Features of Remyelination after Transplantation of Olfactory Ensheathing Cells with Neurotrophic Factors into Spinal Cord Cysts.

This paper shows for the first time that co-transplantation of human olfactory ensheathing cells with neurotrophin-3 into spinal cord cysts is more effective for activation of remyelination than transplantation of cells with brain-derived neurotrophic factor and a combination of these two factors. The studied neurotrophic factors do not affect proliferation and migration of ensheathing cells in vitro. It can be concluded that the maximum improvement of motor function in rats receiving ensheathing cells with neurotrophin-3 is largely determined by activation of remyelination.

[Main Types of Cysts in Dermatopathology: Part 2]. / Principales tipos de quistes en dermatopatología: Parte 2.

This is the second article in a two-part series published in this journal, in which we examine the histopathological characteristics, as well as the differential diagnosis, of the main entities that present as cystic and pseudocystic structures in cutaneous biopsy. In this second article, we address ciliated cutaneous cysts, branchial cysts, Bartholin's cysts, omphalomesenteric cysts, thymic cysts, thyroglossal duct cysts, synovial cysts, and median raphe cysts, as well as mucocele, ganglion, and auricular and digital myxoid pseudocysts.

Clinical characteristics and surgical outcomes of transcutaneous versus transconjunctival excision of Wolfring gland ductal cysts.

PURPOSE: To analyze the clinicopathological characteristics and surgical outcomes of patients with Wolfring gland ductal cysts (WGDCs). METHODS: A retrospective, consecutive, interventional comparative case series was performed over a period of 7 years. Data on demographic and clinical characteristics, pathological findings and outcomes of surgically excised cysts were collected. A comparison between the transconjunctival and transcutaneous approaches was also assessed. RESULTS: Forty-eight patients (48 eyelids) were included in the final analysis. The most common presenting symptom was painless eyelid swelling (81.3%). The median symptom duration was 11.5 months (IQR, 18.25). The upper eyelid was involved in 31 (64.6%) patients, 29/31 of whom had cysts in a medial or centromedial location. Forty-five (93.8%) cysts were bluish gray and transilluminable with clear contents on lid eversion and a median largest dimension of 22 mm (IQR, 8). A transverse conjunctival fibrotic band was observed along the proximal tarsal border in the cyst area in all patients. Signs of chronic trachoma were noted in 38 (79.2%) patients. Preoperative significant ptosis was present in 28/31 (90.3%) of the upper eyelid cysts. Thirty cysts (62.5%) were excised through the skin, and 18 cysts (37.5%) were excised transconjunctivally. Intraoperative cyst rupture, the need for conjunctival grafting and postoperative residual upper lid ptosis were significantly greater in the transconjunctival group (p = 0.009, p < 0.001, and p = 0.016, respectively). CONCLUSION: The present study highlights the clinicopathological characteristics of a relatively large series of surgically excised WGDCs. Transcutaneous excision of WGDCs has proven to be an effective treatment with fewer adverse sequelae than the transconjunctival approach.

Enteric duplication cysts in paediatric population along with literature review.

The clinical presentation of enteric duplication cysts is dependent on the location of the cyst with symptoms varying from nausea and vomiting to abdominal distension, pain and perforation. Four patients were identified who were diagnosed with enteric duplication cysts within the period from 2019 to 2023. Three of the patients presented with signs of intestinal obstruction-abdominal distension and pain, while one had an antenatally detected abdominal mass. There were three boys and one girl with ages ranging from 4 months to 14 years. Three cases of ileal and one case of caecal duplication cyst were reported. Most of the cases showed ileal/caecal mucosa while one case demonstrated ectopic gastric mucosa. The treatment of these cysts includes surgical excision. Although radiological investigations help in arriving at a provisional diagnosis, the final diagnosis can be confirmed only after histopathological examination. Early treatment prevents complications and results in a good prognosis for the patient.

Phenotypes and outcome of diffuse pulmonary non-amyloid light chain deposition disease.

BACKGROUND: Light chain deposition disease (LCDD) is a very rare entity. Clinical manifestations of LCDD vary according to the organs involved. Data on pulmonary LCDD are scarce and limited to small series or case reports. This study aimed to describe the characteristics and outcome of diffuse pulmonary non-amyloid LCDD localized to the lungs. STUDY DESIGN AND METHODS: A multicenter retrospective cohort study was conducted. Clinical characteristics were collected, and chest CTs were centrally reviewed. The diagnosis of pulmonary non-amyloid LCDD was confirmed by immunohistochemistry. RESULTS: Thirty-one cases were identified (68% female), with a median age at diagnosis of 50 years (IQR 20). Baseline FEV1/FVC was < 0.70 in 45% of patients. Mean (± SD) FEV1 and DLCO were 86% ± 26.2 and 52% ± 23.9, respectively. CT revealed peculiar patterns of thin-walled cysts (58%) and thin-walled cystic bronchiectases (27%). Increased serum kappa light chain was found in 87% of patients. Histological analysis showed kappa light chain deposits in all patients, except one with lambda chain deposits. Median annual FEV1 decline was 127 ml (IQR 178) and median DLCO decline was 4.3% (IQR 4.3). Sixteen patients received immunomodulatory treatment or chemotherapy; serum light chain levels decreased in 9 cases (75%), without significant improvement in FEV1 (p = 0.173). Overall, 48% of patients underwent bilateral lung transplantation. Transplant-free survival at 5 and 10 years were 70% and 30%, respectively. An annual FEV1 decline greater than 127 ml/year was associated with increased risk of death or transplantation (p = 0.005). CONCLUSIONS: Diffuse pulmonary LCDD is characterised by female predominance, a peculiar imaging pattern with bronchiectasis and/or cysts, progressive airway obstruction and severe DLCO impairment, and poor outcome. Lung transplantation is a treatment of choice.

Orofacial Cysts: A Single Institution Experience of 85 Cases in Enugu, Nigeria.

BACKGROUND: Orofacial cysts are pathologic cavities that could be symptomatic and may cause facial disfigurement. The only epidemiologic report of such lesions in Southeast Nigeria studied jaw cysts from 1987 to 1996. New studies reflecting recent research findings and classifications on the subject in Southeast Nigeria are lacking. AIM: To determine the prevalence and distribution of orofacial cysts in a tertiary hospital in Enugu, Southeast Nigeria. METHODS: A 10-year retrospective study of patients with orofacial cysts diagnosed by histology was carried out. RESULTS: Orofacial cysts constitute 9.5% (85) of 897 orofacial lesions identified. The male-to-female gender ratio was 1.2:1. The mean age (± standard deviation) at the onset of the cystic lesion was 28.58 (±16.98) years. Developmental odontogenic cysts 52.9% (45) and salivary cysts 18.8% (16) were the most common group of orofacial cysts. The most prevalent orofacial cysts were odontogenic keratocysts at 25.9% (22), mucoceles 16.5% (14), and dentigerous cysts 14.1% (12). Straw-colored aspirates 34.8% (16) and dark brown aspirates 28.3% (13) were the predominant cystic contents. The mandible 45.9% (39) and maxilla 27.1% (23) were the commonest sites for orofacial cysts, while the lip 9.4% (8) was the most frequent soft tissue site. A significant association exists between anatomical site and cyst type at a 95% confidence interval with P = 0.000, X2 = 247.17. Unilocular radiolucency 62.5% (20) and multilocular radiolucency 34.4% (11) were the most common radiographic features. CONCLUSION: Developmental odontogenic cysts particularly odontogenic keratocysts were most prevalent while mucocele was the most common soft tissue cyst.

Giant intraperitoneal non-pancreatic pseudocyst: a case report.

INTRODUCTION: Non-pancreatic pseudocysts are rare lesions that typically form from the omentum and mesentery. These cysts have a thick fibrotic wall made up of fibrous tissue and may show signs of calcifications and inflammatory changes. The fluid inside them can vary, ranging from hemorrhage and pus to serous or sometimes chylous content. In most cases, these cysts appear as a result of trauma, surgery, or infection. CASE PRESENTATION: A 35-year-old male patient from Ethiopia presented with swelling in his lower abdomen that had been present for 2 years. Initially, the swelling was small but gradually increased in size. The patient experienced frequent urination but no pain or difficulty during urination, urgency, intermittent urination, or blood in the urine. The swelling was initially painless but became painful 2 months prior to his presentation. Abdominal computed tomography scans revealed a well-defined, lobulated peritoneal lesion measuring 16 × 12 × 10 cm, consisting primarily of fluid-filled cysts with a thick, enhancing wall and septa. Additionally, there was a large, heterogeneous enhancing soft tissue component measuring 8 × 6 cm. As a result, the cystic mass was surgically removed in its entirety with partial removal of the bladder wall, and the patient was discharged in an improved condition. CONCLUSION: Primary non-pancreatic pseudocysts are extremely rare lesions that must be differentiated from other possible causes of cystic lesions within the peritoneal or retroperitoneal regions. Surgeons should be aware of the potential occurrence of these lesions, which may have an unknown origin.

Cytological Features of Fibrin-associated Diffuse Large B-cell Lymphoma Arising in Retroperitoneal Pseudocyst: The First Case Report.

BACKGROUND/AIM: Fibrin-associated diffuse large B-cell lymphoma (FA-DLBCL) is frequently associated with the Epstein-Barr virus (EBV) and manifests as non-mass-forming microscopic lesions within fibrin-rich lesions. Herein, we describe the cytological features of FA-DLBCL. CASE REPORT: A 72-year-old man presented with a large retroperitoneal cystic mass that was treated by cyst aspiration and laparoscopic excision. Individually dispersed large, atypical cells in a necrotic background contained scant cytoplasm and hyperchromatic nuclei with irregular nuclear contours, frequent karyorrhectic debris, and mitotic figures. A fibrinous exudate with necrotic material attached to the inner surface of the cystic mass contained large, atypical cells that were individually scattered or arranged in small clusters. These were positive for cluster of differentiation 20 and Epstein-Barr virus-encoded RNA in situ hybridization. CONCLUSION: We cytologically characterized FA-DLBCL as large, atypical cells that were individually scattered or arranged in small clusters in a necrotic background. To the best of our knowledge, we revealed the cytological features of FA-DLBCL.

[Aging-associated Cyst Formation and Fibrosis].

Cysts are abnormal fluid-filled sacs found in various human organs, including the liver. Liver cysts can be associated with known causes such as parasite infections and gene mutations, or simply aging. Among these causes, simple liver cysts are often found in elderly people. While they are generally benign, they may occasionally grow but rarely shrink with age, indicating their clear association with aging. However, the mechanism behind the formation of simple liver cysts has not been thoroughly investigated. Recently, we have generated transgenic mice that specifically overexpress fibroblast growth factor (FGF)18 in hepatocytes. These mice exhibit severe liver fibrosis without inflammation and spontaneously develop liver cysts that grow with age. Our findings suggest that simple liver cysts can be induced by fibrosis accompanied by sterile inflammation or injury, whereas fibrosis accompanied by severe inflammation or injury may lead to cirrhosis. We also discuss the detrimental effects of disease- and aging-associated fibrosis in various organs, such as the heart, lungs, and kidneys. Additionally, we provide a brief summary of the two currently approved anti-fibrotic drugs for idiopathic pulmonary fibrosis, nintedanib and pirfenidone, as well as their possibility of future expansion of application toward other fibrotic diseases.

An elderly woman with Birt-Hogg-Dubé syndrome having multiple pulmonary cysts mimicking lymphangioleiomyomatosis.

The characteristics of the pulmonary cysts on the high-resolution computed tomography (HRCT) chest images are an important diagnostic clue to distinguish among cystic lung diseases. The diagnostic accuracy of HRCT was reported to be as high as 90% by experienced pulmonologists and radiologists. Herein, we report the case of an elderly woman with Birt-Hogg-Dubé syndrome (BHDS) whose HRCT images displayed lymphangioleiomyomatosis-like features of the pulmonary cysts, rendering it difficult for us to diagnose BHDS. This case illustrates the significance of a thorough anamnesis, physical examination, and skin biopsy of facial papules to establish an accurate diganosis.

Systematic review on surveillance for non-resected branch-duct intraductal papillary mucinous neoplasms of the pancreas.

BACKGROUND: The management of branch-duct type intraductal papillary mucinous neoplasms (BD-IPMN) varies in existing guidelines. This study investigated the optimal surveillance protocol and safe discontinuation of surveillance considering natural history in non-resected IPMN, by systematically reviewing the published literature. METHODS: This review was guided by PRISMA. Research questions were framed in PICO format "CQ1-1: Is size criteria helpful to determine surveillance period? CQ1-2: How often should surveillance be carried out? CQ1-3: When should surveillance be discontinued? CQ1-4: Is nomogram predicting malignancy useful during surveillance?". PubMed was searched from January-April 2022. RESULTS: The search generated 2373 citations. After screening, 83 articles were included. Among them, 33 studies were identified for CQ1-1, 19 for CQ1-2, 26 for CQ1-3 and 12 for CQ1-4. Cysts <1.5 or 2 cm without worrisome features (WF) were described as more indolent, and most studies advised an initial period of surveillance. The median growth rate of cysts <2 cm ranged from 0.23 to 0.6 mm/year. Patients with cysts <2 cm showing no morphological changes and no WF after 5-years of surveillance have minimal malignancy risk of 0-2%. Two nomograms created with over 1000 patients had AUCs of around 0.8 and appear to be feasible in a real-world practice. CONCLUSIONS: For patients with suspected BD-IPMN <2 cm and no other WF, less frequent surveillance is recommended. Surveillance may be discontinued for cysts that remain stable during 5-year surveillance, with consideration of patient condition and life expectancy. With this updated surveillance strategy, patients with non-worrisome BD-IPMN should expect more streamlined management and decreased healthcare utilization.

Can imaging determine if a rotator cuff tear is traumatic?

AIM: We reviewed the last decade of literature to update a previous publication on this topic by the senior author. In New Zealand, traumatic causation has implications for entitlement for treatment though the Accident Compensation Corporation (ACC). Acuity and chronicity may also be relevant in determining repairability. METHODS: Literature was reviewed regarding acromial morphology, greater tuberosity (GT) cysts, acromiohumeral interval (AHI), fatty degeneration and atrophy, acromioclavicular (AC) arthrosis, tendinopathy, bursal changes and other features. RESULTS: Some factors can be considered normal for those middle aged and older, including AC arthrosis, type 1 and 2 acromion and tendinopathy. Some factors may indicate acuity, including haemorrhage and debris, GT oedema, mid-substance soft tissue tear, kinking of the tendon and isolated complete subscapularis tears. Other factors may be associated with chronicity, including significant fatty degeneration, positive tangent sign for atrophy, anterior GT cysts, type 3 acromion, critical shoulder angle (CSA) >35 degrees and acromial index (AI) <0.7. CONCLUSION: A multitude of factors on imaging may infer, to a varying degree, the likelihood of acuity or chronicity. The patient history is also of importance in determining causation.

Early Pneumatocele Identification Post-Robotic Assisted Bronchoscopy Cryobiopsy: A Case Series Experience.

INTRODUCTION: The use of cryobiopsy in conjunction with robotic assisted bronchoscopy is on the rise due to the safety and increased diagnostic yield of cryobiopsy. The incorporation of 3D fluoroscopy in the procedure improves the workflow and helps confirm the accuracy of sampling of peripheral pulmonary nodules. METHODS: We describe an observational series of 12 patients comprising 14 nodules where cryobiopsy was performed during shape-sensing robot-assisted bronchoscopy cryobiopsy under general anesthesia. 3D fluoroscopy was used to confirm accurate placement of the cryoprobe. All these patients underwent a second spin with the 3D fluoroscopy either to sample a second lesion intraoperatively or to investigate suspected pneumothorax. RESULTS: The development of a pneumatocele was noted after cryobiopsy in each of the cases. The majority of these were in the upper lobe with the median size of a sampled nodule being 14 mm. The majority of patients were asymptomatic with 1 patient developing mild hemoptysis and 4 patients developing chest tightness or dyspnea. None of the patients required an intervention for the pneumatocele. CONCLUSION: The development of pneumatoceles appears to be a fairly frequent and benign occurrence following cryobiopsy, likely due to increased tissue destruction. The increased use of intraoperative 3D fluoroscopy is likely to highlight changes to the pulmonary parenchyma that were previously not known. The occurrence of pneumatoceles does not appear to adversely impact the safety or tolerability profile of cryobiopsy.

Inhibiting centrosome clustering reduces cystogenesis and improves kidney function in autosomal dominant polycystic kidney disease.

Autosomal dominant polycystic kidney disease (ADPKD) is a monogenic disorder accounting for approximately 5% of patients with renal failure, yet therapeutics for the treatment of ADPKD remain limited. ADPKD tissues display abnormalities in the biogenesis of the centrosome, a defect that can cause genome instability, aberrant ciliary signaling, and secretion of pro-inflammatory factors. Cystic cells form excess centrosomes via a process termed centrosome amplification (CA), which causes abnormal multipolar spindle configurations, mitotic catastrophe, and reduced cell viability. However, cells with CA can suppress multipolarity via "centrosome clustering," a key mechanism by which cells circumvent apoptosis. Here, we demonstrate that inhibiting centrosome clustering can counteract the proliferation of renal cystic cells with high incidences of CA. Using ADPKD human cells and mouse models, we show that preventing centrosome clustering with 2 inhibitors, CCB02 and PJ34, blocks cyst initiation and growth in vitro and in vivo. Inhibiting centrosome clustering activates a p53-mediated surveillance mechanism leading to apoptosis, reduced cyst expansion, decreased interstitial fibrosis, and improved kidney function. Transcriptional analysis of kidneys from treated mice identified pro-inflammatory signaling pathways implicated in CA-mediated cystogenesis and fibrosis. Our results demonstrate that centrosome clustering is a cyst-selective target for the improvement of renal morphology and function in ADPKD.

Dysgenetic Polycystic Disease of the Salivary Glands: A Case Report of This Rare Entity Occurring for the First Time in the Minor Salivary Glands of the Tongue, and a Review of the Literature.

Dysgenetic polycystic disease, also known just as polycystic disease, is a very rare developmental abnormality affecting the salivary gland duct system. This entity has been reported in only 21 patients previously, although a careful review suggests only 16 patients have histological evidence of the disease. In previously reported cases, this lesion most commonly presents as either an incidental finding or as a swelling affecting the parotid glands bilaterally, or rarely the submandibular glands bilaterally. This case report details the first time dysgenetic polycystic disease is found affecting the minor salivary glands of the tongue in a 55-year-old male. Histochemical and immunohistochemical stains are presented and include positivity for AE1/AE3 and p63, and negativity for progesterone receptor, androgen receptor, mammaglobin, S100 and BRAF V600E. PAS-D and Congo Red highlight special microamyloid spheroliths structures intraluminally.

AI models for automated segmentation of engineered polycystic kidney tubules.

Autosomal dominant polycystic kidney disease (ADPKD) is a monogenic, rare disease, characterized by the formation of multiple cysts that grow out of the renal tubules. Despite intensive attempts to develop new drugs or repurpose existing ones, there is currently no definitive cure for ADPKD. This is primarily due to the complex and variable pathogenesis of the disease and the lack of models that can faithfully reproduce the human phenotype. Therefore, the development of models that allow automated detection of cysts' growth directly on human kidney tissue is a crucial step in the search for efficient therapeutic solutions. Artificial Intelligence methods, and deep learning algorithms in particular, can provide powerful and effective solutions to such tasks, and indeed various architectures have been proposed in the literature in recent years. Here, we comparatively review state-of-the-art deep learning segmentation models, using as a testbed a set of sequential RGB immunofluorescence images from 4 in vitro experiments with 32 engineered polycystic kidney tubules. To gain a deeper understanding of the detection process, we implemented both pixel-wise and cyst-wise performance metrics to evaluate the algorithms. Overall, two models stand out as the best performing, namely UNet++ and UACANet: the latter uses a self-attention mechanism introducing some explainability aspects that can be further exploited in future developments, thus making it the most promising algorithm to build upon towards a more refined cyst-detection platform. UACANet model achieves a cyst-wise Intersection over Union of 0.83, 0.91 for Recall, and 0.92 for Precision when applied to detect large-size cysts. On all-size cysts, UACANet averages at 0.624 pixel-wise Intersection over Union. The code to reproduce all results is freely available in a public GitHub repository.

Dual-task kidney MR segmentation with transformers in autosomal-dominant polycystic kidney disease.

Autosomal-dominant polycystic kidney disease is a prevalent genetic disorder characterized by the development of renal cysts, leading to kidney enlargement and renal failure. Accurate measurement of total kidney volume through polycystic kidney segmentation is crucial to assess disease severity, predict progression and evaluate treatment effects. Traditional manual segmentation suffers from intra- and inter-expert variability, prompting the exploration of automated approaches. In recent years, convolutional neural networks have been employed for polycystic kidney segmentation from magnetic resonance images. However, the use of Transformer-based models, which have shown remarkable performance in a wide range of computer vision and medical image analysis tasks, remains unexplored in this area. With their self-attention mechanism, Transformers excel in capturing global context information, which is crucial for accurate organ delineations. In this paper, we evaluate and compare various convolutional-based, Transformers-based, and hybrid convolutional/Transformers-based networks for polycystic kidney segmentation. Additionally, we propose a dual-task learning scheme, where a common feature extractor is followed by per-kidney decoders, towards better generalizability and efficiency. We extensively evaluate various architectures and learning schemes on a heterogeneous magnetic resonance imaging dataset collected from 112 patients with polycystic kidney disease. Our results highlight the effectiveness of Transformer-based models for polycystic kidney segmentation and the relevancy of exploiting dual-task learning to improve segmentation accuracy and mitigate data scarcity issues. A promising ability in accurately delineating polycystic kidneys is especially shown in the presence of heterogeneous cyst distributions and adjacent cyst-containing organs. This work contribute to the advancement of reliable delineation methods in nephrology, paving the way for a broad spectrum of clinical applications.

Prevalence of thyroid diffuse goiter and its association with body mass index and the presence of cysts and nodules in children and adolescents: the Fukushima Health Management Survey.

The main cause of diffuse thyroid goiter is autoimmune chronic thyroiditis, otherwise known as Hashimoto's thyroiditis. Thyroid hormones play pivotal roles in growth and development during childhood. However, the prevalence of diffuse goiter and the relationships between diffuse goiter, thyroid volume, cysts and nodules, and anthropometric measurements in children are not well known. Among 789,459 participants who participated in thyroid ultrasound examinations, 320,206 participants (male: 161,728; female: 158,478) aged 1-23 years were analyzed. Logistic regression analyses were conducted to calculate the odds ratios of the standard deviation score of body mass index (BMI-SDS), the SDS of bilateral width multiplied thickness area (BWTAR-SDS) as a provisional determination of thyroid volume, and the presence of nodules or cysts for positive diffuse goiter compared with negative diffuse goiter after correction for sex and age. The prevalence of diffuse goiter increased in a female-dominant manner with aging. Compared with the absence of diffuse goiter, the age- and sex-adjusted odds ratios (95% confidence intervals) for BMI-SDS (1 SD), BWTAR-SDS (1 SD), cysts, and nodules were 1.24 (1.21-1.27), 3.21 (3.13-3.29), 0.53 (0.50-0.58), and 1.38 (1.17-1.64), respectively. The odds ratios of nodules for positive diffuse goiter were 4.18 (1.08-16.08), 1.76 (1.01-3.07), 1.80 (1.32-2.45), and 1.34 (1.08-1.67) in the age groups 1-7, 8-11, 12-15, and 16-23 years, respectively. The age-dependent increase in the prevalence of diffuse goiter was independently associated with increased BMI and positive prevalence of nodules in young individuals.

Successful Surgical Resection of an Ependymal Cyst in the Fourth Ventricle of a Dog.

Ependymal cysts represent congenital brain malformations rarely described in human medicine, where surgical resection is the treatment of choice. In veterinary medicine, only three cases have been previously reported, with one partially resected with surgery. A 6 yr old entire male American Staffordshire terrier was referred with a 4 mo history of incoordination and collapsing episodes with extensor rigidity. Neurological examination localized the lesion to the left central vestibular system and cerebellum. A brain computed tomography scan showed a hypoattenuating lesion with peripheral contrast enhancement in the fourth ventricle consistent with a cyst and secondary hydrocephalus. Treatment with prednisone was initiated, but despite an initial improvement, neurologic signs recurred and a suboccipital craniectomy to remove the cyst was performed. The cyst was first drained, and the capsule was carefully resected. The histopathological evaluation revealed a simple cubic to cylindrical epithelium with apical cilia and loose surrounding fibrillar tissue consistent with an intraventricular ependymal cyst. Four and a half years after surgery, the dog only shows short episodes of balance loss when turning abruptly but is otherwise neurologically normal. To the authors' knowledge, this is the first reported ependymal cyst in the fourth ventricle of a dog with successful surgical resection.