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Frontiers in Oncology 2024Non-germinomatous germ cell tumors (NGGCT) accounts for one third of intracranial GCT. While the germinoma group have an excellent overall survival, the standard of...
Intracranial non-germinomatous germ cell tumors in children and adolescents: how can the experience from an uppermiddle-income country contribute to the worldwide effort to improve outcomes?
BACKGROUND
Non-germinomatous germ cell tumors (NGGCT) accounts for one third of intracranial GCT. While the germinoma group have an excellent overall survival, the standard of practice for children with NGGCT is still under evaluation.
AIMS
Describe the results of the of the Brazilian consortium protocol.
METHODS
Since 2013, 15 patients with a diagnosis of NGGCT by histopathology and/or serum/cerebrospinal fluid (CSF) tumor markers, βHCG >200mlU/ml and/or positive alpha-fetoprotein were treated with neoadjuvant chemotherapy with carboplatin, cyclophosphamide and etoposide followed by ventricular radiotherapy (RTV) of 18Gy with boost (32Gy) to the primary site. Metastatic patients underwent craniospinal irradiation (CSI) and "slow responders" to the four initial cycles of CT, to autologous stem cell transplantation (ASCT) followed by CSI.
RESULTS
Mean age, 13.1 years. Thirteen males. Primary sites: pineal (n=12), suprasellar (n=2) and bifocal (n=1). Four patients were metastatic at diagnosis. Eight patients had CSF and/or serum alpha-fetoprotein levels > 1,000ng/ml. Tumor responses after chemotherapy demonstrated complete in six cases and partial in seven, with "second-look" surgery being performed in five cases, and two patients presenting viable lesions being referred to ASCT. The main toxicity observed was hematological grades 3/4. Two patients with metastatic disease, one with Down Syndrome and AFP > 1,000ng/ml and the other with choriocarcinoma and pulmonary metastases, developed progressive disease resulting in death, as well as two other patients without evidence of disease, due to endocrinological disorders. Event-free and overall survival at 2 and 5 years were 80% and 72.7%, respectively, with a mean follow-up of 48 months (range, 7-107).
CONCLUSIONS
Despite the small number of patients, in our series, treatment with six cycles of chemotherapy and RTV with focal boost for localized disease (n=11) and ACST for identified slow responders (n=2) seem to be effective strategies contributing to the overall effort to improve outcomes of this group of patients.
PubMed: 38500657
DOI: 10.3389/fonc.2024.1308128 -
Cancer Reports (Hoboken, N.J.) Aug 2022Intracranial germ cell tumors (GCTs) comprise 3%-5% of pediatric primary central nervous system (CNS) tumors in Western countries. Though they are related in embryonic...
BACKGROUND
Intracranial germ cell tumors (GCTs) comprise 3%-5% of pediatric primary central nervous system (CNS) tumors in Western countries. Though they are related in embryonic origin to gonadal GCTs, which are considered highly treatable with cisplatin-based chemotherapy regimens, intracranial GCTs vary in malignant potential and sensitivity to radiation and chemotherapy, generally carrying a worse prognosis. Metastases of intracranial GCTs outside of the CNS are rare, indicate a poor prognosis, and their salvage treatment is not well established.
CASE
A 15-year-old boy presented with bifocal (suprasellar and pineal) intracranial nongerminomatous germ cell tumors of mixed origin. The tumors were treated to full response with a multimodal approach of neoadjuvant chemotherapy, surgical resection, and adjuvant craniospinal proton radiation. Nine months following treatment completion, the patient presented with an enlarged cervical lymph node determined on excisional biopsy to be a recurrence of pure germinoma from the primary tumors. Salvage treatment involved high-dose chemotherapy and autologous stem cell transplantation; however, the patient denied further treatment prior to planned focal radiotherapy. Thirty months post-treatment, the patient is well with no evidence of recurrence.
CONCLUSION
This case demonstrated the successful salvage treatment of an extraneural recurrence of an intracranial GCT using surgical resection and a high-dose chemotherapy and autologous stem-cell transplantation regimen, highlighting the unique factors which led to the selection of this regimen.
Topics: Adolescent; Brain Neoplasms; Child; Germinoma; Hematopoietic Stem Cell Transplantation; Humans; Lymph Nodes; Male; Neoplasms, Germ Cell and Embryonal; Transplantation, Autologous
PubMed: 34796700
DOI: 10.1002/cnr2.1586 -
British Journal of Neurosurgery Apr 2022Pineal region germinomas are sensitive to radiotherapy. Standard neurosurgical management involves obtaining a tissue biopsy and to relieve the often accompanying...
Pineal region germinomas are sensitive to radiotherapy. Standard neurosurgical management involves obtaining a tissue biopsy and to relieve the often accompanying obstructive hydrocephalus. We present a patient with a suspected hyper-radiosensitive metastatic primary intracranial germinoma where computed tomography scanning resulted in tumor regression before radiotherapy could be administered.
Topics: Biopsy; Brain Neoplasms; Germinoma; Humans; Hydrocephalus; Pineal Gland
PubMed: 30392398
DOI: 10.1080/02688697.2018.1519110 -
Surgical Neurology International 2021Diffuse midline glioma (DMG) is an invasive astrocytic tumor arisen from midline structures, such as the pons and thalamus. Five cases of DMG in the pineal region have...
BACKGROUND
Diffuse midline glioma (DMG) is an invasive astrocytic tumor arisen from midline structures, such as the pons and thalamus. Five cases of DMG in the pineal region have been reported, but the clinical course was poor; there was no case of survival for more than 2 years.
CASE DESCRIPTION
We report the case of a 12-year-old boy with DMG in the pineal region who is living a normal daily life for more than 6 years following multimodal treatment. He complained of a headache accompanied by vomiting that had gradually worsened 1 month previously, and initial magnetic resonance imaging revealed a pineal tumor. Germinoma was initially suspected; however, a combination of chemotherapy using carboplatin and etoposide was ineffective. The first surgery was performed through the left occipital transtentorial approach (OTA); the diagnosis was DMG. After 60 Gy radiotherapy concomitant with temozolomide (TMZ), the tumor enlarged. Second surgery was performed through bilateral OTAs, and 90% of the tumor was removed. In addition, stereotactic radiotherapy (30 Gy, six fractions) was administered, and the local equivalent dose in 2 Gy/fraction reached 97.5 Gy. Maintenance chemotherapy using TMZ and bevacizumab was continued for 2 years. After finishing chemotherapy, the enhancing lesion enlarged again, and bevacizumab monotherapy was effective. Now, at 6 years after diagnosis, the patient leads an ordinary life as a student.
CONCLUSION
Maximum resection and high-dose radiotherapy followed by bevacizumab may have been effective in the present case.
PubMed: 34992928
DOI: 10.25259/SNI_1141_2021 -
Radiology Case Reports Sep 2022Patients with pineal tumors are often asymptomatic and the symptoms depend on the location of the mass. In fact, around 3%-8% of pediatric brain tumor cases are pineal...
Patients with pineal tumors are often asymptomatic and the symptoms depend on the location of the mass. In fact, around 3%-8% of pediatric brain tumor cases are pineal tumors. Children with pineal tumors may present with dizziness and vertigo as early signs. These symptoms are common conditions among 5-15 years old children and could probably lead to misdiagnoses. We present a case of 14-year-old who came to the emergency room of DR. Soetomo Hospital Surabaya with neurotologic symptoms. After a series of radiographic and laboratory examinations, he was diagnosed with germinoma. A ventriculoperitoneal shunt was performed in the emergency room and intravenous dexamethasone was administered.
PubMed: 35789558
DOI: 10.1016/j.radcr.2022.06.001 -
The Malaysian Journal of Pathology Apr 2019Intratumoral calcification is a feature that is more often observed in pineal parenchymal tumour than germinoma. We describe a 13-year-old male with pineal region...
INTRODUCTION
Intratumoral calcification is a feature that is more often observed in pineal parenchymal tumour than germinoma. We describe a 13-year-old male with pineal region germinoma demonstrating extensive intratumoral calcification.
CASE REPORT
He presented with worsening headache that was associated with fatigue, nausea and vomiting. Radiologic examination revealed a multilobular mass in the pineal region with internal calcifications. Biopsy showed a pure germinoma with unusually extensive calcification.
DISCUSSION
Although a diagnosis may be suggested with a careful evaluation of imaging, there is no pathognomonic pattern. Thus, histologic verification is necessary for most pineal region masses.
Topics: Adolescent; Calcinosis; Germinoma; Humans; Male; Pinealoma
PubMed: 31025642
DOI: No ID Found -
JMA Journal Apr 2023Intracranial germ cell tumors are uncommon brain tumors; germinoma is the most common tumor in children and young adults, and the most common regions affected are pineal...
Intracranial germ cell tumors are uncommon brain tumors; germinoma is the most common tumor in children and young adults, and the most common regions affected are pineal gland and suprasellar region. Germinomas of the suprasellar region are accompanied by endocrine alterations, with adipsia being a rare presentation. Here, we present the case of a patient with an extensive intracranial germinoma whose initial presentation was adipsia, without any other endocrinological alteration, with development of severe hypernatremia and unusual manifestations derived from it, such as deep vein thrombosis, myopathy with rhabdomyolysis, and neurological axonal damage.
PubMed: 37179712
DOI: 10.31662/jmaj.2022-0194 -
World Journal of Orthopedics Sep 2021Osteochondromas are the most common benign bone tumor, accounting for 36% of benign bone tumors. Often found within the appendicular skeleton, osteochondromas of the...
BACKGROUND
Osteochondromas are the most common benign bone tumor, accounting for 36% of benign bone tumors. Often found within the appendicular skeleton, osteochondromas of the spine are rare, comprising 4% to 7% of primary benign spinal tumors.
CASE SUMMARY
We report a case of a solitary lumbar osteochondroma in an 18-year-old male with a history of a suprasellar pineal germinoma treated with combined chemotherapy and radiation. He underwent mass excision and partial laminectomy with the ultrasonic bone scalpel (Misonix, Farmingdale, NY, United States) at the L5 Level without the use of adjuvants. The patient returned to work and full activities without back pain at 3 mo postoperatively.
CONCLUSION
Osteochondromas are common tumors of the appendicular skeleton but rarely occur within the spine. This case discussion supplements current osteochondroma literature by describing an unusual presentation of this tumor.
PubMed: 34631455
DOI: 10.5312/wjo.v12.i9.720 -
Journal of the ASEAN Federation of... 2022We report a case of a 24-year-old Filipino male who complained of general weakness, polydipsia, weight loss, bitemporal headaches, loss of libido and behavioral changes....
We report a case of a 24-year-old Filipino male who complained of general weakness, polydipsia, weight loss, bitemporal headaches, loss of libido and behavioral changes. Endocrine work-up revealed neurogenic diabetes insipidus and panhypopituitarism. Brain MRI showed multiple intracranial tumors in the left frontal lobe, pineal and suprasellar region with moderate non-communicating hydrocephalus. Intracranial mass biopsy with ventriculo-peritoneal shunting was done. Histopathology of the mass and CSF revealed a germinoma. He underwent chemoradiotherapy while on maintenance hormone replacement.
Topics: Male; Adult; Humans; Young Adult; Hypopituitarism; Germinoma; Brain Neoplasms; Treatment Outcome; Diabetes Insipidus, Neurogenic
PubMed: 35800587
DOI: 10.15605/jafes.037.01.12 -
Asian Journal of Neurosurgery 2018Germ cell tumors (GCTs) are rare intracranial tumors with a strong predilection for children. Commonly, these tumors arise either in the suprasellar or the pineal...
Germ cell tumors (GCTs) are rare intracranial tumors with a strong predilection for children. Commonly, these tumors arise either in the suprasellar or the pineal region. The basal ganglia-thalamus complex represents a rare site of nonmidline intracranial GCTs. Such basal ganglionic GCTs have been reported to produce certain interesting clinico-radiological features, the knowledge of which may provide important diagnostic clues preoperatively. We present the case of a 9.5-year-old boy who presented with right hemiparesis and precocious puberty. Imaging revealed a heterogeneously enhancing mass involving the left capsuloganglionic region, sparing the thalamus. There was little perilesional edema and midline shift. Because of the deep location of the mass and a lack of mass effect, a neuronavigation-guided tumor biopsy was performed which unraveled a pure germinoma. The child was referred for adjuvant radiotherapy following an uneventful postoperative course. At the time of writing the report, the child was on radiotherapy and doing well.
PubMed: 29682070
DOI: 10.4103/ajns.AJNS_284_16