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.
Cuticular poroma is a rare variant of poroma composed of exclusively or predominantly cuticular cells, namely of large cells with ample eosinophilic cytoplasm. We report 7 cases of this rare tumor identified among 426 neoplasms diagnosed as poroma or porocarcinoma. The patients were 4 males and 3 females, ranging in age from 18 to 88 years. All presented with a solitary asymptomatic nodule. The location included knee (2 cases), shoulder, thigh, shin, lower arm, and neck (each 1). All lesions were surgically removed. No evidence of disease was observed in 5 patients with available follow-up (range 12-124 months).Microscopically, all neoplasms were composed of variably sized, focally closed packed, or interconnecting nodules constituted mostly of cuticular cells. Small poroid cells were a focal feature in 5 tumors, whereas in the remaining 2 cases, poroid cells with conspicuous but still in minority. Five neoplasms were somewhat asymmetric, with irregular outlines. Ductal differentiation and intracytoplasmic vacuoles were seen in 6 tumors. Other features variably encountered were conspicuous intranuclear pseudoinclusions, cystic change, occasional multinucleated cells, increased mitoses, and stromal desmoplasia. Four of the 5 tumors analyzed with next-generation sequencing yielded YAP1::NUTM1 fusions. In addition, various mutations, mostly of unknown significance were identified in one neoplasm.
- MeSH
- dospělí MeSH
- ekrinní porokarcinom * genetika MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladiství MeSH
- mladý dospělý MeSH
- nádory potních žláz * genetika patologie MeSH
- porom * genetika patologie MeSH
- senioři nad 80 let MeSH
- senioři MeSH
- transkripční faktory genetika MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladiství MeSH
- mladý dospělý MeSH
- mužské pohlaví MeSH
- senioři nad 80 let MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
Autoři popisují případ 52letého muže s četnými modročernými makulopapulami na tvářích přítomnými 10 let bez progrese a bez příznaků. Histopatologické vyšetření prokázalo apokrinní hidrocystom. Článek poskytuje přehled současných poznatků o tomto onemocnění.
The authors describe the case of a 52-year-old man with numerous blue-black maculopapules on the cheeks present for 10 years without progression or symptoms. Histopathological examination revealed an apocrine hidrocystoma. The article provides an overview of cur- rent knowledge of this disease .
- MeSH
- apokrinní žlázy patologie MeSH
- ekrinní žlázy patologie MeSH
- hidrocystom * diagnóza klasifikace patologie MeSH
- lidé středního věku MeSH
- lidé MeSH
- nádory kůže diagnóza klasifikace MeSH
- nádory obličeje diagnóza klasifikace patologie MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- Publikační typ
- kazuistiky MeSH
BACKGROUND: Although syringoma is a common benign tumour of the sudoriferous gland, there is also an extremely rare malignant form known as syringoid eccrine carcinoma (SEC). SEC usually exhibits slow growth with deep invasion and a frequent tendency to relapse. The treatment of choice is radical wide resection, which poses a difficult reconstructive problem, especially when the tumour is located in the centre of the face. CASE PRESENTATION: In this case, a 70-year-old man was diagnosed with an SEC at the same location as a benign syringoma of the upper lip and nasal base that had undergone primary excision 7 years prior. Primary radical resection was performed with immediate Abbé flap reconstruction. Nevertheless, histology revealed positive margins, and 3 additional re-excisions were needed to achieve clear margins. Four months after the initial resection, the patient had undergone an innovative reconstruction technique including not only the Abbé flap but also a turbinate flap harvested with functional endonasal surgery and a three-stage forehead flap. CONCLUSION: To the best of our knowledge, this is the first case report of a suspect malignant transformation of a benign syringoma after 7 years. In addition, from oncoplastic and reconstructive points of view, the bilateral use of the turbinate flap for reconstructing the intranasal lining of the alar base is unusual, and the use of functional endonasal surgery in nasal reconstruction for reducing the risk of damaging the vascular supply of the flap is innovative.
- MeSH
- čelo chirurgie MeSH
- karcinom * chirurgie MeSH
- lidé MeSH
- lokální recidiva nádoru chirurgie MeSH
- nádory kožních adnex MeSH
- nádory kůže MeSH
- nádory potních žláz * chirurgie MeSH
- nosní skořepy chirurgie MeSH
- ret chirurgie MeSH
- senioři MeSH
- syringom * chirurgie MeSH
- zákroky plastické chirurgie * metody MeSH
- Check Tag
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
ABSTRACT: Trichoblastoma (TB) is a benign biphasic follicular neoplasm with differentiation toward the germinative cells and a specific follicular mesenchyme. We subtyped 349 sporadic TB according to a classification proposed by Ackerman. Two hundred forty-six (246/349, 70.5%) neoplasms were comprised of mixed subtypes. TB composed exclusively of a single pattern was less common (103/349, 29.5%). The most common pure subtype was cribriform TB followed by small nodular TB. Twelve cases (12/349, 3.4%) had unique features and are reported herein as novel histopathologic subtypes of the neoplasm.
- MeSH
- dítě MeSH
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladiství MeSH
- mladý dospělý MeSH
- nádory kožních adnex patologie MeSH
- nádory kůže patologie MeSH
- nemoci vlasů patologie MeSH
- senioři nad 80 let MeSH
- senioři MeSH
- vlasový folikul patologie MeSH
- Check Tag
- dítě MeSH
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladiství MeSH
- mladý dospělý MeSH
- mužské pohlaví MeSH
- senioři nad 80 let MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
Syringocystadenoma papilliferum (SCAP) je veľmi zriedkavý benígny nádor kožných adnexodvodený z apokrinných potných žliaz. Typickým miestom vzniku je hlava a krk, ktoré za-hrňuje až 90 % všetkých prípadov. Zaujímavou klinickou črtou SCAP je jeho častá koinci-dencia so sebaceóznym névom, ktorú možno pozorovať asi v jednej tretine prípadov. Autoropisuje prípad 56-ročnej ženy s diagnostikovanou mierne prominujúcou tumoróznou lézioukože za ľavou ušnicou. Histologické vyšetrenie potvrdilo SCAP, ktorý vznikol v teréne nevussebaceus. Keďže táto pseudotumorózna jednotka je často asociovaná aj s inými typmi nádorov,každá lézia, ktorá makroskopicky imponuje ako nevus sebaceus, by mala byť kompletne ex-stirpovaná a dôkladne histologicky vyšetrená. Tým sa zvyšuje predpoklad odhalenia prípadnejkoexistencie inej, prognosticky závažnejšej nádorovej lézie.
Syringocystadenoma papilliferum (SCAP) is a very rare benign adnexal tumour of the skinderived from the apocrine glands. It typically develops on the head and neck, which representup to 90% of all cases. An interesting clinical feature of SCAP is a frequent coincidence withsebaceous naevus that can be found in about one-third of cases. The author describes a casereport of a 56-year-old female who was diagnosed to have a slightly elevated skin tumour inthe left retroauricular region. Histological examination revealed a SCAP arising from seba-ceous naevus. Since the latter pseudotumour entity is often associated even with other neo-plasms, any lesion that grossly appears like a sebaceous naevus should be completely removedand histologically thoroughly examined. This increases the chances of detecting possible co-existence of another, prognostically more unfavourable tumour lesion.
Sebaceous carcinoma in situ (SCIS) is a rare intraepidermal neoplasia, mostly occurring on the eyelid and the face. Despite abundant literature on invasive sebaceous carcinoma, there are relatively few articles about SCIS. We report the case of 78-year-old woman suffering from SCIS of the left cheek, presenting as an erythematous skin lesion. The histological examination revealed intraepidermal and intraadnexal population of atypical cells with foamy cytoplasm and with immunohistochemical adipophilin-, cytokeratin-7- and EMA-positivity in the tumor cells.
Autoři popisují případ 90letého muže, který 4 měsíce pozoroval krvácející nodul na zádech. Histologické vyšetření prokázalo diagnózu sebaceomu. CT vyšetření hrudníku, břicha a pánve bylo negativní. Dva roky po excizi metastatické onemocnění nebylo prokázáno. V přehledu jsou uvedeny současné poznatky o klasifikaci nádorů se sebaceózní diferenciací a jejich vztahu k Muir-Torre syndromu.
The authors describe a case of a 90-year-old man, who was aware of a bleeding nodule on his back for 4 months. Histological examination established the diagnosis of sebaceoma. CT scan of the chest, abdomen and pelvis was negative. Two years after excision, metastatic disease was not detected. The current knowledge of the classification of tumors with sebaceous differentiation and their relationship to Muir-Torre syndrome is presented.
- MeSH
- lidé MeSH
- nádory kožních adnex MeSH
- sebaceózní adenokarcinom * diagnóza chirurgie MeSH
- senioři nad 80 let MeSH
- Torrého-Muirův syndrom etiologie MeSH
- záda anatomie a histologie patologie MeSH
- Check Tag
- lidé MeSH
- mužské pohlaví MeSH
- senioři nad 80 let MeSH
- Publikační typ
- kazuistiky MeSH
- práce podpořená grantem MeSH
Syringocystadenoma papilliferum (SCAP) is a benign tumor most commonly located on the head and neck area often associated with nevus sebaceus. In its usual location, the human papillomavirus (HPV) DNA and mutations in the RAS/mitogen-activated protein kinase signaling pathway have been detected in SCAP. We studied 16 cases of SCAP in the anogenital areas and buttock where this neoplasm is rare and attempted to find out whether SCAP in these sites have different histopathological and molecular biological features. It seems that there is no significant difference between the morphology of anogenital SCAP and SCAP in other locations. Several tumors in our cohort demonstrated features resembling those seen in warts, but HPV DNA was not found in these lesions. On the contrary, we identified DNA of HPV high-risk types in some tumors without HPV-related morphology. Our study confirms the role of HRAS and BRAF V600 mutations in the pathogenesis of SCAP, including SCAP in the anogenital areas and buttock.
- MeSH
- adenomy potních žláz genetika patologie virologie MeSH
- anální kanál patologie MeSH
- dospělí MeSH
- hýždě patologie MeSH
- infekce papilomavirem epidemiologie MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladiství MeSH
- mladý dospělý MeSH
- mutace MeSH
- nádory mužských pohlavních orgánů genetika patologie MeSH
- nádory potních žláz genetika patologie virologie MeSH
- nádory ženských pohlavních orgánů genetika patologie virologie MeSH
- Papillomaviridae MeSH
- protoonkogenní proteiny B-raf genetika MeSH
- protoonkogenní proteiny p21(ras) genetika MeSH
- senioři nad 80 let MeSH
- senioři MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladiství MeSH
- mladý dospělý MeSH
- mužské pohlaví MeSH
- senioři nad 80 let MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- MeSH
- apokrinní žlázy patologie MeSH
- hamartom * MeSH
- lidé MeSH
- nádory kůže * MeSH
- nádory potních žláz klasifikace MeSH
- sebaceózní adenokarcinom klasifikace MeSH
- vlasový folikul patologie MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- přehledy MeSH
