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Aggressive digital papillary adenocarcinoma (Digital Papillary
Adenocarcinoma) is a rare variant of sweat gland carcinoma of the digits
and volar surfaces which has the potential for highly aggressive
biological behaviour.
Papillary lesions of the digits were first described in 1987.
Aggressive digital papillary
adenoma and adenocarcinoma. A clinicopathological study of 57 patients,
with histochemical, immunopathological, and ultrastructural
observations.J
Cutan Pathol. 1987
Jun;14(3):129-46.
Based mostly on histologic
criteria these lesions were subtyped as:
i)
Aggressive digital papillary adenoma ii) Aggressive digital papillary adenocarcinoma
.
Subsequent follow-up has
shown that it was not possible to distinguish reliably these two
entities . Neither clinical nor histologic features were indicative of
biologic behavior of these tumours. Metastatic
disease occurred equally with either
Aggressive digital
papillary adenoma
or Aggressive digital papillary adenocarcinoma .
The term 'adenoma' was
abandoned as all papillary digital neoplasms seemed to have an
aggressive behaviour despite benign histologic appearance.
Site:
Occurs
exclusively on fingers, toes and adjacent skin of palms and soles.
Clinical
presentation: Presents as a solitary, painless, cystic
nodule, usually less than 2cm in diameter.
Gross features:
Tumour has been described
as being tan-gray to white-pink and rubbery.
Microscopic features:
A poorly circumscribed lesion which
involves dermis and subcutis ;
Consists of both solid and cystic
components ;
Tubulo-alveolar
and ductal structures with areas of papillary projections protruding
into cystic lumina ; Cribriform pattern may be present ; Glandular structures contain eosinophilic
material ;
Solid
area is characterized by back-to-back glands lined by cuboidal or low
columnar epithelium ;
Backround stroma displays thin fibrous septa to areas of dense
hyalinized collagen ; Mitotic figures are noted ; Cytologic atypia is usually not striking.
Some cases show poor glandular differentiation, poorly defined
borders, clear cut infiltrative properties, focal necrosis and
cytological evidence of malignancy.
There may be invasion of soft tissues, blood vessels and underlying
bones.
Immunohistochemistry:
Immunopositive for S100
protein, CEA and cytokeratin.
Recognition of these
tumors is important because of a potential risk of local recurrence ( 40
- 50% of patients) and distant metastases (14% of patients), usually involving lymph nodes and/or lungs.
Aggressive surgical
treatment consisting of wide local excision with clear margins and
close surveillance for signs of recurrence or metastasis are indicated
for this rare sweat gland neoplasm.
Differential
diagnosis:
May be misdiagnosed particularly for a metastasis
of papillary adenocarcinoma originating in the colon, thyroid, or
breast. Clinicopathological correlation is essential to rule out a
possible risk of metastatic carcinoma of the skin.
Papillary eccrine adenoma: The ductal structures are larger and more dilated than those
in the papillary eccrine adenoma
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