scholarly journals Sinonasal mucosal melanoma: treatment strategies and survival rates for a rare disease entity

Author(s):  
Alexandros Andrianakis ◽  
Peter Kiss ◽  
Markus Pomberger ◽  
Axel Wolf ◽  
Dietmar Thurnher ◽  
...  

Summary Background Sinonasal mucosal melanoma (SNMM) is a rare disease entity comprising 0.4–1.3% of all melanomas. Surgery with free margins has been the primary treatment over decades. Neither the addition of radiotherapy nor chemotherapy could significantly improve outcome rates of this devastating malignancy. This study presents our clinical experience with SNMM over a 19-year period and summarizes the current body of literature on SNMM. Methods This retrospective analysis included 12 patients with SNMM treated from 2001 to 2019 at an academic center. Additionally, a literature review of the last 29 years on treatment and survival data of SNMM was conducted. Results Main initial symptoms were epistaxis and nasal obstruction. Of the patients 9 underwent endoscopic surgery, 6 received adjuvant therapy. 3 patients who did not undergo surgery, received chemoradiotherapy, radiotherapy alone, and chemotherapy alone, respectively. At the time of diagnosis 2 patients had distant metastases and 4 patients developed distant metastases during the course of the disease. Mean overall survival (OS) was 30.6 months, 3‑year and 5‑year OS were 25%, and 18.2%, respectively. Conclusion Unspecific symptoms and hidden anatomic locations lead to delayed diagnosis and increased rates of metastatic dissemination. Distant metastasis is the main treatment failure in SNMM. Surgery with free margins remains the primary treatment for SNMM. Adjuvant radiotherapy might improve local control in individual cases but efficient systemic therapy is needed to improve outcome rates. To evaluate and define more effective targeted treatment options and improve outcome rates, homogeneous data and prospective multicentric analysis are needed.

2020 ◽  
Vol 31 (3) ◽  
pp. 234-238
Author(s):  
Therency Kamila dos Santos ◽  
Lucas Vinicius Machado Mamadi ◽  
Matheus Muller Gomes ◽  
Fabio Alex Fonseca Viegas ◽  
Mario Rodrigues Montemor Neto ◽  
...  

The central nervous system (CNS) is a common site of metastasis for a number of malignant tumors. Lung, breast, skin (melanoma), kidney and colorectal neoplasms form the most common group of primary sites that send distant metastases to the CNS, always via the hematogenous route. Delayed diagnosis, tumor aggressiveness, and failed primary treatment contribute to this evolution of secondary brain involvement. CNS metastases of salivary gland neoplasms are rare, with few cases reported in the literature. In the case here presented, a young male patient presents a peculiar situation of a carcinoma ex pleomorphic adenoma that developed metastasis to the brain by contiguity through the facial nerve. The outcome, as expected, was poor and the patient died.


2007 ◽  
Vol 25 (18_suppl) ◽  
pp. 14507-14507
Author(s):  
T. Liersch ◽  
J. Meller ◽  
T. Lorf ◽  
C. Sahlmann ◽  
M. Niessner ◽  
...  

14507 Background: As shown recently (JCO 2005; 23:6763–70), a single application of RAIT improved both, median overall survival (OS), and 5-year survival rates of colorectal cancer (CRC) patients (pts) post salvage resection of liver metastases (LM) compared to controls without RAIT (P=0.004). In an ongoing phase II trial we are evaluating the safety and efficacy of repeated RAIT at doses of 2x 40–50 mCi/m2 (3 mos apart) post salvage resection of LM. Methods: To date, 26 pts (8x f, 18x m; age: 63 ± 9 ys) who underwent surgery for CRC-LM have received the first dose of 131I-labetuzumab (Immunomedics, In., NJ, USA), a humanized monoclonal antibody against CEA, within 2 months of LM surgery. Three months after the first RAIT, a second infusion of 40–50 mCi/m2 has been applied to all pts after completion of standardized re-staging procedures. Results: The primary tumor sites were 17 colonic and 9 rectal cancers; primary tumor stages were 5x UICC-II, 7x UICC-III, 14x UICC-IV. 13 pts received adjuvant therapy. In 11 pts preoperative chemotherapy (FOLFOX or FOLFIRI) was given to achieve resectability of bilobular LM. After resection of LM (y)mTNM tumor stages were 1x mTNM-I, 6x mTNM-II, 6x mTNM-III and 4x mTNM-IV, respectively. After first RAIT, hematologic Grade 3 and 4, toxicity (WBC/platelet count) occurred in 8/14 and 5/3 pts, respectively. No cumulative toxicity was seen after repeated RAIT, with complete bone marrow recovery observed in all cases so far. To date, all pts are alive. Of the total, 17 pts received RAIT with adjuvant intention (as classified by FDG-PET and CT scans at pre-RAIT re-staging). In these, DFS was 70% post salvage resection of LM during ongoing follow-up of 15 months (median; range: 4–23 mos). As of Dec. 20, 2006, cancer recurrence was detected in 5/17 pts (3x pulmonary, 1x intrahepatic, 1x both) and in 4 pts R0-resection of distant metastases was done. 1 patient with pulmonary and intrahepatic relapses receives polychemotherapy with palliative intention. The pts‘ compliance to repeated RAIT has been 100%. Conclusion: RAIT re-treatment to date appears to be safe, feasible, and well accepted. Extended follow-up of the encouraging survival data will be presented. No significant financial relationships to disclose.


2019 ◽  
Vol 6 (4) ◽  
pp. 1176
Author(s):  
Zubair Afzal Khan

Background: Large number of the patients with gastric carcinoma (GC) remain asymptomatic, symptoms developed only in GC with advanced lesions with local or distant metastases. GC contributes significant morbidity and mortality related to malignancy, worldwide. The objectives of this study were to study the clinical presentation, surgery type and post-operative mortality and survival in case of patients with gastric carcinoma.Methods: The study included the patients with histologically proven cases of adenocarcinoma of stomach over a period of 5 years. The study excluded the patients with proven distant metastatic disease and/or in whom only palliative surgery was performed. All the patients were followed-up regularly till 5 years after the surgery.Results: The age-group of 51-60 years had maximum number of patients with GC followed by the age-group of 41-50 years. A majority of patients were presented with epigastric pain, weight loss, and anorexia. 30 (14.22%) patients had stage I GC while 77 (36.49%) and 104 (49.29%) patients had stage II and III GC, respectively. Overall survival of the patients was 75.62%, 56.22% and 44.78% at the end of 1-year, 2-years and 5-years.Conclusions: Delayed diagnosis of gastric carcinoma responsible for this poor survival rates. The screening programs must be implemented for early diagnosis which can improve survival as well as quality of life of the patients with gastric carcinoma.


2013 ◽  
Vol 51 (1) ◽  
pp. 22-30
Author(s):  
M. Jangard ◽  
J. Hansson ◽  
B. Ragnarsson-Olding

Objective: To establish population-based trends for sinonasal mucosal melanoma (SNMM) in Sweden. Methods: We identified 186 patients from the Swedish National Cancer Registry diagnosed with primary melanomas arising from the nasal cavity, paranasal sinuses, or both, during the period 1960 through 2000. Incidence, gender and age, primary anatomical sites, geographic distribution, treatment and survival were investigated. Results: The age-standardized incidence of SNMM increased significantly during the 41-year-period, with a higher overall incidence for females than males, but with a more rapid increase for males than for females. The incidence increased with age, peaking after the eightieth year in both genders. About 70 % of the cases were clinically amelanotic. The most common primary treatment was surgery. Five-year, disease-specific survival rates were poor for all these patients, but women had a significantly better survival time than men. For both genders the survival rate lengthened during the study period, irrespective of therapeutic strategy. Conclusion: SNMM is a rare disease, but the incidence in Sweden has increased significantly from 1960 through 2000, although not at the same pace as that of cutaneous malignant melanoma. Both the incidence and the survival were significantly higher in females than in males, but the reason for these gender differences is unknown.


Author(s):  
Maria Enrica Miscia ◽  
Giuseppe Lauriti ◽  
Dacia Di Renzo ◽  
Angela Riccio ◽  
Gabriele Lisi ◽  
...  

Abstract Introduction Esophageal atresia (EA) is associated with duodenal atresia (DA) in 3 to 6% of cases. The management of this association is controversial and literature is scarce on the topic. Materials and Methods We aimed to (1) review the patients with EA + DA treated at our institution and (2) systematically review the English literature, including case series of three or more patients. Results Cohort study: Five of seventy-four patients with EA had an associated DA (6.8%). Four of five cases (80%) underwent primary repair of both atresia, one of them with gastrostomy placement (25%). One of five cases (20%) had a delayed diagnosis of DA. No mortality has occurred. Systematic Review: Six of six-hundred forty-five abstract screened were included (78 patients). Twenty-four of sixty-eight (35.3%) underwent primary correction of EA + DA, and 36/68 (52.9%) underwent staged correction. Nine of thirty-six (25%) had a missed diagnosis of DA. Thirty-six of sixty-eight underwent gastrostomy placement. Complications were observed in 14/36 patients (38.9 ± 8.2%). Overall mortality reported was 41.0 ± 30.1% (32/78 patients), in particular its incidence was 41.7 ± 27.0% after a primary treatment and 37.0 ± 44.1% following a staged approach. Conclusion The management of associated EA and DA remains controversial. It seems that the staged or primary correction does not affect the mortality. Surgeons should not overlook DA when correcting an EA.


2019 ◽  
Vol 72 (8) ◽  
pp. 1523-1526
Author(s):  
Oleksandr O. Lytvynenko ◽  
Volodymyr F. Konovalenko ◽  
Anton Yu. Ryzhov

Introduction: The treatment of patients with malignant fibrous histiocytoma as well as other soft tissue sarcomas is not sufficiently effective up to date, and has largely changed and reflects the alterations, occurred in oncology as a whole. The number of amputation decreased over the last 10-15 years. Some researchers associate the improvement of treatment outcomes with the development of combined and complex methods. The aim of the study is an improvement of the results of treatment of patients with soft tissue malignant histiocytoma on the basis of determination of factors, influencing local recurrence development. Materials and methods: The basis of our study was a comprehensive analysis of examination and treatment results of 130 patients with MFH of the soft tissue of limbs, of them in 84 patients (64.6%) the recurrences developed. The group included 45 (53.6%) males and 39 (46.4%) females. The major part of patients – 82.1% (60 patients) were older than 40 years. Results and conclusions: The number of recurrences after the treatment in general surgical facilities is 86.9%, whereas in the patients after the treatment in the specialized oncological facilities this figure is twice lower (40%). The characteristic of the medical facility where the patient receives his/her primary treatment largely affects the development of local recurrences, patients’ quality of life and overall survival rates. The surgical method remains the leading modality in the treatment of MFH of ST. Wide and radical excision of tumors in the specialized oncological facilities allows achieving better survival outcomes of the patients.


2020 ◽  
Vol 22 (Supplement_2) ◽  
pp. ii138-ii138
Author(s):  
Iyad Alnahhas ◽  
Appaji Rayi ◽  
Yasmeen Rauf ◽  
Shirley Ong ◽  
Pierre Giglio ◽  
...  

Abstract INTRODUCTION While advocacy for inmates with cancer has recently gained momentum, little is known about management of brain tumors in inmates. Delays in acknowledging or recognizing nonspecific initial symptoms can lead to delayed diagnosis and treatment. Inmates with cancer are reported to either be ignored or receive substandard care due in part to cost or logistics (American Civil Liberties Union; ASCO Post 2018). METHODS In this retrospective study, we identified inmates with gliomas seen in the Ohio State University Neuro-oncology Center between 1/1/2010-4/20/2019. RESULTS Twelve patients were identified. Median age at presentation was 39.5 years (range 28-62). Eleven patients were Caucasian and one was African American. Diagnoses included glioblastoma (GBM) (n=6), anaplastic astrocytoma (n=1), anaplastic oligodendroglioma (n=1), low-grade astrocytoma (n=3) and anaplastic pleomorphic xanthroastrocytoma (n=1). Patients were more likely to present early after seizures or focal neurologic deficits (9/12) than after headaches alone. Patients with GBM started RT 12-71 days after surgery (median 34.5). One patient’s post-RT MRI was delayed by a month and another with GBM had treatment held after 4 cycles of adjuvant temozolomide (TMZ) due to “incarceration issues”. For one patient who received adjuvant TMZ, the facility failed to communicate with the primary team throughout treatment. Two patients suffered significant nausea while on chemotherapy due to inability to obtain ondansetron in prison, or due to wrong timing. 7/12 (58%) patients were lost to follow-up for periods of 3-15 months during treatment. Three patients refused adjuvant treatment. CONCLUSIONS Although this is a small series, our results highlight the inequities and challenges faced by inmates with gliomas who are more likely to forego treatments or whose incarceration prevents them from keeping appropriate treatment and follow-up schedules. Additional studies are needed to define and address these deficiencies in the care of inmates with brain tumors and other cancers.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1103.1-1104
Author(s):  
N. Iniesta-Arandia ◽  
G. Espinosa ◽  
A. Guillen del Castillo ◽  
C. Tolosa ◽  
G. M. Lledó ◽  
...  

Background:Anti-PM/Scl antibodies are associated to systemic sclerosis (SSc) but are not specific to SSc. The true prevalence of anti-PM/Scl antibodies in SSc is unknown, ranging from 2.5% to 12.5%. An association between anti-PM/Scl antibodies with muscular involvement, pulmonary fibrosis, calcinosis, and a relatively benign prognosis have been described.Objectives:To compare the clinical manifestations and prognosis of SSc patients according the presence of anti-PM/Scl antibodies in the cohort of RESCLE (Spanish Scleroderma Registry).Methods:From the Spanish Scleroderma Study Group database, we selected patients in whom anti-PM/Scl antibodies had been tested. We compared demographic features, clinical manifestations, laboratory characteristics, and survival data between patients according the anti-PM/Scl antibodies status.Results:72 out of 947 (7%) patients tested positive for anti-PM/Scl antibodies. As presenting SSc manifestations, patients with anti-PM/Scl antibodies had higher prevalence of puffy fingers (11% versus 2%; p=0.002) and arthralgias (11% versus 4%; p=0.03), and lower prevalence of Raynaud’s phenomenon (65% versus 82%, p=0.002). Regarding cumulative manifestations, myositis (51% versus 15%; p<0.001), arthritis (43% versus 22%; p=0.001), and interstitial lung disease (ILD) (60% versus 45%, p=0.014) were more prevalent in patients with anti-PM/Scl antibodies. In fact, those patients with anti-Pm/Scl antibodies presented with FVC (77.4% ± 23.1% versus 85.8% ± 23,1%; p=0.006) and more severe ILD defined as FVC <70% (41% versus 24%; p=0.004). Death rate was similar in patients with and without PM/Scl antibodies (18% versus 17%; p=0.871).We did not find differences in terms of death rate nor in the causes of death (SSc and non-SSc related) according to the anti-PM/Scl antibodies profile.The 5- and 10-years survival rates of patients with anti-PM/Scl antibodies were 91% and 82% respectively, without differences with those without these antibodies (93% and 85%, respectively).Conclusion:In Spanish SSc patients, the presence of anti-PM/Scl antibodies confer a distinctive clinical profile. However, anti-PM/Scl antibodies do not play a role in the prognosis of these patients.References:[1]Stochmal A, Czuwara J, Trojanowska M, Rudnicka L. Antinuclear antibodies in systemic sclerosis: an update. Clin Rev Allergy Immunol 2020;58(1):40-51. doi: 10.1007/s12016-018-8718-8.Acknowledgments:We gratefully acknowledge all investigators who are part of the RESCLE Registry. We also thank the RESCLE Registry Coordinating Centre, S&H Medical Science Service, for their quality control data, logistic and administrative support and Prof. Salvador Ortiz, Universidad Autónoma de Madrid and Statistical Advisor S&H Medical Science Service for the statistical analysis of the data presented in this paper.Disclosure of InterestsNerea Iniesta-Arandia: None declared, Gerard Espinosa Speakers bureau: Glaxo-Smith-Kline, Janssen, Boehringer, Rovi, Alfredo Guillen del Castillo: None declared, Carles Tolosa Consultant of: Actelion pharmaceuticals, GSK, MSD., Gema Maria Lledó: None declared, Dolores Colunga Argüelles Consultant of: Actelion pharmaceuticals, GSK, MSD., Cristina González-Echávarri: None declared, Luis Sáez-Comet: None declared, Norberto Ortego: None declared, Jose Antonio Vargas-Hitos: None declared, Manuel Rubio-Rivas: None declared, Mayka Freire: None declared, Juan José Rios: None declared, Monica Rodriguez-Carballeira: None declared, Luis Trapiella Martínez: None declared, Vicent Fonollosa Pla Speakers bureau: Actelion, Carmen Pilar Simeón-Aznar Consultant of: Actelion pharmaceuticals, GSK, MSD., on behalf of RESCLE Investigators, Autoimmune Diseases Study Group (GEAS): None declared


2021 ◽  
Vol 0 (0) ◽  
Author(s):  
Maximilian Richter ◽  
Lena Sonnow ◽  
Amir Mehdizadeh-Shrifi ◽  
Axel Richter ◽  
Rainer Koch ◽  
...  

Abstract Objectives To evaluate how the certification of specialised Oncology Centres in Germany affects the relative survival of patients with colorectal cancer (CRC) by means of national and international comparison. Methods Between 2007 and 2013, 675 patients with colorectal cancer, treated at the Hildesheim Hospital, an academic teaching hospital of the Hannover Medical School (MHH), were included. A follow-up of the entire patient group was performed until 2014. To obtain international data, a SEER-database search was done. The relative survival of 148,957 patients was compared to our data after 12, 36 and 60 months. For national survival data, we compared our rates with 41,988 patients of the Munich Cancer Registry (MCR). Results Relative survival at our institution tends to be higher in advanced tumour stages compared to national and international cancer registry data. Nationally we found only little variation in survival rates for low stages CRC (UICC I and II), colon, and rectal cancer. There were notable variations regarding relative survival rates for advanced CRC tumour stages (UICC IV). These variations were even more distinct for rectal cancer after 12, 36 and 60 months (Hildesheim Hospital: 89.9, 40.3, 30.1%; Munich Cancer Registry (MCR): 65.4, 28.7, 16.6%). The international comparison of CRC showed significantly higher relative survival rates for patients with advanced tumour stages after 12 months at our institution (77 vs. 54.9% for UICC IV; raw p<0.001). Conclusions Our findings suggest that patients with advanced tumour stages of CRC and especially rectal cancer benefit most from a multidisciplinary and guidelines-oriented treatment at Certified Oncology Centres. For a better evaluation of cancer treatment and improved national and international comparison, the creation of a centralised national cancer registry is necessary.


Kidney Cancer ◽  
2021 ◽  
pp. 1-14
Author(s):  
Melissa Bersanelli ◽  
Camillo Porta

Background: The SARS-CoV-2 pandemic still has a huge impact on the management of many chronic diseases such as cancer. Few data are presently available reagarding how the management of renal cell carcinoma (RCC) has changed due to this unprecedented situation. Objective: To discuss the challenges and issues of the diagnosis and treatment of RCC in the COVID-19 era, and to provide recommendations based on the collected literature and our personal experience. Methods: Systematic review of the available Literature regarding the management of RCC during the SARS-CoV-2 pandemic. Results: Our review showed a prevalence of narrative publications, raising the issue of the real relevance of the evidence retrieved. Indeed, the only original data about RCC and COVID-19 found were a small retrospective case series and two surveys, providing either patients’ or physicians’ viewpoints. Conclusions: The expected delayed diagnosis of RCC could lead to an increase of advanced/metastatic cases; thus, proper therapeutic choices for patients with small renal masses should be carefully evaluated case by case, in order to avoid negative effects on long-term survival rates. The controversial interaction between immune checkpoint blockade and COVID-19 pathogenesis is more hypothetical than evidence-based, and thus immunotherapy should not be denied, whenever appropriate. To avoid treatments which won’t have an impact on patients’ survival, a honest and accurate evaluation of the cost/benefit ratio of each treatment option should be always performed. Finally, SARS-CoV-2 swab positivity should not prevent the continuation of ongoing active treatments in asymptomatic cases, or or after symptoms’ resolution.


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