Combined adrenal medullary hyperplasia and myelolipoma: a mimicker of pheochromocytoma/Adrenal meduller hiperplazi ve myelolipom kombinasyonu: bir feokromositoma taklitcisi.
A 53-year-old female patient with long-standing hypertension was
evaluated for left flank pain. Abdominal CT scan revealed a 2,8 cm left
adrenal mass. Metanephrine, normetanephrine and vanylmandelic acid
levels in a 24-hour urine sample were increased. The levels of serum
cortisol, renin, aldosteron, calcitonin, parathormone, calcium and
phosphate were normal. Left adrenalectomy was performed. There was a
nodular mass with a red cut surface in medullary region. The medulla was
enlarged in other parts. On microscopic examination, the mass was
composed of mature adipose tissue admixed with hematopoietic cells. The
medulla was hyperplastic with a corticomedullary ratio of 1:5. After
surgery, blood pressure and catecholamine levels normalized. Although
myelolipomas are incidental findings, they can rarely present with
endocrine dysfunction. In conclusion, surgical excision should be
considered in adrenal incidentalomas with characteristic radiographic
features of myelolipoma, presenting with biochemical abnormalities.
Key words: Myelolipoma, adrenal medullary hyperplasia, pheochromocytoma
Uzun suredir hipertansif olan 53 yasinda kadin hasta sol yan agrisi nedeniyle tetkik edildi. Batin BT de sol adrenalde 2,8 cm capinda kitle saptandi. 24 saatlik idrarda metanefrin, normetanefrin ve vanil mandelik asit seviyeleri yuksekti. Serum kortizol, renin, aldosteron, kalsitonin, fosfat seviyeleri normaldi. Sol adrenalektomi uygulandi. Meduller bolgede kesit yuzu kirmizi olan noduler kitle goruldu .Medullanin diger alanlari genislemisti. Mikroskopik incelemede kitle matur yag dokusu ve hematopoetik hucrelerden olusuyordu. Kortikomeduller oran ise 1:5 idi. Cerrahi sonrasi kan basinci ve katekolamin seviyeleri normale dondu. Myelolipomlar insidental lezyonlar olmakla birlikte nadiren endokrin disfonksiyonla ortaya cikabilirler. Sonuc olarak biyokimyasal anormallikle ortaya cikan adrenal insidentalomalar radyolojik olarak karakteristik myelolipom ozellikleri gosterse bile cerrahi eksizyon dusunulmelidir.
Anahtar kelimeler: Myelolipom, adrenal meduller hiperplazi, feokromositoma
|Article Type:||Case study|
Connective tissue tumors
Connective tissue tumors (Care and treatment)
Connective tissue tumors (Case studies)
Adrenalectomy (Health aspects)
Ertugrul, Derun Taner
|Publication:||Name: Turkish Journal of Endocrinology and Metabolism Publisher: Galenos Yayincilik Audience: Academic Format: Magazine/Journal Subject: Health Copyright: COPYRIGHT 2009 Galenos Yayincilik ISSN: 1301-2193|
|Issue:||Date: Dec, 2009|
|Topic:||Canadian Subject Form: Connective tissue tumours; Connective tissue tumours; Connective tissue tumours|
|Geographic:||Geographic Scope: Turkey Geographic Code: 7TURK Turkey|
Adrenal myelolipomas are rare, benign, hormonally nonfunctioning tumors that are composed of mature adipose tissue and normal hematopoietic tissues. They are usually asymptomatic and are often found incidentally on radiographic studies. The pathogenesis of adrenal myelolipomas remains unclear, but most of the evidence supports the metaplastic changes of reticuloendothelial cells of blood capillaries as the etiology (1).
These benign tumors usually remain small, but occasionally they reach massive proportions and become symptomatic (2). Most of the cases are not associated with any specific endocrine dysfunction. Adrenal myelolipoma, which is a nonfunctional tumor, may be found coincidentally in patients affected by Cushing's syndrome, hyperaldosteronism, pheochromocytoma, adrenogenital syndrome, and virilization (3,4,5).
Here, we report a case of myelolipoma associated with medullary hyperplasia presenting with the clinical findings of pheochromocy-toma.
[FIGURE 1 OMITTED]
Adrenal incidentalomas are adrenal masses that are discovered serendipitously during a radiologic examination performed for indications other than evaluation of adrenal diseases. Adrenal incidentalomas are found in 0.3% to 5% of patients undergoing abdominal CT, and adrenal myelolipoma accounts for about 1.9% of adrenal incidentalomas (6). In a recent prospective study, during the follow-up after 24 months, it was observed that 10.2% of adenomas showed increase in tumor diameter (7). The 2002 National Institutes of Health state-of-the-science statement on management of incidentalomas proposed surgical removal of lesions larger than 6 cm, either adrenalectomy or close follow-up for intermediate lesions between 4 and 6 cm, and a conservative approach for lesions smaller than 4 cm (8). In our case, although the tumor was smaller than 4 cm, adrenalectomy was performed because metanephrine, normetanephrine and vanillylmandelic acid levels in a 24-hour urine sample were increased. Myelolipomas are benign tumors of the adrenal gland. These lesions have been recognized with increasing frequency, because of their characteristic appearance on CT scanning and MRI, which is helpful in establishing the diagnosis. These tumors are generally smaller than 5 cm, unilateral, asymptomatic and benign, containing hematopoietic and fatty elements. They sometimes grow to a very large size and cause pressure effects which manifest as abdominal pain. Treatment of adrenal myelolipoma is usually conservative. Periodic follow-up with ultrasound or CT scan over a period of 1 to2 years has been recommended, with surgery reserved for symptomatic patients.
The lesions are rarely hormonally active and can present with endocrine dysfunction. In the literature, several cases of adrenal myelolipomas associated with hyperaldosteronism, congenital adrenal hyperplasia and Cushing's syndrome were reported (3,4,5). An association with homolateral or contralateral pheochromocytoma has also been reported (9,10). Although the cause of the lesion is unknown, combination of myelolipoma with hormonally active neoplasms, as in our case, supports the theory that hormonal microenvironment plays a role in the development of these lesions (4). To our knowledge, only one case associated with adrenal medullary hyperplasia was reported to date (11). We believe this to be the second case of adrenal medullary hyperplasia associated with myelolipoma causing a syndrome of pheochromocytoma. In the previous case, myelolipoma and medullary hyperplasia were fused, while in our case, they were not fused, but both were located in the medullary region of the gland. These findings support the theory of myelolipomas being a metaplastic change (1). We did not perform RET mutation testing in this patient who appears to be a sporadic pheochromocytoma case and has no family history of MEN 2 syndrome. Measurements of serum parathormone and basal serum calcitonin measurements were normal, and the patient had undergone subtotal thyroidectomy previously, which did not show any sign of medullary thyroid car cinoma. The previous case of adrenal medullary hyperplasia combined with myelolipoma was also a unilateral, sporadic case showing no abnormality in RET proto-oncogene (11). In conclusion, myelolipomas can rarely be associated with other hormone-secreting tumors, and appropriate biochemical and radiologic evaluation should be performed in cases with unusual symptoms and signs.
Recevied: 23.11.2009 Accepted: 26.11.2009
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(2.) Lamont JP, Lieberman ZH, Stephen JS. Giant adrenal myelolipoma. Am Surg 2002; 68: 392-394.
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(7.) Comlekci A, Yener S, Ertilav S, Secil M, Akinci B, Demir T, Kebapcilar L, Bayraktar F, Yesil S, Eraslan S. Adrenal incidentaloma, clinical, metabolic, follow-up aspects: single centre experience. Endocrine, 2009 Oct 30.
(8.) NIH Consens State Sci Statements 2002; 19: 1-25.
(9.) Ukimura O, Inui E, Ochiai A, et al. Combined adrenal myelolipoma and pheochromocytoma J Urol 1995; 154: 1470.
(10.) Rocher L, Youssef N, Tasu JP, et al. Adrenal pheochromocytoma and contralateral myelolipoma. Clin Radiol 2002; 57: 534-536.
(11.) Ishay A, Dharan M, Luboshitzky R. Combined adrenal myelolipoma and medullary hyperplasia. Horm Res 2004; 62: 23-26.
Address for Correspondence: Servet Guresci, MD, Kecioren Training and Research Hospital, Pathology, Ankara, Turkey Email: firstname.lastname@example.org
Servet Guresci, Cengiz Kara *, Derun Taner Ertugrul **, Ali Unsal *
Kecioren Training and Research Hospital, Pathology, Ankara, Turkey * Kecioren Training and Research Hospital, Urology, Ankara, Turkey ** Kecioren Training and Research Hospital, Internal Medicine, Ankara, Turkey
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