Immunoglobulin G4-Related Disease, A Diagnosis to Take into Account: About a Case

Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated and multisystemic condition associated with developing fibroinflammatory lesions in any organ. The diagnosis is made based on the sum of clinical, serological, radiological, and histopathological criteria; however, this is often difficult due to its similarity to neoplasms, infections, or other immune-mediated diseases. Treatment is based on corticosteroids, in a possible combination with immunomodulators. The present case concerns a 59-year-old man with a history of jaundice syndrome and weight loss, admitted for suspected malignant neoplasia of the bile duct. Imaging revealed stricture with dilation of the intrahepatic bile ducts, prominent pancreas, pancreatic duct stricture, and nodular renal lesions. Due to the history of left submandibulectomy two years before the current disease and histology compatible with Küttner's tumor, plus the high IgG4 serum values, the diagnosis of IgG4-RD was established. He started treatment with corticosteroids and was asymptomatic during follow-up.

La enfermedad relacionada con inmunoglobulina G4 (ER-IgG4) es una condición inmunomediada y multisistémica asociada al desarrollo de lesiones fibroinflamatorias en cualquier órgano. El diagnóstico se realiza bajo la suma de criterios clínicos, serológicos, radiológicos e histopatológicos; sin embargo, este suele ser difícil debido a su similitud con neoplasias, infecciones u otras enfermedades inmunomediadas. El tratamiento se basa en corticosteroides, en una posible combinación con inmunomoduladores. El presente caso trata de un varón de 59 años con historia de síndrome ictérico y baja de peso, admitido por sospecha de neoplasia maligna de la vía biliar. Las imágenes revelaron estenosis con dilatación de las vías biliares intrahepáticas, páncreas prominente, estenosis del conducto pancreático y lesiones nodulares renales. Debido al antecedente de submandibulectomía izquierda 2 años antes de la enfermedad actual e histología compatible con tumor de Küttner, más los valores elevados de IgG4 sérico, se estableció el diagnóstico de ER-IgG4. Inició tratamiento con corticosteroides y cursó asintomático durante el seguimiento.

Sincronismo colorrectal: adenocarcinoma de colon ascendente y carcinoma neuroendocrino de células pequeñas de recto / Colorectal synchronism: ascending colon adenocarcinoma and small-cell neuroendocrine carcinoma of the rectum

RESUMEN El cáncer colorrectal (CCR) es el tercer cáncer más frecuente en el mundo y la segunda causa de muerte de origen neoplásico. El sincronismo en CCR es de aproximadamente 3-6%. El tracto gastrointestinal es el lugar más frecuente de asiento de neoplasias neuroendocrinas (NNE), y de estos los carcinomas neuroendocrinos (CNE) son poco frecuentes. El tratamiento es un desafío, debido a la agresividad de la neoplasia y la falta de protocolos bien establecidos. Todo esto conlleva a la necesidad de un enfoque multidisciplinario, sin embargo, en la mayoría de casos el pronóstico resulta no ser el mejor. Presentamos el caso de un varón de 83 años que acude a emergencia con historia de 3 meses de diarrea, dolor perianal, pérdida ponderal y hematoquezia. El estudio endoscópico evidenció dos neoplasias y el estudio histológico confirmó la presencia de CNE de células pequeñas de recto y adenocarcinoma de colon ascendente, recibió quimioterapia sistémica sin embargo evolucionó desfavorablemente falleciendo a las 3 semanas.

ABSTRACT Colorectal cancer (CRC) is the third most frequent cancer in the world and the second cause of death of neoplastic origin. Synchronism in CCR is approximately 3-6%. The gastrointestinal tract is the most frequent place where neuroendocrine neoplasms (NNE) settle and a special type of these neuroendocrine carcinomas (CNE) are rare. Treatment is challenging, due to the aggressiveness of the malignancy and the lack of well-established protocols. Therefore a multidisciplinary approach is needed, however, in most cases the result is not the best. We present the case of an 83-year-old man who has an emergency with a 3-month history of diarrhea, perianal pain, weight loss, and hematochezia. The endoscopic study shows evidence of two malignancies and the histological study confirms the presence of CNE in small cells at the rectum and adenocarcinoma in the ascending colon. He underwent systematic chemotherapy, however, he evolved unfavorably, dying after 3 weeks.

[Colorectal synchronism: ascending colon adenocarcinoma and small-cell neuroendocrine carcinoma of the rectum]. / Sincronismo colorrectal: adenocarcinoma de colon ascendente y carcinoma neuroendocrino de células pequeñas de recto.

Colorectal cancer (CRC) is the third most frequent cancer in the world and the second cause of death of neoplastic origin. Synchronism in CCR is approximately 3-6%. The gastrointestinal tract is the most frequent place where neuroendocrine neoplasms (NNE) settle and a special type of these neuroendocrine carcinomas (CNE) are rare. Treatment is challenging, due to the aggressiveness of the malignancy and the lack of well-established protocols. Therefore a multidisciplinary approach is needed, however, in most cases the result is not the best. We present the case of an 83-year-old man who has an emergency with a 3-month history of diarrhea, perianal pain, weight loss, and hematochezia. The endoscopic study shows evidence of two malignancies and the histological study confirms the presence of CNE in small cells at the rectum and adenocarcinoma in the ascending colon. He underwent systematic chemotherapy, however, he evolved unfavorably, dying after 3 weeks.