Extraskeletal myxoid chondrosarcoma: A high incidence of metastatic disease to lymph nodes.

BACKGROUND: Extraskeletal myxoid chondrosarcoma (ESMC) is a rare type of soft-tissue sarcoma with limited series reporting outcome of treatment. Currently there is limited data on the incidence and impact on patient outcome in those with metastatic disease to lymph nodes in ESMC. METHODS: Thirty (21 males, 9 females) patients, mean age 50 ± 16 years, with ESMC were reviewed. The tumors were most commonly located in the lower extremity (n = 23, 77%) and the mean tumor size and volume were 9 ± 5 cm and 490 ± 833 cm3 . Mean follow up was 7 ± 4 years. RESULTS: Six (20%) patients either presented (n = 3, 10%) or developed (n = 3, 10%) lymph node metastatic disease. When comparing patients without, with lymph node metastasis and metastasis elsewhere, patients with lymph nodes metastasis had worse survival than those without metastasis, however better 10-year disease specific survival than those with metastasis elsewhere (100% vs 62% vs 0%; P < .001). CONCLUSION: There is a high incidence of lymph node metastatic disease in patients with ESMC. Although survival in these patients is worse compared to those without metastasis, their survival is better than those with metastasis elsewhere. Due to the high incidence of lymph node metastatic disease, preoperative staging of the lymph node should be considered.

Benefit of Radiotherapy in Extraskeletal Myxoid Chondrosarcoma: A Propensity Score Weighted Population-based Analysis of the SEER Database.

OBJECTIVES: Extraskeletal myxoid chondrosarcoma (EMC) is a rare malignancy for which the role of radiotherapy is not well-defined. We examine the effect of external beam radiotherapy (EBRT) on cancer-specific survival (CSS) for patients with localized EMC, in a propensity score weighted, population-based analysis. MATERIALS AND METHODS: The Surveillance, Epidemiology, and End Results database (1973 to 2012) was queried for cases of localized EMC arising from soft connective tissues of the trunk and extremities treated with surgery and/or EBRT. Inverse probability treatment weighting was utilized, with survival analysis by weighted Cox regression and Kaplan-Meier analysis with log-rank testing. The primary endpoint was CSS. RESULTS: One hundred seventy-two patients were identified, diagnosed from 2004 to 2012. Ninety-four percent and 32% of 156 assessable patients underwent surgery and EBRT, respectively. By inverse probability treatment weighting, balancing covariates of age group, sex, race, grade, T stage, N stage, receipt of surgery, and anatomic site, we observed CSS of 97% versus 85% and 94% versus 85% in patients receiving EBRT versus no EBRT, at 3 and 5 years, respectively, at median follow-up of 33 months, P=0.01. A trend toward an overall survival benefit associated with EBRT was noted, P=0.06. Further adjusting for type of resection performed, CSS benefit persisted, 97% versus 85% at 3 years and 94% versus 85% at 5 years, P=0.02, with trend toward an overall survival benefit, P=0.08. CONCLUSIONS: The receipt of EBRT is associated with a CSS benefit in localized EMC. Aggressive local therapy, including EBRT, should be considered in these patients.

Epidemiology of musculoskeletal tumors in a national referral orthopedic department. A study of 3482 cases.

AIM OF THE STUDY: Musculoskeletal tumors are relatively rare, and their geographic distribution varies greatly around the world. In this study, we present the incidence, age distribution and localization of musculoskeletal tumors diagnosed and/or treated at a tertiary referral orthopedic department, catering to an entire Southeastern European country. METHODS: This was a retrospective study of prospectively collected data, in which all patients diagnosed and/or treated for musculoskeletal tumors at our Department in the period of 30 years (1981-2010) were included. RESULTS: Data of a total of 3482 patients with musculoskeletal tumors were collected. Average age of patients was 33.5 years (range, 2 months-88 years), with even distribution according to sex. Malignant tumors were seen in 20.7% of patients, more often in men (56.9%). Most common malignant tumors were osteosarcoma (estimated incidence: 1.68/million/year), chondrosarcoma (0.79/million/year) and Ewing sarcoma (0.76/million/year). Benign tumors and tumor-like lesions were found in 79.3% of patients, with slight female predominance. Most common benign bone lesions were osteochondroma (5.81/million/year), simple bone cyst (2.13/million/year), and enchondroma (2.05/million/year). CONCLUSION: This report represents a first of its kind in our region, and gives representative results to be compared to other middle and south European countries. Further nationwide studies are needed to improve strategies in bone tumor diagnosis and treatment.

Submucosal neoplasms of the laryngeal introitus.

BACKGROUND: The differential diagnosis of endolaryngeal mesenchymal neoplasms includes a wide spectrum of benign and malignant pathologies, which have been rarely photo-documented and assessed as a group. METHODS: Non-epithelial neoplasms of the endolarynx seen at our centre from 2002 to 2011 (n = 38; 36 treated at our institution) were retrospectively reviewed, with attention to clinical presentation, radiographic imaging, operative management, histology, and pre- and post-operative endoscopy. Submucosal squamous cell carcinomas, mucosal cysts, amyloid and Teflon granulomas were excluded. RESULTS: Twenty-three of a total of 36 patients underwent definitive endoscopic surgical treatment. Supraglottic pathologies included lymphoma, lipoma, neuroendocrine carcinoma, lymphangioma, oncocytoma, haemangioma, synovial cell sarcoma and benign spindle cell neoplasm. Transglottic pathologies included synovial cell sarcoma and granular cell tumour. Glottic pathologies included granular cell tumour, osteoma, rhabdomyoma, rhabdomycosarcoma and myofibroblastic sarcoma. Subglottic pathologies included chondrosarcoma, neurofibroma, adenoid cystic carcinoma and vascular malformation. CONCLUSION: The site of origin, degree of malignant behaviour and sensitivity to adjuvant treatment determined the course of surgical management, i.e. endolaryngeal versus transcervical, and limited removal versus wider resection.

Fibroelastoma dorsi: un tumor infrecuente e infradiagnosticado / Elastofibroma Dorsi: An Uncommon and Under-Diagnosed Tumour

El elastofibroma dorsi es un tumor poco frecuente de los tejidos blandos localizados a nivel infraescapulary/o subescapular. Su incidencia es variable, produciéndose entre la cuarta y la séptima década de la vida,y es más común en las mujeres. Se han revisado 16 casos de elastofibroma diagnosticados en 12 pacientes(7 mujeres, 58,3%), de las cuales 4 pacientes presentaron fibroelastomas bilaterales. El síntoma másfrecuente fue el dolor. El diagnóstico se realizó mediante exploración física, tomografía computarizaday/o resonancia magnética nuclear para confirmar el diagnóstico en casos dudosos. La cirugía de exéresisse realizó bajo anestesia general sin observarse complicaciones mayores. El elastofibroma es un tumorde la pared torácica infrecuente y/o infradiagnosticado con un impacto incierto que requiere su exéresissolo en pacientes sintomáticos(AU)

Elastofibroma dorsi is a relatively rare soft-tissue tumour localized at the infra-scapular level and/orsubscapular regions. It usually occurs between the fourth and seventh decade of life, and is morecommon in females. We reviewed sixteen elastofibromas diagnosed in 12 patients (7 females, 58.3%).Four patients had bilateral elastofibromas. The most common symptom was pain. Presumptive diagnosiswas made by physical examination. Chest ultrasound, computed tomography and/or magnetic resonanceimaging were performed to confirm the diagnosis. Surgery was performed under general anaesthesia.No major complications were observed. Elastofibromas are tumours of the chest wall with an uncertainimpact. Surgical resection is indicated only in symptomatic patients(AU)

Elastofibroma, apariencia por TC y RM / Elastofibroma dorsi: CT and MRI appearence

Propósito: Describir los hallazgos y apariencia del elastofibroma por TC y RM. Material y métodos: Se estudiaron siete pacientes de sexo femenino cuyas edades oscilaron entre 60 y 75 años. Todas las pacientes presentaban masas unilaterales de partes blandas sobre la región periescapular (cuatro del lado izquierdo y tres del lado derecho); una paciente desarrolló una lesión contralateral al año. Cuatro de las pacientes presentaban dolor; en ellas las lesiones fueron extirpadas quirúrgicamente, en dos se realizaron punciones biopsias percutáneas y la restante se encuentra bajo seguimiento clínico imagenológico. Resultados: Tomográficamente las lesiones presentaron densidad similar a la del músculo pero con presencia de bandas hipodensas correspondientes a bandas de tejido adiposo. Luego de la administración de contraste por vía e.v. (en cinco casos) la densidad persistía igual a la del músculo. En RM en la secuencia de T1 las lesiones se caracterizaron por ser isointensas con respecto al músculo y con áreas hipertensas lineales correspondiente a las bandas de tejido graso. En las secuencias de FSE T2 las lesiones se presentaron levemente hipertensas. Tras la administración de gadolinio por vía e.v. se constató en la secuencia de T1 con supresión grasa un refuerzo marcado de las tumoraciones. No se observó compromiso de estructuras óseas ni musculares vecinas. Conclusión: La TC y la RM son métodos de diagnóstico útiles para la detección de fibroelastomas periescapulares (AU)

Treatment options for orthopaedic oncologic entities.

Over the past 2 decades, tremendous advancement in the understanding of tumor natural history and treatment has occurred. If the basic principles are followed, the evaluation and appropriate treatment of musculoskeletal tumors can be reproduced successfully by any conscientious surgeon. Many benign bone and soft-tissue tumors can and probably should be treated by the community orthopaedic surgeon, and this chapter is biased toward treatment of those lesions. The encounter of a malignant lesion is probably beyond the scope of practice of most practicing orthopaedic surgeons. The assessment of the patient and treatments rendered in the first meetings may well dictate the ultimate outcome of survival and limb preservation: thus, patients with such lesions should be treated by experienced orthopaedic oncologists. With the small numbers of these lesions and the extreme consequences of mishandling them, it would be imprudent to do otherwise.

Epidemiologia dos sarcomas / Epidemiology of the sarcomas

Os estudos epidemiológicos são grandes auxiliares na luta contra o câncer, pois descrevem a ocorrência das neoplasias malignas, fazendo comparações geográficas e análises da tendência ao longo do tempo. Da mesma maneira, através dos estudos epidemiológicos observacionais, pode-se estudar seus fatores de risco e os prognósticos, fornecendo subsídios para se atuar em três níveis de preservação. No primeiro deles, através da eliminação ou redução dos fatores de risco, propriamente dito. No segundo, com a detecção precoce poder-se-ia exercer influência no desenvolvimento do tumor, em sua fase inicial. E, por último, a atuação seria no sentido não só de prolongar a sobrevida do paciente, mas a sua qualidade de vida. Neste trabalho são apresentados os coeficientes de incidência, segundo sexo, para neoplasia de ossos e neoplasias malignas do tecido conjuntivo e partes moles, como um todo e para alguns tipos específicos, como rabdomiossarcoma, osteossarcoma e sarcoma de Ewing. Alé disso foi feita uma revisão dos principais fatores de risco e fatores prognósticos, bem como as taxas de sobrevida para pacientes acometidos por esses tumores. Optou-se por expor cada um dos grupos separadamente devido às características diferentes de cada um. Embora os coeficientes se incidência tenham declinado, principalmente na Europa, nos últimos anos e as taxas de sobrevida tenham melhorado consideravelmente, ainda permanece obscuro quais seriam os grupos de risco que mereceriam atenção especial dos profissionais da área da saúde. Isso possibilitaria a detecção precoce e, conseqüentemente, evitaria a utilização de terapias mais agressivas...