LETTER TO EDITOR
Year : 2001 | Volume
: 11 | Issue : 3 | Page : 153--155
Papillary cystic neoplasm of the pancreas
L Upreti, S Kumar, SK Bhargava, V Rathi, R Gupta
University College of Medical Sciences & GTB Hospital, Delhi University, Delhi 110095, India
University College of Medical Sciences & GTB Hospital, Delhi University, Delhi 110095
|How to cite this article:|
Upreti L, Kumar S, Bhargava S K, Rathi V, Gupta R. Papillary cystic neoplasm of the pancreas.Indian J Radiol Imaging 2001;11:153-155
|How to cite this URL:|
Upreti L, Kumar S, Bhargava S K, Rathi V, Gupta R. Papillary cystic neoplasm of the pancreas. Indian J Radiol Imaging [serial online] 2001 [cited 2020 Feb 28 ];11:153-155
Available from: http://www.ijri.org/text.asp?2001/11/3/153/28396
Papillary cystic neoplasm (PCN) is a rare pancreatic tumor ,,,. The lesion is mostly seen in young women. In only about 7% of occurrences, men are affected . The neoplasm is usually large in size at the time of diagnosis. Incidence of local and metastatic spread is uncommon. The prognosis following surgical resection is good. We report US and CT appearances of this rare tumor. and discuss the differential diagnosis of the lesion.
A twenty-years-old woman presented with a history of pain in the left upper quadrant of the abdomen. She had experienced this for the past one year. She also had occasional vomiting. There was no significant past history or family history. Vital signs were normal. No abnormal findings were noted on physical examination. Routine laboratory examinations were normal. Evaluation by abdominal US revealed a mass lesion measuring 9.8 x 8.1 cm in the tail of pancreas [Figure 1]. The lesion had well-defined margins. It was slightly hypoechoic relative to the adjacent normal pancreatic parenchyma. No calcification was noted in the lesion. No evidence of lymphadenopathy or any other abnormality was seen in the abdomen.
CT of the abdomen with oral and intravenous contrast showed a large well-defined mass lesion in the tail of the pancreas [Figure 2]. It was hypodense relative to the pancreas on NCCT and CECT. Few scattered areas of low attenuation were seen. No evidence of calcification was noted. The mass could be delineated separately from the adjacent stomach. The left adrenal gland was normal. No evidence of lymphadenopathy or any other abdominal pathology was found. Fine needle aspiration biopsy under sonographic guidance revealed papillary cystic neoplasm of the pancreas. At laparotomy a large mass lesion was found in the tail of the pancreas. Distal pancreatectomy with splenectomy was done. Post-operative recovery was uneventful. Histopathological examination of the resected tumor was consistent with papillary cystic neoplasm of the pancreas.
Papillary cystic neoplasm (PCN) of the pancreas is a rare tumor. In the past, these lesions have been incorrectly diagnosed as adenocarcinomas, islet cell tumors and cystic pancreatic neoplasms . Other names by which this lesion has been described in the literature include papillary epithelial neoplasm, papillary solid neoplasm, solid cystic papillary neoplasm, solid and papillary epithelial neoplasm, solid and cystic acinar cell tumor, papillary cystic epithelial neoplasm, papillary cystic carcinoma, low grade papillary neoplasm and Frantz's tumor. The incidence of this neoplasm has been reported to be between 0.17% and 2.5% . Women form the majority of patients; the incidence in men is about seven percent. These lesions have a low malignant potential despite their large size. Malignant lesions are in fact rare and are mostly seen in the older patients . Thus surgical resection of these lesions is mandatory.
Clinically, the lesion causes few symptoms; most often it is either an incidental finding or late diagnosis. It may also present with abdominal pain, which can be associated with vomiting due to compression of the adjacent gastrointestinal tract . Rarely it may present as an acute emergency due to rupture and hemoperitoneum. Pathologically, the lesion usually has a thick capsule, a complex inner structure with solid and cystic components and often, hemorrhagic necrosis. Calcification is uncommon, but may be present within the tumor or in the capsule. Due to varying degree of hemorrhage and necrosis, different patterns occur which affect the imaging appearances. Three different patterns of this lesion have been recognized on imaging . Completely solid masses are enclosed by fibrous capsule. On US, the neoplasm is isoechoic or hypoechoic relative to surrounding normal pancreatic parenchyma, few small echo-free areas may be seen. On CT, the lesion is isodense or hypodense relative to normal pancreas on both non-contrast and contrast enhanced images. Few cystic areas may be present. The capsule, which is difficult to evaluate on US, may be seen on CT scan as a hypodense, enhancing area . The main differential diagnosis of this type of the lesion is a non-functioning islet cell tumor. PCN is more common in young patients and more frequent in women. On CECT the non-functioning islet cell tumor enhances to a greater degree. Sometimes angiography may be needed for differentiation. In such cases, PCN always appears hypovascular or avascular; islet cell tumors on the other hand are almost always hypervascular . Fine needle aspiration biopsy almost always allows correct diagnosis.
Masses with mixed contents are the most frequently encountered lesions. This type shows both solid and cystic areas within the lesion. The cystic areas are due to the presence of hemorrhage and necrosis. On sonography, a mixed mass lesion is encountered. The solid component of the lesion may show papillary projections in the cystic component, which may appear like septations. Calcification is rarely seen. On CT scan the hemorrhagic foci are hypodense versus the normal parenchyma, but hyperdense versus the fluid contents . Degree of cystic change does not correlate with the size of the tumor. Imaging features of this type of lesion appear somewhat distinctive and in the appropriate clinical settings, this lesion may be suggested as the possible primary diagnosis. Ductal adenocarcinoma with necrosis, almost never reaches the size of a PCN. These lesions are invasive and evidence of early local/distant spread is seen. Cystic neoplasm will show septations and the solid component is never as prominent as in the PCN . Atypical pseudocyst with hemorrhagic fluid and septations may be confused with this tumor. In these cases, clinical or laboratory evidence of pancreatitis will be present. On CT, the debris of the pseudocyst will not enhance, whereas enhancement will be seen in the solid part of the PCN. PCN may be completely cystic. The margins of these cystic lesions are well-defined; the capsule may be calcified in some cases. They are usually indistinguishable from other pancreatic masses. Metastatic spread of the PCN is uncommon. The spread may occur to regional lymph nodes and the liver. The metastatic masses show imaging findings similar to the primary lesion with well-defined margins and solid and cystic areas within the mass.
To conclude, papillary cystic neoplasm of the pancreas is a rare low-grade malignant lesion, which most often present in young women with few clinical symptoms. It is mostly heterogeneous on imaging, with varying proportion of solid and cystic areas. Metastatic lesions, although rare, appear identical to the primary neoplasm. Awareness of presence and imaging characteristics of this tumor is important, as prognosis for this lesion is extremely favorable following surgical resection.
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