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.

Mucinous cystadenoma and carcinoid tumor arising from an ovarian mature cystic teratoma in a 60 year-old patient: a case report.

BACKGROUND: Mature cystic teratomas (MCT) of the ovary are benign ovarian germ cell neoplasms. Malignant transformation is possible but rare and ovarian carcinoid tumors in MCT are among the most extremely rare subtypes. CASE PRESENTATION: We report a case of a 60-year-old Iranian woman suffering from postmenopausal bleeding and hypogastric pain for the last 40 days. An adnexal mass was detected during the physical examination. Ultrasound imaging showed a (55 × 58) mm mass in the left ovary. Total abdominal hysterectomy, bilateral salpingooophorectomy and comprehensive staging surgery were performed for the patient. Intraoperative frozen section of the left ovarian mass was indicative of a malignant tumor. She was diagnosed with a carcinoid tumor with benign mucinous cystadenoma arising on MCT of the ovary, confirmed in the histopathology and immunohistochemistry examination. The tumor was classified as low grade and no chemotherapy cycles were considered. The patient was followed up long-term and no recurrence was observed during 14 months of examinations. CONCLUSION: Ovarian carcinoids arising from MCT are rare neuroendocrine neoplasms, and proper diagnosis of these tumors requires careful histopathology evaluation and appropriate examination. Therefore, it is necessary to consider these tumors as a possible differential diagnosis and evaluate them in individuals (especially postmenopausal women) who have abdominal pain or abnormal bleeding and a palpable mass.

The frequency and prognostic role of P53 and P16 immunoexpression in primary ovarian mucinous tumors.

BACKGROUND: Primary ovarian mucinous tumors are uncommon. Factors leading to invasive progression and metastatic disease have not been fully delineated yet. The aim of this study is to determine the rates of p53 and p16 immunoexpressions in primary ovarian mucinous tumors, to investigate their relationship with clinicopathologic factors and their impact on prognosis and survival. METHODS: Seventy-eight primary ovarian mucinous tumors (30 mucinous cystadenomas, 30 mucinous borderline tumors (MBOT), 18 mucinous carcinomas (MOC)) were evaluated immunohistochemically with p53 and p16 staining. The demographic, clinicopathological data, and postoperative follow-up findings of the patients were analyzed. RESULTS: Mutation-type p53 staining was present in 1/30 (3.3 %) cystadenoma, 10/30 (33.3 %) MBOT and 9/18 (50 %) MOC (p = 0.001). p16 overexpression was detected in 3/30 (10.0 %) MBOT and 5/18 (27.8 %) MOC, but not in any cystadenoma (p = 0.04). The frequency of mutation-type p53 staining in MBOTs with microinvasion was higher (71.4 %) than in those without (28.6 %, p = 0.026). The frequencies of p16 or p53 mutations were similar in MBOTs with and without intraepithelial carcinoma, or mural nodule (p > 0.05). In MOCs with ovarian surface involvement, mutation-type p53 staining was detected in 66.7 % (6/9) and p16 overexpression in 55.6 % (5/9) of the cases. A significant difference was found between MOCs with or without ovarian surface involvement regarding the frequency of p16 overexpression (p = 0.029). Any relationship was not detected between survival and p53 and p16 expression in MOCs (p > 0.05). CONCLUSION: p53 and p16 mutation rates were higher in MOCs compared to mucinous cystadenomas and MBOTs and suggest a relevant role in the development of primary ovarian mucinous carcinoma, however further studies are needed in this regard.

Primary mucinous tumors of the renal pelvis: Clinical, histopathological, and molecular analysis of three cases.

Primary mucinous tumors of the renal pelvis are extremely rare and pose challenges in terms of diagnosis and treatment. This study reviewed the clinical and pathological characteristics of mucinous tumors of the renal pelvis, including mucinous cystadenocarcinomas and mucinous cystadenomas. Immunohistochemical analysis was conducted in three cases, along with KRAS gene detection using the Amplification Refractory Mutation System (ARMS) method. The results revealed mucinous epithelium with acellular mucinous pools in all cases, and acellular mucinous pools were observed in the renal parenchyma and perirenal fat capsules. All tumors expressed CK20 and CDX2, and one case showed KRAS gene mutation. The study suggests that mucinous cystadenomas of the renal pelvis may exhibit borderline biological behaviors. This study is the first to report a KRAS gene mutation in a mucinous cystadenoma of the renal pelvis, offering valuable insights into the diagnosis and treatment of this rare condition.

Mucinous cystadenoma and benign mesonephric-like proliferation in the ovary - Further evidence for clonal relationship.

Mesonephric-like adenocarcinomas rarely occur in the uterus and the ovary. Benign mesonephric-like (ML) proliferations and hyperplasia have been described solely within the ovary. Pathogenetic data are very limited. We report a case with microscopic focus of benign ML-proliferation in association with mucinous cystadenoma in the ovary. The immunophenotype was distinct (mucinous tumor: focal weak nuclear positivity for PAX-8, CK 7, patchy cytoplasmic positivity for p16 and negativity for estrogen receptor, CD 10, TTF-1, p53 wildtype; mesonephric component: diffusely positive for PAX-8, CK 7, luminal CD 10, TTF-1, focal staining for estrogen receptor, patchy cytoplasmic for p16, p53 wildtype). On NGS-analysis there was clonal mutation of KRAS p.G12C. The data provide additional evidence for the concept of transdifferentiation (Müllerian tissue representing Wolffian/mesonephric features on histology and immunostaining) within the pathogenesis of mesonephric proliferation of the female genital tract and demonstrate the clonal relationship between these distinct morphologic components.

Full-term pregnancy with retroperitoneal giant mucinous cyst: A case report and literature review.

RATIONALE: Retroperitoneal benign cysts during pregnancy are extremely rare and often remain asymptomatic until they attain a very large size. Diagnosis typically relies on a pathological tissue biopsy. The decision to pursue 1-step or 2-step surgical treatment should be tailored to each individual case rather than generalized. PATIENT CONCERNS: This case report presents the unique scenario of a pregnant woman with a confirmed pregnancy complicated by a large retroperitoneal cyst. The patient had a retroperitoneal cyst during her initial pregnancy, which went undetected during the first cesarean section. However, it was identified during her second pregnancy by which time it had grown to 13.0 cm × 15.0 cm × 25.0 cm, and extended from the liver margin to right ovarian pelvic infundibulopelvic ligament. Consequently, it was removed smoothly during her second cesarean section. DIAGNOSES: Postoperative pathology results indicated a massive retroperitoneal mucinous cystadenoma. INTERVENTIONS: The giant retroperitoneal cyst was smoothly excised during the second cesarean delivery for 1-step surgical treatment. OUTCOMES: Under the combined spinal and epidural anesthesia, a live female infant was delivered at 38 3/7 gestational weeks and the neonatal weight was 3200g. Under general anesthesia with endotracheal intubation, the giant retroperitoneal cyst was excised smoothly without complications. LESSONS: The findings of this case report contribute to the understanding of the diagnostic modalities, surgical approaches and postoperative considerations of giant retroperitoneal cysts associated with pregnancy.

Genomic analysis of primary epithelial neoplasms of the seminal vesicle identifies a subset of mucinous cystic tumours driven by KRAS mutations.

BACKGROUND: Carcinomas of the seminal vesicle are exceedingly rare, with a limited number of cases described in the literature. Reported cases span a relatively wide morphological spectrum, and their genomic features remain unexplored. DESIGN: In this study, we interrogated five primary epithelial neoplasms of the seminal vesicle using a targeted DNA sequencing platform (OncoPanel, 447 genes). RESULTS: The tumours included one adenocarcinoma with intestinal phenotype presenting after external beam radiation (for prostatic adenocarcinoma), one carcinoma with Müllerian-type clear cell phenotype, two mucinous tumours resembling low-grade mucinous neoplasms of the appendix (LAMN) and one mucinous cystadenoma. The post-radiation mucinous adenocarcinoma had genomic findings consistent with bi-allelic inactivation of TP53, as well as multiple copy-number changes with regional and chromosomal arm-level copy-number losses. The Müllerian-type clear cell carcinoma exhibited a complex copy-number profile with numerous regional and arm-level copy-number changes, as well as focal amplification events, including copy-number gain of 8q and amplification of a region within 20q13. Both low-grade mucinous tumours resembling LAMN harboured hot-spot gain-of-function KRAS variants (p.G12V and p.G13D) as the only genomic alteration. No genomic alterations were detected inthe lesion diagnosed as mucinous cystadenoma. CONCLUSION: Our results suggest that primary low-grade mucinous neoplasms of the seminal vesicle may represent a distinct entity equivalent to appendiceal counterparts, driven by gain-of-function variants of RAS GTPases. The remaining tumours showed genomic features that closely resembled those of neoplasms with comparable phenotypes and/or biological characteristics arising in other sites, suggesting that they could be managed similarly, with special considerations related to their anatomical location.

Neoplasia quística mucinosa hepática, reporte de un caso y análisis de la patología / Hepatic mucinous cystic neoplasm, case report and pathology analysis / Neoplasia cística mucinosa hepática, relatório de um caso e análise da patologia

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.

A rare combination of Dermoid cyst and Cystadenoma: Are collision tumors in the ovary a real entity?

Collision tumors have been reported in various organs like the gastrointestinal tract, lung, skin, adrenals, central nervous system, lymph nodes, uterus, but are rarely seen in the ovary. Collision tumors are two histologically distinct neoplasms in the same organ without any intermixture between them. Here we present a case of a collision tumor of the ovary represented by a mucinous cystadenoma and teratoma. It is imperative for a surgical pathologist to correctly diagnose the collision tumor components and differentiate them from mixed tumors as it will dictate the appropriate treatment based on the individual biological aggressiveness of each component.

Cistoadenoma mucinoso de la válvula ileocecal: tumoración intestinal infrecuente en un niño de 1 año. Reporte de caso / Mucinous cystadenoma of the ileocecal valve. Uncommon intestinal tumor in a oneyear-old boy. Case report

RESUMEN El cistoadenoma mucinoso se encuentra usualmente en el ovario, páncreas y el apéndice, pero su presentación en el intestino es extremadamente rara. En este reporte de caso, presentamos a un niño con obstrucción parcial intestinal debido a un cistoadenoma mucinoso en la válvula ileocecal. En la cirugía se retiró el íleo terminal, válvula ileocecal, ciego y apéndice, seguido de anastomosis ileocecal. El paciente evolucionó favorablemente en el postoperatorio y se recuperó sin contratiempos. A nuestro entender, este es el primer reporte de presentación de este tumor en dicha localización.

ABSTRACT Mucinous cystadenoma is usually found in the ovary, pancreas and appendix but its presentation in the intestine is extremely rare. In this case report we present an infant with partial intestinal occlusion due to a mucinous cystadenoma of the ileocecal valve. We performed an excision of the terminal ileum, ileocecal valve, cecum and appendix, followed by ileocolic anastomosis. The patient did well after the procedure and recovered uneventfully. To our knowledge, this is the first case report of this tumor in this location.

Cistoadenoma mucinoso gigante de ovario de bajo grado de malignidad / Giant mucinous ovarian cystadenoma of low malignancy

El cistoadenoma mucinoso de ovario representa aproximadamente 15 por ciento de las lesiones neoplásicas benignas de ovario y en 5 por ciento de los casos pueden ser bilaterales. Suele manifestarse en edades medias de la vida y son poco frecuentes en la edad pediátrica y en la adolescencia. El objetivo del trabajo es profundizar en el diagnóstico y la conducta terapéutica de esta enfermedad. Se realizó una revisión del tema y se presentó un caso relevante por las dimensiones del quiste. Se presenta una adolescente de 16 años de edad que presentó un cuadro de distensión abdominal y dolor en hemiabdomen derecho de un mes de evolución. Se le realizó cirugía y se encontró un tumor gigante de ovario izquierdo con 10 kilogramos de peso, se corroboró el diagnóstico histológico de cistoadenoma mucinoso de ovario. La paciente evolucionó satisfactoriamente(AU)

Ovarian mucinous cystadenoma accounts for approximately 15 percent of benign ovarian neoplastic lesions and in 5 percent of cases may be bilateral. It usually manifests in middle ages of life and they are rare in pediatric age and adolescence. The objective of this paper work is to deepen on the diagnosis and therapeutic state of this disease. A review of the topic was made and a case was presented because of the significant dimensions of the cyst. We present a 16-year-old girl who presented abdominal distension and pain in the right hemiabdomen for a month of evolution. Surgery was performed and a giant tumor on the left ovary was found. It weighed 10 kilograms, corroborating the histological diagnosis of mucinous ovarian cystadenoma. The patient evolved satisfactorily(AU)

Tumor carcinoide apendicular / Carcinoid of the appendix. A case report

Tumors of the appendix constitute an heterogeneous group of malignancies with a variable evolution and prognosis...The aim of this reporte was to determine the prevalence of carcinoid tumors of the cecal appendix and its relationship with age, sex, clincal features and introspctive suspicion.

Neoplasias quísticas pancreáticas / Cystic neoplasms of the pancreas

BACKGROUND: Cystic neoplasms of the pancreas represent between 5% and 10% of all pancreatic tumors. Due to their malignant potential, surgery is indicated. Because of the uncommon nature of this pathology, the aim of this study was to present a series of six patients who were operated on for a cystic tumor and to analyze the morphological characteristics, surgical experience and follow-up. METHODS: This was a retrospective study. Clinical files were studied, as well as surgical and pathological registries from patients diagnosed with cystic neoplasms of the pancreas. Five females and one male were included with a mean age of 54 +/- 15 years. The main symptom was abdominal pain. RESULTS: Clinical course and CT scan results were the criteria for suspecting the diagnosis. In 4/6 patients, the tumor was located within the body and tail of the pancreas. The most frequent intervention was distal pancreatectomy. There was no mediate operative mortality in a 30-day period. Histopathological diagnoses were three serum cystadenomas, two mucinous cystadenomas and one cystadenocarcinoma. After a 4-year follow-up, no tumor recurrences have been reported in the surviving patients. CONCLUSIONS: The natural history of cystic neoplasms of the pancreas is generally benign, but complications may occur by direct invasion of neighboring structures. Clinical course, tomography, pancreatography and punction are fundamental for its treatment.

Tumor borderline mucinoso ovárico gigante con microinvasión / Borderline mucinous ovarian tumor with microinvasion

Se presenta caso clínico de tumor borderline mucinoso gigante de ovario derecho operado. Se describe una breve revisión de la literatura, incluyendo los conceptos de microinvasión, arquitectura micropapilar, implantes, así como algunas características de su patogenia y tratamiento.

Tumores quísticos del páncreas / Cystic tumors of the pancreas

Los tumores quísticos del páncreas son raros y constituyen sólo 10 a 15 por ciento de las lesiones quísticas del páncreas y 1 por ciento de las neoplasias malignas. Pueden ser benignas o malignos y generalmente son circunscritos y bien localizados. Su identificación, el diagnóstico diferencial y el tratamiento son difíciles y un ejemplo de ello es que hasta una tercera parte pueden confundirse con seudoquistes del páncreas. Los más importantes son los cistadenomas microquísticos serosos, los cistadenomas mucinosos, los cistadenocarcinomas mucinosos, los adenocarcinomas productos de mucina y los adenocarcinomas asociados a seudoquiste o a quiste simple. Los estudios de mayor utilidad para el diagnóstico son el ultrasonido, la tomografía axial computada, la colangiopancreatografía retrógrada endoscópica, la punción guiada del quiste con estudio del líquido y la biopsia. La elección del tipo de tratamiento depende de la variedad del tumor de que trate, de su localización y extensión, del riesgo quirúrgico, de la experiencia del cirujano y de los recursos institucionales. En general, el pronóstico de estos tumores es más favorable que el del adenocarcinoma ductal, aun cuando sean malignos

Cistadenocarcinoma mucinoso retroperitoneal de tipo ovárico : presentación de un caso y análisis de la literatura / Mucinous retroperitoneal cistadenocarcinoma of ovarian type : case report and literature analysis

Se presenta una paciente de 24 años de edad que consultó por una masa abdominal palpable. Una ecografía evidenció una formación quística multilocular en abdomen. En la cirugía se observó un gran tumor quístico retroperitoneal, ambos ovarios y el útero no contactaban con la masa y no presentaron alteraciones a la inspección quirúrgica. El estudio anatomopatológico mostró un cistadenocarcinoma mucinoso, con áreas de tipo borderline en continuidad con un epitelio mucoso benigno y sectores de aspecto mesotelial. El estudio inmunohistoquímico demostró positividad de las células tumorales para CK7, CK AE1/AE3, EMA y CEA. La histogénesis de este tipo de tumor ocurriría a partir de una metaplasia mucinosa de epitelio celómico. La posibilidad de una metástasis de un adenocarcinoma mucinoso queda virtualmente excluída ya que: 1) La inspección quirúrgica descartó alteraciones uteroováricas. 2) Histológicamente se observó la transición de un epitelio de tipo mesotelial a uno mucinoso benigno y de éste hacia un epitelio francamente tumoral