|Year : 2019 | Volume
| Issue : 2 | Page : 97-99
Syringofibroadenoma: Benign eccrine sweat gland tumor – A rare case report
Banyameen Iqbal, Prachi Khandekar, Indranil Dey, Harsh Kumar
Department of Pathology, Dr. D. Y. Patil Medical College, Hospital and Research Center, Pune, Maharashtra, India
|Date of Web Publication||28-Nov-2019|
Dr. Banyameen Iqbal
Department of Pathology, Dr. D.Y. Patil Medical College, Hospital and Research Centre, Pimpri, Pune, Maharashtra
Source of Support: None, Conflict of Interest: None
Eccrine syringofibroadenoma (ESFA) is a rare adnexal tumor. It was first described by Mascaro in 1963. They are predominantly seen on the extremities of middle aged to elderly patients. Clinically, ESFA is classified into five subtypes. Solitary ESFA, the most common subtype, usually presents as a skin-colored papule, plaque, or nodule. The histomorphological and immunohistochemical features are necessary for their diagnosis and are also required to differentiate them from their malignant counterpart, eccrine syringofibrocarcinoma.
Keywords: Benign sweat gland, eccrine sweat gland, syringofibroadenoma
|How to cite this article:|
Iqbal B, Khandekar P, Dey I, Kumar H. Syringofibroadenoma: Benign eccrine sweat gland tumor – A rare case report. Indian J Dermatopathol Diagn Dermatol 2019;6:97-9
|How to cite this URL:|
Iqbal B, Khandekar P, Dey I, Kumar H. Syringofibroadenoma: Benign eccrine sweat gland tumor – A rare case report. Indian J Dermatopathol Diagn Dermatol [serial online] 2019 [cited 2020 Jun 1];6:97-9. Available from: http://www.ijdpdd.com/text.asp?2019/6/2/97/271943
| Introduction|| |
Eccrine syringofibroadenoma (ESFA) is a rare adnexal tumor. It was first described by Mascaro in 1963. It is considered to arise from the excretory portion of eccrine sweat glands. The clinical appearance of ESFA varies considerably to include multiple papules, erythematous plaques, or a solitary nodule. They are predominantly seen on the extremities of middle aged to elderly patients. According to clinical presentation, ESFA is classified into five subtypes. The histopathological characteristics are typical and common to all the subtypes. The most common subtype is solitary ESFA. To illustrate a typical presentation, we report a case of solitary ESFA on the scalp of a middle-aged male.
| Case Report|| |
A 42-year-old male presented with a painful swelling over the scalp. On physical examination, the swelling was erythematous, tender, measuring 2.5 cm × 2 cm. There was no previous history of trauma to the scalp. Furthermore, there was no significant past or family history. The surgical department suspected it to be a dermoid cyst. An excisional biopsy of the lesion was performed, and the lesion was sent to pathology department for histopathological examination. On histopathology, the sections showed small ducts with narrow to slightly dilated lumen lined by a two-layered epithelium. Between these ducts, fibrovascular stroma was present [Figure 1], [Figure 2], [Figure 3]. Sections also showed focal areas of keratinization along with areas of hemorrhage [Figure 4]. No atypia or mitotic figures were seen. No neural invasion was seen. Immunohistochemical staining was done. Positive staining was seen for carcinoembryonic antigen and epithelial membrane antigen [Figure 5] and [Figure 6], which further substantiated our diagnosis.
|Figure 1: Photomicrograph showing anastomosing reticulated cords and strands of cells extending from the basal layer of epidermis into dermis [H and E, ×10]|
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|Figure 2: Photomicrograph showing small ducts with narrow to slightly dilated lumen and lined by a two-layered epithelium [H and E, ×40]|
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|Figure 3: Photomicrograph showing small ducts with comma-shaped extensions (also referred to “paisley tie” pattern or “tadpole” pattern) (H and E, ×100)|
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|Figure 4: Photomicrograph showing focal areas of keratinization (H and E, × 100)|
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|Figure 6: Photomicrograph showing epithelial membrane antigen positivity|
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| Discussion|| |
ESFA is a rare benign adnexal tumor with eccrine acrosyringeal differentiation. Starink classified ESFA into four clinical subtypes: (1) multiple ESFA associated with hidrotic ectodermal dysplasia, (2) multiple ESFA without associated cutaneous features, (3) unilateral linear ESFA, and (4) solitary ESFA, and French subsequently proposed the fifth subtype, reactive ESFA., Solitary ESFA, the most common subtype, usually presents as a skin-colored papule, plaque, or nodule on the extremity. Although the most common site is extremity, face, back, abdomen, buttocks, and rarely the nails can also be affected. Few other dermatoses closely resemble ESFA, making the clinical diagnosis difficult. Hence, histopathology and immunohistochemistry is necessary to confirm the diagnosis. Histologically, ESFAs show anastomosing cords of varied sizes composed of pale cuboidal epithelial cells extending from epidermis into dermis. The strands contain tubular structures that resemble eccrine ducts which are surrounded by fibrovascular stroma rich in acid mucopolysaccharides with lymphocytes and plasma cells. Histopathological differential diagnoses include eccrine poroma, acrosyringeal nevus, syringofibroadenocarcinoma, fibroepithelial tumor of Pinkus (variety of basal cell carcinoma [BCC]), pseudoepitheliomatous hyperplasia, squamous cell carcinoma, and reticulated seborrheic keratosis. Acrosyringeal nevus shows strong PAS positivity and plasma cell infiltrate. Eccrine poroma shows uniform epithelial cell proliferation with vertical thick strands of cells extending into dermis. Syringofibroadenocarcinoma shows area of transformation displaying cytological atypia. Fibroepithelial tumor of Pinkus shows focal changes typical of BCC with peripheral palisading and loose fibrous stroma. Complete surgical excision remains the mainstay of treatment. The clinical course of ESFA is typically benign. However, malignant transformation to eccrine syringofibrocarcinoma and the association with squamous cell carcinoma has been reported in cases of ESFA. Malignant changes have been associated with a gradual increase in size, pain, ulcer and crust formation, and persistent lesions despite extensive treatments. The risk is however low; therefore, close observation and follow-up may be an alternative, especially when complete excision is difficult due to involvement of large areas. Rare treatments with variable patient outcomes include cryotherapy, curettage, electrodessication, laser, and radiotherapy. Complications of ESFA includes the association with squamous cell carcinoma and malignant transformation into syringofibrocarcinoma and eccrine porocarcinoma.
In conclusion, solitary eccrine syringofibroadenoma is a nonhereditary solitary nodule or verrucous mass predominantly found on the extremities of the middle aged and elderly. Our case of solitary ESFA showed a rare presentation on the scalp of a middle aged male. The histomorphological and immunohistochemical features were consistent with the diagnosis of ESFA. Excisional biopsy was performed as a diagnostic as well as definitive treatment.
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.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Bottino CB, Guimarães TF, Gomes FR, D'Acri AM, Lima RB, Martins CJ. Solitary eccrine syringofibroadenoma – Case report. An Bras Dermatol 2015;90:235-8.
Tiwary AK, Jahan F, Mishra DK, Chaudhary SS. Reactive solitary eccrine syringofibroadenoma: A very rare adnexal tumour of skin. Am J Med Case Rep 2016;4:71-3.
Starink TM. Eccrine syringofibroadenoma: Multiple lesions representing a new cutaneous marker of the Schöpf syndrome, and solitary nonhereditary tumors. J Am Acad Dermatol 1997;36:569-76.
French LE. Reactive eccrine syringofibroadenoma: An emerging subtype. Dermatology 1997;195:309-10.
Arora P, Bansal S, Garg VK, Khurana N, Lal B. Solitary eccrine syringofibroadenoma with nail involvement: A rare entity. Indian J Dermatol 2015;60:103.
] [Full text]
Schadt CR, Boyd AS. Eccrine syringofibroadenoma with co-existent squamous cell carcinoma. J Cutan Pathol 2007;34 Suppl 1:71-4.
Cho E, Lee JD, Cho SH. A case of reactive eccrine syringofibroadenoma. Ann Dermatol 2011;23:70-2.
Temnithikul B, Jerasutus S, Sudtikoonaseth P, Voravutinon N, Kootiratrakarn T, Kattipathananpong P. Eccrine syringofibroadenoma (ESFA): A report of two cases. Dermatol Pract Concept 2016;6:5-8.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]