Brain Tumor Res Treat.  2024 Jul;12(3):200-203. 10.14791/btrt.2024.0025.

A Rare Case of Intracranial Growing Teratoma Syndrome in a Young Adult

• Gwak YG
• Yang SH
• Yoo YJ
• Kim HH
• Lee Y
• Kim YI

• KMID: 2558439
• DOI: http://doi.org/10.14791/btrt.2024.0025

Abstract

Intracranial growing teratoma syndrome (iGTS) is a rare phenomenon in patients with non-germinomatous germ cell tumor (NGGCT) after chemotherapy or radiotherapy. It manifests as paradoxical growth of teratomatous components, with multiple cystic lesions on cranial imaging despite normalized tumor markers. This paper presents a 22-year-old male with iGTS, diagnosed one month after chemotherapy against NGGCT. Initially diagnosed with presumptive pineal NGGCT causing obstructive hydrocephalus, the patient underwent endoscopic third ventriculostomy and extraventricular drainage with tumor biopsy followed by two chemotherapy cycles. Despite normalization of tumor markers, follow-up MRI showed increased tumor size with honeycomb-like cystic patterns. The patient underwent suboccipital craniotomy for tumor removal via combined telovelar and infratentorial supracerebellar approaches. The final pathology confirmed mature teratoma. However, postoperative bleeding and left thalamic infarction occurred, resulting in severe neurological deficits. Despite challenges, the patient eventually regained the ability to follow simple commands. To understand iGTS pathophysiology, several hypotheses, including the differentiation of immature components and the uninhibited growth of mature components induced by chemotherapy or radiotherapy, were explored. Surgical intervention remains as an ideal treatment, while clinical trials investigate chemotherapy options. Frequent imaging followups are crucial for early detection in iGTS for NGGCT patients.

Keyword

Teratoma; Intracranial neoplasms; Germ cell tumor; Chemotherapy; Magnetic resonance imaging

Figure

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  Fig. 1 Gadolinium-enhanced magnetic resonance (MR) brain images of intracranial growing teratoma syndrome (iGTS) patients. A and B: Initial MR images of germ cell tumor show heterogeneously enhanced mass obstructing the fourth ventricle. C and D: MR images after two cycles of chemotherapy show increased size of mass with honeycomb-like cystic patterns corresponding to iGTS.

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  Fig. 2 Hematoxylin and eosin staining of the resected tumor. A: Tissue from ETV with biopsy shows only squamous epithelium containing keratin debris. B and C: Tissue from subtotal tumor resection shows typical mature cystic teratoma histopathology. It consists of different types of mature tissue components such as chondroid tissue (*) with columnar epithelium (**), cartilaginous tissue (black arrow), and respiratory epithelium (white arrow).

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