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Carcinoma en cuirasse is a type of cutaneous metastasis from different malignancies. En cuirasse although is a rare entity, most commonly occurs in cases of recurrent breast carcinoma after surgery or very rarely months or years after diagnosis of primary tumor. We report a rare case of neck carcinoma en cuirasse in a 47-year-old Indian female who is a known case of recurrent carcinoma left breast postmodified radical mastectomy, chemotherapy, and radiotherapy referred for fluorodeoxyglucose positron emission tomography-computed tomography following another six cycles of chemotherapy for response assessment.
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We describe a rare occurrence of bilateral acute severe sensorineural hearing loss in a middle-aged man that heralded the diagnosis of metastatic gastric cancer.
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OBJECTIVE: PSMA expression is seen in many solid tumours in addition to prostate cancer and several studies and case reports have shown PSMA expression and 68Ga-PSMA imaging of hepatocellular carcinoma (HCC). Our prospective study evaluates the role of 68Ga-PSMA in HCC patients and compares it to conventional imaging (CE-CT/MRI). METHODS: Patients with radiologically and/or histopathologically confirmed HCC were included and all had undergone serum alpha-fetoprotein (S.AFP) assessment as well as CE-CT/MRI prior to PSMA PET/CT. Acquired whole-body PET/CTs were analysed both visually and quantitatively by two experienced nuclear medicine physicians. RESULTS: Forty-one (41) patients (36 male; 5 female) with known HCC and a mean age of 53.9 ± 10.9 years underwent 68Ga-PSMA PET/CT. All patients had lesions on conventional imaging but only 38/41 patients showed 68Ga-PSMA uptake. Conventional imaging revealed 18 patients with single lesions, all of which were tracer avid. Twenty-three (23) of 41 patients had multifocal (> 2) hepatic lesions on CE-CT/MRI of which 3 patients showed no 68Ga-PSMA uptake, 7 showed tracer uptake in a single lesion only and 13 patients had multifocal tracer avid lesions. There was no correlation observed between S. AFP level and tumour SUVmax on 68Ga-PSMA PET/CT. CONCLUSION: 68Ga-PSMA PET/CT imaging of HCC may complement conventional imaging and identify patients for potential theranostic intervention.
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Carcinoma Hepatocelular , Isótopos de Galio , Neoplasias Hepáticas , Neoplasias de la Próstata , Humanos , Masculino , Adulto , Persona de Mediana Edad , Carcinoma Hepatocelular/diagnóstico por imagen , Carcinoma Hepatocelular/terapia , Tomografía Computarizada por Tomografía de Emisión de Positrones/métodos , Estudios Prospectivos , alfa-Fetoproteínas , Neoplasias Hepáticas/diagnóstico por imagen , Neoplasias Hepáticas/radioterapia , Radioisótopos de Galio , Neoplasias de la Próstata/patologíaRESUMEN
BACKGROUND: Despite the acknowledged diagnostic detection rate of prostate-specific membrane antigen (PSMA) positron emission tomography (PET) imaging in prostate cancer, little is known about the quality of evidence, particularly focusing on prospective studies. Most systematic reviews are based on retrospective reports. RATIONALE AND OBJECTIVES: To conduct systematic review and meta-analysis of prospective studies reporting the diagnostic detection rate of PSMA PET (computed tomography (CT) and MR) for the detection of biochemically recurrent metastatic prostate cancer. MATERIALS AND METHODS: We systematically searched PubMed, MEDLINE, Embase, and Scopus, from database until March 1, 2023 for randomized controlled trials and prospective studies using PSMA PET imaging in prostate cancer. The primary endpoint was to assess diagnostic detection rate of PSMA PET imaging in the detection of recurrent prostate cancer in men with biochemical relapse following radical treatment. We calculated the pooled overall diagnostic detection rate with 95% CI using a random-effects model and assessed the heterogeneity between the studies including risk of biases estimation. RESULTS: A total of 6800 patients from 32 articles were included in this study. The overall detection rate of PSMA PET for prostate cancer was 0.67 (95% CI, 0.63, 0.71). For histologically confirmed lymph nodes, the PPV from 13 prospective studies containing 1496 patients was 0.96 (95% CI, 0.93, 0.99). We performed a subgroup analysis of PSMA PET detection rates according to categorically grouped Prostate Specific Antigen (PSA) values of 0-0.5, 0.5-1.0, 1.0-2.0, and >2.0 ng/ml and obtained detection rates of 0.44, 0.63, 0.82, and 0.94, respectively. The detection rate of 18F PSMA was better in men with a PSA between 1 ng/ml and 2 ng/ml in comparison to 68Ga PSMA (0.91 with 95% CI 0.81-0.99 vs. 0.79 with 95% CI 0.73, 0.85). CONCLUSION: PSMA PET imaging provides a good detection rate for the metastatic recurrence of prostate cancer in men with biochemical relapse following radical treatment. The detection rate improves significantly above a serum PSA value of 1 ng/ml. The diagnostic detection rate of 18F-PSMA is best at PSA values between 1 and 2 ng/ml, in comparison to 68Ga PSMA. This conclusion is heavily biased, further research needs to focus on better methodology to minimize the risk of biases.
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Antígeno Prostático Específico , Neoplasias de la Próstata , Masculino , Humanos , Estudios Prospectivos , Estudios Retrospectivos , Recurrencia Local de Neoplasia/diagnóstico por imagen , Neoplasias de la Próstata/diagnóstico por imagen , Neoplasias de la Próstata/terapia , Neoplasias de la Próstata/patología , Tomografía de Emisión de Positrones , Tomografía Computarizada por Tomografía de Emisión de Positrones/métodos , RecurrenciaRESUMEN
PURPOSE OF STUDY: 18F-FDG PET/CT plays a major role in diagnosis and staging of head and neck cancer; however, FDG has lower uptake in adenoid cystic carcinoma (AdCC). Prostate-specific membrane antigen (PSMA) expression is found to be associated with endothelial cells or tumor neovasculature in malignant AdCC and salivary duct carcinoma. Thus, present study is aimed to compare the role of 68Ga-PSMA and 18F-FDG PET/CT in patients with primary and/or metastatic AdCC. MATERIALS AND METHODS: Histopathologically proven AdCC patients were intravenously injected with 370 MBq (10 mCi) of 18F-FDG and 111-185 MBq (3-5 mCi) of 68Ga-PSMA. Images were acquired at 60 and 45 minutes postinjection for 18F-FDG and 68Ga-PSMA, respectively, on dedicated PET/CT scanners. Visual and semiquantitative analyses of PSMA expression in regional and metastatic sites were performed by 2 experienced nuclear medicine physicians. RESULTS: Seventeen patients (7 men, 10 women) having mean age of 44 ± 14.19 years were prospectively included in the study. Of 17 patients, FDG PET/CT was performed in only 14 (82%) patients. PSMA and FDG uptakes were seen at the primary site in 16 (94%) and 13 (93%) patients, respectively, whereas 1 patient was postradical tumor excision. Lung lesions (n = 7) and lymph nodes (n = 5) were detected on both FDG and PSMA PET scans. However, cerebellar and meningeal metastasis (n = 1, 6%) and bony lesions (n = 2, 12%) were detected only on PSMA PET/CT but not visualized on FDG PET/CT scan. CONCLUSIONS: PSMA may have theranostic importance in unresectable or metastatic AdCC, besides having a role in staging/restaging.
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Carcinoma Adenoide Quístico , Neoplasias de la Próstata , Masculino , Humanos , Adulto , Persona de Mediana Edad , Tomografía Computarizada por Tomografía de Emisión de Positrones/métodos , Fluorodesoxiglucosa F18 , Carcinoma Adenoide Quístico/diagnóstico por imagen , Estudios Prospectivos , Células Endoteliales/patología , Tomografía Computarizada por Rayos X , Radioisótopos de Galio , Tomografía de Emisión de Positrones , Ácido Edético , Neoplasias de la Próstata/patologíaRESUMEN
Purpose: Hereditary tumor syndrome Von Hippel-Lindau (VHL) disease is characterized by various benign and malignant tumors that are known to express somatostatin receptors (SSTR). We evaluated the role of 68Ga-DOTANOC PET/CT scan in patients with positive germline mutation of the VHL gene, presented initially or on follow-up, for the detection of recurrent or synchronous/metachronous lesions. Methods: Fourteen patients (8 males; 6 females) with mean age 30 ± 9.86 years were retrospectively analyzed, were tested positive for VHL on gene dosage analysis, and underwent 68 Ga-DOTANOC PET/CT scan for disease evaluation. The number and site of lesions were determined. The tracer uptake was analyzed semi-quantitatively by calculating the maximum standardized uptake values (SUVmax) of lesion. Results: Four of the 14 patients underwent scan for initial diagnosis as baseline, 6 patients for post-therapy disease status, and 4 patients for initial diagnosis as well as follow-up evaluation of the disease. A total of 67 lesions were detected in 14 patients. The sites of lesions were cerebellar/vertebral/spinal (17; mean SUVmax = 7.85); pancreatic neuroendocrine tumor (NET) (11; mean SUVmax = 20.64); retina (3; mean SUVmax = 10.46); pheochromocytoma (10; mean SUVmax = 16.32); paragangliomas (3; mean SUVmax = 10.65); pancreatic cyst (9; mean SUVmax = 2.54); and renal cyst (8; mean SUVmax = 1.56) and miscellaneous lesions constituted 6 lesions. Conclusion: Our results show that 68 Ga-DOTANOC PET/CT may be a useful modality for screening and follow-up of associated tumors in patients with germline gene mutation for VHL. It can be used as a one-stop imaging modality for VHL patients and may substitute for separate radiological investigations, making it more convenient for patients in terms of time and cost.
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Paraneoplastic neurological syndromes are immune-mediated neurological attacks triggered by malignancies. They are commonly associated with lung, breast, thymus, gynaecological and haematological malignancies. We report a case of a male patient in his late 40s with paraneoplastic encephalomyelitis due to a colonic adenocarcinoma emphasising a low threshold for extensive cancer evaluation in all subacutely presenting neurological syndromes. We also emphasise that the absence of a positive onconeural antibody does not preclude the diagnosis of a paraneoplastic syndrome.
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Carcinoma , Neoplasias del Colon , Síndromes Paraneoplásicos del Sistema Nervioso , Síndromes Paraneoplásicos , Masculino , Humanos , Neoplasias del Colon/complicaciones , Neoplasias del Colon/diagnóstico , Síndromes Paraneoplásicos del Sistema Nervioso/diagnóstico , Síndromes Paraneoplásicos del Sistema Nervioso/etiología , Síndromes Paraneoplásicos/diagnóstico , Síndromes Paraneoplásicos/etiologíaRESUMEN
PURPOSE: Patients with Germ cell tumours (GCT) are at risk of long-term toxicities due to multimodality therapy. It is debatable whether there is an impact on the quality of life(QoL) of GCT survivors. METHODS: A case-control study was conducted at a tertiary care centre in India, using the EORTC QLQ C30 questionnaire, to compare the QoL between GCT survivors(disease free > 2 years) and healthy matched controls. A multivariate regression model was used to identify factors affecting QoL. RESULTS: A total of 55 cases and 100 controls were recruited. Cases had a median age of 32 years (interquartile range, IQR 28-40 years), ECOG PS of 0-1(75%), advanced stage III (58%), chemotherapy (94%) and 66% were > 5 years from diagnosis. The median age of controls: 35 years (IQR 28-43 years). A statistically significant difference was seen for emotional (85.8 ± 14.2 vs 91.7 ± 10.4, p 0.005), social(83.0 ± 22.0 vs 95.2 ± 9.6, p < 0.001) and global scales (80.4 ± 21.1 vs 91.3 ± 9.7, p < 0.001). Cases had more nausea and vomiting(3.3 ± 7.4 vs 1.0 ± 3.9, p 0.015), pain(13.9 ± 13.9 vs 4.8 ± 9.8, p < 0.001), dyspnea(7.9 + 14.3 vs 2.7 ± 9.1, p 0.007), and appetite loss(6.7 ± 14.9 vs 1.9 ± 7.9, p 0.016) and greater financial toxicity(31.5 ± 32.3 vs 9.0 ± 16.3, p < 0.001). Adjusting for age, performance status, BMI, stage, chemotherapy, RPLND, recurrent disease, and time since diagnosis, no predictive variables were significant. CONCLUSION: There is a detrimental impact of history of GCT in long term survivors of GCT.
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Objective The incidence of inoperable hepatocellular carcinoma (HCC) with/without malignant portal vein thrombosis (PVT) is increasing in India for the last decade; thus, Bhabha Atomic Research Centre (BARC), Mumbai, India, developed diethydithiocarbamate (DEDC), a new transarterial radionuclide therapy (TART) agent. 188 Re-N-DEDC lipiodol is an emerging radiotherapeutic agent for inoperable HCC treatment due to its simple and onsite labeling procedure, cost-effectiveness, and least radiation-induced side effects. This study aimed to evaluate in-vivo biodistribution and clinical feasibility of 188 Re-N-DEDC lipiodol TART in HCC and optimization of labeling procedure to assess post-labeling stability and radiochemical yield of labeled lipiodol with 188 Re-N-DEDC complex. Materials and Methods DEDC kits were obtained as gift from BARC, Mumbai. Therapy was given to 31 HCC patients. Post-therapy planar and single-photon emission computed tomography/computed tomography (SPECT/CT) imaging were performed to see tumor uptake and biodistribution. Clinical feasibility and toxicity were decided by Common Terminology Criteria for Adverse Events version 5.0 (CTCAE v 5.0). Statistical Analysis Descriptive statistics was done for data using SPSS v22. Values was expressed as mean ± standard deviation or median with range. Results Post-therapy planar and SPECT/CT imaging showed radiotracer localization in hepatic lesions. Few patients showed lungs uptake due to hepato-pulmonary shunt (lung shunt < 10%). Maximum clearance was observed through urinary tract with very less elimination through hepatobiliary route due to slow rate of leaching of tracer. No patient showed myelosuppression or any other long-term toxicity over median follow-up of 6 months. Mean overall % radiochemical yield of 188 Re-N-DEDC lipiodol was 86.04 ± 2.35%. The complex 188 Re-N-DEDC was found to be stable at 37°C under sterile condition over a period of 1 hour without any significant change on the % radiochemical purity (90.83 ± 3.24%, 89.78 ± 3.67%, 89.22 ± 3.77% at 0, 0.5, 1 hours, respectively). Conclusion Human biodistribution showed very high retention of radiotracer in hepatic lesions with no long-term toxicity with this therapy. The kit preparation procedure is ideally suited for a busy hospital radio-pharmacy. By this procedure, 188 Re-N-DEDC lipiodol can be prepared in high radiochemical yield within a short time (â¼45 minutes). Thus, 188 Re-N-DEDC lipiodol can be considered for TART in advanced and/or intermediate HCC.
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ABSTRACT: Renal cell carcinoma (RCC) accounts for ~2% of global cancers and deaths. Survival depends on initial staging and shows poor survival rate in metastatic disease. CT and MRI are used for evaluating RCC, and PET/CT is used for metastatic disease assessment. We report a case of RCC, where both 18F-FDG and 68Ga-PSMA PET/CT showed increased uptake in liver metastatic lesions; however, a subhepatic peritoneal deposit showed uptake only in PSMA. Also, liver lesions were seen better in PSMA owing to lesser background uptake, suggesting a possibility of 68Ga-PSMA being a potential tracer for RCC evaluation.
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Carcinoma de Células Renales , Neoplasias Renales , Humanos , Carcinoma de Células Renales/diagnóstico por imagen , Carcinoma de Células Renales/patología , Tomografía Computarizada por Tomografía de Emisión de Positrones , Fluorodesoxiglucosa F18 , Neoplasias Renales/diagnóstico por imagen , Neoplasias Renales/patología , Radioisótopos de GalioRESUMEN
ABSTRACT: We present the case of a 60-year-old man who was being evaluated for 2 intra-abdominal masses. The masses were tracer avid on 68 Ga-DOTANOC PET/CT and were suggestive of neuroendocrine tumor. Histopathology, however, confirmed the masses to be hemangiopericytoma. Hemangiopericytoma comes under the umbrella of solitary fibrous tumors, a rare tumor group arising from the mesenchymal cells. Solitary fibrous tumors commonly arise from the pleura but can occur anywhere in the body. Intra-abdominal hemangiopericytoma are extremely rare and should be kept in mind as one of the differential diagnoses for 68 Ga-DOTA peptide-avid tumors.
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Hemangiopericitoma , Tumores Neuroendocrinos , Compuestos Organometálicos , Tumores Fibrosos Solitarios , Masculino , Humanos , Persona de Mediana Edad , Tomografía Computarizada por Tomografía de Emisión de Positrones , Tumores Neuroendocrinos/patología , Hemangiopericitoma/patologíaRESUMEN
ABSTRACT: Langerhans cell histiocytosis is a disorder of the mononuclear phagocytic system. Virtually any organ can be involved, the bone, skin, lungs, and pituitary gland being the most common. We describe the case of a 30-year-old man who presented with painless neck swelling. 18 F-FDG PET/CT showed bilateral bulky parotid and submandibular glands with increased metabolic activity. Histopathology confirmed the diagnosis of Langerhans cell histiocytosis. To our knowledge, there has been no description of 18 F-FDG PET/CT findings in Langerhans cell histiocytosis of salivary glands in literature. We aim to aid in the diagnosis and management of this rare presentation.
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Fluorodesoxiglucosa F18 , Histiocitosis de Células de Langerhans , Masculino , Humanos , Adulto , Tomografía Computarizada por Tomografía de Emisión de Positrones , Radiofármacos , Glándula Submandibular/patología , Histiocitosis de Células de Langerhans/patologíaRESUMEN
Solitary fibrous tumor (SFT) is a rare tumor of mesenchymal origin that most commonly involves the pleura but can be found anywhere in the body. SFT can range from indolent benign tumors to aggressive malignant tumors, and pre-operative diagnosis is based mainly on imaging. In this case report, we describe an extremely rare case of SFT of left maxilla on F-18 FDG PET-CT and Ga-68 DOTANOC PET-CT. Very few cases of PET-CT findings in SFT are reported in literature; and to our knowledge, none involving the maxilla. By highlighting the findings on F-18 FDG and GA-68 DOTANOC PET-CT, we aim to further add on to the role of both the tracers in the diagnosis and management of this tumor group.
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We plan to evaluate the various variables associated with the complications of thyroidectomy performed at our department in the last 5 years. Medical records of the patients who underwent thyroidectomy during 2014-2018 were collected. Complications of hypocalcemia and recurrent laryngeal nerve palsy were analysed in terms of the demography, cytopathology and the extent of surgery. Student's t-test, Mann-Whitney U-test, Fisher exact test and chi square test were applied to look for any significant associations. P value < 0.05 was considered significant. 123 patients were analysed (87 females, 38 males). Mean age was 38.3 years (range 11-71 years). Most common cytopathology was papillary carcinoma thyroid (Bethesda VI) - 43/123 (35%). 107 of these 123 patients underwent primary surgery, 10 underwent revision surgery while 6 underwent completion thyroidectomy. Seven patients incurred RLN palsy out of which 3 were temporary. RLN palsy was seen in only malignant cases (p < 0.05). Incidence was higher in T4a stage (p < 0.05). However, it had no association with a simultaneous central or lateral neck dissection. Hypocalcemia was seen in 22 patients (17.8%), out of whom 9 patients developed permanent hypocalcemia. It was seen significantly higher in patients undergoing central neck dissection (p < 0.05) and in malignant thyroid lesions (p < 0.05). Gender, age and the cytopathology had no bearing on RLN palsy and hypoparathyroidism. Malignant thyroid lesions had a significantly higher incidence of RLN palsy and hypoparathyroidism. A thorough anatomical knowledge can reduce the incidence of these complications.
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Aim: To study the role of check point inhibitors (CPI) in sarcoma and gastrointestinal stromal tumors. Materials & methods: Retrospective data of 15 patients diagnosed with advanced sarcoma or gastrointestinal stromal tumors and treated with CPI. Results: 3/14 patients (21.4%) responded to treatment with a disease control rate of 42.8% (6/14). After a median follow-up of 14 months (range: 2-24 months), 11 (73.3%) patients progressed, the median progression-free survival was 4 months (95% CI: 1.7-6.3) and median overall survival was 14 months (95% CI: 2.6-25.7). Only one patient experienced a grade IV adverse event. Conclusion: Our data represent the first real-world application of CPI in sarcoma from India. We believe that CPI should be further evaluated in clinical trials.
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OBJECTIVE: Tc-99m methyl diphosphonate (MDP) bone scintigraphy is used to assess disease activity in rheumatoid arthritis (RA). Somatostatin receptor (SSTR) expression in RA has been reported previously. SSTR-based PET/computed tomography (CT) may be superior to bone scintigraphy to know disease extent and in locating inflammatory joints that can be further targeted with peptide receptor radionuclide therapy thereby opening up new theranostic avenues. Based on these facts, the present study was designed to compare Tc-99m MDP bone scintigraphy and Ga-68 DOTANOC PET/CT in patients with RA. MATERIALS AND METHODS: Patients with a clinical diagnosis of RA were injected with 111-185 MBq of Ga-68 DOTANOC and 740 MBq of Tc-99m MDP intravenously. Images were acquired 30-45 min postinjection for Ga-68 DOTANOC on dedicated PET/CT scanners. Triple-phase bone scans were acquired on a dual-head gamma camera. PET/CT and MDP scan images were visually assessed by two experienced nuclear medicine physicians. RESULTS: Nineteen patients (16 women and 3 men) with a clinical diagnosis of RA were included in the study. Clinically, 196 joints in these 19 patients were diagnosed positive for RA. Of these 196 joints, Tc-99m MDP uptake was seen in 157 joints (80%). On Ga-68 DOTANOC scan, tracer uptake was seen in 151 of 196 joints (77%) with a lesion to background ratio of at least 2 in most of the joints. CONCLUSION: Ga-68 DOTANOC is equally good as an MDP scan for detection of RA with the added advantage of being used as a theranostic modality. However, further evaluation with a larger sample size and joint-to-joint comparison is warranted.
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Radioisótopos de GalioRESUMEN
Aim: Hemangioendotheliomas (HEs) are malignant vascular tumors with sparse descriptions in literature owing to their rarity. Study design: Ours is a retrospective study among patients of advanced HEs registered between September 2015 and April 2021. Results: There were 13 patients with median age 34.6 (range: 4-69 years), male preponderance (69%) and predominant subtype of epithelioid HE (76.9%). Common primary sites were viscera (46.2%) and bone (30.8%). Tyrosine kinase inhibitors (TKIs) yielded objective responses in 30% patients whereas chemotherapy only produced disease stabilization in 7.7%. Conclusion: We recognize an aggressive subset of HEs with manifestations such as acute liver failure and splenic rupture. Currently no biomarkers predict the efficacy of TKIs over chemotherapy; however, TKIs showed promising outcomes in this series.
Hemangioendotheliomas are rare malignant tumors with vascular origin. This retrospective study on 13 patients displays a male preponderance, the with epithelioid subtype being the commonest in the study population. The disease also showed aggressive manifestations such as acute liver failure and ruptured spleen. The viscera and bone were the commonest primary locations in 46.2 and 30.8% patients, respectively. Treatment with tyrosine kinase inhibitors showed a promising 30% response rate while chemotherapy showed stabilization of disease in 7.7% patients.
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BACKGROUND: Tuberculosis (TB) of posterior pharyngeal wall (PPW), either primary or secondary, is extremely rare and can mimic malignancy in elderly patients. CASE REPORT: A 36-year-old female patient presented with complains of nonhealing ulcer at the back of her throat for 4 months. On examination, there was an ulceroproliferative growth covered with slough over PPW. Positron emission tomography scan showed uptake in PPW. There was no uptake in any other part of body. A biopsy from PPW lesion showed epithelioid cell granulomas with Langhans type giant cells. Auramine-rhodamine staining showed few beaded bacilli consistent with TB. CONCLUSION: Tuberculosis of PPW is an extremely uncommon entity and can mimic malignancy. It should be kept as one of the differential diagnosis as it can be cured solely by giving anti-tubercular therapy.
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Neoplasias , Tuberculosis , Adulto , Anciano , Benzofenoneido , Femenino , Humanos , Faringe , Rodaminas , Tuberculosis/diagnóstico , Tuberculosis/tratamiento farmacológicoRESUMEN
BACKGROUND: Carney's triad is a rare syndrome comprising gastrointestinal stromal tumor, extra-adrenal paraganglioma, and pulmonary chondroma along with newer additions of adrenal adenoma and esophageal leiomyoma. The triad is completely manifest in only 25-30% cases, with most patients presenting with two out of three parts of the syndrome. Wild-type succinate-dehydrogenase-deficient gastric gastrointestinal stromal tumor forms the most common component of Carney's triad and is usually multicentric and multifocal. It usually demonstrates indolent behavior and resistance to imatinib; hence, the management remains predominantly surgical. Pulmonary chondromas are commonly unilateral and multiple with slow-growing nature, which allows for conservative management. Adrenocortical adenomas are found in 20% of patients and are usually detected as incidentalomas. CASE PRESENTATION: A 49-year-old Asian male presented with upper gastrointestinal bleed and was diagnosed with multiple gastric succinate-dehydrogenase-deficient gastrointestinal stromal tumors. On evaluation, he was found to have left pulmonary chondroma and non-secretory adrenal adenoma, thus completing the Carney's triad. He underwent surgery with sleeve gastrectomy and excision of the antral tumor nodule, while the adrenal and pulmonary tumors have been under close follow-up. CONCLUSION: Literature regarding Carney's triad is scarce, especially from the Indian setting. Our report aims to highlight the various manifestations of this syndrome with emphasis on management of wild-type succinate-dehydrogenase-deficient gastrointestinal stromal tumor. Radical gastric surgeries do not offer a survival advantage in this condition; hence, more conservative modalities of resection can be adopted.
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Condroma , Leiomiosarcoma , Neoplasias Pulmonares , Neoplasias Primarias Múltiples , Paraganglioma Extraadrenal , Neoplasias Gástricas , Adulto , Condroma/diagnóstico por imagen , Condroma/cirugía , Humanos , India , Masculino , Persona de Mediana Edad , Paraganglioma Extraadrenal/diagnóstico , Paraganglioma Extraadrenal/cirugía , Centros de Atención TerciariaRESUMEN
Background There is sparse literature on trabectedin in advanced soft-tissue sarcomas from developing world. It would be interesting to know about use and outcomes of trabectedin in Indian patients. Method In a retrospective study, consecutive patients treated with trabectedin from 2016 to 2019 were analyzed. Patients with L-sarcomas were treated at a dose of 1.5 mg/m 2 , while those with translocation-related sarcomas were treated at a dose of 1.2 mg/m 2 as a 24-hour infusion through peripherally inserted central catheter line. From July 2019, infusions were administered through an ambulatory elastomeric pump, while before that patients were admitted for 24 hours. We used SPSS version 23.0 for statistical calculation. Result A total of 20 patients received trabectedin with a total of 116 infusions. The median age was 46 years (range: 22-73 years). The male ( n = 11, 55%) and female patients were almost equal ( n = 9, 45%). Thirteen patients (65%) had Eastern Cooperative Oncology Group Performance Status 1. Majority of the patients had leiomyosarcoma ( n = 8, 40%); remaining comprised of liposarcoma (3, 15%), translocation-related sarcomas excluding myxoid liposarcoma ( n = 8, 40%) and others ( n = 1,5%). Most common site was extremity ( n = 11, 55%) followed by retroperitoneal ( n = 3, 15%), visceral ( n = 3, 15%), and others ( n = 3,15%). Median number of previous lines received was 2 (range: 0-4). Median number of trabectedin cycles received was 4 (range: 1-17). Best response assessed was stable disease ( n = 10, 50%), progressive disease ( n = 6, 30%), partial response ( n = 1, 5%), and not assessed in 3 patients. After a median follow-up of 19 months, median progression-free survival was 4 months. Conclusion In this heavily treated population (composed of L-sarcomas and translocation-related sarcomas) with many patients with poor performance status, the outcome with trabectedin is in synchrony with literature. However, the need of 24-hour admission might deter quality of life. Elastomeric pump seems to be a reasonable alternative to admission and can be a breakthrough in administering trabectedin, especially in developing countries.