GLORIEASTER B.
SABANDO DDM5A
I. BENIGN TUMORS OF MUSCLE TISSUE ORIGIN
1. LEIOMYOMA
- The leiomyoma is a benign tumor
- derived from smooth muscle
- is found in a variety of anatomic sites: skin;
subcutaneous tissues; oral cavity
- occur in the uterus: the so-called fibroids
- nearly identical in every site.
- Clinically, they are soft tissue tumors that
present with pain
- They are uncommon in the oral cavity
probably because of the general absence of
smooth muscle there except in blood vessel
walls and, occasionally, in the circumvallate
papillae of the tongue.
 Clinical features:
- majority of cases have occurred on : posterior
portion of the tongue, palate, cheeks, gingiva,
lips and salivary glands
- predilections:
- majority of oral leiomyomas occur in
adults in the middle decades of life
- 65% being found in patients older
than 30 years of age
- cases are described in young children
in the first decade.
- The oral leiomyoma:
- is a slow-growing
- painless lesion
- superficial and often pedunculated
- Presenting symptoms:
- sore throat
- tumor in the throat
- The tumor does not ulcerate and
resembles the normal mucosa in
color and texture
ORAL PATHOLOGY II
- A central leiomyoma of the jaw is also known
to occur but is exceedingly rare
- A case in the mandible, with ultrastructural
confirmation, has been reported by Goldblatt
and Edesess, who have also reviewed the
literature on the central lesions of bone.
 Histologic Features:
- is composed of interlacing bundles of smooth
muscle fibers interspersed by varying
amounts of fibrous connective tissue.
- muscle nuclei: spindle-shaped with
blunt ends and quite
vesicular
- bundles of fibers appear to form whorls
because of their fascicular arrangement in
varying planes
- Intra cytoplasmic myofibrils are present and
can be demonstrated by phospho-tungstic
acid-hematoxylin special stain
- Masson’s trichrome stain is also commonly
used to differentiate between collagen and
smooth muscle
- Leiomyoma cells are positive for smooth
muscle markers like desmin, vimentin, actin,
myosin and alpha smooth muscle actin.
 Treatment and Prognosis:
- conservative surgical excision
- does not tend to recur or become malignant.
GLORIEASTER B. SABANDO
2. ANGIOMYOMA (Vascular Leiomyoma, Angioleiomyoma)
- Leiomyomas and angiomyomas have
commonly been treated as two forms of the
same basic lesion in the past and reported
together as an entity
- Thus, some lesions are quite vascular, being
composed of large numbers of blood vessels
of an atypical nature with disoriented smooth
muscle layers (the angiomyoma), while others
are relatively avascular
- The suggestion has even been made that
there may be a progression of lesions:
 Hemangioma: angioma with much
nonstriated muscle
 vascular leiomyoma: leiomyoma
with many vessels and solid
leiomyoma
- Thus, it has been proposed that the vascular
leiomyoma may be only a stage in the
continuous process of smooth muscle
proliferation
- Most investigators nowadays believe that the
angiomyoma probably represents a
hamartomatous malformation, while the solid
leiomyoma represents a true neoplasm and
that, therefore, these two entities should be
clearly separated
3. RHABDOMYOMA
- The term rhabdomyoma was introduced by
Zenker (1864) to indicate a benign tumour
showing skeletal muscle cell with varying
degree of differentiation and maturity
- benign neoplasm of striated muscle tissue
- consisting usually of polygonal frequently
vacuolated glycogen containing cells with a
DDM5A ORAL PATHOLOGY II
fine granular deeply acidophilic cytoplasm
resembling myofibril in cut section
- The term ‘rhabdomyoma’ was used for benign
tumor, arising from the cardiac muscle, often
associated with a hamartoma complex,
including sebaceous adenomas, tuberous
sclerosis, and hamartomas of the kidney and
other organs
- Seventy to 90% of extra cardiac
rhabdomyomas are found in the head and
neck region but it is still a rare neoplasm of
the maxillofacial region
- They are subdivided into: adult, fetal and
genital histological subtypes
- The etiology is unknown
- However, clonal balanced translocation
(reciprocal) has been found in chromosomes
15 and 17 in adult rhabdomyoma tumors of
head and neck
 Clinical Features:
- Predilections:
- The adult form of rhabdomyoma
occurs primarily in the middle-aged
in the 16–82 years old (mean age 52
years).
- There is a marked male
predominance of almost 5:1.
- The most frequent head and neck sites of
involvement are :
- the pharynx
- oral cavity
- laryngeal lesions
- Within the mouth, the oral floor is most often
affected, pharyngeal lesions occur most
frequently in the base of the tongue and the
soft palate
GLORIEASTER B. SABANDO
- Fetal rhabdomyoma:
- usually occurs in newborns and young
children
- but also reported in patients as old as
50 years of age
- also has a strong male predilection
- The most common sites are post-or,
preauricular region, or face, followed by
nasopharynx but not in the mouth.
- Both tumor types present as a nodule or
submucosal mass which can become several
centimeters in size
- Multinodular tumors have been described,
with two or more discrete nodules closely
adjacent to one another.
- Rarely, separate tumors may be found at
different anatomic sites
 Histologic Features
- composed of: large, round cells that have a
granular, eosinophilic vacuolated cytoplasm
and show irregular cross-striations
- The cytoplasm is rich in glycogen and
glycoprotein
- A fibrous stroma is, present and mitotic
activity is extremely low
- Many cases demonstrate occasional
degeneration vacuoles or clear spaces
between the tumor cells
- The fetal rhabdomyoma:
- is comprised of less mature, somewhat
pleomorphic, polygonal muscle cells
admixed with spindle-shaped cells
- is typically more cellular than the adult
type and often has a myxoid stroma
- Mitotic activity is minimal but the more
pleomorphic examples can be mistaken
for rhabdomyosarcoma
DDM5A ORAL PATHOLOGY II
- Cross-striations and crystalline
structures are more readily identified
with the phosphotungstic acid-
hematoxylin (PTAH) stain, and oil red O
staining will often reveal intracellular
lipid
- Lesional cells are immunoreactive with
myoglobin, desmin and vimentin.
Muscle specific actin, myoglobin may
show focal positivity
- Treatment and Prognosis
- Both are treated by conservative
surgical excision
- Recurrence has been reported but
is uncommon
- Malignant transformation not
reported.
4. GRANULAR CELL MYOBLASTOMA (Myoblastic myoma,
granular cell tumor, granular cell schwannoma)
- It is not clear whether or not granular cell
tumor is a true neoplasm, a developmental
anomaly, or a trauma-induced proliferation
- The original interpretation of the tumor being
of muscle origin (granular cell myoblastoma)
has been abandoned
- The basic cell of origin is now thought to be
neural, although past reports frequently
indicated an origin from striated muscle, or
less frequently an origin from histiocytes,
fibroblasts or pericytes
- The tumor is widely distributed throughout
the body, but more than half of all cases occur
in the oral cavity. The other head and neck
site likely to be involved is the larynx.
GLORIEASTER B. SABANDO
 Clinical Features:
- Predilections:
- The lesion is typically diagnosed between the
ages of 30 and 60, but it can arise at any age.
- There is no gender predilection for oral cases,
but overall almost twice as many cases are
diagnosed in women as in men
- More than one third of all granular cell tumors
occur on :
- the lingual dorsum, usually as a sessile,
painless, somewhat firm, immovable
nodule less than 1.5 cm in greatest
diameter
- Appearance: demonstrate pallor or yellowish
discoloration and typically have a smooth
surface. Other oral and pharyngeal sites of
involvement include the soft palate, uvula,
labial mucosa, oral floor and gingiva.
- As many as 15% of patients will have granular
cell tumors of multiple anatomic sites, with as
many as 50 individual lesions in one patient.
 Histologic Features:
- The granular cells are:
- large polygonal, oval or bipolar cell of
about 20–40 μ in diameter
- with abundant, fine or coarsely granular
eosinophilic cytoplasm
- a small, pale-staining or vesicular
nucleus eccentrically located in the cell
- The cell membrane is moderately
distinct
- Granular cells often occur in ribbons
- separated by fibrous septa
- The cells may also appear to be
streaming off from or metaplastically
arising from underlying muscle fibers.
DDM5A ORAL PATHOLOGY II
- Older lesions tend to become desmoplastic
with a few scattered nests of granular cells in
a densely fibrotic background.
- If malignant type :
- Granular cells demonstrating nuclear
enlargement, hyperchromatism and
pleomorphism
- with mitotic activity
- increased cellularity
- Ultrastructural studies have described the
cytoplasmic granules as autophagic vacuoles
containing cellular debris, including
mitochondria and fragmented endoplasmic
reticulum, as well as myelin
- Background stroma is minimal
- Granular cells are positive for S100 protein,
neuron-specific enolase (NSE), laminin and
myelin basic proteins
- Staining is negative for neurofilament proteins
and glial fibrillary acidic protein (GFAP)
- Granules are PAS positive and diastase
resistant
- It is particularly common for the surface of
the lesion to be covered by a layer of
stratified squamous epithelium exhibiting
remarkable pseudoepitheliomatous
hyperplasia which has been confused with
epidermoid carcinoma.
 Treatment and Prognosis:
- Conservative excision
- Recurrence is seen thus treated, even if
granular cells extend beyond the surgical
margins of the biopsy sample
- A few reported metastasizing granular cell
tumors have appeared to be histologically
benign, and for this reason, tumors that recur,
grow rapidly or reach a size greater than 5 cm
should be viewed with grave suspicion.
GLORIEASTER B. SABANDO
5. CONGENITAL EPULIS OF THE NEWBORN
- Congenital granular cell lesion or ‘congenital
epulis’ is a rare lesion of the newborn
- also known as: Neumann’s tumor
- is benign in nature, and mostly occurs as a
single tumor but rarely as multiple
- The histogenesis and natural clinical history
of the lesion remain obscure
- Even with the advent of modern
histopathological techniques, it has not been
possible to depict specific cellular features
unique to this lesion.
- The congenital epulis of the newborn bears
an unusual resemblance to the granular cell
myoblastoma and is considered by some
persons to be the same lesion
- The congenital epulis is present at birth,
- and in this regard is distinctly different from
the granular cell myoblastoma
- typical site: maxilla
- It has been suggested that a protuberant
mass of the maxilla, would be more obvious
than a lesion in the substance of the tongue,
the usual site of the granular cell tumor, and
thus would be apt to be discovered at a far
earlier age than the tongue lesion
- On this basis, it is conceivable that the two
tumors are similar in nature, although
actually the maxilla has been found to be the
most unusual site for the occurrence of a
granular cell tumor
 Etiology:
- these congenital epulides are
malformations of the dental
blastema and should be regarded as
a type of embryonal hamartoma
and not a true neoplasm
DDM5A ORAL PATHOLOGY II
- the basis for such a belief is the
presence of numerous epithelial
rests in some sections of these
tumors
- Remember, however, that such
epithelial inclusions are remnants
of the dental lamina and may be
found normally in most jaws of
infants
- Their occurrence in the
congenital epulis is more likely to
be coincidental than associated
with the development of the
lesion
- Other etiology theories include
the fibroblastic, histiocytic,
myogenic and neurogenic
 Clinical Features:
- This tumor is present at birth and is located
on the maxillary or mandibular gingiva,
although it is somewhat more common on
the maxilla than the mandible, by a ratio of
approximately 2:1
- It is usually a pedunculated lesion found in
the incisor region, apparently arising on the
crest of the alveolar ridge or process
- It may vary considerably in size from just a
few millimeters in diameter to several
centimeters.
- Predilections: 80.5% were females, 10.6%
were males and 8.9% were of unstated sex
 Histologic Features:
- The congenital epulis is histologically similar
to the granular cell tumor, although
pseudoepithelio-matous hyperplasia does
not occur in the former lesion
GLORIEASTER B. SABANDO DDM5A ORAL PATHOLOGY II
- sheets of large, closely packed cells showing
fine, granular, eosinophilic cytoplasm
comprise the tumor mass
- Neither mitoses nor cross-striations are
visible, but capillaries are numerous
- Study by means of special staining
techniques has not been highly informative

 Treatment:
- surgical excision
- little possibility of recurrence
- However the natural history of this lesion is
one of spontaneous regression and that no
treatment is required unless dictated by
feeding or respiratory problems.
GLORIEASTER B. SABANDO
II. MALIGNANT TUMORS OF MUSCLE TISSUE ORIGIN
1. LEIOMYOSARCOMA
- a malignant tumor of smooth muscle origin
- It is very rare in the oral cavity and whether it
develops through malignant transformation of a
leiomyoma or de novo is not known
- It probably arises at these sites from smooth
muscle cells, especially those found in blood vessel
walls, and from undifferentiated mesenchymal cells
 Clinical Features:
- presents as painful, lobulated, fixed mass of the
submucosal tissues
- Predilections:
- in a middle-aged or older individual
- It is exceedingly rare in children
- No gender predilection was apparent in
these cases
- Lesions are usually less than 2 cm in diameter at
diagnosis and are slow-growing
- secondary ulceration of the mucosal surface has
been reported
- common sites:
- cheek
- floor of mouth
 Histologic Features:
- is composed of:
- fascicles of interlacing spindle-shaped cells
- with abundant eosinophilic cytoplasm
- moderately large, centrally located, cigar-
shaped or blunt-ended nuclei
- often with mild atypia
- Cellularity and cellular differentiation can vary
considerably between tumors and between different
areas of the same tumor
DDM5A ORAL PATHOLOGY II
- The well-differentiated lesion shows the spindled
cells streaming or interweaving in fascicles in a
fashion similar to that seen in leiomyoma.
- Nuclear palisading may be seen in several areas of
the tumor, ischemic areas show stromal fibrosis and
hyalinization.
- The epithelioid variant, called malignant
leiomyoblastoma or epithelioid leiomyosarcoma, is
most prevalent in the gastrointestinal and
genitourinary tracts and has rarely been reported in
oral or pharyngeal locations
 Treatment and Prognosis:
- Radical surgery with adjunctive chemotherapy or
radiotherapy
- The prognosis is poor, with numerous recurrences
and distant metastases
- Overall five-year survival is approximately 35–50%.
2. RHABDOMYOSARCOMA
- the malignant tumor of striated muscle
- is a relatively uncommon tumor in the oral cavity
- it is derived from primitive mesenchyme that
retained capacity for skeletal muscle
differentiation
- The first published example of
rhabdomyosarcoma was probably a tongue lesion
reported in 1854
- There are four separate types of
rhabdomyosarcoma based on histologic
appearance, and many of the clinical features are
quite characteristic of certain of these
- The four forms of rhabdomyosarcoma are:
• Pleomorphic
• Alveolar
• Embryonal
• Botryoid
GLORIEASTER B. SABANDO
- The embryonal form of rhabdomyosarcoma is
recognized as having a marked predilection for
occurrence in the head and neck area
 Clinical and histologic features:
A. Embryonal rhabdomyosarcoma
- This is the most common subtype observed in
children
- accounting for 60–70% of all
rhabdomyosarcoma cases in this age group
- can occur at any site, but they are most
commonly observed either in the genitourinary
region or the head and neck region
- They occur more commonly in the head and
neck area than any of the other forms
- arises chiefly from the orbital, facial, and
cervical musculature
- Predilections:
- The average age of this group of
patients was six years, ranging from
16 months to 16 years
- with no gender predilection
- the cheek, mandible and gingiva were found to
be additional sites of occurrence
- has been described as exhibiting a mixture of
four cell types:
• Eosinophilic spindle cells: usually
arranged in interlacing fascicles.
• Round eosinophilic cells: large and
intermediate in size, with a small
nucleus and a granular eosinophilic
cytoplasm generally interspersed among
other cell types.
• Broad elongated eosinophilic cells,
occasionally with cross-striations.
• Small round and spindle cells with dark-
staining nuclei and little cytoplasm
DDM5A ORAL PATHOLOGY II
B. Botryoid rhabdomyosarcoma
- has been long recognized as a malignant tumor
of the vagina, prostate, and base of the bladder
in young children
- Today, it is generally accepted as a variant of
embryonal rhabdomyosarcoma and has been
reported also involving the maxillary sinus,
nasopharynx, common bile duct and middle ear
- This tumor accounts for 10% of all
rhabdomyosarcoma cases
- It was formerly separated out as an entity
because of its unusual clinical growth pattern
- demonstrates a diffuse myxoid or mucoid
matrix with sparsely scattered primitive
mesenchymal cells
- The characteristic feature of this type is a
peripheral zone of increased cellularity,
sometimes known as the ‘cambium layer’
- Regardless of the histologic subtype, special
stains are often quite useful for differentiating
rhabdomyosarcoma from other neoplasms
- The trichrome stain colors rhabdomyoblasts
bright red while myofilaments and cross-
striations are stained by PTAH (deep purple
color)
- Myxoid stroma may be positive for
hyaluronidase with acid mucopolysaccharide
staining, although many other tumors also
have positive stroma with these stains
C. Alveolar rhabdomyosarcoma
- This subtype making up about 20% of all
rhabdomyosarcomas
- is reported to occur much earlier in life,
generally between 10 and 20 years of age
with a median of 15 years
- However, the range in this group was five
months to 58 years
GLORIEASTER B. SABANDO
- While the majority of cases of this type also
occurred in the extremities, approximately
18% were found in the head and neck region
- comprised of:
- relatively small, poorly differentiated
round and oval cells
- aggregated into irregular clusters or
nests separated by fibrous septa
- Degenerated cells in the center of
the clusters show lack of
cohesiveness, while the peripheral
cells adhere in a single layer to the
septal walls
- Multinucleated giant cells may be
seen and mitotic figures are common
and sometimes bizarre
- It is differentiated from the alveolar
soft part sarcoma by its less regular
tissue pattern and more pleomorphic
cells
D. Pleomorphic rhabdomyosarcoma
- least common of all rhabdomyosarcoma
- is a form of the tumor which, occurs more
frequently in the extremities than in other sites
and is generally seen in older individuals
- the average age was 53 years.
- The chief presenting complaint of the patient is
usually swelling, but pain may be present if
there is nerve involvement
- Depending upon the site of the lesion, the
following phenomena may be recognized:
divergence of an eye, abnormal phonation,
dysphagia, cough, aural discharge or deviation
of the jaw
- The lesions are occasionally ulcerated and may
invade underlying bone and develop distant
metastases
DDM5A ORAL PATHOLOGY II
- The most common site of presentation is:
- head
- neck region (35 %)
- composed chiefly of:
- spindle cells in a haphazard arrangement
- These cells are generally large and show
considerable variation in appearance
- The nuclei are ovoid or elongated with
packed chromatin
- A characteristic feature of this form of
tumor is the large bizarre cells, the nuclei
situated often in an expanded end of the
cell, the ‘racquet’ cell
- ‘Strap’ and ‘ribbon’ cells typically show
processes of long streaming cytoplasm
- Mitoses, particularly atypical, are
common
- The cytoplasm is eosinophilic, and
intracytoplasmic longitudinal fibrils as
well as transverse cross-striations may be
seen
- Cytoplasmic vacuoles are also present as
a result of large amounts of glycogen in
the cell
- This tumor is often so undifferentiated
that the identification of the cell of origin
is difficult or impossible
- Positive immunostains for desmin and
myoglobin are very helpful in such cases
 Treatment and Prognosis:
- radical surgical excision followed by
multiagent chemotherapy
- Postoperative radiotherapy is used for those
cases which cannot be completely resected
GLORIEASTER B. SABANDO DDM5A ORAL PATHOLOGY II
- The lesional cell is large and polygonal with a distinct
3. ALVEOLAR SOFT-PART SARCOMA (MALIGNANT cell border, a vesicular nucleus, and dense, abundant
GRANULAR CELL MYOBLASTOMA) granular, eosinophilic or vacuolated cytoplasm
- is a tumor of uncertain histogenesis originally - There is minimal variation in size and shape between
described under this name by Christopherson and cells and mitotic activity is sparse
his coworkers in 1952 - These cells have a uniform pseudoalveolar or
- It is a rare tumor, thought by some investigators to organoid pattern, arranged in relation to numerous
be of striated muscle origin delicate endothelium-lined vascular channels and
- believe that it may be of neural origin and a septa
variant of malignant granular cell tumor or a - The pattern is reminiscent of that seen in the
malignant nonchromaffin paraganglioma nonchromaffin paraganglioma
- Studies showed alveolar soft-part sarcomas are
myogenic by immunophenotyping in a number of  Treatment and Prognosis:
cases
- In recent years, pathologists have shown this - Radical surgical excision
tumor to be a variant of a rhabdomyosarcoma, a - high frequency of recurrence, metastases and death
sarcoma of skeletal muscle of patients
- Chromosome rearrangement at 17q25 and Xp11.2 - recurrence or metastatic rate as 70%
in alveolar soft-part sarcoma was demonstrated - five-year survival being uncommon
- Lieberman and his coworkers stated that they knew
 Clinical Features: of no lifetime cures.
- The initial report of Christopherson indicated that
this is predominantly a tumor of females,
occurring usually in the teens or early 20s.
- Occasional cases in older adults are reported
- Approximately one in four lesions occur in the
head and neck region, usually the oral cavity,
pharynx and orbit
- However, the greatest predilection is for the
muscles of the extremities, although lesions in the
tongue and floor of the mouth have been reported
- The lesions are usually slow-growing, well-
circumscribed masses with no distinguishing gross
features

 Histologic Features:
- This tumor is composed of large cells with a finely
granular cytoplasm that is not as eosinophilic as the
cell of the rhabdomyosarcoma