Spontaneous rupture of malignant papillary craniopharyngioma with CSF seeding and metastatic deposits: A case report and literature review
Introduction: Craniopharyngiomas are benign tumours mainly confined to the cranial cavity in the suprasellar region. Research Question and Case Description: We present a rare case of an aggressive papillary craniopharyngioma with disseminated spinal intradural disease. A 67-year-old woman presented...
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Format: | Article |
Language: | English |
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Elsevier
2022-01-01
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Series: | Brain and Spine |
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Online Access: | http://www.sciencedirect.com/science/article/pii/S2772529422000625 |
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author | Asfand Baig Mirza Melika Akhbari Christopher Murphy Steve Connor Mark R. Howard Zita Reisz Sinan Barazi Nick Thomas Istvan Bodi Eleni C. Maratos |
author_facet | Asfand Baig Mirza Melika Akhbari Christopher Murphy Steve Connor Mark R. Howard Zita Reisz Sinan Barazi Nick Thomas Istvan Bodi Eleni C. Maratos |
author_sort | Asfand Baig Mirza |
collection | DOAJ |
description | Introduction: Craniopharyngiomas are benign tumours mainly confined to the cranial cavity in the suprasellar region. Research Question and Case Description: We present a rare case of an aggressive papillary craniopharyngioma with disseminated spinal intradural disease. A 67-year-old woman presented with a 4-month history of headache, visual disturbance, acute confusion and radicular leg pain. Previous history of breast carcinoma (ER + PR + HER2-) was noted. The importance of histological diagnosis prior to treatment of sellar or suprasellar lesions with atypical or aggressive features is explored. Materials and methods: MRI demonstrated a partly solid and partly cystic pituitary mass lesion in the sellar and suprasellar region with chiasmal compression and hypothalamic involvement. The sella was mildly enlarged and there were no calcifications. Whole neuraxis MRI revealed intradural deposits involving the ventricular system, spinal cord and conus. Within a month, the lesion rapidly increased in size. The patient underwent a craniotomy and transventricular resection of the sellar and suprasellar mass. Cranial lesion histology favoured papillary craniopharyngioma, confirmed by BRAF V600 mutation. Lumbar puncture CSF cytology confirmed craniopharyngioma with BRAF mutation and no evidence of metastatic breast cancer. Results: The patient remained confused postoperatively without focal neurological deficit and underwent palliative whole brain radiotherapy. She died 4 months later. A review of the literature identified 29 reports of ruptured craniopharyngioma. Discussion and Conclusion: Ruptured craniopharyngioma presents with a suprasellar mass and drop lesions in the spinal canal, characteristics radiologically indistinguishable from metastatic disease. The importance of histological diagnoses in directing the management of these cases is highlighted. |
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id | doaj.art-a51ef4cd11e04b52a1bcb5c94cfd60f6 |
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issn | 2772-5294 |
language | English |
last_indexed | 2024-04-11T05:12:06Z |
publishDate | 2022-01-01 |
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series | Brain and Spine |
spelling | doaj.art-a51ef4cd11e04b52a1bcb5c94cfd60f62022-12-25T04:20:33ZengElsevierBrain and Spine2772-52942022-01-012100921Spontaneous rupture of malignant papillary craniopharyngioma with CSF seeding and metastatic deposits: A case report and literature reviewAsfand Baig Mirza0Melika Akhbari1Christopher Murphy2Steve Connor3Mark R. Howard4Zita Reisz5Sinan Barazi6Nick Thomas7Istvan Bodi8Eleni C. Maratos9Skull Base Unit, Department of Neurosurgery, King's College Hospital NHS Foundation Trust, London, UKGKT School of Medical Education, King's College London, London, UK; Corresponding author.Skull Base Unit, Department of Neurosurgery, King's College Hospital NHS Foundation Trust, London, UKDepartment of Clinical Neuroradiology, King's College Hospital NHS Foundation Trust, London, UKDepartment of Histopathology, King's College Hospital NHS Foundation Trust, London, UKDepartment of Clinical Neuropathology, King's College Hospital NHS Foundation Trust, London, UKSkull Base Unit, Department of Neurosurgery, King's College Hospital NHS Foundation Trust, London, UKSkull Base Unit, Department of Neurosurgery, King's College Hospital NHS Foundation Trust, London, UKDepartment of Clinical Neuropathology, King's College Hospital NHS Foundation Trust, London, UKSkull Base Unit, Department of Neurosurgery, King's College Hospital NHS Foundation Trust, London, UKIntroduction: Craniopharyngiomas are benign tumours mainly confined to the cranial cavity in the suprasellar region. Research Question and Case Description: We present a rare case of an aggressive papillary craniopharyngioma with disseminated spinal intradural disease. A 67-year-old woman presented with a 4-month history of headache, visual disturbance, acute confusion and radicular leg pain. Previous history of breast carcinoma (ER + PR + HER2-) was noted. The importance of histological diagnosis prior to treatment of sellar or suprasellar lesions with atypical or aggressive features is explored. Materials and methods: MRI demonstrated a partly solid and partly cystic pituitary mass lesion in the sellar and suprasellar region with chiasmal compression and hypothalamic involvement. The sella was mildly enlarged and there were no calcifications. Whole neuraxis MRI revealed intradural deposits involving the ventricular system, spinal cord and conus. Within a month, the lesion rapidly increased in size. The patient underwent a craniotomy and transventricular resection of the sellar and suprasellar mass. Cranial lesion histology favoured papillary craniopharyngioma, confirmed by BRAF V600 mutation. Lumbar puncture CSF cytology confirmed craniopharyngioma with BRAF mutation and no evidence of metastatic breast cancer. Results: The patient remained confused postoperatively without focal neurological deficit and underwent palliative whole brain radiotherapy. She died 4 months later. A review of the literature identified 29 reports of ruptured craniopharyngioma. Discussion and Conclusion: Ruptured craniopharyngioma presents with a suprasellar mass and drop lesions in the spinal canal, characteristics radiologically indistinguishable from metastatic disease. The importance of histological diagnoses in directing the management of these cases is highlighted.http://www.sciencedirect.com/science/article/pii/S2772529422000625PapillaryMalignant craniopharyngiomaSpontaneous ruptureBrain tumourBRAF V600K mutationBreast carcinoma |
spellingShingle | Asfand Baig Mirza Melika Akhbari Christopher Murphy Steve Connor Mark R. Howard Zita Reisz Sinan Barazi Nick Thomas Istvan Bodi Eleni C. Maratos Spontaneous rupture of malignant papillary craniopharyngioma with CSF seeding and metastatic deposits: A case report and literature review Brain and Spine Papillary Malignant craniopharyngioma Spontaneous rupture Brain tumour BRAF V600K mutation Breast carcinoma |
title | Spontaneous rupture of malignant papillary craniopharyngioma with CSF seeding and metastatic deposits: A case report and literature review |
title_full | Spontaneous rupture of malignant papillary craniopharyngioma with CSF seeding and metastatic deposits: A case report and literature review |
title_fullStr | Spontaneous rupture of malignant papillary craniopharyngioma with CSF seeding and metastatic deposits: A case report and literature review |
title_full_unstemmed | Spontaneous rupture of malignant papillary craniopharyngioma with CSF seeding and metastatic deposits: A case report and literature review |
title_short | Spontaneous rupture of malignant papillary craniopharyngioma with CSF seeding and metastatic deposits: A case report and literature review |
title_sort | spontaneous rupture of malignant papillary craniopharyngioma with csf seeding and metastatic deposits a case report and literature review |
topic | Papillary Malignant craniopharyngioma Spontaneous rupture Brain tumour BRAF V600K mutation Breast carcinoma |
url | http://www.sciencedirect.com/science/article/pii/S2772529422000625 |
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