Search

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.

Objective: To elucidate the clinicopathologic characteristics of atypical epithelioid trophoblastic lesions with cyst and fistula formation after cesarean section. Methods: The clinical and pathological data of 4 cases of post-cesarean atypical epithelioid trophoblastic lesions with cyst and fistula formation diagnosed at Women's Hospital, School of Medicine, Zhejiang University during April 2007 to June 2018 were evaluated by hematoxylin and eosin stain and EnVision two-step immunohistochemical staining technique. Results: The age of the 4 patients ranged from 32 to 41 years, with a mean age of 36.5 years. Three patients recieved cystectomy and one underwent subtotal hysterectomy. Histologically, the lesions were well circumscribed and consisted of uniform cells of medium size, irregularly enlarged with hyperchromatic nuclei and 1 to 2 inconspicuous nucleoli embedded in abundant hyalinized matrix with fibrinoid material in the center. The cells exhibited immunohistochemical feature of chorionic-type intermediate trophoblastic cells (CK18+, p63+ and CD146-). All patients were alive without recurrence during follow-up of 1 to 40 months (mean = 22 months). Conclusion: Atypical epithelioid trophoblastic lesion with cyst and fistula formation after cesarean section has unique histological features, and its biological behavior and prognosis are still unclear, which need further exploration.

Objective: To investigate the clinicopathologic features, diagnostic and differential diagnostic aspects of pigmented microcystic chromophobe renal cell carcinoma (ChRCC). Methods: Five cases of pigmented microcystic ChRCC were collected at Zhejiang Provincial People's Hospital from January 2013 to January 2018. The clinical features, gross and histological appearances, immunohistochemistry and prognosis were analyzed and the relevant literature was reviewed. Results: There were 3 men and 2 women with age range of 45 years to 72 years (mean 57 years). All tumors were incidentally identified by imaging examinations. Grossly, the tumors were well-demarcated and showed diameters ranging from 1.8 cm to 4.0 cm(mean 2.9 cm). On cross section, the tumors were brown to gray tan with solid cut-surface mixed with multiple cysts of variable sizes. Hemorrhage was common, central scar was not seen. Microscopically, the tumors were composed predominantly of irregular and variable-sized microcystic or tubulocystic patterns, with extensive cribriform structures formation and focal adenomatous rearrangements seen in one case each, and focal pseudo-papillary structures (lacking true fibro-vascular cores) seen in two cases. Microscopic calcifications and psammoma bodies were present in all tumors. Four tumors composed mostly of eosinophilic cells whereas 1 predominated in plant-like cells. Brown pigmentations, either intracytoplasmic or extracytoplasmic, were noted in all five cases. The tumor cells had irregular, low-grade nuclei (Paner grade: 1) frequently with binucleation and perinuclar halos. Tumor necrosis or sarcomatous transformation was not seen. By immunohistochemistry, the tumor cells expressed CK, EMA, and E-cadherin diffusely and strongly in five cases; and CK7 and CD117 diffusely in four cases. They were negative for vimentin, CD10, CA9, AMACR/P504s, TFE3, HMB45, Melan A, S-100 protein, synaptophysin and chromogranin. Partial nephrectomies were performed for all five patients; there was no tumor recurrences or metastases at a follow-up of 2 to 55 months (mean, 17 months). Conclusions: Pigmented microcystic ChRCC is a rare histological variant of ChRCC with relatively indolent behavior, and shows morphologic heterogeneity which can elicit a wide range of differential diagnoses. Careful attentions to search for typical features of classic ChRCC with the use of immunohistochemistry can help to distinguish this tumor from its many mimickers.

Objective: To investigate the diagnosis and treatment of ejaculatory duct cyst., Methods: This study included 2 male patients present at the hospital for hemospermia and abnormal sensation in the perineal region in July and August 2014. Both underwent transrectal ultrasonography, routine semen examination, CT, MRI, cystoscopy, and vesiculography before transurethral fenestration of the cysts and pathological examination of the cyst wall specimens. Analyses were made on the clinical presentations, imaging features, pathological characteristics, differential diagnosis and treatment of ejaculatory duct cyst and relevant literature was reviewed., Results: The cyst wall was mainly composed of smooth muscle, the inner wall lined with pseudostratified ciliated columnar epithelia, and with positive expressions of CD10 and Muc6 proteins on immunohistochemical staining, which indicated renal iatrogenic ejaculatory duct cyst. The patients were followed up for 18 and 20 months, respectively. All symptoms disappeared and no recurrence occurred after surgery. Routine semen examination for the two patients showed the semen volumes to be 3.5 and 3.1 ml, sperm concentrations 35 and 32 ×10⁶/ml, grade a sperm 32.0 and 26.0%, grade b sperm 18.0 and 31.0%, and semen liquidation time 30 and 34 minutes, respectively., Conclusions: Pelvic cystic masses can be detected by transrectal ultrasonography, CT and MRI, but definite diagnosis relies on vesiculography, pathological examination and immunohistochemical staining. Transurethral fenestration is safe and effective for the treatment of ejaculation duct cyst.

Objective: To study the effect of small incision combined with PVC slice through suture in the treatment of auricular pseudocyst. Method: The clinical data of 55 patients with auricular pseudocyst were analyzed retrospectively. The operation method of small incision combined with PVC was adopted. The anterior cartilage of the cyst was not need to remove and the integrity of the auricle stent and the elasticity of the auricle were preserved. Postoperative use of pruning PVC slice through the suture fixed pressure, then observe the recovery of the ear. Result: Fifty-five cases (57 ears) were cured in one time,the cyst disappeared and the auricle shape recovered well. All patients were followed-up for 6-12 months, no recurrence. Conclusion: The combination of small incision and PVC slice through the suture treatment of auricular pseudocyst, the operation is simple, short course, good effect, the patient pain is small, not easy to relapse, safe and reliable, maintaining the original physiological function and natural appearance. It is worthy in clinical application., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.)

Purpose: To discuss the clinical and pathological characteristics and management of parotid gland cyst., Methods: Forty-five resected samples of parotid gland tumors were selected from Shengjing Hospital of China Medical University from January 2000 to June 2014 whose postoperative pathological diagnosis were parotid gland cysts. The clinico-pathological characteristics in different types of parotid gland cysts were analyzed., Results: Of 45 cases of parotid gland cyst, 25 were males, 20 were females. The median age was 51 years old. 21 cases were located on the left side of parotid gland, while 24 on the right side. The main clinical symptom was painless swelling in parotid gland region. The sizes varied from 0.8 cm×0.6 cm×0.5 cm to 10 cm×2.8 cm×0.6 cm. Nine cases were accompanied with pain, and 36 without. 4 cases were seen with lymph node enlargement, and 41 without pain. The main treatment modality was simple resection of cyst without any injury of facial nerve. No recurrence was seen after following up for 1-10 years. Of the 45 postoperative histopathological results, 29 cases (64.4%) were simple cysts, 9 cases (20.0%) were polycystic disease cysts, and 7 cases (15.6%) were lymphoepithelial cysts. Besides the above-stated pathological manifestations, some non-specific microscopic changes were accompanied with parotid gland cysts, including tumor-like hyperplasia of fibrous tissue, inflammations, calcifications, synovial cyst-like changes, foreign-body giant cell reactions and granulomatous changes., Conclusions: The main symptom of parotid gland cyst is progressive enlargement, and simple resection of cyst is the first-line treatment without facial nerve injury or reoccurrence. The main pathological classifications are simple cysts,lymphoepithelial cysts and polycystic disease cysts.

Autosomal dominant polycystic kidney disease (ADPKD) is a most common inherited renal disease, about 50% with a family history, although the exact etiology not yet clear. To date, ADPKD, a multisystem disorder without effective preventive and therapeutic means, has been shown to be detrimental to human health. Recent studies show that severe oligoasthenozoospermia, necrospermia, immotile sperm, azoospermia, epididymal cyst, seminal vesicle cyst, and ejaculatory duct cyst found in male ADPKD patients may lead to male infertility, though the specific mechanisms remain unknown. Structural anomaly of spermatozoa, defect of polycystin, mutation of PKD genes, and micro-deletion of the AZF gene could be the reasons for the higher incidence of abnormal semen quality in male ADPKD patients. Assisted reproductive techniques can increase the chances of pregnancy, whereas the health of the offspring should be taken into consideration. This article presents an overview of reproductive issues concerning infertile male ADPKD patients from the perspective of the morbidity, pathophysiological mechanism, diagnosis, and management of the disease.

Objective: To investigate the value of magnetic resonance three dimensional sampling perfection with application optimized contrasts using different flip angle evolution (3D-SPACE) sequences in diagnosis and surgical strategy modification for obstructive hydrocephalus., Methods: From March 2013 to July 2014, there were 152 cases admitted in People's Liberation Army General Hospital suffered from hydrocephalus, including 88 male patients and 64 female patients aging from 8 months to 79 years. All patients were performed magnetic resonance T2WI and 3D-SPACE sequence scanning before operation. Surgical strategy was made after evaluation of 3D-SPACE sequence. Non-communicating hydrocephalus was treated with endoscopic third ventriculostomy (ETV) and communicating hydrocephalus was treated with ventriculo-peritoneal shunt. According to MR images of direct observation to site of obstruction to determine the detection rate. MRI 3D-SPACE and cranial CT examination were performed in regular follow-up studies., Results: The relevance ratio of 3D-SPACE for the diagnosis of non-communicating hydrocephalus was 98.3% (114/116), while the relevance ratio of conventional T2-weighted MRI was 72.4% (84/116). Among the 152 patients, there were 36 cases with cerebral aqueduct film obstruction, 22 cases with space-occupying lesions in pineal region, posterior part of the third ventricle, or space-occupying lesions in quadrigeminal bodies area, 10 cases with Dandy-Walker symptom, 18 cases with cyst of the anterior pool of the bridge, 16 cases with cysticercosis, 4 cases with cyst of lateral ventricle, 2 cases with cyst of fourth ventricle, 2 cases with space-occupying lesion in foramen ofmonro, 2 cases with foramen ofmonro atresia, 4 cases with craniopharyngioma, 36 cases with communicating hydrocephalus. There were 112 hydrocephalus cases (73.7%) were treated with ETV, without shunt catheter insertion in follow-up study from 1 to 18 months (average (14±9) months)., Conclusions: For obstructive hydrocephalus, MRI 3D-SPACE sequence image has high diagnostic yield rate for providing more detailed anatomical information than conventional MRI. Hence, the advanced imaging methods are helpful for surgical treatment strategy decision making.

Objective: To study the pathology, imaging and clinical features of a child with trisomy 21 syndrome associated interstitial lung disease., Method: Data 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., Result: The 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., Conclusion: Trisomy 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.

Objective: To study the clinicopathologic features of clear cell papillary renal cell carcinoma (CCPRCC)., Methods: The clinical, morphologic and immunohistochemical characteristics of 6 cases of CCPRCC were reviewed, with analysis of follow-up data., Results: There were altogether 3 men and 3 women. The mean age of patients was 56 years. The size of tumors ranged from 1.0 to 4.5 cm in greatest dimension. They had solid or solid-cystic cut surface. Histologically, the tumors were encapsulated and showed several morphologic patterns, with tubules, papillae, acini, interconnecting ribbons and macro/microcysts lined by single layer of cells with clear or small amount of eosinophilic cytoplasm and low-grade nuclei (corresponding to Fuhrman grade 1 or 2). Mitotic figures were rarely seen. Characteristically, there was linear arrangement of the nuclei away from the basement membrane, conferring an appearance similar to that of endometrial glands in early secretory phase. Tubules and cysts contained serosanguineous fluid or colloid-like secretion were identified. No foamy histiocytes, psammomatous calcifications or hemosiderin was present in the papillary areas. Two of the tumors showed focal or extensive angioleiomyoma/leiomyoma-like components. No coagulative necrosis, sarcomatoid dedifferentiation, nor microscopic vascular invasion was observed. Immunohistochemically, all tumors showed strong co-expression of CK7 and CA9 (with characteristic "goblet" staining pattern). The staining for EMA, CK (AE1/AE3), vimentin, CK8, CK18, CK19 and PAX-8 were also positive in all cases. Ki-67 was expressed in less than or about 5% of the tumor cell nuclei. The staining for CD10, P504S, CD117, TFE3 and TFEB was negative. Follow-up data were available in all patients, with mean duration of 14 months (range = 7 to 27 months). All of the patients were disease-free after operation., Conclusion: CCPRCC is a special type of low-grade renal neoplasm with characteristic histopathologic and immunohistochemical features. It needs to be distinguished from clear cell renal cell carcinoma or papillary renal cell carcinoma.

Patients with bilateral nasal vestibule area swelling of 1 week, no obvious pain and symptoms of nasal congestion, bilateral nasal vestibule area was highly uplifted, nostril was significantly smaller, both sides existed a palpable 2. 0 cm × 2. 2 cm and 2. 0 cm × 2. 0 cm cystic mass. Ultrasound showed both sides of the nasal bonehad cystic lesions. Sinus CT showed local quasicircular soft tissue shadow at the bottom of bilateral piriform aperture. The postoperative pathology report: the cystiform tissue is covered with squamous epithelium, infiltration of inflammatory cells could be seen and is consistent with the diagnosis of bilateral nasalvestibular cyst.

Objective: To study the clinicopathological and immunohistochemical features, histogenesis and prognosis of pleuropulmonary blastoma (PPB) in children., Methods: PPB specimens from 16 pediatric cases with an age ranging from 1 year and 7 months to 5 years and 3 months (mean age of 3 years) were retrieved and analyzed by routine histological, immunohistochemical and electron methods., Results: Among 16 patients, there were 2 type I, 7 type II and 7 type III PPB cases. Type I PPB as multilocular cystic structure, consisted of thin fibrous wall lining the respiratory epithelium, subepithelial primitive blastema or immature mesenchymal cells, with or without rhabdomyoblastic differentiation or cartilage; Type II PPB as cystic-solid tumor, comparing with type I, consisted of intracystic components with appearance of anaplastic tumor cells. Type III PPB consisted of completely solid mass, the same as the solid region of type II, had mixed pattern including blastema, undifferentiated spindle-cell proliferations and sarcomas. In addition, anaplastic tumor cells and intra-and extra- cytoplasmic eosinophilic globules were also commonly present. Epithelial components in PPB were benign. Immunohistochemical study showed primitive mesenchymal differentiation of tumors. All cases were positive for vimentin, desmin, myogenin and SMA in tumors with skeletal muscle differentiation, S-100 was positive in tumors with cartilage differentiation. All tumors were negative for synaptophysin, CD99, and CD117. Benign epithelial components were positive for AE1/AE3 and EMA. In 12 cases, electron microscopy revealed few organelles in the primitive mesenchymal cells and rich heterochromatin in mesenchymal cells, the latter also demonstrating cytoplasmic myofilament dysplasia. Nine cases had clinical follow-up ranging from 5 to 48 months, of which 4 patients died., Conclusions: PPB is a rare lung neoplasm of children under the age of 6 years, with distinct pathological morphology. PPB may arise from lung or pleura mesenchymal cells and has a poor clinical outcome.

Objective: To investigate the clinicopathological characteristics and the diagnosis of multilocular cystic renal cell carcinoma (MCRCC)., Methods: The clinicopathological data of 19 MCRCC cases were collected and immunohistochemical staining assays were carried out. Forty-six cases of other cystic kidney lesions within the same period were collected as controls, including extensively cystic clear cell RCC (12 cases), clear cell tubulopapillary renal cell carcinoma (6 cases), tubulocystic carcinoma (2 cases), simple cortical cysts (22 cases), multilocular cystic nephroma (1 cases) and multicystic kidney (3 cases)., Results: The patients included 14 males and 5 females. The ages ranged from 31 to 66 years (median age = 50 years). Most of the MCRCC cases were detected incidentally in physical examination, occasionally accompanied with hematuria, back pain or other symptoms. The follow-up period of 17 patients ranged from 6 to 170 months. All patients were alive without evidence of tumor recurrence or metastasis. Pathological findings showed that macroscopically, tumor size ranges from 1.5 to 7.0 cm in the maximum diameter, generally a entirely of various sized. The cysts contain serous, hemorrhagic or turbid fluid. Solid areas or substantially discernible mural nodules were absent; histologicallly, single layer of cuboidal and flattened epithelial tumor cells were lined in the cysts, described as clear cytoplasm, small nuclear, no nucleoli and low Fuhrman nuclear grade (I or II). Multilayer tumor cells could be observed in a few cysts, with granular cytoplasm and small intracystic papillae formed. The clear tumor cell clusters, similar as cystic lined tumor cells, were seen within pathological fibrous in almost all cases, and significant myofibroblastic proliferation was found in 14 cases. Immunohistochemically, the cysts lined epithelial cells and the clear tumor cell clusters were positive for epithelium markers, including CKpan(19/19), EMA(16/19) and CK7 (15/19); higher percentage of CAIX (17/19) and PAX8(15/19) than control groups, but lower percentage of CD10 (7/19), RCC (6/19) and AMACR(2/19); and all were negative for 34βE12, CD117 and CD68., Conclusions: Multilocular cysts, clear cells clusters of low Fuhrman grade within fibrous septa and capillary vessel proliferation under epithelium are important features of MCRCC. The united using of CAIX, CK7, CD10 and RCC is helpful for differentiating variable cystic renal tumor. MCRCC usually has an excellent prognosis, nephron sparing surgery is first recommended as a therapeutic strategy.

Objective: To analysis the clinical and pathological characteristics of primary iris stromal cyst., Methods: Retrospective cases series study. 14 cases of primary iris stromal cyst which were treated in Tianjin Eye Hospital from Jan.1979 to Dec. 2012 were collected. They were all proved by pathological results. Their clinical and pathological features were analyzed., Results: The age of 14 patients were 5 months to 50 years old, the median is 10 years old.10 patients (71.4%) were attacked from children or adolescence, among which onset age of 8 patients were less than 1-year-old. 4 patients (28.6%) occurs when they were adults. 10 cases were female and 4 cases were males. 10 cases were left eyes, 4 cases were right eyes. All cases were unilateral involvement. In these cases, 11 cases appeared as a single translucent or gray-white cystic mass on the iris surface, which 6 cases located in the inferior aspect of the iris, 2 cases appeared with diffused iris cyst and another 1 case's cyst was found occasionally in the eyeball enucleating specimen. 8 cases had corneal opacity or leucoma, 7 cases had secondary glaucoma, and 3 cases had complicated cataract. Pathological features showed: cysts located on the iris surface or in the stroma layer. The cysts of 9 cases were constructed of non-cuticular stratified squamous epithelium cells. 3 cases' cysts were constructed of lens epithelium cells and lens capsular analogical tissues, and the cysts adhesion to the lenstightly. The other 2 cysts were constructed of single or stratified epithelium cells, similar to ciliary epithelium cells. 2 cases complicated with crystalline lens dysplasia, 3 cases complicated with iris tissue dysplasia, 2 cases complicated with ciliary cysts and 1 case had chamber angle dysplasia., Conclusions: Primary iris stoma cyst is rare and it usually occurs in infant, more often in the children less than 1-year-old. Pathological examination showed that most of the cysts originated from the conjunctivaepithelium, minority cysts originated from crystalline lens epithelium or ciliary epithelium. Some cases complicated with iris, ciliary or crystalline lens malformation.

Objective: To 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., Methods: HRCT 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., Results: On 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)., Conclusions: On 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.

A 55 year old man complained of a painless mass at the left maxillofacial region. The mass had continuously grown over 10 years. Upon physical examination a cystic mass with size of 5 cm in length with bulging smooth surface was seen on the left maxillofacial region. Computed tomography (CT) scan showed a giant cyst with bone destruction and invasion in the left maxilla, maxillary sinus and nasal cavity. Needle aspiration of the mass yielded 80 milliliter of brown fluid. The excisional biopsy was made which revealed ghost cells and dysplastic dentin that were features of dentinogenic ghost cell tumor. Finally, a dentinogenic ghost cell was diagnosed.

Objective: To report a rare case of ectopic mesonephric duct cyst with ectopic testicular malignancy and improve the diagnosis and treatment of the disease., Methods: We 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., Results: A 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., Conclusion: Ectopic 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.

Objective: To improve the diagnostic ability of leukoencephalopathy with cerebral calcifications and cysts (LCC), a rare central nervous system disease., Methods: The clinical manifestations, neuroimages and neuropathological features of a 19-year-old male patient were analyzed. A total of 20 cases from 14 literatures were reviewed., Result: The patient was admitted with right limb weakness, cognitive decline, headache and blurred eyesight. Head CT scan showed multiple calcifications, cysts formation and leukoencephalopathy. Brain MRI showed several cysts in bilateral hemisphere, basal ganglia, thalamus and paraventricular areas. A mural nodule was noted inside one of the cyst, which was enhanced on the contrasted MRI. The wall of the cysts was partially enhanced, but not with the fluid inside the cysts. The corresponding CT calcifications foci showed on T1 and T2 with either both hyperintensity or both hypointensity, which was also partial enhanced. Extensive leukoencephalopathy was formed around the cysts and the ventricles. But neither Cho nor NAA changed a lot on MRS. Amplitude diagram of SWI series exhibited multiple round small dark signals all over the affected areas with mixed signals showed in the phase diagram, which indicated both calcifications and microbleeding at the lesions. Neuropathological examinations found no tumor cells in the operated cyst, and showed angiomatous small blood cells were dominant in the cyst wall. Hyaline degenerations, microcalcifications and hemosiderin deposition were observed. No obvious demyelination was discovered, while gliosis, numerous Rosenthal fibers and fibrinoid vascular necrosis were found around the lesions. The clinical, neuroimaging and pathological features of this patient were in accordance with the cases reported in the literatures., Conclusions: Neuroimaging is the most important method for the diagnosis of LCC. As small vessel lesions are probably closely related to the pathophysiology of LCC, SWI could be recommended to further reveal the etiology of LCC.

Objective: To explore HEPACAM mutations in a Chinese family with megalencephalic leukoencephaloptathy with subcortical cysts (MLC)., Method: Genomic DNA samples were extracted from peripheral blood of the proband and her parents. All exons and exon-intron boundaries of HEPACAM and MLC1 were amplified in the MLC family by polymerase chain reaction (PCR) followed by direct DNA sequencing., Result: Two heterozygous mutations of HEPACAM located in exon 2, c.203A > T(p.K68M) and c.395C > A(p.T132N), were identified in the proband. The proband's mother had the heterozygous mutations c.203A > T(p.K68M), and her father had the heterozygous mutation-c.395C > A(p.T132N). There was no variation found in MLC1 gene., Conclusion: The proband was heterozygous compound MLC patient carrying on one allele with the c.203A > T(p.K68M) mutation inherited from her mother, and the other allele with the c.395C > A(p.T132N) mutation inherited from her father. The parents both are heterozygous carriers with normal phenotype. The disease-causing gene for this family was resulted in HEPACAM mutation other than MLC1 mutation.

Objective: To investigate the prevalence and anatomy features of iridociliary body cysts in patients with narrow anterior chamber angle., Methods: Retrospective case series study. The prevalence and anatomy features of iridociliary body cysts in 223 patients (402 eyes) were analyzed retrospectively with ultrasound biomicroscopy (UBM). All of the patients were examined for susceptive narrow anterior chamber angle without complaint. The age of the patients, the site, diameter and number of cysts, the anterior chamber angle and the central anterior chamber depth were measured., Results: Iridociliary body cysts were found in 19 patients (23 eyes) out of 223 patients (402 eyes), the prevalence is 5.7%. Fifteen patients were unilateral and four patients bilateral. Two cases originated from the ciliary process, eighteen cases from the iris root, and three from both the root and posterior surface of the iris. Twenty one cases were single cysts while two cases were multiple cysts. The diameter of the cysts ranged from 0.5 to 3.1 mm, averaged (0.71 ± 0.53) mm. The average age and the central anterior chamber depth of the eyes with iridociliary body cysts were (55.32 ± 10.74) years and (2.25 ± 0.39) mm, with no significant difference (t = 0.534, 0.783; P > 0.05) as compared to that of patients without cysts, which were (57.46 ± 10.52) years and (2.14 ± 0.34) mm. The anterior chamber angle in iridociliary body cysts group was 8.2° (21.0°, 0.0°), with no significant difference (Z = -0.062, P > 0.05) as compared to that of patients without cysts, which was 8.9° (21.4°, 0.0°)., Conclusions: The prevalence rate of iridociliary body cysts in this study is 5.7%, central anterior chamber depth and anterior chamber angle in patients with cysts do not differ form patients without cysts.

Objective: To study the clinical pathology and treatments on the pseudocyst of auricle., Methods: Sixty cases of auricular pseudocyst were treated by surgery from 1993 to 2008 in our hospital. Their operation effects and the clinic pathological features were analyzed., Results: The clinic pathological data showed that the source of serous effusion of auricular pseudocyst origin from cartilage membrane in the top wall. In the early stage of the cyst, the top wall of auricular pseudocyst was the cartilage membrane. With the course progresses, the cartilage membrane in the top wall of auricular pseudocyst was proliferating, thickened and generated new cartilage. The new cartilage was formed from small piece to the big one, and eventually became an entire new cartilage on the top wall of auricular pseudocyst. Serous effusion at this time was terminated, and this cyst became intra-cartilaginous effusion of auricle. Finally the fluid between cartilages was absorbed and organized. In the cyst, the new cartilage and auricle cartilage were organized and adhered together each other. The auricle became thickened and deformed. The observation of capsule wall under light microscope showed that there were a few fibrous desmoplasia, anapetia and lymphocyte infiltrating in the fibrous tissue, as well as that there were cartilage cell layers from firmness to thicker. The cartilage cells and their lacunes were small, and the cartilage capsule and the basilaris substantia was showed as eosin. This data indicated that the cartilage was neogenesis but not degenerating. Sixty patients were followed up from 3 months to 1 year. The effect of surgical treatment for the auricular pseudocyst was satisfactory. There was no auricular deformation in these patients with the operation., Conclusions: Auricular pseudocyst can be divided into the early period (acute exudative period), the medium period (cartilage formation period) and the late period (proliferative and organized period). The treatment should be based on the pathological findings of auricular pseudocyst. The operation is easy, safe and reliable. The key of the operation is the complete removal of perichondrium and cartilage at the top of auricular pseudocyst.

Objective: To evaluate therapeutical effect of combined hepatic resection and fenestration on patients with severe adult polycystic liver disease (APLD)., Methods: Preoperative 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., Results: The 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)., Conclusion: Combined hepatic resection and fenestration is a safe and acceptable procedure for the treatment of severe APLD.

Objective: To study the clinical and pathological characteristics of nonfunctioning parathyroid cysts., Method: Six cases of nonfunctioning parathyroid cysts who were hospitalized during 1997-2007 were retrospectively analyzed. The clinical features, serum calcium, B-ultrasonic, CT and nuclide scanning were performed. Postoperative pathologic and immunohistochemistry detection in nonfunctioning parathyroid cysts were made., Result: Tumors in the 6 cases were underwent surgical ablation and diagnosis was verified by pathology. CgA (chromogranin A), Syn (synaptophysin), NSE (and neuron specific enolase) expressions were positive. Follow-up six months and five years after operation, there is no recurrence., Conclusion: Surgical resection is most effective in treating nonfunctioning parathyroid cysts and diagnosis is made by pathologic examination.

Objective: To investigate the pathogenesis of nasal vestibular cyst., Method: Forty two case of nasal vestibular cyst were retrospectively analyzed, including clinical manifestation, pathological findings, surgical therapy efficacy., Result: Of these 42 cases of the type of lining epithelia, pseudostratified columnar epithelium was identified in 24 cases, stratified squamous epithelium in five cases, simple cuboidal epithelium in five cases, and two kinds of above mentioned epithelia in eight cases. Goblet cells were present in 23 cases. Surgical excision was performed via the labiogingival groove approach for all patients. The duration of follow-up ranged from 6 months to 10 years. No postoperative recurrences were found., Conclusion: The abnormal development of nasolacrimal duct systems may take an important role in the pathogenesis of nasal vestibular cyst. According to this, we considered the total excision via the sublabial approach is more reasonable surgery than endoscopic marsupialization, but it is need further observation.

Objective: To explore the hepatic injury induced by CCl4in SD rat., Method: 40 SD rats were allocated to male and female group, consisting of 20 animals/sex/group. SD rats were given at 2 mL x kg(-1) of 10% CCl4 through celiac injection per 3 day for 12 days. All rats were killed by anaesthesia of ethyl ether and bleeding through abdominal aorta at 12th day. Liver tissue was fixed in 10% neutral formalin, embedded in paraffin, cut at a nominal thickness of 3 microm, stained with hematoxylin and eosin ( H&E) , evaluated at by microscopic examination., Result: 19 cases with local necrosis, 8 cases with fatty degeneration, 9 cases with cystic degeneration and 2 cases with fibrosis were seen in group male. 20 cases with local necrosis, 9 cases with fatty cases degeneration, 1 case with cystic degeneration and 1 case with fibrosis were seen in group female. The incidence of cystic degeneration in male group was found significantly higher than that in female group (P < 0. 05) , but the incidence of other lesions was no significant difference between male and female group., Conclusion: CCl4 induces local necrosis , fatty degeneration, fibrosis and cystic degeneration in SD rat. The incidences of local necrosis , fatty degeneration and fibrosis were no significantly difference between male and female rat, but the incidence of cystic degeneration in male rats was significant higher than that in female rats.

Objective: To investigate the value of ultrasonography in the diagnosis of adrenal gland cysts., Methods: Six patients with adrenal gland cysts were examined by two-dimensional ultrasonography and color Doppler flow imaging (CDFI), and the results were compared with the pathological findings., Results: The diagnoses by ultrasonography and CDFI were consistent with the pathological findings in 5 cases., Conclusion: Ultrasonography and CDFI can be an accurate diagnostic modality for adrenal gland cysts.

Objective: To elucidate the relationship between the auricular pseudocyst and the immunological function of patients., Method: The cyst fluid and blood sample had been detected for contents of IgG, IgA, IgM and complement C3 by radial immunodiffusion, immunocomplex (IC) contents by polyethylene glycol turbidimetry in 55 cases, anti-nuclear antibody (ANA) by immuno-fluorescent technique in 23 cases, extractable nuclear antibody (ENA) by immunotransfer technique in 24 cases. The frozen sections of cyst wall tissue of 24 cases had been detected for immunocomplex by immunoenzyme histochemistric method. The reactions were observed separately between the auricle tissue of healthy white rat, human embryo and cyst fluid, serum of patient and normal serum., Result: Contents of IgG, IgA, IgM and C3 in the cyst fluid were lower than the serum significantly (P < 0.01). No IC, ANA and ENA could been found both in serum and cyst fluid, but reactive immunocomplex was discovered on the auricle cartilagous tissue of patient, immunocomplex also be observed on human embryo aurical tissue when it reacted with cyst fluid, but complex hadn't be found when the auricle tissue of white rat and human embryo reacted with serum of patient and normal people., Conclusion: The reason of auricular pseudocyst may be related with the local autoimmune status of patient.

The etiology and clinical pathological analysis of 147 cases of epididymal mass were presented. The result showed that the most common mass in inflammatory (66%), and the majority was tuberculosis, the next was cystic diseases (30%). However, neoplasm was relatively less and malignant mass was rare. The diagnosis can be established by careful history, physical examination, and BUS. The selective surgery depends on the nature of diseases, the demand of the patients and the effect of conservative therapy.

Among 1352 eyes examined histopathologically, ciliary body epithelial cysts were found in 198 eyes. The cysts can be divided into 3 types: non-pigmented epithelial, pigmented epithelial and intra-epithelial cysts, the non-pigmented epithelial cysts being the most commonly seen. The former 2 types are formed by the proliferation of the inner and outer layer of the optic cup respectively and the formation of the latter one is related to the separation of the 2 layers of the cup. The cysts can be single or multiple, at most 14 non-pigmented ciliary epithelial cysts with 2 iris epithelial cysts being present in one eye. The incidence of ciliary epithelial cysts is as high as 14.6%. Because of the hidden position, clinical doctors seldom notice them. They can be misdiagnosed as melanoma, they can cause secondary glaucoma and localized cataract and they can be detached spontaneously. Therefore, clinical doctors ought to pay attention to this disease.

Six patients with adrenal cysts, a rare disease, were treated. Among 5 patients undergoing operations, 4 had pseudocysts and one had epithelial cyst. Most of the patients had no symptoms. B-ultrasonography and CT scanning played a leading role in the diagnosis of the disease. The incidence, etiology, pathological classification and treatment of adrenal cysts are discussed.

16 ischemic femoral heads from human adult were studied with radiologically and histologically. The two heads from fresh fracture of femoral neck of 1 month looked grossly normal; radiograms and CT showed no abnormality though necrosis surrounded by living bone tissue without cystic formation was found on the top area histologically. The pathology including the 12 collapsed heads from old neck fracture and 2 osteoarthritic heads were similar but varied in degree and extent of mineralization with time. Numerous cysts, were walled off by a layer of osteosclerosis on the necks side. While dense fibrovascular tissues some containing bits of dead bone trabeculae and cartilage filled certain cystic areas, empty cysts were found in 8 of the specimens. Beyond the cystic area, the bone and bone marrow adequately congested and had fairly good blood supply. Based on these findings, the authors suppose that leaking of joint fluid through fissures into deeper part of the head form cisterns which expand with increasing joint fluid pressure may be one of the causes of cyst formation. In early stage of ischemia with head in normal contour, replacement of the degenerated area with bone grafting may serve as a good choice of treatment.

One hundred and seventy one patients with mesentery tumors confirmed by surgery and/or pathology in five hospitals of Hubei Province from 1966 to 1986 are analyzed retrospectively. The main manifestations were abdominal pain, abdominal mass and emaciation. Cyst and leiomyomas were common in benign tumors. Malignant lymphoma, leiomyosarcoma and liposarcoma were predominant in malignant tumors. The morbidity, clinico-pathologic characteristics, diagnosis and differential diagnosis of this group of tumor are discussed.