Van Wyk-Grumbach syndrome (VWGS) is a rare complication of prolonged untreated juvenile hypothyroidism characterized by precocious puberty and enlarged multicystic ovaries. A 13-year-old girl visited our outpatient clinic due to menstrual irregularities. She had precocious puberty, pituitary hyperplasia and multiple cystic ovaries in addition to clinical signs of severe congenital hypothyroidism. After the initiation of L-thyroxine therapy, the symptoms were alleviated in a short time. This rare syndrome is easy to be misdiagnosed as pituitary and ovarian tumor. High degree of suspicion and timely diagnosis can prevent unnecessary surgical procedures because the symptoms can be reversed with thyroid hormone supplementation.
Adolescent ; Congenital Hypothyroidism ; complications ; diagnosis ; etiology ; Diagnosis, Differential ; Diagnostic Errors ; Female ; Humans ; Hyperpituitarism ; Hyperplasia ; Menstruation Disturbances ; etiology ; Ovarian Cysts ; diagnosis ; etiology ; Ovary ; pathology ; Pituitary Gland ; pathology ; Puberty, Precocious ; diagnosis ; etiology ; Syndrome ; Thyroxine ; therapeutic use

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

OBJECTIVETo study the pathology, imaging and clinical features of a child with trisomy 21 syndrome associated interstitial lung disease.

METHODData of a case with trisomy 21 syndrome associated interstitial lung disease confirmed by lung imaging and pathology were collected, analyzed and the related reports in literature were reviewed.

RESULTThe patient was a one year and 7 months old boy who suffered from severe pneumonia and recurrent infection during his hospital stay. When his disease was stable, he did not have shortness of breath and cyanosis, but a chest computed tomography (CT) showed ground-glass opacity, regional emphysema, band-like change in lung parenchyma, which indicated interstitial lung diseases. Unequal air inflation in bilateral lungs and diffuse over-distension of peripheral air spaces in lung surface were seen through thoracoscope. Pathological examination indicated that alveolar, alveolar ducts and alveolar sac were enlarged, alveolar septa was expanded. There were two reports in lung pathology of trisomy 21 syndrome, alveolar growth abnormalities was seen in 86%-88% cases. The multiple subpleural cysts in chest CT was characteristic. Clinically, trisomy 21 syndrome had high morbidity of respiratory tract infection and progress to respiratory failure frequently. Prolonged postoperative desaturation was constant which required long duration of respiratory support.

CONCLUSIONTrisomy 21 syndrome associated alveolar growth abnormalities were confirmed, which manifest as alveolar simplification in pathology and interstitial lung diseases in imaging. The risk of respiratory failure in these cases caused by infection and surgery should be considered.

Cysts ; pathology ; Down Syndrome ; complications ; Humans ; Infant ; Lung ; pathology ; Lung Diseases, Interstitial ; diagnosis ; etiology ; Male ; Postoperative Period ; Pulmonary Alveoli ; pathology ; Respiratory Insufficiency ; Respiratory Tract Infections ; Tomography, X-Ray Computed

Clear cell chondrosarcoma is a rare cartilaginous tumour of low-grade malignancy. Although it has a characteristic histological appearance, its radiological features and clinical presentation often mimic a benign lesion. Herein, we describe the case of a patient with a clear cell chondrosarcoma of the right proximal femur that had an atypical appearance of chronic avascular necrosis on initial plain radiographs, which made preoperative diagnosis a challenge. In addition, the tumour also had extensive areas of aneurysmal bone cyst-like changes, which is not only a rare histologic phenomenon in clear cell chondrosarcoma, but also a confounding factor in the interpretation of the radiologic findings.
Adult ; Bone Cysts, Aneurysmal ; complications ; diagnostic imaging ; Bone Neoplasms ; complications ; diagnostic imaging ; Chondrosarcoma ; complications ; diagnostic imaging ; Female ; Femur ; diagnostic imaging ; pathology ; Hip ; pathology ; Humans ; Magnetic Resonance Imaging ; Necrosis ; pathology ; Osteoarthritis ; diagnostic imaging ; Radiography ; Whole Body Imaging

OBJECTIVETo evaluate the high resolution CT (HRCT) features of cyst-like lung adenocarcinoma, explore the correlation between HRCT image features and histopathological characteristics, and observe the pathological basis of air-containing space.

METHODSHRCT and histopathologic findings of cyst-like lung adenocarcinoma in 86 patients were investigated retrospectively. The image features of both tumor and air-containing space were analyzed. All surgically resected specimens were reviewed. The pathological analysis included histologic subtype, differentiation degree, and the pathological basis of air-containing space formation. The correlation between HRCT image features and histopathologic grades was analyzed.

RESULTSOn HRCT, intratumoral necrosis was detected in 17 cases (19.8%), air-containing space with septa in 40 cases (46.5%), wall nodule in the air-containing space in 16 cases (18.6%), mixed thick and thin walls of air-containing space in 49 cases (57.0%). Air-containing space and its wall were observed in 63 cases on histological specimens, among which destruction of the alveolar wall by tumor cells might be the pathological basis of air-containing space in 42 (66.7%) cases. Differences of tumor attenuation (P = 0.030), intratumoral necrosis (P = 0.003) and proportion of thin-wall in air-containing space (P = 0.014) among different histopathologic grades were significant. The proportion of thin-wall in air-containing space was negatively correlated with histological grade (r = 0.267, P = 0.015). Differences of tumor contour (P = 0.002), tumor attenuation (P = 0.006), intratumoral necrosis (P < 0.001), septa in air-containing space (P = 0.016) and proportion of thin-wall in the air-containing space (P = 0.005) among different differentiation degrees were significant. The proportion of thin-wall in air-containing space was positively correlated with differentiation degree (r = 0.266, P = 0.013).

CONCLUSIONSOn HRCT, cyst-like lung adenocarcinoma may manifest as an air-containing space with septa and mixed thin and thick walls, whereas wall nodule of air-containing space and intratumoral necrosis are not common. There is a certain correlation of HRCT manifestation with pathological subtype grading and tumor differentiation. Analysis of HRCT image features is helpful in prediction of the histopathologic grading and histological differentiation degree of the tumors. Destruction of the alveolar structure by tumor cells may be the main pathological basis of air-containing space formation in cyst-liked lung adenocarcinoma.

Adenocarcinoma ; complications ; diagnostic imaging ; pathology ; Adult ; Aged ; Aged, 80 and over ; Cysts ; complications ; diagnostic imaging ; pathology ; Female ; Humans ; Lung Diseases ; complications ; diagnostic imaging ; pathology ; Lung Neoplasms ; complications ; diagnostic imaging ; pathology ; Male ; Middle Aged ; Retrospective Studies ; Tomography, X-Ray Computed ; methods

Cysts ; complications ; Exophthalmos ; complications ; Frontal Sinus ; pathology ; Humans ; Male ; Middle Aged ; Paranasal Sinus Diseases ; complications

OBJECTIVE: To explore a rare anatomic malformation of ethmoid and its clinical features, as well as an effective way of treatment. METHOD: Four cases from 2000-2009 of the first affiliated hospital of china medical university were studied according to the symptom,CT scanning, pathological examination and treatment process, respectively. RESULT: Ethmoid bone gasification complicated with infection resulted in a rare anatomic and pathological disorder. The symptom and treatment were largely dependent on the size of gasification and degree of infection. CONCLUSION CT Scanning plays a critical role in the diagnosis. In most case,a surgical treatment under endoscope is generally applied as a suitable way for clinical therapy. The regular reexamination is recommended for preventing recurrence.
Adolescent ; Bone Cysts ; complications ; diagnosis ; surgery ; Child ; Child, Preschool ; Ethmoid Bone ; abnormalities ; pathology ; Female ; Humans ; Male ; Tomography, X-Ray Computed

OBJECTIVETo evaluate therapeutical effect of combined hepatic resection and fenestration on patients with severe adult polycystic liver disease (APLD).

METHODSPreoperative clinical symptoms, postoperative complications and prognoses from 33 patients with severe adult polycystic liver disease (APLD) treated with combined hepatic resection and fenestration were recorded. According to the number and location of cysts before surgery and the remnant liver parenchyma after operation, all patients were classified into two types: class A and B. And patients in each type were further classified into three grades: Grade I, II and III. The frequency of postoperative complications of two types patients was compared.

RESULTSThe mean follow-up time was 57 months. There were three patients with recurrence of symptoms at 81, 68 and 43 mouths after operation. Two patients died of renal failure due to polycystic kidney disease at 137 and 85 mouths after operation. And one patient with postoperative hepatic inadequacy received an orthotopic liver transplantation. The total number of patients with postoperative complications was 26 cases, including one patient with bleeding, two patients with bile leakage, fourteen patients with mild ascites, twelve patients with severe ascites and eighteen patients with pleural effusion, and the overall incidence was 78.8%. There were 22 patients with imaging data, including 6 patients within A type and sixteen patients within B type. The frequencies of postoperative complications were 4 and 31, respectively, and the difference was statistically significant (Chi-square test = 4.99, P less than 0.05).

CONCLUSIONCombined hepatic resection and fenestration is a safe and acceptable procedure for the treatment of severe APLD.

Adult ; Aged ; Ascites ; epidemiology ; etiology ; Cysts ; diagnostic imaging ; pathology ; surgery ; Female ; Follow-Up Studies ; Hepatectomy ; methods ; Humans ; Liver ; diagnostic imaging ; pathology ; surgery ; Liver Diseases ; diagnostic imaging ; pathology ; surgery ; Male ; Middle Aged ; Pleural Effusion ; epidemiology ; etiology ; Postoperative Complications ; epidemiology ; prevention & control ; Prognosis ; Recurrence ; Retrospective Studies ; Severity of Illness Index ; Tomography, X-Ray Computed ; Treatment Outcome

Cysts ; complications ; pathology ; Epithelial Cells ; pathology ; Humans ; Male ; Middle Aged ; Recurrent Laryngeal Nerve Injuries ; Thyroid Diseases ; complications ; pathology ; Voice Disorders ; etiology

Gastritis cystica profunda (GCP) is a rare disease which shows multiple cystic gastric glands within the submucosa of the stomach. GCP lesions mainly develop at the site of gastroenterostomy and exhibit benign behavior. However, there have been a number of debates over its malignant potential. Several reports have documented GCP accompanied by gastric carcinomas, but the relationship between the two conditions remains uncertain. Here we report two cases of GCP with dysplasia accompanied by synchronous multiple early gastric cancers without previous gastric surgery.
Aged ; Cysts/*diagnosis/pathology ; Early Detection of Cancer ; Gastric Mucosa/pathology ; Gastritis/complications/*diagnosis/pathology ; Humans ; Male ; Neoplasms, Multiple Primary/*diagnosis/pathology ; Stomach Neoplasms/complications/*diagnosis/pathology