| Author | 20 Posts |
dr_jojo
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29. A 16-year-old boy with neurofibromatosis is brought for a follow-up examination. His uncle also has neurofibromatosis. He has a 1-year history of headaches during which his parents say he appears pale. Six months ago, he underwent operative treatment for an optic nerve glioma. His blood pressure is 164/105 mm Hg, pulse is 102/min, and respirations are 14/min. The thyroid glands are not enlarged. No murmurs are heard, and radial pulses are equal. Abdominal examination shows no abnormalities. Which of the following is the most likely cause of this patient's high blood pressure?
A
) Catecholamine-producing tumor (pheochromocytoma)
B
) Carcinoma of the thyroid gland
C
) Essential hypertension
D
) Overproduction of aldosterone from an adrenal adenoma
E
) Postsubclavian coarctation of the aorta
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| Aashi
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C) Essential hypertension
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| huyniid
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A. This pt is belonged to MEN-2B NF+Pheo
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| robin082006
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A
Neurofibromatosis------>high risk neuroblastoma------> hypertension
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| doc_clotaire
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I agree with A
should be evaluated for MEN SD
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| eaguin
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a
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| Aashi
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How do u classify this as a case of MEN 2B----->has involvment of thyroid( medullary CA--UNIQUE),MUCOSAL NEUROMAS( on the lips ,cornea,mouth, G.I( diarrhea) and pheochromocytoma--->All MEN 2B guys has MARFANOID HABITUS( thin body with long and slender arms pectus excavatum etc)..the presence of just HTN and 2 point increase in HR doesnt explain this as pheochromocytoma, Nor MEN 2B--when all other syndromes are negative,--->this guy has a normal thyroid( infact MEN 2B pts has to get their thyroid removed by the age of 5) and pheochromocytoma itself presents as sweating, palpitation, headache, tachycardia, tremors and episodic HTN and typically the pt feels that as if they he/she is going to die, a sense of doom..not so subtle like this pt
HTN in NF can be seen at ANY AGE-->and the MCC is essential HTN, but pheochromocytoma( JUST 1% OF NF has this) and renal artery stenosis due to fibromuscular dysplasia , Coa Aorta also has to be ruled out,coz even this 2 can cause HTN in NF..but the s/s mentioned above is not enough TO DEFINE this as Pheochromocytoma/renal artery stenosis or COAorta or a MEN 2B
So I stick with C
GL
Edited by Aashi on 03/12/07 - 08:58 PM
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| robin082006
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A fore sure
Neuroblastoma is one of pheochromocytoma.
This patient has neurofibromatosis---->chance of neuroblastoma
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| huyniid
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Essential HTN age : 25-55, this pt is too young. When I check in the pheochromocytoma section, CMDT :
Familial pheochromocytomas are usually bilateral (70% of cases) and may be associated with the following: calcitonin-secreting medullary thyroid carcinoma and hyperparathyroidism (MEN type 2), medullary thyroid carcinoma and the syndrome of multiple mucosal neuromas (MEN type 2B), neurofibromatosis (Recklinghausen's disease), and islet cell tumors (rare)
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| Aashi
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Robin, neuroblastoma is uniquely found in infants ( MC TUMOR OF INFANCY) max dx by the age of 5 yrs. By 1 yr of age they have mets all over,which carries worse prognosis...and the MC presentation is an abd mass..THis guy is 16 yrs old...and he doesnt have any abd mass...This case is higly UNLIKELY of neuroblastoma..He cud be a pheochromocytoma,its just that i dont support the s/s that the pt has,is typical of pheochromocytoma...And if u have read somever abt neuroblastoma in adolesence pls let me know the source,so that i can correct myself
.
GL
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| Aashi
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Dear Huyniid ..you are not getting the point i am trying to make..You are telling me this as a case of MEN2B..when u clearly mention from an extract of CMDT that Medullary CA of the thyroid is a part and parcel of this particular syndrome and if u read the q u will know that this guy has got a perfectly normal thyroid..medullary ca is so aggressive that these guy has to remove the thyroid by the age of 5 yr with radical dissection of l.n and neck..this guy is 16 yr old..MEN2b is a syndrome,and just the presence of NF doesnt make it a part of this syndrome and HTN in NF is NOT uniquely due to phemochromo.,its just seen in 1% of NF..and yes i do agree with u ,that this guy is young and secon HTN is MC in this age gp..HTN in NF can be caused by renal artery stenosis and CoAorta.not just Pheochromo..but the P/EX doesnt fit for this at all
If the q gave a clue abt thyroidectomy at age <5,and marfan like habitus then i will surely go for pheochromo thinking abt MEN2B..with such limited info from the q regarding MEN2B,i just cant justify this as MEN 2B ..and thatz y i picked C..
Hope i got through u this time..and if u got any new info,plss paste it down..again this is an NBME q,so we can just assume that our answers are right..
GL
Edited by Aashi on 03/12/07 - 11:57 PM
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| robin082006
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In patient with neurofibromatosis, neuroblastoma can occur at any age.
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| Aashi
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wow robin ..I knew that malignant neoplasms can appear at any age in NF,but i dint know abt neuroblastomas occuring at the age of 16..anyways,it isnt neccessarily required that i have to know everythin..
but i would like to know the source...in infancy they r presented as an abd mass,with wt loss, bone pain etc .what abt adulthood,this guy has no palpable mass in the abdomen and noneof the signs that this guy has got a tumor...any input on this..
Thanks and GL
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| huyniid
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Asahi@
Thank you for your discussion which inspired me much for reading more about the fact. I agree with you about the point that MEN2B is quite severe if it has Medullary Thyroid CA as in its definition.
The problem here is : this is an NBME question with vague data as you stated, and we the examinee should stick with the common sense.
I reread the Qs and get the point of No thyroid enlargement.
I think I was wrong !!! High possibility of Essential HTN
Thanks
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| vanshita
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complication of neurofibromatosis Blindness caused by a tumor in an optic nerve (optic glioma)
Malignant transformation of tumors (in approximately 5-10% of affected individuals)
Chance of pheochromocytoma, which causes very high blood pressure
Loss of nerve function in nerves where a neurofibroma's growth has caused prolonged pressure on the nerve
Attention deficit hyperactivity disorder (ADHD)
Cosmetically significant tumors of the face, skin, and other exposed areas
Bowing or breakage of the leg bones with poor healing
Scoliosis or curvature of the spine
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| krsna
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I thought there was a increased chance of getting phaeochromocytoma in a pt with neurofibromatosis....i still think the ans is A
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| NE
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AAshi is right- MCC of HTN in neurofibromatosis is ESSENTIAL HTN!
From emedicine.com
"Hypertension is frequent in patients with NF1 and may develop at any age. In most patients, the hypertension is essential, but vascular dysplasia can occur in association with NF1 and may produce renal artery stenosis, coarctation of the aorta, or other vascular lesions associated with severe hypertension in adult patients with NF1. Pheochromocytoma can cause severe hypertension in patients with NF1.”
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| fongch
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But essential HTN cannot explain the 1 year history of episodic headache and pallor.
NE wrote:
AAshi is right- MCC of HTN in neurofibromatosis is ESSENTIAL HTN!
From emedicine.com
"Hypertension is frequent in patients with NF1 and may develop at any age. In most patients, the hypertension is essential, but vascular dysplasia can occur in association with NF1 and may produce renal artery stenosis, coarctation of the aorta, or other vascular lesions associated with severe hypertension in adult patients with NF1. Pheochromocytoma can cause severe hypertension in patients with NF1.”
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| selsibeel
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"About two in 10 pheochromocytomas runs in families or is associated with an inherited condition, such as:
* Multiple endocrine neoplasia, type II (MEN-II). In addition to a pheochromocytoma, people with MEN-II also have thyroid cancer. Other forms of MEN-II include pheochromocytoma with thyroid cancer and hyperparathyroidism (MEN-IIA), and pheochromocytoma with thyroid cancer and tumors of nerves in the lips, mouth, eyes and digestive tract (MEN-IIB).
* Von Hippel-Lindau (VHL) disease. People with this rare multisystem disorder are at high risk of pheochromocytoma, kidney cancer, and brain and eye tumors.
* Neurofibromatosis 1 (NF1). Pheochromocytomas can occur in a small percentage of people with NF1, a syndrome that includes multiple tumors in the skin (neurofibromas), pigmented skin spots, tumors of the optic nerve of the eye, and bone lesions.
Most pheochromocytomas are noncancerous (benign) and don't spread to other parts of the body. However, cancerous (malignant) pheochromocytomas can spread throughout the body, often to the brain, lungs or bone."
Due to the information that I found on Health Library --> I think the answer is A.
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| RX 135
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nf with htn
1.child--wilms
2.adult-pheochromo..
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