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POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, multiple myeloma and skin changes) with cranial vault plasmocytoma and the role of surgery in its management: a case report
© Plata Bello and Garcia-Marin; licensee BioMed Central Ltd. 2013
Received: 6 April 2013
Accepted: 29 August 2013
Published: 18 October 2013
POEMS syndrome (an acronym of polyneuropathy, organomegaly, endocrinopathy, multiple myeloma and skin changes) is a paraneoplastic disorder related to an underlying plasma cell dyscrasia. The development of such a syndrome is rare and its association with calvarial plasmocytoma is even less common, with only two previous reported cases. We describe, in detail, an unusual presentation of cranial plasmocytoma associated with POEMS syndrome and briefly discuss the possible role of surgery in the management of this disease.
We present the case of a 45-year-old Caucasian man who was admitted to our department presenting with progressive weakness in his lower limbs, enlarged lymph nodes and a large mass on the scalp with intense bone erosion. POEMS criteria were present and pathological studies confirmed a Castleman’s variant plasmocytoma. Clinical status improved noticeably after the excision of the plasmocytoma and the treatment was completed with radiotherapy and steroid pulse therapy.
Cranial vault plasmocytoma and its association with POEMS syndrome are rare conditions with few previously reported cases. Although the role of surgery is not clearly defined in POEMS syndrome guidelines, the fact that there seems to be a better prognosis and clinical outcome when surgery is used as a part of the management in POEMS syndrome with cranial vault plasmocytoma is worth discussing.
Criteria for the diagnosis of POEMS syndrome
Mandatory major criteria
Monoclonal plasmaproliferative disorder
Other major criteria
Sclerotic bone lesions
Elevated serum or plasma vascular endothelial growth factor (VEGF) levels
Organomegaly (splenomegaly, hepatomegaly, or lymphadenopathy)
Extravascular volume overload (peripheral edema, ascites or pleural effusion)
Endocrinopathy (adrenal, thyroid, pituitary, gonadal, parathyroid, pancreatic)
Skin changes (hyperpigmentation, hypertrichosis, plethora, hemangiomata, white nails)
Thrombocytosis or polycythemia
We report the case of a patient who suffered a POEMS syndrome associated with a cranial vault plasmocytoma managed with surgery, radiotherapy and steroids.
A 45-year-old Caucasian man was admitted to the Accident and Emergency Department of our hospital after presenting with a two-week history of progressive walk impairment associated with paresthesias around his mouth and the fingers on his right hand. The most remarkable features of his medical history were the presence of type 2 diabetes (recently diagnosed), dislipemia, high blood pressure and morbid obesity (body mass index: 41.3).
A medical examination showed a lower limb paresis (grade 3/5), which was more prominent in dorsal flexion of both feet, an absence of reflexes and a loss of superficial sensitivity in his left limb. The presence of enlarged lymph nodes in the cervical area was also evident; they were variable in size (some of them of more than 1cm) and mobile. Furthermore, a large scalp mass was identified in the parietal-occipital area; it was stuck to the skull, had an irregular shape, a tough texture, and palpation was not painful. Apart from these findings, the presence of a bilateral gynecomastia and a hyperpigmentation of the skin, mostly of the areoles, were noteworthy.
An electromyography (EMG) test was performed to better determine the neurological symptoms. The EMG showed a motor-dominant polyneuropathy with demyelinating features in his lower limbs. The administration of steroids only partially improved his neurological symptoms.
Laboratory test results at our patient’s initial assessment
Blood cell count
Red blood cell count
White blood cell count
Erythrocyte sedimentation rate
Immunoglobulin G (IgG)
Immunoglobulin A (IgA)
Immunoglobulin M (IgM)
Kappa light chain (plasma)
Lambda light chain (plasma)
Vascular endothelial growth factor (VEGF)
Lambda light chains are observed; alternatively, a homogeneous IgG band is shown.
Carcinoembryonic antigen (CEA)
Thyroid-stimulating hormone (TSH) (basal)
Thyroxine (T4) (free)
(0.70 - 1.85)
The decision was taken to surgically remove the parietal-occipital mass, with a previous embolization of the main arteries feeding the tumor in order to avoid excessive bleeding during surgery. The patient was operated on under general anesthesia. We performed a circular craniectomy around the tumor and a complete excision of the lesion by dissecting its adherences with the dura mater, which did not seem to be damaged (Figure 2a and 2b). A titanium mesh was placed in the osseous defect.
Pathological analyses concluded that the tumor was a well-differentiated plasmocytoma with expression of CD38, IgG and lambda light chain (Figure 2c). A bone marrow biopsy showed reactive changes with intense plamocytosis.
Subsequently, according to the definitive pathological results, dexamethasone pulse therapy was initiated (three pulses per month of 20mg/day for four days) and the patient also received radiotherapy to the skull to complete the treatment, with an accumulated dose of 50Gy in the surgical field.
As mentioned above, his neurological status improved slightly with the initial prescription of steroids, but neurological deficits recovered progressively after surgery. The rest of the POEMS features also improved after surgery and even more so when steroids and radiotherapy were initiated (two weeks after surgery).
We report a rare case of a Castleman’s disease variant of POEMS syndrome with a cranial plasmocytoma with a noticeable clinical improvement after surgical excision of the cranial tumor.
Differentiating POEMS syndrome from other plasma cell disorders (for example multiple myeloma) is based on the specific combination of multi-systemic symptoms, lambda chain- restricted M protein and high serum VEGF levels . Although the reported case presented not only high levels of lambda chain in serum but also of kappa light chain in serum (Table 2), the high levels of VEGF as well as that established by the diagnosis criteria (Table 1) led to the final diagnosis of POEMS syndrome. The high specificity of VEGF should be mentioned when differentiating among POEMS syndrome, multiple myeloma, amyloidosis, monoclonal gammapathy and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). While this factor shows selectively increased serum levels in POEMS syndrome, the other entities usually present normal levels .
The skull is an uncommon location for such hematological lesions, and the frequency is even lower when only the cranial vault is considered. In 2000, Khouja et al. reviewed the reported cases of plasmocytomas with cranial vault location. At that time, 36 cases had been published  and in the last 12 years different authors have reported five more cases. Therefore, a total of 41 cases of cranial vault plasmocytoma have been reported most of which were located in the parietal and occipital bones; frontal localization seems to be less frequent. Not all cases had erosion of the underlying bone and most of them showed an improvement of the symptoms after surgical removal, which is generally followed by radiotherapy [8, 9].
The association of POEMS syndrome and cranial vault plasmocytoma is even more uncommon than skull plasmocytoma. To date, there are only two reported cases presenting with such a cranial lesion associated with POEMS syndrome [10, 11]. One of them also presented with an osteolytic lesion in the parietal-occipital area, which was treated with surgery and radiation therapy . The other reported case did not show bone erosion and the patient developed an immune complex vasculitis . In both cases, a clinical improvement was reported after surgical excision of the plasmocytoma. Surgery may be also recommended in cranial plasmocytomas when the bone defect is large enough to consider it necessary to reconstruct the cranial vault [12, 13].
However, neither Dispenzieri (2012) in a review about the management of POEMS syndrome nor Li and Zhou (2013) in another review about the new advances in the diagnosis and treatment of POEMS syndrome included surgery as a therapeutic element [7, 14]. Differences in the management of the plasmocytoma depending on its location were not specified by the above-mentioned authors and, as we have described, the cranial vault seems to be a special location where surgery might have a certain role to play.
Therefore, POEMS syndrome with a cranial vault plasmocytoma might have a better prognosis and clinical outcome if surgery is included in the management of the disease. Needless to say, some case reports do not provide a great deal of evidence about the role of surgery in this disease, but the low frequency of skull plasmocytomas with POEMS syndrome and the outcome of the reported cases suggest that surgery should be taken into consideration in the global management of the disease.
We report a new case of a POEMS syndrome associated with a cranial vault plasmocytoma. Adding surgery to the management of this disease might improve clinical outcome and prognosis. More case series are necessary to have a greater level of evidence about the proper management of these patients and, especially, the role played by surgery.
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
We acknowledge the support of all members of the neurosurgical staff in the Hospital Universitario de Canarias.
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