A case of accessory spleen located in the tail of the pancreas in a stillbirth male foetus is reported. The congenital anomaly was revealed at autopsy. The intrapancreatic
spleen was well demarcated and was composed of red and white pulp; however, same pancreatic ducts were intermingled with the splenic parenchyma. As well as the intrapancreatic lesion another minute accessory spleen was also found at the hilum of the proper organ. Since a lack of morphological features of trisomy 13 syndrome were found in the foetus, the ectopic spleens were regarded as incidental findings

Bukharin, S.

Burdan, F.

Fronczek, A.

Szumiło, J.

English

Via Medica Journals

Folia Morphol. Vol. 71, No. 1, pp. 45–47Copyright © 2012 Via MedicaISSN 0015–5659www.fm.viamedica.plC A S E   R E P O R T45Address for correspondence: J. Szumiło, MD, PhD, Department of Clinical Pathomorphology, Medical University of Lublin,ul. Jaczewskiego 8, 20–059 Lublin, Poland, tel: +48 81 718 73 25; fax: +48 81 718 73 24, e-mail: jszumilo@wp.plIntrapancreatic accessory spleenJ. Szumiło1, A. Fronczek1, S. Bukharin1, F. Burdan21Department of Clinical Pathomorphology, Medical University of Lublin, Poland2Depatment of Human Anatomy, Medical University of Lublin, Poland[Received 23 September 2011; Accepted 1 December 2011]A case of accessory spleen located in the tail of the pancreas in a stillbirth malefoetus is reported. The congenital anomaly was revealed at autopsy. The intra-pancreatic spleen was well demarcated and was composed of red and whitepulp; however, same pancreatic ducts were intermingled with the splenic pa-renchyma. As well as the intrapancreatic lesion another minute accessory spleenwas also found at the hilum of the proper organ. Since a lack of morphologicalfeatures of trisomy 13 syndrome were found in the foetus, the ectopic spleenswere regarded as incidental findings. (Folia Morphol 2012; 71, 1: 45–47)Key words: accessory spleen, anatomical variation, congenitalmalformationINTRODUCTIONAccessory spleens are a relatively common con-genital abnormalities, and they are found in 10–30%of non-selected autopsies [3]. Moreover, the con-trast-enhanced abdominal computed tomography(CT) examination revealed that the anomaly is visi-ble in about 16% of consecutive patients [12]. Itsprevalence is much higher in children who have un-dergone splenectomy — 45–65% [1]. At autopsymost accessory organs (80%) are located at or closeto the hilum of the normal spleen [3, 12]. The sec-ond most common site is the tail of the pancreas[3]. However, in the CT examination intrapancreaticlocation was rare — seen in about 1.3% of patientswith accessory organs [12]. In the current papera new case of that anomaly is presented.CASE REPORTA male stillborn was delivered at the 39th weekof gestation by a 34-year-old woman (G4P4A0) atthe Gynaecological and Obstetric Ward of Lublin’District General Hospital in February 2011. An au-topsy of the foetus (weight 3030 g; length 510 mm)was performed at the Department of Clinical Patho-morphology of the Medical University of Lublin. Thefeatures of intrauterine asphyxia with minute ecchy-moses of the pericardial sac, focal pulmonary intra-alveolar haemorrhages, and amniotic fluid aspira-tion were revealed. Major congenital malformationswere not found. The intrauterine death was mostlikely associated with the pathology of the placentaand the umbilical cord; however, it was not receivedfor the gross and histological examination.Upon dissection of the abdominal cavity, a small,6 mm accessory spleen located at the hilum of themain proper organ was seen. Furthermore, at the lon-gitudinal section of the pancreas a minute dark-redarea in the tail was unexpectedly noted arousing sus-picion of local haemorrhage. However, the microscopicexamination showed another — an intrapancreaticaccessory spleen (Fig. 1). The lesion was well circum-scribed but unencapsulated. It was composed of thetypical red and white pulp (Fig. 2). A few pancreaticducts lined by one layer of the cuboidal epitheliumand entrapped in the spleen were also observed.To highlight some details of the morphology ofthe pancreas a set of immunohistochemical reactionswere performed on formalin-fixed paraffin-embed-46Folia Morphol., 2012, Vol. 71, No. 1ded sections. Monoclonal mouse anti-human anti-bodies against epithelial cells — pancytokeratin(clone MNF116; dilution 1:100) as well as againstlymphocytes — leukocyte common antigen CD45(clones 2B11+PD7/26; dilution 1:100), B cells —CD20 (clone L-26; 1:100), and T cells — CD3 (clone F7,2.38; dilution 1:50) were applied. The incuba-tion with antibodies was performed for 0.5 hoursat normal temperature. According to the manufac-turer’s directions the main reactions were precededby antigen retrieval procedures, i.e. for pancytoke-ratin — pretreatment with proteinase K (20 mL/mL)for 4 min, for CD45 incubation with 0.01 M citratebuffer (pH 6.0) in microwave oven (3 ¥ 5 min), andfor CD20 and CD3 incubation in water bath (temp95oC) with target retrieval solution (pH 9.0) for20 min. EnVisionTM+/HRP visualisation system wasused for 0.5 hours at normal temperature and then3,3’-diamonobenzidine (DAB) as a chromogen wasapplied. All reagents were from Dako, Denmark.Positive immunostaining for cytokeratin was seenin epithelial cells of pancreatic acini and ducts(Fig. 3A). Lymphocytes forming white pulp of the acces-sory intrapancreatic spleen exhibited a strong posi-tive reaction for CD45. They were predominantlyCD20-positive B cells (Fig. 3B), whereas CD3-posi-tive T cells were rare (Fig. 3C).Figure 1. Normal spleen as well as hilar and intrapancreatic(arrow) accessory spleens (haematoxylin & eosin).Figure 2. Well-demarcated intrapancreatic accessory spleencomposed of red and white pulp surrounding a few pancreaticducts (haematoxylin & eosin; objective magn. 5¥).Figure 3. Positive immunostaining for (A) pancytokeratin in epi-thelial cells of pancreatic acini and ducts, as well as for (B) CD20in B — and for (C) CD3 in T cells of white pulp of intrapancreaticspleen (DakoEnVisionTM+/HRP; objective magn. 10¥).ABC47 J. Szumiło et al., Intrapancreatic accessory spleenDISCUSSIONThe accessory spleens originated most likely fromhillocks of mesenchymal cells in the dorsal meso-gastrium, which migrated between leaves of themesentery and did not fuse to create the main spleenat the 5th week of gestation. Nests of those cells maybe entrapped by the pancreatic tail, which, at thesame time, derived from the mass of cells situatedcaudally to the splenic hillocks [4].Some childhood (including that presented here)and probably most adult cases of the intrapancre-atic spleen seem to be sporadic lesions without ge-netic predisposition. However, splenopancreaticfield abnormalities are typical for the trisomy13 syndrome (Patau syndrome) [2, 5]. The incidenceof accessory spleens among individuals with suchchromosomal abnormality is much higher than inthe normal population, reaching 30–60% [5]. In thelarge series by Gomi et al. [2] the classification ofgross splenopancreatic abnormalities, intrapancre-atic accessory spleens, and/or fusion between thepancreatic tail and the spleen/accessory spleen wasfound in all children with trisomy 13 (n = 21). Con-versley, those anomalies were noted just in sevencases of non-trisomic controls (n = 1060). Further-more, pancreatic dysplasia, including intralobularducts with goblet cells, microcysts, and ductulo-insular complexes, are typical of the trisomy [5].Splenopancreatic field abnormalities were also re-ported in new-borns with an abnormal karyotype(47, XX + 21) and dysmorphic features, osteocran-iostenosis, and prune belly syndrome or with con-genital heart malformations [13].Since intrapancreatic spleens are usually asymp-tomatic, they are regarded as lesions without spe-cial clinical significance. Occasionally, they can bea source of secondary pathological lesions like theepidermoid cyst [7, 8], serous cystic neoplasm [6],or littoral cell angioma [14]. Nevertheless, the mostimportant clinical problem is their incorrect diagno-sis as a true pancreatic neoplasm, usually a non-se-creting endocrine tumour [10, 11]. Appropriate dia-gnosis is a crucial for further treatment. The intra-pancreatic spleen does not require any treatment,but for true tumours surgical resection is recom-mended. Radionuclide imaging with 99mTc-sulfurcolloid or 99mTc-tagged heat-damage erythrocytes aswell as superparamagnetic ion oxide (SPIO)-en-hanced magnetic resonance imaging or intravenous-ly enhanced ultrasonography are non-invasive andsensitive methods of visualisation of the splenic pa-renchyma [9, 10]. Endoscopic ultrasound-guidedfine-needle aspiration biopsy can also be helpful inconfirmation of the diagnosis [11]. Large aggregatesof CD8-positive spindle cells found in smears seemto be typical of intrapancreatic accessory spleenscomplicated by epidermoid cyst [8].REFERENCES1. Eraklis AJ, Filler RM (1972) Splenectomy in childhood:a review of 1413 cases. J Pediatr Surg, 7: 382–388.2. Gomi K, Sato Y, Tanaka M, Ijiri R, Kato K, Aoki I, Tanaka Y(2009) Specificity of splenopancreatic field abnormali-ty in trisomy 13 syndrome: macroscopic and histologi-cal analysis in 21 autopsy cases. Pathol Int, 59: 147––151.3. Halpert B, Gyorkey F (1959) Lesions observed in acces-sory spleens of 311 patients. J Clin Pathol, 32: 165––168.4. Harding R, Bocking AD (2001) Foetal growth and de-velopment. Cambridge University Press, Cambridge.5. Hashida Y, Jaffe R, Yunis EJ (1983) Pancreatic patholo-gy in trisomy 13: specificity of the morphologic lesion.Pediatr Pathol, 1: 169–178.6. Hori S, Nara S, Shimada K, Ojima H, Kanai Y, Hiraoka N(2010) Serous cystic neoplasm in an intrapancreaticaccessory spleen. Pathol Int, 60: 681–684.7. Horn AJ, Lele SM (2011) Epidermoid cyst occurring within an intrapancreatic accessory spleen. A case reportand review of the literature. JOP, 12: 279–282.8. Hwang HS, Lee SS, Kim SC, Seo DW, Kim J (2011) Intra-pancreatic accessory spleen: clinicopathologic analysisof 12 cases. Pancreas, 40: 956–965.9. Kim SH, Lee JM, Han JK, Lee JY, Kim KW, Cho KC, Choi BI(2008) Intrapancreatic accessory spleen: findings on MRimaging, CT, US and scintigraphy, and the pathologicanalysis. Korean J Radiol, 9: 162–174.10. Kurmann A, Michel JM, Stauffer E, Egger B (2010) In-trapancreatic accessory spleen misdiagnosed as a non--secreting endocrine tumor: case report and review ofthe literature. Case Rep Gastroenterol, 4: 210–214.11. Lin J, Jing X (2010) Fine-needle aspiration of intrapan-creatic accessory spleen, mimic of pancreatic neo-plasms. Arch Pathol Lab Med, 2134: 1474–1478.12. Mortele KJ, Mortele B, Silverman SG (2004) CT featuresof the accessory spleen. Am J Roentgenol, 183: 1653––1657.13. Peres LC, de Sales Barbosa GHT, Cerata RS, Nassif CM,de Pina-Neto JM, Giuliani LR, Martinhago CD, Gomy I(2004) Splenopancreatic field abnormality is not uniqueto trisomy 13. Pediatr Dev Pathol, 7: 91–94.14. Pilz JB, Sperschneider T, Lutz T, Loosli B, Maurer CA(2011) Littoral cell angioma in main and accessory in-trapancreatic spleen presenting as splenic rupture. AmJ Surg, 201: e15–e17.

Intrapancreatic accessory spleen

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Intrapancreatic accessory spleen

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