Microcystic Adnexal Carcinoma Research Articles

Microcystic Adnexal Carcinoma: Review of 51 Japanese Patients

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.

Microcystic Adnexal Carcinoma of the Vulva

Background. Microcystic adnexal carcinoma (MAC) is a subset of sweat gland carcinoma first described as a specific entity by D. J. Goldstein, R. J. Barr, and D. J. Santa Cruz (Cancer 1982;50:566–72). We report the first case of MAC occurring on the vulva and review the literature pertaining to this rare tumor.Case. A 43-year-old multiparous black woman presented initially to Kings County Hospital Medical Center with a chief complaint of a vulvar lesion arising on the left labia majora which she had noted for 4 years prior to presentation. Aside from increasing paresthesia in the area, she denied any constitutional symptoms. Her past medical history was significant only for hyperthyroidism and mild hypertension and surgical history was noncontributory. Gynecologic history was unremarkable, with sporadic care over the last 20 years. Physical examination revealed a 1.5 × 2.0-cm raised, well-circumscribed, firm mobile lesion on the left labia majora. It was noted to be yellow in color with the surrounding tissue being unremarkable in character. The remainder of her gynecologic examination and lymph node survey was unremarkable. Preoperative chest X ray was negative as was the CAT scan of the abdomen and pelvis. All laboratory values were within normal limits. A Pap smear done preoperatively was significant for atypical squamous and glandular cells of undetermined significance. Subsequent colposcopic examination of the cervix was remarkable for cervicitis and was adequate, with the entire transformation zone visualized. Both endocervical curettage and endometrial biopsy were normal. Initially, an excisional biopsy was performed with final pathology demonstrating microcystic adnexal carcinoma with positive surgical margins. She subsequently underwent a left radical hemivulvectomy with bilateral inguinal groin lymph node dissection. At the time of surgery, the left labia majora was noted to be well healed, with a residual surgical scar easily discernible. No areas of discoloration were noted and digital palpation of the area was unremarkable. Microscopic residual tumor was noted; however, all surgical margins and lymph nodes were negative for tumor. Her postoperative course was unremarkable. The patient has continued to do well since the time of her surgery and is being followed conservatively.Conclusion. Radical vulvectomy should be performed when MAC occurs in the vulva to secure negative margins of resection. Groin dissection should be reserved for cases in which the inguinal lymph nodes are clinically suspicious or in cases of tumor recurrence.

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. (J Am Acad Dermatol 2001;45:283-5.)

Microcystic Adnexal Carcinoma-- Mohs Surgery vs. Simple Excision

Microcystic adnexal carcinoma is a relatively rare skin cancer that is often invasive and extensive and that is frequently accompanied by perineural

Microcystic Adnexal Carcinoma

Microcystic adnexal carcinoma, or sclerosing sweat duct carcinoma, is an uncommon cutaneous neoplasm associated with extensive local invasion. The standard of care with regard to the best excisional method in treating microcystic adnexal carcinoma has not been established. To perform a retrospective study comparing patients treated by Mohs micrographic surgery with those treated by wide excision and to elucidate the epidemiological features of microcystic adnexal carcinoma. A retrospective analysis of a case series involving 48 primary and referral patients diagnosed as having microcystic adnexal carcinoma using standardized criteria. All cases were reviewed by the same dermatopathologists. Microcystic adnexal carcinoma predominantly affects the left side of the face of middle-aged women. Microcystic adnexal carcinoma is misdiagnosed 30% of the time. The recurrence rate is 1.98% per patient-year. Mohs micrographic surgery and simple excision show comparable complication rates. Clear margins were obtained in fewer procedures and, therefore, fewer office visits when the lesions were treated with micrographic surgery. The defect surface area after full extirpation following Mohs micrographic surgery was a mean of 4 times that of the clinically apparent size. The wide range of difference between the pre- and the post-Mohs micrographic surgery surface area noted in our data indicates that a margin cannot be safely predicted. Microcystic adnexal carcinoma is a predominantly left-sided, locally aggressive facial tumor, which results in significant morbidity. Our data do not support the use of standardized predictable margins. Mohs micrographic surgery is a reasonable initial treatment, as it accomplishes cure in fewer office visits and does not rely on predicted margins.

Merkel cells and sclerosing epithelial neoplasms.

Merkel cells are normal constituents of the basal layer of the epidermis and the follicular epithelium. They have been identified in benign neoplasms with follicular germinative differentiation but seem to be absent in basal cell carcinomas (BCCs). Because sclerosing epithelial neoplasms are often sampled by small biopsies, any method that enables distinction among them would be welcome. We used immunohistochemical staining for cytokeratin 20 to assess the presence of Merkel cells in 14 cases of desmoplastic trichoepithelioma (DTE), 12 specimens of syringoma, 11 samples of morpheiform BCC, and 8 specimens of microcystic adnexal carcinoma (MAC). Merkel cells were found in association with all 14 specimens of DTE and in 1 of 11 cases of morpheiform BCC (p < 0.005) but in none of the specimens of syringoma or MAC. Our study supports previous findings that Merkel cells are seen in association with cutaneous neoplasms that are benign and of a follicular germinative origin. Although MAC may differentiate along follicular-sebaceous-apocrine lines, the absence of Merkel cells within it is consistent with its malignancy. The identification of Merkel cells in a sclerosing epithelial neoplasm of the skin points to DTE as the most likely diagnosis.

Microcystic adnexal carcinoma induced by multiple radiation therapy

We present an 84‐year‐old Caucasian man (Fitzpatrick classification: skin type II) with microcystic adnexal carcinoma (MAC) on his left cheek and a 15‐year history of recurrent squamous cell carcinoma (SCC) of the head, treated with numerous surgical interventions and multiple palliative 60‐Gy radiation therapy.In 1996, the patient developed a nontender, indurated, irregularly marked, erythematous lesion on his left cheek (1.5 × 1 cm). Furthermore, the patient suffered from radiodermatitis due to previous radiotherapy ( Fig. 1). Punch biopsy and a subsequent wedge excision showed features of both SCC and eccrine carcinoma. Histopathologic and immunohistochemical tests of the tumor revealed a diagnosis of MAC. The patient underwent Mohs' micrographically controlled surgery to obtain tumor‐free peripheral soft tissue margins. There was no evidence of any lymphatic invasion or distant metastasis in the physical and laboratory examination. So far, the patient has not developed any recurrences.Clinical picture of MAC showing features of radiodermatitisimageFollowing excision, the biopsy specimen (4 × 2.6 × 1 cm) was fixed in Dubosq‐Brazil and routinely processed for staining with hematoxylin and eosin. The neoplasm extended into the subcutaneous tissue with rare connection to the overlying epidermis ( Fig. 2). Follicular cysts with amorphous eosinophilic keratin and comma‐like tails of aggregates of epithelial cells were a prominent feature ( Fig. 3). Perineural invasion was evident, but there were only a very few mitotic figures. Immunohistochemistry on paraffin‐embedded tissue blocks was performed, showing strong labeling on dilated ducts for carcinoembryonic antigen (CEA), epithelial membrane antigen (EMA) in the lumina of tumor cells, and positive staining for anticytokeratin KL1 (55–57 kDa) and S‐100 protein. The latter was negative in ductal structures, but stained dendritic cells.Panoramic view of the tumor demonstrating poorly circumscribed and deeply infiltrative growth patterns. Rare connection of the tumor with epidermal structures is evident (hematoxylin and eosin, × 40)imageComma‐like tails of aggregates of epithelial cells and follicular cysts with amorphous eosinophilic keratin (hematoxylin and eosin, × 150)image

DNA image cytometry in malignant and benign sweat gland tumours.

The histopathological differentiation between well-differentiated carcinomas and atypical adenomas of sweat gland origin may be difficult, even if immunohistochemical methods are used. Therefore, additional techniques may be helpful. We previously demonstrated that DNA image cytometry (ICM-DNA) can be useful in distinguishing between malignant and benign clear cell hidradenoma. In the present study, a larger series of sweat gland tumours, with a clear-cut diagnosis as malignant or benign on histopathological criteria, was examined by ICM-DNA. Enzymatic cell separation specimens were prepared from paraffin-embedded tissues of 18 sweat gland carcinomas (14 porocarcinomas, one classic eccrine adenocarcinoma, two microcystic adnexal carcinomas and one mostly ductal apocrine carcinoma) and 47 benign sweat gland tumours (three syringocystadenomas, five spiradenomas, 14 cylindromas, three syringomas, seven nodular hidradenomas, 10 cutaneous mixed tumours, four poromas and one apocrine hidrocystoma). Specimens were examined by ICM-DNA according to the current recommendations of the European Society for Analytical Cellular Pathology with the AutoCyte QUIC-DNA workstation using mesenchymal cells as an internal reference. DNA aneuploidy was detected by the stemline interpretation according to Böcking and/or at least three 5[c]-exceeding events. DNA aneuploidy was detected in 16 of 18 (89%) of the sweat gland carcinomas, but in none of the 47 adenomas. These results suggest that the detection of DNA aneuploidy in sweat gland tumours using ICM-DNA is a clear and specific indicator of prospective malignancy.

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. (J Am Acad Dermatol 1999;41:225-31.)

Author's Reply: Sebaceous Differentiation in Microcystic Adnexal Carcinoma

Author's Reply: Sebaceous Differentiation in Microcystic Adnexal Carcinoma

Sweat Gland Carcinoma: A Clinicopathologic Analysis of an Expanded Series in a Single Institution

Primary adenocarcinoma of sweat glands is a rare tumor; approximately 220 cases have been reported in the last 30 years. We reviewed the charts of patients with primary diagnosis of this tumor treated at the Mayo Clinic between 1935 and 1995. We included only cases with initial histology slides available for re-examination. Tumors were classified into five recognizable histologic patterns (solid, ductal, mucinous, microcystic adnexal, and adenocystic carcinoma) and graded by the Broder system. Statistical analysis consisted of Kaplan-Meier product limit method and Cox multiple regression test. In total, 55 patients were identified, and age ranged from 13 to 85 years (mean 59 years). Thirty-six patients (65 percent) presented to the Mayo Clinic for initial treatment; all except one had disease limited to the primary site. Microcystic adnexal carcinoma was the most frequent type, and more than 50 percent were grade 2 tumors. Among these 36 patients, 4 had some type of recurrence. Patients who developed metastasis had a high-grade tumor in the initial biopsy. Nineteen patients were referred with recurrence; 13 had local recurrence, 4 had regional diseases, and 2 had distant metastases. The histologic distribution showed 47 percent solid tumors, and 37 percent of them were grade 3. Multiple regression analysis did not show a difference in recurrence or survival when gender, age, tumor location, or histologic pattern was evaluated. In addition, there was no difference in the outcome between wide surgical resection and micrographic surgery. The only predictive factor for distant metastases and/or death (p < 0.003) was histologic grade. Overall 10-year survival rate was 86 and 60 percent for primary and referred patients, respectively. We conclude that histologic diagnosis of sweat gland carcinoma must be complemented by clinical examination to evaluate metastases. Clinical behavior depends on the histologic type of tumor, degree of differentiation, and clinical stage. On recurrence, the likelihood of further recurrences and mortality increases dramatically. Aggressive initial local ablation with tumor-free margins is recommended. In high-grade tumors, prophylactic regional lymph node dissection may further characterize tumor aggressiveness and may justify adjuvant radiotherapy as part of the primary treatment. (Plast. Reconstr. Surg. 102: 705, 1998.)

Sweat gland carcinoma: a clinicopathologic analysis of an expanded series in a single institution.

Primary adenocarcinoma of sweat glands is a rare tumor; approximately 220 cases have been reported in the last 30 years. We reviewed the charts of patients with primary diagnosis of this tumor treated at the Mayo Clinic between 1935 and 1995. We included only cases with initial histology slides available for re-examination. Tumors were classified into five recognizable histologic patterns (solid, ductal, mucinous, microcystic adnexal, and adenocystic carcinoma) and graded by the Broder system. Statistical analysis consisted of Kaplan-Meier product limit method and Cox multiple regression test. In total, 55 patients were identified, and age ranged from 13 to 85 years (mean 59 years). Thirty-six patients (65 percent) presented to the Mayo Clinic for initial treatment; all except one had disease limited to the primary site. Microcystic adnexal carcinoma was the most frequent type, and more than 50 percent were grade 2 tumors. Among these 36 patients, 4 had some type of recurrence. Patients who developed metastasis had a high-grade tumor in the initial biopsy. Nineteen patients were referred with recurrence; 13 had local recurrence, 4 had regional diseases, and 2 had distant metastases. The histologic distribution showed 47 percent solid tumors, and 37 percent of them were grade 3. Multiple regression analysis did not show a difference in recurrence or survival when gender, age, tumor location, or histologic pattern was evaluated. In addition, there was no difference in the outcome between wide surgical resection and micrographic surgery. The only predictive factor for distant metastases and/or death (p < 0.003) was histologic grade. Overall 10-year survival rate was 86 and 60 percent for primary and referred patients, respectively. We conclude that histologic diagnosis of sweat gland carcinoma must be complemented by clinical examination to evaluate metastases. Clinical behavior depends on the histologic type of tumor, degree of differentiation, and clinical stage. On recurrence, the likelihood of further recurrences and mortality increases dramatically. Aggressive initial local ablation with tumor-free margins is recommended. In high-grade tumors, prophylactic regional lymph node dissection may further characterize tumor aggressiveness and may justify adjuvant radiotherapy as part of the primary treatment.

Unusual cutaneous malignancies

The most commonly occurring cutaneous malignancies are basal cell and squamous cell carcinoma. There are, however, other rare malignancies that are encountered and should be included in the differential, in which both the clinical and the histological diagnosis may be difficult. Here, the clinical and histological features, management, and prognostic factors of merkel cell carcinoma, microcystic adnexal carcinoma, leiomyosarcoma, dermatofibrosarcoma protuberans, and angiosarcoma are reviewed.

１６年間追跡したＭｉｃｒｏｃｙｓｔｉｃ Ａｄｎｅｘａｌ Ｃａｒｃｉｎｏｍａ １６年間追跡しているＭｉｃｒｏｃｙｓｔｉｃ Ａｄｎｅｘａｌ Ｃａｒｃｉｎｏｍａの１難治例

Microcystic adnexal carcinoma (MAC) is an aggressive, locally destructive cutaneous neoplasm with a high rate of recurrence. This tumor is often misdiagnosed clinically and histologically. The authors described again a 54-year-old woman with a large MAC, which had already been reported in 1988, focusing on 16-year follow-up and treatments of similar, uncontrollable cases in the literatures. The initial biopsy specimen must be large enough to demonstrate the identifying histologic features. Debulking of tumor for gross recurrences in the face may be a viable option in certain patients, depending on their overall health and the extent of deformity resultant from total tumor extirpation.

臨床的浸潤範囲と組織学的浸潤範囲に大きな差が認められたＭｉｃｒｏｃｙｓｔｉｃ ａｄｎｅｘａｌ ｃａｒｃｉｎｏｍａの１例

A 51-year-old male was referred to our clinic in November, 1996, because of slowly progressive indurated erythematous plaque with smooth, shiny surface and small teleangiectasia for 3 years duration. In microscopic examination, the tumor cells were present in the whole dermis and showed basaloid tumor strands with inmature eccrine differentiation. This biopsy specimen was interpreted as microcystic adnexal carcinoma (MAC), and the tumor was excised with 5mm tumor-free-margin in accord to previous mapping biopsy by 4mm trephine punch. But the margin of resection were involved tumor cells in deeper dermis and subcutis. Additional wide resection was performed with 5mm wider margin and the tumor was completely excised.MAC is invasive in deeper and pathological invasion much differs from clinical apperance.

Cytogenetic and fluorescence in situ hybridization analyses of a microcystic adnexal carcinoma with del(6)(q23q25)

Cytogenetic analysis of a case of microcystic adnexal carcinoma (MAC) revealed three unrelated clones with the karyotypes: 46,XX,del(6)(q23q25)[17]/46,XX,1∼2dmin[4]/46,XX,t(1;3) (p10;q10)[2]. Analysis of the 6q- by fluorescence in situ hybridization (FISH) showed that it had resulted from a long arm deletion. The finding of a clonal 6q deletion as the sole karyotypic change in a MAC is of special interest because we previously have identified 6q deletions in different types of malignant salivary gland tumors. This observation, together with the histopathologic similarities between salivary gland tumors and sweat gland tumors, further emphasize the histogenetic relationships between these tumor types.

Microcystic Adnexal Carcinoma with Extensive Sebaceous Differentiation

We report two cases of microcystic adnexal carcinoma showing extensive sebaceous differentiation. Multiple cellular nests and strands within a moderately sclerotic stroma involving the full thickness of the dermis were observed. Clusters of basaloid cells with extensive sebaceous differentiation were present. Foci of sebaceous ductal differentiation were observed in the more superficial areas. Neither strikingly atypical cells nor mitotic figures were present. Perineural invasion was present in the deep areas of both tumors. Clinically, the lesions were solitary whitish-pink papules with a central dell on the faces of 2 men (aged 78 and 73 years old). We propose a relationship between these tumors and other cytologically bland but locally aggressive adnexal carcinomas. Sebaceous differentiation itself in a poorly circumscribed neoplasm does not indicate conventional extraocular sebaceous carcinoma. We propose a simple classification of locally aggressive adnexal carcinomas that takes into account the full range of adnexal differentiation that can occur in such lesions.

Ｍｉｃｒｏｃｙｓｔｉｃ ａｄｎｅｘａｌ ｃａｒｃｉｎｏｍａの１例

We reported a case of microcystic adnexal carcinoma occuring on the right upper lip of a 51-year-old man. Microscopically, small solid nests and strands of tumor were distributed throughout the whole dermis with infiltration into subcutaneous tissue. In the upper dermis, there were small cysts of varying size. In the middle dermis, there were small ductal structures or tadpole-shaped epithelial islands.Immunohistochemically, CEA was focally positive some ductal structures. S-100 protein stain was negative. 34β E12 was positive in all three components of the tumors. 34 β H11 was moderately positive in some cysts and ductal structures. CA19-9 and EMA were positive in most cysts and ductal structures.These observations confirm that this tumor appeared at least capable of eccrine sweat gland differentiation.

Excision of selected skin tumours using Mohs' micrographic surgery with horizontal paraffin-embedded sections.

Histological interpretation of frozen sections made during Mohs' micrographic surgery may be difficult, depending on the morphological and staining characteristics of the tumour and on the nature of the associated inflammatory infiltrate. We have employed an adaptation of micrographic surgery in which horizontal, formalin-fixed, paraffin-embedded sections were used to improve histological assessment in the excision of 18 non-melanoma skin tumours in which frozen sections had been or were likely to be unsatisfactory. We describe our experience of this method in the management of squamous cell carcinomas (11), extramammary Paget's disease (two), microcystic adnexal cell carcinomas (two), dermatofibrosarcoma protuberans (two), and primary cutaneous neuroendocrine carcinoma (Merkel cell carcinoma) (one). The use of horizontal paraffin-embedded sections lengthens the duration of the procedure but facilitates accurate assessment of histological sections in selected tumours.

Microcystic adnexal carcinoma of the skin. An underestimated tumor

Microcystic adnexal carcinoma is a rare semimalignant cutaneous neoplasm characterised by slow but locally aggressive growth. Its histogenesis and nomenclature are still a subject of controversy. Under-recognition and the tumor's bland cytologic features lead to frequent misdiagnoses. In addition, subclinical tumour invasion explains the high incidence of local recurrence. Thus, the tumour makes great demands on the planning of its surgical removal. We report the case of an 81-year-old woman with a microcystic adnexal carcinoma of the lower lip, which was treated by micrographic surgery. On the basis of this case, differential diagnoses, histogenesis and micrographic surgery as the treatment of choice are discussed.