Complete regression of adult pineoblastoma following radiotherapy: A case report and review of the literature

Abstract

Abstract. Adult pineoblastomas (PBs) are rare central nervous system tumors. Little is known with regard to the clinical features and outcomes of adult PB, and optimal treatment strategies for adult PB remain to be determined. The current report describes a case of PB in a 46-year-old male, who presented with obstructive hydrocephalus due to a large pineal region mass. Considering the potential effect on quality of life, the patient underwent a partial resection. Postoperative radiotherapy, comprising prophylactic craniospinal irradiation at a dose of 34.2 Gy followed by a local 25.3-Gy 'boost' to the tumor site for a total dose of 59.5 Gy, resulted in the complete regression of the tumor without neurological deficits. The patient has remained free of recurrence for 36 months after radiotherapy. This case highlights a minimally invasive strategy to treat a rare pineal region tumor with significant involvement of critical structures that resulted in a favorable response and an excellent neurological outcome. The radiographic and histopathological features of PB are also reviewed, and the various treatment options reported in the literature are discussed. IntroductionPineal region tumors, which account for <1% of intracranial tumors (1), encompass a heterogeneous group of neoplasms that may be divided into four main categories: Germ cell tumors, glial cell tumors, pineal parenchymal tumors (PPTs) and other miscellaneous tumors and cysts. PPTs originate from cells in the pineal gland called pinealocytes, and represent only 0.3% of all primary tumors of the central nervous system (2). The current World Health Organization classification of PPTs includes well-differentiated pineocytoma (PC), PPT of intermediate differentiation and poorly differentiated pineoblastoma (PB) (3).PB is more common in children than in adults. It has been reported that the peak incidence of PB occurs in the first 4 years of life, with tendency to arise in the first and second decades (4). In addition, adult cases of PB account for <10% of published cases (5). Due to the rarity of PB, relevant data is limited, particularly regarding PB in adults. Thus, the biology, standard management and prognosis of PB are not well understood at present.The current report describes a case of PB occurring in a 46-year-old male who presented with obstructive hydrocephalus due to a large pineal region mass. This case highlights a minimally invasive strategy to treat a rare pineal region tumor with significant involvement of critical structures in an adult. Written informed consent was obtained from the patient. Case reportA 46-year old male was admitted to West China Hospital of West China Medical School (Sichuan University, Chengdu, China) in June 2011 with a 3-month history of mild headache, dizziness and impaired vision. No abnormalities were observed upon physical examination and laboratory tests. An ophthalmological exam revealed no evidence of papilledema.Computed tomography (CT) of the brain was performed, and a solid mass measuring 5 cm in diameter wa...

<p>Adverse Factors of Treatment Response and Overall Survival in Pediatric and Adult Patients with Pineoblastoma</p>

“…6,8 Previous studies showed that heterogeneous enhancement is common in PBs. 4,9 Our study also confirmed this observation and found that hypo-intense on T1 weighted images and hyper-intense on T2 weighted images occurred mainly for the radiologic features.…”

“…While the administration of adjuvant radiotherapy for PBs in adults has been identified to be useful to increase OS, a statistically significant effect on OS was not demonstrated owing to limited case reports. 6 , 9 , 12 One case report advocated adjuvant chemotherapy for adult patients. 15 In a previous large series of 34 adult cases, the contribution of chemotherapy to a favorable OS remained unclear.…”

Post-Surgical Prognosis of Patients with Pineoblastoma: A Systematic Review and Individual Patient Data Analysis with Trends over Time

“…Of the 2701 articles retrieved in the search, 44 studies with IPD on 298 patients with pineoblastoma were included in the systematic review ( Table 1 ) [ 5 , 6 , 8 , 9 , 10 , 11 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 53 , 54 , 55 , 56 , 57 , 58 , 59 , 60 , 61 ]. Figure 1 presents the PRISMA flowchart for this systematic review [ 18 ].…”

A Case Report of Adult Pineoblastoma Occurring in a Pregnant Woman

“…As mentioned above, starting from the second decade, adult cases are very rare and represent less than 10% of published literature (6). For this reason, limited data are currently available in the effort of developing a standard management of the disease, with a lack of consensus about the optimal combination of a multimodality treatment involving surgery, radiotherapy and/or chemotherapy.…”