Skin nonmelanocytic tumor

Adnexal tumors

Sweat gland derived (apocrine & eccrine glands)

Eccrine syringofibroadenoma



Last author update: 6 February 2024
Last staff update: 6 February 2024

Copyright: 2002-2024, PathologyOutlines.com, Inc.

PubMed Search: Eccrine syringofibroadenoma

Nohra Ghaoui, M.D.
Candice E. Brem, M.D.
Page views in 2023: 7,821
Page views in 2024 to date: 2,743
Cite this page: Ghaoui N, Brem CE. Eccrine syringofibroadenoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skintumornonmelanocyticacrosyringealadenomatosis.html. Accessed April 14th, 2024.
Definition / general
  • Rare, benign eccrine proliferation of anastomosing cords with ductal structures set in a fibrovascular stroma, often located on the extremities
Essential features
  • Erythematous, solitary or multiple papules and nodules often on acral sites
  • Association with Schöpf-Schulz-Passarge syndrome and Clouston syndrome
  • Thin anastomosing cords and strands of basaloid monomorphous cuboidal cells extending from the basal layer of epidermis into dermis with ductal structures and set in a fibrovascular stroma
Terminology
  • Eccrine syringofibroadenoma (of Mascaro)
  • Other names include
    • Eccrine syringofibroadenomatous hyperplasia
    • Acrosyringeal adenomatosis
    • Eccrine poromatosis
    • Linear eccrine poroma
    • Acrosyringeal nevus or acrosyringeal nevus of Weedon and Lewis
    • Nevus syringoadenomatosus papilliferum
ICD coding
  • ICD-O: 8392/0 - syringofibroadenoma
  • ICD-10: D23 - other benign neoplasms of skin
  • ICD-11
    • 2F22 - benign neoplasms of epidermal appendages
    • XH06Y5 - syringofibroadenoma
Epidemiology
  • Rare
  • Wide age range, most cases presenting in the seventh to eighth decade (Am J Dermatopathol 1992;14:328)
    • Schöpf-Schulz-Passarge syndrome associated cases usually present in adolescence; slightly later presentation in Clouston syndrome
  • No clear racial or gender predilection
Sites
Pathophysiology
  • Morphologic, immunohistochemical and electron microscopic evidence supports origin from eccrine duct cells
  • Reactive subtype postulated to be induced by repair / remodeling process (An Bras Dermatol 2021;96:255)
Etiology
  • Unclear; neoplastic / tumoral, hamartomatous and reactive origins postulated
Clinical features
  • Presents as slow growing, solitary or multiple, flesh colored nodule(s)
  • 5 major clinical types (J Am Acad Dermatol 1997;36:569)
    • Solitary: most common form, often on lower extremities
    • Multiple with hidrotic ectodermal dysplasia (Schöpf-Schulz-Passarge syndrome and Clouston syndrome): palms and soles, younger patients (15 - 25 years old)
    • Multiple without associated cutaneous findings: palms and soles
    • Nevoid (i.e., nonfamilial unilateral linear eccrine syringofibroadenoma [ESFA]): rare, unilateral linear papules and plaques
    • Reactive, as can be seen in association with the following
      • Inflammatory dermatoses / processes (i.e., erosive lichen planus, uncontrolled psoriasis, bullous pemphigoid, epidermolysis bullosa, primary cutaneous amyloidosis, thermal scar, venous stasis, chronic diabetic foot ulcer, neuropathy, peristomal skin)
      • Infectious etiologies (i.e., leprosy, HPV)
      • Neoplasms / tumors (i.e., nevus sebaceus, clear cell acanthoma, acantholytic dyskeratotic acanthoma, squamous cell carcinoma, basal cell carcinoma and Merkel cell carcinoma)
  • Associated syndromes
Diagnosis
  • Biopsy / histologic examination and clinicopathologic correlation necessary for diagnosis
Prognostic factors
Case reports
Treatment
Clinical images

Images hosted on other servers:
Vegetating erythematous plaque

Vegetating erythematous plaque

Skin colored fibrotic nodule

Skin colored fibrotic nodule

Pink nodules and plaques

Pink nodules and plaques

Coalescing palmar papules

Coalescing palmar papules

Multiple irregular nodules

Multiple irregular nodules

Fleshy nodules of ESFA

Fleshy nodules of ESFA

Gross description
  • Verrucous erythematous papules, nodules and ulcerative plaques
  • Multiple nodules may be arranged in a symmetrical or unilateral nevoid fashion
Microscopic (histologic) description
  • Proliferation of thin anastomosing reticulated epithelial cords and strands often forming a lattice and extending from the epidermis into the dermis (J Cutan Aesthet Surg 2022;15:335)
  • Lesional cells are monomorphous, basaloid, cuboidal and slightly smaller than adjacent keratinocytes
  • Epithelial cords contain structures resembling eccrine ducts (Ann Dermatol 2020;32:57)
  • Background fibrovascular stroma
Microscopic (histologic) images

Contributed by Candice E. Brem, M.D.
Circumscribed papulonodular lesion

Circumscribed papulonodular lesion

Background fibrous stroma

Background fibrous stroma

Background fibrous stroma

CCA with ESFA

ductal structures

Ductal structures


epidermal based interconnected strands

Epidermal based interconnected strands

lattice-like architecture

Lattice-like architecture

basaloid cuboidal lesional cells

Basaloid cuboidal lesional cells

Positive stains
Negative stains
Electron microscopy description
  • Tumor cells with tonofilaments / desmosomes, numerous glycogen granules
  • Basal lamina
  • Globular keratohyalin granules around ducts / cysts
  • No lamellar granules
  • Poorly developed cornified cell envelopes (Am J Dermatopathol 1996;18:207)
Videos

Eccrine syringofibroadenoma: rare skin adnexal tumor / pattern

Sample pathology report
  • Leg, shave biopsy:
    • Benign appendageal neoplasm with eccrine differentiation consistent with an eccrine syringofibroadenoma (see comment)
    • Comment: The lesion extends to the deep margin. The specimen exhibits an epidermal based proliferation of monomorphous basaloid cells arranged as anastomosing cords forming a lattice with intermixed ductal structures and set in a fibrovascular stroma. These findings support the histologic diagnosis.
Differential diagnosis
Board review style question #1

A 74 year old woman presents with a 1 year history of asymptomatic, erythematous papules on the left heel. A subsequent shave biopsy is performed (see image above). Few intermixed ducts in the epithelial cords are also noted on examination of multiple tissue levels. What is the best histologic diagnosis for this lesion?

  1. Basal cell carcinoma, infiltrative type
  2. Eccrine syringofibroadenoma
  3. Porocarcinoma
  4. Poroma
  5. Squamous cell carcinoma
Board review style answer #1
B. Eccrine syringofibroadenoma (ESFA). The clinical presentation paired with the histologic features of anastomosing reticulated cords and strands of basaloid monomorphous cuboidal cells extending from the epidermis into dermis with few ductal structures set in a fibrovascular stroma are consistent with eccrine syringofibroadenoma. Answer A is incorrect because basal cell carcinoma demonstrates peripheral palisading, clefting and a fibromucinous stroma, while generally lacking intermixed ductal structures. Answer D is incorrect because poroma lacks the reticular and corded architecture of ESFA. Answer C is incorrect because porocarcinoma lacks the corded architecture and has malignant cytologic changes. Answer E is incorrect because squamous cell carcinomas often demonstrate eosinophilic to glassy keratinocytes, cytologic atypia and an infiltrative growth pattern.

Comment Here

Reference: Eccrine syringofibroadenoma
Board review style question #2
In the pediatric population, eccrine syringofibroadenoma (ESFA) has been associated with which genetic disorder?

  1. Birt-Hogg-Dubé
  2. Brooke-Spiegler
  3. Rubinstein-Taybi
  4. Schöpf-Schulz-Passarge
Board review style answer #2
D. Schöpf-Schulz-Passarge. ESFA has been recognized as a cutaneous marker of Schöpf-Schulz-Passarge syndrome. Answer C is incorrect because to date, no cases of ESFA have been seen in Rubinstein-Taybi, a syndrome usually associated with pilomatricomas, hemangiomas and keloids. Answer A is incorrect because Birt-Hogg-Dubé patients have a high frequency of developing fibrofolliculomas, trichodiscomas and lipomas. Answer B is incorrect because Brooke-Spiegler, due to a CYLD mutation, has an increased risk of developing spiradenomas, cylindromas, trichoepitheliomas and basal cell carcinomas.

Comment Here

Reference: Eccrine syringofibroadenoma
Back to top
Image 01 Image 02