Tumour-like mass lesion: an under-recognised presentation of primary angiitis of the central nervous system

Molloy, Eamonn; Singhal, Aneesh B.; Calabrese, Leonard H.

doi:10.1136/ard.2008.096800

Cited by 109 publications

(99 citation statements)

References 29 publications

(18 reference statements)

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“…Given that the prognosis of PACNS may be poor in the absence of specific therapy, early diagnosis and treatment is essential. Diagnosis is often delayed due to the extremely heterogeneous clinical presentations and non-specific MRI features (4). PACNS presenting as a CNS mass lesion is extremely rare, and thus, PACNS is usually not considered in the differential diagnosis of mass lesions.…”

Section: Discussionmentioning

confidence: 99%

A rare case of tumor-mimicking primary angiitis of the central nervous system

Sun

Zhu

Zhao

et al. 2016

Molecular and Clinical Oncology

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Abstract. Primary angiitis of the central nervous system (PACNS) is a rare, but severe vascular disease. The present study reports the case of a 42-year-old male who developed PACNS. Magnetic resonance imaging (MRI) scans initially led to a misleading diagnosis of malignant glioma, and surgery was performed. The mass was resected, and a pathological examination confirmed a cerebral vasculitis. Single therapy with high doses of steroid did not improve the patient's condition, while a subsequent lesion appeared on the opposite side one year later. Combined therapy with methylprednisone and cyclophosphamide resulted in a great improvement for the patient. No relapse occurred during one year's follow-up. Although a tumor-mimicking PACNS has no established imaging features, a diagnosis of tumor-mimicking PACNS should be suspected when the MRI reveals inappropriate presentations of a tumor. Greater awareness of this potential manifestation of PACNS may facilitate more prompt diagnosis and treatment.

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Section: Discussionmentioning

confidence: 99%

A rare case of tumor-mimicking primary angiitis of the central nervous system

Sun

Zhu

Zhao

et al. 2016

Molecular and Clinical Oncology

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“…No statistically significant differences in outcome were noted between the three histological patterns, 10) although deposition of amyloid protein is related to poorer outcomes in PACNS. 11) Eight of 31 patients with PACNS diagnosed by biopsy specimens were positive for amyloid protein. 14) The histological features in our case were consistent with a granulomatous inflammatory pattern and staining for amyloid was negative.…”

Section: Discussionmentioning

confidence: 99%

“…5,8,15) Single or multiple mass lesions are also known in PACNS. 8,11,12,19) Approximately 5% of cases with PACNS present as a tumor-like mass lesion. 11) One of the mechanisms of formation of mass lesion is breakdown of the blood-brain barrier of the small vessels by infiltration of inflammatory cells in the perivascular and parenchymal regions, resulting in the mass mimicking enhanced lesion.…”

Section: Discussionmentioning

confidence: 99%

Primary Angiitis of the Central Nervous System Mimicking Tumor-Like Lesion -Case Report-

Tanei

Nakahara

Takebayashi

et al. 2011

Neurol. Med. Chir.(Tokyo)

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A 60-year-old man presented with a rare case of primary angiitis of the central nervous system mimicking a tumor-like lesion and manifesting as slight disorientation, left hemiparesis, and motor aphasia. Computed tomography showed multiple low density lesions in the left frontal lobe, brain stem, and right parietal lobe. Magnetic resonance images revealed a slightly enhanced mass lesion in the right parietal lobe with surrounding brain edema. Serum, cerebrospinal fluid, and other image examinations did not show any abnormal findings, so surgical removal of the right parietal mass was performed. Histological examination revealed that the mass consisted of hemorrhagic infarction without cellular atypia. Proliferations of endothelial cells in small and medium arteries, and infiltration of macrophages in the perivascular space were detected in the infarction tissues. The histological diagnosis was primary angiitis of the central nervous system.

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“…Leptomeningeal enhancement may be seen in 10% of cases [31]. Mass lesions may be seen in 5% of cases, and generally mimic a tumour or abscess [23]. Rarely, confluent white-matter lesions may be seen [5,33,41].…”

Section: Neuroimagingmentioning

confidence: 99%

“…They identified a total of 38 ML: eight of 202 (4.0%) patients from CC/MGH and 30 of 535 (5.6%) patients identified from the medical literature. Excision of the lesion may be curative; however, in some patients aggressive immunosuppressive therapy has led to a favourable outcome obviating the need of surgery [23].…”

Section: Clinical Profilementioning

confidence: 99%