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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