Radiation therapy for recurrent cardiac undifferentiated pleomorphic sarcoma after three operations.

We report the case of a 57-year-old female suffering from recurrent malignant undifferentiated pleomorphic sarcoma of the left atrium. Metastasis to the posterior mediastinum was detected upon first presentation. Incomplete resections were carried out twice before mitral valve replacement was finally performed. The tumor recurred 16 months later and was treated with radiation therapy, which has proved to be effective in bringing about tumor regression for 2 years, to date. The patient has survived for 7 years since the first surgery.

Treatment outcomes of malignant fibrous histiocytoma of the maxillary sinus.

BACKGROUND: Malignant fibrous histiocytoma (MFH) is an uncommon neoplasm of maxillary sinus. This study was designed to investigate the clinical courses and summarize the experience of the treatment outcomes. MATERIALS AND METHODS: This is a retrospective analysis which included patients with MFH of the maxillary sinus from January 1980 to December 2008 treated in our institute. Survival data were analyzed by means of the Kaplan-Meier method using univariate analysis and Cox regression model using multivariate analysis. RESULTS: The study included 31 patients. Of which, one patient received radical radiotherapy, and thirty patients received surgical resection. Of these, thirty patients received preoperative radiotherapy, and 11 patients received postoperative radiotherapy. Radical resection with clear surgical margins was obtained in twelve patients (80%) after preoperative radiotherapy, and in eight patients (53.3%) with postoperative radiotherapy (P = 0.221). The 3-year overall survival rate (OS) and recurrence-free survival rate (RFS) were 59.0% and 43.5%, respectively. Patients treated with a radical resection had significantly better survival than patients with a nonradical resection. Three-year OS was 79.8% and 28.1%, 3-year RFS was 61.9% and 18.5%, respectively (P = 0.001 and 0.029, respectively). Local recurrence rate (LRR) was lower in patients resected with clear surgical margins than patients with unclear or uncertain margins, and the 3 years LRR was 33.3% and 72.2%, respectively (P = 0.052). CONCLUSIONS: Surgical resection is the mainstay of treatment for MFH of the maxillary sinus. A radical resection with clear margins is essential for excellent local control and long-term survival.

High-grade myxofibrosarcoma of the abdominal wall.

The authors present a case of a 57-year-old man, who presented to the surgical clinic with a mass in the suprapubic region. A CT scan revealed a well-circumscribed lobular, heterogeneous soft tissue mass measuring 12×8.6×7.8 cm. The final histopathological diagnosis from the resection of the lesion was a myxofibrosarcoma (MFS), grade 3. The management of MFS includes surgical and oncological options which are reviewed here. These are aimed at complete excision and reducing the risk of local occurrence.

En bloc spondylectomy for primary malignant fibrous histiocytoma of the thoracic spine with aortic involvement: case report.

In this article, the authors describe a 48-year-old man who initially presented with progressively worsening back pain. Magnetic resonance imaging revealed a soft-tissue mass involving the T10-11 vertebral bodies with extension anteriorly into the aorta as well as epidural extension without spinal cord compression. A biopsy of the mass showed findings consistent with a malignant fibrous histiocytoma (MFH). A total en bloc spondylectomy with resection and reconstruction of the involved aorta using a vascular graft was performed. The patient received postoperative radiation therapy and is neurologically intact at 18 months postoperatively. To the authors' knowledge, this is the first reported case of a spinal MFH resection with aortic reconstruction.

Anticipated intraoperative electron beam boost, external beam radiation therapy, and limb-sparing surgical resection for patients with pediatric soft-tissue sarcomas of the extremity: a multicentric pooled analysis of long-term outcomes.

PURPOSE: To perform a joint analysis of data from 3 contributing centers within the intraoperative electron-beam radiation therapy (IOERT)-Spanish program, to determine the potential of IOERT as an anticipated boost before external beam radiation therapy in the multidisciplinary treatment of pediatric extremity soft-tissue sarcomas. METHODS AND MATERIALS: From June 1993 to May 2013, 62 patients (aged <21 years) with a histologic diagnosis of primary extremity soft-tissue sarcoma with absence of distant metastases, undergoing limb-sparing grossly resected surgery, external beam radiation therapy (median dose 40 Gy) and IOERT (median dose 10 Gy) were considered eligible for this analysis. RESULTS: After a median follow-up of 66 months (range, 4-235 months), 10-year local control, disease-free survival, and overall survival was 85%, 76%, and 81%, respectively. In multivariate analysis after adjustment for other covariates, tumor size >5 cm (P=.04) and R1 margin status (P=.04) remained significantly associated with local relapse. In regard to overall survival only margin status (P=.04) retained association on multivariate analysis. Ten patients (16%) reported severe chronic toxicity events (all grade 3). CONCLUSIONS: An anticipated IOERT boost allowed for external beam radiation therapy dose reduction, with high local control and acceptably low toxicity rates. The combined radiosurgical approach needs to be tested in a prospective trial to confirm these results.

Radiation therapy for retroperitoneal sarcoma.

PURPOSE: Retroperitoneal sarcomas (RPS) are rare tumours with an annual reported incidence of 2.7 per million persons. In spite of improvements in both diagnostic imaging and therapeutic strategies, patients afflicted by RPS still have poor prognoses. There are currently many different therapeutic strategies for these rare tumours and combining several different multi-modality strategies have not proved to have superior long-term clinical results. This review analyses the available published data and discusses multi-modality management of this rare entity. In particular, the role of radiation therapy, treatment-related side effects and the use of modern radiation treatment techniques will be discussed. MATERIALS AND METHODS: A comprehensive literature search was conducted using PubMed in January 2011. Relevant international articles published from January 1980 to January 2011 were assessed. The keywords for search purposes were: retroperitoneum, sarcoma, radiotherapy, and radiation therapy. The search was limited to articles published in English. All articles were read in full by the authors and selected for inclusion based on relevance to this article. CONCLUSIONS: The addition of radiation therapy (RT) to wide surgical excision for RPS has improved local control rates when compared with surgery alone. Preoperative RT is preferred over postoperative RT. New types and delivery techniques in radiation therapy could further improve patient outcomes. Emerging therapies that employ charged particles (such as protons and carbon ions) are expected to be superior in sparing of normal tissues and efficacy over conventional photon therapy radiation, due to their physical and radiobiological properties.

Combined chemoradiation for head and neck region myxofibrosarcoma of the maxillary sinus.

AIMS AND BACKGROUND: Adult sarcomas of the head and neck region (HNSs) are considered a rare clinicopathological entity. They account for only 2-15% of all adult sarcomas and for less than 1% of all head and neck malignancies. The preferred initial treatment option is wide surgical excision. Whenever surgery is considered infeasible, a frontline combined-modality approach including radiotherapy and chemotherapy might be proposed. We here report on a case of localized sarcoma of the maxillary sinus treated with induction chemotherapy and subsequent intensity-modulated radiation therapy (IMRT), achieving a persistent complete remission status. METHODS: A 66-year-old man was referred to our institution hospital for left-sided facial pain with swollen left cheek and ipsilateral facial palsy. Magnetic resonance imaging showed a mass within the left maxillary sinus extending to the orbital floor and adjacent alveolar bones. Histological examination of the biopsy specimen demonstrated a myxofibrosarcoma. The patient underwent induction chemotherapy with gemcitabine 900 mg/m2 (days 1-8) and taxotere 80 mg/m2 every 3 weeks for 3 cycles and sequential simultaneous integrated boost (SIB) IMRT up to a total dose of 70 Gy/35 fractions to the macroscopic disease with 59.5 Gy/35 fractions to the level IB-II lymph nodes in the left neck. RESULTS: Treatment was well tolerated with mild acute toxicity. Complete remission was achieved at restaging MRI 6 months after the end of the combined modality approach. The patient remains in complete, unmaintained clinical and instrumental complete remission 18 months after treatment, with no late side effects. CONCLUSION: Combination therapy with induction chemotherapy and sequential SIB-IMRT could therefore be a promising modality for head and neck sarcomas, allowing for simultaneous tumor control and normal tissue sparing.

Histiocitoma fibroso maligno / Malignant fibrous histiocytoma

Introducción: los sarcomas son tumores malignos de partes blandas que representan 0,7 porciento de los tumores malignos en general, con una tasa de 3 por cada 100 000. El histiocitoma fibroso maligno resulta un tumor de alto grado incluido en el grupo de los sarcomas de partes blandas. Objetivo: exponer las características citohistológicas, el tratamiento y la evolución de este tumor en un caso estudiado y tratado en el Hospital General Provincial Docente Roberto Rodríguez Fernández de Ciego de Ávila. Descripción: se presentó el caso de un varón de 60 años con una gran masa de partes blandas en el muslo izquierdo, de crecimiento progresivo. La masa se trató quirúrgicamente con exéresis en bloque, previos estudios analíticos, arteriografía femoral y ecografía. Conclusiones: el diagnóstico anatomopatológico fue de histiocitoma fibroso maligno variedad pleomórfica; fue remitido al servicio de oncología donde lo trataron con radioterapia. A los 6 meses se encontraba libre de la enfermedad. Se realizó una revisión de la literatura y se analizó su presentación clínica, también los hallazgos histológicos, las pruebas diagnósticas de imagen y el procedimiento terapéutico

Introduction: the sarcomas are soft tissue malignant tumors accounting for the 0.7 percent of malignant tumors in general with a rate of 3 by 100 000. The malignant fibrous histiocytoma is a high grade tumor included in the group of soft tissue tumors. Objective: to expose the cytohistologic features, treatment and course of this tumor in study case and treated in Roberto Rodríguez Fernández Teaching Provincial Hospital of Ciego de Avila municipality. Description: this is the case of a male patient aged 60 presenting with an increasing large soft tissue mass in the left thigh. The mass was operated on with block exeresis, analytical previous studies, femoral arteriography and echography. Conclusions: the anatomic and pathologic diagnosis was a malignant fibrous histiocytoma pleomorphous type; the patient was referred to Oncology service where was treated with radiotherapy. At 6 months he was free of disease. A review of literature was performed analyzing its clinical presentation also, its histological findings, the imaging diagnostic test and the therapeutical procedure

[Multimodal treatment for MFH originating from the psoas muscle-a case report].

A 51-year-old male presented with lower abdominal mass and thigh and lumbar pain. CT scan showed a large retroperitoneal tumor compressing the lumbar vertebrae and IVC. Arterial infusion of mitomycin C, epirubicin and DSM was performed for unresectable tumor. The tumor was shrunken and the infusion repeated in 6 and 10 weeks later until right femoral palsy occurred. Radical resection with lumbar fascicles and psoas muscle and IORT (25 Gy) was performed. Local recurrence appeared before the sacrum in the next year. Arterial infusion and resection was done. Local recurrence invading the right common iliac artery was found five years later. The tumor and the artery were resected, followed by extra-anatomical ilio-iliac bypass. Local recurrences occurred in the 6th, 7th and 9th year. Radiation therapy was effective. But for the last recurrence, no radiation was applicable because of over dosage. As the most important thing in the treatment of MFH is to resect the tumor with curative margin at the initial operation, and the retroperitoneum and pelvic cavity are not good places to obtain good margins, multimodal treatment including irradiation should have been considered even preoperatively. Also consultation with orthopedic oncologists, radiologists, vascular surgeons and team practice are essential to get curative margins.

[Helical tomotherapy for axial and paraspinal tumours: experience of Institut Bergonié (14 cases)]. / Expérience de l'institut Bergonié à propos de 14 cas de tomothérapie hélicoïdale de tumeurs axiales et para-axiales.

PURPOSE: To evaluate the feasibility of helical tomotherapy intensity-modulated radiotherapy for the treatment of axial and paraspinal tumours. PATIENTS AND METHODS: Fourteen consecutive patients with axial and paraspinal tumours at the Bergonié Institute between June 2007 and June 2009 were analysed. Various localisations were observed: four paravertebral tumours, five primitive vertebral tumours and six iliosacral or sacral tumours. RESULTS: The dose prescribed to the median of the planning target volume (PTV) varied from 45 to 68Gy depending on histology. The maximum dose delivered to the PTV (Dnear max or D2%) was 60.6Gy (range 46.3-69.1). In the five cases where the tumour was located close to the spinal cord, the median value of Dnear max was 40.1Gy (range 37.8-45.4). In the seven where the tumour was situated close to the cauda equina, the median value of Dnear max was 46.5Gy (range 39.2-60). For two cases in whom the sciatic nerve was the nervous structure at risk, the Dnear max was 64.4 and 67Gy. Six patients were in complete remission, six patients suffered a relapse at a median follow-up of 14.5 months (4-24), two died following local tumour progression and metastasis, tumoral stabilisation had been obtained in two patients. Treatment was well tolerated and was never interrupted in all patients. CONCLUSIONS: Results from this preliminary series are encouraging, particularly concerning the good tolerance of this new intensity-modulated radiotherapy presently more available than proton therapy.

Adjuvant radiotherapy for pediatric and young adult nonrhabdomyosarcoma soft-tissue sarcoma.

PURPOSE: To evaluate the prognostic factors, outcomes, and complications in patients aged ≤30 years with resectable nonrhabdomyosarcoma soft-tissue sarcoma treated at the University of Florida with radiotherapy (RT) during a 34-year period. METHODS AND MATERIALS: A total of 95 pediatric or young adult patients with nonrhabdomyosarcoma soft-tissue sarcoma were treated with curative intent with surgery and RT at the University of Florida between 1973 and 2007. The most common histologic tumor subtypes were synovial sarcoma in 22 patients, malignant fibrous histiocytoma in 19, and malignant peripheral nerve sheath tumor in 11 patients. The mean age at RT was 22 years (range, 6-30). Of the 95 patients, 73 had high-grade tumors; 45 had undergone preoperative RT and 50 postoperative RT. The prognostic factors for survival, local recurrence, and distant recurrence were analyzed. RESULTS: The median follow-up was 7.2 years (range, 0.4-30.5). The actuarial 5-year local control rate was 88%. A microscopically negative margin was associated with superior local control. Although 83% of local recurrence cases initially developed in the absence of metastases, all patients with local failure ultimately died of their disease. The actuarial estimate of 5-year overall survival and disease-free survival was 65% and 63%, respectively. Of all the deaths, 92% were disease related. An early American Joint Committee on Cancer stage, tumor<8 cm, and the absence of neurovascular invasion were associated with superior disease-free survival. The National Cancer Institute Common Toxicity Criteria, version 3, Grade 3-4 treatment complication rate was 9%. No secondary malignancies were observed. CONCLUSION: In the present large single-institution study, we found positive margins and locally advanced features to be poor prognostic factors for both local progression and survival. The results from the present study have helped to characterize the therapeutic ratio of RT in pediatric and young adult sarcoma patients and have provided a basis for identifying high-risk patients for whom treatment intensification might be justified.

Bilateral symmetrical metachronous myxofibrosarcoma: a case report and review of the literature.

Soft tissue sarcomas (STS) are rare. Affected patients are at increased risk of developing a second primary malignancy, particularly another primary STS. These rarely affect the extremities, tending to occur in the abdomen, head or neck. We describe a patient with myxofibrosarcoma of the buttock who developed a second primary tumour of the same histological type in the contralateral buttock thirty months after excision. Remarkably, the second tumour was symmetrical and there were no predisposing factors or evidence of metastatic spread. The second tumour was a significant size at presentation with encasement of the sciatic nerve. Awareness of the sarcoma patient's increased risk of developing a second tumour could lead to earlier diagnosis and improved outcome. A review of the literature on multiple soft tissue sarcomas is also presented.

Radiation dose distribution in the external auditory canal with different prosthetic materials: a clinical report.

Radiotherapy (RT) is an essential component of cancer treatment and aims to deliver higher doses at target volumes and lower doses to surrounding organs to achieve higher tumor control with fewer side effects. Significant dose heterogeneities can occur during treatment of irregular surfaces with electron beams, especially in head, neck, and breast tissue. The external ear and external auditory canal have irregular surfaces, which may cause dose heterogeneity, primarily resulting in excessive doses in the normal tissues lining and adjacent to the ear canal. The purpose of this clinical report is to describe the use of an acrylic resin and an elastomeric material to fill the air cavities of the external auditory canal and compare the dosimetric parameters. The complications that occur after electron-beam therapy of the ear canal can be significantly reduced by filling the ear cavity with acrylic resin, which is a simple, feasible, and cost-effective method.

Management of acral myxoinflammatory fibroblastic sarcoma.

BACKGROUND: Acral myxoinflammatory fibroblastic sarcoma (AMFS) is a rare, low-grade sarcoma that commonly affects the distal extremities. From the published cases, therapy for AMFS to date has been comprised of excision or amputation, with limited use of radiotherapy (RT) or chemotherapy. In this report, the outcome of 17 patients with AMFS treated at the study institution was reported. METHODS: A retrospective review of all cases of AMFS identified in the Sarcoma Database in the Department of Radiation Oncology at the study institution was conducted. Treatment records and data from follow-up visits of patients were reviewed. RESULTS: Seventeen patients were identified. All the patients underwent surgical resection (15 excisions and 2 amputations). Positive surgical margins after excisions were noted in 5 patients and were widely positive in 1 patient. Of the 17 patients, 14 patients received some form of RT. The average total dose was 56.4 Gray (Gy). Eight patients received preoperative RT alone, 5 patients received preoperative RT and postoperative RT, and 1 patient received preoperative RT and intraoperative RT. Median follow-up was 24.5 months. One patient presented with recurrent disease and was treated with resection, and both pre- and postoperative RT. He was free of disease 23 months after his last treatment. No local recurrence was noted in the remaining patients. Of the 14 patients undergoing preoperative RT, complete pathologic necrosis or no tumor was noted in 1 of the patients. No metastatic disease was observed in any of the patients. There was no significant radiation toxicity observed in any of the patients. CONCLUSIONS: Data were consistent with local control of distal extremity sarcomas with resection and RT, suggesting that limb-sparing surgery with this treatment combination is an appropriate option in the limb-sparing control of patients with AMFS, even those with positive surgical margins.

A case of malignant fibrous histiocytoma of the maxillary sinus.

Malignant fibrous histiocytoma (MFH) is recognized as one of the most common soft tissue sarcomas arising in late adulthood. Most MFHs arise from the extremities and the retroperitoneum, thus the incidence of head and neck MFH is relatively low. Because of its rare incidence, there are very few reports focusing on the imaging features of MFH arising in the maxillary sinus. A case of MFH of the maxillary sinus in a 67-year-old male patient is reported including imaging and pathological features.

Histiocitoma fibroso maligno primario del músculo temporal: presentación de un caso / Primary malignant fibrous histiocytoma temporalis muscle: case report

El Histiocitoma fibroso maligno (HFM) fue descrito por Obrien y Stout en 1964 representando al 1-3 por ciento de los sarcomas del tejido blando de cabeza y cuello. Su histogénesis es incierta y el comportamiento biológico es propenso a la recidiva local y a las metástasis a distancia, preferentemente a ganglios linfáticos regionales, pulmón, hígado y huesos. Se presenta una paciente de 38 años de edad, con una lesión tumoral epicraneal recidivante, de consistencia blanda, fluctuante, no dolorosa, de aproximadamente 5 por 3 cm en región temporal derecha, se le realiza Tomografía computarizada de cráneo e Imagen por resonancia magnética, es intervenida quirúrgicamente resecándose totalmente una tumoración predominantemente quística en intima relación con el músculo temporal y reintervenida quince días después, el Rx de tórax, el ultrasonido abdominal y el survey óseo fueron negativos, el estudio histopatológico arrojó un Histiocitoma fibroso maligno pleomorfico por lo que es enviada al servicio de oncología para tratamiento adyuvante (radioterapia). Actualmente pasado un año se encuentra libre de enfermedad. Concluimos que el HFM primario del músculo temporal es una rara localización entre los sarcomas de partes blandas y aun más entre los de cabeza y cuello, donde el aumento de volumen no doloroso fue la forma clínica de presentación, la tomografía computarizada y principalmente las imágenes por resonancia magnética mostraron la relación músculo tumor, lográndose con el empleo de la cirugía y la radioterapia adyuvante alcanzar buenos resultados.

Accumulation of MRI contrast agents in malignant fibrous histiocytoma for gadolinium neutron capture therapy.

Neutron-capture therapy with gadolinium (Gd-NCT) has therapeutic potential, especially that gadolinium is generally used as a contrast medium in magnetic resonance imaging (MRI). The accumulation of gadolinium in a human sarcoma cell line, malignant fibrosis histiocytoma (MFH) Nara-H, was visualized by the MRI system. The commercially available MRI contrast medium Gd-DTPA (Magnevist, dimeglumine gadopentetate aqueous solution) and the biodegradable and highly gadopentetic acid (Gd-DTPA)-loaded chitosan nanoparticles (Gd-nanoCPs) were prepared as MRI contrast agents. The MFH cells were cultured and collected into three falcon tubes that were set into the 3-tesra MRI system to acquire signal intensities from each pellet by the spin echo method, and the longitudinal relaxation time (T1) was calculated. The amount of Gd in the sample was measured by inductively coupled plasma atomic emission spectrography (ICP-AES). The accumulation of gadolinium in cells treated with Gd-nanoCPs was larger than that in cells treated with Gd-DTPA. In contrast, and compared with the control, Gd-DTPA was more effective than Gd-nanoCPs in reducing T1, suggesting that the larger accumulation exerted the adverse effect of lowering the enhancement of MRI. Further studies are warranted to gain insight into the therapeutic potential of Gd-NCT.