Radiological Regression Research Articles

Significance of longitudinal Epstein–Barr virus DNA combined with multipoint tumor response for dynamic risk stratification and treatment adaptation in nasopharyngeal carcinoma

Dynamic therapy response is strongly associated with cancer outcomes. This study aimed to evaluate the significance of longitudinal Epstein–Barr virus (EBV) DNA and radiological tumor regression in risk stratification and response-adaptive treatment in locally-advanced nasopharyngeal carcinoma (LA-NPC). In total, 1312 patients from two centers were assigned to the training and validation cohorts. Based on the multipoint examination of EBV-DNA and tumor response, four post-induction chemotherapy, four mid-radiotherapy, and four post-radiotherapy subgroups were established. Then seven phenotypes were further generated according to different permutations and combinations. These phenotypes were subsequently congregated into four response clusters, which reflect distinct biological treatment responses. The four response clusters correlated with an evident 5-year progression-free survival in both the training and external validation cohorts (5-year: training cohort 91.1 %, 82.8 %, 30.6 %, and 10.0 %; external validation 94.4 %, 55.6 %, 40.0 %, and 12.7 %) had superior prognostic performance compared to TNM staging and nomogram model (concordance index: training cohort—0.825 vs. 0.603 vs. 0.756 and external validation—0.834 vs. 0.606 vs. 0.789). Importantly, the response clusters exhibited an excellent capability in selecting candidates who can benefit from adjuvant chemotherapy. In conclusion, risk stratification based on the dynamic assessment of both radiological and biological responses can significantly enhance prognostic insights and shed light on individualized treatment modifications in LA-NPCs.

Thunderclap headache as a first manifestation of acute disseminated encephalomyelitis: case report and literature review

BackgroundAcute Disseminated Encephalomyelitis (ADEM) is an acute demyelinating disorder of the central nervous system, characterize by multiple white matter hyperintensities on T2 MRI. Patients usually present with subacute progressive encephalopathy and polyfocal neurological deficits. Possible treatments are corticosteroids, immunoglobulins and plasma exchange. Full clinical recovery is seen in more than half of the cases.CaseWe describe a case of a 62-year-old patient presenting with thunderclap headache as the first symptom, two weeks after an upper respiratory tract infection. The clinical course was complicated by progressive coma and intracranial hypertension mandating external ventricular drainage and sedation. Initial treatment with methylprednisolone was unsuccessful but clinical resolution and radiological regression was achieved after plasma exchanges and cyclophosphamide.ConclusionTo our knowledge, this is the first reported case of ADEM presenting with thunderclap headache. Intracranial hypertension with the need for invasive neuromonitoring and pressure management is also a very rare complication of ADEM. In this report, we describe the findings of the literature review concerning ADEM, thunderclap headache and intracranial hypertension.

Regression of Chiari malformation type 2 following early postnatal meningomyelocele repair-a retrospective observation from an institutional series of patients.

Spontaneous regression of Chiari malformation type 2 (CM2) is observed rarely, as CM2 is associated with meningomyelocele (MMC) that issurgically repaired either pre- or early postnatally. While the radiological regression of CM2 occurs frequently following prenatal repair of MMC, it has been reported in only a few studies after postnatal repair. From the consecutive series of children with postnatally repaired MMC, we reviewed the clinical and radiological data regarding CM2, particularly its regression either spontaneously or following CSF diversion. Eighteen children underwent postnatal repair of MMC between February 2011 and April 2023. CM2 was present in 16 (89%), and hydrocephalus in 15 children (83%), requiring shunting in 14 of them. During the mean clinical observation time (from birth to April 2023) of 59 ± 51months, three children with CM2 (19%) underwent 1-2 foramen magnum decompressions (FMD), five children (28%) 1-4 surgical untethering procedures and 13 children with shunted hydrocephalus (93%) 1-5shunt revisions. Out of sixteen children with CM2, we observed regression of CM2 on MRI in only one case (6%) during the mean radiological follow-up (from birth to the last MRI taken) of 49 ± 51months. In our experience, spontaneous regression of CM2 in children with postnatally repaired MMC occurs quite rarely. Pathophysiological mechanisms behind the development of CM2 in children with MMC remain unclear, but our observation supports the hypothesis of an association between the downward displacement of the hindbrain and the low intraspinal pressure secondary to CSF leakage in children born with MMC.

A rare case of lipoid pneumonia due to diesel aspiration

Acute exogenous lipoid pneumonia is a rare chemical pneumonia following aspiration of volatile hydrocarbon compounds. In this case report, we present a 63-year-old male patient who developed acute exogenous lipoid pneumonia following accidental aspiration of diesel oil from the fuel tank of his vehicle with a hose. The patient presented with sore throat, cough, dyspnoea and tachypnoea. Posteroanterior chest radiography showed paracardiac infiltrations in the right lower and left lower lung fields.The patient presented again on the 21st day after aspiration with cough and sputum production. Chest X-ray showed a cavitary lesion with air-fluid level in the lower right probe. One month after the treatment, radiological regression of the consolidations was observed in the chest radiograph. In conclusion, this rare disease occurs after inhalation of hydrocarbon products. Symptoms and radiological findings develop rapidly and may lead to serious pulmonary complications and sequelae.

PD-1 blockade enhances the effect of targeted chemotherapy on locally advanced pMMR/MSS colorectal cancer.

Patients with DNA mismatch repair-proficient/microsatellite stable (pMMR/MSS) colorectal cancer (CRC), which accounts for 85% of all CRC cases, display a poor respond to immune checkpoint inhibitors (i.e., anti-PD-1 antibodies). pMMR/MSS CRC patients with locally advanced cancers need effective combined therapies. In this pilot study, we administered six preoperative doses of each 2-week cycle of the anti-PD-1 antibody sintilimab (at a fixed dose of 200 mg), oxaliplatin, and 5-FU/CF (mFOLFOX6) combined with five doses of bevacizumab (the number of doses was reduced to prevent surgical delays) to patients with cT4NxM0 colon or upper rectal cancers. And radical surgery was performed approximately 2 weeks after the last dose of neoadjuvant therapy. The primary endpoint was a pathologic complete response (pCR). We also evaluated major pathologic response (MPR, ≤10% residual viable tumor), radiological and pathological regression, safety, and tumor mutation burden (TMB), and tumor microenvironment (TME) characteristics. By the cutoff date (September 2023), 22 patients with cT4NxM0 pMMR/MSS colon or upper rectal cancers were enrolled and the median follow-up was 24.7 months (IQR: 21.1-26.1). All patients underwent R0 surgical resection without treatment-related surgical delays. pCR occurred in 12 of 22 resected tumors (54.5%) and MPR occurred in 18 of 22 (81.8%) patients. At the cutoff date, all patients were alive, and 21/22 were recurrence-free. Treatment-related adverse events of grade 3 or higher occurred in of 2/22 (9.1%) patients. Among the pCR tumors, two were found to harbor POLE mutations. The degree of pathological regression was significantly greater than that of radiological regression (p = 1.35 × 10-8). The number of CD3+/CD4+ cells in the tumor and stroma in pretreated biopsied tissues was markedly lower in pCR tumors than in non-pCR tumors (p = 0.038 and p = 0.015, respectively). Neoadjuvant sintilimab combined with bevacizumab and mFOLFOX6 was associated with few side effects, did not delay surgery, and led to pCR and non-pCR in 54.5% and 81.8% of the cases, respectively. Downregulation of CD3/CD4 expression in the tumor and stroma is related to pCR. However, the molecular mechanisms underlying PD-1 blockade-enhanced targeted chemotherapy require further investigation.

Results of omalizumab treatment in chronic eosinophilic pneumonia: Real-life data.

Recurrences occur when corticosteroid therapy is discontinued or reduced during the treatment of chronic eosinophilic pneumonia (CEP). The probability of recurrence is once in 50% of patients and twice or more in 25%. In such instances, new treatment options are deemed necessary. This study aims to assess the efficacy of omalizumab treatment as a steroid-sparing drug in patients with CEP. The clinical features of patients treated with omalizumab for recurrent CEP were evaluated retrospectively before and after treatment. All data from patients and diagnoses were reviewed. The effects of treatment on recurrence rate, oral corticosteroid (OCS) use and lung functions, peripheral eosinophil values, and symptom scores were evaluated. Radiological regression was also evaluated. In the final analysis, we included ten patients with a median follow-up of 22 months after initiation of omalizumab. During this follow-up period, the results were associated with a significant reduction in the number of asthma attacks per year, the number of CEP relapses, the rate of hospitalization, the amount of corticosteroids consumed daily, and the total corticosteroid dose. In addition, improvement was observed in the symptom scores and lung functions of the patients. Systemic steroids were completely discontinued in two patients receiving omalizumab treatment. In other patients, the mean steroid dose was reduced by 77.2 percent in the first year of omalizumab treatment and 82 percent in the second year, respectively. Nevertheless, there was no elevation in peripheral eosinophil count, and radiological regression was observed. Omalizumab can be an effective treatment for CEP and can be used as a steroid-sparing agent.

Neoadjuvant Chemotherapy Alone or Combined with EGFR-Directed Targeted Therapy or Anti-PD-1 Immunotherapy for Locally Advanced Lacrimal Sac and Nasolacrimal Duct Carcinomas

ABSTRACT Background/aims We describe our findings in patients with locally advanced lacrimal sac and nasolacrimal duct (NLD) carcinoma who received neoadjuvant systemic therapy. Methods We identified patients with locally advanced primary lacrimal sac/NLD carcinoma treated with neoadjuvant systemic intravenous therapy at our institution during 2017–2019. Results The study included seven patients, four men and three women; the mean age was 60.4 years (range: 43–76). All patients had locally advanced disease with significant orbital soft tissue invasion with or without skull base invasion making eye-sparing surgery not feasible as an initial step. Three patients had poorly differentiated squamous cell carcinoma; two, invasive carcinoma with basaloid and squamous features; one, high-grade carcinoma with features suggestive of sebaceous differentiation; and one, undifferentiated carcinoma. The neoadjuvant regimens were cisplatin and docetaxel (n = 1); carboplatin and docetaxel (n = 1); paclitaxel and cetuximab (n = 1); carboplatin, paclitaxel, and cetuximab (EGFR inhibitor) (n = 2); cisplatin, docetaxel, and pembrolizumab (anti-PD-1 immunotherapy) (n = 1); and carboplatin, paclitaxel, and pembrolizumab (n = 1). All patients had radiologic disease regression, and one patient had radiologic near-complete response. After neoadjuvant therapy, all patients underwent wide local excision and adjuvant concurrent chemoradiation. Two patients had a complete pathologic response. At a median follow-up period of 13 months after chemoradiation (range, 8–54 months), all patients were alive without evidence of disease. One patient had nodal metastasis treated with lymph node dissection and adjuvant chemoradiation. Conclusions Neoadjuvant systemic therapy can shrink tumors in patients with locally advanced primary lacrimal sac/NLD carcinoma with orbital or skull base invasion.

Initial surveillance in men with marker negative clinical stage IIA non-seminomatous germ cell tumours.

To assess whether extended surveillance with repeated computed tomography (CT) scans for patients with clinical stage IIA (CS IIA; <2 cm abdominal node involvement) and negative markers (Mk-) non-seminomatous germ cell tumours (NSGCTs) can identify those with true CS I. To assess the rate of benign lymph nodes, teratoma, and viable cancer in retroperitoneal lymph node dissection (RPLND) histopathology for patients with CS IIA Mk- NSGCT. Observational prospective population-based study of patients diagnosed 2008-2019 with CS IIA Mk- NSGCT in the Swedish and Norwegian Testicular Cancer Group (SWENOTECA) registry. Patients were managed with surveillance, with CT scans, and tumour markers every sixth week for a maximum of 18 weeks. Patients with radiological regression were treated as CS I, if progression with chemotherapy, and remaining CS IIA Mk- disease with RPLND. The end-point was the number and percentage of patients down-staged to CS I on surveillance and rate of RPLND histopathology presented as benign, teratoma, or viable cancer. Overall, 126 patients with CS IIA Mk- NSGCT were included but 41 received therapy upfront. After surveillance for a median (range) of 6 (6-18) weeks, 23/85 (27%) patients were in true CS I and four (5%) progressed. Of the remaining 58 patients with lasting CS IIA Mk- NSGCT, 16 received chemotherapy and 42 underwent RPLND. The RPLND histopathology revealed benign lymph nodes in 11 (26%), teratoma in two (6%), and viable cancer in 29 (70%) patients. Surveillance with repeated CT scans can identify patients in true CS I, thus avoiding overtreatment. The RPLND histopathology in patients with CS IIA Mk- NSGCT had a high rate of cancer and a low rate of teratoma.

COVID-19 and Spontaneous Resolution of Lumbar Disk Prolapse: A Retrospective Cohort Study of Patients Awaiting Microdiscectomy.

Between individual patients with lumbar disk prolapse (LDP), the natural course of disease is significantly variable. Spontaneous resolution is reported to occur in up to 70% of cases. However, we currently cannot predict for whom and when this will occur. Neurosurgical intervention is indicated for LDP patients with nontolerable pain after at least 8 to 12 weeks of conservative management, or significant neurologic deficit. Channeling essential resources in the National Health Service (NHS) to fight the COVID-19 pandemic led to the postponement of most elective operations, including microdiskectomy. This left many LDP patients previously considered to be surgical candidates with conservative-only options in the interim. To our knowledge, we are the first center to report the specific impact of the peri- and postpandemic period on waiting list times, delayed elective microdiskectomy, and the incidence of spontaneous LDP resolution. Retrospective case series of a prospectively collected electronic departmental database identified LDP patients who would have been impacted by the COVID-19 pandemic at some point in their care pathway (March 2020-February 2022). Further information was obtained from electronic patient records. In total, 139 LDP patients were listed for elective microdiskectomy at the time of postponement of elective surgery. Over a third of LDP patients (n = 47, 33.8%), in shared decision with the responsible neurosurgeon, had their rescheduled microdiskectomy canceled due to clinical improvement (14.1%), radiologic regression (6.5%), or both (12.2%). Our single-center retrospective analysis revealed that for over a third of LDP patients, the prolonged postpandemic waiting list times for elective microdiskectomy resulted in their surgery not taking place either due to spontaneous clinical improvement or proven radiologic regression. Considering this, a prolonged conservative approach to LDP may be appropriate in some patients, allowing time for natural resolution, while avoiding perioperative risks.

Regression of multiple intracranial meningiomas after cessation of long-term synthetic progesterone (megestrol) medication: case report and autopsy.

We report the history of a woman who developed four intracranial meningiomas during 11 years of therapy with the synthetic progesterone agonist megestrol. After discontinuation of the drug at age 75 years, she improved clinically and a CT scan showed near complete regression of the meningiomas by 78 years. Autopsy was performed at 83 years of age following an accidental death. At the tumor sites, we found both collagenous tissue with small islands of low grade meningioma having strong nuclear immunoreactivity for progesterone receptor and lipomatous tissue. A literature review showed similar cases of radiologic meningioma regression following discontinuance of progestins. Our case is the first one with histopathologic characterization of the end point.

P03.01.A “TO BE SMART” IN FOLLOW-UP AFTER CRANIAL RADIOTHERAPY: REPORT OF TWO CASES

Abstract BACKGROUND Stroke-like Migraine Attacks after Radiation Therapy Syndrome (SMART) is a rare, delayed complication of radiation therapy and its real incidence is unknown. In this study, clinical and treatment features of two cases with clinical and radiological diagnosis of SMART are presented. METHODS Case-1: 49 years old male was diagnosed with grade-3 Oligodendroglioma; after gross total surgery treated with 60 Gy local cranial radiotherapy and temozolomide. Patient presented with acute left-sided weakness and numbness, episodic myoclonic jerking of the left arm and leg, 6 years after treatment. Contrast-enhanced MRI and MR angiography revealed no sign of a recurrent tumor but contrast uptake at right parietooccipital cortical giriform in the lobe of the right temporal lobe on post-contrast images (FLAIR and T1) and an increase in cortical blood volume are observed in perfusion MRI (Figure A-B). The clinical and radiologic diagnosis was SMART syndrome. Pulse dose steroids was planned as the primary treatment, and neurological symptoms was completely disappeared. After 3.5 years of the primary episode, patient re-applied with acute headache, homonymous hemianopsia of the left eye and left sided hemiparesia. Cranial MRI revealed the similar features and after treatment with steroids for 7 days complete radiological and clinical regression was experienced. Case-2: 60 years old female with a right occipital grade-3 oligodendroglioma treated with resection followed by local cranial radiotherapy (60 Gy) and temozolomide chemotherapy. Ten years after the treatment she presented with severe headache, expressive aphasia and homonymous hemianopsia of the left eye. Contrast-enhanced MRI and MR angiography revealed no sign of a recurrent tumor but contrast uptake at right occipital cortical giriform on post-contrast images (FLAIR and T1) and an increase in cortical blood volume are observed in perfusion MRI (Figurec. The diagnosis was SMART syndrome. After one week of steroid treatment she completely recovered. Similar complaints recurred 9 months after the first episode and completely recovered with steroids in one week. Radiological finding disappeared in 2 months. CONCLUSION SMART syndrome represents an acute-onset rare condition which may result in serious consequences if not diagnosed and treated properly. It usualy appears years after cranial radiation therapy. Clinical signs and radiological findings may resemble tumor recurrence and distinction from real tumor progression is essential. Treatment is symptomatic, most patients recover with steroids and radiological signs disappear completely, however SMART episodes may recur during the follow-up which can be treated similarly.

Does the number of cycles of neoadjuvant therapy affect the efficacy of neoadjuvant chemoimmunotherapy for non-small cell lung cancer in locally advanced stage? Retrospective experience based on a single center.

The number of cycles of neoadjuvant therapy programmed cell death 1 (PD-1) inhibitor for locally advanced non-small cell lung cancer (NSCLC) remains controversial. From October 2019 to March 2022, neoadjuvant chemoimmunotherapy followed by radical surgery for NSCLC patients with stage II-III were retrospectively reviewed in Shanghai Pulmonary Hospital. The radiologic response was assessed according to the Response Evaluation Criteria for Solid Tumors version 1.1. The major pathological response was defined as no more than 10% residual tumor. Student'st-test, chi-square test, and Mann-Whitney test were used for univariate analysis, logistic regression analysis was used for multivariate analysis. All statistical analyses were calculated by SPSS software (version 26). Among 108 patients, the number of patients who received 2-cycle (2-cycle group) and more than 2-cycle (>2-cycle group) neoadjuvant chemoimmunotherapy were 75 (69.4%) and 33 (30.6%), respectively. Compared with patients inthe >2-cycle group, patients in the 2-cycle group had significantly smaller diagnostic radiological tumor size (37.0mm vs. 49.6mm, p=0.022) and radiological tumor regression rate (36%vs. 49%, p=0.007). However, no significant difference in pathological tumor regression rate was observed between patients in the 2-cycle group and>2-cycle group. Further logistic regression analysis demonstrated that the neoadjuvant chemoimmunotherapy cycle could independently affect the radiographic response (odds ratio [OR]: 0.173, 95% confidence interval [CI]: 0.051-0.584, p=0.005) but not for pathological response (OR: 0.450, 95% CI: 0.161-1.257, p=0.127). For patients diagnosed with stage II-III NSCLC, the number of neoadjuvant cycles administered can significantly influence the radiographic efficacy of chemoimmunotherapy.

Global circulating free DNA methylation and fragmentome deconvolution in patients with metastatic renal cell carcinoma treated with immunotherapy (GOLDEN).

4544 Background: Metastatic renal cell carcinoma (mRCC) lacks molecular biomarkers. Cell-free methylated DNA immunoprecipitation sequencing (cfMeDIP-seq) is a new, non-invasive approach to the detection of RCC-derived DNA in the circulation. In the GOLDEN study (NCT03702309), we explored the role of cfMeDIP-seq and fragmentomic dynamics as a biomarker, during immune checkpoint inhibition (ICI)-based treatment in mRCC patients (pts). Methods: Blood samples from mRCC pts were collected before and during treatment. Response to treatment was defined by the treating oncologist as: response, any degree of radiological tumor regression; stable disease (SD), no radiological change; or progressive disease (PD), radiological/clinical progression. More than 10ng of plasma cfDNA was subject to cfMeDIP-seq. A prognostic pan-cancer methylation signature previously generated using TCGA tissue methylation arrays was applied to cfMeDIP-seq output and cancer-signal methylation (CSM) score was calculated. An RCC-specific methylation signature, previously generated by identifying differentially methylated regions between RCC pts and controls, was applied ( Nuzzo et al, Nat Med 2020). Genome-wide fragmentation profiles were generated using DELFI. Using medians as cut off, log-rank test was performed to assess overall survival (OS) and progression-free survival (PFS). Results: Thirty-five patients were enrolled and n=33 received systemic treatment. 31/35 pts (89%) had clear cell and 4/35 (11%) non-clear cell histology. Median age was 61 (30-81) years and the M:F ratio was 6:1. Patients’ IMDC prognostic risk score was favorable n=6/35 (17%), intermediate n=23/35 (65%) and poor n=6/35 (17%). 20/33 (61%) patients received ipilimumab/nivolumab, 10/33 (30%) nivolumab and n=3/33 (9%) pembrolizumab/axitinib. There were n=14/33 (42%) responders, n=4/33 (12%) pts with SD and 15/33 (45%) pts with PD. Patients with high pan-cancer CSM score at baseline trended towards worse OS (HR 3.86, p = 0.079). Fragmentomics signature, consisting of increased proportion of short cfDNA fragments, showed a trend for worse PFS (HR = 3.04, p = 0.075) in on-treatment samples. Using the RCC-specific methylation signature, we found that those with an increase in signature score from baseline to on-treatment had better PFS (HR=2.4x10-10, p=0.002, n=10) and a trend towards better OS (HR=0.17, p=0.074, n=10). We hypothesize that RCC tissue-specific cell death early during ICI treatment could underpin this increase in the methylation score. Conclusions: This small study is the first report on the role of cfMeDIPseq-derived methylation and fragmentomic signatures in patients with mRCC undergoing ICI-based treatment. We found several trends and one significant association between these signatures and pt outcomes, which support further validation in larger cohorts. Clinical trial information: NCT03702309 .

Early Circulating Tumor DNA Dynamics Predict Neoadjuvant Therapy Response and Recurrence in Colorectal Liver Metastases: A Prospective Study.

For patients with colorectal liver metastases (CRLM) who receive neoadjuvant therapy (NAT), reliable indicators that can early and accurately predict treatment response are lacking. This study was conducted to prospectively investigate the potential of early circulating tumor DNA (ctDNA) dynamics as a precise predictor of NAT response and recurrence in CRLM. This study prospectively enrolled 34 patients with CRLM who received NAT, with blood samples collected and subjected to deep targeted panel sequencing at two time points: 1 day before the first and the second cycles of NAT. Correlations of ctDNA mean variant allele frequency (mVAF) dynamics and treatment response were assessed. The performance of early ctDNA dynamics in predicting treatment response was assessed and compared with those of carcinoembryonic antigen (CEA) and cancer antigen 19-9 (CA19-9). The baseline ctDNA mVAF was significantly associated with pre-NAT tumor diameter (r = 0.65; P < 0.0001). After one cycle of NAT, the ctDNA mVAF declined remarkably (P < 0.0001). The dynamic change in ctDNA mVAF of 50% or more was significantly correlated with better NAT responses. The discriminatory capacity of ctDNA mVAF changes was superior to that of CEA or CA19-9 in predicting radiologic response (area under the curve [AUC], 0.90 vs 0.71 vs 0.61) and pathologic tumor regression grade (AUC, 0.83 vs 0.64 vs 0.67). The early changes in ctDNA mVAF but not CEA or CA19-9 were an independent indicator of recurrence-free survival (RFS) (hazard ratio, 4.0; P = 0.023). For CRLM patients receiving NAT, an early ctDNA change is a superior predictor of treatment response and recurrence compared with conventional tumor markers.

DIPG-like MYB-altered diffuse astrocytoma with durable response to intensive chemotherapy.

Pontine gliomas represent difficult to treat entity due to the location and heterogeneous biology varying from indolent low-grade gliomas to aggressive diffuse intrinsic pontine glioma (DIPG). Making the correct tumor diagnosis in the pontine location is thus critical. Here, we report a case study of a 14-month-old patient initially diagnosed as histone H3 wild-type DIPG. Due to the low age of the patient, the MRI appearance of DIPG, and anaplastic astrocytoma histology, intensive chemotherapy based on the HIT-SKK protocol with vinblastine maintenance chemotherapy was administered. Rapid clinical improvement and radiological regression of the tumor were observed with nearly complete remission with durable effect and excellent clinical condition more than 6.5years after diagnosis. Based on this unexpected therapeutic outcome, genome-wide DNA methylation array was employed and the sample was classified into the methylation class "Low-grade glioma, MYB(L1) altered." Additionally, RT-PCR revealed the presence of MYB::QKI fusion. Taken together, the histopathological classification, molecular-genetic and epigenetic features, clinical behavior, and pontine location have led us to reclassify the tumor as a pontine MYB-altered glioma. Our case demonstrates that more intensive chemotherapy can achieve long-term clinical effect in the treatment of MYB-altered pontine gliomas compared to previously used LGG-based regimens or radiotherapy. It also emphasizes the importance of a biopsy and a thorough molecular investigation of pontine lesions.

Neonatal osteoblastic tumor with a novel PTBP1::FOSB fusion.

Congenital/neonatal bone neoplasms are extremely rare. We present the case of a patient with a neonatal bone tumor of the fibula that had osteoblastic differentiation and a novel PTBP1::FOSB fusion. FOSB fusions are described in several different tumor types, including osteoid osteoma and osteoblastoma; however, these tumors typically present in the second or third decade of life, with case reports as young as 4 months of age. Our case expands the spectrum of congenital/neonatal bone lesions. The initial radiologic, histologic, and molecular findings supported the decision for close clinical follow-up rather than more aggressive intervention. Since the time of diagnosis, this tumor has undergone radiologic regression without treatment.

MP38-10 MULTI-INSTITUTIONAL VALIDATION OF THE PRECISE CRITERIA ON PROSTATE MRI DURING ACTIVE SURVEILLANCE

You have accessJournal of UrologyCME1 Apr 2023MP38-10 MULTI-INSTITUTIONAL VALIDATION OF THE PRECISE CRITERIA ON PROSTATE MRI DURING ACTIVE SURVEILLANCE Francesco Giganti, Armando Stabile, Riccardo Leni, Mark Emberton, Caroline M. Moore, Massimo Valerio, Veeru Kasivisvanathan, and Precise Study Group Francesco GigantiFrancesco Giganti More articles by this author , Armando StabileArmando Stabile More articles by this author , Riccardo LeniRiccardo Leni More articles by this author , Mark EmbertonMark Emberton More articles by this author , Caroline M. MooreCaroline M. Moore More articles by this author , Massimo ValerioMassimo Valerio More articles by this author , Veeru KasivisvanathanVeeru Kasivisvanathan More articles by this author , and Precise Study GroupPrecise Study Group More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000003276.10AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: Currently, AS programmes vary across the world, with different timings in terms of follow-up on MRI and biopsy. The PRECISE criteria provide a dedicated 1-to-5 scale to evaluate the radiological change on serial MRI: a PRECISE score of 1 or 2 denotes radiological regression, PRECISE 3 indicates stability and PRECISE 4 or 5 implies progression. We report the first validation of the PRECISE score in a multicentre international setting. METHODS: We collected data from 22 centres across the world and applied two entry criteria: i) at least 2 MR scans (baseline and follow-up); ii) at least 2 biopsies (baseline and follow-up, the latter after the second MR). Local radiologists reported the scans using PRECISE. Histological progression was defined as any increase in Gleason Score from baseline biopsy. Progression-free survival (PFS) was estimated using Kaplan-Meier curves with landmark time starting from date of first follow-up MRI (i.e., second MR), and multivariable Cox proportional hazards model tested the predictive role of PRECISE. RESULTS: A total of 1,556 patients were included, 1389 (89%) of which had Gleason 3+3 and 167 (11%) had Gleason ≥ 3+4 at baseline. Median follow-up was 48 months, and 513 (33%) patients experienced histological progression. The overall 5- and 10-year progression-free survival (PFS) were 66% and 41%, with time starting at diagnosis. For PRECISE 1-2 (n=158), PFS was 88% at 2 years from first follow-up MRI and 77% at 5 years, while for PRECISE 4-5 (n=413), PFS was 52% and 35%. In those with PRECISE 3 [stability] and visible lesions (n=597), 2- and 5-year PFS was 76% and 50%, while for PRECISE 3 [stability] and non-visible lesions (n=388), PFS was similar to PRECISE 1-2 (2- and 5-year PFS: 91% and 80%) (Log Rank p<0.001). At multivariable analysis, PRECISE 4-5 remained an independent predictor of histological progression (HR: 2.63; p<0.0001). CONCLUSIONS: Our work has validated the PRECISE score in a multicentre setting and has the potential to be included in future risk models that incorporate both clinical and radiological data to fully benefit from prostate MRI and targeted biopsies. Source of Funding: Prostate Cancer Foundation / CRIS Cancer Foundation (Francesco Giganti is a recipient of the 2020 Young Investigator Award). © 2023 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 209Issue Supplement 4April 2023Page: e527 Advertisement Copyright & Permissions© 2023 by American Urological Association Education and Research, Inc.MetricsAuthor Information Francesco Giganti More articles by this author Armando Stabile More articles by this author Riccardo Leni More articles by this author Mark Emberton More articles by this author Caroline M. Moore More articles by this author Massimo Valerio More articles by this author Veeru Kasivisvanathan More articles by this author Precise Study Group More articles by this author Expand All Advertisement PDF downloadLoading ...

A mouth watering case of Mycoplasma salivarium native mitral valve endocarditis: ‘case report’

BackgroundMycoplasma salivarium is part of our commensal oral flora and readily resides in dental plaque. Although considered indolent, few case reports have documented its pathogenic potential in humans. To this day no case of Mycoplasma salivarium infectious endocarditis has ever been described.Case presentationOur report describes a challenging case of Mycoplasma salivarium endocarditis, with a patient presenting with oligoarticular joint swelling, and later on in the course of his disease developed signs of right-sided heart failure. The diagnosis was initially mistaken for septic gonarthritis and was later established on the basis of echocardiography and eubacterial PCR of joint fluid.ConclusionThis report describes a first documented case of Mycoplasma salivarium culture negative endocarditis that was successfully treated with targeted antimicrobial therapy. Specific antimicrobial therapy targeting Mycoplasma spp, lead to clinical improvement, with radiological regression of the lesion and the resolution of the serum inflammation biomarkers.

Long-term outcomes of transcutaneous retrobulbar amphotericin B in COVID-19-associated mucormycosis.

To describe the long-term outcomes of transcutaneous retrobulbar amphotericin B (TRAMB) in COVID-19-associated mucormycosis. In total, 18 cases of COVID-19-associated mucormycosis were reviewed. In addition to the recommended treatment protocol, all patients were to be given 3.5 mg/ml/day of TRAMB for five days. Of the 18 patients, 2 presented with stage 3a disease, 13 had stage 3c disease, and 3 patients had central nervous system (CNS) involvement (stage 4a and 4c). In addition to planned retrobulbar doses, five patients were given more while two patients received fewer injections (i.e., <5). At the last mean follow-up of 34.67 (±8.88) weeks, 11 patients were in radiological regression and 4 had stable disease while 2 patients had to undergo exenteration; one mortality was observed because of disease progression. Clinical regression in terms of visual and ptosis improvement was seen in seven and nine patients, respectively. Rhino-orbito-cerebral mucormycosis is a serious condition which warrants an aggressive treatment strategy. In unprecedented situations witnessed recently, TRAMB turned out to be an effective and economical alternative. Though large randomized studies are needed to establish its efficacy, TRAMB still manages to halt progression and salvage the globe in significant number of patients, and hence its use should be encouraged on a case-to-case basis especially in developing countries with limited resources.

Endoscopic third ventriculostomy for management of hydrocephalus associated with Chiari malformation type II in children

BackgroundHydrocephalus is commonly associated with Chiari malformation (CM) particularly CM type II. The traditional treatment of hydrocephalus in these patients has been cerebrospinal fluid diversion by shunts. Endoscopic third ventriculostomy (ETV) has emerged as an alternative procedure in these patients.PurposeAssessment of the clinical and radiological outcomes of ETV in the management of hydrocephalus in children with CM II.MethodsThis is a prospective study conducted on 18 patients with CM II associated with hydrocephalus admitted to Cairo University hospitals between January 2020 and June 2021. These patients had been managed surgically by ETV. Clinical outcome was assessed based on improvement of manifestations of increased intracranial pressure while radiological outcome was based on the findings of postoperative computed tomography. In cases with early failure, serial lumbar puncture (LP) was performed for 2 days.ResultsETV was performed as a secondary procedure in 4 cases. The overall success rate of the procedure was 72%, and its success rate as a secondary procedure was 100%. Serial LP was effective in decreasing early failure in 44.4% of cases. Radiological regression of hydrocephalic changes was detected in 50% of the cases.ConclusionETV is an efficient and safe procedure in the treatment of hydrocephalus in children with Chiari malformation II, particularly when performed as a secondary procedure. Serial LP following the procedure increases the success rate in patients with early failure.