Craniopharyngioma

2019 ◽  
pp. 207-216
Author(s):  
Lissa C. Baird

Pediatric craniopharyngioma is a histologically benign neoplasm with potential for malignant clinical behavior. Presenting vision and endocrine deficits are common, and the natural history carries unacceptable morbidity, including blindness, complete hypothalamic and pituitary dysfunction, and death. The goal of initial treatment for craniopharyngioma in the pediatric population should be permanent tumor control or cure in order to minimize morbidity from recurrent and progressive disease. Treatment-related morbidity is acute with surgical intervention and requires immediate postoperative endocrinological care. Radiotherapy is associated with delayed treatment morbidity, and long-term monitoring is critical. Inadequate tumor control after initial treatment will result in inevitable recurrence, with more complex and less effective treatment options and higher associated treatment morbidity. Unexpected intraoperative complications can be avoided with careful preoperative planning and good surgical technique.

2018 ◽  
Vol 2018 ◽  
pp. 1-6 ◽  
Author(s):  
Kannan NB ◽  
Piyush Kohli ◽  
Bhanu Pratap Singh Pangtey ◽  
Kim Ramasamy

Aim. This paper aims at evaluating refractive outcome and complication profile of sutureless, glueless, flapless, intrascleral fixation of intraocular lens (SFIOL) in pediatric population. Methods. This retrospective study included patients ≤18 years of age who underwent SFIOL for ectopia lentis. Details obtained included preoperative uncorrected visual acuity (UCVA), cycloplegic refraction, and best-corrected visual acuity (BCVA); intraoperative complications; and postoperative UCVA, cycloplegic refraction, and BCVA and complications. Results. Median pre- and postoperative UCVA was logMAR 1.78 (Snellen 20/1200) and logMAR 0.30 (Snellen 20/40), respectively, (p<0.001). Median pre- and postoperative BCVA was logMAR 0.24 (Snellen 20/34) and logMAR 0.18 (Snellen 20/30), respectively. UCVA ≥20/60 was attained in 90% of eyes. BCVA ≥20/30 was attained in 85.0% of eyes. Most common early postoperative complications were hyphaema (10%), transient vitreous hemorrhage (2.5%), and ocular hypotony (2.5%). None of these developed any long-term sequelae. Only one case of subluxation of IOL was seen. No case of late endophthalmitis or retinal detachment was seen. Conclusion. Since refractive error induced is minimal, the procedure is suitable for IOL implantation in children, who are noncompliant with spectacles. The complication profile is similar to that reported in adults.


Blood ◽  
2020 ◽  
Vol 136 (16) ◽  
pp. 1803-1812 ◽  
Author(s):  
Stephen P. Hunger ◽  
Elizabeth A. Raetz

Abstract Relapsed acute lymphoblastic leukemia (ALL) has remained challenging to treat in children, with survival rates lagging well behind those observed at initial diagnosis. Although there have been some improvements in outcomes over the past few decades, only ∼50% of children with first relapse of ALL survive long term, and outcomes are much worse with second or later relapses. Recurrences that occur within 3 years of diagnosis and any T-ALL relapses are particularly difficult to salvage. Until recently, treatment options were limited to intensive cytotoxic chemotherapy with or without site-directed radiotherapy and allogeneic hematopoietic stem cell transplantation (HSCT). In the past decade, several promising immunotherapeutics have been developed, changing the treatment landscape for children with relapsed ALL. Current research in this field is focusing on how to best incorporate immunotherapeutics into salvage regimens and investigate long-term survival and side effects, and when these might replace HSCT. As more knowledge is gained about the biology of relapse through comprehensive genomic profiling, incorporation of molecularly targeted therapies is another area of active investigation. These advances in treatment offer real promise for less toxic and more effective therapy for children with relapsed ALL, and we present several cases highlighting contemporary treatment decision-making.


2014 ◽  
Vol 2014 ◽  
pp. 1-10 ◽  
Author(s):  
Vítor M. Gonçalves ◽  
Álvaro Lima ◽  
João Gíria ◽  
Nuno Carvalho ◽  
José Parreira ◽  
...  

Sacral giant cell tumors are rare neoplasms, histologically benign but potentially very aggressive due to the difficulty in achieving a complete resection, their high recurrence rate, and metastization capability. Although many treatment options have been proposed,en blocexcision with tumor-free margins seems to be the most effective, being associated with long term tumor control, improved outcome, and potential cure. An exemplifying case of a 29-year-old female with progressive complaints of pain and paresthesias in the sacral and perianal regions, constipation, and weight loss for 6 months is presented. The surgical technique foren blocexcision of a large sacral giant cell tumor through a modified Kraske procedure with mid-sacrectomy and coccygectomy is described. Complete resection with wide tumor-free margins was achieved. At 5 years of follow-up the patient is neurologically intact, without evidence of local recurrence on imaging studies. A multidisciplinary surgical procedure is mandatory to completely remove sacral tumors. In the particular case of giant cell tumors, it allows minimizing local recurrence preserving neurovascular function, through a single dorsal and definitive approach.


2007 ◽  
Vol 107 (4) ◽  
pp. 745-751 ◽  
Author(s):  
Toshinori Hasegawa ◽  
Yoshihisa Kida ◽  
Masayuki Yoshimoto ◽  
Joji Koike ◽  
Hiroshi Iizuka ◽  
...  

Object The aim of this study was to evaluate long-term outcomes, including tumor control and neurological function, in patients with cavernous sinus meningiomas treated using Gamma Knife surgery (GKS). Methods One hundred fifteen patients with cavernous sinus meningiomas, excluding atypical or malignant meningiomas, were treated with GKS between 1991 and 2003. Forty-nine patients (43%) underwent GKS as the initial treatment. The mean tumor volume was 14 cm3, and the mean maximum and margin doses applied to the tumor were 27 and 13 Gy, respectively. The median follow-up period was 62 months. During the follow-up, 111 patients were able to be evaluated with neuroimaging. Results The actuarial 5- and 10-year progression-free survival rates were 87 and 73%, respectively. Similarly, the actuarial 5- and 10-year focal tumor control rates were 94 and 92%, respectively. Regarding functional outcomes, 43 patients (46%) experienced some degree of improvement, 40 (43%) remained stable, and 11 (12%) had worse preexisting or newly developed symptoms. Patients who underwent GKS as the initial treatment experienced significant improvement of their symptoms (p = 0.006). Conclusions Gamma Knife surgery is a safe and effective treatment over the long term in selected patients with cavernous sinus meningiomas. Tumor progression is more likely to occur from the lesion margin outside the treatment volume. In small to medium-sized tumors, GKS is an excellent alternative to resection, preserving good neurological function. For relatively large-sized tumors, low-dose radiosurgery (≤ 12 Gy) is acceptable for the prevention of tumor progression.


Author(s):  
Maciej Szabłowski ◽  
Michał Andrzej Okruszko ◽  
Katarzyna Pochodowicz ◽  
Paweł Abramowicz ◽  
Jerzy Konstantynowicz ◽  
...  

AbstractThe study was aimed to review a rare coexistence of type 1 diabetes (T1D) and juvenile idiopathic arthritis (JIA) regarding different clinical approaches to the management and treatment options. Medical complications of the two autoimmune disorders in children and adolescents have been evaluated, particularly in those treated with glucocorticosteroids (GCS) and insulin. A review of the literature regarding reports on concomitant T1D and JIA was conducted using resources available in Medline, Google Scholar, and Web of Science databases, with a specific focus on the combination of T1D and JIA in a pediatric population. The review was extended by our analysis of two patients treated in a single center for this comorbidity. Eligible reports of four cases were found, and including our two original records, a total of six pediatric patients (5 females) were analyzed, of which three had also other autoimmune diseases (thyroiditis, coeliac disease, autoimmune hepatitis), whereas four had been treated with a long-term GCS, and two were receiving biological therapy (etanercept or adalimumab). Only one of them had good metabolic control of diabetes. Diabetes in childhood may coexist with other autoimmune diseases, including rheumatologic conditions. Hyperglycemia can worsen JIA therapy by induction and maintaining inflammation. Using modern diabetes technologies (like personal insulin pumps and continuous glucose monitoring) helps to minimize the deteriorating effect of JIA exacerbations and the rheumatoid treatment on metabolic control of diabetes.


Author(s):  
Andrea K. Erickson ◽  
Penny J. Regier ◽  
Meghan M. Watt ◽  
Kathleen M. Ham ◽  
Sarah J. Marvel ◽  
...  

Abstract OBJECTIVE To evaluate outcomes of dogs with parathyroid carcinoma (PTC) treated by surgical excision and to describe the incidence of postoperative hypocalcemia, degree of hypocalcemia, duration of hospitalization, duration of calcium supplementation, and survival time ANIMALS 100 client-owned dogs with PTC admitted to academic, referral veterinary institutions. PROCEDURES In a retrospective multi-institutional study, medical records of dogs undergoing surgical excision of PTC between 2010 to 2019 were reviewed. Signalment, relevant medical history, clinical signs, clinicopathologic testing, imaging, surgical findings, intraoperative complications, histologic examination, and survival time were recorded. RESULTS 100 dogs with PTC were included, and 96 dogs had clinical or incidental hypercalcemia. Common clinical signs included polyuria (44%), polydipsia (43%), hind limb paresis (22%), lethargy (21%), and hyporexia (20%). Cervical ultrasonography detected a parathyroid nodule in 91 of 91 dogs, with a single nodule in 70.3% (64/91), 2 nodules in 25.3% (23/91), and ≥ 3 nodules in 4 (4/91)% of dogs. Hypercalcemia resolved in 89 of 96 dogs within 7 days after surgery. Thirty-four percent of dogs developed hypocalcemia, on the basis of individual analyzer ranges, within 1 week after surgery. One dog had metastatic PTC to the prescapular lymph node, and 3 dogs were euthanized for refractory postoperative hypocalcemia. Estimated 1-, 2-, and 3-year survival rates were 84%, 65%, and 51% respectively, with a median survival time of 2 years. CONCLUSIONS AND CLINICAL RELEVANCE Excision of PTC results in resolution of hypercalcemia and excellent long-term tumor control. Surgical excision of PTC is recommended because of resolution of hypercalcemia and a good long-term prognosis. Future prospective studies and long-term follow-up are needed to further assess primary tumor recurrence, metastasis, and incidence of postoperative hypocalcemia.


2019 ◽  
Author(s):  
D Kohoutova ◽  
A Tringali ◽  
G Paparella ◽  
V Perri ◽  
I Boškoski ◽  
...  

2015 ◽  
Vol 156 (45) ◽  
pp. 1824-1833 ◽  
Author(s):  
Árpád Illés ◽  
Ádám Jóna ◽  
Zsófia Simon ◽  
Miklós Udvardy ◽  
Zsófia Miltényi

Introduction: Hodgkin lymphoma is a curable lymphoma with an 80–90% long-term survival, however, 30% of the patients develop relapse. Only half of relapsed patients can be cured with autologous stem cell transplantation. Aim: The aim of the authors was to analyze survival rates and incidence of relapses among Hodgkin lymphoma patients who were treated between January 1, 1980 and December 31, 2014. Novel therapeutic options are also summarized. Method: Retrospective analysis of data was performed. Results: A total of 715 patients were treated (382 men and 333 women; median age at the time of diagnosis was 38 years). During the studied period the frequency of relapsed patients was reduced from 24.87% to 8.04%. The numbers of autologous stem cell transplantations was increased among refracter/relapsed patients, and 75% of the patients underwent transplantation since 2000. The 5-year overall survival improved significantly (between 1980 and 1989 64.4%, between 1990 and 1999 82.4%, between 2000 and 2009 88.4%, and between 2010 and 2014 87.1%). Relapse-free survival did not change significantly. Conclusions: During the study period treatment outcomes improved. For relapsed/refractory Hodgkin lymphoma patients novel treatment options may offer better chance for cure. Orv. Hetil., 2015, 156(45), 1824–1833.


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