- Email: [email protected]
Soft tissue plasmacytomas in multiple myeloma
Clinical Picture
Soft tissue plasmacytomas in multiple myeloma David P Breen*, Ciara L Freeman*, Rajith N De Silva, Shahram Derakhshani, Jane Stevens
A 50-year-old man was transferred to our hospital with a 3-week history of back pain and a mass on his skull that had been growing slowly for the past 7 months. The mass was smooth, firm, and non-tender. Despite his long hair, it gave him the appearance of having a high domed forehead. Examination was otherwise un remarkable. Laboratory investigations showed renal impairment, hypercalcaemia, anaemia, and immuno paresis. Lambda light chains were detected in the urine and the serum. Magnetic resonance scans of the brain (figure) and spine (appendix) showed soft tissue lesions over the skull and thoracic spine. A biopsy sample of the thoracic lesion showed a monomorphic population of plasma cells that were strongly positive for CD138 and lambda light chain restricted, consistent with a plasma cytoma. Examination of a bone marrow aspirate showed a low level infiltration of plasma cells. He was unable to tolerate bone marrow biopsy. We made a diagnosis of multiple myeloma with extramedullary plasmacytoma and started treatment with high-dose dexamethasone, with good initial response (appendix). He had radiotherapy to the symptomatic mass from T7 to T9 (20 Gy over five fractions) and started cyclophosphamide with thalidomide and dexamethasone (the approved induction therapy for patients eligible for stem cell transplantation in the UK at the time), but was poorly tolerant of the steroid component and required multiple dose reductions. After four cycles the skull lesions began to grow again and we changed his treatment to twice-weekly bortezomib and dexa methasone. Unfortunately, during the second cycle of this A
regimen, he was admitted to hospital with pneumonia, thrombocytopenia, epistaxis, and renal failure. Despite aggressive management, he died in the high dependency unit from his acute illness, about 6 months after his initial haematological diagnosis. Multiple myeloma is a mature B-cell neoplasm with an age-adjusted incidence of 4·7 per 100 000 per year in the UK. It is defined by the presence of more than 10% clonal plasma cells in the bone marrow (or biopsy-proven extramedullary plasmacytoma), together with end-organ damage attributable to the plasma cell disorder. 7–20% of patients have clinical or radiological evidence of extra medullary plasmacytoma at the time of diagnosis. Most tumours occur as a result of infiltration from the under lying bone marrow (with the axial skeleton and chest wall being the most commonly affected sites), but haematogenous spread can occur. Factors that influence prolif eration of plasma cells beyond the bone marrow microenvironment are poorly understood, although mech anisms such as acquisition of some cytogenetic character istics (eg, deletion at 17p13) have been proposed. The presence of extramedullary plasmacytoma at diagnosis is associated with reduced survival, although aggressive thera peutic approaches, such as high-dose therapy, bortezomib, or autologous stem cell transplantation, can help improve prognosis. Contributors CLF and JS were the haematologists who managed the case, SD interpreted the radiology, and DPB and RNDS provided neurology input to the patient’s care. All authors contributed to writing of the report. Written consent to publication was obtained from the family.
Lancet 2017; 390: 2083 Published Online August 22, 2017 http://dx.doi.org/10.1016/ S0140-6736(17)31471-X *Joint first authors Morton and Gloria Shulman Movement Disorders Centre and Edmond J Safra Program in Parkinson’s Disease, Toronto Western Hospital, Toronto, ON, Canada (D P Breen PhD); Department of Medical Oncology, British Columbia Cancer Agency, Vancouver, BC, Canada (C L Freeman MSc); and Department of Neurology (R N De Silva FRCP), Department of Neuroradiology (S Derakhshani MD), and Department of Haematology (J Stevens FRCP), Queen’s Hospital, Romford, UK Correspondence to: Dr David P Breen, Morton and Gloria Shulman Movement Disorders Centre and Edmond J Safra Program in Parkinson’s Disease, Toronto Western Hospital, Toronto, ON M5T 2S8, Canada [email protected] See Online for appendix
B
Figure: Large soft tissue plasmacytoma of the skull (A) Post-gadolinium coronal T1-weighted magnetic resonance (MR) scan of the brain showing a vividly enhancing soft tissue mass arising from the skull. (B) MR venogram showing the extent of the tumour vascularity.
www.thelancet.com Vol 390 November 4, 2017
2083
Soft tissue plasmacytomas in multiple myeloma David P Breen*, Ciara L Freeman*, Rajith N De Silva, Shahram Derakhshani, Jane Stevens
A 50-year-old man was transferred to our hospital with a 3-week history of back pain and a mass on his skull that had been growing slowly for the past 7 months. The mass was smooth, firm, and non-tender. Despite his long hair, it gave him the appearance of having a high domed forehead. Examination was otherwise un remarkable. Laboratory investigations showed renal impairment, hypercalcaemia, anaemia, and immuno paresis. Lambda light chains were detected in the urine and the serum. Magnetic resonance scans of the brain (figure) and spine (appendix) showed soft tissue lesions over the skull and thoracic spine. A biopsy sample of the thoracic lesion showed a monomorphic population of plasma cells that were strongly positive for CD138 and lambda light chain restricted, consistent with a plasma cytoma. Examination of a bone marrow aspirate showed a low level infiltration of plasma cells. He was unable to tolerate bone marrow biopsy. We made a diagnosis of multiple myeloma with extramedullary plasmacytoma and started treatment with high-dose dexamethasone, with good initial response (appendix). He had radiotherapy to the symptomatic mass from T7 to T9 (20 Gy over five fractions) and started cyclophosphamide with thalidomide and dexamethasone (the approved induction therapy for patients eligible for stem cell transplantation in the UK at the time), but was poorly tolerant of the steroid component and required multiple dose reductions. After four cycles the skull lesions began to grow again and we changed his treatment to twice-weekly bortezomib and dexa methasone. Unfortunately, during the second cycle of this A
regimen, he was admitted to hospital with pneumonia, thrombocytopenia, epistaxis, and renal failure. Despite aggressive management, he died in the high dependency unit from his acute illness, about 6 months after his initial haematological diagnosis. Multiple myeloma is a mature B-cell neoplasm with an age-adjusted incidence of 4·7 per 100 000 per year in the UK. It is defined by the presence of more than 10% clonal plasma cells in the bone marrow (or biopsy-proven extramedullary plasmacytoma), together with end-organ damage attributable to the plasma cell disorder. 7–20% of patients have clinical or radiological evidence of extra medullary plasmacytoma at the time of diagnosis. Most tumours occur as a result of infiltration from the under lying bone marrow (with the axial skeleton and chest wall being the most commonly affected sites), but haematogenous spread can occur. Factors that influence prolif eration of plasma cells beyond the bone marrow microenvironment are poorly understood, although mech anisms such as acquisition of some cytogenetic character istics (eg, deletion at 17p13) have been proposed. The presence of extramedullary plasmacytoma at diagnosis is associated with reduced survival, although aggressive thera peutic approaches, such as high-dose therapy, bortezomib, or autologous stem cell transplantation, can help improve prognosis. Contributors CLF and JS were the haematologists who managed the case, SD interpreted the radiology, and DPB and RNDS provided neurology input to the patient’s care. All authors contributed to writing of the report. Written consent to publication was obtained from the family.
Lancet 2017; 390: 2083 Published Online August 22, 2017 http://dx.doi.org/10.1016/ S0140-6736(17)31471-X *Joint first authors Morton and Gloria Shulman Movement Disorders Centre and Edmond J Safra Program in Parkinson’s Disease, Toronto Western Hospital, Toronto, ON, Canada (D P Breen PhD); Department of Medical Oncology, British Columbia Cancer Agency, Vancouver, BC, Canada (C L Freeman MSc); and Department of Neurology (R N De Silva FRCP), Department of Neuroradiology (S Derakhshani MD), and Department of Haematology (J Stevens FRCP), Queen’s Hospital, Romford, UK Correspondence to: Dr David P Breen, Morton and Gloria Shulman Movement Disorders Centre and Edmond J Safra Program in Parkinson’s Disease, Toronto Western Hospital, Toronto, ON M5T 2S8, Canada [email protected] See Online for appendix
B
Figure: Large soft tissue plasmacytoma of the skull (A) Post-gadolinium coronal T1-weighted magnetic resonance (MR) scan of the brain showing a vividly enhancing soft tissue mass arising from the skull. (B) MR venogram showing the extent of the tumour vascularity.
www.thelancet.com Vol 390 November 4, 2017
2083