[Collision sellar lesions: pituitary adenoma and Rathke cleft cyst]. / Lesiones sincrónicas selares: adenoma hipofisario y quiste de la bolsa de Rathke.

Rathke's cleft cyst (RCC) are a slow-growing, benign, cystic lesions that arises from the remnants of the primitive ectoderm and Rathke's pouch. They present a common origin with pituitary adenomas (PA), however, the concomitant presentation of a PA and a RCC rarely occur. We present a case of a 41-year-old female with Cushing's disease. Magnetic resonance imaging (MRI) showed two synchronic lesions in the sellar region. An endoscopic transnasal approach was performed, with complete excision of both. The histological studies revealed an ACTH secreting PA and a RCC. The patient presented clinical and endocrinological remission six months after surgery. With the presence of cystic lesion at the sellar region, and the concomitant finding of a pituitary adenoma, RCC should be considered.

El quiste de la bolsa de Rathke (QBR) es una entidad benigna de crecimiento lento que proviene del remanente del ectodermo primitivo. Presenta un origen común con los adenomas hipofisarios (AH), sin embargo, la presentación sincrónica de un AH y un QBR es infrecuente. Presentamos el caso de una mujer de 41 años con enfermedad de Cushing. Se realizó resonancia magnética con el hallazgo de dos lesiones en región selar. Se hizo un abordaje transnasal endoscópico, con exéresis completa de ambas. El informe de anatomía patológica reveló un AH corticotropo y un QBR. Tuvo una remisión clínica analítica endocrinológica a los seis meses postquirúrgicos. Ante el hallazgo de una imagen quística a nivel selar concomitante con un adenoma hipofisario, debe ser considerada la posibilidad diagnóstica de un QBR.

[Current role of bilateral adrenalectomy in Ectopic Cushing syndrome.] / Rol actual de la adrenalectomía bilateral en el manejo del síndrome de Cushing Ectópico.

OBJECTIVES: The aim of this paper is to describe the clinical features and the perioperative results of the bilateral laparoscopic adrenalectomy (BLA) of salvaje in 5 patients treated for an Ectopic Cushing Syndrome (CS) who were refractory to medical treatment. MATERIALS AND METHODS: We performed a retrospective analysis of the clinical records of our institution. Five BLAs were performed in patients with Ectopic CS refractory to medical treatment during the period from January 2009 and June 2017. All patients were evaluated by a multidisciplinary team that jointly decided the most appropriate time for surgery. The following protocol data were analyzed: age, sex, location of the secreting tumor, duration of hypercortisolism, reason for hospitalization, initial therapeutic management), biochemical data (pre-op serum cortisol, plasma ACTH and free urinary cortisol), surgical data (surgical approach, surgical time, type of surgery, need for conversion toopen surgery), perioperative data (post op surgical stay, post op complications according to the Clavien-Dindo scale 14, mortality, clinical cure rate and biochemical cure rate, follow up time). RESULTS: Five patients underwent BLA. The approach for the BLA was minimally invasive, either trans peritoneal (n=3) or posterior retroperitoneoscopic (n=2). Mean hospitalization time was 23.4 days (r=4-81). None of the patients died because of surgical complications, and all of them achieved biochemical and clinical remission of their hypercortisolism after surgery. CONCLUSIONS: BLA is an effective salvage alter native to control the symptoms associated with overproduction of corticosteroids in patients with ectopic CS refractory to medical treatment.

OBJETIVO: Describir las características clínicas y los resultados perioperatorios de la adrenalectomía bilateral laparoscópica (ABL) en 5 pacientes tratados con diagnóstico de Síndrome de Cushing Ectópico (SCE).MATERIALES Y MÉTODOS: Se realizó un estudio retrospectivo y descriptivo de todos los pacientes con SCE sometidos a una ABL, entre enero de 2009 y junio de 2017. Todos los pacientes fueron evaluados por un equipo multidisciplinario que decidió conjuntamente el momento más adecuado para la cirugía. Fueron analizados los siguientes datos protocolares: edad, sexo, origen del tumor secretor, duración del hipercortisolismo, motivo de internación, manejo terapéutico inicial, datos bioquímicos (cortisol sérico preoperatorio, ACTH plasmática y cortisol libre urinario), datos quirúrgicos (vía de abordaje, tiempo quirúrgico, tipo de cirugía, necesidad de conversión a cirugía abierta), datos perioperatorios (tiempo de internación postoperatoria, complicaciones según la escala de Clavien-Dindo, mortalidad, tasa de curación clínica y tasa de curación bioquímica, tiempo de seguimiento). RESULTADOS: Cinco pacientes fueron sometidos a ABL. El abordaje fue mínimamente invasivo, ya sea transperitoneal (n=3) o retroperitoneoscópico posterior (n=2). El tiempo de internación fue de 23,4 días (r=4-81). Ningún paciente falleció por complicaciones quirúrgicas, y todos lograron la curación bioquímica y clínica de su hipercortisolismo después de la cirugía. CONCLUSIONES: ABL es un procedimiento de rescate eficaz para controlar los síntomas asociados al hipercortisolismo en pacientes con SCE refractario al tratamiento médico.

Bilateral pheochromocytoma after kidney transplantation in neurofibromatosis type 1.

We present the case of a 25-year-old male with a history of neurofibromatosis type 1 and bilateral pheochromocytoma 4 years after kidney transplantation that was successfully treated with simultaneous bilateral posterior retroperitoneoscopic adrenalectomy. Learning points: Hypertensive patients with NF1 should always be screened for pheochromocytoma. Pheochromocytoma is rarely associated with transplantation, but it must be ruled out in patients with genetic susceptibility. Posterior retroperitoneoscopic adrenalectomy (PRA) allows more direct access to the adrenal glands, especially in patients with previous abdominal surgeries.

Functional plurihormonal adrenal oncocytoma: case report and literature review.

We present a 27-year-old woman with an adrenal oncocytoma. This is a very rare entity. We provide a review of the clinical, biochemical and pathological features of cases reported in the literature.

[Obstructive jaundice associated Burkitt's lymphoma mimicking pancreatic carcinoma]. / Ictericia obstructiva asociada a linfoma de Burkitt en un adulto inmunocompetente.

The primary compromise of the pancreas in lymphomas is uncommon. However, in advanced stages of Non-Hodgkin's lymphomas (LNH) the secondary invasion of the pancreas is observed more frequently. Jaundice due to extrahepatic cholestasis as a presentation form is extremely rare, with only few cases described in the literature. The aim is to present a case of an obstructive jaundice as an expression of Burkitt's lymphoma probably due to a diffuse pancreatic infiltration in an adult without immunodeficiency with a rapid response of cholestasis to low dose of hydrocortisone. Skin tumor simultaneously present with jaundice allowed the histologic diagnosis with skin biopsies. After a unique dose of 100 mg hydrocortisone, jaundice improved and cholestatic enzymes decreased, pancreas became smaller and common bile duct diameter became normal at ultrasound and CT scan, also skin tumors turn pale and diminished in size. There are isolated reports of Burkitt's lymphoma cases with associated obstructive jaundice due to pancreatic infiltration or by compression by lymph nodes of the bile ducts, many of them are pediatric cases or immunodepressed HIV patients. In the case presented, surgical resection of the pancreatic infiltration and biliary drainage, either surgical or endoscopic during the same procedure was not necessary for the histopathologic diagnosis of the illness like is described in the literature. The diagnosis was suspected by the rapid decrease of cholestatic features after a single dose of hydrocortisone and the histology was easy done by a skin biopsy. We think the interest in this case is the quick response to low doses of corticoids, which avoided the necessity of surgical procedure for the diagnosis of the biliary tree obstruction, allowing a quick implementation of the specific chemotherapeutic treatment of the lymphoma without any surgical or endoscopic procedures to heal the jaundice.

Lesiones sincrónicas selares: adenoma hipofisario y quiste de la bolsa de Rathke / Collision sellar lesions: pituitary adenoma and Rathke cleft cyst

Resumen El quiste de la bolsa de Rathke (QBR) es una entidad benigna de crecimiento lento que proviene del remanente del ectodermo primitivo. Presenta un origen común con los adenomas hipofisarios (AH), sin embargo, la presentación sincrónica de un AH y un QBR es infrecuente. Presentamos el caso de una mujer de 41 años con enfermedad de Cushing. Se realizó resonancia magnética con el hallazgo de dos lesiones en región selar. Se hizo un abordaje transnasal endoscópico, con exéresis completa de ambas. El informe de anatomía patológica reveló un AH corticotropo y un QBR. Tuvo una remisión clínica analítica endocrinológica a los seis meses postquirúrgicos. Ante el hallazgo de una imagen quística a nivel selar concomitante con un adenoma hipofisario, debe ser considerada la posibilidad diagnóstica de un QBR.

Abstract Rathke's cleft cyst (RCC) are a slow-growing, benign, cystic lesions that arises from the remnants of the primitive ectoderm and Rathke's pouch. They present a common origin with pituitary adenomas (PA), however, the concomitant presentation of a PA and a RCC rarely occur. We present a case of a 41-year-old female with Cushing's disease. Magnetic resonance imaging (MRI) showed two synchronic lesions in the sellar region. An endoscopic transnasal approach was performed, with complete excision of both. The histological studies revealed an ACTH secreting PA and a RCC. The patient presented clinical and endocrinological remission six months after surgery. With the presence of cystic lesion at the sellar region, and the concomitant finding of a pituitary adenoma, RCC should be considered.

Síndrome de Turner: nuestra experiencia en la creación del Registro Institucional y de la Unidad Interdisciplinaria en el Hospital Italiano de Buenos Aires: un primer paso para el mejor seguimiento / Turner syndrome: our experience in the creation of the Registry Institutional and Interdisciplinary Unit at the Italian Hospital of Buenos Aires: a first step to the best follow-up

El síndrome de Turner (ST) resulta de la ausencia completa o parcial del segundo cromosoma sexual en fenotipos femeninos. Tiene una incidencia de 1:2000- 2500 nacidas vivas. Recién en la última década se ha puesto atención a la salud de las adultas con ST. La mortalidad es 3 veces superior respecto de la población general debido al riesgo de disección aórtica por anomalías cardiovasculares estructurales y aterosclerosis vinculada a hipertensión arterial, diabetes, dislipidemia y obesidad. También presentan elevada prevalencia de enfermedades autoinmunitarias. Objetivo: evaluar la calidad del seguimiento clínico de pacientes adultas con ST, comparando los controles de salud preconformación y posconformación del Registro y de la Unidad Interdisciplinaria. En el año 2017 fuimos convocados para integrar el Programa de Enfermedades Raras del Hospital Italiano de Buenos Aires. A partir de la creación del Registro Institucional y del equipo multidisciplinario obtuvimos mejoría significativa en los controles por las especialidades de cardiología, endocrinología y otorrinolaringología, en los controles bioquímicos del metabolismo lipídico, hidrocarbonado, hepatograma, TSH y anticuerpos para celiaquía e imágenes cardiovasculares y densitometría ósea. En conclusión, el seguimiento sistematizado e institucional, mediante el Registro y la creación de la Unidad Interdisciplinaria de Síndrome de Turner, permitió encontrar las falencias del sistema de atención y optimizar el seguimiento de esta población. (AU)

Turner syndrome (TS) results from the complete or partial absence of the second sex chromosome in female phenotypes. It has an incidence of 1: 2000-2500 girls born alive. Only in the last decade has been paid attention to the health of adults women with TS. Mortality is 3 times higher than in the general population due to the risk of aortic dissection cause to structural cardiovascular anomalies and atherosclerosis related to hypertension, diabetes, dyslipidemia and obesity. They also have a high prevalence of autoimmune diseases. Until nowadays in Argentina do not exist a national registry of this disease that complies with the international follow-up recommendations for these patients. We proposed to develop the institutional register at 2014 and a multidisciplinary team was created to care and follow up girls and women with TS during 2015. It was indexed to Italian Hospital of Buenos Aires' Rare Diseases Program since 2017. After the creation of the institutional registry and the multidisciplinary team we obtained a significant improvement in cardiology, endocrinology and otorhinolaryngology schedule visits, in lipids and hydrocarbon metabolism, liver, thyroid and celiac diseases biochemical controls and in the performance of cardiovascular MNR and bone densitometry. In conclusion, the systematized and institutional follow-up, through the registry and the creation of the Interdisciplinary Unit of Turner Syndrome, allowed us to find the flaws of the care system and to optimize the follow up of this population. (AU)

Adrenalectomía laparoscópica: ¿abordaje retroperitoneal posterior o transperitoneal lateral? / Laparoscopic Adrenalectomy: posterior retroperitoneoscopic or lateral transperitoneal approach?

Objetivo: comparar los resultados obtenidos en adrenalectomía retroperitoneoscópica posterior (ARP) con los obtenidos por vía transperitoneal lateral (ATL) a corto y mediano plazo. Material y métodos: estudio de cohortes prospectivo. Se incluyeron 22 pacientes a quienes se les realizaron 24 adrenalectomías (12 ARP y 12 ATL) entre los meses de enero de 2015 y mayo de 2016. En el grupo ARP se realizó a 1 paciente adrenalectomía bilateral simultánea y a 1 paciente, bilateral sincrónica. Fueron comparados datos basales, clínicos, quirúrgicos y evolutivos. Resultados: la mediana de tiempo de seguimiento fue de 6,5 meses (r 1-16). Los grupos ARP y ATL fueron comparables en edad, sexo, BMI y presencia de cirugías previas. Los adenomas fueron las lesiones predominantes (41,7% en cada grupo). El promedio de tiempo operatorio en ART y ATL fue 104,3 ± 21,2 y 146 ± 32,9 minutos, respectivamente (p 0,05). No hallamos diferencias en tiempo de internación, sangrado y complicaciones. Todos los pacientes lograron curación clínica. Conclusiones: la técnica ARP es una vía segura, con resultados comparables a ATL y menor tiempo operatorio. También permite intervenir de manera simultánea y sincrónica ambas glándulas adrenales sin necesidad de reposicionar al paciente.

Objetive: to compare the results of posterior retroperitoneoscopic (PRA) or lateral transperitoneal approach (LTA) in laparoscopic adrenalectomy in the short and medium term. Material y methods: prospective cohort study. We included 22 patients who underwent 24 adrenalectomies (12 PRA and 12 LTA) between January 2015 and May 2016. In the PRA group, two simultaneous bilateral laparoscopic adrenalectomies were performed (1 synchronous bilateral adrenalectomy). Baseline, clinical and surgical outcome were compared. Results: the median follow-up time was 6.5 months (range: 1-16). The PRA and LTA groups were comparable in age, gender, BMI and presence of previous surgeries. Adenomas were the predominant lesions (41.7% in each group). The average operating time in PRA and LTA was 104.3 ± 21.2 and 146 ± 32.9 minutes, respectively (p=0.05). We found no differences in length of hospital stay, bleeding and complications. All patients achieved clinical cure. Conclusions: the PRA technique is a safe approach, with results comparable to LTA and shorter operating time. It also allows to intervene simultaneously and synchronously both adrenal glands without the need of repositioning the patient.

Ictericia obstructiva asociada a linfoma de Burkitt en un adulto inmunocompetente / Obstructive jaundice associated Burkitt's lymphoma mimicking pancreatic carcinoma

El compromiso primario del páncreas en los linfomas es muy poco frecuente, sin embargo, en los estadios avanzados de los linfomas no Hodgkin la invasión secundaria de la glándula es observada con mayor frecuencia. El objetivo de esta presentación es describir un caso de linfoma de Burkitt en un adulto inmunocompetente que presentó como manifestación relevante colestasis extrahepática secundaria probablemente a infiltración pancreática difusa y tumores cutáneos cuya histología permitió hacer el diagnóstico. Luego de una dosis única de hidrocortisona de 100mg, mejoró la ictericia, disminuyeron las enzimas de colestasis, las lesiones cutáneas y disminuyó el tamaño del páncreas en la ecografía y en la tomografía computada. Existen en la literatura reportes aislados de casos de linfoma tipo Burkitt que se asocian a ictericia obstructiva secundaria y a infiltración pancreática o del hilio hepático, tratándose en su mayoría de casos pediátricos o de individuos afectados por el virus de la inmunodeficiencia humana (VIH). Creemos que el interés de este caso radica en la rápida respuesta a dosis bajas de corticoides de la colestasis, lo que evitó la necesidad de un procedimiento quirúrgico tanto diagnóstico como terapéutico de la obstrucción biliar, como está referido en la literatura, permitiendo instaurar rápidamente el tratamiento quimioterapéutico específico de esta entidad sin maniobras quirúrgicas o endoscópicas.

The primary compromise of the pancreas in lymphomas is uncommon. However, in advanced stages of Non- Hodgkin’s lymphomas (LNH) the secondary invasion of the pancreas is observed more frequently. Jaundice due to extrahepatic cholestasis as a presentation form is extremely rare, with only few cases described in the literature. The aim is to present a case of an obstructive jaundice as an expression of Burkitt’s lymphoma probably due to a diffuse pancreatic infiltration in an adult without immunodeficiency with a rapid response of cholestasis to low dose of hydrocortisone. Skin tumor simultaneously present with jaundice allowed the histologic diagnosis with skin biopsies. After a unique dose of 100 mg hydrocortisone, jaundice improved and cholestatic enzymes decreased, pancreas became smaller and common bile duct diameter became normal at ultrasound and CT scan, also skin tumors turn pale and diminished in size. There are isolated reports of Burkitt’s lymphoma cases with associated obstructive jaundice due to pancreatic infiltration or by compression by lymph nodes of the bile ducts, many of them are pediatric cases or immunodepressed HIV patients. In the case presented, surgical resection of the pancreatic infiltration and biliary drainage, either surgical or endoscopic during the same procedure was not necessary for the histopathologic diagnosis of the illness like is described in the literature. The diagnosis was suspected by the rapid decrease of cholestatic features after a single dose of hydrocortisone and the histology was easy done by a skin biopsy. We think the interest in this case is the quick response to low doses of corticoids, which avoided the necessity of surgical procedure for the diagnosis of the biliary tree obstruction, allowing a quick implementation of the specific chemotherapeutic treatment of the lymphoma without any surgical or endoscopic procedures to heal the jaundice.