Figure 1. 4.4
x 3.5 cm mass in the tail of the pancreas.
Cancer
Therapy Vol 5, 515-518, 2007
Recurrent
multiple myeloma mimicking metastatic pancreatic carcinoma
Alejandro
Calvo1,*, Mauricio Carballo2, Satheesh Kathula1,Veronica
Camacho2
1Department of Hematology and Medical Oncology,
Kettering Medical Center Network, Dayton, OH.
2UMSS School of Medicine, Division of Internal
Medicine, Cochabamba, Bolivia.
__________________________________________________________________________________
*Correspondence: Alejandro R. Calvo, M.D., Kettering Medical
Center, 3535 Southern Blvd., Kettering,
OH 45409, USA; e-mail: Alejandro.Calvo@kmcnetwork.org
Key words: Multiple Myeloma, Extramedullary plasmacytoma,, Pancreatic
carcinoma, Liver metastasis
Abbreviations: Gastrointestinal, (GI); Multiple Myeloma, (MM)
Summary
We describe a patient with a history of
recurrent multiple myeloma with several relapses despite various treatments,
who presented with a new onset of anterior abdominal pain and an epigastric
mass. Computed tomography scan of the abdomen showed a 4.4 x 3.5 cm mass in the
tail of the pancreas and a 5.3 x 6.3 cm low-density mass in the right lobe of
the liver consistent with metastatic pancreatic carcinoma. A CT-guided biopsy
of the liver and pancreatic lesions however revealed plasmacytoma. The vast
majority of extramedullary plasmacytomas present as part of multiple myeloma,
they are rare occurring in less than 5% of patients with plasma cell dyscrasia.
Plasmacytoma involving the gastrointestinal tract is rare at presentation but
has been reported in advanced cases. Most of these lesions mimic carcinoma or
lymphoma and pose difficult diagnostic challenges. When a pancreatic mass is
noticed in patients with a history of myeloma, secondary extramedullary
plasmacytoma of the pancreas should be considered in the differential
diagnosis.
A plasmacytoma is a mass composed of monoclonal
neoplastic plasma cells. It can exist isolated as a solitary plasmacytoma or
associated to multiple myeloma.
These soft tissue tumors can be associated to
bone or be confined to the aerodigestive tract, known as extramedullary
plasmacytomas. They can occasionally cause compression, invasion and/or
destruction of adjacent organs resulting in airway or gastrointestinal
obstruction, vertebral compression fractures, and nerve root or cord
compression.
An elevated index
of suspicion must be maintained in myeloma patients developing soft tissue
masses as they can lead to diagnostic confusion and treatment delays. The
treatment of these lesions is usually successful with either chemotherapy or
radiotherapy.
II. Case report
A 64-yr-old female presented
with an extramedullary plasmacytoma at L4 level. A few months later she
progressed to multiple myeloma. Despite aggressive chemotherapy she developed
paraplegia secondary to cord compression and chronic pain secondary to
recurrent episodes of pathologic fractures of the humeri and femurs.
Her treatment consisted
initially of radiation to L4, and later she received multiple lines of
treatment including thalidomide/dexamethasone, liposomal doxorubicin
/vincristine/dexamethasone, and bortezomib. She received several courses of
palliative radiation therapy to different segments of her skeleton, including
her back, femurs, humeri, and skull, for recurrent bone events. Her treatment
at the time of this presentation consisted of lenalidomide and dexamethasone.
She complained of anterior
abdominal pain and her physical exam revealed a palpable epigastric mass. There
was no history of nausea, vomiting, weight loss or changes in her bowel habits.
A CT scan of the abdomen with contrast revealed the presence of a 4.4 x 3.5 cm
mass in the tail of the pancreas (Figure
1) and a 5.3 x 6.3 cm low-density mass in the right lobe of the liver
consistent with metastatic pancreatic carcinoma (Figure 2) CT guided fine needle aspiration of the right liver lobe
and pancreatic tail mass showed extramedullary plasmacytoma (Figure 3).
Due to a very poor performance status
and refractory nature of myeloma she was deemed a poor candidate for external
beam radiation therapy or chemotherapy and referred to hospice were she expired
several days later.
Figure 1. 4.4
x 3.5 cm mass in the tail of the pancreas.
Figure 2. 5.3
x 6.3 cm low-density mass in the right lobe of the liver.
Figure 3. Liver biopsy showed poorly
differentiated plasma cells. Pancreatic biopsy was similar.
III. Discussion
Multiple Myeloma (MM) belongs to the family of monoclonal,
inmunoproliferative plasma-cell neoplasms (Alexiou et al, 1999). Plasmacytomas share many of
the biologic features of other plasma cell disorders. Myeloma cells may spread and form masses in the soft tissues and are
referred to as extramedullary plasmacytomas. However, the majority of these
lesions are actually paraskeletal bone marrow tumors, with only one third being
true distal metastasis (Griffiths et al, 1997). Liver, spleen, and lymph nodes
are most commonly involved at advanced stages of MM (Hayes et al, 1952). Gastrointestinal (GI)
involvement at the time of initial diagnosis of MM is much more rare than GI
involvement later in the course of the disease, and it often develops in
patients with relapsing disease after stem cell transplantation. In a recently reported series, almost half of the patients with GI
involvement by extramedullary plasmacytomas were initially asymptomatic; their
lesions were found incidentally by imaging studies or at autopsy. In this
series 25% of patients had pancreatic
lesions (Talamo et al, 2006). In a different study looking at autopsy findings
in all myeloma patients, pancreatic plasmacytomas were found in only 4 % of the
cases (Hayes et al, 1952).
Computed tomography is the best method for evaluating
pancreatic masses. On CT images, extramedullary plasmacytoma of the pancreas is
usually described as a hypodense multilobular homogeneous solid mass. It is
very difficult to differentiate plasmacytomas from other tumors of the pancreas
by radiographic criteria (Mitchell and Hill, 1985; Fukuya et al, 1989; Wilson
et al, 1989). Most of these lesions mimic carcinoma or lymphoma and pose
difficult diagnostic challenges.
Plasmacytomas are very sensitive to radiation therapy. Therefore the
treatment of choice for patients with localized extramedullary plasmacytoma of
the pancreas is radiotherapy, occasionally distal pancreatectomy may be
indicated for a solitary extramedullary plasmacytoma localized in the tail of
the pancreas. In multiple myeloma with pancreatic metastasis multi-agent
chemotherapy would be appropriate (Wiltshaw, 1976; Whittaker, 1986).
When a pancreatic mass is noticed in patients with a
history of myeloma, secondary extramedullary plasmacytoma of the pancreas
should be considered as a differential diagnosis. The prognosis of some these
patients may be significantly better than patients with true adenocarcinoma of
the pancreas, hence it is imperative to make a correct diagnosis and give
appropriate treatment.
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