A teen aged girl with rapidly developing pelvic mass, associated with weight loss and left leg weakness, was evaluated and found to have Ewing\u27s sarcoma of non-osseous origin from pelvis, which is a rare situation. She was treated by a multidisciplinary approach by surgery, chemotherapy and rehabilitation by physiotherapy to effective response

Naru, Tahira

Nawaz, Fauzia Haq

Rizvi, Javed

English

eCommons@AKU

eCommons@AKUDepartment of Obstetrics & Gynaecology Division of Woman and Child HealthJanuary 2007Juvenile ewing sarcoma presenting as a pelvic massTahira NaruAga Khan University, tahira.naru@aku.eduFauzia Haq NawazAga Khan University, fauzia.nawaz@aku.eduJaved RizviAga Khan University, javed.rizvi@aku.eduFollow this and additional works at: http://ecommons.aku.edu/pakistan_fhs_mc_women_childhealth_obstet_gynaecolPart of the Female Urogenital Diseases and Pregnancy Complications Commons, and theObstetrics and Gynecology CommonsRecommended CitationNaru, T., Nawaz, F. H., Rizvi, J. (2007). Juvenile ewing sarcoma presenting as a pelvic mass. Journal of the College of Physicians andSurgeons Pakistan :JCPSP, 17(1), 53-54.Available at: http://ecommons.aku.edu/pakistan_fhs_mc_women_childhealth_obstet_gynaecol/69JCPSP 2007, Vol. 17 (1): 53-54 53INTRODUCTIONEwing’s sarcoma is a highly malignant tumor of osseous ornon-osseous origin. It is the second most common bone tumorof childhood and adolescence. More than half of all patientspresent in the second decade of life and is uncommon inadults older than 30 years. There is a male preponderance ofapproximately 1.5:1. Ewing’s sarcoma rarely occurs in blackand Chinese children.1The Ewing’s sarcoma usually arises in the metaphysis ordiaphysis of the long bones of extremities. Pelvic area, ribsand scapulae are less frequent sites although any bone can beinvolved. The lungs, bones and bone marrow are the mostcommon sites of metastasis.1, 2 This report describes a caseof extra osseous Ewing sarcoma of the pelvic in teen-agefemale.CASE REPORTA 15-year-old Pakistani girl presented in the clinic in August2004 with lower abdominal pain, palpable abdominal massand left leg weakness. Since 2 months, the abdomen hadincreased in size with lower abdominal discomfort andsignificant loss of appetite and weight.  It was followed, a fewweeks later, by sudden onset of left lower limb weakness andmuscular atrophy with difficulty in walking and severe painstarting from back of gluteal region to mid calf.She was dehydrated and emaciated. Her vital signs andgeneral examination were normal. On abdominal examination,a mass was palpated from left lateral pelvic wall, reaching tojust below the epigastrium, which was firm, well circumscribedand non-tender. Liver and spleen were not palpable andshifting dullness was absent. There was no lymphadenopathy.Specific neurological examination of the lower limbs revealedleft leg muscular atrophy with less sensory motor power andfoot drop. Complete blood counts, coagulation profile, renal and liverfunctions were normal. Laboratory investigation revealedhemoglobin of 10.9 gm/dl, hematocrit of 40.1%, total leukocytecount of 10,000/mm3, platelet count of 275,000 /mm3 and ESRof 39 mm/hr.  Tumor markers CA-125          (73.63 iu/ml) andLDH (1100 iu/L) were raised. Serum humanchoriongonadotrophin and alpha feto-protein were in normalrange (<2.010 and 1.76 respectively). Chest X-ray showedno evidence of pleural effusion or any other  active lesion. Ultrasonography of liver, spleen, pancreas and gall-bladderwere normal. No para-aortic lymph nodes were seen. Due topressure effect of the mass, there was  bilateral pelvicalycealdilatation by 1 cm. A solid mass of 17.9 x 9.9 cm was seen inlower abdomen with possibility of uterine sarcoma. Bothovaries were normal.Doppler ultrasound showed high vascular tumor withpossibility of sarcoma. MRI  of pelvis revealed a solid mass of18.0 x 12.0 cm, arising from pelvis and left adnexa, involvingthe upper part of vagina, obturator internus and gluteusminimus  muscles   (Figure 1a  and 1b).Because of retroperitoneal nature of tumor and suspicion ofuterine sarcoma laparotomy was performed.  Pelvic peritonealwashing were obtained but there was no free fluid in peritonealcavity.  A large retroperitoneal mass of 18 x12 cm was arisingfrom left lateral pelvic wall to hollow of sacrum, and denselyadherent to the posterior wall of uterus.  Both ovaries werenormal.  Due to high vascularity of tumor, partial debulking wasperformed.  Hemostasis was secured. Frozen section ofspecimen revealed malignant undifferentiated anaplasticsarcoma with differential diagnosis of Ewing’s sarcoma orlymphoma.  Postoperatively, she was kept in Special Care Unit(SCU) and received packed cell transfusion and furtherhospital course was unremarkable.CASE REPORTJUVENILE EWING  SARCOMA PRESENTING AS A PELVIC MASS Tahira Naru, Fauzia Haq Nawaz and Javed Rizvi ABSTRACTA teen aged girl with rapidly developing pelvic mass, associated with weight loss and left leg weakness, was evaluated and found tohave Ewing’s sarcoma of non-osseous origin from pelvis, which is a rare situation. She was treated by a multidisciplinary approachby surgery, chemotherapy and rehabilitation by physiotherapy to effective response.KEY WORDS: Pelvis mass.    Juvenile.    Ewing sarcoma.   Non-osseus.Department of Obstetrics and Gynecology. The Aga Khan University Hospital,Karachi-74800.Correspondence: Dr. Fauzia Haq Nawaz, Department of Obstetrics and Gynecology,The Aga Khan University Hospital, P.O. Box 3500, Karachi-74800. E-mail:fuzia.nawaz@aku.edu.Received June 13, 2006;  accepted  November 20, 2006.Figure 1a and 1b:  T2 weighted MRI showing the pelvis tumour extent in sagittal(a) and  (b) axial sections.54 JCPSP 2007, Vol. 17 (1): 53-54The diagnosis of Ewing’s sarcoma was supported by theidentification of glycogen within the tumor cells and by thedemonstration of the MIC2 gene product on the tumor cellmembrane. The immunohistochemical marker (vimentin andS-100) were positive. Bone scan and bone marrow biopsywere performed, which were negative for metastasis.Emphasis was given on limb physiotherapy with variousexercises.  On 6th postoperative day, she received  first cycleof multi-agent chemotherapy of IVAC (Ifosfamide, vincristine,adriamycin, cyclophosphamide).  She was discharged on 10thpostoperative day.  On follow-up visit, the pelvic mass wasregressing, so patient and her parents were counseled aboutcompliance for further chemotherapy cycles.  After 9 cycles ofIVAC, tumor was not palpable abdominally. A repeatultrasound revealed no pelvic mass. On follow up visits, shewas doing well.DISCUSSION Ewing’s sarcoma is the second most common malignant bonetumor in young age group, and is commonly diagnosed in thesecond decade with male preponderance. The annualincidence rate averages less than 2 cases per millionchildren.1,3 In addition to primitive neuroectodermal tumor ofthe uterus and mesenchymal lesion of the uterine cervix, thereare reports of pelvic mass of osseous and non-osseous originin either gender. 4-6A delay in early symptoms and diagnosis is quite common,particularly of pelvic tumors in which this mass is not palpableuntil it is quite large.1,7 The most important and earliestsymptom is pain, which may radiate to the limbs, especiallywith tumor in the vertebral or pelvic region. Neurological signssuch as nerve root sign or cord compression are present inone-half of patients with the involvement of axial skeleton.Patient with metastatic   Ewing’s sarcoma may also presentwith systemic signs and symptoms such as weight loss andfever. 1This patient presented with lower abdominal pain, largepalpable pelvic mass, left leg weakness, weight loss, andfever.  Laboratory studies in this case showed an increasederythrocyte sedimentation rate, elevated lactatedehydrogenate (LDH), and absence of metastasis. The majorprognostic factors in Ewing’s sarcoma are: age, site (pelvis,sacrum), volume of tumor (more than 10 cm), and presence ofmetastasis at the time of diagnosis.7MRI imaging is preferred method in assessing the involvementof soft tissue and bone marrow. Molecular and cytogeneticanalysis should be considered as the standard practice in thediagnostic evaluation of Ewing’s sarcoma.1With the advent of modern chemotherapy, control of localdisease has become more important with increased long-termsurvival rates. A recent study of Ewing’s sarcoma of the pelvisalso found an increased 5 years survival rate for patients whohad resection of their primary tumor. The patients with non-metastatic Ewing’s sarcoma, treated with chemotherapy andoperative resection had a 75% overall 5-year survival rate,compared with 25% overall survival rate where surgery wasnot performed.  Surgery eliminates the residual tumor that areleft behind after chemo- or radiation therapy.4,7The development of multi-agent chemotherapy has increasedthe survival rate of patients with Ewing’s sarcoma from 10 %to 70%. More recently, Ifosfamide has emerged as a veryeffective chemotherapeutic agent, especially in patientsresistant to other drugs. With multidisciplinary treatment, long-term survival can be achieved in 70 to 80% of patientspresenting with non metastatic disease.7-9 In this case,laparotomy was planned first due to the suspicion of uterinesarcoma, and the mass being retroperitoneal. With thisapproach, not only histological confirmation of Ewing’ssarcoma was possible, but also more than  half of the tumorvolume was reduced and further tumor reduction was done bymeans of multiagent chemotherapy.Surgical resection, multiagent chemotherapy and radio-therapyare  the mainstay of treatment. The treatment plan should beindividualized for each patient, which should be based on age,location, stage, size of the tumor and response to therapy. REFERENCES1. Strauss LG. Ewing’s sarcoma, [online]2005 [cited 2006 Nov 2]Available from URL: http: // www.emedicine.com/radio/topic 275 htm. 2. Primitive neuroectodermal tumors and related lesions. In: Weiss SW,Goldblum JR, (edi). Enzinger and Weiss’s soft tissue tumors. 4th ed. StLouis: Mosby, 2001: 1265-321.3. Shah SH, Muzaffar S, Soomro IN, Pervez S, Hasan SH.  Clinico –morphological pattern and frequency of bone cancer. J Pak Med Assoc1999; 49: 110-2. 4. Gostout BS, Lindor NM, DiMarco CS, Peethambaram PP, Clayton AC.Pelvic primitive neuroectodermal tumor associated with a cluster ofsmall round cell tumors: case report and review of current literature.Gynecol Oncol 2003; 91: 247-53.5. Karseladze AI, Filipova NA, Navarro S, Llombart–Bosch A. Primitiveneuroectodermal tumor of the uterus: a case report.            J ReprodMed 2001; 46: 845-8.6. Frassica FJ, Frassica DA, Pritchard DJ, Schomberg PJ, World LE, SimFH. Ewing’s sarcoma of the pelvis. Clinicopathological features andmanagement. J Bone Joint Surg Am 1993; 75: 1457-65.7. Vlasak R, Sim FH. Ewing’s sarcoma. Orthop Clin North Am 1996; 27:591-603.8. Aslam E, Imran M. Faridi NM. Bilateral parietal extradural metastaticEwing’s sarcoma simulating acute epidural hematoma. J CollPhysicians Surg Pak 2006; 16: 543-4.9. Approach to the diagnosis of soft tissue tumor. In: Weiss SW, GoldblumJR, (edi). Enzinger and Weiss’s soft tissue tumors. 4th ed. St Louis:Mosby, 2001: 189-97.Tahira Naru, Fauzia Haq Nawaz and Javed Rizvi l l l l lO l l l l l

Juvenile ewing sarcoma presenting as a pelvic mass

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Juvenile ewing sarcoma presenting as a pelvic mass

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