Cranial MRI abnormalities and long-term follow-up of the lesions in 770 girls with Central Precocious Puberty

2021 ◽  
Author(s):  
Didem Helvacıoğlu ◽  
Serap Demircioğlu Turan ◽  
Tülay Güran ◽  
Zeynep Atay ◽  
Adnan Dağçınar ◽  
...  
Keyword(s):  
Precocious Puberty ◽  
Central Precocious Puberty ◽  
Final Decision ◽  
Low Grade ◽  
Long Term Outcome ◽  
Neoplastic Lesions ◽  
Cranial Mri ◽  
Cns Lesions

Abstract Context Central precocious puberty(CPP) may arise from central nervous system(CNS) lesions in a few affected girls. Recently, the incidence of girls with CPP has increased mostly in 6-8 year-olds, in whom the necessity of magnetic resonance imaging(MRI) is debated. Objective To investigate the frequency, long-term outcome and potential predictors of CNS lesions in a large cohort of girls with CPP. Design and Method A multi-center cohort of 770 Turkish girls with CPP who had systematic cranial MRI between 2005-2017. Age at puberty onset was < 6 years in 116 and 6-8 years in 654. CNS lesions were followed until final decision(6.2±3.1years). Potential predictors of CNS lesions were evaluated by univariate analyses. Results 104/770(13.5%) girls had abnormal brain MRI. Of these, 2.8% were previously known CNS lesions, 3.8% had newly detected and causally related CNS lesions, 3.1 % were possibly related and 3.8% were incidental. Only two(0.25%) neoplastic lesions (one low grade glioma and one meningioma) were identified; neither required intervention over follow-up of 6 and 3.5 years respectively. Age at breast developmen <6 years [OR(95%CI); 2.38(1.08-5.21)] and the peak LH/FSH ratio >0.6 [OR(95%CI); 3.13 (1.02-9.68)] were significantly associated with CNS lesions. However, both patients with neoplastic lesions were >6 years old. Conclusion Although age and LH/FSH ratio are significant predictors of CNS lesions, their predictive power is weak. Thus, systematic MRI seems to be the most efficient current approach to avoid missing an occult CNS lesion in girls with CPP, despite the low likelihood of finding a lesion requiring intervention.

Anthropometric, metabolic, and reproductive outcomes of patients with central precocious puberty treated with leuprorelin acetate 3-month depot (11.25 mg)

2021 ◽  
Vol 0 (0) ◽  
Author(s):  
Carolina O. Ramos ◽  
Ana P M Canton ◽  
Carlos Eduardo Seraphim ◽  
Aline Guimarães Faria ◽  
Flavia Rezende Tinano ◽  
...  
Keyword(s):  
Precocious Puberty ◽  
Adult Height ◽  
Central Precocious Puberty ◽  
Bone Age ◽  
Overweight And Obesity ◽  
Leuprorelin Acetate ◽  
The Mean ◽  
Hormone Analogs

Abstract Objectives Longer-acting gonadotropin-releasing hormone analogs (GnRHa) have been widely used for central precocious puberty (CPP) treatment. However, the follow-up of patients after this treatment are still scarce. Our aim was to describe anthropometric, metabolic, and reproductive follow-up of CPP patients after treatment with leuprorelin acetate 3-month depot (11.25 mg). Methods Twenty-two female patients with idiopathic CPP were treated with leuprorelin acetate 3-month depot (11.25 mg). Their medical records were retrospectively evaluated regarding clinical, hormonal, and imaging aspects before, during, and after GnRHa treatment until adult height (AH). Results At the diagnosis of CPP, the mean chronological age (CA) was 8.2 ± 1.13 year, and mean bone age (BA) was 10.4 ± 1.4 year. Mean height SDS at the start and the end of GnRHa treatment was 1.6 ± 0.8 and 1.3 ± 0.9, respectively. The mean duration of GnRHa treatment was 2.8 ± 0.8 year. Mean predicted adult heights (PAH) at the start and the end of GnRH treatment was 153.2 ± 8.6 and 164.4 ± 7.3 cm, respectively (p<0.05). The mean AH was 163.2 ± 6.2 cm (mean SDS: 0.1 ± 1). All patients were within their target height (TH) range. There was a decrease in the percentage of overweight and obesity from the diagnosis until AH (39–19% p>0.05). At the AH, the insulin resistance and high LDL levels were identified in 3/17 patients (17.6%) and 2/21 patients (9.5%), respectively. The mean CA of menarche was 12.2 ± 0.5 years. At the AH, PCOS was diagnosed in one patient (4.8%). Conclusions Long-term anthropometric, metabolic, and reproductive follow-up of patients with CPP treated with longer-acting GnRHa revealed effectivity, safety, and favorable outcomes.

Long-term outcome of hypothalamic/chiasmatic astrocytomas in children treated with conservative surgery

1995 ◽  
Vol 83 (4) ◽  
pp. 583-589 ◽  
Author(s):  
Leslie N. Sutton ◽  
Patricia T. Molloy ◽  
Heidi Sernyak ◽  
Joel Goldwein ◽  
Peter L. Phillips ◽  
...  
Keyword(s):  
Radical Surgery ◽  
Conservative Surgery ◽  
Low Grade ◽  
Term Outcome ◽  
Term Survival ◽  
Long Term Outcome ◽  
Content Type ◽  
Fine Print

✓ The feasibility of radical surgery for astrocytomas of the optic chiasm/hypothalamus has been reported by several groups. Such surgery carries significant risks, however, including permanent damage to the pituitary gland, optic apparatus, hypothalamic structures, and carotid arteries. The benefits of radical surgery, both in terms of efficacy and toxicity, should, therefore, be evaluated against standard therapy, as is usually done for new chemotherapeutic protocols. To this end, a retrospective review was performed of 33 patients treated at Children's Hospital of Philadelphia between 1976 and 1991 who met criteria that would have made them eligible for radical surgery in many centers today, but were treated with either no surgery or conservative surgery (< 50% resection) or biopsy followed by adjuvant therapy with local radiation therapy (29 patients) and/or chemotherapy with actinomycin-D and vincristine (18 patients). The review encompassed all children with a globular enhancing mass of at least 2 cm in the hypothalamic/chiasmatic region, no evidence of optic nerve involvement or involvement of the optic radiations by computerized tomography or magnetic resonance imaging, and follow up of at least 3 years. All but one patient had tissue confirmation of a low-grade or pilocytic astrocytoma. Thirteen of the patients were 2 years of age or younger at diagnosis. Five individuals died: three of tumor progression, one of acute shunt malfunction, and one of intercurrent infection. The remaining 28 were alive at last follow up, a mean of 10.9 years from diagnosis. Twenty-three surviving patients have functional vision in at least one eye, 12 require no endocrine replacement, and 16 are in or have completed schooling with regular academic requirements. If radical surgery is to become standard care for children with low-grade astrocytomas of the hypothalamic/chiasmatic region, long-term survival and functional outcome will have to equal or surpass those of historical controls who were treated conservatively.

Hypothalamic hamartoma simulating a suprasellar arachnoid cyst: resolution of precocious puberty following microsurgical lesion resection

2014 ◽  
Vol 14 (1) ◽  
pp. 101-107 ◽  
Author(s):  
Sunil Manjila ◽  
Timothy W. Vogel ◽  
Yunwei Chen ◽  
Mark S. Rodgers ◽  
Alan R. Cohen
Keyword(s):  
Arachnoid Cyst ◽  
Precocious Puberty ◽  
Central Precocious Puberty ◽  
Hypothalamic Hamartoma ◽  
Suprasellar Region ◽  
Cystic Changes ◽  
Long Term Follow Up ◽  
Suprasellar Arachnoid Cyst

Hypothalamic hamartomas (HHs) are rare developmental lesions arising from the inferior hypothalamus that may cause gelastic seizures and central precocious puberty. Cystic changes in HHs are rare, usually occurring in giant lesions. The authors describe an unusual case of cystic HH masquerading as a suprasellar arachnoid cyst in an 18-month-old girl presenting with precocious puberty. Microsurgical removal of the lesion led to complete resolution of the precocious puberty on long-term follow-up. This case is the first reported HH with pathological demonstration of corticotropin-releasing hormone immunostaining in the solid tumor and glial cells in the cyst wall of the lesion. The clinical and radiological characteristics of HHs are reviewed, along with the unique surgical strategies used to manage cystic lesions in the suprasellar region.

scholarly journals Long-Term Outcome of Patients with Marginal Zone Non-Hodgkin Lymphoma (MZL) Treated with Yttrium-90 Ibritumomab Tiuxetan: The Mayo Clinic Experience

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 1544-1544
Author(s):  
Muhamad Alhaj Moustafa ◽  
Ricardo Parrondo ◽  
Gregory Wiseman ◽  
Jennifer Peterson ◽  
Thomas E. Witzig ◽  
...  
Keyword(s):  
Median Time ◽  
Research Funding ◽  
Low Grade ◽  
Ibritumomab Tiuxetan ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Grade 3 ◽  
Yttrium 90

Background MZL is a low-grade non-Hodgkin's lymphoma (NHL) which involves lymph nodes, extranodal sites, or spleen. It is sensitive to radiation therapy, which is used in localized disease with curative intent. Yttrium-90 ibritumomab tiuxetan [(90)Y-IT; Zevalin] is a radio-immunoconjugate (RIC) that targets CD20. It is approved for relapsed/refractory low grade and follicular NHL. The data on its use in MZL is limited. We present long-term outcome of the largest reported cohort of MZL patients who received (90)Y-IT. Methods Medical records of patients who received treatment with (90)Y-IT at Mayo Clinic Cancer Center between January 2004 and December 2018 were analyzed. We selected patients with MZL and reviewed clinical data including age, gender, MZL type, clinical stage (Ann Arbor Staging System), treatment response, (90)Y-IT related adverse effects (AEs), as well as lymphoma and treatment related events. All patients received (90)Y-IT according to the standard treatment guidelines. Overall response rate (ORR) and complete response rate (CR) were calculated. Progression-free survival (PFS), time to next therapy (TTNT), and overall survival (OS) were analyzed using the Kaplan-Meier method. Results Twenty-one patients were identified (Table 1). The median age at diagnosis was 60 years (range, 11-81) and 71% (15/21) were female. 52% (11/21) were previously-untreated (UMZL) while 48% (10/21) were relapsed (RMZL). The median number of pretreatments in RMZL patients was 2 (range, 1-3). ECOG performance status at the time of treatment was 0 in 90% (19/21) and 1 in 10% (2/21). 62% (13/21) were stage III/IV disease at the time of (90)Y-IT therapy. The median follow-up was 8.5 years (95% CI; 4.5, 12.4); 17 (81%) patients remain alive. The ORR was 91% (19/21) with the two non-responders being in the RMZL group. The CR rate was 81% (17/21) and 65% (11/17) remain in CR at a median follow-up of 5.7 years (95% CI; 1.4, 11). Nine (43%) patients had a relapse during the study period. More relapses occurred in the RMZL group (7/10; 70%) compared to (2/11; 18%) in the UMZL group. Median PFS (whole cohort) was 10 years (95% CI; 2.1, NR) and TTNT (whole cohort) was not reached (NR) (95% CI; 2.1 years, NR). Median PFS was significantly higher in UMZL group compared to RMZL group NR (95% CI; 2.5 years, NR) vs 2.1 years (95% CI; 0.17, 9.9), respectively (Figure 1-A).Median OS (whole cohort) was 19.3 years (95% CI; 8.9, 19.3) without statistical difference in between UMZL group and RMZL group NR (95% CI; NR, NR) vs 16.6 years (95% CI; 9, 19.4), respectively (Figure 1-B). None of the 11 UMZL patients died at median follow up of 4.7 years (95% CI; 1.6, 9.2). All 4 deaths were in the RMZL group with 3 dying of transformation to high-grade lymphoma at 8, 22, and 25 months post-(90)Y-IT treatment. One patient died of myelodysplastic syndrome 7.3 years post-(90)Y-IT treatment while in CR. Toxicities were primarily hematologic. Grade ³3 neutropenia was observed in 6/21 (29%) patients with median time to nadir of 48.5 days (range, 19-70) and median time to recovery to normal absolute neutrophil count of 39.5 days (range, 7-476). Grade ³3 thrombocytopenia was observed in 3 (14%) patients with median time to nadir of 35 days (range, 19-357) and median time to recovery of 21 days (range, 2-538). Grade ³3 anemia was observed in only one patient. Only two patients required transfusions and growth factor support. Non-hematologic AEs included mild to severe fatigue in 4 patients. Conclusion RIC with (90)Y-IT is efficacious and well-tolerated in patients with previously untreated as well as relapsed MZL. As expected it appears to be more efficacious in previously untreated patients. Long-term complete remission (&gt;5 years) was observed in 52% of the study population (43% of UMZL and 9% of RMZL). Combination of efficacy, tolerability, and treatment schedule most convenient for patients makes (90)Y-IT a reasonable alternative to systemic therapy with immunotherapy, chemotherapy, or chemo-immunotherapy in management of MZL. Figure 1: (A) Progression-free survival; comparing time to progression or death after (90)Y-IT treatment between previously untreated patients (UMZL) and patients with relapsed MZL (RMZL), (B) Overall survival; comparing time to death from all causes after (90)Y-IT treatment between UMZL patients and RMZL patients. Disclosures Tun: Curis: Research Funding; TG Therapeutics: Research Funding; BMS: Research Funding; DTRM Biopharma: Research Funding; Celgene: Research Funding; Mundi-pharma: Research Funding. OffLabel Disclosure: The use of Yttrium-90 ibritumomab tiuxetan as a first line treatment for marginal zone lymphoma

xxx

2007 ◽  
Vol 25 (18_suppl) ◽  
pp. 8123-8123
Author(s):  
C. Tarella ◽  
M. Zanni ◽  
A. Rambaldi ◽  
F. Benedetti ◽  
R. Passera ◽  
...  
Keyword(s):  
Complete Remission ◽  
High Dose ◽  
Low Grade ◽  
High Grade ◽  
Term Outcome ◽  
Term Survival ◽  
Long Term Outcome ◽  
Long Term Survival

8123 Background: The high-dose sequential (HDS) chemotherapy approach, including early dose-intensification and autograft with peripheral blood progenitor cells (PBPC), was introduced several years ago (Gianni & Bonadonna, 1989); subsequently, it has been broadly used in the management of both non-Hodgkin s (NHL) and Hodgkin s Lymphoma (HL). The outcome of a large series of lymphoma patients treated with the HDS approach at 10 GITIL Centers is reported. Methods: Data have been collected on 1,266 patients, who received either the original or slightly modified HDS regimens. There were 213 HL and 1,053 NHL (630 intermediate/high-grade, 423 low-grade); median age was 46 yrs. Overall, 671 (53%) patients had refractory/relapsed disease, 595 (47%) were at diagnosis. Most patients were autografted with PBPC; 158 (12%) patients did not undergo autografting due to toxicity, disease progression or poor harvests. Results: Overall, 1,013 (80%) patients reached Complete Remission (CR) following HDS. As to December 2006, 93 (7%) patients died for early/late toxicities, 328 (26%) died for lymphoma, 844 are known to be alive. At a lead follow-up of 18 years, and a median follow-up of 5 yrs, the 5-yr Overall Survival (OS) projection is 64% (S.E.: 2%). The long-term survival was quite favorable in patients achieving a Complete Remission (CR), with a 5-yr OS projection of 76%. The prolonged OS in patients achieving CR was consistent in all lymphoma subtypes, i.e. both low and high-grade NHL (5-yr OS: 77% in both), and HL (5-yr OS: 72%). Patients at diagnosis had a significantly better outcome compared to patients treated for relapsed/refractory disease, again CR achievement was associated with prolonged survival in both subgroups (82% and 69%, respectively, at 5 yrs.). On multivariate Cox survival analysis, CR achievement was the most powerful predictor of long-term survival (HR 0.13, c.i.: 0.10–0.17). Lastly, achieving substantial tumor reduction before autografting had a major influence on the clinical outcome. Conclusions: 1. the HDS program is feasible in a multicenter setting; 2. the long-term outcome is well influenced by the CR status after HDS; 3. the influence of CR achievement on the long-term survival holds true in all lymphoma subtypes, including indolent lymphomas; 4. an adequate pre-autograft tumor debulking may contribute to a favorable long-term outcome. [Table: see text]

Outcome and Long Term Toxicity of the LNH- PRO Trial after 12 Year Median Follow-up, in Patients with Indolent Non Hodgkin's Lymphoma Who Received Immunochemotherapy with CVP and Interferon-alfa2b (IFNα2b)

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4465-4465 ◽  
Author(s):  
Jimena Cannata-Ortiz ◽  
Concepción Nicolás ◽  
Ana García-Noblejas ◽  
Javier Lopez ◽  
Pilar Sabin ◽  
...  
Keyword(s):  
Bone Marrow Involvement ◽  
Long Term Results ◽  
Low Grade ◽  
Secondary Malignancies ◽  
Bulky Disease ◽  
Long Term Outcome ◽  
Number Of Patients ◽  
Number Of Cycles

Abstract Introduction: Indolent B cell non-Hodgkin lymphomas are entities without curative treatment nowadays. However, survival has significantly improved since the incorporation of immunomodulatory agents and now immunochemotherapy has become the gold standard. Most treatment strategies use progression free survival (PFS) as a surrogate marker for overall survival (OS), although updated long term results are frequently lacking. Since 1990 our group introduced IFNα-2b to Bagley’s CVP induction regimen, for naïve indolent NHL (LNH-pro study). Herein we report our long term results. Aim: To evaluate long term outcome and late toxicities of patients who received immunochemotherapy with IFN α-2b plus CVP. Patients and Methods: From February 1990 to November 2001, patients from 7 Spanish institutions were included. Induction therapy consisted of Cyclophosphamide (400 mgs/m2 po) and Prednisone (100 mg/m2 po) daily for 5 days, Vincristine (1.4mg/m2 iv) on day 1, and subcutaneous IFN α-2b (3 MU/m2, three times a week, for a total of 36 doses). Patients received the number of cycles necessary to achieve maximum response. Updated clinical data were retrieved from participating centres up to March 2012. Results. A hundred and seventy patients with low-grade NHL were analyzed. Included entities were: 65% grade 1-2 follicular lymphoma (FL), 21% lymphocytic lymphoma and 14% marginal zone lymphoma. Median age was 56 yo (range 22-78 yo), elevated LDH and β2-microglobuline were 13.6% and 26% respectively, 57.6% had bone marrow involvement and 7.6% bulky disease (>7cm). According to FLIPI, 33% were high risk, 40% intermediate and 27% low risk FL. Median number of cycles was 6, and overall response rate achieved was 90%, with 68% complete remissions. Median follow up of surviving patients was 12.5 years (range 3-21 ys), with only 14.7% of patients lost to follow-up. Median PFS for all patients was 12.5 years (95% CI 10.5 – 14.5 years) and not reached for FL patients (20-year PFS of 63%; 95%CI: 54-72%). Median OS has not been reached, with a 20-year OS of 59.7% (CI 95%, 50.5-69%) for all low-grade NHL patients and 62% (IC 95%, 50-74%) for FL patients. Long-term toxicity is detailed in table 1. Incidence of secondary malignancies is 13.5%. At time of analysis, 57 out of 170 patients have died (33.5%), mainly due to lymphoma (58% of patients) and other non-lymphoma events (42%). Table Secondary malignancies 23 cases (13.5%) - MDS / AML 3 cases - Solid tumors 18 cases - Dermatologic neoplasia 2 cases Causes of death Number of patients (%) Induction toxicity events 4 (7%) Lymphoma progression / relapse 29 (51%) Secondary malignancies 9 (16%) Other non-lymphoma events 15 (26%) - Miocardiopathy 4 - Chronic Pulmonary disease 3 - Hepatic failure 2 - Brain traumatic injury 1 - Unknown cause 5 Figure 1 Figure 1. Conclusions: Our results confirm that immunochemotherapy with IFN α-2b plus CVP regimen induces a median PFS of 12.5 years and a 20-year OS of 59.7% (median not reached). With a median follow-up of 12.5 years, 58 % died due to lymphoma, 16% from secondary malignancies and 26% for non-lymphoma events. These results highlight the importance of performing long term follow-up in order to assess the real survival benefit of any treatment. Disclosures No relevant conflicts of interest to declare.

Stereotactic Brachytherapy With Iodine-125 Seeds for the Treatment of Inoperable Low-Grade Gliomas in Children: Long-Term Outcome

2011 ◽  
Vol 29 (31) ◽  
pp. 4151-4159 ◽  
Author(s):  
Maximilian I. Ruge ◽  
Thorsten Simon ◽  
Bogdana Suchorska ◽  
Ralph Lehrke ◽  
Christina Hamisch ◽  
...  
Keyword(s):  
Pediatric Patients ◽  
Tumor Volume ◽  
Local Treatment ◽  
Low Grade ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Stereotactic Brachytherapy ◽  
Iodine 125

Purpose Resection is generally considered the gold standard for treatment of low-grade (WHO grades I and II) gliomas (LGGs) in childhood. However, approximately 30% to 50% of these tumors are inoperable because of their localization in highly eloquent brain areas. A few reports have suggested stereotactic brachytherapy (SBT) with implantation of iodine-125 (125I) seeds as a safe and effective local treatment alternative. This single-center study provides a summary of the long-term outcome after SBT in one of the largest reported patient series. Patients and Methods All pediatric patients treated with SBT (125I seeds; cumulative therapeutic dose 50-65 Gy within 9 months) by our group for LGG with follow-up of more than 6 months were included. Clinical and radiologic outcome, time to progression, and overall survival were evaluated. Prognostic factors (age, sex, Karnofsky performance score, tumor volume, and histology) for survival and disease progression were investigated. Results In all, 147 of 160 pediatric patients treated with SBT (from 1982 through 2009) were analyzed in detail. Procedure-related mortality was zero, and the 30-day morbidity was transient and low (5.4%). Survival rates at 5 and 10 years were 93%, and 82%, respectively, with no significant difference between WHO grades I and II tumors (median follow-up, 67.1 ± 57.7 months). Twenty-one (14.8%) of 147 patients presented with tumor relapse. The remaining 126 patients revealed complete response in 24.6%, partial response in 31.0%, and stable disease in 29.6%. Neurologic status improved (57.8%) or remained stable (23.0%). None of the evaluated factors had significant impact on the study's end points except tumor volume more than 15 mL, which caused significantly higher rates of tumor recurrence (P < .05). Conclusion We demonstrate that SBT represents a safe, minimally invasive, and highly effective local treatment option for pediatric patients with inoperable LGG WHO grades I and II.

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