CASE 12483 Published on 25.02.2015

Solid and pseudopapillary neoplasm of the pancreas

Section

Abdominal imaging

Case Type

Clinical Cases

Authors

Marta Oñate Miranda, Nuria Saturio Galán

H. U. La Paz, Radiology
Paseo de la Castellana 261
28046 Madrid, Spain;
Email:marcha14@hotmail.com
Patient

30 years, female

Categories
Area of Interest Abdomen, Pancreas ; Imaging Technique CT, MR
Clinical History
A 30-year-old woman who underwent staging CT after an excision of a dermatofibrosarcoma protuberans on the right arm presented to our hospital.
Imaging Findings
On CT, there is a heterogeneous cystic lesion with solid areas and a focus of calcification on the head of the pancreas. On MR, the lesion has cystic and solid areas with a hyperintense focus on T1 in phase and out of phase in relation to blood. The pancreatic and bile ducts are not dilated and there is progressive enhancement after contrast administration.
Discussion
The solid and pseudopapillary tumour is a rare tumour of the pancreas, representing less than 3% of pancreatic tumours [1]. In the majority of cases it appears on non-Caucasian young women [1, 2], under 35 years of age. It is more frequently located in the tail and the head of the pancreas [1] and is usually asymptomatic, although it can produce non-specific symptoms [1-3] such as nausea, vomiting, pain, or mass effect.
On CT, this tumour is usually a big well-defined heterogeneous mass with a thick wall (fibrous pseudocapsule), showing solid and cystic areas with haemorrhagic and cystic components [2]. It does not have septa and it shows calcifications in up to 20-30% of cases [1, 3].
On MR, on T1 weighted images, there are hypointense areas representing the cystic-necrotic component and hyperintense areas of haemorrhage [1]. On T2 weighted images, the cystic areas are hyperintense. The pancreatic duct is usually non-dilated [1].
There is progressive enhancement of the solid areas after contrast administration [3].
Although it has a low malignant potential [2], the elective treatment is surgery [3] with a good prognosis after complete resection [1].
The main differential diagnosis must be made with a cystic neuroendocrine tumour of the pancreas, which is rare before the age of 30, and has a low signal intensity on T1 weighted images without areas of haemorrhage. The non-cystic area is hypervascular on arterial phase after contrast administration. [1]
We did not include metastasis from dermatofibrosarcoma protuberans in our differential diagnosis, as it is a low grade mesenchymal sarcoma of the dermis and metastases are rare. [4]
In this case, the typical appearance of the tumour (young woman with an incidentally discovered tumour in the pancreatic head with solid, cystic and haemorrhagic areas) did not indicate further diagnostic workup. However, when encountered with a cystic pancreatic mass, if we do not reach a diagnosis with imaging alone, we could perform an endoscopic ultrasound with US-guided Fine-Needle Aspiration for fluid analysis. [2, 3]
Our patient was sent to surgery and the tumour was excised. The diagnosis was a solid pseudopapillary tumour and the patient is now free of disease.
Differential Diagnosis List
Solid and pseudopapillary neoplasm of the pancreas.
Neuroendocrine tumour of the pancreas with cystic degeneration
Mucinous cystic pancreatic tumour
Gastrointestinal stromal tumour
Other pancreatic cystic tumours: pancreatic serous cystadenoma
Final Diagnosis
Solid and pseudopapillary neoplasm of the pancreas.
Case information
URL: https://www.eurorad.org/case/12483
DOI: 10.1594/EURORAD/CASE.12483
ISSN: 1563-4086