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...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.展开更多
BACKGROUND Intracranial tuberculoma is a rare and serious type of tuberculosis,which mostly occurs in the frontoparietal and cerebellar hemispheres,with predominance in the gray-white matter junction area,while tuberc...BACKGROUND Intracranial tuberculoma is a rare and serious type of tuberculosis,which mostly occurs in the frontoparietal and cerebellar hemispheres,with predominance in the gray-white matter junction area,while tuberculomas only in the cistern are extremely rare with only a few reported cases in the literature.We describe a unique case of isolated tuberculoma in the suprasellar cistern,with only right ocular motility disorder and upper eyelid ptosis.CASE SUMMARY A 5-year-old boy without any medical history presented with right ocular motility disorder and upper eyelid ptosis one month ago.He had no history of fever,headache,vomiting,convulsions,or limb weakness.Neurological examination showed right third cranial nerve palsy with restrictions of eye movements and ptosis,pupil dilation and negative light reflex.Imaging suggested a spaceoccupying lesion in the suprasellar cistern with calcification and ring-enhancement.Moreover,no Mycobacterium tuberculosis was found in cerebrospinal fluid by polymerase chain reaction(PCR).The lesion was initially diagnosed as a tumor,while postoperative pathology combined with PCR indicated tuberculoma.The patient continued postoperative anti-tuberculosis treatment.At present,the patient's condition is stable and the symptoms are partially relieved compared with those before surgery.CONCLUSION This case confirmed that isolated intracranial tuberculoma can occur in the suprasellar cistern.Therefore,for space-occupying lesions in the suprasellar cistern,tuberculoma should be included in the differential diagnosis even if there is no history or indication of tuberculosis infection.展开更多
Objective: To evaluate the feasibility and therapeutic effectiveness of the transsphenoidal microsurgical removal of pituitary adenomas with suprasellar extensions. MethodS: We reviewed the diagnosticmodes, surgical t...Objective: To evaluate the feasibility and therapeutic effectiveness of the transsphenoidal microsurgical removal of pituitary adenomas with suprasellar extensions. MethodS: We reviewed the diagnosticmodes, surgical technique, and outcomes of 152 patients suffering from pituitary adenomas with suprasellarextensions who were treated by transsphenoidal microsurgery in our department. Diagnosis was confirmed byCT or MRI scanning. All tumours with diameter >10 mm were characterized by suprasellar extensions. Op.erations were performed via either sublabio-septo-sphenoidal approach or naso-vestibulo-sphenoidal approachunder microscope. A subarachnoid catheter was preoperatively inserted in the lumbar cistern, through whichsaline was slowly injected during operation to increase the intracranial pressure (ICP) so as to deliver thesuprasellar tumour into the operative field to aid the removal. Result: Of the 152 cases, the gross total removal of adenoma in 106 cases (69. 7% ) and subtotal removal in 37 cases (24. 4% ) were achieved, and partial removal was carried out in the remaining 9 cases (5. 9 % ) of fibrous or dumbbell-shaped adenomas. Therewere no deaths after operation in this group. Follow-up review (median 3. 5 years) in 137 patients revealedgood recovery in 97 patients (70. 8% ), and late recurrence in 40 patients (29. 2% ) who need reoperativemanagement, drug therapy, radiotherapy, or radiosurgery used either alone or combined. Conclusion: Microsurgical technique via transsphenoidal approach is a safe and effective way to remove the pitoitary adenomas with suprasellar extensions but not for fibrous or dumbbell-shaped ones.展开更多
Background:Meningiomas are rather uncommon tumors in the pediatric population,differing significantly from those found in adults by their atypical location,higher rate of more malignant types,consequently higher risk ...Background:Meningiomas are rather uncommon tumors in the pediatric population,differing significantly from those found in adults by their atypical location,higher rate of more malignant types,consequently higher risk of recurrence and a less favorable outcome.Even in children,suprasellar meningiomas without dural matrix are rare findings mimicking more common suprasellar lesions.Case presentation:Here we describe a case of a 12-year-old girl who presented with a rapidly progressing chiasmal syndrome and was diagnosed by MRI with an unusual suprasellar tumor that could not fit the diagnoses expected in a case of a parasellar mass in a child,similar to a craniopharyngioma or optic pathway glioma.After multiple clinical investigations,the tumor etiology was still unclear,so the preferred option of treatment was surgical resection.An endoscope-assisted gross total resection through a supraorbital keyhole approach was performed uneventfully,with total vision recovery in a short time.Benign meningiomas located in the skull base without dural attachment appear to be rare,even in pediatric patients.Conclusion:Differential diagnoses of suprasellar and para sellar tumor lesions in pediatric patients can be confusing.There are peculiar features of pediatric tumor diseases that should be considered while working out the management strategy.The main principle of meningioma treatment is the highest possible extent of resection minimally affecting the quality of life.展开更多
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 subseque...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.展开更多
文摘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.
文摘BACKGROUND Intracranial tuberculoma is a rare and serious type of tuberculosis,which mostly occurs in the frontoparietal and cerebellar hemispheres,with predominance in the gray-white matter junction area,while tuberculomas only in the cistern are extremely rare with only a few reported cases in the literature.We describe a unique case of isolated tuberculoma in the suprasellar cistern,with only right ocular motility disorder and upper eyelid ptosis.CASE SUMMARY A 5-year-old boy without any medical history presented with right ocular motility disorder and upper eyelid ptosis one month ago.He had no history of fever,headache,vomiting,convulsions,or limb weakness.Neurological examination showed right third cranial nerve palsy with restrictions of eye movements and ptosis,pupil dilation and negative light reflex.Imaging suggested a spaceoccupying lesion in the suprasellar cistern with calcification and ring-enhancement.Moreover,no Mycobacterium tuberculosis was found in cerebrospinal fluid by polymerase chain reaction(PCR).The lesion was initially diagnosed as a tumor,while postoperative pathology combined with PCR indicated tuberculoma.The patient continued postoperative anti-tuberculosis treatment.At present,the patient's condition is stable and the symptoms are partially relieved compared with those before surgery.CONCLUSION This case confirmed that isolated intracranial tuberculoma can occur in the suprasellar cistern.Therefore,for space-occupying lesions in the suprasellar cistern,tuberculoma should be included in the differential diagnosis even if there is no history or indication of tuberculosis infection.
文摘Objective: To evaluate the feasibility and therapeutic effectiveness of the transsphenoidal microsurgical removal of pituitary adenomas with suprasellar extensions. MethodS: We reviewed the diagnosticmodes, surgical technique, and outcomes of 152 patients suffering from pituitary adenomas with suprasellarextensions who were treated by transsphenoidal microsurgery in our department. Diagnosis was confirmed byCT or MRI scanning. All tumours with diameter >10 mm were characterized by suprasellar extensions. Op.erations were performed via either sublabio-septo-sphenoidal approach or naso-vestibulo-sphenoidal approachunder microscope. A subarachnoid catheter was preoperatively inserted in the lumbar cistern, through whichsaline was slowly injected during operation to increase the intracranial pressure (ICP) so as to deliver thesuprasellar tumour into the operative field to aid the removal. Result: Of the 152 cases, the gross total removal of adenoma in 106 cases (69. 7% ) and subtotal removal in 37 cases (24. 4% ) were achieved, and partial removal was carried out in the remaining 9 cases (5. 9 % ) of fibrous or dumbbell-shaped adenomas. Therewere no deaths after operation in this group. Follow-up review (median 3. 5 years) in 137 patients revealedgood recovery in 97 patients (70. 8% ), and late recurrence in 40 patients (29. 2% ) who need reoperativemanagement, drug therapy, radiotherapy, or radiosurgery used either alone or combined. Conclusion: Microsurgical technique via transsphenoidal approach is a safe and effective way to remove the pitoitary adenomas with suprasellar extensions but not for fibrous or dumbbell-shaped ones.
文摘Background:Meningiomas are rather uncommon tumors in the pediatric population,differing significantly from those found in adults by their atypical location,higher rate of more malignant types,consequently higher risk of recurrence and a less favorable outcome.Even in children,suprasellar meningiomas without dural matrix are rare findings mimicking more common suprasellar lesions.Case presentation:Here we describe a case of a 12-year-old girl who presented with a rapidly progressing chiasmal syndrome and was diagnosed by MRI with an unusual suprasellar tumor that could not fit the diagnoses expected in a case of a parasellar mass in a child,similar to a craniopharyngioma or optic pathway glioma.After multiple clinical investigations,the tumor etiology was still unclear,so the preferred option of treatment was surgical resection.An endoscope-assisted gross total resection through a supraorbital keyhole approach was performed uneventfully,with total vision recovery in a short time.Benign meningiomas located in the skull base without dural attachment appear to be rare,even in pediatric patients.Conclusion:Differential diagnoses of suprasellar and para sellar tumor lesions in pediatric patients can be confusing.There are peculiar features of pediatric tumor diseases that should be considered while working out the management strategy.The main principle of meningioma treatment is the highest possible extent of resection minimally affecting the quality of life.
文摘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.
