Desmoid tumor of the pancreas: a case report.

Żaneta Słowik-Moczydłowska,Jadwiga Małdyk,Przemysław Kluge,Andrzej Kamiński,Robert Rogulski,Anna Piotrowska

doi:10.1186/s13256-015-0591-y

Żaneta Słowik-Moczydłowska, Jadwiga Małdyk + Show 4 more

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https://doi.org/10.1186/s13256-015-0591-y

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Abstract

IntroductionDesmoid tumor is a rare, benign, usually asymptomatic fibromatous lesion. The etiology is unknown and the diagnosis is based on histopathological examination. The treatment is complete resection of the tumor. Pancreatic desmoid tumor is extremely rare. In the literature there have been only 11 cases described, most of them as solid or solid-cystic masses. We report the case of a patient with an isolated cystic pancreatic desmoid tumor that is, to the best of our knowledge, the second reported case.Case presentationA 13-year old Caucasian boy presented with recurrent pain of two months’ duration in the left hypochondrium of his abdomen. An ultrasound examination and computed tomography scan revealed the presence of a cystic mass located in his splenic hilum, tightly adjacent to the pancreatic tail. A splenic cyst was suspected. Operative findings showed a 10x10cm cystic mass tightly connected to the pancreatic tail and left colonic flexure, adherent to the spleen, splenic vein and artery. Distal splenopancreatectomy with en bloc resection of the left colonic flexure was performed. Histological analysis confirmed that the resection was complete. The mass had infiltrated the pancreatic parenchyma. All tumor cells were positive for anti-beta-catenin staining characteristic for desmoid tumor. No abnormalities in the spleen and colon were found.ConclusionsIsolated sporadic pancreatic desmoid tumor with cyst formation is extremely rare and its diagnosis can be difficult, especially because of uncharacteristic symptoms and radiological findings, as in our patient. This case report should be of interest not only to surgeons, as the treatment of choice is radical resection, but also gastroenterologists, considering it is in close relation with familial adenomatous polyposis, and oncologists as the reason for differentiation with other pancreatic tumors.

Highlights

Desmoid tumor is a rare, benign, usually asymptomatic fibromatous lesion
Isolated sporadic pancreatic desmoid tumor with cyst formation is extremely rare and its diagnosis can be difficult, especially because of uncharacteristic symptoms and radiological findings, as in our patient. This case report should be of interest to surgeons, as the treatment of choice is radical resection, and gastroenterologists, considering it is in close relation with familial adenomatous polyposis, and oncologists as the reason for differentiation with other pancreatic tumors
We report the case of a patient with an isolated cystic mass located in the pancreatic tail diagnosed by betacatenin immunohistochemical staining as a Desmoid tumor (DT); it is, to the best of our knowledge, the second reported case

Summary

Conclusions

Isolated sporadic pancreatic desmoid tumor with cyst formation is extremely rare and its diagnosis can be difficult, such as in our case, where it preoperatively presented as a splenic cyst. It is considered that when possible, the treatment of choice for DT is radical resection with clear margins, as in our patient. Consent Written informed consent was obtained from the patient’s legal guardian(s) for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal. Authors’ contributions ZSM, RR and AP participated in the whole diagnostic and treatment process of the described patient. AK conceived of the study, participated in its design and coordination and helped to draft the manuscript. All authors read and approved the final manuscript

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