1/46. Paget's disease of the vulva associated with local adenocarcinoma and previous breast adenocarcinoma: report of two cases.We report two women in whom vulval Paget's disease occurred in association with local adenocarcinoma and previous breast adenocarcinoma. The first patient presented at the age of 83 years with moist erythematous changes over the perineum and an indurated area near the anus. biopsy of the indurated area showed Paget's cells throughout the epidermis and, below, adenocarcinoma infiltrating the dermis. Ten years previously, she had undergone a left mastectomy for infiltrating ductal carcinoma of the breast. The second patient was diagnosed as having Paget's disease at the age of 74 years. A vulval biopsy showed Paget's cells in the epidermis but, in addition, there were changes suggestive of adenocarcinoma of the sweat glands. Her symptoms of vulval itching had started at the age of 45 years and had led to a simple vulvectomy at the age of 57 years. Retrospective review of this vulvectomy specimen showed Paget's disease. She had also previously been treated for infiltrating ductal adenocarcinoma of the breast and adenocarcinoma of the rectum. The management of Paget's disease is difficult because of its high recurrence rate and, as illustrated by our two cases, treatment is difficult if the patients are elderly and in poor general health.- - - - - - - - - - ranking = 1keywords = epidermis (Clic here for more details about this article) |
2/46. Malignant hidroacanthoma simplex: a case report.Hidroacanthoma simplex is a benign tumor of the skin originating from or showing differentiation to the sweat glands. It grossly resembles seborrheic keratosis of bowen's disease and histologically shows intraepidermal focal growth of epithelial cells. Malignant transformation of this tumor is rare. We report a case of pigmented hidroacanthoma with malignant transformation in a 67-year-old woman. There was a 20-year history of a skin lesion on the right thigh, which first appeared as a small verrucous papule, progressed to a dark-brown colored patch, and then to a pigmented plaque. Histologically, the primary tumor was composed of small squamoid cells with marked cellular atypia. Most of the tumor cells were located in the epidermis. Immunohistochemically, the cytoplasm of some tumor cells showed a positive reaction for epithelial membrane antigen, but not for either carcino-embryonic antigen or the S-100 protein.- - - - - - - - - - ranking = 0.5keywords = epidermis (Clic here for more details about this article) |
3/46. Apocrine acrosyringeal keratosis in association with syringocystoadenoma papilliferum.We report the clinical and histopathological features of a keratosis that developed in association with syringocystadenoma papilliferum. This tumour shows a pinkish, pedunculated, spherical nodule with a cerebriform surface and visible keratinous plugs. In addition to the typical features of syringocystadenoma papilliferum, the tumour shows many hyperkeratotic columns surrounded by acanthotic epidermis with the characteristics of trichilemmal keratinization and keratohyalin granules. This keratosis seems to be derived from the middle to lower portion of the apocrine acrosyringium, based on the distribution of keratohyalin granules and the direct connection with the apocrine acrosyringium in an early lesion. Accordingly, we propose to identify this rare keratosis as apocrine acrosyringeal keratosis.- - - - - - - - - - ranking = 0.5keywords = epidermis (Clic here for more details about this article) |
4/46. Excision of multiple eyelid apocrine hidrocystomas via an en-bloc lower eyelid blepharoplasty incision.A 39-year-old man presented with upper and lower eyelid apocrine hidrocystomas that had recurred after each of three prior attempts at excision. These multiple, cystic tumors were adherent to the epidermis, thus precluding complete dissection and excision of each individual lesion. We report a surgical technique using a lower eyelid blepharoplasty incision to remove the confluent tumors of the lower eyelids en-bloc. We paid careful attention to both removing the lesions without rupturing the cysts and to achieving adequate depth of excision. histology revealed features typical of apocrine hidrocystomas. The patient has remained free of recurrence three years since the en-bloc excision of the lesions.- - - - - - - - - - ranking = 0.5keywords = epidermis (Clic here for more details about this article) |
5/46. Extramammary Paget disease with underlying hidradenoma papilliferum: guilt by association?Extramammary Paget disease (EMPD) is a heterogenous entity representing either an intraepidermal adenocarcinoma in situ with apocrine differentiation or an expression of underlying malignancy of the skin or of the intestinal or genitourinary tract. The coexistence of EMPD with a benign underlying hidradenoma papilliferumn (HP) is, however, exceptional. We present the case of a 79-year-old woman with diffuse and patchy gray-white lesions involving her left vulva as well as an underlying 0.7-cm asymptomatic firm nodule. Histologically, the epidermis and dermis showed features characteristic of EMPD and HP, respectively. Malignant transformation in HP giving rise to EMPD in the overlying epithelium has been reported. In our case, however, failure to demonstrate continuity between the two lesions together with the lack of cytologic atypia, mitoses, and necrosis in the HP lends additional support to the possibility that the HP is "innocent" and that its association with EMPD is thus coincidental. A common histogenetic derivation of these two lesions from the mammary-like glands or from related germinative cells in the epidermis is suggested to explain this rare presentation.- - - - - - - - - - ranking = 1keywords = epidermis (Clic here for more details about this article) |
6/46. An unusual melanocytic lesion associated with eccrine duct fibroadenomatosis and syringoid features.The intimate association of nevomelanocytic nevi with eccrine ducts commonly seen in congenital nevi was emphasized by Mishima, who described as eccrine-centered nevi those lesions characterized by nevomelanocytic cells predominantly proliferating around and within the eccrine sweat duct walls. However, there were no changes in the overlying epidermis, dermis, or eccrine acrosyringeal or dermal duct proliferation in these lesions. We present the case of a 16-year-old boy with a 1-year-history of a 0.6-cm diameter single tan papule on the right heel, clinically thought to be a Spitz nevus. Histopathologic examination revealed a compound nevomelanocytic nevus associated with epidermal hyperplasia, thin anastomosing cords of acrosyringeal epithelium extending within the dermis, and eccrine ductal proliferation in a syringoma-like pattern associated with a dense fibrous stroma. Features that distinguish our case from eccrine-centered nevus are that the latter lacks epidermal and eccrine duct hyperplasia and a dense fibrous stroma. The location of the lesion on the heel in our case suggests the possibility that the pathologic changes observed could result from repetitive trauma.- - - - - - - - - - ranking = 0.5keywords = epidermis (Clic here for more details about this article) |
7/46. Metastatic eccrine porocarcinoma: response to docetaxel (Taxotere) chemotherapy.BACKGROUND: eccrine porocarcinoma is an uncommon neoplasm of the intra-epidermal sweat gland duct. patients AND methods: A case of eccrine porocarcinoma in a female renal transplant patient aged 45 years is described with a review of pertinent literature. RESULTS: The primary tumour was highly pleomorphic. In places large and small cells merged and focally the former component infiltrated the epidermis in a manner akin to Paget's disease of the breast. The majority of the tumour was high grade; using the modified Bloom and Richardson grading system, usually applied to mammary ductal carcinomas, the tumour graded as 3. Metastatic disease developed nine months following primary surgical treatment. The metastatic eccrine porocarcinoma was resistant to epirubicin but responded to docetaxel chemotherapy. CONCLUSIONS: There are no data to support the use of adjuvant therapy in the management of eccrine porocarcinoma. The use of the modified Bloom and Richardson grading system may define cases at high risk of relapse in which adjuvant therapy might be considered. Metastatic eccrine porocarcinoma has proven resistant to many chemotherapeutic agents. We report the first use of docetaxel in the management of this disease. The treatment was well tolerated and resulted in marked symptomatic and radiological responses. Treatment with docetaxel should be considered in future cases of this rare tumour.- - - - - - - - - - ranking = 0.5keywords = epidermis (Clic here for more details about this article) |
8/46. Clear-cell porocarcinoma in situ: a cytologic variant of porocarcinoma in situ.Poromas are benign neoplasms composed of poroid and cuticular cells. Four histopathologic variants of poromas are accepted, according to the architectural features of the neoplasm: hidroacanthoma simplex or intraepidermal poroma; eccrine poroma, which is a poroma connected to the epidermis that extends to superficial dermis; dermal duct tumor, which develops when the neoplasm is composed of small, solid aggregations of poroid and cuticular cells confined to the dermis with little or no connection with the epidermis; and poroid hidradenoma, which is a solid-cystic, dermal poroma. The malignant counterpart of hidroacanthoma simplex is named malignant hidroacanthoma simplex or porocarcinoma in situ. This report describes an example of clear-cell malignant hidroacanthoma simplex, a cytologic variant of porocarcinoma in situ, which, to our knowledge, has not been previously reported. In contrast with other clear-cell neoplasms, a relation with diabetes mellitus could not be clearly established in this case.- - - - - - - - - - ranking = 1keywords = epidermis (Clic here for more details about this article) |
9/46. Eccrine nevus presenting as a perianal skin tag: a case report and review of the literature.We present the case of a 9-year-old girl with a perianal skin tag. This asymptomatic lesion was removed for cosmetic reasons and demonstrated a polypoid lesion with a slightly acanthotic epidermis. The dermis was filled with mature-appearing eccrine ducts and glands but with no other cutaneous appendages, adipocytes, or abnormal vascularity. We believe this to represent a polypoid eccrine nevus and that its presentation is unique.- - - - - - - - - - ranking = 0.5keywords = epidermis (Clic here for more details about this article) |
10/46. Carcinomatous transformation of eccrine syringofibroadenoma.BACKGROUND: While squamous cell carcinoma and pseudocarcinomatous hyperplasia have been documented as pre-existing lesions in cases of reactive eccrine syringofibroadenoma (ESFA), to the best of our knowledge carcinoma occurring in a solitary ESFA has not yet been reported. We present one such case in a 91-year-old female who had a dome-shaped, reddish tumor on the extensor side of the left forearm. methods: We review the histopathological, immunophenotypical and ultrastructural findings of this tumor, including the keratin expression profile. RESULTS: Histopathologically, long, branching, anastomosing, thin and thick strands of small cuboidal epithelial cells were extending from the surface epidermis into the dermis. In the center of the tumor, there were irregular-shaped nests of atypical tumor cells invading downward into the dermis. Ultrastructurally, duct-like lumina lined with cuboidal tumor cells were present in the epithelial cords. From these findings, the present case was diagnosed as solitary eccrine syringofibroadenocarcinoma (ESFAC). Keratin expression studies revealed that cells of the thick strands, except for the luminal and basal cells, were positive for differentiation-specific keratins, keratins 1 and 10, and that cells of the thin strands were positive for keratins 5 and 14. CONCLUSIONS: Histopathological, immunophenotypical and ultrastructural evidence, as well as the pattern of keratin expression, suggest differentiation of the present malignant tumor towards the eccrine dermal duct. This case is the first reported case of ESFAC as far as we know.- - - - - - - - - - ranking = 0.5keywords = epidermis (Clic here for more details about this article) |
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