Gorlin-Goltz syndrome.

BACKGROUND: Gorlin-Goltz syndrome is an autosomal dominant inherited disorder characterized by a predisposition to various cancers. Clinicopathological findings of syndrome are very diverse and many symptoms begin to manifest in a certain period of life. CASE: The authors describe a case report of a man who, at the age of 34 years, presented to a dermatologist with multiple tumor lesions of the skin. The lesions started to develop when he was 30 years old and thereafter increased in number. Histology revealed superficial, superficial-nodular and nodular basal cell carcinomas. A total of 11 basal cell carcinomas were surgically removed and microscopically investigated. The others were treated locally with imiquimod cream and cryotherapy. In addition, he was found to have multiple odontogenic keratocysts in the jaw and mandible, as well as supernumerary and retinated teeth. Stomatologic and maxillofacial surgery interventions were performed. Further clinical and imaging examinations confirmed macrocephaly, hypertelorism, calcification of falx cerebri, and abnormalities of the cervical vertebrae. The spectrum of pathological findings met the diagnostic criteria of Gorlin-Goltz syndrome. CONCLUSION: Although Gorlin-Goltz syndrome is very rare in routine practice, it usually represents a serious disease with multiple organ system involvement. From a prognostic point of view, early diagnosis with adequate therapy is critical. If a diagnosis is confirmed, lifetime dispensary care with interdisciplinary medical cooperation is necessary. The authors would like to thank all physicians who participated in the diagnostics and therapy of the presented patient. The authors declare they have no potential confl icts of interest concerning drugs, products, or services used in the study. The Editorial Board declares that the manuscript met the ICMJE recommendation for biomedical papers. Submitted: 30. 8. 2018 Accepted: 8. 1. 2019.

Fibroepithelioma of Pinkus.

Basal cell carcinoma (BCC) is recently the most common cancer in humans characterized by several histopathological subtypes. Fibroepithelioma of Pinkus (FEP) is traditionally classified as a very rare variant of BCC, however, it manifests clinical and morphological differences that distinguish it from most other types BCCs. This study was performed to evaluate the incidence of FEP and clinical-pathological characteristics of patients diagnosed with this tumor. Four cases of primary FEP (3 females, 1 male, mean age 53.4 y) were analyzed retrospectively. The prevalence of FEP was 0.7 % of all diagnosed BCCs. Topographically, tumors were localized on the right brachium, right gluteal region, left mesogastrium, and right side of the abdomen. Histological examination showed typical anastomosing cords of basaloid cells extending from the overlying epidermis into the loose fibrous stroma in the dermis. Mitotic activity or significant cellular atypia, as well as sign of solar dermatosis were absent. Mean horizontal and vertical diameter of the lesions were 7.7 and 2.8 mm, retrospectively. We did not observe infiltration of deeper skin structures. All lesions were removed completely and classified as pathological stage pT1. Three cases manifested typical picture of a "pure" FEP, one lesion had partially a feature of nodular type BCC, too. Although FEP is conventionally considered as indolent BCC variant with a favorable clinical outcome, recent evidences also favors its classification as a form of trichoblastoma. This is appropriate example how some types of cutaneous tumors can overlap in terms of dignity and clinical-morphological characteristics. This should be taken into account in differential diagnosis and in predicting of biological behaviour of the individual tumors of the skin (Tab. 2, Fig. 3, Ref. 26).

Comparision of histological types of primary and subsequent relapsing basal cell carcinomas of the skin.

BACKGROUND: Basal cell carcinoma (BCC) recurrences are relatively frequent event in a routine dermatologic practice. One of the most important factor which impacts risk of their development is a histomorphological appearance of tumor. DESIGN: The purpose of our study was to compare histological types of primary and corresponding relapsing BCCs of the skin. MATERIAL AND METHODS: The study included 36 cases of BCC recurrences from 34 patients, 17 women and 17 men. The patients ranged in age from 32 to 97 years, with a mean age of 67.1 years at the time of (the first) recurrence. RESULTS: Both tumor groups generally exhibited the same proportion of indolent and aggressive histological phenotype. In 21 cases (58.4%), we found an identical histological BCC type in primary and subsequent relapsing lesion. In 3 cases (8.3%), primary lesion showed indolent histological features without aggressive--growth component, while recurrent tumor already manifested it. Conversely, in next 3 cases (8.3%) primary tumor exhibited focal infiltrative-growth features and corresponding relapsing lesion did not. Of the remaining 9 cases (25%), histomorphological phenotype was not identical, but it showed the same prognostic histological tumor variant. CONCLUSION: Based on the results of our study it can be assumed that a BCC recurrence is a dynamic histogenetic process, during which the phenotypic transformation and the changes in histomorphological picture of lesions occur, probably as a result of the interactions between cancer cells and re-modulated surrounding stroma.

Immunohistochemical and histomorphological analysis of rat mammary tumors after simvastatin treatment.

The results of experimental studies have indicated the pleiotropic effects of statins in organism, e.g. the influence on cell cycle, apoptosis or angiogenesis. In this study, the effects of simvastatin on selected parameters of apoptosis and proliferation in chemocarcinogen-induced mammary tumorigenesis in female rats were determined. Simvastatin was administered dietary at a dose of 18 mg/kg and highly effective dose of 180 mg/kg the entire experiment (18 weeks). At autopsy mammary tumors were removed and prepared for immunohistochemical and histomorphological analysis. In treated animals (simvastatin 180 mg/kg), significant decrease by 12% in Bcl-2 protein expression and non-significant decrease by 27% of Ki67 protein expression in tumor cells compared to tumor cells in control animals were observed after semiquantitative evaluation. Morphometrical analysis has shown significant proapototic shift in Bcl-2/Bax ratio in tumor cells. In high grade control carcinoma cells, the expression of Ki67 increased by 37% (non-significantly) in comparison with control low grade carcinomas. A histomorphological analysis of malignant tumors has revealed a shift from high grade to low grade carcinomas after simvastatin treatment. The noticeable decrease of mammary tumor frequency and incidence in rats after simvastatin treatment was accompanied with antiapoptotic Blc-2 protein decrease and proapoptotic Bax protein increase in this experiment.

[Giant cutaneous basal cell carcinoma of the head with intracranial propagation--a case report]. / "Gigantický" bazocelulárny karcinóm koze hlavy s intrakraniálnou propagáciou--kazuistika.

Basal cell carcinoma (BCC) of the skin is generally characterised by a favourable clinical outcome. The slow and mostly local character of growth helps in its early recognition, thus the vast majority of cases are diagnosed in the early phase of disease. However, in cases of long-term neglect of clinical symptoms, certain cancers may reach huge proportions and may significantly destroy surrounding tissue. BCCs larger than 5 cm are called giant BCCs. The authors of the article present a case report of woman suffering from a giant BCC of the head with a history of 15 years of lasting growth, during which she had refused a medical examination. Finally, she was forced to go into hospital due to episodes of unconsciousness and convulsions. Clinical investigations revealed a huge ulcerating tumour in the fronto-parietal region infiltrating the skull and penetrating into the cranial cavity with compression of the brain. A surgical extirpation of the tumor-affected soft tissue and the calva was performed with plastic reconstruction of dura mater and skin. Microscopic examination of biopsy specimens confirmed a diagnosis of mixed BCC with nodular, infiltrative and metatypical features, which had completely infiltrated calva and dura mater. It was not possible to surgically remove a part of the tumour-affected bones of the left orbita, thus the patient is going to undergo local radiotherapy. This case report emphasizes the fact that BCC, in spite of its usually "benign" biological behaviour, should never be underestimated because it may progress to the advanced stage of the disease, for which treatment is much more difficult with a larger negative impact and a significantly worse prognosis for the patient.

[Solitary distal femoral osteosarcoma in 89-year-old woman. Case report]. / Solitárny osteosarkóm distálneho femuru diagnostikovaný u 89-rocnej zeny.

The authors describe the case of an 89-year-old patient with a one-month history of pain in the right knee and a pathological fracture of the distal femur. Excisional biopsy showed a classical osteogenic sarcoma. The patient died after six months of palliative surgical and oncological therapy; her primary disease generalised and progressed with numerous metastases. The authors discuss the possibilities of histological diagnostics of osteogenic sarcoma and its differential diagnosis. They draw attention to a possible occurrence of this type of sarcoma also in elderly patients, and not only in adolescents.

[Basal cell carcinoma of the skin--biological behaviour of the tumor and a review of the most important molecular predictors of disease progression in pathological practice]. / Bazocelulárny karcinóm koze--biologické správanie nádoru a prehl'ad najvýznamnejsích molekulových ukazovatel'ov progresie ochorenia v praxi patológa.

Basal cell carcinoma of the skin is currently the most frequent malignancy in human population. Basal cell carcinoma represents a heterogeneous group of tumors with a variable clinical and morphological picture. Based on its biological behaviour, we generally differentiate between indolent (superficial and nodular) and aggressive type (infiltrative, micronodular, and metatypical) of basal cell carcinoma. Because of the different biological characteristics of these tumors, it is questionable whether they are a part of a continuous spectrum of carcinogenesis, starting with indolent and ending with aggressive forms, or they represent separate developmental lines. In the current clinical practice, there is an increasing demand for identification of tumors that are prognostically more adverse and their impact on the overall health status of patients is more serious. Recent advances in pathology and molecular medicine allow identification of various biomarkers from tumor tissue that are significantly involved in the mechanisms of malignant cell transformation. Detection of these biomarkers is of great importance in predicting further clinical behaviour of the cancer. The authors of the paper present basic information about biological behaviour of cutaneous basal cell carcinoma and provide an overview of the most important biomarkers that influence the clinical outcome and disease progression and are detectable through a routine biopsy tissue examination. It is now necessary to search for novel histological and molecular parameters that, in the future, could have a prognostic value in diagnostic and therapeutic process of this disorder.

WITHDRAWN: The efficiency of polyphenolic compounds on allergen induced hyperreactivity of the airways.

The Publisher regrets that this article is an accidental duplication of an article that has already been published, http://dx.doi.org/10.1016/j.bionut.2010.09.002. The duplicate article has therefore been withdrawn.

Congenital polycystic kidney disease in one of the twins.

Cystic renal disorders generally comprise a heterogeneous group of conditions. The most common genetic form is a polycystic kidney disease. This nosologic entity can be inherited as either an autosomal recessive or autosomal dominant trait. Among all cystic renal disorders, autosomal recessive polycystic kidney disease is the most frequent heritable disease manifesting in infancy and childhood and is among those that come to clinical attention early. The characteristic pathologic changes occur in the kidneys and the liver, however, several other organ systems can be affected secondarily. Both kidneys are enlarged because of multiple progressive cystic dilatation of the renal tubules that results to renal failure. The liver is characterized by periportal fibrosis with bile ducts abnormalities. The renal and hepatic manifestations are more or less inversely proportional in individual patients. Therefore, the morphological features and clinical presentation of this disorder can vary substantially. Moreover, there is also a great variability in the severity and manifestations even between individuals from the same family who carry the identical mutations. The authors present a case report of a fatal clinical course of the perinatal congenital polycystic kidney disease in one of the twins (Fig. 4, Ref. 19).

[Perforating folliculitis. Case report and differential diagnosis]. / Folliculitis perforans kazuistický prípad a diferenciálna diagnostika choroby.

A 26-year-old woman suddenly noticed an onset of disseminated papular exanthema during her institutional treatment for alcohol abuse. After 9 weeks of slowly progressive course a dermatologist was consulted, who suggested a diagnosis of pityriasis lichenoides. Skin biopsy revealed cystical enlargement of hair follicles that were filled up with keratinous and parakeratotic masses interspersed with particles of fragmented collagen. Numerous inflammatory cells were present within the lesion. Many focal perforations of infundibular epithelium draining a cellular debris were prominent. Severely disturbed follicles were engulfed by giant cell inflammatory infitrate containing remnants of collagen and elastic fibers. Considering all the histopathologic findings and clinical course, the case was finally diagnosed as perforating folliculitis, a distinct type from the family of perforating dermatoses.

Side effects of anastrozole in the experimental pre-menopausal mammary carcinogenesis.

UNLABELLED: The aim of this study was to assess side effects of aromatase inhibitor anastrozole in the prevention of N-methyl-N-nitrosourea - induced pre-menopausal mammary carcinogenesis in female Sprague-Dawley rats. This model mimicked situation in healthy, but from the point of view of the development of breast cancer, high-risk pre-menopausal women.

Aromatase inhibitor anastrozole was used as a chemopreventive agent taken by the animals in the food during the whole period of time of the experiment. Group 1 - the control group had taken food without anastrozole, the groups 2 and 3 with anastrozole in various concentrations - 0.05 mg/1 kg of food (ANA 0.05) and 0.5 mg/1 kg of food (ANA 0.5).

In anastrozole-treated animals in comparison with untreated animals, macroscopic changes of uterus and vagina were not found. The values of absolute and relative wet weight of uterus and vagina in the groups ANA 0.05 and ANA 0.5 were comparable with the control. Histological examination did not show atrophic changes in endometrium of uterus and in epithelium of vagina in anastrozole-treated animals. In the group ANA 0.5 myometrium was significantly grosser than in the group ANA 0.05 (P<0.05). Anastrozole neither affects parameters of plasma lipid metabolism (triacylglycerols, total cholesterol, low - density lipoprotein cholesterol and high - density lipoprotein cholesterol) nor serum levels of sex hormones (estradiol, testosterone, dehydroepiandrosterone). Compact bone thickness in the groups with anastrozole was significantly increased in comparison with untreated animals (P<0.001). A significant increase in body weight was found in the group ANA 0.5 compared with the control group (P<0.01). The significant increase in body weight gain was not attended by the significant increase in food intake.

The side effects of aromatase inhibitor anastrozole in the prevention of N-methyl-N-nitrosourea - induced pre-menopausal mammary carcinogenesis in female Sprague-Dawley rats on myometrium, compact bone thickness and body weight gain were observed.

Effects of flavin7 on allergen induced hyperreactivity of airways.

Some studies have suggested that the polyphenolic compounds might reduce the occurrence of asthma symptoms. The aim of our experiments was to evaluate the effects of 21 days of the flavonoid Flavin7 administration on experimentally induced airway inflammation in ovalbumin-sensitized guinea pigs. We assessed tracheal smooth muscle reactivity by an in vitro muscle-strip method; changes in airway resistance by an in vivo plethysmographic method; histological picture of tracheal tissue; and the levels of interleukin 4 (IL-4), and interleukin 5 (IL-5) in bronchoalveolar lavage fluid (BALF). Histological investigation of tracheal tissue and the concentrations of the inflammatory cytokines IL-4 and IL-5 in BALF were used as indices of airway inflammation. Administration of Flavin7 caused a significant decrease of specific airway resistance after histamine nebulization and a decline in tracheal smooth muscle contraction amplitude in response to bronchoconstricting mediators. Flavin7 minimized the degree of inflammation estimated on the basis of eosinophil calculation and IL-4 and IL-5 concentrations. In conclusion, administration of Flavin7 showed bronchodilating and anti-inflammatory effects on allergen-induced airway inflammation.

[Bloch-Sulzberg syndrome in pathology]. / Blochov-Sulzbergov syndróm v patológii.

The authors present some pathological findings in the skin and hair of the child affected by rather rare Bloch-Sulzberg syndrome manifested in incontinentia pigmenti, followed for 10 years. In this work are presented also some recent data about pathogenesis of the disease with X-chromosome dominant heredity, primary of neuroectodermal origincaused by mutation of nuclear factor kappa-B of essential modulator (NEMO) of the gene (chromosomal locus Xq28).

[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.

[Appendiceal mucocele causing pseudomyxoma peritonei]. / Appendixmukozele als Ursache von Pseudomyxoma peritonei (Gallertbauch).

The authors present the case of a very rare pseudomyxoma peritonei as a late complication of appendectomy. The definite diagnosis was established 11 years after appendectomy for appendiceal mucocele. In the paper the authors emphasize the need for correct retrospective evaluation of all data in patients with unusual diagnoses.

[Reactive angioendotheliomatosis--a rare disease mimicking Kaposi sarcoma: a case report]. / Reaktívna angioendoteliomatóza--zriedkavé kozné ochorenie napodobnujúce Kaposiho sarkóm: kazuistika.

Reactive angioendotheliomatosis (RAE) is a rare disorder with clinical presentation of multiple cutaneous lesions. RAE mimics mainly Kaposi sarcoma (KS) and, rarely angiosarcoma, and it usually arises in association with other systemic disease. The associated disease is mostly chronic infection. RAE presents diagnostic problems for both the clinician and pathologist. We present RAE in a 46-year-old male patient with a history of alcoholism and pulmonary tuberculosis. Multiple, rapidly progressive, itching and burning, and focally confluent cutaneous erythematous and purple macules, plaques and nodules of various size and wide distribution appeared during a course of antituberculotic therapy. The suggested clinical diagnosis was KS. The lesions were repeatedly evaluated by biopsy. Histologically, there were poorly marginated proliferations of capillaries in the dermis which extended focally into the subcutaneous tissue. Associated erythrocyte extravasations, stromal hemosiderin depositions and mild chronic inflammatory infiltrates were also present in the lesion. Adnexal and neuronal extension of the lesional vessels was an intriguing finding, not described in the literature until now. The microscopical findings were interpreted by surgical pathologists as capillary hemangioma and as "angioblastoma of Nakagawa" (one and two times, respectively).

[Granulomatous skin disease after an exotic injury]. / Granulomatózne ochorenie koze po exotickom poranení.

Piranhas are feared freshwater fishes commonly found in the waters of southern Venezuela to the north of Argentina. At present this species is a favourite domesticated article of our aquarists. The authors describe the histopathologic picture of a cutaneous lesion of the right hand thumb of a 60-year old piranha keeper who was bitten by this predatory fish. A firm node with increasing soreness gradually developed in the area of the lesion, and after 7 months the symptoms of the carpal tunnel syndrome were also present. Granulomatous foci--some sarcoid-like, others with central necrosis--were permeated with fibrinoid substance. Epithelioid granuloma cells with huge incorporated multinuclear elements were covered mainly by T-lymphocytes. Ziehl-Neelsen stain did not prove the presence of diagnostic acid-fast rods. Only in some macrophages rare acid-fast spheric microstructures were present in the cytoplasm. Both the local dermatologic finding and histological examination gave evidence that the skin was infected with atypical mycobacteria--swimming pool granuloma.

[Massive localized lymphedema: a reactive lesion simulating liposarcoma (case report]. / Masívny lokalizovaný lymfedém: reaktívna lézia simulujúca liposarkóm (Kazuistika).

The authors present a case-history of massive localized lymphedema in a 54 year old female patient (height 167 cm, weight 113 kg). The history of the lymphedema lasted about 1 year. Its growth was not accompained with subjective complaints. It was diagnosed as a pendulous tumor of soft tissues in the thigh, 70 x 65 cm large. In preoperative diagnosis it was classified as a liposarcoma. The tumor lesion was removed and sent for bioptic examinations. Both histological and immunohistochemical biopsies denied benign or malignant nature of the soft tissue tumors and confirmed the diagnosis of a massive localized lymphedema.