[Adult hepatoblastoma. A case report]. / Hepatoblastoma del adulto. Presentación de un caso.

Adult hepatoblastoma is a rare pathology. Its pathogeny is not well understood and prognosis is very bad. We pre-sent a case of adult hepatoblastoma treated in our centre. A 65 year-old male, without previous hepatopathy, who consulted due to right hypochondrial pain with a subacute evolution. The pathological diagnosis was adult epithelial hepatoblastoma, with free surgical margins. The patient recei-ved a second surgical intervention 5 months later due to early recurrence and died 10 months after the diagnosis due to a new massive recurrence. His definitive diagnosis is histological. Radical surgery is the only treatment that increases survival, but recurrence is frequent. There are no well-defined patterns of adjuvant chemotherapy nor is there any trans-plant experience.

Hepatoblastoma del adulto. Presentación de un caso / Adult hepatoblastoma. A case report

El hepatoblastoma del adulto es una patología inusual, de patogenia no bien conocida y de muy mal pronóstico. Presentamos un caso de hepatoblastoma del adulto tratado en nuestro centro. Varón de 65 años, sin hepatopatía previa, que consulta por dolor en hipocondrio derecho de evolución subaguda. El diagnóstico anatomopatológico fue hepatoblastoma epitelial del adulto, con bordes quirúrgicos libres. Fue reintervenido 5 meses después por recidiva precoz y falleció 10 meses después del diagnóstico por nueva recidiva masiva. Su diagnóstico definitivo es histológico. La cirugía radical ofrece el único tratamiento que aumente la supervivencia, pero frecuentemente recidiva. No existen pautas bien definidas de quimioterapia adyuvante, ni experiencia en trasplante (AU)

Adult hepatoblastoma is a rare pathology. Its pathogeny is not well understood and prognosis is very bad. We present a case of adult hepatoblastoma treated in our centre. A 65 year-old male, without previous hepatopathy, who consulted due to right hypochondrial pain with a subacute evolution. The pathological diagnosis was adult epithelial hepatoblastoma, with free surgical margins. The patient received a second surgical intervention 5 months later due to early recurrence and died 10 months after the diagnosis due to a new massive recurrence. His definitive diagnosis is histological. Radical surgery is the only treatment that increases survival, but recurrence is frequent. There are no well-defined patterns of adjuvant chemotherapy nor is there any transplant experience (AU)

[Epithelioid angiosarcoma of the gallbladder. Case report and review of the literature]. / Angiosarcoma epitelioide de vesícula.

Angiosarcoma of the gallbladder is an infrequent pathology but has a high morbidity and mortality. There are only 10 references in the international literature. We present a case treated in our center and we review the cases published since 1956. An 81 year-old male patient with abdominal pain, asthenia and dyspnea. Analytically anemia and leukocytosis. Exploration found a distended abdomen, right hypochondrium pain, with defense. Abdominal echography and a CT were requested with a diagnosis of acute cholecystitis and he was admitted for antibiotic treatment. The patient did not evolve favorably and was subjected to emergency surgery, which found a haemoperitoneum and a gallbladder with a tumoral appearance that could not be totally extirpated. He died 20 days after the operation. The report from pathological anatomy was compatible with epithelioid angiosarcoma of the biliary gallbladder. Gallbladder angiosarcoma is a neoplasia with a bad prognosis, whose clinical presentation can be mistaken for acute cholecystitis. Improving the prognosis of this disease involves carrying out early diagnosis and surgical treatment.

Angiosarcoma epitelioide de vesícula. Presentación de un caso y revisión bibliográfica / Epithelioid angiosarcoma of the gallbladder. Case report and review of the literature

El angiosarcoma de vesícula es una patología poco frecuente pero con una alta morbi-mortalidad del que sólo hay 10 referencias en la literatura internacional. Presentamos un caso tratado en nuestro centro y realizamos una revisión de los casos publicados desde 1956. Paciente varón de 81 años con dolor abdominal, astenia y disnea. Analíticamente anemia y leucocitosis. En la exploración destacaba un abdomen distendido, doloroso en hipocondrio derecho, con defensa. Se solicitó ecografía abdominal y un TC con diagnóstico de colecistitis aguda y se ingresó para tratamiento antibiótico. El paciente no evolucionó favorablemente y se intervino de urgencia hallando un hemoperitoneo y una vesícula de aspecto tumoral que no se pudo extirpar en su totalidad. Falleció a los 20 días de la intervención. El informe de anatomía patológica fue compatible con angiosarcoma epiteloide de vesícula biliar. El angiosarcoma de vesícula es una neoplasia de mal pronóstico, cuya presentación clínica puede confundirse con la colecistitis aguda. Conseguir mejorar el pronóstico de esta enfermedad pasa por realizar un diagnóstico y tratamiento quirúrgico precoces (AU)

Angiosarcoma of the gallbladder is an infrequent pathology but has a high morbidity and mortality. There are only 10 references in the international literature. We present a case treated in our center and we review the cases published since 1956. An 81 year-old male patient with abdominal pain, asthenia and dyspnea. Analytically anemia and leukocytosis. Exploration found a distended abdomen, right hypochondrium pain, with defense. Abdominal echography and a CT were requested with a diagnosis of acute cholecystitis and he was admitted for antibiotic treatment. The patient did not evolve favorably and was subjected to emergency surgery, which found a haemoperitoneum and a gallbladder with a tumoral appearance that could not be totally extirpated. He died 20 days after the operation. The report from pathological anatomy was compatible with epithelioid angiosarcoma of the biliary gallbladder. Gallbladder angiosarcoma is a neoplasia with a bad prognosis, whose clinical presentation can be mistaken for acute cholecystitis. Improving the prognosis of this disease involves carrying out early diagnosis and surgical treatment (AU)

Retrospective analysis of pathological response in colorectal cancer liver metastases following treatment with bevacizumab

AIMS: Pathological response has been shown to be a predictor for survival after preoperative chemotherapy and surgical resection of colorectal cancer liver metastases. This retrospective analysis evaluated the effect on pathological response of adding bevacizumab to standard neoadjuvant chemotherapy in patients with metastatic colorectal cancer (mCRC) and liver metastases. METHODS: Patient records from two Spanish centres were retrospectively examined for this analysis. Patients were included if they had stage IV mCRC with liver metastases, were unresectable or marginally resectable tumour before chemotherapy, and had oxaliplatin- or irinotecan-based chemotherapy, with or without bevacizumab, before resection. Tumour response was evaluated using response evaluation criteria in solid tumours (RECIST). Pathological response was assessed by pathologists blinded to treatment. RESULTS: Ninety-five patients were included. Good pathological responses (PR0/PR1) were observed in 37 patients (39 %). The RECIST response rate was 51 %. Only 42 % of patients with a good pathological response had a complete or partial response according to RECIST, while 57 % of those with a poor pathological response had a complete or partial response according to RECIST. RECIST response rates were similar with and without bevacizumab, although 49 % of bevacizumab-treated patients had a good pathological response versus 27 % of those receiving chemotherapy alone (χ (2) P = 0.0302). CONCLUSION: Pathological response may be a better indicator of treatment efficacy than RECIST for patients with mCRC receiving bevacizumab in the neoadjuvant setting. Adding bevacizumab to chemotherapy has the potential to increase pathological response rates. Well-designed prospective clinical studies are required to establish the efficacy and tolerability of this approach (AU)

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Retrospective analysis of pathological response in colorectal cancer liver metastases following treatment with bevacizumab.

AIMS: Pathological response has been shown to be a predictor for survival after preoperative chemotherapy and surgical resection of colorectal cancer liver metastases. This retrospective analysis evaluated the effect on pathological response of adding bevacizumab to standard neoadjuvant chemotherapy in patients with metastatic colorectal cancer (mCRC) and liver metastases. METHODS: Patient records from two Spanish centres were retrospectively examined for this analysis. Patients were included if they had stage IV mCRC with liver metastases, were unresectable or marginally resectable tumour before chemotherapy, and had oxaliplatin- or irinotecan-based chemotherapy, with or without bevacizumab, before resection. Tumour response was evaluated using response evaluation criteria in solid tumours (RECIST). Pathological response was assessed by pathologists blinded to treatment. RESULTS: Ninety-five patients were included. Good pathological responses (PR0/PR1) were observed in 37 patients (39 %). The RECIST response rate was 51 %. Only 42 % of patients with a good pathological response had a complete or partial response according to RECIST, while 57 % of those with a poor pathological response had a complete or partial response according to RECIST. RECIST response rates were similar with and without bevacizumab, although 49 % of bevacizumab-treated patients had a good pathological response versus 27 % of those receiving chemotherapy alone (χ (2) P = 0.0302). CONCLUSION: Pathological response may be a better indicator of treatment efficacy than RECIST for patients with mCRC receiving bevacizumab in the neoadjuvant setting. Adding bevacizumab to chemotherapy has the potential to increase pathological response rates. Well-designed prospective clinical studies are required to establish the efficacy and tolerability of this approach.

Valor pronóstico de la afectación del margen circunferencial en pacientes con cáncer de recto localmente avanzado / Prognostic value of circumferential resection margin involvement in patients with locally-advanced rectal cancer

Introducción. La afectación del margen circunferencial (MC) en los pacientes intervenidos por neoplasia de recto es un factor de mal pronóstico en cuanto a posibilidad de recidiva local, recidiva a distancia y supervivencia, y esta afectación se ha relacionado con la calidad de la cirugía. Analizamos la tasa de afectación del MC en pacientes con cáncer de recto localmente avanzado, sometidos a tratamiento neoadyuvante, y su relación con la supervivencia libre de enfermedad (SLE) y la supervivencia específica (SE). Material y métodos. Se incluye a 101 pacientes diagnosticados de adenocarcinoma de recto entre enero de 2001 y diciembre de 2005, e intervenidos tras recibir radioquimioterapia neoadyuvante. El MC se consideró positivo cuando la distancia del tumor al borde quirúrgico fue menor de un milímetro. La afectación del MC se relaciona con la SLE y la SE mediante el test log-rank. Resultados. La media de edad fue de 66,6 años. La tasa de afectación del MC fue del 10,8% (11 casos), en 7 casos por cercanía o contacto del MC con el tumor, en 2 por cercanía de adenopatías, en un caso por infiltración perineural en el MC y en otro por crecimiento discontinuo del tumor. Con un seguimiento medio de 25,4 meses, se diagnosticó recidiva de la enfermedad en 13 pacientes: en 3 (2,97%) como recidiva local y en 10 (9,9%) como metástasis a distancia. Once (10,8%) pacientes fallecieron por evolución de la enfermedad. La afectación del MC se relacionó significativamente con la SLE (p = 0,0167) y con la SE (p = 0,0176). Conclusión. En pacientes intervenidos por cáncer de recto después de tratamiento neoadyuvante con radioquimioterapia, la afectación del MC es un factor pronóstico negativo para SLE y SE (AU)

Introduction. Circumferential resection margin (CRM) involvement in patients undergoing surgery for rectal tumors is a factor predicting poor prognosis in terms of the possibility of local recurrence, distant recurrence, and survival. CRM involvement has been related to the quality of the surgery. We analyzed the rate of CRM involvement in patients with locally-advanced rectal cancer undergoing neoadjuvant therapy and its relation with disease-free survival (DFS) and disease-specific survival (DSS). Material and methods. A total of 101 patients diagnosed with rectal adenocarcinoma between January 2001 and December 2001 who underwent surgery after receiving neoadjuvant radiochemotherapy were included. The CRM was considered positive when the distance between the tumor and the surgical border was less than 1 mm. The relation between CRM involvement and DFS and DSS was evaluated using the log-rank test. Results. The mean age was 66.6 years. The rate of CRM involvement was 10.8% (11 patients); CRM involvement was due to proximity or contact of the CRM with the tumor in 7 patients, proximity of enlarged nodes in 2 patients, perineural invasion in the CRM in 1 patient and discontinuous tumoral growth in 1 patient. With a mean follow-up of 25.4 months, disease recurrence was diagnosed in 13 patients: local recurrence occurred in 3 (2.97%) patients and distant metastases in 10 (9.9%). Eleven (10.8%) patients died from disease progression. CRM involvement was significantly related to DFS (p = 0.0167) and DSS (p = 0.0176). Conclusion. In patients undergoing surgery for rectal cancer after neoadjuvant radiochemotherapy, CRM involvement is a negative prognostic factor for DFS and DSS (AU)

[Gastric necrosis caused by acute gastric distention and superior mesenteric artery syndrome]. / Necrosis gástrica por distensión aguda del estómago y síndrome de la pinza mesentérica.

We report the case of a 14 year-old female patient who was admitted complaining of abdominal pain due to gastric distension after an episode of bulimia. The patient was diagnosed as having gastric intramural necrosis by CT. An emergency laparotomy was performed and a massive gastric necrosis and duodenal compression due to the superior mesenteric artery syndrome was found. The patient did well after total gastrectomy and Roux-en-Y procedure.

[Hemoperitoneum secondary to acute cholecystitis in kidney transplant]. / Hemoperitoneo secundario a colecistitis aguda en el trasplante renal.

We present two cases of acute cholecystitis occurring more than two years after renal transplantation. The course of the acute event was complicated by the presence of hemobilia in one of the patients and severe hemoperitoneum in both patients. We comment the possible etiologic factors, the high efficiency of the diagnostic procedures and the importance of prompt cholecystectomy as the best treatment method.

[Adenocarcinoma in a choledochal cyst. Report of a case and review of the literature]. / Adenocarcinoma sobre quiste de colédoco. Aportación de un caso y revisión de la literatura.

We present a case of cancer of a choledochal cyst in a patient with antecedents of cholecystectomy, who complained of pain in the right hypochondrium. Echography and CAT disclosed a cystic mass of biliary location, and the diagnosis was confirmed by intraoperative cholangiography and biopsy of the cyst margin. Cysto-jejunostomy on a Roux-en-Y loop was performed. The patient survived 11 months and died of tumoral dissemination. We reviewed 130 cases of cancer of a choledochal cyst published up until 1986 and possible etiopathogenic causes, and we discuss the diagnostic problems and related treatment.

[Duodenal duplication in adults]. / Duplicación duodenal en el adulto.

A case is presented of intestinal duplication in the duodenum of a 17-year-old patient initially diagnosed as anorexia nervosa due to her extreme thinness and scant accompanying symptomatology. The etiopathogenesis, anatomic, clinical and diagnostic features, and different treatments of duodenal duplication are briefly reviewed.

[Liver transplant in fibrolamellar hepatocarcinoma: apropos of a case. Review of the literature]. / Trasplante hepático en hepatocarcinoma fibrolamelar: a propósito de un caso. Revisión de la literatura.

A case is presented of fibrolamellar hepatocarcinoma in a 12 year-old male treated by liver transplantation; there is no evidence of tumoral recurrence at 28 months. Donos and receptor were ABO incompatible. The immunosuppressive regimen used was cyclosporine A and low doses of steroids. Fibrolamellar hepatocarcinoma is an infrequent histologic variety that usually affects young people and is generally not associated with hepatitis B infection or cirrhosis. It is often a single tumor, is more susceptible to surgical resection than other varieties of hepatocarcinoma, and is characterized by a relatively unagressive tumoral biology.

[Digestive hemorrhage associated with ectopic pancreas]. / Hemorragia digestiva asociada a páncreas ectópico.

Pancreatic heterotopia is pancreatic tissue with no direct or vascular connection to the pancreas that results form a ontogenic anomaly. It is usually an incidental findings, although it has been sometimes associated to nonspecific symptoms or symptoms due to its location. Massive gastrointestinal hemorrhage due to pancreatic ectopia has been described occasionally and is considered an infrequent form of presentation. Two cases are presented of massive digestive hemorrhage associated with pancreatic duodenal ectopia that were treated surgically by excision of the ectopic tissue. The patients remain asymptomatic after surgery. A review is made of the literature on this topic.