Clinical and pathological analysis of 10 patients with microcystic adnexal carcinoma / 中华皮肤科杂志

Objective To discuss the diagnosis and differential diagnosis of microcystic adnexal carcinoma (MAC).Methods Totally,10 patients with MAC were enrolled from Hospital for Skin Diseases,Chinese Academy of Medical Sciences from 2003 to 2017.Their clinical manifestations,histopathological and immunohistochemical features,treatment and prognosis were retrospectively analyzed.Results Of the 10 patients,3 were males and 7 were females.Their average age at the onset of MAC was 51.65 years.Skin lesions all occurred on the face,and on the upper lip in 6 cases.The lesions usually presented as solitary plaque or nodule,and ulceration occurred in 4 cases.Histopathologically,skin lesions consisted of epithelial cords with different numbers of keratinous cysts and tubular structures,and neural involvement occurred in 6 cases.However,mitotic figures were rare.Immunohistochemical staining showed epithelial cells and keratinous cysts stained positive for cytokeratin,as well as tubular structures and glandular cavities stained positive for carcinoembryonic antigen and epithelial membrane antigen.All the patients received surgical excision,and one patient experienced in situ recurrence 13 years later.No distant metastasis occurred in these patients.Conclusions MAC mainly presents as red plaques with occasional ulceration on the upper lip.Its definite diagnosis depends on characteristic histopathological changes in bidirectional differentiation into hair follicles and sweat glands,and immunohistochemical features are helpful to distinguish MAC from other adnexal tumors.

A Case of Microcystic Adnexal Carcinoma with Secondary Cicatricial Alopecia / 대한피부과학회지

Microcystic adnexal carcinoma (MAC) was first described in 1982 by Goldstein. Considered a rare malignant skin appendageal tumor, it is often underdiagnosed due to its clinical and histopathological resemblance to other cutaneous neoplasms. MAC is locally aggressive with infiltration of perineural spaces, subcutaneous tissue, skeletal muscles, and so on. Aggressive treatment including wide local excision, Mohs micrographic surgery, or radiation therapy is necessary owing to the high recurrence rate. Herein, we report a case of a 47-year-old Korean woman with a skin-colored hardened plaque on the scalp with a clinical diagnosis of cicatricial alopecia and histopathological diagnosis of MAC. After treatment by Mohs micrographic surgery, the patient is being followed up regularly without any sign of recurrence. This case demonstrates an uncommon topography of MAC on the scalp with secondary cicatricial alopecia and highlights the need for awareness of the potential for MAC in the diagnosis of alopecia with a slow-growing tumor.

Microcystic Adnexal Carcinoma of the Lower lip: A Case Report

PURPOSE: Microcystic adnexal carcinoma is a rare malignant appendage tumor, first described by Goldstein et al in 1982. Here, we present our experience in treatment of a case on the lower lip. METHODS: A 52-year-old female with an asymptomatic nodule on the chin, previously misdiagnosed as trichoadenoma by needle aspiration biopsy, was treated by wide excision combined with multiple circumferential frozen biopsies. RESULTS: Pathological examination revealed typical features of microcystic adnexal carcinoma, such as basaloid and squamous cells forming nests and cord-like patterns, horn cysts, and minimal cytologic atypia. The patient has been followed up for 6 months. No sign of recurrence is noted to date. CONCLUSION: Differentiation from other benign adnexal neoplasms is important for its appropriate treatment. Differentiation can be difficult histologically because it is difficult to acquire an adequate biopsy due to its invasiveness, and clinically due to its asymptomatic and slow growing features. Complete excision is the key treatment, but it may not always be the best solution considering the huge defect that may result and the low incidence of metastasis & deaths owing to the tumor. We add this case to the approximately 300 cases reported worldwide with a review of literature.

A Case of Microcystic Adnexal Carcinoma on the Scalp / 대한피부과학회지

Microcystic adnexal carcinoma is a rare, locally aggressive tumor with a high potential for local recurrence. It usually presents in elderly patients as a solitary, slowly growing, firm, indurated plaque on the face, and particularly on the upper lip or central face. It has occasionally been found in extra-facial locations, including the nipple, axilla and lower extremities, yet scalp involvement has been rarely reported. Histopathologically, it is a poorly circumscribed dermal tumor that shows both follicular and ductal differentiation, and it may extend into the subcutis and skeletal muscle. We report here on a case of microcystic adnexal carcinoma at an unusual site: the scalp. A 42-year-old woman presented with a 6-month history of a painful, solitary, 1 cm-sized, firm, fixed, dome-shaped nodule on the scalp, and this was accompanied by ulceration and oozing. A biopsy specimen demonstrated features of microcystic adnexal carcinoma, with both follicular and ductal differentiation. Immunohistochemical staining for CEA (carcinoembryonic antigen) was positive in the ductal structures, but this was negative in the follicular structures.

A Case of Microcystic Adnexal Carcinoma on the Scalp / 대한피부과학회지

Microcystic adnexal carcinoma is a rare, locally aggressive tumor with a high potential for local recurrence. It usually presents in elderly patients as a solitary, slowly growing, firm, indurated plaque on the face, and particularly on the upper lip or central face. It has occasionally been found in extra-facial locations, including the nipple, axilla and lower extremities, yet scalp involvement has been rarely reported. Histopathologically, it is a poorly circumscribed dermal tumor that shows both follicular and ductal differentiation, and it may extend into the subcutis and skeletal muscle. We report here on a case of microcystic adnexal carcinoma at an unusual site: the scalp. A 42-year-old woman presented with a 6-month history of a painful, solitary, 1 cm-sized, firm, fixed, dome-shaped nodule on the scalp, and this was accompanied by ulceration and oozing. A biopsy specimen demonstrated features of microcystic adnexal carcinoma, with both follicular and ductal differentiation. Immunohistochemical staining for CEA (carcinoembryonic antigen) was positive in the ductal structures, but this was negative in the follicular structures.

Microcystic Adnexal Carcinoma Treated by Mohs' Micrographic Surgery / 대한피부과학회지

Microcystic adnexal carcinoma (MAC) is an uncommon skin appendage neoplasm with both follicular and sweat duct differentiation. It usually extends microscopically far beyond assessed clinical margins and spreads locally in the dermal, subcutaneous fat, muscle, and perineural tissue planes. Although its metastasis is very rare, local recurrence rate after standard excision is 40~59%. Recently, Mohs micrographic surgery (MMS) was introduced for the treatment of MAC and a more favorable cure rate has been reported. We report a case of MAC, which was treated by MMS, which concurred with thymoma and thyroid papillary carcinoma in a 50-year-old woman.

A Case of Desmoplastic Trichoepithelioma, Showing Similar Histological Features to Microcystic Adnexal Carcinoma / 대한피부과학회지

Desmoplastic trichoepithelioma is a rare benign tumor which is most commonly found on the face of young females. The tumor has a raised, annular border and a depressed center. The histologic features are often confused with other skin diseases, especially with microcystic adnexal carcinoma. We report, herein, a case of desmoplastic trichoepithelioma in a 26-year-old man, confirmed by re-biopsy and CEA immunohistochemical study.

A Case of Microcystic Adnexal Carcinoma on the Scalp / 대한피부과학회지

Microcystic adnexal carcinoma, also known as sclerosing sweat duct carcinoma, is an uncommon skin appendage neoplasm with both follicular and sweat duct differentiation. It typically occurs on the upper lip or central face of middle-aged patients but rarely affects the scalp. We report a case of microcystic adnexal carcinoma which developed on the scalp. A 65-year-old woman presented with a 10-year history of an asymptomatic, 1x1.5cm sized, firm, fixed, and dome-shaped mass on the scalp. A biopsy specimen showed a dermal tumor extending into the subcutis. Both the follicular and ductal structure, and CEA staining revealed a weakly positive reaction in the glandular structure of the tumor.

A Clinical Experience of Microcystic Adnexal Carcinoma in Lower lip and Back

Microcystic adnexal carcinoma(MAC), or sclerosing sweat duct carcinoma, may best be considered as a sclerosing variant of ductal eccrine carcinoma. This tumor is a deeply invasive and aggressive neoplasm, which most commonly occurs on the skin of the upper lip, but it also occurs on the chin, nasolabial fold, or cheek. Local recurrence is common, however,metastases have not been reported. We have experienced 2 cases of microcystic adnexal carcinoma or sclerosing sweat ductal carcinoma, in lower lip including mandible bone, and in back. In the case of lower lip, the wide excision and partial mandiblectomy including 6 teeth and supraomohyoid neck dissection was performed, and the defect was covered with radial forearm free flap. For the prevention of lower lip drooping, we used the palmaris longus tendon which is harvested at the same time of flap elevation for the suspension of free radial forearm tissue. As the recipient vessel, the facial artery and facial vein was used. Immediately after the operation, the flap survival result was excellent, and the excised mandible bone and 6 teeth were replaced with denture. Fourteen months after the operation, the local recurrence is not notified. There was no difficulty of mastication nor speech, no lower lip drooping nor flap drooping. In case of back lesion, wide excision and split thickness skin graft was performed. We excised the lesion above level of the fascia. The split thickness skin was harvested from the left thigh, skin graft was performed on the fascia and completed in 10 days without loss.There is no local recurrence or specific complication except mild hypertrophic scar in the 10th month after the operation. We report these 2 cases with the review of literature that provide the guidance in treatment of microcystic adnexal carcinoma.

Combined microcystic adnexal carcinoma and squamous cell carcinoma arising in the ovarian cystic teratoma: a brief case report

Malignant transformation of mature cystic teratoma of the ovary can develop with an incidence of 1-2%. Squamous cell carcinoma is the most common malignant tumor arising in benign cystic teratoma. The authors have recently experienced a case of combined microcystic adnexal carcinoma and squamous cell carcinoma arising in a benign cystic teratoma of the ovary in a 72-year-old Korean woman. The right ovarian cystic mass had been ruptured and firmly adhered with salpinx and omental fat tissue on operation. Thickened cystic wall with yellowish white solid infiltrative lesion was noted grossly, and two different malignant tumors of microcystic adnexal carcinoma exhibiting both eccrine and hair follicular differentiation and squamous cell carcinoma were observed microscopically. PAS and CEA positivities suggested eccrine differentiation in areas of microcystic adnexal carcinoma.

Microcystic Adnexal Carcinoma: Report of two cases

The clincopathologic features of microcystic adnexal carcinoma are presented. Microcystic adnexal carcinoma is a recently described, very rare neoplasm characterized by a locally aggressive growth pattern, and as far as we know, only two reports on the microcystic adnexal carcinoma have been published in the Korean literature. Recently we experienced two cases of microcystic adnexal carcimoma occurred in a 32-year-old male and 27-year-old female patient. They had 2.5 x 2.0 x 2.0 cm and 1.2 x 0.9 x 0.9 cm sized, slowly growing mass in the glabella and below the lower lip, respectively. The immunohistochemical staining for CEA antigen stains the glandular structures but not pilar structures. So we thinked that this tumor would be originated from primitive adnexal cells differentiating into both pilar and eccrine structures.

Microcystic Adnexal Carcinoma: Report of a case

Microcystic adenxal carcinoma is an unusual, locally aggressive neoplasm that has recently been recongized as a clincopathologic entity. Its histologic appearance includes both pilar and eccrine differentiation. Microscopically, the tumor consisted of small cysts and gland-like structures in superficial portion. In other area, basaloid cell nests and abortive hair follicles in the sclerotic stroma were seen. The cysts were filled with secretory eosinophilic material, which was positively stained with Periodic acid-Schiff and carcinoembryonic antigen. Immuno-peroxidase staining for carcinoembryonic antigen supported the dual differnetiation of this neoplasm. Despite the benign histologic appearance, there was deep and extensive infiltration of the subcutaneous tissue.