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   2019| September-December  | Volume 3 | Issue 3  
    Online since December 27, 2019

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Management of Gorham–Stout disease presenting as intractable pleural effusion in an adolescent: A case report and review of literature
TK Jayakumar, Daisy Khera, Vivek Manchanda, Kirtikumar J Rathod
September-December 2019, 3(3):62-65
Gorham–Stout disease (GSD) is a very rare disorder of unknown etiology, characterized by the abnormal proliferation of lymphatic endothelial cells, and also called “vanishing bone disease,” “phantom bone disease,” and “massive osteolysis,” as osteolysis is a characteristic feature. Patients present with a variety of symptoms such as swelling, pain, physical disability and deformity, shortness of breath, and neurological symptoms. It is a progressive disease, and owing to its rarity, the research and treatment options are limited. Our patient is a 15-year-old male, who presented with large mass over abdomen and chest, chylothorax, and osteolysis. This patient was initially treated with chest drains, subcutaneous drains, and sclerotherapy. Eventually, symptoms were worsened. After literature search, the patient was diagnosed with GSD, and sirolimus therapy was started. After 2 months of sirolimus therapy, the lesions subsided and well controlled.
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Cancer and stigma: Present situation and challenges in India
Soumya Swaroop Sahoo, Dinesh Prasad Sahu, Madhur Verma, Pragyan Paramita Parija, Udit Kumar Panda
September-December 2019, 3(3):51-53
India is going through a significant epidemiological transition with the rising of chronic diseases such as cancer. An accompanying aspect is social stigma and negative perceptions regarding cancer. This not only affects cancer care and treatment but also puts a strain on prevention efforts. It is particularly worrisome in a low- and middle-income country like India with low levels of health literacy and the lack of access to cancer care. There have been attempts by the government and positive changes toward mitigating stigma, but a lot needs to be done to address this issue. The family and the community need to be significant players in synergy with the health system in the efforts to minimize the stigma in cancer.
  577 128 -
Chronic myeloid leukemia with isolated central nervous system blast crisis a rare case presentation
Amit Kichloo, Jyoti Poddar, Sakina Mankada, U Suryanarayana
September-December 2019, 3(3):59-61
Most cases of chronic myeloid leukemia (CML) manifest in chronic phase with high granulocyte count. Medical management with imatinib renders complete hematological and cytogenetic remission in almost all patients. Only 5%–10% of the patients progress to accelerated phase and terminal phase, i.e., blast crisis. Blast crisis is defined as the presence of ≥20% blasts in the bone marrow (BM) or peripheral blood, or a large focus of blasts in the BM, or presence of extramedullary infiltration with blast cells. The penetration of imatinib in the central nervous system (CNS) is very poor, and thus, CNS may become a sanctuary site in patients on prolonged treatment with imatinib. The CNS as a site of extramedullary crisis is extremely rare. We report a case of Philadelphia-positive CML on imatinib with cerebrospinal fluid cytology positive and approach to its management.
  577 106 -
Assessment and results of early active nutritional interventions during radiotherapy with or without chemotherapy of head-and-neck cancer
Harikesh Bahadur Singh, Rohini Khurana, Madhup Rastogi, Sambit Swarup Nanda, Satyajeet Rath, Anju Mishra
September-December 2019, 3(3):54-58
Background: Patients of head-and-neck cancer (HANC) undergoing chemoradiotherapy (CRT) are at high risk of malnutrition due to acute effects of radiotherapy (RT). This study was intended to assess weight loss and change in body mass index (BMI) during CRT with active diet counseling and nutritional supplementation. Aims and Objective: The primary end point was to assess loss of weight and BMI during treatment. The secondary end point is assessment of compliance to stipulated treatment time, frequency of nasogastric tube feeding, intravenous support, and duration of hospital admission. Materials and Methods: This was a prospective observational study performed in Radiation Oncology department of a tertiary care center. Consecutive patients of HANC requiring definitive or adjuvant RT (±chemotherapy) were included. Nutritional status assessment and diet counseling were done before start of RT, and further weekly assessment of body weight and BMI was done. Results: Between December 2017 and December 2018, 128 patients were included in this study. Sitewise distribution were oral cavity, oropharynx, hypopharynx, and larynx in 69, 33, 9, and 17 patients, respectively. Weight loss and BMI comparison were done at the start and end of RT (mean weight: 53.86 kg; 95% confidence interval [CI]: ± 1.71, mean BMI: 21.52; 95% CI: ± 0.95 [at the start of RT] and mean weight: 48.30 kg; 95% CI: ± 1.58, mean BMI: 19.18; 95% CI: ± 0.88 [at the end of RT]).There was a significant reduction in weight (P < 0.0001, S) and BMI (P = 0.00034, S), respectively. During the treatment, a total of 14 patients were put on nasogastric tube feeding, and 23 patients were admitted for parenteral nutrition with median hospital stay of 3 days (range: 1–5 days). Conclusions: There is a significant weight loss and change in BMI during CRT in HANC patients. Regular assessment and active nutritional intervention are required in all patients to improve compliance.
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Dermatofibrosarcoma protuberans, lymphedema, and breast cancer: A rare association
Kaalindi Singh, Selvamani Backianathan, Anne Jennifer, DN Susitra, Reena George
September-December 2019, 3(3):66-69
A woman with postmastectomy lymphedema presented with a progressive nodular swelling on the affected limb. The biopsy was reported as dermatofibrosarcoma protuberans (DFSP). There is only one other case in literature, where a truncal dermatofibrosarcoma was reported in association with breast cancer. Both tumors had the P53 mutation. In our patient, the breast cancer and the DFSP were both negative for the P53 mutation and it is possible that chronic lymphedema was causative in the formation of the DFSP. More evidence is needed to understand the etiopathogenesis of DFSP occurring in breast cancer patients.
  421 91 -
Ulcerated duodenal gastrointestinal stromal tumor – Demonstration of an interesting radiological sign (the Torricelli-Bernoulli sign)
Pradosh Kumar Sarangi, Nachiketa Mangaraj, Keshaba Panigrahy, Ankush Jajodia
September-December 2019, 3(3):70-72
The Torricelli-Bernoulli sign is a useful computed tomographic sign seen in ulcerated/necrotic gastrointestinal stromal tumor (GIST) or leiomyosarcoma characterized by the collection of air in the nondependent aspect of larger gastrointestinal cavitating tumors. This sign is based on the physical principle of the law of Torricelli's and Bernoulli's principle. Herein, we describe this interesting imaging sign in an adult male with malignant duodenal GIST with extensive hepatic, peritoneal, omental, and lymph nodes (though rare in GIST) metastasis. The patient succumbed few days after admission.
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