Search

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.

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., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

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.

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 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., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

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.

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.

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., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

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., (Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.)

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.

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., (Celsius.)

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., (2016 BMJ Publishing Group Ltd.)

Adrenal myelolipomas are rare non functional benign tumours which are incidentally diagnosed in asymptomatic patients. Despite the fact that several theories have been discussed, the origin of myelolipoma remains unclear. Our patient, 19 years old male presented with history of abdominal pain for past 7 years. Computed tomography scan of abdomen with oral and intravenous contrast revealed well defined round heterogenous enhancing mass seen in right supra renal area involving the adrenal gland. Patient underwent surgery by open access through thoracoabdominal approach. Histopathology of resected mass showed myelolipoma along with trilineage haematopoesis with mature adipose tissues.

Context: Male patients with congenital adrenal hyperplasia (CAH) may develop bilateral testicular adrenal rest tumours (TARTs). These tumours, in most cases, regress with glucocorticoid therapy and their histological differentiation from Leydig-cell tumors is quite difficult., Objective: The aim of this study was to differentiate the histological and clinical features of the TARTs from those of the Leydig-cell tumours., Methods: The authors report a case of bilateral Leydig-cell tumour associated with giant bilateral adrenal myelolipoma in a male with adrenogenital syndrome who was submitted to a bilateral orchiectomy., Results: Testicular neoplasia continue to grow despite adequate hormonal treatment and a bilateral orchiectomy was performed. The histopathological examination of the specimen ultimately confirmed the diagnosis of bilateral Leydig-cell tumour., Conclusions: This case shows the importance of all the relevant examinations, blood chemistry analysis, and instrumental tests in the differential diagnosis of TARTs and other testicular tumours.

The paper describes a case of von Hippel--Lindau-related pancreatic neuroendocrine tumor and adrenal myelolipoma in a 44-year-old woman. The pancreatic tumor and a left retroperitoneal mass were removed in the women in July 2014 and May 2015. Histological examination of the pancreatic tumor revealed that the latter consisted of clear cells forming tubular and tubercular structures showing the expression of chromogranin A, synaptophysin, and cytokeratins 18 and 19 and a negative response to CD10 and RCC. The adrenal medullary mass presented as clear-cell alveolar structures with inclusions of adipose tissue mixed with erythroid, myeloid, and lymphoid cells. The clear-cell component of the adrenal gland expressed neuroendocrine markers with a negative response to cytokeratins, CD10, and RCC. Molecular genetic examination yielded a signal corresponding to two copies of the VHL gene. No deletions or amplifications of the gene were detected. Cases of von Hippel--Lindau disease concurrent with adrenal pheochromocytoma and myelolipoma and simultaneous pancreatic involvement were not found in the literature.

Introduction: Myelolipomas and extramedullary hematopoietic tumors are uncommon benign tumors. They are variably composed of mature adipose tissue and hematopoietic tissue. Myelolipoma is usually observed in the adrenal gland and extramedullary hematopoietic tumors in the liver and spleen but may occasionally be found within solid tumors., Case Report: A 62-year-old man without previous haematological history presented with a voluminous solitary bilateral renal tumor. Contrast-enhanced ultrasound CT-scan and scintigraphy with technetium-99m-nanocolloid and indium-111-chloride bone marrow were highly suggestive of extramedullary hematopoietic tumor. CT-guided biopsy suggested a diagnosis of myelolipoma. An atypical hereditary spherocytosis, undiagnosed until now, was demonstrated., Conclusion: We report, for the first time to our knowledge, a border form between extramedullary hematopoiesis tumor and myelolipoma of renal localisation revealing a hereditary spherocytosis in an adult patient., (Copyright © 2015 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.)

Myelolipomas are uncommon benign tumors composed of mature adipose tissue mixed with hematopoietic elements; these tumors can occur in both the adrenal glands and extra-adrenal locations, the presacral region being the most frequent extra-adrenal site. We present a case of presacral myelolipoma diagnosed by fine needle aspiration (FNA) and core needle biopsy (CNB) in a 55-year-old woman with concurrent invasive ductal breast cancer. TC and RM imaging were consistent with the diagnosis of presacral myelolipoma. The lesion was discovered incidentally during the staging procedure for breast malignancy. The purpose of our work is to describe the FNA and CNB finding in combination with the imaging features of this uncommon lesion., (Copyright © 2014 Elsevier GmbH. All rights reserved.)

Sickle cell anemia is an inherited abnormality of the globin chain with very high prevalence in the Indian subcontinent. A significant proportion of these patients present late in life and are at a risk of complications like acute chest syndrome and painful episodes till a definitive diagnosis is reached and appropriate treatment is started . We report a novel triad of abdominal imaging findings which is not reported in literature until now and which may suggest a diagnosis of sickle cell anemia in retrospect. Patients with this triad of abdominal findings should be suspected to have an underlying hemoglobinopathy and should be referred for further hematological workup. Although in our case the patient was diagnosed to have sickle cell anemia depending on the abnormal morphology of red cells and hemoglobin electrophoresis, it should be remembered that this triad of findings may be seen in other hemoglobinopathies which induce a state of chronic anemia.

This report presents a case of 69-year-old woman, who was operated due to renal tumor. Apart from renal neoplasm, the adjacent adrenal gland contained another one tumor in medulla of the organ. The renal lesion was diagnosed renal cell carcinoma, clear cell type with undifferentiated cell sarcomatoid component. The adrenal neoplasm was composed of wavy S100-positive, Schwann-like cells and dispersed chromogranin A-reactive ganglion cells to be consistent with mature ganglioneuroma. It was accompanied by coexistent myelolipoma that contained hematopoietic cells including clearly visible megakaryocytes and foci of fat. To our knowledge, our paper is the first to report sporadic clear cell renal cell carcinoma with sarcomatoid cell type component and mature adrenal ganglioneuroma with myelolipoma in the same patient.

Myelolipomas are rare, benign, non-functioning tumors composed of an admixture of mature adipose tissue and hematopoietic elements. Extra-adrenal myelolipomas are extremely rare, but have been reported in multiple sites including the omentum, presacral, and retroperitoneal areas, along with the thorax, kidneys, liver and stomach. We report a case of a 68-year-old man with low-grade B-cell lymphoma arising in a background of recurrent multifocal extra-adrenal myelolipoma. Pathological evaluation of the lesion and bone marrow showed foci of lymphoid aggregate that were confirmed to be monoclonal B lymphoma by flow cytometry. To our knowledge, this is only the third reported case to feature such a rare combination of diseases. The clinical, radiological, and pathological differential diagnostic findings are discussed.

Adrenal myelolipomas are rare, usually benign unilateral tumors. Their management has controversially been discussed. The authors here present a 53-year-old African American female Jehovah's witness with postmenopausal uterine bleeding on chronic anticoagulation and episodic right flank pain who was found to have bilateral myelolipomas in addition to primary hyperparathyroidism. In collaboration with gynecology, midline laparotomy was performed to remove uterus and both ovaries in addition to the right adrenal gland for a 62 × 79 mm myelolipoma. An open biopsy of the left adrenal mass measuring 42 × 43 mm revealed myelolipoma and ruled out malignancy. Pathology confirmed bilateral myelolipomas, endometrial polyps, and leiomyomata uteri. After an uneventful recovery, the patient then underwent a right inferior parathyroidectomy for parathyroid adenoma. This case illustrates the challenges in deciding when to surgically intervene for bilateral adrenal myelolipoma and, for the first time, associates various other endocrinopathies, although no known endocrine neoplasia syndrome variant was present.

Myelolipoma is a benign tumour consisting of mature fat with scattered foci of haematopoietic elements resembling bone marrow. Extra-adrenal myelolipomas are an infrequent pattern of presentation. We report the case of a 64-year-old woman who presented a heterogeneous 1.4×2.5×3 cm paravertebral thoracic mass detected by chest tomography during the study of a leukocytosis of unknown aetiology. The CT findings and pathology revealed the diagnosis of myelolipoma.

Posterior retroperitoneoscopic approach (PRA) has become an established procedure for retroperitoneal lesions. The authors encountered a 30-year-old male patient who had β-thalassemia major and received splenectomy 15 years previously. Bilateral suprarenal tumors were noted on follow-up sonography. Subsequent computed tomography of the abdomen showed a right adrenal tumor and a left intraperitoneal lesion with homogenous enhancement. Successful bilateral PRA was performed, and histology confirmed the diagnosis of right adrenal myelolipoma and an accessory spleen. For the present case who had a large abdominal scar, PRA is technically feasible for treating patients with intraperitoneal and/or retroperitoneal suprarenal lesions while eliminating the necessity of dissection for intra-abdominal adhesions and patient repositioning for bilateral lesions.

Adrenal myelolipoma associated with hyperandrogenemia is extremely rare. We present such a case here. The patient was a 34-year-old Chinese woman with a history of growth retardation, virilization and lumbago. Adrenal imaging showed a circular soft tissue mass in the right adrenal gland. Endocrine examinations showed increased levels of plasma testosterone and dehydroepiandrosterone sulfate, significantly lower levels of follicle-stimulating hormone and luteinizing hormone, with other biochemical parameters being basically normal. After a histopathological study confirming the mass to be an adrenal myelolipoma, the patient was managed with a right adrenalectomy and then recovered. A search of the English and Japanese language literature suggests this is the first detailed and documented case of adrenal myelolipoma associated with hyperandrogenemia., (© 2012 The Japanese Urological Association.)

This report describes hepatic multiple myelolipoma with severe coelomic edema in a 14-year-old, male red-bellied tamarin (Saguinus labiatus). Multiple small and large nodules were formed in all lobes of the liver. Histopathologically, the nodules comprised mature and normal adipocytes and hematopoietic elements at various ratios that were composed of granulocytic, erythrocytic, and megakaryocytic series in various phases of maturation. All nodules were encapsulated and demarcated hepatocytes around masses. Myelolipoma in the liver is rare, and there are no reports of any cases to date. To our knowledge, this is the first report of hepatic multiple myelolipoma in a red-bellied tamarin.

Adrenal myelolipoma is a benign neoplasm composed of an admixture of hemopoietic elements and mature adipose tissue. The incidence of adrenal myelolipoma is reported as between 4% and 5% of adrenal incidentaloma. The association of an adrenal myelolipoma and adrenal nodular hyperplasia or adrenal adenoma is rare. Four cases of adrenal myelolipomas in the material of 702 incidentally discovered adrenal lesions treated in our center are presented in this paper (in a group of 294 operated patients). Two myelolipomas have been reported as isolated adrenal masses and two - in association with adrenocortical nodular hyperplasia.

Background: Increased frequencies of adrenal tumours and testicular adrenal rest tumours (TART) have been reported in patients with 21-hydroxylase deficiency (21OHD)., Objective: Patients, methods and design From a cross-sectional population-based study of 101 adult Norwegian patients with 21OHD, sixty-two participated in this study (23 men, 39 women; age range 18-75); thirty-two were salt wasting (SW) and 30 simple virilizing (SV); they were assessed with adrenal computed tomography (CT), testicular ultrasound and hormone measurement in the morning after overnight medication fast., Results: Nine adrenal tumours were detected in seven (11%) patients (bilateral in 2); four were myelolipomas and one a phaeochromocytoma. Seventeen (27%) had normal adrenal size, whereas 36 (58%) had persisting hyperplasia, and seven (11%) adrenal hypoplasia. Abnormal adrenals were more common in SW than in SV. TART occurred exclusively in SW and was present in seven (57%) of these men. Testicular volumes were small compared with normative data. Morning ACTH and 17-hydroxyprogesterone levels correlated positively with adrenal dimensions and frequency of TART., Conclusion: In this unselected population of patients with classical 21OHD, we found high frequencies of adrenal tumours, particularly myelolipomas, and of hyperplasia and hypoplasia, and TART in SW. It is important that physicians are aware that benign adrenal and testicular tumours occur frequently in 21OHD. Furthermore, these findings may reflect inappropriate glucocorticoid therapy, making a case for the advancement of novel physiological treatment modalities., (© 2011 Blackwell Publishing Ltd.)

Objective: To review the association between congenital adrenal hyperplasia (CAH) and adrenal myelolipomas and report a case of bilateral, giant adrenal myelolipomas in a patient with untreated CAH due to 21-hydroxylase deficiency., Methods: We describe the patient's clinical presentation, imaging findings, and laboratory test results and review the relevant English-language literature concerning patients with both CAH and myelolipomas., Results: A 45-year-old man with untreated CAH due to 21-hydroxylase deficiency presented with increasing abdominal girth and abdominal pain. Computed tomography of the abdomen demonstrated very low-density adrenal masses (22 × 11 cm on the left side and 6 × 5.5-cm on the right side) consistent with adrenal myelolipomas. The left adrenal myelolipoma was resected (24.4 × 19.0 × 9.5 cm; 2557 g). The mass was composed of mature adipose tissue with areas of hematopoietic cells of myeloid, erythroid, and megakaryocytic cell lines. Islands of adrenal cortical cells were scattered between the adipose and hematopoietic tissue. Including the present case, we identified 31 patients with both CAH and myelolipomas who have been described in the English-language literature. The details of these cases were reviewed., Conclusions: Persons with CAH may be at increased risk of developing adrenal myelolipomas, particularly if their CAH is poorly controlled. How and whether chronic exposure of the adrenal glands to high corticotropin levels increases the risk of developing myelolipomas remains a matter of speculation.

Adrenal myelolipoma is a rare, benign and usually hormonally inactive tumour. We report a case of a young female who presented with hormonally active tumour causing virilization and associated type-2 Diabetes mellitus. Laparoscopic adrenalectomy was done after CT finding of a large left adrenal mass that was producing large amounts of androgens. Adrenal myelolipoma with cortical hyperplasia was diagnosed on histopathological examination. Her diabetes progressively regressed after the removal of tumour and glucose tolerance remained normal up to 1 year of follow-up after surgery and there was no recurrence of tumour. She also had a lump in her breast which proved to be a fibroadenoma. We report this case due to its rarity, multiplicity of tumours and adrenal cortical hyperplasia-presenting as an unusual cause of severe virilization.

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.

Hepatic myelolipoma incarcerated in a peritoneopericardial diaphragmatic hernia was diagnosed in an 11-year-old, desexed female Persian cat. The cat was initially referred for investigation of tachypnoea and dyspnoea. Peritoneopericardial diaphragmatic hernia is a common incidental finding in cats and is usually asymptomatic. Myelolipoma is an extremely rare benign tumour, composed of extramedullary haematopoietic cells and adipose tissue. Myelolipomas are hypothesised to result from metaplastic alteration, rather than a neoplastic process, although this theory cannot be substantiated. The present case is only the fourth report of such an unusual occurrence in cats and displays significant differences to previous reports. Hepatic entrapment and burgeoning of the mass within the pericardial sac resulted in cardiac tamponade and overt signs of right-sided cardiac failure. Surgical intervention was successful and despite concerns regarding the cat's clinical presentation and the gross appearance of the lesion(s), a good long-term outcome is anticipated.

We report here a case of hepatic myelolipoma with systemic amyloidosis in a goose (Anser cygnoides domesticus), which died suddenly following the short history of weakness and greenish diarrhea. At necropsy, multiple yellowish-white foci were observed on the surface of the prominently enlarged liver. Histologically, there were multiple foci of adipose tissue admixed with myeloid elements in various proportions in the liver as well as amyloid deposition in several organs including the liver, intestine, spleen, kidney, and ovary. Ultrastructurally, erythroblast-like cells and myelocytes, which showed various stages of differentiation, were observed in the foci of the liver. These findings shared characteristics of hepatic myelolipoma which is very rare in birds.