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research-article2014

IJSXXX10.1177/1066896914525234International Journal of Surgical PathologyDeSimone et al

Images in Pathology

Granular Cell Tumor of the Breast Eliciting Exuberant Pseudoepitheliomatous Hyperplasia

International Journal of Surgical Pathology 2014, Vol. 22(2) 156­–157 © The Author(s) 2014 Reprints and permissions: sagepub.com/journalsPermissions.nav DOI: 10.1177/1066896914525234 ijs.sagepub.com

Robert A. DeSimone, MD1, Paula S. Ginter, MD1, and Yao-Tseng Chen, MD, PhD1

Figure 1.  (A) Cut-section of tumor with overlying polypoid area on skin surface. (B) Papillary projections of epidermis and dermis with aggregates of chronic inflammatory cells (hematoxylin–eosin, 20× magnification). (C) Irregular epithelial projections of pseudoeptheliomatous hyperplasia (PEH) associated with compact nests of cells present in the dermis (hematoxylin–eosin, 200× magnification). (D) Granular cell tumor (GCT) cells (hematoxylin–eosin, 400× magnification).

A 63-year-old female presented with a slow-growing left breast mass first detected 10 years ago. She had declined surgical intervention despite increasing size of the mass and overlying skin changes. In addition, the patient had been experiencing cognitive decline with increasing forgetfulness, confusion, and weight loss for the past 3 years.

1

Weill Cornell Medical College, New York, NY, USA

Corresponding Author: Robert A. DeSimone, Department of Pathology and Laboratory Medicine, Weill Cornell Medical College, 525 East 68th Street, Starr 1036, New York, NY 10065, USA. Email: [email protected]

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DeSimone et al The patient underwent a segmentectomy after a diagnostic needle core biopsy. The resection specimen shows a polypoid lesion on the skin surface with intervening areas of retraction, resembling clusters of drupelets that comprise a mulberry (Figure 1A, inset). Cut section shows a 6 cm, yellow to white tumor with an infiltrative border directly beneath this polypoid area (Figure 1A). Microscopically, there are papillary projections of epidermis and dermis with aggregates of chronic inflammatory cells, but fibrovascular stalks that would be indicative of a papillary epithelial neoplasm are lacking (Figure 1B; 20× magnification). Instead, there are irregular epithelial projections of pseudoeptheliomatous hyperplasia (PEH) associated with compact nests of cells present in the dermis (Figure 1C; 200× magnification). The tumor itself is composed of nests and sheets of polygonal cells with abundant eosinophilic granular cytoplasm, round to oval nuclei with open chromatin and inconspicuous nucleoli, separated by strands of collagenous fibrous tissue (Figure 1D; 400× magnification), morphologically diagnostic of granular cell tumor (GCT). Immunohistochemical stains, previously performed on a needle core biopsy, showed the tumor cells were positive for S100 and negative for cytokeratin AE1/AE3, confirming the diagnosis of GCT. GCT is a benign neoplasm that can occur throughout the body and was first described in the breast in 1931.1 Although initially thought to be derived from muscle cells,2 more recent hypotheses suggest that these tumors are derived from Schwann cells of peripheral nerves or their precursors.3,4 Granular cell tumors, including those of the breast, are malignant in less than 1% of cases.5 Features that favor malignancy include necrosis, spindling, large nucleoli, pleomorphism, increased mitotic activity, and high nuclear to cytoplasmic ratio,6 none of which were observed in this case. PEH, characterized by hyperplasia of the epidermis and adnexal epithelium, is a benign

epithelial response to the underlying tumor and has been described in association with granular cell tumors7,8 and other tumors such as dermatofibroma.9 However, PEH is usually a microscopic finding that can potentially lead to the misdiagnosis of squamous cell carcinoma in a superficial shave biopsy. This case, on the other hand, represents an unusual case in which PEH is so exuberant that it grossly mimics a papillary neoplasm of the skin. References 1.  Abrikossof AI. Weitere untersuchungen über Myoblasten­ myome. Virchows Arch Pathol Anat. 1931;280:723-740. doi:10.1007/bf02038883. 2.  Abrikossof AI. Über myome, ausgehend von der quergestreifter willkürlicher Muskulatur. Virchows Arch Pathol Anat. 1926;260:215-233. doi:10.1007/bf02078314. 3.  Becelli R, Perugini M, Gasparini G, Cassoni A, Fabiani F. Abrikossoff’s tumor. J Craniofac Surg. 2001;12(1):78-81. 4.  Le BH, Boyer PJ, Lewis JE, Kapadia SB. Granular cell tumor: immunohistochemical assessment of inhibin-alpha, protein gene product 9.5, S100 protein, CD68, and Ki-67 proliferative index with clinical correlation. Arch Pathol Lab Med. 2004;128:771-775. 5. Rosen PP. Rosen’s Breast Pathology. 3rd ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2009. 6.  Fanburg-Smith JC, Meis-Kindblom JM, Fante R, Kindblom LG. Malignant granular cell tumor of soft tissue: diagnostic criteria and clinicopathologic correlation. Am J Surg Pathol. 1998;22:779-794. 7.  Brannon RB, Anand PM. Oral granular cell tumors: an analysis of 10 new pediatric and adolescent cases and a review of the literature. J Clin Pediatr Dent. 2004;29:69-74. 8. Torrijos-Aguilar A, Alegre-de Miquel V, Pitarch-Bort G, Mercader-Garcia P, Fortea-Baixauli JM. Cutaneous granular cell tumor: a clinical and pathologic analysis of 34 cases. Actas Dermosifiliogr. 2009;100:126-132. 9. Eng AM, Worobec S. Cytologic features of hyperplastic epidermis. J Cutan Pathol. 1977;4:258-265. doi:10.1111/ j.1600-0560.1977.tb00914.x.

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Granular cell tumor of the breast eliciting exuberant pseudoepitheliomatous hyperplasia.

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