Microcystic Adnexal Carcinoma: A Review of the Literature.

BACKGROUND: Microcystic adnexal carcinoma (MAC) is a rare, locally aggressive cutaneous neoplasm that commonly occurs on the face. OBJECTIVE: The purpose of this article is to comprehensively review the current literature on MAC pertaining to epidemiology, pathogenesis, clinical presentation, histology, immunohistochemistry, prognosis, follow-up, and treatment. MATERIALS AND METHODS: An extensive literature review was conducted using OVID MEDLINE and PubMed to identify articles relating to MAC. RESULTS: Microcystic adnexal carcinoma typically presents as a skin-colored nodule on the face. The pathogenesis is mostly related to pilar and eccrine differentiation. Histologically, MAC can mimic syringoma, desmoplastic trichoepithelioma, and infiltrative basal cell carcinoma. Diagnosis is challenging because superficial shave biopsies may reveal only benign findings that do not warrant further management. A deep biopsy is mandatory for the correct diagnosis, and Mohs micrographic surgery provides the highest cure rate. CONCLUSION: Microcystic adnexal carcinoma is a locally aggressive disease with histological margins that often far surpass what is clinically suspected. Mohs micrographic surgery is the standard of care for removal of these lesions. Patients with a history of MAC should be examined at least every 6 months for recurrence, metastasis, and development of additional skin cancers.

Angiotropic syringomatous carcinoma.

Syringomatous carcinoma (SC) is a slow-growing malignant skin tumor that usually affects the face or scalp. An 83-year-old female developed SC on the sole, a rare location. Histopathologically, numerous ducts with few keratinizing cysts were seen in the upper dermis, and cords, strands and nests with sclerotic stroma were seen in the deep dermis and subcutis. In addition to the perineural and intraneural invasion of the tumor, the tumor cells had also invaded the vessel walls. There was no intravasation of tumor cells or interruption of the endothelium. Because melanoma with vascular wall invasion without intravasation of melanoma cells or interruption of the endothelium has been called angiotropic melanoma, we termed the present tumor angiotropic SC. Tumor cells showed wide local invasion.

[Pathologic diagnosis and differential diagnosis of low-grade adenosquamous carcinoma of breast and syringomatous adenoma of nipple].

OBJECTIVE: To investigate the pathologic and immunohistochemical features, diagnosis and differential diagnosis of low-grade adenosquamous carcinoma of the breast and syringomatous adenoma of the nipple. METHODS: Six cases of low-grade adenosquamous carcinoma of the breast and four cases of syringomatous adenoma of the nipple were examined histologically and immunohistochemically (MaxVision method), and the literature was reviewed. RESULTS: The two types of tumors were similar in morphology, but located in different regions with low-grade adenosquamous carcinoma being present in the deep parenchyma and syringomatous adenoma in nipple. Both types of tumors were composed mainly of well-differentiated glands with angulated, comma shaped or polliwog appearance in a disordered infiltrative pattern. The tumor cells also formed solid tubules, strips or nests, with frequent areas of squamoid differentiation. Mitosis was rare. The interstitial tissue showed abundant spindle cells or sclerotic fibrosis with mixed inflammatory cells infiltration. One case of low-grade adenosquamous carcinoma showed a concomitant malignant adenomyoepithelioma, and another case showed concomitant spindle cell metaplastic carcinoma. One case of syringomatous adenoma involved the deep parenchyma. Immunohistochemistry of low-grade adenosquamous carcinoma showed that CK5/6 and p63 were positive in the outer layer of the glands and the squamoid epithelium, and CD10 was also positive in the outer layer of the glands. ER and HER2 were negative, and PR was also negative except for one case in which the spindle cells were positive for CK5/6, AE1/AE3 and PR focally. Immunostaining of syringomatous adenoma demonstrated that p63 and CK5/6 were positive in the outer layer of the glands and the squamoid epithelium. Calponin, SMA, ER, PR and HER2 were all negative. CONCLUSIONS: Low-grade adenosquamous carcinoma of the breast and syringomatous adenoma of the nipple are similar in morphology and immunohistochemical phenotype, while the biological features are opposite due to different locations. The differential diagnoses include tubular carcinoma, adenosquamous carcinoma, radial sclerosing lesions and others.

An immunohistochemical study of the origin of the solid strand in syringoma, using carcinoembryonic antigen, epithelial membrane antigen, and cytokeratin 5.

BACKGROUND: Although much research has been conducted into the origin of syringoma, the histogenesis and differentiation of it remains controversial. The published studies examined various antibodies, and our study is an additional immunohistochemical work-up. OBJECTIVE: We attempted to identify the cell that acts as the precise origin of a syringoma, based on a comparative analysis of carcinoembryonic antigen (CEA), epithelial membrane antigen (EMA), and cytokeratin (CK) 5 through immunohistochemical staining in the solid strand of basophilic epithelial cells of syringoma. METHODS: A total of 31 patients with biopsy-confirmed syringoma were included in this study. Each sample was analyzed with antibodies to CEA, EMA, and CK5. These markers were indicating each part of the normal sweat gland structure: CEA stains the luminal surface of sweat ductal structures; EMA stains the peripheral cells of normal dermal ducts and the intraepidermal duct; CK5 stains the outer cells of the dermal duct and lower intraepidermal duct but does not stain the intraepidermal duct located in the upper epidermis. RESULTS: We were able to confirm that the solid strands stained for EMA and CK5, as did the outer cells of the ductal structure. However, the solid strands did not stain with CEA. CONCLUSIONS: The results indicated that solid strands observed in syringomas originate from the outer cells of the two layers of cells that compose the lower epidermal duct or the transitional portion between the intraepidermal duct and dermal duct in the normal eccrine or apocrine structure. Thus, we surmise that a syringoma is developed by the proliferation of these cells.

Diagnostic pitfalls in syringocystadenocarcinoma papilliferum: case report and review of the literature.

We report the first case, to our knowledge, of syringocystadenocarcinoma papilliferum with p63-verified squamous differentiation and extensive dermal invasion accompanying in situ components. An 86-year-old woman presented with a neoplasm on the neck, and the intralesional heterogeneity typical of these neoplasms led to an initial diagnosis on needle biopsy favoring squamous cell carcinoma. Excision illustrated diverse morphology, raising a broad differential diagnosis, including more common extracutaneous malignancies, such as breast, gastrointestinal, and ovarian primary tumors. Fortuitous sectioning revealed a focal connection to the skin surface with evidence of apocrine differentiation allowing final diagnosis as syringocystadenocarcinoma papilliferum. Our literature review shows the histologic and immunohistochemical features of syringocystadenocarcinoma papilliferum are not well defined outside of their clear morphologic overlap with syringocystadenoma papilliferum. We describe our findings and diagnostic pitfalls to help pathologists encountering this unusual apocrine neoplasm.

Chondroid syringoma with tyrosine crystals: case report and review of the literature.

Chondroid syringoma (CS) is a relatively rare cutaneous mixed tumor arising from sweat glands. It usually presents in the head and neck area as an asymptomatic, slow-growing, firm, circumscribed, lobulated nodule within the dermis or subcutaneous fat. CSs share morphologic similarities with their salivary gland counterparts, pleomorphic adenomas (benign mixed tumors). Although the presence of tyrosine-rich crystalloids in mixed tumors of the salivary gland is well recognized, to our knowledge, this finding has not been previously described in mixed tumors of the skin. We report a case of tyrosine crystalline structures in a CS and review the pertinent literature.

Syringocystadenocarcinoma papilliferum: a case report.

Syringocystadenocarcinoma papilliferum (SCACP) is a rare form of adenocarcinoma of the skin. This is the malignant counterpart of syringocystadenoma papilliferum (SCAP) and usually develops on the scalp in a long-standing lesion identified clinically as SCAP. We describe a 65-yr-old Korean man with a nodule on the right supra-pubic area with a 2-yr duration. Histologically this tumor had a similar overall configuration as in SCAP, but the tumor was asymmetric and poorly circumscribed, extending into the deep dermis and showed cytologic atypia. The tumor cells showed positive reaction to GCDFP-15, but negative reaction to CEA and HMFG-1. We established the diagnosis of SCACP in the patient, and a wide excision was performed to remove the tumor. The patient has been well without relapse or metastasis for 2 yr.

Syringoma: a clinicopathologic and immunohistologic study and results of treatment.

The purpose of our study was to describe clinical and histopathological features of sixty one patients with histological diagnosis of syringoma over four year period in our dermatology clinic in Korea. Female:male ratio was 6.6:1 with onset of age during 2nd and 3rd decades in more than half of the patients in our study. The most frequently involved site was eyelids (43 cases, 70.5%) and the most common color of lesion was skin-color (30 cases, 49.2%). In 34 cases, characteristic tad-pole appearances (55.7%) were observed. Basal hyperpigmentation was observed more frequently in brown-colored lesion (p=0.005). Fibrosis was observed more frequently in erythematous lesion (p=0.033). Keratin cyst was observed less frequently in genital involved group (p=0.006). We also performed immunohistochemical stain for the presence of progesterone receptor (PR) and estrogen receptor (ER) in fifty six cases with negative results.

Syringomatous adenoma of the nipple: a case report.

We present a case of a 33-year-old woman who underwent excisional breast biopsy due to a left nipple mass. Histological examination revealed the morphologic and immunohistochemical pattern of syringomatous adenoma of the nipple. This is a rare lesion of the breast that can clinically mimic breast carcinoma, but harbors a benign and only locally aggressive course. Awareness of both the clinician and the pathologist for the possibility of diagnosing this tumor in the nipple region is mandatory to avoid mastectomy and lymph node dissection.

Expression of CD10 in basal cell carcinoma.

We investigated the expression of CD10 by an immunohistochemical method in 51 basal cell carcinomas (BCCs), eight pilomatricomas, five trichoblastomas, two trichofolliculomas, three sebaceomas, five sebaceous carcinomas, ten syringomas, two spiradenomas, ten poromas, four porocarcinomas, one eccrine duct carcinoma (not otherwise specified, NOS), six mixed tumors of apocrine origin, and nine squamous cell carcinomas (SCCs). We detected strong expression of CD10 in tumor cells of BCC (86%), and found that the smaller the number of positive tumor cells, the larger the number of positive stromal cells, in particular in sclerosing BCCs. Spearman's rank correlation test revealed a significant negative correlation in BCCs between the expression of CD10 in tumor cells and that in stromal cells (P = 0.001). In all pilomatricomas (100%) and in four trichoblastomas (80%), strong expression was also detected in tumor cells. There was no detectable expression in trichofolliculomas. One sebaceoma (33%) and two sebaceous carcinomas (40%) expressed CD10 in a similar fashion to BCCs. All tumors of eccrine gland origin, including syringoma, spiradenoma, poroma, porocarcinoma, and eccrine duct carcinoma (NOS), did not express CD10. Five mixed tumors (83%) were immunopositive. In SCC, CD10 was overexpressed only in the stromal cells. These findings support the hypothesis that BCC is derived from the folliculo-sebaceous apocrine unit, especially having the same origin as trichoblastoma and pilomatricoma. CD10 might be an indicator of tumor invasiveness if it is expressed in stromal cells, while it might be a marker of follicular differentiation if it is expressed in the actual tumor cells of cutaneous epithelial neoplasms.

Milium-like syringoma: a case study on histogenesis.

BACKGROUND: Milium-like syringoma is a variant of syringoma first described in 1987. Only few cases have been reported in the literature. It may be misleading clinically, and its histogenesis has not been clarified. CASE REPORT: We present a case of periorbital milium-like syringoma, with studies on the histopathologic, histochemical, and immunohistochemical features. RESULTS: Histology showed a large keratin-filled cyst in the upper portion of the lesion approximating the epidermis. Serial sections revealed that the cyst connected with the underlying syringomatous epithelial strands. Melanin was absent in the wall of the cyst, as demonstrated by Fontana-Masson stain. Cytokeratin 7 was expressed neither in the milia nor in the solid epithelial parts. Carcinoembryonic antigen (CEA) reactivity was seen in the luminal cells of the keratinous cysts. However, in the largest keratin-filled cyst clinically suggesting a milium, only the lower half of the cyst was positive for CEA. These results proved that the milia were part of syringoma with eccrine duct differentiation. Fusion of the upper half of the largest cyst with the epidermis may explain the absence of CEA positivity in this part, analogous with eccrine duct milia. Review of the literature indicated that this variant of syringoma occurs more often in Asians. CONCLUSION: We present evidence, and propose the histogenesis of milium-like syringoma, that it is a variant of syringoma with a prominent cystic component showing features of eccrine duct milia. Recognition of its nature is of therapeutic significance. Further studies are required to verify its clinical characteristics as compared with ordinary syringomas.

Carcinomatous transformation of eccrine syringofibroadenoma.

BACKGROUND: While squamous cell carcinoma and pseudocarcinomatous hyperplasia have been documented as pre-existing lesions in cases of reactive eccrine syringofibroadenoma (ESFA), to the best of our knowledge carcinoma occurring in a solitary ESFA has not yet been reported. We present one such case in a 91-year-old female who had a dome-shaped, reddish tumor on the extensor side of the left forearm. METHODS: We review the histopathological, immunophenotypical and ultrastructural findings of this tumor, including the keratin expression profile. RESULTS: Histopathologically, long, branching, anastomosing, thin and thick strands of small cuboidal epithelial cells were extending from the surface epidermis into the dermis. In the center of the tumor, there were irregular-shaped nests of atypical tumor cells invading downward into the dermis. Ultrastructurally, duct-like lumina lined with cuboidal tumor cells were present in the epithelial cords. From these findings, the present case was diagnosed as solitary eccrine syringofibroadenocarcinoma (ESFAC). Keratin expression studies revealed that cells of the thick strands, except for the luminal and basal cells, were positive for differentiation-specific keratins, keratins 1 and 10, and that cells of the thin strands were positive for keratins 5 and 14. CONCLUSIONS: Histopathological, immunophenotypical and ultrastructural evidence, as well as the pattern of keratin expression, suggest differentiation of the present malignant tumor towards the eccrine dermal duct. This case is the first reported case of ESFAC as far as we know.

Immunohistochemical analysis of keratin expression in clear cell syringoma. A comparative study with conventional syringoma.

Immunophenotypes, especially expression of cytokeratins, in 9 cases of clear cell syringoma were examined using antibodies against epithelial membrane antigen (EMA), and 17 kinds of monoclonal anti-keratin antibodies to investigate its histogenesis. In addition, 7 cases of conventional syringoma were selected for parallel assessment. Conventional syringoma expressed CK1 and CK10, which exists in the acrosyringium and the transitional portion between the acrosyringium and the dermal duct. Based on immunostaining with RCK102 and 35 beta H11, syringoma was thought to express CK5. Because expression of CK5 was observed in the basal cells of sweat duct ridge (lower acrosyringium) and the outer cells of the dermal duct, but not in the acrosyringium located at upper epidermis, we speculated that syringoma differentiated toward the transitional portion between the acrosyringium and the dermal duct. A comparative study of keratin expression between conventional and clear cell syringoma showed that there was no difference in the immunoreactivities. Based on the above observations, we confirmed that clear cell syringoma is a metabolic variant of conventional syringoma, and differentiates into the transitional portion between the acrosyringium and the dermal duct.