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  Table of Contents    
CASE REPORT  
Year : 2018  |  Volume : 61  |  Issue : 1  |  Page : 106-108
Syringocystadenoma papilliferum and trichoblastoma arising in the nevus sebaceous


1 School of Graduates, Beijing University of Chinese Medicine, Beijing, China
2 Department of Dermatology and Venerology, China-Japan Friendship Hospital, Beijing, China

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Date of Web Publication22-Mar-2018
 

   Abstract 


Nevus sebaceous (NS) is a clinically common benign tumor and has a high potential to develop into a great diversity of neoplasms of epidermal and adnexal origins. However, it is a rare phenomenon of the coexistence in a single NS with two or more skin tumors. We report a case of a 58-year-old woman with two kinds of neoplastic proliferation including syringocystadenoma papilliferum and trichoblastoma arising in NS on the scalp.

Keywords: Nevus sebaceous, syringocystadenoma papilliferum, trichoblastoma

How to cite this article:
Wang F, Wu Y, Zheng Z, Bai Y. Syringocystadenoma papilliferum and trichoblastoma arising in the nevus sebaceous. Indian J Pathol Microbiol 2018;61:106-8

How to cite this URL:
Wang F, Wu Y, Zheng Z, Bai Y. Syringocystadenoma papilliferum and trichoblastoma arising in the nevus sebaceous. Indian J Pathol Microbiol [serial online] 2018 [cited 2019 Dec 13];61:106-8. Available from: http://www.ijpmonline.org/text.asp?2018/61/1/106/228183





   Introduction Top


Nevus sebaceous (NS) is a benign congenital hamartoma that was originally described in 1985 by Jadassohn. NS has the potential to differentiate into epidermal, adnexal, mesenchymal cell tumors,[1] presenting as a yellowish, waxy, hairless plaque on the scalp, face, and neck. It occurs at birth or shortly after birth. Clinically, 25% NS patients have one or more secondary benign and malignant tumors. The most common secondary benign tumor arising from NS is syringocystadenoma papilliferum (SCAP) and trichoblastoma (TB), but it is extremely rare for both of them to occur simultaneously.[2],[3] Herein, we report a case of the simultaneous occurrence of SCAP and TB with NS which has never been reported in China and only reported seven cases in PubMed.


   Case Report Top


A 58-year-old healthy woman reported a 2-year history of itching and bleeding after scratching in a yellowish lesion that had been present on her scalp since birth. The patient had no previous history of cutaneous or internal malignancy, and the complete system review was negative. There was no remarkable medical or familial history. Physical examination of the lesion on scalp revealed a 1 cm × 2 cm yellowish, verrucous plaque with ulceration on its surface [Figure 1]. Complete excision of the entire plaque was performed and submitted for histopathological examination.
Figure 1: Pink-red, yellowish, verrucous plaque on the top of scalp, about 2 cm × 1.5 cm, with irregular border and local mild erosion and crust on the surface

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Histopathological findings of the lesion by hematoxylin and eosin staining showed that the cysts were formed by the invaginations of epidermis with papillary structures covered by a double layer of epithelium constituted by an external layer of cuboid cells with round nuclei and scarce cytoplasm and an internal layer of cylindrical cells with ovoid nuclei and weak eosinophilic cytoplasm with decapitation secretion. The papillary structures were plasmocyte-rich inflammatory infiltrate [Figure 2]a. Multiple well-circumscribed tumor lobules were constituted of basaloid cells with round or ovoid nuclei and basophilic cytoplasm in the dermis and showed a round inferior border with peripheral palisading of neoplastic cells. Some cells presented vacuolization without atypia and rare mitotic figure of the tumor. Dense stroma was present in the surrounding tumor lobules. No artifactual retraction spaces closed to tumor lobules [Figure 2]b and [Figure 2]c.
Figure 2: (a) An invagination of the epidermis with papillary structures and multiple well-circumscribed tumor lobules in the upper dermis (H and E, ×40). (b) The papillary structures constituted by an external layer of cuboid cells with round nuclei and scarce cytoplasm and an internal layer of cylindrical cells with ovoid nuclei and decapitation secretion and plasmocyte-rich inflammatory infiltrate (H and E, ×400). (c) Tumor lobules were constituted by basaloid cells with a round inferior border with peripheral palisading. Dense stroma was present in the surrounding tumor lobules (H and E, ×400)

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Immunohistochemistry showed that the lesion was stained positive for CK14, partial positive for BerEP4 and CK19, partial weakly positive for Bcl2, negative for CD34, and 15% positive in general and 80% focally positive for Ki67 [Figure 3]. Combined with the examination of histopathological and immunohistochemistry, a diagnosis of NS with SCAP and TB was made.
Figure 3: The lesion showed positive for CK14, partial positive for BerEP4 and CK19, partially weak positive for Bcl-2, negative for CD34, and 15% positive in general and 80% focally positive for Ki67 (CK14, BerEP4, CK19, Bcl-2, CD34, Ki67, ×40)

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   Discussion Top


NS, also known as organoid nevus, is reported (with an) incidence of 0.5%–1% of all dermatologic patients.[4] Initially, NS always has immature hair germ structures which can differentiate into mature sebaceous gland or rarely differentiate into adnexal tumors due to pubertal hormones. Twenty-five percent of the NS patients will develop a wide variety benign or malignant skin neoplasms on the basis of the lesion; SCAP and TB were the most frequent two benign tumors. Basal cell carcinoma (BCC) was the most frequent malignant tumor; other malignant tumors which were less frequently seen include squamous cell carcinoma, sebaceous carcinoma, apocrine carcinoma, and microcystic adnexal carcinoma.[2],[3] Previous studies showed that the formation of the secondary tumors arising from NS may due to the mutation of HRAS/KRAS which influence the development of tumors by regulating the MAPK and P13pAkt pathway.[5],[6]

SCAP is an uncommon benign hamartomatous adnexal neoplasm derived from apocrine and eccrine glands. SCAP has a varied clinical appearance, often present as single or multiple patches and nodules with vesicle or exudation sometimes. Linear papules are occasionally present. Clinically, malignant changes of SCAP are extremely rare, but we should give consideration to that problem when it bursts or enlarges suddenly. There are also reported cases of squamous cell carcinoma and syringocystadenocarcinoma papilliferum as a progression of SCAP, and CO2 laser was recommended to remove the tumors at early stage.[7]

TB belongs to trichogenic tumor. TB is constituted largely of follicular germinative cells and has the common embryonic origin with hair follicle, sebaceous gland, and apocrine gland. Sometimes, TB is difficult to differentiate histopathologically from nodular BCC. Formation of papillary mesenchymal bodies in the dermis, no epidermal connection, and no artifactual retraction spaces between the tumor and stroma can be used to diagnose TB and exclude BCC. And also, immunohistochemistry may sometimes helpful to differentiate the two.[8] Pitarch and Botella-Estrada [9] used dermoscopy revealed that the branching telangiectases were wider in caliber and tended to branch more in nodular BCC compared with TB and other benign cutaneous tumors. Hence, dermoscopy may be used as an effective and noninvasion method to differentiate TB and BCC.

NS frequently accompany with benign and malignant tumors, and it is difficult to identify them only by clinical feature, so it may be significant to do prophylactic complete excision or local photodynamic therapy at early stage of NS.[8] Otherwise, close clinical follow-up is necessary.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Premalata CS, Kumar RV, Malathi M, Shenoy AM, Nanjundappa N. Cutaneous leiomyosarcoma, trichoblastoma, and syringocystadenoma papilliferum arising from nevus sebaceus. Int J Dermatol 2007;46:306-8.  Back to cited text no. 1
    
2.
Idriss MH, Elston DM. Secondary neoplasms associated with nevus sebaceus of Jadassohn: A study of 707 cases. J Am Acad Dermatol 2014;70:332-7.  Back to cited text no. 2
    
3.
Hsu MC, Liau JY, Hong JL, Cheng Y, Liao YH, Chen JS, et al. Secondary neoplasms arising from nevus sebaceus: A retrospective study of 450 cases in Taiwan. J Dermatol 2016;43:175-80.  Back to cited text no. 3
    
4.
Kamyab-Hesari K, Seirafi H, Jahan S, Aghazadeh N, Hejazi P, Azizpour A, et al. Nevus sebaceus: A clinicopathological study of 168 cases and review of the literature. Int J Dermatol 2016;55:193-200.  Back to cited text no. 4
    
5.
Aslam A, Salam A, Griffiths CE, McGrath JA. Naevus sebaceus: A mosaic RASopathy. Clin Exp Dermatol 2014;39:1-6.  Back to cited text no. 5
    
6.
Dore E, Noe MH, Swick BL. Trichoblastoma, syringocystadenoma papilliferum, desmoplastic trichilemmoma and tumor of the follicular infundibulum with signet-ring cells, all arising in nevus sebaceus. J Cutan Pathol 2015;42:645-51.  Back to cited text no. 6
    
7.
Nascimento BA, Carneiro CM, Carvalho AH, Bittencourt Mde J, Drago MG, Freitas LK, et al. Syringocystadenoma papilliferum in an unusual location. An Bras Dermatol 2015;90:900-2.  Back to cited text no. 7
    
8.
Gozel S, Donmez M, Akdur NC, Yikilkan H. Development of six tumors in a sebaceus nevus of Jadassohn: Report of a case. Korean J Pathol 2013;47:569-74.  Back to cited text no. 8
    
9.
Pitarch G, Botella-Estrada R. Dermoscopic findings in trichoblastoma. Actas Dermosifiliogr 2015;106:e45-8.  Back to cited text no. 9
    

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Correspondence Address:
Yanping Bai
No. 2, Yinghua East Street, Chaoyang District, Beijing
China
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_613_16

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    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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