///2009 Abstract Details
2009 Abstract Details2018-05-01T17:45:11+00:00

First Reported Case of Vaginal Delivery in a Patient with an Extraosseus Ewing's Sarcoma of the Adrenal Gland

Abstract Number: 205
Abstract Type: Case Report/Case Series

Michelle R. Beam D.O.,M.B.A.1 ; H. Jane Huffnagle D.O., F.A.O.C.A.2; Suzanne Huffnagle D.O., F.A.O.C.A.3; Elia Elia M.D.4; Wlodzimierz Grodecki M.D.5; Aseem Gupta M.D.6

INTRODUCTION: Ewings Sarcoma usually presents in childhood as a skeletal tumor. We present a case of extraskeletal Ewings Sarcoma diagnosed during pregnancy.

CASE REPORT: A 31 y/o G4P2 Hispanic woman with an adrenal mass discovered at 24 weeks gestation presented for delivery. Initial diagnosis of the 14x13.4x10cm complex mass was an adrenal hematoma. PMH included an ectopic pregnancy with salpingectomy five years prior. Pheochromacytoma workup was negative. A dexamethasone suppression test was positive and the patient received stress dose steroids and dilaudid for severe tumor pain. At 33 weeks, the mass had grown to 14.1x23.2x26 cm. Differential diagnosis now included adrenal cortical carcinoma, lymphoma, and ovarian carcinoma. The decision was made to proceed with a vaginal delivery under epidural anesthesia (definitive surgical treatment later). Labor was induced with oxytocin, an epidural was placed, and a baby girl was delivered without complication. On post delivery day #32, an exploratory laparotomy, ascites evacuation, and biopsy was performed. No resection was possible secondary to tumor size and encasement of the celiac axis and aorta. Pathology revealed an extraskeletal Ewings Sarcoma (primitive neuroectodermal tumor - PNET). The patient underwent tumor embolization and began aggressive chemotherapy.

DISCUSSION: Malignancies in pregnancy are uncommon (0.02% to 0.10% of all pregnancies (1)), with adrenal tumors even more so. A Mayo Clinic review found only 4 cases in pregnancy over 22 years - 1 pheochromacytoma; 1 neoplasm associated with Von Hippel Lindau disease; and 2 benign adrenal adenomas (2). Ewings sarcoma, especially PNET is extremely rare during pregnancy. Of 7 reported cases of peripartum Ewings sarcoma, only 1 was extraosseous, 2 had symptoms beginning after conception, and all were delivered via C/S (3,4,5). When PNET occurs in a solid organ, it has been found in the kidney (most often), ovaries, uterus, and lung (6). The maternal prognosis is poor, but fetal outcomes have been good despite chemotherapy prior to delivery. Anesthetic considerations in our patient included a pheochromacytoma workup, adrenal function testing, mode of delivery (possibility of severe hemorrhage or tumor rupture) pain issues, and possible tumor resection at time of delivery.

CONCLUSION: This is the first reported case of a vaginal delivery in a patient with PNET arising from the adrenal gland during pregnancy. These rare tumors are difficult to treat and require cooperation of a diverse medical team.

REFERENCES:

1. Gambling DR, Douglas MJ, McKay RSF. Obstetric Anesthesia and

Uncommon Disorders, Cambridge University Press (2008):371.

2. Harrington J, et al. World J Surg 1999;23:182-6.

3. Loguidice V, et al. Clin Orthop Relat Res 1986;210:132-136.

4. Merimsky O., et al. Ann Onc 1999;10:345-50.

5. Haerr R, et al. Cancer 1985; 56:1028-33.

6. Adair A., et al. J.R.Coll Surg Edinb 2001; 46:372-4.

SOAP 2009