BRIEF REPORTSolid-Pseudopapillary Tumor of the Pancreas

Michael Schroth, MD,1* Stefan Razeghi, MD,1 Ute Janig, MD,2

Hans Peter Hummer, MD,3 Jorn D. Beck, MD,1 and Johann Greil, MD1

Key words: solid-pseudopapillary pancreatic tumor; childhood cancer; pancreaticcarcinoma; abdominal tumors

Pseudopapillary tumor of the pancreas is a very rare,benign pancreatic tumor, and surgical resection of thetumor is regarded as curative treatment [1–10]. It has sofar been reported mainly in adults and more often inwomen [1–6,10]. Only a few affected children have beenreported, so our experience with such a child is of interest[8].

A 12-year-old boy was referred to our pediatric de-partment because of an abdominal mass. The boy hadnoticed the tumor a few weeks before in the right upperabdomen while taking a shower and had also noticedincreasing periumbilical pain. No other symptoms werepresent. The family history was remarkable only for al-lergies.

On physical examination the patient was a nearly 12-year-old, well-nourished, well-developed boy. The abdo-men was soft, with a palpable, nontender mass 7 cmdiameter in the right epigastrium. The reminder of thephysical examination was unremarkable. Laboratorywork showed normal values for complete blood count(CBC), electrolytes, lactate dehydrogenase (LDH), liverfunction tests, lipase, amylase, creatinine, urea, C-reactive protein (CrP), erythrocyte sedimentation rate(ESR), carcinoembryonic antigen (CEA), anda1-antitrypsin. Bone marrow aspiration revealed normal cy-tology. Abdominal ultrasound showed a solid, well-vascularized mass measuring 6 × 6 × 7 cm in therightupper abdomen adjacent to the gallbladder, liver hilus,and pancreatic head. The liver, spleen, and both kidneyswere normal, and there was no lymphadenopathy. A CTscan confirmed the ultrasound findings (Fig. 1). Lapara-tomy revealed a tumor in the head of the pancreas, whichwas displacing the duodenum, ductus choledochus, andmajor abdominal vessels. There was no infiltrativegrowth, and the tumor was enucleated completely. His-tolgic findings are shown in Figure 2. Follow-up visitsshowed no evidence of recurrent disease 15 months aftersurgery.

DISCUSSION

Pancreatic tumors in children can be devided intothree categories: exocrine and ductal tumors, endocrinetumors, and mesenchymal neoplasms. Most pancreatictumors in children are benign and are treated by excisiononly. The exception is pancreatic carcinoma, which ishighly malignant and requires radical surgery. It has of-ten spread to the liver and lung by the time of diagnosis,however, and is inoperable. Presenting features includean abdominal mass and pain, gastrointestinal distur-bances, and, rarely, obstructive jaundice. Chemotherapyand radiotherapy are ineffective [1].

Solid pseudopapillary tumor is uncommon, and its eti-ology is so far unknown. An increasing frequency inrecent years has been reported, but still it accounts foronly approximately 1–2% of all exocrine pancreatic tu-mors. Most patients are female and over 30 years of age[1–6, 10]. Rarely, children and adolescents are affected,Matsunou and Konishi [8] reported 7 patients aged 8–24years, all of them female. Thus, both the age and the sexof our patient are atypical.

The clinical manifestations are rather nonspecific ac-cording to most reports [1–3,8]. Frequently, a palpablemass is found by the patient and results in further radio-logic procedures, which reveal the pancreatic tumor [8].

1Klinik mit Poliklinik fu r Kinder und Jugendliche der Friedrich-Alexander-Universita¨t Erlangen-Nu¨rnberg, Erlangen, Germany2Institut fur Paidopathologie der Christian-Albrechts-Universita¨t zuKiel, Kiel, Germany3Kinderchirurgische Klinik der Chirurgischen Universita¨tsklinik derFriedrich-Alexander-Universita¨t Erlangen-Nu¨rnberg, Erlangen, Ger-many

*Correspondence to: Dr. med. Michael Schroth, Klinik mit Poliklinikfur Kinder und Jugendliche, Friedrich-Alexander-Universita¨t Erlan-gen-Nurnberg, Loschgestraße 15, D-91054 Erlangen, Germany. E-mail: http://www.michaelschroth@gmx.de or www.michael.schroth@rzmail.uni-erlangen.de

Received 18 January 1999; Accepted 5 March 1999

Medical and Pediatric Oncology 33:508–509 (1999)

© 1999 Wiley-Liss, Inc.

Our case also came to our attention after the mass hadbecome large enough to be felt by the patient. Shortepisodes of abdominal tenderness and discomfort as wellas long-lasting colicky pain have been described [1–3].Pancreatic carcinoma or other abdominal tumors, e.g.,rhabdomyosarcoma, neuroblastoma, and hepatoblas-toma, are initially suspected, but in our patient the diag-nosis of this rare tumor was made histologically.

Surgery is regarded as the therapeutic procedure ofchoice [1–10]. Ohiwa et al. [1] as well as Tsunoda et al.[4] reported patients with pseudopapillary pancreatic tu-mor who underwent radical pancreaticoduodenectomy(Whipple procedure) because intraoperatively pancreaticcancer was suspected. In our case, a well-encapsulatedmass was found during surgery and was excised com-pletely without radical resection of the surrounding struc-tures. Siech et al. [2] followed seven patients, all of themadults, up to 8 years postsurgery who showed no evi-dence for recurrent disease or complications. Metastasesand local relapse are rarely reported. Matsunou and Ko-nishi [8] discuss two adult patients who developed livermetastases. Although these tumors could be surgicallyresected, both patients died after surgery. Matsunou andKonishi suggest that metastases might most likely befound in patients with long-lasting disease and thus re-gard pseudopapillary pancreas tumor as “semimalignantor semibenign.” The small number of reported cases sofar does not permit accurate estimates of long-term sur-vival. There has been no evidence of disseminated dis-ease in our patient, who remains well 15 months afterexcision of the mass.

REFERENCES

1. Ohiwa K, Igarashi M, Nagasue N, et al. Solid and cystic tumor(SCT) of the pancreas in an adult man. HPB Surg 1997;10:315–321.

2. Siech M, Merkle E, Mattfeldt T, et al. Solid pseudopapillary tu-mors of the pancreas. Chirurgica 1996;67:1012–1015.

3. Pelosi G, Iannucci A, Zamboni G, et al. Solid and cystic papillaryneoplasm of the pancreas: A clinico-cytopathologic and immuno-cytochemical study of five new cases diagnosed by fine-needleaspiration cytology and a review of the literature. Diagn Cytopa-thol 1995;13:233–246.

4. Tsunoda T, Eto T, Tsurifune T, et al. Solid and cystic tumor of thepancreas in an adult male. Acta Pathol Jpn 1991;41:763–770.

5. Ueda N, Nagakawa T, Ohta T, et al. Clinicopathological studieson solid and cystic tumors of the pancreas. Gastroenterol Jpn1991;26:497–502.

6. Matsunou H, Konishi F, Yamamichi N, et al. Solid, infiltratingvariety of papillary cystic neoplasm of the pancreas. Cancer 1990;65:2747–2757.

7. Eguchi K, Ishibashi H, Dohmen K, et al. Solid and cystic tumor ofthe pancreas with prominent calcification. Fukuoka Igaku Zasshi1990;81:261–265.

8. Matsunou H, Konishi F. Papillary-cystic neoplasm of the pan-creas. A clinicopathologic study concerning the tumor aging andmalignancy of nine cases. Cancer 1990;65:283–291.

9. Horie A, Haratake J, Jimi A, et al. Pancreatoblastoma in Japan,with differential diagnosis from papillary cystic tumor (ductuloa-cinar adenoma) of the pancreas. Acta Pathol Jpn 1987;37:47–63.

10. Lieber MR, Lack EE, Roberts JR Jr., et al. Solid and papillaryepithelial neoplasm of the pancreas. An ultrastructural and immu-nocytochemical study of six cases. Am J Surg Pathol 1987;11:85–93.

Fig. 1. Abdominal CT scan with contrast. Apparently encapsulatedmass of 7 cm diameter close to the liver hilus without evidence ofinfiltrative growth, liver metastases, or pathologically enlarged lymphnodes along the major abdominal vessels. (1, tumor; 2, liver; 3,muscle).

Fig. 2. Solid-pseudopapillary tumor, hematoxylin and eosin. The tu-mor shows a solid and pseudopapillary pattern consisting of uniformcells with round nuclei. The cells are growing around fine fibrovas-cular stalks. Small gland-like structures and pseudocystic formationsare seen. Mitoses are rare. Some cells have hyaline granules in thecytoplasm, staining positive for periodic acid Schiff. The tumor cellsstain positive for vimentin, NSE, and Cam 5.2. There are negativereactions for S100 protein, chromogranin A, synaptophysin, and 013.The staining for progesterone was negative as well. These findings arecompatible with a solid-pseudopapillary pancreatic tumor. ×280.

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