11 09 2022 – ONCOLOGY

HEREDITARY DISEASES
• Cherubism
• Osteopetrosis
• Osteogenesis Imperfecta
• Cleidocranial Dysplasia
• Crouzon's syndrome (Craniofacial Dysostosis) OSTEOPETROSIS
• Treacher collins syndrome (Mandibular Dysostosis)
• Pierre Robin Syndrome
• Marfan’s Syndrome
• Ehler’s-Danilos Syndrome
• Down Syndrome (Trisomy 21)
• Hemifacial Hypertrophy
• Clefts of the Lip and Palate
• Fragile X-syndrome
• Mutations of SNX10 gene
CHERUBISM • Symptoms in pelvic area and bones in the head
• When fibrous dysplasia occurs in jaw, there is • Also known as Marble Bone Disease or Albers-
bilateral swelling of the angle of the jaw Schonberg Disease
*fibrous dysplasia – abnormal growth of tissue in
• It is an extremely rare inherited disorder whereby
place of a normal bone
the bones harden, becoming more denser
• Accompanied by upturned eyes from facial fibrous
• It can cause osteosclerosis
dysplasia, gives a cherubic look to the face.
*cherub = angelic
Cause
• Mutations in the SH3BP2 gene have been identified
• Normally, bone growth is a balance between
in about 80% of people with cherubism
osteoblasts (cells that create bone tissue) and
osteoclasts (cells that destroy bone tissue)
General Features:
• Sufferers of osteopetrosis have a deficiency of
• Autosomal dominant condition
osteoclasts.
• Self limiting, stabilizes after puberty
*mild case Symptoms
• Microscopically is a giant cell lesion • More brittle bone than normal
o Characteristic condensation sometimes
• Mild osteopetrosis may cause no symptoms and
perivascular collagen present
present no problems
• Serious forms can result in stunted growth,
Clinical Features:
deformity, increased likelihood of fractures and
anemia
• It can also result in blindness, facial paralysis and
deafness, due to the increased pressure put on the
nerves by the extra bone

Treatment
• Symmetric, asymptomatic swelling of the jaws • There is no cure, although curative therapy with
• Painless symmetric enlargement of the posterior bone marrow transplantation is being investigated
region of the mandible with expansion of the • If complications occur in children, patients can be
alveolar process and ascending ramus. treated with vitamin D
*can result to masticatory speech, swallowing • Erythropoetin has been used to treat any
difficulty associated anemia
• Corticosteriods may alleviate both the anemia and
Radiographic Features:
stimulate bone resorption
• Soap bubble radiolucencies
• Fractures and osteomyelitis can be treated as usual
OSTEOGENESIS IMPERFECTA CLEIDOCRANIAL DYSPLASIA
• Mutation of COL101 and COL102 gene • Cleidocranial Dysplasia (cleido = collar bone, +
cranial = head, + dysplasia = abnormal forming),
Features: also known as Cleidocranial Dysostosis
• Frequent bone fractures that may begin before • A condition characterized by defective development
birth that results from little or no trauma of the cranial bones and by the complete or partial
• Blue sclerae, short stature, hearing loss, respiratory absence of the collar bones (clavicles)
problems
• Associated with the disorder of tooth development
called dentinogenesis imperfecta

Characteristics include:
• Delayed closure (ossification) of the space between
the bones of the skull (fontanels)
• Premature closing of the coronal suture
• Protruding jaw (mandible) and protruding brow
bone (frontal bossing)
*mango or moon shape
• Wide nasal bridge due to increased space between
the eyes (hypertelorism)
• High arched palate or possible cleft palate
• The most severe forms of osteogenesis imperfecta, • Short stature
particularly type II, has the following features: • Scoliosis of the spine
o An abnormally small, fragile rib cage and
underdeveloped lungs Cause:
*shortened of breath • It is transmitted as an autosomal dominant trait
o Infants with these abnormalities have life- • The cause is not yet known, but several
threatening problems with breathing and chromosome abnormalities have been linked with
often die shortly after birth this syndrome, including chromosome 6p21
• And mutation of RUNX2

The child may have the ff. problems:

• Dental abnormalities – failure to lose the baby teeth
(deciduous) at the expected time; slow eruption of
secondary teeth; extra teeth; delayed or absent
formation of teeth
• Ability to touch the shoulders together in front of
the body
• Wide pelvic bone
• Loose joints
• Hearing loss and/or frequent infections
• Type I – Female, Age:30 yrs old, Height: 171 cm
• Type I – Female, Age:63 yrs old, Height: 137 cm Treatment Options:
• Type IV - Male, Age:40 yrs old, Height: 90 cm • Apply dentures over the unerupted teeth
• Type IV - Female, Age:35 yrs old, Height: 124 cm • Teeth removal as they erupt, because very little
• Type III - Female, Age:27 yrs old, Height: 94 cm bone structure would be left if the supernumerary,
• Type III - Male, Age:40 yrs old, Height: 84 cm impacted and unerupted teeth were all extracted at
once
• Some doctors suggest that the removal of primary • The protein coded by this gene is called treacle and
or supernumerary teeth does not promote eruption has been hypothesis to assist in protein sorting
of unerupted permanent teeth. In addition, during particular stages in embryonic development,
permanent teeth may be difficult to extract due to particularly that of the structures of the head and
malformed roots face.
• The disorder is inherited in an autosomal dominant
CROUZON’S SYNDROME (Craniofacial Dysostosis) pattern
• Crouzon syndrome is caused by mutations in the • People with the syndrome can undergo surgeries on
FGFR2 gene, which is mapped to chromosome the face to improve appearance, get hearing aids,
locus 10q25 to 10q26 and can also undergo surgery on a cleft palate.
• facial characteristics = cleft lip and palate
• No specific treatment

PIERRE ROBIN SYNDROME

• Mutation of GAD67, PVRL1 and SOX9 genes
• Pierre Robin Sequence (PRS)
• Also known as PIERRE ROBIN SYNDROME or PIERRE
Characterized by: ROBIN MALFORMATION.
• Triad of calvarial deformities, facial anomalies and • A congenital condition of facial abnormalities in
exophthalmos (popping out of the eyes) humans.
• Premature closure of calvarial and cranial base • As PRS is not caused by a single defect gene, it is not
sutures as well as those of the orbit and maxillary a genetic syndrome, but rather a sequence: a chain
complex (craniosynostosis) of certain development malformations, one
• Other clinical features include hypertelorism, entailing the next.
exophthalmos, strabismus, beaked nose, short • A 5-month old aby with pierre robin sequence and
upper lip, hypoplastic maxilla and relative severe micrognathia
mandibular prognathism *micrognathia = mandibular retraction

TREACHER COLLINS SYNDROME (Mandibular

Dysostosis)
• Aka Mandibulo-facial dysostosis
• Treacher collins syndrome is a rare genetic disorder
characterized by facial deformities
• Found in 1 in 10,000 births
• Downward slanting eyes • A patient with pierre robin sequence and small
• Micrognathia (a small lower jaw) mandible.
*happens in maxilla
• Conductive hearing loss
• Underdeveloped zygoma, dropping part of the
lateral lower eyelids and malformed or absent ears
*bird-like or fish like appearance

PRS is characterized by:

• Micrognathia
• Posterior displacement or retraction of the tongue
(glossoptosis) and upper airway obstruction
• Incomplete closure of the roof of the mouth (cleft
• One known cause of this syndrome is a mutation in palate commonly U-shaped)
the TCOF1 gene at chromosome 5q32-q33.1
 MARFAN’S SYNDROME
Cause:
• (Marfan syndrome or Marfan’s syndrome) is a
genetic disorder of the connective tissue
• It is carried by a gene called FBN1, which encodes a
connective protein called fibrillin-1

Characteristics:
*u shaped and v shaped • Skeletal = long limbs
• Ocular = dislocated lenses
CLEFTS OF THE LIP AND PALATE • Cardiovascular = aortic root dilation
• Cleft lip (cheiloschisis) and cleft palate • Other characteristics include a long, narrow face,
(palatoschisis), which can also occur together as • Roof of the mouth may be arched causing teeth to
cleft lip and plate be crowded
• Clefting is congenital deformity caused by • Other skeletal abnormalities may include a
abnormal facial development during gestation protruding or indented breastbone, curvature of the
• Cleft lips or palates occur in somewhere between spine, and/or flat feet
one in 600-800 births • They are typically very tall, slender and loose
jointed. The long bones of the skeleton, arms, legs
fingers and toes may be noticeably long in relation
to the rest of the body

• Types:
o Unilateral incomplete
o Unilateral complete
• Dislocation of lens (ectopia lentis)
o Bilateral complete
• Myopia (nearsightedness)
• Ascending aortic dilation

• Chest deformity:
o Pectus carinatum (protrusion of breast
*most common = unilateral complete cleft lip and bone)
palate o Pectus excavatum (indentation of breast
bone)

• A baby being fed using a customized bottle

customized bottle = bent upto 45 to 90 degrees
• The upright sitting position allows gravity to help
the baby swallow the milk more easily
• Parts of the Body Affected by Marfan Syndrome
o Eyesight
▪ Near-sighted (myopic)
▪ Eye (or ocular) lens dislocation
▪ Retinal detachment
o Lungs
▪ Spontaneous lung collapes
(pneumothorax)
o Cardio-Vascular System
▪ Aorta widening or dilatation
▪ Aortic aneurysms
▪ Mitral and/or aortic valve(s)
prolapse/ leakage • Race
o Skeleton o No racial predominance seems to exist;
▪ Curvatire of the spine (scoliosis) however, some believe that whites
▪ Pigeon or funnel chest (pectus probably are affected more than other
deformity) races
▪ Tall stature • Sex
▪ Loose jointedness o The sex-related prevalence are almost equal
• Age
Management o Begins in early childhood. Ehlers-Danlos
• There is no cure for Marfan syndrome syndrome is usually diagnosed in young
• The syndrome is treated by annual medical adults.
examination to check condition of the heart, eyes
and skeletal system. Supportive treatment include Orofacial features:
the following: • Narrow maxilla
o Restricted physical activity • Flattened midface
o Antibiotic prophylaxis for infective • Wide nasal bridge
endocarditis • Fragility of gingival and mucosal tissues
o Beta blockers (propranolol) to reduce aortic • Temporomandibular joint dysfunction
stress • Marked extensibility of the tongue
o Composite grafts to replace aortic valve
Treatment and Prognosis
EHLER’S-DANLOS SYNDROME • Sudden death in youth or early adult life may occur
Cause: as a result of aneurysmal and ruptured arteries
• Inherited defects in collagen metabolism • Joint ligament repair is often unsuccessful because
of suture failure, delayed wound healing, prolonged
Characterized by: healing
• Joint hypermobility • Osteoarthritis is a common complication in patients
• Cutaneous fragility with repeated dislocation
• Hyperextensibility
• Type IV is a severe form. Patients often have a DOWN’S SYNDROME (Trisomy 21)
shortened lifespan because of the spontaneous Cause:
rupture of a large artery (eg, splenic artery, aorta) • Down’s syndrome is a genetic condition that is
or the perforation of internal organs caused by the presence of an extra chromosome
*there are 13 subtypes but type IV is the severe • There are 23 pairs of chromosomes in every cell,
form Vascular Ehler’s-Danlos Syndrome which makes 46 all together
• Those with Down’s Syndrome have an extra copy o
Characteristics: chromosome 21, which makes 47
• Joint hypermobility
• Skin hyperextensibility and fragility Features:
• 1 in 600 births
• Short broad nose
• Epicanthal fold *sunken appearance
• Small oral cavity • Unknown cause: maybe due to trauma, dysfunction
• Large furrowed tongue of peripheral nervous system (NS), infection, and
• Large irregular teeth genetic abnormalities
• IQ from 20-50 • Early sign - painless cleft or furrow near the midline
of the face
• Orally, tongue, lips and salivary glands may show
hemiatrophy
• Dental abnormalities include incomplete root
formation, delayed eruption, severe facial
asymmetry resulting in facial deformation and
difficulty with mastication

Treatment
• Spots on the iris known as Brushfield spots • Presently there is no known definitive treatment
but all available treatment schemes are adapted to
Most common manifestations: the specific dysmorphology of individual patients
• Characteristic facial features which is geared to improving the facial profile and
• Cognitive impairment also the masticatory efficiency of the patient
• Congenital heart disease (typically a ventricular
septal defect) HEMIFACIAL HYPERTROPHY
*most common symptom of trisomy 21
• Hearing deficits (maybe due to sensory-neural
factors, or chronic serous otitis media, also known
as Glue-ear)
*Glue-ear – there is a fluid in the space behind the
eardrum, symptom = hearing difficulty
• Short stature
• Thyroid disorders • Condition which involves the enlargement of half of
• Alzheimer’s disease the head with enlarged teeth on the involved side
• Other less common serious illnesses include • Types:
leukemia, immune deficiencies, and epilepsy o Segmental
o Simple
Oral features: o Complex
• Fissured tongue often exhibit macroglossia
*macroglossia = large tongue FRAGILE X SYNDROME
• Small oral cavity • Mutation of FMR1 gene, aka. Fragile X Mental
• Open mouth posture Retardation 1
• Protruding tongue and habitual mouth breathing • Fragile X Syndrome (a condition primarily affecting
males that causes learning disabilities and cognitive
• Decreased palatal width and length
impairment)
• Bifid uvula and cleft lip and palate are occasionally
• The most common cause of inherited mental
observed
impairment
Treatment and Prognosis • This impairment can range from learning disabilities
to more severe cognitive or intellectual disabilities.
• Infants with significant congenital heart disease
(sometimes referred to as mental retardation)
have poor prognosis
• FXS is the most common known cause of autism or
• Regular ophthalmogic and audiologic follow-ups
“autistic-like” behaviors. Symptoms also can
• Prevention of dental caries and periodontal disease
include characteristic physical and behavioral
features and delays in speech and language
HEMIFACIAL ATROPHY
development
Features:
• This disorder is not so much observed physically but
• Progressive unilateral atrophy
seen more on cognitive part of the patient
 Disease HPV Type
Common warts 2, 7
Plantar warts 1, 2, 4
Flat warts 3, 10
Anogenital warts 6, 11, 42, 43, 44, 55 and
others
Genital cancers 16, 18, 31, 33, 35, 39, 45,
51, 52, 56, 58, 59, 68, 73,
82
Epidermodysplasia More than 15 types
verruciformis
Focal epithelial 13, 32
hyperplasia (oral)
ORAL PAPILLOMAS 6, 7, 11, 16, 32, 18

Oral Manifestations
• Several types of HPV, particularly type 16, have
Oral Manifestations: been found to be associated with oropharyngeal
• Prognathism squamous-cell carcinoma, a form of head and neck
• high-arched palate cancer
• prominent lateral palatine ridges
• anterior and posterior dental cross-bites
• increased occlusal attrition
• Other findings:
o cleft palate
o associated with Pierre Robin syndrome

11 17 2022 – ONCO Clinical Features:

SEXUALLY TRANSMITTED DISEASES • Oral squamous papillomas may be found on the
• HPV (Human Papillomavirus) vermillion portion of the lips, hard and soft palate
and uvula
• Acquired Immune Deficiency Syndrom (Kaposis
Sarcoma) • Lesion measures less than 1 cm in greatest
dimension, appears pink to white exophytic
granular or cauliflower-like surface alteration
• Solitary and generally asymptomatic

1. HPV (HUMAN PAPILLOMAVIRUS)

• Sexually transmitted HPVs also cause a major *wartlike appearance
fraction of of anal cancers and approximately 25% *Prevention is Sex abstinence
of cancers of the mouth and upper throat (known as
the oropharynx) HPV Treatments for Tissue Changes
• The latter commonly present in the tonsil area and • Watch and wait. Sometimes the cell changes –
HPV is linked to the increase in oral cancers in non- called cervical dysplasia, precancerous cell changes,
smokers or cervical intraepithelial neoplasia – will heal on
• Engaging in anal sex or oral sex with an HPV- their own
infected partner may increase the risk of developing *dysplasia – formation of new cell, cancerous
these types of cancers
• Cryotherapy – involves freezing the abnormal cells • People who have kidney transplants are also at risk
with liquid nitrogen for Kaposi’s sarcoma
• Conization – is a procedure, also known as cone *why? They are immunocompromised
biopsy that removes the abnormal areas • African Kaposi’s sarcoma is fairly common in young
• LEEP or Loop Electrosurgical Excision Procedure. adult males living near the equator. One form is also
The abnormal cells are removed with a painless common in young African children.
electrical current
Treatment:
2. KAPOSIS SARCOMA • Treatment decisions depend on the extent and
• Is a cancerous tumor of the connective tissue, and location of the lesions, as well as the person’s
is often associated with AIDS symptoms and degree of immunosuppression.
• Precursor of AIDS is HIV, AIDS is not cancerous, Antiviral therapy against the AIDS virus can shrink
Cancerous type is Kaposis sarcoma the lesions
*long term medication for AIDS
Characteristics: • Radiation therapy or cryotherapy can be used for
• It causes patches of abnormal tissue to grow under lesions in certain areas
the skin, in the lining of the mouth, nose, and throat • Combination chemotherapy may also be used.
or in other organs However, lesions may return after treatment
• If the cancer spreads to the digestive tract or lungs,
bleeding can result Prognosis:
• Lung tumors can make breathing hard • Treatment and remission of Kaposi’s sarcoma does
not improve the chances of survival from AIDS itself.
The outlook depends on the immune status and HIV
viral load of the patient
*you may treat kaposi sarcoma but the virus we
cant treat it, no treatment in HIV/AIDS today. We
just limit the virus

Possible Complications:
• The patches are usually red or purple and are made
• Kaposi’s sarcoma can involve the lungs and cause
of cancer cells and blood cells
significant symptoms, including cough and
shortness of breath. This diagnosis is made by a CT
scan of the chest and a bronchoscopy. The tumors
can return even after apparently successful
treatment
• Kaposi’s sarcoma can be fatal for a person with AIDS
• An aggressive form of African Kaposi’s sarcoma can
spread quickly to the bones
• Another form found in African children does not
affect the skin. Instead, it spreads through the
lymph nodes and vital organs, and can quickly
become fatal

• The red and purple patches often cause no Prevention:

symptoms, though they may be painful • Safe sexual practices can prevent infection with HIV,
which in turn prevents the development of AIDS
Cause: and its complications, including Kaposi’s sarcoma
• In people with AIDS, Kaposi’s sarcoma is caused by
an interaction between HIV, a weakend immune
system, and the human herpesvirus-8 (HHV-8).
Occurrence of Kaposi’s sarcoma has been linked to
the spread of HIV and HHV-8 through sexual activity
*AIDS is severe form of HIV
 CONGENITAL AND DEFORMITY DISEASES
1. MELANOMA

*abnormal increase in the production of melanin

• Is a malignant tumor of melanocytes which are
found predominantly in skin but also in the bowel
and the eye
• It is due to uncontrolled growth of pigment cells,
called melanocytes
• Melanoma is the most serious type of skin cancer.
Often the first sign of melanoma is a a change in the
size, shape, color, or feel of a mole
• Most melanomas have a black or black-blue area.
Melanoma may also appear as a new mole. It may *red pigmented color
be black, abnormal or “ugly looking”

A popular method for remembering the signs and

symptoms of melanoma is the mnemonic “ABCD”
• Asymmetrical skin lesion
• Border of the lesion is irregular
• Color – melanomas usually have multiple colors
• Diameter – moles greater than 6 mm are more
likely to be melanomas than smaller moles

Intraoal Lesion
• Intraoral melanomas are usually darkish brown to
black in color, but melanotic lesions have also been
reported. Most oral melanomas present as solitary
lesions, however, multiple or synchronous lesions
have also been reported

*Evolving – changing size, shape or color • Malignant melanoma of the oral cavity is a rare
condition, accounting for about 1-2% of all
Clinical Feature: melanomas
• Usually have multiple colors
• Oral melanomas have extremely poor prognosis. o sometimes with mucosal ulceration or
Therefore, pigmented lesions of undetermined erosion of underlying bone
origin should be routinely biopsied
HISTOPATHOLOGIC FEATURES
Prevention • Characterized by large binucleated cells called
• Minimizing exposure to sources of ultraviolet Reed-Sternberg cells and a lymphoid stroma
radiation (sun and sunbeds) composed of large numbers of non-neoplastic cells
• Following the sun protection measures
• Wearing sun protective clothing (long-sleeved WHO CLASSIFICATION OF HL
shirts, long trousers, and broad-brimmed hats) that (1) lymphocyte-rich = classic type
can offer protection o most favorable prognosis
(2) nodular sclerosis
Treatment: o most common form of HL
• Surgery is the first choice therapy for localized (3) mixed cellularity
cutaneous melanoma o combination of lymphocytes, eosinophils,
neutrophils, plasma cells, and macrophages,
and many Reed-Sternberg cells
I. ANGIOSARCOMA o intermediate prognosis between nodular
• Rare neoplasm of endothelial cell origin sclerosing type and the lymphocyte
• Unknown cause depletion form
• May arise from either blood or lymphatic vessels (4) lymphocyte depletion
• Scalp – usual location o worst prognosis
• Maxillary sinus and oral cavity – occasional
• Unencapsulated proliferation of anaplastic TREATMENT
endothelial cells enclosing irregular luminal spaces • external radiation therapy andor multiple-agent
chemotherapy.

III. NON-HODGKIN’S LYMPHOMA (NHL)

• more common than HL
• associated with HIV patients that often occur in
extranodal head and neck sites
• can arise in lymph nodes or extranodal lymph nodes
o most common site extranodal – GIT
o 2nd common site – head and neck, most
• Aggressive clinical course cases arise in Waldeyer’s ring
• Radical surgical excision or radiation therapy or • B-Cell lymphomas are the most common phenotype
both in extranodal sites
• Poor prognosis
MODIFIED WHO CLASSIFICATION OF LYMPHOMAS
II. HODGKIN’S LYMPHOMA (HL) B-Cell Neoplasms T-Cell and
• Unknown but may be linked to Epstein-Barr virus Postulated NK-
infection Cell Neoplasms
• Disease of lymph nodes Precursor Precursor B- Precursor T-
• Very rare in oral cavity cell lymphoblastic lymphoblastic
neoplasms lymphoma/leukemia lymphoma/leukemia
CLINICAL FEATURES Peripheral B-cell chronic T-cell chronic
• painless enlargement of lymph nodes or extranodal (mature) lymphocytic lymphocytic
lymphoid tissue cell leukemia/small leukemia
neoplasms lymphocytic
• ORAL MANIFESTATIONS:
lymphoma (B-
o tonsillar enlargement – unilateral
CLL/SLL)
• Extranodal sites:
o submucosal swellings Lymphoplasmacytoid
lymphoma
 Large granular Mantle cell Peripheral
lymphocytic lymphoma T-cell
leukemia (T-cell or lymphoma
Mantle cell NK-cell type) Age Adults Any Children,
lymphoma young
Mycosis fungoides adults
Marginal zone B-cell Stage at High (.80% Any High
lymphoma Peripheral T-cell presentation stages III
(extranodal or lymphoma, and IV)
nodal) unspecified Tumor Slow; Fast Very fast;
growth rate proliferative proliferative
Splenic marginal fraction is fraction
zone B-cell Angioimmunoblastic low .95%
lymphoma lymphoma Bone Yes Uncommon Common
marrow
involvement
Hairy cell leukemia Natural Indolent, Patient Patient
Intestinal T-cell history if usually death in 1-2 death in
lymphoma untreated takes years years weeks to
Plasmacytoma to kill months
Adult T-cell patient
lymphoma/leukemia Response to Poor Responsive Very
Diffuse large B-cell treatment responsive
lymphoma Anaplastic large cell
lymphoma
Burkitt’s lymphoma

CHARACTERISTIC CYTOGENETIC FINDINGS IN

SELECTED, SPECIFIC LYMPHOMAS
Lymphoma Translocation Oncogene or
Type Tumor
Suppressor
Genes
Follicular t(14;18) Bcl-2
lymphoma
Extranodal t(11;18) AP12, MLT
marginal zone t(1;14) Bcl-10
Mantle cell t(11;14) Bcl-1 (cyclin D1)
lymphoma

Burkitt’s t(8;14) c-Myc

lymphoma t(8;22)
t(2;8)

Anaplastic large t(2;5) NPM, ALK

cell
lymphoma

COMPARISON OF CLINICAL FEATURES

Indolent Aggressive Highly
Aggressive
Examples of Follicular Diffuse B- Burkitt’s
types lymphoma cell lymphoma
B-CLL/SLL lymphoma
 C. FOLLICULAR B-CELL LYMPHOMAS
• follicular center B lymphocytes
• accounts for 22% to 40% of all NHLs in whites but
only 5% to 10% of NHLs in Asians
• older adults
• slowly growing, painless enlargement
• rare in oral cavity
• protracted clinical course
• incurable
• HISTOPATHOLOGY:
o follicular center–like cells
o including small and large cleaved cells
o large noncleaved cells occasionally
DIFFERENT KINDS OF NHL
D. EXTRANODAL MARGINAL ZONE B-CELL
A. SPECIFIC LYMPHOMAS
LYMPHOMA
• 2 histomorphologic groups:
• Previously known as lymphoma of MALT (mucosa-
o Diffuse form
associated lymphoid tissue)
▪ abnormal cells are distributed
• Indolent – little or no pain
uniformly
o Follicular (nodular) lymphomas • Sites:
o Mucosa
▪ malignant cells arranged in
o extranodal tissues
uniformly sized nodules distributed
o GIT
throughout a lymph node or
o Salivary glands
extranodal site
o Lung
▪ more favorable prognosis
o thyroid gland
• normal architecture of the lymphoid tissue is
o skin
destroyed
• Any age can be affected
B. DIFFUSE B-CELL LYMPHOMA (DLBCL) • Some settings - associated with Sjögren’s syndrome
– female predominance
• aggressive, rapidly growing neoplasm
• Predisposing Factors:
• arises de novo
o Hashimoto’s thyroiditis
• occur over a wide age range
o Sjögren’s syndrome
• slight male predilection
o Helicobacter pylori gastritis
• lymphadenopathy or in extranodal sites
o Borrelia burgdorferi skin infection (Lyme
• HISTOPATHOLOGY: disease)
o sheets of large lymphoid cells showing
• HISTOPATHOLOGY:
abundant cytoplasm and nuclei
o unifocal or multifocal involvement
o some are associated with EBV infection
o composed of centrocyte-like (CCL)
• TREATMENT:
o Most cases - local-regional therapy
o Excellent prognosis
o 5-year survival on the order of 75%
E. MANTLE CELL LYMPHOMA o abdominal mass
• inappropriate overexpression of cyclin D1 protein o Jaw lesions less common than endemic BL –
• occurs in middle-aged or older adults 10%
• male predominance o Most patients are are adults with marked
• presents as lymphadenopathy, but extranodal immunosuppression
disease including that in the spleen and o Chemotherapy - 5-year survival rate is
gastrointestinal tract greater than 75% for stages I to III, but only
• clinical course is progressive 25% for stage IV disease
• poor outcome • AIDS associated BL
• HISTPATHOLOGY: o Poor prognosis
o diffuse, vaguely nodular or nodular pattern • HISTOPATHOLOGY:
of lymphocytes o monotonous sheets of densely packed,
o Cells are monotonous and small with medium medium-sized neoplastic
indented or angulate nuclei lymphocytes
o Pleomorphic or blastoid variants are o very high mitotic rate
recognized and pursue an even more o classic starry sky appearance
aggressive clinical course o translocation of the c-myc gene on
chromosome 8 with one of the
F. B-Cell Chronic Lymphocytic Leukemia/Small immunoglobulin genes on chromosome 2,
Lymphocytic Lymphoma 14, or 22 define the disease
• Indolent and slow progression
• Most cases – leukemic presentation
• rarely localized
• affects older patients
• Bone marrow involvement is common
• some develop autoimmune hemolytic anemia
• HISTOPATHOLOGY:
o effacement of lymph nodes by small
lymphocytes with small, rounded nuclei,
H. LYMPHOMAS ASSOCIATED WITH HUMAN
condensed chromatin, inconspicuous
IMMUNODEFICIENCY VIRUS INFECTION
nucleoli, and little cytoplasm
• rare complication
• TREATMENT:
• HISTOPATHOLOGY:
o single-agent chemotherapy
o immunoblastic lymphoproliferations and
plasmablastic lymphoma
G. BURKITT’S LYMPHOMA (BL)
o occurs primarily - marked depression of CD4
• highly aggressive B-cell lymphoma
T cells
• afflicts children and adolescents
• Sites
• 3 Forms:
o Most common - gingiva, palate, and fauces
o endemic type – africa
• In AIDS, B-cell lymphomas predominate
o sporadic form - North America
• plasmablastic, immunoblastic, or Burkitt’s-like
o form associated with immunodeficiency –
lymphoma.
europe
• Plasmablastic
• ENDEMIC BL
o aggressive B-cell
o children
o occurs in the setting of HIV infection
o endemic malaria - pathogenetic cofactor
o predilection for the oral cavity
o 95% is associated with EBV infection
o EBV and human herpesvirus 8 (HHV8) have
o Jaw involvement – 50%
been implicated in the development
o Chemotherapy - 5-year survival rate is
o Poor prognosis
greater than 75% for stages I to III, but only
25% for stage IV disease
I. ANAPLASTIC LARGE CELL LYMPHOMA (ALCL)
• SPORADIC BL
• aggressive lymphoma of T-cell or natural killer (NK)-
o young adults
cell lineage
• characteristically expresses the CD30 (Ki-1 or Ber- o resembles traumatic ulcerative granuloma
H2) antigen of the oral mucosa
• cytogenetic abnormality t(2;5) • TREATMENT:
• bimodal age distribution o simple excision
• affects adolescents and older adults
• males are more affected IV. LEUKEMIA
• HISTOPATHOLOGY: • neoplastic proliferation of bone marrow lymphocyte
o different patterns: or myeloid precursors that replace the marrow
o prototypic form • genetic factors:
▪ large or very large cells o chromosome translocations (t[9;22] in chronic
▪ rounded or horseshoe shaped myeloid leukemia
single or multiple nuclei • Environmental factors:
o Occasionally, nuclei are arranged in a o Benzene
wreathlike pattern o ionizing radiation
• TREATMENT: • Virus factors:
o single- or multiple-agent chemotherapy – 5 o human T-cell lymphotrophic virus type 1
(HTLV-1) in adult T-cell leukemia
year median survival rate of 77%.
• ACUTE LEUKEMIA
o immature cells and a fulminant (severe and
J. NATURAL KILLER/T-CELL LYMPHOMA, NASAL TYPE
sudden) clinical course
• Progressive, ulcerative destruction of the palate,
o bleeding due to thrombocytopenia
nose, and paranasal structures o fatigue due to anemia
• midline lethal granuloma o infection due to agranulocytosis
• Nasofacial NK/T-cell lymphoma (a) ACUTE LYMPHOCYTIC LEUKEMIA (ALL)
o Aggressive a. Children
o Adults b. Between 60% and 90% of patients -
o Men are more affected achieve remission
o Nasal symptoms are the most common c. TREATMENT:
presenting feature with epistaxis i. Medicine
occassionally (b) ACUTE MYELOID LEUKEMIA (AML)
o Some may present swelling of the soft or a. Adults
hard palate b. 80% of patients – complete remission
o Over time, it evolves to frank ulceration and c. TREATMENT:
destruction of the palatal and nasal tissues i. aggressive chemotherapy
leading to - oronasal fistula • CHRONIC LEUKEMIA
• HISTOPATHOLOGY: o better-differentiated, mature cells
o varying amounts of granulation tissue and o more indolent clinical course
necrosis (a) CHRONIC MYELOID LEUKEMIA (CML)
o mixture of acute and chronic inflammatory a. adult
cells intermingled with atypical lymphocytes b. highest in the fourth and fifth decades of
o Angiocentricity and epithelio-tropism life
• TREATMENT: c. rare in children
o chemotherapy d. asymptomatic
o radiation therapy e. some may have - fatigue, weight loss,
o or a combination of both fever, and night sweats
f. splenomegaly may also occur
g. generalized gingival hypertrophy
K. EOSINOPHIL-RICH CD30-POSITIVE i. red, boggy, and atous, and it
LYMPHOPROLIFERATIVE DISORDER bleeds easily
• Solitary ulcer – tongue ii. initial presenting feature of CML
iii. due to infiltration by neoplastic
• HISTOPATHOLOGY:
myeloid cells
o stromal cellular infiltrate
o large cells express CD3 and CD30 antigens
 • Poor prognosis
• TREATMENT:
o Chemotherapy

VI. NEOPLASMS OF THE SALIVARY GLANDS

A. BENIGN NEOPLASMS
1. MIXED TUMOR (PLEOMORPHIC ADENOMA)
• Histogenesis:
o relates to dual proliferation and comingling
h. Difficult to cure of cells with ductal or myoepithelial
i. Chemotherapy - typically with features in a stroma of mucoid, myxoid, and
hydroxyurea or busulfan less commonly, chondroid quality
(b) CHRONIC LYMPHOCYTIC LEUKEMIA (CLL) CLINICAL FEATURES:
a. adult • most common tumor of the major and minor
b. occurs more commonly than other types salivary glands
of leukemia • Sites:
c. seventh decade of life o Parotid Glands – 85%
d. asymptomatic o submandibular gland – 8%
e. as the disease progresses - o intraoral minor salivary glands – 7%
lymphadenopathy, splenomegaly, and • Any age
hepatomegaly may occur • Favor males more than females
f. Difficult to cure • Mobile, except when they occur in the hard palate
g. often is not treated if the patient is
• Firm, painless swellings but not cause ulceration of
elderly or asymptomatic
the overlying mucosa
h. Symptomatic CLL patients and those with
• Most common intraoral site - PALATE > UPPER LIP >
extensive disease - alkylating
chemotherapy, although cure is unlikely BUCCAL MUCOSA

V. GRANULOCYTIC SARCOMA
• A.k.a extramedullary myeloid tumor
• CLINICAL FEATURES:
o localized soft tissue mass
o less frequently intraosseous presentation
o 3 Settings:
▪ patient previously known to have
AML
▪ sign of blast transformation in a HISTOPATHOLOGY
patient with CML or chronic • Encapsulated; variable glandular patterns; epithelial
myeloproliferative disorder and myoepithelial differentiation; no mitoses
▪ patient who was previously healthy • plasmacytoid cells or spindled cells
• HISTOPATHOLOGY: • Positive for S-100 protein, calponin, p63, and alpha-
o large cell lymphoma, poorly differentiated smooth muscle actin
carcinoma or plasmacytoma • positive for several cytokeratins - 3, 6, 10, 11, 13,
o Crystalline, rodlike, intracytoplasmic and 16
acidophilic bodies (Auer rods) • positive for keratin 7 but negative for keratin 20
TREATMENT AND PROGNOSIS
• surgical excision
• occasional recurrence in major glands

2. BASAL CELL ADENOMA (BCA)

• group of benign salivary neoplasms of histologic
uniformity
• constitute 1% to 2% of all salivary gland adenomas
• Sites: o Membranous form
o Major - Parotid gland – 70% > ▪ nodular fashion with variably sized
submandibular gland islands of tumor tissue surrounded
o Minor – Upper lip > palate > buccal mucosa by a thick periodic acid–Schiff (PAS)-
> lower lip positive hyaline membrane
CLINICAL FEATURES: ▪ predominantly in the parotid gland
• slow growing, solitary, painless masses (>90% of cases)
• clinically distinct and firm on palpation, but can be
multifocal and multinodular TREATMENT AND PROGNOSIS
• Patients - between 35 and 80 years, mean age 60 • benign and rarely recur – except for membranous
yrs form
• male predilection • membranous form - significant rate of recurrence
• conservative surgical excision including a margin of
HISTOPATHOLOGY: normal uninvolved tissue
• FOUR SUBSETS:
o Solid form 3. CANALICULAR ADENOMA
▪ islands or sheets of isomorphic • Different from BCA, because it occurs exclusively
basaloid cells often show peripheral within oral cavity
palisading • 6% of all minor salivary gland neoplasms

CLINICAL FEATURES:
• older than 50 years of age
• women predilection
• Most common site - Upper lip
• freely movable, asymptomatic, range in size

HISTOPATHOLOGY
o Trabecular form • bilayered strands of basaloid cells that branch and
▪ thin trabeculae and cords of anastomose within a delicate stroma that is highly
epithelial cells separated by a vascular and contains few fibroblasts and little
delicately vascularized stroma collagen
• occasionally may not be totally encapsulated
• 20% of cases are multifocal

o Tubular form
▪ ductal structures as the dominant
feature with lining cells of cuboidal
TREATMENT AND PROGNOSIS
type surrounded by single or
• surgical excision
multiple layers of basaloid cells
• may recur
 4. MYOEPITHELIOMA ii. WHARTHIN’S TUMOR (PAPILLARY CYSTADENOMA
• composed entirely of myoepithelial cells LYMPHOMATOSUM)
• immunohistochemical staining - antibodies to p63, • arise within lymph nodes as a result of entrapment
actins, cytokeratin, and S-100 protein of salivary gland elements early in development.
• most common salivary gland tumor to occur
CLINICAL FEATURES bilaterally
• Mostly within parotid gland > intraoral minor and to be synchronously associated with other
salivary glands > submandibular gland salivary tumors
• third through ninth decades
• equal to genders CLINICAL FEATURES:
• 7% of epithelial neoplasms of salivary glands
HISTOPATHOLOGY • majority occurring within the parotid gland
• circumscribed painless masses • rare intraorally
• composed of plasmacytoid cells or spindle cells in • predominant in men
varying proportions • fifth and eighth decades of life
• 70% spindle cells • strong positive association between the
• 20% plasmacytoid cells development of Warthin’s tumor and cigarette
smoking
TREATMENT AND PROGNOSIS • doughy to cystic mass
• Conservative excision - minor salivary glands
• Superficial parotidectomy - parotid gland
• Prognosis is excellent, no recurrences

5. ONCOCYTIC TUMORS
i. ONCOCYTOMA (OXYPHILIC ADENOMA)
• oncocytes - large granular acidophilic cells filled
with mitochondria
• HISTOGENETIC SOURCE:
o salivary duct epithelium, in particular the
striated duct

CLINICAL FEATURES:
• predominantly in the parotid gland HISTOPATHOLOGY:
• solid, ovoid encapsulated • encapsulated, smooth to lobulated surface and a
• rare – intraoral round outline
• growth rate is slow, and the course is benign • numerous cystic spaces of irregular outline contain
papillary projections lined by columnar eosinophilic
HISTOPATHOLOGY cells (oncocytes)
• non-neoplastic and multicentric cellular change –
oncocytosis
• oncocytoma cells are polyhedral with granular
eosinophilic cytoplasm
• histochemical stain - phosphotungstic acid
hematoxylin (PTAH)
o highlighting the intracytoplasmic
mitochondria

TREATMENT AND PROGNOSIS

• conservative, superficial parotidectomy – parotid TREATMENT AND PROGNOSIS
gland • Radiotherapy
• surgical excision – minor salivary glands • May recur but believed to represent second primary
• recurrece is rare lesions
iii. SEBACEOUS ADENOMA TREATMENT AND PROGNOSIS:
• originate in intralobular ducts • simple excision
• rare – less than 0.5% of all salivary gland adenomas • little risk of recurrence
• predominantly of sebaceous gland–derived cells
• well differentiated – benign iii. INTRADUCTAL PAPILLOMA (IP)
• moderately to poorly differentiated – malignant CLINICAL FEATURES:
• antibody to adipophilin - useful to identify • arises from a greater depth within the ductal system
sebocytes and sebaceous lesions • presents a salivary obstruction caused by
• Primary site – parotid gland intraluminal exophytic growth
o May be seen intraorally in buccal mucosa
and RMP HISTOPATHOLOGY:
• a single or double layer of cuboidal to columnar
TREATMENT • covers several papillary fronds
• Parotidectomy – parotid gland • no proliferation in the wall of the cyst
• Surgical excision - intraoral neoplasms
TREATMENT AND PROGNOSIS
6. DUCTAL PAPILLOMA • same with IDP
• arise within the interlobular and excretory duct
portions of the salivary gland B. MALIGNANT NEOPLASMS
1. MUCOEPIDERMOID CARCINOMA
i. SIALADENOMA PAPILLIFERUM • most common salivary gland malignancy
CLINICAL FEATURES: • exhibits biological behaviors that range from
• Sites – buccal mucosa and palate relatively indolent (low grade) to clinically ag-
• painless exophytic papillary lesion of the mucosa gressive (high grade)
and salivary duct epithelium • are epithelial mucin-producing tumors
• age – 5th to 8th decades, mainly male • arise from reserve cells in the interlobular and
• clinical impression – simple papilloma intralobular segments of the salivary duct system

HISTOPATHOLOGY: CLINICAL FEATURES:

• lined by a layer of epithelium two to three cells • most common site - parotid gland (60 – 90%)
thick • most common salivary gland malignancy of
• supported by a core of fibrovascular CT childhood
• superficial - squamous epithelial lining • 34% - parotid, 20% - submandibular gland, 30% -
• deep - cuboidal to columnar minor salivary gland
• late stage - mucous membrane becomes papillary to • third through fifth decades of life
verrucous • equal gender predilection
TREATMENT AND PROGNOSIS • LOW GRADE:
• conservative surgery o prolonged period of painless enlargement
• little chance of recurrence o oral cavity – this lesion often resembles an
extravasation or retention-type mucocele
ii. INVERTED DUCTAL PAPILLOMA (IDP) • HIGH GRADE:
CLINICAL FEATURES: o grow rapidly, often accompanied by pain
• nodular submucosal mass that resembles fibroma or and mucosal ulceration
lipoma o major salivary glands - may present with
• equal gender distribution evidence of facial nerve involvement
• age – adults

HISTOPATHOLOGY:
• marked proliferation of ductal epithelium
• Crypts and cyst-like spaces lined by columnar cells
with polarized nuclei
• endophytic growth pattern
HISTOPATHOLOGY:
• lobular infiltration of adjacent tissue, often well TREATMENT AND PROGNOSIS
circumscribed • LOW GRADE:
• LOW GRADE: o Excellent prognosis (>95%)
o cuboidal to columnar mucus- mucus- o Surgery
secreting cells arranged around microcystic o Neck dissection is rare
structures • HIGH GRADE:
o with an intermingling of epithelial, or o Fair prognosis (<40%)
“intermediate,” cells with a few epidermoid o surgery plus postoperative radiotherapy to
cells the primary site
o are PAS and mucicarmine positive o Neck dissection is usually require this tx
o margin of low-grade tumors – the pattern is
often one of broad “pushing” fronts 2. POLYMORPHOUS LOW-GRADE ADENOCARCINOMA
• Second most common malignant salivary gland
tumor
• putative source is believed to be reserve cells in the
most proximal portion of the salivary duct
• relatively indolent course and lower risk of
recurrence and metastasis compared with adenoid
cystic carcinoma
• HIGH GRADE: CLINICAL FEATURES:
o composed chiefly of epidermoid cells that • fifth through eighth decades of life; no gender
are more solid, with fewer mucin-containing predilection
cystic spaces and scattered mucous cells • accounts for 26% of all salivary carcinomas
o Cellular pleomorphism, nuclear • appears almost exclusively in minor salivary glands
hyperchromatism, and mitotic figures are • most frequently reported site – PALATE
noted • asymptomatic submucosal mass
• most cases show small nerve invasion but no effect
on prognosis
• Firm, elevated, nonulcerated nodular swellings
(nontender)
• Size – between 1 to 4 cm in diameter
• Painless mass that may have been present for a
long time with slow growth rate
• INTERMEDIATE GRADE: o Associated with bleeding, discomfort,
o Mucous cells and microcystic spaces are telangiectasia, ulceration occasionally
apparent but are not as numerous as in • Tumor can erode or infiltrate the underlying bone
low-grade lesions
• TRANSLOCATION
o t(11;19)(q14-21;p12-13)
HISTOPATHOLOGY: • infiltrative, slow-growing unilobular mass although
• Well circumscribed but unencapsulated with with occasional pain or tenderness
infiltrating streams of cells and a general lobular • Adults; palatal mass/ulceration
morphology • Late stage lesion
• Homogeneous population of cells with prominent, o Pain
bland, uniform, and often-vesicular nuclei o facial nerve weakness
surrounded by minimal cytoplasm o paralysis
• Wide range of histomorphologic patterns between • Distant spread to the lungs is more common than
and within tumors metastasis to regional lymph nodes
• Cells exhibit different growth patterns, hence the
term polymorphous HISTOPATHOLOGY:
o Patterns can be: • 3 basic histomorphologic patterns
▪ Solid -*solid pattern that resembles o Cribriform
adenoid cystic carcinoma ▪ best-recognized pattern
▪ Ductal ▪ prototypical one that typifies the
▪ Cystic tumor
▪ Tubular - *tubular pattern that ▪ pseudocystic spaces contain
resembles also adenoid cystic sulfated mucopolysaccharides
carcinoma o Tubular
▪ Cribriform - *again resembles ▪ composed of smaller islands of cells
adenoid cystic carcinoma with distinct duct-like structures
• Tumor stroma can be of mucoid quality or centrally
demonstrate hyalinization o Solid
• Perineural invasion is common, invading the ▪ shows little duct formation
adjacent tissue in a single-file fashion ▪ composed of larger islands of small
• Mitotic figure is uncommon to medium-sized cells with small,
• Immunohistochemical staining (*to distinguish from dark nuclei
other salivary gland tumors) o show more pleomorphism than the others
o shows a p63+/p40 - immunophenotype in o associated with a poorer outcome
contrast to both adenoid cystic carcinoma • Spread through perineural spaces
and cellular pleomorphic adenoma that
shows a concordant/consistent p63+/p40+ TREATMENT AND PROGNOSIS:
(most common) • Local recurrence and metastasis; lung > nodes
• 5-year survival 70%; 15-year survival 10%
TREATMENT AND PROGNOSIS • Surgery
• Low-grade malignancy - good prognosis • If parotid gland is involved – wide resection in the
• surgical excision form of a superficial parotidectomy or superficial
• recurrence rate <10% and deep lobectomy
• Occasional metastasis • Intraorally - wide excision often with removal of
underlying bone
3. ADENOID CYSTIC CARCINOMA • Radiation therapy - postoperative modality and
CLINICAL FEATURES: locally recurrent disease
• accounts for approximately 23% of all salivary gland
carcinomas 4. CLEAR CELL CARCINOMA
• 50% to 70% of all reported cases – occur in minor • A.k.a hyalinizing clear cell carcinoma
salivary glands of the head and neck, chiefly of the • low-grade tumor
palate • occurs predominantly in the minor salivary glands
• In major salivary gland – parotid is more affected • Most present as submucosal masses in the palate
• fifth through seventh decades of life
• no gender predilection
• High-grade salivary gland malignancy
 • exhibits a solid microscopic pattern; although one
third of lesions have a microcystic pattern
• demonstrate clear cell element zones, probably as a
result of inadequate fixation

TREATMENT AND PROGNOSIS:

• Surgery
• seldom metastasize but have tendency to recur
• 89% at 5 years and 56% at 20 years
HISTOPATHOLOGY:
• composed of uniform bland cells, predominantly
C. RARE TUMORS
with clear cytoplasm
1. CARCINOSARCOMA
• typically trabecular, although nests and sheets of
• Carcinoma ex-mixed tumor
cells may be seen
o arises from an untreated benign mixed
• positive for glycogen, but negative for mucin, S-100
tumor known to be present for several
protein, and muscle-specific actin
years, or from a benign mixed tumor that
• demonstrates a consistent EWSR1-ATF1 fusion
has had many recurrences over many years
o 68% are found in parotid
o 18% in minor salivary glands
o SYMPTOMS - fixation of the mass to
surrounding tissues ulceration, and regional
lymphadenopathy
o TREATMENT:
▪ Surgery
▪ radical neck dissection – if there is
cervical lymph node involvement
TREATMENT AND PROGNOSIS: • CARCINOSARCOMA - both epithelial and
• excision mesenchymal components are malignant
• recurrence is very uncommon • Metastasizing mixed tumor is characterized by a
histologically benign mixed tumor that for some
5. ACINIC CELL CARCINOMA reason metastasizes while still retaining its bland,
• occurs predominantly in the major salivary glands - benign histologic appearance.
especially the parotid
• putative source is the intercalated duct reserve cell

CLINICAL FEATURES:
• found in all age groups
• No gender predilection
• accounts for 14% of all parotid gland tumors and 9%
of the total of salivary gland carcinomas of all sites
• Unusual feature - frequency of bilateral parotid
gland involvement in approximately 3% of cases PROGNOSIS:
• Site • Local recurrence – ½ of PX with primary parotid
o Parotid gland – 80% neoplasms and ¾ of px with submandibular and
o submandibular gland – 4% minor salivary gland tumors
o minor salivary glands – 17% • 10% of cases present with uncontrollable lymphatic
• Within oral cavity disease – 1/3 of these show metastasis to distant
o Most cases – palate and buccal mucosa sites in lung and bone
• slow-growing lesion • Cure rates at 5, 10, and 15 years after treatment in
• pain is a common presenting symptom one study were 40%, 24%, and 19%, respectively; in
another study, 30% of those monitored for 10 years
HISTOPATHOLOGY: were free of disease.
• intraglandular mass, well circumscribed
2. EPIMYOEPITHELIAL CARCINOMA 4. BASAL CELL ADENOCARCINOMA (BCA)
• is a clear cell–containing malignancy of salivary • malignant counterpart of basal cell adenoma
gland (predominantly the major glands) • low-grade, minimally invasive malignancy
• origin characterized with a biphasic morphology
• seventh and eighth decades of life HISTOPATHOLOGY:
• 2:1 female predilection • histologic resemblance to ductal carcinoma of the
• intermediate grade breast
• composed of nests, cords, and solid zones of
HISTOPATHOLOGY: basaloid cells
• lobular growth pattern • 2 cytologic types of cells:
• Glycogen, actins, and S-100 protein are present in o small, compact cells and larger, polygonal
the bordering clear cells, supporting their myo- cells
epithelial origin o former may often be seen surrounding the
latter, frequently in a palisade fashion
• DISTINCT FEATURE of BCA from basal cell
adenoma:
o exhibits an infiltrative growth pattern and
has the ability to metastasize
o finding of small nests of neoplasm in
adjacent normal structures
• Infiltration of nerves

PROGNOSIS:
• Recurrences have most often been associated with
lesions larger than 3 cm

3. SALIVARY DUCT CARCINOMA

• high-grade malignancy of the major salivary glands
• distinct predominance in the parotid gland (more
than 80% of cases) and submandibular gland
accounts for the remainder of cases
• 80% of cases are men TREATMENT AND PROGNOSIS:
• Peak is seventh decade • Local recurrence and distant metastasis
• firm, painless mass

HISTOPATHOLOGY:
• resembles to ductal carcinoma originating in the
breast
• Architectural features:
o papillary cribriform pattern
o solid growth pattern
o desmoplastic stroma
o central or comedo-type necrosis
• Expression of androgen receptor (AR)

TREATMENT AND PROGNOSIS:

• Surgical excision
• More than 50% of patients die of their disease
within 5 months to 6 years after treatment
• Pulmonary and osseous metastases