Adrenal myelolipomas.

Adrenal myelolipomas are benign, lipomatous tumours with elements of myeloid cells, most of which present as adrenal incidentalomas and comprise 3·3-6·5% of all adrenal masses. Adrenal myelolipomas are usually unilateral (in 95% of cases), variable in size, most often found during midlife, and affect both sexes almost equally. On imaging, adrenal myelolipomas show pathognomonic imaging features consistent with the presence of macroscopic fat. Large adrenal myelolipomas can cause symptoms of mass effect, and can occasionally be complicated by haemorrhage. In the event of a concomitant adrenal cortical adenoma or hyperplasia, adrenal hormone excess might be detected in patients with adrenal myelolipoma. Patients with congenital adrenal hyperplasia exhibit a higher prevalence of adrenal myelolipomas than other patient groups, and are at risk of developing large and bilateral lesions. This Review discusses the pathogenesis, clinical presentation, and management of adrenal myelolipomas.

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

Simultaneous adrenal and extra-adrenal myelolipoma - an uncommon incident: case report and review of the literature.

BACKGROUND: Extra-adrenal myelolipomas are rare benign tumours. Other soft tissue tumours such as well-differentiated liposarcomas appear morphological almost identical. Preoperative imaging and especially biopsy are important tools to diagnose these lesions. CASE PRESENTATION: We report a very seldom case of a simultaneous myelolipoma of the adrenal gland in association with an extra-adrenal myelolipoma in an 75-year-old man. With a review of the literature we describe and discuss the aetiology, differential diagnosis and treatment of patients with respect to adrenal and extra-adrenal lesions. CONCLUSION: The appearance of a simultaneous adrenal and extra-adrenal myelolipoma is a rare incident. We conclude that such lesions should be considered in the differential diagnosis of a fat-containing tumour in the retroperitoneal tissue/compartment.

[Extraadrenal perirenal myelolipoma: report of a case and review of the literature]. / Mielolipoma extraadrenal perirenal: aportación de un caso y revisión de la literatura.

Extra-adrenal myelolipomas are rare bening tumours that are componed of mature fat cells and myeloid elements. These lesions are usually asyntomatic and often found incidentally on radiographic studies. We report a case of extra-adrenal perirenal myelolipoma.

Bilateral adrenal myelolipoma associated with adrenogenital syndrome.

Adrenal myelolipoma is a rare benign tumor, occasionally reported in association with endocrine disorders. We report herein a case of bilateral adrenal myelolipoma associated with adrenogenital syndrome caused by 21-hydroxylase deficiency. A diagnosis of 21-hydroxylase deficiency was confirmed by mutation analysis of the CYP21 gene. Our case represents only the second case of bilateral adrenal myelolipoma associated with adrenogenital syndrome caused by 21-hydroxylase deficiency.

A rare case of a multifocal extra-adrenal myelolipoma with markedly hypocellular bone marrow.

Extra-adrenal myelolipomas are rare, generally solitary, non-functioning tumors consisting of adipose tissue and hematopoetic elements. These lesions are not known to be associated with underlying hematologic disorders or documented bone marrow abnormalities. We describe the extremely rare occurrence of multifocal, extra-adrenal myelolipomas in a 35-yr-old male. A markedly hypocellular bone marrow is noted, despite normal peripheral blood counts. To our knowledge, this is the first case report of a markedly abnormal marrow examination in a patient with extra-adrenal myelolipoma, and implicates the extra-osseous masses as the driving force behind his normal hematopoesis.

Mielolipoma retroperitoneal / Retroperitoneal myelolipomas

El retroperitoneo es el espacio que se encuentra delimitado: por detrás, por la columna lumbar y los músculos psoas ilíaco y el cuadrado lumbar; por delante, por el peritoneo parietal posterior; por arriba, el diafragma; por debajo, el piso pelviano. Los tumores retroperitoneales representan el 1,8 por ciento del total de los tumores abdominales en nuestro servicio. Los mielolipomas comprenden el 0,08-0,4 por ciento de los mismos, siendo ellos tumores benignos compuestos por tejido adiposo y hematopoyético (incluye colonias eritroides, mieloides y megacariocíticas. El objetivo de este trabajo es definir alternativas terapéuticas en tumor benigno retroperitoneal

Presentation and therapy of myelolipoma.

BACKGROUND: Adrenal myelolipoma is a rare and benign, hormonally inactive tumor frequently discovered incidentally. Because of the increasing rate of detection of adrenal myelolipoma, use of the correct diagnostic examination and treatment, with respect to surgical excision or regular controls, is continually gaining importance. We report herein on the largest series of surgically treated patients with adrenal myelolipoma from a single institute. METHODS: The clinical charts of 12 patients who underwent adrenal surgery for myelolipoma were reviewed. Follow up was carried out by means of re-examination or by personal contact with the primary physician. It was possible to perform a re-examination on eight of the 12 patients 6.9 years after the operation: one patient had died 5.2 years after surgery. RESULTS: Diagnosis of adrenal myelolipoma was made incidentally in seven patients, and during examination for reasons of unspecific abdominal or flank pain in five patients. A transabdominal approach was taken in five patients, a subcostal approach was taken in three patients and a translumbal approach was taken in four patients. At the follow up, all formerly symptomatic patients were free of symptoms. No recurrence could be seen; however, in one patient a contralateral adrenal myelolipoma had developed. CONCLUSIONS: Symptomatic tumors, growing tumors or tumors larger than 10 cm should be excised surgically by means of an endoscopic or conventional approach, depending on the size. Surgical indication should not be liberalized by the introduction of more gentle operative techniques such as the translumbal or the endoscopic approach. A close follow up should be maintained in the case of patients free of symptoms, and for those with a definite diagnosis from imaging procedures.

[Isolated leiomyomatosis of the right renal vein associated with hepatic myelolipoma: diagnostic and therapeutic problems]. / Léiomyomatose isolée d'une veine rénale droite associée à un myélolipome hépatique: problèmes diagnostiques et thérapeutiques.

The authors report a case of isolated intravenous leiomyomatosis involving a vein of the right renal hilum associated with operated hepatic myelolipoma with a diagnosis of metastatic renal cancer. These tumours are very rare and are usually diagnosed on histological examination of the resection specimen. Although they have a benign histology, they have a very different prognosis and therapeutic management. Although the association of these two exceptional tumours may appear to be purely incidental, a common aetiological factor may nevertheless exist.

[Adrenal gland myelolipoma. Report of a new case]. / Mielolipoma suprarrenal. Aportación de un nuevo caso.

OBJECTIVE: We report a new case of symptomatic myelolipoma of the adrenal gland. We analyse clinical, diagnostic, histopathologic and therapeutic aspects. METHOD AND RESULTS: Surgical excision was performed by lumbotomy. The patient was asymptomatic after surgery. DISCUSSION: Adrenal myelolipoma is norfunctioning benign tumour constituted by fat tissue and small islands of haemathopoyetic in a variable rate. The patients are usually asymptomatic. In symptomatic patients the most common symptoms are nonspecific abdominal pain, haematuria and hypertension. The diagnosis is achieved by ultrasound, CT and PAFF. The management is controversial: Surgical treatment or watching are options depending on the size and/or symptoms.

Mielolipoma suprarrenal: a propósito de un caso: revisión bibliográfica / Suprarrenal mielolipoma: with regard to a case: bibliographical overhaul

Presenta un caso de Mielolipoma suprarrenal, tumor raramente descrito, endocrinológicamente benigno. Analizamos la presentación Clínica y Quirúrgica. Como revisión de la literatura.

Symptomatic adrenal myelolipoma: therapeutic considerations.

Four cases of symptomatic myelolipoma of the adrenal gland are presented. Three were treated at laparotomy and one with a laparoscopic approach. On the basis of these four cases we discuss the aetiology, pathogenesis, clinical manifestations, diagnosis and treatment of this rare disease. We also report the follow-up of a patient with bilateral myelolipoma, who underwent monolateral excision. We conclude that myelolipoma, if correctly diagnosed, can be treated conservatively with careful follow-up, limiting surgery to symptomatic cases.