Prognostic factors of alveolar soft part sarcoma in children and adolescents: A population-based study.

PURPOSE: Alveolar Soft Part Sarcoma (ASPS) is an exceedingly rare and aggressive cancer in children. Our objective was to conduct a population-based cohort study to forecast overall survival (OS) in pediatric ASPS patients. METHODS: We utilized the Surveillance, Epidemiology, and End Results (SEER) database to identify all pediatric ASPS patients diagnosed between 1975 and 2019. Kaplan-Meier estimations were employed to construct survival curves based on various criteria. Survival curves were compared using the log-rank test. Cox proportional-hazards regression was utilized to determine variables associated with OS. Additionally, we constructed a nomogram to predict overall survival in pediatric ASPS patients. RESULTS: A total of 103 pediatric ASPS patients were identified. Predominantly, the tumors affected females (62.2 %), and most of them located in the extremities (53.4 %). The majority of patients underwent surgery (83.5 %). Survival rates declined with increasing tumor size, and patients with localized tumors exhibited significantly better prognoses than those with distant tumors. Surgery conferred superior survival outcomes compared to no surgery. Cox proportional hazard regression analysis identified SEER stage and surgery as important independent predictors of survival. CONCLUSIONS: Our study highlights SEER stage and surgery as key predictors of OS in pediatric ASPS, offering crucial epidemiological insights for clinical management.

Spectrum of Biopsy Proven Extraocular Muscle Tumours of Non-Thyroid Origin.

OBJECTIVE: To describe different types of primary extraocular muscle (EOM) tumours based on the results of imaging studies, peroperative clinical picture and their histopathological diagnosis. STUDY DESIGN: Case series. PLACE AND DURATION OF STUDY: Al-Shifa Trust Eye Hospital, Rawalpindi, from July 2001 to January 2017. METHODOLOGY: A retrospective analysis of 640 diagnosed orbital tumours was carried out using non-randomised sampling technique, and the prevalence of primary EOM tumours was determined. Based on the results of imaging studies, the clinical picture observed during surgery (orbitotomy) and the histopathological diagnosis, primary EOM tumours were divided into different types, accordingly. RESULTS: Nineteen (n=19) primary EOM tumours (frequency of 2.96%) had 12 types of histopathological diagnoses, and were categorised into inflammatory tumours (n=8, 42%), vascular tumours (n=4, 21%), lymphoproliferative tumours (n=3, 16%), neurogenic tumours (n=2, 10.5%) and myogenic tumours (n=2, 10.5%). The recti were involved more frequently than obliques (n=15, 78.94% and n=4, 21.06%, respectively). All the patients presented with proptosis of varying degree with some degree of globe rotation and had surgical excision/appropriate management. Visual acuity was not affected in any of the patients. Four (n=4, 21.05%) tumours were malignant (NHL, ASPS, myeloid sarcoma and rhabdomyosarcoma) and these patients underwent chemotherapy and/or radiotherapy. CONCLUSION: Biopsy-proven primary EOM tumours were devisable into five broad categories. Patients with primary EOM tumours presented with proptosis and impaired ocular motiliy. The primary EOM tumours involved both the recti and the obliques and were excised surgically with favourable outcomes in most cases.

Situación actual de los sarcomas de partes blandas: registro de un instituto oncológico de Latinoamérica / Current situation of soft tissue sarcomas: Registry of a Latin American cancer institute

Objetivo: Los sarcomas de partes blandas (SFTS) conforman un grupo de neoplasias poco frecuentes y heterogéneas (menos del 1% en adultos y 15% en pediátricos), de los cuales no se cuenta con registros actualizados en población latinoamericana. El estudio tiene como objetivo describir la situación actual de los pacientes tratados en un instituto oncológico de Latinoamérica. Métodos: Se obtuvo un registro de 250 pacientes con diagnóstico de SFTS, tratados en el Instituto de Enfermedades Neoplásicas del Perú (INEN), durante el periodo 2009-2013, con una media de seguimiento de 62 meses, registrándose datos: epidemiológicos, clínicos, tratamiento y seguimiento; realizándose el análisis de sobrevida global mediante el modelo proporcional de Cox. Resultados: Los SFTS mostraron una mayor frecuencia en género masculino (60,8%), con un pico incidencia a partir de los 50 años (69,6%), la localización tumoral predominante fue en extremidades inferiores (64,4%), siendo los subtipos histológicos más frecuentes: sarcoma pleomórfico indiferenciado (34%) y liposarcomas (25,6%); el estadio clínico iii es el de mayor registro (30,8%). Se obtuvo una tasa de sobrevida global a 5 años del 63,9%, encontrándose en el análisis estadístico asociación significativa entre la sobrevida global y las variables: edad (> 50 años), tamaño tumoral (> 5cm), profundidad (subfascial), grado histológico (G3), recurrencia local y a distancia, mostrando en este grupo rangos menores de sobrevida. Conclusiones: Se ha logrado precisar la epidemiología, el tratamiento y el pronóstico, así como las variables que repercuten en la sobrevida de los pacientes latinoamericanos con SFTS estudiados

Introduction: Soft tissue sarcomas (SFT) are a group of rare and heterogeneous neoplasms (representing less than 1% of cancer in adults and 15% in pediatric patients), for which there is no updated records in the Latin American population. This study aims to describe the current situation of patients treated at a cancer institute in Latin America. Methods: We obtained records from 250 patients with a diagnosis of SFT, treated at the National Institute of Neoplastic Diseases of Peru (INEN) during the period 2009-2013, with a mean follow-up of 62 months. The following data were recorded: epidemiological, clinical, treatment and follow-up. The analysis of global survival was done with the Cox proportional hazards model. Results: SFT showed a greater frequency in males (60.8%), with a peak incidence after 50 years of age (69.6%). Tumor location was predominantly in the lower extremities (64.4%), and the most frequent histologic subtypes were: undifferentiated pleomorphic sarcoma (34%) and liposarcomas (25.6%); clinical stage iii was the most frequent (30.8%). The 5-year overall survival rate was 63.9%, while the statistical analysis found a significant association between global survival and the variables: age (>50 years), tumor size (>5cm), depth (subfascial), histologic grade (G3), local and distant recurrence, showing shorter survival times in these groups. Conclusions: This study has clarified the epidemiology, treatment and prognosis, as well as the variables that have an impact on the survival of the Latin American patients with SFT studied

Alveolar soft-part sarcoma: can MRI help discriminating from other soft-tissue tumors? A study of the French sarcoma group.

OBJECTIVES: To investigate the imaging features of alveolar soft-part sarcomas (ASPS) on pre-treatment MRI in order to identify relevant criteria to distinguish ASPS from other soft-tissue tumors. METHODS: A series of 25 patients (mean age, 18.5 years old) with histologically proven ASPS from five French comprehensive cancer centers was compared to a control cohort of 292 patients with various histologically proven benign and malignant soft-tissue tumors representative of the 10-year long activity of one center. All had a baseline MRI with contrast-agent administration. Two radiologists independently reviewed the MRIs. Features assessing location, size, signal, architecture, periphery, and vascularization were reported. Their association with the histological diagnosis of ASPS was evaluated with chi-square or Fisher's test. Their prevalence, sensitivity, specificity, odds ratio, and reproducibility were calculated. RESULTS: Eight MRI features were significantly associated with ASPS: deep location (p < 0.001), high signal intensities on T1-weighted imaging (p < 0.001), central area of necrosis (p = 0.001), absence of fibrotic component (p = 0.003), infiltrative growth pattern (p = 0.003), absence of tail sign (p = 0.001), presence of intra- and peritumoral flow-voids (p < 0.001), and number of flow-voids ≥ 5 (p < 0.001). Twenty out of the 25 (80%) ASPS showed at least 7 of these 8 features compared to only four out of 292 (1.4%) tumors of the control cohort (1 benign vascular tumor, 1 solitary fibrous tumor, 2 high-grade soft-tissue sarcomas). The five ASPS with less than 7 out of 8 features measured less than 40 mm. CONCLUSION: The striking histological uniformity of ASPS translates into imaging. However, ASPS may be misdiagnosed as benign tumors or pseudo-tumors, notably intramuscular benign vascular tumors or vascular malformations. KEY POINTS: • ASPS are rare aggressive mesenchymal tumors displaying recurrent MRI features highly reminiscent of the diagnosis. • Deep-seated tumors presenting with mainly high signal intensity on T1-weighted imaging, an absence of fibrotic component, ill-defined margins without aponeurotic extension, and more than five central and peripheral flow-voids are very likely to be ASPS. • ASPS may be misdiagnosed as intramuscular benign vascular tumor or vascular malformation, which occur in the same age group.

Brain, the last fortress of sarcoma: similar dismal outcome but discrepancy of timing of brain metastasis in bone and soft tissue sarcoma.

BACKGROUND AND OBJECTIVE: Brain metastasis is a rare but dismal event in sarcomas. However, the pattern of occurrence and the prognostic factors associated with post-brain metastasis survival (PBMS) are not yet well-characterized. METHODS: Sarcoma patients treated at one institute within 10-year period were retrospectively reviewed and those with brain metastasis were identified. The incidence of brain metastasis was demonstrated by case per person-years and cumulative incidence curves. Univariate factors associated with PBMS were analyzed. RESULTS: Among 611 sarcoma patients, 20 (3.3%) developed brain metastasis. Alveolar soft part sarcoma (ASPS) and osteosarcoma were the most common subtypes. Overall, the cumulative incidence was 3.9% at 5 years and 8.4% at 10 years. However, the incidence in STS patients continued to rise up to 10 years after primary diagnosis, whereas it reached a plateau in bone sarcoma patients at 3 years. Median PBMS was 1.67 months. Univariate factors associated with better PBMS included ASPS histology, initial surgical treatment, and brain irradiation for non-surgically treated patients. CONCLUSION: Our study revealed a discrepancy in the timing of occurrence of brain metastasis between STS and bone sarcoma. However, patients with brain metastasis had a poor prognosis, implicating the brain as the last fortress of sarcoma.

Liposarcoma de pared torácica: presentación de un caso / Liposarcoma in the thoracic wall: case study

Se presenta el caso de un paciente masculino de 86 años de edad, de la raza blanca, que refirió haberse operado 1 año atrás por una tumoración en la espalda, y que en los 6 últimos meses, comenzó a aumentar progresivamente de tamaño, acompañado esto de supuración no fétida desde hacía 1 mes. Al examen físico se observó gran tumoración ulcerada, de unos 20 cms de diámetro con secreción amarillenta dependiente de la pared torácica, la cual al ser examinada luego de su exéresis, resultó ser un linfosarcoma pleomorfo grado II, con extensas zonas de necrosis, diámetro de 20x14x7 cms y un peso de 746 grs. La decisión de dar a conocer este caso, se fundamenta en su carácter infrecuente, al punto de ser el único diagnosticado en nuestra provincia(AU)

Presentation of the case of a white male (86 years old) referring to have being operated one year ago due to a tumor in his back which has being enlarging in the last six months including not fetid suppuration in the last month. The physical examination showed a big ulcerated tumor of about 20 cm of diameter with yellow secretion. It turned to be a lymphosarcoma grade II, with wide necroses zones, diameter of 20x14x7 cm and weighing 746grs. This is a very infrequent case, the only one reported in our province(AU)

Dermatofibrosarcoma protuberans: review of 20-years experience

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Introduction. Dermatofibrosarcoma protuberans(DFSP) is an uncommon soft tissue neoplasm withlow-intermediate grade of malignancy. It is a locallyaggressive tumour with a high recurrence rate. Surgicalexcision with adequate margins is the maintreatment.Materials and methods. We describe the clinicopathologicalfeatures of 21 cases of DFSP. Themean size of the lesions was 5.6 cm, mostly locatedin trunk. 61.9% of the cases underwent sugical excisionwithout previous biopsy. 52.4% of the patientspresented positive margins, that required surgicalextension.Results. The recurrence rate was 28.6% (6 cases),five of them local recurrences treated with new surgicalexcision with wide margins. Median periodfree of illness was 32.5 months. Medium follow-upperiod was 33.25 months.Conclusions. DFSP has a locally high recurrencerate, that has been associated to inadequate surgicalmargins. The histological knowledge preoperativelywould permit surgical excision with adequatemargins, and probably this could reduce the recurrencerate. Radiotherapy could avoid the surgicalextension of margins in these cases with positiveones. Chemotherapy could be indicated in metastasiccases. Most recurrences appear in the first 3years, but it is important a long-term follow-up ofthese patients

Sarcoma de partes moles / Sarcoma soft part

Os autores fazem uma revisão bibliográfica dos sarcomas de partes moles (spm), buscando relacionar as apresentações clínicas, tipos histológicos, formas de tratamento e fatores prognósticos.

A proposed explanation for female predominance in alveolar soft part sarcoma. Noninactivation of X; autosome translocation fusion gene?

BACKGROUND: Alveolar soft-part sarcoma (ASPS) is a rare malignant soft tissue tumor with both clinically and morphologically distinct features. It often involves the extremities of adolescents and young adults and shows a predilection for females. Recently, ASPS was found to have a nonreciprocal der(17)t(X;17) translocation with the corresponding fusion gene located in chromosome 17. Because females have an extra X-chromosome, their likelihood of developing an X;autosome translocation is theoretically double that of males, and thus, this extra X-chromosome is a likely explanation for female predominance of ASPS. METHODS: The authors used data from the National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) registry program, which included 87 ASPS cases (33 males and 54 females), and published ASPS cases, which included 317 cases (121 males and 196 females), to test our hypothesis. The authors compared the observed proportion of female cases with that expected under the two X-chromosomes-double-risk hypothesis including the consideration of X-inactivation status. RESULTS: The hypothesis that the fusion gene is not subject to X-inactivation is supported by data (P = 0.6, 0.24, and 0.20 for SEER cases, published cases, and their combination, respectively). In contrast, the competing hypothesis that the fusion gene is subject to X-inactivation is rejected (P = 0.007, < 0.00001, and < 0.00001 for SEER cases, published cases, and their combination, respectively). CONCLUSIONS: Therefore, the authors found a statistical association between the female predominance observed in ASPS and female possession of an extra X-chromosome/noninactivation of the ASPS X;autosome translocation fusion gene.

[Christopherson's tumor. Review of the literature apropos of a retroperitoneal localization]. / La tumeur de Christopherson. Revue de la littérature à propos d'une localisation rétropéritonéale.

Alveolar soft-part sarcoma is rare, and its retroperitoneal localization in psoas major muscle, exceptional. Concerning a case occurred in a young woman, the authors focus on the characters of this tumor of unknown histogenesis which affects predominantly young adults, mainly females. This disease, which symptomatology is poor and which evolution is particularly long for such a neoplasm, leads inexorably to death despite surgical treatment that represents to date, the unique efficient therapeutic modality.