Irregular Borders Research Articles

PD10-06 RADIOLOGIC CORRELATES OF pT3a KIDNEY CANCER: IMPORTANCE OF IRREGULAR TUMOR SINUS BORDER

You have accessJournal of UrologyImaging/Radiology: Uroradiology I (PD10)1 Sep 2021PD10-06 RADIOLOGIC CORRELATES OF pT3a KIDNEY CANCER: IMPORTANCE OF IRREGULAR TUMOR SINUS BORDER Rebecca Campbell, Yunlin Ye, Diego Aguilar Palacios, Alain Rizk, Carlos Munoz-Lopez, Emily Abramcyzk, Gustavo Roversi, Jianbo Li, Robert Abouassaly, Erick Remer, and Steven Campbell Rebecca CampbellRebecca Campbell More articles by this author , Yunlin YeYunlin Ye More articles by this author , Diego Aguilar PalaciosDiego Aguilar Palacios More articles by this author , Alain RizkAlain Rizk More articles by this author , Carlos Munoz-LopezCarlos Munoz-Lopez More articles by this author , Emily AbramcyzkEmily Abramcyzk More articles by this author , Gustavo RoversiGustavo Roversi More articles by this author , Jianbo LiJianbo Li More articles by this author , Robert AbouassalyRobert Abouassaly More articles by this author , Erick RemerErick Remer More articles by this author , and Steven CampbellSteven Campbell More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000001978.06AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: Preoperative assessment of T3a renal cell carcinoma (RCC) in absence of main renal vein involvement or lymph node enlargement is challenging but important for counseling and prognosis. METHODS: Retrospective review of 1129 cT1-T3aN0M0 RCC patients managed with partial/radical nephrectomy (PN/RN) in our institution (2012-2014) was performed. Exclusion criteria included inadequate imaging and radiological evidence of main renal vein involvement or substantial lymphadenopathy (>1.5 cm). Eleven findings were assessed for correlation with pT3a status, including perinephric findings (stranding, enhancing nodule, collateral vessels, or irregular perinephric tumor contour), findings within the sinus (stranding, collecting system invasion, branch vein enlargement, or irregular tumor sinus border [ITSB]), tumor necrosis, infiltrative features, and tumor size. Studies were reviewed blinded to final pathology. Sensitivity/specificity and logistic regression analyses assessed the performance of each imaging finding for detecting pT3a tumors. RESULTS: Median tumor size was 4.0cm and R.E.N.A.L. was 8. Median follow-up was 53 months (IQR:28-64). pT3a tumors were found in 281 patients (25%) and strongly correlated with local and systemic recurrence (p <0.02). ITSB was found in 350 patients (31%) and was the strongest predictor of pT3a status. Sensitivity/specificity/PPV/NPV/OR/C-Index for ITSB were 75%/84%/61%/91%/15.8(11.4-21.9)/0.80, for correlation with pT3a, respectively. The best predictive model included ITSB and tumor size as a continuous variable (C-index=0.84). Addition of other imaging findings did not improve the model (C-index=0.84). ITSB was the strongest contributor in all multivariable models and also strongly correlated with recurrence-free survival. Inter/intraobserver correlations for assessment of ITSB were 0.89/0.98, respectively. CONCLUSIONS: Our data suggest ITSB and tumor size can predict pT3a RCC, which could impact patient-counseling. ITSB is a promising, reproducible imaging parameter for prediction of pT3a RCC. Source of Funding: None © 2021 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 206Issue Supplement 3September 2021Page: e128-e129 Advertisement Copyright & Permissions© 2021 by American Urological Association Education and Research, Inc.MetricsAuthor Information Rebecca Campbell More articles by this author Yunlin Ye More articles by this author Diego Aguilar Palacios More articles by this author Alain Rizk More articles by this author Carlos Munoz-Lopez More articles by this author Emily Abramcyzk More articles by this author Gustavo Roversi More articles by this author Jianbo Li More articles by this author Robert Abouassaly More articles by this author Erick Remer More articles by this author Steven Campbell More articles by this author Expand All Advertisement Loading ...

A practical approach to prenatal diagnosis of malformations of cortical development

<h2>Abstract</h2> Malformations of cortical development (MCD) can frequently be diagnosed at multi-disciplinary Fetal Neurology clinics with the aid of multiplanar neurosonography and MRI. The patients are usually referred following prenatal sonographic screening that raises the suspicion of a possible underlying MCD. These indirect findings include, but are not limited to, ventriculomegaly (lateral ventricles larger than 10 mm), asymmetric ventricles, commissural anomalies, absent cavum septum pellucidum, cerebellar vermian and/or hemispheric anomalies, abnormal head circumference (microcephaly or macrocephaly), multiple CNS malformations, and associated systemic defects. The aim of this paper is to suggest a practical approach to prenatal diagnosis of malformations of cortical development utilizing dedicated neurosonography and MRI, based on the current literature and our own experience. We suggest that an MCD should be suspected in utero when the following intracranial imaging signs are present: abnormal development of the Sylvian fissure; delayed achievement of cortical milestones, premature appearance of sulcation; irregular ventricular borders, abnormal cortical thickness (thick, thin); abnormal shape and orientation of the sulci and gyri; irregular, abnormal, asymmetric, and enlarged hemisphere; simplified cortex; non continuous cortex or cleft; and intraparenchymal echogenic nodules. Following the putative diagnosis of fetal MCD by neurosonography and MRI, when appropriate and possible (depending on gestational age), the imaging diagnosis is supplemented by genetic studies (CMA and trio whole exome sequencing). In some instances, no further studies are required during pregnancy due to the clear dire prognosis and then the genetic evaluation can be deferred after delivery or termination of pregnancy (in countries where allowed).

Epithelioid Leiomyosarcoma of the Uterus: Modern Outcome-based Appraisal of Diagnostic Criteria in a Large Institutional Series.

Epithelioid leiomyosarcoma of the uterus is rare and poorly understood. Herein, we characterize a large institutional series of epithelioid leiomyosarcomas aiming to define outcome-determinant diagnostic pathologic features. We also retrieved epithelioid smooth muscle tumors of unknown malignant potential and evaluated a consecutive cohort of leiomyomas for epithelioid subtypes. Of a total of 1177 uterine leiomyosarcomas, 81 (7%) were categorized as epithelioid after review. Epithelioid leiomyosarcoma was strictly defined as having round to polygonal cells with visible pink cytoplasm and round to ovoid nuclei in ≥50% of the tumor volume. Average age was 55 years (range: 26 to 81 y). Median tumor size was 11 cm; tumor was >5 cm in 93% of subjects; 47% were stage 1 at presentation. An infiltrative tumor border was observed, grossly and/or microscopically, in 89% of cases; necrosis was noted in 80%, and vascular invasion in 47%. Mitotic count in 2.4 mm2 (totalling 10 high-power fields, each field 0.55 mm in diameter) ranged from 3 to 100 (median: 26). All cases had moderate, severe or highly pleomorphic atypia. All cases had 2 or 3 of the following: necrosis, at least moderate atypia and ≥4 mitoses in 2.4 mm2. Immunohistochemistry revealed frequent expression of smooth muscle markers including SMA (96%), desmin (95%), and caldesmon (81%). HMB45 and Melan-A were negative in 92% and 100% of cases, respectively. Estrogen and progesterone receptors were expressed by 65% and 54% of tumors, respectively. Follow-up information was available in 68 subjects (median: 23 mo, range: 1 to 254); cancer-related death occurred in 63%, and an additional 15% had recurrent or metastatic disease at last follow-up. Disease-specific survival was shorter in epithelioid leiomyosarcoma patients (median: 44 mo; 35% at 5-y) than in a matched cohort of nonepithelioid leiomyosarcoma (median: 55 mo; 46% at 5-y) (P=0.03). Three epithelioid smooth muscle tumors of unknown malignant potential were evaluated, all <5 cm in size and with atypia and/or irregular borders but mitotic count below the threshold for malignancy. Two of these had follow-up available, which was uneventful. Of 142 consecutive leiomyomas assessed, none had epithelioid morphology as defined. Epithelioid leiomyosarcoma is an aggressive neoplasm, sometimes with a remarkably low mitotic count. In the setting of an epithelioid smooth muscle tumor of the uterus, we postulate that the diagnosis of malignancy is made in the presence of ≥2 of the following: moderate or severe atypia, ≥4 mitoses/2.4 mm2 and tumor cell necrosis. In their absence, the finding of tumor size ≥5 cm, vascular invasion, infiltrative edges or atypical mitoses should be treated with caution, and designation as of at least uncertain malignant potential is warranted.

Evaluation of pterygium severity with en face anterior segment optical coherence tomography and correlations with in vivo confocal microscopy

To describe en face anterior segment optical coherence tomography (EF-OCT) characteristics of pterygia and their correlation with in vivo confocal microscopy (IVCM). In this observational case series, we prospectively included 21 eyes of 17 subjects with pterygium. All subjects underwent detailed ophthalmic examination, anterior segment photography, an ocular surface disease index (OSDI) questionnaire, IVCM, and EF-OCT. Eyes were divided into two groups according to pterygium severity (Modified Pterygium Classification System) and OSDI score. EF-OCT images for both groups were analyzed for surface area of Fuchs Patches (FP). The IVCM activity score was based on the number of inflammatory cells, blood vessels, activated keratocytes and the appearance of the cornea/pterygium at the head of the pterygium. The correlations between EF-OCT and IVCM images were then analyzed and compared in both groups. EF-OCT permits clear visualization and evaluation of FPs and the border between the pterygium and the adjacent cornea. The severe pterygium group was characterized by irregular borders and larger FPs (0.13±0.06 mm2 versus 0.06±0.02 mm2 respectively) (P=0.003). The mean IVCM activity score was 2.36±0.81 in the severe pterygium group and 1.2±0.42 in the mild pterygium group (P=0.0013). There was a positive correlation between FP surface area and IVCM activity score. A larger FP surface area was associated with a higher activity score on IVCM. EF-OCT allows good evaluation of pterygium extension, borders and FP surface area. EF-OCT analysis of pterygium could represent a simple, non-invasive and reproducible method to evaluate pterygium severity and activity.

Chromoblastomycosis evolving to sarcomatoid squamous cell carcinoma: A case report.

Chromoblastomycosis (CMB) is a cutaneous fungal infection caused by dematiaceous fungi. According to the World Health Organization CMB has been elected as a tropical disease, and it is prevalent in tropical and subtropical regions. The lower extremities are the most affected areas, and the lesions progress with erythema, papules, nodules, verrucose plates and/or ulcerations. So far, few works have demonstrated neoplastic transformation in chronic CMB lesions, and it may be a consequence of prolonged inflammatory response. In the present case report, we described a neoplastic transformation from CMB lesion of a 55- year-old man, presenting lesions only in the left leg for 35 years. After treatment, a verrucous white plate with thick and irregular borders emerged in the ankle, which was identified as a sarcomatoid squamous cell carcinoma. The present case report highlights the importance of an early diagnosis and treatment.

Clitoral HOOD granulosa cell tumor; case report and review of literature

Background: Granulosa Cell tumors (GCT) are a sex-cord stroma tumor comprising of 1-2% of ovarian malignancies. Derived from Schwann cells, GCTs are comprised of granulosa cells, a cell of the ovarian stroma. Patients with these tumors present with signs of increased estrogen such as vaginal bleeding, irregular menstruation and rarely hirsutism and virilization.8,10-12 The majority of GCTs are found in the skin, subcutaneous tissue and submucosa with a handful of case reports discussing primary GCTs in the clitoris.3,7 Surgery is required for definitive tissue diagnosis and staging. In this case report, we present a case with tissue diagnosis for granulosa cell tumor found on the clitoral hood. Case: A 56-year-old, female, G6P3033 initially presented for evaluation of persistent clitoral cyst for &gt;2 years Initial evaluation was significant for 1 cm clitoral cyst that was firm, smooth and fluctuant with no irregular borders; with worsening vulvodynia. The patient underwent clitoral hood mass excision. The mass was excised entirely and the post-operative recovery was uncomplicated. Pathology findings were significant for completely excised granulosa cell tumor; margins were negative for tumor. Immunohistochemical stain was positive for CD56, CD68, Vimentin, and S-100, and negative for AE1/13, CD31, CD34, SMA, and Desmin. Conclusion: When evaluating chronic, persistent, or recurrent masses, it is important to take into consideration both benign and malignant causes. Definitive diagnosis is established pathologically. Patients with confirmed GCT should be thorough evaluated pre-operatively with blood work and appropriate imaging. Upon diagnosis, patient with GCT should be regularly followed for recurrence and surveillance.

Cytology of peritoneal implants of borderline serous tumor of ovaries in ascitic fluid.

Objectives:Peritoneal fluid cytology is done routinely in cases with serous carcinoma of ovary. However, morphologic features of borderline serous tumors (BSTs) of ovary in ascitic fluid have been rarely described. The aim of our study was to evaluate the morphologic features of BST with and without ascitic fluid involvement (BST+ and BST-, respectively) and compare with those of serous carcinomas, both in conventional and liquid-based cytology (LBC) smears.Material and Methods:Out of 30 BST cases reported in 3 years, seven cases had BST+. We compared the cytomorphology of seven cases of BST+, seven cases of BST-, and seven cases of serous adenocarcinoma with positive ascitic fluid cytology. Both conventional and LBC smears were studied in all cases and compared. Histopathology of omentum in these cases was also studied.Results:Most cases with BST+ had regular papillary fragment borders with nuclei showing mild-to-moderate pleomorphism, fine nuclear chromatin with small nucleoli as compared with serous carcinomas all of which had irregular borders with moderate-to-severe nuclear pleomorphism, coarse chromatin, and macronucleoli.Conclusion:A combination of cytoarchitectural and nuclear features can help in suspecting BST in ascitic fluid. Ascitic fluid cytology together with tissue histology can increase the rate of the detection of peritoneal implants.

Resveratrol suppresses cholestasis-induced liver injury and fibrosis in rats associated with the inhibition of TGFβ1-Smad3-miR21 axis and profibrogenic and hepatic injury biomarkers.

Cholestasis caused by slowing or blockage of bile flow is a serious liver disease that can lead to liver fibrosis and cirrhosis. The link between transforming growth factor beta 1 (TGFβ1), Smad family member 3 (Smad3), and microRNA 21 (miR21) in bile duct ligation (BDL)-induced liver fibrosis in the presence and absence of the anti-inflammatory and antioxidant compound, resveratrol (RSV), has not been previously studied. Therefore, we tested whether RSV can protect against BDL-induced liver fibrosis associated with the inhibition of the TGFβ1-Smad3-miR21 axis and profibrogenic and hepatic injury biomarkers. The model group of rats had their bile duct ligated (BDL) for 3weeks before being killed, whereas, the BDL-treated rats were separated into three groups that received 10, 20, and 30mg/kg RSV daily until the end of the experiment. Using light microscopy and ultrasound examinations, we documented in the BDL group, the development of hepatic injury and fibrosis as demonstrated by hepatocytes necrosis, bile duct hyperplasia, collagen deposition, enlarged liver with increased echogenicity, irregular nodular border and dilated common bile duct, which were more effectively inhibited by the highest used RSV dosage. In addition, RSV significantly (p≤0.0027) inhibited BDL-induced hepatic TGFβ1, Smad3, miR21, the profibrogenic biomarker tissue inhibitor of metalloproteinases-1 (TIMP-1), malondialdehyde (MDA), interleukin-17a (IL-17a), and blood levels of alanine aminotransferase (ALT), aspartate aminotransferase (AST), and bilirubin. These findings show that RSV at 30mg/kg substantially protects against BDL-induced liver injuries, which is associated with the inhibition of TGFβ1-Smad3-miR21 axis, and biomarkers of profibrogenesis, oxidative stress, and inflammation.

A Concept of "Boundary Determination" by the Combination of a Local Flap and Free Tissue Transfer Useful for the Prevention of Postoperative Complications After Complex and Widespread Skull Base Reconstruction.

The transplanted tissue is sutured to the stumps of the bones in skull base reconstruction. Concerning reconstruction by free tissue transfer, it is difficult that the tissue is accurately sutured/fixed to a complex three-dimensional structure. The authors devised a technique in which free tissue transfer is combined with a local flap. A pericranial flap is sutured/fixed onto the irregular border between the cranial and nasal cavities. A free tissue graft is transplanted cranial or caudal to the determined border. Clinical presentation 1 is a patient with carcinoma of the frontal sinus (T4bN0M0). Resection of the frontal bone/nasal bone/medial and superior walls of the orbit, frontal lobectomy was performed. Clinical presentation 2 is a patient with carcinoma of the maxillary sinus (T4aN0M0). Extended total maxillectomy, simultaneous resection of the facial skin/temporal muscle were performed. There were no serious postoperative complications related to compression or occlusion of the cerebral parenchyma or paranasal sinus.

Contradictory migration management? Differentiated security approaches to visa overstay and irregular border crossings in the European Union

ABSTRACT While the construction of migration as a security threat in Europe has been thoroughly examined, how different groups of migrants become targets of security concerns has not received similar attention. In its fight against irregular immigration, the European Union uses visa liberalisation agreements with neighbouring states as an incentive for cooperation on migration control. At a first glance, this strategy appears somewhat contradictory, as visa liberalisation potentially increases the share of visa overstayers among irregular migrants. Through analysis of the annual “Risk Analysis” reports between 2015 and 2020 published by EU’s border and migration management agency, FRONTEX, this article shows that visa overstay is routinely left out of the agency’s security concerns of irregular migration, thus rendering risk assessments asymmetrically occupied with irregular migration by means of “illegal entry”. Although visa overstayers are not conceptualised as threats to security in discourse on par with other categories of irregular migrants, we find that they are increasingly subjected to a rationale of surveillance and risk.

Squamous cell carcinoma during long term hydroxyurea treatment: A case report.

BackgroundHydroxyurea (HU) is a ribonucleotide diphosphate reductase inhibitor that interferes with the S phase of cell replication and inhibits DNA synthesis, with limited or no effect on RNA or protein synthesis. The cutaneous side effects of hydroxyurea treatment are diverse and frequent. Squamous cell carcinoma is one of the most challenging side-effect.Case presentationThe authors report the case of a healthy 59-year-old woman on long-term therapy with Hydroxyurea 500 mg daily for essential thrombocytosis, presented with a painless slow-growing lesion of the jaw that had persisted and increased in size for six months, the appearance of the lesion is correlated to the administration of the hydroxyurea treatment. Clinical examination revealed a large nodular lesion 4 × 4 cm with irregular borders of the right cheek, infiltrated into underlying tissue, the lesion extending to the free border of the right lower eyelid without sensory disturbances or diplopia. The surgery was indicated. The surgical procedure had the aim of the restoration of the anatomic landmarks after a large excision of the tumor and reconstruction of full-thickness eyelid defect with a local flap under general anesthesia.ConclusionMaxillofacial surgeons must be aware of the side effects of hydroxyurea including facial cancer, ulceration, etc. Rigorous follow-up of patients on hydroxyurea is required.

THE CHALLENGING DIAGNOSIS OF SERONEGATIVE ORAL SYPHILIS IN AN HIV-INFECTED PATIENT

<h3>Background</h3> In people living with HIV/syphilis-coinfection, overlapping of clinical stages and misleading anti-treponemal serologic tests have been described. This is the report of serological (false-negative) non-reactive, polymerase chain reaction (PCR)-confirmed syphilis in an HIV-infected patient. <h3>Case Description</h3> An HIV-infected male patient, 28-years-old, receiving antiretrovirals (TDF/FTC/DTG) with therapeutic success (CD4: 667 cells/mm³, undetectable HIV-plasma viral load), was referred to our Oral Pathology & Medicine Service in Mexico City. A 3-month asymptomatic ulcerated lesion on the right side of the soft palate and anterior pillar, with granular appearance, irregular borders, and measuring 2 to 3 cm in diameter, was observed. A painless, right cervical lymph node was present. A quantitative serum Venereal Disease Research Laboratory (VDRL) assay was nonreactive (serum was diluted 6-fold to rule out a prozone phenomenon and remained negative). Histopathologic analysis revealed an intense mixed inflammatory infiltrate; differential diagnosis included secondary syphilis, lymphoproliferative disorder, and deep mycotic infection. The immunohistochemistry (IHC) for <i>T. pallidum</i> was negative. DNA extraction was performed, and the <i>T. pallidum</i> gene polA was amplified by PCR; as a consequence, the patient received benzathine-penicillin G (2.4 million units) single dose. VDRL and treponemal FTA-abs were done twice, with negative results. A new biopsy revealed positive IHC<i>-T. pallidum</i>. Two months later, a mucous patch appeared on the tonsillar pillar, with histopathologic features of secondary syphilis and positivity for IHC-<i>T. pallidum</i>. VDRL and FTA-abs assays remained non-reactive. Oral lesions resolved after 3 doses of benzathine-penicillin G. <h3>Conclusions</h3> In absence of serological evidence, the diagnosis of oral syphilis is a challenge. PCR and IHC may represent supplementary helpful diagnostic tools.

Quantitative cytomorphological comparison of SurePath and ThinPrep liquid-based cytology using high-grade urothelial carcinoma cells.

In The Paris System for Reporting Urinary Cytology (TPS), the important cytomorphological features for diagnosing high-grade urothelial carcinoma (HGUC) are a nuclear-to-cytoplasmic (N:C) ratio exceeding 0.7, hyperchromasia, coarse chromatin, and irregular nuclear borders. However, quantitative cytomorphological assessments of HGUC cells using SurePath slides are rare. Therefore, we evaluated HGUC cells on SurePath slides quantitatively using a digital image analysis system and compared these data with ThinPrep data. The same urine samples were divided into two aliquots and used to prepare SurePath and ThinPrep slides. We used ImageJ to measure the N:C ratio, hyperchromasia, and irregular nuclear borders for HGUC cells on SurePath and ThinPrep slides. The total number of analysed HGUC cells on SurePath slides was 981, versus 889 on ThinPrep slides. Hyperchromasia and irregular nuclear borders were significantly more severe on SurePath than on ThinPrep slides. Conversely, the N:C ratio did not differ between the methods. Additionally, HGUC cells with N:C ratios exceeding 0.7 were present on almost all slides for both methods. Our data indicated the reasonableness of using the N:C ratio as the major criterion for TPS on both SurePath and ThinPrep slides, and an N:C ratio cut-off of 0.7 as suitable for identifying HGUC cells. However, the severity of hyperchromasia and irregular nuclear borders differed between the processing methods.

Post Natal Gross Anatomical Studies on the Harderian Gland of Pati Duck (Anas platyrhynchos domesticus) of Assam

Background: The agro-climatic condition of Assam is suitable for duck rearing. Duck rearing contributes a major part to uplift the socio-economic condition of the rural poor people of Assam as well as others states located in the coastal regions of India. The Pati duck (Anasplatyrhynchos domesticus) population constitutes a major indigenous non-descript economically important duck variety in the state of Assam. The Harderian gland acts as a part of the Head Associated Lymphoid Tissue (HALT) Olah et al. (1992) and provides local innate immunity to the upper respiratory system, to the eye and oral cavity. Since literature on the postnatal development of Harderian gland of Pati duck is scant, hence the present study was designed to establish the basic anatomical details on Harderian gland at different stages of postnatal development of Pati duck of Assam. Methods: The present study was conducted on total forty five (45) numbers Pati duck of Assam at different stages of postnatal development. The experimental birds were brought to the Department of Anatomy and Histology, College of Veterinary Science, Assam Agricultural University, Khanapara, Guwahati-22 and was sacrificed according to the method of Gracy (1986). After slaughter, the birds were placed on a clean dissecting table and skin and fascia were reflected carefully without disturbing the other organs of the head region. The Harderian gland was collected by opening the orbital cavity. Thereafter, length, breadth and thickness of the Harderian gland of all age group were recorded with the help of the Vernier Callipers (McCance, 1974) and topographical location of the gland was observed. The weight of the Harderian gland of all age group was also recorded with the help of electronic balance. Result: The Harderian gland of Pati duck (Anas platyrhynchos domesticus) was located within the orbit. The gland was flat, oval and coma shaped with irregular border. The gland had two surfaces i.e. the parietal and visceral surfaces, two borders and two blunt poles. The parietal surface was found convex and attached to fascia covered by the nasal bones and interorbital septa. The visceral surface was concave and it was attached loosely to the eye ball with fascia. The lobes of the Harderian gland became more prominent with the advancement of the age. The gland was light pink in colour. The arterial blood and the venus drainage was by provided by the ophthalmic artery and vein, respectively. The slender branch of oculomotor nerve innervated the Harderian gland. The result reflected an ascending trend from 0 week age group to 42 week age group in regard to all the gross parameters. There were slight difference between left and right glands for all the gross parameters but such variations were not statistically significant (P greater than 0.05). Each lobe of the Harderian Gland of Pati duck (Anas platyrhynchos domesticus) had a single short duct which opened into the conjunctival sac at the base of the 3rd eyelid.

RARE-16. A PATIENT WITH MOSAIC POST-ZYGOTIC KRAS-G12D PATHOGENIC VARIANT PRESENTING WITH A SYMPTOMATIC SPINAL NEUROFIBROMA AND LARGE SEGMENTAL TRUNCAL CAFÉ AU LAIT SPOT

An 11 year old boy presented with a three month history of progressive bilateral lower extremity weakness associated with recent intermittent incontinence. Spine MRI showed a right-sided T11-T12 paraspinal mass extending through the neural foramina into the epidural space and causing severe spinal cord compression. Skin showed a large macular lightly-hyperpigmented café au lait spot with irregular borders along the T10-T12 dermatome extending from the spine to approximately the anterior-axillary line. He was suspected to have segmental neurofibromatosis type 1 (NF1) as no additional clinical findings, radiographic features or family history of NF1 were identified. Patient underwent T10-12 laminectomy for resection of the epidural tumor component. Post-operative MRI showed resolution of the mass effect on the thecal sac and cord, with expected tumor residual lateral to the neural foramen. His residual spinal tumor and mild scoliosis remained stable over the two years of follow up to date. Pathological and molecular analysis of the resected tumor revealed a neurofibroma harboring an activating KRAS c.35G>A, p.Gly12Asp (KRAS-G12D) pathogenic variant at 27% variant allele frequency. Melanocytes cultured from two hyperpigmented skin biopsies showed the same KRAS-G12D pathogenic variant. This KRAS-G12D pathogenic variant was not found in leukocytes, indicating a post-zygotic origin. No NF1 pathogenic variant was identified in tumor tissue, melanocytes or leukocytes. The clinical findings were consistent with a mosaic KRASopathy due to a post zygotic KRAS-G12D pathogenic variant. The presence of the KRAS variant in the spinal neurofibroma and overlaying café au lait spot without an NF1 etiology in associated tissues demonstrates overlapping variability of presentations of RAS-MAPK pathway disorders. This case highlights the need for full clinical and genetic evaluation of patients presenting with segmental neurocutaneous disorders.

18F-FDG PET/CT Imaging in an Unusual Case of Cutaneous Melanoma Arising From Congenital Melanocytic Nevus in a Two-year-old Girl

Childhood malignant melanoma (MM) is extremely uncommon. We report an unusual case of cutaneous melanoma that developed from a medium-sized congenital melanocytic nevus (CMN) in a two-year-old girl. The patient had a history of CMN on the right hip, and she presented with a new ulcerative area with irregular borders and bleeding on CMN. Histopathological examination of the nevus revealed a MM. 18Florfluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography was performed for metastatic evaluation. The scan demonstrated metastatic increased 18F-FDG uptake in the right external iliac and inguinal lymph nodes.

Krukenberg tumor as an incidental finding in a full-term pregnancy: a case report

BackgroundKrukenberg tumor is a rare metastatic tumor of the ovary with histopathological features known as signet ring cells. It usually develops in women around 45 years of age. However, here we describe an uncommon case in a 38-year-old pregnant woman. We report this case due to our unusual findings, the uncommon presentation in this younger age bracket, its diagnostic challenge, and poor prognosis.Case presentationWe describe a unique case of a young Mexican woman with a history of painful vaginal bleeding at 13 weeks of pregnancy and treated for abruptio placentae. In her routine prenatal visit at week 20 of pregnancy, she was found to have a uterine fundus exceeding the expected measure for her gestational age and was referred to the hospital to discard polyhydramnios. Upon admission, a pelvic ultrasound was performed displaying normal findings in a 25-week pregnancy, and also showing bilateral enlarged ovaries with heterogeneous echogenicity. Magnetic resonance imaging revealed a left tumoral lesion with dimensions of 22.1 × 13.6 × 16.3 cm presenting regular lobulated contours with displacement of peripheral structures and mild compression of the bladder, the left ureter, and the inferior vena cava. The lesion was heterogeneous with irregular borders. The patient was scheduled for a cesarean section; during the operation, the abdominal cavity showed bilateral tumors compatible with MRI findings. The ovarian tumors were sent to pathology, and the results showed poorly differentiated mucinous adenocarcinoma (World Health Organization grade III) with extensive signet ring cells, indicative of a Krukenberg tumor.ConclusionThis case report describes an uncommon example of a young pregnant woman without identifiable risk factors for gastric cancer who manifested a Krukenberg tumor. This incidental finding suggests that pregnancy obscured the cancer’s clinical appearance. The rapid deterioration in the patient’s condition corresponds to what is described in the literature. The limited information regarding this neoplasm in Mexico and the torpid evolution of the case highlight the importance of conducting additional studies to generate therapeutic interventions aimed at increasing overall survival.

Endoscopic features of isolated and traditional serrated adenoma-associated superficially serrated adenomas of the colorectum.

Superficially serrated adenoma (SuSA) is a recently proposed subtype of colorectal serrated lesions. It is characterized by distinct clinicopathological and molecular features, including mixed serrated and adenomatous histology and frequent genetic alterations involving KRAS and RSPO. This study aimed to characterize the endoscopic features of isolated and traditional serrated adenoma (TSA)-associated SuSAs. We retrospectively evaluated the endoscopic findings of 25 isolated SuSAs and 21 TSA-associated SuSAs that were histologically and molecularly characterized. SuSAs appeared as a sessile polyp or slightly elevated lesion located mostly in the sigmoid colon and rectum (88%). The size was between 3 and 20 mm (median, 6 mm). Most of them exhibited KRAS mutations (96%) and RSPO fusions/overexpression (92%). Endoscopically, many lesions had a whitish color (84%), a distinct border (96%), an irregular border (76%), and a lobulated surface (72%). However, diminutive lesions exhibited overlapping features with hyperplastic polyps. On narrow-band imaging, vessel patterns were invisible or appeared as lacy microvessels in most lesions (80%). Chromoendoscopy invariably showed stellar or elongated/branched stellar pits, indicating a serrated microarchitecture. Most TSA-associated SuSAs typically presented as polyps with a two-tier raised appearance, consisting of whitish lower and reddish higher components corresponding to a SuSA and a TSA, respectively. SuSAs exhibit several characteristic endoscopic features on white-light and image-enhanced endoscopy. Diminutive lesions exhibit endoscopic features overlapping with hyperplastic polyps. Nonetheless, the endoscopic diagnosis of larger and TSA-associated SuSAs may be feasible.

POS0718 CLINICAL CHARACTERISTICS ASSOCIATED WITH GLANDULAR INVOLVEMENT EVALUATED BY SALIVARY GLAND ULTRASONOGRAPHY IN SJÖGREN’S SYNDROME

Background:Sjögren’s syndrome (SS) is a systemic autoimmune disease characterized by periductal lymphocytic infiltration of the salivary and lacrimal glands, which results in reduced secretory functions and oral and ocular dryness (1). In addition, patients often have extraglandular manifestations, such as interstitial pneumonia and interstitial nephritis, and the appearance of autoantibodies such as anti-Ro/SS-A and La/SS-B antibodies. Salivary gland ultrasonography (SGUS) is typically used to evaluate the findings of salivary glands; thus, we can evaluate the severity of salivary gland disorders due to SS using SGUS in real time (2).Objectives:To identify clinical indices, including disease activity, associated with glandular involvement evaluated using SGUS in patients with SS.Methods:We enrolled patients with SS (n=115) and non-SS sicca subjects (n=90) who visited Nagasaki University Hospital between 1995 and 2019. The patients’ SS classifications were based on the 2002 American–European Consensus Group (AECG) SS classification criteria (3). The non-SS sicca subjects exhibited sicca symptoms but did not fulfill the AECG SS classification criteria. SGUS and clinical indices such as age, sex, the focus score (FS), sicca symptoms, the Saxon test results, Schirmer’s test results, anti-SS-A/Ro antibody positivity, anti-SS-B/La antibody positivity, anti-centromere antibody (ACA) positivity, serum immunoglobulin G levels, and the clinical European League Against Rheumatism SS disease activity index were examined. The ultrasonography (US) score was calculated based on SGUS imaging (hypoechoic area, hyperechoic band, and irregular border) (4).Results:The US score was significantly higher in patients with SS than that in non-SS sicca subjects. In addition, we found significant correlations between the US score and FS in patients with SS. Multivariate analysis revealed the FS, Saxon test positivity, and ACA positivity as the variables independently associated with the US score in patients with SS. These results were the same in the primary SS patient group (n=96). Patients with ACA positivity had significantly higher US scores compared to those in patients with ACA negativity, whereas the FS was not significantly high. In addition, patients with ACA positivity had significantly greater positivity of hyperechoic bands than that in patients with ACA negativity.Conclusion:This study indicated that ACA positivity, which is not reflected in sialadenitis of SS, is associated with the US score in patients with SS. These results suggest that US findings of patients with ACA positivity might show specific changes in salivary glands, such as fibrosis, and not only sialadenitis (5).

Carney Complex With Endocrine Involvement Isolated to the Thyroid Gland

Abstract Introduction: Carney complex is a rare autosomal dominant disorder characterized by pigmented lesions of skin and mucosae, endocrine neoplasms or overactivity, and myxomas of the heart, skin, and other organs. Most patients have at least two affected endocrine organs at time of diagnosis. We present a case of Carney complex with endocrine involvement limited to the thyroid gland. Case: A 48-year-old female was referred for evaluation of thyroid nodules incidentally discovered on imaging for submandibular salivary gland swelling. Ultrasound evaluation of the thyroid revealed numerous, bilateral nodules that were mostly cystic or spongiform, though some had irregular borders and microcalcifications. A brother with Carney complex had been diagnosed with papillary carcinoma, and the patient decided to undergo thyroidectomy. Fortunately, no thyroid carcinoma was found on postsurgical histopathology. Initial biochemical evaluation showed no evidence of hypercortisolemia (8 AM cortisol 1.7 mcg/dL on 1 mg dexamethasone suppression test; 24-h urine cortisol 26.1 mcg [reference: 4-50]), growth hormone excess (IGF-1 190 ng/mL [reference: 52-328]), or hyperprolactinemia (prolactin 10 ng/mL [reference: 2.74-26.72]). Imaging showed no pituitary or adrenal masses. The patient underwent total hysterectomy with bilateral salpingo-oophorectomy for endometrial cancer prior to referral. She was diagnosed with Carney complex at age 19 years, and her manifestations included atrial and ventricular myxomas, intraductal adenoma of the breast, multiple skin lesions (lentigines, blue nevi, and cutaneous myxomas), and myxomas of the external auditory canals. She is in a Carney complex kindred that includes her mother, two brothers, and a niece. Discussion: Carney complex is usually caused by inactivating mutations or large deletions in the protein kinase A type I alpha regulatory subunit (PRKAR1A) gene located on chromosome 17q22-24. Most mutations are inherited in an autosomal dominant manner, though approximately 30% of cases are due to de novo mutations. In a review of 365 cases, the median age at diagnosis was 20 years. Growth hormone adenomas or somatomammotroph hyperplasia occurs in approximately 75% of patients, and most have at least one additional endocrine abnormality including multinodular goiter, primary pigmented nodular adrenocortical hyperplasia, and testicular or ovarian masses. There is an approximately 10% chance of developing well differentiated thyroid carcinoma. Treatment for Carney complex is individualized depending on the patient’s presentation and includes excision or surgical removal of myxomas, neoplasms, and skin lesions, as well as regular evaluation for cardiac myxomas and endocrine overactivity. This case of Carney complex is unusual because the only endocrinopathy is multinodular goiter.