Schwannomas occurring in the pancreatic head are rare benign non-recurring mesenchymal neoplasms and are reported to show classic morphologic features. Herein we report a case of a 62 year old male presenting with a 5 cm mass in the pancreatic head encasing the portal vein and the truncus coeliacus. Preoperative fine needle aspiration revealed malignant tumour cells consistent with a moderately differentiated adenocarcinoma. A Whipple surgery was performed after palliative chemotherapy. Histological evaluation revealed a multinodular unencapsulated tumour with focal infiltration into pancreas parenchyma and a striking microcystic/reticular growth pattern. Anastomosing and intersecting strands of spindle cells with eosinophilic cytoplasm set in a myxoid partly collagenous stroma were observed. The tumour cell nuclei were round oval and tapered and showed inconspicuous small nucleoli. Degenerative nuclear atypia was seen. Mitotic activity was sparse (1/50 HPF). Pleomorphism or necrosis was absent. The tumour cells showed strong nuclear and cytoplasmic positivity for S-100 protein, and focal positivity for glial fibrillary acidic protein. The diagnosis of a microcystic/reticular schwannoma was made. The awareness of and, to some extent, the knowledge about this rare tumour are needed to achieve the correct diagnosis and to avoid confusion, especially with malignant pancreatic neoplasms.

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http://dx.doi.org/10.1111/j.1440-1827.2010.02614.xDOI Listing

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  • * The presence of this serpentine pattern correlated with the fusion status, suggesting it could be used as a predictive marker, along with tumor size, patient age, and location, while advanced RNA techniques confirmed the fusion's existence.
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Introduction And Importance: Diagnosis and treatment of rare diseases are challenging because experience and evidence are limited. Primary tracheal tumors have a low prevalence but awareness of these is important to avoid misdiagnoses. We present a first case of a tracheal microcystic reticular schwannoma in which diagnosis and treatment recommendations were revised several times resulting in months of anxious uncertainty for the patient before complete resection and extensive histopathologic examination were performed.

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