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BACKGROUND: The presence of ovarian-type stroma defines mucinous cystic neoplasm (MCN). Criteria for surgical resection differ between current consensus guidelines (IAP, AGA, and Europe). This meta-analysis aims to describe pre-surgical clinical parameters that predict malignant transformation of MCN of the pancreas. METHODS: A systematic review and meta-analysis of articles published from 2006 to the time of manuscript authorship in December 2022. The electronic databases included English publications in Ovid MEDLINE In-Process & Other Non-Indexed Citations, Ovid MEDLINE, Ovid EMBASE, and Scopus. RESULTS: 17 studies were identified and included 1058 patients with MCN treated with pancreatectomy. The mean cohort age was 48.2 years (standard deviation [SD] ± 7.9) with an expected female predominance (96 %). The presenting symptom for most was abdominal pain (55.6 %), however, nearly 20 % of patients were asymptomatic. Most patients were treated with distal pancreatectomy (70.5 %), and the mean tumor size was 45 mm. The rate of invasive cancer was 13.8 %. Cysts with mural nodules had a higher risk of developing invasive tumors than those that did not (OR 26.47, 95%CI 12.57-55.74, p < 0.001, I2:0 %). Other clinical factors such as the presence of intramural calcifications or an elevated serum CA 19-9 (>37U/mL) were not predictive of malignancy. CONCLUSION: The present meta-analysis did not clarify establishing reliable predictors for malignant transformation other than mural modularity, which may represent tumors that have already undergone transformation. It may be used as a criterion in treatment decision-making.

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Breast malignancy in men is an exceedingly rare condition, representing a small fraction of all diagnosed breast cancer cases. The most common histological subtype is invasive ductal carcinoma, while the mucinous type is extremely rare. This pathology has a high mortality rate due to its poor prognosis and diagnosis in advanced stages, often initially overlooked with limited screening. Surprisingly, more men have died from breast cancer than from testicular cancer. This report details a case of invasive mucinous carcinoma in a 75-year-old male who presented with a five-week history of chronic non-productive cough and signs of pleural effusion. A breast magnetic resonance imaging (MRI) revealed a retroareolar breast tumor, and a second-look ultrasound confirmed the presence of a BI-RADS 4C solid nodule. Histopathological and immunohistochemical results were confirmed by ultrasound-guided tru-cut biopsy, identifying invasive mucinous carcinoma and luminal B (HER2+) subtype. Staging studies were negative for metastasis, and a modified radical mastectomy was performed, yielding favorable intraoperative findings. The incidental diagnosis in this patient highlights the necessity of comprehensive imaging in atypical presentations. Despite its rarity, awareness and early detection of mucinous carcinoma are essential for optimizing patient outcomes. This case also underscores the disparity in breast cancer outcomes between low gross domestic product (GDP) and high-GDP countries, emphasizing the need for improved access to diagnostic and therapeutic resources. Enhanced clinical awareness and early detection are crucial for improving outcomes in patients with rare histological subtypes, particularly in underserved regions.

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Background: Intraductal papillary mucinous neoplasm (IPMN)-associated pancreatic cancer is becoming a common subtype of pancreatic cancer found in resected specimens. The prognostic of this subtype is still under evaluation. The study aims to evaluate the prognosis of IPMN-associated pancreatic adenocarcinoma compared to the conventional pancreatic adenocarcinoma. Methods: In this study, patients with resected pancreatic neoplasms and IPMN treated at Hospital Israelita Albert Einstein, from January 2016 to December 2020, were analyzed. Overall survival (OS) was estimated using the Kaplan-Meier method, and correlations between the variables of interest and the disease specific OS was assessed by multivariate analysis. Results: Of 187 patients undergoing resection for pancreatic adenocarcinoma or IPMN, 125 (67%) had pancreatic adenocarcinoma, 33 (18%) had IPMN-associated pancreatic adenocarcinoma, and 29 (16%) had IPMN. Resected IPMN was associated with long-term OS for most of the patients. Similar OS was identified in this study in upfront resected pancreatic cancer associated or not with IPMN. No statistical differences in median OS were identified between resected pancreatic adenocarcinoma and IPMN-associated pancreatic adenocarcinoma (48 vs. 44 months, P=0.44). Size of the tumor [hazard ratio (HR), 1.33], resected stage III (HR, 1.31), perineural invasion (HR, 1.58), lymphovascular invasion (HR, 1.44), positive lymph nodes (HR, 1.34), and neoadjuvant treatment (HR, 1.70) were associated with worse outcomes. Conclusions: Our findings confirm that resected pancreatic cancer has a poor prognosis and IPMN-associated pancreatic adenocarcinoma has the same prognosis as a conventional pancreatic adenocarcinoma. More than half of the cases of IPMN-associated adenocarcinoma already had positive lymph nodes. The impact of neoadjuvant treatment in this group of patients should be investigated in larger cohorts.

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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.

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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.

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BACKGROUND: Salivary gland cystadenoma (SGCA) is a rare benign tumor that predominantly occurs in the parotid gland. SGCAs affecting the minor salivary glands are uncommon and often resemble, clinically and histopathologically, other salivary gland lesions. METHODS: This study aimed to describe a series of four cases of SGCA affecting intraoral sites and performed a literature review of well-reported SGCA published in the English-language literature. RESULTS: SGCA cases included in this series were diagnosed in the buccal mucosa, lip, and hard palate of female patients aged between 19 and 78 years. All cases underwent excisional biopsy and were histologically characterized by a multicystic growth with variable degrees of capsule formation and were lined by several types of epithelium, including some cell types that are infrequently reported in SGCA. In some cases, a small collection of lymphocytes was observed adjacent to cystic formations. All SGCA were positive for periodic acid-Schiff, and immunohistochemical reactions were positive for CK7 and p63. The follow-up time ranged widely from 3 to 53 months, and to date, no recurrence has been observed. CONCLUSION: The literature review revealed a total of 33 published studies accounting for 55 SGCA cases.

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Resumen El pseudomixoma peritoneal (PMP) es una afección poco común, inicialmente descrita en 1884 por Werth en relación con la ascitis y tumores mucinosos de ovario, y posteriormente en 1901 por Frankel, asociado a tumores mucinosos apendiculares. Se ha observado una alta prevalencia de mutaciones en el gen K-RAS y TP53 en pacientes con PMP de bajo grado, lo que desencadena la proliferación y producción excesiva de moco. Los estudios han demostrado que la cavidad peritoneal, especialmente la superficie hepática, es el sitio principal de depósito de estos tumores. La tomografía computada se considera el estándar de oro para el diagnóstico, mientras que la resonancia magnética es más sensible para detectar el origen tumoral y evaluar la extensión de la enfermedad. Aunque la laparotomía exploratoria es el método tradicional para la toma de biopsias, se están explorando alternativas menos invasivas como la biopsia guiada por ultrasonido y tomografía computarizada, que han demostrado ser eficaces. El diagnóstico diferencial incluye la endometriosis y tumores mixoides, con énfasis en la invasión al mesenterio y las características quísticas. Es crucial reconocer las diferencias en etapas avanzadas, ya que el PMP tiende a invadir los órganos desde afuera, mientras que los tumores mixoides presentan metástasis sólidas a distancia.

Abstract Pseudomyxoma peritonei (PMP) is a rare condition, first described by Werth in 1884 in association with ascites and ovarian mucinous tumors, and later in 1901 by Frankel, associated with appendicular mucinous tumors. High prevalence of mutations in the K-RAS and TP53 genes has been observed in patients with low-grade PMP, triggering proliferation and excessive mucus production. Studies have shown that the peritoneal cavity, especially the hepatic surface, is the main site for deposition of these tumors. Computed tomography is considered the gold standard for diagnosis, while magnetic resonance imaging is more sensitive for detecting the tumor origin and assessing disease extent. Although exploratory laparotomy is the traditional method for biopsy, less invasive alternatives such as ultrasound-guided and computed tomography-guided biopsy are being explored, which have proven to be effective. The differential diagnosis includes endometriosis and mixoid tumors, with emphasis on mesentery invasion and cystic characteristics. Recognizing differences in advanced stages is crucial, as PMP tends to invade organs from the outside, while mixoid tumors present distant solid metastases.

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Aunque el cistoadenoma mucinoso de ovario tiene un crecimiento benigno, puede crecer hasta tener grandes dimensiones. Se presenta el caso de una paciente de 70 años que acudió a consulta por presentar dolor abdominal recurrente y difuso. A la exploración física se palpó una gran masa que ocupaba toda la cavidad abdominal, hasta la apófisis xifoides, redondeada, renitente, de bordes definidos, no dolorosa, no movible. Presencia de edema en miembros inferiores. Durante el acto operatorio se identificó útero miomatoso y quiste gigante de ovario izquierdo, aproximadamente de 40 x 30 cm y 13,6 kg de peso. Se realizó histerectomía total abdominal y salpingooforectomía bilateral. La biopsia confirmó el diagnóstico de cistoadenoma mucinoso benigno de ovario y leiomiomatosis uterina. El posoperatorio transcurrió favorablemente. La incidencia de cistoadenoma gigante de ovario es desconocida, debido a la falta de un concepto estandarizado y las modalidades de imagen avanzadas disponibles en la actualidad(AU)

Although ovarian mucinous cystadenoma has a benign growth, it can grow to be very large. We present the case of a 70-year-old patient who came to the clinic for recurrent and diffuse abdominal pain. On physical examination, a large mass was palpated that occupied the entire abdominal cavity, up to the xiphoid appendix, rounded, retentive, with defined edges, non- painful, non-movable. Presence of edema in lower limbs. During the surgical act, a myomatous uterus and a giant left ovarian cyst measuring approximately 40 x3 0 cm and weighing 13.6 kg were identified. Total abdominal hysterectomy and bilateral salpingo-oophorectomy were performed. The biopsy confirmed the diagnosis of benign ovarian mucinous cystadenoma and uterine leiomyomatosis. The postoperative period progressed favorably. The incidence of giant ovarian cystadenoma is relatively unknown due to the lack of a standardized concept and currently available advanced imaging modalities(AU)

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Desde los primeros reportes en la bibliografía, la nomenclatura de las lesiones quísticas hepatobiliares se ha ido modificando, habiéndose descripto dos tipos de lesiones: las serosas y las mucinosas. En 2010 la Organización Mundial de la Salud estableció una nueva clasificación donde los términos cistoadenomas y cistoadenocarcinomas hepatobiliares son reemplazados por entidades más específicas como la neoplasia mucinosa quística y los tumores quísticos intraductales (neoplasia papilar intraductal, neoplasma tubulopapilar intraductal y neoplasma oncocitico papilar). En cuanto a la neoplasia mucinosa quística, la presencia de estroma ovárico le confiere características distintivas en lo patológico y biológico, siendo esto un requisito en la clasificación de la OMS. Esta característica lo diferencia de los hamartomas biliares, los quistes congénitos y la enfermedad de Caroli. Dichas neoplasias son infrecuentes, con una incidencia menor al 5% de las lesiones quísticas hepáticas y ocurren casi exclusivamente en mujeres, frecuentemente perimenopáusicas. Su potencial de malignización ha sido descrito, siendo éste la indicación de tratamiento quirúrgico resectivo. Presentamos el caso clínico de una paciente portadora de una neoplasia quística mucinosa hepática, catalogada como cistoadenoma hepático según la antigua clasificación.

Since the early reports in the literature, the nomenclature of hepatobiliary cystic lesions has been modified, with two types of lesions being described: serous and mucinous. In 2010, the World Health Organization established a new classification in which the terms hepatobiliary cystadenomas and cystadenocarcinomas were replaced by more specific entities such as mucinous cystic neoplasms and intraductal cystic tumors (intraductal papillary neoplasm, intraductal tubulopapillary neoplasm, and intraductal oncocytic papillary neoplasm). Regarding mucinous cystic neoplasms, the presence of ovarian stroma confers distinctive pathological and biological characteristics, which is a requirement in the WHO classification. This characteristic differentiates it from biliary hamartomas, congenital cysts, and Caroli's disease. Such neoplasms are rare, with an incidence of less than 5% of hepatic cystic lesions, and occur almost exclusively in women, often perimenopausal. Their potential for malignancy has been described, and this is the indication for surgical resection treatment. We present a clinical case of a patient with a mucinous cystic hepatic neoplasm, classified as a hepatic cystadenoma according to the old classification.

Desde os primeiros relatos na literatura, a nomenclatura das lesões císticas hepatobiliares tem sido modificada, sendo descritos dois tipos de lesões,asserosas e as mucinosas. Em 2010, a Organização Mundial da Saúdeestabeleceuuma nova classificação, naqual os termos cistoadenomas e cistoadenocarcinomas hepatobiliares foramsubstituídos por entidades mais específicas, como a neoplasia mucinosa cística e os tumores císticos intraductais (neoplasia papilar intraductal, neoplasma tubulopapilar intraductal e neoplasma oncocítico papilar). Em relação à neoplasia mucinosa cística, a presença de estroma ovarianoconfere características distintas do ponto de vista patológico e biológico, sendoesseum requisito naclassificação da OMS. Essa característica a diferencia dos hamartomas biliares, cistoscongênitos e doença de Caroli. Essas neoplasias são raras, comumaincidência menor que 5% das lesões císticas hepáticas, e ocorremquase exclusivamente em mulheres, frequentementeperimenopáusicas. Seu potencial de malignizaçãotem sido descrito, sendoesta a indicação para tratamentocirúrgicoressectivo. Apresentamos o caso clínico de uma paciente portadora de uma neoplasia cística mucinosa hepática, classificada como cistoadenoma hepático de acordocom a antigaclassificação.

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Appendiceal mucinous lesions' classification and nomenclature has been modified several times along the last decades, reflecting their great heterogeneity and making difficult to compare results and draw conclusions. Despite its nearby origin, appendiceal mucinous lesions have a distinctive behaviour compared to colorectal cancer, including their molecular and genetic markers. Due to their low frequency, their management is not well standardised. However, surgery is considered the cornerstone of treatment. Their indolent behaviour has encouraged surgeons to apply more aggressive treatments, such as cytoreductive surgery (CRS) and heated intraperitoneal chemotherapy (HIPEC), that may extend overall survival. Chemotherapy is reserved for unresectable and/or disseminated disease and could play a role in the adjuvant and neoadjuvant setting. Pressurised intraperitoneal aerosol chemotherapy (PIPAC) is recently emerging as a possible alternative for treatment in advanced disease although its results in long-term survival are lacking Hereby, we review the available evidence in the management of appendiceal mucinous lesions, including localised and disseminated disease, with a special emphasis on the oncological perspective, focusing on the lights and shadows of the systemic treatments.

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Resumen Caso clínico sobre un paciente masculino, 35 años, deportista, conocido sano, que presenta una historia clínica con síntomas gastrointestinales indeterminados, a quien se le realizan estudios complementarios, detectándose una lesión fusiforme, de apariencia quística, con contenido seroso, de aproximadamente 9cm, perteneciente al apéndice ileocecal. Inicialmente, debido a los hallazgos, en los estudios realizados a nuestro paciente, se sospecha de mucocele apendicular. Según la teoría el mucocele apendicular se origina de una obstrucción del apéndice ileocecal, lo cual provoca una acumulación retrograda de material mucinoso y eso ocasiona una dilatación de la luz apendicular, convirtiéndose en una masa quística. Con dicho diagnóstico presuntivo, se realiza apendicectomía con hemicolectomía derecha. El abordaje quirúrgico que se decide para este caso es la cirugía abierta versus laparoscópica, debido al riesgo de cistoadenocarcinoma mucinoso. Posteriormente se envía la lesión para realización de estudio patológico, donde se evidencia una neoplasia mucinosa de bajo grado del apéndice.

Abstract Clinical case of a 35-year-old male patient, an athlete, known to be healthy, who presented a clinical history with indeterminate gastrointestinal symptoms, who underwent complementary studies, detecting a fusiform lesion with a cystic appearance, with serous content, approximately 9cm, belonging to the ileocecal appendix. Initially, due to the findings in the studies carried out on our patient, an appendiceal mucocele was suspected. According to the theory, the appendicular mucocele originates from an obstruction of the ileocecal appendix, which causes a retrograde accumulation of mucinous material and this causes a dilation of the appendicular lumen, becoming a cystic mass. With this presumptive diagnosis, appendectomy with right hemicolectomy was performed. The surgical approach chosen for this case is open versus laparoscopic surgery, due to the risk of mucinous cystadenocarcinoma. Subsequently, the lesion is sent for a pathological study, where a low-grade mucinous neoplasm of the appendix is evidenced.

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BACKGROUND: Non-operative management has been suggested as a therapy for uncomplicated appendicitis. Notwithstanding, the risk of missing an appendiceal tumor must be considered, being the surgical piece crucial to rule out neoplasms. Therefore, we aim to determine the incidence of appendiceal neoplasms in patients with acute appendicitis, tumor types and the importance of the anatomopathological study of the surgical piece. STUDY DESIGN: Retrospective study in which we described patients who underwent emergent appendectomy with histopathological findings of appendiceal neoplasms from January 2012 to September 2018. Descriptive analysis included demographic variables, diagnostic methods, and surgical techniques. RESULTS: 2993 patients diagnosed with acute appendicitis who underwent an emergency appendectomy. 64 neoplasms of the appendix were found with an incidence of 2,14%. 67.2% were women, the mean age was 46,4 years (± 19.5). The most frequent appendiceal neoplasms were neuroendocrine tumors (42,2%), followed by appendiceal mucinous neoplasms (35,9%), sessile serrated adenomas (18,8%), and adenocarcinomas (3,1%). In 89,1% of the cases, acute appendicitis was determined by imaging, and 14% of cases were suspected intraoperatively. Appendectomy was performed in 78,1% without additional procedures. CONCLUSIONS: Appendiceal tumors are rare and must be ruled out in patients with suspected acute appendicitis. The incidence of incidental neoplasms is higher in this study than in the previously reported series. This information must be included in decision-making when considering treatment options for acute appendicitis.

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Introduction: Primary carcinoma of the duodenum is an infrequent malignancy; it represents 0.3-0.5% of all carcinomas of the GI tract and 33-45% of all carcinomas of the small intestine. Clinical case: A 57-year-old female patient with nonspecific GI symptoms and unintentional weight loss was diagnosed with diffuse, infiltrating, and ulcerated adenocarcinoma in the duodenum. Surgical management with duodenopancreatectomy was considered; however, it could not be performed due to deteriorating nutritional status. She later died due to abdominal sepsis. Conclusion: The clinical manifestations of adenocarcinoma of the duodenum are variable and nonspecific. It is a challenge to make an early diagnosis. We recommend considering the diagnostic possibility in patients with nausea, emesis, abdominal pain, and weight loss, which requires esophagogastroduodenoscopy and histological and immunohistochemical confirmation.

Introducción: el carcinoma primario de duodeno es una neoplasia maligna infrecuente; representa el 0,3%-0,5% de todos los carcinomas del tracto gastrointestinal y el 33%-45% de todos los carcinomas de intestino delgado. Caso clínico: paciente de sexo femenino de 57 años, con sintomatología gastrointestinal inespecífica y pérdida de peso no intencional a quien se le diagnosticó un adenocarcinoma difuso, infiltrante y ulcerado en el duodeno. Se planteó el manejo quirúrgico con duodenopancreatectomía; sin embargo, no pudo realizarse debido al deterioro del estado nutricional y posteriormente falleció como consecuencia de una sepsis abdominal. Conclusión: las manifestaciones clínicas del adenocarcinoma de duodeno son variables e inespecíficas, es un reto realizar un diagnóstico precoz. Se recomienda considerar la posibilidad diagnóstica en pacientes con síntomas de náuseas, emesis, dolor abdominal y pérdida de peso, que requiere la realización de esofagogastroduodenoscopia y confirmación histológica e inmunohistoquímica.

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Abstract Isthmocele is a discontinuation of the myometrium at the uterine scar site in a patient with a previous cesarian section (CS). The cause of isthmocele appears to be multifactorial. Poor surgical technique, low incision location, uterine retroflection, obesity, smoking, inadequate healing of scars, and maternal age are possible related factors. Most patients with this condition are asymptomatic. However, women can present with postmenstrual bleeding, pelvic pain, subfertility, dysmenorrhea, infertility, and scar abscess. Brazil has one of the world s highest cesarean section rates. One of the consequences of the rising rate of CS is the isthmocele, an emerging female health problem. Here we report a case of mucinous cystadenoma arising in a uterine isthmocele, a complication, as far as we could investigate, not yet described in the literature.

Resumo Istmocele é a descontinuidade do miométrio no local da cicatriz uterina em paciente com cesariana anterior. A causa da istmocele parece ser multifatorial. Má técnica cirúrgica, baixa localização da incisão, retroflexão uterina, obesidade, tabagismo, cicatrização inadequada de cicatrizes e idade materna são possíveis fatores relacionados. A maioria dos pacientes com esta condição é assintomática. No entanto, as mulheres podem apresentar sangramento pós-menstrual, dor pélvica, subfertilidade, dismenorreia, infertilidade e abscesso cicatricial. O Brasil tem uma das maiores taxas de cesariana do mundo. Uma das consequências da taxa crescente de cesarianas é a istmocele, um problema emergente de saúde feminina. Aqui relatamos um caso de cistoadenoma mucinoso originado em uma istmocele uterina, uma complicação ainda não descrita, até onde pudemos investigar.

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The incidence of ovarian cancer during pregnancy is low. Most adnexal tumors removed during pregnancy are benign, with ovarian carcinomas found in approximately 1: 10,000-1: 50,000 pregnancies. Literature on this disease is scarce and consists mostly of retrospective studies and case reports. We report the case of a pregnant patient who presented with a primary intestinal-type mucinous adenocarcinoma of the ovary and underwent unilateral salpingo-oophorectomy, with no additional surgical or chemotherapy treatment after the histological diagnosis, despite an infiltrative stromal invasion pattern. To the best of our knowledge, no such case has been previously reported. Conservative treatment in this case of early ovarian carcinoma is possible during pregnancy and should be performed in the Department of Gynecological Oncology and Obstetrics of a tertiary referral hospital. Given the possibility of disease recurrence, such patients require strict clinical oncological surveillance, specialized prenatal care, and assistance from a multidisciplinary team to improve the maternal and perinatal outcomes.

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Resumen El carcinoma mucinoso es una estirpe poco frecuente de cáncer de mama, la cual representa menos del 4% de todos los cánceres primarios. Suele presentarse en pacientes postmenopáusicas, alrededor de la séptima década de la vida. Clínicamente se caracteriza por manifestarse como un nódulo palpable, rara vez acompañado de otra sintomatología. Las herramientas de imagen, como la mastografía y el ultrasonido, son fundamentales para su diagnóstico; sin embargo, en algunas situaciones se puede subestimar el diagnóstico dado a las características similares que comparte con otras lesiones benignas. El diagnóstico definitivo se realiza por medio de histopatología. Debido a la rareza de estos tumores, no existe un consenso sobre el tratamiento más adecuado. Muchos autores concuerdan que la intervención quirúrgica continúa siendo la piedra angular, ya que tiene un impacto positivo en la supervivencia y baja incidencia de recurrencias. Esta se puede acompañar posteriormente de terapias endocrinas adyuvantes. Afortunadamente, el pronóstico de este tipo de tumores suele ser favorable, incluso la supervivencia supera el 90% a los 5 años.

Abstract Mucinous carcinoma is a rare type of breast cancer, which represents less than 4% of all primary cancers. It usually occurs in postmenopausal patients, around the seventh decade of life. Clinically, it is characterized by the presence of a palpable nodule, rarely accompanied by other symptoms. Imaging tools, such as mammogram and ultrasound, are essential for its diagnosis, however, in some situations the diagnosis can be underestimated due to the similar characteristics that it shares with other benign lesions. Definitive diagnosis is made by histopathology. Regarding treatment, there is no consensus on the most appropriate, due to the low incidence of these tumors. Many authors agree that surgical intervention continues to be the best option, showing a positive impact on survival and low recurrences. This can be accompanied later by adjuvant endocrine therapies. Fortunately, the prognosis of this type of tumor is usually favorable, even survival exceeds 90% at 5 years.

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El cistoadenoma apendicular es una neoplasia poco frecuente, que tiene una incidencia de 0,2 % a 0,3 % en todas las apendicetomías; esta afección predomina en pacientes féminas y su presentación es poco específica en cuanto a los síntomas, los cuales pueden compararse a un cuadro de apendicitis aguda, una masa abdominal, un cuadro obstructivo o ginecológico, o manifestaciones urológicas, que son las menos frecuentes. Se presentó una paciente femenina de 59 años de edad con dolor abdominal localizado en la fosa ilíaca derecha de 4 meses de evolución; se realizaron varios exámenes complementarios, una laparoscopia diagnóstica, una laparotomía exploratoria y también una apendicetomía. Después de estos exámenes se realizó un diagnóstico histológico de cistoadenoma mucinoso apendicular.

Appendiceal cystadenoma is a rare neoplasm, with an incidence of 0.2% to 0.3% among all appendectomies; this condition predominates in female patients and its presentation is unspecific in terms of symptoms, which can be compared to acute appendicitis, an abdominal mass, obstructive or gynecological symptoms, or urological manifestations, which are the least frequent. We present a 59-year-old female patient with an abdominal pain over 4 months located in the right iliac fossa; several complementary tests were performed such as a diagnostic laparoscopy, an exploratory laparotomy and an appendectomy. After these examinations, a histological diagnosis of appendiceal mucinous cystadenoma was made.

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Introducción. El cistoadenoma mucinoso biliar es una neoplasia rara con alta probabilidad de malignidad. Su diagnóstico es un reto ya que se asemeja a otras masas benignas que pueden encontrarse en el hígado. Caso clínico. Mujer de 21 años con sensación de masa en hipocondrio derecho, a quien se le realizan marcadores tumorales y estudios de imágenes concluyendo que se trataba de un cistadenoma mucinoso biliar. Resultado. Se presenta el caso de una paciente con cistoadenoma mucinoso biliar, diagnosticada y tratada exitosamente con cirugía. Conclusión. El diagnóstico de cistoadenoma mucinoso biliar se confirma mediante marcadores tumorales y estudios radiológicos, y su tratamiento es quirúrgico debido al riesgo de malignidad

Introduction. Biliary mucinous cystadenoma is a rare neoplasm with a high probability of malignancy. Its diagnosis is a challenge since it resembles other benign masses that can be found in the liver. Clinical case. A 21-year-old woman with a sensation of a mass in the right hypochondrium, who underwent tumor markers and imaging studies, concluding with a diagnosis of biliary mucinous cystadenoma. Result. A case of a patient with biliary mucinous cystadenoma diagnosed and successfully treated by surgery is presented. Conclusion. The diagnosis of biliary mucinous cystadenoma is confirmed by tumor markers and radiological studies, and its treatment is surgical due to the risk of malignancy

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Resumen ANTECEDENTES: Los tumores del apéndice son bastante excepcionales y el adenocarcinoma mucinoso es el más frecuente de estos tumores. Esta patología es aún más infrecuente en pacientes embarazadas. CASO CLÍNICO: Paciente de 43 años, en curso de las 38.1 semanas del primer embarazo. Antecedentes: diabetes gestacional y obesidad mórbida. Se hospitalizó para practicarle la cesárea de urgencia, indicada por restricción del crecimiento intrauterino, sin posibilidad de una conducta expectante. En la ecografía de control prenatal del primer trimestre se describió la existencia de una masa quística, tabicada, de aproximadamente 12 x 12 cm. En la resonancia magnética nuclear de abdomen se encontró una lesión quística en el flanco derecho, de probable origen de masa anexial derecha de 12 x 9 cm. El estudio histopatológico reportó el hallazgo de una neoplasia mucinosa de bajo grado, asociada con extravasación extramural de mucina del apéndice cecal y un pseudomixoma peritoneal. CONCLUSIÓN: El hallazgo de una neoplasia mucinosa del apéndice en una paciente embarazada es sumamente raro. Su diagnóstico supone un reto, sobre todo en quienes no tienen síntomas. Aunque hay padecimientos más frecuentes en las mujeres, como los tumores de ovario o la apendicitis, vale la pena tener en cuenta este cuadro clínico en futuras pacientes. Se requiere mayor investigación de esta neoplasia en embarazadas para optimizar los métodos diagnósticos y poder tratarla de manera oportuna y evitar dañar al feto o la madre.

Abstract BACKGROUND: Tumours of the appendix are quite rare, with mucinous adenocarcinoma being the most frequent of these tumours. This pathology is even rarer in pregnant patients. CLINICAL CASE: 43-year-old patient, in the course of 38.1 weeks of her first pregnancy. History: gestational diabetes and morbid obesity. She was hospitalised for emergency caesarean section, indicated for intrauterine growth restriction, with no possibility of expectant management. The first trimester prenatal ultrasound scan showed a cystic mass, septate, measuring approximately 12 x 12 cm. Magnetic resonance imaging of the abdomen revealed a cystic lesion in the right flank, probably caused by a right adnexal mass measuring 12 x 9 cm. Histopathological study reported the finding of a low-grade mucinous neoplasm associated with extramural extravasation of mucin from the cecal appendix and a peritoneal pseudomyxoma. CONCLUSION: The finding of a mucinous neoplasm of the appendix in a pregnant patient is extremely rare. It is challenging to diagnose, especially in those without symptoms. Although there are more common conditions in women, such as ovarian tumours or appendicitis, this condition is worth considering in future patients. Further investigation of this neoplasm in pregnant women is needed to optimise diagnostic methods to treat it in a timely manner and to avoid harm to the foetus or the mother.

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Introdução: Neoplasia cística mucinosa é tumor mucinoso benigno (cistoadenoma mucinoso) ou maligno (cistoadenocarcinoma mucinoso), que não se comunica com os ductos pancreáticos. Objetivo: Apresentar revisão da literatura sobre o tema. Método: Ênfase nas diretrizes das principais sociedades médicas mundiais na orientação do diagnóstico, tratamento e a vigilância da neoplasia cística mucinosa. Resultado: A quase totalidade dessas neoplasias ocorre no gênero feminino de 40-50 anos de idade. Como raras exceções, esta neoplasia é encontrada na cauda/corpo do pâncreas. Para estabelecer o diagnóstico é necessário a presença de estroma similar ao do ovário na parede do cisto no exame patológico. Exames de imagem de alta resolução, como tomografia, ressonância magnética e ecoendoscopia apresentam elevada precisão para identificar esta neoplasia. O tratamento cirúrgico consiste na pancreatectomia distal com linfadenectomia e esplenectomia. A via laparoscópica ou robótica é preferida para tumores <5-7 cm. Devido a possibilidade de rotura do tumor e disseminação da neoplasia, as lesões >5-7 cm devem ser submetidos à ressecção laparotômica. Conclusão: Não existe uniformidade internacional na conduta terapêutica. O tratamento cirúrgico deve ser indicado para todos os pacientes com condições cirúrgicas e que apresentam neoplasia ≥3-4 cm, dependendo do consenso.

Introduction: Mucinous cystic neoplasia is a benign mucinous tumor (mucinous cystadenoma) or malignant (mucinous cystadenocarcinoma), which does not communicate with the pancreatic ducts. Objective: To present a review of the literature on the topic. Method: Emphasis on the guidelines of the main global medical societies in guiding the diagnosis, treatment and surveillance of mucinous cystic neoplasia. Result: Almost all of these neoplasms occur in females aged 40-50 years. As a rare exception, this neoplasm is found in the tail/body of the pancreas. To establish the diagnosis, the presence of stroma similar to that of the ovary in the cyst wall is necessary on pathological examination. High-resolution imaging exams, such as tomography, magnetic resonance imaging and endoscopic ultrasound, are highly accurate in identifying this neoplasm. Surgical treatment consists of distal pancreatectomy with lymphadenectomy and splenectomy. The laparoscopic or robotic route is preferred for tumors <5-7 cm. Due to the possibility of tumor rupture and dissemination of the neoplasm, lesions >5-7 cm must undergo laparotomic resection. Conclusion: There is no international uniformity in therapeutic conduct. Surgical treatment should be indicated for all patients with surgical conditions and who have neoplasia ≥3-4 cm, depending on the consensus.

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