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      Pancreatic schwannoma: A rare case and a brief literature review

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          • Pancreatic schwannoma is an extremely rare tumor.

          • Pancreatic schwannoma was stained with S-100 and vimentin.

          • The preoperative diagnosis of pancreatic schwannoma is an important in terms of choose surgery method.

          • Surgical resection is a curative treatment method for pancreatic schwannoma.



          Pancreatic schwannoma (PS) is an extremly rare benign tumor. Less than 50 cases of pancreatic schwannoma have been described in the English literature over the past thirty years.

          Presentation of case report

          A 63-year-old female underwent left modified radical mastectomy 2 years ago due to breast cancer. During her routine check-up, a 65 × 63 × 55 mm measured calcified, well-demarcated, cystic-mass having septations and calcifications that localized to the pancreatic head was detected by abdominal computerized tomography. She was asymptomatic and her tumor markers were in normal ranges. A standard Whipple procedure was performed, and the histo-pathological diagnosis of the resected specimen was reported as ancient schwannoma with clear surgical margins. Patient’s postoperative course was eventful. She had a biliary leakage after surgery which was managed conservatively. She is under follow-up.


          Pancreatic schwannoma also known as neurilemoma or neuroma is a slowly growing, encapsulated, mostly benign tumor with smooth well-delineated margins that originates from myelin producing schwann cells located on the nerve sheath of the peripheral epineurium of either the sympathetic or parasympathetic autonomic fibers. PS’s are extremly rare. The head of pancreas being involved in the vast majority of cases (40%), followed by its body (20%). Management of pancreatic schwannomas remains largely controversial. Both enucleation and radical surgical resections have revealed great therapeutic efficiency. with a well prognosis without recurrences.


          Although rare, PS’s should be considered in the differential diagnosis of the other solid or cystic masses of the pancreas.

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          Most cited references 21

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          Value of S-100 protein in the diagnosis of soft tissue tumors with particular reference to benign and malignant Schwann cell tumors.

          Two hundred and two benign and malignant soft tissue lesions were studied for the presence of S-100 protein by means of the peroxidase-antiperoxidase technique on formalin-fixed, paraffin-embedded tissue. Virtually all benign nerve sheath tumors (neurofibroma, neurilemoma, and granular cell tumor) contained numerous immunoreactive S-100-positive cells. Only one-half (18 of 36) of malignant schwannomas contained the protein, suggesting that its presence is an expression of differentiation in Schwann cell tumors. S-100 protein was not identified within pure neuroblastic tumors (neuroblastoma, neuroepithelioma) but could be identified within rare cells of the ganglioneuroblastoma and within the Schwann cell component of ganglioneuroma. It was also identified within most melanocytic tumors (cellular blue nevus, clear cell sarcoma, and melanoma). In fact, its constant presence in melanoma indicates that it may prove to be an independently reliable method for diagnosing amelanotic forms. It is also sporadically present within a variety of mesenchymal lesions including lipoma, liposarcoma, synovial chondromatosis, chondrosarcoma, fibromatosis, histiocytosis X, and chordoma. Although S-100 protein is highly characteristic of neural crest-derived tumors, it is not restricted to them and, consequently, must be interpreted cautiously. It may prove helpful in select situations such as the distinction of (a) benign nerve sheath tumors from other benign mesenchymal tumors such as fibrous histiocytomas, (b) cellular neurilemomas from malignant schwannomas, (c) malignant schwannomas from conventional fibrosarcoma (d) malignant melanomas from many carcinomas, and, possibly (e) juvenile xanthogranulomas from histiocytosis X.
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            Pancreatic schwannoma: Case report and an updated 30-year review of the literature yielding 47 cases.

            Pancreatic schwannomas are rare neoplasms. Authors briefly describe a 64-year-old female patient with cystic pancreatic schwannoma mimicking other cystic tumors and review the literature. Databases for PubMed were searched for English-language articles from 1980 to 2010 using a list of keywords, as well as references from review articles. Only 41 articles, including 47 cases, have been reported in the English literature. The mean age was 55.7 years (range 20-87 years), with 45% of patients being male. Mean tumor size was 6.2 cm (range 1-20 cm). Tumor location was the head (40%), head and body (6%), body (21%), body and tail (15%), tail (4%), and uncinate process (13%). Thirty-four percent of patients exhibited solid tumors and 60% of patients exhibited cystic tumors. Treatment included pancreaticoduodenectomy (32%), distal pancreatectomy (21%), enucleation (15%), unresectable (4%), refused operation (2%) and the detail of resection was not specified in 26% of patients. No patients died of disease with a mean follow-up of 15.7 mo (range 3-65 mo), although 5 (11%) patients had a malignancy. The tumor size was significantly related to malignant tumor (13.8 ± 6.2 cm for malignancy vs 5.5 ± 4.4 cm for benign, P = 0.001) and cystic formation (7.9 ± 5.9 cm for cystic tumor vs 3.9 ± 2.4 cm for solid tumor, P = 0.005). The preoperative diagnosis of pancreatic schwannoma remains difficult. Cystic pancreatic schwannomas should be considered in the differential diagnosis of cystic neoplasms and pseudocysts. In our case, intraoperative frozen section confirmed the diagnosis of a schwannoma. Simple enucleation may be adequate, if this is possible.
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              Zur kenntnis der neurofibrome

               J Verocay,  J VEROCAY (1910)

                Author and article information

                Int J Surg Case Rep
                Int J Surg Case Rep
                International Journal of Surgery Case Reports
                26 March 2016
                26 March 2016
                : 22
                : 101-104
                [a ]Sakarya University of Faculty of Medicine, Department of General Surgery, Sakarya, Turkey
                [b ]Sakarya University of Faculty of Medicine, Department of Pathology, Sakarya, Turkey
                Author notes
                [* ]Corresponding author. mhmtaziret@ 123456gmail.com
                © 2016 The Authors

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                Case Report


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