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Year : 2015  |  Volume : 11  |  Issue : 4  |  Page : 1040

Myxoid adrenal cortical adenoma in an infant: An unusual morphology

1 Department of Pathology, Institute of Post Graduate Medical Education and Research, Kolkata, India
2 Department of Radiology, Medical College and Hospital, Kolkata, West Bengal, India
3 Department of Pathology, KPC Medical College and Hospital, Jadavpur, Kolkata, West Bengal, India
4 Department of Pediatric Surgery, KPC Medical College and Hospital, Jadavpur, Kolkata, West Bengal, India

Date of Web Publication15-Feb-2016

Correspondence Address:
Uttara Chatterjee
57/11A, Ballygunje Circular Road, Kolkata - 700 019, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.150358

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 > Abstract 

Myxoid adrenal cortical neoplasms are rare. To the best of our knowledge, no such case has been reported in pediatric or infantile age group till now. Here we report a case of non-functional myxoid adrenocortical adenoma (ACA) in a 7-month-old girl, who presented with a large mass in the abdomen. Microscopically, the tumor was composed of alveolar clusters of cells with focal pseudoglandular architecture in a background of abundant alcian blue positive myxoid matrix. Compressed rim of adrenal tissue was identified at periphery. The patient was put on a close follow-up in view of scarce literature on the subject. She has been doing fine without any recurrences. Myxoid adrenal cortical tumors expand the differential diagnoses of a myxoid neoplasm in retroperitoneum.

Keywords: Infantile adrenal neoplasm, myxoid adrenal cortical tumor, pediatric adrenal cortical tumor

How to cite this article:
Chatterjee G, Dasgupta S, Bhattacharya K, Banerjee M, Ghosh R, Chatterjee U, Banerjee S. Myxoid adrenal cortical adenoma in an infant: An unusual morphology . J Can Res Ther 2015;11:1040

How to cite this URL:
Chatterjee G, Dasgupta S, Bhattacharya K, Banerjee M, Ghosh R, Chatterjee U, Banerjee S. Myxoid adrenal cortical adenoma in an infant: An unusual morphology . J Can Res Ther [serial online] 2015 [cited 2021 Dec 1];11:1040. Available from: https://www.cancerjournal.net/text.asp?2015/11/4/1040/150358

 > Introduction Top

Adrenocortical adenoma (ACA) and Adrenocortical Carcinoma (ACC) present with a variety of histological patterns including diffuse, alveolar, or trabecular. One of the rarer histological variants is myxoid type, which shows alcian blue positive myxoid material in the stroma. Literature is scarce on the subject with few cases reported in children. ACA and ACC occur primarily in adults with pediatric adrenocortical tumors being rare. [1],[2] Pediatric adrenal cortical tumors behave differently from their histologically similar adult counterparts. The applicability of Weiss criteria in separating ACA from ACC in children is controversial. [3] To our knowledge, myxoid pediatric adrenal cortical tumor has not been reported in literature till date. We take this opportunity to report a case of a non-functional myxoid ACA occurring in an infant and describe its histopathological features.

 > Case report Top

A 7-month-old girl presented with a rapidly increasing mass in abdomen for 4 months. Antenatal Ultrasonography (USG) was unremarkable. On examination, a huge mass was palpable in the abdomen. Her blood pressure was normal. There were no endocrine symptoms or bladder/bowel abnormalities. The routine investigations were normal except for a marginally raised alpha fetoprotein (46 ng/mL).

USG showed a large intra-abdominal mass. Computed tomography (CT) scan showed a retroperitoneal mass pushing the liver forwards and kidney downwards [Figure 1]a-c].
Figure 1: (a) Scout AP film of the abdomen showing soft tissue density in the right hypochondrium pushing the bowel loops inferiorly, (b) CECT abdomen coronal reconstruction showing large, rounded well-defined, variegated, predominantly hypodense mass lesion associated with the upper pole of right kidney pushing the kidney downwards, (c) CECT abdomen sagittal reconstruction showing the mass located posterior to the liver, pushing it anteriorly and the right kidney downwards, (d) Gross appearance showing a large, ovoid mass with myxoid and slimy areas

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She was operated upon and the mass was mobilized by reflecting the duodenum to the left. The mass was separated from the midline major vessels and it was arising from the right adrenal region. The mass was friable, translucent, and jelly-like. It was by and large scooped out in large chunks. Its outer rind was completely removed. Post-operative course was uneventful.

Pathologic features

The tumor appeared bosselated, slimy, measured 15 × 10 × 6 cm and weighed 425 g. The cut surface showed gelatinous and myxoid areas with cystic changes [Figure 1]d].

The mass was encapsulated and compressed adrenal cortical tissue was identified at the periphery. The tumor was predominantly composed of alveolar clusters of lipid-rich cells in a background of abundant myxoid matrix which constituted about 80% of the total tumor. Most of the cells showed round nuclei with presence of occasional nucleoli. A few areas showed pseudoglandular arrangement of the tumor cells. Mitotic figures were hardly seen. Evidence of obvious cellular atypia, necrosis, capsular or vascular invasion or periadrenal extension was lacking. Extracellular myxoid areas werealcian blue positive [Figure 2]. Ki-67 labelling index (MIB-1) was <1%. Based on these features, a diagnosis of ACA was made.
Figure 2: (a) Photomicrograph from the mass showing alveolar clusters of lipid rich cells in an abundant myxoid background (H and E, ×400), (b) Photomicrograph shows compressed adrenal rim at the periphery adjacent to the mass (H and E, ×40). Inset shows the adrenal tissue under higher power (H and E, ×100), (c) Photomicrograph showing pseudoglandular arrangement of the tumor cells (H and E,×400), (d) Photomicrograph shows presence of abundant alcian blue positive myxoid stroma within the tumor mass (×100)

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The patient is being followed up with USG scans at 3 monthly intervals. She is doing fine without any recurrence or metastasis in the last 1 year.

 > Discussion Top

Myxoid change in an ACA/ACC is rare. Since the first case reported by Tang et al. [4] in 1979, only about 50 cases have been reported in literature with an age range of 16-72 years. In the largest study till date, Brown et al. [5] described 14 cases of myxoid adrenal cortical neoplasms which constituted <3% of the ACCs and <1% of the ACAs. In a recent study, Zhang et al. [6] studied the clinicopathological profile and epidermal growth factor receptor (EGFR) gene status in 10 cases of myxoid adrenal cortical tumors. Till now, myxoid change has not been described in pediatric adrenocortical tumors.

The myxoid adrenal cortical tumors usually show trabecular/alveolar arrangement of tumor cells floating in amyxoid background. The proportion of myxoid stroma in these series varied from 10-90%. [7] Many of these series have reported pseudoglandular arrangement of tumor cells. [7],[8] De Padua and Rajagopal. [8] suggested that the myxoid stroma may promote pseudoglandular architecture as it has not been reported in conventional adrenal cortical tumors.

The extracellular myxoid substance usually shows alcian blue positivity with a few cases showing focal PAS/mucicarmine positivity. The pathogenesis of myxoid change in adrenal tumors is not clear. Forsthoefel et al.[9] proposed that mucin production is the result of a degenerative process. Honda et al.[10] suggested that adrenocortical cells produce myxoid matrix reflecting their mesodermal origin. However, intracellular mucin has not been demonstrated in these tumors so far.

The most important task for a pathologist in these cases is to separate ACA from ACC to predict the prognosis. The malignant potential of myxoid change in adrenal cortical tumors is not clear. Some series have shown that myxoid change is associated with more aggressive clinical behavior. [11],[12] However, Brown et al.[5] and Zhang et al. [6] have recommended use of usual clinicopathological criteria to classify these lesions as benign or malignant.

The situation in pediatric adrenal cortical tumors is however different. [1],[2] Adrenal cortical neoplasms in the pediatric age-group are rare with ACC forming the majority. [2] It is well-established that pathological criteria for malignancy in adults cannot be reliably applied to pediatric tumors. In the largest study till date, Wieneke et al. [1] analyzed the histopathology of 83 pediatric adrenal cortical tumors and proposed a clincopathologic criteria for malignancy in the pediatric population. Nine parameters were included, of which presence of 4 or more indicate malignancy, whereas 2 or less suggest a benign nature. Applying this criteria, our case was in the benign group [Table 1].
Table 1: Wieneke criteria as applied to our case

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However, Wieneke et al.[1] suggested that cases should be assessed on an individual basis as no single pathologic feature can accurately separate benign from malignant pediatric adrenal cortical tumors. The significance of myxoid change has not been addressed in pediatric adrenal cortical tumors. In our case, the baby is doing well on a 1-year follow-up.

So to conclude, myxoid change in an adrenal cortical tumor should be kept in mind in the differential diagnosis of myxoid neoplasms in the retroperitoneum. The differential diagnoses in pediatric population include chordoma, malignant peripheral nerve sheath tumor, and well-differentiated liposarcoma with myxoid change, germ cell tumors with myxoid background. In view of scarcity of literature on the subject, each case should be assessed individually with careful attention to clinicopathologic parameters. A close follow-up is required to know more about the nature of these tumors.

 > Conclusion Top

We report a case of infantile adrenal cortical adenoma showing extensive myxoid change, which to our knowledge is the first reported case of its type in the pediatric age-group.

 > References Top

Wieneke JA, Thompson LD, Heffess CS. Adrenal cortical neoplasms in the pediatric population: A clinicopathologic and immunophenotypic analysis of 83 patients. Am J Surg Pathol 2003;27:867-81.  Back to cited text no. 1
Dehner LP, Hill DA. Adrenal cortical neoplasms in children: Why so many carcinomas and yet so many survivors? Pediatr Dev Pathol 2009;12:284-91.  Back to cited text no. 2
Weiss LM. Comparative histologic study of 43 metastasizing and nonmetastasizing adrenocortical tumors. Am J SurgPathol 1984;8:163-9.  Back to cited text no. 3
Tang CK, Harriman BB, Toker C. Myxoid adrenal cortical carcinoma: A light and electron microscopic study. Arch Pathol Lab Med 1979;103:635-8.  Back to cited text no. 4
Brown FM, Gaffey TA, Wold LE, Lloyd RV. Myxoid neoplasms of the adrenal cortex: A rare histologic variant. Am J SurgPathol 2000;24:396-401.  Back to cited text no. 5
Zhang J, Sun J, Liang Z, Gao J, Zeng X, Liu T. Myxoid adrenocortical neoplasms: A study of the clinicopathologic features and EGFR gene status of ten Chinese cases. Am J Clin Pathol 2011;136:783-92.  Back to cited text no. 6
Bollito ER, Papotti M, Porpiglia F, Terzolo M, Cracco CM, Capia S, et al. Myxoid adrenal cortical adenoma with a pseudoglandular pattern. Virchows Arch 2004;445:414-8.  Back to cited text no. 7
De Padua M, Rajagopal V. Myxoid adrenal adenoma with focal pseudoglandular pattern. Indian J Med Sci 2008;62:199-203.  Back to cited text no. 8
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Forsthoefel KF. Myxoid adrenal cortical carcinoma: A case report with differential diagnostic considerations. Arch Pathol Lab Med 1994;118:1151-3.  Back to cited text no. 9
Honda K, Kashima K, Daa T, Gamachi A, Nakyama I, Yokoyama S. Myxoid adrenal cortical adenoma. Pathol Int 2001;51:887-91.  Back to cited text no. 10
Papotti M, Volante M, Duregon E, Delsedime L, Terzolo M, Berruti A, et al. Adrenocortical tumors with myxoid features: A distinct morphologic and phenotypical variant exhibiting malignant behaviour. Am J Surg Pathol 2010;34:973-83.  Back to cited text no. 11
Weissferdt A, Phan A, Suster S, Moran C. Myxoid adrenocortical carcinoma: A clinicopathologic and immunohistochemical study of 7 cases, including 1 case with lipomatous metaplasia. Am J Clin Pathol 2013;139:780-6.  Back to cited text no. 12


  [Figure 1], [Figure 2]

  [Table 1]


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