Tumor Extension Research Articles

DIPG-41. CONVECTION ENHANCED DELIVERY-MEDIATED RADIOSENSITIZATION FOR DIFFUSE MIDLINE GLIOMA

Abstract BACKGROUND Diffuse midline glioma (DMG) is the most aggressive primary brain tumor in children. The only approved standard of care is radiation therapy (RT). Studies examining the role of other systemic agents have failed to demonstrate a survival benefit, partially due to tumor extension to areas of the brain where the blood-brain barrier (BBB) remains intact. As our laboratory has recently determined active NAD(P)H Quinone Dehydrogenase 1 (NQO1) expression in patient-derived DMG tumor samples, we sought to investigate NQO1 bioactivatable agent Napabucasin to augment reactive oxygen species (ROS) production and overcome radioresistance in DMG. Furthermore, we explore the safety and feasibility of convection-enhanced deliver (CED) to overcome limitations of the BBB. METHODS Using two DMG cell lines, we performed clonogenic survival assays with increasing doses of RT with and without Napabucasin. We then created subcutaneous xenograft mouse models, treating with (1) vehicle control, (2) Napabucasin, (3) 2 Gy of RT daily, or (4) combination treatment with RT and Napabucasin. Using a syngeneic orthotopic DMG mouse model, we implanted 7-day osmotic infusion pumps containing either vehicle control or 80uM Napabucasin. Mice then underwent a second randomization with or without daily RT for 5 days. RESULTS We identified a robust radiosensitizing effect with Napabucasin using in vitro clonogenic survival assays. In subcutaneous flank models, combination treatment led to a statistically significant smaller tumor volume post-treatment completion when compared to any monotherapy. In our syngeneic orthotopic DMG mouse model, RT after CED pump implantation in the brainstem did not lead to significant morbidity or mortality. Combination RT and CED of Napabucasin led to a significant survival benefit when compared to monotherapy. CONCLUSIONS In this study, we show Napabucasin as a promising radiosensitizer in DMG. Furthermore, this is the first preclinical study showing safety/efficacy of CED with concurrent RT in DMG.

Genomics to select treatment for patients (pts) with metastatic cancer: Final analysis of Molecular Tumor Board (MTB) evaluations in the ROME trial.

3157 Background: The ROME trial (NCT04591431) investigates the efficacy of a tailored treatment (TT), driven by extensive genomic tests and Molecular Tumor Board (MTB) evaluation, compared to standard treatment (SoC) in patients (pts) with refractory metastatic cancer. Here we report the prevalence of actionable variants identified and the personalized treatment received by the patients enrolled. Methods: A centralized extensive NGS test (FoundationOne CDx and Liquid CDx) was performed on both tissue and blood samples. Pts with at least one potentially targetable alteration matched to the available drugs were discussed at the MTB. MTB evaluation criteria were: genomic variant type, tumor mutational burden (TMB), VAF, availability of preclinical or clinical data of efficacy and patients’ clinical characteristics. COSMIC, ClinVar, OncoKB and VarSome datasets were used to evaluate the clinical actionability of genomic alterations. MTB indications were: randomization to TT vs SoC, screening failure (SF), or indication to other trial/access to the drug. Results: From November 2020 to August 2023, 1794 pts were screened. A total of 127 weekly MTB meetings were conducted and 899 cases were discussed (7 cases/meeting on average). After MTB discussion, a targetable genomic alteration was identified in 425 pts, who received an indication to be randomized. MTB assigned ICIs to 190 pts (155 to ipilimumab plus nivolumab; 8 to nivolumab) due to high (≥10 mut/mb) TMB. 234 pts had indications to target therapy: 86 pts (37%) to PI3K/AKT/mTOR inhibitors(i), 59 (25%) anti-HER2 agents, 34 (15%) FGFRi, 31 (13%) BRAF/MEKi, 7 (3%) PARPi, 5 (2%) CDK4/6i, 4 (2%) ALKi, 2 (>1%) NTRKi, 2 (>1%) RETi, 2 (>1%) Hedgehog-i, 1 (>1%) JAKi, 1 (>1%) MEKi. 27 pts had indication to ICIs plus target therapy combinations. Additionally, 150 pts with potentially hereditary variants were addressed to genetic counselling and germline testing. Finally, according to the genomic evaluation, 63 pts were referred by MTB to other clinical trial even if considered SF for the ROME trial, 34 patients received from the MTB a modification of the initially proposed standard therapy and 4 had both suggestions. Overall, 652 pts (36%) received an indication following NGS test and MTB evaluation. Conclusions: Our study provides evidence that MTB discussion of comprehensive genomic profiling data identifies a personalized treatment in a large (36%) fraction of patients, thus outperforming the traditional histological approach. Clinical trial information: NCT04591431 .

Adjuvant chemotherapy in clinical local advanced CRC following preoperational therapies and pT0-3N0M0 diagnosis (CANWATCH).

TPS3645 Background: Adjuvant chemotherapy is often deemed unnecessary in pathological stage II colorectal cancer (CRC) following initial surgery without high-risk factors for recurrence. However, the efficacy of adjuvant chemotherapy for pT1-3N0M0 CRC following preoperative chemotherapy or chemoradiotherapy remains uncertain. Some cases of clinical locally advanced CRC (cTxN1-2M0), which are down-staged to pT0-3N0M0 after preoperative chemotherapy or chemoradiotherapy, may not truly require adjuvant chemotherapy due to the down-staging effects of preoperative treatments or false-positive imaging diagnosis of lymph nodes. Methods: CANWATCH is a multicenter, open-label, randomized, phase III study with a parallel design conducted in China. Patients were randomly assigned (1:1) to the wait-and-watch group or the adjuvant chemotherapy group. Enrollment and random assignment were performed using a computerized randomization management system. Patients in the wait-and-watch group did not receive adjuvant chemotherapy but were followed up periodically, while patients in the adjuvant chemotherapy group continued to receive adjuvant chemotherapy based on 5-FU or 5-FU analogues (mFOLFOX6 or Capeox). This study is the first prospective randomized controlled study to assess the necessity of adjuvant chemotherapy for patients with locally advanced CRC who received neoadjuvant therapy and achieved a down-staging to pT0-3N0M0. The study aims to provide precise clinical evidence for individualized precision treatment in locally advanced CRC patients. Key inclusion criteria included preoperative clinical tumor stage III (cTxN1-2M0) CRC, pathologically confirmed CRC adenocarcinoma by endoscopic biopsy, postoperative pathological stage T0-3N0M0 without high-risk factors for recurrence, and patient ability to understand and sign written informed consent. Main exclusion criteria were a history of other malignant tumors, complications requiring emergency surgery (occlusion, sub-occlusion, massive hemorrhage, and abscesses), colorectal tumor extension toward the abdominal wall and/or adjacent organs preventing immediate R0 resection of liver metastases, non-resectable lymph node metastasis, American Society of Anesthesiologists (ASA) grading ≥ IV, and/or Eastern Cooperative Oncology Group (ECOG) score ≥ 2. A total of 650 patients are planned to be enrolled in this study, and 301 patients have been enrolled as of February 6, 2024. The study is registered with ClinicalTrials.gov (NCT03748485) and is ongoing. Clinical trial information: NCT03748485 .

Therapeutic Outcome of Multidisciplinary Treatment in Unresectable Biliary Tract Cancer: A Multicenter Retrospective Analysis.

There is little established evidence regarding treatment strategies for unresectable biliary tract cancer (BTC). This study aimed to clarify the situation of multidisciplinary treatment for unresectable BTC in the 2000s when there was no international standard first-line therapy. We retrospectively reviewed 315 consecutive patients with unresectable BTC who had been treated at seven tertiary institutions in Kanagawa Prefecture, Japan between 1999 and 2008. The unresectable factors were as follows: locally advanced, 101 cases (32.1%); hematogenous metastases, 80 cases (25.4%); and peritoneal dissemination, 30 cases (9.5%). Chemotherapy or radiation therapy was administered to 218 patients (69.2%). The best supportive care was provided in 97 cases (30.8%). The most common regimen was gemcitabine monotherapy, followed by gemcitabine combination therapy and S-1 monotherapy. The 1- and 2-year survival rates of all patients were 34.6% and 12.2%, respectively. The median survival time (MST) was 8 months in all patients. The 1-year survival rate was 65%, and the MST was 12 months among the locally advanced patients, whereas patients with peritoneal dissemination had the worst outcome; the 1-year survival rate was 7%, and the MST was 5 months. Among treated 90 cases of perihilar cholangiocarcinoma, patients who received chemoradiotherapy (n = 24) had a significantly better outcome than those who received chemotherapy alone (MST: 20 vs. 11 months, P < 0.001). Unresectable BTC has heterogeneous treatment outcomes depending on the mode of tumor extension and location. Multidisciplinary treatment seems useful for patients with locally advanced BTC, whereas patients with metastatic disease still have a poor prognosis.

Neoadjuvant chemotherapy as an indication to preserve nipple areolar complex in advanced breast cancer.

e12655 Background: Nipple areolar skin sparing mastectomy (NASSM) has similar oncologic and surgical safety compared to skin sparing mastectomy (SSM) and conventional mastectomy. It provides a superior cosmetic outcome, patient satisfaction and quality of life. With the increasing rate of pathological response rate with neoadjuvant chemotherapy (NAT), nipple preservation can be achieved. The aim of our study is to evaluate whether NASSM is an appropriate surgical procedure for patients in whom there is radiological evidence of extension or involvement of nipple areolar complex (NAC) prior to NAT. Methods: Patients with advanced breast cancer received neoadjuvant chemotherapy and NASSM or SSM between June 2006 and December 2021 at Asan Medical Center were retrospectively reviewed. We excluded patients with metastatic breast cancer on presentation, bilateral cancer and inflammatory breast cancer. Kaplan-Meier survival analysis and Cox proportional hazard models were applied to determine clinicopathological and radiological factors to determine NAC preservation. Our primary objective to assess tumor cells persistence in the NAC after radiological complete response or resolution of extension to NAC. Results: Total of 1,105 patients included. 71.7% of patients underwent NASSM and 17.2% SSM as a final operation. 144 patients (13%) had a change in the pre- NAT type of surgery decision. 83(7.5%) patients had nipple involvement on final pathology. Out of 312 patients who underwent SSM, only 76 patients had nipple involvement on final pathology When comparing patients who had nipple involvement to those who didn’t on final pathology, clinical T &amp; N stage, histologic &amp; nuclear grade, breast cancer subtype, presence of multifocality/multicentric disease on MRI pre or post NAT, distance of mass to NAC and mass size pre and post NAT, the presence of non mass extension to NAC post NAT, NAC thickening on any image pre and post NAT and presentation symptoms of nipple retraction and skin changes were statistically significant. A multivariate regression analysis was performed to compare all the above factors and showed that breast cancer subtype, tumor nipple distance on MRI pre NAT, nipple areolar thickening on MRI pre NAT, clinical response and nipple retraction on MMG post NAT were statistically significant and indicators of nipple involvement on final pathology. Only 8 patients developed NAC recurrence in the NASSM group. Conclusions: NASSM is a safe procedure in patients who had tumor extension to NAC prior to NAT and showed improvement post NAT as evident on MRI. Further prospective studies are needed to establish and criteria to preserve NAC in this subgroup of patients.

Real-world effectiveness and safety of PD-1 inhibitors combined with recombinant human endostatin and chemotherapy in refractory, recurrent/metastatic nasopharyngeal carcinoma.

e18007 Background: Nasopharyngeal carcinoma(NPC) is one of the most common head and neck malignancies with a high overall survival rate. However, the treatment of refractory, recurrent or metastatic NPC is complicated. This study aimed to evaluate the safety and effectiveness of GEP (Gemcitabine combined with recombinant human endostatin (Rh-endostatin) and anti-PD-1 antibody) in patients with refractory, recurrent or metastatic NPC that cannot be treated locally in the real-world setting. Methods: Patients with refractory, recurrent or metastatic NPC (Refractory patients were defined as those unsuitable for local treatment due to extensive tumor recurrence/metastasis or necrosis of ulcers) treated in our hospital from December 2022 to October 2023 were retrospectively reviewed and analyzed. with the GEP regimen. Patients received 1-6 cycles of GEP therapy, including Gemcitabine (1.0 g /m2 ond1 and d8) combined with Rh-endostatin (180-210 mg/continuous intravenous pump 72-120h) and PD-1 antibody, Objective response rate (ORR) and disease control rate (DCR) were determined by Clopper-Pearson's method. The incidence of adverse events (AES). Results: A total of 25 patients who completed ≥ 2 cycles of treatment and at least one efficacy assessment were included in the efficacy analysis. The median age was 59 years (range: 33-75 years), 36% of patients ≥ 65 years. There was significant liquefaction necrosis in 17 cases (68%). 68% of the patients were treated with second-line or above GEP. 32% (8/25) of patients had tumor progression after anti-vascular therapy, and 44% (11/25) had disease progression after immunotherapy. Based on RECIST 1.1, the ORR was 44.00% (95%CI: 26.67%, 62.93%), and DCR was 92.00% (95%CI: 70.04%, 95.83%). The history of immunotherapy was a significant influence factor on ORR. 30 patients who received at least one course of GEP therapy were included in the safety analysis. Grade 3 AEs were observed in 7 patients (20%), with hematological toxicity (6/7) and hypertension (1/7). Other AEs with more than 20% incidence were hematological toxicity (93.0%), weight loss (67.0%), abdominal distension and constipation (47.0%), hypertension (47%), infection (33.0%), liver dysfunction (27.0%), nausea and vomiting (23.0%), and infusion reaction (20.0%). The recurrent lesions of 15 patients with recurrent NPC were accompanied by significant liquefaction necrosis and ulcers. The ORR was 46.7%, and DCR was 100%. Among them, 5 patients had grade 1 nosebleed, and the other grade 2 or above AEs were hematological toxicity, and there were no grade 4 treatment-related AEs. Conclusions: Real-world data demonstrated that GEP was well-tolerated, safe and effective in patients with refractory, recurrent or metastatic NPC.

Pineal Tumor Surgery—The Choice of the Approach Related to Tumor Characteristics and Posterior Fossa Anatomy

This research aimed to determine whether an adequate surgical approach can be chosen based on clearly defined values of anatomical landmarks (tentorial angle) and tumor size and extension. We conducted a retrospective analysis of patients operated on because of pineal tumors. The cohort was divided depending on the surgical approach. On preoperative magnetic resonance imaging, we measured maximal diameters, tumor volume, and tumor propagation. In the group of patients operated with the supracerebellar infratentorial approach, we also tested the correlation of tentorial angle with residual tumor. Differences among groups in resection, complications rate, and outcome were tested by the χ2 test. Finally, in both groups, the correlation of residual tumor with tumor volume, propagation, and diameters was tested using the receiver operating characteristic curve. In the group operated with a supracerebellar approach, total resection was achieved in 78% of the patients. The critical value of cranio-caudal diameter correlated with tumor residue was 31mm, for lateral-lateral diameter 25mm, for the lateral extension 14mm, and tumor volume 12cm3. Tentorial angle did not influence the extent of the resection. In the group operated with an occipital transtentorial approach, the critical tumor volume related to tumor residue was 9mm3, anterior-posterior diameter 29mm, and cranio-caudal diameter 28mm. The extent of the resection was significantly higher in the supracerebellar group. In both approaches, tumors larger than 3cm show an increased risk of subtotal resection. Except when most tumor volume is localized above the venous system, we advocate a supracerebellar corridor as an effective approach that is not limited by tentorial angle.

Management of Bilateral Borderline Ovarian Tumor in a 26-year-old Patient in a Resource-Limited Setting

&amp;lt;i&amp;gt;Background&amp;lt;/i&amp;gt;: borderline ovarian tumors are rare epithelial tumors, more common before the age of 40, with challenging preoperative diagnosis. Our aim is to describe the management of a case in a resource-limited context in Burkina Faso. &amp;lt;i&amp;gt;Case report:&amp;lt;/i&amp;gt; a 26-year-old primigravida with a 4-year-old child presented with an abdominal mass evolving over one year in the context of secondary amenorrhea. Clinical examination revealed weight loss, cutaneous-mucosal pallor, increased abdominal volume with tender deep palpation, and an irregular, mildly firm mass around the umbilicus extending to the iliac fossae, with a depressed area along the midline. Speculum examination was normal. On vaginal examination, the cervix was unremarkable, the uterus was of normal size, but the lateral fornices and Douglas pouch were filled. Further investigations revealed microcytic hypochromic anemia and a borderline normal CA 125 level. The rest of the laboratory tests were normal. Abdominal and pelvic ultrasound and CT revealed a strong suspicion of a bilateral ovarian tumour, with no signs of secondary localisation. MRI was not available. The diagnosis of probably malignant bilateral ovarian tumour was retained, and laparotomy was indicated. When the parietal peritoneum was opened, two ovarian tumours with a budding appearance occupied the entire lower half of the abdomen. A part of the omentum was in contact with both masses. Bilateral adnexectomy, total hysterectomy and partial omentectomy were performed in the absence of an extemporaneous histological study. Histological study of the surgical specimens concluded that the tumour was borderline ovarian. Follow-up at 3 years showed no signs of recurrence or tumour extension. &amp;lt;i&amp;gt;Conclusion&amp;lt;/i&amp;gt;: borderline ovarian tumours are managed surgically, and the choice of conservative or radical surgery is not easy when there is a desire to have children, and when the ovaries are bilaterally involved. Fortunately, the vital prognosis is favourable.

Analysis of MRI features and associated factors of the pituitary neuroendocrine tumor streaky sign.

Observation of pituitary neuroendocrine tumors with streaky sign on MRI, analysis of their features on imaging and further investigation of the relationship between the direction of the streak sign and the direction of optimal tumor expansion. The MR images of 237 patients with pituitary neuroendocrine tumors were retrospectively analyzed. The streaky-like high signal with a substantial length of more than 10 mm and obvious enhancement on T1WI was defined as the streaky sign. Finally, 66 patients were included in the study, comprising 33 patients with streaky sign pituitary neuroendocrine tumors and 33 randomly selected patients with non-streaky sign pituitary neuroendocrine tumors. The general condition of these 66 patients, the imaging features of the tumor, and the measurement and analysis of the direction of the streaky sign in relation to the direction of optimal tumor extension were observed and analyzed. On MRI, 85 streaky signs were observed. The average deviation between the direction angle of all the streaky signs and the optimal extension direction angle of the tumor was approximately 11°. The longest streaky sign angle was positively correlated with the optimal extension angle of the tumor, with a correlation coefficient of 0.967. The presence of a streaky sign of pituitary neuroendocrine tumors may indicate a dilated sinus or a small blood vessel. Its direction is highly consistent with the optimal extension direction of the tumor, which has a certain supporting effect on the long-distance growth of the tumor.

The Discovery of Oral Cancer Prognostic Factor Ranking Using Association Rule Mining.

A 5-year survival rate is a predictor for the assessment of oral cancer prognosis. The purpose of this study is to analyze oral cancer data to discover and rank the prognostic factors associated with oral cancer 5-year survival using the association rule mining (ARM) technique. This study is a retrospective analysis of 897 oral cancer patients from a regional cancer center between 2011 and 2017. The 5-year survival rate was assessed. The multivariable Cox proportional hazards analysis was performed to determine prognostic factors. ARM was applied to clinicopathologic and treatment modalities data to identify and rank the prognostic factors associated with oral cancer 5-year survival. The 5-year overall survival rate was 35.1%. Multivariable Cox proportional hazards analysis showed that tumor (T) stage, lymph node metastasis, surgical margin, extranodal extension, recurrence, and distant metastasis of tumor were significantly associated with overall survival rate (p < 0.05). The top associated death within 5 years rule was positive extranodal extension, followed by positive perineural and lymphovascular invasion, with confidence levels of 0.808, 0.808, and 0.804, respectively. This study has shown that extranodal extension, and perineural and lymphovascular invasion were the top ranking and major deadly prognostic factors affecting the 5-year survival of oral cancer.

Bioinformatics analysis and validation of mesenchymal stem cells related gene MT1G in osteosarcoma.

Osteosarcoma (OS) is a primary malignant bone tumor arising from mesenchymal cells. The standard clinical treatment for OS involves extensive tumor resection combined with neoadjuvant chemotherapy or radiotherapy. OS's invasiveness, lung metastasis, and drug resistance contribute to a low cure rate and poor prognosis with this treatment. Metallothionein 1G (MT1G), observed in various cancers, may serve as a potential therapeutic target for OS. OS samples in GSE33382 and TARGET datasets were selected as the test cohorts. As the external validation cohort, 13 OS tissues and 13 adjacent cancerous tissues from The Second Affiliated Hospital of Nanchang University were collected. Patients with OS were divided into high and low MT1G mRNA-expression groups; differentially expressed genes (DEGs) were identified as MT1G-related genes. The biological function of MT1G was annotated using Kyoto Encyclopedia of Genes and Genomes (KEGG), Gene Ontology (GO) and gene set enrichment analysis (GSEA). Gene expression correlation analysis and competing endogenous RNA (ceRNA) regulatory network construction were used to determine potential biological regulatory relationships of DEGs. Survival analysis assessed the prognostic value of MT1G. MT1G expression increased in OS samples and presented higher in metastatic OS compared with non-metastatic OS. Functional analyses indicated that MT1G was mainly associated with spliceosome. A ceRNA network with DEGs was constructed. MT1G is an effective biomarker predicting survival and correlated with increased recurrence rates and poorer survival. This research identified MT1G as a potential biomarker for OS prognosis, highlighting its potential as a therapy target.

Correlation of microscopic tumor extension with tumor microenvironment in esophageal cancer patients

ObjectiveIn the era of image-guided adaptive radiotherapy, definition of the clinical target volume (CTV) is a challenge in various solid tumors, including esophageal cancer (EC). Many tumor microenvironmental factors, e.g., tumor cell proliferation or cancer stem cells, are hypothesized to be involved in microscopic tumor extension (MTE). Therefore, this study assessed the expression of FAK, ILK, CD44, HIF-1α, and Ki67 in EC patients after neoadjuvant radiochemotherapy followed by tumor resection (NRCHT+R) and correlated these markers with the MTE.MethodsFormalin-fixed paraffin-embedded tumor resection specimens of ten EC patients were analyzed using multiplex immunofluorescence staining. Since gold fiducial markers had been endoscopically implanted at the proximal and distal tumor borders prior to NRCHT+R, correlation of the markers with the MTE was feasible.ResultsIn tumor resection specimens of EC patients, the overall percentages of FAK+, CD44+, HIF-1α+, and Ki67+ cells were higher in tumor nests than in the tumor stroma, with the outcome for Ki67+ cells reaching statistical significance (p < 0.001). Conversely, expression of ILK+ cells was higher in tumor stroma, albeit not statistically significantly. In three patients, MTE beyond the fiducial markers was found, reaching up to 31 mm.ConclusionOur findings indicate that the overall expression of FAK, HIF-1α, Ki67, and CD44 was higher in tumor nests, whereas that of ILK was higher in tumor stroma. Differences in the TME between patients with residual tumor cells in the original CTV compared to those without were not found. Thus, there is insufficient evidence that the TME influences the required CTV margin on an individual patient basis.Trial registration number and dateBO-EK-148042017 and BO-EK-177042022 on 20.06.2022, DRKS00011886, https://drks.de/search/de/trial/DRKS00011886.

Surgical resection of advanced intrathoracic tumors through a combination of the hemiclamshell and transmanubrial approaches

PurposeThe hemiclamshell (HCS) approach provides a comprehensive view of the anterior mediastinum, whereas the transmanubrial osteomuscular sparing approach (TMA) allows sufficient exposure of the cervico-thoracic transition. We assessed the effectiveness and the outcomes of the combined HCS plus TMA approach to resect thoracic malignant tumors.MethodsWe reviewed five patients with thoracic malignant tumors invading the thoracic outlet who underwent surgery using an HCS and TMA approach between 2018 and 2021.ResultsThe preoperative diagnosis was myxofibrosarcoma, lung cancer, thymic cancer, thymoma, and neurofibromatosis type1 in one patient each, respectively. Cardiovascular reconstruction was done on the aortic arch in two patients, on the descending aorta in one, and on the superior vena cava in one, combined with resection of the vagus nerve in three patients, of the phrenic nerve in two, and of vertebra in one, with overlap in some cases. The TMA was added because all patients required dissection of the periphery of the subclavian artery, and two had tumor extension to the neck. Macroscopic complete resection was achieved in four patients. There was no postoperative mortality.ConclusionThe combination of the HCS and TMA approaches at the same operation provides a comprehensive view of the mediastinum, lung, and cervico-thoracic transition and allows safe access to the thoracic great vessels and subclavian vessels.

The telovelar approach for fourth ventricular tumors in children: is removal of the posterior arch of C1 necessary?

PurposeVarious surgical nuances of the telovelar approach have been suggested. The necessity of removing the posterior arch of C1 to accomplish optimal tumor exposure is still debated. Therefore, we report on our experience and technical details of the fourth ventricular tumor resection in a modified prone position without systematic removal of the posterior arch of C1.MethodsA retrospective analysis of all pediatric patients, who underwent a fourth ventricular tumor resection in the modified prone position between 2012 and 2021, was performed.ResultsWe identified 40 patients with a median age of 6 years and a M:F ratio of 25:15. A telovelar approach was performed in all cases. In 39/40 patients, the posterior arch of C1 was not removed. In the remaining patient, the reason for removing C1 was tumor extension below the level of C2 with ventral extension. Gross or near total resection could be achieved in 34/39 patients, and subtotal resection in 5/39 patients. In none of the patients, a limited exposure, sight of view, or range of motion caused by the posterior arch of C1 was encountered, necessitating an unplanned removal of the posterior arch of C1. Importantly, in none of the cases, the surgeon had the impression of a limited sight of view to the most rostral parts of the fourth ventricle, which necessitated a vermian incision.ConclusionA telovelar approach without the removal of the posterior arch of C1 allows for an optimal exposure of the fourth ventricle provided that critical nuances in patient positioning are considered.

A Rare Case of Clival Hemangioma Simulating Chordoma

AbstractPrimary intraosseous hemangiomas are rare, benign, vascular malformations that account for ∼1% of all primary bone neoplasias. A 59-year-old female patient with unknown comorbidities had a history of headache, visual impairment and dizziness that led to the diagnosis of a clivus tumor. Two resections were attempted through transcranial and transnasal transsphenoidal approach in the last two years in another hospital. The initial MRI scan showed an expansive lesion with T2 hyperintense signal and diffuse, heterogenic contrast enhancement. Clival chordoma was the main diagnostic hypothesis done. A CT scan was performed to evaluate the extent of clival invasion, the sinus anatomy, and the clival destruction - all simulating clival chordoma. The interdisciplinary tumor board decided to proceed with endoscopic endonasal tumor resection. There were no postoperative complications and the histopathological analysis revealed a primary intraosseous haemangioma. Skull base intraosseous hemangiomas are rare entities, with a limited number of case reports found after literature reviews, especially in the clival region. The clinical pattern and imaging characteristics can vary widely according to the tumor extension and development, simulating some other common tumors found at this topography. We present a case report of a clival intraosseous hemangioma presenting as an isolated abducens paresis with a positive outcome after intranasal endoscopic resection after two years of follow-up.

Anterior Chest Wall Sarcoma: An Aggressive Tumor

Abstract Patients with chest wall tumors present diagnostic and therapeutic challenges. The differential diagnosis of these tumors is broad because they can present a heterogeneous spectrum of diseases such as primary benign or malignant tumors, metastasis, local extension of adjacent tumors, and infectious or inflammatory conditions. Among that, primary chest wall tumors are rare.

Interdisciplinary plastic and reconstructive surgery of head and neck squamous cell carcinomas.

Squamous cell carcinomas are the most common malignancies in the oral cavity, pharynx, and larynx. Even in the age of the most modern drug treatment methods, radical resection of these tumors is and currently remains the therapeutic gold standard. The loss of anatomical structures associated with surgery inevitably increases the functional deficits caused by the tumor itself. In this context, the extent of functional deficits is largely determined by the extent of resection. Complete organ resections, such as glossectomy, complete palate resection, laryngectomy, or transverse pharyngo-laryngectomy, lead to severe functional deficits, such as swallowing disturbances with life-threatening aspiration and articulation disorders up to the inability to speak. With the help of plastic reconstructive surgery, the lost tissue can be replaced and the specific functions of the upper aerodigestive tract can be preserved or restored.In recent decades, reconstructive surgical procedures have developed enormously in the treatment of malignant tumors of the head and neck. In order to make optimal use of them, a comprehensive, interdisciplinary therapy concept is a prerequisite for positive oncological and functional outcome. In addition to general medical and social parameters, surgical parameters play a crucial role in the choice of the reconstruction method. The extent to which the surgical measures must be interdisciplinary depends on the localization of the defects in the head and neck region and on the type of replacement tissue required. Here, the expertise of plastic surgery, oral and maxillofacial surgery, and abdominal surgery comes into play in particular. The use of different tissues, the combination of different grafts and flaps, or the preforming of donor regions allow reconstructions far beyond the level of simply restoring surface integrity. The functional results and thus the quality of life of patients after surgical therapy of extensive tumors of the mentioned localizations depend decisively on the type of reconstruction. Therefore, in the following review, special emphasis 1 be placed on the choice of reconstruction method and reconstruction technique for tissue loss after resections of HNSCC.

Clinicopathological Outcome of Early and Late Onset Renal Cell Carcinoma: An Observational Study

ABSTRACTIntroduction Renal Cell Carcinoma (RCC) is considered a disease of old age and is typically found in the 6th-8th decade of life. The diagnosis of RCC is increasing more rapidly in younger age patients. This study was conducted to evaluate the clinicopathological pattern and outcome of early onset RCC and compare them with the older age group patients.MethodsThis was a single center retrospective observational study. All the patients undergoing radical or partial nephrectomy during the period of November 2019 to October 2022 for renal masses and whose final histopathology report showed RCC were included in the study. Clinicopathological patterns and outcome were compared between the two age groups.ResultsAmong 47 patients, 17 (36.2%) patients were at or below the age of ≤46 years and 30 (63.8%) patients in older age group. There was significant difference in male (29): female (18) ratio between the two age groups (p &lt; 0.005). Younger age patients (≤46 yrs) had higher stage of disease at presentation compared with the older age (&gt;46 yrs) patients [tumor extension into major veins and perinephric tissue (T3) and above 26 vs 21] and they had significantly higher mortality rate (29.4% vs 3.33%, p =0.010).ConclusionPatients aged ≤46 years of age were more frequently affected by RCC with female predominance. Younger age group had higher stage of disease at presentation with higher mortality in comparison to older age group.KeywordsClinicopathological; early onset; late onset; renal cell carcinoma

Pulmonary Infundibular Stenosis Secondary to a Mediastinal Hodgkin Lymphoma Tumor Invasion in a Young Adult Male: What a Rare Occurrence!

Introduction: Cardiac involvement is a rare unusual localization of Hodgkin lymphoma, as it’s known that primary lymphomas of the heart are rare. They develop primarily in the right atrium, and extend to the pericardium in about one third of the cases. Pulmonary infundibular stenosis secondary to mediastinal mass Hodgkin lymphoma invasion is rare but not impossible especially in relapse cases. Case Report: A rare case of a young adult patient diagnosed with Hodgkin Lymphoma under chemotherapy presents a relapse of his pathology associated with heart failure; predominantly, right heart failure due to extension of the mediastinal mass tumor to the right cavities of the heart causing pulmonary infundibular stenosis which is a rare occurrence. He was put under an intense chemotherapy and symptomatic heart failure treatment with close follow-up after multi-disciplinary discussions including cardiologist, onco-hematologists and cardiothoracic surgeons. Three months later, a significant reduction of the mass was observered. Conclusion : Pulmonary infundibular stenosis secondary to mediastinal Hodgkin lymphoma invasion is a rare entity of secondary tumors of the heart. Multimodal imagery is the key tool for diagnosis. There prognosis depend on multiple factors but generally good in younger adult patients compared to aged individuals.

TUMOUR THROMBUS ASSOCIATED WITH RENAL CELL CARCINOMA: A RARE CASE OF DELICATE SURGERY

Metastatic renal cell carcinoma is in itself a serious and potentially fatal cancer, but when renal vein thrombus is added, the patients short-term prognosis worsens and surgery becomes even more complicated and mutilating. Four to ten per cent of kidney tumours invade the inferior vena cava and develop tumour extension into the renal vein and inferior vena cava. Only 1% has thrombus extension into the right atrium, a rare situation but one with a guarded prognosis. Cytoreductive nephrectomy is associated with an improvement in overall survival in patients without thrombus and in those with tumour thrombus limited to the renal vein and infra-diaphragmatic inferior vena cava with the advent of immunotherapy. Our article presents the case of a 73-year-old female patient who was admitted to Mohamed V Military Hospital of instruction for investigation of lumbar pain and imaging combined with a biopsy of the renal mass confirmed a metastatic renal tumour with thrombus extending to the junction of the renal vein with the inferior vena cava. The multidisciplinary consultation meeting decided to perform an extended total nephrectomy prior to systemic treatment and the challenge was to also remove the tumour thrombus from the renal vein and avoid any associated complications, which could be fatal.