Today we will talk about tumors of connective tissue thatinclude benign and malignant

*Connective tissue neoplasm will appear clinically as overgrowth

* Connective tissue in our body are : bone , cartilages ,fibrous tissue , , neurons , adipose tissue

tumors of the fibrous tissue* it`s rare* cell of origin is fibroblast* benign tumor for the fibrous tissue is true fibroma

We have 4 types of the benign tumors of the fibrous tissue( ONLY you must know the name of it without knowing thehistology of it )

(1) the peripheral odontogenic fibroma(2) fibrous histiofibroma(3) nodular fasciitis ( neoplastic like lesion )(4) fibromatosis

remember :the most common lesion of the reactive hyperplasia ofconnective tissue is

fibroepithelial polyp ( irritation fibroma ) and it`shypocellular and contains collagens

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look to the gingival fibromatosis it is not neoplastic andthe patient need incisions to allow the teeth to erupt ( THIS

IS NOT the fibromatosis that we are talking about )

the fibromatosis we talked about will look like a mass whichis big and aggressive and it may lucent the teeth

but microscopically it is not malignant ( idon`t seehyerchromatism , mitotic figures , pleomorphism and othermalignant features ) only I will see hypercellularityand to distinguish this lesion from irritation fibroma we must

know that irritation fibroma is hypocellular

** so this picture is showing hypercellularity but it doesn`tshow malignant features so it`s called fibromatosis ( benigntumor )

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*

Fibrosarcoma

I may give you the same clinical picture for the benign

tumor but in histology I will see pleomorphism , elongatednucleus , rounded nucleus mitotic figures , hyperchromatismso I will consider it malignant tumor

** You should distinguish the irritation fibroma , theaggressive fibromatosis and the fibrosarcomaclinically and histologically

Tumors of adipose tissue

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* lipoma : benign tumor of the adipose tissue* it is usually circumscribed ( the borders are obvious )

Clinically

(1) it look yellowish(2) we can also see blood vessels on the surface(3) if you cut it will floats in formalin >> depending on thedensity of fat

Some infants and young children are presented to the clinicwith ulcerated tumor-like masses of fat in the buccal mucosawhich aren't actually tumors but traumatic herniation of the

buccal pad of fat

So if there is trauma in cheek the buccal pad of fat willherniate into the oral cavity in between muscles as a massso this will cause herniation due to trauma

WHAT is herniation ??For the intestine to be herniated , it will protrude into theabdomen muscles in weak areas

** according to histology we can say that the lipoma is(1) Fibrolipoma: which I have a lot of collagen in associationwith fat(2) angiolipoma: if I have a lot of blood vessels(3) myxolipoma : if the lipoma is with loose tissue which isthe myxoid tissue that have fibrils

NOW how do you feel lipoma ? it is soft or compressible , ifthere is a lot of fibrous tissue it may be firm ,, but commonlyit is soft

so according to the texture of the mass I can distinguish thetype of lesion that I have

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**To distinguish the benign from the malignant tumor wemust know that

(1) in BENIGN the the cell is mature and the nucleus of the

adipose cells is in the peripheral of the cell .(2) in MALIGNANT tumor the cell is immature , the nucleus isin the central and we have the malignant features .

Lipomaliposarcoma

Tumors of the vascular tissue

Hemangioma : some books consider it as true benign tumorand some said that it`s hematoma (hamartoma : is a normal tissue in it`s normal location butwith abnormal excessive Amount )

So in hemangioma we have increase of the blood vessels ,collection of blood vessels increase in number but in normal

location

Hemangioma may be present in(1) mucosa in the oral cavity(2) deep inside the muscles(3) inside the bone

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(4) any where . because we have blood vesselsanywhere

Maybe occur in(1) children

(2) young adults(3) some lesions will appear later in life

* parotid gland may have hemangioma because we haveblood vessels there* if we have multiple hemangiomas we should think aboutsturge weber syndrome

Clinical presentation

- Blue to purple in color ( it`s not red because bloodvessels are deep in the tissue or surfaced by tissue )

- Soft or comprisable ( it mean that i`m having a fluidinside this lesion , the fluid is blood )

-- they blanch on pressure (get white when pressurized

with a glass plate) and the color in the pressing areawill become pale , however some lesions havethrombosis or calcifications and get hard and so

they don't blanch- hemangioma may increase in size if bleeding ,thrombosis , calcification and secondary inflammationoccur inside the lesion

- it can be one lobule or it can be multi lobulated

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Multilobulated hemangioma multilobulatedhemangioma in the

in the lower lip tongue as a purplemass and it maybe of many years

durationhistological picture

- According to the size of vascular spaces we may havedilated blood spaces

(you should keep in mind that the blood spaces are lined byendothelial cells unlike the aneurysmal bone cyst which isalso collection of red blood cells in spaces but the spacesare not lined by endothelial cells )

- the hemangioma maybe capillary , cavernous and mixed

- cavernous hemangioma : when the blood vessels aredilated

- The location of the blood vessels are not superficial , theyare deep in the tissue , below the epithelium , it goes down

- In lymphangioma the lymphatic vessel or channels aresuperficial

- Capillary with cavernous are called mixed

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**this picture shows cellular hemangioma because I see alot of endothelial cells , they are forming tube capillary size

vessels but they are sheets of endothelial cells so weconsider it cellular hemangioma not capillary hemangioma

** we use a stain called CD 34 to be sure that these cells areendothelial cells >>>> so this test is positive in endothelialcells

In this picture some people will say that it is capillaryhemangioma because the endothelial cells will form likecapillary tube vessels so it`s cellular and in worst

possibilities it chould be capillary but not cavernous or mixed

**Cellular hemangioma occur commonly in young agechildren

WE HAVE other malformation other than hemangioma like :

(1) arteriovenous malformation : when the there is arterynext to the vein

NORMALLY : we have the artery then arteriole thencapillary then veniole then vein .

*but here you have thick muscular artery ( with smoothmuscle wall ) next to thin walled vein whithout passingthrough capillary stage

* in this lesion you will feel pulsation

(2) malignant vascular lesion

Malignant tumors of endothelial cells :

(1) angiosarcoma :

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**it will metastasize**the malignant cells are endothelial cells** we will see the malignant features ( pleomorphism ,hyperchromatism , hyper mitotic figure , variation in size

and shape )

(2) Kaposi sarcoma :** intraorally or in the skin ( permented patch , in sunexposed or non exposed areas )** it will not metastasizeWe will talk about it when we talk about AIDS later on ..

Angiomatous syndromes

(1) sturge weber syndrome

Here in this syndrome we will see multiple hemangiomasfollowing the branches of the trigeminal nerve extraorallyand intraorally

We can see :(1) enlargement of the gingival

(2) redness(3) macroglossia ( increasing the size of the tongue )

** also hemangiomas in the meninges can occur over thecerebral cortex in the same side and convulsion occur inthe opposite side of the body

(2)Hereditary hemorrhagic telangiectasia:

- An autosomal disorder characterized by multiple knots ofdilated malformed and fragile capillaries in skin, mucousmembranes and may be the internal organs

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- Frequent nose bleeding (epistaxis) is the commonestpresenting symptom

(3)Lymphangioma

** dilated lymphated channels located superficially inmucosa so you can see small bubbles in mucosa** increase in size may occur due to inflammation orcalcification

`

Here you can see the tongue with small lobules because ofthe dilated lymphatic channel underneath the epithelium .

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* here we can see empty spaces and some spaces containlymph

** Some times in lymphangioma there will be apical locationand also containing RBC >> it maybe secondaryhemorrhage or bleeding at the time of operation

In the presence of the RBC the location of the channel willthe indication that will help us distinguishing betweenlymphangioma and hemangioma

** we have special stains to the endothelial lining of thelymphatic channels

Cystic hygroma:*It is a Lymphangiomatous malformation that occur earlyin development of lymphatic system* Lesions are detected at birth and present as large swellingoften up to 10 cm in diameter

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*They most frequently affect the head & neck region, butmay extend to involve the base of the tongue, the floor ofthe mouth, and less commonly buccal mucosa

Tumors of the peripheral nerves

1. Nerve sheath tumors:1. Neurofibroma

- Solitary- Multiple (neurofibromatosis)

2. Shwannoma2. Traumatic neuroma3. Multiple mucosal neuroma

NEUROFIBROMA

clinically :

I have a mass, this mass is soft (compressible) or firm???It will be FIRM because it contain cells (Schwann cells &fibroblast).** neurofibroma may be well defined or it may be diffuse

(both clinically & microscopically) ,so sometime clinically it

will be well defined ,thats mean I can determine the bordersof the mass but most of the time it will be diffuse .

HISTOLOGICALLY:

we have 2 components , look here >>I have amass containing spindle cells as I see, I may thinkthese are fibroblast , but if we look carefully , we will seecells with wavy nuclei (coma shape nuclei \ or dote cell like

nuclei ) these cells are considered as Schwann cells mixedwith other spindle cells .

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Immediately I should ask for a stain called s100 stain ,which is positive for Schwann cells , and in this case thelesion will not be 100% positive coz we have a fibroblast( not all of the cells are Schwann cells ,so we will have somepositive & some negative ) .sometimes we can point a few nerve axons (u should knowthat the Schwann cells are not as the same as nerve axon,Schwann cells are nerve sheathe cells at periphery of thenerve, but the axon are the axon of the nerve itself!! andcontain myelin inside it) so, if I ask for stain for myelin, willsee occasional nerve fibers among these cells.

Look at these cells (in slide 81) >> they are wavy cellsand bind with coma shape nuclei.

NEUROFIBROMATOSIS I

In this syndrome we have a mutation in NF1 gene (tumorsuppressor gene) what is the characteristic of thissyndrome???

1- multiple neurofibromas of cutanous nerves ( nerve thatspreads all over the skin)

if this neurofibromas are removed surgically , they showthat they will carry higher tendency for malignancytransformation .2-there will be also freckeling , and this freckeling is in thearea that are not sun exposed(axillary freckeling).3-caf-au lait spots, they have brown pigmented spot ontheir skin & these spots are big & have smooth borders.((The DR asks: give me another syndrome include caf-au

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lait spot???McCeune Albright syndrome))4- malignant transformation 5-15% of all cases , but it willincrease with recurrent surgical removal of these masses.

Kind of neurofibroma that is most commonly associated iscalledPLEXIFORM NEUROFIBROMAso it is the most common with this syndrome, so if I havediagnosed microscopically that the pt have plexiformneurofibroma, I should write note for the clinician to examinethe pt for neurofibromatosis.

SCHWANNOMA

in schwannoma mainly or almost all of the lesion masscomposed of Schwann cells .In this case the tumor arises adjacent to the nerve (forexample: we have the mental nerve & adjacent to it we havemass that attached to the nerve itself).Usually the nerve fibers dont pass through the lesion, theypass over it or they attached to it (this feature is important).so its like this

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Here we have a mass, its a

benign tumor, its keep enlarging,and its next to the mentalnerve.It has acharctarstic appearancethat there will be parallelarrangement of the nuclei of theSchwann cells, thats meanthese cells are arranged inparallel bundles withpalisaded nuclei.so, u will see cellular area &acellular area (I dont see this infibroma ).if I do stain for this lesion (S100stain) , what do u think that uwill see?? 100% positivity or50%???


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TRUMATIC NEUROMA

we dont have a defiant pattern of traumatic neuromas

for example, if the pt have an extraction & there was a cut inthe mental nerve after extraction, then this nerve will givean attempt to regenerate and try to make healing, buthealing will not occur, and we will see small nerveshaphazardly scattered throughout the mass in addition toSchwann cells at the periphery &maybe fibroblast (scartissue).

So we have a mixture of nerves +Schwann cells+fibroblast in haphazard organization.Clinical features:

1- painful to palpation coz it contain nerves inside it.2-firm coz it contain cells & it may be fixed to thesurrounding str3-it is occur usually at big or large nerves such as mentalnerve*** u should keep in mind that this is not neoplastic it isreactiveso , neurofibroma + schwannoma >>>neoplastic

traumatic neoromas >>>reactive (to cutting in the

nerve)

NOW, if I saw this appearance in multiple region inside theoral cavity (this neuromas ,this histological appearance )y3ne the pt have multiple lesion with similar diagnosis(multiple mucosal neuromas ) , I should think aboutsyndrome called MULTIPLE ENDOCRINE NEOPLASIATYPE IIb(MEN) .

-in this type , we will have multiple mucosal neoromas , andin this case mucosal neoroumas are NOT due to cut in thenerve , coz they are appear as a component of the syndrome( but in the traumatic neoroumas they found there duo tocutting in the nerve).-mutation in RET oncogene lead to this syndrome .

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-in this syndrome the pt has medullary thyroid carcinoma(this is the dangerous part of the syndrome).**medullary thyroid carcinoma is dangerous & fatal &diagnosed late in life.

**so if u diagnosed your pt as having multiple neoromas ,umaybe the one to save the life of the pt coz he may have thethyroid gland to be removed completely (prophylacticthyroidectomy) before medullary thyroid carcinoma occur .SO, WE HAVE >>1-multiple mucosal neoronas2-phaecromocytoma(tumor of adrenal medulla)3-medullary thyroid carcinoma-RET oncogene screening can be used for prophylacticdetection of this pt ,if u are thinking that your pt have

MEN ,then RET oncogene screening is important.

GRANULAR CELL TUMOR

- the cytoplasm of the cells in this lesion contain granules-it is originate from Schwann cells ,previously they thoughtthat it is originate from skeletal muscle (they thought that itis a muscular tumor) but later on they found that this tumororiginate from Schwann cells(thats mean the granular cells

will stain with S100 stain).-it is a benign tumor, probably of Schwann cells.-previously , it was called myoblastoma (coz they thoughtthat its originate from skeletal muscle )-the best location for granular cell tumor is the TONGUE (cozit contains skeletal muscle) but it also may occurs at bucclemucosa or anywhere inside the oral cavity .-it is firm to palpation & it is attached to the overlyingmucosa and the underlying tissue so that it is not welldemarcated .

The Histology picture:

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-in the histological picture u can see skeletal muscle fibers,

and we have a sheet of cells contain granular cytoplasm,these granules are lysosome.-we have abundant or plenty cytoblast (granular cells) ,thesecells are not small,they are big cells with granules ,and theyinterpreting with skeletal muscle fibers, for this reason , theyused to be called myoblastoma (coz they thought it arisefrom skeletal muscle due to this appearance).

PSUEDO-EPITHELIOMATOUS HYPERPLASIA

-the surface epithelium is hyperplasic but it will give u anindication that it is as a malignant since it is invading theunderlying tissue(this feature seen in some lesion likecapillary(sorry I cant hear) hyperplasia ,granular celltumor ,& later on we will talk about deep fungal infection( when we talk about the candidosis & deep fungal inf)SO,-it is actually hyperplasia, but it will give u an indication thatit is a malignant.

_there is an intimate contact with the surface & theunderlying structure for this reason the granular cell tumor isnot movable (fixed to the surrounding structure, but it is abenign).

NOW, based on these features, does u think that we need anaggressive treatment???Wide surgical excision (for granule

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cell tumor)???Actually NO despite the fact that it is intermingling theskeletal muscle & it is ill-defined but it doesnt need anaggressive treatment, they found that it will not recur after

conservative surgical treatment.

TUMORS OF the MUSCLE

you should know the terminology:*benign tumors of smooth muscle are called >>leiomyoma*malignant tumors of smoothe muscle are called>>leiomyosarcoma*benign tumor of skeletal muscle is called>>rhabdomyoma*malignant tumors of skeletal muscle are called>>rabdomyosarcoma

-leiomyoma sometimes, it is vascular, it is appearing likehemangiomawhy???Coz the smooth muscle occurs in association of blood vesselso some times leiomyoma maybe vascular.

LYMPHOMAtypes of lymphoma:1-hodgkins lymphoma2-non-hogdkins lymphomawhat is the lymphoma that start in the lymph node??Hodgkins lymphomaalso Hodgkins lymphoma prefer to occur in the cervicallymph node**non-Hodgkins lymphoma usually start in extra nodal

location and then it is metastasis later on or spread andreach the lymph node.

1-HODGKINS LYMPHOMA-30% of all lymphoma

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(EBV may be linked to the most ofthe types of lymphomas , coz thelatency period of EBvirus is inside thelymphocyte especially B cells, so theyfound that it is correlated withl m homa


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-it is prefer young age group-it is prefer cervical lymph node-they found that EBV may be linked to Hodgkins lymphoma .-genetic favor may play role, coz they found that in twins ,if

one child haveHodgkins lymphoma there will be a high chance that theanother childe to get lymphoma.-prognosis depends on staging & grading WHAT IS THEDEFFERANCE??Staging >>depend on clinical featuregrading >>depend on histological feature.

Look to the cervical chain ,look how the lymph node showdiffuse enlargement ,and they are linked ( ) u cant

move them , and the texture is rubbery and firm ,it is notsoft like in reactive lymph node ( when u have tonsillitis ,uhave a lymph node enlargement but the texture is soft andthe lymph node usually tender (painful )) but in this case NO,they are rubbery ,firm ,& attached to each others like onechain .

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These cells are called REED- STERNBERG cells; it is themalignant components in the Hodgkins lymphoma.

These cells are:1-big cells2-with 2 nuclei3-or one big cell with one nucleus in pop-corn appearance.

The cells that surrounding these reed-Sternberg cells arelymphocytes and they arenot malignant.SO, what does u think is better for the pt, to have highnumber of

reed-Sternberg cells??Y3ne to have lymphocytespredominant orreed-Sternberg predominant???Lymphocytes predominate.

For this reason in the histopathological type thelymphocytes predominatehas the best prognosis then mixed cellularity thennodular sclerosis then lymphocytedepletion?

2-NON-HODGKINS LYMPHOMA

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SO, as the numbers of lymphocytes decrease, then theprognosis will be bad for the pt


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-it starts extra nodally .**may occur in the mucosa then it will be called MALTlymphoma (mucosa associated lymphoid tissue lymphoma).

**it may occur in the stomach in association with H-pylori (uknow that H pylori infection associated with lymphoma, soanyone have H pylori infection in the stomach, he shouldget a treatment, since it may coz lymphoma).**it may occur inside the salivary gland itself especially insjogren syndrome .**it may occur inside the bone**in may occur in association with AIDS pt-cell types:mainly they are B-cell types, but T-cell or NK cells also may

present.

here we have inside the oral cavity an aggressive mass withbleeding , it is destroying the teeth ,there is also ulceration ,it is ugly , rapidly growing , we should think about malignantprocess ,if we are not sure , we take abiopsy ,send it to thelab,and then in the lab the histopathlogist will see a sheet ofsmall cells ( lymphocyte ) but there will be hyperchromatisim& mitosis in some of them , and we will order especial stain

then we will tell the clinician that we are having here B-celltype lymphoma ( if we have, for example, CD20 positive cells).

BURKITS LYMPHOMA

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it is endemic & sporadic (endemic means it is occur incertain georafephic location, sporadic ).- in certain geographic location like Africa ,when there is

EBV and malaria together then the immune system willaltered ,then the pt will have burkits lymphoma .-the clinical presentation for endemic burkits lymphoma isdiffer from sporadic**in the endemic >> it will start in the jaws**in the sporadic >>it will start in the abdomenSO in the endemic burkits lymphoma the pt will have jawlesion then spread will occur .-there is a specific chromosomal translocation that it is 8,14which will lead to c-myc activation .

-the cells in burkits lymphoma >> B-cellshistologicallywe will see something called starry sky appearance ,u willsee a blue areas and stars, the stars are non-neoplasticcomponent which are the macrophages (u know that themacrophages are big cells & have abundant cytoplasm andthe color of the cytoplasm is pale compared to the nucleus ,so it appears pale , and look to the cytoplasm it is containinglymphocyte coz the macrophages trying to engulf the

malignant cells in order to kill the malignant cells).

NK\T CELLS LYMPHOMA

-it is called angiocentric T-cell lymphoma.-it is occurring around the blood vessel.-it is occurring in the mid line.-it used to be called lethal midline granoloma (coz it cozdeath if it occurs in the palate)-if it occur in the palate, it will coz perforation of the palate, itwill reach the nasal cavity.-the cells either T-cell or NK.-they found that EB virus in these cells, and it is also presentin most of other types of lymphoma.

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Done by : sondos harbieh and haya al momani

Date post:	04-Apr-2018
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Connective Tissue Neoplasms (1)

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