Germinoma staging

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2]

Overview

There is no established system for the staging of germ cell tumors, and most investigators utilize the TM system employed for staging medulloblastomas. Predominantly because of their midline location, staging for the size of the tumor at the time of diagnosis or after surgery (T-stage evaluation), has not been routinely employed in germ cell tumors.

Staging

The diagnostic work-up for CNS germ cell tumors GCTs should include MRI of the spine and brain, measurement of the tumor markers AFP and β -hCG in both serum and CSF, and histopathological confirmation by biopsy. Appropriate staging is important because patients with metastatic disease may receive higher total doses of radiation and extended radiation fields. Staging evaluation of central nervous system GCTs includes the following:[1][2][3][4]

  • MRI of the spine and brain are essential for diagnosis, MRI also assesses extent of intracranial disease and detect metastatic disease
  • Postoperative MRI of the brain is essential to assess residual tumor
  • CSF cytology is used to detect malignant cells
  • Measurement of serum and CSF tumor markers including β-hCG and AFP. Lumbar CSF should be obtained for the measurement of tumor markers (alpha-fetoprotein [AFP] and beta subunit human chorionic gonadotropin [beta-HCG]) and for cytopathologic review, when medically permissible.
  • Serum tumor markers are often obtained for AFP and beta-HCG; but, they do not serve as a substitute for CSF tumor markers
  • Evaluation of the disease outside the CNS is usually unnecessary

Germ cell tumors, specifically germinomas, can disseminate the neuraxis at the time of diagnosis or early in the course of illness. GCTs may be disseminated throughout the neuraxis at any disease stage or at the time of diagnosis. Several unusual patterns of spread may occur in germinomas, such as subependymal dissemination in the third or lateral ventricles and parenchymal infiltration. Rarely, extracranial spread to lung and bone has also been reported. Staging of patients with bifocal intracranial germinomas limited to the suprasellar and pineal region is controversial, as some classify these tumors as localized disease and others classify such presentations as disseminated disease. There is no specific staging system which has been universally accepted for germ cell tumors, and most investigators utilize the TM system employed for staging medulloblastomas. Predominantly because of their midline location, staging for the size of the tumor at the time of diagnosis or after surgery (T-stage evaluation), has not been routinely employed in germ cell tumors.[5][6][7][8]

M system for staging germ cell tumors is shown below in a tabular form:

M type of germ cell tumors Features
M0
  • M0 are those tumors without evidence of metastatic disease as determined by pre- or post diagnosis MRI of the entire brain and spine and cerebrospinal fluid (CSF) cytological examination. Patients with localized disease and negative CSF cytology are considered to be M0. M0 are considered to be metastatic negative.
M1
  • M1 are those patients with free-floating tumor cells, which is relatively common in patients with germ cell tumors and normal spinal and brain neuroimaging finding at the time of diagnosis. Patients with positive CSF cytology or patients with drop metastasis such as spinal or cranial subarachnoid metastases that arise from intracranial lesions are considered to be M+ or metastatic-positive
M2 or M3
  • M2 and M3 patients are those with lump disease in the spinal region or cranial subarachnoid space


References

  1. Kim A, Ji L, Balmaceda C, Diez B, Kellie SJ, Dunkel IJ; et al. (2008). "The prognostic value of tumor markers in newly diagnosed patients with primary central nervous system germ cell tumors". Pediatr Blood Cancer. 51 (6): 768–73. doi:10.1002/pbc.21741. PMID 18802946.
  2. Calaminus G, Bamberg M, Harms D, Jürgens H, Kortmann RD, Sörensen N; et al. (2005). "AFP/beta-HCG secreting CNS germ cell tumors: long-term outcome with respect to initial symptoms and primary tumor resection. Results of the cooperative trial MAKEI 89". Neuropediatrics. 36 (2): 71–7. doi:10.1055/s-2005-837582. PMID 15822019.
  3. Shinoda J, Sakai N, Yano H, Hattori T, Ohkuma A, Sakaguchi H (2004). "Prognostic factors and therapeutic problems of primary intracranial choriocarcinoma/germ-cell tumors with high levels of HCG". J Neurooncol. 66 (1–2): 225–40. PMID 15015791.
  4. Shibamoto Y, Oda Y, Yamashita J, Takahashi M, Kikuchi H, Abe M (1994). "The role of cerebrospinal fluid cytology in radiotherapy planning for intracranial germinoma". Int J Radiat Oncol Biol Phys. 29 (5): 1089–94. PMID 8083078.
  5. Weksberg DC, Shibamoto Y, Paulino AC (2012). "Bifocal intracranial germinoma: a retrospective analysis of treatment outcomes in 20 patients and review of the literature". Int J Radiat Oncol Biol Phys. 82 (4): 1341–51. doi:10.1016/j.ijrobp.2011.04.033. PMID 21669501.
  6. Gay JC, Janco RL, Lukens JN (1985). "Systemic metastases in primary intracranial germinoma. Case report and literature review". Cancer. 55 (11): 2688–90. PMID 3995478.
  7. Jennings MT, Gelman R, Hochberg F (1985). "Intracranial germ-cell tumors: natural history and pathogenesis". J Neurosurg. 63 (2): 155–67. doi:10.3171/jns.1985.63.2.0155. PMID 2991485.
  8. Packer, Roger J., Bruce H. Cohen, and Kathleen Cooney. "Intracranial germ cell tumors." The Oncologist 5.4 (2000): 312-320.


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