Novel case of primary intracranial solitary plasmacytoma presenting with significant intratumoral hemorrhage
- Department of Neurosurgery, The University of Tokyo Hospital, Tokyo,
- Department of Neurosurgery, Higashi Yokohama Hospital, Yokohama,
- Department of Pathology, The University of Tokyo Hospital, Tokyo, Japan.
Shunsaku Takayanagi, Department of Neurosurgery, The University of Tokyo Hospital, Tokyo, Japan.
DOI:10.25259/SNI_66_2022Copyright: © 2022 Surgical Neurology International This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, transform, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
How to cite this article: Daisuke Sato1, Shunsaku Takayanagi1, Hirokazu Takami1, Tetsuaki Iwamoto2, Masashi Nomura1, Shohei Nambu1, Masako Ikemura3, Shota Tanaka1, Nobuhito Saito1. Novel case of primary intracranial solitary plasmacytoma presenting with significant intratumoral hemorrhage. 22-Apr-2022;13:157
How to cite this URL: Daisuke Sato1, Shunsaku Takayanagi1, Hirokazu Takami1, Tetsuaki Iwamoto2, Masashi Nomura1, Shohei Nambu1, Masako Ikemura3, Shota Tanaka1, Nobuhito Saito1. Novel case of primary intracranial solitary plasmacytoma presenting with significant intratumoral hemorrhage. 22-Apr-2022;13:157. Available from: https://surgicalneurologyint.com/surgicalint-articles/11558/
Background: Solitary plasmacytoma is a localized lesion comprising monoclonal neoplastic proliferation of plasma cells. This disease is rarely encountered and few reports have described primary intracranial solitary plasmacytoma (PISP).
Case Description: We report a case of PISP that presented initially as status epilepticus and exhibited massive intratumoral hemorrhage at the subcortical area. To the best of our knowledge, this is the first recorded presentation of this pathology in this manner. Following evacuation of the hematoma and decompressive craniectomy, the patient underwent radiation therapy and showed no sign of tumor recurrence at 3 years after diagnosis.
Conclusion: This case reveals that PISP can present as subcortical intraparenchymal hemorrhage. It should be emphasized that the precise diagnosis of this disease is of utmost importance, because solitary plasmacytoma without a background of multiple myeloma responds well to radiation therapy.
Keywords: Decompressive craniectomy, Intratumoral hemorrhage, Primary intracranial solitary plasmacytoma, Radiotherapy
In general, plasma cell neoplasms are characterized by monoclonal proliferation of neoplastic plasma cells; these neoplasms include multiple myeloma, monoclonal gammopathy of undetermined significance, and solitary plasmacytoma.[
A 54-year-old female presented to hospital with status epilepticus. She had a previous history of subarachnoid hemorrhage (World Federation of Neurological Surgeons’ Grade II) due to a ruptured aneurysm at the bifurcation of the right middle cerebral artery that was treated by open clipping surgery when the patient was 44 years old. The initial computed tomography (CT) revealed a low-density area around the right central sulcus, apparently involving the primary motor area. Magnetic resonance imaging (MRI) demonstrated an intracerebral mass lesion that showed diffusion restriction, hypointensity on T1-weighted images, and hyperintensity on T2-weighted images [
MRI examination shows involvement of the lesion in the precentral gyrus and postcentral gyrus. The lesion shows strong diffusion restriction on diffusion-weighted imaging (a); and hypointensity on T1-weighted (b) and hyperintensity on T2-weighted imaging (c). The structural preservation of the gyri and sulci is a characteristic of this case and may reflect the pathology.
Histopathological sections revealed numerous red blood cells with fibrin and debris and massive infiltration of atypical round blue cells [
Histopathologically, numerous red blood cells with fibrin and debris are present, with massive infiltration of atypical round blue cells (a: ×40). The infiltrating cells are composed of clumped nuclear chromatin and eccentric eosinophilic cytoplasm (b: ×200), suggesting a plasma cell origin. Immunohistochemistry study shows CD138 positivity (b; inset). In situ hybridization demonstrates uniform lambda light chain positivity and negative kappa signal, reinforcing the diagnosis of plasma cell neoplasm (c and d: ×100).
Further imaging studies performed following the diagnosis, including F-18 fluorodeoxyglucose positron emission tomography and bone scintigraphy, revealed no involvement of other organs. Serum and urine electrophoresis showed no increase in monoclonal protein. Bone marrow biopsy showed plasma cell infiltration of 5%–10%, but without light chain restriction. A final diagnosis of PISP was confirmed. Although the patient was obtunded and showed severe left hemiparesis soon after the surgery, correlating with the focus of the lesion in the right central sulcus, postoperative CT revealed apparent brain decompression [
Postoperative CT shows evacuation of the hematoma and relaxation of the brain (a). Gadolinium-enhanced MRI demonstrates resection of the lesion, with a small amount of contrast enhancing tumor at the periphery of the cavity (b and c). Imaging obtained after intensity-modulated radiation therapy reveals a complete disappearance on gadolinium-enhanced MRI (d and e).
We report a case of PISP that presented with significant intratumoral hemorrhage at the subcortical level, causing considerable mass effect requiring surgical decompression.
This case did not exhibit involvement of other organs. No monoclonal protein was detected. The bone marrow biopsy showed plasma cell infiltration of 5–10% in the bone marrow, which did not reach the diagnostic criteria of multiple myeloma. Furthermore, she did not exhibit other features of multiple myeloma (anemia, renal failure, hypercalcemia, and osteolytic lesions). Therefore, we diagnosed as PISP.
Intracranial involvement of plasmacytoma is rare, most frequently involving the cranial vault, skull base, nose, or paranasal sinuses. Primary dural or leptomeningeal involvement is less frequent, and intraparenchymal involvement is exceedingly rare and seldom described.[
Although there have been several reports of intratumoral hemorrhage from intracranial lesions in the setting of multiple myeloma, there are no reports of intra-axial hemorrhage from PISP, with only acute subdural hematoma[
In general, multiple myeloma is known to have a poor prognosis; it is sometimes termed a “disseminated form.”[
In a 2018 study that pooled and analyzed the clinical characteristics of 17 cases of PISP, multivariate analysis revealed a negative outcome if radiotherapy was not performed.[
PISP is an exceedingly rare lesion and each case is valuable in terms of the accumulation of treatment experience. Here, we reported the first case of PISP exhibiting massive subcortical hemorrhage. Despite its rarity, solitary plasmacytoma should be considered among the etiologies that cause clinically significant intraparenchymal hemorrhage. It should be noted that careful examination to exclude multiple myeloma is essential, because the clinical course and treatment regimens differ substantially. Following appropriate surgical resection of PISP, radiation therapy is of utmost importance to achieve an optimal outcome.
The authors certify that they have obtained all appropriate patient consent.
There are no conflicts of interest.
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