Case 2 -

Aggressive Digital Papillary Adenocarcinoma Alireza Sepehr, Beth Israel Deaconess Medical Center, Boston, MA

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Clinical History A 32-year-old man presented with a mass on his left index finger in 1993. A biopsy was performed. Pertinent Laboratory Data: In gross pathologic examination, the specimen consisted of gray to tan-pink nodular fragments of soft tissue and measured 3.0 x 1.0 x 0.5 cm in aggregate. The cut surface revealed a multilocular appearance with semi-viscous fluid contents. Case 2 - Figure 1 Case 2 - Figure 2 Case 2 - Figure 3 Case 2 - Figure 4 Case 2 - Figure 5 Case 2 - Figure 6 Case 2 - Figure 7 Case 2 - Figure 8 Case 2 - Figure 9 Case 2 - Figure 10 Case 2 - Figure 11 Case 2 - Figure 12 Case 2 - Figure 13 Case 2 - Figure 14 Case 2 - Figure 15 Case 2 - Figure 16 Case 2 - Figure 17 Case 2 - Figure 18 Case 2 - Figure 19 Case 2 - Figure 20 Case 2 - Figure 21 Introduction: Clinical History: A 32-year-old man presented with a mass on his left index finger in 1993. A biopsy was performed. In gross pathologic examination, the specimen consisted of gray to tan-pink nodular fragments of soft tissue and measured 3.0 x 1.0 x 0.5 cm in aggregate. The cut surface revealed a multilocular appearance with semi-viscous fluid contents. The case was diagnosed in 1993 as: "Papillary eccrine adenoma, cystic variant". The tumor recured after 5 years. A wide resection was performed. In gross pathologic examination, the specimen consisted of gray to tan-pink nodular fragments of soft tissue and measured 4.0 x 1.8 x 0.8 cm in aggregate. The cut surface revealed a multilocular appearance with semi-viscous fluid contents. The diagnosis in 1998 was: "Recurrent papillary digital adenoma with progressive atypia, margins close but free, with a note stating: this recurrent lesion was seen in three separate lobules and comes within 0.1 mm of the inked margin. It is a recurrence of the lesion seen in 1993, which was reviewed. In the recurrence, there is now much nuclear hyperchromasia and although similar in structure to the original lesion, there are now more solid areas. This lesion may now be indicating evidence of aggressive behavior. It is recommended that the lesion be re-excised once more with a much more adequate margin to prevent further recurrence." The tumor recured after 2 years. A resection was performed. In gross pathologic examination, the specimen consisted of a skin ellipse measuring 0.6 x 0.3 resected to a depth of 0.3 cm. The diagnosis in 2000 was: "Papillary eccrine digital adenoma, recurrent, tumor present at inked deep margin." The tumor recured after 10 years in 2010. A resection was performed. In gross pathologic examination, the specimen consisted of a skin ellipse measuring 0.6 x 0.3 resected to a depth of 0.3 cm. Pathological/Microscopic Findings and any Immunohistochemical or Other Studies: Key Histopathologic Findings: Multinodular proliferation Solid and cystic growth pattern Grenz zone No epidermal attachment Variable circumscription Variable tubule formation Solid nests Hyalinized stroma Focal necrosis True papillary and micro-papillary structures Glandular differentiation with columnar cells Clear cell changes Apocrine secretion Squamous differentiation Variable mitotic activity Variable nuclear atypia (often low grade) Differential Diagnoses: "Aggressive digital papillary adenoma" Syringocystadenoma papilliferum Hidradenoma papilliferum Hidradenocarcinoma Eccrine spiradenocarcinoma Metastases (breast, thyroid, colon) Final Diagnosis: Aggressive Digital Papillary Adenocarcinoma Case Discussion: History: Aggressive digital papillary tumors are very rare sweat gland neoplasms first described by Helwig in 1979. Earlier rare reports by Horn (1944) and Pinkus (1954) of tumors with similar characteristics exist. Clinical Presentation: Usually, it is a single nodule near tip of the digit with/without ulceration, crusting, bleeding. The location is predominantly digital, i.e. volar aspects of fingers or toes. Mean age is in the 5-6th decade of life and they are predominantly seen in the male population (M/F = 48/5). Potential for aggressive local growth with a high recurrence rate and possibility of metastasis exist. Biologic and Clinical Behavior: In 1987 Kao et al. published a series of 57 rare sweat gland tumors from AFIP, and coined the terms: Aggressive digital papillary adenoma (ADP-A) and Aggressive digital papillary adenocarcinoma (ADP-ACA). They used the following criteria to separate these two entities: Poor glandular differentiation Necrosis Cytologic atypia Mitotic rate Invasion of soft tissue, bone, and blood vessels In 2000, Duke et al. proposed ADP-ACA is a malignant neoplasm without a benign counterpart. They studied 67 cases with ~ 6yrs follow up on 45 (67%) of the patients and showed clinical/histopathologic parameters are not predictive of recurrence or metastasis, do not distinguish between benign and malignant, and do not predict biologic behavior. In fact, the discovery of three metastatic cases originally diagnosed as adenoma prompted the study. Of 30 cases originally diagnosed at AFIP as aggressive digital papillary adenoma, 9 recurred and 3 metastasized. They proposed the term 'aggressive digital papillary adenoma' should not be used. Review of the Literature/Treatment Options (if applicable): Treatment Strategies: Wide local excision or amputation with or without sentinel lymph node biopsy are current treatment modalities. In the study by Duke et al., 50% of the patients that did not go through wide local excision and/or amputation after the initial presentation, had a recurrence. Moreover, 7/11 patients in the same "not-treated" group had a second recurrence. Our recommendation at the initial presentation is wide local excision with adequate margins or amputation. Metastatic disease was found in 6/43 patients (14%) in the study by Duke: two patients at the time of initial presentation, two patients without local recurrence, and in two patients after initial presentation accompanied by local recurrence. Metastatic disease was seen in 2/11 patients whose tumors never re-excised or amputated. In another study by Bogner et al, they studied five malignant eccrine tumors including 2 ADP- ADCs with sentinel lymph node biopsy (SLNB) performed. Four of 18 SLNs in 3 of the 5 patients (60%) were found to be positive, all with negative completion lymphadenectomy. This included 2/5 nodes in one case of ADP-ADC. Conclusion(s): Aggressive digital papillary adenocarcinoma (ADP-ACA): Is a rare tumor often in the male population and in a specific site Usually has low malignant potential Demonstrates local aggressive behavior Has potential for metastasis Is recommended to undergo local aggressive therapy (wide excision/amputation) Data on the role of sentinel lymph node biopsy are limited but may be considered in specific scenarios References: Hsu HC, Ho CY, Chen CH, Yang CH, Hong HS, Chuang YH. Aggressive digital papillary adenocarcinoma: a review. Clin Exp Dermatol. 2010 Mar;35(2):113-9. Frey J, Shimek C, Woodmansee C, Myers E, Greer S, Liman A, Adelman C, Rasberry R. Aggressive digital papillary adenocarcinoma: a report of two diseases and review of the literature. J Am Acad Dermatol. 2009 Feb;60(2):331-9. Bogner PN, Fullen DR, Lowe L, Paulino A, Biermann JS, Sondak VK, Su LD. Lymphatic mapping and sentinel lymph node biopsy in the detection of early metastasis from sweat gland carcinoma.Cancer. 2003 May 1;97(9):2285-9. Duke WH, Sherrod TT, Lupton GP. Aggressive digital papillary adenocarcinoma (aggressive digital papillary adenoma and adenocarcinoma revisited). Am J Surg Pathol. 2000 Jun;24(6):775-84. Kao GF, Helwig EB, Graham JH. Aggressive digital papillary adenoma and adenocarcinoma. A clinicopathological study of 57 patients, with histochemical, immunopathological, and ultrastructural observations. J Cutan Pathol. 1987 Jun;14 (3):129-46