Skin nonmelanocytic tumor
Adnexal tumors
Sweat gland derived (apocrine & eccrine glands)
Eccrine syringofibroadenoma
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Last staff update: 6 November 2020
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PubMed Search: Eccrine syringofibroadenoma [title]
Table of Contents
Definition / general | Terminology | Epidemiology | Sites | Etiology | Pathophysiology | Clinical features | Case reports | Treatment | Gross description | Clinical images | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Electron microscopy description | Differential diagnosisCite this page: Sriharan A, Shalin SC. Eccrine syringofibroadenoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skintumornonmelanocyticacrosyringealadenomatosis.html. Accessed June 8th, 2023.
Definition / general
- A rare benign eccrine proliferation with anastomosing cords in a fibrovascular stroma
- Usually presents as a single lesion but may present as multiple nodules
Terminology
- Also called eccrine syringofibroadenoma (of Mascaro), acrosyringeal adenomatosis
- Other names include:
- Eccrine poromatosis
- Linear eccrine poroma
- Acrosyringeal nevus of Weedon and Lewis
- Nevus syringoadenomatosus papilliferum
Epidemiology
- Rare; only 75 cases reported
- Wide age range (16 - 80), most cases present in 6th to 7th decade, but cases associated with Schöpf-Schulz-Passarge syndrome usually present in adolescents
- No clear racial or gender predilection
Sites
- Predilection for extremities, especially acral sites
- Usually presents as a slow growing, solitary, flesh colored nodule
- Gradual and symmetric spread of papular lesions over large areas of body; occasional association with neoplastic or inflammatory conditions
- 5 major clinical types:
- Solitary: most common form, more likely to present on lower extremities
- Multiple with hidrotic ectodermal dysplasia (Schöpf-Schulz-Passarge syndrome): usually on palms and soles
- Multiple without associated cutaneous findings: usually on palms and soles of elderly
- Nevoid
- Extremely rare; also called nonfamilial, unilateral linear acrosyringeal adenomatosis
- Unilateral plaques and papules in a linear arrangement
- Often on extremities of young adults
- Reactive: associated with neoplasia; also venous stasis, nail trauma, chronic foot ulcer, burn ulcer, nevus sebaceus, enterostomy site
Etiology
- Unclear if neoplastic, hamartomatous, reactive or a nevus
- Case reports indicate an association with leprosy (Clin Exp Dermatol 2007;32:533)
- Single cases have been found to harbor HPV10 and HPV107
Pathophysiology
- Morphologic, immunohistochemical and electron microscopic evidence supports origin from eccrine duct cells
Clinical features
- Schöpf-Schulz-Passarge syndrome: autosomal dominant with eccrine syringofibroadenoma, palmoplantar keratoderma, hypodontia, hidrocystomas (Acta Derm Venereol 2008;88:607): due to defect localized to chromosome 13
- Clouston Syndrome:
- 4 cases were associated with this hereditary ectodermal dysplasia of nail dystrophy, alopecia and palmoplantar hyperkeratosis (Am J Dermatopathol 2009;31:157)
- Due to defect in GJB6 gene on chromosome 13
- Several cases have been associated with squamous cell carcinoma
- One case had spontaneous involution (Clin Exp Dermatol 2009;34:e66)
Case reports
- 55 year old man with reactive eccrine syringofibroadenoma arising in peristomal skin (J Am Acad Dermatol 2008;58:691)
- 63 year old man (Am J Dermatopathol 1992;14:328)
- 72 year old woman with eccrine syringofibroadenoma and a burn scar ulcer (Br J Dermatol 2000;143:591)
- 82 year old man with 3 cm plaque in dorsum of left hand (Am J Dermatopathol 1997;19:58)
- Clear cell syringofibroadenoma (of Mascaro) of nail (Br J Dermatol 2001;144:625)
- Eccrine syringofibroadenoma treated with a dual pulse width flashlamp pumped pulsed dye laser (Dermatol Surg 1999;25:418)
Treatment
- Benign condition
- Single lesions are treated by surgical excision
- Therapy for cases with multiple lesions depends on size and location
- Laser therapy with dual pulse width flashlamp has been used successfully
- Etretinate topical therapy has been used successfully for diffuse lesions
Gross description
- Verrucous papules, nodules and ulcerative plaques
- Sometimes multiple nodules arranged in a symmetrical nevoid fashion
Clinical images
Images hosted on other servers:
Multiple irregular nodules
Fleshy nodules of ESFA
Microscopic (histologic) description
- Thin anastomosing reticulated cords and strands of basaloid monomorphous cuboidal cells extending from the basal layer of epidermis into dermis
- Cells are slightly smaller than neighboring keratinocytes
- Loose fibrovascular stroma
- Cords have scattered ductal structures resembling eccrine ducts lined by CEA+ cells
- Nests of clear cell change may occur
- Typically lacks a rich plasmacytic infiltrate
Microscopic (histologic) images
Contributed by Sara C. Shalin, M.D., Ph.D.
Eccrine syringofibroadenoma
Images hosted on other servers:
Eccrine
syringofibroadenoma
(Mascaro)
Strands of epithelial cells
Electron microscopy description
- Tumor cells with tonofilaments/desmosomes, numerous glycogen granules
- Basal lamina
- Globular keratohyaline granules around ducts
- No lamellar granules
- Poorly developed cornified cell envelopes (Am J Dermatopathol 1996;18:207)
Differential diagnosis
- Acrosyringeal nevus: some authors consider to be the same entity as acrosyringeal adenomatosis, but others differentiate it on the basis of a rich plasmacytic infiltrate in the dermis
- Clear cell acanthoma
- Fibroepithelioma of Pinkus: has buds of basilar epithelium with the characteristics of basal cell carcinoma
- They lack ductule formation within the cords of cells
- IHC in fibroepithelioma of Pinkus will reflect basaloid and not eccrine differentiation
- The clinical presentation will often be different as well
- Poroma: shares the eccrine histogenesis but lacks the reticular, corded architecture of Acrosyringeal Adenomatosis
- Porocarcinoma: lacks the corded architecture and has malignant cytology
