Microcystic Adnexal Carcinoma Research Articles

Surveillance, Epidemiology, and End Results (SEER) Database Analysis of Microcystic Adnexal Carcinoma (Sclerosing Sweat Duct Carcinoma) of the Skin

Microcystic Adnexal Carcinoma (MAC), also known as sclerosing sweat duct carcinoma, malignant syringoma, syringoid carcinoma, and trichofolliculoma with perineural invasion, is a very rare cancer of the skin. It has been described previously in small case reports and retrospective series, and is thought to be a slow growing, locally aggressive and recurrent cancer. Prior reports have also noted a predilection for the head and neck. In addition, prior radiotherapy is thought to be a risk factor for MAC. This is the largest report of MAC in the literature. The National Cancer Institute (NCI) SEER 1973-2003 database was investigated, and patients with sclerosing sweat duct carcinoma of the skin were detected. A descriptive analysis was performed, investigating age, gender, site of disease, use of radiation, extent of surgery, and reason for death. Overall survival was calculated by using the Kaplan-Meier method. Relative survival was calculated in SEER∗Stat 5, using age-, race-, gender- and year of diagnosis-matched U.S. mortality data. MAC of the skin was recorded in 164 patients. Ten patients received radiation. Ninety-two percent of patients received surgery. Sixty percent of subjects were female. Median age at diagnosis was 67.5 years old. The median recorded tumor size was 15 mm (range 1 mm–150 mm), though tumor size was not recorded for 102 patients. Race distribution was 91% white, 4% black or asian/pacific islander, and 5% unknown/other. As expected, the site of disease predominated in the head and neck (74%). The remaining reported sites included trunk 10%, upper limb/shoulder 9%, and lower limb/shoulder 6%. Seventy-six percent of disease was confined to the dermis or localized but not otherwise specified. Seven percent had invaded through the entire dermis, and 7% had invaded locally to underlying soft tissue and bone. Disease invasion was unknown in 9%. There was no recorded metastatic disease though these were possibly coded as non-skin sites and not captured in our analysis. Lymph nodes were removed in only 6 cases. The 5-year overall survival was 90% ([95% CI: 80%–95%]). The 5 and 10-year relative survival 99.6% (SE: 3.1% and 3.9%, respectively). All 8 deaths occurred before 3 years (Fig.). This SEER database analysis confirms previously published characteristics. MAC tends to occur in the skin of the head and neck (74%), in whites (91%), and in females (60%). It is locally invasive and rarely metastasizes to lymph nodes. Overall and relative survival is excellent. Radiation is rarely used for treatment.

Monoclonal antibody Ber‐EP4 reliably discriminates between microcystic adnexal carcinoma and basal cell carcinoma

Sclerosing cutaneous neoplasms often represent a diagnostic challenge. The monoclonal antibody Ber-EP4 recognizes two glycopolypeptides found in most human epithelial cells. It is diagnostically highly reliable in the differentiation between basal cell carcinoma and cutaneous squamous cell carcinoma. In this study, we report its application in the differential diagnosis of microcystic adnexal carcinoma, desmoplastic trichoepithelioma and basal cell carcinoma. Biopsy samples from 28 sclerosing and infiltrating basal cell carcinomas, 13 microcystic adnexal carcinomas and 16 desmoplastic trichoepitheliomas were examined after immunohistochemical staining with Ber-EP4. Ber-EP4 did not label any of the microcystic adnexal carcinomas, whereas all 28 basal cell carcinomas were Ber-EP4 positive. Twenty-seven of the 28 showed moderate or strong staining intensity, with the majority being strong. Only one basal cell carcinoma was weakly positive. Twelve of the 16 desmoplastic trichoepitheliomas were immunoreactive with Ber-EP4 and the staining was more variable than those of basal cell carcinomas. Ber-EP4 reliably differentiates microcystic adnexal carcinoma from basal cell carcinoma to the same extent as it distinguishes the latter tumor from squamous cell carcinoma. While it stains the majority of desmoplastic trichoepitheliomas, these tumors still have to be considered in the differential diagnosis with microcystic adnexal carcinoma, when Ber-EP4 is applied.

The expression of CD23 in cutaneous non‐lymphoid neoplasms

Cluster designation 23 (CD23) is generally used as a lymphoid marker. Its utility in cutaneous epithelial tumors has never been studied. In our routine practice, we observed that CD23 reacted strongly with eccrine and apocrine secretory coils. Immunohistochemical staining of CD23 was performed in a total of 131 cases of apocrine, eccrine, follicular and other cutaneous non-lymphoid tumors. CD23 expression was detected in all benign apocrine tumors and in half of benign eccrine tumors, particularly those derived from secretory coils. CD23 staining was seen in 42% (8/19) of microcystic adnexal carcinoma (MAC), while no staining was observed in tumor cells of desmoplastic trichoepithelioma, morpheaform basal cell carcinoma and syringoma. All mammary and extramammary Paget's disease were labeled with CD23. In comparison, pagetoid Bowen's disease, melanoma in situ and sebaceous carcinoma exhibited negative staining. In addition, CD23 reacted diffusely with cutaneous mucinous eccrine carcinoma in a manner similar to breast or colonic adenocarcinoma. CD23 appears to be a reliable immunohistochemical marker of the eccrine/apocrine secretory coil and helpful in identifying sweat gland tumors of such origin. It is of ancillary value in differentiating MAC from its mimicker. CD23 is a useful addition to the diagnostic immunohistochemical panels for Paget's disease.

Microcystic Adnexal Carcinoma in African-Americans

Microcystic Adnexal Carcinoma in African-Americans

Basaloid skin tumours: Mimics of basal cell carcinoma

Summary This article is the second of two papers relating to the histopathological diagnosis of basaloid skin tumours and the uses of immunohistochemistry. The first paper focused on basal cell carcinoma and variants, and this paper will concentrate on tumours that may be confused with basal cell carcinoma. The basaloid tumours of the skin can be classified according to the general classification of skin tumours, including epidermal, appendageal (hair follicle and sweat gland derived) and others including cutaneous metastases. The areas of discussion concentrate on the distinction of basal cell carcinoma from basaloid squamous cell carcinoma, infiltrative basaloid tumours (desmoplastic trichoepithelioma, infiltrative basal cell carcinoma, microcystic adnexal carcinoma and eccrine epithelioma), follicular induction overlying dermatofibroma, basaloid proliferations in naevus sebaceus, trichoepithelioma, trichoblastoma, trichoepithelioma-like basal cell carcinoma, pilomatrical tumours and selected sweat gland tumours. The immunohistochemical stains that may be of use in differential diagnosis are discussed, including BerEP4, epithelial membrane antigen, CD10, bcl-2, Cam5.2, CK20, carcinoembryonic antigen and p53.

Microcystic Adnexal Carcinoma

Microcystic adnexal carcinoma (MAC) is an uncommon, locally aggressive neoplasm of the skin appendages that exhibits eccrine and follicular differentiation. The tumor is typically described as slow growing but destructive. Histologic sections demonstrate keratinized cysts, eccrine ducts, and bland tumor cords within a desmoplastic stroma. MAC is characteristically devoid of striking atypia, mitotic figures, and necrosis. The deeply invasive process starts in the superficial dermis but may extend into the subcutaneous tissue and skeletal muscle. Bland clinical and histologic features combined with characteristically superficial or small incomplete biopsies often result in a misdiagnosis and delay curative treatment. In this article, we report a case of MAC followed by a comparative discussion of the most common histologic differential diagnoses.

Microcystic Adnexal Carcinoma in the Nasolabial Area: Resection and Reconstruction with a Free Fasciocutaneous Lateral Arm Flap with a Sandwich Technique

Microcystic Adnexal Carcinoma in the Nasolabial Area: Resection and Reconstruction with a Free Fasciocutaneous Lateral Arm Flap with a Sandwich Technique

Mohs Micrographic Surgery in the Treatment of Rare Aggressive Cutaneous Tumors: The Geisinger Experience

Mohs micrographic surgery (MMS) offers high cure rates and maximum tissue preservation in the treatment of more common cutaneous malignancies, but its effectiveness in rare aggressive tumors is poorly defined. Evaluate the effectiveness of MMS in the treatment of six rare aggressive cutaneous malignancies as seen by Mohs surgeons working at a referral center. Retrospective chart review of 26,000 cases treated with MMS at the Geisinger Medical Center Department of Dermatology during a 16-year period with the following diagnoses: poorly differentiated squamous cell carcinoma (PDSCC), dermatofibrosarcoma protuberans (DFSP), microcystic adnexal carcinoma (MAC), extramammary Paget's disease (EMPD), Merkel cell carcinoma (MCC), and sebaceous carcinoma (SEB CA). Patient demographic data, tumor measurements, treatment characteristics, and marginal recurrence rates were compiled and evaluated. The mean numbers of cases identified per year for each tumor type were as follows: PDSCC, 6.19; DFSP, 2.44; MAC, 1.63; and EMPD, 0.63. For PDSCC, 85 cases were available for follow-up with a local recurrence rate of 6% at a mean follow-up time of 45 months. For DFSP, there were 35 cases with no local recurrence at a mean follow-up of 39 months. For MAC, there were 25 cases with a local recurrence rate of 12% at a mean follow-up of 39 months. For EMPD, there were 10 cases with no local recurrences at a mean follow-up of 34 months. Collectively, our data on PDSCC, DFSP, MAC, and EMPD, combined with other studies in the literature, show that MMS is the most effective therapy for these rare aggressive cutaneous malignancies.

基底細胞癌に合併したＭｉｃｒｏｃｙｓｔｉｃ Ａｄｎｅｘａｌ Ｃａｒｃｉｎｏｍａの１例

66歳，男性。10年前より頭部に黒色小丘疹が出現し，2005年9月他院での皮膚生検で基底細胞癌と診断され当院へ紹介受診となった。頭部に4×4cmの紅斑局面とそれに隣接して線状に並ぶ黒色小丘疹数個を認めた。基底細胞癌と診断し，切除術および分層植皮術施行。組織所見では，線状に並ぶ黒色小丘疹は基底細胞癌と診断し，紅斑局面は，真皮内に基底細胞様細胞からなる腫瘍巣，角質嚢腫様構造，管腔構造がみられmicrocystic adnexal carcinomaと診断した。これらの合併例は本邦では本症例のみであった。また鑑別点につき文献的考察を加え報告する。

Microcystic Adnexal Carcinoma with Mandibular Bone Marrow Involvement

Microcystic adnexal carcinoma is a rare, locally aggressive cutaneous neoplasm with a high probability of persistence locally but a low probability of metastasis. We report a case of a 69-year-old female patient with an indurated plaque at the mental region. Histologically, the tumor cells invaded the subcutaneous tissue and mandibular bone. The tumor consisted mainly of squamous and basaloid epithelial nests and cords embedded in a desmoplastic stroma. A few keratin-filled microcysts and ductal structures were also observed. Perineural encroachment was also noted but there was no mitosis, cytologic features of malignancy, or metastasis. The epithelial nests were positive to various cytokeratins except for CK20 and the lumina of the ductal structures were positive to carcinoembryonic antigen. Our results indicate that microcystic adnexal carcinoma consists of tumor cells capable of both follicular and eccrine differentiation. It is locally aggressive, extends far beyond its clinical presentation and may involve the bone. It may persist and remain asymptomatic for so many years without metastasis. A lifetime postsurgery monitoring is mandatory to ensure early and proper management.

Microcystic Adnexal Carcinoma in the Mental Region

Microcystic adnexal carcinoma is a rare tumour, first described in 1982. Prior to the recognition of microcystic adnexal carcinoma, instances of unusual basal cell carcinoma, sweat gland carcinoma, and trichofolliculoma had been reported. Microcystic adnexal carcinomas are slow growing and demonstrate aggressive local behaviour and a high recurrence rate. The mainstay of treatment is surgical excision using frozen sections to check the peripheral and deep margins. This report is of a microcystic adnexal carcinoma in the mental region of a 69-year-old woman.

High (Nuclear) Grade Adnexal Carcinoma With Microcystic Adnexal Carcinoma-Like Structural Features

Microcystic adnexal carcinoma (MAC) is a slow growing, locally aggressive sweat gland tumor. It predominantly affects the face and tends to recur despite local excision. Microscopically, MAC is characterized by a stratified proliferation of microcysts, cords, and ducts of cells that show squamous or adnexal differentiation. Atypia and mitoses are almost completely absent and metastatic deposits are rare and mostly limited to the regional lymph nodes; rather than real metastases, they might be the result of local extension of the tumor through perineurial spaces. We report a case of adnexal carcinoma with architectural features of MAC that displayed also marked nuclear pleomorphism and hyperchromasia with squamous pearl formation and a widespread strong p53 immunoreaction. The lesion behaved as a high-grade neoplasm with rapid growth, carcinosarcomatous metaplastic transformation in a relapse, and what were clinically suspected to be metastases. The literature contains several other examples reported as metastatic high-grade MAC, one of them with widespread distant metastases. We therefore want to sound an alert about the possible existence of tumors displaying microscopic findings characteristic of the aggressive forms of sweat gland carcinoma (nuclear pleomorphism and hyperchromasia, vascular invasion, and necrosis) in addition to architectural features of MAC. Whether these tumors should be called high-grade MACs or belong to a separate category remains an open issue until more cases are reported and bridge cases are eventually documented.

High Grade Microcystic Adnexal Carcinoma

From the International Society of Dermatopathology: Abstracts: Abstracts Presented at the ISDP 8th Joint Meeting, New Orleans, Louisiana February 16-17, 2005

Microcystic Adnexal Carcinoma Involving a Large Portion of the Face: When Is Surgery Not Reasonable?

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. To review the literature about what is known about therapy for MAC and what options are available to patients who have this disease. Case report and review of the literature. Of the 274 cases of MAC thus far reported, there are 6 cases of metastases, only 1 of which resulted in death. 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.

Microcystic Adnexal Carcinoma Involving a Large Portion of the Face

Microcystic Adnexal Carcinoma Involving a Large Portion of the Face

Cutaneous lip tumours treated with Mohs micrographic surgery: clinical features and surgical outcome

The Australian Mohs micrographic surgery (MMS) database was initiated in 1993 by the Skin and Cancer Foundation Australia (SCFA) with the aim of collecting prospective data, and involved all Mohs surgeons in the country. To present a large series of patients with cutaneous lip tumours treated with MMS in Australia between 1993 and 2002. This prospective multicentre case series included all patients with cutaneous lip tumours who were monitored by the SCFA. The main outcome measures were patient demographics, reason for referral, duration of tumour, site, preoperative tumour size and postoperative defect size, recurrences prior to MMS, histological subtypes, perineural invasion and 5-year recurrence after MMS. There were 581 patients (66.1% women and 33.9% men, P < 0.0001) with a mean +/- SD age of 58 +/- 15 years. The upper lip was the most common site involved (81.1%). Basal cell carcinoma (BCC) was diagnosed in 82.3%, squamous cell carcinoma (SCC) in 16.5%, Bowen's disease (BD) in 0.7% and microcystic adnexal carcinoma (MAC) in 0.5% of cases. BCC was more common on the upper lip and in women, whereas SCC was more common on the lower lip and in men (P < 0.0001). Most upper lip tumours occurred in women (75.4%), whereas most lower lip tumours occurred in men (73.6%). SCC was associated with a larger tumour and postoperative defect size compared with the other tumours. The 5-year recurrence for BCC was 3.0%, and there were no cases of recurrence for SCC, BD or MAC. BCC was the most common cutaneous lip tumour managed by MMS, and was significantly more common on the upper lip and in women. The low 5-year recurrence rate emphasizes the importance of margin-controlled excision.

Microcystic adnexal carcinoma: report of four cases treated with Mohs’ micrographic surgical technique

Microcystic adnexal carcinoma (MAC) is a rare and aggressive malignant tumor of the sweat glands. Clinically, it often presents as a firm subcutaneous nodule on the head and neck regions. On histology, MAC exhibits both pilar and sweat duct differentiation with a stroma of dense collagen. It often extends beyond the clinical margins with local spreading in the dermal, subcutaneous, and perineural tissue planes. It has a high local recurrence rate after standard excision. Recent preliminary reports have indicated more favorable cure rates with Mohs' micrographic surgery (MMS). To present our data on four cases of MAC treated by MMS. We also compared our findings with more recently reported series in the English language literature. We reviewed the medical records of four patients (two males and two females) with MAC treated by MMS over the last 3 years. We also obtained follow-up data. In all four patients with MAC treated by MMS, there were no recurrences, with a mean follow-up of 1 year. We report an additional four MAC cases treated by MMS. The accumulated data continue to confirm that, if the diagnosis of MAC is made early, and if the anatomic location is accessible to excision by MMS, a favorable outcome can be expected.

A Microcystic Adnexal Carcinoma in the Auditory Canal 15 Years after Radiotherapy of a 12-Year-Old Boy with Nasopharynx Carcinoma

Radiogenic malignancies require cure of the primary disease and a prolonged survival. The introduction of high-volt technology in the 1950s and 1960s made radical radiotherapy feasible and successful in terms of higher cure rates and longer survival. We are already in a time when a higher number of patients with radiogenic secondary malignancies must be expected. A 12-year-old boy is reported who suffered from an advanced nasopharynx carcinoma and was treated with radical irradiation in 1983. 15 years later he developed a rare microcystic adnexal carcinoma of the auditory canal inside the volume of the target dose. The secondary malignant neoplasm was resected and required another radiation treatment (1 Gy b.i.d.) due to involved margins. The entity of microcystic carcinoma is discussed with a review of the literature on biology, diagnosis, and treatment.

Orbital and Periorbital Microcystic Adnexal Carcinoma

To review the clinical and histopathologic features of patients with microcystic adnexal carcinoma of the orbital and periorbital tissues. This study was designed as a noncomparative interventional case series of three patients seen and treated at two oculoplastic surgical departments and a review of the literature. Clinical presentation, histopathologic findings, outcome of surgery, and clinical recurrence are presented. Three patients underwent surgery for lesions temporal to the lateral canthus. All had initial histopathology diagnosed as squamous cell carcinoma but were subsequently diagnosed as microcystic adnexal carcinoma after clinical recurrence. All patients had multiple recurrences and extensive perineural spread. Two patients required radiotherapy. Review of the literature shows that microcystic adnexal carcinoma affecting the orbital and periorbital tissues is difficult to differentiate clinically and microscopically from other conditions including squamous cell carcinoma. Eighty-one percent of cases that have histopathology checked in the initial assessment are still misdiagnosed. A high degree of suspicion is necessary if the lesion extends beyond an apparently adequate surgical margin, multiple recurrences occur, or superficial perineural spread is present. Mohs technique is the treatment of choice and may be improved if paraffin sections or immunohistocytochemistry are performed. Wide excision should be carried out once clear margins are obtained. Radiotherapy may be necessary in cases with multiple recurrences.

Microcystic adnexal carcinoma: Treatment with Mohs micrographic surgery

Microcystic adnexal carcinoma (MAC) is reported to have a high rate of recurrence with standard wide local excision. To report a large series of patients with MAC treated with Mohs micrographic surgery (MMS). This prospective, multi-center case series included all patients in Australia treated with MMS for MAC, who were monitored by the Skin and Cancer Foundation between 1993 and 2002. There were 44 cases; most of them (90.9%) were located in the head and neck area. In 31.8% of cases it was a recurrent tumor. In 32.5% of cases the tumor was initially misdiagnosed as basal cell carcinoma or squamous cell carcinoma. Perineural invasion was recorded in 17.5% of cases; most of them (85.7%) were previously recurrent tumors. There was only one case of recurrence (5%) out of 20 patients who completed a 5 year follow-up period after MMS. The low 5-year recurrence rate of MAC with MMS emphasizes the importance of margin-controlled excision.