scholarly journals The characteristics and outcomes of patients with malignant pheochromocytoma and paraganglioma: Single-Center Experience

Acta Medica ◽  
2020 ◽  
Vol 51 (2) ◽  
pp. 25-29
Author(s):  
Asena Gökçay Canpolat ◽  
Demet Çorapçıoğlu

ABSTRACT Introduction: Metastatic pheochromocytomas (PCCs) and paragangliomas (PGLs) (PPGLs) are defined by the presence of metastatic disease in nonchromaffin tissue. It is rare, and there is no guidance for clinical, histological, or biochemical predictors of metastatic behavior. So, we aimed to evaluate our PPGL patients and investigate the features and treatment outcomes of our cohort. Methods: Between 2010 and 2019, 35 PCC and 3 PGL were included in the study. The age, clinical features, demographic, biochemical, radiological data, and treatment outcomes of the patients were analyzed. Results: The mean age was 38.3 ± 15.3 years, and the female to male ratio was 22/16. Twenty-one patients had hereditary causes of PCC (Multiple endocrine neoplasia (MEN) type 2A n:17; MEN2B n:4 and Neurofibromatosis n:1). The metastatic disease was seen in four of PCCs and one of PGLs. The metastatic cases were sporadic both in PCC and PGL patients. The mean greatest dimension of the primary tumor of metastatic PPGL cases was 4.5±3.2 cm. The sites for metastasis for PCC were liver, bones, lungs, and lymph nodes in all cases. Surgery was performed, cytotoxic chemotherapy, and 131I-MIBG radionuclide therapy were given for all PCCs and external beam radiation for PGL. Three of the cases were stable with cytotoxic chemotherapy and 131I-MIBG, but one case declined, and after a total of four cycles of 177Lu therapy, she died. Conclusion:: In our tertiary referral center, the majority of the patients who were followed with PGGLs have hereditary/genetic etiology. It is observed that malignant PPGLs constitute 10.5 % of our patients, the same as the literature. Patients should be directed to centers where chemotherapy and theranostic treatments with a multidisciplinary approach are present in cases of metastatic disease in an individualized manner.   Keywords: Pheochromocytoma, paraganglioma, metastatic disease, theranostic treatment                          

2021 ◽  
Vol 39 (15_suppl) ◽  
pp. 2061-2061
Author(s):  
Andrew J. Brenner ◽  
Ande Bao ◽  
William Phillips ◽  
Gregory Stein ◽  
Vibhudutta Awasthi ◽  
...  

2061 Background: While external beam radiation therapy (EBRT) remains a central component of the management of primary brain tumors, it is limited by tolerance of the surrounding normal brain tissue. Rhenium-186 NanoLiposome (186RNL) permits the delivery of beta-emitting radiation of high specific activity with excellent retention in the tumor. We report the results of the phase 1 study in recurrent glioma. Methods: A Phase 1 dose-escalation study of 186RNL in recurrent glioma utilizing a standard 3+3 design was undertaken to determine the maximum tolerated dose of 186RNL. 186RNL is administered by convection enhanced delivery (CED). Infusion is followed under whole body planar imaging and SPECT/CT. Repeat SPECT/CT imaging is performed immediately following, and at 1, 3, 5, and 8 days after 186RNL infusion to obtain dosimetry and distribution. Subjects were followed until disease progression by RANO criteria. Results: Eighteen subjects were treated across 6 cohorts. The mean tumor volume was 9.4 mL (range 1.1 – 23.4). The infused dose ranged from 1.0 mCi to 22.3 mCi and the volume of infusate ranged from 0.66 mL to 8.80 mL. From 1 – 4 CED catheters were used. The maximum catheter flow rate was 15 µl/min. The mean absorbed dose to the tumor volume was 239 Gy (CI 141 – 337; range 9 - 593), to normal brain was 0.72 Gy (CI 0.34 – 1.09; range 0.005 – 2.73), and to total body was 0.07 Gy (CI 0.04 – 0.10; range 0.001 – 0.23). The mean absorbed dose to the tumor volume when the percent tumor volume in the treatment volume was 75% or greater (n = 10) was 392 Gy (CI 306 – 478; range 143 – 593). Scalp discomfort and tenderness related to the surgical procedure did occur in 3 subjects. The therapy has been well tolerated, no dose-limiting toxicity has been observed, and no treatment-related serious adverse events have occurred despite markedly higher absorbed doses typically delivered by EBRT in patients with prior treatment. Responses have been observed supporting the clinical activity. Final results from the dose escalation will be presented. Conclusions: 186RNL administered by CED to patients with recurrent glioma results in a much higher absorbed dose of radiation to the tumor compared to EBRT without significant toxicity. The recommended Phase 2 dose is 22.3 mCi in 8.8 mL of infusate. Clinical trial information: NCT01906385. [Table: see text]


2013 ◽  
Vol 7 (3-4) ◽  
pp. e202-6 ◽  
Author(s):  
Colin I. Tang ◽  
Perakaa Sethukavalan ◽  
Patrick Cheung ◽  
Gerard Morton ◽  
Geordi Pang ◽  
...  

Background: The purpose of this study was to monitor patient pain score with transperineal prostatic gold seed implantation in the absence of conscious sedation.Methods: All patients who were scheduled for image-guided external beam radiation (IGRT) and referred for gold seed fiducials were eligible to participate. Gold seed implants were performed by two radiation oncologists between December 2007 and April 2008. Patients received only local and deep anesthetic. No patients had prophylactic IV cannulation for the procedure. Three gold seeds were inserted transperineally into the prostate. A visual analogue scale from 0 to 10 was used to assess the pain at baseline, local and deep anesthetic infiltration, with each seed drop, and after the completion of the procedure.Results: A total of 30 patients were accrued to this study. The highest recorded increase in pain score was at the time point of deep local anesthesia, at which the mean pain score was 3.8. The mean pain scores at each seed drop were 0.8 (standard deviation [SD]=1.24), 1 (SD=1.26), and 0.5 (SD=0.90), respectively. All gold seed insertion procedures were well-tolerated, with no patient shaving significant pain post-procedure, and no significant procedural complications. There were only slight increases in dysuria, urinary frequency, constipation, urinary retention and flatulence in 7 patients – none of which required intervention.Interpretation: Transperineal ultrasound-guided gold seed implantation without conscious sedation is well-tolerated and associated with a low complication rate. It is a convenient outpatient procedure obviating the need for resource intensive postoperative monitoring.


2011 ◽  
Vol 114 (3) ◽  
pp. 747-755 ◽  
Author(s):  
Marco Schiariti ◽  
Pablo Goetz ◽  
Hussien El-Maghraby ◽  
Jignesh Tailor ◽  
Neil Kitchen

Object Hemangiopericytomas are rare tumors that behave aggressively with a high rate of local recurrence and distant metastases. With the aim of determining the outcome and response to various treatment modalities, a series of 39 patients who underwent microsurgical resection for primary meningeal hemangiopericytoma over a 24-year period is presented. Methods Patients with hemangiopericytoma were identified from histopathology records and their medical records were analyzed retrospectively by 2 independent reviewers to collect data on surgical treatment, adjuvant therapy, postoperative course, local or distant recurrence, and follow-up. Results Of the 39 patients, 19 were male and 20 were female. Mean patient age was 44.1 years. Thirty-four tumors were intracranial and 5 were spinal. The mean follow-up period was 123 months. Twenty-eight patients developed local recurrence. The recurrence rate at 1, 5, and 15 years was 3.5%, 46%, and 92%, respectively. Extraneural metastasis occurred in 8 patients (26%) at an average of 123 months after initial surgery. Recurrences and metastases were treated by surgical excision, external beam radiation therapy (EBRT), chemotherapy, and/or stereotactic radiosurgery. Adjuvant EBRT following initial surgery was found to extend the disease-free interval from 154 months to 254 months, although it did not prevent the development of metastasis. In those patients with EBRT and complete resection, the mean recurrence-free interval was found to be 126.3 months longer (p = 0.04) and overall survival 126 months longer than without EBRT. Furthermore, adjusting for resection, patients undergoing EBRT had 0.33 times increased risk of recurrence compared with those who did not (p = 0.03). A majority of patients remained able to live independently despite revision surgery for recurrence. Conclusions The mean follow-up of this patient series represents the longest follow-up duration published to date and demonstrates extended survival in a significant number of patients with hemangiopericytoma. Gross-total resection followed by adjuvant EBRT provides patients with the highest probability of an increased recurrence-free interval and overall survival. Prolonged survival justifies long-term follow-up and aggressive treatment of initial, recurrent, and metastatic disease.


2021 ◽  
Vol 12 ◽  
Author(s):  
Jan Calissendorff ◽  
Freja Bjellerup-Calissendorff ◽  
Robert Bränström ◽  
C. Christofer Juhlin ◽  
Henrik Falhammar

PurposeNeuroendocrine tumors of the pancreas (Pan-NETs) are usually hormonally inactive with a capacity to metastasize. Since Pan-NETs are rare, more knowledge is needed.MethodsWe reviewed all patients’ medical files with Pan-NET treated at a tertiary center (2006-2019). Grade 1 (G1) and grade 2 (G2) tumors were compared. The latter group was subdivided arbitrarily based on proliferation index into G2a (3-9.9%) and G2b (10-19.9%).ResultsWe found 137 patients (76 females, 61 males; G1 n=66, G2 n=42), the median age at diagnosis 61 years (interquartile range (IQR) 50–71), and tumor size 2 cm (1.3–5 cm). The initial surgery was performed in 101 patients. The remaining (n=36) were followed conservatively. Metastatic disease was evident in 22 patients (16%) at diagnosis while new lesions developed in 13 out of 22 patients (59%). In patients without previous metastatic disease, progressive disease was discovered in 29% of G1 vs. 55% of G2 patients (P=0.009), 47% of G2a vs. 75% of G2b patients (NS). Survival was poorer in patients with metastasis at diagnosis vs. those with local disease (P<0.001). During follow-up of 74 months, Pan-NET related death was found in 10 patients. Survival was not different between G1 vs. G2 or G2a vs. G2b, or if tumors were functional. Size ≤2 cm was associated with a better outcome (P=0.004). During the follow-up of small tumors (≤2 cm, n=36) two were resected.ConclusionIn small non-functional Pan-NETs, active surveillance is reasonable. Progressive disease was more common in G2, but survival was similar in G1, G2 and between G2 subgroups. Survival was poorer in patients with metastasis at diagnosis.


1996 ◽  
Vol 14 (4) ◽  
pp. 1093-1097 ◽  
Author(s):  
G E Hanks ◽  
A L Hanlon ◽  
G Hudes ◽  
W R Lee ◽  
W Suasin ◽  
...  

PURPOSE The patterns of failure (local and/or regional v metastatic) have been determined for patients with prostate cancer and pretreatment prostate-specific antigen (PSA) levels > or = 20 ng/mL treated with radiation alone with the purpose to design appropriate multimodal treatments. MATERIALS AND METHODS One hundred twenty patients with pretreatment PSA levels > or = 20 ng/mL were treated with external-beam radiation alone between February 1988 and October 1993. They were arbitrarily divided by PSA levels, 20 to 29.9 ng/mL, 30 to 49.9 ng/mL, and > or = 50 ng/mL, and analyzed in terms of freedom from any failure (no evidence of biochemical disease [bNED], and PSA level < 1.5 ngm/mL and not increasing), as well as freedom from imaging evidence of distant metastasis (fdm). RESULTS There was no significant difference in short-term outcome by pretreatment PSA level, and thus all patients were pooled for analysis. At 4 years, 81% were fdm and 28% were free of any failure. This suggests that approximately 50% have recurred with local and/or regional disease or undetectable metastatic disease. Multivariate analysis indicated that low palpation stage and higher center of prostate dose were associated with better bNED survival. Multivariate analysis indicated that increasing stage and younger age are significantly associated with increasing distant metastasis. CONCLUSION Patients with pretreatment PSA levels > or = 20 ng/mL are not optimally treated by irradiation alone. The pattern of failure suggests improvement may come from systemic treatment of metastatic disease and high-dose radiation to improve locoregional disease. To evaluate this, we have begun a multimodal trial of chemohormonal therapy followed by extended-field irradiation.


2004 ◽  
Vol 17 (2) ◽  
pp. 68-72 ◽  
Author(s):  
Michael Lim ◽  
Iris C. Gibbs ◽  
John R. Adler ◽  
Steven D. Chang

Object Since the mid-1990s the use of radiosurgery for glomus jugulare tumors has grown in popularity. Despite its increased use, follow-up periods for radiosurgery are short and the numbers of patients reported are small. To add to the available information, the authors report their experience with the application of linear accelerator (LINAC) or CyberKnife modalities in 13 patients with 16 tumors. Methods All patients were treated with frame-based LINAC or CyberKnife radiosurgery, with doses ranging from 1400 to 2700 cGy. Patients were retrospectively assessed for posttreatment side effects, which included hearing loss, tongue weakness, and vocal hoarseness. The patients' most recent magnetic resonance (MR) images were also assessed for changes in tumor size. The median follow-up duration was 41 months and the mean follow-up period was 60 months. All tumors remained stable or decreased in size on follow-up MR images. All patients had stable neurological symptoms, and one experienced transient ipsilateral tongue weakness and hearing loss, both of which subsequently resolved. One patient experienced transient ipsilateral vocal cord paresis; however, this individual had received previous external-beam radiation therapy. Conclusions The authors' findings continue to support radiosurgery as an effective and safe method of treatment for glomus jugulare tumors that results in low rates of morbidity.


2020 ◽  
Vol 9 (10) ◽  
pp. 3363
Author(s):  
Julie Wong ◽  
Luke Witherspoon ◽  
Eugenia Wu ◽  
Sara Sheikholeslami ◽  
Wen Liao ◽  
...  

Prostate cancer (PC) treatment leads to impairment of sexual function. The Prostate Cancer Supportive Care (PCSC) Program’s Sexual Rehabilitation clinic (SRC) assists patients and their partners with sexual recovery using a biopsychosocial approach to rehabilitation. This study characterizes patients seen in the SRC between July 2013–1 July 2019. Data was retrospectively abstracted from clinic records. In total, 965 patients were seen over 3391 appointments during the study period. Median age (standard deviation (SD)) was 66 years (SD = 7.1), 82.0% were partnered, yet 81.7% attended appointments alone. 88.0% were treated with surgery, 5.1% with brachytherapy, 3.7% with external beam radiation (EBRT), 1.8% with combined brachytherapy and EBRT, and 1.4% with androgen deprivation therapy. In total, 708 patients (73.4%) attended ≥1 follow-up appointment. Median time (SD) between end of prostate cancer treatment to first SRC appointment was 270 days (range 0–7766). The mean (SD) self-reported overall sexual satisfaction (extracted from International Index of Erectile Function-5 (IIEF-5)) significantly increased both with erectile aids (1.69 (SD = 1.52) to 2.26 (SD = 1.66), p < 0.001, n = 148) and without erectile aids (1.71 (SD = 1.44) to 2.35 (SD = 1.57), p < 0.001, n = 235). This study provides guidance for further investigation to refine treatment, wait-times, support, and/or resource offerings in this type of program.


2004 ◽  
Vol 89 (11) ◽  
pp. 5303-5307 ◽  
Author(s):  
Anca M. Avram ◽  
Robert Gielczyk ◽  
Lyndon Su ◽  
Andrew K. Vine ◽  
James C. Sisson

Abstract A patient with widely metastatic papillary thyroid cancer who had been previously treated with 131I and external beam radiation presented with purple nodular lesions on his face and scalp. On biopsy, the nodules were papillary carcinoma with cells that stained for thyroglobulin. Subsequently he developed decreased left eye visual acuity, and fundoscopy revealed lesions typical of choroidal metastases. Dermal and choroidal metastases of papillary thyroid carcinoma are both rare. However, the significance of these clinical manifestations may be overlooked and ignored unless the diagnosis is considered. New skin nodules or visual acuity decline in a patient with papillary thyroid cancer may represent manifestations of distant metastatic disease and should prompt thorough evaluation with dermatological examination and fundoscopy. Choroidal and skin metastases have almost always occurred in patients with advanced disease, but initial presentation with these lesions is possible, and in such instances a thorough search for additional sites of metastatic disease is recommended. Occasionally such metastases may respond to 131I therapy or external beam radiation.


2020 ◽  
Vol 7 (50) ◽  
pp. 3061-3066
Author(s):  
Niharika Darasani

BACKGROUND Cervical cancer is one of the commonest malignancies among women in India. The main stay of treatment is the combination of External Beam Radiation Therapy (EBRT) and Intracavitary Brachytherapy (ICBT) in these patients. We compared conventional point A based treatment planning and MRI based volume optimized planning in ICBT of cervical carcinoma along with doses to organs at risk in both plans. We also compared the radiation doses to organs at risk in both the plans by International Committee on Radiation Units (ICRU) recommended points and dose volume histograms. METHODS Eighteen Patients with cancer cervix (72.22% with stage IIB) received EBRT on linear accelerator by four field technique using 15 MV energy for a dose of 46 Gray (Gy) – 50 Gy in 23-25 fractions 2.0 Gy per fraction, five days per week, for 5 weeks to whole pelvis. Most of the patients received at least three doses of chemotherapy. A total of 50 high-dose rate intracavitary brachytherapy (HDR-ICBT) applications done in 18 patients were used for analysis in the study. Clinical history, gynaecology examination and punch biopsy were undertaken. The patients were assessed during EBRT after 2 weeks, for ICBT application and suitable patients were selected for the procedure. CT based point A planning and MRI based volume optimised planning were done for each ICBT application before intracavity brachytherapy. Contouring of rectum, bladder, right and left femoral heads, and small bowel were done. RESULTS The median age of patients in this study was 50.4 ± 03.25 years. 72.22% (13/18) of the patients were of stage IIB. The mean dose delivered to 90% high-risk clinical target volume (D90- HR-CTV) for all 50 applications by volume optimized planning was 06.87 ± 0.942 Gy. The mean D90-HR-CTV by point A based conventional planning was 13.69 ± 1.06 GY. The mean D100-HR-CTV by volume optimized planning was 05.30 Gy (± 0.20). The mean D100-HR-CTV by point A based conventional planning was 08.91 ± 0.74 Gy. Maximum doses in the bladder and rectum were significantly lower (p<0.05) for MRI planning than for the conventional approach (06.49 GY Vs. 07.45 GY) for bladder; (04.57 GY vs. 05.06 GY) for rectum respectively. Both bladder (D2cc) and rectum (D2cc) doses could be reduced significantly by volume optimization. CONCLUSIONS D90-HR-CTV adequately covered by MRI based volume optimized planning was superior to conventional point A based planning in terms of both conformity of target coverage and evaluation of Organ at Risk (OARs), including the rectum and bladder. Both bladder and rectum doses in the most irradiated 2cc volume are significantly reduced in volume optimized planning. Hence, volume optimized planning would be more beneficial in large volume diseases to get better target coverage at the same time sparing the organs in small volume diseases. Hence, the use of MRI-based volume optimization brachytherapy for patients with large volume tumours with parametrial invasion is beneficial. KEYWORDS Cancer, Cervix, Woman, Radiotherapy, Brachytherapy, EBRT, ICBT, Prognosis


Sign in / Sign up

Export Citation Format

Share Document