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Bone Scans in Neurofibromatosis: Neurofibroma, Plexiform Neuroma and Neurofibrosarcoma

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Bone Scans in Neurofibromatosis: Neurofibroma, Plexiform Neuroma and Neurofibrosarcoma about half of the hyperplastic glands were still visible on the 12. Vogel RA. Kirch D, Lefree M, et al. A new method of multiplanar emission tomography using a seven pinhole collimator and an Anger scintillation camera. J Nucí planar view 1 hr after tracer injection compared to more than Med 1978:19:648-654. 80% of adenomas. 13. Jarritt PH, Ell PJ, Myers MJ, et al. A new transverse section brain imager for single gamma emitters. J NucíMed 1979:20:319-328. 14. Jarritt PH, Ell PJ. A new emission tomographic body scanner. NucíMed Commun CONCLUSION 1980:1:94-101. SPECT increases the sensitivity and specificity of parathy 15. Budinger TF. Physical attributes of single-photon tomography. J NucíMed 1980:21: roid scintigraphy. It appears particularly useful for the location 579-592. 16. Cullum ID. Jarritt PH, Ell PH. Performance parameters, minimal detectable lesions and of mediastinal glands. Our method, which combines the advan partial volume effects for two emission tomographic body units. NucíMed Commun tages of 99mTc-MIBI, FADS and SPECT, allows increased 1980:1:153. 17. Jaszczak RJ. Chang LT. Murphy PH. SPECT using a multi-slice fan beam collimator. scintigraphic accuracy. IEEE Trans NucíSci 1979:26:610-618. 18. Ell PJ. Khan O. Emission computerized tomography: clinical applications. Semin Nucí Med 1981:11:50-60. REFERENCES 19. Billotey C, Aurengo A. Najean Y, et al. Identifying abnormal parathyroid glands in the 1. Potts JT. Ackerman IP. Barker CF. et al. Diagnosis and management of asymptomatic thyroid uptake area using Tc-99m-sestamibi and factor analysis of dynamic structures. primary hyperparathyroidism: consensus development conference statement. Ann J NucíMed 1994:35:1631-1636. Intern Med 1991;! 14:593-597. 20. Coakley AJ, Kettle AG, Wells CP. et al. Technetium-99m-sestamibi: a new agent for 2. Norton JA Controversies, advances in primary hyperparathyroidism. Ann Surg parathyroid imaging. NucíMed Commun 1989:10:791-794. 1992:215:297-299. 21. O'Deherty MJ, Kettle AG, Wells CP, et al. Parathyroid imaging with Tc-99m- 3. Beazley RM, Costa J, Ketcham AS. Reoperative parathyroid surgery. Am J Surg sestamibi: preoperative localization and tissue uptake studies. J NucíMed 1992:33: 1975:130:427-429. 313-318. 4. Brennan MF, Marx SJ, Doppman J, et al. Results of reoperation for persistent and 22. Geatti O. Shapiro B. Prolo G. et al. Location of parathyroid enlargement by recurrent hyperparathyroidism. Ann Surg 1981:194:671-676. Tc-99m-MIBI and 2U1T1scintigraphy, ultrasound and CT [Abstract]. J NucíMed 5. Granberg P-O. Johansson G, Lindval! N, et al. Reoperation for primary hyperparathy 1992:33:894. roidism. Am J Surg 1982:143:296-300. 23. Casara D, Rubello G, Saladini G, et al. Preoperative imaging of pathologic parathyroid 6. Wang CA Parathyroid re-exploration. A clinical and pathological study of 112 cases. glands (PG): comparison of Tc-99m-MIBI scintigraphy. Tl-201 scintigraphy. neck Ann Surg 1977;186,n°2:140-145. echography (NE), computed tomography (CT) and magnetic resonance (MR) [Ab 7. Prinz RA. Gamvros OI. Allison DJ. Flechter DR. Lynn AL. Reoperations for stract]. Eur J NucíMed 1992:19:684. hyperparathyroidism. Surg Gynecol Obslet 1981:152:760-764. 24. Sarfati E, De Ferron P, Gossot D, Assens P, Dubost C. Parathyroid adenoma: atypical 8. Katz AD. Hopp D Parathyroidectomy. Review of 338 consecutive cases for histology, sites ectopie or not? J Chir 1987;l:24-29. location and reoperation. Am J Surg 1982:144:411-415. 25. Miller DL, Doppman JL. Shawker, et al. Localization of parathyroid adenomas in 9. Palmer JA, Rosen IB. Reoperative surgery of hyperparathyroidism. Am J Surg patients who have undergone surgery. Radiolog)- 1987:162:133-137. 1982:144:406-410. 26. Brennan MF, Doppman JL, Kurdy AG, et al. Assessment of techniques for preoper 10. Anger HO. Tomography and other depth discrimination techniques. In: HiñeGJ, ative parathyroid gland localization in patients undergoing reoperation for hyperpara Sorenson JA, eds. Instrumentation in nuclear medicine, vol. 2. New York. NY: thyroidism. Surgen- 1982:91:6-11. Academic Press; 1974:61-100. 27. Aufferman W, Gooding GAW. Okerlund MD, et al. Diagnosis of recurent hyperpara 11. Mathieu L, Budinger TF. Pinhole digital tomography. In: Proceedings of the First thyroidism: comparison of MR imaging and other imaging techniques. Am J Roent- World Congress of Nuclear Medicine. Tokyo, Japan: 1974:1264-1266. genol 1988:150:1027-1033. Bone Scans in Neurofibromatosis: Neurofibroma, Plexiform Neuroma and Neurofibrosarcoma Richard T. Kloos, Vittoria Rufini, Milton D. Gross and Brahm Shapiro Division of Nuclear Medicine, Department of Internal Medicine, University of Michigan, and Department of Veterans Affairs Medical Centers, Ann Arbor, Michigan Key Words: bone diseases; neurofibroma; neurofibrosarcoma; Neurofibromatosis type 1 or von Recklinghausen's disease is one of peripheral nerve neoplasms the most common autosomal dominant genetic disorders. Between J NucíMed 1996; 37:1778-1783 29% and 77% of patients may suffer from a wide range of skeletal abnormalities and, thus, patients with neurofibromatosis frequently undergo skeletal scintigraphy, at which time the common peripheral IN eurofibromatosis type 1 or von Recklinghausen's disease nerve soft-tissue tumors that occur in this syndrome (neurofibro- (1-4) is one of the most common autosomal dominant disor mas, plexiform neuromas and neurofibrosarcomas) may be demon strated. Methods: Single or multiphase 99nTc methylenediphos- ders with a frequency rate of 1 in 3000 live births, an estimated prevalence of 30 patients per 200,000 population. This disease phonate (MDP) bone scans were performed in five patients with affects about 100,000 people in the United States with about neurofibromatosis as part of their clinical evaluation. Results: We 50% of cases representing new mutations (5-8). The gene imaged neurofibrosarcomas in three patients, cutaneous neurofi- bromas in one patient and a plexiform neuroma in one patient. responsible for its genesis has recently been mapped and cloned Conclusion: Single- or multiphasic bone scans may localize com (9,10). Affected tissues include those of neuro-ectodermal, mon soft-tissue tumors in neurofibromatosis. mesenchymal and endoderma! origins. The phenotypic mani festations are protean and may vary from no more than six cafe-au-lait spots 15 mm in diameter (5 mm in prepubescent Received Aug. 21, 1995; revision accepted Dec. 13, 1995. patients) to amongst the most grotesque deforming lesions For correspondence or reprints contact: B. Shapiro, MB, ChB, PhD, University of encountered in clinical medicine (5-7). The diagnostic criteria Michigan Medical Center, Division of Nuclear Medicine, Box 0028, 1500 E. Medical Center Drive, Ann Arbor, Ml 48109-0028. are listed in Table 1. The syndrome of bilateral acoustic 1778 THE JOURNALOFNUCLEARMEDICINE•Vol. 37 •No. 11 •November 1996 TABLE 1 Diagnostic Criteria for von Recklinghausen's Disease (Neurofibromatosis Type 1)* Type 1 Genetic: (a) Identification of the presence of the gene. (b) One or more first degree relatives meeting the clinical criteria for diagnosis of neurofibromatosis 1. Cutaneous: (a) Café-au-laitmacules, 6 or more with greatest diameter over 15 mm (5 mm in prepubescent children). (b) Two or more neuroflbromas of any type. (c) One or more plexiform neuromas. (d) Axillary or inguinal freckling. Ocular: (a) Optic glioma. (b) Two or more hamartomas of the iris (Lisch nodules). Skeletal: (a) Distinctive sphenoid dysplasia. (b) Cortical thinning of long bones (with or without pseudarthrosis). "Clinical diagnosis is made if a patient has positive proof of carrying the FIGURE 1. Plexiform neuroma radiographie features (Patient 1). Note the gene or manifests two or more of the clinical criteria [based on reference extensive lobulated soft tissue masses, abnormal atrophie metacarpals and phalanges affecting the 3rd, 4th and 5th rays of the right hand. (11)]- sparse (27-29) and the phenomenon is listed are "rare" in a neuromas (neurofibromatosis type 2) should not be confused standard compendium of scintigraphic findings (30). with von Recklinghausen's disease, as they are genetically distinct (5-7). There are rare cases of neurofibromatosis which METHODS share characteristics of both syndromes. Patients Von Recklinghausen's disease is relatively common and Patient 1. A 34-yr-old man with neurofibromatosis, previous 29%-77% of cases are associated with various with skeletal cosmetic facial surgery for disfiguring neurofibromas, right abnormalities (5,6,12-14) (Table 2). Consequently, patients hand plexiform neuroma (Fig. 1) and thoracic kyphosis, devel with this disorder commonly are referred to nuclear medicine oped an enlarging, painful, left forearm mass (17 cm). Fine specialists for skeletal scintigraphy. In addition to the expected depiction of skeletal abnormalities (26), 99mTc methylene- needle aspiration (FNA) demonstrated neurofibrosarcoma. CT revealed significant soft tissue and bone destruction by the diphosphonate is taken up on three phase and delayed bone forearm mass. A 99mTc-MDPbone scan (Figs. 2, 3) excluded scintigraphy by a variety of soft-tissue lesions in von Reckling distant osseous metastatic disease in anticipation of an above- hausen's disease, including neurofibromas, plexiform neuromas elbow amputation. Pre-operative MR1 demonstrated a 6 cm and neurofibrosarcomas. Prior reports of such uptake have been paraspinal (C2-C5) mass confirmed by FNA to represent neurofibrosarcoma. Despite five cycles of palliative
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