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NOTES ON A SERIES OF INTRACRANIAL TUMORS AND CONDITIONS SIMULATING THEM: TUMOR SUSPECTS; TUMORS UNVERIFIED; TUMORS VERIFIED
doi: 10.1001/archneurpsyc.1923.02190300002001pmid: N/A
Abstract This discussion may be opened by calling attention to Table 1 as a point of departure for the several matters I shall briefly touch on in connection with the patients with proved or presumptive tumors at present in the hospital wards. It gives in summary a working subdivision of the cases in our tumor series up to May 1 of this year. Table 1.—Cases in the Tumor Series Number Percentage Total Tumors histologically verified..... 868 Gliomas (so diagnosed) of all types..... 362 41.7 Adenomas (chiefly pituitary)..... 169 19.5 Meningiomas ("dural endotheliomas")..... 99 11.4 Neurinomas (acoustic)..... 77 8.9 Congenital tumors..... 54 6.2 Craniopharyngeal pouch cysts..... 42 Cholesteatomas and dermoids..... 8 Teratomas..... 4 Granulomatous tumors..... 36 4.2 Tuberculomas..... 23 Syphilomas..... 13 Metastatic and invasive tumors (varia)..... 34 3.9 Papillomas (choroid-plexus tumors)..... 13 1.5 Angiomas..... 8 0.9 Miscellaneous and unclassified..... 16 1.8 Tumors unverified but presence unquestioned... 476 Tumor suspects: probably not tumor References 1. The list comprises the "suspects" admitted to the clinic during the past few years since we have been recording these conditions under tumor. With it have been included a few cases from the earlier series, then designated as "pseudo tumor." 2. Horrax, Gilbert: A Consideration of the Dermal Versus the Ependymal Cholesteatomas Having Their Attachment in the Cerebral Envelopes , Arch. Neurol. & Psychiat. 8:265 ( (Sept.) ) 1922. 3. This, with other samples of intracranial aneurysm of the internal carotid which have been diagnosed during life, has been reported by Dr. C. P. Symonds in Guy's Hospital Reports for April, 1923, p. 139. 4. Shall We Decompress for Choked Disk? Tr. Am. Neurol. Assn. 48:75. 1922. 5. Subsequent Note.—On June 8, another attempt was made to disclose this woman's tumor. It could be detected by a blunt exploratory needle deep in the hemisphere. The appearance of the ventriculograms, showing as they did a filling defect of the ventricle on each side, made it seem probable that the growth was median; and consequently bone was removed up to the longitudinal sinus so that it was possible to look between the hemisphere and the falx, and at a depth of about 4 cm. the upper surface of what was taken to be an endothelioma was disclosed lying astride the margin of the falx. The patient's condition did not justify an attempt at its removal. It was impossible to replace the boneflap, so that it had to be stripped away to permit a closure. She did remarkably well after this step—so well, in fact, that I determined to make a transcortical incision in an attempt to remove the growth. This was done June 13. It proved to be a huge frontal glioma which was growing into the ventricle. A mass of the tumor, as large as a fist and weighing 150 gm., was removed. She again made an excellent surgical recovery and at the time of discharge, July 21, was up and about on her feet and rapidly regaining the use of her arm; free from discomforts, with normal vision and with her decompression area flat. The tumor is what we designate a protoplasmic glioma (Fig. 7).From the first we were utterly at sea in this woman's case misled by the clinical symptoms as well as by the ventriculograms. The decompression, with its nine months' period of complete relief, is possibly the best thing that could have been done for her at that time and unquestionably saved her vision. She now promises, after all our struggles, to do extremely well and will have further roentgen-ray treatments, for we think a tumor of this type is particularly amenable to deep radiation. 6. Subsequent Note.—This man's condition remained unchanged for weeks. On July 5, in despair, under local anesthesia the left ventricle was punctured and found to be dilated with xanthochromic fluid. A ventriculogram showed that the air did not pass from the left to the right ventricle. Following the puncture the pressure symptoms, bad enough before, became greatly aggravated and though the air was promptly removed he died suddenly about twelve hours later. The necropsy showed a huge glioma of the third ventricle (Fig. 8). 7. Subsequent Note.—She began having fever on May 30, passed into coma and delirium, and died June 3. Examination of the brain revealed marked hydrocephalus produced by a local lesion of unusual site and appearance (Fig. 9). A nodular growth the size of a hickory nut projected from the brachium pontis on the left side of the dilated fourth ventricle. It involved the restiform body of the cerebellum, and must have caught principally the left vestibular nucleus as well as the nucleus of the left abducens and the loop of facial fibers which embrace it. Diagnosis: "Tuberculoma of brain stem: verified." 8. Subsequent Note.—We agonized over this man until June 16 when a left transfrontal operation was performed. The chiasm and nerves appeared to be normal though there was so much thickening of the arachnoid I thought for a time a chronic arachnoiditis might be the cause. The temporal lobe appeared normal. With the intent of puncturing the third ventricle to relieve the hydrocephalus, the posterior margin of the chiasm was exposed and a brain needle introduced. Instead of clear fluid it struck coffee-colored fluid rich in cholesterin crystals—evidently a pharyngeal pouch cyst. A portion of its wall was removed for verification (Fig. 10). (I have never before encountered one of these cysts in this posterior situation.) From a dull somnolent state he was transformed to an alert individual, and made an excellent recovery, in spite of a wave of postoperative polyuria accompanied by nausea and inappetence. 9. —Subsequent Note.—To determine whether there was tumor or a dilated ventricle in the right brain, a puncture was made on June 4. The needle entered a huge gliomatous cyst from which 100 c.c. of yellow clotting fluid was with-drawn and replaced by 80 c.c. of air. The roentgenogram (Fig. 12) gives some idea of the size of the cyst. On June 7 an osteoplastic resection was made; the cyst was opened, its walls were fixed with formalin, and the only nodule of tumor which could be detected was removed from the cyst wall. It showed a glioma. The child almost immediately began to regain the use of his paralyzed side after the puncture and did well for a few weeks but symptoms again returned and the flap became raised. Consequently on July 16 the flap was re-elevated. The position of the cyst was occupied by a huge tumor mass of whose presence there had been no indication before. It was removed en bloc. The mass removed weighed 144 gm. Histologically, it shows (Fig. 13) a non-fibrillar glioma of peculiar character. The boy (except for his unfortunate loss of vision) had almost completely recovered at the time of his discharge August 12. 10. Subsequent Note.—Operation was not performed until June 18. Aside from tension and marked herniation of the cerebellum into the spinal canal and internal hydrocephalus, no lesion was disclosed. She did extremely well and was discharged July 7, her choked disks completely subsided. She was given one treatment with deep radiation and will return periodically for others. Diagnosis: "Intra cerebellar tumor (glioma?), unverified." 11. Subsequent Note.—June 5, a suboccipital exploration was made by Dr. Horrax. There was internal hydrocephalus, moderate pressure cone, a some-what enlarged cerebellar hemisphere; otherwise a negative exploration. The child did extremely well as a result of this decompression and was soon able to sit up. The choked disks rapidly subsided, and the discomforts were relieved—on further vomiting, no headaches. At the time of her discharge, June 21, the local paralyses remained much as before. She was given deep radiotherapy. The case remains as yet "pontile tumor: unverified: tuberculoma or glioma?" 12. Subsequent Note.—During the next ten days the nystagmus became slightly more pronounced: right corneal areflexia with slight hypesthesia of the right face was detected: also some slight incoordination of the right hand on the usual tests. There was right suboccipital tenderness, and possibly some loss of acoustic acuity on the right. A possible recess tumor was suspected. The suboccipital exploration on June 12 was entirely negative except for hydrocephalus. She has done well as a result of the decompression; the headaches were relieved and on her discharge, July 19, after two deep radiotherapeutic treatments, the choked disks were flat with vision preserved. Diagnosis: "Cerebellar tumor (Glioma?): unverified." 13. The photographs (omitted) of this case show the original tumor with its slightly torn surface and the recurrent 80 gm. tumor removed intact with dura attached. 14. Later Note.—The tumor is found to be one of the rarer types of endothelioma—a so-called perivascular endothelioma (perithelioma) (Fig. 21), which is supposed to originate from the vessels of the leptomeninges and is unlike the meningiomas proper, which are of mesothelial rather than of endothelial origin. From its extreme vascularity, an attempt to remove the tumor, even had it been more accessible, would probably have been fatal. It is curious that there should have been two of these rare tumors (Cf. Case 27) in this short series of thirty cases. 15. Further Concerning the Acoustic Neuromas , The Laryngoscope 31:209, 1921 16. Subsequent Notes.—Before discharge on June 15, she had begun to show marked and progressive improvement in her fields though the abducens palsy persisted. On July 23, she wrote that her eyes have straightened and her vision is "back to normalcy," which the laity at least can understand. 17. Subsequent Note.—He was not operated on until June 17, the vision at that time being reduced to 20/200 on the left and 20/100 on the right, and the fields (to a 3 mm. disk) showing a sharp bitemporal hemianopia. The left eye was blind to a 1 mm. disk. The upper cyst wall was removed together with a good deal of the calcareous material from the sella. There were no complications. He experienced immediate improvement in vision, and six days after the operation his fields had widened out to those shown in Figure 29 B. He was discharged July 4, in excellent condition. 18. Arch. Ophthal. 52:209, 1923. 19. —Subsequent Note.—An exploratory operation on June 6 revealed a primary tumor of the chiasm—probably a glioma. No fragments were taken, and the case therefore remains one of "tumor unverified." He was given deep radiotherapy, though our experience is that this type of glioma is not benefited thereby. His condition on discharge June 21 remained practically unaltered. He died shortly after. No necropsy was performed. 20. —Subsequent Note.—At operation, June 9, the lesion proved to be a pituitary adenoma. A large portion of the gland, sufficient to free the distorted nerves from pressure, was scooped out from between the legs of the chiasm. Owing to the vascularity of the adenoma there was a good deal of difficulty in getting complete hemostasis before closure. His wound healing was not perfect because of a too tight bandage. Vision promptly improved, but he had marked postoperative exacerbation of his diabetes insipidus. This was controlled by intranasal pituitrin. He was discharged June 29 in excellent condition with his fields of vision practically normal, having improved from 20/100 to 20/50 in his left eye and from 20/50 to 20/20 in his right. He has subsequently done extremely well and his wave of polyuria has fortunately receded. It could have been avoided entirely, I believe, had his operation been a transsphenoidal one. The case, therefore, now classified as "pituitary adenoma: verified," was wrongly diagnosed because of a misleading series of roentgenograms. It will be seen that the preoperative pathologic diagnosis of each of these last two cases was wrong and that they do not belong with this group of craniopharyngeal pouch tumors. 21. There was no suspicion that the tumor in Case 10, unverified at the time this meeting was held, might be a tuberculoma. 22. Bremer, F.: Global Aphasia and Bilateral Apraxia Due to an Endothelioma Compressing the Gyrus Supramarginalis , Arch. Neurol. & Psychiat. 5:663 ( (June) ) 1921. 23. Certain tests were made before the Association to see if there were any possible traces of the patient's former apraxia. He performed complicated acts promptly. Possibly the most interesting incident was his voluntary statement regarding his remembered confusion when he was asked to do similar acts during his convalescence. 24. Dr. Martin's paper, "La chirurgie des gliomes cavitaires" will shortly appear in the Archives Franco-Belges de chirurgie. 25. Later Note.—The tumor of this case had been repor,ed by the pathologists as the time of the meeting to be a glioma and they still adhere to this diagnosis. It, however, is a peculiar tumor which Dr. Bailey and I are inclined to believe is a perithelioma similar to the tumor in Case 20, which at operation was taken to be a vascular meningioma. We make this diagnosis reluctantly for if it is correct we will have to modify considerably our stand in regard to the justification of recording diagnoses of "glioma: verified" on the character of the cyst contents alone. In our opinion, therefore, the case stands as "cerebellar tumor: verified; cystic perithelioma" and is not a glioma at all (Fig. 33). 26. Subsequent Note.—The child made an astonishingly good recovery with preservation of considerable vision. She received her first roentgen-ray treatment and was discharged June 24. 27. Subsequent Note.—She was discharged June 23, greatly improved, with her choked disks flat. 28. Subsequent Note.—She continued to do extremely well. By June 6, she was up and able to get about the ward alone. Her choked disks had subsided without impairment of vision. She had received one roentgen-ray treatment, and was discharged June 18, walking from the hospital with hardly a perceptible limp. 29. Dr. C. E. Case in the Clifton Medical Bulletin, 1923, p. 22