Crowned Dens Syndrome Occurring after Endoscopic Submucosal Dissection for Early Gastric Cancer

Crowned dens syndrome (CDS) is a rare form of pseudogout which causes acute neck pain due to calcium pyrophosphate dehydrate deposition surrounding the odontoid process, commonly causing neck pain with rigidity. While invasive procedures such as surgery are known to present a risk of acute pseudogout, reports of occurrence after endoscopic procedures are scarce. We report the case of a 75-year-old man who presented with sudden neck pain after endoscopic submucosal dissection (ESD) for gastric cancer. He could nod but could not rotate his head. Computed tomography showed calcifications surrounding the odontoid process consistent with CDS. Prolonged dietary restrictions and proton pump inhibitor use following the ESD procedure may have caused hypomagnesemia, a precipitating factor for CDS. We prescribed colchicine 1 mg/day and symptoms resolved completely in 3 days. This is the first report of CDS after ESD. CDS should be included in the differential diagnosis of neck pain after endoscopic procedures.

Calcified chondroid mesenchymal neoplasms with FN1-receptor tyrosine kinase gene fusions including MERTK, TEK, FGFR2, and FGFR1: a molecular and clinicopathologic analysis

Translocations involving FN1 have been described in a variety of neoplasms, which share the presence of cartilage matrix and a variable extent of calcification. Fusions of FN1 to FGFR1 or FGFR2 have been reported in nine soft tissue chondromas, mostly demonstrated indirectly by FISH analysis. Delineation of FN1 fusions with various partner genes will facilitate our understanding of the pathogenesis and diagnostic classification of these neoplasms. In this study, we present molecular, clinical and pathologic features of 9 cartilaginous soft tissue neoplasms showing a predilection for the TMJ region and the extremities. We analyzed for gene fusions with precise breakpoints using targeted RNA-seq with a 115-gene panel, including FN1, FGFR1 and FGFR2. All 9 cases were positive for a gene fusion, including two novel fusions, FN1-MERTK and FN1-TEK, each in one case, recurrent FN1-FGFR2 in 5 cases, FN1-FGFR1 without the Ig3 domain in one case, and FGFR1-PLAG1 in one case. The breakpoints in the 5′ partner gene FN1 ranged from exons 11-48, retaining the domains of signal peptide, FN1, FN2, and/or FN3, while the 3 ′ partner genes retained the trans-membrane domain, tyrosine kinase domains and /or Ig domain. The tumors with FN1-FGFR1, FN1-FGFR2 and FN1-MERTK fusions are generally characterized by nodular/lobular growth of polygonal to stellate cells within a chondroid matrix, often accompanied by various patterns of calcification. These features resemble those as described for the chondroblastoma-like variant of soft tissue chondroma. Additional histologic findings include calcium pyrophosphate dehydrate deposition and features resembling tenosynovial giant cell tumor. Overall, while the tumors from our series show significant morphologic overlap with chondroblastoma-like soft tissue chondroma, we describe novel findings that expand the morphologic spectrum of these neoplasms and have therefore labeled them as calcified chondroid mesenchymal neoplasms. These neoplasms represent a distinct pathologic entity given the presence of recurrent FN1-receptor tyrosine kinase fusions.

Síndrome de la apófisis odontoides coronada, una manifestación inusual de dolor cervical. Presentación de un caso

The calcium pyrophosphate dehydrate (CPPD) or hydroxyapatite (HA) crystal deposition disease can appear in any joint and the accumulation fo crystals in the cervical spine may be painful. Crowned dens syndrome is a rare clinical condition that involves crown-like calcification of the ligaments around the odontoid process. A 70-year-old man presented cervical pain, fever and a headache for over a week, therefore, a neurological condition was suspected. A CT scan revealed lentiform calcifications of the transverse ligament of the atlas. Steroid treatment and a non-steroidal anti-inflammatory diminished the symptoms. A proper clinical history and imaging studies avoid unnecessary procedures and makes it possible to include this entity as a differential diagnosis in acute cervical pain. Key words: Crystal deposition disease; acute cervical pain; crowned dens syndrome; Odontoid process

Extensive Bone Infarctions - an Unexpected Consequence of Corticosteroid Treatment in Idiopatic Polymiositis

Secondary hemochromatosis is a condition generated by defects in the iron metabolism such as elevated intestinal iron absorption and increased iron stores in tissues and organs. The current case report presents a 35-year-old female admitted for inflammatory pain of the left ankle, second and third right MCP joints and right sternoclavicular joint associated with Celsian clinical signs, subcutaneous edema and loss of articular function. The patient is known with beta thalassemia major, secondary hemochromatosis and chronic hepatitis C as a consequence of repeated blood transfusions. We pleaded for the existence of a HC-A associated with microcrystalline calcium pyrophosphate dehydrate crystal deposition (CPPD) disease associated with secondary osteoarthritic changes.

Iron Synovitis in Hemochromatosis Associated Arthropathy-A Great Mimicker

Secondary hemochromatosis is a condition generated by defects in the iron metabolism such as elevated intestinal iron absorption and increased iron stores in tissues and organs. The current case report presents a 35-year-old female admitted for inflammatory pain of the left ankle, second and third right MCP joints and right sternoclavicular joint associated with Celsian clinical signs, subcutaneous edema and loss of articular function. The patient is known with beta thalassemia major, secondary hemochromatosis and chronic hepatitis C as a consequence of repeated blood transfusions. We pleaded for the existence of a HC-A associated with microcrystalline calcium pyrophosphate dehydrate crystal deposition (CPPD) disease associated with secondary osteoarthritic changes.

Neutrophil Extracellular Traps and Microcrystals

Neutrophil extracellular traps represent a fascinating mechanism by which PMNs entrap extracellular microbes. The primary purpose of this innate immune mechanism is thought to localize the infection at an early stage. Interestingly, the ability of different microcrystals to induce NET formation has been recently described. Microcrystals are insoluble crystals with a size of 1–100 micrometers that have different composition and shape. Microcrystals have it in common that they irritate phagocytes including PMNs and typically trigger an inflammatory response. This review is the first to summarize observations with regard to PMN activation and NET release induced by microcrystals. Gout-causing monosodium urate crystals, pseudogout-causing calcium pyrophosphate dehydrate crystals, cholesterol crystals associated with atherosclerosis, silicosis-causing silica crystals, and adjuvant alum crystals are discussed.

Chondrocalcinosis related to familial hypomagnesemia with hypercalciuria and nephrocalcinosis

Calcium pyrophosphate dehydrate (CPPD) crystal deposition disease (also known as chondrocalcinosis, CC) is a rare metabolic arthropathy mostly seen in elderly patients. Chondrocalcinosis may be associated with metabolic diseases such as hypomagnesemia when it occurs in young people. We report here a case with hypomagnesemia due to familial hypomagnesemia with hypercalciuria and nephrocalcinosis (FHHNC) who developed CC during clinical follow-up. To our best knowledge this is the first case of a young patient with CPPD associated with FHHNC.