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

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)

Prevalencia de mielolipoma como diagnóstico incidental de masas adrenales en tomografías abdominales realizadas en el Hospital Universitario San Vicente Fundación de Medellín / Prevalence of adrenal myelolipoma during abdominal computed tomography at Hospital Universitario San Vicente Fundación

RESUMEN Objetivo: el mielolipoma es un tumor suprarrenal poco frecuente, benigno y no funcional. Representa el 2,6-3 % de los casos reportados de masas adrenales incidentales. Es más frecuente en adultos entre la quinta y séptima década de la vida. El objetivo del estudio es evaluar la prevalencia de mielolipoma como masa adrenal incidental en tomografías abdominales en 1 año, en un hospital universitario de cuarto nivel. Metodología: estudio retrospectivo. Se revisaron todas las tomografías de abdomen simples o contrastadas realizadas en el periodo 2014 al 2015 en el Hospital Universitario San Vicente Fundación de Medellín. De estas, se extrajeron las que reportaban masas adrenales incidentales con diagnóstico de mielolipoma. Se revisaron las historias clínicas de los pacientes con este hallazgo y sus características clínicas respectivas. Resultados: se revisaron 4.288 estudios tomográficos de abdomen correspondientes a 3.709 pacientes. Se encontraron 108 masas adrenales incidentales y los mielolipomas fueron el 1,85 % de estos. En los pacientes evaluados con TAC abdominal por diferentes razones, la posibilidad de identificar mielolipomas fue de 0,05 %. El promedio de edad de presentación fue 57 años. Ambos casos de mielolipomas fueron masas unilaterales, derechas, con un tamaño promedio de 31 mm y con un promedio de -84,5 unidades Hounsfield (UH). En ningún caso se realizó estudio hormonal o de manejo quirúrgico. Discusión: en este estudio se presenta la prevalencia y características clínicas del mielolipoma adrenal con presentación inicial como masas adrenales incidentales. Se encontró que el 1,85 % de las masas adrenales incidentales tienen como etiología el mielolipoma. En nuestro conocimiento no hay estudios en Colombia que evalúen la prevalencia de mielolipoma en TAC abdominales. En conclusión, el mielolipoma es un tumor adrenal infrecuente.

SUMMARY Objective: Adrenal myelolipoma is an infrequent benign tumor. Myelolipoma represents 2,6-3 % of all adrenal incidentalomas. This tumor is more frequent in aged individuals. Our aim was to evaluate the prevalence of myelolipoma in patients with adrenal incidentaloma detected by abdominal computed tomography (CT) during 1 year at a university tertiary care hospital. Methods: Retrospective study. All abdominal CT done at the Hospital Universitario San Vicente Fundación, Medellín, Colombia, with or without contrast, were reviewed from 2014 to 2015. Of all adrenal incidentalomas, those with diagnosis of adrenal mielolipoma were further evaluated. The clinical and laboratory data were extracted. Results: 4288 abdominal CT were reviewed in 3709 patients. 108 adrenal incidentalomas were found. Of those, two cases were adrenal myelolipomas. The frequency of mielolipoma presenting as adrenal incidentalomas was 1.85 % with a prevalence in abdominal CT of 0.05 %. The mean age at presentation was 57 years. Both cases were unilateral right masses; the mean size was 31 mm and a mean of -84.5 Hounsfield units. Hormonal studies or surgical management was no performed. Discussion: This study report the prevalence and clinical characteristics of adrenal myelolipoma presenting as adrenal incidentalomas. 1.85 % of adrenal incidentalomas are myelolipomas. To our knowledge, there are no studies in Colombia evaluating the prevalence of this disease in abdominal CT scans. In conclusion, myelolipoma is an infrequent adrenal tumor.

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)

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.

Mielolipoma adrenal. Reporte de caso y revisión de la literatura / Adrenal myelolipoma. Case reportand review of literature

El mielolipoma adrenal es un tumor benigno de etiología desconocida y de presentación infrecuente. Generalmente asintomático y poco asociado a producción hormonal. Conlleva dificultades diagnósticas y terapéuticas de acuerdo a cada caso en particular. Se presenta el caso de un mielolipoma adrenal derecho en mujer adulta eritrocitica, obesa, hipertensa, con síndrome adherencial severo secundario a múltiples cirugías abdominales. Planteó dificultad diagnóstica y terapéutica por asociación a elevaciones de presión arterial y posibilidad de tumor funcionante.

Mielolipoma en una paciente con hiperplasia suprarrenal congénita / Adrenal myelolipoma in a patient with congenital adrenal hyperplasia

Introducción: El mielolipoma es un tumor benigno caracterizado por la presencia de tejido adiposo y elementos de la médula ósea. Se ha visto que puede estar relacionado con niveles elevados de ACTH como en la Hiperplasia Suprarrenal Congénita (HSC). Objetivo: Presentación de un caso clínico. Reporte de caso: Mujer de 64 años de edad con antecedente de Hiperplasia Suprarrenal Congénita en la que se diagnostica de forma incidental un mielolipoma. Conclusiones: Ante lesiones mayores a 5 centímetros, sintomáticas o que sufren algún cambio (clínico o radiológico) durante el seguimiento, se debería considerar el tratamiento quirúrgico con abordaje laparoscópico, el cual sería la mejor opción.

Introduction: Myelolipoma is a benign tumor characterized by the presence of fat and bone marrow elements. We have seen that may be related to elevated levels of ACTH and Congenital Adrenal Hyperplasia (CAH). Objective: Presentation of a case report. Case report: Female 64 years old with a history of congenital adrenal hyperplasia where incidentally diagnosed myelolipoma. Conclusions: In lesions larger than 5 inches, symptomatic or suffering any change (clinical or radiological) during follow-up, should consider surgical treatment with laparoscopic approach, which would be the best option

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.

Endoscopic Ultrasound-Guided Fine-Needle Aspiration of the Adrenal Glands: Analysis of 21 Patients

BACKGROUND/AIMS: Endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) cytology of adrenal masses helps in etiological diagnosis. The aim of this study was to evaluate the diagnostic yield of EUS-FNA of adrenal masses in cases where other imaging methods failed and/or were not feasible. METHODS: Twenty-one consecutive patients with adrenal masses, in whom adrenal FNA was performed because conventional imaging modalities failed and/or were not feasible, were prospectively evaluated over a period of 3 years. RESULTS: Of the 21 patients (mean age, 56+/-12.2 years; male:female ratio, 2:1), 12 had pyrexia of unknown origin and the other nine underwent evaluation for metastasis. The median lesion size was 2.4x1.6 cm. Ten patients were diagnosed with tuberculosis (shown by the presence of caseating granulomas [n=10] and acid-fast bacilli [n=4]). Two patients had EUS-FNA results suggestive of histoplasmosis. The other patients had metastatic lung carcinoma (n=6), hepatocellular carcinoma (n=1), and adrenal lipoma (n=1) and adrenal myelolipoma (n=1). EUS results were not suggestive of any particular etiology. No procedure-related adverse events occurred. CONCLUSIONS: EUS-FNA is a safe and effective method for evaluating adrenal masses, and it yields diagnosis in cases where tissue diagnosis is impossible or has failed using conventional imaging modalities.

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.

Bilateral Video-Assisted Thoracoscopic Surgery Resection for Multiple Mediastinal Myelolipoma: Report of a Case

Myelolipoma in the mediastinum is an extremely rare entity. In this report, we present the case of a 79-year-old asymptomatic man who had three bilateral paravertebral mediastinal tumors. The three tumors were resected simultaneously using bilateral three-port video-assisted thoracoscopic surgery (VATS). There has been no evidence of recurrence within four years after the operation. Multiple bilateral mediastinal myelolipomas are extremely rare. There are no reports in the English literature of multiple bilateral thoracic myelolipomas that were resected simultaneously using bilateral VATS. We also present characteristic features of myelolipomas, which are helpful for diagnosis.

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.

Mielolipoma, una lesión quirúrgica poco frecuente de la glándula adrenal / Myelolipoma, a rare surgical lesion of the adrenal gland

El mielolipoma adrenal es un tumor benigno poco frecuente, compuesto de tejido adiposo maduro y elementos hematopoyéticos diversos. Estos tumores pueden localizarse en las glándulas adrenales aunque también pueden encontrarse en sitios extradrenales. Las manifestaciones clínicas son variadas tales como sangramientos digestivos, dolor abdominal en los flancos, masa abdominal palpable, hematuria, hemorragia retroperitoneal y pérdida de peso. Entre los métodos diagnósticos figuran el ultrasonido, la tomografía axial computarizada y la resonancia magnética. La resección quirúrgica es el tratamiento de elección, sobre todo cuando haya sintomatología o en tumores mayores de 4 cm, por el riesgo de ruptura espontánea con hemorragia retroperitoneal. Presentamos la historia clínica de una paciente de 42 años de edad con antecedentes de dolores en hipocondrio derecho de aproximadamente un año de evolución, la cual sufrió un cólico nefrítico que cedió con tratamiento médico. Posteriormente en la consulta externa, en el examen fisico, se constata una masa tumoral en hipocondrio derecho, la cual fue diagnosticada por ultrasonido y tomografía axial computarizada como un tumor adrenal. Se intervino la paciente y se confirma el diagnóstico. La evolución posoperatoria fue buena y la paciente continúa asintomática. El objetivo de este trabajo es presentar un nuevo caso de esta enfermedad, poco frecuente, la cual fue diagnosticada en una paciente de 42 años de edad y realizar una breve revisión de la literatura(AU)

Mielolipoma is a rare benign tumor of the adrenal gland, which is formed by mature adipose tissue and several hematopoietic elements. They are generally located in the adrenal glands, although they can occur in other sites. The clinical manifestations are varied such as gastrointestinal bleeding, abdominal pain in the flanks, abdominal mass, retroperitoneal hemorrhage, hematuria and loss of weight. Among the diagnosing methods are ultrasound, computerized axial tomography and magnetic imaging resonance. The surgical excision is the treatment of choice, mainly when symptoms are present or in case of over 4 cm tumors, because of the spontaneous rupture risk with retroperitoneal hemorrhage. The objective of this paper was to present a new case of this rare disease together with a brief literature review. A 42 years-old female with a history of abdominal pain located in the right upper quadrant of the abdomen, who suffered nephritic colic that was successfully treated. The physical exam at the outpatient service revealed a tumorous mass diagnosed as adrenal tumor by means of ultrasound and axial computerized tomography. She was operated on and the diagnosis was confirmed. Her postoperative recovery was good and the patient is still asymptomatic(AU)

Left-sided giant adrenal myelolipoma secreting catecholamine.

Adrenal myelolipoma (AML) is a rare benign tumor composed of mature adipose and hematopoietic tissue. Most of these patients are asymptomatic and the tumors are non-secreting. We present a case with a large functional adrenal myelolipoma, wherein the patient was hypertensive and biochemistry revealed increase in 24 hours urinary Vanillylmandelic Acid (VMA), a metabolite of catecholamine. The mass was removed surgically and diagnosed as adrenal myelolipoma on histopathological examination. Both his blood pressure and urinary VMA returned to normal following surgery, which suggested that the mass was functioning and was secreting catecholamine. To the best of our knowledge, a catecholamine secreting adrenal myelolipoma has been reported in the literature only once previously. The association of hypertension and adrenal myelolipoma may not be entirely coincidental, as it may be associated with secreting catecholamine, as seen in our case. We also review the literature on functioning adrenal myelolipoma.

Mielolipoma suprarrenal gigante / Giant adrenal myelolipoma

Myelolipomas are rare benign tumors mostly located in the adrenal glands. They are usually asymptomatic with just a few millimeters in size, but they can grow over 30 cm becoming a life threat for the patient. This is a case of a 49 years old male patient who was diagnosed with a giant myelolipoma of the right adrenal gland while being studied for other reasons.

Los mielolipomas son tumoraciones benignas poco frecuentes con mayor localización en las glándulas suprarrenales. Su tamaño puede variar desde unos pocos milímetros hasta varias decenas de centímetros y dependiendo del tamaño pueden llegar a ser sintomáticos. Se presenta el caso de un paciente masculino de 49 años al que incidentalmente se le diagnostica un mielolipoma suprarrenal derecho gigante de 22 x 15 x 10 cm.

Pulmonary-bronchus myelolipoma and review on extra-adrenal myelolipomas in Chinese literature / 中华医学杂志(英文版)

Extra-adrenal myelolipomas are extremely rare, especially in bronchus and lung. Up to now, only nine cases of intra pulmonary lesions have been reported all over the world. Here we describe a new discovered pulmonary-bronchus myelolipoma in a 53-year-old man, which is different from the previously reported ones. And we mainly comment on the pathology and diagnosis, comparing with the findings of the extra-adrenal cases reported in Chinese literature.

Robotic Adrenalectomy Using a Lateral Transperitoneal Approach: Initial Experience of Fifteen Consecutive Cases / 대한내분비외과학회지

PURPOSE: In this study, we report on our initial experience using the da Vinci-S® robotic surgical system in performance of laparoscopic adrenalectomy. METHODS: Fifteen patients, 11 with left adrenal tumors and four with right adrenal tumors, underwent robotic adrenalectomies using the lateral transperitoneal approach with the da Vinci-S® surgical system at the Korea University Anam Hospital, Seoul, Korea, between October 2009 and May 2012. RESULTS: The mean age of the patients was 45.5 (22~61) years, and the mean body mass index was 23.81 (18.0~28.57) kg/m². Eleven left adrenalectomies, four for primary aldosteronisms, four for pheochromocytomas, two for primary adrenal Cushing's adenoma, and one for myelolipoma, and four right adrenalectomies, two for the primary adrenal Cushing's adenomas, one for pheochromocytoma, and one for primary aldosteronism, were performed robotically. There was no conversion to traditional laparoscopic or open surgery. The mean size of the tumor was 2.54 (1.0~5.5) cm. The mean operative time was 208.2 (120~320) minutes, and the mean console time was 127.6 (75~212) minutes. No major postoperative morbidity was observed, and the mean length of hospital stay was 5.86 days. CONCLUSION: We found that the robotic systems, with magnified stereoscopic three-dimensional vision, elimination of tremor, and the ability to articulate and rotate the instruments, were ideal surgical tools for operation of adrenal lesion. Robotic adrenalectomy may be a safe and effective alternative to traditional laparoscopic adrenalectomy.

Extra-Adrenal Paravertebral Thoracic Myelolipoma

Extra-adrenal myelolipoma is a rare benign tumor which can occur in the abdomen, thorax, or even in the face. We present a case of 58-year-old woman patient with thoracic myelolipoma, which has manifested as three paravertebral masses at the level of T9 and T10. The computed tomography scan showed heterogenous enhancing masses, which contained fat component. She underwent video-assisted thoracoscopic surgery, and the pathologic examination revealed adipose tissue with many hematopoietic cells, consistent with myelolipoma.

Manifestation of Giant Bilateral Symptomatic Adrenal Myelolipomas in an Adult Patient with Congenital Adrenal Hyperplasia

Adrenal myelolipoma is an uncommon non-functioning tumor that is composed of variable amounts of mature adipose tissue and scattered islands of hematopoietic elements, including erythroid, myeloid, lymphoid series, and megakaryocytes. Adrenal myelolipoma should be differentiated from other fat-containing adrenal masses, such as teratoma, lipoma, and liposarcoma. This case report describes a 50-year-old adult who was raised as a male and developed giant adrenal myelolipomas that presented as symptomatic adrenal masses, and which were misdiagnosed as liposarcoma on radiologic examination. The patient had been raised as a male despite ambiguous genitalia, and a thorough investigation was never carried out because of his poor socioeconomic status. Physical examination showed profound short stature (<-3.0 standard deviation score), hyperpigmentation, and a micropenis without palpable gonads. Both a uterus and ovaries were evident in the pelvic cavity on abdominopelvic computed tomography. Adrenocorticotropic hormone stimulation tests confirmed adrenal insufficiency. Steroid replacement therapy was initiated before bilateral adrenalectomy and the histologic findings indicated myelolipoma. The patient's karyotype was 46, XX and mutation analysis of the CYP21A2 gene identified compound heterozygosity consisting of p.I173N and p.Q319*. The patient was treated with once-daily 0.5 mg dexamethasone and once-daily 0.1 mg fludrocortisone. Because the subject had been raised as a male, additional procedures such as an oophorohysterectomy are currently under consideration. We here describe an adrenal myelolipoma in this case that was complicated by a 21-hydroxylase deficiency. We conclude from our analysis that patients with congenital adrenal hyperplasia should be screened for incidental adrenal masses to avoid unnecessary surgical procedures.

Initial Experience of Single Port Transperitoneal Laparoscopic Adrenalectomy / 대한내분비외과학회지

PURPOSE: Single port laparoscopic surgery is an area of active investigation in abdominal surgery. A standard procedure for single port laparoscopic adrenal surgery has not been established. We retrospectively investigated intraoperative and postoperative outcomes following laparoscopic adrenalectomy via mono-port (LAMP). METHODS: Between March 2009 and December 2009, 10 patients underwent LAMP at Kangbuk Samsung Hospital. The same surgeon performed all surgeries. The first 5 cases underwent LAMP using an Alexis Wound Retractor (Applied Medical, Rancho Santa Margarita, CA, USA) with surgical gloves, and others were done with an OCTO Port (Dalim Surgnet, Korea). RESULTS: Of the 10 patients, 5 were male and 5 were female. The mean age was 43.7±9.9 years (range, 34~62), and the mean BMI was 24.1±4.0 kg/m² (Range, 17.1 ~30.0). The mean tumor size was 32.5±16.9 mm (range, 12~60),mean operative time was 127.0±29.5 min (range, 90~180), and mean hospital stay was 4.5 days (range, 3~7). Three patients were diagnosed with non-functioning cortical adenoma, 3 with Cushing's syndrome, 2 patients with pheochromocytoma, and others with primarily hyperaldosteronism and myelolipoma. Major postoperative morbidity, blood transfusions, or conversion to open surgery did not occur. CONCLUSION: The outcome of LAMP demonstrates the safety and feasibility of this procedure. With increasing surgeon experience and refinement in instrument technology, we believe LAMP is likely to become a standard approach to adrenal disease.