Chapter 12 lecture 3 soft tissue tumors Flashcards
What is multiple endocrine neoplasia characterized by?
- Parathyroid tumors
- Pituitary tumors
- Pancreatic tumors
- Adrenal gland tumors
- Thyroid tumors
- Mucosal neuromas
What are adrenal gland tumors also known as?
-Pheochromocytoma
What are thyroid tumors also known as?
-Medullary carcinoma
Patients with multiple endocrine neoplasia have what type of build?
- Marfanoid build (long thin face)
- Elongated limbs
What is the typically the first sign of multiple endocrine neoplasia?
-Oral mucosal neuromas
What are bilateral neuromas of the commissural mucosa highly characteristic of?
-Multiple endocrine neoplasia
What is a rare pigmented tumor that usually occurs during the 1st year of life?
-Melanotic neuroectodermal tumor of infancy
What does a melanotic neuroectodermal tumor of infancy appear as clinically?
-Black and or blue rapidly expanding mass usually found on the anterior maxilla
How do they diagnose melanotic neuroectodermal tumor of infancy?
-Test for high urinary levels of vanillymandelic acid
What is a granular cell tumor?
-Benign soft tissue neoplasm that shows a predilection for the oral cavity
What is the most common site for a granular cell tumor?
-Tongue with the dorsal surface being the most common
What does a granular cell tumor appear as clinically?
-Yellow or mucosal colored, asymptomatic sessile nodule that is less than than 2 cm.
What can a granular cell tumor cause?
-Pseudoepitheliomatous hyperlasia (also caused by blastomycosis)
What is S-100 positive associated with: granular cell tumor or congenital epulis?
-Granular cell tumor
What can occur almost exclusively on the alveolar ridge of newborns?
-Congenital epulis
What does a congenital epulis appear as clinically?
-Mucosal colored, smooth surfaced, polypoid mass typically less than 2 cm
What arch do you typically see a congenital epulis?
-Maxillary on the ridge
What differentiates a congenital epulis from a granular cell tumor?
-a congenital epulis does not have PEH (pseudoepithelomatous hyperlasia) and is S-100 negative
What gender does a congenital epulis most commonly occur in?
-Female
What is the most common tumor of infancy?
-Hemangioma
What is the most common location for a hemangioma?
-Head and neck
What are the two types of hemangiomas?
- Capillary
- Cavernous
What might you do to tell if it is a capillary hemangioma?
-May not blanch clinically and is usually red in color
What might you do to tell if it is a cavernous hemangioma?
-Typically blanches, and is a darker red to purple color
What is a rare, nonhereditary developmental condition caused by the persistence of a vascular plexus around the cephalic portion of the neural tube?
-Sturge-Weber Angiomatosis
Patients with sturge-weber angiomatosis are born with a dermal capillary vascular malformation known as what?
- Port wine stain
- Nevus flammeus
T/F if a pt has port wine stain they have sturge-weber angiomatosis
False
-Not all people with the stain have sturge weber angiomatosis
T/F Sturge-weber angiomatosis is unilateral and is usually associated with the trigeminal nerve
True
What is a benign tumor of lymphatic vessels?
-Lymphangioma
What are the types of lymphangioma?
- Capillary
- Cavernous
- Cystic
Where are cavernous lymphangiomas more frequently found?
-In the mouth
What are cystic lymphangiomas most often found?
-Lateral Neck
Oral lymphangiomas occur most frequently where?
-Anterior two thirds of the tongue resulting often in macroglossia
What age do most cases of lymphangiomas occur?
-Before 2 yrs of age
What is a benign tumor of smooth muscle?
-Leiomyoma
What is the most common location of Leiomyoma?
- Uterus
- GI tract
- Skin
What is a benign neoplasm of skeletal muscle?
-Rhabdomyoma
Who does the adult type of Rhabdomyoma occur in?
-Men
In adult Rhabdomyoma where does it most commonly occur?
- Pharynx
- FOM
In fetal Rhabdomyoma who and where does it occur?
- Young male pts
- Predilection for the face
What are six soft tissue sarcomas?
- Fibrosarcoma
- Malignant fibrous histiocytoma
- Liposarcoma
- Leiomyosarcoma
- Angiosarcoma
- Malignant peripheral nerve sheath tumor
What are the four clinical presentations of Kaposi’s sarcoma?
- Classic
- Endemic
- Iatrogenic immunosuppression associated
- AIDS related
What ethnicities can get the classic kaposi’s sarcoma?
- Italian
- Jewish
- Slavic descent
Where is the endemic type of kaposi’s sarcoma found?
-Africa
What is a malignancy of skeletal muscles?
-Rhabdomyosarcoma
Where are the most common locations of Rhabdomyosarcoma?
-Face and orbit in children
Head and neck metastases in the absence of lung metastases is possible because of what?
-Batson’s plexus
What is Batson’s plexus?
-Valveless vertebral venous plexus that might allow retrograde spread of tumor cells and bypass the lungs
What is the most common site for metastases to the soft tissues?
-Gingiva
