Benign Adnexal Tumors Research Articles

Squamous cell apocrine hidradenoma

Apocrine hidradenoma is a benign adnexal neoplasm with apocrine differentiation. The neoplasm is composed of four different types of epithelial cells, including pale or clear cells, polygonal cells, mucinous cells and squamous cells, with variable proportions of them from case to case. In most examples of this neoplasm, clear or the polygonal cells are predominant, whereas the other types of neoplastic cells are less abundant. We report two cases of apocrine hidradenoma mostly composed of squamous cells. Histopathologic examination showed that the neoplasms were composed of both solid and cystic areas. The solid aggregations of neoplastic cells were composed of a peripheral layer of basaloid polygonal cells, whereas squamous cells forming the bulk of the aggregations. These squamous cells showed large eosinophilic cytoplasm and vesicular nuclei with prominent nucleoli. In one case, small foci of mucinous cells could also be seen in some aggregations of neoplastic cells, mostly around ductal structures. In both the cases, some of the tubular structures lined by epithelial cells showed evidence of decapitation secretion in their luminal border. The neoplastic stroma consisted of sclerotic collagen bundles when compared with adjacent normal dermis, and artefactual clefts separated the neoplasms from the surrounding tissue. The rare cases described in this report are exceptional because most of the neoplastic cells showed squamous appearance and for that reason we think that squamous cell apocrine hidradenoma is the most appropriate name for these neoplasms.

Eccrine syringofibroadenoma with co‐existent squamous cell carcinoma

Eccrine syringofibroadenoma (ESFA) is a rare, benign adnexal tumor arising most often on the extremities of elderly individuals. It is typically a slow-growing, flesh- to reddish-colored nodule or plaque. Histologically, the tumor consists of anastomosing cords of cuboidal epithelial cells surrounded by a fibrovascular stroma containing plasma cells. The cords contain scattered ductal structures lined with cuboidal cells resembling eccrine ducts. The co-existence of ESFA with squamous cell carcinoma has been described, eliciting the term eccrine syringofibroadenoma. The differential diagnosis includes poroma, porocarcinoma, fibroepithelioma of Pinkus and clear cell acanthoma. ESFA stain positively with epithelial membrane antigen and carcinoembryonic antigen. Cytokeratin studies have been inconsistent.

Congenital desmoplastic trichoepithelioma

Desmoplastic trichoepithelioma (DT) is a rare benign adnexal neoplasm considered to have follicular differentiation. It usually presents as an asymptomatic, firm, annular plaque with a raised border. The most common site of occurrence is the face, usually on the cheek. Females are more often affected than males and the age range of patients previously reported is 8-79 years. We present a case of congenital desmoplastic trichoepithelioma. A girl was born at term to a healthy mother after an uneventful pregnancy and was noted to have widespread erythematous plaques with milia-like lesions over the right scalp, face and neck, with some areas of atrophic scarring. Histology and immunohistochemistry of incisional biopsies of the lesions were consistent with a diagnosis of DT. To our knowledge, this is the first reported case of congenital DT.

The Treatment of Syringomas by CO2Laser Using a Multiple-Drilling Method

Syringomas are relatively common benign adnexal tumors, predominantly developing in middle-aged women. They frequently involve the periorbital areas and cause cosmetic problems. Up to now, various treatment modalities such as electrodesiccation, laser ablation, cryosurgery, and some chemical peelings have been tried. All of them, however, have the possibilities of frequent recurrences and postoperative complications such as scarring or pigmentary changes. The objective of this study was to determine the effectiveness of a multiple-drilling method in the treatment of syringomas. We tried a new multiple-drilling method using CO(2) laser for 11 patients having syringomas. Clinical improvement and complications were evaluated by medical records and pre- and postlaser photograph review. All patients had good or excellent cosmetic results. No complications, such as scarring, erythema, and pigmentary changes, were observed. The multiple-drilling method by CO(2) laser might be an alternative to gain good cosmetic results without complications if applied repeatedly. The authors have indicated no significant interest with commercial supporters.

Siringocistoadenoma papilífero de presentación atípica

Syringocystoadenoma papilliferum is benign adnexal tumor derived from the sweat glands that is located in the head and neck in 70-80% of patients, and commonly presents as a papule or a solitary nodule. We report a case of syringocystoadenoma papilliferum with an atypical presentation given its location in the thigh and the peculiar histologic features, unrepresentative of this entity.

Syringocystadenoma papilliferum: report of first case on the pinna

Syringocystadenoma papilliferum is a benign adnexal skin tumour, which, in a third of cases, arises from an organoid nevus on the head and neck. We report on a 17-year-old man with a syringocystadenoma papilliferum on his right pinna of three-years duration. The clinical and histopathological features are described. Following excision and skin grafting, the patient remains asymptomatic four years after surgery. This is the first report of a syringocystadenoma papilliferum on the pinna.

Impaired regulation of NF-κB and increased susceptibility to colitis-associated tumorigenesis in CYLD-deficient mice

Cylindromatosis (CYLD) is a deubiquitinating enzyme that is altered in patients with familial cylindromatosis, a condition characterized by numerous benign adnexal tumors. However, the regulatory function of CYLD remains unsettled. Here we show that the development of B cells, T cells, and myeloid cells was unaffected in CYLD-deficient mice, but that the activation of these cells with mediators of innate and adaptive immunity resulted in enhanced NF-kappaB and JNK activity associated with increased TNF receptor-associated factor 2 (TRAF2) and NF-kappaB essential modulator (NEMO) ubiquitination. CYLD-deficient mice were more susceptible to induced colonic inflammation and showed a dramatic increase in the incidence of tumors compared with controls in a colitis-associated cancer model. These results suggest that CYLD limits inflammation and tumorigenesis by regulating ubiquitination in vivo.

OC156: External validation of mathematical models to distiguish benign and malignant adnexal tumors: a multicenter study by the international ovarian tumor analysis (IOTA) group

complex based on ultrasound and this, with CA125, generates a risk of malignancy index (RMI). Objective: The purpose of this study was to determine whether RCOG guidelines on complex ovarian cysts are applicable to a subgroup of post-menopausal women with PMB. Methods: Clinical and ultrasound data for consecutive women in our PMB clinic from September 2001 until April 2004 were retrieved from a database. All women found to have an ovarian cyst had serum CA125 measurement and RMI calculated. Those with complex cysts or an increased RMI (> 250) were counselled and offered surgery according to RCOG guidelines. Those with simple cysts and a low RMI (<250) were managed conservatively. Pathology reports were obtained for all resected samples. Results: 753 women underwent TVS. 76 (10.1%) were found to have ovarian cysts. 59 had simple cysts, and all of them had normal CA125 levels and RMIs of less than 250. 17 (2.25%) women had complex cysts: 11 had normal CA125 and thus low RMI scores and 6 had elevated CA125 giving an elevated RMI. All women with complex cysts were offered surgery. 6 of the 11 with low RMI elected to have surgery and all had benign disease. The remaining 5 had follow-up CA125 and TVS and all had ultrasound resolution of their cysts between 6 months and 2 years. All 6 women with increased RMI underwent surgery. 5 had primary ovarian malignancy with one complex benign lesion. Conclusion: RMI calculations are a reliable predictor of ovarian malignancy in women with complex ovarian cysts and PMB. RCOG guidelines for the management of complex ovarian lesions in the post-menopausal population are safely applicable to this important sub-group of women.

Logistic Regression Model to Distinguish Between the Benign and Malignant Adnexal Mass Before Surgery: A Multicenter Study by the International Ovarian Tumor Analysis Group

To collect data for the development of a more universally useful logistic regression model to distinguish between a malignant and benign adnexal tumor before surgery. Patients had at least one persistent mass. More than 50 clinical and sonographic end points were defined and recorded for analysis. The outcome measure was the histologic classification of excised tissues as malignant or benign. Data from 1,066 patients recruited from nine European centers were included in the analysis; 800 patients (75%) had benign tumors and 266 (25%) had malignant tumors. The most useful independent prognostic variables for the logistic regression model were as follows: (1) personal history of ovarian cancer, (2) hormonal therapy, (3) age, (4) maximum diameter of lesion, (5) pain, (6) ascites, (7) blood flow within a solid papillary projection, (8) presence of an entirely solid tumor, (9) maximal diameter of solid component, (10) irregular internal cyst walls, (11) acoustic shadows, and (12) a color score of intratumoral blood flow. The model containing all 12 variables (M1) gave an area under the receiver operating characteristic curve of 0.95 for the development data set (n = 754 patients). The corresponding value for the test data set (n = 312 patients) was 0.94; and a probability cutoff value of .10 gave a sensitivity of 93% and a specificity of 76%. Because the model was constructed from multicenter data, it is more likely to be generally applicable. The effectiveness of the model will be tested prospectively at different centers.

OC26.01: First trimester screening for trisomy 21: a statewide evaluation

Objective: To compare the usefulness of two angiogenesis markers: VCAM-1 and CD-105 measurements with Doppler and 3D vascular blood flow indices in women with benign and malignant adnexal masses. Methods: Preoperative transvaginal color Doppler followed by 3D power Doppler sonography (Voluson 730, GE, Austria) was performed in 79 patients. Doppler blood flow indices: PI, RI and PSV were calculated in detected tumor vessels. Tumor volume and 3D vascular blood flow indices: VI, FI and VFI were measured (VOCAL software) in all cases. Serum concentrations of VCAM-1 and CD-105 (RD p = 0.0003), RI (0.62 vs 0.48; p = 0.0013), VI (3.48 vs 1.28; p = 0.00002) and VFI (0.46 vs 1.87; p = 0.001). Mean (±SD) serum VCAM-1 concentrations were not significantly different in malignant (297.2 ± 148 ng/ml) and benign (357.8 ± 227 ng/ml) adnexal tumors (p > 0.05). Also, mean serum CD105 concentrations were not significantly different in malignant (4.15 ± 1.35 ng/ml) vs benign (4.46 ± 1.03 ng/ml) masses (p > 0.05). Significant correlations between sonographic and serum markers were only found for PI and VCAM-1 (p = 0.033) and RI and VCAM-1 (p = 0.0002). Endoglin and VCAM-1 concentrations were not correlated with each other (p = 0.37). Conclusions: Three-dimensional sonoangiography and Doppler assessment of adnexal tumors blood flow and vascularity, but not soluble VCAM-1 or endoglin serum concentrations are effective in differential diagnosis of angiogenesis in detected benign or malignant lesions. Acknowledgement: This study was supported by the Polish Ministry of Science grant # KBN 3P0 5E 115 24.

OC25.04: Malignant and benign adnexal tumor vascularity assessment with three‐dimensional (3D) power Doppler sonography, soluble vascular adhesion molecule‐1 (VCAM‐1) and soluble endoglin (CD‐105) measurements

OC25.04: Malignant and benign adnexal tumor vascularity assessment with three‐dimensional (3D) power Doppler sonography, soluble vascular adhesion molecule‐1 (VCAM‐1) and soluble endoglin (CD‐105) measurements

Intravenous contrast ultrasound in gynecology

Contrast Tuned Imaging (CnTI), is a new contrast dedicated technology, applied on the transvaginal probe utilizing the second generation contrast agent SonoVue®, which consists of sulphur hexafluoride microbubbles. We evaluated the efficacy of this technology as compared with the standard examination in different gynecological pathologies in order to detect and characterize ovarian and uterine lesions. Each patient underwent an unenhanced (baseline) and a contrast enhanced ultrasound evaluation. In patients with uncertain pelvic masses and tumors suspected to represent recurrence of malignancy, the application of CnTI-SonoVue was helpful to exclude malignancy; lesions that did not show any kind of vascularization proved to be benign. In the malignant cases where color Doppler mode could not detect blood flow, CnTI-SonoVue® usually detected vascularization or enhanced the blood vessels already seen with color Doppler. As far as endometrial cancer, endometrial hyperplasia and endometrial polyps are concerned, the CnTI-SonoVue® technology did not show superiority over the B-mode evaluation in defining the margins of the lesion and in assessing the depth of myometrial invasion of endometrial cancer. In conclusion, the qualitative use of CnTI-SonoVue® technology has improved our confidence in excluding pelvic malignancy. Furthermore, the quantitative assessment seems to be a promising tool for a better distinction between malignant and benign adnexal tumors.

Hidroadenoma apocrino pigmentado

Apocrine hidradenoma is a benign adnexal neoplasm. It has no specific site predilection, and usually affects middle-aged people. The same as other tumors of the sweat glands, there is a pigmented variety. We present the case of a 92-year-old male who consulted his physician for a slow-growing asymptomatic lesion in the right groin which had been developing for 2 years. After the histopathological study, the diagnosis was established as pigmented apocrine hidradenoma.

Brooke-Spiegler Syndrome

Brooke-Spiegler syndrome is an autosomal dominantly inherited disease with predisposition to cutaneous adnexal neoplasms, most commonly cylindromas and trichoepitheliomas. We report a patient in whom 11 lesions were removed from the scalp and face for various reasons over a period of 3 years. The histopathological survey revealed a plethora of benign adnexal neoplasms showing apocrine, follicular, and sebaceous differentiation occurring independently and conjointly. The histopathological spectrum in our patient included cylindromas, spiradenomas, trichoepitheliomas, small nodular trichoblastomas, and lymphadenomas. Many lesions had hybrid features of two or more neoplasms. By far the most common composite tumor was spiradenocylindroma. Some spiradenocylindromas demonstrated prominent sebaceous or trichoblastomatous differentiation or both. We suggest the terms "sebaceous spiradenocylindroma" and "trichospiradenocylindroma" for these lesions. The occurrence of sebaceous and trichoblastic differentiation in spiradenocylindromas is a further proof that spiradenoma and cylindroma are not eccrine tumors but neoplasms of the folliculosebaceousapocrine unit.

Expression of P63 in Primary Cutaneous Adnexal Neoplasms and Adenocarcinoma Metastatic to the Skin

p63, a recently identified homologue of p53 gene, has been reported to be essential in the development of epithelia and is mainly expressed by basal and myoepithelial cells. The purpose of this study was to investigate the pattern of p63 expression in cutaneous adnexal neoplasms and to assess its possible value in the differential diagnosis of primary cutaneous neoplasms versus adenocarcinomas metastatic to the skin. Immunohistochemical analysis using p63 monoclonal antibody (clone 4A4) was performed on formalin‐fixed, paraffin‐embedded archival tissue from 20 benign adnexal tumors, 10 malignant adnexal tumors, and 14 adenocarcinomas metastatic to the skin. The expression of p63 was evaluated in epidermal cells, skin appendages and metastatic tumor cells. p63 was consistently expressed in the basal/suprabasal cells of epidermis and cutaneous appendages, including the basal/myoepithelial cells of sweat glands. Thirteen out of 20 (65%) benign adnexal tumors showed strong p63 expression; the remaining 7(35%) cases had over 50% p63‐positive nuclei. All primary cutaneous carcinomas, including adenocarcinomas, expressed p63. In contrast, none of the metastatic adenocarcinomas to the skin was positive for p63 (p &lt; 0.0001). Based on our findings, analysis of p63 expression may be a helpful tool in the differential diagnosis of primary versus metastatic cutaneous adenocarcinomas.

Expression of p63 in primary cutaneous adnexal neoplasms and adenocarcinoma metastatic to the skin

p63, a recently identified homologue of the p53 gene, has been reported to be essential in the development of epithelia and is mainly expressed by basal and myoepithelial cells. The purpose of this study was to investigate the pattern of p63 expression in cutaneous adnexal neoplasms and to assess its possible value in the differential diagnosis of primary cutaneous neoplasms vs adenocarcinomas metastatic to the skin. Immunohistochemical analysis for p63 was performed on formalin-fixed, paraffin-embedded archival tissue from 20 benign adnexal tumors, 10 malignant adnexal tumors and 14 adenocarcinomas metastatic to the skin. The expression of p63 was evaluated in epidermal cells, skin appendages and metastatic tumor cells. p63 was consistently expressed in the basal and suprabasal cells of epidermis and cutaneous appendages, including the basal/myoepithelial cells of sweat glands. Out of 20 benign adnexal tumors, 13 (65%) showed strong (score 3) p63 expression; the remaining seven (35%) cases had score 2. All primary cutaneous carcinomas, including adenocarcinomas, expressed p63. In contrast, none of the metastatic adenocarcinomas to the skin was positive for p63 (P<0.001). Based on our findings, analysis of p63 expression may help in the differential diagnosis of primary vs metastatic cutaneous adenocarcinomas.

Syringocystadenoma Papilliferum: Report of the First Case on the Lower Leg

Syringocystadenoma Papilliferum (SCAP) is a benign adnexal tumor which most frequently arises from an organoid nevus on the head and neck. Although they are rarely found on the trunk and limbs, we treated a case of this disorder on the lower leg. A 26-year-old man had an asymptomatic tumor on his lower leg. Histopathological examination showed it to be a typical SCAP on organoid nevus. This is the first report of SCAP on the lower leg.

Siringomas vulvares

Abstract—Syringomas are benign adnexal tumors that present as multiple flesh-colored papules on the scalp, forehead, neck, anterior thorax, axillae, lower half of the abdomen and limbs. Their appearance in the genital area is infrequent. They develop after puberty, more frequently in women, and are usually asymptomatic. We present the case of a 19-year-old patient with symmetrical and bilateral syringomas in the vulvar region.

The tumor suppressor CYLD interacts with TRIP and regulates negatively nuclear factor kappaB activation by tumor necrosis factor.

Cylindromas are benign adnexal skin tumors caused by germline mutations in the CYLD gene. In most cases the second wild-type allele is lost in tumor tissue, suggesting that CYLD functions as tumor suppressor. CYLD is a protein of 956 amino acids harboring a functional deubiquitinating domain at the COOH-terminal end. To shed more light on the function of CYLD, we have performed a yeast two hybrid screen using an HaCaT cDNA library that identified the RING finger protein TRIP (TRAF-interacting protein) as interactor with full-length CYLD. Mapping of the interacting domains revealed that the central domain of CYLD binds to the COOH-terminal end of TRIP. Far Western analysis and coimmunoprecipitations in mammalian cells confirmed that full-length CYLD binds to the COOH-terminal domain of TRIP. Because TRIP is an inhibitor of nuclear factor (NF)-κB activation by tumor necrosis factor (TNF), the effect of CYLD on NF-κB activation was investigated in HeLa cells. The results established that CYLD down-regulates NF-κB activation by TNF-α. The inhibition by CYLD depends on the presence of the central domain interacting with TRIP and its deubiquitinating activity. These findings indicate that cylindromas arise through constitutive NF-κB activation leading to hyperproliferation and tumor growth.

Benign ocular adnexal tumours of a ocrine eccrine or hair follicle origin

Background: The differential diagnosis of malignant eyelid tumours, particularly basal cell carcinoma (BCC), includes tumours of skin appendages. The incidence of these adnexal tumours has not been well established. This study aimed to determine the incidence and review the main clinicopathological features of benign tumours arising from skin appendages of the eyelid with apocrine, eccrine or hair follicle differentiation.Methods: The histopathological diagnoses of 7751 ophthalmic specimens retrieved from 6967 patients between September 1993 and March 2002 at the Henry C. Witelson Ophthalmic Pathology Laboratory and Registry, McGill University, Montreal, were retrospectively reviewed. Clinical data and histopathological diagnoses were obtained for 228 benign adnexal tumours of apocrine, eccrine or hair follicle origin. New histopathological slides were made from the paraffin-embedded specimens and stained with hematoxylin-eosin and periodic acid-Schiff's reagent.Results: Of the 228 benign adnexal tumours, 182 were diagnosed as apocrine or eccrine hydrocystoma (79.80, 12 pilomatrixoma (5.3%), 12 syringoma (5.3%), 11 trichilemmoma (4.8%), 5 syringocystadenoma papilliferum (2.2%), 3 trichoepithelioma (1.3%) and 3 trichofolliculoma (1.3%). Discrepancies between clinical and histopathological diagnoses were noted in 22 cases (9.6%).Interpretation: Benign tumours originating from skin appendages of the eyelid are rare and frequently have apocrine or eccrine differentiation. These tumours, particularly those originating from the hair follicle, should be considered in the differential diagnosis of BCC of the eyelid.