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Central Nervous System
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2020
The management of pineal tumors includes imaging the entire neuraxis, immediate decompression of hydrocephalus, and establishing a tissue diagnosis. Pineocytoma is a surgical disease and may be cured by total resection. Pineoblastoma is less likely to be cured by surgery, although complete resection should be attempted where possible. There is evidence that whole-neuraxis radiotherapy with the boost directed at the tumor improves outcome, particularly if resection is incomplete. Chemotherapy responses can also be seen. Intermediate (Grade 2/3) tumors present particular difficulties in management. Again, complete surgical excision should be attempted where possible. The need for radiotherapy will depend on the completeness of resection, the mitotic rate, and evidence of dissemination away from the primary.
Radiotherapy for Pediatric Central Nervous System Tumors – Techniques and Strategies
Published in David A. Walker, Giorgio Perilongo, Roger E. Taylor, Ian F. Pollack, Brain and Spinal Tumors of Childhood, 2020
Pineoblastoma was previously classified as an embryonal tumor but in the 2016 WHO classification is grouped with the pineal parenchymal tumors. However, management strategies still follow those of embryonal tumors with surgery, CSI, and local boost and multiagent chemotherapy.44,45
Oncology
Published in Stephan Strobel, Lewis Spitz, Stephen D. Marks, Great Ormond Street Handbook of Paediatrics, 2019
Gill A. Levitt, Penelope Brock, Tanzina Chowdhury, Mark Gaze, Darren Hargrave, Judith Kingston, Antony Michalski, Olga Slater
Cerebellar tumours present with ataxia and signs of raised ICP. Patients with tumours in the pineal region (pineoblastoma) may have Parinaud syndrome (failure of upward gaze, dilated pupils that react to convergence but not light, nystagmus and lid retraction).
Novel RB1 germline mutation in a healthy man
Published in Ophthalmic Genetics, 2022
Eugenia M. Ramos-Dávila, Lucas A. Garza-Garza, Rocío Villafuerte-de la Cruz, Dione Aguilar-Y-Mendez, Héctor J. Morales-Garza, Manuel Garza-Leon, Raul E. Ruiz-Lozano, David Ancona-Lezama
A 5-month-old girl with no relevant background was referred to the Ocular Oncology Service due to the presence of leukocoria in the right eye (OD) first detected 30 days prior. Clinical examination revealed a visual acuity of no-fixation OD and fix and follow in the left eye (OS). Additionally, she rejected OS occlusion during examination. Eyelids and adnexa were normal in both eyes (OU). Examination under anesthesia (EUA) revealed a normal anterior segment in both eyes (OU). Fundoscopy evidenced a single tumoral mass OD and three retinoblastoma lesions OS (Figure 1). Vitreous seeds were present in OD. Initial staging determined OD with Group “D” and OS with Group “B,” according to the International Classification of Retinoblastoma (ICRB) (13). Ocular ultrasound (US) was performed during the EUA (Figure 2), confirming clinical findings. Cryotherapy was applied to every lesion in OS and the mid-periphery of OD. In addition, the pediatric oncologist administered six cycles of intravenous chemotherapy (IVC) consisting of Vincristine, Etoposide, and Carboplatin. Magnetic Resonance Imaging (MRI) of the brain confirmed the absence of pineoblastoma and normal anatomy. Following treatment, all tumors OU experimented complete regression. At six-month follow-up, the patient is disease-free (Figure 2).
Comment: conservative management of massive choroidal relapse in retinoblastoma patients?
Published in Ophthalmic Genetics, 2018
In case 1, the authors state that the child was initially diagnosed with unilateral bifocal IIRC group B tumor at 7 months of age. Was genetic testing performed for the child to look for hertitable pattern of retinoblastoma? This could have lead to more frequent and close monitoring of the child with magnetic resonance imaging (MRI) for screening and early detection of pinealoblastoma. The authors state that SD OCT could detect early signs of choroidal invasion 3 weeks prior to the visit when the lesion was clinically detected. What was the timing of the first examination at the subclinical stage of the lesion? Did it coincide with the time of starting intensive induction adjuvant chemotherapy for pinealoblastoma? In that case, how does one explain the choroidal mass growing in size while the child continued to receive cycles of intensive adjuvant chemotherapy and then finally regressing completely, while attributing the regression to adjuvant chemotherapy itself.
Intracranial hemangiopericytoma after radiation treatment: first case in the literature
Published in British Journal of Neurosurgery, 2023
Gulden Demirci Otluoglu, Emre Zorlu, Baran Yılmaz, Akın Akakın, Ozlem Yapıcıer, Turker Kilic
A twenty-five-year-old male patient was admitted with sudden onset headache, upward gaze palsy and altered consciousness. Magnetic Resonance (MR) showed a heterogeneously enhancing lesion in the pineal region and hydrocephalus. He had a VP shunt surgery first and then a left occipital craniotomy and a gross total excision of the tumor. Histology was of a pineoblastoma. In the post-operative period, he had radiotherapy with IMRT (Intensity Modulated Radiation Treatment) method (6000 cGy in 30 fractions). Forty-one month later he had a new extra-axial lesion heterogeneously enhancing in the right parietal parafalcine area. Second surgery was performed via right parietal craniotomy and gross total excision was achieved (Figure 1). Histology was of cellular mesenchymal tumor composed of fibroblastic type cells showing a rich thin-walled branching staghorn-like vessels. Tumor showed a delicate and rich network of reticulin fibers typically investing individual tumor cells. A mitotic count of 3 mitoses per 10 high-power fields was seen. Necrosis was not identified. Immunohistochemical analysis was carried out. Epithelial membrane antigen (EMA), progesterone receptor (PR) and Glial fibrillary acidic protein (GFAP) immunostains were negative. Tumor cells were only positive for CD34 and STAT6. The diagnosis was consistent with hemangiopericytoma, grade II (David N. Louis6) (Figure 2). He had adjuvant radiotherapy again with IMRT (Intensity Modulated Radiation Therapy) method to the second surgical excision area (5000 cGy in 30 fractions). He is now under follow up without any new intracranial lesion or extracranial metastasis 42 months since his last surgery.