|Year : 2017 | Volume
| Issue : 1 | Page : 32-34
Primary conus medullaris germinoma: A 22-year follow-up
Chi-Wen Chang1, Jung-Shun Lee2, Hsing-Hong Chen3
1 Department of Surgery, Neurosurgical Division, Kuo General Hospital, Tainan, Taiwan
2 Department of Surgery, Neurosurgical Division, National Cheng-Kung University College of Medicine and Hospital, Tainan, Taiwan
3 Department of Neurosurgery, Taipei Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, New Taipei City, Taiwan
|Date of Web Publication||28-Feb-2017|
Department of Neurosurgery, Taipei Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, No. 289, Jianguo Road, Xindian, New Taipei City 23142
Source of Support: None, Conflict of Interest: None
Primary intramedullary spinal cord germinoma (PISCG) is very rare, and its preoperative diagnosis is therefore difficult. In addition, its treatment protocols and long-term outcomes are not yet established. Here, we report the case of a patient with a primary conus medullaris germinoma who initially presented with ejaculatory disturbance and coccygeal hypoesthesia. After the total excision of the germinoma and radiotherapy, no tumor recurrence was noted for 22 years. We have also reviewed the distribution of PISCG in spinal segments and its treatment modalities.
Keywords: Conus medullaris germinoma, primary intramedullary spinal cord germinoma, radiotherapy
|How to cite this article:|
Chang CW, Lee JS, Chen HH. Primary conus medullaris germinoma: A 22-year follow-up. Formos J Surg 2017;50:32-4
| Introduction|| |
Central nervous system germinomas are commonly located in the pineal region, suprasellar region, and basal ganglia. These germinomas are associated with a spinal metastasis rate of 10%–20%. Primary intramedullary spinal cord germinoma (PISCG) is very rare. Here, we report the case of a patient with a primary conus medullaris germinoma who was postoperatively followed for 22 years. In addition, we review literature on the epidemiology and treatment modalities of PISCG.
| Case Report|| |
A 25-year-old man initially presented with ejaculatory disturbance, which was followed by urinary hesitancy for approximately 8 months. Two months later, he started to experience constipation. Neurological examination demonstrated hypoesthesia in S3–5 area. Magnetic resonance imaging (MRI) revealed an intramedullary spinal tumor at levels L1 and L2 without intracranial lesions [Figure 1]a. We made a tentative diagnosis of a spinal ependymoma in the conus medullaris and filum terminale. Postoperatively, urinary function recovered within 1 month; however, the ejaculatory disturbance has sustained ever since.
|Figure 1: (a) Sagittal T1-weighted image (TR = 500 ms, TE = 15 ms) with gadolinium enhancement: A sausage-shaped tumor with three segments (T) was noted at levels L1 and L2. The tumor was not separable from the conus medullaris (C). (b) The tumor (white arrow) was elastic, reddish, sausage-shaped, and measured 4 cm × 0.8 cm × 0.8 cm in size and was located at the conus medullaris and cauda equina (black arrow). (c) After a postoperative follow-up period of 22 years, postgadolinium sagittal T1-weighted image (TR = 572 ms, TE = 15 ms) spine imaging did not reveal the presence of any tumor|
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The patient underwent L1–L3 laminectomy and total excision of the tumor in 1993. The tumor was elastic, reddish, sausage-shaped, and measured 4 cm × 0.8 cm × 0.8 cm extending from the conus medullaris to the filum terminale [Figure 1]b. The tumor was well defined except on the superior end, which had an ill-defined margin with the conus medullaris. The pathological diagnosis was germinoma [Figure 2]a and [Figure 2]b. The serum human chorionic gonadotropin (HCG) level after operation was within the normal range. Radiotherapy (30.6 Gy) was administered as adjuvant therapy. Postoperatively, the patient had occasional constipation and urinary retention but recovered gradually. During the 22-year follow-up, no clinical or radiological evidence of recurrence has been noted [Figure 1]c.
|Figure 2: (a) Nests of tumor cells (T) with abundant clear cytoplasm. The tumor is infiltrated by numerous lymphocytes (L) (low-power magnification). (b) The tumor cells have large nuclei (N) and prominent nucleoli (high-power magnification)|
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| Discussion|| |
PISCG was first described by Hisa et al. in 1985, and thereafter, only thirty cases have been reported [Table 1].,,,,,, Including the present case, the mean age at diagnosis was 25 years (range, 5–43 years). Male predominance in PISCG (1.58:1) is similar to that in intracranial germinoma. Most patients were of Japanese descent (19/31). The tumors were located at different levels in the spinal cord: cervical (4/31), thoracic (15/31), thoracolumbar (7/31), and lumbar (5/31). No characteristic findings were observed on neuroimaging, which can distinguish PISCG from other enhanced intramedullary spinal tumors. Surgical exploration or biopsy and excision remain the diagnostic modality of choice.
|Table 1: Summary of 31 cases of primary intramedullary spinal cord germinomas|
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Because of the rarity of PISCG, there is no consensus on its treatment protocol. Most of the treatments are derived from those for intracranial germinoma. Therefore, biopsy followed by radiotherapy with or without chemotherapy is the most common treatment protocol. Chen et al. postulated that a reduced dose of radiation is effective in treating intracranial germinoma, and chemotherapy should be used only for disseminated cases. The treatment modalities in the reported cases included total excision (5/30), partial excision (16/30), and biopsy (7/30). Two cases (2/30) without surgery were diagnosed by either an increase in the HCG level preoperatively or computed tomography and MRI findings and the response to radiotherapy. Adjuvant therapy protocols included radiotherapy (9/30), combined radiotherapy and chemotherapy (17/30), and chemotherapy alone (1/30). During the follow-up period from 3 months to 28 years, most of the treated patients with PISCG exhibited favorable outcomes. Only two cases had recurrence: one was treated using biopsy, radiotherapy, and chemotherapy; another was treated using partial excision., Additional data on long-term outcomes are required to establish definitive treatment protocols.
This study describes the first case of conus medullaris PISCG, which underwent gross total removal with a long-term follow-up period. Following excision and radiotherapy, the patient has been disease free for 22 years. Because germinomas are sensitive to radiotherapy, the role of total surgical excision should not be overemphasized. In general, surgical excision should not be an option for germinomas. Difficulties in preoperative diagnosis and the mass effect caused by a germinoma in a relatively small spinal canal prompt surgical excision of the tumor. However, the amount of tumor excision should prevent further damage to the neural tissue. In summary, the treatment of PISCG is biopsy and surgical excision, followed by radiotherapy. Adjuvant chemotherapy is controversial and can be adjusted according to the existence of a residual lesion. Annual follow-up MRI scans during the first 5 years is suggested.
We acknowledge Dr. Al-Rahim Tailor for the English revision.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]