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Ewing sarcoma of the liver with multilocular cystic mass formation: a case report.

Ozaki Y, Miura Y, Koganemaru S, Suyama K, Inoshita N, Fujii T, Hashimoto M, Tamura T, Takeuchi K, Takano T - BMC Cancer (2015)

Bottom Line: Tumor cells were positive for periodic acid-Schiff reaction and immunoreactive for glycoprotein C99 and gene NKX2.2, as well as the neuroendocrine markers, CD56 and synaptophysin.We found no evidence of recurrence 15 months after completing chemotherapy.Imaging examinations of the other three reported cases showed solid tumors and a diffuse enlarged liver without mass lesion.

View Article: PubMed Central - PubMed

Affiliation: Department of Medical Oncology, Toranomon Hospital, 2-2-2 Toranomon, Minato-ku, Tokyo, 105-8470, Japan. 1755ozaki@gmail.com.

ABSTRACT

Background: Ewing sarcoma is a rare tumor that occurs commonly in the long bones of children or adolescents that can also arise in soft tissues including the extremities, retroperitoneum, chest wall, and rarely in the liver as primary sites. We report a case of Ewing sarcoma arising primarily in the liver and, to our knowledge, this is the fourth reported case of Ewing sarcoma occurring in the liver.

Case presentation: A 27-year-old Japanese woman was admitted with sudden onset right upper abdominal pain. Clinical examination revealed a multilocular cystic mass consisting of thickened, irregular septa and nodal walls in the right hepatic lobe. Ultrasound-guided aspiration biopsy of the liver mass showed clusters of small atypical round cells and the clinical preoperative diagnosis was mucinous cystadenoma of the liver. The patient underwent an extended right hepatectomy and histopathological findings revealed sheet-like proliferation of small- to medium-sized round cells. Tumor cells were positive for periodic acid-Schiff reaction and immunoreactive for glycoprotein C99 and gene NKX2.2, as well as the neuroendocrine markers, CD56 and synaptophysin. EWS-FLI-1 fusion transcript type 1 was detected by reverse transcriptase polymerase chain reaction. Pathological and molecular analysis confirmed the diagnosis of Ewing sarcoma arising primarily in the liver and the patient received adjuvant systemic chemotherapy with vincristine, doxorubicin, and cyclophosphamide, alternating with ifosfamide and etoposide. We found no evidence of recurrence 15 months after completing chemotherapy.

Conclusion: We present an extremely rare case of Ewing sarcoma arising primarily in the liver. To our knowledge, this is the fourth reported case of Ewing sarcoma occurring in the liver, and the first case with a multilocular cystic liver mass. Imaging examinations of the other three reported cases showed solid tumors and a diffuse enlarged liver without mass lesion. Clinicians should consider the possibility of Ewing sarcoma in young patients with a multilocular cystic mass with thick and/or irregular cyst walls in the liver.

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Color Doppler and contrast-enhanced ultrasonography showing the multilocular cystic liver mass. Color Doppler ultrasonography (A) and vascular phase contrast-enhanced ultrasonography using Sonazoid (92 seconds after bolus injection) (B) showed the multilocular cystic mass consisted of thickened irregular septa and nodal walls with blood flow.
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Fig2: Color Doppler and contrast-enhanced ultrasonography showing the multilocular cystic liver mass. Color Doppler ultrasonography (A) and vascular phase contrast-enhanced ultrasonography using Sonazoid (92 seconds after bolus injection) (B) showed the multilocular cystic mass consisted of thickened irregular septa and nodal walls with blood flow.

Mentions: The patient was transferred to our hospital three weeks later. Color Doppler ultrasonography showed the multilocular cystic mass consisted of thickened irregular septa and nodal walls with blood flow (Figure 2A). Contrast-enhanced ultrasonography using Sonazoid (Daiichi-Sankyo, Tokyo, Japan) described the enhancement of septa and nodal walls in the vascular phase to the same degree as the surrounding parenchyma (Figure 2B). Magnetic resonance imaging was performed but provided no additional information. We made a presumed diagnosis of mucinous cystadenoma of the liver based on the imaging studies and the patient underwent an extended right hepatectomy in August 2012 (Figure 3) with no postoperative complications including hepatic failure. R0 resection was achieved. Histopathological examination revealed monotonous solid and sheet-like proliferation of small- to medium-sized round cells with rosette formation (Figure 4A). Tumor cells were positive for periodic acid-Schiff (PAS) reaction and immunoreactive for CD99 (Figure 4B), NKX2.2 and the neuroendocrine markers CD56 and synaptophysin. EWS-FLI-1 fusion transcript type 1, which is the result of translocation between exon 7 of the EWSR1 gene on chromosome 22q12 and exon 6 of the FLI1 gene on chromosome 11q24, was detected by reverse transcriptase real-time polymerase chain reaction. Pathological and molecular analysis confirmed the diagnosis of Ewing sarcoma. Postoperative technetium bone scintigraphy and fluorodeoxyglucose positron emission tomography revealed no bone or soft tissue lesion as a primary site of Ewing sarcoma, no residual lesion in the liver, and no distant metastases. Also, bone marrow aspiration and biopsy revealed no malignant cells. These findings supported a diagnosis of localized Ewing sarcoma arising in the liver. The patient received adjuvant systemic chemotherapy as follows: VDC (vincristine 1.4 mg/m2, doxorubicin 75 mg/m2, and cyclophosphamide 1200 mg/m2 followed by mesna) alternating with IE (ifosfamide 1.8 g/m2 with mesna each day for five days, and etoposide 100 mg/m2 for five days) until September 2013. We found no evidence of recurrence 15 months after completing chemotherapy.Figure 2


Ewing sarcoma of the liver with multilocular cystic mass formation: a case report.

Ozaki Y, Miura Y, Koganemaru S, Suyama K, Inoshita N, Fujii T, Hashimoto M, Tamura T, Takeuchi K, Takano T - BMC Cancer (2015)

Color Doppler and contrast-enhanced ultrasonography showing the multilocular cystic liver mass. Color Doppler ultrasonography (A) and vascular phase contrast-enhanced ultrasonography using Sonazoid (92 seconds after bolus injection) (B) showed the multilocular cystic mass consisted of thickened irregular septa and nodal walls with blood flow.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4307901&req=5

Fig2: Color Doppler and contrast-enhanced ultrasonography showing the multilocular cystic liver mass. Color Doppler ultrasonography (A) and vascular phase contrast-enhanced ultrasonography using Sonazoid (92 seconds after bolus injection) (B) showed the multilocular cystic mass consisted of thickened irregular septa and nodal walls with blood flow.
Mentions: The patient was transferred to our hospital three weeks later. Color Doppler ultrasonography showed the multilocular cystic mass consisted of thickened irregular septa and nodal walls with blood flow (Figure 2A). Contrast-enhanced ultrasonography using Sonazoid (Daiichi-Sankyo, Tokyo, Japan) described the enhancement of septa and nodal walls in the vascular phase to the same degree as the surrounding parenchyma (Figure 2B). Magnetic resonance imaging was performed but provided no additional information. We made a presumed diagnosis of mucinous cystadenoma of the liver based on the imaging studies and the patient underwent an extended right hepatectomy in August 2012 (Figure 3) with no postoperative complications including hepatic failure. R0 resection was achieved. Histopathological examination revealed monotonous solid and sheet-like proliferation of small- to medium-sized round cells with rosette formation (Figure 4A). Tumor cells were positive for periodic acid-Schiff (PAS) reaction and immunoreactive for CD99 (Figure 4B), NKX2.2 and the neuroendocrine markers CD56 and synaptophysin. EWS-FLI-1 fusion transcript type 1, which is the result of translocation between exon 7 of the EWSR1 gene on chromosome 22q12 and exon 6 of the FLI1 gene on chromosome 11q24, was detected by reverse transcriptase real-time polymerase chain reaction. Pathological and molecular analysis confirmed the diagnosis of Ewing sarcoma. Postoperative technetium bone scintigraphy and fluorodeoxyglucose positron emission tomography revealed no bone or soft tissue lesion as a primary site of Ewing sarcoma, no residual lesion in the liver, and no distant metastases. Also, bone marrow aspiration and biopsy revealed no malignant cells. These findings supported a diagnosis of localized Ewing sarcoma arising in the liver. The patient received adjuvant systemic chemotherapy as follows: VDC (vincristine 1.4 mg/m2, doxorubicin 75 mg/m2, and cyclophosphamide 1200 mg/m2 followed by mesna) alternating with IE (ifosfamide 1.8 g/m2 with mesna each day for five days, and etoposide 100 mg/m2 for five days) until September 2013. We found no evidence of recurrence 15 months after completing chemotherapy.Figure 2

Bottom Line: Tumor cells were positive for periodic acid-Schiff reaction and immunoreactive for glycoprotein C99 and gene NKX2.2, as well as the neuroendocrine markers, CD56 and synaptophysin.We found no evidence of recurrence 15 months after completing chemotherapy.Imaging examinations of the other three reported cases showed solid tumors and a diffuse enlarged liver without mass lesion.

View Article: PubMed Central - PubMed

Affiliation: Department of Medical Oncology, Toranomon Hospital, 2-2-2 Toranomon, Minato-ku, Tokyo, 105-8470, Japan. 1755ozaki@gmail.com.

ABSTRACT

Background: Ewing sarcoma is a rare tumor that occurs commonly in the long bones of children or adolescents that can also arise in soft tissues including the extremities, retroperitoneum, chest wall, and rarely in the liver as primary sites. We report a case of Ewing sarcoma arising primarily in the liver and, to our knowledge, this is the fourth reported case of Ewing sarcoma occurring in the liver.

Case presentation: A 27-year-old Japanese woman was admitted with sudden onset right upper abdominal pain. Clinical examination revealed a multilocular cystic mass consisting of thickened, irregular septa and nodal walls in the right hepatic lobe. Ultrasound-guided aspiration biopsy of the liver mass showed clusters of small atypical round cells and the clinical preoperative diagnosis was mucinous cystadenoma of the liver. The patient underwent an extended right hepatectomy and histopathological findings revealed sheet-like proliferation of small- to medium-sized round cells. Tumor cells were positive for periodic acid-Schiff reaction and immunoreactive for glycoprotein C99 and gene NKX2.2, as well as the neuroendocrine markers, CD56 and synaptophysin. EWS-FLI-1 fusion transcript type 1 was detected by reverse transcriptase polymerase chain reaction. Pathological and molecular analysis confirmed the diagnosis of Ewing sarcoma arising primarily in the liver and the patient received adjuvant systemic chemotherapy with vincristine, doxorubicin, and cyclophosphamide, alternating with ifosfamide and etoposide. We found no evidence of recurrence 15 months after completing chemotherapy.

Conclusion: We present an extremely rare case of Ewing sarcoma arising primarily in the liver. To our knowledge, this is the fourth reported case of Ewing sarcoma occurring in the liver, and the first case with a multilocular cystic liver mass. Imaging examinations of the other three reported cases showed solid tumors and a diffuse enlarged liver without mass lesion. Clinicians should consider the possibility of Ewing sarcoma in young patients with a multilocular cystic mass with thick and/or irregular cyst walls in the liver.

Show MeSH
Related in: MedlinePlus