Periprosthetic metastases in carcinoma of unknown primary: A rare association.

ABSTRACT: Septic or aseptic loosening may cause bone loss around artificial prosthesis leading to prosthesis failure. This occurrence due to metastatic infiltration of bone or surrounding soft tissues is rare but has been occasionally reported. We report a case of an elderly lady presenting with swelling and pain at the site of previous hemiarthroplasty performed for traumatic injury. On evaluation, she was found to have a lytic femur lesion with a large soft-tissue component around the prosthetic joint. Biopsy suggested a metastatic carcinoma of renal origin, but screening of kidneys did not reveal any primary lesion. She had additional skeletal metastatic lesions but no other primary site was detected either. She was given palliative radiotherapy and systemic therapy (sunitinib) based on the histologic diagnosis of renal cell origin but did not tolerate it. Thereafter, she is continuing on zoledronate every 4 weeks and best supportive management since 4 months from diagnosis.

Neuroendocrine tumour of the lesser omentum.

A man in his early 20s presented to us in the outpatient department with a history of diarrhoea for 4 months. Investigations revealed elevated serum chromogranin levels and an intensely avid lesion in the gastrohepatic ligament in Gallium DOTATATE positron emission tomography (PET). The tumour was excised laparoscopically, and no other lesions were seen. The patient improved clinically and had a normal serum chromogranin level postoperatively. He is currently much improved at the 1year follow-up. We did an extensive workup to look for a primary tumour. It was concluded that it was a de novo tumour arising from the lesser sac. The recommended investigations in case of neuroendocrine tumour (NET) with unknown primary include blood investigations to look for the functional status of the tumour, histopathological examination, including immunohistochemistry, and radiological imaging, which must include a Gallium DOTATATE PET. An isolated NET of the lesser sac has not been reported in the literature.

Delineating and sparing the ileal conduit in adjuvant radiotherapy for bladder cancer with modulated radiotherapy.

Purpose: We undertook a prospective planning study to describe the delineation of ileal conduit (IC) loop on radiotherapy planning computed tomography (RTP CT) scan as an organ at risk (OAR) and its sparing using volumetric modulated arc therapy (VMAT) during adjuvant irradiation of bladder malignancies. Materials and Methods: Fifteen patients with bladder malignancy needing adjuvant radiotherapy postoperatively and having normal renal function underwent delayed phase RTP CT from June 2020 to March 2021, with certain modifications (Foley's catheter through stoma, additional delayed scans). We identified the course of ureters, external stoma, IC, and uretero-ileal (right and left) anastomotic sites. VMAT plans were generated. Results: A step-by-step description is given. Genitourinary OARs include kidneys, ureters, uretero-ileal anastomoses, and IC. The contrast on delayed scan opacifies ureters and IC. IC can be seen three-dimensionally as a structure with two fixed ends (blind proximal end anterior to the right sacroiliac joint and the open distal end over the right anterior abdominal wall in parasagittal location) and a 15-20 cm hanging intraabdominal loop that lies adjacent to the right iliac vessels. For prescription doses (PD) of 50.4 gray and 54 gray, respectively, VMAT plan achieved IC dose maximum to less than PD and V50 lower than 10 cc. Stoma sparing traditionally used as a surrogate for IC sparing is insufficient due to the variable intraabdominal location of IC loop. Conclusions: Delineation of IC as an OAR is feasible with slight modifications in the RTP protocols. VMAT (or other forms of intensity modulated radiation therapy) can help IC sparing and should be considered when it lies in close proximity to target volumes and the risk of additional morbidity is considerable.

Primary retroperitoneal squamous cell carcinoma: a literature review.

PURPOSE: Retroperitoneal squamous cell carcinoma is an extremely rare histological variant of retroperitoneal tumors. The exact etiology and origin of the same is still unknown. To date, only a few case reports have been published in the literature. Due to rarity, standard treatment protocol is not available. The article aims to review the literature and treatment options available for this uncommon entity, based on available data. METHODS: We searched the databases like PubMed, PubMed Central, Scopus, and Google Scholar with the keywords "Squamous cell carcinoma" and "retroperitoneal tumors". Peer-reviewed and recent articles were screened. RESULTS: Seven relevant articles comprising 14 cases were found. Due to the small number of reports, tabulation of treatment details and outcome was done. Like the sarcoma variant, these tumors also present with a large mass in the abdomen and abdominal discomfort. Association with human papillomavirus appears to be the most common factor that gives rise to squamous histology. CONCLUSION: Retroperitoneal SCC is an uncommonly diagnosed entity. Although no specific treatment guidelines exist for this uncommon malignancy; surgery followed by adjuvant or definitive radiotherapy with concurrent chemotherapy (in inoperable cases) seems a feasible option. Multicentre trials should be conducted for establishing definitive treatment strategies for this ailment.

Treatment outcomes of adjuvant radiotherapy in adrenocortical carcinoma - A 13-years experience from a tertiary care centre.

Purpose: Adrenocortical carcinoma (ACC) is a rare and highly aggressive malignant neoplasm, usually diagnosed in advanced stage. Role and efficacy of adjuvant radiotherapy has not been well defined. The objective of this study is to describe the various clinical characteristics and prognostic factors affecting the survival of ACC along with the role radiotherapy on overall survival and relapse free survival. Methods: A retrospective analysis of 30 patients registered between 2007 and 2019 was carried out. The medical records containing clinical and treatment details were analysed. Data was analysed using SPSS 25.0. Survival curves were computed using Kaplan-Meier method. Univariate and multivariate analyses were performed to analyze the prognostic factors affecting the outcome. A p value of less than 0.05 was considered to be statistically significant. Results: The median age of patients was 37.5 years (range, 5-72 years). 20 patients were females. Twenty-six patients had advanced stage (III/IV) disease while only four patients presented in early stage. Twenty-six patients underwent total adrenalectomy. Eighty three percent patients received adjuvant radiation therapy. The median follow up was 35.5 months (range, 7 monthss-132months). The estimated three- and 5-years overall survival (OS) was 67.2% and 23.3%, respectively. Capsular invasion and positive margins were the independent prognostic factors influencing both OS and relapse free survival (RFS). Out of 25 patients who received adjuvant radiation, only three patients had local relapse. Conclusion: ACC is a rare and aggressive neoplasm with majority of patients presenting in advanced stage. Surgical resection with negative margins remains the mainstay of treatment. Capsular invasion and positive margins are independent prognostic factors for survival. Adjuvant radiation reduces the risk of local relapse and is well tolerated. Radiation can be used effectively in adjuvant and palliative settings in ACC.

Increased PSMA Expression in 177 Lu-PSMA-617 Scan in Metastatic Castrate-Resistant Prostate Cancer Patient Treated With PSMA Radioligand Therapy and Enzalutamide : A Potential Game-Changer.

ABSTRACT: Androgen deprivation therapy plays an integral role in the treatment algorithm of advanced prostate cancer. Enzalutamide has shown great benefit in castrate-sensitive as well as resistant prostate cancer. Few studies have shown that enzalutamide can potentially increase the PSMA expression on 68 Ga-PSMA-11 PET/CT imaging in patients with metastatic castrate-resistant prostate cancer. We present an interesting case where addition of short course of enzalutamide resulted in increased localization of 177 Lu-PSMA-617 in metastatic lesions on posttherapy scan pointing to the added benefit of PSMA RLT.

Primary angiosarcoma of the seminal vesicle.

Angiosarcomas are rare but highly aggressive malignancies originating from lymphatic or vascular endothelial cells and may arise from any site in the body. Angiosarcomas of the genitourinary tract, especially of seminal vesicle origin, are extremely rare with only five reported cases. Surgery forms the mainstay of therapy in localised disease while adjuvant therapies are still being refined. We present the case of a 40-year old gentleman who presented with lower urinary tract symptoms and, on evaluation, was found to have a localised angiosarcoma originating in right seminal vesicle and offered laparoscopic resection, adjuvant paclitaxel (12 weekly cycles) and adjuvant radiation therapy (66 gray in 30 fractions). He developed a peritoneal nodular recurrence after 6 months of radiotherapy that was successfully salvaged with excision and metronomic chemotherapy, which he is currently receiving. Localised angiosarcomas need multimodality management despite small size. Attempts should be made for surgical salvage of limited recurrences whenever feasible.

Expanding role of radiotherapy in adenoid cystic carcinoma of the tracheobronchial tree: a new horizon.

BACKGROUND: Primary adenoid cystic carcinomas (ACCs) of central trachea-bronchi system are rare and heterogeneous tumors. Definitive radiotherapy (RT) is the recommended treatment in surgically unresectable or incomplete resection or in the presence of severe comorbidities. OBJECTIVE: To evaluate the clinical features and outcomes of patients with ACC of trachea-bronchi treated with radiotherapy. METHODS: Retrospective medical records review was done in all patients with histologically confirmed ACC of trachea-bronchi between January 2010 and December 2019. Patient disease and treatment characteristics and toxicity data were analyzed. Overall survival (OS), local recurrence-free survival (LRFS), and distant metastasis-free survival (DMFS) were computed using Kaplan-Meier method (log-rank test). RESULTS: Nineteen patients (10 women and 9 men) were included in this analysis with median age of 40 years (range, 14-70). Of these patients, 63.2% (n = 12) presented in stage IV disease. Twelve and three patients received definitive (median dose 67.8 Gy) and adjuvant (median dose 50 Gy) RT, respectively. The median follow-up was 42.5 months (range, 4-120); 15 patients were alive and 4 were dead at that time. Local recurrence or progression was observed in 52.6% and distant metastasis found in 47.3% of patients. The 5-year OS, LRFS, and DMFS for all patients were 81.2%, 52.8%, and 39.6%, respectively. Baseline lymph node involvement showed significant impact on OS (56.3% vs 100%, p = 0.011). Among patients receiving definitive RT, patients with higher RT dose (⩾66 Gy) had significantly better survival outcomes (5-year LRFS: 75% vs 16.7%, p = 0.013). CONCLUSION: Definitive RT is an exemplary treatment for unresectable disease. Higher dose is recommended to improve long-term outcomes.

Leiomyosarcoma of urinary bladder with unusual recurrence in intestinal mucosa and peritoneum: a case report.

BACKGROUND: Leiomyosarcomas of urinary bladder constitute rare malignant sarcomas with very few cases reported in literature. CASE PRESENTATION: Here, we present a case of bladder leiomyosarcoma in a well-preserved female. She failed to respond to standard chemotherapy and had a rapidly downhill course with unusual metastases in anastomotic site and peritoneum soon after surgery. Despite multimodality management including resection of primary and metastatic site, systemic therapy and pelvic radiotherapy, our patient had dismal prognosis with an overall survival of 1.7 years. CONCLUSION: Leiomyosarcomas of bladder are aggressive tumors and have a very poor prognosis; thus, future research should focus on optimizing more effective treatment regimes.

A Systematic Review of Radiation-Related Lymphopenia in Genito-urinary Malignancies.

BACKGROUND: The impact of radiation-related lymphopenia on clinical outcomes has been reported in various solid malignancies such as high grade gliomas, head and neck cancers, thoracic malignancies and gastro-intestinal malignancies but its impact is not clearly known in the context of common genito-urinary (GU) malignancies. METHODOLOGY: To better understand the effect of radiation-associated lymphopenia in prostate and bladder cancer, we undertook this systematic review of clinical studies that have studied radiation-related lymphopenia in GU malignancies. A systematic methodology search of PubMed, Embase, and Cochrane library resulted in 2125 abstracts. Ten studies fulfilled the inclusion criteria which included any prospective, retrospective study or cohort study of prostate, urinary bladder, kidney, ureter, urethra, penile cancer in humans, and radiation should be part of treatment and intent has to be in definitive or adjuvant settings. Finally the study should have data on radiation-related lymphopenia. RESULTS: Four studies reported on the cancer-specific outcomes related to the lymphopenia. The incidence of low lymphocyte counts were documented in all the studies. Three studies analyzed the factors associated with the Lymphocyte depletion. Pooled incidence of severe lymphopenia was 29.25% and mild to moderate lymphopenia was 60.75%. Bone marrow volume receiving 40 Gy was associated with the incidence of lymphopenia. CONCLUSION: One-third of the patients suffer from severe lymphopenia after radiation in prostate and bladder cancer. There are no clear data to support the correlation between severe lymphopenia and disease outcomes. Bone marrow dosimetry can affect the incidence and severity of lymphopenia. There is need of prospective datasets to identify the impact of radiation-related lymphopenia in GU malignancies focusing on long-term side effects, recurrence rates, and overall survival.

Dosimetric comparison between interstitial brachytherapy and volumetric-modulated arc therapy for tumor bed boost in breast cancer.

PURPOSE: To dosimetrically compare high-dose-rate interstitial brachytherapy (HDR-BT) with volumetric-modulated arc therapy (VMAT) for tumor bed boost, following breast conservative treatment. MATERIAL AND METHODS: 50 patients with early-stage breast cancer who underwent breast conservation surgery, followed by either HDR-BT (n = 25) of 15 Gy in 6 fractions over a period of 3 days, or VMAT dose of 16 Gy in 8 fractions (n = 25) for tumor bed boost, were retrospectively reviewed. All patients received whole breast irradiation of 46 Gy in 23 fractions. Dosimetric parameters for organs at risk (OARs), including ipsilateral and contralateral lungs, heart, contralateral breast, skin, and ribs, were evaluated with the help of dose-volume histograms (DVH). RESULTS: Heart sparing was similar in both modalities (left-sided breast irradiation, HDR-BT D2cc 20.5% vs. VMAT 30.2%, p-value = 0.243; right-sided breast irradiation, D2cc 6.5% vs. 4.4%, p-value = 0.165). Left-sided cases received higher dose to heart compared to right-sided patients. Interstitial brachytherapy resulted in significantly less dose to contralateral breast (D2cc 4.3% vs. 9.6%, p-value < 0.0001), ipsilateral lung (D2cc 27.6% vs. 73.2%, p-value < 0.0001), contralateral lung (D2cc 4.2% vs. 14.5%, p-value < 0.0001), ribs (D2cc 24.1% vs. 41.2%, p-value < 0.0001), and skin (D2cc 77.3% vs. 95%, p-value < 0.0001). CONCLUSIONS: HDR-BT-based tumor bed boost irradiation results in significantly lower doses to most organs at risk with similar heart sparing compared to VMAT.

Recurrent angiosarcoma of scalp with opsoclonus myoclonus syndrome: role of salvage treatment.

Cutaneous angiosarcoma is a type of rare and locally aggressive malignancy requiring individualised treatment owing to paucity of randomised trials. We present the case of a middle-aged cancer survivor with locally advanced angiosarcoma of scalp managed with surgery, radiotherapy, chemotherapy and targeted therapy over a course of 6 years for two recurrences. The first recurrence was preceded by opsoclonus myoclonus syndrome, a type of paraneoplastic neurological syndrome (PNS), rarely reported in sarcomas. The second recurrence had a rapid clinical course, which led to a therapeutic dilemma of best supportive care versus active management. A trial of weekly paclitaxel was started that was continued for a total of 12 cycles with good objective clinical response. Presently, he is tolerating maintenance pazopanib well and is symptom free for 6 months. In cutaneous angiosarcoma patients, PNS may be a harbinger of recurrence and aggressive, multimodality treatment helps prolong survival.

The patient-reported outcome measures in oropharyngeal, laryngeal and hypopharyngeal cancer patients treated with Volumetric Modulated Arc based simultaneous integrated boost radiotherapy.

OBJECTIVE: To assess the change in the quality of life (QOL) in head and neck cancer patients treated with Simultaneous Integrated Boost (SIB) by Volumetric Modulated Arc Therapy (VMAT) technique. METHODS: Thirty patients with localised head and neck cancers (Stage II- IVa) were treated with VMAT and SIB technique. The three-dose levels prescribed were 68.2 Gy at 2.2 Gy/fraction, 62 Gy at 2 Gy/fraction and 55.8 Gy at 1.8 Gy/fraction to the high, intermediate and low-risk volumes respectively. Concurrent chemotherapy with cisplatin 100 mg/m2 was administered once in three weeks. Acute toxicities were evaluated and scored according to the RTOG grading system. Quality of life (QOL) was assessed using European Organization of Research and Treatment of Cancer (EORTC) QLQC30 and HN35 questionnaires at baseline and in three instances (immediately, one month and three months after the radiotherapy). RESULTS: Out of the total 30, 80% patients had a complete response (CR) at the median follow up of 12 months, while three patients died because of progression, and the remaining 3 had stable disease. All planning objectives were achieved for organs at risk and planning target volume(PTV). There was a statiscally significant(p value < 0.001) reduction in global quality of life scores at the end of treatment when compared to baseline scores, but by three months, there was the return in the QOL scores in most scales similar to the baseline value. CONCLUSION: VMAT based Simultaneous boost radiotherapy is a feasible and safe strategy in terms of toxicity profile with an acceptable transient change in the quality of life and allows a faster return to baseline quality of life.

Synchronous splenic metastases from squamous cell carcinoma of oesophagus: a case report and review of literature.

Synchronous splenic metastases from oesophageal squamous cell carcinomas are extremely rare. Most of the cases of splenic metastases reported in the literature are mainly metachronous and occur usually from adenocarcinoma primary. The treatment options range from splenectomy to palliative chemotherapy with standard doses in fit individuals. However, in cases with poor performance status, the management is often the best supportive care only due to the fear of tolerance and toxicities with standard dose chemotherapy. Herein, we report a case of squamous cell carcinoma of the distal thoracic oesophagus in a poorly fit elderly male diagnosed with synchronous splenic metastases and successfully treated with palliative chemotherapy with reduced flat doses and radiotherapy with no significant toxicities.

The Effect of High Dose Rate Interstitial Implant on Early and Locally Advanced Oral Cavity Cancers: Update and Long-Term Follow-Up Study.

Introduction Brachytherapy, with or without external beam radiation therapy (EBRT), can be an alternative to surgery for organ preservation in early and locally advanced oral cavity cancers. This study aims to evaluate the effect of high dose rate (HDR) interstitial brachytherapy on early and locally advanced squamous cell carcinoma (SCC) of the oral cavity when used alone or as a boost to EBRT. Methods A total of 125 patients with histologically proven stage T1-3/N0-1 SCC of the oral cavity were included in the study. A total of 15 patients with stage I disease received an interstitial implant dose of 3,850 cGy at 350 cGy per fraction, two fractions a day. Another 53 patients had stage II, and 57 patients had stage III disease; these patients received EBRT of 50 Gy in 25 fractions along with an HDR brachytherapy boost of 21 Gy in seven fractions of 3 Gy per fraction twice daily. The stage III patients also received concurrent chemotherapy with injections of cisplatin (70 mg/m2) given every three weeks for three days in divided doses. All node-positive patients received a boost to the node of up to 64 Gy by external beam radiation. Disease response rates, five-year disease-free survival rates, and toxicities were analyzed. Results The median follow-up was 60 months. Among the patients, 103 (82.4%) had a complete response, while 22 (17.6%) had residual disease and were referred for surgical salvage. The five-year disease-free survival was 100% in stage I, 83% in stage II, and 77.2% in stage III; 4% of patients developed grade 3 acute skin toxicity and 23.2% developed acute grade 3 mucositis. Eleven patients died during the follow-up period. Two patients died due to myocardial infarction but had achieved a complete tumor response. One patient had pulmonary tuberculosis and died due to fulminant infection after three years of disease-free survival period. One patient developed a second primary in the brain stem that presented with quadriplegia and expired. Seven patients died due to the progression of the initial disease. Conclusions Proper brachytherapy technique and meticulous planning can minimize the toxicity while providing better tumor control and achieve high local control rates. Brachytherapy, with or without EBRT, can be a surrogate to surgery in early oral cavity cancers as it can achieve organ preservation while providing good functional outcomes.

Real world clinical outcomes of adjuvant sequential chemoradiation in patients with gallbladder carcinomas with poor performance status.

PURPOSE: The purpose of the study is to analyze the overall survival, relapse-free survival, and relapse patterns of adjuvant sequential chemoradiation for gallbladder cancers after curative resection in patients with poor performance status. MATERIALS AND METHODS: We retrospectively reviewed clinical records of gallbladder patients with pathologic stage T2-4 or node positivity treated with sequential chemoradiation at our institute between January 2015 and January 2019. Sequential chemoradiotherapy protocol consisted of six cycles of gemcitabine 1,000 mg/m2 and oxaliplatin 100 mg/m2 administered every 2 weekly and postoperative radiation therapy (45 Gy in 25 fractions over 5 weeks) by three-dimensional conformal technique. RESULTS: A total of 36 patients were included. The median overall survival and relapse-free survival was 26 months (95% confidence interval [CI], 21.4-30.5) and 21 months (95% CI, 11.8-30.1), respectively. The 2-year overall and relapse-free survival rates were 55.1% (95% CI, 37.9%-72.3%) and 44.7% (95% CI, 27.5%-61.9%), respectively. Locoregional, systemic, and combined recurrence were noted in 2 (5.5%), 14 (38.8%), and 3 (8.3%) patients, respectively. On univariate analysis, tumour grading significantly influenced relapse free survival; nodal stage and overall stage demonstrated a statistically significant influence on overall survival (p < 0.05) with a trend towards significance for lymphovascular invasion. On multivariate analysis, no significant factors were found. Grade 3 and 4 haematological adverse events were observed only in 2 (5.5%) with chemotherapy. No grade 3 and 4 adverse events were observed due to radiation therapy. CONCLUSION: Sequential chemoradiation is feasible and tolerable with acceptable efficacy in the adjuvant setting in patients unfit for concurrent chemoradiotherapy.

Measurement of Cervical Regression and Optimizing Brachytherapy Schedule Concurrently with External Beam Radiation Therapy in Cervical Carcinoma.

Introduction This study aimed to measure cervical regression during external beam radiation therapy (EBRT) and optimize the scheduling of brachytherapy concurrently with EBRT. Methods Fifty consecutive patients with carcinoma of the cervix stage IIA to IIIB received concurrent chemoradiotherapy with weekly Cisplatin 40 mg/m2. Cervical regression was evaluated using serial CT scans obtained before and during concurrent chemoradiotherapy (on the third, fourth, and fifth weeks). High dose rate brachytherapy was introduced after 30Gy of EBRT. A total of 25.5Gy in three fractions were delivered during the third, fourth, and fifth weeks of EBRT. Cervical volumes were recorded from the CT scan for cervical regression. Results The mean cervical volume at baseline (i.e., before the start of treatment) was 85.53 cubic centimetres (cc). The mean cervical volumes at the end of the third, fourth, and fifth week were 28.95cc, 24.92cc, and 21.80cc, respectively. The mean cervical regression from baseline to the end of the third, fourth, and fifth week was 60%, 65%, and 69%, respectively. The time for 50% cervical regression was calculated to be 18 days and occurred around 27Gy of EBRT. Conclusion More than 50% of cervical regressions occur at the end of the third week (i.e., after delivery of 30Gy of EBRT), so it is optimal to introduce brachytherapy at the end of the third week. A conventional point-based plan can cover the high-risk clinical target volume (HRCTV) if the volume is <25cc, but an HRCTV >25cc may be well covered with optimization or a combination of intracavitary and interstitial brachytherapy.

Gastric Adenocarcinoma in a 16-Year-Old Female.

BACKGROUND: Primary gastric adenocarcinoma commonly occurs in the elderly in the sixth to seventh decade of life but is exceedingly rare in children and adolescents, accounting for only 0.05% of pediatric gastrointestinal malignancies. Because of the nonspecific clinical presentation, gastric adenocarcinomas in children and adolescents are often metastatic when they are discovered. CASE REPORT: We describe a case of gastric adenocarcinoma in a 16-year-old female who was diagnosed early and successfully treated with surgery and chemoradiation by volumetric modulated arc technique. CONCLUSION: The possibility of gastric adenocarcinoma should be considered in children and adolescents with persistent nonspecific symptoms of vomiting and upper abdominal pain so that the diagnosis can be made at early stages and radical treatment can be offered.