- Email: [email protected]
Intrasellar dermoid cyst mimicking pituitary apoplexy: A case report and review of the literature
Journal of Clinical Neuroscience xxx (2017) xxx–xxx
Contents lists available at ScienceDirect
Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn
Case report
Intrasellar dermoid cyst mimicking pituitary apoplexy: A case report and review of the literature Yuan-Bo Pan, Zhao-Liang Sun, Dong-Fu Feng ⇑ Department of Neurosurgery, Ninth People’s Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, PR China
a r t i c l e
i n f o
Article history: Received 14 February 2017 Accepted 22 May 2017 Available online xxxx Keywords: Dermoid cyst Intracranial Pituitary apoplexy Tumor
a b s t r a c t Intrasellar dermoid cysts are extremely unusual lesions, with only four cases reported to date, and have not been previously reported in association with sudden-onset symptoms. Here, we present the case of an intrasellar dermoid cyst with sudden-onset symptoms mimicking pituitary apoplexy in an elderly woman. A 69 year-old woman presented with sudden onset of headache, dizziness, and decreased visual acuity. Magnetic resonance imaging of the sellar region showed an intrasellar lesion, which showed mixed hyper- and hypointense signal on T1-weighted and T2-weighted images and enhanced peripherally. Endocrine workup showed pituitary hormones within normal levels. According to these findings, the initial diagnosis of nonsecreting pituitary macroadenoma apoplexy was made. Intraoperatively, a large amount of whitish-yellow purulent material was found in the mass and the lesion was partially removed, owing to tight adhesion between remanent mass and surrounding neurovascular structures. Pathology showed a dermoid cyst with abundant neutrophil infiltrations. Ó 2017 Elsevier Ltd. All rights reserved.
1. Introduction Intracranial DCs are rare, benign, congenital lesions, and they represent 0.04–0.6% of intracranial tumors [1]. Most of them develop along the midline structures, whereas the epidermoid cysts are away from the midline [2]. Most intracranial DCs have fairly typical features and locations, which allows the radiological characteristics to facilitate the diagnosis and to help in operative planning [2]. Nevertheless, intrasellar DCs have been reported infrequently. To date, only four cases have been reported in the international English publications [3–6]. Moreover, intrasellar DC with sudden-onset symptoms has never been described in the literature. In this report, we describe a case of an intrasellar DC in an elderly woman with sudden-onset symptoms mimicking pituitary apoplexy, along with a literature review.
2. Case report A 69 year-old woman presented to our clinic with a sudden onset of headache, dizziness, and bilaterally decreased visual acuity for 2 days. Physical examination showed blurring of visual acuity, ⇑ Corresponding author at: Department of Neurosurgery, Ninth People’s Hospital, Shanghai Jiao Tong University School of Medicine, No. 280 Mo-He Road, Shanghai 201900, PR China. E-mail address: [email protected] (D.-F. Feng).
but no other focal neurological deficit. Ophthalmological assessment revealed her vision to be 0.3 in the right eye and 0.25 in the left. Perimetry showed no visual field defect in either eye. Funduscopic examination was normal. Endocrine workup showed levels of pituitary hormones were within normal limits. CT scan of brain showed a mixed hyper- and hypo-dense sellar regional lesion (Fig. 1), which seemed to originate from the pituitary gland. Sellar regional MRI demonstrated a 2.6 cm 2.8 cm 2.5 cm intrasellar lesion with slight suprasellar extension that was mixed hyper and hypointense signal on T1-weighted and T2-weighted images. Obvious gadolinium enhancement was noted at the periphery. The optic chiasm was compressed (Fig. 2). The clinical, radiological, and endocrinological characteristics indicated a nonsecreting macroadenoma. Consequently, we considered the most likely diagnosis of pituitary macroadenoma apoplexy with cystic change. We also performed a CT angiogram, which excluded the presence of intracranial aneurysms. After got the informed consent of the patient, we took her to surgery for transnasal–sphenoidal resection of the mass. After incision of the mass, a significant amount of whitish-yellow purulent material was aspirated under pressure. subsequently, all foreign material was eliminated for decompression, and the mass was partially removed because part of the mass was fixed to surrounding neurovascular structures and pituitary. In addition, surgical field was washed with normal saline after the mass was resected. The microbiological cultures and smear examination of the pus
http://dx.doi.org/10.1016/j.jocn.2017.05.023 0967-5868/Ó 2017 Elsevier Ltd. All rights reserved.
Please cite this article in press as: Pan Y-B et al. Intrasellar dermoid cyst mimicking pituitary apoplexy: A case report and review of the literature. J Clin Neurosci (2017), http://dx.doi.org/10.1016/j.jocn.2017.05.023
2
Y.-B. Pan et al. / Journal of Clinical Neuroscience xxx (2017) xxx–xxx
provided negative results. Histopathological examination of the specimen showed a DC with abundant neutrophil infiltrations (Fig. 3). The postoperative course was uneventful, and the headache and blurring of visual acuity were significantly improved. The patient was discharged home on the day 9 post surgery without any new neurological deficit. At 9 months’ follow-up, the patient presented with no signs of recurrence. 3. Discussion
Fig. 1. Axial CT image showing a mixed hyper- and hypo-dense intrasellar lesion.
Intracranial DCs are benign, slowly growing lesions. They are thought to arise from the ectopic ectodermal remnants that are incorporated in the neural tube during its closure from the 3rd to 5th month of embryonic development [7]. DC has a fibrous capsule composed of stratified squamous epithelium, and the cyst contains dermal appendages such as hair follicles, sebaceous glands, or sweat glands [2]. About two-thirds of them are located in the posterior fossa [8]. However, intrasellar DCs are relatively rare. To the best of our knowledge, only four cases of intrasellar DCs have been reported to date [3–6]. The reported cases of intrasellar DCs and clinical and radiological characteristics are summarized in Table 1. The clinical manifestations of patients with intracranial DCs depend on the location of the cyst, its size, and whether or not it has ruptured [9–11]. The major clinical features of an intrasellar mass are quite characteristic of intrasellar DCs, and include endocrinologic abnormalities, visual field defects, and blurring of visual acuity [12]. Arseni et al. [5] described a case of intrasellar
Fig. 2. Preoperative MRI of the patient. (A–C): Coronal T1-weighted MR image (A), coronal T2-weighted MR image (B) and sagittal T1-weighted MR image (C) showing an intrasellar lesion measuring approximately 2.6 cm 2.8 cm 2.5 cm, with slight suprasellar extension. The left part of the lesion (arrows) on coronal section displayed hypointensity on T1-weighted MRI and hyperintensity on T2-weighted MRI. The upper right part of the lesion (arrowheads) showed hyperintensity on T1-weighted MRI and hypointensity on T2-weighted MRI. The optic chiasm (curved arrow) was compressed. D: contrast-enhanced coronal T1-weighted images showing obvious enhancement at the periphery.
Please cite this article in press as: Pan Y-B et al. Intrasellar dermoid cyst mimicking pituitary apoplexy: A case report and review of the literature. J Clin Neurosci (2017), http://dx.doi.org/10.1016/j.jocn.2017.05.023
3
Y.-B. Pan et al. / Journal of Clinical Neuroscience xxx (2017) xxx–xxx
Fig. 3. Histopathology of the dermoid cyst. (A) The capsule of the dermoid cyst was lined with stratified squamous epithelium. (B–C) the cyst contained sweat glands and sebaceous glands. (D) A large amount of neutrophils (arrow). H & E, original magnification 200 (A–D).
Table 1 Review of intrasellar dermoid cysts, listed in historical order. Authors/year
Age/sex
Signs and symptoms
Arseni et al. [5]
52/M
Gradually decreased visual acuity for 1 year, anosmia, central facial paresis on the right, marked visual field deficit 4-month history of decreased libido, secondary amenorrhea, emotional lability and insomnia, three episodes of transient bitemporal hemianopsia, milky discharge from the right breast Short stature (height 111 cm) had a weight of 19 kg and a bone age of 5 years
Klonoff et al. [6]
43/F
Madhusudhan et al. [3]
11/M
Mistry et al. [4]
38/M
Left temporal visual field deficit, gynecomastia
Current case
69/F
Worsening severe headache, dizziness and diminution of visual acuity for 2 days
Endocrine workup
CT
MR T1
T2
Enhancing
Treatment
Recurrence/ Follow-up (mo)
Normal
NA
NA
NA
NA
Total removal
NA/ NA
Elevated serum prolactin level
Hypodensity
Hypo
NA
NA
Incomplete removal
No/58
Low levels of all the anterior pituitary hormones Mildly elevated serum prolactin level Normal
NA
Hyper
NA
NA
NA
NA/ NA
NA
NA
NA
NA
Total removal
NA/ NA
Hyperdensity/ hypodensity
Hyper/ hypo
Hyper/ hypo
Obvious enhancement at the periphery
Incomplete removal
No/9
FU, follow-up; NA, not available; mo, month; hyper, hyperintense; hypo, hypointense.
DC with suprasellar extension in which the patient presented with gradually decreased visual acuity for 1 year and marked visual field. Klonoff et al. [6] reported another case of intrasellar DC with suprasellar extension, which was marked by galactorrhea,
amenorrhea, and hyperprolactinemia, along with three episodes of transient bitemporal hemianopsia. The authors suggested that hyperprolactinemia and galactorrhea were probably caused by the compressive effect of the pituitary stalk, which decreased
Please cite this article in press as: Pan Y-B et al. Intrasellar dermoid cyst mimicking pituitary apoplexy: A case report and review of the literature. J Clin Neurosci (2017), http://dx.doi.org/10.1016/j.jocn.2017.05.023
4
Y.-B. Pan et al. / Journal of Clinical Neuroscience xxx (2017) xxx–xxx
inhibition of prolactin release [6]. We reported our case of intrasellar DC with slight suprasellar extension in which the elderly woman experienced sudden severe headache and dizziness with diminution of visual acuity, mimicking pituitary apoplexy. Although major features of intracranial DCs include headaches, the onset was not sudden. Conversely, they manifested gradually because of the linear growth pattern of the intracranial DCs [2,13]. In addition, sudden severe headache was probably caused by rupture of intracranial DCs [14]. In our case, however, neither punctate foci of T1 hyperintensity within the subarachnoid space nor fluid-fluid level in the ventricles could be found radiologically (Fig. 2). On examination, decreased serum T3, T4, FT3, and FT4 levels with all normal pituitary hormone levels were found. The features of the suddenonset symptoms and absence of endocrine manifestations were part of the reason why we initially considered that pituitary macroadenoma apoplexy was the most likely diagnosis. Radiologically, DCs are generally hypodense on CT scan, and they usually present as homogeneous hyperintensity on T1-weighted MRI sequences with hypo-signal to mixed signal on T2-weighted imaging because of abundant lipid in the cysts. Furthermore, absence of peripheral edema, no enhancement on contrast, and avascular pattern on digital subtraction angiography are other radiological features [2]. In our case, the radiological manifestations were quite atypical (Fig. 2). Preoperatively, the left part of the mass on coronal section displayed hypointensity on T1weighted MRI and hyperintensity on T2-weighted MRI, which was considered to be cystic change in pituitary adenoma (Fig. 2). In addition, the upper right part of the mass showed hyperdensity on CT scan (Fig. 1), hyperintensity on T1-weighted MRI, and hypointensity on T2-weighted MRI, which was thought to be subacute hemorrhage (Fig. 2). Thus, these findings in association with the apoplectiform symptoms led us to make a radiological diagnosis of pituitary macroadenoma apoplexy with cystic change. However, no blood clot was found intraoperatively. Moreover, a large amount of whitish-yellow purulent material was aspirated under pressure after incision of the mass, and pathology showed a DC with abundant neutrophil infiltrations (Fig. 3), which indicated acute inflammation in cyst, accounting for the obvious enhancement at the periphery to contrast on MRI. The hyperdense part seen on CT scans was thought to be due to the intermixture of microcalcification, partially liquefied cholesterol, saponification of keratinized debris, or lipid and proteinaceous content [15]. Above all, the patient’s sudden-onset symptoms were unlikely to be related to the bleeding episode or due to tumor growth because of the linear growth pattern of intracranial DCs. On the other hand, the complete blood count and routine biochemical profile were normal. Moreover, microbiological cultures was negative, because of which we considered it a localized chemical inflammation probably caused by necrotic material in cyst. Our patient may have had an asymptomatic preexisting intrasellar DC, and then material in cyst or tissue of cyst showed necrosis, which caused acute exudative inflammation. In addition, the sudden-onset symptoms in our patient may be explained by the compression on the dura mater because of the increasing volume of inflammatory exudate.
We simply eliminated the contents of the mass for decompression and partially removed the mass via the transnasal-sphenoidal approach given the risk of neurovascular and hypophyseal injury due to a portion of the mass being fixed to the surrounding neurovascular structures and pituitary. 4. Conclusions Intrasellar DCs are rare lesions that sometimes can be mistaken for pituitary adenoma because of uncharacteristic images and clinical manifestations. Our case emphasizes the importance of considering intrasellar DC as a differential diagnosis for sudden headache and visual diminution in elderly patients. Conflicts of interest None. Acknowledgement This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. References [1] Raghunath A, Indira Devi B, Bhat DI, et al. Dermoid cysts of the posterior fossa– morbid associations of a benign lesion. Br J Neurosurg 2013;27:765–71. [2] Akdemir G, Daglioglu E, Ergungor MF. Dermoid lesion of the cavernous sinus: case report and review of the literature. Neurosurg Rev 2004;27:294–8. [3] Madhusudhan KS, Kandpal H. Pituitary stalk duplication with intrasellar dermoid and herniation of the third ventricle. Pediatric Radiol 2009;39:1013. [4] Mistry D, Figueroa B. An elongated pituitary stalk resembling the lining of a dermoid cyst during endoscopic endonasal approach. Otolaryngol Head Neck Surg 2015;153:150–1. [5] Arseni C, Danaila L, Constantinescu AI, et al. Cerebral dermoid tumours. Neurochirurgia 1976;19:104–14. [6] Klonoff DC, Kahn DG, Rosenzweig W, et al. Hyperprolactinemia in a patient with a pituitary and an ovarian dermoid tumor: case report. Neurosurgery 1990;26:335–9. [7] Salcini C, Hatiloglu D, Evrensel A, et al. Episodic headache due to ruptured intracranial dermoid cyst. BMJ Case Rep 2015:2015. [8] Orakcioglu B, Halatsch ME, Fortunati M, et al. Intracranial dermoid cysts: variations of radiological and clinical features. Acta Neurochir 2008;150:1227–34. [9] Lunardi P, Missori P. Supratentorial dermoid cysts. J Neurosurg 1991;75:262–6. [10] Rubin G, Scienza R, Pasqualin A, et al. Craniocerebral epidermoids and dermoids. A review of 44 cases. Acta Neurochir 1989;97:1–16. [11] Chen JC, Chen Y, Lin SM, et al. Sylvian fissure dermoid cyst with intratumoral hemorrhage: case report. Clin Neurol Neurosurg 2005;108:63–6. [12] Bang M, Suh JH, Park JB, et al. Pure intrasellar meningioma mimicking pituitary macroadenoma: magnetic resonance imaging and review of the literature. World Neurosurg 2016;91(675):e1–4. [13] Fanous AA, Gupta P, Li V. Analysis of the growth pattern of a dermoid cyst. J Neurosurg Pediatr 2014;14:621–5. [14] Indulkar S, Hsich GE. Spontaneous rupture of intracranial dermoid cyst in a child. Neurology 2011;77:2070. [15] Wang X, Yu Y, Zhang X, et al. Unusual imaging appearance of a huge intracranial dermoid cyst located across the anterior and middle skull base. J Neurol Surg 2013;74(Suppl 1):e185–7.
Please cite this article in press as: Pan Y-B et al. Intrasellar dermoid cyst mimicking pituitary apoplexy: A case report and review of the literature. J Clin Neurosci (2017), http://dx.doi.org/10.1016/j.jocn.2017.05.023
Contents lists available at ScienceDirect
Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn
Case report
Intrasellar dermoid cyst mimicking pituitary apoplexy: A case report and review of the literature Yuan-Bo Pan, Zhao-Liang Sun, Dong-Fu Feng ⇑ Department of Neurosurgery, Ninth People’s Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, PR China
a r t i c l e
i n f o
Article history: Received 14 February 2017 Accepted 22 May 2017 Available online xxxx Keywords: Dermoid cyst Intracranial Pituitary apoplexy Tumor
a b s t r a c t Intrasellar dermoid cysts are extremely unusual lesions, with only four cases reported to date, and have not been previously reported in association with sudden-onset symptoms. Here, we present the case of an intrasellar dermoid cyst with sudden-onset symptoms mimicking pituitary apoplexy in an elderly woman. A 69 year-old woman presented with sudden onset of headache, dizziness, and decreased visual acuity. Magnetic resonance imaging of the sellar region showed an intrasellar lesion, which showed mixed hyper- and hypointense signal on T1-weighted and T2-weighted images and enhanced peripherally. Endocrine workup showed pituitary hormones within normal levels. According to these findings, the initial diagnosis of nonsecreting pituitary macroadenoma apoplexy was made. Intraoperatively, a large amount of whitish-yellow purulent material was found in the mass and the lesion was partially removed, owing to tight adhesion between remanent mass and surrounding neurovascular structures. Pathology showed a dermoid cyst with abundant neutrophil infiltrations. Ó 2017 Elsevier Ltd. All rights reserved.
1. Introduction Intracranial DCs are rare, benign, congenital lesions, and they represent 0.04–0.6% of intracranial tumors [1]. Most of them develop along the midline structures, whereas the epidermoid cysts are away from the midline [2]. Most intracranial DCs have fairly typical features and locations, which allows the radiological characteristics to facilitate the diagnosis and to help in operative planning [2]. Nevertheless, intrasellar DCs have been reported infrequently. To date, only four cases have been reported in the international English publications [3–6]. Moreover, intrasellar DC with sudden-onset symptoms has never been described in the literature. In this report, we describe a case of an intrasellar DC in an elderly woman with sudden-onset symptoms mimicking pituitary apoplexy, along with a literature review.
2. Case report A 69 year-old woman presented to our clinic with a sudden onset of headache, dizziness, and bilaterally decreased visual acuity for 2 days. Physical examination showed blurring of visual acuity, ⇑ Corresponding author at: Department of Neurosurgery, Ninth People’s Hospital, Shanghai Jiao Tong University School of Medicine, No. 280 Mo-He Road, Shanghai 201900, PR China. E-mail address: [email protected] (D.-F. Feng).
but no other focal neurological deficit. Ophthalmological assessment revealed her vision to be 0.3 in the right eye and 0.25 in the left. Perimetry showed no visual field defect in either eye. Funduscopic examination was normal. Endocrine workup showed levels of pituitary hormones were within normal limits. CT scan of brain showed a mixed hyper- and hypo-dense sellar regional lesion (Fig. 1), which seemed to originate from the pituitary gland. Sellar regional MRI demonstrated a 2.6 cm 2.8 cm 2.5 cm intrasellar lesion with slight suprasellar extension that was mixed hyper and hypointense signal on T1-weighted and T2-weighted images. Obvious gadolinium enhancement was noted at the periphery. The optic chiasm was compressed (Fig. 2). The clinical, radiological, and endocrinological characteristics indicated a nonsecreting macroadenoma. Consequently, we considered the most likely diagnosis of pituitary macroadenoma apoplexy with cystic change. We also performed a CT angiogram, which excluded the presence of intracranial aneurysms. After got the informed consent of the patient, we took her to surgery for transnasal–sphenoidal resection of the mass. After incision of the mass, a significant amount of whitish-yellow purulent material was aspirated under pressure. subsequently, all foreign material was eliminated for decompression, and the mass was partially removed because part of the mass was fixed to surrounding neurovascular structures and pituitary. In addition, surgical field was washed with normal saline after the mass was resected. The microbiological cultures and smear examination of the pus
http://dx.doi.org/10.1016/j.jocn.2017.05.023 0967-5868/Ó 2017 Elsevier Ltd. All rights reserved.
Please cite this article in press as: Pan Y-B et al. Intrasellar dermoid cyst mimicking pituitary apoplexy: A case report and review of the literature. J Clin Neurosci (2017), http://dx.doi.org/10.1016/j.jocn.2017.05.023
2
Y.-B. Pan et al. / Journal of Clinical Neuroscience xxx (2017) xxx–xxx
provided negative results. Histopathological examination of the specimen showed a DC with abundant neutrophil infiltrations (Fig. 3). The postoperative course was uneventful, and the headache and blurring of visual acuity were significantly improved. The patient was discharged home on the day 9 post surgery without any new neurological deficit. At 9 months’ follow-up, the patient presented with no signs of recurrence. 3. Discussion
Fig. 1. Axial CT image showing a mixed hyper- and hypo-dense intrasellar lesion.
Intracranial DCs are benign, slowly growing lesions. They are thought to arise from the ectopic ectodermal remnants that are incorporated in the neural tube during its closure from the 3rd to 5th month of embryonic development [7]. DC has a fibrous capsule composed of stratified squamous epithelium, and the cyst contains dermal appendages such as hair follicles, sebaceous glands, or sweat glands [2]. About two-thirds of them are located in the posterior fossa [8]. However, intrasellar DCs are relatively rare. To the best of our knowledge, only four cases of intrasellar DCs have been reported to date [3–6]. The reported cases of intrasellar DCs and clinical and radiological characteristics are summarized in Table 1. The clinical manifestations of patients with intracranial DCs depend on the location of the cyst, its size, and whether or not it has ruptured [9–11]. The major clinical features of an intrasellar mass are quite characteristic of intrasellar DCs, and include endocrinologic abnormalities, visual field defects, and blurring of visual acuity [12]. Arseni et al. [5] described a case of intrasellar
Fig. 2. Preoperative MRI of the patient. (A–C): Coronal T1-weighted MR image (A), coronal T2-weighted MR image (B) and sagittal T1-weighted MR image (C) showing an intrasellar lesion measuring approximately 2.6 cm 2.8 cm 2.5 cm, with slight suprasellar extension. The left part of the lesion (arrows) on coronal section displayed hypointensity on T1-weighted MRI and hyperintensity on T2-weighted MRI. The upper right part of the lesion (arrowheads) showed hyperintensity on T1-weighted MRI and hypointensity on T2-weighted MRI. The optic chiasm (curved arrow) was compressed. D: contrast-enhanced coronal T1-weighted images showing obvious enhancement at the periphery.
Please cite this article in press as: Pan Y-B et al. Intrasellar dermoid cyst mimicking pituitary apoplexy: A case report and review of the literature. J Clin Neurosci (2017), http://dx.doi.org/10.1016/j.jocn.2017.05.023
3
Y.-B. Pan et al. / Journal of Clinical Neuroscience xxx (2017) xxx–xxx
Fig. 3. Histopathology of the dermoid cyst. (A) The capsule of the dermoid cyst was lined with stratified squamous epithelium. (B–C) the cyst contained sweat glands and sebaceous glands. (D) A large amount of neutrophils (arrow). H & E, original magnification 200 (A–D).
Table 1 Review of intrasellar dermoid cysts, listed in historical order. Authors/year
Age/sex
Signs and symptoms
Arseni et al. [5]
52/M
Gradually decreased visual acuity for 1 year, anosmia, central facial paresis on the right, marked visual field deficit 4-month history of decreased libido, secondary amenorrhea, emotional lability and insomnia, three episodes of transient bitemporal hemianopsia, milky discharge from the right breast Short stature (height 111 cm) had a weight of 19 kg and a bone age of 5 years
Klonoff et al. [6]
43/F
Madhusudhan et al. [3]
11/M
Mistry et al. [4]
38/M
Left temporal visual field deficit, gynecomastia
Current case
69/F
Worsening severe headache, dizziness and diminution of visual acuity for 2 days
Endocrine workup
CT
MR T1
T2
Enhancing
Treatment
Recurrence/ Follow-up (mo)
Normal
NA
NA
NA
NA
Total removal
NA/ NA
Elevated serum prolactin level
Hypodensity
Hypo
NA
NA
Incomplete removal
No/58
Low levels of all the anterior pituitary hormones Mildly elevated serum prolactin level Normal
NA
Hyper
NA
NA
NA
NA/ NA
NA
NA
NA
NA
Total removal
NA/ NA
Hyperdensity/ hypodensity
Hyper/ hypo
Hyper/ hypo
Obvious enhancement at the periphery
Incomplete removal
No/9
FU, follow-up; NA, not available; mo, month; hyper, hyperintense; hypo, hypointense.
DC with suprasellar extension in which the patient presented with gradually decreased visual acuity for 1 year and marked visual field. Klonoff et al. [6] reported another case of intrasellar DC with suprasellar extension, which was marked by galactorrhea,
amenorrhea, and hyperprolactinemia, along with three episodes of transient bitemporal hemianopsia. The authors suggested that hyperprolactinemia and galactorrhea were probably caused by the compressive effect of the pituitary stalk, which decreased
Please cite this article in press as: Pan Y-B et al. Intrasellar dermoid cyst mimicking pituitary apoplexy: A case report and review of the literature. J Clin Neurosci (2017), http://dx.doi.org/10.1016/j.jocn.2017.05.023
4
Y.-B. Pan et al. / Journal of Clinical Neuroscience xxx (2017) xxx–xxx
inhibition of prolactin release [6]. We reported our case of intrasellar DC with slight suprasellar extension in which the elderly woman experienced sudden severe headache and dizziness with diminution of visual acuity, mimicking pituitary apoplexy. Although major features of intracranial DCs include headaches, the onset was not sudden. Conversely, they manifested gradually because of the linear growth pattern of the intracranial DCs [2,13]. In addition, sudden severe headache was probably caused by rupture of intracranial DCs [14]. In our case, however, neither punctate foci of T1 hyperintensity within the subarachnoid space nor fluid-fluid level in the ventricles could be found radiologically (Fig. 2). On examination, decreased serum T3, T4, FT3, and FT4 levels with all normal pituitary hormone levels were found. The features of the suddenonset symptoms and absence of endocrine manifestations were part of the reason why we initially considered that pituitary macroadenoma apoplexy was the most likely diagnosis. Radiologically, DCs are generally hypodense on CT scan, and they usually present as homogeneous hyperintensity on T1-weighted MRI sequences with hypo-signal to mixed signal on T2-weighted imaging because of abundant lipid in the cysts. Furthermore, absence of peripheral edema, no enhancement on contrast, and avascular pattern on digital subtraction angiography are other radiological features [2]. In our case, the radiological manifestations were quite atypical (Fig. 2). Preoperatively, the left part of the mass on coronal section displayed hypointensity on T1weighted MRI and hyperintensity on T2-weighted MRI, which was considered to be cystic change in pituitary adenoma (Fig. 2). In addition, the upper right part of the mass showed hyperdensity on CT scan (Fig. 1), hyperintensity on T1-weighted MRI, and hypointensity on T2-weighted MRI, which was thought to be subacute hemorrhage (Fig. 2). Thus, these findings in association with the apoplectiform symptoms led us to make a radiological diagnosis of pituitary macroadenoma apoplexy with cystic change. However, no blood clot was found intraoperatively. Moreover, a large amount of whitish-yellow purulent material was aspirated under pressure after incision of the mass, and pathology showed a DC with abundant neutrophil infiltrations (Fig. 3), which indicated acute inflammation in cyst, accounting for the obvious enhancement at the periphery to contrast on MRI. The hyperdense part seen on CT scans was thought to be due to the intermixture of microcalcification, partially liquefied cholesterol, saponification of keratinized debris, or lipid and proteinaceous content [15]. Above all, the patient’s sudden-onset symptoms were unlikely to be related to the bleeding episode or due to tumor growth because of the linear growth pattern of intracranial DCs. On the other hand, the complete blood count and routine biochemical profile were normal. Moreover, microbiological cultures was negative, because of which we considered it a localized chemical inflammation probably caused by necrotic material in cyst. Our patient may have had an asymptomatic preexisting intrasellar DC, and then material in cyst or tissue of cyst showed necrosis, which caused acute exudative inflammation. In addition, the sudden-onset symptoms in our patient may be explained by the compression on the dura mater because of the increasing volume of inflammatory exudate.
We simply eliminated the contents of the mass for decompression and partially removed the mass via the transnasal-sphenoidal approach given the risk of neurovascular and hypophyseal injury due to a portion of the mass being fixed to the surrounding neurovascular structures and pituitary. 4. Conclusions Intrasellar DCs are rare lesions that sometimes can be mistaken for pituitary adenoma because of uncharacteristic images and clinical manifestations. Our case emphasizes the importance of considering intrasellar DC as a differential diagnosis for sudden headache and visual diminution in elderly patients. Conflicts of interest None. Acknowledgement This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. References [1] Raghunath A, Indira Devi B, Bhat DI, et al. Dermoid cysts of the posterior fossa– morbid associations of a benign lesion. Br J Neurosurg 2013;27:765–71. [2] Akdemir G, Daglioglu E, Ergungor MF. Dermoid lesion of the cavernous sinus: case report and review of the literature. Neurosurg Rev 2004;27:294–8. [3] Madhusudhan KS, Kandpal H. Pituitary stalk duplication with intrasellar dermoid and herniation of the third ventricle. Pediatric Radiol 2009;39:1013. [4] Mistry D, Figueroa B. An elongated pituitary stalk resembling the lining of a dermoid cyst during endoscopic endonasal approach. Otolaryngol Head Neck Surg 2015;153:150–1. [5] Arseni C, Danaila L, Constantinescu AI, et al. Cerebral dermoid tumours. Neurochirurgia 1976;19:104–14. [6] Klonoff DC, Kahn DG, Rosenzweig W, et al. Hyperprolactinemia in a patient with a pituitary and an ovarian dermoid tumor: case report. Neurosurgery 1990;26:335–9. [7] Salcini C, Hatiloglu D, Evrensel A, et al. Episodic headache due to ruptured intracranial dermoid cyst. BMJ Case Rep 2015:2015. [8] Orakcioglu B, Halatsch ME, Fortunati M, et al. Intracranial dermoid cysts: variations of radiological and clinical features. Acta Neurochir 2008;150:1227–34. [9] Lunardi P, Missori P. Supratentorial dermoid cysts. J Neurosurg 1991;75:262–6. [10] Rubin G, Scienza R, Pasqualin A, et al. Craniocerebral epidermoids and dermoids. A review of 44 cases. Acta Neurochir 1989;97:1–16. [11] Chen JC, Chen Y, Lin SM, et al. Sylvian fissure dermoid cyst with intratumoral hemorrhage: case report. Clin Neurol Neurosurg 2005;108:63–6. [12] Bang M, Suh JH, Park JB, et al. Pure intrasellar meningioma mimicking pituitary macroadenoma: magnetic resonance imaging and review of the literature. World Neurosurg 2016;91(675):e1–4. [13] Fanous AA, Gupta P, Li V. Analysis of the growth pattern of a dermoid cyst. J Neurosurg Pediatr 2014;14:621–5. [14] Indulkar S, Hsich GE. Spontaneous rupture of intracranial dermoid cyst in a child. Neurology 2011;77:2070. [15] Wang X, Yu Y, Zhang X, et al. Unusual imaging appearance of a huge intracranial dermoid cyst located across the anterior and middle skull base. J Neurol Surg 2013;74(Suppl 1):e185–7.
Please cite this article in press as: Pan Y-B et al. Intrasellar dermoid cyst mimicking pituitary apoplexy: A case report and review of the literature. J Clin Neurosci (2017), http://dx.doi.org/10.1016/j.jocn.2017.05.023