Oncology | Craniopharyngioma

0:00Hi everyone, welcome back to the Med Bullets Step 2 and 3 podcast. In today's episode, we covered the topic of Cranio for Ingeoma, found under the oncology section at MedBullets.com. Let's begin with a clinical snapshot. A 68-year-old woman presents to the emergency room with profound hypovalemia. She is given IV fluids and also complains of a headache that has been plaguing her for a year, but attributed it to severe migraines. It is continuous, however, it seems to have gone worse recently. She also reports recent weight gain. On physical exam, she is notably slow-deep tendon reflexes with facial mixidema. Serum labs come back with hypoglycemia and hypercalemia. Concern for possibility of a pituitary lesion, she is sent for a CT scan of the head. Let's continue with an introduction to Cranio for Ingeoma. As a reminder, this refers to a benign brain tumor arising from Rathke's pouch.

1:05In terms of the epidemiology, this is the most common childhood super-tintorial tumor, and it demonstrates a bimodal distribution, affecting children between 5 and 14 years of age, and adults between 65 and 74 years of age. In terms of the pathogenesis, it is derived from remnants of Rathke's pouch. It is benign on histology, slow growing, and it may have a malignant behavior with invasion and local structures, so it may recur after a section, but metastases are rare. Also remember that the blood supplies typically from the anterior circulation of the brain. On tumor characteristics include dystrophic calcification, cyst formation, and hemorrhage, moving on to the presentation. Symptoms will most commonly include headaches, this may be progressive, dull, and continuous, and there may be papilladema from increased intracranial pressure.

2:05Patients may present with bytemporal hemianopsia, recall that the pituitary adenoma is present similarly, and this is from compression of the optic chiasm. They can compress the pituitary gland, which may cause hypopetuitarism. This may result in hypothyroidism, which may present with non-pitting adema, a slow return phase of deep tendon reflexes, weight gain, and fatigue. There may be adrenal failure, which can present with orthostatic hypotension, hypoglycemia, and hypercalemia, and then patients may also present with diabetes and syphilis, which will present with frequent urination. In terms of further imaging, a CT scan with contrast is used for diagnosis. This may demonstrate a supercellar calcified cyst, and MRI is used to assess the surgical approach. In terms of further studies, histology may demonstrate cholesterol crystals and calcifications,

3:06and one should assess endocrine function if it is suspicious. This would include serum electrolytes, serum in urine osmolality, thyroid studies, growth hormone levels, and LH and FSH levels. In terms of the differential, make sure to think about pituitary adenoma, glioma, megyloblastoma, and meningioma. In terms of treatment, options include surgical resection. If it is not completely resectable, then this should be followed with radiotherapy. In terms of the prognosis, remember that there is a better prognosis for patients that are less than 20 years of age, with the 5-year survival rate of about 99%. There is a poorer prognosis for older than 65 years, with the 5-year survival rate around 38%. And lastly, complications related to cranial foreigngeoma include vision loss. Now that we've discussed the major points relating to cranial foreigngeoma, let's walk

4:10through some questions to apply what we've learned and get a sense of how the topic might be tested. For the first question, consider the following clinical scenario. A 68-year-old woman with type 2 diabetes maladies, hypertension, and hypothyroidism presents to the emergency room with 6 months of worsening headaches and vision changes. Initially, her headaches were dull and infrequent, but they now occur daily and have become quite severe. She states that her symptoms are worse in the morning. They do not improve substantially with ibuprofen or acetaminophen. The patient requests prescription medication as she can no longer tolerate the pain. She has also been experiencing fatigue, nausea, increased thirst, and frequent urination. Her current medications include insulin, metformin, aturvastatin, lysinopril, hydrochlorothiazide, and levothyroxine.

5:11She confirms that she has been taking all of her medications as prescribed. She also reveals that her peripheral vision has been getting worse, though she thinks this may be due to old age. Her temperature is 97.9 degrees Fahrenheit or 36.6 degrees Celsius. Her temperature is 139 over 89, pulse is 82 beats per minute, respirations are 11 breaths per minute, and oxygen saturation is 98% on room air. Physical exam is notable for mild impairment of her lateral visual fields. A non-contrast CT skin of the head is performed, which demonstrates a calcified cystic mass in the supercellar region. Each of the following is the most likely diagnosis, and the answer choices are choice 1, berry aneurysm, choice 2, cranioferangioma, choice 3, meningioma, choice 4, pituitary

6:13macromatinoma, or choice 5, raffky cleft cyst. The best answer to this question is choice 2, cranioferangioma. This patient presents with new onset worsening headaches, polyureia, and polydipsia, which is concerning for diabetes and sypitis, and peripheral vision loss, which is concerning for bytemporal hemianopsia. These are all findings suggestive of a growing intracranial mass in the cellar region. The un-enhanced head CT demonstrates a supercellar calcified cystic mass, which is most consistent with a cranioferangioma. Cranioferangioma is often grow slowly and compress the pituitary and optic chiasm, classically causing panhypopetuitarism, due to compression of the pituitary gland. Central diabetes and sypitis, due to low EDH secretion, resulting from compression of

7:15the pituitary stock, or hypothalamus, bytemporal hemianopsia, due to compression of the optic chiasm, and headaches in nausea, due to obstructive hydrocephalus, and increased intracranial pressure from compression of the ventricular system. Central diabetes and sypitis occurs secondary to decreased EDH production, and can present increased urination. So panhypopetuitarism is a possible presentation, notably it is not easily diagnosed. Finally, cellar masses typically present with vision changes, increased intracranial pressure, and central diabetes and sypitis. Let's also discuss whether the other choices are incorrect. Choice one, very aneurysms can present with symptoms of cranial nerve or brainstem compression. On CT, an aneurysm appears as a well-defined, round, hyper-atenuating lesion.

8:16Choice three, meningioma typically presents with seizures in a young woman in her thirties. meningiomas appears dual-based masses, and demonstrate contrast enhancement on CT. Choice four, pituitary macrosetanomas present with hormonal imbalance and mass effect, typically on the optic chiasm. Adenomas typically have attenuation similar to brain tissue, demonstrate moderate contrast enhancement, and rarely are calcified. Choice five, rathky cleft cysts are supercellar epithelium line cysts, and are embryologic remnants of rathky's pouch. Typically, they are asymptomatic and discovered incidentally as non-enhancing and non-calcified masses. Finally, a bullet summary. Cranial foreign geomas often compress the pituitary stock and optic chiasm, causing severe headaches, panhypopetuitarism, and bytemporal hemianopsia.

9:18For the second question, consider the following clinical scenario. An 11-year-old boy is brought to the pediatrician by his mother for vomiting. The patient has been vomiting for the past week, and his symptoms have not been improving. His symptoms are worse in the morning, and tend to improve throughout the day. The patient also complains of occasional headaches, and had diarrhea several days ago. The patient eats a balanced diet, and does not drink soda or juice. The patient's brother both had diarrhea recently that resolves spontaneously. His temperature is 99.5 degrees Fahrenheit, or 37.5 degrees Celsius. Blood pressure is 80 over 45. Pulse is 90 beats per minute, respirations are 16 breaths per minute, and oxygen saturation is 98% on room air. On physical exam, the patient appears to be in no acute distress.

10:18Cardiopulmonary exam reveals a minor flow murmur. Neurological exam reveals cranial nerves 2-12 as grossly intact, with mild narrowing of the patient's visual fields. The patient's gate is stable, and he is able to jump up and down. Which of the following is the most likely direct cause of this patient's presentation? In the answer choices are, choice one, a non-enveloped, positive sense single-stranded RNA virus. Choice two, gram-negative micro-arrophilic bacteria. Choice three, gram-positive interotoxin. Choice four, intracerebeller mass. Or choice five, remnant of wrath-kes-pouch. The best answer to this question is, choice five, remnant of wrath-kes-pouch. This patient is presenting with signs of increased intracranial pressure, and a narrowing

11:20of his visual field, suggesting a diagnosis of cranial for angioma. Cranial for angiomas are remnants of wrath-kes-pouch. Cranial for angiomas can present with signs of increased intracranial pressure, including headache and vomiting, which is typically worse in the morning and improves throughout the day. Other specific symptoms of this cranial mass include bytemporal hemianopsia, which occurs when the mass compresses the optic chiasm. These patients should have their head imaged with the CT or MRI and should be treated surgically. These also discuss whether their choices are incorrect. Choice one, a non-enveloped positive sense single-stranded RNA virus, describes neurovirus, which can present with vomiting and diarrhea. Choice two, gram-negative micro-arrophilic bacteria represents Campalobacterja juni, which can cause an acute diarrhea illness. Choice three, gram-positive interotoxin represents the exotoxin produced by Staphylococcus

12:25Aureus, which classically causes food poisoning, which would present with vomiting and diarrhea. Choice four, intracirabella mass represents a diagnosis of mengeloblastoma, which would present with the cerebellar symptoms such as a taxia, abnormal finger-to-nose testing, and hielshin tests, rather than a narrowing of the visual field. Finally, a bullet summary. Neoporingeoma is a remnant of Rathge's pouch that can present with signs of increased intracranial pressure and compresses the optic chiasm, causing bytemporal hemianopsia. That's all for this review about cranioferingeoma. We hope that was helpful. This is the Med Bullets Step Two and Three podcast, a daily audio review session for Med Bullets, the free learning and collaboration community for medical student education. As a reminder, you can follow along with these podcast episodes by reviewing the topics directly on medbullets.com.

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