1,721,034 research outputs found
Pazopanib as a possible option for the treatment of metastatic non-clear cell renal carcinoma patients: a systematic review
No full textEffective systemic treatment of non-clear cell renal carcinoma (nccRCC) is still an unmet clinical need, with few studies to support an evidence-based approach. To date, the only recommended standard first-line treatment is sunitinib. Pazopanib may also be used in nccRCC but its place in therapy is not clearly established. It has comparable efficacy and better tolerability than sunitinib in clear cell renal carcinoma. Our objective was to review the use of pazopanib in metastatic nccRCC
Lung Manifestation of IgG4-related Disease. A multifaceted disorder. Presentation of two cases and short review of literature
No full textAbstract. Immunoglobulin (Ig) G4-related disease (IgG4-RD) is a recently described systemic inflammatory
disease associated with elevated circulating levels of IgG4. IgG4-RD may affect one or more organs and lesions
can present synchronously or metachronously in different organs. Pulmonary involvement of IgG4-related disease
includes airway, lung parenchyma, pleura and mediastinum. In this article, we report two cases of IgG4-RD
to show the multifaceted manifestations of this disease in the lungs. (Sarcoidosis Vasc Diffuse Lung Dis 2018; 35:
74-80
Giant intercostal nerve schwannoma in a patient with neurofibromatosis type 2
No full textTo describe a case of giant intercostal nerve schwannoma successfully resected in a patient with neurofibromatosis type 2
SOLITARY FIBROUS TUMOR OF THE PLEURA ASSOCIATED WITH SEVERE HYPOGLICEMIA: THE DOEGE-POTTER SYNDROME
No full textPili canaliculi: a familial study
No full textThe pili canaliculi is a rare defect, first described in 1973 by Dupre’ et al. as “le syndrome des cheveux incoiffables. The condition, characterized by dry, frizzy, unruly, rough and blond hair probably is due to a keratinization defect. The disorder may be autosomal dominant, recessive with variable levels of penetrance or sporadic . Sometimes
it is associated with ectodermal dysplasia , retinal dysplasia , dental anomalies, ichthyosis vulgaris, atopic eczema, progressive alopecia areata, hamartomas, lichen sclerosus, woolly hair nevus, brachydactyly, pili tort, digit and nail abnormalities. We report a three years old child with thin, blond, uncombable hairs, atopic dermatitis and onychoschizia. The maternal grandmother were said to have the same hairs in infancy. The mother had normal hairs. A study with scanning electron microscopy revealed the presence of typical triangular cross-sectional shapes with indentation, longitudinal grooving and flattening of the hair surfaces. These morphologic features were discontinuous and found on different surfaces along the length of the hair. The overlying cuticle of the hair was instead normal. These findings were present in many hairs of child and grandmother, but in few hairs of the mother. The repeating of this peculiar aspect in the hairs within this family suggests that inheritance pattern is autosomal dominant. Moreover, the characteristic hair morphology can be observed in relatives, without hair abnormality. Since, the disorder could be associated to other extracutaneous abnormalities, it is indispensable a follow-up of the affected patient
Placental Transmogrification of the Lung Presenting as a Giant Bulla Associated with a Pulmonary Hamartoma
Full text linkCryptogenic Fibrosing Pleuritis
Full text linkWe report the case of a 46-year-old male patient who was referred for chest pain and bilateral pleural effusion. Despite treatment with antibiotics and steroids, the pleural effusion worsened over a few months until pulmonary function was halved. The CT scan showed bilateral pleural thickening with right basal opacity. Histology revealed extensive fibrotic tissue with focal collections of lymphocytes and giant cells without traces of asbestos bodies. Since no evidence of an infectious, embolic or occupational aetiology was found, this bilateral pleural effusion progressing to diffuse pleural thickening was diagnosed as cryptogenic fibrosing pleuritis, a rare pleural disease
Computed tomography-histologic correlations in lung cancer
No full textThe multidisciplinary approach is ideal in the management of patients with lung cancer. Multidisciplinary evaluation strengthens the differential diagnosis of aspecific radiological findings, indeed. Notably, the differential diagnosis of early stage lung cancer is a current challenge of CT imaging because the earlier the detection, the lower the accuracy of radiological features. This is particularly true for the most common subtype of lung cancer, adenocarcinoma, because it shows various radiological features. Such variety is also reflected by the 2011 classification of lung cancer, that likely affected the diagnostic agreement between radiologist and clinician. This review discusses the common issues of lung cancer diagnosis by paired radiological-histologic interpretation of CT findings
GIANT PRIMARY DEDIFFERENTIATED LIPOSARCOMA OF THE ANTERIOR MEDIASTINUM: AN EXTREMELY RARE OCCURRENCE
No full textPrimary Large Cell Neuroendocrine Carcinoma of the Mediastinum
No full textBACKGROUND: to present a rare case of primary neuroendocrine carcinoma of the mediastinum treated by multimodal therapy.
METHODS: a 50-year-old man, non-smoker, with unremarkable past medical history, presented for asthenia, dyspnea and sub-sternal discomfort. A chest CT-scan showed a huge mass of 10,7x5,5x9,5cm in the anterosuperior mediastinum. The mass seems infiltrate both brachiocephalic veins, the superior vena cava, pericardium and both lungs (Fig.1a/b).
PET-CT showed an intense hyperactivity of the mediastinal mass. CT-guided needle biopsy allows diagnosis of neuroendocrine carcinoma. After multisciplinary discussion, a multimodal approach was planned. The patient underwent 4 cycles of chemotherapy with cisplatin and gemcitabine. Since chest CT-scan showed a reduction of the tumor (7,4x5,6x9cm), a surgical resection was proposed.
RESULTS: a median sternotomy was performed. On exploration, both lungs were marginally infiltrated. The pericardium was partially excised; the left anonymous vein was almost totally invaded by the tumor. The right anonymous vein was infiltrated at the confluence with the superior vena cava. After total caval clamping (clamping time: 27’), a partial section and reconstruction with bovine pericardium was performed (Fig.1c). The patient was discharged uneventfully on postoperative-day 9. The patient underwent 25 sessions of adjuvant radiotherapy. Currently, he’s free of disease after 30 months. Macroscopically the mass measured 9x8x3cm, looks whitish with tense-elastic consistency. Microscopically it showed clusters of small uniform cells, sometimes with cuboidal morphology, with nuclear pleomorphism and small nucleoli, arranged in trabeculae, nests and lobules and immersed in a hyaline stroma with foci of necrosis (Fig.2a). Perineural and vascular invasion were present. Immunohistochemistry showed the tumor cells were positive for chromogranin (Fig.2c), synaptophysin (Fig.2d), cytokeratins and negative for TTF1, FAP, PSA. The proliferation index Ki-67 was 10% (Fig.2b). Considering the radiological, morphological and immunophenotypic characteristics, the diagnosis was consistent with primary neuroendocrine carcinoma of the mediastinum.
CONCLUSIONS: mid-term survival was achieved after aggressive multimodal therapy
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