Neurosurgical treatment of low-grade cerebellar astrocytoma in children and adolescents: a single consecutive institutional series of 100 patients

Object The objective of this study was to delineate the long-term results of surgical treatment of pediatric low-grade cerebellar astrocytoma. Methods One hundred consecutive children and adolescents (0–19 years old) who underwent primary tumor resection for a low-grade cerebellar astrocytoma during the years 1980–2011 were included in this retrospective study on surgical morbidity, mortality rate, academic achievement, and/or work participation. Gross motor function and activities of daily living were scored according to the Barthel Index. Results Of the 100 patients, 61 children were in the 1st decade, and 39 were 10–19 years old. The male/female ratio was 1.13:1 (53 males, 47 females). No patients were lost to follow-up. There were no deaths in this series and all 100 patients are currently alive. In 29 patients, the follow-up duration was less than 10 years, in 37 it was between 10 and 19 years, and in 34 it was between 20 and 31 years. The Barthel Index was 100 (normal) in 97 patients, 90 in 2 patients, and 40 in the last patient. A total of 113 tumor resections were performed. Two patients underwent further tumor resection due to MRI-confirmed residual tumor demonstrated on the immediate postoperative MR image (obtained the day after the initial procedure). Furthermore, 9 children underwent repeat tumor resection after MRI-confirmed progressive tumor recurrence up to 10 years after the initial operation. Two of these patients also underwent a third resection, without subsequent radiation therapy, and have experienced 8 and 12 years of tumor-free follow-up thereafter, respectively. A total of 15% of the patients required treatment for persistent hydrocephalus. Conclusions Low-grade cerebellar astrocytoma is a surgical disease, in need of long-term follow-up, but with excellent long-term results. Nine percent of the children in this study underwent repeated surgery due to progressive tumor recurrence, and 15% were treated for persistent hydrocephalus.

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Neurosurgical treatment of pediatric pleomorphic xanthoastrocytomas: long-term follow-up of a single-institution, consecutive series of 12 patients

Journal of Neurosurgery Pediatrics ◽

10.3171/2018.11.peds18449 ◽

2019 ◽

Vol 23 (4) ◽

pp. 512-516

Author(s):

Tryggve Lundar ◽

Bernt Johan Due-Tønnessen ◽

Radek Frič ◽

Bård Krossnes ◽

Petter Brandal ◽

...

Keyword(s):

Adjuvant Therapy ◽

Primary Tumor ◽

Barthel Index ◽

Tumor Resection ◽

Long Term Results ◽

Primary Tumor Resection ◽

Low Grade ◽

Initial Operation

OBJECTIVEThe authors conducted a study to delineate the long-term results of the surgical treatment of pediatric pleomorphic xanthoastrocytomas (PXAs).METHODSAll consecutive children and adolescents (0–20 years) who underwent primary tumor resection for a PXA during the years 1972–2015 were included in this retrospective study on surgical morbidity, mortality rate, academic achievement, and/or work participation. Gross motor function and activities of daily living were scored according to the Barthel Index.RESULTSOf the 12 patients, 8 patients were in the 1st decade of life and 4 in the 2nd. The male/female ratio was 6:6. No patient was lost to follow-up. One patient presented with severe progressive tumor disease and died within 3 months after repeated resection. Another child died 3 days following a second surgical procedure involving gross-total resection (GTR) 8 years after the initial operation. The other 10 patients were alive at the latest follow-up when they reached the median age of 34 years (range 11–60 years). The median follow-up duration was 22 years (range 2–41 years). Barthel Index score was 100 in all 10 survivors. A total 18 tumor resections were performed. Five patients underwent a second tumor resection after MRI/CT confirmed recurrent tumor disease, from 6 months up to 17 years after the initial operation. Only one of our patients received adjuvant therapy: a 19-year-old male who underwent resection (GTR) for a right-sided temporal tumor in 1976. This particular tumor was originally classified as astrocytoma WHO grade IV, and postoperative radiotherapy (54 Gy) was given. The histology was reclassified to that of a PXA. Seven of 8 children whose primary tumor resection was performed more than 20 years ago are alive as of this writing—i.e., 88% observed 20-year survival. These are long-term survivors with good clinical function and all are in full- or part-time work.CONCLUSIONSPediatric patients with PXA can be treated with resection alone with rewarding results. Recurrences are not uncommon, but repeated surgery is well tolerated and should be considered in low-grade cases before adjuvant therapy is implemented. Follow-up including repeated MRI is important during the first postoperative years, since individual patients may have a more aggressive tumor course.

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Neurosurgical treatment of pediatric low-grade midbrain tumors: a single consecutive institutional series of 15 patients

Journal of Neurosurgery Pediatrics ◽

10.3171/2014.9.peds1462 ◽

2014 ◽

Vol 14 (6) ◽

pp. 598-603 ◽

Cited By ~ 7

Author(s):

Tryggve Lundar ◽

Bernt Johan Due-Tønnessen ◽

Arild Egge ◽

David Scheie ◽

Petter Brandal ◽

...

Keyword(s):

Barthel Index ◽

Tumor Resection ◽

Long Term Results ◽

Low Grade ◽

Surgical Morbidity ◽

Acute Hemorrhage ◽

Female Ratio ◽

Initial Resection

Object The authors delineate the long-term results of surgical treatment for pediatric low-grade midbrain glioma. Methods A series of 15 consecutive patients (age range 0–15 years) who underwent primary tumor resection for a low-grade midbrain glioma during the years 1989–2010 were included in this retrospective study on surgical morbidity, mortality rate, academic achievement, and/or work participation. Gross motor function and activities of daily living were scored according to the Barthel Index. Results Of the 15 patients, 10 were in their 1st decade (age 0–9 years) and 5 were in their 2nd decade of life (age 10–15 years) at the time of surgery. The male/female ratio was 0.50 (5:10). No patients were lost to follow-up. One patient died in the postoperative period (32 days posttreatment). Another 2 patients died during follow-up. One patient succumbed to acute bleeding in the resection cavity 8 months after surgery, and the other died of shunt failure 21 years after initial treatment. Twelve patients are alive at the time of this writing, with follow-up periods from 3 to 24 years (median 8 years). Among the 12 survivors, the Barthel Index scores were normal (100) in 11 patients and 80 in 1 patient. A total of 25 tumor resections were performed. In 1 patient, further resection was performed 5 days after initial resection due to MRI-confirmed residual tumor. Another 5 patients underwent repeat tumor resection after MRI-confirmed progressive tumor disease and clinical deterioration ranging from 3 months to 4 years after the initial operation. Three of these 5 patients also underwent a third resection, and 1 of the 3 underwent a fourth operation. Six children received adjuvant therapy: local radiotherapy in 2 patients, chemotherapy in 3 patients, and both in 1 patient. Twelve (80%) of the 15 patients needed treatment for persistent hydrocephalus. Conclusions Selected cases of low-grade midbrain gliomas may clearly benefit from resection with favorable results, even for prolonged periods. Three patients in the present series died, one of whom had a prolonged survival period of 21 years. Among the 12 survivors, stable long-term results appeared obtainable in at least 9. One patient died of acute hemorrhage 8 months after initial resection; otherwise, rapid tumor progression and death were not observed. Forty percent of the patients received adjuvant treatment, with local radiotherapy, chemotherapy, or both.

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Surgical treatment of pulmonary artery sarcoma: a report of 17 cases

Pulmonary Circulation ◽

10.1177/2045894020986394 ◽

2021 ◽

Vol 11 (1) ◽

pp. 204589402098639

Author(s):

Wu Song ◽

Long Deng ◽

Jiade Zhu ◽

Shanshan Zheng ◽

Haiping Wang ◽

...

Keyword(s):

Pulmonary Artery ◽

Survival Rates ◽

Tumor Resection ◽

Long Term Results ◽

Pulmonary Endarterectomy ◽

Pulmonary Artery Sarcoma ◽

The Mean ◽

Operative Findings

Pulmonary artery sarcoma (PAS) is a rare and devastating disease. The diagnosis is often delayed, and optimal treatment remains unclear. The aim of this study is to report our experience in the surgical management of this disease. Between 2000 and 2018, 17 patients underwent operations for PAS at our center. The medical records were retrospectively reviewed to evaluate the clinical characteristics, operative findings, the postoperative outcomes, and the long-term results. The mean age at operation was 46.0 ± 12.4 years (range, 26–79 years), and eight (47.1%) patients were male. Six patients underwent tumor resection alone, whereas the other 11 patients received pulmonary endarterectomy (PEA). There were two perioperative deaths. Follow-up was completed for all patients with a mean duration of 23.5 ± 17.6 months (1–52 months). For all 17 patients, the median postoperative survival was 36 months, and estimated cumulative survival rates at 1, 2, 3, and 4 years were 60.0%, 51.4%, 42.9%, and 21.4%, respectively. The mean survival was 37.0 months after PEA and 14.6 months after tumor resection only ( p = 0.046). Patients who had no pulmonary hypertension (PH) postoperatively were associated with improved median survival (48 vs. 5 months, p = 0.023). In conclusion, PAS is often mistaken for chronic pulmonary thromboembolism. The prognosis of this very infrequent disease remains poor. Early detection is essential for prompt and best surgical approach, superior to tumor resection alone, and PEA surgery with PH relieved can provide better chance of survival.

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A follow-up study of long-term results after cochlear implantation in children and adolescents

European Archives of Oto-Rhino-Laryngology ◽

10.1007/bf00615114 ◽

1996 ◽

Vol 253 (3) ◽

pp. 158-166 ◽

Cited By ~ 13

Author(s):

J. Kiefer ◽

V. Gall ◽

C. Desloovere ◽

R. Knecht ◽

A. Mikowski ◽

...

Keyword(s):

Children And Adolescents ◽

Cochlear Implantation ◽

Long Term Results ◽

Follow Up Study

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Long-term Results of Low Grade Appendiceal Mucinous Neoplasm (LAMN): A Retrospective Analysis of 24 Patients

Archives of Iranian Medicine ◽

10.34172/aim.2021.87 ◽

2021 ◽

Vol 24 (8) ◽

pp. 615-621

Author(s):

Mustafa Gok ◽

Ugur Topal ◽

Muhammet Akyüz ◽

Abdullah Bahadır Öz ◽

Erdogan Sozuer ◽

...

Keyword(s):

Age Distribution ◽

Long Term Results ◽

Pathological Examination ◽

Resection Margins ◽

Low Grade ◽

Common Symptom ◽

Mucinous Neoplasm ◽

The Mean

Background: Appendix tumors are rare tumors found in the gastrointestinal tract, observed at a rate of about 0.2%–0.3%. Our aim in this study was to present the clinicopathological classification, treatment and long-term prognosis of patients with low grade appendiceal mucinous neoplasm (LAMN). Methods: Patients who underwent surgery in the Erciyes University Department of (Kayseri, Turkey), Department of General Surgery between December 2010 and December 2018, and who had LAMN as a result of pathology were included in our study. Demographic data, clinical and pathological features of the disease, their treatment and follow-up results after treatment were reviewed retrospectively. Results: We included 24 patients in the study. Of these patients, 10 (41.6%) were male. The mean age distribution was 56.4 ± 20.3 (21–91) years. Appendectomy was performed in 14 patients, and additional organ resections were performed in 8 patients. The most common symptom at the time of presentation was abdominal pain (79.1%; 95% CI, 58.3–91.7). The most common preliminary diagnosis in the preoperative period was acute appendicitis (50%; 95% CI, 29.2–70.8). Mean postoperative hospitalization time was 7.4 ± 7.96 (2–31) days. On pathological examination, appendectomy resection margins were positive in two patients. The mean (median) postoperative follow-up was 31.25 ± 23.9 (27) (1–90) months. One-year survival was 91.6%, and 5-year survival was 83.3%. Recurrence was detected in three patients during the follow-up period. Conclusion: If appendix mucinous neoplasia (AMN) is suspected in patients undergoing surgery with an initial diagnosis of acute or plastron appendicitis, care should be taken to remove the lesion without perforation. Pseudomyxoma peritonei, which may develop as a result of perforation, is associated with recurrence and decreased survival.

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Long-Term Neurological and Neurosurgical Outcomes After Surgery Only in Children with Low-Grade Brain Tumors

Journal of Neurosurgery Pediatrics ◽

10.3171/ped/2008/1/4/paper16 ◽

2008 ◽

Vol 1 (4) ◽

pp. A354-A355 ◽

Cited By ~ 1

Author(s):

Liliana Goumnerova ◽

Dan Drzymalski ◽

Mark Kieran ◽

Scott Pomeroy ◽

R. Michael Scott ◽

...

Keyword(s):

Brain Tumors ◽

Tumor Recurrence ◽

Treatment Options ◽

Low Grade ◽

Neurological Sequelae ◽

Institutional Review ◽

Initial Surgery ◽

Extent Of Surgery

Introduction Brain tumors are the most common solid tumors of childhood. As treatment options such as surgery, chemotherapy, and radiotherapy improve, and affected children's survival increases, the focus has shifted toward understanding long-term outcomes. We hypothesized that tumor recurrence and long-term neurological outcomes can be correlated with age at diagnosis, site and size of tumor, histology, extent of surgery, and presence or absence of hydrocephalus. Thus, the aims of this study were to determine predictive factors for tumor recurrence and long-term morbidity in children who undergo surgery only for low-grade brain tumors. The study was approved by the institutional review board. Methods Medical records were reviewed for each child and information collected regarding sex, age at surgery, histology site, size of tumor, presence of hydrocephalus, extent of surgery, and date of tumor recurrence. Preoperative, postoperative, and last follow-up neurological exams were reviewed and patients were evaluated for ataxia, hemiparesis, impaired vision, cranial nerve deficits, presence of seizures or headaches, dysmetria, nystagmus, and depression. Results Forty-nine children (29 male, 20 female) ranging from 2.2 to 16.9 years of age at first operation were included; 31% had recurrence/progression. This was significantly correlated with tumors that were subtotally resected (p = 0.002) located in the supratentorial region (p = 0.017) and not astrocytoma or craniopharyngioma (p = 0.041). At last follow-up, 42% of children had no neurological sequelae, 53% had mild neurological sequelae, and 5% had moderate neurological sequelae. No children had experienced depression before or immediately after their initial surgery. However, 6 patients (16% of 38 patients) had depression at long-term follow-up. Conclusion These data indicate that we may be able to predict recurrence/progression of low-grade brain tumors based on extent of surgery location and histology. We also see that more than half of these children will experience a variety of surgical and neurological burdens throughout their lives.

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Does combined dose intensification radiotherapy improve disease control in resectable retroperitoneal sarcoma? Long-term results of a phase I/II trial.

Journal of Clinical Oncology ◽

10.1200/jco.2012.30.15_suppl.10050 ◽

2012 ◽

Vol 30 (15_suppl) ◽

pp. 10050-10050

Author(s):

Myles JF Smith ◽

Paul F Ridgway ◽

Charles N Catton ◽

Amanda Cannell ◽

Brian O'Sullivan ◽

...

Keyword(s):

Phase I ◽

Disease Control ◽

Retroperitoneal Sarcoma ◽

Long Term Results ◽

Low Grade ◽

Term Survival ◽

Primary Disease ◽

Upper Abdomen

10050 Background: Late failure is a challenging problem in retroperitoneal sarcoma (RPS) and reported 10 yr overall survival (OS) rates range from 20-30%. Use of preoperative external beam radiotherapy (XRT) in the management of RPS remains controversial. No RCT and very few prospective trials of any type have been completed. We investigated the effects of preop XRT plus dose escalation with early postop brachytherapy (BT) on long term survival and recurrence in RPS. Methods: From 06/96 to 10/00, 40 patients (25 female) with resectable RPS were entered onto a phase I/II trial of preop XRT (50 Gy) plus postop BT (20-25 Gy). As previously reported, BT to the upper abdomen was associated with significant grade III-V postop toxicity, and from 12/98 on, BT was applied only to cases where the “field at risk” excluded the upper abdomen. Kaplan Meier survival curves were constructed; OS and recurrence free survival (RFS) were compared by log rank (SPSS 19.0). Results: Median age at study entry was 58 (38-70) yrs. Twenty nine patients presented to our center with primary disease (73%), and 22 (55%) had high grade tumors. All patients had preop XRT and total gross resection, while half (n=19) received BT. As of 12/2011, median follow-up time is 108 mos. For the entire study cohort, OS at 5 and 10 yrs were 70% and 65%, respectively; RFS at 5 and 10 yrs were 65% and 58%, respectively. RFS at 5 yrs was reduced in high vs. low grade RPS (50% vs. 83%, p=0.028), but by 10 yrs. was similar in high and low grade tumors (50% vs. 67%, p=ns). RFS was reduced in patients who presented with recurrent vs. primary disease (27% vs. 69% at 10 yrs., p=0.018), as was OS (36% vs. 76% at 10 yrs., p=0.034). Neither OS nor RFS was improved in the cohort of patients who received BT compared to the cohort who did not: at 10 yrs. RFS was 53% +BT and 62% -BT, while OS was 53% and 76%, respectively, p=ns. Conclusions: In this prospective study with mature follow-up, long term OS and RFS in patients who underwent combined preop XRT plus resection of RPS compare favorably with those reported in retrospective institutional and population-based series. Postoperative BT did not contribute to disease control.

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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 ◽

10.1182/blood.v124.21.4465.4465 ◽

2014 ◽

Vol 124 (21) ◽

pp. 4465-4465 ◽

Cited By ~ 1

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.

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