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The second case of de novo intracranial germinoma association with Klinefelter's syndrome
Surgical Neurology 66 (2006) 332 – 340 www.surgicalneurology-online.com
Letters to the Editor The second case of de novo intracranial germinoma association with Klinefelter’s syndrome Re: De novo germinoma in the brain in association of Klinefleter’s Syndrome: case report and review of literature (Kaido et al, Surg Neurol 2003;60:553-9). I report on a patient diagnosed with Klinefelter’s syndrome (47, XXY) when he was 29 years old and presented to medical attention for his tall stature and mild mental retardation, but the hormonal profiles were unusual with typical hypergonadotrophic hypogonadism. Instead, this patient had multiple pituitary dysfunction. He had very low follicle-stimulating hormone, luteinizing hormone, low baseline cortisol, and no response to insulin-induced hypoglycemia, borderline free thyroid hormone with normal thyrotropin. Prolactin and growth hormone levels were normal. Testosterone was low and estradiol was very high. I thought germ cell tumor that produces gonadotropin or estrogen was the differential diagnosis for the odd hormonal profiles. During workup, the patient developed diabetes insipidus, and hence magnetic resonance imaging of the brain was performed, which revealed moderate hyperintense posterior pituitary mass with crowded pituitary stalk. Transsphenoidal mass removal was done and pathologic examination reported dysgerminoma. The patient was to receive chemotherapy. This was a very rare case, so I searched the literature and found that Kaido et al had reported a case of de novo intracranial germinoma association with Klinefelter’s syndrome in Surgical Neurology. This is now the second case of intracranial germinoma without previous history of germ cell tumor and is the first case present with diabetes insipidus. I agree with the author about the association between Klinefelter’ syndrome and such type of tumor. The theory of germ cell dysmigration and the role of extra X chromosome appear appropriate. Environmental factors may play a role as this patient, as well as the previous case, was from an Asian population. I would also like to alert clinicians to recognize germ cell tumor as a cause of hypopituitarism and diabetes insipidus in Klinefelter’s syndrome. Prasit Phowthongkum, MD Faculty of Medicine Chulalongkorn University Bangkok, Thailand DOI of original article:10.1016/j.surneu.2005.11.034 doi:10.1016/j.surneu.2006.05.041 0090-3019/$ – see front matter D 2006 Elsevier Inc. All rights reserved.
Response We thank Dr Phowthongkum for his interest in our case and for emphasizing the association of Klinefelter’s syndrome and germ cell tumor. The case of Dr Phowthongkum is the eighth case in which an intracranial germ cell tumor occurred in association with Klinefelter’s syndrome. De novo, however, means no confirmation of existence in prior examinations. Our case had revealed no brain tumor with magnetic resonance imaging 2 years before onset of hemiparesis. Magnetic resonance imaging at the onset demonstrated a mass in the temporal lobe and hypothalamus. We, therefore, cannot agree that the case of Dr Phowthongkum is the second case of de novo intracranial germinoma association with Klinefelter’s syndrome. Takanobu Kaido, MD, PhD Department of Neurosurgery Musashi Hospital National Center of Neurology and Psychiatry Tokyo, 187-8551 Japan doi:10.1016/j.surneu.2006.06.040
Does abdominal aorta clamping, as a method of spinal ischemia in rats, really work? Re: Small petrosal approach to the middle portion of the mediobasal temporal region: technical case report (Tu¨re U and Pamir MN. Surg Neurol 2004;61:60-7). Over the years, several animal models of spinal cord ischemia have been used to study its pathophysiology, neurologic sequelae, and neuroprotective strategies. Abdominal aortic cross-clamping, which was used by Usul et al [3], is one of the easiest models. However, consistent reproducibility of neurologic deficits and stimulation of human disease remain to be major concerns. Thus, the aim of this letter is 2-fold: (1) to test the hypothesis that clamping abdominal aorta results in spinal cord ischemic injury and (2) to explain reasons behind the findings. Sixteen male rats weighing 150 to 200 g were randomly divided into 2 groups: sham-operated group and crossclamped group. In the cross-clamped group, animals were anesthetized and subjected to laparotomy. A Yasargil aneurysm clip was placed just distal to the renal arteries across the aorta and vena cava and then removed after 45 minutes [3]. Then, the abdomen was closed appropriately
Letters to the Editor The second case of de novo intracranial germinoma association with Klinefelter’s syndrome Re: De novo germinoma in the brain in association of Klinefleter’s Syndrome: case report and review of literature (Kaido et al, Surg Neurol 2003;60:553-9). I report on a patient diagnosed with Klinefelter’s syndrome (47, XXY) when he was 29 years old and presented to medical attention for his tall stature and mild mental retardation, but the hormonal profiles were unusual with typical hypergonadotrophic hypogonadism. Instead, this patient had multiple pituitary dysfunction. He had very low follicle-stimulating hormone, luteinizing hormone, low baseline cortisol, and no response to insulin-induced hypoglycemia, borderline free thyroid hormone with normal thyrotropin. Prolactin and growth hormone levels were normal. Testosterone was low and estradiol was very high. I thought germ cell tumor that produces gonadotropin or estrogen was the differential diagnosis for the odd hormonal profiles. During workup, the patient developed diabetes insipidus, and hence magnetic resonance imaging of the brain was performed, which revealed moderate hyperintense posterior pituitary mass with crowded pituitary stalk. Transsphenoidal mass removal was done and pathologic examination reported dysgerminoma. The patient was to receive chemotherapy. This was a very rare case, so I searched the literature and found that Kaido et al had reported a case of de novo intracranial germinoma association with Klinefelter’s syndrome in Surgical Neurology. This is now the second case of intracranial germinoma without previous history of germ cell tumor and is the first case present with diabetes insipidus. I agree with the author about the association between Klinefelter’ syndrome and such type of tumor. The theory of germ cell dysmigration and the role of extra X chromosome appear appropriate. Environmental factors may play a role as this patient, as well as the previous case, was from an Asian population. I would also like to alert clinicians to recognize germ cell tumor as a cause of hypopituitarism and diabetes insipidus in Klinefelter’s syndrome. Prasit Phowthongkum, MD Faculty of Medicine Chulalongkorn University Bangkok, Thailand DOI of original article:10.1016/j.surneu.2005.11.034 doi:10.1016/j.surneu.2006.05.041 0090-3019/$ – see front matter D 2006 Elsevier Inc. All rights reserved.
Response We thank Dr Phowthongkum for his interest in our case and for emphasizing the association of Klinefelter’s syndrome and germ cell tumor. The case of Dr Phowthongkum is the eighth case in which an intracranial germ cell tumor occurred in association with Klinefelter’s syndrome. De novo, however, means no confirmation of existence in prior examinations. Our case had revealed no brain tumor with magnetic resonance imaging 2 years before onset of hemiparesis. Magnetic resonance imaging at the onset demonstrated a mass in the temporal lobe and hypothalamus. We, therefore, cannot agree that the case of Dr Phowthongkum is the second case of de novo intracranial germinoma association with Klinefelter’s syndrome. Takanobu Kaido, MD, PhD Department of Neurosurgery Musashi Hospital National Center of Neurology and Psychiatry Tokyo, 187-8551 Japan doi:10.1016/j.surneu.2006.06.040
Does abdominal aorta clamping, as a method of spinal ischemia in rats, really work? Re: Small petrosal approach to the middle portion of the mediobasal temporal region: technical case report (Tu¨re U and Pamir MN. Surg Neurol 2004;61:60-7). Over the years, several animal models of spinal cord ischemia have been used to study its pathophysiology, neurologic sequelae, and neuroprotective strategies. Abdominal aortic cross-clamping, which was used by Usul et al [3], is one of the easiest models. However, consistent reproducibility of neurologic deficits and stimulation of human disease remain to be major concerns. Thus, the aim of this letter is 2-fold: (1) to test the hypothesis that clamping abdominal aorta results in spinal cord ischemic injury and (2) to explain reasons behind the findings. Sixteen male rats weighing 150 to 200 g were randomly divided into 2 groups: sham-operated group and crossclamped group. In the cross-clamped group, animals were anesthetized and subjected to laparotomy. A Yasargil aneurysm clip was placed just distal to the renal arteries across the aorta and vena cava and then removed after 45 minutes [3]. Then, the abdomen was closed appropriately