[Downloded free from http://www.mhsr.org] Originl Article Adnexl Tumors of Skin: An Experience t Tertiry Cre Center t Delhi Pujni M, Mdn GB 1, Jirjpuri ZS 2, Jetley S 2, Hssn MJ 2, Khn S 2 Deprtment of Pthology, ESIC Medicl College, Fridd, Hryn, 1 Deprtment of Pthology, Dr. RML Hospitl, 2 Deprtment of Pthology, Hmdrd Institute of Medicl Sciences nd Reserch, New Delhi, Indi Address for correspondence: Dr. Jetley S, Deprtment of Pthology, Hmdrd Institute of Medicl Sciences nd Reserch, New Delhi, Indi. E mil: drjetley2013@gmil.com Astrct Bckground: Adnexl skin tumors re heterogeneous group of uncommon tumors usully misdignosed cliniclly due to huge vriety of types nd their vrints. Histopthology usully helps in estlishing the dignosis. Aims: The study ws undertken to nlyze the morphologicl, clinicl, nd histologicl fetures of dnexl tumors (ATs) of the skin t our center over period of 4 yers. Sujects nd Methods: retrospective study ws conducted over period of 4 yers (April 2010 Mrch 2014), comprising 25 ATs of skin dignosed in the Deprtment of Pthology, Hmdrd Institute of Medicl Sciences nd Reserch, Jmi Hmdrd, New Delhi. All the consecutively reported AT cses were reviewed nd reclssified s AT rising from seceous glnds, hir follicles, or swet glnds. The concordnce of clinicl nd histopthologicl dignosis ws lso ssessed. Results: Most of the ATs were enign (24/25) with hed nd neck eing the most common loction (72%). Nerly 56% of the tumors exhiited swet glnd differentition, 28% hir follicle differentition, nd seceous glnd tumors ccounted for 16%. The most common vrieties of tumors encountered in the present study included hidrdenom ppilliferum nd pilomtricom. The concordnce etween clinicl nd histopthologicl dignosis ws found to e 50% pproximtely. Conclusions: ATs of the skin re rre neoplsms with enign tumors eing fr more common. They re often misdignosed cliniclly, so histopthology remins the gold stndrd for estlishing n ccurte dignosis of skin ATs. Keywords: Adnexl tumors, hir follicle differentition, seceous, swet glnd Introduction Adnexl tumors (ATs) re the tumors rising from the ppendges of the skin such s swet glnds, seceous glnds, nd hir follicles. Adnexl skin tumors re usully misdignosed cliniclly, nd histopthology usully provides dignostic confirmtion. ATs re heterogeneous group of skin tumors with mostly enign ehvior. These re usully found s solitry, spordic lesions; however, certin specific types of multiple tumors mye n indiction of some complex genetic syndromes, for exmple, Cowden s syndrome nd Muir Torre syndrome. [1,2] Occsionlly, significnt ssocition hs een oserved with certin internl mlignncies. [3] Access this rticle online Quick Response Code: Wesite: www.mhsr.org DOI: 10.4103/mhsr.mhsr_339_14 This study ws undertken to nlyze the morphologicl, clinicl, nd histologicl fetures of ATs of the skin t our center over period of 4 yers. Sujects nd Methods The present study ws retrospective study over period of 4 yers (April 2010 Mrch 2014), including ll the ATs of the skin dignosed in the Deprtment of Pthology t Hmdrd Institute of Medicl Sciences nd Reserch, Jmi Hmdrd, New Delhi. All the consecutively histopthologiclly dignosed ATs of the This is n open ccess rticle distriuted under the terms of the Cretive Commons Attriution NonCommercil ShreAlike 3.0 License, which llows others to remix, twek, nd uild upon the work non commercilly, s long s the uthor is credited nd the new cretions re licensed under the identicl terms. For reprints contct: reprints@medknow.com How to cite this rticle: Pujni M, Mdn GB, Jirjpuri ZS, Jetley S, Hssn MJ, Khn S. Adnexl tumors of skin: An experience t tertiry cre center t Delhi. Ann Med Helth Sci Res 2016;6:280-5. 280 2017 Annls of Medicl nd Helth Sciences Reserch
[Downloded free from http://www.mhsr.org] skin, irrespective of their clinicl dignoses, were included in the study. Ptients clinicl detils such s ge, sex, provisionl clinicl dignosis, size, nd loction were documented. The excised specimens were sujected to gross nd microscopic exmintion using hemtoxylin nd eosin stin. A few cses were sujected to periodic cid Schiff (PAS) stin nd lcin lue stin s nd when required for estlishing correct dignosis. All the slides were reviewed nd reclssified s AT rising from seceous glnds, hir follicles, or swet glnds. The concordnce of clinicl nd histopthologicl dignosis ws lso ssessed. If clinicl suspicion of AT ws kept efore iopsy, such cses were considered concordnt. Results In the present study, over period of 4 yers, totl of 25 ATs of the skin were dignosed. Benign ATs constituted the mjority of tumors with 24 out of 25 cses (96%) while only one cse of mlignnt AT ws encountered. The swet glnd tumors ccounted for the lrgest numer of cses 56% (14/25), followed y hir follicle tumors (28%) nd seceous glnd tumors (16%) [Figure 1]. The mle to femle rtio ws pproximtely equl (0.92:1). The ge of the ptients rnged from 8 yers to 58 yers, with medin ge of 32 yers. The highest incidence of ATs ws encountered in 21 40 yers ge group (56%). The hed nd neck region ws the most frequently involved loction for ATs of the skin ccounting for 72% of the cses followed y extremities (20%) nd trunk (8%). Cliniclly, these ptients were suspected to hve cysts, soft tissue tumors, or ATs. In 48% of ptients (12/25), ATs were cliniclly suspected y the clinicin efore excision, while in 52% of ptients, ATs were dignosed only on histopthology. The tumor size rnged from 0.5 cm to 5 cm in mximum dimension, with 80% of the tumors eing <2.5 cm in dimeter. The clinicopthologicl chrcteristics of the study group re shown in Tle 1. The most common vrieties of tumors encountered in the present study included hidrdenom ppilliferum nd pilomtricom with four cses ech. The frequency distriution of the vrious dnexl skin tumors is shown in Tle 2. Tle 1: Clinicopthologicl chrcteristics of the study group Chrcteristics Totl (n=25), n (%) Age (yers) 0-10 1 (4) 11-20 3 (12) 21-30 7 (28) 31-40 7 (28) 41-50 5 (20) >50 2 (8) Sex Mles 12 (48) Femles 13 (52) Loction Hed nd neck 18 (72) Extremities 5 (20) Trunk 2 (8) Size (mximum dimension in cm) <1.5 13 (52) 1.6-2.5 7 (28) 2.6-4.0 4 (16) >4.1 1 (4) Differentition Seceous 4 (16) Swet 14 (56) Hir follicle 7 (28) Behvior Benign 24 (96) Mlignnt 1 (4) 7 14 4 Seceous Swet Hir follicle Figure 1: Pie chrt showing distriution of the cses ccording to line of differentition (n = 25) Tle 2: The frequency distriution of the vrious dnexl skin tumors Tumor Ctegory n (%) Hidrdenom ppilliferum Swet glnd 4 (16) Pilomtricom Hir follicle 4 (16) Apocrine hidrdenom Swet glnd 2 (8) Proliferting trichilemml tumor Hir follicle 2 (8) Cylindrom Swet glnd 2 (8) Chondroid syringom Swet glnd 2 (8) Nevus seceous Seceous glnd 2 (8) Eccrine porom Swet glnd 1 (4) Syringom Swet glnd 1 (4) Seceous hyperplsi Seceous glnd 1 (4) Seceous crcinom Seceous glnd 1 (4) Trichoepitheliom Hir follicle 1 (4) Nodulr hidrdenom Swet glnd 1 (4) Syringocystdenom ppilliferum Swet glnd 1 (4) Annls of Medicl nd Helth Sciences Reserch Volume 6 Issue 5 Septemer-Octoer 2016 281
[Downloded free from http://www.mhsr.org] Discussion The enign tumors of epiderml ppendges cn e clssified into four groups depending on the line of differentition into tumors with differentition towrd hir follicles, towrd seceous glnds, towrd pocrine glnds, nd towrd eccrine glnds. The mlignnt ATs re of three mjor types: crcinom of seceous glnds, of eccrine glnds, nd of pocrine glnds. Occsionl cses of mlignnt tumors of hir follicle differentition hve lso een reported such s pilomtricl crcinom, mlignnt proliferting trichilemml cyst, nd trichilemml crcinom. [1,2] The incidence of enign ATs outnumers the mlignnt ones in most of the studies in literture. [4 17] In the present study lso, 96% (24/25) of the tumors were enign with only single mlignnt tumor. The dignosis of ATs poses gret dignostic difficulties owing to vriety of resons, nmely, the enormous types of tumor with their vrints, the occurrence of multiple lines of differentition in single tumor s well s the complicted nomenclture. ATs originte from multipotentil undifferentited stem cells, rther thn mture cells, which hve the cpility to differentite long prticulr pthwys, mye multiple. [1,2] Among the ATs, the most frequent line of differentition encountered ws swet glnd differentition (56%), followed y hir follicle differentition (28%) nd the lest frequent eing seceous glnd differentition (16%). These oservtions re in concordnce with tht of Rdhik et l., [14] Shrm et l., [17] Pntol et l., [15] nd Gythri et l. [13] On the contrry, Kmy Hesri et l. [16] found seceous tumors to e the most common type which is unlike most of the studies in literture. A comprtive nlysis of mny studies from Indin literture hs een shown in Tle 3 with regrd to the line of differentition of ATs. The mle to femle rtio of the ptients ws found to e pproximtely equl (0.92:1) in our study group, which is comprle to tht of Shrm et l., [17] Kmy Hesri et l., [16] nd Jindl nd Ptel [12] Severl other reserchers otined vrile results, nmely, Nir et l. [9] found mle to femle rtio of 1:2.3 while Pntol et l. [15] found the rtio of 1.8:1. In the Indin popultion, the overll incidence of skin ATs is very low s documented y severl studies. [9,11 15,17] The loction of ATs vries with the histologic type. By fr, hed nd neck region is the most common loction where ATs re frequently encountered, the other sites eing xill, trunk, legs, etc. In the present study, in 72% of the cses, the site of predilection ws hed nd neck followed y extremities (20%) nd trunk (8%). The predominnce of ATs in the hed nd neck region is well documented fct supported y most of the series in literture. This is ccounted for y the fct tht this region is rich in piloseceous units, pocrine s well s eccrine glnds, therey providing fertile environment for the development of ATs. [18] Most of the ATs in our study (52%) were <1.5 cm in mximum dimensions. Very rrely, the size of these tumors exceeds 4 cm in dimeter. Jindl nd Ptel [12] lso oserved tht 76% of tumors in their study group were <2 cm in size. Hidrdenom ppilliferum ws the most commonly encountered tumor in our series with totl of four cses. It is usully found in women in li mjor or in perinel or perinl region. We cme cross n interesting cse of cystic swelling in the right li minor in which the clinicl impression ws Brtholin s cyst. It is well circumscried tumor locted in dermis composed of tuulr nd cystic structures with ppillry folds projecting into cystic spces [Figure 2]. We lso cme cross four cses of pilomtricom or clcifying epitheliom of Mlhere. It is tumor with differentition towrd hir follicles, usully seen s solitry lesion in the fce nd upper extremities. Microscopiclly, islnds of epithelil cells of two types re seen: sophilic cells nd shdow cells [Figure 3]. [1,2] Cylindrom is tumor where the differentition in most cses is towrd pocrine structures ut rrely towrd eccrine structures. The tumor is composed of vrily shped islnds of tumor Tle 3: A comprtive nlysis of Indin literture with regrd to the line of differentition of dnexl tumors Study Yer Numer of cses (n) Seceous differentition, n (%) Swet glnd differentition, n (%) Hir follicle differentition, n (%) Vishnv nd Dhrkr [4] 1974 48 3 (6.3) 38 (79.2) 6 (12.5) Krth et l. [5] 1980 82 3 (3.66) 45 (54.88) 35 (42.7) Reddy et l. [6] 1982 85 18 (21.18) 54 (63.53) 13 (15.3) Nir [9] 2008 33 2 (6.06) 19 (58.1) 12 (36.36) Sh et l. [11] 2011 23 3 (13) 13 (57) 7 (30) Jindl nd Ptel [12] 2012 25 1 (4) 13 (52) 11 (44) Gythri et l. [13] 2012 29 3 (10.3) 15 (51.72) 11 (37.93) Rdhik et l. [14] 2013 35 7 (20) 17 (48.57) 11 (31.43) Pntol et l. [15] 2013 70 4 (5.7) 42 (60) 24 (34.3) Shrm et l. [17] 2014 56 12 (21.43) 24 (42.86) 20 (35.71) Present study 25 4 (16) 14 (56) 7 (28) 282 Annls of Medicl nd Helth Sciences Reserch Volume 6 Issue 5 Septemer-Octoer 2016
[Downloded free from http://www.mhsr.org] cells rrnged like jigsw puzzle. The islnds re surrounded y hyline sheth nd occsionl islnds lso show droplets of hyline mteril [Figure 4]. PAS stin ws done to highlight the eosinophilic sement memrne mteril surrounding the nests of cells in the cses encountered in this study. Chondroid syringom, lso known s enign mixed tumor of the skin, is n uncommon dnexl neoplsm tht usully occurs on the hed nd neck s in the present series. Tuulr or ductl structures of either eccrine or pocrine type re loosely emedded in myxoid chondroid mtrix in the mid to deep dermis. The strom ws intensely positive for lcin lue stin in one of the cses. Syringom represents n denom of intrepiderml eccrine ducts. These re usully multiple, smll skin colored ppules locted usully on the lower eyelids, cheeks, xille, etc. The tumor is composed of Figure 2: Photomicrogrph of hidrdenom ppilliferum showing () tumor composed of tuulr nd cystic structures with ppillry folds projecting into cystic spces (H nd E, 100) () these re lined y two lyers of cells with picl secretions (H nd E, 400) Figure 4: Photomicrogrph of cylindrom showing () tumor composed of vrily shped islnds of tumor cells rrnged like jigsw puzzle (H nd E, 100), () The islnds re surrounded y hyline sheth nd occsionl islnds lso show droplets of hyline mteril (rrow) (H nd E, 400) numerous smll ducts lined y two lyers of epithelil cells. Some of the ducts hve comm like tils, giving tdpole like ppernce [Figure 5].[1] Proliferting trichilemml tumor is lmost lwys locted on the sclp s solitry lesion. Microscopiclly, it is well circumscried tumor comprising vrily sized loules of squmous epithelium showing rupt kertiniztion. At plces, horn perls re lso seen [Figure 6]. The only mlignnt tumor encountered in our series ws seceous crcinom. It occurs most frequently on the eyelids s seen in our cse. The tumor is composed of irregulr loulr formtions composed of seceous cells s well s undifferentited cells with moderte to mrked nucler pleomorphism [Figure 7].[1] Figure 3: Pilomtricom () Grossly, it is cpsulted tumor with gryish white cut surfce () Photomicrogrph showing sloid s well s shdow cells (white nd lck rrow) long with foreign ody gint cell rection (lck rrow) (H nd E, 400) Figure 5: Photomicrogrph of () chondroid syringom showing tuulr nd ductl structures emedded in chondromyxoid strom (rrow) (H nd E, 100) () Syringom composed of numerous smll ducts with comm like tils, giving tdpole like ppernce (rrows) (H nd E, 100) Annls of Medicl nd Helth Sciences Reserch Volume 6 Issue 5 Septemer-Octoer 2016 283
[Downloded free from http://www.mhsr.org] Figure 6: Photomicrogrph of proliferting trichilemml tumor showing vrily sized loules of squmous epithelium showing rupt kertiniztion (rrow with lck order), horn perls re lso seen (lck rrows with white order) (H nd E, 400) The dignosis of ATs is not suspected cliniclly in most of the cses. ATs of the skin present either s symptomtic ppules or nodules, which re suspected to e either kertinous cysts or soft tissue nodules. Hence, the discrepncy etween clinicl nd histopthologicl dignosis is often encountered. However, the loction, single/multiple lesions, nd their distriution cn provide importnt dignostic clues. In the present study, the concordnce etween clinicl nd histopthologicl dignosis ws found to e 50% pproximtely. Kmy Hesri et l.[16] found slightly higher (64%) concordnce etween clinicl nd histopthologicl dignoses. This further stresses upon the fct tht histopthology still remins the gold stndrd for the dignosis of ATs s the clinicl presenttion is quite indistinctive. Cytochemicl stins such s PAS, mucicrmine, lcin lue, nd reticulin my id in estlishing the dignosis. PAS stin highlights the chrcteristic eosinophilic gloules in spirdenom s well s the sement memrne mteril surrounding the nests of tumor cells nd smll round droplets in cse of cylindrom. Alcin lue stin helps in demonstrting the intense stining of the strom in cse of chondroid syringom.[1,2] Immunohistochemistry (IHC) hs little, if ny, role in differentiting etween the vrious types of ATs. Crcinoemryonic ntigen positivity indictes ductl differentition, cytokertin (CK) indictes folliculr differentition while seceous differentition is suggested y positive epithelil memrne ntigen. IHC my ply n importnt role in distinguishing primry cutneous dnexl crcinom from metsttic crcinom. P63 nd CK 5/6 positivity fvors primry cutneous dnexl crcinom over metsttic crcinom.[1,2] Figure 7: Photomicrogrph of () nevus seceous showing seceous glnd hyperplsi (H nd E, 100) () Seceous crcinom of irregulr loulr formtions composed of seceous cells (cler cells mrked y rrow) s well s undifferentited cells with moderte to mrked nucler pleomorphism (H nd E, 400) histopthology remins the gold stndrd for estlishing n ccurte dignosis of skin ATs. Finncil support nd sponsorship Nil. Conflicts of interest There re no conflicts of interest. References 1. Klein W, Chn E, Seykor JT. Tumors of the epiderml ppendges. In: Elder DE, Elenitss R, Johnson BL Jr., Murphy GF, editors. Lever s Histopthology of the Skin. 9th ed. Phildelphi: Lippincott Willims nd Wilkins; 2005. p. 867 926. 2. Snt Cruz DJ. Tumors of the skin. In: Fletcher CD, editor. Dignostic Histopthology of Tumors. 3 rd ed., Vol. 2. Phildelphi: Churchill Living Stone Elsevier; 2007. p. 1423 526. 3. Knitkis J. Adnexl tumours of the skin s mrkers of cncer prone syndromes. J Eur Acd Dermtol Venereol 2010;24:379 87. 4. Vishnv VP, Dhrkr DD. Adnexl tumours of skin. Indin J Pthol Bcteriol 1974;17:33 8. 5. Krth CC, Shnkr SK, Bhuyn UN. Benign mixed tumour of skin A histopthologic study of 7 cses. Indin J Pthol Microiol 1980;23:1 6. 6. Reddy MK, Velith AJ, Ngrjn S, Auror AL. A clinicopthologicl study of dnexl tumours of skin. Indin J Med Res 1982;75:882 9. 7. Wong TY, Suster S, Cheek RF, Mihm MC Jr. Benign cutneous dnexl tumors with comined folliculoseceous, pocrine nd eccrine differentition: Clinicopthologicl nd immunohistochemicl study of eight cses. Am J Dermtopthol 1996;18:124 8. 8. Yqoo N, Ahmd Z, Muzffr S, Gill MS, Soomro IN, Conclusions ATs of the skin re rre neoplsms with enign tumors eing fr more common. They re often misdignosed cliniclly, so 284 Annls of Medicl nd Helth Sciences Reserch Volume 6 Issue 5 Septemer-Octoer 2016
[Downloded free from http://www.mhsr.org] Hsn SH. Spectrum of cutneous ppendge tumors t Ag Khn University Hospitl. J Pk Med Assoc 2003;53:427 31. 9. Nir PS. A clinicopthologic study of skin ppendgel tumors. Indin J Dermtol Venereol Leprol 2008;74:550. 10. Smil MO. Adnexl skin tumors in Zri, Nigeri. Ann Afr Med 2008;7:6 10. 11. Sh A, Ds NK, Ghrmi RC, Chowdhury SN, Dtt PK. A clinico histopthologicl study of ppendgel skin tumors, ffecting hed nd neck region in ptients ttending the dermtology opd of tertiry cre centre in Estern Indi. Indin J Dermtol 2011;56:33 6. 12. Jindl U, Ptel R. Study of dnexl tumors of the skin: A three yer study of 25 cses. Int J Pthol 2012;13:3. 13. Gythri SS, Alvndr E, Kumr SA. An nlysis of skin ppendgel tumors in South Indi. J Evol Med Dent Sci 2012;1:907 10. 14. Rdhik K, Phneendr BV, Rukmngdh N, Reddy MK. A study of iopsy confirmed skin dnexl tumours: Experience t tertiry cre teching hospitl. J Clin Sci Res 2013;2:132 8. 15. Pntol C, Kl S, Agrwl A, Amit S, Pntol S. Cutneous dnexl tumours: A clinicopthologicl descriptive study of 70 cses. World J Pthol 2013;2:77 82. 16. Kmy Hesri K, Blighi K, Afshr N, Aghzdeh N, Rhr Z, Serj M, et l. Clinicopthologicl study of 1016 consecutive dnexl skin tumors. Act Med Irn 2013;51:879 85. 17. Shrm A, Prichrk DG, Nigm JS, Rewri S, Soni PB, Omhre A, et l. Histopthologicl study of skin dnexl tumours Institutionl study in South Indi. J Skin Cncer 2014;4:543756. 18. Burns T, Brethnch S, Cox N, Griffiths C. Tumors of the skin ppendges. In: Mckie RM, Clonje E, editors. Rook s Textook of Dermtology. 7 th ed. London: Blckwell Pulishing Compny; 2004. p. 1 34. Annls of Medicl nd Helth Sciences Reserch Volume 6 Issue 5 Septemer-Octoer 2016 285