Significant Palliation Research Articles

Epilepsy surgery in children with genetic etiologies: A prospective evaluation of current practices and outcomes

ObjectiveThis study assesses current practices and outcomes of epilepsy surgery in children with a genetic etiology. It explores the pre-surgical workup, types of surgeries, and post-surgical outcomes in a broad array of disorders. MethodsPatients ≤18 years who completed epilepsy surgery and had a known genetic etiology prior to surgical intervention were extrapolated from the Pediatric Epilepsy Research Consortium (PERC) surgery database, across 18 US centers. Data were assessed univariably by neuroimaging and EEG results, genetic group (structural gene, other gene, chromosomal), and curative intent. Outcomes were based on a modified International League Against Epilepsy (ILAE) outcome score. ResultsOf 81 children with genetic epilepsy, 72 % had daily seizures when referred for surgery evaluation, which occurred a median of 2.2 years (IQR 0.3, 5.2) after developing drug resistance. Following surgery, 68 % of subjects had >50 % seizure reduction, with 33 % achieving seizure freedom [median follow-up 11 months (IQR 6, 17). Seizure freedom was most common in the monogenic structural group, but significant palliation was present across all groups. Presence of a single EEG focus was associated with a greater likelihood of seizure freedom (p=0.02). SignificanceThere are meaningful seizure reductions following epilepsy surgery in the majority of children with a genetic etiology, even in the absence of a single structural lesion and across a broad spectrum of genetic causes. These findings highlight the need for expedited referral for epilepsy surgery and support of a broadened view of which children may benefit from epilepsy surgery, even when the intent is palliative.

Comparative Study of Hypofractionated Palliative Radiotherapy 17 Gy in 2 Fractions vs. 30 Gy in 10 Fractions in Lethal Locally Advanced Non-small Cell Lung Cancer

<i>Background</i>: Lung cancer kills the most people worldwide. Most Bangladeshi patients had regionally advanced surgically inoperable Non-Small Cell Lung Cancer (NSCLC), a deadly disease. Radiotherapy to main tumor and regional lymphatics with chemotherapy is conventional treatment. <i>Objective</i>: This study was done to investigate the symptoms relief and treatment related toxicities in hypo fractionated 17 Gy in 2 fractions which is effectively comparable to 30 Gy in 10 fractions. <i>Methods</i>: A total 60 patients were divided equally into Arm A and Arm B with locally advanced stage IIIA, and stage IIIB NSCLC patients with the objective to compare palliation of symptoms and to assess the toxicity profile. This Quasi Experimental study was carried out from January 2018 to December 2018 in the department of Radiation oncology, National Institute of Cancer Research and Hospital. Cases of Arm A were treated with 17 Gy radiotherapy in 2 fractions and those of Arm B were treated with 30 Gy radiotherapy in 10 fractions. <i>Results</i>: The mean age was 49.3±8.8 and 52.2±8.1 years for the Arm- A and the Arm-B respectively. Most patients in both groups were males, 80% in group A and 90% in group B. Adenocarcinoma (Arm A-70%, Arm B- 66.7%) was the most common pathological subtype in both groups, followed by Squamous cell carcinoma (30%, 33.3% respectively). This study showed that there was a significant palliation of these symptoms following radiotherapy as reported by patients and as assessed clinically with no statistically significant difference between two Arms. Hemoptysis had the highest rate of improvement, 100% in both arms and improvement in chest pain by 83.3% in arm A and 70% in Arm B. Cough was improved in 83.3% and 67.7% and dyspnea was palliated in 80.0% and 70.0% of patients in Arm A and Arm B, respectively as per clinical assessment of the patients. The early toxicities in both arms had no significant difference. Patients with locally advanced inoperable NSCLC have poor prognosis and less survival. The first goal was acceptable symptoms control as cough, dyspnoea, haemoptysis and chest pain. <i>Conclusion</i>: The result of this study showed that a significant palliation of symptoms with minimum toxicities improving quality of life following radiotherapy as reported by patients also assessed clinically and radiographically with no statistically significant difference among both arms.

Eradication of Long-Term Vaginismus Type of Genito-Pelvic Pain/ Penetration Disorder by Treating with Dextroamphetamine Sulfate

There are many therapies for the vaginismus type of genito-pelvic pain/penetration disorder. However, as frequently found when there are multiple treatments for a given pathological disorder, not any of them are highly effective for this problem. It has been hypothesized that increased tissue permeability may be the etiologic factor causing inflammation in various tissues leading to a variety of different pain syndromes in various organ systems, including, but not limited to, the pelvis. Treatment with dextroamphetamine sulfate has resulted in significant palliation of the pain in the majority of cases treated. The hypothesized mechanism is that this amphetamine releases dopamine from sympathetic nerve fibers, which, in turn, diminishes excessive infiltration of irritants, which are the etiologic factor for the pain. Ideally, the best proof of efficacy for the treatment of this type of vaginismus/dyspareunia syndrome by dextroamphetamine sulfate would be to conduct a randomized prospective double-blinded multi-center study. However, because of the generic status of this drug, it is highly unlikely that any pharmaceutical company would fund such a study. The most practical feasible method to determine efficacy would be to try the drug on a very convincing cases of long-term severe vaginismus/dyspareunia syndrome that failed to respond to standard therapy. Indeed, such a case was found in a 32-year-old woman whose vaginismus was detected at puberty, that was associated with severe dyspareunia 10 years later, so that neither insertion of tampons nor intercourse had been possible. Within one month of treatment her vulvodynia/dyspareunia were eradicated. The relief has persisted for three years. Hopefully this case report may encourage treating physicians who treat a lot of similar cases to evaluate a large series or conduct a randomized study comparing amphetamine therapy vs. their best treatment option to date.

Abstract 605: Evidence that a wide variety of cancers may use the progesterone induced blocking factor protein to escape immune surveillance as evidenced by significant palliation following treatment with progesterone receptor antagonists in advanced metastatic treatment resistant cancers

Abstract A search of the literature and abstracts from national oncology meetings was conducted to determine what types of human cancers have been reported to show some type of palliative benefit from treatment with progesterone (P) receptor antagonists. Though the original concept was to try P receptor antagonists for cancers known to be associated with the classic nuclear P receptor, recent data suggest that the real beneficial target is a membrane P receptor that helps produce a unique immunomodulatory protein called the progesterone induced blocking factor (PIBF) that inhibits natural killer (NK) cells and cytotoxic T cells in the tumor microenvironment and thus may allow cancer cells to metastasize. The following human cancers known to be associated with the classic nuclear receptor that have responded to either the P receptor modulator mifepristone or onapristone, include breast, uterine, ovarian, and prostate cancer. Human cancers not associated with the classic nuclear P receptor that have demonstrated significant palliative benefits and/or increased longevity following P receptor modulator therapy include colon, thymic cell epithelial cancer, transitional cell carcinoma of the renal pelvis, leiomyosarcoma, pancreatic carcinoma, malignant fibrous histiocytoma, glioblastoma multiforme grade IV, renal cell carcinoma, fibrous osteosarcoma, small cell lung cancer, non-small cell lung cancer (including those negative for tumor markers, or those positive for PD-1, or positive for the EGFR mutation). The wide variety of very advanced cancers that have responded to the P receptor modulators as evidenced by extension of a better quality of life despite the fact that they had progressed despite standard chemo or immunotherapy, is consistent with the concept that PIBF may be a protein needed by a large variety of cancers to advance or metastasize, and thus may provide a unique target to treat with P receptor modulators, and thus provide significant palliative benefit. In contrast to most anti-cancer agents, P receptor antagonists are extremely well tolerated, and are without serious risk of complications. Larger series are needed to determine the true beneficial effect of this type of therapy. Positive beneficial effects in case reports, at least provide the basis for selection of which cancers to evaluate in a larger series. Citation Format: Jerome H. Check, Diane Check, Carrie Wilson. Evidence that a wide variety of cancers may use the progesterone induced blocking factor protein to escape immune surveillance as evidenced by significant palliation following treatment with progesterone receptor antagonists in advanced metastatic treatment resistant cancers [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2021; 2021 Apr 10-15 and May 17-21. Philadelphia (PA): AACR; Cancer Res 2021;81(13_Suppl):Abstract nr 605.

Analysis of 40 patients who underwent tracheal resection due to benign complex tracheal stenosis

BackgroundBenign tracheal stenosis is a common complication in patients followed up in intensive care units. We aimed to analyze the etiology, diagnostic approaches, treatment methods for benign tracheal stenosis, and the predicting factors for complications after tracheal resection for benign stenosis. Material-methodForty patients who underwent tracheal resection reconstruction due to benign tracheal stenosis were analyzed retrospectively. Predictive factors for complications were determined by statistical analysis. ResultsThere were 23 patients (57.5%) in the intubation group, 11 patients (27.5%) in the tracheostomy group, and 6 patients (15%) in the subsequent tracheostomy group. Preoperatively, rigid dilatation was applied to all patients between 2 and 6 sessions (median = 3). Tracheal resections were performed in all patients after rigid dilatations. The mean of the resected segment lengths is 32.1 ± 8.8 mm. There was a statistically significant difference between preoperative bronchoscopic measurements, preoperative tomography measurements, and intraoperative measurements of the stenosis segment (ꭓ2 (2) = 71,500; p < 0.001). The patients' mean follow-up period was 27.4 ± 21.7 months (3–84). Mortality due to tracheal surgery and major anastomotic complications were not observed. The minor anastomotic complication rate was 12.5%, the non-anastomotic complication rate was 17.5%. The effect of resection length and surgical experience were found to be statistically significant risk factors for anastomotic complications.Conclusions: Rigid dilatation does not provide significant palliation in complex stenosis. Bronchoscopic measurements give closer results than CT measurements in the preoperative estimation of resection length. The risk of anastomotic complications increases when the length of the resection increases and when the surgical experience is less.

Customization of stent design for treating malignant airway stenosis with the aid of three-dimensional printing.

The treatment of malignant stenosis involving the carina or bronchi is challenging due to complicated anatomy with individual variation, which makes it necessary to customize stents for each patient. Therefore, this study aims to evaluate the feasibility of a novel metallic segmented airway stent customized with the aid of three-dimensional (3D) printing for such cases. The stents were individually customized with the aid of a 3D printed mold based on computed tomography (CT) images according to the anatomical features of the airway. A segmented design was applied on the junction part of the main stem and the branches to fit the dynamic changes of the carina angle. In 12 patients with airway stenosis caused by malignancies including esophageal cancer (EC) and lung cancer (LC), the stents were implanted. The technical and clinical success of the stenting procedure, Hugh-Jones (HJ) classification, Karnofsky performance status (KPS), and stent-related complications of patients were evaluated. The stenting procedure was technically successful in all patients, and 11 patients showed significant palliation of dyspnea after stenting. The HJ and KPS classification of patients after stent insertion improved significantly compared with those before stenting (P=0.003 and P=0.006, respectively). During follow-up, granulation tissue proliferation and sputum retention were found in two and four patients, respectively. This study shows that the implantation of a novel stent designed with the aid of 3D printing is feasible for relieving dyspnea and improving performance status of patients with inoperable malignant stenosis involving the carina or bronchi.

A Novel Segmented Stent Customized with the Aid of Three-Dimensional Printing for Inoperable Malignant Airway Stenosis Involving Carina or Distal Bronchi: A Proof-of-Concept Study

Background: Malignant airway stenosis is life-threatening and leads to a reduced quality of life. The treatment of malignant stenosis involving the carina and distal bronchi is challenging because of the complicated anatomy that is variable among individual patients, which makes it necessary to customize stents individually. Methods: The stents were individually customized with the aid of 3D printing mold according to the anatomic features of the airway in individual patients. A segmented design was applied on the junction part of the main stem and the branches to fit the dynamic changes of carina angle. 10 patients underwent insertion of such stents for dyspnea due to airway stenosis caused by malignancies including esophageal cancer and lung cancer. The technical and clinical success of stenting procedure, and Hugh-Jones (HJ) classification, Karnofsky performance status (KPS) and stent-related complications of patients were evaluated. Findings: The stenting procedures were technically successful in all patients, and 9 patients showed significant palliation of dyspnea after stenting. The HJ and KPS classification of patients after stent insertion improved significantly compared with those before stenting (P = 0.007 and P=0.015, respectively). During follow-up, granulation tissue proliferation and sputum retention were found in 2 and 3 patients, respectively. Interpretation: The implantation of the novel segmented stent is feasible for relieving dyspnea and improving performance status of patients with inoperable malignant stenosis involving the carina and distal bronchi distal. Funding Statement: Supported by the Shanghai key specialty construction project (No. ZK2019A02); Clinical key specialist construction project of Shanghai municipal health commission (Interventional Radiology & 3D Printing); Shanghai municipal commission of health and family planning (No. 201640087). Declaration of Interests: Zhongmin Wang is a co-inventor on a patent of the stent used in this study. The owner of the patent is Micro-Tech, Nanjing, China. All other authors declare no conflict of interest. All authors declare that they conducted an independent research in the absence of any financial relationship with Micro-Tech, Nanjing, China. Ethics Approval Statement: This study was approved by the institutional review board, and written informed consent was obtained from all patients.

225Ac-PSMA-617 in Chemotherapy-naive Patients with Advanced Prostate Cancer

225Ac- Prostate-specific membrane antigen (PSMA)-617 is a highly promising novel compound for therapy of prostate cancer. A remarkable therapeutic efficacy has been demonstrated in heavily pre-treated metastatic castration-resistant prostate cancer (mCRPC) patients, with xerostomia as the main side effect. A promising strategy for minimization of side effects is based on optimization of the dosing regime while maintaining sufficient therapeutic efficacy for several cohorts of patients, including chemotherapy-naïve patients. Fifty-seven patients with progressive advanced prostate cancer that had exploited established first-line or second line therapies available in South Africa or that were not eligible or refused certain established therapies were selected for treatment with 225Ac-PSMA-617 on the basis of compassionate use. Therapy was performed in 2 months intervals, with initial dose of 8 MBq (or 10 MBq in case of very advanced disease, superscan), then de-escalation to 6 or 4 MBq in case of good response. The patients were divided into two groups: A: patients that have undergone standard therapy (surgery, radiation therapy and androgen deprivation) and some second line therapies (N=17). B: patients that have undergone only standard therapy or only parts (N=40), while some patients were completely treatment naïve (n=10). Prostate-specific antigen (PSA) and blood cell count were measured every 4 weeks. Monitoring and follow-up included Eastern Cooperative Oncology Group score, pain symptoms, treatment-related toxicity, PSA-response and ALPresponse. 68Ga-PSMA-PET/CT was used for baseline staging and imaging follow-up every 8 weeks. Good antitumor activity by means of objective radiologic response or tumor marker decline was observed in 71% of patients in group A while a remarkable 92% response rate was observed in group B. Chemotherapy-naïve patients exhibited significantly increased rate of response, and of complete response. Both groups presented with significant palliation of bone pain and reduced toxicity to salivary glands due to de-escalation. These interim results also show a favourable hematological and renal toxicity profile and quality of life improvements. The remarkable therapeutic efficacy of 225Ac-PSMA617 reported earlier is confirmed with even better clinical outcomes in chemotherapy-naive advanced prostate cancer patients treated. Reduced toxicity to salivary glands due to de-escalation should be further explored for informing clinical practice and clinical trial design.

Catheter based treatments for fibrosing mediastinitis.

Fibrosing mediastinitis is a rare, often debilitating and potentially lethal disease characterized by an exuberant fibroinflammatory response within the mediastinum. Patients typically present with insidious symptoms related to compression of adjacent structures including the esophagus, heart, airways, and cardiac vessels. Fibrosing mediastinitis is most often triggered by Histoplasmosis infection; however, antifungal and anti-inflammatory therapies are largely ineffective. While structural interventions aimed at alleviating obstruction can provide significant palliation, surgical interventions are challenging with high mortality and clinical experience with percutaneous interventions is limited. Here, we will review the presentation, natural history, and treatment of fibrosing mediastinitis, placing particular emphasis on catheter-based therapies.

225Ac-PSMA-617 in chemotherapy-naive patients with advanced prostate cancer: a pilot study

BackgroundA remarkable therapeutic efficacy has been demonstrated with 225Ac-prostate-specific membrane antigen (PSMA)-617 in heavily pre-treated metastatic castration-resistant prostate cancer (mCRPC) patients. We report our experience with 225Ac-PSMA-617 therapy in chemotherapy-naïve patients with advanced metastatic prostate carcinoma.MethodsSeventeen patients with advanced prostate cancer were selected for treatment with 225Ac-PSMA-617 in 2-month intervals, with initial activity of 8 MBq, then de-escalation to 7 MBq, 6 MBq or 4 MBq in cases of good response. In one patient, activity was escalated to 13 MBq in the third cycle. Fourteen patients had three treatment cycles administered, while in three patients treatment was discontinued after two cycles due to good response. Six out of 17 patients received additional treatments after the third cycle. Prostate-specific antigen (PSA) was measured every 4 weeks for PSA response assessment. 68Ga-PSMA-PET/CT was used for functional response assessment before each subsequent treatment cycle. Serial full blood count, renal function test, and liver function were obtained to determine treatment-related side effects.ResultsGood antitumor activity assessed by serum PSA level and 68Ga-PSMA-PET/CT was seen in 16/17 patients. In 14/17 patients, PSA decline ≥90% was seen after treatment, including seven patients with undetectable serum PSA following two (2/7) or three cycles (5/7) cycles of 225Ac-PSMA-617. Fifteen of 17 patients had a > 50% decline in lesions avidity for tracer on 68Ga-PSMA-PET/CT including 11 patients with complete resolution (PET-negative and either stable sclerosis on CT for bone or resolution of lymph node metastases) of all metastatic lesions. Grade 1/2 xerostomia was seen in all patients, and none was severe enough to lead to discontinuation of treatment. One patient had with extensive bone marrow metastases and a background anemia developed a grade 3 anemia while another patient with solitary kidney and pre-treatment grade 3 renal failure developed grade 4 renal toxicity following treatment. The group presented with significant palliation of bone pain and reduced toxicity to salivary glands due to de-escalation.Conclusions225Ac-PSMA-617 RLT of chemotherapy-naïve patients with advanced metastatic prostate carcinoma led to a ≥ 90% decline in serum PSA in 82% of patients including 41% of patients with undetectable serum PSA who remained in remission 12 months after therapy. The remarkable therapeutic efficacy reported in this study could be achieved with reduced toxicity to salivary glands due to de-escalation of administered activities in subsequent treatment cycles. This necessitates further exploration for informing clinical practice and clinical trial design.

Effectiveness of Positron Emission Tomography/Computed Tomography as a Guide for Palliative Radiation Therapy for Spinal Metastases

As back pain is the presenting symptom in 95% of patients with epidural spinal metastases, appropriately identifying and treating the most symptomatic levels can provide significant palliation. The purpose of this study was to analyze the ability of combined positron emission tomography (PET)/computed tomography (CT) to identify spinal metastases with high metabolic activity and guide radiotherapy. We sought to correlate improvement in back pain with reduction in standard uptake value (SUV) after treatment. Retrospective review was performed of 72 patients with spinal metastases treated with stereotactic ablative radiation therapy at a single center between 2002 and 2014. PET/CT was used to calculate SUVs for spinal metastases, and treatment planning was based on PET/CT results. Preoperative and postoperative pain levels were assessed in all patients. Reduction in pain scores was found in 78% of treated patients. A significant reduction in pain was identified in patients with >5 metastases compared with fewer lesions (P < 0.05). Degree of change in SUV did not correlate significantly with pain relief. However, comparing pretreatment and posttreatment PET/CT, patients with improved pain consistently displayed decreased SUV. PET/CT was shown to be a useful adjunct in radiation treatment planning with change in SUV correlating with symptomatic improvement. This study paves the way for future prospective studies to further assess utility and cost-effectiveness of this imaging modality in radiation treatment planning for spinal metastases.

Chapter 14 - Intracranial dural, calvarial, and skull base metastases

Metastatic disease to the intracranial dura, the calvarium, and the skull base is relatively uncommon but presents unique diagnostic and management challenges in the patient with cancer. Modern imaging techniques have facilitated the detection of intracranial tumor deposits, leading to increased incidence. While dural and calvarial metastases often present with nonspecific symptoms, skull base metastases present with distinct clinical syndromes dependent on the local neurovascular structures affected. Intracranial dural metastases can often be confused with meningioma and pose a diagnostic challenge, as well as significant neurologic morbidity, especially in the setting of hemorrhage. Surgical intervention may be helpful in selected patients for symptomatic relief as well as survival benefit. Management paradigms need to take into account the relative risks, benefits, and likely outcomes for each possible modality of treatment. Surgical excision is useful in many patients and in combination with radiation therapy can provide significant palliation. While medical therapy is rarely an initial therapy in these entities, it may be of added benefit dependent on the underlying tumor histology and prior treatment history. Occasionally treatment with curative intent is justified.

Management of Malignant Peritoneal Mesothelioma Using Cytoreductive Surgery and Perioperative Chemotherapy.

Malignant peritoneal mesothelioma is a rare disease, with approximately 800 new patients per year in the United States. Its natural history is defined by progression restricted to the peritoneal space. In the past, patients with this disease had a limited lifespan of approximately 1 year. Numerous single-institution studies as well as a systematic review have reported median survival of 3 to 5 years with a combination of cytoreductive surgery and hyperthermic perioperative chemotherapy. These markedly improved survival statistics were achieved in experienced centers with 1% mortality and 20% morbidity rates. Data have shown that knowledgeable patient selection is required to prevent patients unlikely to benefit from undergoing these interventions. The conclusion is that patients with peritoneal mesothelioma can experience long-term progression-free survival or significant palliation with cytoreductive surgery plus hyperthermic perioperative chemotherapy. This management plan should be considered the standard of care for properly selected patients with malignant peritoneal mesothelioma at experienced centers around the world.

A reappraisal of mortality after epilepsy surgery.

To assess whether epilepsy surgery is associated with a reduction in mortality rate and if postoperative seizure frequency and severity affect mortality. A total of 1,110 patients were evaluated (1,006 surgically and 104 nonsurgically treated) for a total follow-up of 8,126.62 person-years from 1986 to 2013. Deaths were ascertained through database and Social Security Death Index query. Patients were grouped by surgery type and seizure status; standardized mortality ratio and deaths per 1,000 person-years were calculated. Survival analysis and Cox proportional hazard regression were performed. Eighty-nine deaths were observed. Surgically treated patients had a lower mortality rate (8.6 per 1,000 person-years [95% confidence interval (CI) 6.58-11.15]) than nonsurgically treated patients (25.3 per 1,000 person-years [14.50-41.17]; p < 0.001). Seizure-free patients had a lower mortality rate (5.2 per 1,000 person-years [95% CI 2.67-9.02]) than non-seizure-free patients (10.4 per 1,000 person-years [95% CI 7.67-13.89] p = 0.03). More frequent postoperative tonic-clonic seizures (>2 per year) were associated with increased mortality (p = 0.006) whereas complex partial seizure frequency was not related to death rate. Mortality was similar in temporal and extratemporal epilepsy patients (p = 0.7). Brain surgery is associated with a reduction in mortality rate in drug-resistant epilepsy, both when seizures are abolished and when it results in significant palliation of tonic-clonic seizure frequency. These observations provide further rationale for earlier consideration of epilepsy surgery.

Chemoradiotherapy with paclitaxel demonstrated significant palliation effect by local control of an anaplastic transformed thyroid carcinoma : a case report

Chemoradiotherapy with paclitaxel demonstrated significant palliation effect by local control of an anaplastic transformed thyroid carcinoma : a case report

The role of anthracyclines in small cell lung cancer.

Small cell lung cancer (SCLC) represents the 15% of the totally of lung cancer. The percentage of cases in women is arising due to the differences in smoking patterns; it occurs almost exclusively in smokers and appears to be most common in heavy smokers. The stage of disease at presentation is the most important prognostic factor in patients with SCLC; for patients with extended stage disease, the median survival is around 10 months, and the five-year survival rate is 1 to 2 percent. The standard regimen for patients with extensive disease is cisplatin based chemotherapy. Second line chemotherapy is generally less effective than the initial treatment but it can provide significant palliation for many patients. We make a review here of the different options of second line chemotherapy and the role of anthracyclines in it.

Hypofractionated palliative radiotherapy in locally advanced inoperable head and neck cancer: CMC vellore experience

Background:A novel, short duration, palliative radiotherapy schedule for inoperable head and neck cancer was evaluated in terms of palliation of cancer-related symptoms and acute toxicities.Materials and Methods:Thirty-six patients with inoperable head and neck cancer were included in the study (2010-2012). All patients received 40 Gy in 10 fractions (equivalent dose: 49.8 Gy in conventional fractionation) with 2 fractions per week. Treatment-related toxicity was assessed using Radiation Therapy Oncology Group criteria. Functional Assessment of Cancer Therapy (Head and Neck, FACT H and N) quality of life (QOL) tool was administered before starting and at the completion of radiotherapy. Mean value before and after treatment was compared (paired t-test, P = 0.05, two-tailed for significance).Results:Thirty-three patients (male: 29, female: 4, mean age: 57.8 ± 9.7 years) were included in the analysis (three patients discontinued treatment due to socioeconomic reasons). All patients had advanced inoperable head and neck cancers (27% IVA, 61% IVB, 9% IVC, TNM stage and 3% recurrent disease). Distressing pain at primary site (42%), dysphagia (18%), neck swelling (30%), and hoarseness (10%) were common presentations. Incidence of grade III mucositis and dermatitis and pain was 18%, 3%, and 24%, respectively. Planned radiotherapy without any interruptions was completed by 73% patients. QOL assessment showed improvement in social well-being (17.4 vs. 20.01, P = 0.03), but no significant change was observed in head and neck specific score (25.1 vs. 25.0, P = NS) after treatment. Reduction of pain was observed in 88% patients and 60% patients had improvement of performance status. Median overall survival of the cohort was 7 months.Conclusions:The study shows that this short duration palliative radiotherapy schedule is a clinically viable option for advanced inoperable head and neck cancer to achieve significant palliation of the main presenting symptoms like pain, dysphagia, and throat pain.

Editorial Comment: Pulmonary artery sarcoma

Angiosarcomas carry a poor prognosis, and those of the pulmonary artery are no exception. Pulmonary artery sarcomas generally derive from the endothelial cells of the pulmonary artery, and tend to spread within the pulmonary vasculature. Rarely, the pulmonary parenchyma itself is invaded (usually only in the later stages), and, unlike angiosarcomas elsewhere, pulmonary artery sarcomas do not have a high rate of lymph node and systemic metastases. This is perhaps because of the particular anatomy of the lungs—the pulmonary artery being a low-pressure system carrying venous blood and the pulmonary parenchyma having a dual blood supply (the bronchial arteries). Pulmonary artery sarcomas generally remain asymptomatic until a pulmonary vascular obstruction occurs, and, unless routine screening tests such as X-rays show an abnormality, it is generally the patient presenting with pulmonary hypertension that signals the presence of the intravascular tumour. Treatment is rarely curative. Since the tumour arises from the endothelium, surgical resection is by endarterectomy and concentrates on restoring the blood flow to the affected areas of the lung to relieve pulmonary hypertension and to restore oxygenation. To date, neither chemotherapy nor radiotherapy has provided significant palliation. A surgical cure of this bad malignancy occurs therefore largely by chance, in a very unusual case when an early diagnosis of unilateral disease allows a pneumonectomy, since pulmonary endarterectomy rarely removes all microscopic cells. In this paper by Mussot et al .[ 1], 31 patients underwent operations for pulmonary artery sarcomas over a 14-year period by a surgical team experienced in pulmonary endarterectomy and lung transplantation. Such a concentration of cases of this rare disease is unusual, and results from their referral base in patients with thromboembolic disease of the lungs. Apart from the autopsy series, the only others reporting such a series have also had pulmonary endarterectomy programmes; there have for example been previous reports from Mayer’s group [2], the group in Cambridge [3] and our group in San Diego [4]. Mayer’s report, as mentioned in this paper, contained a surprisingly small number of sarcomas in their otherwise large practice; perhaps this represents a difference in referral patterns. However, we have now encountered 45 pulmonary artery sarcomas in a series of close to 3000 pulmonary endarterectomy operations. As in the paper presented here, in our series, the diagnosis was generally known or suspected before the operation. Pulmonary artery sarcomas usually present with a different radiographic appearance from thromboembolic disease on angiography, generally being more central and bulky in character, and often apparently unilateral. As in the experience presented here, in our series, the operation was commonly performed for palliative reasons, and life expectancy considerably extended; the operation was rarely curative. This is because the disease is always bilateral if the patient presents with pulmonary hypertension, and even in the absence of pulmonary hypertension in an apparently unilateral case, bilateral seeding has probably occurred by the time the patient presents. For that reason, we have not performed a pneumonectomy—one would have to be sure that the tumour is confined to one lung to favour this approach, and this is almost never the case. The review presented here is retrospective, and therefore has the disadvantages of all retrospective studies. It is not clear why patients with lung parenchymal sarcomas were excluded, as certainly the tumours in these cases originated in the pulmonary vasculature. Similarly, it is unclear why patients with ‘inoperable primary pulmonary artery sarcomas in a fully palliative situation’ were excluded, since obviously these patients are part of a spectrum of the generally palliative nature of surgical treatment for this disease. It would have been interesting to know the subsequent survival of these patients and why they were considered inoperable.

Parathyroid Carcinoma: Update and Guidelines for Management

Parathyroid carcinoma is one of the rarest known malignancies that may occur sporadically or as a part of a genetic syndrome. It accounts for approximately 1% of patients with primary hyperparathyroidism. The majority (90%) of parathyroid cancer tumors are hormonally functional and hypersecrete parathyroid hormone (PTH). Thus, most patients exhibit strong symptomatology of hypercalcemia at presentation. Sometimes, it can be difficult to diagnose parathyroid cancer preoperatively due to clinical features shared with benign causes of hyperparathyroidism. Imaging techniques such as neck ultrasound and 99mTc sestamibi scan can help localize disease, but they are not useful in the assessment of malignancy potential. Fine needle aspiration (FNA) prior to initial operation is not recommended due to technical difficulty in differentiating benign and malignant disease on cytology specimens and the possible associated risk of tumor seeding from the needle track. Complete surgical resection with microscopically negative margins is the recommended treatment and offers the best chance of cure. Persistent or recurrent disease occurs in more than 50% of patients with parathyroid carcinoma. Surgical resection is also the primary mode of therapy for recurrence since it can offer significant palliation for the metabolic derangement caused by hyperparathyroidism and allows hypercalcemia to become more medically manageable. However, reoperation is rarely curative and eventual relapse is likely. Chemotherapy and external beam radiation treatments have been generally ineffective in the treatment of parathyroid carcinoma. Typically, these patients require repeated operations that predispose them to accumulated surgical risks with each intervention. In inoperable cases, few palliative treatment options exist, although treatment with calcimimetics can effectively control hypercalcemia in some patients. Most patients ultimately succumb to complications of hypercalcemia rather than from tumor burden or infiltration.

Alveolar Soft Part Sarcoma: A Single-Center 26-Patient Case Series and Review of the Literature

Background. Alveolar soft part sarcoma (ASPS) is a rare tumor, and little information is available regarding its clinical features and appropriate treatments. Methods. A retrospective review of 26 consecutive ASPS patients (12 male, 14 female; mean age of 27 years) treated at our institution over 30 years (mean followup; 71 months) was performed. Results. The primary tumor developed in the lower extremity (12), trunk (8), and upper extremity (6), with an average size of 7.2 cm (range, 2–14 cm). The AJCC stage at presentation was IIA (7), III (3), and IV (16). Surgical excision was performed in 20 patients (R0 18, R1 plus radiotherapy 2) without local recurrence. Six patients (stage IIA 3/7, stage III 3/3) later developed metastases after an average period of 28.7 months. The median survival of the 26 patients was 90 months, with overall 5/10-year survival rates of 64%/48%. AJCC stage and tumor size were significant prognostic factors. Significant palliation and slowing of metastasis progression were achieved with gamma knife radiotherapy. Nine patients receiving chemotherapy showed no objective response. Conclusions. ASPS is indolent but has a high propensity for metastasis. Early diagnosis and complete excision of the small primary tumor are essential in the treatment of ASPS.