title: Squamous Cell Carcinoma of the Pancreas in a Patient with Germline BRCA2 Mutation-Response to Neoadjuvant Radiochemotherapy.
abstract: Primary squamous cell carcinoma of the pancreas is a rare malignant neoplasia, accounting for approximately 0.5-2% of all malignant pancreatic tumors. These lesions are characterized by poor prognosis. Here we report on a case of a 57-year-old female patient with known BRCA2 germline mutation presenting with primary squamous cell carcinoma of the pancreas as the only malignancy. The tumor was locally advanced at the first presentation but responded almost completely to neoadjuvant radio-chemotherapy. Our case highlights the facts (i) that pancreatic carcinomas belong to the tumor spectrum of patients with the BRCA2-associated hereditary breast and ovarian cancer syndrome (HBOC) and (ii) that tumors of the pancreas can represent the first or even the only manifestation of HBOC. Furthermore, this case of a nonkeratinizing squamous cell carcinoma indicates that HBOC-associated carcinomas of the pancreas might be characterized by a broader morphological spectrum than was previously thought. Since BRCA mutations cause deficiency of DNA double-strand breakage repair in tumors, neoadjuvant treatment regimens might become a reasonable option in HBOC-associated pancreatic carcinomas. To our knowledge, this is the first reported case of a primary pancreatic squamous cell carcinoma in a patient with this particular genetic background of BRCA2-associated HBOC.
authors: Institute of Pathology, University Hospital Cologne, Kerpener Straße 62, 50924 Cologne, Germany.
authors: Anne M
authors: AM
authors: Schultheis
authors: Evangelisches Krankenhaus Köln-Kalk, Cologne, Germany.
authors: Gia Phuong
authors: GP
authors: Nguyen
authors: Institute of Pathology, University Hospital Cologne, Kerpener Straße 62, 50924 Cologne, Germany.
authors: Monika
authors: M
authors: Ortmann
authors: Evangelisches Krankenhaus Köln-Kalk, Cologne, Germany.
authors: Wolfgang
authors: W
authors: Kruis
authors: Institute of Pathology, University Hospital Cologne, Kerpener Straße 62, 50924 Cologne, Germany.
authors: Reinhard
authors: R
authors: Büttner
authors: Institute of Pathology, University Hospital Cologne, Kerpener Straße 62, 50924 Cologne, Germany ; Institute of Pathology, University Hospital Göttingen, Göttingen, Germany.
authors: Hans-Ulrich
authors: HU
authors: Schildhaus
authors: Institute of Pathology, University Hospital Cologne, Kerpener Straße 62, 50924 Cologne, Germany.
authors: Birgid
authors: B
authors: Markiefka