- Email: [email protected]
Sellar xanthogranuloma manifesting as obstructive hydrocephalus
Case Reports / Journal of Clinical Neuroscience 15 (2008) 929–933
The common primary locations of angiosarcoma are face and scalp, liver, trunk, heart, extremities, spleen, bone and retroperitoneal cavity. Primary cerebral angiosarcomas are rare.5 Naka et al.6 found that 5% of angiosarcomas metastasised to brain, of which face and scalp, and extremities were the most common primary sites. The most reported metastatic sites were lung, bone, liver, lymph nodes, adrenal gland, spleen and pleura. Except for malignant lymphoma, primary malignant tumours of the spleen are rare. Angiosarcoma, haemangiosarcoma, and haemangioendolethialsarcoma are most likely the same tumour,7 with angiosarcoma the term most widely used. The mean age of patients with splenic angiosarcoma is typically 50–60 years.2 The pathogenesis of splenic angiosarcoma is unknown. Primary splenic angiosarcoma has a poor prognosis. It usually disseminates after the diagnosis is made, and most patients die within 9 months.2 Prognosis may improve with splenectomy (26% live for 1–5 years);8 however, spontaneous rupture occurs in 32% of patients before resection.9 Of the previous reported six cases of brain metastasis from splenic angiosarcoma, the median onset of brain metastasis was 6 months from primary disease,1,2 with diagnosis confirmed on autopsy. Our patient is unusual with respect to the delay from primary presentation to brain metastasis, and she is the only reported patient with craniotomy and resection of metastasis.
929
The optimal therapy for brain metastasis of splenic angiosarcoma has not been established. Complete resection of the primary followed by radiation results in better longterm control of disease than surgery alone.4 References 1. Sondenaa K, Heikkila R, Nysted A, et al. Diagnosis of brain metastases from a primary hemangiosarcoma of the spleen with magnetic resonance imaging. Cancer 1993;71:138–41. 2. Neuhauser TS, Derringer GA, Thompson LD, et al. Splenic angiosarcoma: a clinicopathologic and immunophenotypic study of 28 cases. Mod Pathol 2000;13:978–87. 3. Tally PW, Laws ER, Scheithauer BW. Metastases of central nervous system neoplasms. Case report. J Neurosurg 1988;68:811–6. 4. Mark RJ, Poen JC, Tran LM, et al. Angiosarcoma – A report of 67 patients and a review of the literature. Cancer 1996;77: 2400–6. 5. Akutsu H, Tsuboi K, Sakamoto N, et al. Cerebral metastasis from angiosarcoma of the aortic wall: Case report. Surg Neurol 2004;61:68–71. 6. Naka N, Ohsawa M, Tomita Y, et al. Angiosarcoma in Japan – a review of 99 cases. Cancer 1995;75:989–96. 7. Morgenstern L, Rosenberg J, Geller SA. Tumors of the spleen. World J Surg 1985;9:468–76. 8. Smith VC, Eisenberg BL, McDonald EC. Primary splenic angiosarcoma – case report and literature review. Cancer 1985;55: 1625–7. 9. Wick MR, Scheithauer BW, Smith SL, et al. Primary non-lymphoreticular malignant neoplasms of the spleen. Am J Surg Pathol 1982;6:229–42.
doi:10.1016/j.jocn.2007.03.028
Sellar xanthogranuloma manifesting as obstructive hydrocephalus Zhuo-Hao Liu, Wen-Ching Tzaan, Yih-Ying Wu, Hsien-Chih Chen * Departments of Neurosurgery and Pathology, Chang Gung Memorial Hospital and Chang Gung University, 222 Mai-Chin Road, Keeling 204, Taiwan Received 4 March 2007; accepted 29 May 2007
Abstract Xanthogranuloma of the sellar region is rare. It typically causes symptoms of pituitary dysfunction or visual change due to compression of the optic chiasm. However, to the best of our knowledge, no case of xanthogranuloma-associated hydrocephalus has been documented in the literature. This study describes a 32-year-old man afflicted with xanthogranuloma of the sellar region who presented with acute change in consciousness and associated obstructive hydrocephalus. A literature review and discussion regarding therapeutic options for this condition are presented. Ó 2007 Elsevier Ltd. All rights reserved. Keywords: Xanthogranuloma; Sellar tumor; Hydrocephalus; Sellar xanthogranuloma
*
Corresponding author. Tel.: +886 2 24313131x2670; fax: +886 2 24332655. E-mail addresses: [email protected], [email protected] (H.-C. Chen).
930
Case Reports / Journal of Clinical Neuroscience 15 (2008) 929–933
1. Introduction Xanthogranuloma of the sellar region is rare.1–3 To the best of our knowledge, Paulus was the first clinician to describe and classify xanthogranuloma of the sellar region as a distinct entity in 1999, after which the World Health Organization (WHO) accepted xanthogranuloma as a specific brain tumor classification in 2000. Xanthogranuloma of the sellar region differs from classical craniopharyngioma in several ways; age at onset, anatomical site and localization of the lesion, and symptoms and prognosis.3 The prevalence of xanthogranulomas appears to peak at 20–40 years of age. Predominantly located within the intrasellar region, xanthogranulomas present with endocrinological symptoms, and patients typically experience favorable outcomes.3 Histopathologically, a xanthogranuloma of the sellar region is characterized by the presence of cholesterol-cleft granulomas and hemosiderin deposition.3 Herein, we report a patient with a histopathologically typical xanthogranuloma of the sellar region presenting with symptoms of change in consciousness associated with hydrocephalus. 2. Clinical presentation A 32-year-old man was sent to our emergency department for medical assessment due to fluctuating consciousness over the preceding 2 days. According to his family the patient had complained recently of blurred vision and insomnia, symptoms that had been controlled with hypnotic medication. Physical examination findings were unremarkable except for pronounced somnolence and confusion. The patient was non-cooperative for the assessment of gait, visual-field testing and all neurological examinations. A CT scan revealed of a sellar mass featuring an enlarged sella and asymmetric lateral ventricle dilatation (Fig. 1). That same day, MRI revealed a clearly defined, 3.4 3.8 4.2 cm well-defined lobuated cystic supra-sellar mass (Fig. 2). The lesion appeared hyperintense on T1- and T2-weighted images. A small focal lobule featuring T1- and T2-weighted hypointensity without enhancement was located at the base of this lesion. The lesion displaced the third ventricle posteriorly, resulting in obstruction of both foramina of Monro and non-communicating hydrocephalus. Endocrine assessment revealed a slightly elevated serum prolactin level (39.4 ng/mL), while other pituitary-function studies yielded normal results. On the following day, we removed the lesion via a transcranial approach under a presumptive diagnosis of pituitary adenoma, noting that the cystic tumor membrane was tightly adherent to the sellar-floor dura. The cyst, which contained yellow-brown fluid together with crystallike material, was drained, and a small tissue sample was taken for histological examination. Histopathologically, the tumor primarily consisted of cholesterol clefts with surrounding multinucleated foreign-body giant cells and xan-
Fig. 1. Axial cranial CT scan revealing a cystic hypodense mass with a hyperdense ‘‘wall” in the suprasellar region, which was associated with hydrocephalus.
thoma cells. Also present were hemosiderin depositions, focal necrotic debris and dystrophic calcification (Fig. 3). Considering the clinical data, including the patient’s age and tumor location, the tumor was diagnosed as a typical xanthogranuloma of the sellar region. Postoperatively, transient diabetes insipidus was observed. The patient’s level of consciousness and alertness improved substantially once his sodium serum level had normalized postoperatively. Ophthalmic follow-up during a 6-month period subsequent to surgery revealed improved visual acuity and visual fields. 3. Discussion Xanthogranulomas have been reported to occur at various sites inside and outside the cranial vault, including the middle ear, the mastoid bone, the paranasal sinuses and the choroid plexus.3 Xanthoma cells have been reported to be associated with certain systemic illnesses (e.g. ErdheimChester disease and Hand-Schuller-Christian disease),4,5 and have also been reported to be present in association with adamantinomatous craniopharyngioma.1,3 Intracranial xanthogranulomas arise most commonly in the choroid plexus, being almost uniformly located in the trigone of the lateral ventricle, whereas xanthogranulomas of the sellar region are quite rare. As a consequence of the infrequent occurrence of xanthogranulomas of the sellar region, there appears to exist an extremely limited understanding of this tumor’s natural history, pathogenesis and clinical presentation. Further, the primary nature and location of such lesions within the sellar region also appears to be poorly defined. In addition, it remains unclear whether this tumor is actually a secondary reaction to other lesions, for example Rathkes cysts, colloid cysts or, craniopharyngioma,3 or if it is, in
Case Reports / Journal of Clinical Neuroscience 15 (2008) 929–933
931
Fig. 2. MRIs showing a well-defined lobulated cystic hyperintense lesion. (a) Axial T1-weighted image. (b) Axial T2-weighted image revealing focal hypointensity at the tumor base. (c) T1-weighted image with contrast revealing no definite enhancement of tumor. (d) Sagittal T1-weighted image revealing posterior displacement of the third ventricle and midbrain.
Fig. 3. (a) The tumor consisted mainly of cholesterol cleft granulomas. (b) Surrounding the cholesterol clefts were multinucleated foreign body giant cells, xanthoma cells and lymphoplasma cells. (c) There was also hemosiderin deposition and (d) focal necrotic debris.
932
Case Reports / Journal of Clinical Neuroscience 15 (2008) 929–933
fact, a specific pathological entity. Although significant overlap exists as regards the histopathological findings for xanthomatous pituitary lesions,1 the histological findings in the present case were typical for the WHO brain tumor classification. A Medline English-language literature search commencing from the year 2000 revealed six separate reports of cases of sellar xanthogranuloma.1,4,6,7 A xanthogranuloma of the sellar region usually causes symptoms of an endocrinological nature,1,4,6,7 or when the lesion is of sufficient size, causes visual changes due to compression of the visual pathway.4,7 To the best of our knowledge, this is the first report in the literature of a xanthogranuloma arising within the sellar region and causing subacute progressive obtundation due to hydrocephalus. To the best of our knowledge, no typical radiological signs exist for xanthogranulomas.7 The cholesterol deposits of a xanthogranuloma appear hyperintense on T1-weighted imaging; however, calcification, focal hemorrhage and hemosiderin deposits can frequently result in heterogenous cystic lesions. For all six of the reviewed cases, MRIs were performed (Table 1). For five of the total of seven cases (including the present case) of xanthogranuloma, nonhomogenous lesions were seen on T1-weighted imaging with or without enhancement. These lesions varied from 1 to 4 cm in diameter. In the present case, differentiating xanthogranuloma of the sellar region from the other six possible diagnoses (craniopharyngioma, pituitary adenoma, Rathke’s cyst, malignant metastases, meningioma and hypophysitis), proved quite difficult. Thus, we suggest that an accurate preoperative diagnosis of xanthogranuloma remains very difficult.
The appropriate therapeutic strategy for most cases of xanthogranuloma of the sellar region appears to be first to correct the underlying endocrine problem. Surgical intervention is always necessary for successfully treating such a lesion. To this end, both transphenoidal and transcranial approaches may be used to access the mass. For our case, a transfrontal approach was used to access the lesion. Subsequent to the tumor having been removed, our patient gradually recovered consciousness. The application of a shunting procedure for acute hydrocephalus proved unnecessary for our patient once the mass had been removed. Furthermore, shunting prior to tumor removal may actually increase the difficulty of the surgical approach for lesion removal and increase the cerebrospinal fluid infection rate. It should be noted that sudden deterioration of neurological condition and/ or death can occur as a consequence of inhibited hypothalamic function. Thus, patient condition and consciousness should be monitored closely throughout the hospitalization period. In the six cases of xanthogranuloma reviewed from the literature, no examples of recurring xanthogranuloma of the sellar region were noted, and the overall outcome for patients with this condition appeared to be favorable. Our patient’s bitemporal anopia was examined postoperatively. Unfortunately, we were unable to determine whether our patient’s vision defect had arisen prior to surgical intervention or not, although his blurred vision improved gradually subsequent to discharge. In conclusion, this investigation documents one patient with a xanthogranuloma of the sellar region presenting with confusion and obstructive hydrocephalus. We have
Table 1 Reviewed cases of xanthogranuloma Author
Sex/ age
Symptoms
Clinical findings
Radiology findings (MRI)
Operative approach
Pathology
Follow up
Reithmeier et al.4
M/ 51
Unavailable
Transphenoidal
Xanthogranuloma
Doing well after 3 months
Burt et al.1
M/ 29
Transphenoidal
Xanthometous lesion of the pituitary
Doing well after 18 months
M/ 26
Lethargy, weight loss
Panhypopituitarism, mild hyperprolactinemia
T1-weighted hyperintense signal heterogeneous enhancement T1-weighted hyperintense signal, T2-weighted heterogeneous signal T1-, T2-weighted heterogeneous signal, heterogeneous enhancement T1-, T2-weighted hyperintense signal
Xanthogranuloma
M/ 67
Hypophyseal insufficiency, visual deficits Hyponatramia, panhypopituitarism
Transfrontal
Yonezawa et al.6
Pallor, loss of libido General fatigue, weight loss Headache, nausea
Transfrontal
M/ 57
Headache
Bitemporal hemianopia
Required medical supplement after 8 months Unavailable
M/5
Headache, weakness, loss of appetite Conscious change
Secondary adrenal insufficiency, diabetes insipidus, hypothyroidism Blurred vision, disorientation
Jung et al.7
Liu et al.
M/ 32
Hyponatramia, low thyroid hormone, low testosterone
T1-, T2-weighted hyperintense signal, heterogeneous enhancement T1-, T2-weighted heterogeneous signal
Transphenoidal
Xanthomatous lesion of the pituitary Xanthogranuloma
Transcranial
Xanthogranuloma
Unavailable
T1-, T2-weighted hyperintense signal
Transfrontal
Xanthogranuloma
Doing well, vision improvement after 6 months
Case Reports / Journal of Clinical Neuroscience 15 (2008) 933–937
demonstrated that a xanthogranuloma of the sellar region can be removed rapidly surgically, and thus shunting for hydrocephalus appears to be unnecessary. Because xanthogranulomas of the sellar region are rare, their nature and clinical course remain somewhat unclear. Thus, further reports are required in order for clinicians to gain greater insight into the clinical course and associated outcome. References 1. Burt MG, Morey AL, Turner JJ, et al. Xanthomatous pituitary lesions: A report of two cases and review of the literature. Pituitary 2003;6:161–8.
933
2. Freda PU, Post KD. Differential diagnosis of sellar masses. Endocrinol Metabol Clin N Am 1999;28:81–117. 3. Paulus W, Honegger J, Keyvani K, et al. Xanthogranuloma of the sellar region: A clinicopathological entity different from adamantinomatous craniopharyngioma. Acta Neuropathol 1999;97:377–82. 4. Reithmeier T, Trost HA, Wolf S, et al. Xanthogranuloma of the Erdheim-Chester type within the sellar region: Case report. Clin Neuropathol 2002;21:24–8. 5. Jamjoom ZA, Raina V, Al-Jamali A, et al. Intracranial xanthogranuloma of the dura in Hand-Schuller-Christian disease. J Neurosurg 1993;78:297–300. 6. Yonezawa K, Shirataki K, Sakagami Y, et al. Panhypopituitarism induced by cholesterol granuloma in the sellar region: Case report. Neurol Med Chir (Tokyo) 2003;43:259–62. 7. Jung CS, Schanzer A, Hattingen E, Plate KH, Seifert V. Xanthogranuloma of the sellar region. Acta Neurochir 2006;148:473–7.
doi:10.1016/j.jocn.2007.05.028
Idiopathic spinal cord herniation through the inner layer of duplicated anterior dura: Evaluation with high-resolution 3D MRI Masaki Ishida a,*, Masayuki Maeda a, Yuichi Kasai b, Atsumasa Uchida b, Kan Takeda a a
Department of Radiology, Mie University School of Medicine, 2-174 Edobashi, Tsu, Mie 514-8507, Japan b Department of Orthopedic Surgery, Mie University School of Medicine, Mie, Japan Received 6 July 2006; accepted 19 October 2006
Abstract We report a case of idiopathic spinal cord herniation through the inner layer of duplicated anterior dura. High-resolution threedimensional (3D) MRIs clearly depicted the duplicated dura mater ventral to the spinal cord and herniation of the cord through the inner layer of dura mater, which was confirmed at surgery. Presurgical high-resolution 3D MRI thus enables precise determination of the site of cord herniation in patients with this condition. Ó 2007 Elsevier Ltd. All rights reserved. Keywords: Spinal cord; Herniation; Magnetic resonance imaging
1. Introduction Although idiopathic spinal cord herniation through a dural defect is considered a rare neurosurgical entity associated with slowly progressive myelopathy, it has recently been increasingly reported and recognized clinically. The literature includes reports of about 90 such cases that have been treated and confirmed surgically. This condition involves herniation of the spinal cord through an anterior dural defect.1,2 Although the pathogenesis of the initial dural defect is not well understood, congenitally duplicated *
Corresponding author. Tel.: +81 59 231 5029; fax: +81 59 232 8066. E-mail address: [email protected] (M. Ishida).
anterior dura mater with a defect in the inner layer is a possible cause.3,4 It has been suggested that CT myelography is a more suitable method for detection of ventral spinal cord herniation than MRI.5,6 With recent advances in MRI hardware and software, high-resolution three-dimensional (3D) MRI techniques, such as fast imaging employing steady-state acquisition (3D-FIESTA), true fast imaging with steady-state precession (true-FISP), and the constructive interference in steady state (CISS) technique, have been used for assessment of small structures in patients with central nervous system diseases.7–11 We report a case of idiopathic spinal cord herniation through the inner layer of duplicated anterior dura evaluated using high-resolution 3D MRI.
The common primary locations of angiosarcoma are face and scalp, liver, trunk, heart, extremities, spleen, bone and retroperitoneal cavity. Primary cerebral angiosarcomas are rare.5 Naka et al.6 found that 5% of angiosarcomas metastasised to brain, of which face and scalp, and extremities were the most common primary sites. The most reported metastatic sites were lung, bone, liver, lymph nodes, adrenal gland, spleen and pleura. Except for malignant lymphoma, primary malignant tumours of the spleen are rare. Angiosarcoma, haemangiosarcoma, and haemangioendolethialsarcoma are most likely the same tumour,7 with angiosarcoma the term most widely used. The mean age of patients with splenic angiosarcoma is typically 50–60 years.2 The pathogenesis of splenic angiosarcoma is unknown. Primary splenic angiosarcoma has a poor prognosis. It usually disseminates after the diagnosis is made, and most patients die within 9 months.2 Prognosis may improve with splenectomy (26% live for 1–5 years);8 however, spontaneous rupture occurs in 32% of patients before resection.9 Of the previous reported six cases of brain metastasis from splenic angiosarcoma, the median onset of brain metastasis was 6 months from primary disease,1,2 with diagnosis confirmed on autopsy. Our patient is unusual with respect to the delay from primary presentation to brain metastasis, and she is the only reported patient with craniotomy and resection of metastasis.
929
The optimal therapy for brain metastasis of splenic angiosarcoma has not been established. Complete resection of the primary followed by radiation results in better longterm control of disease than surgery alone.4 References 1. Sondenaa K, Heikkila R, Nysted A, et al. Diagnosis of brain metastases from a primary hemangiosarcoma of the spleen with magnetic resonance imaging. Cancer 1993;71:138–41. 2. Neuhauser TS, Derringer GA, Thompson LD, et al. Splenic angiosarcoma: a clinicopathologic and immunophenotypic study of 28 cases. Mod Pathol 2000;13:978–87. 3. Tally PW, Laws ER, Scheithauer BW. Metastases of central nervous system neoplasms. Case report. J Neurosurg 1988;68:811–6. 4. Mark RJ, Poen JC, Tran LM, et al. Angiosarcoma – A report of 67 patients and a review of the literature. Cancer 1996;77: 2400–6. 5. Akutsu H, Tsuboi K, Sakamoto N, et al. Cerebral metastasis from angiosarcoma of the aortic wall: Case report. Surg Neurol 2004;61:68–71. 6. Naka N, Ohsawa M, Tomita Y, et al. Angiosarcoma in Japan – a review of 99 cases. Cancer 1995;75:989–96. 7. Morgenstern L, Rosenberg J, Geller SA. Tumors of the spleen. World J Surg 1985;9:468–76. 8. Smith VC, Eisenberg BL, McDonald EC. Primary splenic angiosarcoma – case report and literature review. Cancer 1985;55: 1625–7. 9. Wick MR, Scheithauer BW, Smith SL, et al. Primary non-lymphoreticular malignant neoplasms of the spleen. Am J Surg Pathol 1982;6:229–42.
doi:10.1016/j.jocn.2007.03.028
Sellar xanthogranuloma manifesting as obstructive hydrocephalus Zhuo-Hao Liu, Wen-Ching Tzaan, Yih-Ying Wu, Hsien-Chih Chen * Departments of Neurosurgery and Pathology, Chang Gung Memorial Hospital and Chang Gung University, 222 Mai-Chin Road, Keeling 204, Taiwan Received 4 March 2007; accepted 29 May 2007
Abstract Xanthogranuloma of the sellar region is rare. It typically causes symptoms of pituitary dysfunction or visual change due to compression of the optic chiasm. However, to the best of our knowledge, no case of xanthogranuloma-associated hydrocephalus has been documented in the literature. This study describes a 32-year-old man afflicted with xanthogranuloma of the sellar region who presented with acute change in consciousness and associated obstructive hydrocephalus. A literature review and discussion regarding therapeutic options for this condition are presented. Ó 2007 Elsevier Ltd. All rights reserved. Keywords: Xanthogranuloma; Sellar tumor; Hydrocephalus; Sellar xanthogranuloma
*
Corresponding author. Tel.: +886 2 24313131x2670; fax: +886 2 24332655. E-mail addresses: [email protected], [email protected] (H.-C. Chen).
930
Case Reports / Journal of Clinical Neuroscience 15 (2008) 929–933
1. Introduction Xanthogranuloma of the sellar region is rare.1–3 To the best of our knowledge, Paulus was the first clinician to describe and classify xanthogranuloma of the sellar region as a distinct entity in 1999, after which the World Health Organization (WHO) accepted xanthogranuloma as a specific brain tumor classification in 2000. Xanthogranuloma of the sellar region differs from classical craniopharyngioma in several ways; age at onset, anatomical site and localization of the lesion, and symptoms and prognosis.3 The prevalence of xanthogranulomas appears to peak at 20–40 years of age. Predominantly located within the intrasellar region, xanthogranulomas present with endocrinological symptoms, and patients typically experience favorable outcomes.3 Histopathologically, a xanthogranuloma of the sellar region is characterized by the presence of cholesterol-cleft granulomas and hemosiderin deposition.3 Herein, we report a patient with a histopathologically typical xanthogranuloma of the sellar region presenting with symptoms of change in consciousness associated with hydrocephalus. 2. Clinical presentation A 32-year-old man was sent to our emergency department for medical assessment due to fluctuating consciousness over the preceding 2 days. According to his family the patient had complained recently of blurred vision and insomnia, symptoms that had been controlled with hypnotic medication. Physical examination findings were unremarkable except for pronounced somnolence and confusion. The patient was non-cooperative for the assessment of gait, visual-field testing and all neurological examinations. A CT scan revealed of a sellar mass featuring an enlarged sella and asymmetric lateral ventricle dilatation (Fig. 1). That same day, MRI revealed a clearly defined, 3.4 3.8 4.2 cm well-defined lobuated cystic supra-sellar mass (Fig. 2). The lesion appeared hyperintense on T1- and T2-weighted images. A small focal lobule featuring T1- and T2-weighted hypointensity without enhancement was located at the base of this lesion. The lesion displaced the third ventricle posteriorly, resulting in obstruction of both foramina of Monro and non-communicating hydrocephalus. Endocrine assessment revealed a slightly elevated serum prolactin level (39.4 ng/mL), while other pituitary-function studies yielded normal results. On the following day, we removed the lesion via a transcranial approach under a presumptive diagnosis of pituitary adenoma, noting that the cystic tumor membrane was tightly adherent to the sellar-floor dura. The cyst, which contained yellow-brown fluid together with crystallike material, was drained, and a small tissue sample was taken for histological examination. Histopathologically, the tumor primarily consisted of cholesterol clefts with surrounding multinucleated foreign-body giant cells and xan-
Fig. 1. Axial cranial CT scan revealing a cystic hypodense mass with a hyperdense ‘‘wall” in the suprasellar region, which was associated with hydrocephalus.
thoma cells. Also present were hemosiderin depositions, focal necrotic debris and dystrophic calcification (Fig. 3). Considering the clinical data, including the patient’s age and tumor location, the tumor was diagnosed as a typical xanthogranuloma of the sellar region. Postoperatively, transient diabetes insipidus was observed. The patient’s level of consciousness and alertness improved substantially once his sodium serum level had normalized postoperatively. Ophthalmic follow-up during a 6-month period subsequent to surgery revealed improved visual acuity and visual fields. 3. Discussion Xanthogranulomas have been reported to occur at various sites inside and outside the cranial vault, including the middle ear, the mastoid bone, the paranasal sinuses and the choroid plexus.3 Xanthoma cells have been reported to be associated with certain systemic illnesses (e.g. ErdheimChester disease and Hand-Schuller-Christian disease),4,5 and have also been reported to be present in association with adamantinomatous craniopharyngioma.1,3 Intracranial xanthogranulomas arise most commonly in the choroid plexus, being almost uniformly located in the trigone of the lateral ventricle, whereas xanthogranulomas of the sellar region are quite rare. As a consequence of the infrequent occurrence of xanthogranulomas of the sellar region, there appears to exist an extremely limited understanding of this tumor’s natural history, pathogenesis and clinical presentation. Further, the primary nature and location of such lesions within the sellar region also appears to be poorly defined. In addition, it remains unclear whether this tumor is actually a secondary reaction to other lesions, for example Rathkes cysts, colloid cysts or, craniopharyngioma,3 or if it is, in
Case Reports / Journal of Clinical Neuroscience 15 (2008) 929–933
931
Fig. 2. MRIs showing a well-defined lobulated cystic hyperintense lesion. (a) Axial T1-weighted image. (b) Axial T2-weighted image revealing focal hypointensity at the tumor base. (c) T1-weighted image with contrast revealing no definite enhancement of tumor. (d) Sagittal T1-weighted image revealing posterior displacement of the third ventricle and midbrain.
Fig. 3. (a) The tumor consisted mainly of cholesterol cleft granulomas. (b) Surrounding the cholesterol clefts were multinucleated foreign body giant cells, xanthoma cells and lymphoplasma cells. (c) There was also hemosiderin deposition and (d) focal necrotic debris.
932
Case Reports / Journal of Clinical Neuroscience 15 (2008) 929–933
fact, a specific pathological entity. Although significant overlap exists as regards the histopathological findings for xanthomatous pituitary lesions,1 the histological findings in the present case were typical for the WHO brain tumor classification. A Medline English-language literature search commencing from the year 2000 revealed six separate reports of cases of sellar xanthogranuloma.1,4,6,7 A xanthogranuloma of the sellar region usually causes symptoms of an endocrinological nature,1,4,6,7 or when the lesion is of sufficient size, causes visual changes due to compression of the visual pathway.4,7 To the best of our knowledge, this is the first report in the literature of a xanthogranuloma arising within the sellar region and causing subacute progressive obtundation due to hydrocephalus. To the best of our knowledge, no typical radiological signs exist for xanthogranulomas.7 The cholesterol deposits of a xanthogranuloma appear hyperintense on T1-weighted imaging; however, calcification, focal hemorrhage and hemosiderin deposits can frequently result in heterogenous cystic lesions. For all six of the reviewed cases, MRIs were performed (Table 1). For five of the total of seven cases (including the present case) of xanthogranuloma, nonhomogenous lesions were seen on T1-weighted imaging with or without enhancement. These lesions varied from 1 to 4 cm in diameter. In the present case, differentiating xanthogranuloma of the sellar region from the other six possible diagnoses (craniopharyngioma, pituitary adenoma, Rathke’s cyst, malignant metastases, meningioma and hypophysitis), proved quite difficult. Thus, we suggest that an accurate preoperative diagnosis of xanthogranuloma remains very difficult.
The appropriate therapeutic strategy for most cases of xanthogranuloma of the sellar region appears to be first to correct the underlying endocrine problem. Surgical intervention is always necessary for successfully treating such a lesion. To this end, both transphenoidal and transcranial approaches may be used to access the mass. For our case, a transfrontal approach was used to access the lesion. Subsequent to the tumor having been removed, our patient gradually recovered consciousness. The application of a shunting procedure for acute hydrocephalus proved unnecessary for our patient once the mass had been removed. Furthermore, shunting prior to tumor removal may actually increase the difficulty of the surgical approach for lesion removal and increase the cerebrospinal fluid infection rate. It should be noted that sudden deterioration of neurological condition and/ or death can occur as a consequence of inhibited hypothalamic function. Thus, patient condition and consciousness should be monitored closely throughout the hospitalization period. In the six cases of xanthogranuloma reviewed from the literature, no examples of recurring xanthogranuloma of the sellar region were noted, and the overall outcome for patients with this condition appeared to be favorable. Our patient’s bitemporal anopia was examined postoperatively. Unfortunately, we were unable to determine whether our patient’s vision defect had arisen prior to surgical intervention or not, although his blurred vision improved gradually subsequent to discharge. In conclusion, this investigation documents one patient with a xanthogranuloma of the sellar region presenting with confusion and obstructive hydrocephalus. We have
Table 1 Reviewed cases of xanthogranuloma Author
Sex/ age
Symptoms
Clinical findings
Radiology findings (MRI)
Operative approach
Pathology
Follow up
Reithmeier et al.4
M/ 51
Unavailable
Transphenoidal
Xanthogranuloma
Doing well after 3 months
Burt et al.1
M/ 29
Transphenoidal
Xanthometous lesion of the pituitary
Doing well after 18 months
M/ 26
Lethargy, weight loss
Panhypopituitarism, mild hyperprolactinemia
T1-weighted hyperintense signal heterogeneous enhancement T1-weighted hyperintense signal, T2-weighted heterogeneous signal T1-, T2-weighted heterogeneous signal, heterogeneous enhancement T1-, T2-weighted hyperintense signal
Xanthogranuloma
M/ 67
Hypophyseal insufficiency, visual deficits Hyponatramia, panhypopituitarism
Transfrontal
Yonezawa et al.6
Pallor, loss of libido General fatigue, weight loss Headache, nausea
Transfrontal
M/ 57
Headache
Bitemporal hemianopia
Required medical supplement after 8 months Unavailable
M/5
Headache, weakness, loss of appetite Conscious change
Secondary adrenal insufficiency, diabetes insipidus, hypothyroidism Blurred vision, disorientation
Jung et al.7
Liu et al.
M/ 32
Hyponatramia, low thyroid hormone, low testosterone
T1-, T2-weighted hyperintense signal, heterogeneous enhancement T1-, T2-weighted heterogeneous signal
Transphenoidal
Xanthomatous lesion of the pituitary Xanthogranuloma
Transcranial
Xanthogranuloma
Unavailable
T1-, T2-weighted hyperintense signal
Transfrontal
Xanthogranuloma
Doing well, vision improvement after 6 months
Case Reports / Journal of Clinical Neuroscience 15 (2008) 933–937
demonstrated that a xanthogranuloma of the sellar region can be removed rapidly surgically, and thus shunting for hydrocephalus appears to be unnecessary. Because xanthogranulomas of the sellar region are rare, their nature and clinical course remain somewhat unclear. Thus, further reports are required in order for clinicians to gain greater insight into the clinical course and associated outcome. References 1. Burt MG, Morey AL, Turner JJ, et al. Xanthomatous pituitary lesions: A report of two cases and review of the literature. Pituitary 2003;6:161–8.
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2. Freda PU, Post KD. Differential diagnosis of sellar masses. Endocrinol Metabol Clin N Am 1999;28:81–117. 3. Paulus W, Honegger J, Keyvani K, et al. Xanthogranuloma of the sellar region: A clinicopathological entity different from adamantinomatous craniopharyngioma. Acta Neuropathol 1999;97:377–82. 4. Reithmeier T, Trost HA, Wolf S, et al. Xanthogranuloma of the Erdheim-Chester type within the sellar region: Case report. Clin Neuropathol 2002;21:24–8. 5. Jamjoom ZA, Raina V, Al-Jamali A, et al. Intracranial xanthogranuloma of the dura in Hand-Schuller-Christian disease. J Neurosurg 1993;78:297–300. 6. Yonezawa K, Shirataki K, Sakagami Y, et al. Panhypopituitarism induced by cholesterol granuloma in the sellar region: Case report. Neurol Med Chir (Tokyo) 2003;43:259–62. 7. Jung CS, Schanzer A, Hattingen E, Plate KH, Seifert V. Xanthogranuloma of the sellar region. Acta Neurochir 2006;148:473–7.
doi:10.1016/j.jocn.2007.05.028
Idiopathic spinal cord herniation through the inner layer of duplicated anterior dura: Evaluation with high-resolution 3D MRI Masaki Ishida a,*, Masayuki Maeda a, Yuichi Kasai b, Atsumasa Uchida b, Kan Takeda a a
Department of Radiology, Mie University School of Medicine, 2-174 Edobashi, Tsu, Mie 514-8507, Japan b Department of Orthopedic Surgery, Mie University School of Medicine, Mie, Japan Received 6 July 2006; accepted 19 October 2006
Abstract We report a case of idiopathic spinal cord herniation through the inner layer of duplicated anterior dura. High-resolution threedimensional (3D) MRIs clearly depicted the duplicated dura mater ventral to the spinal cord and herniation of the cord through the inner layer of dura mater, which was confirmed at surgery. Presurgical high-resolution 3D MRI thus enables precise determination of the site of cord herniation in patients with this condition. Ó 2007 Elsevier Ltd. All rights reserved. Keywords: Spinal cord; Herniation; Magnetic resonance imaging
1. Introduction Although idiopathic spinal cord herniation through a dural defect is considered a rare neurosurgical entity associated with slowly progressive myelopathy, it has recently been increasingly reported and recognized clinically. The literature includes reports of about 90 such cases that have been treated and confirmed surgically. This condition involves herniation of the spinal cord through an anterior dural defect.1,2 Although the pathogenesis of the initial dural defect is not well understood, congenitally duplicated *
Corresponding author. Tel.: +81 59 231 5029; fax: +81 59 232 8066. E-mail address: [email protected] (M. Ishida).
anterior dura mater with a defect in the inner layer is a possible cause.3,4 It has been suggested that CT myelography is a more suitable method for detection of ventral spinal cord herniation than MRI.5,6 With recent advances in MRI hardware and software, high-resolution three-dimensional (3D) MRI techniques, such as fast imaging employing steady-state acquisition (3D-FIESTA), true fast imaging with steady-state precession (true-FISP), and the constructive interference in steady state (CISS) technique, have been used for assessment of small structures in patients with central nervous system diseases.7–11 We report a case of idiopathic spinal cord herniation through the inner layer of duplicated anterior dura evaluated using high-resolution 3D MRI.