A 53 year-old female with complaints of vague upper left abdominal pain for one year was referred to our hospital. There was a personal history of breast cancer and she had undergone partial mastectomy followed by radiochemotherapy.
Contrast-enhanced CT (Figure 1) and MRI (Figure 2) revealed a well circumscribed cystic lesion, with multiple septations located in the pancreatic cephalic area, measuring 13,6 x 10,3 cm, with smaller cystic areas extending to the pancreatic body and tail. Celiac trunk, hepatic and splenic arteries, mesenteric artery and portal vein were patent. There was no evidence of lymphadenopathy.
Figure 1. Abdominal CT after iodine injection, depicting large septated cystic lesion. Note mycrocistic pattern of the inferior segment of the pancreatic head and uncinate process.
Figure 2. Abdominal MRI: Large septated cystic lesion of the pancreatic head, with high signal intensity on T2 FS (left image) and low signal intensity on T1 FS (right image), without enhancement after gadolinium.
Unremarkable. Serologic screening for the tumor markers AFP, CA 19–9 and CEA revealed normal serum levels.
Endoscopic ultrasound (EUS):
EUS identified a voluminous pancreatic cystic lesion with internal septations, fully involving the pancreatic gland, formed of non pure content. The larger loca were in the cephalic/istmus region and the distal pancreatic segments presented a microcystic pattern. It was not possible to document a relationship with the pancreatic duct, given the size of the lesion.
Figure 3. EUS (linear array): Pancreatic cystic lesion with septations in the cephalic pancreatic region.
A yellowish opaque fluid sample (negative string sign) was obtained using a 19 gauge FNA needle (Figure 4). Cyst fluid analysis revealed a lipemic sample with pancreatic amylase and CEA in the normal range. Cytologic examination showed dense mature lymphocyte groups, which is in line with the diagnosis of pancreatic lymphangioma.
Figure 4. Yellowish opaque fluid sample obtained by EUS-FNA.
Lymphangioma is a benign tumor of the lymphatic vessels. Its etiology remains unclear; a well established theory suggests that lymphangiomas arise from sequestrations of lymphatic tissue during embryologic development. However, it has been suggested that abdominal trauma, lymphatic obstruction, inflammatory process, surgery or radiation therapy may lead to the secondary formation of such a tumour (1). Lymphangiomas of the pancreas are extremely rare, accounting for less than 1% of this type of tumor (2). There are no specific blood markers to confirm the diagnosis of lymphangioma of the pancreas; moreover, radiological imaging (US, CT, MRI) often are not able to perform a correct preoperative diagnosis (3,4). EUS can depict the internal details of a cystic pancreatic lesion in great detail, and also be used to aspirate the lesion for diagnostic studies.
1. Schneider G, Seidel R, Altmeyer K, et al. Lymphangioma of the pancreas and the duodenal wall: MR imaging findings. Eur Radiol 2001;11:2232-5.
2. Abe H, Kubota K, Noie T, Bandai Y, Makuuchi M. Cystic lymphangioma of the pancreas: a case report with special reference to embryological development. Am J Gastroenterol 1997;92:1566–1567
3. Gray G, Fried K, Iraci J. Cystic lymphangioma of the pancreas: CT and pathologic findings. Abdominal Imaging 1998; 23 (1): 78–80.
4. Schneider G, Seidel R, Altmeyer K, et al. Lymphangioma of the pancreas and the duodenal wall: MR imaging findings. European Radiology 2001; 11 (11): 2232–2235.
Mariana Nuno Costa1, José Pereira2, Diana Carvalho1, Ricardo Rio-Tinto1, Gonçalo Ramos1
1. Serviço de Gastrenterologia do Centro Hospitalar de Lisboa Central, EPE.
2. Serviço de Imagiologia do Centro Hospitalar de Lisboa Central, EPE.