Atypical fibroxanthoma and pleomorphic dermal sarcoma: Local recurrence and metastasis in a nationwide population-based cohort of 1118 patients.

BACKGROUND: The prognosis of patients with atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) remains uncertain and no standardized follow-up programs have been established. OBJECTIVE: To recommend a standardized follow-up program of patients with AFX and PDS based on nationwide long-term estimates of local recurrence and metastasis. METHODS: All patients with AFX and PDS in Denmark between 2002 and 2022 were included. Danish National Registries were used to estimate the risks of local recurrence and metastasis for AFX and PDS. RESULTS: The 5-year risk of local recurrence was 10% for AFX and 17% for PDS. The 5-year risk of metastasis was 0.8% for AFX and 16% for PDS. PDS metastasized within 3 years in >90% of the patients with the lungs as the primary metastasis site (50%). Invasion beyond the subcutis, perineural/intravascular infiltration, and increasing age significantly increased the risk of PDS relapse. LIMITATIONS: Risk of misclassification and lack of detailed surgical information. CONCLUSION: The follow-up of patients with AFX can be limited to clinical visits for 4 years. Patients with PDS should be followed with clinical visits and PET/CT twice a year for the first 3 years and once a year for a minimum of 1 year.

Patterns of head and neck sarcoma in Australia.

BACKGROUND: Sarcomas affecting the head and neck often require complex management due to the combination of anatomic, aesthetic and oncological considerations. The incidence and patterns of presentation are poorly understood and have not been reviewed in the Australian population. METHOD: This study sourced incidence and demographic data from the National Cancer Registry at the Australian Institute of Health and Welfare for the years 1982-2009 (corresponding to 97.3% of the Australian population). All cases of sarcoma, according to ICD-O-3 classification ((International Classification of Diseases for Oncology, 3rd edition), were assessed. RESULTS: A total of 3911 new cases of sarcoma affecting the head and neck were recorded during the period 1982-2009, including 1383, 2106 and 442 cases arising from skin, soft tissue and bone, respectively. The annual incidence rate of sarcomas affecting the head and neck was 1.59 per 100 000 population. The incidence of head and neck sarcoma rose substantially in older age groups (age 65 years and above) and was most common in male patients (69%). Malignant fibrous histiocytoma (MFH) was the most common pathology. There was an increase in incidence in skin-origin sarcoma in the head and neck, particularly affecting elderly males. CONCLUSION: The incidence of head and neck sarcoma in Australia is higher than that reported for an equivalent European population. The increase in MFH arising from the skin in elderly male patients mirrors the patterns of common cutaneous malignancy, particularly melanoma, suggesting that ultraviolet radiation is an epidemiological factor. Management of head and neck sarcoma is complex and best managed in a specialist multidisciplinary environment.

Malignant fibrous histiocytoma: Database review suggests a favorable prognosis in the head and neck.

OBJECTIVE: The malignant fibrous histiocytoma (MFH) is the most common soft tissue sarcoma of the head and neck. Currently, most of the data on this tumor relies on small retrospective studies. The objective of this study is to use the Surveillance, Epidemiology, and End Results (SEER) database to compare characteristics of this tumor based on location to better understand its prognosis in the head and neck region. This article represents the largest study analyzing prognosis of this tumor in the head and neck to date. STUDY DESIGN: Retrospective analysis of SEER database. METHODS: Using the SEER database, 395 patients with MFH of the head and neck were compared with 3,968 patients with MFH of the trunk and extremities. Disease-specific survival was carried out comparing these two cohorts, as well as univariate and multivariate analysis to determine hazard ratios. RESULTS: Head and neck MFH had a significantly higher disease-specific survival compared with trunk and extremity disease. However, head and neck tumors were more frequently a smaller size (P < .0001) and lower grade (P < .0001). Larger tumors and grade III and IV tumors conferred a worse prognosis (P < .0001). CONCLUSION: Head and neck malignant fibrous histiocytoma presents at a smaller size and lower grade, likely due to earlier presentation in this region. Because of this, head and neck malignant fibrous histiocytoma represents a more favorable survival prognosis compared with trunk and extremity disease. LEVEL OF EVIDENCE: 4. Laryngoscope, 128:885-888, 2018.

[Soft tissue tumors : Epidemiology, classification and staging]. / Weichteiltumoren : Epidemiologie, Klassifikation und Stadieneinteilung.

Benign, intermediate and malignant soft tissue tumors can be differentiated histologically. Furthermore, the tumors can be subdivided according to their linear differentiation. In the new World Health Organization (WHO) classification of soft tissue tumors from 2013 changes have been made relating to the allocation of known entities, e. g. undifferentiated sarcomas have been formed into a new subgroup and are no longer assigned to the fibrohistiocytic tumors. The term malignant fibrous histiocytoma has been replaced by the undifferentiated sarcoma. Furthermore, two new subgroups were incorporated, the nerve sheath tumors and gastrointestinal stromal tumors. These were previously included in the tumor classification of other organ systems. These changes in the new classification are related to the rapid increase in knowledge of the genetics and the cell biology of soft tissue tumors. Malignant soft tissue tumors only represent 1% of all malignant tumors in adults. The largest subgroup of soft tissue tumors in adults is the adipocytic tumors. The liposarcoma, which belongs to this subgroup is one of the most common malignant soft tissue tumors in adults. In childhood malignant soft tissue tumors represent 15% of malignant tumors and rhabdomyosarcoma is the most common malignant soft tissue tumor.

Clinico-epidemiological profile of breast cancer patients and the retrospective application of Gail model 2: An Indian perspective.

AIM: To study the clinical and epidemiological profile of patients of breast cancer presenting at our center at New Delhi, India and to evaluate the applicability of Gail model 2 as a means of measuring 5-year and lifetime risk in our already diagnosed cases of breast cancer. METHODS: This was a retrospective study conducted at Lady Hardinge Medical College Hospital in New Delhi, India, between January 2011 and July 2014. Two hundred and twenty two diagnosed cases of breast cancer were included. Information was collected retrospectively on a Performa from the medical record section and the Pathology department of the hospital.The predicted five-year and lifetime risk was calculated using GM2 prediction model from the NCI's breast cancer risk assessment tool website. RESULTS AND CONCLUSIONS: Breast cancer in India is a far more biologically aggressive disease than in the west with a widely different spectrum of presentation and behavior and late presentation in an advanced stage. The accepted risk factors routinely associated with breast cancer in western literature do not appear to be relevant in the Indian population. Accepted western models do not seem to apply in the Indian scenario.

Increased incidence of malignant melanoma and other rare cutaneous cancers in the setting of chronic lymphocytic leukemia.

BACKGROUND: Patients with chronic lymphocytic leukemia (CLL), small lymphocytic lymphoma (SLL), or non-Hodgkin lymphoma (NHL) are at increased risk for the development of skin malignancies. OBJECTIVES: This study was conducted to estimate the incidences of rare skin malignancies in patients with CLL/SLL or NHL. METHODS: Patients with a diagnosis of CLL/SLL or NHL recorded in the Surveillance, Epidemiology and End Results (SEER) database during 1992-2007 were identified. Diagnoses of specific skin malignancies were identified from SEER files. RESULTS: During 1992-2007, a total of 128,674 patients with first diagnoses of CLL/SLL or NHL were recorded in SEER; 4743 were excluded because follow-up data were unavailable. Among the remaining 123,931 patients, 28,964 had CLL/SLL and 94,967 had NHL. Standardized incidence ratios (SIRs) for invasive malignant melanoma, Merkel cell carcinoma, malignant fibrous histiocytoma, dermatofibrosarcoma protuberans, Kaposi's sarcoma, and sebaceous carcinoma were 2.3, 8.2, 3.6, 2.5, 2.9, and 1.4, respectively, in CLL/SLL patients and 1.6, 3.2, 1.5, 1.3, 17.6, and 0.8, respectively, in NHL patients. When invasive melanoma was stratified by patient age and sex, the highest SIR (17.8) was found in men aged 0-49 years with CLL (P < 0.001). CONCLUSIONS: Patients with CLL/SLL or NHL have a higher risk for the subsequent development of rare skin cancers. Given the more aggressive nature of these malignancies in this setting, regular monitoring for the development and prompt treatment of cutaneous malignancy is prudent in patients with NHL and particularly in patients with CLL. Regular use of sun protection may decrease the morbidity associated with skin cancer in this immunosuppressed population.

Bone malignancies in orthopaedic hospital Igbobi Lagos, Nigeria.

AIMS AND OBJECTIVES: To document the pattern of bone malignancies in a highly populated orthopaedic hospital in Lagos Nigeria; PATIENTS AND METHODS: A total of 21 cases of primary malignant bone tumours were studied. This comprised 12 cases of Osteosarcoma, 7 cases of Malignant Fibrous Histiocytoma (MFH) and 2 cases of Chondrosarcoma. Males (13) were affected more than females (8) giving a male to female ratio of 1.6 to 1. The age range was 7 to 45 years with a median age of 24 years. The diameter of the swelling ranged from 6 to 20 cm with a median of 12 cm. All patients had ablative surgery except for those with affectation of the ilium. Data was analysed using the Statistical Package for Social Sciences (SPSS 16). Enneking's classification was used to grade the tumour. The duration of symptoms of all the patients before presentation ranged from 3 weeks to 4 years with a mean of 7 months. The commonest site affected was around the knee (76.2%); distal femur had 42.9% and proximal tibia 33.3%. RESULTS: Osteosarcoma was the most common malignant bone tumour in this series and accounted for 57.1%. The peak incidence was found in the 2nd decade of life. The youngest patient was 7 years old and the oldest 43 years. The tumour was found primarily around the knee. 7 cases were in the distal part of the femur, 4 in the proximal part of the tibia and 1 case was found in the distal radius. Out of the 12 patients with osteosarcoma, 8 had paraosteal type (5 high grade, 3 intermediate grade), the remaining 4 had periosteal (all high grade) Malignant Fibrous Histiocytoma was found in 7 patients and accounted for 33.3%. The peak incidence was found in 3rd and 4th decades. 4 out of the 7 patients were high grade pleomorphic osteosarcoma, 2 were myxoid high grade dedifferentiated and one was low grade giant cell tumour type. Chondrosarcoma was found in 2 patients, accounting for 9.5%. both cases were in the ilium CONCLUSION: Primary malignant bone tumours occurred in children and young adult in this study. It is commoner among males and most of the patients presented late to the hospital. Osteosarcoma is the commonest followed by Malignant Fibrous Histiocytoma, both occurred commonly around the knee and chondrosarcoma on the ilium.

Head and neck sarcomas: epidemiology, pathology, and management.

Sarcomas of the head, neck, and skull base represent a heterogeneous group of tumors with distinct prognostic features. There have been significant improvements in characterizing these sarcomas using traditional morphologic assessments and more recent immunohistochemical analysis. Surgery is the mainstay of treatment followed by radiation therapy. Treatment modalities have changed in select pediatric sarcomas, for which new chemotherapeutic combinations have improved survival statistics. The high rate of distant failure emphasizes the need for novel systemic and directed molecular therapies. Tumor grade, size, and margin status are key factors in survival.

Clinicohistopathological analysis of 5 Nigerian cases of malignant fibrous histiocytoma of the jaws.

BACKGROUND: Malignant fibrous histiocytoma (MFH), is a soft tissue sarcoma that occurs predominantly in the soft tissue of the extremities. It rarely occurs in facial bones. Few cases in the jaws have been reported. OBJECTIVE: To report and review the relevant clinicopathologic features of 5 cases of jaw MFHin Nigerians. METHODS: All cases in the records of the Department of Oral Pathology of the Lagos University Teaching Hospital which were histologically diagnosed as MFH were retrieved. Hematoxylin and eosin slides of cases were re-examined to confirm diagnosis. Information retrieved included age, sex, location, x-ray, estimated duration, recurrence, and histological presentation. Data was analyzed using SPSS statistical package. RESULTS: Age ranged between 12-42 years with a male sex predilection (3 cases/60.0%) and sole maxillary site predilection (5 cases/100.0%). Estimated duration of lesion ranged from 2 -12 months. Two cases recurred post surgical treatment. All cases presented as the storiform-pleomorphic type with one case presenting with a delicate/scanty connective tissue stroma. Chronic inflammation was scanty in the two cases that recurred. CONCLUSION: MFH of the jaws is rare among Nigerians. Its clinico radiological presentation maymimick other more aggressive or less benign lesions of the jaws thereby resulting in misdiagnosis and in appropriate patient management.

Uncommon cutaneous neoplasms of the head and neck.

This article concentrates on the less-common cutaneous malignancies such as merkel cell, atypical fibroxanthoma, malignant fibrous histiocytoma, dermatofibrosarcoma protuberans, microcystic adnexal carcinoma, and sebaceous carcinoma. The clinical and histopathologic descriptions of each, most current and emerging etiologies, diagnosis, staging, treatment, and prognosis are discussed.

A fifty-year review of soft tissue sarcomas in Jamaica: 1958-2007 / Cincuenta años de revisión de los sarcomas de tejidos blandos en Jamaica: 1958-2007

OBJECTIVE: To determine the distribution of histologic subtypes of soft tissue sarcomas (STS) in Kingston and St Andrew, Jamaica, according to age and topography. METHODS: From the Jamaica Cancer Registry (JCR) archives, all cases of STS diagnosed between 1958 and 2007 were extracted. For each case, age, gender, histological diagnosis and anatomical site of tumour were recorded. Patients were categorized according to age at diagnosis as: children (0-14 years) and adults (> 14 years), and the distribution of histologic diagnoses with respect to age and anatomical site were analysed. RESULTS: There were 432 cases (67 children, 364 adults, one person of unknown age) of STS recorded in the JCR over the 50-year period (218 males, 214 females). The commonest STS in adults were "sarcoma, not otherwise specified [NOS]" (20.1%), malignant fibrous histiocytoma [MFH] (17.9%), fibrosarcoma (12.4%), liposarcoma (10.7%) and malignant peripheral nerve sheath tumour [MPNST] (10.2%). In children, they were neuroblastoma (38.8%), rhabdomyosarcoma (23.9%), "sarcoma, NOS" (9%), fibrosarcoma (6%) and MFH (6%). In adults, the lower limb was the commonest location, followed by trunk and/or upper limb for MFH, fibrosarcoma and liposarcoma, and head and neck for MPNST. In children, head and neck was the commonest site for rhabdomyosarcoma, head and neck and upper limb for MFH, retroperitoneum for neuroblastoma and trunk for fibrosarcoma. CONCLUSION: A high proportion of soft tissue sarcomas in Jamaica are unclassified and the anatomical distribution of common classified sarcomas shows some differences with the literature. Limited access to immunohistochemistry/molecular diagnostics and increasing core biopsy diagnosis may contribute to these phenomena.

OBJETIVO: Determinar la distribución de subtipos histológicos de sarcomas de tejido blando (STB) en Kingston y Saint Andrew, Jamaica, según la edad y la topografía. MÉTODOS: De los archivos del Registro de Cáncer de Jamaica, se extrajeron todos los casos de STB diagnosticados entre 1958 y 2007. Para cada uno de los casos, se registró la edad, el género, el diagnóstico histológico, y el sitio anatómico del tumor. Los pacientes fueron clasificados de acuerdo con la edad en el momento del diagnóstico, bajo las categorías de niños (0-14 años) y adultos (> 14 años), y se analizó la distribución de diagnósticos histológicos con respecto a la edad y el sitio anatómico. RESULTADOS: Se registraron 432 casos de STB (67 niños, 364 adultos, una persona de edad desconocida) en el JCR en un período de 50 años (218 varones, 214 hembras). Los STB más comunes en los adultos fueron "el sarcoma no especificado [NE]" (20.1%), el histiocitoma fibroso maligno [HFM] (17.9%), el fibrosarcoma (12.4%), el liposarcoma (10.7%), y el tumor maligno de la vaina del nervio periférico [TMVNP] (10.2%). En los niños, se trató de los neuroblastomas (38.8%), los rabdomiosarcomas (23.9%), "los sarcomas NE" (9%), los fibrosarcomas (6%), y los HFM (6%). En los adultos, los miembros inferiores fueron el lugar más común, seguido del tronco y/o los miembros superiores para el HFM, el fibrosarcoma y el liposarcoma; y la cabeza y el cuello para el TMVNP. En los niños, la cabeza y el cuello fueron el sitio más común para el rabdomiosarcoma; la cabeza, el cuello y los miembros superiores para el HFM; el retroperitoneo para el neuroblastoma; y el tronco para el fibrosarcoma. CONCLUSIÓN: Una proporción alta de sarcomas de tejidos blandos en Jamaica no están clasificados, y la distribución anatómica de sarcomas clasificados comunes muestran algunas diferencias con la literatura. El acceso limitado a los diagnósticos moleculares/inmunohistoquímicos, y el aumento de los diagnósticos centrales, pueden contribuir a estos fenómenos.

Sarcomas other than Kaposi sarcoma occurring in immunodeficiency: interpretations from a systematic literature review.

PURPOSE OF REVIEW: In immunodeficiency, an increased sarcoma risk is confirmed for Kaposi's sarcoma. Whether rates of other sarcoma subtypes are elevated in the setting of immunodeficiency is not known. We therefore reviewed published case reports on HIV and AIDS patients and organ transplant recipients with sarcomas. For comparison, we assessed sarcomas in the U.S. general population using Surveillance Epidemiology End Results (SEER) data. RECENT FINDINGS: A total of 176 non-Kaposi sarcoma were identified, 75 in people with HIV and AIDS and 101 in transplant recipients. Leiomyosarcomas (n = 101) were the most frequently reported sarcomas, followed by angiosarcomas (n = 23) and fibrohistiocytic tumors (n = 17). Leiomyosarcomas were reported with two age peaks, in children and young adults. Epstein-Barr virus (EBV) was detected in the tumor cells in 85 and 88% of leiomyosarcomas in HIV-infected people and transplant recipients, respectively. Angiosarcomas and fibrohistiocytic tumors were most frequently reported in men. Among kidney transplant recipients, 20% of sarcomas arose at the site of an arteriovenous fistula. In comparison, leiomyoscarcomas, angiosarcomas, and fibrohistiocytic tumors comprised 16.9, 3.8, and 18.7% of sarcomas in the U.S. general population. SUMMARY: Leiomyosarcoma and angiosarcoma may occur disproportionately in immunodeficiency. Leiomyosarcomas appear causatively linked to EBV, whereas angiosarcomas might be correlated with an arteriovenous fistula. Additional studies are necessary to understand the contribution of immunodeficiency to the cause of these sarcomas.

A fifty-year review of soft tissue sarcomas in Jamaica: 1958-2007.

OBJECTIVE: To determine the distribution of histologic subtypes of soft tissue sarcomas (STS) in Kingston and St Andrew, Jamaica, according to age and topography. METHODS: From the Jamaica Cancer Registry (JCR) archives, all cases of STS diagnosed between 1958 and 2007 were extracted. For each case, age, gender, histological diagnosis and anatomical site of tumour were recorded. Patients were categorized according to age at diagnosis as: children (0-14 years) and adults (> 14 years), and the distribution of histologic diagnoses with respect to age and anatomical site were analysed. RESULTS: There were 432 cases (67 children, 364 adults, one person of unknown age) of STS recorded in the JCR over the 50-year period (218 males, 214 females). The commonest STS in adults were "sarcoma, not otherwise specified [NOS]" (20.1%), malignant fibrous histiocytoma [MFH] (17.9%), fibrosarcoma (12.4%), liposarcoma (10.7%) and malignant peripheral nerve sheath tumour [MPNST] (10.2%). In children, they were neuroblastoma (38.8%), rhabdomyosarcoma (23.9%), "sarcoma, NOS" (9%), fibrosarcoma (6%) and MFH (6%). In adults, the lower limb was the commonest location, followed by trunk and/or upper limb for MFH, fibrosarcoma and liposarcoma, and head and neck for MPNST. In children, head and neck was the commonest site for rhabdomyosarcoma, head and neck and upper limb for MFH, retroperitoneum for neuroblastoma and trunk for fibrosarcoma. CONCLUSION: A high proportion of soft tissue sarcomas in Jamaica are unclassified and the anatomical distribution of common classified sarcomas shows some differences with the literature. Limited access to immunohistochemistry/molecular diagnostics and increasing core biopsy diagnosis may contribute to these phenomena.

Mast cell tumour and cutaneous histiocytoma excision wound healing in general practice.

OBJECTIVE: To assess whether wounds from incomplete mast cell tumour excisions are at greater risk of healing complications than wounds from complete excisions, or cutaneous histiocytomas. METHODS: Mast cell tumours and cutaneous histiocytomas submitted to Nationwide Laboratories between November 1, 2007 and April 30, 2008 were selected. Questionnaires were sent to submitting veterinarians requesting details of tumour characteristics, clinical approach to the tumour and wound healing. RESULTS: Three hundred and eighty-six mast cell tumours and 524 cutaneous histiocytomas were identified. One hundred and eighty-five mast cell tumours and 244 cutaneous histiocytomas questionnaires were returned (47% response). Wound complications arose in 20% of mast cell tumours and 21% of cutaneous histiocytomas. Multivariable analysis confirmed that larger tumours, tumours on the feet and a soft/"baggy" appearance, were significantly associated with a greater frequency of problems, leading to delayed wound healing and dehiscence. CLINICAL SIGNIFICANCE: Incomplete mast cell tumour excision does not lead to greater risk of wound complications. Mast cell tumour surgical wounds have a similar rate of wound complications as cutaneous histiocytoma wounds.

Increased metastasis of malignant fibrous histiocytoma in patients with chronic lymphocytic leukemia and non-hodgkin lymphoma.

OBJECTIVE: To further understand the characteristics and behavior of malignant fibrous histiocytoma (MFH) in the clinical setting of chronic lymphocytic leukemia (CLL) or non-Hodgkin lymphoma (NHL). PATIENTS AND METHODS: The patient database of MHF diagnosed at our institution from January 1, 1976, to December 31, 2008, was reviewed. For each MFH case with prior NHL or CLL, 3 matched controls from the same cohort without prior CLL or NHL were randomly selected. A retrospective chart review collected patient data, including sex; age; pathologic confirmation of MFH; tumor grade, size, and location; time since MFH diagnosis; history of chemotherapy or irradiation; treatment of MFH; recurrence; metastasis; and death. The Kaplan-Meier method was used to estimate overall survival, survival free of recurrence, and survival free of metastasis. Cox proportional hazards models were fit to evaluate associations between case status and outcomes. RESULTS: Fifteen MFH cases with prior NHL or CLL were identified. Cases had frequent bone (n=7 [47%]) and cutaneous (n=5 [33%]) involvement. Five cases (33%) had previous irradiation, 6 (40%) had metastases, and 4 (27%) had recurrence. Overall survival and survival free of recurrence were not significantly different between the cases and controls (5-year overall survival, 49.9% and 58.7%; 12-month survival free of recurrence, 79.4% and 90.3%). However, cases were significantly more likely than controls to have metastasis (hazard ratio, 3.79; 95% confidence interval, 1.22-11.79; P=.02). In addition, survival free of metastasis at 12 months was 65.2% and 90.7%, respectively. CONCLUSION: In the presence of CLL or NHL, MFH seems to behave more aggressively, suggested by the increased likelihood of metastases compared with controls without prior CLL or NHL. However, overall survival does not appear to be worse in cases of MFH and CLL or NHL than in MFH alone.

Abdominal soft tissue sarcoma: a multicenter retrospective study.

BACKGROUND: Soft tissue sarcomas (STS) are rare mesenchymal neoplasms with a variety of histological subtypes. However, in Japan, data on the clinical characteristics and prognostic profiles of these tumors are lacking. The purpose of the present study was to clarify the clinical features and outcomes of Japanese patients with retroperitoneal and abdominal STS. METHODS: We reviewed and analyzed retrospectively the data for 82 patients who underwent surgery for retroperitoneal and abdominal STS at Osaka University and affiliated hospitals from 2000 to 2007. The factors analyzed included patient demographics and clinical features. RESULTS: The histological subtypes included leiomyosarcoma in 32 patients (39.0%), liposarcoma in 30 (36.6%), malignant fibrous histiocytoma in 10 (12.2%), and other miscellaneous subtypes in 10 (12.2%). The overall survivals were 92, 69, and 62%, respectively, at 1, 3, and 5 years after primary surgery. The overall survival of patients with low-grade sarcoma was significantly better than that of patients with high-grade sarcoma. Complete resection was done in 63 patients (77%) and their recurrence-free survivals were 73, 34, and 23%, respectively, at 1, 3, and 5 years after the surgery. Subgroup analysis of differences between leiomyosarcoma and liposarcoma revealed that liposarcomas were mainly located in the retroperitoneum and leiomyosarcomas were located equally in the retroperitoneum and abdominal cavity. The tumor size of liposarcomas was larger than that of leiomyosarcomas; however, the recurrence-free survival was better in patients with liposarcoma than in those with leiomyosarcoma. CONCLUSION: Our results showed the clinical features and prognoses of retroperitoneal and abdominal STS in Japan. Further large-scale nationwide studies are required to clarify the detailed clinical behavior of retroperitoneal and abdominal STS in Japan.

Giant solitary fibrous tumor of the pleura.

BACKGROUND: Solitary fibrous tumor is the second primary malignancy of the pleura and can reach up to 39 cm in diameter; however, to be referred to as 'giant' it must occupy at least 40% of the affected hemithorax. Although this tumor usually shows a benign behavior, malignancy criteria have been described. The aim of the study was to assess the initial evaluation, diagnostic procedures, surgical management, treatment outcome, and prognosis. METHODS: We performed a descriptive, observational, longitudinal, and retrospective study from 2002 to 2006 on patients who underwent surgery with a diagnosis of giant solitary fibrous tumor of the pleura. RESULTS: Six patients were included; 83.3% were females. Mean age was 48 years. All patients were symptomatic, mainly dyspnea, cough and chest pain; 66.7% were left-sided. Preoperative angiography and embolization were performed in 83.3% cases with successful surgical resection. The predominant blood supply was derived from the internal mammalian artery. Intraoperative complication rate was 17%. A vascular pedicle was found in 66.7%. The largest lesion was 40 cm in diameter and weighed 4500 g. Only one case showed high mitotic activity. Mean follow-up to date is 14 months. CONCLUSIONS: Symptomatology found was consistent with previous reports but in higher percentages. Accurate diagnosis is critical because surgical resection involves a potential cure; however, long-term follow-up is mandatory. Preoperative embolization is recommended due to tumor size.

Tumor fibroso solitario gigante de la pleura / Giant solitary fibrous tumor of the pleura

Introducción: El tumor fibroso solitario es el segundo tumor primario de la pleura y puede alcanzar hasta 39 cm de diámetro; para tener la denominación de “gigante” debe ocupar al menos 40 % del hemitórax afectado. Por lo general su comportamiento es benigno, pero existen criterios de malignidad. El objetivo de esta investigación fue efectuar una revisión de la evaluación inicial, diagnóstico, manejo quirúrgico, resultado del tratamiento y pronóstico. Material y métodos: Estudio descriptivo, observacional, longitudinal y retrospectivo, realizado de 2002 a 2006, en pacientes operados con diagnóstico de tumor fibroso solitario gigante de la pleura. Resultados: Se incluyeron seis pacientes, 83.3 % del sexo femenino, con edad promedio de 48 años; todos sintomáticos con predominio de disnea, tos y dolor; en 66.7 % se encontró del lado izquierdo; a 83.3 % se realizó angiografía y embolización preoperatorias, logrando resección completa en todos; predominó aporte arterial de la arteria mamaria interna. Se encontró una tasa de complicaciones transoperatorias de 17 %. En 66.7 % se identificó un pedículo ascular; el tumor mayor midió 40 cm de diámetro con peso de 4500 g; solo uno presentó actividad mitótica elevada. El seguimiento promedio fue de 14 meses. Conclusiones: La sintomatología encontrada fue acorde con informes previos, aunque en porcentajes mayores. El diagnóstico correcto es de vital importancia, ya que con la resección quirúrgica el tumor fibroso solitario es potencialmente curable, sin embargo, requiere seguimiento a largo plazo. Dado el tamaño de este tipo de tumores es aconsejable llevar a cabo embolización preoperatoria.

BACKGROUND: Solitary fibrous tumor is the second primary malignancy of the pleura and can reach up to 39 cm in diameter; however, to be referred to as 'giant' it must occupy at least 40% of the affected hemithorax. Although this tumor usually shows a benign behavior, malignancy criteria have been described. The aim of the study was to assess the initial evaluation, diagnostic procedures, surgical management, treatment outcome, and prognosis. METHODS: We performed a descriptive, observational, longitudinal, and retrospective study from 2002 to 2006 on patients who underwent surgery with a diagnosis of giant solitary fibrous tumor of the pleura. RESULTS: Six patients were included; 83.3% were females. Mean age was 48 years. All patients were symptomatic, mainly dyspnea, cough and chest pain; 66.7% were left-sided. Preoperative angiography and embolization were performed in 83.3% cases with successful surgical resection. The predominant blood supply was derived from the internal mammalian artery. Intraoperative complication rate was 17%. A vascular pedicle was found in 66.7%. The largest lesion was 40 cm in diameter and weighed 4500 g. Only one case showed high mitotic activity. Mean follow-up to date is 14 months. CONCLUSIONS: Symptomatology found was consistent with previous reports but in higher percentages. Accurate diagnosis is critical because surgical resection involves a potential cure; however, long-term follow-up is mandatory. Preoperative embolization is recommended due to tumor size.

Imaging findings, prevalence and outcome of de novo and secondary malignant fibrous histiocytoma of bone.

OBJECTIVE: To evaluate the radiographic and magnetic resonance (MR) imaging features of primary and secondary malignant fibrous histiocytoma in bone and determine the demographics, prevalence and outcome of patients with this tumor. MATERIALS AND METHODS: A retrospective search of files from two institutions identified 28 patients with malignant fibrous histiocytoma (MFH) of bone. Microscope slides were reviewed to confirm diagnosis and identify any pre-existing lesions. Medical records were reviewed with respect to patients' demographic characteristics and outcomes. RESULTS: Radiographic features demonstrated an aggressive osteolytic lesion with a permeative pattern of bone destruction. Periosteal reaction was seen in three of 13 lesions. T1-weighted images (T1WIs) demonstrated signal intensity iso- to slightly hyperintense to muscle. T2-weighted images (T2WIs) demonstrated mildly higher signal intensity than that of muscle. The 5-year survival rate was 53%. The tumor arose secondarily in pre-existing lesions in 43% of patients. Metastases occurred in 46% of patients during the course of the disease, with pulmonary and osseous metastases being the most common. CONCLUSION: Secondary MFH of bone was slightly less common than primary MFH and had a prognosis similar to that of primary MFH of bone. MR imaging showed variable and somewhat unusual low to intermediate T2 signal characteristics for a radiographically malignant osteolytic lesion.