Intrahepatična cistična bolest s kongenitalnom fibrozom (Carolijev kompleks) - prikaz slučaja i pregled literature

A female patient affected by Caroli\u27s disease with congenital fibrosis (Caroli\u27s complex), aged 27 years, is described. Caroli\u27s disease had been asymptomatic to the present. It was recognized as an intraoperative finding during the left hepatectomy procedure after an acute abdominal crisis episode. The main reason for this surgery was the incidence of malignant transformation to cholangiocarcinoma of the cells of the cystic walls. The complex Caroli\u27s disease is more common than other forms. The case report is supplemented with literature review and discussion on the etiopathogenetic mechanisms hypothesized.Opisan je slučaj 27-godišnje bolesnice s Carolijevom bolešću i kongenitalnom fibrozom (Carolijev kompleks). Carolijeva bolest dotad je bila asimptomatska. Prepoznata je kao intraoperacijski nalaz tijekom postupka lijevostrane hepatektomije nakon akutne epizode abdominalne krize. Glavni razlog za operacijski zahvat bila je maligna pretvorba u kolangiokarcinom stanica cističnih stjenka. Carolijev kompleks češći je od drugih oblika bolesti. Prikaz slučaja je dopunjen pregledom literature i raspravom o pretpostavljenim etiopatogenetskim mehanizmima

Prevlast CD4 pozitivnih stromalnih stanica koštane srži u bolesnika s ranim stupnjem Hodgkinove bolesti miješane celularnosti

The aim of the study was to determine the possible bone marrow involvement in patients with early stages of classic Hodgkin\u27s disease mixed cellularity variant diagnosed by lymph node biopsy at initial presentation not responding to radiotherapy alone. The study cohort consisted of 20 patients (18 displaying B-cell genotype and two T-cell genotype) with stages I-II Hodgkin\u27s disease according to Ann Arbor classification treated with radiotherapy alone, seven of them not responding to therapy. Southern blot hybridization using a specific EBV Bam H1W fragment probe showed the presence of EBV genomes in two patients. All 20 patients underwent iliac crest trephine biopsy and a panel of antibodies including CD45, CD20, CD4, CD8, CD45RO, CD56, CD30, ALK-1, CD-15, EMA, CD61, and CD68 were performed. A statistically significant predominance of CD45, CD45RO and CD4 positive stromal cells was found in seven patients that failed to respond to therapy (c2-test: p=0.021, p=0.019 and p=0.015, respectively). The predominance of CD4 positive cells in the bone marrow stroma might be suggestive of involvement by Hodgkin\u27s disease in the early stage (I-II) patients (indicating upstaging) who fail to show remission on radiotherapy alone, and could explain the abnormal cytokine production, which may contribute to diminished T-cell immunity and inefficient antitumor responses despite a vast majority of infiltrating reactive immune cells.Cilj rada bio je utvrditi moguću zahvaćenost koštane srži u bolesnika s ranim stadijima klasične Hodgkinove bolesti, varijanta miješane celularnosti, dijagnosticirane biopsijom limfnog čvora, koji pri prvom dolasku nisu odgovorili na samu radioterapiju. Bolesnička skupina sastojala se je od 20 bolesnika (18 s B-staničnim genotipom i dvoje s T-staničnim genotipom) u I.-II. stadiju prema klasifikaciji iz Ann Arbora, koji su bili liječeni samo radioterapijom. Sedmoro bolesnika nije odgovaralo na ovu vrst liječenja. Southern blot hibridizacija uz primjenu specifične fragmentne sonde EBV Bam H1W pokazala je prisutnost EBV genoma u dvoje bolesnika. Svih 20 bolesnika podvrgnuto je trepanacijskoj biopsiji ilijačnog grebena i provedeno je ispitivanje panelom protutijela uključujući CD45, CD20, CD4, CD8, CD45RO, CD56, CD30, ALK-1, CD-15, EMA, CD61 i CD68. U sedmoro bolesnika koji nisu odgovarali na liječenje utvrđena je statistički značajna prevlast stromalnih stanica pozitivnih na CD45, CD45RO i CD4 (c2-test: p=0,021, p=0,019 odnosno p=0,015). Prevlast stanica pozitivnih na CD4 u stromi koštane srži moglo bi ukazivati na zahvaćenost koštane srži Hodgkinovom bolešću u bolesnika s ranim stadijem (I.-II.) (tj. porast stadija) u kojih ne dolazi do remisije bolesti nakon same radioterapije, te bi moglo objasniti nenormalnu proizvodnju citokina, što možda doprinosi smanjenoj T-imunosti i nedostatnom protutumorskom odgovoru usprkos goleme većine infiltriranih reaktivnih imunih stanica

Prevlast CD4 pozitivnih stromalnih stanica koštane srži u bolesnika s ranim stupnjem Hodgkinove bolesti miješane celularnosti

The aim of the study was to determine the possible bone marrow involvement in patients with early stages of classic Hodgkin\u27s disease mixed cellularity variant diagnosed by lymph node biopsy at initial presentation not responding to radiotherapy alone. The study cohort consisted of 20 patients (18 displaying B-cell genotype and two T-cell genotype) with stages I-II Hodgkin\u27s disease according to Ann Arbor classification treated with radiotherapy alone, seven of them not responding to therapy. Southern blot hybridization using a specific EBV Bam H1W fragment probe showed the presence of EBV genomes in two patients. All 20 patients underwent iliac crest trephine biopsy and a panel of antibodies including CD45, CD20, CD4, CD8, CD45RO, CD56, CD30, ALK-1, CD-15, EMA, CD61, and CD68 were performed. A statistically significant predominance of CD45, CD45RO and CD4 positive stromal cells was found in seven patients that failed to respond to therapy (c2-test: p=0.021, p=0.019 and p=0.015, respectively). The predominance of CD4 positive cells in the bone marrow stroma might be suggestive of involvement by Hodgkin\u27s disease in the early stage (I-II) patients (indicating upstaging) who fail to show remission on radiotherapy alone, and could explain the abnormal cytokine production, which may contribute to diminished T-cell immunity and inefficient antitumor responses despite a vast majority of infiltrating reactive immune cells.Cilj rada bio je utvrditi moguću zahvaćenost koštane srži u bolesnika s ranim stadijima klasične Hodgkinove bolesti, varijanta miješane celularnosti, dijagnosticirane biopsijom limfnog čvora, koji pri prvom dolasku nisu odgovorili na samu radioterapiju. Bolesnička skupina sastojala se je od 20 bolesnika (18 s B-staničnim genotipom i dvoje s T-staničnim genotipom) u I.-II. stadiju prema klasifikaciji iz Ann Arbora, koji su bili liječeni samo radioterapijom. Sedmoro bolesnika nije odgovaralo na ovu vrst liječenja. Southern blot hibridizacija uz primjenu specifične fragmentne sonde EBV Bam H1W pokazala je prisutnost EBV genoma u dvoje bolesnika. Svih 20 bolesnika podvrgnuto je trepanacijskoj biopsiji ilijačnog grebena i provedeno je ispitivanje panelom protutijela uključujući CD45, CD20, CD4, CD8, CD45RO, CD56, CD30, ALK-1, CD-15, EMA, CD61 i CD68. U sedmoro bolesnika koji nisu odgovarali na liječenje utvrđena je statistički značajna prevlast stromalnih stanica pozitivnih na CD45, CD45RO i CD4 (c2-test: p=0,021, p=0,019 odnosno p=0,015). Prevlast stanica pozitivnih na CD4 u stromi koštane srži moglo bi ukazivati na zahvaćenost koštane srži Hodgkinovom bolešću u bolesnika s ranim stadijem (I.-II.) (tj. porast stadija) u kojih ne dolazi do remisije bolesti nakon same radioterapije, te bi moglo objasniti nenormalnu proizvodnju citokina, što možda doprinosi smanjenoj T-imunosti i nedostatnom protutumorskom odgovoru usprkos goleme većine infiltriranih reaktivnih imunih stanica

Otkrivanje delecije kromosoma 1 pomoću tehnike FISH na otiscima ependimoma ukazuje na regiju izvan kromosoma 22 važnu za recidiv tumora

Ependymomas are glial tumors. They constitute approximately 5%-10% of intracranial tumors. Ependymomas are tumors which can recur. Predictive factors of outcome in ependymomas are not well established. Karyotypic studies are relatively scarce and loss of chromosome 22 has been described to correlate with recurrence. We are unaware of any reports involving chromosome 1 aberrations in the malignant progression of ependymomas. Cytogenetic analysis of 4 ependymomas was performed using double-target fluorescent in situ hybridization (FISH) and focusing on chromosomes 1 and 22. One patient had recurrent tumor. FISH was performed on 500 nuclei/tumors. All four cases showed a loss of chromosome 22q, while only one showed an additional loss of chromosome 1p, and it was the one with tumor relapse. We support the presence of tumor suppressor gene on 1p associated with relapse in ependymomas and suggest that the chromosome 1p status by FISH may identify a high risk group of patients harboring this tumor. Additional studies in this direction are needed, as our results refer to a minimal number of individuals analyzed.Ependimomi su glialni tumori. Oni čine otprilike 5%-10% intrakranijskih tumora. Ependimomi su tumori koji se mogu ponavljati. Čimbenici koji bi ukazivali na ishod ependimoma nisu dobro utvrđeni. Kariotipska ispitivanja su relativno rijetka, a opisano je kako gubitak kromosoma 22 korelira s ponovnom pojavom tumora. Nisu nam poznata izvješća o upletenosti aberacija kromosoma 1 u malignoj progresiji ependimoma. U ovoj studiji je citogenetska analiza 4 ependimoma provedena pomoću dvociljne fluorescentne in situ hibridizacije (FISH) i fokusiranja na kromosomima 1 i 22. U jednog se bolesnika radilo o ponovnoj pojavi tumora. FISH je izvedena na 500 jezgara/tumora. Sva četiri slučaja pokazala su gubitak kromosoma 22q, dok je samo jedan, i to onaj s ponovnim razvojem tumora, pokazao dodatni gubitak kromosoma 1p. Priklanjamo se mišljenju kako je prisutnost gena tumorske supresije na 1p udru žena s recidivom kod ependimoma i ukazujemo na to da bi status kromosoma 1p utvrđen pomoću FISH mogao identificirati visoko rizičnu skupinu bolesnika koji nose ovaj tumor. Potrebne su daljnje studije u ovom smjeru, jer se naši rezultati odnose na mali broj analiziranih osoba

Intrahepatična cistična bolest s kongenitalnom fibrozom (Carolijev kompleks) - prikaz slučaja i pregled literature

A female patient affected by Caroli\u27s disease with congenital fibrosis (Caroli\u27s complex), aged 27 years, is described. Caroli\u27s disease had been asymptomatic to the present. It was recognized as an intraoperative finding during the left hepatectomy procedure after an acute abdominal crisis episode. The main reason for this surgery was the incidence of malignant transformation to cholangiocarcinoma of the cells of the cystic walls. The complex Caroli\u27s disease is more common than other forms. The case report is supplemented with literature review and discussion on the etiopathogenetic mechanisms hypothesized.Opisan je slučaj 27-godišnje bolesnice s Carolijevom bolešću i kongenitalnom fibrozom (Carolijev kompleks). Carolijeva bolest dotad je bila asimptomatska. Prepoznata je kao intraoperacijski nalaz tijekom postupka lijevostrane hepatektomije nakon akutne epizode abdominalne krize. Glavni razlog za operacijski zahvat bila je maligna pretvorba u kolangiokarcinom stanica cističnih stjenka. Carolijev kompleks češći je od drugih oblika bolesti. Prikaz slučaja je dopunjen pregledom literature i raspravom o pretpostavljenim etiopatogenetskim mehanizmima

Otkrivanje delecije kromosoma 1 pomoću tehnike FISH na otiscima ependimoma ukazuje na regiju izvan kromosoma 22 važnu za recidiv tumora

Ependymomas are glial tumors. They constitute approximately 5%-10% of intracranial tumors. Ependymomas are tumors which can recur. Predictive factors of outcome in ependymomas are not well established. Karyotypic studies are relatively scarce and loss of chromosome 22 has been described to correlate with recurrence. We are unaware of any reports involving chromosome 1 aberrations in the malignant progression of ependymomas. Cytogenetic analysis of 4 ependymomas was performed using double-target fluorescent in situ hybridization (FISH) and focusing on chromosomes 1 and 22. One patient had recurrent tumor. FISH was performed on 500 nuclei/tumors. All four cases showed a loss of chromosome 22q, while only one showed an additional loss of chromosome 1p, and it was the one with tumor relapse. We support the presence of tumor suppressor gene on 1p associated with relapse in ependymomas and suggest that the chromosome 1p status by FISH may identify a high risk group of patients harboring this tumor. Additional studies in this direction are needed, as our results refer to a minimal number of individuals analyzed.Ependimomi su glialni tumori. Oni čine otprilike 5%-10% intrakranijskih tumora. Ependimomi su tumori koji se mogu ponavljati. Čimbenici koji bi ukazivali na ishod ependimoma nisu dobro utvrđeni. Kariotipska ispitivanja su relativno rijetka, a opisano je kako gubitak kromosoma 22 korelira s ponovnom pojavom tumora. Nisu nam poznata izvješća o upletenosti aberacija kromosoma 1 u malignoj progresiji ependimoma. U ovoj studiji je citogenetska analiza 4 ependimoma provedena pomoću dvociljne fluorescentne in situ hibridizacije (FISH) i fokusiranja na kromosomima 1 i 22. U jednog se bolesnika radilo o ponovnoj pojavi tumora. FISH je izvedena na 500 jezgara/tumora. Sva četiri slučaja pokazala su gubitak kromosoma 22q, dok je samo jedan, i to onaj s ponovnim razvojem tumora, pokazao dodatni gubitak kromosoma 1p. Priklanjamo se mišljenju kako je prisutnost gena tumorske supresije na 1p udru žena s recidivom kod ependimoma i ukazujemo na to da bi status kromosoma 1p utvrđen pomoću FISH mogao identificirati visoko rizičnu skupinu bolesnika koji nose ovaj tumor. Potrebne su daljnje studije u ovom smjeru, jer se naši rezultati odnose na mali broj analiziranih osoba

Cytologic Differential Diagnostic Problems in Ulcerative Cervicitis

Objective: To improve the detection rates in the determination of signs consistent with ulcerative cervicitis against signs of intraepithelial neoplasia, in PAP smears. Study design: We investigated the frequency of establishing a diagnosis of a simple ulcerative cervicitis by histology in a cohort of 58 females with cervical cytology suggestive of intraepithelial neoplasia. Results: There was found a detection rate of 81.58% in the determination of ulcerative cervicitis by cytology and a rate of 100% in the determination of HPV-infection, CIN1, CIN2 and CIN3 respectively. There was no statistically significant difference between cytology and histology in the diagnosis of ultracerative cervicitis (p>0.5, chi-square). Conclusions: There is an overestimation of the cytological signs in the diagnosis of ulcerative cervicitis 18.42 %). There is also an excellent agreement in the diagnosis of HPV-infection and CIN grades (100 %) between cytology and histology, discordant with the literature (2.4 %–71 %)

Pancreatic-Polypeptide in the Human Pancreas: Expression and Quantitative Variation During Development and in Ductal Adenocarcinoma

Aim: To determine the immunoreactivity of pancreatic-polypeptide (PP) during the development of the human fetal pancreas and ductal pancreatic adenocarcinoma, given that, PP positive cells were demonstrated either into its embryonic anlage or into pancreatic cancer. Methods: Tissue sections from 15 pancreatic fetal specimens, and equal number of ductal adenocarcinoma specimens, were assessed. Results: The density of positive cells in the primitive exocrine ductal epithelium and endocrine epithelium was significantly higher than the relevant density in the neoplastic pancreatic tissue of mixed (ductal – endocrine) and pure ductal type (p1=0.001, p2<0.0005, p3 =0.046 and p4<0.0005 respectively). The above values were estimated during the 10th to 12th week. There was no significant difference in the density of positive cells in the mantle zone of the islets from the 13th to the 24th week, and the neoplastic tissue of mixed (p5=0.11) and pure ductal type (p6=0.23). Conclusion: The immunostaining for PP identifies a subgroup of pancreatic ductal adenocarcinomas with a neuroendocrine component, initially considered as pure ductal tumors, and mixed ductal and neuroendocrine tumors. This pattern of expression in neoplasms recapitulates the normal pattern during the embryonal development of the organ, raising the question of therapeutic efficacy of PP and analogues as potential adjuvant treatment of pancreatic cancer

Malignant Mucoepidermoid Tumor Arising in the Accessory Parotid Gland: A Case Report

Purpose: The head and neck surgeon’s fascination with parotid surgery arises from the gland’s spectrum of histopathological presentations, as well as the diversity of its morphological features. A mass arising in the mid-cheek region may often be overlooked as a rare accessory lobe parotid neoplasm. This report serves to revisit the topic of accessory parotid gland neoplasms to emphasize proper management, particularly the surgical aspects, so that consequences of salivary fistula, facial nerve paralysis, and recurrence are avoided. Case report: We report a case of mucoepidermoid carcinoma which was assessed pre-operatively as arising from the accessory parotid gland of a 11-year-old female. She had complained of a painless and round mass of the left cheek for a duration of 12 months. Sialography, ultrasonography, CT scan and MRI were performed preoperatively. Sialography revealed a small duct separating from the Stensen’s duct. CT and MRI showed that the tumor with smooth outline was lying on the masseter muscle and detached from the main parotid gland. The preoperative diagnosis was an accessory parotid gland tumor. The tumor was removed without facial nerve injury via standard parotidectomy incision. The tumor was composed of mucous, intermediate and epidermoid cells. The pathological diagnosis was low-grade mucoepidermoid carcinoma. Conclusions: Accessory parotid gland neoplasms are rare and may present as innocuous extraparotid mid-cheek masses. A high index of suspicion, prudent diagnostic skills (including fine-needle aspiration [FNA] biopsy followed by computed tomography [CT] imaging), and scrupulous surgical approach (extended parotidectomy-style incision and limited peripheral nerve dissection when possible) are the keys to successful management of these lesions