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© 2001 American Society for Clinical Oncology
Unusual Presentations of Germ Cell TumorsCase 2. Seminoma of the Conus MedullarisSydney Cancer Centre, Royal Prince Alfred Hospital, Sydney, Australia A 43-year-old white man presented with a 2-day history of perineal paraesthesia, urinary retention, impotence, and constipation on a background of a 2-month history of lumbar pain. His past history was noncontributory. On examination, the patient had proximal leg weakness (4/5), brisk reflexes, and normal plantar responses. Sensation was objectively intact. He had a distended bladder that could be percussed to the level of the umbilicus and lax anal tone. The remainder of his examination was unremarkable. Magnetic resonance imaging of the lumbar and sacral spine revealed an intradural extramedullary mass lesion at L1-L2 compressing the cauda equina ( Fig. 1A, T1-weighted image; Fig 1B, T2-weighed image). A T12-L3 laminectomy showed a well-encapsulated tumor arising from the conus medullaris, adherent to the roots of the cauda equina. The tumor resection involved dissection of the capsule from the nerve roots; however, full macroscopic clearance was achieved. The histology revealed sheets of neoplastic polygonal cells interspersed with lymphocytes consistent with pure seminoma ( Fig 2, magnification x 33). Postoperatively, the patient underwent magnetic resonance imaging of the brain which demonstrated two metastatic subependymal nodules in the lateral ventricles (Fig 3A and 3B). Serum beta-human chorionic gonadotropin and alpha-fetoprotein levels were normal, as were results of a scrotal ultrasound. The diagnosis of extragonadal seminoma arising from the conus was made.
Three cycles of postoperative bleomycin, etoposide, and cisplatin (BEP) were planned. After the first cycle, the patient was admitted with progressive dyspnea and pleuritic chest pain. He was hypoxic with a bibasal interstitial infiltrate. A presumptive diagnosis was made of bleomycin pneumonitis, and corticosteroid therapy was instituted. He recovered well and continued chemotherapy without bleomycin. Repeat imaging showed a partial response with significant decrease in the size of the subependymal nodules. The patient subsequently received craniospinal irradiation (36 Gy in 24 fractions) with a 14-Gy boost to both the L1-L2 area and the nodules in the lateral ventricles. At 8 months after treatment was completed, the patient was progression-free with a small enhancing subependymal area in the right lateral ventricle. He mobilized well but still had significant sphincter dysfunction, requiring self-catheterization twice daily. Germ cell tumors of the CNS are rare, accounting for less than 5% of all CNS tumors in the Western world. For unknown reasons, the rate is higher (approximately 10%) in Asia. They usually arise in the pineal or suprasellar regions; however, 5% are found in other midline structures, such as the third ventricle and thalamus1. Seminomatous germ cell tumor (germinoma) arising from the conus medullaris has been reported previously in only three patients (aged 16, 24, and 31 years).2-4 The current patient is unique in the literature, as subependymal metastases were demonstrated in addition to the conus disease. These were not biopsied, but their response to chemotherapy/radiotherapy would argue in favor of metastatic disease. In all previous reports, patients had disease localized to the conus. The conventional treatment for patients with CNS germinomas is radiotherapy; however, there have been a number of studies examining the role of chemotherapy.5,6 Germinomas are extremely radiosensitive, with 5-year survival rates of 80% to 91%.7-9 The major difficulty with irradiation is the long-term neuropsychologic sequelae in a young, curable patient group.10 Balmaceda et al5 treated 71 patients with CNS germ cell tumors with chemotherapy alone. Despite 78% of patients achieving a complete response, 50% relapsed with a high rate of radiotherapy salvage. A later study showed that primary chemotherapy did not affect the success of subsequent radiotherapy.11 In previous case reports, radiotherapy has been the sole treatment modality. However, this patient had metastatic disease, and as such, chemotherapy was considered to be an important part of treatment. REFERENCES 1. Raghavan D, Boyer MJ: Malignant extragonadal germ cell tumours in adults, in Horwich A (ed): Testicular Cancer: Investigation and Management. Chapman and Hall Medical, 1996, pp 345-369 2. Matsuoka S, Itoh M, Shinonome T, et al: Intramedullary spinal cord germinoma: Case report. Surg Neurol 35: 122-126, 1991[Medline] 3. Slagel DD, Goeken JA, Platz CA, et al: Primary germinoma of the spinal cord: A case report with 28-year follow-up an review of the literature. Acta Neuropathol Berl 90: 657-659, 1995[Medline] 4. Miyauchi A, Matsumoto K, Kohmura E, et al: Primary intramedullary spinal cord germinoma: Case report. J Neurosurg 84: 1061, 1996 5. Balmaceda C, Heller G, Resenblum M, et al: Chemotherapy without irradiation: A novel approach for newly diagnosed CNS germ cell tumours—Results of an international cooperative trial. J Clin Oncol 14: 2908-2915, 1996[Abstract]
6.
Buckner JC, Peethambaram BB, Smithson WA, et al: Phase II trial of primary chemotherapy followed by reduced-dose radiation for CNS germ cell tumours. J Clin Oncol 17: 933-940, 1999 7. Dearnaley DP, A’Hern RP, Whittaer S, et al: Pineal and CNS germ cell tumours: Royal Marsden Hospital experience 1962-1987. Int J Radiat Oncol Biol Phys 18: 773-781, 1990[Medline] 8. Wolden SL, Wara WM, Larson DA, et al: Radiation therapy for primary intracranial germ-cell tumours. Int J Radiat Oncol Biol Phys 32: 943-949, 1995[Medline] 9. Haddock MG, Schild SE, Scheithauer BW, et al: Radiation therapy for histologically confirmed primary central nervous system germinoma. Int J Radiat Oncol Biol Phys 38: 915-923, 1997[Medline]
10.
Silber JH, Radcliffe J, Peckham V, et al: Whole-brain irradiation and decline in intelligence: The influence of dose and age on IQ score. J Clin Oncol 10: 1390-1396, 1992
11.
Merchant TE, Davis BJ, Sheldon JM, et al: Radiation therapy for relapsed CNS germinoma after primary chemotherapy. J Clin Oncol 16: 204-209, 1998
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Copyright © 2001 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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