Mesenchymal Tumors

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Mesenchymal Tumors
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Lipoma

• Benign tumor of adipose tissue
• Adult patients
• Slow-growing, non-tender, soft, doughy, usually
  encapsulated
• Common in head and neck occasionally found
  intraorally
• Yellow if close to the surface

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Lipoma - Histopathology

• Demarcated or encapsulated collection of mature
  fat cells

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Lipoma

• Enucleation or conservative surgical excision
• Little or no tendency to recur
• No evidence of malignant transformation

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Traumatic Neuroma

• Represents an uncommon reaction to the sectioning
  of a nerve
• Smooth-surfaced, dome-shaped papule, usually less
  than one cm.
• Tongue, buccal vestibule are often affected
• May be tender on palpation

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Traumatic Neuroma

• Microscopically, a tangled mass of peripheral
  nerve fibers is seen
• Usually set in a collagenous background

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Neurilomoma (Schwannoma)

• Benign tumor of schwann cell origin
• Most are identified in adults
• Slow-growing, solitary, encapsulated,
  rubbery-firm, non-tender mass
• Lips, tongue, buccal mucosa
• May be seen within the mandible

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Neurilemoma

• Well-developed connective tissue capsule
• Benign proliferation of spindle-shaped schwann
  cells
• Antoni A and Antoni B patterns are seen, with
  Antoni A characterized by palisaded nuclei
  arranged around Verocay bodies

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Neurilemoma

• Treatment consists of conservative excision
• Lesion usually shells out due to dense
  connective tissue capsule
• Virtually no tendency to recur
• Extremely rare malignant transformation

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Neurofibroma

• Benign tumor of neural fibroblast origin
• Over 90 are solitary remainder are multiple and
  associated with neurofibromatosis
• Soft, dome-shaped, non-tender, superficial nodule
  affecting skin or mucosa
• Demarcated, but unencapsulated

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Neurofibroma

• Collection of fibroblastic cells that have wavy
  nuclei
• Usually quite a few mast cells are seen within
  the lesion
• Lesional tissue tends to mingle with the adjacent
  normal tissue

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Neurofibroma

• Treatment consists of simple, conservative
  excision
• Prognosis is generally good
• Uncommon possibility of malignant transformation
  to malignant peripheral nerve sheath tumor
  especially rare for small, superficial lesions

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Neurofibromatosis

• One of the most common autosomal genetic problems
  that affects humans
• Occurs at a frequency of approximately 1 in 3,000
  live births
• Approximately half are transmitted as autosomal
  dominant trait other half appear to be new
  mutations

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Neurofibromatosis

• Highly variable gene expression some cases are
  very mild, others are quite severe
• Variety of manifestations, both cutaneous and oral

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Neurofibromatosis

• Skin lesions include
• Café-au-lait spots
• Light brown (the color of coffee with milk)
  macules with smooth (coast of California)
  borders
• Usually 6 or more, greater than 1.5 cm in the
  adult patient

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Neurofibromatosis

• Skin lesions include
• Café-au-lait spots
• Multiple neurofibromas
• These can be small, discrete lesions or massive,
  pendulous ones

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Neurofibromatosis

• Skin lesions include
• Café-au-lait spots
• Multiple neurofibromas
• Axillary freckling

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Neurofibromatosis

• Oral lesions consist of neurofibromas that may
  affect the tongue, gingivae or bone

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Neurofibromatosis

• Treatment consists of removing traumatized
  neurofibromas or disfiguring lesions
• Genetic counseling
• Follow for potential malignant transformation

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Neurofibromatosis

• Prognosis should be considered fair to guarded
• If malignant transformation occurs, prognosis is
  poor

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Melanotic Neuroectodermal Tumor of Infancy

• Rare tumor seen in the first 2-3 years of life
• Most develop in the anterior maxilla
• Rapid growth
• May be brown or black in color

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Melanotic Neuroectodermal Tumor of Infancy

• Radiographic appearance is characteristic
• Radiolucency of anterior maxilla
• Deciduous maxillary incisor is pushed labially,
  appearing as a tooth floating in space

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Melanotic Neuroectodermal Tumor of Infancy

• Proliferation of small, dark, neuroectodermal-appe
  aring cells that are in nested aggregates
• Surrounded by plump, epithelioid cells that
  produce melanin

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Melanotic Neuroectodermal Tumor of Infancy

• Conservative excision is usually curative
• Good prognosis
• Sporadic reports of aggressive behavior are
  probably over-represented in the literature

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Granular Cell Tumor

• Older term for this was granular cell
  myoblastoma, but current evidence indicates
  neural differentiation
• Develop on any cutaneous or mucosal surface, but
  40 occur on the tongue

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Granular Cell Tumor

• Slow-growing, demarcated (though unencapsulated),
  non-tender submucosal nodule
• Most are less than one cm. in size
• Tongue is favorite site, but buccal mucosa and
  floor of mouth may be affected

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Granular Cell Tumor

• Collection of mesenchymal cells with a
  granular-appearing cytoplasm
• PEH (pseudoepitheliomatous hyperplasia) is
  present in about 30 of granular cell tumors
  may be mistaken for squamous cell carcinoma
  microscopically

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Granular Cell Tumor

• Conservative excision is usually curative
• Prognosis is excellent

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Congenital Epulis

• Rare lesion of undetermined histogenesis
• Found at birth on the maxillary ridge of girl
  babies
• Smooth-surfaced, often pedunculated
• Vary in size

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Congenital Epulis

• Microscopically shows a benign proliferation of
  cells having granular cytoplasms
• No pseudoepitheliomatous hyperplasia
• No S-100 positivity

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Congenital Epulis

• Conservative excision
• No tendency to recur
• Some reports of spontaneous involution without
  surgery

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Hemangioma

• Some consider this to be a vascular hamartoma
  rather than a true neoplasm
• Most common tumor of childhood
• Head and neck region is frequently affected
• Tongue is most common intraoral site

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Hemangioma

• Usually red in color, but can range through
  various shades of purple, depending on the
  caliber of vessels
• Most are elevated, but macular lesions are also
  seen (port wine stain nevus flammeus)
• Variable in size

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Hemangioma

• Diascopy may be helpful in determining whether a
  red lesion is due to extravasated blood or to
  blood that is contained within vessels
• Press on lesion with glass slide blood within
  vessels will be pushed into adjacent vessels and
  lesion will blanch

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Hemangioma - Treatment

• Surgical excision, cryotherapy, embolization or
  use of sclerosing agents if cosmetically
  unacceptable
• If lesion impairs vision, treat as soon as
  possible
• Otherwise, wait until child is at least 6 years
  of age

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Encephalotrigeminal Angiomatosis

• Also known as Sturge-Weber syndrome (really
  should be anomalad)
• Congenital abnormality, not an inherited or
  genetic problem
• Hemangiomatous process affecting superficial and
  deeper tissues in region of cranial nerve V

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Encephalotrigeminal Angiomatosis

• Patients exhibit nevus flammeus in distribution
  of 1st, and sometimes 2nd or 3rd, division of
  C.N. V
• Involvement of deeper soft tissues as well as
  meninges of brain
• Seizure disorders, mental retardation, and
  hemiplegia may be present

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Encephalotrigeminal Angiomatosis

• Classic radiographic finding of tram-line
  calcifications seen on skull film
• Parallel calcifications probably represent the
  calcified walls of abnormal blood vessels

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Lymphangioma

• Benign neoplasm of lymphatic vessel
  differentiation
• Often present at birth
• Tongue is most common intraoral site
• Surface has a vesicular appearance, similar to
  frog eggs or tapioca pudding

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Cystic Hygroma

• The cystic hygroma is simply a very large
  lymphangioma
• May affect the neck, mediastinum, axilla and the
  oral cavity
• Important because this lesion may cause airway
  obstruction, particularly if it becomes
  secondarily infected

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Lymphangioma

• Management depends on size of lesion
• More difficult to treat than hemangioma because
  it is often difficult to discern the borders of
  the tumor
• Surgical excision is the treatment of choice
• Relatively high recurrence rate up to 40 in
  some series

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Leiomyoma

• Benign tumor of smooth muscle differentiation
• Most in the oral region probably arise from
  vascular smooth muscle
• Well-demarcated, rubbery firm, less than 1 cm in
  diameter

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Leiomyoma

• May have a reddish or purplish color due to their
  vascular nature
• Most common in the upper lip, buccal mucosa and
  palate

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Leiomyoma

• Benign proliferation of cells that resemble
  smooth muscle
• Spindle-shaped, with cigar-shaped nuclei and
  eosinophilic cytoplasms
• No significant atypia, and no mitotic activity
• Positive for markers of muscle differentiation

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Leiomyoma - Treatment

• Treatment consists of conservative excision
• Prognosis is excellent
• Virtually no chance of recurrence

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Rhabdomyoma

• Very rare, benign tumor of skeletal muscle
  differentiation
• Most rhabdomyomas arise in the heart (cardiac
  rhabdomyoma) and are associated with the
  syndrome tuberous sclerosis

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Rhabdomyoma

• Extra-cardiac rhabdomyomas usually develop in the
  head and neck region
• Non-tender, well-circumscribed mass in the
  submandibular or base of tongue region

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Rhabdomyoma - Treatment

• Conservative surgical excision is appropriate
• Recurrence has been reported, but is not common