Construction of a Prognostic Prediction Model of Patients with Pathologic N0 in Resected Invasive Mucinous Adenocarcinoma of the Lung / 中国肺癌杂志

BACKGROUND@#Invasive mucinous adenocarcinoma (IMA) was a rare and specific type of lung adenocarcinoma, which was often characterized by fewer lymphatic metastases. Therefore, it was difficult to evaluate the prognosis of these tumors based on the existing tumor-node-metastasis (TNM) staging. So, this study aimed to develop Nomograms to predict outcomes of patients with pathologic N0 in resected IMA.@*METHODS@#According to the inclusion criteria and exclusion criteria, IMA patients with pathologic N0 in The Affiliated Lihuili Hospital of Ningbo University (training cohort, n=78) and Ningbo No.2 Hospital (validation cohort, n=66) were reviewed between July 2012 and May 2017. The prognostic value of the clinicopathological features in the training cohort was analyzed and prognostic prediction models were established, and the performances of models were evaluated. Finally, the validation cohort data was put in for external validation.@*RESULTS@#Univariate analysis showed that pneumonic type, larger tumor size, mixed mucinous/non-mucinous component, and higher overall stage were significant influence factors of 5-year progression-free survival (PFS) and overall survival (OS). Multivariate analysis further indicated that type of imaging, tumor size, mucinous component were the independent prognostic factors for poor 5-year PFS and OS. Moreover, the 5-year PFS and OS rates were 62.82% and 75.64%, respectively. In subgroups, the survival analysis also showed that the pneumonic type and mixed mucinous/non-mucinous patients had significantly poorer 5-year PFS and OS compared with solitary type and pure mucinous patients, respectively. The C-index of Nomograms with 5-year PFS and OS were 0.815 (95%CI: 0.741-0.889) and 0.767 (95%CI: 0.669-0.865). The calibration curve and decision curve analysis (DCA) of both models showed good predictive performances in both cohorts.@*CONCLUSIONS@#The Nomograms based on clinicopathological characteristics in a certain extent, can be used as an effective prognostic tool for patients with pathologic N0 after IMA resection.

Mucinous tubular and spindle cell carcinoma of kidney: Clinicopathology and prognosis / 北京大学学报(医学版)

OBJECTIVE@#To investigate and summarize the clinicopathological features, immunophenotype, differential diagnosis and prognosis analysis of mucinous tubular and spindle cell carcinoma (MTSCC).@*METHODS@#The data of thirteen cases of MTSCC were retrospectively analyzed, the clinical and pathological characteristics and immunohistochemical expression were summarized, and fluorescence in situ hybridization was detected.@*RESULTS@#Among the thirteen patients, four were males and nine females, with a male-to-female ratio of 1 ∶2.25. The average age was 57.1 years, ranging from 39 to 78 years. The maximum diameter of the tumor was 2-12 cm. All cases had no symptoms, and were accidentally discovered, 3 cases underwent partial renal resection, 10 cases underwent radical renal resection, 9 cases were located in the left kidney, and 4 cases were located in the right kidney. Most of the cases showed the classical morphological changes, with 11 cases of nuclear grading [World Health Organization (WHO)/International Society of Urological Pathology (ISUP) grading system] being G2 and 2 cases being G3. There were 6 cases of stage PT1a, 3 cases of PT1b, 2 cases of PT2a, and 1 case of PT2b and 1 case of PT3a. The positive rates of immunohistochemical staining were: vimentin, AE1/AE3, α-methylacyl-CoA racemase (αMACR) and cytokeratin (CK) 8/18, 100% (13/13); CK7, 92.3% (12/13); epithelial membrane antigen (EMA), 92.3% (12/13); CK20, 46.2% (6/13); CD10, 30.8% (4/13); synaptophysin (Syn), 7.7% (1/13); chromogranin A (CgA), CD57, WT1 and Ki-67, 0 (0/13), and fluorescence in situ hybridization showed that no trisomy of chromosomes 7 and 17 were observed in any of the cases. The follow-up period was 6 months to 7 years and 6 months, 2 cases died after lung metastasis (one with ISUP/WHO grade G3, one with necrosis), and the remaining 11 cases had no recurrence and metastasis.@*CONCLUSION@#MTSCC is a unique type of low-grade malignancy kidney tumor, occurs predominantly in females, widely distributed in age, the current treatment method is surgical resection, and cases with necrosis and high-grade morphology are prone to recurrence and metastasis, although most cases have a good prognosis, but they still need close follow-up after surgery.

Skip metastasis at the esophageal resection margin in radical gastrectomy: clinical characteristics of 30 cases / 中华胃肠外科杂志

Objective: To summarize the clinical characteristics of patients with skip metastasis at esophageal resection margin during radical gastrectomy. Methods: This is a descriptive study of case series. Relevant data from 2006 to 2022 were collected from two major gastric cancer consultation and treatment centers: Nanjing Drum Tower Hospital and Jinling Hospital.Characteristics, surgical approach, number of dissected lymph nodes, immunohistochemical staining, and pathological staging were summarized and analyzed. The distribution of residual tumor cells at the esophageal margins was further analyzed at the cellular and tissue levels. Skip metastasis at the esophageal resection margin was defined as a negative esophageal margin with a positive margin in the cephalad donut. Results: Thirty (0.33%, 30/8972) eligible patients, 24 (80.0%) of whom were male, were identified in the two centers. The mean age was 63.9±11.0 years. Seventeen (56.7%) of these patients had papillary or tubular adenocarcinomas, including 13 (43.3%) poorly- and four (13.3%) moderately-differentiated tumors; four (13.3%) had signet-ring cell carcinomas; four (13.3%) mucinous adenocarcinomas; three (10.0%) mixed adenocarcinomas, including two with poorly-differentiated tubular adenocarcinomas mixed with signet-ring cell carcinoma and mucinous adenocarcinoma; and one had a poorly differentiated tubular adenocarcinoma mixed with signet-ring cell carcinoma. Two patients (6.7%) had other types of cancer, namely adenosquamous carcinoma in one patient and undifferentiated carcinoma in the other one. The predominant tumor sites were the lesser curvature (n=26, 86.7%) and the cardia (n=24, 80.0%). The mean tumor diameter was 6.6 cm, mean distance between tumor and esophageal resection margin was 1.5 cm, and proportions of tumor invasion into the dentate line, nerves, and vessels were 80.0% (24/30), 86.7%(26/30), and 93.3% (28/30), respectively. The mean number of lymph nodes resected was 20.4±8.9. The pathological stage was mainly T4 (n=18, 60.0%) and N3 (n=21, 70.0%), the median Ki67 was 52.7%, and the rates of positivity for HER2, EGFR, VEGFR, E-cadherin and PD-L1 were 40.0% (12/30), 46.7% (14/30), 80.0% (24/30), 86.7% (26/30) and 16.7% (5/30), respectively. At the cellular level, cancer cells were mainly distributed in small focal areas, as cell masses, or as tumor thrombi; large numbers of widely distributed atypic cells were seldom observed. At the tissue level, cancer cells were located in the mucosal layer in seven patients (23.3%), in the submucosal layer in 18 (60.0%), and in the muscular layer in five (16.7%); no cancer cells were identified in the outer membrane. Five of the seven tumors were located in the lamina propria, two in the muscularis mucosae, and none in the mucosal epithelium. Conclusion: Patients with skip metastasis at the esophageal resection margin at radical gastrectomy have unfavorable tumor biology and a high proliferation index, are at a late pathological stage, and the residual cancer is mostly located in the submucosa.

Manejo con cirugía conservadora de tumor mucinoso infiltrante ovárico coexistente con tumor mucinoso borderline contralateral en gestante de 8 semanas / Conservative surgery for management of invasive mucinous ovarian tumor with concurrent borderline mucinous tumor on contralateral ovary in a woman in 8th week of pregnancy

INTRODUCCIÓN: Aproximadamente un 4% de mujeres embarazadas presentan tumoraciones anexiales en el primer trimestre de gestación, siendo la mayoría quistes anexiales simples. Solo el 1,5% persisten tras el primer trimestre y en torno al 0,9% son malignos. CASO CLÍNICO: Paciente de 31 años que consultó en urgencias por sangrado y amenorrea de 5 semanas. La ecografía transvaginal evidenció un saco gestacional intraútero y una tumoración anexial izquierda compleja de 68 mm, multilocular, sólida, de ecogenicidad mixta, con septos gruesos y un área sólida vascularizada de 40 mm, sospechosa de neoplasia de ovario. Se realizó manejo quirúrgico conservador mediante anexectomía bilateral laparoscópica en la octava semana de gestación. El diagnóstico fue de adenocarcinoma mucinoso infiltrante bien diferenciado en ovario derecho, coexistente con tumor mucinoso borderline en ovario izquierdo (etapa IC FIGO). El embarazo cursó con normalidad, a excepción de feto pequeño para la edad gestacional en la semana 39. Se finalizó la gestación en la semana 40 mediante parto vaginal. Debido al deseo de la paciente, la cirugía se realizó en el primer trimestre de embarazo en lugar de en el segundo como se recomienda. La cirugía se completó meses después del parto, con histerectomía y apendicectomía laparoscópica. DISCUSIÓN: El cáncer de ovario es el segundo tumor ginecológico más diagnosticado durante el embarazo. Suele diagnosticarse en el primer trimestre debido al cribado ecográfico gestacional. Ocurre en mujeres con baja paridad y en sus últimos años de edad reproductiva. Esto podría verse reflejado en un aumento de su incidencia debido al retraso de la edad materna al primer embarazo. Normalmente se diagnostica en etapa I, siendo entonces la supervivencia superior al 90% a los 5 años. En etapas iniciales en mujeres embarazadas, la laparoscopia es tan válida como la laparotomía.

INTRODUCTION: Approximately 4% of pregnant women present adnexal tumors in the first trimester of gestation, the majority being simple adnexal cysts. Only 1.5% persist after the first trimester and around 0.9% are malignant. CASE REPORT: 31-year-old patient who came to the emergency room because of spotting and 5-week amenorrhea. A transvaginal ultrasound revealed an intrauterine gestational sac and a 69 mm complex multiocular-solid left adnexal tumor, with thick septa and a solid vascularized area of 40 mm, with suspicion of ovarian neoplasia. Conservative surgical management was performed through laparoscopic bilateral salpingo-oophorectomy in eighth week of gestation. The diagnosis was a well-differentiated infiltrating mucinous adenocarcinoma in the right ovary, and a coexisting borderline mucinous tumor in the left ovary (FIGO stage IC). The pregnancy proceeded normally except for a small for gestational age fetus at week 39 and pregnancy was completed at week 40 by vaginal delivery. Due to the patients wishes, the surgery was performed in the 1st trimester of pregnancy instead in the 2nd as is recommended. Final surgery was completed after giving birth, with laparoscopic hysterectomy and appendectomy. DISCUSSION: Ovarian cancer is the second most diagnosed gynecological tumor during pregnancy. It is usually diagnosed during the first trimester with gestational ultrasound screening. It occurs in women with low parity and in their later years of reproductive age. This could be reflected in an increase in its incidence due to the delay in the age of first pregnancy. Normally it is diagnosed in stage I, when the survival rate is superior to 90% after 5 years. In early stages, laparoscopic surgery in pregnant women is as valid as laparotomy.

Mucinous adenocarcinoma of the appendix with inguinal node metastases

Mucinous adenocarcinoma of the appendix is a rare neoplasm with a low propensity for lymph node metastasis. The present case refers to an appendicular mucinous adenocarcinoma with inguinal lymph node metastasis. A 71-year-old woman underwent an appendectomy due to a clinical presentation of acute appendicitis. However, the histological examination of the surgical specimen revealed a mucinous adenocarcinoma of the appendix. After staging, the patient underwent a right hemicolectomy and was proposed for adjuvant chemotherapy. At the 3rd year of follow-up, inguinal lymphadenopathy was diagnosed, which biopsy confirmed inguinal node metastases from primary colorectal cancer, with areas of extracellular mucin. Restaging revealed liver and peritoneal metastasis, and the patient was proposed for palliative chemotherapy. Appendicular neoplasms, due to their rarity, represent a diagnostic and therapeutic challenge. This clinical case depicts an unusual metastasis pathway for an unusual neoplasm.

Metachronic breast and cerebellar neoplasm in a young patient / Neoplasias mamária e cerebelar metacrônicas em uma paciente jovem

Abstract Several factors trigger the development of genetic mutations that are responsible for causing a neoplasm. Medulloblastoma is a malignant and invasive cerebellar neoplasm, that affects children and young adults. Mucinous carcinoma is a special type of breast cancer. Being a special atypical subtype of invasive carcinoma, it most frequently affects women of advanced age and represents 1 to 7% of all breast cancers. The reported case aims to show the rarity of the occurrence of desmoplastic medulloblastoma and mammary mucinous carcinoma in a young patient in a short period of time, in different sites, without direct anatomical attachment and without occurrence of metastasis. Initially, this patient had a desmoplastic medulloblastoma and was treated with lumpectomy and radiotherapy. After 13 months, the patient was diagnosed with a mucinous breast carcinoma, underwent mastectomy, adjuvant chemotherapy and is currently undergoing endocrinotherapy. We conclude, based on the metachronous characteristic of the neoplasia and clinical characteristics, that the patient is likely to have Li-Fraumeni syndrome, an autosomal dominant disease with mutation of the TP53 gene, which is the the main involved. Because the patient does not present all the characteristics of the phenotype of the syndrome, she can thus be classified as having Li-Fraumeni variant or Li-Fraumeni-like syndrome.

Resumo Diversos fatores desencadeiam o desenvolvimento de mutações genéticas que são responsáveis por originar uma neoplasia. O meduloblastoma é uma neoplasia cerebelar maligna e invasiva que acomete crianças e adultos jovens. O carcinoma mucinoso é um tipo de câncer de mama especial por ser um subtipo atípico de carcinoma invasivo, que acomete com maior frequência mulheres de idade avançada e representa entre 1 a 7% do total de neoplasias mamárias. O caso relatado tem como objetivo mostrar a raridade da ocorrência do meduloblastoma desmoplásico e carcinoma mucinoso mamário em uma paciente jovem em um curto período de tempo, em diferentes sítios sem ligação anatômica direta e sem ocorrência de metástase. Inicialmente, esta paciente possuía um meduloblastoma desmoplásico e foi tratada com tumorectomia e radioterapia. Após 13 meses, a paciente foi diagnosticada com carcinoma mucinoso de mama, sendo submetida a mastectomia, quimioterapia adjuvante e atualmente está sendo tratada com endocrinoterapia. Concluímos, com base na característica metacrônica da neoplasia e características clínicas, que a paciente apresenta a síndrome de Li-Fraumeni, doença autossômica dominante com mutação do gene TP53, que é o principal gene envolvido nesta síndrome. Por não apresentar as características completas do fenótipo da síndrome, a paciente pode assim ser classificada como portadora de uma variante da síndorme de Li-Fraumeni ou síndrome do tipo Li-Fraumeni.

Cáncer de colon con infiltración a pared abdominal: un reto diagnóstico / Colonic cancer with infiltration to the abdominal wall: a diagnostic challenge

Se presenta el caso de una paciente mujer de 53 años procedente de Piura con un tiempo de enfermedad de 20 meses, caracterizado por dolor en sitio de herida quirúrgica por colecistectomía abierta realizada hace 3 años, asociado a presencia de una masa en dicha zona, posteriormente se absceda y comienza a drenar secreción alimentaria. Los estudios de imágenes revelaron una masa dependiente de colon transverso en contacto con estómago y pared abdominal que presentaba fistulas hacia piel. Dicha masa fue extraída durante la cirugía con resultado anatomopatológico de adenocarcinoma mucinoso de colon. El caso representó un reto diagnóstico para el equipo médico y en vista de las diversas manifestaciones clínicas del cáncer de colon, sugerimos mantenerlo presente como diagnóstico diferencial en cuadros de absceso de pared abdominal y fistulizaciones entéricas.

We present the case of a 53-year-old woman with a time of illness of 20 months, characterized by pain at the site of surgical intervention for opened cholecystectomy 3 years ago, associated with a presence of a mass in said area, with the following abscess formation and fistulization of food content. Imaging studies revealed a mass dependent of the transverse colon, in contact with stomach and abdominal wall and presenting fistulas to the skin. Said mass was extracted during surgery with anatomopathological result of mucinous colon adenocarcinoma. The case represented a diagnostic challenge for the medical team and in view of the variaty of clinical manifestations of colonic cancer, we suggest that it should be consider as a differential diagnosis in cases of abdominal wall abscess and enteric fistulas.

Pseudomyxoma peritonei in a pediatric patient: A case report and literature review / Pseudomixoma peritoneal em paciente pediátrico: relato de caso e revisão de literatura

Summary Introduction: Pseudomyxoma peritonei (PMP) is a rare clinical condition, with an incidence of 1-2 cases per million, characterized by the dissemination of mucinous implants on the peritoneal surface and progressive gelatinous ascites. Although it usually presents an indolent behavior, its non-specific clinical presentation contributes to many cases remaining undiagnosed until a laparotomy is performed. With late diagnosis, performed after a long period of clinical deterioration and disease progression, it is common to find complications such as the formation of intestinal fistulas and obstruction. Method: Review of the medical record and search for references in the Medline, Lilacs, SciELO and MD Consult databases. Results: There are rare case reports found in the literature demonstrating atypical PMP presentations. Our report is that of a 17-year-old adolescent with a sporadic tumor diagnosed in a primary site in the transverse colon, contrary to data commonly found in the literature that mention a more frequent occurrence in women in the fifth decade of life and with a primary site in the ovary and appendix. The development of mucinous adenocarcinoma is rare in the pediatric population, and topography in the transverse colon and non-familial sporadic pattern are unusual. Conclusion: The case reported not only raises awareness about the atypical presentations of the disease, but also emphasizes the use of imaging examinations for diagnosis, which has an important impact on prognosis and survival if performed timely.

Resumo Introdução: O pseudomixoma peritoneal (PMP) é uma condição clinica rara, com incidência de 1-2 casos por milhão, caracterizada pela disseminação de implantes de natureza mucinosa pela superfície peritoneal e acúmulo progressivo de ascite gelatinosa. Embora apresente geralmente um comportamento indolente, a apresentação clínica inespecífica contribui para que muitos casos permaneçam sem diagnóstico até a realização de laparotomia. Com o diagnóstico tardio, realizado após um longo período de deterioração clínica e progressão de doença, é comum encontrar complicações, como a formação de fístulas e obstruções intestinais. Método: Revisão do prontuário médico e pesquisa bibliográfica nas bases de dados Medline, Lilacs, SciELO e MD Consult. Resultados: São raros os relatos de caso encontrados na literatura que demonstram apresentações atípicas do PMP. O presente estudo apresenta o caso de um adolescente com 17 anos ao diagnóstico e sítio primário no colón transverso com tumor esporádico, contrário aos dados comumente encontrados na literatura, que referem acometimento mais comum em mulheres na quinta década de vida e com sítio primário em ovário e apêndice. O desenvolvimento de adenocarcinoma mucinoso é raro na população pediátrica e a topografia no cólon transverso e padrão esporádico não familial também são pouco usuais. Conclusão: O caso relatado alerta para as apresentações atípicas da doença e enfatiza o uso de exames de imagem para o diagnóstico, que, se realizado precocemente, impacta de maneira importante o prognóstico e a sobrevida.

A Case Report: Cavitary Infarction Caused by Pulmonary Tumor Thrombotic Microangiopathy in a Patient with Pancreatic Intraductal Papillary Mucinous Neoplasm

Pulmonary tumor embolism is commonly discovered at autopsy, but is rarely suspected ante-mortem. Microangiopathy is an uncommon and distinct form of simple tumor pulmonary embolism. Here, we present a 52-year-old male with tumor thrombotic microangiopathy and pulmonary infarction, which might have originated from intraductal papillary mucinous tumor of the pancreas. Multiple wedge-shaped consolidations were found initially and aggravated with cavitation. These CT features of pulmonary infarction were pathologically confirmed to result from pulmonary tumor thrombotic microangiopathy.

Synchronous Pancreatic Ductal Adenocarcinomas Diagnosed by Endoscopic Ultrasound-Guided Fine Needle Biopsy

Cases of pancreatic ductal adenocarcinoma with multiple masses accompanying underlying pancreatic diseases, such as intraductal papillary mucinous neoplasm, have been reported. However, synchronous invasion without underlying pancreatic disease is very rare. A 61-year-old female with abdominal discomfort and jaundice was admitted to our hospital. Abdominal computed tomography (CT) revealed cancer of the pancreatic head with direct invasion of the duodenal loop and common bile duct. However, positron emission tomography-CT showed an increased standardized uptake value (SUV) in the pancreatic head and tail. We performed endoscopic ultrasound-guided fine needle biopsy (EUS-FNB) for the histopathologic diagnosis of the pancreatic head and the evaluation of the increased SUV in the tail portion of the pancreas, as the characteristics of these lesions could affect the extent of surgery. As a result, pancreatic ductal adenocarcinomas were confirmed by both cytologic and histologic analyses. In addition, immunohistochemical analysis of the biopsy specimens was positive for carcinoembryonic antigen and p53 in both masses. The two masses were ultimately diagnosed as pancreatic ductal adenocarcinoma, stage IIB, based on EUS-FNB and imaging studies. In conclusion, the entire pancreas must be evaluated in a patient with a pancreatic mass to detect the rare but possible presence of synchronous pancreatic ductal adenocarcinoma. Additionally, EUS-FNB can provide pathologic confirmation in a single procedure.

The role of intratumoral lymphovascular density in distinguishing primary from secondary mucinous ovarian tumors

OBJECTIVE: Ovarian mucinous metastases commonly present as the first sign of the disease and are capable of simulating primary tumors. Our aim was to investigate the role of intratumoral lymphatic vascular density together with other surgical-pathological features in distinguishing primary from secondary mucinous ovarian tumors. METHODS: A total of 124 cases of mucinous tumors in the ovary (63 primary and 61 metastatic) were compared according to their clinicopathological features and immunohistochemical profiles. The intratumoral lymphatic vascular density was quantified by counting the number of vessels stained by the D2-40 antibody. RESULTS: Metastases occurred in older patients and were associated with a higher proportion of tumors smaller than 10.0 cm; bilaterality; extensive necrosis; extraovarian extension; increased expression of cytokeratin 20, CDX2, CA19.9 and MUC2; and decreased expression of cytokeratin 7, CA125 and MUC5AC. The lymphatic vascular density was increased among primary tumors. However, after multivariate analysis, the best predictors of a secondary tumor were a size of 10.0 cm or less, bilaterality and cytokeratin 7 negativity. Lack of MUC2 expression was an important factor excluding metastasis. CONCLUSIONS: The higher intratumoral lymphatic vascular density in primary tumors when compared with secondary lesions suggests differences in the microenvironment. However, considering the differential diagnosis, the best discriminator of a secondary tumor is the combination of tumor size, laterality and the pattern of expression of cytokeratin 7 and MUC2. .

Pseudomixoma peritoneal por tumor del apéndice cecal / Peritoneal pseudomyxoma due to cecal appendix tumor

Objetivo: presentar un caso de una patología de muy baja incidencia y realizar una revisión bibliográfica de la misma. Paciente y método: paciente femenina de 87 años de edad que ingresa por guardia por cuadro compatible con hernia inguinal atascada, por lo que se decide cirugía de urgencia. En el acto operatorio se identifica un saco herniario de paredes engrosadas, con contenido intestinal con buena vitalidad, sin signos de necrosis, identificando además abundante secreción mucinosa en el saco herniario y proveniente de la cavidad abdominal. Se decide terminar con la hernioplastia y realizar estudios complementarios diferidos. Luego de los mismos, el comité de tumores decide la realización de nueva cirugía (hemicolectomía derecha con ileotransverso anastomosis por tumor mucinoso de apéndice), durante la cual se constata ascitis gelatinosa. La paciente evoluciona favorablemente y es dada de alta a las 72 horas del post operatorio. La anatomía patológica informa adenocarcinoma mucinoso de apéndice bien diferenciado. Discusión: el Pseudomixoma Peritoneal es una entidad muy poco frecuente, que hace referencia a la diseminación peritoneal de un tumor cuyas células producen gran cantidad de mucina. La supervivencia global de los pacientes es de aproximadamente 75 y 68% durante 5 y 10 años. La clasificación más aceptada es la de Ronnet. Tradicionalmente, el tratamiento ha sido la cirugía de citoreducción, aunque hay autores que proponen procedimientos más agresivos como la peritonectomía más quimioterapia hipertérmica intraoperatoria.

Objective: to present a case of a very low incidence disease and a bibliographic review of it. Patient and method: 87 years old female patient with a compatible history and physical exam for a stuck inguinal hernia, for which we decided to perform emergency surgery. During the surgical procedure, we find a thickened hernia sac, with mucus in it and in the abdominal cavity. The small intestine was vital and not complicated. We finished the hernioplasty, and decided to perform complementary ambulatory studies to the patient. After having them done, we decided, in an interdisciplinary committee, to perform an exploratory surgery, (hemicolectomy with ileum transverse anastomosis for an appendix tumor), during which gelatinous ascites was noticed. The patient evolve favorably and was discharged 72 hours post operation. The surgical biopsy informed an appendix mucinous adenocarcinoma well differentiated. Discussion: the peritoneal pseudomyxoma is a very infrequent entity. The overall survival is 75 and 68% for 5 and 10 years, respectively. Ronnet had published the most accepted histological classification. The accepted treatment is the cytoreductive surgery, although some authors proposed a more aggressive treatment such peritonectomy with hyerthermic intraperitoneal chemotherapy.

CDX-2, MUC-2 and B-catenin as intestinal markers in pure mucinous carcinoma of the breast

BACKGROUND: Pure mucinous adenocarcinoma of the breast is a rare entity characterized by the production of variable amounts of mucin comprising 1% to 6% of breast carcinomas. Some mucinous adenocarcinomas have shown expression of intestinal differentiation markers such as MUC-2. This study examines the expression of intestinal differentiation markers in this type of breast carcinoma. RESULTS: Twenty-two cases of pure mucinous adenocarcinoma of the breast were assessed. Immunochemistry was performed for beta-catenin, CDX-2 and MUC-2. All cases were positive for B-catenin. MUC-2 positivity was observed in all cases; 63. 6% were 3 plus positive. All cases were negative for CDX-2. CONCLUSIONS: These results suggest that mucinous breast carcinomas express some markers of intestinal differentiation, such as MUC-2 and beta-catenin; however, future studies with a larger series of cases and using molecular techniques that help affirm these results are needed.

The Expression of Multiple Proteins as Prognostic Factors in Colorectal Cancer: Cathepsin D, p53, COX-2, Epidermal Growth Factor Receptor, C-erbB-2, and Ki-67

BACKGROUND/AIMS: A single gene mutation alone cannot explain the poor prognosis of colorectal cancer. This study aimed to establish a correlation between the expression of six proteins and the prognosis of colorectal cancer patients. METHODS: Tissue samples were collected from 266 patients who underwent surgery for colorectal cancer at our institution from January 2006 to December 2007. The expression of six proteins were determined using immunohistochemical staining of specimens. RESULTS: Cathepsin D, p53, COX-2, epidermal growth factor receptor, c-erbB-2, and Ki-67 expression were detected in 38.7%, 60.9%, 37.6%, 35.7%, 30.1%, and 74.4% of the samples, respectively. The expression of cathepsin D was significantly correlated with reduced cancer-free survival (p=0.036) and colorectal cancer-specific survival (p=0.003), but the other expression levels were not. In a multivariate analysis, cathepsin D expression was found to be an independent prognostic factor for poorer colorectal cancer-specific survival (hazard ratio, 8.55; 95% confidence interval, 1.07 to 68.49). Furthermore, patients with tumors expressing four or more of the proteins had a significantly decreased cancer-free survival rate (p=0.006) and colorectal cancer-specific survival rate (p=0.002). CONCLUSIONS: Patients with cathepsin D positivity had a poorer outcome than patients who were cathepsin D-negative. Thus, cathepsin D may provide an indicator for appropriate intensive follow-up and adjuvant chemotherapy.

Unclassified Mucinous Renal Cell Carcinoma: A Rare Histopathological Entity

Renal cell carcinoma (RCC) with mucin production is extremely rare. We present the case of a previously healthy 76-year-old woman who underwent a robotic-assisted laparoscopic right nephrectomy for a 5-cm heterogeneously enhancing right renal mass. Pathology revealed mucin-producing epithelial RCC. We discuss the presentation and pathological features of this case and comment on its definitive treatment.

Carcinoma mucinoso ecrino primario de la piel: reporte de un caso clínico patológico y revisión de la literatura / Primary mucinous eccrine carcinoma of skin: a case report and literature review

El carcinoma mucinoso ecrino primario de la piel es infrecuente, existen cerca de 100 casos publicados en la literatura. Fue descrito por primera vez por Lennox y colaboradores el año 1952. Presentamos un caso de carcinoma mucinoso ecrino primario de la piel, en un hombre de 82 años de edad, quien consultó por una masa cutánea retroauricular de 10 años de evolución, sin evidencias de enfermedad sistémica ni recurrencia local al año del control postoperatorio

The primary mucinous eccrine carcinoma of skin is infrequent, nearby 100 cases are published in the literature. It was described by the first time by Lennox and cols. at 1952. We present a case of primarily mucinous eccrine carcinoma of the skin, in an 82 year old man, who consulted for a retroauricular cutaneous mass of 10 years of evolution, without evidences of systemic disease or local recurrence at the year of the after surgery.

Cistoadenocarcinoma de ovario y apéndice / Mucinous cystic adenocarcinoma of the ovary and appendix

We report a 73 years old woman presenting with a right ovarian tumor. Preoperative carcinoembryonic antigen and CA-125 levels were above normal levels. The patient was operated, performing a bilateral oopho-rectomy an appendectomy. The pathological study of the surgical piece reported a mucinous ovarian cystic adenocarcinoma that infiltrated the wall but did not reach the capsule. A mucinous cystadenoma was found in the contralateral ovary. A mucinous adenocarcinoma was detected in the appendix, involving the wall but sparing the serosa. The patient was subjected to chemotherapy and six months after the first operation a tumor relapse was detected. A new excision was carried out and the patient continues with adjuvant chemotherapy. The ovarian an appendiceal tumors could be synchronic or a primary and secondary localization.

Introducción: El cistoadenocarcinoma de apéndice es una neoplasia poco frecuente. La asociación de tumores apendiculares y ováricos es conocida cuando existe pseudomixoma peritoneal. Sin embargo, la relación de la patogénesis entre los tumores es todavía desconocida. Caso clínico: Mujer de 73 años con clínica de plenitud postprandial y dispepsia de un año de evolución. La RMN abdómino-pélvica demuestra una gran tumoración de 15x13x9 cm, politabicada, con origen en ovario derecho. Presenta un CEA preoperatorio de 50 y un CA-125 de 30. Se realizó laparotomía infraumbilical con anexectomía bilateral y apendicectomía. El resultado anátomo-patológico informa de cistoadenocarcinoma mucinoso de ovario bien diferenciado que infiltra casi toda la pared, abierta, sin alcanzar la cápsula ovárica; el ovario contralateral presenta un cistoade-noma mucinoso; el apéndice cecal presenta adenocarcinoma mucinoso bien diferenciado, con infiltración de casi toda la pared, respetando la serosa. Discusión: Presentamos el caso de una tumoración pélvica con diagnóstico anátomo-patológico de cistoadenocarcinoma mucinoso de ovario. Asimismo se demuestra afectación de apéndice cecal por adenocarcinoma mucinoso. Nos planteamos, por tanto, la posibilidad de que se trate de una infiltración metastásica o dos tumores sincrónicos. Revisamos el concepto de cistoadenocarcinoma apendicular y ovárico, y analizamos los posibles mecanismos de asociación entre ellos. Conclusión: Destacamos la necesidad de una exhaustiva revisión de la cavidad abdominal ante este tipo de patología, dada su asociación con otros procesos neoplásicos intraabdominales.

Bilateral diffuse mucinous cystic adenocarcinoma of the lungs complicated by recurrent pneumothorax in a pregnant woman

The incidence of lung cancer continues to rise among young females. The pulmonary mucinous cystic tumor is very rare with few reported cases and it is an uncommon histological type of primary lung adenocarcinoma. The cystic nature of this type of carcinoma makes it unique radiologically. We report a very rare case of bilateral diffuse mucinous cystic adenocarcinoma of the lungs in a young pregnant woman

Comparison of Efficacy of Enucleation and Pancreaticoduodenectomy for Small (<3 cm) Branch Duct Type Intraductal Papillary Mucinous Neoplasm Located at the Head of Pancreas and the Uncinate Process

PURPOSE: Accurate indications and the extent of surgery for branch duct intraductal papillary mucinous neoplasm (IPMN) of the pancreas are still debatable. In particular, small tumor is located at the head portion of pancreas presents a dilemma. The purpose of this study is to compare the efficacy of enucleation (EN) with that of pancreaticoduodenectomy (PD) in patients with small (2 cm

Adenocarcinoma mucoproductor de colon con infiltración de estómago y metástasis ováricas (tumor de Krukenberg) / Colon mucoproducing adenocarcinoma with stomach infiltration and ovarian metastases (Krukenberg's tumor)

Se presenta una paciente femenina de 29 años, operada de urgencia por presentar un gran tumor que incluía cuerpo gástrico y colon trasverso, con una perforación gástrica. Se realizó una gastrectomía subtotal con colectomía trasversa en bloque que incluyó el epiplón mayor. El diagnóstico histológico fue adenocarcinoma túbulo papilar mucoproductor de origen colónico, que infiltra hasta la serosa y pared gástrica. Se realizó tratamiento adyuvante con poliquimioterapia. Diez meses después presenta un tumor en hipogastrio, que al tacto vaginal, correspondía a los órganos genitales, sospecha clínica que confirman el ultrasonido abdominal y la tomografía axial computarizada. El hallazgo transoperatorio fueron tumores voluminosos de ambos ovarios, y otro tumor que afectaba la unión rectosigmoide. Se realizó una histerectomía radical con ooforectomía bilateral y sigmoidectomía, se reseca la porción proximal del recto, y se cierra tipo Hartman. El diagnóstico histológico final fue metástasis en serosa uterina e intestinal, y en ambos ovarios de adenocarcinoma mucoproductor, túbulo papilar de intestino previamente diagnosticado (tumor de Krukenberg). Se complementó el tratamiento con poliquimioterapia adyuvante(AU)

This is the case of a woman aged 29 operated on of emergency due to a tumor involving gastric body and transverse colon with gastric perforation. A subtotal gastrectomy with block transverse colectomy including the greater omentum was carried out. The histological diagnosis was a mucoproducing papillary tubular adenocarcinoma of colonic origin infiltrating to serosa and gastric wall. An adjuvant treatment was applied with poly-chemotherapy. Ten months later appears a hypogastric tumor which at vaginal manual examination corresponding to genital organs, clinical suspicion confirmed by abdominal ultrasound and computerized axial tomography. The transoperative findings were bulky tumors of both ovaries and another tumor involving the rectosigmoid joint. A radical hysterectomy with bilateral oophorectomy and sigmoidectomy was carried out with resection of proximal rectal portion with type Hartman closure. The final histological diagnosis was a metastasis in uterine and intestinal serosa and in both ovaries a mucoproducing adenocarcinoma, previous papillary tubule was also diagnosed (Krukenberg's tumor). Treatment was applied using adjuvant poly-chemotherapy(AU)