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 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

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 adrenal bilateral ligado a hipotiroidismo primario / Bilateral adrenal myelolipoma linked to primary hypothyroidism

Los mielolipomas adrenales son tumores benignos, no funcionantes e infrecuentes, generalmente de diagnóstico incidental, que eventualmente provocan síntomas locales. Tras una revisión de la literatura previa publicada, se realizó la descripción de un caso clínico de mielolipoma adrenal bilateral, que coexiste con hipotiroidismo primario, relación no reportada hasta el presente. Dichas masas tumorales se diagnosticaron en una mujer de 66 años, aquejada de dolor abdominal recurrente, que además padecía hipotiroidismo e hipercolesterolemia, a la que se le practicó con éxito la exéresis de la lesión de mayor tamaño. Se propuso un tratamiento diagnóstico-terapéutico para este tipo de tumores(AU)

The adrenal myelolipomas are benign tumors, non-functioning and infrequent, generally of incidental diagnosis which by chance provoke local symptoms. After a review of published previous literature it was possible to describe of a clinical case of bilateral adrenal myelolipoma coexisting with a primary hypothyroidism with a relation not reported until now. Such tumoral masses were diagnosed in a woman aged 66 with recurrent abdominal pain and hypothyroidism and hypercholesterolemia who undergoes successfully a exeresis of the bigger lesion. A diagnostic-therapeutic treatment was proposed for this type of tumors(AU)

Mielolipoma adrenal bilateral asociado a disfunción endocrina / Bilateral adrenal myelolipoma associated with an endocrine dysfunction

Se presentó un caso de mielolipoma adrenal bilateral asociado a hiperplasia adrenal congénita por déficit de enzima 21 hidroxilasa en un joven de 27 años de edad, que abandonó tratamiento sustitutivo con acetato de cortisona a los 14 años. Estuvo asintomático hasta su ingreso en el hospital, al cual es remitido por dolor abdominal, vómitos y fiebre, constatándose tumoración abdominal gigante en hemiabdomen izquierdo. Las características clínicas, hormonales y radiológicas halladas son comentadas y correlacionadas con lo registrado en la literatura médica. Hasta donde se revisó, es el primer caso de mielolipoma adrenal bilateral asociado a disfunción endocrina que se publica en Cuba(AU)

Authors present a case of bilateral adrenal myelolipoma associated with a congenital adrenal hyperplasia by deficit of hydroxilase enzyme 21 in a young aged 27 who leaves the substitution treatment with cortisone acetate at 14 years old. He was asymptomatic until its hospital admission due to abdominal pain, vomiting and fever and a high abdominal tumor in left hemi-abdomen. Clinical hormonal and radiological features founded are discussed and correlated with those registered in medical literature. As far as we know, this is the first case of bilateral adrenal myelolipoma associated with an endocrine dysfunction published in Cuba(AU)

Giant adrenal myelolipoma associated with 21-hydroxylase deficiency: unusual association mimicking an androgen-secreting adrenocortical carcinoma / Mielolipoma adrenal gigante associado à deficiência da 21-hidroxilase: associação não usual simulando um carcinoma adrenocortical secretor de androgênios

The objective of this study was to describe a case of giant myelolipoma associated with undiagnosed congenital adrenal hyperplasia (CAH) due to 21-hydroxylase (21OH) deficiency. Five seven year-old male patient referred with abdominal ultrasound revealing a left adrenal mass. Biochemical investigation revealed hyperandrogenism and imaging exams characterized a large heterogeneous left adrenal mass with interweaving free fat tissue, compatible with the diagnosis of myelolipoma, and a 1.5 cm nodule in the right adrenal gland. Biochemical correlation has brought concerns about differential diagnosis with adrenocortical carcinoma, and surgical excision of the left adrenal mass was indicated. Anatomopathologic findings revealed a myelolipoma and multinodular hyperplasic adrenocortex. Further investigation resulted in the diagnosis of CAH due to 21OH deficiency. Concluded that CAH has been shown to be associated with adrenocortical tumors. Although rare, myelolipoma associated with CAH should be included in the differential diagnosis of adrenal gland masses. Moreover, CAH should always be ruled out in incidentally detected adrenal masses to avoid unnecessary surgical procedures.

O objetivo deste trabalho foi descrever um caso de mielolipoma gigante associado à hiperplasia adrenal congênita (HAC) por deficiência da 21-hidroxilase (21OH). Paciente do sexo masculino, 57 anos de idade, encaminhado por achado ultrassonográfico de massa adrenal esquerda. Investigação bioquímica revelou hiperandrogenismo e exames de imagem revelaram grande lesão sólida em adrenal esquerda de aspecto heterogêneo, entremeada de tecido gorduroso, compatível com diagnóstico de mielolipoma, e um nódulo de 1,5 cm na adrenal direita. Os achados bioquímicos sugeriam o diagnóstico de carcinoma adrenocortical, indicando cirurgia para retirada da massa adrenal esquerda. O anatomopatológico confirmou mielolipoma e hiperplasia multinodular do córtex adrenal. A investigação subsequente diagnosticou HAC por deficiência da 21OH. Concluiu-se que a HAC tem sido descrita em associação com tumores adrenocorticais. Apesar de raro, o mielolipoma associado à HAC deve ser incluído nas possibilidades diagnósticas de massa adrenal. Adicionalmente, a HAC deve ser sempre afastada nos casos de massa adrenal de achado incidental, evitando cirurgias desnecessárias.

Mielolipoma suprarrenal gigante: reporte de un caso y revisión de la literatura / Giant Adrenal myelolipoma: a case report and literature review

Presentamos un caso de mielolipoma suprarrenal gigante, para ilustrar la expresión y el manejo de esta rara patología. Este tumor benigno poco frecuente, compuesto de tejido hematopoyético maduro y tejido adiposo en proporciones variables, generalmente se diagnostica de forma incidental y sólo tiene indicación quirúrgica en aquellos casos en los que producen síntomas o alcanza grandes dimensiones. Describimos el caso de una paciente de 56 años, asintomática, con el hallazgo incidental de tumor suprarrenal de grandes dimensiones, tratado mediante resección quirúrgica en el servicio de Cirugía III del Hospital Universitario de Caracas

Case report of giant adrenal myelolipoma to describe the presentation and the treatment for this rare entity. This uncommon benign tumor, composed of variables proportions of mature hemopoyetic tissue and fat, is diagnosed incidentally and has indication of surgery only in the few cases in which it turns symptomatic or it reach big dimensions. We report the case of a 56-years-old asymptomatic female, in who is incidentally detected a giant adrenal tumor, surgically resected at the Hospital Universitario de Caracas, Servicio Cirugia III

Mielolipoma gigante bilateral da glândula adrenal / Bilateral giant adrenal myelolipoma

Mielolipomas são tumores benignos relativamente raros, freqüentemente associados à glândula adrenal. Geralmente são não-funcionantes, assintomáticos e medem menos de 5 cm. Mielolipomas adrenais bilaterais e gigantes são raros. Relatamos o primeiro caso de mielolipoma gigante bilateral da glândula adrenal da literatura latino-americana: paciente do sexo feminino, 57 anos, com queixa de dor no flanco esquerdo. A tomografia computadorizada do abdome mostrou massas bilaterais da glândula adrenal, medindo a direita 9 x 7 x 6,5 cm e a esquerda 23 x 19 x 8 cm. As massas eram radiologicamente consistentes com tecido adiposo. O diagnóstico pré-operatório foi de lipossarcoma. A paciente foi submetida à laparotomia com retirada das massas. O exame microscópico diagnosticou mielolipomas bilaterais. Embora sejam tumores benignos, seu tratamento deve ser cirúrgico, pois somente o exame anatomopatológico pode descartar uma neoplasia maligna.

Myelolipomas are relatively rare benign tumors, frequently associated with the adrenal glands. Usually, they are non-functioning, asymptomatic and measure less than 5 cm. Bilateral giant adrenal myelolipomas are rare. We report the first case of bilateral giant adrenal myelolipomas of the Latin American literature: a 57-year old woman who presented with left abdominal pain. The computed tomography scanning revealed bilateral suprarenal masses measuring 9 x 7 x 6.5 cm on the right and 23 x 19 x 8 cm on the left. The masses were radiographically consistent with fatty tissue. The preoperative diagnosis was liposarcoma. Surgery was performed with excision of the masses. The histologic analysis identified bilateral adrenal myelolipomas. Although they are benign tumors, surgery must be performed because only the histologic analysis can exclude malignancy.

Adrenal myelolipoma--report of a case.

Adrenal myelolipoma is a rare, benign, non-secreting tumour composed of adipose and haematopoietic tissue. One such rare case in a 32-year-old female who presented with'pain in right lumbar region is reported here. CT scan revealed a mass in the suparenal region measuring 5.6 x 5 cm. In view of the size and symptomatic nature of the mass, surgical resection was performed. Histological examination revealed the features of myelolipoma. The outcome was favourable without recurrence after a follow-up of one year.

Mielolipoma adrenal e hiperaldosteronismo primário / Adrenal myelolipoma and primary hyperaldosteronism

Objetivo: Mielolipomas são tumores raros e benignos do córtex adrenal de etiologia desconhecida. São compostos de células gordurosas maduras e tecido hematopoiético em vários graus de diferenciação. A maioria desses tumores são nódulos pequenos e assintomáticos. Assim, o diagnóstico desses tumores no passado era geralmente realizado por ocasião da realização de autópsia. Entretanto, devido aos atuais métodos de imagem, tem-se aumentado a descoberta ocasional desses tumores em pacientes portadores de outras patologias. Os mielolipomas são quase sempre não-funcionantes, mas há relatos desses tumores associados a distúrbios endócrinos.Relato de caso: Paciente JHN, 60 anos, masculino,portador de diabetes mellitus controlado com insulina,hipertensão arterial e hipercolesterolemia, evoluindocom insuficiência renal crônica abordada por tratamentodialítico, seguida por transplante renal. O paciente apresentou quadro de hipopotassemia sintomática na vigência de função renal limítrofe, tendo sido aventada a hipótese diagnóstica de hiperaldosteronismo primário. Essa hipótese foi confirmada pelas dosagens de renina e aldosterona plasmáticas, além do achado de massa adrenal esquerda através de tomografia computadorizada e de ressonância nuclear magnética. Foi realizada adrenalectomia esquerda videolaparoscópica, sendo que o paciente evoluiu com nomalização da hipopotassemia e da hiperglicemia, incluindo a suspensão da insulinoterapia. O estudo anatomopatológico foi compatível com o diagnóstico de mielolipoma, sem achados sugestivos de aldosteronoma. Conclusão: É bastante evidente neste relato de caso a relação do mielolipoma adrenal com o hiperaldosteronismo primário, reforçando a hipótese de queeste tipo de tumor possa associar-se às endocrinopatiase a outras condições clínicas potencialmente reversíveisou controláveis pela ressecção do mielolipoma, como foi o caso desse relato

Diagnosis of adrenal myelolipoma by imaging and guided biopsy.

Adrenal myelolipoma is a rare, benign, endocrinologically inactive, and asymptomatic tumour which is usually found incidentally at imaging or autopsy. Symptomatic tumours are very rare. We describe a case of adrenal myelolipoma where the diagnosis was made on the basis of radiological features and image-guided fine needle biopsy, without resorting to an operation.

Resección laparoscópica de un mielolipoma de la glándula suprarrenal. Presentación de un caso y revisión de la literatura / Laparoscopic resection of an adrenal myelolipoma. A case report and literature review

Los mielolipomas de la glándula suprarrenal son tumores benignos raros, no funcionantes, caracterizados por la presencia de tejido adiposo, y elementos de la médula ósea en varios grados de diferenciación. La mayoría son asintomáticos y descubiertos incidentalmente. Presentamos el caso de un mielolipoma de glándula suprarrenal derecha localizado incidentalmente en el ultrasonido y en la tomografía computada a un paciente de 39 años de edad, que se encontraba bajo estudios para hipertensión arterial. Se sometió a resección laparoscópica de la glándula exitosamente, el estudio histopatológico reportó un tumor de glándula suprarrenal derecha de 6 cm de diámetro correspondiente con un mielolipoma, la evolución del paciente fue satisfactoria