Report of A Case of Papillary Thyroid Carcinoma Following Intracranial Germinoma

Introduction The occurrence of second primary thyroid malignancy should be noticed in survivors of brain germinoma treated by irradiation. Case Report A patient with a history of intracranial germinoma who underwent chemoradiotherapy was referred to our endocrine clinic due to impotency and infertility. The patient received replacement therapy due to hypopituitarism. He returned to us 11 years after brain radiation with enlargement of the thyroid gland. Thyroid ultrasonography showed enlargement with a dominant 62*37 mm solid hypoechoic nodule in the right thyroid lobe. Several lymph nodes at both sides of the neck were seen. Total thyroidectomy revealed PTC with regional metastasis. Conclusion Endocrine disruption must be considered in adult cancer survivors and the importance of long-term follow-up should be emphasized in these patients.

NCOG-35. THE LATE INTRACRANIAL ADVERSE EVENTS OCCUR MORE FREQUENTLY IN INTRACRANIAL GERMINOMAS TREATED WITH HIGH DOSE RADIOTHERAPY

OBJECTIVE The standard treatment for intracranial germinoma has been radiotherapy covering the whole ventricle together with chemotherapy. Radiotherapy is important, but it is a cause of the late brain damages. Therefore, the recent clinical trials have been planned to evaluate the reduced radiation dose. The aim of this study was to evaluate the intracranial adverse events in the patients with intracranial germinomas treated in our hospital. PATIENTS AND METHODS 65 patients were diagnosed as intracranial germinoma. Patients with hCG > 100 IU/l and/or AFP > 10 ng/ml were excluded. Patients, who were diagnosed as germinoma by imaging without histology, were included. RESULTS Follow-up time was from 2 to 467 months (median 136 months). Until 2005, 37 patients were treated with radiotherapy >30 Gy alone or with chemotherapy. After then, 23 patients received whole-ventricle radiotherapy 24-30 Gy with chemotherapy. 2 patients were treated with chemotherapy alone, 3 were unknown. 10-year PFS was 82.05% in radiotherapy >30 Gy alone, 86.36% in radiotherapy >30 Gy with chemotherapy and 100% in radiotherapy 24-30 Gy with chemotherapy. The intracranial adverse events after the initial treatment were identified, such as pituitary dysfunction: 6 (9.2%), hearing disturbance: 2 (3.1%), neurocognitive dysfunction 6 (9.2%), microbleeds 10 (15.4%), cavernous angioma 6 (9.2%), brain tumor 1 (1.5%), cerebral artery stenosis 1 (1.5%). The frequency of late adverse brain events is higher in radiotherapy >30 Gy with/without chemotherapy than 24-30 Gy (total events, 25 vs. 9, P< 0.03). CONCLUSION Patients with intracranial germinoma obtain long-term survival but suffer from the late intracranial adverse events. The late intracranial adverse events occur more frequently in intracranial germinomas treated with radiotherapy >30 Gy than 24-30 Gy. Long-term follow-up is important to promptly identify and deal with the late brain damages.

P06.04 Tertiary center experience in the treatment of intracranial germinoma

BACKGROUND Intracranial germinoma is a rare malignant neoplasm, accounting for approximately two-thirds of central nervous system germ cell tumors, which occurs in the suprasellar region, pineal gland, or basal nuclei in children and young adolescents. Due to the rare occurrence, there is relatively high variability in treatment modalities. This article aims to assess the symptoms, and treatment outcomes of radiotherapy and chemotherapy for intracranial germinoma, based on tertiary center experience. METHODS The study includes retrospectively gathered data from 35 consecutive patients treated for intracranial germinoma between 1999–2020 at a single institution. The statistical analysis used basic statistical tools and the Kaplan-Meier method. RESULTS The median age at diagnosis was 17 years (IQR 14–22), 85.7% of the patients were male. The majority of the patients had histopathological confirmation of the diagnosis (88.5%), 76% of which were non-secreting germinomas. The primary tumor was most frequently localized in the area of the pineal gland (34.3%), followed by bifocal (25.7%), suprasellar (17.1%), and basal germinoma (8.6%). The remaining patients had disseminated disease (14.3%). The most common symptoms at the onset of the disease were headache (54.3%), visual impairment (45.7%), vomiting (25.7%), polydipsia (22.9%), weight loss (17.1%), or minor neurological deficits (11.4%). As much as 54.3% of the patients presented with neuroendocrine disorders at diagnosis, and 42.9% of the patients presented radiological symptoms of hydrocephalus. Each patient received radiotherapy, which was preceded by chemotherapy in 77.1%, and surgery in 31.4% of the cases. The majority of the patients received radiotherapy to the ventricular system (51.4%), craniospinal irradiation (34.3%), or whole-brain radiotherapy (8.6%) followed by a focal boost in 88%. The remaining patient was treated with focal radiotherapy only. The 5- and 10-year overall survival rate was found to be 97% and 87% respectively, including two patients who died despite disease control (pulmonary and ventricular embolism). The 5-year progression-free survival was 83%. CONCLUSIONS Despite variability in treatment methods, there is a relatively high cure and survival rate in patients with intracranial germinoma. However, the rare occurrence of the disease seems to negatively impact the diagnostic process. The tumor is often diagnosed at an advanced stage, presenting significant, often irreversible symptoms.

Right Basal Ganglia Germinoma: Case Report and Literature Review

Background: Among intracranial germinomas, germinoma of basal ganglia has been rarely reported. We discuss a new case of basal ganglia germinoma(BGG) and perform the literature review over the last two decades, with the aim of emphasizing the diagnosis and treatment in early-stage BGG.Case presentation: A seven years old Chinese boy presented with 4 months history of left limb movement disorder and oblique right mouth corner. The human chorionic gonadotropin(HCG) level in cerebrospinal fluid(CSF) was slightly increased. Magnetic resonance imaging(MRI) showed ipsilateral brain and brainstem atrophy. Susceptibility weighted imaging(SWI) revealed obvious hypointensity in right globus pallidus. Pathological diagnosis on biopsy was confirmed with germinoma. The patient had a favorable relief of symptoms after chemoradiotherapy.Conclusion: Intracranial germinoma, a potentially curable tumor, the early diagnosis is essential for the prognosis. An elevated HCG level of CSF or serum can be used as a reference indicator. MRI, especially SWI, plays an important role in early diagnosis. Patients should be treated with standardized chemoradiotherapy early rather than surgery.

Necessity for craniospinal irradiation of germinoma with positive cytology without spinal lesion on MR imaging - A controversy

Background Cerebrospinal fluid (CSF) cytology and spinal MR imaging are routinely performed for staging before treatment of intracranial germinoma. However, the interpretation of the results of CSF cytology poses two unresolved clinical questions: 1) Does positive CSF cytology correlate with the presence of spinal lesion before treatment?; and 2) Is craniospinal irradiation (CSI) necessary for patients with positive CSF cytology in the absence of spinal lesion? Methods Multicenter retrospective analyses were performed based on a questionnaire on clinical features, spinal MR imaging finding, results of CSF cytology, treatments, and outcomes which was sent to 86 neurosurgical and 35 pediatrics departments in Japan. Pretreatment frequencies of spinal lesion on MR imaging were compared between the patients with positive and negative cytology. Progression-free survival (PFS) rates were compared between patients with positive CSF cytology without spinal lesion on MR imaging treated with CSI and with whole brain or whole ventricular irradiation (non-CSI). Results A total of 92 germinoma patients from 45 institutes were evaluated by both CSF cytology and spinal MR images, but 26 patients were excluded because of tumor markers, the timing of CSF sampling or incomplete estimation of spinal lesion. Of the remaining 66 germinoma patients, spinal lesions were equally identified in patients with negative CSF cytology and positive cytology (4.9% and 8.0%, respectively). 11 patients treated with non-CSI had excellent PFS comparable to 11 patients treated with CSI. Conclusion CSI is unnecessary for germinoma patients with positive CSF cytology without spinal lesions on MR imaging.

Primary intracranial germ cell tumour originating from right brachium Pontis with hypertrophic Olivary degeneration: a case report

Background Primary right brachium pontis germinoma with hypertrophic olivary degeneration (HOD) is extremely rare. A preoperative diagnosis is challenging due to the absence of characterized clinical and neuroimaging features, and biopsy should be considered. Case presentation A 20-year-old male patient presented with a case of primary intracranial germinoma originating from right brachium pontis with HOD manifesting as ocular myoclonus, nystagmus in both eyes, ataxic gait and incoordination of the limbs. Magnetic resonance imaging (MRI) revealed an irregular patchy lesion with hyperintensity on T2-weighted images (T2WI) and T2 fluid-attenuated inversion recovery (FLAIR) without enhancement by gadolinium (Gd). Furthermore, a focal hyperintense nodule on T2WI in the left inferior olive nucleus (ION) of the medulla oblongata was considered hypertrophic olivary degeneration (HOD) based on the patient’s symptoms and neuroimaging findings. Due to suspected demyelinating disease and low-grade glioma (LGG), a biopsy was planned. The pathological diagnosis was germinoma. Subsequently, he received chemoradiation therapy, resulting in the improvement of neurological deficits and the disappearance of the lesion on MRI. Conclusion A case of “Primary right brachium pontis germinoma with HOD” is reported for the first time. A preoperative diagnosis is challenging due to the fact of absence of clinical signs and symptoms and neuroimaging characteristics. However, patients can have favourable prognoses with appropriate evaluation and treatment.

Bilateral Optic Neuropathy Mimicking Neuromyelitis Optica in the Setting of Intracranial Germinoma

Bilateral optic neuropathy is uncommon in adults with a prevalence varying from 19% -50% of all optic neuropathies [1]. The most common etiology is Neuromyelitis Optica (NMO) or Devic’s disease. Other less frequent etiologies include myelin-oligodendrocyte-glycoprotein (MOG) antibody, syphilis, meningioma, post-vaccinal, post-infectious (chicken pox, human herpes virus 6), acute disseminated encephalomyelitis and idiopathic. Sequential optic neuropathy is seen in multiple sclerosis, but bilateral simultaneous optic neuropathy is rare [2]. When optic neuropathy involves posterior nerve segments, long segments and the optic chiasm, NMO should be suspected. If there is involvement of the hypothalamus, basal meninges, other cranial neuropathies, sarcoidosis must be considered [3]. Primary intracranial germinoma is a rare condition and should be included in the differential diagnosis, especially if there are atypical features and inadequate response to standard treatment [4].