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CANCER April 1, 1998 / Volume 82 / Number 7
that techniques are available that can find a single cancer
cell within 1–10 million lymphocytes, we must now go
back to the drawing board and describe lymph node positivity by the method used to determine it. For example,
a patient with a T1 lesion may be N0 by H & E staining
but N1 by IHC results. Does this patient have Stage I
(T1N0) breast carcinoma or stage IIA (T1N1) disease (or
something in between)? Staging is valuable only when it
reflects prognosis correctly, thereby affecting treatment.
As ever more sensitive techniques, such as polymerase chain reaction (PCR), become more widely used, we
will have cases that are N0 (by H & E) , N0 (by IHC) , and N1 (by
PCR) . These new data will have to be equated with outcome
to determine the proper stage. In the meantime, lymph
node positivity must be recorded by the method used
to determine it. At some point in time, it may become
necessary to rework the staging system to reflect the
method used to determine lymph node involvement.
Silverstein MJ, Barth A. Use of primary breast carcinoma
characteristics to predict lymph node metastases–Reply.
Cancer 1997;79:1862–4.
Melvin J. Silverstein, M.D.
The Breast Center
Van Nuys, California
Is Primitive Neuroectodermal Tumor
of the Kidney a Distinct Entity?
Although no PNET of the kidney was documented in
these reports, these data show clearly that patients
with metastatic peripheral PNET are hardly curable
regardless of the site of the primary tumor.
All four patients described by Rodriguez-Galindo et
al. had primary metastases and extensive local tumor
volume at diagnosis. We believe that the advanced stage
of disease strongly influenced the outcome of these patients, which was not different from the outcome of patients with metastatic peripheral PNET of other locations.2–4 PNET is histologically characterized by poorly
differentiated, small, round, and blue cells forming
Homer–Wright rosettes or pseudorosettes.2,3,5,6 Immunohistochemically, tumor cells stained positive to various degrees for different neural markers, such as protein
S-100, Leu 7 (HNK-1), and particularly neuron specific
enolase.5,6 Further characterization is achieved with
CD99 (HBA-71; O 13), a monoclonal antibody that can
detect a recently described cell membrane glycoprotein
(p 30/32MIC2).6 The balanced t(11;22)(q24;q12) chromosomal translocation is a typical genotypic marker unique
to tumors of the Ewing’s sarcoma family.2,7 PNET of the
kidney generally shows these characteristics,1,8,9,10 which
are observed primarily in nonrenal PNET, indicating that
renal and nonrenal PNET do not differ with regard to
morphologic, immunohistochemical, and cytogenetic
features. Thus, we do not believe that the location alone
justifies defining PNET of the kidney as a distinct entity
unless additional clinical characteristics or specific biologic markers are established that allow the distinction
of renal PNET from nonrenal PNET.
e read the article by Rodriguez-Galindo et al.1 and
agree with the authors that primitive neuroectodermal tumor (PNET) of the kidney is generally associated with an unfavorable prognosis and poor response
to conventional combined-modality treatment. They
conclude that renal PNET is a distinct clinical entity
with more aggressive behavior than PNET at other locations. We believe, however, that peripheral PNET is
associated with a poor prognosis irrespective of tumor
location if patients present with advanced local or
metastatic disease. In the study by Kushner et al., all
patients (11 of 54) with nonrenal PNET who had distant metastases at diagnosis died.2 In another study,
9 of 10 patients with nonrenal PNET and primary metastases progressed or relapsed, and only 1 patient
survived.3 In addition, 8 of these 10 patients with initial
metastases had tumors with a volume above 100 mL.3
In the series of Marina et al., 9 of 10 patients with
retroperitoneal or pelvic PNET had Stage III or IV disease, and only 1 of them was a long term survivor.4
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03-11-98 15:52:08
Rodriguez-Galindo C, Marina NM, Fletcher BD, Parham DM,
Bodner SM, Meyer WH. Is primitive neuroectodermal tumor
of the kidney a distinct entity? Cancer 1997;79:2243–50.
Kushner BH, Hajdu SI, Gulati SC, Erlandson RA, Exelby PR,
Lieberman PH. Extracranial primitive neuroectodermal tumors: the Memorial Sloan-Kettering Cancer Center experience. Cancer 1991;67:1825–9.
Jürgens H, Bier V, Harms D, Beck J, Brandeis W, Etspüler G, et al.
Malignant peripheral neuroectodermal tumors: a retrospective
analysis of 42 patients. Cancer 1988;61:349–57.
Marina NM, Etcubanas E, Parham DM, Bowman LC, Green
A. Peripheral primitive neuroectodermal tumor (peripheral
neuroepithelioma) in children: a review of the St. Jude experience and controversies in diagnosis and management.
Cancer 1989;64:1952–60.
Schmidt D, Herrmann C, Jürgens H, Harms D. Malignant
peripheral neuroectodermal tumor and its necessary distinction from Ewing’s sarcoma: a report from the Kiel Pediatric Tumor Registry. Cancer 1991;68:2251–9.
Ambros IM, Ambros PF, Strehl S, Kovar H, Gadner H, SalzerKuntschik M. MIC2 is a specific marker for Ewing’s sarcoma
and peripheral primitive neuroectodermal tumors: evidence
for a common histogenesis of Ewing’s sarcoma and peripheral primitive neuroectodermal tumors from MIC2 expression and specific chromosome aberration. Cancer 1991;67:
W: Cancer
Whang-Peng J, Triche TJ, Knutsen T, Miser J, Douglass EC,
Israel MA. Chromosome translocation in peripheral neuroepithelioma. N Engl J Med 1984;311:584–5.
8. Chan YF, Llewellyn H. Intrarenal primitive neuroectodermal
tumor. Br J Urol 1994;73:326–7.
9. Sheaff M, McManus A, Scheimberg I, Paris A, Shipley J, Baithun S. Primitive neuroectodermal tumor of the kidney confirmed by fluorescence in situ hybridization. Am J Surg Pathol 1997;21:461–8.
10. Marley EF, Liapis H, Humphrey PA, Nadler RB, Siegel CL, Zhu X,
et al. Primitive neuroectodermal tumor of the kidney—another
enigma: a pathologic, immunohistochemical, and molecular diagnostic study. Am J Surg Pathol 1997;21:354–9.
Martin Benesch, M.D.
Christian Urban, M.D.
Division of Pediatric Hematology/Oncology
University Children’s Hospital
University of Graz
Graz, Austria
Author Reply
We have read with great interest the comments made
by Benesch and Urban on our recent description of
the distinctive features of primitive neuroectodermal
tumor (PNET) of the kidney.1 We agree that PNET of
the kidney should be considered part of the Ewing’s
sarcoma family of tumors (ESFT), sharing the same
histologic, cytochemical, cytogenetic, and molecular
features.2,3 Our purpose in describing these patients
was twofold: first, we wished to inform oncologists and
pathologists that PNET may present as a primary renal
tumor; and second, we suggested that PNET arising in
the kidney may have an aggressive clinical course.
PNET arising primarily in the kidney is rare. As
our series illustrates, this entity must be included in
the differential diagnosis of primary renal tumors, particularly in children and young adults. Furman et al.4
noted that lymphoma, carcinoid tumors, small cell
neuroendocrine carcinoma, and rhabdomyosarcoma
may present as primary small cell tumors of the kidney. As we discussed, Wilms’ tumor of blastemic type,
renal neuroblastoma, and malignant rhabdoid tumor
may share histologic features with PNET. Benesch and
Urban reiterate our recommendation that immunohistochemistry for CD99 is mandatory in such cases,
and we agree that molecular characterization of these
tumors should be performed when tissue is available.
Four other recent case reports also note the rarity
of this tumor and the importance of considering renal
PNET a distinct clinical entity.4 – 7 Of the 11 cases described (8 with metastatic disease at diagnosis), 1 patient died after surgery, 7 patients died of disease progression at a median of 5 months after diagnosis, and
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03-11-98 15:52:08
the 3 survivors were still receiving treatment at the
time the reports were made.1,4 – 10
Given the small number of cases of PNET of the
kidney, statistically significant comparisons of the outcomes of patients with ESFT by this primary site are
not possible. Nevertheless, PNET of the kidney appears to represent a subgroup with very aggressive
behavior. We agree that patients with advanced local
or metastatic disease are at higher risk of overall treatment failure; however, PNET, arising at other sites typically respond well to initial therapeutic interventions.
Kushner et al.11 reported a 20-year retrospective series
that excluded a number of long term survivors with
PNET ‘‘because their small tumors were inadequate
for confirming the diagnosis.’’ In that report, 9 of 11
patients who received more that 1200 mg/m2/course
of cyclophosphamide initially responded to chemotherapy. Using very aggressive short term therapy, this
group recently reported initial responses in all 32 evaluable patients with peripheral PNET, 77% event free
survival in 24 patients with locoregional disease, and
5 of 6 patients with lung metastases progression free.12
This report suggests that patients with metastatic peripheral PNET may be curable with aggressive therapy.
In other reports, 13 of 14 patients13 and 16 of 17 patients14 with peripheral PNET responded to initial
combination chemotherapy. Although we previously
reported poor outcome for patients with PNET of soft
tissues,15 we have recently updated these data.16 In
our recent group of 17 patients with soft tissue PNET
treated since 1988, all 9 patients evaluable for initial
response to chemotherapy responded. The 5-year progression free survival is 62 { 16% for the entire group,
including 8 patients with tumors ú8 cm and 3 with
metastatic disease at diagnosis. In contrast, 2 of our
patients with renal PNET, all diagnosed after 1990 and
receiving modern, aggressive therapy, showed primary
resistance to agents that are very active against the
ESFT, dying 1 and 4 months after diagnosis. The patients described by Scheaff et al.,6 Chan and Llewellyn,8 and Mor et al.,9 2 with localized disease and 1 with
disease spread only to retroperitoneal lymph nodes, all
died within 10 months of diagnosis.
Our series and the four other new case reports4 – 7
suggest that PNET of the kidney is a distinct clinical
entity that must be distinguished from other primary
renal tumors. Therapeutic approaches for this tumor
should be the same as for other sites of ESFT, but may
be less successful.
Rodriguez-Galindo C, Marina NM, Fletcher BD, Parham DM,
Bodner SM, Meyer WH. Is primitive neuroectodermal tumor
of the kidney a distinct entity? Cancer 1997;79:2243–50.
Dehner LP. Primitive neuroectodermal tumor and Ewing’s
sarcoma. Am J Surg Pathol 1993;17:1–13.
W: Cancer
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