Journal of International Academy of Forensic Science & Pathology (JIAFP)

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1 Journal of International Academy of Forensic Science & Pathology (JIAFP) ISSN MICROCYSTIC ADNEXAL CARCINOMA-A CASE REPORT WITH REVIEW OF LITERATURE Case Report Sulakshana M S 1,Natarajan M 2 *, Rajkumar 3,Jayaram R 4 1 Tutor, 2 Professor, 3 Post Graduate;Department of Pathology; Bangalore Medical College and Research Institute, Fort, K R Road, Bangalore,Karnataka.India. 4 Consultant Pathologist; Doctors Diagnostic Centre, # 83, N K Complex, Kammanahalli Main Road, Bangalore *Corresponding Author: 2 Dr Natarajan M; Address:No 52, 1 st Cross, Vivekanandnagar,M S Nagar P O,Bangalore Mob no: ; drmjan@gmail.com Accepted: June 12, 2015 Published: July, 2015 Citation: Sulakshana M S 1, Natarajan M 2 *, Rajkumar 3,Jayaram R 4 (2015). Microcystic Adnexal Carcinoma-A Case Report with Review of Literature.Vol 02;No 02. Copyright: 2015 Sulakshana M S, This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution and reproduction in any medium, provided the original author and source are credited. ABSTRACT Microcystic adnexal carcinoma, or sclerosing sweat duct carcinoma may best be considered a sclerosing variant of ductal eccrine carcinoma. This is commonly seen on the upper lip, occasionally on chin, nasolabial fold, cheek and very rarely on extremities. It is an aggressive neoplasm that invades deeply. Local recurrence is common, and very few cases of metastases have been reported. Micrographic Moh s surgery is the preferred treatment of choice. We report a case of microcystic adnexal carcinoma in an 80 year old male who presented with a tiny skin lesion over his right elbow. Keywords:microcystic adnexal carcinoma, eccrine, invasion, recurrence. INTRODUCTION: Microcystic adnexal carcinoma, or sclerosing sweat duct carcinoma, may best be considered as a sclerosing variant of ductal eccrine carcinoma. This tumor is most commonly seen on the skin of the upper lip, but occasionally also on the chin, nasolabial fold, or cheek. It is very rare in the extremities. [1]. The tumour presents in a wide age group (11-90 years) although the majority of those affected are between the ages of 40 and 60 years, with no sex

2 predilection[2]. A characteristic histological feature is epithelial proliferation that progressively involves microcysts, strands, and cords as the tumour invades the deeper tissues from the skin surface. It has a severe tendency to neurotropic spread and presents with widely infiltrated margins [3]. Its importance lies in the fact that it can be easily confused with benign adnexal tumours, particularly desmoplastictrichoepithelioma, trichoadenoma and syringoma[4]. CASE REPORT: An 80 year old male presented with a slow growing elevated skin lesion measuring 1.5x1.5 cm on his right elbow since 2 years. Histopathological examination of the lesion revealed a poorly circumscribed tumour in the dermis comprising of cells arranged in ductal and glandular patterns. The ducts and glands were lined by two-layered benign looking cuboidal epithelial cells. No mitoses/significant cellular atypia were noted. The tumour at places was seen invading into the subcutis with involvement of deep surgical margins. Pic 1: Microcystic adnexal carcinoma in the dermis (H&E, 4X)

3 Pic 2:Microcystic adnexal carcinoma showing ductal and glandular elements lined by two-layered bland looking epithelium (H&E, 10X). Pic 3:Microcystic adnexal carcinoma invading the subcutis (H&E, 40X)

4 Pic 4-A & B:Microcystic adnexal carcinoma extending upto the deep surgical resected margin (H&E, 4X and 10X). DISCUSSION: First defined by Goldstein and colleagues in 1982, there have been only approximately 150 cases of microcystic adnexal carcinoma described in the English medical literature. Microcystic adnexal carcinoma is a slow-growing albeit locally aggressive adnexal carcinoma with a high tendency to recur despite excision. Its aggressive nature is often due to delay in diagnosis, inadequate tissue sampling and thus, detection at an advanced stage of disease with invasion beyond skin into underlying tissue. Thus, a high index of suspicion is necessary in assessing these tumours and adequate deep biopsies for diagnosis are essential [2]. Initially it was believed that these tumors do not metastasize, but now it has been well reported [5]. Clinically, the neoplasm can present as a slow growing, flesh-coloured, yellow or erythematous, firm or hard plaque or nodule which may be associated with hyperkeratosis.margins are typically difficult to delineate and ulceration is unusual. Lesions are usually asymptomatic, but they may be painful or have paraesthesia due to perineural infiltration [6]. They most commonly occur in the head and neck region and are very are in the extremities. Caucasians are affected most frequently and there does not appear to be a gender predisposition. The tumour presents in a wide age group (11-90 years) although the majority of those affected are between the ages of 40 and 60 years [2]. Microcystic adnexal carcinoma is a poorly circumscribed dermal tumor that may extend into the subcutis and skeletal muscle. Continuity with the epidermis or follicular epithelium may be seen. Two components within a desmoplastic stroma may be evident. In some areas, basaloid keratinocytes are seen, some of which contain horn cysts and abortive hair follicles; in other areas, ducts and gland-like structures lined by a two-cell layer predominate. The tumor islands typically reduce in size as the tumor extends deeper into the dermis. Cells with clear cytoplasm may be present, and sebaceous differentiation has been reported. Cytologically, the cells are bland without significant atypia; mitoses are rare or absent. Perineural invasion may be seen, a feature that may account for the high recurrence rate[1]. The differential diagnoses for MAC include desmoplastictrichoepithelioma, syringoma and infiltrative or morphaeform basal cell carcinoma. Making a specific diagnosis may be impossible on a superficial biopsy. In contrast to desmoplastictrichoepithelioma and syringoma, MAC is a poorly circumscribed tumor that extends deep into the reticular dermis and often involves the subcutaneous fat. Perineural invasion is characteristic of MAC and

5 may also occur in basal cell carcinoma, but is not observed in desmoplastictrichoepithelioma and syringoma. Basal cell carcinoma will usually show a focus with characteristic clefts between the neoplastic basaloid epithelium and a mucin-rich stroma. These findings are not observed in MAC [7]. The cell of origin appears to be pluripotent adnexal keratinocytes, which is capable of both, follicular and sweat gland differentiation [5]. MAC expresses various glycoproteins of CEA (carcino embryonic antigen) family. These can help in diagnosis but cannot differentiate it from metastatic carcinoma [8]. The appropriate management of MAC is not yet clearly known, as there are few case reports. A variety of treatment approaches have been used including primary excision, Moh s excision and radiotherapy. Moh s excision has been reported to be preferred method of surgical excision [5]. CONCLUSIONS: Microcystic adnexal carcinoma is a rare benign appearing malignant adnexal neoplasm with a high local recurrence rate. A high index of suspicion and a deep biopsy with wide excision are required for a correct and prompt diagnosis. Caution must be exercised with superficial biopsies which will miss the deep invasion of this tumour which otherwise has a benign morphology. ACKNOWLEDGEMENTS My Prayers to God; my sincere gratitude and thanks to my Professors for their valuable support and guidance presenting this case report.also my heartfelt thanks to the histopathology laboratory technician for providing me good sections for this study. REFERENCES: 1. Elder, David E. Lever s Histopathology of the Skin. Carcinoma of apocrine glands. Lippincott; 2005, 9th Ed; Kwok T, Narayan B. Microcystic adnexal carcinoma. University Of Western Ontario Medical Journal 2007; 76 (1): Beltramini GA, Baj A, Moneghini L. Microcystic adnexal carcinoma of the centrofacial region: a case report. ActaOtorhinolaryngolItal 2010; Aug: 30 (4) Goldstein DI, Barr RJ, Santa Crux DJ. Microcystic adnexal carcinoma: a distinct clinicopathological entity. Cancer 1982; 50: Sharma L, Biswas G. Microcystic adnexal carcinoma in a 10 year old child: A case report and review of literature. Indian Journal of Medical and Paediatric Oncology. 2004; Vol 25(2): Kumar K, McGregor JC, Watson JD. Microcystic adnexal carcinoma-a report of three cases. JR CollSurgEdinb. Dec 1998; 43: Pilch B Z. Dermatopathology of the Head and Neck. Head and Neck Surgical Pathology. Lippincott Williams and Wilkins 2001: 13: Metz D, Grunert F. Neoplasm with sweat gland differentiation expressing various glycoproteins of CEA family. J Cutaneous Pathology 1996; 23:1-11