Cases reported "Sweat Gland Neoplasms"

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1/155. 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.
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2/155. 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.
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3/155. 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.
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4/155. 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.
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5/155. 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.
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6/155. Poroid hidradenoma. Report of a case with cytologic findings on fine needle aspiration.

    BACKGROUND: Fine needle aspiration cytology (FNAC) can be used for establishing a diagnosis of cutaneous lesions, especially in cases with cyst formation. Poroid hidradenoma is eccrine neoplasm with both solid and cystic components. CASE: A 77-year-old female presented with a slightly elevated nodule in the skin on her left elbow. The tumor was well demarcated, 2.7 x 2.4 cm and soft, and overlying skin was slightly reddish. FNAC revealed two types of cell: one had abundant cytoplasm in which small to large, occasionally multinucleated nuclei with small but distinct nucleoli were evident. chromatin was finely granular, and nuclear membrane was thin and almost smooth. Another type of cell had scanty cytoplasm and a round to oval nucleus with small but prominent nucleoli. Histologic diagnosis was poroid hidradenoma. CONCLUSION: FNAC can be useful for diagnosing intradermal cystic lesions before surgical resection.
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7/155. Spiradenocylindromas of the skin: tumors with morphological features of spiradenoma and cylindroma in the same lesion: report of 12 cases.

    Twelve cases of spiradenocylindromas, which revealed features of both spiradenoma and cylindroma in the same tumor mass, are presented. Nine female patients had multiple neoplasms occurring mostly on the scalp, and two female and one male patient had a solitary cutaneous lesion. Three of the female patients with multiple cutaneous tumors had a familial history of similar cutaneous neoplasms. In one of the patient's family, the multiple cutaneous tumors were known to occur in multiple family members in four consecutive generations. One patient with multiple cutaneous lesions was known to have associated multiple kidney cysts as confirmed by computed tomography. Histologically, spiradenocylindromas are composed of intermixed areas that are either of typical spiradenoma in appearance or of typical cylindroma appearance. Apocrine and trichoepitheliomatous differentiation seen in two cases in the present series points to spiradenomas, as well as cylindromas, having complex hair follicle (folliculo-sebaceous apocrine) rather than eccrine differentiation. The presence of lymphoid tissue was a histological feature in the present series, which was prominent in all the spiradenomatous parts of the tumors and which was scanty or practically absent in all the cylindromatous parts. The selective presence of lymphocytes in spiradenoma and an absence in cylindroma suggest that spiradenomas have the unique property of attracting lymphocytes. The malignant tumors arising in three patients in the present series had the morphology of a poorly differentiated epithelioid neoplasm. Three patients died of the disease and the other patients were either free of disease or alive with disease 1-30 years on follow up.
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8/155. Malignant eccrine poroma in an elderly African-American woman.

    BACKGROUND: eccrine porocarcinoma is a rare, slow-growing malignant tumor arising from the eccrine sweat gland. OBJECTIVE: We present a case of eccrine porocarcinoma of the ear in an elderly African-American woman. Cases of eccrine porocarcinoma have been reported in the literature occurring in facial, extremity, scalp, and genital sites and primarily in Caucasian patients. Rarely are cases described in African-American patients or on the ear. methods: We describe the history of the lesion, discuss the current cancer statistics for skin cancer in african americans, and histology and treatment choices are compared with previously reported cases. RESULTS: We present a case of malignant eccrine poroma of the ear in a 71 year old African-American woman. The tumor was completely excised by mohs surgery. Transposition flap repair of the Mohs surgical defect was performed. CONCLUSIONS: While african americans are at low risk of developing skin cancers, it is important to remember that skin cancers can affect patients of any ethnicity and skin tone. Treatment regimens for skin cancers in African-American patients must be chosen appropriately by site of tumor, size of tumor and extent of disease as would be expected for any skin cancer.
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keywords = cancer
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9/155. Spiradenoma arising in a nevus sebaceus of Jadassohn: case report and literature review.

    Nevus sebaceus (NS) of Jadassohn is usually a verrucous plaque on the scalp or face that arises secondary to disordered development of epithelial, pilar, sebaceous, and apocrine structures. The emergence of neoplasia is a late stage in the natural history of NS. Although most neoplastic proliferations are benign, several malignant tumors have arisen in this lesion. We describe the first case of a benign spiradenoma arising in an NS on the scalp in a 72-year-old Caucasian woman. Reexcision was recommended to prevent the development of a second neoplastic process and to avoid the rare occurrence of a malignant transformation of the existing neoplasia. The patient declined reexcision and remains under observation. The spectrum of tumors arising in NS are described and are categorized according to behavior. Syringocystadenoma papilliferum is the most commonly observed benign growth, whereas basal cell carcinoma is the most frequently seen malignant process. The signs of tumor development (benign or malignant) within an NS are reviewed, and treatment recommendations are provided. The clinical course of rare and unique aggressive neoplasms originating in NS is summarized.
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10/155. eccrine porocarcinoma treated with Mohs micrographic surgery: A report of five cases.

    BACKGROUND: eccrine porocarcinoma is a rare, locally aggressive, potentially fatal neoplasm. While wide local excision has traditionally been the treatment of choice, recurrences following excision are common. OBJECTIVE: The purpose of this study was to review the traditional treatments of eccrine porocarcinoma as well as to introduce Mohs micrographic surgery as an alternative to wide local excision. methods: We reviewed all cases of eccrine porocarcinoma seen at Emory University between 1985 and 1999. All cases were treated definitively with Mohs micrographic surgery. The clinical characteristics and outcome of each case are summarized. RESULTS: Five patients with eccrine porocarcinoma were treated with Mohs micrographic surgery. There have been no recurrences to date, with an average follow-up of 2.1 years (ranging from 5 months to 4 years). CONCLUSION: Follow-up of five patients supports the view that Mohs micrographic surgery may be an effective treatment for eccrine porocarcinoma.
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