scholarly journals The radiation therapy options of intracranial hemangiopericytoma: An overview and update on a rare vascular mesenchymal tumor

2018 ◽  
Author(s):  
Maria Paola Ciliberti ◽  
Rosa D'Agostino ◽  
Laura Gabrieli ◽  
Anna Nikolaou ◽  
Angela Sardaro
Keyword(s):  
Surgical Resection ◽  
Adjuvant Radiotherapy ◽  
Therapeutic Option ◽  
Treatment Options ◽  
Distant Metastases ◽  
Complete Resection ◽  
Complete Surgical Resection ◽  
Hypervascular Tumor ◽  
The Central Nervous System ◽  
Radiotherapy Treatment

Hemangiopericytoma (HPC) is an extremely rare hypervascular tumor of mesenchymal lineage. It tends to recur and to develop distant metastases even many years after primary surgical resection. The management of recurrent and metastatic disease is not always so well defined. A complete surgical resection does not eliminate the high risk of local recurrences that occur in the central nervous system, often in the same surgical bed. However, treatment with adjuvant radiotherapy even in cases of complete resection remains controversial. Because of its rarity, there is no standard for treatment. We focused on radiotherapy treatment options, analyzing the literature and making a base on conduct further studies to establish the standard radiation dose to be used for locoregional control of such a complex and extremely rare disease and to help specialists to take the most appropriate therapeutic option.

A randomized, double-blinded, placebo-controlled, multi-institutional, cross-over, phase II.5 study of saracatinib (AZD0530), a selective Src kinase inhibitor, in patients with recurrent osteosarcoma localized to the lung.

2013 ◽  
Vol 31 (15_suppl) ◽  
pp. TPS10591-TPS10591 ◽  
Author(s):  
Kristin Baird ◽  
Denise K. Reinke ◽  
Joseph Gerald Pressey ◽  
Leo Mascarenhas ◽  
Noah Federman ◽  
...  
Keyword(s):  
Surgical Resection ◽  
Phase Ii ◽  
Kinase Inhibitor ◽  
Treatment Guidelines ◽  
Src Kinase ◽  
Progression Free Survival ◽  
Primary Treatment ◽  
Complete Resection ◽  
Controlled Study ◽  
Complete Surgical Resection

TPS10591 Background: Osteosarcoma is a rare cancer and 33% of patients who have completed primary treatment will recur. The Src pathway has been implicated in the metastatic behavior of several tumors including osteosarcoma where 95% of samples express Src or have evidence of downstream activation of this pathway. Saracatinib (AZD0530) is a potent and selective Src kinase inhibitor. The recommended phase II dose in adults was found to be 175mg daily. The primary goal of this study is to determine if treatment with Saracatinib can increase progression free survival (PFS) for patients who have undergone complete resection of metastatic osteosarcoma nodules in the lung. Secondary goals are evaluation of overall survival, time to treatment failure, and evaluation of several biological correlatives. Methods: This is a multi-institutional, phase II.5, placebo-controlled study with an accrual goals of 88 randomized patients. Patients between 15 and 75 years, with histological confirmation of recurrent osteosarcoma, localized to the lung, who have potential for complete surgical resection, are eligible for enrollment. After complete resection, patients are randomized to treatment with saracatinib or placebo, of a daily oral dose of 175 mg, continuously for up to 1 year or until progression. Patients who recur in the lung while on-study and who are amenable to complete surgical resection will be un-blinded. Those patients who received placebo may have the option to undergo surgical resection. If fully resected, they will be offered therapy with saracatinib under the same treatment guidelines as above. As of January 2013, 38 patients have enrolled and 32 patients met the criteria to be randomized and began oral therapy with either saracatinib or placebo. An interim analysis is planned after 40 patients have been randomized. Clinical trial information: NCT00752206.

scholarly journals Short-term comparative results of segmental resection versus radical resection in two patients with myxofibrosarcoma of the limbs - case series

2020 ◽  
Vol 3 (1) ◽  
pp. 9-14
Author(s):  
Sergiu-Andrei Iordache ◽  
Bogdan Şerban ◽  
Andreea Vlad ◽  
Adrian Cursaru
Keyword(s):  
Adjuvant Radiotherapy ◽  
Therapeutic Option ◽  
Case Series ◽  
Distant Metastases ◽  
Radical Resection ◽  
Segmental Resection ◽  
Lower Grade ◽  
Short Term ◽  
Comparative Results ◽  
Challenging Situation

AbstractIntroduction: Myxofibrosarcoma is a rare subtype of soft tissue sarcoma with a locally infiltrative behavior and ability to determine distant metastases.Materials and methods: We presented two myxofibrosarcoma cases who benefited from segmental or radical resection.Management and outcome: In the case of the 80-year-old woman, with grade 3 myxofibrosarcoma, we practiced radical surgery with scapulohumeral disarticulation followed by adjuvant radiotherapy.The therapeutic option for the 77-year-old man with grade 2 myxofibrosarcoma was segmental resection followed by adjuvant radiotherapy. After three months, the patient was in a good clinical condition with no sign of local recurrence, but with the presence of pulmonary metastases for the patient who benefited of segmental resection.Discussion: The radical resection had better short-term results, with no local or distant metastases at three months after surgery, although the tumor had a higher grade (G3), compared to segmental resection practiced for a lower grade tumor (G2), in which case the patient developed pulmonary metastasis at three months follow up.Conclusion: Myxofibrosarcoma represents a challenging situation regarding the management due to its unpredictive clinical course. Our cases raised the following question: should we consider treating it more aggressively in order to obtain good local control and reduce the risk of metastasis?

scholarly journals Primary Epithelioid Sarcoma Manifesting as a Fungating Scalp Mass - Imaging Features and Treatment Options. A Case Report and Literature Review

2021 ◽  
Vol 15 (11) ◽  
pp. 1-9
Author(s):  
Yonghao Zhang ◽  
Tarun Mohan Mirpuri ◽  
Chi Long Ho
Keyword(s):  
Case Report ◽  
Adjuvant Radiotherapy ◽  
Multimodality Imaging ◽  
Treatment Options ◽  
Epithelioid Sarcoma ◽  
Distant Metastases ◽  
Imaging Features ◽  
Rare Malignancy ◽  
Scalp Mass

Primary epithelioid sarcoma is an extremely rare malignancy of the scalp. To date, less than a dozen such cases have been reported in the literature. The diagnosis often is a challenge to both radiologists and clinicians. This is largely attributed to the lack of literature on the imaging features of scalp epithelioid sarcoma. In this report, we highlight the role of multimodality imaging in the diagnosis of primary scalp epithelioid sarcoma and review the epidemiology, imaging, treatment options, and prognosis of these malignant scalp tumors. Displaying a multilobulated morphology, heterogeneous enhancement, and restricted diffusion on MRI, these tumors typically show central degeneration with hemorrhage, necrosis, and calcification. Wide surgical resection and adjuvant radiotherapy are the mainstays of treatment for localized scalp tumors. Nevertheless, the prognosis of patients with distant metastases at diagnosis is extremely poor compared to those with local or regional diseases.

scholarly journals Characteristics associated with complete surgical resection of primary malignant mediastinal tumors

2009 ◽  
Vol 35 (9) ◽  
pp. 832-838 ◽  
Author(s):  
Jefferson Luiz Gross ◽  
Ulisses Augusto Correia Rosalino ◽  
Riad Naim Younes ◽  
Fábio José Haddad ◽  
Rodrigo Afonso da Silva ◽  
...  
Keyword(s):  
Surgical Resection ◽  
Superior Vena ◽  
Vena Cava ◽  
Complete Resection ◽  
Incomplete Resection ◽  
Imaging Studies ◽  
Curative Intent ◽  
Mediastinal Tumors ◽  
Complete Surgical Resection ◽  
Single Facility

OBJECTIVE: To identify preoperative characteristics associated with complete surgical resection of primary malignant mediastinal tumors. METHODS: Between 1996 and 2006, 42 patients with primary malignant mediastinal tumors were submitted to surgery with curative intent at a single facility. Patient charts were reviewed in order to collect data related to demographics, clinical manifestation, characteristics of mediastinal tumors and imaging aspects of invasiveness. RESULTS: The surgical resection was considered complete in 69.1% of the patients. Cases of incomplete resection were attributed to invasion of the following structures: large blood vessels (4 cases); the superior vena cava (3 cases); the heart (2 cases); the lung and chest wall (3cases); and the trachea (1 case). Overall survival was significantly better among the patients submitted to complete surgical resection than among those submitted to incomplete resection. The frequency of incomplete resection was significantly higher in cases in which the tumor had invaded organs other than the lung (as identified through imaging studies) than in those in which it was restricted to the lung (47.6% vs. 14.3%; p = 0.04). None of the other preoperative characteristics analyzed were found to be associated with complete resection. CONCLUSIONS: Preoperative radiological evidence of invasion of organs other than the lung is associated with the incomplete surgical resection of primary malignant mediastinal tumors.

scholarly journals Neurological heterotopic ossification: novel mechanisms, prognostic biomarkers and prophylactic therapies

Bone Research ◽  
2020 ◽  
Vol 8 (1) ◽  
Author(s):  
Ker Rui Wong ◽  
Richelle Mychasiuk ◽  
Terence J. O’Brien ◽  
Sandy R. Shultz ◽  
Stuart J. McDonald ◽  
...  
Keyword(s):  
Heterotopic Ossification ◽  
Surgical Resection ◽  
Treatment Options ◽  
Prognostic Biomarkers ◽  
Future Research ◽  
Cns Injury ◽  
Ectopic Bone ◽  
Targeted Treatments ◽  
The Central Nervous System ◽  
Polytrauma Patients

AbstractNeurological heterotopic ossification (NHO) is a debilitating condition where bone forms in soft tissue, such as muscle surrounding the hip and knee, following an injury to the brain or spinal cord. This abnormal formation of bone can result in nerve impingement, pain, contractures and impaired movement. Patients are often diagnosed with NHO after the bone tissue has completely mineralised, leaving invasive surgical resection the only remaining treatment option. Surgical resection of NHO creates potential for added complications, particularly in patients with concomitant injury to the central nervous system (CNS). Although recent work has begun to shed light on the physiological mechanisms involved in NHO, there remains a significant knowledge gap related to the prognostic biomarkers and prophylactic treatments which are necessary to prevent NHO and optimise patient outcomes. This article reviews the current understanding pertaining to NHO epidemiology, pathobiology, biomarkers and treatment options. In particular, we focus on how concomitant CNS injury may drive ectopic bone formation and discuss considerations for treating polytrauma patients with NHO. We conclude that understanding of the pathogenesis of NHO is rapidly advancing, and as such, there is the strong potential for future research to unearth methods capable of identifying patients likely to develop NHO, and targeted treatments to prevent its manifestation.

scholarly journals Conjunctival Leiomyosarcoma, a Rare Neoplasm Always Originating at the Limbus? Report of a New Case and Review of 11 Published Cases

2019 ◽  
Vol 5 (5) ◽  
pp. 333-339 ◽  
Author(s):  
V. De Groot ◽  
E. Verhelst ◽  
P.C.W. Hogendoorn ◽  
R.J.W. de Keizer
Keyword(s):  
Adjuvant Radiotherapy ◽  
Myogenic Differentiation ◽  
Distant Metastases ◽  
Excisional Biopsy ◽  
Complete Resection ◽  
Favorable Prognosis ◽  
Strontium 90 ◽  
Malignant Mesenchymal Tumor ◽  
Histopathological Investigation

Purpose of the Study: To describe the differential diagnosis and management of a rare conjunctival malignancy. Procedures: A 79-year-old man presented with a conjunctival mass at the limbus. Excisional biopsy revealed a malignant mesenchymal tumor with myogenic differentiation. Six months later, three suspect lesions developed at the border of the previous excision. Pathological diagnosis pointed to a leiomyosarcoma. Adjuvant radiotherapy with strontium-90 brachytherapy was applied. After 3 years, there was neither recurrence nor distant metastases. A literature review revealed 11 cases of conjunctival leiomyosarcoma. Results: All 12 cases seemed to originate at the limbal conjunctiva. Scleral invasion was found only in one patient with multiple previous resections. Corneal invasion was reported in two patients. Four patients had a globe-sparing resection. In two of them, margins were not tumor free and additional brachytherapy gave a tumor-free follow-up of 1 and 3 years. Four cases underwent an exenteration. Conclusion and Message: Primary conjunctival leiomyosarcoma is a rare tumor with a favorable prognosis due to early detection and consequently limited size. Diagnosis involves histopathological investigation including immunohistochemistry. If possible, complete resection has the best prognosis. Adjunctive radiotherapy can be effective when the margins are not free and should be considered.

scholarly journals EPCO-03. REPORT OF NTRK FUSION IN A PEDIATRIC PATIENT WITH SPINAL GANGLIOCYTOMA

2020 ◽  
Vol 22 (Supplement_2) ◽  
pp. ii69-ii69
Author(s):  
Avanti Gupte ◽  
Stephanie Toll ◽  
Neena Marupudi ◽  
Hamza Gorsi
Keyword(s):  
Surgical Resection ◽  
Therapeutic Option ◽  
Pediatric Brain Tumors ◽  
Who Grade ◽  
Dna And Rna ◽  
Complete Surgical Resection ◽  
Adult Head ◽  
Magnetic Resonance Imaging Mri ◽  
Histopathologic Analysis ◽  
Trk Inhibitors

Abstract BACKGROUND Gangliocytomas are rare WHO grade 1 central nervous system tumors of neuronal/ mixed neuronal-glial classification that most commonly occur in the cerebrum, favoring the temporal lobe. They are also reported in cerebellum in the setting of Lhermite-Duclos disease. Spinal gangliocytomas are exceedingly rare, constituting 3–10% of reported pediatric cases. Maximal safe surgical resection is the mainstay of therapy, as response to irradiation or traditional chemotherapy is variable. Given the rarity of these tumors, little is known about the molecular makeup or the role of targeted therapy in this population. CASE Our patient presented as a 7-year-old male with thorocolumbar scoliosis. A magnetic resonance imaging (MRI) of his spine incidentally demonstrated a heterogeneously enhancing, expansile solid and cystic intramedullary thoracic mass from T6 through T11. MRI brain did not show any intracranial mass. He subsequently underwent a laminectomy with laminoplasty for the intramedullary mass. Histopathologic analysis confirmed a WHO grade I gangliocytoma with moderately cellular abnormal central neural tissue composed of irregular clusters and cords of mature-appearing neurons in a coarse neuropil background. Because of the unusual location and presentation of his disease, molecular analysis including DNA and RNA sequencing was performed which revealed a somatic BCR-NTRK2 fusion. DISCUSSION NTRK gene fusions are known oncogenic drivers, reported with varying frequency in many pediatric and adult tumors. They are reported in < 1% of adult head and neck cancers but greater than 90% of congenital infantile fibrosarcomas. NTRK fusions are seen in pediatric brain tumors like infantile high grade gliomas, however they have not been previously reported in gangliocytoma. As TRK inhibitors are efficacious in treating a number of NTRK-rearranged malignancies, this novel finding provides a therapeutic option for NTRK fusion positive gangliocytoma where complete surgical resection is not safely possible.

scholarly journals Metastatic Malignant Gastric Glomus Tumour: Multidisciplinary Diagnosis & Treatment of a Rare Clinical Entity

2021 ◽  
Author(s):  
Jordan W. Francheville ◽  
Thomas Arnason ◽  
Michael D. Carter ◽  
Kian Khodadad ◽  
Carman Giacomantonio ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Targeted Therapy ◽  
Surgical Resection ◽  
Treatment Options ◽  
Genomic Analysis ◽  
Multidisciplinary Care ◽  
Distant Metastases ◽  
Glomus Tumour ◽  
Rare Clinical Entity ◽  
Glomus Tumors

Abstract Glomus tumours are uncommon neoplasms that are usually benign, solitary and most often found in the skin and soft tissue of distal extremities. Primary gastric glomus tumors are rare, but well described. Fewer than 15 cases of gastric glomus tumour have been reported to have malignant behaviour with distant metastases. Although surgical resection is effective if feasible, recurrence can occur and there is a paucity of evidence on medical treatment options. Here we present the case of a 69-year-old male with a gastric glomus tumour with metastases to abdominal viscera requiring multidisciplinary care for diagnosis, surgical resection, and multiple lines of systemic/radiation therapy guided by available evidence. Genomic analysis revealed a NOTCH2 rearrangement, described in only two prior works and highlighting future possibilities for targeted therapy.

scholarly journals Sclerotherapy for Oral Pyogenic Granuloma – A case report

2017 ◽  
Vol 13 (2) ◽  
pp. 290-292 ◽  
Author(s):  
Pragya Regmee ◽  
Jyotsna Rimal ◽  
Iccha Kumar Maharjan ◽  
Sushma Pandey ◽  
Deepa Niroula ◽  
...  
Keyword(s):  
Case Report ◽  
Surgical Resection ◽  
Treatment Options ◽  
Pyogenic Granuloma ◽  
Vascular Tumor ◽  
Intralesional Injection ◽  
Sodium Tetradecyl Sulfate ◽  
Inflammatory Mass ◽  
Complete Surgical Resection ◽  
One Year

First described by Hullihen in 1844, Pyogenic granuloma (PG) is a benign, exophytic vascular tumor. It is not infective, purulent or granulomatous but a reactive inflammatory mass of blood vessels with few fibroblasts within the mucosa or skin. Without any general agreement, there exist a variety of treatment options (complete surgical resection, cryosurgery, curettage, lasers etc). However, recurrence has been frequently reported.  Sclerotherapy in PG (a vascular tumor) is not as commonly reported as in cases of hemangioma.Herein, we present a case of twenty-eight years old female with a clinical diagnosis of PG, treated with intralesional sodium tetradecyl sulfate (STS). Intralesional injection of 0.2 ml of undiluted STS was given for three visits between 10th of January and 21st of February 2016. The lesion healed completely at the patient’s fourth visit and recurrence was not reported till the patient was followed up for one year, i.e February 2017. 

scholarly journals The Value of Surgery for Retroperitoneal Sarcoma

Sarcoma ◽  
2009 ◽  
Vol 2009 ◽  
pp. 1-6 ◽  
Author(s):  
Sepideh Gholami ◽  
Charlotte D. Jacobs ◽  
Daniel S. Kapp ◽  
Layla M. Parast ◽  
Jeffrey A. Norton
Keyword(s):  
Overall Survival ◽  
Surgical Resection ◽  
Malignant Tumors ◽  
Tumor Grade ◽  
Complete Resection ◽  
Retroperitoneal Sarcoma ◽  
High Grade ◽  
Complete Surgical Resection ◽  
Retroperitoneal Sarcomas ◽  
Stage 1

Introduction. Retroperitoneal sarcomas are uncommon large malignant tumors.Methods. Forty-one consecutive patients with localized retroperitoneal sarcoma were retrospectively studied.Results. Median age was 58 years (range 20–91 years). Median tumor size was 17.5 cm (range 4–41 cm). Only 2 tumors were <5 cm. Most were liposarcoma (44%) and high-grade (59%). 59% were stage 3 and the rest was stage 1. Median followup was 10 months (range 1–106 months). Thirty-eight patients had an initial complete resection; 15 (37%) developed recurrent sarcoma and 12 (80%) had a second complete resection. Patients with an initial complete resection had a 5-year survival of 46%. For all patients, tumor grade affected overall survival (). Complete surgical resection improved overall survival for high-grade tumors ().Conclusions. Tumor grade/stage and complete surgical resection for high-grade tumors are important prognostic variables. Radiation therapy or chemotherapy had no significant impact on overall or recurrence-free survival. Complete surgical resection is the treatment of choice for patients with initial and locally recurrent retroperitoneal sarcoma.

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