CT urografija (CTU) je slikovna metoda specifično prilagođena oslikavanju i procjeni gornjeg mokraćnog sustava i mokraćnog mjehura. Visoka dijagnostička točnost u detekciji primarnih i recidivnih ...tumora urotela, razlikovanje malignih od benignih lezija, karakterizacija anatomskih varijacija i malformacija te procjena relevantnih patomorfoloških promjena u okolnim tkivima čine CT urografiju vrijednom metodom za oslikavanje mokraćnog sustava. Hematurija, inicijalna procjena proširenosti i praćenje tumora gornjeg mokraćnog sustava i mokraćnog mjehura najvažnije su indikacije za CTU. „Triple phase” i „split bolus” tehnike su najčešće korišteni CTU protokoli koje sačinjavaju nativna, nefrografska i ekskretorna faza, dobivene skeniranjem regije tri ili dva puta. CTU protokoli skeniranja su dizajnirani s ciljem ostvarivanja najbolje kvalitete slike te optimalne distenzije i opacifikacije mokraćnog sustava uz najmanju moguću dozu zračenja, u rasponu od 5 – 15 mSv, što je usporedivo s dozama zračenja intravenske urografije. Detaljna anamneza, klinički pregled i analiza urina ključni su za pravilan probir pacijenata i ciljani odabir CTU protokola, te su ujedno najučinkovitiji način poboljšavanja dijagnostičke točnosti i smanjivanja doze zračenja CT urografije.
With the aim of improving personalized treatment of patients on chemotherapy, the objective of the study was to assess the degree of association between selected Quality of life (QoL) indicators and ...both clinical and imaging cardiac status indicators when detecting deterioration in QoL of these patients.
In a cohort clinical study in Hamburg, from August 2017 through October 2020, 59 cancer patients, aged 18-80 years, were evaluated before chemotherapy, and at several follow-ups, using EQ-5D and SF-36 QoL questionnaires, fast strain-encoded (fast-SENC) cardiac magnetic resonance (CMR), conventional CMR, and echocardiography, and further received a clinical and biomarker examination. Data was analyzed using survival analyses. A decline of more than 5% in each observed QoL metric value was defined as the observed event. Patient were separated into groups according to the presentation of cardiotoxicity as per its clinical definition, the establishment of the indication for cardioprotective therapy initiation, and by a worsening in the value of each observed imaging metric by more than 5% in the previous follow-up compared to the corresponding pre-chemotherapy baseline value.
Among clinical cardiac status indicators, the indication for cardioprotective therapy showed statistically good association with QoL scores (EQ-5D p=0.028; SF-36 physical component p=0.016; SF-36 mental component p=0.012). In terms of imaging metrics, the MyoHealth segmental myocardial strain score was the only one demonstrating consistently good QoL score association (EQ-5D p=0.005; SF-36 physical component p=0.056; SF-36 mental component p=0.002).
Established fast-SENC CMR scores are capable of highlighting patients with reduced QoL, who require more frequent/optimal management.
Dijagnostička kategorija mijelodisplastične/mijeloproliferativne neoplazme (MDS/MPN) obuhvaća klonske hematopoetske neoplazme koje u vrijeme postavljanja dijagnoze istodobno pokazuju klinička, ...laboratorijska i/ili morfološka obilježja i mijelodisplastičnog sindroma (MDS) i mijeloproliferativne neoplazme (MPN). Citopenija i displazija jedne ili više mijeloidnih loza (obilježja MDS-a) mogu se vidjeti istodobno s leukocitozom, trombocitozom i/ili organomegalijom (obilježja koja su češće povezana s MPN-om). Bolesnici s prije dijagnosticiranim MPN-om, kod kojih se razviju mijelodisplastične promjene kao posljedica evolucije bolesti ili kemoterapije, ne ubrajaju se u ovu dijagnostičku kategoriju. Prema klasifikaciji Svjetske zdravstvene organizacije (engl. World Health Organization – WHO) iz 2008. godine te njezinoj reviziji iz 2016. godine, MDS/MPN obuhvaća pet entiteta: kroničnu mijelomonocitnu leukemiju (engl. chronic myelomonocytic leukemia – CMML), juvenilnu mijelomonocitnu leukemiju (engl. juvenile myelomonocytic leukemia – JMML), atipičnu kroničnu mijeloičnu leukemiju, BCR-ABL1- (engl. atypical chronic myeloid leukemia – aCML), MDS/MPN s prstenastim sideroblastima i trombocitozom (engl. MDS/MPN with ring sideroblasts and thrombocytosis – MDS/MPN-RS-T) i neklasificirani MDS/MPN (engl. MDS/MPN, unclassifiable – MDS/MPN, U).
Malignant acanthosis nigricans is a rare obligate paraneoplastic dermatosis which accounts for 20% of all acanthosis nigricans cases. The clinical features of the disease are the same as in the ...benign forms: symmetrical, hyperpigmented, velvety papillomatous lesions mostly involving the axillae, neck, groins, periumbilical cubital and popliteal areas, mammary areolae and less often mucous membranes. However, unlike other forms, it is characterized by sudden onset and rapid spread, commonly (80%) after the age of 40, which may be a marker of malignancy and a key to early diagnosis, indicating the need for a detailed examination. It is a disorder that has no gender differences. Most cases are detected at the moment of cancer diagnosis (61.3%), in fewer cases (about 20%) prior to cancer diagnosis, and in 21% at a later stage of malignant disease. Acanthosis nigricans is usually associated with one of the three or all three forms of paraneoplastic lesions: florid cutaneous papillomatosis, acanthosis palmaris (tripe palms, pachydermatoglyphia) involving the palms and soles, as well as multiple seborrheic keratosis (sign of Leser-Trélat).
We report on a female patient with clinically established three paraneoplastic syndromes: malignant acanthosis nigricans, florid cutaneous papillomatosis, and acanthosis palmaris, which appeared before the diagnosis of advanced gastric adenocarcinoma, leading to fatal outcome.
Pachydermodactyly is a rare, benign form of digital fibromatosis, characterized by asymptomatic and progressive, periarticular and usually symmetrical soft tissue finger swelling, specifically on the ...lateral aspects of the proximal interphalangeal joints mainly of the second, third, and fourth fingers; it mostly affects young adolescents and is probably due to repeated mechanical injury of the skin (such as repeated clasping or rubbing of crossed fingers), sometimes as a result of obsessive-compulsive disorder, which must be distinguished from obsessive “chewing pads”. This paper presents a male patient aged 19, who presented with first symptoms at the age of 12, and was diagnosed with periarticular hypertrophy: localized soft tissue thickening around the proximal interphalangeal joints of all fingers except the thumbs; slight hypertrophy of the skin; absence of subjective complaints; normal joint function. Dermatological status on admission revealed: symmetrical soft tissue swelling of all fingers of both hands except the thumbs at the level of the proximal interphalangeal joints; normal appearance of the distal parts of all fingers; thickening at the level of the proximal interphalangeal joints, bilateral, almost symmetrical hypertrophy (ulnar and radial) of phalanges of the affected fingers except both index fingers, affecting only the ulnar side. The skin lesions were pain-free on palpation, with homogeneous texture and elastic consistency, freely movable over underlying structures. The affected joints showed no functional deficit. The test results, sonography, radiography and histopathology confirmed our clinical diagnosis-pachydermodactyly. The review of the currently available literature, published between 1973 and 2014, including 99 papers and 160 patients, provided important insight into the characteristics and variations of the disease.
Cylindromas are benign appendage tumors mainly found on the scalp, but they can occur on any hair-bearing skin. Mutations in the cylindromatosis (CYLD) gene, a tumor suppressor gene located on ...chromosome 16q12–13, are responsible for multiple cylindromas, which are usually inherited in an autosomal dominant way, as in familial cylindromatosis and Brooke-Spiegler syndrome. The latter is characterized by the presence of multiple cylindromas, multiple trichoepitheliomas and spiradenomas. Based on genetic studies and the identification of heterozygous mutations in the same CYLD gene in familial cylindromatosis, multiple familial trichoepitheliomas, and the Brooke–Spiegler syndrome, it is suggested that these three conditions have the same genetic basis and are phenotypic expressions of the same disease. The diagnosis of each of the tree conditions is based on the dominant tumor type: cylindroma in familiar cyindromas, trichoepithelioma in multiple familial trichoepitheliomas, or a variety of skin appendage tumors including cylindromas, spiradenomas and trichoepitheliomas in Brooke-Spiegler syndrome. The onset of the disease is usually in the early adulthood, but may also occur in childhood or adolescence.
We report on two sisters, 37 and 43 years of age, with multiple cylindromas on the face and the scalp. Both patients reported that their mother also had multiple tumors on her head. Dermoscopy revealed arborizing vessels on a white-ivory or pink background, resembling dermoscopic features of basal cell carcinoma, though histopathological analysis revealed cylindroma.
In conclusion, in this study we report two cases of a very rare familial cylindromatosis, presenting with multiple benign cylidromas with dermoscopic features of basal cell carcinoma. All patients with multiple cylindromas in familial cylindromatosis should be counseled about increased risk for developing further tumors. Systemic and multidisciplinary approach with follow up is strongly recommended.
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