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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 J, Garcia-Marin V - J Med Case Rep (2013)

Bottom Line: POEMS syndrome (an acronym of polyneuropathy, organomegaly, endocrinopathy, multiple myeloma and skin changes) is a paraneoplastic disorder related to an underlying plasma cell dyscrasia.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.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.

View Article: PubMed Central - HTML - PubMed

Affiliation: Neuroscience Department, Hospital Universitario de Canarias, C/Ofra s/n, La Laguna, 38320, Santa Cruz de Tenerife, Spain. jplata5@hotmail.com.

ABSTRACT

Introduction: 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.

Case presentation: 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.

Conclusion: 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.

No MeSH data available.


Related in: MedlinePlus

Operative and pathological views. (a) and (b) show the circular craniectomy and the complete excision of the mass. Observe that the dura mater does not seem to be infiltrated or damaged; (c) hematoxilin and eosin preparation from the mass showing multiple plasmatic cell infiltration; (d) hematoxilin and eosin preparation from the lymph node biopsy with peripheral follicular proliferation and hyalinization of the vessels (black arrow) and the presence of plasma cells. These are the typical findings of Castleman’s disease.
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Figure 2: Operative and pathological views. (a) and (b) show the circular craniectomy and the complete excision of the mass. Observe that the dura mater does not seem to be infiltrated or damaged; (c) hematoxilin and eosin preparation from the mass showing multiple plasmatic cell infiltration; (d) hematoxilin and eosin preparation from the lymph node biopsy with peripheral follicular proliferation and hyalinization of the vessels (black arrow) and the presence of plasma cells. These are the typical findings of Castleman’s disease.

Mentions: His laboratory test results showed a significant increase of immunoglobulin G (IgG) with a clear peak in the proteinogram, increase of lambda and kappa light chains in serum and high levels of vascular endothelial growth factor (VEGF) (Table 2). No Bence Jones proteinuria was identified. A bone marrow biopsy showed reactive changes and intense plasmocytosis and pathological analysis of one of the cervical lymph nodes showed reactive changes with peripheral follicular proliferation, hyalinization of the vessels and the presence of plasma cells (Figure 2d). These findings were compatible with Castleman’s disease. Serological tests for human herpes virus-8 (HHV-8) and human immunodeficiency virus (HIV) infection were performed, with negative results, because of the association between Castleman’s disease and these viral infections [5,6]. Castleman’s disease occurs in about 11 to 30% of patients with POEMS syndrome [7].


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 J, Garcia-Marin V - J Med Case Rep (2013)

Operative and pathological views. (a) and (b) show the circular craniectomy and the complete excision of the mass. Observe that the dura mater does not seem to be infiltrated or damaged; (c) hematoxilin and eosin preparation from the mass showing multiple plasmatic cell infiltration; (d) hematoxilin and eosin preparation from the lymph node biopsy with peripheral follicular proliferation and hyalinization of the vessels (black arrow) and the presence of plasma cells. These are the typical findings of Castleman’s disease.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4016595&req=5

Figure 2: Operative and pathological views. (a) and (b) show the circular craniectomy and the complete excision of the mass. Observe that the dura mater does not seem to be infiltrated or damaged; (c) hematoxilin and eosin preparation from the mass showing multiple plasmatic cell infiltration; (d) hematoxilin and eosin preparation from the lymph node biopsy with peripheral follicular proliferation and hyalinization of the vessels (black arrow) and the presence of plasma cells. These are the typical findings of Castleman’s disease.
Mentions: His laboratory test results showed a significant increase of immunoglobulin G (IgG) with a clear peak in the proteinogram, increase of lambda and kappa light chains in serum and high levels of vascular endothelial growth factor (VEGF) (Table 2). No Bence Jones proteinuria was identified. A bone marrow biopsy showed reactive changes and intense plasmocytosis and pathological analysis of one of the cervical lymph nodes showed reactive changes with peripheral follicular proliferation, hyalinization of the vessels and the presence of plasma cells (Figure 2d). These findings were compatible with Castleman’s disease. Serological tests for human herpes virus-8 (HHV-8) and human immunodeficiency virus (HIV) infection were performed, with negative results, because of the association between Castleman’s disease and these viral infections [5,6]. Castleman’s disease occurs in about 11 to 30% of patients with POEMS syndrome [7].

Bottom Line: POEMS syndrome (an acronym of polyneuropathy, organomegaly, endocrinopathy, multiple myeloma and skin changes) is a paraneoplastic disorder related to an underlying plasma cell dyscrasia.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.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.

View Article: PubMed Central - HTML - PubMed

Affiliation: Neuroscience Department, Hospital Universitario de Canarias, C/Ofra s/n, La Laguna, 38320, Santa Cruz de Tenerife, Spain. jplata5@hotmail.com.

ABSTRACT

Introduction: 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.

Case presentation: 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.

Conclusion: 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.

No MeSH data available.


Related in: MedlinePlus