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Year : 2017  |  Volume : 1  |  Issue : 1  |  Page : 16-18

Microcystic adnexal carcinoma of face a rare aggressive pathology

1 Department of Dentistry, Pandit Raghunath Murmur Medical College, Baripada, Odisha, India
2 Department of Oral and Maxillofacial Pathology, SCB Dental College and Hospital, Cuttack, Odisha, India
3 Department of Oral Pathology and Microbiology, SCB Dental College and Hospital, Cuttack, Odisha, India

Date of Web Publication27-Sep-2017

Correspondence Address:
Punyasloka Pati
Department of Oral and Maxillofacial Pathology, SCB Dental College and Hospital, Cuttack - 753 007, Odisha
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/oji.oji_8_17

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Microcystic adnexal carcinoma (MAC) is an uncommon, locally aggressive, malignant cutaneous tumor having pilar and eccrine differentiation and most commonly occurs in the head and neck region but rare among skin cancer. It is characterized by slow, but locally aggressive growth pattern with infiltration into surrounding structures which is 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 ductal differentiation. We report a case of MAC of right cheek in a 50-year-old male patient with diagnostic challenges. MAC may include in the differential diagnosis list of slowly growing tumors of the face.

Keywords: Differential diagnosis, eccrine, keratinocytes, microcystic adnexal carcinoma

How to cite this article:
Debata T, Pati P, Das S. Microcystic adnexal carcinoma of face a rare aggressive pathology. Oncol J India 2017;1:16-8

How to cite this URL:
Debata T, Pati P, Das S. Microcystic adnexal carcinoma of face a rare aggressive pathology. Oncol J India [serial online] 2017 [cited 2022 Jan 27];1:16-8. Available from: https://www.ojionline.org/text.asp?2017/1/1/16/215721

  Introduction Top

Microcystic adnexal carcinoma (MAC) was first described as a slowly growing but locally aggressive adnexal neoplasm in 1982.[1] It occurs in those anatomic locations which are functionally and cosmetically remarkable area like face. The diagnostic accuracy plays a vital role in treatment planning for fruitful outcome. However, MAC may be confused with other benign or malignant neoplasms, such as desmoplastic trichoepithelioma, basal cell carcinoma, squamous cell carcinoma, and syringoma due to the similarity in histopathological appearance.[2] Here, we report a case of MAC in a 50-year-old male patient.

  Case Report Top

A 50-year-old male patient presented with multiple small swellings over right side mid face region close to the angle of mouth for 3 years and history of reduced mouth opening for last 2 years [Figure 1]. The nodular mass was gradually increased in size from a pimple like structures. There was no history of any form of tobacco habits. The medical history was noncontributory. Extraoral findings revealed yellowish firm, smooth, multinodular swelling with an irregular margin over right cheek region with scarring, and intraorally, there was no ulceration, no fibrous bands were noted with <10 mm mouth opening. Incisional biopsy performed and revealed features of MAC. Wide local excision was done [Figure 2]a.
Figure 1: Clinical picture

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Figure 2: (a) Excised gross specimen, (b) H and E, ×100: Basaloid neoplastic cells arranged in small nests and cords, (c) H and E, ×100: Keratin filled horn/cyst formation, and (d) H and E, ×400: Neoplastic cells with perineural invasion

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Gross examination of the excised specimen showed grayish yellowish firm tissue specimen (m) 3 cm × 2.5 cm with multiple nodular growths on the surface [Figure 2]a. Microscopic examination revealed overlying stratified squamous keratinized epithelium with skin appendages and underlying dense fibrocollagenous stroma having basaloid neoplastic cells with minimal atypia arranged in small nests and cords with keratin filled cyst formation [Figure 2]b and [Figure 2]c. Duct like structures lined by cuboidal cells and neoplastic cells with perineural invasion and infiltration into deeper muscles layers also seen with areas of calcification [Figure 2]d. The above histopathological findings confirmed the diagnosis of MAC. No recurrence was seen after 1-year follow-up.

  Discussion Top

In 1982, Goldstein et al. first described MAC as a distinct histopathologic entity. It belongs to the spectrum of locally aggressive adnexal carcinomas and has recently been proposed to be an apocrine tumor. MAC has also been referred to as malignant syringoma, sweat gland carcinoma with syringomatous features, and sclerosing sweat duct carcinoma.[3]

MAC almost always occurs in the head and neck. Therefore, exposure to solar or X-ray radiation may be of etiologic significance. It can present variable features from a smooth flesh-colored or yellow plaque to a cystic nodule with slow, but infiltrative and destructive local growth. In approximately, 80% of cases, an extensive perineural spread of the tumor is observed as in the present case, and patients may experience pain or paresthesia.[4],[5]

Microscopically, MAC is typically stratified and characterized by a superficial component of keratinous cysts and a deeper component of smaller nests and strands of cells embedded in a markedly hyalinized stroma. However, these features may not be apparent in a superficial shave biopsy specimen. Misdiagnosis of MAC as desmoplastic trichoepithelioma, morphea form basal cell carcinoma, eccrine carcinoma with squamous differentiation or even squamous cell carcinoma has been reported in 30%–52% of cases.[6]

Immunohistochemical staining has a less important role in the diagnosis of MAC, but staining for carcinoembryonic antigen-positive tumor cells can improve the accuracy of tumor margin determination and in cases with extensive perineural infiltration. Hence, it can help to differentiate MAC from other adnexal tumors. The most important histopathologic differential diagnoses of MAC are scleroderma form basal cell carcinoma and desmoplastic trichoepithelioma. Syringoma can be distinguished from MAC by infrequent calcification, lack of prominent horn cyst formation and infrequent single file strand formation. The deeply invasive growth and perineural spread make it possible to distinguish MAC from syringoma and desmoplastic trichoepithelioma.[1],[7],[8]

In some patients, histopathological misdiagnosis may occur in superficial biopsy specimen who can be misinterpreted as a benign adnexal neoplasm, such as a syringoma or trichoepithelioma.[6],[9]

In this case report, here, we have given more emphasis on early diagnosis of such uncommon event as compare to basal cell carcinoma and squamous cell carcinoma in head and neck regions as MAC can be cured by an early radical excision of the tumor controlled by Mohs' micrographic surgery (MMS) or histographic surgery.[10]

There is no unanimity regarding the follow-up period for MAC, but the recurrence rates for conventional excision may be as high as 47%, usually within the first 3 years. For MMS, the recurrence rates range from 0% to 22% with a 5-year follow-up. Therefore, irrespective of treatment modality, all patients should be monitored closely for several decades.[3],[10]

Defects and deformities on the face resulting from wide excision are not avoidable, despite the reconstructive efforts of surgeons. Facial swellings may be the outcome of inflammation, infection, cystic or neoplastic processes, but rare and aggressive skin lesions as in the present case should also be considered in the differentials of facial swellings. The general physician and dentist should be aware of these facts. However, to make this diagnosis, it is imperious that an adequate biopsy to be obtained to demonstrate both its biphasic growth pattern as well as its extensive local invasion. Although the long-term prognosis of these tumors is unknown, most appear to act in a locally aggressive fashion without metastases. As such, the tumor should be completely excised with adequate surgical margins.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Goldstein DJ, Barr RJ, Santa Cruz DJ. Microcystic adnexal carcinoma: A distinct clinicopathologic entity. Cancer 1982;50:566-72.  Back to cited text no. 1
Koh SH, Kang KR, Yang JH, Jung SW, Lee HJ. Microcystic adnexal carcinoma misdiagnosed as desmoplastic trichoepithelioma on preoperative biopsy. Arch Craniofac Surg 2015;16:43-6.  Back to cited text no. 2
Fischer S, Breuninger H, Metzler G, Hoffmann J. Microcystic adnexal carcinoma: An often misdiagnosed, locally aggressive growing skin tumor. J Craniofac Surg 2005;16:53-8.  Back to cited text no. 3
Schwarze HP, Loche F, Lamant L, Kuchta J, Bazex J. Microcystic adnexal carcinoma induced by multiple radiation therapy. Int J Dermatol 2000;39:369-72.  Back to cited text no. 4
Hodgson TA, Haricharan AK, Barrett AW, Porter SR. Microcystic adnexal carcinoma: An unusual cause of swelling and paraesthesia of the lower lip. Oral Oncol 2003;39:195-8.  Back to cited text no. 5
Chiller K, Passaro D, Scheuller M, Singer M, McCalmont T, Grekin RC. Microcystic adnexal carcinoma. Forty-eight cases, their treatment, and their outcome. Arch Dermatol 2000;136:1355-9.  Back to cited text no. 6
Ohtsuka H, Nagamatsu S. Microcystic adnexal carcinoma: Review of 51 Japanese patients. Dermatology 2002;204:190-3.  Back to cited text no. 7
Rütten A. Eccrine sweat gland carcinoma of the skin. Pathologe 2002;23:79-88.  Back to cited text no. 8
Abbate M, Zeitouni NC, Seyler M, Hicks W, Loree T, Cheney RT. Clinical course, risk factors, and treatment of microcystic adnexal carcinoma: A short series report. Dermatol Surg 2003;29:1035-8.  Back to cited text no. 9
Diamantis SA, Marks VJ. Mohs micrographic surgery in the treatment of microcystic adnexal carcinoma. Dermatol Clin 2011;29:185-90, viii.  Back to cited text no. 10


  [Figure 1], [Figure 2]


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