Post Radiation Research Articles

Postradiation Therapy Changes of the Superior Cervical Ganglion.

HomeRadiology: Imaging CancerVol. 5, No. 2 PreviousNext Images in CancerClinical ResearchPostradiation Therapy Changes of the Superior Cervical GanglionLoai Aker , Ryan M. McAllister, Surjith VattothLoai Aker , Ryan M. McAllister, Surjith VattothAuthor AffiliationsFrom the Department of Diagnostic Radiology, Hamad Medical Corporation, PO Box 3050, Qatar (L.A.); and Departments of Diagnostic Radiology (R.M.M.) and Radiology, Neuroradiology Section (S.V.), University of Arkansas for Medical Sciences, Little Rock, Ark.Address correspondence to L.A. (email: [email protected]).Loai Aker Ryan M. McAllisterSurjith VattothPublished Online:Feb 17 2023https://doi.org/10.1148/rycan.220144MoreSectionsFull textPDF ToolsImage ViewerAdd to favoritesCiteTrack CitationsPermissionsReprints ShareShare onFacebookTwitterLinked In

A case report of an ischaemic stroke, caused by a primary cardiac intimal sarcoma

BackgroundIntimal sarcomas are an extremely rare type of primary cardiac malignancy. They most commonly present with symptoms of cardiac dysfunction. We present a case of intimal sarcoma identified without any cardiac signs or symptoms. Cardiac sarcomas historically carry a very poor prognosis.PresentationA 57-year-old man presented with a sudden onset of left limb weakness and disorientation. MRI brain identified an acute ischaemic stroke in the right anterior temporal lobe. Four months later, he presented again with transient left arm weakness. The patient had a normal cardiovascular examination and ECG. All other initial investigations for cryptogenic stroke were non-contributory. The patient did not initially get an echocardiogram. When this investigation was performed, after his second presentation, a large pedunculated mass was present in his left atrium. This was resected and identified histologically as a primary intimal sarcoma of his left atrium. The patient was treated with post-operative radiotherapy but declined chemotherapy. He recovered well post-operatively but subsequently passed away 14 months after diagnosis.ConclusionsIt is possible for primary cardiac malignancies to present with only symptoms of systemic emboli. For this reason, echocardiography is a crucial investigation in cases of cryptogenic stroke. Some stroke guidelines do not definitively support routine echocardiography. Primary intimal cardiac sarcoma is a very rare condition with a poor prognosis. The literature is limited to case reports and optimal management is with surgical resection where possible. The role of post operative radiotherapy and chemotherapy is uncertain.

Canadian Cancer Trials Group HE.1: A phase III study of palliative radiotherapy for symptomatic hepatocellular carcinoma and liver metastases.

LBA492 Purpose: To determine if more patients (pts) with painful liver cancer have improved pain 1 month following radiation therapy (RT), compared to best supportive care (BSC). Methods: This multi-centre, phase III trial randomized pts with painful hepatocellular carcinoma (HCC) or liver metastases (LM) 1:1 to BSC alone or with single fraction RT (8 Gy). Eligible pts had end-stage disease unsuitable for local, regional or systemic therapies, > 4 weeks since chemotherapy or TACE, > 2 weeks since targeted therapy or immunotherapy, and no planned systemic therapy. The primary objective was to determine if the proportion of pts with improved liver cancer pain "intensity at worst" on Brief Pain Inventory (BPI) by ≥2 points from baseline to 1 month was higher following RT vs. BSC alone. Secondary endpoints included proportion of pts 1) alive at 3 months, 2) with improvement ≥2 points in BPI at 1 and 3 months, 3) with a 25% reduction in opioids at 1 month and 4) with improved quality of life (QOL): Functional Assessment of Cancer Therapy-Hepatobiliary (FACT-Hep) hepatobiliary subscale (HBS) change score ≥5 and Trial Outcome Index ≥7 at 1 month. Results: Sixty-six pts were randomized, 43 with LM (12 colorectal, 5 breast, 4 pancreas, 3 lung, 19 other), 23 with HCC. 59% had ECOG performance status 2 or 3, 64% Child Pugh (CP) score A vs. 36% CP B or C. The clinical target volume irradiated was the whole liver or near whole liver (median 2013 cc). Forty-two pts (24 on RT and 18 on BSC) completed baseline and 1-month assessments. The average baseline pain at worst score was 7/10. A significant improvement in the primary endpoint, ‘worst’ pain score on BPI, from baseline to 1 month was seen in 67% of pts on RT and 22% on BSC (p = 0.004). The proportion of pts with improved ‘worst’ pain at 1 month, with no increase in opioid use was 21% on RT vs 0% with BSC (p = 0.07). From baseline to 1 month, the proportion of pts with a significant improvement in BPI “pain at its least” was higher for pts on RT vs. BSC (63% vs. 28%, p = 0.03), as was BPI “percentage relief in pain by treatment” (59% vs. 25%, p = 0.04). A sensitivity analysis of all pts, treating those with no 1 month assessment as having ‘no improvement’, found improvements in BPI ‘worst’ pain in 49% of pts on RT and 12% for BSC alone (p = 0.002). Eleven patients on the BSC arm crossed over to receive RT at 1 month. A trend to improvement in FACT HBS post RT vs BSC was seen at 1 month (p = 0.07), with no statistically significant difference in other FACT-Hep subscales. The proportion of pts with grade ≥2 AE (CTCAEv4.0) at day 30 was 58% on RT vs 33% on BSC (p = 0.05). Grade ≥3 AE were uncommon. There was a trend for improved 3 month survival with RT (51% vs. 33% for BSC alone, p = 0.07). Conclusions: Single fraction RT (8 Gy) improves hepatic pain in the majority of patients with end-stage HCC or liver metastases, with a trend to improved survival. This research is funded by the Canadian Cancer Society (Grant #703547). Clinical trial information: NCT02511522 .

Post Radiation Transformation to a Sarcomatous Meningioma: a Case Report in an Adolescent Male with NF2 Genetic Mutation

Atypical meningioma is often treated adequately by complete surgical resection and subsequent radiation therapy. Nevertheless, radiation therapy should be reassessed in the management of younger patients and particularly in individuals harboring NF2 genetic mutation. To the best of our knowledge this is the third case of post radiation transformation of atypical meningioma in an adolescent male after reviewing the available English literature. Kew wards: Post radiation, atypical meningioma, transformation, sarcomatous meningioma, NF2 mutation.

Identification of single-dose, dual-echo based CBV threshold for fractional tumor burden mapping in recurrent glioblastoma.

Relative cerebral blood volume (rCBV) obtained from dynamic susceptibility contrast (DSC) MRI is widely used to distinguish high grade glioma recurrence from post treatment radiation effects (PTRE). Application of rCBV thresholds yield maps to distinguish between regional tumor burden and PTRE, a biomarker termed the fractional tumor burden (FTB). FTB is generally measured using conventional double-dose, single-echo DSC-MRI protocols; recently, a single-dose, dual-echo DSC-MRI protocol was clinically validated by direct comparison to the conventional double-dose, single-echo protocol. As the single-dose, dual-echo acquisition enables reduction in the contrast agent dose and provides greater pulse sequence parameter flexibility, there is a compelling need to establish dual-echo DSC-MRI based FTB mapping. In this study, we determine the optimum standardized rCBV threshold for the single-dose, dual-echo protocol to generate FTB maps that best match those derived from the reference standard, double-dose, single-echo protocol. The study consisted of 23 high grade glioma patients undergoing perfusion scans to confirm suspected tumor recurrence. We sequentially acquired single dose, dual-echo and double dose, single-echo DSC-MRI data. For both protocols, we generated leakage-corrected standardized rCBV maps. Standardized rCBV (sRCBV) thresholds of 1.0 and 1.75 were used to compute single-echo FTB maps as the reference for delineating PTRE (sRCBV < 1.0), tumor with moderate angiogenesis (1.0 < sRCBV < 1.75), and tumor with high angiogenesis (sRCBV > 1.75) regions. To assess the sRCBV agreement between acquisition protocols, the concordance correlation coefficient (CCC) was computed between the mean tumor sRCBV values across the patients. A receiver operating characteristics (ROC) analysis was performed to determine the optimum dual-echo sRCBV threshold. The sensitivity, specificity, and accuracy were compared between the obtained optimized threshold (1.64) and the standard reference threshold (1.75) for the dual-echo sRCBV threshold. The mean tumor sRCBV values across the patients showed a strong correlation (CCC = 0.96) between the two protocols. The ROC analysis showed maximum accuracy at thresholds of 1.0 (delineate PTRE from tumor) and 1.64 (differentiate aggressive tumors). The reference threshold (1.75) and the obtained optimized threshold (1.64) yielded similar accuracy, with slight differences in sensitivity and specificity which were not statistically significant (1.75 threshold: Sensitivity = 81.94%; Specificity: 87.23%; Accuracy: 84.58% and 1.64 threshold: Sensitivity = 84.48%; Specificity: 84.97%; Accuracy: 84.73%). The optimal sRCBV threshold for single-dose, dual-echo protocol was found to be 1.0 and 1.64 for distinguishing tumor recurrence from PTRE; however, minimal differences were observed when using the standard threshold (1.75) as the upper threshold, suggesting that the standard threshold could be used for both protocols. While the prior study validated the agreement of the mean sRCBV values between the protocols, this study confirmed that their voxel-wise agreement is suitable for reliable FTB mapping. Dual-echo DSC-MRI acquisitions enable robust single-dose sRCBV and FTB mapping, provide pulse sequence parameter flexibility and should improve reproducibility by mitigating variations in preload dose and incubation time.

Dysbiosis of the oral microbiota composition is associated with oral squamous cell carcinoma and the impact of radiotherapy: A pilot study.

Radiotherapy can potentially influence the diversity and composition of the oral microbiome. We performed a study comparing the composition of oral microbiota in patients with oral squamous cell carcinoma (OSCC) before radiotherapy (n = 6), at three months (n = 6), and six months (n = 6) post-radiotherapy, and controls (n = 6). We profiled the oral microbiome by 16S rRNA gene sequencing using Illumina MiSeq. Alpha diversity (Chao1 index) showed significant differences in species richness between healthy controls and OSCC patients (p = 0.014). Conversely, no noteworthy distinctions were observed in the Chao1 index when comparing the pre-and post-radiation periods at both three and six months. The beta diversity of the oral microbiota differed significantly between the controls and OSCC patients (p = 0.014). However, no significant differences were observed in beta diversity between pre- and post-radiation at three months, whereas a significant difference was observed at six months (p = 0.038). Linear Discriminant Analysis Effect Size (LEfSe) demonstrated lower abundance of Corynebacterium, Actinomyces, Veillonella, and Haemophilus, and higher abundance of Selenomonas and Mycoplasma in OSCC patients than in healthy controls. The oral microbiome composition varied among healthy controls, patients with OSCC, and post-radiation therapy patients with OSCC. The observed recovery in the numerical dominance of specific beneficial oral taxa and the reduction in pathogenic bacteria after radiation therapy highlights the need for further investigations into their clinical implications.

Lactobacillus rhamnosus GG ameliorates radiation-induced lung fibrosis via lncRNASNHG17/PTBP1/NICD axis modulation

Radiation-induced pulmonary fibrosis (RIPF) is a major side effect experienced for patients with thoracic cancers after radiotherapy. RIPF is poor prognosis and limited therapeutic options available in clinic. Lactobacillus rhamnosus GG (LGG) is advantaged and widely used for health promotion. However. Whether LGG is applicable for prevention of RIPF and relative underlying mechanism is poorly understood. Here, we reported a unique comprehensive analysis of the impact of LGG and its’ derived lncRNA SNHG17 on radiation-induced epithelial–mesenchymal transition (EMT) in vitro and RIPF in vivo. As revealed by high-throughput sequencing, SNHG17 expression was decreased by LGG treatment in A549 cells post radiation and markedly attenuated the radiation-induced EMT progression (p < 0.01). SNHG17 overexpression correlated with poor overall survival in patients with lung cancer. Mechanistically, SNHG17 can stabilize PTBP1 expression through binding to its 3′UTR, whereas the activated PTBP1 can bind with the NICD part of Notch1 to upregulate Notch1 expression and aggravated EMT and lung fibrosis post radiation. However, SNHG17 knockdown inhibited PTBP1 and Notch1 expression and produced the opposite results. Notably, A549 cells treated with LGG also promoted cell apoptosis and increased cell G2/M arrest post radiation. Mice of RIPF treated with LGG decreased SNHG17 expression and attenuated lung fibrosis. Altogether, these data reveal that modulation of radiation-induced EMT and lung fibrosis by treatment with LGG associates with a decrease in SNHG17 expression and the inhibition of SNHG17/PTBP1/Nothch1 axis. Collectively, our results indicate that LGG exerts protective effects in RIPF and SNHG17 holds a potential marker of RIPF recovery in patients with thoracic cancers.

NUTRITIONAL VALUE ASSESSMENT AND PROXIMATE COMPOSITION OF GAMMA IRRADIATED GRAPES

&#x0D; &#x0D; &#x0D; Grapes (Vitis vinifera L.) belong to the family Vitaceae that are the best source of energy. The present research was focused to increase the shelf life and controlling the amount of fruit losses by optimizing the dose of gamma radiation and proximate analysis post gamma radiation. In the present study, grape samples were exposed to three doses of gamma radiation i.e. 0.5, 0.8 and 1 KGy. Nutritional value of grapes after gamma radiation was analyzed to determine the change in proximate composition. Through experimentation, it was observed that 0.8 KGy was optimized dose as the sensory properties remained unchanged and shelf life of grapes was increased up to 14 days. However the overall effect of gamma radiation enhanced the shelf life with a non-significant effect. Hence, gamma radiation is a promising technique in increasing country’s economy.&#x0D; &#x0D; &#x0D;

Radiation associated angiosarcoma of the breast: a pictorial review of eleven cases highlighting a devastating complication of breast radiotherapy

Radiation Associated Angiosarcoma (RAAS) of the breast is a rare secondary malignancy following breast radiotherapy and associated with considerable diagnostic and therapeutic challenges. We report the experience of one UK breast unit with a case series diagnosed over the last 22 years. Data was prospectively collected on all patients who presented with RAAS from 2001 to 2023, including photographs obtained following formal patient consent. The characteristics of the original breast carcinoma, treatment received, presenting features of the RAAS, subsequent management and patient outcomes were recorded. Eleven patients were identified, all women, with a median age of 57 (range 39–82) at initial breast cancer diagnosis. The dose of breast radiation received varied from 40 to 70 Gy (median 50), including boosts. The commonest presentation of RAAS was with recent-onset skin discolouration at a median time of 70 months (range: 53–168 months) after radiotherapy. Mammograms and ultrasounds were often negative. Diagnosis was confirmed largely by punch biopsy, and 82% (n = 9) went on to have surgery. The median survival from the diagnosis of RAAS was 13 months (range 7–101). RAAS is a devastating complication of breast radiotherapy, with poor prognosis. As it often presents after the traditional 5-year follow-up period for breast cancer, can appear similar to post-radiotherapy changes and be associated with “normal” imaging, so the diagnosis hinges almost entirely on clinical suspicion. Thus, it is important to be aware of the possibility of RAAS in post radiotherapy breast cancer patients.

Robotic right renal autotransplant with venous extension graft and arterial reconstruction

Background: Our patient is a 33-year-old female with a history of cervical cancer, status post pelvic radiation, complicated by ureteral stricture and recurrent urinary tract infections unamenable to cystoscopy.

Depression, anxiety and pain predicted quality of life in patients with differentiated thyroid cancer post radiotherapy ablation in Taiwan

Depression, anxiety and pain predicted quality of life in patients with differentiated thyroid cancer post radiotherapy ablation in Taiwan

REPERCUSSÕES PSICOSSOCIAIS DO TRATAMENTO RADIOTERÁPICO PARA O CÂNCER DO COLO UTERINO: UMA ABORDAGEM QUALITATIVA

ABSTRACT Objective: to understand the psychosocial repercussions of post radiotherapy treatment for cervical cancer. Method: qualitative study, with the participation of patients in post radiotherapy treatment for cervical cancer in an onco-gynecologic nursing outpatient clinic, located in the Municipality of Rio de Janeiro - Brazil. Data were collected during the months of March and April 2019 by means of open interview and submitted to content analysis. Results: four categories were defined: the construct of social roles: the loss of female identity; the sexuality of post-treated women for cervical cancer: marital repercussions; difficulties in adapting to the new life condition: rebuilding the body image; living with cervical cancer: rethinking life and rebuilding values through the support network. Final considerations: the results contribute to the direction of care, increasing the quality of life and helping these women reorganize their professional, social, and family lives.

Role of kali cyanatum 30C in improving post radiation oral mucositis in cases of oral squamous cell carcinoma (Pilot Study)

Role of kali cyanatum 30C in improving post radiation oral mucositis in cases of oral squamous cell carcinoma (Pilot Study)

CLEAR CELL VARIANT OF SQUAMOUS CELL CARCINOMA PENIS – A CASE REPORT

Carcinoma penis is a rare malignancy in males. The World Health Organization Classification of Tumors of the Urinary System and Male Genital Organs classifies squamous cell carcinoma of the penis into human papillomavirus (HPV) and non HPV related. Among the HPV-related Squamous cell carcinomas of the penis, clear cell variant is a rare. Here we report a case of clear cell variant of squamous cell carcinoma of penis with bilateral inguinal lymph node metastasis. We have treated this patient with radical surgery, wide excision and bilateral inguinal lymph node dissection followed by post operative chemo radiation. But during radiation itself the disease progressed, with rapid recurrence of inguinal lymph node metastasis, finally end up with palliative chemotherapy. Clear cell carcinoma is an aggressive form of cancer, with frequent metastasis to the inguinal lymph nodes, as seen in this case. Immunohistochemistry examination P16 was absent. So it is considered as clear cell variant of squamous cell carcinoma. Clinical experience with penile carcinoma is limited because of their rarity.

151: PLANT PATHOGENS CAUSING PERICARDITIS

Introduction: Typical organisms associated with purulent pericarditis include numerous bacteria, such as Staphylococcus aureus, Streptococcus pneumoniae, Mycobacterium tuberculosis, Salmonella sp., and anaerobes, as well as various fungal organisms. We present an unusual case of pericarditis resulting from Burkholderia spp. Description: 79-year-old male with history of esophageal cancer (8 years ago, status post chemotherapy and radiation, currently in remission), esophageal stricture (status post stent placement), atrial fibrillation, arthritis, hypothyroidism, and anemia presented with fatigue, poor oral intake, lethargy, shortness of breath and productive cough. On arrival, he was hypotensive with focused cardiac ultrasound demonstrating a large circumferential pericardial effusion with right ventricular diastolic collapse and arterial line showing pulsus paradoxus on waveform analysis. White cell count was 11.62 k/mm3, hemoglobin 12.7 g/dl, potassium 7.1 mEq/L, bicarbonate 15.8 mEq/L, blood urea nitrogen 158 mg/dl, creatinine 7.79 mg/dl, brain natriuretic peptide 23,141 pg/ml. Computed tomography scan showed large pericardial effusion, bilateral pleural effusions and ascites which was confirmed on transthoracic echocardiogram (along with tamponade physiology). Empiric antibiotics, intravenous fluids and renal replacement therapy were started. Pericardiocentesis was performed (with removal of 200 ml of purulent fluid) and bilateral chest tubes were inserted. Pericardial fluid analysis showed 497,520 white cells/mm3; culture grew pansensitive Burkholderia gladioli/glumae/plantarii; fungal/tubercular cultures were negative, and cytology did not show any malignant cells. Course complicated by shock needing vasopressors and atrial fibrillation with rapid ventricular rate. Given worsening clinical condition, the patient opted for comfort care measures and succumbed to his illness shortly thereafter. Discussion: Burkholderia plantarii/glumae/gladioli are gram negative bacilli which are mainly pathogenic for plants. They have been shown to cause respiratory infections in cystic fibrosis and lung transplant patients. This patient with complex esophageal issues demonstrates an exceedingly rare case of Burkholderia-associated purulent pericarditis resulting from common rice pathogens.

Systematic risk analysis of radiation pneumonitis in breast cancer: role of cotreatment with chemo-, endocrine, and targeted therapy

PurposeA major complication of sequential and concomitant chemoradiation in breast cancer treatment is interstitial pneumonitis induced by radiation therapy (RT), systemic therapy, or a combination of both. Dose and volume of co-irradiated lung tissue directly correlate with the risk of radiation pneumonitis. Especially in case of combined treatment, it is often unclear which of the used therapeutic agents promote pneumonitis.MethodsThis was a prospective monocentric study including 396 breast cancer patients. A systematic analysis of single and combined therapeutic measures was performed in order to identify treatment-related factors enhancing the risk of pneumonitis post RT.ResultsOverall incidence of pneumonitis of any grade was 38%; 28% were asymptomatic (grade 1) and 10% were symptomatic (> grade 1). Pneumonitis > grade 2 did not occur. Beside age, smoking status, and mean lung dose, the combined treatment with goserelin and tamoxifen significantly enhanced the risk of pneumonitis in a supra-additive pattern (odds ratio [OR] 4.38), whereas each agent alone or combined with other drugs only nonsignificantly contributed to a higher pneumonitis incidence post RT (OR 1.52 and OR 1.16, respectively). None of the other systemic treatments, including taxanes, increased radiation pneumonitis risk in sequential chemoradiation.ConclusionCommon treatment schedules in sequential chemoradiation following breast-conserving surgery only moderately increase lung toxicity, mainly as an asymptomatic complication, or to a minor extent, as transient pneumonitis ≤ grade 2. However, combined treatment with tamoxifen and the LHRH analog goserelin significantly increased the risk of pneumonitis in breast cancer patients after chemoradiation. Thus, closer surveillance of involved patients is advisable.

Survey of experiences and barriers to dental care post–head and neck cancer in OraRad study participants

Dental care after head and neck cancer (HNC) treatment is crucial to maintaining oral health and preventing/treating oral complications. This survey investigated the experiences and barriers to dental care post-radiation therapy (RT). Participants of the Clinical Registry of Dental Outcomes in patients with head and neck cancer (OraRad) were surveyed at approximately 4 years post-RT. Participants completed a 20-question survey which assessed perceptions of dental care and education, barriers to receiving care, and ongoing physical symptoms post-RT. One hundred fifty-three of the 505 available OraRad participants completed the survey. Almost all of the respondents (n=141; 92%) either strongly agreed or agreed that they understand the effects of cancer and its treatment on the teeth, mouth, and jaws. The majority (n=119; 80%) strongly agreed or agreed that their dentist provided them with information on how to keep teeth, mouth, and jaws healthy after treatment. Most participants reported dry mouth (n=114; 75%). Other sequelae were problems swallowing (n=57; 38%), dental caries (n=33; 22%), and difficulty keeping their mouth open during dental procedures (n=26; 17%). The OraRad respondents reported few barriers to dental care post-HNC treatment. Patients continue to suffer oral/maxillofacial side effects of radiation treatment, most notably xerostomia.

Role of Radiotherapy in Thyroid-Related Ophthalmopathy: A Case Series

Purpose To explore the role of radiotherapy (RT) in management of moderate to severe thyroid-related eye disease in terms of efficacy, treatment outcome, and response. Material and methods A case series of 5 patients with moderate to severe Graves Ophthalmopathy treated with orbital RT between July 2020 and March 2021. Patient symptoms and ophthalmic findings prior to RT and after 6 months post RT were analyzed. Computed tomography was performed before and after RT to compare orbital (muscles) volume change. Endpoints including improvement in ocular symptoms such as diplopia, proptosis, extraocular movement were compared. Acute and long-term complications were documented to assess its safety and treatment response. Results Addition of RT improved diplopia, visual acuity, proptosis, and color vision significantly. RT also decreased upper lid edema, optic nerve compression, and reduced intraocular muscle bulk. Extraocular muscles diameter reduced significantly. Patients experienced relief of symptoms and significant reduction in diplopia clinically. Conclusion External beam radiation is a safe and effective intervention in thyroid ophthalmopathy. Our study demonstrated that radiation is effective in decreasing inflammatory signs, symptoms, and muscle bulk. This treatment modality is well tolerated, and long-term complications are tolerable.

Malignant peripheral nerve sheath tumor in children: A clinicopathologic and molecular study with parallels to the adult counterpart.

Malignant peripheral nerve sheath tumors (MPNST) are aggressive neoplasms, arising either sporadically, in the setting of neurofibromatosis type I (NF1) or post radiation. Most MPNST occur in adults and their pathogenesis is driven by the loss of function mutations in the PRC2 complex, regardless of their clinical presentation. In contrast, pediatric MPNST are rare and their pathogenesis has not been elucidated. In this study, we investigate a large cohort of 64 MPNSTs arising in children and young adults (younger than the age of 20 years) to better define their clinicopathologic and molecular features. Sixteen (25%) cases were investigated by MSK-IMPACT, a targeted NGS panel of 505 cancer genes. Most patients (80%) were aged 11-20 years. A history of NF1 was established in half of the cases. Mean tumor size was 8.5cm. The most common locations included the extremities (34%) and abdomen/pelvis (27%). Histologically, 89% of high-grade MPNST showed conventional features, while the remaining three cases showed a predominant epithelioid phenotype. Heterologous differentiation occurred in 25% of high grade cases, with half showing rhabdomyoblastic differentiation. Tumors arose in a background of a plexiform neurofibroma (16%), neurofibroma (13%), and schwannoma in two cases (3%). Immunohistochemically, H3K27me3 expression was lost in 82% of conventional high-grade MPNST analyzed, while loss of SMARCB1 expression was seen in one epithelioid MPNST. Genomically, all cases showed more than one genetic abnormality, with 53% showing mutations in EED / SUZ12 genes, and 47% of cases harboring alterations in NF1 and CDKN2A/CDKN2B genes. At the last follow-up, 30% patients died of disease, 28% were alive with disease and 42% had no evidence of disease. NF1 status did not correlate with overall survival. In conclusion, half of pediatric and young adult MPNST were NF1-related and showed loss of function alterations in PRC2 complex, NF1, and CDKN2A, similar to the adult counterpart. Thus, H3K27me3 loss of expression may be used in the diagnosis of high grade MPNSTs in children. Moreover, a small subset of pediatric MPNST have an epithelioid morphology with different pathogenesis.

Glomus jugulare tumors treatment by gamma knife radiosurgery: A single center study.

Glomus jugulare tumor are benign vascular tumors and surgical resection is almost impossible. We have treated these tumors by Gamma knife radiosurgery and share our experience. This study was conducted at the Neurospinal and Cancer Care Institute, Karachi from January 2010 to May 2020. Thirty-four patients with glomus jugulare tumors treated with gamma knife radiosurgery were included in the study. The comprehensive clinical and demographic characteristics of all patients were collected through a manually designed questionnaire. Computed tomography, digital subtraction angiography and magnetic resonance imaging were used to make the diagnosis. Data was incorporated and analyzed by SPSS version 26. A total of 34 patients were included in the study of which 16(47%) were males and 18(53%) were females with first follow up after 6-month up to two year clinical and radiological follow-up. The mean age of the patients was 42.5±13.5 with a minimum age of 20 years and maximum age of 65 years. The KPS scale was 2.09±0.45 and the volume of the tumor was 33.8±22.5 cm3. The improvement was shown in 27 patients of which 14 were males and 13 were females showing insignificance post radiation change. Of all 34 patients, the outcome was recorded as 3(9%) for excellent, 22(64%) for good, 6(17%) for fair and 3(9%) were poor results. Gamma Knife radiosurgery is a safe and effective primary therapy and salvage therapy for residual and recurrent cases of glomus jugulare and tympanicum tumors.