Skin nonmelanocytic tumor

Adnexal tumors

Sweat gland derived (apocrine & eccrine glands)

Syringocystadenoma papilliferum



Last author update: 10 March 2022
Last staff update: 24 January 2023

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

Mahyar Khazaeli, M.D.
Kiran Motaparthi, M.D.
Page views in 2022: 21,028
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Cite this page: Khazaeli M, Motaparthi K. Syringocystadenoma papilliferum. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skintumornonmelanocyticpapillarysyringadenoma.html. Accessed January 28th, 2023.
Definition / general
Essential features
  • Benign adnexal tumor, most commonly occurring in the head and neck in early childhood (J Dermatol 2004;31:939)
  • Most are solitary papules but variable clinical presentation
  • Macroscopic: pink, hairless plaque or nodule
  • Microscopic: cystic invaginations of the infundibular epithelium extending into the dermis with a double cell layer of inner columnar and outer cuboidal cells
Terminology
Epidemiology
Sites
Pathophysiology
Etiology
  • 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
Diagnosis
  • Skin biopsy
Case reports
Treatment
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
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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