Neurosurg. Rev. 15 (1992) 139-142
A case o f Foster Kennedy syndrome without frontal lobe or anterior cranial fossa involvement Ahmet Yildizhan Department of Neurosurgery, Vakif Gureba Hospital, Istanbul, Turkey
Abstract
2 Case report
Foster Kennedy syndrome is a very rare syndrome which includes ipsilaterat optic atrophy and central scotoma, anosmia, contralateral papilledema, and, occasionally, ipsilateral proptosis. A large frontal lobe, olfactory groove, or medial third sphenoidal wing tumor, usually a meningioma, creates this syndrome. In this report, the author presents a case of metastastic cerebral tumor with Foster Kennedy syndrome but without frontal lobe or anterior cranial fossa involvement.
A 55-year-old female was admitted to the hospital on 12. 04. 1990 complaining of blindness in the right eye, headaches, nausea, vomiting, difficulty in swallowing, and epistaxis. The headaches and vision difficulties had started a year ago and the right eye had been blind for the last 8 months. Three months previously it had been noticed that her right eye was beginning to protrude. For about 1.5 months she had been vomiting, usually in the morning.
Keywords: Foster Kennedy syndrome, frontal lobe tumors, metastatic tumors.
1 Introduction The most common primary site for metastatic brain tumors is the lungs [1]. While many patients with carcinoma of the lung, melanoma, or cancer of the colon display multiple cerebral metastases, patients with cancer of the breast, renal carcinorna, ovarian carcinoma, and osteogenic sarcoma more frequently produce single metastases [3, 6, 7, 8, 9, 11, 13]. Malignant cells can reach the nervous system by various routes. Most commonly they spread hematogenically [1@ but they may also spread directly. Passage may occur from the nasopharynx or the lung into the cranium where they may encroach on the brain. The third possible route of dissemination is Batson's intraspinal plexus [5]. In this paper I present a patient with a metastatic tumor which has passed from the nasopharynx through the skull base and extended directly into the middle cranial fossa and brain. This tumor has caused a Foster Kennedy syndrome, without spreading to the anterior cranial fossa or frontal lobe. O 1992by Walter de Gruyter & Co. Berlin 9New York
A physical examination of the patient established that there had been a considerable weight loss. There was a 2 x 2 x 3 cm mass on the neck behind the fight angulus mandibulae which was sensitive to palpation. Examination of the throat revealed that the area around the right tonsils was swollen. Proptosis was present in the right eye (Figure 1, 2). A neurological examination showed that there was no sight in the right eye and ptosis was present. The right pupil was constantly dilated and there was no direct or indirect reaction to light. In the left eye there was also no indirect light reflex. There were no eye movements on the right. An examination of the optic fundus established papilledema on the left and optic atrophy on the right (Foster Kennedy syndrome) as well as anosmia on the right side. The patient had difficulty swallowing and no gag reflex. Blood and urine tests as well as plain chest x-rays were normal. Contrast enhanced computed tomography revealed a mass beginning in the right nasopharynx and extending through the skull base to the right middle cranial fossa and temporal lobe. This mass had also invaded the right orbita causing proptosis on the right but did not extend into the anterior cranial fossa and frontal lobe (Figures 3, 4, 5, 6).
140
Yildizhan, Foster Kennedy syndrome without frontal lobe
Figure 1. Photograph taken from the front showing ptosis and proptosis on patient's right side,
Figure 2. Photograph of the right side showing proptosis.
Figure 3. Contrast enhanced CT showing a mass origi-
Figure 4. Contrast enhanced CT showing the metastasis in the right middle cranial fossa and orbita.
nating in the fight nasopharynx invading the skull base and right orbita causing proptosis.
Figure 5. Contrast enhanced CT showing the tumor occupying the fight middle cranial fossa and basal cisterns.
Figure 6. Contrast enhanced CT showing the tumor's spread to the right temporal lobe but not invading the frontal lobe. Neurosurg. Rev. 15 (1992)
Yildizhan, Foster Kennedy syndrome without frontal lobe The patient was diagnosed as having a metastatic tumor. A biopsy was taken from the nasopharynx. Histopathological investigations revealed a carcinoma with slightly differentiated squamous cells. Dexamethasone relieved the patient partially. Radiotherapy was recommended but the patient refused this treatment. At her own request she was discharged from hospital.
3 Discussion Foster Kennedy syndrome is extremely rare. It occurs in only 1.5% of cases with masses situated in the anterior cranial fossa [14]. The syndrome is defined by ipsilateral central scotoma and primary optic atrophy on the side of the lesion and contralateral papilledema [2]. In addition, ipsilateral anosmia and/or proptosis may occasionally occur [10, 12]. Ipsilateral central scotoma and optic atrophy occur due to pressure on the optic nerve; contralateral papilledema occurs as a result of the increased intracranial pressure. Ipsilateral anosrnia is caused by pressure on the olfactory tract, and ipsilateral proptosis, which is rarely seen, develops with the tumor's invasion of the orbita [10]. Tumors of the frontal lobe, sphenoidal wing, or olfactory groove can cause this syndrome [15]. Meningiomas situated in the medial portion of the sphenoidal wing are the most frequent specific cause. Anosmia is not seen when the lesion is situated more laterally. However, anosmia does occur bilaterally when the tumor spreads from the
141 midline line in the anterior cranial fossa towards the opposite side [12]. In our patient, ipsilateral optic atrophy, anosmia and proptosis, as well as contralateral papilledema were all present. However, these symptoms were not caused by a tumor situated in the frontal lobe, anterior cranial fossa, or sphenoidal wing, but rather as a result of a nasopharyngeal carcinoma spreading directly from the skull base towards the middle cranial fossa and orbita. The tumor, which had invaded the optic nerve and olfactory tract from below on the right side, had created pressure and, as a consequence, optic atrophy and anosmia. The tumor had continued to advance from the optic nerve occupying the right orbit, thus causing proptosis. The tumor also invaded the basal cisterns and had broken the circulation of the cerebrospinal fluid. As a result, the intracranial pressure was increased, causing contralateral papilledema (Figures 5, 6), nausea and vomiting. Differential diagnosis of patients with these symptoms should consider ischemic optic neuropathy, diabetes mellitus, hypertensive cardiovascular disease, collagen vascular disease, syphilis, local arachnoiditis, and cranial arteritis [4]. None of the above were found in our patient. This syndrome suggests a mass in the areas of the frontal lobe, k n o w n as the brain's silent area. In our patient, however, this was not the case; Foster Kennedy syndrome had developed without frontal lobe or anterior cranial fossa involvement (Figure 6). This possibility should always be kept in mind during diagnosis and differential diagnosis.
References [1] G~.MACn~ FW JR, JB POSNER, RH PATTERSONJR: Metastatic brain tumors. In: YOUMANSJR (ed): Neurological surgery. WB Saunders, Philadelphia 1982 [2] KENNEDYF" Retrobutbar neuritis as an exact diagnostic sign of certain tumors and abscesses in the frontal lobes. Amer J Med Sci 142 (1911) 355-368 [3] LANG EF, J SEATER:Metastatic brain tumors. Resuits of surgical and non-surgical treatment. Surg Clin N Amer 44 (1964) 865-872 [4] Mc CRARYJA: Neurophthalmology. In: YOUMANS JR (eds): Neurological surgery. WB Saunders, Philadelphia 1982 [5] ONUIGBOWI: Batson's theory of vertebral venous metastasis. A review. Oncology 32 (1975) 145-150 (6] POSNERJB: Diagnosis and treatment of metastases to the brain. Clin Bull 4 (1974) 4 7 - 5 7 Neurosurg. Rev. 15 (1992)
[7] RANSOHOFFJ: Surgical management of metastatic tumors. Seminars Oncol 2 (1975) 2 1 - 2 7 [8] RASYdNDR, SR WEISS,RE WERMUTH:Single metastatic brain tumors. Treatment and follow-up in 41 cases. Amer Surg 35 (1969) 510-515 [9] RASKINDR, SR WEISS,JJ MANNING,RE WERMUTH: Survival after surgical excision of single metastatic brain tumors. Amer J Roentgen 111 (1971) 323328 [10] RENGACHARYSS: Cranial nerve examination. In: WmKINSRH, SS R~NGACHARY(eds): Neurosurgery. McGraw-Hill, New York 1985 [11] RICHARDS P, W MC KISSOCK: Intracranial metastases. Brit Med J 1 (1963) 15-18 [12] TALBERT OR: General methods of clinical examination: In: YOUMANSJR (ed): Neurological surgery. WB Saunders, Philadelphia 1982
142 [13] VIETIt RG, GL ODOM: Intracranial metastases and their neurosurgical treatment. J Neurosurg 23 (1965) 375-383 [14] VON WOWERN F: Foster Kennedy syndrome. Evaluation of its diagnostic value. Acta Neurol Scand 34 (1967) 205--214 [15] WALTONJ: Disease of the nervous system. Oxford University Press, Oxford 1985
Yildizhan, Foster Kennedy syndrome without frontal lobe [16] WILSONCB, D FEWER: Role of neurosurgery in the management of patients with carcinoma of the breast. Cancer 28 (1971) 1681-1685 Submitted June 6, 1990. Accepted September 9, 1990. Ahmet Yildizhan, M.D. Department of Neurosurgery Vakif Gureba Hospital ~ehremini-Istanbul Turkey
Neurosurg. Rev. 15 (1992)
A case o f Foster Kennedy syndrome without frontal lobe or anterior cranial fossa involvement Ahmet Yildizhan Department of Neurosurgery, Vakif Gureba Hospital, Istanbul, Turkey
Abstract
2 Case report
Foster Kennedy syndrome is a very rare syndrome which includes ipsilaterat optic atrophy and central scotoma, anosmia, contralateral papilledema, and, occasionally, ipsilateral proptosis. A large frontal lobe, olfactory groove, or medial third sphenoidal wing tumor, usually a meningioma, creates this syndrome. In this report, the author presents a case of metastastic cerebral tumor with Foster Kennedy syndrome but without frontal lobe or anterior cranial fossa involvement.
A 55-year-old female was admitted to the hospital on 12. 04. 1990 complaining of blindness in the right eye, headaches, nausea, vomiting, difficulty in swallowing, and epistaxis. The headaches and vision difficulties had started a year ago and the right eye had been blind for the last 8 months. Three months previously it had been noticed that her right eye was beginning to protrude. For about 1.5 months she had been vomiting, usually in the morning.
Keywords: Foster Kennedy syndrome, frontal lobe tumors, metastatic tumors.
1 Introduction The most common primary site for metastatic brain tumors is the lungs [1]. While many patients with carcinoma of the lung, melanoma, or cancer of the colon display multiple cerebral metastases, patients with cancer of the breast, renal carcinorna, ovarian carcinoma, and osteogenic sarcoma more frequently produce single metastases [3, 6, 7, 8, 9, 11, 13]. Malignant cells can reach the nervous system by various routes. Most commonly they spread hematogenically [1@ but they may also spread directly. Passage may occur from the nasopharynx or the lung into the cranium where they may encroach on the brain. The third possible route of dissemination is Batson's intraspinal plexus [5]. In this paper I present a patient with a metastatic tumor which has passed from the nasopharynx through the skull base and extended directly into the middle cranial fossa and brain. This tumor has caused a Foster Kennedy syndrome, without spreading to the anterior cranial fossa or frontal lobe. O 1992by Walter de Gruyter & Co. Berlin 9New York
A physical examination of the patient established that there had been a considerable weight loss. There was a 2 x 2 x 3 cm mass on the neck behind the fight angulus mandibulae which was sensitive to palpation. Examination of the throat revealed that the area around the right tonsils was swollen. Proptosis was present in the right eye (Figure 1, 2). A neurological examination showed that there was no sight in the right eye and ptosis was present. The right pupil was constantly dilated and there was no direct or indirect reaction to light. In the left eye there was also no indirect light reflex. There were no eye movements on the right. An examination of the optic fundus established papilledema on the left and optic atrophy on the right (Foster Kennedy syndrome) as well as anosmia on the right side. The patient had difficulty swallowing and no gag reflex. Blood and urine tests as well as plain chest x-rays were normal. Contrast enhanced computed tomography revealed a mass beginning in the right nasopharynx and extending through the skull base to the right middle cranial fossa and temporal lobe. This mass had also invaded the right orbita causing proptosis on the right but did not extend into the anterior cranial fossa and frontal lobe (Figures 3, 4, 5, 6).
140
Yildizhan, Foster Kennedy syndrome without frontal lobe
Figure 1. Photograph taken from the front showing ptosis and proptosis on patient's right side,
Figure 2. Photograph of the right side showing proptosis.
Figure 3. Contrast enhanced CT showing a mass origi-
Figure 4. Contrast enhanced CT showing the metastasis in the right middle cranial fossa and orbita.
nating in the fight nasopharynx invading the skull base and right orbita causing proptosis.
Figure 5. Contrast enhanced CT showing the tumor occupying the fight middle cranial fossa and basal cisterns.
Figure 6. Contrast enhanced CT showing the tumor's spread to the right temporal lobe but not invading the frontal lobe. Neurosurg. Rev. 15 (1992)
Yildizhan, Foster Kennedy syndrome without frontal lobe The patient was diagnosed as having a metastatic tumor. A biopsy was taken from the nasopharynx. Histopathological investigations revealed a carcinoma with slightly differentiated squamous cells. Dexamethasone relieved the patient partially. Radiotherapy was recommended but the patient refused this treatment. At her own request she was discharged from hospital.
3 Discussion Foster Kennedy syndrome is extremely rare. It occurs in only 1.5% of cases with masses situated in the anterior cranial fossa [14]. The syndrome is defined by ipsilateral central scotoma and primary optic atrophy on the side of the lesion and contralateral papilledema [2]. In addition, ipsilateral anosmia and/or proptosis may occasionally occur [10, 12]. Ipsilateral central scotoma and optic atrophy occur due to pressure on the optic nerve; contralateral papilledema occurs as a result of the increased intracranial pressure. Ipsilateral anosrnia is caused by pressure on the olfactory tract, and ipsilateral proptosis, which is rarely seen, develops with the tumor's invasion of the orbita [10]. Tumors of the frontal lobe, sphenoidal wing, or olfactory groove can cause this syndrome [15]. Meningiomas situated in the medial portion of the sphenoidal wing are the most frequent specific cause. Anosmia is not seen when the lesion is situated more laterally. However, anosmia does occur bilaterally when the tumor spreads from the
141 midline line in the anterior cranial fossa towards the opposite side [12]. In our patient, ipsilateral optic atrophy, anosmia and proptosis, as well as contralateral papilledema were all present. However, these symptoms were not caused by a tumor situated in the frontal lobe, anterior cranial fossa, or sphenoidal wing, but rather as a result of a nasopharyngeal carcinoma spreading directly from the skull base towards the middle cranial fossa and orbita. The tumor, which had invaded the optic nerve and olfactory tract from below on the right side, had created pressure and, as a consequence, optic atrophy and anosmia. The tumor had continued to advance from the optic nerve occupying the right orbit, thus causing proptosis. The tumor also invaded the basal cisterns and had broken the circulation of the cerebrospinal fluid. As a result, the intracranial pressure was increased, causing contralateral papilledema (Figures 5, 6), nausea and vomiting. Differential diagnosis of patients with these symptoms should consider ischemic optic neuropathy, diabetes mellitus, hypertensive cardiovascular disease, collagen vascular disease, syphilis, local arachnoiditis, and cranial arteritis [4]. None of the above were found in our patient. This syndrome suggests a mass in the areas of the frontal lobe, k n o w n as the brain's silent area. In our patient, however, this was not the case; Foster Kennedy syndrome had developed without frontal lobe or anterior cranial fossa involvement (Figure 6). This possibility should always be kept in mind during diagnosis and differential diagnosis.
References [1] G~.MACn~ FW JR, JB POSNER, RH PATTERSONJR: Metastatic brain tumors. In: YOUMANSJR (ed): Neurological surgery. WB Saunders, Philadelphia 1982 [2] KENNEDYF" Retrobutbar neuritis as an exact diagnostic sign of certain tumors and abscesses in the frontal lobes. Amer J Med Sci 142 (1911) 355-368 [3] LANG EF, J SEATER:Metastatic brain tumors. Resuits of surgical and non-surgical treatment. Surg Clin N Amer 44 (1964) 865-872 [4] Mc CRARYJA: Neurophthalmology. In: YOUMANS JR (eds): Neurological surgery. WB Saunders, Philadelphia 1982 [5] ONUIGBOWI: Batson's theory of vertebral venous metastasis. A review. Oncology 32 (1975) 145-150 (6] POSNERJB: Diagnosis and treatment of metastases to the brain. Clin Bull 4 (1974) 4 7 - 5 7 Neurosurg. Rev. 15 (1992)
[7] RANSOHOFFJ: Surgical management of metastatic tumors. Seminars Oncol 2 (1975) 2 1 - 2 7 [8] RASYdNDR, SR WEISS,RE WERMUTH:Single metastatic brain tumors. Treatment and follow-up in 41 cases. Amer Surg 35 (1969) 510-515 [9] RASKINDR, SR WEISS,JJ MANNING,RE WERMUTH: Survival after surgical excision of single metastatic brain tumors. Amer J Roentgen 111 (1971) 323328 [10] RENGACHARYSS: Cranial nerve examination. In: WmKINSRH, SS R~NGACHARY(eds): Neurosurgery. McGraw-Hill, New York 1985 [11] RICHARDS P, W MC KISSOCK: Intracranial metastases. Brit Med J 1 (1963) 15-18 [12] TALBERT OR: General methods of clinical examination: In: YOUMANSJR (ed): Neurological surgery. WB Saunders, Philadelphia 1982
142 [13] VIETIt RG, GL ODOM: Intracranial metastases and their neurosurgical treatment. J Neurosurg 23 (1965) 375-383 [14] VON WOWERN F: Foster Kennedy syndrome. Evaluation of its diagnostic value. Acta Neurol Scand 34 (1967) 205--214 [15] WALTONJ: Disease of the nervous system. Oxford University Press, Oxford 1985
Yildizhan, Foster Kennedy syndrome without frontal lobe [16] WILSONCB, D FEWER: Role of neurosurgery in the management of patients with carcinoma of the breast. Cancer 28 (1971) 1681-1685 Submitted June 6, 1990. Accepted September 9, 1990. Ahmet Yildizhan, M.D. Department of Neurosurgery Vakif Gureba Hospital ~ehremini-Istanbul Turkey
Neurosurg. Rev. 15 (1992)
