Sclerosing epithelioid fibrosarcoma of bone with hybrid features: clinicopathologic, radiologic, and molecular analysis of three cases.

Sclerosing epithelioid fibrosarcoma (SEF) occurring as a primary bone tumor is exceptionally uncommon. Even more rare are cases of SEF that show morphologic overlap with low-grade fibromyxoid sarcoma (LGFMS). Such hybrid lesions arising within the bone have only rarely been reported in the literature. Due to their variegated histomorphology and non-specific radiologic features, these tumors may pose diagnostic difficulties. Herein we describe three molecularly confirmed primary bone cases of sclerosing epithelioid fibrosarcoma that demonstrated prominent areas showing the features of LGFMS and with areas resembling so-called hyalinizing spindle cell tumor with giant rosettes (HSCTGR). Two patients were female and one was male aged 26, 47, and 16, respectively. The tumors occurred in the femoral head, clavicle, and temporal bone. Imaging studies demonstrated relatively well-circumscribed radiolucent bone lesions with enhancement on MRI. Cortical breakthrough and soft tissue extension were present in one case. Histologically the tumors all demonstrated hyalinized areas with SEF-like morphology as well as spindled and myxoid areas with LGFMS-like morphology. Two cases demonstrated focal areas with rosette-like architecture as seen in HSCTGR. The tumors were all positive for MUC4 by immunohistochemistry and cytogenetics, fluorescence in-situ hybridization, and next-generation sequencing studies identified EWSR1 gene rearrangements confirming the diagnosis in all three cases.Hybrid SEF is exceedingly rare as a primary bone tumor and can be difficult to distinguish from other low-grade spindled and epithelioid lesions of bone. MUC4 positivity and identification of underlying EWSR1 gene rearrangements help support this diagnosis and exclude other tumor types.

Imaging Spectrum of Calvarial Abnormalities.

Calvarial abnormalities are usually discovered incidentally on radiologic studies or less commonly manifest with symptoms. This narrative review describes the imaging spectrum of the abnormal calvaria. The extent, multiplicity, and other imaging features of calvarial abnormalities can be combined with the clinical information to establish a final diagnosis or at least narrow the differential considerations. Prior trauma (congenital depression, leptomeningeal cysts, posttraumatic osteolysis), surgical intervention (flap osteonecrosis and burr holes), infection, and inflammatory processes (sarcoidosis) can result in focal bone loss, which may also be seen with idiopathic disorders without (bilateral parietal thinning and Gorham disease) or with (Parry-Romberg syndrome) atrophy of the overlying soft tissues. Anatomic variants (arachnoid granulations, venous lakes, parietal foramina) and certain congenital lesions (epidermoid and dermoid cysts, atretic encephalocele, sinus pericranii, and aplasia cutis congenita) manifest as solitary lytic lesions. Other congenital entities (lacunar skull and dysplasia) display a diffuse pattern of skull involvement. Several benign and malignant primary bone tumors involve the calvaria and manifest as lytic, sclerotic, mixed lytic and sclerotic, or thinning lesions, whereas multifocal disease is mainly due to hematologic or secondary malignancies. Metabolic disorders such as rickets, hyperparathyroidism, renal osteodystrophy, acromegaly, and Paget disease involve the calvaria in a more diffuse pattern. Online supplemental material is available for this article. ©RSNA, 2021.

Ipsilateral Osteochondritis Dissecans-like Distal Femoral Lesions in Children with Blount Disease: Prevalence and Associated Findings.

PURPOSE: Our goal was to assess the prevalence of ipsilateral distal femoral osteochondritis dissecans (OCD)-like lesions in children with Blount disease, including factors associated with this finding. MATERIALS AND METHODS: Characteristics of patients with an OCD-like lesion on an imaging study [(X-ray and/or magnetic resonance imaging (MRI)] were compared with those without such a finding. RESULTS: Over a 12-year period, 6/63 (10%) skeletally immature patients (9/87 limbs) with Blount disease had an OCD-like lesion visible on plain radiographs. Based on available MRI, 7/37 (19%) patients or 10/53 (19%) limbs had an OCD-like distal femoral lesion. All lesions were noted in the posterior third of the weight-bearing portion of the medial femoral condyle with intact overlying articular cartilage. All patients with OCD-like lesions were followed for an average of 1.9 years (range: 1-2.6 years), and complete radiographic resolution of lesion was noted in 7/9 limbs (78%). There was no association of the presence of OCD-like lesion with early- vs late-onset disease, gender, age at imaging, laterality, magnitude of deformity [mean mechanical axis deviation (MAD) 63.3 vs 71.9 mm], mean mechanical lateral distal femoral angle (mLDFA; 91.3 vs 89.7°), and mean medial proximal tibial angle (MPTA; 71.7 vs 71.8°). Children with an OCD-like lesion tended to have a lower mean body mass index (BMI; 21 vs 36, p = 0.003). CONCLUSION: The overall prevalence of OCD-like lesions in the medial femoral condyle in children with Blount disease lesions is 10% using plain radiographs and at least 19% on MRI. Based on the numbers available, we were unable to demonstrate any associations between the presence of such lesions and the patient's age, gender, or magnitude of varus deformity. Further research is needed to fully ascertain the aetiology and natural history of these benign appearing osteochondral imaging findings in children with Blount disease. Our current data support that these lesions do resolve with time and that no surgical intervention targeted at the femoral OCD-like lesion is warranted. LEVEL OF EVIDENCE: Diagnostic study Level III. HOW TO CITE THIS ARTICLE: Edobor-Osula F, Wenokor C, Bloom T, et al. Ipsilateral Osteochondritis Dissecans-like Distal Femoral Lesions in Children with Blount Disease: Prevalence and Associated Findings. Strategies Trauma Limb Reconstr 2019;14(3):121-125.

Glomangiomatosis: a case report.

Glomangiomatosis is a benign vascular variant of a glomus tumor. The lesion represents only 5% of glomus tumors with unusual or atypical features and even fewer glomus tumors with typical features. The lesions are most commonly located in the distal extremities and are multiple, deep, extensive, and often pain producing. They develop from small arteriovenous anastamoses and are most often identified in young adults. The lesions may recur. We present a case of a 33-year-old male who presented clinically with multiple slowly enlarging masses of the leg over a 5-year period, of which one caused significant pain. One of the lesions was reported to be present at birth. Imaging studies were performed and were suggestive of neurofibromatosis. Biopsies from multiple lesions led to an eventual diagnosis of multiple glomangiomatosis.

Leiomyoma of deep soft tissue mimicking calcific myonecrosis.

Leiomyoma of deep soft tissue is an unusual entity reported in less than 60 cases in the English literature. The lesion is now accepted as a rare neoplasm. Leiomyomata of deep soft tissue have been divided into 2 groups, those occurring primarily in women in the retroperitoneum, histologically similar to leiomyomata of the uterus, and those occurring equally in both sexes in the somatic deep soft tissues. Irrespective of location, these lesions can demonstrate calcification, and even less commonly ossification. We report a unique case of a leiomyoma of deep soft tissue that mimicked the clinical and radiographic features of calcific myonecrosis, also a rare mass forming soft-tissue lesion. Clinical and radiographic information are often critical in the diagnosis of soft-tissue lesions; however, this case demonstrates that a lesion can deviate from the standard clinical and radiographic interpretations most commonly attributed to it.

Giant cell tumor of bone with secondary aneurysmal bone cyst-like change producing ß-human chorionic gonadotropin.

Giant cell tumor of bone is a benign, locally aggressive neoplasm that is composed of sheets of neoplastic mononuclear cells interspersed amongst non-neoplastic, uniformly distributed, osteoclast-like giant cells. They represent approximately 4-5% of primary bone tumors. Rarely, bone tumors have been noted to produce human chorionic gonadotropin, a finding most often reported in osteosarcoma. We present the case of a young woman who presented with a low-level human chorionic gonadotropin level which, after resection of her recurrent giant cell tumor of bone with secondary aneurysmal bone cyst-like change, became undetectable in her blood. Furthermore, cells within the aneurysmal bone cyst component were immunohistochemically positive for ß-human chorionic gonadotropin. This is the first report of such a finding in the literature.

Intra-articular morphology of the knee joint in children with Blount disease: a case-control study using MRI.

BACKGROUND: The clinical and radiographic abnormalities of the lower limb in children with Blount disease are well known. However, there is controversy regarding the intra-articular morphologic changes in the involved knee joint. The purpose of our study was to evaluate the meniscal and articular surface morphology in children with Blount disease with use of magnetic resonance imaging and to compare these findings with similar measurements in a control group. METHODS: Preoperative magnetic resonance imaging scans of the knee of children with Blount disease were compared with those of a control group. Coronal and sagittal morphometric parameters including the height and width of the menisci, thickness of the unossified proximal tibial epiphysis, tibial condylar and meniscal inclination, and presence of signal changes and/or tears of the menisci were documented. RESULTS: Twenty-six children (thirty-three) knees with Blount disease (mean age, 10.5 years) and twenty children without Blount disease (mean age, 9.6 years) were identified. The midcoronal medial meniscal height and width were greater in the Blount disease group (p < 0.0001). Abnormal signal changes were noted in the medial meniscus in twelve limbs (39%) in the Blount group and one limb (5%) in the control group (p = 0.008). The mean thickness of the unossified cartilage of the proximal medial tibial epiphysis was also greater in the Blount disease group (p = 0.0005). The morphology of the menisci and unossified cartilage in the lateral compartment did not differ between the two groups. The mean tibial condylar and bimeniscal inclinations in the coronal and sagittal planes were also similar in both groups. In a multivariate regression analysis, only body mass index correlated with the height of the medial meniscus in the coronal plane (p = 0.0035) and of the posterior horn of the medial meniscus in the sagittal plane (p = 0.0037) in children with Blount disease. CONCLUSIONS: Children with Blount disease have increased thickness of the chondroepiphysis of the proximal medial aspect of the tibia, increased height and width of the medial meniscus, and greater frequency of abnormal signals in the posterior horn of the medial meniscus. These morphologic changes may compensate for the diminished height of the ossified portion of the medial proximal aspect of the tibia in patients with Blount disease.

Acute symptomatic intervertebral disk calcification in a child with retropharyngeal edema: computed tomography and magnetic resonance findings.

Intervertebral disk calcification in children is an uncommon self-limiting disease, which can cause symptoms like neck pain or torticollis, and can be treated with conservative management. The calcified disk material can herniate anteriorly, inducing dysphagia, or herniate posteriorly, causing neurologic symptoms secondary to spinal cord compression. We report computed tomography and magnetic resonance findings of a symptomatic intervertebral disk calcification at the C2-3 level with retropharyngeal edema caused by anterior herniation of calcified material in a 7-year-old boy.

Mycobacterium marinum Hand Infection in a "Sushi Chef".

OBJECTIVE: We present the case of a sushi chef with pain and swelling of his index finger and wrist for a year, unresponsive to antibiotics. METHODS: Biopsy showed a xanthogranulomatous reaction and positive culture results for Mycobacterium marinum. RESULTS: He was treated with minocycline, clarithromycin, and ethambutol. In addition, he underwent radical synovectomy of the lesion. CONCLUSION: The combined medical and surgical approach resulted in a positive outcome.

Intradiscal pressure monitoring in the cervical spine.

BACKGROUND: Discography has been widely used in the lumbar and cervical spine as a diagnostic tool to identify sources of discogenic pain that may be amenable to surgical treatment. Discography in the cervical spine is currently performed without the benefit of pressure monitoring, and corresponding pressure parameters have not been determined. OBJECTIVE: The purpose of this study was to develop the framework for intradiscal pressure monitoring in the cervical spine and the basis for a pressure curve that will reflect clinically significant cervical internal disc disruption. We also sought to determine whether there is any pressure increase in adjacent discs during cervical discography that might result in false-positive diagnosis during in-vivo discography. An additional goal was to establish safe upper parameters for infusion volume and intradiscal pressure in the cervical spine. DESIGN: Investigation of fresh-frozen discs in the cervical spine. METHODS: Investigated were 26 discs in 5 fresh-frozen cadaveric cervical spines aged 45 to 68 with no prior history of cervical spine disease. A T2 MRI was performed on each specimen and radiographically abnormal discs were noted. Pressure-controlled, fluoroscopically guided discography was performed on each level using a right lateral approach. Opening pressure, rupture pressure, volume infused, and location of rupture were recorded. Pressures were simultaneously recorded at each adjacent disc level using additional pressure monitors and identical needle placement. Immediately following discography, CT was performed on each specimen according to the discography protocol. RESULTS: Twenty-six discs C2-3 to C7-T1 were grossly intact for evaluation. The median opening pressure was 30 psi (range 14-101 psi). Two discs did not rupture and were pressurized to 367 psi. In 24 discs, the median intradiscal rupture pressure was 40 psi (range 14-171 psi). The median volume infused at rupture was 0.5 ml (range 0.25-1.0 ml). When grouped, the median intradiscal rupture pressure in the C2-3, C3-4, and C7-T1 discs was 53 psi (range 16-171 psi) compared to 36.5 psi (range 14-150 psi) in the C4-5, C5-6, and C6-7 discs (p=0.18). There was no measurable pressure change in any of the 30 adjacent disc levels evaluated. CONCLUSION: In the cervical spine, iatrogenic disc injury may be caused at significantly lower pressures and volumes infused than in the lumbar spine. There was no measurable pressure change in any of the adjacent disc levels evaluated at maximum intradiscal pressurization. Further cadaveric testing will be necessary to develop parameters for intradiscal pressure monitoring in the cervical spine.