Clinical Neurology and Neurosurgery 137 (2015) 38–43

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Review

Hard calcified intrasellar schwannoma mimicking pituitary adenoma: A case report and review of the literature Guive Sharifi a , Mehrdad Hosseinzadeh Bakhtevari a,∗ , Mohsen Alghasi b , Masood Saberi c , Mahmood Dehghan a , Farahnaz Bidari d , Omidvar Rezaei a a

Department of Neurosurgery, Loghman e Hakim Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Department of Neurosurgery, Firouzgar Hospital, Iran University of Medical Sciences, Tehran, Iran c Department of Neurosurgery, Erfan General Hospital, Tehran, Iran d Department of Neuropathology, Loghman e Hakim Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran b

a r t i c l e

i n f o

Article history: Received 1 April 2015 Received in revised form 23 May 2015 Accepted 2 June 2015 Available online 18 June 2015 Keywords: Calcified Pituitary Sellar Schwannoma

a b s t r a c t Background: Intrasellar location of schwannoma is extremely rare, although intracranial schwannomas are common in the central nervous system. The aim of the present study is to describe a calcified intrasellar schwannoma case. Materials and methods: We represent a 45-year-old woman who had suffered from headaches; right side facial pain and visual disturbance which had worsen during the last week prior to admission. Physical examinations were normal except for the bitemporal visual field hemianopia which match with perimetry examination. MRI demonstrated an unusual seemingly calcified mass lesion in the sellar region which was mimicking pituitary macro adenoma. Result: Total resection of the tumor achieved through endoscopic transnasal transsphenoidal approach by extracapsular dissection and pathologic examination of the tumor revealed calcified schwannoma. Conclusion: The differential diagnoses of sellar and suprasellar lesions include pituitary adenomas, craniopharyngiomas, meningiomas, and many others. However, schwannoma is not usually included, because the occurrence of schwannoma in the sellar or suprasellar region is extremely rare. Only few cases of intrasellar schwannomas have been reported in the literature, all of which presented a suprasellar extension similar to that of our case. Fascinating surgical point is managing very firm tumor through transsphenoidal corridor which we handle it by very sharp, debulking and extracapsular removal. © 2015 Elsevier B.V. All rights reserved.

Contents 1. 2. 3. 4.

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 39 Materials and methods . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 39 2.1. Case report . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 39 Discussion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 39 Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 42 Conflict of interest . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 42 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 43

∗ Corresponding author. Tel.: +98 912 1874506; fax: +98 21 55414065; mobile: +98 912 1874506. E-mail address: [email protected] (M.H. Bakhtevari). http://dx.doi.org/10.1016/j.clineuro.2015.06.001 0303-8467/© 2015 Elsevier B.V. All rights reserved.

G. Sharifi et al. / Clinical Neurology and Neurosurgery 137 (2015) 38–43

1. Introduction Schwannomas are relatively common tumors comprising 8–10% of all primary intracranial neoplasms; however the intrasellar location of schwannoma is extremely rare [1]. Schwannomas generally originate from the sensory nerves and have the potential to occur anywhere Schwann cells exist. This is while they have been recorded in virtually every site itracranically, including intracerebrally [2]. We present an unusual case of a calcified intrasellar schwannoma radiographically and clinically simulating a pituitary adenoma. The patient had been suffering from headaches, right side facial pain and visual disturbance. Intracranial schwannomas commonly arises from the eighth cranial nerve in the cerebellopontine angle. Schwannoma arising from the sella and extending into the suprasellar region are very rare and are easily mistaken for pituitary adenoma.

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Postoperatively, the patient awoke without difficulty and was transferred to the intensive care unit for 24 h after surgery. There was no postop diabetes insipidus. Visual complaints abated satisfactorily. There was no complaint of facial pain anymore and she released from hospital on day three. Postop course was uneventful. MRI 3 months after surgery showed total resection of the tumor, saved pituitary gland and small fat inside the sella which we used for prevention of CSF leak (Fig. 5). Hormonal profile was normal after surgery and the patient did not need any hormonal replacement therapy. Because of her preoperative facial pain and tumor origin from the right cavernous sinus and finding schwannoma on histopathology it would be reasonable to postulate the tumor was a schwannoma of ophthalmic or maxillary branch of trigeminal nerve, although we cannot prove that. After surgery no extraocular muscle problem occurred and corneal sensation and facial senses was intact.

2. Materials and methods 3. Discussion 2.1. Case report A 45-year-old woman came to us with headaches; right side facial pain and visual disturbance from 6 months ago which had worsen during the last week. Physical examinations were all normal except bitemporal visual field hemianopia which matches with perimetry examination. Laboratory findings and hormonal profile were normal. Magnetic resonance imaging (MRI) demonstrated a lesion in the sellar region which was isointense to hypointense on T1-weighted and hypointense on T2-weighted images extending to suprasellar cistern and compressing the optic chiasm. Following contrast injection there was an enhancement in the dome of lesion which represented compressed pituitary gland and another globular enhancement next to right carotid (Fig. 1). Because of the presence of flow void like signal on coronal T1-weighted image near the carotid artery (although it also characterize calcification) we performed CT angiography to rule out vascular anomaly such as carotid aneurysm (Fig. 1 H and I). It showed normal vascular anatomy and separate adjacent dens calcification near the carotid artery. Some contradictory imaging findings as presence of bulky calcification in the sellar region and unusual intrinsic intensity (such as hypointensity on T2-weighted MRI and unusual enhancement pattern) was harbinger of an unfamiliar pathology other than the everyday pituitary macroadenoma.Patient’s right facial pain (constant burning pain in periorbit and cheek) also added to our concern about diagnosis. We approached the lesion through transsphenoidal corridor. Assisted by our rhinologist colleague we exposed sellar floor endoscopically. After drilling the sellar floor and opening the dura we encountered grayish firm tumor (Fig. 2). Its consistency was rubbery that it could not be cut easily by knife. We decompress the lesion by punching it by otolaryngology throughcut punch. After internal debulking we proceed by extracapsular mean to dissect and resect the tumor. Its adherence to right cavernous sinus and carotid was significant. Superiorly diaphragm was opened and we tried to save compressed pituitary gland and stalk. By exploring the suprasellar cistern we removed the tumor totally and mammillary bodies and vascular structures showed up (Fig. 3). Repair of the sellar defect was done with autologous fat and fascia lata. A nasoseptal flap turned and covered the defect. Histopathologic examination (Fig. 4) recommended schwannoma versus meningioma which immunohistochemistry (IHC) confirmed its diagnosis in favor of schwannoma which was highly positive for S100 antigen, positive for vimentin and negative for epithelial membrane antigen (EMA).

Most neoplasms growing in the sella turcica are pituitary adenomas. The remaining neoplasms are benign tumors (craniopharyntgiomas and meningiomas), malignant tumors (primary germ cell tumors, chordomas, lymphomas, and pituitary adenocarcinomas, or metastatic disease mainly from breast and lung cancer), and other uncommon conditions (cysts, abscesses, arteriovenous fistulas, lymphocytic hypophysitis) [3]. As mentioned before, schwannoma accounts for up to 8% of primary brain tumors [4], and is usually associated with the cranial nerves and shows a special predilection for the vestibular portion of the 8th cranial nerve at the cerebellopontine angle, while intrasellar localization is exceptional. The most common differential diagnoses of sellar and suprasellar lesions include pituitary adenomas, craniopharyngiomas, and meningiomas; however, schwannoma is not usually included. Only few cases of intrasellar schwannomas have been reported previously (Table 1), all of which presented a suprasellar extension similar to that of our case [5]. The clinical and radiological presentation of intrasellar schwannomas is consistent with the findings of pituitary adenomas. The most common signs and symptoms of intracranial schwannomas are headaches, seizures, and focal neurologic deficits [1]; similar to our case which was developed with headaches, and bitemporal hemianopia. Other pathologic characteristic and imaging features of intraparenchymal schwannomas include calcification, cyst formation, peritumoral edema and/or gliosis, and superficial or periventricular location [1]. As expected, the MRI findings of this study showed that the lesion has cystic component in the sellar region and has been calcified [2]. In a study conducted by Whee et al. it was revealed that the enlargement of the sella turcica and an enhancing sellar mass with suprasellar extension are most often associated with pituitary adenomas [5] and radiological findings in these cases were not significantly different from the usual pituitary macroadenomas. In an excellent review on the unusual lesions in the region of sella, Chadduck [6] reported one patient with a trigeminal schwannoma secondarily involving the suprasellar region. Afterwards, Goebel et al. [7] described a schwannoma reportedly arising from tuberculum sella without associated extension into the sella. As there is no obvious nerve within the sella, the origin of primary intrasellar schwannomas supposed to be from lateral sellar nerve plexus, perivascular schwann cells, or sensory nerves of the dura [8–10]. Firstly, lateral sellar nerve plexus is a distribution center for visceromotor and sensory nerves, which innervate cerebral

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G. Sharifi et al. / Clinical Neurology and Neurosurgery 137 (2015) 38–43

Fig. 1. Brain MRI showing suprasellar mass which is isointense on T1W hypointense on T2W and heterogeneous enhancement after gadolinium contrast.

arteries, orbital structures, and the dura mater [8]. The potential deficiency of the medial wall of the cavernous sinus has also been increasingly appreciated. Secondly, Penfield demonstrated the presence of perivascular schwann cells. Cerebral arteries as small as 10–15 ␮m in diameter receive adrenergic supply. The occurrence of schwannomas within the substance of the spinal cord has been attributed to the proliferation of schwann cells from

nerve plexuses accompanying the perivascular spaces of perforating branches. In the third place, an origin from schwann cells that encircle small dural sensory branches of the trigeminal nerve or vasomotor nerves has been proposed [8–11]. As mentioned previously, the patient in our case demonstrated a calcified mass lesion in the sellar region. The gross total excision of the lesion was done via an endoscopic transnasal

Fig. 2. Intraoperative photograph of the calcified tumor during surgery and after resection.

G. Sharifi et al. / Clinical Neurology and Neurosurgery 137 (2015) 38–43

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Fig. 3. Postoperative photograph revealed total removal of the tumor (O, optic nerve; M, mammillary body).

Fig. 4. Left: Hematoxylin and eosin staining demonstrated spindle shaped cells with rod shape nuclei and dens reticulin consistent with antoni A, and stellate cells with smaller nuclei and less reticulin and prominent cytoplasmic processes consistent with antoni B. Right: Palisading of nuclei forming a verocay body.

Fig. 5. Post-operative MRI revealed total resection of the tumor (reconstruction of sellar defect with fat and fascia lata).

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G. Sharifi et al. / Clinical Neurology and Neurosurgery 137 (2015) 38–43

Table 1 Literature review of intrasellar schwannoma. Authors (year)

Age (years), sex

Symptoms & signs

Endocrine function

Management

Goebel et al. (1979) [7] Perone et al. (1984) [12]

25, F 39, M

Moderate hypogonadism Nl

Transcranial resection Transsphenoidal resection

Wilberger et al. (1989) [2]

62, F

Grandmal seizure Incidentally findings after head trauma Headache & visual disturbance

Panhypopituitarism

Guenot et al. (1994) [13]

67, F

Panhtpopituitarism

Civit et al. (1997) [10]

41, M

Headache & decreased left visual acuity & left occulomotor nerve palsy Bitemporal hemianopia

1. Incomplete transsphenoidal resection 2. Gross total transcranial resection Transsphenoidal resection

Kim et al. (2002) [14]

39, M

Whee et al. (2002) [5]

39, M

Maartens et al. (2003) [15]

1.33, F 2.56, F

Esposito et al. (2004) [16]

73, M

Perez et al. (2004) [17] Yoon et al. (2005) [18]

71, F 1.34, M 2.62, M

Honegger et al. (2005) [19] Moreland et al. (2006) [20]

79, F 41, M

Two episodes of syncope Headache & mood changes & fatigue

Shin et al. (2006) [21] Rodriguez et al. (2007) [22]

a

41, M

Chadduck et al. [23] Ishige et al. [24] Kasantikul et al. [25]

53, F 64, F 48, F

Park et al. (2009) [26]

49, F

Bhagat et al. (2010) [29] Mohammed et al. (2010) [30]

68, M 1.19, F 2.45, F

Koutourousiou et al. (2010) [27] Boj-carcellar et al. (2012) [3]

56, F

Cugati et al. (2012) [28]

64, M

1. Normal function before surgery 2. Postoperative pituitary insufficiency Pituitary insufficiency

1. Transsphenoidal biopsy 2. Transcranial subfrontal resection 3 months later

Panhypopituitarism

Transsphenoidal resection

1. NL 2. NL

NL NL

1. Transsphenoidal total resection 2. Transsphenoidal and then transcranial total resection Endoscopic transnasal transsphenoidal approach Transsphenoidal resection 1. Six months thyroid hormone therapy with miss-diagnosis of thyroid hyperplasia and then transsphenoidal biopsy & resection 2. Transsphenoidal resectiom Transsphenoidal total resection Transsphenoidal resection

a

a

a

Tingling of right side of the face from 4 years ago & Bitemporal hemianopia from 12 months ago & 4th and 6th nerve paresis Headache Headache & Facial pain Headache & visual disturbance & subarachnoid hemorrhage Headache & vomiting & visual disturbance

NL

Right pterional transcranial resection (80% resection)

NL NL NL

Transcranial resection Transsphenoidal resection Transcranial resection

NL

1. Transsphenoidal partial resection 2. Transcranial total resection 5 months later Transsphenoidal resection 1. Transcranial bifrontal resection 2. Transsphenoidal resection Transsphenoidal resection

Incidentally findings after sinusitis work out, Bitemporal visual field deficiency Visual disturbance & decreased libido 1. Headache 2. Headache & Bitemporal hemianopia & Hydrocepalous Lypothimia & mental changes & Bitemporal hemianopia Visual field defect 1. Headache 2. Headache & visual disturbance & polyuria with polydipsia

Visual disturbance 1. Left eye visual field disturbance 2. Headache & Facial pain Presenting with Growth hormone secreting adenoma Headache

Asymptomatic, incidentally finding

Panhypopituitarism Nl 1. Hupothyroidism 2. Nl

Panhypopituitarism 1. NL 2. Mild panhypopituitarism Elevated Growth hormone and IGF-1 1. Normal before surgery 2. Panhypopituitarism after surgery Panhypopituitarism after surgery

Sublabial transsphenoidal near total resection

1. Transsphenoidal incomplete resection 2. Transcranial right frontal complete resection Transsphenoidal resection

Summary of previously reported intrasellar schwannoma cases. a Korean journal, we had no access.

extracapsular transsphenoidal approach and a post-operative MRI showed no residual tumor. 4. Conclusion To sum up, it is not irrelevant to mention that a reference should be made to the question of the origin of the intrasellar schwannomas [4]. This is due to the fact that Schwann cells are not located in the central nervous system. Actually the remote possibility of an intrasellar schwannoma should be considered if different

diagnosis of pituitary lesions, and immunohistochemistry is determinant in the final diagnosis of these rare sellar masses. The very limited cases reported to date do not allow the sellar schwannoma profile to be established with certainty.

Conflict of interest All authors certify that they have no affiliations with or involvement in any organization or entity with any financial interest, or

G. Sharifi et al. / Clinical Neurology and Neurosurgery 137 (2015) 38–43

non-financial interest in the subject matter or materials discussed in this manuscript. There is no funding or conflict of interest There is no financial disclosures. References [1] Russell DS, Rubinstein LJ. Pathology of Tumours of the Nervous System. Williams and Wilkins Co.; 1977. [2] Wilberger Jr JE. Primary intrasellar schwannoma: case report. Surg Neurol 1989;32(2):156–8. [3] Boj Carceller D, Albero Gamboa R, Verdes Sanz G, Vicente Arregui S, Alvarez Alegret R. Intrasellar schwannoma: a rare case of pituitary incidentaloma. Endocrinol Nutr 2012;59(5):336–8. [4] Woodruff J, Kourea H, Louis D, Scheithauer B. Schwannoma. World Health Organization classification of tumors. Pathol Genet Nerv Syst 2000:164–8. [5] Whee SM, Lee JI, Kim JH. Intrasellar schwannoma mimicking pituitary adenoma: a case report. J Korean Med Sci 2002;17(1):147–50. [6] Chadduck WM. Unusual lesions involving the sella turcica. South Med J 1973;66(8):948–55. [7] Goebel HH, Shimokawa K, Schaake T, Kremp A. Schwannoma of the sellar region. Acta Neurochir 1979;48(3–4):191–7. [8] Bleys RL, Janssen LM, Groen GJ. The lateral sellar nerve plexus and its connections in humans. J Neurosurg 2001;95:102–10. [9] Gibson AA, Hendrick EB, Conen PE. Case reports. Intracerebral schwannoma. Report of a case. J Neurosurg 1996;24:552–7. [10] Civit T, Pinelli C, Klein M, Auque J, Baylac F, Hepner H:. Intrasellar schwannoma. Acta Neurochir 1997;139:160–1. [11] Penfield W. Intracerebral vascular nerves. Arch Neurol Psychiatr 1958;21:92–4. [12] Perone TP, Robinson B, Holmes SM. Intrasellar schwannoma: case report. Neurosurgery 1984;14:71–3. [13] Guenot M, Bataille B, Wager M. Intrasellar neurinoma. Apropos of a case and review of the literature. Neurochirurgie 1994;40(4):263–6. [14] Kim NR, Suh YL. Primary Intrasellar Schwannoma - A Case Report. Korean J Pathol 2002;36:274–7. [15] Maartens NF, Ellegala DB, Vance ML, Lopes MB, Laws Jr ER. Intrasellar schwannomas: report of two cases. Neurosurgery 2003;52:1200–5 [discussion 1205–6].

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[16] Esposito V, Fraioli B, Ferrante L, et al. Intrasellar tuberculomas: case report. Neurosurgery. 21:721–23. [17] Perez MT, Farkas J, Padron S, Changus JE, Webster EL. Intrasellar and parasellar cellular schwannoma. Ann Diagn Pathol 2004;8:142–50. [18] Wan-Soo Yoon, Ik Seong Park, Min-Woo Baik. Intrasellar scwannoma. J Korean Neurosurg Soc 2005;37:157–9. [19] Honegger J, Koerbel A, Psaras T, Petrick M, Mueller K. Primary Intrasellar Schwannoma, clinical, aetiopathological and surgical consideration. Britis J Neurosurg 2005;19(5), http://dx.doi.org/10.1080/0268869050039039. [20] Moreland DB. Intrasellar pituitary schwannoma. J Clin Neurosci 2006;13:771–4. [21] Shin JL, Asa SL, Woodouse LJ, et al. Cystic lesions of the pituitary: clinicopathological features distinguishing craniopharyngioma, Rathke’s cleft cyst, and arachnoid cyst. J Clin Endocrinol Metab 1999;84:3972–82. [22] de Asís Bravo-Rodríguez F, et al., editors. Learning Neuroimaging, Learning Imaging. © Springer-Verlag Berlin Heidelberg; 2012., http://dx.doi.org/10.1007/978-3-642-22999-2 2. [23] Chadduck WM. Unusual lesions involving the sella turcica. South Med J 1973;66(8):948–55. [24] Ishige N, Ito C, Saeki N, Oka N. Neurinoma with intrasellar extension: a case report. No Shinkei Geka 1985;13:79–84. [25] Kasantikul V, Brown WJ, CahanF L.D. Intracerebral neurilemmoma. J Neurol Neurosurg Psychiatry 1981;44:1110–5, http://dx.doi.org/10.1136/ jnnp.44.12.1110. [26] Hyun-Woong Park, Shin Jung, Tae-Young Jung, Kyung-Sub Moon. IntraSuprasellar Schwannoma Originating from the Diaphragma Sellae. J Korean Neurosurg Soc 2009;45:375–7. [27] Koutourousiou M, Kontogeorgos G, Wesseling P, Grotenhuis AJ, Seretis A. Collision sellar lesions: experience with eight cases and review of the literature. Pituitary 2010;13:8–17. [28] Goutham Cugati, Manish Singh, Nigel Peter Symss, Anil Pande, T C Yasha, Madhabushi Chakravarthy Vasudevan, Ravi Ramamurthi. Primary intrasellar schwannoma. J Clin Neurosci 2012;19(11):1584–5. [29] Bhagat S, Smith C, Teasdale GM, McFadzean RM. Nerve sheath tumors of the sellar region. J Neuroophthalmol 2002;22(4):275–8. [30] Mohammed S, Kovacs K, Munoz D, Cusimano MD. A short illustrated review of sellar region schwannomas. Acta Neurochir (Wien) 2010;152(5):885–991, http://dx.doi.org/10.1007/s00701-009-0527-7. Epub 2010 Feb 4.

Hard calcified intrasellar schwannoma mimicking pituitary adenoma: a case report and review of the literature.

Intrasellar location of schwannoma is extremely rare, although intracranial schwannomas are common in the central nervous system. The aim of the prese...
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