IMAGING

A Rare Case of Extramedullary Adrenal Plasmacytoma Associatedwith IVC Thrombus: A Case Report with Review of Literature

M. A. Elbaset1 & Ibrahim El-said1& Rasha T. Abouelkheir2 & Mohamed Badawy2 & M. Abd Elhameed3

& Yasser Osman1

Accepted: 12 May 2020# Springer Nature Switzerland AG 2020

AbstractExtramedullary plasmacytoma (EMP) is one of the rare plasma cell malignancies. It accounts for 3% of all plasma cell tumors andmost frequently presented in the aerodigestive tract. EMP may likewise involve the urinary tract, central nervous system (CNS),thyroid, breast, testis, parotid gland, lymph nodes, and skin. Only nine cases of the adrenal EMP were reported in the literature.Whether none of these cases were described to be concomitant with venous thrombus. Herein, we presented a 61-year-old malepatient who presented with an incidentally discovered non-functioning right suprarenal mass. Magnetic resonance imaging(MRI) venography showed right non-adenomatous adrenal mass measured 11 × 9.5 × 12 cm. The tumor was associated withvenous thrombus extending into the intrahepatic portion of the inferior vena cava (IVC). Bone scan suspected bony hot areas, butskeletalMRI excluded any lytic bony lesions ormetastatic deposits. Core biopsywas taken andmicroscopic examination showedsheets of loosely cohesive malignant small rounded cells; most of them were plasma cells. Immunohistochemical staining waspositive for Vimentin, CD-138, and CD-56. The pathological data were revised by two other eminent pathologists, and theyconfirmed the final pathological diagnosis to be adrenal plasmacytoma. We could conclude that, adrenal EMP is a truly rareentity, it can be associated with venous thrombus. Imaging is helpful in this setting for the identification of associated venousthrombus and to exclude any lytic bone lesions.

Keywords Plasmacytoma . Adrenal . IVC Thrombus . Tumor .Multiplemyeloma . Plasma cell

Introduction

Plasma cell tumors can be classified into four groups: multiplemyeloma (MM), plasma cell leukemia, solitary plasmacytoma(SP) , and extramedul lary plasmacytoma (EMP).Plasmacytomas are defined as a clonal growth of atypicalplasma cells. SPs commonly occur in bone [1] whereasEMP is one of the infrequent plasma cell malignancies.EMP accounts for only 3% of all plasma cell tumors and mostcommonly affects the aerodigestive tract. Besides, it can affect

the urinary tract, CNS, thyroid, breast, testes, parotid gland,lymph nodes, and skin [1]. In the literature, adrenal EMP wasdescribed in nine places only [2].

The association of adrenal tumors with venous thrombus istypically scarce. In a relatively large series, adrenal masseswere reported concomitantly associated with venous throm-bosis in only 2.9% (6/206) [3]. The highest level of thrombusextension reported in this series was identified in the ipsilateralrenal vein [3]. No previous reports described the association ofadrenal EMP with venous thrombus. Here, we reported thefirst case of incidentally discovered adrenal EMP concomi-tantly associated with venous thrombus extending to theintrahepatic portion of the IVC.

Case Presentation

A 61-year-old hypertensive male patient presented to our out-patient clinic with an incidentally discovered right suprarenalmass. He was receiving β-blocker with controlled hyperten-sive status. Neither lymphadenopathy, limb edema, ascites nor

This article is part of the Topical Collection on Imaging

* M. A. Elbasetabdelbaset.m.i@gmail.com

1 Urology department, Urology and Nephrology Center, MansouraUniversity, Mansoura, Egypt

2 Radiology department, Urology and Nephrology Center, MansouraUniversity, Mansoura, Egypt

3 Pathology department, Urology and Nephrology Center, MansouraUniversity, Mansoura, Egypt

SN Comprehensive Clinical Medicinehttps://doi.org/10.1007/s42399-020-00321-0

/Published online: 25 May 2020

(2020) 2:792–796

organomegaly was detected. All laboratory investigations in-cluding hemoglobin, serum creatinine, and serum calciumwere all within normal values. Hormonal profile to assesstumor functionality was also normal. Serum alkaline phospha-tase was slightly elevated (130 IU) (normal, 90–125 IU) withno associated bony aches. Plasma viscosity was 1.9 unit (nor-mal, 1.2–1.7 unit). Serum β2-microglobulin was 12.17 mg/dl(normal, 0.8–2.2 mg/dl). Free light chain assay revealed anormal immunoglobulin pattern with normal Kappa/Lambdaratio. Serum protein electrophoresis (SPEP) excluded MMwith no evidence of plasma cells. Urinary immunoelectropho-resis revealed kappa Bence-Jones proteinuria. Bone marrowaspiration did not show any abnormal plasma cell infiltrate

either numerically or morphologically. Bone marrow cytoge-netics were also normal.

Abdominal ultrasonography (US) revealed right isoechoicmass at the site of the adrenal gland with normal both kidneys.MRI venography (Fig. 1) showed a right non-adenomatousadrenal mass measured 11 × 9.5 × 12 cm. The mass was seendisplacing the inferior surface of the liver; in addition, tumorthrombus was noted extending to the intrahepatic portion ofthe IVC. The mass was separable from the right kidney.

Contrast-enhanced computed tomography (CECT) of thechest was done, and enlarged multiple mediastinal lymphnodes were noticed. A bone scan was considered because ofhigher alkaline phosphatase. Multiple hot areas were seen

Fig. 1 MRI of the abdomen a andb, coronal, and axial T2WI showa large right suprarenal soft tissuemass (arrows) displaying hetero-geneous SI with central areas ofhigh SI denoting high cellularity.c In phase chemical shift MRI andd opposed phase out chemicalshift MRI show no drop of SI atthe opposed phase in comparisonto in phase denoting non-adenomatous nature of themass. eand f Axial and coronal post-contrast T1WI with fat suppres-sion show mild heterogeneouspost-contrast enhancement of themass with tumor thrombus in-vades the contrast filled hepaticportion of the IVC and confluenceof hepatic veins (arrows)

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located in the right hip joint and right clavicle. We suspectedthese hot areas either to be metastatic deposits or due to de-generative bony changes. So, whole skeletal MRI was doneand excluded the presence of bony lytic metastatic deposits.

Core biopsy was taken and microscopic examinationshowed sheets of loosely cohesive malignant small roundedcells; most of them were plasma cells with eccentric nucleiand granular cytoplasm. Immunohistochemical staining waspositive for Vimentin, CD-138, and CD-56 and negative forCD-79R, CD-45, CD-20, Synaptophysin, and pan-CK. Also,Ki-67 proliferation was detected in 60% of the sample taken.Tumor cells were negatively stained for Kappa and positivelystained for Lambda (Fig. 2). The final pathology was con-firmed to be adrenal plasmacytoma. For the disease rarity,slides were examined by two other different eminent oncolog-ical pathologists to confirm our diagnosis.

According to our multidisciplinary meeting including on-cologists considering the final diagnosis of EMP concomitant

with a higher level of IVC thrombus in association with extraregional mediastinal lymph nodes, the decision was taken tosend the patient for chemotherapy.

Discussion

Plasmacytoma is considered an early stage of plasma cell ma-lignancy, which may lead to MM along the spectrum of plas-ma cell disorders. It is hypothesized that repeated trauma tothe plasma cells might trigger cell proliferation and clonalinfiltration and might lead to plasmacytoma formation [4].

In a previous review of literature discussing adrenal EMP[2], only nine cases were reported without a description of anyconcomitant venous thrombus (Table 1). Demographically,males were more likely affected than females (6 vs 3 patients).Age of presentation ranged from 26 to 77 years, with a medianage of 57 years. The right side was commonly affected than

Fig. 2 a and b Parts of adrenalcortical tissue with evidence oftumoral proliferation formed ofdiffuse infiltration of atypicalplasma cells with basophiliccytoplasm, large central roundednuclei with prominent nucleoli.Focal areas of necrosis werenoticed (HX and E × 200). c, d, e,and f: Immunohistochemicalstaining negative for MUM1,positive for lambda light chain,negative for Kappa, and positivefor CD138, respectively (100×)

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the left side (5 vs 1). Whether bilateral lesions were describedin the other remaining cases.

There was no typical clinical presentation. Two-third of thereported patients presented with different symptoms, includ-ing back or abdominal pain or generalized fatigue. The otherthird of patients were asymptomatic. Only one case is present-ed with metabolically active adrenal mass [5].

In the differential diagnosis with other hematopoietic-related malignancies, CRAB features (elevated serum calci-um, renal impairment, decreased hemoglobin level, and manybone lesions) are the main presentation associated with MM.Conversely, in bone marrow biopsy, plasma cell infiltrationbesides the absence of osteolytic bony lesions or other tissueinvolvement is considered indicative criteria for EMP in com-parison withMM [6, 7]. EMPmust also be distinguished fromlymphoma. This could be done by the primary lesion biopsy.In EMP, the infiltrate consists entirely of plasma cells not Bcell infiltrate in cases of lymphoma. By the way, CD-138,MUM1/IRF4, CD-20, and PAX5 are the most beneficialmarkers for lymphoma diagnosis. Nevertheless, CD-20 andPAX5 could be occasionally expressed in plasma cell malig-nancies [8].

In our case, the diagnosis of EMP was established in dif-ferent aspects of clinical, laboratory and pathological findings.But, suspected bony metastasis noted in the bone scan (whichis considered a criterion for MM) [9] was the only contradic-tory point to our diagnosis. Confirmatory skeletal MRI wasbeneficial in excluding bony metastasis. Also, MRI was ofvalue identifying concomitant venous thrombus.

Generally, adrenal tumors associated with venous throm-bus were rarely documented [3]. In a previous series

discussing adrenal masses, associated venous thrombosis waslimited to the adrenal vein in 4 patients, while it extended tothe ipsilateral renal vein in two other patients. Pathological diag-noses of these adrenal masses with concomitant venous throm-bosis were neuroblastoma, pheochromocytoma, adrenocorticalcarcinoma, and pleomorphic sarcoma. In another case reports[10–12], venous thrombosis reaching the right atrium was rec-ognized to be concomitant with adrenocortical carcinomas.

Conclusion

Adrenal EMP is a truly rare entity; it can be associated withvenous thrombus. Diagnosis is entirely based on history,physical examination, a bone marrow aspiration, serum pro-tein electrophoresis, and urine testing for Bence-Jones protein-uria. Also, imaging is helpful in this setting for the identifica-tion of associated venous thrombus and to exclude any lyticbone lesions. These findings may fashion the managementplan for such patients.

Availability of Data and Material Data and material are available.

Author’s Contribution M.A.E, manuscript writing and data collection;I.S, data collection; R.T.A and M. B, radiological supervision; M.Elhameed, pathology revision; and Y. O, manuscript revision and super-vision. All authors have read and approved the manuscript.

Compliance with Ethical Standards

Competing Interests The authors declare that they have no conflict ofinterest.

Table 1 Patient’s demographics, characters, and diagnosis of previous case reports

Study, year Age Sex Site Symptoms functionality Tumorsize

Bone marrowaspirate

Urinary Bence-Jonesprotein

Serumelectrophoresis

Kahara et al 2001(14)

52 Male Right Incidentaloma No 4 Negative ……… Positive

Asahi et al,2001(15)

52 Male Right Incidentaloma No 4 Negative ………. Positive

Fujikataet al,2002 (5)

77 Male Right Pain Yes 10 Negative Negative Positive

Rogers 2004 (16) 75 Female Right Pain No 3.5 Negative Negative Normal

Li 2007 (17) 84 Female Bilateral Pain No Right 8Left 4

Negative Negative Normal

Ahmed et al.2009(6)

47 Male Bilateral Incidentaloma No Right 8Left 8

Negative Positive Positive

Blanco et al. 2011(18)

78 Female Left …….. No 6 – – –

Cao et al. 2014(1)

26 Male Right Pain No 3.4 Negative Negative Positive

Townend 2016(2)

57 Male Bilateral Abdominalcramping

No Right5.5

Left 9.5

Negative Positive Positive

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Ethics Approval and Consent to Participate Informed written consentwas taken from the patient. Internal review board approval not needed asit is a case report.

Consent for Publication Written informed consent was obtained fromall individual participants included in the study.

Abbreviation EMP, Extra-medullary plasmacytoma; MRI, Magneticresonance imaging; CECT, Contrast enhanced computed tomography;CNS, Central nervous system; IVC, Inferior vena cava; MM, Multiplemyeloma; SP, Solitary plasmacytoma

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