Benign neoplasm of muscle

By/ dr: kamal Abd El Rahman kamal

 Oral leiomyoma

By/ dr: kamal Abd El Rahman kamal

Leiomyoma:
It is a benign neoplasm of smooth muscle.
•!• In the oral cavity it arises from smooth muscle in
the wall of blood vessels

By/ dr: kamal Abd El Rahman kamal

 Clinically:
• The oral leiomyoma is characterized by a firm
and sometimes with vascular appearance of small
sized nodular and slow growth.

• Site: commonly in tongue, lips, palate and buccal

mucosa.

• Frequently, it is asymptomatic.

By/ dr: kamal Abd El Rahman kamal

By/ dr: kamal Abd El Rahman kamal
Histopathologically, there are three types of leiomyomas:

• Leiomyoma (solid),

• Angiomyoma (vascular leiomyoma), and

• Epitheloid leiomyoma (leioblastoma), the rare form.

• Solid type: large number of small sized blood vessels (slit-like).
• Tumors of the venous type have venous blood vessels with thick
muscular walls. The outer layers of the smooth muscle in the
vascular walls blend with intervascular smooth muscle bundles.
• Cavernous type are composed of dilated vascular vessels with
small amounts of smooth muscle, and the muscular walls of these
vessels are difficult to distinguish from smooth muscle bundles
Rhabdomyoma
Rhabdomyomas are rare benign tumors derived from
striated muscle, and can be classified generally into two
types:
• Cardiac type: occurs in the pediatric age.
• The cardiac type represents a hamartomatous growth.

•• Extracardiac type: can be divided into three groups

1. The adult type: is a slowly growing mass in head and
neck of elderly patients.
2.The fetal type, involves the head and neck region at
younger ages.
3. The genital type is almost in the vulvovaginal
region of middle-aged women
Of the oral mucosa is a rare benign neoplasm of skeletal muscle origin.
• It is located only where skeletal muscle is (tongue, gingival, palate, etc)
• The most common site is the tongue. It is fixed to surrounding
structures.

By/ dr: kamal Abd El Rahman kamal

Histopathologically there are large round, oval, and polygonal cells of varying sizes
with abundant, pale, eosinophilic, fine, granular cytoplasm with peripherally
located nuclei. Many tumor cells have variable vacuolization. In most cells the
vacuoles were located in the periphery, others resulted in a spider web appearance.
Mitoses and necrosis were absent.
 Granular cell myoblastoma

By/ dr: kamal Abd El Rahman kamal

It is a benign neoplasm previously called granular cell
myoblastoma
The tumor cells are of controversial cell origin.

1. Striated muscle, but they . are absent in regions where the

tumor existed.

2. Histiocytes, because they arc granular cells

contain phagocytosed materials.

3. Connective tissue of nerves, granular cell neural

fibroma (neural theory)

4. Stem cells, they are specialized smooth muscle cells with

leiomyotiba·illogenic capacity.
•:• The lesion is fixed to surrounding structures.
•:• Site: the most common location is the dorsum of the tongue.
Microscopically, the lesion often appears infiltrative, however, conscvative
excision and microscopic diagnosis is usually curative.
Congenital epulis of the newborn
congenital epulis of the newborn :It is a benign soft tissue
enlargement new born
The tumor cells are of controversial cell origin.

1. Myogenic

2. Histiocytic

3. Neurogenic

4. Fibroblastic

5. Epithelial, electron microscope study revealed junctional

complex between some of the granular cells (hamartoma)
Site: on the alveolar mucosa of infants, commonly anterior
maxilla.
•:• Sex: almost 90% of these lesions occur in females.
Microscopically, this tumor is similar to the granular cell myoblastoma except,
neither mitosis or cross striation are present and The covering epithelium is
normal or atrophic (no pseudoepitheliomatous
l malignant muscle tissue tumors

1: Leiomyosarcoma
2: Rhabdomyosarcoma
3: Alveolar soft part sarcoma
 Malignant nerve tissue tumors

malignant sclmannoma
Tumors of nerve tissue origin
Neuritis
 Neurolemmoma
(schwannoma)
•:• It is a benign neoplasm of Schwann cells.

•:• It usually presents as a slow growing encapsulated mass

that is typically asymptomatic.

•:• It is firm, encapsulated, and often freely moveable.

In the oral cavity, these tumors may arise from the tongue,
palate, buccal mucosa, lip and gingival.
Circumscribed firm-elastic swelling, usually asymptomatic otherwise.
Histologically, schwannomas are descried as
Antoni type A or Antoni B.

• Antoni type A present a parallel arragement of fibrocyte-like cells,

and Verocay bodies.

Antoni-A cells” (with elongated nuclei and arranged in a palisade pattern around
acellular collagen-like structures, so-called Verocay bodies
• Antoni B, is less cellular and shows microvacuolation of the
intercellular substance
 Neurofibroma
(Von Recklinghausen’s disease of skin)
It is a benign neoplasm of Schwann cells, commonly occurs as:
It is a tumor of nerve tissue origin arising from cels of Schwann,
fibroblasts and occasional perineural cells with intermingled neurites
or axons

•:• A solitary lesion: is fixed to surrounding structures and may be

firm or compressible upon palpation.

•:• Multiple neurofibromas: are a characteristic feature of

neurofibromatosis (von Recklinghausen disease of skin): Multiple
neurofibromas throughout body. Autosomal dominant inheritance
(gene is on chromosome 17).

5-10% chance of malignant development.

Cafe au lait spots (brown skin patches).
In the other form: There are deeper, more diffuse lesions which are of greater
proportions than the superficial nodules sometimes called “Elephantiasis
neuromatosa”.
Histologically, it is composed of:

• Fibrous connective tissue with partial encapsulation.

• Highly cellular connective tissue, wavy collagen fibers and

myxomatous areas interspersed with areas of nerve bundles
(peripheral nerve tissues within the perineural sheath), elongated
spindle shaped cells with wavy nuclei. Mast cells and dilated blood
vessels
 Traumatic neuroma

It is a reactive proliferation of nerve bundles following severing of

a nerve. a hyperplasia of nerve fibers and their supporting
tissues.

•:• It arises most commonly in locations containing

relatively large peripheral nerves, such as the mental foramen,
tongue and lower lip.

•:• It is often, but not always, painful to palpation.

Histologically:

• Nonencapsulated, poorly circumscribed lesions and is

characterized by disorderly outgrowth of a nerve fascicle.

• The components include tangled masses of axons, Schwann

cells, endoneurial cells, and perineurial cells in a dense
collagenous matrix with surrounding fibroblasts
The lesion is formed of a mass of an irregular and often interlacing neurofibrils
and Schwann cells situated in connective tissue stroma, much of this connective
tissue probably is derived from the perineurium.
Melanotic neuroectodermal tumor of
infancy

)pigmented ameloblastoma(
:Histogenesis

It has controversial origin: Odontogenic origin (pigmented

analage of the retina of the eye). it is represents an atavism of
sensory neuroectodermal development

Nneural crest cells: is the most accepted theory, which strongly

suggested by the following:

Presence of the high urinary excretion of vanillylmandelic acid

 Clinical Features

' it has predilection for occurrence before the age of six

months (All Cases Reported).

its color is darkly pigmented swelling .

* if it affects the alveolar ridge mucosa at the incisor region of
mandible or maxilla with maxillary predilection
Rdiographic Features:

Diffuse radiolucency similar to invasive malignant

 Histologic Features

It is characteristic, non-capsulated infiltrating tu mor masses arranged

in alveolus like spaces each one lined by cuboidal cells, many of them
contain melanin pigment the central portions of the alveolar spaces
contain small round neuroblast-like cells which show little
cytoplasm the centrally occupying cells exhibit a round, deeply
staining nucleus.
 Multiple Endocrine Neoplasia Syndrome.· ( MEN Sndmme)

It is a group of syndromes characterized by tumors of various

endocrine organs occurring in association with a variety of other
pathologic features. *It appears to be inherited as an autosomal
dominant characteristic.
Type I
(MEN I)

:It consists of the following•!•

• Tumors of pituitary,
• Tumors of the parathyroids,
• Tumors of the adrenal cortex and
• Tumors of the pancreatic islets
• It has peptic ulcers and gastric hypersecretion
Type IIa
:It consists of the following
• Hyperplasia or neoplasia of the parathyroids
• Pheochromocytomas of the adrenal medulla
• Medullary carcinoma of the thyroid gland
• No tumors of the pancrea no peptic ulce
Type IIb
:It consists of the following•!•
• Mucocutaneous neuromas
• Pheochromocytomas of the adrenal medulla
• Medullary carcinoma of the thyroid gland
• Hypertrophied corneal nerves
• Other skeletal defects
• Gastrointestinal difficulties
Oral Manifestations:

•!• Presence of neuromas: may be multiple, in the lip, described as "bumpy

lips, sometimes buffy and diffuse. In buccal mucosa and in tongue, at the
anterior two third
Histological features: Composed of tortuous masses of netrve fibers
surrounded by a thickened perineurium similar to traumatic neuroma.
Treatment and prognosis:

•!• The most important aspect of this syndrome is the medullary

carcinoma of the thyroid because of its ability to metastasize and cause
death. therefore, detection of mucosal neuromas may alert the clinician
for early diagnosis and prompt the treatment of the thyroid and adrenal
lesions.