Malignant Gastrointestinal Neuroectodermal Tumor: A Case Report and Literary Review for a Rare Differential Diagnosis
Cinzia Baccaro,
Noemi Zorzetti,
Manuela Cuoghi,
Adele Fornelli,
Tania Franceschini,
Sara Coluccelli,
Vincenzo Cennamo,
Giuseppe Giovanni Navarra
Affiliations
Cinzia Baccaro
Department of General Surgery, “A. Costa” Hospital—Alto Reno Terme, 40046 Bologna, Italy
Noemi Zorzetti
Department of General Surgery, “A. Costa” Hospital—Alto Reno Terme, 40046 Bologna, Italy
Manuela Cuoghi
Department of General Surgery, “A. Costa” Hospital—Alto Reno Terme, 40046 Bologna, Italy
Adele Fornelli
Department of Pathological Anatomy, Maggiore “Carlo Alberto Pizzardi” Hospital, 40133 Bologna, Italy
Tania Franceschini
Department of Pathological Anatomy, Maggiore “Carlo Alberto Pizzardi” Hospital, 40133 Bologna, Italy
Sara Coluccelli
Solid Tumor Molecular Pathology Laboratory, Department of Medical and Surgical Sciences (DIMEC), IRCCS Azienda Ospedaliero-Universitaria, 00144 Bologna, Italy
Vincenzo Cennamo
Department of Gastroenterology, Maggiore “Carlo Alberto Pizzardi” Hospital, 40133 Bologna, Italy
Giuseppe Giovanni Navarra
Department of General Surgery, “A. Costa” Hospital—Alto Reno Terme, 40046 Bologna, Italy
Malignant gastrointestinal neuroectodermal tumor (GNET) is an infrequent soft-tissue sarcoma, formerly referred to as clear-cell sarcoma-like gastrointestinal tumor (CCSLGT) and frequently reported in the literature as clear-cell sarcoma of the gastrointestinal tract (CCS-GI); it is characterized by an absence of melanocytic differentiation and the presence of nontumoral osteoclast-like giant cells (OLGCs). The current study reports a case of a 79 year old woman admitted to the emergency department (ED) with symptoms of constipation and intestinal obstruction; a mass was found within the ileal wall necessitating of surgical approach. Immunohistochemically, tumor cells surprisingly had the hallmark of GNETs. Unfamiliarity with tumors with the features of GNETs can easily lead to a misdiagnosis by surgical pathologist. Therefore, comprehensive evaluation, including morphology and additional studies, is required for an appropriated diagnosis. Furthermore, without a high index of suspicion, there is actually no consensus on staging or treatment.
