





































Clinical Medicine Insights
Clinical Medicine Insights
DOI: https://doi.org/10.52845/CMI/2022-3-1-1 MEERP LTD
CMI 3 (1), 244−249 (2022)
Received 15 Nov 2021 | Revised 30 Nov 2021 | Accepted 25 Dec 2021 |Online Available 8 Jan 2022

CASE REPORT

OPEN ACCESS ISSN (O) 2694-4626

Primary malignant large B-cell non-Hodgkin’s lymphoma of the scalp
and cranial vault: a case report and an overview

Fatma Kolsi1  | Mehdi Borni2∗ | Ines Cherif3  | Marouen Taallah4 | Slim Charfi5 | Mohamed 

Zaher Boudawara
6

Abstract
Primary non-Hodgkin’s malignant lymphoma of the bone is a rare entity, accounting for 4% of all non-Hodgkin’s malignant
lymphomas (NHL). Cranial vault involvement is rarer, found only in 0.2% of cases (1)(2). Only few cases have been reported
previously in the literature (3)(4).
Here, the authors are reporting a new case of primary malignant large B-cell non-Hodgkin’s lymphoma of the cranial vault
because of its rare occurrence.

Key words: nonHodgkin’s malignant lymphoma, scalp, surgery

1 INTRODUCTION

Primary non-Hodgkin’s malignant lymphoma
of the bone is a rare entity, accounting for 4%
of all non-Hodgkin’s malignant lymphomas

(NHL). Femur, tibia and pelvis are the most common
sites. Cranial vault involvement is rarer, found only
in 0.2% of cases (1) (2). The absence of involvement
of the cerebral parenchyma and systemic or skeletal
manifestation is also rare. Only few cases have been
reported previously in the literature (3) (4). Primary
malignant lymphoma of the bone is defined as an iso-
lated bone lesion with no signs of other sites involve-
ment and no blood-borne spreadwithin sixmonths of
diagnosis (5).
Here, the authors are reporting a new case of primary
malignant large B-cell non-Hodgkin’s lymphoma of
the cranial vault because of its rare occurrence.
Case report

A 30-year-old young lady with no medical or surgi-
cal history was admitted from our outpatient depart-
ment of neurosurgery for progressively growing of
a painless scalp lump at the level of the parieto-
occipital right bone. There were no signs of raised
intra cranial pressure mostly without headache. The
patient confirmed no prior head trauma, fever, or
general condition impairment. Upon neurological
examination, she was awake, alert and well oriented
with no neurological disorder. Local examination of
her scalp swelling revealed a hard and well-defined
nontender immovable solitary subcutaneous painless
lump, measuring 7cm in diameter. She had no lym-
phadenopathy or hepatosplenomegaly and the rest of
her body check-up showed no abnormalities includ-
ing her lymph node areas. The complete blood
count showed hemoglobin level of 11.4 g/dL, white
blood cell count of 8500/mm3 with a platelet count
of 331000/mm3. Both blood serum ionogram and
hemostasis assessment and evaluation were normal.

12346
Department of Neurosurgery – UHC  Habib Bourguiba –Sfax (Tunisia),

 5
Department of Pathology – UHC Habib Bourguiba –Sfax (Tunisia), 12346.   

Address correspondence to: Mehdi, Borni, Department of Neurosurgery – UHC Habib Bourguiba –Sfax (Tunisia)

Supplementary informationThe online version of this article (https://doi.org/10.52845/CMI/2022-3-1-1) contains supplementary material, which is available to authorized users.
Fatma Kolsi et al., 2022; Published by MEERP LTD, Inc. This Open Access article is distributed under the terms of the Creative Commons License
(http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Fig. 1: Axial brain computed tomography (CT) scan in 
parenchymal window (a, b) and bone window (c) showing 
expansive osteoly c lesion (a, b; yellow arrows) (c: white 
arrows) in the right parieto-occipital bone measuring 3.6 * 1 
cm in diameter without perilesional edema or a midline shi ; 
d: three-dimensional CT reconstruc on showing bone lysis 
(black arrow). Note the subcutaneous lump clearly visible (a, 
b; red arrows).

Fig. 2: Axial brain magne c resonance imaging showing a
fairly ly c bone mass inisosignal on T1-weighted image (a;
white arrow) and discreet hypersignal on T2 sequence (b;
white arrow) with homogenous enhancement a er Gadolin-
ium chelates injec on (c; white arrows). Fluid a enuated
inversion recovery sequence showed discreet hypersignal (d)
and GRE T2

∗
weighted image revealed low intensity signal in

favor of bone signal (e; ; yellow arrows). Diffusion weighted
image showing a significant restricted signal (f).

Further investigation through magnetic resonance
imaging (MRI) (Figure 2) confirmed the fairly lytic
bone mass in isosignal on T1-weighted image and
discreet hypersignal on T2 sequence with homoge-
nous enhancement after Gadolinium chelates injec-
tion. The lesion comes into contact with the superior
sagittal venous sinus without infiltrating it. Diffu-
sion weighted image showed a significant restricted
signal with low apparent diffusion coefficient (ADC)
value on ADC mapping. As part of the extension
assessment, the thoraco-abdominopelvic CT, breast
and thyroid ultrasound, as well as bone scintigraphy
were without abnormalities. All these finding sug-
gested thus a primary bone scalp lymphoma.

MEERP LTD CMI 3 (1), 244−249 (2022) 245

Fig. 3: Preopera ve (le  image) and intraopera ve (right 
image) photographs in the opera ng room showing the 
supine posi on with pa ent's head fixed in Mayfield head 
clamp turned slightly to the le  side biparietal skin incision 
(red arrow) was performed and an excision of her bone mass 
through. Preopera vely the mass was subcutaneous, hard, 
grayish, and easily cleavable (black arrows). The dura mater 
was intact and the bone scalp was found to be moth eaten 
(white arrows).

A brain computed tomography (CT) scan (Figure 1) 
was thus performed showing an expansive 
osteolytic lesion in the right parieto-occipital bone 
measuring 3.6 * 1 cm in diameter without 
perilesional edema or a midline shift.



Primary malignant large B-cell non-Hodgkin's lymphoma of the scalp and cranial
vault: a case report and an overview

Fig.

 

4:

 

Immediate

 

axial

 

postopera ve

 

CT

 

scan

 

in

 

parenchymal

 

window

 

before

 

(a)

 

and

 

a er

 

enhancement

 

(b)

 

showing

 

the

 

absence

 

of

 

tumor

 

residue.

 

Axial

 

CT

 

in

 

bone

 

window

 

(c)

 

and

 

three-dimensional

 

reconstruc on

 

(d)

 

correct

 

placement

 

of

 

the

 

cranioplasty

 

(e).

CMI 3 (1), 244−249 (2022) MEERP LTD 246

The patient underwent, under general anesthesia 
and in supine position with his head fixed in a 
Mayfield head clamp turned slightly to the left 
side (Figure 3), an excision of her bone mass 
through a large biparietal skin incision. The mass 
was subcutaneous, hard, grayish, and easily 
cleavable. The dura mater was intact and the bone 
scalp was found to be moth eaten. By the end a 
cranioplasty was performed covering the tumor 
bed. The mass along with the involved bone were 
sent for histopathological examination. 

Postoperative course was uneventful. Immediate 
postoperative CT scan (Figure 4) showed the 
absence of tumor residue as well as correct 
placement of the cranioplasty.

Fig. 5: Photomicrographs of a surgical specimen showing 
tumor prolifera on of similar morphology; tumor cells were 
round, of large size, with round nucleus, lobulated vesicles 
and small nucleoli, more or less prominent; mitoses were 
very numerous; this prolifera on massively infiltrates the 
bone ssue; it was in places delimited by a small cell 
lymphoid sleeve (a; HE x 100). Immunohistochemistry (b) 
showing diffuse immunoreac vity for leukocyte CD20.

Histopathological examination (Figure 5) was 
con-ducted showing tumor proliferation of similar 
mor-phology on all samples; it is a dense 
proliferation in diffuse, partly lobulated layers; 
the tumor cells were round, of large size, with 
round nucleus, lobu-lated vesicles and small 
nucleoli, more or less promi-nent; mitoses were 
very numerous; this proliferation massively 
infiltrates the bone tissue; it was in places 
delimited by a small cell lymphoid sleeve. 
There were small areas of necrosis. On 
immunohistochem-istry tumor cells were 
massively immunoreactive for leukocyte common 
antigen (LCA), CD20, Oct 2, and  Bcl-6 and they 



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Kolsi ET AL.

Fig. 5: Photomicrographs of a surgical specimen showing 
tumor prolifera on of similar morphology; tumor cells 
were round, of large size, with round nucleus, lobulated 
vesicles and small nucleoli, more or less prominent; 
mitoses were very numerous; this prolifera on massively 
infiltrates the bone s-sue; it was in places delimited by a 
small cell lymphoid sleeve (a; HE x 100). 
Immunohistochemistry (b) showing diffuse 
immunoreac vity for leukocyte CD20.

Histopathological examination (Figure 5) was 
con-ducted showing tumor proliferation of 
similar mor-phology on all samples; it is a 
dense proliferation in diffuse, partly lobulated 
layers; the tumor cells were round, of large size, 
with round nucleus, lobu-lated vesicles and small 
nucleoli, more or less promi-nent; mitoses were 
very numerous; this proliferation massively 
infiltrates the bone tissue; it was in places 
delimited by a small cell lymphoid sleeve. 
There were small areas of necrosis. On 
immunohistochem-istry tumor cells were 
massively immunoreactive for leukocyte common 
antigen (LCA), CD20, Oct 2, and Bcl-6 and they 
were weakly positive for CD 10. The 
proliferation index Ki 67 was estimated to be 
90%. All these feature were in favor of diffuse 
large B-cell non-Hodgkin lymphoma

Fig. 6: Brain MRI in axial plane at 01 month postopera vely
showing no signs of tumor recurrence on T1 and T2 weighted
images as well as a er enhancement.

Our patient underwent, at our out-patient clinic,
a physical examination, complete hemogram, and
brain MRI (Figure 6). All these investigations did
not revealed no signs of tumor recurrence.

2 DISCUSSION:

Primary bone lymphoma is a rare malignant tumor,
which accounts for only 4% of non-Hodgkin lym-
phomas (1). This entity is defined by the iso-
lated presence of lymphoma in the bone with-
out other distant localization within six months of
diagnosis (5). Bone involvement primarily affects
the femur, humerus, pelvis, spine, mandible, and
scapula. Primary localization to the cranial vault
remains extremely rare (6). It has been reported in
immunocompromised or trauma patients. However
primary NHL with extra- and/or intra-cranial exten-
sion without systemic or skeletal involvement in a
nonimmunocompromised or nontraumatic context is
extremely uncommon (4). To our knowledge, only
19 cases had been reported previously in the litera-
ture (1) .
The inaugural clinical signs revealing the disease
are painless lump in the scalp observed in 90% of
patients, headache due to bone destruction or tumor
infiltration of the meninges in 30% of cases, and

MEERP LTD CMI 3 (1), 244−249 (2022) 247

were weakly positive for CD 10. The 
proliferation index Ki 67 was estimated to be 
90%.

The patient was discharged of our department at 
day 4 postoperatively and addressed to the 
oncology’s department for further management. 
She received 3 cycles of systemic chemotherapy. 
The cycles were repeated after 3 weeks.

signs of focal neurological palsy secondary to tumor 
infiltration of the cerebral parenchyma in 10% of 
cases (1) (6). Lymphoma also may infiltrate the 
spaces within the diploe and along the emissary veins 
reaching the soft tissues on either side of the bone. In 
our patient, the painless scalp lump was the reveal-
ing mode with no other neurological symptoms. The 
parieto-occipital bone destruction as well as the spar-
ing of the dura were both obvious peroperatively.

Iconographic data is not sufficient to confirm the 
diagnosis. Thus, on MRI, these lesions are rel-
atively nonspecific, appearing in hypointense on 
T1-weighted sequences and hypersignal on that 
of T2 (2). Possible differential diagnoses are  



Primary malignant large B-cell non-Hodgkin's lymphoma of the scalp and cranial
vault: a case report and an overview

Iconographic data is not sufficient to confirm 
the diagnosis. Thus, on MRI, these lesions are 
relatively nonspecific, appearing in hypointense 
on T1-weighted sequences and hypersignal on 
that of T2 (2). Possible differential 
diagnoses are osteomyelitis, metastatic or 
primary skull tumors, and intraosseous 
meningioma. These lesions are accompanied by 
significant cortical bone destruction unlike 
lymphoma.

Histologically, most cranial vault lymphomas 
are non-Hodgkin’s large B-cell lymphomas 
expressing CD20 but not CD5, CD10 and CD23 
(6). In our patient, immunohistochemistry showed 
a tumor cells immunoreactivity for LCA (CD 45), 
CD20, Oct 2, and Bcl-6 with less important 
immunoreaction for CD 10.

Due to the rarity of cases described in the 
literature, the optimal treatment of primary 
lymphoma of the cranial vault has not been well 
established and no definite conclusion can be 
drawn. Some authors rec-ommend surgery 
followed by radiotherapy in case of localized 
disease, and chemotherapy in case of metastatic 
progression (7). It must be remembered that first 
of all, since primary lymphoma of the cen-tral 
nervous system (CNS) is multifocal and infiltrat-
ing, the role of surgery, unlike other brain tumors, 
is purely diagnostic. The place of surgery in 
primary bone lymphomas located in the cranial 
vault remains limited to the diagnosis of 
histopathology (bone biopsy). This dogma was 
recently challenged by a second analysis of the 
largest randomized trial of pri-mary CNS 
lymphomas, which included 526 patients and found 
a survival benefit from first surgery (8) . Further 
tests are needed to confirm these results and to this 
day it remains confined to its diagnostic role.

In view of the low incidence and the paucity of the
reported cases, it is still difficult to establish a defini-
tive prognosis for this disease. El Asri et al. (6)

no systemic involvement at the time of diagnosis 
and continued to be symptom-free after treatment 
with follow-ups ranging from five months to 72 
months.
Although it remains a rare entity, primary non-
Hodgkin’s malignant lymphoma should be cited in 
the differential diagnoses of expanding scalp 
lesions. The treatment must necessarily involve a 
multidisci-plinary therapeutic approach, including 
several spe-cialties including neurosurgery, 
radiotherapy and oncology

3 CONCLUSION

Primary lymphoma of the cranial vault remains an
extremely rare tumor. Its treatment is still not well
standardized. The most optimal therapeutic option is
based on the combination of surgery and / or radio-
therapy and chemotherapy. Therefore, a thorough
search is needed to improve the management of this
disease as well as its prognosis.

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reported that 13 were still alive among 16 
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How to cite this article: Kolsi F., Borni M.,
Cherif I., Taallah M., Charfi S., Boudawara
M.Z. Primary malignant large B-cell non-
Hodgkin’s lymphoma of the scalp and cranial
vault: a case report and an overview. Clinical
Medicine Insights. 2022;244−249. https://doi.or
g/10.52845/CMI/2022-3-1-1

MEERP LTD CMI 3 (1), 244−249 (2022) 249


	Introduction
	Discussion:
	Conclusion



