2243

Is Primitive Neuroectodermal Tumor of the Kidney aDistinct Entity?

BACKGROUND. Primitive neuroectodermal tumors (PNETs) constitute a family ofCarlos Rodriguez-Galindo, M.D.1

Neyssa M. Marina, M.D.1,4 neoplasms of presumed neuroectodermal origin, most often presenting as bone

or soft tissue masses in the trunk or axial skeleton in adolescents and young adults.Barry D. Fletcher, M.D.2,4,5

David M. Parham, M.D.3,6 As a soft tissue neoplasm, PNET arising in the kidney has not been well described,

with only three cases previously reported.Sara M. Bodner, M.D.3,6

William H. Meyer, M.D.1,4 METHODS. Four patients with PNET of the kidney were diagnosed and treated at

St. Jude Children’s Research Hospital. The authors reviewed the clinical, radiologic,1 Department of Hematology-Oncology, St. and pathologic features and outcomes of these cases and of those previously de-Jude Children’s Research Hospital, Memphis, scribed.Tennessee. RESULTS. The authors’ patients were age 4–20 years. They presented with unilateral2 Department of Diagnostic Imaging, St. Jude renal masses and metastatic disease in the lymph nodes (three patients), lungsChildren’s Research Hospital, Memphis, Ten- (three patients), bone (two patients), liver (one patient), and bone marrow (onenessee.

patient). Treatment included surgery, radiotherapy, and multiagent chemotherapy.3 Department of Pathology, St. Jude Children’s Three of the patients died of progressive disease within 14 months of diagnosis.Research Hospital, Memphis, Tennessee. Features and outcomes were similar to those of the three previously reported cases.4 Department of Pediatrics, University of Tennessee CONCLUSIONS. PNET of the kidney appears to be a distinct entity. Although rare,School of Medicine, Memphis, Tennessee. it must be included in the differential diagnosis of renal tumors in children and5 Department of Radiology, University of Tennessee young adults. Patients usually present with advanced disease and show poor re-School of Medicine, Memphis, Tennessee. sponse to combined-modality therapy. Cancer 1997;79:2243–50.

q 1997 American Cancer Society.6 Department of Pathology, University of TennesseeSchool of Medicine, Memphis, Tennessee.

KEYWORDS: primitive neuroectodermal tumor (PNET), peripheral neuroepithelioma,renal neoplasms, malignant rhabdoid tumor, intrarenal neuroblastoma, Wilms’ tu-

Supported in part by Grants P30 CA-23099 andmor.P30 CA-21765 from the National Cancer Insti-

tute, by the American Lebanese Syrian Associ-ated Charities (ALSAC), and by Grant 93/5431 Ffrom the Fondo de Investigaciones Sanitarias of

irst described by Stout in 1918,1 the concept of primitive neuroec-todermal tumor (PNET) has evolved to include a group of small

the Spanish National Institute of Health. round cell malignancies of ubiquitous location and presumed neu-roectodermal origin. They are defined by expression of the same pro-The authors thank Sharon Naron for editorialtooncogene2 and the presence of a balanced t(11;22)(q24;q12) chro-assistance.mosomal translocation or a (21;22) rearrangement that results in the

Dr. Marina’s current address: Department of Pe- fusion of the gene EWS with the FLI1 or ERG gene, respectively.3 PNETdiatrics, Stanford University Medical Center,

manifests a continuum of neurogenic differentiation, with Ewing’sStanford University School of Medicine, Stan-sarcoma representing the least differentiated and peripheral neuroe-ford, California.pithelioma the most differentiated forms.4 In general, PNET is a very

Dr. Parham’s current address: Department of aggressive neoplasm, with 25–50% of patients presenting with meta-Pediatric Pathology, Arkansas Children’s Hospi- static disease, and a 5-year disease free survival rate of 45–55%.5–7

tal, Little Rock, Arkansas.Renal location of PNET is extremely rare, with only three previouslyreported cases8–10 and a small series from the National Wilms TumorAddress for reprints: William H. Meyer, M.D.,

Dept. of Hematology-Oncology. St. Jude Chil- Study (NWTS) published only in abstract form.11

dren’s Research Hospital, 332 N. Lauderdale, Between March 1962 and August 1996, four cases of PNET of theMemphis, TN 38105-2794. kidney were diagnosed and treated at St. Jude Children’s Research

Hospital (SJCRH). The authors describe the clinical characteristics,Received October 18, 1996; revision receivedFebruary 6, 1997; accepted February 6, 1997. pathologic features, clinical course, response to treatment, and out-

q 1997 American Cancer Society

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come of these patients and review the cases previously tient was subsequently referred to SJCRH. At the time,catecholamine urine excretion was normal. CT scandescribed. Their results suggest that this tumor is a

distinct entity with a very aggressive behavior and poor of the chest revealed multiple, noncalcified bilateralpulmonary nodules consistent with metastases. 99mTc-response to therapy that must be included in the dif-

ferential diagnosis of renal neoplasms in children and methylene diphosphonate (MDP) skeletal scintigraphyshowed a small focus of increased activity involvingyoung adults.the left seventh rib. Review of pathology at SJCRH didnot allow a distinction between neuroblastoma andCase Reports

A review of the records of all children with PNET diag- peripheral neuroepithelioma and a thoracotomy wasperformed to obtain more tissue for further tumornosed and treated between March 1962 and August

1996 at SJCRH identified four patients with a primary evaluation. Immunohistochemical stains on the sec-ond sample were positive for NSE, CD99 (HBA-71) (cy-renal tumor. A previous histologic review of patients

with renal tumors diagnosed and treated between toplasmic surface membrane staining) (Fig. 1B), andbeta-2-microglobulin, and negative for CD56, synap-March 1962 and December 1984 revealed no cases

of PNET.12 The clinical course, imaging studies, and tophysin, and chromogranin. Cytogenetic studies re-vealed a t(11;22)(q24;q12) chromosomal transloca-histologic sections of the patients’ tumors were re-

viewed. Two patients had been diagnosed and pre- tion, confirming the diagnosis of PNET.The patient received 3 induction chemotherapyviously treated at other institutions before referral to

SJCRH. In all cases, histologic sections were prepared courses with ifosfamide, carboplatin, and etoposide(ICE), followed by radiation therapy to the left hemiab-from formalin fixed, paraffin embedded tissue and

stained with hematoxylin and eosin and periodic acid- domen (3600 centigrays [cGy]) and both lungs (1650cGy), along with weekly doses of vincristine and bi-Schiff stains. Immunohistochemical testing used the

standard avidin-biotin complex procedure with spe- weekly administration of actinomycin D. After reevalu-ation showed resolution of the lung metastases, thecific monoclonal antibodies. Electron microscopy

studies were performed in selected cases. The diagno- patient was treated with four courses each of cyclo-phosphamide/doxorubicin and ifosfamide/etoposidesis was confirmed in all four cases by two of the au-

thors (D.M.P. and S.M.B.) using previously described in rotation. Twelve months after diagnosis (4 monthsafter completing therapy), he developed new bilateralhistopathologic criteria.4,6 Clinical characteristics,

pathologic features, treatment, and outcome (includ- pleuropulmonary metastases without evidence of localrecurrence. He was treated with topotecan in a Phaseing the data from previously reported cases) are sum-

marized in Table 1. I study but had no response, and died of tumor pro-gression 14 months after diagnosis. An autopsy wasnot performed.Case 1

An 18-year-old white male was examined at his localhospital in October 1991 after an 8-month history of Case 2

In June 1992, a 20-year-old white male was examined atabdominal pain. Computed tomography (CT) scan ofthe abdomen revealed a mass nearly replacing the left his local hospital with a 4-month history of abdominal

pain, a right-sided abdominal mass, and massive hema-kidney, with small areas of calcification and mild retro-peritoneal lymph node enlargement. The patient un- turia. CT scan of the abdomen (Fig. 2A) showed an inho-

mogeneous, partially calcified 12 cm X 10 cm X 16 cmderwent a left nephrectomy. On pathologic examina-tion, the tumor measured 12.5 cm X 9.5 cm X 6.5 cm, mass arising from the right kidney, and retrocaval lymph

node enlargement. CT scan of the chest showed a soli-was spongy and soft, distorted the renal parenchyma,and penetrated the renal capsule without extension to tary left upper lobe pulmonary nodule. 99mTc-MDP skele-

tal scintigraphy (Fig. 2B) showed abnormally increasedthe renal vein or ureter. Microscopically, it was ahighly cellular neoplasm that infiltrated the renal pa- activity in the right kidney mass with no skeletal abnor-

malities. After a right nephrectomy and a diagnosis ofrenchyma and was comprised of sheets of small,round-to-oval cells with round nuclei and pale pink Stage IV Wilms’ tumor of blastemic type, the patient was

referred to SJCRH. A repeat CT scan of the chest showedcytoplasm. There were foci of Homer Wright rosetteformation (Fig. 1A) and extensive areas of hemorrhage bilateral pulmonary metastases. Review of the tumor tis-

sue showed monomorphic sheets of cells with round toand necrosis.Immunohistochemical stains were positive for oval nuclei and abundant eosinophilic cytoplasm, with

some areas showing distinct Homer Wright rosettes. Im-neuron specific enolase (NSE), S-100, and vimentinand negative for CD56, actin, and cytokeratin. These munohistochemical stains were positive for CD99 (cyto-

plasmic surface membrane) and NSE, which confirmedfindings were consistent with a primitive neural tu-mor, favoring the diagnosis of neuroblastoma. The pa- the diagnosis of metastatic peripheral neuroepithelioma.

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TABLE 1Clinical and Pathologic Features of PNET of the Kidney

Age Metastases atPatient (yrs) Gender Race Symptoms diagnosis Pathology Treatment Outcome

1 18 M C Abd. pain RPLN, lung, LM: Homer Wright rosettes Sx: resection DOD, 14 mosbone IH: NSE /, S-100 /, Vim /, CD99 /, Chx: [IFO, CBP, VP-16] X 3

b2 mcg /, CD56 0, Act 0, Ker 0 [CYC, ADR] X 4 [IFO,t(11;22)(q24;q12) VP-16] X 4

Rtx: 3600 cGy abdomen1650 cGy lungs

2 20 M C Abd. pain, hematuria RPLN, lung LM: Homer Wright rosettes Sx: resection DOD, 4 mosIH: CD99 /, NSE/ Chx: [VCR, ADR, ActD] X 1

[IFO, VP-16] X 43 4 F C Abd. pain, fever RPLN, liver IH: S-100/, CD99/, NSE/, Ker/, Act0, Sx: Biopsy DOD, 1 mos

Vim0, Chromogr0 Chx: [IFO, CBP, VP-16] X 1EM: Microtubules, neurosecretory granules Rtx: 1400 cGy

4 14 M C Bone pain, weight Lung, bone, LM: Homer Wright rosettes On treatmentloss bone IH: CD99/, NSE/, Vim/, Synapto/, S-

marrow 1000, NFP0, Chromogr0, Ker0, Act0,PCR: EWS/FLI-1, EWS/ERG0EM: microtubules, neurosecretory granules

58 22 F C Abd. pain No LM: pseudorosettes Sx: resection DOD, 10 mosIH: NSE/, S-100/, Synapto0 Chromogr0, ChX: CYC, VCR, ADR

NFP0 Rtx: NSEM: neurosecretory granules

69 61 M C Abd. pain, weight RPLN IH: NSE/, Chromogr/, Ker0, Vim0, Sx: resection DOD, 6 mosloss Synapto0 Chx: VCR, ADR, CYC, ActD

710 24 M C Abd. pain, weight RPLN, lung LM: Homer Wright rosettes Sx: resection Dead after surgeryloss, hematuria IH: CD99/, NSE/, S-1000 NFP0,

Chromogr0, Ker0, Synapto0

PNET: primitive neuroectodermal tumor; M; male; F: female; C: caucasian; Abd: abdominal; RPLN: retroperitoneal lymph nodes; LM: light microscopy; IH: immunohistochemistry; EM: electron microscopy; PCR:

polymerase chain reaction; Sx: surgery; Chx: chemotherapy; Rtx: radiotherapy; cGy: centigrays; DOD: dead of disease; NS: not specified; VCR: vincristine; ActD: actinomycin D; CYC: cyclophosphamide; VP-16:

etoposide; ADR: doxorubicin; IFO: ifosfamide; CBP: carboplatin; NSE; neuron specific enolase; Vim: vimentin, b2 mcg: b2 microglobulin; Act: actin; ker: keratin; Chromogr: chromogranin; Synapto: synaptophysin;

NFP: neurofilament.

The patient received four courses of ifosfamide/etopo- clumps. Nuclei were ovoid, and contained prominenteosinophilic nucleoli. Cells contained amphophilic toside. However, his metastatic lung disease progressed,

and he developed bone marrow disease and died 4 slightly eosinophilic cytoplasm with occasional acido-philic paranuclear inclusion bodies. A diagnosis of ma-months after diagnosis. An autopsy was not performed.lignant rhabdoid tumor (MRT) was initially considered.Immunohistochemical stains were positive for cytokera-Case 3tin, NSE, S-100, and CD99 (cytoplasmic surface mem-A 4-year-old white female was referred to SJCRH inbrane), and negative for actin, vimentin, and chro-March 1993 with a 2-week history of a palpable rightmogranin. Electron microscopy showed microtubules,flank mass and low grade fever. Physical examinationneurosecretory granules, and intermediate filaments inrevealed a large mass extending 9 cm below the rightsome cells and cytoplasmic processes in many cells.costal margin. Laboratory evaluation included a lactateThese findings were consistent with PNET. The patientdehydrogenase (LDH) level of 1689 U/dL and a ferritinreceived an initial course of ICE but developed progres-level of 154 mg/dL. CT scan of the abdomen showed asive disease. She had no response to radiotherapy (14008.4 cm X 7.5 cm X 7 cm mass in the lower pole of thecGy) and died of multiorgan failure 3 weeks after diagno-right kidney, with extensive retroperitoneal lymph nodesis. Postmortem studies showed a chemo/radiothera-enlargement and multiple low attenuation lesions inpeutic effect, with marked rhabdoid features in the resid-both hepatic lobes. Both CT scan and ultrasonographyual tumor, and metastatic lesions.showed tumor extension into the right renal vein. CT

scan of the chest and 99mTc-MDP skeletal scintigraphy Case 4A 14-year-old white male was referred to SJCRH inwere normal. A core needle biopsy revealed extensive

areas of tumor necrosis and cells arranged in sheets and August 1996 with a 2-month history of generalized

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FIGURE 1. Case 1. (A) Mi-croscopic view of renal primi-tive neuroectodermal tumor(PNET). Numerous rosetteswere visible (H & E, originalmagnification 150). (B) CD99immunostain of renal PNET.There was strong membra-nous positivity. Stromal cellswere negative (avidin–biotin-complex technique, originalmagnification 1500).

bone pain, decreased appetite, and a 15-kg weight loss. heterogeneous mass arising from the upper pole ofthe right kidney, measuring 16 cm X 15 cm X 13 cmPhysical examination disclosed a firm mass, palpable

6 cm below the right costal margin. Laboratory evalua- and containing a small focus of calcification. A 99mTc-MDP skeletal scintigram showed multiple skeletal focition was significant for an LDH level of 1186 U/dL,

ferritin level of 1266 ng/mL, and NSE level of 87 ng/ of abnormally increased activity and uptake of radio-pharmaceutical within the tumor. A CT scan of themL. Urine catecholamine levels were within normal

limits. A CT scan of the abdomen demonstrated a large brain confirmed a left frontal skull metastasis with as-

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FIGURE 2. Case 2. (A) Contrast-enhanced computed tomography (CT) scanof the abdomen showed a large mass replacing most of the right kidney. Theunusual geometric areas of increased attenuation (also present on CT sectionsobtained before contrast injection) indicate calcification. (B) Skeletal equilib-rium phase of 99mTc-methylene diphosphonate scintigram (posterior view)showing abnormal activity in the tumor (arrow) arising from the superior poleof the right kidney.

sociated epidural soft tissue mass, without evidence hyperchromatic cells with indistinct cell margins andpale, fibrillar, amphophilic cytoplasm. Some areasof parenchymal involvement. A bone marrow aspirate

showed a bone marrow replaced by a metastatic small showed Homer Wright rosettes. Immunohistochemis-tries were positive for CD99 (cytoplasmic surfaceround cell tumor with extensive cohesive nests of tu-

mor cells. A core needle biopsy showed sheets of small membrane), NSE, vimentin, and synaptophysin, and

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negative for S-100, chromogranin, keratin, actin, des- lungs, there were several features in the patients inthe current study that were atypical for Wilms’ tumor,min, and neurofilament. Although these findings were

consistent with a diagnosis of PNET, the polymerase such as the presence of calcifications in three of thetumors, the tumor uptake of the bone-seeking radio-chain reaction did not show the presence of either

the EWS/FLI-1 or the EWS/ERG chimeric transcripts. pharmaceutical that was apparent in two cases (Fig.2B), and the bone metastases suggested by the skeletalElectron microscopy was thus performed, and showed

neurosecretory granules and microtubules. At last fol- scintigram in Cases 1 and 4.14 The presence of calcifi-cation and bone involvement were more consistentlow-up, the patient was undergoing treatment with

intensified chemotherapy with vincristine, ifosfamide, with neuroblastoma or, in younger patients, clear cellsarcoma of the kidney.cylophosphamide, doxorubicin, and etoposide, and

was scheduled to receive consolidation treatment with In one of the current study cases and in one re-ported case,9 a diagnosis of Wilms’ tumor of blastemicautologous bone marrow transplantation.type was made initially. The distinction betweenWilms’ tumor of blastemic type and PNET may beDISCUSSION

Renal PNET appears to be a unique clinical entity that difficult because Wilms’ tumor with blastemic ele-ments may occasionally test positive for CD99,15 andbehaves more aggressively than PNET arising at other

sites. The current series suggests that patients with renal tubules may also test positive.16 The diagnosisof renal PNET must be especially considered in casesrenal PNET are usually children and adolescents who

commonly have metastatic disease at presentation of adult Wilms’ tumor because the latter is most com-mon in young adults, an age group in whom PNET isand a poor response to therapy. All patients in the

current series received multimodal treatment shown also common, and these patients appear to have moreadvanced disease at diagnosis.17,18to be effective for PNETs at other locations (radical

surgery, radiation therapy, and chemotherapy includ- Several reports have documented the occurrenceof renal neuroblastoma, which also appears to be char-ing alkylating agents).5–7 However, three patients died

of progressive disease within 1 year of diagnosis. A acterized by extensive disease and unusually aggres-sive behavior.19–24 Interestingly, PNET may be histo-similar outcome is reported for the previously de-

scribed cases.8–11 In the brief NWTS report, only 3 of logically indistinguishable from classic neuro-blastoma, and also has been termed peripheral17 patients with Stages II to IV disease survived, 2

of whom had Stage IV disease and were treated with neuroblastoma.25 Neuroblastoma-like features mayoccur in PNETs26 and some patients may present withmyeloablative chemoradiotherapy followed by autolo-

gous bone marrow rescue.11 The absence of clinical a moderate increase in catecholamine excretion, se-rum ferritin, and plasma NSE.5 However, current im-response to an alkylator-based regimen in these pa-

tients may reflect a different biologic behavior with munohistochemistries, including CD99, can usuallyconfirm the diagnosis of PNET.27 Because CD99 hasinherent drug resistance.

The four cases of renal PNET in the current study only been available in recent years, it is possible thatsome of the previously reported cases of adult intrare-were confirmed by positive cytoplasmic surface mem-

brane staining for CD99 and by the presence of the nal neuroblastoma may in fact be PNET of the kidney.These cases presented in young adults, with normalt(11;22)(q24;q12) translocation in one of the two cases

tested. In the patient in Case 4, although the histologic or only mildly elevated urinary catecholamine levelsand very aggressive disease behavior.22–24 The behav-features were clearly consistent with the diagnosis of

PNET, polymerase chain reaction did not reveal the ior of renal PNET will be better defined as more casesare unequivocally identified via selective immunohis-presence of either the EWS/FLI-1 or the EWS/ERG fu-

sion transcripts. It is possible that this patient repre- tochemical staining of the cytoplasmic surface mem-brane with CD99 and/or the presence of the definingsents the 5% of patients with PNET in whom these

hybrid transcripts are not detected,3 and who have cytogenetic abnormalities.In one of the patients in the current study, a diag-other atypical cytogenetic abnormalities.13

The diagnosis of renal PNET must thus be consid- nosis of MRT was initially considered. The histologicdistinction between PNET and MRT can sometimesered in patients with renal neoplasms, particularly

those with advanced disease at presentation. It is espe- be difficult, due to the high phenotypic diversity ofthe latter,28 and the presence of different cytogeneticcially important to distinguish PNET of the kidney

from Wilms’ tumor, because therapeutic approaches abnormalities may be the only definitive factor.29–31

Weeks et al., in a histopathologic review of 56 renaland results of therapy are quite different in these twoentities. The findings of the current study revealed neoplasms mimicking MRT, reported 8 cases that ap-

peared to be PNET of the kidney and all had prominentimaging findings that may aid in the diagnosis. Al-though all renal neoplasms can metastasize to the filamentous cytoplasmic inclusions, the hallmark of

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11. Roloson GJ, Beckwith JB. Primary neuroepithelial tumorsMRT.32 These inclusions have been described in sev-of kidney in children and adults. A report from the NWTSeral tumors of neuroectodermal origin. In a review ofPathology Center [abstract]. Mod Pathol 1993;6:67.

42 cases diagnosed as extrarenal MRT, Parham et al.12. Webber BL, Parham DM, Drake LG, Wilimas JA. Renal tu-

found 4 tumors of neuroectodermal origin.33 Interest- mors in childhood. Pathol Annu 1992;1:191–232.ingly, the clinical course of PNET of the kidney is simi- 13. Murray JC, Langston C, Dreyer ZE, Stephenson CT, Pokornylar to that of MRT, characterized by a poor response WJ, Horowitz ME, et al. Atypical cytogenetic aberrations in

two childhood peripheral primitive neuroectodermal tu-to therapy and a mortality rate of 80%. However, MRTmors. Genes Chromosom Cancer 1995;12:142–7.usually occurs during the first 2 years of life.28 There

14. Gururangan S, Wilimas J, Fletcher BD. Bone metastases inalso is some suggestion that MRT may be of neuroec-Wilms’ tumor. Report of three cases and review of literature.

todermal origin,29,30 and it has been associated with Pediatr Radiol 1994;24:85–7.brain tumors of neuroectodermal origin.28,34 This hy- 15. Stevenson AJ, Chatten J, Bertoni F, Miettinen M. CD99 (p30/pothesis is reinforced by the presence of a 22q11.2 32MIC2) neuroectodermal/Ewing’s sarcoma antigen as an im-

munohistochemical marker. A review of more than 600 tu-breakpoint in close proximity to the EWS and NF2mors and the literature experience. Appl Immunohistochemgenes.31 Therefore, it is possible that the two tumors1994;2:231–40.

share the same primitive cell of origin, which may16. Weidner N, Tjoe J. Immunohistochemical profile of mono-

explain the similar clinical behavior. Further molecu- clonal antibody O13:antibody that recognized glycoproteinlar study of these two tumors is essential to elucidate p30/32MIC2 and is useful in diagnosing Ewing’s sarcoma and

peripheral neuroepithelioma. Am J Surg Pathol 1994;18:their relationship.486–94.The authors believe that PNET of the kidney con-

17. Kattan J, Tournade MF, Culine S, Terrier-Lacombe MJ, Drozstitutes a clinical entity different from typical PNET.JP. Adult Wilms’ tumour. Review of 22 cases. Eur J Cancer

It is characterized by very aggressive clinical behavior, 1994;30A:1778–82.which in some ways is similar to MRT. PNET must be 18. Arrigo S, Beckwith J, Sharples K, D’Angio G, Haase G. Betterincluded in the differential diagnosis of renal neo- survival after combined modality care for adults with Wilms’

tumor. A report from the National Wilms’ Tumor Study.plasms and must be differentiated from intrarenalCancer 1990;66:827–30.neuroblastoma, MRT, and Wilms’ tumor. Cytogenetic

19. Rosenfield NS, Leonidas JC, Barwick KW. Aggressive neuro-studies and immunohistochemistry for CD99 shouldblastoma simulating Wilms’ tumor. Radiology 1988;166:165–7.

be performed in any patient in whom PNET is consid-20. Panuel M, Bourliere-Najean B, Gentet JC, Scheiner C, De-

ered. larue A, Faure F, et al. Aggressive neuroblastoma with initialpulmonary metastases and kidney involvement simulatingWilms’ tumor. Eur J Radiol 1992;14:201–3.REFERENCES

1. Stout AP. A tumor of the ulnar nerve. Proc of the NY Pathol 21. Nisen PD, Rich MA, Gloster E, Valderrama E, Saric O, ShendeSoc 1918;18:2–12. A, et al. N-myc oncogene expression in histopathologically

2. Thiele CJ. Pediatric peripheral neuroectodermal tumors, on- unrelated bilateral pediatric renal tumors. Cancer 1987;61:cogenes, and differentiation. Cancer Invest 1990;8:629–39. 1821–6.

3. Delattre O, Zucman J, Melot T, Sastre Garau X, Zucker JM, 22. Ahmed I, Qureshi ID. Neuroblastoma of the kidney. J PakLenoir GM, et al. The Ewing family of tumors. A subgroup Med Association 1992;42:143–5.of small-round-cell tumors defined by specific chimeric 23. Verma L, Sandramouly S, Gary SP, Vashist S. Intrarenal neu-transcripts. N Engl J Med 1994;331:294–9. roblastoma presenting as orbital and multiple skeletal me-

4. Dehner LP. Primitive neuroectodermal tumor and Ewing’s tastases. Indian Pediatr 1993;30:673–6.sarcoma. Am J Surg Pathol 1993;17:1–13. 24. Gohji K, Nakanishi T, Hara I, Hamami G, Kamidona S. Two

5. Jurgens H, Bier V, Harms D, Beck J, Brandeis W, Etspuler cases of primary neuroblastoma of the kidney in adults. JG, et al. Malignant peripheral neuroectodermal tumors. A Urol 1987;137:966–8.retrospective analysis of 42 patients. Cancer 1988;61:349– 25. Shinoda M, Tsutsumi Y, Hata J, Yokohama S. Peripheral neu-57. roepithelioma of childhood. Immunohistochemical demon-

6. Marina NM, Etcubanas E, Parham DM, Bowman LC, Greenstration of epithelial differentiation. Arch Pathol Lab Med

A. Peripheral PNET (peripheral neuroepithelioma) in chil-1988;112:1155–8.

dren. A review of St. Jude experience and controversies in26. Schmidt D, Mackay B, Ayala AG. Ewing’s sarcoma with neu-diagnosis and management. Cancer 1989;64:1952–60.

roblastoma-like features. Ultrastruct Pathol 1982;3:143–51.7. Kushner BH, Hajdu SI, Gulati SC, Erlandson RA, Exeelby27. Pappo AS, Douglass EC, Meyer WH, Marina NM, ParhamPR, Lieberman PH. Extracranial primitive neuroectodermal

DM. Use of HBA-71 and anti beta-2-microglobulin to distin-tumors. The Memorial Sloan-Kettering Cancer Center expe-guish peripheral neuroepithelioma from neuroblastoma.rience. Cancer 1991;67:1825–9.Hum Pathol 1993;24:880–5.8. Chan YF, Llewellyn H. Intrarenal primitive neuroectodermal

28. Weeks DA, Beckwith JB, Mierau GW, Luckey DW. Rhabdoidtumor. Br. J Urol 1994;73:326–7.tumor of the kidney. A report of 111 cases from the National9. Mor Y, Nass D, Raviv G, Neumann Y, Natin O, GoldwasserWilms’ Tumor Study Pathology Center. Am J Surg PatholB. Malignant peripheral primitive neuroectodermal tumor1989;13:439–58.(PNET) of the kidney. Med Pediatr Oncol 1994;23:437–40.

29. Ota S, Crabbe DCG, Tran TN, Triche TJ, Shimada H. Malig-10. Mentzel T, Bultitude MI, Fletcher CDM. Primarer primitivenant rhabdoid tumor. A study with two established cell lines.neuroektodermaler tumor der niere bei einem erwachsenen.

Pathologe 1994;15:124–8. Cancer 1993;71:2862–72.

/ 7b57$$1103 05-07-97 09:09:02 canal W: Cancer

2250 CANCER June 1, 1997 / Volume 79 / Number 11

30. Handgretinger R, Kimmig A, Koscielnak E, Schimdt D, Ru- port from the National Wilms’ Tumor Study Pathology Cen-ter. Am J Surg Pathol 1991;15:1042–54.dolph G, Wolburg H, et al. Establishment and characteriza-

tion of a cell line (Wa–2) derived from an extrarenal rhab- 33. Parham DM, Weeks DA, Beckwith JB. The clinicopathologi-cal spectrum of putative extrarenal rhabdoid tumors. Andoid tumor. Cancer Res 1990;50:2177–82.

31. Newsham I, Daub D, Bernard-Guerin C, Cavenee W. Molec- analysis of 42 cases studied with immunohistochemistry orelectron microscopy. Am J Surg Pathol 1994;18:1010–29.ular sublocalization and characterization of the 11;22 trans-

location breakpoint in a malignant rhabdoid tumor. Geno- 34. Chang CH, Ramirez N, Sakr WA. Primitive neuroectodermaltumor of the brain associated with malignant rhabdoid tu-mics 1994;19:433–40.

32. Weeks DA, Beckwith JB, Mierau GW, Zuppan CW. Renal mor of the liver. A histologic, immunohistochemical andelectron microscopy study. Pediatr Pathol 1989;9:307–19.neoplasms mimicking rhabdoid tumor of the kidney. A re-

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