A case report of a rare adnexal tumour: aggressive digital papillary adenocarcinoma

Abstract

We report the case of a 78-year-old Caucasian gentleman who presented with a painful swelling in the nail bed of the right middle finger. Following amputation of the right middle phalanx histopathology confirmed aggressive digital papillary adenocarcinoma (ADPA). Further surgical treatment was offered but declined. Approximately 17 months later, the patient was found to have pulmonary metastasis. ADPA is a rare neoplasm of the eccrine sweat glands, which commonly presents as a slow-growing mass between the nail bed and distal interphalangeal joint. The disease is classically aggressive with a 14% chance of metastatic spread. The chance of recurrence is 50% with no or sub-optimal treatment, which reduces to 5% following removal with adequate resection margins. This case shows a prolonged period of disease-free survival, but highlights the need for thorough and aggressive management in cases of ADPA as well as frequent and long-term follow-up.

An Aggressive Digital Papillary Adenocarcinoma and a Grossly Similar, Contralateral Callus

“…Traumatic or chronic wound is a feature of some of the reported cases. Coulsen et al reported on a case with a remote history of phalanx fracture, followed by an increase in swelling over a period of 6 months leading to diagnosis 8 . Frey et al reported on a patient with a chronic traumatic wound, diagnosed with ADPA 2 years later, and treated with ray amputation 9 .…”

“…Several reports describe a history of antecedent trauma, chronic nonhealing wound, or a long career in a profession involving repetitive trauma resulting in a chronic lesion. Typically, these stable lesions subsequently undergo an accelerated period of pain and enlargement leading to ADPA diagnosis, suggesting repetitive epidermal trauma as a potential risk factor [8][9][10][11] .…”

Digital Papillary Adenocarcinoma, a Rare Malignant Tumor

“…The age at onset is usually between the fifth and seventh decades (the average is 52 years old) [2], affecting males more often than females (7–9:1) [1, 8]. The tumor commonly presents as a solitary mass, may be located between the nail bed and distal interphalangeal joint [8], but it has also been reported on the palmar surface of the hand, plantar surface of the foot, lower leg, and on the web spaces of hands and feet, with rare cases reported on the lips and ears [9]. Often located on the dermis, DPA can invade deeper structures such as muscles, tendons or bones.…”

“…Local recurrence can occur in 30–50% of the cases after surgical excision, which can be reduced to 5% after excision with a clear resection of the margins [2]. The overall rate of reported metastatic disease is between 14 and 26% [2, 5, 8], being more prevalent in the lungs, but other areas have been observed, such as lymph nodes, brain, skin, bones, and kidneys [8, 9].…”

Challenges in the diagnosis of cutaneous adnexal tumours

“…Metastatic adenocarcinomas with papillary architecture can be distinguished from DPACs using a combination of a comprehensive patient history and IHC studies. For example, CDX2 and CK20 label tumours of gastrointestinal origin while thyroglobulin and TTF-1 are positive in thyroid papillary carcinomas 1 3 7 80 84 85…”