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

Author(s):  
Jordan W. Francheville ◽  
Thomas Arnason ◽  
Michael D. Carter ◽  
Kian Khodadad ◽  
Carman Giacomantonio ◽  
...  

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.

2021 ◽  
Vol 14 (1) ◽  
pp. 51
Author(s):  
Brinda Balasubramanian ◽  
Simran Venkatraman ◽  
Kyaw Zwar Myint ◽  
Tavan Janvilisri ◽  
Kanokpan Wongprasert ◽  
...  

Cholangiocarcinoma (CCA), a group of malignancies that originate from the biliary tract, is associated with a high mortality rate and a concerning increase in worldwide incidence. In Thailand, where the incidence of CCA is the highest, the socioeconomic burden is severe. Yet, treatment options are limited, with surgical resection being the only form of treatment with curative intent. The current standard-of-care remains adjuvant and palliative chemotherapy which is ineffective in most patients. The overall survival rate is dismal, even after surgical resection and the tumor heterogeneity further complicates treatment. Together, this makes CCA a significant burden in Southeast Asia. For effective management of CCA, treatment must be tailored to each patient, individually, for which an assortment of targeted therapies must be available. Despite the increasing numbers of clinical studies in CCA, targeted therapy drugs rarely get approved for clinical use. In this review, we discuss the shortcomings of the conventional clinical trial process and propose the implementation of a novel concept, co-clinical trials to expedite drug development for CCA patients. In co-clinical trials, the preclinical studies and clinical trials are conducted simultaneously, thus enabling real-time data integration to accurately stratify and customize treatment for patients, individually. Hence, co-clinical trials are expected to improve the outcomes of clinical trials and consequently, encourage the approval of targeted therapy drugs. The increased availability of targeted therapy drugs for treatment is expected to facilitate the application of precision medicine in CCA.


Author(s):  
Maria Paola Ciliberti ◽  
Rosa D'Agostino ◽  
Laura Gabrieli ◽  
Anna Nikolaou ◽  
Angela Sardaro

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.


2015 ◽  
Vol 5 (1) ◽  
Author(s):  
Hamidreza Namazi ◽  
Vladimir V. Kulish ◽  
Albert Wong

Abstract Cancer is a class of diseases characterized by out-of-control cells’ growth which affect DNAs and make them damaged. Many treatment options for cancer exist, with the primary ones including surgery, chemotherapy, radiation therapy, hormonal therapy, targeted therapy and palliative care. Which treatments are used depends on the type, location and grade of the cancer as well as the person’s health and wishes. Chemotherapy is the use of medication (chemicals) to treat disease. More specifically, chemotherapy typically refers to the destruction of cancer cells. Considering the diffusion of drugs in cancer cells and fractality of DNA walks, in this research we worked on modelling and prediction of the effect of chemotherapy on cancer cells using Fractional Diffusion Equation (FDE). The employed methodology is useful not only for analysis of the effect of special drug and cancer considered in this research but can be expanded in case of different drugs and cancers.


2011 ◽  
Vol 152 (28) ◽  
pp. 1120-1124
Author(s):  
Zsolt Baranyai ◽  
Dániel Sinkó ◽  
Valéria Jósa ◽  
Attila Zaránd ◽  
Dániel Teknős

Radiation enteritis is one of the most feared complications after abdominal and pelvic radiation therapy. The incidence varies from 0.5 to 5%. It is not rare that the slowly progressing condition will be fatal. During a period of 13 years 24 patients were operated due to the complication of radiation enteritis. Despite different types of surgery repeated operation was required in 25% of cases and finally 4 patients died. Analyzing these cases predisposing factors and different therapeutic options of this condition are discussed. Treatment options of radiation induced enteritis are limited; however, targeted therapy significantly improves the outcome. Cooperation between oncologist, gastroenterologist and surgeon is required to establish adequate therapeutic plan. Orv. Hetil., 2011, 152, 1120–1124.


Digestion ◽  
2021 ◽  
pp. 1-8
Author(s):  
Haruna Miyashita ◽  
Takuji Yamasaki ◽  
Yoshihiro Akita ◽  
Yoshitaka Ando ◽  
Yuki Maruyama ◽  
...  

<b><i>Background and Aims:</i></b> In gastrointestinal neuroendocrine tumors (GI-NETs), tumor size and grading based on cellular proliferative ability indicate biological malignancy but not necessarily clinically efficient prognostic stratification. We analyzed tumor size- and grading-based prevalence of lymphovascular invasion in GI-NETs to establish whether these are true biological malignancy indicators. <b><i>Methods:</i></b> We included 155 cases (165 lesions), diagnosed histologically with GI-NETs, that had undergone endoscopic or surgical resection. Patient age, sex, method of treatment, tumor size, invasion depth, lymphovascular invasion positivity according to Ki-67 index-based neuroendocrine tumor grading, distant metastases, and outcome were evaluated. The primary endpoints were the prevalence of lymphovascular invasion according to tumor size and grading. <b><i>Results:</i></b> Overall, 24.8% were positive for lymphovascular invasion. There was a high rate of lymphovascular invasion positivity even among grade 1 cases (22.8%). The rate of lymphovascular invasion was 3.4% for grade 1 cases &#x3c;5 mm, with a lymphovascular invasion rate of 8.7% for those 5–10 mm. Lymphovascular invasion ≤10% required a tumor size ≤8 mm, and lymphovascular invasion ≤5% required a tumor size ≤6 mm. A cutoff of 6 mm was identified, which yielded a sensitivity of 79% and a specificity of 63%. Even small GI-NETs grade 1 of the whole GI tract also showed positive for lymphovascular invasion. <b><i>Conclusions:</i></b> GI-NETs ≤10 mm had a lymphovascular invasion prevalence exceeding 10%. The lymphovascular invasion impact in GI-NET development is incompletely understood, but careful follow-up, including consideration of additional surgical resection, is crucial in cases with lymphovascular invasion.


2021 ◽  
pp. 739-745
Author(s):  
Zane Blank ◽  
Richard Sleightholm ◽  
Beth Neilsen ◽  
Michael Baine ◽  
Chi Lin

Juvenile nasopharyngeal angiofibroma (JNA) is a relatively uncommon, benign neoplasm of the nasopharynx that can be very difficult to diagnose early due to inconspicuous and seemingly harmless presenting symptoms. Early diagnosis and treatment of JNA are essential for a good prognosis. JNA typically responds well to radiation therapy (RT), but when it does not, the most appropriate next course of action has not been readily defined due to the limited occurrence and experience with this neoplasm. Herein, we describe a JNA patient, who continued to progress after surgery and 36 Gy of adjuvant radiation, but after an additional 14.4 Gy, he has remained in remission for over 2 years. An 11-year-old boy who presented with JNA underwent treatment with embolization and surgical resection. Unfortunately, the tumor progressed within 2 months of surgical intervention and he required RT for adequate local control. While undergoing RT, he again demonstrated signs of progression; so his radiation regimen was increased from 3,600 cGy in 20 fractions to 5,040 cGy in 28 fractions. Since completing RT, the tumor has continued to decrease in size, and the patient is stable and has been without signs of disease progression for over 24 months now. Thus, escalating the radiation regimen to 5,040 cGy may improve local control in rapidly progressive JNA.


2021 ◽  
pp. 000348942110081
Author(s):  
Sara Behbahani ◽  
Gregory L. Barinsky ◽  
David Wassef ◽  
Boris Paskhover ◽  
Rachel Kaye

Objective: Primary tracheal malignancies are relatively rare cancers, representing 0.1% to 0.4% of all malignancies. Adenoid cystic carcinoma (ACC) is the second most common histology of primary tracheal malignancy, after squamous cell carcinoma. This study aims to analyze demographic characteristics and potential influencing factors on survival of tracheal ACC (TACC). Methods: This was a retrospective cohort study utilizing the National Cancer Database (NCDB). The NCDB was queried for all cases of TACC diagnosed from 2004 to 2016 (n = 394). Kaplan-Meier (KM) and Cox proportional-hazards models were used to determine clinicopathological and treatment factors associated with survival outcomes. Results: Median age of diagnosis was 56 (IQR: 44.75-66.00). Females were affected slightly more than males (53.8% vs 46.2%). The most prevalent tumor diameter range was 20 to 39 mm (34.8%) followed by greater than 40 mm in diameter (17.8%). Median overall survival (OS) was 9.72 years with a 5- and 10-year OS of 70% and 47.5%, respectively. Localized disease was not associated with a survival benefit over invasive disease ( P = .388). The most common intervention was surgery combined with radiation therapy (RT) at 46.2%, followed by surgery alone (16.8%), and standalone RT (8.9%). When adjusting for confounders, surgical resection was independently associated with improved OS (HR 0.461, 95% CI 0.225-0.946). Tumor size greater than 40 mm was independently associated with worse OS (HR 2.808; 95% CI 1.096-7.194). Conclusion: Our data suggests that surgical resection, possibly in conjunction with radiation therapy, is associated with improved survival, and tumor larger than 40 mm are associated with worse survival.


2012 ◽  
Vol 2012 ◽  
pp. 1-5 ◽  
Author(s):  
William Yoon ◽  
Kari Paulson ◽  
Paul Mazzara ◽  
Sweety Nagori ◽  
Mohammed Barawi ◽  
...  

Schwannomas are generally slow growing asymptomatic neoplasms that rarely occur in the GI tract. However, if found, the most common site is the stomach. Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract, and 60–70% of them occur in the stomach. Owing to their typical presentation as submucosal neoplasms, gastric schwannomas and GISTs appear grossly similar. Accordingly, the differential diagnosis for a gastric submucosal mass should include gastric schwannomas. Furthermore, GI schwannomas are benign neoplasms with excellent prognosis after surgical resection, whereas 10–30% of GISTs have malignant behavior. Hence, it is important to distinguish gastric schwannomas from GISTs to make an accurate diagnosis to optimally guide treatment options. Nevertheless, owing to the paucity of gastric schwannomas, the index of suspicion for this diagnosis is low. We report a rare case of gastric schwannoma in 53-year-old woman who underwent laparoscopic partial gastrectomy under the suspicion of a GIST preoperatively but confirmed to have a gastric schwannoma postoperatively. This case underscores the importance of including gastric schwannomas in the differential diagnosis when preoperative imaging studies reveal a submucosal, exophytic gastric mass. For a gastric schwannoma, complete margin negative surgical resection is the curative treatment of choice.


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