Anaplastic meningioma with recurrence

Case contributed by Dr Rajalakshmi Ramesh

Presentation

Past medical history of prostate cancer, now presents following an unwitnessed unconscious collapse and a generalized tonic clonic seizure.

Patient Data

Age: 77
Gender: Male
Modality: CT

Erosion of the lateral aspect of the right greater wing of sphenoid and adjacent squamous temporal bone is noted. Aggressive regional periosteal reaction is noted in the region. Associated with this is a contrast enhancing irregular shaped extra axial mass conforming to the contour of the sphenoid wing. This is a solitary lesion. There is moderate underlying vasogenic oedema. No midline shift. Basal cisterns are not effaced.

Conclusion: Aggressive extra-axial mass centred on the right sphenoid wing with osseous invasion. Atypical meningioma is favoured with invasive dural metastasis (e.g. prostate primary) remaining a differential.

Modality: MRI

Extra-axial intermediate T2 signal avidly enhancing lobulated lesion is noted centred on the right greater wing of the sphenoid / squamous temporal bone. There is destruction of the calvarium with extension into the overlying temporalis muscle. On CT scan, the bony margins are quite destructive. Slight reduction in diffusion is noted in the lesion suggestive of a cellular lesion. The lesion measures 2.9 x 5.5 x 4.6 cm (trans x ap x cc). Small amount of associated dural thickening is noted. The lesion appears to be solitary. Underlying vasogenic oedema is noted in the temporal and frontal lobe with slight mass effect. Given the degree of osseous destruction the possibility of a more aggressive lesion such as a haemangiopericytoma or a metastases (if the patient is known to have a malignancy) should also be considered.

In light of his malignant history, CT imaging of his chest, abdomen and pelvis was performed, which failed to identify any metastatic disease. He underwent pre-operative embolization of the extra-axial space occupying lesion, followed by a stereotactic right fronto-temporal craniectomy and resection of the tumor.

 

Histopathology:

MACROSCOPIC DESCRIPTION: "Brain tumour":  An oval shaped piece of friable fawn tissue 12mm in maximum dimension.  Portion for frozen section.

FS DIAGNOSIS:  Favour haemangiopericytoma. 

MICROSCOPIC DESCRIPTION:

The sections show a densely hypercellular tumour with a broad dural base. Tumour cells have moderately pleomorphic round and oval vesicular nuclei and a variable amount of pale cytoplasm and are arranged in diffuse sheets and perivascular pseudorosettes. Poorly formed papillary structures are noted in some areas. There are frequent mitotic figures (7 in 10 high power fields) as well as scattered foci of necrosis. Tumour cells show moderate cytoplasmic immunostaining for epithelial membrane antigen (EMA), E-cadherin and vimentin and patchy moderate staining for cytokeratin AE1/AE3. No staining for GFAP, progesterone receptor (PgR) or CD34 is seen in tumour cells. The features are of anaplastic (malignant) papillary meningioma.  The topoisomerase labeling index is approximately 10%.

DIAGNOSIS: Brain tissue:  Anaplastic (malignant) papillary meningioma WHO Grade III

 

The patient was treated with post-operative radiotherapy. His postoperative recovery was complicated by partial complex seizures, which were medically managed.  One month following his craniectomy surgery, the patient underwent a stereotactic right fronto-temporal cranioplasty.

 

Modality: CT

Non-contrast postoperative scans show right frontal cranioplasty. Deep to this, there is a collection of mixed low attenuation and air with a maximum depth of 7 mm but no appreciable mass effect. Small amount of right frontal lobe low attenuation is longstanding. No intracranial haemorrhage. Ventricular size is normal.

He remained under close radiological surveillance, with his one year post-operative scan shown below. 

Modality: MRI

Comparison is made to prior examinations. No evidence of recurrence of the right middle cranial fossa meningioma – some minor post-operative meningeal enhancement lies deep to the craniotomy. Moderate gliosis involves the lateral right temporal lobe as well as the frontal operculum. Cerebral parenchymal signal intensity in architecture, and ventricular volume is otherwise stable. Conclusion:

Stable appearances - no evidence of tumour recurrence.

Unfortunately, 1.5 years following his diagnosis, the patient suffered a stroke, leaving him with a residual dense left facial, upper and lower limb hemiparesis. He was commenced on aspirin therapy and underwent rehabilitation therapy.

Modality: MRI

6x1.0cm (axial) acute infarct in right corona radiata immediately adjacent to lateral ventricle body. There is associated bright FLAIR signal.

Within the limits of noncontrast study, no extraaxial tumour recurrence is seen around the right middle cranial fossa. There are gliosis and atrophy in the adjacent right frontal and temporal lobes. The extent of underlying white matter bright flair/T2 signal has however increased markedly, now extending posteriorly to the trigonal area (where previously it was fairly localized to around the anterior horn region). Radiotherapy can potentially produce this appearance.

At his two year post-operative juncture, routine surveillance imaging unfortunately demonstrated recurrence. 

Modality: MRI

Focal rounded extraaxial enhancing mass is seen in the right temporoparietal region, measuring 35mm AP, 28 mm SI and 24 mm transverse. Irregular enhancement is seen in the adjacent brain. Extensive T2 and FLAIR signal abnormality seen in the white matter throughout the right cerebral hemisphere. Ex-vacuo dilatation related to an old infarct in the right Corona radiata. Encephalomalacia in the anterolateral aspect of the right temporal lobe related to prior surgery. There is a further enhancing 8 x 8.5mm lobulated nodule in this region. Conclusion: Recurrent aggressive appearing meningioma-large nodule with possible brain invasion into the right temporoparietal region and smaller nodule anterolaterally in the right middle cranial fossa.

He underwent a redo fronto-temporal craniotomy and tumor resection. 

Modality: MRI

Recent right temporoparietal craniotomy noted, with resected posterolateral right temporal convexity tumour, compatible with stated meningioma invaginating/invading the temporoparietal junction. Subtle fluffy ill- defined parenchymal enhancement marginates the posterior and medial margins of the gliotic cavity, with trace parenchymal tumour residuum not excluded. The separate 5-6 mm enhancing extra-axial tumour nodule, centred laterally in the right middle cranial fossa, remains stable. Of note, a large area of diffusion restriction, consistent with infarction, has developed inferiorly and posteriorly in the right MCA territory amongst extensive pre-existing right cerebral vasogenic oedema and gliosis, with no overt positive mass effect demonstrated.

Histopathology: 

MACROSCOPIC DESCRIPTION: "Brain tissue": A wedge shaped piece of fawn tissue 8x8x6 mm.  All for frozen section.

FS DIAGNOSIS: Meningioma with atypical feature

DIAGNOSIS: Brain tissue: Recurrent anaplastic (malignant) meningioma (WHO Grade III).

 

The patient had an uneventful post-operative recovery and underwent further rehabilitation. He elected not to have any further adjuvant therapy nor surgery in the future. He has also declined any further radiological surveillance and follow-up. 

Case Discussion

This case describes an anaplastic malignant WHO grade 3 tumor (papillary meningioma) with aggressive recurrence. 

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Case Information

rID: 34452
Case created: 21st Feb 2015
Last edited: 12th Jul 2016
Inclusion in quiz mode: Included

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