Pancreatic Involvement by Plasma Cell Neoplasms

Abstract

Introduction Pancreatic involvement by plasma cell neoplasms is an extremely rare event, with only 50 cases described in the literature. They can present as a primary solitary extramedullary plasmacytoma or plasmacytoma secondary to a plasma cell myeloma. Clinical manifestations are due to the presence of a pancreatic mass usually in the pancreas head, which causes extra-biliary obstruction and abdominal pain. Methods Abdominal imaging including CT scan or endoscopic ultrasound with fine-needle aspiration tissue sampling is essential for the initial diagnostic procedure. However, immunohistochemical analysis of the biopsy specimen or flow cytometry of the aspirated material is crucial to prove the monoclonality and the final diagnosis of a plasma cell neoplasm. Discussion Management of these situations include radiotherapy, chemotherapy, surgery or combined therapy. Novel medications including the immunomodulatory drugs or the proteasome inhibitors followed by consolidation with intensive chemotherapy and haematopoietic stem cell transplantation are nowadays used as upfront treatment in the cases associated to a plasma cell myeloma. Conclusion Despite the rarity, plasma cell neoplasms should be considered in the differential diagnosis of obstructive jaundice and pancreatic neoplasms since they are potentially treatable situations.

Nonepithelial Neoplasms of the Pancreas, Part 2: Malignant Tumors and Tumors of Uncertain Malignant PotentialFrom the Radiologic Pathology Archives

“…Approximately 3%-5% of plasma cell neoplasms manifest as extraosseous (extramedullary) plasmacytomas, which most often occur in the upper respiratory tract. Rarely, plasma cell neoplasms can involve the pancreas, occurring as an extraosseous manifestation of plasma cell myeloma or a solitary extramedullary plasmacytoma (EMP) (72,74).…”

“…Clinical Features.-EMP accounts for less than 0.1% of all pancreatic masses and, like PPL, is more commonly seen in middle-aged men (median age, 55 years). However, plasma cell neoplasms are generally twice as common in African Americans (72,74). Similar to PPL, the pancreatic head is the most common EMP location reported; however, unlike PPL and similar to PDAC, obstructive jaundice is the most common symptom at presentation (74).…”

“…EMP is highly sensitive to radiation; therefore, the treatment of choice is local radiation therapy, which produces local control rates of 90%-97%. Approximately 70% of patients thus remain disease free at 10 years (72,74), and regional recurrence is seen in up to 25% of patients.…”

“…At CT, EMPs are well-defined, lobulated, homogeneous soft-tissue masses, which are usually hypoattenuating compared to normal pancreatic tissue (Fig 15b-15d) (77,78). Diffuse infiltration of the pancreas has been described in only a few cases (Fig 16) (74).…”

Extramedullary Plasmacytoma of the Pancreas: A Rare Entity

“…Plasma cell tumors tend to be very radiosensitive and chemosensitive, so the treatment of choice for extramedullary plasmacytomas is generally some combination of surgery, chemotherapy and radiation therapy [9, 10]. ERCP with stenting of the biliary system is often performed for symptomatic relief of obstructive jaundice.…”