Skin nonmelanocytic tumor
Adnexal tumors
Sweat gland derived (apocrine & eccrine glands)
Papillary eccrine adenoma


Minor changes: 29 September 2020

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PubMed Search: Papillary eccrine adenoma

Masoud Asgari, M.D.
Sheng Chen, M.D., Ph.D.
Page views in 2019: 3,129
Page views in 2020 to date: 2,871
Cite this page: Asgari M, Chen S. Papillary eccrine adenoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skintumornonmelanocyticpapillaryeccrineadenoma.html. Accessed October 24th, 2020.
Definition / general
Essential features
  • Solitary nodule, mostly in lower extremity of young black women
  • Well circumscribed in the dermis, composed of tubules with intraluminal papillary, micropapillary or cribriform and peripheral flat myoepithelial cells
  • Nuclear hyperchromasia, single cell necrosis and mitotic figures present
Terminology
ICD coding
  • ICD-10: D23.9 - Other benign neoplasm of skin, unspecified
  • ICD-11: 2F22 - Benign neoplasms of epidermal appendages
Epidemiology
  • Usually occurs in young people
  • Blacks > whites
  • Women > men
Sites
  • Usually extremities, lower more than upper
Pathophysiology
Etiology
  • Unknown
Clinical features
Diagnosis
  • Incisional or excisional biopsy should be performed with histologic examination for a definite diagnosis
Prognostic factors
  • Excellent prognosis; no recurrence following simple conservative complete excision
  • Can have recurrence / persistence after incomplete removal
Case reports
Treatment
  • Simple surgical excision
Microscopic (histologic) description
  • Nodular, well circumscribed and often asymmetrical from scanning magnification
  • Tubular or cystic structures with intraluminal papillary, micropapillary or cribriform growth (common)
  • Ductal lining is bilayered with luminal cuboidal cells and peripheral flat myoepithelial cells
  • Occasional findings
Microscopic (histologic) images

Contributed by Masoud Asgari, M.D. and Sheng Chen, M.D., Ph.D.
Well circumscribed

Well circumscribed

Tubules with micropapillae Tubules with micropapillae

Tubules with micropapillae

Epithelial and myoepithelial layers Epithelial and myoepithelial layers

Epithelial and myoepithelial layers


Necrosis

Necrosis

Mitosis

Mitosis

p63

p63

Ki67

Ki67

Positive stains
Negative stains
Electron microscopy description
  • Cells of the inner layer have features of eccrine secretory (clear) cells
  • Cells of the outer layer have characteristics of both ductal basal cells and glandular myoepithelial cells (J Dermatol Sci 1990;1:65)
Sample pathology report
  • Skin, right foot, excision:
    • Papillary glandular neoplasm (see comment)
    • Margins negative for tumor
    • Comment: The findings are consistent with a papillary eccrine adenoma (also called papillary adenocarcinoma in situ).
Differential diagnosis
Board review style question #1


A 41 year old African American woman with a solitary, 1 cm nodule localized on the right leg comes to the office. Incisional biopsy was performed and the findings are illustrated above. Which of the following statements regarding this neoplasm is correct?

  1. Identical features may be seen in nipple adenoma
  2. It is composed of solid proliferation of both myoepithelial cells and epithelial cells
  3. Many plasma cells are present
  4. Myoepithelial cell layer may be absent at the periphery of some tubular structures
  5. This is an aggressive neoplasm with a high rate of metastasis (15%)
Board review answer #1
A. Identical features may be seen in nipple adenoma. This is a papillary eccrine adenoma. Nipple adenoma and papillary eccrine adenoma have identical histopathologic features and therefore choice A is correct. So called papillary eccrine adenoma is not an aggressive neoplasm (choice E) but it may recur if it is not completely excised. The lesion was initially considered benign and called adenoma by Rulon and Helwig on the basis of available follow up information. However, adenocarcinoma in situ, when completely removed, behaves in the same way, namely, no recurrence or metastasis. Choice B is a cardinal feature of so called aggressive digital papillary adenocarcinoma. Choice C is a feature of syringocystadenoma papilliferum. Choice D may suggest an invasive focus within the neoplasm, given the absence of myoepithelial layer around some tubular structures.

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Reference: Papillary eccrine adenoma
Board review style question #2
Which of the following gene mutations is usually seen in papillary eccrine adenoma?

  1. BRAF V600E
  2. CYLD1
  3. FLCN
  4. MLH1
  5. PTCH
Board review answer #2
A. BRAF V600E. Mutation in BRAF V600E is frequently seen in papillary eccrine adenoma, similar to its analogue nipple adenoma. Mutation in CYLD1 is usually seen in cylindroma. Mutation in PTCH is usually seen in adenoid basal cell carcinoma. Mutations in MMR genes (such as MLH1, MSH2, MSH6 and PMS2) are associated with sebaceous carcinoma. FLCN may be mutated in fibrofolliculoma / trichodiscoma.

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Reference: Papillary eccrine adenoma
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