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Biliary hamartomas (BHs) are rare malformative cystic/cystic-like lesions of the liver affecting the biliary tree, named after Hanns von Meyenburg who described them for the first time and still known with this eponym to this day. They usually lack clinical symptoms, and abnormalities in liver function tests are unusual; thus, it is typically an incidental finding of liver imaging. Despite being benign lesions, BHs can pose clinical challenges; the first one is differential diagnosis with other more relevant pathological conditions. Therefore, knowledge of MR imaging findings of BHs is helpful for a prompt and correct diagnosis, avoiding unnecessary invasive procedures and/or an excessive number of radiological investigations. This pictorial review is aimed to depict the most typical MR imaging features of multiple biliary hamartomas (von Meyenburg Complex), in order to familiarize the diagnosis and facilitate the differentiation from other hepato-biliary cystic diseases.

Isolated polycystic disease of the pancreas is a rare condition with few reported cases in the literature. It refers to multiple dysontogenetic, or congenital, cysts spanning the pancreas excluding those with inherited autosomal cystic diseases of abdominal organs, cystic fibrosis, and Von Hippen-Lindau disease with or without the involvement of cysts in other organs. These cystic lesions prove difficult to distinguish from those of neoplastic nature, including but not limited to intraductal papillary mucinous neoplasms, mucinous cystic neoplasms, and serous cystic neoplasms, potentially leading to unnecessary treatment. We report an incidental finding of the polycystic pancreas in a 73-year-old male upon CT for diverticulitis without any pancreas-related symptoms. A comprehensive collection of all reported cases of the isolated polycystic pancreas in the literature to date revealed that we report the oldest known patient to be diagnosed, as all other patients are between ages 13–62 with 64% being female. A thorough evaluation of the literature was completed and a diagnostic algorithm to diagnose IPDP was created to assist in the appropriate evaluation of these benign lesions to reduce the morbidity associated with unnecessary surgeries prompted by diagnostic uncertainty and the possibility of pre-malignant lesions.

Congenital and hereditary cystic lesions of the abdomen are relatively rare. Correct diagnosis is critical as they may simulate several other benign and malignant acquired diseases of the abdomen. With the correct and appropriate use of imaging, diagnosis may be relatively straightforward and clinical management may be implemented appropriately. The purpose of this article is to describe imaging findings of common and uncommon congenital and hereditary cystic disease of the abdominal organs.

Pediatric renal cystic diseases include a variety of hereditary or non-hereditary conditions. Numerous classifications exist and new data are continuously published. Ultrasound is the primary technique for evaluating kidneys in children: conventional and high-resolution US allows a detailed visualization of renal parenchyma and of number, size and location of the cysts, hence representing the most important diagnostic imaging technique for the first diagnosis and follow-up of these young patients. The purpose of this pictorial essay is to review the spectrum of renal cystic lesions in children from simple, complex or malignant single cysts to the several poly/multicystic kidney diseases.

Objective Lymphatic malformations (LMs) are rare and benign diseases. This article aimed to present a series of surgically treated giant cervicofacial LMs in children. Methods A retrospective analysis of giant cervicofacial LMs (tumor diameter greater than 10 cm) in children over the past 8 years was performed in our department. Results The 10 patients included 4 males and 6 females. Macrocystic lesion presented in 4 patients, mixed cystic lesion presented in 4 patients, and microcystic lesion presented in 2 patients. All the patients underwent surgical resection combined with bleomycin sclerotherapy. Despite the wide range of lesions, most patients (7/10, 70%) received only a single treatment, 3 patients had a recurrent lesion. Seven patients experienced complete or almost complete resolution of the disease with an average follow-up time of 26.4 months. Conclusions Surgical resection is the preferred method for the treatment of giant LMs, combination of bleomycin sclerotherapy can reduce the recurrence rate. The curative effect of macrocystic disease is relatively enhanced compared with microcystic and mixed cystic diseases.

Renal cystic lesions are considered the most common abnormality associated with the kidneys. Most renal cysts are usually uncomplicated simple cysts that are not life-threatening; however, fatal renal cystic diseases can develop from these space-occupying lesions. Although renal cystic diseases are similar in presentation, they possess distinct features, variable prognoses, and complications later in life. Early identification and effective management of these respected diseases has led to longer survival rates and better quality of life. The purpose of this review is to provide a comprehensive analysis of the most prevalent cystic diseases of the pediatric population in hopes to aid in early distinction and appropriate treatment.

Neonatal renal cystic diseases have a great impact on the morbidity and mortality of the affected neonates and infants. A good insight into the pathophysiology, diagnosis and treatment options of various neonatal renal cystic diseases aid in early diagnosis and intervention, thereby preventing complications.PubMed search was done for articles on "neonatal renal cystic diseases" and relevant publications including reviews were considered for our article.Both hereditary and nonhereditary causes of cystic kidney diseases can result in severe morbidity and mortality. The main diagnostic modality is ultrasound imaging and most of the neonatal renal cystic diseases are detected during prenatal ultrasound screening. Commonly encountered neonatal renal cystic diseases are autosomal dominant polycystic kidney disease, autosomal recessive polycystic kidney disease and multicystic dysplastic kidney.A thorough knowledge of various renal cystic diseases can be of extreme prognostic value. Physicians should be aware of the impact of early diagnosis and intervention on the lives of those affected. Further research about treatment of these diseases is ongoing and can result in breakthrough therapies for these patients.

Cystic diseases of the kidney range from indolent, incidentally diagnosed simple renal cysts to severe life-threatening renal insufficiency seen in neonates and infants with autosomal recessive polycystic kidney disease (ARPKD). Multicystic dysplastic kidney (MCDK) and autosomal dominant cystic disease (ADPKD) are examples of the most common cystic kidney diseases seen in clinical practice. This chapter provides a practical overview of the investigation and management for the most common cystic kidney diseases taking into account possible limitations encountered in low-resource settings.

Glycolytic activity is increased in proliferating cells, leading to the concept that glycolysis could be a therapeutic target in cystic diseases and kidney cancer. Preclinical studies using the glucose analog 2-deoxy-d-glucose have shown promise; however, inhibiting glycolysis in humans is unlikely to be without risks. While proximal tubules are predominantly aerobic, later segments are more glycolytic. Understanding exactly where and why glycolysis is important in the physiology of the distal nephron is thus crucial in predicting potential adverse effects of glycolysis inhibitors. Live imaging techniques could play an important role in the process of characterizing cellular metabolism in the functioning kidney. The goal of this review is to briefly summarize recent findings on targeting glycolysis in proliferative kidney diseases and to highlight the necessity for future research focusing on glycolysis in the healthy kidney.

Objectives: The aim of this study was to determine the distribution of morphologic patterns of neoplastic and nonneoplastic cystic ovarian lesions, and their distribution in different age groups in a tertiary-care center in Southern India. Materials and Methods: This was a prospective study of all cystic ovarian lesions diagnosed during a period of 3 years. Results: There was a total 303 cases of cystic ovarian lesions, of which 232 cases were neoplastic. Of the neoplastic lesions, benign tumors comprised 214 cases, borderline tumors 5 cases, and malignant tumors 13 cases. Of 232 cases having neoplastic lesions, 26 were associated with various nonneoplastic lesions as well. Surface epithelial tumors emerged as the most common, accounting for 190 cases (81.90%); followed by germ-cell tumors, with 40 cases (17.24%); and sex-cord stromal tumors being least common, with 2 cases (0.86%). Among the malignant tumors, immature teratoma was the commonest type, with 4 cases (30.77%); followed by papillary ...

A number of inherited renal diseases present with renal cysts and often lead to end-stage renal disease. With recent advances in genetics, increasing number of genes and mutations have been associated with cystic renal diseases. Although genetic testing can provide a definite diagnosis, it is often reserved for equivocal cases or for ongoing investigational research. Therefore, imaging findings are essential in the routine diagnosis, follow-up, and detection of complications in patients with inherited cystic renal diseases. In this article, the most recent classification, genetic analysis, clinical presentations, and imaging findings of inherited cystic renal diseases will be discussed.

Inherited renal cystic diseases comprise a spectrum of disorders that are typically characterized by dysfunction of the so-called cilia. These hair-like cellular appendages are involved in numerous cellular signalling processes. During development, cilia are important for the development of the left–right axis and for the establishment of cell polarity. Consequently, depending on the specific nature of the underlying ciliary defect, renal cystic diseases can be associated with a large spectrum of other organ manifestations, including congenital heart disease, retinopathy, polydactyly, and hepatic abnormalities, such as ductal plate malformation. Based on clustering of such symptoms, specific clinical syndromes, such as nephronophthisis, Bardet–Biedl syndrome, Joubert syndrome, and the various forms of polycystic kidney diseases, have been assigned. Subsequent genetic investigations have shown that these clinical distinctions are not necessarily consistent with the underlying genetic alterations, so that, for example, genes initially associated with Joubert syndrome can phenocopy autosomal recessive polycystic kidney disease. Thus, identification of the genetic cause can inform the clinical management to assess for potentially involved organ manifestations.

Renal cystic diseases are conditions in which parts of or entire nephrons become enlarged and create fluid-filled cysts. These cysts occur in many genetic diseases. Most of the products of causative genes, termed cysto-proteins, are localized in cilia and/or centrioles. In addition, mutant mice lacking cilia develop renal cysts. Therefore, cilia are thought to have an important role in renal cystogenesis. The cilium is a tiny projection from the cell surface; however, it can be divided into several subcompartments. These subcompartments have specific roles. This review attempts to classify cysto-proteins based on their localization in ciliary subcompartments with the aim of defining relationships among them and of identifying their exact intraciliary functions.

Parapelvic renal cysts are more likely to be symptomatic compared with that of simple peripheral renal cysts, and their treatment remains challenging. This study aimed to assess the feasibility and safety of flexible ureteroscopy in managing symptomatic endogenous renal cystic diseases.Thirty-five patients with symptomatic endogenous renal cystic diseases were treated by ureteroscopic unroofing and drainage into the collecting system. Surgical procedure, patient's outcome, and postoperative complications were retrospectively evaluated.Ureteroscopic unroofing and drainage were successful in 35 patients without conversion to open surgery. For patients with successful surgery, the mean operation and hospitalization times were 25.38 ± 3.71 min and 3.01 ± 0.57 d, respectively, with a curative standard rate of 74.3% (26/35). Three patients experienced Clavien grade I and II complications but no serious perioperative complications occurred. During the follow-up period averaging 36 mo, no cysts were detected in 74.3% of patients (26/35); noticeable relief was observed in four patients showing a simple renal cyst (diameter2 cm). In five patients who presented with polycystic kidneys, the renal volume was decreased by 93.76 ± 7.38 mL per side, on average, compared with pretreatment values. Pain was relieved in all 30 patients with renal cysts. Hydronephrosis disappeared in all 15 patients diagnosed with this condition at presentation. No secondary cyst lesions (such as infection, hematoma formation, and solid cystic changes) occurred.The transurethral flexible ureteroscopic incision and drainage for symptomatic endogenous renal cystic diseases has multiple advantages such as minimal trauma, rapid recovery, and a definite curative effect.

Resumen En esta unidad de actualizacion se estudian las alteraciones renales congenitas y hereditarias. Considerando las alteraciones congenitas aquellas que se manifiestan o existen desde el nacimiento, pero que han sido adquiridas por el feto durante su periodo intrautero, a efectos de diferenciarlas de aquellas que son hereditarias, que se transmiten a traves de anomalias cromosomicas y con repercusion sobre la familia. Dentro de las anomalias congenitas, los autores presentan las malformaciones mas frecuentes tanto del parenquima renal como de la via urinaria. Entre las ultimas, incluiremos las enfermedades quisticas y aquellas con dano predominantemente glomerular o tubulointersticial.

Etiologic and pathogenetic data are useful if not necessary if we are to understand the segmentary nature of some malformative conditions, including cystic diseases of the kidney. As mentioned earlier, the development of the kidney and urinary tracts occurs over a series of stages that take place within a limited space and time. If this development is abnormal it can cause malformative nephrouropathies. The most serious of the malformative nephrouropathies is renal dysplasia: primitive tubules, embraced by stroma and a muscular collar surrounded by areas of nephrogenic mesenchyme together with undeveloped or less developed renal tissue and with non-renal structures like cartilage, muscles, and other structures, termed mesenchymal metaplasia [1, 2].

The safety and efficacy of multi-planar reconstruction (MPR) image post-processing technique-computed tomography (CT) urography (CTU) combined with precise intraoperative ultrasonography guided flexible ureteroscope in renal cyst incision and drainage in the treatment of cystic diseases of kidney were evaluated. A total of 68 patients were randomly divided into control and observation group (n=34). All the patients were treated with renal cyst incision and drainage under flexible ureteroscope. The control group was under ultrasound guidance. The observation group was combined with MPR-CTU, the safety and efficacy was compared. There was no significant difference between the two groups in the success rate and the time of cyst treatment (P>0.05). The incidence of intraoperative and postoperative complications of the observation group was significantly lower than that of the control group. After 1 month follow-up, the total effective rate of the observation group was significantly higher than that of the control group, the difference was statistically significant (P

Unilateral renal cystic disease (URCD) is a rare, non-familial, non-progressive, unilateral cystic disorder of the kidney that is not associated with cysts or disorders in other organs and it is not related to other genetic cystic diseases. Only a few cases have been reported in the literature. We report a case of URCD in an adult male 49 years of age. Absence of a family history, benign clinical course and limitation of the disease to one kidney distinguish this condition from autosomal dominant polycystic kidney disease (ADPKD).

We provide an overview of assessment of the kidneys at autopsy, with special considerations for pediatric versus adult kidneys. We describe the approach to gross examination, tissue allocation when needed for additional studies of potential medical renal disease, the spectrum of congenital abnormalities of the kidneys and urinary tract, and approach to cystic diseases of the kidney. We also discuss common lesions seen at autopsy, including acute tubular injury, ischemic versus toxic contributions to this injury, interstitial nephritis, and common vascular diseases. Infections commonly involve the kidney at autopsy, and the key features and differential diagnoses are also discussed.

Head and neck teratoid cysts are the least common congenital cysts in the head and neck region, accounting for approximately 1.8% of all dermoid cysts. Teratoid cysts exhibiting mesodermal elements may be lined by gastric, intestinal, respiratory, squamous, or cilitated epithelium. We present a case of huge submandibular and neck teratoid cyst in newborn with airway obstruction and feeding difficulty. Surgical extirpation is the treatment of choice. However, before operation, some other cystic diseases in the head and neck region needs to be excluded. Fine-needle aspiration biopsy was necessary in differential diagnosis of lesions before treatment, especially that the principle of treatment of those diseases is different.

Renal cysts constitute the most commonly found benign lesions in adults during the diagnostic imaging: a renal cyst is detected in 10 % up to 50 % at abdominal ultrasound [1]. Also the prevalence of renal cysts is more than 50 % in adults 50 years old or older detected in autopsy studies [2].

OBJECTIVE. The purpose of this clinical perspective is to describe a decision-tree approach to the finding of hyperechoic kidneys as signs of congenital renal cystic disease in fetuses and children. This approach takes into account the latest classification of inherited renal cystic diseases. The basis of the approach is a detailed sonographic analysis in addition to assessment of clinical data and the familial history. CONCLUSION. With the decision-tree approach, typical sonographic patterns can be described and used for accurate diagnosis of isolated renal cystic diseases and polymalformative syndromes. In some cases, however, the diagnosis is not achieved, and complementary examinations are needed.

Context.—Cystic lesions of the kidney may be accompanied by a range of neoplasms with distinct prognoses and future risks of developing additional tumors. In addition, some renal tumors, with or without accompanying renal cysts, may show a prominent cystic component. In the adult population, neoplasms occurring in a background of renal cystic diseases and cystic renal neoplasms often pose diagnostic challenges because of their many overlapping features.Objective.—To review the clinicopathologic characteristics of common entities in the spectrum of neoplastic and potential preneoplastic cystic lesions encountered in adults, with an emphasis on renal cystic diseases associated with tumor development and on renal neoplasms with predominantly cystic morphology.Data Sources.—The relevant English-language literature was reviewed, accompanied by the authors' experience at their practicing institution.Conclusions.—The presence of multiple renal cysts, both acquired and syndromic, can be associated with a variety of renal tumors. The morphology of the cysts and associated tumor types can help predict the genetic or acquired basis of the lesions, and particularly in specimens with no accompanying pertinent clinical history, such potential associations should be suggested in surgical pathology reports.

Cystic diseases in the female pelvis are common. Cysts of the female genital tract comprise a large number of physiologic and pathologic cysts. The majority of cystic pelvic masses originate in the ovary, and they can range from simple, functional cysts to malignant ovarian tumors. Non-ovarian cysts of female genital system are appeared at least as often as ovarian cysts. In this review, we aimed to discuss the most common cystic lesions the female genital system.

Cystic diseases of the kidney constitute a heterogeneous family. Most renal cysts are benign and asymptomatic, and don't have any impact on renal function. These "simple" cysts are usually managed conservatively. However, some renal cysts may be symptomatic or may have atypical radiological presentation, leading to suspicion of malignant tumour; puncture or surgical investigation may be indicated in such cases. The other cystic renal diseases include specific entities, either genetic or acquired during development. Some malformative syndromes are often associated, and renal function may be impaired. In such cases, nephrectomy is required.

This manuscript provides a concise surgical review of cystic diseases of the liver. Attention is paid to diagnosis, pre-surgical evaluation, surgical techniques, including laparoscopy, and post-surgical care of the patient with cystic disease of the liver.

The high frequency of benign and asymptomatic liver cysts must not underevaluate the potential diagnostic difficulties of liver cystic diseases. Complicated liver cyst or cystic mass of various origin, such as developmental, neoplastic or inflammatory, are important to be recognized by the radiologist. The diagnostic approach is depending on the number of lesions and their dissemination. The presence of a wall, the internal structure of the lesion, particularly the MR signal and the proximity of the biliary tree are the main diagnostic criteria. Some less frequent but characteristic unusual features need to be memorized.

BACKGROUND: Multiple cerebral hydatid cysts are very rare with only a few reports in the literature detailing diagnostic workup, medical management, surgical techniques, possible complications, and outcomes. CASE DESCRIPTION: We present the case of an 8-year-old boy who presented with progressively worsening headaches, vomiting, and intermittent fever since 20 days. Diagnostic workup was performed, and magnetic resonance imaging revealed multiple intracranial cysts predominantly in the right frontal region with significant mass effect. A total of 19 intracranial cysts were removed surgically, and the child recovered uneventfully. CONCLUSIONS: Neurosurgeons should keep hydatidosis in the list of differentials when evaluating patients with cystic diseases of the brain. Although the removal of such cysts is challenging, outcomes are excellent when cysts are evacuated without rupture and patients show complete resolution of symptoms.

This article cannot comprehensively cover the enormous strides made in defining the molecular and cellular basis of renal cystic diseases over the last decade. Therefore, it provides a brief overview and categorization of inherited, developmental, and acquired renal cystic diseases, providing a relevant, up-to-date bibliography as well as a useful list of informative Internet Web sites. Its major focus is the translational biology of polycystic kidney disease. It demonstrates how emerging molecular and cellular knowledge of the pathophysiology of particular diseases such as autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ADPKD) can translate into innovative therapeutic insights.

Inherited renal cystic diseases constitute an important set of single-gene disorders that frequently progress to end stage renal disease (ESRD). Transmitted as autosomal dominant, autosomal recessive, or X-linked traits, renal cystic diseases are phenotypically diverse with respect to age at onset, rate of disease progression, and associated extra-renal manifestations. These disorders involve defects in a set of gene products commonly referred to as cystoproteins that, while functionally distinct, appear to co-localize, at least in part, with the cilia/centrosome complex. Therefore, investigations are increasingly focused on the role of this complex in the pathogenesis of renal cystic disease. Sorting out the functional relationship between these cystoproteins and the cilia/centrosome complex will undoubtedly provide a better understanding of renal cystic disease pathogenesis and, potentially, identify new targets for therapeutic intervention.

It is rare for inflammation to involve only the sphenoid sinus.Surgeons must be careful to avoid nerves, blood vessels and base of the skull around the sphenoid sinus circumference. The surgical method can be either the transendothelial sphenoid sinus method or the transendothelial ethmoid sinus method, depending on the needs of the individual case. We examined four isolated sphenoiditis cases treated by one of the authors during the past three years. Case of postoperative sphenoid sinus cystic diseases were excluded.All 4 cases were women. Three cases involved bacterial inflammation, and one case demonstratedfungal disease. The chief complaint in 2 cases was headache, diplopia in one case and facial numbness in one case. General anaesthesia was administered in 2 cases and local anesthesia in 2 cases. Surgical methods were transendothelial ethmoid sinus in 2 cases and transendothelial sphenoid sinus in 2 cases. All cases healed successfully.

This review aims to assist in the categorization of inherited, developmental, and acquired cystic disease of the kidney as well as to provide a pertinent, up-to-date bibliography. The conditions included are autosomal-dominant polycystic kidney disease, autosomal-recessive polycystic kidney disease, unilateral renal cystic disease (localized cystic disease), renal simple cysts, multicystic dysplastic kidney, pluricystic kidney of the multiple malformation syndromes, juvenile nephronophthisis and medullary cystic disease, medullary sponge kidney, primary glomerulocystic kidney disease, and glomerulocystic kidney associated with several systemic disorders mainly of genetic or chromosomal etiology, cystic kidney in tuberous sclerosis, and in von Hippel-Lindau syndrome, cystic nephroma, cystic variant of congenital mesoblastic nephroma, mixed epithelial stromal tumor of the kidney, renal lymphangioma, pyelocalyceal cyst, peripylic cyst and perinephric pseudocyst, acquired renal cystic disease of long-term dialysis, and cystic renal cell carcinoma and sarcoma. Whereas the gross and histologic appearance of some of these conditions may be diagnostic, clinical and sometimes molecular studies may be necessary to define other types.

Cystic diseases of the kidney in infants and children include a large number of clinicopathological disorders that may be inherited or sporadic in origin. Lesions can be detected on antenatal ultrasound; they may be unilateral or bilateral, symptomatic at birth or detected later in life, with a variety of manifestations [1]. Cystic diseases can present with enlarged, normal or shrunken kidneys, with or without other extrarenal manifestations, which presents a considerable challenge to the radiologist. Ultrasound is the primary imaging modality in the diagnosis of most cystic renal diseases, and recourse to other modalities is rarely required. Although Wilms’ tumour (nephroblastoma) and mesoblastic nephroma can have a very cystic appearance, they additionally have solid components, which affords easy identification from the cystic conditions described here. With the exception of multilocular cystic nephroma, neoplastic lesions will not be discussed in this review. There are various approaches to classify cystic renal diseases in children. A popular approach to classification is based upon genetic or non-genetic causes (Table 1). Various eponymous syndromic conditions are also associated with cysts in the kidneys, some of which are shown in Table 2.

Cystic diseases of the biliary tract encompass a complex group of congenital disorders. Some of the disorders, such as Caroli disease and the hepatobiliary cysts of autosomal-dominant polycystic kidney disease, share common embryologic origins, whereas others, such as choledochal cysts, biliary diverticula, and choledochoceles, have unclear origins. This article reviews the embryologic, clinical, pathologic, and imaging features of biliary cystic disease.

A cystic lesion in the lung is defined as a parenchymal region that has a well-defined wall and is filled with contents of variable density. This definition includes a variety of diseases, such as bronchogenic cyst, abscess formation, lymphangiomyomatosis, Langerhans cell histiocytosis, emph

Cardiac hydatid cyst is an uncommon disease, mostly involving the myocardium. Involvement of the pericardium or isolated pericardial hydatid cysts are extremely rare and therefore can mimic other pericardial cystic diseases. Herein we present the radiological and surgical findings of an asymptomatic isolated pericardial hydatid cyst, Kardiyak hidatik kist sıklıkla miyokardı tutan sık görülmeyen bir hastalıktır. Perikardiyumun tutulması veya izole perikardiyal hidatik kist son derece nadirdir ve bu yüzden diğer perikardiyal kistik hastalıkları taklit edebilir. Bu yazıda asemptomatik izole perikardiyal hidatik kistin radyolojik ve cerrahi bulgularını sunuyoruz

Renal cysts are commonly encountered in clinical practice. Although most cysts found on routine imaging studies are benign, there must be an index of suspicion to exclude a neoplastic process or the presence of a multicystic disorder. This article focuses on the more common adult cystic diseases, including simple and complex renal cysts, autosomal-dominant polycystic kidney disease, and acquired cystic kidney disease.

Kidney disease is a significant medical and public health problem. The National Institute of Diabetes and Digestive and Kidney Diseases recently asked the community to identify research objectives, which, if addressed, could improve understanding of basic kidney function and aid in prevention, treatment, and reversal of kidney disease. The Kidney Research National Dialogue invited interested parties to submit, discuss, and prioritize ideas using an interactive website; 1600 participants posted more than 300 ideas covering all areas of kidney disease, including the cystic kidney diseases. Although much is known about the genetics and pathogenesis of cystic diseases, there remain challenges to our understanding of the fundamental mechanisms of cyst formation, what genes act as modifiers to cause variable responses in different people, and how to detect and monitor disease progression. This article summarizes key research questions for cystic kidney diseases.