Tumor de pequenas células redondas desmoplásico intra-abdominal: relato de caso

Os autores relatam um caso de tumor de pequenas células redondas desmoplásico intra-abdominal acometendo paciente do sexo masculino, de 21 anos de idade, atendido com quadro de dor abdominal, trombose do membro inferior direito e perda da função renal, de causa obstrutiva. A investigação demonstrou volumosa lesão abdominopélvica, sólida, bocelada, com áreas císticas internas, situada posteriormente à bexiga, causando obstrução ureteral, compressão da veia ilíaca direita e oclusão parcial do reto, além de acometimento de linfonodos intra e retroperitoneais. São descritos os achados cirúrgicos, de ultra-sonografia, tomografia computadorizada e ressonância magnética, bem como aqueles do estudo macroscópico, microscopia e imuno-histoquímica.

Bilateral ptosis: Lesion in the oculomotor nuclei or supranuclear lesion?

BACKGROUND: Bilateral ptosis is a very interesting clinical challenge for doctors because of the multiple possible localizations of a lesion which can lead to this neurological sign. OBJECTIVES: Through this case report, we aim to determine the difference between an apraxia of lid opening (ALO) with difficulty in initiating the act of lid elevation, in spite of adequate understanding, motor control and cranial nerve pathways, and a bilateral ptosis with a lesion in the oculomotor nucleus or blepharospasm. METHODS: The case report of a 50-year-old patient presenting bilateral ptosis and multiple ischemic lesions in the brainstem and bilateral frontal lobe lesions after the emergency removal of a large frontal tumor. RESULTS: Our patient had an ALO according to the neurological follow-up and showed the ability, after a few weeks, of initiating the act of opening her eyes with her hand. The ophthalmic evaluation confirmed that in her case the ALO was associated with a nuclear lesion of the oculomotor nerve secondary to a midbrain lesion. CONCLUSION: Our case report confirms multiple differential diagnoses in bilateral ptosis and the importance of clinical examination in spite of good neurological imaging.

Tumores ósseos raros da calota craniana: sarcoma de Ewing e tumor de Triton

O sarcoma de Ewing e o tumor de Triton representam dois tumores ósseos raros da calota craniana, os quais possuem clínica e aspecto imagenológico inespecíficos. Entretanto, os métodos de imagem são importantes para a detecção das lesões durante a investigação diagnóstica, para a avaliação da extensão das lesões ósseas, do possível acometimento de partes moles e do parênquima encefálico, e avaliação de recorrência local e/ou metástases a distância. A confirmação diagnóstica é determinada pela histologia e auxiliada pela imuno-histoquímica. Os autores relatam dois casos desses tumores e seus achados pelos métodos de imagem.

Fluoroscopic-Guided Radial Endobronchial Ultrasound Without Guide Sheath For Peripheral Pulmonary Lesions: A Safe And Efficient Combination.

BACKGROUND: Several guidelines recommend computed tomography scans for populations with high-risk for lung cancer. The number of individuals evaluated for peripheral pulmonary lesions (PPL) will probably increase, and with it non-surgical biopsies. Associating a guidance method with a target confirmation technique has been shown to achieve the highest diagnostic yield, but the utility of bronchoscopy with radial probe endobronchial ultrasound using fluoroscopy as guidance without a guide sheath has not been reported. METHODS: We conducted a retrospective analysis of bronchoscopy with radial probe endobronchial ultrasound using fluoroscopy procedures for the investigation of PPL performed by experienced bronchoscopists with no specific previous training in this particular technique. Operator learning curves and radiological predictors were assessed for all consecutive patients examined during the first year of application of the technique. RESULTS: Fifty-one PPL were investigated. Diagnostic yield and visualization yield were 72.5 and 82.3% respectively. The diagnostic yield was 64.0% for PPL ≤20mm, and 80.8% for PPL>20mm. No false-positive results were recorded. The learning curve of all diagnostic tools showed a DY of 72.7% for the first sub-group of patients, 81.8% for the second, 72.7% for the third, and 81.8% for the last. CONCLUSION: Bronchoscopy with radial probe endobronchial ultrasound using fluoroscopy as guidance is safe and simple to perform, even without specific prior training, and diagnostic yield is high for PPL>and ≤20mm. Based on these findings, this method could be introduced as a first-line procedure for the investigation of PPL, particularly in centers with limited resources.

Persistence of tracer in the application site -a potential confounding factor in nerve regeneration studies

Selective reinnervation of peripheral targets after nerve injury might be assessed by injecting a first tracer in a target before nerve injury to label the original neuronal population, and applying a second tracer after the regeneration period to label the regenerated population. However, altered uptake of tracer, fading, and cell death may interfere with the results. Furthermore, if the first tracer injected remains in the target tissue, available for 're-uptake' by misdirected regenerating axons, which originally innervated another region, then the identification of the original population would be confused. With the aim of studying this problem, the sciatic nerve of adult rats was sectioned and sutured. After 3 days, to allow the distal axon to degenerate avoiding immediate retrograde transport, one of the dyes: Fast Blue (FB), Fluoro-Gold (FG) or Diamidino Yellow (DY), was injected into the tibial branch of the sciatic nerve, or in the skin of one of the denervated digits. Rats survived 2-3 months. The results showed labelled dorsal root ganglion (DRG) cells and motoneurones, indicating that late re-uptake of a first tracer occurs. This phenomenon must be considered when the model of sequential labelling is used for studying the accuracy of peripheral reinnervation.

Collagen (NeuraGen®) nerve conduits and stem cells for peripheral nerve gap repair

Le système nerveux périphérique est responsable de la transmission des impulses motrices, ainsi que de la réception des afférences sensorielles. Les lésions traumatiques des nerfs périphériques conduisent à une impotence fonctionnelle qui peut être dévastant, notamment chez les travailleurs manuels,. La récupération fonctionnelle est donc le but principal dans chirurgie des nerfs périphériques. Malheureusement, une suture directe des moignons nerveux est souvent impossible dans le contexte des traumatismes complexes qui surviennent lors des accidents. La suture nerveuse par interposition d'autogreffe reste le gold standard dans la pratique chirurgicale mais nécessite le sacrifice d'un nerf donneur, avec dysesthésie et possibles douleurs neuropathiques conséquentes. Alternativement, des guides tubulaires pour les nerfs peuvent être utilisées si le gap nerveux est inférieur à 3 cm. Plusieurs guides résorbables en collagène sont approuve en Europe et aux Etas Unis (FDA). Dans cette étude, des conduits de collagène ont été associe a des cellules régénératives (cellules souches adultes) comme stratégie supplémentaire de régénération. Une fois testé le rapport des cellules avec le biomatériau (NeuraGen® nerve guides) in vitro, une étude in vivo dans le rat a été effectuée. Les différents groupes de conduits ont été supplémentés respectivement avec Schwann cells (SC); avec cellules souches adultes dérivées de la moelle épinière, différentiées en cellules "Schwann-like" (dMSC); avec cellules souches adultes dérivées de la graisse, différentiées en cellules "Schwann-like" (dASC). Un groupe de conduits avec du milieu de culture sans cellules a été utilisé comme group control. Les conduits ont été utilisés pour combler un gap de 1cm dans un model de section totale du nerf sciatique chez le rat. Deux semaines post implantation, une analyse immuno-histochimique a été effectuée pour évaluer la régénération axonales et l'infiltration de cellules de Schwann au niveau du conduit. Les cellules ont montré une adhérence efficace aux parois de collagène. En particulier, les cellules de Schwann ont montré une amélioration significative au niveau du sprouting distale. Par contre, aucune différence significative n'a été remarquée entre les groupes pour le sprouting axonale proximal. De plus, si les cellules souches ont montré un pattern de sprouting diffus, les cellules de Schwann ont par contre garanti un cône de croissance typique, associé a une affinité remarquable pour les parois de collagène. NeuraGen® guides pourraient donc être un moyen adapté a l'association avec la thérapie cellulaire en raison de la bonne adhérence des cellules au biomatériau. Des modifications de surface dans le but d'améliorer la performance neurotrophique cellulaire in vivo (e.g. peptides de matrice extracellulaire) pourront être utilisées dans des applications futures.

Acute pulmonary hypertension caused by tumor embolism: a report of two cases.

Acute pulmonary hypertension leading to right ventricular failure and circulatory collapse is usually caused by thromboembolic obstruction of the pulmonary circulation. However, in rare instances, other causes can be associated with a similar clinical presentation. We present and discuss the clinical histories of two patients with acute right ventricular failure due to an atypical cause of pulmonary hypertension, disseminated pulmonary tumor embolism.

Programmed death ligand 1 expression and tumor-infiltrating lymphocytes in glioblastoma.

BACKGROUND: Immune checkpoint inhibitors targeting programmed cell death 1 (PD1) or its ligand (PD-L1) showed activity in several cancer types. METHODS: We performed immunohistochemistry for CD3, CD8, CD20, HLA-DR, phosphatase and tensin homolog (PTEN), PD-1, and PD-L1 and pyrosequencing for assessment of the O6-methylguanine-methyltransferase (MGMT) promoter methylation status in 135 glioblastoma specimens (117 initial resection, 18 first local recurrence). PD-L1 gene expression was analyzed in 446 cases from The Cancer Genome Atlas. RESULTS: Diffuse/fibrillary PD-L1 expression of variable extent, with or without interspersed epithelioid tumor cells with membranous PD-L1 expression, was observed in 103 of 117 (88.0%) newly diagnosed and 13 of 18 (72.2%) recurrent glioblastoma specimens. Sparse-to-moderate density of tumor-infiltrating lymphocytes (TILs) was found in 85 of 117 (72.6%) specimens (CD3+ 78/117, 66.7%; CD8+ 52/117, 44.4%; CD20+ 27/117, 23.1%; PD1+ 34/117, 29.1%). PD1+ TIL density correlated positively with CD3+ (P < .001), CD8+ (P < .001), CD20+ TIL density (P < .001), and PTEN expression (P = .035). Enrichment of specimens with low PD-L1 gene expression levels was observed in the proneural and G-CIMP glioblastoma subtypes and in specimens with high PD-L1 gene expression in the mesenchymal subtype (P = 5.966e-10). No significant differences in PD-L1 expression or TIL density between initial and recurrent glioblastoma specimens or correlation of PD-L1 expression or TIL density with patient age or outcome were evident. CONCLUSION: TILs and PD-L1 expression are detectable in the majority of glioblastoma samples but are not related to outcome. Because the target is present, a clinical study with specific immune checkpoint inhibitors seems to be warranted in glioblastoma.

Comparative study of two needle models in terms of deflection during inferior alveolar nerve block

Objectives: The purpose of this study is to determine the possible differences in deflection between two needles of same length and external gauge but with different internal gauges during truncal block of the inferior alveolar nerve. The initial working hypothesis was that greater deflection may be expected with larger internal gauge needles. Study design: Four clinicians subjected 346 patients to inferior alveolar nerve block and infiltrating anesthesia of the buccal nerve trajectory for the surgical or conventional extraction of the lower third molar. A nonautoaspirating syringe system with 2 types of needle was used: a standard 27-gauge x 35-mm needle with an internal gauge of 0.215 mm or an XL Monoprotect® 27-gauge x 35-mm needle with an internal gauge of 0.265 mm. The following information was systematically recorded for each patient: needle type, gender, anesthetic technique (direct or indirect truncal block) and the number of bone contacts during the procedure, the patient-extraction side, the practitioner performing the technique, and blood aspiration (either positive or negative). Results: 346 needles were used in total. 190 were standard needles (27-gauge x 35-mm needle with an internal gauge of 0.215 mm) and 156 were XL Monoprotect®. Incidence of deflection was observed in 49.1% of cases (170 needles) where 94 were standard needles and 76 XL Monoprotect®. Needle torsion ranged from 0º and 6º. Conclusions: No significant differences were recorded in terms of deflection and internal gauge, operator, patient-extraction side, the anesthetic technique involved and the number of bone contacts during the procedure

Tumor de Wilms extra-renal: relato de caso

Um caso de tumor de Wilms extra-renal de localização retroperitoneal em uma paciente do sexo feminino de dois anos de idade é apresentado, associado a revisão de literatura. Foram realizados exames de radiografia simples do abdome, urografia excretora, ultra-sonografia e tomografia computadorizada sem e com contraste, que evidenciaram a presença de massa retroperitoneal adjacente ao rim direito. A paciente foi submetida a intervenção cirúrgica, com ressecção de toda a massa, sendo o diagnóstico de tumor de Wilms confirmado com exame anatomopatológico. O tumor de Wilms extra-renal é uma entidade extremamente rara e maligna, descrito na literatura principalmente sob a forma de relato de caso. Pode ocorrer no retroperitônio, útero e ovários, canal inguinal, testículos, pele, e até mesmo no tórax. O mecanismo exato que poderia explicar a ocorrência deste tumor em tecido extra-renal não foi bem estabelecido ainda. O diagnóstico é feito através do estudo anatomopatológico da lesão, geralmente após intervenção cirúrgica.

Tumor neuroectodérmico melanocítico da infância (progonoma): relato de caso enfatizando os aspectos tomográficos e revisão da literatura

O tumor neuroectodérmico melanocítico da infância, também conhecido como progonoma, é uma enfermidade rara, benigna, originária da crista neural e que aparece no primeiro ano de vida, acometendo preferencialmente a maxila. Os autores relatam um caso raro deste tumor na maxila de uma criança de dez meses de idade, dando ênfase aos aspectos diagnósticos na tomografia computadorizada, e fazem uma revisão da literatura.

Perivascular epithelioid cell tumor (PEComa) of the uterus: A case report

Background. Perivascular epithelioid cell tumors (PEComas) are a rare family of mesenchymal tumors arising in a wide array of anatomic locations and characterized by coexpression of melanocytic and muscle markers. The uterus accounts for around one-fourth of the overall PEComa cases reported in the literature. Methods. We report a case of PEComa of the uterus with multiple malignancy features. Results. A uterine mass suspect for leiomyosarcoma was found in a 53-year-old woman with post-menopausal bleeding. Total hysterectomy and bilateral adnexectomy was performed. The tumor measured 7 cm in diameter, was unique, well-circumscribed, nodular, and whiteyellow without haemorrhage or necrosis. Microscopically, two populations of cells could be seen: small fusiform cells growing in fascicles resembling a smooth muscle tumor, and large epithelioid cells with abundant pale vacuolated cytoplasm growing in a diffuse pattern. Cytologic atypias were marked and mitoses numerous and often atypical in the second component. The tumor infiltrated into the myometrium with lymphovascular invasion. Immunostains showed positivity for MelanA, HMB45, smooth muscle actin, CD10, TFE3 and cathepsin K. Conclusions. This PEComa case presents several of the recently precised criteria for malignancy (Schoolmeester JK et al. Perivascular epithelioid cell neoplasm (PEComa) of the gynecologic tract: Clinicopathologic and immunohistochemical characterization of 16 cases. Am J Surg Pathol 2014; 38:176-188).

Tumor fibroso solitário do rim: descrição de caso

Apresentamos um caso de tumor renal num paciente de 27 anos de idade, que foi submetido a estudo de imagem por tomografia computadorizada e ressonância magnética. As características radiológicas da massa induziam para um diagnóstico de tumor renal maligno de origem intraparanquimatosa. No entanto, o estudo histopatológico revelou tumor fibroso solitário benigno originário da pelve e porção calicial do rim direito.

Direct tumor recognition by a human CD4(+) T-cell subset potently mediates tumor growth inhibition and orchestrates anti-tumor immune responses.

Tumor antigen-specific CD4(+) T cells generally orchestrate and regulate immune cells to provide immune surveillance against malignancy. However, activation of antigen-specific CD4(+) T cells is restricted at local tumor sites where antigen-presenting cells (APCs) are frequently dysfunctional, which can cause rapid exhaustion of anti-tumor immune responses. Herein, we characterize anti-tumor effects of a unique human CD4(+) helper T-cell subset that directly recognizes the cytoplasmic tumor antigen, NY-ESO-1, presented by MHC class II on cancer cells. Upon direct recognition of cancer cells, tumor-recognizing CD4(+) T cells (TR-CD4) potently induced IFN-γ-dependent growth arrest in cancer cells. In addition, direct recognition of cancer cells triggers TR-CD4 to provide help to NY-ESO-1-specific CD8(+) T cells by enhancing cytotoxic activity, and improving viability and proliferation in the absence of APCs. Notably, the TR-CD4 either alone or in collaboration with CD8(+) T cells significantly inhibited tumor growth in vivo in a xenograft model. Finally, retroviral gene-engineering with T cell receptor (TCR) derived from TR-CD4 produced large numbers of functional TR-CD4. These observations provide mechanistic insights into the role of TR-CD4 in tumor immunity, and suggest that approaches to utilize TR-CD4 will augment anti-tumor immune responses for durable therapeutic efficacy in cancer patients.