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Background The study was aimed at evaluating prognostic factors and treatment outcomes of pediatric parameningeal rhabdomyosarcoma (PM-RMS) at two major pediatric oncology centers in Pakistan. Methods PM-RMS age 1–16 years diagnosed from 2005 to 2015 at Aga Khan University Hospital and The Indus Hospital were identified. Factors relevant to survival and relapse were analyzed. Results Forty-Six patients were identified. Thirty-three (72%) patients were analyzed. Seventeen (52%) patients were stage III (group-III), 15 (46%) were stage II (group-III) and 1 (3%) was stage IV (group-IV). Twenty-nine (88%) were embryonal. Majority (n = 25, 76%) received vincristine, actinomycin and cyclophosphamide while three (9%) received high-risk protocol. Twenty-three (69.6%) received radiation whereas two (6%) patients each were treated with surgery and surgery + radiation. Improved 3-year overall survival (OS) (77%, P = 0.04) was associated with a dose of 50Gy radiation and (77%, P = 0.02) with baseline nutritional status Z-scores ≥ - 2SD. Age ≥10 years (33%, P = 0.80), stage III disease (33%, P = 0.08) and regional nodal involvement (25%, P = 0.12) showed inferior 3-year event free survival (EFS). Three-year overall survival (OS) was 37% (95% CI: 16%-58%), 3-year (OS) excluding treatment abandonment was 47% (95% CI: 21%-70%) and 3-year event free survival with relapse, deaths and treatment abandonment was 23% (95% CI: 9%-41%) respectively. Conclusion Our study demonstrates improved survival with adequate dose of radiation and nutritional status (Z-scores) ≥ - 2SD. Less advanced disease and lack of nodal involvement correlates well with outcomes. Treatment abandonment remains problematic in developing countries. Retrospective analysis restricted meaningful outcomes, suggesting future collaborative studies.

A retrospective review was conducted In Aga Khan University from January 2013-December 2020 for patients who received Total Skin Electron Beam Therapy (TSEBT) on the management of Mycosis Fungoides (MF). The clinical response rate and disease-free interval (DFI) was evaluated for three patients. All of the patients received a 3600 cGy dose of radiation. Clinical complete remission (cCR) was observed in two out of three patients, while one patient showed clinical partial response. Following the cCR to TSEBT, the DFI of the first patient with stage 1B lasted 30 months, while the second patient with stage IIB remained disease free for five months. TSEBT is an effective treatment for MF disease. It can thus be expected that patients with limited disease in the early stage can acquire a cCR with a longer DFI. The clinical outcomes of our study show concordance with the literature. The patients who had extensive (T3) skin involvement and had refractory diseases showed lower DFI with radiation.

To determine the acute vaginal mucosal toxicity and clinical response of cervical cancer after definitive treatment with external beam radiotherapy (EBRT) and three fractions each of 8 Fray (Gy) high dose rate intracavitary brachytherapy (HDR-BT). Study design Descriptive study. Place and duration Radiation Oncology Section, Department of Oncology, The Aga Khan University Hospital, Karachi, Pakistan from January 2008 till December 2015. Methodology Protocol was formulated for carcinoma cervix to complete treatment in 7 weeks. Patients were treated with chemotherapy and pelvic EBRT to a total dose of 45 Gy/25 fractions, followed by three intracavitary HDR brachytherapy fractions of 8 Gy each. Vaginal toxicity and local clinical response was assessed at the end of treatment, at 4 and 8 weeks. Results A total of 57 patients were treated with HDR brachytherapy and 49 patients were evaluated for assessment of toxicity and response. According to FIGO staging system, two had stage IB2, one had IIA, thirty-six had IIB, seven had IIIB, one had IVA disease and two had IVB with para aortic nodes. Concurrent gemcitabine and cisplatin were given to 26 (46%); whereas, 28 (49%) received concurrent cisplatin alone. Grade III acute vaginal mucosal toxicity was seen in 52 and Grade IV acute vaginal mucosal toxicity was observed in 08 patients. At completion of treatment, 40 patients had complete clinical response, at 4 weeks follow-up, complete regression of disease was found in 3 more and at 8 weeks none had clinical residual disease. Conclusion This regimen of HDR brachytherapy treatments is feasible, efficacious, and well-tolerated for carcinoma cervix in a setup with cost constraints. Long term toxicity and disease control remains to be reported with longer follow-up. Key Words: Carcinoma cervix, High dose rate brachytherapy, Acute toxicity, Local response, External beam radiation therapy, Intracavitary brachytherapy.

Low- and middle-income countries sustain the majority of pediatric cancer burden, with significantly poorer survival rates compared to high-income countries. Collaboration between institutions in low- and middle-income countries and high-income countries is one of the ways to improve cancer outcomes.Patient characteristics and effects of a pediatric neuro-oncology twinning program between the Hospital for Sick Children in Toronto, Canada and several hospitals in Karachi, Pakistan over 7 years are described in this article.A total of 460 patients were included in the study. The most common primary central nervous system tumors were low-grade gliomas (26.7%), followed by medulloblastomas (18%), high-grade gliomas (15%), ependymomas (11%), and craniopharyngiomas (11.7%). Changes to the proposed management plans were made in consultation with expert physicians from the Hospital for Sick Children in Toronto, Canada. On average, 24% of the discussed cases required a change in the original management plan over the course of the twinning program. However, a decreasing trend in change in management plans was observed, from 36% during the first 3.5 years to 16% in the last 3 years. This program also led to the launch of a national pediatric neuro-oncology telemedicine program in Pakistan.Multidisciplinary and collaborative efforts by experts from across the world have aided in the correct diagnosis and treatment of children with brain tumors and helped establish local treatment protocols. This experience may be a model for other low- and middle-income countries that are planning on creating similar programs.

PURPOSE To evaluate and report the frequency of changes in radiation therapy treatment plans after peer review in a simulation review meeting once a week. MATERIALS AND METHODS Between July 1 and August 31, 2016, the radiation plans of 116 patients were discussed in departmental simulation review meetings. All plans were finalized by the primary radiation oncologist before presenting them in the meeting. A team of radiation oncologists reviewed each plan, and their suggestions were documented as no change, major change, minor change, or missing contour. Changes were further classified as changes in clinical target volume, treatment field, or dose. All recommendations were stratified on the basis of treatment intent, site, and technique. Data were analyzed by Statistical Package for the Social Sciences and are presented descriptively. RESULTS Out of 116 plans, 26 (22.4%) were recommended for changes. Minor changes were suggested in 15 treatment plans (12.9%) and a major change in 10 (8.6%), and only one plan was suggested for missing contour. The frequency of change recommendations was greater in radical radiation plans than in palliative plans (92.3% v 7.7%). The head and neck was the most common treatment site recommended for any changes (42.3%). Most of the changes were recommended in the technique planned with three-dimensional conformal radiation therapy (50%). Clinical target volume (73.1%) was identified as the most frequent parameter suggested for any change, followed by treatment field (19.2%) and dose (0.08%). CONCLUSION Peer review is an important tool that can be used to overcome deficiencies in radiation treatment plans, with a goal of improved and individualized patient care. Our study reports changes in up to a quarter of radiotherapy plans.

Some cancers and some cancer treatments can lead to temporary or permanent infertility. Infertility is a special issue which need to be considered and addressed in cancer patients who may face this issue due to their malignancy itself or due to the treatment offered to them. Our team had reviewed contemporary literature and came to the opinion that it is vitally important to establish a specialized board of experts who can have regular board meetings in which all selected cases of cancer patients are discussed who can potentially suffer from some form of temporary or permanent infertility. The relationship between infertility and cancer is being documented in various studies and systemic reviews published lately [1, 2]. Counselling of patients and their next of kin is also an important aspect of this proposed multi-disciplinary team (MDT) Board .Before making any consideration regarding the relevance of the issue of infertility in selected cases of cancer the multidisciplinary team has to develop robust processes in order to make sure that we will not be missing this important problem which our cancer patients may face at the time of diagnosis, treatment or later on as a late sequela of oncological treatment. Practice committee on onco-fertility consortium made clear recommendations highlighting the importance of expert team involvement. The proposals are being published in January 2021 issue of Journal of assisted reproduction [3]. This document provides a valuable insight regarding the establishment healthcare quality processes and their practical implications.

We report a case of a male patient. He presented with nasal obstruction and epistaxis. The MRI of the brain showed a mass in the nasopharynx and enlarged cervical lymph nodes. Besides this, there was an extra-axial, dural-based lesion in brain and subcentimetre nodules in both lungs. He received induction chemotherapy followed by chemoradiation therapy to the primary site and dural-based metastatic deposit. He re-presented with bilateral lower limb weakness. The MRI of the spine showed metastatic deposits within the thoracic cord parenchyma and meningeal deposits at the lumbar region. Palliative radiation was used to treat spinal cord and meningeal metastases. He died a few months later because of systemic disease progression. Considering the rarity of this presentation in nasopharyngeal carcinoma and lack of clear guidelines for standard treatment, we report this case to share our experience of management.

Merkel cell carcinoma is an aggressive non-melanomatous cutaneous tumour of neuroendocrine origin with an increasing incidence in the recent years. It is a tumour of the elderly and immunosuppressed, which most often appears on sun-exposed areas of the body. The clinical features of the cutaneous or subcutaneous lesions hardly contribute to the diagnosis, and, hence, histopathology and immunohistochemistry play a vital role in diagnosis. The latest staging system by the American Joint Committee on Cancer includes non-nodal invasion to adjacent structures i.e. bone, muscle, fascia, or cartilage into the criteria, in additionto size and depth of invasion. The management reliesheavily on a multidisciplinary approach due to rarity of incidence of this disease. According to the international guidelines, surgical management is still the preferred choice. The beneficial role of adjuvant radiotherapy has now been more clearly documented. Data is insufficient to assess whether chemotherapy improves disease-free or overall survival.

Cerebral metastasis as an initial clinical presentation of prostate carcinoma is extremely rare. Usually, patients have widespread metastasis in the body before presenting with brain metastasis. In the absence of extensive metastasis, especially without bony metastasis, only brain metastasis is an unusual presentation of the disease. We report a case of a 59-years-old patient who presented with a lack of concentration and decreased vision. Magnetic resonance imaging (MRI) of the brain revealed a large right parietal-occipital space-occupying lesion. He underwent surgery and the pathological diagnosis of the tumor turned out to be metastatic prostate carcinoma. Further evaluation by a whole-body computed tomography (CT) scan revealed an enlarged prostate with no other metastatic deposit and a mildly raised level of prostate-specific antigen (PSA). It was possible for us to provide this patient with multi-modality treatment with the help of multidisciplinary tumor board meetings. Further studies addressing the biological as well as clinical characteristics of prostate carcinoma with this rare metastatic presentation will help us to define prognostic factors and therapeutic intervention and will help us to understand the basis of this unique presentation without bone metastasis.

Congress of the Pediatric-Radiation-Oncology-Society (PROS) -- JUN 19-22, 2019 -- Bangkok, THAILAND, WOS: 000491350500054, [No abstract available], Pediat Radiat Oncol Soc

Hidradenocarcinoma is a rare and locally aggressive tumor rendering a poor prognosis. Furthermore, very few cases present with nodal metastasis. Diagnosing such an entity, and then differentiating it from a benign counterpart, poses a great challenge to the clinicians. There are no established treatment guidelines for the management of this disease, particularly in patients with nodal involvement. We present a case of a young male who was diagnosed with hidradenocarcinoma of the scalp, along with a neck swelling. A thorough diagnostic evaluation was done with endoscopy, pathological, and radiological investigations. He was successfully treated with resection of the scalp lesion and right-sided neck dissection followed by adjuvant concurrent chemoradiation. He remains free of any local and distant disease after five years of regular follow-up.

A 45-year-old man presented with left-sided facial swelling. Initial excisional biopsy was done somewhere else without prior imaging and it came out to be pleomorphic adenoma; local imaging after biopsy showed mass over the left parotid gland with ipsilateral lymphadenopathy. On examination, there was a well-circumscribed mass with signs of left facial nerve palsy. He underwent left radical parotidectomy with ipsilateral modified radical neck dissection, and per operatively, it came out to be neoplastic lesion. Final histopathology was plasma cell neoplasm of the parotid gland. After surgery, the patient received radiation therapy to the left parotid region on account of uncertain surgical margins. It has been 3 years down the road that there is no evidence of local recurrence or transformation into multiple myeloma. Available evidence reveals that mere surgery for extramedullary plasmacytoma is not enough for local control whenever there is doubt of residual disease consider radiation therapy.

Purpose To report the long-term outcome in lymph nodal–metastatic cervical squamous cell cancer after chemoradiation followed by adjuvant chemotherapy. Patients and Methods Between 2010 and 2013, five patients were diagnosed with advanced cervical cancer with clinically involved para-aortic lymph nodes (ie, International Federation of Gynecology and Obstetrics stage IVB). These patients were treated with concurrent chemoradiation therapy followed by adjuvant chemotherapy. Concurrent chemoradiation consisted of cisplatin given once per week concomitantly with extended-field radiation therapy followed by high-dose-rate brachytherapy. Adjuvant chemotherapy comprised four courses of carboplatin and paclitaxel given every three weeks. The primary outcomes were local and distant failures. Results None of the patients had local recurrence or distal failure after a minimum follow-up time of 3 years. Conclusion Adjuvant chemotherapy after chemoradiation has a probable role in the management of lymph nodal–metastatic cervical cancer.

Medulloblastoma is the most common malignant brain tumour in children and is a major cause of mortality and morbidity, particularly in low- and middle-income countries. It has been risk-stratified on the basis of clinical (age, metastasis and extent of resection) and histological subtypes (classic, desmoplastic and anaplastic). However, recently medulloblastoma has been sub-grouped by using a variety of different genomic approaches, such as gene expression profiling, micro-ribonucleic acid profiling and methylation array into 4 groups, namely Wingless, Sonic hedgehog, Group 3 and Group 4. This new sub-grouping has important therapeutic and prognostic implications. After acute leukaemia, brain tumour is the second most common malignancy in the paediatric age group. The improvement in outcome of acute lymphoblastic leukaemia in low- and middle-income countries reflects the relative simplicity of diagnostic procedures and management. Unlike leukaemia, the management of brain tumours requires a complex multidisciplinary approach, including neuro-radiologists, neurosurgeons with a paediatric expertise, neuropathologists, radiation oncologists and neuro-oncologists. In addition, the equipment required for the diagnosis (magnetic resonance imaging scan, histological, molecular and genetic techniques) and the management (operating room, radiation facilities) is a limiting factor in countries with limited resources. In Pakistan, there are very few centres able to treat children with brain tumours. The current literature review was planned to provide an update on the management of this tumour.

Pediatric radiotherapy is a critical part of pediatric oncology protocols and the quality of the radiotherapy may determine the future quality of life for long-term survivors. Multidisciplinary team decision making provides the basis for high-quality care. However, delivery of high-quality radiotherapy is dependent on resources. This article provides guidelines for delivery of good quality radiation therapy in resource-limited countries based on rational procurement and maintenance planning, protocol development, three-dimensional planning, quality assurance, and adequate staff numbers and training.

Squamous cell carcinoma of uterine cervix is potentially a curable disease; however, many patients treated with definitive chemoradiotherapy develop distant metastases, with few of them having a single metastatic deposit. There are no guidelines for the treatment of patients with oligometastatic cervical cancer. We present a case of a patient with International Federation of Gynecology and Obstetrics (FIGO) Stage IIB squamous cell carcinoma of uterine cervix. She was successfully treated with concurrent chemoradiotherapy with definitive intent. One year later, she developed a solitary pulmonary nodule for which she underwent resection followed by chemotherapy. She is free of any local or distant disease at 5 years of regular follow-up.

To determine the frequency and severity of acute toxicity of pelvic radiotherapy for gynecological cancer.A case series.Department of Oncology, The Aga Khan University Hospital, Karachi, from March 2011 to June 2012.A total of 99 patients with histologically proven uterine and cervical cancer, receiving radiation therapy, were enrolled into the study after informed consent on justification of inclusion and exclusion criteria. Patients were evaluated for the frequency and severity of pelvic radiotherapy’s side effects according to toxicity criteria based on RTOG/EORTC and CTC version 2 criteria at the start, during and at the end of treatment. The data was analyzed by using SPSS version 16.Out of the 99 enrolled patients, 58 (58.6%) had uterine and 41 (41.4%) had cervical cancer. Mean age was 54.54 ± 10.29 years. Thirty-five (35.4%) patients received chemotherapy with RT. Mean RT dose was 60.72 ± 7.15 Gy. The most common gastrointestinal adverse effect was diarrhea in 64 (64.6%) followed by proctitis in 55 (55.5%), nausea in 33 (33.3%) and vomiting in 16 (16.2%) patients. Grade (G) 1 was the most frequently observed severity. The most common hematological toxicity was anemia in 37.8% (n=31/82) {(G1=18 (21.9%), G2=11 (13.4%), G3=2 (2.4%)} followed by thrombocytopenia in 22.8% (21/92) {(G1=16 (17.3%), G2=2 (2.1%), G3=3 (3.2%)} and neutropenia in 21 (21.2%) {(G1=12 (12.1%), G2=5 (5%), G3=3 (3%), G4=1 (1%)}. Urinary toxicity was observed in 49 (49.5%) patients. On stratification, chemotherapy and higher RT dose were strong predictor of increased hematological and upper gastrointestinal toxicity (p0.05) and age60 years for diarrhea (p0.05).The frequency and severity of acute toxicity of pelvic radiotherapy in women with gynecologic cancers was found intermediate to high.

To determine the frequency, demographic characteristics, associated co- morbidities and extraarticular manifestations in patients with rheumatoid arthritis (RA) visiting the rheumatology clinic at a tertiary care hospital, Karachi.A retrospective medical chart review of 4900 patients, who visited the rheumatology clinic at Liaquat National Hospital, from January 2005 to June 2007, was conducted. All patients with RA, of both gender and ages 16 years and above, who fulfilled the 1987 ACR criteria were included. Demographic characteristics, base line co-morbidity and extra-articular manifestations were recorded according to pre-defined criteria.Among 4900 patients, 633 (12.9%) visited the rheumatology clinic with RA, female to male ratio being 4:1. The mean age of onset was 38.5 +/- 12.4 years in females and 44.8 +/- 13.12 years in males. Among the age group 16-29 years females were more affected, whereas greater number of males presented between 50-75 years of age. Co-morbidities were found in 35.38% of RA patients. Cardiovascular disease including hypertension (13.79%) and ischaemic heart disease (6.6%) were the most common co-morbidities. RA factor was positive in 85.05% of the patients with hypertension and 88.09% of the patients with ischaemic heart disease. Extra-articular manifestations were reported in 3.47% of patients. Interstitial lung disease (1.57%) was the most common extra-articular manifestation.Significant proportion of patients with female predominance visited the rheumatology clinic at a tertiary care hospital due to RA. Rheumatoid factor may predict the risk of developing cardiovascular disease in patients with RA. The frequency of extra-articular manifestation was lower than that reported in western population.