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| CASE REPORT |
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| Year : 2020 | Volume
: 3
| Issue : 2 | Page : 98-100 |
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Primary trans-calvarial lymphoma: A rare entity
Ayush Jain, Shilpa Sankhe, Soniya Patankar
Department of Radiology, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra, India
| Date of Submission | 14-Jul-2020 |
| Date of Acceptance | 07-Aug-2020 |
| Date of Web Publication | 26-Dec-2020 |
Correspondence Address:
Dr. Soniya Patankar
Department of Radiology, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra
India
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/IJNO.IJNO_17_20
Primary trans-calvarial lymphoma with both intracranial and extracranial extension is a scarcely described entity in published literature. Most often, these lesions are misdiagnosed as hematomas or meningiomas. We hereby report an interesting case of a 60-year-old female presenting with a large scalp mass. The diagnosis at the time of referral was an organized scalp hematoma. Magnetic resonance imaging of the brain revealed a large isointense enhancing mass lesion with transcalvarial extension involving the scalp as well as the meninges. Histopathology and immunohistochemistry confirmed a diagnosis of diffuse large B-cell lymphoma. Our case was unique in that the presentation was with a large scalp lesion with a reasonable meningeal component and invasion of the superior sagittal sinus without any associated focal neurological deficit.
Keywords: Calvarial lymphoma, diffuse large B-cell lymphoma, scalp mass
How to cite this article:
Jain A, Sankhe S, Patankar S. Primary trans-calvarial lymphoma: A rare entity. Int J Neurooncol 2020;3:98-100 |
| Introduction | |  |
Primary non-Hodgkin's lymphoma (NHL) of the cranial vault and dura is a rare entity and trans-calvarial involvement is even rarer, though its incidence has increased in recent years due to rise in number of acquired immunodeficiency syndrome (AIDS) and organ transplant patients.[1] Skeleton is the primary site for lymphoma in up to 4% of the cases.[2] Most of the cases present with a scalp mass, although a minority of the patients may present with seizures or parenchymal infiltration.[3]
We present a rare case of a primary transcalvarial lymphoma (PTCL) presenting with a scalp swelling.
| Case Report | | |
A 75-year-old female presented to the outpatient department with complaints of a painful scalp mass lesion in the midline. There was no history of trauma or any operative procedure. The swelling had been painless, to begin with, but had been increasing in size for the past 6 months with recent onset of diffuse pain over the scalp. There was no history of fever or weight loss. On clinical examination, there was a diffuse 12 cm × 9 cm sized firm lesion at the vertex, which was fixed to the underlying bone. No ulceration or signs of inflammation were seen at the local site. No pulsations were felt over the lesion. The clinical differential diagnoses at this stage were chronic scalp hematoma, metastatic disease, and a primary calvarial neoplasm.
The patient was then referred to radiology for a local ultrasound, which showed homogenous hypoechoic soft tissue with internal vascularity at the local site. The mild irregular periosteal reaction was also seen in the underlying bone, which was confirmed on a lateral skull radiograph [Figure 1]. Magnetic resonance imaging (MRI) of the brain was then performed for further characterization [Figure 2]. The study revealed T1/T2 isointense soft tissue at the local site. Mild cortical irregularity of the underlying bone was seen with diffuse T2/T1 hypointense signal within, suggestive of sclerosis. There was also a fairly large soft-tissue component intracranially extending along the dura, causing compression of the underlying brain parenchyma. Mild signal abnormality with cortical thickening was seen in the left superior frontal gyrus. The lesions were isointense on fluid-attenuated inversion recovery and showed mild restricted diffusion on diffusion-weighted imaging. There was a homogenous enhancement on a postcontrast study with the intracranial component seen to extend into the leptomeninges. There was nonopacification of the superior sagittal sinus at the involved site, suggestive of tumor involvement. A computed tomography (CT) scan of the head was then performed to confirm a periosteal reaction and to look for any bony defects [Figure 3]. The study showed perpendicular spiculated periosteal reaction extending both into the scalp and intracranially. Extensive sclerosis of the underlying calvarial bone was seen without any focal bony defect. Based on the imaging findings, differential diagnoses of metastatic disease and PTCL were considered. Screening ultrasound of the neck and abdomen was negative for any underlying primary neoplasm. | Figure 1: Frontal (a) and lateral (b) radiographs of the skull showing a soft-tissue swelling overlying the vertex (red asterisk). The underlying bone shows mild irregular periosteal reaction with associated sclerosis (white arrow)
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 | Figure 2: Coronal fluid-attenuated inversion recovery (a) image of the brain shows the isointense lesion (blue arrow) with a significant dural component (yellow arrow). Mild cerebral cortical thickening and hyperintensity in the left superior frontal gyrus. The lesion is an isointense on Sagittal T1 nonfat saturated image (c) with homogenous enhancement on postcontrast study (b and d). Note the nonvisualization of the superior sagittal sinus, and a significant leptomeningeal (green arrow) and pachymeningeal component in (b). Furthermore, note T2/T1 hypointense signal in the underlying bone in all sequences- suggestive of sclerosis (red asterisk)
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 | Figure 3: Sagittal (a) and coronal (b) sections of bone windows of unenhanced CT show a large ill-defined scalp mass in the midline near the vertex. The underlying bone is sclerotic (red arrow) with perpendicular spiculated periosteal reaction extending both intra-and extra-cranially (blue arrow). No lytic lesions are seen
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Positron emission tomography-CT (PET-CT) showed the lesion to be intensely avid with 18 fluoro-deoxy-glucose (FDG) having a standardized uptake value of 12. Few FDG avid occipital lymph nodes were seen on the left side. No other abnormal FDG avid lesion was seen elsewhere.
The patient then underwent an ultrasound-guided biopsy of the scalp mass, which revealed that diffuse large B-cell lymphoma. Immunohistochemistry was positive for CD-45 (leukocyte common antigen). Cerebrospinal fluid study was performed, which was negative for lymphomatous involvement. She was also tested for HIV due to its high association with lymphoma and was negative for the same. The patient was then referred to oncology for further management wherein a decision concurrent chemoradiation therapy (CCRT) was made. Surgery was deferred due to focal leptomeningeal involvement on MRI. At the time that this case report is being written, the patient is undergoing CCRT.
| Discussion | | |
NHL accounts for 3%–4% of all the neoplasms in the general population.[4] Even rarer is the occurrence of PTCL. Primary bone lymphoma is defined as a solitary mass with no evidence of disease at any other site and no systemic disease within 6 months of tumor detection.[5] Only 26 cases of PCL have been documented in published literature.[3],[4] The incidence of NHL, in general, is rising due to the increased number of patients with AIDS and organ transplants. PTCL most commonly arises from the cerebral convexities; however, other primary sites such as falx, tentorium, and sella have been reported.[6] The patients commonly present between the fifth and eighth decades of life with a scalp mass, which may or may not be painful depending on bone infiltration and meningeal involvement. Seizures or focal neurological deficits may be associated with infiltration of the cerebral cortex. There is no gender predilection.
Imaging modalities for the diagnosis of PCL include CT, MRI, and PET-CT. The lesions appear hyperdense on unenhanced CT owing to their high cellularity.[7] Our case also showed a perpendicular periosteal reaction in addition to the hyperostosis of the underlying bone. MRI demonstrates T1 isointense soft tissue showing restricted diffusion and significant enhancement on the postcontrast study.[3],[4],[8] The underlying bone shows varying appearances from lysis to hyperostosis. Due to the permeative nature of the neoplasm, infiltration may not be always seen,[3] as was the case in our patient. The spread of the neoplasm occurs through the diploic space and emissary veins further into the meninges and adjacent parenchyma. It may also spread through the cortical veins or directly into the dural venous sinuses, as was seen in our case. The most characteristic pattern of disease is the involvement of all compartments of the cranial vault, which is seen very well on imaging.[9] In a review by El Asri et al., bony lesions were lytic in 75% of the cases and hyperostotic in 5%. No bony changes were seen in 17.5% of the cases.[9] In our case, extensive sclerosis with periosteal reaction was seen.
Differential diagnoses for PTCL include aggressive meningioma, metastatic disease, and primary cutaneous lymphoma of the scalp. Aggressive noncalcified meningiomas may mimic PTCL; however, there would be contiguity between the dural and the scalp component.[10] In case of a diagnostic dilemma, digital subtraction angiography may be performed, wherein meningiomas are hypervascular, whereas lymphomas would appear hypovascular.[9] The metastatic disease usually presents with lytic bony lesions and associated soft tissue. Primary cutaneous lymphoma of the scalp is another important differential, which is less frequently associated with intracranial extension.[7]
Further investigations in patients with suspected lymphoma involve PET-CT to decide the mode of follow-up along with an image-guided biopsy for confirmation of the diagnosis.
Due to the paucity of reported cases in the literature, the optimal management of PTCL has not been standardized. In cases of focal extranodal disease limited to a single site, complete resection is considered the treatment of choice. However, combined chemoradiation has also been used for the management of this disease with promising results.[11]
It is, therefore, important to consider rarer pathologies such as PTCL in elderly patients presenting with scalp masses. CT, MRI, and PET-CT have a primary role to play in the diagnosis, extent evaluation, and follow-up of these patients.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]
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