Pseudomyxoma peritonei secondary to epithelial appendicular neoplasms. Experience in a non-specialised centre.

INTRODUCTION: Pseudomyxoma peritonei is an infrequent entity, defined by collections of gelatinous material in the abdomen and pelvis and mucinous implants on peritoneum, secondary to the rupture of a mucinous lesion, usually of ovarian or appendiceal origin. MATERIALS AND METHODS: We present our experience of 11 cases (6 males and 5 females) diagnosed with pseudomyxoma peritonei secondary to epithelial appendicular neoplasms over 27 years. The mean age of the patients was 68 years. Clinical manifestations were abdominal distension (55%), right lower quadrant pain (45%) suggesting acute appendicitis and constitutional syndrome (36%). An abdominal mass was detected at physical examination in 4 patients. CT scan revealed a tumour in right iliac fossa in 4 patients, peritoneal enlargement in 1 and a liquid collection in 1. Preoperative diagnosis was acute abdomen in 5 patients, peritoneal carcinomatosis in 3 and undetermined abdominal mass in 3. RESULTS: Surgical findings suggested pseudomyxoma peritonei in 8 patients and peritoneal carcinomatosis in 3. Appendicectomy was performed in 9 patients, and in 3 of them bilateral anexectomy was also performed. One patient underwent ileocaecal resection and another a right hemicolectomy. In all the cases, mucinous material was eliminated as much as possible. Pathology revealed mucinous cystoadenoma in 6 cases, mucinous cystoadenocarcinoma in 3 and epithelial hyperplasia in 2 patients. Median survival was 54 months, with a 5- year survival rate of 40%. The last case we treated was sent to a reference centre for the treatment of pseudomyxoma peritonei. CONCLUSIONS: There is no consensus on the best treatment for pseudomyxoma peritonei. We recommend avoiding incomplete surgical resections in non-reference centres and submitting patients to a reference centre to undergo adequate treatment.

Pseudomyxoma peritonei secondary to epithelial appendicular neoplasms. Experience in a non-specialised centre

INTRODUCTION: Pseudomyxoma peritonei is an infrequent entity, defined by collections of gelatinous material in the abdomen and pelvis and mucinous implants on peritoneum, secondary to the rupture of a mucinous lesion, usually of ovarian or appendiceal origin. MATERIALS AND METHODS: We present our experience of 11 cases (6 males and 5 females) diagnosed with pseudomyxoma peritonei secondary to epithelial appendicular neoplasms over 27 years. The mean age of the patients was 68 years. Clinical manifestations were abdominal distension (55%), right lower quadrant pain (45%) suggesting acute appendicitis and constitutional syndrome (36%). An abdominal mass was detected at physical examination in 4 patients. CT scan revealed a tumour in right iliac fossa in 4 patients, peritoneal enlargement in 1 and a liquid collection in 1. Preoperative diagnosis was acute abdomen in 5 patients, peritoneal carcinomatosis in 3 and undetermined abdominal mass in 3. RESULTS: Surgical findings suggested pseudomyxoma peritonei in 8 patients and peritoneal carcinomatosis in 3. Appendicectomy was performed in 9 patients, and in 3 of them bilateral anexectomy was also performed. One patient underwent ileocaecal resection and another a right hemicolectomy. In all the cases, mucinous material was eliminated as much as possible. Pathology revealed mucinous cystoadenoma in 6 cases, mucinous cystoadenocarcinoma in 3 and epithelial hyperplasia in 2 patients. Median survival was 54 months, with a 5- year survival rate of 40%. The last case we treated was sent to a reference centre for the treatment of pseudomyxoma peritonei. CONCLUSIONS: There is no consensus on the best treatment for pseudomyxoma peritonei. We recommend avoiding incomplete surgical resections in non-reference centres and submitting patients to a reference centre to undergo adequate treatment (AU)

[Renal cell carcinoma with liver extension: a report of a new case and literature review]. / Tumor renal con invasión hepática: aportación de un nuevo caso y revisión de la literatura.

Locally advanced renal cell carcinoma (RCC) with involvement to adjacent organs is uncommon and the prognosis is poor. Radical surgery remains the only effective treatment. We report the case of a woman with RCC and direct liver extension who was surgically treated. A literature review is made.

Extraosseous retroperitoneal Ewing's sarcoma.

Ewing's sarcoma/primitive neuroectodermal tumour (ES/PNET) belongs to the group of paediatric small round blue-cell tumours. ES/PNET is classically a tumour of the soft tissue or bone in children and young adults. The case of a 21-year-old woman with a retroperitoneal localisation of Ewing's sarcoma is described.

[Diagnosis and treatment of pancreatic insulinoma]. / Manejo diagnóstico y terapéutico del insulinoma pancreático.

INTRODUCTION: insulinoma is the most frequent pancreatic endocrine tumor. Its preoperative diagnosis has been controversial for many years. The aim of this study was to evaluate the experience in the management and treatment of this kind of tumor at Hospital Ramón y Cajal. MATERIAL AND METHODS: between January 1999 and July 2006, 12 patients were operated on in our hospital (9 females and 3 males) who had been diagnosed with insulinoma, with a mean age of 56 years (16-72 years). RESULTS: octreotide scintigraphy allowed a diagnosis in 33.3% of cases, abdominal CT in 83.3%, and echoendoscopy in 100%. Intraoperative ultrasonography confirmed the presence of an insulinoma in 100% of cases. In all cases a complete excision was possible, with 9 enucleations and 3 distal pancreatectomies. Two patients developed a pancreatic fistula, and one a pancreatic pseudocyst that healed spontaneously without surgery. The overall cure rate was 100%. After a mean follow-up of 48 months no recurrences have been reported. CONCLUSION: in our experience, we consider advisable that abdominal CT and echoendoscopy be performed before surgery. However, the initial procedure of choice would be pancreas palpation and intraoperative ultrasonography. Surgery cured 100% of cases, and the procedure selected depends on size, location, distance from the main pancreatic duct, and relation to multiple endocrine neoplasm 1 (MEN-1).

Extraosseous retroperitoneal Ewing's sarcoma

Ewing's sarcoma/primitive neuroectodermal tumour (ES/PNET) belongs to the group of paediatric small round blue-cell tumours. ES/PNET is classically a tumour of the soft tissue or bone in children and young adults. The case of a 21-year-old woman with a retroperitoneal localisation of Ewing's sarcoma is described (AU)

Tumor renal con invasión hepática: aportación de un nuevo caso y revisión de la literatura / Renal cell carcinoma with liver extension: a report of a new case and literature review

El carcinoma de células renales (CCR) con invasión directa de órganos adyacentes es un hallazgo infrecuente y de mal pronóstico en el que el único tratamiento potencialmente efectivo es la cirugía radical. Se presenta el caso de una mujer con gran masa renal y afectación hepática y diafragmática sometida a tratamiento quirúrgico, y se realiza revisión de la literatura

Locally advanced renal cell carcinoma (RCC) with involvement to adjacent organs is uncommon and the prognosis is poor. Radical surgery remains the only effective treatment. We report the case of a woman with RCC and direct liver extension who was surgically treated. A literature review is made

Manejo diagnóstico y terapéutico del insulinoma pancreático / Diagnosis and treatment of pancreatic insulinoma

Introducción: el insulinoma es el tumor endocrino pancreático más frecuente. Su diagnóstico preoperatorio ha sido motivo de controversia durante muchos años. El objetivo del trabajo es evaluar la experiencia del Hospital Ramón y Cajal en el manejo diagnóstico y terapéutico de este tipo de tumores. Material y métodos: en el periodo comprendido entre enero de 1999 y julio de 2006 fueron intervenidos en nuestro hospital 12 pacientes (9 mujeres y 3 varones) con el diagnóstico de insulinoma, con una edad media de 56 años (16-72 años). Resultados: la gammagrafía con octreótido nos permitió realizar el diagnóstico en el 33,3% de los casos, el TAC abdominal en el 83,3% y la ecoendoscopia en el 100%. El 100% de los insulinomas fue diagnosticado durante la cirugía gracias al uso de la ecografía intraoperatoria. En todos los casos se realizó la excisión completa del tumor, mediante 9 enucleaciones y 3 pancreatectomías distales. Dos pacientes presentaron una fístula pancreática y otro un pseudoquiste pancreático que se resolvieron de forma conservadora. La tasa de curación fue del 100%. El seguimiento fue de 48 meses, no encontrándose ningún caso de recidiva. Conclusión: en nuestra experiencia, creemos aconsejable la realización de TAC abdominal y ecoendoscopia como pruebas previas a la cirugía. Sin embargo, la técnica de elección sería la palpación del páncreas y la realización de una ecografía intraoperatoria. La cirugía es curativa en el 100% de los casos, y la técnica efectuada dependerá del tamaño, localización y distancia del tumor al conducto pancreático, así como su relación con el síndrome MEN-1

Introduction: insulinoma is the most frequent pancreatic endocrine tumor. Its preoperative diagnosis has been controversial for many years. The aim of this study was to evaluate the experience in the management and treatment of this kind of tumor at Hospital Ramón y Cajal. Material and methods: between January 1999 and July 2006, 12 patients were operated on in our hospital (9 females and 3 males) who had been diagnosed with insulinoma, with a mean age of 56 years (16-72 years). Results: octreotide scintigraphy allowed a diagnosis in 33.3% of cases, abdominal CT in 83.3%, and echoendoscopy in 100%. Intraoperative ultrasonography confirmed the presence of an insulinoma in 100% of cases. In all cases a complete excision was possible, with 9 enucleations and 3 distal pancreatectomies. Two patients developed a pancreatic fistula, and one a pancreatic pseudocyst that healed spontaneously without surgery. The overall cure rate was 100%. After a mean follow-up of 48 months no recurrences have been reported. Conclusion: in our experience, we consider advisable that abdominal CT and echoendoscopy be performed before surgery. However, the initial procedure of choice would be pancreas palpation and intraoperative ultrasonography. Surgery cured 100% of cases, and the procedure selected depends on size, location, distance from the main pancreatic duct, and relation to multiple endocrine neoplasm 1 (MEN-1)