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| CORRESPONDENCE |
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| Year : 2017 | Volume
: 13
| Issue : 2 | Page : 378-380 |
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Intrapancreatic mass: A rare cause
Maximilian Sohn1, Martin Fuchs2, Helmut Rohrbach3, Igors Iesalnieks1, Ayman Agha1, Friederieke Liesche1
1 Department of General, Abdominal, Endocrine and Minimally Invasive Surgery, Bogenhausen Hospital, Munich City Hospital Ltd., München, Germany
2 Department of Gastroenterology, Hepatology and Gastroenterologic Oncology, Bogenhausen Hospital, Munich City Hospital Ltd., München, Germany
3 Department of Pathology, Bogenhausen Hospital, Munich City Hospital Ltd., München, Germany
| Date of Web Publication | 23-Jun-2017 |
Correspondence Address:
Maximilian Sohn
Department of General, Abdominal, Endocrine and Minimally Invasive Surgery, Städtisches Klinikum München GmbH - Klinikum Bogenhausen, Englschalkinger Straße 77, 81925 München
Germany
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0973-1482.193124
We found a case of pancreatic extraintestinal gastrointestinal stroma tumor (pEGIST) in 2014. The patient, initially suspected to suffer from pancreatic adenocarcinoma, underwent open left hemipancreatectomy and en bloc splenectomy in May 2014. Postoperative histopathology showed the unexpected manifestation of a pEGIST. Recovery was well, and a 23-month follow-up was free from recurrency by now. Keywords: Gastrointestinal stroma tumor, pancreatic extraintestinal gastrointestinal stroma tumor, pancreatic gastrointestinal stroma tumor
How to cite this article:
Sohn M, Fuchs M, Rohrbach H, Iesalnieks I, Agha A, Liesche F. Intrapancreatic mass: A rare cause. J Can Res Ther 2017;13:378-80 |
| > Introduction | |  |
Pancreatic extraintestinal gastrointestinal stroma tumor (pEGIST) is reported to be extremely rare. The annual incidence is estimated to be 3/1,000,000.[1] By April 2016, only 33 cases have been published. This seldom entity of pancreatic tumors characterized by the proliferation of spindle or epithelioid cells and the expression of CD117 is suspected to arise from intrapancreatic Cajal cells.
| > Case Report | | |
We report on a 65-year-old woman presenting with an intrapancreatic mass. A history of left-sided breast cancer in 2009 was reported. At the time, the therapeutical regime had included neoadjuvant chemotherapy, followed by local surgery, adjuvant radiation, and antihormonal therapy. Current computed tomography scans were performed for systemic restaging, while the patient did not suffer from any symptoms.
Physical exam showed an alert, mentally orientated patient in good general and nutritional condition with bilaterally ventilated pulmo, rhythmic heart action and soft and painless abdomen on palpation.
Laboratory findings at admission
White blood cell 3.4/nl, Hb 12.4 g/dl, platelet count 217/nl, prothrombin time 99%, international normalized ratio 1, creatinin 0.8 mg/dl, glomerular filtration rate 84 ml/min, C-reactive protein 1.6 mg/L, bilirubin 1.0 mg/dl, aspartate aminotransferase 28 U/L, alanine aminotransferase 15 U/L, gamma-glutamyltransferase 15 U/L, alkaline phosphatase 34 U/L, pancreatic-amylase 29.7 U/L, carcinoembryonic antigen 1.7 μg/L, and cancer antigen 19–9 9 U/ml.
Abdominal computed tomography
A 54.38 mm × 26.99 mm measuring inhomogeneous pancreatic tail mass suspected for primary pancreatic cancer or metastatic growth [Figure 1]. | Figure 1: Computed tomography scan: 54.38 mm × 26.99 mm measuring inhomogeneous pancreatic tail mass
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Left-sided hemipancreatectomy and en bloc splenectomy was performed. The postoperative course was regular except for a small, self-limiting pancreatic fistula, and the patient was discharged on postoperative day 7.
Histological examination showed an intrapancreatic GIST with a moderate risk of malignancy, mitotic count <5/50 high power fields, four tumor-free lymph nodes, pT3, pN0 (0/4), L0, V0, Pn0, and R0. Molecular genetic examination showed in frame deletion of two nucleotides in exon 11 of c-Kit [Figure 2],[Figure 3],[Figure 4],[Figure 5]. | Figure 2: Overview transitional zone pancreas-gastrointestinal stroma tumor, enlargement: ×40
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 | Figure 3: Enlargement: ×400 = HPF, gastrointestinal stroma tumor with mitosis
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 | Figure 4: Gastrointestinal stroma tumor detailed: Enlargement: ×400 = HPF, immunohistochemistry CD117 (c-Kit)
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| > Discussion | | |
Primarily suspected to be pancreatic cancer, the above-described tumor was identified as intrapancreatic GIST (pEGIST) by histopathological and molecular genetic examination. Because of the moderate risk of malignancy, we did not recommend adjuvant therapy with imatinib but focused close-meshed follow-up. Twenty-three months after resection, the patient is in good clinical condition without any signs of tumor recurrence. pEGIST is reported to be extremely rare. By April 2016, only 33 cases have been published. The pathogenesis is discussed controversially. The current model of gastrointestinal stroma tumors is based on interstitial cells of Cajal to be the source of GIST, which is furthermore characterized by the proliferation of spindle or epithelioid cells and the expression of CD117.[2],[3] Yamaura et al. first identified CD117 and c-Kit positive cells in human exocrine pancreas which potentially could serve as the origin of GIST.[4] While some authors propose that pEGIST develops as a result of the extramural expansion of primary intramural GIST of the gut,[2] today some more recent references describe Cajal cells to coexist in pancreatic tissue which could be demonstrated for the first time by Popescu et al. in 2005 in rat and in human exocrine pancreas.[5] Thereby, primary intrapancreatic development of pEGIST may be possible. However, gastrointestinal stroma tumors should be included in the differential diagnosis of pancreatic mass since the therapeutical regimen differs from that of other malignant tumors of the pancreas such as adenocarcinoma. Small tumors can be treated by organ sparing resection if an acceptable safety distance of 2–3 cm is realizable. A positive effect of lymphadenectomy has not been reported. Extended resection as multivisceral resection of locally advanced tumors can be accepted as a potentially curative approach if R0-resection is achieved.
| > Conclusion | | |
As the first step, we recommend intraoperative exploration of the tumor with a frozen section in case of unknown histologic entity to plan an individual operative strategy. If preoperative findings show a nonresectable tumor and the diagnosis of GIST is histologically proven, neoadjuvant therapy could be discussed as a nonestablished attempt for downsizing of the tumor under study conditions. Since the biological behavior of pEGIST is virtually unknown, lifelong follow-up should be established.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| > References | | |
| 1. | Aziret M, Çetinkünar S, Aktas E, Irkörücü O, Bali I, Erdem H. Pancreatic gastrointestinal stromal tumor after upper gastrointestinal hemorrhage and performance of whipple procedure: A case report and literature review. Am J Case Rep 2015;16:509-13.  |
| 2. | Beltrame V, Gruppo M, Pastorelli D, Pizzi S, Merigliano S, Sperti C. Extra-gastrointestinal stromal tumor of the pancreas: Case report and review of the literature. World J Surg Oncol 2014;12:105. [ PUBMED] |
| 3. | Padhi S, Sarangi R, Mallick S. Pancreatic extragastrointestinal stromal tumors, interstitial Cajal like cells, and telocytes. JOP 2013;14:1-14. [ PUBMED] |
| 4. | Yamaura K, Kato K, Miyazawa M, Haba Y, Muramatsu A, Miyata K, et al. Stromal tumor of the pancreas with expression of c-kit protein: Report of a case. J Gastroenterol Hepatol 2004;19:467-70. |
| 5. | Popescu L, Hinescu M, Radu E. CD117/c-kit positive interstitial (Cajal-like) cells in human pancreas. J Cell Mol Med 2005;9:738-9. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]
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