Downloaded from www.ajronline.org by 117.56.227.126 on 10/17/15 from IP address 117.56.227.126. Copyright ARRS. For personal use only; all rights reserved
339
Case Report
I.::fi
:‘
:
..
. .
‘
POEMS Syndrome: Dyscrasia Shashi Aggarwal,1’2 Michael J. Mitchell,4
..
A Rare
Rajinder Kumar Goulatia,1 and Alka Kumar1’2
.
.
Ajay
Sood,3
Report
A 35-year-old athy
of 5 years
man presented duration;
with progressive
it involved
all four
peripheral
limbs
and
was
neuropaccom-
Received July 6, 1989; accepted after revision February 20, 1990. 1 2
Department of Neuroradiology, Present address: Department
Aggarwal. 3 Department 4
Department
AJR 155:339-341,
of Neurology,
Centre,
All India Institute
of Medical
panied tia,
1990 0361-803X/90/1
552-0339
© American
Roentgen
:
..
:
#{149}
#{149}
revealed
loss,
Gulshan
and
of pedal
stimulating examination
spike
hormone revealed
impotence,
gynecomas-
Physical
axillary
Investigations on serum
Ahuja,3
edema.
bilateral
papilledema.
an M protein
Kumar
hypertrichosis,
episodes
hepatosplenomegaly,
and bilateral with
Prasad,3
#{149}
Cell
by hyperpigmentation,
weight
examination
lymphadenopathy,
revealed
electrophoresis,
hyperglobulinemia elevated
thyroid-
level, and non-Bence Jones proteinuria. CSF elevated protein levels. A myelogram obtained
elsewhere was reported to be normal. A cranial CT scan was normal. Decreased esophageal penistalsis was demonstrated on barium swallow. A skeletal survey detected several abnormalities. There was a large lytic lesion with an irregular, densely sclerotic margin in the left ilium (Fig. 1A). New bone was noted arising from the posterior elements of lumbar and lower dorsal vertebrae, diskovertebral junctions, lower costovertebral tibial and fibular margins,
demonstrated skin,
muscle,
A CT-guided
which
articulations, and pelvis
inferior border (Fig. 1 A). CT
of lower ribs, of the spine
this new bone in greater detail (Fig. 1 B). Biopsy of the lymph nodes failed to establish a uniform diagnosis.
and
biopsy
demonstrated
from
the
sheets
left
iliac
of plasma
lesion
was
cells.
After
then
performed,
irradiation
of the
iliac lesion and a course of immunosuppressive chemotherapy, the patient’s general status improved with resolution of the increased level of serum M protein.
Discussion Two other similar cases are illustrated. In one, a densely sclerotic fourth lumbar vertebra with prominent osseous proliferation was seen (Fig. 2), as was an irregular sclerotic lesion in the upper left femur; biopsy of the femur revealed mildly
Sciences, Ansari Nagar, New Delhi 1 10029, Rd., Halifax, Nova Scotia, Canada B3H 2Y9.
Sciences,
Ansari
of Radiology, Victoria General Hospital, Halifax, Nova Scotia, Canada B3H 2Y9. August
.
.
of Plasma
Kameshwar
Neurosciences Centre, All India Institute of Medical of Radiology, Victoria General Hospital, 1278 Tower
Neurosciences
.,
.
Variety
POEMS is an acronym, first proposed by Bardwick et al. [1 ], for a rare but unique multisystem disorder characterized by polyneunopathy (predominantly sensonimoton), onganomegaly (hepatosplenomegaly, lymphadenopathy), endocrinopathy (including gynecomastia, impotence, amenorrhea, diabetes mellitus, and hypothyroidism), elevated M protein level, and skin changes (such as hyperpigmentation, hypertnichosis, and thickening). Other major findings include papilledema, peripheral edema, ascites, and clubbing [2]. Affected patients may not exhibit all these features, and sometimes presentations are atypical. The syndrome was first reported in Japan more than two decades ago. Most patients have been of Japanese descent [2], but a number of recent reports, which indicates an increasing clinical awareness and lack of ethnic predilection of this entity, testify to its occurrence in North American, Europe, and Australia. No patients with this syndrome have previously been reported from India. Although POEMS syndrome has received much emphasis in the clinical literature, it has received little attention in the nadiologic literature. This is unfortunate, as awareness of the syndrome and its virtually diagnostic nadiologic manifestations may allow the radiologist to suggest the diagnosis first.
Case
.
Ray Society
Nagar,
New Delhi
1 1 0029,
India.
India. Address
reprint
requests
to S.
Downloaded from www.ajronline.org by 117.56.227.126 on 10/17/15 from IP address 117.56.227.126. Copyright ARRS. For personal use only; all rights reserved
Fig. 1.-A, Anteroposterior radiograph of pelvis shows large lytic lesion with well-defined, lobulated, sclerotic margin in left ilium. Spicules of new bone can be seen arising from transverse processes of fifth lumbar vertebra and superior margin of sacrum and ilium, and along obturator rings, more apparent on left side (arrowheads). B, Axial CT section through lower dorsal vertebra. New bone formation is
well observed along posterior elements (arrows). Other sections also showed new bone arising anterioriy along vertebral bodies.
Fig. 2.-56-year-old man who had had diabetes for 9 years and peripheral neuropathy, tightness of skin of both
hands, pedal edema, gynecomastia,
in-
creased hair growth, and skin darkening for 3 years. Serum electrophoresis
revealed increased M protein level. Radiograph shows sclerotic fourth lumbar vertebra. Spicules of new bone are prominent (arrowheads).
Fig. 3.-42-year-old man with 8-year history of peripheral neuropathy, hyperpigmentation, gynecomastia, clubbing, and axillary lymphadenopathy. There was mild hyperglobulinemia but no M protein component on serum electrophoresis. Radiograph of spine
shows new bone along posterior dcments (small arrows). Loss of normal smooth concavity of anterior border of vertebral bodies is due to irregular bony excrescences (large arrows). Pantopaque is seen in spinal canal. 2
hypercellular
3
bone
marrow
with
an
increased
number
of
plasma cells. A skeletal survey in the other case showed new bone arising from the lumbar spine (Fig. 3) and along the margins of the fibula; the study was otherwise unremarkable. In both patients, 99mTc radionuclide bone scans were normal exccpt for focal increased uptake of the radionuclide in the fourth lumbar vertebra and upper left femur in the first patient. The age at onset of the disease varies from 26 to 80 years; 25% of patients are younger than 40 years old [i 2]. It is more common in men than in women. All patients have a plasma cell dyscrasia that manifests itself as myeloma in more than half the patients and as extramedullary plasmacytoma, isolated monoclonal gammopathy, on isolated polyclonal gammopathy in the remainder [2]. The monoclonal gammopathy usually is due to lambda light chains. The number of plasma cells in the bone marrow is less than 5% in the great majority of patients [21. The treatment, which includes immunosup,
pressive chemotherapy and/or tumonicidal radiotherapy, often can produce remarkable results. There are several theories on the pathogenesis of this syndrome. It has been suggested that the plasma cells may secrete a substance, as yet unchanactenized, that is toxic to many organs [i 2]. Reulecke et al. [3] believe that POEMS syndrome is an immune disorder in which antibodies against the pituitary gland and perhaps also against related structures of the hypophyseohypothalamic axis induce secondary endocrinopathy and skin changes. This immunopathogenetic mechanism has been supported by others. Analogous to patients with AIDS, who frequently have lymphadenopathy, it has been hypothesized that an infectious agent (possibly viral) cannot be controlled because of immune deficiency, leading to its dissemination and generalized lymphadenopathy. This may subsequently progress to B cell neoplasia in the form of a plasma cell dyscrasia, which in turn is responsible for producing the POEMS syndrome [4]. The exact pathogenesis ,
of POEMS syndrome, however, still remain obscure. Pathologic examination of material obtained at biopsy (nerve, skin, lymph node, other tissues) and autopsy has failed to reveal any specific abnormality. Many patients with POEMS syndrome have skeletal abnonmalities in the form of nonspecific focal lesions (i.e., myeloma) and a subtle but specific pattern of bony proliferation Ii 2, 5]. Typically, the focal lesions are distributed primarily in the axial and proximal appendicular skeleton and are usually sclerotic, infrequently mixed lytic-sclenotic, and rarely purely lytic [2]. Osteosclerotic lesions appear as localized solid or targetlike areas of increased density. Mixed lytic-sclerotic lesions usually have a lytic center and a sclerotic rim. The differential diagnosis of localized sclerotic lesions commonly includes skeletal metastases; mastocytosis; tubenous sclenosis; and, with a solitary dense vertebral body, Hodgkin disease. But, these conditions are clinically distinct from POEMS syndrome, and the typical proliferative changes observed in POEMS syndrome are not observed in any of these disorders. However, in atypical cases of POEMS syndrome and especially in those not exhibiting the proliferative new bone, these conditions would have to be systematically excluded by appropriate investigations; biopsy may be required if the results are negative. It is distinctive that osteosclerosis predominates in this form of myeloma, as it is otherwise rare in untreated “classic” myeloma. Classic myeloma also differs from POEMS syndrome in that it has a later age of onset; a shorter survival time; presenting features are bone pain, anemia, or renal failure; and there is 2:1 predominance of kappa light chains over lambda light chains [6}. However, osteosclerotic myeloma, a rare variant of myeloma in which the bone lesions are sclerotic from the beginning, shares many of the clinical features of POEMS syndrome. It has been suggested that these two conditions, as well as certain other conditions characterized by osteosclerosis in association with plasma cell infiltration of bone such as plasma cell granuloma, chronic symmetric plasma cell osteomyelitis, and sternoclavicular hypenostosis, may represent an overlapping spectrum with the common pathogenetic mechanism of mechanism of an abnormal plasma cell secreting an osteoblast-inducing or osteoclast-inhibiting factor [7]. Proliferative osseous changes are the hallmark of the disease. These enthesopathic alterations are seen at both axial and extraaxial sites, including the apophyseal joints, laminae, transverse processes, diskovertebral junctions, costovertebral joints, obturator rings, upper margins of the ilium and sacrum, and along the tibia and fibula [5]. They impart to the bone an irregular, spiculated appearance that, while subtle, is virtually pathognomonic. In two of our patients, both types of lesions were demonstrated, whereas only proliferative new bone formation was seen in the third. Of all the sites, the dorsolumban spine appears to be involved most strikingly by this new bone. Although the new bone is most conspicuous in the posterior elements, it is also observed along the vertebral bodies. At the latter site, absence of degenerative changes in the spine and the relatively young age of the patient point toward its nondegenenative nature. Other enthesopathic entities commonly involving the spine, such as diffuse idiopathic skeletal hypenostosis, ossification of the posterior longitudinal liga-
Downloaded from www.ajronline.org by 117.56.227.126 on 10/17/15 from IP address 117.56.227.126. Copyright ARRS. For personal use only; all rights reserved
,
ment, and the senonegative spondyloarthritides (ankylosing spondylitis, Reiter syndrome, and psoriatic arthritis), are easily distinguished from POEMS syndrome by their different clinical features; associated skeletal findings; and the different size, site, and extent of new bone formation. In our experience, the delicate spicules of new bone arising from the posterior vertebral elements are unique to POEMS syndrome. This new bone is too subtle to be demonstrated by nadionuclide bone scanning, and, not surprisingly, the bone scans (obtained in two patients) did not show these changes. In contrast, Viard et al. [8] noted a diffuse hyperfixation of 99”Tc in their patient; the significance of this finding is unclean. Proliferative new bone formation in this syndrome was first documented by Bardwick et al. [1 ] and its distinctive nature emphasized by Resnick et al. [5, 7]. These proliferative changes have not been substantiated in subsequent reports, until this one. Therefore, it would appear that these findings are uncommon, but this may also be a reflection of the lack of awareness of these subtle changes. Alternatively, these changes may occur only in a particular subtype of POEMS syndrome. Whatever the explanation, these changes are specific for POEMS syndrome and should serve as a marker in its identification. In fact, in one patient bony proliferation was the sole radiologic manifestation, emphasizing that proliferative changes may occur in the absence of a focal lesion, hitherto unreported. Demonstration of these abnormalities on either routine radiographs or CT therefore is of major diagnostic importance, especially in patients in whom only a limited form of the syndrome is exhibited. Most patients with POEMS syndrome have a long history of medical problems. Apart from predisposing to complications from the underlying abnormalities, a delay in diagnosis may also increase the tumor cell burden. It is hoped that familiarity with the characteristic skeletal abnormalities observed in this syndrome may enable a radiologist to make an early diagnosis when treatment may effect a more positive outcome.
REFERENCES
1 . Bardwick PA, Zvaifler NJ, Gill GN, Newman D, Greenway Plasma cell dyscrasia with polyneuropathy, organomegaly, thy, M protein,
GD, Aesnick D.
endocrinopathe POEMS syndrome. Report on two of the literature. Medicine (Baltimore) 1980:59:
and skin changes:
cases and a review
311-322 2. Nakanishi T, Sobue I, Toyokura V. et al. The Crow-Fukase study of 1 02 cases in Japan. Neurology 1984:34:712-720
syndrome:
a
3. Reulecke M, Dumas M, Meier C. Specific antibody against neuroendocrine tissue in a case of POEMS syndrome with lgG gammopathy. Neurology 1988;38:614-616
4. Case records of the Massachusetts EnglJ Med
General Hospital (case 10-1987). N
1987:316:606-618
5. Resnick D, Greenway
GD, Bardwick
PA, zvaifler
NJ, Gill GN, Newman
DR. Plasma cell dyscrasia with polyneuropathy, organomegaly, nopathy, M-protein, and skin changes: the POEMS syndrome: radiographic abnormalities. Radiology 1981:140:17-22 6. Kyle RA. Multiple myeloma: review of 869 cases. Mayo 1975:50:29-40
7. Sartoris DJ, Pate sclerosis of bone: 25-34 8. Viard JP, Lesaure systemic sclerosis. giopathic
endocridistinctive C/in
Proc
D, Haghighi P. Greenway G. Resnick D. Plasma cell a spectrum of disease. Can Assoc Radio! J 1986:37:
P. Boitard C, et al. POEMS syndrome presenting as Clinical and pathologic study of a case with microanglomerular lesions. Am J Med 1988:84 : 524-528
339
Case Report
I.::fi
:‘
:
..
. .
‘
POEMS Syndrome: Dyscrasia Shashi Aggarwal,1’2 Michael J. Mitchell,4
..
A Rare
Rajinder Kumar Goulatia,1 and Alka Kumar1’2
.
.
Ajay
Sood,3
Report
A 35-year-old athy
of 5 years
man presented duration;
with progressive
it involved
all four
peripheral
limbs
and
was
neuropaccom-
Received July 6, 1989; accepted after revision February 20, 1990. 1 2
Department of Neuroradiology, Present address: Department
Aggarwal. 3 Department 4
Department
AJR 155:339-341,
of Neurology,
Centre,
All India Institute
of Medical
panied tia,
1990 0361-803X/90/1
552-0339
© American
Roentgen
:
..
:
#{149}
#{149}
revealed
loss,
Gulshan
and
of pedal
stimulating examination
spike
hormone revealed
impotence,
gynecomas-
Physical
axillary
Investigations on serum
Ahuja,3
edema.
bilateral
papilledema.
an M protein
Kumar
hypertrichosis,
episodes
hepatosplenomegaly,
and bilateral with
Prasad,3
#{149}
Cell
by hyperpigmentation,
weight
examination
lymphadenopathy,
revealed
electrophoresis,
hyperglobulinemia elevated
thyroid-
level, and non-Bence Jones proteinuria. CSF elevated protein levels. A myelogram obtained
elsewhere was reported to be normal. A cranial CT scan was normal. Decreased esophageal penistalsis was demonstrated on barium swallow. A skeletal survey detected several abnormalities. There was a large lytic lesion with an irregular, densely sclerotic margin in the left ilium (Fig. 1A). New bone was noted arising from the posterior elements of lumbar and lower dorsal vertebrae, diskovertebral junctions, lower costovertebral tibial and fibular margins,
demonstrated skin,
muscle,
A CT-guided
which
articulations, and pelvis
inferior border (Fig. 1 A). CT
of lower ribs, of the spine
this new bone in greater detail (Fig. 1 B). Biopsy of the lymph nodes failed to establish a uniform diagnosis.
and
biopsy
demonstrated
from
the
sheets
left
iliac
of plasma
lesion
was
cells.
After
then
performed,
irradiation
of the
iliac lesion and a course of immunosuppressive chemotherapy, the patient’s general status improved with resolution of the increased level of serum M protein.
Discussion Two other similar cases are illustrated. In one, a densely sclerotic fourth lumbar vertebra with prominent osseous proliferation was seen (Fig. 2), as was an irregular sclerotic lesion in the upper left femur; biopsy of the femur revealed mildly
Sciences, Ansari Nagar, New Delhi 1 10029, Rd., Halifax, Nova Scotia, Canada B3H 2Y9.
Sciences,
Ansari
of Radiology, Victoria General Hospital, Halifax, Nova Scotia, Canada B3H 2Y9. August
.
.
of Plasma
Kameshwar
Neurosciences Centre, All India Institute of Medical of Radiology, Victoria General Hospital, 1278 Tower
Neurosciences
.,
.
Variety
POEMS is an acronym, first proposed by Bardwick et al. [1 ], for a rare but unique multisystem disorder characterized by polyneunopathy (predominantly sensonimoton), onganomegaly (hepatosplenomegaly, lymphadenopathy), endocrinopathy (including gynecomastia, impotence, amenorrhea, diabetes mellitus, and hypothyroidism), elevated M protein level, and skin changes (such as hyperpigmentation, hypertnichosis, and thickening). Other major findings include papilledema, peripheral edema, ascites, and clubbing [2]. Affected patients may not exhibit all these features, and sometimes presentations are atypical. The syndrome was first reported in Japan more than two decades ago. Most patients have been of Japanese descent [2], but a number of recent reports, which indicates an increasing clinical awareness and lack of ethnic predilection of this entity, testify to its occurrence in North American, Europe, and Australia. No patients with this syndrome have previously been reported from India. Although POEMS syndrome has received much emphasis in the clinical literature, it has received little attention in the nadiologic literature. This is unfortunate, as awareness of the syndrome and its virtually diagnostic nadiologic manifestations may allow the radiologist to suggest the diagnosis first.
Case
.
Ray Society
Nagar,
New Delhi
1 1 0029,
India.
India. Address
reprint
requests
to S.
Downloaded from www.ajronline.org by 117.56.227.126 on 10/17/15 from IP address 117.56.227.126. Copyright ARRS. For personal use only; all rights reserved
Fig. 1.-A, Anteroposterior radiograph of pelvis shows large lytic lesion with well-defined, lobulated, sclerotic margin in left ilium. Spicules of new bone can be seen arising from transverse processes of fifth lumbar vertebra and superior margin of sacrum and ilium, and along obturator rings, more apparent on left side (arrowheads). B, Axial CT section through lower dorsal vertebra. New bone formation is
well observed along posterior elements (arrows). Other sections also showed new bone arising anterioriy along vertebral bodies.
Fig. 2.-56-year-old man who had had diabetes for 9 years and peripheral neuropathy, tightness of skin of both
hands, pedal edema, gynecomastia,
in-
creased hair growth, and skin darkening for 3 years. Serum electrophoresis
revealed increased M protein level. Radiograph shows sclerotic fourth lumbar vertebra. Spicules of new bone are prominent (arrowheads).
Fig. 3.-42-year-old man with 8-year history of peripheral neuropathy, hyperpigmentation, gynecomastia, clubbing, and axillary lymphadenopathy. There was mild hyperglobulinemia but no M protein component on serum electrophoresis. Radiograph of spine
shows new bone along posterior dcments (small arrows). Loss of normal smooth concavity of anterior border of vertebral bodies is due to irregular bony excrescences (large arrows). Pantopaque is seen in spinal canal. 2
hypercellular
3
bone
marrow
with
an
increased
number
of
plasma cells. A skeletal survey in the other case showed new bone arising from the lumbar spine (Fig. 3) and along the margins of the fibula; the study was otherwise unremarkable. In both patients, 99mTc radionuclide bone scans were normal exccpt for focal increased uptake of the radionuclide in the fourth lumbar vertebra and upper left femur in the first patient. The age at onset of the disease varies from 26 to 80 years; 25% of patients are younger than 40 years old [i 2]. It is more common in men than in women. All patients have a plasma cell dyscrasia that manifests itself as myeloma in more than half the patients and as extramedullary plasmacytoma, isolated monoclonal gammopathy, on isolated polyclonal gammopathy in the remainder [2]. The monoclonal gammopathy usually is due to lambda light chains. The number of plasma cells in the bone marrow is less than 5% in the great majority of patients [21. The treatment, which includes immunosup,
pressive chemotherapy and/or tumonicidal radiotherapy, often can produce remarkable results. There are several theories on the pathogenesis of this syndrome. It has been suggested that the plasma cells may secrete a substance, as yet unchanactenized, that is toxic to many organs [i 2]. Reulecke et al. [3] believe that POEMS syndrome is an immune disorder in which antibodies against the pituitary gland and perhaps also against related structures of the hypophyseohypothalamic axis induce secondary endocrinopathy and skin changes. This immunopathogenetic mechanism has been supported by others. Analogous to patients with AIDS, who frequently have lymphadenopathy, it has been hypothesized that an infectious agent (possibly viral) cannot be controlled because of immune deficiency, leading to its dissemination and generalized lymphadenopathy. This may subsequently progress to B cell neoplasia in the form of a plasma cell dyscrasia, which in turn is responsible for producing the POEMS syndrome [4]. The exact pathogenesis ,
of POEMS syndrome, however, still remain obscure. Pathologic examination of material obtained at biopsy (nerve, skin, lymph node, other tissues) and autopsy has failed to reveal any specific abnormality. Many patients with POEMS syndrome have skeletal abnonmalities in the form of nonspecific focal lesions (i.e., myeloma) and a subtle but specific pattern of bony proliferation Ii 2, 5]. Typically, the focal lesions are distributed primarily in the axial and proximal appendicular skeleton and are usually sclerotic, infrequently mixed lytic-sclenotic, and rarely purely lytic [2]. Osteosclerotic lesions appear as localized solid or targetlike areas of increased density. Mixed lytic-sclerotic lesions usually have a lytic center and a sclerotic rim. The differential diagnosis of localized sclerotic lesions commonly includes skeletal metastases; mastocytosis; tubenous sclenosis; and, with a solitary dense vertebral body, Hodgkin disease. But, these conditions are clinically distinct from POEMS syndrome, and the typical proliferative changes observed in POEMS syndrome are not observed in any of these disorders. However, in atypical cases of POEMS syndrome and especially in those not exhibiting the proliferative new bone, these conditions would have to be systematically excluded by appropriate investigations; biopsy may be required if the results are negative. It is distinctive that osteosclerosis predominates in this form of myeloma, as it is otherwise rare in untreated “classic” myeloma. Classic myeloma also differs from POEMS syndrome in that it has a later age of onset; a shorter survival time; presenting features are bone pain, anemia, or renal failure; and there is 2:1 predominance of kappa light chains over lambda light chains [6}. However, osteosclerotic myeloma, a rare variant of myeloma in which the bone lesions are sclerotic from the beginning, shares many of the clinical features of POEMS syndrome. It has been suggested that these two conditions, as well as certain other conditions characterized by osteosclerosis in association with plasma cell infiltration of bone such as plasma cell granuloma, chronic symmetric plasma cell osteomyelitis, and sternoclavicular hypenostosis, may represent an overlapping spectrum with the common pathogenetic mechanism of mechanism of an abnormal plasma cell secreting an osteoblast-inducing or osteoclast-inhibiting factor [7]. Proliferative osseous changes are the hallmark of the disease. These enthesopathic alterations are seen at both axial and extraaxial sites, including the apophyseal joints, laminae, transverse processes, diskovertebral junctions, costovertebral joints, obturator rings, upper margins of the ilium and sacrum, and along the tibia and fibula [5]. They impart to the bone an irregular, spiculated appearance that, while subtle, is virtually pathognomonic. In two of our patients, both types of lesions were demonstrated, whereas only proliferative new bone formation was seen in the third. Of all the sites, the dorsolumban spine appears to be involved most strikingly by this new bone. Although the new bone is most conspicuous in the posterior elements, it is also observed along the vertebral bodies. At the latter site, absence of degenerative changes in the spine and the relatively young age of the patient point toward its nondegenenative nature. Other enthesopathic entities commonly involving the spine, such as diffuse idiopathic skeletal hypenostosis, ossification of the posterior longitudinal liga-
Downloaded from www.ajronline.org by 117.56.227.126 on 10/17/15 from IP address 117.56.227.126. Copyright ARRS. For personal use only; all rights reserved
,
ment, and the senonegative spondyloarthritides (ankylosing spondylitis, Reiter syndrome, and psoriatic arthritis), are easily distinguished from POEMS syndrome by their different clinical features; associated skeletal findings; and the different size, site, and extent of new bone formation. In our experience, the delicate spicules of new bone arising from the posterior vertebral elements are unique to POEMS syndrome. This new bone is too subtle to be demonstrated by nadionuclide bone scanning, and, not surprisingly, the bone scans (obtained in two patients) did not show these changes. In contrast, Viard et al. [8] noted a diffuse hyperfixation of 99”Tc in their patient; the significance of this finding is unclean. Proliferative new bone formation in this syndrome was first documented by Bardwick et al. [1 ] and its distinctive nature emphasized by Resnick et al. [5, 7]. These proliferative changes have not been substantiated in subsequent reports, until this one. Therefore, it would appear that these findings are uncommon, but this may also be a reflection of the lack of awareness of these subtle changes. Alternatively, these changes may occur only in a particular subtype of POEMS syndrome. Whatever the explanation, these changes are specific for POEMS syndrome and should serve as a marker in its identification. In fact, in one patient bony proliferation was the sole radiologic manifestation, emphasizing that proliferative changes may occur in the absence of a focal lesion, hitherto unreported. Demonstration of these abnormalities on either routine radiographs or CT therefore is of major diagnostic importance, especially in patients in whom only a limited form of the syndrome is exhibited. Most patients with POEMS syndrome have a long history of medical problems. Apart from predisposing to complications from the underlying abnormalities, a delay in diagnosis may also increase the tumor cell burden. It is hoped that familiarity with the characteristic skeletal abnormalities observed in this syndrome may enable a radiologist to make an early diagnosis when treatment may effect a more positive outcome.
REFERENCES
1 . Bardwick PA, Zvaifler NJ, Gill GN, Newman D, Greenway Plasma cell dyscrasia with polyneuropathy, organomegaly, thy, M protein,
GD, Aesnick D.
endocrinopathe POEMS syndrome. Report on two of the literature. Medicine (Baltimore) 1980:59:
and skin changes:
cases and a review
311-322 2. Nakanishi T, Sobue I, Toyokura V. et al. The Crow-Fukase study of 1 02 cases in Japan. Neurology 1984:34:712-720
syndrome:
a
3. Reulecke M, Dumas M, Meier C. Specific antibody against neuroendocrine tissue in a case of POEMS syndrome with lgG gammopathy. Neurology 1988;38:614-616
4. Case records of the Massachusetts EnglJ Med
General Hospital (case 10-1987). N
1987:316:606-618
5. Resnick D, Greenway
GD, Bardwick
PA, zvaifler
NJ, Gill GN, Newman
DR. Plasma cell dyscrasia with polyneuropathy, organomegaly, nopathy, M-protein, and skin changes: the POEMS syndrome: radiographic abnormalities. Radiology 1981:140:17-22 6. Kyle RA. Multiple myeloma: review of 869 cases. Mayo 1975:50:29-40
7. Sartoris DJ, Pate sclerosis of bone: 25-34 8. Viard JP, Lesaure systemic sclerosis. giopathic
endocridistinctive C/in
Proc
D, Haghighi P. Greenway G. Resnick D. Plasma cell a spectrum of disease. Can Assoc Radio! J 1986:37:
P. Boitard C, et al. POEMS syndrome presenting as Clinical and pathologic study of a case with microanglomerular lesions. Am J Med 1988:84 : 524-528
