neuroblastoma ( nb ) is a tumor derived from the neural crest that usually originates in the adrenal medulla , but it may arise anywhere within the sympathetic nervous system . nb is a common malignancy in children but rarely occurs in adults ; less than 10% of all cases are diagnosed after the age of 10 years . the incidence for patients between 30 and 39 years of age is approximately 0.2 cases per million person - years . nb has different biological features in adolescents and adults , and a longer course in both of these groups than in children . we report the effective use of multimodal therapy to achieve long - term survival in adult retroperitoneal nb with multiple recurrences . in february 2002 , a previously healthy 25-year - old man presented with a supraclavicular left node that was completely resected with a diagnosis of neurofibrosarcoma . physical examination revealed an abdominal mass . computed tomography ( ct ) showed a multinodular 13.5  10-cm tumor in the retroperitoneal region , covering the aorta and repulsing the inferior vena cava . guided needle biopsy of the lesion revealed adult nb upon histopathological examination at an outside institution . histologically , the tumor consisted of small and round - shaped pseudorosette - forming cells with hyperchromatic nuclei . immunohistochemistry showed cytoplasmic positivity for neuronal specific enolase ( nse ) , chromogranin a , and synaptophysin . the serum levels of nse and catecholamine metabolites , including dopamine , were in the normal range . the patient was diagnosed as having stage iv nb . combination chemotherapy with a cado/ cddp - vp16 regimen according to the pediatric protocol was started with cyclophosphamide ( cpm 1,500 mg / m per cycle ) , doxorubicin ( ad 60 mg / m per cycle ) , vincristine ( 2 mg per cycle ) , cisplatin ( cddp 100 mg / m per cycle ) , and etoposide ( vp16 500 mg / m per cycle ) with g - csf . after 5 courses of chemotherapy , the mass was surgically removed and a lomboaortic lymphadenectomy performed . the mass was nb with 70% necrosis . external irradiation of para - aortic lymph nodes was performed postoperatively , delivering 35 gray ( gy ) with 1.8 gy per day , 5 days per week . semestrial follow - up studies , including mibg scintigraphy , ct , and catecholamine values in the urine and blood were performed . the patient was asymptomatic when he presented for the visit . physical examination and routine blood levels were normal , but the serum dopamine level was elevated ( 655 g / g creatinine for normal range 96 - 463 ) . mibg scintigraphy demonstrated an increased uptake in the upper mediastinal region , and ct scans of the chest revealed a 25-mm mediastinal mass . the patient underwent thoracotomy and the mass measuring 40  60 mm was removed completely . one month later , the postoperative scintigraphy showed abnormal uptake in the left supraclavicular area , corresponding to a metastatic lymph node . because mibg scintigraphy confirmed disease progression , we administrated carboplatin / vp16 ( carboplatin 800 mg / m per cycle and vp16 500 mg / m per cycle with g - csf ) therapy . after 2 courses of chemotherapy , fdg scintigraphy showed a partial response with a diminishing suvmax from 5.2 to 3.3 . a 3rd surgical resection was performed , guided by a perioperative detection probe 24 h after the injection of 5.2 mci of iodine-123-labeled mibg . postoperative metabolic imaging performed at 1 , 3 , 6 , 12 , 15 , 21 , and 24 months did not reveal any abnormality in connection with the disease . however , the postoperative fdg - tep in the 15th month showed an increased uptake in the left popliteal area , corresponding to a less than 1-cm nodule localized in the lateral part of the superior extremity of the left leg . surgical biopsy revealed a hemangioma with schwannoma . in august 2008 , fdg scintigraphy showed abnormal uptake in the left jugular carotid and nodular pleural uptake on the right , but mibg scintigraphy was normal . physical examination was normal and did not find any catecholaminergic manifestations . in january 2009 , fdg - tep was carried out ( 33 months after the last surgery ) and showed a single lesion , measuring 25 mm at its greatest diameter , localized in the pleural area with close contact at the 6th rib and involved the intercostal muscle on the right . an mri of the chest confirmed the metabolic imaging data , but mibg scintigraphy was still normal . the histological findings determined that the lesion was a parietal and visceral pleuritic localization of the nb demonstrated with r1 resection . the patient is still alive and has no new metastases more than 8 years after diagnosis . surgical resection , histology , ct , and mibg scintigraphy are the methods of choice for diagnosis . in general , catecholamine metabolites in urine or serum , nse , and lactate dehydrogenase are useful parameters for diagnosis and follow - up . however , an abnormal tumor marker leads to further assessment with detection of relapse or progression in only 14% of patients . for our patient , currently , a reported specificity of 99% and cumulative sensitivity of roughly 90% in paraganglioma make i - mibg the most important nuclear imaging method . however , false negatives have been reported and may be explained by the absence of catecholamine reuptake and/or dedifferentiated features of malignant tumors . in our case , for paraganglioma , fdg - pet diagnoses malignancy better than mibg scintigraphy and reveals more metastases . however , fdg - pet has lower sensitivity and , in particular , lower specificity than mibg scintigraphy for detecting paragangliomas . moreover , there are some false positive cases with fdg - tep , as we reported for our patient . the perioperative detection probe is a supplementary diagnostic tool for guiding the exeresis of recurrent tumors , which are sometimes difficult to access surgically . several publications have reported the use of a perioperative detection probe after the injection of mibg - labeled with iodine-125 , or iodine-123 , or in - pentetrotide . stage iii and iv tumors are often initially treated with chemotherapy followed by tumor resection , as was done in our patient . the most frequently used cytostatic agents , either alone or in combination , include alkylating agents ( cyclophosphamide / ifosfamide ) , platinum - based agents ( cisplatin / carboplatin ) , etoposide , adriamycin , and vinca - alkaloids . clinical data on the survival outcomes of adult patients ( defined as 20 years of age and older ) with nb are scarce due to the rarity of the disease . data from the publicly accessible surveillance epidemiology and end results confirm these findings , but there has been a trend of improved survival for the past decade , which may reflect advancements in treatment . nb in adolescents and adults exhibits different biological characteristics and has a longer course than in children , with multiple recurrences and/or chronic persistent disease . currently , there are no standard treatment guidelines for patients with adult nb . in patients with metastatic disease and at late stages of disease , follow - up of patients with nb should include metabolic imaging studies and tumor marker determination . the collection and evaluation of data on adult patients with this rare tumor are warranted in order to optimize treatment strategies . whether more intensive treatment or myeloablative therapy with stem cell support will improve the outcome of nb in adults is unclear .
neuroblastoma ( nb ) rarely occurs in adults , and less than 10% of the cases occur in patients older than 10 years . currently , there are no standard treatment guidelines for adult nb patients . 
 we report the case of a young man suffering from nb in adulthood with multiple recurrences . 
 treatment included multiple resections , chemotherapy , and radiotherapy . 
 this patient remains free of clinical disease more than 7 years after diagnosis .