Answer of Dermatopathology Case 11

Aggressive Digital Papillary Adenocarcinoma

Visit: Pathology of Aggressive Digital Papillary Adenocarcinoma
Visit: Dermatopathology site

Abstract:

Aggressive digital papillary adenocarcinoma: a review.Clin Exp Dermatol. 2009 Oct 23.
Summary Vigorous treatment of aggressive digital papillary adenocarcinoma (ADPA), including amputation, has been recommended by most authors, but then appropriateness and effectiveness of excision as an alternative to amputation has not been systematically evaluated. To evaluate the appropriateness and effectiveness of excision as an alternative to amputation in the treatment of ADPA, we reviewed the clinical presentations, treatments and patient outcomespresented in case reports on ADPA available on Ovid MEDLINE. We also assessed theresults of immunohistochemical staining for proliferation markers in one patient in order to explain the nonaggressive nature of ADPA noted in that patient. Except for the duration of lesions, there was no significant difference inclinical outcome between the excision and amputation groups. We also found that p63 may be a useful marker for distinguishing primary ADPA from metastatic adenocarcinomas. In addition, the intensity of Ki67 expression in tumour cellsmay be a marker of aggressive behaviour and thus be helpful in therapeutic decision-making. Wide excision with or without sentinel lymph-node biopsy is afeasible alternative to amputation. It should be considered in patients who present with a long-standing history of ADPA without evidence of underlying bone invasion or distant metastasis and with low-intensity expression of proliferation markers.

Aggressive digital papillary adenocarcinoma: a report of two diseases and review of the literature. J Am Acad Dermatol. 2009 Feb;60(2):331-9.
Aggressive digital papillary adenocarcinoma (ADPAca) is a rare, underreported,and often misdiagnosed malignant tumor of the sweat glands most commonly occurring in males in their fifties to seventies. We report two cases of ADPAca with important clinical implications. A 54-year-old man presented 3 years after digit amputation for ADPAca with new blue nodules on his arm, lymphadenopathy, and a lung nodule; he was diagnosed with and treated for metastatic ADPAca. Heunder went chemotherapy, but died 4 months later. A 15-year-old boy presented withan enlarging tumor on his finger occurring after a trauma 3 years earlier. The tumor was suspected to be a deep fungal infection or pyogenic granuloma; however, results of excisional biopsy revealed an ADPAca. The patient underwent amputation and sentinel lymph node examination. No signs of metastases were found, and he isalive and well. These cases highlight both the importance of high clinicalsuspicion of digital tumors, even in children, enabling prompt diagnosis and treatment and also emphasize the metastatic potential of the tumor and the need for aggressive treatment and close long-term follow-up.

Human papillomavirus-58 and -73-associated digital squamous cell carcinoma in apatient with aggressive digital papillary adenocarcinoma. Am J Dermatopathol. 2009 Jun;31(4):375-8.
Aggressive digital papillary adenocarcinoma (ADPA) is a rare tumor that is considered to arise from eccrine sweat glands of the skin. It occurs predominantly in men with a mean age in the sixth decade. It shows a strong tendency for local recurrence and has the potential to metastasize to distant sites. Prompt diagnosis and regular follow-up are important to ensure the bestpossible outcome. We discuss a case of recurrent ADPA associated with subsequent squamous cell carcinoma (SCC) in different contralateral digits in a 55-year-old man. One SCC lesion tested positive for human papilloma virus (HPV)-58.HPV-associated digital SCCs have been reported; most cases are HPV-16 positive.This report describes a rare case of an HPV-58-positive invasive digital SCC and an HPV-73-positive SCC in situ associated with ADPA.

Aggressive digital papillary adenocarcinoma on the palm with pulmonary metastases. J Dermatol. 2008 Jul;35(7):468-70.
A 41-year-old Japanese male had aggressive digital papillary adenocarcinoma with pulmonary metastases. He had an asymptomatic, solitary, dome-shaped, skin-colored firm nodule on his left palm for half year. The tumor consisted of multiplelobules of anaplastic epithelial cells with central necrosis. The neoplasticcells were immunohistochemically positive for cytokeratin and S-100 protein. Two years after the lesion was removed, pulmonary nodular lesions were found on chest X-ray. On histopathological examination, the pulmonary biopsy specimens showed lobular proliferation of acantholytic tumor cells and some ductal structures associated with papillary projections. Five years after the initial removal of the lesion, the patient was referred to our hospital because of a recurrent skin nodule on his left palm. The recurrent skin tumor was found to have lobular proliferation of anaplastic cells. On immunohistochemistry, the pulmonary metastasis and the palmar skin nodules were identical. The tumor was diagnosed as an aggressive digital papillary adenocarcinoma. This report is a rare case ofaggressive digital papillary adenocarcinoma that was diagnosed based on the histopathology of the pulmonary metastases, which showed ductal structuresassociated with papillary projections.