Reactive eccrine syringofibroadenoma arising in peristomal skin: An unusual presentation of a rare lesion.

We report the third case of eccrine syringofibroadenoma (ESFA) arising in peristomal skin. A 55-year-old man presented with a 15- x 10-cm pale pink verrucous, exophytic, intermittently tender plaque involving his ileostomy site. He had undergone proctocolectomy with ileostomy creation 33 years prior for ulcerative colitis. The clinical differential diagnosis included granulomatous dermatitis, infection (fungus or atypical mycobacterium), or neoplasm. A punch biopsy specimen was performed and showed ESFA. Although ESFA is considered to be benign, recent reports have demonstrated an association of ESFA with malignancy or malignant transformation of ESFA. Furthermore, ESFA and reported cases of ileostomy carcinoma share similar clinical symptoms at presentation including pain, irritation, ulceration, bleeding, and the presence of a fungating mass. The lesion was, therefore, excised in toto and the excisional specimen showed no evidence of malignancy. We speculate that ESFA is a reaction to chronic irritation and, analogous to other long-standing reactive processes such as lichen sclerosis or burn scar ulcers, may be associated with malignant transformation. Because of this possibility and the clinical overlap with ileostomy carcinoma, peristomal ESFA should be treated with complete excision. If it is not amenable to complete excision because of lesion size or anatomic complexity, generous sampling and close clinical follow-up are recommended.

Post-radiotherapy vascular proliferations in mammary skin: a clinicopathologic study of 11 cases.

BACKGROUND: Post-radiotherapy atypical vascular lesions (AVL) in mammary skin show significant clinical and histopathologic overlap with well-differentiated angiosarcoma (AS) and pose a considerable diagnostic and managerial challenge when encountered. OBJECTIVE: We review Stanford's experience with diagnosing AVL and formulate a clinicopathologic approach to these lesions. METHODS: We performed a clinicopathologic study on 11 cases that were initially diagnosed as AVL and examined whether there are specific clinical or histopathologic features that delineate AVLs from well-differentiated AS. RESULTS: Clinically, all patients were women with a mean age of 68.1 years, had a history of infiltrating breast carcinoma, and were treated by excision with postoperative radiation therapy. All lesions were located in mammary skin within the prior radiation field. The clinical presentation included erythema, telangiectasias, papules, plaques, and nodules. All patients were diagnosed with AVL on initial biopsy. Six patients showed no recurrence or progression of disease following incomplete excision with no further therapy (3/6) or re-excision with negative margins (3/6). The remaining 5 patients were shown to have AS in the re-excision specimen. The patients diagnosed with AS were older and had a shorter interval from radiation as compared to those who did not experience an adverse outcome. Histologically, all initial biopsy specimens were transected and were characterized by complex, anastomosing vascular proliferations with dilated spaces. Each case was morphologically evaluated according to the AVL criteria of Fineberg and Rosen. Three cases met all of the criteria for AVL, and these patients showed no progression of disease. The remaining cases met most but not all diagnostic criteria for AVL and showed some features of AS, but fell short of a definitive diagnosis of AS, including the 5 cases that were subsequently diagnosed as angiosarcoma. LIMITATIONS: This retrospective study utilized a small number of cases from a single consultation service; therefore, some inherent selection bias may exist. CONCLUSION: We could not identify unequivocal clinical or histologic criteria that allows for a sharp separation between AVL and AS. Dermatologists and pathologists need to be aware of the overlap between AVL and well-differentiated AS and all patients who receive a diagnosis of AVL should undergo complete excision with close clinical follow-up and biopsy of any new lesions.

Cutaneous peripheral T-cell lymphoma associated with a proliferation of B cells.

Although the new World Health Organization-European Organization for Research and Treatment of Cancer classification focuses on providing uniformity in the diagnosis of cutaneous lymphomas, cutaneous peripheral T-cell lymphoma (PTL) remains a poorly defined subgroup. As follow-up to a study of systemic PTL complicated by a proliferation of B cells, we studied 16 cases of cutaneous PTL that contained morphologically atypical T cells associated with a significant infiltrate of B cells (about 20%-50%). A clonal T-cell receptor gamma chain gene rearrangement was present in all cases. In contrast, a clonal immunoglobulin heavy chain gene rearrangement was present in only 1 case. Clinical staging in 14 cases identified systemic involvement in 2. At last follow-up, both patients with systemic involvement had died of disease, and the majority of patients with primary cutaneous disease were alive (11/12). The presence of numerous atypical B cells and T cells caused diagnostic confusion in these cases. Comprehensive pathologic studies, coupled with clinical staging, are necessary for the accurate diagnosis of this unusual manifestation of cutaneous PTL.