■•■•.'1991-92 S. Karger AG. Brnel 10 16-2291/91 -92/0172-0139$ 2.75/0
Pediatr Neurosurg 1991-92:17:139-141
Epidural Metastasis of Testicular Yolk Sac Tumor: An Unusual Cause of Spinal Cord Compression Case Report Ahmet Çolak, Kemal Benli, Mustafa Berker. Behsan Ö?iol Departments of Neurosurgery and Pathology, Hacettepe University School of Medicine. Ankara. Turkey
Key Words. Metastasis • Spinal cord compression • Yolk sac tumor Abstract. Testicular tumors in the pediatric age group are rare. Yolk sac tumor (YST) is the most common malig nant tumor of germinal origin in this group, and it may spread to the retroperitoneal lymph nodes. We have been unable to find any previously published report of metastasis of testicular YST to the epidural space of the spinal cord present ing as a cause of spinal cord compression.
Testicular tumors in children are rare, representing about 2% of all testicular tumors seen [ 1—4J. Among ma lignant childhood tumors, they are seventh in frequency [1.3]. Yolk sac tumor (YST) is the most common malig nant primary testicular tumor of germinal origin in child hood 11-3, 5]. It is also known as Teilum's tumor, orchioblastoma, embryonal cell carcinoma, endodermal sinus tumor, infantile adenocarcinoma, adenocarcinoma with clear cells, and embryonal adenocarcinoma of the infant testis 11, 2], Although YST is usually located in the testis and most often spreads to the adjacent tissues, retroperitoneal lymph node metastases may be observed in a few cases [6, 7|. We report a case of testicular YST with metastasis to the spinal epidural space in a 4-year-old boy. In a review of the literature we failed to find any other report relating to spinal cord compression caused by testicular YST [ 1- 10].
Case Report A 4-year-old hoy was admitted to the Department of Neurosurgery. Hacettepe University School of Medicine with a 10-day history of pro gressive paraparesis and neurogenic bladder. He had been operated on 6 months previously in the Department of Pediatric Surgery because of a testicular mass. Histopathological exam ination of the material revealed YST of the testis. 6.000 rad radiation was given postoperatively and chemotherapy, consisting of vincristine, actinomycin D and cyclophosphamide, was administered. The preoper ative a-fetoprotein (AFP) value of 140 ng/ml in the serum came down to normal in the postoperative period (normal: 2-10 ng/ml). On physical and neurological examination on admission the second time, inguinal lymphadenopathy and hepatomegaly were found. Cra nial nerves were intact. He had paraparesis and hypocsthesia below the T 10 level. Deep tendon reflexes were hypoaclive in the lower ex tremities. He also had urinary retention. AFP level in the serum was 350 ng/ml. Plain X ray and myelography were performed. Lumbar myelogra phy with metrizamide contrast revealed a spinal block in the T I l-L I region (fig. I). Urgent surgical intervention was performed. After lami nectomies of T 11. T 12, and L I. a huge gray-yellow tumoral mass cov ering the epidural space like a cuff was seen and removed subtotally. The dura mater was intact. Histopathological examination showed me-
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Introduction
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tastasis of the testicular YST (fig. 2). During the postoperative period, chemotherapy, consisting of vincristine, adriamycin. cyclophospha mide and dacarbazine (DTIC), was administered and radiation was given. A lull rehabilitation scheme was started. Inguinal lymphadenopathics, hepatomegaly, and bladder dysfunction decreased and patient could walk. He was alive 6 months after the second operation.
Discussion YST is the seventh most common pediatric neoplasm representing 1% of all malignant tumors in children [1,3]. It secretes AFP which can be detected in over 80% of the patients with YST |5, 7]. Therefore, it can be used as a re liable marker in the diagnosis and follow-up of the dis ease. In our case, AFP was found to be increased at the time of metastasis. YST behaves in a more benign fashion than other testicular tumors. To our knowledge, epidural metastasis of this tumor has not yet been reported in the English medical literature [ 1-10]. Breast, lung, and prostate tumors are those best known for metastasizing to the spinal epidural space [8, 10|. Compression of the spinal cord is a major cause of mor bidity in these cases. Several pathways are implicated in the pathogenesis of neoplastic epidural cord compression; they include hematogenous metastasis to the vertebral col umn, direct extension from a paravertebral tumoral mass,
spread along the perineurium of the spinal nerves, or di rect extension through the intervertebral foramina by lym phatic dissemination. In adults, the metastasis generally invades the epidural space from a vertebral site, whereas in children the epidural space is mostly infiltrated by di rect spread from paraspinous sites |8. 1()|. In our case, we thought that there had been lymphatic dissemination from the retroperitoneal lymph nodes involved by the testicular YST or direct spread from the retroperitoneal tumoral mass. We wish to point out that this tumor may metasta size to the spinal epidural space and that it should be in cluded in the differential of causes of metastatic spinal cord compression.
References 1 Brosman SGB: Testicular tumors in children: in Johnson JH. Goodvin WE (eds): Reviews in Paediatric Urology. New York. American Elsevier Publishing, 1974. vol. 7. pp 131—139. 2 Exelby PR: Testicular cancer in children. Cancer 1980:45: 1803-1809. 3 Green DM: The treatment of advanced or recurrent malignant genitourinary tumors in children. Cancer 1987:60:602-611. 4 Kaplan GW: Yolk sac tumors. Dialog Pediatr Urol 1982:5:1-12. 5 Pierce BG, Bullock WK. Huntington RW: Yolk sac tumors of testis. Cancer 1970:25:644-657. 6 Caldwell WL: Why retroperitoneal lymphadcncctomy for testicular tumors? J Urol 1978:119:754-758.
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Fig. 1. Lumbar myelography showing a spinal block.
Fig. 2. Microscopic findings of resected tumor show typical yolk sac neoplasm, consisting of clear cells seperaied by densely staining embryonic endodermal cells, x 460.
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Yolk Sac Tumor
Editorial Comment Spinal cord compression by extradural tumor that is metastatic from a distant source is rare in children, in con trast to adults, and it is worthwhile to be reminded o f this unusual possibility. Despite this rarity, epidural metastases o f endodermal sinus (yolk sac) tumor have been re ported in the past, even if not specifically o f testicular or igin [ 1-3 f Perhaps o f greater importance here is the reminder that interval measurement o f appropriate tumor markers can be advantageous in the continuing care o f tumor-bear ing patients. An earlier appreciation o f elevated a-fetoprotein and prompt nonsurgical treatment might have
10 Wright RL: Malignant tumors in the spinal extradural space. Results of surgical treatment. Ann Surg 1960:157:227-231. Ahmet Çolak, MD Tip Fakiiltcsi Caddesi 36/5 Abidinpasa Ankara (Turkey)
averted a neurosurgical emergency in this case. The au thors are to be congratulated on an excellent outcome in a difficult situation. David M. Klein. MD References 1 Baten M. Vannucci RC: Intraspinal metastatic disease in childhood cancer. J Pediatr 1977:90:207-212. 2 Lewis DW. Packer RJ. Raney B. Rak IW. Bclasco J. Lange B: Inci dence. presentation, and outcome of spinal cord disease in children with systemic cancer. Pediatrics 1986:78:438-443. 3 Raffel C. Ncave VCD. Lavine S. McComb JG: Treatment of spinal cord compression by epidural malignancy in childhood. Neurosur gery 1991;28:349-352.
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7 Flameni F. Nlhoul-Feleke C, Lemcrle J: Optimal treatment of clini cal stage I yolk sac tumor of the testis in children. J Pediatr Surg 1986:21:108-111. 8 Allen CJ: Management of metastatic epidural disease in children. J Pediatr 1984;104:241-242. 9 Nelson K, Sten/.el P: Intrapcricardial yolk sac tumor in an infant girl. Cancer 1987:60:1567-1569.
Pediatr Neurosurg 1991-92:17:139-141
Epidural Metastasis of Testicular Yolk Sac Tumor: An Unusual Cause of Spinal Cord Compression Case Report Ahmet Çolak, Kemal Benli, Mustafa Berker. Behsan Ö?iol Departments of Neurosurgery and Pathology, Hacettepe University School of Medicine. Ankara. Turkey
Key Words. Metastasis • Spinal cord compression • Yolk sac tumor Abstract. Testicular tumors in the pediatric age group are rare. Yolk sac tumor (YST) is the most common malig nant tumor of germinal origin in this group, and it may spread to the retroperitoneal lymph nodes. We have been unable to find any previously published report of metastasis of testicular YST to the epidural space of the spinal cord present ing as a cause of spinal cord compression.
Testicular tumors in children are rare, representing about 2% of all testicular tumors seen [ 1—4J. Among ma lignant childhood tumors, they are seventh in frequency [1.3]. Yolk sac tumor (YST) is the most common malig nant primary testicular tumor of germinal origin in child hood 11-3, 5]. It is also known as Teilum's tumor, orchioblastoma, embryonal cell carcinoma, endodermal sinus tumor, infantile adenocarcinoma, adenocarcinoma with clear cells, and embryonal adenocarcinoma of the infant testis 11, 2], Although YST is usually located in the testis and most often spreads to the adjacent tissues, retroperitoneal lymph node metastases may be observed in a few cases [6, 7|. We report a case of testicular YST with metastasis to the spinal epidural space in a 4-year-old boy. In a review of the literature we failed to find any other report relating to spinal cord compression caused by testicular YST [ 1- 10].
Case Report A 4-year-old hoy was admitted to the Department of Neurosurgery. Hacettepe University School of Medicine with a 10-day history of pro gressive paraparesis and neurogenic bladder. He had been operated on 6 months previously in the Department of Pediatric Surgery because of a testicular mass. Histopathological exam ination of the material revealed YST of the testis. 6.000 rad radiation was given postoperatively and chemotherapy, consisting of vincristine, actinomycin D and cyclophosphamide, was administered. The preoper ative a-fetoprotein (AFP) value of 140 ng/ml in the serum came down to normal in the postoperative period (normal: 2-10 ng/ml). On physical and neurological examination on admission the second time, inguinal lymphadenopathy and hepatomegaly were found. Cra nial nerves were intact. He had paraparesis and hypocsthesia below the T 10 level. Deep tendon reflexes were hypoaclive in the lower ex tremities. He also had urinary retention. AFP level in the serum was 350 ng/ml. Plain X ray and myelography were performed. Lumbar myelogra phy with metrizamide contrast revealed a spinal block in the T I l-L I region (fig. I). Urgent surgical intervention was performed. After lami nectomies of T 11. T 12, and L I. a huge gray-yellow tumoral mass cov ering the epidural space like a cuff was seen and removed subtotally. The dura mater was intact. Histopathological examination showed me-
Downloaded by: Stockholm University Library 130.237.165.40 - 1/20/2019 8:34:45 AM
Introduction
Çolak/Bcnli/Berker/Ônol
140
tastasis of the testicular YST (fig. 2). During the postoperative period, chemotherapy, consisting of vincristine, adriamycin. cyclophospha mide and dacarbazine (DTIC), was administered and radiation was given. A lull rehabilitation scheme was started. Inguinal lymphadenopathics, hepatomegaly, and bladder dysfunction decreased and patient could walk. He was alive 6 months after the second operation.
Discussion YST is the seventh most common pediatric neoplasm representing 1% of all malignant tumors in children [1,3]. It secretes AFP which can be detected in over 80% of the patients with YST |5, 7]. Therefore, it can be used as a re liable marker in the diagnosis and follow-up of the dis ease. In our case, AFP was found to be increased at the time of metastasis. YST behaves in a more benign fashion than other testicular tumors. To our knowledge, epidural metastasis of this tumor has not yet been reported in the English medical literature [ 1-10]. Breast, lung, and prostate tumors are those best known for metastasizing to the spinal epidural space [8, 10|. Compression of the spinal cord is a major cause of mor bidity in these cases. Several pathways are implicated in the pathogenesis of neoplastic epidural cord compression; they include hematogenous metastasis to the vertebral col umn, direct extension from a paravertebral tumoral mass,
spread along the perineurium of the spinal nerves, or di rect extension through the intervertebral foramina by lym phatic dissemination. In adults, the metastasis generally invades the epidural space from a vertebral site, whereas in children the epidural space is mostly infiltrated by di rect spread from paraspinous sites |8. 1()|. In our case, we thought that there had been lymphatic dissemination from the retroperitoneal lymph nodes involved by the testicular YST or direct spread from the retroperitoneal tumoral mass. We wish to point out that this tumor may metasta size to the spinal epidural space and that it should be in cluded in the differential of causes of metastatic spinal cord compression.
References 1 Brosman SGB: Testicular tumors in children: in Johnson JH. Goodvin WE (eds): Reviews in Paediatric Urology. New York. American Elsevier Publishing, 1974. vol. 7. pp 131—139. 2 Exelby PR: Testicular cancer in children. Cancer 1980:45: 1803-1809. 3 Green DM: The treatment of advanced or recurrent malignant genitourinary tumors in children. Cancer 1987:60:602-611. 4 Kaplan GW: Yolk sac tumors. Dialog Pediatr Urol 1982:5:1-12. 5 Pierce BG, Bullock WK. Huntington RW: Yolk sac tumors of testis. Cancer 1970:25:644-657. 6 Caldwell WL: Why retroperitoneal lymphadcncctomy for testicular tumors? J Urol 1978:119:754-758.
Downloaded by: Stockholm University Library 130.237.165.40 - 1/20/2019 8:34:45 AM
Fig. 1. Lumbar myelography showing a spinal block.
Fig. 2. Microscopic findings of resected tumor show typical yolk sac neoplasm, consisting of clear cells seperaied by densely staining embryonic endodermal cells, x 460.
141
Yolk Sac Tumor
Editorial Comment Spinal cord compression by extradural tumor that is metastatic from a distant source is rare in children, in con trast to adults, and it is worthwhile to be reminded o f this unusual possibility. Despite this rarity, epidural metastases o f endodermal sinus (yolk sac) tumor have been re ported in the past, even if not specifically o f testicular or igin [ 1-3 f Perhaps o f greater importance here is the reminder that interval measurement o f appropriate tumor markers can be advantageous in the continuing care o f tumor-bear ing patients. An earlier appreciation o f elevated a-fetoprotein and prompt nonsurgical treatment might have
10 Wright RL: Malignant tumors in the spinal extradural space. Results of surgical treatment. Ann Surg 1960:157:227-231. Ahmet Çolak, MD Tip Fakiiltcsi Caddesi 36/5 Abidinpasa Ankara (Turkey)
averted a neurosurgical emergency in this case. The au thors are to be congratulated on an excellent outcome in a difficult situation. David M. Klein. MD References 1 Baten M. Vannucci RC: Intraspinal metastatic disease in childhood cancer. J Pediatr 1977:90:207-212. 2 Lewis DW. Packer RJ. Raney B. Rak IW. Bclasco J. Lange B: Inci dence. presentation, and outcome of spinal cord disease in children with systemic cancer. Pediatrics 1986:78:438-443. 3 Raffel C. Ncave VCD. Lavine S. McComb JG: Treatment of spinal cord compression by epidural malignancy in childhood. Neurosur gery 1991;28:349-352.
Downloaded by: Stockholm University Library 130.237.165.40 - 1/20/2019 8:34:45 AM
7 Flameni F. Nlhoul-Feleke C, Lemcrle J: Optimal treatment of clini cal stage I yolk sac tumor of the testis in children. J Pediatr Surg 1986:21:108-111. 8 Allen CJ: Management of metastatic epidural disease in children. J Pediatr 1984;104:241-242. 9 Nelson K, Sten/.el P: Intrapcricardial yolk sac tumor in an infant girl. Cancer 1987:60:1567-1569.
