Extraskeletal Ewing’s sarcoma: An adrenal localization

Introduction: Ewing’s sarcoma is a frequent bone malignant tumor in children and young adults. Its extraskeletal primitive localization is less common which makes its diagnosis less obvious. Case Report: We report a case of a 16-year-old female who presented at our department for one month history of pleuritic chest pain. The physical examination and the thoracic radiography results supported a pleural effusion. The results of the first thoracentesis and pleural biopsy were not conclusive, thus a second thoracentesis was attempted but did not withdraw any pleural fluid. A thoracoabdominal computed tomography (CT) scan was performed which found a large infraphrenic tumor suspecting an adrenal neuroblastoma. A CT guided biopsy was done and the histopathology study revealed a malignant proliferation made of medium sized round cells. The immunohistochemistry concluded to Ewing’s sarcoma. The diagnosis of primitive adrenal gland neuroectodermal tumor was established and adjuvant chemotherapy was recommended. The patient passed away during her first chemotherapy session. Conclusion: The adrenal localization of Ewing’s sarcoma is rare. A neuroblastoma is the first brought up diagnosis in such presentation but an extraskeletal neuroectodermal tumor is also possible. (This page in not part of the published article.) International Journal of Case Reports and Images, Vol. 7 No. 11, November 2016. ISSN – [0976-3198] Int J Case Rep Images 2016;7(11):762–765. www.ijcasereportsandimages.com Batahar et al. 762 CASE REPORT OPEN ACCESS Extraskeletal Ewing’s sarcoma: An adrenal localization Salma Ait Batahar, Safae Elidrissi, Salwa Berrada, Hanane Rais, Lamyae Amro


INTRODUCTION
Primitive neuroectodermal tumors belong to the family of Ewing's sarcoma. This cancer usually occurs in children and young adults and the skeletal form is the most frequent presentation [1]. It is a highly malignant tumor made of small round cells originating from neuroectoderm. The clinical and pathological presentations are divers which make the diagnosis difficult [2]. Extraskeletal Ewing's sarcoma is a rare presentation of these tumors which makes the diagnosis even more difficult. The case that we are presenting is an adrenal localization of Ewing's sarcoma which mimicked a neuroblastoma.

CASE REPORT
A 16-year-old female high school student presented to our department for one month history of dry cough, dyspnea and left chest pain. She had no prior medical history or trauma, did not smoke and there was no notable family medical history. The physical examination revealed absent tactile fremitus, dullness to percussion and decreased breath sounds in the left side of the thorax. The vital signs were stable: temperature 37°C, blood pressure 110/60 mmHg and respiratory rate 22 cpm. There was no cyanosis or digital clubbing. The chest radiography showed a homogenous opacity of the lower two-thirds of the left hemithorax which blunted both the cardiophrenic and costophrenic angles and showed a meniscus ( Figure 1).
The diagnosis of pleural effusion was suspected and a thoracentesis was performed which retrieved bloody pleural fluid. The chemical analysis found an exudate with a protein rate at 55.6 g/l. The cytology was inflammatory containing mainly lymphocytes with no malignant cells. The pathology study of the pleural biopsy found a pleuritis with no specific patterns or signs of malignancy. The diagnosis of an isolated pleural effusion was less probable and a thoracoabdominal contrast enhanced computed tomography scan was performed. This examination showed a large left infraphrenic mass measuring 107x128x159 mm and repressing both the left kidney and the spleen. It also showed a left pleural effusion and a collapsed left lung. The CT scan concluded to an adrenal neuroblastoma (Figures 2).
The catecholamine urine test was normal: epinephrine 0.01 µmol/24 h, norepinephrine 0.22 µmol/24 h and dopamine at 1.44 µmol/24 h. The complete blood count showed an anemia (hemoglobin 8.5 g /dL) and a thrombocytosis (platelets at 523x10 3 /µL). The blood ionogram revealed an elevated alkaline phosphatase (ALP) at 301 U/L. The endocrine blood tests including: parathormone, prolactine, cortisol thyreostimuline (TSHus) and T4L were normal. These results were not compatible with the diagnosis of an adrenal tumor. Computed tomography guided biopsy was performed and the pathology with immunohistochemistry analysis found medium sized round cells with oval nuclei and dense chromatin. The immunohistochemistry was positive for CD 99 and FLI1. These results concluded to Ewing's Sarcoma (Figure 3-5). The patient passed away one month after the diagnosis was made during her first chemotherapy session.

DISCUSSION
Ewing's sarcoma is a frequent malignant bone tumor occurring in adolescents and young adults. It mainly affects axial bones and involves a soft tissue infiltration [1]. The classical clinical presentation is a localized sore tumefaction or swelling resulting in a lytic bone lesion   with a periosteal reaction [2,3]. Our patient had both clinical and radiological presentation of a pleural effusion.
The extraskeletal Ewing's sarcoma (EES) belongs to the family of neuroectodermal tumors [4]. This malignant tumor can occur anywhere in the human body and presents as a soft tissue mass affecting adjacent organs [5]. In this case, the tumor was infraphrenic, it seemed to have developed from the adrenal gland and it repressed both the spleen and the kidney.
On imagery, extraskeletal Ewing's sarcoma presents as a bulky mass which is heterogeneous on computed tomography (CT scan) [5]. The CT scan of our patient showed a large adrenal tumor with a heterogeneous contrast enhancement. Celli and Cai reported this same aspect regarding primitive kidney Ewing sarcoma as the imagery reveals an ill-defined renal mass, with heterogeneous contrast enhancement and intermixed necrosis areas and hemorrhage [6]. The magnetic resonance imagery (MRI) scan allows a better appreciation of the involvement of soft tissues in Ewing's tumor [7]. In this case, the MRI scan should have been performed in order to assess clearly the infiltration of the adjacent organs as well as the chest and abdominal walls.
Based on imagery, differential diagnosis of an infraphrenic extraskeletal Ewing's sarcoma encompasses: neuroblastoma, carcinoid tumor, lymphoma, and desmoplastic small round blue cell tumor [8]. The CT scan of our patient showed an adrenal tumor which made the first suspected diagnosis a neuroblastoma.
The metastatic sites of extraskeletal Ewing's sarcoma are multiple. The most frequent metastatic sites are respectively: lymph nodes, bone, lung, abdominal solid organs, peritoneum, pleura and brain [5]. At the time of diagnosis, our patient had a left pleural effusion with an atelectasis of the left lung. The thoracoabdominal CT scan did not show any mediastinal or abdominal lymph nodes and there were no abnormalities in the right lung.
The histopathology study of the biopsy is the key to the confirmation of the diagnosis of extraskeletal Ewing's sarcoma. On gross examination, the median size of the tumor is about 13 cm and is characterized by confluent areas of necrosis and hemorrhage [6]. Histologically, neuroectodermal tumors are composed of uniform small round cells. These cells have a high nuclear/cytoplasm ratio, the nuclei are round with condensed chromatin [6]. The immunohistochemistry for CD99 helps for differential diagnosis even if it is not specific [9]. In our case, the pathology found round medium sized cells with an immunohistochemistry positive for CD99 and FLI1.
The ideal treatment of neuroectodermal tumors is surgical resection. As these tumors are considered chemo-radio-sensitive, thus adjuvant chemotherapy or radiotherapy is preconized in order to control the tumor locally and generally [10].

CONCLUSION
Primitive adrenal neuroectodermal tumors are a rare entity, only some cases have been reported in literature. The clinical and radiological presentation is not specific and there are many differential diagnoses. The MRI scan is the best imaging method to appreciate the tumor and the involvement of the adjacent structures. The confirmation of the diagnosis relies on the histopathology study and especially the immunohistochemistry and the molecular analysis. *********

Author Contributions
Salma Ait Batahar -Substantial contributions to conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Final approval of the version to be published Safae Elidrissi -Acquisition of data, Drafting the article, Final approval of the version to be published

Edorium Journals: An introduction
Edorium Journals Team

But why should you publish with Edorium Journals?
In less than 10 words -we give you what no one does.

Vision of being the best
We have the vision of making our journals the best and the most authoritative journals in their respective specialties. We are working towards this goal every day of every week of every month of every year.

Exceptional services
We care for you, your work and your time. Our efficient, personalized and courteous services are a testimony to this.

Editorial Review
All manuscripts submitted to Edorium Journals undergo pre-processing review, first editorial review, peer review, second editorial review and finally third editorial review.

Peer Review
All manuscripts submitted to Edorium Journals undergo anonymous, double-blind, external peer review.

Early View version
Early View version of your manuscript will be published in the journal within 72 hours of final acceptance.

Manuscript status
From submission to publication of your article you will get regular updates (minimum six times) about status of your manuscripts directly in your email.

Favored Author program
One email is all it takes to become our favored author. You will not only get fee waivers but also get information and insights about scholarly publishing.

Institutional Membership program
Join our Institutional Memberships program and help scholars from your institute make their research accessible to all and save thousands of dollars in fees make their research accessible to all.

Our presence
We have some of the best designed publication formats. Our websites are very user friendly and enable you to do your work very easily with no hassle. Something more...
We request you to have a look at our website to know more about us and our services.
We welcome you to interact with us, share with us, join us and of course publish with us.

Invitation for article submission
We sincerely invite you to submit your valuable research for publication to Edorium Journals.

Six weeks
You will get first decision on your manuscript within six weeks (42 days) of submission. If we fail to honor this by even one day, we will publish your manuscript free of charge.*

Four weeks
After we receive page proofs, your manuscript will be published in the journal within four weeks (31 days). If we fail to honor this by even one day, we will publish your manuscript free of charge and refund you the full article publication charges you paid for your manuscript.* This page is not a part of the published article. This page is an introduction to Edorium Journals and the publication services. * Terms and condition apply. Please see Edorium Journals website for more information.