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In the name of God

In the name of God. Intrarenal Neuroblastoma (IRNB ). معرفی بیمار. شرح حال بیمار پسر 2.5 ساله با شکایت بی اشتهائی و استفراغ از حدود 2- 3 ماه قبل از مراجعه. درمانهای سرپائی که نتیجه ای نگرفته است. سونوگرافی شکم: توده شکمی

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In the name of God

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  1. In the name of God IntrarenalNeuroblastoma (IRNB)

  2. معرفی بیمار • شرح حال بیمار • پسر 2.5 ساله با شکایت بی اشتهائی و استفراغ از حدود 2- 3 ماه قبل از مراجعه. • درمانهای سرپائی که نتیجه ای نگرفته است. • سونوگرافی شکم: توده شکمی • مراجعه به بیمارستان مفید 9/8/ 91بستری بخش جراحی 2 - ارزیابی بیمارستان مفید

  3. Lab data WBc:5100 PMN= 52 Lymph= 45 Hb=8 Plat= 109000 ESR: 7 Uric acid:6 LDH:15714 Bun :15 Cereatinin: 0.7 SGOT:260 SGPT: 25 BilT:2.2 Bil D:0.9 PT:13 PTT:36

  4. Imaging CT:9/9/91 A huge large 109×133mm heterogen mass with central hypodence foci ( necrosis) without calcification in central part of abdomen that extend bilaterally especially to R side. Displacement of Aorta and pressure effect on adjacent organs. Liver: Nl No pathologic findings in bilateral adrenal glands. Moderate hydronephrosis bilaterally especially in R Kidney. Involvement of R sinus kidney . Mass in left paravertebral region that extends to midline and bulged into the right azigoesophageal recess and sub carinal region. Paraaortic LAP. A connective component between mediastinal and abdominal masses in aortic hiatus of diaphragm DD: Neuroblastoma- Lymphoma Brain CT: NL

  5. Imaging MRI: 13/9/91 Bilateral pleural effusion Large lubulatedparaspinal soft tissue lesion is seen with extension from the abdomen up ward ly into the thoracic cavity . In thorax its significant bulk is in the left side of thoracic spine and has encased thoracic aorta and has displaced azygus vein. involvement of proximal part of some Left Rib. Larglubulated soft tissue mass lesion of abdominal cavity with enhancement of abdomianal vessels No intra canal extension is noted.

  6. Imaging Bone survey 9/9/91: multiple suspicious lucent area in R ischium and R femur and left tibia are noted. Bone scan: 13/9/91 Nl BMA & BMB : Nl 12/9/91 Urine VMA: 2 Nl range < 5.2

  7. Laparatomy Laparatomy. Biopsy of abdominal mass 11/ 9/91 Pathology Report ;25/9/91 Neuroblastoma. Poor stroma, poor diff, MKI< 100/5000 neuroblast- Unfavorable IHC: NSE, Chromo, Syn : Positive NMYC AMP: ? Diagnosis: Neuroblastoma stage 4 Starting chemo: day 6 after surgery , Protocol: N6 ( Course 1- VCR, CPM , ADR)

  8. Post surgery Day 6- post Surgery-: increasing of blood pressure , dyspnea , increased pleural effusion . pleural tap was done. sever e bleeding was seen , chest tube and supportive therapy ( transfusion of blood products including FFP , Plat- cryo ) Other problems during treatment: Increasing of KT , LFT, Increasing of BP During Chemotheropay: GI bleeding and Hematuria Fibrinogen : 140 FDP : >320 D-Dimer : 32/9 , Pancytopenia , PT & PTT prolonge

  9. Post surgery Continous supportive therapy including Novo seven also octeroitide Anti biotic treatment ,GCSF. Now clinically he is better and stable. He is extubated. He is on O2. CBC: 6/10/91 WBC:3200 Hb:13 Plate:35000 PT: Nl PTT : Nl KT: Nl

  10. Primary IntrarenalNeuroblastoma: A Clinical Pathologic Study of 8 Cases—Reply to the Letter to the Editor From Drs Joshi and Shimada.Am J SurgPathol .Volume 36, Number 12, December 2012. Rong Fan, MD Indiana University, Indianapolis, IN • Dr Joshi and Dr Shimada raised an academically interesting issue about the precise definition of primary renal neuroblastomasand, consequently, its estimated incidence. • My position has been an empirical one—if the tumor primarily involves the kidney without evidence of involvement of other primary sites, then it is considered a primary renal tumor. • It is possible, and in fact speculated, that some so called primary renal neuroblastomas actually arose from the immediate pararenal/hilar region, which, for all practical purposes, should be managed in the same or similar manner.

  11. Primary IntrarenalNeuroblastoma: A Clinical Pathologic Study of 8 Cases—Reply to the Letter to the Editor From Drs Joshi and Shimada.Am J SurgPathol .Volume 36, Number 12, December 2012.Rong Fan, MD. Indiana University, Indianapolis, IN This hilum/pelvis connection was indeed considered a “salient” feature of renal neuroblastomas in my original article. Although the word primary, as opposed to the words secondary and metastatic, is mostly used in pathologic terminology, we should also remember that its use in the English language in general also suggests the meaning of principle, basic, and fundamental. If a clearer separation of primary perirenal and primary renal neuroblastoma is still desirable for borderline cases, I suggest that clinical and radiologic impression as Wilms tumor can be added as another qualifier. As in many other situations, there will be borderline cases that some people would classify as perirenal cases, such as the case illustrated in figure1A from my original paper, but using the criteria I cited thus far, this case qualifies as a primary renal neuroblastoma case.

  12. Primary IntrarenalNeuroblastoma: A Clinical Pathologic Study of 8 Cases—Reply to the Letter to the Editor From Drs Joshi and Shimada.Am J SurgPathol .Volume 36, Number 12, December 2012. Rong Fan, MD Indiana University, Indianapolis, IN • A quick search of reports found a total of 380 patients registered in our institution during the same 15-year period (1993 through 2011) who were diagnosed with neuroblastoma. • Of these neuroblastomas, 231 were from the abdominal area. • resulting in the final count of 8 cases for my study. • This puts the incidence of primary renal neuroblastomacases in the proximity of 1% to 2%, which is comparable with 11 neuroblastoma cases simulating Wilms tumor reported in Shamberger et al’s paper, of 868 cases in a 10-year span. • The frequency is higher than originally expected; nonetheless, the 8-case collection from a single institution does point to the belief that the incidence of renal neuroblastoma is perhaps higher than it was generally believed.

  13. REFERENCES 1. Fan R. Primary renal neuroblastoma—a clinical pathologic study of 8 cases. Am J Surg Pathol. 2012;36:94–100. 2. Marchal AL, Hoeffel JC, Freyd S, et al. Arterial hypertension caused by extrinsic compression of the renal artery of tumor origin in a child. Pediatrie. 1986;41:465–480. 3. Herman TE. Special imaging casebook: congenital adrenal neuroblastoma with renovascu- lar hypertension. J Perinatol. 1999;19:468–472. 4. Shinohara M, Shitara T, Hatakeyama S, et al. An infant with systemic hypertension, renal artery stenosis, and neuroblastoma. JPediatr Surg. 2004;39:103–106. 5. Shamberger RC, Smith EI, Joshi VV, et al. The risk of nephrectomy during local control r in abdominal neuroblastoma. J Pediatr Surg. 1998;33:161–164.

  14. Radiology. 1988 Jan;166(1 Pt 1):165-7. Aggressive neuroblastoma simulating Wilms tumor. Rosenfield NS, Leonidas JC, Barwick KW. • Abstract • Solid intrarenalneoplasmsin children are usually Wilms tumors. • Occasionally, however, a neuroblastoma appears to be intrarenal. • In 6 cases where this phenomenon was observed, the histologic findings of each tumor were identical: undifferentiated neuroblastoma. • In each case the child's condition rapidly and progressively deteriorated. • These 6 cases demonstrate the possibility that intrarenal origin or extension of neuroblastoma may be confused with Wilms tumor.

  15. IntrarenalNeuroblastoma. Diagnosed by Fine-Needle Aspiration: A Report of Two Cases. Rosario Serrano, M.D.Vol 27, No 5.2002. Spain Neuroblastoma is the most common extracranial solid tumor of childhood Renal neuroblastoma is believed to originate from adrenal rests within renal tissue or from the intrarenal sympathetic ganglia. There are 8 reported cases of intrarenalneuroblastoma. 2–6 of them in children, but none diagnosed by means of FNA cytology. 2 cases of primary neuroblastoma of the kidney diagnosed by fine-needle aspiration (FNA) :cytology

  16. IntrarenalNeuroblastoma. Diagnosed by Fine-Needle Aspiration: A Report of Two Cases. Rosario Serrano, M.D. Diagnostic Cytopathology, Vol 27, No 5. Spain Case Reports Case 1 A 6-yr-old boy presented with a mass in the lefthypochondria extending up to the umbilicus. Physical examination :revealed a unilateral, palpable, firm, abdominal mass extending 10 cm below the left costal margin. Urine :normal levels of (VMA) & (HVA). Bone marrow :NL A chest roentgenogram : multiple bilateral lung nodules consistent with metastatic disease.

  17. IntrarenalNeuroblastoma. Diagnosed by Fine-Needle Aspiration: A Report of Two Cases. Rosario Serrano, M.D.Vol 27, No 5.2002. Spain Case Reports Case 1 A Ultrasound examination and CT scan showed a left intrarenal solid mass with a small cystic component in the lower pole of the kidney with distortion of the caliceal system (Fig. 1) The adrenal gland was normal. The patient underwent FNA with CT guidance. After cytological diagnosis the patient received chemotherapy for 1 yr and underwent surgical nephrectomy. The resected kidney contained an extensively necrotic tumor with many calcifications. No viable tumoral tissue was found on pathologic study. One year later, the patient was histopathologically diagnosed as having brain metastatic neuroblastoma with meningeal extension (Fig. 2). The patient died with disseminated disease 2 yr after initial diagnosis.

  18. Case 1. CT scan with contrast showing a large moderately calcified mass occupying the left kidney

  19. Photomicrograph showing neuroblastoma cells in aspiration smears. Numerous small neuroblastic cells demonstrating mild variation in nuclear size and shape, scanty cytoplasm, and nuclear molding. Cells are arranged in a characteristic fibrillary background (Case 1)

  20. IntrarenalNeuroblastoma. Diagnosed by Fine-Needle Aspiration: A Report of Two Cases. Rosario Serrano, M.D.Vol 27, No 5.2002. Spain Case 2 A 7-mo-old boy presented with left-sided abdominalmass. PE: revealed a 10 cm, smooth, firm, left abdominal mass. lab data :normal Urinary VMA was 42 g/mg creatinine (Cr) (normal 2.9 –23) and HVA 34 g/mg Cr (normal 3.5–32). BMA:NL

  21. Case 2 Ultrasound examination and CT scan revealed a 10 × 11 ×10 cm, intrarenal mass with foci of calcification, displacing the collecting system (Fig. 3). Chest and skeleton roentgenograms were normal, as well as technetium-99 liver–spleen scan and brain scan. The patient underwent FNA with CT guidance through the anterior abdominal wall. After cytological diagnosis of neuroblastomathe patient underwent a left radical nephrectomy. • Pathologic study of the left nephrectomy and adrenelectomy revealed a poorly differentiated intrarenalneuroblastoma affecting 80% of the kidney with no vascular or lymphatic invasionand no adrenal involvement (Fig. 4). • Two years after excision the patient is currently well without recurrences or metastases.

  22. Case 2. CT scan showing an intrarenal left mass with foci ofcalcification replacing the kidney

  23. IntrarenalNeuroblastoma. Diagnosed by Fine-Needle Aspiration: A Report of Two Cases. Rosario Serrano, M.D.Vol 27, No 5.2002. Spain • Very rarely intrarenalneuroblastoma may develop in children. • In none of the 6 reported cases of intrarenalneuroblastoma was the diagnosis clinically suspected. • They were properly diagnosed only after kidney removal. • The cytologic features of intrarenalneuroblastoma are similar to the classic adrenal neuroblastoma.

  24. IntrarenalNeuroblastoma. Diagnosed by Fine-Needle Aspiration: A Report of Two Cases. Rosario Serrano, M.D.Vol 27, No 5.2002. Spain In summary: Although neuroblastoma is an extremely infrequent tumor in kidney, our two cases demonstrate the accuracy of CT-guided FNA in diagnosing intrarenal masses in children, avoiding unnecessary invasive techniques. The distinction of Wilms’ tumor from neuroblastoma is an important challenge since both tumors have major differences in prognostic and therapeutic response.

  25. Intrarenalneuroblastoma - a diagnostic dilemma: A report of three cases.AnupamLall, MinuBajpai, Devendra Kumar GuptaDepartment of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India.2001 • True IRNB from either seques­tered adrenal rests during the fetal life or intrarenal sympathetic ganglia. • Renal invasion by neuroblastoma may also masquerade as Wilms' tumor. • Hypertension - elevated urinary catecholamines are usually associ­ated with the tumor. • Patients who have a renal mass on imaging, with clinical features of rapid deterioration in general condi­tion & evidence of bony secondaries, should undergo work-up for neuroblastoma pre-operatively to confirm the diagnosis.

  26. Intrarenalneuroblastoma - a diagnostic dilemma: A report of three cases.AnupamLall, MinuBajpai, Devendra Kumar GuptaDepartment of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India • Materials and Methods   The case records and the investigations of the 3 pa­tients with IRNB managed during 1990-99 were reviewed. • 2 cases were diagnosed post-operatively, whereas in the last case, taking lesson from our previous follies, a pre­operative diagnosis was achieved on the basis of elevated urinary catecholamines and bone marrow involvement.4 other patients with bulky abdominal neuroblastoma who had only an intrarenal invasion from outside as found on imaging and subsequently proved on exploration, have been excluded from this retrospective study.

  27. Intrarenalneuroblastoma - a diagnostic dilemma: A report of three cases.AnupamLall, MinuBajpai, Devendra Kumar GuptaDepartment of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India • Case 1A 3½-year-old boy presented - left-sided abdominal mass, since four-months' ago • PE: elevated blood pressure (150/90 mm of Hg) • 19 x 5 cm, mass -intra-abdomi­nally. • A 5 x5 cm spherical swelling present on the frontal bone • Ultrasound showed a large heterogenous mass in the left lumbar region, compressing the inferior vena cava (IVC) and crossing the midline. • Left kidney could not be visual­ised. • Contrast-enhanced CT scan of the abdomen showed a large heterogenous solid mass in the retroperitoneum on the left side with nonenhancing areas. • Aorta, IVC were displaced and encased by the mass at D12 level [Figure - 1].

  28. Intrarenalneuroblastoma - a diagnostic dilemma: A report of three cases.AnupamLall, MinuBajpai, Devendra Kumar GuptaDepartment of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India • Case 1Left kidney was not visualised in any of the cut sections and right kid­ney was normal. • CT scan of the head showed soft tissue mass suggestive of bony metastasis • FNA :suggested to be either a teratoid or clear cell sarcoma of the kidney. • Urinary :elevated levels (VMA) - (HVA)

  29. Intrarenalneuroblastoma - a diagnostic dilemma: A report of three cases.AnupamLall, MinuBajpai, Devendra Kumar GuptaDepartment of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India • Case 1Bone scan showed increased tracer concentration in the right frontal bone, multiple sites in the ribs suggestive of bony metastasis. • Bone marrow aspiration showed round cell infiltration. • diagnosis of intrarenal n euroblastomawas made and the child was started on chemotherapy. • The child has been on follow-up since 3 months and is tolerating the therapy well. • Follow-up CT scan has not shown any reduction in the size of the abdominal mass and forehead swelling.

  30. Intrarenalneuroblastoma - a diagnostic dilemma: A report of three cases.AnupamLall, MinuBajpai, Devendra Kumar GuptaDepartment of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India • Case 2A 4-year-old male child with a mass, no­ticed by the mother during bathing since last 15 days. • No other significant complaints were present. • PE: blood pressure of 120/80 mm of Hg • Ultrasonog­raphy :heterogeneous mass arising from the right kidney. • CT scan revealed a large retro­peritoneal solid mass originating from the right kidney.

  31. Intrarenalneuroblastoma - a diagnostic dilemma: A report of three cases.AnupamLall, MinuBajpai, Devendra Kumar GuptaDepartment of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India.2001 • Case 2 Ret­roperitoneal lymph nodes were enlarged. • FNA : round cell tumor. • With provisional diagnosis of Wilms' tumor the child underwent right nephroureterectomy, tumor was found to arise from midportion of the kidney. • Infrahilar, suprahilar, retrocaval and contralateral lymph nodes were grossly enlarged. The tumor encased the major vessels -IVC and aorta • His­topathology of the mass revealed a composite immature ganglio-neuroblastoma of nodular type. • Subsequently the child was started on Saint Jude's hospital regimen for neu­roblastoma . • bone scan, bone marrow -skeletal survey done :sec­ondary metastasis. • Urinary catecholamine levels were el­evated and upon 6 months of follow-up CT scans showed a significant residual tumor lifting the great vessel

  32. Intrarenalneuroblastoma - a diagnostic dilemma: A report of three cases.AnupamLall, MinuBajpai, Devendra Kumar GuptaDepartment of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India.2001 • Case 3A 2-month-old male child presented with lumps in the ab­domen noticed by the mother about 15 days back. • a large 8 x 10 cm mass was palpa­ble in the left flank,. • 4 subcutaneous nodules measuring 0.5­1 cm on the abdominal wall, near left nipple and right arm. • Ultrasound revealed a mass arising out of the kidney, pushing it medially and obliterating its outline. • Fine needle aspiration from the abdominal lump showed features consistent with round cell tumor morphology, further characterization to be done by open biopsy.

  33. Intrarenalneuroblastoma - a diagnostic dilemma: A report of three cases.AnupamLall, MinuBajpai, Devendra Kumar GuptaDepartment of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India • Case 3On exploration, an well-encapsulated mass aris­ing from the left kidney, about 12x8 cm in size was present. • Left lobe of liver and capsule of the right kidney was studded with umbilicated nodules. • Left nephroureterectomy, excision biopsy of the right kidney nod­ule, left lobe of the liver and subcutaneous nodule was taken. • Histopathological : neurob­lastoma from all the specimens.

  34. Intrarenalneuroblastoma - a diagnostic dilemma: A report of three cases.AnupamLall, MinuBajpai, Devendra Kumar GuptaDepartment of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India.2001 • Case 3Urinary catecholamines elevated. • Skeletal survey was normal, • bone scan:multiple abnormal radiotracer uptake in right occipito-parietal, • Bone marrow biopsy : round cell infiltration. • The child was given vincristine, adriamycin, and cyclophosphamide, for six courses. • The child died after a follow-up of 2 years with bone and liver secondaries

  35. Intrarenalneuroblastoma- adiagnostic dilemma: A report of three cases.AnupamLall, MinuBajpai, Devendra Kumar GuptaDepartment of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India.2001 • Discussion   Primary IRNB is a rare clinical entity. It mimics Wilms' tumor both clinically and radiologically. Intrarenalneu­roblastoma is believed to originate from either sequestration of adrenal medullary tissue in the kidney during the fetal development or originate from intrarenal sympathetic ganglion or a spread of aggressive neuroblastoma infiltrating into the kidney.

  36. Intrarenalneuroblastoma- adiagnostic dilemma: A report of three cases.AnupamLall, MinuBajpai, Devendra Kumar GuptaDepartment of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India.2001 • Discussion   Neuroblastoma is an aggressive malignancy as com­pared to Wilms' tumor and usually presents with secondaries at the time of initial presentation in approximately 75% of the patients.[6],[7] • Renal invasion by neuroblastoma occurs by direct penetration through the renal capsule and/or lymphatic perivascular spread.

  37. Intrarenalneuroblastoma - a diagnostic dilemma: A report of three cases.AnupamLall, MinuBajpai, Devendra Kumar GuptaDepartment of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India.2001 • Renal invasion occurs in approximately 20.4% of cases of abdominal neuroblastoma.[5] • Generally, the renoinfiltrativeneuroblastomas are extensive and have unfavourable histological features as well as lymph node involvement; they are either stage III or IV. • In all our cases with IRNB, there was evidence of secondary metastasis to the bones at the time of presentation.

  38. Intrarenalneuroblastoma - a diagnostic dilemma: A report of three cases.AnupamLall, MinuBajpai, Devendra Kumar GuptaDepartment of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India • Others have reported patients with intrarenalneuroblastoma presenting with proptosis,[8] subcutaneous temporal mass.[4] • A higher incidence of hypertension (66-100%) has been associated with intrarenalneuroblastoma as compared to 27% reported in literature for neuroblastoma.[4] • Hypertention : probably because of compression of renal vessels, increased renin release from the kidney and a high circulating level of catecholamines. • Kessler et al reported hypertension in all his cases of intrarenalneuroblastoma. • Urinary catecholamines may be negative and the imaging modalities may at times be unable to differentiate between neuroblastoma and Wilms' tumor

  39. Intrarenalneuroblastoma - a diagnostic dilemma: A report of three cases.AnupamLall, MinuBajpai, Devendra Kumar GuptaDepartment of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India.2001 • All our cases were males, with an age rang­ing from 2 months to 4 years. • A male preponderance has also been reported in literature.[4],[8] • Our case that presented at 2 months of age, to our knowledge, is the youngest reported case of true intrarenalneuroblastoma in the literature.

  40. Intrarenalneuroblastoma - a diagnostic dilemma: A report of three cases.AnupamLall, MinuBajpai, Devendra Kumar GuptaDepartment of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India.2001 • The features of vascular (aorta) encasement, non-visualisation of the kidney or its displacement, bony or muscular invasion and suprarenal location favours the diagnosis of neuroblastoma • There is always a risk of 5-10% of misdiagnosis on these imaging modalities. • Therefore, where there is a doubt about the diagnosis, skeletal survey, urinary catecholamine levels, bone marrow biopsy and bone scan should be done. • Intra-renal neuroblastoma is usually associated with a poor prognosis as the tumor presents with secondary metastasis at the time of the initial presentation

  41. Intrarenalneuroblastoma - a diagnostic dilemma: A report of three cases.AnupamLall, MinuBajpai, Devendra Kumar GuptaDepartment of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India.2001 • Kessler reported mortality in 3 of his 5 cases of intrarenalneuroblastoma. • 2 of our 3 cases are on follow-up of 3 months and 6 months respec­tively and are on chemotherapy. • One of our patients who pre­sented at 2 months of life, the age at which it is regarded to have a good prognosis, died after a follow-up of 2 years. • The follow-up of other cases is short, so to comment on the ulti­mate prognosis of the disease is not possible. • To draw clear conclusions about the outcome of IRNB a larger series is needed, stratified by biological factors and compared with tumors in other series.

  42. Intrarenalneuroblastoma - a diagnostic dilemma: A report of three cases.AnupamLall, MinuBajpai, Devendra Kumar GuptaDepartment of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India.2001 • Presentation with hypertension and evidence of second­aries with a renal lump should alert the clinician towards the possibility of neuroblastoma. • Similar to the prognosis of neuroblastoma at other sites, IRNB carries a grave prog­nosis and aggressive combined therapeutic modalities of surgery, multiagent chemotherapy with bone marrow rescue, and radiation is recommended.

  43. Can Urol Assoc J. 2012 August; 6(4): E144–E146. 2012 Canadian Urological Association or its licensorsAdult intrarenalneuroblastoma presenting as renal cell carcinoma. Omer Bayrak, MD,*IlkerSeckiner, MD, Turkey; • Case report • A 37-year-old female was referred to our clinic by a nephrologist for evaluation of renal mass. • Abdominal ultrasonography showed a 81 × 61 mm mass in the right kidney. • U/A microscopic hematuria • CT : 70 × 60 mm regular, heterogen and centrally necrotic mass in the lower pole of the right kidney Both chest radiographs and bone scan showed no evidence of metastatic disease.

  44. Can Urol Assoc J. 2012 August; 6(4): E144–E146. 2012 Canadian Urological Association or its licensorsAdult intrarenalneuroblastoma presenting as renal cell carcinoma. Omer Bayrak, MD,*IlkerSeckiner, MD, Turkey; • Case report • Right nephroureterectomy and ipsilateraladrenelectomy • Tumour cells were reacted with synaptophysin, whereas CD-99, chromogranin, WT-1, neuron specific enolase (NSE) and pancytokeratin were found negative. • Diagnosis:Neuroblastoma. • The postoperative period was uneventful and the patient was discharged on postoperative day 3.

  45. Can Urol Assoc J. 2012 August; 6(4): E144–E146. 2012 Canadian Urological Association or its licensorsAdult intrarenalneuroblastoma presenting as renal cell carcinoma. Omer Bayrak, MD,*IlkerSeckiner, MD, Turkey; • Bone marrow biopsy was performed on the 45th day after the operation and found normocellular bone marrow. • The patient was assumed as stage 1 disease. • 6 months after the operation, there were no abnormal findings on her CT. • The patient received no adjuvant therapy, and she had no evidence of recurrrent residual disease 30 months after surgery.

  46. Can Urol Assoc J. 2012 August; 6(4): E144–E146. 2012 .Canadian Urological Association or its licensors.Adultintrarenalneuroblastoma presenting as renal cell carcinoma. Omer Bayrak, MD,*IlkerSeckiner, MD, Turkey • Primary neuroblastoma of the kidney had not been described in the literature until 1986. • Kazuo Gohji and colleagues reported two unusual cases of neoroblastoma of the kidney in adults.6 In both patients right radical nephrectomy was performed.. The first patient received 3000 rad of irradiation to the tumour bed and para aortic area. There was no evidence of tumour recurrence at follow-up. Progressive disseminated disease was documented in the second patient despite postoperative adjuvant chemotherapy with combined cisplatinum and epipodophyllotoxin, and combined vincristine, cyclophosphamide, doxorubicin and dimethyl-triazenoimidazolecarboxamine. Chemotherapy was not effective and the patient’s condition gradually worsened.6 • McLean and colleagues presented the case of a 39-year-old man with stage 1 neuroblastoma with unfavourablehistologic features; he was treated with surgery alone.7 • No treatment recommendations existed for the treatment of neuroblastoma in adults, so they treated this patient according to pediatric guidelines. The patient received no adjuvant therapy, and he had no evidence of recurrrent of residual disease 21 months after surgery.7 Like this case, we treated our patient with surgery alone. • The patient received no adjuvant therapy, and she had no evidence of recurrrent of residual disease 30 months after surgery.

  47. Can Urol Assoc J. 2012 August; 6(4): E144–E146. 2012 .Canadian Urological Association or its licensors.Adultintrarenalneuroblastoma presenting as renal cell carcinoma. Omer Bayrak, MD,*IlkerSeckiner, MD. Turkey • Conclusion • Neuroblastoma is extremely rare in adults. There are only 3 cases of kidney neuroblastoma in the literature. • However, in patients undergoing radical nephrectomy for renal mass, renal neuroblastoma should be included in the differential diagnosis. • If these tumours are limited to the kidney, prognosis is favourable after radical nephrectomy.

  48. Ryan Duhn, MD MervynCohen, MB, ChB, MD. Indiana university • Introduction • We present a case of neuroblastoma who presented with both extensive renal invasion and multiple, well defined, nodular lung metastasis. Either of these two features alone is rare for neuroblastoma. • The presence of these two features together is very unusual, creating a radiographic appearance very suggestive of Wilms’ tumor.

  49. NeuroblastomaMimicsWilms’ Tumor . Ryan Duhn, MD. MervynCohen, MB, ChB, MD. Indiana university

  50. NeuroblastomaMimicsWilms’ Tumor . Ryan Duhn, MD. MervynCohen, MB, ChB, MD. Indiana university

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