ABSTRACT
BACKGROUND: Digital papillary adenocarcinoma (DPA), formerly known as aggressive DPA, is a rare adnexal cancer of sweat gland differentiation with metastatic potential. DPA epidemiology and patient outcome data are a prerequisite to develop diagnostic and therapeutic guidance, which is lacking for this rare cancer. OBJECTIVES: To report the incidence, patient demographics and treatment of patients with DPA in England from 1 January 2013 to 31 December 2020 using national cancer registry data. METHODS: DPA diagnoses in England during 2013-2020 were identified from the National Cancer Registration Dataset using morphology and behaviour codes. These were registered from routinely collected pathology reports and cancer outcomes and services datasets. The 2013 European age-standardised incidence rates (EASRs) were calculated. RESULTS: In total, 36 DPA (7 in females and 29 in males) were diagnosed. The median age at diagnosis for the cohort was 54 years (interquartile range 46-64). The most frequently affected sites were upper limbs (81%). All patients in the cohort received surgical excisions. The European age-standardised incidence rate (EASRs) was 0.10 [95% confidence interval (CI) 0.07-0.14] per 1,000,000 person-years (PY)]. CONCLUSION: This study reports the incidence and variation of DPA in England between 2013 and 2020. DPA was more common in older men and predominantly affected the upper limbs. This supports the need to develop a national policy for the reporting and management of DPA as well as clinical guideline development.
ABSTRACT
A woman in her 60s presented with a longstanding history of a purplish, fleshy and pedunculated nodule on the right shin on a background of bilateral lower limb lymphoedema. A shave biopsy with double curettage of the base of the lesion revealed a nodular tumour with hyperchromatic basaloid cells arranged in a cribriform pattern and encircling eosinophilic substance. Immunohistochemistry staining showed cells positive for pancytokeratin, low molecular weight keratin, BerEP4 and negative for cytokeratin 20. There were no clinical or radiological features of primary visceral malignancy. These histological and immunohistochemical features favour a diagnosis of primary cribriform carcinoma of the skin. This is a rare, indolent skin appendage tumour of presumed apocrine origin with no reported cases in the literature of metastasis or local recurrence after excision.