Bone: Osteoblastoma

Review on Bone: Osteoblastoma, with data on clinics, and the genes involved

Malignização primária no tumor de células gigantes: um estudo de caso

CONTEXT: Primary malignancy in giant cell tumor (PMGCT) is rare. It is defined as a high-grade sarcoma originating in a giant cell tumor (GCT) and seems to behave less aggressively than its secondary counterpart does. CASE REPORT: This report presents the case of a 39-year-old female with pain in her left shoulder for one month. Radiography showed a pathological fracture of the proximal humerus associated with an osteolytic lesion. Histopathological analysis showed typical areas of GCT juxtaposed with a sarcomatous component. CONCLUSIONS: PMGCT seems to behave less aggressively than secondary malignancy in GCT, and it may simulate its more common benign counterpart clinically and radiographically. However, it requires a more aggressive type of treatment.CONTEXTO: Malignização primária no tumor de células gigantes (MPTCG) é rara. Ela é definida como um sarcoma de alto grau originário de um tumor de células gigantes que parece ser menos agressivo que o tipo secundário. RELATO DE CASO: Relatamos um caso de uma paciente de 39 anos de idade, com dor no ombro esquerdo há um mês. A radiografia mostrou uma fratura patológica do úmero proximal associada a uma lesão osteolítica. O exame histopatológico revelou típicas áreas de tumor de células gigantes justapostas por um componente sarcomatoso. CONCLUSÃO: MPTCG parece se comportar menos agressivamente que a neoplasia secundária do tumor de células gigantes (TCG), e pode simular o TCG, que é mais comum, tanto clínica como radiograficamente. Entretanto, exige tratamento mais agressivo

Endoscopy assisted extended curettage in a rare case of aneurysmal bone cyst involving the 2nd metatarsal of a child

Aneurysmal bone cysts account for only 1% of all bone tumours and of these only a handful of reported cases occur in the foot. They can mimic other bone tumours on imaging studies, thus making histological diagnosis mandatory. Aneurysmal bone cyst of a metatarsal is very rare especially in children and only few cases have been reported in the literature. We report the novel management option for a rare case of an Aneurysmal bone cyst localized to the second metatarsal in a 5 years old boy who presented with limping, local pain, and minimal swelling in his left foot. Tissue diagnosis was confirmed on the basis of needle biopsy. Surgery was in the form of extended curettage using an endoscope with a high-speed burr and cautery. The remnant cavity was filled up with artificial bone graft. Histopathology analysis of the resected tissue was consistent with Aneurysmal bone cyst. There was complete healing at final follow-up at 2 years. Endoscopy assisted extended curettage appears to be an ideal treatment option as it provides a perfect visualisation of the interior of the entire cyst and use of cautery and burr more accurately under direct vision which potentially leaves only a minimal scope for recurrence. Use of artificial bone grafts instead of conventional autogenous bone grafts to reduce graft site morbidity in children and stimulate local foreign body reaction for enhancing residual tumour cell kill if any and also to promote bony sclerosis for healing

A rare case of low-grade myofibroblastic sarcoma of the femur in a 38-year-old woman: a case report

<p>Abstract</p> <p>Introduction</p> <p>Primary myofibroblastic sarcoma of the bone is a rare spindle cell tumour with, to the best of our knowledge, only eight cases reported in the available English language literature. The disease's rarity and its low-grade features make an accurate diagnosis difficult in most cases. The differential diagnoses of this unusual tumour include various benign entities as well as other sarcomas. Due to the difference in prognosis, a precise pathologic diagnosis is essential, which requires a combination of thorough morphologic examination, immunohistochemistry and electron microscopy wherever available.</p> <p>Case presentation</p> <p>We report the case of a 38-year-old Indian woman with a lytic lesion in her left femur. The tumour was associated with cortical destruction and soft tissue extension. A biopsy from the soft tissue component showed features suggestive of a low-grade malignant mesenchymal tumour. Excision of the tumour was performed and histopathological examination showed a low-grade spindle cell sarcoma with collagenous stroma. Expressions of vimentin and smooth muscle actin were also noted. Ultrastructural examination confirmed its myofibroblastic nature. A final diagnosis of low-grade myofibroblastic sarcoma of the left femur was thus rendered.</p> <p>Conclusion</p> <p>Low-grade myofibroblastic sarcoma is one of the rarer osseous spindle cell sarcomas depicting a favourable prognosis in the cases reported so far. Its diagnosis requires ancillary techniques like immunohistochemistry and electron microscopy. To the best of our knowledge, we report the ninth case in the literature and the first case from our subcontinent.</p

Chondromyxoid fibroma management: a single institution experience of 22 cases

Background: Several different strategies have been reported for the treatment of chondromyxoid fibromas, all with variable outcomes and high recurrence rates. Methods: We report on 22 consecutive cases of chondromyxoid fibromas treated by intralesional curettage, four of which had adjuvant cementation at our institution between 2003 and 2010. We assessed the functional outcome using the Musculoskeletal Tumour Society (MSTS) scoring system. Results: Nine males and 16 females with a mean age of 36.5 years (range 11 to 73) and a mean follow-up of 60.7 months were included in the study. Local recurrence occurred in two patients (9%) within the first 2 years following the index procedure. This was treated by re-curettage only of the residual defect. Two postoperative complications occurred: a superficial wound infection in one patient and a transient deep peroneal nerve neurapraxia in the other. The mean postoperative MSTS score was 96.7%. Conclusions: Intralesional curettage and cementation is as an effective treatment strategy for chondromyxoid fibromas, providing satisfactory functional results with a low recurrence rate. Careful case selection with stringent clinical and radiographic follow-up is recommended

Focal Myositis in paediatric age.

BACKGROUND: Focal Myositis is a rare pseudotumor of unknown aetiology that is often difficult to diagnose and treat. Typically afflicting people in adulthood, it has occasionally been reported also among children. PURPOSE: the aim of this study is to review the literature of Focal Myositis in paediatric age in order to compare the clinical manifestation and the various treatment suggested by different authors. METHODS: this article describes a 6-year-old boy with focal myositis in gracilis muscle successfully treated by conservative methods, including nocturnal leg traction, intensive physiokinesi therapy and articulated knee orthosis guided to progressive extension. Furthermore a systematic review of literature concerning focal myositis in paediatric age is reported. CONCLUSION: our case and the review of literature suggests that conservative methods should be the first-choice treatment for FM in paediatric age and that surgery should be strictly reserved for selected cases where non-invasive methods have previously faile

The combination of temozolomide-irinotecan regresses a doxorubicin-resistant patient-derived orthotopic xenograft (PDOX) nude-mouse model of recurrent Ewing's sarcoma with a FUS-ERG fusion and CDKN2A deletion: Direction for third-line patient therapy.

The aim of the present study was to determine the usefulness of a patient-derived orthotopic xenograft (PDOX) nude-mouse model of a doxorubicin-resistant metastatic Ewing's sarcoma, with a unique combination of a FUS-ERG fusion and CDKN2A deletion, to identify effective drugs for third-line chemotherapy of the patient. Our previous study showed that cyclin-dependent kinase 4/6 (CDK4/6) and insulin-like growth factor-1 receptor (IGF-1R) inhibitors were effective on the Ewing's sarcoma PDOX, but not doxorubicin, similar to the patient's resistance to doxorubicin. The results of the previous PDOX study were successfully used for second-line therapy of the patiend. In the present study, the PDOX mice established with the Ewing's sarcoma in the right chest wall were randomized into 5 groups when the tumor volume reached 60 mm3: untreated control; gemcitabine combined with docetaxel (intraperitoneal [i.p.] injection, weekly, for 2 weeks); irinotecan combined with temozolomide (irinotecan: i.p. injection; temozolomide: oral administration, daily, for 2 weeks); pazopanib (oral administration, daily, for 2 weeks); yondelis (intravenous injection, weekly, for 2 weeks). All mice were sacrificed on day 15. Body weight and tumor volume were assessed 2 times per week. Tumor weight was measured after sacrifice. Irinotecan combined with temozolomide was the most effective regimen compared to the untreated control group (p=0.022). Gemcitabine combined with docetaxel was also effective (p=0.026). Pazopanib and yondelis did not have significant efficacy compared to the untreated control (p=0.130, p=0.818). These results could be obtained within two months after the physician's request and were used for third-line therapy of the patient

The role of thallium-201 and pentavalent dimercaptosuccinic acid for staging cartilaginous tumours

INTRODUCTION: Heterogeneity of cartilage tumours may confound accurate diagnosis and grading resulting in under and over treatment. Improved preoperative assessment of malignancy and grade would be invaluable for developing a rational plan for treatment. We examined correlations between nuclear tracer avidity and malignancy grade in cartilage tumours. METHODS: Between 1996 and 2000, 92 consecutive patients with cartilaginous tumours (50 benign, 42 non-metastatic malignant) underwent nuclear scanning. Thallium-201 (TL-201) and pentavalent dimercaptosuccinic acid (DMSAV) were used as nuclear isotopes. Scanning with these agents was performed on separate days 48 hours apart. Static and SPECT images were obtained at 30 m and 4 h after injection of nuclear tracer. Pathology review was undertaken blinded to the results of the nuclear scans and correlations between histologic results and trace uptake at 4 hours examined. RESULTS: 25 patients with negative DMSAV had benign tumours. 15/17 tumours with positive TL-201 had malignant tumours. 11/13 patients with both positive DMSAV and TL-201 scans had intermediate or high grade tumours and 4 of these developed metastases. We have developed an algorithm for the management of patients with tumours that aims to avoid over treatment of low grade tumours and under treatment of high grade tumours. CONCLUSION: Functional nuclear scanning with TL-201 and DMSAV complements other imaging modalities in the management of cartilaginous tumours

Lipomas profundos

Se han revisado retrospectivamente 10 lipomas profundos localizados en los miembros. Todos los pacientes fueron intervenidos quirúrgicamente y el diagnóstico de certeza fue histopatológico. Siete de los casos eran lipomas intra o intermusculares y los restantes angiolipoma, hibernoma y fibrolipoma intraneural, respectivamente. El seguimiento medio postoperatorio fue de 48 meses (mínimo: 14, y máximo: 84). No ha habido recidiva en ningún caso.This article is a retrospective review of 10 deep lipomata localized in the limbs. In all the cases the treatment required surgery, and the diagnosis was made by histopathological analysis. Seven cases were intra or intermuscular lipomata, and the other three were angiolipoma, hibernoma and endoneural fibrolipoma. The average postoperative follow-up time was 48 months (14 min-84 max). None showed recurrence at review