rare tumors
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2021 ◽  
pp. 149-154
Author(s):  
Murat Ozdemir ◽  
Özer Makay
Keyword(s):  

2021 ◽  
Vol 8 ◽  
Author(s):  
Cecilie Mullerup Kiel ◽  
Preben Homøe

We report a case of a large, ulcerating proliferating trichilemmal cyst in a 76-year-old woman, with clinical, radiological, macroscopic, and microscopic correlation. The outbreak of the Coronavirus pandemic delayed her treatment. We review the literature on proliferating trichilemmal cysts, which are relatively rare tumors, which, generally, are considered benign. However, we found a high rate of malign cases, which stresses the importance of rapid surgical excision and histological diagnosis. Even though our proband had delayed treatment, the tumor did not transform into a malignant form.


2021 ◽  
Author(s):  
Felicitas Hippert ◽  
Lena Desing ◽  
Sonja Diez ◽  
Andrea Witowski ◽  
Benedikt Bernbeck ◽  
...  

AbstractBackground Very rare tumors (VRT) in children and adolescents have such a low incidence that until recently, they have not been integrated into the clinical and scientific network of pediatric oncology. Data is very limited and consistent treatment strategies are missing. Thus, VRTs are classic orphan diseases. To counteract this problem, the Arbeitsgemeinschaft für Seltene Tumorerkrankungen in der Pädiatrie (STEP) was founded. Here we report on patient recruitment during the first 10 years. Patients Patients aged up to 18 years and not included in any other clinical trial or GPOH registry were included in this analysis. Methods Data was collected from 2008 to 2018 by means of a standardized form. The recorded diagnoses were descriptively analyzed focusing on histology, localization, and year of report. Results A total of 623 patients with VRTs were registered. During 2008–2014, the annual number of registrations was around 40 and is around 90 since 2015. Most frequent diagnoses included tumors of the skin (n=150), tumors of the gastrointestinal tract (n=102), tumors of the gonads (n=77), the ENT region (n=68), and miscellaneous tumors (n=107). Discussion With the establishment of central structures for clinical consultation and documentation of VRTs, the number of registrations increased. Comprehensively, VRTs are as common as other classic pediatric oncology tumors, but extremely heterogeneous in terms of localization, histology, and prognosis. By a centralized and complete registration and analysis of VRTs, also in collaboration with international partners, it is possible to develop treatment strategies and thus greatly increase treatment quality.


2021 ◽  
pp. 030089162110523
Author(s):  
Shushan Hovsepyan ◽  
Lusine Hakobyan ◽  
Armen Mkhitaryan ◽  
Monica Terenziani ◽  
Andrea Ferrari ◽  
...  

Background: The lack of internationally recognized guidelines for very rare tumors, such as juvenile granulosa cell tumors (JGCTs), which are nonepithelial, unusual ovarian tumors, is a challenge for pediatric oncologists, especially in developing countries with limited resources and experience in treating rare tumors. Methods: We report clinical data of 2 girls with JGCTs treated at the Pediatric Cancer and Blood Disorders Center of Armenia with the assistance of the EXPeRT (European Cooperative Study Group for Pediatric Rare Tumors) international cooperation panel. Case presentation: Two girls (16 and 15 years old) with JGCTs of the ovaries, stage Ic, underwent surgery and, with consultation through an online advisory board ( http://vrt.cineca.it/ ), received 4 cycles of chemotherapy according to the PEI regimen (cisplatin, etoposide, ifosfamide). Conclusion: Very rare tumors, especially in advanced stages, have limited data and a low survival rate. International collaboration with the EXPeRT group is beneficial for physicians with limited experience and facilitates research in pediatric oncology.


2021 ◽  
Author(s):  
Dansou Gaspard GBESSI ◽  
Freddy Houéhanou Rodrigue GNANGNON ◽  
Aboudou Raïmi KPOSSOU ◽  
Prudent Pacifique GBETCHEDJI ◽  
Falilatou Seidou ◽  
...  

Abstract Background: GISTs are rare tumors but the most frequent mesenchymal tumors of the digestive tract. Diagnosis and treatment are challenging in low-income countries due to relatively poor access to immunohistochemistry and targeted therapy. In Africa there are few studies about it. This study described the diagnosis and therapeutic management of GIST in Cotonou, Benin.Methods: This was a descriptive cross-sectional study, with retrospective data collection over a 10-year period from 2010 to 2020, focused on patients with histological confirmed gastro-intestinal stromal tumor (GIST). Data analysis was performed with Epidata Analysis software version 3.0.0.1.Results: Fifteen GISTs were identified during the study period. The median age was 52 and the sex ratio was 2. The most frequent symptom was abdominal pain (n = 12). Delay in care seeking after onset of symptoms ranged from 24 hours to 15 years. The most common site for GISTs was the stomach (n = 8). The median tumor size was 11 cm and the majority (n=10) was metastatic or locally advanced at the time of diagnosis. The tumors were often spindle-shaped at histology (n = 13) and the majority expressed KIT (n = 14). Most of the tumors (n = 12) were at high risk of recurrence according to the Joensuu scoring system. The availability of imatinib have improved the outcome of GIST with response in all cases it was used in neoadjuvant setting (n = 7).Conclusion: GISTs are rare tumors and preferentially affect the stomach in Cotonou. Most of the tumors were large, unresectable at the time of diagnosis and at high risk of recurrence. Access to imatinib has revolutionized the management of those tumors in our country.


2021 ◽  
Vol 6 (4) ◽  
pp. 14-25
Author(s):  
Amjad Zafar ◽  
Rabia Iqbal ◽  
Amina Jafar ◽  
Taimoor Bajwa ◽  
Sobia Yaqub ◽  
...  

Introduction: Neuroendocrine tumors (NETs) are rare tumors with varied clinical presentations. Entero-pancreatic and respiratory systems are usually involved but it can also affect unusual sites like the liver. Purpose: This paper presents a case of a 45 years female who developed progressive and disabling symptoms of mass effect and carcinoid but remained undiagnosed for many years due to extremely low suspicion of such a tumor in liver. Methods: Diagnosis was made after extensive radiological, histopathological, and biochemical investigations. By that time, disease had spread to her breast which is also not a typical feature of NETs. Findings: Diagnosis of rare tumors at an unusual site is challenging and requires high clinical suspicion and appropriate workup.


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