Panhypopituitarism caused by a suprasellar germinoma: A case report

BACKGROUND Suprasellar germinomas are rare intracranial tumors frequently associated with permanent endocrine disorders.We present the clinical picture,treatment,and complications of suprasellar germinoma at pediatric age which,besides being lifethreatening,has lifelong endocrinological consequences.CASE SUMMARY A 12-year-old female patient was presented having had intensive headaches for three weeks and visual disturbances for six months.An ophthalmological examination revealed bilateral papilledema and a marked loss of vision.Emergency brain magnetic resonance imaging(MRI)showed a suprasellar tumor,involving the infundibulum and the optic chiasm,extending to the third ventricle.Laboratory tests confirmed decreased levels of thyroxine,cortisol,gonadotropins,and insulin-like growth factor 1.Maximal tumor reduction was performed,and immunohistopathology established the diagnosis of suprasellar germinoma.MRI of the spine and cerebrospinal fluid cytology confirmed the localized disease.Adjuvant chemotherapy and radiotherapy were performed according to the SIOP CNS GCT II protocol.A post-treatment MRI showed no residual tumor,but pituitary function had not recovered.Three and a half years after the end of the treatment,the patient is in a complete remission,requiring hormonal replacement therapy,continuous education,and psychological support.CONCLUSION This complex case highlights the importance of timely diagnosis,a multidisciplinary approach,and close follow-up in children with suprasellar germinomas.

The MRI manifestations of intramedullary germinoma of the spinal cord:a case report and review of the literature

Primary germinoma in the spinal cord is very rare. Preoperative diagnosis is important because germinomas are sensitive to radiation therapy and chemotherapy so that treatment trends and prognosis differ from other intramedullary spinal cord tumors. Preoperative radiologic diagnosis is very difficult because of this rarity. In this case a 22-year-old woman with a 4-month history of gradual numbness and weakness of both lower extremities was found to have a solid tumor in the thoracic cord between the T9 and T11 vertebral bodies. No other neoplastic lesion was found inside or outside the central nervous system. The patient underwent surgery, and the intrarnedullary lesion was almost totally resected. Serum HCG was elevated postoperatively without pregnancy. The pathological diagnosis was conclusively that of a germinoma. The previous 23 cases of primary spinal cord germinoma were reviewed for comparison.

Clinical analysis of intracranial germinoma's craniospinal irradiation using helical tomotherapy

Objective： To evaluate the short-term clinical outcomes of intracranial germinoma patients treated with craniospinal irradiation （CSI） using helical tomotherapy （HT） system in our center. Methods： Twenty-three patients who were treated with CSI in our center from January 2008 to July 2012 were collected, with an average age of 20. M1 of the patients＇ CSI used the HT system. The total doses were 27-36 Gy/15-20 F （1.5-2 Gy per fraction）, and total local doses were 46-60 Gy/30-50 F （5 fractions per week）. M1 female patients for CSI were treated with left-right parallel-opposed field irradiation to protect their ovarian functions. Median follow-up time was 30.9 months （range, 5-67 months）. The SPSS19.0 software was used, and the overall survival （OS） was calculated using the Kaplan-Meier method. Results： Among 17 patients with assessable tumors, 9 cases （52.9%） were CR, 7 cases （41.2%） were PR, and 1 case （5.9%） was SD. Hematological toxicity was the severest side-effect occurred in the procedure of CSI. The level 1-4 acute leukopenia were 8.7%, 30.4%, 34.8% and 21.7% and the level 1-4 acute thrombopenia were 8.7%, 30.4%, 21.7% and 8.7%, respectively. Conclusions： For primary intracranial germinomas, HT can be used to implement CSI for simplifying radiotherapy procedures, improving radiotherapy accuracy, enhancing protection of peripheral organs at risk （ORA） and guaranteeing therapeutic effects. With the acceptable acute and long-term toxicity, CSI using HT in intracranial germinoma patients can be a safe and alternative mode.

Germinomas of the basal ganglia and thalamus: Four case reports

BACKGROUND The early diagnosis of basal ganglia and thalamus germinomas is often difficult due to the absence of elevated tumor markers,and atypical clinical symptoms and neuroimaging features.CASE SUMMARY Four male children aged 8 to 15 years were diagnosed with germinomas in the basal ganglia and thalamus by stereotactic biopsy from 2017 to 2019.All patients developed hemiplegia except patient 4 who also had cognitive decline,speech disturbance,nocturnal enuresis,polydipsia,polyuria,precocious puberty and abnormalities of thermoregulation.All four cases were alpha-fetoprotein and beta-human chorionic gonadotrophin(p-HCG)negative except patient 3 who had slightly elevated p-HCG in cerebrospinal fluid(CSF).No malignant cells were detected in the patients'CSF.Brain magnetic resonance imaging findings were diverse in these patients with the exception of the unique and common characteristics of ipsilateral hemisphere atrophy,especially in the cerebral peduncle.All patients were diagnosed with germinomas of the basal ganglia and thalamus by stereotactic brain biopsy.CONCLUSION Stereotactic brain biopsy is necessary to confirm the diagnosis of ectopic germinomas.Serial neuroimaging studies can not only differentiate disease but also determine the biopsy site.

Outcome of Vision Impairment and Diabetes Insipidus in Suprasellar Region Germinoma

Aim: Many cases of suprasellar region germinoma occurs in diabetes insipidus (DI), but a patient initially may come to the hospital for the chief complaint of visual impairment. The aim of this study is to determine the etiology of initial symptom presentation and the outcomes of visual impairment and DI in suprasellar region germinoma. Methods: We investigated eleven cases of single lesion suprasellar germinomas that were diagnosed and treated in our hospital. For each, a magnetic resonance imaging (MRI) was performed. Results: At the hospital visit, decreased visual acuity was found in 5/11 cases, while DI was found in all cases. The decreased visual acuity was improved in 4/5 cases by treatment, but DI improved in only 2/11 cases. In 10 cases, DI occurred earlier than visual impairment. As the initial symptom, visual impairment occurred earlier than DI in only one case and did not improve by treatment. In this case, a pituitary stalk of the normal thickness could be identified by MRI, and the optic nerve was swollen. In ten cases except this case, no significant enlargement of optic nerve was detected, and a swollen pituitary stalk was confirmed. Conclusions: In suprasellar germinomas, it is rare, but the optic nerve can significantly swell at onset, while the pituitary stalk may be intact. In such cases, visual impairment occurs earlier than DI, and visual impairment may not be improved.

Diabetes insipidus with impaired vision caused by germinoma and perioptic meningeal seeding:A case report

BACKGROUND Germinoma is a type of germ cell tumor that most frequently arises in the midline axis of the brain.Impaired vision is a clinical manifestation of germinnoma.Although rare,intracranial germinoma seeding to the perioptic arachnoid space is one cause of visual acuity decrease.CASE SUMMARY An 11yearold girl who presented with polyuria and polydipsia and subsequently developed diminution of vision.Imaging showed bilateral heterogeneous enhancement of the optic nerve sheaths and atrophy of the optic nerve,and transsphenoidal biopsy revealed a germinoma.The patient experienced poor visual recovery following chemotherapy and radiotherapy.Germinomas are rare and they are mostly identified in children and adolescents.The manifestations include diabetes insipidus,pituitary dysfunction,visual complaints,etc.The mechanisms that lead to visual loss include intracranial hypertension,compression of optic chiasma,and tumor invasion.A literature review was performed to summarize the cases with a tumor infiltrating the optic nerve.Most of the reported patients were adolescents and presented with anterior pituitary hormone deficiency.Enhancement of optic nerve sheaths and optic disc pallor could be identified in most of the cases.The purpose of this report is to provide awareness that in cases where a germinoma is associated with visual loss,though rare,perioptic meningeal seeding should be taken into consideration.CONCLUSION The case report suggests that children with diabetes insipidus need a complete differential diagnosis.

Delayed intra-tumoural haemorrhage in pineal germinoma: Case report and review

Intraparenchymal haemorrhage in a pineal germinoma is a very rare, though clinically significant event. We report the first case of a significantly delayed intraparenchymal haemorrhage in a pineal germinoma, 14 days after endoscopic third ventriculostomy (ETV), causing precipitous patient deterioration. We discuss potential contributing pathophysiological factors, and seek to illustrate that knowledge of its occurrence, and associated morbidity, is clinically significant in managing pineal germinoma patients with acute deterioration post obstructive hydrocephalus CSF diversion.

Interstitial brachytherapy of intracranial germinomas

Objective: To observe the process of tumor response to interstitial brachytherapy of intracranial germinomas during and immediately after the therapy. Methods: Fractionated brachytherapy using 192 Ir was used in 13 patients with intracranial germinomas. The average age at diagnosis was 14.3 years (range 5-27 years). The frequency of fractionation had been increased stepwise from 3 fractionations within 5 d in the past to 26-30 fractionations in 15-20 d at present. Results: Of 9 pineal tumors, the average reduction of tumor volume was 73.2% at a cumulated radiation dose of 20 Gy and 85.3% at 30 Gy, respectively. In 4 cases of basal ganglia and thalamus areas tumors, the reduction of tumor volume was from an average 56.1% at a cumulated radiation dose of 30 Gy to 75.2% at the end of therapy. That intratumoral hemorrhage was absorbed quickly accompanying tumor regression was demonstrated in all but one patient; 6 patients died; 7 patients were still in follow-up without any evidence of tumor recurrence. Conclusion: Interstitial brachytherapy could be given as the first optional therapy for intracranial germinomas. The histological diagnosis of germinomas can be verified by stereotactic biopsy simultaneously. Germinoma is one of the sensitive tumors to interstitial brachytherapy. The radiation injury to surrounding brain tissue could be reduced by using a conformal dynamic brachytherapy.

Role of MR imaging in the diagnosis of intracranial germinoma

Objective: To investigate the role of MRI in the diagnosis of intracranial germinoma. Methods: MRI features of 19 cases of intracranial germinoma confirmed by operations and pathological findings were analyzed retrospectively. Results: Germinomas were found in the sellar region in 10 patients (including 5 males and 5 females), in the pineal region in 6 and in the thalamus and basal ganglia in 3, the 9 patients in the latter 2 groups all being males. The characteristic MRI findings of intracranial germinomas were as follows: (1) Lesions were isointense or slightly hypointense on T1WI while isointense or slightly hyperintense on T2WI. The germinomas in the sellar region and pineal region showed no edema, but lesions in the thalamus basal ganglia showed mild to moderate edema and space-occupying effects. (2) Homogeneous or inhomogeneous Gd-DTPA enhancement were seen in most of the tumors. Conclusion: Multiaxial imaging and Gd-DTPA enhancement in MRI are helpful in the diagnosis and differentiation of intracranial germinomas on the basis of the patient's gender, the location of the tumor and its imaging characteristics.

弥漫播散性室管膜下生殖细胞瘤1例

患者男,29岁,视物成双伴视力下降半年,头痛伴双眼胀痛1月余,头痛加重2周,1周前非喷射性呕吐1次,无发热;既往体健。专科检查:双眼直接及间接对光反射迟钝,上视不能,左眼外展不及边,双眼视乳头水肿。实验室检查:血清人绒毛膜促性腺激素0.55 mIU/ml。腰椎穿刺提示脑脊液压力>300 mmH_(2)O。

Ocular manifestations of intracranial germinomas： three cases report and literature review

Intracranial germinomas are malignant neoplasms of gonadal origin, which have some features in terms of age, sex, and clinical manifestations. They mainly occur in children and adolescents. Patients with intracranial germinomas mainly manifest with hypothalamic pituitary axis dysfunction and/or compression syndromes. Visual disturbance is one of the most Significant clinical presentations, which is mainly caused by tumor cell infiltration into the optic pathway. In this article, we present three cases of patient with intracranial germinoma to analyze the ocular manifestations. All the three patients presented with endocrine symptoms in the early stage and with visual disturbances （including decreased visual acuity and visual field defects） later. In general, germinoma is diagnosed by its characteristic radiological appearance, supported by tumor markers and/or stereotactic biopsy. However, decisive diagnoses were established when ocular manifestations were presented. A suspicion for germinoma should be considered, when young patients manifest visual disturbances accompanied by endocrine symptoms.

How to interpret low human chorionic gonadotropin levels in cerebrospinal fluid of patients with intracranial germinoma？

Human chorionic gonadotropin （hCG） level in cerebrospinal fluid （CSF hCG） is very useful for thediagnosis of intracranial germinoma. We have previously reported on two patients with germinoma located in the basal ganglia. Their CSF hCG levels were extremely low upon presentation.1 However, significantly increased CSF hCG levels were detected when these two patients had a relapse. The experience acquired in these two cases is helpful to appropriately interpret the CSF hCG levels that are used to detect intracranial germinoma, evaluate the effectiveness of treatment and monitor recurrence.

Emergency irradiation with 3.4 Gy/2f in sellar/suprasellar germinoma patients with rapid visual acuity decline

Background: Rapid visual acuity (VA) decline was a common complaint in patients with sellar/suprasellar germinoma. In our hospital, 3.4 Gy/2f of emergency irradiation was applied to save patient VA and enable subsequent chemoradiotherapy. This study aimed to investigate the efficacy of emergency irradiation with 3.4 Gy/2f in patients with sellar/suprasellar germinoma who had rapid VA decline. Methods: From January 2014 to December 2017, 33 patients with sellar/suprasellar germinoma who complained of VA decline within 3 months received 3.4 Gy/2f of emergency irradiation in Beijing Tiantan Hospital. The best-corrected VA (BCVA) and mean deviation (MD) were measured. Correlations between visual function change and clinical factors, including age at diagnosis, duration of VA decline, extent of tumor regression, serum level of tumor markers, were analyzed. Results: Among 33 patients with sellar/suprasellar germinoma, the median diameter and volume of sellar/suprasellar lesions were 32 mm (range: 5–55 mm) and 12.9 cm3 (range 0.6–58.5 cm3), respectively. Data on pre- and post-emergency-irradiation BCVA were obtained in 32 patients. For the right eyes, BCVA was improved in 23 patients (71.9%), unchanged in 7 (21.9%), and worsened in 2 (6.2%);and for the left eyes, these numbers were 27 (84.4%), 4 (12.5%), and 1 (3.1%), respectively. In terms of the logarithm of the minimum angle of resolution (logarithm of the minimum angle of resolution = Log (1/BCVA) score, the improvement was significant in both eyes (P < 0.001). In terms of MD, six patients had paired data and the improvement was marginal in the right eyes (P = 0.068) and significant in the left eyes (P = 0.043). However, no clinical factor was found to have correlation with visual function improvement. Conclusion: In sellar/suprasellar germinoma patients with VA decline, 3.4 Gy/2f of emergency irradiation was effective in improving visual function.

Emergency irradiation of 3.4Gy/2f in pineal gland germinoma patients with symptomatic hydrocephalus

Surgical interventions including ventriculostomy and ventriculo-peritoneal shunt were usually administrated in pineal germ cell tumor patients with symptomatic hydrocephalus. Considering higher sensitivity of germinoma to anti-tumor therapy, we explored emergency irradiation as non-invasive measure in this situation. Methods: Data of 35 germinoma patients with symptomatic hydrocephalus who received emergency irradiation of 3. 4 Gy/2f were studied retrospectively. The maximum width of frontal horn and the minimum width of trunk of corpus cal osum (TCC) were measured to evaluate hydrocephalus changing. Besides, mean deviation (MD) of Humphrey perimetry was employed to evaluate visual field defect. Correlations between hydrocephalus changing and clinical factors, including age, percentage of tumor regression, radiographic re-evaluation interval, and serum beta-human chorionic gonadotropin (β-HCG) level, were analyzed. Results: The median maximum diameter and volume of pineal lesions was 27 mm (range 10–55 mm) and 6.5cm3 (range 0.4–74.1 cm3), respectively. At median 8 days after irradiation, the median percentage of tumor remission was 55% (range 10–100%). The median maximum width of FN and the median minimum width of TCC were 11.6 mm and 39.0 mm, and 8.0 mm and 31.4 mm, before and after irradiation, respectively. The improvement of both parameters reached significant level (p < 0.001). However, none clinical factor was found to have correlation with their improvement. In 14 patients with paired data of pre- and post-irradiation MD, its change did not reach the significant level for both eyes. Al patients successful y received subsequent chemoradiotherapy without surgical intervention. Conclusions: Emergency irradiation of 3.4 Gy/2f was an effective non-invasive measure to relief hydrocephalus in pineal germinoma patients.

TREATMENT OF INTRACRANIAL GERMINOMA

In 31 patients with intracranial germinoma who received radiotherapy, 15 were operated on, and 13 were treated with CSF shunt (7) and external ventricular drainage (6). After radiotherapy, clinical symptoms were improved in 93.5% of the patients. Follow-up of 16 patients for 2 to 14 years showed better results. We consider that initial treatment with radiotherapy may be appropriate for germinomas because of its high radiosensitivity. If hydrocephalus developed, external ventricular drainage combined with radiotherapy was performed, but shunting operation might be avoided because of potential peritoneal seedings of germinomas. In addition to chemotherapy, re-irradiation is an effective method for recurrent germinoma.

原发性颈胸髓内生殖细胞瘤1例

患者女,47岁,颈后及背部疼痛伴四肢麻木、无力1月余;既往体健。查体:颈椎及上段胸椎压痛、叩击痛阳性,四肢皮肤感觉减退,病理征未引出。颈胸椎CT/MRI:C7、T2椎体水平椎管内分别见10 mm×16 mm×20 mm、9 mm×11 mm×13 mm稍高欠均匀密度团块影,不规则欠均匀T1等信号(图1A),T2等或稍高信号(图1B),病变段脊髓增粗,其上下段脊髓水肿呈片状稍低T1、高T2信号。

卵巢原始生殖细胞分化及其衍生肿瘤中Oct4、CD34、p63的表达

目的研究Oct4、CD34、p63在原始生殖细胞衍生肿瘤的表达及其与肿瘤分化的关系。方法应用免疫组化SP法分别检测34例卵巢生殖细胞肿瘤及10例正常卵巢组织中Oct4、CD34、p63的表达。结果Oct4表达于无性细胞瘤和胚胎性癌,而在卵巢生殖细胞肿瘤的其他类型及正常卵巢组织中不表达(P<0·01)。CD34主要表达于卵黄囊瘤/内胚窦瘤,阳性率为81·3%,并且在卵黄囊瘤/内胚窦瘤的不同组织学形态间表达的差异有显著性(P<0·01)。p63主要表达于未成熟畸胎瘤,在卵巢生殖细胞肿瘤的其他类型及正常卵巢组织中不表达(P<0·01)。结论卵巢生殖细胞肿瘤中无性细胞瘤和胚胎性癌分化水平最接近于生殖母细胞至胚泡阶段。卵黄囊瘤CD34的分化表型与其组织学形态结构相关。Oct4、p63可分别作为进一步鉴别无性细胞瘤和未成熟畸胎瘤的标记物。

颅内生殖细胞瘤诊断性放疗剂量的初步探讨

目的探索颅内生殖细胞瘤诊断性放疗最低有效剂量,减少患者的无谓照射。方法回顾性分析了经放疗证实的生殖细胞瘤患者75例共104个病灶(其中单发病灶者51例,2个病灶者19例,3个病灶者5例),观察应用10Gy和20Gy的照射剂量时,病灶反应数目的变化。结果2种实验性放射疗法剂量对肿瘤病灶数目的影响未见显著性差异。结论在颅内生殖细胞瘤的诊断性放射疗法中,10Gy可作为排除部分非生殖细胞瘤的初步剂量。