1/681. Detection of human papillomavirus type 10 dna in eccrine syringofibroadenomatosis occurring in Clouston's syndrome. Syringofibroadenomatosis is often associated with an underlying condition such as diabetes mellitus or hidrotic ectodermal dysplasia. By reason of these associations, a reactive or hamartomatous cause is suspected. We report a case of a 71-year-old woman with Clouston's syndrome in whom progressive multiple palmoplantar syringofibroadenomas developed over a 10-year period. The syringofibroadenomas formed flat-topped papules simulating verruca plana; the widespread distribution and chronic progressive course resembled epidermodysplasia verruciformis. Contiguous with the syringofibroadenoma's characteristic epithelial-stromal proliferation were epidermal changes of verruca plana. Evidence of human papillomavirus (HPV) infection was verified by immunolabeling with antibodies to bovine papillomavirus type 1 and detection of HPV 10 viral dna by means of polymerase chain reaction. Rather than a hamartomatous process, these findings suggest that syringofibroadenomas occurring in the setting of Clouston's syndrome could represent an HPV-induced epithelial proliferation. ( info) |
2/681. Apocrine poroma: a distinctive case in a patient with nevoid basal cell carcinoma syndrome. Traditionally, poromas have been classified as eccrine neoplasms, but several recent reports of poroid tumors with sebaceous, follicular, and apocrine differentiation have challenged this idea. In support of alternative differentiation, a case of an "apocrine" poroma is reported in a 19-year-old man with the nevoid basal cell carcinoma syndrome. A papule on the right cheek, thought clinically to be a basal cell carcinoma, was excised. Anastomosing lobules of small uniform basaloid (poroid) cells formed small ductular structures lined by eosinophilic cuticles and extended into the superficial reticular dermis. The neoplasm originated from follicular infundibula and was surrounded by a myxoid stroma. Focally, primitive hair bulb and papillae differentiation was present, and some of the ducts were lined by cells suggesting decapitation secretion. The histologic pattern and the common embryologic origin of the folliculosebaceous-apocrine unit support apocrine differentiation of this tumor. The association with the nevoid basal carcinoma syndrome appears to be unique. This case, in addition, demonstrates overlapping features with the infundibulocystic type of basal cell carcinoma commonly seen in the basal cell nevus syndrome. ( info) |
3/681. A reactive acrosyringeal proliferation in a patient with ectodermal dysplasia: eccrine syringofibroadenoma-like lesion. A 33-year-old man with ectodermal dysplasia (ED) has suffered from keratotic, exudative, erythematous plaques on the genital area, thighs, and soles since age 17. Verrucous soft nodules in a cobblestone arrangement developed on the erythematous plaque on his left thigh when he was 31 years old. Histologic examination of the verrucous nodules demonstrated that they were composed of anastomosing thin cords of uniform, cuboidal, epithelial cells and a fibrovascular stroma. The changes are indicative of eccrine syringofibroadenoma of Mascaro (ESFA), which has been reported as a neoplasm, a hamartoma, or a nevus. With etretinate treatment, the verrucous nodules completely disappeared within two months. Similar, but much flatter, verrucous lesions recurred and disappeared twice during the subsequent two years period. These verrucous lesions were likely induced by irritation from urine, stool, and/or mechanical friction. This case of ESFA in a patient with ED clearly showed a reactive process which was successfully managed with oral etretinate. ( info) |
4/681. Acanthotic type of eccrine poroma on the arm: report of a case. A 72-year-old Japanese man with eccrine poroma on the arm is described. To the best of our knowledge, he is the fifth patient with this tumor on the arm reported from japan. The histopathological type of the tumor of our patient was unique because it was acanthotic; those of the previous patients were all of the intradermal type. ( info) |
5/681. Malignant eccrine spiradenoma: a previously unreported presentation and review of the literature. Malignant eccrine spiradenomas (MESs) are rare tumors arising from previously benign eccrine spiradenomas. A review of the literature reveals only 25 published reports of malignancy arising from eccrine spiradenoma and no prior reports of an MES of the scalp. The tumors have a metastasis rate of >50 per cent in reported cases with high resultant mortality rates. We present the first case report of a single MES of the scalp. Multiple resections were required for local control. Both magnetic resonance imaging and lymphoscintigraphy were used to assess regional spread. A review of the literature follows to include histopathology, diagnosis, and both surgical and adjuvant therapeutic options. ( info) |
6/681. Papillary transitional cell carcinoma of the breast: a report of five cases with distinction from eccrine acrospiroma. Papillary carcinomas of the female breast exhibit a spectrum of morphologic appearances and might be mistaken for benign intraductal papillary lesions or papillary adnexal neoplasms. We report herein five cases of papillary carcinoma in which the epithelium closely resembled transitional cells of the urinary bladder. Grossly, the tumors had a nodular or papillary appearance, white, tan, or red in color. The microscopic features were those of an intraductal papillary proliferation of solid layers of epithelial cells overlying fibrovascular cores. The proliferating cells assumed a whorled or streaming growth pattern, with flattening of superficial cells. One case showed microinvasion. Comparison with a similar number of cases of the solid variant of papillary carcinoma of the breast showed a greater range of nuclear pleomorphism, mitotic counts, and a more varied immunohistochemical profile in the papillary carcinomas with transitional cell features. Eight cases of eccrine acrospiroma occurring in the female breast also displayed a solid or solid papillary pattern, with flattened superficial cells. These occurred in a younger age group, were located in the dermis or subcutis, and usually had zones of clear cells visible at low magnification. No evidence of recurrent or metastatic disease was found in the four patients for whom follow-up was available; the length of follow-up ranged from 18 months to 11 years. The stimulus for the development of this unusual phenotype is unclear, but the transitional-like variant seems to behave in a fashion similar to that of other types of papillary carcinoma of the breast. Distinction of this malignant lesion from various benign lesions that occur in the same region is mandatory. ( info) |
7/681. MRI appearance of clear cell hidradenoma. We present the first reported MR imaging findings of a histologically proven clear cell hidradenoma. A fluid level was noted on all pulse sequences in this lesion, which demonstrated a prominent hemorrhagic component on sectioning. The presence of an enhancing nodule was also noted, differentiating this lesion from a post-traumatic hematoma. Fluid levels in a well-defined subcutaneous soft tissue mass should suggest the possibility of a hidradenoma. ( info) |
8/681. Apocrine gland carcinoma of the axilla: review of the literature and recommendations for treatment. Apocrine gland carcinoma is a rare form of sweat gland neoplasm with a distinctive cytologic appearance. Although the region of the axilla remains the most common site for these tumors, apocrine gland carcinoma of the anogenital region, eyelid, ear, chest, wrist, lip, foot, toe, and finger have been reported. Classically, these slow-growing lesions present as painless, colorless or reddish, firm or cystic nodules. More than half of the reported patients with apocrine carcinoma had lymph node metastases at the time of diagnosis. Wide local excision is standard therapy for these lesions. A therapeutic lymph node dissection is indicated for confirmed lymph node metastases and may have a role in the setting of a large or highly aggressive tumor with narrow surgical margins. Although apocrine gland carcinoma responds poorly to chemotherapy, adjuvant radiotherapy may be used in advanced local or regional disease. The authors describe a 69-year-old man with a large recurrent apocrine gland carcinoma of the axilla treated with en bloc excision with axillary dissection and offer a pertinent review of the English literature and recommendations for treatment. ( info) |
9/681. Sclerosing sweat duct carcinoma of the eyelid margin: unusual presentation of a rare tumor. OBJECTIVE: Sclerosing sweat duct carcinoma (SSDC) is a rare, slow-growing, locally invasive skin tumor of eccrine and pilar origin. It is usually located on the face, particularly the upper lip, cheek, and forehead. It has been infrequently reported on the eyelid, secondarily involved from adjacent cheek and brow tumors. Only four previous cases have reported primary eyelid tumors. The authors present four cases of primary eyelid margin involvement, which show the variability in clinical presentations. DESIGN: Retrospective case series. The authors present four case studies of lower eyelid margin tumors diagnosed as SSDC. PARTICIPANTS/methods: The history of this recently recognized neoplasm is discussed in relation to the cases presented and the role of the ophthalmologist and pathologist in such cases. RESULTS: Primary SSDC of the eyelid margin is a reportedly rare entity. This particular presentation can occur in all age groups; can mimic benign, acanthotic, or basal cell-like tumors; and is usually misdiagnosed initially. This can lead to a delay in definitive treatment for a tumor that classically presents late in its natural history to health professionals. All eight cases of primary eyelid SSDC now reported in the literature have occurred in the lower lid. CONCLUSION: This rare but aggressive tumor is difficult to diagnose from a simple biopsy and may be more common than previously believed. Initial or early diagnosis is important because of unusually invasive characteristics. recurrence is common and usually leads to extensive tissue loss via direct invasion or subsequent wide resection. Correct histologic diagnosis at the time of initial tumor removal will likely aid in achieving complete excision with fewer recurrences. ( info) |
10/681. Extensive naevoid eccrine spiradenoma. We describe a 19-year-old girl with a painful naevoid eccrine spiradenoma affecting the right side of the body. This represents an extremely rare variant of this benign eccrine sweat gland tumour, and is the most extensive lesion described in the U.K. to date. ( info) |