Value of direct immunohistochemical staining in assisting intraoperative frozen diagnosis of bronchiolar adenoma / 中华病理学杂志

Objective: To explore the feasibility and application value of intraoperative direct immunohistochemical (IHC) staining in improving the diagnosis accuracy in difficult cases of bronchiolar adenoma (BA). Methods: Nineteen cases with single or multiple pulmonary ground-glass nodules or solid nodules indicated by imaging in Cancer Hospital of Chinese Academy of Medical Sciences from January to July 2021 and with difficulty in differential diagnosis at frozen HE sections were selected. In the experimental group, direct IHC staining of cytokeratin 5/6 (CK5/6) and p63 was performed on frozen sections to assist the differentiation of BA from in situ/micro-invasive adenocarcinoma/adenocarcinoma/invasive mucinous adenocarcinoma. In the control group, two pathologists performed routine frozen HE section diagnosis on these 19 cases. The diagnostic results of paraffin sections were used as the gold standard. The sensitivity and specificity of BA diagnosis, consistency with paraffin diagnosis and time used for frozen diagnosis were compared between the experimental group and the control group. Results: The basal cells of BA were highlighted by CK5/6 and p63 staining. There were no basal cells in the in situ/microinvasive adenocarcinoma/adenocarcinoma/invasive mucinous adenocarcinoma. In the experimental group, the sensitivity and specificity with aid of direct IHC staining for BA were 100% and 86.7%, respectively, and the Kappa value of frozen and paraffin diagnosis was 0.732, and these were significantly higher than those in the control group (P<0.05). The average time consumption in the experimental group (32.4 min) was only 7 min longer than that in the control group (25.4 min). Conclusions: Direct IHC staining can improve the accuracy of BA diagnosis intraoperatively and reduce the risk of misdiagnosis, but require significantly longer time. Thus frozen direct IHC staining should be restricted to cases with difficulty in differentiating benign from malignant diseases, especially when the surgical modalities differ based on the frozen diagnosis.

Absceso del psoas ilíaco secundario a un tumor de colon izquierdo fistulizado al retroperitoneo: caso clínico y análisis de la literatura / Iliopsoas abscess resulting from penetrating left colon cancer to the retroperitoneum: case report and literature review / Abscesso do iliopsoas secundário a tumor de cólon esquerdo fistulizado para o retroperitônio: caso clínico e análise da literatura

Los abscesos del psoas ilíaco secundarios a un tumor de colon fistulizado son excepcionales y potencialmente graves. La mayoría son adenocarcinomas de tipo mucinoso. Su tratamiento es complejo ya que, para lograr una resección oncológica pretendidamente curativa, es necesario realizar una resección ampliada con mayor morbimortalidad. Presentamos el caso de una paciente con un adenocarcinoma mucinoso de colon izquierdo fistulizado al músculo ilíaco y la pared anterolateral del abdomen en la que se realizó una resección multivisceral que incluyó el colon izquierdo, el músculo y la cresta ilíaca y parte de la pared anterolateral del abdomen.

Iliopsoas abscess secondary to perforation of colon cancer is an extremely rare and potentially life-threatening condition. Most tumors are mucinous adenocarcinomas. Its treatment its complex, as most patients need radical extended resections to achieve good oncological results, which are in turn, graved with higher morbidity and mortality. We present the case of a patient with a left colon mucinous adenocarcinoma penetrating to the iliopsoas muscle and the anterolateral abdominal wall that required a multivisceral resection including left colon, iliac muscle and crest and part of the anterolateral abdominal wall.

Abscessos do iliopsoas secundários a um tumor de cólon fistulizado são raros e potencialmente graves. A maioria são adenocarcinomas do tipo mucinoso. Seu tratamento é complexo, pois, para se obter uma ressecção oncológica supostamente curativa, é necessário realizar uma ressecção ampliada com maior morbimortalidade. Apresentamos o caso de um paciente com adenocarcinoma mucinoso de cólon esquerdo fistulizado para o músculo ilíaco e parede ântero-lateral do abdome no qual foi realizada ressecção multivisceral que incluiu cólon esquerdo, músculo e crista ilíaca e parte do a parede anterolateral do abdome.

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.

Histerectomia radical após parto cesáreo por adenocarcinoma de colo uterino: relato de caso / Radical hysterectomy after c-section in cervical adenocarcinoma: a case report

Mundialmente, o câncer de colo uterino ocupa o quarto lugar das neoplasias em mulheres, porém, nos países em desenvolvimento, as taxas de incidência superam de forma impactante os casos de países desenvolvidos. Apesar de ser um evento incomum durante a gestação, é cada vez mais observado, o que talvez possa ser atribuído às gestações programadas em idades mais avançadas. O caso descrito refere-se a uma paciente de 32 anos de idade, diagnosticada no terceiro trimestre da gestação com adenocarcinoma de origem endocervical com estadiamento anatomopatológico final (FIGO 2018) 1B2. Ela foi submetida à abordagem cirúrgica como tratamento inicial. Foi adotada como conduta a resolução da gestação, com boa vitalidade fetal, ao final da 34ª semana, após corticoterapia para maturação pulmonar fetal. Foi realizado parto cesariano seguido de histerectomia radical tipo C1 na classificação de Querleu e Morrow associado a linfadenectomia pélvica, no mesmo ato operatório.(AU)

Worldwide, cervical cancer ranks fourth in female cancers, but when assessing data from developing countries, incidence rates are significantly higher than in developed countries. Although it is an uncommon event during pregnancy, it is increasingly observed, which may perhaps be justified due to pregnancies postponed at older ages. The case described relates to a 32-year-old woman diagnosed in the third trimester of pregnancy with endocervical adenocarcinoma, whose final anatomopathological staging (FIGO 2018) was IB2. The same was submitted to the surgical approach as an initial treatment. It was adopted as a conduct, the resolution of pregnancy, with good fetal viability, at the end of the 34th week, after corticosteroid therapy for fetal lung maturation. The patient underwent cesarean section followed by radical type C1 hysterectomy in the classification of Querleu and Morrow associated with pelvic lymphadenectomy in the same surgery.(AU)

Clinicopathological analysis and surgical strategy of primary appendiceal neoplasms / 中华胃肠外科杂志

Objective: To investigate the clinicopathological features, surgical methods and prognosis of primary appendiceal neoplasms. Methods: A descriptive case series study was performed. Clinical data of patients diagnosed with primary appendiceal neoplasms in Peking University First Hospital from 2006 to 2017 were retrospectively analyzed. Patients who underwent surgery and were confirmed as appendiceal neoplasms by postoperative pathology were included. Cases of cecal tumor invading the appendix and other organ tumors implanting in the appendix and cases of recurrent appendix tumors were excluded Pathological classification was based on the 4th edition of the WHO classification of digestive tract tumors (2010 edition), and the efficacy of operation methods of low grade appendiceal mucinous neoplasm (LAMN) were analyzed. Results: A total of 115 patients were enrolled, including 52 males and 63 females with a median age of 59 (51, 71) years. Clinical symptoms usually manifested as dormant pain in the right lower quadrant, migrating right lower abdominal pain, fever and bloating. Twenty-four cases were accidentally discovered during surgery, and 21 cases were found by physical examination. The preoperative diagnosis rate of CT and ultrasound was 40.2% (43/107) and 25.5% (24/94) respectively. The postoperative pathological types contained 83 cases of LAMN, 12 cases of mucinous adenocarcinoma, 9 cases of appendiceal neuroendocrine neoplasms (aNEN), 2 cases of mucinous adenocarcinoma with signet ring cells, 3 cases of serrated adenoma, 2 cases of goblet cell carcinoid, 2 cases of lymphoma, 1 case of leiomyoma and 1 case of schwannomas. All the patients underwent surgical resection, including 41 cases of appendectomy, 21 cases of partial cecectomy, 48 cases of right hemicolectomy, and 5 cases of combined organ resection due to appendiceal tumor infiltration or dissemination. Eighteen cases were diagnosed with pseudomyxoma peritonei (PMP) during operation. A total of 98 patients were enrolled for follow-up. The median follow-up time was 58 (5-172) months. The 5-year disease-free survival (DFS) rate was 84.5% and 5-year overall survival (OS) was 88.2%. Multivariate analysis revealed that high-grade malignancy tumors (HR=25.881, 95% CI: 2.827-236.935, P=0.004) and PMP formation (HR=42.166, 95% CI: 3.470-512.439,P=0.003) were independent risk factors for prognosis. Patients undergoing right hemicolectomy presented longer operation time, more blood loss and higher morbidity of complication as compared to those undergoing appendectomy and partial cecectomy (all P<0.05), while no significant differences in 5-year DFS (P=0.627) and 5-year OS (P=0.718) were found. Conclusions: Primary appendiceal neoplasms usually have no typical features, accompanied with low preoperative diagnosis rate. The common pathological types are LAMN, mucinous adenocarcinoma and aNEN. Appendectomy or partial cecectomy for LAMN may achieve satisfactory prognosis. High-grade malignancy tumors and PMP formation are independent risk factors for prognosis.

Simultaneous thoracoscopic approach in a patient with long-standing persistent atrial fibrillation and primary lung cancer: the first described case

Abstract Cardiac rhythm disorders are common in many patients with cancer. The management of synchronous long-standing persistent atrial fibrillation and pulmonary lesions remains a serious surgical dilemma due to the lack of clinical data and surgical guidelines. To the best of our knowledge, this is the first described case of simultaneous thoracoscopic pulmonary segmentectomy and left atrial posterior wall and pulmonary vein isolation combined with left atrial appendage resection in a patient with early-stage primary lung cancer and long-standing persistent atrial fibrillation.

Carcinoma mucinoso primario de piel / Primary mucinous skin carcinoma

El adenocarcinoma mucinoso primario de piel es una neoplasia anexial, maligna e infrecuente, que afecta principalmente a hombres en una relación 2:1 con respecto a las mujeres. Presenta mayor incidencia entre la sexta y séptima década de la vida, y se manifiesta como un tumor único, asintomático, de tamaño variable. La localización más frecuente es la región periorbitaria y el cuero cabelludo. Se comunica el caso de una paciente de 92 años, con diagnóstico de carcinoma mucinoso primario de piel, sin evidencia de enfermedad sistémica ni recurrencia local al año de la resección por cirugía micrográfica de Mohs. Describimos sus principales características clínicas, dermatoscópicas y hallazgos histopatológicos. (AU)

Primary mucinous adenocarcinoma of the skin is an adnexal, malignant, and infrequent neoplasm that mainly affects men with a 2: 1 ratio, with respect to women. It presents higher incidence between the sixth and seventh decade of life, and manifests as a single tumor, asymptomatic, of variable size. The most frequent location is the periorbital region and scalp. We report the case of a 92-year-old patient with a diagnosis of primary mucinous skin carcinoma, without evidence of systemic disease or local recurrence one year after resection by Mohs micrographic surgery. We describe its main clinical features, dermatoscopic and histopathological findings. (AU)

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.

Enterovesical fistula caused by ileal primary adenocarcinoma / Fístula enterovesical causada por adenocarcinoma ileal primário

ABSTRACT Introduction: Small bowel neoplasms are rare entities, with only 3.2% of gastrointestinal tumors localized in this segment. Adenocarcinoma is the second most common histologic type among small bowel neoplasms. The disease's symptoms are non-specific, with abdominal pain being the most common. Among the complications, fistulas are a far rare condition. Case report: We present the case of an 86-year-old woman, hypertensive, diabetic, and former smoker, with lower abdominal pain, hematuria, fecaluria and fever associated with weight loss. Upon physical examination presented pale mucous membranes. Magnetic resonance imaging of the abdomen showed parietal thickening in the distal ileum segment with an anterior wall bladder fistula. Absence of metastases. Subjected to transurethral endoscopic biopsy of the bladder lesion, which anatomopathological study was compatible with invasive mucinous adenocarcinoma. An enterectomy was performed with primary enteroanastomosis, associated with partial cystectomy by videolaparoscopy. Anatomopathological study of the surgical specimens concluded mucinous adenocarcinoma with signet ring cells located in the small bowel and bladder. Immunohistochemical exam has identified findings compatible with mucinous adenocarcinoma of origin in the small bowel. The patient evolved well, being discharged, and returned to postoperative follow-up without signs of relapse of the disease. Conclusion: The relative inaccessibility of the small bowel and the malignant neoplasms' non-specific symptoms make an early diagnosis difficult. Discovery of the disease, often only at an advanced stage, results in complications and less effective therapy. The laparoscopic approach might be advantageous and effective in the treatment of advanced small bowel cancer with invasion of adjacent structures.

RESUMO Introdução: As neoplasias do intestino delgado são entidades raras com apenas 3,2% dos tumores gastrointestinais localizando-se neste segmento. O adenocarcinoma é o segundo tipo histológico mais comum entre as neoplasias do intestino delgado. Os sintomas da doença são inespecíficos, sendo a dor abdominal o mais comum, dificultando o diagnóstico precoce. Dentre as complicações, as fistulas são de ocorrência ainda mais rara. Relato de caso: Mulher de 86 anos, hipertensa, diabética e ex-tabagista. Quadro de dor em abdome inferior, hematúria, fecalúria, febre eventual e perda ponderal. Ao exame físico pele e mucosas hipocoradas. Ressonância Magnética de abdome evidenciou espessamento parietal em segmento do íleo distal com fístula para a parede vesical anterior. Ausência de metástases. Submetida à biopsia endoscópica transuretral da lesão vesical, cujo estudo anatomopatológico evidenciou adenocarcinoma mucinoso invasivo. Realizada enterectomia com enteroanastomose primária, associada à cistectomia parcial por videolaparoscopia. Estudo anatomopatológico das peças cirúrgicas concluiu adenocarcinoma mucinoso com células em anel de sinete localizado em intestino delgado e bexiga. A Imunohistoquímica identificou achados compatíveis com adenocarcinoma mucinoso de origem em intestino delgado. A paciente evoluiu bem no pós-operatório, recebendo alta e retornando para seguimento pós-operatório sem sinais de recidiva da doença. Conclusão: A relativa inacessibilidade do intestino delgado e os sintomas inespecíficos das neoplasias malignas deste sítio dificulta o diagnóstico precoce. O reconhecimento da doença, muitas vezes somente em estágio avançado, resulta em complicações e sequelas com terapêutica menos eficaz. A abordagem laparoscópica pode ser vantajosa no tratamento do câncer avançado do intestino delgado com invasão de estruturas adjacentes.

Renal mucinous tubular and spindle cell carcinoma combined with multiple bone metastasis: a case report and literature review / 北京大学学报(医学版)

Mucinous tubular and spindle cell carcinoma (MTSCC) is a rare sub-type of renal cell carcinoma (RCC). It has been considered to be a kind of "indolent" tumor with low-grade fashion, weak invasive capacity and relatively favorable prognosis. However, in the current case, a 3.7 cm×2.8 cm spherical mass with contrast enhancement was found in the left kidney incidentally by computed tomography (CT) in a 60-year-old male patient. A lesion in the right humerus (2.1 cm×1.6 cm×3.1 cm) was found at the same time without any symptoms or sign of pathological fracture by magnetic resonance (MR) imaging. Further positron emission tomography (PET)/CT scan which was ordered immediately after admission suggested multiple bone destruction including skull, pelvis, sternum, right humerus and femur, left scapula, multiple vertebrae and libs. Pathological examination after radical nephrectomy and palliative resection with internal fixation of the lesion in the right humerus indicated that both renal (3.0 cm×3.0 cm×2.5 cm) and bone lesions were MTSCC with the features of high-grade ovoid epithelioid cells, cord-like spindle cells and mucinous matrix under light microscope. The diagnosis of renal MTSCC concurrent with multiple bone metastasis was made. This case report suggested the necessity of general evaluation, especially bone scan for possible distant metastasis, as MTSCC might present unexpected advanced behaviors without any orthopedic symptoms. The behavior of bone metastasis might be associated with male and elderly age. MTSCC has similar enhancement features to papillary RCC on CT scan. As results, attentions are needed to differentiate MTSCC from papillary RCC as they both tend to show lesser enhancement degrees than cortex. Rather than exhibiting a dedifferentiating appearance, the pathological characteristics of bone metastasis lesion were close to those of primary renal lesion. The reason of distant metastasis to the bone remained unclear, negative expression of cytokeratin (CK) 7 might be attributed to. Though immunotherapy, chemotherapy and target therapy could all be methods for systematic therapies, procedures to remove renal lesions and prevent skeletal related events are still highly recommended.

Neoplasias mucinosas papilares intraductales de páncreas en el Hospital Nacional Guillermo Almenara Irigoyen, Lima, Perú / Intraductal papillary mucinous neoplasms of the pancreas in Hospital Nacional Guillermo Almenara Irigoyen, Lima, Peru

Objetivo: Analizar en forma retrospectiva las características clínicas, radiológicas e histopatológicas, así como los resultados quirúrgicos de los pacientes con diagnóstico de neoplasia mucinosa papilar intraductal de páncreas (NMPI). Materiales y métodos: Usando la base de datos del servicio de Cirugía de Páncreas, Bazo y retroperitoneo del Hospital Nacional Guillermo Almenara Irigoyen (HNGAI) se identificaron a los pacientes con diagnóstico de NMPI sometidos a resección pancreática desde enero del 2006 a setiembre del 2013. Resultados: Se identificaron 11 pacientes (9 mujeres, 2 varones) con una edad promedio de 67,36 años. 2 casos fueron tipo conducto principal, 3 tipo rama y 6 tipo mixta. Todos los pacientes fueron sintomáticos, siendo el dolor el síntoma más frecuente, 27% presentaron pancreatitis. Para el diagnóstico 100% contaron con tomografía, 90% con resonancia magnética. En 10 pacientes se realizó una duodenopancreatectomía y en uno una pancreatectomía distal. Dos pacientes tuvieron fístula pancreática tipo C. Se reoperaron dos pacientes y hubo un fallecido. En 5 pacientes se observó displasia de bajo grado, en 2 displasia de moderado grado, en 1 displasia de alto grado y en 3 carcinoma invasor. Conclusiones: Esta patología está presente en nuestro medio, siendo más frecuente en mujeres. Las NMPI de tipo mixta son las de mayor frecuencia. La fístula pancreática es la complicación de mayor incidencia y la mortalidad es baja. El potencial de malignidad encontrado fue alto.

Objective: To analyze retrospectively the clinical, radiologic and pathologic features as well as the surgical results of the patients with diagnosis of intraductal papillary mucinous neoplasms of the pancreas (IPMN). Materials and methods: Patients with diagnosis of IPMN who underwent pancreatectomy were identified from January 2006 to September 2013, using the prospective data base of the Pancreas, Spleen and retroperitoneum HNGAI service. Results: A total of 11 patients were found (9 females and 2 males). The mean age was 67.36 years. 2 were main duct type IPMN, 3 branch type and 6 mixed type. All patients had symptoms. The pain was the most frequent symptom, 27% had pancreatitis. For the diagnosis 100% had CT scan, 90% MRI. 10 patients underwent pancreaticoduodenectomy and 1 distal pancreatectomy. 2 patients had type C pancreatic fistula. 2 patients underwent reoperation and 1 died. 5 patients had low grade dysplasia, 2 moderate dysplasia, 1 high grade dysplasia and 3 invasive carcinoma. Conclusions: This entity is present in our country being more common in women. The mixed type IPMN is the most frequent type. Complication with the highest incidence is the pancreatic fistula and the mortality rate is low. The IPMN´s malignancy potential founded was high.

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.

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.

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)

Carcinoma mucinoso da mama masculina- relato de caso / Mucinous breast cancer in men - case report

O câncer de mama no homem é pouco frequente entre 0,5% e 1% dos carcinomas mamários. O tipo histológico que predomina é o carcinoma ductal; outros tipos são raros. Os autores relatam o caso de um paciente com nódulo em mama esquerda de lenta evolução, com exames de imagem suspeitos e confirmação histológica de carcinoma mucinoso. Foi submetido à mastectomia com pesquisa de linfonodo sentinela e radioterapia adjuvante. O paciente está em uso de tamoxifeno e livre de doença há vinte e nove meses. A literatura demonstra que o homem pode apresentar quase todos os tipos histológicos de câncer de mama encontrados no sexo feminino, considerando-se a excepcionalidade do tipo histológico lobular. Com base nos artigos pesquisados, foram encontrados relatos de outros cinco casos de carcinoma mucinoso em mama masculina.

Neoplasia mucinosa intraductal del páncreas: [revisión] / Intraductal papillary mucinous neoplasm of the pancreas: [review]

Intraductal papillary mucinous neoplasm of the pancreas is characterized by a dilatation of the main pancreatic duct and/or secondary ducts, mucin production and the absence of ovarian ¡ike struma. The symptoms are non-specific and often the diagnosis is incidental. The treatment of choice is surgery, since these tumors may become malignant. The prognosis depends on the type of lesion, whether the excision is complete and lymph node involvement. The aim of this review is to analyze the clinical, diganostic, therapeutic and pathological characteristics of this disease.

Peritoneal pseudomyxoma associated with synchronic malignant mucinous neoplasias of the cecum, appendix and rectum: case report and review of the literature

Peritoneal pseudomyxoma is a pathological condition that compromises the peritoneum, characterized by the production of large quantities of mucinous liquid, which progressively fills the peritoneal cavity, originating in general from mucinous appendicular or ovarian tumors. We report a peritonial pseudomyxoma associated with mucinous adenocarcinoma of the appendix synchronic with adenocarcinoma of the rectum in 44 years old patient, where the initial diagnosis was rectal adenocarcinoma. Tumour of the appendix and peritonial pseudomyxoma were incidental and found intraoperativelly. We focus the histological patterns of the lesions, diagnosis and the treatment, with revision of the literature.

O pseudomixoma peritoneal é uma condição patológica que acomete o peritoneo, caracterizada pela produção de grandes quantidades de líquido mucinoso, que progressivamente preenche a cavidade peritoneal, tendo em geral como origem tumores mucinosos apendiculares ou de ovário. Relatamos a ocorrência de um pseudomixoma peritoneal associado a adenocarcinoma mucinoso do apêndice sincrônico e adenocarcinoma do reto em paciente de 44 anos, cujo diagnóstico inicial foi de adenocarcinoma do reto. A neoplasia do apêndice e o pseudomixoma peritonial foram achados incidentais, intra-operatórios. Enfocamos as principais características anatomo-patológicas das lesões, o diagnóstico e tratamento, através de ampla revisão da literatura.

Urothelial-Type adenocarcinoma of the prostate mimicking metastatic colorectal adenocarcinoma

Adenocarcinoma arising in urinary bladder or prostatic urethra is uncommon. When they occur, the tumor can be mistaken for metastatic lesions, especially from the colon. Here we report the fifth case of a primary urothelial-type adenocarcinoma arising in the prostate which showed enteric differentiation. The patient was a 55 year-old male whose prostatic needle core biopsy showed a high grade adenocarcinoma which was initially thought to be metastatic colon cancer. A follow-up colonoscopy was unremarkable. Subsequent prostatectomy revealed a high grade adenocarcinoma which was positive for cytokeratins 7 and 20, carcinoembryonic antigen, CDX2, and high molecular weight cytokeratin, and negative for prostate specific antigen, prostate specific acid phosphatase and AMACR. A diagnosis of urothelial-type adenocarcinoma of the prostate was rendered. We review the literature regarding this entity, and discuss the differential diagnosis, emphasizing utility of immunohistochemistry in making the diagnosis. Finally, we speculate on the behavior of these rare tumors.

Adenocarcinoma de apêndice: relato de dois casos e revisão da literatura / Adenocarcinoma of the appendix: two cases report and literature review

O adenocarcinoma apendicular é enfermidade rara, geralmente identificada com um quadro clínico de apendicite aguda. O diagnóstico pré-operatório é difícil e incomum. Objetivo - apresentar dois casos de adenocarcinoma apendicular, singulares por situarem-se em faixa etária jovem, associado à revisão de literatura / The appendicular adenocarcinoma is a rare disease, generally identifiedd as an acute appendicitis clinical presentation. Pre-operative diagnosis is extremely difficult and uncommon. Aim - to present two cases of appendicular adenocarcinoma in young people, and literature review...