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Intravascular Papillary Endothelial Hyperplasia of the Pineal Region: A Case Report and Review of the Literature
Journal Pre-proof Intravascular Papillary Endothelial Hyperplasia of the Pineal Region: A Case Report and Review of the Literature Amber A. Retzlaff, MD, Karen Arispe, MD, Elizabeth J. Cochran, MD, Nathan T. Zwagerman, MD PII:
S1878-8750(19)32452-0
DOI:
https://doi.org/10.1016/j.wneu.2019.09.022
Reference:
WNEU 13321
To appear in:
World Neurosurgery
Received Date: 1 May 2019 Revised Date:
4 September 2019
Accepted Date: 5 September 2019
Please cite this article as: Retzlaff AA, Arispe K, Cochran EJ, Zwagerman NT, Intravascular Papillary Endothelial Hyperplasia of the Pineal Region: A Case Report and Review of the Literature, World Neurosurgery (2019), doi: https://doi.org/10.1016/j.wneu.2019.09.022. This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. © 2019 Elsevier Inc. All rights reserved.
Intravascular Papillary Endothelial Hyperplasia of the Pineal Region: A Case Report and Review of the Literature
Authors: Amber A. Retzlaff, MD, Department of Radiation Oncology Karen Arispe, MD, Department of Pathology Elizabeth J. Cochran, MD, Department of Pathology Nathan T. Zwagerman, MD, Department of Neurosurgery Institution: Departments of Neurosurgery, Pathlogy, and Radiation Oncology, Froedtert and the Medical College of Wisconsin, Milwaukee, WI
Abbreviations: IPEH = Intravascular papillary endothelial hyperplasia
MRI = magnetic resonance imaging FLAIR = fuid-atenuated inversion recovery CT = computed tomography GFAP = glial firillary acidic protein
Title: Intravascular Papillary Endothelial Hyperplasia of the Pineal Region: A Case Report and Review of the Literature Abstract: Intravascular papillary endothelial hyperplasia (IPEH) is a benign vascular lesion that is uncommon in the central nervous system. Reports of central nervous system involvement include intraparenchymal, sellar, cerebellar, brainstem, meningeal, venous sinus, calvarial, skull base, superior orbital fssure, and spinal cord/meningeal lesions. There has been only one previous report of occurrence in the pineal region. We report a second case and a review of the literature. Key Words: Masson’s tumor, intravascular papillary endothelial hyperplasia, IPEH, pineal, intracranial Introduction: Intravascular papillary endothelial hyperplasia (IPEH) is a benign vascular lesion believed to result from endothelial proliferaton in response to thrombus reorganizaton and accounts for approximately 2% of vascular tumors1-18, 23, 46. These lesions have been described in the skin of the head, neck, trunk, and fngers as well as in the subcutaneous tssues, nasal cavity, oral cavity, pharynx, larynx, digestve tract, and various solid organs; however, they are uncommon in the central nervous system 2, 4-10, 12-17, 19-29, 46. Reports of central nervous system involvement include intraparenchymal, sellar, cerebellar, brainstem, meningeal, venous sinus, calvarial, skull base, superior orbital fssure, and spinal cord/meningeal lesions 9-12, 17-19, 24-25, 46. There is also one report of occurrence within a posterior inferior cerebellar aneurysm 11 and one report of occurrence on the scalp with intracranial extension15. There has been only one report of occurrence in the pineal region thus far 5, 30-32. Case Report: A 28-year-old previously healthy woman presented to her primary care physician with one month of throbbing headaches and visual auras. She was otherwise asymptomatc and neurologically intact. She was initally treated with a course of steroids and oral pain medicaton without relief. A magnetc resonance imaging (MRI) scan of the brain demonstrated a 2.6 x 1.8 x 1.3 cm T1 hypointense, T2/FLAIR hyperintense pineal region mass that was overall non-enhancing aside from a punctate region of enhancement on the lef aspect of the lesion and a small posterior rim of enhancement (Figure 1). The mass was noted to compress and deform the collicular plate and the upper aspect of the midbrain resultng in obstructon of the cerebral aqueduct and noncommunicatng hydrocephalus of the third and lateral ventricles with transependymal cerebrospinal luid low. Her cerebrospinal luid and serum were negatve for both alpha-fetoprotein and beta human chorionic gonadotropin making pineal germinoma an unlikely etology. Other laboratory studies (cytology of the cerebrospinal luid, complete blood count, basic metabolic panel, and coagulaton studies) were unremarkable. Ophthalmologic evaluaton for papilledema was negatve. She underwent placement of a right extraventricular drain for management of hydrocephalus untl operatve management of the mass could be arranged. A pre-operatve head CT angiogram/venogram was negatve for vascular malformatons and demonstrated patent dural venous sinuses; the distal internal cerebral veins and the distal veins of Rosenthal were noted to drape over the pineal region lesion. Four days afer extraventricular drain placement, she underwent resecton of the mass via a lef supracerebellar infratentorial approach. The mass was noted to be cystc in nature with a 1
small red nodule on the lef aspect of the mass. Two samples were collected for permanent pathologic sectons (one from the cyst wall and one from the small red nodule). Histologic analysis of the cyst wall specimen revealed pineal gland tssue as well as anastomosing papillary networks lined by endothelial cells without evidence of cytologic atypia; fndings were consistent with intravascular papillary endothelial hyperplasia (Figure 2). Analysis of the red nodule again revealed anastomosing papillary networks as well as an organized thrombus. Immunohistochemistry with synaptophysin and glial fbrillary acidic protein (GFAP) antbodies was positve, supportng the presence of normal pineal gland components, and CD34 immunoreactvity demonstrated the presence of endothelial cells (Figure 3). Post-operatvely, the patent remained neurologically intact. A post-operatve brain MRI demonstrated complete resecton with expected post-operatve changes including pneumocephalus and reduced hydrocephalus (Figure 4). The extraventricular drain remained in place but clamped for two days and was then removed afer a head CT demonstrated decompression of the lateral and third ventricles. She was discharged home on post-operatve day four and evaluated in clinic two weeks later at which tme her headaches had signifcantly improved. Her 9-month follow up indicated no residual lesion and resoluton of her hydrocephalus. Discussion: Descriptio aod Cl assiic atio Intravascular papillary endothelial hyperplasia is a benign vascular lesion believed to result from endothelial proliferaton in response to thrombus reorganizaton 1-18, 23, 46. The reacton may represent an atempt at recanalizaton in the presence of vascular thrombus 33, 46. Many alternatve names have historically been used including reactve papillary endothelial hyperplasia, Masson’s tumor, Masson’s hemangioma, Masson’s pseudoangiosarcoma, Masson’s vegetant intravascular hemangioendothelioma, vegetant intravascular hemangioendothelioma, and intravascular angiomatosis 4, 6, 9, 12, 15, 17, 21, 27, 29, 46. Three types of IPEH have been described. Type 1 occurs within a normal vessel, whereas type 2 occurs in abnormal vasculature (hemangioma, cavernous angioma, lymphangioma, arteriovenous malformaton, pyogenic granuloma, vascular hamartoma, etc.). Extravascular IPEH lesions represent a third category that has been reported in associaton with organizing hematomas 1-4, 6-7, 9-10, 12-14, 18, 20-21, 23, 25, 27, 29, 34, 46. The incidence of type 1, type 2, and extravascular IPEH are believed to be approximately 56%, 40%, and 4%, respectvely 1, 7. Demigr aphics In studies related to extracranial sites, IPEH has been found to occur more frequently in females and those in the fourth decade of life. Intracranial lesions also occur predominantly in females, although there doesn’t seem to be a clear associaton with a partcular age group based on published case reports thus far. Female predominance led to the propositon that hormonal inluence may play a role in the pathogenesis of IPEH 4, 6-7, 9, 11, 13, 15-17, 24, 28-29, 46 . Preseot atio Presentaton is dependent on locaton of the intracranial mass and may include headache, seizure, increased intracranial pressure, hemiparesis, hemianopsia, and various cranial nerve palsies (diplopia, facial palsy, hearing loss, vertgo, and dysphagia)5, 7, 16-18, 22, 27, 46. Intracranial hypertension with associated papilledema may also develop as a consequence of sinus thrombosis in these patents 7, 17, 27-28, 32. In the case of pineal region lesions, upward gaze palsy, sleep disturbance, and obstructve hydrocephalus may also be present 34. In our 2
patent, obstructve hydrocephalus was initally managed with an extraventricular drain and resolved afer surgical resecton of the mass. Im agiog IPEH lesions ofen appear circumscribed with variable density on CT scans, hypo- to iso-intensity on T1 MRI, and iso- to hyper-intensity on T2 MRI; contrast enhancement is ofen present as well 1-2, 4-5, 9, 11, 13-15, 17-18, 24, 29, 35, 46. In additon, there may also be associated hemorrhage and underlying vascular lesions (cavernous angioma, venous angioma, arteriovenous malformaton) 10, 15-16, 27. Masses within or near bone may reveal lytc changes 10. Radiographic features are similar to those of other vascular lesions creatng a broad diferental based on imaging alone; tssue sampling is therefore imperatve for accurate diagnosis 1, 6, 14-16, 22, 35. P athiligy Upon hematoxylin and eosin staining, IPEH appears as a single layer of normal endothelial cells forming irregular anastomosing vascular channels with papillary projectons; there is ofen thrombotc material within the vascular lumen as well3, 5-7, 9-10, 12, 15-18, 25, 27, 29. Immunohistochemistry for CD31, CD 32, and CD34 is characteristcally positve1, 4-5, 12-14, 17-18, 20, 27. Immunohistochemistry for smooth muscle actn 1, 20, ferritn20, laminin18, vimentn9, 14, 18, 20, 27, and factor VIII1, 4-5, 10, 13-14, 17 are also usually positve, and Ki-67 index elevaton may be present as well3, 12, 17-18, 27. On a histologic basis, IPEH may share some features with malignant enttes including Kaposi sarcoma, angiosarcoma, and epithelioid hemangioendothelioma 10-12, 20, 25. IPEH is distnguished by the single layer of endothelial cells, the characteristc staining paterns described above, and the absence of prominent nucleoli, mitoses, and necrosis (although nuclear pleomorphism may be present) 1, 4-7, 9-10, 12-15, 17, 21-22, 2529, 35 . In our case, alpha fetoprotein and human chorionic gonadotropin were also utlized to rule out hormonesecretng germ cell tumors of the pineal region 36-37. Surgic al M ao agemeot Complete surgical resecton is considered the standard of care for management of IPEH 46. The pineal region has historically been considered a challenge to access 38-39. Current surgical approaches to the pineal gland include an infratentorial supracerebellar approach and a sub-occipital trans-tentorial approach 40-42. The supracerebellar approach is most commonly used and ofers a midline trajectory while avoiding morbidity associated with supratentorial approaches 42. The sub-occipital approach is performed less commonly but provides a shorter trajectory with enhanced visualizaton of the pineal gland, the third ventricle, and the thalamus43. Both approaches are generally performed in the sitng positon with the neck lexed to promote natural visualizaton of deep midline structures and to avoid prolonged retracton on adjacent structures 42-43. Endoscopic approaches may also be used for biopsy or resecton of pineal masses and for treatment of hydrocephalus41, 44. Pre-operatve embolizaton of vascular lesions may be utlized to assist with control of intra-operatve hemorrhage18, 21, 29. R adi atio Ther apy Radiaton therapy (external beam or stereotactc radiosurgery) may be considered if complete surgical resecton is not atainable. In the setng of subtotal resecton, radiaton may reduce the volume of residual disease or perhaps provide more durable local control even without tumor volume reducton 2, 11, 13-18, 23-24, 27, 29, 38, 41, 45-46 . Optmal dose-fractonaton shedules for radiaton in the adjuvant setng have not been determined; doses utlized thus far fall within the range of 45-54 Gy 9, 17, 21, 46. Interestngly, recent studies have proposed an associaton between central nervous system IPEH and prior cranial radiaton therapy. Proposed mechanisms for this include genetc aberratons in endothelial cells and alteratons in venous low afer radiaton treatment2, 5, 7, 12-13, 17, 23, 35, 45. Risk of secondary malignancy with radiaton therapy must be weighed against risk of local progression, partcularly in the intracranial setng. Prigoisis 3
The overall prognosis for IPEH is reported to be quite positve. Surgical excision provides curatve treatment in the short term, although the long-term behavior of these lesions is not clearly defned 2-5, 7, 9, 12, 14-18, 24, 26-27, 29, 35. In challenging locatons, partal resecton relieves mass efect but is associated with recurrence up to nine years afer treatment1-2, 5, 9-13, 16, 21, 29, 35, 45. Long-term survival is stll achievable with subtotal resecton, although local progression can result in varying degrees of neurologic consequence 4, 12, 16. For that reason, further study with regard to adjuvant treatment optons is warranted. Conclusion: Our case represents the second reported case of IPEH in the pineal region. Intracranial IPEH is rare, and occurrence in the pineal region is exceedingly rare based on current reports. Our case is unique in that the patent presented with obstructve hydrocephalus that was managed pre-operatvely with an extraventricular drain; surgical resecton of the mass provided relief of pre-operatve symptoms as well as defnitve management of the hydrocephalus. Although rare, IPEH is an entty that should be considered in the diferental diagnosis for intracranial masses with radiographic features characteristc of vascular lesions. Tissue sampling is imperatve for distncton from more malignant lesions and will ultmately guide the appropriate course of treatment. Complete resecton is curatve and is the standard of care when feasible. Given the risk of local progression and neurologic compromise with subtotal reseton, further study with regard to adjuvant treatment optons is warranted. References: 1. Anez, E. M., et al. "Massons Tumor in the Maxillary Sinus." J Otil aryogil ENT Res 4.5 (2016): 00115. 2. Barrit, A. W., Merve, A., Epaliyanage, P., Aram, J. (2017). Intracranial papillary endothelial hyperplasia (Masson’s tumour) following gamma knife radiosurgery for temporal lobe epilepsy. Pr actc al Neuriligy, 17(3), 214-217. 3. Bologna-Molina, R., Amezcua-Rosas, G., Guardado-Luevanos, I., Mendoza-Roaf, P. L., GonzálezMontemayor, T., Molina-Frechero, N. (2010). Intravascular Papillary Endothelial Hyperplasia (Masson’s Tumor) of the Mouth–A Case Report. C ase repirts io derm atiligy, 2(1), 22-26. 4. Çagli, S., Oktar, N., Dalbast T., Islekel, S., Demirtas, E., Özdamar, N. (2004). Intravascular papillary endothelial hyperplasia of the central nervous system. Neuriligi a medici-chirurgic a, 44(6), 302-310. 5. Charalambous, L. T., Penumaka, A., Komisarow, J. M., Hemmerich, A. C., Cummings, T. J., Codd, P. J., & Friedman, A. H. (2018). Masson’s tumor of the pineal region: case report. Journal of neurosurgery, 128(6), 1725-1730.
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32. Motolese, C., Szathmari, A., Beuriat, P. A. (2015). Incidence of pineal tumours. A review of the literature. Neurichirurgie, 61(2), 65-69. 33. Levere, S. M., Barsky, S. H., Meals, R. A. (1994). Intravascular papillary endothelial hyperplasia: a neoplastc “actor” representng an exaggerated atempt at recanalizaton mediated by basic fbroblast growth factor. The Jiuro al if h aod surgery, 19(4), 559-564. 34. Rousselle, C., des Portes, V., Berlier, P., Motolese, C. (2015). Pineal region tumors: clinical symptoms and syndromes. Neurichirurgie, 61(2), 106-112. 35. Ong, S. S., Bruner, J., Schellingerhout, D., Puduvalli, V. K. (2011). Papillary endothelial hyperplasia presentng as recurrent malignant glioma. Jiuro al if oeuri-iociligy, 102(3), 491-498. 36. Faure-Conter, C. (2015). Tumoral markers in tumors of the pineal region. Neurichirurgie, 61(2), 143145. 37. Frappaz, D., Conter, C. F., Szathmari, A., Valsijevic, A., Motolese, C. (2015). The management of pineal tumors as a model for a multdisciplinary approach in neuro-oncology. Neurichirurgie, 61(2), 208-211. 38. Balossier, A., Blond, S., Touzet, G., Sarrazin, T., Lartgau, E., Reyns, N. (2015). Role of radiosurgery in the management of pineal region tumours: indicatons, method, outcome. Neurichirurgie, 61(2), 216222. 39. Choudhry, O., Gupta, G., Prestgiacomo, C. J. (2011). On the surgery of the seat of the soul: The pineal gland and the history of its surgical approaches. Neurisurgery Clioics if Nirth Americ a, 22(3), 321-333. 40. MBaye, M., Jouanneau, E., Motolese, C., Simon, E. (2015). Alternatves approaches to the suboccipital transtentorial route for pineal tumors: How and when I do it?. Neurichirurgie, 61(2), 184-192. 41. Motolese, C., Szathmari, A. (2015). History of the pineal region tumor. Neurichirurgie, 61(2), 61-64. 42. Motolese, C., Szathmari, A., Ricci-Franchi, A. C., Gallo, P., Beuriat, P. A., Capone, G. (2015). Supracerebellar infratentorial approach for pineal region tumors: Our surgical and technical consideratons. Neurichirurgie, 61(2), 176-183. 43. Motolese, C., Szathmari, A., Ricci-Franchi, A. C., Beuriat, P. A., Grassiot, B. (2015). The sub-occipital transtentorial approach revisited base on our own experience. Neurichirurgie, 61(2), 168-175. 44. Motolese, C., Szathamari, A., Beuriat, P. A., Grassiot, B., Simon, E. (2015). Neuroendoscopy and pineal tumors: a review of the literature and our consideratons regarding its utlity. Neurichirurgie, 61(2), 155-159. 45. Crocker, M., Epaliyanage, P., Bodi, I., Deasy, N., Selway, R. (2007). Massonss tumour in the right parietal lobe afer stereotactc radiosurgery for cerebellar AVM: case report and review. Clioic al oeuriligy aod oeurisurgery, 109(9), 811-815. 46. Prat, G. P., Jimenez, M. S., Caro, P. C., Ruiz-Valdepeñas, E. C., Garvia, M. R., Rivas, F. J. M. (2018). Staged Management of Intracranial Masson Tumor: An Unexpected Gauntlet: Case Report and Review of the Literature. Wirld oeurisurgery, 114, 194-203.
6
S1878-8750(19)32452-0
DOI:
https://doi.org/10.1016/j.wneu.2019.09.022
Reference:
WNEU 13321
To appear in:
World Neurosurgery
Received Date: 1 May 2019 Revised Date:
4 September 2019
Accepted Date: 5 September 2019
Please cite this article as: Retzlaff AA, Arispe K, Cochran EJ, Zwagerman NT, Intravascular Papillary Endothelial Hyperplasia of the Pineal Region: A Case Report and Review of the Literature, World Neurosurgery (2019), doi: https://doi.org/10.1016/j.wneu.2019.09.022. This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. © 2019 Elsevier Inc. All rights reserved.
Intravascular Papillary Endothelial Hyperplasia of the Pineal Region: A Case Report and Review of the Literature
Authors: Amber A. Retzlaff, MD, Department of Radiation Oncology Karen Arispe, MD, Department of Pathology Elizabeth J. Cochran, MD, Department of Pathology Nathan T. Zwagerman, MD, Department of Neurosurgery Institution: Departments of Neurosurgery, Pathlogy, and Radiation Oncology, Froedtert and the Medical College of Wisconsin, Milwaukee, WI
Abbreviations: IPEH = Intravascular papillary endothelial hyperplasia
MRI = magnetic resonance imaging FLAIR = fuid-atenuated inversion recovery CT = computed tomography GFAP = glial firillary acidic protein
Title: Intravascular Papillary Endothelial Hyperplasia of the Pineal Region: A Case Report and Review of the Literature Abstract: Intravascular papillary endothelial hyperplasia (IPEH) is a benign vascular lesion that is uncommon in the central nervous system. Reports of central nervous system involvement include intraparenchymal, sellar, cerebellar, brainstem, meningeal, venous sinus, calvarial, skull base, superior orbital fssure, and spinal cord/meningeal lesions. There has been only one previous report of occurrence in the pineal region. We report a second case and a review of the literature. Key Words: Masson’s tumor, intravascular papillary endothelial hyperplasia, IPEH, pineal, intracranial Introduction: Intravascular papillary endothelial hyperplasia (IPEH) is a benign vascular lesion believed to result from endothelial proliferaton in response to thrombus reorganizaton and accounts for approximately 2% of vascular tumors1-18, 23, 46. These lesions have been described in the skin of the head, neck, trunk, and fngers as well as in the subcutaneous tssues, nasal cavity, oral cavity, pharynx, larynx, digestve tract, and various solid organs; however, they are uncommon in the central nervous system 2, 4-10, 12-17, 19-29, 46. Reports of central nervous system involvement include intraparenchymal, sellar, cerebellar, brainstem, meningeal, venous sinus, calvarial, skull base, superior orbital fssure, and spinal cord/meningeal lesions 9-12, 17-19, 24-25, 46. There is also one report of occurrence within a posterior inferior cerebellar aneurysm 11 and one report of occurrence on the scalp with intracranial extension15. There has been only one report of occurrence in the pineal region thus far 5, 30-32. Case Report: A 28-year-old previously healthy woman presented to her primary care physician with one month of throbbing headaches and visual auras. She was otherwise asymptomatc and neurologically intact. She was initally treated with a course of steroids and oral pain medicaton without relief. A magnetc resonance imaging (MRI) scan of the brain demonstrated a 2.6 x 1.8 x 1.3 cm T1 hypointense, T2/FLAIR hyperintense pineal region mass that was overall non-enhancing aside from a punctate region of enhancement on the lef aspect of the lesion and a small posterior rim of enhancement (Figure 1). The mass was noted to compress and deform the collicular plate and the upper aspect of the midbrain resultng in obstructon of the cerebral aqueduct and noncommunicatng hydrocephalus of the third and lateral ventricles with transependymal cerebrospinal luid low. Her cerebrospinal luid and serum were negatve for both alpha-fetoprotein and beta human chorionic gonadotropin making pineal germinoma an unlikely etology. Other laboratory studies (cytology of the cerebrospinal luid, complete blood count, basic metabolic panel, and coagulaton studies) were unremarkable. Ophthalmologic evaluaton for papilledema was negatve. She underwent placement of a right extraventricular drain for management of hydrocephalus untl operatve management of the mass could be arranged. A pre-operatve head CT angiogram/venogram was negatve for vascular malformatons and demonstrated patent dural venous sinuses; the distal internal cerebral veins and the distal veins of Rosenthal were noted to drape over the pineal region lesion. Four days afer extraventricular drain placement, she underwent resecton of the mass via a lef supracerebellar infratentorial approach. The mass was noted to be cystc in nature with a 1
small red nodule on the lef aspect of the mass. Two samples were collected for permanent pathologic sectons (one from the cyst wall and one from the small red nodule). Histologic analysis of the cyst wall specimen revealed pineal gland tssue as well as anastomosing papillary networks lined by endothelial cells without evidence of cytologic atypia; fndings were consistent with intravascular papillary endothelial hyperplasia (Figure 2). Analysis of the red nodule again revealed anastomosing papillary networks as well as an organized thrombus. Immunohistochemistry with synaptophysin and glial fbrillary acidic protein (GFAP) antbodies was positve, supportng the presence of normal pineal gland components, and CD34 immunoreactvity demonstrated the presence of endothelial cells (Figure 3). Post-operatvely, the patent remained neurologically intact. A post-operatve brain MRI demonstrated complete resecton with expected post-operatve changes including pneumocephalus and reduced hydrocephalus (Figure 4). The extraventricular drain remained in place but clamped for two days and was then removed afer a head CT demonstrated decompression of the lateral and third ventricles. She was discharged home on post-operatve day four and evaluated in clinic two weeks later at which tme her headaches had signifcantly improved. Her 9-month follow up indicated no residual lesion and resoluton of her hydrocephalus. Discussion: Descriptio aod Cl assiic atio Intravascular papillary endothelial hyperplasia is a benign vascular lesion believed to result from endothelial proliferaton in response to thrombus reorganizaton 1-18, 23, 46. The reacton may represent an atempt at recanalizaton in the presence of vascular thrombus 33, 46. Many alternatve names have historically been used including reactve papillary endothelial hyperplasia, Masson’s tumor, Masson’s hemangioma, Masson’s pseudoangiosarcoma, Masson’s vegetant intravascular hemangioendothelioma, vegetant intravascular hemangioendothelioma, and intravascular angiomatosis 4, 6, 9, 12, 15, 17, 21, 27, 29, 46. Three types of IPEH have been described. Type 1 occurs within a normal vessel, whereas type 2 occurs in abnormal vasculature (hemangioma, cavernous angioma, lymphangioma, arteriovenous malformaton, pyogenic granuloma, vascular hamartoma, etc.). Extravascular IPEH lesions represent a third category that has been reported in associaton with organizing hematomas 1-4, 6-7, 9-10, 12-14, 18, 20-21, 23, 25, 27, 29, 34, 46. The incidence of type 1, type 2, and extravascular IPEH are believed to be approximately 56%, 40%, and 4%, respectvely 1, 7. Demigr aphics In studies related to extracranial sites, IPEH has been found to occur more frequently in females and those in the fourth decade of life. Intracranial lesions also occur predominantly in females, although there doesn’t seem to be a clear associaton with a partcular age group based on published case reports thus far. Female predominance led to the propositon that hormonal inluence may play a role in the pathogenesis of IPEH 4, 6-7, 9, 11, 13, 15-17, 24, 28-29, 46 . Preseot atio Presentaton is dependent on locaton of the intracranial mass and may include headache, seizure, increased intracranial pressure, hemiparesis, hemianopsia, and various cranial nerve palsies (diplopia, facial palsy, hearing loss, vertgo, and dysphagia)5, 7, 16-18, 22, 27, 46. Intracranial hypertension with associated papilledema may also develop as a consequence of sinus thrombosis in these patents 7, 17, 27-28, 32. In the case of pineal region lesions, upward gaze palsy, sleep disturbance, and obstructve hydrocephalus may also be present 34. In our 2
patent, obstructve hydrocephalus was initally managed with an extraventricular drain and resolved afer surgical resecton of the mass. Im agiog IPEH lesions ofen appear circumscribed with variable density on CT scans, hypo- to iso-intensity on T1 MRI, and iso- to hyper-intensity on T2 MRI; contrast enhancement is ofen present as well 1-2, 4-5, 9, 11, 13-15, 17-18, 24, 29, 35, 46. In additon, there may also be associated hemorrhage and underlying vascular lesions (cavernous angioma, venous angioma, arteriovenous malformaton) 10, 15-16, 27. Masses within or near bone may reveal lytc changes 10. Radiographic features are similar to those of other vascular lesions creatng a broad diferental based on imaging alone; tssue sampling is therefore imperatve for accurate diagnosis 1, 6, 14-16, 22, 35. P athiligy Upon hematoxylin and eosin staining, IPEH appears as a single layer of normal endothelial cells forming irregular anastomosing vascular channels with papillary projectons; there is ofen thrombotc material within the vascular lumen as well3, 5-7, 9-10, 12, 15-18, 25, 27, 29. Immunohistochemistry for CD31, CD 32, and CD34 is characteristcally positve1, 4-5, 12-14, 17-18, 20, 27. Immunohistochemistry for smooth muscle actn 1, 20, ferritn20, laminin18, vimentn9, 14, 18, 20, 27, and factor VIII1, 4-5, 10, 13-14, 17 are also usually positve, and Ki-67 index elevaton may be present as well3, 12, 17-18, 27. On a histologic basis, IPEH may share some features with malignant enttes including Kaposi sarcoma, angiosarcoma, and epithelioid hemangioendothelioma 10-12, 20, 25. IPEH is distnguished by the single layer of endothelial cells, the characteristc staining paterns described above, and the absence of prominent nucleoli, mitoses, and necrosis (although nuclear pleomorphism may be present) 1, 4-7, 9-10, 12-15, 17, 21-22, 2529, 35 . In our case, alpha fetoprotein and human chorionic gonadotropin were also utlized to rule out hormonesecretng germ cell tumors of the pineal region 36-37. Surgic al M ao agemeot Complete surgical resecton is considered the standard of care for management of IPEH 46. The pineal region has historically been considered a challenge to access 38-39. Current surgical approaches to the pineal gland include an infratentorial supracerebellar approach and a sub-occipital trans-tentorial approach 40-42. The supracerebellar approach is most commonly used and ofers a midline trajectory while avoiding morbidity associated with supratentorial approaches 42. The sub-occipital approach is performed less commonly but provides a shorter trajectory with enhanced visualizaton of the pineal gland, the third ventricle, and the thalamus43. Both approaches are generally performed in the sitng positon with the neck lexed to promote natural visualizaton of deep midline structures and to avoid prolonged retracton on adjacent structures 42-43. Endoscopic approaches may also be used for biopsy or resecton of pineal masses and for treatment of hydrocephalus41, 44. Pre-operatve embolizaton of vascular lesions may be utlized to assist with control of intra-operatve hemorrhage18, 21, 29. R adi atio Ther apy Radiaton therapy (external beam or stereotactc radiosurgery) may be considered if complete surgical resecton is not atainable. In the setng of subtotal resecton, radiaton may reduce the volume of residual disease or perhaps provide more durable local control even without tumor volume reducton 2, 11, 13-18, 23-24, 27, 29, 38, 41, 45-46 . Optmal dose-fractonaton shedules for radiaton in the adjuvant setng have not been determined; doses utlized thus far fall within the range of 45-54 Gy 9, 17, 21, 46. Interestngly, recent studies have proposed an associaton between central nervous system IPEH and prior cranial radiaton therapy. Proposed mechanisms for this include genetc aberratons in endothelial cells and alteratons in venous low afer radiaton treatment2, 5, 7, 12-13, 17, 23, 35, 45. Risk of secondary malignancy with radiaton therapy must be weighed against risk of local progression, partcularly in the intracranial setng. Prigoisis 3
The overall prognosis for IPEH is reported to be quite positve. Surgical excision provides curatve treatment in the short term, although the long-term behavior of these lesions is not clearly defned 2-5, 7, 9, 12, 14-18, 24, 26-27, 29, 35. In challenging locatons, partal resecton relieves mass efect but is associated with recurrence up to nine years afer treatment1-2, 5, 9-13, 16, 21, 29, 35, 45. Long-term survival is stll achievable with subtotal resecton, although local progression can result in varying degrees of neurologic consequence 4, 12, 16. For that reason, further study with regard to adjuvant treatment optons is warranted. Conclusion: Our case represents the second reported case of IPEH in the pineal region. Intracranial IPEH is rare, and occurrence in the pineal region is exceedingly rare based on current reports. Our case is unique in that the patent presented with obstructve hydrocephalus that was managed pre-operatvely with an extraventricular drain; surgical resecton of the mass provided relief of pre-operatve symptoms as well as defnitve management of the hydrocephalus. Although rare, IPEH is an entty that should be considered in the diferental diagnosis for intracranial masses with radiographic features characteristc of vascular lesions. Tissue sampling is imperatve for distncton from more malignant lesions and will ultmately guide the appropriate course of treatment. Complete resecton is curatve and is the standard of care when feasible. Given the risk of local progression and neurologic compromise with subtotal reseton, further study with regard to adjuvant treatment optons is warranted. References: 1. Anez, E. M., et al. "Massons Tumor in the Maxillary Sinus." J Otil aryogil ENT Res 4.5 (2016): 00115. 2. Barrit, A. W., Merve, A., Epaliyanage, P., Aram, J. (2017). Intracranial papillary endothelial hyperplasia (Masson’s tumour) following gamma knife radiosurgery for temporal lobe epilepsy. Pr actc al Neuriligy, 17(3), 214-217. 3. Bologna-Molina, R., Amezcua-Rosas, G., Guardado-Luevanos, I., Mendoza-Roaf, P. L., GonzálezMontemayor, T., Molina-Frechero, N. (2010). Intravascular Papillary Endothelial Hyperplasia (Masson’s Tumor) of the Mouth–A Case Report. C ase repirts io derm atiligy, 2(1), 22-26. 4. Çagli, S., Oktar, N., Dalbast T., Islekel, S., Demirtas, E., Özdamar, N. (2004). Intravascular papillary endothelial hyperplasia of the central nervous system. Neuriligi a medici-chirurgic a, 44(6), 302-310. 5. Charalambous, L. T., Penumaka, A., Komisarow, J. M., Hemmerich, A. C., Cummings, T. J., Codd, P. J., & Friedman, A. H. (2018). Masson’s tumor of the pineal region: case report. Journal of neurosurgery, 128(6), 1725-1730.
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