Acute Small Bowel Obstruction and Small Bowel Perforation as a Clinical Debut of Intestinal Endometriosis: A Report of Four Cases and Review of the Literature.

Endometriosis is a quite common pathology, however, intestinal endometriosis is a rare condition, which typically occurs with chronic symptoms. Its acute presentation is very infrequent. We herein report four cases of intestinal endometriosis, in which the clinical debut occurred acutely: two as an acute small bowel obstruction and two as a small bowel perforation. None of the cases had a preoperative diagnosis of endometriosis. The interest of these cases lies in this exceptional form of presentation, such as a surgical acute abdomen. Therefore, intestinal endometriosis should be taken into account in the differential diagnosis of an acute obstructive or perforative process of the small or large bowel.

Endoscopic submucosal dissection (ESD) of antral subepithelial lesion suspected of malignancy.

Subepithelial gastric tumours comprise a heterogeneous group of lesions. Endoscopic ultrasonography with fine-needle aspiration (EUS-FNA) is a useful approach but cannot always offer a definitive diagnosis to guide future therapeutic decisions. In the case we describe, biopsy samples of an antral subepithelial lesion and cytological analysis obtained with an EUS-FNA suggested the diagnosis of an adenocarcinoma. Endoscopic submucosal dissection (ESD) allowed en bloc resection of the tumour ensuring diagnosis and providing a definitive treatment.

Endoscopic submucosal dissection (ESD) of antral subepithelial lesion suspected of malignancy

Subepithelial gastric tumours comprise a heterogeneous group of lesions. Endoscopic ultrasonography with fine-needle aspiration (EUS-FNA) is a useful approach but cannot always offer a definitive diagnosis to guide future therapeutic decisions. In the case we describe, biopsy samples of an antral subepithelial lesion and cytological analysis obtained with an EUS-FNA suggested the diagnosis of an adenocarcinoma. Endoscopic submucosal dissection (ESD) allowed en bloc resection of the tumour ensuring diagnosis and providing a definitive treatment (AU)

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Pathological features and diagnosis of intraductal papillary mucinous neoplasm of the pancreas.

Intraductal papillary mucinous neoplasm (IPMN) of the pancreas is a noninvasive epithelial neoplasm of mucin-producing cells arising in the main duct (MD) and/or branch ducts (BD) of the pancreas. Involved ducts are dilated and filled with neoplastic papillae and mucus in variable intensity. IPMN lacks ovarian-type stroma, unlike mucinous cystic neoplasm, and is defined as a grossly visible entity (≥ 5 mm), unlike pancreatic intraepithelial neoplasm. With the use of high-resolution imaging techniques, very small IPMNs are increasingly being identified. Most IPMNs are solitary and located in the pancreatic head, although 20%-40% are multifocal. Macroscopic classification in MD type, BD type and mixed or combined type reflects biological differences with important prognostic and preoperative clinical management implications. Based on cytoarchitectural atypia, IPMN is classified into low-grade, intermediate-grade and high-grade dysplasia. Based on histological features and mucin (MUC) immunophenotype, IPMNs are classified into gastric, intestinal, pancreatobiliary and oncocytic types. These different phenotypes can be observed together, with the IPMN classified according to the predominant type. Two pathways have been suggested: gastric phenotype corresponds to less aggressive uncommitted cells (MUC1 -, MUC2 -, MUC5AC +, MUC6 +) with the capacity to evolve to intestinal phenotype (intestinal pathway) (MUC1 -, MUC2 +, MUC5AC +, MUC6 - or weak +) or pancreatobiliary /oncocytic phenotypes (pyloropancreatic pathway) (MUC1 +, MUC 2-, MUC5AC +, MUC 6 +) becoming more aggressive. Prognosis of IPMN is excellent but critically worsens when invasive carcinoma arises (about 40% of IPMNs), except in some cases of minimal invasion. The clinical challenge is to establish which IPMNs should be removed because of their higher risk of developing invasive cancer. Once resected, they must be extensively sampled or, much better, submitted in its entirety for microscopic study to completely rule out associated invasive carcinoma.

Pathological aspects of so called "hilar cholangiocarcinoma".

Cholangiocarcinoma (CC) arising from the large intrahepatic bile ducts and extrahepatic hilar bile ducts share clinicopathological features and have been called hilar and perihilar CC as a group. However, "hilar and perihilar CC" are also used to refer exclusively to the intrahepatic hilar type CC or, more commonly, the extrahepatic hilar CC. Grossly, a major distinction can be made between papillary and non-papillary tumors. Histologically, most hilar CCs are well to moderately differentiated conventional type (biliary) carcinomas. Immunohistochemically, CK7, CK20, CEA and MUC1 are normally expressed, being MUC2 positive in less than 50% of cases. Two main premalignant lesions are known: biliary intraepithelial neoplasia (BilIN) and intraductal papillary neoplasm of the biliary tract (IPNB). IPNB includes the lesions previously named biliary papillomatosis and papillary carcinoma. A series of 29 resected hilar CC from our archives is reviewed. Most (82.8%) were conventional type adenocarcinomas, mostly well to moderately differentiated, although with a broad morphological spectrum; three cases exhibited a poorly differentiated cell component resembling signet ring cells. IPNB was observed in 5 (17.2%), four of them with an associated invasive carcinoma. A clear cell type carcinoma, an adenosquamous carcinoma and two gastric foveolar type carcinomas were observed.

Análisis de la supervivencia del cáncer de mama durante el decenio 1999-2008 en un hospital público de Madrid / Analysis of the Breast Cancer Survival in a Public Hospital during the Period 1999-2008

Fundamentos: El estudio de la supervivencia explora la calidad de la atención proporcionada en un hospital. El objetivo del presente trabajo es conocer las variables demográficas y clinicopatológicas así como la supervivencia y evolución a lo largo del decenio 1999-2008 de las mujeres diagnosticadas de cáncer de mama. Métodos: Se recogió la información relativa a las 2.132 mujeres incluidas en el Registro Hospitalario de tumores en esa década. Su seguimiento se realizó hasta el 31 de marzo de 2011 utilizando como fuente de información el Índice Nacional de Defunciones y la fecha del último contacto de la paciente con el hospital. Se calculó la supervivencia observada, estimada mediante el método actuarial, y la supervivencia relativa, calculada por el método Ederer II, para cada uno de los primeros cinco años de seguimiento junto con su intervalo de confianza al 95%. Resultados: La edad media fue 59,9 ± 14,2 años. 12,3% presentaron carcinoma "in situ", 51,8% localizado, 30% con ganglios positivos y 5% diseminado. Según el tipo histológico, 72,8% fueron ductales y 11,8% lobulillares. Se aplicó cirugía en 90,8%, quimioterapia en 23,4% y radioterapia en 56,7%. La supervivencia global relativa fue 88% a los 5 años; 99,9% en carcinomas "in situ"; 94,3% en localizados; 83,7% en regionales y 25,7% en diseminados. Al tercer año de seguimiento las mujeres diagnosticadas en 2008 mostraron una supervivencia relativa de 94,8% versus 89,5% en las de 1999, pero la estimación a lo largo del seguimiento fluctuó sin existir una tendencia significativa ni de manera global ni por estadios. Conclusiones: La evolución de la supervivencia por año de diagnóstico no mostró incremento estadísticamente significativo, ni comparándola por cohortes de los dos quinquenios ni desglosándola por cohortes de estadio al diagnóstico. No se ha conseguido variar la supervivencia cuando se exploró durante 10 años(AU)

Background: This report shows the effect of period of diagnosis and treatment in the breast cancer survival in a public hospital between 1999 and 2008. The main aim of this article is the knowledge of the different demographic, clinical and pathological variables among the women collected in our database in order to establish if there have been variations in the evolution of the survival rate in the this decade. Methods: 2132 women were included in the Hospital Cancer Registry between 1999 and 2008. These patients were followed-up by the National Death Index Agency and the last date of medical consultation, up to 31st march 2011. Observed survival was estimated by the actuarial method, and the relative survival was calculated by the Ederer II method. Survival was presented by fixed time point, as such 1, 3 and 5 year, after diagnosis. Confidence Interval 95% was included. Results: The average age at diagnosis was 59,9 ± 14,2 years old. At the time of diagnosis, 12,3% presented in situ carcinoma; 51,8% localized carcinoma; 30% positive lymph nodes and 5% disseminated carcinoma. 72,8% of the cases were ductal carcinomas and 11,8% lobular carcinomas. Surgery resection was applied in the 90,8% of the cases, chemotherapy was administered in 23,4% and radiotherapy in the 56,7%. The overall relative survival was 88% in 5 years; 99, 9% for "in situ" carcinoma, 94,3% for localized carcinoma, 83,7% for regional carcinomas and 25,7% for disseminated carcinoma. In the third year of follow-up, women diagnosed in 2008 presented a relative survival of 94,8% versus 89,5% of those diagnosed in 1999. However, during the follow-up the estimation fluctuated without any significant tendency as a whole or by stages. Conclusions: Evolution of relative survival per year of diagnosis didn’t show any significant increase, even when it was compared across the cohorts of two five-years periods or when it was studied by cohorts of cancer stage at diagnosis. In a 10 year period, relative survival hasn’t changed in the population treated in this hospital(AU)

[Analysis of the breast cancer survival in a public hospital during the period 1999-2008]. / Análisis de la supervivencia del cáncer de mama durante el decenio 1999-2008 en un Hospital Público de Madrid.

BACKGROUND: This report shows the effect of period of diagnosis and treatment in the breast cancer survival in a public hospital between 1999 and 2008. The main aim of this article is the knowledge of the different demographic, clinical and pathological variables among the women collected in our database in order to establish if there have been variations in the evolution of the survival rate in the this decade. METHODS: 2132 women were included in the Hospital Cancer Registry between 1999 and 2008. These patients were followed-up by the National Death Index Agency and the last date of medical consultation, up to 31st march 2011. Observed survival was estimated by the actuarial method, and the relative survival was calculated by the Ederer II method. Survival was presented by fixed time point, as such 1, 3 and 5 year, after diagnosis. Confidence Interval 95% was included. RESULTS: The average age at diagnosis was 59,9 ± 14,2 years old. At the time of diagnosis, 12,3% presented in situ carcinoma; 51,8% localized carcinoma; 30% positive lymph nodes and 5% disseminated carcinoma. 72,8% of the cases were ductal carcinomas and 11,8% lobular carcinomas. Surgery resection was applied in the 90,8% of the cases, chemotherapy was administered in 23,4% and radiotherapy in the 56,7%. The overall relative survival was 88% in 5 years; 99, 9% for "in situ" carcinoma, 94,3% for localized carcinoma, 83,7% for regional carcinomas and 25,7% for disseminated carcinoma. In the third year of follow-up, women diagnosed in 2008 presented a relative survival of 94,8% versus 89,5% of those diagnosed in 1999. However, during the follow-up the estimation fluctuated without any significant tendency as a whole or by stages. CONCLUSIONS: Evolution of relative survival per year of diagnosis didn't show any significant increase, even when it was compared across the cohorts of two five-years periods or when it was studied by cohorts of cancer stage at diagnosis. In a 10 year period, relative survival hasn't changed in the population treated in this hospital.

Neuroendocrine tumor of the pancreas in a patient with tuberous sclerosis: a case report and review of the literature.

A rare case of pancreatic neuroendocrine neoplasm in a patient with tuberous sclerosis complex is described. The patient was a 31-year-old man who had multiple congenital subependymal nodules, bilateral cortical tubers, and seizures of difficult control. A 2.3 cm × 2 cm well-delimitated solid tumor in the tail of the pancreas was discovered during a monitoring abdominal computed tomography. A distal pancreatectomy was performed. Histologically, the tumor was formed by uniform cells with moderated cytoplasm arranged in a combined trabecular and nested pattern. The nuclear features were bland, and mitosis was infrequent. There was no vascular invasion. Immunoreactivity for cytokeratine AE1/AE3, chromogranin A, and synaptophysin confirmed the neuroendocrine nature of this neoplasia. Pancreatic hormones were negatives. One of the 5 lymph nodes isolated from the peripancreatic adipose tissue was positive for metastases. Small series and case reports have documented that in tuberous sclerosis many endocrine system alterations might occur, affecting the function of the pituitary, parathyroid, and other neuroendocrine tissue, including islet cells of the pancreas. However, the true association of these pathological conditions remains uncertain. As far as we know, there are 10 cases reported of pancreatic neuroendocrine tumors in a setting of tuberous sclerosis complex, in which 2 cases resulted in malignant, nonfunctioning pancreatic neuroendocrine tumors.

Primary retroperitoneal liposarcoma: clinical and histological analysis of ten cases

El liposarcoma retroperitoneal constituye una neoplasia infrecuente y localmente agresiva. Realizamos un análisis retrospectivo de los 10 pacientes (6 varones; edad media: 63,2±11años) con diagnóstico histológico de liposarcoma retroperitoneal atendidos en nuestra institución entre 1999–2007. La presencia de una masa abdominal palpable constituyó la forma de presentación más frecuente. Todos los pacientes fueron sometidos a resección quirúrgica, obteniéndose en cuatro casos márgenes negativos. El análisis histológico reveló lo siguientes subtipos: bien diferenciado (6 casos), desdiferenciado (dos casos), pleomórfico (un caso) y mixoide/células redondas (un caso). En cinco casos fue necesaria la resección concomitante de órganos adyacentes. La mitad de los pacientes presentaron recurrencia tumoral, fundamentalmente limitada al retroperitoneo o a la cavidad abdominal. La supervivencia media libre de recurrencia fue de 43,3 meses (IC 95%: 25,7–60,8), con una probabilidad de supervivencia global a los 3 y 5 años del 79 y 61%. Los pacientes sometidos a resección quirúrgica completa con márgenes negativos presentaron una tendencia hacia una mayor supervivencia libre de recurrencia (62,9 vs. 29,3 meses; p=0.06) (AU)

Retroperitoneal liposarcoma constitutes an uncommon and locally aggressive malignancy. We performed a retrospective analysis of 10 patients (6 males; mean age: 63.2±11 years) with histologically proven retroperitoneal liposarcoma seen at our institution between 1999 and 2007. Presence of a palpable abdominal mass was the main symptom at diagnosis. All patients underwent complete surgical resection. Negative microscopic margin was achieved in four cases. Histological analysis revealed the following subtypes: well-differentiated (6 cases), dedifferentiated (two cases), pleomorphic, and myxoid/round cell (one case each). Concomitant resection of adjacent organs was needed in five cases. Half of the patients developed tumor recurrence, mainly limited to the retroperitoneum or abdominal cavity. The mean recurrence-free survival was 43.3 months (95%CI: 25.7–60.8), with 3- and 5-year overall survival rates of 79% and 61%, respectively. Patients undergoing complete resection with clear margins showed a near-significant trend toward increased recurrence-free survival (62.9 vs. 29.3 months; P=0.06) (AU)

Primary retroperitoneal liposarcoma: clinical and histological analysis of ten cases.

Retroperitoneal liposarcoma constitutes an uncommon and locally aggressive malignancy. We performed a retrospective analysis of 10 patients (6 males; mean age: 63.2+/-11 years) with histologically proven retroperitoneal liposarcoma seen at our institution between 1999 and 2007. Presence of a palpable abdominal mass was the main symptom at diagnosis. All patients underwent complete surgical resection. Negative microscopic margin was achieved in four cases. Histological analysis revealed the following subtypes: well-differentiated (6 cases), dedifferentiated (two cases), pleomorphic, and myxoid/round cell (one case each). Concomitant resection of adjacent organs was needed in five cases. Half of the patients developed tumor recurrence, mainly limited to the retroperitoneum or abdominal cavity. The mean recurrence-free survival was 43.3 months (95%CI: 25.7-60.8), with 3- and 5-year overall survival rates of 79% and 61%, respectively. Patients undergoing complete resection with clear margins showed a near-significant trend toward increased recurrence-free survival (62.9 vs. 29.3 months; P=0.06).

Comorbidity negatively influences prognosis in patients with extrahepatic cholangiocarcinoma.

AIM: To study the outcome and prognostic factors in a series of patients with extrahepatic cholangiocarcinoma and determine the impact of comorbidity on survival. METHODS: A retrospective analysis of 68 patients with extrahepatic cholangiocarcinoma (perihilar, n=37; distal, n=31) seen at a single tertiary-care institution during the period 1999-2003 was performed. Data on presentation, management, and outcome were assessed by chart review. Pathologic confirmation was obtained in 37 cases (54.4%). Comorbidity was evaluated by using the Charlson comorbidity index (CCI). RESULTS: Mean age at diagnosis was 73.4+/-11.5 years. Jaundice was the most common symptom presented (86.8%). Median CCI score was 1 (range, 0 to 4). Nineteen patients (27.9%) underwent tumor resection. Palliative biliary drainage was performed in 39 patients (57.4%), and 6 patients (8.8%) received only best supportive care. Tumor-free margin status (R0) was achieved in 15 cases (78.9% of resection group). Baseline serum carbohydrate antigen 19-9 (CA 19-9) level was revealed to be an independent predictor of surgical treatment (P=0.026). Overall median survival was 3.1+/-0.9 mo, with 1- and 2-year survival rates of 21% and 7%, respectively. In the univariate analysis, tumor resection, CCI score, and serum CA 19-9 levels correlated significantly with outcome. In the multivariate analysis, only resection (HR 0.10; 95% CI, 0.02-0.51, P=0.005) and a CCI score>or=2 (HR 3.36; 95% CI, 1.0-10.9, P=0.045) were found to independently predict survival. CONCLUSION: Tumor resection and comorbidity emerged as significant prognostic variables in extrahepatic cholangiocarcinoma. Comorbidity evaluation instruments should be applied in the clinical management of such patients.

Predicción pronóstica en pacientes con carcinoma hepatocelular no subsidiario de tratamiento locorregional / Prognostic prediction in patients with hepatocellular carcinoma not amenable to locoregional treatment

Fundamento y objetivo: Los pacientes con carcinoma hepatocelular (CHC) no subsidiarios de tratamiento locorregional presentan una serie de características (cirrosis descompensada o extensión extrahepática) que obligan a evaluar de forma específica la utilidad de los distintos sistemas de estadificación propuestos para esta entidad. Pacientes y método: Analizamos 100 pacientes (69 varones) con una edad media (DE) de 70,2 años (10,7), y establecimos su estadificación al diagnóstico del CHC según los modelos de Okuda, Cancer of the Liver Italian Program, Barcelona Clinic Liver Cancer (BCLC) y Japan Integrated Staging score. Empleando la mortalidad a los 6 y a los 12 meses como objetivo, calculamos el área bajo la curva (ABC) de características operativas del receptor. Resultados: El modelo BCLC mostró la mejor capacidad de predicción, tanto a los 6 (ABC: 0,871) como a los 12 meses (ABC: 0,924), y mantuvo la significación estadística en el análisis multivariante de Cox (hazard ratio: 9,14 para el estadio BCLC C frente a A [p<0,001] y 9,53 para el estadio BCLC D frente a A [p=0,004]) (AU)

Background and objective: Patients with hepatocellular carcinoma (HCC) not amenable to locoregional therapy have some specific clinical characteristics (advanced cirrhosis or extrahepatic spread) that lead us to ascertain the prognostic efficacy of the different staging systems proposed for this entity. Patients and method: We analyze 100 patients (69 males) with a mean age of 70.2 (10.7) years. Tumor staging was performed at baseline according to the following systems: Okuda, Cancer of the Liver Italian Program (CLIP), Barcelona Clinic Liver Cancer (BCLC), and Japan Integrated Staging (JIS) score. We determine the area under the receiver operating characteristic curve (AUC) using the 6 and 12-month mortality as endpoint. Results: The BCLC system had the best predictive power for mortality both at 6 (AUC, 0.871) and 12 months (AUC, 0.924) from diagnosis. In the Cox multivariate analysis, this model showed an independent prognostic effect [hazard ratio: 9.14 for BCLC stage C versus A (p<0.001), and 9.53 for stage D versus A (p=0.004)]. Conclusions: The BCLC system provided the best prognostic stratification for patients with HCC not amenable to locoregional therapy (AU)

[Prognostic prediction in patients with hepatocellular carcinoma not amenable to locoregional treatment]. / Predicción pronóstica en pacientes con carcinoma hepatocelular no subsidiario de tratamiento locorregional.

BACKGROUND AND OBJECTIVE: Patients with hepatocellular carcinoma (HCC) not amenable to locoregional therapy have some specific clinical characteristics (advanced cirrhosis or extrahepatic spread) that lead us to ascertain the prognostic efficacy of the different staging systems proposed for this entity. PATIENTS AND METHOD: We analyze 100 patients (69 males) with a mean age of 70.2 (10.7) years. Tumor staging was performed at baseline according to the following systems: Okuda, Cancer of the Liver Italian Program (CLIP), Barcelona Clinic Liver Cancer (BCLC), and Japan Integrated Staging (JIS) score. We determine the area under the receiver operating characteristic curve (AUC) using the 6 and 12-month mortality as endpoint. RESULTS: The BCLC system had the best predictive power for mortality both at 6 (AUC, 0.871) and 12 months (AUC, 0.924) from diagnosis. In the Cox multivariate analysis, this model showed an independent prognostic effect [hazard ratio: 9.14 for BCLC stage C versus A (p<0.001), and 9.53 for stage D versus A (p=0.004)]. CONCLUSIONS: The BCLC system provided the best prognostic stratification for patients with HCC not amenable to locoregional therapy.

Multiple primary malignancies in Spanish patients with hepatocellular carcinoma: analysis of a hospital-based tumor registry.

BACKGROUND AND AIM: Little is known about the etiological associations and clinical features of extrahepatic primary malignant (EHPM) neoplasms in subjects with hepatocellular carcinoma (HCC). The aim of this study was to characterize this phenomenon in a consecutive series of Spanish patients in order to define its natural history and influence on survival. METHODS: A retrospective analysis of 245 patients with HCC during the period 1999-2003 was performed. Subjects identified with a second primary malignancy elsewhere constituted the EHPM group and were compared to patients with HCC alone. RESULTS: Eighteen patients (7.3%) had one or two associated extrahepatic malignancies (mean age 67.7 +/- 9.7 years); of these, 17 had double cancer and one patient, triple. Nine of the 19 EHPM occurred before HCC diagnosis. The associated cancers included five cases of colorectal carcinoma, four cases of head and neck carcinoma, three cases of genitourinary cancer, two cases of lymphoproliferative disorder, one lung carcinoma, one skin melanoma, one breast carcinoma, and two cancers of unknown origin. Age and sex distribution, etiology of underlying hepatopathy, and liver function tests did not differ significantly between both groups. There was no difference between the overall survival rates. CONCLUSIONS: EHPM is not rare among Spanish patients with HCC, although no specific clinicopathological features were detected in this population. Our results suggest that the association of another primary tumor with HCC does not imply a worse prognosis. The possibility of development of EHPM should be kept in mind when deciding on therapy and follow-up of HCC.