Nodule Enhancement Research Articles

7085 Myoclonus Seizure with Pseudo-Adrenal Insufficiency as the Clinical Manifestations Leading to the Diagnosed Multi-focal Insulinomas that Mimic Multiple Endocrine Neoplasia Type 1 Syndrome

Abstract Disclosure: P. Kiatpanabhikul: None. Background: Insulinoma is a rare disease that presents with varying symptoms and can be sporadic or associated with hereditary syndromes (1). These rare points of views of insulinoma-patient on clinical manifestations, hormonal responses and surgical pathology result for final diagnosis and management. Clinical Case: A 59-year-old woman presented with myoclonic seizure of right arm with consciousness duration 5-10 minutes every midday twice a week for 6 months and generalized convulsion for 20 minutes ago. She had a low FPG level (59 mg/dL; n 70-100) and was admitted to doing 72-hour fasting test that revealed hypoglycemia at 12-hour of fasting with a nadir glucose level of 55 mg/dL, insulin 3.7 uIU/mL (cutoff > 5), C-peptide 0.4 ng/mL (cutoff > 0.6), cortisol 6.22 mcg/dL (n 5-25) and normal morning ACTH level (29.8 pg/mL; n 10-65). These were appropriated response of endogenous insulinemia with inappropriate response of serum cortisol level in hypoglycemic feature. MRI pituitary gland was considered to evaluate secondary hypocortisolism which showed a small less enhancing lesion left anterior lobe 0.3x0.45-cm without any pituitary hormonal abnormalities that were considered non-functioning pituitary microadenoma which could not explain inappropriate response of cortisol level to hypoglycemic period in this patient. Five months later, she presented at the emergency room with myoclonic seizure on her extremities, blurred consciousness and slurred speech after having lunch for 1-2 hour and revealed CPG 30 mg%, severe hypoglycemia (26 mg/dL), hypocortisolism (4.49 mcg/dL), with endogenous hyperinsulinemia (insulin 55.2 uIU/mL, C-peptide 2.5 ng/mL). 250-mcg ACTH stimulation test was done and showed normal response of her HPA-axis function (serum cortisol level at 0, 30, 60 min was 9.12, 17.87, 18.61 mcg/dL). CT pancreatic protocol showed several arterial enhancing nodules scattering at pancreatic tails isoattenuation to pancreatic parenchyma on portovenous phase up to 1.7*1.6 cm. She was operated on to distal pancreatectomy with intraoperative insulin/glucose ratio monitoring. Surgical pathology confirmed 4-well-differentiated pancreatic neuroendocrine tumors with insulin-producing, sizes 0.5-1.7 cm in diameter. Her final diagnosis was multi-focal insulinomas with non-functioning pituitary microadenoma that mimic MEN-1 syndrome without secondary adrenal insufficiency. She underwent successful surgical resection with complete resolution of symptoms during 4 years of followed-up with no recurrence and no having any hypercalcemia and hyperprolactinemia. Conclusion: Multi-focal insulinomas can be presented as sporadic, more severe hypoglycemia and endogenous hyperinsulinemia during period after meal with myoclonic seizure and pseudo-adrenal insufficiency in older-aged, non-diabetes person without recurrence after treatment for 4-year of followed-up. Presentation: 6/2/2024

A Novel TEK::GAB2 Gene Fusion in Pediatric Angiosarcoma of Pelvic soft Tissue: A Case Report and Literature Review.

Pediatric angiosarcoma of soft tissue, an extremely rare entity, remains poorly understood from a genetic standpoint. Herein, we present the case of a previously healthy 17-year-old girl with acute left hip pain. Subsequent magnetic resonance imaging revealed a 21.8 cm left pelvic sidewall mass with heterogeneous enhancement and multiple lung nodules. Biopsy of the tumor showed an infiltrative, hemorrhagic neoplasm composed primarily of atypical spindle to epithelioid cells. Focal vasoformative architecture was appreciated. Immunohistochemically, the tumor cells were strongly positive for CD31, ERG, and FLI-1, supporting the diagnosis of angiosarcoma. Genetic analysis identified a novel TEK::GAB2 gene fusion. TEK belongs to the angiopoietin receptor family, and its fusion with GAB2 is predicted to mediate tumorigenesis. This report expands the current knowledge on the spectrum of gene rearrangements of angiosarcoma.

EP264 - Dermatofibrosarcoma protuberance of the perianal region

Abstract Introduction Dermatofibrosarcoma protuberans (DFSP) is a rare low-grade sarcoma affecting dermis and subcutaneous fat. Typically presents as slow-growing lump on the trunk, extremities or head and neck.(1) It can be locally aggressive with a high recurrence rate.(2) Only 6 reported cases of DFSP in peri-anal region. Case Description A 26-year-old male presented with slow-growing peri-anal lump for over a year with intermittent localised pain and episodes of bleeding. No other symptoms or trauma to peri-anal area noted. No relevant medical or surgical history. Examination revealed an irreducible two-by-five by one-centimetre semi-pedunculated perianal lump extending from the seven o’clock to eleven o’clock position. It was firm and tethered to the skin. Case Description Flexible sigmoidoscopy was normal. Excision biopsy histology reported diffuse infiltration of spindle cells with storiform pattern of growth without clear margins. Immunohistochemistry analysis expressed CD34 with minimal focal expression of SMA and variable Ki-67 proliferation indexed up to 15-20% in most active areas. SOX10, S100, desmin, factor XIIIA, MNF116 and CD31 were negative. Diagnosis was DFSP. Local lower gastrointestinal multi-disciplinary arranged urgent imaging and referral to tertiary centre. Magnetic resonance imaging of the pelvis identified no definite underlying localised restricted diffusion or solid enhancing nodule. Wide local excision at tertiary centre with histopathology confirmed clear margins. Conclusion Patient is under follow-up locally and at the tertiary. Urgency and a singular episode of stool incontinence was reported at two-weeks follow-up which subsequently normalised. He is currently under 3-6 monthly reviews with repeat MRI prior to next appointment. Patient has returned to normal activities.

Performance of O-RADS MRI Score in Differentiating Benign From Malignant Ovarian Teratomas: MR Feature Analysis for Differentiating O-RADS 4 From O-RADS 2.

The aim of the study is to evaluate the performance of the ovarian-adnexal reporting and data system magnetic resonance imaging (O-RADS MRI) score and perform individual MRI feature analysis for differentiating between benign and malignant ovarian teratomas. In this institutional review board-approved retrospective study, consecutive patients with a pathology-proven fat-containing ovarian mass imaged with contrast-enhanced MRI (1.5T or 3T) from 2013 to 2022 were included. Two blinded radiologists independently evaluated masses per the O-RADS MRI lexicon, including having a "characteristic" or "large" Rokitansky nodule (RN). Additional features analyzed included the following: nodule size/percentage volume relative to total teratoma volume, presence of bulk/intravoxel fat in the nodule, diffusion restriction in the nodule, angular interface, nodule extension through the teratoma border, presence/type of nodule enhancement pattern (solid versus peripheral), and evidence for metastatic disease. An overall O-RADS MRI score was assigned. Patient and lesion features associated with malignancy were evaluated and used to create a malignant teratoma score. χ 2 , Fisher's exact tests, receiver operating characteristic curve, and κ analysis was performed. One hundred thirty-seven women (median age 34, range 9-84 years) with 123 benign and 14 malignant lesions were included. Mean teratoma size was 7.3 cm (malignant: 14.4 cm, benign: 6.5 cm). 18/123 (14.6%) of benign teratomas were assigned an O-RADS 4 based on the presence of a "large" (11/18) or "noncharacteristic" (12/18) RN. 12/14 malignant nodules occupied >25% of the total teratoma volume ( P = 0.09). Features associated with malignancy included the following: age <18 years, an enhancing noncharacteristic RN, teratoma size >12 cm, irregular cystic border, and extralesional extension; these were incorporated into a malignant teratoma score, with a score of 2 or more associated with area under the curve of 0.991 for reviewer 1 and 0.993 for reviewer 2. Peripheral enhancement in a RN was never seen with malignancy (64/123 benign, 0/14 malignant) and would have appropriated downgraded 9/18 overcalled O-RADS 4 benign teratomas. O-RADS MRI overcalled 15% (18/123) benign teratomas as O-RADS 4 but correctly captured all malignant teratomas. We propose defining a "characteristic" RN as an intravoxel or bulk fat-containing nodule. Observation of a peripheral rim of enhancement in a noncharacteristic RN allowed more accurate prediction of benignity and should be added to the MRI lexicon for improved O-RADS performance.

Dynamic contrast enhanced ultrasound in differential diagnosis of hepatocellular carcinoma: A systematic review and meta-analysis.

Non-invasive differential diagnosis between hepatocellular carcinoma (HCC) and other liver cancer (i.e. cholangiocarcinoma or metastasis) is highly challenging and definitive diagnosis still relies on histological exam. The patterns of enhancement and wash-out of liver nodules can be used to stratify the risk of malignancy only in cirrhotic patients and HCC frequently shows atypical features. Dynamic contrast-enhanced ultrasound (DCEUS) with standardized software could help to overcome these obstacles, providing functional and quantitative parameters and potentially improving accuracy in the evaluation of tumor perfusion. To explore clinical evidence regarding the application of DCEUS in the differential diagnosis of liver nodules. A comprehensive literature search of clinical studies was performed to identify the parameters of DCEUS that could relate to histological diagnosis. In accordance with the study protocol, a qualitative and quantitative analysis of the evidence was planned. Rise time was significantly higher in HCC patients with a standardized mean difference (SMD) of 0.83 (95%CI: 0.48-1.18). Similarly, other statistically significant parameters were mean transit time local with a SMD of 0.73 (95%CI: 0.20-1.27), peak enhancement with a SMD of 0.37 (95%CI: 0.03-0.70), area wash-in area under the curve with a SMD of 0.47 (95%CI: 0.13-0.81), wash-out area under the curve with a SMD of 0.55 (95%CI: 0.21-0.89) and wash-in and wash-out area under the curve with SMD of 0.51 (95%CI: 0.17-0.85). SMD resulted not significant in fall time and wash-in rate, but the latter presented a trend towards greater values in HCC compared to intrahepatic cholangiocarcinoma. DCEUS could improve non-invasive diagnosis of HCC, leading to less liver biopsy and early treatment. This quantitative analysis needs to be applied on larger cohorts to confirm these preliminary results.

Abstract PO5-20-09: LOCOREGIONAL MANAGEMENT OF DE NOVO STAGE IV HORMONE-POSITIVE BREAST CARCINOMA AFTER CDK 4/6 INHIBITOR TREATMENT

Abstract Systemic therapy remains the backbone for treatment of metastatic breast carcinoma. Locoregional treatment of the primary tumor remains controversial. While it has an established role in the palliative setting, its survival benefit remains unclear. The rationale behind locoregional approach is the reduction of tumor burden and the removal of cancer stem cells which may propagate the disease. In previous literature, hormone-positive breast cancer seems to be the best candidate for locoregional treatment in the presence of synchronous metastases. We present a case of a 43 year-old, female, with no known comorbidities, who sought consult due to note of a hard lump on her right breast. Core needle biopsy of the right breast mass was performed which showed invasive breast carcinoma, no special type, Nottingham histologic grade 2. On immunohistochemistry, tumor was ER +8, PR +8, Ki 67 90%, Her-2 neu negative. Baseline positron emission tomography/CT (PET/CT) scan showed hypermetabolic, heterogeneously-enhancing lobulated right breast mass, 3 × 6.7 × 7.7 cm, with smaller hypermetabolic adjacent nodules. There was also note of multiple enhancing, hypermetabolic lung and pleural nodules, and lymphadenopathies in the mediastinal, right axillary, right cardiophrenic and perigastric areas. Well-defined, hypermetabolic heterogeneously hypoenhancing masses were also seen in segments II and VII of the liver. There was also note of right-sided pleural effusion. Ultrasound-guided thoracentesis was done which turned positive for malignant cells on pleural fluid analysis. She underwent 4 cycles of chemotherapy with Doxorubicin and Paclitaxel. Reevaluation PET-CT scan revealed partial response. Patient was then started on Ribociclib plus Letrozole with ovarian function suppression for 18 months. On latest reevaluation PET/CT scan, there was no evidence of hypermetabolic metastatic disease, with demonstration of hypermetabolic activity only in the right breast. The patient then underwent right total mastectomy, with histopathologic findings of residual invasive carcinoma, Nottingham histologic grade 3, residual tumor size of 3.5 cm, with 95% tumor cellularity and lymphovascular invasion identified, ypT2. All surgical margins were negative for tumor. Currently, the patient has no evidence of disease, with ribociclib and letrozole continued until disease progression or unacceptable toxicity. In this case, surgery of the primary tumor was performed in the presence of a responsive metastatic disease, with the primary tumor being the only evidence of disease. With the advent of newer therapy options like cyclin-dependent kinase 4/6 inhibitors offering outstanding survival benefit for metastatic disease, a more aggressive and synergistic approach with locoregional therapy may be done to eradicate the tumor burden, translating to better long-term survival. Locoregional management of the primary tumor should be discussed in the context of a multidisciplinary team approach and should be individualized based on the individual patient’s need. Citation Format: Raye Angeli Abella, Rubi Li. LOCOREGIONAL MANAGEMENT OF DE NOVO STAGE IV HORMONE-POSITIVE BREAST CARCINOMA AFTER CDK 4/6 INHIBITOR TREATMENT [abstract]. In: Proceedings of the 2023 San Antonio Breast Cancer Symposium; 2023 Dec 5-9; San Antonio, TX. Philadelphia (PA): AACR; Cancer Res 2024;84(9 Suppl):Abstract nr PO5-20-09.

The influencing factors for tumor thrombus in patients with hepatocellular carcinoma.

Microvascular invasion (MVI) is considered to be an important factor in the early invasion and metastasis of liver cancer, and the survival rate of patients with MVI is much lower than that of patients without MVI. Therefore, it is crucial to accurately predict the independent predictors of tumor thrombus formation. This study aimed to assess the risk factors for tumor thrombus grades in patients with hepatocellular carcinoma (HCC). Between August 2011 and December 2022, the data of 231 patients diagnosed with HCC were collected and divided into the following three groups: an MVI-negative group, an MVI-positive group, and a portal vein tumor thrombus (PVTT) group. Univariate analysis was used to compare the differences between the three groups in terms of clinical features, pathology, and imaging features. Multiple logistic regression analysis was used to analyze the risk factors associated with tumor thrombus grades, and the cutoff value was finally calculated by using the receiver operating characteristic (ROC) curve. The incidence of MVI and PVTT in the patients with HCC were 10.0% and 6.1%, respectively; univariate analysis revealed statistically significant differences in tumor diameter, alpha fetoprotein level, Ki-67 expression level, gender, tumor quantity, arteriovenous fistula, peritumoral enhancement, and satellite nodules among the three groups (P<0.05). Multiple logistic regression analysis showed that Ki-67 expression level, tumor diameter, and peritumoral enhancement were independent risk factors for tumor thrombus grades (P<0.05). The area under the curve (AUC) of Ki-67 expression level and tumor diameter was 0.713 [95% confidence interval (CI): 0.626-0.800] and 0.753 (95% CI: 0.669-0.837), respectively, and the AUC of the combination analysis was 0.805 (95% CI: 0.723-0.888), with a cutoff value of 17.5% and 4.1 cm, respectively (P<0.05). Tumor diameter, Ki-67 expression level, and peritumoral enhancement can be used as independent predictors of tumor thrombus in patients with HCC. The combination of tumor diameter and Ki-67 expression level can further improve diagnostic efficacy.

The Incidences and Related CT Features of Vascular Lake Phenomenon on Angiography Before Chemoembolization.

To elucidate incidence rates of vascular lake phenomenon (VLP) in hepatocellular carcinoma (HCC), intrahepatic cholangiocarcinoma (ICC), hepatic metastasis (HMT) on transarterial angiography before chemoembolization, and to identity CT features predictive for it. A comprehensive evaluation involved 665 subjects for incidence analysis, comprising 527 of HCC, 33 of ICC and 105 of HMT. VLP was characterized as intratumoral contrast material pool persisting late into venous phase. Incidences were cataloged on both super-selective and common hepatic artery angiography. For CT features analysis, a subset of 182 cases were analyzed. Enhancement ratio served as an index for comparative analysis of nodule enhancement degrees. In HCC, incidence of VLP ascertained via super-selective angiography was 13.5%, whereas it as 7.8% on common hepatic artery angiography. Remarkably, no incidences of VLP were recorded in either ICC or HMT cases. On pre-interventional CT, the prevalence of pseudocapsule was statistically greater in VLP group than Non-VLP group (66.6% vs. 37.6%, P = 0.015). The Houndsfield units (HU) of tumors in plain scan (P = 0.007), arterial phase (P = 0.001), venous phase (P = 0.041), arterial phase enhancement ratio (P < 0.001) were statistically higher in VLP group compared to Non-VLP group. Arterial phase enhancement ratio (P = 0.025), presence of pseudocapsule (P = 0.001), HU of tumor in plain scan (P = 0.035) serve as independent risk factors for VLP manifestation. VLP is a distinct angiography phenomenon uniquely associated with HCC. High arterial phase enhancement ratio, presence of pseudocapsule, high HU of tumor in plain scan are independent risk factors for VLP.

Inoperable Optic Pathway Glioma: A Seven-Year-Old Male with &gt;35 Years Overall Survival Following Treatment with Antineoplastons

Optic pathway glioma (OPG) is a low-grade tumor developing along the pre-cortical optic pathways and can involve the optic nerve, optic chiasm, optic tracts, and hypothalamus. Objective: A male child with newly diagnosed and unresectable OPG is presented to 1) review the earliest use of Antineoplastons A10 and AS2-1 for the treatment of brain tumors and 2) demonstrate the efficacy of Antineoplastons in the treatment of OPG. On April 18, 1988, a seven-year-old male child presented to the Burzynski Clinic (BC) with headaches. Based on prior, non-enhanced MRIs and biopsy, an unresectable suprasellar OPG was diagnosed. Antineoplaston therapy was started on a “proof of principle” basis. Tumor response was measured by magnetic resonance imaging (MRI) scans of the brain. Results: A brain MRI, performed on May 31, 1989, demonstrated an enhancing suprasellar nodule measuring 4.37 cm2. On August 24, 1990, brain MRI demonstrated a 0.96 cm2 enhancing nodule, indicating the achievement of a partial response (PR). Following higher dose intravenous (IV) and oral Antineoplastons, brain MRI performed on January 24, 1997, demonstrated a residual 0.4 cm2 enhancing nodule, indicating an enduring PR. All Antineoplaston therapy was discontinued on August 21, 2000. At last follow-up, &gt; 35 years since the start of Antineoplaston therapy, the patient was healthy and showed no evidence of tumor progression. “Proof of principle” Antineoplaston therapy utilized in a seven-year-old male with unresectable OPG produced an objective response (OR) and prolonged overall survival. Antineoplaston therapy is an attractive therapeutic option for children with OPG.

Two Distinct Characteristics of Immune Microenvironment in Human Hepatocellular Carcinoma with Wnt/β-Catenin Mutations

Introduction: Immunotherapy is becoming a promising approach for unresectable-hepatocellular carcinoma (HCC); the anti-tumor response is affected by the tumor microenvironment (TME). Although Wnt/β-catenin mutations are reported to cause non-inflamed phenotype, their role on TME remains controversial. We aimed to clarify the heterogeneity of immunophenotype in HCC with Wnt/β-catenin mutations. Methods: This study includes 152 resected HCCs; mutations in the catenin beta-1, adenomatous polyposis coli, or AXIN1, or AXIN2 genes were defined as Wnt/β-catenin mutations. With hierarchical cluster analyses, TME was classified into inflamed or non-inflamed classes based on the gene expressions associated with T-cell activation. Expression profiles of molecules related to cell differentiation and biliary-stem cell markers were compared between the TME classes to investigate whether differences in tumor traits were associated with TME. Results: Forty of 152 (26.3%) HCCs carried the Wnt/β-catenin mutations. Of these, 33 were classified as non-inflamed (33/40, 82.5%) and 7 as inflamed (7/40, 17.5%). Non-inflamed class was characterized by low number of CD3+, CD4+, and CD8+ cells on immunostaining, and high mRNA expressions of AXIN2 and GLUL, which are involved in the canonical Wnt/β-catenin signaling and hepatocyte differentiation, respectively. Non-inflamed tumors showed higher enhancement on the hepatobiliary-phase of gadolinium-ethoxybenzyl-diethylenetriamine (Gd-EOB-DTPA)-enhanced magnetic resonance imaging (MRI) compared to inflamed tumors. HCCs classified as inflamed class are revealed to have high numbers of CD3+, CD4+, and CD8+ tumor infiltrating lymphocytes on immunostaining. This class is associated with increased expression of anti-epithelial cell adhesion molecule and FOXM1 accompanied by upregulation of genes related to interferon-gamma signaling, dendritic cell migration, regulatory T cells, and myeloid-derived suppressor cell activation and recognized as low enhancement nodule on Gd-EOB-DTPA-enhanced MRI. Conclusion: Heterogeneity of tumor traits and TME was observed in HCC with Wnt/β-catenin mutation. The potential was indicated that tumor traits and TME are determined not only by the activation of the HNF4A but also by FOXM1, both of which are downstream transcription factor of the Wnt/β-catenin signaling pathway.

SAT187 A Case Of CDC73 Mutation Related Primary Hyperparathyroidism With Ectopic Parathyroid Hyperplasia

Abstract Disclosure: T. Wu: None. N. Dhir: None. M. Bollineni: None. Introduction: Primary hyperparathyroidism is the most common cause of hypercalcemia, and most cases are sporadic. However, in 5-10% cases, it may occur as a syndromic and hereditary disease, like Multiple endocrine neoplasia type 1, type 2A, type 4, or CDC73 mutation related hereditary benign or malignant hyperparathyroidism. In patients with severe or early onset primary hyperparathyroidism, screening for genetic etiology should be considered. Case Presentation: A 35-year-old woman with a medical history of hypertension, kidney stones, and post-operative hypothyroidism presented to the endocrinology clinic due to recurrent hyperparathyroidism. She initially had a parathyroidectomy in 2008 and her calcium corrected at that time. She was again diagnosed with hypercalcemia due to primary hyperparathyroidism in 2021. She underwent re-exploration and had a right thyroid lobectomy with removal of three parathyroid glands. Her PTH was still 309 pg/mL (9-77 pg/mL) and calcium was 11.2 mg/dL (8.6-10.5 mg/dL), and she was started on Cinacalcet. The patient continued to have elevated calcium, bone pain, fatigue, and constipation. A 4D CT scan was ordered, which localized activity to a 1.5 cm arterial enhancing nodule in the anterior mediastinum, with intrathymic location. She underwent right video-assisted thoracoscopic total thymectomy with en bloc and the nodule was found to be ectopic parathyroid tissue. Post-operatively, her calcium normalized to 8.6 mg/dL and PTH was undetectable. She underwent genetic testing due to her recurrent hyperparathyroidism, which showed a CDC73 mutation. She is currently on calcitriol and calcium supplements, and serum calcium has been maintained in the low-normal range. Discussion: The CDC73 gene encodes a protein called parafibromin, which acts as a tumor suppressor, prohibiting cells from proliferating. Mutations in this gene are linked to jaw-tumor syndrome, familial isolated primary hyperparathyroidism (FIHP), and parathyroid carcinoma. These three CDC73-related disorders are clinically overlapping and inherited in an autosomal dominant manner. Biallelic mutations of the CDC73 gene are associated with a high risk of parathyroid cancer since the parafibromin protein is usually absent in parathyroid carcinoma and occasionally in atypical adenomas. FIHP tends to have a more severe clinical presentation with earlier onset (younger than 45 years). Additionally, patients with CDC73 related disorders are more prone to developing ossifying fibroma(s) of the jaw, uterine tumors, and various renal lesions. In summary, this case highlights the diagnostic and therapeutic challenges in a patient with persistent primary hyperparathyroidism after a failed second surgery. Genetic testing is vital for early detection, surveillance, and familial genetic counseling in patients with CDC73 related disorders. Presentation: Saturday, June 17, 2023

The efficacy of the new Bosniak classification v.2019 in benign lesions prediction within the higher Bosniak cysts classes

IntroductionIdentification of benign lesions among higher classes of renal Bosniak cysts who are vulnerable to active surveillance instead of surgical approach is still questionable. We aimed in this study to delineate the efficacy of the new Bosniak v2019 classification in benign lesions identification among those cases with higher Bosniak classes in comparison with the final histopathology. MaterialsIn a retrospective review between 2010 and 2021 for patients diagnosed as higher classes Bosniak renal masses was done. Patients’ demographics and radiological data i.e.,: age, gender, and final Bosniak v2019 categorization for class III: (1) Enhancing thick wall/septa >4 mm (III-WS) and (2) Enhancing irregular wall/septa or convex protrusion with obtuse margins <3 mm (III-OP) and for class IV as: (1) Enhancing nodule or convex protrusion with obtuse margins >4 mm (IV-OP) and (2) Enhancing nodule or convex protrusion with acute margins of any size (IV-AP). ResultsA total of 137 patients were included. Bosniak III was identified in 56 patients. Malignancy was detected in 74.5% of resected masses. Among resected Bosniak III cyst, 46.4% were benign histopathologically. Male gender and Bosniak III-OP were independent risks for malignancy among the resected Bosniak III cysts. Conversely, in resected Bosniak IV renal cysts, only 9 of resected masses were benign. In univariate analysis, male gender, absence of multilocular cyst and endophytic masses were predictors for malignancy in resected Bosniak IV cyst. None of the previous predictors was significant in multivariate analysis. ConclusionThe Bosniak subclassification v.2019 can define benign lesions. Bosniak III-OP was an independent risk for malignancy detection among the resected Bosniak III cysts.

PD08-10 HIGHER CLASSES OF BOSNIAK CYSTS: A ROSE WITH THORNS

You have accessJournal of UrologyCME1 Apr 2023PD08-10 HIGHER CLASSES OF BOSNIAK CYSTS: A ROSE WITH THORNS M. Elbaset, Fady Ghobrial, Ali Elmeniar, Rawdy Ashour, Ahmed Elhussien Abolazm, Mohamed Adel, Islam Reda, Rasha Taha Abouelkheir, Mohamed Hussiny, Ahmed Elqaffas, Ahmed Essam, Mostafa Elgamal, Yasser Osman, and Ahmed Mosbah M. ElbasetM. Elbaset More articles by this author , Fady GhobrialFady Ghobrial More articles by this author , Ali ElmeniarAli Elmeniar More articles by this author , Rawdy AshourRawdy Ashour More articles by this author , Ahmed Elhussien AbolazmAhmed Elhussien Abolazm More articles by this author , Mohamed AdelMohamed Adel More articles by this author , Islam RedaIslam Reda More articles by this author , Rasha Taha AbouelkheirRasha Taha Abouelkheir More articles by this author , Mohamed HussinyMohamed Hussiny More articles by this author , Ahmed ElqaffasAhmed Elqaffas More articles by this author , Ahmed EssamAhmed Essam More articles by this author , Mostafa ElgamalMostafa Elgamal More articles by this author , Yasser OsmanYasser Osman More articles by this author , and Ahmed MosbahAhmed Mosbah More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000003239.10AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: In surgically managed Bosniak class III cysts, malignancy could be detected in approximately 50%. While Bosniak IV masses are mostly defined to be malignant (approximately 90%). We aimed in this study to delineate if the new Bosniak v2019 classification can predict benign lesions of higher Bosniak classes preoperatively in comparison with the final histopathology to define masses eligible for active surveillance. METHODS: We performed a retrospective review between January 2010 and January 2021 for patients who were managed by radical nephrectomy (RN) or nephron-sparing surgery (NSS) for preoperatively diagnosed cystic renal masses (≤25% solid enhancing component). This diagnosis was established after CECT or MRI. Patients’ demographics and radiological data included: age, gender, focal thickening, multilocularity, mass diameter, endophytic or exophytic nature, and final Bosniak v2019 categorization for class III as the following: 1) Enhancing thick wall/septa measuring >4 mm (III-WS) & 2) Enhancing irregular wall/septa or convex protrusion with obtuse margins measuring <3 mm (III-OP) and for class IV as:1) Enhancing nodule or convex protrusion with obtuse margins measuring >4 mm (IV-OP) and 2) Enhancing nodule or convex protrusion with acute margins of any size (IV-AP). RESULTS: One hundred thirty-seven patients (101 males and 36 females) with higher-stage Bosniak cystic renal masses were included. The mean age for the entire group was 55.5±11.3 years. Bosniak III and IV were identified in 56 and 81 patients, respectively. RN was done in 39 patients. Also, 74.5% of resected masses (102 renal masses) were malignant. In patients who were diagnosed with Bosniak III cyst preoperatively, almost half of the resected masses (46.4%) had benign nature histopathologically. The incidence of malignancy in Bosniak III-WS was 32.1% while, in Bosniak III-OP was 55.3%. In univariate analysis, male gender, exophytic masses, and Bosniak III-OP are predictors for malignant features at the final histopathology (P=0.008, 0.01, and 0.001, respectively). In multivariate analysis, male gender and Bosniak III-OP were independent risks for malignancy among the resected Bosniak III cysts. On the other hand, in patients with Bosniak IV renal cysts, nine resected masses (11.1%) were benign histopathologically. The incidence of malignancy was 84.7% and 100% in Bosniak IV-OP and AP, respectively. In univariate analysis, male gender, absence of multilocular cysts, and endophytic masses were predictors for malignancy. None of the previous predictors were significant risks for malignancy detection in multivariate analysis. CONCLUSIONS: The new version of the Bosniak sub-classification had an important role in defining benign lesions. Bosniak III-OP were independent risks for malignancy detection among the resected Bosniak III cysts. But in general, the new classification lost its sensitivity in malignant masses detection on Bosniak IV cysts. Source of Funding: none © 2023 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 209Issue Supplement 4April 2023Page: e236 Advertisement Copyright & Permissions© 2023 by American Urological Association Education and Research, Inc.MetricsAuthor Information M. Elbaset More articles by this author Fady Ghobrial More articles by this author Ali Elmeniar More articles by this author Rawdy Ashour More articles by this author Ahmed Elhussien Abolazm More articles by this author Mohamed Adel More articles by this author Islam Reda More articles by this author Rasha Taha Abouelkheir More articles by this author Mohamed Hussiny More articles by this author Ahmed Elqaffas More articles by this author Ahmed Essam More articles by this author Mostafa Elgamal More articles by this author Yasser Osman More articles by this author Ahmed Mosbah More articles by this author Expand All Advertisement PDF downloadLoading ...

Metabolic delineation of IDH1 wild-type glioblastoma surgical anatomy: how to plan the tumor extent of resection.

Magnetic resonance imaging (MRI) is the current standard for preoperative planning of glioblastoma (GBM) surgery. However, recent data on the use of 11C-methionine positron emission tomography (11[C]-MET PET) suggest its role in providing additional information beyond MRI. The purpose of this study is to establish if there is a correlation between anatomical and metabolic data. We retrieved all GBM cases treated from 2014 to January 2021. Preoperative MRI (Enhancing Nodule -EN-, FLAIR and Total Tumor Volume -TTV-), PET volumes and histological samples obtained from the different tumor regions were evaluated to analyze potential correlations between anatomical, metabolic and pathological data. 150 patients underwent surgery for GBM and 49 of these were also studied preoperatively with 11[C]-MET PET; PET volume was evaluated in 47 patients. In 33 patients (70.21%) preoperative 11[C]-MET PET volume > preoperative EN volume and in 11 (23.4%) preoperative 11[C]-MET PET volume > preoperative TTV. We found a significant correlation between preoperative TTVs and PET volumes (p = 0.016) as well as between preoperative EN volumes and PET volumes (p = < 0.001). Histologically, 109 samples were evaluated. ENs samples exhibited the conventional GBM morphology while samples from the FLAIR regions showed white matter tissue, with focal to diffuse tumor cells infiltration and areas of reactive astrogliosis. We submit that 11[C]-MET PET volume generally overcome EN. The presence of neoplastic cells confirm these metabolic data. It should be considered in the surgical planning to achieve a Supra Total Resection (SupTR).

Hepatic Venous Occlusion Type of Budd-Chiari Syndrome versus Pyrrolizidine Alkaloid-Induced Hepatic Sinusoidal Obstructive Syndrome: A Multi-Center Retrospective Study.

(1) Background: Hepatic venous occlusion type of Budd-Chiari syndrome (BCS-HV) and pyrrolizidine alkaloid-induced hepatic sinusoidal obstructive syndrome (PA-HSOS), share similar clinical features, and imaging findings, leading to misdiagnoses; (2) Methods: We retrospectively analyzed 139 patients with BCS-HV and 257 with PA-HSOS admitted to six university-affiliated hospitals. We contrasted the two groups by clinical manifestations, laboratory tests, and imaging features for the most valuable distinguishing indicators.; (3) Results: The mean patient age in BCS-HV is younger than that in PA-HSOS (p < 0.05). In BCS-HV, the prevalence of hepatic vein collateral circulation of hepatic veins, enlarged caudate lobe of the liver, and early liver enhancement nodules were 73.90%, 47.70%, and 8.46%, respectively; none of the PA-HSOS patients exhibited these features (p < 0.05). DUS showed that 86.29% (107/124) of patients with BCS-HV showed occlusion of the hepatic vein, while CT or MRI showed that only 4.55%(5/110) patients had this manifestation (p < 0.001). Collateral circulation of hepatic veins was visible in 70.97% (88/124) of BCS-HV patients on DUS, while only 4.55% (5/110) were visible on CT or MRI (p < 0.001); (4) Conclusions: In addition to an established history of PA-containing plant exposure, local hepatic vein stenosis and the presence of collateral circulation of hepatic veins are the most important differential imaging features of these two diseases. However, these important imaging features may be missed by enhanced CT or MRI, leading to an incorrect diagnosis.

Combining Preoperative Clinical and Imaging Characteristics to Predict MVI in Hepatitis B Virus-Related Combined Hepatocellular Carcinoma and Cholangiocarcinoma

Combined hepatocellular carcinoma and cholangiocarcinoma (cHCC-CCA) is a rare form of primary liver malignancy. Microvascular invasion (MVI) indicates poor postsurgical prognosis in cHCC-CCA. The objective of this study was to investigate preoperative predictors of MVI in hepatitis B virus (HBV) -related cHCC-CCA patients. A total of 69 HBV-infected patients with pathologically confirmed cHCC-CCA who underwent hepatectomy were included. Univariate and multivariate analyses were conducted to determine independent risk factors that were then incorporated into the predictive model associated with MVI. Receiver operating characteristic analysis was used to assess the predictive performance of the new model. For the multivariate analysis, γ-glutamyl transpeptidase (OR, 3.69; p = 0.034), multiple nodules (OR, 4.41; p = 0.042) and peritumoral enhancement (OR, 6.16; p = 0.004) were independently associated with MVI. Active replication of HBV indicated by positive HBeAg showed no differences between MVI-positive and MVI-negative patients. The prediction score using the independent predictors achieved an area under the curve of 0.813 (95% CI 0.717-0.908). A significantly lower recurrence-free survival was observed in the high-risk group with a score of ≥1 (p < 0.001). γ-glutamyl transpeptidase, peritumoral enhancement and multiple nodules were independent preoperative predictors of MVI in HBV-related cHCC-CCA patients. The established prediction score demonstrated satisfactory performance in predicting MVI pre-operatively and may facilitate prognostic stratification.

Xanthogranulomatous Cholecystitis: A Distinctive Subtype of Importance.

A 53-year-old female with a history of hiatal hernia repair presented with right upper quadrant pain and jaundice. Physical examination revealed tenderness of the right upper quadrant. Laboratory tests showed elevated C-reactive protein of 109 mg/L (normal <10) and total bilirubin of 181 μmol/L (normal 0–21). Abdominal computed tomography highlighted a gallstone within the main bile duct and an enlarged gallbladder with heterogenous enhancement and intramural hypoattenuating nodules (Figure A). These radiological features established the diagnosis of xanthogranulomatous cholecystitis (XC).

Newly Diagnosed Glioblastoma: Partial Response and &gt; 27 Years Overall Survival in 37-Year-Old Male Treated with Antineoplastons (Treatment of Glioblastoma with Antineoplastons)

Glioblastoma (GBM), with its very poor prognosis, accounts for 57% of gliomas and 48% of malignant central nervous system (CNS) tumors. After standard therapy, patients with GBM usually die within six months. The case of an adult male with a newly-diagnosed GBM is presented here to detail/discuss the efficacy of ANP therapy (Antineoplaston A10 {Atengenal} and Antineoplaston AS2-1 {Astugenal}) in the treatment of GBM and to permit a review of the Phase II Protocol BT-07. Objectives: This patient was treated at the Burzynski Clinic (BC), according to the Phase II Protocol, BT-07, which utilized IV ANP therapy in the treatment of patients with newly-diagnosed GBMs. ANP therapy was delivered via subclavian catheter and infusion pump. Tumor response was measured by sequential magnetic resonance imaging (MRI) of the brain utilizing gadolinium enhancement. Findings: At age 37, this patient was diagnosed with GBM of the right frontal lobe after subtotal tumor resection performed elsewhere. At age 37 years and six months, he presented to the BC with this newly diagnosed disease. He had a recent history of forgetfulness and personality change. Neurologic examination was otherwise normal. Baseline brain MRI at the BC revealed a measurable enhancing nodule (4.0 cm x 2.0 cm) in the right frontal lobe. Intravenous (IV) ANP therapy began in November 1995 and a partial response (PR) was achieved within three months. After another 5 months of IV ANP therapy, the patient underwent a complete tumor resection, then additional ANP therapy. Now, &gt; 27 years later, the patient is doing well and showing no evidence of tumor recurrence. Conclusions: The utilization of ANP therapy to facilitate a cure in a patient with newly-diagnosed GBM is presented. We conclude that ANP therapy is an attractive therapeutic option for adults with a GBM who are ineligible for or refuse standard therapy.

The small peptide CEP1 and the NIN-like protein NLP1 regulate NRT2.1 to mediate root nodule formation across nitrate concentrations.

Legumes acquire fixed nitrogen (N) from the soil and through endosymbiotic association with diazotrophic bacteria. However, establishing and maintaining N2-fixing nodules are expensive for the host plant, relative to taking up N from the soil. Therefore, plants suppress symbiosis when N is plentiful and enhance symbiosis when N is sparse. Here, we show that the nitrate transporter MtNRT2.1 is required for optimal nodule establishment in Medicago truncatula under low-nitrate conditions and the repression of nodulation under high-nitrate conditions. The NIN-like protein (NLP) MtNLP1 is required for MtNRT2.1 expression and regulation of nitrate uptake/transport under low- and high-nitrate conditions. Under low nitrate, the gene encoding the C-terminally encoded peptide (CEP) MtCEP1 was more highly expressed, and the exogenous application of MtCEP1 systemically promoted MtNRT2.1 expression in a compact root architecture 2 (MtCRA2)-dependent manner. The enhancement of nodulation by MtCEP1 and nitrate uptake were both impaired in the Mtnrt2.1 mutant under low nitrate. Our study demonstrates that nitrate uptake by MtNRT2.1 differentially affects nodulation at low- and high-nitrate conditions through the actions of MtCEP1 and MtNLP1.

TAIL GUT CYST- A RARE FINDING- CASE REPORT AND REVIEW OF LITERATURE

Introduction: Tailgut cysts are rare congenital entity that arises from the embryological hindgut and usually located in presacral space. Here we report a case of tail gut cyst which is an infrequent condition. Case report: Here, we report a nineteen year old male with lower abdominal pain. The relevant investigations were performed. The CT abdomen revealed a well-dened cystic lesion in the pelvis in lower presacral region and posterior to rectum with small calcied foci suggestive of tail gut cyst with scalloping of sacrum. MRI pelvis suggested large well dened unilocular cystic lesion in the retro-rectal and pre-sacral region extending from S2 to mid coccygeal level showing thin imperceptible walls few tiny projections and small rounded non enhancing nodule along its posterior wall with thin focal calcication seen in its wall with no evidence of malignancy and no signs of osseous destruction. The patient was explored and intraoperatively ndings revealed a cyst of size 8 cm * 5 cm behind the rectum and in front of sacrum which was densely adhered to the posterior wall of rectum but free from sacrum. The mass was resected as a whole and sent for histopathological exam which was suggestive of tailgut cyst. Tailgut cysts is infrequent condition which manifests as chronic lower abdominal pai Conclusion: n. It is crucial that such entity be diagnosed and surgically excised early in its presentation. It is important to rule out malignancy in the post operative biopsy