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Year : 2011  |  Volume : 54  |  Issue : 3  |  Page : 635-637
A rare association of giant adrenal myelolipoma in a young female double heterozygous for HbD Punjab and β-thalassemia trait


1 Department of Histopathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Radiodiagnosis, Post Graduate Institute of Medical Education and Research, Chandigarh, India
3 Department of Haematology, Post Graduate Institute of Medical Education and Research, Chandigarh, India

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Date of Web Publication20-Sep-2011
 

How to cite this article:
Kumaresan K, Gupta K, Kalra N, Das R. A rare association of giant adrenal myelolipoma in a young female double heterozygous for HbD Punjab and β-thalassemia trait. Indian J Pathol Microbiol 2011;54:635-7

How to cite this URL:
Kumaresan K, Gupta K, Kalra N, Das R. A rare association of giant adrenal myelolipoma in a young female double heterozygous for HbD Punjab and β-thalassemia trait. Indian J Pathol Microbiol [serial online] 2011 [cited 2020 Dec 2];54:635-7. Available from: https://www.ijpmonline.org/text.asp?2011/54/3/635/85126


Adrenal myelolipomas are rare benign tumors composed of mature adipose tissue and hematopoietic elements that resemble bone marrow. These are usually asymptomatic, and most cases are incidentally found at radiological examination or autopsy. But, with increasing size, as seen in this case, myelolipomas can cause flank pain and abdominal distention. Myelolipomas rarely exceed 5 cm in diameter; however, giant myelolipomas have been described in the literature as sporadic case reports.[1],[2],[3] Here, we describe a rare association of giant adrenal myelolipoma in a young female double heterozygous for HbD Punjab and b thalassemia trait, which has not been reported in the literature, to the best of our knowledge.

A 24-year-old woman, double heterozygous for HbD Punjab and b thalassemia trait, was admitted with a history of abdominal pain and right-sided abdominal lump for four months duration. Ultrasound examination of the mass revealed a large exophytic heterogeneous mass in relation to inferior part of the liver and extending till mid abdomen, with inferior displacement of the right kidney [Figure 1]. The computed tomography scan revealed a right retroperitoneal mass with fat density, measuring 24 x 17 x 14 cm, with anteroinferior displacement of the ipsilateral kidney, supero-medial displacement of pyloroduodenum, and head of pancreas [Figure 1]. The differential diagnoses considered on radiological investigations were a giant adrenal myelolipoma, large hepatic adenoma, and giant hemangioma of the liver. The retroperitoneal mass replacing the right adrenal gland was resected en bloc.
Figure 1: Axial CECT section shows a large mass in the right suprarenal location with soft tissue and fat density and a small speck of calcification. The planes of the mass with the liver are lost. The left suprarenal gland is normal (arrow)

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The mass was encapsulated, weighing 3,000 g and measured 30 x 20 x 18 cm. The cut surface showed large zones of pale yellow colored areas consistent with adipose tissue [Figure 2]a separated by red-colored irregular areas, with a soft consistency. No areas of cystic change or necrosis were identified. No normal adrenal gland was identified at the periphery. Histologically, the lesion revealed islets of myeloid, erythroid, and megakaryocytic cell lines in varying proportions, resembling the mature bone marrow elements [Figure 2]b. These hematopoietic islands were separated by large clusters and sheets of mature adipocytes admixed with hemorrhagic foci [Figure 2]c. Islands of normal adrenal gland were identified randomly distributed between the adipocytes and hematopoietic islands [Figure 2]d. Based on the gross and microscopic findings, diagnosis of a giant myelolipoma was made. There was no associated endocrinological disorder. Interestingly, on laboratory investigations, this patient was found to have mild anemia as she was double heterozygous for HbD Punjab [b 121(GH4)Glu>Gln] and b thalassemia trait with a raised level of HbA2. The hemoglobin (Hb) electrophoresis at alkaline pH of 8.6 showed predominantly a band in the Hb S/D/G region and raised HbA2 [Figure 3]a. High-performance liquid chromatography (HPLC) revealed a dominant peak of 82.2% in the HbD window and HbA2 of 6.2% [Table 1] and [Figure 3]b.
Table 1: Percentage and retention time of haemoglobin subtypes found on HPLC

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Figure 2: (a) Cut surface revealing large areas of hemorrhage and pale yellow colored areas consistent with adipose tissue. (b) Islets of myeloid, erythroid and megakaryocytic cell lines in varying proportions admixed with adipocytes [hematoxylin and eosin (H and E) ×400, original magnification]. (c) Hematopoietic islands were separated by large clusters and sheets of mature adipocytes admixed with hemorrhagic foci (H and E ×200, original magnification). (d) Islands of normal adrenal gland randomly distributed between adipocytes and hematopoietic islands (H and E ×200, original magnification)

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Figure 3: (a) Hb electrophoresis showing a prominent band in the SDG and A2CE regions, with reduced normal HbA0. (b) HPLC revealed dominant HbD peak (82.2%), HbA2 (6.2%), and HbA0 (2.2%)

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   Discussion Top


Myelolipomas are benign tumors composed of variable amounts of mature fat and hematopoietic elements, typically found in the adrenal gland. These are relatively rare, with an estimated incidence at autopsy in the range of 0.08-0.8%. [1] The largest myelolipoma of 34-cm diameter and weighing 5,900 g was described by Boudreaux et al . [1] The second largest (diameter 30 cm, weight 5,500 g) was described by Wihelmus et al., [2] and the third largest (diameter 30 cm, weight 4,370 g) reported by O'Daniel-Pierce et al . [3] The present case manifested with huge abdominal mass measured 30 20 18 cm and weighed 3,000 g. Histopathological evaluation revealed islands of adrenal cortex (zona glomerulosa) scattered in between the adipocytes and hematopoietic islands.

The etiology and pathogenesis of myelolipoma remains obscure; various theories have been proposed including metaplasia of embryonic nests of stem cells located in the adrenal glands. Large adrenal myelolipomas have been reported in patients with chronic hemolysis, including hereditary spherocytosis, thalassemia intermedia and thalassemia major, as well as sickle cell anemia [4],[5],[6],[7] [Table 2]. Such an association suggests that tumor growth is also under the extrinsic stimulation of erythropoietin. Thus, extramedullary adrenal hematopoiesis may contribute to the pathogenesis of adrenal myelolipomas.
Table 2: Summary of previous reports of adrenal myelolipoma with underlying hematological association

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In this case, development of adrenal myelolipoma may be incidentally associated with b thalassemia trait, but it is also possible that hematopoietic stimulus by chronic anemia of thalassemia might induce compensatory hematopoiesis in the adrenal gland. In conclusion, this case describes this rare association between myelolipomas and the underlying hematological disorder, which may often be incidental; however, rare red cell disorders should be carefully excluded.

 
   References Top

1.Boudreaux D, Waisman J, Skinner DG, Low R. Giant adrenal myelolipoma and testicular interstitial cell tumor in a man with congenital 21-hydroxylase deficiency. Am J Surg Pathol 1979;3:109-23.  Back to cited text no. 1
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2.Wilhelmus JL, Schrodt GR, Alberhasky, Alcorn MO. Giant adrenal myelolipomas: Case report and review of the literature. Arch Pathol Lab Med 1981;105:532-35.  Back to cited text no. 2
    
3.O'Daniel-Pierce ME, Weeks JA, McGrath PC. Giant adrenal myelolipoma. Southern Med J 1996;89:1116-8.  Back to cited text no. 3
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4.Sekido N, Kawai K, Takeshima H, Uchida K, Akaza H, Koiso K. Adrenal myelolipoma associated with hereditary spherocytosis. Int J Urol 1996;3:61-3.  Back to cited text no. 4
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5.Au WY, Tam PC, Ma SK, Lam KY. Giant myelolipoma in a patient with thalassemia intermedia. Am J Hematol 2000;65:265-6.  Back to cited text no. 5
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6.Kelekis NL, Alexopoulou E, Brountzos EN, Ladis V, Boussiotou A, Kelekis DA. Giant adrenal myelolipoma with minimal fat content in a patient with homozygous beta-thalassemia: Appearance on MRI. J Magn Reson Imaging 2003;18:608-11.  Back to cited text no. 6
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7.Gamss C, Chia F, Chernyak V, Rozenblit A. Giant hemorrhagic myelolipoma in a patient with sickle cell disease. Emerg Radiol 2009;16:319-22.  Back to cited text no. 7
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Correspondence Address:
Kirti Gupta
Department of Histopathology, PGIMER, Chandigarh - 160 012
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.85126

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    Figures

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    Tables

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