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.
Humans ; Liver Diseases/pathology* ; Cysts/pathology* ; Caroli Disease/diagnosis* ; Liver Neoplasms/therapy* ; Hamartoma

OBJECTIVES: Pulmonary Langerhans cell histiocytosis (PLCH) is a clonal disease, characterized by proliferation of Langerhans cells that derived from bone marrow infiltrating the lungs and other organs. Due to the rarity of the disease, the current understanding of the disease is insufficient, often misdiagnosed or missed diagnosis. This study aims to raise clinicians' awareness for this disease via summarizing the clinical characteristics, imaging features, and treatment of PLCH. METHODS: We retrospectively analyzed clinical and follow-up data of 15 hospitalized cases of PLCH from September 2012 to June 2021 in the Second Xiangya Hospital of Central South University. RESULTS: The age of 15 patients (9 men and 6 women, with a sex ratio of 3 to 2) was 21-52 (median 33) years. Among them, 8 had a history of smoking and 5 suffered spontaneous pneumothorax during disease course. There were 3 patients with single system PLCH and 12 patients with multi-system PLCH, including 7 patients with pituitary involvement, 7 patients with lymph node involvement, 6 patients with bone involvement, 5 patients with liver involvement, 2 patients with skin involvement, 2 patients with thyroid involvement, and 1 patients with thymus involvement. The clinical manifestations were varied but non-specific. Respiratory symptoms mainly included dry cough, sputum expectoration, chest pain, etc. Constitutional symptoms included fever and weight loss. Patients with multi-system involvement experienced symptoms such as polyuria-polydipsia, bone pain, and skin rash. All patients were confirmed by pathology, including 6 by lung biopsy, 3 by bone biopsy, 2 by lymph node biopsy, and 4 by liver, skin, suprasternal fossa tumor, or pituitary stalk biopsy. The most common CT findings from this cohort of patients were nodules and/or cysts and nodular and cystic shadows were found in 7 patients. Three patients presented simple multiple cystic shadows, 3 patients presented multiple nodules, and 2 patients presented with single nodules and mass shadows. Pulmonary function tests were performed in 4 patients, ventilation dysfunction was showed in 2 patients at the first visit. Pulmonary diffusion function tests were performed in 4 patients and showed a decrease in 3 patients. Smoking cessation was recommended to PLCH patients with smoking history. Ten patients received chemotherapy while 2 patients received oral glucocorticoid therapy. Among the 11 patients with the long-term follow-up, 9 were in stable condition. CONCLUSIONS PLCH is a neoplastic disease closely related to smoking. The clinical manifestations and laboratory examination are not specific. Pneumothorax could be the first symptom which is very suggestive of the disease. Definitive diagnosis relies on histology. There is no unified treatment plan for PLCH, and individualized treatment should be carried out according to organ involvement. Early smoking cessation is essential. Chemotherapy is the main treatment for rapidly progressing PLCH involved multiple organs. All diagnosed patients can be considered for the detection of BRAF^V600E gene and relevant targeted therapies have been implemented recently.
Adult ; Cysts ; Female ; Histiocytosis, Langerhans-Cell/therapy* ; Humans ; Lung/pathology* ; Male ; Retrospective Studies ; Smoking/adverse effects* ; Smoking Cessation

PURPOSE: Although angiogenesis has been implicated in the promotion of renal cyst growth in autosomal dominant polycystic kidney disease, no studies have investigated the role of angiogenesis in the growth of simple renal cysts. The aim of current study was to investigate the effect of chemotherapy with the antivascular endothelial growth factor antibody bevacizumab on renal cyst development and growth in cancer patients. MATERIALS AND METHODS: We retrospectively reviewed the medical records of 136 patients with a variety of cancers that were treated with bevacizumab-based chemotherapy for metastatic disease. The presence of and changes in renal cysts were evaluated by retrospective analysis of computed tomography scans performed for assessment of tumor response to bevacizumab-based therapy. RESULTS: The median age of the patients was 64 years. Renal cysts were identified in 66 patients, in whom 33 (50%) had a single cyst and the rest had 2 or more cysts. The average dose of bevacizumab was 2.68 mg/kg per week. Median duration of treatment was 33 weeks. Average cyst size was 1.9±2.4 cm at the beginning of the study and the majority of the cysts (54 patients, 84%) did not change in size or shape during bevacizumab treatment. No patients were identified with new cysts. Cyst size changed in 10 patients (16%): an increase of 15% to 40% from the baseline size in 5 patients and a decrease in size of 10% to 70% in another 5 patients. The duration of bevacizumab therapy was significantly longer in the subgroup of patients with diminished or increased cyst size than in the patients with stable cyst size: 62 weeks versus 29 weeks, respectively (p=0.0002). CONCLUSIONS: Our data demonstrated that simple renal cysts were stable in size and number in the vast majority of cancer patients treated with bevacizumab.
Adult ; Aged ; Aged, 80 and over ; Angiogenesis Inhibitors/administration & dosage/*therapeutic use ; Bevacizumab/administration & dosage/*therapeutic use ; Cysts/complications/*drug therapy/pathology ; Disease Progression ; Dose-Response Relationship, Drug ; Drug Evaluation/methods ; Female ; Humans ; Kidney Diseases/complications/*drug therapy/pathology ; Male ; Middle Aged ; Neoplasms/complications/drug therapy ; Retrospective Studies ; Vascular Endothelial Growth Factor A/*antagonists & inhibitors

Adolescent ; Adult ; Arteries ; Bone Cysts, Aneurysmal ; therapy ; Child ; Embolization, Therapeutic ; Female ; Humans ; Male ; Sacrum ; pathology ; Spinal Diseases ; therapy ; Treatment Outcome ; Young Adult

BACKGROUNDAneurysmal bone cyst (ABC) is a benign lesion with the potential to be locally aggressive. The optimal treatment of cervical spine lesions remains controversial. This retrospective study was designed to evaluate the different treatments for primary ABCs of the cervical spine.

METHODSThis series included eight men and six women diagnosed between 2002 and 2012. A retrospective review of the hospital charts, operating room reports, office charts, and radiographs was performed. The data collected included patient age, sex, radiological features, pathology, treatment methods, outcomes, and complications from biopsies and treatments. The mean age at diagnosis was 17.5 years old with a range of 6-35 years. All patients had experienced local pain for a mean of 7.3 months (range, 0.5-18.0 months), and three patients had neurological deficits (one with radiculopathy and two with myelopathy). The Frankel classification before treatment was E in 12 cases, D in one case, and C in one case.

RESULTSFour patients received radiotherapy alone. Ten patients underwent surgery, including five total spondylectomies, two local resections, and three curettages. Three patients received preoperative selective arterial embolization, and four received adjuvant radiotherapy. The mean follow-up time was 44.5 months (range 12-96 months), and no recurrence was identified. Three patients with neurological deficits achieved complete recovery, as noted at the final follow-up examination. One patient received radiotherapy without appropriate immobilization as prescribed and developed C1-C2 subluxation with severe spinal cord compression but without myelopathy. He refused further treatment.

CONCLUSIONSThe results can be achieved by different treatments (surgical resection/curettage, selective arterial embolization, and radiotherapy) for ABC of the cervical spine. Reconstruction of stability is also important for the treatment of cervical ABC.

Adolescent ; Adult ; Bone Cysts, Aneurysmal ; therapy ; Cervical Vertebrae ; pathology ; Child ; Embolization, Therapeutic ; Female ; Humans ; Male ; Retrospective Studies ; Spinal Diseases ; therapy ; Young Adult

Gefitinib is regarded as a relatively safe agent for the treatment of an advanced non-small cell lung cancer (NSCLC). Pulmonary toxicity such as interstitial lung disease associated with gefitinib is uncommon with an estimated all time incidence around 1% worldwide. Moreover, a case of gefitinib associated with pulmonary cystic changes has not been reported yet. In this report we present a case of progressive multiple air cystic changes in both lungs in a patient with NSCLC and intrapulmonary metastases who underwent a gefitinib therapy.
Antineoplastic Agents/*adverse effects ; Brain Neoplasms/secondary ; Carcinoma, Non-Small-Cell Lung/*drug therapy/secondary ; Cysts/*chemically induced ; Female ; Humans ; Lung/pathology ; Lung Diseases/*chemically induced ; Lung Diseases, Interstitial ; Lung Neoplasms/*drug therapy ; Middle Aged ; Quinazolines/*adverse effects

OBJECTIVETo report a rare case of ectopic mesonephric duct cyst with ectopic testicular malignancy and improve the diagnosis and treatment of the disease.

METHODSWe retrospectively analyzed the clinical data of a case of ectopic mesonephric duct cyst with ectopic testicular malignancy, reviewed relevant literature at home and abroad, and investigated the pathogenesis, diagnosis and treatment of the disease.

RESULTSA large cyst and the right ectopic malignant testis were removed via abdominal incision, and the left undescended testis was lowered into the scrotum. Pathological examination confirmed the lesion to be right ectopic mesonephric duct cyst with right ectopic testicular seminoma. No metastasis was found during a year of follow-up.

CONCLUSIONEctopic mesonephric duct cyst with ectopic testicular malignancy was a rare disease. Imaging examination contributes to its diagnosis, but it has to be confirmed by pathology. Surgical removal should be performed as early as possible and follow-up treatment depends on the pathologic type and stage of ectopic testicular malignancy.

Cryptorchidism ; therapy ; Cysts ; pathology ; Humans ; Male ; Neoplasms, Germ Cell and Embryonal ; surgery ; Seminoma ; surgery ; Testicular Neoplasms ; surgery ; Testis ; surgery ; Wolffian Ducts ; pathology

INTRODUCTIONEndoscopic transenteric stenting is the standard treatment for pseudocysts, but it may be inadequate for treating infected collections with solid debris. Surgical necrosectomy results in significant morbidity. Direct endoscopic necrosectomy (DEN), a minimally invasive treatment, may be a viable option. This study examined the efficacy and safety of DEN for the treatment of infected walled-off pancreatic necrosis and infected pseudocysts with solid debris.

METHODSThis study was a retrospective analysis of data collected from a prospective database of patients who underwent DEN in the presence of infected walled-off pancreatic necrosis or infected pseudocysts with solid debris from April 2007 to October 2011. DEN was performed as a staged procedure. Endoscopic ultrasonography-guided transgastric stenting was performed during the first session for initial drainage and to establish endoscopic access to the infected collection. In the second session, the drainage tract was dilated endoscopically to allow transgastric passage of an endoscope for endoscopic necrosectomy. Outcome data included technical success, clinical success and complication rates.

RESULTSEight patients with infected walled-off pancreatic necrosis or infected pseudocysts with solid debris (mean size 12.5 cm; range 7.8-17.2 cm) underwent DEN. Underlying aetiologies included severe acute pancreatitis (n = 6) and post-pancreatic surgery (n = 2). DEN was technically successful in all patients. Clinical resolution was achieved in seven patients. One patient with recurrent collection opted for surgery instead of repeat endotherapy. No procedural complications were encountered.

CONCLUSIONDEN is a safe and effective minimally invasive treatment for infected walled-off pancreatic necrosis and infected pseudocysts.

Adult ; Aged ; Cysts ; diagnosis ; diagnostic imaging ; Endoscopy ; methods ; Female ; Humans ; Male ; Middle Aged ; Minimally Invasive Surgical Procedures ; methods ; Necrosis ; Pancreas ; pathology ; Pancreatic Diseases ; diagnosis ; diagnostic imaging ; surgery ; Pancreatitis, Acute Necrotizing ; diagnostic imaging ; surgery ; therapy ; Singapore ; Stents ; Treatment Outcome ; Ultrasonography

Polycystic liver is the most common extra-renal manifestation associated with autosomal dominant polycystic kidney disease (ADPKD), comprising up to 80% of all features. Patients with polycystic liver often suffer from abdominal discomfort, dyspepsia, or dyspnea; however, there have been few ways to relieve their symptoms effectively and safely. Therefore, we tried transcatheter arterial embolization (TAE), which has been used in treating hepatocellular carcinoma. We enrolled four patients with ADPKD in Seoul National University Hospital, suffering from enlarged polycystic liver. We embolized the hepatic arteries supplying the dominant hepatic segments replaced by cysts using polyvinyl alcohol particles and micro-coils. The patients were evaluated 12 months after embolization for the change in both liver and cyst volumes. Among four patients, one patient was lost in follow up and 3 patients were included in the analysis. Both liver (33%; 10%) and cyst volume (47.7%; 11.4%) substantially decreased in two patients. Common adverse events were fever, epigastric pain, nausea, and vomiting. We suggest that TAE is effective and safe in treating symptomatic polycystic liver in selected ADPKD patients.
Aged ; Catheterization ; Cysts/*therapy ; Embolization, Therapeutic/instrumentation/*methods ; Female ; Hepatic Artery ; Humans ; Liver/pathology/physiology ; Liver Diseases/pathology/*therapy ; Middle Aged ; Polycystic Kidney, Autosomal Dominant/diagnosis/*therapy ; Polyvinyl Alcohol/therapeutic use ; Tomography, X-Ray Computed

OBJECTIVETo evaluate curative effect of the fibrinogen gel for treating sacral canal arachnoid cyst.

METHODSNineteen patients with sacral canal arachnoid cysts included 7 males and 12 females; The average age was 48.4 years ranging from 19 to 68 years. The course was from 2 weeks to 7 months. Of all the patients, 9 were in level of S1, 4 were in level of S1 to S2, 5 were in level of S2, 1 was in level of S1 to S3. Cystis wall greater partial excision adopted in 11 cases, partial resection in 8, then all patients were treated by spray painting fibrinogen gel.

RESULTSNineteen patients were followed-up for 13 to 30 months (mean 21.3 months). The clinical symptom disappeared completely in 18 patients, and only one patient urinated incapably, but after 2 weeks returned to normal. No one found recurrence by MRI after 12 months.

CONCLUSIONThis method of fibrinogen gel for treating sacral canal arachnoid cyst has advantages of easy performing, safety, achieve good results, less neck symptoms and early commencing of mobilization.

Adult ; Aged ; Arachnoid Cysts ; pathology ; therapy ; Female ; Fibrinogen ; administration & dosage ; Gels ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Sacrum