Chapter 12 - Part 2 Flashcards
What is Multiple endocrine neoplasia Type 2B characterized by?
Parathyroid tumors, pituitary tumors, pancreatic tumors, adrenal gland tumors, thyroid tumors, mucosal neuromas
What are adrenal gland tumors called, and how many MEN patients get it?
Pheochromocytoma – 50% develop
What are thyroid tumors called, and how many MEN patients get it?
Medullary carcinoma – 90% develop
In addition to systemic disease, what is characteristic about the patient’s features?
A marfanoid build, with thin elongated limbs, and a narrow face with thick protuberant lips
What is typically the first sign of multiple endocrine neoplasia?
Oral mucosal neuromas – bilateral neuromas at the commissural mucosa
When is multiple endocrine neoplasia usually diagnosed?
Between the ages of 18-25
Why is it important to catch multiple endocrine neoplasia early on?
Multiple endocrine neoplasia has a marked propensity for metastasis
What term describes a rare pigmented tumor that usually occurs during the 1st year of life?
Melanotic neuroectodermal tumor of infancy
Where do melanotic neuroectodermal tumors of infancy usually occur?
Stirking predilection for the anterior maxilla (61%)
What is the clinical presentation of melanotic neuroectodermal tumors of infancy?
Rapidly expanding mass that is clinically black and/or blue
What urine test is associated with melanotic neuroectodermal tumor of infancy?
High urinary levels of vanillylmandelic acid
Are melanotic neuroectodermal tumor of infancy usually benign or malignant?
Most are benign
What is a granular cell tumor?
A benign soft tissue neoplasm that shows a predilection for the oral cavity
What is the most common site for a granular cell tumor?
The dorsal tongue
At what age do granular cell tumors usually occur?
Around 40, rare in kids
What is the clinical presentation for a granular cell tumor?
Asymptomatic, sessile nodule less than 2 cm, that may be yellow or mucosal colored
What population might experience multiple granular cell tumors?
African Americans
What is a significant microscopic feature of granular cell tumors?
Pseudoepitheliomatous hyperplasia
What other thing causes PEH? Blastomycosis infection
What test is used to diagnose a granular cell tumor?
The S-100 test, and it will be positive!!!
What term describes a mucosal colored, smooth surfaced mass that is present at birth?
Congenital epulis
Where does a congenital epulis commonly occur?
3x more common in the maxillary ridge (almost exclusively on the alveolar ridges),
In what population do congenital epulis (epuli/epulises??) commonly occur?
90% occur in females
What tests are done and test results are found to diagnose congenital epulis?
No pseudoepitheliomatous hyperplasia, and S-100 test is negative
How is a congential epulis treated?
Surgical excision
How is a granular cell tumor treated?
Surgical excision
What term describes a benign tumor of blood vessels?
Hemangioma
What is the most common tumor of infancy?
Hemangioma
In what population are hemangiomas most common?
White females
What is the most common location of hemangiomas?
The head and neck
What are the 2 types of hemangiomas?
Capillary and cavernous
What is the difference between capillary and cavernous hemangioma?
Capillary hemangiomas may not blanch and are usually red in color, where cavernous hemangiomas typically blanches and may be darker red to purple
–> diascopy: poke it
What term describes a rare, nonhereditary developmental condition?
Sturge-Weber angiomatosis
What is the cause of sturge-weber angiomatosis?
Caused by the persistence of a vascular plexus around the cephalic portion of the neural tube
What are the congenital dermal capillary vascular malformations referred to as?
Port wine stain or nevus flammeus
*not all patients with port wine stain have sturge-weber angiomatosis
What nerve is usually involved with Sturge-weber angiomatosis?
The trigeminal nerve = unilateral distribution
In addition to nevus flammeus, what do sturge-weber angiomatosis patients also have?
Leptomeningeal angiomas that overlie the ipsilateral cerebral cortex, and may result in mental retardation and contralateral hemiplagia (paralysis)
What is sturge-weber angiomatosis associated with?
A convulsive disorder (anticonvulsants = drug induced gingival hyperplasia)
Why are dentist’s and oral pathologists concerned about sturge-weber angiomatosis?
Intraoral involvement is common and results in hypervascular changes to the ipsilateral mucosa
What may occur if sturge-weber angiomatosis lesions are manipulated?
Hemorrhage
What term describes a benign tumor of lymphatic vessels?
Lymphangioma
What are the 3 types of lymphangiomas?
Capillar, cavernous, and cystic
Where are cavernous lymphangiomas commonly found?
In the mouth
Where are cystic lymphangiomas commonly found?
In the neck
When are lymphangioma lesions commonly noted?
Half of all lesions are noted at birth, and 90% develop by 2 years of age
Where do oral lymphangiomas occur most frequently occur?
On the anterior 2/3 of the tongue and often result in MACROGLOSSIA
What do lymphangiomas clinically look like?
Frog eggs, or tapioca pudding
What term describes a benign tumor of smooth muscle?
Leiomyoma
Where do leiomyomas most commonly occur?
Uterus, GI tract, skin (can be a neoplasia of smooth muscle wall around blood vessels)
What term describes a benign tumor or skeletal muscle?
Rhabdomyoma
Where do rhabdomyomas usually occur?
Extracardiac, but very rarely they will occur in the head and neck
What are the 2 types of rhabdomyomas?
Adult and fetal
What is the difference in adult and fetal type rhabdomyoma?
Adult rhabdomyomas occurs in adult men in the pharynx and FOM, where fetal rhabdomyomas occur in young males and have a predilection for the face
What is the treatment for leiomyomas and rhabdomyomas?
Surgical excision
What are the common soft tissue sarcomas that can (but rarely) occur in the oral and maxillofacial region?
Fibrosarcomas, malignant fibrous histiocytoma, liposarcoma, leiomysocarcoma, angiosarcoma, malignant peripheral nerve sheath tumor
What virus is Kaposi’s sarcoma caused by?
HHV-8
What are the 4 clinical presentations of Kaposi’s sarcoma?
Classic, Endemic, Iatrogenic immunosuppression associated, AIDS related
What is the most common clinical presentation of Kaposi’s sarcoma?
Classic type
Describe the classic type of Kaposi’s sarcoma.
It is hereditary, occurs in older men or Italian, Jewish or Slavic descent (Mediterranean). Multiple blue-purple lesions occur on the lower extremities. BUT it has 90% survival
What are the 4 subtypes of the Endemic (African) type of Kaposi’s sarcoma?
Benign nodular, aggressive, florid and lymphadenopathic… endemic has the poor prognosis
In what population do iatrogenic type kaposi’s sarcoma commonly occur?
Transplant patients
What does the treatment of Kaposi’s sarcoma depend on?
Type, stage, location
How is Kaposi’s sarcoma treated?
Radiation, surgery, and chemotherapy
What term describes a malignant tumor of skeletal muscles?
Rhabdomyosarcoma
Rhabdomyosarcoma accounts for how many soft tissue sarcomas of childhood?
60% of soft tissue sarcomas of childhood
How many of rhabdomyosarcomas occur in the head and neck?
35%, face and orbit are common locations
What is the most common oral soft tissue site of metastases?
Gingiva, then tongue
What do metastases to the oral soft tissues most commonly represent?
Carcinomas rather than sarcomas
What is one possible explanation of Head and neck metastases in the absence of lung metastases?
Batson’s plexus, which is a valveless vertebral venous plexus that might allow retrograde spread of tumor cells and bypass the lungs
