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Case summary: This report describes the case of a 7-year-old male neutered domestic mixed-breed cat that was initially referred to the Veterinary Hospital of the State University of Londrina for evaluation of a 2-week history of abdominal distension and a 2-day history of anorexia, infected with feline immunodeficiency virus (FIV). Abdominal ultrasound revealed an expansive mass located around the pancreas and right hepatic lobe. In the transoperative macroscopic observation, multiple white nodules were visualized in the liver, pancreas, mesentery, intestine, stomach and peritoneal wall. Immunohistochemical examination revealed that neoplastic cells demonstrated a strong positivity for AE1/AE3 and CK20. A sparse immunoreactivity to chromogranin A was observed, which demonstrates neuroendocrine cell labeling. The histopathologic changes associated with the immunohistochemical profile confirmed the diagnosis of metastatic carcinoma with neuroendocrine differentiation, originating from the pancreas. Relevance and novel information: Neuroendocrine tumors of the pancreas are rare and are associated with a poor prognosis in humans. In humans, approximately 7% of neuroendocrine tumors develop in the pancreas, and the 5-year survival rate for a pancreatic neuroendocrine tumor is 53%, according to the American Cancer Society. To our knowledge, only one case has been described in the feline species so far. Due to the rarity of this type of tumor in cats, there is little information about predisposition related to age, sex or breed, as well as the main clinical signs presented, survival time and treatment options.

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RESUMEN 19- Las dermatosis paraneoplásicas son un grupo heterogéneo de manifestaciones cutáneas que tienen fuerte asociación con patología maligna interna. Su patogenia es poco clara y no se conoce su prevalencia exacta. El tratamiento consiste en el abordaje terapéutico de la enfermedad subyacente. La importancia del conocimiento de las mismas radica en la posibilidad de realizar un diagnóstico temprano de una neoplasia. 20- Se presenta el caso clínico de un paciente con dermatosis paraneoplásica asociada a tumor neuroendócrino de páncreas.

ABSTRACT 24- Paraneoplastic dermatoses are an heterogeneous group of cutaneous manifestations that have a strong association with internal malignancy. Their pathogenesis is unclear and prevalence is unknown. Treatment consists of a therapeutic approach to the underlying disease. The importance of recognizingthem lies in the possibility of making an early diagnosis oftheneoplasm. 25- We report theclinical case of a patient with paraneoplastic dermatosis associated with a pancreatic neuroendocrine tumor.

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BACKGROUND: Tuberous Sclerosis Complex (TSC) is a complex and heterogeneous genetic disease that has well-established clinical diagnostic criteria. These criteria do not include gastrointestinal tumors. CASE PRESENTATION: We report a 45-year-old patient with a clinical and molecular diagnosis of TSC and a family history of cancer, presenting two rare associated findings: gastrointestinal polyposis and pancreatic neuroendocrine tumor. This patient was subjected to a genetic test with 80 cancer predisposing genes. The genetic panel revealed the presence of a large pathogenic deletion in the TSC2 gene, covering exons 2 to 16 and including the initiation codon. No changes were identified in the colorectal cancer and colorectal polyposis genes. DISCUSSION AND CONCLUSIONS: We describe a case of TSC that presented tumors of the gastro intestinal tract that are commonly unrelated to the disease. The patient described here emphasizes the importance of considering polyposis of the gastrointestinal tract and low grade neuroendocrine tumor as part of the TSC syndromic phenotype.

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Secretory tumors of vasoactive intestinal polypeptides (VIPomas) are rare neuroendocrine pancreatic neoplasms usually associated with secretory diarrhea. Most cases present themselves with diarrhea, weight loss, and hypokalemia. Although VIPoma patients share easily distinguishable symptoms, early diagnosis remains a challenge. Therefore, tumors are usually found in large proportions and with later staging. The presenting case portrays a unique situation where the tumor found was still in a small size, is worth reporting it to the current literature. The diagnosis was made through laboratory evaluation and imaging studies. The primary treatment consists of exeresis surgery, but cytoreductive surgery also can provide several benefits. This way is necessary to pay attention to diagnoses also in the ways of treatment.

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Introducción: los insulinomas son tumores neuroendocrinos pancreáticos secretores de insulina, la incidencia en la población en general estimada es de 1 a 4 casos en un millón de habitantes por año. Los insulinomas constituyen el 60% de los tumores de páncreas, de los cuales el 90% son benignos y únicos; el 10% son múltiples, la mayoría asociados a MEN 1 (Neoplasia Endocrina Múltiple) y del 5 al 10% son malignas. La posibilidad de recidiva obliga a un diagnóstico y seguimiento a largo plazo. La característica clínica del insulinoma es la hipoglucemia en ayunas, con signos y síntomas de neuroglucopenia, que pueden estar precedidos o no por manifestaciones adrenérgicas. El tratamiento de elección es el quirúrgico; en etapas irresecables o avanzadas se proponen tratamientos paliativos o sintomáticos con quimioterapia o procedimientos mediante radiología intervencionista con pobres respuestas. Se ha demostrado que con nuevos tratamientos con inhibidores de la tirosina kinasa e inhibidores de la vía de la rapamicina en tumores neuroendocrinos pancráticos bien diferenciados las respuestas tanto en sobrevida libre de progresión así como sobrevida global mejoran importantemente. Caso: presentamos el caso de un hombre de 44 años, con diagnóstico de insulinoma y MEN 1, debutó con convulsiones tónico clónicas generalizadas tratado con pancreatectomia parcial, siete años más tarde, tras haber permanecido asintomático nuevo episodio de convulsiones, documentándose hipoglicemias severas y múltiples metástasis hepáticas, sometido a una pancreatoduodenectomia y como tratamiento sintomático recibió corticoides en altas dosis, diazóxido, octreotide mensual y dos líneas de quimioterapia, además de embolización de las metástasis hepáticas de mayor tamaño y por progresión de enfermedad se inició inhibidores de tirosin-kinasa por un período de tres meses y el paciente fallece.

Introduction: the Insulinomas are pancreatic neuroendocrine tumors secreting insulin. The incidence in the general population is estimated 1-4 per 1 000 000 yearly Insulinoma accounts for 60% of pancreatic tumors, of which 90% are benign and unique, 10% are multiple, mostly associated with MEN 1 (Multiple Endocrine Neoplasia), and 5-10% is malignant. The possibility of recurrence requires diagnosis and long-term monitoring. The clinical characteristic of insulinoma is fasting hypoglycemia, with signs and symptoms of neuroglucopenia, which may be preceded or not by adrenergic manifestations. Surgery is the treatment of choice, in irrsecable; and in advanced stages the proposed palliatives or symptomatic treatments are with chemotherapy or procedures through interventional radiologist with poor responses. It has been shown that new treatments with inhibitors of tyrosine kinase inhibitors and rapamycin pathway for pancreatic neuroendocrine well differentiated tumors, the response in both progression-free survival and overall survival signifcantly improved. Case study: we report the case of a 44 year old male of, diagnosed with insulinoma and MEN 1, debuted with tonic clonic seizures treated with partial pancreatectomy, 7 years later after a new episode of seizures documented severe hypoglycemia and multiple liver metastases, underwent a pancreatoduodenectomy and as symptomatic treatment received high-dose corticosteroids, diaxozido, octreotide monthly and 2 lines of chemotherapy, in addition to embolization of the larger liver metastases masses, and due to disease progression tyrosine kinase inhibitors was initiated for a period of 3 months and the patient dies.

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We report a 28 years old woman consulting for weight gain, progressive muscle weakness and appearance of facial plethora. Laboratory showed high cortisol and ACTH levels. Magnetic resonance of sella turcica was normal and an abdominal magnetic resonance showed a pancreatic mass, suggestive of a neuroendocrine tumor. A PET/CT with somatostatin analogues demonstrated an intense over-expression of somatostatin receptors in the tumor. The patient was subjected to a pancreatectomy and the day after surgery, ACTH levels decreased to less than 5 pg/ml. The patological study of the surgical piece showed a neuroendocrine carcinoma. The patient had a good postoperative evolution.

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Os tumores neuroendócrinos do pâncreas não-funcionantes são raros. Os sintomas são crônicos, o diagnóstico pré-operatório é difícil e o tratamento cirúrgico exige a presença de um cirurgião de larga experiência clínico-cirúrgica. O objetivo é apresentar um paciente com tumor gigante de corpo e cauda de pâncreas, com acometimento de órgãos adjacentes e com metástases hepáticas. O paciente submeteu-se à ressecção de múltiplos órgãos e colostomia terminal, com boa evolução pós-operatória. Recebeu tratamento quimioterápico e mesmo com a remanescência da lesão após 14 meses, sobreviveu por 21 meses. A apresentação justifica-se pela baixa incidência de tumores dessa natureza, pela contribuição ao conhecimento da evolução clínica desses pacientes e para aumentar a casuística da doença, impossível de ser estabelecida de outra forma senão com relatos de casos.

The nonfunctioning neuroendocrine tumors of the pancreas are rare. The symptoms are chronic, the preoperative diagnosis is difficult and the surgical treatment demands an experienced surgeon with clinical surgical practice. The objective is to present a patient with a huge tumor in the body and tail of the pancreas with extension to adjacent organs and liver metastases. The patient was submitted to multiple organs resection and terminal colostomy, and made good postoperative progress. The patient received chemotherapy treatment and, even with the lesion remaining for the past 14 months, survived for 21 months. The presentation is justified by the low-incidence of this kind of tumor, the contribution to knowledge of the clinical evolution of these patients and the contribution to increasing the casuistry of the disease, which is only possible to be established by reporting of cases.

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