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PRIMITIVE NEUROECTODERMAL TUMOR OF THE ADRENAL GLAND
0022-5347/00/1636-1855/0 THE JOURNAL OF UROLOGY® Copyright © 2000 by AMERICAN UROLOGICAL ASSOCIATION, INC.®
Vol. 163, 1855–1856, June 2000 Printed in U.S.A.
Case Reports PRIMITIVE NEUROECTODERMAL TUMOR OF THE ADRENAL GLAND JOHN F. PIRANI, C. STEPHEN WOOLUMS, MEGAN K. DISHOP
AND
JAMES R. HERMAN
From 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 classified in a group of small round cell tumors associated with highly malignant neoplasms, such as Ewing’s sarcoma, neuroblastoma and rhabdomyosarcoma.1 These tumors typically develop in the pediatric population, and arise from the thoracic and pulmonary regions. We report a case of an adrenal primitive neuroectodermal tumor in adulthood with extensive tumor thrombus involving the right atrium. CASE REPORT
A 57-year-old white man presented to a local emergency room complaining of progressive bilateral lower extremity pain and edema 2 months in duration. Duplex ultrasound revealed extensive bilateral deep venous thrombosis extending proximal to the external iliac veins. Abdominopelvic magnetic resonance imaging showed a 15 cm. adrenal mass with thrombus extending to the right atrium (fig. 1). Whole body bone scan demonstrated no metastatic bony lesions. Urinary metanephrine, 17-ketosteroid, aldosterone and free cortisol were within normal limits. Preoperatively medical evaluation was done and informed consent was obtained. Right radical nephrectomy with tumor thrombectomy was performed using hypothermic circulatory arrest and cardiopulmonary bypass in association with the 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 of the United States Navy or Department of Defense.
cardiothoracic service. The tumor did not infiltrate adjacent organs but a dense desmoplastic reaction resulted in difficult dissection around the liver, duodenum and pancreas. Intraoperatively transesophageal echocardiography also showed a large thrombus into the right atrium that filled the right ventricle during systole. Surgical pathological evaluation demonstrated a peripheral 15 cm. primitive neuroectodermal tumor arising from the right adrenal gland. Tumor thrombus involved the inferior vena cava, extending to the right atrium. Histological examination revealed a monotonous pattern of small round cells (fig. 2). Strongly positive immunohistochemical staining with CD-99 supported the diagnosis of a neoplasm within the Ewing sarcoma family of tumors. Further staining for neuron specific enolase was focally positive, chromogranin weakly positive and synaptophysin strongly positive focally, indicating neural differentiation and confirming the diagnosis of a peripheral primitive neuroectodermal tumor. Immunohistochemical staining for low molecular weight cytokeratin was negative. Cytogenetic analysis of cultured tumor cells revealed a reciprocal translocation of t(11; 22)(q24;q12), characteristic of this malignancy. Pulmonary artery lymph node 0/1 was negative for disease. DISCUSSION
In the pediatric population primitive neuroectodermal tumors involving the chest wall are well known but genitourinary involvement is rare in adulthood.2 To date 9 cases of a primary renal primitive neuroectodermal tumor have been reported3 but to our knowledge we report the first case of a primary adrenal tumor. The biological behavior of this disease involves rapid progression and infiltration of surrounding tissues. This
FIG. 2. Primitive neuroectodermal tumor. Monotonous pattern of FIG. 1. Magnetic resonance image shows large adrenal tumor undifferentiated small round cells surrounding occasional vascular with thrombus extending into right atrium. structures. H & E, reduced from ⫻500. 1855
1856
PRIMITIVE NEUROECTODERMAL TUMOR OF ADRENAL GLAND
behavior was described in renal lesions and we noted it in our patient with primary adrenal involvement. Extensive tumor thrombus added to the uniqueness of our case. These neoplasms are easily misdiagnosed. The proper diagnosis is made only by sophisticated immunostaining with a high index of suspicion. Currently surgical intervention remains the mainstay of treatment for local disease with the addition of chemotherapeutic agents, including doxorubicin, vincristine, actinomycin D and cyclophosphamide, for local recurrence or metastatic disease.
REFERENCES
1. Martinez Ibanez, V., Abad, P., Toran, N. et al: Primitive neuroectodermal 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 (Ewing’s-PNET) of the chest wall in children. J Pediatr Surg, 29: 179, 1994 3. Quezado, M., Benjamin, D. R. and Tsokos, M.: EWS/FLI-1 fusion transcripts in three peripheral primitive neuroectodermal tumors of the kidney. Hum Pathol, 28: 767, 1997
Vol. 163, 1855–1856, June 2000 Printed in U.S.A.
Case Reports PRIMITIVE NEUROECTODERMAL TUMOR OF THE ADRENAL GLAND JOHN F. PIRANI, C. STEPHEN WOOLUMS, MEGAN K. DISHOP
AND
JAMES R. HERMAN
From 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 classified in a group of small round cell tumors associated with highly malignant neoplasms, such as Ewing’s sarcoma, neuroblastoma and rhabdomyosarcoma.1 These tumors typically develop in the pediatric population, and arise from the thoracic and pulmonary regions. We report a case of an adrenal primitive neuroectodermal tumor in adulthood with extensive tumor thrombus involving the right atrium. CASE REPORT
A 57-year-old white man presented to a local emergency room complaining of progressive bilateral lower extremity pain and edema 2 months in duration. Duplex ultrasound revealed extensive bilateral deep venous thrombosis extending proximal to the external iliac veins. Abdominopelvic magnetic resonance imaging showed a 15 cm. adrenal mass with thrombus extending to the right atrium (fig. 1). Whole body bone scan demonstrated no metastatic bony lesions. Urinary metanephrine, 17-ketosteroid, aldosterone and free cortisol were within normal limits. Preoperatively medical evaluation was done and informed consent was obtained. Right radical nephrectomy with tumor thrombectomy was performed using hypothermic circulatory arrest and cardiopulmonary bypass in association with the 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 of the United States Navy or Department of Defense.
cardiothoracic service. The tumor did not infiltrate adjacent organs but a dense desmoplastic reaction resulted in difficult dissection around the liver, duodenum and pancreas. Intraoperatively transesophageal echocardiography also showed a large thrombus into the right atrium that filled the right ventricle during systole. Surgical pathological evaluation demonstrated a peripheral 15 cm. primitive neuroectodermal tumor arising from the right adrenal gland. Tumor thrombus involved the inferior vena cava, extending to the right atrium. Histological examination revealed a monotonous pattern of small round cells (fig. 2). Strongly positive immunohistochemical staining with CD-99 supported the diagnosis of a neoplasm within the Ewing sarcoma family of tumors. Further staining for neuron specific enolase was focally positive, chromogranin weakly positive and synaptophysin strongly positive focally, indicating neural differentiation and confirming the diagnosis of a peripheral primitive neuroectodermal tumor. Immunohistochemical staining for low molecular weight cytokeratin was negative. Cytogenetic analysis of cultured tumor cells revealed a reciprocal translocation of t(11; 22)(q24;q12), characteristic of this malignancy. Pulmonary artery lymph node 0/1 was negative for disease. DISCUSSION
In the pediatric population primitive neuroectodermal tumors involving the chest wall are well known but genitourinary involvement is rare in adulthood.2 To date 9 cases of a primary renal primitive neuroectodermal tumor have been reported3 but to our knowledge we report the first case of a primary adrenal tumor. The biological behavior of this disease involves rapid progression and infiltration of surrounding tissues. This
FIG. 2. Primitive neuroectodermal tumor. Monotonous pattern of FIG. 1. Magnetic resonance image shows large adrenal tumor undifferentiated small round cells surrounding occasional vascular with thrombus extending into right atrium. structures. H & E, reduced from ⫻500. 1855
1856
PRIMITIVE NEUROECTODERMAL TUMOR OF ADRENAL GLAND
behavior was described in renal lesions and we noted it in our patient with primary adrenal involvement. Extensive tumor thrombus added to the uniqueness of our case. These neoplasms are easily misdiagnosed. The proper diagnosis is made only by sophisticated immunostaining with a high index of suspicion. Currently surgical intervention remains the mainstay of treatment for local disease with the addition of chemotherapeutic agents, including doxorubicin, vincristine, actinomycin D and cyclophosphamide, for local recurrence or metastatic disease.
REFERENCES
1. Martinez Ibanez, V., Abad, P., Toran, N. et al: Primitive neuroectodermal 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 (Ewing’s-PNET) of the chest wall in children. J Pediatr Surg, 29: 179, 1994 3. Quezado, M., Benjamin, D. R. and Tsokos, M.: EWS/FLI-1 fusion transcripts in three peripheral primitive neuroectodermal tumors of the kidney. Hum Pathol, 28: 767, 1997