Skin - Nonmelanocytic tumors
Adnexal tumors - eccrine sweat glands
Acrosyringeal adenomatosis / Eccrine syringofibroadenoma

Author: Aravindhan Sriharan, M.D. (see Authors page)
Editor: Sara Shalin, M.D.

Revised: 5 October 2016, last major update April 2015

Copyright: (c) 2002-2016, PathologyOutlines.com, Inc.

PubMed Search: Eccrine syringofibroadenoma [title]

Cite this page: Acrosyringeal adenomatosis / Eccrine syringofibroadenoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/skintumornonmelanocyticacrosyringealadenomatosis.html. Accessed December 10th, 2016.
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)
  • 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:
    1. Solitary: most common form, more likely to present on lower extremities
    2. Multiple with hidrotic ectodermal dysplasia (Schöpf-Schulz-Passarge syndrome): usually on palms and soles
    3. Multiple without associated cutaneous findings: usually on palms and soles of elderly
    4. Nevoid
      • Extremely rare; also called nonfamilial, unilateral linear acrosyringeal adenomatosis
      • Unilateral plaques and papules in a linear arrangement
      • Often on extremities of young adults
    5. Reactive: associated with neoplasia; also venous stasis, nail trauma, chronic foot ulcer, burn ulcer, nevus sebaceous, 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
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

Coalescent pink papules

Micro 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
Micro Images

Images hosted on other servers:

Eccrine syringofibroadenoma (Mascaro)

Strands of epithelial cells

Courtesy of Dr. Sara Shalin

Positive Stains
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