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What Is the Cause of Severe Headache in this 28-year-old Asian Man? A Rare Case of Intracranial Mixed Germ Cell Tumor.

Authors

Maveric Abella, MS3 (1); Christina Park, MD (2); Kuo-Chiang Lian, MD (3) 1 John A. Burns School of Medicine, University of Hawai鈥檌, Honolulu, HI 2 University of Hawai鈥檌 Internal Medicine Residency Program, Honolulu, HI 3 The Queen's Medical Center, Honolulu, HI

Introduction

Germ cell tumors (GCTs) typically arise in the gonads and have a predilection for brain metastasis in about 15% of cases. Extragonadal GCTs typically arise in the anterior mediastinum, retroperitoneum, pineal and suprasellar regions in adults, and sacrococcygeal and intracranial regions in children. This case features a rare primary extragonadal GCT found in the corpus callosum of an adult male.

Case Presentation

A 28-year-old Filipino male with a history of thalassemia minor presented with left-sided weakness and generalized headache described as cramping, associated with nausea and vomiting for two days. The patient reported no trauma or fall, nor any focal neurologic symptoms. Initial exam was notable for decreased strength in left upper and lower extremities, grade 2/5 and 4/5, respectively. MRI brain demonstrated a 4.5 cm corpus callosum hemorrhagic mass extending into the right lateral ventricle with obstructive hydrocephalus, suggestive of central neurocytoma. Neurosurgery was consulted for tumor resection. While awaiting surgery, the patient had a partial seizure, which was resolved with levetiracetam. Subsequently, he underwent craniotomy, ventriculocisternostomy of the third ventricle, and external ventricular drain placement. Postoperative MRI revealed residual tumor, edema of the resection site and an acute lacunar infarction of the right centrum semiovale. Pathology of resected tissue revealed a malignant mixed germ cell tumor with immature teratoma (75%), germinoma (20%), and yolk sac tumor (5%), with positive OCT 3/4, Glypican-3, AFP, and hCG markers, suggestive of nongerminomatous germ cell tumor (NGGCT). At this time, a scrotum exam was performed and ultrasound was obtained, which was negative for any mass. CT of chest, abdomen and pelvis, and spine MRI did not show metastasis. On postoperative day one, the patient had a left facial droop and worsened muscle strength, 0/5 in left upper and 1/5 in left lower extremities. This was presumed to be from post-surgical edema and was treated with steroid taper. Within two weeks of steroids and physical therapy, the patient's strength improved to 4+/5 in all extremities. The patient was discharged to a rehabilitation facility with plans to initiate chemoradiation therapy.

Discussion

Primary intracranial GCTs are very rare. Most common sites are pineal gland and suprasellar regions. Peak incidence is during teenage years, and with male predominance. In the United States, there is a higher incidence among Asians and Pacific Islanders. Common symptoms include headache, vomiting, and lethargy;endocrinopathies may be present when involving hypothalamic and pituitary glands. GCTs can be categorized histopathologically into germinoma and NGGCT consisting of yolk sac tumor, choriocarcinoma and teratomas. The standard of care for localized germinoma is radiation as it is exquisitely sensitive to radiation with 90% progression-free survival. In contrast, NGGCTs have poor prognosis and require multimodality therapy consisting of neoadjuvant chemotherapy and craniospinal irradiation. There is no definitive data to suggest total resection of NGGCT at time of diagnosis improves survival. However, if there is residual cancer post-treatment, a 鈥渟econd-look鈥 surgery is recommended.

References

  1. Bokemeyer C, Nichols CR, Droz JP, et al. Extragonadal germ cell tumors of the mediastinum and retroperitoneum: results from an international analysis. J Clin Oncol. 2002;20:1864-1873. [PMID: 11919246] doi:10.1200/JCO.2002.07.062
  2. Schmoll HJ. Extragonadal germ cell tumors. Ann Oncol. 2002;13 Suppl 4:265-272. [PMID: 12401700] doi:10.1093/annonc/mdf669
  3. Raina V, Singh SP, Kamble N, et al. Brain metastasis as the site of relapse in germ cell tumor of testis. Cancer. 1993;72:2182-2185. [PMID: 8397061] doi:10.1002/1097-0142(19931001)72:7<2182:aid-cncr2820720719>3.0.co;2-p

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