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