Ewing Sarcoma Developed at the Site of Previous Mast Cell Proliferation.

KIT gene mutations in Ewing sarcomas are rare; however, they are much more frequent in other neoplasms, namely mastocytosis. We describe a case of an adult male with a one-year duration of recurrent episodes of pain, swelling, and redness on the proximal phalanx of the third finger of his right hand. A core biopsy suggested a possible mastocytosis. After four years of recurrent episodes and worsening symptoms, an incisional biopsy revealed an Ewing sarcoma with a KIT gene mutation (M541L, on exon 10). KIT gene mutations with gain-of-function were identified in 2.6% of Ewing sarcomas. In this case, the detection of a KIT mutation in an Ewing sarcoma developed at the site of previous mast cell proliferation raises the hypothesis of a possible sarcomatous evolution of the original lesion. To the best of our knowledge, similar cases are not described in the current literature. This is also the first report describing the KIT M541L mutation (exon 10) in Ewing sarcoma.

Specialist management of testicular cancer: Report of the last 10 years at a Portuguese tertiary referral academic centre.

OBJECTIVES: To describe our experience on testicular cancer (TC) management, underlining the clinical/pathological scope, administered treatments, outcomes, and challenges. TC incidence is rising globally. The predominant histology is germ cell tumour (GCT). In most patients, orchiectomy is curative. Still, a significant proportion of patients will need further tailored treatment. Specialist Reference Centres have proven themselves successful in this setting. Published data regarding TC in Northern Portugal is lacking. METHODS: Retrospective review of consecutive TC patients at a specialist tertiary referral academic centre between January 2010 and December 2020. Statistical analysis was performed using the STATA® version 13.1 software. Multivariate logistic and survival analyses were performed. RESULTS: 125 patients met the inclusion criteria. The median age is 35 (28-40) years; 19% of patients had risk factors for TC - infertility being the most common (11%); 50% of patients wanted sperm cryopreservation prior to treatment; 68% of patients had stage I GCT, 16% stage II, and 17% stage III. Compared to seminoma, non-seminomatous GCT were associated with younger age (p < .001) and higher stages at diagnosis (p = .02); 24% of stage IA/B GCT underwent adjuvant chemotherapy; 47% of patients with metastatic GCT at presentation had refractory disease, requiring tailored treatment. The median follow-up time is 33 (13-65) months. There was no late relapse. The 5-year OS rate is 98.0%. The 5-year survival of metastatic disease is 95.8%. CONCLUSIONS: Despite contemporary excellent cure rates, the challenges of testicular cancer management still endure, especially in advanced stages. Therefore, public awareness is recommended, in order to avoid late presentations - special attention should be given to those who have known risk factors. The existence of Reference Centres is of paramount importance in order to achieve the best outcomes possible.

Complications of Biliary Drainage in Patients with Malignant Biliary Obstruction.

PURPOSE: Biliary tract obstruction in cancer patients is usually associated with a poor prognosis. The obstruction may cause distressing symptoms, such as pruritus. As this situation occurs mostly in advanced cancer, the primary objective of the treatment is in many cases symptom control and not prolonging life. However, some patients can be candidates for chemotherapy. To see the outcomes of stenting insertion in patients of our oncology center. METHODS: A retrospective study of patients who have undergone this procedure between 1 October 2011 and 31 December 2018 was carried out. RESULTS: Insertion of a biliary stent was performed in 171 patients. The most common diagnoses were gastric and colorectal cancers, each with 42 (24%), followed by pancreatic (34 (20%)) and biliary tract cancer (25 (14%)). Most stents (155 (91%)) were placed percutaneously. Complications were seen in 91 (53%) patients and the most common was cholangitis in 48 (53%) patients, and the median survival was 75.5 days (3-1246). A total of 168 (98%) patients were referred to palliative care. In a multivariable analysis, the ECOG performance status was associated with survival, with the ECOG 0, 1, and 2 associated with better survival and peritoneal metastases associated with lower survival. CONCLUSIONS: For many patients with advanced cancers, it may not be clear if the benefits of palliative biliary stents outweigh the risks. Therefore, the problem should be discussed with the patients and their families, making clear the goals of care and the potential benefits and risks that can be expected.