腰椎骨转移为首发表现的乳头状粘液性囊腺癌一例

腰椎骨转移为首发表现的乳头状粘液性囊腺癌一例 A misdiagnosis case of the pancreas papillary mucinous cyst-adenocarcinoma Qiao peng-fei, Niu guang-ming, Gao yang * (Department of MRI, Affiliated hospital of Inner Mongolia Medical University,hohhot,010050,China) Abstract: The morbidity of papillary cyst-adenocarcinoma of the pancreas is very low, and is seldom first found in Spinal metastasis , so it is easily misdiagnosis before surgery. We report and discuss a case of papillary cyst-adenocarcinoma which for thoracolumbar metastasis as the starting performance in a 56-year-old male patient. Adenocarcinoma is a kind of tumors grown in gland epithelial, when it secreted more mucous called mucinous carcinoma. Furthermore, the mucinous carcinoma with a large number of papillary structures called papillary adenocarcinoma, cystic carcinoma with lumens highly expended called cyst-adenocarcinoma. Sometimes the cyst-adenocarcinoma accompanied with the growth of papillary, which is called papillary cyst-adenocarcinoma. Case Report. A 56-year-old male patient was admitted for backache more than 7 months, pain exacerbated for half a month. 6 months ago the patient felt lumbar acid and backache under no obvious predisposing causes. Once he received medical advice in affiliated hospital of Inner Mongolia medical university, make the lumbar CT, result: Three-quarters fiber ring of waist bulged. For Chinese medicine pills (dosage and composition is unknown) and plaster external treatment, but the results were not very effective. Half a month ago, the patient feel lumbar acid, pain exacerbated, with weakness of double lower limbs, activity limitations, urine is dark brown. Consulted doctor in the second affiliated hospital of Inner Mongolia medical university ,CT considered multiple myelomata, Chest 12/ waist 1 vertebral level spinal stenosis (Figure 1). Figure 1 In order to further diagnosis and treatment, the patient is hospitalized in orthopedics. Since the onset, the patient alternated diarrhea with constipation, about 30 pounds weight loss .His family history was not contributory. Physical and library examination revealed no abnormalities. An lumbar vertebrae MRI scan showed the abnormal signal in Chest 12- waist 3 vertebral body and spine, with long T1, long T2 signal, and the signal was uneven ，the corresponding level back muscle and soft tissue showed mixed T1、mixed T2 signals, with multiple cystic long T1, long T2 signals.Disc structure showed normal, the perturbation of the spinal cord and cauda equinal nerve coursed naturally，no abnormal signal in the spinal cord. Enhancement scanning:The lesion was inhomogeneous enhancement ，cystic lesions showed ring enhancement (Figure 2). Plain scan MRI T2 T1 STIR Enhancement Figure 2 In order to further understand the vertebral involvement, he was taken thoracic MRI (Figure 3)，found abnormal signals of 3, 4 thoracic spinous processes and the posterior soft tissues. T2 T1 STIR Figure 3 According to the performance of the CT, MRI and other imaging ，we drew two views after the consultation: 1 multiple myeloma, 2 multiple bone metastasis. So the Bence-Jones protein (BJP) test and biopsy were carried out. Laboratory results showed BJP negative, which was not supported the first view. The pathological diagnosis: (lumbar 2 para-vertebral and vertebral) fibrous connective tissue and bone tissue in the papillary mucinous epithelial cyst, coating. Combining with the history,it was considered as malignant papillary mucinous cyst-adenocarcinoma (Figure 4). Figure 4 Integrated the imaging and pathological diagnosis, sources of Spinal multiple malignant tumor was not completely clear, and malignant papillary mucinous cyst-adenocarcinoma mostly should be derived from contains glands of the respiratory tract, gastrointestinal tract,etc. For respiratory tract, digestive tract and related laboratory examination and imaging, the tumor was found in the body of the pancreas (Figure 5). Plain scan T2 Fat sat T1 in phase T1 out phase Dynamic contrast enhancement scan mask arterial phase venous phase delayed phase Figure 5 In June 2013 the patient died because the body generalized transfer of the papillary cyst-adenocarcinoma. Now retrospectively analyzed its duration，if the initial treatment timely detected the pancreatic lesions ，early treatment, the prognosis may be very good. Discussion. Solid cystic papillary tumor of the pancreas(SCPT), is a rare form of pancreatic cancer，it was first reported by Frantz in 1959 [1].The name of the disease is more confusion, such as pancreatic solid and papillary tumor, pancreatic solid and papillary epithelial tumor, pancreatic papillary cystic solid tumor, pancreatic papillary adenoma / carcinoma and pancreatic solid cystic papillary tumor， etc[2,3]. WHO(2004) tumor histologic classification unified named solid false papilloma, listed as a source of pancreatic exocrine tumors . SCPT often occurs in the pancreatic tail, growth is relatively slow, and the clinical symptoms are insidious. The tumors are round or oval, clear boundary, a fibrous capsule, section presents the structure of solid and cystic lesions, which is full of bloody or gelatin. Under light microscope, the tumor cells are consistent, clear or eosinophilic cytoplasm, round or oval nuclei, no obvious atypic, mitotic figures are rare, the characteristic changes, the tumor cells around the vascular complex layer are arranged in papillary, papillary and solid areas alternately, and different degrees of visible hemorrhage and cystic change[4]. The origin of SCPT tissue is controversial, it can be derived from the outside of pancreas, or from the pancreas [5].Immunohistochemical studies report the diversification of the tumor, and have no specific findings. The neuron specific enolase (84.1%), the antitrypsin (83.1%) and the vimentin(72.1%) of the tumor are always positive, the positive rate of progesterone and estrogen receptor are 35.1% and 5.1%[6]. It is very difficult to identify the imaging findings between papillary cyst-adenoma and cyst-adenocarcinoma ,the main points of diagnosis is cyst-adenomas has clear boundary of the circular or oval cystic lesions, generally do not involve the main pancreatic duct. Cyst fluid CT for low density, MRI high signal in T2WI, generally low signal in T1WI, Enhanced scan without enhancement. Cystic wall and septum CT as soft tissue density, can strip or curved calcification; MRI T2WI relatively low signal, T1WI soft tissue signal, enhanced scan showed mild enhancement. Mural nodules attached to the wall or interval, for soft tissue density or signal intensity, mild to severe enhancement. The more solid component is, the more likely the borderline or malignant tumor is; some scholars believe that the cyst-adenoma and cyst-adenocarcinoma are different stages of lesion development. In this case ,the patients had backache , lumbar discomfort for starting symptom, no bone destruction and other positive signs on CT ，and doctors were often not familiar with this disease, so it was difficult to make correct diagnosis, delaying an illness. Operation resection is the main method for treating SCPT, including local tumor resection, resection of pancreatic body and tail and pancreatectomy. SCPT has the characteristics of good prognosis and survival period, literature reported its 2 years survival rate was 97%, and the 5 years survival rate was 95%[7]. In conclusion, because the low incidence and more concealed of SCPT, the lack of specific learning in laboratory examination and imaging examination, the clinical doctors should consider the possibility of SCPT when the location or imaging features are not typical, especially the first symptom was metastases. Reference 1 Frantz VK(Tumors of the pancreas(Anonymous Atlas of Tumor. Pathology[M](Washington DC:Armed forces Institute of Pathology,1959:32—33( 2 Klimstra DS，Wenig BM，Heffess CS(Solid—pseudopapillary tumor of the pancreas:a typically cystic carcinoma of low malignant potential[J](Semin Diagn Pathol，2000，17(1):66 —80( 3 Notohara K，Hamazaki S,Tsnkayama C，et a1(Solid— pseudopapillary tumor of the pancreas:immunohistochemical localization of neuroendocrine markers and CD10[J](Am J Surg Pathol，2000，24(10):1361—1371( 4 YIN Yong,LI Zhao-li,WANG Qin，et al(Meta analysis of solid pseudopapillary tumors of the pancreas[J](CHINESE JOURNAL OF PANCREATOLOGY，2010，10(5):341—344( 5 Basu A, Jha A. Solid and cystic tumor arising from an extrapan-creatic site-a case report[J]. Nepal Med Coll J, 2003, 5(2):107-108. 6 FENG Yan-fenRAO Hui-lanWU Qiu-liang，et al(Clinicopathological and Immunohistochemical Analysis of Solid-pseudopapillary Neoplasm of Pancreas [J](JOURNAL OF SUN YATSEN UNIVERSITY(MEDICAL SCIENCES)，2009， 30(2):200—204( 7 Papavramidis T,Papavramidis S(Solid pseudopapillary tumors of the pancreas:review of 7 1 8 patients reported in English literature[J](J Am Coil Surg，2005，200(6):965—972(