PLEOMORPHIC ADENOMA

PLEOMORPHIC ADENOMA:  A benign tumor, neoplastic in nature, that affects the salivary glands.  This is the most common parotid and salivary gland tumor. Consists of mixed epithelial cells and mesenchymal cells and is considered a mixed tumor.
PRESENTATION:  It is slow growing, painless, and firm and is often solitary. Smaller nodules originate from the main tumor. Mobile unless attached to the palatine plate (tumor usually in parotid gland). At this location atrophy to the ramus of the mandible usually occurs. The earlobe may appear everted if tumor located in parotid tail. The tumor may grow and progress to malignant over time--carcinoma ex-pleomorphic adenoma. The tumor may return after resection.
HISTOLOGY:  Tissue appears biphasic due to the mixture of the epithelial and myoepithelial cells. Contained within a pseudocapsule, extends within the parenchyma in the form of pseudpodia--finger-like. Exhibits characteristic chromosomal translocations between #3 and #8. Results in PLAG gene being juxtaposed to gene necessary for beta catenin causing inappropriated cell division.
DIAGNOSIS: 
1. FNA--fine needle aspiration--determine malignancy--90% accurate
2. Core needle biopsy--determine malignancy-->97% accurate
3. Ultrasound
4. CT
5. MRI
TREATMENT:
1. Superficial or Parotidectomy:  Surgical excision of the tumor is the standard treatment with biopsy prior to to confirm diagnosis.
2. Tricky surgey due to relationship of facial nerve to the parotid gland. Early detection is key to recurrent tumors.
3. Submandibular Gland:  Excised, careful not to disturb the trigeminal,  hypoglossal, or lingual nerves.
4. Malignant tumors:  Wide, local resection of the tumor in conjunction with radiation therapy if necessary. Sequellae include but not limited to:  cranial nerve damage, cosmetic issues, Frey's syndrome--neurological condition where the ears and/or cheeks become sweaty or flushed, especially when eating spicy, hot, bitter foods or chocolate, as a result of parotid gland surgery.
SURVIVAL RATE:
The rate is at the most 44% with total histiological resection of the tumor.
The mortality rate for the 44% is high at 87%.
The locoregional control rate for malignant tumors is only 66% over a 5 year recovery period making the recurrenc of the cancer undoubtedly fatal. 
REFERENCES: 
1. Frey's syndrome. (2011, January 26). Retrieved from http://www.wrongdiagnosis.com/f/freys_syndrome/intro.htm.
2. Hajiioannou, J., Vlastos, Y., Lachanas, V., & Kyrmizakis, D. (2006). Giant pleomorphic adenoma of parotis. Internet Journal of Otorhinolaryngology, 4(2), http://www.ispub.com/ostia/index.php?xmlFilePath=journals/ijorl/vol4n2/adenoma.xml.
3. Pleomorphic adenoma. (2011 , March 6). Retrieved from http://en.wikipedia.org/wiki/Adenoma%2C_pleomorphic.
5. Templer, J., & Meyers, A. (2009, July 6). Parotitis. Retrieved from http://emedicine.medscape.com/article/882461-overview
4. Yabuuchi, H., Matsuo, Y., Kamitani, T., Setoguchi, T., & Okafugi, T. (2008). Parotid gland tumors: can addition of diffusion weighted mr imaging to dynamic contrast-enhanced mr imaging improve diagnostic accuracy in characterization?. Radiology, 249(3909-916), http://radiology.rsna.org/content/249/3/909/F6.expansion.

Location of parotids and accessoy glands.

 

Pleomorphic tumor-benign, diagnosis confirmed by FNA biopsy.
Tumor has been growing for over 20 years.
Patient denied treatment.



Pleomorphic tumor of right parotid
Axial  MR T1--Post-contrast

MOYAMOYA DISEASE

MOYAMOYA DISEASE:  A disease that progressively blocks the cerebral vasculature flow in the brain, in particular the Circle of Willis and the feeding vessels.
SYMPTOMS: 
Children:  Hemiparesis, monoparesis, sensory impairment, involuntary movements, headaches, dizziness, or seizures, menatal retardation and neurological deficits may be present. Tend to suffer cerebral ischemic events than adults.
Adults:  Symptoms similar to children with possible intraventricular, subarachnoid, or intracerebral hemorrhage. Tend to suffer hemorrhage more than children.
DIFFERENTIAL DIAGNOSIS:
Stroke
Clots
Cerebral aneurysms
Blood discrasias
Cavernous sinus syndromes
Intracranial hemorrhage
DIAGNOSIS
Labs:  protein s, protein c, antithrombin III, homocysteine, factor V Leiden, ESR, TFTs, thyroid autoantibodies
Imaging studies:  MRA, cerebral angiography
TREATMENT:  No definite treatment as for medication is available. Treatment and therapy are directed towards the complications of the disease.
Conservative Approach:
Intracerebral hemorrhage:  manage HTN
Severe stroke:  ICU monitoring until patient stable
Ischmeic stroke:  anticoagulation (coumadin or heparin) therapy to prevent further strokes
Consult with experienced neurologist for guidance
Angiogram to determine stenosis or occlusion
Surgical Approach:
STA-MCA--superficial temporal artery-middle cerebral artery anastomosis
EDAS--encephaloaduroarteriosynangiosis
EDAMS--encephaloduroarteriomyosynangiosis, pial synangiosis, and omental transplantation
CONSULTATIONS:
Neurological consult absolute must
Neuroradiology consult to determine what testing is needed
PROGNOSIS:
Outcome--dependent upon the extent and nature of hemorrhage
Prognosis--dependent upon the recurrence of the attacks
REFERENCES:
Hoch, D. U. S. National Library of Medicine, (2010). Circle of willis Bethesda, MD: Retrieved from http://www.nlm.nih.gov/medlineplus/ency/imagepages/18009.htm

Junath, M. (2009, December 19). Moyamoya disease [Web log message]. Retrieved from http://manju-imagingxpert.blogspot.com/2009/12/moyamoya-disease.html
Sekhar, L., & Kim, L. (2011). Neurological surgery clinical services moyamoya syndrome. Manuscript submitted for publication, Department of Neurological Surgery, University of Washington, Washington. Retrieved from http://neurosurgery.washington.edu/patientcare/clinicalservices/moyamoya.asp.
Suchoeiki, R., Kao, A. , & , Initials. (2011, Marc 23). Moyamoya disease. Retrieved from http://emedicine.medscape.com/article/1180952-overview.



Angiogram





MRI T1 weighted



JUVENILE ANGIOFIBROMA

JUVENILE ANGIOFIBROMA:  A benign tumor that is grows in the back of the nasal cavity, usually very aggressive and highly vascular. It is not very common. Most always found in adolescent males. Present with obstruction to one side and recurrent bleeding.
SYMPTOMS: 
1. Frequent nosebleeds or bloody mucus.
2. Nasal blockage, stuffy nose, or runny nose.
3. Obstruction of eustachian tube affecting the hearing.
4. Double-vision, d/t erosion into the cranial cavity placing pressure on the optic chiasm.
5. Recurrent ear infections, eye pain, and anosmia.
6. Difficulty breathing.
7. Easy bruising.
DIAGNOSIS: 
1. Submucosal mass located in posterior nasal cavity noted on physical exam.
2. CT.
3. MRI.
4. Arteriogram.
5. X-ray.
6. Biopsy may be necessary.
DIFFERENTIAL DIAGNOSIS:
1. Antro-choanal polyp--benign neoplasm.
2. Rhinosporidiosis--fungal infection of the nose.
3. Cancer.
4. Nasopharyngeal cyst.
5. Pyogenic granuloma.
TREATMENT: 
1. Surgical intervention, careful to minimize blood loss d/t the increased vascularity of the area.
2. Hypotensive anesthesia is the most common way of reducing blood loss.
3. Diethylstilbestrol is given 2-3 weeks prior to surgery to minimize blood loss.
4. Embolization via angiography may be performed to minimize blood loss.
5. Radiation therapy may be used for tumors that cannot be retrieved by surgery or have spread up into the cranial cavity, or where recurrence is the main issue.
6. Endoscopy may be used in conjuction with embolization.
7. Lateral rhinotomy is the most common treatment used today. The maxillary antrum is explored through an incision made along the lateral nasal border.
PROGNOSIS:
There is no prevention or cure. Some tumors may disappear on there own. Tumors may return after surgery.
COMPLICATIONS:
1. Anemia.
2. Pressure on the brain.
3. Spread of the tumor to adjacent structures or sinuses.
REFERENCES:
1. Angiofibroma definition: juvenile nasopharyngeal angiofibroma . (n.d.). Retrieved from http://www.health-res.com/juvenile-nasopharyngeal-angiofibroma.
2.Angiofibroma picture - photo gallery of angiofibroma ::disease gallery. (n.d.). Retrieved from http://health.bayaw.com/zoom/1464/Angiofibroma.
3. Juvenile angiofibroma. (2007). Retrieved from http://neuroradiologyonthenet.blogspot.com/2007_07_01_archive.html.
4. Juvenile angiofibroma angiogram. (n.d.). Retrieved from http://www.sciencephoto.com/images/download_lo_res.html?id=670034609.
5. Vorvick, L. (2009, September 9). Juvenile angiofibroma. Retrieved from http://www.healthline.com.



Angiogram