Dermatomyositis as paraneoplastic syndrome of peritoneal and ovarian relapse after long-term complete remission in patient with metastatic bilateral breast cancer [Dermatomiozitis kao paraneoplastički sindrom peritonealnog i ovarijalnog relapsa nakon dugog perioda potpune remisije u bolesnice s metastatskim bilateralnim rakom dojke]

Dermatomyositis is a rare disease characterised by inflammatory muscle affection and characteristic cutaneous changes. When occuring in a patient with cancer, dermatomyositis may indicate recurrence or progression and poor outcome. Herein, the treatment of metastatic breast cancer, metastatic pattern, characteristics of long-term survivors, and link between dermatomyositis and breast cancer are discussed and the literature reviewed. We report a 57-year old female patient with metastatic bilateral breast cancer whose ovarian and peritoneal relapse after long-term remission was disclosed by occurence of paraneoplastic dermatomyositis. The patient previously had a 15-year long disease free-period after primary treatment for breast cancer before onset of pulmonary dissemination. Following antracycline-based chemotherapy, the complete remission lasting another 15 years was accomplished. Dermatomyositis had been resolved upon induction of second-line taxane-based chemotherapy. After completion of six cycles of gemcitabine and paclitaxel chemotherapy, check-up revealed further progression. The patient subsequently underwent six cycles of third-line CAP chemotherapy (cyclofosfamide, doxorubicine, cisplatin) but disease progressed and oral capecitabine chemotherapy was initiated. The patient received four cycles of capecitabine followed by further vast progression and finally expired following massive pulmonary embolism. Our case stresses the need of thorough staging and check-up when dermatomyositis arises in patients with breast cancer, regardless of previous stable long-term complete remission. Furthermore, we believe that treatment with curative intent in young patients with metastatic breast cancer, who have good performance statuses and no comorbidities is required, because it is more likely to produce long-term complete remission. However, following disease relapse a poor outcome can be expected

Čvorovi štitne žlijezde - stvarna epidemija ili poboljšana dijagnostika

The incidence of thyroid nodules has been rising steadily during the last 30 years, since the introduction of new diagnostic methods such as ultrasonography and computerized tomography, thus posing a real challenge in determining the best approach strategy for treatment of this new \u27epidemic\u27. We analyzed and compared data from several studies showing the prevalence of thyroid nodules on autopsy, palpation and ultrasonography to be 13%-60%, 0.5%-6.5% and 13.4%-46%, respectively. This demonstrates that thyroid ultrasonography is a very sensitive and accurate diagnostic tool the use of which, however, entails an increased number of incidentally discovered thyroid nodules without clinical significance. Therefore, ultrasonography of the thyroid should not be performed without clinical indication determined by thyroid specialist.Incidencija čvorova štitne žlijezde u stalnom je porastu posljednjih 30 godina, od uvođenja novih dijagnostičkih metoda poput ultrazvuka i kompjutorizirane tomografije, što predstavlja pravi izazov pri određivanju najboljeg pristupa u liječenju ove nove \u27epidemije\u27. Analizirali smo i usporedili podatke iz nekoliko istraživanja koja navode učestalost čvorova štitne žlijezde utvrđenih na autopsiji, palpacijom i ultrazvukom od 13%-60%, 0,5%-6,5%, odnosno 13,4%-46%. To pokazuje kako je ultrazvuk vrlo osjetljiva i točna dijagnostička metoda, međutim, njegova primjena dovodi do sve većeg broja slučajno otkrivenih, klinički neznačajnih čvorova štitnjače. Stoga ultrazvuk štitnjače ne treba primjenjivati bez kliničke indikacije koju je postavio specijalist za štitnu žlijezdu

Postoperative Use of Radioiodine (131-I): Review of Recommendations and Guidelines

In the management of large number of patients with differentiated thyroid cancer, the radioactive iodine (131-I) administration plays an important role. The guidelines of numerous international and national medical societies regarding the issue of postoperative 131-I administration have been published and updated in the last few years. The guidelines differ in the shape and content, and contain some specific features. The different methods for evaluation and analysis of clinical evidence level and resulting grades of recommendations have been used in line with the very guidelines. The postoperative 131-I administration refers to the radioiodine ablation as a form of adjuvant treatment and radioiodine therapy in the management of patients with recurrent cancer, persistent disease and regional or distant metastases. According to the indications for the postoperative 131-I administration, the patients could be divided into the three risk groups: the very low risk group in which there is no indication for the postoperative131-I administration, the low risk group in which the indication could be considered, and the high risk group in which there is a clear indication for the131-I administration. The different criteria for distribution of patients into these three groups are expressed in a certain guidelines. There are different opinions about the necessary dosage of 131-I for the efficient ablation in the low risk group. Moreover, the opinions are also divided regarding the conduction of postoperative (preablative or pretherapeutic) scintigraphy with 131-I. As regards the instructions on preparation of patients for the radioiodine ablation and therapy, all the guidelines recommend the low iodine diet and endogenous or exogenous stimulation of TSH. The endogenous stimulation is accomplished by the withdrawal of thyroid hormones, whereas the recombinant human TSH (rhTSH) is used for exogenous stimulation. For conducting the therapy with 131-I the level of TSH has to be >25–30 mU/L

Dozimetrijska verifikacija radioterapijskih planova intezitet-modulirajuće radioterapije u bolesnika s rakom prostate

Intensity modulated radiotherapy (IMRT) has become widely used as a standard radiation therapy technique for the treatment of localized prostate cancer. The transition from conformal radiotherapy (3D CRT) to a more complex IMRT technique triggered the need for more thorough verification of the accuracy in the dose delivery. In this work we present the clinical workflow and the results of patient specific quality assurance (PSQA) procedures for 40 prostate cancer patients who have been treated with step and shot IMRT ever since its implementation in our routine clinical practice. PSQA procedures include dosimetric verification of each treatment plan with dedicated rotational phantom and high-resolution matrix detector system Octavius 4D (PTW Freiburg) that allows three-dimensional comparison of the calculated and delivered radiation dose distribution. Our results proved the compliance with the universal tolerance limits recommended for those procedures (1), assuring the safety of the treatment and providing the possibility for the adoption of more stringent constraints in the future.Radioterapija moduliranog intenziteta (eng. intensity modulated radiotherapy –IMRT) u posljednjem desetljeću je postala uobičajena radioterapijska metoda za terapiju lokaliziranih karcinoma prostate. Prelazak s konformalne radioterapije na napredniju i tehnički složeniju IMRT tehniku, donio je i potrebu za detaljnijom i sveobuhvatnom provjerom točnosti isporuke doze zračenja. U ovom radu predstavljamo provođenje postupaka dozimetrijske verifikacije radioterapijskih planova poznatih pod engleskim nazivom patient specific QA (PSQA) te rezultate za 40 bolesnika s karcinomom prostate koji su primili IMRT terapiju. U tu svrhu koristimo posebni dozimetrijski sustav s rotacijskim fantomom i visoko razlučivom detektorskom matricom, Octavius 4D (PTW Freiburg). Pokazalo se kako su sva dobivena odstupanja između planirane i mjerene trodimenzionalne raspodjele doze bila unutar preporučenih tolerancija (1) što nam daje povjerenje u sigurnost provođenja ovakve terapije te otvara mogućnost za primjenu strožijih ograničenja u budućnosti

Dostatan unos joda u školske djece s područja Zagreba: procjena određivanjem tireoglobulina u suhoj kapi krvi kao novog funkcionalnog biljega nedostatnog unosa joda

Current methods for assessment of iodine intake in a population comprise measurements of urinary iodine concentration (UIC), thyroid volume by ultrasound (US-Tvol), and newborn TSH. Serum or dried blood spot thyroglobulin (DBS-Tg) is a new promising functional iodine status biomarker in children. In 1996, a new act on universal salt iodination was introduced in Croatia with 25 mg of potassium iodide per kg of salt. In 2002, Croatia finally reached iodine sufficiency. However, in 2009, median UIC in 101 schoolchildren from Zagreb, the capital of Croatia, was 288 μg/L, posing to be excessive. The aim of the study was to assess iodine intake in schoolchildren from the Zagreb area and to evaluate the value of DBS-Tg in schoolchildren as a new functional biomarker of iodine deficiency (and iodine excess). The study was part of a large international study in 6- to 12-year-old children supported by UNICEF, the Swiss Federal Institute of Technology (ETH Zurich) and the International Council for the Control of Iodine Deficiency Disorders (ICCIDD). According to international study results, the median cut-off Tg 40 µg/L indicate iodine sufficiency. The study included 159 schoolchildren (median age 9.1±1.4 years) from Zagreb and a nearby small town of Jastrebarsko with measurements of UIC, US-Tvol, DBSTg, T4, TSH and iodine content in salt from households of schoolchildren (KI/kg of salt). Overall median UIC was 205 µg/L (range 1-505 µg/L). Thyroid volumes in schoolchildren measured by US were within the normal range according to reference values. Median DBS-Tg in schoolchildren was 12.1 μg/L with 3% of Tg values >40µg/L. High Tg values were in the UIC range 300 µg/L (U-shaped curve of Tg plotted against UIC). All children were euthyroid with geometric mean TSH 0.7±0.3 mU/L and arithmetic mean T4 62±12.5 nmol/L. The mean KI content per kg of salt was 24.9±3.1 mg/kg (range 19-36 mg/kg). Study results indicated iodine sufficiency in schoolchildren from the Zagreb area. Thyroglobulin proved to be a sensitive indicator of both iodine deficiency and iodine excess in children. Iodine content in salt from households of schoolchildren was in good compliance with the Croatian act (20-30 ,mg KI/kg of salt).Metode koje se primjenjuju za procjenu unosa joda u populaciji su mjerenje izlučivanja joda mokraćom (urinary iodine concentration, UIC), procjena stanja gušavosti mjerenjem volumena štitnjače ultrazvukom (Tvol) i tireotropina (TSH) u novorođenčadi. Određivanje biljega tireoglobulina (Tg) u serumu ili suhoj kapi krvi je novi obećavajući funkcionalni biljeg stanja unosa joda u djece. Novi zakon o obveznom univerzalnom jodiranju soli uveden je u Hrvatskoj 1996. s 25 mg kalij-jodida po kilogramu soli. Hrvatska je 2002. g. postigla dostatan unos joda. Međutim, 2009. godine medijan izlučivanja joda mokraćom u školske djece iz Zagreba iznosio je 288 μg/L, što je blizu granice prekomjernog unosa joda. Cilj istraživanja bio je odrediti unos joda u školske djece s područja Zagreba i vrijednost određivanja Tg u suhoj kapi krvi u školske djece kao novog funkcionalnog biljega nedostatnog i/ili prekomjernog unosa joda. Istraživanje je dio velikog međunarnodnog projekta UNICEF-a, Švicarskog federalnog instituta tehnologije (ETH Zürich) i Međunarodnog vijeća za kontrolu poremećaja uzrokovanih nedostatkom joda (ICCIDD). Prema rezultatima međunarodnog projekta, granični prijelomni (cut-off) medijan Tg 40 µg/L upućuju na dostatan unos joda. U istraživanje je bilo uključeno 159 školske djece (medijan dobi 9,1±1,4 god.) iz Zagreba i obližnjeg gradića Jastrebarsko. Istraživanje je provedeno određivanjem UIC, Tvol, Tg u suhoj kapi krvi, T4, TSH i sadržaja joda u soli iz kućanstava školske djece (KI/kg soli). Ukupni medijan UIC iznosio je 205 µg/L (raspon 1-505 µg/L). Volumeni štitnjače u školske djece izmjereni ultrazvukom bili su unutar granica normale u usporedbi s referentnim vrijednostima. Medijan Tg u suhoj kapi krvi iznosio je u školske djece 12,1 μg/L, a 3% rezultata Tg bilo je >40 µg/L. Visoke vrijednosti Tg zabilježene su u rasponu vrijednosti UIC 300 µg/L (U-oblik krivulje Tg u odnosu na UIC). Sva djeca su bila u eutireozi s geometrijskom sredinom TSH 0,7±0,3 mU/L i aritmetičkom sredinom T4 62±12,5 nmol/L. Prosječni sadržaj kalij jodida po kg soli iznosio je 24,9±3,1 mg/kg (raspon 19-36 mg/kg). Prikazani podaci upućuju na dostatan unos joda u školske djece s područja Zagreba. Tireoglobulin je osjetljiv pokazatelj ne samo nedostatnog, nego i prekomjernog unosa joda u djece. Sadržaj joda u soli iz kućanstava školske djece bio je u skladu s hrvatskim zakonom o obveznom jodiranju soli (20-30 mg KI/kg soli)

Dostatan unos joda u školske djece s područja Zagreba: procjena određivanjem tireoglobulina u suhoj kapi krvi kao novog funkcionalnog biljega nedostatnog unosa joda

Current methods for assessment of iodine intake in a population comprise measurements of urinary iodine concentration (UIC), thyroid volume by ultrasound (US-Tvol), and newborn TSH. Serum or dried blood spot thyroglobulin (DBS-Tg) is a new promising functional iodine status biomarker in children. In 1996, a new act on universal salt iodination was introduced in Croatia with 25 mg of potassium iodide per kg of salt. In 2002, Croatia finally reached iodine sufficiency. However, in 2009, median UIC in 101 schoolchildren from Zagreb, the capital of Croatia, was 288 μg/L, posing to be excessive. The aim of the study was to assess iodine intake in schoolchildren from the Zagreb area and to evaluate the value of DBS-Tg in schoolchildren as a new functional biomarker of iodine deficiency (and iodine excess). The study was part of a large international study in 6- to 12-year-old children supported by UNICEF, the Swiss Federal Institute of Technology (ETH Zurich) and the International Council for the Control of Iodine Deficiency Disorders (ICCIDD). According to international study results, the median cut-off Tg 40 µg/L indicate iodine sufficiency. The study included 159 schoolchildren (median age 9.1±1.4 years) from Zagreb and a nearby small town of Jastrebarsko with measurements of UIC, US-Tvol, DBSTg, T4, TSH and iodine content in salt from households of schoolchildren (KI/kg of salt). Overall median UIC was 205 µg/L (range 1-505 µg/L). Thyroid volumes in schoolchildren measured by US were within the normal range according to reference values. Median DBS-Tg in schoolchildren was 12.1 μg/L with 3% of Tg values >40µg/L. High Tg values were in the UIC range 300 µg/L (U-shaped curve of Tg plotted against UIC). All children were euthyroid with geometric mean TSH 0.7±0.3 mU/L and arithmetic mean T4 62±12.5 nmol/L. The mean KI content per kg of salt was 24.9±3.1 mg/kg (range 19-36 mg/kg). Study results indicated iodine sufficiency in schoolchildren from the Zagreb area. Thyroglobulin proved to be a sensitive indicator of both iodine deficiency and iodine excess in children. Iodine content in salt from households of schoolchildren was in good compliance with the Croatian act (20-30 ,mg KI/kg of salt).Metode koje se primjenjuju za procjenu unosa joda u populaciji su mjerenje izlučivanja joda mokraćom (urinary iodine concentration, UIC), procjena stanja gušavosti mjerenjem volumena štitnjače ultrazvukom (Tvol) i tireotropina (TSH) u novorođenčadi. Određivanje biljega tireoglobulina (Tg) u serumu ili suhoj kapi krvi je novi obećavajući funkcionalni biljeg stanja unosa joda u djece. Novi zakon o obveznom univerzalnom jodiranju soli uveden je u Hrvatskoj 1996. s 25 mg kalij-jodida po kilogramu soli. Hrvatska je 2002. g. postigla dostatan unos joda. Međutim, 2009. godine medijan izlučivanja joda mokraćom u školske djece iz Zagreba iznosio je 288 μg/L, što je blizu granice prekomjernog unosa joda. Cilj istraživanja bio je odrediti unos joda u školske djece s područja Zagreba i vrijednost određivanja Tg u suhoj kapi krvi u školske djece kao novog funkcionalnog biljega nedostatnog i/ili prekomjernog unosa joda. Istraživanje je dio velikog međunarnodnog projekta UNICEF-a, Švicarskog federalnog instituta tehnologije (ETH Zürich) i Međunarodnog vijeća za kontrolu poremećaja uzrokovanih nedostatkom joda (ICCIDD). Prema rezultatima međunarodnog projekta, granični prijelomni (cut-off) medijan Tg 40 µg/L upućuju na dostatan unos joda. U istraživanje je bilo uključeno 159 školske djece (medijan dobi 9,1±1,4 god.) iz Zagreba i obližnjeg gradića Jastrebarsko. Istraživanje je provedeno određivanjem UIC, Tvol, Tg u suhoj kapi krvi, T4, TSH i sadržaja joda u soli iz kućanstava školske djece (KI/kg soli). Ukupni medijan UIC iznosio je 205 µg/L (raspon 1-505 µg/L). Volumeni štitnjače u školske djece izmjereni ultrazvukom bili su unutar granica normale u usporedbi s referentnim vrijednostima. Medijan Tg u suhoj kapi krvi iznosio je u školske djece 12,1 μg/L, a 3% rezultata Tg bilo je >40 µg/L. Visoke vrijednosti Tg zabilježene su u rasponu vrijednosti UIC 300 µg/L (U-oblik krivulje Tg u odnosu na UIC). Sva djeca su bila u eutireozi s geometrijskom sredinom TSH 0,7±0,3 mU/L i aritmetičkom sredinom T4 62±12,5 nmol/L. Prosječni sadržaj kalij jodida po kg soli iznosio je 24,9±3,1 mg/kg (raspon 19-36 mg/kg). Prikazani podaci upućuju na dostatan unos joda u školske djece s područja Zagreba. Tireoglobulin je osjetljiv pokazatelj ne samo nedostatnog, nego i prekomjernog unosa joda u djece. Sadržaj joda u soli iz kućanstava školske djece bio je u skladu s hrvatskim zakonom o obveznom jodiranju soli (20-30 mg KI/kg soli)

Dileme u radioterapiji/kemoterapiji bolesnika s metastazama u limfne čvorove na vratu tumora nepoznatoga primarnog sijela

SAŽETAK Incidencija karcinoma nepoznatoga primarnog sijela s metastazama u limfne čvorove glave i vrata je niska te prema literaturi varira između 2 i 9%, te je u daljnjem padu zbog sve preciznije dijagnostike. Histološki, najčešći su planocelularni karcinomi. Osim općega fizikalnog pregleda s osobitim usmjerenjem na područje glave i vrata, nužan je endoskopski pregled s vizualizacijom nazofarinksa, orofarinksa, larinksa i hipofarinksa te radiološka slikovna obrada (CT i/ili MR te u slučaju indikacije PET/CT). Kirurško liječenje najstarija je, no još uvijek vrlo važna metoda liječenja ovih bolesnika te je preferirana opcija inicijalnog liječenja. S druge strane, primarna radioterapija predstavlja važnu opciju liječenja za tumore glave i vrata, posebno u bolesnika koji nisu kandidati za kirurško liječenje, a kod bolesnika s uznapredovalim nalazom na vratu (N2-3) preporučuje se kombinirati je s kemoterapijom. Nakon inicijalnog kirurškog liječenja i patohistološkog nalaza N2 ili N3 bolesti, radioterapija s kemoterapijom ili bez nje indicirana je u svih bolesnika, a može se razmotriti i kod onih sa statusom N1. Neovisno radi li se o primarnoj ili adjuvantnoj radioterapiji, opseg polja zračenja i nadalje je kontroverzna tema. Opcije liječenja se kreću od operativnog liječenja ipsilateralne strane vrata bez adjuvantnog liječenja, operativnog liječenja s adjuvantnom primjenom (kemo)radioterapije do primarnog liječenja – zračenja obje strane vrata i svih mjesta potencijalnog sijela primarnog tumora. Ranije je u smjernicama zagovaran agresivniji pristup koji je uključivao radioterapiju cijelog volumena ždrijela (nazofarniks, orofarinks i hipofarinks) i grkljana te obje strane vrata. S vremenom je primjetan pomak prema poštednijem liječenju, najčešće u smislu operativnog liječenja metastaza na vratu s primjenom adjuvantne ipsilateralne radioterapije, bez zračenja čitave sluznice ždrijela i grkljana. Najvažniji parametri u odluci o primjeni konkomitantne kemoterapije jesu proboj čahure limfnog čvora i resekcija R1/2

Tireoglobulin: tumorski biljeg diferenciranih karcinoma štitnjače - klinička razmatranja

Initial treatment of the majority of patients with differentiated thyroid cancer (DTC) includes total thyroidectomy. Postoperative ablation therapy with radioactive iodine (I-131) is indicated in all high-risk patients, however, there is disagreement regarding its use in low- and intermediate-risk patients. Over the last few decades, thyroglobulin (Tg) has been established as the primary biochemical tumor marker for patients with DTC. Thyroglobulin can be measured during thyroid hormone therapy or after thyroid-stimulating hormone (TSH) stimulation, through thyroid hormone withdrawal or the use of human recombinant TSH. In many studies, the cut-off value for adequate Tg stimulation is a TSH value ≥30 mIU/L. However, there is an emerging body of evidence suggesting that this long-established standard should be re-evaluated, bringing this threshold into question. Recently, a risk stratification system of response to initial therapy (with four categories) has been introduced and Tg measurement is one of the main components. The relationship between the Tg/TSH ratio and the outcome of radioiodine ablation has also been studied, as well as clinical significance of serum thyroglobulin doubling-time. The postoperative serum Tg value is an important prognostic factor that is used to guide clinical management, and it is the most valuable tool in long term follow-up of patients with DTC.Početno liječenje većine bolesnika s diferenciranim karcinomom štitnjače uključuje kirurško odstranjenje štitnjače. Poslijeoperacijsko liječenje radioaktivnim jodom (I-131) indicirano je u većine visokorizičnih bolesnika, dok je primjena I-131 u bolesnika niskog i srednjeg rizika predmet rasprave. U prethodnih nekoliko desetljeća tireoglobulin (Tg) je prihvaćen kao primarni biokemijski tumorski biljeg visoke osjetljivosti i specifičnosti u bolesnika s diferenciranim karcinomom štitnjače. Mjerenje Tg može se izvoditi tijekom uzimanja hormonske terapije L-tiroksinom ili uz stimulaciju tireotropinom (TSH). Stimulacija putem TSH može se postići prekidom hormonske terapije L-tiroksinom ili uz primjenu rekombinantnog humanog TSH (rhTSH). Dugi niz godina se granična vrijednost TSH ≥30 mIU/L smatrala zadovoljavajućom za stimulaciju Tg, međutim, novije spoznaje osporavaju tu granicu i zahtijevaju nova istraživanja i preporuke. Nedavno je uvedena podjela bolesnika u 4 rizične skupine ovisno o odgovoru na liječenje jodom-131 nakon totalne tireoidekotmije, a mjerenje Tg jedna je od glavnih odrednica podjele. U posljednje vrijeme se značenje pridaje omjeru Tg-TSH u predviđanju ishoda ablacije jodom-131, kao i prognostičko značenje vremena udvostručenja Tg. Poslijeoperacijska serumska vrijednost Tg je važan prognostički čimbenik i odrednica daljnjeg liječenja, a određivanje Tg je osnovna metoda dugoročnog praćenja bolesnika s diferenciranim karcinomom štitnjače

Neurosifilis kod angloameričkih skladatelja i jazz glazbenika

Syphilis is a sexually transmitted, systemic disease caused by the spirochete bacterium Treponema pallidum. Th e most common mechanism of transmission is sexual intercourse. Although there are several hypotheses, the exact origin of the disease remains unknown. Newly published evidence suggests that the hypothesis supporting the theory of the American origin of the disease is the valid one. Among 1500 analyzed pathographies of composers and musicians, data on ten Anglo-American composers and jazz musicians having suff ered from neurosyphilis (tertiary stage of the disease) were extracted for this report. In this group of Anglo-American composers and musicians, most of them died from progressive paralysis while still in the creative phase of life. Additionally, diagnoses of eleven other famous neurosyphilitic composers, as well as basic biographic data on ten less known composers that died from neurosyphilis-progressive paralysis are also briefl y mentioned. In conclusion, neurosyphilis can cause serious neurological damage, as well as permanent disability or death, preventing further work and skill improvement.Sifilis je spolno prenosiva, sistemska bolest uzrokovana spirohetnom bakterijom Treponema pallidum. Najčešći način prijenosa bolesti je spolni odnos. Iako ima više hipoteza, točno podrijetlo bolesti još je uvijek nepoznato. Nedavno objavljeni podaci pokazuju da je najvjerojatnije točna hipoteza koja podupire teoriju američkog podrijetla bolesti. Među 1500 analiziranih patografi ja skladatelja i glazbenika za ovaj prikaz izdvojeni su podaci o deset angloameričkih skladatelja i jazz glazbenika koji su bolovali od neurosifi lisa (tercijarni stadij bolesti). U analiziranoj skupini angloameričkih skladatelja i glazbenika većina ih je umrla od progresivne paralize dok su još uvijek bili u kreativnoj fazi života. Dodatno, ukratko su prikazane dijagnoze jedanaestorice drugih slavnih skladatelja neurosifi litičara te također osnovni biografski podaci desetorice manje poznatih skladatelja koji su umrli od neurosifi lisa odnosno progresivne paralize. U zaključku, neurosifi lis može uzrokovati značajno neurološko oštećenje, a u težim slučajevima također dovesti do trajnog invaliditeta ili smrti i time spriječiti daljnji rad i profesionalni razvoj

Diferencirani karcinom štitnjače u pedijatrijskoj populaciji (≤18 godina): poslijeoperacijsko liječenje radioaktivnim jodom (I-131)

The aim is to present data on the treatment and follow-up in a cohort of patients with pediatric thyroid cancer who underwent total thyroidectomy and received postoperative radioactive iodine (I-131) therapy. The study was conducted in a tertiary high-volume thyroid center, in pediatric patients with differentiated thyroid cancer who were consecutively treated during the 1965-2015 period. A total of 45 patients aged ≤18 years having undergone total thyroidectomy with or without selective neck dissection were included in the study. Decision on postoperative I-131 ablation was based on tumor characteristics, postoperative thyroglobulin level, preablative whole body scintigraphy, and/or neck ultrasound. Median age at diagnosis was 15 years. The presence of cervical lymph node metastases was significantly associated with papillary thyroid cancer, larger tumor size, involvement of two thyroid lobes, and multifocal disease. The presence of distant metastases was significantly associated with larger tumor size. None of the patients died during follow-up period, and the 5-year and 10-year overall survival rates were 100%. The 5-year and 10-year progression-free survival (PFS) rates were 87% and 73%, respectively. Male gender (p=0.046), age ≤15 years (p=0.029) and tumor size >15 mm (p=0.042) were significantly associated with inferior PFS. A significant positive trend of increase in the number of newly diagnosed patients was observed over time (p=0.011). Clinical management of pediatric thyroid cancer is challenging, especially in the light of increasing incidence in this population. Male patients younger than 15 years and with tumors of more than 15 mm in size require additional caution due to lower PFS observed.Cilj je prikazati podatke o liječenju i praćenju kohorte bolesnika s pedijatrijskim rakom štitnjače, kod kojih je učinjena totalna tireoidektomija te provedena poslijeoperacijska terapija radioaktivnim jodom (I-131). Studija je provedena u tercijarnom centru za štitnjaču visokog volumena, u bolesnika s pedijatrijskim diferenciranim karcinomom štitnjače koji su liječeni konsekutivno u razdoblju od 1965. do 2015. godine. U studiju je bilo uključeno 45 bolesnika (18 godina i mlađi) kod kojih je učinjena totalna tireoidektomija sa selektivnom disekcijom vrata ili bez nje. Odluka o indikaciji za provođenje poslijeoperacijske ablacije pomoću I-131 je donesena na temelju karakteristika tumora, poslijeoperacijske razine tireoglobulina, predablacijske scintigrafije cijelog tijela i/ili ultrazvuka vrata. Medijan dobi pri postavljanju dijagnoze je iznosio 15 godina. Prisutnost metastaza u limfne čvorove vrata je bila značajno povezana s papilarnim rakom štitnjače, većom veličinom tumora, zahvaćenosti dvaju režnjeva štitnjače te multifokalnom bolešću. Prisutnost udaljenih metastaza je bila značajno povezana s većom veličinom tumora. Tijekom razdoblja praćenja nijedan bolesnik nije preminuo, a 5-godišnje i 10-godišnje stope ukupnog preživljenja su iznosile 100%. Petogodišnje i 10-godišnje preživljenje bez progresije bolesti (PPB) je iznosilo 87% odnosno 73%. Muški spol (p=0,046), dob ≤15 godina (p=0,029) i veličina tumora >15 mm (p=0,042) su bili značajno povezani s nižim PPB. Zamijećen je značajan trend porasta broja novodijagnosticiranih bolesnika tijekom vremena (p=0,011). Klinički postupak u populaciji dece s diferenciranim karcinomom štitnjače je izazovan, osobito u svjetlu porasta incidencije u ovoj populaciji. Osobitu pozornost treba posvetiti muškim bolesnicima mlađim od 15 godina s tumorima većim od 15 mm zbog opaženog nižeg PPB