Case No. :	74
Month :	February
Year :	2005
Contributor :	Dr. Tejaswini Deshmukh

Other Cases

A 13-year-old girl presented with a gradually increasing lump in the epigastrium since six months. It was not associated with pain or any gastrointestinal symptoms.

RADIOLOGICAL Examination:

Ultrasonography of the abdomen revealed an isoechoic mass lesion in the region of the head of the pancreas. It had a few hypoechoic areas within.

Fig. 1	Fig. 2

Contrast enhanced CT scan of the abdomen revealed a large, hypodense mass lesion ( Fig 3 and Fig 4 ) in relation to the head of pancreas; few low attenuation areas were seen within it.

Fig. 3	Fig. 4

No calcification was noted in the lesion. The bowel loops were displaced to the periphery. The common bile duct could be traced along the posterior aspect and showed mild dilatation. The portal and superior mesenteric veins were seen in close proximity but separate from the mass. The right kidney, renal vein and the IVC were seen along the posterior aspect of the mass lesion. There was no abdominal lymphadenopathy. A radiologic diagnosis of a solid-cystic tumor of the pancreas was made. The patient was operated for the same. At laparotomy a large mass

Fig. 5	Fig. 6

was seen arising from the head of the pancreas. It was splaying the porto-splenic confluence. Whipple's surgery ( pancreatico-duodenectomy ) was performed. Post surgery she had an uneventful recovery.

Fig. 7

DISCUSSION:

Papillary cystic neoplasm or Gruber-Frantz tumor is the rarest of cystic neoplasm of the pancreas. The incidence of this neoplasm has been reported to be between 0.17% and 2.5% . It characteristically occurs in young women. Although known for its benign clinical course, a low malignant potential has been described in approximately 1 out of 7 patients, generally older than 20 years and more commonly in men. This may be in the form of local disease recurrence or infiltration to adjacent organs and/or vessels. Metastases to liver are reported in 7% of patients. Exceptionally, metastases to peritoneum, lung and skin have been reported.

The most frequent symptom of these tumors is upper abdominal pain - seen in nearly half the patients. Other presentations are either related to the upper digestive system or the presence of a mass. Symptoms may be present for years before the correct diagnosis is made.

Diagnosis:

Ultrasonographic features include an isoechoic or hypoechoic mass relative to surrounding normal pancreatic parenchyma. It contains cystic areas due to hemorrhage and necrosis.

Amongst all the available diagnostic modalities, CT still remains the most sensitive diagnostic tool. On CT, the lesion is isodense or hypodense relative to normal pancreas on both non-contrast and contrast enhanced images. It shows fluid-debris levels indicating blood products in areas of cystic degeneration and necrosis. The differential diagnosis includes atypical pseudocyst with hemorrhagic fluid and septations , cystic neoplasms and endocrine tumors of pancreas.

Pathology

These tumors have a predilection for the pancreatic tail. Macroscopically, they are round to oval and encapsulated by a fibrotic capsule and contain intermingling solid, cystic and hemorrhagic areas. Microscopically, the tumor displays sheets and cords of polygonal cells with pseudopapillary components containing fibrovascular stalks, pseudorosettes and foamy macrophages with eosinophilic inclusion bodies. Cellular atypia and mitotic figures are rare

Treatment

All these tumors should be viewed as malignant tumors and treated with anatomic pancreatic resection , which usually consists of a distal pancreatectomy or a partial, pylorus-preserving, duodenopancreatectomy (Whipple's procedure) if the tumor arises from the pancreatic head. Any lesser procedure results in invasion into porta hepatis, liver, major vascular structures and viscera. Resection offers an excellent prognosis, with 10 year survival approaching 100% .

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