|Year : 2016 | Volume
| Issue : 2 | Page : 96-98
Syringocystadenoma papilliferum: A rare case report with review of literature
Prasmit Amit Shah, Vikas Shyamraj Singh, Surekha Bhalekar, S Sudhamani, Ekta Paramjit
Department of Pathology, DY Patil University School of Medicine, Navi Mumbai, Maharashtra, India
|Date of Web Publication||18-May-2016|
Prasmit Amit Shah
Department of Pathology, DY Patil University School of Medicine, Nerul, Navi Mumbai - 400 706, Maharashtra
Source of Support: None, Conflict of Interest: None
Syringocystadenoma papilliferum also known as syringocystadenomatosus papilliferus is a benign adnexal skin tumor presenting as exuberant proliferating lesion of apocrine or eccrine type of differentiation. It is said to be common at birth or during puberty but cases in adults have also been reported. It typically develops as a nodular plaque lesion and is reportedly associated with nevus sebaceous in 40% of cases. Most the tumors are located in the scalp and face that present as multiple warty papules. We report a case of scalp swelling in a 19-year-old female who was first clinically diagnosed with keratoacanthoma but later histopathologically she was confirmed to have syringocystadenoma papilliferum.
Keywords: Adnexal tumor, nevus sebaceous, syringocystadenoma papilliferum
|How to cite this article:|
Shah PA, Singh VS, Bhalekar S, Sudhamani S, Paramjit E. Syringocystadenoma papilliferum: A rare case report with review of literature. J Sci Soc 2016;43:96-8
|How to cite this URL:|
Shah PA, Singh VS, Bhalekar S, Sudhamani S, Paramjit E. Syringocystadenoma papilliferum: A rare case report with review of literature. J Sci Soc [serial online] 2016 [cited 2020 Aug 9];43:96-8. Available from: http://www.jscisociety.com/text.asp?2016/43/2/96/182608
| Introduction|| |
Syringocystadenoma papilliferum is a rare benign hamartomatous adnexal tumor. Fifty percent of the cases are reportedly present at birth while 15-30% are present during puberty.  The tumor has varied clinical presentation. It presents as a hairless area on the scalp and is said to be associated with sebaceous nevus of Jadassohn.  Malignant change is very rare and is indicated by rapid increase of size and/or change in appearance of metastatic lymph nodes.  With increasing size, a prominent papillary configuration develops and the surface can become scabbed, which is typical of this tumor.  Histopathological examination is confirmatory and immunohistochemistry is required only for further differentiation between apocrine and eccrine tumors. 
| Case report|| |
A 19-year-old female presented with complaint of ulcerative lesion on the scalp since 6-7 months, gradually increasing in size. The patient also had complaints of bleeding on touch and bleeding on combing hair. The history of tenderness and itching was also present [Figure 1]. The clinical diagnosis of keratoacanthoma was made and the patient was advised fine-needle aspiration cytology (FNAC) of the lesion.
FNAC smears revealed plenty of keratinized and nonkeratinized squamous epithelial cells along with basaloid cells and acute inflammatory infiltrate. The squamous cells were devoid of nuclear atypia. The diagnosis of benign adnexal skin tumor was made [Figure 2].
|Figure 2: Papanicolaou-stained smear 4× showing keratinized and nonkeratinized squamous cells (red arrows) and inflammatory infi ltrate|
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The patient underwent excision of the lesion under local anesthesia. The lesion was excised completely with a normal 1 cm margin around the lesion and was sent for histopathological examination.
A gross examination showed two gray brown hairy skin covered soft tissue masses altogether measuring 3 cm × 3 cm, with areas of hemorrhage. The microscopy showed varying degrees of papillomatosis and several cystic invaginations extending downward from the epidermis lined by squamous keratinizing cells. The papillary projections and the lower portion of invagination were lined by glandular epithelium and consisting of two rows of cells that have oval nuclei and eosinophilic cytoplasm with some cells showing active decapitation secretion. Dense chronic inflammatory infiltrate was seen in the papillae. Based on these features, the diagnosis of syringocystadenoma papilliferum was made [Figure 3].
|Figure 3: H and E stained section 4× showing varying degrees of papillomatosis (red arrow) and cystic invaginations|
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The patient was followed up for 7 months and showed no evidence of recurrence.
| Discussion|| |
Syringocystadenoma papilliferum is rare nonmalignant adnexal sweat gland neoplasm characterized by asymptomatic, skin colored to pink papules, or plaques of highly variable appearance. Most common sites are head and neck region; however, tumor in other areas, such as vulva, external ear, lower leg, and scrotum, have also been reported.  In our case, the lesion was in the scalp.
It usually appears at birth or during infancy and around the time of puberty. In about one-third of cases, syringocystadenoma papilliferum is reportedly associated with nevus sebaceous. Multiple tumors of adnexal origin, such as trichoblastoma, apocrine adenoma, hidradenoma papilliferum, and trichilemmoma, are being reported to arise along with nevus sebaceous.  In this case, the tumor was neither present since birth nor associated with other tumors.
Three clinical types have been described:
- Plaque type: It presents as alopecic patch on the scalp that enlarges during puberty to become verrucous, nodular, or crusted plaques.
- Linear type: It consists of multiple reddish, firm papules, or umbilicated nodules 1-10 mm in size.
- Solitary nodular type: These are domed pedunculated nodules 5-10 mm in size and located near trunk shoulder and axilla. 
It reportedly evolves within three stages:
During the period of adult stage, variety of benign or malignant lesions can develop.  The malignant tumors reported in association with syringocystadenoma papilliferum are squamous cell carcinoma, basal cell carcinoma, and ductal carcinoma. Basal cell carcinoma is the most common symptom and have been reported in 10% of the cases while only two cases of squamous carcinoma have been reported. 
- Infantile stage: It appears as alopecic orange yellow plaque.
- Adolescent stage: Under androgenic influence the plaques undergoes various changes such as hyperkeratosis, hyperpigmentation, and sebaceous gland formation.
- Adult stage: It is characterized by the presence of large sebaceous glands, ectopic apocrine glands, and epidermal hyperplasia. 
Approximately, one-third of the cases are said to arise in precursor lesions such as organoid nevi. Yamamoto et al. reported the origin of pluripotent cells on immunohistochemistry and ultrastructural studies by investigating the immunohistological patterns of 12 different anti-cytokeratin (CK) antibodies, and several other markers in five cases of this tumor concluded that tumor epithelium was composed of several cell types demonstrating various stages from the primitive clear cells to the basal cells that support the concept that SCAP is a hamartomatous tumor that arises from pluripotent cells. Boni et al. reported mutation of PTCH and p16. 
Histopathology typically shows varying degrees of papillomatosis along with cystic invaginations and malformed sebaceous glands. Immunohistochemistry helps in differentiating the origin of the tumor, i.e. either eccrine or apocrine, but is of no clinical significance. Positive immunoreactivity for proteins 15 and 24 and zinc-2 glycoprotein demonstrates evidence of apocrine differentiation, while positivity for CKs demonstrates eccrine differentiation.  Immunohistochemistry was not done in the present case.
Smooth muscle actin (SMA) positivity is normally the feature of myoepithelial cells but syringocystadenoma papilliferum do not contain myoepithelial cells and hence the positivity indicates immaturity of the tumor. 
The only treatment for syringocystadenoma papilliferum is excision biopsy that also helps in confirming the diagnosis. Other modalities rarely used for treatment are CO 2 laser therapy while excision and grafting are unfavorable. 
| Conclusion|| |
Syringocystadenoma papilliferum is an uncommon sweat gland tumor with a widely variable clinical appearance. The presentation of tumor may generate multiple differential diagnosis, thus it must be histologically confirmed. Radiotherapy and other destructive procedures are ineffective and should be best avoided. Surgical excision with reconstruction is the treatment of choice.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Vyas SP, Kothari DC, Goyal VK. Syringocystadenoma papilliferum of scalp: A rare case report. IJSS 2015;2:182-5.
Sangma MM, Dasiah SD, Bhat VR. Syringocystadenoma papilliferum of the scalp in an adult male: A case report. J Clin Diagn Res 2013;7:742-3.
Godkhindi VM, Meshram DP, Deshpande SA, Suvernekar SV. Syringocystadenoma Papilliferum - Case report. IOSR JDMS 2013;5:43-6.
Xu D, Bi T, Lan H, Yu W, Wang W, Cao F, et al
. Syringocystadenoma papilliferum in the right lower abdomen: A case report and review of literature. Onco Targets Ther 2013;6:233-6.
Chauhan A, Gupta L, Gautam RK, Bhardwaj M, Gopichandani K. Linear syringocystadenoma papilliferum: A case report with review of literature. Indian J Dermatol 2013;58:409.
[Figure 1], [Figure 2], [Figure 3]