Mohiuddin M. Taher^1,2,3, Abdulaziz Abdulnasser Alhussini³, Muhammad Saeed⁴, Mohammad Athar^1,2,3, Najwa Abdalkabeer A. Bantan⁴, Raid A. Jastania^5,6, Kamal Bakour Balkhoyour⁷, Tahani H. Nageeti⁸

¹ Science and Technology Unit, Umm-al-Qura University, Makkah 21955, Saudi Arabia.
² Deanship of Scientific Research, Umm-Al-Qura University, Makkah 21955;
³ Department of Medical Genetics, Faculty of Medicine, Umm-Al-Qura University, Makkah 21955, Saudi Arabia
⁴ Department of Radiology, Al-Noor Specialist Hospital, Makkah 24242, Saudi Arabia.
⁵ Department of Pathology, College of Medicine, Umm-Al-Qura University, Makkah 24381;
⁶ Department of Pathology, College of Medicine, King Abdul Aziz Medical City, Jeddah 21423:
⁷ Department of Neurosurgery, Al-Noor Specialist Hospital, Makkah 24242, Saudi Arabia.
⁸ Radiation Oncology Department, King Abdullah Medical City, Makkah 24246, Saudi Arabia;

Objectives: Primary intracranial myxopapillary ependymomas (MPE) are very rare. In order to determine genomic changes in an intracranial MPE, we analyzed its mutation patterns by next generation DNA sequencing.

Methods: Tumor DNA was sequenced using an Ion PI v3 chip on Ion Proton instrument and the data were analyzed by Ion Reporter 5.6.

Results: In this tumor, NGS generated 6,298, 354 mapped reads using the Ion PI v3 Chip. The average reads per amplicon was 29,365, 100% of amplicons had at least 500 reads and the amplicons read end-to-end were 97.58%. In this tumor, NGS data analysis identified 12 variants, of which two were missense mutations, seven were synonymous mutations and three were intronic variants. Missense mutation in c.395G>A; in exon 4 of the IDH1 gene, and a missense mutation in c.215C>G; in exon 4 of the TP53 gene were found in this tumor were previously reported. The known synonymous mutations were found in this tumor were, in exon 14 of FGFR3 in c.1953G>A; in exon 12 of PDGFRA in c.1701A>G; in exon 18 of PDGFRA c.2472C>T; in exon 20 of EGFR in c.2361G>A; in exon 13 of RET in c.2307G>T; in exon 16 of APC in c.4479G>A; and in exon 2 of MET in c.534C>T. Additionally, a known intronic variant was identified in KDR and a known acceptor site splice variant in FLT3 (rs2491231) and a SNP in the 3’-UTR of the CSF1R gene (rs2066934) were also identified. Except, the frequency of IDH1 variant, the frequencies of other variants were high, and the p-values were significant and Phred scores were high for all of these mutations.

Conclusion: The variants reported in this tumor have not been detected in myxopapillary grade I ependymoma tumor by NGS analysis previously and we therefore report these variants in this case for the first time.

Keywords: Intracranial ependymoma, Myxoid degeneration, Myxopapillary ependymoma, Palisading necrosis, Ion Proton, Next Generation DNA sequencing, pediatric brain tumors.

Evaluation Of Pathological Response And Its Predictors In Carcinoma Rectum Following Neoadjuvant Chemoradiation

Shoaib Nawaz¹, Sangeetha.k.Nayanar¹, Nabeel Yahiya¹

¹ Malabar Cancer Centre, Thalassery, Kerala, India.

Background: Neoadjuvant chemoradiation followed by surgery is the standard of care in locally advanced rectal tumors. Better pathologic response after chemoradiation is associated with better outcomes. Pathologic response may depend on various, patient and tumor related factors. The aim of our study was to assess the pathological response using a modified Ryan scoring system and to study various factors which influence the response.

Methods and Materials: This is a retrospective study carried out at a tertiary cancer centre in India. Patient details and histopathology reports of rectal cancer patients who took neoadjuvant chemoradiation from January 2016 to December 2018 were analyzed. Demographic details, pathological response assessed by modified Ryans tumor regression grade (TRG) score and various factors which influence the pathological response were studied. Those with TRG score 0 (complete response) and1(near complete response) were grouped together as good responders and those with score 3 (partial response) and 4 (poor or no response) as poor responders. Univariate and multivariate analyses were performed using logistic regression to determine factors which influence pathologic response.

Results: There were a total of 83 patients. Males and females were equally distributed. 43.4%(n=36) of patients had lower rectal tumors,32.5%(n=27) had mid rectal tumors and 24.1%(n=20) had upper rectal tumors. 46% of patients were good responders which includes complete responders, 17% (n=14) and those with a near complete response, 29% (n=24). 54% of patients were poor responders, which includes those with incomplete response, 36% (n=34) and with no or poor response,18% (n=15). Among the upper rectal tumors, only 20% had good response and among the mid and lower rectal tumors 54% had good response.(p value 0.02) .63% of males were good responders in comparison to 37% among females (p value 0.05).

Discussion: Response to neoadjuvant chemoradiation with capecitabine in locally advanced rectal tumors in our institute is similar to the literature data with a complete response in 16.9%, near complete response in 28.9% partial response in 36.1% and no response in 18.1% of patients, according to modified Ryan score. It was found that upper rectal tumors had a poorer response when compared to mid and lower tumors and females had a poorer response compared to males.

Conclusion: Even though neoadjuvant chemoradiation remains the standard of care in locally advanced rectal carcinomas, its benefit in upper rectal tumors needs to be validated in larger studies.

Keywords: Rectal cancer, Neoadjuvant chemoradiation, Modified Ryan TRG score, Pathological response

Maha Ismail¹, Marwa M. Shakweer², Hesham El Wakiel¹, Dalia Abd El Ghany¹, Ahmed Gaballah¹

Introduction: Tumor microenvironment plays crucial role in cancer evolution. There is a dynamic and continuous relation between immune cells and cancer cells’ resistance. Tumor infiltration CD8-lymphocytes and programmed death ligand-1 have proved important prognostic role in different malignancies. We aimed at evaluating this role in laryngeal cancer.

Patients and Methods: We prospectively analyzed laryngeal cancer patients’ specimens, to identify the CD8-lymphocytes and the PD-L1 expression. A total score formed of the sum of percentage and intensity of PD-L1. A final rate was considered as negative or low when combined percentage and intensity scores 0 to 4, and high when scores 5-7. CD8-lymphocyte infiltration was divided into strong (= 10/100 of epithelial cells or =20/100 stromal cell infiltration) or weak (< 10/100 epithelial cells or < 20/100 stromal cell infiltration).

Results: Forty patients were included; twelve had stage 1 or 2 and 28 with advanced stages. PD-L1 expression was positive in 92.5%. Neither the PD-L1 nor CD8- lymphocytes had overall survival impact, however high PD-L1 correlated with better survival in advanced stage subgroup (p = 0.036), high CD8-lymphocytes infiltration had better survival but did not reach significance. There was significant correlation between the CD8-lymphocyte infiltration; whether epithelial or stromal, and tumor PD-L1 expression; p-value of 0.001 and < 0.0001 respectively. Subgroup of patients with low CD8+ infiltration and low PD-L1 had the worst survival.

Conclusions: There is a correlation between CD8- lymphocytes infiltration and PD-L1 expression in laryngeal cancer and high PD-L1 expression is associated with better OS in advanced stages.

Keywords: PD-L1, CD8, laryngeal cancer, tumor microenvironment

The Prognostic Value Of The ART Score Before The Second Transarterial Chemoembolization

IFatima Zahra. Hamdoun¹, Younes Hassani², Hakima. Abid^1/strong>, Youssef. Lamrani Alaoui², Mounia. El Yousfi¹, Dafr-allah Benajah¹, Moustapha. Maaroufi², Mohammed. El Abkari¹, SidiAdil. Ibrahimi¹, Nada. Lahmidani¹

Background: The transarterial chemoembolization (TACE) is a firstline therapeutic option for advanced hepatocellular carcinoma (HCC). Their indications are clearly defined by learned societies but the challenge is to determine the optimal number of TACE sessions that will benefit patients before switching to other therapies. For this reason, the Assessment for Retreatment with Transarterial chemoembolization (ART) score has been developed. The objective of our work is to show the prognostic value of the ART score before the second TACE.

Methods: This is a retrospective and prospective study of patients with hepatocellular carcinoma on cirrhosis liver who received a TACE between January 2012 to July 2019. The diagnosis of HCC was made according to the non-invasive criteria of EASL with the use of histology for doubtful cases. The ART score was calculated after the first chemoembolization. Patients were divided into 2 groups: group A with an ART score between 0 and 1.5 and group B with a score =2.5.

Results: During the study period, 58 patients with HCC on cirrhosis liver received a TACE: 55.17% had an ART score between 0 - 1.5 before the second session and 44.8% had an ART score =2,5. Both groups were comparable regarding age, circumstances of discovery and Child’s score. The size of the HCC as well as the value of the AFP was further increased in the group B. We observed a significant difference in the radiological response, the Child score and aspartate transaminase rate between the two groups after the first TACE. The overall survival rate at 3 years was 81% in group A versus 19% in group B.

Conclusion: The ART score has an independent prognostic value and should be taken into account in the therapeutic strategy before the second TACE.

Keywords: hepatocellular carcinoma, transarterial chemoembolization, ART score.

Introduction: Cytochrome P450 (CYPs) are enzymes belonging to the family of heme-containing proteins, most commonly found in the endoplasmic reticulum and mitochondria. These enzymes catalyze a variety of functions including metabolism of steroids, fatty acids, natural compounds, drugs and carcinogenic chemicals. The inherent association of CYPs with disease conditions have turned the focus into the genetic alterations or variations associated with phenotypes such as drug responsiveness, chemical toxicity and bioconversion of procarcinogens to active carcinogens.

Materials and Methods: The present observation study utilizes several computational tools to identify and predict the possible outcomes of gene alterations in CYP3 family of genes with head and neck squamous cell carcinoma (HNSCC). cBioportal hosts an exhaustive collection of datasets of various cancers which was the primary source of analysis. Oncoprint data obtained was further analysed using tools such as PROVEAN, I-Mutant and gnomAD.

Results: A total of 8 genes of the CYP3 family were analyzed, among which 4 genes were found to harbour gross abnormalities and variations. The genes CYP3A4, CYP3A5, CYP3A7, CYP3A43 showed a common pattern of gene amplification in a group of patients. Truncating and missense variants were also identified of which rs199908125 of CYP3A4 and rs768530577 of CYP3A5 were reported in different populations.

Discussion: The gnomAD analysis revealed a few polymorphic rare variants with minor allele frequency less than 0.01, which could have a putative association with HNSCC. Five out of eight variants identified were found to be deleterious exhibiting decreased protein stability.

Conclusion: Further screening of the genetic abnormalities through experimental validation in different populations are warranted to derive an association between the gene identifiers and disease phenotype.

Keywords: Cytochrome P450; HNSCC; In silico, mutations, amplification, deletions

Fahimeh Pakravan¹, Niloofar Heydari Salehabad², Fatemeh Karimi², Mehdi Nasr Isfahani³

¹ Department of Oral Medicine, Dental Implants Research Center, Dental Research Institute, School of Dentistry, Isfahan University of Medical Sciences, Isfahan, Iran
² Dental Students Research Committee, School of Dentistry, Isfahan University of Medical Sciences, Isfahan, Iran
³ Department of Emergency Medicine, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran

Background: Oral mucositis is a common complication induced by radiation in head and neck cancer patients. OM can cause pain, dysphagia, dehydration and impaired quality of life. The main objective of this study was to assess the effectiveness of licorice mucoadhesive film on symptoms of head and neck radiotherapy-induced mucositis.

Methods: sixty head and neck cancer patients who have been scheduled to undergo radiotherapy were randomly assigned to receive radiotherapy plus licorice mucoadhesive film (30) or radiotherapy plus placebo mucoadhesive film. In this double blinded randomized clinical trial, oral mucositis was estimated by world health organization scales, a quantitative scale, and symptoms were evaluated by visual analog scale.

Results: The results showed a meaningful difference in pain score between two groups in third and fourth weeks (P-value <0.05). Also, there was a significant difference in scale of mucositis between the two groups in weeks 3 and 4 (P-value <0.05). However, there were no meaningful differences with regard to pain and scale of mucositis in weeks 0, 1, 2. (P-value>0.05)

Conclusion: This study showed that licorice mucoadhesive film can be effective in decreasing pain and level of radiation-induced mucositis and it could be administered as an alternative agent in OM management.

Keywords: Oral mucositis, Radiotherapy, Licorice, Mucoadhesive film, Placebo.

Ahmed Abu-Zaid^1,2,3, Osama Alomar^1,2, Ahmed Nazer² Hany Salem^1,2 Tarek Amin³ Ismail A. Al-Badawi^1,2

¹ College of Medicine, Alfaisal University, Riyadh, Saudi Arabia
² Department of Obstetrics & Gynecology, King Faisal Specialist Hospital & Research Centre, Riyadh, Saudi Arabia
³ King Faisal Oncology Centre, King Faisal Specialist Hospital & Research Centre, Riyadh, Saudi Arabia

Objectives: To report our pilot experience (feasibility, morbidity and postoperative outcomes) of cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) for the treatment of patients with recurrent ovarian cancer and peritoneal carcinomatosis.

Methods: Thirty nine patients were retrospectively analyzed for perioperative details.

Results: The vast majority of patients had platinumsensitive disease (69.2%). Complete (CC-0) and incomplete (CC-1/2) resections were achieved in 24 (61.5%) and 15 (38.5%) patients, respectively. The median peritoneal cancer index (PCI) was 14 (range: 2-28). Cisplatin (50 mg/m2) and doxorubicin (15 mg/m2) was the most frequently used HIPEC regimen (87.2%). No intraoperative morbidity/mortality happened. A total of eight patients developed III-IV postoperative complications (20.5%). Median follow-up time was 41 months (range: 3-106). No 60-day readmission/mortality happened. At the last date of follow-up, there were 13 patients who were alive without disease (33.4%); mortality occurred in 10 patients (25.6%). For all patients, the mean diseasefree survival (DFS) and overall survival (OS) were 46.3 months (95% CI: 33.7–58.9) and 81 months (95% CI: 68.6–93.3), respectively. PCI >14 was correlated with statistically significant poor DFS and OS at univariate analysis (p=0.046). When compared to CC-0, CC-1/2 was correlated with poor DFS and OS, however, without statistical significance. Cox multivariate analyses of DFS and OS failed to demonstrate PCI score, CC score and platinum-sensitivity as independent prognostic factors of DFS and OS.

Conclusion: Our study demonstrated the feasibility, safety and favorable clinical outcomes of CRS and HIPEC in patients with recurrent ovarian cancer and peritoneal carcinomatosis.

Keywords: Cytoreductive surgery; hyperthermic intraperitoneal chemotherapy; peritoneal carcinomatosis; ovarian cancer; recurrent; Saudi Arabia

Suad Al Kharusi¹, Bahaaeldin Baraka¹, Laila Al Balushi¹, Mahmoud Nassar²

¹ National oncology center - Royal Hospital, Muscat, Oman
² Icahn school of medicine at Mount Sinai / Queens, New York, USA

Introduction : Breast cancer is the commonest cancer amongst females. The incidence of breast cancer is estimated to be around 260K yearly. Oral hormonal medication is an essential part of the management of breast cancer for hormone receptor-positive patients. Adjuvant hormonal medication is recommended to be taken daily for 5-10 years. Adjuvant hormonal medication reduces mortality by 30% and the recurrence rate in receptor-positive patients.

Patients and Methods: This study’s primary goal is to evaluate the rate of nonadherence to Endocrine Therapy for hormone receptor-positive breast cancer patients at Oman National Oncology Center. This study included patients taking hormonal therapy (either with Tamoxifen or Aromatase inhibitor) and presented for regular followup between June 2019 and February 2020 at the National oncology center, Oman. Data was collected using a written questionnaire. Descriptive analysis was done by using SPSS. A cross-sectional descriptive study for patients taking oral hormonal therapy. 131 patients were included.

Results: One hundred thirty-one patients were included, Tamoxifen was used by 73 (55.73%). 71 (54%) of breast surgery was “WLE.” The majority of patients 95 (72.5%) did not identify a specific reason for non-compliance. The most commonly reported adverse effects were musculoskeletal symptoms by 75 patients (57.3%), with other reported side effects included hot flashes (33.6%), anxiety (30.5%), gynecological toxicity (29.8%), decreased concentration (19.1%), neurological symptoms (16%), and depression (9.9%).

Discussion: We reported that patients with hormone receptor-positive breast cancer have a high adherence rate to the medication than developing countries; selfreported non-compliance to oral hormonal medication is 41.22% below the average of non-compliance to chronic disease therapy of developing countries as WHO report. Medical insurance, unemployment, or drug cost is not a cause for non-compliance to medication.

Conclusion: The self-reported nonadherence to oral hormonal medication is (41,22%). Most of the patients (72.5%) did not report a specific cause for non-adherent to medication. Close follow-up is recommended increasing compliance to medication.

Keywords: Hormonal, therapy, breast neoplasms, Compliance, Adjuvant Endocrine.

Simrandeep Singh¹, Ratika Gupta², Tejinder paul Singh³, S. L. Jakhar¹, Neeti Sharma¹, H. S. Kumar¹

¹ ATRCTRI, Bikaner/Rajasthan, India² VMMC, Delhi, India³ GMC, Amritsar/Punjab, India

Introduction : The standard treatment for unresectable stage III non-small-cell lung cancer (NSCLC) is concurrent chemoradiotherapy. This study was undertaken to evaluate whether induction chemotherapy along with concurrent chemoradiotherapy would result in better tumor control, improved symptom control and any variation in toxicity as compared to concurrent chemoradiotherapy alone.

Patients and Methods: Between February 2015 to September 2016, 25 patients each were randomized to control group, in which they received concurrent chemoradiotherapy with weekly cisplatin 40 mg/m2 intravenous, during chest radiotherapy of 66Gy in 33 fractions for 6.5 weeks, and study group, in which patients received three cycles of induction chemotherapy with Cisplatin 75 mg/m2 and Paclitaxel 175 mg/m2 administered every 21 days followed by identical chemoradiotherapy.

Results: The two groups of patients (with induction vs. without induction chemotherapy) were similar in age, performance status, histology, grade, and stage. At 6^th month follow-up, complete response was seen in 6 patients in control arm and 7 patients in study arm (?² = 1.603, p = 0.205) and partial response was seen in 13 and 12 patients in control and study arms respectively (?² = 1.932, p = 0.165). Symptom control of cough, hemoptysis, chest pain and dyspnoea were also similar in both groups.

Discussion: In our study, no difference in treatment outcome with respect to the two groups was observed, which was similar to studies which have been conducted previously. Radiation is a good modality for symptom control of cough, hemoptysis, chest pain and dyspnoea. In toxicities, pneumonitis and hematological toxicity was slightly higher in study group even at 6th month follow up.

Conclusion: Slight increase in toxicity with no added benefit in locoregional tumor control and symptom regression, was seen in patients receiving induction chemotherapy followed by chemoradiotherapy. Concurrent chemoradiotherapy alone can thus be used as only modality of treatment in unresectable stage III NSCLC.

Keywords: Non small cell lung cancer, concomitant chemoradiotherapy, locoregional control, toxicity.

Mohammed S Saeed², Taha Al-Lawati¹, Fatma Al Lawati³, Raymond N. Elias⁴

¹ Dept. of Breast Surgical Oncology, Royal Hospital and Muscat Private Hospital, Muscat, Oman.
² Dept. of Pathology, Muscat Private Hospital.
³ Dept. of Pathology Royal Hospital Oman.
⁴ Dept. of Surgery Muscat Private Hospital

Background: Intraoperative evaluation of axillary sentinel lymph node (SLN) in patients with breast carcinoma reduces the need of re-operations for axillary lymph node dissection. Various methods such as touch imprint cytology (TIC) and frozen section histology (FS) have been used to determine the SLN status intra-operatively. The sensitivity of intraoperative TIC examination on SLN is not consistent and varies in different studies. The aim of this study was to determine the specificity and sensitivity of TIC, and its feasibility in clinical use.

Methods: A prospective study was conducted on 24 female and 1 male patients with histologically proven breast carcinoma and an at most clinical stage of cT2N0, between March 2017 and 2020. Axillary lymph nodes were not detected/palpable on physical examination. The patient underwent breast-conserving surgery i.e. quadrantectomy with sentinel lymph node biopsy. The intact lymph nodes were sent to histopathology laboratory for intraoperative TIC. The nodes were bisected, touch smears made and stained using the H&E and May- Grunwald-Giemsa methods. The remaining tissue was processed in formalin fixed paraffin-embedded blocks and the slides were stained with H&E.

Results: In the three- year period (2017– 2020), sentinel lymph node intraoperative touch imprint cytology was performed on 25 patients’ lymph nodes with primary breast cancer and clinically negative axillary lymph nodes in Muscat Private Hospital, Muscat, Oman. The average age of the patients was 54.69 year. SLN- TIC revealed 88.9% sensitivity, 93.75% specificity, 11.1% false negative rate and 6.25% false positive with an overall accuracy 92%.

Conclusion: Touch imprint cytology has high sensitivity and specificity with an accepted accuracy. Intraoperative TIC is practical, time-efficient, and cost-effective procedure requiring minimal tissue preparation for SLN evaluation especially in clinical practice where FS is unavailable. Intraoperative touch imprint cytology can detect macrometastasis and micrometastasis to a lesser extent.

Keywords: breast carcinoma, sentinel node biopsy, intraoperative diagnosis, touch imprint cytology, micrometastasis

Abdulaziz A. Binzaid¹, Omar J. Baqal^2,3, Mohammed Soheib³, Mohammad Al Nahedh⁴, Hadeel H. Samarkandi⁴, Mahmoud Aljurf⁵

¹ Heart Centre, King Faisal Specialist Hospital and Research Centre, Riyadh 11211, Kingdom of Saudi Arabia.
² Department of Internal Medicine, Mayo Clinic Arizona, Phoenix, AZ 85054, USA.
³ College of Medicine, Alfaisal University, Riyadh 11533, Saudi Arabia
⁴ College Pharmaceutical Care Division, King Faisal Specialist Hospital and Research Centre, Riyadh 11211, Kingdom of Saudi Arabia.
⁵ Oncology Center, King Faisal Specialist Hospital and Research Centre, Riyadh 11211, Kingdom of Saudi Arabia.

Abstract: Treatment of Chronic myeloid leukemia (CML) typically entails a long-term course of tyrosine kinase inhibitors (TKI) therapy. This review provides a summary on the cardiotoxic effects of TKIs. Five small molecular TKIs were evaluated in our review. The cardiotoxic effects of TKIs can range from superficial edema to potentially fatal conditions such as congestive heart failure (HF) and acute coronary syndrome (ACS). With the constant introduction of newer generations of TKIs, it has been demonstrated that different TKIs have distinct cardiovascular safety profiles. Amongst which, the first-generation TKI - imatinib appears to have the safest profile, mainly causing edema along with nausea, rash and muscle cramps. Other TKIs, like the second-generation dasatinib, bosutinib, and nilotinib, have shown an increased incidence of pleural effusion and QT prolongation. Ponatinib, a third generation TKI, has shown a relatively high incidence of serious adverse effects including thrombotic vascular occlusion and heart failure, particularly in patients with a prior history of cardiovascular impairment. Therefore, it is advisable that at-risk patients taking TKIs be screened with an Electrocardiogram (ECG) and have a careful cardiovascular risk assessment before starting TKI therapy to avoid potential cardiotoxic effects such as arrhythmias, acute coronary syndrome (ACS), congestive heart failure, and pleural effusion.

Keywords: tyrosine kinase inhibitor, TKI, chronic myelogenous leukemia, CML, cardiotoxicity, side effects, imatinib, dasatinib, bosutinib, nilotinib, ponatinib

Nik Mohd Syahrul Hafizzi Awang^1,2, Ali Haron¹, Baharudin Abdullah²

Background: The parapharyngeal tumour is a rare case of head and neck tumour. Benign and malignant tumours can arise from any of the structures contained within the parapharyngeal space. Pleomorphic adenoma is the most typical salivary gland tumour that is found in this space and surgical resection is the main treatment for this tumour. Since the modern era of the medicine, different kinds of diagnostic imaging and surgical approaches have been introduced to evaluate this tumour.

Case Presentation: We present a case report of a parapharyngeal space mass that was found to be pleomorphic adenoma of minor salivary gland, their clinical presentation, radiological imaging and literature review of the transoral surgical approaches for parapharyngeal tumour.

Discussion and Conclusion: The transoral approach remains one of the classical and effective methods for the resection of the benign prestyloid parapharyngeal tumour. The surgeons need to have a good knowledge of diagnostic imaging and surgical techniques to determine which surgical procedure is the best for the patients in order to eradicate the diseases and minimize the complications ultimately.

Keywords: parapharyngeal tumour, pleomorphic adenoma, salivary gland, transoral approach, prestyloid

Nur Adillah Lamry¹, Khairunnisak Misron², Tengku Kamalden², Sakinah Mohamad¹

¹ Department of Otorhinolaryngology-Head and Neck Surgery, School of Medical Sciences, Universiti Sains Malaysia Health Campus, Kota Bharu, Kelantan.
² 2Department of Otorhinolaryngology Head and Neck Surgery, Hospital Sultan Ismail, Johor Bahru.

Abstract: Soft tissue sarcoma, especially synovial sarcoma is extremely rare in infancy. Only few cases were reported, and challenges lies in disease eradication and subsequent adjuvant therapy. Due to particularly small size of head and neck region with important structures are in intimate location with each other, surgical resection is very challenging, in order to ensure total disease resection as well as maintaining function and cosmetic outcome post-operatively. We present an uncommon case of synovial sarcoma of infratemporal fossa diagnosed in a 3-month-old infant. Due to extreme age, it poses difficulty to the managing team with regards to surgical intervention and oncological regimes.

Keywords: Synovial sarcoma; infratemporal fossa; infant

Reem M. Hersi^1,2, Bashair Y. AlHidri^1,2, Hatim M. Al-Jifree^1,2,3, Mohammad Althobaiti^1,2,4, Hatim Q. Almaghraby^1,2,5

¹ College of Medicine, King Saud Bin Abdulaziz University for Health Sciences, Jeddah, Saudi Arabia
² King Abdullah International Medical Research Center, Jeddah, Saudi Arabia
³ Department of Oncology, Ministry of National Guard Health Affairs, Jeddah, Saudi Arabia
⁴ Department of Medical Imaging, Ministry of National Guard Health Affairs, Jeddah, Saudi Arabia
⁵ Department of Pathology and Medical Labratory, Ministry of National Guard Health Affairs, Jeddah, Saudi Arabia

Abstract: Endometrial Stromal Sarcoma (ESS) are very uncommon malignant tumors that make around 0.2% of the entire uterine related malignancies. They represent the endometrial stromal cells in the proliferative stage. The annual incidence of ESS is 1-2 per million women. We present an unusual case of ESS, which originates from the uterine artery through the Inferior Vena Cava (IVC) and extends to the right atrium. A 48 years old woman presented with menorrhagia and right flank pain for seven months. Physical examination was normal except for a palpable suprapubic mass. Computed tomography (CT) showed the right gonadal vein and IVC tumoral thrombus extending into the right atrium. An echocardiogram and Transesophygeal echocardiogram revealed a large non-mobile echogenic density in the right atrium. Total abdominal hysterectomy with bilateral salpingo-oophorectomy was done. Extensive myometrial and vascular invasion was noted along with the extensive lymphovascular invasion of the uterus. Eventually, distal IVC resections were done, and there was no chance to remove the tumor because it was too attached and invaded the IVC filter. Owing to the unresectability of the tumor, the patient was assigned for palliative chemotherapy.

Keywords: Endometrial Stromal Sarcoma, Right Atrium, Inferior Vena Cava, Spindle Cell Sarcoma.