Case Report: Carcinoma en Cuirasse in a Middle-Aged Woman Mimicking Postirradiation Morphea

Breast carcinoma en cuirasse (CeC) is an extremely rare form of cutaneous metastases of breast cancer, characterized by diffuse sclerodermoid induration of the skin. It may be difficult to distinguish CeC from some skin diseases, including postirradiation morphea, inflammatory breast cancer, radiation dermatitis, and other cutaneous metastases, but it can be easily discerned by histology. Because of the small number of documented cases, the treatment consensus has not been clearly defined. Here, we show a 45-year-old woman with grade III infiltrating ductal carcinoma manifesting as CeC to the chest wall. Early diagnosis and treatment are essential to prevent the catastrophic natural progression of this rare malignancy.

Kidney involvement in monoclonal gammopathies: multidisciplinary approach in oncohematology and nephrology

The pathogenetic relationship of kidney damage and an aberrant clone of the B-cell line producing nephrotoxic monoclonal immunoglobulin underlies the concept of “monoclonal gammopathy of renal significance” (MGRS). Herein the aberrant clone does not reach the criteria necessary for initiating antitumor therapy according to oncohematological indications. MGRS is a new nosology in modern nephrology and oncohematology. Monoclonal protein’s pathological effects on kidney parenchyma result in irreversible decline of kidney function till the end stage renal disease that in line with the position of International Consensus of hematologists and nephrologists determinates critical necessity for clone specific treatment in patients with MGRS despite the absence of hematological indications for treatment initiation. Main challenge of MGRS in Russian Federation is an inaccessibility of an in-time diagnostic and appropriate treatment for the great majority of patients due to the following reasons: i) limited knowledge about the MGRS among hematologists and nephrologists; ii) lack of necessary diagnostic resources in most health-care facilities; iii) lack of approved clinical recommendations and medical economic standards for treatment of this pathological entity. In order to overcome these limitations, leading oncohematologists and nephrologists of the Russian Federation on behalf of professional communities at the end of 2019 published a conciliation document: “Monoclonal gammopathy of renal significance: Consensus of hematologists and nephrologists of Russia on the establishment of nosology, diagnostic approach and rationale for clone specific treatment”. Consensus document comprises the opinion of experts – leading nephrologists and hematologists of Russian Federation – on the problem of MGRS including the incoherence in nosology classification, diagnostics approach and rationale for clone specific treatment. Consensus document is based on conclusions and agreements reached during the conference of leading nephrologists and hematologists of Russia which was held in the framework of symposia “Plasma cell dyscrasias and lymphoproliferative diseases: modern approaches to therapy”, 15–16 of March 2019, Pavlov First Saint Petersburg State Medical University, Saint Petersburg, Russia. Consensus is intended to define the principal practical steps to resolve the problem of MGRS in Russian Federation that are summarized as final clauses which we present here.

Complete Response to R-EPOCH in Primary Cardiac Lymphoma

Primary cardiac lymphoma (PCL) is a rare extranodal lymphoma involving only the heart and/or the pericardium. Most common presenting signs and symptoms are nonspecific including dyspnea, pericardial effusion, and arrhythmia. Prognosis of PCL patients remain poor compared to non‐cardiac lymphoma patients. Since most of the information about PCL comes from case reports or case series, there is no treatment consensus. Anthracycline containing chemotherapy remains main treatment modality which is potentially cardiotoxic. We present a case of PCL that achieved complete remission using R-EPOCH (rituximab, etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin). We also used dexrazoxane in an effort to reduce cardiotoxicity of chemotherapy.

Comprehensive and epidemiological characterization of urachal adenocarcinoma: A pan-Canadian collaboration.

516 Background: Urachal cancer is a rare form of urothelial cancer representing 0,1-0,2% of bladder cancers. The exact underlying cause of urachal cancer is still widely unknown and there is much speculation on multiple factors such as genetic and environmental ones that may play a role. Moreover, given the lack of treatment consensus, we proposed the first study that assesses the clinical, genetic and molecular features of this disease in Canada. Methods: This ongoing study is recruiting in Canadian centers from the year 2005 onwards. The clinical database is constructed using the hospital electronic medical file. In addition, pathological tissues are centralized for molecular and genetic analysis. Results: To this date, 15 patients were included, 12 males and 3 females. Median age was of 49 years old with a disease distribution of Stage II (4), Stage III (3) and Stage IV (4). 8/15 patients developed metastasis after initial cystectomy in the lungs (n = 7), peritoneum (n = 2), lymph nodes (n = 4), and bone (1). Median survival of all patients was 23 months. Metastatic patients after cystectomy had a shorter overall survival (OS) of 21 months compared to non-metastatic patients (OS – not reached). The majority of metastatic patients (6/8) died of their underlying malignancy. Conclusions: This study is an ongoing country-wide effort to expand knowledge of this poorly studied disease. With ongoing accrual, the clinical features will be better defined and further molecular characterization will be performed to better predict response to therapy.