Mandal et al., J Clin Exp Oncol 2015, 4:3
http://dx.doi.org/10.4172/2324-9110.1000144
Journal of Clinical &
Experimental Oncology
Case Report
a SciTechnol journal
A Case of Multiple Myeloma
Presenting with Plasmacytoma
of Body of Sphenoid Bone: A
Case Report
Sanchayan Mandal1, Madhuchanda Kar2, Rakesh Roy2,
Tamohan chaudhuri1 and Shravasti Roy3
Abstract
Plasmacytoma (plasma cell neoplasm) presented as multiple
myeloma, solitary plasmacytoma of bone (SBP) and extramedullary
plasmacytoma (EMP). Types of plasmacytoma vary from one
another in terms of clinical findings, treatment options and survival.
Intracranial involvement of multiple myeloma is extremely rare
event and rarely the first presentation of Multiple myeloma. A case
of 58 years old patient reported with an unusual clinical presentation
and behaviour of multiple myeloma where intracranial lesion arising
from body of sphenoid. Initial biopsy proved plasmacytoma and
later confirmed the case as multiple myeloma. Patient received
palliative radiation and planned to receive chemotherapy. Hence,
in any skull based lesion, multiple myeloma should be kept in the
differential diagnosis.
Keywords
Plasmacytoma; Intracranial; Body of sphenoid ; Multiple myeloma
Introduction
Plasmacytoma refers to a tumor consisting of abnormal plasma
cells that grows within the soft tissue (extramedullary plasmacytoma)
or bony skeleton (solitary bone plasmacytoma). It can be present as
a part of a systemic disorder - called multiple myeloma (with bone
marrow involvement along with monoclonal gammopathy and
skeletal lesions). Solitary bone plasmacytoma (SBP) affects fewer
than 5% of patients with plasma cell disorders [1,2] and develops
into multiple myeloma in 50-60% of patients [3]. Extramedullary
plasmacytoma (EMP) represents approximately 3% of all plasma cell
neoplasm and progresses to multiple myeloma in 11-30% of patients
at 10 years. The thoracic vertebrae are most commonly involved,
followed by lumbar, sacral, and cervical vertebrae. Rib, sternum,
clavicle, or scapula involved in 20% of cases [4]. Median over all
survival time of patients with solitary bone plasmacytoma is 10 years
[5], compared to multiple myeloma ranging from 29 to 62 months
[6]. We report an unusual presentation of plasmacytoma involving
body of sphenoid bone which eventually turned out to be a case of
multiple myeloma. Studies showed involvement of orbit, grater
wing of sphenoid, frontal bones, mandible but body of sphenoid
*Corresponding author:Sanchayan Mandal MBBS, DNB Radiotherapy
(Resident), Saroj Gupta Cancer Centre and Research Institute (SGCCRI),
Department of Radiotherapy, Kolkata, 700104, India, Tel: 09804345392; E-mail:
dr.sanchayan2012@gmail.com
Received: August 08, 2015 Accepted: October 15, 2015 Published: October
22, 2015
International Publisher of Science,
Technology and Medicine
involvement is typically rare. Our case is unique in terms of not
only location (Body of sphenoid bone) but also presentation despite
intracranial involvement.
Case Report
A 58 years old diabetic, non-smoker male presented at our hospital
with dull headache, gradual loss of hearing, generalized weakness,
and body pain for last one month duration. There was no associated
history of vomiting, loss of vision, convulsion or sleep disturbance.
There was also no prior history of trauma, migraine or cerebro vascular
accidents (CVA). On clinical examination, general survey revealed
nothing significant. There was no abnormality noted on central and
peripheral nervous system examination. Fundus exam normal, There
was no evidence of bony pain. MRI (Magnetic Resonance Imaging) of
Brain done, showed a space occupying lesion (4.6 cm × 6.3 cm × 2.9
cm) involving clivus and body of sphenoid extending to both petrous
apices with replacement of sphenoid sinus and encroachment into
left posterior ethmoidal cells. Lesion also encasing cavernous sinus,
both internal and external carotid arteries (Figure 1). A differential
diagnosis included Chordoma, Plasmacytoma, Chondrosarcoma,
Ewing’s tumour, Osteosarcoma, Undifferentiated sarcoma,
Meningioma, Paranasal sinus tumor extension, Primary CNS
lymphoma, Lymphoma &Metastasis. Pituitary tumor was ruled out
due to absence of vision symptoms (bitemporal hemianopsia) or any
hormonal dysbalance or any cranial neuropathy. On hematological
investigations showed very high ESR, reversed Albumin – Globulin
ratio found with normal LDH. This made us to investigate for
systemic disease. He had undergone Biopsy of skull based lesion
revealed high suspicion of plasmacytoma (Figure 2). Bone marrow
aspiration study from sternum showed presence of 15-18% of plasma
Figure 1: MRI BRAIN showing A space occupying lesion (4.6 cm × 6.3 cm
× 2.9 cm) involving clivus and body of sphenoid extending to both petrous.
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Citation: Mandal S, Kar M, Roy R, Chaudhuri T, Roy S (2015) A Case of Multiple Myeloma Presenting with Plasmacytoma of Body of Sphenoid Bone: A Case
Report J Clin Exp Oncol 4:3.
doi:http://dx.doi.org/10.4172/2324-9110.1000144
cells, plasma blast cells. Then he was advised to undergo a series of
investigations like skeletal survey, Immunohistochemistry, Serum
protein electrophoresis, immunofixation electrophoresis. Skeletal
survey showed extensive punched out lesions on skull bones
(Figure 3) and evidence of less pattern of upper femur but no bony
erosion on X-ray of pelvis. Ultrasounds of abdomen, chest x-ray were
within normal limit. Immunohistochemistry (IHC) done from the
skull based lesion, were positive to MUM 1(Figure 4) and CD 138
(Figure 5). Serum electrophoresis study showed presence of M band
in gamma region. On immunofixation electrophoresis study proved
presence of monoclonal gammopathy in IgG and Kappa region.
Bence Jones protein detected on urine examination. Hence, diagnosis
made as multiple myeloma with intracranial plasmacytoma involving
body of sphenoid bone. Patient was initially treated with radiation
therapy to skull (30 Gray in 10 fractions; 300 cGY per fraction).
Though the patient was young and fit; he refused induction high dose
therapy followed by autologous stem cell transplantation (ASCT).
So, he is put on Bortezomib, Immunomodulator (Thalidomide) and
Steroid (VTD regimen).
Figure 4: Immunohistochemistry study of skull based lesions showing cells
positive to MUM 1.
Discussion
Plasmacytoma (plasma cell neoplasm) presented as multiple
myeloma, solitary plasmacytoma of bone (SBP) and extramedullary
plasmacytoma (EMP). They represent distinct manifestations of
a disease continuum, whereby the clinical findings are critical to
Figure 5: Immunohistochemistry study of based bone lesions showing cells
positive to CD 138.
Figure 2: Histopathology study of skull based lesion showing presence of
plasma cells.
Figure 3: X-ray of skull showed extensive punched out lesions on skull
bones.
Volume 4 • Issue 3 • 1000144
diagnosis. These types of plasmacytoma varies one from the other
has significant implications for treatment and survival [5,6]. There
are studies showed involvements of orbit, greater wing of sphenoid
bone, frontal bones, skull base, sphenoid sinus [7-9]. Plasmacytoma
may also present as non-functional pituitary macro adenoma [10], or
mandibular involvement [11], although the intracranial involvement
in case of multiple myeloma is rare phenomena. Most of the cases
cranial neuropathy was common due to intracranial involvement
.The authors unable to find cases with specifically involvement of
body of sphenoid bone in a case of multiple myeloma presented with
intracranial involvement at the initial presentation but without any
cranial neuropathy or any typical signs and symptoms of intracranial
involvement. Multiple myeloma is the most common primary
malignancy of skeletal tissue. It is the clinical syndrome of plasma
cell myeloma [12] that is the neoplastic proliferation of plasma cells
in the bone marrow, associated with production of monoclonal
immunoglobulin in high levels enough to reach and be detectable in
the serum or/and urine [12-14]. The diagnosis of multiple myeloma
requires cytological, radiological and laboratory criteria. A differential
diagnosis from imaging of sphenoidal lesion included Chordoma,
Plasmacytoma, Chondrosarcoma, Ewing’s tumour, Osteosarcoma,
Undifferentiated sarcoma, Meningioma, Paranasal sinus tumor
extension, Primary CNS lymphoma, Lymphoma &Metastasis. A skull
based biopsy proved to be plasmacytoma. Generally, intracranial
involvement presented with significant complains like lethargy,
• Page 2 of 3 •
Citation: Mandal S, Kar M, Roy R, Chaudhuri T, Roy S (2015) A Case of Multiple Myeloma Presenting with Plasmacytoma of Body of Sphenoid Bone: A Case
Report J Clin Exp Oncol 4:3.
doi:http://dx.doi.org/10.4172/2324-9110.1000144
nausea, vomiting, headaches, confusion, paresthesias, and seizures
as well as visual, gait, and speech disturbances but surprisingly this
patient referred only with vague headache, weakness and decreased
hearing. Although it was present by the side of vital cranial structures
there was no associated cranial nerve palsy. Initially it was thought
to be a case of solitary plasmacytoma of bone but later more
investigations proved a case of multiple myeloma. After discussing
treatment options with patient relatives and the patients ,tumor
board of our hospital decided to give radiotherapy to skull (30 grey
in 10 fractions, 300 cGY per fraction ) and patient is about to receive
systemic chemotherapy with Bortezomib (1.3 mg/m2 on days 1, 4, 8,
and 11 at 3-week intervals), Thalidomide 200 mg daily (escalating
doses in the first cycle: 50 mg on days 1 to 14, and 100 mg on days
15 to 28), and Dexamethasone 40 mg orally on days 1-4 and 9-12
at 4-week intervals for 6 cycles [15]. Radiation therapy (RT) is the
standard treatment for Solitary plasmacytoma. For patients treated
with surgical excision, RT is still indicated to eradicate microscopic
residual disease. Surgery alone without RT leads to an unacceptably
high local recurrence rate [16]. In treatment of multiple myeloma,
a superior response rate when the combination of bortezomib,
thalidomide, and dexamethasone (VTD regimen) and it was
compared with thalidomide plus dexamethasone in a large phase III
study: 93% in the bortezomib-thalidomide-dexamethasone (VTD)
arm versus 80% in the thalidomide-dexamethasone (TD) arm, in
which patients went on to receive tandem autologous stem cell
transplantation [17]. Laura Rosinol et al. showed the progressive
disease (PD) rate during induction was significantly lower with VTD
than with TD (7% vs 23%, P = 0.0004). In patients with extramedullary
soft-tissue plasmacytoma, the CR rate was significantly higher with
VTD compared with TD (42% vs 14%, P = 0.02) and the PD rate
was significantly lower with VTD (12% vs 40%, P = 0.02. Also, PFS
(Progression Free Survival) and OS (Overall Survival) better in VTD
groups. Bortezomib and Linalidomide also showed improved result
in maintenance therapy in multiple myeloma [18,19].
report and review of the literature. Surg Neurol 37: 388-393.
9. Jagadeesan J, Oudit D, Hardwicke J, Shariff Z, McCoubrey G, et al. (2006)
Solitary plasmocytoma of frontal bone presenting as an asymptomatic
forehead lump. Dermatol Online J. 12: 24.
10.Sinnott BP, Hatipoglu B, Sarne DH (2006) Intrasellar plasmacytoma
presenting as a non-functional invasive pituitary macro-adenoma: case report
& literature review. Pituitary 9: 65-72.
11.Elias HG, Scott J, Metheny L, Quereshy FA (2009) Multiple myeloma
presenting as mandibular ill-defined radiolucent lesion with numb chin
syndrome: a case report. J Oral Maxillofac Surg 67: 1991-1996.
12.Rosai J (2011) Plasma Cell Dyscrasias. In: Juan Rosai. Rosai and Ackerman’s Surgical Pathology, 10th ed, ch 23. Elsevier Inc
13.Pingali SR, Haddad RY, Saad A (2012) Current Concepts of Clinical
Management of Multiple Myeloma. Disease-a-Month 58: 195-207.
14.Campo E, Swedlow SH, Harris NL, Pileri S, Stein H, et al. (2011) The 2008
WHO classification of lymphoid neoplasms and beyond: evolving concepts
and practical applications. Blood 117: 5019-5032.
15.http://www.bloodjournal.org/content/120/8/1589?sso-checked=true
16.Ozsahin M, Tsang RW, Poortmans P, Belkacemi Y, Bolla M, et al. (2006)
Outcomes and patterns of failure in solitary plasmacytoma: A multicenter
Rare Cancer Network study of 258 patients. Int J Radiat Oncol Biol Phys
64: 210-217.
17.Cavo M, Patriarca F, Tacchetti P, Galli M, Perrone G, et al. (2007)
Bortezomib (Velcade[R])-thalidomide-dexamethasone (VTD) vs thalidomidedexamethasone (TD) in preparation for autologous stem-cell (SC)
transplantation (ASCT) in newly diagnosed multiple myeloma (MM) [abstract
73]. Blood 110:130a
18.Yang B, Yu RL, Chi XH, Lu XC (2013) Lenalidomide treatment for multiple
myeloma: systematic review and meta-analysis of randomized controlled
trials. PLoS One 8: e64354.
19.Sonneveld P, Schmidt-Wolf IG, van der Holt B, El Jarari L, Bertsch U, et
al. (2012) Bortezomib induction and maintenance treatment in patients with
newly diagnosed multiple myeloma: results of the randomized phase III
HOVON-65/ GMMG-HD4 trial. J Clin Oncol 30: 2946-2955.
Conclusion
Intracranial bony lesions even without systemic symptoms
should be suspected as a differential diagnosis of multiple myeloma
and investigation should proceed accordingly.
Acknowledgement
We are thankful to the department of pathology for providing us enormous
support.
References
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7. Nofsinger YC, Mirza N, Rowan PT, Lanza D, Weinstein G (1997) Head and
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plasmacytoma of the sphenoid sinus involving the pituitary fossa: a case
Volume 4 • Issue 3 • 1000144
Author Affiliations
Top
Department of Radiotherapy, Saroj Gupta Cancer Centre and Research
Institute (SGCRI), Kolkata
2
Department of Medical Oncology, Saroj Gupta Cancer Centre and Research
Institute (SGCRI), Kolkata
3
Department of Pathology, Saroj Gupta Cancer Centre and Research Institute
(SGCRI), Kolkata
1
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