Microcystic adnexal carcinoma in a non-Caucasian patient: A case report and review of the literature

Abstract

Abstract. Microcystic adnexal carcinoma (MAC) is extremely rare among Asians, with the majority of cases presenting in Caucasian individuals. The current study describes the case of a 38-year-old Chinese woman who presented with a 10 year history of a mass in the upper lip. A biopsy resulted in a diagnosis of MAC. The patient underwent complete surgical resection and the tumor was successfully excised. During 6 months of follow-up, there was no evidence of recurrence. To the best of our knowledge, the present case is the first Chinese case to be reported in the English literature, and is presented with the aim of increasing the awareness and aiding in the management of MAC in non-Caucasian populations. IntroductionMicrocystic adnexal carcinoma (MAC) was originally defined in 1982 (1), and is now also termed sclerosing sweat duct carcinoma (2). MAC is a rare, slow-growing, locally aggressive tumor of the eccrine sweat glands, and is characterized by pilar and eccrine differentiation (3). Sweat gland malignancies, combining all types, are particularly rare, accounting for 0.005% of all malignant epithelial neoplasms (4), with a total of ~350 cases currently described in the English literature. It has been reported that the preferential location for the development of this neoplasm is the head and neck region (5).When referring to the English literature, it is evident that MAC primarily affects the Caucasian population, with only two cases occurring in non-Caucasian patients (6,7). Fang et al (8) described two cases involving Chinese individuals in the Chinese literature; however, to the best of our knowledge, there are no cases of Chinese patients with MAC currently reported in the English literature. The present study reviewed all patients that were referred to the Hainan Province People's Hospital (Haikou, China) between January 1994 and January 2014, and identified only one patient with a MAC diagnosis. This case is described herein along with a review of the relevant literature. Case reportA 38-year-old woman was referred to the Department of Oral and Maxillofacial Surgery at the Hainan Province People's Hospital with a mass on the left upper-lip that had been present for >10 years (Fig. 1). During physical examination, it was noted that the tumor was positioned in the left-upper lip and subnasal region. The lesion was a firm, solitary mass, and measured 2x1 cm in size. No submandibular or cervical lymph nodes were palpable during the physical examination. Abdominal ultrasonography and computed tomography (CT; Brilliance iCT; Philips Medical Systems, Amsterdam, Holland) of the head, neck and thorax demonstrated no evidence of systemic or local metastasis. As presented in Fig. 2, the head and neck CT scans revealed the lesion located in the subcutaneous tissue of the left-upper lip, with a wide base inseparable from overlying skin.An incisional biopsy was performed with the patient under local anesthesia. The diagnosis of the specimen was confirmed as carcinoma. The patient underwent complete surgical resection...

An atypical presentation of extensive centrofacial microcystic adnexal carcinoma responding to radiation: A case report and review of the literature

“…It often presents as an indolent, non-ulcerated, smooth-surfaced, flesh-colored to yellowish asymptomatic nodule, papule, plaque, or cystic lesion with indistinct margins with a predilection for the head and neck, predominantly arising in the central face. 1,4,[11][12][13][14][15][16][17][18] It is paramount to include MAC as a differential diagnosis among the other head and neck lesions to enable early diagnosis and treatment to reduce morbidity and mortality. Deep incisional or excisional biopsy for histopathology is the gold standard for initial diagnosis.…”

“…It often presents as an indolent, non-ulcerated, smooth-surfaced, flesh-colored to yellowish asymptomatic nodule, papule, plaque, or cystic lesion with indistinct margins with a predilection for the head and neck, predominantly arising in the central face. 1 , 4 , 11 – 18 …”

“…To date, there is insufficient evidence whether radiation is an effective monotherapy in patients with MAC due to rarity of the disease and the limited use as a management option. 2 , 4 , 11 – 18 Due to radiation therapy resulting in worsening of one MAC case and being a risk factor for MAC, radiation as monotherapy should be considered prudently. 1 Radiation may also be sought as treatment when standard surgical therapy may prove difficult and result in poor cosmetic outcome.…”

“… 1 – 26 It is characterized as a malignant, aggressive, and locally invasive tumor with a high recurrence rate. 2 – 4 , 8 , 9 , 11 – 17 , 19 MAC typically does not metastasize; however, there are reports of local and distant progression of the disease. 1 , 13 , 15 Although recognized as a distinct clinicopathologic entity, 3 MAC has proven to be diagnostically challenging as it can mimic other similar neoplasms.…”

“…Clinically, MAC typically manifests as an indurated, growing flesh-colored plaque although variations of clinical morphology have been reported. 1 , 4 , 11 – 18 Microscopically, the tumor often extends beyond clinical margins, spreading locally into the dermal, subcutaneous, and occasionally perineural tissues. 4 Histological features of MAC include infiltrated well-differentiated nests and cords of keratinocytes displaying follicular and ductal differentiation in a sclerotic stroma.…”