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A Primary Pigmented Choroid Plexus Papilloma Located Within the Sella Turcica: Case Report and Literature Review
Accepted Manuscript A primary pigmented choroid plexus papilloma located within the sella turcica: case report and literature review Xuan Gong, MD, PhD, Chao Liu, MD, PhD, Longbo Zhang, MD, PhD, Zhenyan Li, MD, PhD, Christopher M. Bartley, MD, PhD, Zhixiong Liu, MD, PhD PII:
S1878-8750(17)31052-5
DOI:
10.1016/j.wneu.2017.06.155
Reference:
WNEU 6026
To appear in:
World Neurosurgery
Received Date: 26 January 2017 Revised Date:
22 June 2017
Accepted Date: 24 June 2017
Please cite this article as: Gong X, Liu C, Zhang L, Li Z, Bartley CM, Liu Z, A primary pigmented choroid plexus papilloma located within the sella turcica: case report and literature review, World Neurosurgery (2017), doi: 10.1016/j.wneu.2017.06.155. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. 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.
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Title: A primary pigmented choroid plexus papilloma located within the sella turcica: case report and literature review
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Authors: Xuan Gong, MD, PhD; Chao Liu, MD, PhD; Longbo Zhang, MD, PhD; Zhenyan Li, MD, PhD; Christopher M Bartley, MD, PhD; Zhixiong Liu, MD, PhD Xuan Gong, Xiangya Hospital, Central South University, Department of Neurosurgery, 87 Xiangya Rd, Changsha, Hunan, P.R.China, 410008
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Chao Liu, Xiangya Hospital, Central South University, Department of Oncology, 87 Xiangya Rd, Changsha, Hunan, P.R.China, 410008
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Longbo Zhang, Xiangya Hospital, Central South University, Department of Neurosurgery, 87 Xiangya Rd, Changsha, Hunan, P.R.China, 410008 Zhenyan Li, Xiangya Hospital, Central South University, Department of Neurosurgery, 87 Xiangya Rd, Changsha, Hunan, P.R.China, 410008
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Christopher M. Bartley, University of California, San Francisco, Department of Psychiatry, 401 Parnassus Avenue, San Francisco, CA, USA, 94143-0984
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Corresponding Author: Zhixiong, Liu, M.D. PhD. Department of Neurosurgery, Xiangya Hospital, Central South University 87 Xiangya Rd, Changsha, Hunan, P.R.China, 410008 Phone: +8673189753537 Fax: +8673189753537 [email protected] Xuan Gong: [email protected] Chao Liu: [email protected] Longbo Zhang: [email protected] Zhenyan Li: [email protected] Christopher M Bartley: [email protected]
Key Words: Choroid Plexus Papilloma, pituitary, sella turcica, melanotic, pigmented
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Abstract: Background: Choroid plexus papillomas (CPPs) are rare benign tumors and the
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pigmented subtype is even more rarely observed.
Case Description: We present the case of a 43-year-old woman with complaints of
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headache and progressive left monocular visual deterioration, whose initial plain CT
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showed an ovate high density tumor located within in sellar region. MRI revealed a homogeneously contrast-enhancing tumor extending from the sella turcica to the suprasellar cistern. Single-nostril transsphenoidal endoscopic resection followed by subfrontal subtotal resection was performed in this patient. Postoperative histology
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demonstrated that the tumor consisted of hyperchromatic tissue with papillary features. Higher resolution examination of the tissue revealed this tissue was comprised of hyperplastic columnar epithelial cells with hyperchromatic cytoplasmic pigment.
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Subsequent immunohistochemistry identified the lesion as a pigmented choroid plexus
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papilloma. Herein we review the current literature, discuss the origin of the tumor, the differential of diagnosis, as well as the role of surgery and radiotherapy.
Conclusion: Our study provides important clinical information for the evaluation, diagnosis, and treatment of Pigmented CPP in sellar region.
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Background: Choroid plexus papillomas (CPPs) represent approximately 1% of
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intracranial tumors1. They are most frequently found in the lateral ventricles in infants and children1, 2 and in the fourth ventricles in adults3. Less commonly they are found in the third ventricle4 and cerebellopontine angles5, 6 and in rare instances, they can occur in
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the suprasellar region7, sacral canal8, posterior fossa9, brain stem10, or sella turcica11-13. The pigmented subtype of CPPs is rare and was only first reported in 1987 in Japan14,
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however subsequent cases have been reported15, 16. Upon literature review we failed to find a report of a melanin-containing CPP located within the sella turcica. To our knowledge, we present the first case report of a primary pigmented choroid plexus
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papilloma in the sella turcica. The clinical presentation of the tumor was similar to a pituitary adenoma however the diagnosis of CPP was definitively made by postoperative pathology, histological examination, and subsequent immunohistochemical analysis. The
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differential diagnosis and role of surgery and radiotherapy are herein discussed.
Case Description: A 43-year–old woman presented to our hospital with five months of intermit headaches and two months of progressive left monocular visual deterioration. She denied irregular menstruation, lactation, or motor/sensor deficits. On formal ophthalmologic examination she was found to have impaired visual acuity and bitemporal hemianopsia.
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Endocrine studies revealed a mildly decreased free thyroxine 4 level, and a moderately
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elevated prolactin level. Other pituitary hormones were within normal limits.
Initial x-ray computed tomography (CT) imaging revealed an elliptic high-density tumor
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located in the sellar region however, high resolution CT did not find evidence of
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calcification. Magnetic Resonance imaging (MRI) revealed a large and homogenous sellar mass occupying the entire sella turcica and extending into the suprasellar cistern that was hyperintense and hypointense on T1- and T2-weighted imaging respectively (Fig. 1A-B). No enhancement was observed after intravenous injection of contrast (Fig. 1C-D).
ventricles or cisterns.
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The pituitary stalk could not be clearly visualized and no abnormalities were found in the
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Based on the patient’s symptoms, hormone alterations, and radiologic findings, a
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preliminary preoperative diagnosis of pituitary adenoma was considered and single-nostril transsphenoidal endoscopic surgery was performed. Intraoperatively the tumor was found to be black and cauliflower-like in shape (Fig. 3F). Intraoperatively the tough texture and abundant blood flow prevented total resection of the tumor therefore a subtotal resection was performed (Fig. 2). Postoperatively, the patient’s vision in her left eye improved.
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Postoperative histology revealed that some of the tissue exhibited a papillary architecture, overlying a fibrovascular stroma covered by a layer of hyperplastic columnar epithelial
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cells. The cytoplasm of these hyperplastic columnar epithelial cells was hyperchromatic and the nuclei were moderately heteromorphic and infrequently mitotic (Fig. 3A-B. Immunohistochemistry indicated that the neoplastic cells were positive for melanocyte
for
GFAP
(Fig.
3E),
melanocyte
cytokeratin
marker
CK-L
and
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negative
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markers S-100 (Fig. 3C), Melan-A (Fig. 3D), and HMB-45 (data not shown), but
adenohypophysical elements including FSH, HGH, ACTH, PRL, TSH and LH (data not shown). Based on image data, histology, and immunohistochemical analysis, a diagnosis of suprasellar pigmented choroid plexus papilloma was made.
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Three months after single-nostril transsphenoidal subtotal resection of the mass, the patient represented with a complaint of worsening vision in her left eye and an MRI
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revealed interval enlargement of the residual tumor (Figure 4 A-B). In order to salvage her vision, a second surgery was performed utilizing a subfrontal approach. Because the
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tumor was tough and tightly adhered to the surrounding vital structures, we opted in favor of a partial resection to avoid catastrophic neurovascular injury (Figure 4 C-D). The surgery was followed by adjunctive radiotherapy and the patient has been asymptomatic the two years.
Discussion: A choroid plexus papilloma was first described by Guerarard in 1832 in a
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case of a 3-year old child. CPPs are usually derived from normal choroid plexus and are most often found in the fourth ventricle, lateral ventricle, and in the third ventricle owing
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to the normal anatomic location of the choroid plexus. Occasionally, exophytic extension of normal choroid tufts into or through the foramens of Luschka and Magendie result in CPPs located in the cerebellopontine angle17 or foramen magnum18. In rare cases, ectopic
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choroid plexus or metaplastic transformation of ependymal results in extraventricular
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primary CPPs such as in brain stem, posterior fossa, sacral canal, or suprasellar or intrasellar region.
We are aware of four prior cases of CPPs originating from the sellar region 11-13, 19 and all
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were female between the ages of 31-51 years old. As in our case, there was no evidence that the CPP was contiguous with ventricular choroid plexus or that they were products of metastasis. As such, these cases likely originated from ectopic choroid plexus or
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metaplastic transformation.
A number of cases of extrasellar pigmented CPPs have been reported, including: a 14-year-old white male with pigmented CPP in the lateral ventricle15; a case of 35-year-old man with a pigmented CPP in the fourth ventricle16; and a 45-year-old man with a pigmented CPP in the left lateral ventricle14. To our knowledge, our case of a 43-year-old woman is the only reported case of a primary pigmented CPP located outside of the ventricles.
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Several other primary pigmented neoplasms are known to occur within the region of the 21
, both of which symptomatically
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sella tucica including melanoma and melanocytoma20
mimic pituitary adenoma. Other, rarer sellar pigmented tumors have been reported including a teratoma with melanotic progonoma22. One case of pigmented papillary
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epithelial neoplasm arising within the pituitary fossa has been reported23, however
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subsequent follow-up found that this patient’s tumor later developed into a carcinosarcoma and as such does not belong to a known tumor type.
The differential diagnosis for pigmented CPPs includes papillary ependymoma, papillary
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pituitary adenoma, and metastatic melanoma. Papillary ependymomas, (which occasionally occur with pigmentation)24,
25
, are typically densely cellular with
perivascular rosettes and pseudorosettes26. The core of papillary ependymomal papillary
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structures are composed of the neuroglial stroma rather than the fibrovascular stroma
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seen in CPPs27. They also exhibit characteristic cilia and a tubular structure. Immunohistochemically, the neoplastic cells of ependymomas are often positive for GFAP but negative for cytokeratin, but there are exceptions27. In pituitary adenomas, anterior pituitary hormones are often reactive but S-100 is not, and a pigmented pituitary adenoma has not yet been reported. Metastatic melanomas can display papillary structures (such as thyroid papillary carcinoma), however they typically exhibit characteristics of malignant neoplasm such as a high mitotic index, nuclear pleomophism
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and necrosis 28. CPPs are often immunopositive for CK7 however a previous study found the approximately 25% of definitively diagnosed CPPs are negative for this marker and
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the expression profile of pigmented CPPs has not been well defined29.
The pigmented CPPs generally owe their color to the expression of melanin or
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neuromelanin and are radiologically distinct from conventional CPPs15. CPPs are
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generally isointense on T1 and T2 however their T2 signature is more variable. Melanomas and some other melanotic tumors present hyperintense on T1-weighted images and hypointense on T2-weighted images30. This characteristic MR signature of melanin-containing lesions is sometimes referred to as “melanin scavenging” and is
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thought to be due to melanin binding of paramagnetic metals31. Consistent with this melanin signature, the tumor in our case demonstrate high signal on T1-weighted and low signal on T2-weighted images. However, MRI imaging of CNS melanotic lesions often
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also suggests haemorrhagic or proteinaceous content32. Moreover, the histological
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architecture of our tumor is highly inconsistent with melanoma making this diagnosis unlikely.
Total microsurgical extirpation of the CPPs is the recommended treatment and adjunctive radiotherapy is typically reserved for choroid plexus carcinomas33. For sellar melanocytic tumors, transsphenoidal surgery is recommended with fractionated radiotherapy if total resection is not feasible34. In our case, the tumor was first partially resected via an
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endonasal trans-sphenoidal followed by a trans-cranial approach. Owing to the abundant blood flow, indistinct border, and adhesions to surrounding tissue, the surgery was limited
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to a subtotal resection35, 36.
The role of radiotherapy in the treatment of CPPs is uncertain and this probably reflects
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the rarity of these tumors, in particular those associated with recurrent or metastatic disease after total and subtotal resection37, however in primary sellar melanocytic tumor,
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radiotherapy is recommended34. In our case, the tumor progressed following subtotal resection, so postoperative adjunctive radiotherapy was employed with complete
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resolution of symptoms to date.
Conclusion: To our knowledge, this is the first sellar pigmented choroid plexus papilloma that has been reported in the literature. Our study provides important clinical
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information for the diagnosis and management of this rare disease entity.
The authors declare that they have no conflict of interest.
References:
1.
Laurence KM, Hoare RD, Till K. The diagnosis of the choroid plexus papilloma of the lateral
ventricle. Brain : a journal of neurology 1961;84:628-641.
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2.
Tominaga A, Fujioka Y, Monden S, Kawamoto Y, Uozumi T, Konishi N. [Choroid plexus papilloma of
the third ventricle in infancy: a case report]. No shinkei geka Neurological surgery 1992;20:1273-1276. 3.
Wolff JE, Sajedi M, Brant R, Coppes MJ, Egeler RM. Choroid plexus tumours. British journal of
cancer 2002;87:1086-1091. Fortuna A, Celli P, Ferrante L, Turano C. A review of papillomas of the third ventricle. One case
report. Journal of neurosurgical sciences 1979;23:61-76. 5.
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4.
Talacchi A, De Micheli E, Lombardo C, Turazzi S, Bricolo A. Choroid plexus papilloma of the
cerebellopontine angle: a twelve patient series. Surgical neurology 1999;51:621-629. 6.
Kieserman S, Linstrom C, McCormick S, Petschenik AJ. Choroid plexus papilloma of the
cerebellopontine angle. The American journal of otology 1996;17:119-122. 7.
Keskin F, Erdi F, Kaya B, Toy H. Sellar-Suprasellar Extraventricular Choroid Plexus Papilloma : A
8.
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Case Report and Review of the Literature. J Korean Neurosurg Soc 2016;59:58-61.
Kurtkaya-Yapicier O, Scheithauer BW, Van Peteghem KP, Sawicki JE. Unusual case of extradural
choroid plexus papilloma of the sacral canal. Case report. Journal of neurosurgery 2002;97:102-105. Garcia-Valtuille R, Abascal F, Garcia-Valtuille AI, et al. Adult choroid plexus papilloma of the
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9.
posterior fossa mimicking a hemangioblastoma. Case report. Journal of neurosurgery 2000;92:870-872. 10. Pillai A, Rajeev K, Chandi S, Unnikrishnan M. Intrinsic brainstem choroid plexus papilloma. Case report. Journal of neurosurgery 2004;100:1076-1078.
11. Bian LG, Sun QF, Wu HC, Jiang H, Sun YH, Shen JK. Primary choroid plexus papilloma in the pituitary fossa: case report and literature review. Acta neurochirurgica 2011;153:851-857. 12. Ma YH, Ye K, Zhan RY, Wang LJ. Primary choroid plexus papilloma of the sellar region. Journal of neuro-oncology 2008;88:51-55.
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13. Sameshima T, Tanikawa R, Sugimura T, et al. Choroid plexus papilloma originating in the sella turcica--case report. Neurologia medico-chirurgica 2010;50:144-146. 14. Yamazaki S, Ito U, Tomita H, Takada Y, Inaba Y, Okeda R. [Melanosis of choroid plexus papilloma of the lateral ventricle: a case report]. No Shinkei Geka 1987;15:1033-1037. 15. Reimund EL, Sitton JE, Harkin JC. Pigmented choroid plexus papilloma. Arch Pathol Lab Med
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1990;114:902-905.
16. Vajtai I, Varga Z, Bodosi M, Voros E. Melanotic papilloma of the choroid plexus: report of a case with implications for pathogenesis. Noshuyo byori = Brain tumor pathology 1995;12:151-154.
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17. Martin N, Pierot L, Sterkers O, Mompoint D, Nahum H. Primary choroid plexus papilloma of the cerebellopontine angle: MR imaging. Neuroradiology 1990;31:541-543. 18. Nomura H, Momma F, Furuichi S, Okamoto J. Primary choroid plexus papilloma of the foramen magnum--case report. Neurologia medico-chirurgica 1997;37:685-687. 19. Kimura M, Takayasu M, Suzuki Y, et al. Primary choroid plexus papilloma located in the suprasellar region: case report. Neurosurgery 1992;31:563-566. 20. Tuttenberg J, Fink W, Back W, Wenz F, Schadendorf D, Thome C. A rare primary sellar melanoma. Case report. Journal of neurosurgery 2004;100:931-934. 21. Coulibaly B, Bouvier C, Paula AM, Fernandez C, Dufour H, Figarella-Branger D. [Pituitary melanocytoma mimicking an adenoma]. Annales de pathologie 2011;31:50-52. 22. Tobo M, Sumiyoshi A, Yamakawa Y. Sellar teratoma with melanotic progonoma. A case report. Acta
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neuropathologica 1981;55:71-73. 23. Fuller CE, Smith M, Miller DC, Schelper R. Pigmented papillary epithelial neoplasm of the pituitary fossa: a distinct lesion of uncertain histogenesis. Archives of pathology & laboratory medicine 2001;125:1242-1245. 24. Chan AC, Ho LC, Yip WW, Cheung FC. Pigmented ependymoma with lipofuscin and neuromelanin
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production. Archives of pathology & laboratory medicine 2003;127:872-875.
25. McCloskey JJ, Parker JC, Jr., Brooks WH, Blacker HM. Melanin as a component of cerebral gliomas: the melanotic cerebral ependymoma. Cancer 1976;37:2373-2379.
26. Grajkowska W, Matyja E, Pronicki M, et al. Papillary ependymoma with unique superficial cortical location: immunohistochemical and ultrastructural studies. A case report. Folia neuropathologica / 2009;47:354-361.
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Association of Polish Neuropathologists and Medical Research Centre, Polish Academy of Sciences 27. Park SH, Park HR, Chi JG. Papillary ependymoma: its differential diagnosis from choroid plexus papilloma. Journal of Korean medical science 1996;11:415-421.
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28. Sidiropoulos M, Syro LV, Rotondo F, et al. Melanoma of the sellar region mimicking pituitary adenoma. Neuropathology : official journal of the Japanese Society of Neuropathology 2012. 29. Gyure KA, Morrison AL. Cytokeratin 7 and 20 expression in choroid plexus tumors: utility in differentiating these neoplasms from metastatic carcinomas. Mod Pathol 2000;13:638-643. 30. Albayram S, Urger E, Oz B, Kafadar A, Islak C, Kocer N. MR Imaging of pial melanosis secondary to a posterior fossa melanotic ependymoma. AJNR American journal of neuroradiology 2005;26:804-808. 31. Enochs WS, Petherick P, Bogdanova A, Mohr U, Weissleder R. Paramagnetic metal scavenging by melanin: MR imaging. Radiology 1997;204:417-423.
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32. Wang YY, Norris A, du Plessis D, Gnanalingham KK. Melanoma of the sellar region. Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia 2011;18:154-156. 33. Furuya K, Sasaki T, Saito N, Atsuchi M, Kirino T. Primary large choroid plexus papillomas in the cerebellopontine angle: radiological manifestations and surgical management. Acta neurochirurgica 1995;135:144-149.
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34. Vezzosi D, Capuani C, Loubes-Lacroix F, et al. Primary sellar melanocytic tumor: report of new case and literature review. Pituitary 2009;12:51-56. 35. Liu L, Liu ZX, Liu YS, et al. Applied anatomy for pituitary adenoma resection. Chin Med J (Engl)
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2011;124:2269-2274.
36. Song Y, Li H, Liu H, et al. Endoscopic endonasal transsphenoidal approach for sellar tumors beyond the sellar turcica. Acta Otolaryngol 2014;134:326-330. 37. Jinhu Y, Jianping D, Jun M, Hui S, Yepeng F. Metastasis of a histologically benign choroid plexus papilloma: case report and review of the literature. Journal of neuro-oncology 2007;83:47-52.
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Figure Legends: Fig. 1. Pre-operative, diagnostic MRI showing a mass within the sella turcica and
suprasellar cistern. A) Axial T1-weighted image showing a hyperintense mass. B) Axial
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T2-weighted image showing a hypointense mass. C) Coronal and D) Sagittal
T1-weighted images demonstrated that the hyperintense mass in minimally enhanced.
contiguous with choroid plexus.
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There is no evidence of extension of the mass into the third ventricle and the mass is not
Fig. 2. MRI demonstration subtotal resection of the intrasellar mass following
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transsphenoidal surgery. A) Axial T1 B) Axial T2 C) coronal T1 D) sagittal T1
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Fig. 3 Histological staining demonstrates a papillary structure with one or more layers of columnar epithelial cells around a fibrovascular core (hematoxylin and eosin A)10× B)
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20×. Immunohistochemistry indicates that the tumor cell express C) S-100 and D) Melanin-A, and negative for E) GFAP. Gross specimen of the resected tumor F).
Fig. 4. MRI demonstrating that the residual intrasellar tumor was enlarged 3 months after transsphenoidal surgery (A, B), Post-craniotomy MRI showing the tumor has been partially removed (C, D).
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Highlights We report a rare disease pigmented Choroid plexus papillomas in sellar region for the first time
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Treatment plan for this disease is proposed.
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We review the literatures and describe the clinical characters of this disease.
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Abbreviation List
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Choroid Plexus Papillomas Computed Tomography Magnetic Resonance Imaging Prolactin Free Thyroxine 4 Follicle-stimulating Hormone Human Growth Hormone Adrenocorticotropic Hormone Thyroid Stimulating Hormone Luteinizing Hormone Cytokeratin
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CPPs: CT： MRI： PRL： FT-4: FSH: HGH: ACTH: TSH: LH: CK:
S1878-8750(17)31052-5
DOI:
10.1016/j.wneu.2017.06.155
Reference:
WNEU 6026
To appear in:
World Neurosurgery
Received Date: 26 January 2017 Revised Date:
22 June 2017
Accepted Date: 24 June 2017
Please cite this article as: Gong X, Liu C, Zhang L, Li Z, Bartley CM, Liu Z, A primary pigmented choroid plexus papilloma located within the sella turcica: case report and literature review, World Neurosurgery (2017), doi: 10.1016/j.wneu.2017.06.155. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. 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.
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Title: A primary pigmented choroid plexus papilloma located within the sella turcica: case report and literature review
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Authors: Xuan Gong, MD, PhD; Chao Liu, MD, PhD; Longbo Zhang, MD, PhD; Zhenyan Li, MD, PhD; Christopher M Bartley, MD, PhD; Zhixiong Liu, MD, PhD Xuan Gong, Xiangya Hospital, Central South University, Department of Neurosurgery, 87 Xiangya Rd, Changsha, Hunan, P.R.China, 410008
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Chao Liu, Xiangya Hospital, Central South University, Department of Oncology, 87 Xiangya Rd, Changsha, Hunan, P.R.China, 410008
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Longbo Zhang, Xiangya Hospital, Central South University, Department of Neurosurgery, 87 Xiangya Rd, Changsha, Hunan, P.R.China, 410008 Zhenyan Li, Xiangya Hospital, Central South University, Department of Neurosurgery, 87 Xiangya Rd, Changsha, Hunan, P.R.China, 410008
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Christopher M. Bartley, University of California, San Francisco, Department of Psychiatry, 401 Parnassus Avenue, San Francisco, CA, USA, 94143-0984
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Corresponding Author: Zhixiong, Liu, M.D. PhD. Department of Neurosurgery, Xiangya Hospital, Central South University 87 Xiangya Rd, Changsha, Hunan, P.R.China, 410008 Phone: +8673189753537 Fax: +8673189753537 [email protected] Xuan Gong: [email protected] Chao Liu: [email protected] Longbo Zhang: [email protected] Zhenyan Li: [email protected] Christopher M Bartley: [email protected]
Key Words: Choroid Plexus Papilloma, pituitary, sella turcica, melanotic, pigmented
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Abstract: Background: Choroid plexus papillomas (CPPs) are rare benign tumors and the
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pigmented subtype is even more rarely observed.
Case Description: We present the case of a 43-year-old woman with complaints of
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headache and progressive left monocular visual deterioration, whose initial plain CT
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showed an ovate high density tumor located within in sellar region. MRI revealed a homogeneously contrast-enhancing tumor extending from the sella turcica to the suprasellar cistern. Single-nostril transsphenoidal endoscopic resection followed by subfrontal subtotal resection was performed in this patient. Postoperative histology
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demonstrated that the tumor consisted of hyperchromatic tissue with papillary features. Higher resolution examination of the tissue revealed this tissue was comprised of hyperplastic columnar epithelial cells with hyperchromatic cytoplasmic pigment.
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Subsequent immunohistochemistry identified the lesion as a pigmented choroid plexus
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papilloma. Herein we review the current literature, discuss the origin of the tumor, the differential of diagnosis, as well as the role of surgery and radiotherapy.
Conclusion: Our study provides important clinical information for the evaluation, diagnosis, and treatment of Pigmented CPP in sellar region.
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Background: Choroid plexus papillomas (CPPs) represent approximately 1% of
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intracranial tumors1. They are most frequently found in the lateral ventricles in infants and children1, 2 and in the fourth ventricles in adults3. Less commonly they are found in the third ventricle4 and cerebellopontine angles5, 6 and in rare instances, they can occur in
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the suprasellar region7, sacral canal8, posterior fossa9, brain stem10, or sella turcica11-13. The pigmented subtype of CPPs is rare and was only first reported in 1987 in Japan14,
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however subsequent cases have been reported15, 16. Upon literature review we failed to find a report of a melanin-containing CPP located within the sella turcica. To our knowledge, we present the first case report of a primary pigmented choroid plexus
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papilloma in the sella turcica. The clinical presentation of the tumor was similar to a pituitary adenoma however the diagnosis of CPP was definitively made by postoperative pathology, histological examination, and subsequent immunohistochemical analysis. The
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differential diagnosis and role of surgery and radiotherapy are herein discussed.
Case Description: A 43-year–old woman presented to our hospital with five months of intermit headaches and two months of progressive left monocular visual deterioration. She denied irregular menstruation, lactation, or motor/sensor deficits. On formal ophthalmologic examination she was found to have impaired visual acuity and bitemporal hemianopsia.
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Endocrine studies revealed a mildly decreased free thyroxine 4 level, and a moderately
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elevated prolactin level. Other pituitary hormones were within normal limits.
Initial x-ray computed tomography (CT) imaging revealed an elliptic high-density tumor
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located in the sellar region however, high resolution CT did not find evidence of
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calcification. Magnetic Resonance imaging (MRI) revealed a large and homogenous sellar mass occupying the entire sella turcica and extending into the suprasellar cistern that was hyperintense and hypointense on T1- and T2-weighted imaging respectively (Fig. 1A-B). No enhancement was observed after intravenous injection of contrast (Fig. 1C-D).
ventricles or cisterns.
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The pituitary stalk could not be clearly visualized and no abnormalities were found in the
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Based on the patient’s symptoms, hormone alterations, and radiologic findings, a
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preliminary preoperative diagnosis of pituitary adenoma was considered and single-nostril transsphenoidal endoscopic surgery was performed. Intraoperatively the tumor was found to be black and cauliflower-like in shape (Fig. 3F). Intraoperatively the tough texture and abundant blood flow prevented total resection of the tumor therefore a subtotal resection was performed (Fig. 2). Postoperatively, the patient’s vision in her left eye improved.
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Postoperative histology revealed that some of the tissue exhibited a papillary architecture, overlying a fibrovascular stroma covered by a layer of hyperplastic columnar epithelial
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cells. The cytoplasm of these hyperplastic columnar epithelial cells was hyperchromatic and the nuclei were moderately heteromorphic and infrequently mitotic (Fig. 3A-B. Immunohistochemistry indicated that the neoplastic cells were positive for melanocyte
for
GFAP
(Fig.
3E),
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cytokeratin
marker
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and
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negative
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markers S-100 (Fig. 3C), Melan-A (Fig. 3D), and HMB-45 (data not shown), but
adenohypophysical elements including FSH, HGH, ACTH, PRL, TSH and LH (data not shown). Based on image data, histology, and immunohistochemical analysis, a diagnosis of suprasellar pigmented choroid plexus papilloma was made.
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Three months after single-nostril transsphenoidal subtotal resection of the mass, the patient represented with a complaint of worsening vision in her left eye and an MRI
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revealed interval enlargement of the residual tumor (Figure 4 A-B). In order to salvage her vision, a second surgery was performed utilizing a subfrontal approach. Because the
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tumor was tough and tightly adhered to the surrounding vital structures, we opted in favor of a partial resection to avoid catastrophic neurovascular injury (Figure 4 C-D). The surgery was followed by adjunctive radiotherapy and the patient has been asymptomatic the two years.
Discussion: A choroid plexus papilloma was first described by Guerarard in 1832 in a
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case of a 3-year old child. CPPs are usually derived from normal choroid plexus and are most often found in the fourth ventricle, lateral ventricle, and in the third ventricle owing
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to the normal anatomic location of the choroid plexus. Occasionally, exophytic extension of normal choroid tufts into or through the foramens of Luschka and Magendie result in CPPs located in the cerebellopontine angle17 or foramen magnum18. In rare cases, ectopic
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choroid plexus or metaplastic transformation of ependymal results in extraventricular
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primary CPPs such as in brain stem, posterior fossa, sacral canal, or suprasellar or intrasellar region.
We are aware of four prior cases of CPPs originating from the sellar region 11-13, 19 and all
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were female between the ages of 31-51 years old. As in our case, there was no evidence that the CPP was contiguous with ventricular choroid plexus or that they were products of metastasis. As such, these cases likely originated from ectopic choroid plexus or
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metaplastic transformation.
A number of cases of extrasellar pigmented CPPs have been reported, including: a 14-year-old white male with pigmented CPP in the lateral ventricle15; a case of 35-year-old man with a pigmented CPP in the fourth ventricle16; and a 45-year-old man with a pigmented CPP in the left lateral ventricle14. To our knowledge, our case of a 43-year-old woman is the only reported case of a primary pigmented CPP located outside of the ventricles.
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Several other primary pigmented neoplasms are known to occur within the region of the 21
, both of which symptomatically
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sella tucica including melanoma and melanocytoma20
mimic pituitary adenoma. Other, rarer sellar pigmented tumors have been reported including a teratoma with melanotic progonoma22. One case of pigmented papillary
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epithelial neoplasm arising within the pituitary fossa has been reported23, however
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subsequent follow-up found that this patient’s tumor later developed into a carcinosarcoma and as such does not belong to a known tumor type.
The differential diagnosis for pigmented CPPs includes papillary ependymoma, papillary
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pituitary adenoma, and metastatic melanoma. Papillary ependymomas, (which occasionally occur with pigmentation)24,
25
, are typically densely cellular with
perivascular rosettes and pseudorosettes26. The core of papillary ependymomal papillary
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structures are composed of the neuroglial stroma rather than the fibrovascular stroma
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seen in CPPs27. They also exhibit characteristic cilia and a tubular structure. Immunohistochemically, the neoplastic cells of ependymomas are often positive for GFAP but negative for cytokeratin, but there are exceptions27. In pituitary adenomas, anterior pituitary hormones are often reactive but S-100 is not, and a pigmented pituitary adenoma has not yet been reported. Metastatic melanomas can display papillary structures (such as thyroid papillary carcinoma), however they typically exhibit characteristics of malignant neoplasm such as a high mitotic index, nuclear pleomophism
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and necrosis 28. CPPs are often immunopositive for CK7 however a previous study found the approximately 25% of definitively diagnosed CPPs are negative for this marker and
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the expression profile of pigmented CPPs has not been well defined29.
The pigmented CPPs generally owe their color to the expression of melanin or
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neuromelanin and are radiologically distinct from conventional CPPs15. CPPs are
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generally isointense on T1 and T2 however their T2 signature is more variable. Melanomas and some other melanotic tumors present hyperintense on T1-weighted images and hypointense on T2-weighted images30. This characteristic MR signature of melanin-containing lesions is sometimes referred to as “melanin scavenging” and is
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thought to be due to melanin binding of paramagnetic metals31. Consistent with this melanin signature, the tumor in our case demonstrate high signal on T1-weighted and low signal on T2-weighted images. However, MRI imaging of CNS melanotic lesions often
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also suggests haemorrhagic or proteinaceous content32. Moreover, the histological
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architecture of our tumor is highly inconsistent with melanoma making this diagnosis unlikely.
Total microsurgical extirpation of the CPPs is the recommended treatment and adjunctive radiotherapy is typically reserved for choroid plexus carcinomas33. For sellar melanocytic tumors, transsphenoidal surgery is recommended with fractionated radiotherapy if total resection is not feasible34. In our case, the tumor was first partially resected via an
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endonasal trans-sphenoidal followed by a trans-cranial approach. Owing to the abundant blood flow, indistinct border, and adhesions to surrounding tissue, the surgery was limited
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to a subtotal resection35, 36.
The role of radiotherapy in the treatment of CPPs is uncertain and this probably reflects
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the rarity of these tumors, in particular those associated with recurrent or metastatic disease after total and subtotal resection37, however in primary sellar melanocytic tumor,
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radiotherapy is recommended34. In our case, the tumor progressed following subtotal resection, so postoperative adjunctive radiotherapy was employed with complete
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resolution of symptoms to date.
Conclusion: To our knowledge, this is the first sellar pigmented choroid plexus papilloma that has been reported in the literature. Our study provides important clinical
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information for the diagnosis and management of this rare disease entity.
The authors declare that they have no conflict of interest.
References:
1.
Laurence KM, Hoare RD, Till K. The diagnosis of the choroid plexus papilloma of the lateral
ventricle. Brain : a journal of neurology 1961;84:628-641.
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2.
Tominaga A, Fujioka Y, Monden S, Kawamoto Y, Uozumi T, Konishi N. [Choroid plexus papilloma of
the third ventricle in infancy: a case report]. No shinkei geka Neurological surgery 1992;20:1273-1276. 3.
Wolff JE, Sajedi M, Brant R, Coppes MJ, Egeler RM. Choroid plexus tumours. British journal of
cancer 2002;87:1086-1091. Fortuna A, Celli P, Ferrante L, Turano C. A review of papillomas of the third ventricle. One case
report. Journal of neurosurgical sciences 1979;23:61-76. 5.
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4.
Talacchi A, De Micheli E, Lombardo C, Turazzi S, Bricolo A. Choroid plexus papilloma of the
cerebellopontine angle: a twelve patient series. Surgical neurology 1999;51:621-629. 6.
Kieserman S, Linstrom C, McCormick S, Petschenik AJ. Choroid plexus papilloma of the
cerebellopontine angle. The American journal of otology 1996;17:119-122. 7.
Keskin F, Erdi F, Kaya B, Toy H. Sellar-Suprasellar Extraventricular Choroid Plexus Papilloma : A
8.
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Case Report and Review of the Literature. J Korean Neurosurg Soc 2016;59:58-61.
Kurtkaya-Yapicier O, Scheithauer BW, Van Peteghem KP, Sawicki JE. Unusual case of extradural
choroid plexus papilloma of the sacral canal. Case report. Journal of neurosurgery 2002;97:102-105. Garcia-Valtuille R, Abascal F, Garcia-Valtuille AI, et al. Adult choroid plexus papilloma of the
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9.
posterior fossa mimicking a hemangioblastoma. Case report. Journal of neurosurgery 2000;92:870-872. 10. Pillai A, Rajeev K, Chandi S, Unnikrishnan M. Intrinsic brainstem choroid plexus papilloma. Case report. Journal of neurosurgery 2004;100:1076-1078.
11. Bian LG, Sun QF, Wu HC, Jiang H, Sun YH, Shen JK. Primary choroid plexus papilloma in the pituitary fossa: case report and literature review. Acta neurochirurgica 2011;153:851-857. 12. Ma YH, Ye K, Zhan RY, Wang LJ. Primary choroid plexus papilloma of the sellar region. Journal of neuro-oncology 2008;88:51-55.
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13. Sameshima T, Tanikawa R, Sugimura T, et al. Choroid plexus papilloma originating in the sella turcica--case report. Neurologia medico-chirurgica 2010;50:144-146. 14. Yamazaki S, Ito U, Tomita H, Takada Y, Inaba Y, Okeda R. [Melanosis of choroid plexus papilloma of the lateral ventricle: a case report]. No Shinkei Geka 1987;15:1033-1037. 15. Reimund EL, Sitton JE, Harkin JC. Pigmented choroid plexus papilloma. Arch Pathol Lab Med
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1990;114:902-905.
16. Vajtai I, Varga Z, Bodosi M, Voros E. Melanotic papilloma of the choroid plexus: report of a case with implications for pathogenesis. Noshuyo byori = Brain tumor pathology 1995;12:151-154.
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17. Martin N, Pierot L, Sterkers O, Mompoint D, Nahum H. Primary choroid plexus papilloma of the cerebellopontine angle: MR imaging. Neuroradiology 1990;31:541-543. 18. Nomura H, Momma F, Furuichi S, Okamoto J. Primary choroid plexus papilloma of the foramen magnum--case report. Neurologia medico-chirurgica 1997;37:685-687. 19. Kimura M, Takayasu M, Suzuki Y, et al. Primary choroid plexus papilloma located in the suprasellar region: case report. Neurosurgery 1992;31:563-566. 20. Tuttenberg J, Fink W, Back W, Wenz F, Schadendorf D, Thome C. A rare primary sellar melanoma. Case report. Journal of neurosurgery 2004;100:931-934. 21. Coulibaly B, Bouvier C, Paula AM, Fernandez C, Dufour H, Figarella-Branger D. [Pituitary melanocytoma mimicking an adenoma]. Annales de pathologie 2011;31:50-52. 22. Tobo M, Sumiyoshi A, Yamakawa Y. Sellar teratoma with melanotic progonoma. A case report. Acta
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neuropathologica 1981;55:71-73. 23. Fuller CE, Smith M, Miller DC, Schelper R. Pigmented papillary epithelial neoplasm of the pituitary fossa: a distinct lesion of uncertain histogenesis. Archives of pathology & laboratory medicine 2001;125:1242-1245. 24. Chan AC, Ho LC, Yip WW, Cheung FC. Pigmented ependymoma with lipofuscin and neuromelanin
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production. Archives of pathology & laboratory medicine 2003;127:872-875.
25. McCloskey JJ, Parker JC, Jr., Brooks WH, Blacker HM. Melanin as a component of cerebral gliomas: the melanotic cerebral ependymoma. Cancer 1976;37:2373-2379.
26. Grajkowska W, Matyja E, Pronicki M, et al. Papillary ependymoma with unique superficial cortical location: immunohistochemical and ultrastructural studies. A case report. Folia neuropathologica / 2009;47:354-361.
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Association of Polish Neuropathologists and Medical Research Centre, Polish Academy of Sciences 27. Park SH, Park HR, Chi JG. Papillary ependymoma: its differential diagnosis from choroid plexus papilloma. Journal of Korean medical science 1996;11:415-421.
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28. Sidiropoulos M, Syro LV, Rotondo F, et al. Melanoma of the sellar region mimicking pituitary adenoma. Neuropathology : official journal of the Japanese Society of Neuropathology 2012. 29. Gyure KA, Morrison AL. Cytokeratin 7 and 20 expression in choroid plexus tumors: utility in differentiating these neoplasms from metastatic carcinomas. Mod Pathol 2000;13:638-643. 30. Albayram S, Urger E, Oz B, Kafadar A, Islak C, Kocer N. MR Imaging of pial melanosis secondary to a posterior fossa melanotic ependymoma. AJNR American journal of neuroradiology 2005;26:804-808. 31. Enochs WS, Petherick P, Bogdanova A, Mohr U, Weissleder R. Paramagnetic metal scavenging by melanin: MR imaging. Radiology 1997;204:417-423.
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32. Wang YY, Norris A, du Plessis D, Gnanalingham KK. Melanoma of the sellar region. Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia 2011;18:154-156. 33. Furuya K, Sasaki T, Saito N, Atsuchi M, Kirino T. Primary large choroid plexus papillomas in the cerebellopontine angle: radiological manifestations and surgical management. Acta neurochirurgica 1995;135:144-149.
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34. Vezzosi D, Capuani C, Loubes-Lacroix F, et al. Primary sellar melanocytic tumor: report of new case and literature review. Pituitary 2009;12:51-56. 35. Liu L, Liu ZX, Liu YS, et al. Applied anatomy for pituitary adenoma resection. Chin Med J (Engl)
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2011;124:2269-2274.
36. Song Y, Li H, Liu H, et al. Endoscopic endonasal transsphenoidal approach for sellar tumors beyond the sellar turcica. Acta Otolaryngol 2014;134:326-330. 37. Jinhu Y, Jianping D, Jun M, Hui S, Yepeng F. Metastasis of a histologically benign choroid plexus papilloma: case report and review of the literature. Journal of neuro-oncology 2007;83:47-52.
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Figure Legends: Fig. 1. Pre-operative, diagnostic MRI showing a mass within the sella turcica and
suprasellar cistern. A) Axial T1-weighted image showing a hyperintense mass. B) Axial
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T2-weighted image showing a hypointense mass. C) Coronal and D) Sagittal
T1-weighted images demonstrated that the hyperintense mass in minimally enhanced.
contiguous with choroid plexus.
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There is no evidence of extension of the mass into the third ventricle and the mass is not
Fig. 2. MRI demonstration subtotal resection of the intrasellar mass following
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transsphenoidal surgery. A) Axial T1 B) Axial T2 C) coronal T1 D) sagittal T1
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Fig. 3 Histological staining demonstrates a papillary structure with one or more layers of columnar epithelial cells around a fibrovascular core (hematoxylin and eosin A)10× B)
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20×. Immunohistochemistry indicates that the tumor cell express C) S-100 and D) Melanin-A, and negative for E) GFAP. Gross specimen of the resected tumor F).
Fig. 4. MRI demonstrating that the residual intrasellar tumor was enlarged 3 months after transsphenoidal surgery (A, B), Post-craniotomy MRI showing the tumor has been partially removed (C, D).
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Highlights We report a rare disease pigmented Choroid plexus papillomas in sellar region for the first time
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Treatment plan for this disease is proposed.
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We review the literatures and describe the clinical characters of this disease.
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Abbreviation List
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Choroid Plexus Papillomas Computed Tomography Magnetic Resonance Imaging Prolactin Free Thyroxine 4 Follicle-stimulating Hormone Human Growth Hormone Adrenocorticotropic Hormone Thyroid Stimulating Hormone Luteinizing Hormone Cytokeratin
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CPPs: CT： MRI： PRL： FT-4: FSH: HGH: ACTH: TSH: LH: CK: