scholarly journals Pineocytoma with diffuse dissemination to the leptomeninges

Rare Tumors ◽  
2011 ◽  
Vol 3 (4) ◽  
pp. 163-165 ◽  
Author(s):  
Caitlin Gomez ◽  
Jeffrey Wu ◽  
Whitney Pope ◽  
Harry Vinters ◽  
Antonio DeSalles ◽  
...  

Pineal parenchymal tumors are rare. Of the three types of pineal parenchymal tumors, pineocytomas are the least aggressive and are not known to diffusely disseminate. In this paper, we report the successful treatment of a case of pineocytoma with diffuse leptomeningeal relapse following initial stereotactic radiotherapy. A 39-year-old female presented with headaches, balance impairment, urinary incontinence, and blunted affect. A pineal mass was discovered on magnetic resonance imaging (MRI). A diagnosis of pineocytoma was established with an endoscopic pineal gland biopsy, and the patient received stereotactic radiotherapy. Ten years later, she developed diffuse leptomeningeal dissemination. The patient was then successfully treated with craniospinal radiation therapy. Leptomeningeal spread may develop as late as 10 years after initial presentation of pineocytoma. Our case demonstrates the importance of long-term follow-up of patients with pineal parenchymal tumors following radiation therapy, and the efficacy of craniospinal radiation in the treatment of leptomeningeal dissemination.

2019 ◽  
Vol 1 (Supplement_2) ◽  
pp. ii17-ii17
Author(s):  
Takamasa Hiraki ◽  
Kouhei Fukuoka ◽  
Yusuke Tsumura ◽  
Kyohei Inoue ◽  
Osamu Tomita ◽  
...  

Abstract Medulloblastoma is one of the most common malignant brain tumors in children. Despite multi-disciplinary treatment for medulloblastoma, including surgery, chemotherapy, and radiation, which have resulted in significant improvement of the prognosis, about 30% of patients still experience recurrence. Most recurrences occur within the first 15 months from diagnosis and late relapse of the tumor is quite rare. We report a case of a 15-year-old female patient with recurrent medulloblastoma 9 years after the primary tumor. At the age of 6, this patient developed a posterior fossa tumor without metastasis and underwent near-total resection. The pathological diagnosis was medulloblastoma with focal desmoplasia. After the surgery, she received multi-agent chemotherapy and radiation therapy consisting of 18 Gy craniospinal irradiation and 51.2 Gy local irradiation. She was in complete remission for 9 years after the treatment. However, gait disturbance began to gradually appear, and magnetic resonance imaging (MRI) showed an intradural lesion in her thoracic spine. The lesion was biopsied, and the pathological findings confirmed the recurrence of medulloblastoma. Currently, we plan to administer local radiation therapy concomitantly with temozolomide to the patient. The case reminds us of the importance of long-term careful follow-up of patients with medulloblastoma. Further studies are warranted for the treatment of relapsed medulloblastomas due to the limited information available at present.


1991 ◽  
Vol 30 (06) ◽  
pp. 265-271 ◽  
Author(s):  
J. Rütt ◽  
A. Linden ◽  
Kamilla Smolarz ◽  
E. Voth ◽  
H. Schicha ◽  
...  

The value of conventional radiology, bone scintigraphy and magnetic resonance imaging (MRI) in the early diagnosis of Legg-Calvé-Perthes disease (LCPD) was assessed. The initial results were compared with the clinical and radiological findings of long-term follow-up in 43 children. Radiological and scintigraphic examination resulted in a relatively high number of equivocal findings (16% and 10%, respectively). MRI findings were less equivocal (3%). Depending on whether such findings were classified as normal or as pathological, the diagnostic accuracy ranged as follows: radiography 88–93%, bone scintigraphy 88-91%, and MRI 97–99%. Therefore, MRI by itself seems to be sufficient to detect or exclude LCPD. The results of this study and the radiation exposure associated with radiography and scintigraphy raise the question whether MRI should be the diagnostic method of choice in patients with suspected LCPD.


2004 ◽  
Vol 130 (6) ◽  
pp. 327-333 ◽  
Author(s):  
Hans Geinitz ◽  
Frank B. Zimmermann ◽  
Reinhard Thamm ◽  
Monika Keller ◽  
Raymonde Busch ◽  
...  

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii390-iii391
Author(s):  
Aaron Mochizuki ◽  
Anna Janss ◽  
Sonia Partap ◽  
Paul Fisher ◽  
Yimei Li ◽  
...  

Abstract INTRODUCTION Medulloblastoma is one of the most common malignant brain tumors in children. To date, the treatment of average-risk (non-metastatic, completely resected) medulloblastoma includes craniospinal radiation therapy and adjuvant chemotherapy. Modern treatment modalities and now risk stratification of subgroups have extended the survival of these patients, exposing the long-term morbidities associated with radiation therapy. METHODS We performed a single-arm, multi-institution study, seeking to reduce the late effects of treatment in patients with average-risk medulloblastoma prior to advances in molecular subgrouping. To do so, we reduced the dose of craniospinal irradiation by 25% to 18 gray with the goal of maintaining the therapeutic efficacy as described in CCG 9892 with maintenance chemotherapy. RESULTS 28 patients aged 3–30 years were enrolled across three institutions between April 2001 and December 2010. Median age at enrollment was 9 years with a median follow-up time of 11.7 years. The 3-year relapse-free (RFS) and overall survival (OS) were 78.6% (95% CI 58.4% to 89.8%) and 92.9% (95% CI 74.4% to 98.2%), respectively. The 5-year RFS and OS were 71.4% (95% CI 50.1% to 84.6%) and 85.7% (95% CI 66.3% to 94.4%), respectively. Toxicities were similar to those seen in other studies; there were no grade 5 toxicities. CONCLUSIONS Given the known neurocognitive adverse effects associated with cranial radiation therapy, studies to evaluate the feasibility of dose reduction are needed. In this study, we demonstrate that select patients with average-risk medulloblastoma may benefit from reduced craniospinal radiation dose of 18 gray without impacting relapse-free or overall survival.


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