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Patient 1 with a history of IgG kappa multiple myeloma presented with acute renal insufficiency. Non-enhanced CT-scan (panel A ) and MRI (panel B ; Té weighted) showed a retroperitoneal mass (arrow on CT scan and MRI) infiltrating the left proximal ureter and the renal pelvis. Following treatment, the mass regressed and only mild residual infiltration of the left proximal ureter (arrow) was noted on a control MRI performed 2 years later (panel C ). A biopsy of the mass revealed the presence of plasma cell proliferation (panel D , light microscopy, haematoxylin phloxine saffron staining, × 50) that stained positive for CD 138 (panel E ) and kappa light chain (panel F ).
Source publication
Extramedullary plasmacytomas (EMP) rarely occur during the course of multiple myeloma (MM). Most frequent reported sites are
superior respiratory airways, pleura, lung, lymph nodes, skin, subcutaneous and soft tissues, testicles and liver. EMP involving
the urinary tract are very uncommon and have been ill-described in the literature. We report two...
Contexts in source publication
Context 1
... showed dilatation of the left renal pelvis and proximal ureter. CT scan and abdominal MRI (Figure 1) revealed an 8 cm obstructive retroperitoneal tu- mour infiltrating the left ureter and renal pelvis and respon- sible for ureterohydronephrosis. A transparietal biopsy of the tumour was performed. ...
Context 2
... transparietal biopsy of the tumour was performed. Pathology examination showed a proliferation of large plasmablastic cells that stained pos- itive for CD138 and kappa light chains and negative for lambda light chains and CD20 (Figure 1). Diagnosis of ureteral EMP was made. ...
Citations
... In symptomatic MM, obstruction of the urinary tract due to expansion of bladder or ureteral extramedullary plasmocytoma has been rarely described. 23 Similarly, renal parenchymal plasma cell infiltration is uncommon and is exceptional as the sole cause of renal impairment. In a recent randomized controlled study, where 176 patients with MM and AKI due to LCCN underwent a kidney biopsy, malignant plasma cells invading the tubulointerstitial compartment were found in 4 (2.2%) and always concurrent with LCCN. ...
Symptomatic multiple myeloma (MM) is commonly complicated by acute kidney injury (AKI), through various mechanisms. The most frequent is the precipitation of monoclonal free light chains (FLC) with uromodulin in the distal tubules, defining light chain cast nephropathy (LCCN). Early diagnosis and identification of the cause of AKI are required for optimizing management and avoiding chronic kidney injury that strongly affects quality of life and patient survival. In LCCN, often manifesting with severe AKI, renal recovery requires urgent intervention based on vigorous rehydration, correction of precipitating factors and efficient anti-plasma cell chemotherapy to rapidly reduce the secretion of nephrotoxic FLC. Currently, the association of the proteasome inhibitor bortezomib with high-dose dexamethasone is the standard regimen in newly diagnosed patients. The addition of another drug such as cyclophosphamide or an immunodulatory agent may improve FLC response, but raises tolerance concerns in frail patients. Further studies are warranted to confirm the role of anti-CD38 monoclonal antibodies, which efficacy and tolerance have been documented in patients without renal impairment. Despite controversial results from randomized studies, recent data suggest that in patients with LCCN and AKI requiring dialysis, the combination of chemotherapy with FLC removal through high-cutoff hemodialysis, may increase renal response recovery rates. Kidney biopsy may be helpful for guiding management and assessing renal prognosis that appears to depend on the extent of cast formation and interstitial fibrosis/tubular atrophy. Due to continuous improvement in life expectancy of MM patients, renal transplantation is likely to be increasingly considered in selected candidates.
... Most frequent sites are superior respiratory airway, pleura, lung, lymphnode, skin, subcutaneous and soft tissues, testicles and liver (8) . Despite many recent progressions in diagnostic modalities, occasionally the initial manifestation of the disease may be misleading. ...
Extramedullary plasmacytoma (EMP) is an uncommon entity that most commonly involves nasopharynx and upper repository tract. Involvement of GIT occurs in approximate 10% of cases. According to WHO plasma cell tumors have been classified into two main groups: Multiple myeloma and plasmacytoma. Plasmacytoma includes solitary plasmacytoma of bone and solitary extramedullary plasmacytoma. EMP can be either primary without evidence of bone marrow involvement or may occur simultaneously with multiple myeloma representing extramedullary spread of the disease. It may occur in association with multiple myeloma and it may precede, accompany or follow the onset of multiple myeloma. Diagnosis of primary EMP requires the exclusion of associated multiple myeloma as shown by negative Bence Jones Proteins in urine, normal serum electrophoresis, normal bone marrow biopsy, normal skeletal survey and normal calcium levels. Here we present a case of 55-year male who came to Nephrology Department for urinary tract infection and pain abdomen. Patient was referred to Radiology for ultrasonography which revealed bilateral renal parenchymal disease with a well-defined mass in the mesentry which was further confirmed on computed tomography. Patient was surgically operated and diagnosis of primary EMP of mesentry was made on histopathological examination. Only three cases have been reported so far in the literature. Keywords: Extramedullary plasmacytoma, mesentry, plasma cell dyscrasias.
63-year-old man with past history of multiple myeloma recently started on a regimen of daratumumab, carfilzomib, and dexamethasone was referred to our emergency department for a rapidly rising serum creatinine as high as 10 mg/dL. He complained of fatigue, nausea, and poor appetite. Exam revealed hypertension, but no edema or rales. Labs were consistent with AKI without hypercalcemia or evidence of hemolysis or tumor lysis. Urinalysis and urine sediment were bland without proteinuria, hematuria, or pyuria. Initial concern was for hypovolemia or myeloma cast nephropathy. POCUS revealed no overt evidence of volume overload or depletion, instead revealing bilateral hydronephrosis. Bilateral percutaneous nephrostomies were placed with resolution of the AKI. Ultimately, referral imaging revealed interval progression of bulky retroperitoneal extramedullary plasmacytomas compressing the ureters bilaterally related to the underlying multiple myeloma.
Multiple myeloma (MM) is a monoclonal plasma cell neoplasia that commonly involves the kidney. Renal impairment is a serious complication during the course of the disease, and it is associated with increased morbidity and mortality. The most frequent mechanism of injury is represented by the precipitation of monoclonal free light chains (FLCs) in the distal tubule of nephron, defining a dramatic condition known as light chain cast nephropathy (LCCN). A prompt and early identification of the cause of renal disease, particularly in case of acute kidney injury (AKI), is mandatory for its effective management, avoiding the development of chronic kidney disease (CKD). In case of LCCN, in order to achieve renal recovery, it is needed, besides preventive measures, urgent intervention based on vigorous rehydration, correction of precipitating factors and effective anti-plasma cell chemotherapy. Currently, the association of the Proteasome Inhibitor Bortezomib with high-dose of Dexamethasone represents the standard association in newly diagnosed patients. The addition of another drug such as Cyclophosphamide or an Immunomodulatory Drugs may improve FLCs reduction but could be toxic. Interesting is the role of the newest therapeutic agents, particularly anti-CD38 Monoclonal Antibodies, whose efficacy and tolerance have been documented in patients without renal impairment. Despite controversial results from randomized studies, recent data suggest that in patients with LCCN and AKI requiring dialysis the association of systemic therapy with an extra-corporeal approach of FLCs removal, may increase renal response recovery rates. In this chapter, it is summarized physio-pathological basis of MM renal impairment, clinical manifestations, diagnostic procedures, and therapeutic management, included autologous stem cell transplantation.
From 2009-2017, we identified 9 cases of plasma cell neoplasms on biopsies of the bladder in patients without a history of plasma cell myeloma or transplantation (6 male, 3 female). Four of the nine showed amyloid deposition, of which one additionally revealed a clear cell adenocarcinoma of the bladder. Follow-up was obtained in 7 cases. Of 3 cases (including 2 with amyloid) for which electrophoresis and immunofixation results were obtained, the 2 amyloid cases showed evidence of serum or urine paraproteins: serum IgM kappa in a patient with kappa light chain-restricted plasma cell neoplasm and urine IgA lambda in a patient with lambda light-chain restricted plasma cell neoplasm. By contrast, 1 case with a kappa light-chain restricted plasma cell neoplasm in the absence of amyloid showed no serum monoclonal protein. Bone marrow biopsy results were obtained on the 2 amyloid cases revealing a population of 5 percent or less plasma cells with no assessment of clonality, and thus, were not diagnostic of plasma cell myeloma. In congruence, the 2 amyloid cases also showed no radiologic evidence of systemic plasma cell myeloma. One patient with plasma cell neoplasm only received chemotherapy and radiation without subsequent biopsies; one patient with plasma cell neoplasm, amyloid, and clear cell adenocarcinoma received radiation with an absence of neoplastic disease on subsequent biopsies. Additionally, no evidence of systemic amyloid was found in the cases with bladder amyloidosis. Plasma cell neoplasms of the bladder, with and without amyloid deposition, are rare; this is the first known case series. In 7 cases with follow-up, plasma cell myeloma did not appear to manifest in a 1-127 month follow-up. However, paraproteins were identified on further testing in 2 cases with amyloid. While bladder plasma cell neoplasms with and without amyloid tend to have a favorable prognosis in short-term follow-up, our study supports the need for additional workup for systemic disease, particularly in those with concurrent amyloidosis.
A 71-year-old female presented to our hospital with malaise. Laboratory tests revealed kidney dysfunction (creatinine: 7.49 mg/dL) and monoclonal proteinemia, and computed tomography showed bladder wall thickening and bilateral hydronephrosis. Bilateral ureteral catheters were inserted from the bladder, and hemodialysis was performed with a central venous catheter. Bone marrow aspiration was conducted, and the patient was diagnosed with multiple myeloma. Furthermore, cystoscopy revealed that the bladder wall thickening was due to a submucosal tumor, and a tissue biopsy demonstrated a bladder plasmacytoma. The patient was treated with BD therapy (bortezomib + dexamethasone), and her kidney function gradually improved. She was withdrawn from hemodialysis. It is extremely rare for multiple myeloma to be complicated with bilateral hydronephrosis due to an extramedullary bladder lesion.
About 3–5 % of patients with plasma cell dyscrasias present with either a single bone lesion, or less commonly, a soft tissue mass made up of monoclonal plasma cells without evidence of bone marrow involvement or end-organ damage known as a solitary plasmacytoma (SP). SP of bone mostly occurs in axial skeleton, while it most often affects the head and neck region in case of extramedullary tumors. Their median age is 55 years and mostly present with bony pain, spinal cord, or nerve root compression. A whole body (WB) or spine and pelvic magnetic resonance imaging (MRI) scan or WB fluorodeoxyglucose–positron emission tomography (FDG–PET) scan should be included in staging of these patients. The standard of care for SP is radiotherapy (RT) given with curative intent. Surgery may be required for patients with retropulsed bone, structural instability of the bone, or rapidly progressive neurological symptoms from spinal cord compression. The role of adjuvant RT after complete surgical resection, adjuvant chemotherapy, or adjuvant bisphosphonates is not well defined and hence not recommended.
