Cutaneous Adnexal Tumors Research Articles

The Risk of Lymph Node Involvement in Malignant Cutaneous Adnexal Tumors

Malignant cutaneous adnexal tumors (MCATs) are rare neoplasms that do not have a well-studied treatment algorithm. They are generally treated by excision alone. Given its successful application in other cutaneous malignancies, sentinel lymph node biopsy (SLNB) has been advocated by some for use in MCATs. A retrospective chart review was performed. Clinicopathological factors, recurrence patterns, and long-term follow-up were documented. Survival analysis was performed. Forty-eight subjects were identified. Mean age was 69 years with locations on the face (52%), including periocular sites (16%), extremities (25%), and all other sites (7%). Histologic distribution was sebaceous carcinoma (56%), porocarcinoma (17%), eccrine carcinoma (13%), adenocarcinoma (10%), and hidradenocarcinoma (4%). Mean follow-up was 3.9 years (range, 0 to 17 years). Nine subjects (18.8%) recurred locally, and recurrence was inversely associated with age (P = 0.04). Four (8.3%) demonstrated lymph node involvement, none without first developing local recurrence. Five-year disease-specific survival was 97 per cent (confidence interval, 81.4 to 99.6%). Despite being one of the largest reported analyses of MCATs, this study is limited by the small number of subjects. MCATs seemingly develop nodal recurrence only after demonstrating aggressive local biology. SLNB may best be applied selectively, possibly only in patients with local recurrence.

Primary Adenoid Cystic Carcinoma of the Skin Metastatic to the Lymph Nodes

Primary cutaneous adenoid cystic carcinoma (PCACC) is a rare adnexal skin tumor first described in 1975, of which merely 62 cases have so far been studied in detail and reported in the English literature. PCACC is usually regarded as apocrine in origin/differentiation, but its precise histogenesis is still not well known. PCACC has in most cases a rather indolent course but can produce local recurrences and, more rarely, regional (lymph node) and distant (pulmonary) metastases. We report herein a Greek woman with a long-standing PCACC that grew slowly over several years and produced metastasis in the regional lymph nodes, highlighting the potentially aggressive course of this tumor. The primary and metastatic tumors were studied immunohistochemically and proved to express several (sweat gland-related) antigens (such as keratin 7, epithelial membrane antigen, CD10, and CD117) but neither hormonal receptors nor p63 or Gross Cystic disease Fluid Protein 15. The salient clinicopathologic features of this rare cutaneous adnexal tumor are reviewed.

Transgenic mouse model of cutaneous adnexal tumors.

TMEM207 was first characterized as being an important molecule for the invasion activity of gastric signet-ring cell carcinoma cells. In order to unravel the pathological properties of TMEM207, we generated several transgenic mouse lines, designated C57BL/6-Tg (ITF-TMEM207), in which murine TMEM207 was ectopically expressed under a truncated (by ~200 bp) proximal promoter of the murine intestinal trefoil factor (ITF) gene (also known as Tff3). Unexpectedly, a C57BL/6-Tg (ITF-TMEM207) mouse line exhibited a high incidence of spontaneous intradermal tumors with histopathological features that resembled those of various human cutaneous adnexal tumors. These tumors were found in ~14% female and 13% of male 6- to 12-month-old mice. TMEM207 immunoreactivity was found in hair follicle bulge cells in non-tumorous skin, as well as in cutaneous adnexal tumors of the transgenic mouse. The ITF-TMEM207 construct in this line appeared to be inserted to a major satellite repeat sequence at chromosome 2, in which no definite coding molecule was found. In addition, we also observed cutaneous adnexal tumors in three other C57BL/6-Tg (ITF-TMEM207) transgenic mouse lines. We believe that the C57BL/6-Tg (ITF-TMEM207) mouse might be a useful model to understand human cutaneous adnexal tumors.

Trichogerminoma, a rare cutaneous follicular neoplasm with indolent clinical course: report of two cases and review of literature

Trichogerminoma is a rare cutaneous adnexal neoplasm of the hair germ cell and usually associated with benign clinical course and favorable outcome. Since its first description by Sau et al. in 1992, only a few cases have been reported up to date. Herein, we report two additional cases occurring in the hip and right thigh, respectively. Both patients are male, one is 78 years old, the other is 29 years old. Histological examination reveals well-circumscribed dermal nodule composed of lobules of basaloid cells with surrounding pseudocapsule. The distinct characteristic of the tumor is that most of the lobules display a special pattern of round nests or cell balls arranged in the central part with the peripheral palisading. Immunostaining showed ring-like fashion of CK5/6, P63 and Bcl-2 with negative or weak staining in the “cell balls”. There was no recurrence after complete excision during the period of follow-up. To the best of our knowledge, this is the first report of trichogerminoma in Chinese population. In contrast to the previously reported cases, ours present the similar morphological features with distinct immunohistochemical characteristics. We consider the concept of trichogerminoma exists with no doubt by its identifiable morphological features, and it should be classified as a variant of trichoblastoma. Because of its malignant potential, complete excision is a prior choice of treatment for this rare but distinctive tumor.Virtual slidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1558612241110439.

What is New in Adnexal Tumors of the Skin?

This article reviews the recent dermatopathology literature regarding cutaneous adnexal neoplasms, with emphasis on new and underrecognized entities, "old entities" with new findings, advances in immunohistochemistry, and new findings in relation to inherited disorders associated with cutaneous adnexal neoplasms.

Survival after resection of cutaneous adnexal carcinomas with eccrine differentiation: Risk factors and trends in outcomes

Current staging systems do not specifically address cutaneous adnexal carcinomas with eccrine differentiation. Due to their rarity, prognosis and management strategies are not well established. A population-based study was performed to determine prognostic factors and survival. Patients diagnosed with cutaneous adnexal carcinomas with eccrine differentiation were identified using the surveillance, epidemiology, and end results population-based cancer registry. Associations between risk factors, treatment modalities, and survival were calculated using logistical regression, Kaplan-Meier estimates and log-rank analysis. The incidence of distinct eccrine subtypes was determined within 1,045 patients with cutaneous adnexal tumors containing eccrine differentiation. All-cause 5-year survival (OS) was 82%, while age-adjusted survival was 94%. Patients with microcystic adnexal carcinoma had improved OS (90%) compared to patients with hidradenocarcinoma (74%), spiradenocarcinoma (77%), porocarcinoma (79%), and eccrine adenocarcinoma (81%). The majority of patients were treated with surgical excision and a small subset with surgery plus radiation, with similar OS. Patients with well-to-moderately differentiated tumors demonstrated improved OS compared to those with poorly differentiated/anaplastic disease. Histological subtype and grade were associated with survival, and should be specified in biopsies and excised specimens. Surgical excision is appropriate, and the addition of adjuvant radiation may not be associated with survival. These results highlight survival data and high-risk prognostic factors that warrant prospective validation, and may augment current staging systems.

A Case of Tubular Apocrine Adenoma with Syringocystadenoma Papilliferum of External Auditory Canal Removed by Postauricular Approach

Tubular apocrine adenoma (TAA) and syringocystadenoma papilliferum (SCAP) are uncommon cutaneous adnexal neoplasms often found in the head, perianal area, but rarely in external auditory canal (EAC). SCAP in EAC generally presents itself with a mass in the EAC along with conductive hearing loss. We present here a case of a 50-year-old man presenting TAA with SCAP confined to EAC, which was surgically removed. The clinical and histopathological features were described. The patient has been well without relapse or metastasis. � Korean J Otorhinolaryngol-Head Neck Surg 2013;56:229-32 Key WordsZZAdenoma ㆍApocrine glands ㆍExternal auditory canal ㆍNeoplasms.

How to differentiate mucinous eccrine carcinoma from cutaneous metastasis of breast carcinoma?

Eccrine skin tumors are rare and represent only 0,05% of all cutaneaous neoplasms. They represent a pitfall especially with cutaneous metastases of carcinoma which are more frequent. We report the case of a 60-year-old woman presented with a frontal scalp mass whose histologic and immunohistochemical features concluded initially to a cutaneous metastasis of breast carcinoma. The diagnosis was reviewed because of the absence of a breast lesion. The final diagnosis was primary sweet gland carcinoma. Histologic distinction between cutaneous metastatic breast carcinoma and primary cutaneous adnexal neoplasms can be very challenging or even impossible. This case illustrates this difficulty and puts emphasis on the necessity of keeping in mind the distinctive features between these two entities.

Radiofrequency ablation: A safe and economical modality in treatment of Brooke-Spiegler syndrome

Brooke-Spiegler syndrome is an uncommon disease. Patients have a predisposition to develop cutaneous adnexal neoplasms such as cylindromas, trichoepitheliomas, spiradenomas, trichoblastomas, basal cell carcinomas, follicular cysts, and organoid nevi. Malignant transformation of preexisting tumors also occurs in these individuals. Various techniques have been used for the treatment of trichoepitheliomas and cylindromas including excision, electrocautery, carbon dioxide laser ablation, cryosurgery, and radiotherapy. In our case, cylindromas were ablated by radiofrequency in multiple sittings. Trichoepitheliomas were ablated using coagulation mode with power # 3 to 3.5. Cosmetically acceptable results were obtained in 100 percent of the cylindromas and 70 percent of the trichoepitheliomas (Visual Analog Scale). The radiofrequency ablation technique under different modes can be used in both large tumors as well as smaller ones, especially in developing countries because it is very cost effective and easily accessible.

Syringocystadenocarcinoma Papilliferum With Transition to Areas of Squamous Differentiation

Syringocystadenocarcinoma papilliferum (SCACP) is an exceedingly rare cutaneous adnexal neoplasm, which is typically located in the head and neck, and perianal area. Very few cases have been reported in the literature. Here, we report a case of SCACP with evident transition to squamous differentiation. A 75-year-old white woman presented with 1-year history of a solitary tender nodule in the left upper arm. Physical examination revealed a single, 1.5 × 1.1-cm, erythematous ulcerated nodule within a background of red patch. Biopsy showed an adnexal carcinoma connected to the epidermis and composed of cystic papillary projections admixed with solid basaloid areas with marked cytologic atypia. The basaloid tumor cells appeared to blend with the squamous component that demonstrated ductal formation, which was highlighted by carcinoembryonic antigen. Tumor cells were reactive for both cytokeratins 5/6 and 7. This case represents SCACP arising from syringocystadenoma papilliferum in the upper arm, with distinct transition to areas of squamous differentiation.

Cytologic features of clear cell adnexal carcinoma in 3 dogs

Clear cell adnexal carcinoma is a rare cutaneous adnexal neoplasm without definitive apocrine, sebaceous, or follicular differentiation. Differential diagnoses include sebaceous carcinoma, liposarcoma, and balloon cell melanoma. Immunohistochemical analysis, with positive immunoreactivity for pancytokeratin and vimentin, aids in the diagnosis. Cytologic features of clear cell adnexal carcinoma have not been described previously. The aim of this study was to describe cytologic features of canine clear cell adnexal carcinoma. Fine-needle aspirates (FNA) obtained prior to biopsy of cutaneous neoplasms with a histologic diagnosis of clear cell adnexal carcinoma confirmed by immunohistochemical analysis were reviewed retrospectively. Slides prepared from FNA had been stained with modified Wright-Giemsa or automated aqueous Romanowsky stain. Of 20 neoplasms diagnosed as clear cell adnexal carcinoma in dogs, FNA of the mass had been performed in 3 cases. Cytologic features were similar and included high cellularity, marked cellular pleomorphism, loose arrangement of neoplastic cells, and a light blue to gray background resulting from streaming of cytoplasm from ruptured cells. Neoplastic cells were oval to polygonal to spindle-shaped with wispy cytoplasmic projections. Cytoplasmic eosinophilic stippling, globular deposits, or pink needle-shaped inclusions were noted. Criteria of malignancy included marked anisocytosis, anisokaryosis and anisonucleoleosis, multinucleation, karyomegaly, macronucleoli, and atypical mitotic figures. Clear cell adnexal carcinoma should be included in the cytologic differential diagnosis for a canine cutaneous mass even if an epithelial origin is not readily identified owing to lack of characteristic epithelial features, such as highly cohesive cell clusters, evident cell-to-cell junctions, and distinct cytoplasmic edges.

Trichoblastic Fibroma of the Eyelid

Trichoblastic fibroma (TBF), also known as trichoblastoma, small nodular type, is a rare benign cutaneous adnexal tumor that shows trichogenic (follicular) differentiation that can be mistaken clinically and histologically for basal cell carcinoma (BCC), a locally aggressive trichogenic carcinoma. As the clinical behaviors of these two follicular neoplasms are opposite from one another and their management is thus different, recognition of TBF is crucial for effective therapy. Although TBF most commonly is first seen on the face, this lesion has not previously been reported in the eyelid. In this report, we document the first case of TBF affecting the ocular adnexa and detail the histologic features that differentiate TBF from BCC.

Nodular hidradenoma of anterior abdominal wall

Nodular hidradenoma is a variant of benign cutaneous adnexal tumors which arises from eccrine sweat glands. They occur frequently on the scalp, face, abdomen, and extremities. The clinical appearance of this lesion is not specific and differential diagnosis can only be done after complete removal of the lesion. We report a case of nonhealing ulcer on anterior abdominal wall in a 35 year male. The ulcer occurred following excision of a recurrent anterior abdominal wall swelling of 4 year duration. Wedge biopsy was done in this institution and it was inconclusive. The ulcer was managed with antiseptic dressing, followed by skin grafting. Patient developed recurrent ulcero-proliferative lesion involving the same area in 3 months period. Wide excision of the ulcer revealed features of nodular hidradenoma. Nodular hidradenoma is reported rarely.

A Cutaneous Adnexal Neoplasm With Features of Adamantinoid Trichoblastoma (Lymphadenoma) in the Benign Component and Lymphoepithelial-Like Carcinoma in the Malignant Component: A Possible Case of Malignant Transformation of a Rare Trichoblastoma Variant

The authors describe a case of cutaneous adnexal tumor with a component reminiscent of adamantinoid trichoblastoma (lymphadenoma) that shows gradual transition to a high-grade carcinoma resembling lymphoepithelial-like carcinoma. The tumor was located above the upper lip of a 57-year-old woman and metastasized in regional lymph nodes. This seems to be the first documented report of malignant transformation of adamantinoid trichoblastoma. We discuss also other possibilities of histogenesis of this unique neoplasm.

PLAG1 expression in cutaneous mixed tumors: an immunohistochemical and molecular genetic study

Cutaneous mixed tumors, also known as chondroid syringomas, are benign cutaneous adnexal tumors that exhibit remarkable histopathological similarities to pleomorphic adenoma of the salivary gland. Thus far, there is little information on the genetic profiles of cutaneous mixed tumors, although specific genetic aberrations including fusion genes involving PLAG1 and HMGA2 have been demonstrated in pleomorphic adenomas. In the present study, we conducted an immunohistochemical evaluation of PLAG1 and a reverse transcription-polymerase chain reaction (RT-PCR) assay to detect fusion gene transcripts associated with pleomorphic adenoma, including the CTNNB1-PLAG1, LIFR-PLAG1, CHCHD7-PLAG1, TCEA1-PLAG1, HMGA2-FHIT, HMGA2-NFIB, and HMGA2-WIF1 fusion transcripts; this was performed using formalin-fixed paraffin-embedded tumor tissue specimens of 16 cutaneous mixed tumors including one sample with an adenocarcinoma component. All 16 cutaneous mixed tumors were immunoreactive to PLAG1, which was predominantly expressed in cells with myoepithelial or chondroid differentiation accounting for >80% of cells, whereas PLAG1 expression in glandular or squamous tumor cells was restricted to <20% of cells. The carcinoma component in the mixed tumor was also positive for PLAG1. On the other hand, all eight cutaneous adnexal tumors other than the mixed tumor were negative for PLAG1. In RT-PCR analysis, no fusion gene transcripts involving PLAG1 or HMGA2 were identified in any of the cases. Concordant with previous studies, our results support the close relationship between cutaneous mixed tumors and pleomorphic adenomas of the salivary gland. However, the mechanism of PLAG1 expression in cutaneous mixed tumors appears to be possibly different from that of pleomorphic adenomas.

Rare Tumors Through the Looking Glass

To identify prognostic factors related to malignant cutaneous adnexal tumors. Population-based study using the Surveillance, Epidemiology, and End Results database from January 1, 1988, through December 31, 2006. Seventeen population-based cancer registries. A total of 4032 patients with malignant cutaneous adnexal tumors. Overall survival (OS) and disease-specific survival (DSS). Ten-year OS and DSS rates were 54% and 97%, respectively. Unfavorable factors for OS were increasing age (hazard ratio [HR] 1.08; 95% confidence interval [CI], 1.07-1.09; P < .001), distant metastases (3.26; 2.34-4.53; P < .001), no surgical procedure (1.27; 1.01-1.59; P = .04), grade IV tumors (1.97; 1.18-3.28; P = .009), nodal metastases (2.19; 1.40-3.43; P = .001), and T3 tumors (1.37; 1.00-1.87; P = .04). Favorable factors for OS were a wide surgical excision (HR, 0.78; 95% CI, 0.68-0.89; P < .001), female sex (0.73; 0.65-0.82; P < .001), malignant eccrine spiradenoma (0.72; 0.53-0.99; P = .04), and histologic findings of sweat duct carcinoma (0.63; 0.44-0.90; P = .01). Unfavorable factors for DSS included N1 status (HR, 6.77; 95% CI, 2.11-21.68; P < .001), distant metastases (12.24; 6.03-24.85; P < .001), histologic findings of malignant eccrine spiradenoma (5.62; 1.25-25.34; P = .02), and no surgical procedure (2.81; 1.09-7.23; P = .03). Favorable factors for DSS included female sex (HR, 0.52; 95% CI, 0.30-0.91; P = .02). Five-year survival among patients with malignant cutaneous adnexal tumors is good in the absence of distant metastases. Wide resection may be preferable to less aggressive excision. The prognostic importance of lymph node metastases warrants consideration of lymph node basin staging.

Cutaneous Neoplasms With Prominent Verocay Body–Like Structures: The So-called “Rippled Pattern”

A striking appearance resulting from alternating areas of epithelial cell cords and stroma seen in some cutaneous adnexal neoplasms has been referred to as the "rippled pattern." Histologically, this pattern may be indistinguishable from Verocay bodies described in schwannomas. A number of common and clinically diverse cutaneous neoplasms can be linked by the presence of this unusual growth pattern. The heterogeneous group of tumors that have been known to demonstrate this feature includes those with epithelial, adnexal, fibrohistiocytic, mesenchymal, and melanocytic lineage. The objective of this review is to alert the dermatopathologist to the range of neoplasms, which can potentially show this attribute, so that a misdiagnosis can be avoided.

Cutaneous Mixed Tumor, Eccrine Variant: A Clinicopathologic and Immunohistochemical Study of 50 Cases, With Emphasis on Unusual Histopathologic Features

Mixed tumor, eccrine type, is a rare cutaneous adnexal neoplasm, mostly reported as isolated case reports. A systematic analysis of its histopathologic and immunohistochemical features has not previously been performed on a large series. The purpose of our investigation was to study a large number of cutaneous eccrine mixed tumors so as to fully characterize the entire spectrum of changes in the epithelial and stromal components, with an emphasis on unusual histopathologic features that may represent a diagnostic pitfall. This article reports a light microscopic and immunohistochemical study of 50 cases of eccrine mixed tumor, complemented by a literature review. Our study identified some unusual histopathologic features, thus extending the morphologic spectrum of this neoplasm. These included prominent cribriform areas, clear cell change, pseudorosette structures, prominent osseous metaplasia, and physaliphorous-like cells. Most of these features have not been previously recorded in eccrine mixed tumors and may represent a potential diagnostic pitfall.

Recurrent CYLD Nonsense Mutation Associated With a Severe, Disfiguring Phenotype in an African American Family With Multiple Familial Trichoepithelioma

To the Editor:Recently, Kazakov et al1 performed a clinicopathologic and genotypic analysis on a series of 16 patients suffering from multiple (familial) trichoepitheliomas (MFT: MIM 601606) and found mutations in the CYLD gene in 46%. Based on the frequent presence of CYLD mutations in MFT, it, alo

Linear syringocystadenoma papilliferum: An uncommon event with a favorable prognosis

Syringocystadenoma papilliferum (SCAP) is an uncommon cutaneous adnexal tumor of uncertain histogenesis. Various authors have postulated that the tumor differentiates toward apocrine gland, eccrine gland, or the apo-eccrine gland. We present a patient with an unusual linear form.