© Georg Thieme Verlag KG Stuttgart · New York
Pancreatic head mass of unusual etiology: multiple myeloma diagnosed by endoscopic ultrasound-guided fine needle aspiration
07 October 2010 (online)
Pancreatic involvement in multiple myeloma (MM) is a rare event and can occur as a primary isolated form or as secondary manifestation of systemic disease. The endoscopic ultrasound (EUS) features of this entity have not been described.
A 64 year-old woman presenting with anemia, renal insufficiency, and multiple lytic bone lesions was diagnosed as having kappa light chain multiple myeloma. She was prescribed doxorubicin, dexamethasone, and thalidomide, and showed a good response. She then underwent autologous stem cell transplantation (ASCT) and stayed in remission for 9 months. Later, an abdominal ultrasound was performed for abnormal liver enzymes, and was suspicious for a pancreatic head mass. This was confirmed by abdominal computed tomography, which revealed a 35-mm pancreatic head mass ([Fig. 1]).
Fig. 1 Abdominal computed tomographic (CT) scan showing a hypovascular, 35-mm pancreatic head mass.
There was no evidence of biliary or pancreatic duct dilatation. CA 19.9 levels were normal. She was then referred to our institution for further evaluation. EUS with a linear echoendoscope (Olympus Medical Systems Corp., Tokyo, Japan) showed a 4-cm heterogeneous, overall hypoechoic pancreatic head mass. This lesion was in close proximity with the superior mesenteric vein just below the confluence, although the vascular adventitia appeared to be preserved ([Fig. 2]).
Fig. 2 Endoscopic ultrasound showing a heterogeneous, hypoechoic pancreatic head mass in close proximity to the superior mesenteric vein (SMV).
The common bile duct was not dilated. EUS fine-needle aspiration (EUS-FNA) was carried out with a 22-gauge needle (Olympus Medical Systems Corp., Tokyo, Japan), with a total of three passes ([Fig. 3]).
Fig. 3 Endoscopic ultrasound guided fine-needle aspiration.
The consistency of the mass was noted to be medium hard. FNA smears and cellblock sections showed a homogeneous plasma cell population with occasional multinucleated cells and discrete nuclear atypia. The cells were strongly immunoreactive for CD38 and CD138 and negative for CD79a ([Fig. 4]).
Fig. 4 Cytological specimen. a Cellblock section (hematoxylin and eosin, magnification × 400). Uniform plasma cell population and discrete nuclear atypia. b Intense CD38 positive staining seen in virtually all cells. c Intense CD138 positive staining in 100 % of plasma cells.
Serum/urine electrophoresis and a myelogram did not show any evidence of relapse and the patience was referred for radiation therapy.
Extramedullary multiple myeloma (MM) involvement is not an uncommon presentation, occurring in 10 % – 15 % of patients. To date, fewer than 30 cases of extramedullary plasmacytomas involving the pancreas have been reported , with an estimated prevalence rate of 2.3 % based on autopsy studies . Although the head of the pancreas is usually involved, with most cases reported as large masses (> 4 cm) presenting with obstructive jaundice, other pancreatic locations and diffuse involvement have also been described. When carried out for biliary obstruction, endoscopic retrograde cholangiopancreatography (ERCP) usually demonstrates smooth intrapancreatic biliary stenosis . According to the literature, the patterns of multiple myeloma relapse after ASCT differ from the presenting clinical scenario and there are now a few reports of extramedullary relapses. To the best of our knowledge, there has only been one case report of pancreatic relapse in this setting .
EUS-FNA has a proven record for staging pancreatic cancer and for cytological evaluation. About 6 % of cytology results are “atypical”, that is, they do not have features of pancreatic adenocarcinoma . The cytological/histological diagnosis in the majority of cases reporting multiple myeloma of the pancreas have been based on surgical specimens/surgical biopsies, imaging-guided percutaneous biopsy, and postmortem evaluation. We could find only one report in which EUS had been carried out, although no description of the procedure or images were provided . In summary, pancreatic mass in the setting of multiple myeloma, especially after ASCT, should alert the clinician to the possibility of extramedullary disease.
Competing interests: None
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