A giant adrenal myelolipoma in a beta‐thalassemia major patient: Does ineffective erythropoiesis play a role?

The average age for its diagnosis was 51 years, and no gender difference was observed. The average size of tumors was 10.2 cm. Congenital adrenal hyperplasia was associated to 10% of all cases analyzed, while other adrenal hypersecretory disorders (cortisol, aldosterone) were found in 7.5% of cases. Computed tomography and magnetic resonance imaging can be reliably used for its differential diagnosis. If the diagnosis of an adrenal myelolipoma is unambiguous, and no associated symptoms or hormonal activity are established, surgical intervention is usually not necessary.

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“…Another hormonal overstimulation theory suggests stimulation via higher level of erythropoietin caused by chronic anemia [20,28].…”

Section: Pathogenesismentioning

confidence: 99%

Adrenal myelolipoma: a comprehensive review

et al. 2017

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“…In these conditions, endogenous ACTH could be elevated. It is of interest that some adrenal myelolipomas were associated with haematological diseases in which haemolytic anaemia or ineffective erythropoiesis is present (Au et al 2000, Motta et al 2016. These haematological conditions could contribute to the presence of extramedullary haematopoiesis that was postulated to trigger the myeloid component in adrenal myelolipoma.…”

Section: Myelolipomamentioning

confidence: 99%

Lipomatous tumours in adrenal gland: WHO updates and clinical implications

Lam¹

2017

Endocrine-Related Cancer

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Adrenal lipomatous tumour is a group of adrenal tumours with a significant component of adipose tissue. According to the current World Health Organization (WHO) classification of tumours of endocrine organs, adrenal myelolipoma is the only entity amongst the group of tumours being described. In the literature, other more recently documented adrenal lipomatous tumours included 24 lipomas, 32 teratomas and 16 angiomyolipomas. Rare fatty tumours of the adrenal gland comprised liposarcoma, hibernoma, adrenocortical tumours with fat component and rare adrenal tumours with fat component. Myelolipoma comprises approximately 3% of primary adrenal tumour. It is noted more commonly in females and in the right adrenal gland. Approximately 40 bilateral myelolipomas were reported. The tumour is most frequently recorded in patients between fifth and seventh decades of life. Adrenal lipomas are often seen in males and in the right adrenal gland. They were commonly noted in patients in the sixth decade of life. The diagnosis could only be possible on examination of the surgically removed specimen. Adrenal teratomas were more common in females and with a bimodal age distribution. Slightly over 60% of the patients with adrenal teratoma are symptomatic. Adrenal angiomyolipomas were often symptomatic, more common in females and in the fifth decades of life. To conclude, adrenal lipomatous tumour is uncommon. They are often benign and non-functional. It is important to recognize the features of this group of lipomatous tumours in the adrenal gland as they are being detected on increasing incidence as a result of the wide-spread use of modern imaging modalities.

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“… 3 Myolipomas are frequently found as incidental findings with CT scans and are more common in patients with thalassaemia, although not commonly in the adrenal glands. 4 , 11 , 14 , 17 , 18 …”

Section: Discussionmentioning

confidence: 99%

A rare case of adrenal extramedullary haematopoiesis in a Cypriot woman with β-thalassaemia

Georgiou

Lisacek-Kiosoglous

Mariannis

et al. 2024

annals

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We report a rare case of adrenal extramedullary haematopoiesis (EMH) in a thalassaemia patient in Cyprus. A 40-year-old woman with β-thalassaemia presented with a 2-day history of non-specific right-sided abdominal pain on routine follow-up for her thalassaemia treatment. Her laboratory tests were not dissimilar to her routine results and no palpable mass was detected. Computed tomography findings revealed a 5.8×4.2×4.6cm solid lesion in the right adrenal gland. Surgical excision was advised for this symptomatic large tumour with the possibility of malignancy in a young patient, and a laparoscopic adrenalectomy was performed. Postoperative follow-up was uneventful. A review of the literature in PubMed and MEDLINE revealed 14 case reports worldwide with adrenal EMH secondary to β-thalassaemia. EMH tumours in patients with thalassaemia have been reported incidentally, which stresses the importance of considering this in the list of differentials of adrenal incidentalomas in this patient population.

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A giant adrenal myelolipoma in a beta‐thalassemia major patient: Does ineffective erythropoiesis play a role?

Cited by 6 publications

References 8 publications

Adrenal myelolipoma: a comprehensive review

Adrenal myelolipoma: a comprehensive review

Lipomatous tumours in adrenal gland: WHO updates and clinical implications

A rare case of adrenal extramedullary haematopoiesis in a Cypriot woman with β-thalassaemia

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