Solid pseudopapillary tumors of the pancreas are usually described as rare lesions with low malignant potential and unpredictable behavior. The prognosis may be favorable even with local or distant dissemination. Despite the steady increase of cases diagnosed, the pathogenesis of this neoplasm remains unclear. We present a case of an incidental tumor discovered because of an acute abdomen in a postpartum woman without previous evidence of a tumor during the pregnancy

Corso, Carlos Otavio

Cunha, Lenine

Grezzana Filho, Tomáz de Jesus Maria

Meine, Mario Henrique Mendes de Mattos

Schlindwein, Eduardo S.

Zettler, Claudio Galleano

English

Lume 5.8

POSTPARTUM INCIDENTAL SOLID PSEUDOPAPILLARY... Grezzana et al. RELATOS DE CASOS240 Revista da AMRIGS, Porto Alegre, 50 (3): 240-243, jul.-set. 2006Postpartum Incidental Solid PseudopapillaryTumor of the Pancreas: Case ReportRELATOS DE CASOSTOMAZ DE JESUS MARIA GREZZA-NA – Department of Hepatobiliary Surgery– Irmandade Santa Casa de Misericórdia-ISCMPA-Porto Alegre, Brazil.MÁRIO HENRIQUE MATTOS MEINE– Department of Hepatobiliary Surgery – Ir-mandade Santa Casa de Misericórdia-ISCMPA-Porto Alegre, Brazil.EDUARDO SOARES SCHLINDWEIN –Department of Hepatobiliary Surgery – Ir-mandade Santa Casa de Misericórdia-ISCMPA-Porto Alegre, Brazil.LENINE CUNHA – Department of Hepa-tobiliary Surgery – Irmandade Santa Casa deMisericórdia-ISCMPA-Porto Alegre, Brazil.CLAUDIO GALLEANO ZETTLER – De-partment of Pathology – Irmandade SantaCasa de Misericórdia – ISCMPA – Porto Ale-gre, Brazil.CARLOS OTÁVIO CORSO – Departmentof Morphological Sciences – UniversidadeFederal do Rio grande do Sul – UFRGS-PortoAlegre – Brazil. Address correspondence to:Tomaz de J. M. GrezzanaRua Irmão José Otão, 170/40690035-060 – Porto Alegre – RS, BrasilFone: (55)(51) 3214-8211 tomgrez@uol.com.brRecebido: 5/8/2005 – Aprovado: 20/3/2006ABSTRACTSolid pseudopapillary tumors of the pancreas are usually described as rare lesionswith low malignant potential and unpredictable behavior. The prognosis may be favorableeven with local or distant dissemination. Despite the steady increase of cases diagnosed,the pathogenesis of this neoplasm remains unclear. We present a case of an incidentaltumor discovered because of an acute abdomen in a postpartum woman without previousevidence of a tumor during the pregnancy.KEY WORDS: Solid Pseudopapillary Tumor of the Pancreas, Franz Tumor, CysticNeoplasm of the Pancreas.I NTRODUCTIONIn 1959 Franz recognized a raretype of neoplasm of the exocrine pan-creas with papillary and cystic charac-teristics in a 2-year-old boy, who diedduring an attempted pancreaticoduode-nectomy. Since the original descrip-tion, the frequency of pseudopapillarytumors has been increasing, probablydue to the better awareness of theselesions by physicians (1). These tumorsaccount for less than 2% of all pancrea-tic tumors, and have specific clinical,pathological, and radiographic featu-res that can help to differentiate theirdiagnosis from other malignant neo-plasms of the pancreas (2). These tu-mors are found predominantly in girlsor young women and present as large,encapsulated masses with areas of ne-crosis, hemorrhage, and cystic dege-neration. Until 2002, around 450 ca-ses were reported, most of them in thelast 10 years.C ASE REPORTA 32-year-old woman was admit-ted to our Institution with a 2-week his-tory of lower abdominal pain, fever,asthenia, and weight loss. She un-derwent a cesarean delivery 3 weeksbefore and was treated during 14 dayswith oral antibiotics due to a supposedendometrial infection. On physicalexamination, the patient had importantright lower quadrant tenderness withperitoneal signs. Laboratory exams re-vealed significant leukocytosis and theultrasonography (US) revealed a fluidcollection on the right iliac fossa, com-patible with acute ruptured appendici-tis, and also a 7-cm cyst in the left kid-ney. She underwent a laparotomy, thediagnosis of acute perforated appendi-citis with abscess was confirmed andthe treatment performed. An abdomi-nal US on day 5 did not show any com-plication but, instead of the left kid-ney cyst as seen in the first US scan,the exam suggested the possibility ofa left adrenal neoplasm. However, acomputed tomography (CT) scan of theabdomen showed a tumor measuring7.0-X 6.0-x 6.5-cm in the tail of thepancreas, predominantly cystic, butenhancing a rim of solid tissue, sugges-ting a mucinous cystadenoma (Figure 1).Carcinoembryonic antigen and Ca-125tumor markers were within normal li-mits.Two months later, she underwent anew laparotomy to remove this tumor.The mass was well-limited and sur-rounded by large veins, showing closerelation to the splenic vein. No signsof distant dissemination or local inva-sion were seen. The patient underwenta complete surgical resection of thetumor, including a minimal part of thepancreas tail. The spleen was removedin bloc because of vascular damage ofthe splenic vein (Figure 2). The post-operative course was uneventful andshe was discharged from the hospitalon day 7. No adjuvant therapy was usedand there were no signs of recurrenceof the tumor 18 months later.Macroscopically, the tumor wasuniloculated, filled with dark liquid andsome clots, suggesting previous blee-ding. The inner surface was irregular,with friable trabecula and tanned-grayzones. Light microscopy demonstratedpredominantly solid areas with a sheet-like arrangement of polygonal cells andscarce stroma (Figure 3-A), areas withsolid and cystic arrangement (Figure3-B), and areas with predominant pseu-dopapillary formations (Figure 3-C). No signs of vascular invasion weredetected.Untitled-11 21/9/2006, 06:27240POSTPARTUM INCIDENTAL SOLID PSEUDOPAPILLARY... Grezzana et al. RELATOS DE CASOSRevista da AMRIGS, Porto Alegre, 50 (3): 240-243, jul.-set. 2006 241D ISCUSSIONBefore the new classification ofpancreatic neoplasm’s was created bythe World Health Organization in 1996,misdiagnosis between pseudopapilla-ry tumors, adenocarcinoma, islet celltumors, cystadenomas, papillary cys-tadenocarcinoma and cystadenocarci-noma were common (3).There is a clear female predominan-ce (>90%) and most of these tumorsare diagnosed in the second and thirddecades of life. Fifty-two percent of thepatients are under 20 at the moment ofthe diagnosis (4). The distribution ofthe tumor in the pancreas is the samein all age groups (head and neck, 35%,body, 25%, tail, 40%). The etiopatho-geny is unknown, but some authors,based on morphological and immuno-histological features, support the ideathat they originate from pluripotentembryonic cells, and grow by the ac-tion of female hormones. This hypo-thesis is supported by the presence ofprogesterone receptors on the epithe-lia in 90% of the tumors. In contrast,the role of estrogen receptors hasshown conflicting results in the presen-ted series (2).In the present report, there was noclinical evidence of the tumor duringthe pregnancy, but the US was not di-rected to this diagnosis. However, sexhormones play an important role in thegrowth of the solid pseudopapillarytumors and pregnancy is associatedwith stimulation of tumor growth (6,7).Figure 3 – Solid areas with a sheet-like arrangement (A). Areas with solid and cystic components (B). Predominant pseudopa-pillary formations (C). HE, 100X.Figure 1 – CT scan ofthe abdomen shows a7.0-x 6.5-cm cysticpancreatic mass withan enhancing rim ofsolid component (ar-row).Figure 2 – Macrosco-pic appearance of re-sected solid pseudo-papillary tumor. Thelesion is well limited inclose relation to thesplenic vein.(A) (B) (C)Untitled-11 21/9/2006, 06:28241POSTPARTUM INCIDENTAL SOLID PSEUDOPAPILLARY... Grezzana et al. RELATOS DE CASOS242 Revista da AMRIGS, Porto Alegre, 50 (3): 240-243, jul.-set. 2006The diagnosis is often casualthrough an image examination madefor other purpose or during the inves-tigation of symptomatic palpable mas-ses in young women, which is respon-sible for the frequently delayed diag-nosis. Mild abdominal pain or earlysatiety occurs due to compression ofneighboring organs. Thus, usuallythese tumors are quite large at the ini-tial presentation. The median size in aseries of 24 patients was 8 cm (range,1-20). In 8% of the cases, the initialpresentation is an acute abdomen (3).The US usually reveals a well-li-mited, cystic or solid-cystic mass. Theecogenicity of the tumor is quite va-riable and depends on the predominan-ce of the cystic (hipoechogenic) or so-lid (hyperechogenic) component. De-pending on the tumor location, preo-perative assessment may suggest thatit originates from the adrenal glandsor that it is a pancreatic endocrine tu-mor (2).CT scans of the abdomen usuallyshow a well-encapsulated mass withboth solid and cystic components.There may be calcifications in the pe-riphery of the mass and also intrave-nous contrast enhancement. However,none of these radiographic features isclearly diagnostic. Despite these fea-tures, the accuracy of CT scan in cha-racterization of cystic pancreatic mas-ses was about 60% in one series (6).Some authors consider that a correctpreoperative diagnosis by CT or USguided aspiration biopsy might be fea-sible (6). In adults, fine needle biopsi-es have become a mainstay in the eva-luation of pancreatic masses, particu-larly those that are cystic in nature. Thereported sensitivity in the diagnosis ofpancreatic malignancies with this tech-nique ranges from 75% to 88% (1).However, some authors consider thebiopsies unnecessary due to the risk ofextra pancreatic dissemination and be-cause the definitive diagnosis is obtai-ned only on anatomopathological ba-sis (4). Differential diagnosis for cys-tadenomas, cystadenocarcinomas,islet cell tumors, and pseudocysts issometimes very difficult. Frozen sec-tions can miss the correct diagnosis,but usually are useful to discard pan-creatic adenocarcinoma (1,2).Around 7-14% of these tumorsdevelop malignant features, with lo-cal invasion and distant metastasis(1,2). Since the malignant potentialof these tumors is low, prognosis isexcellent when complete resection isachieved, even in the presence ofmetastatic disease. Local recurrencesafter complete resection are rare.Some authors have defined this tu-mor as “an infrequent carcinoma ofgood prognosis”. The most commonsites of metastatic disease are the li-ver (42%), lymph nodes (25%) andperitoneal spread (42%). This tumorhas a high propensity for peritonealspread, similar to the ovarian tumors.Fortunately, long–term survival isalso possible even with disseminateddisease (6). Pathologic factors likemitotic rates; nuclear pleomorphismand vascular invasion were not foundto correlate with prognosis. However,an unusual variant with aggressivebehavior, high mitotic rates, and in-creased nuclear pleomorphism was res-ponsible for two deaths in one series(3). Evidence suggests that a translo-cation between chromosomes 13 and17 might be a marker of aggressive-ness (5). Chemotherapy and hormonaltherapy had disappointing results. Iso-lated reports using high dosages of ra-diotherapy have shown partial respon-se (3).The pathologic diagnosis of solidpseudopapillary tumors is based on thepresence of characteristic light micros-copic features. Solid areas alternatingwith pseudopapillary formations, evi-dence of cellular degeneration, inclu-ding cholesterol clefts and aggregatesof foamy histiocytes, nuclear groovesand aggregates of hyaline cytoplasmicglobules are found, at least focally (3).Solid pseudopapillary tumors of thepancreas have a characteristic immu-nophenotype. Markers of acinar (tryp-sin, chemotrypsin) and ductal (glyco-proteins) differentiation are consis-tently negative. The immunoreactivi-ty to vimentin, á-1-antitrypsin, á-1-an-tichymotripsin, phospholipase A2, Leu–M1 and Ki-M1P is usual, but most ofthe neuroendocrine markers (NSE, sy-naptophisyn, chromogranin) are nega-tive, whereas others (CD 56) are posi-tive. Therefore, the absence of distinctimmunoreactivity pattern lessens anyphenotypic relation with the cell liningof the pancreas (2,3).The excision of the tumor is thetreatment of choice and most of theselesions can be managed with local ex-cision or distal pancreatectomy. In thecase of tumors in the head of the pan-creas, a pancreaticoduodenectomywith pylorus-preserving technique isadvised. Liver metastases are resectedwhen possible. Recurrences occur in2-6% after complete removal (1,2).Even in the reported cases that wereconsidered malignant, the survival ofpatients was prolonged after adequatesurgical resection. Long survival withmetastasis or residual disease has beenrelated (4,6).In summary, solid pseudopapilla-ry pancreatic tumors represent an in-termediate group in terms of malig-nant disease of the pancreas. Al-though rare, they should always bein mind when a mass in a young wo-man is assessed. Radical surgical re-section is advised to avoid local anddistant recurrences.R EFERÊNCIASBIBLIOGRÁFICAS1. NADLER EP, NOVIKOV A, LANDZ-BERG BR POCHAPIN MB, CENTENOB, FAHEY T, SPIGLAND N. The use ofendoscopic ultrasound in the diagnosisof solid psudopapillary tumors of the pan-creas in children. J Pediatr Surg, 2002;37:1370-73.2. CANZONIERI V, BERETTA M, BUO-NADONNA A, LIBRA M, VASQUEZE, BARBAGALLO E, BEARZ A, BE-RETTA S. Solid pseudopapillary tumourof the pancreas. Lancet Oncol, 2003; 4:255-6.3. MARTIN RCG, KLIMSTRA DS, BREN-NAN MF, CONLON KC. Solid-pseudo-papillary tumor of the pancreas: a surgi-cal enigma? Ann Surg Oncol, 2001; Jan-Feb;9(1):35-40.4. PODEVIN J, TRIAU S, MIRALLIÈ E,LE BORGNE J. Solid-pseudopapillaryUntitled-11 21/9/2006, 06:28242POSTPARTUM INCIDENTAL SOLID PSEUDOPAPILLARY... Grezzana et al. RELATOS DE CASOSRevista da AMRIGS, Porto Alegre, 50 (3): 240-243, jul.-set. 2006 243tumor of the pancreas: a clinical study offive cases, and review of the literature.Ann Chirur, 2003; 128: 543-48.5. SIRVENT N, MONPOUX F, VARINI JP,BORNET P, TOMMASI C, MENNE-GUZ C, MARIANI R.Tumeur kystiqueet papillaire du pancréas: une case rarede masse abdominale. Arch Pédiatr,1998; 5: 637-40.6. HONG K, TAN PH, THANG CH, OOILL. Solid Pseudopapillary tumour of thepancreas. ANZ J Surg, 2003; 73: 410-15.7. HUERTA S, JANSSEN M, MARCUSDR. Solid pseudopapillary tumor of thepancreas diagnosed postpartum. Surgery,2005; 137:480-1.Untitled-11 21/9/2006, 06:28243

Postpartum incidental solid pseudopapillary tumor of the pancreas : case report

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Postpartum incidental solid pseudopapillary tumor of the pancreas : case report

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