Solid pseudopapillary tumor associated with agenesis of the dorsal pancreas: a case report

ABSTRACT

Solid pseudopapillary tumors (SPTs) of the pancreas are rare tumors with low potential for malignancy, uncertain lineage, and favorable prognosis in most cases. The SPT has an excellent prognosis, and the standard treatment is surgical resection. The agenesis of the dorsal pancreas (ADP) is an extremely rare type of congenital pancreatic malformation and is characterized by partial or total loss of the body and tail of the gland. Its association with SPT has been reported only in two studies. We report a case of SPT associated with total ADP. A 36-year-old woman was diagnosed with a complex mass on pancreatic head topography, measuring 7.8×5.5cm, associated with complete agenesis of the body and tail of the pancreas. She underwent gastropancreatoduodenectomy with a successful postoperative outcome. The anatomopathological examination suggested an SPT and it was confirmed by immunohistochemistry.

RESUMO

Os tumores sólidos pseudopapilares (TSPs) do pâncreas são tumores raros com baixo potencial de malignidade, linhagem incerta e prognóstico favorável na maioria dos casos. O SPT tem excelente prognóstico e o tratamento padrão é a ressecção cirúrgica. A agenesia dorsal do pâncreas (ADP) é um tipo extremamente raro de malformação congênita do pâncreas e é caracterizada pela perda parcial ou total do corpo e cauda da glândula. Sua associação com TSP foi relatada apenas em dois estudos. Relatamos um caso de TSP associado à ADP total. Uma mulher de 36 anos foi diagnosticada com uma massa complexa na topografia da cabeça do pâncreas, medindo 7,8×5,5cm, associada à agenesia completa do corpo e cauda do pâncreas. Ela foi submetida à gastropancreatoduodenectomia com sucesso pós-operatório. O exame anatomopatológico sugeriu TSP e foi confirmado por imunohistoquímica.

Keywords:

Pancreatic neoplasms - Pancreatic cancer - Pancreatectomy - Pancreas - Agenesis.

Descritores:

Neoplasias pancreáticas - Câncer pancreático - Pancreatectomia - Pâncreas - Agenesia

Publication History

Received: 29 March 2021

Accepted: 05 August 2021

Article published online:
07 December 2021

• REFERENCES

• 1 Nakamura Y, Egami K, Maeda S, Hosone M, Onda M. Solid and papillary tumor of the pancreas complicating agenesis of the dorsal pancreas. J Hepatobiliary Pancreat Surg 2001; 8 (05) 485-489
• 2 Ulusan S, Bal N, Kizilkilic O, Bolat F, Yildirim S, Yildirim T. et al. Solid-pseudopapillary tumour of the pancreas associated with dorsal agenesis. Br J Radiol 2005; Jun; 78 (929) 441-443
• 3 Cienfuegos J, Lozano M, Rotellar F, Martí P, Pedano N, Arredondo J. et al. Solid pseudopapillary tumor of the pancreas (SPPT). Still an unsolved enigma. Rev Esp Enferm Dig 2010; Dec; 102 (12) 722-728
• 4 Frantz VK. Papillary tumors of the páncreas: benign or malignant? Tumors of the pancreas. Atlas of tumor Pathology, 1st ed. Washington DC: Armed Forces Institute of Pathology; 1959: 32-33
• 5 Klöppel G, Solcia E, Longnecker DS, Capella C, Sobin LH. Histological typing of tumors of the exocrine pancreas. 2nd. Berlin: Springer;; 1996
• 6 Costa-Neto G, Amico E, Costa G. Tumor sólidocístico pseudopapilar do pân- creas (tumor de Frantz). Estudo de quatro casos. Arq Gastroenterol 2004; Dez; 41 (04) 259-262
• 7 Law JK, Ahmed A, Singh VK, Akshintala VS, Olson MT, Raman SP. et al. A systematic review of solid-pseudopapillary neoplasms. Are these rare lesions? Pancreas 2014; Apr; 43 (03) 331-337
• 8 Guimarães AB, Guimarães CA, Manso JEF. Agenesia ou pseudoagenesia do pâncreas dorsal. Rev Col Bras Cir 2015; 42 (05) 352-355
• 9 Cienfuegos JA, Rotellar F, Salguero J, Benito A, Solórzano JL, Sangro B. Agenesis of the dorsal pancreas: systematic review of a clinical challenge. Rev Esp Enferm Dig 2016; Aug; 108 (08) 479-484
• 10 Antoniou E A, Damaskos C, Garmpis N, Salakos C, Margonis GA, Kontzglou K. et al. Solid pseudopapillary tumor of the pancreas: a singlecenter experience and a review of the literature. In Vivo 2017; Jul/Aug; 31 (04) 301-510
• 11 Cerron-Vela CR, Navarro PM, Araujo-Banchon WJ. Rev Facul Cienc Méd Córdoba. 2020; 77 (01) 1-6
• 12 Salla C, Chatzipantelis P, Konstantinou P, Karoumpalis I, Pantazopoulou A, Dappola V. Endoscopic ultrasound-guided fineneedle aspiration cytology diagnosis of solid pseudopapillary tumor of the pancreas: a case report and literature review. World J Gastroenterol 2007; Oct; 13 (38) 5158-5163
• 13 Santini D, Poli F, Lega S. Solid-papillary tumors of the pancreas: histopathology. JOP 2006; Jan; 7 (01) 131-136
• 14 Schnedl WJ, Piswanger-Soelkner C, Wallner SJ, Reittner P, Krause R, Lipp RW. et al. Agenesis of the dorsal pancreas and associated diseases. Dig Dis Sci 2009; Mar; 54 (03) 481-487
• 15 Mei W, Cao F, Li F. Two cases of agenesis of the dorsal pancreas and a review of the literature. BMC Gastroenterol 2020; Apr; 20: 94
• 16 Erotokritou A, Gerharz CD, Sagir A. Agenesis of dorsal pancreas associated with pancreatic neuroendocrine tumor: a case report and review of the literature. J Med Case Rep 2018; Jun; 12 (01) 185