Digital Papillary Adenocarcinoma

 Associated with low recurrence and metastatic rates






Microscopic




• Multinodular solid or cystic tumor based in dermis

• Solid areas composed of dense collections of cells with focal ductal lumina

• Cystic/glandular areas with papillary projections often seen (although may be focal or absent in some cases)

• Mitotic figures often seen; can be numerous

• Focal necrosis may be present


Top Differential Diagnoses




• Eccrine carcinoma
image Head and neck location

image More infiltrative, small cords and nests of basophilic cells

• Apocrine carcinoma
image Axillary and groin locations

image Infiltrative lobules, nests, and cords of eosinophilic-staining cells

• Apocrine adenoma

• Spiradenoma

• Cylindroma

image
Digital Papillary Adenocarcinoma
Digital papillary adenocarcinoma shows a nodular, basaloid tumor in the mid to deep dermis. Although the tumor is relatively well-circumscribed in most areas image, there are other areas that appear more infiltrative image.


image
Intermediate Magnification of Nodular Area
This nodular area is composed of crowded basaloid cells forming irregular cords and focal ductal lumina image. There is surrounding stromal sclerosis and inflammation image.

image
High Magnification of Cellular Area
This more cellular area is composed of irregular, anastomosing cords of crowded basaloid cells showing nuclear hyperchromasia.

image
High Magnification of Glandular Area
This focus is composed of irregular, anastomosing glandular structures lined by cuboidal epithelial cells. Although the cells are small and relatively bland appearing, they show nuclear crowding and hyperchromasia.


TERMINOLOGY


Abbreviations




• Digital papillary adenocarcinoma (DPA)


Synonyms




• Aggressive DPA

• Aggressive digital papillary adenoma (misnomer, as all are considered malignant)


Definitions




• Malignant sweat gland tumor that typically presents on digits of young adult patients


ETIOLOGY/PATHOGENESIS



Unknown




• May be associated with solar damage

• One case possibly associated with HPV infection


CLINICAL ISSUES


Epidemiology




• Incidence
image Rare tumors

• Age
image Young to middle-aged adults (mean age: 43 years)
– Some cases also reported in children

• Sex
image Most cases occur in men


Presentation




• Papular or nodular lesion on digit
image Typically present on distal finger (most commonly) or toe but some cases on proximal digit/webspace

image Often slowly growing and painless (leading to delayed diagnosis)


Treatment




• Complete and wide excision or amputation
• Sentinel lymph node biopsy has been advocated, given significant incidence of metastasis

image However, most metastases involve lungs


Prognosis




• Wide excision or amputation associated with low recurrence and metastatic rates
image However, some cases present with metastatic disease

image Metastatic disease can also develop years after initial diagnosis, so long-term follow-up required


MACROSCOPIC


General Features



Apr 24, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Digital Papillary Adenocarcinoma

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