Case Report Cylindroma of the Breast: A Rare Case Report Access this article online Website: Roopak Aggarwal, Oneal Gupta1, Yogesh Kumar Yadav2, Shruti Dogra1 www.ijtrichology.com DOI: Departments of Pathology, School of Medical Sciences and Research, Sharda Hospital, Greater Noida, 2Integral Institute of Medical Sciences and Research, 10.4103/0974-7753.122967 Lucknow, Uttar Pradesh, 1Safdarjung Hospital and V.M.M. College, New Delhi, India Quick Response Code:

ABSTRACT

Cylindroma is a benign adnexal tumor, which occurs as solitary dermal nodules on the scalp and forehead. Cylindroma of the breast is a rare lesion. Fine‑needle aspiration is often the first line of investigation. In this communication, we illustrate the cytomorphological feature of cylindroma in a 61‑year‑old female patient who presented with a breast lump. Fine‑needle aspiration cytology in this case reveals the islands of tumor cells formed a “jig‑saw” pattern, Address for correspondence: along with the bland, basaloid cells associated with globular, extracellular material. These Dr. Yogesh Kumar Yadav, features are typical of cylindroma, but differential diagnosis of adenoidcystic carcinoma 145, A‑Block, should always be kept in mind as the management of both tumors are different. South City, Rae Barely Road, Lucknow ‑ 226 025, Uttar Pradesh, India. E‑mail: [email protected] Key words: Adnexal tumor, breast lump, cylindroma

INTRODUCTION breast. Subsequently, fine‑needle aspiration cytology was done using 22‑G needle. Papanicolaou staining was ylindromas are benign skin appendage tumors. They done. Smears show numerous basaloid cells with scanty Cmost commonly occur on the head and neck as cytoplasm and round nuclei seen lying in jigsaw puzzle solitary or multiple tumors. Cylindroma of the breast is an pattern [Figure 2]. Diagnosis of “probably benign” (C2) extremely rare entity. To the best of our knowledge only was made and biopsy was advised. The nodule was excised. eleven cases have been published so far.[1‑4] Cylindroma of Histological sections stained by hematoxylin and eosin the breast is morphological and immunophenotypically revealed nests of basaloid cells arranged in a complex identical to benign dermal cylindroma. We discuss here the jigsaw puzzle like pattern. Many nests are surrounded salient cytomorphological feature of breast cylindroma so by dense eosinophilic basement membrane material and that they can be correctly diagnosed and managed. thus confirmed the diagnosis of cylindroma [Figure 3]. The patient was followed for 12 months, which remained uneventful. CASE REPORT

The present case report is about a 61‑year‑old woman DISCUSSION who presented with freely mobile, firm, non‑tender right breast mass of 2 cm diameter in upper inner quadrant Cylindromas are uncommon dysplasias of the skin since 4 years. There were no skin or nipple changes and no appendages. The word cylindroma was first used by axillary lymphadenopathy. The patient had no significant billroth in 1959 in the description of adenoid cystic family history or skin nodules. Mammography showed carcinoma (ADCC). It is derived from the histopathological a well‑circumscribed mass in the 1 o’clock position of appearance of tumor where the nests of cells surrounded the right breast [Figure 1]. According to The Breast by hyaline, resemble a cylinder in cross section. Cylindromas Imaging Reporting and Data System the score was 3 are relatively benign skin adnexal tumors occurring most which mean that mammogram was probably normal, commonly on the head and neck as a solitary nodule. but a repeat was suggested. Ultrasonography showed Multiple cylindromas may grow together in a “hat‑like” well‑defined heterogeneous mass noted in the right configuration, the so‑called “turban tumor.” Familial

International Journal of Trichology / Apr-Jun 2013 / Vol-5 / Issue-2 83 Aggarwal, et al.: Cylindroma of the breast: A rare case report cylindromatosis or Brooke‑Spiegler syndrome is a rare familial condition with autosomal dominant mode of inheritance and tends to form numerous adnexal tumors, in particular trichoepithelioma, cylindroma and occasionally spiradenoma. The lesions are widely distributed not only on the head and neck, but also the trunk and extremities. It is associated with mutations in the CYLD 1 gene.[5] Cylindroma are conventionally classified as a neoplasm of apocrine differentiation, but the ultrastructural and immunohistochemical evidence has also been presented in favor of an eccrine origin of cylindroma.[6,7] On the other hand, the fact that cylindromas exclusively arise in hair follicle‑bearing regions of the integument, but not in eccrine rich palmoplantar skin, which is devoid of Figure 1: Mammogram image showing a well-circumscribed mass pilosebaceous units and apocrine glands, already indicate in the 1 o’clock position of the right breast (mediolateral oblique view) against an eccrine origin.

According to Massoumi et al.[8] cylindroma epithelium likely originates from epithelial hair follicle, whose exact differentiation pathway (follicular vs. apocrine vs. sebaceous and may be even eccrine) may be dictated by the nature of the epithelial–mesenchymal signaling in a given patient and skin location as in every normal hair follicle.

Despite the deployment of a wide range of immunohistological, histochemical markers and ultrastructural studies, the histogenesis of cylindromas has remained a subject of continuous debate.

Cylindroma of the breast is a rare entity first described in 2001 by Gokaslan et al.[1] Extensive literature search showed [1‑4] Figure 2: Smears show numerous basaloid cells with scanty cytoplasm only eleven reported cases of breast cylindromas. Breast and round nuclei seen lying in jigsaw puzzle pattern (arrow) (Pap is considered as a modified sweat gland. Hence it can give stain, ×200). Insat: Smears show numerous basaloid cells with scanty rise to sweat gland type neoplasm; cylindroma of the breast cytoplasm and round nuclei (Pap stain, ×400) is one such type of neoplasm.

Fine‑needle aspiration is often the first line of investigation of breast lesions. The Cytomorphology of cylindroma of breast is indistinguishable from dermal cylindroma. Cytological smears are usually richly cellular comprising of two types of cells arranged in diffuse fashion or poorly formed acinar pattern. Small cells show scant cytoplasm and darkly stained round to oval nuclei and the other large cells with relatively abundant cytoplasm and round to oval vesicular pale staining nuclei. The cells may be associated with hyaline globules.

Histologically, the lesions are identical morphologically to dermal cylindromas. Cylindromas consist of nests and trabeculae of cells that mold in a “jigsaw” or “mosaic” Figure 3: Sections revealed nests of basaloid cells arranged in a complex jigsaw puzzle like pattern. Many nests are surrounded by appearance. The nests are composed of peripheral dense eosinophilic basement membrane material (H and E, ×200) basaloid cells with bland darkly stained nuclei and central

84 International Journal of Trichology / Apr-Jun 2013 / Vol-5 / Issue-2 Aggarwal, et al.: Cylindroma of the breast: A rare case report larger cells with oval vesicular nuclei. The nests also Primary Breast cylindroma are extremely rare benign show few duct‑like lumina with or without secretions. tumor. These should be distinguished from ADCC as the The nests are separated by thick basement membrane correct diagnosis may obviate the need for unnecessary material, which shows periodic acid Schiff positivity mastectomy. with diastase resistance and immunoreactivity to collagen type‑IV. Hyaline globules are present focally within the cell nests. Immunohistochemically, basaloid cells show REFERENCES diffuse positivity for p63 whereas larger central cells show [4] 1. Gokaslan ST, Carlile B, Dudak M, Albores‑Saavedra J. Solitary CK7‑positivity. cylindroma (dermal analog tumor) of the breast: A previously undescribed neoplasm at this site. Am J Surg Pathol 2001;25:823‑6. Cylindroma of the breast has to be differentiated from 2. Albores‑Saavedra J, Heard SC, McLaren B, Kamino H, Witkiewicz AK. solid type of ADCC both on cytological and histological Cylindroma (dermal analog tumor) of the breast: A comparison with cylindroma of the skin and adenoid cystic carcinoma of the breast. Am J examinations. Morphologically both tumors have a nodular Clin Pathol 2005;123:866‑73. and trabecular appearance, they also share basaloid and 3. Nonaka D, Rosai J, Spagnolo D, Fiaccavento S, Bisceglia M. Cylindroma central paler cells. Both tumors also show hyaline globules, of the breast of skin adnexal type: A study of 4 cases. Am J Surg Pathol 2004;28:1070‑5. basement membrane material and duct like lumina. 4. Mahmoud A, Hill DH, O’Sullivan MJ, Bennett MW. Cylindroma of the However, ADCC shows moderate to marked nuclear atypia breast: A case report and review of the literature. Diagn Pathol 2009;4:30. and brisk mitotic activity which are absent in cylindroma.[9] 5. Fenske C, Banerjee P, Holden C, Carter N. Brooke‑Spiegler syndrome locus ADCC may be associated with mucin production, a finding assigned to 16q12‑q13. J Invest Dermatol 2000;114:1057‑8. 6. Klein W, Chan E, Seykora JT. Tumors of the epidermal appendages. absent in cylindroma. In: Elder DE, Elenitsas R, Johnson BL, Murphy GF, editors. Lever’s Histopathology of the Skin. 9th ed. Ch. 30. Philadelphia: Lippincott Williams ADCC has an excellent prognosis, but there have and Wilkins; 2005. p. 897-8. been reported cases with local recurrence and distant 7. Cotton DW, Braye SG. Dermal cylindromas originate from the eccrine sweat metastases.[10] Breast cylindroma always runs a completely gland. Br J Dermatol 1984;111:53‑61. 8. Massoumi R, Podda M, Fässler R, Paus R. Cylindroma as tumor of hair benign course when treated with wide local excision. follicle origin. J Invest Dermatol 2006;126:1182‑4. Whereas, mastectomy or excisional biopsy with adjuvant 9. Shin SJ, Rosen PP. Solid variant of mammary adenoid cystic carcinoma with radiotherapy has been recommended as treatment of basaloid features: A study of nine cases. Am J Surg Pathol 2002;26:413‑20. [9] 10. Herzberg AJ, Bossen EH, Walther PJ. Adenoid cystic carcinoma of the solid‑variant of mammary ADCC. breast metastatic to the kidney. A clinically symptomatic lesion requiring surgical management. Cancer 1991;68:1015‑20. In this case, the mass was small and well ‑circumscribed for 4 years and also there was benign impression How to cite this article: Aggarwal R, Gupta O, Yadav YK, on cytology and imaging. This made the surgeon to Dogra S. Cylindroma of the breast: A rare case report. Int J proceed with excision of the mass rather than going for Trichol 2013;5:83-5. mastectomy. Source of Support: Nil, Conflict of Interest: None declared.

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