PRIMITIVE NEUROECTODERMAL TUMOR OF THE ADRENAL GLAND
JOHN F. PIRANI, C. STEPHEN WOOLUMS, MEGAN K. DISHOP AND JAMES R. HERMANFrom the Division of Urology, and Department of Pathology and Laboratory Medicine, University of Kentucky, Lexington, Kentucky
KEY WORDS: neuroectodermal tumors, adrenal glands, nephrectomy
Primitive neuroectodermal tumors are rare lesions classi-fied in a group of small round cell tumors associated withhighly malignant neoplasms, such as Ewings sarcoma, neu-roblastoma and rhabdomyosarcoma.1 These tumors typicallydevelop in the pediatric population, and arise from the tho-racic and pulmonary regions. We report a case of an adrenalprimitive neuroectodermal tumor in adulthood with exten-sive tumor thrombus involving the right atrium.
A 57-year-old white man presented to a local emergencyroom complaining of progressive bilateral lower extremitypain and edema 2 months in duration. Duplex ultrasoundrevealed extensive bilateral deep venous thrombosis extend-ing proximal to the external iliac veins. Abdominopelvic mag-netic resonance imaging showed a 15 cm. adrenal mass withthrombus extending to the right atrium (fig. 1). Whole bodybone scan demonstrated no metastatic bony lesions. Urinarymetanephrine, 17-ketosteroid, aldosterone and free cortisolwere within normal limits.
Preoperatively medical evaluation was done and informedconsent was obtained. Right radical nephrectomy with tumorthrombectomy was performed using hypothermic circulatoryarrest and cardiopulmonary bypass in association with the
cardiothoracic service. The tumor did not infiltrate adjacentorgans but a dense desmoplastic reaction resulted in difficultdissection around the liver, duodenum and pancreas. Intra-operatively transesophageal echocardiography also showed alarge thrombus into the right atrium that filled the rightventricle during systole. Surgical pathological evaluationdemonstrated a peripheral 15 cm. primitive neuroectodermaltumor arising from the right adrenal gland. Tumor thrombusinvolved the inferior vena cava, extending to the rightatrium.
Histological examination revealed a monotonous pattern ofsmall round cells (fig. 2). Strongly positive immunohisto-chemical staining with CD-99 supported the diagnosis of aneoplasm within the Ewing sarcoma family of tumors. Fur-ther staining for neuron specific enolase was focally positive,chromogranin weakly positive and synaptophysin stronglypositive focally, indicating neural differentiation and con-firming the diagnosis of a peripheral primitive neuroectoder-mal tumor. Immunohistochemical staining for low molecularweight cytokeratin was negative. Cytogenetic analysis of cul-tured tumor cells revealed a reciprocal translocation of t(11;22)(q24;q12), characteristic of this malignancy. Pulmonaryartery lymph node 0/1 was negative for disease.
In the pediatric population primitive neuroectodermal tu-mors involving the chest wall are well known but genitourinaryinvolvement is rare in adulthood.2 To date 9 cases of a primaryrenal primitive neuroectodermal tumor have been reported3
but to our knowledge we report the first case of a primaryadrenal tumor. The biological behavior of this disease involvesrapid progression and infiltration of surrounding tissues. This
Accepted for publication January 18, 2000.The opinions and assertions contained herein are the private views
of the authors and are not to be construed as reflecting the views ofthe United States Navy or Department of Defense.
FIG. 1. Magnetic resonance image shows large adrenal tumorwith thrombus extending into right atrium.
FIG. 2. Primitive neuroectodermal tumor. Monotonous pattern ofundifferentiated small round cells surrounding occasional vascularstructures. H & E, reduced from 3500.
0022-5347/00/1636-1855/0THE JOURNAL OF UROLOGY Vol. 163, 18551856, June 2000Copyright 2000 by AMERICAN UROLOGICAL ASSOCIATION, INC. Printed in U.S.A.
behavior was described in renal lesions and we noted it in ourpatient with primary adrenal involvement. Extensive tumorthrombus added to the uniqueness of our case.
These neoplasms are easily misdiagnosed. The proper di-agnosis is made only by sophisticated immunostaining with ahigh index of suspicion. Currently surgical intervention re-mains the mainstay of treatment for local disease with theaddition of chemotherapeutic agents, including doxorubicin,vincristine, actinomycin D and cyclophosphamide, for localrecurrence or metastatic disease.
1. Martinez Ibanez, V., Abad, P., Toran, N. et al: Primitive neuro-ectodermal tumors: difficult tumors versus modern oncology.Cir Pediatr, 11: 5, 1998
2. Shamberger, R. C., Tarbell, N. J., Perez-Atayde, A. R. et al:Malignant small round cell tumor (Ewings-PNET) of the chestwall in children. J Pediatr Surg, 29: 179, 1994
3. Quezado, M., Benjamin, D. R. and Tsokos, M.: EWS/FLI-1 fusiontranscripts in three peripheral primitive neuroectodermal tu-mors of the kidney. Hum Pathol, 28: 767, 1997
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PRIMITIVE NEUROECTODERMAL TUMOR OF THE ADRENAL GLANDCASE REPORTDISCUSSIONREFERENCES