Spiradenomas are benign cutaneous adnexal neoplasms with sweat gland differentiation that can manifest a broad spectrum of histomorphologic appearances. While they show a characteristic histopathologic phenotype and clinical management involves surgical excision with a low risk of recurrence, there have been unusual histopathologic variants of spiradenoma reported, including cases with adenoid cystic carcinoma (ACC)-like changes. Primary cutaneous ACC is a low-grade malignancy presenting as a subcutaneous mass with the potential for local invasion, perineural invasion, and high rates of local recurrence after excision. The diagnosis of spiradenomas with ACC-like features can be challenging, especially when only the ACC-like component is present for evaluation. A retrospective analysis of 21 cases of spiradenoma with ACC-like changes were obtained from large academic institutions, was performed, and summarized below. All cases showed background of conventional spiradenoma, and the ACC-like areas represented a component in all lesions. The percentage of ACC-like component ranged from 5% to 40% in all cases. The ACC-like component was multifocal and without pleomorphism, perineural and/or vascular invasion, necrosis, or increased mitotic activity. MYB translocation and protein expression was studied in 16 cases by fluorescence in situ hybridization, polymerase chain reaction, and immunohistochemistry. All studied cases were negative for MYB / NFIB , MYB L1, and MYB F by fluorescence in situ hybridization and polymerase chain reaction and 3 cases were positive for MYB expression by immunohistochemistry. Our study expands on spiradenomas with ACC-like features that ought to be considered in the differential diagnosis of cutaneous neoplasms such as primary cutaneous ACC. Our results indicate that a thorough histopathologic inspection and strict application of well-defined histologic criteria are necessary to support the diagnosis of this unusual histopathologic variant. These tumors can be difficult to diagnose, and awareness of their histomorphologic spectrum will facilitate definitive diagnosis and avoid misdiagnosis with other conditions.

• MeSH
• adenoidně cystický karcinom * patologie   MeSH
• akrospirom * chirurgie   MeSH
• hybridizace in situ fluorescenční MeSH
• lidé MeSH
• nádory kůže * patologie   MeSH
• nádory potních žláz * chirurgie   patologie   MeSH
• retrospektivní studie MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH

To determine whether a subset of primary extramammary Paget disease (EMPD) may originate in anogenital mammary-like glands (AGMLG), the authors studied 181 specimens of EMPD, detailing alterations in AGMLG. The latter were identified in 33 specimens from 31 patients. All patients were women, ranging in age from 38 to 93 years (median, 65 y). In all cases, lesions involved the vulva and in 1 patient the perianal skin was affected. Histopathologically, AGMLG manifested changes identical to columnar cell change (CCC) (87.1%), usual ductal hyperplasia (22.6%), columnar cell hyperplasia (CCH) (9.7%), oxyphilic (apocrine) metaplasia (6.5%), and atypical duct hyperplasia (3.2%). Four cases (12.9%), in addition to intraepidermal carcinoma, harbored invasive carcinoma. In all 4 of these, AGMLG displayed a range of alterations including ductal carcinoma in situ, CCC, and CCH. Three further cases (9.7%) showed ductal carcinoma in situ without any definite invasive carcinoma. Colonization of AGMLG by neoplastic Paget cells was noted in 6 cases. As CCC and CCH may be encountered in normal AGMLG, these alterations are unlikely to play a significant role in the pathogenesis of the disease. However, by analogy with mammary Paget disease, rare cases of primary EMPD may originate in AGMLG with a subsequent upward migration of the neoplastic cells into the epidermis and possible later breach through the basal membrane. Usual ductal hyperplasia and atypical duct hyperplasia can then be regarded as earlier precursor lesions, linking both ends of the spectrum.

• MeSH
• anální kanál patologie   MeSH
• dospělí MeSH
• extramamární Pagetova nemoc etiologie   patologie   MeSH
• lidé středního věku MeSH
• lidé MeSH
• nádory anu etiologie   patologie   MeSH
• nádory vulvy etiologie   patologie   MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• vulva patologie   MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH

Extramammary Paget disease (EMPD) is a rare neoplasm usually presenting in the anogenital area, most commonly in the vulva. Adnexal involvement in primary EMPD is a very common feature and serves as a pathway for carcinoma to spread into deeper tissue. The depth of carcinomatous spread along the appendages and the patterns of adnexal involvement were studied in 178 lesions from 146 patients with primary EMPD. Hair follicles and eccrine ducts were the adnexa most commonly affected by carcinoma cells. The maximal depth of involvement was 3.6 mm in this series. When planning topical therapy or developing novel local treatment modalities for EMPD, this potential for significant deep spread along adnexa should be taken into account.

• MeSH
• biopsie MeSH
• dospělí MeSH
• ekrinní žlázy patologie   MeSH
• extramamární Pagetova nemoc patologie   terapie   MeSH
• lidé středního věku MeSH
• lidé MeSH
• nádory anu patologie   terapie   MeSH
• nádory kožních adnex patologie   terapie   MeSH
• nádory potních žláz patologie   terapie   MeSH
• nádory vulvy patologie   terapie   MeSH
• prognóza MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• vlasový folikul patologie   MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH
• Geografické názvy
• Evropa MeSH
• Západní Austrálie MeSH

Primary extramammary Paget disease (EMPD) is a form of intraepithelial adenocarcinoma. Different morphological changes may accompany EMPD, including the presence of syringoma-like structures. The authors report 10 cases of EMPD, all of which manifested syringoma-like structures within the dermis both in areas involved by the carcinoma and beyond, including at the margins of the excisions. All patients were women, whose ages ranged from 49 to 93 years (median 75 years). The possible pathogenesis of the syringoma-like lesions is discussed. Awareness of these structures in vulvectomy specimens for EMPD is important to prevent misinterpretation of the syringoma-like lesions as an invasive component of the EMPD.

• MeSH
• dospělí MeSH
• extramamární Pagetova nemoc patologie   MeSH
• lidé středního věku MeSH
• lidé MeSH
• nádory kůže patologie   MeSH
• nádory vulvy patologie   MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• syringom patologie   MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH

The authors describe 10 cases of sebaceoma that manifested prominent infundibulocystic structures in all cases and, additionally, conspicuous squamous metaplasia in 6 neoplasms. All tumors occurred on the scalp or the face (2 cases lacked clinical information) and presented as a solitary lesion, measuring from 5 to 20 mm. The patients' age ranged from 22 to 89 years. The main component of all tumors was small, uniform basaloid cells (immature sebocytes) intermixed with mature sebocytes clearly arranged in nodules, classifying the lesions as a sebaceoma. In all neoplasms, the tumor cells showed organoid growth patterns of sebaceoma, including rippled, sinusoidal/labyrinthine, and carcinoid-like, occurring alone or in combination. Additionally, numerous infundibulocystic structures were readily noticed and were either distributed multifocally or unilocular within the tumors. In some cases, they were segregated from the main tumor bulk. The authors posit that these structures, which are different from both sebaceous ductal differentiation and squamous metaplasia, represent an authentic follicular differentiation. The infundibulocystic features (combined with squamous metaplasia), when prominent and in a limited biopsy specimen, may cause a confusion with trichoadenoma or even microcystic adnexal carcinoma.

• MeSH
• dospělí MeSH
• epitelové buňky patologie   MeSH
• lidé středního věku MeSH
• lidé MeSH
• metaplazie patologie   MeSH
• mladý dospělý MeSH
• nádory hlavy a krku patologie   MeSH
• nádory mazových žláz patologie   MeSH
• senioři MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladý dospělý MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH

Hidradenoma papilliferum (HP), also known as papillary hidradenoma, is the most common benign lesion of the female anogenital area derived from anogenital mammary-like glands (AGMLG). HP can be viewed conceptually as the cutaneous counterpart of mammary intraductal papilloma. The authors have studied 264 cases of HP, detailing various changes in the tumor and adjacent AGMLG, with emphasis on mammary-type alterations. In many HP, the authors noticed changes typical for benign breast lesions, such as sclerosing adenosis-like changes, usual, and atypical ductal hyperplasia. Almost in a third of cases, remnants of AGMLG adjacent to the lesion were evident, manifesting columnar changes reminiscent of those seen in breast lesions. This study shows that the histopathological changes in HP run a broad spectrum comparable with that in the mammary counterpart and benign breast disease.

• MeSH
• akrospirom patologie   MeSH
• anální kanál patologie   MeSH
• biopsie MeSH
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladý dospělý MeSH
• mléčné žlázy lidské patologie   MeSH
• nádory análních žláz patologie   MeSH
• nádory potních žláz patologie   MeSH
• nádory vulvy patologie   MeSH
• prediktivní hodnota testů MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladý dospělý MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH

Desmoplastic melanoma (DM) is histologically characterized by a proliferation of spindle melanocytes dispersed in a collagenous stroma that can be mistaken for a variety of neoplasms. The purpose of this study was to analyze 40 cases of DM with a comprehensive panel of immunohistochemical markers (KBA.62, p16, Ezrin, WT-1, MITF-1, SOX-10, CD117, SOX-2, nestin, PNL2, p75, MART-1, gp100 and S100p) to obtain a more complete understanding of the potential use of these antibodies in the diagnosis of DM. We found that all cases of DM expressed p16, WT-1, SOX-10, nestin and S100p and 95% of cases expressed p75. There was variable expression with Ezrin, SOX-2, KBA.62, MART-1 and HMB-45. Most DMs did not express MITF-1, PNL2 and CD117. Conditions that may enter in the histologic differential diagnosis of DM, including dermal scars, fibromatosis and dermatofibromas were also studied. Nearly all control cases also stained positive for p16 but were negative for WT1, SOX10, nestin, p75 and S-100p, as well as for most of the other markers tested. We conclude that a panel of S-100p, WT1, SOX10, p75 and nestin may constitute the optimal panel with the most sensitive and specific combination of immunostain available for the diagnosis of DM.

• Klíčová slova
• desmoplastic melanoma, immunohistochemistry, melanoma, spindle cell melanoma,
• MeSH
• barvení a značení metody   MeSH
• dospělí MeSH
• imunohistochemie MeSH
• lidé středního věku MeSH
• lidé MeSH
• melanom metabolismus   patologie   MeSH
• mladiství MeSH
• nádorové proteiny metabolismus   MeSH
• nádory kůže metabolismus   patologie   MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladiství MeSH
• mužské pohlaví MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• Názvy látek
• nádorové proteiny MeSH

BACKGROUND: Localized lymphedema is a nonneoplastic condition associated with obesity and predominantly involving the legs. This condition has distinctive clinical and histologic features and only rarely has been mentioned in the dermatologic literature. OBJECTIVE: We sought to evaluate the clinical and histopathologic features. METHODS: The clinicopathologic features in patients with localized lymphedema of the genital region were studied. RESULTS: We identified 18 patients with localized lymphedema clinically presenting as large polypoid or verrucous lesions. The patients were 5 men and 13 women with a mean age of 46.5 years. Twelve patients were obese at diagnosis. Thirteen patients presented with tumors involving the vulva, 4 patients with tumors in the penis and scrotum, and 1 patient with scrotal and pubic lesions. Histologically, all cases showed marked dermal edema along with dilated lymphatic spaces, fibroplasia, and verrucous epidermal changes (papillomatous and hyperplastic epidermis). LIMITATIONS: Only 18 cases were included in our study. CONCLUSIONS: This condition is an uncommon and recently described entity that could potentially be clinically and histologically misdiagnosed as a neoplasm; thus, it needs to be included in the differential diagnosis of polypoid and verrucous skin tumors with extensive dermal edema and fibroplasia.

• Klíčová slova
• localized lymphedema, massive localized lymphedema, penile lymphedema, scrotal lymphedema, vulvar lymphedema,
• MeSH
• dospělí MeSH
• edém komplikace   MeSH
• kožní nemoci komplikace   patologie   MeSH
• lidé středního věku MeSH
• lidé MeSH
• lymfedém komplikace   patologie   MeSH
• nádory penisu komplikace   MeSH
• nádory vulvy komplikace   MeSH
• obezita komplikace   MeSH
• retrospektivní studie MeSH
• senioři MeSH
• skrotum MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH

Low-grade B-cell lymphomas of the skin, especially, primary cutaneous follicle center cell lymphoma has several distinctive features when compared with nodal/systemic follicular lymphomas, as they are frequently negative for bcl-2 and CD10, and only fewer than 25% of the cases show a bcl-2 rearrangement. The risk of transformation of a cutaneous low-grade B-cell lymphoma, such as primary cutaneous follicle center cell lymphoma, to primary cutaneous diffuse large B-cell lymphomas (PCDLBCL) has not been clearly delineated in the literature. Transformation of systemic/nodal follicular lymphoma into aggressive DLBCL is associated with rapid disease progression, refractoriness to treatment, and poor prognosis. The authors studied 82 cases of primary cutaneous DLBCL using antibodies for follicular dendritic cells (FDCs), CD21, and CD35 to detect networks of FDCs that could possibly indicate transformation of preexisting low-grade B-cell lymphoma to PCDLBCL. All cases were classified as PCDLBCL using strict histologic and immunophenotypic criteria. Fifty-three cases were classified as primary cutaneous DLBCL of "leg type," and 29 cases were classified as primary cutaneous DLBCL, "NOS" category. Immunohistochemical studies were performed in all 82 cases; in 15 cases, a CD21/CD35+ network of FDCs was noted within the tumor, indicating the presence of remnants of residual germinal centers, suggesting the possibility of a transformed low-grade B-cell lymphoma. In summary, the authors' findings seem to indicate that some cases of primary cutaneous DLBCL may result from transformation of a low-grade B-cell lymphoma. Further studies are necessary to evaluate the significance of their findings by using ancillary techniques including genetic analysis.

• MeSH
• B-buněčný lymfom patologie   MeSH
• difúzní velkobuněčný B-lymfom patologie   MeSH
• dospělí MeSH
• imunofenotypizace MeSH
• imunohistochemie MeSH
• lidé středního věku MeSH
• lidé MeSH
• nádorová transformace buněk patologie   MeSH
• nádorové biomarkery analýza   MeSH
• nádory kůže patologie   MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• stupeň nádoru MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• Názvy látek
• nádorové biomarkery MeSH

Spitz nevi are acquired melanocytic lesions with a wide histomorphological spectrum; reliable distinction from spitzoid melanoma is often difficult. Misdiagnoses of benign spitzoid tumors as spitzoid melanomas and vice versa are attributable to a frequently disturbing morphology and inconsistent or poorly defined histological criteria for diagnosis. Many recognized histological variants of Spitz nevi have been described, including the intradermal Spitz. Histopathologic descriptions of intradermal Spitz nevi have been done in the past; however, large studies addressing their histological spectrum have been lacking. We have retrospectively assessed the morphological features in 74 cases of intradermal Spitz nevi, excluding tumors clearly defined as atypical Spitz nevi and Spitzoid melanomas, to further delineate their histological spectrum. The patients' ages ranged from 5 to 81 years (median: 27). Anatomic location included: the upper extremities (27 cases), followed by head and neck (22 cases), lower extremities (9 cases), back (8 cases), buttock (5 cases), chest (1 case), and vulva (1 case). In 1 case, the anatomic location of the lesion was not available. Different histological variants were observed including hyalinized, polypoid, desmoplastic, angiomatoid, and halo Spitz. Morphological features evaluated included symmetry (100%), cell type (epithelioid 42%, spindle 16%, mixed 42%), maturation (85%), pigmentation (26%), chronic inflammation (24%), and mitotic activity (38%). Mild atypia was seen in 36 cases (49%), moderate atypia was seen in 28 cases (38%), and severe atypia was seen in 10 cases (14%). Intradermal Spitz nevus is a distinctive type of Spitz nevus that sometimes can be difficult to define given the unusual features that these lesions can show; thus, strict application of well-defined histological criteria and awareness of their morphological spectrum will facilitate definitive diagnosis.

• MeSH
• diferenciální diagnóza MeSH
• dítě MeSH
• dolní končetina MeSH
• dospělí MeSH
• epiteloidní a vřetenobuněčný névus diagnóza   patologie   MeSH
• hlava MeSH
• horní končetina MeSH
• krk MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• nádory kůže diagnóza   patologie   MeSH
• předškolní dítě MeSH
• retrospektivní studie MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• záda MeSH
• Check Tag
• dítě MeSH
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• mužské pohlaví MeSH
• předškolní dítě MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH