Intraparenchymal papillary meningioma of brainstem: case report and literature review
- Xiao-Bing Jiang†1,
- Chao Ke†1,
- Zhi-An Han2,
- Shao-Hua Lin2,
- Yong-Gao Mou1,
- Rong-Zhen Luo3,
- Shao-Xiong Wu4 and
- Zhong-Ping Chen1Email author
© Jiang et al; licensee BioMed Central Ltd. 2012
Received: 14 September 2011
Accepted: 12 January 2012
Published: 12 January 2012
Both intraparenchymal papillary meningioma and papillary meningioma with cyst formation of brainstem have never been reported. The authors present an extremely rare case of patient with intraparenchymal papillary meningioma of brainstem. A 23-year-old Chinese male presented with a 4-month history of progressive left upper limb and facial nerve palsy. Magnetic resonance imaging revealed a cystic-solid, heterogeneously enhancing mass in pons and right cerebral peduncle with no dural attachment. The tumor was totally removed via subtemporal approach. During surgery, the lesion was found to be completely intraparenchymal. Histological and immunohistochemical examinations were compatible with the diagnosis of papillary meningioma. The lesion recurred nine months after primary surgery, a second surgery followed by radiotherapy was performed. Till to now (nearly 2 years after the treatment), the patient is tumor free survival. Intraparenchymal meningioma of brainstem with cystic formation is very rare, however, it should be considered as a differential diagnosis of a brainstem neoplasm. The present case strongly recommended that postoperative radiotherapy was essential for the patients with papillary meningiomas.
KeywordsMeningioma Papillary Brain stem Intraparenchymal Cyst
Meningiomas account for nearly 35% of all primary intracranial neoplasms, deriving from the arachnoid cap or meningothelial cells . Therefore, they usually display a dural attachment . Unusually, they could present with no dural attachment [3–6]. The majority of the meningiomas without dural attachment are supratentorial, whereas the infratentorial ones are relatively less frequent. In reviewing the literatures, only thirteen infratentorial meningiomas without dural attachment excluding the one in the fourth ventricle have been reported, and only one case was in the brainstem . Intraparenchymal papillary meningiomas of brainstem, to our knowledge, have never been reported before.
Cyst formation is uncommon in the meningioma, which most commonly develops in fibroblastic and meningothelial meningiomas. Papillary meningiomas demonstrating cystic changes were reported only in five cases in previous literatures . Here, we report a case of intraparenchymal meningioma of brainstem. This should be the first case of intraparenchymal papillary meningioma of brainstem, as well as the first papillary meningioma with cyst formation located in brainstem.
The patient underwent a total surgical resection of the tumor mass via a right subtemporal approach, under general anesthesia. The cystic-like part of the mass was full of xanthochromic fluid, and the solid part was found to be fragile and hemorrhagic, with defined margin from the adjacent tissue. The lesion was purely intraparenchymal, and no dural or leptmeninges attachment was observed during the operation. The tumor was removed totally, the postoperative course was uneventful. His left side facial palsy was completely relieved and his left upper limb returned to be normal after operation.
Infratentorial meningiomas without dura attachment are uncommon. Reviewing the literatures [9–12], only forty-four cases have been reported including thirty-one intraventricular meningiomas. In previous literatures [6, 13, 14], infratentorial meningiomas without dural attachment were classified into 5 types: meningioma arising from the choroid of the fourth ventricle and lying wholly within it, meningioma arising from the inferior tela and lying partially in the fourth ventricle and partially in the cerebellar hemisphere, meningioma lying in the cisterna magna, meningioma arising from the choroid plexus and lying in the lateral cerebellomedullary cistern, and intraparenchymal meningioma. However, Zhang et al.  suggested that meningiomas in the ventricular system should be distinguished from the other types of no dural attachment meningiomas, because they were quite different from each other. Intraparenchymal meningioma is considered as a same type of subcortical meningioma, which locates in brain parenchyma without dural attachment, although they can reach the surface of the brain [6, 15]. Based on the imaging and intraoperative findings, the present case should be classified as an intraparenchymal meningioma. To the best of our knowledge, there was only one case of intraparenchymal brainstem meningioma reported , who was a 22-month old child with a clear cell meningioma located in the upper medulla and pons. Similarly, the lesion in present case mainly located in pons and extended to cerebral peduncle. Nevertheless, intraparenchymal papillary meningioma of brainstem has never been described before. Herein, including present case, only fourteen cases of infratentorial meningiomas without dural attachment have been reported in the literatures, except for the one in the fourth ventricle. There were four cases deriving from the inferior tela choroidea , four cases lying in the cisterna magna [16, 17], four cases in the cerebellomedullary cistern [13, 18–20], and two cases were intraparenchymal .
Summary of published infratentorial meningiomas without dural attachment excluding the one in the fourth ventricle (n = 8)
Kim et al.
Left cerebellum-edullary cistern
T2., heterogeneous C.e.,homogenous enhancement
52 × 28
Mild and transient dysphasia
Mixed pattern of meningothelial and fibroblastic
Shibuya et al.
Left cerebellom-edullary cistern
Round enhancing mass with multiple foci of calcification
T2., high intense C.e., heterogeneous enhancement
Ishigaki et al.
From right CPA to the foramen of Luschka
High density without calcification
T2., high intense C.e., heterogeneous enhancement
Nakahara et al.
Lateral site of right cerebellar cortex
Small round non-enhancing lesion
T2., not reported C.e., partial enhancement
Teo et al.
right hemipares-is, facial nerve palsy and dysphagia
Upper medulla and pons
T1., not reported
T2., not reported C.e., multilobulated mass
40 × 40 × 38
Persistent right hemiparesis with right facial nerve palsy
Nicoletti et al.
Nausea and vomiting
T2., not reported C.e., homogeneous enhancement
About 35 × 27
Jung et al.
Neck pain and a tingling sensation of left arm
T2., hyperintense C.e., homogeneous enhancement
33 × 20 × 20
Syncytial and fibroblastic
Left upper limb nerve palsy and impairment of fine movement
Pons and right cerebral peduncle
T2., heterogeneous C.e., cystic-solid mass with heterogen- eous enhancement
35 × 25 × 20
Mild left upper hemiparesis
Infratentorial meningiomas without dural attachment represent surgical challenge, requiring special considerations, because of the vicinity of the brainstem, cranial nerves, and veterbrobasilar arteries. Despite recent improvements in surgical techniques, surgical treatment of meningiomas in this area still carries a high risk of morbidity and mortality. In some cases, the lesion might be strongly adherent to these structures, leading to a partial or subtotal resection. Nevertheless, in more than half of the reports (5/8), a total resection was reached with mild complications. This might be largely ascribed to the distinct relationship between lesion and surrounding tissues.
Papillary meningioma is an aggressive variant of meningioma, which accounts for 1-2.5% of all meningiomas . In comparing with benign meningiomas, they usually display aggressive clinical behavior marked by a high rate of brain parenchymal invasion, local recurrence, and extracranial metastases. It is pathologically defined by the presence of perivascular pseudopapillary pattern either entirely or more commonly in combination with other common histological componets of meningiomas. It has been graded as a WHO grade III tumor [22, 23]. Cyst formation is uncommon in meningiomas, which most commonly develops in fibroblastic and meningothelial type meningiomas. The mechanisms leading to cyst formation are still unclear. Confluence of micro-cysts and intratumoral hemorrhages may be possibilities regarding the formation of intratumoral cysts . Only five cases of papillary meningioma with cyst formation were reported in the literatures . The present case should be the first description of intraparenchymal papillary meningioma with cyst formation arising in brainstem. The presence of cyst formation and heterogeneous enhancement made it mimic other neoplasms, such as gliomas, hemangioblastomas, and metastatic tumors. Nevertheless, a remarkable papillary pattern of tumor tissue, as well as immunohistochemistry findings, strongly support the fact that it is a papillary meningioma. The lesion recurred in only nine months despite total resection of primary tumor, which was also in accordance with the aggressive clinical behavior of papillary meningioma.
Due to the rarity of papillary meningiomas, no clear consensus has achieved regarding the proper management of papillary meningiomas. However, aggressive surgical resection plus postoperative radiotherapy has come to represent the standard care . In current case, both of the primary and recurrent lesions were totally removed with mild complications. If the patient underwent postoperative radiotherapy, recurrent of primary tumor might not occur in such short period. Fortunately, the patient remains free from tumor more than 2 years after repeated resection plus radiotherapy.
The authors described an extremely unusual case with intraparenchymal papillary meningioma of brainstem with cyst formation. Although this is very infrequent, meningiomas should be included in the differential diagnosis of an intraparenchymal brainstem tumor. Papillary meningioma is a distinct variant of meningioma with highly aggressive behavior. The experience of the present case strongly indicates that postoperative radiotherapy is essential for the patients with papillary meningiomas.
Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
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