Mielolipoma primario mediastinal / Primary mediastinal myelolipoma

Resumen El mielolipoma es un tumor benigno no funcional, la mayoría de ellos son asintomáticos y descubiertos de forma incidental a través de estudios de imagen o en es tudios de autopsia. Aun cuando la mayoría de los casos se presenta en la glándula suprarrenal, también se han informado en sitios extra-adrenales. Presentamos el caso de una mujer de 65 años de edad con un mielolipoma primario mediastinal. La tomografía computarizada de tórax mostró un tumor ovoide de bordes bien definidos de 6.5 × 4.2 cm, localizado en el mediastino posterior. Se realizó biopsia transtorácica de la lesión y el estudio microscópico reveló elementos hematopoyéticos y tejido adiposo maduro. Aun cuando los estudios de imagen como la tomografía computarizada y la resonancia mag nética son efectivos en el diagnóstico del mielolipoma primario mediastinal, la evaluación histopatológica es esencial para el diagnóstico definitivo.

Abstract Myelolipoma is a benign non-functional tumor. Most of them are asymptomatic and discovered incidentally, either through imaging studies or at autopsy. While it most commonly occurs in the adrenal gland, it has also been reported at extra-adrenal sites. We present the case of a 65-year-old woman with a primary mediastinal myelolipoma. Computer tomographic scan of the thorax showed an ovoid tumor with well-defined borders of 6.5 × 4.2 cm, located in the posterior mediastinum. A transthoracic biopsy of the lesion was made, and the microscopic observation revealed hematopoietic cells and mature adipose tissue. Although computed tomo graphy and magnetic resonance imaging are effective in diagnosing mediastinal myelolipoma, histopathological examination is essential for the definitive diagnosis.

Mielolipoma gigante unilateral de la glándula suprarrenal. Reporte de caso

Introducción: Los tumores de la glándula suprarrenal son inusuales y por lo general son hallados de forma incidental por estudios de imágenes. Dentro de este grupo los mielolipomas son uno de los tumores más raros, considerados el 2% de los tumores suprarrenales. Caso Clínico: Presentamos una paciente femenina de 60 años de edad con antecedentes de dolor a tipo cólico de forma esporádica a nivel del hipocondrio derecho. La ecografía abdominal detectó colelitiasis y una masa sugerente de adenoma suprarrenal izquierdo. La tomografía abdominal corroboró el tumor suprarrenal gigante y la litiasis vesicular. Se realizó suprarrenalectomía y colecistectomía convencional sin complicaciones. El diagnóstico histopatológico mostró un mielolipoma suprarrenal y una colecistitis crónica. Discusión: El mielolipoma suprarrenal es infrecuente, la etiología se desconoce, por lo general es asintomático y su hallazgo es incidental, habitualmente son unilaterales, menores a 4cm y la incidencia aumenta con la edad. Conclusiones: Cuando los mielolipomas alcanzan dimensiones mayores de 10cm se recomienda realizar una suprarrenalectomía convencional.

Introduction: Adrenal gland tumors are unusual and are usually found incidentally by imaging studies. Within this group, myelolipomas are one of the rarest tumors, considered 2% of adrenal tumors. Clinical case: We present a 60-year-old female patient with a history of sporadically colicky pain at the level of the right hypochondrium. Abdominal ultrasound revealed cholelithiasis and a mass suggestive of a left adrenal adenoma. Abdominal tomography confirmed a giant adrenal tumor and gallstones. An adrenalectomy and conventional cholecystectomy were performed without complications. The histopathological diagnosis showed an adrenal myelolipoma and chronic cholecystitis. Discussion: Adrenal myelolipoma is infrequent, the etiology is unknown, it is usually asymptomatic and its finding is incidental, they are usually unilateral, smaller than 4cm and the incidence increases with age. Conclusions: When myelolipomas reach dimensions greater than 10cm, conventional adrenalectomy is recommended. In selected cases.

Mielolipoma suprarrenal, revisión de literatura en México a propósito de dos casos / Adrenal myelolipoma, review of the literature in Mexico apropos of two cases

Introducción: los mielolipomas suprarrenales (ML) son neoplasias benignas poco frecuentes constituidas por tejido adiposo y mieloide. Clínicamente asintomáticas, suelen ser diagnosticados incidentalmente. En México existen solo 32 casos publicados de ML, presentándose en pacientes de entre 37 a 65 años, siendo la relación hombre-mujer 1:1.1, el síntoma más comúnmente reportado es dolor abdominal inespecífico, y la cirugía abierta es el principal abordaje quirúrgico empleado en nuestro país (89%). Caso clínico: presentamos una recopilación de la literatura actual sobre ML en México, además de dos casos clínicos de pacientes con ML: un hombre de 67 años con enfermedad diverticular y una mujer de 40 años con dolor en hipocondrio; en ambos se realizó resección tumoral, midiendo 9.5 cm y 13.3 cm, respectivamente. Conclusiones: presentamos dos casos nuevos en nuestro país que corresponden a incidentalomas. En ambos casos la cirugía se realizó para confirmar el diagnóstico, así como para prevenir posibles complicaciones.

Background: Adrenal myelolipomas (ML) are rare benign neoplasms compound of adipose and myeloid tissue. Clinically they are usually asymptomatic, being diagnosed generally by incident. In Mexico, there are only 32 published cases of ML, these occur between 37 and 65 years, with the male-female ratio being 1:1.1, clinically they present with abdominal or lumbar pain, open surgery being the main surgical approach (89%). Clinical case: We made a literature review of ML in Mexico and present two clinical cases: a 67-year-old man in followup for diverticular disease and a 40-year-old woman with pain in the left upper quadrant. In both cases, tumor resection was performed measuring 9.5 cm and 13.3 cm long respectively. Conclusions: We present two new cases in our country that correspond to incidentalomas. In both cases, surgery was performed to confirm the diagnosis, as well as to prevent possible complications

Resección laparoscópica de mielolipoma suprarrenal gigante / Laparoscopic resection of giant adrenal myelolipoma

Los mielolipomas suprarrenales son neoplasias suprarrenales benignas compuestas como su nombre lo indica, por tejido adiposo y tejido hematopoyético. Comprenden entre 6 - 16% de los incidentalomas suprarrenales, El tamaño de estos tumores es variable, desde unos pocos milímetros hasta > 10 cm, es entonces cuando se denominan adrenomielolipomas gigantes. Los síntomas de presentación más comunes son: Dolor abdominal: 22,5%, dolor en hipocondrio: 13,9%, dolor en el flanco: 13,9%, masa abdominal: 5,2%, y en raras ocasiones, disnea, dolor en espalda, fiebre, pérdida de peso y virilización. Presentación del caso: paciente masculino de 61 años de edad, con presencia de tumor de 15 x 12 cm, dependiente de glándula suprarrenal derecha, que comprime polo superior de riñón derecho, de -20 UH, compatible con mielolipoma suprarrenal derecho gigante, hallazgos incidentales, durante protocolo de estudio por crecimiento prostático, se realiza procedimiento de resección de tumor suprarrenal por abordaje laparoscópico, con excelentes resultados clínicos, sangrado mínimo y recuperación inmediata. Conclusión: el mielolipoma suprarrenal es un tumor benigno que se diagnostica en la mayoría de los casos de manera incidental, se sabe que clínicamente es no funcional, aunque siempre se deben de hacer estudios hormonales preoperatorios. El tratamiento de los mielolipomas suprarrenales, depende de cada caso clínico, siendo una recomendación el tratamiento quirúrgico de los tumores grandes >5 cm, sugerimos que se pueda optar por el abordaje laparoscópico, por ser el menos invasivo y con menor tasa de complicaciones, además de la recuperación pronta del paciente.

Adrenal myelolipomas are benign adrenal neoplasms composed, as the name implies, of adipose tissue and myeloid (hematopoietic) tissue. They comprise between 6 and 16% of adrenal incidentalomas. The size of these tumors is variable and can vary from a few millimeters to more than 10 cm when they are called giant adrenomyelolipomas. The most common clinical presenting symptoms of adrenal myelolipomas are as follows: Abdominal pain: 22.5%, hypochondrial pain: 13.9%, flank pain: 13.9%, abdominal mass: 5.2%, and rarely, dyspnea, back pain, fever, weight loss and virilization. Presentation of the case: 61-year-old male, with the presence of a 15 x 12 cm tumor, dependent on the right adrenal gland, compressing the upper pole of the right kidney, -20 HU, compatible with giant right adrenal myelolipoma, incidental findings, during study protocol due to prostatic growth, a laparoscopic adrenal tumor resection procedure is performed, with excellent clinical results, minimal bleeding and immediate recovery. Conclusion: adrenal myelolipoma is a benign tumor that is diagnosed incidentally in most cases, it is known to be non-functional clinically, although preoperative hormonal studies should always be done. The treatment of adrenal myelolipomas depends on each clinical case, being a recommendation the surgical treatment of large tumors> 5 cm, we suggest that the laparoscopic approach be chosen, as it is the least invasive and with the lowest rate of complications, in addition of the prompt recovery of the patient.

Splenic myelolipoma in a dog / Mielolipoma esplênico em um cão

ABSTRACT: Myelolipoma is a benign neoplasm composed of mature adipocytes and hematopoietic elements. This tumor is rare in dogs and cats and can develop in several organs and tissues. This report describes a case of splenic myelolipoma in a dog characterizing its clinical-pathological and ultrasonography aspects. A 9-year-old, female, mixed-breed, castrated dog was referred to the veterinary hospital with increased abdominal circumference. Ultrasonography revealed splenomegaly with masses of not measurable dimensions along the entire length of the parenchyma. The spleen was removed, and a fragment was sent for histopathological analysis. Macroscopically, the spleen was enlarged, with irregularly nodular growths that project above the surface of the organ. Nodules were red with small yellow areas. Microscopically, the spleen was effaced by a well-demarcated and not encapsulated mass composed of histologically well-differentiated neoplastic adipose tissue with islands and nests of varying proportions of hematopoietic elements. Histological examination confirmed the diagnosis of myelolipoma. Myelolipomas are observed on ultrasound images as hyperechoic masses or nodules with homogeneous echo texture. Here, ultrasound appearance involved mixed echogenicity and heterogeneous echotexture, due to the mixture of fat and non-fatty material components of the tumor.

RESUMO: Mielolipoma é uma neoplasia composta por adipócitos maduros e elementos hematopoiéticos. Este tumor é raro em cães e gatos e pode se desenvolver em diversos órgãos e tecidos. Descreve-se um caso de mielolipoma esplênico em um cão caracterizando seu aspecto clínico-patológico e ultrassonográfico. Um canino, fêmea, castrada, SRD, de 9 anos de idade foi encaminhada para o Hospital Veterinário com um aumento na circunferência abdominal. A ultrassonografia abdominal revelou esplenomegalia com massas de dimensões imensuráveis em toda a extensão do parênquima. Foi realizada esplenectomia total e um fragmento do baço foi encaminhado para análise histopatológica. Macroscopicamente o baço estava aumentado de tamanho, com crescimentos nodulares irregulares que se projetam acima da superfície do órgão. Os nódulos eram vermelhos com pequenas áreas amarelas. Microscopicamente foi observada uma massa bem demarcada e encapsulada composta por tecido adiposo neoplásico bem diferenciado, com ilhas e ninhos de elementos hematopoiéticos de proporção variada. O diagnóstico foi confirmado pelo exame histopatológico. Mielolipomas são observados nas imagens ultrassonográficas como massas hiperecóicas ou nódulos com ecotextura homogênea. Neste caso, o achado ultrassonográfico mostrou ecogenicidade mista e ecotextura heterogênea, provavelmente devido à mistura dos componentes do tumor com material gorduroso e não gorduroso.

Mielolipoma suprarrenal / Adrenal myelolipoma

Introducción: Los mielolipomas suprarrenales son tumores benignos inusuales compuestos por tejido adiposo maduro y células hematopoyéticas. Objetivo: Presentar un nuevo caso de mielolipoma suprarrenal y describir sus características clínicas, imaginológicas y la terapéutica aplicada. Caso clínico: Paciente femenina de 68 años de edad, con antecedentes de hipertensión arterial, diabetes mellitus tipo 2 y obesidad mórbida, que ingresó en el Servicio de Cirugía General del Hospital Militar Central Dr. Carlos J. Finlay, por presentar dolor en el hipocondrio derecho. Al examen físico presentaba ligero tinte ictérico de piel y mucosas, dolor abdominal difuso a predominio de hipocondrio derecho, sin reacción peritoneal, signo de Murphy negativo. En el tórax se auscultaron crepitantes bibasales y el murmullo vesicular disminuido. Los exámenes de analítica sanguínea demostraron pruebas hepáticas con valores elevados y eritrosedimentación acelerada. La tomografía axial computarizada abdominal reveló una tumoración, que se correspondía conla glándula suprarrenal derecha, que medía110x135x90 mm. Se realizó la suprarrenalectomía derecha que transcurrió sin complicaciones. El diagnóstico histopatológico fue mielolipoma suprarrenal. La paciente evolucionó satisfactoriamente. Conclusiones: El mielolipoma suprarrenal no presenta un cuadro clínico característico y en muchas ocasiones su hallazgo es incidental. La tomografía axial computarizada es la técnica de elección para el diagnóstico imaginológico de esta lesión. En las masas mayores de 6 cm está indicado el tratamiento quirúrgico, y la cirugía convencional, es un método útil para este fin(AU)

Introduction: Adrenal myelolipomas are unusual slow-growing benign tumors, composed of mature adipose tissue and hematopoietic cells. Objective: To present a new case of adrenal myelolipoma as well as to describe its clinical, imaging and applied therapeutic characteristics. Clinical Case: Female patient of 68 years old, with a history of hypertension, diabetes mellitus type 2 and obese, who was admitted to the General Surgery Service of the Hospital Militar Central Dr. Carlos J. Finlay, for presenting pain in the right hypochondrium. In the general physical examination, she presented a morbid obesity with a slight icteric dye of the skin and mucous membranes, and in the abdomen diffuse abdominal pain was observed, predominantly in the right hypochondrium without peritoneal reaction and negative Murphy sign; no palpated visceromegalias. Bi-basal crackles were heard in the thorax with diminished vesicular murmur. The blood tests performed showed liver tests with high values, and accelerated erythrosedimentation. In the computerized axial tomography an abdominal tumor was observed that impressed to correspond with the right adrenal gland, and that it mediates 110x135x90 mm. The right adrenalectomy was performed without complications. The histopathological diagnosis was an adrenal myelolipoma. The patient has evolved satisfactorily. Conclusions: Adrenal myelolipoma does not present a characteristic clinical picture, so its finding is often incidental. Computed tomography is the technique of choice for the diagnosis of this lesion. In the masses greater than 6 centimeters, surgical treatment is indicated, with conventional surgery being a useful route for this(AU)

Very rare giant adrenal myelolipoma: a tumor mimic

Adrenal myelolipoma is a very rare benign neoplasm usually unilateral, and nonsecreting, occurring in the adrenal gland which consist of fatty tissues and hematopoietic elements. Some cases of this type may cause symptoms like chronic abdominal pain however usually these tumors are small and asymptomatic. Surgery is the modality of treatment where there are severe symptoms with life threatening progression with size of tumor reaching more than 6 cm. Here we report an unusual case of a 59-year-old male with history of chronic abdominal pain from a growing right-sided large adrenal mass. With all possible hematological, histo-pathological as well as imaging investigations warranted a high suspicion for tumor mass, hence decided for surgically excision, which was both diagnostic as well as curative. The histopathological study of the excised mass confirmed the initial diagnosis of adrenal myelolipoma. Surgery is the diagnostic and curative modality of treatment for adrenal myelolipoma after all possible investigations to reach for a clinical diagnosis as well for proper planning for surgery. By this we can prevent complications like spontaneous rupture and hemorrhage of the mass, and subsequent cardiovascular shock. Management varies from individual to individual. Open radical adrenalectomy is the standard treatment for giant myelolipomas (size >10 cm), while the minimally invasive approach has been used in only few cases of small sized tumors. Although it is a benign tumor, surgery plays main role for symptomatic cases and high suspicion of malignancy.

Mielolipoma suprarrenal: doce años de experiencia en su diagnóstico, tratamiento y seguimiento / Adrenal myelolipoma: twelve-years' experience in diagnosis, treatment and follow-up

Introducción: Los mielolipomas suprarrenales son tumores benignos e infrecuentes, formados por tejido adiposo y hematopoyético. Se consideran incidentalomas porque se diagnostican fortuitamente en estudios de imagen investigando síntomas abdominales o lumbares o en chequeos rutinarios. Son hormonalmente inactivos casi siempre. Se operan si presentan gran tamaño y usualmente se mantienen estables durante su evolución natural. Objetivos: Identificar las características de los mielolipomas suprarrenales y describir su evolución natural. Métodos: Se realizó un estudio descriptivo retrospectivo en 17 pacientes con mielolipomas suprarrenales diagnosticados por tomografía axial computarizada entre enero de 2006 y abril de 2018. Se estudiaron variables clínicas, hormonales y tomográficas al inicio en 17 pacientes y evolutivamente en 5 pacientes no operados. Se utilizaron medidas de resumen para las variables cualitativas (número y porcentajes) y para las cuantitativas (media y desviación estándar). Resultados: La edad promedio fue 52,9 años y la distribución por sexo: 13 mujeres y 4 hombres (razón 3,2:1). Se indicó tomografía axial computarizada en 11 pacientes por síntomas dolorosos. El tamaño promedio fue 5,6 cm. En 8 tumores el diámetro fue de 6 cm y más. En 9 pacientes se realizó adrenalectomía laparoscópica. Durante la evolución natural, que promedió 5 años y 1 mes, una paciente presentó crecimiento de sus dos masas bilaterales; la derecha se extirpó por sobrepasar los 6 cm. Conclusiones: Los mielolipomas suprarrenales fueron benignos, alcanzaron gran tamaño y provocaron síntomas dolorosos. Fueron hormonalmente inactivos en su mayoría. Evolutivamente, el crecimiento fue muy infrecuente y no hubo transformación maligna ni desarrollo de hiperfunción endocrina(AU)

Introduction: Suprarenal myelolipomas are infrequent benign tumors formed by adipose and hematopoietic tissue. They are considered to be incidentalomas because they are found and diagnosed accidentally in imaging studies intended for abdominal or lumbar problems, or in routine checkups. Suprarenal myelolipomas are almost always hormonally inactive. They are operated on when they are large, and they usually remain stable during their natural evolution. Objectives: Identify the characteristics of suprarenal myelolipomas and describe their natural evolution. Methods: A retrospective descriptive study was conducted of 17 patients with suprarenal myelolipomas diagnosed by computerized axial tomography from January 2006 to April 2018. Clinical, hormonal and tomographic variables were analyzed initially in 17 patients and evolutionarily in 5 non-operated patients. Summary measurements were used for qualitative variables (number and percentages) and for quantitative variables (mean and standard deviation). Results: Mean age was 52.9 years and sex distribution was 13 women and 4 men (ratio of 3.2:1). Computerized axial tomography was indicated for 11 patients with pain symptoms. Average size was 5.6 cm. In 8 tumors the diameter was 6 cm or more. Laparoscopic adrenalectomy was performed on 9 patients. During natural evolution, which averaged 5 years and 1 month, one female patient experienced growth of her two bilateral masses, and the one on the right side was removed for it exceeded 6 cm. Conclusions: The study suprarenal myelolipomas were large, benign and caused pain symptoms. Most were hormonally inactive. In evolutionary terms, growth was very infrequent and there was no malignant transformation or development of endocrine hyperfunction(AU)

Retroperitoneal Laparoscopic Surgery for Giant Adrenal Myelolipoma / 中国微创外科杂志

Objective To explore the surgical features of retroperitoneoscopic excision of adrenal myelolipoma with diameter larger than 6 cm. Methods We retrospectively analyzed clinical data of 28 cases of giant adrenal myelolipoma from March 2010 to December 2015.The diameter of tumor was 6.0-13.7 cm (mean, 8.5 cm).There were 10 left-sided cases and 18 right-sided cases. During the retroperitoneoscopic excision of adrenal myelolipoma , four trocars were used .Two silk sutures were twisted as a loop to entangle one side of the tumor and then pulled it .Blood vessel on the surface of tumor was sealed and cut by ultrasonic scalpel . Adrenal gland was totally or partially removed and the tumor was resected completely . Results The operations were successful in all the 28 cases without hemorrhage during or after the surgery , conversion to open surgery , or injury of adjacent organs .The operation time was 52-117 min (mean, 67.5 min) and the blood loss was 45-110 ml during operation (mean, 60.5 ml).Patients took food 1-2 days after operation and ambulated 3-5 days after operation .The indwelling time of retroperitoneal drainage tube was 3-6 d (mean, 4.1 d).The postoperative hospitalization time was 6 -9 d (mean, 7.5 d). Conclusions Giant myelolipoma leads to seriously changed normal anatomy and is hard to be exposed during operation .Only when the important anatomical landmarks , large blood vessels and adrenal glands are focused , can the operation be safe and controllable .

Clinicopathological analysis of myelolipoma within adrenocortical adenoma / 医学研究生学报

Objective At present, there are few studies about myelolipoma within adrenal cortical adenoma.Our aim was to provide more basis for correct diagnosis and treatment by investigation into its clinical and pathological features.Methods The clinical and pathological data were retrospectively reviewed in 11 patients of myelolipoma within adrenal cortical adenoma, along with relative literature reviews.Results The median age of 11 patients (7 females, 4 males) was 49±9.5 years, among whom 3 patients presented Cushing's syndrome, 1 patient with more than 10 years' recurrent dizzy with hypertension, other 7 patients were found coincidently by routine examination.Adrenal mass were found by imaging examination.Pathologically, myelolipomas were in solitary nodule distribution and/or admixed with adrenal cortical adenomas.Myelolipomas were composed of variable admixture of mature adipose tissue and hematopoietic elements.Surgical treatment was performed for all 11 patients, and no relapse was found in 2 months' to 11 years' follow-up.Conclusion Myelolipoma within adrenal cortical adenoma is extremely rare, which is common in females.The patients may present with Cushing's syndrome, hypertension or without obvious clinical syndrome.All the patients are in favorable prognosis after surgical resection.

Mielolipoma adrenal gigante: Adrenalectomía derecha laparoscópica / Giant adrenal myelolipoma. Right laparoscopic adrenalectomy

El mielolipoma es un tumor adrenal poco frecuente. Se trata de un tumor benigno no funcionante compuesto por tejido adiposo y hematopoyético. Presentamos el caso de una paciente de 33 años de edad con diagnóstico de incidentaloma adrenal de 14 cm de diámetro no funcionante. Los estudios imagenológicos presentaban características sugestivas de mielolipoma. Debido a la naturaleza benigna del tumor se decidió el abordaje laparoscópico. La anatomía patológica informó un mielolipoma de 444 gramos. La paciente evolucionó favorablemente en el postoperatorio. El abordaje laparoscópico para la resección del mielolipoma gigante fue factible y exitoso.

Adrenal myelolipoma is a rare, benign, non-functioning tumor. It is composed by fat and hematopoietic tissues. We present the case of a 33-year-old woman with diagnosis of a 14 cm diameter non-functioning right adrenal incidentaloma, with imaging features suggestive of myelolipoma. Based on the benign nature of the tumor, laparoscopic resection was performed. Histopathology showed a myelolipoma, weighting 444 grams. The patient evolved with an uneventful postoperative period. Laparoscopic adrenalectomy for a giant myelolipoma was feasible and successful.

Hiperplasia suprarrenal congénita y mielolipoma: a propósito de un caso / Congenital adrenal hyperplasia and myelolipoma: a case report

Objetivo: Presentar caso clínico de paciente con hiperplasia suprarrenal congénita (HSC) y mielolipoma bilateral, debido a su baja incidencia en la población. Caso Clínico: Paciente femenina de 32 años, con diagnóstico de HSC clásica perdedora de sal en tratamiento regular con glucocorticoides y mineralocorticoides hasta los 14 años. En febrero 2012 presenta aumento progresivo de circunferencia abdominal, a los 2 meses, dolor punzante en ambos flancos, de leve a moderada intensidad, no irradiado, por lo cual consulta. Laboratorio: ACTH: 102 pg/ml (hasta 37); 17 OH progesterona: 20,47 mg/ml (0,2-2,9); Testosterona libre: 7,6 pg/ml (0.01-7.01); Androstenediona: 26,1 ng/ml (0,94-3,2); DHEA-SO4: 53,7 μg/dl (65-368). Se realiza tomografía axial computarizada de abdomen evidenciando lesión ocupante de espacio en ambas glándulas suprarrenales. Derecha: 16,4x14,2x12,2 cm, izquierda: 20x13x11,2 cm, ambas de densidad heterogénea, sugestivas de mielolipomas. Se realiza laparotomía media xifopúbica en enero 2013 con adrenalectomía bilateral, tumor izquierdo de 1400 g y derecho de 1200 g. La biopsia reportó mielolipomas bilaterales. Conclusión: Tanto la HSC como los mielolipomas son patologías poco frecuentes, pueden estar asociadas en pacientes con HSC clásica que cumplen tratamiento irregular o lo omiten. Los mielolipomas representan aproximadamente el 11% de los tumores adrenales. La incidencia varía desde 0,08 a 0,4%, la edad media de presentación es 50 años. El sexo masculino predomina en una proporción 2:1. La mayoría son unilaterales, solitarios y pequeños. Debido a la baja incidencia de mielolipomas, la forma de presentación de la paciente y su asociación con HSC clásica, se trae a revisión.

Objective: To describe the case of patient with congenital adrenal hyperplasia (CAH) and bilateral myelolipoma at presentation, due to its low incidence in the population. Clinical case: Thirty-two year old female patient, with a diagnosis of classic congenital adrenal hyperplasia with salt-wasting adrenal, on regular treatment with glucocorticoids and mineralocorticoids until 14 years of age. On February 2012, she presents progressive increase of abdominal circumference, 2 months later stabbing pain in both flanks, from mild to moderate intensity, with no irradiation, reasons for medical consultation. Lab test: ACTH: 102 pg/ml (until 37); 17OH progesterone: 20.47 mg/ml (0.2-2.9); free testosterone: 7.6 pg/ml (0.01-7.01); androstenedione: 26.1 ng/ml (0.94-3.2); DHEA-SO4: 53.7 μg/dl (65-368). An abdominal CT-Scan is preformed and a space-occupying lesion on both adrenal glands is observed. Right: 16.4x14.2x12.2 cm, left:20x13x11.2 cm, both have heterogeneous density, suggestive of myelolipoma. A midline xyphopubic laparotomy is performed on the patient in January 2013, with bilateral adrenalectomy, with the left tumor of 1400g and the right one of 1200g. The biopsy reported bilateral myelolipomas. Conclusion: CAH and myelolipomas are both very infrequent pathologies that may appear together in patients with classic CAH diagnosis associated to irregular or complete abandonment of treatment. Myelolipomas represent approximately 11% of adrenal tumors. Their incidence varies between 0.08% and 0.4%, with median age presentation at 50 years. Male sex is predominant (2:1). Most are unilateral, solitary and small. Due to myelolipomas low incidence, its form of presentation in this patient and its association with CAH, this case is being reviewed.

Blepharoptosis and hypertrophic osteoarthropathy: A case report.

A 52‑year‑old male patient presented to our hospital with a history of secondary hypertrophic osteoarthropathy (HOA) associated with an abdominal neoplasia and blepharoptosis. He had finger clubbing, hyperhidrosis, and hypertrichosis. He also had a recent history of extensive abdominal surgery with a pathology report of myelolipoma. Routine blood work was unremarkable. Upper eyelid reconstruction with blepharoplasty, upper eyelid wedge resection, and brow suspension was performed to address his eyelid concerns. By this case report, we would like to attract notice that the eyelid involvement may be a part of HOA and to emphasize the importance of systemic and pathologic evaluation in failed blepharoptosis surgery.

Cirugía laparoscópica en un paciente con mielolipoma suprarrenal derecho / Laparoscopic surgery in a patient with right adrenal myelolipoma

Los mielolipomas suprarrenales son tumores benignos inusuales de crecimiento lento, compuestos por tejido adiposo maduro y células hematopoyéticas. Se presentan en adultos entre la quinta y la sexta década de la vida y usualmente miden entre 3 y 7 cm. El 70 % de los casos son asintomáticos y hacen parte de los denominados 'incidentalomas' de la glándula suprarrenal. El tratamiento puede ser conservador o quirúrgico, dependiendo de los síntomas, el tamaño de la masa y el crecimiento tumoral en estudios consecutivos de imágenes diagnósticas. La suprarrenalectomía laparoscópica es un procedimiento seguro, que tiene una recuperación rápida, no requiere instrumentos, dispositivos o fuentes de energía costosos, y estéticamente es superior a la técnica abierta. Se presenta el caso de un hombre de 63 años con antecedentes de hipertensión arterial sistémica, diabetes mellitus de tipo 2 e hiperlipidemia, quien presentó como hallazgo incidental en imágenes diagnósticas una masa suprarrenal derecha. El examen físico y los estudios paraclínicos descartaron que el tumor fuera funcional. Se practicó suprarrenalectomía derecha laparoscópica sin complicaciones y se diagnosticó un mielolipoma por histopatología.

Adrenal myelolipomas are rare slow-growing benign tumors composed of mature adipose tissue and hematopoietic cells. They most often occur in adults in the 5th-6th decade, and usually measure between 3 and 7 cm; 70% of cases are asymptomatic and are part of the so called incidentalomas of the adrenal gland. Treatment can be conservative or surgical, depending on the symptoms, the size of the mass and the growth of the tumor in consecutive imaging studies. We report a 63-year old man with history of systemic arterial hypertension, diabetes mellitus type 2 and hyperlipidemia. A right adrenal mass was reported as incidental finding in diagnostic imaging. Physical examination and paraclinical studies ruled out a functioning tumor. Right laparoscopic adrenalectomy was performed without complications and a myelolipoma was diagnosed by histopathology. Myelolipomas should be included in the differential diagnosis of the adrenal gland incidentalomas. Laparsocopic adrenalectomy is a safe procedure with rapid recovery, does not require special instruments or devices, and is cosmetically superior to the open technique.

Giant retroperitoneal myelolipoma: case report and literature review / Mielolipoma gigante em retroperitônio: relato de caso e revisão da literatura

Adrenal myelolipomas are unusual benign tumors with an average age of 60 years at onset, often associated with adrenal gland. A 63-year-old female presenting with abdominal discomfort and a large expanding mass in retroperitoneum occupying the right hemiabdomen, with extrinsic compression of adjacent organs, underwent tumor resection. Macroscopic examination of the surgical specimen showed a large yellowish homogeneous lesion with areas of hemorrhage, covered by a thin fibrous capsule. Microscopic analysis revealed a neoplasm composed of mature adipocytes permeated by hematopoietic tissue. There was residual adrenal cortex around the lesion.

Mielolipomas são tumores benignos pouco comuns, com média de incidência de 60 anos, frequentemente associados à glândula adrenal. Relata-se caso de paciente do sexo feminino de 63 anos que apresentava quadro clínico de desconforto abdominal e volumosa formação expansiva em retroperitônio, ocupando hemiabdômen direito com compressão extrínseca de órgãos adjacentes, sendo submetida à ressecção tumoral. A análise macroscópica da peça cirúrgica mostrou volumosa lesão homogênea amarelada com áreas de hemorragia, recoberta por fina cápsula fibrosa. A análise microscópica revelou neoplasia constituída por adipócitos maduros permeados por tecido hematopoiético, notando-se cortical da suprarrenal residual na periferia da lesão.

Adrenal myelolipoma with congenital 17a-hydroxylase deficiency: a pedigree report and review of the literature / 中华内分泌代谢杂志

Objective To analyse the clinical and pathological characteristics of congenital adrenal hyperplasia (CAH) with adrenal myelolipoma (AML),and to improve the awareness of CAH.Methods The P450c17a gene in a pedigree with CAH and the clinical and pathological characteristics of three sisters of the pedigree with CAH and adrenal myelolipoma were studied;and the pertinent literatures reported abroad were analyzed and summarized.Results (1) A heterozygous point mutation 1178 base A to T in exon 6,and a heterozygous deletion mutation 1 457-1 465 bases TCGACTCTT of exon 8 were detected in P450c17 (CYP17) gene of three sisters of the pedigree with CAH.(2) The clinical characteristics of three sisters were hypertension,hypokalemia,primary amenorrhea,and gonadal dysgenesis.Without regular treatment after the diagnosis of CAH,the three sisters experienced many years of elevated serum ACTH levels,and developed AML.Conclusion The long-term high ACTH level plays an important role in the development of AML,although the exact mechanism of CAH with adrenal myelolipoma remains still unclear.CAH patients require long-term regular treatment.

A Case of Bilateral Adrenal Myelolipomas with Congenital Adrenal Hyperplasia / 대한내과학회지

Adrenal myelolipoma (AML) is a rare, usually benign, and nonfunctioning tumor. About 7-15% of adrenal incidentalomas are AMLs, composed of normal hematopoietic elements and mature adipose tissue. AML is usually unilateral and < 4 cm. It is often discovered incidentally on abdominal computed tomography or magnetic resonance imaging. It is related to a chronic increase in adrenocorticotropic hormone, such as that observed in patients with congenital adrenal hyperplasia, Cushing disease, Conn's syndrome, and pheochromocytoma. Here, we report a 28-year-old man diagnosed with non-salt-losing congenital adrenal hyperplasia with huge bilateral AMLs and a literature review.

Mielolipoma suprarrenal incidental / Incidental Detection of Adrenal Myelolipoma

La implementación de nuevas tecnologías y la ampliación de la cobertura a nivel de imágenes, han facilitado encontrar casos incidentales, siendo el más común el adenoma suprarrenal no funcionante. El mielolipoma suprarrenal es una neoplasia benigna, rara, en la mayoría de casos asintomática compuesta por tejido adiposo maduro y elementos hematopoyéticos. Se presenta un caso clínico de una paciente remitida al servicio de radiología por presentar en ecografía previa, una masa en región suprarrenal derecha incidentalmente. La paciente se encontraba asintomática, por lo cual se realiza tomografía axial computarizada abdominal. A la paciente por su ausencia de síntomas se le continúo con seguimiento periódico sin detectar cambios evolutivos (MÉD.UIS. 2014;27(2):105-107) .

The implementation of new technologies and expanding coverage to level of images, have provided incidental finding cases, the most common nonfunctioning adrenal adenoma. Myelolipoma adrenal benign tumors, rarely, in most cases consisting of asymptomatic mature adipose tissue and hematopoietic elements (myeloid and erythroid cells). We present a case of a patient referred to the radiology department for filing in previous ultrasound a right adrenal mass incidentally region. The patient was asymptomatic, which is done by abdominal CT. A patient by the absence of symptoms is continued with regular monitoring undetected evolutionary changes (MÉD.UIS. 2014;27(2):105-107).

Mielolipoma. Presentación de un caso / Myelolipoma. Presentation of a case

Se presenta el caso de un paciente de 44 años de edad, del sexo femenino, ingresado en el Servicio de Cirugía del Hospital Militar Docente Dr. Mario Muñoz Monroy, de Matanzas, porque se encontraba bajo seguimiento de un absceso hepático de probable etiología amebiana, en cuyos exámenes complementarios, dados por el ultrasonido abdominal, así como la tomografía contrastada de abdomen se aprecia una tumoración de la glándula suprarrenal derecha, resultando finalmente un mielolipoma. Se somete a resección quirúrgica de la glándula de forma exitosa, confirmando el estudio histopatológico, un tumor de glándula suprarrenal derecha, correspondiente a un mielolipoma, se obtiene una evolución satisfactoria de la paciente.

We present the case of a 44-years-old female patient who entered the Surgery Service of the Matanzas Military Hospital, Dr. Mario Muñoz Monroy, because of she was followed for a hepatic abscess of probably amoebic etiology. The complementary examination, abdominal ultrasound and abdominal contrasted tomography, showed a tumor of the right suprarenal gland, finally resulting a myelolipoma. The gland was successfully resected, and the histopathologic study confirmed a right suprarenal gland tumor, correspondent to a myelolipoma. The evolution of the patient was satisfactory.

Surgical Approach of the Rare Benign Adrenal Tumor / 대한내분비외과학회지

PURPOSE: Benign adrenal tumors other than hyper-functioning tumor and non-functioning cortical adenoma are extremely rare. The purpose of this study is to review the clinical features of these rare benign adrenal tumors and to analyze their prevalence and the proper surgical approach for them. METHODS: Among patients who underwent adrenalectomy in Samsung Medical Center, between 1997 and 2013, patients with benign adrenal tumor except non-functioning cortical adenoma, malignancy, and hyper-functioning tumor such as pheochromocytoma, aldosteronoma, and cortisol producing tumor were included. Clinical details, radiologic findings and pathologic findings as well as data associated with the surgical procedure were analyzed retrospectively. RESULTS: The percentage of these tumors among 703 surgically removed adrenal tumors was 10.7% (75 cases). They included 30 adrenal cysts, 18 ganglioneuromas, 11 myelolipomas, seven schwannomas, and the other six were rare adrenal tumors. Approximately 40% of the patients were operated under a diagnosis different from original tumors. Operation time and hospital stay of patients who underwent laparoscopic adrenalectomy were shorter than those of patients who underwent open adrenalectomy. CONCLUSION: These benign adrenal tumors are very rare and difficult to diagnose preoperatively. For the surgical treatment of these tumors, laparoscopic adrenalectomy is more preferable to conventional open adrenalectomy.