Safia K. Ahmed

Adult Ewing Sarcoma: Survival and Local Control Outcomes in 102 Patients with Localized Disease

Objectives. To assess the clinical features and local control (LC) outcomes in adult patients with localized Ewing Sarcoma (ES). Methods. The records of 102 ES patients with localized disease ≥18 years of age seen from 1977 to 2007 were reviewed. Factors relevant to prognosis, survival, and LC were analyzed. Results. The 5-year overall survival (OS) and event-free survival (EFS) were 60% and 52%, respectively, for the entire cohort. Treatment era (1977–1992 versus 1993–2007) remained an independent prognostic factor for OS on multivariate analysis, with improved outcomes observed in the 1993–2007 era (P = 0.02). The 5-year OS and EFS for the 1993–2007 era were 73% and 60%, respectively. Ifosfamide and etoposide based chemotherapy and surgery were more routinely used in the 1993–2007 era (P < 0.01). The 5-year local failure rate (LFR) was 14%, with a 5-year LFR of 18% for surgery, 33% for radiation, and 0% for combined surgery and radiation in the 1993–2007 era (P = 0.17). Conclusion. Modern survival outcomes for adults with localized ES are similar to multi-institutional results in children. This improvement over time is associated with treatment intensification with chemotherapy and increased use of surgery. Aggressive LC (combined surgery and radiation) may improve outcomes in poor prognosis patients.

Patient-reported functional and quality of life outcomes in a large cohort of long-term survivors of Ewing sarcoma

Little data exist regarding long‐term functional and quality of life (QOL) outcomes for survivors of Ewing sarcoma (ES). Specifically, there are few reports assessing the impact of patient characteristics and local therapy modalities on patient‐reported outcomes (PRO).

Adult Ewing sarcoma: survival and local control outcomes in 36 patients with metastatic disease.

Objectives:To assess the clinical features and outcomes in adult patients with metastatic Ewing sarcoma (ES). Methods:The records of 36 ES patients with metastatic disease ≥18 years seen from 1977 to 2007 at the Mayo Clinic were studied retrospectively. Factors relevant to prognosis, survival, and local control (LC) were analyzed. Results:The 4-year overall survival (OS) and event-free survival (EFS) rates were 20% and 11%, respectively. Patients treated from 1993 to 2007 had significantly improved outcomes compared with those treated from 1977 to 1992: 4-year OS and EFS of 31% and 16%, respectively, versus 0% (P=0.01). Primary tumor (P=0.005 and 0.04) and metastatic sites (P=0.05) were independent EFS prognostic factors. Four patients (11%) received surgery, 18 (50%) radiation therapy (RT), 4 (11%) surgery+radiation therapy (S+RT), and 10 (28%) received no LC. The 4-year EFS rates were 0% for surgery, 21% for RT, 0% for S+RT, and 0% for no LC (P=0.0001). OS in patients who received vincristine, doxorubicin, and cyclophosphamide alternating with ifosphamide and etoposide (VDC/IE) chemotherapy was improved (P=0.04). Relapses were documented in 18 patients (excluding patients who received no LC). In total, 44% of patients had all of their metastatic site(s) treated. Patients who received treatment to all extrapulmonary metastases had significantly improved outcomes compared with those who did not receive treatment to all sites: 4-year OS and EFS of 33% and 11%, respectively, versus 0% (P=0.04 and 0.02). Conclusions:Our results suggest outcomes for adult patients with metastatic ES are similar to pediatric cohorts in the modern era. VDC/IE chemotherapy and treatment to all metastatic lesions is associated with improved outcomes.

Adaptation of the Stanford technique for treatment of bulky cutaneous T-cell lymphoma of the head

Electron beam radiation therapy is an effective treatment for cutaneous T-cell lymphoma (CTCL).1,2 The first description of total skin electron therapy came from Stanford University.1,3 Prolonged treatment to ≥3000 cGy in 6 to 7 weeks is not feasible for many patients in a palliative setting. Hypofractionated regimens are associated with high response rates.4-8 We describe a case of bulky CTCL of the head treated with a unique adaptation of the Stanford technique.

Pelvis Ewing sarcoma: Local control and survival in the modern era

Local control for Ewing sarcoma (ES) has improved in modern studies. However, it is unclear if these gains have also been achieved for pelvis tumors. The purpose of this study is to evaluate local control and survival in pelvis ES patients treated in the modern era.

Patterns of failure and optimal radiotherapy target volumes in primary intradural extramedullary Ewing sarcoma

Soft tissue Ewing sarcoma (ES) is a member of the Ewing sarcoma family of tumors that includes osseous Ewing sarcoma and peripheral primitive neuroectodermal tumors (pPNET). When involving the spine, these tumors arise more often in the paravertebral or extradural regions with limited reports of tumors originating from the intradural extramedullary space. Other reported tumors found within the dura, but not involving the cord include central primitive neuroectodermal tumors (cPNET), and more commonly schwannoma, meningioma, neurofibroma, paraganglioma, or ependymoma tumors [1]. Pathologically the distinction between cPNET and pPNET (or ES) can be difficult because they appear similar morphologically as small round blue cell tumors. ES is distinct from cPNET by immunohistochemistry and cytogenetics. ES shares common findings of immunoreactivity for the cell surface glycoprotein CD99 (MIC2 gene product), which is not expressed in cPNET, but found in other primary central nervous system (CNS) tumors including ependymomas, meningeal hemangiopericytomas and leukemic infiltrates. ES tumors also have common translocations involving chromosome 22, most notably t(11;22)(q24;q12). This mutation has not been identified in cPNET [2]. ES is treated with surgery and/or focal radiotherapy in addition to systemic chemotherapy. Previous reports of primary intradural extramedullary ES also suggest standard Ewing sarcoma regimens including focal radiotherapy may be effective in this disease location. In contrast, since as early as 1952 [3], the treatment of cPNET of the spine has included craniospinal radiotherapy due to the 20–32% risk of patients who have occult CNS dissemination at diagnosis [4]. This pattern of failure has not previously been associated with ES, which is traditionally locally invasive and disseminates hematogenously to the lungs and bone marrow. We previously reported two patients with intradural ES treated with surgery, focal radiation and systemic chemotherapy [5]. In this study we present an update on our previously reported patients, two additional patients from our institution, and a review of the previously reported cases from the literature. The purpose of this report is to describe patterns of failure to determine the optimal radiotherapy volume for primary intradural ES.

Pediatric Metastatic Odontogenic Ghost Cell Carcinoma: A Multimodal Treatment Approach.

Odontogenic ghost cell carcinoma (OGCC) is a rare and aggressive tumor wherein optimal treatment remains uncertain. We report the first pediatric metastatic OGCC case treated with multimodal therapy: surgery, adjuvant chemoradiation, and adjuvant immunotherapy. Adjuvant therapy was utilized due to locally advanced disease with pathologic features indicative of high recurrence risk. This multimodal approach was modeled after management of primary head and neck cancer, where adjuvant chemoradiation and immunotherapy are associated with improved outcomes. Our patient is alive and disease free at 14 months indicating a potentially positive role for multimodal therapy in the management of OGCC.

Protons vs Photons for Brain and Skull Base Tumors

The physical characteristics of proton therapy result in steeper dose gradients and superior dose conformality compared to photon therapy. These properties render proton therapy ideal for skull base tumors requiring dose escalation for optimal tumor control, and may also be beneficial for brain tumors as a means of mitigating radiation-related adverse effects. This review summarizes the literature regarding the role of proton therapy compared to photon therapy in the treatment of adult brain and skull base tumors.

Ewing sarcoma and desmoplastic small round cell tumor

Ewing sarcoma is the second most common primary bone tumor. Successful treatment requires a multidisciplinary approach with chemotherapy and local therapy. In this chapter we review the background, presentation, work-up, treatment, and long-term outcomes of Ewing sarcoma. We also review the background and treatment of desmoplastic small round cell tumor.

Clinical efficacy and safety of a highly conformal, supine, hybrid forward and inverse planned intensity modulated radiation therapy technique for craniospinal irradiation

Abstract Purpose: To demonstrate the clinical efficacy and safety of a highly conformal, supine, hybrid forward and inverse planned intensity modulated radiation therapy (IMRT) technique for photon craniospinal irradiation (CSI). Methods: Patients who received supine, hybrid IMRT CSI from 2009 to 2014 were included in this retrospective review. Clinical target volume (CTV) was defined as intracranial contents and thecal sac, including nerve roots. Dose was prescribed such that >99% of CTV received >99% of prescription and >95% of the planning target volume received >95% of prescription, with no attempt to include vertebral bodies. Lateral fields were utilized at the cranium and upper cervical spine. Spine fields were either single posterior or 2–3 obliques. Plans were generated with a hybrid of forward and inverse planned IMRT. Inferior borders of the cranium fields and superior border of the lower spine field were designed with 6–15 cm long, gradual dose gradients by sequential closing of multileaf collimator leaves using forward planned multiple static segment IMRT delivery. The sliding window upper spine IMRT field was created by the inverse planning system to match gradients of the brain and lower spine fields. The lower spine field gradient was similarly completed. Results: The cohort consisted of 34 patients. Median CSI dose was 36 Gy (range: 18–39.6 Gy). With a median follow up of 59.4 months, there were no isolated recurrences or spinal myelopathies at CTV margins or field gradients. Eleven patients had recurrence, all of which were intracranial. Conclusions: Our hybrid forward and inverse planned IMRT supine CSI technique did not result in any isolated recurrences or myelopathies at CTV margins or field gradients. This suggests our target volumes and blended gradients are appropriate for highly conformal three-dimensional planning.