A rare neuronal tumor of the cerebellum with myoid features

We report an extremely rare tumor presenting with myoid features in the left cerebellar hemisphere in a 62-year-old man. This tumor consisted of medium to large round cells with focal lipomatous and myoid differentiation. Immunohistochemically, the tumor cells expressed synaptophysin, GFAP (glial fibrillary acidic protein) and focally desmin. From these findings, we concluded that this tumor was a liponeurocytoma with myoid features. To the best of our knowledge, this report constitutes the second described case of liponeurocytoma with myoid differentiation in the cerebellum

Carcinome métaplasique du sein avec différenciation osseuse extensive: À propos d’un cas

Le carcinome métaplasique du sein est une entité rare et bien individualisé par l'OMS. Il représente moins de 1 % des cancers invasifs du sein et constitue un groupe tumoral hétérogène soit purement épithélial soit à doublecontingent épithélial et mésenchymateuse. Le carcinome métaplasique avec différenciation osseuse extensive est très rare. Il représente 0.2% des carcinomes du sein. Nous rapportant un cas exceptionnel d'un carcinome métaplasique du sein avec différenciation osseuse extensive chez une patiente de 53 ans. A travers ce cas et une revue de la littérature, les caractéristiques anatomo-cliniques, radiologique, thérapeutiques et évolutives seront discutées

Mélanome endobronchique

Le mélanome malin a un potentiel métastatique important. Les métastases pulmonaires du mélanome sont communes cependant la localisation endobronchique reste rare et pose le problème de son origine primitive ou secondaire. Nous rapportons le cas d’un mélanome pulmonaire qui présente des particularités intéressantes: une lésion cutanée présumée primitive totalement régressive, la présentation radio clinique mimant parfaitement un cancer bronchique primitif, un aspect endoscopique bourgeonnant et grisâtre dont l’étude histologique a permis de poser le diagnostic, une agressivité tumorale avec une extension intracardiaque et bourgeon tumoral intra cavitaire. A travers cette observation, les auteurs étudient les caractéristiques radio-cliniques pouvant distinguer le mélanome pulmonaire primitif du secondaire; la localisation endobronchique avec une revue de la littérature sur les métastases endo bronchiques; le bilan d’extension à entamer en cas de mélanome pulmonaire ainsi que les difficultés thérapeutiques posés par ce type de lésion dont le pronostic reste péjoratif

Renal hilar pheochromocytoma: a case report

Paraganglioma is a rare tumor arising from undifferentiated cells of the primitive neural crest. These tumors are most commonly found in the adrenal gland, other localisations are also possible. A 58-year-old woman who presented with history of left lumbar pain, headache, hypertension, palpitation and sweating was found to have a secreting left renal hilar pheochromocytoma. Radical excision of the tumor was therefore undertaken and her hypertension was controlled. From the case report and literature review, the authors suggest a diagnostic and therapeutic strategy for the management of ectopic localization of pheochromocytoma

Radiopaque drug-eluting embolisation beads as fiducial markers for stereotactic liver radiotherapy

OBJECTIVE: To determine the feasibility of using radiopaque (RO) beads as direct tumour surrogates for image-guided radiotherapy (IGRT) in patients with liver tumours after transarterial chemoembolisation (TACE). METHODS: A novel vandetanib-eluting RO bead was delivered via TACE as part of a first-in-human clinical trial in patients with either hepatocellular carcinoma or liver metastases from colorectal cancer. Following TACE, patients underwent simulated radiotherapy imaging with 4-dimensional computed tomography (4D-CT) and cone-beam CT (CBCT) imaging. RO beads were contoured using automated thresholding, and feasibility of matching between the simulated radiotherapy planning dataset (AVE-IP image from 4D data) and CBCT scans assessed. Additional kV, MV, helical CT and CBCT images of RO beads were obtained using an in-house phantom. Stability of RO bead position was assessed by comparing 4D-CT imaging to CT scans taken 6-20 days following TACE. RESULTS: Eight patients were treated and 4D-CT and CBCT images acquired. RO beads were visible on 4D-CT and CBCT images in all cases and matching successfully performed. Differences in centre of mass of RO beads between CBCT and simulated radiotherapy planning scans (AVE-IP dataset) were: 2.0 mm mediolaterally, 1.7 mm anteroposteriorally, 3.5 mm craniocaudally. RO beads in the phantom were visible on all imaging modalities assessed. RO bead position remained stable up to 29 days post-TACE. CONCLUSION: RO beads are visible on IGRT imaging modalities, showing minimal artefact. They can be used for on-set matching with CBCT and remain stable over time. ADVANCES IN KNOWLEDGE: The role of RO beads as fiducial markers for stereotactic liver radiotherapy is feasible and warrants further exploration as a combination therapy approach

Pathological complete response in advanced gastric stromal tumor after imatinib mesylate therapy: a case report

<p>Abstract</p> <p>Introduction</p> <p>Gastrointestinal stromal tumors are a rare neoplasm exhibiting, in most cases, mutations of <it>c-kit</it>. Imatinib mesylate is the standard treatment for patients who have advanced gastrointestinal stromal tumors. Although the response rate in patients treated with imatinib mesylate in prospective clinical studies is above 50%, a complete response is very rare. We report the case of a patient with a gastric gastrointestinal stromal tumor who had a pathological complete response after neoadjuvant treatment with imatinib mesylate.</p> <p>Case presentation</p> <p>We report the case of a 54-year-old Arab woman with a gastrointestinal stromal tumor who had a pathological complete response after neoadjuvant treatment with imatinib mesylate.</p> <p>Conclusion</p> <p>The pathological examination of our patient documented a complete pathological response after imatinib therapy. Recently, it has been confirmed that the kinase genotype of <it>KIT </it>and <it>platelet-derived growth factor receptor α </it>can accurately predict a good response to imatinib mesylate therapy. We propose that this patient had a mutation conferring high sensitivity to imatinib mesylate.</p

VEROnA Protocol: A Pilot, Open-Label, Single-Arm, Phase 0, Window-of-Opportunity Study of Vandetanib-Eluting Radiopaque Embolic Beads (BTG-002814) in Patients With Resectable Liver Malignancies

BACKGROUND: Transarterial chemoembolization (TACE) is the current standard of care for patients with intermediate-stage hepatocellular carcinoma (HCC) and is also a treatment option for patients with liver metastases from colorectal cancer. However, TACE is not a curative treatment, and tumor progression occurs in more than half of the patients treated. Despite advances and technical refinements of TACE, including the introduction of drug-eluting beads-TACE, the clinical efficacy of TACE has not been optimized, and improved arterial therapies are required. OBJECTIVE: The primary objectives of the VEROnA study are to evaluate the safety and tolerability of vandetanib-eluting radiopaque embolic beads (BTG-002814) in patients with resectable liver malignancies and to determine concentrations of vandetanib and the N-desmethyl metabolite in plasma and resected liver following treatment with BTG-002814. METHODS: The VEROnA study is a first-in-human, open-label, single-arm, phase 0, window-of-opportunity study of BTG-002814 (containing 100 mg vandetanib) delivered transarterially, 7 to 21 days before surgery in patients with resectable liver malignancies. Eligible patients have a diagnosis of colorectal liver metastases, or HCC (Childs Pugh A), diagnosed histologically or radiologically, and are candidates for liver surgery. All patients are followed up for 28 days following surgery. Secondary objectives of this study are to evaluate the anatomical distribution of BTG-002814 on noncontrast-enhanced imaging, to evaluate histopathological features in the surgical specimen, and to assess changes in blood flow on dynamic contrast-enhanced magnetic resonance imaging following treatment with BTG-002814. Exploratory objectives of this study are to study blood biomarkers with the potential to identify patients likely to respond to treatment and to correlate the distribution of BTG-002814 on imaging with pathology by 3-dimensional modeling. RESULTS: Enrollment for the study was completed in February 2019. Results of a planned interim analysis were reviewed by a safety committee after the first 3 patients completed follow-up. The recommendation of the committee was to continue the study without any changes to the dose or trial design, as there were no significant unexpected toxicities related to BTG-002814. CONCLUSIONS: The VEROnA study is studying the feasibility of administering BTG-002814 to optimize the use of this novel technology as liver-directed therapy for patients with primary and secondary liver cancer. TRIAL REGISTRATION: ClinicalTrial.gov NCT03291379; https://clinicaltrials.gov/ct2/show/NCT03291379. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/13696

Pseudotumoral tracheobronchial amyloidosis mimicking asthma: a case report

<p>Abstract</p> <p>Introduction</p> <p>Tracheobronchial amyloidosis is an uncommon localized form of amyloidosis that can simulate a tracheal tumor. Clinical signs are not specific and the diagnosis is rarely given before performing a bronchoscopy with multiples biopsies.</p> <p>Case presentation</p> <p>We report the case of a 60-year-old Moroccan woman, complaining of dyspnea and wheezing for three years, who was treated at our institution for management of severe asthma. A bronchoscopy revealed a tumor formation of her trachea; multiples biopsies were performed and a diagnosis made of amyloid light-chain amyloidosis. She successfully received an endoscopic resection.</p> <p>Conclusion</p> <p>This case highlights the importance of routinely carrying out an endoscopy in any patient complaining of atypical bronchial symptoms or with uncontrolled asthma. Tracheal amyloidosis is a rare disease, confirmed by histological examination of bronchial biopsies, and the treatment of choice is based on the bronchoscopic resection.</p

Hyperoxia increases the uptake of 5-fluorouracil in mammary tumors independently of changes in interstitial fluid pressure and tumor stroma

<p>Abstract</p> <p>Background</p> <p>Hypoxia is associated with increased resistance to chemo- and radiation-therapy. Hyperoxic treatment (hyperbaric oxygen) has previously been shown to potentiate the effect of some forms of chemotherapy, and this has been ascribed to enhanced cytotoxicity or neovascularisation. The aim of this study was to elucidate whether hyperoxia also enhances any actual uptake of 5FU (5-fluorouracil) into the tumor tissue and if this can be explained by changes in the interstitium and extracellular matrix.</p> <p>Methods</p> <p>One group of tumor bearing rats was exposed to repeated hyperbaric oxygen (HBO) treatment (2 bar, pO₂= 2 bar, 4 exposures à 90 min), whereas one group was exposed to one single identical HBO treatment. Animals housed under normal atmosphere (1 bar, pO₂= 0.2 bar) served as controls. Three doses of 5FU were tested for dose response. Uptake of [³H]-5FU in the tumor was assessed, with special reference to factors that might have contributed, such as interstitial fluid pressure (P_if), collagen content, oxygen stress (measured as malondialdehyd levels), lymphatics and transcapillary transport in the tumors.</p> <p>Results</p> <p>The uptake of the cytostatic agent increases immediately after a single HBO treatment (more than 50%), but not 24 hours after the last repeated HBO treatment. Thus, the uptake is most likely related to the transient increase in oxygenation in the tumor tissue. Factors like tumor P_ifand collagen content, which decreased significantly in the tumor interstitium after repeated HBO treatment, was without effect on the drug uptake.</p> <p>Conclusion</p> <p>We showed that hyperoxia increases the uptake of [³H]-5FU in DMBA-induced mammary tumors <it>per se</it>, independently of changes in P_if, oxygen stress, collagen fibril density, or transendothelial transport alone. The mechanism by which such an uptake occur is still not elucidated, but it is clearly stimulated by elevated pO₂.</p