Primary retroperitoneal tumor: mucinous cystoadenocarcinoma.

Primary retroperitoneal mucinous cystadenocarcinomas (PRMCs) are extremely rare tumors with limited understanding of their pathogenesis and biological behavior. We describe a case of a 50-year-old female patient who underwent surgical treatment. The patient had a history of previous surgeries for mesenteric mucinous cystadenoma, without evidence of recurrence. During routine abdominal ultrasound a new tumor was found. An abdomen magnetic resonance imaging was done and confirmed the presence of a cystic lesion in the right iliac fossa. After discussion in multidisciplinary committee, surgical complete resection of the tumor, along with bilateral adnexectomy, was performed successfully. Histopathological examination revealed a mucinous adenocarcinoma adjacent to a mucinous cystadenoma. Immunohistochemical analysis supported the diagnosis of a primary retroperitoneal lesion. The patient had an uneventful recovery and has remained disease-free during the two-year postoperative follow-up. PRMCs are challenging to diagnose preoperatively due to nonspecific symptoms. Surgical excision is the mainstay of treatment. The long-term prognosis and optimal therapeutic strategies require further investigation.

Los cistoadenocarcinomas mucinosos primarios retroperitoneales (CMPR) son tumores extremadamente raros con una comprensión limitada de su patogénesis y comportamiento biológico. Describimos el caso de una mujer de 50 años sometida a tratamiento quirúrgico. La paciente tenía antecedentes de cirugías previas por cistodenoma mucinoso mesentérico, sin evidencia de recurrencia. Durante una ecografía abdominal de rutina se encontró un nuevo tumor. Se realizó una resonancia magnética abdomen que confirmó la presencia de una lesión quística en la fosa ilíaca derecha. Luego de discutir el caso en el comité multidisciplinario, se realizó con éxito la resección quirúrgica completa del tumor, junto con la anexectomía bilateral. El examen histopatológico reveló un adenocarcinoma mucinoso adyacente a un cistodenoma mucinoso. El análisis inmunohistoquímico apoyó el diagnóstico de lesión primaria retroperitoneal. La paciente tuvo una buena recuperación y permaneció libre de enfermedad durante dos años de seguimiento postoperatorio. Los CMPR son difíciles de diagnosticar debido a que presentan síntomas inespecíficos. La escisión quirúrgica es la base del tratamiento. El pronóstico a largo plazo y las estrategias terapéuticas óptimas requieren más investigación.

Primary retroperitoneal tumor: mucinous cystoadenocarcinoma / Tumor primario retroperitoneal: cistoadenocarcinoma mucinoso

Abstract Primary retroperitoneal mucinous cystadenocarci nomas (PRMCs) are extremely rare tumors with limited understanding of their pathogenesis and biological be havior. We describe a case of a 50-year-old female patient who underwent surgical treatment. The patient had a history of previous surgeries for mesenteric mucinous cystadenoma, without evidence of recurrence. During routine abdominal ultrasound a new tumor was found. An abdomen magnetic resonance imaging was done and confirmed the presence of a cystic lesion in the right iliac fossa. After discussion in multidisciplinary committee, surgical complete resection of the tumor, along with bilateral adnexectomy, was performed successfully. Histopathological examination revealed a mucinous adenocarcinoma adjacent to a mucinous cystadenoma. Immunohistochemical analysis supported the diagnosis of a primary retroperitoneal lesion. The patient had an uneventful recovery and has remained disease-free du ring the two-year postoperative follow-up. PRMCs are challenging to diagnose preoperatively due to nonspecific symptoms. Surgical excision is the mainstay of treatment. The long-term prognosis and op timal therapeutic strategies require further investigation.

Resumen Los cistoadenocarcinomas mucinosos primarios re troperitoneales (CMPR) son tumores extremadamente raros con una comprensión limitada de su patogénesis y comportamiento biológico. Describimos el caso de una mujer de 50 años some tida a tratamiento quirúrgico. La paciente tenía antece dentes de cirugías previas por cistodenoma mucinoso mesentérico, sin evidencia de recurrencia. Durante una ecografía abdominal de rutina se encontró un nuevo tumor. Se realizó una resonancia magnética abdomen que confirmó la presencia de una lesión quística en la fosa ilíaca derecha. Luego de discutir el caso en el comité multidisci plinario, se realizó con éxito la resección quirúrgica completa del tumor, junto con la anexectomía bilateral. El examen histopatológico reveló un adenocarcinoma mucinoso adyacente a un cistodenoma mucinoso. El análisis inmunohistoquímico apoyó el diagnóstico de lesión primaria retroperitoneal. La paciente tuvo una buena recuperación y permaneció libre de enfermedad durante dos años de seguimiento postoperatorio. Los CMPR son difíciles de diagnosticar debido a que presentan síntomas inespecíficos. La escisión quirúrgica es la base del tratamiento. El pronóstico a largo plazo y las estrategias terapéuticas óptimas requieren más investigación.

Cistoadenocarcinoma biliar / Biliary Cystadenocarcinoma

Introducción: El cistoadenocarcinoma biliar es un tumor quístico poco frecuente, que se origina a partir del epitelio hepatobiliar, cuyo crecimiento es lento y sus síntomas son variables e inespecíficos. Objetivo: Presentar el caso de un paciente portador de un cistoadenocarcinoma biliar. Presentación del caso: Se presenta el caso de una paciente femenina de 56 años de edad que acude a la institución por presentar dolor en el hipocondrio derecho, fiebre y pérdida de peso. Al examen físico se constata una masa palpable en dicha zona abdominal, de bordes lisos, no dolorosa, dura, de escasa movilidad y relacionada con el hígado. Conclusiones: El cistoadenocarcinoma biliar es una neoplasia quística maligna poco frecuente del hígado. La mayoría de los pacientes presentan dolor abdominal, masa palpable y pérdida de peso. El tratamiento quirúrgico es la norma y la resección con márgenes libres se considera la variante de tratamiento más empleada(AU)

Introduction: Biliary cystadenocarcinoma is a rare cystic tumor, which originates from the hepatobiliary epithelium, whose growth is slow, and whose symptoms are variable and nonspecific. Objective: To present the case of a patient with a biliary cystadenocarcinoma. Case presentation: The case is presented of a 56-year-old female patient who came to our institution with pain in the right hypochondrium, fever and weight loss. Physical examination revealed a palpable mass in such abdominal area, with smooth edges, nonpainful, hard, with low mobility and related to the liver. Conclusions: Biliary cystadenocarcinoma is a rare malignant cystic neoplasm of the liver. Most patients present with abdominal pain, a palpable mass and weight loss. Surgery is the standard treatment and margin-free resection is considered the most commonly employed treatment variant(AU)

Social, Genetics and Histopathological Factors Related to Titin (TTN) Gene Mutation and Survival in Women with Ovarian Serous Cystadenocarcinoma: Bioinformatics Analysis.

Several factors may increase the risk of development of ovarian cancer. In this study, we investigated the relationship between social, genetic, and histopathologic factors in women with ovarian serous cystadenocarcinoma and titin (TTN) mutations, whether the TTN gene mutation may be a predictor, and its impact on mortality and survival in these patients. A total of 585 samples from patients with ovarian serous cystadenocarcinoma were collected from The Cancer Genome Atlas and PanCancer Atlas through the cBioPortal for analysis of social, genetic, and histopathological factors. Logistic regression was used to investigate whether TTN mutation could be a predictor, and the Kaplan-Meier method was applied to analyze survival time. TTN mutation frequency did not differ between age at diagnosis, tumor stage, and race, and was related to increased Buffa hypoxia score (p = 0.004), mutation count (p < 0.0001), Winter hypoxia Score (p = 0.030), nonsynonymous tumor mutation burden (TMB) (p < 0.0001), and reduced microsatellite instability sensor score (p = 0.010). The number of mutations (p < 0.0001) and winter hypoxia score (p = 0.008) were positively associated with TTN mutations, and nonsynonymous TMB (p < 0.0001) proved to be a predictor. Mutated TTN affects the score of genetic variables involved in cancer cell metabolism in ovarian cystadenocarcinoma.

Incidence of lymph node metastasis in early-stage low-grade serous ovarian cancer: a systematic review.

OBJECTIVE: The objective of this systematic review was to assess the incidence of lymph node metastasis in patients with clinically presumed early-stage low-grade serous ovarian cancer that underwent primary surgical treatment. METHODS: This study was registered in PROSPERO (CRD42022308923). A systematic literature review was conducted following the Meta-analyses Of Observational Studies in Epidemiology (MOOSE) checklist. PubMed/MEDLINE, EMBASE, Cochrane Central Register of Controlled Trials, Ovid, and Scopus databases were searched since inception and up to March 2022. The inclusion criteria were: pathological confirmation of low-grade serous ovarian cancer (clinically presumed FIGO 2014 stages I-IIA at time of surgery) that underwent primary surgical treatment, including pelvic and/or para-aortic lymph node dissection. RESULTS: The search identified 3763 articles; 59 were considered potentially eligible after removing duplicates, and eight studies finally met the selection criteria. In total, 35 of 277 (12.6%) patients had lymph node metastasis, and only four studies reported upstaging due to lymph node metastasis in 16 of 153 (10.5%) patients. None of the eight studies included reported the rate of complications or complications specifically for the subgroup of patients with early-stage low-grade tumors. CONCLUSION: In patients with early-stage low-grade serous ovarian cancer, lymph node assessment should be discussed when counseling for primary surgical staging.

Papillary cystadenocarcinoma of the parotid gland: a rare sub-variant of salivary gland adenocarcinoma.

Papillary cystadenocarcinoma of the salivary gland is a very rare malignant neoplasm accounting for only 2% of all salivary gland lesions. In 1991 it was first included as a separate entity in the World Health Organization (WHO) classification of salivary gland tumors and in 2017 WHO Classification, the tumor was clubbed as a sub-variant of adenocarcinoma, not otherwise specified. It most commonly occurs in the major salivary glands. Herein we report a case of salivary papillary cystadenocarcinoma in a 54-year-old female, who presented with rapid enlargement of the right parotid swelling. Based on radiology and fine-needle aspiration cytology, a working diagnosis of the malignant tumor involving the superficial lobe of the right parotid gland was made. In view of the malignant nature of the swelling, superficial parotidectomy was done. The histopathology and immunohistochemistry of the mass confirmed the diagnosis of papillary cystadenocarcinoma of the right parotid. With the revised 2017 WHO classification of salivary gland tumors, it is important to report all rare subtypes in order to understand their biology and behavior.

Pseudomixoma peritoneal primario de origen ovárico / Primary pseudomyxoma peritonei originated from ovaries

Resumen ANTECEDENTES: El pseudomixoma peritoneal es muy raro; se caracterizada por ascitis mucinosa e implantes peritoneales relacionados con la rotura y diseminación del contenido de un tumor mucinoso. En 80 al 90% de los casos el tumor primario es apendicular y el ovario es una localización realmente excepcional. CASO CLÍNICO: Paciente de 49 años que acudió a consulta debido a un dolor abdominal. En las pruebas de imagen se visualizó una tumoración anexial izquierda y se advirtieron hallazgos sugerentes de pseudomixoma peritoneal. En la cirugía, la masa ovárica se objetivó parcialmente fragmentada, con el apéndice aumentado de tamaño y extensos implantes peritoneales; además de gran cantidad de mucina libre en la cavidad peritoneal. El análisis anatomopatológico determinó la existencia de un adenocarcinoma mucinoso en el ovario afectado, con inmunohistoquímica positiva para CK7+ y CK20+, múltiples implantes de mucina y el apéndice sin daño. Por lo anterior se diagnosticó: pseudomixoma peritoneal de origen ovárico. Luego de dos intervenciones quirúrgicas no se consiguió la citorreducción completa. La paciente permaneció estable durante siete años, momento en el que fue evidente el avance de los síntomas de la enfermedad, circunstancia que la condujo a la muerte. CONCLUSIÓN: Determinar el origen de un pseudomixoma peritoneal sigue siendo un reto pues, con frecuencia, tanto el apéndice como los ovarios se afectan simultáneamente. Por ello, la apendicectomía y la exploración bilateral de los ovarios deben ser prácticas de rutina. El análisis extenso de las muestras y la inmunohistoquímica pueden facilitar la catalogación de estos infrecuentes tumores.

Abstract BACKGROUND: Pseudomyxoma peritonei is very rare; it is characterized by mucinous ascites and peritoneal implants related to rupture and dissemination of the contents of a mucinous tumor. In 80 to 90% of cases the primary tumor is appendicular and the ovary is a truly exceptional location. CLINICALCASE: A 49 year old female patient presented for consultation due to abdominal pain. Imaging tests showed a left adnexal tumor and findings suggestive of pseudomyxoma peritonei. At surgery, the ovarian mass was partially fragmented, with an enlarged appendix and extensive peritoneal implants; in addition to a large amount of free mucin in the peritoneal cavity. The anatomopathological analysis determined the existence of a mucinous adenocarcinoma in the affected ovary, with positive immunohistochemistry for CK7+ and CK20+, multiple mucin implants and an undamaged appendix. Therefore, a diagnosis was made: pseudomyxoma peritoneum of ovarian origin. After two surgical interventions she did not achieve complete cytoreduction. The patient remained stable for seven years, at which time the symptoms of the disease became evident and led to her death. CONCLUSION: Determining the origin of a pseudomyxoma peritonei remains a challenge as often both the appendix and ovaries are affected simultaneously. Therefore, appendectomy and bilateral ovarian exploration should be routine practice. Extensive specimen analysis and immunohistochemistry can facilitate cataloging of these infrequent tumors.

Papillary cystadenocarcinoma of the parotid gland: a rare sub-variant of salivary gland adenocarcinoma

Papillary cystadenocarcinoma of the salivary gland is a very rare malignant neoplasm accounting for only 2% of all salivary gland lesions. In 1991 it was first included as a separate entity in the World Health Organization (WHO) classification of salivary gland tumors and in 2017 WHO Classification, the tumor was clubbed as a sub-variant of adenocarcinoma, not otherwise specified. It most commonly occurs in the major salivary glands. Herein we report a case of salivary papillary cystadenocarcinoma in a 54-year-old female, who presented with rapid enlargement of the right parotid swelling. Based on radiology and fine-needle aspiration cytology, a working diagnosis of the malignant tumor involving the superficial lobe of the right parotid gland was made. In view of the malignant nature of the swelling, superficial parotidectomy was done. The histopathology and immunohistochemistry of the mass confirmed the diagnosis of papillary cystadenocarcinoma of the right parotid. With the revised 2017 WHO classification of salivary gland tumors, it is important to report all rare subtypes in order to understand their biology and behavior.

Clinical-epidemiological, anatomic-pathological, histochemical and immunohistochemical characterization of renal cystadenocarcinoma-nodular dermatofibrosis syndrome in 11 German Shepherd dogs / Caracterização clínico-epidemiológica, anátomo-patológica, histoquímica e imuno-histoquímica da síndrome cistadenocarcinoma-dermatofibrose nodular em 11 cães Pastor Alemão

Eleven cases of renal cystadenoma/cystadenocarcinoma-nodular dermatofibrosis syndrome (RCND) are described in German Shepherd dogs diagnosed from January 1994 to January 2018 at the Veterinary Pathology Laboratory of the "Universidade Federal de Santa Maria" (LPV-UFSM). The study sample was composed of eight male and three female dogs at a ratio of 2.67:1. Age ranged from six to 12 years (mean=8.7 years). The main clinical signs reported in descending order of frequency were multiple cutaneous nodules (nodular dermatofibrosis), dyspnea, anorexia, weight loss, recurrent hematuria, vomiting, and polydipsia. Results demonstrated that it is not always easy to clinically recognize this syndrome, but its peculiar anatomical-pathological characteristics allow safe diagnosis. Histologically, it was possible to detect all phases (cysts, papillary intratubular hyperplasia, and cystadenomas or cystadenocarcinomas) of a possible pathological continuum of the renal lesions. Uterine leiomyomas were observed in only one of the cases. Through histochemical techniques, it was possible to identify the presence of type I collagen in both cutaneous and renal lesions and consider its possible involvement in the pathogenesis of renal cystadenocarcinoma. Immunohistochemistry (IHC) showed partially satisfactory results in the staining of epithelial cells of renal cysts and neoplasms for pan-cytokeratin.(AU)

São descritos 11 casos da síndrome cistadenoma/cistadenocarcinoma-dermatofibrose nodular (CR-DN) em cães Pastor Alemão, diagnosticados entre janeiro de 1994 e janeiro de 2018 no Laboratório de Patologia Veterinária da Universidade Federal de Santa Maria (LPV-UFSM). Os cães afetados foram oito machos e três fêmeas, estabelecendo-se uma relação de 2,67:1. A idade variou de seis a 12 anos, sendo a média de idade de 8,7 anos. Os principais sinais clínicos relatados foram, em ordem decrescente de frequência, múltiplos nódulos cutâneos (dermatofibrose nodular), dispneia, anorexia, emagrecimento, hematúria recorrente, vômito e polidipsia. Este estudo permitiu estabelecer que o reconhecimento clínico da síndrome nem sempre é fácil, porém suas características anátomo-patológicas peculiares permitem um diagnóstico com segurança. Histologicamente, foi possível detectar todas as fases (cistos, hiperplasia intratubular papilífera, cistadenomas ou cistadenocarcinomas) de um possível continuum patológico das lesões renais. Leiomiomas uterinos foram observados somente em um caso. Através das técnicas histoquímicas foi possível estabelecer que o colágeno tipo I está presente em ambas as lesões, cutâneas e renais, e cogitar seu possível envolvimento na patogênese dos cistadenocarcinomas renais. A técnica de IHQ mostrou resultados parcialmente satisfatórios na imunomarcação das células epiteliais dos cistos e dos neoplasmas renais para pancitoceratina.(AU)

Clinical-epidemiological, anatomic-pathological, histochemical and immunohistochemical characterization of renal cystadenocarcinoma-nodular dermatofibrosis syndrome in 11 German Shepherd dogs / Caracterização clínico-epidemiológica, anátomo-patológica, histoquímica e imuno-histoquímica da síndrome cistadenocarcinoma-dermatofibrose nodular em 11 cães Pastor Alemão

Eleven cases of renal cystadenoma/cystadenocarcinoma-nodular dermatofibrosis syndrome (RCND) are described in German Shepherd dogs diagnosed from January 1994 to January 2018 at the Veterinary Pathology Laboratory of the "Universidade Federal de Santa Maria" (LPV-UFSM). The study sample was composed of eight male and three female dogs at a ratio of 2.67:1. Age ranged from six to 12 years (mean=8.7 years). The main clinical signs reported in descending order of frequency were multiple cutaneous nodules (nodular dermatofibrosis), dyspnea, anorexia, weight loss, recurrent hematuria, vomiting, and polydipsia. Results demonstrated that it is not always easy to clinically recognize this syndrome, but its peculiar anatomical-pathological characteristics allow safe diagnosis. Histologically, it was possible to detect all phases (cysts, papillary intratubular hyperplasia, and cystadenomas or cystadenocarcinomas) of a possible pathological continuum of the renal lesions. Uterine leiomyomas were observed in only one of the cases. Through histochemical techniques, it was possible to identify the presence of type I collagen in both cutaneous and renal lesions and consider its possible involvement in the pathogenesis of renal cystadenocarcinoma. Immunohistochemistry (IHC) showed partially satisfactory results in the staining of epithelial cells of renal cysts and neoplasms for pan-cytokeratin.(AU)

São descritos 11 casos da síndrome cistadenoma/cistadenocarcinoma-dermatofibrose nodular (CR-DN) em cães Pastor Alemão, diagnosticados entre janeiro de 1994 e janeiro de 2018 no Laboratório de Patologia Veterinária da Universidade Federal de Santa Maria (LPV-UFSM). Os cães afetados foram oito machos e três fêmeas, estabelecendo-se uma relação de 2,67:1. A idade variou de seis a 12 anos, sendo a média de idade de 8,7 anos. Os principais sinais clínicos relatados foram, em ordem decrescente de frequência, múltiplos nódulos cutâneos (dermatofibrose nodular), dispneia, anorexia, emagrecimento, hematúria recorrente, vômito e polidipsia. Este estudo permitiu estabelecer que o reconhecimento clínico da síndrome nem sempre é fácil, porém suas características anátomo-patológicas peculiares permitem um diagnóstico com segurança. Histologicamente, foi possível detectar todas as fases (cistos, hiperplasia intratubular papilífera, cistadenomas ou cistadenocarcinomas) de um possível continuum patológico das lesões renais. Leiomiomas uterinos foram observados somente em um caso. Através das técnicas histoquímicas foi possível estabelecer que o colágeno tipo I está presente em ambas as lesões, cutâneas e renais, e cogitar seu possível envolvimento na patogênese dos cistadenocarcinomas renais. A técnica de IHQ mostrou resultados parcialmente satisfatórios na imunomarcação das células epiteliais dos cistos e dos neoplasmas renais para pancitoceratina.(AU)

Cistoadenoma mucinoso hepático / Hepatic mucinous cystadenoma

El cistoadenoma hepático es una neoplasia muy rara, con menos de 250 casos reportados en la literatura mundial, que se da con mayor frecuencia en mujeres. Por la dificultad de diferenciar el quiste simple y el quiste hidatídico con el cistoadenoma hepático; así como su recidiva y posibilidad de displasia o carcinoma, si no es tratado mediante resección completa, es que presentamos el caso de una mujer de 56 años con historia de quiste hepático simple tratado en 2 oportunidades con destechamiento simple y que recidivaron, ya que se trataba de un cistoadenoma hepático.

Hepatic cystadenoma is a very rare neoplasm, with less than 250 cases reported in the world literature, which occurs more frequently in women. Due to the difficulty of differentiating the simple cyst and the hydatid cyst with the hepatic cystadenoma; as well as its recurrence and possibility of dysplasia or carcinoma, if it is not treated by complete resection, we present the case of a woman of 56 years old with history of simple hepatic cyst treated on 2 occasions with simple hepatic cyst unroofing and that they relapsed, so it was a hepatic cystadenoma.

Clinical-epidemiological, anatomic-pathological, histochemical and immunohistochemical characterization of renal cystadenocarcinoma-nodular dermatofibrosis syndrome in 11 German Shepherd dogs

ABSTRACT: Eleven cases of renal cystadenoma/cystadenocarcinoma-nodular dermatofibrosis syndrome (RCND) are described in German Shepherd dogs diagnosed from January 1994 to January 2018 at the Veterinary Pathology Laboratory of the Universidade Federal de Santa Maria (LPV-UFSM). The study sample was composed of eight male and three female dogs at a ratio of 2.67:1. Age ranged from six to 12 years (mean=8.7 years). The main clinical signs reported in descending order of frequency were multiple cutaneous nodules (nodular dermatofibrosis), dyspnea, anorexia, weight loss, recurrent hematuria, vomiting, and polydipsia. Results demonstrated that it is not always easy to clinically recognize this syndrome, but its peculiar anatomical-pathological characteristics allow safe diagnosis. Histologically, it was possible to detect all phases (cysts, papillary intratubular hyperplasia, and cystadenomas or cystadenocarcinomas) of a possible pathological continuum of the renal lesions. Uterine leiomyomas were observed in only one of the cases. Through histochemical techniques, it was possible to identify the presence of type I collagen in both cutaneous and renal lesions and consider its possible involvement in the pathogenesis of renal cystadenocarcinoma. Immunohistochemistry (IHC) showed partially satisfactory results in the staining of epithelial cells of renal cysts and neoplasms for pan-cytokeratin.

RESUMO: São descritos 11 casos da síndrome cistadenoma/cistadenocarcinoma-dermatofibrose nodular (CR-DN) em cães Pastor Alemão, diagnosticados entre janeiro de 1994 e janeiro de 2018 no Laboratório de Patologia Veterinária da Universidade Federal de Santa Maria (LPV-UFSM). Os cães afetados foram oito machos e três fêmeas, estabelecendo-se uma relação de 2,67:1. A idade variou de seis a 12 anos, sendo a média de idade de 8,7 anos. Os principais sinais clínicos relatados foram, em ordem decrescente de frequência, múltiplos nódulos cutâneos (dermatofibrose nodular), dispneia, anorexia, emagrecimento, hematúria recorrente, vômito e polidipsia. Este estudo permitiu estabelecer que o reconhecimento clínico da síndrome nem sempre é fácil, porém suas características anátomo-patológicas peculiares permitem um diagnóstico com segurança. Histologicamente, foi possível detectar todas as fases (cistos, hiperplasia intratubular papilífera, cistadenomas ou cistadenocarcinomas) de um possível continuum patológico das lesões renais. Leiomiomas uterinos foram observados somente em um caso. Através das técnicas histoquímicas foi possível estabelecer que o colágeno tipo I está presente em ambas as lesões, cutâneas e renais, e cogitar seu possível envolvimento na patogênese dos cistadenocarcinomas renais. A técnica de IHQ mostrou resultados parcialmente satisfatórios na imunomarcação das células epiteliais dos cistos e dos neoplasmas renais para pancitoceratina.

Neoplasias quísticas mucinosas del páncreas: caso clínico y revisión de la literatura / Clinical case of pancreatic mucinous cystic neoplasms and literature review

Resumen Las neoplasias quísticas mucinosas del páncreas son lesiones relativamente frecuentes, afectan principalmente a mujeres perimenopáusicas; la mayoría son únicos, localizados en el cuerpo y la cola del páncreas, y no tienen comunicación con el sistema ductal pancreático. Menos del 20% de los casos se asocian con malignidad. La evaluación debe incluir la presentación clínica, las imágenes, la utilización de la ultrasonografía endoscópica y toma de biopsias por punción, la citología y el análisis químico del líquido para la medición de niveles de antígeno carcinoembrionario (ACE). La resección quirúrgica completa es el único tratamiento que mejora la supervivencia a largo plazo en pacientes con lesiones quísticas mucinosas malignas. Se realiza una revisión de la literatura a propósito de un caso diagnosticado por el grupo de gastroenterología clínico-quirúrgica de la Clínica la Presentación, Manizales, Colombia.

Abstract Mucinous cystic neoplasms of the pancreas occur relatively frequently and mainly affect women in the transition to menopause. Most of these neoplasms are unique but are located in the body and tail of the pancreas and have no communication with the pancreatic ductal system. Less than 20% are malignant. Evaluation should include clinical presentation, imaging, endoscopic ultrasonography, puncture biopsies, cytology and chemical analysis of the liquid to measure angiotensin converting enzyme (ACE) levels. Complete surgical resection is the only treatment that can improve long-term survival in patients with malignant mucinous cystic lesions. This article includes a review of the literature related to presentation of a case diagnosed by the surgical clinical gastroenterology group at Clínica la Presentación in Manizales, Colombia.

WT1, p53 and p16 expression in the diagnosis of low- and high-grade serous ovarian carcinomas and their relation to prognosis.

OBJECTIVE: To evaluate the diagnostic and prognostic value of the immunohistochemical expression of WT1, p53 and p16 in low- (LGSOCs) and high-grade serous ovarian carcinomas (HGSOCs). RESULTS: HGSOC had a significantly higher proportion of advanced stage disease, higher CA125 levels, higher proportion of post-surgery residual disease and higher recurrence or disease progression. WT1 was expressed in 71.4% of LGSOCs and in 57.1% of HGSOCs (p = 0.32). Focal and/or complete absence of p53 expression with negative p16 expression was found in 90.5% of LGSOCs, in contrast to the 88.1% of HGSOCs with diffuse or complete absence of p53 expression with positive p16 expression (<0.001). The IHC p53/p16 index and the morphological classification were closely matched (k = 0.68). In the univariate analysis, FIGO stage, post-surgery residual disease and histological grade were significantly associated with progression-free survival (PFS) and overall survival (OS). The IHC p53/p16 index was associated only with PFS. WT1 was not associated with PFS or OS. According to the multivariate analysis, advanced FIGO stage and presence of post-surgery residual disease remained independent prognostic factors for worst PFS, however these features had only a trend association with OS. METHODS: 21 LGSOC and 85 HGSOC stage I-IV cases were included. The morphological classification was assessed according to the World Health Organization (WHO) criteria. Immunohistochemistry (IHC) was performed in tissue microarray slides. IHC p53/p16 index was compared with the morphological classification. CONCLUSIONS: The IHC p53/p16 index was a good marker for the differentiation of LGSOC and HGSOC, but the morphologic classification showed a better association with survival. FIGO stage and post-surgery residual disease remained the only independent prognostic factors for survival.

Papillary mucinous cystadenocarcinoma: rare malignant tumor in minor salivary gland / Cistadenocarcinoma papilar mucinoso: tumor maligno raro em glândula salivar menor

ABSTRACT Clinical case description of a papillary mucinous cystadenocarcinoma, extremely rare malignant tumor in the salivary gland and difficult to diagnose, in geriatric patient. Patient with 86 years old was referred for treatment with nodular lesion on the inner side of left lower lip extending to the bucal mucosa, without any clinical appearances of malignancy. The lesion was asymptomatic, had flabby consistency and slow growth. Despite the favorable clinical features, after surgical removal, was diagnosed as papillary mucinous papillary mucinous cystadenocarcinoma. Nonetheless, the cellular atypia found was considered to be mild and immunohistochemical evaluation the reaction to Ki-67 was low, indicating a low rate of proliferation of the tumor. Medical evaluation confirmed the absence of systemic disorders or distant metastases. Although it is a malignant lesion, due to low degree of proliferation of lesion and the careful follow-up carried out, there was a favorable evolution after conservative treatment in a follow-up period of 11 years and six months.

RESUMO Descrição do caso clínico de um cistadenocarcinoma papilar mucinoso, tumor maligno extremamente raro em glândula salivar e de difícil diagnóstico, em um paciente geriátrico. Paciente com 86 anos foi encaminhado para tratamento com lesão nodular na parte interna do lábio inferior do lado esquerdo estendendo-se para a mucosa jugal sem qualquer aspecto clínico de malignidade. A lesão era assintomática, apresentava consistência flácida e crescimento lento. Apesar das características clínicas favoráveis, após a remoção cirúrgica, o diagnóstico foi de cistadenocarcinoma papilar mucinoso. Entretanto, a atipia celular encontrada foi considerada leve e a reação imunohistoquímica para o Ki67 baixa, indicando uma baixa taxa de proliferação do tumor. A avaliação médica confirmou a ausência de transtornos sistêmicos e de metástases a distância. Apesar de se tratar de uma lesão maligna, devido ao baixo grau de proliferação do tumor e o acompanhamento cuidadosos realizado houve uma evolução favorável após o tratamento conservador após um período de acompanhamento de 11 anos e 6 meses.

Spontaneous rupture of ovarian cystadenocarcinoma: pre- and post-rupture computed tomography evaluation.

Epithelial ovarian tumors are the most common malignant ovarian neoplasms and, in most cases, eventual rupture of such tumors is associated with a surgical procedure. The authors report the case of a 54-year-old woman who presented with spontaneous rupture of ovarian cystadenocarcinoma documented by computed tomography, both before and after the event. In such cases, a post-rupture staging tends to be less favorable, compromising the prognosis.

Os tumores epiteliais correspondem à maioria das neoplasias ovarianas, sendo que eventuais rupturas estão mais relacionadas ao ato cirúrgico. Relatamos um caso de cistoadenocarcinoma ovariano em uma paciente de 54 anos com documentação tomográfica pré- e pós-ruptura espontânea. O estadiamento tumoral pós-ruptura tende a ser menos favorável, comprometendo o prognóstico desses pacientes.

Spontaneous rupture of ovarian cystadenocarcinoma: pre- and post-rupture computed tomography evaluation / Ruptura espontânea de um cistoadenocarcinoma ovariano: avaliação tomográfica prée pós-ruptura

AbstractEpithelial ovarian tumors are the most common malignant ovarian neoplasms and, in most cases, eventual rupture of such tumors is associated with a surgical procedure. The authors report the case of a 54-year-old woman who presented with spontaneous rupture of ovarian cystadenocarcinoma documented by computed tomography, both before and after the event. In such cases, a post-rupture staging tends to be less favorable, compromising the prognosis.

ResumoOs tumores epiteliais correspondem à maioria das neoplasias ovarianas, sendo que eventuais rupturas estão mais relacionadas ao ato cirúrgico. Relatamos um caso de cistoadenocarcinoma ovariano em uma paciente de 54 anos com documentação tomográfica pré- e pós-ruptura espontânea. O estadiamento tumoral pós-ruptura tende a ser menos favorável, comprometendo o prognóstico desses pacientes.

Tumores hepáticos malignos no hepatocelulares. 2.a parte. Aspectos morfológicos y estudios auxiliares de importancia útiles para el diagnóstico histopatológico / Malignant Hepatic Neoplasms. Part 2. Morphology and important ancillary studies useful for histopathological diagnosis

Se continúa con la revisión de los tumores malignos hepáticos primarios. Se hablará de los aspectos más importantes de los tumores primarios no hepatocelulares, siendo más frecuentes los originados en el epitelio del ducto biliar como el colangiocarcinoma, el cistoadenocarcinoma y los tumores mesenquimales, y los más infrecuentes el hemangioendotelioma epitelioide y el angiosarcoma. La principal dificultad radica en lograr el diagnóstico definitivo, el cual se basa en la exclusión de una neoplasia primaria extrahepática o de lesiones hepáticas benignas. Estudios adicionales de inmunohistoquímica, estudios de clonalidad o moleculares pueden ser de mucha utilidad.

We continue with the review of primary malignant liver tumors. This article covers the most important aspects of primary tumors that are not hepatocellular. Those that originate in the epithelium of the bile duct such as cholangiocarcinoma, cystadenocarcinoma and mesenchymal tumors occur most frequently. Epithelioid hemangioendothelioma and angiosarcoma occur less frequently. The main difficulty lies in making a definitive diagnosis which must be based on the exclusion of extrahepatic primary neoplasms and benign liver lesions. Additional immunohistochemistry and molecular studies as well as diagnostic cloning of cells can be very useful.

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.