scholarly journals MON-196 A Single-Center Experience of Patients with Metastatic Pheochromocytoma/Paraganglioma Treated with 177Lu-DOTATATE

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Anna Roslyakova ◽  
Dmitry Beltsevich ◽  
Michail Plotkin ◽  
Pavel Rumyantsev ◽  
Daria Ladygina

Abstract The prevalence of metastatic pheochromocytoma/paraganglioma (PPGL) is reported to be 3% to 36% of all the cases. The five-year overall survival rate of such patients ranges from 40 to 77%. The management of metastatic PPGL is challenging taking into account the fact that the optimal strategies for clinical care beyond surgical resection are not guided yet. Peptide receptor radionuclide therapy (PRRT) using somatostatin analogues is effective in other neuroendocrine tumours, can be indicated in patients with positive scans for the respective radiopharmaceuticals. We report preliminary data of the prospective study aimed to assess the safety and efficacy outcomes of 177Lu-DOTATATE for 6 patients with histologically confirmed PPGLs with metastatic progression after the complete PPGL surgery. The mean age of our cohort was 53 years (range 14–73); an equal number of male and female patients was included. Two of them had germline mutations in RET and SDHB genes, respectively. Radiological response utilized RECIST 1.1 criteria; toxicity was graded according to common terminology criteria for adverse events version 4. PPRT scheme varied between three and four cycles. Partial response (PR) was achieved in one and stable disease (SD) in four 4 patients. One patient had treatment refractory with disease progression and dramatic increase of chromogranin A concentration (+268%). Biochemical response (>50% decrease) of chromogranin A was found in 1/6 patients and of catecholamines in 2/6 patients. No hematological or kidney toxicity grade 3–4 was registered. Median overall survival and median progression-free survival rate will be reported after the end of the study. To date, about 250 PPGL patients have been treated with PRRT. PRRT using 177Lu-DOTA-SSAs has shown promise for treatment of PPGLs with improvement of clinical symptoms and/or disease control in the setting of retrospective small case reports or case series. However, more well-designed prospective studies are required to confirm these findings.

2016 ◽  
Vol 10 (1) ◽  
pp. 15-25 ◽  
Author(s):  
Mseddi M. Amine ◽  
Bouzguenda Mohamed ◽  
Hadjslimane Mourad ◽  
Hamza Majed ◽  
Charfi Slim ◽  
...  

Purpose: The purpose of this study is to study the main epidemiological, clinical, para clinical, pathological, therapeutic, and evolutionary features of patients with testicular neuroendocrine tumors (TNET). Materials and Methods: Nine case series and sixteen case reports were identified by searching PubMed database and qualified for inclusion in this study. We added the data of one case treated in the department of urology in Habib Bourguiba Hospital in Sfax, to the published cases. Results: A total of 132 cases were collected. Median age at diagnosis was 39 years old (range 10- 83 years). The most common presenting symptom was either a testicular mass or a swelling in 38.46% of cases. Carcinoid syndrome was documented in 10.60% of patients. The clinical examination revealed a palpable mass in 44.70% of patients. This mass was painless and firm in most cases. Serum tumor markers (β-gonadotrophine chorionique humaine, α-feto protein, and lactate dehydrogenase) were within normal limits in all patients except in one case. Most testicular neuroendocrine tumors (76.52%) were primary and pure. The tumors were positive for chromogranin (100%), synaptophysin (100%) and cytokeratin (93.10%). Metastases were detected at time of diagnosis in eight cases (6.06%). The main treatment was radical orchiectomy performed in 127 patients (96.21%). The 5-year overall survival rate was 78.70% and the 5-year specific survival rate was 84.30%. Conclusion: The diagnosis of testicular carcinoids is based on the immunohistochemistry study. The treatment of choice for these tumors is radical orchiectomy. Somatostatin analogues were reported to be effective in patients with carcinoid syndrome.


2019 ◽  
Vol 58 (3) ◽  
pp. 282-292
Author(s):  
Katelyn A Pastick ◽  
Elizabeth Nalintya ◽  
Lillian Tugume ◽  
Kenneth Ssebambulidde ◽  
Nicole Stephens ◽  
...  

Abstract Cryptococcal meningitis causes 15% of AIDS-related deaths. Optimal management and clinical outcomes of pregnant women with cryptococcosis are limited to case reports, as pregnant women are often excluded from research. Amongst pregnant women with asymptomatic cryptococcosis, no treatment guidelines exist. We prospectively identified HIV-infected women who were pregnant or recently pregnant with cryptococcosis, screened during a series of meningitis research studies in Uganda from 2012 to 2018. Among 571 women screened for cryptococcosis, 13 were pregnant, one was breastfeeding, three were within 14 days postpartum, and two had recently miscarried. Of these 19 women (3.3%), 12 had cryptococcal meningitis, six had cryptococcal antigenemia, and one had a history of cryptococcal meningitis and was receiving secondary prophylaxis. All women with meningitis received amphotericin B deoxycholate (0.7–1.0 mg/kg). Five were exposed to 200–800 mg fluconazole during pregnancy. Of these five, three delivered healthy babies with no gross physical abnormalities at birth, one succumbed to meningitis, and one outcome was unknown. Maternal meningitis survival rate at hospital discharge was 75% (9/12), and neonatal/fetal survival rate was 44% (4/9) for those mothers who survived. Miscarriages and stillbirths were common (n = 4). Of six women with cryptococcal antigenemia, two received fluconazole, one received weekly amphotericin B, and three had unknown treatment courses. All women with antigenemia survived, and none developed clinical meningitis. We report good maternal outcomes but poor fetal outcomes for cryptococcal meningitis using amphotericin B, without fluconazole in the first trimester, and weekly amphotericin B in place of fluconazole for cryptococcal antigenemia.


2019 ◽  
Vol 18 ◽  
pp. 153473541982883 ◽  
Author(s):  
Wendy Wong ◽  
Bing Zhong Chen ◽  
Allyson Kin Yan Lee ◽  
Adrian Ho Cheung Chan ◽  
Justin Che Yuen Wu ◽  
...  

Background and Aims: Pancreatic cancer has the lowest survival rate of all cancers (4%), and it accounts for 1.9% of new cancer cases in Hong Kong. Combined treatment with Chinese herbal medicine (CHM) and Western medicine has yielded promising results, leading to improved prognosis and overall survival. This retrospective case series aimed to illustrate the improved survival and quality of life outcomes of pancreatic cancer patients administered CHM based on traditional Chinese medicine theory. Methods: To investigate the effectiveness of CHM in prolonging overall survival, 182 patients diagnosed with pancreatic cancer who received CHM treatment were observed from 2005 to 2015. Results: One hundred eighty-two pancreatic cancer patients were treated with CHM; 21 patients died. The mean and median survival of these patients were 29.6 and 15.2 months, respectively; the 1-year survival rate was 76% (range = 4 months to 9 years). These results are better than those reported in patients treated with Western medicine, suggesting the need for further study of CHM. Conclusion: A superior clinical outcome may be obtained with CHM treatment. The case series illustrates the potential benefits and safety issues of CHM in pancreatic cancer patients that could be relevant for developing strategies to increase individualization of pancreatic cancer treatment and improve survival. This study may facilitate interprofessional communication and improved clinical management of pancreatic cancer patients.


2017 ◽  
Vol 156 (3) ◽  
pp. 498-503 ◽  
Author(s):  
Jérôme Donnadieu ◽  
Nathalie Klopp-Dutote ◽  
Aurélie Biet-Hornstein ◽  
Vladimir Strunski ◽  
Geoffrey Mortuaire ◽  
...  

Objective To analyze the survival rate of a nonselected pyriform sinus cancer population. Study Design Case series with chart review. Setting University hospital. Subjects and Methods A total of 122 patients were included in this study covering the 2002-2008 period. All patients had squamous cell carcinoma originating from the pyriform sinus. Survival and prognostic factors were analyzed. Results The 3- and 5-year overall survival rates were 39.7% and 2.4%, respectively. The 3- and 5-year survival rates without recurrence were 34% and 27%, respectively. The median survival rates by UICC stage were as follows: stage 1 and 2 patients, 60 months; stage 3, 40 months; stage 4, 19 months. Stage 4 patients had a lower median survival rate than other stages ( P = .039). The 5-year survival rate was 46% for patients having T3-T4 operable cancers treated by surgery vs 45% for patients treated by laryngeal conservation protocol (not significant). The 5-year survival rate for patients having nonoperable T4 cancers was 17.2%. The 3- and 5-year overall survival rates of N0 patients was significantly higher than N1 patients ( P = .042). N2 and N3 patients had 100% 5-year mortality. Conclusion This study showed that overall survival and therapeutic management depend on the initial stage of pyriform sinus cancer, notably on the N status. In particular, nonoperable T4 pyriform sinus cancer and N2 and N3 patients had a very poor prognosis. A laryngeal conservation protocol seemed as effective as surgical management in terms of survival.


2019 ◽  
Vol 4 (6) ◽  
pp. e001889
Author(s):  
Ussamah El-khani ◽  
Hutan Ashrafian ◽  
Shahnawaz Rasheed ◽  
Harald Veen ◽  
Ammar Darwish ◽  
...  

IntroductionDisaster zone medical relief has been criticised for poor quality care, lack of standardisation and accountability. Traditional patient safety practices of emergency medical teams (EMTs) in disaster zones were not well understood. Improving the quality of healthcare in disaster zones has gained importance within global health policy. Ascertaining patient safety practices of EMTs in disaster zones may identify areas of practice that can be improved.MethodsA systematic search of OvidSP, Embase and Medline databases; key journals of interest; key grey literature texts; the databases of the WHO, Médecins Sans Frontieres and the International Committee of the Red Cross; and Google Scholar was performed. Descriptive studies, case reports, case series, prospective trials and opinion pieces were included with no limitation on date or language of publication.ResultsThere were 9685 records, evenly distributed between the peer-reviewed and grey literature. Of these, 30 studies and 9 grey literature texts met the inclusion criteria and underwent qualitative synthesis. From these articles, 302 patient safety statements were extracted. Thematic analysis categorised these statements into 84 themes (total frequency 632). The most frequent themes were limb injury (9%), medical records (5.4%), surgery decision-making (4.6%), medicines safety (4.4%) and protocol (4.4%).ConclusionPatient safety practices of EMTs in disaster zones are weighted toward acute clinical care, particularly surgery. The management of non-communicable disease is under-represented. There is widespread recognition of the need to improve medical record-keeping. High-quality data and institutional level patient safety practices are lacking. There is no consensus on disaster zone-specific performance indicators. These deficiencies represent opportunities to improve patient safety in disaster zones.


2020 ◽  
Vol 3 (2) ◽  
pp. 136-141
Author(s):  
S.A. Boykov ◽  
◽  
I.Yu. Chernyak ◽  
N.S. Shatokhina ◽  
E.Yu. Gurkina ◽  
...  

Hypophosphatasia (HPP) is a rare multisystem inherited metabolic disorder caused by mutations in ALPL gene that encodes tissue nonspecific alkaline phosphatase responsible for bone mineralization. HPP is characterized by impaired bone mineralization, skeletal abnormalities, and systemic manifestations which result in significant morbidity and mortality. Clinical presentations of HPP vary greatly. Early (perinatal and infantile) HPP is characterized by the most severe symptoms, i.e., respiratory and neurological disorders are of crucial importance being the leading causes of death. Progressive skeletal impairment, rickets-like deformities, reduced mobility, and severe disability are typical of childhood-onset HPP. The biochemical hallmark of HPP is low alkaline phosphatase (ALP) activity. HPP diagnosis is verified by clinical symptoms in combination with persistently low ALP activity (adjusted for age and sex). Molecular genetic test to identify ALPL gene mutation is performed as needed. Three case reports addresses authors’ experience with the diagnosis and treatment for HPP.Keywords: hypophosphatasia, case series, alkaline phosphatase, impaired bone mineralization, asfotase alfa.For citation: Boykov S.A., Chernyak I.Yu., Shatokhina N.S. et al. Hypophosphatasia in children. Three faces of one disease. Russian Journal of Woman and Child Health. 2020;3(2):136–141. DOI: 10.32364/2618-8430-2020-3-2-136-141.


2015 ◽  
Vol 123 (3) ◽  
pp. 808-812 ◽  
Author(s):  
Avetis Azizyan ◽  
Joseph M. Miller ◽  
Ramzi I. Azzam ◽  
Marcel M. Maya ◽  
Pouyan Famini ◽  
...  

OBJECT Pituitary apoplexy is a rare and potentially life-threatening disorder that is most commonly characterized by a combination of sudden headache, visual disturbance, and hypothalamic/hormonal dysfunction. In many cases, there is hemorrhagic infarction of an underlying pituitary adenoma. The resulting clinical symptoms are due to compression of the remaining pituitary, cavernous sinuses, or cranial nerves. However, there are only 2 case reports in the literature describing spontaneous retroclival expansion of hemorrhage secondary to pituitary apoplexy. Ten cases of this entity with a review of the literature are presented here. METHODS This is a single-institution retrospective review of 2598 patients with sellar and parasellar masses during the 10-year period between 1999 and 2009. The pituitary and brain MRI and MRI studies were reviewed by 2 neuroradiologists for evidence of apoplexy, with particular attention given to retroclival extension. RESULTS Eighteen patients (13 men and 5 women; mean age 54 years) were identified with presenting symptoms of sudden onset of headache and ophthalmoplegia, and laboratory findings consistent with pituitary apoplexy. Ten of these patients (8 men and 2 women; mean age 55 years) had imaging findings consistent with retroclival hematoma. CONCLUSIONS Retroclival hemorrhage was seen in the majority of cases of pituitary apoplexy (56%), suggesting that it is more common than previously thought.


Cancers ◽  
2019 ◽  
Vol 11 (8) ◽  
pp. 1180
Author(s):  
Masafumi Kanno ◽  
Norihiko Narita ◽  
Yasushi Fujimoto ◽  
Naohiro Wakisaka ◽  
Tomokazu Yoshizaki ◽  
...  

The present study aimed to clarify the incidence and clinical outcomes of nasopharyngeal carcinoma (NPC) in the Chubu region of Japan from 2006 to 2015, compared with previous reports. A retrospective analysis was conducted based on medical records from 40 hospitals located in the Chubu region in the central Japanese main island, with a population of around 22.66 million individuals. This study was designed in line with to two previous clinical studies into NPC conducted in the same area of Japan. We recruited NPC patients diagnosed in hospitals across this area over a 10-year period (2006–2015) using a questionnaire about sex, age, primary site, clinical symptoms, pathology, Union for International Cancer Control (UICC) staging, serological exam, treatment, and survival. A total of 620 NPC patients were identified. The age-standardized incidence of NPC from 2006 to 2015 was 0.27 per 100,000 individuals per year. There were no significant differences between this study and the previous two studies conducted in the same area of Japan. The five-year overall survival rate for all patients was 75.9%, while those for patients with stages I, II, III, and IVA were 97%, 91%, 79%, and 68%, respectively. The age-standardized annual incidence of NPC in the present study was 0.27 per 100,000 individuals per year, which was relatively low and stable. The five-year overall survival rate for all NPC patients was significantly improved in this decade compared with previous studies. The smoking rates in male and female NPC patients were 64.5% and 18.8%, respectively, thereby suggesting the involvement of smoking in the incidence of NPC.


Materials ◽  
2021 ◽  
Vol 14 (17) ◽  
pp. 5058
Author(s):  
William Banh ◽  
Jared Hughes ◽  
Aaron Sia ◽  
David C. H. Chien ◽  
Santosh K. Tadakamadla ◽  
...  

The purpose of this study was to systematically review the existing literature to assess the clinical survival and success of PICN and ZLS indirect restorations as the clinical evidence for them remains lacking. PubMed, SCOPUS, Embase, Cochrane Library, Web of Science, LILACs, and SciElo databases were searched from 1 January 2000 to 1 February 2021. Clinical trials and cohort studies published in English were included while case studies, case series, and in vitro studies were excluded. Results were analyzed qualitatively and a meta-analysis using a random effects model was performed. A strength of recommendation taxonomy (SORT) analysis was conducted and risk of bias (RoB) was assessed using the Newcastle–Ottawa scale and Cochrane RoB tools. An electronic search through the databases yielded 2454 articles, of which 825 remained after duplicate removal. Five studies investigating PICN and four investigating ZLS indirect restorations remained after assessing for eligibility. The overall survival rate of PICN over 1 year was 99.6% and 99.2% over 2 years. The overall survival rate of ZLS over 1 year was 99%. The main mode of failure for both materials was catastrophic fracture. One study had a high RoB, four had a moderate RoB, and four had a low RoB. Both materials demonstrated moderate strength of recommendation at a level 2 evidence for all studies based on SORT analysis. PICN and ZLS show promising short-term clinical performance as full and partial coverage indirect restorations, but longer follow-up studies are required to confirm their long-term performance.


2015 ◽  
Vol 2015 ◽  
pp. 1-5 ◽  
Author(s):  
Kimihiro Igari ◽  
Toshifumi Kudo ◽  
Takahiro Toyofuku ◽  
Yoshinori Inoue

Profunda femoris artery aneurysm (PFAA) is an extremely rare entity, with most cases being asymptomatic, which makes obtaining an early diagnosis difficult. We herein report a case series of PFAA, in which more than half of the PFAAs, which presented with no clinical symptoms, were discovered incidentally. All PFAAs were treated surgically with aneurysmectomy with or without vascular reconstruction. In cases involving a patent superficial femoral artery (SFA), graft replacement of the profunda femoris artery (PFA) is not mandatory; however, preserving the blood flow of the PFA is necessary to maintain lower extremity perfusion in patients with occlusion of the SFA. Therefore, the treatment of PFAAs should include appropriate management of both the aneurysmectomy and graft replacement, if possible.


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