scholarly journals A case report of atypical malignant melanotic neuroectodermal tumor of infancy

2021 ◽  
Vol 20 (4) ◽  
pp. 168-177
Author(s):  
A. V. Lopatin ◽  
A. Yu. Kugushev ◽  
N. S. Grachev ◽  
S. A. Yasonov ◽  
N. I. Ponomareva ◽  
...  

Melanotic neuroectodermal tumor of infancy is a rare neoplasm that predominantly involves cranial bones and tends to occur during the first year of life. About 500 cases have been described in the literature to date; 6% of them have been reported to be malignant. Treatment for these malignant tumors was not documented and often turned out to be ineffective.Here we report a case of a child aged 2 years and 4 months who presented with a rapidly growing mass in the maxillary region spreading through the orbit into the anterior cranial fossa. The patient’s parents gave consent to the use of their child’s data, including photographs, for research purposes and in publications. He was treated at the Russian Children’s Clinical Hospital from July 2018 to November 2019. The child underwent chemoradiation and staged surgical removal of the tumor. Treatment with ICE and radiation therapy led to a significant reduction of the tumor volume and enabled us to perform cytoreductive surgery with the removal of the mass in the maxilla. Further treatment according to the CWS 2009 guidance for high-risk patients with NRSTS (NonRhabdomyosarcoma Soft Tissue Sarcoma) and radiation therapy resulted in further regression of intraorbital and intracranial components of the tumor and we performed a radical resection of the residual tumor conglomerate. Investigations during the course of treatment revealed no signs of metastatic involvement. The behavior of malignant melanotic neuroectodermal tumors of infancy is unpredictable, that is why in case of the massive involvement of the facial bones when surgery is associated with a high risk of functional impairment or cosmetic deformity, one should consider preoperative chemotherapy to reduce tumor size and intraoperative blood loss. Moreover, chemotherapy in combination with resection makes it possible to minimize the risk of local relapse or metastasis. 

FACE ◽  
2021 ◽  
pp. 273250162110138
Author(s):  
Rebecca Knackstedt ◽  
Peter Taub ◽  
Gary Rogers ◽  
Brian Gastman

The mainstay of curative therapy for head and neck skin cancers relies upon surgery and/or radiation therapy. However, for some aggressive, non-resectable or recurrent tumors, systemic therapy is necessary. Recent emerging classes of drugs have shown to improve survival for high-risk, recurrent, and unresectable variants of these tumors. The goal of this paper is to review options for systemic therapies for head and neck skin cancers including melanoma, non-melanoma skin cancers and other rare and non-malignant tumors.


Author(s):  
Ernest Osei ◽  
Hafsa Mansoor ◽  
Johnson Darko ◽  
Beverley Osei ◽  
Katrina Fleming ◽  
...  

Abstract Background: The standard treatment modalities for prostate cancer include surgery, chemotherapy, hormonal therapy and radiation therapy or any combination depending on the stage of the tumour. Radiation therapy is a common and effective treatment modality for low-intermediate-risk patients with localised prostate cancer, to treat the intact prostate and seminal vesicles or prostate bed post prostatectomy. However, for high-risk patients with lymph node involvement, treatment with radiation will usually include treatment of the whole pelvis to cover the prostate and seminal vesicles or prostate bed and the pelvic lymph nodes followed by a boost delivery dose to the prostate and seminal vesicles or prostate bed. Materials and Methods: We retrospectively analysed the treatment plans for 179 prostate cancer patients treated at the cancer centre with the volumetric-modulated arc therapy (VMAT) technique via RapidArc using 6 MV photon beam. Patients were either treated with a total prescription dose of 78 Gy in 39 fractions for patients with intact prostate or 66 Gy in 33 fractions for post prostatectomy patients. Results: There were 114 (64%) patients treated with 78 Gy/39 and 65 (36%) treated with 66 Gy/34. The mean homogeneity index (HI), conformity index (CI) and uniformity index (UI) for the PTV-primary of patients treated with 78 Gy are 0.06 ± 0.01, 1.04 ± 0.01 and 0.99 ± 0.01, respectively, and the corresponding mean values for patients treated with 66 Gy are 0.06 ± 0.02, 1.05 ± 0.01 and 0.99 ± 0.01, respectively. The mean PTV-primary V95%, V100% and V105% are 99.5 ± 0.5%, 78.8 ± 12.2% and 0.1 ± 0.5%, respectively, for patients treated with 78 Gy and 99.3 ± 0.9%, 78.1 ± 10.6% and 0.1 ± 0.4%, respectively, for patients treated with 66 Gy. The rectal V50Gy, V65Gy, V66.6Gy, V70Gy, V75Gy and V80Gy are 26.8 ± 9.1%, 14.2 ± 5.3%, 13.1 ± 5.0%, 10.8 ± 4.3%, 6.9 ± 3.1% and 0.1 ± 0.1%, respectively, for patients treated with 78 Gy and 33.7 ± 8.4%, 14.1 ± 4.5%, 6.7 ± 4.5%, 0.0 ± 0.2%, 0.0% and 0.0%, respectively, for patients treated with 66 Gy. Conclusion: The use of VMAT technique for radiation therapy of high-risk prostate cancer patients is an efficient and reliable method for achieving superior dose conformity, uniformity and homogeneity to the PTV and minimal doses to the organs at risk. Results from this study provide the basis for the development and implementation of consistent treatment criteria in radiotherapy programs, have the potential to establish an evaluation process to define a consistent, standardised and transparent treatment path for all patients that reduces significant variations in the acceptability of treatment plans and potentially improve patient standard of care.


2018 ◽  
Vol 2 (18) ◽  
pp. 2369-2377 ◽  
Author(s):  
Tao Wu ◽  
Yong Yang ◽  
Su-Yu Zhu ◽  
Mei Shi ◽  
Hang Su ◽  
...  

Abstract This study evaluated the survival benefit of intensity-modulated radiation therapy (IMRT) compared with 3-dimension conformal radiation therapy (3D-CRT) in a large national cohort of patients with early-stage extranodal nasal-type natural killer/T-cell lymphoma (NKTCL). This retrospective study reviewed patients with early-stage NKTCL treated with high-dose radiation therapy (RT; ≥45 Gy) at 16 Chinese institutions. Patients were stratified into 1 of 4 risk groups based on the number of risk factors: low risk (no factors), intermediate-low risk (1 factor), intermediate-high risk (2 factors), and high-risk (3-5 factors). Of the 1691 patients, 981 (58%) received IMRT, and 710 (42%) received 3D-CRT. Unadjusted 5-year overall survival (OS) and progression-free survival (PFS) were 75.9% and 67.6%, respectively, for IMRT compared with 68.9% (P = .004) and 58.2% (P < .001), respectively, for 3D-CRT. After propensity score match and multivariable analyses to account for confounding factors, IMRT remained significantly associated with improved OS and PFS. The OS and PFS benefits of IMRT persisted in patients treated with modern chemotherapy regimens. Compared with 3D-CRT, IMRT significantly improved OS and PFS for high-risk and intermediate-high–risk patients but provided limited benefits for low-risk or intermediate-low–risk patients. A risk-adapted survival benefit profile of IMRT can be used to select patients and make treatment decisions.


2017 ◽  
Vol 35 (8_suppl) ◽  
pp. 203-203 ◽  
Author(s):  
Chelsea Hertel ◽  
Amir Harandi ◽  
Cliff P. Connery ◽  
Dimitrios Papadopoulos ◽  

203 Background: Malnutrition is very common in patients receiving radiation therapy. This can result in significant weight loss, decreased functioning, depression, increased mortality, and dramatic declines in quality of life during and after treatment. Targeting patients at risk with nutritional counseling and progressive intervention can have important clinical implications. Methods: A total of 106 patients at a hospital-based cancer center getting radiation for a wide spectrum of cancers (breast, lung, gastrointestinal, genitourinary, and other types) were evaluated for individualized nutritional counseling and education. Patients with identified risk factors were deemed to be at high risk by nursing staff if meeting pre-specified criteria for weight loss ( > 2.5%), body mass index < 18.5%, and/or gastrointestinal symptoms (poor appetite, diarrhea, or constipation affecting quality of life). After high risk patients were identified by a nursing staff triage questionnaire, an automatic computer generated referral was made to the nutritionist. Results: Prior to the institution of this protocol, 13.7% of patients getting radiation therapy were noted to be at high risk and not receiving any nutritional intervention during their course of radiotherapy. However, after the initiation of adequate screening by nursing staff triggering a nutrition referral, the percentage of high risk patients without an associated nutrition consult declined to 1.1%. Conclusions: This study conveys important information for having a systemic screening process in place to identify those at risk for progressive malnutrition while getting radiotherapy for a broad spectrum of tumor types.


2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Sung Wook Seo ◽  
Jisoo Kim ◽  
Jihye Son ◽  
Sungbin Lim

Abstract Background The impact of adjuvant chemotherapy or radiation therapy on the survival of patients with synovial sarcoma (SS), which is a rare soft-tissue sarcoma, remains controversial. Bayesian statistical approaches and propensity score matching can be employed to infer treatment effects using observational data. Thus, this study aimed to identify the individual treatment effects of adjuvant therapies on the overall survival of SS patients and recognize subgroups of patients who can benefit from specific treatments using Bayesian subgroup analyses. Methods We analyzed data from patients with SS obtained from the surveillance, epidemiology, and end results (SEER) public database. These data were collected between 1984 and 2014. The treatment effects of chemotherapy and radiation therapy on overall survival were evaluated using propensity score matching. Subgroups that could benefit from radiation therapy or chemotherapy were identified using Bayesian subgroup analyses. Results Based on a stratified Kaplan–Meier curve, chemotherapy exhibited a positive average causal effect on survival in patients with SS, whereas radiation therapy did not. The optimal subgroup for chemotherapy includes the following covariates: older than 20 years, male, large tumor (longest diameter > 5 cm), advanced stage (SEER 3), extremity location, and spindle cell type. The optimal subgroup for radiation therapy includes the following covariates: older than 20 years, male, large tumor (longest diameter > 5 cm), early stage (SEER 1), extremity location, and biphasic type. Conclusion In this study, we identified high-risk patients whose variables include age (age > 20 years), gender, tumor size, tumor location, and poor prognosis without adjuvant treatment. Radiation therapy should be considered in the early stages for high-risk patients with biphasic types. Conversely, chemotherapy should be considered for late-stage high-risk SS patients with spindle cell types.


2017 ◽  
Vol 35 (15_suppl) ◽  
pp. 10532-10532
Author(s):  
Jessica Hochberg ◽  
Liana Klejmont ◽  
Lauren Harrison ◽  
Allyson Flower ◽  
Quihu Shi ◽  
...  

10532 Background: Cure rates for CAYA patients with Hodgkin Lymphoma remain high, however are limited by significant toxicity of chemoradiotherapy. Brentuximab Vedotin and Rituximab have shown efficacy in relapsed HL. We hypothesize that the addition of both to combination chemotherapy will be safe in newly diagnosed HL preserving current EFS with elimination of more toxic chemoradiotherapy. Objective: To evaluate the safety and overall response and EFS of Brentuximab and Rituximab in combination with risk adapted chemotherapy in CAYA with newly diagnosed HL. Methods: Age 1-30 yrs with newly diagnosed classical HL given 3 to 6 cycles of chemoimmunotherapy: Brentuximab vedotin with Doxorubicin, Vincristine, Prednisone and Darcarbazine (Bv-AVPD) for Low risk patients or Doxorubicin, Vinblastine, Darcarbazine and Rituximab (Bv-AVD-R) for Intermediate/High risk. Early response measured by PET/CT scan following 2 cycles. Slow responders received an additional 2 cycles of Bv-AVD-R for Intermediate Risk or Ifosfamide/Vinorelbine for High Risk patients. Radiation therapy was given ONLY to those patients not in CR. Results: Total = 19 patients. Median age = 15yr (range 4-23yr). Risk = 2 low, 13 intermediate, 4 high. Toxcity = 1 episode of GrIII mucositis, 1 episode of GrIII infusion reaction to Brentuximab. 17 patients have completed therapy. All 17 patients achieved a complete response to therapy for a CR = 100%. Eleven (58%) have achieved a rapid early response. No patient has required radiation therapy. For 17 patients who have completed therapy, the EFS and OS is 100% with a median follow up time of 915 days (30 months). Conclusions: The addition of Brentuximab vedotin and Rituximab to combination chemotherapy for newly diagnosed Hodgkin Lymphoma appears to be safe. Our early results show significant promise with a CR rate of 100% and 58% rapid early response. We have successfully deleted toxic alkylator, topoisomerase inhibitor, bleomycin and radiation from this treatment regimen. The EFS/OS to date is 100% with a median follow up time of 2.5 years. Further follow up and a larger cohort is needed to determine long term outcomes of this approach. Clinical trial information: NCT02398240.


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