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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

28-year-old man with lymph-node metastasis from pelvic extraskeletal Ewing sarcoma family of tumors.A. Axial contrast-enhanced computed tomography (CT) scan shows perirectal, soft-tissue mass (arrowheads) invading left wall of bladder (arrows), which was proven to be Ewing sarcoma on biopsy. B. Axial contrast-enhanced CT scan shows conglomerated retroperitoneal lymphadenopathy (arrows).
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Figure 5: 28-year-old man with lymph-node metastasis from pelvic extraskeletal Ewing sarcoma family of tumors.A. Axial contrast-enhanced computed tomography (CT) scan shows perirectal, soft-tissue mass (arrowheads) invading left wall of bladder (arrows), which was proven to be Ewing sarcoma on biopsy. B. Axial contrast-enhanced CT scan shows conglomerated retroperitoneal lymphadenopathy (arrows).

Mentions: Lymph node metastases were identified in 22 patients ([histopathological confirmation; n = 7], [imaging diagnosis on both CT and PET; n = 15]) usually manifested as enlarged lymph nodes with or without internal necrosis in the mediastinum (n = 5), retroperitoneum (n = 8), internal and external iliac regions (n = 2), inguinal regions (n = 4), and cervical regions (n = 3) (Fig. 5). In 15 patients who underwent PET/CT, lymph node metastases always showed high FDG avidity. The lymph node metastasis was commonly found in patients with primary EESs of the torso, including the abdomen, lung, peritoneum, pleura, and paravertebral region (n = 18), compared to EESs in soft tissue and extremities, head, and neck (n = 4).


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)

28-year-old man with lymph-node metastasis from pelvic extraskeletal Ewing sarcoma family of tumors.A. Axial contrast-enhanced computed tomography (CT) scan shows perirectal, soft-tissue mass (arrowheads) invading left wall of bladder (arrows), which was proven to be Ewing sarcoma on biopsy. B. Axial contrast-enhanced CT scan shows conglomerated retroperitoneal lymphadenopathy (arrows).
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 5: 28-year-old man with lymph-node metastasis from pelvic extraskeletal Ewing sarcoma family of tumors.A. Axial contrast-enhanced computed tomography (CT) scan shows perirectal, soft-tissue mass (arrowheads) invading left wall of bladder (arrows), which was proven to be Ewing sarcoma on biopsy. B. Axial contrast-enhanced CT scan shows conglomerated retroperitoneal lymphadenopathy (arrows).
Mentions: Lymph node metastases were identified in 22 patients ([histopathological confirmation; n = 7], [imaging diagnosis on both CT and PET; n = 15]) usually manifested as enlarged lymph nodes with or without internal necrosis in the mediastinum (n = 5), retroperitoneum (n = 8), internal and external iliac regions (n = 2), inguinal regions (n = 4), and cervical regions (n = 3) (Fig. 5). In 15 patients who underwent PET/CT, lymph node metastases always showed high FDG avidity. The lymph node metastasis was commonly found in patients with primary EESs of the torso, including the abdomen, lung, peritoneum, pleura, and paravertebral region (n = 18), compared to EESs in soft tissue and extremities, head, and neck (n = 4).

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