- Email: [email protected]
Primary cystic germinoma originating from the midbrain
832
Case Reports / Journal of Clinical Neuroscience 16 (2009) 832–834
Primary cystic germinoma originating from the midbrain Jun Maruya a,*, Eriko Narita a, Keiichi Nishimaki a, Joichi Heianna b, Takaharu Miyauchi b, Takashi Minakawa a a b
Department of Neurosurgery, Akita Red Cross Hospital, 222-1 Nawashirosawa, Saruta, Kamikitate, Akita 010-1495, Japan Department of Radiology, Akita Red Cross Hospital, Akita, Japan
a r t i c l e
i n f o
Article history: Received 5 July 2008 Accepted 19 August 2008
Keywords: Biopsy Cyst Germinoma Midbrain
a b s t r a c t A primary intracranial germinoma that involves the midbrain is rare. We describe an unusual case of primary cystic germinoma originating from the midbrain. A 29-year-old man presented with diplopia, and his MRI showed a cystic, ring-like enhanced lesion in the thalamo–mesencephalic junction. Open biopsy was performed and the diagnosis of germinoma was based upon the histopathological findings. Following chemotherapy and radiotherapy, the symptoms improved and the tumor disappeared. We propose that primary intracranial germinoma should be included in the differential diagnosis of midbrain tumors, because early diagnosis and appropriate treatment for midbrain germinoma improves clinical outcome. Ó 2008 Elsevier Ltd. All rights reserved.
1. Introduction
3. Discussion
Intracranial germinoma usually arises in the pineal or suprasellar region. However, there are other sites of origin, such as the basal ganglia,1 thalamus,1 medulla oblongata,2 and cerebellopontine angle.3 Primary midbrain germinoma is rare, and only four prior cases of pathologically proven primary intracranial germinoma involving the midbrain have been reported.4–7 We describe an unusual case of cystic germinoma originating from the thalamo mesencephalic junction.
Germinoma may spread from a primary tumor in the pineal region to the floor of the third ventricle via the cerebrospinal fluid, and infiltrate the hypothalamus, thalamus, basal ganglia, or pituitary stalk. However, in our present case, the midbrain lesion was considered the primary tumor because the initial MRI disclosed no additional lesion. Furthermore, the angiography disclosed no displacement of the medial posterior choroidal artery and the pineal body was intraoperatively intact. Thus, there was no indication of a pineal mass. There are only five reports of primary midbrain germinoma, including our case (Table 1).4–7 All of these patients were comparatively older (15 29 years of age, mean age 24.4 years) than those with germinomas in the pineal or suprasellar region. Additionally, all five patients were males, suggesting a male dominance for midbrain germinoma. This may be attributed to an earlier closure of anterior neuropores in males than in females.7 Although most patients with germinomas of the basal ganglia and thalamus initially present with hemiparesis,1,8 reported patients with germinomas localized in the upper midbrain or thalamo mesencephalic junction initially presented with Parinaud’s syndrome and nystagmus.4,6 As the tumor enlarged, the thalamus or pons became involved, and hemiparesis or cranial nerve signs appeared.5,7 Gait disturbances or signs of increased intracranial pressure also developed when hydrocephalus occurred.5 In three of the five cases the initial MRIs showed that the tumors had cystic components.5,6 In two cases of mainly cystic tumors, intratumoral hemorrhages may have occurred because the presence of intracystic xanthochromic fluid was confirmed by diagnostic procedures.6 Cystic change and intratumoral hemorrhage are considered common in parenchymal germinomas.8 We propose that the differential diagnosis of a midbrain lesion should include germinoma, as well as glioma, malignant lymphoma, primitive neuroectodermal tumor, metastatic brain tumor, multiple sclerosis, and neuroepithelial cyst.4–7 Germinoma is difficult to differentiate from the other lesions solely by imaging findings. Therefore, histological diagnosis is strongly recommended. In three of the previously reported cases of midbrain germinoma, stereotactic biopsies were performed4,5,7 and in one case neuroendoscopic biopsy was performed.6 In general, stereotactic biopsy, which has a low incidence of morbidity, has diagnostic significance in the differential diagnosis of midbrain lesions. Neuroendoscopic biopsy is also a safe way to obtain a good tissue sample, particu-
2. Case report A 29-year-old man with an 8-month history of diplopia was admitted to our hospital. Neurological examination revealed conjugate upward gaze paresis and retraction nystagmus. His initial MRI showed a cystic, ring-like enhanced lesion in the left thalamo mesencephalic junction (Fig. 1). Hydrocephalus was not evident, and no abnormally enhanced lesions were found in the pineal or suprasellar regions. The angiography showed no tumor stain or displacement of the medial posterior choroidal artery. Laboratory study revealed normal levels of tumor markers including alphafetoprotein, human chorionic gonadotropin, and carcinoembryonic antigen. An open biopsy was performed via the occipital transtentorial approach. Opening of the arachnoid folds of the quadrigeminal cistern exposed the dorsal midbrain, and revealed the pale surface color of the left side and the wall of the cystic lesion. Additionally, the pineal body was seen to be intact intraoperatively. The cystic lesion was punctured and xanthochromic fluid was aspirated. Several specimens were collected for histological examination. Histopathological diagnosis of pure germinoma was confirmed by hematoxylin and eosin staining (Fig. 2A). Immunohistochemical staining showed tumor cells that were positive for placental alkaline phosphatase (Fig. 2B). Postoperatively, three courses of combination chemotherapy using carboplatin and etoposide were administered. After two courses of chemotherapy, whole ventricular radiotherapy was performed using a total dose of 24 Gy. After these treatments, symptoms improved and an MRI showed complete remission of the tumor (Fig. 3). Five years postoperatively, the patient did not show any evidence of local recurrences or metastasis. * Corresponding author. Tel.: +81 18 829 5000; fax: +81 18 829 5255. E-mail address: [email protected] (J. Maruya).
Case Reports / Journal of Clinical Neuroscience 16 (2009) 832–834
833
Fig. 1. Axial (A, B) and sagittal (C) T1-weighted MRI with gadolinium obtained upon admission. The MRI demonstrates a cystic mass lesion in the left thalamo mesencephalic junction with ring-like enhancement. Hydrocephalus is not evident, and there is no abnormally enhanced lesion in the pineal or suprasellar region.
Fig. 2. (A) Photomicrograph of a biopsy specimen showing a typical two-cell pattern germinoma consisting of large primitive germ cells and lymphocytes (hematoxylin and eosin stain, 400). (B) Cell membrane of the large cells showing immunohistochemical staining with placental alkaline phosphatase (placental alkaline phosphatase stain, 400).
Fig. 3. Axial (A, B) and sagittal (C) T1-weighted MRI with gadolinium obtained 1 month after chemotherapy and radiotherapy. The MRI shows complete remission of the cystic mass lesion in the left thalamo mesencephalic junction.
larly in cases of midbrain lesions that protrude into the ventricles. Additionally, application of neuroendoscopic surgery could allow surgeons to perform an endoscopic third ventriculostomy for hydrocephalus.6 All five cases of midbrain germinoma were patho-
logically diagnosed as pure germinoma, and all were treated successfully.4–7 Based on these findings, we can conclude that midbrain germinoma responds well to treatment, as do other intracranial germinomas.
834
Case Reports / Journal of Clinical Neuroscience 16 (2009) 834–837
Table 1 Clinical features of reported cases of germinomas involving the midbrain Authors (year)
Age/ Sex
Matsumoto et al. 27/M (1998)4 Ben Amor et al. 15/M (2004)5
Uchino et al. (2006)6
22/M
Koizumi et al. (2006)7
29/M
Present case
29/M
Symptom
Location
Lesion
Parinaud’s syndrome Nystagmus Right hemiparesis Gait disturbance Left abducens palsy Parinaud’s syndrome Personality changes Parinaud’s syndrome Nystagmus
Left upper midbrain
Solid
Bilateral midbrain thalamus
Solid and Cystic degeneration
Left midbrain thalamus
Cyst
Left pons
Solid
MRI-guided, stereotactic biopsy
Cyst
Open biopsy
Oculomotor palsy Abducens palsy Right hemiparesis Ataxia Neurogenic bladder Parinaud’s syndrome Nystagmus
thalamus
Left midbrain thalamus
The clinical and radiological features of midbrain germinoma show some differences from classical pineal germinoma, but the prognosis of midbrain germinoma is probably as good as for classical germinoma. Although midbrain germinoma is rare, primary intracranial germinoma should be included in the differential diagnosis of midbrain tumors. Additionally, biopsy continues to be important in the early diagnosis of such lesions. Furthermore, appropriate treatment has been shown to improve clinical outcome.
References
Hydrocephalus Diagnostic procedure
Treatment
Outcome
+
MRI-guided, stereotactic biopsy Stereotactic biopsy
Radiotherapy
Alive at 1 month Chemotherapy Alive at 1 year with radiotherapy
+
Neuroendoscopic biopsy
Chemotherapy with radiotherapy Chemotherapy with radiotherapy
Alive at 6 months Alive at 7 months
Chemotherapy Alive at 5 with years radiotherapy
2. Saito A, Yamashita T, Ishiwata Y, et al. A case of germinoma of the fourth ventricle. Shoni No Noshinkei 1983;8:43–8. 3. Nagendran K, Rice-Edwards M, Kendall B, et al. Germinoma in the cerebellopontine angle. J Neurol Neurosurg Psychiatry 1985;48: 955–6. 4. Matsumoto K, Tabuchi A, Tamesa N, et al. Primary intracranial germinoma involving the midbrain. Clin Neurol Neurosurg 1998;100:292–5. 5. Ben Amor S, Siddiqui K, Baessa S. Primary midbrain germinoma. Br J Neurosurg 2004;18:310–3. 6. Uchino M, Haga D, Mito T, et al. Primary midbrain cystic germinoma mimicking glioma: a case with neuroendoscopic biopsy. J Neurooncol 2006;79: 255–8. 7. Koizumi H, Oka H, Utsuki S, et al. Primary germinoma arising from the midbrain. Acta Neurochir (Wien) 2006;148:1197–200. 8. Kim CH, Paek SH, Park IA, et al. Cerebral germinoma with hemiatrophy of the brain: report of three cases. Acta Neurochir (Wien) 2002;144:145–50.
1. Kobayashi T, Kageyama N, Kida Y, et al. Unilateral germinomas involving the basal ganglia and thalamus. J Neurosurg 1981;55:55–62. doi:10.1016/j.jocn.2008.08.024
An atypical case of head trauma with late onset of contrecoup epidural hematoma, cerebellar contusion, and cerebral infarction in the territory of the recurrent artery of Heubner Shinsuke Sato, Tetsuryu Mitsuyama, Akira Ishii, Takakazu Kawamata * Department of Neurosurgery, Tokyo Women’s Medical University Yachiyo Medical Center, 477-96 Owada-Shinden, Yachiyo-shi, Chiba 276-8524, Japan
a r t i c l e
i n f o
Article history: Received 5 July 2008 Accepted 19 August 2008
Keywords: Acute epidural hematoma Cerebellar Contusion Contrecoup Heubner Infarction
a b s t r a c t We encountered a case of head trauma with very unusual delayed events. A 68-year-old woman was admitted to our hospital after receiving a direct impact to her occiput in a traffic accident. Head CT scans showed a thin acute epidural hematoma in the posterior fossa corresponding to a linear fracture, followed by late onset of contrecoup left frontal epidural hematoma and subsequent cerebellar contusion in the right cerebellar hemisphere. Fifteen days after the trauma, the patient developed mild motor weakness of right upper extremity. MRIs demonstrated an infarct in the territory of the left recurrent artery of Heubner. Although rare, atypical late events in patients with head trauma as reported here should be taken into consideration during subacute follow-up periods. Ó 2008 Elsevier Ltd. All rights reserved.
* Corresponding author. Tel.: +81 47 450 6000 (ext.7007); fax: +81 47 458 7047. E-mail address: [email protected] (T. Kawamata).
Case Reports / Journal of Clinical Neuroscience 16 (2009) 832–834
Primary cystic germinoma originating from the midbrain Jun Maruya a,*, Eriko Narita a, Keiichi Nishimaki a, Joichi Heianna b, Takaharu Miyauchi b, Takashi Minakawa a a b
Department of Neurosurgery, Akita Red Cross Hospital, 222-1 Nawashirosawa, Saruta, Kamikitate, Akita 010-1495, Japan Department of Radiology, Akita Red Cross Hospital, Akita, Japan
a r t i c l e
i n f o
Article history: Received 5 July 2008 Accepted 19 August 2008
Keywords: Biopsy Cyst Germinoma Midbrain
a b s t r a c t A primary intracranial germinoma that involves the midbrain is rare. We describe an unusual case of primary cystic germinoma originating from the midbrain. A 29-year-old man presented with diplopia, and his MRI showed a cystic, ring-like enhanced lesion in the thalamo–mesencephalic junction. Open biopsy was performed and the diagnosis of germinoma was based upon the histopathological findings. Following chemotherapy and radiotherapy, the symptoms improved and the tumor disappeared. We propose that primary intracranial germinoma should be included in the differential diagnosis of midbrain tumors, because early diagnosis and appropriate treatment for midbrain germinoma improves clinical outcome. Ó 2008 Elsevier Ltd. All rights reserved.
1. Introduction
3. Discussion
Intracranial germinoma usually arises in the pineal or suprasellar region. However, there are other sites of origin, such as the basal ganglia,1 thalamus,1 medulla oblongata,2 and cerebellopontine angle.3 Primary midbrain germinoma is rare, and only four prior cases of pathologically proven primary intracranial germinoma involving the midbrain have been reported.4–7 We describe an unusual case of cystic germinoma originating from the thalamo mesencephalic junction.
Germinoma may spread from a primary tumor in the pineal region to the floor of the third ventricle via the cerebrospinal fluid, and infiltrate the hypothalamus, thalamus, basal ganglia, or pituitary stalk. However, in our present case, the midbrain lesion was considered the primary tumor because the initial MRI disclosed no additional lesion. Furthermore, the angiography disclosed no displacement of the medial posterior choroidal artery and the pineal body was intraoperatively intact. Thus, there was no indication of a pineal mass. There are only five reports of primary midbrain germinoma, including our case (Table 1).4–7 All of these patients were comparatively older (15 29 years of age, mean age 24.4 years) than those with germinomas in the pineal or suprasellar region. Additionally, all five patients were males, suggesting a male dominance for midbrain germinoma. This may be attributed to an earlier closure of anterior neuropores in males than in females.7 Although most patients with germinomas of the basal ganglia and thalamus initially present with hemiparesis,1,8 reported patients with germinomas localized in the upper midbrain or thalamo mesencephalic junction initially presented with Parinaud’s syndrome and nystagmus.4,6 As the tumor enlarged, the thalamus or pons became involved, and hemiparesis or cranial nerve signs appeared.5,7 Gait disturbances or signs of increased intracranial pressure also developed when hydrocephalus occurred.5 In three of the five cases the initial MRIs showed that the tumors had cystic components.5,6 In two cases of mainly cystic tumors, intratumoral hemorrhages may have occurred because the presence of intracystic xanthochromic fluid was confirmed by diagnostic procedures.6 Cystic change and intratumoral hemorrhage are considered common in parenchymal germinomas.8 We propose that the differential diagnosis of a midbrain lesion should include germinoma, as well as glioma, malignant lymphoma, primitive neuroectodermal tumor, metastatic brain tumor, multiple sclerosis, and neuroepithelial cyst.4–7 Germinoma is difficult to differentiate from the other lesions solely by imaging findings. Therefore, histological diagnosis is strongly recommended. In three of the previously reported cases of midbrain germinoma, stereotactic biopsies were performed4,5,7 and in one case neuroendoscopic biopsy was performed.6 In general, stereotactic biopsy, which has a low incidence of morbidity, has diagnostic significance in the differential diagnosis of midbrain lesions. Neuroendoscopic biopsy is also a safe way to obtain a good tissue sample, particu-
2. Case report A 29-year-old man with an 8-month history of diplopia was admitted to our hospital. Neurological examination revealed conjugate upward gaze paresis and retraction nystagmus. His initial MRI showed a cystic, ring-like enhanced lesion in the left thalamo mesencephalic junction (Fig. 1). Hydrocephalus was not evident, and no abnormally enhanced lesions were found in the pineal or suprasellar regions. The angiography showed no tumor stain or displacement of the medial posterior choroidal artery. Laboratory study revealed normal levels of tumor markers including alphafetoprotein, human chorionic gonadotropin, and carcinoembryonic antigen. An open biopsy was performed via the occipital transtentorial approach. Opening of the arachnoid folds of the quadrigeminal cistern exposed the dorsal midbrain, and revealed the pale surface color of the left side and the wall of the cystic lesion. Additionally, the pineal body was seen to be intact intraoperatively. The cystic lesion was punctured and xanthochromic fluid was aspirated. Several specimens were collected for histological examination. Histopathological diagnosis of pure germinoma was confirmed by hematoxylin and eosin staining (Fig. 2A). Immunohistochemical staining showed tumor cells that were positive for placental alkaline phosphatase (Fig. 2B). Postoperatively, three courses of combination chemotherapy using carboplatin and etoposide were administered. After two courses of chemotherapy, whole ventricular radiotherapy was performed using a total dose of 24 Gy. After these treatments, symptoms improved and an MRI showed complete remission of the tumor (Fig. 3). Five years postoperatively, the patient did not show any evidence of local recurrences or metastasis. * Corresponding author. Tel.: +81 18 829 5000; fax: +81 18 829 5255. E-mail address: [email protected] (J. Maruya).
Case Reports / Journal of Clinical Neuroscience 16 (2009) 832–834
833
Fig. 1. Axial (A, B) and sagittal (C) T1-weighted MRI with gadolinium obtained upon admission. The MRI demonstrates a cystic mass lesion in the left thalamo mesencephalic junction with ring-like enhancement. Hydrocephalus is not evident, and there is no abnormally enhanced lesion in the pineal or suprasellar region.
Fig. 2. (A) Photomicrograph of a biopsy specimen showing a typical two-cell pattern germinoma consisting of large primitive germ cells and lymphocytes (hematoxylin and eosin stain, 400). (B) Cell membrane of the large cells showing immunohistochemical staining with placental alkaline phosphatase (placental alkaline phosphatase stain, 400).
Fig. 3. Axial (A, B) and sagittal (C) T1-weighted MRI with gadolinium obtained 1 month after chemotherapy and radiotherapy. The MRI shows complete remission of the cystic mass lesion in the left thalamo mesencephalic junction.
larly in cases of midbrain lesions that protrude into the ventricles. Additionally, application of neuroendoscopic surgery could allow surgeons to perform an endoscopic third ventriculostomy for hydrocephalus.6 All five cases of midbrain germinoma were patho-
logically diagnosed as pure germinoma, and all were treated successfully.4–7 Based on these findings, we can conclude that midbrain germinoma responds well to treatment, as do other intracranial germinomas.
834
Case Reports / Journal of Clinical Neuroscience 16 (2009) 834–837
Table 1 Clinical features of reported cases of germinomas involving the midbrain Authors (year)
Age/ Sex
Matsumoto et al. 27/M (1998)4 Ben Amor et al. 15/M (2004)5
Uchino et al. (2006)6
22/M
Koizumi et al. (2006)7
29/M
Present case
29/M
Symptom
Location
Lesion
Parinaud’s syndrome Nystagmus Right hemiparesis Gait disturbance Left abducens palsy Parinaud’s syndrome Personality changes Parinaud’s syndrome Nystagmus
Left upper midbrain
Solid
Bilateral midbrain thalamus
Solid and Cystic degeneration
Left midbrain thalamus
Cyst
Left pons
Solid
MRI-guided, stereotactic biopsy
Cyst
Open biopsy
Oculomotor palsy Abducens palsy Right hemiparesis Ataxia Neurogenic bladder Parinaud’s syndrome Nystagmus
thalamus
Left midbrain thalamus
The clinical and radiological features of midbrain germinoma show some differences from classical pineal germinoma, but the prognosis of midbrain germinoma is probably as good as for classical germinoma. Although midbrain germinoma is rare, primary intracranial germinoma should be included in the differential diagnosis of midbrain tumors. Additionally, biopsy continues to be important in the early diagnosis of such lesions. Furthermore, appropriate treatment has been shown to improve clinical outcome.
References
Hydrocephalus Diagnostic procedure
Treatment
Outcome
+
MRI-guided, stereotactic biopsy Stereotactic biopsy
Radiotherapy
Alive at 1 month Chemotherapy Alive at 1 year with radiotherapy
+
Neuroendoscopic biopsy
Chemotherapy with radiotherapy Chemotherapy with radiotherapy
Alive at 6 months Alive at 7 months
Chemotherapy Alive at 5 with years radiotherapy
2. Saito A, Yamashita T, Ishiwata Y, et al. A case of germinoma of the fourth ventricle. Shoni No Noshinkei 1983;8:43–8. 3. Nagendran K, Rice-Edwards M, Kendall B, et al. Germinoma in the cerebellopontine angle. J Neurol Neurosurg Psychiatry 1985;48: 955–6. 4. Matsumoto K, Tabuchi A, Tamesa N, et al. Primary intracranial germinoma involving the midbrain. Clin Neurol Neurosurg 1998;100:292–5. 5. Ben Amor S, Siddiqui K, Baessa S. Primary midbrain germinoma. Br J Neurosurg 2004;18:310–3. 6. Uchino M, Haga D, Mito T, et al. Primary midbrain cystic germinoma mimicking glioma: a case with neuroendoscopic biopsy. J Neurooncol 2006;79: 255–8. 7. Koizumi H, Oka H, Utsuki S, et al. Primary germinoma arising from the midbrain. Acta Neurochir (Wien) 2006;148:1197–200. 8. Kim CH, Paek SH, Park IA, et al. Cerebral germinoma with hemiatrophy of the brain: report of three cases. Acta Neurochir (Wien) 2002;144:145–50.
1. Kobayashi T, Kageyama N, Kida Y, et al. Unilateral germinomas involving the basal ganglia and thalamus. J Neurosurg 1981;55:55–62. doi:10.1016/j.jocn.2008.08.024
An atypical case of head trauma with late onset of contrecoup epidural hematoma, cerebellar contusion, and cerebral infarction in the territory of the recurrent artery of Heubner Shinsuke Sato, Tetsuryu Mitsuyama, Akira Ishii, Takakazu Kawamata * Department of Neurosurgery, Tokyo Women’s Medical University Yachiyo Medical Center, 477-96 Owada-Shinden, Yachiyo-shi, Chiba 276-8524, Japan
a r t i c l e
i n f o
Article history: Received 5 July 2008 Accepted 19 August 2008
Keywords: Acute epidural hematoma Cerebellar Contusion Contrecoup Heubner Infarction
a b s t r a c t We encountered a case of head trauma with very unusual delayed events. A 68-year-old woman was admitted to our hospital after receiving a direct impact to her occiput in a traffic accident. Head CT scans showed a thin acute epidural hematoma in the posterior fossa corresponding to a linear fracture, followed by late onset of contrecoup left frontal epidural hematoma and subsequent cerebellar contusion in the right cerebellar hemisphere. Fifteen days after the trauma, the patient developed mild motor weakness of right upper extremity. MRIs demonstrated an infarct in the territory of the left recurrent artery of Heubner. Although rare, atypical late events in patients with head trauma as reported here should be taken into consideration during subacute follow-up periods. Ó 2008 Elsevier Ltd. All rights reserved.
* Corresponding author. Tel.: +81 47 450 6000 (ext.7007); fax: +81 47 458 7047. E-mail address: [email protected] (T. Kawamata).