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BACKGROUND AND OBJECTIVES: This multicenter study aimed to assess the safety and efficacy of the Woven EndoBridge (WEB) device for treating unruptured wide-neck intracranial bifurcation aneurysms (WIBAs) with short-, mid-, and long-term follow-ups (FUPs). METHODS: Consecutive patients with unruptured WIBAs treated with WEB between December 2014 and January 2018 were included. Patient, aneurysm, and device characteristics were collected and analyzed retrospectively. Morbidity and mortality rates were determined by collecting intraprocedural, periprocedural, and delayed complications. Aneurysm occlusion was assessed at 1, 3, and 5 years using a 3-grade scale: complete occlusion, neck remnant, and residual aneurysm. Complete occlusion and neck remnant were considered as adequate occlusion. Patients who received re-treatment were also evaluated. RESULTS: The study included 104 consecutive patients (55.8% female, mean age 58.6 ± 11.8 years). Aneurysm maximum size, neck, and dome-to-neck mean were, respectively, 6.9 ± 2.1 mm, 4.5 ± 1.2 mm, and 1.4 ± 0.3 mm. One-year FUP was collected for 95 patients, and 3- and 5-year FUPs were collected for 83 patients. Adequate occlusion was observed at 1-year FUP in 90.5% (86/95), 91.6% (76/83) was observed at 3-year FUP, and 92.8% (77/83) at 5-year FUP. None of the aneurysms bled after treatment. During FUP, 6/83 patients (7.2%) were re-treated for residual aneurysm. Morbidity and mortality rates closely related to aneurysm occlusion were 0% (0/104). CONCLUSION: The WEB device was safe and effective for treating unruptured WIBAs, both in short-term and long-term FUPs.
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PURPOSE Renal artery aneurysms (RAAs) are rare in the general population, although the true incidence and natural history remain elusive. Conventional endovascular therapies such as coil embolization or covered stent graft may cause sidebranches occlusion, leading to organ infarction. Flow-diverters (FD) have been firstly designed to treat cerebrovascular aneurysms, but their use may be useful to treat complex RAAs presenting sidebraches arising from aneurysmal sac. To evaluate mid-term follow-up (FUP) safety and efficacy of FD during treatment of complex RAAs. METHODS Between November 2019 and April 2020, 7 RAAs were identified in 7 patients (4 men, 3 women; age range 55-82 years; median 67 years) and treated by FD. Procedural details, complications, morbidity and mortality, aneurysm occlusion and segmental artery patency were retrospectively reviewed. Twelve months computed tomography angiography (CTA) FUP was evaluated for all cases. RESULT Deployment of FD was successful in all cases. One intraprocedural technical complication was encountered with one FD felt down into aneurism sac which requiring additional telescopic stenting. One case at 3 months CTA FUP presented same complication, requiring same rescue technique. At 12 months CTA FUP 5 cases of size shrinkage and 2 cases of stable size were documented. No rescue surgery or major intraprocedural or mid-term FUP complication was seen. CONCLUSION Complex RAAs with two or more sidebranches can be safely treated by FD. FD efficacy for RAA needs a further validation at long term FUP by additional large prospective studies.
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Aneurisma , Embolización Terapéutica , Procedimientos Endovasculares , Aneurisma Intracraneal , Masculino , Humanos , Femenino , Persona de Mediana Edad , Anciano , Anciano de 80 o más Años , Estudios de Seguimiento , Estudios Retrospectivos , Arteria Renal/diagnóstico por imagen , Estudios Prospectivos , Resultado del Tratamiento , Aneurisma/diagnóstico por imagen , Aneurisma/cirugía , Stents , Embolización Terapéutica/métodos , Procedimientos Endovasculares/métodos , Aneurisma Intracraneal/cirugíaRESUMEN
Vascular anomalies of the pediatric orbit represent a heterogeneous group that include both vascular tumors and vascular malformations. The disorder may initially be silent and then associated with symptoms and/or function damage, depending on the type of vascular anomaly and its extension. Vascular tumors include benign, locally aggressive (or borderline) and malignant forms while vascular malformations are divided into "simple", "combined" and syndromic, or "low flow" or "high flow". Both entities can arise in isolation or as part of syndromes. In this review, we describe the imaging findings of the vascular lesions of the orbit in the pediatric population, which are key to obtain a correct diagnosis and to guide the appropriate treatment in the light of the new genetic and molecular discoveries, and the role of the radiologist in their multidisciplinary management. We will also touch upon the main syndromes associated with orbital vascular abnormalities.
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Background: Optic pathway gliomas (OPGs) are rare neoplasms in children with an unpredictable clinical course. Approximately 15% of OPGs occur in patients affected by neurofibromatosis type 1 (NF1): the clinical course of these cases is more indolently than sporadic ones, and NF1 patients less frequently require treatment including surgery. Instead, over 90% of sporadic OPGs require one or more therapeutic approaches. The management of OPG is controversial. They are also characterized by a high risk of morbidity including hypothalamic damage, endocrine deficits, visual deficit and/or neurological impairment. Materials and Methods: In this paper, we evaluated visual and endocrinological outcomes of a population of OPG followed at our center from 2013 to 2021, with a particular emphasis on the role of surgery. Results: Twenty-six patients were included in this study (mean age of 40.7 months). Tumor location on imaging was described by the Dodge classification. Five cases had NF 1. Thirteen cases received biopsy and 13 were partially resected. Histopathology revealed 19 cases of pilocytic astrocytomas, 2 pilomyxoid astrocytoma and 5 ganglioglioma. All the patients required a post-surgical adjuvant treatment according to current indications for low-grade gliomas. Molecular studies (BRAF status and mTOR/pmTOR pathway) have been performed in 24/26 patients, following for the use of target therapy in 11 of these patients. In our study we found that patients underwent biopsy have a better visual and endocrinological outcomes rather than patients with a tumor debulking. The five-year overall survival rate is 98% with a mean follow-up of 60 months. Conclusions: Many children with OPGs survive with a residual tumor. They suffer from chronic diseases such as endocrine dysfunction, visual disturbance, motor deficits and poor quality of life. All patients need comprehensive diagnostic work-up including neuroimaging, clinical evaluations and neuropathology approach; at the same time, they need therapeutic decisions and concepts for the choice of timing and type of neurosurgical intervention, chemotherapy and target therapy as well as surveillance and rehabilitation to maximize survival and overall functional outcomes. Our study showed that minimal invasive surgery with the purpose of molecular characterization of the tumor is desirable to reduce morbidity correlate to surgery.
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Pediatric spinal tumors are rare and account for 10% of all central nervous system tumors in children. Onset usually occurs with chronic nonspecific symptoms and may depend on the intra- or extradural neoplastic location. Meningiomas, schwannomas, and neurofibromas are the most common intradural-extramedullary lesions, while astrocytomas and ependymomas represent the majority of intramedullary tumors. The new molecular discoveries regarding pediatric spinal cancer currently contribute to the diagnostic and therapeutic processes. Moreover, some familial genetic syndromes can be associated with the development of spinal tumors. Currently, magnetic resonance imaging (MRI) is the standard reference for the evaluation of pediatric spinal tumors. Our aim in this review was to describe the imaging of the most frequent intradural intra/extramedullary pediatric spinal tumors and to investigate the latest molecular findings and genetic syndromes.
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Von Hippel-Lindau (VHL) disease is a heritable cancer syndrome in which benign and malignant tumors and/or cysts develop throughout the central nervous system (CNS) and visceral organs. The disease results from mutations in the VHL tumor suppressor gene located on chromosome 3 (3p25-26). A majority of individuals (60-80%) with VHL disease will develop CNS hemangioblastomas (HMG). Endolymphatic sac tumor (ELST) is an uncommon, locally aggressive tumor located in the medial and posterior petrosal bone region. Its diagnosis is based on clinical, radiological, and pathological correlation, and it can occur in the setting of VHL in up to 10-15% of individuals. We describe a 17-year-old male who presented with a chief complaint of hearing loss. Brain and spine Magnetic Resonance Imaging documented the presence of an expansive lesion in the left cerebellar hemisphere, compatible with HMG in association with a second cerebellopontine lesion compatible with ELST. The peculiarity of the reported case is due to the simultaneous presence of two typical characteristics of VHL, which led to performing comprehensive genetic testing, thus allowing for the diagnosis of VHL. Furthermore, ELST is rare before the fourth decade of life. Early detection of these tumors plays a key role in the optimal management of this condition.
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Rosette-forming glioneuronal tumors (RGNTs) are rare, grade I, central nervous system (CNS) tumors typically localized to the fourth ventricle. We describe a 9-year-old girl with dizziness and occipital headache. A magnetic resonance imaging (MRI) revealed a large hypodense posterior fossa mass lesion in relation to the vermis, with cystic component. Surgical resection of the tumor was performed. A RGNT diagnosis was made at the histopathological examination. During follow-up, the patient experienced a first relapse, which was again surgically removed. Eight months after, MRI documented a second recurrence at the local level. She was a candidate for the proton beam therapy (PBT) program. Three years after the end of PBT, the patient had no evidence of disease recurrence. This report underlines that, although RGNTs are commonly associated with an indolent course, they may have the potential for aggressive behavior, suggesting the need for treatment in addition to surgery. Controversy exists in the literature regarding effective management of RGNTs. Chemotherapy and radiation are used as adjuvant therapy, but their efficacy management has not been adequately described in the literature. This is the first case report published in which PBT was proposed for adjuvant therapy in place of chemotherapy in RGNT relapse.
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The spine, a frequently investigated site in children, has a complex development in relation to both nervous and bone/cartilaginous structures and shows several particular features in children compared with adults. We report the main normal variants and pathologies of the pediatric spine, from the prenatal period to adolescence, focusing on a multimodality imaging approach.
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Columna Vertebral , Adolescente , Adulto , Niño , Humanos , Columna Vertebral/diagnóstico por imagenRESUMEN
PURPOSE: Evaluation of orbital proptosis and sutural synostosis pattern along the coronal ring in craniofaciosynostosis patients with or without fibroblastic growth factor receptor 2 (FGFR2) mutation. METHODS: High-resolution computer tomography was used to assess, in children with or without FGFR2 mutation, the early synostotic involvement of the "major" and "minor" sutures/synchondroses of the coronal arch along with the following orbital parameters: interorbital angle, bone orbital cavity volume, globe volume, ventral globe volume, ventral globe index. RESULTS: Infants with FGFR2 mutation showed an increased number of closed minor sutures/synchondroses along the posterior coronal branch while both groups showed a comparable synostotic involvement of the minor sutures of the anterior coronal branch. FGFR2 infants with posterior coronal branch synostotic involvement showed a higher degree of proptosis due to both reduced bony cavity volume and increased globe volume (p<0.05). CONCLUSIONS: Our data show that FGFR2 mutation together with posterior coronal branch synostotic involvement has a synergic effect in causing a more severe degree of orbital proptosis.
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Craneosinostosis , Exoftalmia , Estudios de Casos y Controles , Niño , Suturas Craneales , Craneosinostosis/complicaciones , Craneosinostosis/diagnóstico por imagen , Craneosinostosis/genética , Exoftalmia/diagnóstico por imagen , Exoftalmia/etiología , Humanos , Lactante , Estudios Retrospectivos , Tomografía Computarizada por Rayos XRESUMEN
We present a case demonstrating the performance of different radiographical imaging modalities in the diagnostic work-up of a patient with neurofibromatosis type 1 (NF1) and plexiform neurofibroma (PN). The newborn boy showed an expansive-infiltrative cervical and facial mass presented with macrocrania, craniofacial disfigurement, exophthalmos and glaucoma. A computer tomography (CT) and a magnetic resonance imaging (MRI) were performed. The CT was fundamental to evaluate the bone dysmorphisms and the MRI was crucial to estimate the mass extension. The biopsy of the lesion confirmed the suspicion of PN, thus allowing the diagnosis of NF1. PN is a variant of neurofibromas, a peripheral nerves sheath tumor typically associated with NF1. Even through currently available improved detection techniques, NF1 diagnosis at birth remains a challenge due to a lack of pathognomonic signs; therefore congenital PN are recognized in 20% of cases. This case highlights the importance of using different radiological methods both for the correct diagnosis and the follow-up of the patient with PN. Thanks to MRI evaluation, it was possible to identify earlier the progressive increasing size of the PN and the possible life threatening evolution in order to perform a tracheostomy to avoid airways compression.
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The aim of this study was to identify quantitative tools to classify the severity of trigonocephaly to guide surgical management and predict outcome. METHODS: We reviewed high-resolution computed tomography images of 59 patients with metopic synostosis. We assessed the craniofacial sutural pattern as well as interfrontal and metopic angles, and we related the frontal angulation degree with the sutural pattern, the surgical management, and clinical outcome. RESULTS: We identified 3 groups according to the severity of trigonocephaly. No difference was found between the sutural pattern of nasion complex and severity, whereas the closure of zygomatic maxillary sutures increased with the severity degree (P < 0.05). The operative management was related to the severity degree (P < 0.001) and to the reduced age (P = 0.009). CONCLUSIONS: Interfrontal and metopic angles are complementary measurements to evaluate with high accuracy the degree of frontal angulation. In preoperative assessment, they may guide surgery decision in particular when the choice is not straightforward.
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Suturas Craneales/diagnóstico por imagen , Craneosinostosis/diagnóstico por imagen , Craneosinostosis/cirugía , Tomografía Computarizada por Rayos X , Estudios de Cohortes , Suturas Craneales/patología , Craneosinostosis/clasificación , Craneosinostosis/patología , Femenino , Humanos , Lactante , Masculino , Índice de Severidad de la EnfermedadRESUMEN
PURPOSE: To assess the accuracy of dynamic susceptibility contrast-enhanced perfusion-weighted magnetic resonance imaging in glioma grading and brain tumor characterization of infratentorial tumors, and to investigate differences from supratentorial tumors. METHODS: This retrospective study, approved by the institutional review board, included 246 patients with brain tumors (184 supratentorial, 62 infratentorial), grouped by tumor type: high-grade gliomas (HGG), low-grade gliomas (LGG), metastases (Met), and primary central nervous system lymphoma (PCNSL). Relative cerebral blood volume (rCBV) and mean percentage of signal recovery (PSR) were calculated. For statistical analyses, lesions were grouped by location and histology. Differences were tested with Mann-Whitney U tests. From ROC curves, we calculated accuracy, sensitivity, specificity, PPV, and NPV, for rCBV and PSR. RESULTS: For infratentorial tumors, rCBV was highly accurate in differentiating HGG from LGG (AUC = 0.938). Mean PSR showed high accuracy in differentiating PCNSL and HGG from Met (AUC = 0.978 and AUC = 0.881, respectively). Infratentorial and supratentorial tumors had similarly high rCBV in HGG, high mean PSR in PCNSL, and low mean PSR in Met. The main differences were the optimum threshold rCBV values (3.04 for supratentorial, 1.77 for infratentorial tumors) and the mean PSR, which was significantly higher in LGG than in HGG in supratentorial (p = 0.035), but not infratentorial gliomas. Using infratentorial rCBV threshold values for supratentorial tumors decreased the sensitivity and specificity. CONCLUSION: rCBV and mean PSR were useful in grading and differentiating infratentorial tumors. Proper cutoff values were important in the accuracy of perfusion-weighted imaging in posterior fossa tumors.
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Neoplasias Infratentoriales/diagnóstico por imagen , Neoplasias Supratentoriales/diagnóstico por imagen , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Niño , Medios de Contraste , Imagen de Difusión por Resonancia Magnética , Humanos , Persona de Mediana Edad , Adulto JovenRESUMEN
PURPOSE: Craniofacial dysmorphology varies significantly along a wide spectrum of severity in metopic cranial synostosis (MCS). This study aimed to quantify craniofacial changes, in MCS, to investigate their relationships with the severity of trigonocephaly. METHODS: By combining the metopic ridge and interfrontal angles, we identified three groups of trigonocephaly severity (mild group n.14, moderate group n.19, severe group n.18). We perform a quantitative analysis using high-resolution CT images evaluating (1) cranial fossae dimensions; (2) vault indices and ratios: interparietal/ intercoronal (IPD/ICD), interparietal/intertemporal (IPD/ITD), cephalic index, vertico-longitudinal index; (3) orbito-facial distances (midfacial depth, maxillary height, upper facial index, orbital distances, globe protrusions), maxilla and orbital volumes; (4) supratentorial (ICV) and infratentorial (PCFV) cranial volumes and supratentorial (WBV) and infratentorial (PCFBV) brain volumes. RESULTS: In all groups, middle skull base lengths and upper midface index were increased. In moderate and severe groups: anterior hemifossa lengths were reduced, IPD/ICD and vertico-longitudinal index were changed; midfacial depth, anterior, mild, and lateral interorbital distances were reduced; globe protrusions were increased. The comparison between moderate and severe groups showed an increase of both globe protrusions and IPD/ICD. Among all groups, ICV and WBV were reduced in the severe group. CONCLUSION: This morpho-volumetric study provides new insights in understanding the craniofacial changes occurring in infants at different severity of trigonocephaly. The increase of globe protrusions and the reduction of supratentorial volumes found in the severe group reflect the severity of trigonocephaly; these findings might have a clinical and surgical relevance.
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Craneosinostosis , Craneosinostosis/diagnóstico por imagen , Cara , Humanos , Lactante , Índice de Severidad de la Enfermedad , Cráneo , Tomografía Computarizada por Rayos XRESUMEN
Medulloblastoma is the most common malignant pediatric brain tumor. Medulloblastoma should not be viewed as a single disease, but as a heterogeneous mixture of various subgroups with distinct characteristics. Based on genomic profiles, four distinct molecular subgroups are identified: Wingless (WNT), Sonic Hedgehog (SHH), Group 3 and Group 4. Each of these subgroups are associated with specific genetic aberrations, typical age of onset as well as survival prognosis. Magnetic resonance imaging (MRI) is performed for all patients with brain tumors, and has a key role in the diagnosis, surgical guidance and follow up of patients with medulloblastoma. Several studies indicate MRI as a promising tool for early detection of medulloblastoma subgroups. The early identification of the subgroup can influence the extent of surgical resection, radiotherapy and chemotherapy targeted treatments. In this article, we review the state of the art in MRI-facilitated medulloblastoma subgrouping, with a summary of the main MRI features in medulloblastoma and a brief discussion on molecular characterization of medulloblastoma subgroups. The main focus of the article is MRI features that correlate with medulloblastoma subtypes, as well as features suggestive of molecular subgroups. Finally, we briefly discuss the latest trends in MRI studies and latest developments in molecular characterization.
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BACKGROUND: The role of axillary surgery for early breast carcinoma treated with conservative surgery and radiotherapy is currently the subject of considerable investigation. Recent studies have supported the noninferiority of avoiding axillary surgery in terms of overall survival when sentinel lymph node biopsy (SLNB) presents ≤ 2 positive lymph nodes, thus sparing the patients from complications. There are some ongoing studies investigating the possibility of omitting SLNB. Axillary study seems to be sufficiently replaced by SLNB for staging the disease. Axillary surgery maintains a therapeutic role in the presence of > 2 metastatic lymph nodes at SLNB. PATIENTS AND METHODS: We performed a retrospective analysis of 1156 patients with early breast cancer to estimate the real incidence of patients with T1 tumors presenting > 2 metastatic lymph nodes. RESULTS: Of the 1156 cases, only 106 (9.2%) had > 2 axillary metastatic lymph nodes. More specifically, 38 (4.3%) of 884 T1 cases, and 6 (2.3%) of 257 of T1b cases had > 2 metastatic lymph nodes. CONCLUSION: The advantage of axillary surgery seems to be limited only to a specific subgroup of T1 patients who are undergoing conservative surgery plus radiotherapy. The ongoing studies on avoiding SLNB will likely prove the noninferiority of omitting biopsy because these studies are conducted in the whole population of early breast cancers. It is necessary to identify more accurately the subpopulation of patients who may benefit from axillary surgery.
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Neoplasias de la Mama/cirugía , Tratamiento Conservador/métodos , Escisión del Ganglio Linfático/estadística & datos numéricos , Biopsia del Ganglio Linfático Centinela/estadística & datos numéricos , Ganglio Linfático Centinela/patología , Adulto , Anciano , Anciano de 80 o más Años , Axila , Neoplasias de la Mama/mortalidad , Neoplasias de la Mama/patología , Femenino , Humanos , Metástasis Linfática , Persona de Mediana Edad , Estadificación de Neoplasias , Radioterapia Adyuvante , Estudios RetrospectivosRESUMEN
BACKGROUND: Medulloblastoma is the most common malignant brain tumor in children. To date only few cases of medulloblastoma with hemorrhages have been reported in the literature. Although some studies speculate on the pathogenesis of this anomalous increased vascularization in medulloblastoma, the specific mechanism is still far from clearly understood. A correlation between molecular medulloblastoma subgroups and hemorrhagic features has not been reported, although recent preliminary studies described that WNT-subtype tumors display increased vascularization and hemorrhaging. CASE PRESENTATION: Herein, we describe a child with a Wnt-medulloblastoma presenting as cerebellar-vermian hemorrhagic lesion. Brain magnetic resonance imaging (MRI) showed the presence of a midline posterior fossa mass with a cystic hemorrhagic component. The differential diagnosis based on imaging included cavernous hemangioma, arteriovenous malformation and traumatic lesion. At surgery, the tumor appeared richly vascularized as documented by the preoperative angiography. CONCLUSIONS: The case we present showed that Wnt medulloblastoma may be associated with anomalous vascularization. Further studies are needed to elucidate if there is a link between the hypervascularization and the Wnt/ß-catenin signaling activation and if this abnormal vasculature might influence drug penetration contributing to good prognosis of this medulloblastoma subgroup.
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Neoplasias Cerebelosas/irrigación sanguínea , Meduloblastoma/irrigación sanguínea , Neovascularización Patológica , Vía de Señalización Wnt , Neoplasias Cerebelosas/complicaciones , Neoplasias Cerebelosas/diagnóstico por imagen , Niño , Femenino , Humanos , Hemorragias Intracraneales/complicaciones , Hemorragias Intracraneales/diagnóstico por imagen , Imagen por Resonancia Magnética , Meduloblastoma/complicaciones , Meduloblastoma/diagnóstico por imagen , Neovascularización Patológica/complicaciones , Neovascularización Patológica/diagnóstico por imagen , NeuroimagenRESUMEN
BACKGROUND: Primary melanocytic neoplasms are rare in the pediatric age. Among them, the pattern of neoplastic meningitis represents a peculiar diagnostic challenge since neuroradiological features may be subtle and cerebrospinal fluid analysis may not be informative. Clinical misdiagnosis of neoplastic meningitis with tuberculous meningitis has been described in few pediatric cases, leading to a significant delay in appropriate management of patients. We describe the case of a child with primary leptomeningeal melanoma (LMM) that was initially misdiagnosed with tuberculous meningitis. We review the clinical and molecular aspects of LMM and discuss on clinical and diagnostic implications. CASE PRESENTATION: A 27-month-old girl with a 1-week history of vomiting with mild intermittent strabismus underwent Magnetic Resonance Imaging, showing diffuse brainstem and spinal leptomeningeal enhancement. Cerebrospinal fluid analysis was unremarkable. Antitubercular treatment was started without any improvement. A spinal intradural biopsy was suggestive for primary leptomeningeal melanomatosis. Chemotherapy was started, but general clinical conditions progressively worsened and patient died 11 months after diagnosis. Molecular investigations were performed post-mortem on tumor tissue and revealed absence of BRAF(V600E), GNAQ(Q209) and GNA11(Q209) mutations but the presence of a NRAS(Q61K) mutation. CONCLUSIONS: Our case adds some information to the limited experience of the literature, confirming the presence of the NRAS(Q61K) mutation in children with melanomatosis. To our knowledge, this is the first case of leptomeningeal melanocytic neoplasms (LMN) without associated skin lesions to harbor this mutation. Isolated LMN presentation might be insidious, mimicking tuberculous meningitis, and should be suspected if no definite diagnosis is possible or if antitubercular treatment does not result in dramatic clinical improvement. Leptomeningeal biopsy should be considered, not only to confirm diagnosis of LMN but also to study molecular profile. Further molecular profiling and preclinical models will be pivotal in testing combination of target therapy to treat this challenging disease.
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GTP Fosfohidrolasas/genética , Melanoma/genética , Proteínas de la Membrana/genética , Neoplasias Meníngeas/genética , Mutación , Preescolar , Diagnóstico Diferencial , Resultado Fatal , Femenino , Humanos , Imagen por Resonancia Magnética , Melanoma/diagnóstico , Melanoma/diagnóstico por imagen , Neoplasias Meníngeas/diagnóstico , Neoplasias Meníngeas/diagnóstico por imagenRESUMEN
Primary Neuroendocrine Tumours (NETs) of the gallbladder are rare. Among all NETs of the gallbladder, large cell neuroendocrine carcinoma (LCNEC) is exceedingly rare. In most of the cases LCNECs are combined with other histological components. We reviewed clinical presentation and management of all patients with "pure" LCNEC from published literature since the first case was published in 2000, as well as one patient from our experience. Only 7 cases of "pure" LCNEC has been described in the last 15 years, our case is the eighth. The diagnosis of gallbladder NETs is rarely made preoperatively since the presentation generally consists of non-specific symptoms including upper abdominal pain, discomfort, jaundice, weight loss. The majority of patients are identified incidentally at the time of cholecystectomy for cholelithiasis. It is not possible to differentiate preoperatively between gallbladder adenocarcinoma and gallbladder neuroendocrine carcinoma (NEC) with imaging techniques. The only curative therapeutic modality for LCNECs is a complete en bloc surgical resection, including regional lymph node clearances and hepatic lobectomy, but only in patients without multiple metastasis. LCNECs benefit from an aggressive surgical resection in combination with chemotherapy, if resectability is possible. If the tumour is non-resectable, the primary management is therefore medical and not surgical. All patients with LCNEC presented a poor prognosis with a median survival of 10 months after the initial diagnosis. Only in five patients (62.5%) a wide surgical excision was performed, while in the other cases the tumour was non-resectable or multiple liver metastases were present at diagnosis.
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Carcinoma de Células Grandes/patología , Carcinoma Neuroendocrino/patología , Colecistectomía Laparoscópica , Neoplasias de la Vesícula Biliar/patología , Cálculos Biliares/cirugía , Hallazgos Incidentales , Adenocarcinoma/diagnóstico , Anciano , Carcinoma de Células Grandes/cirugía , Carcinoma Neuroendocrino/cirugía , Diagnóstico Diferencial , Femenino , Neoplasias de la Vesícula Biliar/cirugía , Hepatectomía , Humanos , Neoplasias Hepáticas/secundario , Tumores Neuroendocrinos/cirugíaRESUMEN
INTRODUCTION: The need for performing axillary lymph-node dissection in early breast cancer when the sentinel lymph node (SLN) is positive has been questioned in recent years. The purpose of this study was to identify a low-risk subgroup of early breast cancer patients in whom surgical axillary staging could be avoided, and to assess the probability of having a positive lymph-node (LN). METHODS: We evaluated the cohort of 612 consecutive women affected by early breast cancer. We considered age, tumor size, histological grade, vascular invasion, lymphatic invasion and cancer subtype (Luminal A, Luminal B HER-2+, Luminal B HER-2-, HER-2+, and Triple Negative) as variables for univariate and multivariate analyses to assess probability of there being a positive SLN o nonsentinel lymph node (NSLN). Chi-square, Fisher's Exact test and Student's t tests were used to investigate the relationship between variables; whereas logit models were used to estimate and quantify the strength of the relationship among some covariates and SLN or the number of metastases. RESULTS: A significant positive effect of vascular invasion and lymphatic invasion (odds ratios are 4 and 6), and a negative effect of TN (odds ratios is 10) were noted. With respect to positive NSLN, size alone has a significant (positive) effect on tumor presence, but focusing on the number of metastases, also age has a (negative) significant effect. CONCLUSION: This work shows correlation between subtypes and the probability of having positive SLN. Patients not expressing vascular invasion, lymphatic invasion and, moreover, a triple-negative tumor subtype may be good candidates for breast conservative surgery without axillary surgical staging.
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Neoplasias de la Mama/patología , Toma de Decisiones Clínicas , Escisión del Ganglio Linfático , Estadificación de Neoplasias , Biopsia del Ganglio Linfático Centinela , Adulto , Factores de Edad , Anciano , Anciano de 80 o más Años , Axila , Neoplasias de la Mama/metabolismo , Estudios de Cohortes , Femenino , Humanos , Metástasis Linfática , Persona de Mediana Edad , Invasividad Neoplásica , Receptor ErbB-2/metabolismo , Estudios Retrospectivos , Neoplasias de la Mama Triple Negativas/patologíaRESUMEN
Pancreaticoduodenectomy (PD) is considered the gold standard treatment for periampullory carcinomas. This procedure presents 30%-40% of morbidity. Patients who have undergone pancreaticoduodenectomy often present perioperative malnutrition that is worse in the early postoperative days, affects the process of healing, the intestinal barrier function and the number of postoperative complications. Few studies focus on the relation between enteral nutrition (EN) and postoperative complications. Our aim was to perform a review, including only randomized controlled trial meta-analyses or well-designed studies, of evidence regarding the correlation between EN and main complications and outcomes after pancreaticoduodenectomy, as delayed gastric emptying (DGE), postoperative pancreatic fistula (POPF), postpancreatectomy hemorrhage (PPH), length of stay and infectious complications. Several studies, especially randomized controlled trial have shown that EN does not increase the rate of DGE. EN appeared safe and tolerated for patients after PD, even if it did not reveal any advantages in terms of POPF, PPH, length of stay and infectious complications.