Vol 18, No 5 (2022)
Review paper
Published online: 2022-08-12

open access

Page views 3981
Article views/downloads 375
Get Citation

Connect on Social Media

Connect on Social Media

Primary dural lymphoma: a comprehensive literature review and report of a case

Arash Fattahi1, Alireza Sadeghipour2, Nasrin Shayanfar2, Masoumeh Najafi3, Moein Ravansalar1, Ali Shahhoseini1, Hooman Koohestani1, Parisa Javadnia1
Oncol Clin Pract 2022;18(5):335-348.

Abstract

Primary dural lymphoma (PDL) is a subtype of primary central nervous system (CNS) lymphoma (PCNSL) with only an extra-axial dural location. It accounts for less than 1% of all CNS lymphomas. PDL is a sporadic CNS tumor, and in the preoperative period, because of imaging characteristics, it is usually mimicking a meningioma. Usually, PDL is a low-grade B-cell lymphoma with a relatively good response to surgical resection with or without radiotherapy. Here we reviewed 102 case reports of PDL in the literature. Then, we present the case of our patient with PDL and explain the complexity of our treatment approach.

Review article

Oncology in Clinical Practice

DOI: 10.5603/OCP.2022.0020

Copyright © 2022 Via Medica

ISSN 2450–1654

e-ISSN 2450–6478

Primary dural lymphoma: a comprehensive literature review and report of a case

Arash Fattahi1Alireza Sadeghipour2Nasrin Shayanfar2Masoumeh Najafi3Moein Ravansalar1Ali Shahhoseini1Hooman Koohestani1Parisa Javadnia1
1Department of Neurosurgery, Iran University of Medical Sciences, Tehran, Iran
2Department of Pathology, Oncopathology Research Center, Iran University of Medical Sciences, Tehran, Iran
3Skull Base Research Center, Iran University of Medical Sciences, Tehran, Iran

Address for correspondence:

Parisa Javadnia, MD

Department of Neurosurgery, Rasool

Akram Hospital, Tehran, Iran

e-mail: parisa.javadnia@gmail.com

Received: 14.04.2022 Accepted: 6.06.2022 Early publication date: 12.08.2022

ABSTRACT

Primary dural lymphoma (PDL) is a subtype of primary central nervous system (CNS) lymphoma (PCNSL) with only an extra-axial dural location. It accounts for less than 1% of all CNS lymphomas. PDL is a sporadic CNS tumor, and in the preoperative period, because of imaging characteristics, it is usually mimicking a meningioma. Usually, PDL is a low-grade B-cell lymphoma with a relatively good response to surgical resection with or without radiotherapy. Here we reviewed 102 case reports of PDL in the literature. Then, we present the case of our patient with PDL and explain the complexity of our treatment approach.

Key words: primary dural lymphoma, review, meningioma, CNS

Oncol Clin Pract 2022; 18, 5: 335348

Introduction

Primary central nervous system (CNS) lymphoma (PCNSL) is an extranodal non-Hodgkin lymphoma of the CNS without systemic involvement [1]. Primary dural lymphoma (PDL) is a subtype of PCNSL with only extra-axial dural location; it accounts for less than 1% of all CNS lymphomas [2].

PDL is a sporadic CNS tumor, and in the preoperative period, because of imaging characteristics, it is usually mimicking a meningioma [3]. On the one hand, meningioma is the most common primary brain tumor, and on the other hand, a dural-based PCNSL is a rare intracranial tumor. Misdiagnosis could be a prevalent problem in treating patients with PDL [4, 5].

Nowadays, the mainstay of treatment for patients with PCNSL is gaining a tissue diagnosis (e.g., stereotactic biopsy), followed by high-dose methotrexate (MTX) induction chemotherapy and then consolidation therapy (e.g., whole-brain radiotherapy, WBRT) to treat the residual tumor and improve overall survival [6]. Usually, PDL is a low-grade marginal zone B-cell lymphoma (MZL) with a relatively good response to surgical resection with or without radiotherapy [7].

Here we present our patient with PDL, explain the complexity of our treatment approach, and review all reported PDL cases in the literature.

Methods

We obtained and reviewed data related to all reported cases of PDL in the literature, retrospectively. We did not exclude any case reports. All reported cases, even those with limited data, were included, but we reported missing data as not available (N/A). We found 35 case reports and case series in the literature that reported on patients with PDL. Also, we reported our patient with PDL to explain the possible complexity in facing a patient with PDL.

Results

We reviewed overall 30 men and 71 women with PDL aged 1985 years (mean 53.85 years old). Detailed data of all patients are included in Table 1. Convexity (61%), cavernous (7.9%), and tentorial (7.9%) were the most common sites of PDL, followed by other sites (7.9%) and the falcine (6.9%). Ninety percent of all reported cases had a single lesion. Considering the histologic type of reported PDL, the most common types were marginal zone lymphoma (62.3%), diffuse large B-cell lymphoma (23.7%), B-cell lymphoma (not determined subgroup) (6.9%), and Follicular cell lymphoma (3.9%). The most common signs and symptoms in patients were headache (33.6%), followed by seizure (27.7%), cranial nerve deficits (18.8%), and visual deficit (16.8%). The most common treatment approach in the literature was surgical resection and radiotherapy (31.6%), followed by surgical resection and chemotherapy (16.8%). All detailed results of this review can be seen in Table 2.

Table 1. Results of primary dural lymphoma literature review

Age (year)/ /gender

Symptoms at onset

Lesion location

Number of lesions

Systemic

Pathology

Treatment

Follow up

de la Fuente et al., 2017

47M

Generalized tonic-clonic seizure

Lt tentorium

1

One had CSF+ and one patient had para-aortic lymphadenopathy and bilateral lung nodules

MZL

STR + Focal RT

12.1y

66M

Seizures, progressive gait disorder

Left fronto-parietal

1

MZL

Biopsy + chemotherapy

1.1y

41F

Headache, focal seizures, Rt visual field cut

Lt parieto-occipital

1

MZL

GTR + Focal RT

7.2y

51F

long h/o headache with new worsening

Lt frontal

1

MZL

STR + WBRT + Focal RT

11.3y

49F

Focal Lt face numbness and paresthesia Lt ear tinnitus and otalgia

Lt tentorium (compressing L brainstem and cerebellar hemisphere)

1

MZL

STR + Focal RT

6.8y

69F

Walking difficulty

Lt temporo-parietal convexity

1

MZL

Biopsy + chemotherapy

8.6y

51F

Seizure, Lt homonymous hemianopsia

Rt occipital

1

MZL

Biopsy + WBRT + Focal RT

5.7 y

59M

Headache

Rt temporal, R frontal

2

MZL

GTR of both lesions + NA

NA

49F

Focal seizures

Rt fronto-parietal

1

MZL

STR + WBRT + Focal RT

4.6y

72F

Generalized tonic-clonic seizure

Long lesion extending along the falx cerebri

1

MZL

STR + NA

NA

33F

Generalized tonic-clonic seizure

Lt frontal and Lt parietal

2

MZL

STR + WBRT + Focal RT

0.9y

Headache, L facial weakness

Rt temporo-parietal

1

MZL

Partial resection + WBRT + Chemotherapy

17.2y

39F

Vision loss, focal R-sided paresthesia

Lt frontal, Rt sphenoid region/orbital apex

2

MZL

STR of sphenoid lesion only + WBRT + Focal RT

9m

49F

Headache, seizures, visual loss

Rt temporal, Rt frontal

2

MZL

Biopsy + WBRT

3.1y

39F

Headache

Rt frontal

1

MZL

STR + Focal RT

1.3y

30F

Facial pain

Cavernous sinus

1

MZL

STR + Focal RT

9y

67F

Headache

Rt occipital

1

MZL

STR + NA

NA

47M

Seizures

Lt frontal

1

MZL

Biopsy + Focal RT

5.6y

34M

Seizures

Rt temporal

1

MZL

GTR + Focal RT

5.3y

67M

Seizures

Rt frontal

1

MZL

Biopsy + focal RT

4.7y

51F

Focal paresthesia and numbness

Lt cavernous sinus

1

MZL

Biopsy + focal RT + Chemotherapy

3y

57M

Seizures, headache

Rt temporal

1

MZL

GTR + Focal RT

2.5y

59F

Headache

Suprasellar region

1

MZL

Biopsy + Focal RT

1.9y

48F

Cranial nerve palsy

Cavernous sinus (bilateral)

M

MZL

Biopsy + Chemotherapy

10m

77F

Gait disturbance

Rt cerebellopontine angle

1

MZL

GTR + Focal RT

8m

50 F

Cranial nerve palsy

Cavernous sinus

1

MZL

STR + Focal RT

2m

Jazy et al., 1980

59M

three episodes of bizarre seizure activity, consisting of visual and auditory hallucination

Rt temporal convexity

1

DLBL

Resection + WBRT + Focal RT

16m

Scott, et al., 1990

21F

Seizure with

behavioral change and headache.

hydrocephalus, and infarction of

both dentate nuclei.

NA

CSF+

NA

Biopsy + IT chemotherapy + WBRT

15m

Kumar et al., 1997

40F

Focal numbness, visual field defects

Rt cavernous sinus

1

NA

MZL

RT

5.3y

62F

seizures

Biparietal dura

NA

NA

MZL

Chemotherapy

22m

52F

Seizures, focal numbness

Lt frontal dura

1

NA

MZL

RT + Chemotherapy + IT chemotherapy

7m

43F

Dizziness, headache. blurred vision, focal numbness

Lt tentorial

1

NA

MZL

RT

9m

57F

seizures

Lt anterior falx cerebri

1

NA

MZL

RT

14m

Kambham, et al., 1998

39F

Hearing loss, pain, weakness

Lt cerebellar pontine angle

1

NA

MZL

STR

4y

62F

Recent onset headache

Lt parieto-occipital area

1

NA

MZL

RT

6m

Hodgson, et al., 1999

57F

Headache

Rt sphenoid wing

1

FCL

Resection + WBRT + Focal RT

6m

Altundag, et al., 2000

66F

Syncope, seizure

Rt parietal

1

NA

MZL

Resection + RT

12m

Amaker, et al., 2000

49F

Intermittent headache, vomiting, decreased memory, apathy, and right-sided weakness

Lt frontal

1

T cell rich LBCL

Resection + chemotherapy

14m

Sanjeevi, et al., 2001

46F

Chronic headache, decreasing visual acuity

Lt cavernous sinus

1

MZL

STR + RT

15m

Estevez., et al., 2002

70F

bilateral temporal headache, decrease in Rt hearing and visual acuity

Parasagittal convexity

MZL

RT

1y

Goetz, et al., 2002

64F

Lt hemiparesis

Rt frontoparietal

1

-

MZL

STR + RT

3m

Lehman, et al., 2002

63F

Focal seizure, Rt trigeminal neuralgia

Falcotentorial

1

MZL

STR + RT

NA

Beriwal, et al., 2003

67F

Neck pain

Lt cerebellar hemisphere

1

FCL

Resection + RT

18m

Itoh et al., 2001

28F

Tinnitus, nausea,

headache, bilateral

papilledema

CP angel

1

MZL

GTR

2y

Rottneck et al., 2004

47M

Seizure, visual field defects, memory loss

Lt tentorial

1

MZL

STR + RT

8m

Abdullah, et al., 2005

33M

growing lump in the right frontal area

Rt frontal

1

Lymphoblastic BCL

Resection + RT + chemotherapy

30m

Kelley, et al., 2005

53M

persistent headaches and a generalized tonic-clonic seizure

atrium of the right lateral ventricle

1

MZL

Resection + IT chemotherapy

14m

Iwamoto, et al., 2006

64F

Headache, Lt facial weakness

Rt temporoparietal

1

CSF+

MZL

STR + IT chemotherapy + chemotherapy

6.9y

33F

Simple partial

seizures and

blurry vision

Lt temporal and Lt frontal

2

CSF+

MZL

Biopsy + WBRT + IT chemotherapy

7.5y

35M

Headache,

dizziness, focal paresthesia, and numbness

Lt tentorium, Lt frontoparietal

2

CSF+

MZL

Biopsy + WBRT + IT chemotherapy

4.9y

47M

Tonic-clonic seizure

Lt tentorium

1

MZL

STR + focal RT

2.5y

39F

Visual loss, focal paresthesia

Lt frontal, Rt sphenoidal

2

MZL

STR + WBRT+ Focal RT

1.6y

49F

Seizures and focal

sensory

symptoms

Rt parietal

1

CSF+

MZL

STR + WBRT+ Focal RT

0.9y

51F

headache

Bilateral frontal

2

MZL

Biopsy + WBRT

1.1y

50F

Headache,

seizures, visual loss

Rt frontal

1

MZL

WBRT

0.7y

Tu, et al., 2005

56F

NM

falx

1

MZL

Resection

NA

49M

Seizures

Lt frontal

1

MZL

Resection + chemotherapy + RT

7.6y

66M

Seizures

Rt frontal

1

MZL

Resection + RT

13m

29F

NM

subdural

1

MZL

Resection + RT

3y

61F

Headache, drowsiness, N/V

Rt frontotemporal

1

MZL

Resection + NM

21m

62F

Ataxia

Lt occipital

1

MZL

Resection + RT

25m

47M

Facial droop, numbness, dysarthria

Parietal

1

MZL

Resection + NM

NA

57F

Rt arm pain

Lt frontoparietal

1

MZL

Resection + chemotherapy

5.5y

70F

Visual deficit

Tentorium

1

MZL

Resection + RT

3.3y

59F

Unsteady gait, visual deficit

Falx

1

MZL

Resection + RT

2.8y

53F

Headache, visual deficit

Sella, Suprasella

1

MZL

Resection + RT

11m

48F

Headache, ear pain

Tentorium, falx

1

MZL

Resection + Chemotherapy + RT

20m

Brito et al., 2014

52F

right hemifacial paresthesia

Rt parieto occipital

1

DLBCL

Biopsy + chemotherapy

22m

Yamada et al., 2006

59F

severe frontal headaches

Bilateral frontal

1

DLBCL

Resection + chemotherapy

30m

Galarza et al., 2006

61M

generalized headache

vertex

1

DLBCL

Resection + chemotherapy+ WBRT

23m

Sacho et al., 2010

46F

sudden collapse, reduced level of consciousness, and focal seizures

Rt parietal subdural

1

DLBCL

Resection + chemotherapy

Dead

Said et al., 2011

42F

Rt hemiparesia, hemiparesthsia, retro orbital pain & headache

Lt vertex

1

DLBCL

Resection + chemotherapy

34m

Parekh, et al., 1993

65F

Scalp lump, Rt hemiparesia

Lt parietal

1

NHL

Resection + RT

6y

Landys, et al., 1995

62M

Headaches, malaise, unsteady gait

Frontoparietal

1

NHL

Resection + chemotherapy

5y

Paige & Bernstein, 1995

51M

Scalp mass, Headaches

Bilateral occipital

1

LBCL

Resection + RT + chemotherapy

NA

71M

enlarging

painless mass

Lt temporal

1

LBCL

Resection + RT + chemotherapy

NA

Curty, et al., 1997

19M

Scalp lump, Headaches

Rt parietal

1

CSF+

bone

BCL

Resection + chemotherapy

NA

Pardhanani, et al., 2000

77M

Ocular proptosis

Lt orbitofrontal

1

LBCL

Biopsy + RT

died

Karschnia et al., 2020

64F

Headaches [10 of 20 patients

(50%)], cranial nerve deficits [affecting cranial nerve II,

III, IV, or VI in most cases; 7 of 20 (35%)], limb weakness

or dysesthesias [7 of 20 (35%)], gait instability, or vertigo

[5 of 20 (25%)], word-finding difficulties or confusion

[5 of 20 (25%)], and painless growth of a subcutaneous mass

Bilateral frontal

1

Bone+

DLBCL

Biopsy + chemotherapy

0.2y

60F

Lt frontal

1

MZL

GKRS

3.7y

61F

Rt frontal

1

Bone+

DLBCL

GTR + chemotherapy

5.2y

73F

Rt tentorium & Rt hemisphere

1

CSF+

MZL

Biopsy + steroid

3.8y

48F

Sella turcica

1

MZL

Biopsy + Chemotherapy

2.1y

68M

Lt frontal

1

Bone+

DLBCL

Biopsy + Chemotherapy

0.1y

54F

Rt wall of cavernous sinus

1

CSF+

T cell NHL

Biopsy + chemotherapy

0.5

71F

Lt frontal

1

Bone+

FCL

GTR + chemotherapy

9.3

46F

Lt frontal, Lt parietal

2

Bone+

MZL

STR

0.1y

38F

Rt frontal

1

Bone+

CSF+

MZL

GTR + chemotherapy

15.7y

73F

Lt frontal

1

Bone+

DLBCL

Biopsy + chemotherapy

1.8y

75F

Rt wall of cavernous sinus

Bone+

CSF+

DLBCL

chemotherapy

1.6y

85M

Rt frontotemporal

1

Bone+

DLBCL

Biopsy + chemotherapy

0.5y

76M

Clivus

1

Bone+

DLBCL

Biopsy + chemotherapy

4.7y

55M

Rt sphenoid wing

1

Bone+

DLBCL

GTR + chemotherapy

4.6y

62M

Lt frontal

1

Bone+

DLBCL

GTR + chemotherapy

6.3y

57M

Lt middle cranial fossa

1

Bone+

CSF+

FCL

STR + chemotherapy

15.4y

Adbel Aziz & van Loveren, 1999

40F

Facial numbness & pain

Lt Meckel’s cave

1

NA

Unspecified BCL

Resection + chemotherapy + RT

NA

Saraceni et al., 2016

42M

Headache & lack of coordination

Rt parietal

1

Lymphoblastic BCL

Resection + chemotherapy + RT

5m

Raguz et al., 2018

34M

Intermittent headache

Rt frontal

1

BM+

CSF–

DLBCL

Resection + chemotherapy

4m

Kulkarni et al., 2012

39F

Painless progressive blurred vision

Rt optic canal extension to Rt cavernous

1

Low-grade BCL

Resection + RT

NA

Dobran et al., 2020

49M

Personality &mood change

Rt frontal

1

DLBCL

Resection +RT

3y

64F

Rt handed & lat hemianopsia

Lt occipitoparietal

1

DLBCL

Resection

8y

26F

Lt arm weakness

Rt frontoparietal

1

BCL

Resection

2y

Fattahi et al., 2022

56F

Acute loss of consciousness (LOC)

bilateral frontal parasagittal

1

BCL

Resection +RT

1y

Table 2. Summary of table one information

Age

Range

1985y

Mean

53.85y

Sex

Male

30 (29.7%)

Female

71 (70.3%)

Site of lesion

Convexity

62 (61%)

Cavernous

8 (7.9%)

Tentorial

8 (7.9%)

Falcine

7 (6.9%)

Sellar

3 (2.9%)

Cerebellopontine angle

3 (2.9%)

Sphenoid wing

2 (1.9%)

Other

8 (7.9%)

Number of lesions

Single

91 (90%)

Multiple

10 (10%)

Pathology

Marginal zone lymphoma

63 (62.3%)

Diffuse large B-cell lymphoma

24 (23.7%)

B-cell lymphoma (not determined subgroup)

7 (6.9%)

Follicular cell lymphoma

4 (3.9%)

Lymphoblastic cell lymphoma

1 (0.9%)

T cell rich B-cell lymphoma

1 (0.9%)

T cell lymphoma

1 (0.9%)

Clinical signs and symptoms

Headache

34 (33.6%)

Seizure

28 (27.7%)

Focal

23

Tonic-colonic

5

Cranial nerve deficit

19 (18.8%)

Visual deficit

17 (16.8%)

Focal neurological deficits

12 (11.8%)

Just motor

5

Just sensory

4

Sensory + motor

3

Neuropsychiatric problems

10 (9.9%)

Treatment approach

Surgical resection (S) alone

12 (11.8%)

Radiotherapy (R) alone

6 (5.9%)

Chemotherapy (C) alone

2 (1.9%)

Biopsy (B) alone

1 (0.9%)

S + R

32 (31.6%)

S + C

17 (16.8%)

B + C

10 (9.9%)

B + R

7 (6.9%)

R + C

1 (0.9%)

S + C + R

10 (9.9%)

B + R + C

2 (1.9%)

Corticosteroid alone

1 (0.9%)

An illustrative case

A 56-year-old female was admitted to the emergency department with acute loss of consciousness (LOC). On neurological examination, she had a Glasgow coma scale (GCS) score of 12 with bilateral reactive pupils. She had no neurological deficit. A computed tomographic (CT) scan of the brain revealed a bilateral frontal parasagittal mildly hyperdense extra-axial mass with extensive bi-frontal brain edema (Fig. 1A, B). On magnetic resonance imaging (MRI) of the brain and venography (MRV), the mass resembled a giant parasagittal meningioma with a completely occluded anterior superior sagittal venous sinuous (SSS) (Fig. 1CH); because of inappropriate bi-frontal edema in comparison to the lesion size, cortical vein thrombosis was considered as an important concomitant event, preoperatively. After a short course of treatment with intravenous (IV) anticoagulant and hyper-hydration, the patient was scheduled for surgical resection of the tumor.

In the operating room, the patient was placed in the supine position, and we approached her via bi-coronal incision and bi-frontal one-piece craniotomy. The tumor was an extra-axial parasagittal meningioma, which was resected gross totally. Adjacent anterior SSS, falx and dura were resected simultaneously. The dura was replaced with a pedunculated pericranial galeal patch. The adjacent parasagittal brain was very fragile and pale, but we could dissect it from the tumor well. After surgery, the patient felt good with no deficit and had GCS 13. On the first post-operation day, the patient acutely deteriorated, and with GCS 5 and right fixed mydriasis and severe bi-frontal malignant edema on a CT scan (Fig. 2B, C), she underwent an emergent decompressive craniectomy. Then, the patient felt good and was discharged with GCS 15 and no deficit on the 10th post-operation day. After one month, she was scheduled for bone flap replacement. The histopathologic exam revealed a low-grade B-cell lymphoma (not determined subgroup) (Fig. 3). Our radio-oncologist treated the patient as a PDL based on negative results for systematic involvement with lymphoma, i.e., negative chest and abdominopelvic CT scan (a course of focal radiotherapy). On one year follow-up, the patient felt good with no sign of recurrence on the following brain MRI (Fig. 2D, E).

Figure 1. Preoperative axial brain CT scan (A, B) revealed an extra-axial bi-frontal parasagittal mass with peritumoral edema with iso-signal on T1 and T2 sequence (C, D) of MRI. Also, the lesion had bright enhancement (EG) on MRI with gadolinium enhancement of the adjacent dura mater known as dural tail. On MRV, we can see the superior sagittal sinus completely occluded (H) in the location of the tumor
Figure 2. Postoperative brain CT scan (A) of the patient after 1 day revealed diffuse bi-frontal edema (B) which elevates the fixed bone flap (C). On one year follow-up, MRI with gadolinium (D, E) revealed no remnant or recurrence of the tumor
Figure 3. Histopathologic exam of the case. Diffuse meningeal infiltration by cellular sheets of lymphocytes, plasma cells, and lymphoplasmacytoid cells with a vague focal nodular growth pattern (A, H&E, ×100). A higher-power view (B) showed mainly small lymphocytes and plasma cells. Also, many small and intermediate-sized lymphoid cells are positive for CD20 (C. Immunohistochemistry, anti-CD20 antibody, ×200). We can see the predominant population of cells with lymphoplasmacytoid and plasma cell morphologies showing positive immunoreactivity for CD138 (D. Immunohistochemistry, anti-CD138 antibody, ×200). Image E shows many of the cells in the previous figure showing cytoplasmic immunoreactivity for Kappa light chain (E. Immunohistochemistry, anti-kappa anti-body, ×200). Fewer than 1% of the cells are immunoreactive for lambda light chain (F. Immunohistochemistry, anti-lambda antibody, ×200). Image G shows immunostaining for CD3 highlights scattered reactive T cells. (G. Immunohistochemistry, anti-CD3 anti- body, ×200). Also, we can see the low proliferation capacity of infiltrating lymphoid cells (H. Immunohistochemistry, MIB-1 antibody, ×200)

Discussion

PDLs have distinct clinical-pathological entities that separate them from intraparenchymal lymphoma. They are primarily low-grade and have a favorable prognosis. They include marginal zone lymphoma (MZL), small cell lymphoma, diffuse large B-cell lymphoma (DLBCL), and lymphoblastic and follicular cell lymphoma, in order of prevalence [8].

The pathogenesis of PDL is not well understood due to the lack of lymphoid tissue in the dura. There are 2 hypotheses for their development. One is the presence of meningothelial cells throughout the arachnoid membrane, and the other is chronic environmental antigenic stimulation, and resultant inflammatory conditions, which could precede the malignant transformation of lymphoid cells [9–11].

Meningothelial cells are analogous to epithelioid cells at other sites where MZL arises. These cells are present in the arachnoid membrane but are concentrated in the arachnoid villi with dural venous sinuses. Interestingly, most case reports localized MZL lymphoma in these regions [12–14].

However, a previous history of primary autoimmune disease, meningeal infiltration, or infection was primarily absent in our literature review. A limited number of case reports with underlying autoimmune diseases were reported, including Hashimoto thyroiditis, Grave’s disease, Sjogren’s syndrome, systemic lupus erythematosus (SLE), and relapsing-remitting multiple sclerosis (MS) [15–20].

Clinical presentation and diagnostic findings

PDL, especially MZL, most of the time presents as a single mass lesion without systemic involvement and has insidious long-lasting symptoms; there were only 7 case reports with multiple lesions which originated from the convexity [11].

The most frequent complaints among the patients with PDL were headaches and seizures. Other accompanying symptoms of PDL originated from convexity. Frontal, parietal, temporal, and occipital were relevant neurological deficits according to their site of origin, including focal sensory, motor deficit, visual disturbance, and ataxia, respectively. Case reports with PDL originating from the skull base, including the sella and parasellar region (3 cases), cavernous sinus (8 cases), cerebellopontine angle (3 cases), and Meckel’s cave (1 case), were rare and their symptoms were determined by involved surrounding cranial nerves. The most pathologic subtype of skull base PDLs was peculiarly diffuse large B-cell lymphoma (DLBL) [1, 9, 15, 17, 21, 22]. There is just 1 case report of PDL, which originates from the atrium of the lateral ventricle; its clinical presentation was headache and generalized seizure, and its subtype was MZL [16].

In many earlier reports, PDLs were non-tender, not pulsatile, subcutaneous mass with a permeation pattern of growth. However, this feature (being a non-tender, non-pulsatile subcutaneous mass) is still an accompanying symptom. There were some case reports with less common clinical presentations; 2 cases primarily present ocular symptoms. One of them presented with progressive proptosis, which originates from the orbitofrontal lobe, and the other patient had a unilateral painless progressive blurred vision his lesion had originated from the optic canal and extended toward the cavernous sinus [1, 23, 24]. There were also case reports of PDL with psychiatric manifestations such as bizarre behavior, hallucination, and unsteady mood [2, 25].

PDL is often initially interpreted as meningioma due to their clinical and radiographic features having a lot in common. Both appear as an iso-hypo intensity signal mass on T1 weighted magnetic resonance (MR) images with homogenous enhancement and hyperintensity signal mass on T2 weighted MR images. Dural tail sign, calvarial hyperostosis, or infiltration, en plaque thickening of the sphenoid bone, bone erosion or destruction, and invasion of the superior sagittal sinus are their common features [11, 26–28].

Few studies demonstrated in some detail images that were more favorable for PDL, such as calcification, the ratio of vasogenic edema to the tumor, the intensity of dural tail enhancement compared to lesion enhancement, fuzzy tumor-brain interface, and pattern of diffusion restriction. PDL in the apparent diffusion coefficient (ADC) map has a lower signal intensity than meningioma; however, the low signal intensity on the ADC map might also be observed in atypical and malignant meningioma. None of those mentioned above details was sufficient to verify a definite diagnosis [10, 21, 29].

There were 2 case reports in which PDLs were primarily misdiagnosed with acute subdural hematoma (SDH). In one of these reports, according to the preoperative image, a subdural hematoma was presumed, but during the operation, the suspicious diagnosis was substituted by meningioma. This patient had massive cerebral edema surrounding the lesion. The craniotomy and evacuation of the lesion were done. After 5 weeks, the patient returned with recurrent mass. Due to massive cerebral edema, the surgeons did not replace the bone flap, and despite all of their efforts to reduce intracranial pressure, the patient eventually died. Ultimately the histologic assessment revealed DLBCL [20]. The other case report with acute SDH had a mild presentation with no devastating manifestations; the pathology subtype in that patient was MZL [12].

Some case reports of MZL were misdiagnosed as pseudolymphomatous lesions, like plasma cell granuloma, pseudolymphoma, inflammatory pseudotumor, etc. Differentiation of lymphoma from inflammatory processes may be only possible after examining cytologic properties in the frozen section. However, Itoh and his colleagues demonstrated that the cluster of differentiate 20 (CD20) staining pattern is essential to confirm MZL diagnosis, and immunohistochemical assessment only is insufficient [22].

Neurological staging of PDL is essential to choosing a better treatment option, so all patients with presumed PDL should undergo an evaluation to exclude systemic involvement beyond the CNS. These evaluations include a lumbar puncture (seeking lymphoma cells in CSF), computed tomography of the chest, abdomen and pelvis, as well as a bone marrow biopsy. There were 16 case reports in which CSF was involved. The most popular pathology subtype in these studies was DLBCL [1, 11].

Therefore, when encountering a young patient with a short duration of neurological symptoms, rapid progression in symptoms, or systemic symptoms, caution should be taken in choosing conservative management with suspicion of meningioma diagnosis due to overlooking PDL, in which cases resection, in addition to adjuvant therapy, is necessary [10].

Many patients with PDL were reported to have chronic or acute neurologic symptoms before diagnosis [30]. In our case, the presentation of the tumor was entirely acute with LOC, and the patient had no symptoms before the day of admission. Generally, based on the location of the PDL, we could expect some previous neurological symptoms, but the presence or lack of these symptoms usually could not guide us to a preoperative diagnosis of PDL. Because of acute loss of consciousness and a cut-off point presentation in SSS on MRV, initially we suspected this extensive bi-frontal vasogenic edema is due to cortical vein thrombosis in the territory of the closed SSS, so she was treated by intravenous anticoagulant and hyper-hydration.

Treatment

According to previous reports, various therapeutic strategies were applied for PDL. The favorable clinical course of PDL is comparable with PCNSL, which had a better prognosis and less aggressiveness [31]. In PDL, compared to other PCNSL, the role of systemic chemotherapy in relapsing is unknown, so if leptomeningeal is involved, IT chemotherapy or WBRT should be added to surgery as adjuvant therapy because most of the time, due to infiltrative nature and relapse, gross total resection (GTR) is impossible [1, 2, 32].

The most popular treatment option for low-grade MZL with a single site of origin was resection combined with adjuvant therapy, chemotherapy, or radiotherapy. The dura mater is outside the blood brain barrier (BBB), so chemotherapy seems an excellent option to reach it without passing BBB11. DLBL had a poor prognosis in which 5-year survival after chemotherapy and radiotherapy was less than 10%. This poor prognosis may be due to a high proliferation fraction that causes rapid growth and recurrence. Treatment was unsatisfying with high doses of MTX either alone or with radiotherapy [20].

Like in our case, PDL represented a large frontal parasagittal mass in one study with occlusion of the anterior third of the superior sagittal sinus and severe cerebral edema. In that study, thallium accumulation in scintigraphy in both early and late phases determined tumor aggressiveness, and the bicoronal craniotomy was done with bone replacement and systemic chemotherapy, used due to their mentioned-above benefits connected with eradiation of the remnant (not passing BBB) [18].

Although most therapeutic strategies for MZL were effective in achieving complete remission during their clinical follow-up, in a study by de la Fuente and his colleagues, 4 patients had progression, 2 of whom had local recurrent tumors (at the resection site, just one of them received suboptimal focal radiotherapy) [21]. In another study by Iwamoto and his colleagues, in 3 of 8 patients, relapse occurred after 6 years. However, they did not confirm that relapse occurred from the exact clone as the PDL; however, histopathologically they were similar. One patient in their study developed treatment-induced leukoencephalopathy after high doses of MTX and WBRT, which seems to suggest that so chemotherapy may be unnecessary [8, 11].

Beriwal and his colleagues described a well-defined ovoid mass overlying the left cerebellopontine angle with follicular subtype. They treated their patient with radiotherapy after resection, which is the standard treatment for early-stage of follicular lymphoma in other sites [33].

One study reported a PDL with T cell rich B-cell lymphoma. This subtype of PDL appeared iso-intense in all MRI sequences, and angiography revealed prominent neovascularization so that the patient underwent obliteration of neovascular blush, and the following day embolization craniotomy was done [27].

They are limited reports of complications following surgery, such as hematoma, hygroma, infection, etc. There was just one study that reported wound infection at the site of craniotomy that responded well to antibiotic therapy without bone flap removal.

Considering the treatment approach to our patient, the patient was comatose with anisocoria, and we had to operate on her, on postoperative day 1, for decompressive craniectomy despite gross total resection of the large mass and expansile duraplasty with pedunculated patch and complete intravenous treatments for relieving brain edema. As a result, we think that in patients with extensive brain edema, suspected preoperative PDL, or an unusual meningioma diagnosis, it is better to not replace the bone flap at the time of surgical resection of the tumor, as it was done in former studies. Also, based on preoperative imaging, if histopathology shows it could be PDL, we could treat the patient with other options: biopsy followed by radiotherapy, etc. We recommend designating a study to reach the goal of PDL management without unwanted surgery-related complications.

Conflict of interest

All authors declare no conflicts of interest.

References

  1. Karschnia P, Batchelor TT, Jordan JT, et al. Primary dural lymphomas: clinical presentation, management, and outcome. Cancer. 2020; 126(12): 28112820, doi: 10.1002/cncr.32834, indexed in Pubmed: 32176324.
  2. Dobran M, Paracino R, Mancini F, et al. Primary dural lymphoma: clinical cases and literature review. Case Rep Med. 2020; 2020: 2812487, doi: 10.1155/2020/2812487, indexed in Pubmed: 32373176.
  3. Johnson MD, Kinney MC, Scheithauer BW, et al. Primary intracerebral Hodgkin’s disease mimicking meningioma: case report. Neurosurgery. 2000; 47(2): 454456; discussion 456-457, doi: 10.1097/00006123-200008000-00038, indexed in Pubmed: 10942021.
  4. Buetow MP, Buetow PC, Smirniotopoulos JG. Typical, atypical, and misleading features in meningioma. Radiographics. 1991; 11(6): 10871106, doi: 10.1148/radiographics.11.6.1749851, indexed in Pubmed: 1749851.
  5. Alessandro L, Pastor Rueda JM, Villalonga JF, et al. Retrospective study of 48 cases of primary central nervous system lymphoma. Medicina (B Aires). 2017; 77(1): 1723, indexed in Pubmed: 28140306.
  6. Liu Y, Yao Q, Zhang F. Diagnosis, prognosis and treatment of primary central nervous system lymphoma in the elderly population (Review). Int J Oncol. 2021; 58(3): 371387, doi: 10.3892/ijo.2021.5180, indexed in Pubmed: 33650642.
  7. Pavlou G, Pal D, Bucur S, et al. Intracranial non-Hodgkin’s MALT lymphoma mimicking a large convexity meningioma. Acta Neurochir (Wien). 2006; 148(7): 7913; discussion 793, doi: 10.1007/s00701-006-0761-1, indexed in Pubmed: 16570114.
  8. Iwamoto FM, DeAngelis LM, Abrey LE. Primary dural lymphomas: a clinicopathologic study of treatment and outcome in eight patients. Neurology. 2006; 66(11): 17631765, doi: 10.1212/01.wnl.0000218284.23872.eb, indexed in Pubmed: 16769960.
  9. Kumar S, Kumar D, Kaldjian EP, et al. Primary low-grade B-cell lymphoma of the dura: a mucosa associated lymphoid tissue-type lymphoma. Am J Surg Pathol. 1997; 21(1): 8187, doi: 10.1097/00000478-199701000-00009, indexed in Pubmed: 8990144.
  10. Brito AB, Reis F, de Souza CA, et al. Intracranial primary dural diffuse large B-cell lymphoma successfully treated with chemotherapy. Int J Clin Exp Med. 2014; 7(2): 456460, indexed in Pubmed: 24600506.
  11. Iwamoto FM, Abrey LE. Primary dural lymphomas: a review. Neurosurg Focus. 2006; 21(5): E5, doi: 10.3171/foc.2006.21.5.6, indexed in Pubmed: 17134121.
  12. Goetz P, Lafuente J, Revesz T, et al. Primary low-grade B-cell lymphoma of mucosa-associated lymphoid tissue of the dura mimicking the presentation of an acute subdural hematoma. Case report and review of the literature. J Neurosurg. 2002; 96(3): 611614, doi: 10.3171/jns.2002.96.3.0611, indexed in Pubmed: 11883850.
  13. Fuller GN, Burger PC. Central nervous system. In: Sternberg SS. ed. Histology for pathologists. LippincottRaven, New York 1997: 243284.
  14. Burger PC, Scheithauer BW, Vogel FS. Surgical Pathology of the Nervous System and Its Coverings, ed 3. Churchill Livingstone, New York 1991: 67142.
  15. Sanjeevi A, Krishnan J, Bailey PR, et al. Extranodal marginal zone B-cell lymphoma of malt type involving the cavernous sinus. Leuk Lymphoma. 2001; 42(5): 11331137, doi: 10.3109/10428190109097736, indexed in Pubmed: 11697633.
  16. Kelley TW, Prayson RA, Barnett GH, et al. Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue arising in the lateral ventricle. Leuk Lymphoma. 2005; 46(10): 14231427, doi: 10.1080/10428190500205895, indexed in Pubmed: 16194887.
  17. Tu PH, Giannini C, Judkins AR, et al. Clinicopathologic and genetic profile of intracranial marginal zone lymphoma: a primary low-grade CNS lymphoma that mimics meningioma. J Clin Oncol. 2005; 23(24): 57185727, doi: 10.1200/JCO.2005.17.624, indexed in Pubmed: 16009945.
  18. Yamada SM, Ikawa N, Toyonaga S, et al. Primary malignant B-cell-type dural lymphoma: Case report. Surg Neurol. 2006; 66(5): 539543; discussion 543, doi: 10.1016/j.surneu.2006.02.033, indexed in Pubmed: 17084207.
  19. Isaacson PG, Spencer J. Malignant lymphoma and autoimmune disease. Histopathology. 1993; 22(5): 509510, doi: 10.1111/j.1365-2559.1993.tb00169.x.
  20. Sacho RH, Kogels M, du Plessis D, et al. Primary diffuse large B-cell central nervous system lymphoma presenting as an acute space-occupying subdural mass. J Neurosurg. 2010; 113(2): 384387, doi: 10.3171/2010.2.JNS091554, indexed in Pubmed: 20225921.
  21. de la Fuente MI, Haggiagi A, Moul A, et al. Marginal zone dural lymphoma: the Memorial Sloan Kettering Cancer Center and University of Miami experiences. Leuk Lymphoma. 2017; 58(4): 882888, doi: 10.1080/10428194.2016.1218006, indexed in Pubmed: 27649904.
  22. Itoh T, Shimizu M, Kitami K, et al. Primary extranodal marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue type in the CNS. Neuropathology. 2001; 21(3): 174180, doi: 10.1046/j.1440-1789.2001.00392.x, indexed in Pubmed: 11666014.
  23. Parekh HC, Sharma RR, Keogh AJ, et al. Primary malignant non-Hodgkin’s lymphoma of cranial vault: a case report. Surg Neurol. 1993; 39(4): 286289, doi: 10.1016/0090-3019(93)90007-n, indexed in Pubmed: 8488447.
  24. Kulkarni KM, Sternau L, Dubovy SR, et al. Primary dural lymphoma masquerading as a meningioma. J Neuroophthalmol. 2012; 32(3): 240242, doi: 10.1097/WNO.0b013e31825103a5, indexed in Pubmed: 22573226.
  25. Jazy FK, Shehata WM, Tew JM, et al. Primary intracranial lymphoma of the dura. Arch Neurol. 1980; 37(8): 528529, doi: 10.1001/archneur.1980.00500570076016, indexed in Pubmed: 6893408.
  26. Lehman NL, Horoupian DS, Warnke RA, et al. Dural marginal zone lymphoma with massive amyloid deposition: rare low-grade primary central nervous system B-cell lymphoma. Case report. J Neurosurg. 2002; 96(2): 368372, doi: 10.3171/jns.2002.96.2.0368, indexed in Pubmed: 11838814.
  27. Rottnek M, Strauchen J, Moore F, et al. Primary dural mucosa-associated lymphoid tissue-type lymphoma: case report and review of the literature. J Neurooncol. 2004; 68(1): 1923, doi: 10.1023/b:neon.0000024704.70250.42, indexed in Pubmed: 15174517.
  28. Paige ML, Bernstein JR. Transcalvarial primary lymphoma of bone. A report of two cases. Neuroradiology. 1995; 37(6): 456458, doi: 10.1007/BF00600092, indexed in Pubmed: 7477857.
  29. Abdullah S, Morgensztern D, Rosado MF, et al. Primary lymphoblastic B-cell lymphoma of the cranial dura mater: a case report and review of the literature. Leuk Lymphoma. 2005; 46(11): 16511657, doi: 10.1080/10428190500215126, indexed in Pubmed: 16334908.
  30. Gordon AS, Fallon KE, Riley KO. Meningioma interdigitated with primary central nervous system B-cell lymphoma: A case report and literature review. Surg Neurol Int. 2011; 2: 181, doi: 10.4103/2152-7806.90716, indexed in Pubmed: 22276235.
  31. Estevez M, Chu C, Pless M. Small B-cell lymphoma presenting as diffuse dural thickening with cranial neuropathies. J Neurooncol. 2002; 59(3): 243247, doi: 10.1023/a:1019913611512, indexed in Pubmed: 12241122.
  32. Assaf C, Coupland S, Hummel M, et al. Relapse of primary extranodal marginal-zone B-cell lymphoma of the dura mater. The Lancet Oncology. 2005; 6(3): 187189, doi: 10.1016/s1470-2045(05)01771-7.
  33. Abdel Aziz KM, van Loveren HR. Primary lymphoma of Meckel’s cave mimicking trigeminal schwannoma: case report. Neurosurgery. 1999; 44(4): 859862; discussion 862, doi: 10.1097/00006123-199904000-00096, indexed in Pubmed: 10201312.