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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.

Huh J, Kim KW, Park SJ, Kim HJ, Lee JS, Ha HK, Tirumani SH, Ramaiya NH - Korean J Radiol (2015)

Bottom Line: We selected subjects who were histologically confirmed to have Ewing sarcoma of extraskeletal origin.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%).Lymph nodes are the most frequent metastasis sites.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul 138-736, Korea.

ABSTRACT

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.

No MeSH data available.


Related in: MedlinePlus

19-year-old man with primary extraaxial brain extraskeletal Ewing sarcoma family of tumors.A. Coronal T2-weighted magnetic resonance imaging (MRI) scan shows hyperintense, dural-based mass in right frontal region. B. Sagittal post-gadolinium T1-weighted MRI scan shows intense contrast enhancement.
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Figure 4: 19-year-old man with primary extraaxial brain extraskeletal Ewing sarcoma family of tumors.A. Coronal T2-weighted magnetic resonance imaging (MRI) scan shows hyperintense, dural-based mass in right frontal region. B. Sagittal post-gadolinium T1-weighted MRI scan shows intense contrast enhancement.

Mentions: Primary abdominal and pelvic EES tumors appeared as large retroperitoneal masses (n = 4), as peritoneal masses in the omentum, small bowel mesentery, or mesocolon (n = 4), as bulky subepithelial masses in the stomach, mimicking a gastrointestinal stromal tumor (n = 4), as exophytic masses in the kidney, mimicking renal cell carcinoma (n = 3) (Fig. 1), and as a mass in the uterus (n = 1) and prostate gland (n = 2). Tumors in the thorax appeared as pleural-based tumors (n = 4) (Fig. 2), chest-wall masses (n = 4), pulmonary parenchymal masses (n = 4), a mediastinal mass (n = 1), and as a heart mass (n = 1). Eight patients had paravertebral-located masses (four in the cervical spine and four in the sacrum), which manifested as intradural extramedullary or extradural masses with neural foraminal extension (Fig. 3). Of the six EESs in the head and neck, three were located in the paranasal sinuses, two were in the brain (Fig. 4), and one was in the oropharynx.


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.

Huh J, Kim KW, Park SJ, Kim HJ, Lee JS, Ha HK, Tirumani SH, Ramaiya NH - Korean J Radiol (2015)

19-year-old man with primary extraaxial brain extraskeletal Ewing sarcoma family of tumors.A. Coronal T2-weighted magnetic resonance imaging (MRI) scan shows hyperintense, dural-based mass in right frontal region. B. Sagittal post-gadolinium T1-weighted MRI scan shows intense contrast enhancement.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4499542&req=5

Figure 4: 19-year-old man with primary extraaxial brain extraskeletal Ewing sarcoma family of tumors.A. Coronal T2-weighted magnetic resonance imaging (MRI) scan shows hyperintense, dural-based mass in right frontal region. B. Sagittal post-gadolinium T1-weighted MRI scan shows intense contrast enhancement.
Mentions: Primary abdominal and pelvic EES tumors appeared as large retroperitoneal masses (n = 4), as peritoneal masses in the omentum, small bowel mesentery, or mesocolon (n = 4), as bulky subepithelial masses in the stomach, mimicking a gastrointestinal stromal tumor (n = 4), as exophytic masses in the kidney, mimicking renal cell carcinoma (n = 3) (Fig. 1), and as a mass in the uterus (n = 1) and prostate gland (n = 2). Tumors in the thorax appeared as pleural-based tumors (n = 4) (Fig. 2), chest-wall masses (n = 4), pulmonary parenchymal masses (n = 4), a mediastinal mass (n = 1), and as a heart mass (n = 1). Eight patients had paravertebral-located masses (four in the cervical spine and four in the sacrum), which manifested as intradural extramedullary or extradural masses with neural foraminal extension (Fig. 3). Of the six EESs in the head and neck, three were located in the paranasal sinuses, two were in the brain (Fig. 4), and one was in the oropharynx.

Bottom Line: We selected subjects who were histologically confirmed to have Ewing sarcoma of extraskeletal origin.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%).Lymph nodes are the most frequent metastasis sites.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul 138-736, Korea.

ABSTRACT

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.

No MeSH data available.


Related in: MedlinePlus