Person:

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.

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.

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.

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.

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.

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.