1/6. diagnosis and management of uncommon cutaneous cancers.Uncommon types of cutaneous cancers are mainly cited in the literature as case reports and their etiology, pathogenesis and prognosis have to be surmised because of their rarity. Within this group exist the rare and also the unusual, for instance a relatively common carcinoma arising in a strange circumstance. Initial management of such tumors involves taking a history and performing a thorough examination, allowing a diagnosis to be made. These tend to follow one of three patterns: the lesion is confidently recognized; the lesion is unknown but a likely diagnosis can be made and; the lesion is unknown. It is within the latter two groupings that the uncommon cutaneous cancers exist. A biopsy is then performed to confirm the diagnosis. For large lesions a punch or incisional biopsy is taken which must include a portion of normal skin at the lesion edge. If the lesion is small enough to allow direct closure, an excision biopsy is performed, with a minimum margin of 2mm. Shave biopsy can be employed to confirm a diagnosis, but care must be taken that the subsequent management of the lesion is not adversely affected. With the rare tumors diagnosis can be difficult and there may not be enough tissue in a biopsy for a definitive diagnosis. The whole lesion may therefore need to be excised to obtain a confident diagnosis with further surgical treatment planned as required. Once the diagnosis is established a decision on the method of treatment can be made. The limited literature would suggest that rare skin tumors are unresponsive to radiotherapy and chemotherapy, so the mainstay of treatment is surgery. When there are no established guidelines for the treatment of a particular rare tumor a pathologist can usually provide advice as to the probable nature of the lesion. This allows surgical treatment to be positioned into one of three main groups: lesions that behave like basal cell carcinomas (BCC); lesions that behave like squamous cell carcinomas (SCC) and; lesions that behave like soft tissue sarcomas (STS). It is helpful to have a management plan into which each variety of rare tumor can be fitted, giving guidance as to the best and most appropriate management. Grouping treatment into BCC, SCC or STS-like treatment has been useful. As more becomes known regarding these malignancies their subsequent management can be adjusted accordingly. Currently, it would appear wise to treat each under a broader subgroup.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/6. Microcystic adnexal carcinoma of the breast: a very rare breast skin tumor.BACKGROUND: Microcystic adnexal carcinoma is a rare, slowly progressing, malignant tumor of sweat gland origin. OBJECTIVE: A case of microcystic adnexal carcinoma of the breast with a history of 20 years is presented. methods: On initial examination, the ulcerative cutaneous mass was fixed to the underlying breast tissue and chest wall. Both the macroscopic appearance of the tumor and its location suggested a glandular breast carcinoma. However, an incisional biopsy revealed the nature of the tumor as microcystic adnexal carcinoma of the breast skin. Following the diagnosis, a wide excision of the tumor was carried out for the final treatment. RESULTS: To our knowledge this is the first case of microcystic adnexal carcinoma arising in the breast skin presented in the literature. CONCLUSION: This case demonstrates that microcystic adnexal carcinoma can occur on the breast skin and should be treated with wide excision due to its locally aggressive behavior.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/6. Microcystic adnexal carcinoma involving a large portion of the face: when is surgery not reasonable?BACKGROUND: We report a case of microcystic adnexal carcinoma (MAC) involving a large portion of the face, one of the largest of any MAC reported thus far in this area, and review the literature regarding the nature of the tumor and available treatments. We also review all of the reported cases of metastases and the possible role of radiation in the etiopathogenesis of this tumor. OBJECTIVE: To review the literature about what is known about therapy for MAC and what options are available to patients who have this disease. MATERIALS AND methods: Case report and review of the literature. RESULTS: Of the 274 cases of MAC thus far reported, there are 6 cases of metastases, only 1 of which resulted in death. CONCLUSION: mohs surgery should be the treatment of choice for this tumor; however, when extirpation entails sufficiently large morbidity, given the low rate of metastases and mortality, observation is a reasonable alternative.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/6. bowen's disease with invasive adnexal carcinoma: the pluripotential nature of bowen's disease cells.bowen's disease rarely exhibits multiple combinations of premalignant and/or malignant skin lesions. bowen's disease with invasive adnexal carcinoma was originally described by Kao, but is not well recognized by clinicians due to its rarity and lack of specific clinical features of this condition. Herein, we describe three unusual cases of bowen's disease with invasive adnexal carcinoma. The two distinct neoplastic areas exhibited continuity both clinically and histologically. The plaque lesions possessed clinical features typical of bowen's disease. In cases 1 and 3, we confirmed the adnexal tumor within tumors of bowen's disease, the diagnosis of which is eccrine porocarcinoma. The tumor in case 2 was characteristic to trichilemmal carcinoma. Immunohistochemically, the tumor cells of bowen's disease and the adnexal carcinoma differed in antigenicities. The present cases support a notion that bowen's disease maintains a pluripotential nature.- - - - - - - - - - ranking = 5keywords = nature (Clic here for more details about this article) |
5/6. Microcystic adnexal carcinoma: management options based on long-term follow-up.Microcystic adnexal carcinoma (MAC), a recently described neoplasm that frequently affects the head and neck, presents a confusing problem for the clinician due to its unusual behavior. The individual cells have a bland microscopic appearance, and there is a predilection for neural invasion. Four cases of MAC are reported. All four cases demonstrate the difficulty with pathologic diagnosis. Follow-up of as long as 33 years begins to delineate the protracted nature of MAC. In addition, this paper includes the first report of a case of lymph node metastasis. Although resection may result in a significant defect, negative margins may not be achieved. Despite this, the defect can heal, as demonstrated by the cases described. In addition, MAC may recur many years later, irrespective of the status of the margins at the time of surgery. Given these unusual characteristics and the slowly progressive nature of MAC, strong consideration must be given to less radical surgical procedures, with close follow-up for grossly recurrent disease.- - - - - - - - - - ranking = 2keywords = nature (Clic here for more details about this article) |
6/6. Microcystic adnexal carcinoma.Microcystic adnexal carcinoma (MAC) is a rare adnexal tumour which has only recently been recognized as a separate clinicopathological entity. It typically affects the face of the middle-aged and often requires extensive surgical excision, due to its locally invasive nature. Its clinical significance is that, despite being locally invasive, MAC is typified by a lack of metastatic spread. We present a case and review of the literature.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |