Skin nonmelanocytic tumor

Adnexal tumors

Sweat gland derived (apocrine & eccrine glands)

Syringocystadenoma papilliferum

Minor changes: 17 November 2021

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PubMed Search: Syringocystadenoma papilliferum [TI] "last 5 years"[DP]

Mahyar Khazaeli, M.D.
Kiran Motaparthi, M.D.
Page views in 2021: 16,729
Page views in 2022 to date: 992
Cite this page: Khazaeli M, Motaparthi K. Syringocystadenoma papilliferum. website. Accessed January 21st, 2022.
Definition / general
Essential features
  • Often observed in association with other benign adnexal neoplasms, such as nevus sebaceus (8 - 19% of patients), apocrine nevus, tubular apocrine adenoma, apocrine hidrocystoma, apocrine cystadenoma and clear cell syringoma (Pathologica 2006;98:178)
Clinical features
  • Skin biopsy
Case reports
Clinical images

Images hosted on other servers:

Posterior cranium, exophytic mass

Rose colored papule, left flank

Erythematous plaque, central crustation

Papillomatous exophytic, temporal scalp

Gross description
Microscopic (histologic) description
  • Cystic invaginations of the infundibular epithelium projecting into the dermis, covered by a double cell layer (An Bras Dermatol 2017;92:721):
    • Innermost layer is composed of columnar cells with decapitation secretion
    • Outermost layer is composed of cuboidal cells with papillary projections
  • Verrucous (papillomatous) epidermal hyperplasia with hyperkeratosis and hypergranulosis (Pathologica 2006;98:178)
  • Exoendophytic configuration with a gradual transition from stratified squamous epithelium at the epidermal surface to a bilayered ductal epithelium (Pathologica 2006;98:178)
  • Papillary fronds extend upward from the base and plasma cells are common in the stroma of each frond (Pathologica 2006;98:178)
  • Many irregular duct-like structures and cystic spaces (Pathologica 2006;98:178)
  • Ducts, containing papillary processes and lined by 2 epithelial cell layers, connect to the surface (Indian J Dermatol Venereol Leprol 2009;75:170)
  • Ducts invaginate from the surface into the dermis (J Clin Diagn Res 2014;8:QD03)
  • Edoendophytic architecture, verrucous epidermal hyperplasia, sometimes pseudoepitheliomatous hyperplasia and connection of glands to epidermis (An Bras Dermatol 2017;92:721)
  • Background features of nevus sebaceus may be observed (An Bras Dermatol 2017;92:721)
Microscopic (histologic) images

Contributed by Kiran Motaparthi, M.D.

Glandular proliferation

Cystic proliferation

Glands with epidermal connection

Glands with double layer

Glands forming papillae

Cystic and papillary appearance glands

Papillary architecture

Double layer epithelium

Positive stains
Molecular / cytogenetics description
Sample pathology report
  • Scalp, biopsy:
    • Syringocystadenoma papilliferum (see comment)
    • Comment: Several cystic invaginations arise from a papillomatous epidermis. These invaginations demonstrate papillae lined by 2 rows of cuboidal to columnar epithelial cells, with oval nuclei and a pale eosinophilic cytoplasm. The deep dermis contains tubular glands with apocrine decapitation secretion. The stroma contains a dense mononuclear infiltrate, which is comprised predominantly of plasma cells.
Differential diagnosis
Board review style question #1

A 38 year old man presents with a verrucous nodule on the scalp, present for the past 12 years. Representative histopathology is shown in the above image. Which of the following statements is correct?

  1. More common in adulthood
  2. Represents a malignant adnexal tumor
  3. Subset of tumors have loss of heterozygosity for PTCH or p16
  4. Usually does not communicate with surface epithelium
Board review style answer #1
C. Subset of tumors have loss of heterozygosity for PTCH or p16

Comment Here

Reference: Syringocystadenoma papilliferum
Board review style question #2

An 8 year old girl presents with a slowly growing, 3 cm, erythematous nodule with smooth surface on the left labium majus, present since birth. A biopsy is performed (see above image). Which of the following is most associated with this tumor?

  1. Basal cell carcinoma
  2. Merkel cell carcinoma
  3. Nevus sebaceus
  4. Squamous cell carcinoma
Board review style answer #2
C. Nevus sebaceus

Comment Here

Reference: Syringocystadenoma papilliferum
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