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Journal of Indian Association of Pediatric Surgeons
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Table of Contents   
CASE REPORT
Year : 2022  |  Volume : 27  |  Issue : 6  |  Page : 771-774
 

Abdominal wall extraskeletal Ewing's sarcoma in an 8-year-old child


Department of Pediatric Surgery, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, India

Date of Submission26-Mar-2022
Date of Decision09-Jun-2022
Date of Acceptance30-Jul-2022
Date of Web Publication14-Nov-2022

Correspondence Address:
Vinay Kumar Konamme
271, 5th Cross, 5th Main, Canara Bank Layout, Vidyaranyapura Post, Bengaluru - 560 097, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaps.jiaps_46_22

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   Abstract 


Nonrhabdomyosarcoma soft-tissue sarcomas (NRSTSs) are a heterogeneous group of neoplasms of presumed mesenchymal origin. The precise diagnosis of the type of NRSTS tumor is complex. Among them, Ewing's sarcoma is very rare. We report a case of abdominal wall Ewing's sarcoma in an 8-year-old child. There are very few cases on pediatric abdominal wall Ewing's sarcoma reported. An 8-year-old female child presented with a progressively increasing swelling in the left iliac fossa for 3 months. On evaluation it was found to have a 4 cm × 2.87 cm × 4.1 cm circumscribed, heterogeneous exophytic mass arising from oblique muscles in the left lower abdominal wall with no intraperitoneal extension. On trucut biopsy and karyotyping, it was diagnosed to be an extraskeletal Ewing's sarcoma (EES) of the abdominal wall. Due to the proximity to the pelvic bone and the possible large size, the child was given six cycles of VAEC-IE as neoadjuvant chemotherapy to downsize the tumor. Following chemotherapy, wide excision of the tumor was done requiring partial resection of the external oblique, internal oblique, transverse abdominis, and leaving the transversalis fascia intact. The defect was closed using a polypropylene mesh. Histopathology showed tumor-free margins, with minimal histological tumor response to chemotherapy. Hence, the child underwent radiotherapy (45 Gy in 25 fractions). Follow-up fluorodeoxyglucose-positron emission tomography–computed tomography showed no recurrent lesion. There was no recurrence on 18-month follow-up. Extraskeletal Ewing's sarcoma is a rare tumor arising from the abdominal wall. The evaluation and management are similar to EES. Treatment is multimodal; however, surgery is the mainstay. Wide excision with tumor negative margin has a good outcome.


Keywords: Abdominal wall Ewing's sarcoma, abdominal wall reconstruction, Extraskeletal Ewing's sarcoma, nonrhabdomyosarcoma soft tissue sarcoma


How to cite this article:
Shankar G, Konamme VK. Abdominal wall extraskeletal Ewing's sarcoma in an 8-year-old child. J Indian Assoc Pediatr Surg 2022;27:771-4

How to cite this URL:
Shankar G, Konamme VK. Abdominal wall extraskeletal Ewing's sarcoma in an 8-year-old child. J Indian Assoc Pediatr Surg [serial online] 2022 [cited 2022 Dec 7];27:771-4. Available from: https://www.jiaps.com/text.asp?2022/27/6/771/360960





   Introduction Top


Nonrhabdomyosarcoma soft-tissue sarcomas (NRSTSs) are a heterogeneous group of neoplasms of presumed mesenchymal origin.[1] The precise diagnosis of the type of NRSTS tumor is complex. It requires information from traditional histopathologic techniques, immunohistochemical, ultrastructural, cytogenetic, molecular, and biochemical factors that influence response to oncologic management and prognosis.

Extraskeletal Ewing's sarcoma (EES) arising from the soft tissue accounts for <1% of all Ewing's sarcoma/primitive neuroectodermal tumors (PNETs). There are very few cases of abdominal wall Ewing's sarcoma cases reported in children. In this article, we are reporting a case of abdominal wall Ewing's sarcoma in 8 years of female child, diagnosis, and management.


   Case Report Top


An 8-year-old female child presented with a progressively increasing swelling in the left iliac fossa for 3 months. On clinical examination, she was found to have a 5 x 4 cm size, ovoid-shaped mass arising from the abdominal wall in the left iliac fossa region, the inferior border being 1 cm cranial to the iliac crest, the superior border was 6 cm below the left costal margin, the lateral border was 3 cm medial to the midaxillary line, and the medial border was 4 cm medial to the midline.

Magnetic resonance imaging (MRI) showed a 4 cm × 2.87 cm × 4.1 cm circumscribed, heterogeneous exophytic mass arising from oblique muscles in the left lower abdominal wall. No intraperitoneal extensions were noted. The pelvic bones, joints, musculature, and vessels were normal. There was no evidence of lymphadenopathy. For histological confirmation of the soft-tissue sarcoma, trucut biopsy of the lesion was done which was suggestive of a neoplasm composed of uniform small round cells arranged in sheets and occasional rossets with round nuclei, stippled chromatin, and scant clear to eosinophilic cytoplasm with indistinct cytoplasmic borders.

Genetic analysis showed translocation of chromosomes 11 and 22 (t[11-22]) suggestive of Ewing's sarcoma.

In view of the large size of the tumor, requiring extensive abdominal wall muscle loss and possible need for bone resection to achieve adequate margins, the child was advised neoadjuvant chemotherapy. She received six cycles of vincristine, Adriamycin, cyclophosphamide, doxorubicin, ifosfamide, and etoposide 3 weekly regimen.

Follow-up computed tomography (CT) scan [Figure 1] showed a decrease in the size of the mass and hence was planned for excision. An elliptical incision around the mass was given to include the trucut biopsy site. Tumor was found to be involving the internal oblique and part of the external oblique muscle. A wide excision was done about 1 cm away from the tumor including the external oblique, internal oblique, and transverse abdominis muscle leaving the peritoneum and transverse abdominis aponeurosis intact and there was no breach of the peritoneum [Figure 2]a and [Figure 2]b. The defect was closed over an 8 cm × 6 cm tailored polypropylene mesh [Figure 2]c. A small vacuum drain was inserted and removed on postoperative day 3.
Figure 1: CECT postneoadjuvant chemotherapy. CECT: Contrast-enhanced computed tomography

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Figure 2: (a) Resected mass. (b) Tumor bed picture postexcision. (c) Reconstruction using polypropylene mesh

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The histopathology sections of the excised lesion showed residual small round cell tumor with all resected margins free of tumor [Figure 3]. On immunohistochemistry, neoplastic cells were positive for CD99, NKX2.2 with Ki-67 index of 5% and negative for desmin, synaptophysin, and myogenin consistent with Ewing's sarcoma.
Figure 3: Histopathological specimen showing small round blue cell tumor

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As per the histopathology report, there was a poor histological response to VIDE induction chemotherapy which is an indication for radiotherapy despite clear surgical margins. She completed radiotherapy, a total radiation of 45 Gy as one daily fraction dose of 1.8 Gy, five fractions per week for 5 weeks with a total of 25 fractions.

Follow-up positron emission tomography (PET/CT) scan was done to assess treatment response which showed no definite enhancing or fluorodeoxyglucose avid lesions suggestive of complete response to treatment. No recurrence was seen on 18-month follow-up clinical examination and ultrasonography, and the child needs further long-term follow-up.


   Discussion Top


The soft-tissue sarcomas of childhood are relatively rare and account for 7% of all cancers in children younger than 20 years.[2] Rhabdomyosarcoma is the most common soft-tissue sarcoma in children, approximately half of soft-tissue sarcomas.[1]

Although soft-tissue sarcomas occur in any anatomic site, 45% occur in the extremity, most commonly in the thigh, followed in order of frequency by visceral (20%), retroperitoneal (15%), truncal or thoracic (10%), and other locations (10%).[3]

The initial evaluation involves an ultrasound of the lesion which helps in the assessment of the size, location, and consistency, particularly when located in the abdomen or extremities and differentiation between cystic and solid lesions.

The pathologist plays a key role in the diagnosis and thus future management of children with soft-tissue sarcomas. A pretreatment biopsy to diagnose and grade sarcoma is highly preferred. Biopsy should be carried out by an experienced surgeon (or a radiologist) and may be accomplished by open incisional or needle technique.[4]

Contrast-enhanced CT or MRI of the abdomen with plain CT of the chest is performed for staging the disease. Askri et al.[5] reported the features of soft-tissue Ewing's sarcoma on various modalities of imaging. On sonography, a hypoechoic mass with intra-tumor flow signals is seen. Whereas on CT, a sharply delineated relatively hypodense or isodense mass compared to adjacent muscle can be seen. Intratumor necrosis can be seen in large tumors with intense postcontrast enhancement. On the other hand, MRI shows low-to-intermediate signal intensity on T1-weighted images and high signal intensity on T2-weighted images with heterogeneous contrast enhancement.

PET/CT scan may be useful in staging, prognostication, grading, and determining response to neoadjuvant therapy.[4] Metastatic disease at the time of initial presentation occurs in approximately 15% of children with NRSTSs.[6] The lung is the most common site of distant metastases, although metastases to bone, liver, and mesentery have been reported.[7]

Treatment generally involves multimodality therapy which provides a better chance of successful outcome. For subcutaneous or intramuscular sarcomas smaller than 5 cm, surgery alone should be considered if adequate wide excision with a good 1 cm cuff of surrounding fat and muscle can be achieved.[3]

EES/PNET is an aggressive tumor with a high incidence of local recurrence and distant metastasis. Major prognostic factors include the absence or presence of a metastasis, tumor size, the extent of necrosis, the initial response to chemotherapy, and the presence of EWS/FLI1 fusion transcripts.[8]

EES accounts for <1% of Ewing's sarcoma overall. A generalized treatment protocol is yet to be established. It is necessary to formulate a patient individualized protocol on neoadjuvant chemotherapy, imaging for assessment of tumor response, timing of surgery, plan for abdominal wall reconstruction, and follow-up. A local wide resection is the recommended operation, with the goal of complete resection of tumor lesions, and negative surgical margin. The timing of definitive reconstruction must be optimized as much as possible to minimize the risk of reconstructive failure, wound infection, and hernia development. If there is any concern for positive margins on the resection specimen, wound contamination, or patient instability, the reconstruction should be done at a second stage.[9]

The first Pediatric Oncology Group and Children's Cancer Group study INT-0091 achieved 69% 5-year event-free survival with VADC+IE (vincristine, actinomycin, doxorubicin, cyclophosphamide, ifosfamide, and etoposide).[10]

In pediatric Ewing's sarcoma, postoperative radiotherapy is indicated in patients with-poor histological response to VIDE induction therapy, marginal resection, and intralesional resection-after second look surgery (if feasible), surgery at the time of diagnosis even if this was considered R0, unless a wide second-look operation is feasible and large pelvic tumors also in the patients with wide resections.[11]

The child which we are reporting presented with a left iliac fossa abdominal wall mass. Savic et al.[12] reported a 15-year-old boy presenting as acute right iliac fossal pain. With suspicion of acute appendicitis, the child underwent laparotomy and was found to have a hemorrhagic mass arising from the abdominal wall which was excised. He needed repeat surgery as on the postoperative imaging persistence of the lesion was seen. Following which chemoradiotherapy was given and was successfully managed.

Another case of Ewing's sarcoma of the abdominal wall in an 18-year-old female patient presenting as small bowel obstruction was reported by Geens et al., where the patient underwent surgical resection followed by adjuvant chemotherapy.[13]

Liaqat et al.[14] reported a large Ewing's sarcoma lesion arising from the left anterolateral abdominal wall mass in a day 7 old neonate. The mass was successfully excised and needed a skin grafting for closure of the defect. No adjuvant therapy was given for the neonate due to the possible hazards of chemotherapy and radiotherapy.

Another case of a 65-year-old male patient with abdominal wall Ewing's sarcoma reported by Aydinli et al.,[15] primary excision of the lesion with defect closure by composite mesh was done and following which six cycles of chemotherapy were given.

In our case, since the child had a large mass with possible large defect after excision, neoadjuvant chemotherapy was given. Once the tumor reduced in size, the child successfully underwent R0 resection of the lesion with preperitoneal polypropylene mesh placement. Following which chemotherapy was given. The child also underwent radiotherapy as on the histopathology poor response was seen.


   Conclusion Top


Extraskeletal Ewing's sarcoma is a rare tumor arising from the abdominal wall. The evaluation and management are similar to EES. Treatment is multimodal, however, surgery is the mainstay. Multimodality therapy including wide excision with tumor negative margins has a good outcome.

The child's parents have given consent for publication.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given her consent for images and other clinical information to be reported in the journal. The guardian understands that her names and initials will not be published and due efforts will be made to conceal the patient's identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
PDQ Pediatric Treatment Editorial Board. Childhood Soft Tissue Sarcoma Treatment (PDQ®): Health Professional Version. 2021 Dec 29. In: PDQ Cancer Information Summaries. Bethesda (MD): National Cancer Institute (US); 2002. Available from: https://www.ncbi.nlm.nih.gov/books/NBK65923/2. [Last accessed on 2022 Feb 03].  Back to cited text no. 1
    
2.
Qureshi SS, Bhagat M. Non-rhabdomyosarcoma soft-tissue sarcomas in children: Contemporary appraisal and experience from a single centre. J Indian Assoc Pediatr Surg 2015;20:165-9.  Back to cited text no. 2
[PUBMED]  [Full text]  
3.
Samuel Singer. Soft tissue sarcomas. In: Townsend CM, Beauchamp RD, Evers BM, Mattox KL, editors. Sabiston Textbook of Surgery. 19th ed. Philadelphia, PA:Elsevier; 2013. p. 768-82.  Back to cited text no. 3
    
4.
NCCN. NCCN Clinical Practice Guidelines in Oncology (NCCN guidelines), Soft Tissue Sarcoma. Available from: www.nccn.com. [Last accessed on 2020 Jul 31].  Back to cited text no. 4
    
5.
Askri A, Farhat LB, Ghariani B, Rabeh A, Dali N, Said W, et al. Extraskeletal Ewing sarcoma of the abdominal wall. Cancer Imaging 2008;8:156-8.  Back to cited text no. 5
    
6.
Pappo AS, Rao BN, Jenkins JJ, Merchant T, Poquette CA, Cain A, et al. Metastatic nonrhabdomyosarcomatous soft-tissue sarcomas in children and adolescents: The St. Jude children's research hospital experience. Med Pediatr Oncol 1999;33:76-82.  Back to cited text no. 6
    
7.
Andrassy RJ. Soft tissue sarcoma. Carachi R, Grosfeld JL, Azmy AF, editors. The Surgery of Childhood Tumors. 2nd ed. Arnold, London:Springer; 2008. p. 273-304.  Back to cited text no. 7
    
8.
Lee WS, Kim YH, Chee HK, Hwang JJ, Kim JS, Lee SA, et al. Multimodal treatment of primary extraskeletal Ewing's sarcoma of the chest wall: Report of 2 cases. Cancer Res Treat 2009;41:108-12.  Back to cited text no. 8
    
9.
Spera LJ, Danforth RM, Hadad I. Incisions and reconstruction approaches for large sarcomas. Transl Gastroenterol Hepatol 2018;3:86.  Back to cited text no. 9
    
10.
Grier HE, Krailo MD, Tarbell NJ, Link MP, Fryer CJ, Pritchard DJ, et al. Addition of ifosfamide and etoposide to standard chemotherapy for Ewing's sarcoma and primitive neuroectodermal tumor of bone. N Engl J Med 2003;348:694-701.  Back to cited text no. 10
    
11.
Dunst J, Schuck A. Role of radiotherapy in Ewing tumors. Pediatr Blood Cancer 2004;42:465-70. doi: 10.1002/pbc.10446. PMID: 15049022.  Back to cited text no. 11
    
12.
Savic D, Djuricic S, Milickovic M, Djokic D, Grujic B, Vukadin M, et al. Extraskeletal Ewing sarcoma in the anterior abdominal wall. Srp Arh Celok Lek 2017;146:126.  Back to cited text no. 12
    
13.
Geens L, Robays JV, Geert V, der Speeten KV. An unusual location of extraosseous Ewing's sarcoma. Case Rep Oncol 2013;6:293-302.  Back to cited text no. 13
    
14.
Liaqat N, Dar SH, Iqbal A, Khan FA, Sultan N, Extraskeletal Ewing sarcoma involving abdominal wall in a neonate. J Ped Surg Case Rep 2014;2:23-4.  Back to cited text no. 14
    
15.
Aydinli B, Ozturk G, Yildirgan MI, Polat KY, Basoglu M, Gundogdu C, et al. Extraskeletal Ewing's sarcoma in the abdominal wall: A case report. Acta Oncol 2006;45:484-6.  Back to cited text no. 15
    


    Figures

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