scholarly journals Histologic Diagnosis and Nosologic Placement of So-called “Cerebellar Neuroblastoma”

1981 ◽  
Vol 21 (3) ◽  
pp. 295-302
Author(s):  
MASAHIRO KURISAKA ◽  
GARY S. PEARL ◽  
YOSHIO TAKEI
2016 ◽  
Vol 25 (2) ◽  
pp. 205-211 ◽  
Author(s):  
Antonio Giorgio ◽  
Luca Montesarchio ◽  
Piero Gatti ◽  
Ferdinando Amendola ◽  
Paolo Matteucci ◽  
...  

  Background & Aims: Disappearance of portal blood flow and arterial vascularization is the hallmark of hepatocarcinogenesis. The capability of a dynamic imaging modality detecting arterial hypervascularization of small nodules is crucial to promote a rapid diagnostic and therapeutic work-up improving survival. We aimed to evaluate the capability of CEUS to detect arterial vascularization of ≤ 2 cm HCC nodules arising during surveillance so as to shorten the diagnostic and therapeutic work-up. Methods: From October 2009 to September 2014, among 1757 consecutive cirrhotic patients under surveillance with ultrasound (US), 243 patients had new single nodules 7-20 mm; 229/243 had a conclusive histologic diagnosis and comprised the study group. All patients underwent CEUS followed by enhanced MRI and US guided percutaneous 18G needle core biopsy of the nodules. Of the 229 nodules, 27 were hyperechoic, 171 hypoechoic and 31 isoechoic lesions. Results: The histology results revealed that 199/229 nodules were HCC and 30 were benign. Of 199 HCC, CEUS evidenced arterial hypervascularity in 190 nodules (95.5%) (sensitivity 94.48 %, specificity 100%, PPV 100%, NPV 76.92 %). Of the 39 CEUS arterial-unenhanced nodules, 30 were benign and 9 (23%) were well-differentiated HCC. eMRI showed arterial hypervascularity in 199 nodules (86,9%). Of these, only 193 (97%) were histologically HCCs while 6 were benign (sensitivity: 97%, specificity: 80%, PPV: 97%, NPV: 80%). Conclusions: CEUS has a great capability to detect arterial hypervascularity of small HCC. Because only 4.5% of new nodules escape the demonstration of arterial hyervascularity, CEUS must be performed immediately after conventional US to contrast the malignant fate of small lesions arising in a cirrhotic liver.. Abbreviations: CEUS: contrast-enhanced ultrasound; CT: computed tomography; HCC: hepatocellular carcinoma;MRI: magnetic resonance; NPV: negative predictive value; PPV: positive predictive value; US: ultrasonography.


2021 ◽  
pp. 1-6
Author(s):  
Bita Geramizadeh ◽  
Alireza Shojazadeh

<b><i>Background:</i></b> Gastrointestinal stromal tumor (GIST) of the ampulla of Vater is a rare occurrence. To the best of our knowledge, there has been no published review on this rare tumor in the English literature so far. <b><i>Summary:</i></b> In this review, we will discuss all the reported details of the published cases, including demography, clinical presentation, imaging, gross pathology and histopathology, immunohistochemical findings, treatment modalities, and outcome of cases with the diagnosis GIST from the ampulla of Vater in the last 20 years. <b><i>Key Message:</i></b> Twenty-five cases of GIST in the ampulla of Vater have been reported in the last 20 years in the English literature. GIST in the ampulla of Vater are usually small tumors (&#x3c;5 cm) in middle-age patients. The majority of the patients present with lower GI bleeding and abdominal pain. Imaging findings are not characteristic, and most of the patients without biopsy and with no histologic diagnosis were operated with the primary impression of adenocarcinoma, neuroendocrine tumor, and GIST. Perioperative tissue biopsy has been accurate in &#x3c;70% of the cases. The majority of the reported cases of GISTs in the ampulla of Vater have been low risk with spindle-cell morphology, low mitotic figures, and minimal atypia; reactive for C-KIT and DOG-1; and nonreactive for SMA, desmin, and S100. In the majority of the cases, duodenectomy with or without Whipple’s operation has been performed, and most of the cases showed good prognosis.


Author(s):  
Vanessa Menezes ◽  
Juan Carlos Molina ◽  
Clare Pollock ◽  
Philippe Romeo ◽  
Julie Morisset ◽  
...  

Objective Transbronchial lung cryobiopsy (TBLC) is a promising technique that can provide a histologic diagnosis in interstitial lung diseases (ILD) and is an alternative to surgical lung biopsy. The main concerns with the procedure are safety and diagnostic accuracy. The technique is applicable in patients unable to undergo surgical biopsy due to severe comorbidities or when patient transport to the operating room is dangerous. This study reports the initial experience with TBLC on a thoracic surgical service as a first attempt at diagnosis in patients with diffuse parenchymal lung diseases (DPLD). Methods Between May 2018 and July 2020, 32 patients underwent TBLC using bedside flexible bronchoscopy for suspected ILD on a thoracic surgical endoscopy service. Retrospective evaluation of the procedure details, complications, and diagnostic yield were analyzed and reported. Results A total of 89 pathological samples were obtained (mean 2.8 per patient). Pneumothorax and minor bleeding occurred in 25% and 16.7% of patients, respectively. Sixty-seven percent of complications occurred with use of the 2.4 mm cryoprobe ( P = 0.036). Concordance between the histologic diagnosis and final clinical diagnosis was observed in 62.5% of patients and the pathology guided the final treatment in 71% ( P = 0.027) with Kappa-concordance of 0.60 ( P < 0.001). Conclusions Cryobiopsy is becoming part of the diagnostic evaluation in patients with indeterminate DPLD or hypoxemic respiratory failure. TBLC is easy to perform and has a favorable safety profile. Thoracic specialists should consider adding TBLC to their procedural armamentarium as a first option for patients with indeterminate PLD.


2021 ◽  
pp. 1-10
Author(s):  
Glenn M. Chertow ◽  
Gerald B. Appel ◽  
Sharon Andreoli ◽  
Sripal Bangalore ◽  
Geoffrey A. Block ◽  
...  

<b><i>Introduction:</i></b> Alport syndrome is a rare genetic disorder that affects as many as 60,000 persons in the USA and a total of 103,000 persons (&#x3c;5 per 10,000) in the European Union [1, 2]. It is the second most common inherited cause of kidney failure and is characterized by progressive loss of kidney function that often leads to end-stage kidney disease. Currently, there are no approved disease-specific agents for therapeutic use. We designed a phase 3 study (CARDINAL; NCT03019185) to evaluate the safety, tolerability, and efficacy of bardoxolone methyl in patients with Alport syndrome. <b><i>Methods:</i></b> The CARDINAL phase 3 study is an international, multicenter, double-blind, placebo-controlled, randomized registrational trial. Eligible patients were of ages 12–70 years with confirmed genetic or histologic diagnosis of Alport syndrome, eGFR 30–90 mL/min/1.73 m<sup>2</sup>, and urinary albumin to creatinine ratio (UACR) ≤3,500 mg/g. Patients with B-type natriuretic peptide values &#x3e;200 pg/mL at baseline or with significant cardiovascular histories were excluded. Patients were randomized 1:1 to bardoxolone methyl or placebo, with stratification by baseline UACR. <b><i>Results:</i></b> A total of 371 patients were screened, and 157 patients were randomly assigned to receive bardoxolone methyl (<i>n</i> = 77) or placebo (<i>n</i> = 80). The average age at screening was 39.2 years, and 23 (15%) were &#x3c;18 years of age. Of the randomized population, 146 (93%) had confirmed genetic diagnosis of Alport syndrome, and 62% of patients had X-linked mode of inheritance. Mean baseline eGFR was 62.7 mL/min/1.73 m<sup>2</sup>, and the geometric mean UACR was 141.0 mg/g. The average annual rate of eGFR decline prior to enrollment in the study was −4.9 mL/min/1.73 m<sup>2</sup> despite 78% of the patient population receiving ACE inhibitor (ACEi) or ARB therapy. <b><i>Discussion/Conclusion:</i></b> CARDINAL is one of the largest interventional, randomized controlled trials in Alport syndrome conducted to date. Despite the use of ACEi or ARB, patients were experiencing significant loss of kidney function prior to study entry.


2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii372-iii372
Author(s):  
Margot A Lazow ◽  
Austin Schafer ◽  
Mariko D DeWire-Schottmiller ◽  
Adam Lane ◽  
Daniel R Boué ◽  
...  

Abstract INTRODUCTION Recent discoveries have provided valuable insight into the genomic landscape of pediatric low grade gliomas (LGGs) at diagnosis, facilitating molecularly targeted treatment. However, little is known about their temporal and therapy-related genomic heterogeneity. An adequate understanding of the evolution of pediatric LGGs’ genomic profiles over time is critically important in guiding decisions about targeted therapeutics and diagnostic biopsy at recurrence. METHODS Fluorescence in situ hybridization, mutation-specific immunohistochemistry, and exome analyses were performed on paired tumor samples from primary diagnostic and subsequent surgeries. RESULTS 101 tumor samples from 48 patients (43 with 2 specimens, 5 with 3 specimens) from 3 institutions underwent testing. BRAF fusion and BRAFV600E status were conserved in 100% and 97% of paired specimens, respectively. No loss or gain of IDH1 mutations or FGFR1, NTRK2, MYB, or MYBL1 rearrangements were detected over time. Histologic diagnosis remained the same in all tumors, with no acquired H3K27M mutations or malignant transformation. CDKN2A deletions were acquired in 7 patients (including 3 who received chemotherapy [2 with temozolomide] and 1 who received radiation), and were associated with a trend toward shorter time to progression (median: 5.5 vs. 13.0 months [p=0.08]). CONCLUSIONS Most targetable genetic alterations in pediatric LGGs, including BRAF alterations, are conserved at recurrence and following chemotherapy or radiation. However, CDKN2A deletion acquisition was demonstrated and may define a higher risk group. Given potential for targeted therapies for tumors acquiring CDKN2A deletions, performing a biopsy at recurrence may be indicated in certain patients, especially those with rapid progression.


Cardiology ◽  
1971 ◽  
Vol 56 (1-6) ◽  
pp. 327-332 ◽  
Author(s):  
B. Bouchardy ◽  
G. Majno

1994 ◽  
Vol 4 (5) ◽  
pp. 348-351 ◽  
Author(s):  
R. Chenoy ◽  
S. Manohar ◽  
C. W.E. Redman ◽  
D. M. Luesley

Colposcopic assessment may be normal in the presence of severe or persistent minor cytologic abnormality. To assess the significance of negative satisfactory colposcopy in patients with abnormal cervical smears, a retrospective review was carried out on 1170 patients who had undergone out-patient loop diathermy excision for abnormal cervical cytology. Of these, 69 patients were treated for abnormal cervical cytology, despite normal colposcopic findings. Cytologic abnormalities ranged from persistent borderline changes to severe dyskariosis. Histologic assessment of the excision specimens revealed cervical intraepithalial neoplasia (CIN) in 43 (62.3%) cases, of which high-grade CIN accounted for 24 (34.8%) cases. There was good correlation between cytologic and histologic diagnosis. Simple regression analysis showedr= 0.46,P< 0.0001. The cytologic abnormality was highly predictive of the corresponding histologic diagnosis. This analysis has shown that significant intraepithelial lesions may exist despite negative colposcopic examination and highlights the need for histologic evaluation in such cases. In these circumstances, loop cone biopsy permits accurate definition of lesion severity, avoids potential undertreatment of significant lesions and causes less morbidity than conventional cone biopsy.


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