Giant Bilateral Adrenal Myelolipomas in a Non-Compliant Patient with Congenital Adrenal Hyperplasia.

BACKGROUND 21-hydroxylase deficiency, an essential enzyme for glucocorticoid and mineralocorticoid synthesis, is the cause of congenital adrenal hyperplasia (CAH) in more than 95% of cases. It is an autosomal recessive disorder encoded by the CYP21A2 gene, categorized into classical forms, which encompass the salt-wasting (SW) and simple virilizing (SV) forms, as well as the nonclassical form (NC). The aim of medical treatment is to replace missing glucocorticoids and, if necessary, mineralocorticoids, while also reducing elevated adrenal androgens. CASE REPORT We present the case of a 42-year-old woman with CAH who discontinued therapy during adolescence and was admitted to hospital with fatigue, nausea, and severe abdominal pain. A CT scan showed an extreme enlargement of the adrenal glands. Laboratory tests revealed elevated levels of 17-hydroxyprogesterone and other adrenal androgens, along with normal plasma metanephrine levels. Decreased morning cortisol levels suggested partial adrenal insufficiency requiring glucocorticoid replacement therapy. Due to the development of several serious complications and clinical deterioration, the multidisciplinary team recommended bilateral removal of masses measuring 300×250×200 mm on the right side and 250×200×200 mm on the left side. Histological and immunochemical examination confirmed the presence of giant myelolipomas with adrenal cortex hyperplasia. CONCLUSIONS Adrenal tumors, particularly myelolipomas, have a higher prevalence in patients with CAH. Our case report provides further evidence of the suspected link between non-compliant CAH therapy and the development of myelolipomas, along with promotion of their pronounced growth.

Nontraumatic hemoabdomen and pancytopenia secondary to myelolipoma in a cat.

OBJECTIVE: (1) To report an unusual etiology for nontraumatic hemoabdomen in cats, and (2) to describe onset and recovery from severe, unexpected pancytopenia seen after surgical removal of a large intra-abdominal myelolipoma. CASE SUMMARY: A 14-year-old neutered male domestic shorthair cat was presented for emergent treatment of suspected nontraumatic hemoabdomen. A hyperechoic mass, with ultrasonographic echogenicity similar to fat, was found in the right cranial abdomen and believed to be associated with the mesentery. Cytological examination of abdominal fluid identified marked extramedullary hematopoiesis within the hemorrhagic effusion. Exploratory laparotomy identified a hepatic mass, which was resected, and revealed to be a hepatic myelolipoma on histopathological examination. The patient's initial recovery was uneventful. However, continued hyporexia resulted in readmission 4 days postoperatively, at which time the patient was found to have a profound, tri-lineage pancytopenia, and cytological evidence indicative of bone marrow recovery. The pancytopenia resolved with continued medical management and supportive care. NEW OR UNIQUE INFORMATION PROVIDED: Ruptured myelolipoma is not a commonly considered differential for nontraumatic hemoabdomen in cats. Furthermore, severe pancytopenia is unexpected following surgical resection of a myelolipoma. This case provides a unique clinical presentation of both nontraumatic hemoabdomen and bone marrow recovery.

MYELOLIPOMA COEXISTENCE WITH GLUCOCORTICOID AND ANDROGEN SECRETING ADRENOCORTICAL CARCINOMA: SLOW AND BENIGN CLINICAL COURSE.

We present a case of androgen and glucocorticoid secreting adrenocortical carcinoma with concomitant myelolipoma. A giant adrenal tumor which was initially nonfunctional was reassessed four years later due to the patient's refusal to treat. The patient was a 48-year-old woman with hypertension and acne lesions on the face. Laboratory findings were consistent with glucocorticoid and androgen hypersecretion. Computed tomography revealed a heterogeneously contrasting mass of 145x118x100 mm with lobular contour and soft tissue areas. The patient underwent left laparoscopic transperitoneal adrenalectomy with three port technique. There were no complications in the perioperative period. The resected specimen weighed 850 grams. Pathological findings showed a combination of myelolipoma-adrenal cortical cancer. In the postoperative period, hypertension improved and the hormone panel was normalized. Postoperative computed tomography and PET-CT showed no residual mass and metastasis. Although imaging is compatible with benign masses such as myelolipoma, coexistence of ACC-myelolipoma should be kept in mind and functional evaluation should be performed.

Giant secretory multifocal adrenal myelolipoma: a diagnostic dilemma.

A 38-yearr-old man presented with erectile dysfunction and infertility. On examination, he was hypertensive and detected to have a left flank mass. Blood investigations were unremarkable except raised serum noradrenaline levels. Imaging revealed multiple well-defined fat-containing hypodense lesions in left suprarenal area with largest one measuring 14×16 cm, suggestive of left adrenal myelolipoma. Diagnostic dilemma was posed due to discordance between clinical, biochemical and imaging findings. Left adrenal mass resection was planned keeping the possibility of pheochromocytoma. However, histopathology revealed it to be adrenal myelolipoma. Hypertension was resolved in the postoperative period and serum noradrenaline levels were normalised. Final diagnosis of a secretary adrenal myelolipoma was made, which is an extremely rare entity.

Non-secreting adrenal myelolipoma in a middle-aged male patient manifesting with sudden onset of severe lower back pain.

Adrenal myelolipoma (AML) is a rare benign tumor, usually non-functioning and asymptomatic until it reaches large size. AML is mostly detected incidentally by imaging and is composed of adipose tissue and hematopoietic elements. Only symptomatic tumor needs surgical excision. We report the case of a large non-functioning adrenal tumor discovered by means of combined imaging techniques in a middle-aged male patient who complained the sudden onset of severe lower back pain; successful laparoscopic removal was performed, and AML was diagnosed at histopathology.

Adrenal collision tumour comprised of adrenocortical carcinoma and myelolipoma in a patient with congenital adrenal hyperplasia.

Adrenal myelolipoma is a benign tumour characterized by the presence of macroscopic fat. Further workup is not necessary if a diagnosis of adrenal myelolipoma is obtained via imaging. We report the first case of adrenal collision tumour comprised of adrenocortical carcinoma and myelolipoma in a patient with bilateral myelolipomas and congenital adrenal hyperplasia. Computed tomography showed a large right adrenal mass consisting of two different components: soft tissue with peripheral heterogeneous enhancement and macroscopic fat. Imaging findings reflected features of both adrenocortical carcinoma and myelolipoma. Although this entity is rare, collision tumour containing an adrenocortical carcinoma component should be suspected if portions of an adrenal mass partially consist of peripheral heterogeneous enhancement.

Adrenal Incidentaloma: Challenges in Diagnosing Adrenal Myelolipoma.

Adrenal myelolipomas (AMLs) are rare benign adrenal tumors, containing adipose and hematopoietic tissue, a result of reticuloendothelial cell metaplasia. Incidence on autopsy has been reported from 0.08% to 0.4%. AMLs are generally considered nonsecretory. The functional aspect of adrenal incidentaloma should be evaluated. In this article, we report a case of a 40-year-old male, who presented with uncontrolled hypertension and renal failure, with imaging revealing an adrenal incidentaloma. He was started on dialysis for acute fluid overload, and workup for pheochromocytoma revealed an elevated serum norepinephrine level of 1181 pg/mL. Free metanephrine and normetanephrine levels were low when checked pre- and post-dialysis. Complete resection of the encapsulated right adrenal mass was performed. Pathology of the adrenal tumor demonstrates an 11.5 × 9.5 × 7.5 cm well-circumscribed, partially encapsulated proliferation of mature adipose tissue with admixed hemopoietic elements consistent with myelolipoma weighing 29.3 g. This case highlights the inclusion of a full metabolic workup for all adrenal incidentalomas, including AML.

Adrenal Myelolipoma with Adrenocortical Adenoma Presenting with Hypertension Only.

Here, we present a case of a 25-year Chinese female who was diagnosed with non-functional adrenocortical adenoma containing myelolipoma with hypertension as the only symptom. Serum levels of cortisol, aldosterone, angiotensin I/II and renin activity were normal. Myelolipoma is a benign, non-functioning retroperitoneal tumour occurring predominantly in the adrenal gland and relatively uncommon. With the advancement of radiological studies, the incidental detection of myelolipoma has been noted. However, the coexistence of adrenal myelolipoma and adrenal adenoma still remains extremely rare. Though usually benign, the later may present with endocrine dysfunction, such as Cushing's syndrome, and requires proper management. Surgical resection is reserved for symptomatic tumours or large myelolipoma (>7 cm in size). The final diagnosis mainly relies on pathological examination. The left adrenal mass was completely removed via retroperitoneal laparoscopic approach. Postoperative recovery was uneventful and her blood pressure (BP) readings were normal. At 15 months follow-up, the patient was normotensive and there was no recurrence of tumour.

Bilateral adrenal myelolipomas presenting as acute adrenal insufficiency in an adult with congenital adrenal hyperplasia.

Adrenal myelolipomas are relatively rare tumours composed of adipocytes and myeloid cells that arise in response to chronic adrenocorticotropic hormone stimulation. We present the case of bilateral adrenal myelolipomas in a 39-year-old man with untreated congenital adrenal hyperplasia (CAH) presenting with acute adrenal insufficiency and severe virilisation. Phenotypically, he is a man of short stature and has hyperpigmentation of the skin, gingiva and nail beds. Genital examination revealed micropenis and no palpable testes. Laboratory testing was consistent with primary adrenal insufficiency. An abdominal CT showed bilateral adrenal myelolipomas. An MRI of the pelvis revealed female reproductive organs. Chromosome study showed a karyotype of 46,XX. A CYP21A2 gene mutation confirmed diagnosis of CAH with 21-hydroxylase deficiency. The patient was treated with stress dose corticosteroids, subsequently tapered to physiological doses. We review previously reported cases and discussed diagnosis and treatment, including hormonal therapy and psychological approach.

Adrenal Myelolipomas Involved by Plasma Cell Myeloma.

OBJECTIVES: To report the presence and evaluate the frequency of plasma cell neoplasms within adrenal myelolipomas. METHODS: Adrenal myelolipomas within our institution were reviewed for the presence of hematologic neoplasia, and a review of the literature was performed. RESULTS: Two (9%) of 23 adrenal myelolipomas were involved by plasma cell myeloma. The patients were 71 and 81 years old, one woman and one man, with tumors measuring 7 cm and 8.5 cm, respectively. Both tumors contained large aggregates of dysplastic plasma cells occupying at least one ×10 field and demonstrated light chain restriction. Neither had an established diagnosis of plasma cell neoplasm previously. After receiving therapy, one patient exhibited a stable clinical course 1 year after diagnosis while the other died of disease 3 years later. CONCLUSIONS: We report the first two cases of adrenal myelolipoma involved by plasma cell myeloma, a rare and subtle finding that has significant clinical implications.

Delayed presentation of a virilising, pure testosterone-secreting adrenocortical carcinoma with coexistent composite myelolipoma and a venous thrombus extending to the heart.

A 40-year-old normotensive woman presented with abnormal facial hair for 4 years and amenorrhoea for 13 years. Hormonal, biochemical and haematological evaluation showed isolated elevation of serum testosterone and free testosterone. Her follicle-stimulating hormone and luteinising hormone were in the premenopausal range. Until recently she had reconciled to early 'menopause' and visited beauty clinics but never sought medical evaluation. Imaging revealed an enhancing left adrenal mass with fat densities and venous thrombus extending through the inferior vena cava to a 7 cm mass in the right atrium. She underwent left kidney-preserving surgery utilising hypothermic cardiopulmonary bypass with early clamping of the pulmonary artery without circulatory arrest. Histology showed adrenocortical carcinoma with composite incidental myelolipoma and neoplastic thrombus. At 2 months, testosterone has normalised and she is doing well. Isolated testosterone-secreting adrenocortical carcinoma with massive venous thrombus is rare as is coincidental composite macroscopic myelolipoma.

Case 243: Extramedullary Hematopoiesis in an Adrenal Myelolipoma.

History A 30-year-old man presented to the emergency department with epigastric pain. He was vomiting and in distress, and he had a history of thalassemia. Physical examination findings were unremarkable. Pertinent blood results were a hemoglobin level of 10.5 g/dL (6.52 mmol/L) (normal range, 13.5-18.0 g/dL [8.38-11.17 mmol/L]) and a bilirubin level of 62 µmol/L (normal range, 3-17 µmol/L). The remaining hematologic and biochemical results were normal. Aortic dissection was suspected clinically, so the patient was referred for imaging. Unenhanced and arterial phase computed tomographic (CT) images were acquired initially. Ultrasonography (US) (images not shown) and magnetic resonance (MR) imaging were performed subsequently. Because of the imaging findings, the patient was referred for surgery.

Anterior Transabdominal Laparoscopic Adrenalectomy, without Ligatures, for a Symptomatic Right Adrenal Myelolipoma with Intratumoral Hemorrhage.

Myelolipomas represent 3-7% fromthe primary tumors of the adrenal gland. Most often they are incidental findings. In most cases are asymptomatic, rarely they present symptoms (pain, abdominal discomfort, hematuria or signs of internal hemorrhage). Histologically benign, this variety of tumor requires only local excision, in symptomatic forms. Their dimensions are generally up to 4-5 cm, so the laparoscopic approach seems the most appropriate. We present the case of a 65 years old patient, electively operated for a right adrenal tumor formation.Alaparoscopic right adrenalectomy was performed using an anterior transabdominal approach. No ligatures, clips or sutures were used. The intervention was accomplished with the Ligasure Maryland forceps and the Force Triad platform (Covidien Medtronic-USA). The postoperative evolution was favorable and the pathological examination highlighted an adrenal myelolipoma with intratumoral hemorrhage.

Síndrome de Cushing causado por un tumor suprarrenal mixto de origen cortical asociado a un mielolipoma / Cushing’s syndrome caused by mixed adrenal tumor of cortical origin associated with myelolipoma

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Case report of a bilateral adrenal myelolipoma associated with Cushing disease.

RATIONALE: Adrenal myelolipomas are rare benign tumors, composed of a variable mixture of mature adipose tissue and hematopoietic tissue. These tumors are frequently detected incidentally and are usually asymptomatic, and hormonally inactive. PATIENT CONCERNS: During a routine health checkup, a 52-year-old man was found to have a tumor on the bilateral adrenal glands. Abdominal computed tomography revealed a well-defined, heterogeneously enhanced bilateral adrenal mass, suggesting a myelolipoma. DIAGNOSES: The hormonal evaluation revealed adrenocorticotropic hormone (ACTH) dependent Cushing syndrome. INTERVENTIONS: The patient underwent left adrenalectomy, and transsphenoidal resection of a pituitary mass. The final diagnosis was adrenal myelolipoma associated with Cushing disease. OUTCOMES: Growth of right adrenal myelolipoma was detected during the 7-year follow-up. There were enhancing pituitary lesions in repeat magnetic resonance imaging of the sellar region, which implies persistent or recurrent pituitary adenoma. This case reinforces relationship between Cushing disease and adrenal myelolipoma. LESSONS: To the best of our knowledge, this is the first reported pathologically confirmed bilateral adrenal myelolipoma associated with Cushing disease. This report supports the idea that ACTH is associated with the development of adrenal myelolipoma.

Bilateral adrenal myelolipoma in Cushing's disease: a relook into the role of corticotropin in adrenal tumourigenesis.

Adrenal myelolipomas are infrequently encountered benign tumours of unknown aetiology. In the majority of cases they are unilateral, and clinically and hormonally silent, only requiring periodic follow-up. However, bilateral adrenal myelolipomas are sometimes associated with endocrine disorders and warrant appropriate evaluation. Though the understanding of the pathophysiology of adrenal myelolipomas has long been elusive, adrenocorticotropic hormone (ACTH) has been proposed as the main tropic factor in a number of studies. Cushing's disease is rarely associated with bilateral and sometimes giant myelolipomas. In this article, the association of bilateral adrenal myelolipomas with Cushing's disease has been discussed and the role of ACTH in the tumourigenesis has been reviewed.