Craniofaringioma: marcatori prognostico-predittivi di comportamento biologico aggressivo

I craniofaringiomi (CP) sono tumori epiteliali benigni della regione sellare che originano da residui embrionali della tasca di Rathke. Si distinguono due sottotipi, l’adamantinomatoso (adaCP) e il papillare (papCP), che differiscono tra loro per gli aspetti istologici, etiopatogenetici e per l’outcome clinico. L’adaCP è il sottotipo più comune e, pur essendo una neoplasia istologicamente benigna (grado I sec. WHO), mostra un carattere localmente aggressivo che si manifesta con la tendenza a infiltrare le circostanti strutture cerebrali e a recidivare, sia a seguito dell’asportazione totale che di quella parziale associata a radioterapia. Tali aspetti rendono l’adaCP una neoplasia di difficile management terapeutico. Attualmente i meccanismi etiopatogenetici di questa neoplasia ed i fattori associati con la tendenza alla recidiva non sono del tutto noti, né disponiamo di efficaci marcatori prognostico-predittivi per poter individuare i casi di neoplasia caratterizzati da una maggiore aggressività biologica. Sulla base di quanto esposto, abbiamo deciso di effettuare uno studio neuropatologico ed immunoistochimico con correlazioni cliniche su un gruppo selezionato di CP, con gli obiettivi di poter meglio comprendere la patobiologia di questi complessi tumori ed i fattori associati con la tendenza alla recidiva, nello specifico del sottotipo adamantinomatoso, e, soprattutto, di individuare dei potenziali marcatori prognostico-predittivi da utilizzare, mediante metodica immunoistochimica, per identificare ab initio i casi di neoplasie biologicamente più aggressivi e, quindi, a maggior rischio di recidiva. Abbiamo quindi eseguito uno studio retrospettivo su ventisette CP asportati totalmente mediante tecnica endoscopica endonasale, pertanto la radicalità chirurgica ha rappresentato il criterio di inclusione nello studio. I casi sono stati distinti in gruppi sulla base dell’outcome clinico, ovvero presenza o assenza di recidiva tumorale. Abbiamo quindi valutato, mediante metodica immunoistochimica, l’espressione della β-catenina, l’espressione di noti marcatori di staminalità (CD133, CD166) e l’indice di proliferazione (Ki-67). Si tratta di un argomento poco studiato e con solo pochi lavori presenti in letteratura che tuttavia può fornire risultati significativi in termini di outcome prognostico. Ovvero, tali risultati potrebbero modificare il management terapeutico dei pazienti attraverso trattamenti aggiuntivi postoperatori per ridurre il rischio di recidive a lungo termine

Central Pancreatectomy to Treat a Giant Solid Pseudopapillary Tumor of the Pancreas - An Uncommon Surgical Procedure for a Rare Tumor: A Case Report and Review of the Literature

Context Solid pseudopapillary tumor is a rare tumor of the pancreas. Solid pseudopapillary tumor is nine times more frequent in the body and tail than in the head of the pancreas. It usually does not reach a large size. The majority of patients are young females and most of them are asymptomatic. Neoplasms of the midportion of the pancreas, not suitable for enucleation, can be treated with central pancreatectomy. The central pancreatectomy is commonly proposed for tumors that do not exceed 5 cm of dimension. Case report We report a case of seventeen-years-old woman who was admitted to our institution with abdominal pain and a palpable mass in the left hypochondrial area. US, CT and RMN scan revealed a giant (> 10 cm.) well-demarcated and encapsulated solid mass in the body of the pancreas, with a great amount of intralesional cystic-hemorrhagic component. The patient was treated by a variant of central pancreatectomy without splenectomy and pancreatic duct reconstruction. There was no metastatic disease in either the liver or peritoneum. The histological analysis identifies the tumor as a Solid pseudopapillary tumor of the pancreas. Fifteen years' follow-up period showed no recurrence. Conclusions Solid pseudopapillary tumor of the pancreas behaves like a potentially malignant tumor and has good prognosis. Surgical resection is dictated by tumor location and is the treatment of choice. The increasing interest in parenchyma-sparing pancreatic surgery has found large application in treatment of Solid pseudopapillary tumor, with the aim of preserving exocrine and endocrine pancreatic function, and achieving a better quality of life after surgery. Considering the low malignant potential, the central pancreatectomy can be considered an excellent therapeutic strategy for the treatment of this type of tumor also when they have giant dimensions and are localized in the central-pancreatic portion

CD10, BCL6, and MUM1 expression in diffuse large B-cell lymphoma on FNA samples

BACKGROUND: Gene expression profiling has divided diffuse large B-cell lymphoma (DLBCL) into 2 main subgroups: germinal center B (GCB) and non-GCB type. This classification is reproducible by immunohistochemistry using specific antibodies such as CD10, B-cell lymphoma 6 (BCL6), and multiple myeloma oncogene 1 (MUM1). Fine-needle aspiration (FNA) plays an important role in the diagnosis of non-Hodgkin lymphoma, and in some cases FNA may be the only available pathological specimen. The objectives of the current study were to evaluate CD10, BCL6, and MUM1 immunostaining on FNA samples by testing the CD10, BCL6, and MUM1 algorithm on both FNA cell blocks (CB) and conventional smears (CS), evaluating differences in CB and CS immunocytochemical (ICC) performance, and comparing results with histological data. METHODS: Thirty-eight consecutive DLBCL cases diagnosed by FNA were studied. Additional passes were used to prepare CB in 22 cases and CS in 16 cases; the corresponding sections and smears were immunostained using CD10, BCL6, and MUM1 in all cases. The data obtained were compared with histological immunostaining in 24 cases. RESULTS: ICC was successful in 33 cases (18 CB and 15 CS) and not evaluable in 5 cases (4 CB and 1 CS). The CD10-BCL6-MUM1 algorithm subclassified DLBCL as GCB (9 cases) and non-GCB (24 cases). ICC data were confirmed on histologic staining in 24 cases. CONCLUSIONS: CD10, BCL6, and MUM1 ICC staining can be performed on FNA samples. The results herein prove it is reliable both on CB and CS, and is equally effective and comparable to immunohistochemistry data

Prevalence of Spinal Muscular Atrophy in the Era of Disease-Modifying Therapies: An Italian Nationwide Survey

Objective: Spinal muscular atrophy (SMA) is a neurodegenerative disorder caused by mutations in the SMN1 gene. The aim of this study was to assess the prevalence of SMA and treatment prescription in Italy. Methods: An online survey was distributed to 36 centers identified by the Italian government as referral centers for SMA. Data on the number of patients with SMA subdivided according to age, type, SMN2 copy number, and treatment were collected. Results: One thousand two hundred fifty-five patients with SMA are currently followed in the Italian centers with an estimated prevalence of 2.12/100,000. Of the 1,255, 284 were type I, 470 type II, 467 type III, and 15 type IV with estimated prevalence of 0.48, 0.79, 0.79 and 0.02/100,000, respectively. Three patients with SMA 0 and 16 presymptomatic patients were also included. Approximately 85% were receiving one of the available treatments. The percentage of treated patients decreased with decreasing severity (SMA I: 95.77%, SMA II: 85.11%, SMA III: 79.01%). Discussion: The results provide for the first time an estimate of the prevalence of SMA at the national level and the current distribution of patients treated with the available therapeutical options. These data provide a baseline to assess future changes in relation to the evolving therapeutical scenario

TRY plant trait database – enhanced coverage and open access

Plant traits - the morphological, anatomical, physiological, biochemical and phenological characteristics of plants - determine how plants respond to environmental factors, affect other trophic levels, and influence ecosystem properties and their benefits and detriments to people. Plant trait data thus represent the basis for a vast area of research spanning from evolutionary biology, community and functional ecology, to biodiversity conservation, ecosystem and landscape management, restoration, biogeography and earth system modelling. Since its foundation in 2007, the TRY database of plant traits has grown continuously. It now provides unprecedented data coverage under an open access data policy and is the main plant trait database used by the research community worldwide. Increasingly, the TRY database also supports new frontiers of trait‐based plant research, including the identification of data gaps and the subsequent mobilization or measurement of new data. To support this development, in this article we evaluate the extent of the trait data compiled in TRY and analyse emerging patterns of data coverage and representativeness. Best species coverage is achieved for categorical traits - almost complete coverage for ‘plant growth form’. However, most traits relevant for ecology and vegetation modelling are characterized by continuous intraspecific variation and trait–environmental relationships. These traits have to be measured on individual plants in their respective environment. Despite unprecedented data coverage, we observe a humbling lack of completeness and representativeness of these continuous traits in many aspects. We, therefore, conclude that reducing data gaps and biases in the TRY database remains a key challenge and requires a coordinated approach to data mobilization and trait measurements. This can only be achieved in collaboration with other initiatives

Multidisciplinary diagnostic approach combining fine needle aspiration, core needle biopsy and imaging features of a presacral myelolipoma in a patient with concurrent breast cancer

Myelolipomas are uncommon benign tumors composed of mature adipose tissue mixed with hematopoietic elements; these tumors can occur in both the adrenal glands and extra-adrenal locations, the presacral region being the most frequent extra-adrenal site. We present a case of presacral myelolipoma diagnosed by fine needle aspiration (FNA) and core needle biopsy (CNB) in a 55-year-old woman with concurrent invasive ductal breast cancer. TC and RM imaging were consistent with the diagnosis of presacral myelolipoma. The lesion was discovered incidentally during the staging procedure for breast malignancy. The purpose of our work is to describe the FNA and CNB finding in combination with the imaging features of this uncommon lesion

Effects of mycophenolate mofetil on acute ischaemia–reperfusion injury in rats and its consequences in the long term

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Multiple splenic hamartomas and familial adenomatous polyposis: a case report and review of the literature

Splenoma or splenic hamartoma is a rare primary splenic tumor most often discovered radiologically and incidentally. Splenic hamartomas have a strong association with solid and hematological malignancies and, in rare cases, with tuberous sclerosis, but to the best of our knowledge no reports of splenic hamartomas associated with familial adenomatous polyposis have been documented, although it is recognized that familial adenomatous polyposis presents a variety of extracolonic manifestations

Unusual breast lesion mimicking cancer: Diabetic mastopathy

AbstractDiabetic mastopathy represents an uncommon tumor-like proliferation of fibrous tissue of the breast that usually occurs in patients who suffered from type 1 diabetes mellitus for a long time. We report an uncommon case of diabetic mastopathy presenting in a type 2 non-insulin dependent diabetes mellitus 61-year-old postmenopausal woman. Physical examination revealed a hard, low movable mass in the upper outer quadrant of the right breast. Mammography and ultrasonography showed typical features of breast cancer. Ultrasound-guided fine-needle aspiration cytology (US-FNAC) was performed showing inflammatory infiltrate, suggesting excisional biopsy. Histological findings demonstrated typical diabetic mastopathy with fibrosis, histiocytic and limphocytic infiltration without evidence of malignancy