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1.
Invest Ophthalmol Vis Sci ; 63(11): 8, 2022 10 03.
Artigo em Inglês | MEDLINE | ID: mdl-36227606

RESUMO

Purpose: Loss of retinoschisin (RS1) function underlies X-linked retinoschisis (XLRS) pathology. In the retina, both photoreceptor inner segments and bipolar cells express RS1. However, the loss of RS1 function causes schisis primarily in the inner retina. To understand these cell type-specific phenotypes, we decoupled RS1 effects in bipolar cells from that in photoreceptors. Methods: Bipolar cell transgene RS1 expression was achieved using two inner retina-specific promoters: (1) a minimal promoter engineered from glutamate receptor, metabotropic glutamate receptor 6 gene (mini-mGluR6/ Grm6) and (2) MiniPromoter (Ple155). Adeno-associated virus vectors encoding RS1 gene under either the mini-mGluR6 or Ple-155 promoter were delivered to the XLRS mouse retina through intravitreal or subretinal injection on postnatal day 14. Retinal structure and function were assessed 5 weeks later: immunohistochemistry for morphological characterization, optical coherence tomography and electroretinography (ERG) for structural and functional evaluation. Results: Immunohistochemical analysis of RS1expression showed that expression with the MiniPromoter (Ple155) was heavily enriched in bipolar cells. Despite variations in vector penetrance and gene transfer efficiency across the injected retinas, those retinal areas with robust bipolar cell RS1 expression showed tightly packed bipolar cells with fewer cavities and marked improvement in inner retinal structure and synaptic function as judged by optical coherence tomography and electroretinography, respectively. Conclusions: These results demonstrate that RS1 gene expression primarily in bipolar cells of the XLRS mouse retina, independent of photoreceptor expression, can ameliorate retinoschisis structural pathology and provide further evidence of RS1 role in cell adhesion.


Assuntos
Cistos , Retinosquise , Animais , Cistos/metabolismo , Cistos/patologia , Eletrorretinografia , Proteínas do Olho/genética , Proteínas do Olho/metabolismo , Camundongos , Retina/patologia , Células Bipolares da Retina/metabolismo
2.
Zhonghua Gan Zang Bing Za Zhi ; 30(9): 931-933, 2022 Sep 20.
Artigo em Chinês | MEDLINE | ID: mdl-36299185

RESUMO

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.


Assuntos
Doença de Caroli , Cistos , Hamartoma , Hepatopatias , Neoplasias Hepáticas , Humanos , Hepatopatias/diagnóstico , Hepatopatias/terapia , Hepatopatias/patologia , Cistos/diagnóstico , Cistos/terapia , Cistos/patologia , Doença de Caroli/diagnóstico , Neoplasias Hepáticas/terapia
3.
Radiol Clin North Am ; 60(6): 951-962, 2022 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-36202481

RESUMO

Diffuse cystic lung disease refers to multiple rounded lucencies or low-attenuating areas with well-defined interfaces with normal lung. Parenchymal lucencies, such as cavitary disease, may mimic cystic lung disease. Cystic lung disease generally has a nonspecific presentation. Pulmonary cysts may present in isolation or with ancillary imaging features, such as ground-glass opacities or nodules. Clinical features, such as connective tissue disease, can narrow the differential diagnosis. In cases with indeterminate imaging and clinical features, open lung biopsy should be considered. Ultrarare cystic lung diseases, such as light chain deposition disease, can mimic more common diseases.


Assuntos
Cistos , Pneumopatias , Neoplasias Pulmonares , Cistos/diagnóstico por imagem , Cistos/patologia , Diagnóstico Diferencial , Humanos , Pulmão/diagnóstico por imagem , Pulmão/patologia , Pneumopatias/diagnóstico por imagem , Neoplasias Pulmonares/patologia , Tomografia Computadorizada por Raios X/métodos
4.
Tokai J Exp Clin Med ; 47(3): 109-111, 2022 Sep 20.
Artigo em Inglês | MEDLINE | ID: mdl-36073280

RESUMO

BACKGROUND: Peribronchiolar metaplasia (PBM) is a lesion characterized by an abnormal connection between the terminal bronchiole and parabronchiole via the Lambert's canals. We report a rare case of PBM incidentally detected during a surgery for pneumothorax. CASE PRESENTATION: At 38-year-old man was admitted to our hospital with chest pain. He was diagnosed with pneumothorax and treated using a thoracic drain 12 years ago. Chest computed tomography revealed a cyst in the right upper lobe and ground glass lesion with a solid component in the right lower lobe of the lung. Hence, we performed a surgery for pneumothorax management, which revealed a cyst in the right upper lobe and induration with angiogenesis in the right lower lobe. We performed partial resection of the right upper and lower lobes. Pathological examination of the lower lobe nodule revealed small airways with lymphocytic inflammation and bronchiolar metaplasia. Pathological diagnosis of the nodule was PBM. CONCLUSION: Although PBM is considered a lesion with good prognosis, there have been cases associated with early-stage lung cancer. Hence, care should be taken to distinguish PBM from other neoplasms. However, preoperative diagnosis is difficult in most cases so complete surgical resection is recommended, if feasible.


Assuntos
Cistos , Neoplasias Pulmonares , Pneumotórax , Adulto , Cistos/complicações , Cistos/patologia , Humanos , Pulmão/diagnóstico por imagem , Pulmão/patologia , Pulmão/cirurgia , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/patologia , Neoplasias Pulmonares/cirurgia , Masculino , Metaplasia/complicações , Metaplasia/patologia , Pneumotórax/diagnóstico por imagem , Pneumotórax/etiologia , Pneumotórax/cirurgia
5.
Rev Esp Patol ; 55(4): 278-281, 2022.
Artigo em Espanhol | MEDLINE | ID: mdl-36154737

RESUMO

Intestinal (tailgut) cysts or retrorectal cystic hamartomas are rare benign lesions that are included in the category of developmental cystic lesions. Their origin is still uncertain, although several hypotheses have been proposed to explain their development. They are located mainly in the presacral (retrorectal) space and predominately affect middle-aged women (40-60 years). Taking into account location and histological characteristics, the main differential diagnoses include epidermoid cysts, duplication cysts and teratomas. Malignant transformation of these lesions is rare and preferentially into adenocarcinoma and neuroendocrine tumors. We present a case of an intestinal cyst associated with a well-differentiated neuroendocrine tumor (G1) in a 63-year-old woman.


Assuntos
Adenocarcinoma , Cistos , Hamartoma , Tumores Neuroendócrinos , Adenocarcinoma/complicações , Adenocarcinoma/patologia , Cistos/complicações , Cistos/patologia , Diagnóstico Diferencial , Feminino , Hamartoma/patologia , Humanos , Pessoa de Meia-Idade , Tumores Neuroendócrinos/complicações , Tumores Neuroendócrinos/diagnóstico
6.
Radiographics ; 42(7): 2037-2053, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36149823

RESUMO

Normal Cowper glands and ducts are imperceptible at imaging. However, abnormalities of the Cowper glands and ducts are increasingly seen owing to increasing use of cross-sectional imaging. In this article, the authors present a comprehensive review of the normal anatomy of the Cowper glands and ducts and the clinical and imaging findings of conditions that affect them in an effort to help lead to appropriate diagnosis and management. A Cowper duct syringocele is a congenital or acquired dilatation of the Cowper duct in the bulb of the corpus spongiosum. Retrograde urethrography is the standard test to diagnose a communicating (open) Cowper duct syringocele, and MRI is used for further morphologic assessment. Cowperitis refers to inflammation of the gland and/or duct, and recurrent cowperitis is usually associated with an underlying syringocele, a urethral fistulous connection to the Cowper gland, or a perianal fistula. MRI allows detection of the underlying structural abnormality and the superimposed inflammation. Cowper stones are typically radiopaque and are found in both the Cowper gland and duct. A Cowper gland hemorrhagic cyst may be incidentally detected at MRI of the prostate, and follow-up MRI may be required to ensure evolution of hemorrhage and exclude an underlying neoplasm. Cowper gland carcinoma is rare and includes adenocarcinoma and adenoid cystic carcinoma subtypes. Transperineal US may be used for initial evaluation and CT and/or MRI are necessary for further characterization and staging. Cowper gland hyperplasia is a rare entity with few previous reports that may be incidentally detected at imaging and may mimic malignancy. ©RSNA, 2022.


Assuntos
Cistos , Doenças Uretrais , Masculino , Humanos , Glândulas Bulbouretrais/patologia , Diagnóstico Diferencial , Cistos/patologia , Inflamação
7.
J Vet Diagn Invest ; 34(6): 983-989, 2022 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-36056527

RESUMO

An 18-y-old female tufted deer (Elaphodus cephalophus) had a short history of chronic diarrhea, progressive weight loss, and hindlimb instability. Given the poor prognosis, the deer was euthanized and submitted for postmortem examination. The most significant gross finding was segmental and multinodular mural thickening of the proximal colon. On cut surface of the affected colonic segments, 0.5-2-cm diameter, intramural, multiloculated, cystic structures containing gray, translucent, gelatinous material elevated the edematous mucosa. Microscopically, the intramural cystic structures were filled with mucinous matrix admixed with foamy macrophages, and lined by discontinuous segments of well-differentiated columnar, pancytokeratin-positive epithelium with basilar nuclei. Multifocally, transition was observed from hyperplastic mucosal crypt epithelium to dysplastic or neoplastic columnar and flattened epithelium lining submucosal and serosal cysts. Cyst lumina were irregularly disrupted by polypoid ingrowths of collagenous tissue covered by attenuated epithelium. Based on these findings, we diagnosed a well-differentiated mucinous adenocarcinoma. Although intestinal adenocarcinomas have been described in humans and animals, they are considered uncommon in most domestic species, except for sheep, for which genetic and environmental factors appear to influence occurrence. Our report addresses the knowledge gap regarding intestinal adenocarcinomas affecting cervids and specifically the tufted deer, a less-studied, near-threatened Asian cervid.


Assuntos
Adenocarcinoma Mucinoso , Adenocarcinoma , Cistos , Cervos , Doenças dos Ovinos , Humanos , Animais , Feminino , Ovinos , Diagnóstico Diferencial , Colo/patologia , Cistos/patologia , Cistos/veterinária , Adenocarcinoma/diagnóstico , Adenocarcinoma/veterinária , Adenocarcinoma/patologia , Adenocarcinoma Mucinoso/diagnóstico , Adenocarcinoma Mucinoso/veterinária , Adenocarcinoma Mucinoso/patologia , Doenças dos Ovinos/patologia
8.
Head Neck ; 44(11): 2522-2527, 2022 11.
Artigo em Inglês | MEDLINE | ID: mdl-35912938

RESUMO

BACKGROUND: Postoperative sialoceles and fistulas are frequent surgical complications of parotid tumor resection. Extracapsular dissection by the sternocleidomastoid muscle-parotid space approach (ECD-SMPSA) is a minimally invasive technique. To our knowledge, the characteristics of sialoceles and fistulas secondary to ECD-SMPSA have not been reported. METHODS: This prospective study enrolled 52 patients who underwent ECD-SMPSA without sialocele/fistula prevention measures. Postoperative sialoceles and fistulas were evaluated during 2 months of follow-up. RESULTS: Among the 52 patients, only one male patient developed a mild sialocele. No salivary fistulas occurred. The overall rate of sialocele/fistula formation was 1.92%. CONCLUSIONS: When treating clinically benign tumors that involve the sternocleidomastoid muscle-parotid space, ECD-SMPSA may prevent postoperative formation of sialoceles and salivary fistulas.


Assuntos
Cistos , Fístula , Neoplasias Parotídeas , Doenças das Glândulas Salivares , Cistos/patologia , Humanos , Masculino , Músculos/patologia , Glândula Parótida/patologia , Glândula Parótida/cirurgia , Neoplasias Parotídeas/cirurgia , Complicações Pós-Operatórias/patologia , Complicações Pós-Operatórias/prevenção & controle , Estudos Prospectivos , Estudos Retrospectivos
9.
J Neurooncol ; 160(1): 33-40, 2022 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-35921021

RESUMO

INTRODUCTION: Cystic meningiomas are rare, accounting for 2-7% of all intracranial meningiomas. Little is known regarding whether these meningiomas behave differently compared to solid meningiomas. We sought to study this relatively uncommon imaging appearance of meningioma and to evaluate its clinical significance. METHODS: A single-institution retrospective cohort study of surgically-treated meningioma patients between 2000 and 2019 was conducted. Cystic meningioma was defined as a tumor with an intratumoral or peritumoral cyst present on preoperative imaging. Demographics, preoperative imaging, histopathology characteristics, operative data, and surgical outcomes were reviewed. Imaging variables, histopathology and outcomes were reported for cystic meningiomas and compared with non-cystic meningiomas. Univariate/multivariable analyses were conducted. RESULTS: Of 737 total meningiomas treated surgically, 38 (5.2%) were cystic. Gross total resection (GTR) was achieved in 84.2% of cystic meningioma patients. Eighty-two percent of cystic meningiomas were WHO grade I (n = 31), 15.7% were grade II and 2.6% were grade III. Most cystic meningiomas had low Ki-67/MIB-1 proliferation index (n = 24, 63.2%). A total of 18.4% (n = 7) patients with cystic meningioma had recurrence compared to 12.2% (n = 80) of patients with non-cystic meningioma (p = 0.228). No significant difference in median time to recurrence was observed between cystic and non-cystic meningiomas (25.4, Q1:13.9, Q3:46.9 months vs. 13.4, Q1:8.6, Q3:35.5 months, p = 0.080). CONCLUSIONS: A small portion of intracranial meningiomas have cystic characteristics on imaging. Cystic meningiomas are frequently WHO grade I, have low proliferation index, and had similar outcomes compared to non-cystic meningioma. Cysts in meningioma may not be a surrogate to determine aggressive meningioma behavior.


Assuntos
Cistos , Neoplasias Meníngeas , Meningioma , Humanos , Meningioma/diagnóstico por imagem , Meningioma/cirurgia , Meningioma/patologia , Neoplasias Meníngeas/diagnóstico por imagem , Neoplasias Meníngeas/cirurgia , Neoplasias Meníngeas/patologia , Estudos Retrospectivos , Cistos/patologia , Recidiva Local de Neoplasia/cirurgia
10.
Adv Anat Pathol ; 29(6): 358-364, 2022 Nov 01.
Artigo em Inglês | MEDLINE | ID: mdl-35918293

RESUMO

Fine needle aspiration is a well-known procedure for the diagnosis and management of thyroid lesions, representing the first diagnostic tool for the definition of their nature. In clinical practice, a thyroid nodule can be classified as solid, cystic, and partially cystic based on its internal components. Different thyroid imaging reporting systems and cytologic diagnostic systems have focused their attention on solid nodules, which are more frequently linked with a malignant outcome. In fact, numerous papers demonstrated that nodules with microcalcifications, a taller-than-wide shape, hypoechogeneity, and irregular margins, are more likely to be malignant on histology. Nevertheless, according to the literature, the risk of malignancy in a partially cystic thyroid nodule ranges between 3.3 and 17-5%, including, for instance, the possible diagnosis of a cystic papillary thyroid carcinoma and other malignant entities. Therefore, in the current review article, we are going to discuss the approach to thyroid cystic lesions on fine needle aspiration cytology.


Assuntos
Cistos , Neoplasias da Glândula Tireoide , Nódulo da Glândula Tireoide , Humanos , Biópsia por Agulha Fina , Nódulo da Glândula Tireoide/diagnóstico , Nódulo da Glândula Tireoide/patologia , Neoplasias da Glândula Tireoide/diagnóstico , Neoplasias da Glândula Tireoide/patologia , Cistos/patologia
11.
Eur J Endocrinol ; 187(3): 429-437, 2022 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-35894855

RESUMO

Objective: Benign adrenal cysts are rare lesions of the adrenal glands. Limited data are available to guide management. We aimed to describe the presentation and outcomes of patients with benign adrenal cysts. Design: Retrospective longitudinal cohort study. Methods: Consecutive patients with histologically or radiologically confirmed adrenal cysts between 1995 and 2021 were identified. Pheochromocytomas and malignancy were excluded. Results: Benign adrenal cysts were diagnosed in 92 patients (53, 57% women) at a median age of 45 years. Mode of discovery was incidental on imaging in 81 (88%), symptoms of mass effect in 9 (9.8%), and others in 2 (2.2%). Majority (89, 97%) of patients had unilateral cysts (45 right, 44 left) with a median size of 48 mm (range 4-200) at diagnosis. On imaging, most cysts were round/oval (85.4%), homogenous (83.2%) lesions with calcifications (64.0%) and no vascular enhancement (97.7%). During a median follow-up of 65 months (range 7-288), adrenal cysts demonstrated minimal enlargement (median size change 6 mm, median growth rate 2 mm/year). On hormonal evaluation, 10% (5/50 tested) had an abnormal overnight dexamethasone suppression test, and 9.5% (4/42 tested) had an abnormal case detection testing for primary aldosteronism. Patients treated with adrenalectomy (46, 50%) were younger (36.9 years vs 50.8 years, P = 0.0009) and had more rapidly enlarging cysts (median growth rate 5.5 mm/year vs 0.4 mm/year, P = 0.0002). Conclusion: Benign adrenal cysts are usually incidentally discovered, non-functional, homogenous lesions without vascular enhancement that demonstrate minimal growth. Adrenalectomy should be reserved for patients with heterogeneous lesions, abnormal hormonal evaluation, or those with mass effect symptoms.


Assuntos
Neoplasias das Glândulas Suprarrenais , Cistos , Neoplasias das Glândulas Suprarrenais/diagnóstico por imagem , Neoplasias das Glândulas Suprarrenais/patologia , Adrenalectomia , Cistos/diagnóstico por imagem , Cistos/patologia , Feminino , Humanos , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
12.
Jt Dis Relat Surg ; 33(2): 426-434, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35852204

RESUMO

OBJECTIVES: In this study, we aimed to evaluate the short-to-midterm results of the resection and reconstruction of large cystic lesions of the humerus. PATIENTS AND METHODS: Eight male patients (median age: 22.9±10.4 years; range, 12 to 42 years) with large cystic lesions of the humerus operated between January 2017 and December 2019 were retrospectively analyzed. The age of the patients, their previous treatments and follow-up periods, the size and location of the cysts, postoperative functional scores, presence of a union, recurrence of the cyst, and graft resorption were examined. RESULTS: The mean follow-up was 42.8±7.5 (range, 34 to 54) months. Preoperatively, the mean length of the cystic lesions was 15.1±2.6 (range, 10 to 18) cm. At the final follow-up, the patients had a normal range of shoulder flexion-extension, internal rotation-external, abduction-adduction, and elbow flexion-extension, pronation-supination. The patients had a mean DASH score of 1.13±1.1 (range, 0 to 3.3) and MSTS score of 28.75±1.8 (range, 26 to 30) postoperatively. Complications such as pseudoarthrosis, graft resorption, or cyst recurrence were not observed in any of the patients. CONCLUSION: Although the risk of recurrence is low in small cystic lesions of the humerus, it increases as the size of the lesion increases. This reconstruction technique using vascularized fibular grafts, which we applied, seems to be extremely successful in ensuring biological healing and preventing recurrence and complications in patients with large cystic lesions of the humerus.


Assuntos
Neoplasias Ósseas , Cistos , Procedimentos Cirúrgicos Reconstrutivos , Adolescente , Adulto , Neoplasias Ósseas/patologia , Transplante Ósseo/métodos , Criança , Cistos/etiologia , Cistos/patologia , Cistos/cirurgia , Humanos , Úmero/cirurgia , Masculino , Procedimentos Cirúrgicos Reconstrutivos/métodos , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
13.
Nihon Shokakibyo Gakkai Zasshi ; 119(7): 683-691, 2022.
Artigo em Japonês | MEDLINE | ID: mdl-35811126

RESUMO

A 52-year-old female had cholecystoduodenostomy for biliary atresia of type I cyst at 120 days of age. The patient's surgery recovery was uneventful;however, the patient had recurring cholangitis at the age of 27. The patient had high hepatobiliary enzymes in the outpatient clinic and was diagnosed with cholangitis. In general, the Kasai method is the mainstream for biliary atresia, since it has a much-reduced incidence of both early and late postoperative problems. However, this patient had biliary atresia of type I cyst and had undergone cholecystoduodenostomy. We suspected that the obstructive cholangitis was caused by the relatively wide anastomosis opening into the duodenal bulb, where the stomach contents pass through the most, and the poor clearance owing to the convoluted cystic duct;therefore, we chose to place a stent endoscopically. However, to our surprise, Class V was detected in the bile cytology performed as a precaution. Although no tumor was seen on imaging such as contrast-enhanced CT, EUS, and PET/CT, mapping biopsy results showed the presence of cancer at the bifurcation of the cystic duct. The patient had cholangiocarcinoma confined to the extrahepatic bile ducts only;thus, extrahepatic bile duct resection was conducted. The patient was discovered to have biliary intraepithelial neoplasia-3, and the tumor was entirely respectable. The patient had a good postoperative course, with normalization of liver function and no recurrence of cholangitis. In this case, cholangiocarcinoma was detected at an early stage by cytological examination performed as a precaution during endoscopic therapy for recurrent cholangitis. In addition to the fact that the long-term pathogenesis of biliary atresia is still unknown, it is important to note the presence of malignancy, which has the greatest effect on the patient prognosis, considering that the course of the disease varies depending on the operation carried out. Because cholecystoduodenostomy for biliary atresia is a rare approach, and there has been no previous report of related cholangiocarcinoma, we report this case for the benefit of gastroenterologists who may encounter similar cases in the future.


Assuntos
Neoplasias dos Ductos Biliares , Atresia Biliar , Colangiocarcinoma , Colangite , Cistos , Anastomose Cirúrgica/efeitos adversos , Neoplasias dos Ductos Biliares/diagnóstico por imagem , Neoplasias dos Ductos Biliares/cirurgia , Ductos Biliares Intra-Hepáticos/patologia , Atresia Biliar/complicações , Atresia Biliar/patologia , Atresia Biliar/cirurgia , Colangiocarcinoma/complicações , Colangiocarcinoma/diagnóstico por imagem , Colangiocarcinoma/cirurgia , Colangite/etiologia , Cistos/complicações , Cistos/patologia , Feminino , Humanos , Pessoa de Meia-Idade , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada/efeitos adversos
14.
Ophthalmic Plast Reconstr Surg ; 38(4): e112-e113, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35797674

RESUMO

Apocrine hidrocystomas are benign cystic tumors derived from apocrine sweat glands; they are most commonly located in the skin of the head and neck regions. Ophthalmic occurrences typically appear at the lash line and canthi of the eyelid, although rare instances have been described in the conjunctiva, caruncle, and orbit. The authors illustrate an exceptional instance of a mobile episcleral cyst in a 12-year-old girl that developed about 2 years following strabismus surgery. The cyst was located anterior to the insertion of the left medial rectus and was excised in response to the patient's complaints of irritation. Histopathology of the excised specimen displayed an empty cyst lined by a double layer of cuboidal epithelium with the inner layer exhibiting apical decapitation secretion. Confirmatory immunohistochemistry demonstrated reactivity of both layers with CK7 and the outer myoepithelial layer with D2-40. Postoperative and traumatic cysts formed after interruption of the bulbar conjunctiva are usually conjunctival cysts lined by stratified squamous nonkeratinizing epithelium.


Assuntos
Cistos , Hidrocistoma , Estrabismo , Neoplasias das Glândulas Sudoríparas , Glândulas Apócrinas/patologia , Criança , Cistos/diagnóstico , Cistos/etiologia , Cistos/patologia , Feminino , Hidrocistoma/diagnóstico , Hidrocistoma/patologia , Humanos , Estrabismo/patologia , Neoplasias das Glândulas Sudoríparas/diagnóstico , Neoplasias das Glândulas Sudoríparas/patologia , Neoplasias das Glândulas Sudoríparas/cirurgia
15.
Sci Rep ; 12(1): 12865, 2022 07 27.
Artigo em Inglês | MEDLINE | ID: mdl-35896562

RESUMO

To evaluate clinicopathological features and survival outcomes of unilocular cystic renal cell carcinoma (ucRCC) compared with purely solid renal cell carcinoma (sRCC), and to evaluate the oncologic aggressiveness of ucRCC. The relevant data of 957 patients with sporadic unilateral renal cell carcinoma (RCC) underwent surgical treatment in 2 institutions from Jan 2014 to Oct 2018 were obtained. We excluded multilocular cystic renal neoplasm of low malignant potential (MCRNLMP), RCC with multilocular cysts and necrotic RCC. 74 ucRCCs were identified by pathology reports. We performed propensity score matching (PSM) and eventually selected 144 sRCCs. The clinicopathological features and survival outcomes were compared properly. After PSM, age, BMI, Charlson Comorbidity Index, and postoperative Chronic Kidney Disease grade were not significantly different. Both overall survival and progression-free survival of ucRCC were significantly better than sRCC by the log-rank test. Twenty-five cases of sRCCs were in the pT3 or pT4 stage, while no pT3 or pT4 tumors were found in ucRCCs. Fuhrman grade and lymphatic metastasis were found to be significant prognostic factors for the overall survival of ucRCC. Unilocular cystic RCC has a lower Fuhrman grade and pathological stage and a better prognosis compared with solid RCC. Patients with ucRCC still probably have lymphatic metastasis at surgery and may have postoperative metastasis, which is different from MCRNLMP. We recommend that the diagnosis of ucRCC should be reflected in pathology report. Different subtype of cystic RCC should be taken into consideration in counseling and management.


Assuntos
Carcinoma de Células Renais/mortalidade , Cistos/mortalidade , Neoplasias Renais/mortalidade , Carcinoma de Células Renais/patologia , Cistos/patologia , Humanos , Neoplasias Renais/patologia , Metástase Linfática , Prognóstico , Estudos Retrospectivos
16.
Cells ; 11(15)2022 07 22.
Artigo em Inglês | MEDLINE | ID: mdl-35892566

RESUMO

(1) Background: Autosomal dominant polycystic kidney disease (ADPKD) is a frequent monogenic disorder that leads to progressive renal cyst growth and renal failure. Strategies to inhibit cyst growth in non-human cyst models have often failed in clinical trials. There is a significant need for models that enable studies of human cyst growth and drug trials. (2) Methods: Renal tissue from ADPKD patients who received a nephrectomy as well as adult mouse kidney slices were cultured on a chorioallantoic membrane (CAM) for one week. The cyst volume was monitored by microscopic and CT-based applications. The weight and angiogenesis were quantified. Morphometric and histological analyses were performed after the removal of the tissues from the CAM. (3) Results: The mouse and human renal tissue mostly remained vital for about one week on the CAM. The growth of cystic tissue was evaluated using microscopic and CT-based volume measurements, which correlated with weight and an increase in angiogenesis, and was accompanied by cyst cell proliferation. (4) Conclusions: The CAM model might bridge the gap between animal studies and clinical trials of human cyst growth, and provide a drug-testing platform for the inhibition of cyst enlargement. Real-time analyses of mouse kidney tissue may provide insights into renal physiology and reduce the need for animal experiments.


Assuntos
Cistos , Rim Policístico Autossômico Dominante , Adulto , Animais , Proliferação de Células , Cistos/patologia , Humanos , Rim/patologia , Camundongos
17.
Pediatr Dev Pathol ; 25(5): 558-561, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35658744

RESUMO

Angiomyxoma of the umbilical cord is a rare benign vascular malformation with potential for fetal morbidity and mortality. Gross and histologic features of this lesion are identical to those described as "hemangioma," however "angiomyxoma" is a preferable term as current practice restricts the term "hemangioma" to infantile capillary proliferations that express glucose transporter 1. Here we describe the case of an umbilical cord angiomyxoma with associated pseudocysts diagnosed after delivery at 33 weeks. It presented as a heterogeneous-appearing mass near the fetal cord insertion and mimicked serious fetal anomalies throughout gestation. We found fetal MRI helpful for monitoring this lesion, narrowing the differential, and informing management. Proximity to the fetal end of the cord and uncertainty about diagnosis also required surgical resection of the mass after delivery with umbilicoplasty.


Assuntos
Cistos , Hemangioma , Cistos/patologia , Feminino , Transportador de Glucose Tipo 1 , Hemangioma/patologia , Humanos , Gravidez , Ultrassonografia Pré-Natal , Cordão Umbilical/patologia
19.
J. coloproctol. (Rio J., Impr.) ; 42(2): 190-192, Apr.-June 2022. ilus
Artigo em Inglês | LILACS | ID: biblio-1394415

RESUMO

Retrorectal hamartoma, also called tailgut cyst, is a congenital lesion resulting from the non-regression of embryo remnants of the hindgut. We describe in this work the case report of a 68-year-old man, previously healthy, complaining of rectal bulging for 4 months, which was diagnosed by nuclear magnetic resonance imaging as a multi-loculated and mucinous lesion, suggestive of tailgut cyst. In view of the finding, the lesion was surgically resected, due to the potential for future complications, and, through the anatomopathological analysis, there were no findings of malignancy in the specimen. (AU)


Assuntos
Humanos , Masculino , Idoso , Cistos/cirurgia , Hamartoma/diagnóstico por imagem , Cistos/patologia , Cistos/diagnóstico por imagem
20.
Abdom Radiol (NY) ; 47(7): 2356-2370, 2022 07.
Artigo em Inglês | MEDLINE | ID: mdl-35670875

RESUMO

Fibropolycystic liver diseases (FLDs) make up a rare spectrum of heritable hepatobiliary diseases resulting from congenital ductal plate malformations (DPMs) due to the dysfunction of proteins expressed on the primary cilia of cholangiocytes. The embryonic development of the ductal plate is key to understanding this spectrum of diseases. In particular, DPMs can result in various degrees of intrahepatic duct involvement and a wide spectrum of cholangiopathies, including congenital hepatic fibrosis, Caroli disease, polycystic liver disease, and Von Meyenberg complexes. The most common clinical manifestations of FLDs are portal hypertension, cholestasis, cholangitis, and (in rare cases) cholangiocarcinoma. This article reviews recent updates in the pathophysiology, imaging, and clinical management of FLDs.


Assuntos
Neoplasias dos Ductos Biliares , Cistos , Hipertensão Portal , Neoplasias dos Ductos Biliares/patologia , Ductos Biliares Intra-Hepáticos/patologia , Cistos/diagnóstico por imagem , Cistos/patologia , Humanos , Hipertensão Portal/patologia , Fígado/patologia , Cirrose Hepática/patologia , Hepatopatias
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