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|LETTER TO EDITOR
|Year : 2017 | Volume
| Issue : 2 | Page : 420-422
A rare case of giant solitary calvarial plasmacytoma: Can it grow bigger than this?
Dipanker Singh Mankotia1, Sachin A Borkar1, Kavneet Kaur2, Vaishali Suri2, Bhawani S Sharma1
1 Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India
2 Department of Neuropathology, All India Institute of Medical Sciences, New Delhi, India
|Date of Web Publication||10-Mar-2017|
Dr. Sachin A Borkar
Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Mankotia DS, Borkar SA, Kaur K, Suri V, Sharma BS. A rare case of giant solitary calvarial plasmacytoma: Can it grow bigger than this?. Neurol India 2017;65:420-2
A 36-year-old previously healthy gentleman presented to our outpatient department with an off-and-on headache and a large, progressively increasing, scalp swelling that was noticed for the last 6months. The gigantic nature of the swelling was cosmetically unacceptable and at the same time, made it difficult for him to hold his head upright. The swelling also interfered with opening of his left eye completely. Being from a remote area, the patient neglected the swelling initially until it had assumed gigantic proportions. On examination, a huge (18 × 13 cm) and disfiguring frontal scalp swelling with variegated consistency and prominent overlying vessels was noticed [Figure 1]. The swelling was fixed to the underlying bone as well as the overlying scalp. No impulse on coughing or pulsations could be appreciated over the swelling.
|Figure 1: (a-c) Preoperative clinical photograph of patient showing a large swelling in the frontal region. The swelling measured 52 cm in mid circumference (c)|
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Non-contrast computed tomography (NCCT) of the head [Figure 2]a,[Figure 2]b,[Figure 2]c with bone window revealed a large extradural mass with associated extensive bony destruction. The inner as well as the outer tables of the frontal bone was completely thinned out.
|Figure 2: Preoperative non-contrast computed tomography (NCCT) head scan with three-dimensional axial (a), coronal (b), sagittal (c) reconstruction showing a large extradural calvarial tumor, extensive bony destruction, and thinning of both outer and inner cortex. Contrast-enhanced brain axial scans showing the extradural lesion hypointense on T1W (d), hyperintense on T2W (e) images , with an heterogeneous contrast uptake (f)|
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Magnetic resonance imaging (MRI) of the brain [Figure 2]d,[Figure 2]e,[Figure 2]f revealed a 18 × 13 × 10 cm extradural tumorous growth in the fronto-parietal region, which was hypointense on T1- and hyperintense on T2-weighted images. The tumor had caused erosion of the cribriform plate and the medial margin of the orbit. There was heterogeneous contrast uptake inside the tumor on gadolinium injection. The radiological differential diagnoses of a meningioma, eosinophilic granuloma, giant cell tumor, bony metastasis, and plasmacytoma were considered at this point.
After a detailed work-up, the patient was taken up for elective surgery under general anesthesia. A bicoronal skin incision was made and the galeal flap was raised. The tumor was reddish, encapsulated, and highly vascular. Large feeding arteries from the superficial temporal artery were identified early and ligated carefully. The tumor was dissected from all around disrupting the major vascular supply from the skull base. The tumor was completely extradural. It had caused extensive bony destruction with complete erosion of the outer table of the skull while the inner table was grossly thinned out. In view of the highly vascular nature of the tumor, we avoided a piecemeal removal and performed a complete en bloc excision instead. Portions of the tumor extending into the frontal sinuses were removed separately. We exteriorized the exposed frontal sinuses using betadine-soaked gelfoam and vascularized pericranial graft [Figure 3]a,[Figure 3]b,[Figure 3]c. Intraoperatively, there was approximately 2 litres of blood loss, which was replaced adequately. The calvarial defect was reconstructed aesthetically using synthetic bone cement [Figure 3]d. The redundant scalp flap was excised before a tension free approximation of the skin margins. The postoperative course was uneventful. The patient could open his left eye completely and the cosmetic outcome was also satisfactory [Figure 3]e.
|Figure 3: Intraoperative photograph showing the tumor margins after raising the flap (a), the post-excision operative field (b), and the excised en bloc tumor specimen. (c) Postoperative NCCT head showing complete closure of the calvarial defect. (d) Postoperative clinical photograph (e)|
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Histopathological examination [Figure 4] of the excised specimen revealed sheets of plasma cells including the binucleate forms consistent with the diagnosis of a plasmacytoma. On immunohistochemical staining, the cells were positive for CD138. Serum electrophoresis further revealed an M band of <1.7g/dl. Bone marrow aspiration revealed a cellular marrow composed predominantly of hematopoietic cells of all series. Mature plasma cells constituted only 3% of the cells in the marrow aspirate. Urine was found negative for Bence Jones proteins. His erythrocyte sedimentation rate was 20 at the end of first hour and the renal parameters were normal. With skeletal survey ruling out lesions elsewhere in the body, a diagnosis of solitary calvarial plasmacytoma was further confirmed. Postoperatively, the patient received focused radiotherapy at the operative site. At a 3 month follow up, the patient was doing well, without any evidence of local or systemic relapse.
|Figure 4: Low power magnification showing sheets of plasma cells (a, 40× H and E); highly vascular tumor and no amyloid deposition seen (b, 100× H and E). High power view shows sheets of cells with eccentric round or oval nuclei, with a speckled chromatin and abundant cytoplasm suggestive of mature plasma cells including binucleate forms (c, 400× H and E); CD138 immunopositivity in almost all neoplastic cells (d, CD138 immunohistochemistry)|
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Multiple myeloma is a malignant condition of the proliferating plasma cells in the bone marrow and represents a systemic disorder. On the other hand, plasmacytoma is a localized lesion due to plasma cell proliferation without any clinical, histological, or radiological evidence of systemic manifestations, as seen in myeloma.,,, A plasmacytoma rarely occurs in the younger age groups with the median age at presentation being in the sixth decade.,
The axial spine and the long bones of the extremities are the usual sites of involvement in plasmacytomas. Calvarial involvement is, however, very rare.
The diagnosis of solitary plasmacytoma requires demonstration of fewer than 5% plasma cells in the bone marrow, absence of urinary Bence Jones protein, less than 2 g/dl of monoclonal protein on serum electrophoresis, a solitary bony lesion on skeletal survey, and an absence of hypercalcemia or any associated renal impairment.,,, The current case fulfilled all these criteria. To the best of our knowledge, excision of calvarial plasmacytoma of this size has not been reported before.
It is important to differentiate a multiple myeloma associated osteolytic lesion from a solitary plasmacytoma. This differentiation has both management and prognostic significance. Although relatively uncommon, solitary bone plasmacytoma may convert to a frank multiple myeloma over months to years.
The standard management for solitary plasmacytoma is surgical excision with or without adjuvant radiotherapy.,, Plasmacytomas arising from the skull bones are often rich in vascularity. Their blood supply is derived from the superficial temporal, middle meningeal, and sometimes, the external carotid artery itself. According to Arienta et al., primary treatment of plasmacytomas should be surgical excision and radiotherapy should be reserved for recurrence. Being highly radiosensitive, some authors have successfully used surgical decompression with adjuvant high dose radiotherapy for managing these tumors.,,, The role of postoperative chemotherapy is highly controversial and its benefit remains questionable. Solitary plasmacytoma should be aggressively treated as conversion to multiple myeloma and subsequent fatal outcome is well-known. These patients require lifelong hematological, radiological, and clinical follow up.,,,,,
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]