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)

Differentiating Pancreatic Ductal Adenocarcinoma from Pancreatic Serous Cystadenoma, Mucinous Cystadenoma, and a Pseudocyst with Detailed Analysis of Cystic Features on CT Scans: a Preliminary Study

OBJECTIVE: To determine whether or not detailed cystic feature analysis on CT scans can assist in the differential diagnosis of pancreatic ductal adenocarcinoma (PDAC) from serous cystadenoma (SCN), mucinous cystadenoma (MCN), and a pseudocyst. MATERIALS AND METHODS: This study received Institutional Review Board approval and informed patient consent was waived. Electronic radiology and pathology databases were searched to identify patients with PDAC (n = 19), SCN (n = 26), MCN (n = 20) and a pseudocyst (n = 23) who underwent pancreatic CT imaging. The number, size, location, and contents of cysts, and the contour of the lesions were reviewed, in addition to the wall thickness, enhancement patterns, and other signs of pancreatic and peripancreatic involvement. Diagnosis was based on lesion resection (n = 82) or on a combination of cytological findings, biochemical markers, and tumor markers (n = 6). Fisher's exact test was used to analyze the results. RESULTS: A combination of the CT findings including irregular contour, multiple cysts, mural nodes, and localized thickening, had a relatively high sensitivity (74%) and specificity (75%) for differentiating PDAC from SCN, MCN, and pseudocysts (p < 0.05). Other CT findings such as location, greatest dimension, or the presence of calcification were not significantly different. CONCLUSION: The CT findings for PDAC are non-specific, but perhaps helpful for differentiation. PDAC should be included in the general differential diagnosis of pancreatic cystic neoplasms.

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.

Mucinous cystadenoma of the ovary in a 12-year-old girl

Benign mucinous cystadenomas MCA are rare benign neoplasms in the pediatric age group. Only 19 cases have been described before, and they often present as large abdominal masses. We present a 12-year-old patient that had a huge mass arising from the left ovary causing bilateral hydronephrosis and renal insufficiency. The final pathology revealed a benign MCA. Patients with mucinous tumors usually present late, this may lead to renal insufficiency secondary to urinary outflow obstruction. Unilateral salpingo-oophorectomy is usually sufficient treatment for these cases. The diagnosis of MCA should be considered in children who present with huge pelvi-abdominal masses. Early recognition and intervention are necessary to avoid potential complications

Pancreatic mucinous cystadenoma.

We report here the case of a 75-year-old lady who presented to us with a 4-month history of abdominal symptoms. The computed tomography scan revealed a cystic lesion in the tail of the pancreas. Distal pancreatectomy was done and biopsy showed a benign mucinous neoplasm. Because this is potentially malignant it is vital to diagnose it before it becomes malignant; identification of this entity remains a diagnostic and therapeutic challenge.

Mucinous cystadenoma of the renal pelvis with malignant transformation: a case report.

A mucinous cystadenoma of the renal pelvis with malignant transformation is an extremely rare entity. We discuss one such rare occurrence in a 62 year old female who developed mucinous nephrosis due to marked retention of mucin produced by the tumor.

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.

Sarcoma-like mural nodules in ovarian mucinous cystadenomas--a report of two cases.

Sarcoma-like mural nodule is a very rare occurrence in a mucinous tumour of the ovary. Two such nodules having morphologically benign features with osteoclastic giant cells, in the wall of mucinous cystadenoma are described. In addition, these nodules exhibited reactive vascular proliferations. The sarcoma-like nodules are associated with a favourable outcome and must be distinguished from other malignant nodules composed of sarcoma or anaplastic carcinoma that may also occur in the wall of mucinous ovarian tumours.

Ovarian primary neuroendocrine carcinoma of non - small cell type: Report of an exremely rare Neoplasm

Paraffin block sections of a uterus and ovarian mass from a 31 year old female were sent for second opinion to the Pathology Laboratory of Aga Khan University. Histologic examination and immunohistochemical features gave a diagnosis of primary neuroendocrine carcinoma of non-small cell type admixed with benign mucinous cystadenoma. This is a rare tumour with only eight being reported in literature

Laparoscopic cystectomy of an ovarian mucinous cystadenoma in a 12-year-old girl: a possible role of operative laparoscopy for a huge benign adnexal mass.

The case of an obese 12-year-old girl with a huge and mobile pelvic cystic mass is presented. After ultrasonography and determination of tumor markers, a right huge benign adnexal mass was suspected. In order to avoid exploratory laparotomy because severe pain and a large operative scar were expected, an operative laparoscopy was performed. No intra-operative and post-operative complications were observed. With the uneventful recovery, only a one-day hospital stay was needed. Neither residual nor recurrent evidence was suspected after 2-, 6-, 12-, 18- and 24-months follow-up. With proper case selection, good pre-operative counseling and the operation performed under familiarity and good training in laparoscopy, laparoscopic management of a huge suspected benign adnexal mass is technically feasible. The benefits are reducing hospital stay, lowering morbidity, especially less pain and cosmetic acceptance of the operative scar.

Ovarian mucinous cystadenoma in a female with Turner syndrome

The development of an epithelial tumor, especially mucinous type, in a female with a streak gonad is rare and not fully understood. We report a case of a 19-year-old; a single female known to have Turner syndrome presented with an increased abdominal girth and was found to have a huge pelvic and abdominal mass. Ultrasound and magnetic resonance imaging revealed a huge cystic ovarian mass with no ascites. Laparotomy and right oophorectomy were performed for the ovarian mass. Histology revealed a large mucinous cyst adenoma. Further study of these tumors may help to eludicate the underlying cause and pathogenesis

Periampullary carcinoma with bilateral ovarian cystadenoma in pregnancy.

We report a 30-year-old woman with periampullary cancer and a large left ovarian cystadenoma presenting during pregnancy with features of duodenal obstruction. She had a right benign ovarian tumor removed a year back. She delivered an intrauterine-growth-retarded live baby. Laparotomy in the postpartum period revealed an unresectable periampullary growth with hepatic metastasis and a left ovarian cystadenoma. The ovarian tumor was removed and a palliative gastrojejunostomy performed.

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

Surface epithelial tumours of the ovary.

Surface epithelial tumours (SET) constituted 65.7% of all the ovarian tumours. Benign tumours were 182 (71.9%), of low malignant potential (LMP) 11 (4.4%) and frank malignant 60 (23.7%). Maximum number of cases, 102 (40.3%) belonged to 3rd decade. Mean age for serous cystoma was 31.5% years as compared to 30.8 years for mucinous cystoma. The commonest presenting feature was the abdominal lump observed in 182 cases (71.9%) and pain in abdomen in 120 (47.4%).Serous cystomas were t he most frequent tumours and comprised of 32.21% of all the ovarian tumours or 46.01% of all the SET or 65.56% of all the cystic SET. Seventeen (11.7%) of serous tumours were bilateral. Mucinous cystomas constituted 14.55% of all the ovarian tumours or 30.8% of all the SET. These tumours were bulky (78.6%; 15 cm diameter) and multilocular (83.9%).Mucinous cystadenocarcinoma was the commonest malignant epithelial tum our (36.6%). Endometroid carcinoma comprised 3.65% of all the SET or 8.4% of all the ovarian malignancy. Squamous metaplasia was seen in one case whereas 2 cases were of mesodermal mixed tumour with heterologous element as rhabdomyosarcoma. Clear cell carcinoma, Brenner tumour and unclassified group constituted 0.79%, 1.18% and 1.58% of all SET respectively.