DX: Suprasellar mass (Hemorrhagic Rathke's Cleft Cyst versus Craniopharyngioma)

Well, this is the first case that I post without a final diagnosis. Sorry for the delay but I had no time to prepare the conclusion and all of your comments on FB were remarkable and taken in consideration. Thank you!

First, I know..it's kind of frustrating to not have the actual diagnosis, but at the same time is a challenging excercise because one has to search almost all variables in a pathological entity in order to conclude in a definite diagnosis. Therefore, every possibility is valid in first instance. Now let's take a quick review..

The mass has a suprasellar origin, with a regular outlined border and almost entirely cystic component, has a blood-fluid level inside with a peripheral rim calcification.

Ok differentials regarding a suprasellar mass are contained in the acronym SATCHMO wich is a mnemonic wich stands for:

S: sarcoid, sellar tumour (pituitary adenoma)
A : aneurysm
T : teratoma
C : craniopharyngioma, cleft cyst (Rathke) , chordoma
H : hypotalamic glioma, hamartoma of tuber cinereum, hystiocitosis
M : meningioma, mets
O : optic nerve glioma

Some prefer adding an E at the end and moving 'histiocytosis' to this last letter as
E : eosinophilic granuloma

Over all, we have to choose those with cystic (or almost cystic) component and with the clinical presentation here are the most likely --> pituitary adenoma (macroadenoma and pituitary apoplexy), aneurysm, craniopharyngioma, rathke's cleft cyst.


1) Pituitary Macroadenoma
Pituitary macrocadenomas are the most common suprasellar mass in adults, and responsible for the majority of transsphenoidal hypophysectomies. They are defined a as pituitary adenomas greater than 10mm in size and are approximately twice as common as microadenomas.

Patients typically present with symptoms of local mass effect on adjacent structures (especially optic chiasm). Some may present hormal imbalance, with symptoms of hypopituitarism (from compression) or secretion. Hormonal imbalance due to overproduction tends to present earlier and tumours are thus usually small at presentation.

CT
No contrast attenuation can vary depending on haemorrhagic, cystic and necrotic components. Adenomas which are solid, without haemorrhage, typically have attenuation similar to brain (30-40HU) and demonstrates moderate contrast enhancement; less marked than one typically sees in meningiomas. Calcification is rare.

1.1) Pituitary apoplexy
Is the sudden enlargement of a pituitary adenoma, is difficult to diagnose. Sudden infarction, necrosis, or hemorrhage into a pituitary adenoma may be threatening to both vision and life if not treated promptly. Is characterized by the abrupt appearance of severe headache, nausea and vomiting, stiff neck, agitation, mental changes, visual loss and/or diplopia.

Click image to enlarge

2) Craniopharyngioma
A craniopharyngioma is a type of relatively benign (WHO grade I) neoplasm which typically arises in the sellar / suprasellar region. They account for ~ 1 - 5 % primary brain tumours. Craniopharyngiomas are typically very slow growing tumors. They arise from the cells along the pituitary stalk. They are classified by histology as benign; however, as with many brain tumors, their treatment can be difficult, and significant morbidities are associated with both the tumor and treatment.

There is a bimodal distribution, with the first peak between the ages of 10 - 14 years and a second peak in the 5th to 7th decades. There appears to be a similar incidence in both males and females.

There are two main (quite different) pathological types :

• adamantinomatous (paediatric)
• papillary (adult)

Adamantinomatous
This type is seen predominantly in children. It consists of reticular epithelial cells which have appearances reminiscent of the enamel pulp of developing teeth.

There may be single or multiple cysts filled with thick oily fluid high in protein, blood products, and/or cholesterol, creating the so called "machinery oil". "Wet keratin nodules" are a characteristic histological feature. Calcification is usually present : ~ 90%.

Papillary
The papillary sub type is seen in adults and is formed of masses of metaplastic squamous cells. "Wet keratin" is absent. Cysts do form, but these are less of a feature, and the tumour is more solid. Calcification is uncommon.

CT
Typically seen as a heterogeneous mass in the suprasellar region. Calcification (except for the papillary type) and cysts are very common:

Calcification
overall 80 - 87% of craniopharyngiomas are calcified, they occur in a stippled pattern
adamantinomatous : 90%
papillary : rare 4

Cysts : Seen in 70 - 75% of cases (more frequently in the adamantinomatous type)

Click image to enlarge

3) Rathke Celft Cyst

Rathke’s cleft cysts are derived from or are remnants of Rathke’s pouch. Sellar and juxtasellar epithelial cysts comprise a spectrum of lesions that range from craniopharyngioma (aggressive and complex) to Rathke’s cleft cyst (simple and indolent).

Intra- and suprasellar in location, Rathke’s cleft cysts have welldefined margins and midline location. The thin walls are lined by a single layer of epithelium. Mucin-secreting goblet cells may be present. Depending on the activity of the goblet cells and the rate of epithelial desquamation, the material within the cyst ranges from serous to mucoid. This variation in content is reflected on CT, where the attenuation is low (serous) to intermediate (mucoid).

On imaging a Rathke’s cleft cyst is seen as a well defined non enhancing midline cyst within the sella arising between the anterior and intermediate lobes of the pituitary. 40% are purely intrasellar and 60% have suprasellar extension. Purely suprasellar location, although reported, is rare.

CT

Non contrast : it is typically non-calcified and of homogenous low attenuation. Uncommonly it may be of mixed iso- and low attenuation, or contain small curvilinear calcifications in the wall (seen in 10 - 15% of cases)

Post contrast : Typically non enhancing although the cyst wall may enhance in some cases.

Click image to enlarge 

Aneurysm and Cystic Astrocytoma was originaly thought also but as we peer reviewed them was discharched for lack of clinical and imaging foundation.

So to conlude now we have a 75% confident that is a Rathke Cleft Cyst. The only thing that doesn't favor is the calcified rim wich is unusual. Craniopharyngioma is infrecuent as a cystic component (adamantinomatous) on adulthood but I presented a case of a 39 y/o with cystic characteristics, so that's why has to be on the differential.


Thank's to all who participated in the discussion, specially Dr. Wael Nemattalla, Dr. Imran Jindani, Dr. Lijesh Kumar, Dr. Anhuba Pandey (who reminded me to take in consideration Rathke's), Dr. Naweed Ashraf, Dr. Samuel Inbaraja.

CASE 9: 52 y/o male with progressive loss of sight and refractory headache

This next post has only 1 image and has NO final diagnosis, so any differentials may be correct. Unfortunately patient wasn't from our hospital and was shown to me by a friend just to have a second opinion on diagnosis.

This was previously posted by me on FB group COBRA.

Click to enlarge:

Differentials please. Later on I will give my top 3 differentials.

DX: Biloma secondary to Gangrenous cholecystitis

Ok.This case was pretty laborious due to the fact that the clinician was depending on my appreciations to initiate theraphy. As I mentioned previously, US was very tedious because somehow patient did not cooperate and prominent kyphosis worsened the picture. CXR revealed some subphrenic air levels and elevated right hemidiaphragm, so clinician was suspecting hepatic abcess. Findings were a little misleading, did not agree with that impression but however I wasn't giving more options as well. CT scout revealed the same CXR impressions.

CT findings are remarkable and are consistent with pericholecystic fat stranding; enlarged, thick and irregular walls of gall bladder all of them pointing at Acute Cholecystitis without a doubt, but the most intriguing finding was a fluid collection lateral and adyacent to caudate lobe of the liver. It had dense liquid HU and was in the lesser sac. As one comment stated, could be secondary to pancreatitis but pancreas was spare.

What was it?..Ussually a complicated inflammatory process (probably a gangrenous state cholecystitis in this case?) develops fluid collection as an abcess. But in this case had no enhancing wall, relatively low peripheral inflammatory reaction and no air-fluid levels that are consistent with an abcess. So therefore fluid collection near or around biliary tree had to be a Biloma (had to be??..hehe).

Post-op note reported 100 cc of biliary fluid content (Biloma) and a gangrenous (perforated) gall bladder with active leakage.


Not the usual presentation of a biloma and literature only reported 1 case similar to mine. Here are the images:

This article by the AJR described this MRI images as a lamba-shaped fluid collection adyacent to caudate lobe representing a biloma. See the ressemblance to our case in the first image.

So remember, Bilomas are often seen following biliary tract surgery although they can also occur after trauma or erosion into the biliary tree from a malignant or inflammatory process.

Thank you for your time..comments are welcome!

CASE 8: 78 y/o male patient with right upper cuadrant pain

This case is quite interesting..not as impressive as the previous one but yet interesting...feel free to comment.

Here are the CT images. Patient was diabetic with RUC pain, fever and mild jaundice. US was done but pronunciated dorsal kyphosis made it difficult to evaluate, so therefore CT was recommended.

Click to enlarge:

Soon the conclusion..

DX: Extra-adrenal pheochromocytoma (Paraganglioma)

Ok thank you for your comments. It was the first time that this blog reaches so far countries overseas..ok here is the conclusion.

Patient with long standing recurrent hypertension. CT was made first to discard renal artery stenosis as a cause of refractory hypertension. The mass was an incidental finding that turned out to be the actual cause of her illness.

It was an heterogeneous mass with irregular borderline, highly vascular and rapid enhancement with slow washout, located in the left retroperitoneum, lateral to aorta and anterior to psoas muscle along the Zuckerkandl organ. It had a nurturing artery from the posterior aspect of aorta below renal arteries and a draining venous vessel in direction of ipsilateral iliac vein. Renal arteries are spare.

Here is the descriptive image:

Pheochromocitoma has some imaging characteristics but is known as a "chamaleon" for it's wide variations of presentation and location. They are tumors arise from the chromaffin cells of the adrenal medulla and are associated with increased catecholamine production (cause of hypertension). Although chromaffin tissue is also present elsewhere in the body, such as in the mediastinum, along the aorta, and in the pelvis, the term pheochromocytoma is reserved for tumors that arise from the adrenal medulla. Chromaffin cell tumors at other locations are more appropriately called paragangliomas or chemodectomas, although the term extra-adrenal pheochromocytoma is still applied. Pheochromocytoma has been called the 10% tumor because approximately 10% are bilateral, 10% are malignant, 10% occur in children, and 10% are extraadrenal. Malignancy is usually established by local invasion or metastases to nonchromaffin tissues.

Extra-adrenal pheochromocytomas arise also from the sympathetic paraganglia. Sympathetic ganglia are found predominantly in the paraaxial region of the trunk along the prevertebral and paravertebral sympathetic chains and in the connective tissue in or near the walls of pelvic organs. They are associated with the celiac, superior mesenteric, and inferior mesenteric ganglia, which are retroperitoneal in location. The organ of Zuckerkandl is the only macroscopic extraadrenal sympathetic paraganglion, located at the origin of inferior mesenteric artery. Sympathetic ganglia are particularly numerous along the fibers of the inferior hypogastric plexus, leading to and entering the urogenital organs.

AV Malformation and Sclerosing mesenteritis were the differentials given. AVM ussually has more gastrointestinal hemmoraghe manifestations but is a good possiblity, although the presence of refractory hypertension almost rules out this option.

Sclerosing mesenteritis is part of a spectrum (including mesenteric lipodystrophy and mesenteric panniculitis) of idiopathic primary inflammatory and fibrotic processes that affect the mesentery. Pathophysiologically, these processes may affect the integrity of the gastrointestinal lumen and mesenteric vessels by a mass effect. This is not the case.

We have to rely on bloodwork and urine analisis to establish diagnosis so recommend plasmatic metanephrine, vanil mandelic acid and a  24hrs-urine cllection of catecolamines.


http://emedicine.medscape.com/article/379861-overview
http://www.ajronline.org/content/184/3/860.full
http://www.uptodate.com/contents/sclerosing-mesenteritis



Thank you for your comments and suggestions are welcome!

CASE 7: mid 40's female ongoing CT scan..history witheld

Ok here's the next case..this case was provided from the prestigious Tecniscan Diagnostic Center in Guatemala City, with a Siemens Somatom 64 slice CT scan.

This are the images..feel free to click to enlarge:

Describe and conclude in 3 differential diangosis..later on I'll post the conclusion with the history of patient.

Visit from Dr. Bijan Bijan to Guatemala

Recently we had a surprise visit from California, it was Dr. Bijan Bijan who met Dr. Francisco Arredondo (world renowned guatemalan radiologist) in the past RSNA annual meeting that takes place every year in Chicago. Apparently he was going to Central America for vacation purposes but then decided to do a couple of lectures that's why he contacted our Chief of Department Dr. Otto Orellana here in Centro Medico Militar in Guatemala City.

He spend 2 days in Guatemala (3 maybe) in wich he visited the mayan city of Tikal and the colonial city of Antigua. We recieved an excelent dissertation on the use of MRI in acute abdominal pain and Fetal MRI.

Dr. Bijan is a board certified radiologist with training  in cross-sectional Body Imaging/MRI & Nonvascular Interventional Radiology, Montreal General Hospital, McGill University, Quebec, Canada. Member of the Radiology Society of North America; American Ray Roentgen Society; Society of Nuclear Medicine; North American Society for Cardiac Imaging; Society of Thoracic Radiology; International Society of Magnetic Resonance in Medicine.

Here is Dr. Bijan in his lecture:

Here's with the group of residents..I'm on the right of Mr. Bijan:

Visiting our facility and giving and excelent presentation on Fetal MRI:

On the final day, outside our medical facility:

From left to right, back row: Dr. Geovanni Mendez MD; Dr. Edgar Javier Salguero MD (me); Dr. Erick Polanco MD (Deputy Chief of Department); Dr. Otto Orellana MD (Chief of Department); Dr. Bijan Bijan MD, MBA (distinguished guest); Dr. Cesar Milla MD; Dr. Juan Pablo Sanchez MD.

Front row from left to right: Dr. Angela Maselli MD; Dr. Cindy López MD; Dr. Ester Ponce MD; Dr. Ana Caballeros MD.

We are all grateful that he used his vacations to share his knowledge to all of us..for that we thank you.

http://www.sutterhealth.org/findadoctor/dr-bijan-bijan-md-22388.html

DX: Diffuse Idiopatic Skeletal Hyperostosis (DISH)

Ok thanks again for visiting my blog..you can also comment on my Facebook or Twitter account if it's too tricky to suscribe here..anyways, here is the conclusion of another musculoskeletal case that's quite interesting due to the fact that it's not commonly diagnosed, not because of rareness but for misleading findings often commited.

Here are the images once again: (click to enlarge)

Yellow arrows depict three findings: 1) Osteophyte formation ; 2) Endplate vertebral sclerosis; 3) Normal intervertebral space. May I add another one important: calcification of anterior longitudinal ligament.
Red arrow shows calcification of the nuchal ligament.

These findings are almost always diagnosed as osteodegenerative changes aso known as Degenarite Joint Disease (DJD). But there are main features that onse has to consider in order to diagnose DJD.

Brant & Helms great book "Fundamentals of Diagnostic Radiology" define DJD when encounter 3 aspects: 1) Osteophyte, 2) Bone sclerosis, and 3) NARROWING of joint space..if one of these are missing then other differentials need consideration.

The other features of this case are calcification of soft tissue (nuchal ligament and anterior longitudinal ligament [ALL]). In younger patients, we find calcification of ALL in ankylosing spondylitis but in older population as in this case, we refer to it as DISH.

Classic hallmark of DISH include:

• Flowing calcifications and ossifications along the anterolateral aspect of at least 4 contiguous vertebral bodies, with or without osteophytes.
• Preservation of disk height in the involved areas and an absence of excessive disk disease.
• Absence of bony ankylosis of facet joints and absence of sacroiliac erosion, sclerosis, or bony fusion, although narrowing and sclerosis of facet joints are acceptable.

Here's a picture from Brant & Helms book showing a case of DISH. (picture is property of authors and is shown for educational purposes).


Check this link to more detailed information
http://emedicine.medscape.com/article/388973-overview#a19




Thanks again for visiting be sure to check older posts and stay alert for newer ones..comments welcome....blessings

CASE 6: 65 y/o male with chronic cervical and back pain

Ok here is a musculoeskeletal case..hope you all comment!. There is no more clinical history, no trauma acute or chronic, no associated disease. Click on image to better view.

Soon I'll post the conclusion.

DX: Acute-hyperacute stroke of the right MCA, M2 territory

Ok here's the conclusion of the case of an elderly patient with history of sudden loss of conciousness and seizures with a low Glasgow coma scale. This happened 3 hours ago according to a relative that came with patient.

As you all noticed it was divided in two parts. The first one was a complete CT brain scan with no IV contrast. The second part was a single axial brain slice of a Diffusion Weighted Image (DWI) sequence. The whole point of dividing in two parts was to compare subtle CT findings. As I wrote in the previous post, the indication of the DWI study was because of "ambiguity" of those findings.

Here are the CT findings (click in image to enlarge):

a.

In picture (a) we see a bright hyperdense round image in topography of M1 MCA just after leaving the Willis circle. This is what is called a "hyperdense MCA sign" or simply MCA dot sign; it manifests because there's an increased amount of intraluminal blood pressure due to an acute distal trombus. This sign has high specificity but with poor sensibility. It may also seen in the presence of a high hematocrit level or MCA calcification but in both are usually bilateral.

b.

Picture (b.) shows loss of differentiation of white and gray matter in the right insular lobe. There is also associated effacement of sulci and diffuse hypodensity. This is called the "insular ribbon sign". Also a sign seen in acute infarcts.

c.

As it shows in (c.) there is a hipodensity or attenuation of right lenticulostriate nucleus with loss of differentiation of gray and white matter that extends to cortical and subcortical regions of temporal lobe with involment of sulci and gyri due to subtle citotoxic edema.

This are all early CT signs of acute ischemia in the territory of  right MCA. But as the signs are barley noticeable, they asked for a more specific diagnostic tool, so I suggested a DWI sequence on MR. Neurosurgeon agreed.

DWI as I explained in a commentary on an article posted some weeks ago, has a high sensitivity to acute and hyperacute brain ischemia because shows restricted diffusion to water particles in areas where there is no blood perfusion and it's manifested with a high intensity signal.

Here is the comparison between CT and DWI. As you see, DWI is more elocuent.

So, early ischemic CT signs can be useful when there is no MR, or when antithrombotic therapy has to be introduced as quick as possible. It needs a bit of a detailed image analysis and a good correlation with clinical data. Remember, time is brain.


Here are some great articles that can be helpful:








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