Term
What systemic syndrome is associated with Schwannoma? |
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Definition
Neurofibromatosis Type II |
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Term
What systemic syndrome is associated with Neurofibroma? |
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Definition
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Term
What is the systemic syndrome associated with Mucosal Neuromas? |
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Definition
Multiple Endocrine Neoplasia syndrome Type III (MEN III or IIB) |
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Term
What are the smooth muscle neoplasms? |
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Definition
Leiomyoma (benign)
Leiomyosarcoma (malignant) |
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Term
What are the skeletal muscle neoplasms? |
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Definition
Rhabdomyoma (benign)
Rhabdomyosarcoma (malignant) |
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Term
What is the pathogenesis of a Traumatic Neuroma? (Reactive, Neural Lesion) |
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Definition
- Caused by injury to peripheral nerve (ex: tooth extraction, or LA injection, or accident)
- Transection of a sensory nerve can result in inflammation and scarring in the area of injury bc the nerve segment is attempting to proliferate |
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Term
In an Immunohistochemistry profile, what would differentiate a tumor of neural origin? |
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Definition
A tumor of neural origin would be positive for S-100 |
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Term
What are common sites for a Traumatic neuroma? |
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Definition
Soft tissue: lower lip, tongue, buccal mucosa and palate
Intrabony: Mental foramen, or extractions sites in the ant max and post mand |
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Term
Treatment for Traumatic neuroma? |
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Definition
Surgical excision (recurrence is infrequent) |
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Term
What is a related lesion to Granular Cell Tumors? |
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Definition
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Term
What are the clinical features of a Granular Cell Tumor? |
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Definition
- Benign tumor, neural sheath origin - Any age, females slightly more than males - Any site; usualy the tongue - Asymptomatic - Same or lighter in color than mucosa - Intact overlying epithelium |
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Term
Histopathology description: "Large, uniform cells w/ granular cytoplasm, overlying pseudoepitheliomatous hyperplasia, cells positive for S-100"
Which neural neoplasm is this? |
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Definition
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Term
Where is a Schwannoma most likely found? |
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Definition
On the tongue, but could be found in any oral site |
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Term
Where is a neurofibroma most commonly found? |
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Definition
Tongue and buccal mucosa, but could be anywhere. Can also occur intrabony |
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Term
Where are Mucosal Neuromas usually found? |
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Definition
Tongue, buccal mucosa, and Lip***
***If found on the lip (commissure) very like to have the associated systemic syndrome MEN III (IIB) |
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Term
Histology description: "Antony A and Antony B patterns, positive for S-100, actin and desmin stains are negative"
Which neural lesion is this? |
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Definition
Schwannoma (Neurilemmoma) |
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Term
Histology description: "Haphazardly arranged spindle cells (Schwann)"
What neural lesion is this? |
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Definition
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Term
Why is is important to determine if a patient has Neurofibromatosis type I in a patient with neurofibromas? |
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Definition
Because a patient with NF1 has a 5-15% chance that the neurofibroma will undergo malignant transformation to a neurogenic sarcoma |
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Term
What are phenotypic expressions of Neurofibromatosis I? |
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Definition
- Cutaneous Neurofibromas - Neurofibroma on tongue and enlarged fungiform papillae - Cafe au lait pigmentation (smooth edges) - Plexiform Neurofibroma |
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Term
What conditions are associated with Multiple Endocrine Neoplasia syndrome Type III (MEN III)? |
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Definition
- Medullary carcinoma of the thyroid - Pheochromocytoma of adrenal glands (hypertension) - Mucosal Neuromas* - Marfanoid (long) body
*could be found in oral cavity |
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Term
Histological description: "Hamartoma of the nerve tissue with the main difference being the thickening of the perineurium"
What neural lesion is this? |
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Definition
Mucosal neuromas of MEN III syndrome |
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Term
What is the significance of diagnosing mucosal neuromas? |
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Definition
They may be the first sign in the potentially fatal Multiple Endocrine Neoplasia syndrome type III |
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Term
What is the 5 year survival rate for medullary carcinoma of the thyroid (associated with MEN III)? |
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Definition
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Term
Which oral tumor of neural origin is not associated to any systemic syndromes? |
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Definition
Palisaded Encapsulated Neuroma (solitary circumscribed Neuroma) |
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Term
What is the name of a rare malignancy that develops from either a preexisting neurofibroma or de novo, and could also be a complication of neurofibromatosis? |
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Definition
Malignant peripheral nerve sheath tumor |
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Term
What is the common sites for Malignant peripheral nerve sheath tumor? |
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Definition
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Term
Histopathology: The lesion is composed of: - Abundant spindle cells and wavy nuclei - Variable numbers of abnormal mitotic figures - Streaming and palisading of nuclei are often seen - Nuclear pleomorphism
What lesion is this? |
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Definition
Malignant peripheral nerve sheath Tumor |
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Term
What is the tx for malignant peripheral nerve sheath tumor? |
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Definition
Wide surgical excision but recurrence is common 5 year survival rate 40%, if the pt has NF the rate is 15% |
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Term
What lesion is described as a rare malignant lesion that arises from olfactory tissue in the superior portion of the nasal cavity? |
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Definition
Olfactory Neuroblastoma (Esthesioneuroblastoma) |
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Term
What are symptoms of Olfactory Neuroblastoma? |
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Definition
Epistaxis, rhinorrhea, or nasal obstruction
These are very similar characteristics to a benign condition called Juvenile Nasopharyngeal Angiofibroma |
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Term
Histological description: "Small, undifferentiated, round blue cells with little visible cytoplasm. Pseudorosette and rosettes. Compartmentalization" What lesion is this? |
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Definition
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Term
What is the name of an uncommon reactive lesion of skeletal muscles in which ossification occurs? |
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Definition
Myositis Ossificans (muscle, reactive lesion) |
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Term
What is the common sites for Myositis ossificans? |
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Definition
In the head and neck it commonly occurs in masseter and SCM |
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Term
Radiographically what does myositis ossificans look like? |
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Definition
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Term
What would be a differential dx for myositis ossificans? |
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Definition
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Term
Tumor is positive for actin and negative for desmin, what could it be? |
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Definition
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Term
Tumor is positive for actin and negative for desmin, what could it be? |
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Definition
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Term
Tumor is positive for actin and desmin, what could it be? |
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Definition
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Term
Tumor is negative for actin and positive for desmin, what could it be? |
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Definition
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Term
What type of lesion would appear like any other benign soft tissue lesion but have a yellowish hue to it? |
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Definition
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Term
Histology: Irregular fat cells with atypical nuclei.
What is this describing? |
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Definition
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Term
What is the most common soft-tissue sarcoma in pediatric patients? |
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Definition
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Term
Histology: Spindle cells with atypical nuclei, infiltrative invasive borders, necrosis, >2 mitotic figures/10high power field.
What is this? |
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Definition
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