Aggressive Digital Papillary Adenocarcinoma

Background

These rare tumors arise from skin sweat glands. They characteristically occur on the hands, fingers, and toes. They occur predominately in men with a mean age of 52 yrs (19-83 yrs). They average 1.7 cm in size and may be present for 2 months to 15 years with a mean of 29 months. Pain is a frequent presenting complaint. When these tumors were first described, they were divided into an aggressive digital papillary adenoma and a carcinoma. Subsequent follow-up of lesions designated as adenomas found a metastatic potential of 14%. Thus all of these tumors are more correctly classified as carcinomas with no benign counterpart. No consistent microscopic feature has been found which separates benign behaving tumors from those with local recurrence or metastasis.

Under the microscope, these tumor are multinodular, solid, and cystic with papillary projections. Occasional connection to the overlying epidermis is present. A characteristic pattern is fused back-to-back glands lined by cuboidal to low columnar cells. Circumscription with hyalinized stroma is present in some cases. Necrosis is present in about half of the cases. Cytologic atypia is mild to occasionally moderate. The mitotic rate ranges from 0-60 per 10 hpf with a mean of 17.

The prognosis of these tumors is guarded. In the cases of metastasis, two had been treated with re-excision or amputation and had metastasis at the time of their initial presentation. Two others developed metastasis without local recurrence and two patients not treated with re-excision or amputation within 6 months of initial presentation developed subsequent metastasis with local recurrence. A complete excision is recommended since there is a 50% local recurrence rate in patients who do not receive treatment.

OUTLINE

Epidemiology

Disease Associations

Pathogenesis

Laboratory/Radiologic/
Other Diagnostic Testing

Gross Appearance and Clinical Variants

Histopathological Features and Variants

Special Stains/
Immunohistochemistry/
Electron Microscopy

Differential Diagnosis

Prognosis

Treatment

Commonly Used Terms

Internet Links

GROSS DISEASE AND CLINICAL VARIANTS CHARACTERIZATION

Aggressive digital papillary adenocarcinoma of the foot: the clinicopathologic features of two cases.

Bakotic B, Antonescu CR.

Memorial Sloan-Kettering Cancer Center, Department of Pathology, New York, NY, USA.

J Foot Ankle Surg 2000 Nov-Dec;39(6):402-5 Abstract quote

Aggressive digital papillary adenocarcinoma is a rare variant of sweat gland carcinoma of the digits and volar surfaces which has the potential for highly aggressive biologic behavior.

The authors report two cases of aggressive digital papillary adenocarcinoma of the foot. In each instance, the tumor arose on the volar surfaces of the digits. Additionally, in both instances, the tumor's unusual clinical presentations delayed biopsy and definitive diagnosis for several months. Following initial conservative surgery, both patients suffered local recurrences. In one case, local recurrence was followed by widespread distant metastases. Although aggressive digital papillary adenocarcinoma is virtually limited to the hands and feet, to the authors' knowledge it has not been previously reported in the podiatric literature.

In this report, the clinicopathologic features of this rare variant of sweat gland carcinoma are summarized and a brief review of the literature is presented.

HISTOPATHOLOGY CHARACTERIZATION

Aggressive digital papillary adenoma and adenocarcinoma. A clinicopathological study of 57 patients, with histochemical, immunopathological, and ultrastructural observations.

Kao GF, Helwig EB, Graham JH.

J Cutan Pathol 1987 Jun;14(3):129-46 Abstract quote

Fifty-seven examples of a rare eccrine sweat gland tumor (aggressive digital papillary adenoma and adenocarcinoma) were studied by means of light microscopy, electron microscopy, and immunoperoxidase techniques. The neoplasm occurred as a single, painless mass, almost exclusively on the fingers, toes, and adjacent skin of the palms and soles.

Microscopic features were distinct from those of other eccrine sweat gland tumors and often led to the diagnosis of such metastatic carcinoma as that of the breast. The characteristic histologic features included tubuloalveolar and ductal structures with areas of papillary projections protruding into cystic lumina. The stroma varied from thin, fibrous septae to areas of dense, hyalinized collagen. Forty tumors were classified as adenoma (ADPA) and 17 as adenocarcinoma (ADPAca).

Histologically, ADPAca was distinguished from ADPA by its poor glandular differentiation and by necrosis, cellular atypia and pleomorphism, invasion of soft tissue and bone, and invasion of blood vessels. Eighteen (50%) patients with ADPA and 8 (47.0%) who had ADPAca developed recurrent lesions (2 months to 9 years) after surgical removal of the tumor. Seven (41.2%) patients with ADPAca developed metastases, of which 5 involved the lung. Three patients died of metastases, 5 to 20 years after surgical treatment of the primary tumor. The histologic malignant features in ADPAca are indicative of potential for distant metastasis and fatality.

The recognition of aggressive digital papillary adenoma and adenocarcinoma as a distinct clinicopathological eccrine sweat gland neoplasm is important because of the potential for aggressive local growth and distant metastasis.

PROGNOSIS AND TREATMENT CHARACTERIZATION

Prognostic Factors

Aggressive Digital Papillary Adenocarcinoma
(Aggressive Digital Papillary Adenoma and Adenocarcinoma Revisited)
Wayne H. Duke, M.D.; Theresa T. Sherrod, M.D.; George P. Lupton, M.D.

From the Armed Forces Institute of Pathology (AFIP), Department of Dermatopathology, Washington, DC (W.H.D., G.P.L.), and the National Naval Medical Center, Department of Pathology, Bethesda, Maryland (T.T.S.), U.S.A.

Am J Surg Pathol 2000;24:775-784 Abstract quote
In 1987 a clinicopathologic study by the Armed Forces Institute of Pathology (AFIP) of rare sweat gland tumors, termed aggressive digital papillary adenoma and adenocarcinoma, was published. Since that time, the AFIP has continued to collect these tumors for study. Based on additional follow-up data, we think the original classification of these tumors requires revision. Sixty-seven cases of aggressive digital papillary adenoma and adenocarcinoma were studied according to their clinical characteristics and histologic features.

Fifty of these were originally diagnosed as adenoma and 17 as adenocarcinoma. Follow up on 45 (67%) of the patients was obtained. None of the clinical or histologic parameters studied were found to be predictive of recurrence or metastasis, indicating that the originally proposed criteria for distinguishing between benign (adenoma) and malignant (adenocarcinoma) do not predict biologic behavior.

When primary tumors were treated by subsequent re-excision or amputation, only one recurred (5%), when not so treated, 11 recurred (50%) regardless of the original diagnosis (p <0.05). Metastasis occurred in six (14%) cases and in three cases led to the death of the patient. Three of these metastatic cases had met the earlier criteria for adenoma. Pulmonary metastases were observed in five cases. No effective treatment for widespread metastatic disease has yet been developed.

Because histologic features with prognostic significance could not be demonstrated in this retrospective review, we propose that all aggressive digital papillary tumors be designated aggressive digital papillary adenocarcinoma.

Aggressive Digital Papillary Adenocarcinoma A Case Report and Review of the Literature

Debra M. Jih, etal.

Am J Dermatopathol 2001;23:154-157 Abstract quote

We report a case of an aggressive digital papillary adenocarcinoma (ADPA) on the right thumb of a 48-year-old white man. Histologic evaluation of the initial biopsy demonstrated features consistent with those proposed for aggressive digital papillary adenoma; however, re-excision of the remaining lesion revealed histologic features consistent with aggressive digital papillary adenocarcinoma. These tumors have a high rate of local recurrence and can metastasize, occasionally resulting in mortality.

Our case demonstrates that even if the histologic criteria of aggressive digital papillary adenoma are met, the lesion may still represent an aggressive digital papillary adenocarcinoma (ADPAca).

In agreement with a recent study by Duke et al., this case supports the idea that aggressive digital papillary lesions should be classified as aggressive digital papillary adenocarcinoma.

Survival

Parameter
Percentage

Local recurrence if treated with complete excision
5% (1/21)

Local recurrence if not treated
50% (11/22)

Metastasis
14% (6/43)

Treatment Wide excision

Sentinel node biopsy for staging of aggressive digital papillary adenocarcinoma.

Malafa MP, McKesey P, Stone S, Dudley-Walker S, Cockerell CJ.

Department of Surgery, Southern Illinois University School of Medicine, Springfield, Illinois 62794-9638, USA.

Dermatol Surg 2000 Jun;26(6):580-3 Abstract quote

BACKGROUND: Aggressive digital papillary adenocarcinoma is a rare malignancy with a propensity for metastases and recurrence. The role of lymph node staging in this tumor is poorly defined. We describe the use of sentinel lymph node mapping and biopsy in staging this tumor.

OBJECTIVE: To describe and discuss the use of lymphatic mapping in staging aggressive digital papillary adenocarcinoma.

METHODS: Sentinel lymph node mapping and biopsy was performed after excision of an aggressive digital papillary adenocarcinoma of the toe.

RESULTS: Metastatic tumor cells were absent in sentinel lymph nodes by hematoxylin and eosin staining and immunocytochemistry analysis.

CONCLUSION: We describe the first reported case of staging lymph nodes in a patient with aggressive digital papillary adenocarcinoma utilizing sentinel lymph node mapping and biopsy.

Am J Surg Pathol 2000;24:775-784.
J Cutan Pathol 1987;14:129-146.
Henry JB. Clinical Diagnosis and Management by Laboratory Methods. Twentieth Edition. WB Saunders. 2001.
Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 5th Edition. McGraw-Hill. 1999.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.

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Epidemiology
Disease Associations
Pathogenesis
Laboratory/Radiologic/ Other Diagnostic Testing
Gross Appearance and Clinical Variants
Histopathological Features and Variants
Special Stains/ Immunohistochemistry/ Electron Microscopy
Differential Diagnosis
Prognosis
Treatment
Commonly Used Terms
Internet Links

PROGNOSIS AND TREATMENT	CHARACTERIZATION
Prognostic Factors
Aggressive Digital Papillary Adenocarcinoma (Aggressive Digital Papillary Adenoma and Adenocarcinoma Revisited) Wayne H. Duke, M.D.; Theresa T. Sherrod, M.D.; George P. Lupton, M.D. From the Armed Forces Institute of Pathology (AFIP), Department of Dermatopathology, Washington, DC (W.H.D., G.P.L.), and the National Naval Medical Center, Department of Pathology, Bethesda, Maryland (T.T.S.), U.S.A.	Am J Surg Pathol 2000;24:775-784 Abstract quote In 1987 a clinicopathologic study by the Armed Forces Institute of Pathology (AFIP) of rare sweat gland tumors, termed aggressive digital papillary adenoma and adenocarcinoma, was published. Since that time, the AFIP has continued to collect these tumors for study. Based on additional follow-up data, we think the original classification of these tumors requires revision. Sixty-seven cases of aggressive digital papillary adenoma and adenocarcinoma were studied according to their clinical characteristics and histologic features. Fifty of these were originally diagnosed as adenoma and 17 as adenocarcinoma. Follow up on 45 (67%) of the patients was obtained. None of the clinical or histologic parameters studied were found to be predictive of recurrence or metastasis, indicating that the originally proposed criteria for distinguishing between benign (adenoma) and malignant (adenocarcinoma) do not predict biologic behavior. When primary tumors were treated by subsequent re-excision or amputation, only one recurred (5%), when not so treated, 11 recurred (50%) regardless of the original diagnosis (p <0.05). Metastasis occurred in six (14%) cases and in three cases led to the death of the patient. Three of these metastatic cases had met the earlier criteria for adenoma. Pulmonary metastases were observed in five cases. No effective treatment for widespread metastatic disease has yet been developed. Because histologic features with prognostic significance could not be demonstrated in this retrospective review, we propose that all aggressive digital papillary tumors be designated aggressive digital papillary adenocarcinoma.
Aggressive Digital Papillary Adenocarcinoma A Case Report and Review of the Literature Debra M. Jih, etal.	Am J Dermatopathol 2001;23:154-157 Abstract quote We report a case of an aggressive digital papillary adenocarcinoma (ADPA) on the right thumb of a 48-year-old white man. Histologic evaluation of the initial biopsy demonstrated features consistent with those proposed for aggressive digital papillary adenoma; however, re-excision of the remaining lesion revealed histologic features consistent with aggressive digital papillary adenocarcinoma. These tumors have a high rate of local recurrence and can metastasize, occasionally resulting in mortality. Our case demonstrates that even if the histologic criteria of aggressive digital papillary adenoma are met, the lesion may still represent an aggressive digital papillary adenocarcinoma (ADPAca). In agreement with a recent study by Duke et al., this case supports the idea that aggressive digital papillary lesions should be classified as aggressive digital papillary adenocarcinoma.
Survival
Parameter	Percentage
Local recurrence if treated with complete excision	5% (1/21)
Local recurrence if not treated	50% (11/22)
Metastasis	14% (6/43)
Treatment	Wide excision
Sentinel node biopsy for staging of aggressive digital papillary adenocarcinoma. Malafa MP, McKesey P, Stone S, Dudley-Walker S, Cockerell CJ. Department of Surgery, Southern Illinois University School of Medicine, Springfield, Illinois 62794-9638, USA.	Dermatol Surg 2000 Jun;26(6):580-3 Abstract quote BACKGROUND: Aggressive digital papillary adenocarcinoma is a rare malignancy with a propensity for metastases and recurrence. The role of lymph node staging in this tumor is poorly defined. We describe the use of sentinel lymph node mapping and biopsy in staging this tumor. OBJECTIVE: To describe and discuss the use of lymphatic mapping in staging aggressive digital papillary adenocarcinoma. METHODS: Sentinel lymph node mapping and biopsy was performed after excision of an aggressive digital papillary adenocarcinoma of the toe. RESULTS: Metastatic tumor cells were absent in sentinel lymph nodes by hematoxylin and eosin staining and immunocytochemistry analysis. CONCLUSION: We describe the first reported case of staging lymph nodes in a patient with aggressive digital papillary adenocarcinoma utilizing sentinel lymph node mapping and biopsy.