Mucinous cystic neoplasms of the pancreas are un- common and known to occur mainly in middle-aged women (1). The pediatric case has been rarely described in the literature (2). In this report we present an 11-year- old girl with a pancreatic cystadenocarcinoma mimick- ing a traumatic pseudocyst .
Case Report
An 11-year-old girl was referred from outside hospital, where she underwent Roux-en-Y cystojejunostomy un- der the impression of traumatic pancreatic pseudocyst following blunt abdominal trauma four months previ- ously. At that time, she showed nausea and vomiting with mildly elevated serum amylase level(106 U / L ) . Preoperative outside CT scan showed a unilocular cystic mass measuring about 7 cm in diameter in the pancreat- ic tail (Fig. 1). Pathologic specimen was not obtained at the operation. The mass was increased in size on the
postoperative CT scan three months after operation that was performed due to recurrent nausea and vomiting.
At our hospital, US and CT scan were rechecked.
There was a multiloculated cystic mass in the pancreatic tail and the attenuation value of the cyst was 10 HU (Fig.
2A). The mass had slightly thick and irregular wall and septae without mural nodule or solid portion. Two small low attenuating lesions were seen in the liver (Fig. 2B), which were not shown on either preoperative and post- operative outside CT scans.
At laparotomy, there was a cystic mass in the pancreatic tail, adherent to the splenic hilum, root of transverse mesocolon, and the posterior gastric wall. The mass infil- trated to the previous cystojejunostomy site. Distal pan- createctomy with jejunal resection and liver biosy were performed. The gross pathologic examination revealed a multilocular cyst (Fig. 2C). The cysts were lined by a tall, mucin-producing columnar epithelium and filled with mucinous material on the microscopic examination. Some foci of epithelial atypia were identified. Mucinous cystade- nocarcinoma with liver metastasis was diagnosed.
Discussion
Cystic neoplasms of the pancreas are uncommon tu-
J Korean Radiol Soc 1999;41:1 77- 1 7 9
Pa n c reatic Mucinous Cys t a d e n o c a rc i n o m a in an 11 - year-old Girl : A Case Re p o r t1
Kyung-Jae Jung, M.D., Bokyung K. Han, M.D., Hye - Kyung Yoon, M.D., S u n g - Wook Shin, M.D.
Mucinous cystic neoplasms of the pancreas are uncommon and known to occur mainly in middle-aged women. We present a case of pancreatic mucinous cystadeno- carcinoma in an 11-year-old girl who had a past history of blunt abdominal trauma.
The tumor was initially mistaken for a traumatic pseudocyst. US and CT revealed a multiloculated cystic mass in the pancreatic tail. The mass showed good transmission on ultrasonography (US) and had an attenuation value of 10 HU on CT. Metastatic le- sions in the liver were also shown on follow-up study.
Index words :Pa n c r e a s, CT
Pa n c r e a s, neoplasms
N e o p l a s m s, in infants and children
1Department of Radiology, Samsung Medical Center, College of Medicine, Sung Kyun Kwan University
Received August 25, 1998 ; Accepted April 15, 1999
Address reprint requests to : Kyung-Jae Jung, M.D., Department of Radiology, Samsung Medical Center, College of Medicine, Sung Kyung Kwan University, #50 Irwon-Dong, Kangnam-Ku, Seoul 135-710, Korea.
Tel. 82-2-3410-2518 Fax. 82-2-3410-2559
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mors, accounting for 1-13% of pancreatic cystic lesions and 1-3% of pancreatic tumors (3). Neoplastic pancreatic cysts are classified into microcystic (serous) adenomas and mucinous cystic neoplasms, which are subdivided into mucinous (macrocystic) cystadenomas and muci- nous cystadenocarcinomas. Whereas microcystic adeno- mas are benign, mucinous cystic neoplasms are poten- tially malignant or already malignant at the time of initial presentation (4,5).
Pediatric case of pancreatic mucinous cystic neoplasm has been very rarely reported. Grosfeld et al. (2) reported two cases of mucinous cystadenomas in 4- and 10-month- old children. However, they did not described the CT findings of the tumors. In our knowledge, the case of mu- cinous cystadenocarcinoma in children has not been re- ported. Pancreatic mucinous cystic neoplasms are often quite large. They are uni- or multilocular masses formed by cysts larger than 2 cm in size. Papillary projections within the cysts are not uncommon and a fibrous capsule is constantly present (1,2,5). Metastases have occurred at
the time of diagnosis in 25% of mucinous cystadenocar- cinomas (4).
Kyung-Jae Jung, et al : Pancreatic Mucinous Cystadenocarcinoma
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A B
C
Fig. 2. A. Follow-up CT scan performed 4 months after opera- tion. Post-contrast CT scan shows a multiloculated cystic mass with rather thick and irregular wall or septae in the pancreatic tail. The mass has an attenuation value of 10 HU.
B. Two small low attenuating metastatic lesions (arrowheads) are seen in the liver.
C. Gross specimen including transverse mesocolon. A multiloc- ular cyst (arrows) in the pancreatic tail, which is adherent to the root of transverse mesocolon (arrowheads).
Fig. 1. Preoperative post-contrast CT scan reveals about 7-cm sized, a large unilocular cyst having smooth wall in the pan- creatic tail. The cyst showed markedly decrease in size after Roux-en-Y cystjejunostomy on follow-up US (not shown).
Imaging findings of mucinous cystic neoplasms are re- lated to the pathologic features, which show uni- or mul- tilocular cystic masses. On US, mucinous cystic tumors show good transmission and posterior acoustic enhance- ment. The most important findings are the internal sep- tae and the nodular or papillary excrescences, which are better demonstrated on US than CT (5). Pre-contrast CT demonstrates a well-encapsulated mass with attenuation values of near water density. Post-contrast CT shows en- hancing wall, internal septae, and mural nodules.
Differentiation of mucinous cystadenoma from cystade- nocarcinoma is very difficult unless evidence of extra- pancreatic spread or metastasis is present (2,5).
Mucinous cystic neoplasm may mimic pancreatic pseudocyst, especially when the cystic mass is compli- cated by hemorrhage (2,3,5). The most common pancre- atic cysts are pseudocysts, either traumatic or infectious origin (2). Usually the differential diagnosis can be made on the basis of previous history and laboratory findings.
Patients with pancreatic pseudocyst usually have a histo- ry of pancreatitis, cholelithiasis or abdominal trauma in almost all cases and elevated serum amylase level in 75 % of patients (6). However, as in our case, 17 % of pa- tients with mucinous cystic neoplasms also have a clini- cal history of pancreatitis or trauma (5), in which case the differentiation of cystic neoplasm from pancreatic pseudocyst becomes more difficult. Pancreatic pseudo- cyst is usually unilocular and presence of septations within the cyst suggests associated complications such as hemorrhage or infection. Thick wall may be present in both complicated pseudocyst and mucinous cystic neo- plasm. Multilocular appearance with presence of multi- ple septae as in our patients should raise a possibility of
cystic neoplasm (7). Increasing size of the cyst despite of internal drainage procedure and evidence of metastasis as in our patient are the key to the diagnosis.
Other neoplastic pancreatic cysts which could occur in children include microcystic adenoma, necrotic solid tu- mors, and solid and papillary epithelial neoplasm (SPEN) with cystic appearance. In microcystic adenoma, the tu- mor frequently appears solid without visible cysts al- though some of the cysts can be larger than 2 cm in di- ameter. Calcification is more common and a central scar is identified only in microcystic adenomas (2).
In conclusion, a possibility of cystic neoplasm should be considered when pancreatic cyst shows multilocular appearance with multiple septae or increasing size on follow-up imaging studies in spite of the presence of pre- vious history of trauma in children.
R e f e r e n c e s
1 . Mathieu D, Guigui B, Valette PJ, et al. Pancreatic cystic neo- plasms. Radiol Clin North Am 1989 ; 27 : 163-176
2. Grosfeld JL, Vane DW, Rescorla FJ, McGuire W, West KW.
Pancreatic tumors in childhood: analysis of 13 cases. J Pediat Surg 1990 ; 25 : 1057-1062
3. Civello IM, Frontera D, Viola G, Maria G, Crucitti F. Cystic neo- plasm mistaken for pancreatic pseudocyst. H e p a t o g a s t r o e n t e r o l o g y 1996 ; 43 : 967-970
4. Johnson CD, Stepehns DH, Charboneau JW, Carpenter HA, Welch TJ. Cystic pancreatic tumors: CT and sonographic assess- ment. A J R 1988 ; 151 : 1133-1138
5. Fugazzola C, Procacci C, Andreis IAB, et al. Cystic tumors of the pancreas: evaluation by ultrasonography and computed tomogra- phy. Gastrointest Radiol 1991 ; 16 : 53-61
6. Fauci AS, Braunwald E, Isselbacher KJ. H a r r i s o n’s principles of in- ternal medicine. McGraw-Hill, 1998: 1746-1747
7. Friedman AC, Dachman AH. Radiology of the liver, biliary tract, and p a n c r e a s. St. Louis : Mosby, 1994: 825-826
J Korean Radiol Soc 1999;41:1 77- 1 7 9
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1 1세 여아에서 발생한 췌장의 점액성 낭선암 : 1예 보고1
1성균관대학교 의과대학 삼성의료원 진단방사선과
정 경 재・김 보 경・윤 혜 경・신 성 욱
췌장의 점액성 낭성 종양은 드문 췌장종양으로 주로 중년 여성에 발생하는 것으로 알려져 있다. 저자들은 복 부 둔상의 과거력을 가진 1 1세 여아에서 발생한 췌장의 점액성 낭선암을 경험하였기에 영상소견을 보고한다. 처 음 종괴는 외상과 관련한 가성낭종으로 오인되었으며 추적영상검사상 크기가 커졌다. 초음파검사와 C T상 췌장 미부에 위치한 다방성의 낭성종괴로 C T상 종괴의 내부 음영도는 약 10H U로 측정되었고 추적검사상 C T에서 간의 낭성 전이성 결절도 관찰되었다.
대한방사선의학회지 1 9 99;41: 1 77-1 7 9
