122
Surg Neural 1991:35:122-6
Intramedulhry Shirou
Matsuoka,
Spinal Cord Germinoma: Case Report M.D.,
and Akira Tanimura,
Masaharu
Itoh, M.D.,
Shinonome,
M.D.,
M.D.
Departments of Neurosurgery
and Pathology, Shimonoseki Municipal Central Hospital, Shimonoseki, Yamaguchi, Japan
Matsuoka S, Iroh M, Shinonome T, Tanimura A. Intramedullary spinal cord germinoma: case report. Surg Neural 1991;35:122-6. A case of intramedullary spinal cord germinoma within the corms medullaris, with lumbago and pain in the lower extremities, is presented. The intramedullary spinal cord germinoma was determined by a biopsy specimen. After local irradiation of 50 Gy, the tumor markedly decreased in size and clinical symptoms disappeared. KEY WORDS:
Toshiaki
Intramedullary tumor; Spinal cord; Germinoma
Extragonadal germinoma has been known to occur in the midline structures of the body, such as the anterior
mediastinum, retroperitoneum, and brain. In the central nervous system (CNS), the pineal and suprasellar regions are representative sites for germinoma. However, the case of intramedullary spinal cord germinoma has not been reported, except for a case reported by Hisa et al [7] in 1985.
Extraocular movements and visual acuity were intact. Plain films of the lumbosacral spine demonstrated flattening and some erosion of the pedicles at L-l and L-2. Myelography with Isovist (iotrolan; Schering AG, Federal Republic of Germany) introduced through lumbar puncture demonstrated an intradural mass in the region of the conus medullaris with an almost complete block at L-2 (Figure 1). Magnetic resonance imaging (MRI) (Tl-weighted image) showed an isointense intramedullary mass with small cysts between the level of the Th-12 and L-2 vertebrae. After intravenous administration of gadolinium-diethylenetriaminepentaacetic acid, the mass was clearly enhanced (Figure 2). Computed tomography (CT) scan of the head was normal. Blood analysis, urinalysis, and chemical findings were normal. A lumbar puncture yielded a xanthochromic cerebrospinal fluid (CSF); the cell count was 446/3 (mostly lymphocytes), protein 381 mg/dL, and glucose 53 mg/dL. Cytologic study of CSF was negative.
Operation
Case Report A 31-year-old pregnant housewife noted the onset of paresthesia of the posterior calves a few weeks after falling on her buttocks. Five months later, on September 8, 1988, she delivered her third child. In November 1988, she developed lumbago and pain in the lower extremities when in the supine position, mainly at night, and was relieved from pain in the sitting or standing position. On December 31, 1988, she complained of severe lumbago and was admitted to our department as an emergency. She had no history of diabetes insipidus. On admission, the spine was tender to percussion over the lumbosacral region. Strength was normal in all muscle groups. Sensation was intact. There were no sphincter disturbances. Deep tendon reflexes were normal.
Address reprint requests to;Shirou Matsuoka,M.D., Department of Neurosurgery, ShimonosekiMunicipalCentral Hospital,Kouyouchou l-13-1, Shimonoseki 750, Yamaguchi, Japan. Received May 14, 1990; accepted July 5, 1990. 0 1991 by Else&r
Science Publishmg
Co., Inc.
On January 19, 1989, laminectomy was done between the Th-1 1 and L-2 vertebrae. A symmetrically widened intramedullary mass filled the intradural space at Th-12 to L-2, ending at the conus. The filum terminale was slightly enlarged and the cauda equina emerged from the distorted conus (Figure 3). There was no spinal dissemination. Through a midline myelotomy in the conus, pieces of the tumor were taken; the tumor was not resectable. Decompressive duroplasty with lyophilized dura was performed.
Pathological Findings The tumor tissue was composed of clear polygonal cells. The nuclei, which were round or oval, had fine prominent nucleoli (Figure 4). Mitotic figures were seen (2-3/ high-power field). Intracytoplasmic periodic acid-schiffpositive globules were occasionally observed. There were no multinucleated giant cells {so-called syncytiotrophoblastic giant cells (STGCs)] in the specimen. The 0090-3019191153.50
Figure 1. lsovist lumbar myelogram demonstrates a complete block at L-2 on prone position. The injection was made at L4-5. -Left, posteroanterior
view;
right. lateral view.
Figure 2. (A) Preoperative MRI (SE 500/30 Th-12
ms) in sagittal view showing an irointense intramedullary and L-2 vertebrae. (B) Mass is slightly enhanced with GLDTPA (0.2 mLlkg iv).
magi with small cysts between the level of the
124
Surg Neurol 1991;35:122-6
Matsuoka
3. Operative photograph shou\c the hypoza.wuhzv rntramedullq tumor in the CDVUJ nedulhis. (Note the thickenedfihm terminale emev,qi:ing fromthe raudal portion of the tumor.)
et al
Figure
stroma
was infiltrated
by lymphocytes.
diagnosed as germinoma Postoperatively, pain
without in the
The
tumor
STGCs. lower extremities
was im-
proved slightly. Localized radiation therapy (50 Gy over 1 monthj was started. Pain improved markedly and she was able to sleep in a supine position at night. resonance imaging showed a marked decrease the tumor, even after 30 Gy irradiation (Figure at the end of L-2 vertebrae Serum tumor noembryonic beta subunit, dehydrogenase
Hzstological feature of the tumor (bematoxylin and eosin Jtain x 200): two distinct cell type.r are present: large po/ygonaf r-ellswith clear cytoplasm, round OYoval nuclei, and lymphocytes.
Figure 4.
Magnetic in size of 5 A), and
radiation, the spinal cord at the Th-I2 to became almost normal in size (Figure 5 B). markers, such as alpha-fetoprotein, carciantigen, human chorionic gonadotropinplacental alkaline phosphatase, and lactic (LDH) were within normal limits. Pitu-
itary function and gynecological studies revealed no abnormal findings. On March 28, 1989, she was discharged on foot without any complaints. In April 1990, 13 months after radiation therapy, the patient was very well without neurological deficits. Magnetic resonance imaging showed no recurrence of the spinal cord tumor.
Discussion Germ-cell tumors frequently tures of the body [8,11,12]. a rare location. Nine cases
occur in the midline strucHowever, the spinal cord is of intramedullary teratoma
were reported by Garrison and Kasdon [5] in 1980. Hisa et al [7) reported the first case of intramedullary spinal cord germinoma, which produced precocious puberty in a 5.3-year-old boy. Germinornas are the most common type of intracranial germ-cell tumor and are situated almost exclusively in the midline, such as in the pineal or suprasellar region. These tumors, like their gonadal counterparts, have been known to metastasize, usually to the spinal cord or throughout the ventricular system Cl]. Therefore, whether the spinal cord tumor was primary or metastatic is the question. Neurological findings and CT scans of the head did not suggest intracranial germinoma. Diabetes insipidus and
hypothalamopituitary
disturbances
were
not
pres-
Intramedullary
Surg Neurol 1991;35:122-6
Spinal Cord Germinoma
Figure 5. (A) MRI (SE 500/.?0 ~JI in sagittaivieu:after30 Gy radzation therapy shows a marked decrease in size of the tumor. (B) MRI (SE 500130 msi in sagittal z&u’ after JO GJ radiation
therapy.
ent. The hypothesis of spinal metastasis from an ovarian dysgerminoma can be excluded by her normal delivery of three children, ultrasonographic examination of both ovaries, and normal serum LDH value as tumor marker for an ovarian dysgerminoma [3,4,6]. Operative findings suggested an intramedullary tumor in the conus but no seedings on the surface of the cord. Pathologically, the tumor was that of the so-called two-cell pattern germinoma, but STGCs were not found in the specimen, which are said to produce human chorionic gonadotropin as tumor marker {7,9,13}. We diagnosed the tumor as a primary spinal cord germinoma. In 1777, Einhorn and Donohue [2) reported a complete remission rate of 74% in patients with germ-cell tumors of the testis who were treated with cisplatin, vinblastine, and bleomycin chemotherapy. Systemic chemotherapy may be beneficial in certain CNS germ-cell neoplasms refractory to radiotherapy or that recurred after radiotherapy [lo]. In our case, radiation therapy (50 Gy over 1 month) was effective clinically and radiologically, as
125
in intracranial germinomas. As the tumor was highly radiosensitive, chemotherapy was not administered. Follow up CT scan and MRI will be scheduled to demonstrate whether an occult pineal or suprasellar tumor will develop or a spinal tumor will recur.
References Donat JF, Okazaki H, Gomez MR, Reagan TJ, Baker HL, Laws EK. Pineal tumors: a 53.year experience. Arch Neural 1978;35:736-40. Einhorn LH, Donohue J. Cis-diaminedichloroplatinum, vinblastine, and bleomycin combination chemotherapy in disseminated testicular cancer. Ann Intern Med 1977;87:293-8. Friedman M, White RG, Nissenbaum MM, Browde S. Serum lactic dehydrogenase-a possible tumor marker for an ovarian dysgerminoma: a literature review and report of a case. Obstet Gynecol Surv 1984;39:247-51. Fujii S, Konishi I, Suzuki A, Okamura H, Okazaki T, Mori T. Analysis of serum lactic dehydrogenase levels and its isoenzymes in ovarian dysgerminoma. Gynecol Oncol 1985;22:65-72. Garrison JE, Kasdon report and reviewofthe
DL. Incramedullary spinal reraroma: case literature. Neurosurgery 1980;7:509-12.
Gill PG. Abbott R, Jones AM, Thomas DW. The management of metastaric germ cell rumours and the clinical utility of lactate dehydrogenase estimations. Aust NZ J Surg 1985;55:133-40.
126
Matsuoka
Neurol 1991;35:122-6
Surg
Hisa S, Morinaga S, Kobayashi Y, Ojima M, Chikaoka N. Intramedullary spinal cord germinoma producing precocious puberty in a boy. Cancer 1985;55:2845-9. Jellinger K. Primary intracranial thol 1973;25:291-306,
germ cell tumors.
H, Sasano HCG and
Acta Neuropa-
Kapp DS, Kohorn EI, Merion MJ, Li Volsi VA. Pure dysgerminoma of the ovary with elevated serum human chorionic gonadotropin: diagnostic and therapeutic considerations, Gynecol Oncol 1985;20:234-44.
et al
10. Kirshner JJ, Ginsberg SJ, Fitzpatrick AV, Comis RL. Treatment of a primary intracranial germ cell tumor with systemic chemotherapy. Med Pediatr Oncol 1981;9:361-5. 11. Leblanc G, Francoeur cranial dysgerminomas.
J, Copty M, Contreras C, Gagne F. IntraCan J Nemo1 Sci 1976;3:199-203.
12. Waga S, HandaH, YamashiraJ. Intracranialgerminoma: and results. Surg Neural 1979;11:167-72.
treatment
with syn13. Zaloudek CJ, Tavassoli FA, Norris HJ. Dysgermmoma cytiotrophoblastic giant cells. Am J Surg Pathol 1981;5:361-7.
Surg Neural 1991:35:122-6
Intramedulhry Shirou
Matsuoka,
Spinal Cord Germinoma: Case Report M.D.,
and Akira Tanimura,
Masaharu
Itoh, M.D.,
Shinonome,
M.D.,
M.D.
Departments of Neurosurgery
and Pathology, Shimonoseki Municipal Central Hospital, Shimonoseki, Yamaguchi, Japan
Matsuoka S, Iroh M, Shinonome T, Tanimura A. Intramedullary spinal cord germinoma: case report. Surg Neural 1991;35:122-6. A case of intramedullary spinal cord germinoma within the corms medullaris, with lumbago and pain in the lower extremities, is presented. The intramedullary spinal cord germinoma was determined by a biopsy specimen. After local irradiation of 50 Gy, the tumor markedly decreased in size and clinical symptoms disappeared. KEY WORDS:
Toshiaki
Intramedullary tumor; Spinal cord; Germinoma
Extragonadal germinoma has been known to occur in the midline structures of the body, such as the anterior
mediastinum, retroperitoneum, and brain. In the central nervous system (CNS), the pineal and suprasellar regions are representative sites for germinoma. However, the case of intramedullary spinal cord germinoma has not been reported, except for a case reported by Hisa et al [7] in 1985.
Extraocular movements and visual acuity were intact. Plain films of the lumbosacral spine demonstrated flattening and some erosion of the pedicles at L-l and L-2. Myelography with Isovist (iotrolan; Schering AG, Federal Republic of Germany) introduced through lumbar puncture demonstrated an intradural mass in the region of the conus medullaris with an almost complete block at L-2 (Figure 1). Magnetic resonance imaging (MRI) (Tl-weighted image) showed an isointense intramedullary mass with small cysts between the level of the Th-12 and L-2 vertebrae. After intravenous administration of gadolinium-diethylenetriaminepentaacetic acid, the mass was clearly enhanced (Figure 2). Computed tomography (CT) scan of the head was normal. Blood analysis, urinalysis, and chemical findings were normal. A lumbar puncture yielded a xanthochromic cerebrospinal fluid (CSF); the cell count was 446/3 (mostly lymphocytes), protein 381 mg/dL, and glucose 53 mg/dL. Cytologic study of CSF was negative.
Operation
Case Report A 31-year-old pregnant housewife noted the onset of paresthesia of the posterior calves a few weeks after falling on her buttocks. Five months later, on September 8, 1988, she delivered her third child. In November 1988, she developed lumbago and pain in the lower extremities when in the supine position, mainly at night, and was relieved from pain in the sitting or standing position. On December 31, 1988, she complained of severe lumbago and was admitted to our department as an emergency. She had no history of diabetes insipidus. On admission, the spine was tender to percussion over the lumbosacral region. Strength was normal in all muscle groups. Sensation was intact. There were no sphincter disturbances. Deep tendon reflexes were normal.
Address reprint requests to;Shirou Matsuoka,M.D., Department of Neurosurgery, ShimonosekiMunicipalCentral Hospital,Kouyouchou l-13-1, Shimonoseki 750, Yamaguchi, Japan. Received May 14, 1990; accepted July 5, 1990. 0 1991 by Else&r
Science Publishmg
Co., Inc.
On January 19, 1989, laminectomy was done between the Th-1 1 and L-2 vertebrae. A symmetrically widened intramedullary mass filled the intradural space at Th-12 to L-2, ending at the conus. The filum terminale was slightly enlarged and the cauda equina emerged from the distorted conus (Figure 3). There was no spinal dissemination. Through a midline myelotomy in the conus, pieces of the tumor were taken; the tumor was not resectable. Decompressive duroplasty with lyophilized dura was performed.
Pathological Findings The tumor tissue was composed of clear polygonal cells. The nuclei, which were round or oval, had fine prominent nucleoli (Figure 4). Mitotic figures were seen (2-3/ high-power field). Intracytoplasmic periodic acid-schiffpositive globules were occasionally observed. There were no multinucleated giant cells {so-called syncytiotrophoblastic giant cells (STGCs)] in the specimen. The 0090-3019191153.50
Figure 1. lsovist lumbar myelogram demonstrates a complete block at L-2 on prone position. The injection was made at L4-5. -Left, posteroanterior
view;
right. lateral view.
Figure 2. (A) Preoperative MRI (SE 500/30 Th-12
ms) in sagittal view showing an irointense intramedullary and L-2 vertebrae. (B) Mass is slightly enhanced with GLDTPA (0.2 mLlkg iv).
magi with small cysts between the level of the
124
Surg Neurol 1991;35:122-6
Matsuoka
3. Operative photograph shou\c the hypoza.wuhzv rntramedullq tumor in the CDVUJ nedulhis. (Note the thickenedfihm terminale emev,qi:ing fromthe raudal portion of the tumor.)
et al
Figure
stroma
was infiltrated
by lymphocytes.
diagnosed as germinoma Postoperatively, pain
without in the
The
tumor
STGCs. lower extremities
was im-
proved slightly. Localized radiation therapy (50 Gy over 1 monthj was started. Pain improved markedly and she was able to sleep in a supine position at night. resonance imaging showed a marked decrease the tumor, even after 30 Gy irradiation (Figure at the end of L-2 vertebrae Serum tumor noembryonic beta subunit, dehydrogenase
Hzstological feature of the tumor (bematoxylin and eosin Jtain x 200): two distinct cell type.r are present: large po/ygonaf r-ellswith clear cytoplasm, round OYoval nuclei, and lymphocytes.
Figure 4.
Magnetic in size of 5 A), and
radiation, the spinal cord at the Th-I2 to became almost normal in size (Figure 5 B). markers, such as alpha-fetoprotein, carciantigen, human chorionic gonadotropinplacental alkaline phosphatase, and lactic (LDH) were within normal limits. Pitu-
itary function and gynecological studies revealed no abnormal findings. On March 28, 1989, she was discharged on foot without any complaints. In April 1990, 13 months after radiation therapy, the patient was very well without neurological deficits. Magnetic resonance imaging showed no recurrence of the spinal cord tumor.
Discussion Germ-cell tumors frequently tures of the body [8,11,12]. a rare location. Nine cases
occur in the midline strucHowever, the spinal cord is of intramedullary teratoma
were reported by Garrison and Kasdon [5] in 1980. Hisa et al [7) reported the first case of intramedullary spinal cord germinoma, which produced precocious puberty in a 5.3-year-old boy. Germinornas are the most common type of intracranial germ-cell tumor and are situated almost exclusively in the midline, such as in the pineal or suprasellar region. These tumors, like their gonadal counterparts, have been known to metastasize, usually to the spinal cord or throughout the ventricular system Cl]. Therefore, whether the spinal cord tumor was primary or metastatic is the question. Neurological findings and CT scans of the head did not suggest intracranial germinoma. Diabetes insipidus and
hypothalamopituitary
disturbances
were
not
pres-
Intramedullary
Surg Neurol 1991;35:122-6
Spinal Cord Germinoma
Figure 5. (A) MRI (SE 500/.?0 ~JI in sagittaivieu:after30 Gy radzation therapy shows a marked decrease in size of the tumor. (B) MRI (SE 500130 msi in sagittal z&u’ after JO GJ radiation
therapy.
ent. The hypothesis of spinal metastasis from an ovarian dysgerminoma can be excluded by her normal delivery of three children, ultrasonographic examination of both ovaries, and normal serum LDH value as tumor marker for an ovarian dysgerminoma [3,4,6]. Operative findings suggested an intramedullary tumor in the conus but no seedings on the surface of the cord. Pathologically, the tumor was that of the so-called two-cell pattern germinoma, but STGCs were not found in the specimen, which are said to produce human chorionic gonadotropin as tumor marker {7,9,13}. We diagnosed the tumor as a primary spinal cord germinoma. In 1777, Einhorn and Donohue [2) reported a complete remission rate of 74% in patients with germ-cell tumors of the testis who were treated with cisplatin, vinblastine, and bleomycin chemotherapy. Systemic chemotherapy may be beneficial in certain CNS germ-cell neoplasms refractory to radiotherapy or that recurred after radiotherapy [lo]. In our case, radiation therapy (50 Gy over 1 month) was effective clinically and radiologically, as
125
in intracranial germinomas. As the tumor was highly radiosensitive, chemotherapy was not administered. Follow up CT scan and MRI will be scheduled to demonstrate whether an occult pineal or suprasellar tumor will develop or a spinal tumor will recur.
References Donat JF, Okazaki H, Gomez MR, Reagan TJ, Baker HL, Laws EK. Pineal tumors: a 53.year experience. Arch Neural 1978;35:736-40. Einhorn LH, Donohue J. Cis-diaminedichloroplatinum, vinblastine, and bleomycin combination chemotherapy in disseminated testicular cancer. Ann Intern Med 1977;87:293-8. Friedman M, White RG, Nissenbaum MM, Browde S. Serum lactic dehydrogenase-a possible tumor marker for an ovarian dysgerminoma: a literature review and report of a case. Obstet Gynecol Surv 1984;39:247-51. Fujii S, Konishi I, Suzuki A, Okamura H, Okazaki T, Mori T. Analysis of serum lactic dehydrogenase levels and its isoenzymes in ovarian dysgerminoma. Gynecol Oncol 1985;22:65-72. Garrison JE, Kasdon report and reviewofthe
DL. Incramedullary spinal reraroma: case literature. Neurosurgery 1980;7:509-12.
Gill PG. Abbott R, Jones AM, Thomas DW. The management of metastaric germ cell rumours and the clinical utility of lactate dehydrogenase estimations. Aust NZ J Surg 1985;55:133-40.
126
Matsuoka
Neurol 1991;35:122-6
Surg
Hisa S, Morinaga S, Kobayashi Y, Ojima M, Chikaoka N. Intramedullary spinal cord germinoma producing precocious puberty in a boy. Cancer 1985;55:2845-9. Jellinger K. Primary intracranial thol 1973;25:291-306,
germ cell tumors.
H, Sasano HCG and
Acta Neuropa-
Kapp DS, Kohorn EI, Merion MJ, Li Volsi VA. Pure dysgerminoma of the ovary with elevated serum human chorionic gonadotropin: diagnostic and therapeutic considerations, Gynecol Oncol 1985;20:234-44.
et al
10. Kirshner JJ, Ginsberg SJ, Fitzpatrick AV, Comis RL. Treatment of a primary intracranial germ cell tumor with systemic chemotherapy. Med Pediatr Oncol 1981;9:361-5. 11. Leblanc G, Francoeur cranial dysgerminomas.
J, Copty M, Contreras C, Gagne F. IntraCan J Nemo1 Sci 1976;3:199-203.
12. Waga S, HandaH, YamashiraJ. Intracranialgerminoma: and results. Surg Neural 1979;11:167-72.
treatment
with syn13. Zaloudek CJ, Tavassoli FA, Norris HJ. Dysgermmoma cytiotrophoblastic giant cells. Am J Surg Pathol 1981;5:361-7.
