Neural Tissue Tumors Flashcards
what neural tissue tumors are categorized as a hyperplasias?
traumatic neuromas
what neural tissue tumors are considered hamartomas?
multiple endocrine neoplasia syndrome (MEN syndrome)
what is a harmartoma?
a developmental anomaly
what is MEN syndrome?
an AUTOSOMAL DOMINANT condition involving the endocrine system, with one variant (MEN-IIB) having oral manifestations consisting of multiple neuromas on the mucosal surfaces, lips and anterior dorsal tongue
what neural tissue tumors are considered benign neoplasms?
- neurileomas (Schwannoma)
- neurofibromas
- granular cell tumor
- congenital gingival granular cell tumor
- neuroectodermal tumor of infancy
what neural tissue tumor is considered a malignant neoplasm
malignant neoplasms of nerve origin (neurogenic sarcomas)
key features of traumatic neuromas
- these are NOT TRUE NEOPLASMS
- they are HYPERPLASIA
- most common site MENTAL FORAMEN
- PAINFUL NODULAR GROWTH
what are traumatic neuromas?
PAINFUL nodular proliferation of the nerve sheath and fibrous tissue that result from as severed nerve attempting to reunite
what glands does MEN syndrome affect?
endocrine glands (PARATHYROID, PANCREAS, THYROID, ADRENAL GLAND)
what chromosome is affected in the MEN IIB syndrome?
chromosome 10
multiple oral neuromas are often the first indicator of what syndrome?
MEN syndrome
what germline mutation causes MEN IIB syndrome?
RET proto-onco gene
why is it so important to be able to recognize MEN syndrome
because it has the potential to malignantly transform into fatal conditions such as THYROID CARCINOMA or PLEOCHROMOCYTOMA
what are neurilemomas?
benign, PAINLESS, fibroblastic proliferation of SCHWANN CELLS (nuerilemma cells) that produce distinctive patterns referred to as ANTONI A and ANTONI B tissue
key features of neurilemomas
- affects SCHWANN CELLS
- PAINLESS
- form around IA NERVE and DORSAL SURFACE of TONGUE
- can affect the ACOUSTIC nerve (CN VIII) - called acoustic neuroma
- produced distinct tissue patterns called ANTONI A and ANTONI B
what is the difference between ANTONI A and ANTONI B tissue patterns of neurilemomas?
- antoni A - are well organized and leave behind acellular pink areas known as VEROCAY BODIES
- antoni B - have NO ORGANIZATION
in order to diagnose do you need to see both ANTONI A and ANTONI B histologic tissue patterns
NO, you usually see both antoni A and antoni B together, but the presence of ANTONI A alone is DIAGNOSTIC
what are neurofibromas?
benign neoplasm arising from PERINEURAL FIBROBLASTS leading to increased COLLAGEN
key features of neurofibromas
- arise from PERINURAL FIBROBLASTS
- usually single lesions, but can have MULTIPLE lesions
- histology: lots of COLLAGEN and SPINDLE CELLS
MULTIPLE neurofibromas are associated with what condition?
multiple neruofribromatosis, VON RECKLINGHAUSEN DISEASE
key features of von Reckilnghausen disease
- aka neurofibromatosis type I
- multiple skin and oral neurofibromas
- CAFE AU LAIT SPOTS are common
- AUTOSOMAL DOMINANT
- mutation in NF GENE (tumor suppressor factor), located on CHROMOSOME 17
- there is a risk of malignant transformation
- TX: excision (malignant recurrence with a vengeance)
how would you diagnose von Reckilnghausen disease?
there are 7 diagnostic criteria and you must have 2 for diagnosis:
1) 6 or more cafe au lait spots
2) freckling of axial or inguinal regions
3) optic gioma
4) 2 or more Lisch nodules (iris hemotomas)
5) distinct osseous lesion
6) first degree realative with NF1 dx
7) 2 or more neurofibromas
key features of granular cell tumors
- classified as a NERVE TUMOR
- thought to arise from SCHWANN CELLS
- predilection for middle aged males
- 70% of head and neck lesions occur in mouth, most commonly on LATERAL** and DORSAL SURFACES of the TONGUE
- PAINLESS
- SUBMUCOSAL MASS
are granular cell tumor malignant?
NO!
granular cell tumors may be confused with what other disease?
- SQUAMOUS CELL CARCINOMA
- granulomas
what is a congenital gingival granular cell tumor?
a granular cell tumor on the gingival of newborns
do congenital gingival gingival granular cell tumors have PSEUDOEPITHELIALIOMATOUS hyperplasia?
NO!!! That is a histological distinction of granular cell tumors in adults. PSEUDOEPITHELIALIOMATOUS hyperplasia is NOT see in congenital gingival granular cell tumors
who are more at risk of developing congenital granular cell tumors, girls or boys?
newborn GIRLS
what is the most common location of congenital granular cell tumors?
in the MX and MN of newborn GIRLS
are congenital granular cell tumors sessile or pedunculated?
PEDUNCULATED and can become quite LARGE if left untreated
what cells are melanotic neuroectodermal tumors of infancy derived from?
NEURAL CREST CELLS
how can you histologically unique about melanotic neuroectodermal tumors of infancy?
they have two distinct cell types LARGE PIGMENTED cells, producing melanin and SMALL DARK cells
what age group are neuroectodermal tumors more common in?
INFANTS (less then 6 months old)
how do melanotic neuroectodermal tumors of infancy present clinically?
most often present in the MAXILLA (may present in other places) as PIGMENTED (BLACK or BROWN) nodules
what neural tissue tumor is know to be malignant?
malignant neoplasms of nerve origin (neurogenic sarcomas)
malignant neoplasms of nerve origin (neurogenic sarcomas) arise from what cells?
FIBROBLAST or SCHWANN CELLS
are malignant neoplasms of nerve origin (neurogenic sarcomas) common or rare?
they RARELY occur in the oral cavity
what is the rate of hematogenous metastasis for malignant neoplasms of nerve origin (neurogenic sarcomas)?
50%
do neurogenic sarcomas have potential for metastasis?
YES!! the are also known as malignant neoplasms of nerve origin and have a 50% chance of hematogenous metastases
what is the prognosis of malignant neoplasms of nerve origin (neurogenic sarcomas)?
BAD, very POOR prognosis!! most patients die of their disease
malignant neoplasms of nerve origin (neurogenic sarcomas) are often associated with…
nerve trunk lesions → PAIN
