Linfangioma Quístico del Páncreas. Un Tumor poco Frecuente / Pancreas Cystic Lymphangioma. A Rare Tumor

El linfangioma quístico del páncreas (LQP), es un tumor extremadamente raro y representa solo el 1% de los linfangiomas abdominales. El objetivo de este manuscrito fue reportar un caso de LQP intervenido quirúrgicamente; y revisar la evidencia existente respecto de sus características morfológicas, terapéuticas y pronósticas. Mujer de 67 años, con LQP operada en Clínica RedSalud Mayor Temuco en septiembre de 2023. Se verificó un tumor sólido-quístico de 16 cm de diámetro mayor, adherido al páncreas a nivel del cuerpo de la glándula, de 867 gramos de peso, con líquido amarillento en su interior; la que fue extirpada completamente. Después del estudio histológico, se realizaron tinciones inmunohistoquímicas complementarias para CD31, D2-40 y calretina. La paciente tuvo un curso postoperatorio sin incidentes, siendo dado de alta al tercer día postoperatorio. En el control alejado, se encontraba en buenas condiciones generales. El LQP es un tumor muy poco frecuente. Las características clínicas e imágenes de este tipo de lesiones son inespecíficas. Debe considerarse en el diagnóstico diferencial de las lesiones quísticas pancreáticas. La resección quirúrgica completa con márgenes libres es el tratamiento de elección; y el pronóstico del LQP es favorable si se reseca por completo.

SUMMARY: Pancreas cystic lymphangioma (PCL) are extremely rare, accounting for only 1% of abdominal lymphangiomas. The aim of this study was to report a rare case of PCL, who underwent surgery; and review the existing evidence regarding its morphological, therapeutic and prognostic characteristics. 67-year-woman patient with PCL who underwent surgery at Clínica RedSalud Mayor Temuco in September 2023. In this case, a solid-cystic tumor with a major diameter of 16 cm was identified, adhering to the pancreas at the level of the body of the gland, weighing 867 grams and containing yellowish fluid in its interior. The tumor was completely excised. Subsequent to histological examination, additional immunohistochemical staining was performed for CD31, D2-40, and calretinin. The patient experienced an uneventful postoperative course and was discharged on the third postoperative day. During the follow-up, the patient remains in good general condition. LQP is a very rare tumor. Clinical features and images of this type of lesions are nonspecific. It should be considered in the differential diagnosis of pancreatic cystic lesions. Complete surgical resection with free margins is the treatment of choice; and the prognosis of LQP is favorable if it is completely resected.

Malformación linfática quística gigante del hígado: dos casos pediátricos con diferente abordaje quirúrgico / Giant cystic lymphatic malformation of the liver: two pediatric cases with different surgical approach

Introducción. Las malformaciones linfáticas quísticas, también llamadas linfangiomas quísticos, aparecen muy raramente de forma aislada en el hígado. Casos clínicos. Se presentan dos pacientes femeninas de edad preescolar con marcada hepatomegalia, dependiente de lesiones quísticas multitabicadas, secundarias a malformación linfática quística gigante del hígado, que fueron tratadas en el Hospital Pediátrico Universitario William Soler, La Habana, Cuba. Resultados. En ambos casos el diagnóstico se apoyó en los estudios de imágenes, la laparoscopia y el análisis histopatológico. En un caso el tratamiento fue la hepatectomía derecha, mientras que en el otro se empleó la escleroterapia, ambas con evolución favorable. Conclusión. A pesar de su rareza, este diagnóstico no debe obviarse ante un paciente pediátrico con lesiones hepáticas quísticas. El tratamiento de elección es la resección quirúrgica, pero su indicación y envergadura debe valorarse de forma individualizada

Introduction. Cystic lymphatic malformations, also called cystic lymphangiomas, are very rarely found in the liver. Clinical cases. Two pediatric female preschool-age patients. presented with hepatomegaly due to multi-septated cystic lesions of the liver, who received treatment at Hospital Pediátrico Universitario William Soler, La Habana, Cuba. Results. We report two pediatric cases with giant cystic lymphatic malformation of the liver. In both cases, the diagnosis were based on imaging, laparoscopy and pathology. In one case the treatment was right hepatectomy, whereas in the other, sclerotherapy was performed, both with a favorable outcome. Conclusion. Despite its rarity, this diagnosis should be considered in pediatric patients with hepatic cystic lesions. The recommended treatment is surgical resection, but its indication and extent should be assessed individually for each patient.

An innovative airway management of an anticipated difficult airway in a pediatric patient with cervical cystic hygroma: A case report

@#Airway management of a pediatric patient with cervical cystic hygroma can be challenging as the large neck mass may extend in the oral cavity, result in tracheal deviation, and cause possible upper airway obstruction. This is a case of a 4-year-old female admitted due to sudden enlargement of neck mass associated with dysphagia and sialorrhea. Patient was intubated under sedation while maintaining spontaneous ventilation with a pediatric flexible fiberoptic scope through a nasopharyngeal airway serving as a passage guide for ease of scoping and a protective device against trauma.

Use of sirolimus and sclerotherapy for lymphatic malformations in a developing country: a case series / Uso de sirolimus y escleroterapia para malformaciones linfáticas en un país en vías de desarrollo: una serie de casos

Las malformaciones linfáticas y su manejo no han sido bien descritas en República Dominicana. Es por ello, que el objetivo de este artículo es la presentación de tres casos, con diferentes patrones y necesidades de tratamiento, de modo que sirva como referencia para trabajadores de la salud en países en vías de desarrollo.

Lymphatic malformations and its management are not well described in the Dominican Republic. That is why this article's objective is to present 3 cases, with different patterns and treatment needs, so it will work as a reference for healthcare workers in developing countries.

Revisión de la conducta quirúrgica del linfangioma quístico / Review of cystic lymphangioma´s surgical behavior

Introducción: El linfangioma quístico es un tumor benigno infrecuente del sistema linfático que afecta habitualmente a los infantes. La opción terapéutica ideal es la extirpación quirúrgica. Objetivo: Sistematizar contenidos esenciales relacionados con el diagnóstico y tratamiento del linfangioma quístico. Métodos: Se realizó la búsqueda y el análisis de la información en un período de 10 años (2010-2020). Se emplearon las palabras clave: "linfangioma quístico" y "malformación linfática congénita quística", en español e inglés. Se hizo la revisión bibliográfica en un total de 62 artículos publicados en las bases de datos y bibliotecas electrónicas científicas de la salud: PubMed, Google Académico, Pubmed, Medline, Biblioteca Virtual en Salud, LILACS y SciELO, mediante el gestor de búsqueda y administrador de referencias EndNote; de ellos se seleccionaron 50 relevantes para el objetivo de la revisión. Resultados: La información se estructuró en los siguientes aspectos: sinonimia y antecedentes históricos, epidemiología, clasificación, patogenia, bases esenciales para el diagnóstico, tratamiento, complicaciones y pronóstico. Se identificaron controversias en cuanto a la terapéutica, y se mostraron las imágenes de los infantes diagnosticados y tratados por la autora en su colaboración médica en Angola en 2018. Conclusiones: Se sistematizan las bases esenciales para el diagnóstico y tratamiento del linfangioma quístico para que el cirujano general que, en su desempeño profesional, brinda asistencia médico quirúrgica a infantes fuera de Cuba, se empodere de las especificidades de este tumor linfático. También se revela como factible su resección quirúrgica íntegra para evitar recidivas, discapacidad y mejorar la calidad de vida del afectado(AU)

Introduction: Cystic lymphangioma is a rare benign tumor of the lymphatic system that usually affects infants. The ideal therapeutic option is surgical removal. Objective: Systematize essential contents related to the diagnosis and treatment of cystic lymphangioma. Methods: The search and analysis of information was carried out over a period of 10 years (2010-2020). The keywords "cystic lymphangioma" and "cystic congenital lymphatic malformation" were used in Spanish and English. The bibliographical review was carried out in a total of 62 articles published in the databases and electronic scientific health libraries: PubMed, Google Scholar, Pubmed, Medline, Virtual Library in Health, LILACS and SciELO, through the search and reference manager called EndNote; of these, 50 that were important to the objective of the review were selected. Results: The information was structured in the following aspects: synonym and historical backgrounds, epidemiology, classification, pathogenesis, essential bases for diagnosis, treatment, complications and prognosis. Therapeutic disputes were identified, and images of infants diagnosed and treated by the author in her medical collaboration in Angola in 2018 were shown. Conclusions: The essential bases for the diagnosis and treatment of cystic lymphangioma are systematized, so that the general surgeon who, in his-her professional performance, provides surgical medical assistance to infants outside Cuba is informed on the specificities of this lymphatic tumor. Its full surgical resection is also revealed as feasible to prevent recurrence, disability and to improve the quality of life of the affected person(AU)

Reporte de Caso Clínico: Linfangioma quístico de epiplón / Case Report: Cystic lymphangioma of the omentum

INTRODUCCIÓN: El linfangioma mesentérico es un tumor quístico congénito, benigno y raro de los vasos linfáticos, que se presenta en menos del 5% de los casos a nivel abdominal. Se presentan con mayor frecuencia durante la infancia. Esta patología se debe considerar como diagnóstico diferencial de abdomen agudo y masas abdominales pediátricas. CASO CLÍNICO: Niño de 6 años de edad, presentó cuadro de dolor abdominal y peritonismo. Presentó febrícula, signo de rebote positivo, además leucocitosis y neutrofilia. Ecografía evidenció abundante líquido libre en abdomen y pelvis. EVOLUCIÓN: Se realizó laparotomía exploratoria, con hallazgo de masa quística dependiente de epiplón mayor, la misma que se resecó. La histopatología fue negativa para malignidad y compatible con linfangioma quístico, la inmunohistoquímica con marcador D2-40 positi-vo. El paciente evolucionó favorablemente sin complicaciones quirúrgicas y sin evidencia de recurrencia hasta el año de seguimiento. CONCLUSION: El linfangioma quístico mesentérico puede debutar con sintomatología de abdomen agudo, puede también tener una evolución crónica. La resección completa es el tratamiento de elección, actualmente se realizan procedimientos mínimamente invasivos, con resultados favorables. La ecografía es útil para realizar un seguimiento a largo plazo.(AU)

BACKGROUND: Mesenteric lymphangioma is a rare, benign congenital cystic tumor of the lym-phatic vessels, which occurs in less than 5% pf the cases at the abdomen. They appear most often during childhood. This tumor should be considered as a differential diagnosis of acute abdomen and other abdominal masses. CASE REPORT: A 6-year-old boy presented with abdominal pain and peritonism. He presented a low-grade fever, a positive rebound sign, as well as leukocytosis and neutrophilia. Ultrasono-graphy showed abundant free fluid in abdomen and pelvis. EVOLUTION: Exploratory laparotomy was performed, finding a cystic mass dependent on the greater omentum, this mass was resected. Histopathology was negative for malignancy and compatible with cystic lymphangioma, immunohistochemistry was positive for D2-40 marker. The patient evolved favorably without surgical complications and without evidence of recurrence up to one year of follow-up. CONCLUSION: Mesenteric cystic lymphangioma can present with symptoms of acute abdomen, it can also have a chronic evolution. Complete resection is the treatment of choice, currently minimally invasive procedures are performed with favorable results. Ultrasound is useful for long-term follow-up.(AU)

A well-orchestrated EXIT strategy: A case of a successful ex utero intrapartum treatment procedure for Cystic Hygroma / Philippine Journal of Obstetrics and Gynecology

@#A fetal neck mass such as cystic hygroma imposes a challenge in the continuing prenatal care and birth strategy for a 26-year-old gravida 2 para 1 (1001) with a scarred uterus from previous low transverse cesarean section (CS). An intricate delivery for the fetus with a potential airway obstruction necessitates an ex utero intrapartum treatment (EXIT) delivery. The patient was initially scheduled to undergo the procedure at 38 weeks of gestation, but preterm labor ensued. Close antenatal monitoring was done which carried the pregnancy to term. The multidisciplinary approach and planning requires consensus among varied specialties as this maternal–fetal surgery differs immensely from a regular CS. This aims to document and discuss the preoperative, operative and postoperative management done for this patient. The EXIT procedure was performed at 38 weeks' age of gestation with good maternal outcomes. The fetus was delivered safely.

Fístula quilosa posoperatoria / Postoperative chylous fistula

Introducción: La fístula quilosa posoperatoria debida a lesión iatrogénica del conducto torácico es una complicación infrecuente y grave de la cirugía de cabeza y cuello. Objetivo: Describir las opciones de tratamiento de la fístula quilosa cervical posquirúrgica. Caso clínico: Se presenta un paciente de 18 años de edad con diagnóstico de linfangioma quístico de la región lateral izquierda del cuello, intervenido en el Servicio de Cirugía General del Hospital "Mnazi Mmoja", de Zanzíbar, Tanzania. Durante la evolución posoperatoria se constató una fístula quilosa de bajo débito la cual resolvió mediante tratamiento médico. El enfermo egresó curado a los 28 días después de la intervención quirúrgica. Conclusión: El tratamiento conservador es efectivo en la mayoría de las fístulas quilosas cervicales de bajo débito, mientras que en las de débito alto la cirugía ofrece una rápida resolución, aunque no existe consenso en torno al momento ideal para realizarla(AU)

Introduction: Postoperative chylous fistula due to iatrogenic thoracic duct injury is an infrequent and serious complication of head and neck surgery. Objective: To describe the treatment options of postoperative cervical chylous fistula. Clinical case: It was presented an 18-year-old patient with diagnosis of cystic lymphangioma of the left lateral region of the neck, which was removed in the General Surgery Service of the National Hospital Mnazi Mmoja of Zanzibar, Tanzania. During the postoperative evolution, a low-output chylous fistula was found and resolved by medical treatment. The patient withdrew cured at 28 days after the surgical intervention. Conclusion: Conservative treatment is effective in the majority of low-out put cervical chylous fistulas, while in high-debit, surgery offers a rapid resolution, although there is no consensus about the ideal time to perform it(AU)

Linfangioma quístico de recto-sigmoides en un hospital público de Lima, Perú: reporte de caso / Cystic lymphangioma of the rectum-sigmoid in a public hospital in Lima-Peru: case report

RESUMEN Los linfangiomas son lesiones benignas que se deben a una malformación del desarrollo en el sistema linfático que ocurre durante la etapa embrionaria. Son más frecuentes en niños. Pueden encontrarse en cavidad abdominal, siendo la afectación del tracto gastrointestinal en forma ocasional. La presentación clínica es variada, dependiendo de la localización de los linfangiomas, pudiendo tener formas asintomáticas y, en ocasiones, presentarse con dolor abdominal, alteraciones del hábito defecatorio, rectorragia, etc. El diagnóstico se realiza por endoscopía, imágenes auxiliares y se confirma por medio de histología. Presentamos un caso de linfangioma quístico de recto-sigmoides que tuvimos en nuestro hospital; la paciente se presentó con proctalgia, alternancia evacuatoria y rectorragia intermitente. Examen físico sin alteraciones significativas. Durante la colonoscopía, a nivel de recto y sigmoides, se encontró múltiples lesiones protruidas, a modo de protuberancias, cuya superficie era lisa, trasluciente y de coloración azulada, con algunos orificios pseudodiverticulares, a predominio de recto. En la ultrasonografía endoscópica se observó, a nivel del recto, engrosamiento de la submucosa con múltiples imágenes anecoicas, microquísticas, algunas de ellas con tabiques finos. El diagnóstico definitivo se realizó mediante histopatología, que describe conductos linfáticos dilatados, rodeados de células endoteliales, hallazgos consistentes con linfangioma quístico de recto-sigmoides.

ABSTRACT Lymphangiomas are benign lesions due to a developmental malformation in the lymphatic system that occurs during the embryonic stage. They are more frequent in children. They can be found in the abdominal cavity, being the involvement of the gastrointestinal tract occasionally. The clinical presentation is varied, depending on the location of the lymphangiomas, and may have asymptomatic forms and, occasionally, present with abdominal pain, changes in defecation, rectal bleeding, etc. The diagnosis is made by endoscopy, auxiliary images and is confirmed by histology. We present a case of cystic rectal-sigmoid lymphangioma that we had in our hospital; the patient presented with proctalgia, alternating evacuation and intermittent rectal bleeding. Physical examination was without significant alterations. During the colonoscopy, at the level of the rectum and sigmoids, multiple protruded lesions were found, whose surface was smooth, translucent and bluish in color, with some pseudodiverticular holes, predominantly of the rectum. Endoscopic ultrasound revealed thickening of the submucosa at the level of the rectum with multiple anechoic, microcystic images, some of them with fine septa. The definitive diagnosis was made by histopathology, which describes dilated lymphatic ducts, surrounded by endothelial cells, findings consistent with cystic rectal-sigmoid lymphangioma.

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.

Tratamiento quirúrgico de malformaciones linfáticas en pacientes pediátricos / Surgical treatment for lymphatic malformations in children

Introducción: Los linfagiomas quísticos son malformaciones de los conductos linfáticos los cuales forman cavidades con líquido linfático. Objetivos: Determinar las características clínicas, evolución y complicaciones de los pacientes operados de malformaciones linfáticas localizados en la región de la cabeza y el cuello. Diseño: Estudio observacional descriptivo tipo serie de casos. Metodología: Se revisaron las historias clínicas de los pacientes operados con resultado de patología compatible con malformaciones linfáticas, en el periodo desde enero 2008 hasta enero del 2017. Resultados: Se encontraron 254 casos que requirieron cirugía, con una media de edad de 1 año y promedio de 2.9 años. La región cervical tuvo la mayor presentación (77.6%) especificamente en la localización supra e infrahioidea unilateral (30.3%). La aparición de tumoración fue el signo más frecuente (98.8%). Se encontraron 5.9% de recidivas para el tratamiento quirúrgico y 8.2% de casos con paresia o parálisis del nervio submandibular como secuelas. 10 casos requirieron traqueostomía y tres pacientes fallecieron por complicaciones posteriores a la cirugía. Conclusiones: El tratamiento quirúrgico está indicado para los linfangiomas quísticos, minimizando la morbilidad en el paciente y evitando las recidivas futuras.

Introduction: Cystic lymphomas are malformations of the lymphatic ducts, which form cavities with lymphatic fluid. Objective: To determine the clinical characteristics, evolution and prognosis of patients undergoing surgery due to lymphatic malformations located in the head and neck. Design: Case series Methods: The medical records of patients who underwent a lymphatic malformation resection at The Head, Neck and Maxillofacial Surgery Unit, who had a pathological analysis compatible with lymphatic malformations, during the period from January 2008 to January 2017. Results: We found 254 cases that required surgery, with a median age of 1 year and an average of 2.9 years. The cervical region had the highest presentation (77.6%) specifically in the unilateral supra and infrahyoid location (30.3%). The appearance of a tumor was the most frequent sign (98.8%). 5.9% of recurrences were found for surgical treatment and 8.2% of cases with paresis or paralysis of the submandibular nerve as sequelae. 10 cases required tracheostomy and 3 patients died due to complications after surgery. Conclusions: Surgical treatment is indicated for cystic lymphangiomas. It minimizes morbidity and prevents recurrences.

Higroma quístico fetal / Fetal cystic hygroma

El higroma quístico congénito o linfangioma es un tumor del sistema linfático, es de origen embrionario y se origina por la obstrucción del drenaje de los sacos linfáticos. Su localización en orden de frecuencia es en las regiones cervical, axilar, intraperitoneal e inguinal, y muy raro como alteración única en el mediastino anterior. Paciente de 32 años de edad, con embarazo 16 sem ± 6 días y reporte de ecografía: Embarazo de 15 sem ± 20 días, normohidramnia, higroma quístico, Ausencia de hueso nasal además de una comunicación interventricular. Paciente en sala de legrado bajo sedación profunda, se obtiene producto de sexo masculino con alteraciones faciales y aumento de volumen en región cervical. Podemos concluir que, el control prenatal es importante realizar para obtener un diagnóstico precoz con examen ecográfico seriado, estudio genético, ecocardiografía fetal y para dar tratamiento quirúrgico es necesario conformar un equipo multidisciplinario.(AU)

The congenital cystic hygroma or lymphangioma is a tumor of the lymphatic system, is of embryonic origin and is caused by the obstruction of the drainage of the lymphatic sacs. Its location in order of frequency is in the cervical, axillary, intraperitoneal and inguinal regions, and very rare as a single alteration in the anterior mediastinum. A 32-year-old patient with a pregnancy of 16 weeks ± 6 days and an ultrasound report: Pregnancy of 15 weeks ± 20 days, normohydramnia, cystic hygroma, Absence of nasal bone in addition to interventricular communication. Patient in curettage room under deep sedation, male product with facial alterations and volume increase in cervical region is obtained. We can conclude that prenatal control is important to obtain an early diagnosis with serial ultrasound examination, genetic study, fetal echocardiography and to give surgical treatment it is necessary to form a multidisciplinary team.(AU)

Cystic lesion of pectoralis minor muscle: learning from mistakes

Hydatidosis is a frequent infestation in large endemic areas, caused by helminths. Primary localization within the muscle or bone tissues is rare. We report the case of a 52-year-old woman with a cystic lesion located in the right pectoralis minor muscle, who was initially diagnosed with cystic lymphangioma by imaging examination. She was submitted for surgical treatment; in block resection of the tumor along with the involved muscle was performed. The histopathological diagnosis was of hydatid cyst. The contribution of the ancillary lab tests is analyzed for a precise preoperative diagnostic approach. This case well illustrates that the most likely is not always what it appears to be. Facing of a cystic lesion in the lungs, liver or muscle, clinicians should always think on hydatid disease, particularly in endemic areas.

Prognosis of fetuses with cystichygroma and nuchal translucency/nuchal fold thickening on prenatal echography / 浙江大学学报·医学版

OBJECTIVE@#To analyze the prognosis of fetuses with cystic hygroma (CH) or nuchal translucency (NT) or nuchal fold (NF) thickening detected by prenatal echography.@*METHODS@#From January 2014 to December 2015, 124 fetuses with CH and NT/NF thickening on prenatal echography were enrolled from Women's Hospital of Zhejiang University School of Medicine. The basic clinical information, ultrasonic results, pregnancy outcomes and newborn follow-ups were analyzed. The cases were grouped by prognosis and the factors affecting prognosis were analyzed with logistic regression.@*RESULTS@#There were 85 cases of labor induction including one stillbirth and 39 cases delivered. Except one infant who died after birth, all live births survived with good prognosis. Univariate analysis showed that the gestational age at diagnosis of poor prognosis group was earlier than that of good prognosis group (<0.01); and the former group also had higher hydrops fetalis rate and additional structural anomalies rate (all <0.01). Multivariate regression analysis showed that hydrops fetalis (=90.105, <0.05) and additional structural anomalies (=61.854, <0.05) were risk factors of poor prognosis in fetuses with CH and NT/NF thickening.@*CONCLUSIONS@#Fetuses with diagnosed CH or NT/NF thickening on prenatal ultrasonography are likely to be associated with chromosomal abnormality. Early gestational weeks, hydrops fetalis and additional structural anomalies may indicate poor prognosis.

Incidentally found cystic lymphangioma of the adrenal gland in an elderly male cadaver / 영남의대학술지

Adrenal cystic lymphangiomas are extremely rare entities that are often identified incidentally, with less than 60 cases reported to date. We found a protruding ovoid mass consisting of a multiloculated cystic lesion within right adrenal gland in the cadaver of a 75-year-old Korean man. The epithelial cells lining the adrenal cyst were diffusely positive for cluster of differentiation 31 and podoplanin, and negative for pan-cytokeratin. The histopathological diagnosis confirmed a cystic lymphangioma arising from the adrenal gland. Post-mortem findings of the present case are discussed based on the clinicopathological features of adrenal cystic lymphangiomas.

Cutaneous Lymphangioma Circumscriptum Occurred Seven Years after Surgery with Soft Tissue Cystic Lymphangioma

Lymphangiomas are rare benign congenital lymphatic malformations. They can be divided into three groups: cutaneous lymphangioma circumscriptum (CLC), cavernous, and cystic. CLC is the most common type, and rarely occurs with cavernous or cystic lymphangioma under the lesion. Here, we describe the case of a 9-year-old girl who presented with an asymptomatic vesicular lesion on her back. She was finally diagnosed with CLC by clinical manifestations, dermoscopic findings, and histologic findings. Seven years ago, there was a history of surgical operation in the department of general surgery, and the surgery was soft tissue cystic lymphangioma removal surgery. There was no skin lesion for seven years after surgery, but one occurred a month ago. Herein, we present a case of CLC that occurred after a long interval after surgery for soft tissue cystic lymphangioma.

Application of chromosomal microarray analysis in prenatal diagnosis of fetuses with increased nuchal translucency / 中华医学遗传学杂志

OBJECTIVE@#To determine the frequency of chromosomal abnormalities and outcome of pregnancy for fetuses with increased nuchal translucency (NT).@*METHODS@#Between July 2014 and February 2018, 247 fetuses with increased NT (>95th centile)were analyzed by chromosome microarray analysis (CMA). The fetuses were divided into ones with isolated increased NT (168 cases), increased NT with cystic hygroma (20 cases), increased NT with edema (12 cases) or increased NT with other abnormalities (47 cases). All couples were followed up by telephone calls.@*RESULTS@#The rate of chromosomal abnormalities was 31.6% (78/247), which included 66 cases with chromosomal aneuploidies and 12 with copy number variants (CNVs). CNVs accounted for 31.4% (11/35) of total abnormalities among fetuses with isolated increased NT, whilst only 2.3% (1/43) of the total abnormalities among fetuses with non-isolated increased NT. Three fetuses with a normal CMA result had mental and physical retardation. Two of them were diagnosed with single gene disorders by whole exome sequencing.@*CONCLUSION@#CMA can detect more chromosomal microdeletion/microduplications among fetuses with isolated increased NT. Furthermore, fetuses with increased NT and anegative CMA result during pregnancy cannot exclude all adverse outcomes.

Linfagioma quístico mesentérico en el diagnóstico diferencial de la enfermedad in-flamatoria pélvica / Mesenteric cystic lymphangioma in the differential diagnosis of pelvic inflammatory disease

RESUMEN La enfermedad inflamatoria pélvica (EIP) es un síndrome clínico que incluye todas aquellas alteraciones inflamatorias e infecciosas que comprenden los órganos de la pelvis menor. La infección es habitualmente polimicrobiana y está asociada a mujeres jóvenes con vida sexual activa, nulíparas y usuarias de dispositivo intrauterino (DIU Los abscesos tubo-ováricos son una complicación aguda o crónica, asociada a dicho proceso.). CASO CLÍNICO Presentamos el caso de una mujer de 47 años con dolor abdominal intenso, usuaria de DIU. La primera sospecha diagnóstica fue de EIP con presencia de absceso tuboovárico anexial. Sin embargo la exploración física no orientó en este sentido, por lo que se solicitó TAC abdominopélvico, en el que se informó de la posibilidad de linfangioma quístico mesentérico. Por ello, se contactó con el servicio de Cirugía General que procedió a su resección satisfactoria y sin incidencias. El estudio anatomopatológico confirmo la sospecha radiológica. CONCLUSIÓN Una adecuada exploración física es fundamental en el diagnóstico de la EIP y el absceso tuboovárico. El linfangioma quístico mesentérico puede formar parte del diagnóstico diferencial de las masas anexiales en este contexto.

ABSTRACT Pelvic inflammatory disease (PID) is a clinical syndrome involving all those inflammatory and infectious alterations affecting the minor pelvis organs. Any infection is often multibacterial and more frequent in sexually active nulliparous young women and intrauterine device (IUD) users. Tuboovarian abscesses can be an acute or chronic complication associated to that process. Our patient was a 47 year old IUD user consulting about intense abdominal pain. The initial clinical suspicion pointed at a case of PID associated to a tubo-ovarian anexial abscess. However, a physical examination did not support this suspicion and an abdominopelvian CAT scan was therefore requested, reporting a possible mesenteric cystic lymphangioma. The General Surgery service was reached for treatment, successfully excising the growth without further incident. Histological analysis confirmed the radiological diagnosis. An adequate physical examination is instrumental while diagnosing PID and tuboovarian abscesses. A mesenteric cystic lymphangioma should be included in a differential diagnosis of anexial growths in such a context