Advances in management of nonendometrioid endometrial carcinoma, with an emphasis on the sentinel lymph node technique
Purpose of review During the last few years there have been important advances in our understanding of endometrial cancer biology, staging, and therapy. In this article, we discuss updates and controversies in the treatment of nonendometrioid endometrial carcinoma (non-EEC), with an emphasis on the role of sentinel lymph node (SLN) biopsy. Recent findings Lymph node involvement is an important factor in determining prognosis and guiding adjuvant therapy in endometrial carcinoma. SLN biopsy has emerged as a less morbid alternative to lymphadenectomy in surgical staging, and it has generally gained acceptance in t...
Source: Current Opinion in Oncology - August 27, 2021 Category: Cancer & Oncology Tags: GYNECOLOGIC CANCER: Edited by Frédéric Amant and Christianne Lok Source Type: research

Immunotherapy in rare ovarian cancer
Purpose of review Ovarian cancer (OC) is a heterogeneous disease and a mounting body of evidence shows that a ‘one-size-fits-all’ approach is obsolete. Differences in epidemiology, tumor biology, genetic profiles and treatment responses of these different types necessitate a tumor and patient-specific approach. Ninety percentage consists of epithelial OC with 70% being high-grade serous OC. The other rarer subtypes are low-grade serous (5%), clear cell (12%), endometrioid (11%) and mucinous carcinoma (3%). The remaining 10% are nonepithelial rare OCs: germ cell (3%) and sex-cord stromal tumors (7%). Recent findi...
Source: Current Opinion in Oncology - August 27, 2021 Category: Cancer & Oncology Tags: GYNECOLOGIC CANCER: Edited by Frédéric Amant and Christianne Lok Source Type: research

Importance of guidelines and networking for the management of rare gynecological cancers
The objective of our paper is to highlight the need for such recommendations, to reinforce strategies in place and to promote the creation of multidisciplinary networks to provide the most appropriate care to patients and to improve it. Recent findings Gynecological rare cancers are not that rare since they represent around 50% of all gynecological cancers. Surgery remains the cornerstone of management for all subtypes. Apart from malignant ovarian germ-cell tumors for which conventional chemotherapy was proven to be efficient, response to standard systemic treatment for other gynecological cancers are disappointing...
Source: Current Opinion in Oncology - August 27, 2021 Category: Cancer & Oncology Tags: GYNECOLOGIC CANCER: Edited by Frédéric Amant and Christianne Lok Source Type: research

Ten steps to establish a national centre for gestational trophoblastic disease
Purpose of review Gestational trophoblastic disease (GTD) is a group of heterogeneous disorders characterized by abnormal proliferation of trophoblastic tissue. GTD is a rare disease that is curable in the vast majority of patients when managed appropriately. The aim of the review is to discuss the important steps necessary to establish a center of excellence for GTD. Recent findings Care of patients with a rare disease is complicated by lack of strong evidence, scattering of patients across the country and limited expertise of medical professionals. The establishment of a center of excellence requires awareness...
Source: Current Opinion in Oncology - August 27, 2021 Category: Cancer & Oncology Tags: GYNECOLOGIC CANCER: Edited by Frédéric Amant and Christianne Lok Source Type: research

Editorial: The challenge of treating and investigating rare gynaecologic cancers
No abstract available (Source: Current Opinion in Oncology)
Source: Current Opinion in Oncology - August 27, 2021 Category: Cancer & Oncology Tags: GYNECOLOGIC CANCER: Edited by Frédéric Amant and Christianne Lok Source Type: research

Primary vitreoretinal lymphoma: short review of the literature, results of a European survey and French guidelines of the LOC network for diagnosis, treatment and follow-up
Purpose of review The aim of this study was to highlight the diagnostic and management challenges of primary vitreoretinal lymphoma (PVRL) through a review of the literature and a European survey on real-life practices for PVRL. Recent findings The care of PVRL patients is heterogeneous between specialists and countries. Upfront systemic treatment based on high-dose methotrexate chemotherapy, with or without local treatment, might reduce or delay the risk of brain relapse. Ibrutinib, lenalidomide with or without rituximab, and temozolomide are effective for patients with relapsed/refractory PVRL and should be...
Source: Current Opinion in Oncology - August 27, 2021 Category: Cancer & Oncology Tags: LYMPHOMA: Edited by Dominique Bron Source Type: research

Hairy cell leukemia: a brief update on current knowledge and treatment prospects
This article provides a brief update on the recommended diagnosis and treatment strategies for patients with the classic form of hairy cell leukemia (HCL) and HCL variant (HCLv). Recent findings HCL is a chronic B-cell malignancy with multiple treatment options. In recent years, many novel drugs have been assessed for HCL treatment with promising results. The investigated nonchemotherapy options include moxetumomab pasudotox, which targets CD22; vemurafenib or dabrafenib, which target the BRAFV600E protein; trametinib, which targets mitogen-activated protein kinase enzyme; and ibrutinib, which targets Bruton tyrosin...
Source: Current Opinion in Oncology - August 27, 2021 Category: Cancer & Oncology Tags: LYMPHOMA: Edited by Dominique Bron Source Type: research

Hepatosplenic T-cell lymphoma: treatment challenges
Purpose of review Hepatosplenic lymphoma (HSTCL) is a rare T-cell malignancy occurring in young males, associated with immune deficiency in 20% of the cases which, despite aggressive treatments, has a poor survival. Specific recommendations for first-line treatment remain debatable. Recent findings Published data covering case reports or series of HSTCL concur that allogeneic stem cell transplant should be proposed as a consolidation after response to chemotherapy in all patients eligible for transplant. In the light of two recent clinical examples, we also confirm that specific chemotherapy and a first-line con...
Source: Current Opinion in Oncology - August 27, 2021 Category: Cancer & Oncology Tags: LYMPHOMA: Edited by Dominique Bron Source Type: research

Epigenetic focus on angioimmunoblastic T-cell lymphoma: pathogenesis and treatment
Purpose of review Angioimmunoblastic T-cell lymphoma (AITL) is a frequent peripheral T-cell lymphoma affecting elderly patients with a poor outcome when treated with conventional chemotherapy. Molecular studies revealed a homogenous mutational landscape gathering anomalies in genes regulating the DNA methylation and hydroxymethylation and anomalies in T-cell signalling. Recent findings Recent studies indicate that AITL emerges from a TET2 and/or DNMT3A mutated clonal haematopoiesis. This clonal haematopoiesis bearing mutations altering DNA hydroxymethylation can explain the observed coexistence of AITL with myel...
Source: Current Opinion in Oncology - August 27, 2021 Category: Cancer & Oncology Tags: LYMPHOMA: Edited by Dominique Bron Source Type: research

Current treatment options for nodular lymphocyte-predominant Hodgkin lymphoma
Purpose of review Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a rare B cell-derived malignancy. This review aims at providing an overview of recent developments in the management of NLPHL. Recent findings Patients with stage IA NLPHL without risk factors have excellent outcomes. The 8-year progression-free survival (PFS) is roughly 90% and the 8-year overall survival (OS) close to 100% after limited-field radiotherapy (RT) alone. Individuals presenting with early stages other than stage IA without risk factors and intermediate stages have 10-year PFS rates in excess of 70% and 10-year OS rates exc...
Source: Current Opinion in Oncology - August 27, 2021 Category: Cancer & Oncology Tags: LYMPHOMA: Edited by Dominique Bron Source Type: research

Editorial introductions
No abstract available (Source: Current Opinion in Oncology)
Source: Current Opinion in Oncology - August 27, 2021 Category: Cancer & Oncology Tags: EDITORIAL INTRODUCTIONS Source Type: research

The evolving treatment paradigm of advanced hepatocellular carcinoma: putting all the pieces back together
Purpose of review The therapeutic landscape of advanced hepatocellular carcinoma (HCC) has become notably complex in recent years. With this review, we aimed to put the most recent findings in perspective and tried to delineate the rapidly changing treatment algorithm. Recent findings The combination of atezolizumab and bevacizumab has become the new first-line standard of care treatment for unresectable HCC after the positive results of the phase 3 IMbrave150 study. Nivolumab monotherapy failed to demonstrate advantage versus sorafenib in the CheckMate 459 trial, while two different therapeutic strategies (sint...
Source: Current Opinion in Oncology - June 18, 2021 Category: Cancer & Oncology Tags: GASTROINTESTINAL TRACT: Edited by Alain Hendlisz and Francesco Sclafani Source Type: research

State of the art and future directions in the systemic treatment of neuroendocrine neoplasms
Purpose of review Neuroendocrine neoplasms (NENs) are rare and heterogeneous malignancies whose natural evolution may be defined according to various prognostic factors, including localization of the primitive tumour, hormone secretory status, histological grade, tumour burden, tumour growth rate, expression of somatostatin receptors and fluorodeoxyglucose-avidity. The treatment of these tumours in an advanced setting is based on relatively little robust data. Recent findings A recent pathological classification introduced a new category of high-grade but well differentiated neuroendocrine tumours (NET G3), with...
Source: Current Opinion in Oncology - June 18, 2021 Category: Cancer & Oncology Tags: GASTROINTESTINAL TRACT: Edited by Alain Hendlisz and Francesco Sclafani Source Type: research

Anal squamous cell carcinoma: standards of care, new data and ongoing clinical trials
Purpose of review To summarize current standards of care, discuss results of recent studies and present ongoing clinical trials for anal squamous cell carcinoma (ASCC). Recent findings Over the last year, no practice changing studies have been reported in the setting of localised ASCC. A number of retrospective analyses, however, have provided practice-informing data, such as those confirming the negative impact of low compliance to chemoradiotherapy (CRT) on patient outcomes. In contrast, and for the first time, randomized evidence has become available to inform the management of advanced tumours. The InterAACT...
Source: Current Opinion in Oncology - June 18, 2021 Category: Cancer & Oncology Tags: GASTROINTESTINAL TRACT: Edited by Alain Hendlisz and Francesco Sclafani Source Type: research

Management of small bowel adenocarcinoma: making the most of the available evidence to inform routine practice
Purpose of review Small bowel adenocarcinoma (SBA) is a rare disease, for which few studies have been conducted so far. Therefore, most treatment recommendations have been extrapolated from trials in colorectal cancer. In this review, we revise available data that could improve the management of SBA, with a particular focus on systemic therapy. Recent findings For advanced/irresectable disease, first-line doublet chemotherapy remains standard of care. It is uncertain whether extending treatment to triplet chemotherapy brings added benefit. Pembrolizumab is an accepted treatment modality for mismatch repair-defic...
Source: Current Opinion in Oncology - June 18, 2021 Category: Cancer & Oncology Tags: GASTROINTESTINAL TRACT: Edited by Alain Hendlisz and Francesco Sclafani Source Type: research