CC BY-NC-ND 4.0 · J Neurol Surg Rep 2024; 85(04): e167-e177
DOI: 10.1055/a-2444-3438
Review Report

Sinonasal Plasmablastic Lymphoma: A Systematic Review

Sophia Chen
1   Department of Head and Neck Surgery, UCLA Health, Los Angeles, California, United States
,
Haidee Chen
2   David Geffen School of Medicine at UCLA, Los Angeles, California, United States
,
Sophie Song
2   David Geffen School of Medicine at UCLA, Los Angeles, California, United States
3   Department of Pathology and Laboratory Medicine, UCLA Health, Los Angeles, California, United States
,
Marilene B. Wang
1   Department of Head and Neck Surgery, UCLA Health, Los Angeles, California, United States
2   David Geffen School of Medicine at UCLA, Los Angeles, California, United States
› Institutsangaben
Funding None.
 

Abstract

Objective Plasmablastic lymphoma (PBL) is a type of non-Hodgkin's B-cell lymphoma associated with human immunodeficiency virus and Epstein–Barr virus, commonly located in the oral cavity or gastrointestinal tract. Sinonasal involvement is rare, and there is no consensus on treatment.

Data Sources Peer-reviewed published articles served as data sources.

Review Methods A systematic review was conducted of the PubMed database for all cases of sinonasal PBL between 1978 and 2023 with the phrase “plasmablastic lymphoma.” Studies not written in English and that did not separate individual cases of sinonasal PBL from aggregated data were excluded. Age, sex, immune status, treatment, and outcomes were collected.

Conclusion PBL is a rare malignancy in the sinonasal region usually treated with chemotherapy. It most commonly occurs in immunocompromised adults but has also been diagnosed in immunocompromised children and in immunocompetent adults. It is aggressive and has a poor prognosis.

Implications for Practice PBL is a recently described entity with few cases of the sinonasal anatomic variant in the literature. Sinonasal PBL was most frequently treated with chemotherapy alone, closely followed by chemoradiation. The most common chemotherapy regimen utilized in the literature is cyclophosphamide, doxorubicin, oncovin/vincristine, and prednisone, which is also the most common chemotherapy regimen in nonsinonasal PBL. A second commonly used regimen is cyclophosphamide, vincristine/oncovin, doxorubicin/adriamycin, and dexamethasone. However, no treatment has emerged as superior to others with regard to survival. Further data are needed to better understand this rare disease.


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Introduction

Plasmablastic lymphoma (PBL) is a rare and highly aggressive form of non-Hodgkin's B-cell lymphoma that was first described in 1997.[1] Typically, PBL is extranodal and is most commonly seen in the oropharyngeal area, followed by the gastrointestinal tract, lymph nodes, and skin.[1] [2] [3] PBL most commonly presents in males and can occur at any age, although it very rarely presents in children.[4] [5] [6] [7] [8] [9] Additionally, PBL is commonly associated with human immunodeficiency virus (HIV) and Epstein–Barr virus (EBV). It presents with an aggressive clinical course, frequent relapse, and poor prognosis.[1] PBL in HIV-positive patients often presents with more advanced disease than in immunocompetent patients.[5] [10] [11] Respective of immune status, median overall survival is generally less than 12 months.[4] [7]

PBL often presents as tumors in extranodal sites, notably in the oral cavity and/or gastrointestinal tract, with immunosuppressed posttransplant patients showing a higher incidence of nodal and skin involvement.[4] Upon PBL presentation, more than 65% of HIV-positive patients, 50% of posttransplant patients, and 25% of seemingly immunocompetent patients demonstrate Ann Arbor stages III and IV.[2] In HIV-positive patients, bone marrow involvement has been shown in up to 40% of patients.[4]

PBL is a rare disease with no widely accepted evidence-based therapies. The most commonly used PBL treatment regimen is a combination of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP).[10] However, guidelines established by the National Comprehensive Cancer Network (NCCN) on AIDS-related B cell lymphomas recommend more aggressive therapy such as dose-adjusted etoposide, vincristine, and doxorubicin with cyclophosphamide and prednisone (DA-EPOCH).[4] [12] [13] Neither CHOP nor more intensive treatment regimens like DA-EPOCH have been demonstrated to be superior over the other when comparing overall survival.[14]

There exists a paucity of PBL cases that are reported in the sinonasal region, and there is no compilation in the literature of sinonasal PBL. Here we gather all reported cases of sinonasal PBL and analyze them as a cohort to present a comprehensive review of this rare disease.


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Methods

A systematic review of the literature was conducted by doing a comprehensive search of PubMed for all cases of sinonasal PBL. The search was performed with the query of “plasmablastic lymphoma,” which returned 928 articles. Exclusion criteria included articles not written in English. Of the 928 studies, 55 assessed PBL in the nasal cavity and/or nasal sinuses. Two studies were excluded for being inaccessible beyond their abstracts, and three studies with 29 cases were excluded as individual data could not be separated from aggregated data. Two studies were excluded because the full-length article was not available in English. After these studies were excluded, 48 studies presenting case reports or case series of sinonasal PBL between the years of 1978 and 2023 resulted in 73 individual cases. From these 48 articles, patient information including age, sex, immune status, treatment regimen, staging, and outcomes were collected. The article selection process is depicted in [Fig. 1].

Zoom Image
Fig. 1 Preferred reporting items for systematic reviews and meta-analyses showing article selection process.

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Results

Final analysis of the literature search included 73 individual cases of sinonasal PBL from 48 studies ([Table 1]).[3] [7] [8] [15] [16] [17] [18] [19] [20] [21] [22] [23] [24] [25] [26] [27] [28] [29] [30] [31] [32] [33] [34] [35] [36] [37] [38] [39] [40] [41] [42] [43] [44] [45] [46] [47] [48] [49] [50] [51] [52] [53] [54] [55] [56] [57] [58] [59] Age ranged from 6 to 91 years. Five patients were missing sex information, six had no reported immune system status, 41 were without staging information, and nine without treatment status. Individual cases were mostly male 80.9% (55/68). The majority were immunocompetent (42/67, 62.7%). Twenty-six individuals were immunocompromised. In total, 35.8% (24/67) were HIV positive, one of the 67 (1.5%) had been on prolonged steroids for eosinophilic granulomatosis with polyangiitis and chronic rhinosinusitis with polyps, and one (1.5%) was an organ transplant recipient. Of those with staging, 43.8% (14/32) were diagnosed with advanced stage disease, including 40.6% (13/32) who presented with stage IV disease. Of those 13 patients with stage IV disease, two patients had distant metastasis, one to the liver and one to the central nervous system.

Table 1

Cases of sinonasal plasmablastic lymphoma reported in the literature

Paper

Age

Gender

Immunocompromised

Presentation

Stage

Surgery

Chemo

Radiation

Outcome

Chicuellar et al 2022

72

F

No

Unilateral left nasal obstructive symptoms, ipsilateral facial pain and numbness for 3 mo, no B symptoms, left maxillary sinus PBL

Velcade/bortezomib + etoposide, prednisone, vincristine, oncovin (V-EPOCH), cyclophosphamide, doxorubicin hydrochloride ×2 cycles with additional high-dose methotrexate ×3 cycles

Radiation to local site

Significant disease progression with treatment strategy reevaluation at time of writing

DiBlasi et al 2022.

57

M

No

Benign nasal polyposis treated with endoscopic sinus surgery (ESS), polypectomy, recurrent nasal airway obstruction (NAO) and anosmia 1.5 y later progressing to left NAO and intermittent night sweats

IV (involved 8th rib, sub-trochanteric left hip)

ESS, polypectomy b/l for diffuse polyps in bilateral sinuses, followed by 2nd ESS and polypectomy, postradiation 3rd ESS with repeat polypectomy for recurrent polyposis

2 mo postop: 6 cycles of DA-EPOCH and 2 treatments of intrathecal methotrexate (MTX)

4 mo after chemo: consolidation intensity-modulated radiation to left maxilla (hyper-metabolic on PET/CT)

Alive, followed q6-12 mo, with peripheral neuropathy of hands and feet

Lee et al 2022

61

M

No

3 wk of epistaxis, nasal airway obstruction—left nasal cavity mass

Spontaneous regression without treatment, in remission 4 y later

Rapiti et al 2022

13

F

HIV+

Nasopharynx

IV (liver)

CHOP

Lost to follow-up

Rapiti et al 2022

32

M

HIV+

Ethmoid sinus

IV (CNS)

CHOP

Sabry et al 2022

59

M

Extended period of steroids for eosinophilic granulomatosis with polyangiitis and chronic sinusitis with polyps

2 wk of left frontal sinus pain and swelling—left paranasal sinus mass extending into left orbit and left anterior cranial fossa

Endoscopic excision of left sinonasal mass, left anterior and posterior ethmoidectomies, left middle meatal antrostomy, left middle turbinectomy, left sphenoidotomy, left frontal sinusotomy/empyema and pneumocephalus requiring burr hole drainage and 3 craniotomies

Cyclophosphamide, etoposide, oncovin, prednisone (CEOP) and intrathecal methotrexate, weekly rituximab x4; postradiation 2nd cycle of R-CEOP and high dose MTX; 3rd R-CEOP and MTX/refractory and started on lenalidomide, bortezomib, dexamethasone (RVD) for 5 cycles, then lenalidomide maintenance

VMAT for 4000 cGy in 20 fractions over 4 wk

In remission at 3 y

Cai et al 2021

63

M

No

Left epistaxis—left maxillary sinus mass

0

Bortezomib, cyclophosphamide, epirubicin, vindesine, prednisolone (V-CDOP) x5 followed by autologous hematopoietic stem cell transplantation and lenalidomide-based maintenance therapy

Local radiotherapy to nasopharyngeal regions 30 Gy in 15 fractions over 3 wk

Progression-free survival at 22 mo

Castillo et al 2021

79

M

No

Left cheek bulging and facial numbness—left maxillary sinus mass

I

Bortezomib, cyclophosphamide, doxorubicin, vincristine, prednisone (V-CHOP) x4/rapid progression: daratumumab, lenalidomide, and dexamethasone x2 cycles/persistent disease: IV pembrolizumab x4 cycles—started radiation, continued pembro with radiation, stopped at 18 cycles

Post-pembro: adjuvant radiotherapy 30 Gy

Complete response 43 mo after diagnosis (22 mo after last dose of pembro)

Lasrado et al 2021

53

M

No

Right NAO with epistaxis—right maxillary sinus mass

Diagnostic nasal endoscopy and biopsy

Chemotherapy

Mariani et al 2021

17

M

No

Large lobulated mass extending from right nasal septum into oral cavity, 2nd mass in anterior left nasal sidewall—collision tumor (right tumor) comprising both plasmacytoma and nasopharyngeal angiofibroma, also with L2 spine lesion PBL

Excision of both nasal masses

Proton radiotherapy for L2 lesion

Modi et al 2021

6

M

HIV+

Gum hypertrophy, breathlessness, abdominal distension, found to have soft tissue swelling of right obit, right nasal cavity, hard palate, and maxillary alveolus

Chemotherapy

Currently on follow-up

Ando et al 2020

64

M

HIV+

Nasal bleeding—bilateral nasal cavity lesion

EPOCH, salvage ifosfamide, carboplatin, etoposide (ICD), followed by bortezomib, cyclophosphamide, dexamethasone (CyBorD) x2, changed to lenalidomide and dexamethasone due to peripheral neuropathy for 2 y

Partial response at 2 y

Furlan et al 2020

78

F

No

Maxillary sinus

R-CHOP x6 cycles

Asymptomatic at 6 mo

Li et al 2020

42

M

No

Nasopharynx

II

Gemcitabine + oxaliplatin (GEMOX)

Involved-field radiotherapy

Alive and complete remission at 10 mo

Li et al 2020

69

M

No

Nasal cavity

I

CHOP

No

Alive and complete remission at 79 mo

He et al 2020

69

F

No

Left NAO due to nasal cavity mass

Chemotherapy

Radiation

Alive at 18 mo

He et al 2020

26

M

No

Dry eyes—inferior nasal concha, maxillary sinusitis

Radiation

Alive at 71 mo

He et al 2020

64

M

No

Mild rhinorrhagia—mass in left nasal cavity

Extensive resection of nasal neoplasm

Alive at 2 mo

Mai et al 2020

57

M

HIV+

Sinonasal mass

EPOCH

Alive at 34 mo

Mai et al 2020

47

M

HIV+

Nasal mass, maxillary mass

Hyper-CVAD

Alive at 60 mo

Miao et al 2020

64

M

HIV negative

Nasal cavity

II

CHOP

Miao et al 2020

66

M

HIV negative

Nasopharynx

I

CHOP

Shakri et al 2020

56

F

No

Right-sided epistaxis requiring transfusion

Endoscopic exam under anesthesia, biopsy, tumor debridement

6 cycles of hyperfractionated CVAD

Disease-free 18 mo posttreatment (including 2 PET/CT's)

Shwe et al 2020

33

M

No

Intermittent epistaxis with prolonged bleeding, left nasal cavity mass with post-chemotherapy cutaneous involvement

DA-EPOCH with intrathecal methotrexate; after cutaneous involvement: weekly bortezomib

Yap et al 2020

91

M

No

Posterior nasal space

IE

Radiation only

Complete response at 37.5 mo

Perkins et al 2019

–-

M

No

12 mo of left nasal congestion—solitary polypoid left nasal septal mass

I

En bloc removal from the nasal septum

Etoposide, vincristine, doxorubicin, cyclophosphamide, prednisone (EPOCH) x1 cycle and rituximab, switched (due to neutropenic fevers) to CVAD, and rituximab

Rodrigeus et al 2019

37

M

HIV+

Left cheek swelling—left maxillary sinus mass

EPOCH (etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin) x6 cycles

Undergoing chemo still at time of writing

Gibbs et al 2017

76

M

No

(History of T2N2bM0 left hypopharyngeal squamous cell carcinoma), epistaxis—R maxillary sinus mass

Definitive radiation (180-4500 cGy) to R sinus

No recurrence 2 y out

Rafei et al 2017

49

M

HIV+

Nasopharyngeal mass

DA-EPOCH with intrathecal MTX, hydrocortisone, and liposomal cytarabine via lumbar puncture and ommaya reservoir, chemo x6 cycles

Palliative radiation up to 1,500 cGy, interrupted by DA-EPOCH

Free of disease for 12 y

Wang et al 2017

53

M

HIV+

Epistaxis, left maxillary sinus mass

Hyper-CVAD

Lost to follow-up

Yan et al 2017

27

M

No

Nasal obstruction, epistaxis—nasal cavity tumor

Resection

Radiation

Alive and no evidence of disease at 70 mo

Koizumi et al 2016

51

M

HIV+

Sinus

EPOCH + dexamethasone, high-dose cytarabine, cisplatin (RT DHAP)

Radiation

Died of disease at 4.3 mo

Koizumi et al 2016

52

M

HIV+

Sinus

Best supportive care

Died of disease at 2.8 mo

Koizumi et al 2016

39

M

HIV+

Sinus

CHOP

Alive at 26.9 mo, complete response

Koizumi et al 2016

43

M

HIV+

Sinus, bone

CHOP

Died of disease at 13.3 mo

Pinnix 2016

Right maxillary sinus

R-CHOP x6 cycles

IMRT 39.6 Gy

NED

Pinnix 2016

Right nasal cavity, maxillary sinus

Hyper-CVAD x4 cycles

IMRT at 41.4 Gy

NED

Pinnix 2016

Right nasal cavity

Hyper-CVAD x4 cycles

IMRT at 39.6 Gy

NED

Pinnix 2016

Left ethmoid sinus

DA-EPOCH x6 cycles

Volumetric modulated arc therapy at 41.4 Gy

NED

Segal et al 2016

44

M

No

Nasopharyngeal branchial cleft cyst

Endoscopic resection of nasopharyngeal cyst

Conservative treatment by hematologist

No evidence of disease at 1.5 y

Elyamany et al 2015

59

F

No

Nasopharynx

IV

Cyclophosphamide, adriamycin, vincristine, prednisolone, rituximab (R-CHOP)

Radiotherapy

Died of disease at 12 mo

Loghavi et al 2015

26

F

No

Stuffy nose—nasal cavity tumor

Hyper-CVAD x3

Radiation

Alive with no e/o disease at 59.9 mo

Loghavi et al 2015

61

M

No

Epistaxis—right nasal septum

Radiation

Alive w/ no e/o disease at 44.8 mo

Sachsman et al 2015

66

Base of skull and paranasal sinuses

IV

CHOP x6 cycles + gitoxin, then intrathecal MTX

Consolidative proton treatment, 36 Gy (RBE) in 1.2 fx BID

Died of 2nd cancer at 53 mo

Ambrosio et al 2014

74

M

–-

Right maxillary sinus solitary mass infiltrating oral cavity

Surgical excision after chemoradiation

Bortezomib, melphalan, prednisone (VMP scheme)/relapse after CXRT: bortezomib, cyclophosphamide, dexamethasone with minimal response, switched to 3rd line therapy with lenalidomide, cyclophosphamide, and dexamethasone/after surgical resection liposomal doxorubicin and cytosine arabinoside, added bendamustine/continuous lenalidomide treatment with rituximab x3 and acyclovir

50 Gy radiotherapy

Morisco et al 2014

40

M

HIV+

Nasal

Alive at 191 mo

Morisco et al 2014

F

No

Nasal

Alive at 12.8 mo

Yasuhara et al 2014

50

F

No

Left maxillary sinus tumor with intraoral manifestation

II

CHOP x4

Complete remission at 9 mo after chemo

Pather et al 2013

14

M

HIV+

Left nasal, maxillary, and orbital mass

II

BFM '86 B NHL protocol

Death at 15 mo

Saraceni et al 2013

24

M

No

Chronic sinusitis for 2 mo, left face numbness, and asymmetry- involved maxillary sinus

IIE

6 cycles of hyperfractionated CVAD (odd cycles x3), alternating with high doses of methotrexate and cytosine arabinoside (even cycles ×3), intrathecal methotrexate, and cytosine arabinoside with each of the 6 cycles

Postchemo consolidation radiation by intensity-modulated radiation therapy (IMRT) for a total dose of 45 Gy

Alive at time of writing, complete remission of 4 y

Liu et al 2012

67

M

No

Nasal cavity

III

CHOP

partial response, alive at 26 mo

Liu et al 2012

64

M

No

Nasal cavity

I

CHOP

RT

complete response, alive at 55 mo

Liu et al 2012

86

M

No

Nasal cavity

IV

50% CHOP

died of unrelated disease at 1 month

Liu et al 2012

60

M

No

Nasal cavity

I

CHOP

under therapy, alive at 1 month

Liu et al 2012

50

M

No

Nasal cavity

II

Dexamethasone, etoposide, ifosfamide, carboplatin (DeVIC)

RT

complete response, alive at 12 mo

Liu et al 2012

45

M

No

Nasal cavity + bone + LN

IV

Cyclophosphamide, bortezomib, dexamethasone (CVD)

under therapy, alive at 2 mo

Liu et al 2011

60

F

No

Nasal cavity

IV

Hyper-CVAD x4

received radiation

died of disease at 36.5 mo

Liu et al 2011

66

M

No

Sinus

IE

CHOPx6

received radiation

alive and no evidence of disease at 23.9 mo

Liu et al 2011

59

M

No

Nasal cavity

IE

CHOPx6

received radiation

alive and no evidence of disease at 15.5 mo

Suzuki et al 2010

84

M

No

2 mo of left face pain, left maxillary tumor infiltrating left nasal cavity and upper oral cavity

IIA

involved-field radiation therapy of 30Gy/20 fractions and limited local field radiation of 20Gy/10 fractions (total 50 Gy)

new right chest wall tumor with lymphoma infiltration 3 mo after radiation

Kim et al 2009

72

F

No

Paranasal sinus

IA

Chemotherapy

alive at 6 mo

Morley et al 2009

49

M

HIV+

6-mo toothache, intermittent nasal discharge and facial swelling, left maxillary sinus mass with 2nd lesion in ipsilateral ethmoid air cells

CHOP protocol, repeat intrathecal chemotherapy (methotrexate, RSE, hydrocortisone); dexamethasone, cytarabine, cisplatin chemotherapy (DHAP), systemic melphalan and methylprednisolone; weekly prednisolone, mitoxantrone, cyclophosphamide, etoposide, bleomycin, vincristine chemo (PMitCEBO) for 3 wk

died within 3 mo of presentation

Ustun et al 2009

38

M

HIV+

Paranasal sinus mass

IVA

steroid

partial response, died on hospital day 12

Chetty et al 2003

23

F

HIV+

Anosmia and right NAO—maxillary sinus tumor

CHOP x2

alive at 7 mo

Reid-Nicholson et al 2008

40

M

HIV+

Sphenoid sinus

Died 12 d after admission from hematemesis complications

Dong et al 2005

44

M

HIV+

Nasopharynx (adenoid)

50% CHOP intrathecal methotrexate

died of disease at 1 month

Colomo et al 2004

65

M

HIV+

Maxillary sinus

IV

alive at 19 mo

Colomo et al 2004

35

M

HIV+

Maxillary sinus

IV

Alive with disease at 16 mo

Colomo et al 2004

30

M

Posttransplant

Maxillary sinus

IV

dead at 19 mo

Colomo et al 2004

35

M

HIV+

Maxillary sinus

Colomo et al 2004

86

F

No

Maxillary sinus

IV

dead at 4 mo

Schichman et al 2004

41

M

HIV+

Nasal fullness, left nasal polyp, and right frontal bone mass displacing frontal lobe treated with radiation, followed by testicular mass and left tibia mass treated with removal of testicular mass and radiation to leg followed by VAD x2 complicated by plasmacytoma in scapula treated with radiation complicated by left arm lesion

removal of testicular mass

After radiation to tibia: vincristine, dexamethasone, Adriamycin (VAD) x2/for left arm lesion, was placed on CHOP (vincristine, adriamycin, prednisone, cytoxan) and due to resistance started on EPOCH (etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin) and intrathecal MTX x6

1st treatment radiation: 30.4 Gy in 19 fractions to nose and frontal bone/tibia mass treated with 30 Gy in 15 fractions over 21 d/scapula treated with 30 Gy in 15 fractions over 28 d

Nguyen et al 2003

42

M

No

Rapid onset otalgia and hearing loss, nasal obstruction for 3 mo

IEA

3-mo courses of hyper-CVAD, intrathecal methotrexate with each chemo cycle

Postchemo consolidative locoregional radiation: 40 Gy to right nasal cavity, ethmoid, and maxillary sinuses and 36 Gy to bilateral superior cervical areas

No recurrence at 6 mo

Abbreviations: BFM, Berlin–Frankfurt–Munster protocol; CDOP, cyclophosphamide, epirubicin, vindesine, prednisolone; CEOP, cyclophosphamide, etoposide, oncovin/vincristine, and prednisone; CHOP, cyclophosphamide, doxorubicin, oncovin/vincristine, and prednisone; CVAD, cyclophosphamide, vincristine/oncovin, doxorubicin/adriamycin, and dexamethasone; CVD, cyclophosphamide, bortezomib, dexamethasone; DA-EPOCH, dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin; DeVIC, dexamethasone, etoposide, ifosfamide, carboplatin; EPOCH, etoposide, prednisone, oncovin/vincristine, cyclophosphamide, and doxorubicin; GEMOX, gemcitabine and oxaliplatin; IMRT, intensity-modulated radiation therapy; R-CHOP, rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone.


Note: “–" denotes no data reported.


[Table 2] shows the treatment regimens reported in the literature. A total of 84.4% (54/64) received chemotherapy, with 21 patients additionally receiving radiation. Ten (15.6%) underwent surgical resection. Of these 10 patients, two also received chemotherapy (3.1%), another two underwent radiation, and four (6.4%) underwent both chemotherapy and radiation. Five individuals (7.8%) received definitive radiation alone. The most common chemotherapy regimen ([Table 3]) was CHOP, which 37.7% of chemotherapy patients (20/53) received. The second most common chemotherapy regimen was cyclophosphamide, vincristine/oncovin, doxorubicin/adriamycin, and dexamethasone (CVAD), which was given to nine patients (17.0%). EPOCH was another common regimen, and 6 (11.3%) of patients received this. One patient spontaneously regressed without treatment and remained in remission 4 years later.

Table 2

Types of treatment for sinonasal plasmablastic lymphoma

Treatment regimen

Number of patients (%)

Surgical resection alone

2 (3.1%)

Surgery + chemotherapy

2 (3.1%)

Surgery + radiation

2 (3.1%)

Surgery + chemoradiation

4 (6.3%)

Chemotherapy alone

26 (40.6%)

Chemoradiation

21 (32.8%)

Radiation alone

5 (7.8%)

Supportive care

2 (3.1%)

Total

64 (100%)

Table 3

Specific chemotherapy regimens used to treat sinonasal plasmablastic lymphoma

Chemotherapy regimen

Number of patients (%)

CHOP

20 (37.7%)

CVAD

9 (17.0%)

EPOCH

6 (11.3%)

BFM

1 (1.9%)

DeVIC

1 (1.9%)

CVD

1 (1.9%)

GEMOX

1 (1.9%)

Unspecified regimen

4 (7.5%)

Multiple combinations of regimens

10 (18.9%)

Total

53 (100%)

Abbreviations: BFM, Berlin–Frankfurt–Munster protocol; CDOP, cyclophosphamide, epirubicin, vindesine, prednisolone; CEOP, cyclophosphamide, etoposide, oncovin/vincristine, and prednisone; CHOP, cyclophosphamide, doxorubicin, oncovin/vincristine, and prednisone; CVAD, cyclophosphamide, vincristine/oncovin, doxorubicin/adriamycin, and dexamethasone; CVD, cyclophosphamide, bortezomib, dexamethasone; DA-EPOCH, dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin; DeVIC, dexamethasone, etoposide, ifosfamide, carboplatin; EPOCH, etoposide, prednisone, oncovin/vincristine, cyclophosphamide, and doxorubicin; GEMOX, gemcitabine and oxaliplatin.


Of the 13 patients diagnosed with stage IV disease, only four were still alive at time of follow-up. Follow-up ranged from 12 days to 53 months. One patient spontaneously regressed without treatment. Overall, prognosis for all patients varied from death within 12 days (2 patients) to no evidence of disease at 15 years. Overall mortality rate by time of follow-up was 23.0% (14 of 61 patients with a reported follow-up status had expired at time of follow-up). Two of the 73 patients were lost to follow-up, and 10 other individual cases did not comment on follow-up status.


#

Discussion

PBL is an aggressive and rare form of lymphoma that most frequently presents in the oropharynx of individuals with HIV and EBV and is characterized by frequent relapse and poor prognosis. PBL is rarely described in the sinonasal region, and the literature is lacking. To our knowledge, this is the largest analysis of the literature and includes 73 cases of sinonasal PBL with individualized data.

As PBL is a rare disease, and even rarer in the sinonasal region, there is no widely accepted evidence-based treatment of PBL. From this systematic review, the most common treatment used for sinonasal PBL was chemotherapy, with the most common regimen being CHOP. This regimen has been documented as the most widely used treatment regimen for PBL regardless of site of involvement.[2] Castillo et al found that in HIV-positive patients undergoing CHOP, overall response rate was 77%, but overall survival was still low at 14 months, with the high mortality of PBL attributed to a high relapse rate, chemotherapy-resistant disease, and concomitant infections.[60] The NCCN therefore recommends a more intensive treatment approach, specifically suggesting DA-EPOCH, for AIDS-related B cell lymphomas according to their guidelines.[2] [13] However, as shown in previous studies by Castillo et al,[10] [60] and Dodero et al,[61] neither CHOP nor more intensive treatment like DA-EPOCH have been shown to definitively increase overall survival when compared with the other. Various clinical trials are being conducted to assess for treatment efficacy of large B-cell lymphomas such as PBL. Clinical trial NCT05389423 is studying DA-EPOCH with pomalidomide with or without rituximab for HIV-associated lymphomas, including PBL. The use of DA-EPOCH in combination with daratumumab, a monoclonal antibody that targets CD38-expressing cells, which are present in high levels in PBL, is being studied through clinical trial NCT04139304. Other clinical trials include NCT04676360 testing belantamab mafodotin in relapsed or refractory PBL, and NCT04915248 on the use of a combination of daratumumab, dexamethasone, and bortezomib for relapsed or refractory PBL. Results for these clinical trials are pending.

Immunocompromised status, including that measured by HIV status, affects PBL prognosis. Counterintuitively, despite HIV being highly associated with the development of PBL, the possible reversibility of immune system compromise through antiretroviral therapy (ART) may allow HIV-positive patients to have more favorable outcomes. Chemotherapy has been found to generate a better response in HIV-positive patients compared with HIV-negative patients, possibly attributed to the effect of ART on strengthening the immune system.[11] Indeed, having a higher CD4 count is a favorable prognostic factor in PBL,[40] whereas immunosuppression is a negative prognostic factor in HIV-negative individuals.[50] Better survival in EBV-positive and HIV-positive patients has been noted compared with EBV-negative and HIV-negative patients, possibly attributed to a similar mechanism in which initiation of ART allows better virological control.[7] However, this is controversial as Florindez et al[62] concluded that HIV status had no effect on survival in PBL. Morscio et al[7] found no significant difference in the incidence of PBL before and after the initiation of ART, implying that HIV, as a cause of immunosuppression, is still implicated in the development of PBL.

Regardless of HIV status, prognosis of PBL remains poor with a median overall survival < 12 months, and those with HIV initially present with more advanced disease.[2] [4] In our study, almost half of sinonasal PBL patients presented with advanced stage disease, which is slightly lower than but still on par with that seen in the literature,[10] [11] [62] and remains consistent with aggressive disease. In patients with limited-stage (stage I or II) PBL in the head and neck region, the use of involved site radiotherapy may impart a survival benefit.[21] [41] However, despite the achievement of complete remission with PBL treatment, most patients experience relapse of disease with a median progression-free survival period of 6 months.[12]

A limitation of this study stems from its retrospective nature, the limited number of sinonasal region cases of PBL in the literature, and the variation in reporting of these individual cases. These factors collectively diminish the power of the study. Additionally, though 55 studies were identified that described sinonasal PBL, some abstracts could not be accessed and others did not differentiate individualized data from aggregate data, limiting the validity and generalizability of our findings. Furthermore, there are no published randomized controlled trials of treatment regimens for PBL, much less that specifically targeting the sinonasal region.


#

Implications for Practice

PBL is a rare malignancy in the sinonasal region associated with immunosuppression, marked by diverse treatment approaches and high mortality. In fact, the most common stage at presentation was stage IV, demonstrating its aggressive course and late diagnosis. This review reveals the most common treatments used, but no regimen has been found to best treat sinonasal PBL. Most cases are treated with chemotherapy alone, followed by chemoradiation. The NCCN recommends DA-EPOCH for AIDS-related B cell lymphomas. However, the most common chemotherapy regimen utilized in the literature for sinonasal PBL is consistent with the most common chemotherapy used in PBL: CHOP. The second most commonly prescribed chemotherapy regimen is CVAD. Additional research is needed to determine optimal treatment for sinonasal PBL, but the rarity of this condition poses a challenging undertaking for treatment and control of disease.


#
#

Conflict of Interest

None declared.

Authors' Contributions

S.C. contributed to study design, data acquisition and interpretation, and manuscript writing; H.C. was involved in data acquisition and interpretation as well as manuscript writing; S.S. participated in data acquisition and interpretation along with manuscript writing; and M.W. contributed to study design, data acquisition and interpretation, and manuscript writing.


Previous Presentation

This article was presented at the AAO-HNSF 2023 Annual Meeting & OTO Experience, Nashville, Tennessee, September 30–October 4, 2023.


  • References

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Address for correspondence

Sophia Chen, MD
Department of Head and Neck Surgery
UCLA Health
10833 Le Conte Ave. 62-132 CHS, Los Angeles, CA 90095-1624
United States   

Publikationsverlauf

Eingereicht: 01. September 2024

Angenommen: 08. Oktober 2024

Artikel online veröffentlicht:
13. November 2024

© 2024. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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  • References

  • 1 Delecluse HJ, Anagnostopoulos I, Dallenbach F. et al. Plasmablastic lymphomas of the oral cavity: a new entity associated with the human immunodeficiency virus infection. Blood 1997; 89 (04) 1413-1420
  • 2 Bailly J, Jenkins N, Chetty D, Mohamed Z, Verburgh ER, Opie JJ. Plasmablastic lymphoma: An update. Int J Lab Hematol 2022; 44 (Suppl 1, Suppl 1): 54-63
  • 3 Colomo L, Loong F, Rives S. et al. Diffuse large B-cell lymphomas with plasmablastic differentiation represent a heterogeneous group of disease entities. Am J Surg Pathol 2004; 28 (06) 736-747
  • 4 Castillo JJ, Bibas M, Miranda RN. The biology and treatment of plasmablastic lymphoma. Blood 2015; 125 (15) 2323-2330
  • 5 Swerdlow SH, Campo E, Harris NL. et al. WHO classification of tumours of haematopoietic and lymphoid tissues. Vol 2: International agency for research on cancer Lyon, France; 2008
  • 6 Vaughan J, Perner Y, Mayne E, Wiggill T. Plasmablastic lymphoma in Johannesburg, South Africa, in the era of widescale antiretroviral therapy use. HIV Med 2021; 22 (03) 225-230
  • 7 Morscio J, Dierickx D, Nijs J. et al. Clinicopathologic comparison of plasmablastic lymphoma in HIV-positive, immunocompetent, and posttransplant patients: single-center series of 25 cases and meta-analysis of 277 reported cases. Am J Surg Pathol 2014; 38 (07) 875-886
  • 8 Pather S, MacKinnon D, Padayachee RS. Plasmablastic lymphoma in pediatric patients: clinicopathologic study of three cases. Ann Diagn Pathol 2013; 17 (01) 80-84
  • 9 Goedhals J, Stones DK, Botha MC. Plasmablastic lymphoma in childhood: a report of two cases. SAJCH 2014; 8 (01) 39-40
  • 10 Castillo JJ, Furman M, Beltrán BE. et al. Human immunodeficiency virus-associated plasmablastic lymphoma: poor prognosis in the era of highly active antiretroviral therapy. Cancer 2012; 118 (21) 5270-5277
  • 11 Castillo JJ, Winer ES, Stachurski D. et al. Clinical and pathological differences between human immunodeficiency virus-positive and human immunodeficiency virus-negative patients with plasmablastic lymphoma. Leuk Lymphoma 2010; 51 (11) 2047-2053
  • 12 Al-Malki MM, Castillo JJ, Sloan JM, Re A. Hematopoietic cell transplantation for plasmablastic lymphoma: a review. Biol Blood Marrow Transplant 2014; 20 (12) 1877-1884
  • 13 NCCN. AIDS-Related B-Cell Lymphoma. 2021; National Comprehensive Cancer Network (NCCN) Guidelines. Accessed at: https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1480 2024
  • 14 Makady NF, Ramzy D, Ghaly R, Abdel-Malek RR, Shohdy KS. The emerging treatment options of plasmablastic lymphoma: analysis of 173 individual patient outcomes. Clin Lymphoma Myeloma Leuk 2021; 21 (03) e255-e263
  • 15 Chicuellar NR, Sufyan W, Mahendran S. Unilateral maxillary sinus plasmablastic lymphoma in an immunocompetent patient. An unusual occurrence report and literature review. Ear Nose Throat J 2022; 101 (06) NP251-NP255
  • 16 DiBlasi M, Jayne C, McNamara R, Iasiello C, Colden D. Sinonasal plasmablastic lymphoma arising in the setting of recurrent nasal polyposis in an immunocompetent individual. Ear Nose Throat J 2024; 103 (09) 538-542
  • 17 Lee KT, Misron NA, Abdul Aziz N, Wong CH, Liew HK. Spontaneous regression of plasmablastic lymphoma in an immunocompetent patient: case report and review of the literature. Case Rep Hematol 2022; 2022: 1142049
  • 18 Rapiti N, Peer N, Abdelatif N, Rapiti P, Moosa Y. HIV-associated plasmablastic lymphoma: a single-centre 12-year experience in Kwa-Zulu Natal, South Africa. HIV Med 2022; 23 (08) 837-848
  • 19 Sabry W, Wu Y, Kodad SG. Bortezomib, lenalidomide and dexamethasone combination induced complete remission in relapsed/refractory plasmablastic lymphoma: case report of a potential novel treatment approach. Curr Oncol 2022; 29 (07) 5042-5053
  • 20 Cai J, Qiu L, Ma L, Zhang N, Fan FY. Case report: bortezomib plus CDOP followed by sequential autologous hematopoietic stem cell transplantation and lenalidomide-based maintenance therapy in plasmablastic lymphoma. Front Med (Lausanne) 2021; 8: 749863
  • 21 Castillo JJ, Lamacchia J, Silver J, Flynn CA, Sarosiek S. Complete response to pembrolizumab and radiation in a patient with HIV-negative, EBV-positive plasmablastic lymphoma. Am J Hematol 2021; 96 (10) E390-E392
  • 22 Lasrado S, Naaz S, Moras K, D'Souza C, Loka SR. Analysis of disease patterns in patients with unilateral sinonasal diseases: a tertiary care hospital experience. Indian J Otolaryngol Head Neck Surg 2022; 74 (01) 63-69
  • 23 Mariani R, King RL, Liu H. EBV-positive plasmacytomas involving a nasopharyngeal angiofibroma in an adolescent. Pediatr Dev Pathol 2021; 24 (03) 264-268
  • 24 Modi N, Khurana U, Mukhopadhyay S. et al. An unusual case of paediatric plasmablastic lymphoma presenting with malignant effusion and the challenges in its diagnosis. Diagn Cytopathol 2021; 49 (10) E389-E394
  • 25 Ando K, Imaizumi Y, Kobayashi Y. et al. Bortezomib- and lenalidomide-based treatment of refractory plasmablastic lymphoma. Oncol Res Treat 2020; 43 (03) 112-116
  • 26 Furlan K, Miller I, Mahon B, Ocampo Gonzalez FA, Ward N. Plasmablastic lymphoma associated with adjacent mature plasma cell population exhibiting opposite light chain restriction. Case Rep Pathol 2020; 2020: 8875547
  • 27 Li YJ, Li JW, Chen KL. et al. HIV-negative plasmablastic lymphoma: report of 8 cases and a comprehensive review of 394 published cases. Blood Res 2020; 55 (01) 49-56
  • 28 He L, Li Z, Fan X, Shi Q, Wu H, Chen J. Epstein-Barr virus-positive plasmacytoma in immunocompetent patients: a diagnostic dilemma. Int J Clin Exp Pathol 2020; 13 (03) 582-586
  • 29 Mai B, Wang W, Lin M. et al. HIV-associated plasmablastic lymphoma in the era of HAART: a single-center experience of 21 patients. AIDS 2020; 34 (12) 1735-1743
  • 30 Miao L, Guo N, Feng Y. et al. High incidence of MYC rearrangement in human immunodeficiency virus-positive plasmablastic lymphoma. Histopathology 2020; 76 (02) 201-211
  • 31 Shakri NM, Husain S, Zahedi FD, Tan GC. Nasal plasmablastic lymphoma in an HIV-negative immunocompetent patient. Medeniyet Med J 2020; 35 (01) 71-74
  • 32 Shwe S, Sharma AA, Lee BA, Smith JE. Relapse of plasmablastic lymphoma with cutaneous involvement in an immunocompetent male. Ear Nose Throat J 2022; 101 (05) NP222-NP225
  • 33 Yap DRY, Tan GF, Chang EWY. et al. Clinical features of plasmablastic lymphoma: case series from an Asian tertiary cancer center and literature review. J Hematol (Brossard) 2020; 9 (03) 71-78
  • 34 Perkins JN, Chi AW, Patel NJ. Plasmablastic lymphoma of the nasal septum. JAMA Otolaryngol Head Neck Surg 2019; 145 (09) 868-869
  • 35 Rodrigues AI, Cabeçadas J, Martins Fernandes R, Viana Coelho M, Sousa C. Plasmablastic lymphoma of the maxillary sinus. Intern Emerg Med 2019; 14 (08) 1337-1338
  • 36 Gibbs JD, Leon ME, Liu K, Nguyen J, Zhang L. A rare case of Epstein-Barr virus-related plasmacytoma involving maxillary sinus mucosa. Clin Case Rep 2017; 5 (09) 1482-1485
  • 37 Rafei H, El-Bahesh E, Finianos A, Liu ML, Schechter GP. Plasmablastic lymphoma: case report of prolonged survival of an advanced human immunodeficiency patient and literature review. Case Rep Hematol 2017; 2017: 9561013
  • 38 Wang D, Zheng Y, Zeng D. et al. Clinicopathologic characteristics of HIV/AIDS-related plasmablastic lymphoma. Int J STD AIDS 2017; 28 (04) 380-388
  • 39 Yan J, Wang J, Zhang W, Chen M, Chen J, Liu W. Solitary plasmacytoma associated with Epstein-Barr virus: a clinicopathologic, cytogenetic study and literature review. Ann Diagn Pathol 2017; 27: 1-6
  • 40 Koizumi Y, Uehira T, Ota Y. et al. Clinical and pathological aspects of human immunodeficiency virus-associated plasmablastic lymphoma: analysis of 24 cases. Int J Hematol 2016; 104 (06) 669-681
  • 41 Pinnix CC, Shah JJ, Chuang H. et al. Doxorubicin-based chemotherapy and radiation therapy produces favorable outcomes in limited-stage plasmablastic lymphoma: a single-institution review. Clin Lymphoma Myeloma Leuk 2016; 16 (03) 122-128
  • 42 Segal N, Schneider S, Parra AS, Benharroch D. Case report: lymphoma in a nasopharyngeal branchial cleft cyst. B-ENT 2016; 12 (01) 67-71
  • 43 Elyamany G, Alzahrani AM, Aljuboury M. et al. Clinicopathologic features of plasmablastic lymphoma: single-center series of 8 cases from Saudi Arabia. Diagn Pathol 2015; 10: 78
  • 44 Loghavi S, Khoury JD, Medeiros LJ. Epstein-Barr virus-positive plasmacytoma in immunocompetent patients. Histopathology 2015; 67 (02) 225-234
  • 45 Sachsman S, Flampouri S, Li Z, Lynch J, Mendenhall NP, Hoppe BS. Proton therapy in the management of non-Hodgkin lymphoma. Leuk Lymphoma 2015; 56 (09) 2608-2612
  • 46 Ambrosio MR, De Falco G, Gozzetti A. et al. Plasmablastic transformation of a pre-existing plasmacytoma: a possible role for reactivation of Epstein Barr virus infection. Haematologica 2014; 99 (11) e235-e237
  • 47 Yasuhara R, Irié T, Shiozawa E. et al. Plasmablastic lymphoma of the maxillary sinus with intraoral manifestation caused by direct alveolar bone infiltration in an HIV-negative patient. Pathol Int 2014; 64 (11) 588-590
  • 48 Saraceni C, Agostino N, Cornfield DB, Gupta R. Plasmablastic lymphoma of the maxillary sinus in an HIV-negative patient: a case report and literature review. Springerplus 2013; 2 (01) 142
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Fig. 1 Preferred reporting items for systematic reviews and meta-analyses showing article selection process.