Search

A buccal bifurcation cyst (BBC) is a rarely occurring, distinct lesion that is limited exclusively to the buccal bifurcation area of mandibular first and second molars in children and adolescents. A definitive diagnosis is formulated based on specific clinical and radiographic features. Management of such cysts depends on the presence of symptoms and the size of the lesion. This case report details the common features of a BBC in a 13-year-old patient and outlines the surgical approach to managing the cystic entity. The importance of a comprehensive clinical examination and appropriate supplemental investigations is emphasized to facilitate accurate diagnosis.

Autosomal dominant polycystic kidney disease (ADPKD) is driven by mutations in the PKD1 and PKD2 genes, and it is characterized by renal cyst formation, inflammation and fibrosis. Forkhead box protein M1 (FoxM1), a transcription factor of the Forkhead box (Fox) protein super family, has been reported to promote tumor formation, inflammation and fibrosis in many organs. However, the role and mechanism of FoxM1 in regulation of ADPKD progression is still poorly understood. Here, we show that FoxM1 is an important regulator of cyst growth in ADPKD. FoxM1 is upregulated in cyst-lining epithelial cells in Pkd1 mutant mouse kidneys and human ADPKD kidneys. FoxM1 promotes cystic renal epithelial cell proliferation by increasing the expression of Akt and Stat3 and the activation of ERK and Rb. FoxM1 also regulates cystic renal epithelial cell apoptosis through NF-κB signaling pathways. In addition, FoxM1 regulates the recruitment and retention of macrophages in Pkd1 mutant mouse kidneys, a process that is associated with FoxM1-mediated upregulation of monocyte chemotactic protein 1. Targeting FoxM1 with its specific inhibitor, FDI-6, delays cyst growth in rapidly progressing and slowly progressing Pkd1 mutant mouse kidneys. This study suggests that FoxM1 is a central and upstream regulator of ADPKD pathogenesis and provides a rationale for targeting FoxM1 as a therapeutic strategy for ADPKD treatment., (© The Author(s) 2022. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.)

Introduction: Intracranial cysts and cyst-like intracranial lesions are common findings on neuroimaging with a wide range of etiologies. Oftentimes, cystic intracranial lesions are benign but in some parts of the world, infectious etiologies for cystic lesions in the brain are quite common. Prompt identification of the cause of a cystic brain lesion is paramount in determining appropriate treatment if indicated., Areas Covered: In this narrative review article, the authors present a comprehensive description of cystic lesions of infectious or inflammatory etiology. Imaging descriptions are provided for each type of cystic lesion as well as several representative images., Expert Opinion: The majority of diagnoses may be identified by CT and MR imaging. Yet some pathologies are still unable to be determined through standard imaging techniques and biopsy remains necessary for definitive diagnosis in some cases. Advanced neuroimaging such as metabolic/nuclear imaging and advanced MR hold promise with improved diagnostics but are not often available in geographic regions where these illnesses are endemic.

Congenital conjunctival cysts often occur in the medial orbit without adjacent bone destruction and have not been previously reported in a dumbbell configuration. A 46-year-old female patient presented with left proptosis and headache. A radiological study revealed a large, cystic, bilobed mass in the orbital-temporal area with an osseous defect in the lateral wall. A histological examination of the excised lesion showed that the cyst wall consisted of non-keratinized stratified epithelium with goblet cells. There was no recurrence during the follow-up. Of the previously reported 54 cases, only 1 had bone erosion due to a recurrent cyst in the frontal bone. Although rare, a primary conjunctival cyst can create a dumbbell-shaped lesion in the lateral orbit and temporal fossa., Competing Interests: The authors have no conflicts of interest., (Copyright © 2022 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Background: The aim of the study was comparing the diagnostic role of the white light (WL) endoscopy, video laryngostroboscopy (VLS), and narrow-band imaging (NBI) in the evaluation of the benign vocal fold lesions., Material and Methods: From March 2018 to June 2019, a total of 118 cases were enrolled in this study. Ninety-eight patients were suspected with vocal fold nodules, cysts, polyps, and sulcus vocalis and 20 patients without a history of dysphonia. Each patient was examined by WL, VLS, and NBI endoscopy. Recorded images and videos were analyzed and scored by three otorhinolaryngologists who had at least 3 years of experience in phoniatrics field. The evaluation results were compared between the methods and the physicians., Results: A total of 118 cases were grouped by their confirmed diagnosis, vocal fold nodules (n = 28), vocal fold cyst (n = 24), vocal fold polyp (n = 9), and sulcus vocalis (n = 37). When the correct diagnosis rates of the physicians were compared, the statistical significance was found between the physicians in the WL and VLS method (P= 0.014, P= 0.027). No statistically significant difference was found among physicians in NBI method (P = 0.368). The difference between the diagnostic methods was found to be statistically significant in reaching the accurate diagnosis for benign vocal fold lesions (P< 0.001). While the difference between NBI-WL and VLS-WL was statistically significant (P< 0.001 and P< 0.001). The difference between NBI-VLS was not statistically significant while evaluating the vocal fold nodules, cysts, and sulcus vocalis separately (P= 0.102, P = 0.026, P = 0.157). Otherwise, it was statistically significant (P= 0.002) while evaluating total benign lesions in the study. The difference between NBI-VLS combination and VLS, NBI-VLS combination and VLS-WL combination were statistically significant (P< 0.001 and P= 0.001)., Conclusion: This study showed that NBI assessments have similar accuracy and sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) to VLS and have a higher value than WL endoscopy in reaching the diagnosis for benign vocal fold lesions. Therefore, NBI can be accepted as a promising approach to identify benign laryngeal lesions due to its optical properties., (Copyright © 2021 The Voice Foundation. Published by Elsevier Inc. All rights reserved.)

Background: The natural history of branch-duct intraductal papillary neoplasm (BD-IPMN) in BRCA1/2 patients is unknown. Our goal was to estimate the incidence and prevalence of BD-IPMN and other pancreatic lesions in BRCA1/2 patients and compare it to that for average-risk individuals., Methods: We identified a cohort of BRCA1/2 patients followed at our institution between 1995 and 2020. Medical records and imaging results were reviewed to determine prevalence of pancreatic lesions. We then identified those who had undergone follow-up imaging and determined the incidence of new pancreatic lesions. We categorized pancreatic lesions as low, intermediate, or high-risk based on their malignant potential., Results: During the study period, 359 eligible BRCA1/2 patients were identified. Average patient age was 56.8 years, 88.3% were women, and 51.5% had BRCA1 . The prevalence of low-risk pancreatic lesions was 14.4%, intermediate-risk 13.9%, and high-risk 3.3%. The prevalence of BD-IPMN was 13.6% with mean cyst size 7.7 mm (range: 2 to 34 mm). The prevalence of pancreatic cancer was 3.1%. Subsequent imaging was performed in 169 patents with mean follow-up interval of 5.3 years (range: 0 to 19.7 y). The incidence of BD-IPMN was 20.1%, with median cyst size 5.5 mm (range: 2 to 30 mm). The incidence of pancreatic cancer was 2.9%. BRCA2 patients were almost 4-times more likely to develop pancreatic cancer than BRCA1 patients, however, there was no difference in incidence or prevalence of BD-IPMN., Conclusions: Incidence and prevalence of BD-IPMNs in BRCA1/2 patients was similar to that reported for average-risk individuals. BRCA2 patients were more likely than BRCA1 patients to develop pancreatic cancer but had similar rates of BD-IPMN., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Objectives: the aim of this study was to analyze the prevalence of histopathological diagnoses in oral biopsied tissues obtained from a Brazilian pediatric population., Methods: an analytical, cross-sectional retrospective study was performed with biopsy files of patients ≤14 years of age from a Brazilian oral pathology laboratory over a 43-year period. Data included sex, age, location, and diagnoses. The prevalence was calculated by means of relative frequency. Associations between sex, age groups and diagnoses were verified with Pearson's chi-square test., Results: from 19,456 oral biopsies, 1480 (7.6%) were obtained from patients aged ≤14 years. Most children were 10-14 years of age (60.1%) and females (55.1%), with an overall M:F of 1:1.2. Children aged 0-9 years and males had a higher frequency of lesions of the oral mucosa, whilst the 10-14 year age group showed a higher frequency of cysts, odontogenic tumors, and salivary gland lesions. The latter was also significantly higher in females. Samples consisted mostly of soft tissue lesions (53%) obtained from the lower lip (30.7%). Intraosseous lesions showed a slight predilection for the mandible (21.2%). Salivary gland lesions (28.8%) was the most common diagnostic category, followed by reactive lesions (18.8%), and cysts (16.1%). Mucocele (33.5%), dentigerous cyst (6.7%), and fibrous hyperplasia (5.9%) were the top three histopathological diagnoses. Malignant lesions affected only 0.9% of this population., Conclusion: our results were similar to other retrospective studies. Due to the low frequency of oral biopsies in children, data on the prevalence of oral pathology in this population might aid in the clinical and histopathologic diagnoses., Competing Interests: Declaration of competing interest None., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Background: Craniovertebral junction (CVJ) cysts, including retro-odontoid pseudotumors, are challenging pathologies to treat and manage effectively. Surgical intervention is indicated when these lesions result in progressive myelopathy, intractable pain, or instability., Objective: To present a case series of older patients who underwent successful resection retro-odontoid lesions using transdural approach., Methods: A single-center, retrospective observation study of older patients who underwent transdural resection of CVJ cysts at a single institution was performed. Summary demographic information, clinical presentation, perioperative and intraoperative imaging, and Nurick scores were collected and analyzed., Results: Eight patients were included (mean age [±SD] 75.88 ± 9.09 years). All patients presented with retro-odontoid lesions resulting in severe cervical stenosis, cord compression, and myelopathy. The mean duration of surgery was 226 ± 83.7 minutes. The average intraoperative blood loss was 181.2 cc. The average hospital stay was 4.5 days ± 1.3 (range, 3-7 days). The average follow-up time was 12.5 ± 9.5 months. No intraoperative complications were encountered. The Nurick classification score for myelopathy improved at the final postoperative examination (2.38 ± 1.06 vs 1 ± 1.07). Three patients demonstrated a pre-existing deformity prompting an instrumented fusion. Both computed tomography and MRI evidence of complete regression of retro-odontoid cyst were noted in all patients on the final follow-up., Conclusion: Posterior cervical transdural approach for ventral lesions at the CVJ is a safe and effective means of treating older patients with progressive myelopathy. This technique provides immediate spinal cord decompression while limiting neurological complications commonly associated with open or endoscopic anterior transpharyngeal approaches., (Copyright © Congress of Neurological Surgeons 2022. All rights reserved.)

Purpose: Renal cysts comprise benign and malignant entities. Risk assessment profits from CT/MRI imaging using the Bosniak classification. While Bosniak-IIF, -III, and -IV cover complex cyst variants, Bosniak-IIF and -III stand out due to notorious overestimation. Contrast-enhanced ultrasound (CEUS) is promising to overcome this deficit but warrants standardization. This study addresses the benefits of a combined CEUS and CT/MRI evaluation of renal cysts. The study provides a realistic account of kidney tumor boards' intricacies in trying to validate renal cysts., Methods: 247 patients were examined over 8 years. CEUS lesions were graded according to CEUS-Bosniak (IIF, III, IV). 55 lesions were resected, CEUS-Bosniak- and CT/MRI-Bosniak-classification were correlated with histopathological diagnosis. Interobserver agreement between the classifications was evaluated statistically. 105 lesions were followed by ultrasound, and change in CEUS-Bosniak-types and lesion size were documented., Results: 146 patients (156 lesions) were included. CEUS classified 67 lesions as CEUS-Bosniak-IIF, 44 as CEUS-Bosniak-III, and 45 as CEUS-Bosniak-IV. Histopathology of 55 resected lesions revealed benign cysts in all CEUS-Bosniak-IIF lesions (2/2), 40% of CEUS-Bosniak-III and 8% of CEUS-Bosniak-IV, whereas malignancy was uncovered in 60% of CEUS-Bosniak-III and 92% of CEUS-Bosniak-IV. Overall, CEUS-Bosniak-types matched CT/MRI-Bosniak types in 58% (fair agreement, κ = 0.28). CEUS-Bosniak resulted in higher stages than CT/MRI-Bosniak (40%). Ultrasound follow-up of 105 lesions detected no relevant differences between CEUS-Bosniak-types concerning cysts size. 99% of lesions showed the same CEUS-Bosniak-type., Conclusion: The CEUS-Bosniak classification is an essential tool in clinical practice to differentiate and monitor renal cystic lesions and empowers diagnostic work-up and patient care., (© 2022. The Author(s).)

Objectives: To describe ultrasound features of fetal ovarian cysts as reported by the original ultrasound examiner, to apply International Ovarian Tumor Analysis (IOTA) terminology after retrospective analysis of the images and to describe patient management and evolution of fetal cysts during pregnancy and after delivery., Methods: This retrospective observational study included pregnant women diagnosed on ultrasound examination with a fetal ovarian cyst at the Prenatal Diagnosis Division of the Bambino Gesù Children's Hospital, in Rome, between March 2011 and May 2020. Cysts were classified by the original ultrasound examiner as 'simple' (unilocular anechoic cyst) or 'complex' (cyst with other morphology). In addition, three ultrasound examiners, experienced in gynecologic ultrasound, classified retrospectively the fetal ovarian cysts according to IOTA terminology, by reviewing stored ultrasound images. The evolution of these fetal ovarian cysts during pregnancy and after birth was recorded., Results: Included were 51 ovarian cysts in 48 fetuses. Of the 51 cysts, 29 (56.9%) had been classified by the original ultrasound examiner as 'simple', and 22 (43.1%) as 'complex'. Of the simple cysts, the majority (20/29 (69.0%)) resolved spontaneously after delivery, 2/29 (6.9%) resolved following intrauterine aspiration, 2/29 (6.9%) resolved after postnatal aspiration and 5/29 (17.2%) underwent surgery due to persistence after delivery; in all five, normal ovarian parenchyma without signs of necrosis was observed at histology. Of the complex cysts, 7/22 (31.8%) resolved spontaneously. The other 15/22 (68.2%) were removed surgically and, at histology, necrosis was observed in most (12/15 (80.0%)), while a benign epithelial cyst with normal ovarian parenchyma was observed in 3/15 (20%). After reviewing the ultrasound images and applying IOTA terminology, all 51 (100%) fetal cysts were described as unilocular; 29/51 (56.9%) cysts showed anechoic content (described as simple cysts by the original ultrasound examiner), and 10/51 (19.6%) had low-level, 1/51 (2.0%) had ground-glass, 9/51 (17.6%) had hemorrhagic, 1/51 (2.0%) had mixed and 1/51 (2.0%) had undefined content (all described as complex by the original ultrasound examiner). Among the 29 anechoic ovarian cysts, resolution was observed in most (24/29, 82.8%) cases. Similarly, resolution was observed in 7/10 (70.0%) cysts with low-level content. Resolution was not observed in any of the other 12 cysts and all of these cases underwent surgery, with evidence of necrosis being observed in 11 (91.7%)., Conclusions: Applying IOTA terminology provided a more detailed and accurate description of fetal ovarian cysts compared with the original classification into 'simple' and 'complex' categories. Anechoic cysts (described as simple cysts by the original ultrasound examiner) and cysts with low-level content (described as complex by the original ultrasound examiner) frequently resolved spontaneously. Cysts with ground-glass, hemorrhagic, mixed or undefined content were frequently associated with necrosis at histology following surgery. © 2022 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology., (© 2022 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.)

Objective: The size of the adnexal cyst is a limitation to perform laparoscopic adnexal surgery. Large adnexal cysts may constrain the surgery space and may increase the risk of inadvertent cyst rupture during the surgery. Here, we offer a method to solve the difficulty in laparoscopic management of large adnexal cysts., Materials and Methods: This is a retrospective study of 43 patients with large (≧10 cm) adnexal cysts, managed laparoscopically at one center from October 2016 to October 2019. All the surgeries were performed by an experienced laparoscopic surgeon. Malignancy was excluded before the surgeries according to the sonographic features. Cystectomy or salpingo-oophorectomy was decided according to the age, the sonographic pattern of the cyst and the patients' willingness. A 2 cm vertical incision was made at the umbilicus, and an open laparoscopy method was used to enter the peritoneal cavity. A wound protector was applied with a glove and two trocars being set up. The second wound was made at the left abdomen, and a 5 mm trocar was inserted. Then the cyst was punctured by means of the 5 mm trocar, and the content of the cyst was then soon aspirated by means of the air-flow hole of the trocar. Afterward, cystectomy or salpingo-oophorectomy was performed. Data are expressed as mean ± standard deviation unless stated otherwise., Results: The mean operative duration was 54.7 ± 33.4 min, and the result was shorter than the previous study (P < 0.05, CI (-32.92, -12.38)) according to one sample t-test. Most estimated blood loss (EBL) was minimum. No obvious complication was found., Conclusion: Two-port trocar suction in laparoscopic management of large adnexal cysts is feasible after proper evaluation, and it takes short operative time., Competing Interests: Declaration of competing interest All authors have no support, financial or otherwise, has been received from any organization that may have an interest in the submitted work. There are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2023. Published by Elsevier B.V.)

The evaluation of the maxillary sinus is very important in dental practice such as tooth extraction and implantation because of its proximity to the teeth, but it is not easy to evaluate because of the overlapping structures such as the maxilla and the zygoma on panoramic radiographs. When doom-shaped retention pseudocysts are observed in sinus on panoramic radiographs, they are often misdiagnosed as cysts or tumors, and additional computed tomography is performed, resulting in unnecessary radiation exposure and cost. The purpose of this study was to develop a deep learning model that automatically classifies retention pseudocysts in the maxillary sinuses on panoramic radiographs. A total of 426 maxillary sinuses from panoramic radiographs of 213 patients were included in this study. These maxillary sinuses included 86 sinuses with retention pseudocysts, 261 healthy sinuses, and 79 sinuses with cysts or tumors. An EfficientDet model first introduced by Tan for detecting and classifying the maxillary sinuses was developed. The developed model was trained for 200 times on the training and validation datasets (342 sinuses), and the model performance was evaluated in terms of accuracy, sensitivity, and specificity on the test dataset (21 retention pseudocysts, 43 healthy sinuses, and 20 cysts or tumors). The accuracy of the model for classifying retention pseudocysts was 81%, and the model also showed higher accuracy for classifying healthy sinuses and cysts or tumors (98% and 90%, respectively). One of the 21 retention pseudocysts in the test dataset was misdiagnosed as a cyst or tumor. The proposed model for automatically classifying retention pseudocysts in the maxillary sinuses on panoramic radiographs showed excellent diagnostic performance. This model could help clinicians automatically diagnose the maxillary sinuses on panoramic radiographs., (© 2023. The Author(s).)

Background: Vulvar leiomyoma is a rare soft tissue tumor, with only around 300 cases described in the literature. Owing to its low incidence of just 0.03% of all gynecological tumors, it often poses a great diagnostic challenge, especially in teenagers. We report this rare occurrence of vulvar leiomyoma in a teenage girl who was primarily left untreated due to cultural taboos and fear of loss of virginity. The main aim in presenting such rare case studies is to raise awareness and expand the diagnostic horizon of the surgeon for appropriate management., Case Presentation: We describe a case of a 15-year-old North Indian, sexually inactive unmarried girl, who presented with a history of painless swelling in the left labia majora for the last 1 year, which gradually increased in size. There was no associated pain or any other difficulty. Local examination revealed a 14 × 10 cm solid, unilateral nonpedunculated mass on the left labia majora with superficial vascularity. Differential diagnoses of sarcoma, lipoma, Bartholin cyst, and fibroid were kept in mind. Ultrasonography revealed a solid mass with superficial vascularity with normal internal genitalia. The mass was enucleated with an intact capsule under anesthesia. Histopathology confirmed it to be benign vulvar leiomyoma. The patient was discharged after 3 days in a satisfactory condition., Conclusion: Leiomyoma of the vulva is an exceptionally rare tumor and is seldom seen in teenagers. It is often misinterpreted as a Bartholin cyst and should be kept as one of the differential diagnosis in teenage girls presenting with unilateral vulvar swelling. Vulvar leiomyoma can be completely cured by surgical removal if diagnosed timely without compromising virginity, so should never be missed in adolescents., (© 2023. The Author(s).)

Rationale: Leiomyoma of the vulva is a rare, benign mass that is present on the vulva. Most commonly, they are described as painless, well-circumscribed, solitary tumors that are misdiagnosed as Bartholin cysts before surgery., Patient Concerns: A 45-year-old woman presented with a case of vulvar leiomyoma misdiagnosed as Bartholin cyst preoperatively. A solitary swelling mass measuring 3 cm × 2 cm was found in the left labia majora at the Bartholin gland site on physical examination., Diagnoses: A vulvar mass extent and vascularity may be determined by imaging. A color doppler flow imaging of the posterior vaginal wall revealed abundant blood flow., Intervention: To confirm vulvar leiomyoma, surgery and histopathology were performed., Outcome: After 2 months of follow-up, there were no signs of recurrence in the patient., Lessons: Rare vulvar leiomyomas are often mistaken for Bartholin's cysts. It is also difficult to distinguish benign from malignant forms, making vulvar leiomyoma a difficult diagnosis. As there are a few techniques used to differentiate between the nature of the tumor, excisional biopsy seems to be the best current procedure employed in addition to being the treatment of choice for such tumors., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disease. Cyst development in ADPKD involves abnormal epithelial cell proliferation, which is affected by the primary cilia-mediated signal transduction in the epithelial cells. Thus, primary cilium has been considered as a therapeutic target for ADPKD. Since ADPKD exhibits many pathological features similar to solid tumors, we investigated whether targeting primary cilia using anti-tumor agents could alleviate the development of ADPKD. Twenty-four natural compounds with anti-tumor activity were screened in MDCK cyst model, and 1-Indanone displayed notable inhibition on renal cyst growth without cytotoxicity. This compound also inhibited cyst development in embryonic kidney cyst model. In neonatal kidney-specific Pkd1 knockout mice, 1-Indanone remarkably slowed down kidney enlargement and cyst expansion. Furthermore, we demonstrated that 1-Indanone inhibited the abnormal elongation of cystic epithelial cilia by promoting tubulin polymerization and significantly down-regulating expression of anterograde transport motor protein KIF3A and IFT88. Moreover, we found that 1-Indanone significantly down-regulated ciliary coordinated Wnt/β-catenin, Hedgehog signaling pathways. These results demonstrate that 1-Indanone inhibits cystic cell proliferation by reducing abnormally prolonged cilia length in cystic epithelial cells, suggesting that 1-Indanone may hold therapeutic potential to retard cyst development in ADPKD., (© 2022. The Author(s), under exclusive licence to Shanghai Institute of Materia Medica, Chinese Academy of Sciences and Chinese Pharmacological Society.)

Abstract: Pancreatic mucinous cystic neoplasm (MCN) rarely ruptures because of their surrounding fibrotic capsules and has never been reported with detailed information regarding prerupture and postrupture states. We report a case of MCN rupture where performed emergency surgery was performed while waiting for elective surgery. A 54-year-old woman was referred to our department for a pancreatic cystic tumor with slight abdominal pain. A cystic tumor with a nodular lesion was found, with a contrast effect measuring 78 mm in diameter. On day 21, the patient visited our hospital complaining of increased abdominal pain, but few signs of peritonitis were observed. Tests conducted revealed moderate ascites, marginal shrinkage of the cyst diameter, and a slight elevation of inflammatory markers. We suspected an MCN rupture and immediately performed distal pancreatectomy. Brown turbid ascites and rupture of the anterior wall of the cyst were observed. In the ascites, amylase levels were not elevated, and bacterial cultures were negative. The histopathological diagnosis was noninvasive mucinous cystadenocarcinoma. At 9 months after surgery, she started chemotherapy because of a recurrence of the peritoneal dissemination. This case provided valuable insight into the rupture of MCNs using thorough imaging techniques, laboratory, and physical findings before and after rupturing., Competing Interests: The authors declare no conflict of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Head and neck skin angiosarcoma is a rare and aggressive tumor (1 % of head and neck tumors). Prognosis remains poor, with a 5-year survival rate ranging from 10 to 54%, depending on the initial stage. Metastatic disease markedly worsens the prognosis. Metastatic lung involvement is classic and can take on several forms. The cystic form is responsible for numerous complications, particularly pneumothorax. In this case, an 83-year-old patient was diagnosed with bilateral pneumothorax complicating cystic interstitial lung disease, which was revealed by hemoptoic sputum. Skin examination revealed two large necrotic lesions of the calvaria. Anatomo-pathological examination confirmed cutaneous angiosarcoma on both skin biopsy and lung resection. At a metastatic stage, only systemic treatment with paclitaxel can be proposed. The clinical course was unfavorable, leading to death before any specific treatment. This observation highlights the importance of a complete clinical skin examination in the assessment of pulmonary cystic lesions., (Copyright © 2023 SPLF. Published by Elsevier Masson SAS. All rights reserved.)

Objective: To evaluate effectiveness and safety of computed tomography (CT)-guided cyst rupture with intraarticular contrast-enhanced injection of steroid and local anesthetic as first choice therapy in patients with facet joint cyst-induced radicular pain., Design: Retrospective data set analysis., Setting: University hospital., Subjects: One hundred and twenty-one patients suffering from radicular pain attributable to facet joint cysts were included., Methods: The rate of patients without following surgery was assessed and defined as surrogate to measure effectiveness. Patients' characteristics, procedure-associated complications, technical aspects, and imaging findings on magnetic resonance imaging (MRI) were analyzed. A subgroup of 65 patients (54%) underwent telephone interview to assess pain relief and clinical outcome measured by Numeric Rating Scale and Oswestry Disability Index. Analyses between the groups with and without surgery were performed by Fisher exact test and two-sample unpaired t-test, respectively., Results: The effectiveness of CT-guided cyst rupture was found to be 66.1%. Procedure-induced pain yielded in premature abort in two cases (1.7%). The detection of epidural contrast agent was statistically significantly associated with no need for surgery (P = .010). The cyst level was associated with the status of following surgery (P = .026), that is, cysts at lower lumbar spine were easier to rupture than cysts at other locations (cervical, thoracic, or upper lumbar spine). No further significant association was found., Conclusions: CT-guided cyst rupture as the first-choice therapy in patients with cyst-induced radicular pain was safe and effective. Successful cyst rupture was associated with no need for surgery. Cysts at lower lumbar spine revealed the highest success rate., (© The Author(s) 2022. Published by Oxford University Press on behalf of the American Academy of Pain Medicine. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Objectives: This study investigated the early progression patterns of lung squamous cell carcinoma (SqCC) on computed tomography (CT) images., Materials and Methods: In total, 65 patients with SqCC who underwent surgical resection and two CT scans separated by an interval of at least 6 months were enrolled. We categorized the findings of the initial and at-diagnosis CT images into five patterns as previously reported. The volume doubling time (VDT) was calculated for measurable lesions., Results: A single nodule pattern on CT images at-diagnosis was most common in 56 (86.2 %) patients, in line with practical clinical findings. However, the patterns were diverse in the initial images, with 28 (43.1 %) patients displaying atypical findings, including multiple nodules (3.1 %), endobronchial lesions (20.0 %), subsolid nodules (10.8 %), and cyst wall thickening (9.2 %). All endobronchial lesions were located in the central/middle zone of the lung field, whereas lesions presented as multiple nodules, subsolid nodules, and cyst wall thickening were predominantly observed in the peripheral zone. The differences in the developed zones were reflected in the median VDT, and the tumors with an initial endobronchial pattern had a significantly shorter VDT than those with a subsolid nodule pattern (median: 140 days vs 276 days, p < 0.001)., Conclusions: Lung SqCC initiated with various CT image patterns, although most tumors ultimately developed a single nodule pattern by diagnosis. The initial CT image patterns differed between the hilar and peripheral zones, suggesting a difference in the progression scheme, which was also supported by differences in VDT., Competing Interests: Declaration of Competing Interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: Dr. Kusumoto reports contracted studies with Canon Medical Systems; honoraria for lectures from AstraZeneca K.K., Daiichi-Sankyo Inc., Daiichi-Sankyo Co., ltd., outside the submitted work. Dr. Yatabe reports contracted studies with ArcherDx, Chugai-pharma, Thermo Fisher Science, and Konika Minolta, honoraria for lectures and/or advisory board from AbbVie Inc., Amgen, Bayer, Ono-Pharma, Daiichi-Sankyo, Elli-Lily, Merck Bio-Pharma, Pfizer, MSD, Novartis, AstraZeneca, Agilent/Dako, ArcherDx, Sysmex, Chugai-pharma, Boehringer Ingelheim, Yansen-Pharma, Roche/Ventana, Thermo Fisher Science, and Takeda; participation on the advisory board of MSD; Chugai-pharma; AstraZeneca, Novartis, Amgen, Takeda, and Daiichi-Sankyo, outside the submitted work. Other authors have nothing to disclose., (Copyright © 2022 The Authors. Published by Elsevier B.V. All rights reserved.)

Background: Degenerative joint disease (DJD) can be associated with disc displacement (DD) in temporomandibular disorder (TMD) patients. However, the relationship between different types of DDs and DJD remains unclear., Objectives: To investigate the odds ratios of different types of sagittal and coronal DDs confirmed by magnetic resonance imaging (MRI) and DJD confirmed by cone-beam computed tomography (CBCT) in TMD patients., Methods: Radiographic data from 69 males and 232 females were collected for analysis. CBCT was used to diagnose DJD, with criteria including erosion, osteophytes, generalised sclerosis and cysts in the joint. Eight types of DDs were evaluated by sagittal and coronal MRIs: NA, no abnormality; SW, sideways; ADDR, anterior with reduction; ADDR+SW; ADDNR, anterior without reduction; ADDNR + SW; single SW; PDD, posterior; PDD + SW. The odds ratios of DJD in joints with different types of DDs were determined after joint correlation, age and gender adjustment., Results: Compared with NA, the odds ratio of DJD in ADDR was 2.397 (95% CI [confidence interval]: 1.070-5.368), ADDR + SW was 4.808 (95% CI: 1.709-3.528), ADDNR was 29.982 (95% CI: 15.512-57.950) and ADDNR + SW was 25.974 (95% CI: 12.743-52.945). Erosion was significantly increased in ADDR, ADDR + SW, ADDNR and ADDNR + SW; osteophytes were significantly increased in ADDR + SW, ADDNR and ADDNR + SW; and generalised sclerosis and cysts were significantly increased in ADDNR and ADDNR + SW. There were no significant associations between single SW, PDD, PDD + SW and the DJD., Conclusions: ADDR, ADDR+SW, ADDNR and ADDNR+SW were associated with DJD. ADDNR had a significantly higher prevalence of DJD than ADDR. There were no significant relationships between single SW, PDD, PDD + SW and the DJD., (© 2022 John Wiley & Sons Ltd.)

The formation and growth of kidney cysts (fluid-filled structures lined by epithelial cells) is the primary pathological abnormality in polycystic kidney disease (PKD). Multiple molecular pathways are disrupted in kidney epithelial precursor cells, which lead to altered planar cell polarity, increased proliferation, and fluid secretion, which together with extracellular matrix remodelling culminates in the formation and growth of cysts. Three-dimensional (3D) in vitro cyst models serve as suitable preclinical models to screen candidate drugs for PKD. Madin-Darby Canine Kidney (MDCK) epithelial cells form polarized monolayers with a fluid-filled lumen when suspended in a collagen gel, and their growth is accelerated with the addition of forskolin, a cyclic adenosine monophosphate (cAMP) agonist. Candidate drugs for PKD can be screened for their ability to modulate growth of forskolin-treated MDCK cysts by measuring and quantifying cyst images acquired at progressive timepoints. In this chapter, we describe the detailed methods for the culture and growth of MDCK cysts in a collagen matrix and a protocol for their use in testing candidate drugs to prevent cyst formation and growth., (© 2023. The Author(s), under exclusive license to Springer Science+Business Media, LLC, part of Springer Nature.)

Autosomal dominant polycystic kidney disease (ADPKD) involves the development and persistent growth of fluid filled kidney cysts. In a recent study, we showed that ADPKD kidney cyst epithelial cells can stimulate the proliferation and differentiation of peri-cystic myofibroblasts. Although dense myofibroblast populations are often found surrounding kidney cysts, their role in cyst enlargement or fibrosis in ADPKD is unclear. To clarify this, we examined the effect of myofibroblast depletion in the Pkd1RC/RC (RC/RC) mouse model of ADPKD. RC/RC;αSMA tk mice that use the ganciclovir-thymidine kinase system to selectively deplete α-smooth muscle actin expressing myofibroblasts were generated. Ganciclovir treatment for four weeks depleted myofibroblasts, reduced kidney fibrosis and preserved kidney function in these mice. Importantly, myofibroblast depletion significantly reduced cyst growth and cyst epithelial cell proliferation in RC/RC;αSMA tk mouse kidneys. Similar ganciclovir treatment did not alter cyst growth or fibrosis in wild-type or RC/RC littermates. In vitro, co-culture with myofibroblasts from the kidneys of patients with ADPKD increased 3D microcyst growth of human ADPKD cyst epithelial cells. Treatment with conditioned culture media from ADPKD kidney myofibroblasts increased microcyst growth and cell proliferation of ADPKD cyst epithelial cells. Further examination of ADPKD myofibroblast conditioned media showed high levels of protease inhibitors including PAI1, TIMP1 and 2, NGAL and TFPI-2, and treatment with recombinant PAI1 and TIMP1 increased ADPKD cyst epithelial cell proliferation in vitro. Thus, our findings show that myofibroblasts directly promote cyst epithelial cell proliferation, cyst growth and fibrosis in ADPKD kidneys, and their targeting could be a novel therapeutic strategy to treat PKD., (Copyright © 2022 International Society of Nephrology. Published by Elsevier Inc. All rights reserved.)

Objective: Liver lesion characterization is limited by the lack of an established gold standard for precise correlation of radiologic characteristics with their histologic features. The objective of this study was to demonstrate the feasibility of using an ex vivo MRI-compatible sectioning device for radiologic-pathologic co-localization of lesions in resected liver specimens., Methods: In this prospective feasibility study, adults undergoing curative partial hepatectomy from February 2018 to January 2019 were enrolled. Gadoxetic acid was administered intraoperatively prior to hepatic vascular inflow ligation. Liver specimens were stabilized in an MRI-compatible acrylic lesion localization device (27 × 14 × 14 cm 3 ) featuring slicing channels and a silicone gel 3D matrix. High-resolution 3D T1-weighted fast spoiled gradient echo and 3D T2-weighted fast-spin-echo images were acquired using a single channel quadrature head coil. Radiologic lesion coordinates guided pathologic sectioning. A final histopathologic diagnosis was prepared for all lesions. The proportion of successfully co-localized lesions was determined., Results: A total of 57 lesions were identified radiologically and sectioned in liver specimens from 10 participants with liver metastases (n = 8), primary biliary mucinous cystic neoplasm (n = 1), and hepatic adenomatosis (n = 1). Of these, 38 lesions (67%) were < 1 cm. Overall, 52/57 (91%) of radiologically identified lesions were identified pathologically using the device. Of these, 5 lesions (10%) were not initially identified on gross examination but were confirmed histologically using MRI-guided localization. One lesion was identified grossly but not on MRI., Conclusions: We successfully demonstrated the feasibility of a clinical method for image-guided co-localization and histological characterization of liver lesions using an ex vivo MRI-compatible sectioning device., Key Points: • The ex vivo MRI-compatible sectioning device provides a reliable method for radiologic-pathologic correlation of small (< 1 cm) liver lesions in human liver specimens. • The sectioning method can be feasibly implemented within a clinical practice setting and used in future efforts to study liver lesion characterization. • Intraoperative administration of gadoxetic acid results in enhancement in ex vivo MRI images of liver specimens hours later with excellent image quality., (© 2022. The Author(s), under exclusive licence to European Society of Radiology.)

Widespread aberrant gene expression is a pathological hallmark of polycystic kidney disease (PKD). Numerous pathogenic signaling cascades, including c-Myc, Fos, and Jun, are transactivated. However, the underlying epigenetic regulators are poorly defined. Here we show that H3K27ac, an acetylated modification of DNA packing protein histone H3 that marks active enhancers, is elevated in mouse and human samples of autosomal dominant PKD. Using comparative H3K27ac ChIP-Seq analysis, we mapped over 16000 active intronic and intergenic enhancer elements in Pkd1-mutant mouse kidneys. We found that the cystic kidney epigenetic landscape resembles that of a developing kidney, and over 90% of upregulated genes in Pkd1-mutant kidneys are co-housed with activated enhancers in the same topologically associated domains. Furthermore, we identified an evolutionarily conserved enhancer cluster downstream of the c-Myc gene and super-enhancers flanking both Jun and Fos loci in mouse and human models of autosomal dominant PKD. Deleting these regulatory elements reduced c-Myc, Jun, or Fos abundance and suppressed proliferation and 3D cyst growth of Pkd1-mutant cells. Finally, inhibiting glycolysis and glutaminolysis or activating Ppara in Pkd1-mutant cells lowerd global H3K27ac levels and its abundance on c-Myc enhancers. Thus, our work suggests that epigenetic rewiring mediates the transcriptomic dysregulation in PKD, and the regulatory elements can be targeted to slow cyst growth., (Copyright © 2022 International Society of Nephrology. Published by Elsevier Inc. All rights reserved.)

This review aims to provide a detailed analysis of the pathological processes underlying peri anchor cyst formation. As a consequence providing methods that can be implemented to reduce cyst occurrence and also to highlight areas of current weakness in the literature that could be strengthened so as to improve our ability to manage peri anchor cyst formation. We performed a literature review of the National Library of Medicine focused around rotator cuff repair and peri anchor cysts. We summarise the literature whilst incorporating a detailed analysis of the pathological processes underpinning peri anchor cyst formation. There are two theories behind peri anchor cyst occurrence, biochemical and biomechanical. It is our belief that cyst formation occurs as a result of both. The biochemical make up of an anchor plays a crucial role in cyst occurrence and it's timing post-operatively. Consequently anchor material plays a vital role in peri anchor cyst formation. Tear size, degree of retraction, number of anchors and varying bone density within the humeral head are all important biomechanical factors. Further investigation is required into certain aspects of rotator cuff surgery to improve our understanding of peri anchor cyst occurrence. From a biomechanical perspective these include: Anchor configuration to both the tear and each other and also tear type itself. From a biochemical perspective we need to further investigate the anchor suture material. It would also be of benefit if a validated grading criteria of peri anchor cysts was produced.

The nasopharyngeal bursa is an invaginated space that links the remnants of the notochord with the pharyngeal epithelium. Nasopharyngeal bursitis can occur in the midline of the upper portion of the nasopharynx if the notochord remnants and midline recess continue to persist. Nasopharyngeal bursitis, also known as Tornwaldt cyst, can be divided into cystic and crusting types. Crust types are relatively rare. Electrocauterization of the ulcerative lesion of the crust type is an effective and minimally invasive method. This report presents a case of crust-type nasopharyngeal bursitis that heals spontaneously without electrocauterization therapy.

Objectives: We have previously shown that pituitary cysts may affect growth hormone secretion. This study sought to determine cyst evolution during growth hormone treatment in children., Methods: Forty-nine patients with short stature, a pituitary cyst, and at least two brain MRI scans were included. The percent of the pituitary gland occupied by the cyst (POGO) was calculated, and a cyst with a POGO of ≤15% was considered small, while a POGO >15% was considered large., Results: Thirty-five cysts were small, and 14 were large. Five of the 35 small cysts grew into large cysts, while 6 of the 14 large cysts shrunk into small cysts. Of 4 cysts that fluctuated between large and small, 3 presented as large and 1 as small. Small cysts experienced greater change in cyst volume (CV) (mean=61.5%) than large cysts (mean=-0.4%). However, large cysts had a greater net change in CV (mean=44.2 mm 3 ) than small cysts (mean=21.0 mm 3 ). Older patients had significantly larger mean pituitary volume than younger patients (435.4 mm 3 vs. 317.9 mm 3 ) and significantly larger mean CV than younger patients (77.4 mm 3 vs. 45.2 mm 3 ), but there was no significant difference in POGO between groups., Conclusions: Pituitary cyst size can vary greatly over time. Determination of POGO over time is a useful marker for determining the possibility of a pathologic effect on pituitary function since it factors both cyst and gland volume. Large cysts should be monitored closely, given their extreme, erratic behavior., (© 2022 Walter de Gruyter GmbH, Berlin/Boston.)

Normal Cowper glands and ducts are imperceptible at imaging. However, abnormalities of the Cowper glands and ducts are increasingly seen owing to increasing use of cross-sectional imaging. In this article, the authors present a comprehensive review of the normal anatomy of the Cowper glands and ducts and the clinical and imaging findings of conditions that affect them in an effort to help lead to appropriate diagnosis and management. A Cowper duct syringocele is a congenital or acquired dilatation of the Cowper duct in the bulb of the corpus spongiosum. Retrograde urethrography is the standard test to diagnose a communicating (open) Cowper duct syringocele, and MRI is used for further morphologic assessment. Cowperitis refers to inflammation of the gland and/or duct, and recurrent cowperitis is usually associated with an underlying syringocele, a urethral fistulous connection to the Cowper gland, or a perianal fistula. MRI allows detection of the underlying structural abnormality and the superimposed inflammation. Cowper stones are typically radiopaque and are found in both the Cowper gland and duct. A Cowper gland hemorrhagic cyst may be incidentally detected at MRI of the prostate, and follow-up MRI may be required to ensure evolution of hemorrhage and exclude an underlying neoplasm. Cowper gland carcinoma is rare and includes adenocarcinoma and adenoid cystic carcinoma subtypes. Transperineal US may be used for initial evaluation and CT and/or MRI are necessary for further characterization and staging. Cowper gland hyperplasia is a rare entity with few previous reports that may be incidentally detected at imaging and may mimic malignancy. © RSNA, 2022.

Polycystic kidney disease (PKD) is characterized by the formation and progressive enlargement of fluid-filled cysts due to abnormal cell proliferation. Cyclic AMP agonists, including arginine vasopressin, stimulate ERK-dependent proliferation of cystic cells, but not normal kidney cells. Previously, B-Raf proto-oncogene (BRAF), a MAPK kinase kinase that activates MEK-ERK signaling, was shown to be a central intermediate in the cAMP mitogenic response. However, the role of BRAF on cyst formation and enlargement in vivo had not been demonstrated. To determine if active BRAF induces kidney cyst formation, we generated transgenic mice that conditionally express BRAF V600E , a common activating mutation, and bred them with Pkhd1-Cre mice to express active BRAF in the collecting ducts, a predominant site for cyst formation. Collecting duct expression of BRAFV600E (BRaf CD ) caused kidney cyst formation as early as three weeks of age. There were increased levels of phosphorylated ERK (p-ERK) and proliferating cell nuclear antigen, a marker for cell proliferation. BRaf CD mice developed extensive kidney fibrosis and elevated blood urea nitrogen, indicating a decline in kidney function, by ten weeks of age. BRAF V600E transgenic mice were also bred to Pkd1 RC/RC and pcy/pcy mice, well-characterized slowly progressive PKD models. Collecting duct expression of active BRAF markedly increased kidney weight/body weight, cyst number and size, and total cystic area. There were increased p-ERK levels and proliferating cells, immune cell infiltration, interstitial fibrosis, and a decline in kidney function in both these models. Thus, our findings demonstrate that active BRAF is sufficient to induce kidney cyst formation in normal mice and accelerate cystic disease in PKD mice., (Copyright © 2022 International Society of Nephrology. Published by Elsevier Inc. All rights reserved.)

Fine needle aspiration is a well-known procedure for the diagnosis and management of thyroid lesions, representing the first diagnostic tool for the definition of their nature. In clinical practice, a thyroid nodule can be classified as solid, cystic, and partially cystic based on its internal components. Different thyroid imaging reporting systems and cytologic diagnostic systems have focused their attention on solid nodules, which are more frequently linked with a malignant outcome. In fact, numerous papers demonstrated that nodules with microcalcifications, a taller-than-wide shape, hypoechogeneity, and irregular margins, are more likely to be malignant on histology. Nevertheless, according to the literature, the risk of malignancy in a partially cystic thyroid nodule ranges between 3.3 and 17-5%, including, for instance, the possible diagnosis of a cystic papillary thyroid carcinoma and other malignant entities. Therefore, in the current review article, we are going to discuss the approach to thyroid cystic lesions on fine needle aspiration cytology., Competing Interests: The authors have no funding or conflicts of interest to disclose., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

A 7-year-old nulliparous Chinchilla queen was admitted to a veterinary clinic for routine ovariohysterectomy. Examination of the uterus, greater omentum and varicose ovarian veins revealed multiple thin-walled cysts filled with a transparent fluid over the serosal surfaces of these organs. Histologically, the cysts were of variable size, filled with a small amount of mucinous fluid, and had thin walls that contained hypocellular collagen and a few small calibre vessels. The inner and outer epithelium of the cyst walls and the cells that lined the uterine serosa were immunopositive for cytokeratin and vimentin, suggesting a mesothelial origin. Alpha-smooth muscle actin immunolabelling was patchy or continuous in smooth muscle in the wall of many of the cysts., (Copyright © 2022 Elsevier Ltd. All rights reserved.)

An 18-y-old female tufted deer ( Elaphodus cephalophus ) had a short history of chronic diarrhea, progressive weight loss, and hindlimb instability. Given the poor prognosis, the deer was euthanized and submitted for postmortem examination. The most significant gross finding was segmental and multinodular mural thickening of the proximal colon. On cut surface of the affected colonic segments, 0.5-2-cm diameter, intramural, multiloculated, cystic structures containing gray, translucent, gelatinous material elevated the edematous mucosa. Microscopically, the intramural cystic structures were filled with mucinous matrix admixed with foamy macrophages, and lined by discontinuous segments of well-differentiated columnar, pancytokeratin-positive epithelium with basilar nuclei. Multifocally, transition was observed from hyperplastic mucosal crypt epithelium to dysplastic or neoplastic columnar and flattened epithelium lining submucosal and serosal cysts. Cyst lumina were irregularly disrupted by polypoid ingrowths of collagenous tissue covered by attenuated epithelium. Based on these findings, we diagnosed a well-differentiated mucinous adenocarcinoma. Although intestinal adenocarcinomas have been described in humans and animals, they are considered uncommon in most domestic species, except for sheep, for which genetic and environmental factors appear to influence occurrence. Our report addresses the knowledge gap regarding intestinal adenocarcinomas affecting cervids and specifically the tufted deer, a less-studied, near-threatened Asian cervid.

Diffuse cystic lung disease refers to multiple rounded lucencies or low-attenuating areas with well-defined interfaces with normal lung. Parenchymal lucencies, such as cavitary disease, may mimic cystic lung disease. Cystic lung disease generally has a nonspecific presentation. Pulmonary cysts may present in isolation or with ancillary imaging features, such as ground-glass opacities or nodules. Clinical features, such as connective tissue disease, can narrow the differential diagnosis. In cases with indeterminate imaging and clinical features, open lung biopsy should be considered. Ultrarare cystic lung diseases, such as light chain deposition disease, can mimic more common diseases., Competing Interests: Disclosure The authors have nothing to disclose., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Mullerian cysts are mostly described in women, near the genitourinary organs. Exceptional cases of retroperitoneal and posterior mediastinal located Mullerian cysts have been reported. When located in the retroperitoneum, those cysts are often confused with other retroperitoneal cysts such as cystic lymphangioma and cystic mesothelioma of peritoneum because of the poor clinical picture and a nonspecific radiologic presentation. The histopathological analysis is essential to make the diagnosis, and the positive staining of a Müllerian marker is a decisive argument in favor of a Müllerian cyst. Here, we report a case of an incidental finding of a retroperitoneal Müllerian cyst in a 68-years-old woman., (Copyright © 2022 Elsevier Masson SAS. All rights reserved.)

Background: Postoperative sialoceles and fistulas are frequent surgical complications of parotid tumor resection. Extracapsular dissection by the sternocleidomastoid muscle-parotid space approach (ECD-SMPSA) is a minimally invasive technique. To our knowledge, the characteristics of sialoceles and fistulas secondary to ECD-SMPSA have not been reported., Methods: This prospective study enrolled 52 patients who underwent ECD-SMPSA without sialocele/fistula prevention measures. Postoperative sialoceles and fistulas were evaluated during 2 months of follow-up., Results: Among the 52 patients, only one male patient developed a mild sialocele. No salivary fistulas occurred. The overall rate of sialocele/fistula formation was 1.92%., Conclusions: When treating clinically benign tumors that involve the sternocleidomastoid muscle-parotid space, ECD-SMPSA may prevent postoperative formation of sialoceles and salivary fistulas., (© 2022 Wiley Periodicals LLC.)

Purpose: Loss of retinoschisin (RS1) function underlies X-linked retinoschisis (XLRS) pathology. In the retina, both photoreceptor inner segments and bipolar cells express RS1. However, the loss of RS1 function causes schisis primarily in the inner retina. To understand these cell type-specific phenotypes, we decoupled RS1 effects in bipolar cells from that in photoreceptors., Methods: Bipolar cell transgene RS1 expression was achieved using two inner retina-specific promoters: (1) a minimal promoter engineered from glutamate receptor, metabotropic glutamate receptor 6 gene (mini-mGluR6/ Grm6) and (2) MiniPromoter (Ple155). Adeno-associated virus vectors encoding RS1 gene under either the mini-mGluR6 or Ple-155 promoter were delivered to the XLRS mouse retina through intravitreal or subretinal injection on postnatal day 14. Retinal structure and function were assessed 5 weeks later: immunohistochemistry for morphological characterization, optical coherence tomography and electroretinography (ERG) for structural and functional evaluation., Results: Immunohistochemical analysis of RS1expression showed that expression with the MiniPromoter (Ple155) was heavily enriched in bipolar cells. Despite variations in vector penetrance and gene transfer efficiency across the injected retinas, those retinal areas with robust bipolar cell RS1 expression showed tightly packed bipolar cells with fewer cavities and marked improvement in inner retinal structure and synaptic function as judged by optical coherence tomography and electroretinography, respectively., Conclusions: These results demonstrate that RS1 gene expression primarily in bipolar cells of the XLRS mouse retina, independent of photoreceptor expression, can ameliorate retinoschisis structural pathology and provide further evidence of RS1 role in cell adhesion.

Introduction: Cystic meningiomas are rare, accounting for 2-7% of all intracranial meningiomas. Little is known regarding whether these meningiomas behave differently compared to solid meningiomas. We sought to study this relatively uncommon imaging appearance of meningioma and to evaluate its clinical significance., Methods: A single-institution retrospective cohort study of surgically-treated meningioma patients between 2000 and 2019 was conducted. Cystic meningioma was defined as a tumor with an intratumoral or peritumoral cyst present on preoperative imaging. Demographics, preoperative imaging, histopathology characteristics, operative data, and surgical outcomes were reviewed. Imaging variables, histopathology and outcomes were reported for cystic meningiomas and compared with non-cystic meningiomas. Univariate/multivariable analyses were conducted., Results: Of 737 total meningiomas treated surgically, 38 (5.2%) were cystic. Gross total resection (GTR) was achieved in 84.2% of cystic meningioma patients. Eighty-two percent of cystic meningiomas were WHO grade I (n = 31), 15.7% were grade II and 2.6% were grade III. Most cystic meningiomas had low Ki-67/MIB-1 proliferation index (n = 24, 63.2%). A total of 18.4% (n = 7) patients with cystic meningioma had recurrence compared to 12.2% (n = 80) of patients with non-cystic meningioma (p = 0.228). No significant difference in median time to recurrence was observed between cystic and non-cystic meningiomas (25.4, Q1:13.9, Q3:46.9 months vs. 13.4, Q1:8.6, Q3:35.5 months, p = 0.080)., Conclusions: A small portion of intracranial meningiomas have cystic characteristics on imaging. Cystic meningiomas are frequently WHO grade I, have low proliferation index, and had similar outcomes compared to non-cystic meningioma. Cysts in meningioma may not be a surrogate to determine aggressive meningioma behavior., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: The Milan System for Reporting Salivary Gland Cytopathology (MSRSGC) reports a 25% rate of malignancy (ROM) for the Milan I: Nondiagnostic (ND) category. We clarify the ROM of ND salivary gland fine-needle aspirations (SGFNAs) based on our institutional experience and review of the literature., Methods: Overall risk of malignancy (OROM) and that for those with surgical/flow cytometric follow-up (FROM) for each category and "all-comers" were calculated for Emory SGFNAs from January 2010 through March 2021. From a literature review of 50 articles using MSRSGC, distribution of diagnoses, rates of follow-up, FROM, and OROM by category were calculated. FROMs and OROMs between ND FNAs and all-comers were compared. Milan I rate was compared with the ratio of Milan I OROM to all-comer OROM., Results: Of 819 SGFNAs at Emory, 12.8% (n = 105/819) were ND. Thirty-two had known follow-up, with 12 (37.5%) being malignant. Nonmucinous cyst contents accounted for 26.7% of ND SGFNAs (n = 28/105); all 7 with surgical follow-up were benign. Of 50 MSRSGC studies, 18.2% (n = 2384/13,129) of SGFNAs were classified as ND, 26.6% (n = 635/2384) with known follow-up. Total FROM and OROM for ND FNAs (15.7% and 4.1%, respectively) were significantly lower than those for all-comers (24.9% and 11.4%, respectively) (p < .001). There was no relationship between rate of ND SGFNA and ND ROM., Conclusions: The ND category is associated with a lower ROM than that of all-comer SGFNA patients. The "true" ROM for ND SGFNAs is likely best estimated by the 4.1% OROM. SGFNAs showing nonmucinous cyst contents have a particularly low ROM. Rate of ND SGFNAs does not influence ND ROM., (© 2022 American Cancer Society.)

Background: Peribronchiolar metaplasia (PBM) is a lesion characterized by an abnormal connection between the terminal bronchiole and parabronchiole via the Lambert's canals. We report a rare case of PBM incidentally detected during a surgery for pneumothorax., Case Presentation: At 38-year-old man was admitted to our hospital with chest pain. He was diagnosed with pneumothorax and treated using a thoracic drain 12 years ago. Chest computed tomography revealed a cyst in the right upper lobe and ground glass lesion with a solid component in the right lower lobe of the lung. Hence, we performed a surgery for pneumothorax management, which revealed a cyst in the right upper lobe and induration with angiogenesis in the right lower lobe. We performed partial resection of the right upper and lower lobes. Pathological examination of the lower lobe nodule revealed small airways with lymphocytic inflammation and bronchiolar metaplasia. Pathological diagnosis of the nodule was PBM., Conclusion: Although PBM is considered a lesion with good prognosis, there have been cases associated with early-stage lung cancer. Hence, care should be taken to distinguish PBM from other neoplasms. However, preoperative diagnosis is difficult in most cases so complete surgical resection is recommended, if feasible.

The diagnosis of cystic liver disease has made great progress with the advent of enhanced imaging techniques. At the same time, its management has gradually improved over the past few decades, providing the basis for the development of appropriate diagnostic and treatment guidelines. To this end, the European Association for the Study of the Liver has developed clinical guidelines for the diagnosis and treatment of non-infectious cystic liver disease. This guideline put forward recommendations based on an in-depth review of the relevant literature for addressing clinical issues, including the diagnosis and treament of hepatic cysts, hepatic mucocystic tumors, biliary hamartomas, polycystic liver disease, Caroli disease or Caroli syndrome, biliary hamartomas, and peribiliary cyst.

Background: Facet cysts in the thoracic spine are a rare cause of thoracic myelopathy. We aimed to investigate the clinical/radiological features and surgical results of these lesions., Methods: Nine thoracic facet cysts in eight patients (seven men, one woman) were diagnosed based on magnetic resonance imaging (MRI) and computed tomography (CT) with facet arthrography findings and surgically treated. The mean patient age was 71 (59-83) years. The cysts were distributed as follows: one each at T8-9 and T9-10, two each at T1-2 and T11-12, and three at T10-11. The mean follow-up period was 1.8 (1-5) years. Clinical and radiological features were retrospectively investigated, and surgical outcomes were evaluated according to modified Japanese Orthopaedic Association (JOA) scores for thoracic myelopathy (full score: 11)., Results: Neurological examination revealed progressive thoracic transverse myelopathy in all patients with a mean disease duration of 1.2 months (2 weeks-2 months). MRI revealed a total of nine cysts across the eight patients: four in the median region and five in the paramedian portion of the spinal canal. CT revealed degeneration in all involved facet joints. All the cysts were in communication with the neighboring facet joint confirmed by CT facet arthrography. All patients underwent bilateral fenestration, and the cysts were resected with the ligamentum flavum. The mean preoperative and postoperative modified JOA scores were 4.5 and 8.8, respectively. The mean recovery rate was 67.5%. Differences in the degree of local kyphosis were 2° or less between before and after surgery. On histopathology, synovial lining cells were not noted in any case., Conclusions: Decompression surgery is recommended for treating progressive myelopathy in patients with cystic lesions. Our study suggests that thoracic facet cyst resection with satisfactory surgical outcomes would be possible through fenestration., Competing Interests: Declaration of competing interest The authors declare that they have no conflict of interest., (Copyright © 2021 The Japanese Orthopaedic Association. Published by Elsevier B.V. All rights reserved.)

Angiomyxoma of the umbilical cord is a rare benign vascular malformation with potential for fetal morbidity and mortality. Gross and histologic features of this lesion are identical to those described as "hemangioma," however "angiomyxoma" is a preferable term as current practice restricts the term "hemangioma" to infantile capillary proliferations that express glucose transporter 1. Here we describe the case of an umbilical cord angiomyxoma with associated pseudocysts diagnosed after delivery at 33 weeks. It presented as a heterogeneous-appearing mass near the fetal cord insertion and mimicked serious fetal anomalies throughout gestation. We found fetal MRI helpful for monitoring this lesion, narrowing the differential, and informing management. Proximity to the fetal end of the cord and uncertainty about diagnosis also required surgical resection of the mass after delivery with umbilicoplasty.

Purpose: Penetrance estimates of Birt-Hogg-Dubé syndrome (BHD)-associated cutaneous, pulmonary, and kidney manifestations are based on clinically ascertained families. In a health care system population, we used a genetics-first approach to estimate the prevalence of pathogenic/likely pathogenic (P/LP) truncating variants in FLCN, which cause BHD, and the penetrance of BHD-related phenotypes., Methods: Exomes from 135,990 patient-participants in Geisinger's MyCode cohort were assessed for P/LP truncating FLCN variants. BHD-related phenotypes were evaluated from electronic health records. Association between P/LP FLCN variants and BHD-related phenotypes was assessed using Firth's logistic regression., Results: P/LP truncating FLCN variants were identified in 35 individuals (1 in 3234 unrelated individuals), 68.6% of whom had BHD-related phenotype(s), including cystic lung disease (65.7%), pneumothoraces (17.1%), cutaneous manifestations (8.6%), and kidney cancer (2.9%). A total of 4 (11.4%) individuals had prior clinical BHD diagnoses., Conclusion: In this health care population, the frequency of P/LP truncating FLCN variants is 60 times higher than the previously reported prevalence. Although most variant-positive individuals had BHD-related phenotypes, a minority were previously clinically diagnosed, likely because cutaneous manifestations, pneumothoraces, and kidney cancer were observed at lower frequencies than in clinical cohorts. Improved clinical recognition of cystic lung disease and education concerning its association with FLCN variants could prompt evaluation for BHD., Competing Interests: Conflict of Interest D.H.L is an employee of Unified Patient Network, Inc and scientific consultant for Natera, Inc; MyOme, Inc; and Seven Bridges Genomics. All other authors declare no conflicts of interest., (Copyright © 2022 The Authors. Published by Elsevier Inc. All rights reserved.)