Solid Pseudopapillary Tumor of Pancreas

Solid Pseudopapillary Tumor (SPT) is a rare, benign, exocrine pancreatic tumor. It is seen in African or Asian females in 2nd or 3rd decade usually. These tumors can be asymptomatic or may present with a gradually enlarging painless mass in abdomen (as in this case), or with vague abdominal pain or discomfort.

SPTs are well encapsulated lesions with varying degrees of hemorrhage, necrosis and cystic degeneration which make them heterogeneous in both plain CT and post contrast images, with hemorrhage and capsule being the most important distinguishing features from other pancreatic neoplasms. 

The larger lesions have peripheral solid areas and central necrotic / cystic areas. The smaller or early lesions can be entirely solid, and can be difficult to differentiate from islet cell tumors. Some even can have internal septations in the cystic areas adding to the confusion.

SPTs are usually benign, but occasionally can rarely have local aggressive features, capsular invasion, metastasis(liver), lymphadenopathy. These aggressive tumors are more seen in older patients with a male gender predilection. Benign SPTs are usually completely cured by surgery. Long term survival is better with surgical treatment even in the presence of liver metastasis.¹

The following case is a 25yr, female with complaints of feeling of mass in abdomen.

Above CT topogram shows a soft tissue density mass lesion in the left upper quadrant, with compression on stomach and displacing the distal transverse colon and splenic flexure inferiorly. No obvious calcifications detected. No rib erosions / lysis / widening seen. DDs would include Renal, Pancreatic, Adrenal, Gastric lesions. If age is given, possibility of SPT of pancreas should be higher in the list of DDs. This can be a good opening image for FRCR 2B viva discussion.

 

Plain CT images show heterogenous lesion involving tail of pancreas, showing attenuation values ranging from 15-25HU in hypodense areas and rest showing mostly 40-45HU. No obvious hemorrhagic density was seen. Small areas of capsular calcfications were noted in the posterior aspect mainly.

The classic 'Claw Sign' in Radiology.

Post contrast images showing gradually increasing enhancement in few peripheral areas in the lesion. The hypodense areas in plain study showed no significant enhancement.

Lesion was showing well defined margins, with mass effect on adjacent structures and without any evidence of perilesional infiltration.This was reported as SPT.  Patient underwent splenectomy and distal pancreatectomy. Pathology report came as SPT.





References:
1: Solid-pseudopapillary tumor of the pancreas: a surgical enigma?
Martin RC, Klimstra DS, Brennan MF, Conlon KC.
Ann Surg Oncol. 2002 Jan-Feb;9(1):35-40. PubMed PMID: 11833495.

2: Solid Pseudopapillary Tumor of the Pancreas: Typical and Atypical Manifestations.
Jin-Young Choi, Myeong-Jin Kim, Joo Hee Kim, Seung Hyoung Kim, Joon Sok Lim, Young Taik
Oh, Jae-Joon Chung, Hyung Sik Yoo, Jong Tae Lee, and Ki Whang Kim.
American Journal of Roentgenology 2006 187:2, W178-W186.

3: Solid-Pseudopapillary Tumor of the Pancreas.
Kristin M. Coleman, Michael C. Doherty, and Steven A. Bigler.
RadioGraphics 2003 23:6, 1644-1648.

4. CT and MRI of the Whole Body, John.R.Haaga, 6th Edition.

What's your diagnosis? : Case 4 [June 25th, 2017]

25y female, feeling of mass in abdomen.

Post your answers as comment. Case answer and discussion will be posted in few days.

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What's your diagnosis? : Case 3 [June 25th, 2017]

Post your answers as comment below. Answer and brief discussion will be posted in few days.

RARE : Extreme case of bilateral massive renal replacement lipomatosis

Renal Replacement Lipomatosis (RRL) a.k.a Replacement fibrolipomatosis, an extremely rare condition, considered as an advanced form of renal sinus lipomatosis.The latter one is a milder form of the spectrum where the normally present renal sinus fat becomes prominent with ageing, obesity or exogenous steroids. Then again, how extreme can it go?

Renal Replacement Lipomatosis (RRL) is usually associated with infection, long term hydronephrosis, calculi and severe renal parenchymal atrophy. Renal calculous disease along with inflammatory changes are present in ~70% of the cases, and these produced symptoms of flank pain and fever.

The patient is a 60 yr old female patient with non-specific abdominal symptoms.

The above image is the non-contrast (plain) CT images which shows diffuse heterogeneous fatty tissue, filling almost the entire abdomen. On further inspection, we can see the fatty tissue is replacing the normal renal parenchyma (orange arrows), and are displacing the retroperitoneal structures like pancreas and duodenum anteriorly. Both kidneys are markedly enlarged, with near complete fatty replacement of the left kidney. Right kidney shows residual parenchyma (green arrow) in lower pole and in the interpolar region (mid-third). A few discrete fatty lesions (blue arrow) are also seen in the residual right renal parenchyma.

Above image shows the mass effect of the enlarged kidneys.

MIP (31mm) image on left shows the bilateral inferiorly oriented renal arteries, residual enhancing parenchyma of right kidney with fatty lesions. On the right, the axial post contrast images show the marked anterior displacement of the ascending colon (blue arrow) and descending colon (orange arrow).

3D VRT right lateral, frontal and left lateral views again shows the mass effect on colon which are seen markedly displaced anteriorly, against the anterior abdominal wall. Contrast filled small bowel loops are seen in RIF region.

3D VRT image showing the orientation of bilateral main renal arteries.

Delayed MIP images showing contrast excretion into the pelvicalyceal system of both kidneys. Right proximal ureter is marked by the orange arrow.

Delayed MIP axial and coronal images showing the right proximal ureter (orange arrow) and green arrows point to the opacification of pelvicalyceal system. No significant delay in contrast excretion was noted in this patient.

3D VRT of delayed excretory phase showing contrast within the pelvicalyceal system of both kidneys.

As expected renal veins showed moderate dilatation, with left renal vein measuring up to 17mm in diameter.





REFERENCES :

Indian J Nephrol. 2010 Apr; 20(2): 92–93. doi:  10.4103/0971-4065.65303, PMCID: PMC2931141, Renal replacement lipomatosis: A rare type of renal pseudotumor, N. A. Choh, M Jehangir, and S. A. Choh. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2931141/

Setty N, Uma K, Narvekar V N, Desai R S. Bilateral idiopathic replacement lipomatosis of the kidney with posterior mediastinal lipomatosis. Indian J Radiol Imaging [serial online] 2002 [cited 2017 Jun 21];12:251-2. Available from: http://www.ijri.org/text.asp?2002/12/2/251/28457

Acute intracranial hemorrhage with blood-fluid level

Blood-fluid level or hematocrit level or 'Sedimentation level' in spontaneous parenchymal hemorrhage could be due to coagulopathy or when the patient is on anti-coagulation medications. It can also be seen less often in cases of AVM or tumoral bleed (both primary and metastatic), Amyloid Angiopathy and sometimes in Radiation Induced Necrosis.


Approximately 60% of patients with increased PT/APTT can have blood-fluid levels in intracranial hemorrhages as per the reference below. 'Fluid-blood levels in acute intracerebral hemorrhage are moderately sensitive to the presence of coagulopathy (ie, abnormal prothrombin time and partial thromboplastin time) and highly specific for this condition. Thus, an intracerebral hemorrhage with a fluid-blood level should prompt a thorough search for coagulopathy because early treatment of this condition may improve the 40% mortality in these patients'.

AJNR Am J Neuroradiol. 1994 Feb;15(2):217-23.
Sensitivity and specificity of fluid-blood levels for coagulopathy in acute intracerebral hematomas.

What's your diagnosis? CASE 2 [June 20th, 2017]

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