Sociedad Española de Anatomía Patológica y División Española de la International Academy of Pathology  XXV Congreso de la Sociedad Española de Anatomía Patológica
y División Española de la International Academy of Pathology
XX Congreso de la Sociedad Española de Citología
I Congreso de la Sociedad Española de Patología Forense
   Sociedad Española de Patología Forense
Zaragoza, 18 a 21 de mayo de 2011


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Comunicación oral nº 15. Tema: Patología gastrointestinal y hepatología

Neuroendocrine pancreatic tumors in tuberous sclerosis complex: A case report and review of the literature
D Díaz Díaz, A Ballén Barragán, B Pérez Hurtado, J Salazar Tabares, F Colina Ruizdelgado, G López Alonso, R Gómez Sanz, C Ibarrola de Andrés
Hospital Universitario 12 de Octubre

Introducción:Tuberous sclerosis complex (TSC) is an inherited neurocutaneous disorder that is caused by mutations of two tumor supressor genes, TSC1 on chromosome 9 and TSC2 on chromosome 16 and is characterized by multiple hamartomas of the brain, eyes, heart, lung, liver, kidney and skin. Neuroendocrine tumors of the gastro-enteropancreatic system are infrequent tumors that originate from the diffused endocrine system located in the gastrointestinal tract and in the pancreas. As far as we know there are 10 cases reported of endocrine pancreatic tumors in a setting of TSC in which two cases were in pediatric patients with malignant non-functional pancreatic endocrine tumors. We present a case of malignant non-functional pancreatic endocrine tumor in an adult patient with TSC.

Material y métodos:A rare case of pancreatic neuroendocrine tumor in a patient with TSC is described. Using MEDLINE, for collecting data we made a review of articles related to neuroendocrine tumors and TSC since 1959 to 2010. For light microscopic examination, paraffin sections were stained with hematoxylin and eosin. Immunohistochemical staining was performed with monoclonal and polyclonal antibodies .

Resultados:A 31- year- old man diagnosed of TSC since 2008, had multiple congenital subependymal nodules, bilateral cortical tubers, and seizures of difficult control. A 2,3x2 cm well delimitated solid tumor in the tail of the pancreas was discovered during a monitoring abdominal computed tomography scan. Since the patient did not had a clinical endocrine syndrome, serological peptide hormones were not determined. Blood glucose levels and tumor markers were within normal range. After an ecoendoscopic fine needle aspiration biopsy, a distal pancreatectomy was performed. Histologically, the tumor was formed by uniform cells with moderated cytoplasm arranged in a combined trabecular and nested patterns. The nuclear features were bland and mitosis were infrequent. Immunoreactivity for cytokeratin AE1/AE3, chromogranin A and synaptophysin confirmed the neuroendocrine nature of this neoplasia. Pancreatic polypeptides were negatives. One of five lymph nodes isolated resulted positive for metastases. Insulin, glucagon, pancreatic peptide, somatostatin and vasoactive intestinal peptide were negative. Proliferative index MIB-1 was 1%.

Conclusión:Pancreatic endocrine tumors are infrequent in patients with TSC. Most of these tumors are benign and functional. However, we should increase the awareness of the association of TSC and malignant non-functional pancreatic endocrine tumors.

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Actualizado: 11/06/2011