Primary Renal Synovial Sarcoma and Clinical and Pathological Findings: a Systematic Review.

PURPOSE OF REVIEW: To update epidemiological, diagnostic, and therapeutic information on primary synovial sarcoma of the kidney. RECENT FINDINGS: A total of 96 studies were analyzed; age at presentation was 38.6±14.2 years, predominant location of tumor was right kidney; frequent reported symptoms at diagnosis were hematuria and pain. For definitive diagnosis, cytogenetic technique was used. Detected oncogene was available in 37.8% cases with fusion of SS18-SSX in most patients. Surgery is treatment of choice, with adjuvant chemotherapy; most frequently ifosfamide-based associated with doxorubicin or epirubicin. Overall median survival was 34 months. Mortality was 29% of the cases which reported death and the recurrence rate was 39.8%. Risk of death was increased in patients with metastases at diagnosis Primary RSS occurs more often in young men. RSS often presents with symptoms and in an advanced stage. Surgical treatment is the most commonly used and chemotherapy for advanced or recurrent treatment.

Synovial sarcoma of the head & neck: A review of reported cases in the literature.

Synovial sarcoma is a high-grade malignancy that seldom manifests in the head and neck. The purpose of this paper is to provide a comprehensive review of the existing literature on primary HNSS. This review study includes case reports, case series and retrospective cohort studies on primary HNSS published in the English language. All cases identified via PubMed were analyzed individually. Articles were included according to specified eligibility criteria. The total number of cases analyzed was 243 (91 case reports, 53 cases from 16 case series, and 99 cases from 8 retrospective cohort studies). The average age at the time of HNSS diagnosis was 33.5 years. The ratio of male-to-female was 1.64:1. The most frequently occurring histologic subtype was biphasic (59.2%). The most common location was the neck. Hoarseness was the most common presenting symptom. Almost all patients (98.3%) underwent surgical excision. 55.6% of patients received adjuvant radiotherapy. A smaller number of patients (30.7%) received adjuvant chemotherapy. 60.3% of patients were alive with no evidence of disease at follow-up while 28.0% of patients died of HNSS at follow-up. The average follow-up period was 52.0 months. Synovial sarcoma is a rare head and neck malignancy. Surgical excision with adjuvant radiation is the most frequently employed treatment modality. Regardless of treatment modality, the prognosis of the tumor is guarded.

The survival and prognosis analysis of synovial sarcoma subtypes: a Surveillance, Epidemiology, and End Results population-based analysis.

PURPOSE: By comparing epidemiologic and prognostic data of synovial sarcoma subtypes, this study aims to clarify the difference in prognosis and risk factors among different synovial sarcoma subtypes. METHODS: In total, 1692 patients with synovial sarcoma in the USA who were diagnosed during 1975 through 2016 and retrieved from the Surveillance, Epidemiology, and End Results (SEER) program were studied. RESULTS: There were statistically significant differences in the distribution of age and race among three synovial sarcoma subtypes, while no major differences in the distribution of sex, tumor stage, and tumour size were found. The highest five and ten year survival rates were found in the biphasic subtype (69%, 60%), followed by the monophasic subtype (59%, 49%), and lowest in the epithelioid subtype (32%, 26%). Age and SEER historic stage were the two demographic factors that found to have statistically significant impact on survival in all subtypes. Radiation was found to be protective in the monophasic subtype (HR 0.61, p < 0.001). CONCLUSION: Among different synovial sarcoma subtypes, the biphasic subtype favoured the best survival, while the epithelioid cell subtype was associated with the worst. Male and black race were independently associated with worse survival only in the monophasic subtype. Radiotherapy could provide significant benefit for patients with the monophasic synovial sarcoma.

Epidemiology, incidence, and survival of synovial sarcoma subtypes: SEER database analysis.

BACKGROUND: Synovial sarcoma (SyS) is a rare malignancy that is typically located on the limbs and occurs predominantly in adolescents. A study in a large population for SyS comparing subtypes has not yet been reported. METHODS: National Cancer Institute's Surveillance, Epidemiology, and End Results database was queried for patients diagnosed with SyS between January 1975 and December 2016. Patients were classified demographically according to gender, age, race/ethnicity, and marital status, and they were also classified by tumors, subtypes, localization, grade, year of diagnosis, laterality, type of treatment, and follow-up results. RESULTS: A total of 3228 patients were included, with a mean age of 39.3 ± 18.8 (range: 1-94), of which 1521 (47.1%) were females and 1707 (52.9%) were males. According to its subtypes, 47.2% were SyS not otherwise specified, 32.3% were spindle cell, 19.9% were biphasic, and 0.6% were epithelioid type. The overall survival period is 138.0 (95% confidence interval: 113.2-162.8) months. Survival duration was found to be significantly different between groups according to gender (log-rank test; p < 0.001), age groups (log-rank test; p < 0.001), race (log-rank test; p = 0.001), marital status (log-rank test; p < 0.001), tumor subtypes (log-rank test; p < 0.001), tumor location (log-rank test; p < 0.001), tumor laterality (log-rank test; p < 0.001), date of diagnosis (log-rank test; p = 0.025), tumor grade (log-rank test; p < 0.001), historic stage (log-rank test; p < 0.001), state of chemotherapy (log-rank test; p < 0.001), state of radiotherapy (log-rank test; p < 0.001), presence of metastasis (log-rank test; p < 0.001), and total number of malignant tumors (log-rank test; p < 0.001). CONCLUSION: Male gender, being colored individual, being over 35 years at the time of diagnosis, epithelioid type, non-head and neck region localization is associated with poor prognosis. While radiotherapy improves survival, benefit of chemotherapy is unclear. LEVEL OF EVIDENCE: III retrospective analysis.

Sarcoma sinovial con invasión medular / Synovial sarcoma with medullary invasion

Introducción: el sarcoma sinovial es una neoplasia maligna de origen mesenquimal, constituida por células fusiformes y un componente de diferenciación epitelial con grados variables de displasia. Afecta principalmente las extremidades, con predominio de las inferiores. De los sarcomas de partes blandas 5-10 por ciento son sinoviales.Objetivo: presentar el caso de una paciente con sarcoma sinovial a partir de los hallazgos imagenológicos que posibilitaron diagnosticar la enfermedad.Presentación del caso: paciente femenina, de 38 años de edad, con antecedentes de salud. Presentó un cuadro doloroso en la región distal del muslo, de 12 meses de evolución, con aumento de volumen y agravamiento de los síntomas en los últimos cinco meses. En el examen físico se observaron cambios en la textura de la piel de la rodilla derecha, aumento de volumen moderado, y dolor a la palpación más acentuado en la región distal del muslo, con limitación de los movimientos. La resonancia magnética nuclear del muslo derecho con contraste endovenoso mostró una extensa masa de aspecto heterogéneo, realzada con la administración del medio de contraste (Dotarem) localizada predominantemente en el tercio distal del muslo, que invadía el canal medular, asociada con osteolisis y periostitis.Conclusiones: en los casos de enfermos con sintomatología como la presentada, se deben realizar los estudios imagenológicos pertinentes para confirmar la posibilidad diagnóstica de sarcoma sinovial. Por sus características, la resonancia magnética nuclear es la técnica imagenológica de elección para detectar, caracterizar, estadificar y seguir la evolución de estos tumore(AU)

Introduction: synovial sarcoma is a malignant neoplasm of mesenchymal origin, composed of spindle cells and a component of epithelial differentiation with varying degrees of dysplasia. It mainly affects the extremities, with predominance of the lower ones. Of the soft tissue sarcomas 5-10 percent are synovial.Objective: to present the case of a patient with synovial sarcoma from the imaging findings that made it possible to diagnose the disease.Case presentation: female patient, 38 years old, with health history. She presented a painful picture in the distal region of the thigh, of 12 months of evolution, with increased volume and aggravation of symptoms in the last five months. Physical examination revealed changes in the texture of the skin of the right knee, moderate volume increase, and pain on palpation more pronounced in the distal region of the thigh, with limited movement. Nuclear magnetic resonance of the right thigh with intravenous contrast showed an extensive mass of heterogeneous appearance, enhanced with the administration of contrast medium (Dotarem) located predominantly in the distal third of the thigh, which invaded the medullary canal, associated with osteolysis and periostitis.Conclusions: in the cases of patients with symptoms such as the one presented, the pertinent imaging studies should be carried out to confirm the diagnostic possibility of synovial sarcoma. Due to its characteristics, nuclear magnetic resonance is the imaging technique of choice to detect, characterize, stage and follow the evolution of these tumors(AU)

Clinicopathologic features of undifferentiated round cell sarcomas of bone & soft tissues: An attempt to unravel the BCOR-CCNB3- & CIC-DUX4-positive sarcomas.

Background & objectives: Certain genetically defined undifferentiated round cell sarcomas, namely BCOR-CCNB3 and CIC-DUX4 positive, have been described. Here we present detailed clinicopathologic features and molecular results in such cases. Methods: Fifty one cases of undifferentiated round cell sarcomas, including 32 cases, tested for BCOR-CCNB3 and CIC-DUX4 fusions, by reverse transcription polymerase chain reaction technique and 44 tumours, for CCNB3 immunostaining, were analyzed. Results: Twenty seven (52.9%) tumours occurred in males and 24 (47%) in females; in soft tissues (38; 74.5%), commonly, trunk and extremities and bones (13; 25.4%), frequently, femur and tibia. Five of 32 (15.6%) tested cases were positive for BCOR-CCNB3 fusion and seven (21.8%) for CIC-DUX4 fusions. Histopathologically, CIC-DUX4-positive sarcomas comprised nodular aggregates of round to polygonal cells, containing hyperchromatic nuclei, prominent nucleoli and moderate cytoplasm, with focal myxoid stroma and variable necrosis, in certain cases. BCOR-CCNB3- positive sarcomas mostly comprised diffusely arranged, round to oval to short spindly cells with angulated nuclei, vesicular chromatin, inconspicuous nucleoli and interspersed vessels. Immunohistochemically, tumour cells were positive for MIC2 in 24 of 49 (48.9%) and CCNB3 in 12 of 44 (27.2%) cases. Four of five BCOR-CCNB3-positive sarcomas showed CCNB3 immunostaining and 6 of 7 CIC-DUX4-positive sarcomas displayed WT1 immunostaining. Most patients (27/37) (72.9%) underwent surgical resection and chemotherapy. Median overall survival was 12 months, and disease-free survival was seven months. Interpretation & conclusions: Undifferentiated round cell sarcomas are rare; mostly occur in soft tissues of extremities, with CIC-DUX4 positive, as these are relatively more frequent than BCOR-CCNB3 positive sarcomas. CCNB3 and WT1 are useful immunostains for triaging such cases for BCOR-CCNB3 and CIC-DUX4 fusion testing, respectively. Overall, these are relatively aggressive tumours, especially CIC-DUX4-positive sarcomas.

[Analysis of clinicopathological characteristics and prognostic factors of foot and ankle soft tissue and bone tumors].

Objective: To evaluate the clinicopathological characteristics of foot and ankle soft tissue and bone tumor, and to analyze the prognosis and the related factors of malignant tumors in this site. Methods: 74 patients with soft tissue and bone tumors of foot and ankle from January 2006 to February 2017 were retrospectively analyzed. The clinicopathological characteristics, the treatment and survival status of malignant tumors were followed up, and the clinical and therapeutic factors related to prognosis were analyzed. Results: Of the 74 patients, 34 were males and 40 were females. The male to female ratio was 1∶1.18; the age ranged from 12 to 64 years and the median age was 42 years. Tumors located in forefoot of 22 cases, 22 in midfoot, 10 in hind foot, 14 in ankle joint and 6 in multiple sites. 14 cases were bone tumors, including 7 benign and 7 malignant, and 60 cases were soft tissue tumors, including 14 benign and 46 malignant. The most common malignant soft tissue tumors were synovial sarcomas (13 cases), and the most common benign soft tissue tumors were hemangiomas (4 cases). 44 cases of malignant tumors underwent surgery were followed up, of which were 7 bone and 37 soft tissue malignant tumors. Limb salvage surgeries were performed in 33 cases and amputation in 11 cases. The median follow-up time was 69.8 months, and the median survival time was 40.7 months. The 1-year, 3-year and 5-year survival rate of soft tissue malignant tumors was 88.0%, 73.0%, and 63.0%, respectively. The 1-year, 3-year and 5-year survival rate of bone malignant tumors was 86.0%, 57.0% and 57.0%, respectively. Univariate analysis showed that the prognostic factors affecting 5-year survival rate were tumor size and adjuvant therapy (P<0.05). Patient's gender, age, tumor location, histological type and surgical procedure had no effect on overall survival(P>0.05). Multivariate analysis showed that tumor size was an independent prognostic factor (RR=7.262, P=0.005). Conclusions: Forefoot and midfoot are more common in foot and ankle soft tissue and bone tumors. Synovial sarcoma is the most common diagnosis in malignant soft tissue tumors, and hemangioma is the most common diagnosis in benign soft tissue tumors. The prognostic factor of malignant soft tissue and bone tumors in foot and ankle is tumor size. Patients with the tumor size of 5 cm or more have a worse prognosis.

Deep learning approach for survival prediction for patients with synovial sarcoma.

Synovial sarcoma is a rare disease with diverse progression characteristics. We developed a novel deep-learning-based prediction algorithm for survival rates of synovial sarcoma patients. The purpose of this study is to evaluate the performance of the proposed prediction model and demonstrate its clinical usage. The study involved 242 patients who were diagnosed with synovial sarcoma in three institutions between March 2001 and February 2013. The patients were randomly divided into a training set (80%) and a testing set (20%). Fivefold cross validation was performed utilizing the training set. The test set was retained for the final testing. A Cox proportional hazard model, simple neural network, and the proposed survival neural network were all trained utilizing the same training set, and fivefold cross validation was performed. The final testing was performed utilizing the isolated test data to determine the best prediction model. The multivariate Cox proportional hazard regression analysis revealed that size, initial metastasis, and margin were independent prognostic factors. In fivefold cross validation, the median value of the receiver-operating characteristic curve (area under the curve) was 0.87 in the survival neural network, which is significantly higher compared to the area under the curve of 0.792 for the simple neural network (p = 0.043). In the final test, survival neural network model showed the better performance (area under the curve: 0.814) compared to the Cox proportional hazard model (area under the curve: 0.629; p = 0.0001). The survival neural network model predicted survival of synovial sarcoma patients more accurately compared to Cox proportional hazard model.

Nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) in pediatric and young adult patients: Results from a prospective study using limited-margin radiotherapy.

BACKGROUND: Indications for and delivery of adjuvant therapies for pediatric nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) have been derived largely from adult studies; therefore, significant concern remains regarding radiation exposure to normal tissue. The authors report long-term treatment outcomes and toxicities for pediatric and young adult patients with high-grade NRSTS who were treated on a prospective trial using limited-margin radiotherapy. METHODS: Sixty-two patients (ages 3-22 years) with predominantly high-grade NRSTS requiring radiation were treated on a phase 2 institutional study of conformal external-beam radiotherapy and/or brachytherapy using a 1.5-cm to 2-cm anatomically constrained margin. The estimated cumulative incidence of local failure, Gray's method estimated cumulative incidence of local failure, Kaplan-Meier method estimated survival, competing-risk regression model determined predictors of disease outcome, and toxicity was reported according to CTCAE v2.0. RESULTS: At a median follow-up of 5.1 years (range, 0.2-10.9 years), 9 patients had experienced local failure. The 5-year overall cumulative incidence of local failure was 14.8% (95% confidence interval [CI], 7.2%-25%), and all but 1 local failure occurred outside the highest-dose irradiation volume. The 5-year Kaplan-Meier estimates for event-free and overall survival were 49.3% (95% CI, 36.3%-61.1%) and 67.9% (95% CI, 54.2%-78.3%), respectively. Multivariable analysis indicated that younger age was the only independent predictor of local recurrence (P = .004). The 5-year cumulative incidence of grade 3 or 4 late toxicity was 15% (95% CI, 7.2%-25.3%). CONCLUSIONS: The delivery of limited-margin radiotherapy using conformal external-beam radiotherapy or brachytherapy provides a high rate of local tumor control without an increase in marginal failures and with acceptable treatment-related morbidity. Cancer 2017;123:4419-29. © 2017 American Cancer Society.

[Synovial sarcoma. Case report]. / Sarcoma synoviale.

In 2013 there were 94,770 new cancer patients reported in Hungary. Synovial sarcoma accounts for 0.05-0.1% of all cancers and, therefore its incidence is predicted to be 47-94 patients/year in Hungary. The authors report the history of a 18-year-old man who was operated on a right upper abdominal wall tumor with R1 resection. During the next 5 months the tumor grew up to 8 cm in largest diameter. Histology revealed monophasic synovial sarcoma. Immunohistochemistry showed bcl2, focal CD99 and high molecular weight cytokeratin positivity, while smooth muscle actin, S100 and CD34 immunostainings were negative. Becose of this reoperation was not possible, curative six cycles of doxorubicine and ifosfamide with granulocyte colony stimulating factor support and 60 Gy radiotherapy was given to the tumor bed. After these treatments computed tomography scan was negative and the patient attended regular imaging every 3 months. At the age of 20 years the patient developed two neoplastic lesions in the surgical scar measuring 10 mm and 45 × 10 mm in size. R0 resection, partial rib resection and abdominal wall reconstruction were performed. Histology confirmed residual monophasic synovial sarcoma. Radiotherapy was not given because of a risk of intestinal wall perforation. Staging positron emission tomography-computed tomography proved to be negative. At the age of 22 years magnetic resonance imaging scans indicated no tumor recurrence, but after one month a rapidly growing tumorous lesion was found on ultrasound in the surgical scar measuring 20 × 20 × 12 mm in size. Cytology confirmed local recurrence and fluorescence in situ hibridization indicated t(x;18). R0 exstirpation and partial mesh resection were performed and histology showed the same monophasic synovial sarcoma. Because of the presence of vascular invasion and a close resection margin (1 mm) the patient underwent 3 cycles of adjuvant chemotherapy (doxorubicine and ifosfamide) with granulocyte colony stimulating factor support and 3 cycles of ifosfamide. After 2 years follow up at the age of 24 years, imaging studies did not reveal any local or distant recurrence.

Marriage and fertility in long-term survivors of childhood, adolescent and young adult (AYA) high-grade sarcoma.

PURPOSE: We investigated the marital status and the presence or absence of children in survivors of childhood, adolescent and young adult (AYA) high-grade sarcoma and examined the influence of these factors on the quality of life (QOL) of these survivors. METHODS: Thirty-eight survivors of childhood and AYA high-grade sarcoma (18 males, 20 females) participated in a questionnaire survey on marital status and presence or absence of children, as well as on the health-related QOL (HR-QOL), using the Short Form 36 Health Survey. Diagnoses among these survivors were osteosarcoma (28 participants), Ewing's sarcoma (4 participants), synovial sarcoma (4 participants) and others (2 participants). RESULTS: Of the 18 males who participated in the survey, eight (44.4 %) were married, of whom five (62.5 %) had children. Fifteen (75.0 %) of the 20 females were married, of whom 14 (93.3 %) had children. The proportions of surviving male patients who were married and who had children, respectively, were lower than those of surviving female patients. The proportion of ifosfamide-treated men with children was significantly lower than that of non-ifosfamide-treated men (p = 0.018). With respect to the relationship between marital status and HR-QOL, the scores for the vitality and mental health domains of the SF-36 of survivors who were married were significantly higher than those of unmarried survivors. CONCLUSIONS: The results of our questionnaire survey reveal that among the male survivors of high-grade sarcoma, the proportions of those who were married and of those with children were lower than those of female survivors, suggesting that strategies providing support for marriage and child-rearing may be necessary for the male survivor group. In the married group, mental QOL was high.

Primary synovial sarcoma of the abdominal wall: a case report and literature review.

Synovial sarcoma (SS) is the fourth most common type of soft tissue sarcoma, following malignant fibrous histiocytoma, liposarcoma, and rhabdomyosarcoma. It usually occurs in the extremities near the large joints of middle-aged patients. We describe a case of synovial sarcoma of the anterior abdominal wall (SSAW) in an adolescent girl and undertake a review of the literature.

Primary Pulmonary Synovial Sarcoma in a Tertiary Referral Center: Clinical Characteristics, CT, and 18F-FDG PET Findings, With Pathologic Correlations.

The purpose of this study was to describe the patient characteristics, computed tomography (CT) and F-fluorodeoxyglucose positron emission tomography (FDG PET) findings, and clinical outcomes of primary pulmonary synovial sarcoma (PPSS), together with their pathologic correlations. The medical records of 14 patients with pathologically proven PPSS in a tertiary hospital from January 1997 to December 2014 were retrospectively reviewed. The CT findings were evaluated. The maximum standardized uptake value (maxSUV) of the tumors was obtained, and clinical outcomes with respect to tumor recurrence and mortality were assessed by Kaplan-Meier analysis. The median tumor size was 10.2 cm and the most common anatomic location was the lung followed by the pleura/chest wall and mediastinum. Most of the tumors appeared as single lesions and had circumscribed margins. All the cases showed heterogeneous enhancement with necrotic or cystic portions, and intratumoral vessels were frequently seen. Half of the tumors had intratumoral calcifications, and tumor rupture, pleural/chest wall extension, and pleural effusion occurred frequently. However, lymph node enlargement was rare. The median maxSUV of the tumors was 4.35. Patient outcomes with respect to tumor recurrence (n = 8, 57.1%) and death (n = 3, 21.4%) were poor despite their young age, and the mean follow-up period was 28.5 months.In conclusion, PPSS usually occurs in young adults, generally in the lung, presents as a large, circumscribed mass, and tumor rupture or extension of the pleura/chest wall may occur. The tumors often contain calcifications and vessels; they may exhibit triple attenuation on enhanced CT images, and clinical outcomes are poor.

Synovial sarcoma in children and adolescents: the European Pediatric Soft Tissue Sarcoma Study Group prospective trial (EpSSG NRSTS 2005).

BACKGROUND: To report the results of the first European prospective nonrandomized trial dedicated to pediatric synovial sarcoma. PATIENTS AND METHODS: From August 2005 to August 2012, 138 patients <21 years old with nonmetastatic synovial sarcoma were registered in 9 different countries (and 60 centers). Patients were treated with a multimodal therapy including ifosfamide-doxorubicin chemotherapy and radiotherapy, according to a risk stratification based on surgical stage, tumor size and site, and nodal involvement. RESULTS: With a median follow-up of 52.1 months (range 13.8-104.4 months), event-free survival (EFS) was 81.9% and 80.7%, and overall survival (OS) was 97.2% and 90.7%, at 3 and 5 years, respectively. The only significant prognostic variable at univariate analysis was the risk group: 3-year EFS was 91.7% for low-risk, 91.2% for intermediate-risk, and 74.4% for high-risk cases. In 24 low-risk patients (completely resected tumor ≤5 cm in size) treated with surgery alone, there were two local relapses and no metastatic recurrences. Among 67 high-risk patients (unresected, or axial tumor or nodal involvement), 66 underwent surgery after neoadjuvant chemotherapy. Response to chemotherapy was 55.2%, including 22.4% cases with complete or major partial remissions, and 32.8% with minor partial remissions. CONCLUSION: This study demonstrates that collaborative prospective studies on rare pediatric sarcomas are feasible even on a European scale, with excellent treatment compliance. The overall results of treatment were satisfactory, with higher survival rates than those previously published by pediatric groups. Nonetheless, larger, international projects are needed, based on a cooperative effort of pediatric and adult oncologists. CLINICAL TRIALS NUMBER: European Union Drug Regulating Authorities Clinical Trials No. 2005-001139-31.

Synovial sarcoma in children and adolescents.

Synovial sarcoma (SS) is a high-grade soft tissue sarcoma characterized by local invasiveness and a propensity to metastasize, affecting pediatric, adolescent, and adult populations. The peak incidence is observed in the third decade of life and SS is the most common nonrhabdomyosarcoma soft tissue sarcoma in childhood and adolescence. Although pediatric and adult SS appear clinically and radiologically identical, treatment modalities may differ according to the patient's age. For many years, pediatric oncologists have treated SS as a chemosensitive tumor according to the "rhabdomyosarcoma philosophy." In contrast, adult oncologists generally treat this tumor as a poorly chemosensitive tumor and focus on local control. The authors propose an update of SS in the pediatric population and analyze their results to those obtained in adults.

Hoffa's fat pad tumours: what do we know about them?

PURPOSE: We report on a group of patients with tumours in the Hoffa's fat pad (HFP), their clinical presentation, histological type and treatment, including two synovial sarcomas with their clinical follow-up, which have not been described previously in the literature. METHODS: We performed a retrospective review of our prospectively collected database of 25 cases of HFP tumours with at least six months follow-up. RESULTS: The gender, age at presentation (over and under 16 years of age), clinical features, history of trauma, treatment chosen, and complications were recorded. The mean age of the patients was 32 years (three to 47). Six patients were under 16 years old. Pain was the most common symptom, present in 92% (n = 23/25). The final diagnoses included 23 (92%) benign tumours and two (8%) malignant tumours. The most common benign tumour was pigmented villonodular synovitis (PVNS) (48% n = 12). The two malignant tumours were synovial sarcomas and both presented in patients under 16 years old. CONCLUSIONS: Hoffa's fat pad tumours are an uncommon and rarely diagnosed group of lesions that can be misinterpreted as any knee pathology. Although the majority of HFP tumours are benign, malignant tumours should be considered in the differential diagnosis for the paediatric population.

Synovial cell sarcoma of the larynx.

Synovial cell sarcoma represents a rare group of cancers, particularly in the head and neck region, that typically affects young individuals and has a male preponderance. Prognosis varies with patient age, site and size of the malignancy, degree of necrosis, high level of mitotic activity, and neurovascular invasion. Complete surgical resection of the tumor via partial or total laryngectomy is the first-line treatment in locally invasive disease. CO(2) lasers have been shown to be useful in controlling localized disease. There is also a role for adjuvant radiotherapy. Ifosfamide-based chemotherapy is most useful for malignant disease.

Sarcomas across the age spectrum.

In most cancers, the age of the affected patient has a significant influence on how that cancer is approached. This is less often the case in sarcomas in that both children and adults are treated similarly. However, different types of sarcomas are most typical in these populations, and our understanding of these cancers in one group has provided direction for understanding them in the other group. For example, advances at the molecular level in Ewing sarcoma, a disease that affects predominantly children, helped spearhead the uncovering of several signature translocations in adult sarcomas, such as synovial sarcoma and myxoid liposarcomas. The success of chemotherapy in pediatric sarcomas continues to be a benchmark for adult sarcomas to emulate. Conversely, the demonstration of the viability of limb-sparing surgery combined with adjuvant radiation in adult extremity sarcomas helped advance that treatment approach in pediatric sarcomas. To illustrate some of these concepts and to enhance our understanding of sarcomas across the age spectrum, 4 types of sarcoma are discussed. The first 2 are Ewing sarcoma and rhabdomyosarcoma seen mainly, but not exclusively, in children. The other 2 are synovial sarcoma, which can be looked at as a bridge between pediatric and adult sarcomas as it affects mainly young adults, and liposarcoma that is almost exclusively an adult sarcoma.

Primary breast synovial sarcoma: a rare primary breast neoplasm

A primary synovial sarcoma based on the breast is rare. The usual tumours on the breast are carcinomas. Synovial sarcomas account for about 6-9% of soft tissue sarcomas and most commonly develop in the extremity of young adults (80%). The other 20% of synovial sarcomas can arise in non-extremity sites (trunk 8%, retroperitoneal/ abdominal 7%, head and neck 5%) but synovial sarcomas can develop in almost any other anatomical location. We report a case of a young woman who presented with a suspected common breast tumour and started treatment of this tumour with carcinoma neoadjuvant chemotherapy. We were surprised when the pathologist identified a synovial sarcoma in the histopathology study (AU)

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El càncer del desenvolupament. Part 5: sarcoma / No disponible

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