Skin nonmelanocytic tumor
Adnexal tumors
Sweat gland derived (apocrine & eccrine glands)
Digital papillary adenocarcinoma


Minor changes: 9 June 2020

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PubMed Search: Digital papillary adenocarcinoma [TIAB]

Haruto Nishida, M.D., Ph.D.
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Cite this page: Nishinda H. Digital papillary adenocarcinoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/skintumornonmelanocyticaggressivedigital.html. Accessed October 24th, 2020.
Definition / general
  • Malignant adnexal tumor with a marked predilection of acral sites
  • Helwig first described as eccrine acrospiroma in 1979 (J Cutan Pathol 1987;14:129)
Essential features
  • Malignant adnexal tumor with a marked predilection of acral sites
  • Usually fingers and toes, rarely palm and sole
  • Clinically confused with a cystic lesion
  • Tumor cells are basaloid / cuboidal to low columnar epithelial cells and myoepithelial cells with mild to moderate cytologic atypia
  • Rarely, metastasis to lung and lymph node
Terminology
  • Also called digital papillary eccrine adenoma, aggressive digital papillary adenoma, digital adenocarcinoma
ICD coding
  • ICD-O: 8408/3 - Digital papillary adenocarcinoma
Epidemiology
Sites
Pathophysiology
  • Ultrastructural finding (see below) and eccrine glands are abundant in acral sites
    • This is because digital papillary adenocarcinoma is an eccrine tumor
Etiology
  • Some patients have trauma
Clinical features
  • Slow growing, asymptomatic nodule (average 1.7 cm, range: 0.4 - 4.3 cm)
  • Clinically confused with a cystic lesion, ulceration is rare
  • Pain may occur if the tumor involves the underlying bone, joint, nerves
  • Rarely, metastatic disease (lung, lymph node) (Am J Surg Pathol 2012;36:1883, J Am Acad Dermatol 2017;77:549)
Diagnosis
  • Clinically, may be suspected from the site of origin and clinical course
  • Histologic examination of tissue provides a definitive diagnosis
Prognostic factors
  • Local recurrence and metastasis are 5 - 21% and 26 - 50%
  • Histopathological features appear not to be predictive
  • Delayed occurrence of metastases and a protracted course, long term follow up is necessary (Am J Surg Pathol 2012;36:1883)
Case reports
Treatment
Clinical images

Images hosted on other servers:

Finger tumor with ulcer

Finger tumor

Gross images

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Fingertip tumor

Microscopic (histologic) description
  • Well circumscribed multinodular tumors within dermis and superficial subcutis
  • Solid and cystic portions, papillary projections, tubular or ductal structures (Am J Surg Pathol 2012;36:1883)
  • Occasionally show infiltrative margin
  • Uncommonly epidermal hyperplasia (3/31), ulceration (2/31), focal epidermal connection (1/31) (Am J Surg Pathol 2012;36:1883)
  • Tumor cells are basaloid, cuboidal to low columnar epithelial cells and myoepithelial cells with mild to moderate cytologic atypia
  • Severe cytologic atypia (3/31) and tumor necrosis (6/31) is noted in a small subset of tumors (Am J Surg Pathol 2012;36:1883)
  • Mitoses may be inconspicuous or frequent (1 - 15 mm2) (Am J Surg Pathol 2012;36:1883)
  • Spindle cell, squamoid differentiation, clear cell changes may be encountered (Am J Surg Pathol 2012;36:1883)
Microscopic (histologic) images

Contributed by Haruto Nishida, M.D., Ph.D.

Fused glands

Solid area with focal necrosis

Sheet like growth pattern

Focal squamous differentiation

Small nest and lymphatic invasion

Virtual slides

Images hosted on other servers:

Digital papillary adenocarcinoma

Positive stains
Electron microscopy description
  • Ultrastructural studies demonstrated three types of neoplastic cells: clear cells, dark cells and myoepithelial cells
  • Some dark cells have dense granules (J Cutan Pathol 1987;14:129)
Molecular / cytogenetics description
Sample pathology report
  • Skin, left ring finger, punch biopsy:
    • Digital papillary adenocarcinoma (see comment)
    • Comment: The histopathological examination shows a well circumscribed multinodular tumor within the dermis to superficial subcutis. It shows solid and cystic portions with focal papillary projections and tubular structures. Two cell layers are present; inner cells are cuboidal epithelial cells with mild to moderate cytologic atypia and outer cells are myoepithelial cells. Some mitoses are seen (1 - 3 mm2). The inner layer is immunoreactive for EMA and CEA which highlighted the luminal border of tubules. The myoepithelial cell (outer cell) layer is positive for SMA, calponin and p63. These histologic features are consistent with digital papillary adenocarcinoma.
Differential diagnosis
Board review style question #1

    A 56 year old man presented with a tumor on his middle finger. The tumor shows solid and cystic areas with focal papillary projections and tubular structures. Which of the following is most likely the correct diagnosis?

  1. Apocrine hidrocystoma
  2. Bowen disease
  3. Digital papillary adenocarcinoma
  4. Hidradenoma
  5. Papillary eccrine adenoma
Board review answer #1
C. Digital papillary adenocarcinoma

Reference: Digital papillary adenocarcinoma

Comment Here
Board review style question #2
    Which of the following is true about digital papillary adenocarcinoma?

  1. Dual layered cystic structures are characteristic
  2. It arises only in digits
  3. It does not occur in young people
  4. Tumor does not show metastasis
Board review answer #2
A. Dual layered cystic structures are characteristic

Reference: Digital papillary adenocarcinoma

Comment Here
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