J Neurosurg 76:303-306, 1992

Skull base amyloidoma Case report FARUK UNAL, M.D., KEMAL HEPGi]L, M.D., (~I(~EK BAYINDIR, M.D., TURGAY BILGE, M.D., MURAT IMER, M.D., AND INAN TURANTAN, M.D. Departments of Neurosurgery and Neurology, istanbul Faculty of Medicine, and Neurosurgery Clinic, Taksim State Hospital, lstanbul, Turkey ~" A mass lesion of amyloid involving the central nervous system is a rare finding. A 64-year-old woman presented with a large amyloidoma at the skull base causing neural tissue compression. The only accompanying disease was an asymptomatic renal cyst. The mass had caused destruction of the bone elements and pathological calcification as seen on x-ray films, computerized tomography (CT) scans, and magnetic resonance (MR) images, and was enhanced after injection of contrast medium on both CT scans and MR imaging. KEY WORDS 9 amyloid 9 calcification 9 computerized tomography magnetic resonance imaging 9 skull base

MYLO1DOSIS, the extracellular deposition of abnormal protein material, is a well-known entity exhibiting a variety of clinical manifestations. Amyloid may be deposited at local sites or systemically and can affect any tissue; 2: however, a mass lesion of amyloid involving the central nervous system (CNS) is a rare finding. In this report, we present the case of a patient with a large amyloidoma at the skull base causing CNS compression, and we review the literature.

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ventricle, destroying the planum sphenoidale and the clivus. Magnetic resonance (MR) imaging also revealed elevation and compression of the optic chiasm, due to a complete extra-axial mass with heterogeneous signal intensity (Fig. 2). The carotid arteries and cerebral parenchyma were intact. The lesion was enhanced by injection of gadolinium, except for calcified areas at its center. Routine complete blood count, urinalysis, erythrocyte sedimentation rate (ESR), blood biochemistry,

Case Report This 64-year-old woman was admitted to our institution with a 12-month history of gradual impairment of vision in the left eye and a 6-month complaint of nasal obstruction and anosmia. Examination. Neurological examination revealed anosmia, bilateral primary optic atrophy, and bitemporal hemianopsia. Physical examination was normal. Plain skull x-ray films showed erosion of the planum sphenoidale, seIla turcica, and clivus, and calcification in the parasellar area. Computerized tomography (CT) revealed a spontaneous isodense and enhancing mass with nodular calcification on the base of the skull (Fig. 1 left). The mass filled the nasopharynx and the ethmoid and sphenoid sinuses, and ascended to the third

J. Neurosurg. / Volume 76 / February, 1992

FIG. 1. Left: Preoperative computerized tomography scan showing the enhancing calcified mass. Right: Postoperative scan after successful removal of the mass, showing lipoid tissue packed within the cavity. 303

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FIG. 2. Magnetic resonance images, sagittal (left) and coronal (right) sections, showingthe extra-axial mass with heterogeneousintensitycausingneural tissue compression. electrocardiograms, and chest x-ray films were normal. There were normal serum concentrations of prolactin, adrenocorticotrophin, cortisol, follicle-stimulating hormone, luteinizing hormone, thyroxine, tri-iodothyronine, and growth hormone. A preoperative diagnosis of chordoma or chondroma was made. Operation. After insertion of a spinal drainage catheter, a bifrontal craniotomy was performed. The dura was found to be tightly attached to the base of the skull, and an intradural route was selected. An extradural mass was found elevating the chiasm and left optic nerve. The dum was incised along the planum sphenoidale and a reddish, waxy encapsulated tumor came into view. The tumor was poorly vascularized and, after piecemeal removal, a 4 x 4 x 4-cm cavity appeared. A graft of lipomatous tissue and fascia lata taken from the left thigh was packed into this cavity between the medial orbital and the lateral sphenoidal sinus walls, the ethmoid cells, the nasopharynx mucosa and the hard palate, the eroded clivus, and dura (Fig. l right). The osseous defect was repaired with a graft taken from the inner tabula of the frontal bone flap. Pathological Examination. The excised tumor tissue was stained with hematoxylin and eosin and studied by light microscopy. Large acellular deposits of amorphic eosinophilic material were seen surrounded by histiocytes, fibroblasts, giant foreign-body cells, and calcified areas (Fig. 3). The acellular deposits showed positive reaction to periodic acid-Schiff, stained rose with Congo red, and produced green birefringence when viewed under polarized light. After incubation with potassium permanganate, the histochemical reaction to Congo red was negative. The gentian/methyl-crystal violet histochemical reaction showed metachromatism; the deposits stained red while other tissues stained violet. These histological findings were diagnosed as an isolated amyloid deposit. Postoperative Course. The patient's postoperative course was complicated due to mechanical obstruction of the respiratory tract by the edematous nasopharyngeal mucosa, epilepsy, pneumonia, and deep vein 304

thrombosis; however, she recovered gradually. Review of the patient's family history for amyloidosis was negative. After recovery she was examined for primary and secondary amyloidosis and myeloma. Repeat ESR measurements, detection of Bence-Jones proteinuria, serum protein electrophoresis, levels of blood urea nitrogen, creatinine, uric acid, and calcium, and liver function tests were all normal. Abdominal ultrasonography revealed slight hepatomegaly and a small cyst at the lower pole of the right kidney. Whole-body scintigraphy with 99mTc-methylene diphosphonate (MDP) showed accumulation of activity only in the operative field. Liver, subcutaneous fat, gingiva, and rectal biopsies were planned but could not be performed because of anticoagulant therapy for deep vein thrombosis. The patient was discharged on the 39th postoperative day with slight recovery of her visual defect. Physical examination was again normal. Since she lived far from our institution, follow-up examination could not be performed, but 6 months later, she reported by tele-

FIG. 3. Photomicrograph of resected tissue showing amorphic eosinophilic material and giant foreign-bodycell. H&E, x 340. J. Neurosurg. / Volume 76/February, 1992

Skull base amyloidoma TABLE l

TABLE 2

Classification of amyloidosis*

Published cases of amyloidomas involving the central nervous system

Clinical Condition

Fibril Type AL

primary amyloidosis(no preceding or coexistingdisease except multiple myeloma) secondary amyloidosis(coexistenceof inflammatoryinfecAA tious or neoplastic disease) localized amyloid (single organ involvementwithout evidence AL of generalized involvement) familial amyloidosis neuropathic forms AF non-neuropathic forms AA senile amyloid AS * Modified from the classificationof the Third International Symposium on Amyloidosis.t~

phone that she had recovered and declined further hospitalization.

Authors & Year Location Mandl, 1924 spine Bauer & Kuzma, 1949 spine Daly, et aL, 1957 gasserianganglion Borghi & Tagliabue, 1961 gasserianganglion Barr & Lampert, 1972 pituitarygland DeCastro, et al., 1976 gasserianganglion Cohen & Lessell, 1979 orbit Harris & Rayport, 1979 cerebralhemisphere Prasad, et al., 1981 spine Spaar, et al., 1981 cerebral hemisphere Pawar, et al., 1982 spine McAnena, et al., 1982 spine Townsend, el at., 1982 cerebralhemispheres(2 cases) Kuratsu, et al., 1983 pituitarygland Giordano, et al., 1983 temporalbone Mori, et al,, 1985 pituitary gland Matsumoto, et aL, 1985 cerebellopontineangle & jugular foramen Leeson, et al., 1985 spine Dickman, et al., 1988 spine

Discussion Amyloidosis is the focal or systemic deposition of abnormal protein material composed of fibrils measuring 7.5 to 10 n m . 5'~5'~9'2s It is classified according to the predominant fibril type and the underlying pathological process.~S22 Clinical classification of amyloidosis and the fibril protein types are summarized in Table 1. Neurosurgeons occasionally encounter amyloidosis either in the diagnosis of primary cerebral amyloid angiopathy and amyloid neuropathy or in the treatment of intracerebral hematomas or subarachnoid hemorrhage due to amyloid a n g i o p a t h y . 22,2326 Also, amyloid deposits reactive to radiation necrosis ~8or to an arteriovenous malformation have been reported." However, tumor-forming masses of amyloid involving the CNS have rarely been found (Table 2). Amyloid

Tumors

Localized solitary amyloidosis, also known as amyloidoma, can be found in bone, skin, larynx, lymph nodes, endocrine tissue, and the gastrointestinal and urinary tracts. Amyloidomas localized in cerebral tissue, the gasserian ganglion, the cerebellopontine angle, the jugular foramen, the pituitary gland, and the orbital, temporal, and vertebral bones have been reported previously.15. 7-9.14.19.zl.2s.28

Amyloidomas are defined as primary solitary amyloidosis where no plasma-cell dyscrasia or abnormal proteins are detectable. 2 However, some amyloid deposits confining cerebral tissue have been reported in association with pituitary adenomas. L~"2~ When osseous structures are involved with amyloid, they are most commonly associated with multiple myeloma or other plasma-cell dyscrasias2 Other reported amyloidomas have no evidence of systemic disease.7-9~4 19.2s The patient reported here also had an asymptomatic renal cyst which is thought to have no relation to the amyloid tumor. Tissue biopsies for the detection of J. Neurosurg. / V o l u m e 76 / February, 1992

systemic disease or other organ involvement could not be performed due to the patient's anticoagulant therapy and her refusal of further studies. Scintigraphy with ~"mTc-MDP is known to be a sensitive noninvasive screening test for the extent and distribution of amyloidosis. ~2In our patient, whole-body detection revealed accumulation of the radioactive material only at the operative site. Amyloidomas found at multiple sites are reported to contain scattered plasma cells, histiocytes, and occasionally giant foreign-body cells. ~3.22 The etiology and pathogenic mechanisms for this have not yet been determined; however, 5,t4 it has been proposed that the amyloid substance may be produced either from retieuloendothelial ceils or from tumor cells. J4 Since no tumor cells were found on careful examination of the mass in the present case, we suggest that the former theory is preferable. Diagnostic Features

In the present case, bone destruction and calcification were revealed on plain x-ray films, CT scans, and MR images. Tumors that are known to show destruction and calcification of the skull base on plain x-ray films are meningiomas, chondromas, chondrosarcomas, chordomas, and craniopharyngiomas. An amyloid tumor of the gasserian ganglion reported by Daly, et al., 7 was accompanied by decalcification of the middle fossa. Amyloidomas in the cerebropontine angle and jugular foramen reported by Matsumoto, el al., j9 showed erosion of the posterior fossa on skull tomograms and calcification on CT scans. Solitary amyloid tumors of the spine appear as osteolytic masses. 9 Amyloid fibrils are anionic in nature and may contain large amounts of divalent sodium- and calcium-bound proteins. 5 His305

F. l]nal, tological investigation confirmed the presence of calcified areas. Amyloid deposits, while destroying bony elements by slowly expanding mass effect, may contain calcified areas due to the presence of calcium bound to its fibrils. Another characteristic to be stressed is that the amyloid mass was enhanced with injection of iohexol on CT scans and gadolinium on MR images. Cases reported by Malsumoto, et al., ~9 and Townsend, et al., 28 also showed enhancement with injection of contrast medium on CT scans.

Conclusions Localized amyloidosis without evidence of any systemic disease is a benign lesion that enlarges slowly. 2228 It may be treated surgically if indicated and recurs rarely.

Acknowledgment We thank Ms. Fethiye Genqo~ullan for typing this manuscript.

References 1. Barr R, Lampert P: Intrasellar amyloid tumor. Acta New ropathol 21:83-86, 1972 2. Bauer WH, Kuzma JF: Solitary "tumors" of atypical amyloid (paramyloid). Am J Clin Pathol 19:1097-1112, 1949 3. Borghi G, Tagliabue G: Primary amyloidosis of the gasserian ganglion. Acta Nearol Scand 37:105-110, 1961 4. Bruni J, Bilbao JM, Pritzker KPH: Vascular amyloid in the aging central nervous system. Clinico-pathologlcal study and literature review. Can J Neurol Sci 4: 239-244, 1977 5. Cohen AS, Connors LH: The pathogenesis and biochemistry of amyloidosis. J Pathol 151:1-10, 1987 6. Cohen MM, Lessell S: Amyloid tumor of the orbit. Neuroradiology 18:157-159, 1979 7. Daly DD, Love JG, Dockerty MB: Amyloid tumor of the gasserian ganglion. Report of case. J Neurosurg 14: 347-352, 1957 8. DeCastro S, Sparks JT, Lapey JD, et al: Amyloidoma of the gasserian ganglion. Surg Nenrol 6:357-359, 1976 9. Dickman CA, Sonntag VKH, Johnson P, et al: Amyloidoma of the cervical spine: a case report. Neurosurgery 22:419-422, 1988 10. Giordano A, Home DG, Gudbrandsson F: Temporal bone amyloidoma. Otolaryngol Head Neck Surg 91: 104-108, 1983 11. Harris JH, Rayport M: Primary "cerebral amyloidoma." J Neuropathol Exp Neurol 38:3t8, 1979 (Abstract)

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12. Janssen S, Piers DA, van Rijswijk MH, et al: Soft-tissue uptake of 99mTc-diphosphonate and 99mTc-pyrophosphate in amyloidosis. Eur J Nucl Meal 16:663-670, 1990 13. Kisilevsky R: Amyloidosis, in Rubin E, Farber JL (eds): Pathology. London: JB Lippincott, 1988, pp 1178-1193 14. Kuratsu J, Matsukado Y, Miura M: Prolactinoma of pituitary with associated amyloid-like substances. Case report. J Neurosurg 59:1067-1070, 1983 15. Kyle RA, Greipp PR: Amyloidosis (AL). Clinical and laboratory features in 229 cases. Mayo Clin Proc 58: 665-683, 1983 16. Leeson MC, Rechtine GR, Maklay JT, et al: Primary amyloidoma of the spine. A case report and review of the literature. Spine 10:303-306, 1985 17. Mandl J: Ueber lokales Amyloid im Bereiche der Brustwirbels~ule. Virchows Arch (A) 253:639-655, 1924 18. Mandybur TI, Gore I: Amyloid in late postirradiation necrosis of brain. Neurology 19:983-992, 1969 19. Matsumoto T, Tani E, Maeda Y, et al: Amyloidomas in the cerebellopontine angle and jugular foramen. Case report. J Neurosnrg 62:592-596, 1985 20. McAnena OJ, Feely MP, Kealy WF: Spinal cord compression by amyloid tissue. J Neurol Neurosurg Psychiatry 45:1067-t069, 1982 21. Mori H, Moil S, Saitoh Y, et al: Growth hormoneproducing pituitary adenoma with crystal-like amyloid immunohistochemically positive for growth hormone. Cancer 55:96-102, 1985 22. O'Doherty DP, Neoptolemos JP, Wood KF: Place of surgery in the management of amyloid disease. Br J Surg 74:83-88, 1987 23. Ohshima T, Endo T, Nukui H, et al: Cerebral amyloid angiopathy as a cause of subarachnoid hemorrhage. Stroke 21:480-483, 1990 24. Pawar S, Kay CJ, Anderson HH, et al: Primary amyloidoma of the spine. J Comput Assist Tomogr 6: 1175-1177, 1982 25. Prasad BK, Andrews K, Dutton J, et al: Localised amylcid deposit producing paraplegia. Br Med J 283:1087, 1981 26. Rengachary SS, Racela LS, Watanabe I, et al: Neurosurgical and immunological implications of primary cerebral amyloid (congophilic) angiopathy. Neurosurgery 7:1-9, 1980 27. Spaar FW, Goebel HH, Volles E, et al: Tumor-like amyloid formation (amyloidoma) in the brain. J Neural 224: 171-182, 1981 28. Townsend JJ, Tomiyasu U, MacKay A, et al: Central nervous system amyloid presenting as a mass lesion. Report of two cases. J Neurosurg 56:439-442, 1982 Manuscript received January 2, 1991. Accepted in final form July 1 I, 1991. Address reprint, requests to" Faruk Unal, M.D., Neurosurgory Department, Istanbul Faculty of Medicine, Capa 34390, Istanbui, Turkey.

J. Neurosurg. / Volume 76 / February, 1992

Skull base amyloidoma. Case report.

A mass lesion of amyloid involving the central nervous system is a rare finding. A 64-year-old woman presented with a large amyloidoma at the skull ba...
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