Primary retroperitoneal tumor: mucinous cystoadenocarcinoma.

Primary retroperitoneal mucinous cystadenocarcinomas (PRMCs) are extremely rare tumors with limited understanding of their pathogenesis and biological behavior. We describe a case of a 50-year-old female patient who underwent surgical treatment. The patient had a history of previous surgeries for mesenteric mucinous cystadenoma, without evidence of recurrence. During routine abdominal ultrasound a new tumor was found. An abdomen magnetic resonance imaging was done and confirmed the presence of a cystic lesion in the right iliac fossa. After discussion in multidisciplinary committee, surgical complete resection of the tumor, along with bilateral adnexectomy, was performed successfully. Histopathological examination revealed a mucinous adenocarcinoma adjacent to a mucinous cystadenoma. Immunohistochemical analysis supported the diagnosis of a primary retroperitoneal lesion. The patient had an uneventful recovery and has remained disease-free during the two-year postoperative follow-up. PRMCs are challenging to diagnose preoperatively due to nonspecific symptoms. Surgical excision is the mainstay of treatment. The long-term prognosis and optimal therapeutic strategies require further investigation.

Los cistoadenocarcinomas mucinosos primarios retroperitoneales (CMPR) son tumores extremadamente raros con una comprensión limitada de su patogénesis y comportamiento biológico. Describimos el caso de una mujer de 50 años sometida a tratamiento quirúrgico. La paciente tenía antecedentes de cirugías previas por cistodenoma mucinoso mesentérico, sin evidencia de recurrencia. Durante una ecografía abdominal de rutina se encontró un nuevo tumor. Se realizó una resonancia magnética abdomen que confirmó la presencia de una lesión quística en la fosa ilíaca derecha. Luego de discutir el caso en el comité multidisciplinario, se realizó con éxito la resección quirúrgica completa del tumor, junto con la anexectomía bilateral. El examen histopatológico reveló un adenocarcinoma mucinoso adyacente a un cistodenoma mucinoso. El análisis inmunohistoquímico apoyó el diagnóstico de lesión primaria retroperitoneal. La paciente tuvo una buena recuperación y permaneció libre de enfermedad durante dos años de seguimiento postoperatorio. Los CMPR son difíciles de diagnosticar debido a que presentan síntomas inespecíficos. La escisión quirúrgica es la base del tratamiento. El pronóstico a largo plazo y las estrategias terapéuticas óptimas requieren más investigación.

[Fluorodeoxiglucose F18 positron emission tomography imaging (F18FDG) for the assessment of rising levels of serum CA 19-9 in pancreatic mucinous cystadenocarcinoma. Report of one case]. / Estudio de imágenes PET con Flúor18-Desoxiglucosa (F18FDG) en evaluación de elevación de marcador tumoral CA 19-9 en paciente con cistoadenocarcinoma mucinoso de páncreas.

We report a 38 year old female patient with a pancreatic mucinous cystadenocarcinoma. She presented at the onset with a peritoneal rupture that required emergency surgery. Five months later, the patient was subjected to a segmental pancreatectomy and splenectomy. One year later, the patient had a serious gastric bleeding secondary to a gastric ulcer. Due to a persistent increase in her CA 19-9 levels, a Positron Emission Tomography (PET) functional imaging with fluorine 18-deoxyglucose (F18FDG) was done. It showed an intense focal hypermetabolism in the gastric wall reported as a secondary tumour location. The patient was subjected to a total gastrectomy and Roux en Y anastomosis, with a good outcome. The pathological study confirmed the presence of a metastasis of an adenocarcinoma in the gastric wall. The relative value of CA 19-9 markers and FDG PET in pancreatic and gastric carcinomas is discussed.

[Is there pancreatic regeneration? Morphological and functional certification after a corporocaudal splenopancreatectomy/]. / Existe la regeneración pancreática? Certificación morfológica y funcional luego de una esplenopancreatectomía corporocaudal.

INTRODUCTION: The process of pancreatic regeneration, well known and accepted, is less known than the hepatic and includes different mechanisms and factors. Pancreatic regeneration is better known in acute pancreatitis. After an extensive pancreatic necrosis, the morphological and functional regeneration is assessed by dynamic computed tomography associated with normalization of glycemia and the exocrine function. Different groups identified and evaluated experimentally and clinically the actions of multiple factors involved in the process of pancreatic regeneration. Even difficult to assess, pancreatic regeneration after partial pancreatectomy is well documented and of capital importance. CASE REPORT: A 57-year-old woman with discomfort in the upper-left abdominal quadrant. CT scans showed a tumor in the body and tail of the pancreas adherent to the spleen. Preoperative CA 19-9 was normal. She was operated on and the tumor resected en bloc with the spleen. Only the head of the pancreas was preserved. Intraoperative pathological examination of the specimen showed a mucinous cistoadenoma with no malignant degeneration. Postoperative course was uneventful and discharged at p.o. day 10, with ongoing diabetes. Four month later she presented pain in the upper-left quadrant with hyperamylasemia. CT scans showed a normal body and tail with an image of pseudocyst at the top of the pancreatic tail. One year after the initial surgery she remained asymptomatic, without diabetes and with no dietary restrictions. Further CT controls showed images of the entire regeneration of the body and tail of the pancreas. DISCUSSION: Several phenomena are well known and accepted to be associated with the regeneration of the pancreas. In 1965, Tiscornia et al demonstrated the restoration of the pancreatic exocrine function after 6 weeks of selective occlusion of the pancreatic duct. The authors proposed pancreatic regeneration as responsible of morphological, histological and functional changes observed in operated patients where the pancreatic duct was decompressed by an anastomosis to the small bowel and constitutes the rational basis for surgical treatment of chronic pancreatitis. Several humoral factors seems to be involved in pancreatic regeneration acting by a specific receptors-mechanisms, like Bombesin, Octeotride, FGF (Fibroblast Growth Factor) and TGF (Transforming Growth Factor). Friess et al demonstrated increased levels of TGF in acute pancreatitis and considered it to be responsible of the pancreatic regeneration. Waguri et al experimentally demonstrated a double mechanism involved in the regeneration of B-cells: cellular and humoral ways could vary according to different situations. Less evident are the mechanisms involved after surgical pancreatic resection. Kato et al demonstrated the importance of zinc after partial pancreatic resection in dogs. Up to now, there are no other associations experimentally nor in humans. Our case showed in several CT scans control the presence of a normal pancreatic body and tails after a splenopancreatectomy, with restoration of endocrine and exocrine functions. Probably, several mechanisms were involved in this case. Further investigations will elucidate the answered. CONCLUSIONS: Pancreatic regeneration was confirmed by CT scans images and also functionally after an extensive resection. Future similar findings could be of great clinical importance.

Existe la regeneración pancreática? Certificación morfológica y funcional luego de una esplenopancreatectomía corporocaudal / There is pancreatic regeneration? Morphological and functional certification after a corporocaudal splenopancreatectomy

INTRODUCTION: The process of pancreatic regeneration, well known and accepted, is less known than the hepatic and includes different mechanisms and factors. Pancreatic regeneration is better known in acute pancreatitis. After an extensive pancreatic necrosis, the morphological and functional regeneration is assessed by dynamic computed tomography associated with normalization of glycemia and the exocrine function. Different groups identified and evaluated experimentally and clinically the actions of multiple factors involved in the process of pancreatic regeneration. Even difficult to assess, pancreatic regeneration after partial pancreatectomy is well documented and of capital importance. CASE REPORT: A 57-year-old woman with discomfort in the upper-left abdominal quadrant. CT scans showed a tumor in the body and tail of the pancreas adherent to the spleen. Preoperative CA 19-9 was normal. She was operated on and the tumor resected en bloc with the spleen. Only the head of the pancreas was preserved. Intraoperative pathological examination of the specimen showed a mucinous cistoadenoma with no malignant degeneration. Postoperative course was uneventful and discharged at p.o. day 10, with ongoing diabetes. Four month later she presented pain in the upper-left quadrant with hyperamylasemia. CT scans showed a normal body and tail with an image of pseudocyst at the top of the pancreatic tail. One year after the initial surgery she remained asymptomatic, without diabetes and with no dietary restrictions. Further CT controls showed images of the entire regeneration of the body and tail of the pancreas...(AU)