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Extraskeletal Ewing Sarcoma in a Young Patient During Pregnancy | OMICS International
ISSN: 2165-7920
Journal of Clinical Case Reports
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Extraskeletal Ewing Sarcoma in a Young Patient During Pregnancy

Friederika Fluck*, L Menezes Falcão, Raquel Paixão and Isabel Pinheiro
Department of Internal Medicine, University Hospital of Lisbon, Santa Maria, Lisbon, Portugal
Corresponding Author : Friederika Fluck
Department of Internal Medicine
University Hospital of Lisbon
Santa Maria, Lisbon, Portugal
Tel: 00351 919458259
E-mail: [email protected]
Received November 24, 2014; Accepted January 24, 2015; Published January 26, 2015
Citation: Fluck F, Falcão LM, Paixão R, Pinheiro I (2015) Extraskeletal Ewing Sarcoma in a Young Patient During Pregnancy. J Clin Case Rep 5:485. doi:10.4172/2165-7920.1000485
Copyright: © 2015 Fluck F, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

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Abstract

Background: Ewing’s sarcoma is usually identified as a primary malignancy of the bone affecting children and young adults. It is associated with soft tissue extension in 90% of cases. Primarily extraskeletal Ewing’s sarcoma is a rare subtype, and very few data are available concerning optimal treatment modalities.
Case Presentation: We report a case of an 18 year-old female from Cabo Verde in her 34th week of pregnancy presenting with a rapid growing cervical tumor. It had initially started as an indolent swelling at the left cervico-posterolateral region. CT had shown involvement of both superficial and deep cervical fascia. At time of presentation, the histologic diagnosis of a peripheral Primitive Neuroectodermal Tumor/Extraskeletal Ewing’s sarcoma had already been provided. To reduce risk of fetal metastasis, the child was delivered by caesarean section. A chest CT of the patient revealed pulmonary metastasis and compressive atelectasis of the left lung caused by the tumor. Three weeks post partum, she presented with loss of muscular function of the extremities as well as parestesia and bladder dysfunction. A repeat CT confirmed medullar compression at the level of C5-C6, C6-C7 and C7-D1 by the tumor without signs of bone metastasis. 5 sessions of palliative radiotherapy were performed that led to parcial recovery of muscular function of the upper right extremity. Palliative chemotherapy was refused, so that the patient was released from hospital. No metastasis had been detected in the child so far. Conclusion: Extraskeletal Ewing’s sarcoma can manifest as a rapidly growing localized mass causing local compression symptoms. Palliative radiotherapy can achieve local tumor reduction and symptom relief.

Introduction
Ewing’s sarcoma (ES) of the bone is part of the Ewing sarcoma family of tumors (ESFTs), such as the Askin tumors of the chest wall, Primitive Neuroectodermal Tumors (PNET) of bone or soft tissues and Extraskeletal Ewing Sarcoma (EES)/peripheral Primitive Neuroectodermal Tumor (pPNET) [1].
ES is usually identified as a primary malignancy of the bone affecting children and young adults, constituting the second most frequent sarcoma of bone in these age groups [2]. It is associated with soft tissue extension in 90% of cases.
Primarily extraskeletal Ewing’s sarcoma is a rare subtype, and was first described by Tefft et al. [3], reporting four patients with paravertebral soft tissue tumors that histologically resembled ES [3]. Since then several cases of EES have been described. It occurs more frequently in the age range between age 20 and 30 and can develop in almost any soft tissue of the body [1,4]. The tumor usually evolves with a high incidence of local recurrence and distal metastasis.
Few data are available concerning optimal treatment modalities for EES. Some authors report that multimodal therapy used in patients with ES seems to be adequate for those with EES [5,6].
So far, only few cases of EES during pregnancy have been reported.
Case Presentation
We report a case of an 18 year-old female from Cabo Verde in her 34th week of pregnancy presenting with a rapid growing cervical tumor.
Presentation
The tumor started as an indolent swelling on the left cervicopostero- lateral region (Figure 1). Surgical excision of the mass was done in her home country. It is unknown if histopathological examination has been performed.
The cervical tumor continued growing alarmingly fast, causing the patient to seek help in the neighbor country Senegal, where the tumor was excised again and histopathological and immunohistochemical examination was performed.
Histological examination
Histopathological examination revealed massive infiltration of connective and adipose tissue as well as infiltration of the striated muscle by an undifferentiated, round cell tumor. High cellular density was observed.
Cells appeared small and round with indistinct and fine granular cytoplasm. The nuclei were round to oval with indistinct cytoplasmic border and finely dotted chromatin, the nucleoli were rarely visible. Focal necrosis was detected with important hemorrhagic rearrangements.
Immunochemical examination
Immunohistochemical examination showed that tumor cells stained positively for anti-CD99 and anti-vimentine. Some tumor cells stained also positively for anti-CD68 and anti-protein S100. Other markers, such as anti-CD56, anti-CD45, anti-CD20, anti-CD3, antidesmine, anti-myogenine, anti-Bcl2 and anti- epithelial membrane antigen (EMA) were negative.
Based on these findings a peripheral Primitive Neuroectodermal Tumor/Extraskeletal Ewing’s saracoma was diagnosed.
Clinical course
The patient was referred to oncologic treatment. She was in her 25th week of pregnancy and was evacuated to Portugal due to lack of local treatment options.
At Lisbon University Hospital, the child was delivered by cesarean section at her 34th week of pregnancy to reduce fetal metastasis. Computed tomography (CT) scan had shown involvement of both superficial and deep cervical fascia (Figure 2), pulmonary metastasis (Figure 3) and compressive atelectasis of the left lung by the tumor (Figure 4).
Three weeks post-partum, she presented with loss of muscular function of the extremities as well as paresthesia and bladder dysfunction. A repeat CT confirmed medullar compression at the level of C5-C6, C6-C7 and C7-D1 by the tumor without signs of bone metastasis.
In order not to impair postoperative wound healing after cesarean surgery, palliative chemotherapy could not be administered immediately. Later on, the patient refused palliative chemotherapy.
Due to aggravation of symptoms, five sessions of palliative radiotherapy were performed with a total dose of 20 Gy. A partial recovery of muscular function of the upper right extremity could be achieved.
The patient was discharged home with her newborn son according to her wishes. No metastasis had been detected in the child so far.
Discussion
Ewing family of tumors
Ewing’s sarcoma (ES) and Primitive Neuroectodermal Tumor (PNET) are relatively rare and aggressive neoplasms that are both part of Ewing Family of Tumors (ESFTs). Ewing’s sarcoma typically is an undifferentiated primary malignancy of the bone, while neoplasms of the spectrum of PNET demonstrate clear evidence of neural differentiation.
Mitotic figures, necrosis, endothelial hyperplasia and Homer- Wright rosettes are in favor of the diagnosis of PNET instead of Ewing’s sarcoma [7].
Approximately 95% of patients with ESFT have a characteristic t (11; 22) (q24; q12) or t (21; 22) (q22; q12) chromosomal translocation identified by reverse transcription polymerase chain reaction (RTPCR) [8,9]. The differential diagnosis includes central PNET, malign meningioma, rhabdomyosarcoma, neuroblastoma and lymphoma. ES predominantly involves the major bones, pelvis and ribs and is associated with soft tissue extension in 90% of cases.
Extraskeletal Ewing’s sarcoma
Another member of ESFTs is the extraskeletal Ewing’s sarcoma (EES)/peripheral PNET, an uncommon neoplasm of uncharacterized mesenchymal cell origin that occurs most commonly in paravertebral and intercostal regions, such as chest wall, lower extremities and the retroperitoneum in 15% of the cases [10].
Histology typically shows small, round cells with round nucleoli, fine chromatin, scant cytoplasm, indistinct cell borders and necrosis [11]. It can easily be confused with other small round-cell tumors [12].
Immunochemical characteristics
Most of the tumors within the Ewing’s sarcoma family express surface antigens like CD99, 12E7, E2, 013 and HBA71, which are all products of the MIC2 gene. At first it was assumed to be highly specific and antibody staining for CD99 was used to confirm the diagnosis [12,13]. More recent findings show that, although its sensitivity ranges from 84% to 100% in ES/PNET, the specificity seems to be limited [11,14].
Tumor cells may also be positive for vimentin, friend leukemia integration 1 transcription facto (FL1). Reactivity for neuronspecific enolase (NSE), S-100, CD57, synaptophysin, cytokeratin and neurofilament, indicating neuroectodermal differentiation, may support the diagnosis of pPNET [7,10,14].
In our case the diagnosis was based on histopathological examination and imunohistochemical positivity of anti-CD99, antivimentin and anti-protein S-100.
Clinical manifestation
EES/pPNET manifesting as a primary cervical tumor is uncommon [12,15,16]. According to Chao et al. (2000), there were only five out of 118 cases of extraskeletal Ewing’s sarcoma located in the head and neck region [17]. Nearly half of patients have metastatic disease [6,18-21]. EES/pPNET frequently invade adjacent organs and metastasize to the lung [15], as well as to the bone and lymph nodes [22].
In the present case involvement of both superficial and deep cervical fascia with tracheal deviation had occurred, accompanied by lymph node and pulmonary metastasis and compressive atelectasis of the left lung caused by the tumor.
Pregnancy
There is a low incidence of cancer during pregnancy, with approximately 1 diagnosis in 1000 pregnant women [23]. The incidence of EES and ESFTs during pregnancy is extremely low and only a few cases have been reported so far.
In a recent review of the literature Verheecke et al. reported a total of 12 cases with primary ES diagnosed during pregnancy including 3 cases of EES [23].
Overall most of these cases received chemotherapy, radiotherapy and/or surgery during and after pregnancy. The majority of cases included delivery by cesarean section and an overall good outcome for both mother and child could be observed [24-33].
The reported cases of EES during pregnancy manifested as retroperitoneal abdominal mass, as solid mass in the left thigh and in duodenum. A vaginal delivery, cesarean section and termination of pregnancy were described. Radiotherapy, surgery and chemotherapy after pregnancy and chemotherapy during and after pregnancy were the treatment modalities [34-36].
None of the described cases presented metastasis at diagnosis, there is no information about the risk of fetal metastasis so far (Table 1).
Vaginal delivery could not be offered to our patient because of the rapid progression of tumor size and the presence of multiple metastasis. Due to lack of information about the risk of fetal metastasis in cases of EES during pregnancy and because of the aggressiveness of the tumor with high incidence of local recurrence and distant metastasis, we decided to deliver the child by cesarean section in the 34th week of pregnancy.
No metastasis was clinically evident in the newborn until returning to Cabo Verde in his 6th week of life. Continuous monitoring of the child should be performed in order to early EES metastasis detection. No recommendations about follow-up time in this specific situation have been described yet.
During pregnancy no chemotherapy was administered due to the palliative situation of the patient, in order to avoid toxicity for the unborn. The tumor was unresectable, with invasion of adjacent structures and distant metastasis. We could not offer immediate postoperative chemotherapy which led to a faster deterioration of clinical situation with medullar compression symptoms, and only partial recovery with palliative radiotherapy. This situation may have discouraged the patient to accept palliative chemotherapy afterwards.
Treatment options during pregnancy
Due to the small number of cases with ESFTs during pregnancy, little is known about optimal treatment options in this situation.
Patients with EES seem to benefit from the same combined modality treatment as established for patients with ESFTs [5,6]. Nearly every chemotherapy protocol for Ewing sarcoma has been based on four drugs: doxorubicin, cyclophosphamide, vincristine and dactinomycin, alternating with ifosfamide and etoposide [37]. Radiation for local control is recommended for unresectable tumors and when it is unlikely that adequate surgical margins can be achieved [22].
Predictors
Tumor size (>8 cm), metastasis at presentation, high serum lactate dehydrogenase (LDH), poor histological response to chemotherapy, radiotherapy only as local treatment and positive surgical margin have been described as significant predictors of worse overall survival [4,6,18,20-22,38,39].
Unfortunately our patient met several of these criteria (tumor size>8 cm, metastasis at presentation, high serum LDH and radiotherapy was only administered as palliative local treatment).
Olmos et al. [40] described that there is a tendency of these tumors to progress more rapidly in pregnancy [40]. Increased levels of oestrogens, progesterone and increased levels of IGF-1 expression on ES during pregnancy has been discussed [23,40].
Even if the patient had accepted palliative chemotherapy it is uncertain if a significant difference in quality of life and survival could have been achieved.
Conclusion
Extraskeletal Ewing’s sarcoma is a malignant tumor that can manifest as a rapidly growing localized mass causing local compression symptoms. It is an undifferentiated small round cell tumor and has a high recurrence rate and frequent distant metastasis.
This is the fourth case of EES described in a pregnant woman. Early delivery can minimize fetal metastasis. The newborn should be followed for early detection of metastasis. The best treatment modality has to be individualized for each case and according to the patient’s wishes. Palliative radiotherapy can achieve local tumor reduction and symptom relief. Future studies should focus on treatment strategies for these tumors.
References

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