CERVICAL LYMPH NODE METASTASIS AS THE FIRST

MANIFESTATION OF LOCALIZED EXTRAMEDULLARY

PLASMACYTOMA

Two patients who had cervical lymph node metastases as the first symptom of a
localized soft tissue extramedullary plasmacytoma of the epiglottis and
nasopharynx, respectively, are described. Classical multiple myeloma was ex-
cluded by bone marrow biopsies and x-ray studies of the skeleton. In both
patients, the primary tumor was diagnosed 2 years after excision of the cervical
lymph node. One patient had a IgD, X myeloma protein in the serum and
excreted X Bence-Jones protein into the urine. No M-component was found in
the other patient. Both are living with a long survival of 8 and 4 years,
respectively. The presence of a cervical lymph node plasmacytoma should
suggest an upper respiratory tract o r oropharynx plasmacytoma rather than a
primary lymph node plasmacytoma.
Cancer 38:1641-1644, 1976.

S OF?' TISSL'TE EX I'RAMEllL'I.I.AK\' 'Pl.ASMAC\'- examine. Therefore, the first indication of a neo-
tomas may originate in any tissue except plasm in this area may be metastases to cervical
bone marrow as a primary neoplasm of plasma lymph nodes.4
cell^.^^'^ Like plasma cells of classical multiple We describe here two patients whose primary
myeloma, they usually secrete M-components tumors were not diagnosed until 2 years after
that can be detected in serum, urine, or both, by cervical lymph node metastases had occurred, in
either conventional electrophoresis or by im- spite of repeated examinations. O n e patient had
munoelectrophoresis against specific a n t i ~ e r a . ~a plasmacytoma originating in the epiglottis
T h e quantity of hl-components in the serum is that synthesized an IgD, X myeloma protein; the
in part a function of the tumor mass.' Since other had a plasmacytoma of the nasopharynx
extramedullary plasma cell tumors may occur without an h.1-component.
without evidence of multiple myeloma, low lev-
els of hi-components may be formed; this may CASEREPORTS
explain the absence of h/l-components in many
patients with localized extramedullary plas- Case 1
macytomas.' About 75% of soft tissue plasmacy- A 74-year-old man (BULI) was admitted to Veter-
tomas arise in the upper respiratory tract and ans Administration Center-Wadsworth General Hos-
o r o p h a r y n ~ , a~n~ anatomic
'~ area that may har- pital in October 1970, because of a slowly enlarging
bor occult epithelial neoplasms and is difficult to mass on the right side of his neck. His voice occasion-
ally was hoarse in late afternoon, but he denied hav-
ing pain, hemoptysis, voice change, or dysphagia.
From Laboratory Service, \'A(:-Ltadsworth General Hos- l ' w o years previously, a cervical lymph node plas-
pital. 1.0s l\ngcles. Ci\90073; I k p a r t m e n t of Pathology.
UC:I.I\ Srhool of Xledicine. 1.0s Angeles, C X 90024; and
macytoma had been removed under endotracheal
Ikpartmrnt of Immunopathology, Scripps Clinic and Ke- anesthesia a t another hospital. T h e anesthesiologist
searrh Foundation, La Jolla. noted a 1 X 0.5-cm edematous area in the posterior
Supported by \'/\ Project epiglottis but no further investigation occurred as the
C;nited States Public llealth Service ('41-10734) and the patient did not appear for follow-up appointments.
r\rnc.riran l i e a r t ,\ssoriation (73-753). I'ublication No. 980 O n examination, the neck mass was firm and lobu-
lrom Srripps C:linic and Research Foundation. lated but not tender and it measured 13 X 8 X 3 cm.
Address for reprints. U. G . Fishkin, A l l ) , Laboratory A 2 X 3-cm dark reddish smooth pedunculated tumor
Srrviw. \'.\(:-LVadsuorth Genrral Hospital, Los Angeles, was found a t the right margin of the epiglottis. T h e
(:!\ 00071
'l'hc ;inthors thank I h . l i . Jordan K . Parsa a n d C. J . skeleton was normal by x-ray survey. Bone marrow
l,a(;r;iriqe for providing the information o n patient U'YA, aspirations and biopsies from the right and left poste-
and 111.. ,I. Xlills for skillful technical assistance. rior iliac spines showed only 1-2% mature plasma
Iieceibed lor publication January 1.5, 1976. cells. Laboratory tests revealed a 4+ proteinuria and
1641
 10970142, 1976, 4, Downloaded from https://acsjournals.onlinelibrary.wiley.com/doi/10.1002/1097-0142(197610)38:4<1641::AID-CNCR2820380433>3.0.CO;2-Y, Wiley Online Library on [29/01/2024]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
1642 CANCER
October 1976 VOl. 38

t I

NHS
FIG. 1. Cellulose acetate elec-

B UR 10 / 7 0 t r o p h o r e s i s of n o r m a l s e r u m
(NHS), serum of patient B U R ob-
tained in October 1970 and Sep-
tember 1972, and serum of patient
WYA obtained in 1973.

BUR 9 / 7 2
WYA

a small monoclonal spike in the fast gamma region of plasma cells, survey of the skeleton was negative, and
the serum when tested by cellulose acetate elec- no palpable lymph nodes were found in the neck area.
trophoresis (Fig. 1). Radial immunodiffusion' and Bone marrow aspirations and biopsy showed only
irnmunoelectrophoresis'O showed a n IgD, h (308 occasional mature plasma cells. A technicium pyro-
mg/100 ml) M-protein, and the urine contained 38 phosphate scan showed increased uptake in the T 7
mg/100 ml h chains and 8 mg/100 ml K chains (Table and T9, but skeletal x-rays showed only osteoporosis.
1).
Histologic examination of the epiglottis tumor Case 2
showed a plasmacytoma; with immunofluorescent A S6-year-old woman (WYA) was admitted to an
staining," most of the tumor's plasma cells were posi- outside hospital in September 1973, because her right
tive for IgD and h chains (Fig. 2). Cobalt irradiation ear had been stuffy for several months and a bulging
(tumor dose 6415 rads) produced shrinkage of the right soft palate was detected a week earlier. Several
neck mass and a normal epiglottis. Concurrently, the previous examinations by EN'T consultants had not
serum M-protein and the Bence-Jones proteinuria disclosed any abnormality.
disappeared within 4 months (Table 1 ) . T h e patient Two years previously, a 4 X 3 X 2-cm plasmacy-
felt well over the ensuing years. In subsequent quan- toma of a right posterior cervical lymph node had
titations of the serum immunoglobulins, the IgD con- been excised, which had grown slowly over a 2-year
centration rose from 5 mg/ 100 ml in January 1972 to period. T h e physical examination and laboratory
70 mg/100 ml in December 1974. Since this suggested tests, including serum electrophoresis, analyses of ur-
recurrence of the plasmacytoma, the patient was ine for Bence-Jones protein, bone marrow aspira-
readmitted to the hospital. Two of five epiglottis tions, bone and liver scans, and an x-ray survey of the
biopsies showed a moderate increase in mature skeleton, were all negative. The right neck area was
irradiated with cobalt-60 (tumor dose 3400 rads).
T h e patient's serum and concentrated urine, exam-
TABLE
1. Immunoglobulin Concentrations ined periodically by conventional and immunoelect-
(mg/100 mi) In Patient BUR rophoresis, were always normal (Fig. 1).
Serum Urine Physical examination revealed a mild right serous
Date IgD IgG IgA IgM x X otitis media and a firm nasopharyngeal mass that
could be exposed by passage of catheters through the
12/1/70 308 1100 360 90 8 38
nose and into the mouth to support the soft palate. All
1/11/71 350 1000 250 70 10 105
2/9/71 188 laboratory and x-ray studies were normal.
2/18/71 43 A 1 X 1.5 X 2-cm plasmacytoma was removed from
6/30/71 5 1300 255 150 14 4 the nasopharynx causing profuse bleeding and requir-
1/3/72 5 1200 430 145 10 0 ing sutures. Subsequently, the patient's nasopharynx
2/27/73 11 885 300 110 received 4600 rads from a 4 MEU linear accelerator.
4/15/74 28 1285 380 150 Melphalan continues to be administered despite the
6/26/74 41 1315 440 180 10 5 absence of detectable metastasis or other evidence of
12/4/74 70 1400 290 180 5 7
disease.
 10970142, 1976, 4, Downloaded from https://acsjournals.onlinelibrary.wiley.com/doi/10.1002/1097-0142(197610)38:4<1641::AID-CNCR2820380433>3.0.CO;2-Y, Wiley Online Library on [29/01/2024]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
No. 4 EXTRAMEDULLARY
LOCALIZED PLAsMAcY'roMA Fishkin and Spiegelberg 1643

FIG. 2. Immunofluorescent staining of an epiglottis tumor biopsy with fluoresceinisothiocyanate-

coupled rabbit antisera specific for IgD, IgG, A, and K chains.

toma. l'herefore, the presence of a plasma cell

These two patients illustrate that localized
upper respiratory tract extramedullary plas-
macytomas may behave like epithelial carci-
nomas of that area by exhibiting cervical lymph
node metastases as the first symptom of tumor.
More than 60% of upper respiratory tract and
oropharynx epithelial carcinomas metastasize to
cervical lymph nodes; this was the first clinical
manifestation in 870,~15%,' and 25%4 of such
patients. Similarly, extramedullary plasmacy-
tomas located in the same region metastasized
to cervical lymph nodes in 21% (4/19) of Wilt-
shaw'sI3 personal cases; no patients like the ones
described here have been reported, where lymph
node metastases were the first symptoms of a n
apparently localized extramedullary plasmacy-
toma. Primary extramedullary plasmacytomas
of lymph nodes are rare. Dolan and Dewar'
reported only 4 among 161 and W i l t ~ h a w '12
~ insensitivity of the methods employed presum-
among 257 cases of extramedullary plasmacy-
neoplasm in a cervical lymph node should lead
one to suspect a primary tumor in the upper
respiratory tract and oropharynx; this region
should be carefully examined by a n expert. This
may provide a n opportunity for cure when the
area of primary disease is included in the radio-
therapy treatment field. T h e disease course of
our patients also demonstrates the disparity in
the prognosis of epithelial and plasma cell neo-
plasms. Patients with cervical lymph node me-
tastases of epithelial carcinomas have a short
s u r ~ i v a lwhereas
,~ lymph node metastases from
localized plasmacytomas may be associated
with a long survival.l 2 T h e survival times of our
patients to date are 8 and 4 years, respectively.
T h e presence of a n M-component in the se-
rum, urine, or both, may indicate a localized
extramedullary plasmacytoma rather than mul-
tiple mye10ma.~T h e small tumor size and the
ably account for the low incidence of M-com-

1644 CANCER
October 1976
ponents in most reported cases of extra-
medullary plasmacytomas. A 20-g tumor may
produce only 0.2 g/100 ml of a serum M-com-
p ~ n e n twhich
,~ is the lowest quantity that can be
detected by careful cellulose acetate or agar gel
electrophoresis. Immunochemical methods are
more sensitive for the detection of M-com-
ponents in serum and concentrated urine, and
the use of fluoresceinated anti-immunoglobulin
antisera on tumor imprints or sections may
identify the presence of synthesized M-com-
ponents. O n several occasions, immunochemical
studies on serum and concentrated urine from
patient WYA did not demonstrate an M-com-
ponent because af the low tumor mass or per-
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