Outcomes and bioethical implications of lymphadenectomy in mastectomized patients: Importance and indication of ultrasound-guided fine-needle aspiration biopsy / Resultados e implicaciones bioéticas de la linfadenectomía en pacientes mastectomizadas: Importancia y recomendación de biopsia por aspiración con aguja fina guiada por ecografía / Resultados e implicações bioéticas da linfadenectomia em pacientes mastectomizadas: Importância e recomendação da biópsia aspirativa com agulha fina guiada por ultrassom

Introduction: Lymph node involvement is the main prognostic factor in breast cancer. Mastectomized patients usually undergo lymphadenectomy (LA) of micrometastatic sentinel lymph nodes (SLNs) despite the evidence of AMAROS trial to replace this therapy with radiotherapy in select cases. Objective: Demonstrate the ability of ultrasonography to detect non-metastatic or micrometastatic SLNs. Materials and methods: 132 patients who underwent mastectomy were evaluated. Ultrasound-guided fine-needle aspiration biopsy (FNAB) was indicated for suspicious lymph nodes. LA and SNL biopsy (SLNB) were performed in patients with positive and negative FNAB, respectively. LA was performed in FNAB positive or SLNB positive cases, except in the presence of isolated tumor cells and micrometastatic SLNs. The tumor burden after LA in patients with negative FNAB and positive SLNB was measured; the presence of two or fewer positive SLNs was considered a low burden. Results: Sensitivity of FNAB for detecting positive lymph nodes in patients with a high tumor burden was 93% and specificity was 84%. Positive (PPV) and negative predictive value (NPV) were 60% and 79%, respectively. Conclusions: LA could have been avoided in 90% of mastectomized patients with negative FNAB and a low tumor burden who met the AMAROS criteria with a high NPV (79%).

Introducción: La afectación ganglionar es el principal factor pronóstico en cáncer de seno. Generalmente, pacientes mastectomizadas se somenten a linfadenectomia (LA) de ganglios linfáticos centinela micrometastásicos (GLCs), a pesar de la evidencia del ensayo AMAROS en ciertos casos para reemplazarla con radioterapia. Objetivo: Demostrar la importancia de la ecografía para detectar GLCs no metastásicos o micrometastásicos. Materiales y métodos: Se evaluaron132 pacientes sometidas a mastectomía. Se recomendó biopsia aspirativa con aguja fina (BAAF) por ultrasonido para ganglios linfáticos sospechosos. Se realizó Biopsia LA y biopsia de GLCs (BGLC) en pacientes con BAAF positiva y negativa, respectivamente. En casos positivos de BAAF o BGLC se ejecutó LA, excepto en presencia de células tumorales aisladas y GLCs. Se evaluó la carga tumoral posterior a LA en pacientes con BAAF negativa y BGLC positiva. La presencia de dos o menos GLC positivos se consideró carga baja. Resultados: La sensibilidad de BAAF para detectar nódulos linfáticos positivos en pacientes con alta carga tumoral fue del 93%; la especificidad fue del 79%. Valores predictivos positivo (60%) y negativo (79%). Conclusiones: Se podría haber evitado LA en 90% de pacientes mastectomizadas con BAAF negativa y baja carga tumoral que cumplían criterios AMAROS con alto VPN (79%).

Introdução: O comprometimento dos gânglios é o principal fator prognóstico no câncer de mama. Geralmente, pacientes mastectomizadas são submetidas a linfadenectomia (LA) de gânglios linfáticos sentinelas de micrometástases (GLSs), apesar da evidência do estudo AMAROS em certos casos para substituí-la por radioterapia. Objetivo: Demonstrar a importância da ultrassonografia na detecção de GLSs não metastáticos ou micrometástase. Materiais e métodos: Foram avaliadas 132 pacientes submetidas à mastectomia. A biópsia aspirativa com agulha fina (BAAF) ultrassônica foi recomendada para gânglios linfáticos suspeitos. A biópsia LA e a biópsia do GLSs (BGLS) foram realizadas em pacientes com BAAF positivo e negativo, respectivamente. Nos casos positivos de BAAF ou BGLS, a LA foi realizada, exceto na presença de células tumorais isoladas e GLSs. A carga tumoral após a LA foi avaliada em pacientes com BAAF negativa e BGLS positiva. A presença de dois ou menos GLS positivos foi considerada carga baixa. Resultados: A sensibilidade do BAAF para detectar linfonodos positivos em pacientes com alta carga tumoral foi de 93%; a especificidade foi de 79%. Valores preditivos positivos (60%) e negativos (79%). Conclusões: a LA poderia ter sido evitada em 90% das pacientes mastectomizadas com BAAF negativa e baixa carga tumoral que preencheram os critérios AMAROS com alto VPN (79%).

Síndrome da Rede Axilar após Tratamento para Câncer de Mama: Revisão Sistemática com Metanálise / Occurrence and Risk Factors with Axillary Network Syndrome after Breast Cancer Treatment: Systematic Review with Meta-analysis / Ocurrencia y Factores Asociados con el Síndrome de la Red Axilar después del Tratamiento del Cáncer de Mama: Revisión Sistemática con Metaanálisis

Introdução: O câncer de mama é o mais incidente nas mulheres e a cirurgia é o principal tratamento de escolha. A síndrome da rede axilar (SRA) é uma condição recorrente que ocorre em até 86% das pacientes após cirurgia, se apresenta como um único cordão ou múltiplos cordões nos tecidos subcutâneos da axila ipsilateral e gera dor e limitação do movimento. Objetivo: Investigar estudos sobre a ocorrência e fatores associados à SRA após tratamento do câncer de mama. Método: Revisão sistemática com metanálise, nas bases de dados PubMed, LILACS e EMBASE, com a metodologia PECOS, seguindo a diretriz PRISMA. Resultados: Cinco artigos foram selecionados, com taxa de ocorrência da SRA de 35%. Os fatores associados encontrados apresentaram um menor risco relativo (RR) de recorrência para quem realizou biópsia de linfonodo sentinela em comparação aos que se submeteram à dissecção axilar (RR 0,49; IC 95% [0,42; 0,57] I²=95%, p=0,01). Houve uma variação de 35% a 39% de desenvolvimento para a SRA em pacientes que se submeteram à quimioterapia e radioterapia, porém o tipo de cirurgia não teve resultado estatisticamente significativo para o desencadeamento da síndrome. Conclusão: Indivíduos que realizaram biópsia de linfonodo sentinela têm menos chance de desenvolver a SRA quando comparados aos que fizeram dissecção axilar. As terapias oncológicas apresentaram proporções parecidas de aparecimento da SRA e o tipo de cirurgia não interferiu na evolução da patologia.

Introduction: Breast cancer is the most common in women and surgery is the main treatment of choice. Axillary Web Syndrome (AWS) is a recurrent condition that occurs in up to 86% after surgery, presenting as a single cord or multiple cords in the subcutaneous tissues of the ipsilateral axilla, causing pain and limitation of movement. Objective: To investigate studies about the occurrence and factors associated with AWS post breast cancer treatment. Method: Systematic review with meta-analysis based in the PECOS methodology according to PRISMA guidelines at the databases PubMed, LILACS and EMBASE. Results: Five articles were selected with rate of occurrence of 35% of AWS. The associated factors found showed a minor relative risk (RR) of recurrence in those who underwent sentinel lymph node biopsy compared to those who were submitted to axillary dissection (RR 0.49; 95%CI [0.42;0.57] I²=95%, p=0.01). The appearance of AWS ranged from 35% to 39% in patients who submitted to chemotherapy and radiotherapy, but the type of surgery did not have a statistically significant result for triggering the syndrome. Conclusion: Individuals who underwent sentinel lymph node biopsy are less likely to develop the syndrome when compared to those who submitted to axillary dissection. Oncological therapies had similar percentages for the appearance of AWS and the type of surgery did not interfere in the evolution of the pathology.

Introducción: El cáncer de mama es el más común en las mujeres e la cirugía es considerada el tratamiento de elección. El síndrome de la red axilar (SRA) es una condición recurrente que ocurre hasta en un 86% de las pacientes después de la cirugía, se presenta como un cordón único o múltiples cordones en los tejidos subcutáneos de la axila isolateral, y causa dolor y limitación del movimiento. Objetivo: Investigar estudios sobre la ocurrencia y factores asociados al SRA después del tratamiento del cáncer de mama. Método: Revisión sistemática con metaanálisis, en las bases de datos PubMed, LILACS y EMBASE, con la metodología PECOS, siguiendo la guía PRISMA. Resultados: Se seleccionaron cinco artículos, con la tasa de ocurrencia del SRA del 35%. Los factores asociados encontrados mostraron un menor riesgo relativo (RR) de recurrencia para quien realizó biopsia de ganglio centinela en comparación con las que se sometieron a la disección axilar (RR 0,49; IC 95% [0,42;0,57] I²=95%, p=0,01). Hubo una variación del 35% al 39% de desarrollo del SRA en pacientes que se sometieron a la quimioterapia y radioterapia, aunque el tipo de cirugía no tuvo un resultado estadísticamente significativo para desencadenar el síndrome. Conclusión: Las personas que se sometieron a una biopsia de ganglio centinela tienen menos probabilidades de desarrollar el SRA en comparación con aquellas que se sometieron a disección axilar. Las terapias oncológicas presentaron proporciones parecidas de aparición del SRA y el tipo de cirugía no interfirió en la evolución de la patología.

Cervical lymph nodes in rheumatology: A diagnostic dilemma

The case is presented on a 52-year-old male patient, who was seen in the Rheumatology department. He had painless lymph nodes in the cervical, axillary, supraclavicular, and neck region. He also had a fever, and parotid and submaxillary gland enlargement. Complementary studies were performed, showing normocytic-normochromic anemia, thrombocytopenia and eosinophilia, impaired renal function with hypoalbuminaemia and hematuria, ANA 1/5120, Sm+, ACL+. Biopsies were also performed on the compromised tissues, reaching the diagnosis of Rosai-Dorfman Disease and IgG4-related Disease. Differential diagnoses of cervical, axillary and inguinal lymph nodes, with fever, renal and hematological compromise are discussed.

Se describe el caso de un paciente varón de 52 años que consulta al servicio de reumatología por presentar adenopatías indoloras en las regiones cervical, axilar, supraclaviculares y en la nuca, así como fiebre, aumento de tamaño de parótidas y submaxilares. Se realizan estudios complementarios que arrojan como resultado anemia normocítica-normocrómica, trombocitopenia y eosinofilia, alteración de la función renal con hipoalbuminemia y hematuria, FAN 1/5.120, Sm+, ACL+ y biopsia de los tejidos comprometidos, por lo que se arriba al diagnóstico de enfermedad de Rosai-Dorfman y enfermedad relacionada con IgG4. Se discuten diagnósticos diferenciales de adenopatías cervicales, axilares e inguinales, fiebre, compromiso renal y hematológico.

Bone sarcoidosis: an unusual manifestation / Sarcoidosis ósea: una manifestación inusual

ABSTRACT Sarcoidosis is a multi-system disease that involves the lung in 90% of cases. Skeletomuscular involvement of sarcoidosis may involve joints, muscles and/or bones, the latter affecting 313% of patients. Its clinical presentation is asymptomatic in half of the cases, in the other half there is pain, soft tissue edema, and decreased limb function. Radiographically it is accompanied by osteolysis, osteosclerosis, cystic lesions and pathological fractures in advanced stages. A final diagnosis is made by tissue biopsy, finding non-caseating granulomatous process accompanied by Langhans giant cells. The main indication of medical management is the control of symptoms, associated with an improvement in the functioning of the affected limb and quality of life of the patient. The case is presented of a patient with digital sarcoidosis with classic radiographic pattern with no other extra-skeletal involvement.

RESUMEN La sarcoidosis es una enfermedad multisistémica que involucra en el 90% de los casos el pulmón. El compromiso osteomuscular de la sarcoidosis puede incluir articulaciones, músculos o huesos; este último caso afecta al 3-13% de los pacientes. Su presentación clínica es asintomática en la mitad de los casos, el restante se presenta con dolor, edema de tejidos blandos y disminución en la funcionalidad de la extremidad. Radiográficamente se acompaña de osteólisis, osteoesclerosis, lesiones quísticas y fracturas patológicas en estadios avanzados. Su diagnóstico definitivo se realiza por medio de una biopsia tisular, en la que se encuentra un proceso granulomatoso no caseificante acompañado de células gigantes de Langhans. La principal indicación del manejo médico es el control de los síntomas, lo que se asocia con una mejoría en el funcionamiento de la extremidad afectada y en la calidad de vida del paciente. Se presenta el caso de un paciente con sarcoidosis digital con patrón radiográfico clásico, sin otro compromiso extraesquelético.

Malformaciones linfáticas abdominales en una población pediátrica: experiencia en un centro de referencia de Medellín, Colombia / Abdominal lymphatic malformations in a pediatric population: Experience in a referral center in Medellín, Colombia

Introducción. Los linfangiomas son anormalidades benignas del sistema linfático, que corresponden a dilataciones quísticas de estos vasos y se localizan especialmente en el cuello. Solo el 10 % de todas estas malformaciones se encuentran en el abdomen y presentan síntomas variables de acuerdo al tamaño y su ubicación especifica, siendo el dolor abdominal el principal síntoma. Métodos. Se presentan cinco pacientes pediátricos con malformaciones linfáticas abdominales. Se describen su cuadro clínico, localización, tratamiento y la experiencia en el manejo de dicha patología en un hospital de referencia. Resultados. Los métodos más apropiados para hacer una aproximación diagnóstica son la ecografía, la tomografía computarizada y la resonancia nuclear magnética. Dentro de las opciones descritas para el tratamiento están la farmacológica, la escleroterapia y la resección quirúrgica, tanto por vía abierta como por laparoscopia. Conclusión. Existe una variedad de métodos para realizar la resección de los linfangiomas abdominales, pero la cirugía sigue siendo la más efectiva, especialmente cuando se cuenta con la laparoscopia como una herramienta terapéutica.

Introduction. Lymphangiomas are benign abnormalities of the lymphatic system, which correspond to cystic dilations of these vessels and are located especially in the neck. Only 10% of all these malformations are found in the abdomen and present variable symptoms according to size and their specific location, with abdominal pain being the main symptom. Methods. Five pediatric patients with abdominal lymphatic malformations are presented. Their clinical presentation, location, treatment and experience in the management of this pathology in a referral hospital are described. Results. The most appropriate methods to make a diagnostic approach are ultrasound, computed tomography and magnetic resonance imaging. Among the options described for treatment are pharmacological, sclerotherapy and surgical resection, both open and laparoscopic. Conclusion. There are a variety of methods for resecting abdominal lymphangiomas, but surgery remains the most effective, especially when laparoscopy is used as a therapeutic tool.

Experiencia en el manejo de malformaciones linfáticas macroquísticas con alcohol y microquísticas con bleomicina / Experience In the Management of Macrocystic Lymphatic Malformations with Alcohol and Microcystic Malformations with Bleomycin

Las malformaciones linfáticas son anomalías raras de flujo lento y bajo que se presentan en 1 de 6.000 a 1 en 16.000 recién nacidos vivos. Las malformaciones quísticas se clasifican según su tamaño en macroquísticas, microquísticas o mixtas. Esta clasificación tiene impacto sobre el tratamiento y el pronóstico. Las malformaciones linfáticas macroquísticas tienen mejor respuesta al tratamiento, mientras que las microquísticas son difíciles de tratar y con frecuencia recidivan. El objetivo de este trabajo es describir los resultados obtenidos en pacientes con malformaciones linfáticas macro y microquísticas intervenidos con escleroterapia utilizando alcohol y bleomicina, respectivamente. Se realizó un estudio descriptivo de pacientes con malformaciones linfáticas tratadas en el Servicio de Radiología. Se incluyeron 38 pacientes, de los cuales 24 tenían lesiones macroquísticas, 10, microquísticas y 4, mixtas. El 68 % fueron tratados con alcohol y el 32 % con bleomicina. El tratamiento mostró una resolución excelente en 5 pacientes, 25 tuvieron reducción del tamaño de la lesión entre 50 y 90 %, 13 % mostró una respuesta pobre, y solo en un 7 % hubo crecimiento, a pesar del tratamiento esclerosante. De lo anterior se concluye que el tratamiento percutáneo es seguro, reduce el tamaño de las lesiones y hay pocas complicaciones informadas. Se podría utilizar como tratamiento inicial, antes de considerar una cirugía.

Lymphatic malformations are rare slow and low flow abnormalities that occur in 1 of 6,000 to 1 in 16,000 live newborns. Cystic malformations are classified according to their size as macrocystic, microcystic, or mixed. This classification has an impact on treatment and prognosis. Macrocystic lymphatic malformations have a better response to treatment, while microcystic malformations are difficult to treat and frequently recur. The objective of this study is to describe the results obtained in patients with macro and microcystic lymphatic malformations who underwent sclerotherapy using alcohol and Bleomycin, respectively. A descriptive study of patients with lymphatic malformations treated in the Radiology Service of the National Institute of Pediatrics was carried out during the period from 2014 to 2016. Thirty-eight patients were included, 24 with macrocystic, 10 microcystic and 4 mixed lesions. 68% were treated with alcohol and 32% of the patients were treated with Bleomycin. The treatment showed excellent resolution in 5 patients, 25 patients had a lesion size reduction between 50 and 90%, 13% had a poor response, and only 7% had growth despite sclerosing treatment. From the above we conclude that percutaneous treatment is safe, reduces the size of the lesions and there are few reported complications. It could be used as initial treatment before considering surgery.

Advances of Angiopoietin-Tie axis in vascular and lymphatic system-related diseases / 生物工程学报

Endothelial cells that form the inner layers of both blood and lymphatic vessels are important components of the vascular system and are involved in the pathogenesis of vascular and lymphatic diseases. Angiopoietin (Ang)-Tie axis in endothelial cells is the second endothelium-specific ligand-receptor signaling system necessary for embryonic cardiovascular and lymphatic development in addition to the vascular endothelial growth factor receptor pathway. The Ang-Tie axis also maintains vascular homeostasis by regulating postnatal angiogenesis, vessel remodeling, vascular permeability, and inflammation. Therefore, the dysfunction of this system leads to many vascular and lymphatic diseases. In light of the recent advances on the role of the Ang-Tie axis in vascular and lymphatic system-related diseases, this review summarizes the functions of the Ang-Tie axis in inflammation-induced vascular permeability, vascular remodeling, ocular angiogenesis, shear stress response, atherosclerosis, tumor angiogenesis, and metastasis. Moreover, this review summarizes the relevant therapeutic antibodies, recombinant proteins, and small molecular drugs associated with the Ang-Tie axis.

Diferenciación entre linfadenopatía cervical benigna y maligna mediante ecografía en niños / Differentiation of benign from malignant cervical lymphadenopathy by ultrasonography in children

Introducción. Las causas más frecuentes de la linfadenopatía cervical son las afecciones inflamatorias y reactivas; solo unos pocos casos representan una patología seria. El objetivo fue evaluar la relación entre los hallazgos ecográficos y el diagnóstico histopatológico. Población y métodos. Este estudio retrospectivo abarcó la linfadenopatía cervical en los menores de 20 años seguidos en nuestro centro, entre enero de 2007 y diciembre de 2016. Según los informes anatomopatológicos, se dividió a los pacientes en dos grupos: benigno y maligno. Se compararon los resultados anatomopatológicos y los hallazgos ecográficos. Resultados. Después del análisis de los resultados histopatológicos y los hallazgos ecográficos, se incluyó a 107 pacientes con linfadenopatía cervical persistente (44 casos malignos; 63, benignos). La media de edad de los grupos maligno y benigno fue de 14 ± 6,1 años y de 11,9 ± 4,8 años, respectivamente. La presencia de vascularidad hiliar fue estadísticamente significativa (p < 0,0001) en la linfadenopatía benigna, mientras que el flujo periférico y la vascularidad mixta lo fueron (p < 0,05) en la linfadenopatía maligna. No se observó una diferencia significativa en el diámetro máximo (27,3 ± 11,1 mm y 29,8 ± 12,3 mm, respectivamente), pero sí en el diámetro mínimo entre los grupos benigno y maligno (13,7 ± 7,3 mm y 18,7 ± 8,8 mm, respectivamente). Conclusiones. Este estudio sugiere que existe una relación entre los hallazgos ecográficos y de la biopsia para la diferenciación entre la linfadenopatía benigna y maligna, en especial, en el patrón vascular intraganglionar y el hilio ganglionar.

Introduction. The most common causes of cervical lymphadenopathy (LAP) are inflammatory and reactive conditions; only a small proportion have serious pathology, such as malignancy. The objective of this study was to evaluate the relationship between USG findings and histopathological diagnosis of the cervical LAP. Population and Methods. This retrospective study comprised the cases of cervical LAP in patients aged under 20 years old followed in our center between January 2007 to December 2016. Based on pathology reports, we divided the patients into two groups: benign and malignant. Pathology results and USG findings were compared. Results. After the analyze of the histopathological results and USG findings, 107 patients with persistent cervical LAP (44 malignant; 63 benign) were included in the study. Mean age of malignant and benign group were 14 ± 6.1; 11.9 ± 4.8 years, respectively. Hilar vascularity for benign LAP was highly statistically significant (P < 0.0001) and peripheral flow and mixed vascularity for malignant LAP were also statistically significant (p < 0.05). There was not a significant difference in the maximum diameter (27.3 ± 11.1 mm and 29.8 ± 12.3 mm, respectively), however, there was a significant difference in the minimum diameter between benign and malignant groups (13.7 ± 7.3 mm and 18.7 ± 8.8 mm, respectively).Conclusions. The present study suggests that there is a relationship between US and biopsy findings for the differentiation of benign from malignant LAP, especially in terms of nodal hilus and intranodal vascular pattern.

Completion hysterectomy after chemoradiotherapy for locally advanced adeno-type cervical carcinoma: updated survival outcomes and experience in post radiation surgery / 부인종양

OBJECTIVE: To compare patient survival outcomes between completion hysterectomy and conventional surveillance in locally advanced adenocarcinoma of the cervix after concurrent chemoradiotherapy (CCRT).METHODS: Patients with adenocarcinoma of the cervix after CCRT were identified in a tertiary academic center database from 2004 to 2018. Patients received completion hysterectomy or surveillance after CCRT. We compared the progression-free survival (PFS) and overall survival (OS) between the patients with or without adjuvant hysterectomy. Surgery features, operative complications, and pathologic characteristics were documented. Patient outcomes were also analyzed according to clinicopathologic factors.RESULTS: A total of 78 patients were assigned to completion surgery and 97 to surveillance after CCRT. The PFS was better in the surgery group compared to the CCRT only group, at 3 years the PFS rates were 68.1% and 45.2%, respectively (hazard ratio [HR]=0.46; 95% confidence interval [CI]=0.282–0.749; p=0.002). Adjuvant surgery was also associated with a higher rate of OS (HR=0.361; 95% CI=0.189–0.689; p=0.002), at 3 years, 87.9% and 67%, respectively. Tumor stage, size, lymph-vascular space invasion (LVSI), lymphadenopathy were associated with PFS but not with OS. Hysterectomy specimens revealed 64.1% (50/78) of the patients had pathologic residual tumor. Patients age less than 60, tumor size over 4 cm, stage IIB and persistent residual disease after CCRT were most likely to benefit from hysterectomy. Hysterectomy was associated with a lower rate of locoregional recurrence but did not reach statistical significance (5.13% vs. 13.5%, p=0.067).CONCLUSION: Completion hysterectomy after CCRT was associated with better survival outcome compared with the current standard of care.

Impending Cardiac Tamponade and Hemorrhagic Pleural Effusion as Initial Presentations of Incomplete Kawasaki Disease: A Case Report

Kawasaki disease (KD) is an acute febrile illness that is characterized by systemic inflammation usually involving medium-sized arteries and multiple organs during the acute febrile phase, leading to associated clinical findings. The diagnosis is based on the principal clinical findings including fever, extremity changes, rash, conjunctivitis, oral changes, and cervical lymphadenopathy. However, KD diagnosis is sometimes overlooked or delayed because other systemic organ manifestations may predominate in acute phase of KD. As a cardiovascular manifestation, an acute pericarditis usually shows a small pericardial effusion, but large pericardial effusion showing clinical signs of cardiac tamponade is very rare. Here, we described a case of incomplete KD presenting with impending cardiac tamponade, and recurrent fever and pleural effusion.

Enfermedades del timo por exceso y por defecto / Thymus diseases by excess and default

El timo es un órgano cervicotorácico, impar y mediano, situado en la base del cuello y parte superior del mediastino. Junto a la médula ósea es uno de los dos órganos primarios del sistema inmune y ejerce su función en los neonatos y en los niños, fundamentalmente. Entra en regresión a partir de la pubertad, aunque algunos autores plantean que la involución puede comenzar un poco antes, cuando los principales tejidos linfoides están plenamente desarrollados. Interviene sinérgicamente con otras glándulas de secreción interna: tiroides, suprarrenal, hipófisis, para elaborar substancias necesarias para el desarrollo general del organismo. Es un órgano muy sensible a todo influjo. Como todos los órganos de la economía el timo presenta enfermedades producidas tanto por crecimiento exagerado, como por hipoplasias o atrofias. Dentro de las primeras las más comunes son la hiperplasia tímica y el timoma y, entre las últimas el síndrome de DiGeorge ha sido bien caracterizado en la literatura internacional desde la segunda mitad del siglo pasado. Sin embrago, en los últimos tiempos los inmunólogos hablan de la hipoplasia tímica como entidad que puede asociarse o no a estados de inmunodeficiencia. Se describen brevemente estas afecciones(AU)

The thymus is a cervicothoracic organ, odd and medium, located at the base of the neck and upper part of the mediastinum. Next to the bone marrow is one of the two primary organs of the immune system and exerts its function in neonates and children, fundamentally. It regresses after puberty, although some authors suggest that the involution can begin a little earlier, when the main lymphoid tissues are fully developed. It intervenes synergistically with other glands of internal secretion: thyroid, adrenal, pituitary gland, to develop substances necessary for the general development of the organism. It is a very sensitive organ to all influence. Like all the organs of the economy, the thymus presents diseases caused both by exaggerated growth, as by hypoplasias or atrophies. Among the former, the most common are thymic hyperplasia and thymoma and, among the latter, DiGeorge syndrome has been well characterized in international literature since the second half of the last century. However, in recent times immunologists speak of thymic hypoplasia as an entity that may or may not be associated with immunodeficiency states. These conditions are briefly described(AU)

Linfangioma mesentérico en la edad pediátrica / Mesenteric Lynphangioma in the pediatric ages

Resumen Introducción: Dentro del espectro de las malformaciones congénitas con las cuales debe lidiar un cirujano pediatra, los tumores y malformaciones vasculares representan un grupo especial de entidades que continúan actualmente en estudio. Objetivo: Determinar las características clínicoquirúrgicas de una serie de dos casos con malformaciones de origen linfático. Presentacion de casos: Dos pacientes con edades pediátricas, 12 y 4 años, respectivamente, ingresaron en servicio de urgencia con signos de oclusión intestinal y lesiones tumorales quísticas abdominales que requirieron tratamiento quirúrgico con modalidades de técnicas quirúrgicas diferentes. La evolución final fue satisfactoria. Conclusión: Los linfagiomas intestinales forman parte de la gama de presentaciones clínicas de las malformaciones de origen linfático, con una incidencia baja pero que, dada su localización y síntomas, pueden requerir tratamiento quirúrgico de urgencia. Su evolución y resolución es satisfactoria, debido al bajo índice de recidiva tras la exéresis completa(AU)

ABSTRACT Introduction: Within the range of congenital malfomations that surgeons have to deal with, vascular tumors and malformations represent an special group of entities that are still under study. Objective: To determine the clinical-surgical characteristics of a series of two cases with congenital malformations of lymphatic origin. Cases presentation: Two patients of 12 and 4 years old respectively that were admitted in the Emergencies services in the Clinical-Surgical Hospital of Guayaquil, Ecuador, with manifestations of intestinal obstruction and abdominal cystic tumoral lesions that required surgical treatments with different techniques. Final evolution was satisfactory. Conclusion: Intestinal lymphoangiomas are part of the kinds of clinical presentations of lymphatic origin´s malformations. These have a low incidence but due to their localization and symptoms can require emergency surgical treatment. Their evolution and resolution is satisfactory due to the low index of recidive after complete exeresis(AU)

Acoustic radiation force impulse imaging of biopsy-proven Kikuchi disease: initial experiences for evaluating feasibility in pediatric patients

PURPOSE: This study evaluated the feasibility of acoustic radiation force impulse (ARFI) elastography and characterized the sonographic features of lymph nodes (LNs) with Kikuchi disease in pediatric patients. METHODS: Seventy-six cervical LN biopsies were performed for the diagnosis of cervical lymphadenopathy. ARFI imaging was performed, and the characteristic ultrasound features of the biopsied LNs and the contralateral LNs were analyzed. We also reviewed clinical and conventional ultrasonographic findings. RESULTS: On histology, 56 patients were diagnosed with Kikuchi disease. These LNs were large and elongated, with increased perinodal echogenicity and capsular thickening. In 38 of them, ARFI elastography was performed, and the median shear wave velocity (SWV) of the biopsied LNs with Kikuchi disease (2.19 m/sec; range, 1.45 to 4.57 m/sec) was higher than of the contralateral LNs (1.72 m/sec; range, 0.95 to 2.65 m/sec; P < 0.001). In patients with reactive hyperplasia, the mean SWV of the biopsied LNs (2.00 m/sec; range, 1.49 to 2.26 m/sec) was higher than that of the contralateral LNs (1.55 m/sec; range, 1.21 to 2.32 m/sec; P=0.031). CONCLUSION: The SWV of LNs with Kikuchi disease was significantly higher than that of the contralateral LNs. Morphologically, LNs with Kikuchi disease showed an enlarged, elongated, and oval shape, increased perinodal echogenicity, and capsular thickening. In addition to the conventional ultrasonographic findings, the application of ARFI is feasible even in pediatric patients for the evaluation of cervical lymphadenopathy.

Left-Sided Cisterna Chyli: a Case Report on a Rare Normal Anatomic Structure

The cisterna chyli, a dilated lymphatic sac in the retrocrural space, is usually located to the right of the aorta. We report a case of a left-sided cisterna chyli, which was incidentally detected on the radiologic examinations of a preoperative workup for cholangiocarcinoma. Computed tomography (CT) and magnetic resonance (MR) images revealed a cisterna chyli measuring 2.5 cm in length in the left retrocrural space. The dilated lumbar lymphatics joined with the cisterna chyli, which was continuous with the left-sided thoracic duct. To the best of our knowledge, this is the second antemortem case of a left-sided cisterna chyli in literature. The cisterna chyli can mimic retrocrural lymphadenopathy, solid tumor with cystic degeneration, abscess or hematoma. The left-sided cisterna chyli should be referred to as a structure so as to be cautious in surgical approach.

A Case of Recurrent Rosai-Dorfman Disease Successfully Treated with 2-Chlorodeoxyadenosine (Cladribine) / 임상소아혈액종양

Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease (RDD), is a rare histiocytic disorder of unknown etiology. Most patients with RDD have spontaneous remission, but in some patients, the disease recurs after complete remission and may not respond to general treatment. Some patients with RDD involving the extranodal system can have serious symptoms such as vital organ dysfunction due to mass effects, neurological symptoms caused by intracranial involvement, and respiratory distress with airway involvement. We report the case of a 7-year-old girl with severe dyspnea due to refractory extranodal RDD that caused progressive upper airway obstruction. She was admitted because of nasal congestion and persistent cervical lymphadenopathy, and diagnosed as having RDD by cervical lymph node incisional biopsy. The initial prednisone treatment did not improve her symptoms. The following contrast-enhanced neck computed tomography revealed a newly developed airway mass protruding in the upper trachea. After 8 weeks of chemotherapy with vinblastine, methotrexate, and prednisone, complete remission was attained. Seven months after chemotherapy cessation, the disease recurred, and chemotherapy with vincristine, cytarabine, and prednisone was resumed. Despite the chemotherapy and emergency radiotherapy, no improvement was observed in the cervical lymph node enlargement and airway obstructive symptom due to the upper tracheal mass. 2-Chlorodeoxyadenosine (cladribine) therapy was initiated, and the patient got complete remission after 6 cycles of the cladribine treatment and maintained no evidence of disease for 2 years. We suggest that cladribine is an effective treatment option for recurrent/refractory RDD.

A Rare Case of Lymph Node Metastasis from Early Gastric Cancer

Gastric cancers that fulfill the Japanese criteria for curative endoscopic resection show a low risk of lymph node (LN) metastasis. Here, we report a case of LN metastasis from early gastric cancer that fulfilled the curative criteria. A 74-year-old Japanese woman was referred to our hospital for treatment of early gastric cancer identified at the site of a hyperplastic polyp that had been diagnosed 10 years prior to presentation. Contrast-enhanced computed tomography did not show any lymphadenopathy and laparoscopy-assisted distal gastrectomy was performed. Histopathological examination revealed a predominantly moderately differentiated adenocarcinoma that measured 15 mm in size and was confined to the mucosa. However, a single metastatic regional LN was observed. A few cancer cells showed positive staining for alpha-fetoprotein. It should be noted that early gastric cancer can be accompanied by LN metastasis even if it fulfills the criteria for curative endoscopic resection.

How to Improve the Diagnostic Accuracy of EUS-FNA in Abdominal and Mediastinal Lymphadenopathy?

No abstract available.

Predictive Factors for Inaccurate Diagnosis of Swollen Lymph Nodes in Endoscopic Ultrasound-Guided Fine Needle Aspiration

BACKGROUND/AIMS: This study aimed to identify the predictive factors for inaccurate endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) diagnosis of swollen lymph nodes without rapid on-site cytopathological evaluation. METHODS: Eighty-three consecutive patients who underwent EUS-FNA for abdominal or mediastinal lymph nodes from January 2008 to June 2017 were included from a prospectively maintained EUS-FNA database and retrospectively reviewed. The sensitivity, specificity, and accuracy of EUS-FNA for the detection of neoplastic diseases were calculated. Candidate factors for inaccurate diagnosis (lymph node size and location, needle type, puncture route, number of passes, and causative disease) were evaluated by comparison between accurately diagnosed cases and others. RESULTS: The final diagnosis of the punctured lymph node was classified as neoplastic (65 cases: a metastatic lymph node, malignant lymphoma, or Crow-Fukase syndrome) or non-neoplastic (18 cases: a reactive node or amyloidosis). The sensitivity, specificity, and accuracy were 83%, 94%, and 86%, respectively. On multivariate analyses, small size of the lymph node was the sole predictive factor for inaccurate EUS-FNA diagnosis with a significant difference (odds ratios, 19.8; 95% confidence intervals, 3.15–124; p=0.0015). CONCLUSIONS: The lymph node size of <16 mm was the only independent factor associated with inaccurate EUS-FNA diagnosis of swollen lymph nodes.

A Case of Unilateral Vocal Fold Paralysis Secondary to Sarcoidosis

Sarcoidosis is a multisystem granulomatous disease of unknown etiology. Vocal fold paralysis secondary to sarcoidosis is extremely rare but it can develop as a result of compressive lymphadenopathy, granulomatous infiltration, and neural involvement. We report the case of a 56-year-old woman who presented with unilateral vocal fold paralysis and enlarged supraclavicular lymph nodes. Computed tomography of the neck revealed multiple, enlarged, and matted lymph nodes at the cervical level of IV. An ultrasound-guided core needle biopsy of the lymph node was performed, and a histopathological diagnosis of sarcoidosis was made by validating the presence of noncaseating granuloma. After implementation of steroid therapy, the patient exhibited immediate recovery from vocal fold paralysis. Although an extremely rare disease, sarcoidosis should be included in the differential diagnosis of vocal fold paralysis. Accurate diagnosis and prompt steroid treatment may reduce the morbidity of patients with vocal fold paralysis secondary to sarcoidosis.

Efficacy of Annexin A1 Immunostaining in Bone Marrow for the Diagnosis of Hairy Cell Leukemia

Splenic B-cell lymphomas (SBCLs) show characteristically pronounced splenomegaly without significant lymphadenopathy. Distinguishing hairy cell leukemia (HCL) from other SBCLs (splenic marginal zone lymphoma [SMZL], variant HCL [v-HCL], and splenic diffuse red pulp small B-cell lymphoma [SDRPL]) is essential to determine suitable treatments and prognoses. With advances in diagnostic modalities and therapies, splenectomy is not commonly performed, and thus diagnosis of HCL must be based on the results obtained using blood and bone marrow samples. Annexin A1 is known as the most specific marker for HCL. There has yet been no report of the assessment of annexin A1 immunostaining from Korea. In this study we analyzed samples from 13 Korean patients with SBCLs (three HCL, three v-HCL, six SMZL, and one SDRPL) from May 2001 to December 2016. Immunohistochemical analyses for annexin A1 and CD20 were performed using bone marrow sections; molecular analyses for detection of the BRAF V600E mutation were also performed. All HCL patients showed positive results for annexin A1 immunostaining and the presence of the BRAF V600E mutation, and negative results for other SBCLs. Our results confirmed the high specificity of annexin A1 and the BRAF V600E mutation as HCL markers. Molecular analysis requires expensive equipment and substantial manpower. Annexin A1 is a better alternative as an HCL marker than the BRAF V600E mutation in terms of cost-effectiveness.