|Year : 2021 | Volume
| Issue : 2 | Page : 133-135
Primary Ewing’s sarcoma of vagina in young female: A rare entity
Jitin Goyal1, Jeevitesh Khoda1, Ruchir Jyani1, Ankur Kumar2, Sunil Kumar Puri1, Anila Sharma2, Arvind K Chaturvedi1
1 Department of Radiology, Rajiv Gandhi Cancer Institute and Research Centre, Delhi, India
2 Department of Histopathology, Rajiv Gandhi Cancer Institute and Research Centre, Delhi, India
|Date of Submission||15-Dec-2021|
|Date of Acceptance||12-Jan-2022|
|Date of Web Publication||23-Feb-2022|
Dr. Jeevitesh Khoda
Department of Radiology, Rajiv Gandhi Cancer Institute and Research Centre, Sir Chotu Ram Marg, Rohini Institutional Area, Sector 5, Rohini, New Delhi, Delhi
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Goyal J, Khoda J, Jyani R, Kumar A, Puri SK, Sharma A, Chaturvedi AK. Primary Ewing’s sarcoma of vagina in young female: A rare entity. J Curr Oncol 2021;4:133-5
| Case Presentation|| |
Ewing sarcoma (ES) is the second most common primary malignancy occurring in flat bones and diaphysis of long bones with a peak incidence between 10 and 20 years of age. It is included in ES family of tumors along with extraosseous Ewing sarcoma (EES), peripheral primitive neuroectodermal tumor (pPNET), and Askin tumor. EES accounts for 20%–30% of ES with common locations in paravertebral region, retroperitoneum, chest wall, and lower extremities. The occurrence of primary EES in the vagina is extremely rare and till now less than 30 cases have been reported in the literature.,,
In our case, a 15-year-old girl presented with complaints of menorrhagia for 1 month and pervaginal nonfoul-smelling white discharge for 15 days. It was associated with urinary retention and a lobulated mass protruding from the vaginal introitus.
She was evaluated with contrast-enhanced magnetic resonance imaging (MRI) of the pelvis with T1-weighted spin echo (T1W SE), T2-weighted turbo spin echo (T2W TSE), diffusion-weighted imaging (DWI), and short tau inversion recovery (STIR) followed by T1-weighted fat suppressed postcontrast (T1W FS) sequence using body matrix coil and whole body 18-fluorodeoxyglucose (18-FDG) PET-CT (position emission tomography–computed tomography) scan. MRI study revealed an enhancing altered signal intensity lobulated mass with restricted diffusion occupying the vaginal cavity predominantly arising from the right anterior and lateral wall causing vaginal stenosis with resultant hydrocolpos and hydrometra. The fat planes with urinary bladder and rectum were effaced. It was extending up to the right lateral pelvic wall abutting the obturator internus muscle. PET-CT showed metabolically active well-defined mass in the vagina (SUV max 11.92) with no other metabolically active disease elsewhere in the body [Figure 1].
|Figure 1: Axial and sagittal MRI images (A, B, D, and E) showing a hyperintense lobulated mass on T2WI (B) with restricted diffusion on DWI (D) occupying the vaginal cavity predominantly arising from the right anterior and lateral wall causing vaginal stenosis with resultant hydrocolpos and hydrometra. PET-CT (C and F) showing metabolically active defined mass lesion in vagina (SUV max 11.92)|
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Subsequently, a biopsy from the mass showed a poorly differentiated tumor in solid sheets. Tumor cells had round plump nuclei with fine chromatin, inconspicuous nucleoli, and scanty cytoplasm with frequent mitotic activity seen (~12–15/10hpf). On immunohistochemistry, tumor cells expressed NKX2.2 and showed dot-like CK in few cells. EWSR1 gene rearrangement signal was detected in 78% of tumor cells by the fluorescence in situ hybridization (FISH). A final diagnosis of ES of the vagina was made [Figure 2]. Bone marrow aspiration and biopsy did not show any marrow filtration.
|Figure 2: (A) Microphotograph showing a poorly differentiated tumor in sheets. (B) Higher power shows that the tumor is composed of monomorphic cells with scant cytoplasm. (C) The tumor shows brisk mitotic activity. (D) The tumor cells show diffuse nuclear immunostaining with NKX2.2|
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The patient received six cycles of VAC (vincristine, Adriamycin, and cyclophosphamide) combined with IE (ifosfamide and etoposide)-based chemotherapy. On follow-up MRI, the vaginal mass showed significant regression that was consistent with partial response. The patient underwent wide local excision of the vaginal mass with primary closure surgery. Postoperative period was uneventful.
| Discussion|| |
Ewing sarcoma family of tumors (EFTs) include ES, EES, pPNET, and Askin tumor. EES accounts for approximately 20%–30% of all ES cases., EES is rapidly growing superficial or deep mass and may occur in soft tissues at any location but paravertebral region, retroperitoneum, chest wall, and lower extremities are commonly reported. However, lung is the most common site for distant metastasis.,,
CT and FDG PET have the advantage of a high degree of accuracy in staging and restaging of the EFTs. On ultrasonography (USG), it appears as a hypoechoic mass with anechoic areas of hemorrhage/necrosis and increased blood flow on Doppler is shown., On CT, they appear as defined heterogeneous mass, attenuation similar to muscle and low attenuation areas of hemorrhage or necrosis., Calcification is seen in 25%–30% of cases.,,, On MRI, characteristics are a heterogeneous mass similar to that of skeletal muscle on T1-weighted images and intermediate-to-high signal intensity on T2-weighted images is seen.,, MRI is also useful for local tumor staging to evaluate the extent of involvement of surrounding structures and response evaluation after chemotherapy.,
18-FDG PET-CT is useful for local evaluation of primary lesion, detection of systemic metastasis to lung, bone or liver, and response evaluation after chemotherapy., Definitive diagnosis is usually made by biopsy under imaging guidance usually CT or USG.,
Histopathologically, EES shows small round blue cells containing spherical nuclei, inconspicuous nucleoli with scanty cytoplasm in fibrous stroma.,, Areas of hemorrhage and necrosis are common. On immunohistochemistry, cells express staining for CD99 (MIC2), neuron-specific enolase, Leu7 (CD57), FL-1 protein, and vimentin.,, Tumor cells are usually negative for S100 and leukocyte common antigen (CD45). The most common translocation of chromosome t(11;22)(q24;q12) is seen in 90% of cases due to fusion between the FLI gene on 11q24 and the EWSR1 gene on 22q12, creating an EWS/FLI-1 transcript that has the DNA binding domain of FLI-1 instead of the RNA-binding domain of EWS. Another common translocation t(21;22)(q22;q12) is also seen in 5%–10% of cases.,,
Treatment usually includes a combination of neoadjuvant systemic chemotherapy followed by surgery/radiation which may be supplemented by adjuvant CT in localized or metastatic EES. Overall prognosis of EES is better than skeletal ES. Metastatic disease carries a worse prognosis.
| Conclusion|| |
Primary EES of vagina is a very rare occurrence. MR and PET-CT play an important role for local staging and for metastatic workup, respectively. Definitive diagnosis is made by histopathology and immunohistochemical analysis as well as genetic translocations. Early diagnosis and treatment of EES is required for favorable outcomes.
We thank the patients and their families for their munificence in contributing to this study. We would also like to thank all members of the IRB committee who gave their approval for this study.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]