1/27. Microcystic adnexal carcinoma: collaborative series review and update.BACKGROUND: Microcystic adnexal carcinoma (MAC) is a malignant appendageal tumor first described in 1982. It can be clinically and histologically confused with other malignant and benign cutaneous neoplasms, leading to inadequate initial treatment. This neoplasm is locally aggressive and deeply infiltrating, characterized by high morbidity and frequent recurrence. Mohs micrographic surgery has been used to conserve tissue and improve the likelihood for cure. OBJECTIVE: We report our experience using Mohs micrographic surgery for the treatment of MAC and compare with earlier reports in the literature. In addition, we review the epidemiology, clinical and histologic characteristics, and optimal treatment of this rare neoplasm. We also describe a 15-year-old white male patient with MAC on the scalp occurring only 7 years after radiation exposure. methods: The medical records of 11 patients with MAC who were treated by Mohs micrographic surgery were reviewed at both departments, and follow-up data were obtained. RESULTS: In all patients treated with Mohs micrographic surgery, there were no recurrences after a mean follow-up of 5 years. CONCLUSION: Mohs technique enables the detection of clinically unrecognizable tumor spread and perineural invasion often encountered with MAC. Aggressive initial treatment by microscopically controlled excision appears to offer the greatest likelihood of cure for this neoplasm, while providing conservation of normal tissue. In addition, we describe the second youngest patient with MAC and readdress the issue of previous radiotherapy as an important predisposing factor.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/27. Metastatic microcystic adnexal carcinoma in an immunocompromised patient.BACKGROUND: Microcystic adnexal carcinoma is an uncommon, locally aggressive cutaneous neoplasm. To date, there are only two reports of histologically proven lymph node involvement with this tumor. We describe a case of a patient with microcystic adnexal carcinoma who developed multiple local metastasis in transit with histologically proven lymph node involvement and was diagnosed with chronic lymphocytic leukemia. OBJECTIVE: To describe the details of our case and to review what is currently known about this tumor. methods: Mohs micrographic surgery was utilized for tumor removal. RESULTS: This patient developed multiple tumors of the scalp over the period of a 1 year which were histologically proven to be microcystic adnexal carcinoma. All tumors were noncontiguous and presented on the scalp. During the histologic analysis of the last tumor removed by Mohs micrographic surgery a lymph node was resected which revealed infiltrative microcystic adnexal carcinoma. CONCLUSIONS: We present the case of an immunocompromised patient treated for microcystic adnexal carcinoma with Mohs micrographic surgery who proceeded to develop local metastasis in transit.- - - - - - - - - - ranking = 0.25keywords = neoplasm (Clic here for more details about this article) |
3/27. Neglected microcystic adnexal carcinoma: the second reported case in a black patient.BACKGROUND: Microcystic adnexal carcinoma (MAC) is a histologically aggressive appendageal neoplasm that often pursues a beguilingly indolent clinical course. MAC has previously been reported only once in a black patient. OBJECTIVE: To present the second MAC ever recorded in a black patient. methods: We describe a MAC of substantial dimension occurring in a black patient. The literature was subsequently examined for MAC occurring in ethnic patients. RESULTS: This MAC is remarkable not only for its occurrence in a black patient, but also because it is one of the largest MACs ever recorded (8 cm), it occurs in a relatively less common site (scalp), and its latency period before diagnosis is the longest ever definitively documented (31 years). CONCLUSION: MAC is rare among non-Caucasians. Its relatively indolent clinical growth, especially in sites that are poorly accessible to patient observation, may lead to significant delay in diagnosis and treatment.- - - - - - - - - - ranking = 0.25keywords = neoplasm (Clic here for more details about this article) |
4/27. Microcystic adnexal carcinoma: First reported case in an African American man.Microcystic adnexal carcinoma (MAC) is a relatively uncommon adnexal neoplasm that can display aggressive local invasion. MAC occurs most commonly on the central part of the face and can be clinically asymptomatic with a benign appearance. We describe the first reported case of MAC in an African American man who was treated by Mohs micrographic surgery.- - - - - - - - - - ranking = 0.25keywords = neoplasm (Clic here for more details about this article) |
5/27. Microcystic adnexal carcinoma: an uncommon tumor with debatable origin.BACKGROUND: Microcystic adnexal carcinoma is an uncommon skin appendage neoplasm exhibiting both pilar and sweat duct differentiation. This tumor remains a subject of controversy as to its differentiation profile, histogenesis, and classification which is reflected in the nomenclature used to designate the neoplasm in question. Beyond this controversy the tumor remains a diagnostic challenge because of its rarity, the histologic mimicry it may display, and its banal cytologic appearance; it also poses a therapeutic challenge, as it is characterized by slow but aggressive and destructive local growth extending beyond clinical margins together with a high tendency for perineural invasion and recurrence. OBJECTIVE: We report two cases of this unusual tumor illustrating some of its characteristics. Our review emphasizes the divergent opinions concerning its differentiation profile and its origin. An organoid nevus as the origin of microcystic adnexal carcinoma in one of our patients is discussed in this context.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |
6/27. Sebaceous carcinoma, trichoblastoma, and sebaceoma with features of trichoblastoma in nevus sebaceus.A 73-year-old woman had a linear yellowish plaque on the upper part of her right ear since birth. She presented because of the sudden growth of a nodule within the plaque. The plaque was waxy and yellowish, arching around the upper part of the ear. A reddish to yellowish large nodule was seen within the central part of the arc-shaped plaque; in addition, a small pigmented nodule, a small skin-colored nodule, and a few pigmented papules were observed in the anterior half of the arched plaque. Histopathologic examination revealed the large nodule to be sebaceous carcinoma, the small pigmented nodule to be trichoblastoma, the small skin-colored nodule to be sebaceoma with the features of trichoblastoma, a few pigmented papules to be superficial trichoblastomas due to primitive follicular induction, and the linear yellowish plaque to be nevus sebaceus. Although our literature search revealed scanty reports of definite cases of sebaceous carcinoma in nevus sebaceus, the presented case demonstrated the occurrence of sebaceous carcinoma in nevus sebaceus. Malignant neoplasms occurring in nevus sebaceous seem to be extremely rare, but care should be taken when a large nodule suddenly grows in a lesion of nevus sebaceus, especially in older adults. The presented case also suggested a close relation between trichoblastoma and sebaceoma. The cytokeratin staining pattern could not distinguish between sebaceous and follicular neoplasms in our case.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |
7/27. 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 = 0.072015671918055keywords = cancer (Clic here for more details about this article) |
8/27. Recurrent polymorphous sweat gland carcinoma of the skin.Polymorphous sweat gland carcinoma is an unusual, recently described variant of low-grade malignant adnexal neoplasm of the skin characterized by a prolonged clinical course and predilection for the extremities. We describe a case of recurrent polymorphous sweat gland carcinoma in a 56-year-old man who presented with multiple large skin nodules distributed along the flexor surface of his left arm. The lesions were treated by surgical excision; multiple local recurrences, as well as the development of new lesions, were observed over a period of 5 years. No distant metastases have been observed so far. The clinical differential diagnosis and management of these unusual lesions are discussed.- - - - - - - - - - ranking = 0.25keywords = neoplasm (Clic here for more details about this article) |
9/27. Microcystic adnexal carcinoma: an unusual cause of swelling and paraesthesia of the lower lip.Microcystic adnexal carcinoma (MAC) is an uncommon, recently described, cutaneous adnexal malignant neoplasm, associated with significant morbidity as a consequence of its propensity for perineural invasion. The present report details the clinical and histological features of MAC in a young female presenting with lower labial swelling and paraesthesia.- - - - - - - - - - ranking = 0.25keywords = neoplasm (Clic here for more details about this article) |
10/27. Identification of a recurrent mutation in the CYLD gene in Brooke-Spiegler syndrome.Brooke-Spiegler syndrome is an autosomal dominantly inherited disease with predisposition to neoplasms of the skin appendages. The disease has been mapped to 16q, and mutations in the CYLD gene have been identified in families with this disorder. We describe an individual with BSS exhibiting clinical heterogeneity in which a heterozygous frameshift mutation in CYLD, 2172delA, has been identified. These findings extend the body of evidence that mutations in CYLD are involved in Brooke-Spiegler syndrome and provide additional information for phenotype-genotype correlation.- - - - - - - - - - ranking = 0.25keywords = neoplasm (Clic here for more details about this article) |
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