Anogenital mammary-like glands represent a normal anatomic constituent of the anogenital area and may give rise to many benign and malignant tumors that morphologically mimic similar breast diseases. The literature review is complemented by a description of 286 cases of benign tumors and tumor-like processes in the mammary-like glands. The paper presents the clinical and morphological characteristics of papillary hidradenoma, fibroadenoma, benign phyllodes tumor, lactating adenoma, sclerosing adenosis, pseudoangiomatous stromal hyperplasia, etc.
Маммароподобные железы являются нормальным анатомическим компонентом аногенитальной области и могут давать начало многочисленным доброкачественным и злокачественным опухолям, морфологически напоминающим аналогичные заболевания молочной железы. Обзор литературы дополнен описанием 286 случаев доброкачественных опухолей и опухолеподобных процессов маммароподобных желез. В статье представлена клинико-морфологическая характеристика папиллярной гидраденомы, фиброаденомы, доброкачественной филлоидной опухоли, лактирующей аденомы, склерозирующего аденоза, псевдоангиоматозной стромальной гиперплазии и др.
- MeSH
- adenom diagnóza patologie MeSH
- akrospirom diagnóza patologie MeSH
- cystosarcoma phyllodes diagnóza patologie MeSH
- fibroadenom diagnóza patologie MeSH
- laktace fyziologie MeSH
- lidé MeSH
- nádory prsu klasifikace diagnóza patologie MeSH
- nádory klasifikace diagnóza patologie MeSH
- prsy patologie MeSH
- Světová zdravotnická organizace MeSH
- Check Tag
- lidé MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- přehledy MeSH
Lesions affecting anogenital mammary-like glands (AGMLG) are histopathologically very similar to those seen in the breast but whether this morphological similarity is also reflected at the genetic level is unknown. To compare the underlying molecular mechanisms in lesions of AGMLG and their mammary counterparts, we analyzed the mutational profile of 16 anogenital neoplasms including 5 hidradenomas papilliferum (HP), 1 lesion with features of HP and fibroadenoma (FA), 7 FA, 3 phyllodes tumors (PhT)) and 18 analogous breast lesions (6 intraductal papillomas (IDP), 9 FA, and 3 PhT) by high-coverage next generation sequencing (NGS) using a panel comprising 50 cancer-related genes. Additionally, all cases were analyzed for the presence of a mutation in the MED12 gene. All detected mutations with allele frequencies over 20% were independently validated by Sanger sequencing (concordance: 100%). Mutations in PIK3CA, AKT1, MET, ABL1 and TP53 genes were found in lesions of AGMLG and also their mammary counterparts. The PI3K-AKT cascade plays a role in tumors arising at both sites. It appears that some histopathologically similar anogenital and breast lesions develop along similar molecular pathways.
- Klíčová slova
- AKT1, Anogenital mammary-like glands, Breast, Fibroadenoma, Hidradenoma papilliferum, Intraductal papilloma, PIK3CA, Phyllodes tumor, Vulva,
- MeSH
- adenomy potních žláz metabolismus patologie MeSH
- cystosarcoma phyllodes metabolismus patologie MeSH
- fibroadenom metabolismus patologie MeSH
- fosfatidylinositol-3-kinasy třídy I metabolismus MeSH
- fosfatidylinositol-3-kinasy metabolismus MeSH
- lidé středního věku MeSH
- lidé MeSH
- mutace genetika MeSH
- nádory prsu genetika patologie MeSH
- nádory vulvy patologie MeSH
- papilom intraduktální metabolismus patologie MeSH
- prsy patologie MeSH
- senioři MeSH
- vysoce účinné nukleotidové sekvenování metody MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- Názvy látek
- fosfatidylinositol-3-kinasy třídy I MeSH
- fosfatidylinositol-3-kinasy MeSH
- PIK3CA protein, human MeSH Prohlížeč
A 74-year-old woman was admitted in a serious condition due to the failing right heart. A CT scan revealed a tumor infiltration through the interventricular septum in the right heart, spreading from the apex as far as under the tricuspid valve. The tumor penetrated into the conus of the pulmonary artery, bulging and markedly narrowing the lumen. As a result of the tumor infiltration, the patient died from cardiac failure. Histological examination of the tumor revealed atypical elongated cells and areas of large cells with significantly enlarged hyperchromatic and lobulated nuclei. In some portions, the tumors had a biphasic appearance. The tumor cells resembled epithelial tissue but immunohistological analyses to detect cytokeratins yielded negative results. The elongated cells expressed desmin and smooth muscle actin. A vast majority of the tumor was solid or hard, histologically corresponding to osteosarcoma. Later, it was found that the patient undergone right-sided mastectomy for a malignant phyllodes tumor with osteosarcomatous differentiation three years previously. The metastasis to the heart was the only metastasis detected by the autopsy.
- MeSH
- cystosarcoma phyllodes patologie MeSH
- lidé MeSH
- nádory prsu patologie MeSH
- nádory srdce sekundární MeSH
- osteosarkom * MeSH
- sekundární malignity * MeSH
- senioři MeSH
- Check Tag
- lidé MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
Long considered to be ectopic breast tissue representing the caudal remnants of the milk ridges, anogenital mammary-like glands are nowadays thought to represent a normal constituent of the anogenital area. Lesions involving these glands, benign or malignant, epithelial or stromal manifest a striking similarity to their mammary counterparts. This review addresses the recent literature on lesions of anogenital mammary-like glands and our personal experience with various lesions related to these structures. Discussed are the normal anatomy and histology of these glands as well as the clinical presentation, histopathological and immunohistochemical features, molecular biological aspects, and differential diagnosis of various lesions involving anogenital mammary-like glands, including lactating adenoma, hidradenoma papilliferum, hidradenocarcinoma papilliferum, fibroadenomas, phyllodes tumor, pseudoangiomatous stromal hyperplasia, extramammary Paget disease, and other carcinomas. In addition, "nonspecific" epithelial or stromal changes some of which can be likened to similar changes occurring in a range of benign breast disease, including sclerosing adenosis, columnar cell lesions, ductal lesions and various metaplastic changes affecting epithelium and myoepithelium are discussed. Although lesions of anogenital mammary-like glands are often discussed in many dermatopathology textbooks in the context of cutaneous adnexal neoplasms we advocate that the best approach to the diagnosis of these lesions is to relate them to analogous well recognized lesions occurring in the breast, that is, through the eyes of a breast pathologist. This will enable their recognition, precise classification and should introduce greater uniformity in how they are reported in the literature so that more meaningful clinicopathological comparisons and correlations may be made.
- MeSH
- adnexa uteri patologie MeSH
- akrospirom patologie MeSH
- cystosarcoma phyllodes patologie MeSH
- duktální karcinom prsu patologie MeSH
- extramamární Pagetova nemoc patologie MeSH
- fibroadenom patologie MeSH
- laktace MeSH
- lidé MeSH
- lobulární karcinom patologie MeSH
- mléčné žlázy lidské patologie MeSH
- nádory kožních adnex patologie MeSH
- nádory vulvy patologie MeSH
- nemoci děložních adnex patologie MeSH
- vulva cytologie MeSH
- Check Tag
- lidé MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- přehledy MeSH
The authors present a series of 13 fibroepithelial neoplasms involving anogenital mammary-like glands, all occurring in 12 female patients, whose age at diagnosis ranged from 30 to 51 years (mean, 38 y; median, 42 y). All women presented with a solitary asymptomatic nodule in the vulva (n=8), perineum (n=2), or near the anus (n=2) ranging in size from 1.5 to 4.5 cm. Microscopically, 8 lesions were classified as fibroadenoma, and 5, including 1 recurrent tumor, as phyllodes tumor, of which 1 was benign and 4 low-grade malignant. In addition to conventional findings, we describe several hitherto unreported features including juvenile fibroadenoma-like proliferation, fibroadenoma with lactation change, and pseudoangiomatous stromal hyperplasia with multinucleated stromal giant cells in a patient with neurofibromatosis, type 1 all constituting potential diagnostic pitfalls, which are best averted by using the same approach to diagnosis as for their analogous mammary counterparts.
- MeSH
- cystosarcoma phyllodes komplikace metabolismus patologie MeSH
- dospělí MeSH
- fibroadenom komplikace metabolismus patologie MeSH
- imunohistochemie MeSH
- lidé středního věku MeSH
- lidé MeSH
- mléčné žlázy lidské metabolismus patologie MeSH
- nádory anu komplikace metabolismus patologie MeSH
- nádory ženských pohlavních orgánů komplikace metabolismus patologie MeSH
- neurofibromatóza 1 komplikace patologie MeSH
- obrovské buňky patologie MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
Pseudoangiomatous stromal hyperplasia (PASH), first reported in 1986, is nowadays a well-recognized change in the breast. We present three cases of lesions involving anogenital mammary-like glands demonstrating this feature. All patients were females (ages, 42, 43, and 53 years). Each presented with a solitary, 1.5- to 2-cm asymptomatic nodule. Locations included the perianal area, perineum, and labium majus. Histopathologically, one lesion was classified as low-grade phyllodes tumor, another as fibroadenoma, and in the remaining case PASH was found in the background of mild hyperplasia of anogenital mammary-like glands and substantial lipomatous metaplasia. In all lesions, PASH had an identical appearance to that in the breast, that is open, slit-like, often anastomosing channels devoid of erythrocytes and lined by discontinuous, often attenuated, inconspicuous cells without atypia or mitotic activity set in a hyalinized collagenous stroma. Quantitatively, PASH ranged in the above cases, forming a relatively small focus in the fibroadenoma and being quite extensive in the remaining two cases. In the phyllodes tumor, PASH areas exhibited focal hypercellularity and presence of myoid cells. In none of the cases were there cells with intranuclear inclusions or multinucleated cells. The lesions were surgically excised. Two patients with follow-up were disease-free at one and three years after the operation. As to our knowledge, PASH has not been previously described in the anogenital area, this feature seems to have been either overlooked or is genuinely rare in this location. It may occur in a preexisting lesion of anogenital mammary-like glands or may apparently by itself produce a clinically detectable lesion. The clinicopathologic features of PASH in the anogenital area seem to be identical to those in the breast.
- MeSH
- buňky stromatu metabolismus patologie MeSH
- cystosarcoma phyllodes metabolismus patologie MeSH
- diferenciální diagnóza MeSH
- dospělí MeSH
- fibroadenom metabolismus patologie MeSH
- fibrom metabolismus patologie MeSH
- hyperplazie metabolismus patologie MeSH
- imunohistochemie MeSH
- lidé středního věku MeSH
- lidé MeSH
- mnohočetné primární nádory patologie MeSH
- nádory anu metabolismus patologie MeSH
- nádory prsu patologie MeSH
- nádory vulvy metabolismus patologie MeSH
- perineum patologie MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
PURPOSE: Mastectomy without axillary dissection should be the standard treatment in patients with malignant form of cystosarcoma phyllodes. The role of postoperative radiotherapy and chemotherapy remains to be fully established. We evaluate treatment results in a group of patients with cystosarcoma phyllodes (CP) treated at our Institute. PATIENTS AND METHODS: In this report we analyze treatment outcome in 25 patients with malignant cystosarcoma phyllodes treated at Masaryk Memorial Cancer Institute between 1970 and 1995. Mean tumor size was 10 cm in diameter. All patients underwent surgery. Subsequently, 17 patients (68%) received radiotherapy on the breast or chest wall. RESULTS: Median follow-up was 139.5 months. Local recurrence was observed in 16% of all patients and all patients with local recurrence died. Time to local relapse after surgery was 4-11 months. Distant metastases occurred in 5 patients. All patients with local recurrence had distant metastases. Dissemination occurred 3-19 months after local recurrence. Five-year survival of all patients was 80%. CONCLUSION: A specific protocol for the treatment of cystosarcoma phyllodes is missing, probably due to rarity of the disease. The treatment of local recurrent disease remains unsuccessful in most CP patients. We recommend postoperative irradiation on the chest wall in patients with malignant form of CP, because adjuvant radiotherapy decreased the incidence of local relapse in our group of patients.
- MeSH
- analýza přežití MeSH
- chorobopisy MeSH
- cystosarcoma phyllodes diagnóza epidemiologie etiologie patologie terapie MeSH
- dospělí MeSH
- kombinovaná terapie MeSH
- lidé středního věku MeSH
- lidé MeSH
- lokální recidiva nádoru diagnóza epidemiologie etiologie patologie terapie MeSH
- nádory prsu diagnóza epidemiologie etiologie patologie terapie MeSH
- přežití po terapii bez příznaků nemoci MeSH
- retrospektivní studie MeSH
- senioři MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- Geografické názvy
- Česká republika epidemiologie MeSH
Phylloides tumor of the breast is well known entity in pathology for a long time. It includes fibroepithelial tumors of the different biological character. They are generally divided into three groups: benign, low-grade malignant (borderline) and high-grade malignant. Adenoses of the breast are a group of the benign lesions, the main feature of which is the proliferation of the glandular component of the breast. More frequent adenoses occurring in practice are: blunt duct adenosis, sclerosing adenosis, nodular adenosis, adenosis tumor and florid adenosis. The less frequent adenoses are: microglandular adenosis, tubular adenosis, apocrine adenosis, and myoepithelial (adenomyoepithelial) adenosis. In this article two histologically unusual lesions of the breast in middle-aged women are presented, consisting of the benign variants of the phylloides tumor, modified distinctly by various types of adenoses, which can cause, together with intensive myoepithelial proliferation, great diagnostic problems, especially on frozen sections. They may be mistaken with invasive ductal or lobular cancer. Reliable differentiation between these entities is possible only by using of the immunohistochemical methods.
- MeSH
- cystosarcoma phyllodes komplikace patologie MeSH
- dospělí MeSH
- fibrocystická nemoc prsu komplikace patologie MeSH
- lidé středního věku MeSH
- lidé MeSH
- nádory prsu komplikace patologie MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- ženské pohlaví MeSH
- Publikační typ
- anglický abstrakt MeSH
- časopisecké články MeSH
- kazuistiky MeSH
In our study, the expression of Ki-67 antigen in phyllodes tumours of the breast was investigated in order to estimate its significance in assessing the grade of malignancy and in predicting the clinical behaviour of these tumours. Formalin-fixed and paraffin-embedded tissue specimens of 37 cases of phyllodes tumour of the breast (eight graded as malignant and 29 as benign according to histopathological criteria) and 20 breast fibroadenomas were stained by an immunoperoxidase technique, using the monoclonal antibody MIB1 directed against cell proliferation-associated Ki-67 antigen. A MIB1 index was calculated to express the percentage of MIB1 positive proliferating stromal cells. Our data indicate that there are statistically significant differences in MIB1 indices between histologically benign and malignant phyllodes tumours (alpha = 0.001), between benign phyllodes tumour and fibroadenoma (alpha = 0.01), and between malignant phyllodes tumour and fibroadenoma (alpha = 0.001). Our results also indicate a good correlation between the conventional grading of phyllodes tumours based on histological criteria and MIB1 indices. However, little predictive value of the MIB1 index in phyllodes tumours of the breast can be derived from our study.
- MeSH
- antigeny jaderné MeSH
- cystosarcoma phyllodes metabolismus patologie chirurgie MeSH
- dospělí MeSH
- fibroadenom metabolismus patologie chirurgie MeSH
- imunohistochemie metody MeSH
- jaderné proteiny metabolismus MeSH
- lidé středního věku MeSH
- lidé MeSH
- lokální recidiva nádoru MeSH
- mladiství MeSH
- monoklonální protilátky MeSH
- nádory prsu u mužů metabolismus patologie chirurgie MeSH
- nádory prsu metabolismus patologie chirurgie MeSH
- následné studie MeSH
- senioři MeSH
- senzitivita a specificita MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladiství MeSH
- mužské pohlaví MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
- Názvy látek
- antigeny jaderné MeSH
- jaderné proteiny MeSH
- monoklonální protilátky MeSH
The concentrations of three steroid hormones (estrogen, progesteron and 1,25-dihydroxycholecalciferol) receptors (ER, PgR, DR) in tissue cytosol were analyzed in a group of 17 breast phylloid tumors. Comparison with breast carcinoma tissue samples (n = 37) did not reveal significant differences in average values of ER, PgR, and DR. Comparison with another control set of 30 samples of dysplastic tissue of the mammary gland showed significant differences only in PgR values. Only 18% of phylloid tumor samples contained levels above cut-of-line of all three receptors (ER, PgR, DR-5,10,10 resp. fmol/ mg protein). The most frequent combination was ER+PgR+DR-(41%). As far as we know, DR in phylloid breast tumors have never been examined before. In approximately 60% of our samples we found the expression of DR, in 36% the estimated values were above 10 fmol/mg protein. Cells of the tissue not expressing DR seem to belong to a special phenotype. We found no ER+PgR- or ER-PgR-combinations in them. The group which expresses DR is characterized by a higher dispersion of PgR values.
- MeSH
- cystosarcoma phyllodes chemie patologie MeSH
- karcinom chemie MeSH
- lidé MeSH
- nádory prsu chemie patologie MeSH
- prsy chemie MeSH
- receptory kalcitriolu analýza MeSH
- receptory pro estrogeny analýza MeSH
- receptory progesteronu analýza MeSH
- Check Tag
- lidé MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- srovnávací studie MeSH
- Názvy látek
- receptory kalcitriolu MeSH
- receptory pro estrogeny MeSH
- receptory progesteronu MeSH
