Case Of Microcystic Adnexal Carcinoma Research Articles

Expression of Hedgehog signalling molecules inmicrocystic adnexal carcinoma.

Microcystic adnexal carcinoma (MAC) is a rare skin neoplasm that has not been characterized on a molecular basis. To assess expression profiles of Hedgehog (HH) signalling molecules in MAC and control tumours. Immunohistochemistry was performed for Sonic Hedgehog (SHH), Indian Hedgehog (IHH), Patched 1 (PTCH1) and Smoothened (SMO) on patient MAC tissue (n=26) and control tumour tissue, including syringoma (SyG; n=11), trichoepithelioma (TE; n=11) and basal cell carcinoma (BCC; n=12) tissues. Patched 1 and SMO immunoreactivity was significantly higher in BCC than in SyG, TE or MAC (P<0.001 and P<0.03, respectively). The highest IHH expression was observed in BCC and TE compared with SyG and MAC (P<0.04). Notably, the highest SHH protein expression was observed in SyG compared with MAC, TE and even BCC (P<0.001). In patients with MAC, SMO immunoreactivity significantly (r=0.51; P<0.01) correlated with PTCH1 expression. Further correlation studies did not show significant associations between the HH expression markers assessed (P>0.05). Our results indicate that alterations of the HH signalling are unlikely to play a major role in the pathogenesis of MAC, which is in contrast to the morphologically similar BCC and TE. Our observation provides additional information to the limited molecular pathology knowledge on this rare tumour.

A Case of Microcystic Adnexal Carcinoma Arising on the Eyebrow

A Case of Microcystic Adnexal Carcinoma Arising on the Eyebrow

Carcinome annexiel microkystique : à propos d’une observation

We report a case of microcystic adnexal carcinoma in a 80-year-old woman. This is a rare malignant adnexal cutaneous tumor with glandular and follicular differentiation, rare, often asymptomatic, with late diagnosis and slow growth, locally aggressive and rarely metastatic. The distinction with other benign and malignant skin tumors is difficult.

Microcystic adnexal carcinoma—diagnostic criteria and therapeutic methods: case report and review of the literature

Microcystic adnexal carcinoma (MAC) is a rare, infiltrating, locally aggressive cutaneous neoplasm of combined follicular and eccrine/apocrine histogenesis, usually presenting on the upper lip or face. Differentiation from other adnexal tumours is very important because the clinical management of these tumours is radically different, and misdiagnosis may lead to incorrect treatment. A case of recurrent MAC in the upper lip, treated with multiple excisions and postoperative radiation therapy (PORT), is presented herein. There have been no signs or symptoms of recurrence since the subsequent reconstructive surgery and PORT. Based on reports in the literature it appears that although immunohistochemistry can be helpful in distinguishing between MAC and other adnexal tumours, careful histopathological examination is essential for an accurate diagnosis. Perineural and intramuscular invasion strongly suggest the diagnosis of MAC. Its predilection for the facial area often limits the width of surgical excision. In such cases, PORT may be considered.

後頭部microcystic adnexal carcinomaの1例と切除範囲の検討

75歳，女性。10年前から後頭部の皮下腫瘤を自覚していた。生検では，腫瘍は真皮から皮下脂肪織にかけてびまん性に浸潤し，線維化を伴っていた。毛包と汗器官への分化を示しており，病理組織学的にmicrocystic adnexal carcinomaと診断し手術を施行した。水平方向の安全域は文献的考察から20 mmとし，深部方向の安全域は腫瘍細胞が存在する層より一層深い層で切除する原則に則り，僧帽筋の一部と骨膜をつけて切除した。術後，永久標本で切除断端陰性であることを確認し，遊離前外側大腿皮弁を用いて再建した。術後半年が経過し，腫瘍の再発は認めていない。

Correlation of optical coherence tomography and histology in microcystic adnexal carcinoma: a case report

Herein, we report a case of microcystic adnexal carcinoma (MAC), which we correlated and evaluated by optical coherence tomography (OCT) and conventional H&E histology. A commercially available OCT scanner was used for imaging. Several multi-slice images were obtained from the central portion of the lesion. Correlation of OCT findings with histology was performed retrospectively. Microcystic adnexal carcinoma showed characteristic criteria, which were divided into superficial and sub-epidermal findings. The use of OCT can visualize characteristic criteria of MAC, thus enabling prompt diagnosis before surgery.

Microcystic Adnexal Carcinoma With Multiple Areas of Follicular Differentiation Toward Germinative Cells and Specific Follicular Stroma (Trichoblastomatous Areas)

We present a case of microcystic adnexal carcinoma (MAC) occurring on the upper lip of a 63-year-old man, which, in addition to the predominant areas of a conventional MAC, manifested multiple foci of follicular differentiation resulting in trichoblastomatous appearances. Some areas were highly reminiscent of desmoplastic trichoepithelioma (columnar trichoblastoma). We also reviewed 24 cases of MAC in our files, but in none of the cases were similar features observed, indicating that such histopathological findings are rare in MAC.

Microcystic adnexal carcinoma: a case report

A case of microcystic adnexal carcinoma is reported. The patient was a 43-year-old female, who had presented with a plaque on the left upper lip for more than 2 years. Skin examination revealed a demarcated, skin-colored plaque measuring 2.5 cm in diameter with telangiectasia on the surface of the left upper lip. Skin biopsy showed that the tumor lied in the dermis, infiltrating deeply into the underlying nervous and muscle tissue. There were many ductal structures and a small number of ramified glands with apocrine features in the upper dermis, strands of basophilic epithelial component imbedded in a markedly hyalinized stroma in the mid and lower dermis, as well as small and round keratinous cysts in the lower dermis. Immunohistoehemistry revealed strong positive staining of the epithelial components with cytokeratin. The staining for CEA and epithelial membrane antigen (EMA) was noted in the superficial ducts and glands. S-100 stain showed the infiltration of peripheral nerve with cytokeratin-positive epithelial components. A diagnosis of microcystic adnexal carcinoma was made. The patient underwent an excisional surgery and was followed up. Key words: Carcinoma, skin appendage; Sweat gland neoplasms; Rare diseases

上口唇部microcystic adnexal carcinomaに対する治療経験

上口唇部microcystic adnexal carcinomaに対する治療経験

上唇部皮下に発症したｍｉｃｒｏｃｙｓｔｉｃ ａｄｎｅｘａｌ ｃａｒｃｉｎｏｍａの１例

Microcystic adnexal carcinoma (MAC) is a rare malignant tumor of skin appendages. This tumor arises most commonly in the upper lip in the maxillofacial region. MAC is characterizedby a relatively slow clinical course, a strong tendency toward infiltration, and a low frequency of remote metastasis. Recently, we treated a case of MAC arising in the labial subcutaneous region. The patient was a 54-year-old man who noticed a mass in the right upper lip nearly 20 years ago. Since the tumor gradually became larger, he was referred to our hospital by a nearby clinic. At presentation, an extremely hard and immovable induration (20×15mm) was discovered at the upper lip. A benign tumor derived from a skin appendage was diagnosed on the basis of the clinical course of 20 years. Surgery was recommended, but the patient refused. He therefore underwent follow-up observation. When the tumor rapidly enlarged in March 2005, squamous cell carcinoma was diagnosed on the basis of a biopsydone in June. The tumor was resected with the patient under general anesthesia. Histopathological examination revealed no relation between the tumor and the skin, and imaging studies revealed continuous infiltration to the deep muscular layer. Proliferation of atypical basal cell-like squamous cells was found with microcystic lesions in the parenchyma of thetumor. On the basis of these findings, MAC was diagnosed. Neither local recurrence nor remote metastasis has been detected as of 2 years after resection.

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.

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 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 of the Nipple

Microcystic adnexal carcinoma (MAC) is a rare infiltrative cutaneous tumor, but with its locally aggressive nature, high rate of recurrence, and rare metastatic potential, it is an important clinical entity to consider when faced with a nipple lesion. These tumors are frequently misdiagnosed based on superficial biopsy alone. This case of a 15-year-old girl with MAC of the nipple demonstrates the importance of having a high index of suspicion in order to obtain a diagnosis, to determine the extent of disease, and to achieve adequate resection before reconstruction. Close clinical observation is recommended due to the possibility of future recurrences. Between the confusing nomenclature, indolent course, and inadequacy of superficial biopsy to achieve an accurate diagnosis, MAC is a clinical entity that the plastic surgeon must be aware of in order to facilitate proper diagnosis and treatment.

Metastatic Microcystic Adnexal Carcinoma

BACKGROUND Microcystic adnexal carcinoma is a clinically aggressive, local destructive sweat gland carcinoma with a high rate of recurrence, but regional or distant metastasis is quite rare. OBJECTIVE The objective was to describe an autopsy case of microcystic adnexal carcinoma with hematogenous metastasis and perineural intracranial spreads. METHODS This is an autopsy case of a 73-year-old woman with hematogenous metastasis of microcystic adnexal carcinoma followed for more than 20 years. RESULT The autopsy study revealed that the metastatic lesions were located in the perineural intracranial spread, left clavicle bone, bilateral 12th ribs, and liver. CONCLUSION Physicians should be aware that microcystic adnexal carcinoma is capable of metastasis to the liver and bones with intracranial spread along the optic nerve into the cranium.

Carcinoma anexial microquístico de células claras

Abstract—Microcystic adnexal carcinoma is an infrequently occurring, locally aggressive neoplasm. We present a case of microcystic adnexal carcinoma in the nasal dorsum of a 58-year-old woman. Microscopically, the tumor showed calcified horny cysts in the outer surface of the neoplasm, and was primarily made up of nests and cords of clear cells that completely affected the dermis and the subcutaneous cellular tissue, invading the skeletal muscle and the perineural spaces. The stroma that surrounded the neoplasm was desmoplastic, with minimal cytological atypia. The presence of clear cells has occasionally been described in microcystic adnexal carcinoma.

Microcystic adnexal carcinoma (MAC) of the scalp with extensive pilar differentiation.

Microcystic adnexal carcinoma (MAC), or sclerosing sweat duct tumor, is an uncommon, locally aggressive tumor. It typically involves the upper lip or face of middle-aged adults and rarely involves the scalp. It is characterized histologically by both pilar and eccrine differentiation and is associated with frequent perineural invasion. MAC is frequently misdiagnosed because of its bland and asymptomatic clinical presentation. In addition, its defining histologic features may be missed with a superficial biopsy. To describe a case of MAC in the scalp treated with Mohs surgery. We describe a case with extensive pilar differentiation and clear cell changes. In addition, the clinical and histologic characteristics of this rare neoplasm are reviewed. Mohs micrographic surgery (MMS) was performed on this neoplasm. The tumor was successfully excised in one surgery (five stages) and is without evidence of recurrence at 18 months. We present a case of MAC of the scalp in a 64-year-old white man treated successfully with MMS.

Microcystic Adnexal Carcinoma of the Vulva

Background. Microcystic adnexal carcinoma (MAC) is a subset of sweat gland carcinoma first described as a specific entity by D. J. Goldstein, R. J. Barr, and D. J. Santa Cruz (Cancer 1982;50:566–72). We report the first case of MAC occurring on the vulva and review the literature pertaining to this rare tumor.Case. A 43-year-old multiparous black woman presented initially to Kings County Hospital Medical Center with a chief complaint of a vulvar lesion arising on the left labia majora which she had noted for 4 years prior to presentation. Aside from increasing paresthesia in the area, she denied any constitutional symptoms. Her past medical history was significant only for hyperthyroidism and mild hypertension and surgical history was noncontributory. Gynecologic history was unremarkable, with sporadic care over the last 20 years. Physical examination revealed a 1.5 × 2.0-cm raised, well-circumscribed, firm mobile lesion on the left labia majora. It was noted to be yellow in color with the surrounding tissue being unremarkable in character. The remainder of her gynecologic examination and lymph node survey was unremarkable. Preoperative chest X ray was negative as was the CAT scan of the abdomen and pelvis. All laboratory values were within normal limits. A Pap smear done preoperatively was significant for atypical squamous and glandular cells of undetermined significance. Subsequent colposcopic examination of the cervix was remarkable for cervicitis and was adequate, with the entire transformation zone visualized. Both endocervical curettage and endometrial biopsy were normal. Initially, an excisional biopsy was performed with final pathology demonstrating microcystic adnexal carcinoma with positive surgical margins. She subsequently underwent a left radical hemivulvectomy with bilateral inguinal groin lymph node dissection. At the time of surgery, the left labia majora was noted to be well healed, with a residual surgical scar easily discernible. No areas of discoloration were noted and digital palpation of the area was unremarkable. Microscopic residual tumor was noted; however, all surgical margins and lymph nodes were negative for tumor. Her postoperative course was unremarkable. The patient has continued to do well since the time of her surgery and is being followed conservatively.Conclusion. Radical vulvectomy should be performed when MAC occurs in the vulva to secure negative margins of resection. Groin dissection should be reserved for cases in which the inguinal lymph nodes are clinically suspicious or in cases of tumor recurrence.

Microcystic adnexal carcinoma: First reported case in an African American man

Microcystic adnexal carcinoma (MAC) is a relatively uncommon adnexal neoplasm that can display aggressive local invasion. MAC occurs most commonly on the central part of the face and can be clinically asymptomatic with a benign appearance. We describe the first reported case of MAC in an African American man who was treated by Mohs micrographic surgery. (J Am Acad Dermatol 2001;45:283-5.)

Cytogenetic and fluorescence in situ hybridization analyses of a microcystic adnexal carcinoma with del(6)(q23q25)

Cytogenetic analysis of a case of microcystic adnexal carcinoma (MAC) revealed three unrelated clones with the karyotypes: 46,XX,del(6)(q23q25)[17]/46,XX,1∼2dmin[4]/46,XX,t(1;3) (p10;q10)[2]. Analysis of the 6q- by fluorescence in situ hybridization (FISH) showed that it had resulted from a long arm deletion. The finding of a clonal 6q deletion as the sole karyotypic change in a MAC is of special interest because we previously have identified 6q deletions in different types of malignant salivary gland tumors. This observation, together with the histopathologic similarities between salivary gland tumors and sweat gland tumors, further emphasize the histogenetic relationships between these tumor types.