1/12. Melanocytic matricoma: a report of two cases of a new entity.Many reports exist of pigmented adnexal tumors containing dendritic melanocytes such as pigmented basal cell carcinomas and pigmented pilomatricomas. Correspondingly, melanocytes are a known component of the bulbs of anagen follicles. The phenomenon of melanization of adnexal tumors highlights the interrelationship between melanocytes and adnexal epithelium and may represent normal melanocytes colonizing a neoplastic proliferation. We report on two cases of a unique tumor composed of neoplastic matrical cells with a significant component of melanocytes. Both cases presented as pigmented papules in older men (66 and 80 years, forearm and pectoral region, respectively). Histologically, these were well-defined nodular proliferations composed of variably melanized, pleomorphic, and mitotically active matrical and supramatrical cells forming clusters of "shadow cells." Admixed with the epithelial cells were numerous melanized dendritic melanocytes. Shadow cells expressed keratin 13, and a subpopulation of S-100 protein-positive dendritic cells were evident. No recurrence of any type was found after reexcisions 4 months and 2 years later. We propose the name of melanocytic matricoma for these two heretofore unreported cases of a unique neoplasm composed of matrical cells and melanocytes recapitulating epithelial-melanocyte interaction in the follicular anagen bulb. Although their small size, circumscription and clinical course suggest a benign nature, melanocytic matricomas' cytologic atypia disclose the potential for malignant behavior.- - - - - - - - - - ranking = 1keywords = carcinoma (Clic here for more details about this article) |
2/12. Trichilemmal carcinoma in an African American.BACKGROUND: Trichilemmal carcinoma is an uncommon cutaneous neoplasm found primarily on the sun-exposed skin of the elderly. OBJECTIVE: To report a case of trichilemmal carcinoma presenting as a forehead nodule in an African American patient. methods: Case report and review of the literature. RESULTS: Our patient is the first reported case in the English language literature of trichilemmal carcinoma in an African American individual with skin phototype VI. CONCLUSION: The occurrence of trichilemmal carcinoma in our African American patient suggests the diagnosis of trichilemmal carcinoma be added to the differential diagnosis of a nodule on the head region in this patient population.- - - - - - - - - - ranking = 9keywords = carcinoma (Clic here for more details about this article) |
3/12. Multiple familial trichoepitheliomas: a folliculosebaceous-apocrine genodermatosis.We reviewed the pathologic findings on a family with multiple hereditary trichoepitheliomas. Although the majority of the lesions were trichoepitheliomas, basal cell carcinomas, spiradenomas, and spiradenomas with cylindromatous foci (spiradenocylindroma) were present, representing a spectrum of lesions exhibiting folliculosebaceous (trichoepithelioma, basal cell carcinoma) and apocrine (spiradenoma, spiradenocylindroma) differentiation. Multiple familial trichoepitheliomas may be a syndrome whereby tumors develop from undifferentiated germinative cells of the folliculosebaceous-apocrine unit. Published findings regarding the genetics of this syndrome and solitary trichoepitheliomas are reviewed; although the molecular basis for the tumors has yet to be determined, current data suggest that a tumor suppressor gene may be involved.- - - - - - - - - - ranking = 2keywords = carcinoma (Clic here for more details about this article) |
4/12. Trichogerminoma: a rare cutaneous adnexal tumor with differentiation toward the hair germ epithelium.In 1992, Sau and colleagues described 14 cases of a rare cutaneous adnexal tumor with differentiation toward the hair germ epithelium. All cases in their study displayed a unique constellation of histological features which allowed the authors to consider the neoplasm to be a distinct entity and to designate it as 'trichogerminoma' (TG). We present a case of an adnexal tumor identical to that described as TG and report the immunophenotypical features of this neoplasm for the first time. A biopsy from a 41-year-old man revealed a well-demarcated epithelial tumor composed of multiple lobules located in the deep dermis without connection to the overlying epidermis and surrounded by a fibrous pseudocapsule. The tumorous lobules were composed of uniform basophilic cells with vesicular nuclei, dispersed chromatin and prominent nucleoli showing no prominent peripheral palisading. Some of the lobules had the appearance of densely packed 'cell balls' with peripheral condensation of the tumor cells. The stroma showed a moderate number of fibroblasts and mast cells. reticulum staining revealed fine reticulum fibers surrounding the tumor aggregates with accentuation of the 'cell balls'. Immunohistochemically, the tumor cells expressed AE1/AE3, CK5/8, CK5/6, CAM5.2 and stained negatively for CK20, CK7, calretinin, Lu-5 and Thomsen- Friedensreich antigen. There was no increase in the numbers of CK20-positive merkel cells in the epidermis overlying the tumor; however, a few merkel cells were scattered in some tumor lobules. In addition, we stained 6 trichoblastomas (TBs) and found a particular pattern of calretinin expression in this tumor which was not observed in our case of TG. We conclude that pathological features allow the delineation of TG as a distinct adnexal neoplasm. Histological differential diagnosis includes basal-cell carcinoma (pilar type), large nodular TB, trichoblastic fibroma, trichoepithelioma, tricholemmoma, pilomatricoma and matrical carcinoma.- - - - - - - - - - ranking = 2keywords = carcinoma (Clic here for more details about this article) |
5/12. The effect of radiation therapy on microcystic adnexal carcinoma: a case report.BACKGROUND: Microcystic adnexal carcinoma (MAC) is a rare, locally aggressive tumor. Treatment for this neoplasm typically requires extensive local excision leading to morbidity. Therefore, the temptation to use alternative treatment options remains high. methods: We report one case of a nasal dorsum MAC treated with external beam radiation secondary to the patient's poor health status and preference. RESULTS: After initial dramatic clinical resolution, the tumor recurred in a clinically more extensive and histologically more aggressive form. CONCLUSION: On the basis of this case and several detailed in the literature, we therefore hypothesize that radiation therapy is not only an ineffective treatment for MAC, but evidence exists that this modality may induce conversion to a histologically and clinically less favorable neoplasm.- - - - - - - - - - ranking = 5keywords = carcinoma (Clic here for more details about this article) |
6/12. Melanocytic matricoma: case confirmation of a recently described entity.BACKGROUND: In 1999, Carlson et al. reported two cases of a matrical neoplasm that recapitulates the bulb of the anagen hair follicle, which they designated as melanocytic matricoma. methods: Here we report a similar case in a 78-year-old white male, who presented with a 0.4 cm purple-black firm papule in the left preauricular area. RESULTS: Histologically, the tumor is composed of a dual cell population including admixed epithelial matrical and supramatrical cells with "shadow" cell formation and pigmented dendritic melanocytes. Immunohistochemical studies for cytokeratin highlighted the epithelial component and studies for S-100 protein, HMB-45, and vimentin confirmed the melanocytic component. The differential diagnosis considered includes pigmented variants of pilomatricoma, matrical carcinoma, basal cell carcinoma and malignant melanoma. CONCLUSIONS: The case reported herein is the first confirmation of melanocytic matricoma, a distinctive adnexal neoplasm with characteristic clinical and pathologic features, which differentiate it from pigmented pilomatricoma.- - - - - - - - - - ranking = 2keywords = carcinoma (Clic here for more details about this article) |
7/12. External auditory canal eccrine spiradenocarcinoma: a case report and review of literature.BACKGROUND: Eccrine spiradenocarcinoma is a rare dermal appendage carcinoma believed to arise from transformation of a long-standing benign spiradenoma. This tumor demonstrates highly malignant biologic behavior with a high recurrence rate, frequent lymph node metastases, and overall poor survival. methods: We report the first case of eccrine spiradenocarcinoma arising in the external auditory canal. The management of this tumor, its histopathologic characteristics, and a review of literature are presented. RESULTS: A literature review identified 17 cases of eccrine spiradenocarcinoma in the head and neck region. Local recurrence occurred in 58.8% of patients, with an average of 23 months from diagnosis. lymph node metastasis occurred in 35.3%, with an average of 31 months from diagnosis. Other metastatic sites included skin, bone, and lung. Disease-specific mortality was 22.2%. CONCLUSIONS: Eccrine spiradenocarcinoma is an aggressive tumor with a poor prognosis. Primary treatment should include wide local excision with or without regional lymphadenectomy. Isolated successful treatments have been documented with adjuvant hormonal manipulation, chemotherapy, and radiation therapy.- - - - - - - - - - ranking = 9keywords = carcinoma (Clic here for more details about this article) |
8/12. Low-grade trichoblastic carcinosarcoma of the skin.We present an unusual case of cutaneous carcinosarcoma with the epithelial component closely resembling nodular basal cell carcinoma, and the mesenchymal component composed of cells constituting extended follicular papillae. A solitary tumor was excised in an 80-year-old man. Histologic sections revealed an ulcerated, asymmetric, poorly circumscribed neoplasm composed of epithelial cells arranged in lobules with peripheral palisading or in a cribriform pattern. The epithelial cells were darkly basophilic with scant cytoplasm and round or oval nuclei with an indistinct chromatin pattern and nucleoli. Nuclei crowding and mitotic figures were observed. Some lobules contained melanin. There were no shadow cells, sebaceous or apocrine glandular differentiation. Each epithelial nodule was surrounded by multiple rows of cells with pale vesicular nuclei and scant cytoplasm. Smaller epithelial aggregations were encircled by these cells concentrically; in larger ones these cells were aligned across a broad front resembling so-called "continuous papillae". Additionally, numerous small follicular germ-like structures associated with papillae were seen. The cells composing "continuous papillae" showed nuclear pleomorphism, numerous mitotic figures including atypical ones, and nuclear crowding. At foci, the transition from the multilayered arrangement of these cells into their diffuse proliferation in the stroma was seen. There were no transitions between the epithelial and stromal component; both were intermingled as though being mutually dependent, with no areas revealing a high-grade tumor or dedifferentiation. Immunohistochemically, the epithelial cell component stained with cytokeratins. The cells of the mesenchymal component tested positive for vimentin and negative for desmin and cytokeratins. The proliferation index (Ki-67) was high in both components. There were also a high number of p53-positive cells in both compartments. We propose the term "low-grade trichoblastic carcinosarcoma" for this neoplasm. We are not aware of a similar tumor published in the English literature.- - - - - - - - - - ranking = 1keywords = carcinoma (Clic here for more details about this article) |
9/12. Malignant transformation within benign adnexal skin tumours.AIMS: To report five malignant trichogenic tumours arising in longstanding, previously benign adnexal neoplasms through malignant transformation. Malignant trichogenic adnexal tumours are extremely rare neoplasms. methods AND RESULTS: The patients were between 55 years and 79 years of age. Three of the tumours were located on the arms, two on the face. Three of our patients had a history of chronic lymphocytic leukaemia, one patient had a history of colonic adenocarcinoma. The duration of the tumour nodules was reported as between 20 and 40 years before sudden changes occurred. These changes included rapid growth, pain, itching, ulceration and bleeding. Histologically, all tumours were well circumscribed and encapsulated. There was a residual benign tumour component and morphological signs such as bone formation, dystrophic calcification and sclerosis suggesting long duration of the lesions. All patients except for one, who refused further clinical investigation due to her advanced age of 79 years, had an underlying systemic malignancy. CONCLUSIONS: The growth stimulus in these benign adnexal neoplasms resulting in malignant transformation may be attributed to the acquisition of additional genetic events or to immunosuppression due to an underlying neoplastic disease. Therefore, patients with systemic diseases or malignancy should be carefully examined and followed for sudden changes in pre-existing benign cutaneous tumours.- - - - - - - - - - ranking = 1keywords = carcinoma (Clic here for more details about this article) |
10/12. Cutaneous carcinosarcoma: adnexal vs. epidermal types define high- and low-risk tumors. Results of a meta-analysis.OBJECTIVE: We report four cases of cutaneous carcinosarcoma (CS) and perform a meta-analysis of the cutaneous CS literature. RESULTS: CS occurred in elderly patients (mean of 80 years) on sun-damaged skin, and were keratotic papules of short duration. They did not recur after excision. CS exhibited basal cell carcinoma mixed with atypical fibroxanthoma cell populations. immunophenotyping revealed vimentin /keratin- spindle cells and vimentin-/keratin epithelial cells. Three cases exhibited p53 protein expression of both carcinomatous and sarcomatous components. literature review identified 38 cases of cutaneous CS that could be broadly classified into two distinct groups. Epidermal-derived (basal or squamous cell carcinoma epithelial component) CS arose on the sun-damaged skin of the head and neck of elderly males (mean age 72 years) and had a 70% 5-year disease-free survival. In contrast, adnexal CS (spiradenocarcinoma, porocarcinoma, proliferating tricholemmal cystic carcinoma, or matrical carcinoma) occurred in younger patients (mean age 58 years), showed recent growth in a long-standing nodule and had a 25% 5-year disease-free survival. Age less than 65 years, recent growth, long-standing skin tumor, and tumor size greater than 2 cm significantly correlated with poor outcome. CONCLUSIONS: Cutaneous CS is an aggressive skin cancer with high risk for advanced disease. Significant risk factors exist whose identification will allow for better management of CS patients.- - - - - - - - - - ranking = 7keywords = carcinoma (Clic here for more details about this article) |
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