CASE 17952 Published on 29.11.2022

Digital papillary adenocarcinoma

Section

Musculoskeletal system

Case Type

Clinical Cases

Authors

Sara Gomez-Pena1, Álvaro Rueda de Eusebio1, Sonia Lon1, Lorenzo Alarcón2, Antonio Ruiz Ollero1

1. Department of Radiology, Hospital Clínico Universitario San Carlos (Madrid, Spain)

2. Department of Anatomic Pathology, Hospital Clínico Universitario San Carlos (Madrid, Spain)

Patient

78 years, male

Categories
Area of Interest Extremities, Musculoskeletal soft tissue, Oncology ; Imaging Technique Conventional radiography, MR
Clinical History

A 78-year-old male came with swelling, erythema, and pain at the volar aspect of the distal phalanx of the left fourth finger but no suppuration. The patient reported having consulted a year earlier due to a cut with a branch. No improvement after two weeks on antibiotics.

Imaging Findings

A soft tissue lesion was identified on the volar aspect of the distal phalanx of the left fourth finger. The lesion was associated with bony destruction of the distal and middle third of the distal phalanx, without joint involvement, and it was intimately related to the anterior aspect of the distal deep flexor tendon.

The lesion was hypointense on T1-weighted sequences and heterogeneous on T2-weighted sequences, with a mild hypointense solid component and multiple hyperintense nodular lesions of a cystic nature.

Following intravenous gadolinium administration, there was a significant diffuse heterogeneous enhancement of the solid component.

Post-surgical imaging showed an alteration of the signal intensity of the soft tissue at the tip of the stump. Additionally, a multiloculated cystic lesion was identified in the volar aspect of the stump tip, which had the same signal characteristics as the original lesion and was highly suggestive of tumour remnants.

Discussion

Background

Digital papillary adenocarcinoma (DPA) is a rare malignant tumour of the sweat glands commonly found on the volar surface of the fingers and toes (3:1) and the adjacent skin of the palms and soles of white men in their 50s-70s, although it has also been observed in children [1,2].

Clinical perspective

These tumours almost always present as solitary painful masses/nodules with slow growth and no associated constitutional symptoms. Although approximately 75% of patients complain of pain at the site of the lesion, these tumours can be present for months to years before the patient seeks medical attention [2,3]. Often located on the dermis, DPA can invade deeper structures such as muscles, tendons, or bones [4].

Imaging perspective

To our knowledge, only Mangrulkar et al. have previously discussed the imaging findings for DPA. In their case report, the MRI demonstrated a lobulated soft tissue lesion with mixed intermediate signal on T1 images and heterogeneously increased signal on T2 fat-suppressed images, without cortical involvement. In the distal aspect of the lesion, a focal area of haemorrhage or proteinaceous material was identified. Following intravenous gadolinium administration, there was a mild diffuse heterogeneous enhancement of the lesion [3]. The MRI findings aid diagnostically because they may suggest a malignant aetiology and they can clarify the extension of the lesion, as well as the involvement of adjacent structures for better surgical planning. The diagnosis of local recurrence through MRI in the early stages may be difficult because the appearance is similar to a reactive/inflammatory process after surgery [4].

Outcome

Due to the rarity of these tumours, the natural progression of DPA is not well characterized and misdiagnosis may lead to the delay of prompt treatment, which along with its significant locally aggressive behaviour increases the risk of metastasis. After surgery, the local recurrence rate is 30-50%, while the prevalence of metastasis has been reported to be around 14%, particularly in lungs and lymph nodes, with the majority of patients being treated either wide local excision or amputation [1-4]. The mainstay of management of DPA is surgical intervention, either wide local excision or digital amputation. However, even with negative margins, the tumour may have already seeded nearby sources. For this reason, patients should still have scheduled follow up every year with an exam and screening chest X-rays to look for any evidence of distant disease [2].

Written informed patient consent for publication has been obtained.

Differential Diagnosis List
Digital papillary adenocarcinoma with tendon involvement with recurrence in surgical site
Complex ganglion cyst
Metastatic carcinoma
Synovial cell sarcoma
Giant cell tumour of the tendon sheath
Osteomyelitis, pyogenic granuloma, and soft tissue infections
Final Diagnosis
Digital papillary adenocarcinoma with tendon involvement with recurrence in surgical site
Case information
URL: https://www.eurorad.org/case/17952
DOI: 10.35100/eurorad/case.17952
ISSN: 1563-4086
License