The rapidly expanding group of RB1-deleted soft tissue tumors : an updated review

The classification of soft tissue tumors has evolved considerably in the last decade, largely due to advances in understanding the pathogenetic basis of many of these, sometimes rare, tumors. Deletion of Retinoblastoma 1 (RB1), a well-known tumor suppressor gene, has been implicated in the tumorigenesis of a particular group of soft tissue neoplasms. This group of so-called "RB1-deleted soft tissue tumors" has been rapidly expanding in recent years, currently consisting of spindle cell/pleomorphic lipoma, atypical spindle cell/pleomorphic lipomatous tumor, pleomorphic liposarcoma, myofibroblastoma, cellular angiofibroma, and acral fibromyxoma. Most of these neoplasms, except pleomorphic liposarcoma, are considered benign entities and are mainly described in the older adult population. This article will review the currently known morphological, immunohistochemical, and molecular features of this heterogeneous group of mesenchymal tumors with an emphasis on differential diagnosis

Hoarseness as the initial presentation of multiple myeloma

Key Clinical Message Myeloma of the thyroid cartilage is a rare but important differential diagnosis of a laryngeal mass. Although hoarseness as the initial presenting symptom in multiple myeloma is extremely rare, a clinician should always consider it. Abstract Multiple myeloma (MM) is a malignant plasma cell disorder characterized by an uncontrolled proliferation of monoclonal plasma cells. Although the clinical presentation at diagnosis can be quite variable, thyroid cartilage infiltration in MM is rare. Here we discuss a 65‐year‐old Caucasian male presenting to the ENT doctor with continuous hoarseness for 3 months. The initial clinical examination showed a tangible mass at the left lymph node level II–III. Further examination with fiber‐optic laryngoscopy showed a bulging of the aryepiglottic and ventricular fold. Neck and chest CT scan revealed multiple osteolytic bone lesions in addition to the large lesion in the left thyroid cartilage. Laboratory work‐up, PET‐CT scan and biopsy of the thyroid cartilage were performed and eventually all confirmed the presence of a new diagnosis of IgA kappa MM. The patient was referred to the department of hematology to start with chemotherapy

Effect of neoadjuvant radiation dose and schedule on nodal count and its prognostic impact in stage II–III rectal cancer

It is unknown how neoadjuvant treatment schedule affects lymph node count (LNC) and lymph node ratio (LNR) and how these correlate with overall survival (OS) in rectal cancer (RC). Data were used from the Belgian PROCARE rectal cancer registry on RC patients treated with surgery alone, short-term radiotherapy with immediate surgery (SRT), or chemoradiation with deferred surgery (CRT). The effect of neoadjuvant therapy on LNC was examined using Poisson log-linear analysis. The association of LNC and LNR with overall survival (OS) was studied using Cox proportional hazards models. Data from 4037 patients were available. Compared with surgery alone, LNC was reduced by 12.3 % after SRT and by 31.3 % after CRT (p 0.4 was associated with a significantly worse outcome. Nodal counts are reduced in a schedule-dependent manner by neoadjuvant treatment in RC. After chemoradiation, the LNC does not confer any prognostic information. A LNR of > 0.4 is associated with a significantly worse outcome in stage III disease, regardless of neoadjuvant therapy type

Intracranial myxoid mesenchymal tumor with EWSR1-CREB1 fusion

Myxoid mesenchymal tumor with predilection for intracranial location harboring EWSR1 fusions with CREB family transcription factors is a recently described and exceedingly rare neoplasm. While some debate still exists whether this is a true separate entity or a myxoid variant of angiomatoid fibrous histiocytoma, these tumors still deserve separate attention due to localization, fairly distinct histology and higher incidence in the pediatric population. Data regarding outcome of these neoplasms are still sparse in medical literature. We report a case of an intracranial myxoid tumor with EWSR1-CREB1 fusion in a 14-year-old girl

A rare but devastating cause of twin loss in a near-term pregnancy highlighting the features of severe SARS-CoV-2 placentitis

From the start of the global COVID-19 pandemic, a lot of attention has been focused on how SARS-CoV-2 (severe acute respiratory syndrome coronavirus type 2) impacts pregnancy. The current evidence suggests that pregnant women may be at an increased risk for more severe COVID-19 disease and an increase in maternal death rate has been observed worldwide (1,2)

Do we know what we’re looking at? Increasing our understanding of 18F-FDG distribution in oncology by direct coregistration of histopathology and autoradiography in malignancies of the head and neck

Aim/Introduction: Over the last decades, the use of 18F-FDG has taken a pivotal role in oncological diagnosis, staging and follow-up. While endless research has shown the clinical importance of 18F-FDG, we remain unaware how 18F-FDG behaves in and around human malignancies on a submillimetric scale. Unfortunately, as glucose metabolism is an active process, pathological assessment of relevant protein markers was found to be inadequate in correctly predicting 18F-FDG-uptake. Technological improvements are resulting in increasing spatial resolution for PET-scanners, therefore an urgent understanding of 18F-FDG-distribution on these higher resolutions is required. Materials and Methods: In the current study, we developed a methodology that enabled direct coregistration of the radioactivity-distribution in a surgically resected specimen with histopathological assessment. Patients were injected with 4 MBq/kg of 18F-FDG prior to the initiation of standard of care surgical oncological resection. After resection of the malignancy, the surgical specimen was imaged using preclinical micro-PET and -CT devices with a spatial resolution of 800µm and 50µm, respectively. After imaging, the specimen was freshly sliced into thin slices of approximately 2mm by a pathologist. One slice was snap-frozen and frozen sections were imaged using an autoradiographic flm overnight. Following the imaging, frozen sections were stained with standard hematoxylin and eosin staining. Pathological results were overlayed with the results of 18F-FDG PET/CT and autoradiography to coregister the histopathological and imaging results. Results: We performed this methodology on a total of four patients with cutaneous squamous cell carcinoma (n=2), angiosarcoma (n=1) and thyroid medullary carcinoma (n=1). While the mean time between injection and autoradiography was 3h49, we were able to image sufcient radioactivity to directly coregister the results with the clinical pathological frozen sections. All regions with identifed malignant tissue displayed increased 18F-FDG-uptake. However, uptake was not limited to these regions, as a similar increased uptake was identifed in adjacent benign sebaceous glands. Interestingly, we also identifed a heterogeneous 18F-FDGuptake in separate clusters of malignant tissue, which could partly be explained by the cluster’s amount of peritumoral infammatory cells. Conclusion: To the best of our knowledge, these results are the frst to describe direct coregistration of 18F-FDG with the gold standard of histopathology in any human malignancy. These heterogeneous results display an important diversity in metabolic activity between diferent tumor and peritumoral tissues. The use of this methodology could increase our understanding of radiotracer distribution in human diseases on a previously unprecedented scale in clinical nuclear medicine

Developing a methodology to improve our knowledge on radionuclide distribution in clinical oncology using intraoperative submillimetric PET/CT and autoradiography in patients with malignancies of the head and neck

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