Soft Tissue Extension Research Articles

Current status and unanswered questions on the use of Denosumab in giant cell tumor of bone.

Denosumab is a monoclonal antibody to RANK ligand approved for use in giant cell tumour (GCT) of bone. Due to its efficacy, Denosumab is recommended as the first option in inoperable or metastatic GCT. Denosumab has also been used pre-operatively to downstage tumours with large soft tissue extension to allow for less morbid surgery. The role of Denosumab for conventional limb GCT of bone is yet to be defined. Further studies are required to determine whether local recurrence rates will be decreased with the adjuvant use of Denosumab along with surgery. The long term use and toxicity of this agent is unknown as is the proportion of patients with primary or secondary resistance. It is advised that complicated cases of GCT requiring Denosumab treatment should be referred and followed up at expert centres. Collaborative studies involving further clinical trials and rigorous data collection are strongly recommended to identify the optimum use of this drug.

Aspergillus arthritis: analysis of clinical manifestations, diagnosis, and treatment of 31 reported cases.

Aspergillus arthritis is a debilitating form of invasive aspergillosis. Little is known about its epidemiology, clinical manifestations, laboratory features, treatment, and prognosis. Cases of Aspergillus arthritis were reviewed in the English literature from 1967 through 2015 for variables of arthritis with Aspergillus spp. recovered from joint and/or adjacent bone, underlying conditions, symptoms, signs, inflammatory biomarkers, diagnostic imaging, management, and outcome. Among 31 evaluable cases, 87% were males and 13% pediatric. Median age was 50 y (range 1-83 y). Seventeen (55%) patients were immunosuppressed with such conditions as hematological malignancies (26%), corticosteroids (39%), and/or transplantation (26%). Approximately one-half (52%) of patients had hematogenous seeding of the joint, and more than 80% had de novo infection with no prior antifungal therapy. Oligoarticular infection (2-3 joints) occurred in 45% and contiguous osteomyelitis was present in 61%. Clinical manifestations included pain (87%), edema (26%), and limited function (23%), with knees (35%), intervertebral discs (26%), and hips (16%) being most commonly infected. Aspergillus fumigatus constituted 77% of cases followed by Aspergillus flavus in 13%, Aspergillus niger in 3%, and not specified in 7%. Median ESR was 90mm/hr and median CRP was 3.6mg/dl. Median synovial fluid WBC was 17,200/μL (7,300-128,000) with 72% PMNs (range 61-92). Osteolysis occurred in 35%, and soft-tissue extension 47%. Nineteen patients (61%) were managed with combined medical and surgical therapy, 10 (32%) with medical therapy only, and 2 (6%) surgery only. Amphotericin B and itraconazole were the most frequently used agents with median duration of therapy of 219 days (range 30-545). Surgical interventions included debridement in 61%, drainage 19%, and amputation 6%. Complete or partial response was achieved in 71% and relapse occurred in 16%. Medical therapy was reinstituted with successful outcome in these patients. Overall survival was 65%. Aspergillus arthritis mainly develops as a de novo infection involving knees and intervertebral disks in immunocompromised patients with localizing symptoms. Contiguous osteomyelitis is frequently observed. Diagnosis is established by synovial fluid culture. Aspergillus arthritis is therapeutically challenging with most patients undergoing surgery and protracted antifungal therapy.

Muscle lengthening and structural changes in the muscle-tendon complex of triceps surae after 1 year of rehabilitation including a daily self-stretch program in patients with chronic hemiparesis

To explore changes in muscle architectural parameters of the muscle-tendon unit of soleus and medial gastrocnemius (GM) in patients with chronic hemiparesis, after 1 year of rehabilitation. In this prospective study, 20 chronic hemiparetic patients (8 W, mean age: 56 [12], time since lesion 9 [8]) were evaluated. Muscle architectural parameters including muscle fascicle length, pennation angle, thickness, tendon and muscle belly lengths were evaluated in vivo using ultrasonography in passive condition (verified by electromyographic recording) in a seated patient with ankle, knee and hip on the paretic side at 90°. Following the biomechanical analysis, each patient benefited from the Five Step Assessment (FSA), involving the measure of XV1, angle of arrest at slow and strong stretch, which estimates soft tissue extensibility around each muscle. Four muscles of interest in the lower limb were selected for this measure: soleus (XV1Sol), medial gastro-soleus complex (XV1GSC), gluteus maximus (XV1GM) and rectus femoris (XV1RF). Biomechanical and clinical analyses were performed at the beginning and after 1 year of treatment. Two treatments were implemented: – conventional rehabilitation (50%); – guided Self-rehabilitation contract (GSC, 50%). Patients in the GSC group were prescribed a daily self-stretch program with static (> 10 min/muscle/day) and eccentric stretch. Changes in architectural parameters and clinical muscle extensibility of overall patients before and after 1 year of rehabilitation were analyzed (Student's). After 1 year, muscle fascicle length and thickness increased respectively by 6.1 mm (14.2%, P = 0.05), 1.8 mm (13.6%, P = 0.06) in soleus, and by 2.9 mm (9.1%, P = 0.04) and 1.7 mm (13.2%, P = 0.03) in MG. Muscle belly length of MG increased by 2.9 cm (17%, P = 0.0001) and its tendon length decreased by 0.82 cm (5%, P = 0.04). XV1GSC increased by 4.8° (3.3%, P = 0.07), XV1GM by 4.1° (3.3%, P = 0.0004) and XV1RF by 7.2° (6%, P = 0.02). Stretch of soleus and medial gastrocnemius practiced over the long term in patients with spastic hemiparesis allowed structural changes, increasing muscle fascicle length and thickness. Muscle belly length of medial gastrocnemius also increased significantly while its tendon length decreased, suggesting that the tendon may adapt its length to the muscle length changes.

Non-Hodgkin’s Lymphoma of Talus: A Case Report

Introduction: Primary non-Hodgkin’s lymphoma of the bone (PLB) represents about 3% - 5% of all extranodal non-Hodgkin’s lymphoma (NHL) cases and 7% of primary bone tumors. It may occur at any age. The peak incidence for PLB is in the fifth and sixth decades of life, and it has a slight male predominance. The most commonly affected sites are the long bones. A palpable mass due to soft tissue extension of the bony disease is seen in almost half of all cases. Case Presentation: We report the case of an NHL (diffuse large B-cell lymphoma) in the talus bone of a 17-year-old boy who was referred to our center in 2011 - 2012, which had an odd presentation. The patient’s first diagnosis was a simple ankle sprain, and he underwent conservative treatment. Given the patient’s deteriorating symptoms, further paraclinical evaluations were conducted, and fracture of the talus was diagnosed, which was thought to be a cause of further osteonecrosis. Finally, because of lack of pain relief and due to MRI images, the suspicion of malignancy was raised. Open biopsy of the talus showed NHL (diffuse large B-cell lymphoma). Conclusions: The present case is interesting because it expresses how such lesions can be met with diagnostic confusion.

The Prognostic Value of Serum Biomarkers in Localized Bone Sarcoma

OBJECTIVE: Certain biomarkers such as the C-reactive protein, serum albumin, and the neutrophils to lymphocyte ratio are of prognostic significance regarding survival in different types of cancers. Data from sarcoma patients are sparse and mainly derived from soft tissue sarcoma and/or metastatic cases. Adjusting for confounders such as comorbidity and age is an essential safeguard against erroneous conclusions regarding the possible prognostic value of these biomarkers. The aim of this study was to assess the prognostic value of a battery of pretreatment biomarkers in the serum of patients with localized bone sarcomas and to adjust for potential confounders. MATERIAL AND METHODS: All patients diagnosed with localized intermediate and high-grade bone sarcoma during 1994 to 2008 were extracted from the Aarhus Sarcoma Registry. The serum levels of albumin, C-reactive protein, hemoglobin, neutrophils, lymphocytes, and sodium were collected from the patient records. The prognostic values of overall and disease-specific mortality were tested for each individual biomarker as well as for the Glasgow prognostic score (GPS) and for a new composite score incorporating five biomarkers (Aarhus composite biomarker score: ACBS). Adjustments were made for comorbidity as well as other possible prognostic factors, such as size, histological type, margin, chemotherapy, and soft tissue extension, using the Cox proportional hazard model. RESULTS: A total of 172 patients with high- or intermediate-grade localized bone sarcoma were included. Of these patients, 63 were diagnosed with chondrosarcoma and 109 patients with Ewing/osteosarcoma. The median age was 55 years for chondrosarcoma and 19 years for Ewing/osteosarcoma patients. The overall 5-year mortality was 31% [95% confidence interval (CI): 21-44] and 41% (95% CI: 33-51), whereas the 5-year disease-specific mortality was 21% (95% CI: 12-34) and 39% (95% CI: 31-49) for chondrosarcoma and Ewing/osteosarcoma, respectively. Comorbidities were present in 12% of the Ewing/osteosarcoma patients and in 24% of the chondrosarcoma patients. After adjustment for comorbidity and other confounders, it was found that elevated levels of CRP, low hemoglobin, low sodium, high GPS, and high ACBS were associated with increased overall mortality. Furthermore, elevated levels of CRP, low hemoglobin, high GPS, and high ACBS were associated with increased disease-specific mortality. CONCLUSION: Elevated levels of CRP, low hemoglobin, high GPS, and high ACBS were all independent prognostic factors for both overall and disease-specific mortality. ACBS is a new three-level score of five biomarkers, but its value has to be confirmed in an independent data set.

Single-Fraction Spine Stereotactic Body Radiation Therapy for the Treatment of Chordoma

Chordoma is a radioresistant tumor that presents a therapeutic challenge with spine involvement, as high doses of radiation are needed for local control while limiting dose to the spinal cord. The purpose of this study is to determine the efficacy and safety of single-fraction spine stereotactic body radiation therapy for the treatment of spine chordoma. A retrospective review of our institutional database from 2006 to 2013 identified 8 patients (12 cases) with chordoma of the spine who were treated with spine stereotactic body radiation therapy. Surgical resection was performed in 7 of the 12 cases. The treatment volume was defined by the bony vertebral level of the tumor along with soft tissue extension appreciated on magnetic resonance imaging fusion. Medical records and imaging were assessed for pain relief and local control. Treatment toxicity was evaluated using Common Terminology Criteria for Adverse Events version 4.0. Median age was 59 years (range, 17-91). Median target volume was 48 cm3 (1-304), and median prescription dose was 16 Gy (11-16). Median conformality index was 1.44 (1.14-3.21), and homogeneity index was 1.12 (1.05-1.19). With a median follow-up time of 9.7 months (.5-84), local control was achieved in 75% of the cases treated. One patient developed limited grade 2 spinal cord myelopathy that resolved with steroids. There were no other treatment toxicities from spine stereotactic body radiation therapy. Single-fraction spine stereotactic body radiation therapy can be safely delivered to treat chordoma of the spine with the potential to improve pain symptoms. Although the early data are suggestive, long-term follow-up with more patients is necessary to determine the efficacy of spine stereotactic body radiation therapy in the treatment of chordoma of the spine.

Surgery methods and soft tissue extension are the potential risk factors of local recurrence in giant cell tumor of bone.

BackgroundVarious treatments of giant cell tumor of bone (GCTB) included in curettages and resections and with adjuvant are exerted, but the best treatment is controversial. The aim of the study was the identification of individual risk factors after various treatments in GCTB.MethodsA total of 179 patients treated for GCTB between 1998 and 2010 were concluded in the retrospective study. All patients were treated with intralesional curettage, extensive curettage, or wide resection. Mean follow-up was 60.2 ± 18.7 months (36~112 months). Age, gender, tumor location, Campanacci grade, soft tissue extension, pathological features, and surgical methods were performed to univariate Kaplan-Meier survival analysis and multivariate Cox regression analysis.ResultsThe local recurrence rates of intralesional curettage (41.9 %) and extensive curettage (19.0 %) were significantly higher than that of wide resection (7.7 %). The higher risk of local recurrence was found for soft tissue extension (hazard = 7.921, 95 % CI 1.107~56.671), compared with no statistical significances between gender, location, Campanacci grade, pathologic fracture, and local recurrences, which were shown by Kaplan-Meier analysis. However, recurrence-free survival (RFS) of patients younger than 30 was significantly lower than that of patients older than 30. The RFS of pathologic fracture patients with soft tissue extension was significantly lower than that of pathologic fracture patients without soft tissue extension. Multivariate Cox regression analysis indicated that the independent variable that contributed to recurrence-free survival was soft tissue extension and surgical methods. The RFS of extensive curettage had no statistically significant difference with wide resection and was significantly higher than that of intralesional curettage. Use of high-speed burring and bone cement significantly decreased the local recurrence rate.ConclusionsAge (below 30 years), gender, tumor location, Campanacci grade, and pathologic fracture have no statistically significant influence on local recurrences. Soft tissue extension and intralesional curettage of surgical methods increased the RFS. The results of the present study suggested that compared with curettage and wide section, treatment of GCTB by extensive curettage could provide the favorable local control and functional recovery.Electronic supplementary materialThe online version of this article (doi:10.1186/s12957-016-0871-z) contains supplementary material, which is available to authorized users.

Imaging features of primary tumors of the spine: A pictorial essay.

Primary tumors of spine are rare accounting for less than 5% of new bone tumors diagnosed every year. These tumors may exhibit characteristic imaging features that can help in early diagnosis and improved prognosis. Plasmacytoma/multiple myeloma and lymphoproliferative tumors are the most common malignant primary spinal tumors. Hemangioma is the most common benign tumor of the spine. Computed tomography is useful to assess tumor matrix and osseous change. Magnetic resonance is useful to study associated soft tissue extension, marrow infiltration, and intraspinal extension. Confusing one tumor with the other based on only imaging findings is not uncommon. However, radiologic manifestations of these tumors need to be correlated with the age, sex, location, and presentation to arrive at a close clinical diagnosis.

The epidemiological and clinical features of primary giant cell tumor around the knee: A report from the multicenter retrospective study in china

ObjectivesWe aimed to determine the demographic characteristics of giant cell tumor around the knee in China. MethodsBetween March 2000 and June 2014, patients with primary giant cell tumor around the knee were recruited from 6 institutions located in different regions of China, and were reviewed retrospectively the clinical features according to gender and age. Results334 qualified patients were included in this study. The sex ratio was 1.14:1 (178/156), with mean ages of 36.9 years in men and 33.1 years in women, constituting a significant difference (P=0.007). The prevalence of pathological fracture was 32.9% overall (28.7% in men and 37.8% in women). The prevalence of simple fracture was significantly higher in women (26.3%) than in men (15.2%), P=0.042. Tumor location and staging did not differ significantly according to sex (P>0.05). However, comparing with >40 years old, those patients aged ≤40 were more likely to have a right knee tumor (56.7% vs. 44.7%, P=0.042), less likely to have Enneking stage 3 disease (18.6% vs. 35.0%, P=0.005), and less likely to have both soft-tissue extension and a mass (18.6% vs. 34.0%, P=0.009). ConclusionsGiant cell tumor around the knee was more common in men than in women, although female patients were younger on average. Further, cases among patients ≤40 years old were observed to be milder than cases among older patients. The results suggest that efficient treatment and preservation of function should both be valued for young patients with giant cell tumor around the knee.

Role of Percutaneous Image Guided Biopsy in Spinal Lesions: Adequacy and Correlation with MRI Findings

Although, MRI has increased our understanding of spinal pathologies, accurate diagnosis of spinal lesions need biopsy, so that early treatment can be initiated. To evaluate the accuracy of biopsy, safety and yield of percutaneously done image guided spinal biopsy using a large bore needle and correlate between MRI findings and biopsy as well as the importance of various MRI findings in establishing the diagnosis. All spinal lesions after clinical and MRI evaluation were subjected to Jamshidi Needle biopsy using 11 gauge needles. Biopsy material was sent for culture/sensitivity, AFB smear and histopathological examination. The outcome assessment included percentage of patients in whom diagnosis was changed after biopsy, yield in biopsy and complications of biopsy. MRI findings, biopsy findings and final diagnosis were correlated to know the sensitivity and specificity of MRI and biopsy diagnosis. Logistic regression analysis was used to study the importance of each of MRI findings in making a diagnosis. Forty five patients with spinal lesions underwent biopsy using an 11 gauge Jamshidi needle. Initial biopsy was inconclusive in 4 patients giving a positive yield in about 91.2% of cases and a repeat biopsy ensured conclusive report in all cases. Following biopsy there was a change in diagnosis in 8% cases. MRI showed sensitivity of 85.71% and specificity of 93.54% for the diagnosis of malignancy and sensitivity of 85.71% and specificity of 86.48% for the diagnosis of tuberculosis. In contrast, initial biopsy had sensitivity of 92.85% and specificity of 100% for the diagnosis of malignancy and sensitivity of 71.42% and specificity of 100 % for the diagnosis of infection. Logistic regression analysis showed good correlation between malignancy and posterior bugle in the vertebral body in the absence of a fracture (p = 0.007), involvement of pedicles and posterior elements (p = 0.001) and soft tissue extension (p = 0.002); there was good correlation between infection and epidural abscess (p<0.001) as well as paradiscal involvement (p<0.001). Image guided biopsy done with good technique helps in accuracy of diagnosis thus ensuring the correct treatment at the earliest and has minimal complications. This study also shows that presence of epidural abscess and paradiscal involvement in MRI are highly suggestive of infection, while pedicle involvement and posterior bulge of vertebral body before the onset of pathological fracture are suggestive of malignancy, but all spinal lesions should be biopsied to confirm the diagnosis.

Candida Arthritis: Analysis of 112 Pediatric and Adult Cases.

Background. Candida arthritis is a debilitating form of deeply invasive candidiasis. However, its epidemiology, clinical manifestations, management, and outcome are not well understood.Methods. Cases of Candida arthritis were reviewed from 1967 through 2014. Variables included Candida spp in joint and/or adjacent bone, underlying conditions, clinical manifestations, inflammatory biomarkers, diagnostic imaging, management, and outcome.Results. Among 112 evaluable cases, 62% were males and 36% were pediatric. Median age was 40 years (range, <1–84 years). Most patients (65%) were not pharmacologically immunosuppressed. Polyarticular infection (≥3 joints) occurred in 31% of cases. Clinical manifestations included pain (82%), edema (71%), limited function (39%), and erythema (22%) with knees (75%) and hips (15%) most commonly infected. Median erythrocyte sedimentation rate was 62 mm/hr (10–141) and C reactive protein 26 mg/dL (0.5–95). Synovial fluid median white blood cell count was 27 500/µL (range, 100–220 000/µL) with 90% polymorphonuclear neutrophils (range, 24–98). Adjacent osteomyelitis was present in 30% of cases. Candida albicans constituted 63%, Candida tropicalis 14%, and Candida parapsilosis 11%. Most cases (66%) arose de novo, whereas 34% emerged during antifungal therapy. Osteolysis occurred in 42%, joint-effusion in 31%, and soft tissue extension in 21%. Amphotericin and fluconazole were the most commonly used agents. Surgical interventions included debridement in 25%, irrigation 10%, and drainage 12%. Complete or partial response was achieved in 96% and relapse in 16%.Conclusion. Candida arthritis mainly emerges as a de novo infection in usually non-immunosuppressed patients with hips and knees being most commonly infected. Localizing symptoms are frequent, and the most common etiologic agents are C albicans, C tropicalis, and C parapsilosis. Management of Candida arthritis remains challenging with a clear risk of relapse, despite antifungal therapy.

Use of warm Ringer's lactate solution in the management of locally advanced giant cell tumor of bone.

This study was conducted to discover the effectiveness and safety of using warm Ringer's lactate solution (RLS) as a local treatment in the management of locally advanced giant cell tumor of bone with marked soft tissue invasion, including nearby neurovascular bundles. This was a longitudinal cohort study with an average follow-up period of 4.6 ± 0.3 years, ranging from 4.2 to 5.9 years. There were 21 patients (9 male and 12 female), with the ages of subjects ranging from 12 to 64 years. Eight patients (38 %) were tumor recurrence cases. Pathological fracture was found in 15 patients (71 %). After extended curettage, warm RLS (50 °C) was locally applied for 20 min. Bone stabilization and reconstruction were then performed. All patients survived the operation. No additional neurovascular injury resulting from the use of warm RLS was found. Patients who had neurological deficit before the operation experienced significant improvement in motor and sensory function during the follow-up period. Complication was found in one patient (5 %). Two patients (9.5 %), had tumor recurrence and 19 patients (90.5 %) were tumor-free with good to acceptable function. Use of warm Ringer's lactate solution as an adjunctive local treatment during intra-lesional curettage of giant cell tumor with locally soft tissue extension was found to be safe with relatively low recurrence rate. However, additional studies to identify the optimum thermoablation dose at each part of the body should be undertaken before this technique can be used as a standard treatment.

Visual Vignette

Case Presentation: A 73-year-old man with a history of type 2 diabetes, coronary artery disease, 2-cm right thyroid nodule, and untreated subclinical hypothyroidism presents to his cardiologist for progressive shortness of breath. Chest X-ray and echocardiogram were normal. Thyroid-stimulating hormone (TSH) was 4.42 mIU/L (normal range, 0.34 to 3.0 mIU/L). Thyroid ultrasound showed an enlarged heterogeneous right thyroid lobe measuring 6.8 × 3.1 × 3.3 cm with a 2.1 × 1.8 cm solid hypoechoic irregular mid/inferior nodule with soft-tissue extension from the lateral mid-right thyroid lobe to the right jugular vein, with possible invasion in the jugular vein medial wall (Fig. 1). Neck computed tomography scan demonstrated an enlarged right thyroid lobe, with poor definition and demarcation with the adjacent esophagus and fat, adjacent enlarged pathologic lymph nodes, and irregular thyroid cartilage (Fig. 2). What is the diagnosis? Fig. 2 View Large Image Figure Viewer

P78 – 2554: Farber disease in a child with a novel homozygous c.92G&gt;T mutation

Objective Farber disease is an extremely rare autosomal recessive lysosomal storage disease marked by a deficiency in enzyme acid ceramidase that causes an accumulation of fatty material lipids leading to abnormalities in the joints, liver, throat, tissues and central nervous system. To date, less than 25 distinct mutations have been identified in farber patients, but no homozygous c.92G>T mutation have yet been reported. Methods A five-month-old male presented at our institution with a hoarse cry, irritability and weak head control. His physical examination revealed coarse facial appearance, joint stiffness, axial hypotonia and hepatomegaly. Ophtalmoscopic examination revealed macular cherry-red spots. Serebral magnetic resonance imaging (MRI) was consistent with communicating hydrocephalus at six months of age. Serebral and servical spinal MRI revealed noduler soft tissue extension that involved cervicomeduller junction at the atlantoaxial level at eight months of age. There were multiple subcutaneous nodules measuring 0.5 to 1 cm in diameter over the interphalangeal joints of the hand appeared at the age of 16 months. There were no evidence of urine mucopolysaccharide or oligosaccharide screen. Urinary sialic acid were normal. Through the analysis of lysosomal enzymes activities revealed normal. The plasma chitotriosidase was raised 3654umol/lhr, (normal of 4–120). The diagnosis of Farber lipogranulomatosis was suggested and gene sequencing of the ASAH1 gene confirmed the diagnosis of Farber disease Results A homozygous c.92G> T mutation was found in our patient. Conclusion Farber lipogranulomatosis is a rare autosomal recessive lysosomal storage disorder caused by mutations in the ASAH1 gene. To the best of our knowledge, this is the first report of a novel homozygous c.92G> T mutation in ASAH1 gene which is associated with Farber disease

Current trends in mini-invasive management of spine metastases.

The spine is a frequent localization of primary tumours or metastasis involving posterior arch, pedicles and vertebra body, and often causing unsustainable pain. The management of spinal metastasis remains complex, including medical therapy (corticosteroids, chemotherapy), radiotherapy and surgical treatment, or the recent percutaneous mini-invasive approach. The target of all these treatments is to improve the quality of life of patients affected by this type of lesion. Diagnosis of spinal metastasis and then its treatment should be based on the combination of different elements: clinical evaluation, CT, MRI and nuclear medicine patterns, considering the age of the patient, known primary tumour, location of the lesions, single/multiple lesions, pattern of morphology (border, matrix, expansile character, soft tissue extension), density or signal intensity, oncologic instability and expectancy of life. The percutaneous mini-invasive approach for patients affected by secondary lesions involving the spine has as treatment goal of: (1) pain relief improving the quality of life; (2) stability treatment re-establishing the spinal biomechanics, alterated by bone destruction or deformity, preventing pathological fracture; and (3) an anti-neoplastic effect. The aim of this paper is to provide a comprehensive diagnostic and percutaneous approach to the bone metastatic spine lesions, identifying which metamer should be treated to improve patient quality of life, showing the importance of a multi-disciplinary approach to this problem.

Epithelioid hemangioma of the spine: an uncommon cause of spinal cord compression

A 65-year-old female presents to the emergency room with worsening mid-back pain, rapidly progressive numbness starting from below the breasts (T5 sensory level), and bilateral lower extremity weakness. She reported increasing difficulty ambulating and was unsteady on her feet. She had radiating pain into her anterior thighs, occasionally extending into her calves, right greater than left and had new-onset loss of sphincter, bowel and bladder control. Her focused neurologic exam showed quadriceps strength of 4?/5 on the left and 4-/5 on the right. Ankle dorsiflexion was 4-/5 bilaterally. She had a known history of midthoracic back pain for the preceding 4 months. She denied any history of remote or recent spinal trauma. Spinal cord compression was suspected and the initial CT imaging revealed a large lytic lesion at the T7–8 level with involvement of the corresponding ribs. Coronal imaging revealed destruction, expansion and trabeculation of the vertebral bodies of T7–9 and loss of vertebral body height. On axial imaging, there was paravertebral soft tissue extension with central canal narrowing (Figs. 1, 2, 3). An initial differential diagnosis of vascular tumor versus malignancy was considered and a CT-guided biopsy with angiographic embolization of feeding vessels was planned urgently, followed by definitive surgical intervention. She underwent an uneventful biopsy and successful Onyx-18 (eV3, Irvine, California, USA) embolization of feeding vessels (Fig. 3). The post-embolization surgical procedure involved laminectomies from T6 to T9, T7–8 vertebroplasty and partial transpedicular vertebrectomies followed by tumor resection with T4–T11 arthrodesis and posterior spinal fusion (Figs. 4, 5). She regained motor strength within 48 h and near complete resolution of numbness postoperatively. Final pathology was consistent with epithelioid hemangioma (EH).

Correlation of high-resolution computed tomography temporal bone findings with intra-operative findings in patients with cholesteatoma

Aim: To evaluate the role of high-resolution computed tomography (HRCT) temporal bone in patients with active squamosal chronic otitis media (COM). Materials and Methods: Totally, 47 patients with active squamosal COM underwent preoperative HRCT temporal bone followed by surgery. Their intra-operative findings were considered as the gold standard and were compared and correlated with the radiological findings, to calculate the sensitivity, specificity, positive and negative predictive value of HRCT temporal bone. Results: According to the present study, HRCT was highly sensitive for detecting mastoid pneumatization, soft tissue extension, ossicular erosion, tegmen and sigmoid sinus erosion, and less sensitive for fallopian canal erosion and lateral sinus fistula. It was specific for all these parameters. Conclusion: The present study concludes that HRCT can be recommended not only in cases suspected with potential complications but also in all cases of COM to know the extent of disease, varied pneumatization, and the presence of anatomical variations, which should alert the clinician and guide in surgical approach and treatment plan.

男児の下顎骨に生じたランゲルハンス細胞組織球症の1例

Langerhans’-cell histiocytosis （LCH） is caused by monoclonal proliferation of Langerhans’ cells, a type of antigen-presenting cell, and affects multiple organs. We report a case of LCH arising in the mandible of a young boy.The patient was an 11-year-old boy who referred to our hospital because of swelling of the right cheek. Computed tomographic examination showed a destructive bony lesion extending from the right mandibular body to the mandibular angle, with resorption of the buccal and lingual cortical plate. Gadolinium-enhanced T1-weighted magnetic resonance imaging showed contrast enhancement in a large portion of the right mandibular body, with soft tissue extension. A biopsy of the lesion was performed with the patient under general anesthesia because the radiographic findings suggested malignancy. Histopathological examination revealed that the lesion was composed of eosinophilic cells and histiocytes, which were positive for S-100 and CD1a immunohistochemically. These findings led to the diagnosis of LCH. PET-CT showed no evidence of lesions in other organs or bone.Patients with unifocal LCH arising in one organ generally have a high chance of spontaneous remission and favorable outcomes. The present case spontaneously remitted 3 months after the biopsy, without additional treatment. Currently, 1 year 9 months after the biopsy, the patient has made favorable progress, with no recurrence.

Extraskeletal Ewing Sarcoma in a Young Patient During Pregnancy

Background: Ewing’s sarcoma is usually identified as a primary malignancy of the bone affecting children and young adults. It is associated with soft tissue extension in 90% of cases. Primarily extraskeletal Ewing’s sarcoma is a rare subtype, and very few data are available concerning optimal treatment modalities. Case Presentation: We report a case of an 18 year-old female from Cabo Verde in her 34th week of pregnancy presenting with a rapid growing cervical tumor. It had initially started as an indolent swelling at the left cervico-posterolateral region. CT had shown involvement of both superficial and deep cervical fascia. At time of presentation, the histologic diagnosis of a peripheral Primitive Neuroectodermal Tumor/Extraskeletal Ewing’s sarcoma had already been provided. To reduce risk of fetal metastasis, the child was delivered by caesarean section. A chest CT of the patient revealed pulmonary metastasis and compressive atelectasis of the left lung caused by the tumor. Three weeks post partum, she presented with loss of muscular function of the extremities as well as parestesia and bladder dysfunction. A repeat CT confirmed medullar compression at the level of C5-C6, C6-C7 and C7-D1 by the tumor without signs of bone metastasis. 5 sessions of palliative radiotherapy were performed that led to parcial recovery of muscular function of the upper right extremity. Palliative chemotherapy was refused, so that the patient was released from hospital. No metastasis had been detected in the child so far. Conclusion: Extraskeletal Ewing’s sarcoma can manifest as a rapidly growing localized mass causing local compression symptoms. Palliative radiotherapy can achieve local tumor reduction and symptom relief.

(iv) The radiological investigation of musculoskeletal malignancy

Abstract Bone and soft-tissue tumours are rare; primary bone and soft-tissue sarcomas should be referred to specialist centres. Accurate diagnosis is usually achieved with history, clinical examination and plain radiographs. For bone tumours the patient's age, the site, size and location within the bone, margins, matrix and presence of periosteal reaction or soft-tissue extension all determine biological activity and the diagnosis. Other imaging modalities may supplement the radiological diagnosis but are of greater use in local and distal staging of the disease, surgical planning and surveillance. Soft-tissue tumours are much less easily characterised using radiographs, MRI is the most useful modality. Biopsies are frequently guided by radiological imaging but should be carefully planned in conjunction with the surgeon providing the definitive procedure. New radiological modalities are increasingly being used to support and supplement the management of musculoskeletal oncology patients.