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LETTERS TO EDITOR  
Year : 2020  |  Volume : 63  |  Issue : 1  |  Page : 150-151
The chicken or the egg: An intriguing case of Ewing's sarcoma/primitive neuroectodermal tumor, adrenal to breast or vice versa?


Department of Pathology, School of Medicine, D. Y. Patil Deemed to be University, Nerul, Navi Mumbai, Maharashtra, India

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Date of Web Publication31-Jan-2020
 

How to cite this article:
Bhat RU, Ingle KD, Sudhamani S, Roplekar PM. The chicken or the egg: An intriguing case of Ewing's sarcoma/primitive neuroectodermal tumor, adrenal to breast or vice versa?. Indian J Pathol Microbiol 2020;63:150-1

How to cite this URL:
Bhat RU, Ingle KD, Sudhamani S, Roplekar PM. The chicken or the egg: An intriguing case of Ewing's sarcoma/primitive neuroectodermal tumor, adrenal to breast or vice versa?. Indian J Pathol Microbiol [serial online] 2020 [cited 2020 Feb 26];63:150-1. Available from: http://www.ijpmonline.org/text.asp?2020/63/1/150/277376




Editor,

A 27-year-old female presented with pain in left iliac fossa. The patient was operated outside 3 months back for right cystic breast lesion? Galactocele. CT abdomen: thick-walled peripherally enhancing lesion involving upper pole of left kidney—13.7 × 10.1 cm.

HRCT chest: multiple heterogeneously enhancing small lesions in right breast with a lesion in right lung. Left radical nephrectomy with mass was received.

Gross examination revealed well-circumscribed cystic tumor mass—10.5 × 9 × 4.8 cm—abutting the upper pole of kidney but not infiltrating it. It showed friable, gray-brown areas of hemorrhage and necrosis [Figure 1]. Adrenal gland could not be identified separately.
Figure 1: Kidney along with tumor mass

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Microscopically, malignant round cell tumor in lobules, nests, and sheets, involving adrenal gland was seen [Figure 2]a. Tumor cells were monomorphic with coarse stippled chromatin, inconspicuous nucleoli, and scant cytoplasm. Many Homer Wright rosettes and necrosis were noted [Figure 2]b. Mitosis: 7 to 8/10 hpf. Renal parenchyma was unremarkable.
Figure 2: (a). H and E, 400×, infiltrative malignant round cell tumor involving the adrenal gland. (b). H and E, 400×, tumor mass with Homer Wright rosettes. (c). CD99/MIC 2, 400×, adrenal mass, tumor cells show diffuse membranous positivity

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Differentials were Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) and adult neuroblastoma.

Immunohistochemically, strong and diffuse positivity for MIC–2/CD99 and negativity for synaptophysin and chromogranin were noted [Figure 2]c.

Subsequently, we received histopathology and IHC profile of breast mass from outside, which was ES/PNET [Figure 3].
Figure 3: (a). H and E, 400×, breast mass showing infiltrative malignant round cell tumor with Homer Wright rosettes. (b). CD99/MIC 2, 100×,breast mass, tumor cells show membranous positivity

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Correlating the above findings, possibility of primary ES/PNET of left adrenal gland with metastasis to breast, or vice versa was suggested. The patient was started on chemotherapy and was advised to undergo genetic testing; however, the patient was lost for follow-up.

ES/PNET is a tumor derived from neuroectoderm usually seen in young adults.[1]

The literature survey revealed only 13 cases of primary ES/PNET of adrenal gland and 10 cases of primary ES/PNET of breast.

Immunohistochemically, ES/PNET is positive for CD99 and FLI-1, whereas negative in adult neuroblastoma.[2] ES/PNET is defined by specific translocations involving EWSR1 gene, fused to E26 transformation-specific (ETS) family gene (FLI-1, ERG, or ETV-1).[3]

In our case, the distinction between primary ES/PNET of adrenal gland metastasizing to the breast or vice versa was difficult [Table 1].
Table 1: Comparison between ES/PNET of adrenal gland and ES/PNET of breast

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Thus, ours was an intriguing case where the possible primary tumor could not be concluded.[5]

Acknowledgements

The authors would like to thank the Department of Pathology. Sion Hospital, Mumbai, for the Immunohistochemistry studies.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Phukan C, Nirmal TJ, Kumar RM, Kekre NS. Peripheral primitive neuroectodermal tumor of the adrenal gland: A rare entity. Indian J Urol 2013;29:357-9.  Back to cited text no. 1
[PUBMED]  [Full text]  
2.
Zhang F, Yang B, Yan N, Xu H. Primitive neuroectodermal tumor of the breast: A case report and review of the literature. Int J Clin Exp Med 2016;9:8780-7.  Back to cited text no. 2
    
3.
Choi EY, Gardner JM, Lucas DR, McHugh JB, Patel RM. Ewing sarcoma. Semin Diagn Pathol 2014;31:39-47.  Back to cited text no. 3
    
4.
Toogood V, Milliken S, Morey A, Samaras K. Adrenal tumours: How to establish malignancy. BMJ Case Rep 2014;2014:bcr2014203736.  Back to cited text no. 4
    
5.
Vaidya T, Ramani S, Rastogi A. A case series of metastases to the breast from extramammary malignancies. Indian J Radiol Imaging 2018;28:470-5.  Back to cited text no. 5
[PUBMED]  [Full text]  

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Correspondence Address:
Ketan D Ingle
Department of Pathology, School of Medicine, D Y Patil Deemed to be University, Sector 5, Nerul, Navi Mumbai - 400 706, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_326_19

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    Figures

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    Tables

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