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Primary solitary plasmacytoma of the liver – successful treatment with fractionated stereotactic radiotherapy (Cyberknife®): a case report
© The Author(s). 2017
Received: 9 March 2017
Accepted: 19 June 2017
Published: 18 July 2017
Solitary plasmacytoma of the liver is a very rare and aggressive form of plasma cell dyscrasia. To the best of our knowledge, very few cases have been reported without systemic disease. We reported a rare case of hepatic solitary plasmacytoma that successfully responded to fractionated stereotactic radiotherapy.
A 64-year-old white French man had monoclonal gammopathy of the immune globulin G lambda type; he developed a cholestasis and cytolysis with the discovery of a subscapular nodule. A biopsy showed plasma cells and, for several reasons, the decision was made to use the fractionated stereotactic radiotherapy strategy. After 20 months, he is asymptomatic and the immune globulin G component has completely disappeared.
We suggest considering Cyberknife® radiosurgery as an option for the treatment of hepatic solitary plasmacytoma.
Solitary plasmacytoma (SP) is a rare form of plasma cell dyscrasia with a single bone (SBP) or extramedullary location (EMP). It accounts for 3 to 5% of all plasma cell neoplasms . Almost 85% of EMPs involve the head and neck mucosa, especially in the upper respiratory tract . Gastrointestinal, lung, bladder, and testis involvement have also been reported. Only a few cases of hepatic SP have been reported to date [3, 4]. The presence of plasma cells in the liver or spleen is known to be associated with a more aggressive form of multiple myeloma  that usually requires a heavy treatment with chemotherapy or consolidation therapy with autologous hematopoietic stem cell transplantation.
Radiotherapy is the treatment of choice for EMP, with excellent response rates of approximately 86% . Fractionated stereotactic radiotherapy has been used for approximately two decades to deliver ablative high-dose irradiations to small target volumes and low-dose irradiations to adjacent normal tissue. It plays an increasingly important role in the therapeutic arsenal of primary and secondary liver tumors but, to the best of our knowledge, its use has never been described in the treatment of hepatic plasmacytoma. We present a rare case of hepatic SP that successfully responded to a treatment with Cyberknife®.
We describe a very good partial response of a very rare form of hepatic EMP with fractionated stereotactic radiotherapy. The diagnosis was made according to the recently updated recommendations , and differential diagnoses such as other neoplasms  were ruled out by histologic examination.
Radiation therapy is the best option for patients with localized EMP over systemic chemotherapy. Recommendations include a radiotherapy dose of 40 Gy in 20 fractions for tumors <5 cm and up to 50 Gy in 25 fractions for tumors ≥5 cm with at least a 2-cm margin encompassing the primary tumor . Other alternatives exist like surgery or combined chemotherapy treatment, but are not justified outside clinical trials. Hepatic plasmacytomas without systemic disease have been treated either by chemotherapy (melphalan/steroids or vincristine, adriamycin, and dexamethasone combinations) or by radiotherapy . Only a few cases of SP treated by fractionated stereotactic radiotherapy have been reported, and our case is the first reported with a hepatic localization. Wong et al. reported a plasmacytoma of the clivus extending to the foramen magnum treated by fractionated stereotactic radiotherapy with a complete and durable response on MRI at 12 months . Theiler et al. reported the case of a 49-year-old man with SP of the bone, with radiotherapy as first-line treatment and radiosurgery for the treatment of local relapse with disappearance of bone lesion . Other cases reported Cyberknife® success in plasmacytoma in neurological localizations such as cavernous sinus , cranial , and spine cord . To the best of our knowledge, this is the first case of successful fractionated stereotactic radiotherapy use in hepatic SP. The absence of toxicity observed in this unusual observation suggests that other patients with similar presentations could benefit from this treatment modality.
This case shows that stereotactic radiotherapy could be highly effective in localized liver plasmacytoma. The Cyberknife® technology, which combines high precision and surrounding tissue protection, could be an alternative to conventional radiotherapy in some cases [11, 12]. Due to the lack of guidelines and clinical trials, the indication of fractionated stereotactic radiotherapy for the treatment of plasmacytoma should be further studied. In our case, the radiographic response was almost complete, safety was satisfactory, and our patient is still asymptomatic after 20 months. We suggest considering fractionated stereotactic radiotherapy as an option for the treatment of hepatic SP.
Availability of data and materials
Data sharing is not applicable to this article as no datasets were generated or analyzed during the current study.
TC collected the data and wrote the manuscript; IB and LB treated the patient and approved the manuscript; EG wrote and approved the manuscript; JPB and FA provided and analyzed tissue sections; MSR, MB, OH, and EP contributed essential tools. All authors read and approved the final manuscript.
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Consent for publication
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal
The authors declare that they have no competing interests.
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