Solitary Extramedullary Plasmacytoma Research Articles

Imaging features of solitary extramedullary plasmacytoma of liver

Objective To summarize the imaging features of ultrasound, computed tomography (CT)and gadolinium-ethoxybenzyl-diethylenetriamine pentoacetic acid (Gd-EOB-DTPA)enhanced magnetic resonance imaging (MRI)of solitary extramedullary plasmacytoma (SEP)of liver, and investigate the key points of identification and diagnosis. Methods The clinical data of 1 patient with SEP of the liver who was admitted to the Southwest Hospital of the Third Military Medical University at 7 May, 2015 were retrospectively analyzed.The patient received contrast-enhanced ultrasound (CEUS), plain and enhanced scan of CT and Gd-EOB-DTPA enhanced MRI.The patient underwent treatment after preoperative examinations.Pathological examination and immunohistochemical staining were done after operation.The patient was followed up by outpatient examination of color Doppler ultrasonography till 12 November, 2015.The location, size, shape, echo, density or signal, enhancement pattern, secondary performance were recorded by imageological examinations.Surgical treatment, results of pathological examination, immunohistochemical staining, postoperative recovery and recurrence of tumor were recorded. Results CEUS examination demonstrated a hypoechoic hepatic lesion at S7 segment of the right liver measuring 24 mm × 19 mm with clear boundary and dotted blood flow signal in the mass.In the arterial phase, the lesion was enhanced rapidly.Abdominal CT scan showed that the mass at the right liver lobe had slightly low density with clear boundary, the CT value of 34-64 HU, and liver capsule having no significant outer convex.On enhanced CT, the lesion presented a homogeneous enhancement and shape of posterior upper tributaries of right hepatic artery in the arterial phase, the lesion presented continuous enhancement which was slightly lower than that of liver parenchyma in the portal venous phase with the CT value of 77 -102 HU, the lesion presented decreased enhancement with the CT value of 41-98 HU in the equilibrium phase.The maximum density projected image showed that the shape of an enlarged vascular image was found inside the lesion in the arterial phase and the lesion was adjacent to inferior vena cava and right hepatic vein which was compressed and displaced in the portal venous phase.On Gd-EOB-DTPA enhanced MRI, the right liver lobe showed a homogeneous T1-weighted and T2weighted signal with clear boundary and without lipid component, hemorrhage or calcification.The lesion presented obvious enhancement in the arterial phase, homogeneous continuous enhancement which was slightly lower than that of liver parenchyma in the portal venous phase, decreased enhancement in the equilibrium phase. The lesion showed mild signal, adjacent to inferior vena cava and right hepatic vein which was compressed and displaced in the hepatobiliary phase.There was no obvious cirrhotic nodule in the liver.The patient received laparoscopic space-occupying lesion resection at the right liver lobe after finishing inspection.The grayish white lesion in hardness was seen, with clear boundary and capsule, adjacent to anterior wall of inferior vena cava and right hepatic vein in the operation.The results of pathological examination showed that the small tumor cells were scattered in bundle, nuclear were round or oval shape and eccentric with mitosis seen.The results of immunohistochemical staining showed that endothelium cell marker CD34, human multiple myeloma gene MUM1, vimentin, plasmacyte markers 38 and 138, expression of λ light chain protein were positive, the positive cell rate of proliferation activity marker Ki-67 was 10%.The results of blood routine test and blood biochemistry showed that the patient had no anemia, hypercalcemia, abnormal renal function or monoclonal immunoglobulin in the serum or urine.The results of postoperative bone marrow aspiration, immunoglobulin determination and whole body bone scan showed normal.Postoperative examinations confirmed the SEP of right liver.The patient recovered well and was discharged at postoperative day 9.Postoperative change at right liver lobe was detected by color Doppler ultrasonography at 1 month after operation.The patient was followed up for 6 months without tumor recurrence. Conclusion SEP of liver mainly locates at the right lobe of liver, and the imaging features include clear boundary, homogeneous echo, density or signal, adjacent capsule showing no significantly outer convex, surrounding vein demonstrating no violation and shifted by compression ,enhancement pattern as fast-in and fast-out. Key words: Liver neoplasms; Extramedullary plasmacytoma; Tomography, X-ray computed; Magnetic resonance imaging; Gadolinium-ethoxybenzyl-diethylenetriamine pentaacetic acid

Clinical features and prognostic factors in solitary plasmacytoma.

This study aimed to review the clinical features and outcome of 53 patients with solitary plasmacytoma managed at our Institution between 1976 and 2012. Thirty-five patients had bone solitary plasmacytoma and 18 extramedullary solitary plasmacytoma. Tumour sizes were larger in patients with bone involvement (P=0·003). Treatment consisted of local radiotherapy (n=26), radiotherapy+chemotherapy (n=15), surgery (n=4) and chemotherapy (n=8); the local control rate was 94·3%. Progression to multiple myeloma was recorded in 20/35 (57·1%) patients with bone involvement and in 1/18 (5·5%) patients with extramedullary disease (P=0·0003). The 5-year overall survival (OS) rate was 78·4%; bone solitary plasmacytoma patients had a significantly worse OS (71·9% vs. 88·2%, respectively; P=0·029) and 5-year progression-free survival (PFS; 53·0% vs. 88·5%; P=0·0003) compared to extramedullary solitary plasmacytoma patients. On univariate analysis, bone disease and size (≥5cm) impacted negatively on PFS (P=0·0027 and P=0·04, respectively). Bone disease also affected OS (P=0·04). In multivariate analysis bone location was the only independent prognostic factor for PFS (P=0·0041) and OS (P=0·021). Patients with bone solitary plasmacytoma have a significantly worse prognosis than extramedullary solitary plasmacytoma cases.

Retroperitoneal laparoscopic management of a solitary extramedullary plasmacytoma associated with human immunodeficiency virus infection: A case report.

Extramedullary plasmacytoma (EMP) is a rare malignant tumor that is characterized by a malignant plasma cell neoplasm. Such neoplasms in human immunodeficiency virus (HIV)-infected patients are extremely rare. To the best of our knowledge, the present study describes the first case of a solitary adrenal EMP in a patient with HIV. A 35-year-old male who had been diagnosed with HIV 3 months previously presented with a 2-week history of intermittent right flank pain. Abdominal computed tomography revealed a soft-tissue density mass in the right adrenal gland area. The patient subsequently underwent a retroperitoneal laparoscopic adrenalectomy. Post-operative pathological diagnosis revealed a solitary EMP. Although the patient refused to undergo post-operative radiotherapy and chemotherapy, no recurrence was detected after 2 years of follow-up. The present case illustrates the fact that this rare type of solitary EMP associated with acquired immune deficiency syndrome (AIDS) can occur in the adrenal glands, and that retroperitoneal laparoscopic resection of the tumor may be a good method to manage this condition. In addition, although rare, solitary EMP should be considered in the differential diagnosis of an adrenal mass in HIV-infected patients.

Effect of definitive radiotherapy on the long-term outcome in patients with solitary extramedullary plasmacytoma.

Extramedullary solitary plasmacytoma (EMP) is a rare type of malignancy. This paper presents a retrospective review of the experience with EMP at the Krakow Oncology Center. Records of 17 patients with head and neck EMP, treated with definitive radiotherapy between 1976 and 2009, were analyzed. The total tumour dose ranged from 45 to 70 Gy (median 56 Gy). In four patients with partial response after radiotherapy, adjuvant melphalan-based chemotherapy was applied. The median follow-up period was 8.6 years. The treatment was well tolerated. The estimated 10-year overall survival, disease-free survival, and multiple myeloma-free survival were 68.4%, 49.3%, and 55%, respectively. The 10-year local control rate was 90.9%. No in-field local recurrence was observed. During the follow-up, progression into multiple myeloma was observed in five patients, with a mean time to conversion of 24 months. The only factor adversely affecting overall survival on univariate analysis was the age >56 years, whereas a complete tumour regression after radiotherapy was associated with a significant improvement in both disease-free survival and multiple myeloma-free survival. Despite the high effectiveness of local radiotherapy, there is still a significant treatment failure risk due to the EMP conversion into generalized disease. An attempt to identify prognostic factors may facilitate selection of patients with a high risk of progression to multiple myeloma. Copyright © 2015 John Wiley & Sons, Ltd.

Plasmacytoma rectum extending to para-rectal region

Solitary plasmacytoma most commonly presents in bone (80%), while extramedullary plasmacytoma (EMP) is an uncommon (20%) entity that most commonly involves nasopharynx or upper respiratory tract. Involvement of the gastrointestinal tract occurs in approximated 10% of EMP cases, in that also solitary EMP of the rectum is an exceedingly rare entity. Here we are presenting a case of 55-year-old male patient who was diagnosed to have EMP of rectum in September 2012 who was initially operated in which de-bulking surgery with abdominoperineal resection was done with large residual lesion postoperatively. Then patient was treated with postoperative radiotherapy (RT) with conventional portals 40 Gy in 20 fractions with complete response at 3 months. Patient was followed-up until now (1 year and 2 months post-RT), without any symptom or recurrence.

Extramedullary Plasmacytoma of the Larynx Treated by a Surgical Endoscopic Approach and Radiotherapy

Extramedullary plasmacytoma (EMP) is a rare variant of plasma cell myeloma that affects soft tissues. The head and neck region are the most affected sites, although others have also been described. Herein we report an uncommon case of EMP of the larynx in a 65-year-old male who presented with a history of progressive dysphonia and hoarseness. Laryngeal fiberscopy evidenced a reddish pedicled voluminous mass in the left false cords and ventricle. Microscopic suspension laryngoscopy was performed under general anaesthesia and a 4 W Acublade CO2 Laser was used for transoral resection of the lesion. This was followed by adjuvant radiotherapy, with the widely recommended doses on the supraglottic region, to achieve better local control. Diagnosis of EMP is based on immunohistochemistry and the exclusion of systemic plasma cell proliferative disorders. Diagnosis of solitary EMP can be made only if studies for disseminated disease and X-ray and/or magnetic resonance imaging of the spine, pelvis, femurs, and humerus and bone marrow biopsy are negative. As there are no internationally established guidelines, treatment of EMP is mainly based on consensus of expert opinion.

Plasmablastic Lymphoma : A Case Series of the Changing Epidemiology of a Rare Extramedullary Plasmacytoid Neoplasm, Diagnostic Challenges, and Therapeutic Implications

Introduction:Plasmablastic lymphoma (PBL) is a rare and aggressive lymphoma, with an immunophenotype of a terminally differentiated B-lymphocyte. It is more likely to occur in HIV-positive individuals, commonly involves oral sites, and is associated with EBV infection. However, in recent years, there have been several case reports suggesting the changing epidemiology of PBL affecting HIV-negative patients and increasingly involving non-oral sites.In order to investigate the epidemiology of PBL we conducted a retrospective analysis of all consecutive patients diagnosed with PBL at Montefiore Medical Center between Jan 1997 and May 2014. Patient demographics, tumor location and pathological characteristics, laboratory data, treatment received, and survival data were recorded.Results:A total of 16 patients (8 males, 8 females) with PBL were identified. Median age of all patients was 55.5 years (IQR, 37.3- 62.0), 10/16 (62.5%) patients were HIV positive (median age, 39.5; median CD4, 151.5) and 6/16 (37.5%) were HIV negative, including one post renal transplant patient. Most of the patients had extra-oral involvement 11/16 (68.8%) with the commonest extra-oral site being the GI tract. Half of the patients were Ann Arbor Stage III or IV and the median LDH was only mildly increased at 256 (IQR, 200.5-431.5) despite these tumors having a high Ki67. All tumors were CD20 negative and positive for CD138. EBV encoded small RNA (EBER) was positive in 13/16 patients (81.3%) and was more sensitivie than the EBV latent membrane protein 1 which was positive in only 68.8% of patients suggesting EBV infection. HHV-8 was negative in 12/16 (75%) patients. Only half of the patients (62.6%) had evidence of free light chain restriction (Kappa-restricted, 43.8%, Lambda-restricted, 18.8%). Data on Ki67 was available in 12/16 patients, and 8/12 patients had a Ki67 of >=90%. 4/12 (33.3%) patients had a Ki67 =<50%.Most of the patients received chemotherapy (75%), of which, 7/12 (58.3%) patients received combination chemotherapy with EPOCH. 3/12 (25%) received consolidation with autologous stem cell transplantation. The median overall survival calculated as the number of months from diagnosis to last follow up or date of death was 17 months (0-54). It is of note that 3/16 patients were initially diagnosed with solitary extramedullary plasmacytoma (SEP)/plasmacytoid neoplasm, and the disease course led to revision of the diagnosis to PBL.Conclusion:This is an important study highlighting the epidemiological changes in PBL, an aggressive B cell neoplasm, which is not limited to the HIV-positive population, nor to oral sites. In fact, more than half of our patients with PBL presented with tumors involving extra-oral sites. These tumors were CD 20 negative, and CD 138 positive, and the majority were EBER positive and HHV8 negative. A low Ki67 does not rule out the diagnosis of PBL. Distinction of PBL from SEP may be challenging and the aggressive clinical course of the former may occasionally be the only pointer to the diagnosis. Overall prognosis remains poor and new approaches are warranted in the management of this disease. DisclosuresNo relevant conflicts of interest to declare.

Computed tomography findings of a solitary extramedullary plasmacytoma of the spleen: A case report and literature review.

Solitary extramedullary plasmacytoma (EMP) of the spleen is a rare condition. The present study describes the case of a 23-year-old female with an extremely rare solitary EMP of the spleen. Upon examination, the tumor demonstrated unusual and notable multiple-phase spiral computed tomography (CT) findings. The lesion was a solitary, well-defined mass, with areas of variable splenic necrosis and cystic degeneration. Contrast-enhanced CT revealed progressive enhancement of the lesion in the cystic wall, internal septa and solid portion, a finding that has not previously been described. The patient underwent a splenectomy and recovered without complications. No evidence of tumor recurrence has occurred during the past two years of follow-up. To the best of our knowledge, this is the first study to examine the CT findings of a solitary EMP of the spleen. The study aimed to investigate the imaging features of solitary EMP, in particular the multiple-phase spiral CT findings, and raise awareness of the disease to reduce misdiagnoses.

Solitary extramedullary plasmocytoma of the thyroid: a case report and histological approach to plasma cells infiltrate in the thyroid gland

Background:Solitary extramedullary plasmacytoma (SEP) is a rare malignant neoplasm arising from plasma cells. SEP mostly occurs in the upper respiratory tract. Thyroid gland is rarely affected (<78 cases).Methods/results:We describe the case of a 78-year-old woman presenting a rapidly enlarging palpable thyroid mass. Neck computed tomography scan showed enlargement of both thyroid lobes. Laboratory tests were normal, including serum protein level with no monoclonal gamma globulin peak. Cytology was suspicious for lymphoma. Biopsy showed an infiltrating neoplasm composed of atypical tumor cells with abundant cytoplasm and eccentric nuclei. These revealed diffuse immunoreactivity for CD138 and predominant staining for immunoglobulin kappa light chains. Clinical workup for multiple myeloma was negative.Conclusions:SEP should be considered in the differential diagnosis of a rapidly enlarging thyroid nodule and be distinguished from involvement of thyroid in multiple myeloma, mucosa-associated lymphoid tissue lymphoma, plasma cell granuloma and medullary carcinoma. Clinical correlation and immunohistochemistry are crucial in avoiding pitfalls.

Endoscopic Mucosal Resection (EMR) for the Management of Solitary Extramedullary Plasmacytoma of the Esophagus

Introduction: We present a first report of EMR for management of Solitary Extramedullary Plasmacytoma (EMP) of the esophagus. Case Report: A 50-year-old man with chronic intermittent dysphagia secondary to eosinophilic esophagitis (EoE) was referred for management of an incidental mid-esophageal mass which was biopsy proven to be an EBV+ plasma cell neoplasm. Extensive work-up for an underlying plasma cell dyscrasia including multiple myeloma confirmed this to be a solitary EMP. Endoscopic reassessment demonstrated an irregular sessile 1.0-1.5 cm mid-esophageal nodule at 25 cm. EUS showed a hypoechoic nodule involving the mucosa and submucosa with intact muscularis propria. The lesion was successfully resected by EMR using a multi-band mucosectomy device without submucosal injectate. The primary lesion was resected with a single band ligation although a second ligation was performed given a grossly close margin. No additional thermal ablation therapy was used. No endoclip closure was required. No immediate or delayed complications were observed. Pathologic and immunohistochemistry assessment was compatible with solitary EMP with marked expansion of the submucosa by sheets of large plasmacytoid cells without blastic appearance. Despite EBV+, there were no features of plasmablastic lymphoma. Peripheral and deep resected margins from the primary band ligation were free of tumor. Only 5-13% of EMPs occur in the gastrointestinal tract. Standard therapy for solitary plasmacytoma involving extramedullary sites involves definitive radiation therapy, followed by surgery if necessary with an overall good prognosis. Endoscopic management has not been well described given limited data. Only one prior case report has described successful endoscopic management of a subcentimeter mucosal EMP using endoscopic submucosal resection (ESD). However this technique is not yet widely performed and is associated with a higher risk for perforation. EMR is already widely used for the diagnostic and therapeutic management of mucosal and submucosal neoplastic lesions. EMR appears to be a feasible and less invasive alternative to radiation and surgery for select small, well defined EMP lesions limited to the mucosa and submucosa of the esophagus. EUS can help assess those lesions appropriate for EMR. Long term follow up of endoscopic treatment of solitary esophageal EMPs is needed. Surveillance EGD with biopsies will be repeated at three months. Repeat endoscopic management, radiation and surgery remain potential salvage therapeutic options.

Endoscopic Ultrasound Fine Needle Aspiration: A Novel Way to Diagnose a Solitary Extramedullary Plasmacytoma of the Liver

Endoscopic Ultrasound Fine Needle Aspiration: A Novel Way to Diagnose a Solitary Extramedullary Plasmacytoma of the Liver

¹⁸F-FDG PET/CT: a review of diagnostic and prognostic features in multiple myeloma and related disorders.

Conventional radiographic skeletal survey has been for many years the gold standard to detect the occurrence of osteolytic lesions in patients with multiple myeloma (MM). However, the introduction of more sensitive imaging procedures has resulted in an updated anatomic and functional Durie and Salmon "plus" staging system and has remarkably changed the diagnostic and prognostic approach to this tumor. It is now established that (18)fluorine-fluorodeoxyglucose ((18)F-FDG) positron-emission tomography (PET) combined with low-dose computed tomography (CT), shortly designated PET/CT, exhibits a higher screening and diagnostic sensitivity and specificity over the skeleton X-ray. In patients with monoclonal gammopathy of undetermined significance and in those with smoldering MM, PET/CT is consistently unable to detect focal and/or diffuse marrow abnormalities. Conversely, based on a systematic review of 18 studies comprising almost 800 MM patients, PET/CT was able to detect MM osteolytic lesions with a sensitivity of approximately 80-90% and a specificity of 80-100%. Importantly, a poor degree of concordance has also been emphasized between PET/CT and whole-body magnetic resonance imaging (WB-MRI) in that when both techniques were applied to the same patients, double-positive results were recorded in approximately 30% of the cases, but in the majority of them, a higher number of lesions were revealed with PET/CT than with MRI. Double-negative results, on the other hand, were found in about 22% of the patients. Because PET/CT is able to identify tumor foci throughout the body, it can be usefully applied to the study of solitary bone plasmacytoma and extra-medullary plasmacytoma: In both conditions, the detection of additional, previously overlooked sites of skeletal involvement would falsify the diagnosis of single-district disease, upstage the tumor, and therefore require a different therapeutic approach. In addition, although PET/CT is poorly sensitive to diffuse bone marrow infiltration, it can anticipate a site of impending fracture throughout the body and can discriminate old from new pathologic fractures. MRI should, however, be preferred when vertebral bodies are suspected to be involved and the risk of vertebral fracture is to be assessed. PET/CT is a sensitive and reliable procedure to evaluate the response to chemotherapy and/or radiotherapy, which is shown by a remarkable reduction and sometimes total disappearance of FDG accumulation in the involved bony structures, although these structures remain morphologically abnormal. Conversely, an increased focal uptake of FDG in apparent remission patients often precedes clinically overt relapse. PET/CT should be preferred to other imaging techniques to assess the remission status after autologous stem cell transplantation. In patients with primary and remission-induced non-secretory MM, the use of PET/CT may help to early detect single or multiple districts of focal non-secretory relapse. Osteonecrosis of the jaw, its location, and extent in MM patients receiving bis-phosphonates are better defined by both PET/CT and contrast-enhanced MRI compared with dental panoramic views derived from cone beam CT imaging. Little is known as to the possible role of PET/CT in the assessment of disease extension, tumor load, and response to therapy in patients with Waldenström's macroglobulinemia (WM). In a study conducted on 35 WM patients, comparative PET/CT before and after therapy was able to detect positive findings in 83% of the patients, in contrast with the previous results achieved with conventional imaging that reported visceral involvement in much lower percentages. Similarly scanty are the data on the use of PET/CT in localized and systemic amyloidosis, given the small number of patients studied so far. A retrospective study has shown that, at variance from (123)Iodine-serum amyloid P component ((123)I-SAP) scintigraphy, which was found to be positive in about one-third of the patients with localized amyloidosis, an increased FDG uptake was detected at the amyloid site in virtually all of them. On the contrary, none of the patients with systemic amyloidosis showed an increased FDG uptake in sites of known deposition, whereas (123)I-SAP scintigraphy tested positive in the large majority of them. In another study, however, no such remarkable difference of positive PET/CT scans between localized and systemic amyloidosis was reported. Finally, false-positive and false-negative PET/CT findings can occur in different conditions that should be kept in mind to avoid wrong or omitted diagnoses.

Extramedullary plasmacytoma of the frontal sinus: case report and Turkish literature review.

Solitary extramedullary plasmacytomas (EMPs) are nonepithelial neoplasms of plasma cell origin categorized among non-Hodgkin lymphomas, without the bone marrow involvement and systemic spread seen in multiple myeloma. They are uncommon tumors comprising 3% of all plasma cell neoplasias. Although they usually occur in the upper respiratory tract, only 1 case of EMP localized to the frontal sinus has been reported in the English literature. We present in this report a rare case of EMP originated from the left frontal sinus leading to left eyeball proptosis and movement restriction. A survey of sinonasal EMPs in the Turkish literature is reported, as well. Paranasal computerized tomography and magnetic resonance imaging of a 69-year-old female who presented with left eyeball proptosis and left-sided headache revealed a solid mass in the left frontal sinus. Histopathological analysis of the completely excised mass supported the diagnosis of plasmacytoma. The definitive diagnosis of solitary EMP was confirmed with further investigations at hematology and oncology clinics. The patient was treated with surgery followed by local radiotherapy to the head and neck region, and she was disease-free at her 1-year follow-up. Treatment of sinonasal EMP is surgery alone or surgery combined with radiotherapy. Long-term follow-up is a requisite for systemic control because of the disease’s high potential to transform into multiple myeloma.

Multiparameter flow cytometry for staging of solitary bone plasmacytoma: new criteria for risk of progression to myeloma

AbstractSolitary plasmacytoma represents a heterogeneous group of patients; approximately half develop multiple myeloma (MM) in 2 or 3 years, whereas others remain disease-free at 10 years. By definition, these patients do not have morphologic bone marrow (BM) plasma cell (PC) infiltration. Here, we investigated whether sensitive BM evaluation of patients with solitary bone plasmacytoma (SBP; n = 35) and extramedullary plasmacytoma (EMP; n = 29) through multiparameter flow cytometry (MFC) would unravel the presence of clonal PCs in otherwise disease-free BM, and whether BM clonality predicted higher risk of progression. BM clonal PCs were detected in 17 of 35 SBP (49%) and 11 of 29 EMP (38%) patients. Seventy-one percent of flow-positive vs only 8% of flow-negative SBP patients evolved to MM (median time to progression of 26 months vs not reached; hazard ratio, 17.4; P < .001). No significant differences were observed among EMP cases. Our results highlight the importance of MFC for sensitive BM evaluation of SBP patients, to predict risk of developing treatment-requiring MM and to plan disease monitoring.

Neuroimaging of plasmacytoma. A pictorial review.

This pictorial review describes the spectrum of CT/MR imaging findings of solitary extramedullary and bone plasmacytomas in different locations related to neuroradiology. Plasmacytoma is considered a counterpart of multiple myeloma that is described as a solitary and discrete mass of monoclonal neoplastic plasma cells. It may arise from osseous (medullary) or extramedullary sites. Isolated extramedullary plasmacytoma is very rare and comprises less than 4% of all plasma cellular diseases of which more than 80% are localized to the submucosal lymphoid tissue of the nasopharynx, nasal cavity and paranasal sinuses. We will demonstrate imaging findings in ten histopathologically proven plasmacytomas in different locations related to neuroradiology. Extramedullary and osseous plasmacytoma show nonspecific CT and MR imaging findings. MR is the preferred modality for evaluation due to better soft tissue contrast. Features that may suggest the diagnosis of plasmacytoma are bulky soft tissue mass and relatively isointense signal on T2-weighted MR images due to high cellularity.

Secondary Plasmacytoma of The Orbit In A Patient With Refractory Ig G Kappa Multiple Myeloma

Multiple myeloma (MM) is a malignant neoplasm of the plasma cells affecting many organ systems. MM typically presents with bone lesions, anemia, hypercalcemia, kidney disease, bone marrow failure and various infections. Plasmacytoma of soft tissues is not a common manifestation of MM. Primary plasmacytoma can involve soft tissues (solitary extramedullary plasmacytoma) or bone (solitary bone plasmacytoma). It accounts for <10% of plasma cell malignancies and commonly affects the upper respiratory tract and later on invades the orbit. MM accounts for nearly 90% of plasma cell tumors and may present as secondary plasmacytoma.1 Orbital involvement by secondary plasmacytoma is rare and considered as worse prognosis for systemic morbidity.2-5 We report a case refractory Ig G kappa MM with multiple relapses, bone fractures and renal dysfunction who presented with orbital lesions.

Solitary Extramedullary Plasmacytoma of the Parotid Gland Imaged With 18F-FDG PET/CT

A 75-year-old woman presented with a 1-year history of an enlarging mass in the left parotid gland. Biopsy revealed a parotid plasmacytoma, and the patient was referred for a staging F-FDG PET/CT to evaluate the presence of multiple myeloma bone involvement. The PET/CT scan showed intense FDG uptake in the neck mass but no FDG-avid lymphadenopathy or distant metastases. Plasmacytoma involving the parotid gland is extremely rare. We present the F-FDG PET/CT imaging of solitary parotid plasmacytoma.

Clinical features, outcome, and prognostic factors for survival and evolution to multiple myeloma of solitary plasmacytomas: a report of the Greek myeloma study group in 97 patients.

Solitary plasmacytoma (SP) is a rare plasma cell dyscrasia characterized by the presence of bone or extramedullary plasma cell tumors. The treatment of choice is local radiotherapy (R/T) ± surgical excision. The role of adjuvant chemotherapy (C/T) or novel agents (NA) is uncertain. Data related to prognostic factors are inconclusive. Herein, we describe the clinical features, survival and prognosis of 97 consecutive patients, 65 with bone SP (SBP), and 32 with extramedullary SP (SEP), diagnosed and treated in 12 Greek Myeloma Centers. Objective response rate (≥PR) and complete response (CR) was 91.8% and 61.9%, respectively, and did not differ between the 2 groups. Overall, 38 patients relapsed or progressed to multiple myeloma (MM). After a median follow-up of 60 months, 5 and 10-year overall survival (OS) probability was 92% and 89% in SEP and 86% and 69% in SBP, respectively (P = 0.2). The 5- and 10-year MM-free survival (MMFS) probability was 90% and 70% for patients with SEP vs. 59% and 50% for patients with SBP, respectively (P = 0.054). Overall, the 5- and 10-year OS probability, plasmacytoma relapse-free survival (PRFS), progression-free survival and MMFS was 84% and 78%, 72% and 58%, 58% and 43%, and 70% and 59%, respectively. In the multivariate analysis, prolonged PRFS and young age were positive predictors of OS. Achievement of CR was the only positive predictor of PRFS. Immunoparesis was the only negative predictor of progression to MM. The addition of C/T or NA-based treatment increased toxicity without offering any survival advantage over R/T.

Liquid‐based cytologic findings of solitary extramedullary plasmacytoma in thyroid: A case report identified with fine‐needle aspiration cytology

Preoperative cytologic diagnosis of solitary extramedullary plasmacytoma (SEP) presents a challenge to cytopathologists because this tumor rarely occurs as a primary thyroid malignant neoplasm. In this report, we provide the first description of liquid-based cytomorphologic findings of SEP observed on fine-needle aspiration biopsy (FNAB) of the thyroid. A 56-year-old woman with a history of Hashimoto thyroiditis presented with a growing nodule in the thyroid. The liquid-based preparation obtained from FNAB showed numerous dispersed plasmacytoid cells with occasional loosely cohesive aggregates of tumor cells. Cells were round to oval in shape, with eccentrically located nuclei. Hyalinized perinuclear vacuoles were found in the cytoplasm of the tumor cells. Initial cytological findings, including those of immunochemistry using a cell block preparation, were consistent with plasmacytoma. Histopathological examination subsequent to thyroidectomy revealed a plasmacytoma in the thyroid. Plasma cell neoplasms were not concurrently detected in tissues other than the thyroid. On the basis of preoperative FNAB findings, a specific diagnosis of SEP in the thyroid can be difficult because this tumor is rare. Moreover, diagnosis is impeded because SEP in the thyroid resembles other, more common thyroid lesions, including both benign and malignant neoplasms. Careful cytomorphologic examination and supportive studies may be required to fully confirm a diagnosis of SEP.

Head and Neck Solitary Extramedullary Plasmacytoma

Introduction. This study aimed to report the characteristics and treatment outcome of 13 patients with solitary extramedullary plasmacytomas of the head and neck and analytical literature review. Materials and Methods. Nine patients (69%) were treated with gross surgical resection followed by radiotherapy, three (23%) were primarily treated with radiotherapy alone, and one (8%) was treated with surgery alone. Results. There were 3 women and 10 men with the median age of 52 years. Nasal cavity (46%) and paranasal sinuses (23%) were the most common primary sites. After a median follow-up of 28 months, 10 patients are alive and free of disease, one is alive with multiple myeloma, and two died of multiple myeloma. In the literature review the median age was 58 years and male/female ratio was 2.7. Sinonasal tract was the most common primary site. Ten-year local control and progression to multiple myeloma rates were 88% and 23%, respectively. The 5- and the 10-year overall survival rates were 71 and 69%, respectively. Conclusion. Radiation therapy with or without surgery is an effective treatment for patients with head and neck extramedullary plasmacytoma. However, long-term follow-up for detection of local recurrence and progression to multiple myeloma is essential.