Epidermal Nevi and Related Syndromes - Part 2: Nevi Derived from Adnexal Structures. / Nevus epidérmicos y síndromes relacionados. Parte 2: Nevus derivados de estructuras anexiales.

Epidermal nevi are hamartomatous lesions derived from the epidermis and/or adnexal structures of the skin; they have traditionally been classified according to their morphology. New variants have been described in recent years and advances in genetics have contributed to better characterization of these lesions and an improved understanding of their relationship with certain extracutaneous manifestations. In the second part of this review article, we will look at nevi derived from the adnexal structures of the skin and associated syndromes.

Sistemas de clasificación y predicción de riesgo de malignidad de las lesiones anexiales / Classification systems and prediction of risk of malignancy of the adnexal masses

El diagnóstico de una masa anexial es un problema frecuente en la consulta ginecológica. El principal objetivo de la evaluación de una masa anexial es el diagnóstico o exclusión de malignidad, dado que el cáncer de ovario es la neoplasia ginecológica más letal y que una cirugía inicial adecuada es uno de sus principales factores pronósticos. La evaluación ecográfica continúa siendo el mejor método para clasificar las masas anexiales. Si existe la sospecha de malignidad de la lesión, la paciente debe ser remitida a un centro especializado en ginecología oncológica. Sin embargo, si la sospecha es de benignidad, se puede optar por una actitud expectante o por un tratamiento quirúrgico conservador. El objetivo de este artículo es revisar los sistemas de clasificación de las lesiones anexiales más importantes y más ampliamente empleados, analizando su metodología y los resultados de su aplicación en los principales estudios de validación publicados hasta el momento

The diagnosis of an adnexal mass is a common problem in gynaecological consultation. The main objective of an adnexal mass evaluation is the diagnosis or exclusion of malignancy. This is the case because ovarian cancer is the most lethal gynaecological neoplasia and appropriate initial surgery is one of the main prognostic factors. Ultrasound scans continue to be the best method of classifying an adnexal mass. If there is any suspicion of a malignant tumour, the patient must be referred to a specialist gynaecological oncology centre. However, if there is any suspicion of a benign tumour, watchful waiting or minimally invasive surgery may be indicated. The objective of this article is to carry out a review of the most important and widely used classification systems of adnexal masses, analysing their methodology and the results of their application in the main validation studies published to date

Sox10 and DOG1 Expression in Primary Adnexal Tumors of the Skin.

Primary skin adnexal tumors can be challenging to classify and must be discerned from cutaneous adenocarcinoma metastases from various sites. We evaluated expression of Sox10 and DOG1 in normal cutaneous adnexa and in 194 primary skin adnexal tumors, and compared their performance in discriminating primary skin adnexal tumors from cutaneous metastatic adenocarcinomas with that of p40 and p63. In normal skin adnexa, we noted Sox10 expression in both the secretory and myoepithelial cells in eccrine glands, but only in myoepithelial cells in apocrine glands. DOG1 demonstrated canalicular expression in eccrine glands, and weak expression in myoepithelial cells of apocrine glands, germinative cells of sebaceous glands, and outer root sheath of follicular infundibulum. Sox10 was expressed in 100% of cylindromas and spiradenomas, and in variable frequency in other benign and malignant tumors of sweat glands. DOG1 was positive in most cylindromas (87.5%), in only 10.5% of spiradenomas, and was variably expressed in other benign and malignant tumors of sweat glands. All syringomas (n = 20) were negative for Sox10 and DOG1. One out of the 33 follicular neoplasms was positive for Sox10 and DOG1 (3%). All sebaceous neoplasms were negative for Sox10, and 28.1% of them were positive for DOG1. Sox10 was specific (91.9%) but not sensitive (28.4%) for primary skin origin, and was far less accurate (38.5%) than p63 or p40 (95.5% accuracy). Combining Sox10 with p63 or p40 showed only very minimal gain in accuracy (96%). DOG1 expression in tumors showed low sensitivity and specificity for skin adnexal origin.

Challenges in the diagnosis of cutaneous adnexal tumours.

The diagnosis of cutaneous adnexal neoplasms, a heterogeneous group of entities, is often perceived by practising pathologists as challenging. A systematic approach to diagnosis is necessary for classification of these lesions, which establishes the tumour differentiation (follicular, sebaceous, sweat gland or apocrine) and evaluates histological features differentiating between benign and malignant entities. Consideration of clinical history is a necessary adjunct in evaluation of the adnexal neoplasm, as characteristic anatomical sites are described for many adnexal lesions. In some instances, immunohistochemical studies may also be employed to aid the diagnosis. The differential diagnosis between primary cutaneous adnexal neoplasms and cutaneous metastases from visceral tumours may also be difficult. Clinical, radiological, histological and immunohistochemical characteristics will be further discussed, considering that the correct diagnosis has a significant impact on the patient's management and prognosis.

Resultados de la clasificación Gynecologic Imaging Reporting and Data System para la catalogación de masas anexiales / Results of the Gynecologic Imaging Reporting and Data System classification in adnexal masses

Objetivo. Determinar el valor de la clasificación Gynecologic Imaging Reporting and Data System (GI-RADS) para discriminar las masas anexiales malignas de las benignas. Material y métodos. Estudio observacional retrospectivo de 87 pacientes operadas en nuestro hospital con un total de 98 masas anexiales. Se clasificaron mediante el sistema GI-RADS y se compararon con la histología definitiva. Resultados. GI-RADS 3: 75 casos. En todos los casos GI-RADS 3, el diagnóstico definitivo fue de benignidad. GI-RADS 4: 11 casos. En el 27,27% de los casos el diagnóstico definitivo fue de malignidad (un carcinoma y 2 tumores borderline), y en el 72,72% de los casos, de benignidad. GI-RADS 5: 12 casos. En el 91,66% de los casos el diagnóstico histológico informó de malignidad (10 carcinomas y un tumor borderline). Discusión. Nuestros resultados refuerzan la fiabilidad de la clasificación para catalogar correctamente las tumoraciones malignas en los grupos GI-RADS 4 y 5 (AU)

Objective. To assess the clinical usefulness of the Gynecologic Imaging Reporting and Data System (GI-RADS) classification to distinguish between benign and malignant adnexal masses. Material and methods. A retrospective study was conducted in 98 adnexal masses in 87 patients who underwent surgery in our hospital. The masses were classified according to GI-RADS and the results were compared with their definitive histologic diagnosis. Results. Seventy-five masses were classified as GI-RADS 3. All GI-RADS 3 masses had a definitive histologic diagnosis of benignity. Eleven masses were GI-RADS 4, of which 27.27% had a definitive histologic diagnosis of malignancy (one carcinoma and 2 borderline tumors) and 72.72% were diagnosed as benign. Twelve masses were GI-RADS 5, of which 91.66% had a definitive histologic diagnosis of malignancy (10 carcinomas and one borderline tumor). Discussion. Our results support the reliability of the GI-RADS classification to correctly identify malignant tumors in GI-RADS 4 and 5 (AU)

Hybrid eccrine gland and hair follicle hamartoma: a new entity of adnexal nevus.

Eccrine nevus shows increase in number or size of eccrine glands, whereas hair follicle nevus is composed of densely packed normal vellus hairs, and eccrine-pilar angiomatous nevus reveals increase of eccrine, pilar, and angiomatous structures. No case with increased number of both eccrine glands and hair follicles only in the dermis has been previously reported. A 10-month-old girl presented with cutaneous hamartoma with overlying skin hyperpigmentation on her left hypochondrium since 3 months of age, in whom the lesion was completely excised. Histopathology demonstrated evidently increased number of both eccrine glands and hair follicles in the dermis with reactive hyperplasia of collagen fibers. No recurrence occurred after the tumor was completely excised. A term "hybrid eccrine gland and hair follicle hamartoma" is proposed for this unique lesion.

[Cutaneous adnexal and salivary gland tumours. Similarities and differences]. / Hautadnex- vs. Speicheldrüsentumoren. Analoges und Divergentes.

Despite major discrepancies in basic microscopic anatomy, remarkable similarities are manifest within the wide spectrum of cutaneous adnexal and salivary gland tumors. In this study salivary gland and adnexal tumors were identified and investigated with respect to similarities in histology, terminology and pathogenesis. Histological similarities of certain types of salivary gland tumors relate to eccrine, apocrine and rarely sebaceous (but not trichofollicular) types of adnexal tumors. The most striking similarity was found with salivary gland pleomorphic adenoma and cutaneous mixed tumor. Multistep carcinogenesis starting with intraductal carcinoma, identified in carcinoma ex pleomorphic adenoma is identical to that found in cutaneous carcinoma ex spiradenoma. Further histological and terminological similarities are shown for mucinous and mucoepidermoid carcinoma, for lymphadenoma and lymphoepithelial carcinoma, for sebaceous adenoma and carcinoma, for adenoid-cystic carcinoma, as well as for salivary gland basal cell adenoma versus cutaneous spiradenoma. Manifest diagnostic problems related to histologically similar salivary gland and adnexal tumors are rare and are topographically limited to the parotid and oral areas.

[Skin adnexal tumors with follicular differentiation]. / Hautadnextumoren mit Haarfollikeldifferenzierung.

Skin adnexal tumors with predominantly follicular differentiation represent a clinicopathological heterogeneous group of neoplasms and are classified according to the cytologically achieved differentiation of the follicular compartment. Given the complex structure of non-neoplastic hair follicles it is not surprising to find varying differentiations in neoplasms and there are overlapping clinicopathological features between the established entities. The use of immunohistochemical staining has only a limited value in the diagnosis of follicular neoplasms.

Adnexal skin tumors in Zaria, Nigeria.

BACKGROUND: Adnexal skin tumors share many features in common and differentiate along one line. Their detailed morphological classification is difficult because of the variety of tissue elements and patterns seen. They may be clinically confused with other cutaneous tumors. The aim of this report is to review and classify all adnexal tumors seen in a pathology department over a 16-year period. METHOD: A 16-year retrospective analysis of all adnexal skin tumors seen in a large University Teaching Hospital in Nigeria from January 1991- December 2006. All tissue specimens were fixed in 10% formalin, processed in paraffin wax and stained with Haematoxylin and Eosin. Histology slides were retrieved, studied and lesions characterized. RESULTS: Fifty-two adnexal tumors were seen, accounting for 0.9% of all cutaneous tumors seen within the same period. The median age was 33 years (range: 4 days-70 years). Clinical presentations varied from discreet swellings and nodules to ulcerated masses. Five patients presented with recurrent lesions. Only two cases had a clinical diagnosis of adnexal tumor. Twenty-four (46%) of the lesions were distributed in the head and neck region. Duration of symptoms was 2 months to 15 years (median: 12 months). Tumours of the sweat gland were the commonest--41 (78.8%); they comprised predominantly eccrine acrospiroma (17), characterized histologically by solid nests of round to polygonal cells with clear to eosinophilic cytoplasm, forming tubules in areas. Tumours of sebaceous gland were 7 (13.5%); they comprised mainly Nevus sebaceous of Jadassohn (6), composed of immature sebaceous glands and pilar structures microscopically and a solitary sebaceous adenoma. Tumours of hair follicle were 4 (7.7%) and included trichoepithelioma, characterized microscopically by multiple horn cysts and epithelial tracts connecting abortive pilar structures and a trichofolliculoma. Forty-six lesions (88.5%) were benign and six (11.5%) malignant. CONCLUSION: Adnexal skin tumors have distinct histological patterns which differentiates them from other cutaneous tumors. They are commonly distributed in the head, neck and trunk. The commonest variants are those of eccrine sweat gland origin. Malignant adnexal tumors are uncommon in our setting.

[Skin adnexal tumors]. / Tumori degli annessi cutanei.

Adnexal skin tumors are rare neoplasms that develop from hair follicles, sebaceous glands and sweat glands. In the majority of cases these tumors are benign, although metastases have been reported in rare occasion. The diagnosis in always histologic and often it is sufficient to report the lesion simply as benign or malignant. Radical surgery is the treatment of choice. When the tumor is large, the Mohs technique can be used. Local recurrence is frequent in case of incomplete surgical removal. Etastatic disease, although rare, has a poor prognosis. Chemotherapy and radiotherapy experience is very limited. Overall, combination chemotherapy seems to be superior to single agent treatment.

[Diagnostic procedures in skin adnexal tumours]. / Diagnostika adnexálnych tumorov koze.

From histogenetic, morphologic and immunohistochemical point of view the authors try to make possible algorithms that can be employed in a routine diagnosis of adnexal skin tumours. They stress the importance of knowledge of clinical data necessary for orientation classification of tumour skin lesions after biopsies. The authors translated their obtained data into survey tables to be used as guidelines in a routine bioptic practice.

Cutaneous carcinosarcoma: adnexal vs. epidermal types define high- and low-risk tumors. Results of a meta-analysis.

OBJECTIVE: We report four cases of cutaneous carcinosarcoma (CS) and perform a meta-analysis of the cutaneous CS literature. RESULTS: CS occurred in elderly patients (mean of 80 years) on sun-damaged skin, and were keratotic papules of short duration. They did not recur after excision. CS exhibited basal cell carcinoma mixed with atypical fibroxanthoma cell populations. Immunophenotyping revealed vimentin+/keratin- spindle cells and vimentin-/keratin+ epithelial cells. Three cases exhibited p53 protein expression of both carcinomatous and sarcomatous components. Literature review identified 38 cases of cutaneous CS that could be broadly classified into two distinct groups. Epidermal-derived (basal or squamous cell carcinoma epithelial component) CS arose on the sun-damaged skin of the head and neck of elderly males (mean age 72 years) and had a 70% 5-year disease-free survival. In contrast, adnexal CS (spiradenocarcinoma, porocarcinoma, proliferating tricholemmal cystic carcinoma, or matrical carcinoma) occurred in younger patients (mean age 58 years), showed recent growth in a long-standing nodule and had a 25% 5-year disease-free survival. Age less than 65 years, recent growth, long-standing skin tumor, and tumor size greater than 2 cm significantly correlated with poor outcome. CONCLUSIONS: Cutaneous CS is an aggressive skin cancer with high risk for advanced disease. Significant risk factors exist whose identification will allow for better management of CS patients.

Siringocistoadenoma papilífero de la vulva / Siringocystadenoma papilliferum of the vulva

Se comunica el caso de una paciente de 24 años, con un siringocistoadenoma papilífero de la vulva. Se presenta una revisión de la literatura de esta patología infrecuente