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Table of Contents    
LETTER TO EDITOR
Year : 2013  |  Volume : 61  |  Issue : 2  |  Page : 201-204

Primary malignant non-Hodgkin's lymphoma of the skull vault in an immunocompetent patient


1 Department of Neurosurgery, Post Graduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Histopathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India

Date of Submission08-Feb-2013
Date of Decision24-Feb-2013
Date of Acceptance09-Mar-2013
Date of Web Publication29-Apr-2013

Correspondence Address:
Pravin Salunke
Department of Neurosurgery, Post Graduate Institute of Medical Education and Research, Chandigarh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.111161

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How to cite this article:
Salunke P, Garg R, Bal A, Kedia S, Bindal S. Primary malignant non-Hodgkin's lymphoma of the skull vault in an immunocompetent patient. Neurol India 2013;61:201-4

How to cite this URL:
Salunke P, Garg R, Bal A, Kedia S, Bindal S. Primary malignant non-Hodgkin's lymphoma of the skull vault in an immunocompetent patient. Neurol India [serial online] 2013 [cited 2020 Dec 3];61:201-4. Available from: https://www.neurologyindia.com/text.asp?2013/61/2/201/111161


Sir,

Primary non-Hodgkin's lymphoma (NHL) of the cranial vault is rare. [1] We report a young immunocompetent patient with primary cranial vault NHL in this report.

A 30-year-old man presented with generalized headache and progressively increasing firm, non-tender swelling on the left frontal region of his scalp of 3 months duration. In addition to the left frontal swelling, he had neurological deficits. Hematological investigations were within normal limits. Radiology revealed contrast enhancing mass straddling across the left frontal bone on either side. The vault appeared expanded with both lytic and sclerotic areas involving both tables [Figure 1]. The lesion caused displacement of gray-white matter interface with massive cerebral edema [Figure 2]. Malignant meningioma and Ewing's sarcoma were our differentials. Whole-body positron emission tomography-computerized tomography (PET-CT) showed uptake only in the left frontal region [Figure 2]. He underwent left frontal craniotomy and gross total excision of tumor. The bone was brittle and thickened with tumor tissue in the subgaleal as well as the subdural space. The lesion was fleshy, moderately vascular, and had a good plane of cleavage from brain, except at places where it appeared stuck to the underlying brain parenchyma. The bone was shaved clean off the lesion and reposited. Immediate post-operative scan showed excision of lesion with edema [Figure 3]a. Histopathology revealed NHL of diffuse large B-cell type involving the dura and the overlying bone [Figure 4]a and b. The cells tested positive for CD 20 [Figure 4]c. The patient was HIV negative. A postoperative CT scan of chest and abdomen showed no evidence of primary lesion. Bone marrow examination was normal. All these finding were suggestive of primary NHL of the cranial vault without systemic involvement. The patient received post-operative whole-brain radiation of 36 grays in 20 fractions, followed by 6 cycles of chemotherapy (cyclophosphamide 750 mg/m 2 , vincristine 2 mg, adriamycin 50 mg/m 2 on day 1, followed by prednisolone 100 mg/day for 5 days in each cycle). Presently, he is doing well at 6-months follow-up. Magnetic resonance imaging (MRI) at 6-months follow-up showed no recurrent or residual lesion [Figure 3]b.

Bony involvement is a common finding in many types of lymphoma. However, primary osseous NHL is rare and usually involves the femur, tibia and pelvis, spine, mandible, and scapula. [2] Primary lymphoma of the scalp vault is extremely rare. [1] A primary bony lesion due to malignant lymphoma is defined as a solitary mass lesion without any evidence of disease at other sites and no systemic dissemination found within 6 months of detection of tumor. [3] The initial symptoms and signs include a painless scalp lump (90%), headache (30%), focal neurological deficit (20%), and seizures (10%). Rarely there may be unilateral proptosis and diverse features due to diffuse involvement of cranial vault and brain parenchyma. These lesions are reported in older patients with a mean age of 60.5 years. Our patient was HIV negative and presented at a young age as compared to other reported cases. [2]
Figure 1: CT scan revealed mass straddling across the left frontal bone on either side. The vault appeared expanded with both lytic and sclerotic areas in both tables, which was better appreciated on bone window

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Figure 2: The lesion was hypointense on T1 and hyperintense on T2 involving the frontal bone, subgaleal tissue, and subdural space and exerted mass effect on the underlying brain, causing displacement of gray-white matter interface with massive cerebral edema. After contrast administration, the lesion enhanced. PET-CT showed uptake only in the left frontal region

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Figure 3: (a) Immediate post-operative CT images showed excision of lesion with edema. The bone flap was reposited after shaving off the lesion; (b) Contrast MRI (axial and sagittal) images following completion of chemoradiation showed no recurrence or residual lesion

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Figure 4: (a) Photomicrograph showing lymphoma cells infiltrating bony trabeculae (H and E, × 100); (b) Photomicrograph showing sheets of atypical lymphoid cells with background fibrosis (H and E, × 200); (c) Photomicrograph showing CD20 positivity in lymphoma cells (Cd20 immunostain, × 400)

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These lymphomas may extend outside the skull by infiltrating it and destroying it completely. In our patient, the skull was hyperostotic with lytic and sclerotic areas in the outer and inner tables with cerebral edema. Cerebral infiltration of primary cranial vault lymphoma has been described as a rare finding in previously reported cases and may present without neurological impairment and with edema. These tumors grow rapidly and may invade brain if diagnosis is delayed, therefore early diagnosis and treatment is important. [4]

Characteristic CT features of primary osseous lymphoma are a permeative growth pattern, a large soft-tissue component, and very little cortical destruction. On MRI, these tumors are iso to hypointense on T1-weighted and iso to hyperintense on T2-weighted sequences with homogenous enhancement on contrast. [1] Primary malignant lymphoma of the skull vault should be differentiated from metastatic carcinoma, meningioma, osteomyelitis, multiple myeloma, and Ewing's sarcoma. [1] When the diagnosis of cranial vault lymphoma is confirmed through physical examination, complete blood counts, CT scans of chest and abdomen and bone marrow biopsy must be done to rule out systemic involvement. [2] Our patient did not have any evidence of systemic involvement and the lesion was localized to the cranial region. In 60-90% of cases, these lymphomas are predominantly diffuse large cells, immunoblastic, lymphoblastic, or Burkitt's lymphomas. [2]

Due to the paucity of cases in literature, the optimal management of primary bone lymphoma remains uncertain. However surgical removal, followed by radiotherapy and chemotherapy, can be recommended. [4] Prognosis is difficult to define in these patients due to the small number of cases. The 5-year survival rate of 40-60% has been reported. [1] With brain invasion, the median survival reduces to 10.5 months from 17.5 months. [4] To conclude, primary cranial vault lymphoma are rare tumors and should be considered in differential diagnoses of scalp masses. Brain infiltration is exceptional in these cases. Complete surgical excision followed by radiotherapy and chemotherapy seems to offer better outcomes.

 
  References Top

1.Fukushima Y, Oka H, Utsuki S, Nakahara K, Fujii K. Primary malignant lymphoma of the cranial vault. Acta Neurochir (Wien) 2007;149:601-4.  Back to cited text no. 1
    
2.El Asri AC, Akhaddar A, Baallal H, Boulahroud O, Mandour C, Chahdi H, et al. Primary lymphoma of the cranial vault: Case report and a systematic review of the literature. Acta Neurochir (Wien) 2012;154:257-65.  Back to cited text no. 2
    
3.Paige ML, Bernstein JR. Transcalvarial primary lymphoma of bone. A report of two cases. Neuroradiology 1995;37:456-8.  Back to cited text no. 3
    
4.Ciarpaglini R, Otten P. Primary cranial vault lymphoma with brain infiltration: Case report and review of the literature. Br J Neurosurg 2012;26:756-8.  Back to cited text no. 4
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

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