Solitary Dermal Cylindroma: a Rare Case Report

J Med Sci, Volume 51, Number 2, 2019 April: 181-187

Journal of the Medical Sciences (Berkala Ilmu Kedokteran)

Volume 51, Number 2, 2019; 181-187 http://dx.doi.org/10.19106/JMedSci005102201910

Solitary dermal cylindroma: a rare case report

Wahyu Tri Widayati, Irianiwati* Department of Anatomical Pathology, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada, Yogyakarta, Indonesia

ABSTRACT

Submited : 2017-02-10 Dermal cylindromas are rare benign skin appendageal tumors that has two Accepted :2019-04-24 clinical presentation, solitary and multiple form. The diagnosis is unusual. However, it must be considered in clinical practice.A 51-year-old woman hadpainless nodule on right arm. Skin examinatipn revealedtwo fragmented nodules, 1x0.5x0.5cm, tan to white, and rubbery. Histopathological findings revealed a well demarcated and an unencapsulated epithelial dermal tumor, composed of numerous oval and polygonal nests molded into a “jig-saw” or “mosaic” appearance at low power. The nests of cells were composed of basaloid cells with scant cytoplasm and hyperchromatic nuclei and paler cells at the center. Nests of epithelial cells were surrounded and penetrated by a thickened band of basement membrane material that was PAS-positive. Immunohistochemistry (IHC) staining with p63 showed positive staining on basaloid cells. Langerhans dendritic cells in the surrounding nests stained positive with CD1a. Solitary dermal cylindroma occurred sporadically in patients without family history of cutaneous cylindromas.Combination of morphologic, histochemystry and immunohistochemystry staining were needed for accurate diagnosis.

ABSTRAK

Silindromadermaladalah tumor jinak adneksa kulit yang jarang ditemukan dan dapat berupa lesi soliter maupun multipel. Kasus silindromadermaljarang ditemukan, akan tetapi diagnosis ini perlu dipertimbangkan dalam praktik klinis. Wanita berusia 51 tahun dengan keluhan nodul pada lengan kanan yang tidak nyeri. Pemeriksaan lesi kulit ditemukan dua buah nodulberukuran masing-masing 1x0,5x0,5cm, berwarna putih kecoklatan dan kenyal. Pemeriksaanhistopatologi menunjukkan tumor epitelial pada dermis yang berbatas tegas dan tidak berkapsul yang terdiri dari sarang-sarang tumor yang berbentuk oval dan poligonal yang membentuk gambaran jigsaw atau mosaic. Bagian tepi terdiri atas sel-sel basaloid dengan sitoplasma sedikit dan Keywords: inti hiperkromatis, sedangkan sel-sel pada bagian tengah tampak lebih pucat. Dermal cylindroma Sarang-sarang tumor dikelilingi dan dipenetrasi oleh membran basal yang solitary terpulas positif dengan PAS. Pengecatan IHC dengan p63 menunjukkan hasil PAS positif pada sel-sel basaloid. Sel-sel langerhans disekitar sarang-sarang tumor p63 terpulas positif dengan CD1a. Kombinasi pemeriksaan morfologi, histokimia CD 1a dan imunohistokimia diperlukan untuk diagnosis yang akurat.

*corresponding author: [email protected] 181 J Med Sci, Volume 51, Number 2, 2019 April: 181-187

INTRODUCTION an unsual case of solitary cylindroma arose in arm of a 51-year-old woman. Dermal cylindroma is a rare tumor inclinical practice, in whichthe diagnosis CASE is histological supprise because usually diagnosed as another skin lession.1It A 51-year-old woman experienced is a benign cutaneus tumor that have painless right arm nodule that historically been described as sweat accidentally found on lumpectomy gland tumors and have been considered for breast mass. Macroscopic to be an eccrine lineage.1 However, appearance was two fragmented recent study showed that cylindroma nodules, 1x0.5x0.5cm, tan to white, and is arising from pluripotent stem cells in rubbery. Histopathological findings the hair follicles.2 Dermal cylindroma revealed a well demarcated and an most commonly occur on the head and unencapsulatedepithelial dermal neck as solitary or multiple tumors. tumor, composed of numerous oval and Solitary cylindroma occur sporadically polygonal nests molded into a “jig-saw” and typically are not inherited. While or “mosaic” appearance at low power. multiple cylindromas are observed in Some with tubular formation. The nests an autosomal dominantly inherited of cells were composed predominantly manner.3 Multiple cylindromas are of basaloid cells with scant cytoplasm generally observed as a component and hyperchromatic nuclei. At the center of Brooke–Spiegler syndrome (BSS) of the nests, the cells had increased or as the only skin lesion of familial eosinophilic cytoplasm and vesicular cylindromatosis (FC).4 We here reporting nuclei (FIGURE1 and 2).

FIGURE 1. Solid, irregular cell nets with “jigsaw patterns”

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FIGURE 2. The nests surrounded and penetrated by eosinophylic hyalin membrane.

FIGURE 3. The hyalin membrane showed positive

FIGURE 4. The basaloid showed p63 nuclear staining

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FIGURE 5. Langerhans cells showed membrane and cytoplasmic staining with CD 1a.

Nests of epithelial cells are very different.4 surrounded and penetrated by a a Cylindroma has two clinical thickened band of basement membrane presentation, solitary and multiple material that was PAS-positive (FIGURE form. Solitary form occurr sporadically 3). Immunohistochemistry (IHC) staining in patients without family history of with p63 showed positive staining on cutaneous cylindromas. The solitary basaloid cells as shown in FIGURE. 4. form occurs as frequent as the multiple Langerhans cells in the surrounding nests form or even more frequently.10Variant stained positive with CD 1a (FIGURE. 5). with multiple cutaneous cylindromas are inherited in an autosomal dominant DISCUSSION manner. The observation of multiple cylindromas was first made by Brooke Dermal cylindromas are benign in 1892 and later by Spiegler in 1899.4 skin appendage tumors that are most Nowadays, the presence of multiple frequently found in scalp and neck cylindromas is known as the Brooke– skin, with predilection for middle aged Spiegler syndrome. The Brooke–Spiegler and elderly females.5 This neoplasm syndrome is classified by the presence is generally asymptomatic, but some of cylindromas, trichoepitheliomas, patients may experience pain.6 The and occasionally, spiradenomas, on the term cylindroma was first used in 1856 head and neck, beginning in the second by Billroth to describe an orbital tumor decade of life.11 with hyalin and glassy appearance.4,7 Multiple cylindromas may form a The term cylindroma originated from “turban tumors”. The term turban tumor its cylindrical shape seen in transversal is used to describe the localization of the section.8 Currently, three forms of tumor and it’s pathognomonic size that cylindromas are recognized: the can cover the whole scalp.12 Cylindromas benign dermal cylindroma, which may are characterized by irregularly shaped occur as solitary or multiple lesions; islands of basaloid cells arranged the malignant salivary cylindroma in a ‘‘jigsaw puzzle’’ pattern, which (adenoid cystic carcinoma); and the are surrounded by an eosinophilic malignant cylindroma.4,9 Although the hyaline sheath.13 The tumoral isles nomenclature was identical, however typically present two groups of cells: the clinical behavior and prognosis were peripheral cells in palisade with a

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small dark nucleus, which represent signs of malignant transformation are undifferentiated epithelial tumoral cells, ulceration, rapid enlargement, bleeding, and more differentiated centralized and change in the color of the nodules.9 cells, with a big pale nucleus resembling The characteristic histologic features of ductal or secreting cells.4,14 malignant cylindroma are loss of jigsaw The nests of cylindroma are pattern, loss of hyaline sheaths, loss commonly surrounded by a rim of of peripheral palisading at the tumor densely eosinophilic PAS-positive island periphery, loss of bimorphic cell basement membrane material, and composition, marked nuclear anaplasia, the nests are also punctuated by stromal invasion, focal areas of necrosis, small round droplets” with similar pleomorphism of nuclei, and increased staining qualities.14 This material was and abnormal mitoses.8,9 immunoreactive for collagen IV.15 Differential diagnosis dermal Diffuse positivity for p63 in the basaloid cylindroma werespiradenoma and cells and CK7-positivity in the larger adenoid cystic carcinoma. Cylindroma central cells support the morphological may show overlap or even occur together impression of two divergent types of with spiradenoma. Spiradenoma is a cellular differentiation; myoepithelial benign dermal neoplasm that can show and ductal. The extent of positive nuclear either eccrine or apocrine differentiation. staining for p63 supports predominant Spiradenoma classically presents in myoepithelial differentiation in this patients between the ages of 20 and 40 tumor.16 years and appears as a solitary, well- The histogenesis of cutaneous circumscribed, firm nodule, measuring cylindroma has remained a subject of usually less than 1 cm, but giant variants intense and controversial debate.2,13 and multiple lesions have also been There are controversies whether described. The most striking clinical its origin is in eccrine, apocrine or feature of spiradenoma is the presence follicular epithelium.3,4,13,14 The absence of pain or tenderness. Spiradenoma of cylindromas on the palms of hands is distinguished from cylindroma and soles of feet, skin with the highest by the lack of hyaline cylinders. density ofeccrine glands but without Furthermore,spiradenomas tend to be hair follicles, support the idea that more vascular and show more likely hair follicles are the cell of origin for edematous and cystic stromal changes.19 these tumours.17 Immunostaining Tumor cells with tubular differentiation study by Massoumi et al.13 in reassure and lymphocyte infiltration also that cylindroma likely originates from differentiated spiradenoma from dermal the epithelial hair follicle. Another cylindroma.14,20 Cylindroma also needs immunohistochemical study with CD200 to be distinguished from adenoid cystic and other stemcell markers has also carcinoma. The solid variant of adenoid indicated the hair follicle to be the origin cystic carcinoma differs from cylindroma of cylindroma.2 by its infiltrative growth pattern, Dermal cylindroma is usually cytologicatypia, mitotic figures, and the benign, but malignant transformation lack of a thickened basementmembrane of the multiple or solitary dermal around the epithelial structures.15 cylindroma have been reported. However, it is considered to be rare. The CONCLUSION malignant differentiation tendency is higher in the multiple type of cylindroma Solitary dermal cylindroma is than in the solid type.18 The clinical benign appendageal tumour that

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occurr sporadically in patients ojpathology.2016.63016 without family history of cutaneous 7. Ahued S. Cylindroma of the cylindromas. Combination of epiglottis. AMA Arch Otolaryngol morphologic, histochemistry and 1956; 63(4):366-71. immunohistochemistry staining are https://doi.org/10.1001/ needed for accurate diagnosis. archotol.1956.03830100024008 8. Batsakis JG, Brannon RB. Dermal ACKNOWLEDGEMENT analogue tumours of major salivary glands. J Laryngol Otol The authors are grateful to Mrs. 1981;95(2):155-64. Agustina for their excellent technical https://doi.org/10.1017/ assistance. S0022215100090551 9. Kuklani RM, Glavin FL, Bhattacharyya REFERENCES I. Malignant cylindroma of the scalp arising in a setting of multiple 1. Chaitanya Kumar Reddy P, cylindromatosis: a case report. Head Mohammed PH, Prashanth K, Tati SY, Neck Pathol 2009;3(4):315-9. Pai G, Chidananda KV, et al. Dermal https://doi.org/10.1007/s12105-009- cylindroma is a histological surprise: 0138-x a case report. IOSR J Dent Med Sci 10. Bansal C, Batra M, Lal N, Srivastava AN. 2014; 13(12):36-8. Solitary cylindroma with malignant https://doi.org/10.9790/0853- transformation. Indian J Dermatol 131263638 2012;57(2):141-3. 2. Sellheyer K. Spiradenoma and https://doi.org/10.4103/0019- cylindroma originate from the 5154.94289 hair follicle bulge and not from 11. Layegh P, Sharifi-Sistani N, Abadian the eccrine sweat gland: an M, Moghiman T. Brooke-Spiegler immunohistochemical study with syndrome. Indian J Dermatol CD200 and other stem cell markers. J Venereol Leprol 2008;74(6):632-4. Cutan Pathol 2015; 42(2):90-101. https://doi.org/10.4103/0378- https://doi.org/10.1111/cup.12406 6323.45109 3. Chauhan DS, Guruprasad Y. Dermal 12. Akgul GG, Yenidogan E, Dinc S, cylindroma of the scalp. Natl J Pak I, Colakoglu MK, Gulcelik MA. Maxillofac Surg 2012; 3(1):59-61. Malign cylindroma of the scalp https://doi.org/10.4103/0975- with multiple cervical lymph node 5950.102163 metastasis-A case report. Int J Surg 4. Jordão C, de Magalhães TC, Cuzzi Case Rep2013;4(7):589-92. T, Ramos-e-Silva M. Cylindroma: https://doi.org/10.1016/j. an update. Int J Dermatol 2015; ijscr.2013.02.027 54(3):275-8. 13. Massoumi R, Podda M, Fässler R, https://doi.org/10.1111/ijd.12600 Paus R. Cylindroma as tumor of hair 5. Patterson J. Weedon’s Skin Pathology, follicle origin. J Invest Dermatol 2nd edition. London: Churchill- 2006; 126(5):1182-4. Livingstone, 2002;890-1. https://doi.org/10.1038/sj.jid.5700218 6. Watanabe N, Kawase K, Tawada C, 14. McNiff J, McCalmont T, Requena L, Yoneda K, Hirai K, Niwa A, et al. A Sangueza O, Vassallo C, Rosso R, et al. case report of multiple cylindroma Benign tumours with apocrine and with immunohistochemical features. eccrine differentiation. WHO Pathol Open J Pathol 2016;6:134-9. Genet Ski Tumours 2006;144-5. https://doi.org/10.4236/ 15. Albores-Saavedra J, Heard SC,

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McLaren B, Kamino H, Witkiewicz tumour. Lancet Oncol 2015;16(9):460-9. AK. Cylindroma (dermal analog https://doi.org/10.1016/S1470- tumor) of the breast: a comparison 2045(15)00245-4 with cylindroma of the skin 18. Massoumi R. CYLD: a and adenoid cystic carcinoma deubiquitination enzyme with of the breast. Am J Clin Pathol multiple roles in cancer. Future 2005;123(6):866-73. Oncol 2011;7(2):285-97. https://doi.org/10.1309/CRWU-A3K0- https://doi.org/10.2217/fon.10.187 MPQH-QC4W 19. Busam KJ. Dermatophatology. 16. Mahmoud A, Hill DH, O’Sullivan Saunders: Elsevier 2010;11:409-10. MJ, Bennett MW. Cylindroma of the 20. Zheng Y, Tian Q, Wang J, Dong X, breast : a case report and review Jing H, Wang X, et al. Differential of the literature. Diagnostic Pathol diagnosis of eccrine spiradenoma: 2009; 4:30. a case report. Exp Ther Med 2014; https://doi.org/10.1186/1746-1596-4-30 8(4):1097-101. 17. Rajan N, Ashworth A. Inherited https://doi.org/10.3892/ cylindromas: lessons from a rare etm.2014.1906

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