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Year : 2017  |  Volume : 6  |  Issue : 4  |  Page : 267-269

Primary B cell lymphoma of skull mimicking meningioma: A case report

Department of Neurosurgery, Nizam's Institute of Medical Sciences, Punjagutta, Hyderabad, Telangana, India

Date of Web Publication26-Dec-2017

Correspondence Address:
Dr. A S. K Ahamed Sharief
Department of Neurosurgery, Nizam's Institute of Medical Sciences, Punjagutta, Hyderabad - 500 082, Telangana
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Source of Support: None, Conflict of Interest: None


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Primary presentation of a central nervous system (CNS) lymphoma is rare. Primary non-Hodgkin's lymphoma of the skull with extra and intracranial extensions without systemic or skeletal manifestations is extremely rare. This type of lesion often causes dilemma in diagnosis as they mimic other conditions. We herein report a case of primary lymphoma of the skull involving the scalp, skull vault, and meninges and invading the brain parenchyma, which mimicked meningioma radiologically as well as intraoperatively.

Keywords: Lymphoma, meningioma, deangeles protocol

How to cite this article:
Ahamed Sharief A S, Alugolu R, Saradhi M V. Primary B cell lymphoma of skull mimicking meningioma: A case report. J NTR Univ Health Sci 2017;6:267-9

How to cite this URL:
Ahamed Sharief A S, Alugolu R, Saradhi M V. Primary B cell lymphoma of skull mimicking meningioma: A case report. J NTR Univ Health Sci [serial online] 2017 [cited 2021 Mar 5];6:267-9. Available from: https://www.jdrntruhs.org/text.asp?2017/6/4/267/221536

  Introduction Top

Primary extra lymph node lymphomas are not uncommon but they are often difficult to diagnose. Primary lymphoma arising from the skull vault can clinically and radiologically mimic a meningioma.[1],[2] Here, we report a case of lymphoma arising from the skull vault which mimicked a meningioma.

  Case Report Top

A 43-year-old female with no comorbidities presented with progressively increasing swelling over the left frontal region for the past 8 months. It was associated with localized headache. Local examination revealed a 4 × 4 cm, smooth surfaced, nontender, firm swelling adherent to the bone over the left frontal region. Her general and systemic examination revealed no abnormalities. Her routine blood investigations were unremarkable. Screening for human immunodeficiency virus, HBsAg, and hepatitis C virus were negative. X-ray chest, ultrasonography of the abdomen, peripheral smear, and bone marrow examination were unremarkable. Computed tomography (CT) scan of the brain showed a well-defined lesion in the left frontal region with intra and extracranial extension along with bone thickening [Figure 1]. The lesion was brilliantly enhancing with contrast on magnetic resonance imaging (MRI) [Figure 2]. Our working diagnosis was a meningioma. She underwent left frontal craniotomy and total excision of the lesion including the involved bone. Tumor was moderately vascular, fleshy in consistency, and infiltrating the scalp, bone, and brain parenchyma. Histopathology revealed a diffuse, large B cell type of lymphoma. Her immune status was normal. She received postoperative radiotherapy 50 Gy in 25 fractions over 35 days and chemotherapy using DEANGELES protocol (methotrexate, vincristine, cytarabine combination). The patient after 2 years of follow-up is doing fine, awaiting cranioplasty.
Figure 1: CT scan brain showing a well-defined lesion in the left frontal region with intra and extracranial extension with thickening of bone

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Figure 2: MRI axial image of the brain showing brilliant enhancement on intravenous Gadolinium administration

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  Discussion Top

Non-Hodgkin's lymphoma (NHL) represents only 3–4% of all neoplasms in the general population and occurs more frequently in patients with acquired immunodeficiency syndrome. Direct involvement of the central nervous system (CNS) occurs only in 1–2% of patients with lymphoma.[2],[3],[4],[5] These lesions are commonly reported in the seventh and eighth decade.[2],[6],[7],[8],[9] Primary involvement of bone by NHL occurs in 3–4% of the cases.[2]

The clinical symptoms and signs of lymphoma in the skull include a painless scalp lump, headache due to bone destruction, or infiltration of meninges with tumor, seizures, and focal neurological deficits resulting from the cortical infiltration.[7],[9],[10]

The disease can involve the pericranium, underlying meninges, and subcutaneous tissue.

Pathologically, the spread of the disease to the meninges suggests that the lymphoma cells grow through the diploic spaces along the emissary veins and nerves that pass through the dura to the leptomeninges. Because of the characteristic permeating growth pattern of lymphoma with large soft-tissue component, bone destruction may not be seen, as in the present case.[7],[9],[10]

Cerebral CT scan can show extra-intracranial extent and bone and dura mater invasion.[8] On plain CT scan, these lesions are isodense which enhances after contrast administration.[2],[6],[11]

The angiographic findings of these lesions include mild vascularity in the periphery of the tumor and displacement of neural and vascular structures unlike meningiomas.[2] Radiological differences between meningiomas and lymphomas are listed in [Table 1].
Table 1: Lymphoma vs Meningioma

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When there is diffuse vault, meningeal, and parenchymal infiltration, an intraoperative frozen section is recommended because the identification of a lymphoma is likely to influence the decision regarding the extent of the surgical excision.[2],[7] These lesions are effectively treatable by surgery and radiotherapy with a good outcome in most cases.[10],[12],[13] This can be followed by systemic chemotherapy with cyclophosphamide, vincristine, and prednisolone (CVP).[8]

The present case was managed by wide excision of tumor and involved bone followed by radio and chemotherapy with good response. The prognosis of a lymphoma appearing in the skull vault is uncertain but any involvement of the cerebral structures by direct invasion or by leptomeningeal seeding indicates a less favorable prognosis.[14],[15],[16] The 5 and 10-year survival rates for patients with multifocal bone lymphomas were 42 and 35%, respectively, in the Mayo Clinic series.[17] When nodal or soft tissue was involved, the survival rates dropped to 22 and 12%, respectively.[17]

  Conclusion Top

A high index of clinical suspicion, awareness of the characteristic CT features, and aggressive therapy are required for these unusual cases. Although this condition is rare, the diagnosis must be considered in the differential diagnosis in any patient with a scalp mass extending through the skull.

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.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Agbi CB, Bannister CM, Turnbull IW. Primary cranial vault lymphoma mimicking a meningioma. Neurochirurgia (Stuttg) 1983;26:130-2.  Back to cited text no. 1
Jamjoom AB, Jamjoom ZA, Naim-Ur-Rahman, Cheema MA. Primary midline cranial vault lymphoma simulating parasagittal meningioma: The role of angiography in preoperative diagnosis. Neurosurg Rev 1998;21:202-5.  Back to cited text no. 2
Curty B, Kernan J, Favre J. Malignant Non Hodgkins lymphoma of the cranial vault: A case report. Br J Neurosurg 1997;11:433-6.  Back to cited text no. 3
Braus DF, Schwechheimer K, Muller–Hermelink HK, Schwarzkopf G, Volk B, Mundinger F. Primary cerebral malignant non Hodgkins Lymphomas: A retrospective clinical study. J Neurol 1992;239:117-24.  Back to cited text no. 4
Baleydier F, Galambrun C, Manel AM, Guibaud L, Nicolino M, Bertrand Y. Primary lymphoma of the pituitary stalk in an immunocompetent 9 year old child. Med Pediatr Oncol 2001;36;392-5.  Back to cited text no. 5
Tanimura A, Adachi Y, Tanda M, Yuasa H, Ishii Y, Katou Y. Primary Peripheral B cell Lymphoma, Burkitt-like, of the cranial vault. Acta Haematol 2005;113:258-61.  Back to cited text no. 6
Aquilina K, O'Brien DF, Philips JP. Diffuse primary non-Hodgkins lymphoma of the cranial vault. Br J Neurosurg 2004;18:518-23.  Back to cited text no. 7
Magina S, Mesquita-Guimaraes J, Resende C, Bello M, Dias C. Scalp tumor as a sign of systemic B cell lymphoma. J Eur Acad Dermatol Venerol 2004;18:196-8.  Back to cited text no. 8
Parekh HC, Sharma RR, Keogh AJ, Prabhu SS. Primary malignant non-Hodgkin's Lymphoma of cranial vault: A case report. Surg Neurol 1993;39:286.  Back to cited text no. 9
Holtas S, Monajati A, Utz R. Computed tomography of malignant lymphoma involving the skull. J Comp Assist Tomog 1985;9:725-7.  Back to cited text no. 10
Kantarci M, Erdem T, Alper F, Gundogdu C, Okur A, Aktas A. Imaging characteristics of diffuse primary cutaneous B-cell lymphoma of the cranial vault with orbital and brain invasion. Am J Neuroradiol 2003;24:1324-6.  Back to cited text no. 11
Braunstein EM, White SJ. Non Hodgkins Lymphoma of bone. Radiology 1980;135:59-63.  Back to cited text no. 12
Maiuri F, Conero G, Giamundo A. Primary lymphoma of the cranial vault. J Neurosurg Sci 1987;31:183-6.  Back to cited text no. 13
Mongia S, Shukla D, Devi BI, Reddy TV. Primary cranial vault non- Hodgkin's lymphoma. Neurol India 2003;51:293-4.  Back to cited text no. 14
[PUBMED]  [Full text]  
Wendum D, Sebban C, Gaulard P, Coiffier B, Tilly H, Cazals D, et al. Follicular large cell lymphoma treated with intensive chemotherapy: An analysis of 89 cases included in the LNH87 trial and comparision with the outcome of diffuse large B-cell lymphoma: Groupe d'Etude des Lymphomes de l'Adulte. J Clin Oncol 1997;15:1654-63.  Back to cited text no. 15
Lonjon M, Hofman P, Thyss A, Paquis, P, Roche, Lymphome primitif de la vouà te. A propos d'un cas. Neurochirurgie 1993;39:50-3.  Back to cited text no. 16
Ostrowski ML, Unni KK, Banks PM, Shives TC, Evans RG, O'Connell MJ, Taylor WF. Malignant lymphoma of bone. Cancer 1986;58:2646-55.  Back to cited text no. 17


  [Figure 1], [Figure 2]

  [Table 1]


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