In the name of God

In the name of God

IN THE NAME OF GOD Intrarenal Neuroblastoma (IRNB) 2.5 3 -2 . . : /9/8 91 - 2

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 IMAGING

CT:9/9/91 A huge large 109133mm 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 IMAGING MRI: 13/9/91 Bilateral pleural effusion

Large lubulated paraspinal 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 Larg lubulated soft tissue mass lesion of abdominal cavity with enhancement of abdomianal vessels .No intra canal extension is noted 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

LAPARATOMY Laparatomy. Biopsy of abdominal mass 9/91 /11 Pathology Report ;25/9/91 Neuro blastoma. Poor stroma, poor diff, MKI< 100/5000 neuroblastUnfavorable IHC: NSE, Chromo, Syn : Positive : ?NMYC AMP Diagnosis: Neuroblastoma stage 4 , Starting chemo: day 6 after surgery 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 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

PRIMARY INTRARENAL NEUROBLASTOMA: A CLINICAL PATHOLOGIC STUDY OF 8 CASESREPLY TO THE LETTER TO THE EDITOR FROM DRS JOSHI AND SHIMADA. AM J SURG PATHOL .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 neuroblastomas and, consequently, its estimated incidence. My position has been an empirical oneif 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. PRIMARY INTRARENAL NEUROBLASTOMA: A CLINICAL PATHOLOGIC STUDY OF 8 CASESREPLY TO THE LETTER TO THE EDITOR FROM DRS JOSHI AND SHIMADA. AM J SURG PATHOL .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. PRIMARY INTRARENAL NEUROBLASTOMA: A CLINICAL PATHOLOGIC STUDY OF 8 CASESREPLY TO THE LETTER TO THE EDITOR FROM DRS JOSHI AND SHIMADA. AM J SURG PATHOL .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 neuroblastoma cases in the proximity of 1% to 2%, which is comparable with 11 neuroblastoma cases simulating Wilms tumor reported in Shamberger et als 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. REFERENCES 1. Fan R. Primary renal neuroblastomaa clinical pathologic study of 8 cases. Am J Surg Pathol.

2012;36:94100. 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;41:465480. 3. Herman TE. Special imaging casebook: congenital adrenal neuroblastoma with renovascu- lar hypertension. J Perinatol. 1999;19:468472. 4. Shinohara M, Shitara T, Hatakeyama S, et al. An infant with systemic hypertension, renal artery stenosis, and neuroblastoma. JPediatr Surg. 2004;39:103106. 5. Shamberger RC, Smith EI, Joshi VV, et al. The risk of nephrectomy during local control r in abdominal neuroblastoma. J Pediatr Surg. 1998;33:161164. RADIOLOGY. 1988 JAN;166(1 PT 1):165-7. AGGRESSIVE NEUROBLASTOMA SIMULATING WILMS TUMOR. ROSENFIELD NS, LEONIDAS JC, BARWICK KW.

Abstract Solid intrarenal neoplasms in 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. INTRARENAL NEUROBLASTOMA. 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 intrarenal neuroblastoma. 26 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 INTRARENAL NEUROBLASTOMA. 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 left hypochondria 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. INTRARENAL NEUROBLASTOMA. 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. CASE 1. CT SCAN WITH CONTRAST SHOWING A LARGE MODERATELY CALCIFIED MASS OCCUPYING THE LEFT KIDNEY 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) INTRARENAL NEUROBLASTOMA. 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 abdominal mass. 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 3.532). BMA:NL 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 liverspleen scan and brain scan. The patient underwent FNA with CT guidance through the anterior abdominal wall. After cytological diagnosis of neuroblastoma the patient underwent a left radical nephrectomy. Pathologic study of the left nephrectomy and adrenelectomy revealed a poorly differentiated intrarenal neuroblastoma affecting 80% of the kidney with no vascular or lymphatic invasion and no adrenal involvement (Fig. 4). Two years after excision the patient is currently well without recurrences or metastases. CASE 2. CT SCAN SHOWING AN INTRARENAL LEFT MASS WITH FOCI OF CALCIFICATION REPLACING THE KIDNEY

INTRARENAL NEUROBLASTOMA. DIAGNOSED BY FINE-NEEDLE ASPIRATION: A REPORT OF TWO CASES. ROSARIO SERRANO, M.D. VOL 27, NO 5.2002. SPAIN Very rarely intrarenal neuroblastoma may develop in children. In none of the 6 reported cases of intrarenal neuroblastoma was the diagnosis clinically suspected. They were properly diagnosed only after kidney removal. The cytologic features of intrarenal

neuroblastoma are similar to the classic adrenal neuroblastoma. INTRARENAL NEUROBLASTOMA. DIAGNOSED BY FINENEEDLE 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 CTguided 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. INTRARENAL NEUROBLASTOMA - A DIAGNOSTIC DILEMMA: A REPORT OF THREE CASES. ANUPAM LALL, MINU BAJPAI, DEVENDRA KUMAR GUPTA DEPARTMENT 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. INTRARENAL NEUROBLASTOMA - A DIAGNOSTIC DILEMMA: A REPORT OF THREE CASES. ANUPAM LALL, MINU BAJPAI, DEVENDRA KUMAR GUPTA DEPARTMENT OF PEDIATRIC SURGERY, ALL INDIA INSTITUTE OF MEDICAL SCIENCES, NEW DELHI, INDIA Materials and Methods The case records and the investigations of the 3 pa tients 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. INTRARENAL NEUROBLASTOMA - A DIAGNOSTIC DILEMMA: A REPORT OF THREE CASES. ANUPAM LALL, MINU BAJPAI, DEVENDRA KUMAR GUPTA DEPARTMENT OF PEDIATRIC SURGERY, ALL INDIA INSTITUTE OF MEDICAL SCIENCES, NEW DELHI, INDIA

Case 1 A 3-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 x 5 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]. INTRARENAL NEUROBLASTOMA - A DIAGNOSTIC DILEMMA: A REPORT OF THREE CASES. ANUPAM LALL, MINU BAJPAI, DEVENDRA KUMAR GUPTA

DEPARTMENT OF PEDIATRIC SURGERY, ALL INDIA INSTITUTE OF MEDICAL SCIENCES, NEW DELHI, INDIA Case 1 Left 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)

INTRARENAL NEUROBLASTOMA - A DIAGNOSTIC DILEMMA: A REPORT OF THREE CASES. ANUPAM LALL, MINU BAJPAI, DEVENDRA KUMAR GUPTA DEPARTMENT OF PEDIATRIC SURGERY, ALL INDIA INSTITUTE OF MEDICAL SCIENCES, NEW DELHI, INDIA Case 1 Bone 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 euroblastoma was 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. INTRARENAL NEUROBLASTOMA - A DIAGNOSTIC DILEMMA: A REPORT OF THREE CASES. ANUPAM LALL, MINU BAJPAI, DEVENDRA KUMAR GUPTA DEPARTMENT OF PEDIATRIC SURGERY, ALL INDIA INSTITUTE OF MEDICAL SCIENCES, NEW DELHI, INDIA

Case 2 A 4-year-old male child with a mass, no ticed 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. INTRARENAL NEUROBLASTOMA - A DIAGNOSTIC DILEMMA: A REPORT OF THREE CASES. ANUPAM LALL, MINU BAJPAI, DEVENDRA KUMAR GUPTA DEPARTMENT 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 INTRARENAL NEUROBLASTOMA - A DIAGNOSTIC DILEMMA: A REPORT OF THREE CASES. ANUPAM LALL, MINU BAJPAI, DEVENDRA KUMAR GUPTA DEPARTMENT OF PEDIATRIC SURGERY, ALL INDIA INSTITUTE OF MEDICAL SCIENCES, NEW DELHI, INDIA.2001

Case 3 A 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.

INTRARENAL NEUROBLASTOMA - A DIAGNOSTIC DILEMMA: A REPORT OF THREE CASES. ANUPAM LALL, MINU BAJPAI, DEVENDRA KUMAR GUPTA DEPARTMENT OF PEDIATRIC SURGERY, ALL INDIA INSTITUTE OF MEDICAL SCIENCES, NEW DELHI, INDIA Case 3 On exploration, an well-encapsulated mass aris ing 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. INTRARENAL NEUROBLASTOMA - A DIAGNOSTIC DILEMMA: A REPORT OF THREE CASES. ANUPAM LALL, MINU BAJPAI, DEVENDRA KUMAR GUPTA DEPARTMENT OF PEDIATRIC SURGERY, ALL INDIA INSTITUTE OF MEDICAL SCIENCES, NEW DELHI, INDIA.2001

Case 3 Urinary 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 - INTRARENAL NEUROBLASTOMA A DIAGNOSTIC DILEMMA: A REPORT OF THREE CASES. ANUPAM LALL, MINU BAJPAI, DEVENDRA KUMAR GUPTA DEPARTMENT 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. Intrarenal neuroblastoma 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. - INTRARENAL NEUROBLASTOMA A DIAGNOSTIC DILEMMA: A REPORT OF THREE CASES. ANUPAM LALL, MINU BAJPAI, DEVENDRA KUMAR GUPTA DEPARTMENT 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. INTRARENAL NEUROBLASTOMA - A DIAGNOSTIC DILEMMA: A REPORT OF THREE CASES. ANUPAM LALL, MINU BAJPAI, DEVENDRA KUMAR GUPTA DEPARTMENT 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 renoinfiltrative neuroblastomas 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. INTRARENAL NEUROBLASTOMA - A DIAGNOSTIC DILEMMA: A REPORT OF THREE CASES. ANUPAM LALL, MINU BAJPAI,

DEVENDRA KUMAR GUPTA DEPARTMENT OF PEDIATRIC SURGERY, ALL INDIA INSTITUTE OF MEDICAL SCIENCES, NEW DELHI, INDIA Others have reported patients with intrarenal neuroblastoma presenting with proptosis, [8] subcutaneous temporal mass. [4] A higher incidence of hypertension (66-100%) has been associated with intrarenal neuroblastoma 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 intrarenal neuroblastoma. Urinary catecholamines may be negative and the imaging modalities may at times be unable to differentiate between neuroblastoma and Wilms' tumor INTRARENAL NEUROBLASTOMA - A DIAGNOSTIC DILEMMA: A REPORT OF THREE CASES. ANUPAM LALL, MINU BAJPAI, DEVENDRA KUMAR GUPTA DEPARTMENT 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 intrarenal neuroblastoma in the literature. INTRARENAL NEUROBLASTOMA - A DIAGNOSTIC DILEMMA: A REPORT OF THREE CASES. ANUPAM LALL, MINU BAJPAI, DEVENDRA KUMAR GUPTA DEPARTMENT 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 INTRARENAL NEUROBLASTOMA - A DIAGNOSTIC DILEMMA: A REPORT OF THREE CASES. ANUPAM LALL, MINU BAJPAI, DEVENDRA KUMAR GUPTA DEPARTMENT OF PEDIATRIC SURGERY, ALL INDIA INSTITUTE OF

MEDICAL SCIENCES, NEW DELHI, INDIA.2001 Kessler reported mortality in 3 of his 5 cases of intrarenal neuroblastoma. 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. INTRARENAL NEUROBLASTOMA - A DIAGNOSTIC DILEMMA: A REPORT OF THREE CASES. ANUPAM LALL, MINU BAJPAI, DEVENDRA KUMAR GUPTA DEPARTMENT 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. CAN UROL ASSOC J. 2012 AUGUST; 6(4): E144E146. 2012 CANADIAN UROLOGICAL ASSOCIATION OR ITS LICENSORS ADULT INTRARENAL NEUROBLASTOMA PRESENTING AS RENAL CELL CARCINOMA. OMER BAYRAK, MD,* ILKER SECKINER, 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. CAN UROL ASSOC J. 2012 AUGUST; 6(4): E144E146. 2012 CANADIAN UROLOGICAL ASSOCIATION OR ITS LICENSORS ADULT INTRARENAL NEUROBLASTOMA PRESENTING AS RENAL CELL CARCINOMA. OMER BAYRAK, MD,* ILKER SECKINER, MD, TURKEY;

Case report Right nephroureterectomy and ipsilateral adrenelectomy 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. CAN UROL ASSOC J. 2012 AUGUST; 6(4): E144E146. 2012 CANADIAN UROLOGICAL ASSOCIATION OR ITS LICENSORS ADULT INTRARENAL NEUROBLASTOMA PRESENTING AS RENAL CELL CARCINOMA. OMER BAYRAK, MD,* ILKER SECKINER, 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. CAN UROL ASSOC J. 2012 AUGUST; 6(4): E144E146. 2012 .CANADIAN UROLOGICAL ASSOCIATION OR ITS

LICENSORS.ADULT INTRARENAL NEUROBLASTOMA PRESENTING AS RENAL CELL CARCINOMA. OMER BAYRAK, MD,* ILKER SECKINER, 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-triazeno imidazole carboxamine. Chemotherapy was not effective and the patients condition gradually worsened.6

McLean and colleagues presented the case of a 39-year-old man with stage 1 neuroblastoma with unfavourable histologic 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. CAN UROL ASSOC J. 2012 AUGUST; 6(4): E144E146. 2012 .CANADIAN UROLOGICAL ASSOCIATION OR ITS LICENSORS.ADULT INTRARENAL NEUROBLASTOMA PRESENTING AS RENAL CELL CARCINOMA. OMER BAYRAK, MD,* ILKER SECKINER, 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. 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. NEUROBLASTOMAMIMICS WILMS TUMOR . RYAN DUHN, MD. MERVYNCOHEN, MB, CHB, MD. INDIANA UNIVERSITY NEUROBLASTOMAMIMICS WILMS TUMOR . RYAN DUHN, MD. MERVYNCOHEN, MB, CHB, MD. INDIANA UNIVERSITY

NEUROBLASTOMAMIMICS WILMS TUMOR . RYAN DUHN, MD. MERVYNCOHEN, MB, CHB, MD. INDIANA UNIVERSITY Clinical information a 3 year old female with a history of 3 UTI over the previous 5 months,fever and dysuria. abdomen had been increasing in size and firmness over the last several months, but had increased markedly in the last several days prior to presentation. a palpable bump in the left frontoparietal scalp. PE: systolic BP:f 100-150 mmHg. Palpable 4 X 5 cm firm lesion in the left frontoparietal scalp. The abdomen was distended and firm to palpation. NEUROBLASTOMAMIMICS WILMS TUMOR . RYAN DUHN, MD. MERVYNCOHEN, MB, CHB, MD. INDIANA UNIVERSITY Laboratory data at admission:

WBC: 10.5; HB;8.1; Platelets: 537 AST: 137; ALT: 90; LDH: 914; Ferritin: 179 Urine: 24hr VMA=30; HVA=25.7; protein=30; red blood cells=8; Her initial imaging studies strongly suggested a diagnosis of Wilms tumor. However a biopsy revealed the surprising diagnosis of neuroblastoma MIBG scan :positive NEUROBLASTOMAMIMICS WILMS TUMOR . RYAN DUHN, MD. MERVYNCOHEN, MB, CHB, MD. INDIANA UNIVERSITY Intra renalneuroblastoma is a rare entity that can mimic Wilms tumor both clinically and radiographically. In one series: intrarenal neuroblastoma represented 3.8% of all non-Wilms pediatric

renal tumors.5 Intrarenalneuroblastoma typically results from direct renal invasion from an adrenal neuroblastoma. Patients are often hypertensive. The tumors are often very aggressive with poor long term survival.6,7 NEUROBLASTOMA MIMICS WILMS TUMOR . RYAN DUHN, MD. MERVYNCOHEN, MB, CHB, MD. INDIANA UNIVERSITY Conclusion Intra renalneuroblastoma is rare. Lung metastases from

neuroblastomaare rare. The presence of these two findings together is extremely rare and in our patient strongly mimics Wilms tumor. INTRA-ABDOMINAL ROUND CELL TUMOUR OF CHILDHOOD - A DILEMMA OF SITE AND ORIGIN SHANMUGAPRIYA S. SRI RAMACHANDRA JOURNAL OF MEDICINE, JUNE 2009, VOL. II, ISSUE 2 CLINICAL SUMMARY: 8 year old girl -distension of upper abdomen. PE:palpable mass in the right upper abdomen measuring 15x10 cm, not cross the midline. Increased level of Vanillyl mandelic acid (VMA) Intraoperatively, a vascular tumor ,occupying the upper pole and involving 2/3rd of right kidney extending up to the inferior surface of the liver and involving the inferior vena cava. Adrenal gland was not identified

intraoperatively also. Diagnosis: neuroblastoma bone scan :skeletal metastasis INTRA-ABDOMINAL ROUND CELL TUMOUR OF CHILDHOOD - A DILEMMA OF SITE AND ORIGIN SHANMUGAPRIYA S. SRI RAMACHANDRA JOURNAL OF MEDICINE, JUNE 2009, VOL. II, ISSUE 2 DISCUSSION: Primary intrarenal neuroblastoma is a rare clinical entity. It mimics Wilms tumor both clinically and radiologically. Intrarenal neuroblastoma is believed to originate from either sequestration of adrenal medullary tissue in the kidney during the fetal development, from intrarenal sympathetic ganglion or extension of aggressive adrenal neuroblastoma into the kidney.

INTRA-ABDOMINAL ROUND CELL TUMOUR OF CHILDHOOD - A DILEMMA OF SITE AND ORIGIN SHANMUGAPRIYA S. SRI RAMACHANDRA JOURNAL OF MEDICINE, JUNE 2009, VOL. II, ISSUE 2 Renal invasion by neuroblastoma occurs by direct penetration through the renal capsule and /or lymphatic perivascular spread. Generally, the renoinfiltrative neuroblastoma ( a stage III or IV disease) are extensive and have unfavourable histological features with lymph node involvement. INTRA-ABDOMINAL ROUND CELL TUMOUR OF CHILDHOOD - A DILEMMA OF SITE AND ORIGIN SHANMUGAPRIYA S. SRI RAMACHANDRA JOURNAL OF MEDICINE, JUNE 2009, VOL. II, ISSUE 2 .

The features of vascular (aorta) encasement, non-visualization of the kidney or its displacement, bony or muscular invasion and suprarenal location favours the diagnosis of neuroblastoma However, there is always a risk of 5-10% of misdiagnosis on these imaging modalities INTRA-ABDOMINAL ROUND CELL TUMOUR OF CHILDHOOD - A DILEMMA OF SITE AND ORIGIN SHANMUGAPRIYA S. SRI RAMACHANDRA JOURNAL OF MEDICINE, JUNE 2009, VOL. II, ISSUE 2 Review of literature suggested that intrarenal origin or extension of neuroblastoma from adrenal gland may be confused with Wilms tumor[4,5,6,7]. In such situations radiological correlation, urinary catecholamines

levels, immunohistochemistry, cytogenetics studies and bone marrow biopsy would be helpful in estabilishing the diagnosis[4,5,6,7]. INTRA-ABDOMINAL ROUND CELL TUMOUR OF CHILDHOOD - A DILEMMA OF SITE AND ORIGIN SHANMUGAPRIYA S. SRI RAMACHANDRA JOURNAL OF MEDICINE, JUNE 2009, VOL. II, ISSUE 2 Intrarenal neuroblastoma is usually associated with a poor prognosis as the tumor presents with secondary metastasis at the time of the initial presentation. A high proportion of intrarenal neuroblastoma are of unfavourable histology as defined by the International Neuroblastoma Pathology Classification and have a higher incidence of anaplasia(32%) when compared to both their adrenal counterparts and to Wilms tumor. The factors that affect survival are age and health of child, extent of the disease, size, type and location of

the tumor, metastasis, tumors response to therapy and overall childs tolerance to medications. INTRA-ABDOMINAL ROUND CELL TUMOUR OF CHILDHOOD - A DILEMMA OF SITE AND ORIGIN SHANMUGAPRIYA S. SRI RAMACHANDRA JOURNAL OF MEDICINE, JUNE 2009, VOL. II, ISSUE 2 Clinical, radiological and pathological correlation is very essential for diagnosis and appropriate management of this type of unusual cases. To conclude, even though the exact site remained undetermined in this case of small round cell tumour completely replacing the right kidney and adrenal gland, immunohistochemistry played a pivotal role in confirming the diagnosis of neuroblastoma thereby, resolving the dilemma of origin. INTRA RENAL

NEUROBLASTOMA Hyprer tension Elevated VMA & HVA Unfovorable histopathology The features of vascular (aorta) (encasement, non-visualization of the kidney or its displacement, bony or muscular invasion and suprarenal location favours the diagnosis of neuroblastoma) Metastatic at presntation( bone, Bone marrow) Poor Prognosis Acta Paediatrica. Article first published online: 21 JAN 2008 GERD FAXELIUS. DISSEMINATED INTRAVASCULAR COAGULATION AND CONGENITAL N EUROBLASTOMA (pages 667670) . Karolinska sjukhuset, Stockholm, Sweden).

July1975 A newborn infant with congenital neuroblastoma complicated by DIC is described. At birth the infant showed liver and spleen enlargement and shortly thereafter malignant cells were found in the bone marrow. On the fifth day of life the infant started to bleed and coagulation analysis indicated DIC Heparin therapy corrected the coagulation anomaly and irradiation and chemotherapy

temporarily improved the general condition of the infant. The infant finally succumbed from its primary neoplastic disease PEDIATR RADIOL. 2000 JUN;30(6):394-7. HEMOPERITONEUM AND DISSEMINATED INTRAVASCULAR COAGULATION IN TWO NEONATES WITH CONGENITAL BILATERAL NEUROBLASTOMA.VORA D, SLOVIS TL, BOAL DK. DEPARTMENT OF PEDIATRIC IMAGING, CHILDREN'S HOSPITAL OF MICHIGAN, DETROIT 48201, USA We report two neonates with anemia,

hemoperitoneum, and bilateral palpable abdominal masses. Both developed bleeding: a hemoperitoneum in one and visceral hemorrhages secondary to disseminated intravascular coagulation in the other. Each child was thought to have an unusual complication of adrenal hemorrhage, as the masses were of mixed echogenicity. However, histological evaluation revealed neuroblastoma. These cases point out the diagnostic dilemma of a mixed echogenicity suprarenal mass in the neonatal period and emphasize that local and disseminated bleeding can occur as a major manifestation of neuroblastoma. JPN J CLIN ONCOL. 1985 SEP;15(3):483-8. ADVANCED NEUROBLASTOMA AND DISSEMINATED INTRAVASCULAR COAGULATION: REPORT OF SIX CASES. HATAE Y, TAKEDA T, HATTORI T, NAKADATE H, NISHI M

We have experienced 30 patients with neuroblastoma since 1975. (DIC) developed in 6/30 of these patients. They were all in stage IV, namely disseminated neuroblastoma. These 6 cases with DIC proved that some advanced neuroblastomas have the potential to cause coagulopathy in the process of the disease. The plasma concentration of heparin was measured in some patients who were treated with heparin.

The data revealed that the conventionally used intravenous heparin dose is not appropriate in the case of DIC. Effective treatment requires monitoring of the plasma concentration of heparin.

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