Benign multicystic peritoneal mesothelioma: literature review and update

Benign multicystic peritoneal mesothelioma (BMPM) is a rare peritoneal tumor diagnosed predominantly in pre-menopausal women. Associated risk factors include endometriosis and pelvic inflammatory disease in women, and prior abdominal surgery in both genders. To date, the pathogenesis of this disease remains controversial with possible etiologies, including a neoplastic versus a reactive process. Given the risk factors, some authors believe that this disease is secondary to a reactive process. However, because some studies describe cases where there is no prior surgical history or inflammatory milieu present, and because of this entity's predilection for recurrence, some authors believe the origin to be neoplastic. Some genetic and familial associations have also been reported. Malignant transformation is extremely rare, with only two cases reported in the literature, despite the recurrence potential. Like the etiology, the name of this entity is also controversial. Some authors prefer the term "peritoneal inclusion cyst (PCM)" instead of "benign cystic mesothelioma" and argue that the term mesothelioma should only be used when there is evidence of atypia. Most cases of BMPM are discovered incidentally. Others reflect sequela of tumor mass effect. It appears intra-operatively as large, multi-focal, cystic lesions in the peritoneal and pelvic cavity. Diagnosis is achieved through surgical sampling with histopathological examination. Immunobiologically, BMPM exhibits multiple small cystic spaces with flattened lining containing calretinin positive cells without atypical features, mitotic figures, or tissue invasion. Treatment includes cytoreductive surgery. Here we present a case of BMPM in a 60-year-old male - a rare disease in an uncommon patient population.

Le lymphangiome cervico-facial congenital de l'enfant au chu de conakry : analyse de trois cas

Le lymphangiome kystique est une tumeur vasculaire; benigne; rare; d'origine lymphatique. La forme principale est representee par le lymphangiome cervico-facial congenital de l'enfant. Sa pathogenese est inconnue. Cette malformation congenitale du systeme lymphatique est composee de formations kystiques developpees a partir d'endothelium lymphatique et remplies de lymphe et de sang. L'atteinte des voies aerodigestives superieures (VaDS) peut conduire a des complications graves; mettant en jeu le pronostic vital. nous presentons trois observations de lymphangiomes kystiques de diagnostic post natal. Le traitement etait chirurgical dans tous les cas consistant en exerese complete de la tumeur et le diagnostic confirme par l'examen histopathologique de la piece operatoire. apres un recul d'au moins 12 mois; aucune recidive locale n'a ete observee cependant la surveillance reste de mise

Linfangioma abdominal: caso clínico / Abdominal lymphangioma: clinical case

Abdominal lymphangioma is a rare benign congenital malformation of the mesenteric and/or retroperitoneal lymphatics, more frequent in pediatrics. Objective: To report a case with unusual clinical presentation of lymphangioma. Clinical Case: A two-year old boy previously health who presented with abdominal pain, lipothymia, and generalized pallor ensuing an abdominal massage. Abnormal findings included an abdominal mass and Ht of 17.3 percent. Sonogram showed a large retroperitoneal mass with anechogenic areas and vasculari-zed septi. CT scans showed similar results. Surgical removal and biopsy confirmed the diagnosis. Discusion: Abdominal lymphangioma is uncommon in children, presentation varies from asymptomatic to acute abdomen. Images are essential in diagnosis and pre-op studies.

El linfangioma abdominal es una patología poco frecuente, benigna que corresponde a una malformación congénita del desarrollo linfático mesentérico y/o retroperitoneal. Es más frecuente en población infantil. Objetivo: Reportar un caso con presentación atípica de linfangioma. Caso clínico: Paciente de 2 años varón, previamente sano, que se presenta con dolor abdominal, luego lipotimia y palidez generalizada tras recibir un masaje abdominal. Al examen físico se encontró una masa abdominal, sin hepatomegalia, esplenomegalia ni adenopatías palpables. Presentaba un hematocrito de 17,3 por ciento. La Ecografía mostró una gran masa retrope-ritoneal con áreas anecogénicas y septos vascularizados. La TAC mostró hallazgos similares: masa quísti-ca, lobulada probablemente determinada por linfangioma abdominal. Se realizó extirpación quirúrgica y la biopsia confirma el diagnóstico. Discusión: El linfangioma abdominal es una patología infrecuente en niños. La presentación clínica es variable, puede ser desde asintomático hasta presentarse con abdomen agudo. Se requiere estudio imagenológico para su evaluación preoperatoria. La ecografía y TAC tienen un importante rol diagnóstico, en localización, tamaño, anatomía circundante y posibles complicaciones.

Linfangioma microcístico acral: diagnóstico diferencial em lesões verrucosas de extremidades / Acral microcystic lymphangioma: differential diagnosis in verrucous lesions of the extremities

Linfangioma é uma má-formação originária da migração anormal de tecido linfático, levando a falhas na comunicação e na drenagem da linfa. Apresenta-se mais comumente como vésico-pápulas agrupadas de conteúdo translúcido na pele ou mucosas. A apresentação inicial na forma de placa verrucosa limitada a um único pododáctilo é atípica e reforça a relevância deste relato. Ainda que considerado lesão benigna, o linfangioma, a depender de sua localização e dimensão, pode apresentar dor local ou infecções recorrentes, com interferência substancial na qualidade de vida. Relatamos caso de linfangioma circunscrito a pododáctilo de surgimento tardio na idade adulta, na forma de placa verrucosa - apresentação atípica confirmada apenas após histopatológico. A excisão cirúrgica garantiu resposta estética e funcional satisfatória.

Lymphangiomas are a malformation caused by the abnormal migration of lymphatic tissue, leading to failures in the communication and drainage of the lymphatic system. They usually present as groups of translucent papules and vesicles in the skin or mucous membranes. Presentation as a verrucous plaque limited to a single toe is unusual and emphasizes the relevance of this case report. Although considered a benign lesion, depending on the site affected by the lymphangioma and its size, it may lead to localized pain or recurrent infections, substantially affecting the patient's quality of life. The present case report describes a patient with lymphangioma of late onset developing in adulthood in the form of a verrucous plaque confined to a single toe. Diagnosis of this unusual presentation could only be confirmed following histopathology. Surgical excision resulted in a satisfactory functional and cosmetic outcome.

Linfangioma cervical quístico de glándula submaxilar: reporte de un caso clínico / Cystic cervical lymphangioma of submaxillary gland

Los linfangiomas se encuentran com mayor frecuencia en el triángulo posterior del cuello y axila en los niños. El 50 por ciento de los linfangiomas quísticos están presentes al nacer y el 90 por ciento son evidentes a los 2 años de edad. Existen en la literatura algunos reportes de casos de higromas quísticos desarrollados en la edad adulta. A menudo estas lesiones crecen rápidamente, secundarias a una infección respiratoria alta, infiltrando estructuras vitales. Estos cambios pueden conducir a la disfagia, disnea y peligro de vida por la obstrucción de las vías respiratorias. La completa escisión quirúrgica es el tratamiento de elección y ofrece las mejores posibilidades de curación.

Acquired Omental Cystic Lymphangioma after Subtotal Gastrectomy: A Case Report

We herein describe a case of cystic lymphangioma in the greater omentum of the remnant stomach, which is thought it to be related with subtotal gastrectomy 10 yr ago for early gastric cancer. A 76-yr-old man was admitted to our department with postprandial abdominal discomfort and bowel habit change. Intraabdominal multilocular cystic mass was detected by ultrasonography and computed tomography. We performed a complete En-bloc tumor resection including spleen and distal pancreas, and histological examination confirmed cystic lymphangioma originated from the greater omentum of the remnant stomach. Although the etiology of omental lymphangioma remains largely unclear, these findings suggested strongly that obstruction of the lymphatic vessels after gastric resection for gastric carcinoma might be the most plausible cause. The surgical extirpation with resection of organs involved appears to be a treatment of choice for such unusual case.

Linfangioma cístico do mediastino / Cystic lymphangioma of the mediastinum

O linfangioma cístico é um tumor congênito benigno e raro. Resulta da proliferação focal de tecido linfático bem diferenciado com origem num anormal desenvolvimento do sistema linfático. A maioria ocorre em crianças ou adultos jovens nos sacos linfáticos primordiais. Geralmente ocorrem no pescoço (75%) e na região axilar (20%). Apenas 1% desses são localizados no mediastino. Os linfangiomas adquiridos, principalmente encontrados em adultos de meia-idade, são conseqüentes a um processo de obstrução linfática crônica secundária a cirurgia, infecção crônica ou radiação. Os autores apresentam um caso clínico de linfangioma cístico do mediastino num indivíduo do sexo masculino de 50 anos, detectado por achado em radiografia de tórax de rotina.

Cystic lymphangioma is a rare congenital benign tumor. It results from focal proliferation of well-differentiated lymphatic tissue originating from abnormal development of the lymphatic system. Most cystic lymphangiomas occur in the primitive lymph sacs of children or young adults. They generally appear in the neck (75%) and axillary region (20%). Only 1% are located in the mediastinum. Acquired lymphangiomas, which are principally seen in middle-aged adults, are caused by a process of chronic lymphatic obstruction secondary to surgery, chronic infection, or radiation. Here, we present the case of a 50-year-old male with cystic lymphangioma of the mediastinum, detected on a routine chest X-ray.

Fetal Axillary Cystic Hygroma Detected by Prenatal Ultrasonography: A Case Report

Fetal cystic hygroma is a rare developmental congenital anomaly of the lymphatic system, characterized by the formation of a multilocular, variable sized cystic mass. Most of cystic hygromas are found in the neck and other rare locations include axilla, mediastinum, and limbs. There are many papers about cystic hygroma colli, but there are only a few papers about fetal axillary cystic hygroma and no domestic papers. We present a case of fetal axillary cystic hygroma diagnosed antenatally followed by full-term delivery in a 30-yr-old woman. Operation was performed on the 8th day after birth and the mass was excised and confirmed as cystic hygroma.

Cystic hygroma of the gluteal region.

Cystic hygromas occur most commonly in the neck. Rarely are they known to involve the axilla, groin, mediastinum, retroperitoneum, pelvis, mesentery, omentum and spleen. We successfully managed a case of cystic hygroma of gluteal region in a one and half year old child who presented with a cystic, non transilluminant swelling in this region since birth. The diagnosis of cystic hygroma was made by surgery and subsequently confirmed after histopathological examination. Because of rarity of cystic hygroma in gluteal region this case in being reported.

Linfangioma quístico de mesenterio / Cystic lymphangioma of mesentery

El siguiente trabajo tiene por objetivo la presentación de un caso de Linfangioma Quístico de Mesenterio, observado en un paciente jóven de sexo masculino. Es un tumor de escasa frecuencia, de difícil diagnóstico preperatorio, y que en la mayoría de los casos se presenta como un cuadro Abdómen Agudo Inflamatorio. Se hacen consideraciones históricas, etiológicas, anátomopatológicas y clínico-quirúrgicas, tratando de actualizar una patología de características inusuales.

Linfangiomas quísticos abdominales / Abdominal cystic lymphangiomas

Los linfangiomas quísticos abdominales tiene muy baja incidencia, pueden manifestarse a cualquier edad, pero son más frecuentes en los niños y su tratamiento es la extirpación quirúrgica. Sus expresiones clínicas más características son la masa y el dolor abdominal. Los síntomas, a veces de comienzo brusco, suelen conducir en muchos casos a intervenciones quirúrgicas de urgencia. Entre los años 1975 y 1996 se operaron en nuestro servicio de urgencia pediátrica 20 pacientes con esta afección, la edad de los cuales fluctuaba desde el nacimiento hasta 13 años, mediana tres años, con similar distribución por sexo. Doce pacientes fueron operados en el Servicio de Urgencia y 8 en forma electiva. La localización más frecuente del tumor era el mesenterio del íleon. El tratamiento consistió en extirpación de la masa, que en 10 casos requirió resección intestinal. Un paciente falleció en el postoperatorio por neumonía, los otros 19 pacientes evolucionaron de manera satisfactoria, sin complicaciones ni recidiva de la lesión