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Giant pituitary adenoma
Surg Neurol 1983;20:379-82
379
Giant Pituitary Adenoma V . K . S r i v a s t a v a , M . C h . , K . S. N a r a y a n a s w a m y ,
M.Ch., and T. Vasudev Rao, M.D.
Departments of Neurosurgery and Neuropathology, National Institute of Mental Health & Neuro Sciences, Bangalore, India
Srivastava VK, Narayanaswamy KS, Rao TV. Giant Pituitary Adenoma. Surg Neurol 1983;20:379-82.
The clinical, radiologic, and autopsy findings of an unusually large pituitary adenoma are described. A review of the literature revealed such large tumors to be quite rare. KEYWORDS: Pituitary adenoma, large
Pituitary adenomas that call for neurosurgical intervention are not uncommon, having an incidence ranging from 7 to 17.8% o f all intracranial tumors [8]. However, the giant variant is rare. Moreover, although the term "giant pituitary adenoma" is well accepted, there are no rigid criteria, either by way of measurement or morphology, for identifying these lesions. Jefferson [ 1] was the first to report a series of massive adenomas, some of which had a suprasellar extension of more than 7 cm. Symon et al [10], in a recent review, state that they consider an adenoma giant only when it has a suprasellar extension of more than 40 mm or is within 6 mm of the foramen o f Monro. In this communication we describe the clinicopathologic findings in a case of giant pituitary adenoma, which were comparable to those in cases reported earlier [ 1,4].
Case R e p o r t A 46-year-old man presented to our neurosurgical service with bifrontal headaches of 8 years duration. There had been a progressive deterioration in his higher mental functions for 2 years. For 6 months, he had been dull, drowsy, and completely withdrawn from his surroundings. Visual deterioration was noticed for 3 months. Three days before coming to the hospital, the patient had a generalized convulsive seizure. Objectively, the patient was of an average build and stature; however, he looked apathetic, and his higher No reprints available. @ 1983 by Elsevier Science Publishing Co., Inc.
mental functions could not be assessed in detail because of his poor attention span. A funduscopic examination revealed bilateral primary atrophy of the optic nerve; the other cranial nerves were normal. The patient's deeptendon reflexes were brisk, and the right plantar response was extensor. Routine hematologic and biochemical investigations did not contribute any diagnostic information. Plain xray films of the skull showed a ballooned sella turcica with erosion of the medial part of the left lesser wing of the sphenoid bone. Bilateral angiography (Figure 1A and 1B) of the carotid arteries revealed evidence of a large sellar mass with a parasellar and suprasellar extension into both frontal lobes, but to a greater extent toward the left frontal lobe. Upward and posterior displacement of the internal cerebral vein confirmed the posterior extent of the mass. A provisional diagnosis of a large pituitary tumor was entertained. A left frontotemporal craniotomy revealed a tumor immediately after the dura mater had been reflected. Only a thin rim of cortex was seen covering the tumor, which occupied the entire area of the craniotomy. Gentle retraction of the tumor subfrontally revealed optic nerves and chiasma to be flattened and ribbonlike. The left internal carotid artery could not be located. The tumor was gray and highly vascular. Only a partial excision of the tumor could be made. Postoperatively, the patient was conscious and had no neurological deficits. On the fourth postoperative day the patient's condition deteriorated and he developed a right hemiplegia. Upon reexploration, no clot was found in the tumor bed. The adjoining brain was edematous and necrotic. The patient developed meningitis postoperatively and expired 3 weeks later despite appropriate antibiotic therapy. An autopsy limited to the brain was performed. The dura mater was adherent to the underlying leptomeninges at the site of the operation. The leptomeninges covering the superolateral surface were hazy and revealed pockets of subarachnoid pus. The interpeduncular fossa showed a large (7 x 5 x 5 cm) tumor that was encapsulated, of a firm to hard consistency, brownish red, and extracerebral. The surface of the tumor was 0090-3019/83/S3.00
380
Surg Neurol 1983;20:379-82
Srivastava et al
B
Figure 1. (A) Angiogram of the right carotid artery showing the extent of the tumor to the right. (B) Angiogram of the left carotid artery showing the parasellar frontal and temporal extensions of the tumor. The anterior cerebral artery was not visualized. A
nodular, with thick, congested subcapsular vessels (Figure 2A). The anteroposterior extent o f the tumor was from the left orbital surface of the frontal lobe to the left half of the pons. Laterally it was seen abutting against the left temporal lobe and extending along the Sylvian fissure. In the tumor bed, the optic nerves and chiasma were ribbonlike, both of the internal carotid arteries were displaced laterally, and the cerebral peduncles were splayed apart. A coronal cut of the brain revealed a semilunar, slitlike third ventricle forming the superolateral border of the tumor (Figure 2B). The tumor extended up to the left insular cortex, distorting the basal ganglia and the internal capsule. Microscopic section of the tumor revealed a picture typical of a sinusoidal variety of chromophobe pituitary adenoma (Figure 3). The leptomeninges covering the brain exhibited features typical of acute meningitis, and the parenchymal softening of the frontal lobe was due to acute ischemic infarction. Discussion
Giant pituitary adenomas are uncommon tumors. Jefferson, in 1940, was the first to report a series of large tumors, and described them as massive adenomas. Since
then, our knowledge of such tumors has been limited to a few case reports [4,10]. There appear to be three types of giant pituitary adenoma reported in literature. The first is a giant, invasive adenoma that infiltrates the brain and destroys the part that it occupies [2,5,8]. The second type constitutes a giant globular adenoma, which displaces the brain and grows at the expense of the ventricles [4]. The third type of giant adenoma comprises tumors that metastasize into the central nervous system [7]. In our case, the tumor had expanded at the expense of the third ventricle and the frontal and temporal horns of the left lateral ventricle. Kernohan and Sayre [4] reported the postmortem findings in a case of giant adenoma in which the tumor, besides occupying the third ventricle, had extended into both the frontal and parietal lobes The same was true in our case, except that the temporal rather than the parietal lobes were involved. Giant pituitary adenomas are so designated merely because of their size; the histologic features of these tumors in no way differ from those of their usual, smaller counterparts. The largest reported tumor measured 7 cm 2, and ours, which measured 7 x 5 cm, was comparable. Erosion of the lesser wing of the sphenoid bone, which we found in this case, was also reported by Symon
Giant Pituitary Adenoma
Surg Neurol 1983;20:379-82
381
A et al [10]. M o s t o f the g i a n t a d e n o m a s r e p o r t e d so far have b e e n carcinomas, sarcomas, o r c h r o m o p h o b e ade n o m a s . T h e t u m o r in o u r case was a typical c h r o m o p h o b e a d e n o m a s h o w i n g no e v i d e n c e o f any m a l i g n a n t change. A n i n t e r e s t i n g o b s e r v a t i o n in t h e p r e s e n t case
Figure 3. Histomicrograph showing a sinsoida/ variety of chromophobe adenoma (HVE & 5< 40).
was that the t u m o r , d e s p i t e its e n o r m o u s size, had n o t e n g u l f e d the carotid arteries. N o r did the avascular app e a r a n c e o f the t u m o r u p o n a n g i o g r a p h y c o r r e l a t e with its highly vascular n a t u r e as f o u n d u p o n o p e r a t i o n . T h e t r e a t m e n t s c h e d u l e for such large a d e n o m a s is controversial. A n y a t t e m p t at radical excision has a v e r y high m o r t a l i t y rate. H o w e v e r , the t r e a t m e n t m e t h o d f o l l o w e d by S y m o n et al [10] a p p e a r s m o s t feasible. T h e y confirm the t u m o r by b i o p s y , shunt the ventricles, and follow this with r a d i o t h e r a p y .
The authors thank Dr. G.N. Narayana Reddy, Director, NIMHANS for permitting use of the clinical material in this paper for publication, and Mr. K.M. James and Mr. S. Vinayagam for their secretarial help.
References 1. Jefferson G. Extra Sellar extension of pituitary adenomas. Proc R Soc Med 1940:33:433-58. Cited by Martins AN [6]. 2. Jefferson G. The invasive adenomas of the anterior pituitary. Sherrington Lecture No.3. Liverpool: University of Liverpool Press, 1955. Cited by Martins AN [6]. 3. Jefferson A. Chromophobe pituitary adenoma--The size of the suprasellar portion in relation to the safety of operation (abstract) J Neurol Neurosurg Psychiatry 1969:32:632. 4. Kenohan JW, Sayre GP. Tumours of pituitary gland an infundibulum. In atlas of tumor pathology. Washington, D.C.: Armed Forces Institute of Pathology, 1956.
382
Surg N e u r o l 1983;20:379-82
5. Martins AN, Hayes GJ, Kempe LG. Invasive pituitary adenomas. J Neurosurg 1965:22:268-76. 6. Martins AN. Pituitary tumours and intrasellar cysts. In: Vinken PJ, Bruyn GW. eds, Handbook of clinical neurology, Vol. 17. Amsterdam: North Holland, 1974:375-439. 7. Ogilvy K, Jakubowski J. Intracranial dissemination of pituitary adenomata. J Neurol Neurosurg Psychiatry 1973:34:199-205.
Srivastava et al
8. Russell DS, Rubinstin LJ. Pathology of tumours of the nervous system, 3rd Ed., London: Edward Arnold Publishers: 1971:233-240. 9. Solitare G.B., Jatlow P. Adenohyphophyseal carcinoma, J Neurosurg 1967:26:624-32. 10. Symon L, Jakubowski J, Kendall B. Surgical treatment of giant pituitary adenomas. J Neurol Neurosurg Psychiatry 1979: 42:973-82.
379
Giant Pituitary Adenoma V . K . S r i v a s t a v a , M . C h . , K . S. N a r a y a n a s w a m y ,
M.Ch., and T. Vasudev Rao, M.D.
Departments of Neurosurgery and Neuropathology, National Institute of Mental Health & Neuro Sciences, Bangalore, India
Srivastava VK, Narayanaswamy KS, Rao TV. Giant Pituitary Adenoma. Surg Neurol 1983;20:379-82.
The clinical, radiologic, and autopsy findings of an unusually large pituitary adenoma are described. A review of the literature revealed such large tumors to be quite rare. KEYWORDS: Pituitary adenoma, large
Pituitary adenomas that call for neurosurgical intervention are not uncommon, having an incidence ranging from 7 to 17.8% o f all intracranial tumors [8]. However, the giant variant is rare. Moreover, although the term "giant pituitary adenoma" is well accepted, there are no rigid criteria, either by way of measurement or morphology, for identifying these lesions. Jefferson [ 1] was the first to report a series of massive adenomas, some of which had a suprasellar extension of more than 7 cm. Symon et al [10], in a recent review, state that they consider an adenoma giant only when it has a suprasellar extension of more than 40 mm or is within 6 mm of the foramen o f Monro. In this communication we describe the clinicopathologic findings in a case of giant pituitary adenoma, which were comparable to those in cases reported earlier [ 1,4].
Case R e p o r t A 46-year-old man presented to our neurosurgical service with bifrontal headaches of 8 years duration. There had been a progressive deterioration in his higher mental functions for 2 years. For 6 months, he had been dull, drowsy, and completely withdrawn from his surroundings. Visual deterioration was noticed for 3 months. Three days before coming to the hospital, the patient had a generalized convulsive seizure. Objectively, the patient was of an average build and stature; however, he looked apathetic, and his higher No reprints available. @ 1983 by Elsevier Science Publishing Co., Inc.
mental functions could not be assessed in detail because of his poor attention span. A funduscopic examination revealed bilateral primary atrophy of the optic nerve; the other cranial nerves were normal. The patient's deeptendon reflexes were brisk, and the right plantar response was extensor. Routine hematologic and biochemical investigations did not contribute any diagnostic information. Plain xray films of the skull showed a ballooned sella turcica with erosion of the medial part of the left lesser wing of the sphenoid bone. Bilateral angiography (Figure 1A and 1B) of the carotid arteries revealed evidence of a large sellar mass with a parasellar and suprasellar extension into both frontal lobes, but to a greater extent toward the left frontal lobe. Upward and posterior displacement of the internal cerebral vein confirmed the posterior extent of the mass. A provisional diagnosis of a large pituitary tumor was entertained. A left frontotemporal craniotomy revealed a tumor immediately after the dura mater had been reflected. Only a thin rim of cortex was seen covering the tumor, which occupied the entire area of the craniotomy. Gentle retraction of the tumor subfrontally revealed optic nerves and chiasma to be flattened and ribbonlike. The left internal carotid artery could not be located. The tumor was gray and highly vascular. Only a partial excision of the tumor could be made. Postoperatively, the patient was conscious and had no neurological deficits. On the fourth postoperative day the patient's condition deteriorated and he developed a right hemiplegia. Upon reexploration, no clot was found in the tumor bed. The adjoining brain was edematous and necrotic. The patient developed meningitis postoperatively and expired 3 weeks later despite appropriate antibiotic therapy. An autopsy limited to the brain was performed. The dura mater was adherent to the underlying leptomeninges at the site of the operation. The leptomeninges covering the superolateral surface were hazy and revealed pockets of subarachnoid pus. The interpeduncular fossa showed a large (7 x 5 x 5 cm) tumor that was encapsulated, of a firm to hard consistency, brownish red, and extracerebral. The surface of the tumor was 0090-3019/83/S3.00
380
Surg Neurol 1983;20:379-82
Srivastava et al
B
Figure 1. (A) Angiogram of the right carotid artery showing the extent of the tumor to the right. (B) Angiogram of the left carotid artery showing the parasellar frontal and temporal extensions of the tumor. The anterior cerebral artery was not visualized. A
nodular, with thick, congested subcapsular vessels (Figure 2A). The anteroposterior extent o f the tumor was from the left orbital surface of the frontal lobe to the left half of the pons. Laterally it was seen abutting against the left temporal lobe and extending along the Sylvian fissure. In the tumor bed, the optic nerves and chiasma were ribbonlike, both of the internal carotid arteries were displaced laterally, and the cerebral peduncles were splayed apart. A coronal cut of the brain revealed a semilunar, slitlike third ventricle forming the superolateral border of the tumor (Figure 2B). The tumor extended up to the left insular cortex, distorting the basal ganglia and the internal capsule. Microscopic section of the tumor revealed a picture typical of a sinusoidal variety of chromophobe pituitary adenoma (Figure 3). The leptomeninges covering the brain exhibited features typical of acute meningitis, and the parenchymal softening of the frontal lobe was due to acute ischemic infarction. Discussion
Giant pituitary adenomas are uncommon tumors. Jefferson, in 1940, was the first to report a series of large tumors, and described them as massive adenomas. Since
then, our knowledge of such tumors has been limited to a few case reports [4,10]. There appear to be three types of giant pituitary adenoma reported in literature. The first is a giant, invasive adenoma that infiltrates the brain and destroys the part that it occupies [2,5,8]. The second type constitutes a giant globular adenoma, which displaces the brain and grows at the expense of the ventricles [4]. The third type of giant adenoma comprises tumors that metastasize into the central nervous system [7]. In our case, the tumor had expanded at the expense of the third ventricle and the frontal and temporal horns of the left lateral ventricle. Kernohan and Sayre [4] reported the postmortem findings in a case of giant adenoma in which the tumor, besides occupying the third ventricle, had extended into both the frontal and parietal lobes The same was true in our case, except that the temporal rather than the parietal lobes were involved. Giant pituitary adenomas are so designated merely because of their size; the histologic features of these tumors in no way differ from those of their usual, smaller counterparts. The largest reported tumor measured 7 cm 2, and ours, which measured 7 x 5 cm, was comparable. Erosion of the lesser wing of the sphenoid bone, which we found in this case, was also reported by Symon
Giant Pituitary Adenoma
Surg Neurol 1983;20:379-82
381
A et al [10]. M o s t o f the g i a n t a d e n o m a s r e p o r t e d so far have b e e n carcinomas, sarcomas, o r c h r o m o p h o b e ade n o m a s . T h e t u m o r in o u r case was a typical c h r o m o p h o b e a d e n o m a s h o w i n g no e v i d e n c e o f any m a l i g n a n t change. A n i n t e r e s t i n g o b s e r v a t i o n in t h e p r e s e n t case
Figure 3. Histomicrograph showing a sinsoida/ variety of chromophobe adenoma (HVE & 5< 40).
was that the t u m o r , d e s p i t e its e n o r m o u s size, had n o t e n g u l f e d the carotid arteries. N o r did the avascular app e a r a n c e o f the t u m o r u p o n a n g i o g r a p h y c o r r e l a t e with its highly vascular n a t u r e as f o u n d u p o n o p e r a t i o n . T h e t r e a t m e n t s c h e d u l e for such large a d e n o m a s is controversial. A n y a t t e m p t at radical excision has a v e r y high m o r t a l i t y rate. H o w e v e r , the t r e a t m e n t m e t h o d f o l l o w e d by S y m o n et al [10] a p p e a r s m o s t feasible. T h e y confirm the t u m o r by b i o p s y , shunt the ventricles, and follow this with r a d i o t h e r a p y .
The authors thank Dr. G.N. Narayana Reddy, Director, NIMHANS for permitting use of the clinical material in this paper for publication, and Mr. K.M. James and Mr. S. Vinayagam for their secretarial help.
References 1. Jefferson G. Extra Sellar extension of pituitary adenomas. Proc R Soc Med 1940:33:433-58. Cited by Martins AN [6]. 2. Jefferson G. The invasive adenomas of the anterior pituitary. Sherrington Lecture No.3. Liverpool: University of Liverpool Press, 1955. Cited by Martins AN [6]. 3. Jefferson A. Chromophobe pituitary adenoma--The size of the suprasellar portion in relation to the safety of operation (abstract) J Neurol Neurosurg Psychiatry 1969:32:632. 4. Kenohan JW, Sayre GP. Tumours of pituitary gland an infundibulum. In atlas of tumor pathology. Washington, D.C.: Armed Forces Institute of Pathology, 1956.
382
Surg N e u r o l 1983;20:379-82
5. Martins AN, Hayes GJ, Kempe LG. Invasive pituitary adenomas. J Neurosurg 1965:22:268-76. 6. Martins AN. Pituitary tumours and intrasellar cysts. In: Vinken PJ, Bruyn GW. eds, Handbook of clinical neurology, Vol. 17. Amsterdam: North Holland, 1974:375-439. 7. Ogilvy K, Jakubowski J. Intracranial dissemination of pituitary adenomata. J Neurol Neurosurg Psychiatry 1973:34:199-205.
Srivastava et al
8. Russell DS, Rubinstin LJ. Pathology of tumours of the nervous system, 3rd Ed., London: Edward Arnold Publishers: 1971:233-240. 9. Solitare G.B., Jatlow P. Adenohyphophyseal carcinoma, J Neurosurg 1967:26:624-32. 10. Symon L, Jakubowski J, Kendall B. Surgical treatment of giant pituitary adenomas. J Neurol Neurosurg Psychiatry 1979: 42:973-82.