Abstract:A 51-year-old man with microcystic adnexal carcinoma of the face is reported. In addition to extensive soft tissue invasion by the cancer, there was direct bone invasion into the marrow of the mandible as well as perineural spread along the mental and inferior alveolar nerves. This represents the first case of microcystic adnexal carcinoma with documented bone invasion. Recognition of the aggressive nature of this cancer with potential for invasion into the skull is important for proper evaluation and treatmen… Show more
“…The total recurrence rate after traditional excisional surgery is estimated to be 40–59%, after a long-term follow-up [2, 5, 8, 9]. The review of Friedman et al [5], however, represents the largest and longest group of monitored patients treated by Mohs surgery with no recurrences after an average follow-up of 5 years.…”
Section: Discussionmentioning
confidence: 99%
“…Microcystic adnexal carcinoma (MAC) is an aggressive, locally destructive cutaneous neoplasm with a high rate of recurrence [1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11], which is said to occur predominantly in Caucasians. However, the number of cases in Japanese subjects seems to have recently been increasing.…”
Background: Microcystic adnexal carcinoma (MAC) has been reported to occur predominantly in Caucasians. However, the number of cases in Japanese subjects seems to have recently been increasing. Objective: To review the Japanese cases of MAC. Methods: We collected and analyzed the data of 51 Japanese patients recorded in case reports or abstracts over a number of years. Results: Twenty-five of the cases were reported between 1987 and 1996, and the remaining 26 cases between 1997 and 2000. Forty-three lesions were located on the face, 2 on the scalp, 2 on the chest, 1 on the axilla, 1 on the buttock, 1 on the palm and 1 on the toe. One of the above occurred at the site of previous radiotherapy. Fifteen tumors were initially misdiagnosed histopathologically. Conclusion: Our review shows that MAC also occurs in the Japanese, in whom it may be more common than previously observed.
“…The total recurrence rate after traditional excisional surgery is estimated to be 40–59%, after a long-term follow-up [2, 5, 8, 9]. The review of Friedman et al [5], however, represents the largest and longest group of monitored patients treated by Mohs surgery with no recurrences after an average follow-up of 5 years.…”
Section: Discussionmentioning
confidence: 99%
“…Microcystic adnexal carcinoma (MAC) is an aggressive, locally destructive cutaneous neoplasm with a high rate of recurrence [1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11], which is said to occur predominantly in Caucasians. However, the number of cases in Japanese subjects seems to have recently been increasing.…”
Background: Microcystic adnexal carcinoma (MAC) has been reported to occur predominantly in Caucasians. However, the number of cases in Japanese subjects seems to have recently been increasing. Objective: To review the Japanese cases of MAC. Methods: We collected and analyzed the data of 51 Japanese patients recorded in case reports or abstracts over a number of years. Results: Twenty-five of the cases were reported between 1987 and 1996, and the remaining 26 cases between 1997 and 2000. Forty-three lesions were located on the face, 2 on the scalp, 2 on the chest, 1 on the axilla, 1 on the buttock, 1 on the palm and 1 on the toe. One of the above occurred at the site of previous radiotherapy. Fifteen tumors were initially misdiagnosed histopathologically. Conclusion: Our review shows that MAC also occurs in the Japanese, in whom it may be more common than previously observed.
“…The deeper component has smaller nests and strands of cells in a hyalinized stroma, leading to a scirrhous appearance. 6,7,13,16,17,35,38 Immunohistochemical staining plays a less important role in the diagnosis of MAC, but staining for CEA-positive tumor cells can improve the accuracy of tumor margin determination, especially in cases with extensive perineural infiltration, 8,13,17,23,28 and it can help to differentiate MAC from other adnexal tumors. 1 The most important histopathologic differential diagnoses of MAC are sclerodermiform basal cell carcinoma and desmoplastic trichoepithelioma.…”
Microcystic adnexal carcinoma (MAC) belongs to the spectrum of locally aggressive adnexal carcinomas and most commonly occurs in the head and neck region. Recently it has been proposed that MAC is an apocrine tumor. It is characterized by slow, but locally aggressive growth with infiltration of subcutaneous fat tissue, muscles, perichondrium, periosteum, or perineurium. As a result, surgical treatment often leads to ablation defects, which are many times the size of the clinical lesion. In 1982, Goldstein and colleagues first reported MAC to be a distinct histologic entity characterized by a combination of keratinous cysts in the upper dermis, islands and strands of small basaloid, benign-appearing keratinocytes or squamous cells in the deeper dermis within a dense desmoplastic stroma, and areas of ductular differentiation. The authors report the case of a 78-year-old woman in whom a diagnosis of MAC was made when a tumor on the right cheek recurred for the second time. Previous histopathologic diagnoses were squamous cell carcinoma and desmoplastic trichoepithelioma. Local recurrences of the tumor occurred, despite histographic surgery because in hematoxylin and eosin stains, small islands of the deceptively benign-appearing small basaloid cells of MAC were not recognized as tumor cells. The reported case demonstrates the difficulties in diagnosing MAC and indicates that MAC should always be considered in the differential diagnosis of slowly growing tumors in the head and neck region. If MAC is diagnosed too late, it can be inoperable because of its infiltrative growth.
“…MAC may have a highly aggressive local course with the ability of direct bone invasion [21], PNI [10, 13, 21, 28, 29] and/or intraneural extension [28, 29]. However, some cases of regional lymph node metastasis have been reported.…”
Section: Discussionmentioning
confidence: 99%
“…Tumors exhibiting PNI are considered as more aggressive, with higher rates of morbidity and mortality, especially in the head and neck area [13, 20]. In addition, involvement of the subcuticular tissue, muscle and bone may occur [13, 21, 22]. MAC exhibits an immunophenotype that is a hybrid of those seen in pure sweat glandular and follicular neoplasms (reactivity for hard keratin subclasses AE13 and AE14, epithelial membrane antigen, carcinoembryonic antigen and Leu-M1).…”
Background: Microcystic adnexal carcinoma (MAC) is a rare cutaneous neoplasm, with a high rate of local recurrences. Objective: A series of MAC was analyzed and compared to previously published cases. Methods: Seven cases of MAC were identified in the register of the institution. Medical and pathological records were reviewed. Results: The primary MAC were located on the face in all patients, and 85% were initially misdiagnosed. The mean follow-up duration was 108 months. The recurrence rate was high: 4 patients developed recurrences. In 3 patients, the course of the disease was severe: one of them developed pathologically proven lung metastasis. Conclusion: The present study and review of the literature confirm the clinically aggressive evolution of MAC and its rare ability to give rise to metastasis. Long-term clinical follow-ups with imaging investigations are mandatory.
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