Person: Ramaiya, Nikhil H.
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Ramaiya
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Nikhil H.
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Ramaiya, Nikhil H.
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Publication Fluid Retention Associated with Imatinib Treatment in Patients with Gastrointestinal Stromal Tumor: Quantitative Radiologic Assessment and Implications for Management(The Korean Society of Radiology, 2015) Kim, Kyung Won; Shinagare, Atul B.; Krajewski, Katherine; Pyo, Junhee; Tirumani, Sree Harsha; Jagannathan, Jyothi; Ramaiya, Nikhil H.Objective: We aimed to describe radiologic signs and time-course of imatinib-associated fluid retention (FR) in patients with gastrointestinal stromal tumor (GIST), and its implications for management. Materials and Methods In this Institutional Review Board-approved, retrospective study of 403 patients with GIST treated with imatinib, 15 patients with imaging findings of FR were identified by screening radiology reports, followed by manual confirmation. Subcutaneous edema, ascites, pleural effusion, and pericardial effusion were graded on a four-point scale on CT scans; total score was the sum of these four scores. Results: The most common radiologic sign of FR was subcutaneous edema (15/15, 100%), followed by ascites (12/15, 80%), pleural effusion (11/15, 73%), and pericardial effusion (6/15, 40%) at the time of maximum FR. Two distinct types of FR were observed: 1) acute/progressive FR, characterized by acute aggravation of FR and rapid improvement after management, 2) intermittent/steady FR, characterized by occasional or persistent mild FR. Acute/progressive FR always occurred early after drug initiation/dose escalation (median 1.9 month, range 0.3-4.0 months), while intermittent/steady FR occurred at any time. Compared to intermittent/steady FR, acute/progressive FR was severe (median score, 5 vs. 2.5, p = 0.002), and often required drug-cessation/dose-reduction. Conclusion: Two distinct types (acute/progressive and intermittent/steady FR) of imatinib-associated FR are observed and each type requires different management.Publication Intrathoracic malignant peripheral nerve sheath tumors: imaging features and implications for management(Versita, Warsaw, 2013) Kamran, Sophia Constance; Shinagare, Atul; Howard, Stephanie Anne Holler; Nishino, Mizuki; Hornick, Jason Laurence; Krajewski, Katherine Margaret; Ramaiya, Nikhil H.Background: The aim of the study was to analyze the clinical and imaging characteristics of primary intrathoracic malignant peripheral nerve sheath tumors (MPNSTs). Patients and methods. In this institutional review board (IRB)-approved retrospective study, clinical and imaging features of 15 patients (eight men; mean age 50 years [range 18–83)] with pathologically proven malignant peripheral nerve sheath tumors seen from January 1999 to December 2011 were analyzed. Imaging features (CT in 15, MRI in 5 and PET/CT in 4) of primary tumors were evaluated by three radiologists and correlated with clinical management. Results: Of the 15 tumors, six were located in the mediastinum (two each in anterior, middle and posterior mediastinum), four in chest wall, two were paraspinal, and three in the lung. Four patients had neurofibromatosis-1 (NF1); four tumors had heterologous rhabdomyoblastic differentiation (malignant triton tumor). Masses typically were elongated along the direction of nerves, with mean size of 11 cm. The masses were hypo- or isodense to muscles on CT, isointense on T1-weighted images, hyperintense on T2-weighted images and intensely fluorodeoxyglucose (FDG) avid (mean standardized uptake value [SUV]max of 10.5 [range 4.4–23.6]). Necrosis and calcification was seen in four tumors each. Finding of invasion of adjacent structures on imaging led to change in management in seven patients; patients with invasion received chemoradiation. Conclusions: Intrathoracic MPNSTs appear as large elongated masses involving mediastinum, lung or chest wall. Radiological identification of invasion of adjacent structures is crucial and alters therapy, with patients with invasion receiving neoadjuvant or adjuvant chemoradiation.Publication Imaging Features of Primary Tumors and Metastatic Patterns of the Extraskeletal Ewing Sarcoma Family of Tumors in Adults: A 17-Year Experience at a Single Institution(The Korean Society of Radiology, 2015) Huh, Jimi; Kim, Kyung Won; Park, Seong Joon; Kim, Hyoung Jung; Lee, Jong Seok; Ha, Hyun Kwon; Tirumani, Sree Harsha; Ramaiya, Nikhil H.Objective: To comprehensively analyze the spectrum of imaging features of the primary tumors and metastatic patterns of the Extraskeletal Ewing sarcoma family of tumors (EES) in adults. Materials and Methods We performed a computerized search of our hospital's data-warehouse from 1996 to 2013 using codes for Ewing sarcoma and primitive neuroectodermal tumors as well as the demographic code for ≥ 18 years of age. We selected subjects who were histologically confirmed to have Ewing sarcoma of extraskeletal origin. Imaging features of the primary tumor and metastatic disease were evaluated for lesion location, size, enhancement pattern, necrosis, margin, and invasion of adjacent organs. Results: Among the 70 patients (mean age, 35.8 ± 15.6 years; range, 18-67 years) included in our study, primary tumors of EES occurred in the soft tissue and extremities (n = 20), abdomen and pelvis (n = 18), thorax (n = 14), paravertebral space (n = 8), head and neck (n = 6), and an unknown primary site (n = 4). Most primary tumors manifested as large and bulky soft-tissue masses (mean size, 9.0 cm; range, 1.3-23.0 cm), frequently invading adjacent organs (45.6%) and showed heterogeneous enhancement (73.7%), a well-defined (66.7%) margin, and partial necrosis/cystic degeneration (81.9%). Notably, 29 patients had metastatic disease detected at their initial diagnosis. The most frequent site of metastasis was lymph nodes (75.9%), followed by bone (31.0%), lung (20.7%), abdominal solid organs (13.8%), peritoneum (13.8%), pleura (6.9%), and brain (3.4%). Conclusion: Primary tumors of EES can occur anywhere and mostly manifest as large and bulky, soft-tissue masses. Lymph nodes are the most frequent metastasis sites.Publication Clinical and radiologic features of extraskeletal myxoid chondrosarcoma including initial presentation, local recurrence, and metastases(Versita, Warsaw, 2014) Kapoor, Neena; Shinagare, Atul; Jagannathan, Jyothi; Shah, Shaan H.; Krajewski, Katherine; Hornick, Jason; Ramaiya, Nikhil H.Background: The aim of the study was to evaluate the clinical and imaging features of extraskeletal myxoid chondrosarcoma (EMC) including initial presentation, recurrence, and metastases. Patients and methods. In this institutional review board-approved retrospective study, imaging features of 13 patients with pathologically proven EMC seen from August 1995 to December 2011 were analyzed. The group included 3 women and 10 men and the mean age was 54 years (range 29–73 years). Imaging studies were evaluated by two radiologists in consensus. Location, size, and imaging features of primary tumors were recorded as well as the presence of recurrent disease and location of metastases. Results: Among 13 patients, 3 died during the timeframe of this study. Nine patients had primary tumor in the lower extremity, and average tumor size was 9.3 cm (range 3.3–18 cm). On MRI, primary tumors were hyperintense on T2, isointense to muscle on T1, and demonstrated peripheral/septal enhancement. Three patients had local recurrence and 12 had metastatic disease, with lung involvement being the most common. Tumor density on contrast enhanced CT ranged from 8.2 to 82.9 Hounsfield unit (HU). FDG-PET/CT imaging was performed in 3 patients. One patient had no FDG avid disease and 2 patients had metastatic disease with standard uptake values (SUV) of 2.8 and 7.4. The patient with intense FDG uptake demonstrated more solid appearing tumor burden and had the shortest survival. Conclusions: EMC is a rare tumor that often occurs in the lower extremities and frequently metastasizes to the lungs. Increased tumor density and increased FDG uptake may be related to more aggressive disease.Publication Metastatic pattern of uterine leiomyosarcoma: retrospective analysis of the predictors and outcome in 113 patients(Asian Society of Gynecologic Oncology; Korean Society of Gynecologic Oncology, 2014) Tirumani, Sree Harsha; Deaver, Pamela; Shinagare, Atul; Tirumani, Harika; Hornick, Jason; George, Suzanne; Ramaiya, Nikhil H.Objective: To describe metastatic pattern of uterine leiomyosarcomas (ULMS) and correlate it with clinical and histopathologic parameters. Methods: We included 113 women (mean age, 53 years; range, 29 to 72 years) with histopathology-confirmed ULMS from 2000 to 2012. Distribution of metastases was noted from imaging by two radiologists in consensus. Predictors of development of metastases were analyzed with univariate and multivariate analysis. Impact of various clinical and histopathologic parameters on survival was compared using Log-rank test and Cox proportional hazard regression model. Results: Distant metastases were seen in 81.4% (92/113) of the patients after median interval of 7 months (interquartile range, 1 to 21). Lung was most common site of metastases (74%) followed by peritoneum (41%), bones (33%), and liver (27%). Local tumor recurrence was noted in 57 patients (50%), 51 of whom had distant metastases. Statistically significant correlation was noted between local recurrence and peritoneal metastases (p<0.001) and between lung and other common sites of hematogeneous metastases (p<0.05). Age, serosal involvement, local recurrence, and the International Federation of Gynecology and Obstetrics (FIGO) stage were predictive factors for metastases. At the time of reporting, 65% (74/113) of the patients have died; median survival was 45 months. Stage, local recurrence, and age were poor prognostic factors. Conclusion: ULMS metastasizes most frequently to lung, peritoneum, bone, and liver. Local recurrence was associated with peritoneal spread and lung metastases with other sites of hematogeneous metastases. Age, FIGO stage and local recurrence predicted metastatic disease and advanced stage, older age and local recurrence predicted poor outcome.Publication Beyond PET/CT in Hodgkin lymphoma: a comprehensive review of the role of imaging at initial presentation, during follow-up and for assessment of treatment-related complications(Springer Berlin Heidelberg, 2015) Keraliya, Abhishek R.; Tirumani, Sree Harsha; Shinagare, Atul B.; Ramaiya, Nikhil H.Objective: The purpose of this article is to provide a comprehensive review of the role of imaging modalities other than PET/CT in the management of Hodgkin lymphoma (HL). PET/CT is the imaging modality of choice in the management of Hodgkin’s lymphoma (HL). However, imaging modalities other than PET/CT such as plain radiographs, ultrasound, CT, MRI and nuclear imaging can help in various stages of clinical management of HL, including the initial workup and post-treatment surveillance. Both CT and MRI help in detecting recurrences, treatment-related pulmonary, cardiovascular and abdominal complications as well as second malignancies. Familiarity with expected post-treatment changes and complications on surveillance images can help radiologists guide patient management. The purpose of this article is to provide a comprehensive review of the role of imaging modalities other than PET/CT in the management of Hodgkin lymphoma (HL). Main Messages • Surveillance of HL patients is usually performed with plain radiographs and CT. • Follow-up imaging can depict normal post-treatment changes or treatment-related complications. • Imaging is important for the timely detection of second malignancies in HL patients.Publication New targeted molecular therapies for cancer: radiological response in intrathoracic malignancies and cardiopulmonary toxicity: what the radiologist needs to know(BioMed Central, 2014) Souza, Frederico F; Smith, Andrew; Araujo, Cyrillo; Jagannathan, Jyothi; Johnston, Ciaran; O’Regan, Kevin; Shinagare, Atul; Ramaiya, Nikhil H.The emergence of new novel therapeutic agents which directly target molecules that are uniquely or abnormally expressed in cancer cells (molecular targeted therapy, MTT) has changed dramatically the treatment of cancer in recent years. The clinical benefit associated with these agents is typically limited to a subset of treated patients, who in many cases are defined by a specific genomic mutations and expression lesion within their tumor cells. All these new therapy modalities represent new challenges to radiologists as their mechanism of action and side effect profiles differ from conventional chemotherapy agents. In this article we will discuss radiological patterns of response to molecular targeted therapies MTT in lung cancer, typical and atypical radiological responses of targeted molecular therapy for other intra thoracic malignancies, cardiopulmonary toxicity and other side effects of molecular targeted therapy MTT in the thorax.Publication Systemic treatment in breast cancer: a primer for radiologists(Springer Berlin Heidelberg, 2015) Michaels, Aya; Keraliya, Abhishek R.; Tirumani, Sree Harsha; Shinagare, Atul B.; Ramaiya, Nikhil H.Abstract Cytotoxic chemotherapy, hormonal therapy and molecular targeted therapy are the three major classes of drugs used to treat breast cancer. Imaging modalities such as computed tomography (CT), magnetic resonance imaging (MRI), 18F-FDG positron emission tomography (PET)/CT and bone scintigraphy each have a distinct role in monitoring response and detecting drug toxicities associated with these treatments. The purpose of this article is to elucidate the various systemic therapies used in breast cancer, with an emphasis on the role of imaging in assessing treatment response and detecting treatment-related toxicities. Teaching Points • Cytotoxic chemotherapy is often used in combination with HER2-targeted and endocrine therapies. • Endocrine and HER2-targeted therapies are recommended in hormone-receptor- and HER2-positive cases. • CT is the workhorse for assessment of treatment response in breast cancer metastases. • Alternate treatment response criteria can help in interpreting pseudoprogression in metastasis. • Unique toxicities are associated with cytotoxic chemotherapy and with endocrine and HER2-targeted therapies.Publication Anti–PD-1–Related Pneumonitis during Cancer Immunotherapy(New England Journal of Medicine (NEJM/MMS), 2015) Nishino, Mizuki; Sholl, Lynette; Hatabu, Hiroto; Ramaiya, Nikhil H.; Hodi, FrankThe use of antibodies against programmed cell death 1 (PD-1), which block inhibitory T-cell checkpoints, is a promising new therapy for advanced cancers.1 Recent trials have shown substantial clinical activity of anti–PD-1 antibodies in advanced cancers and led to the approvals of these agents, including pembrolizumab for melanoma and nivolumab for melanoma and squamous-cell lung cancer.2-4 Pneumonitis related to the use of antibodies against PD-1 is an immune-mediated toxic effect that resulted in three drug-related deaths in a phase 1 trial.1 Clinical identification and management of pneumonitis are contingent on radiographic assessment. We report three cases of pneumonitis associated with the use of anti–PD-1 antibodies in patients with melanoma.Publication Extracutaneous melanomas: a primer for the radiologist(Springer Berlin Heidelberg, 2015) Keraliya, Abhishek R.; Krajewski, Katherine; Braschi-Amirfarzan, Marta; Tirumani, Sree Harsha; Shinagare, Atul B.; Jagannathan, Jyothi; Ramaiya, Nikhil H.Objective: The purpose of this article is to provide a comprehensive review of the imaging features of extracutaneous melanomas. Conclusion: Extracutaneous melanomas are clinically and biologically distinct from their more common cutaneous counterpart with higher frequency of metastatic disease and poorer overall prognosis. Complete surgical excision is the treatment of choice whenever possible; systemic therapy in the form of conventional chemotherapeutic agents as well as novel targeted agents is used for advanced/ metastatic disease. Multiple imaging modalities including US, CT, MRI and FDG-PET/CT play important roles in the evaluation of the primary tumour, assessment of metastatic disease and monitoring response to treatment. Radiologists should be aware of the typical imaging manifestations of extracutaneous melanoma, the distinct patterns of metastatic involvement as well as treatment response and toxicities associated with newer molecular targeted and immunotherapies to optimally contribute to patient management. Teaching points • Mucosal melanoma is clinically and biologically distinct from cutaneous melanoma. • Mucosal melanoma has a higher rate of metastatic disease than the cutaneous subtype. • Imaging is helpful in assessment of disease and response to treatment.