Sarcomatous transformation of neurofibromas. Comparative imaging with Ga-67, Tl-201, Tc-99m pentavalent DMSA and Tc-99m MIBI.
A 29-year-old man, with a history of von Recklinghausen's disease, presented with progressive dyspnea associated with a rapidly growing mass on the right chest wall. Plain radiograph and CT of the chest revealed a huge soft-tissue mass with central low-density area involving the right upper lung and chest wall. SPECT imaging with Ga-67 citrate, Tl-201 chloride, Tc-99m pentavalent DMSA (V-DMSA), and Tc-99m MIBI were performed to characterize the mass. The tumor concentrated Ga-67, Tl-201, and Tc-99m (V) DMSA, but not Tc-99m MIBI. Punch biopsy of the lesion revealed malignant transformation of a thoracic neuroma (neurofibrosarcoma). Subsequently, findings compatible with the presence of a multidrug resistance-1 (MDR1) gene in the tumor was documented, which may explain the poor uptake of Tc-99m MIBI. The patient did not respond to intensive chemotherapeutic regimens, and died 3 months later. This case demonstrates the potential use of combined radionuclide imaging for the detection of malignant transformation of neurofibroma, as well as for predicting tumor response to chemotherapy.
Lee
J
J
Department of Nuclear Medicine, Kyungpook National University Hospital, Taegu, Korea.
Sohn
S K
SK
Ahn
B C
BC
Chun
K A
KA
Lee
K
K
Kim
C K
CK
D000328:Adult; D001706:Biopsy; D002471:Cell Transformation, Neoplastic / Q000235:genetics / Q000473:pathology*; D019008:Drug Resistance, Neoplasm / Q000235:genetics; D004417:Dyspnea / Q000000981:diagnostic imaging; D017809:Fatal Outcome; D005544:Forecasting; D005710:Gallium Radioisotopes; D019450:Genes, MDR / Q000235:genetics; D006801:Humans; D008175:Lung Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology; D008297:Male; D009455:Neurofibroma / Q000235:genetics / Q000473:pathology*; D009456:Neurofibromatosis 1 / Q000235:genetics / Q000473:pathology*; D011877:Radionuclide Imaging; D019275:Radiopharmaceuticals; D012509:Sarcoma / Q000000981:diagnostic imaging / Q000235:genetics / Q000473:pathology*; D017256:Technetium Tc 99m Sestamibi; D013794:Thallium Radioisotopes; D013899:Thoracic Neoplasms / Q000000981:diagnostic imaging / Q000235:genetics / Q000473:pathology*; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
D005710:Gallium Radioisotopes; D019275:Radiopharmaceuticals; D013794:Thallium Radioisotopes; D017256:Technetium Tc 99m Sestamibi