BACKGROUND: An ocular adnexal apocrine adenocarcinoma (OAAA) is an extremely rare, but potentially aggressive and life-threatening tumor with ill-defined management based only on recommendations from a limited number of reported cases. The development of cervical lymphocele following neck dissection is a very rare complication, but one with well established methods for prevention and treatment. Here we describe a previously unreported case of salvage surgery including neck dissection for OAAA in addition to an emergence of cervical lymphocele. A literature review of current knowledge on both pathological conditions is included. METHODS AND RESULTS: A 58-year-old man suffering from OAAA, previously treated with multiple eye-sparing excisions and adjuvant proton therapy, underwent salvage surgery for locoregional recurrence of the tumor. A partial orbitectomy with orbital exenteration, primary reconstruction and left-sided neck dissection was performed. The procedure was complicated by a cervical lymphocele resolved after the surgical therapy. The patient remained disease-free during the one-year follow-up. CONCLUSION: OAAA is a locally aggressive tumor with potential to local or distant metastatic spread. Whole-body staging, regular clinico-radiological follow-up and stage-dependent therapy with surgery as the first-choice treatment is required. A cervical lymphocele as a complication of especially left-sided neck dissection is managed with a conservative or surgical therapy according to the level of lymph leakage, extent and localization of lesions, presence of local or systemic disorders and the period from primary surgery.

• MeSH
• adenokarcinom * patologie   chirurgie   MeSH
• krční disekce škodlivé účinky   metody   MeSH
• lidé středního věku MeSH
• lidé MeSH
• lokální recidiva nádoru MeSH
• lymfokela * etiologie   chirurgie   MeSH
• nádory prsu * chirurgie   MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• Publikační typ
• kazuistiky MeSH
• přehledy MeSH

Canine apocrine gland anal sac adenocarcinoma (AGASACA) is an aggressive canine tumor originating from the anal sac glands. Surgical resection, with or without adjuvant chemotherapy, represents the standard of care for this tumor, but the outcome is generally poor, particularly for tumors diagnosed at an advanced stage. For this reason, novel treatment options are warranted, and a few recent reports have suggested the activation of the immune checkpoint axis in canine AGASACA. In our study, we developed canine-specific monoclonal antibodies targeting PD-1 and PD-L1. A total of 41 AGASACAs with complete clinical and follow-up information were then analyzed by immunohistochemistry for the expression of the two checkpoint molecules (PD-L1 and PD-1) and the presence of tumor-infiltrating lymphocytes (CD3 and CD20), which were evaluated within the tumor bulk (intratumor) and in the surrounding stroma (peritumor). Seventeen AGASACAs (42%) expressed PD-L1 in a range between 5% and 95%. The intratumor lymphocytes were predominantly CD3+ T-cells and were positively correlated with the number of PD-1+ intratumor lymphocytes (ρ = 0.36; p = 0.02). The peritumor lymphocytes were a mixture of CD3+ and CD20+ cells with variable PD-1 expression (range 0-50%). PD-L1 expression negatively affected survival only in the subgroup of dogs treated with surgery alone (n = 14; 576 vs. 235 days). The presence of a heterogeneous lymphocytic infiltrate and the expression of PD-1 and PD-L1 molecules support the relevance of the immune microenvironment in canine AGASACAs and the potential value of immune checkpoints as promising therapeutic targets.

• Publikační typ
• časopisecké články MeSH

• Publikační typ
• abstrakt z konference MeSH

U osmi psů byly klinicky a histologicky diagnostikovány tumory paranálních váčků a u čtyř jedinců byly tumory apokrinních perianálních žláz. V 10 případech byl zjištěn adenokarcinom, ve dvou byl diagnostikován adenom. Tumory se vyskytly u pěti samic, čtyř samců, dvou samců kastrátů a u jedné kastrované samice a častěji byli postiženi kříženci než příslušníci plemene. Informace o průběhu onemocnění po chirurgické excizi nebyly bohužel k dispozici.

In eight dogs were clinically and histologically diagnosed tumors of the paranal sacs and in four animals were tumors of the apocrine perianal glands. Adenocarcinoma was diagnosed in 10 cases, adenoma was diagnosed in two. Tumors occurred in five females, four males, two neutered males and one neutered female, and crossbreeds were more commonly affected than purebreds dogs. Information on the course of the disease after surgical excision was not unfortunately available.

Salivary glands may give rise to a wide spectrum of different tumors. This review concentrates on 4 salivary gland tumors that have been accepted in the recent literature as new neoplastic entities: mammary analog secretory carcinoma, cribriform adenocarcinoma of minor salivary glands (CASG), sclerosing polycystic adenosis/adenoma (SPA), and the mucinous/secretory variant of myoepithelioma. Mammary analog secretory carcinoma is a distinctive low-grade malignant salivary cancer that harbors a characteristic chromosomal translocation, t(12;15) (p13;q25), resulting in an ETV6-NTRK3 fusion. Cribriform adenocarcinoma (CASG) is a distinct tumor entity that differs from polymorphous low-grade adenocarcinoma by location (ie, most often arising on the tongue), by prominent nuclear clearing, differing alterations of the PRKD gene family, and clinical behavior with frequent metastases at the time of presentation of the primary tumor. Early nodal metastatic disease is seen in most cases of CASG; yet, they are still associated with indolent clinical behavior, making it a unique neoplasm among all low-grade salivary gland tumors. SPA is a rare sclerosing tumor of the salivary glands characterized by the combination of cystic ductal structures with variable cell lining including vacuolated, apocrine, mucinous, squamous, and foamy cells, by prominent large acinar cells with coarse eosinophilic cytoplasmic zymogen-like granules, and by closely packed ductal structures, surrounded by a peripheral myoepithelial layer and stromal fibrosis with focal inflammatory infiltrates. SPA frequently harbors intraductal epithelial dysplastic proliferations ranging from mild dysplasia to severe dysplasia/carcinoma in situ. Moreover, SPA has been proven to be a clonal process by HUMARA assay and is associated with considerable risk of recurrence. Therefore, on the basis of all these newly recognized findings, we believe that SPA is likely a neoplasm, and we suggest the name "sclerosing polycystic adenoma." The mucinous variant of myoepithelioma is a myoepithelial tumor with foci of prominent cytoplasmic clearing frequently containing intracellular mucin material and having signet-ring morphology.

• MeSH
• adenokarcinom diagnóza   patologie   terapie   MeSH
• adenom patologie   MeSH
• diferenciální diagnóza MeSH
• lidé MeSH
• myoepiteliální nádor patologie   MeSH
• nádory slinných žláz patologie   MeSH
• prognóza MeSH
• sekreční karcinom mamárního typu diagnóza   patologie   terapie   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• přehledy 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

Cíl studie: Analýza případu extramamární Pagetovy choroby vulvy (EMPD) s přidruženým dobře diferencovaným adenokarcinomem endometria. Typ studie: Kazuistika. Název a sídlo pracoviště: Gynekologicko-porodnická klinika 3. LF UK a FNKV, Praha. Metodika a výsledky: Na tomto případu názorně prezentujeme současné teoretické poznatky o EMPD, obtížnost diagnostiky, léčby a dispenzarizace pa-cientek s touto vzácnou intraepiteliální nonskvamózní neoplazií. Prezentujeme ojedinělou kazuistiku 62leté pacientky s diagnózou EMPD vulvy a přidruženým dobře diferencovaným adenokarcinomem endometria. Závěr: EMPD je vzácná intraepiteliální nonskvamózní neoplazie, která představuje méně než 1 % tumorů vulvy. Nejčastěji postihuje ženy kavkazské rasy v 6. až 8. dekádě života. EMPD postihuje kožní lokality s výskytem apokrinních žláz – vulvu, perineum, perianální oblast, axilu, skrotum, penis, vzácněji oblast stehen či hýždí. Tato neoplazie je charakterizována výskytem specifických tzv. Pagetových buněk v histologickém obraze (jde o atypické obrovské buňky s vydatnou bledou, často retrahovanou cytoplazmou a s velkým jádrem).

Objective: Analysis of one case of vulvar extramammary Paget´s disease (EMPD) and associated well differentiated endometrial adenocarcinoma. Desing: A case report. Methods and results: On this case report we present current theoretical knowledge about EMPD, difficulty of diagnostics, treatment and dispensarization the patients with this rare intraepithelial non-squamous neoplasia. We report a rare case of a 62 years old female patient with extramammary Paget´s disease of vulva and associated well differentiated endometrial adenocarcinoma. Conclusions: EMPD is a rare intraephitelial non-squamous neoplasia, which represents less then 1% vulvar tumors. Predominantly it affects white women between 60 and 80 years of age. EMPD occurs in cutaneous areas bearing apocrine glands – vulva, perineum, perianal area, axilla, penis, scrotum and rarely region of tights or buttocks. It is characterized microscopically by the presence of specific tumor cells called Paget´s cells – atypical large cells with pale clear cytoplasm and large round nuclei.

• Klíčová slova
• imiquimod,
• MeSH
• adjuvancia imunologická terapeutické užití   MeSH
• aminochinoliny terapeutické užití   MeSH
• endometroidní karcinom * diagnóza   patologie   MeSH
• extramamární Pagetova nemoc * diagnóza   chirurgie   patologie   MeSH
• gynekologické chirurgické výkony metody   MeSH
• induktory interferonů terapeutické užití   MeSH
• lidé středního věku MeSH
• lidé MeSH
• nádory vulvy * diagnóza   chirurgie   patologie   MeSH
• protinádorové látky MeSH
• recidiva MeSH
• vulva chirurgie   patologie   MeSH
• vulvektomie MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• kazuistiky MeSH
• práce podpořená grantem MeSH

• MeSH
• apokrinní žlázy patofyziologie   patologie   MeSH
• cerumen * MeSH
• cysty * diagnóza   chirurgie   komplikace   MeSH
• diferenciální diagnóza MeSH
• histologické techniky metody   využití   MeSH
• hyperplazie diagnóza   komplikace   MeSH
• kočky MeSH
• mazové žlázy patofyziologie   patologie   MeSH
• nádory cystické, mucinózní a serózní diagnóza   chirurgie   MeSH
• sebaceózní adenokarcinom diagnóza   chirurgie   MeSH
• statistika jako téma MeSH
• zvířata MeSH
• zvukovod * patofyziologie   patologie   MeSH
• Check Tag
• kočky MeSH
• mužské pohlaví MeSH
• zvířata MeSH
• Publikační typ
• kazuistiky MeSH

• MeSH
• nádory kůže klasifikace   patologie   MeSH

This volume of the 5th edition of the WHO Classification of Tumors focuses on cancer of the breast and presents an essential tool for diagnostic practice worldwide. Written for professionals.

• MeSH
• mezinárodní klasifikace nemocí MeSH
• nádory prsu klasifikace   MeSH
• Světová zdravotnická organizace MeSH
• Publikační typ
• směrnice pro lékařskou praxi MeSH

• Konspekt
• Patologie. Klinická medicína
• NLK Obory
• onkologie
• NLK Publikační typ
• publikace WHO