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



Orbital Cellulitis

Orbital Cellulitis:  An infection of the soft tissue that makes up the eyelids, eyebrow, and the cheek surrounding the eye itself. It has the potential to be very harmful and affect vision.
Causes: 
     Bacteria (Haemophilus influenzae) from a sinus infection.
     Staphylococcus aureus, Streptococcus pneumoniae, and/or beta-hemolytic streptococci.
     Bug bites, stye, or recent eyelid surgery.
     Seek medical attention immediately.
Symptoms: 
     Painful swelling of upper and lower eyelids, possible spreading into the cheek and eyebrow.
     Proptosis or exophthalmos (bulging eyes).
     Eye pain with movement.
     Difficulty moving the eyes.
     Fever-102 or higher.
     Discomfort.
     Shiny red or purple eyelids.
Tests:
     Blood cultures.
     CBC.
     Spinal tap in extreme cases of kids.
     Sinus x-rays and surrounding areas.
     CT or MRI of orbits and sinuses.
     Culture of throat and eye drainage.
     Throat culture.
Treatment:
     Hospitalization with IV antibiotics.
     Surgery may be necessary to drain the abscess or relieve the pressure.
     Infection may become increasingly worse very quickly. Close, frequent (every few hours) monitoring must performed.
Prognosis:  Complete recovery with prompt treatment.
Possible Complications:
     Cavernous sinus thrombosis:  A blood clot in the cavernous sinus at the base of the brain which returns deoxygenated blood to the heart from the brain.
     Hearing Loss.
     Septicemia.
     Meningitis.
     Optic nerve damage or loss of vision.
Contact Physician Immediately If:
     Swelling of eyelids along with a fever.
Prevention:
     Early treatment of sinus infections, dental infections, and other infections.
     Receiving the HiB vaccine will also help prevent orbital cellulitis.
References:
Garrity, J. (2008, September). Preseptal and orbital cellulitis. Retrieved from http://www.merckmanuals.com
Orbital cellulitis and cavernous sinus thrombosis. (2009, February 23). Retrieved from http://www.allina.com
U. S. National Library of Medicine, National Institutes of Health. (2011). Orbital cellulitis. Bethesda, MD, http://www.nlm.nih.gov

 

PROLACTINOMA

Pituitary Gland: "Master Gland." Important in regulating growth and development, metabolism, and reproduction.

Prolactinoma:  The most common type of pituitary adenoma. Based on postmortem autopsies, 25% of U.S. population have a small pituitary tumor. A benign tumor of the pituitary gland causing an excessive amount of the hormone prolactin (natural hormone that aids in mild production) to be secreted. Symptoms develop when the tumor grows, putting pressure on the surrounding tissues. Hyperprolactinemia is the condition when prolactin increases in the bloodstream.

Function:  Signals growth of breast tissue during pregnancy. Post pregnancy, the levels decrease unless the mother breastfeeds.

Symptoms Caused by Prolactinoma: 
     Women:  Ovulation interference, irregular menstrual cycles, and possible infertility. Non-pregnant, non-breastfeeding women may develop breastmilk. Decrease in sex drive may occur. Vaginal dryness may cause painful intercourse.
     Men:  Impotence most common. Often do not go to doctor until headache and vision problems develop.

Diagnosis: 
     Blood test:  detect elevated prolactin levels.
     MRI:  best test to detect and measure prolactinomas due to its sensitivity. Repeated periodically to monitor progression and effects of therapy.
     CT:  sometimes used, but not as effective as MRI.

Post-Diagnosis Tests:
     Comprehensive eye exam.    
Goals of Treatment: 
     Return of normal prolactin secretion.
     Reduction in tumor size.
     Restore normal vision.
     Restore normal function on the pituitary gland.

Treatment:
     Medications first, surgery considered if medications not tolerated or effective.
     Medications may be used in conjunction with surgery and/or radiation therapy.   
Medications:  Dopamine mimicking medications that inhibit prolactin secretion.
     Parlodel
     Dostinex

Surgical Treatment:  A very delicate surgery (performed by a neurosurgeon) involving an opening into the brain to access the tumor. This is often done by going through the roof of the mouth through the sphenoid sinus. Outcome highly depends on skill of surgeon, size of the tumor,  and prolactin level.

Prognosis:  With medications, sexual function and fertility will return.

References:

Ferry, R. Prolactinoma (pituitary tumor). (n.d) retrieved March 20, 2011, from MedicineNet.com Web Site: http://www.medicinenet.com/

Hurley, D. & K. Ho (2004). Pituitary disease in adults. retrieved March 20, 2011, from The Medical Journal of Australia Web Site: http://www.mja.com.au

Prolactin producing pituitary tumor. (n.d) retrieved March 20, 2011, from Health Resources Web Site: http://www.health-res.com/

Prolactinoma. (April, 2009). retrieved March 20, 2011, from lookforadiagnosis.com Web Site: http://www.lookforadiagnosis.com/

Prolactinoma. (2011). retrieved March 20, 2011, from University of Michigan Health System Web Site: http://www.med.umich.edu/



Coronal Image Prolactinoma

  

Sagittal Image Prolactinoma



Chiari Malformation

Chiari Malformation:  Congenital, anatomical anomaly of cerebellum, brainstem and craniocervical junction. The medulla oblongata and cerebellum protrude downward through the foramen magnum into spinal canal.

Different Types:
Type 0:  No herniation of cerebellum,  but there is some obvious crowding into the foramen magnum.

Type I--AKA--Arnold Chiari Malformation: Most common of all types, signified as a 5mm protrusion of cerebellum into the spinal canal, may or may not experience symptoms.

Type II:  Protrusion of brainstem (medulla oblongata) and ventrical into the spinal canal, commonly seen with spina bifida and meningocele. 

Type III:  Involves an area of the spinal canal that failed to close up, the cerebellum and brainstem protrude out through the opening. This type is rare, often resulting in death.  The ones who do survive suffer with severe neurological deficits, usually other birth defects, and often hydrocephalus.

Type IV:  Most rare of all types. The cerebellum does not fully develop and there is usually some other brain or medulla oblongata malformation that is present as well. Very rarely does person survive infancy.

Diagnosis:  Basic neurological testing (sensation, walking, reflexes, coordination, etc.) is obtained in conjunction with an MRI (the only modality that provides a definitive diagnosis.)

Prognosis:  It's difficult to determine how Chiari malformation will progress. Greater than 80% obtained improvement of symptoms and some were symptom free. In cases anatomical changes and brain damage, these effects were irreversible.

Symptoms:  Symptoms range from no symptoms at all to mild or moderate.
Some common symptoms may include but not limited to: headache, migraines, "lump in throat" feeling, burning sensation in upper extremeties, memory loss, decrease in muscle tone, back pain, ataxia, fatigue, dizziness, and decreased sensation of touch and temperature differences--arms and legs in particular.

Symptoms specific to Type II: Change in breathing patterns, depressed gag reflex, nystagmus (rapid movement of the eyes), upper body strength diminishes. These symptoms occur in conjunction with above symptoms.

Symptoms that require immediate medical attention include nausea and vomitting, sleep apnea, and dyshpagia.

Treatment:  Treatment of symptoms is beneficial for the most part depending on the type and severity of the symptoms. Surgical intervention is usually required at some point.

A Few Other Pathologies That May Be Involved: 
Hydrocephalus:  Abnormal accumulation of  CSF in the brain.

Ehler's Danlos:  A genetic disorder, affects the connective tissues throughout the entire body. The joints, skin, bones, vessels, organs, etc., have little or no strength structure to them.

Syringomyelia:  A chronic condition where a pocket (AKA-syrinx) forms within the spinal cord itself. Over time the pocket grows and compresses on the spinal cord causing neurological symptoms such as pain in back, neck, and upper and/or lower extremeites, weakness, headaches, etc.

Tethered Spinal Cord Syndrome:   The spinal cord is essentially tied to the inside of the spinal canal restricting movement. Eventually the spinal cord becomes stretched out causing neurological problems.

References:
Arnold chiari malformation and syringomyelia. (2011, January). Retrieved from http://www.chiariaustralia.com

Chiari malformation type 1.Retrieved from http://mtn.missouri.edu

Khouri, Chaouki. (2010, September). Chiari malformations. Retrieved from http://www.uptodate.com

Office of Communications and Public Liason, National Institute of Neurological Disorders and Stroke. (2011). Ninds chiari malformation information page Bethesda, MD: Retrieved from http://www.ninds.nih.gov/

Shinagare, A. B., & Patil, N. K. (2008). Imaging of congenital malformations of brain: a pictorial essay. The Internet Journal of Radiology, 9(1), Retrieved from http://www.ispub.com

Tew, John. M., & Nancy, McMahon. (2010, February). Chiari 1 malformation [Web log message]. Retrieved from http://www.mayfieldclinic.com

                                               

Infant with a meningocele.