A peripheral primitive neuroectodermal tumor originating from the pancreas: a case report and review of the literature

Abstract

A peripheral primitive neuroectodermal tumor (pPNET) is a small round cell tumor occurring mostly in children or young adults and categorized into the Ewing sarcoma family of tumors. pPNETs originating from the pancreas are especially rare, and only 25 cases have been reported in the literature. We report a case of a 22-year-old man who had a giant expansive tumor located in the uncinate process of the pancreas, 80 mm in diameter resulting in obstruction in the duodenum. The patient underwent a pancreaticoduodenectomy. The histological examination showed that the pancreatic tumor was composed of atypical small round cells. Immunohistochemical findings were positive for CD99. An Ewing sarcoma breakpoint region 1 gene 22q12 rearrangement was proven by a two-color fluorescence in situ hybridization assay. We diagnosed the tumor as a pPNET of the pancreas, which, according to the literature, is highly aggressive with poor prognosis. A multidisciplinary approach to treat these neoplasms should improve the prognoses.

Cytogenetically confirmed primary Ewing’s sarcoma of the pancreas

“…Nishizawa et al recently reviewed the literature of 25 primary pancreatic ESFTs of which 16 were cytogenetically confirmed. They found a mean age at time of diagnosis of 18.2 years and a median age of 20 years, with no gender predominance 8. Abdominal pain (68%) was the most common presenting finding followed by jaundice and anaemia 8 9.…”

“…Endocrine disorders such as hyperglycaemia and precocious puberty were seen in one and two cases, respectively 9. These tumours have an expansive growth pattern in contrast to the highly infiltrative growth pattern of ductal adenocarcinoma and hence are generally present later in the disease course 8. Most of the tumours occurred in the head of the pancreas (68%) 8 9.…”

“…Most of the tumours occurred in the head of the pancreas (68%) 8 9. At the time of presentation, most of the tumours are large and bulky with an average tumour size of 88 mm reported by Nishizawa and colleagues 8. CT and MRI of the abdomen are important tools to characterise these tumours.…”

A Nonpediatric Extraosseous Ewing Sarcoma of the Pancreas: Differential Diagnosis and Therapeutic Strategies

“…Affected patients can present with anemia, or rarely, hyperglycemia or precocious puberty [5]. When compared to the infiltrative growth pattern of pancreatic adenocarcinoma, EES of the pancreas has an expansile growth pattern; it tends to present late in the disease course [6]. Histologically, there are small round cells (blue on H&E stain) with hyperchromatic nuclei and scant cytoplasm containing neuronal secretory granules, neurofilaments, and pyknotic nuclear granules [7].…”

“…Approximately 30% of all ESFTs are extraosseous, most commonly arising in the soft tissues of the trunk or extremities, but rarely in various locations in the gastrointestinal tract including the biliary tree, stomach, esophagus, and oral cavity. The pancreas is an extremely uncommon extraosseous location, with only 27 cases reported worldwide [5,6]. We report a lethal case of cytogenetically confirmed EES of the pancreas in a young woman who presented with abdominal pain.…”