Skeletal Radiol (2014) 43:1561–1565 DOI 10.1007/s00256-014-1930-6

SCIENTIFIC ARTICLE

Intramuscular plasmacytoma Alexey Surov & Anatolij Tcherkes & Frieder Meier

Received: 1 April 2014 / Revised: 27 May 2014 / Accepted: 28 May 2014 / Published online: 28 June 2014 # ISS 2014

Abstract Objective In multiple myeloma, secondary infiltration of adjacent muscles from bone lesions is common. However, plasmacytoma directly arising within the skeletal musculature is rare. Imaging findings of this rare entity have been described only sporadically. The purpose of this study was to identify the clinical signs and radiological features of intramuscular plasmacytoma (IP). Materials and methods Eleven patients with IP were retrospectively identified in the pathological and radiological databases of our institution. Computed tomography (CT) was performed in nine patients and magnetic resonance imaging (MRI) in four cases. Results IP presented clinically with local pain in four patients. In one case with involvement of the rectus lateralis muscle of the eye, the patient showed a painless bulbus proptosis. In another patient, IP manifested as a massive bilateral forearm swelling with compartment syndrome. In four patients, IP was identified incidentally on computed tomography during staging examination. On imaging, two patterns of IP were found: intramuscular mass (n=5) or diffuse muscle infiltration (n=6). On CT with contrast, IP showed a moderate enhancement. With MRI on T1-weighted images, IP was isointense in comparison to the unaffected musculature, whereas on T2-

A. Surov (*) Department of Radiology, Martin-Luther-University of Halle-Wittenberg, Halle (Saale), Germany e-mail: [email protected] A. Tcherkes Department of Hematology/Oncology, Martin-Luther-University of Halle-Wittenberg, Halle (Saale), Germany F. Meier Department of Pathology, Martin-Luther-University of Halle-Wittenberg, Halle (Saale), Germany

weighted images, IP showed high signal intensity. After intravenous administration of contrast medium, a slight-tomoderate inhomogeneous enhancement was seen in all cases. Conclusions IP should be considered in the differential diagnosis of muscle tumors. It manifests with two radiological patterns, either as intramuscular mass or as diffuse muscle infiltration. Keywords Muscle plasmacytoma . Radiological features . Computed tomography . Magnetic resonance imaging

Introduction According to the literature, five different forms of plasma cell malignant diseases are possible: multiple myeloma with diffuse infiltration of bone marrow, solitary plasmacytoma of bone, extramedullary plasmacytoma without bone marrow involvement, multiple myeloma with extramedullary manifestations, and plasma cell leukemia [1–4]. Multiple myeloma is the most frequently form of plasma cell malignancy [1, 2]. Isolated extramedullary manifestations are rare and represent approximately 5 % of all plasma cell neoplasms [1, 3–5]. Nonosseous extramedullary plasmacytoma can be solitary and multiple [1–3]. As reported previously, it affects the upper respiratory tract predominantly [3, 5]. In contrast to multiple myeloma, in extramedullary plasmacytoma there is no evidence of bone marrow plasmacytic infiltration and of other classic criteria of myeloma, such as hypercalciemia, renal insufficiency, and anemia [2, 3]. In addition, patients with extramedullary plasmacytoma have normal serum or urinary paraprotein level [2, 3]. Extramedullary plasmacytoma has the best prognosis of all plasma cell tumors [1–3]. However, according to the literature, conversion to multiple myeloma developed in 17–45 % of cases with extramedullary plasmacytoma [3, 4].

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Table 1 Cases with intramuscular plasmacytoma (IP) Case no.

Age

Sex

Diagnosis

Localization of IP

Radiological pattern

Clinical signs

1 2 3

63 54 71

F M M

MM MM MM

Mass Mass Infiltration

Incidental finding Incidental finding Pain

4 5 6 7 8 9 10

75 75 70 55 59 60 58

F F M F F F M

MM MM MM MM PEP MM PEP

Right erector spinae muscle Right gluteus maximus muscle Right gluteus maximus muscle, right erector spinae muscle Right intercostals muscle Forearm musculature bilateral Left latissimus dorsi muscle Right psoas muscle Abdominal wall musculature Right erector spinae Orbital rectus lateralis muscle

Mass Infiltration Mass Infiltration Infiltration Mass Infiltration

Incidental finding Compartment syndrome Incidental finding Pain Pain, swelling Pain Bulbus proptosis

11

60

M

MM

Right iliacal muscle

Infiltration

Pain

MM multiple myeloma; PEP primary extramedullary plasmacytoma

Secondary involvement of adjacent muscles from bone lesions is common in myeloma. However, direct formation of plasmacytoma within the skeletal musculature is rare. Muscle plasmacytoma as an extramedullary disease without systemic myeloma has been described only in isolated case reports previously [1, 6]. According Alexiou et al., the frequency of this event has been reported to be 0.6 % [1]. In multiple myeloma, additional extramedullary intramuscular manifestation occurred more frequently. For instance, according to the literature, intramuscular involvement in myeloma was diagnosed in 2.2 % [6]. Altogether, only a few cases of intramuscular plasmacytoma (IP) were reported in the literature [6–8]. Furthermore, imaging findings of this rare entity have been described only sporadically [6, 7]. Therefore, the aim of the present analysis was to identify the radiological features of IP in a small series of patients.

imaging parameters 120 kVp, 36 mAs, collimation 64 × 0.6 mm, and pitch 0.8.

Materials and methods Eleven patients with IP were retrospectively identified in the pathological and radiological databases of our institution (Table 1). Six patients were female and five were male. The median age was 60 years, range, 54–75 years, mean age 63.6 years. In six patients, computed tomography (CT) was performed with a multi-detector device (Somatom Sensation 64, Siemens, Germany) after intravenous application of iodinated intravenous contrast medium using the following imaging parameters: 120 kVp, 150–300 mA, collimation of 64 × 0.6 mm, and pitch 0.8 mm. In one patient, CT without contrast medium was done. In two further patients, low-dose computed tomography (LDCT) without intravenous administration of contrast medium was performed on the same CT scanner with

Fig. 1 CT without intravenous application of contrast medium (a) in a patient with a known history of multiple myeloma showing a slightly hyperdense mass (arrow) within the left latissimus dorsi muscle. Histological investigation (b hematoxilin & eosin stain, ×50) after surgical resection shows intramuscular focus of plasma cells

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Fig. 2 Imaging findings in a 70year-old patient with a known history of multiple myeloma and massive swelling of both forearms. a Low-dose CT documents enlargement of the forearm musculature (arrows). b On MRI, the forearm musculature (anterior and posterior compartiment on the right side and anterior compartiment on the left side) is enlarged and inhomogeneously hyperintense on T2w images with fat saturation (arrows). Additionally, moderate edema of the subcutis is also seen.

In four patients, MRI was performed using a 1.5-T MRI scanner (Magnetom Vision Sonata Upgrade, Siemens, Germany). The protocol included T2-weighted (T2w) images with and without fat saturation, and an axial T1-weighted (T1w) spin echo (SE) or turbo spin echo (TSE) sequences. Additional axial T1w SE/T1TSE and/or T1w SE/TSE fat-suppressed (FS) sequences were performed after intravenous administration of a paramagnetic contrast medium (gadopentate dimeglumine, Magnevist, Bayer Schering Pharma, Leverkusen, Germany) with a dose of 0.1 mg/kg. The slice thickness ranged between 3 and 5 mm. In all patients, the diagnosis of muscle plasmacytoma was confirmed histopathologically on muscle biopsy specimens.

Results In nine patients, intramuscular manifestation occurred in multiple myeloma with bone marrow involvement (Table 1). In these cases, the diagnosis had already been established before. Two cases had extramedullary disease without bone marrow involvement. IP presented clinically with local pain in four patients. In one case with involvement of the rectus lateralis muscle of the eye, the patient showed a painless bulbus proptosis. In another patient, IP manifested as a massive bilateral forearm swelling with compartment syndrome. In four patients, IP was identified incidentally on computed tomography during staging examination. IP was localized in different muscles (Table 1). In eight cases, only one muscle was involved, whereas in three patients

multiple muscle compartments were affected. On imaging, two patterns of IP were identified: intramuscular masses (n= 5) and diffuse muscle infiltration (n=6). The detected masses ranged in size from 20 to 40 mm with a median size of 25 mm. On CT, IP showed in all cases a slightly-to-moderate contrast enhancement. In the three patients, in which CT was performed without administration of contrast medium, the detected lesions were isodense in two cases and slightly hyperdense in one (Figs. 1a and 2a). With MRI, on T1w images, IP was isointense in comparison to the unaffected musculature (Fig. 2c). On T2w images, IP showed high signal intensity (Figs. 2b, and 3a). After venous administration of contrast medium, a moderate inhomogeneous enhancement was seen in all cases (Fig. 3b). Histopathologically, in the cases presented with intramuscular masses, plasmacytoma foci within the muscle were identified (Fig. 1b). In the patients with muscle infiltration, diffuse accumulation of plasma cells between the muscle fibers was seen (Figs. 2d and 3c). All patients with IP and multiple myeloma died because of progress of systemic disease. In the cases with isolated IP, in one patient multiple myeloma occurred 7 years later and one patient was disease-free to date after local radiation.

Discussion Two types of involvement of the skeletal musculature in m a l i g n a n t p l a s m a ce l l d i s e a s e s o c c u r : p r i m ar y extramedullary muscle plasmacytoma and intramuscular manifestation in systemic disease. In our patients, most

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ƒFig. 3

Imaging findings in a patient with extramedullary plasmacytoma. a MRI, T2w image documenting massive enlargement of the abdominal wall musculature (arrows). T1w image after intravenous administration of contrast medium (b) shows an inhomogeneous slight-to-moderate enhancement. Histological investigation (c) after biopsy reveals diffuse muscle infiltration by plasma cells

frequently IP presented as additional extramedullary manifestation in systemic myeloma. IP had no specific clinical signs and can manifest with local pain or swelling. In some patients, IP was identified incidentally on staging examinations. However, serious complications can also occur. In one of our cases, IP presented with compartment syndrome. This complication has been reported previously in muscle lymphoma [9]. On imaging, two patterns of IP were identified: intramuscular masses and diffuse muscle infiltration. Previously, it has been shown that muscle disorders can manifest with different radiological features [10]. For example, five different patterns of the radiological appearance of muscle metastases have been reported in the literature: focal intramuscular masses with homogeneous enhancement (type I), abscess-like intramuscular lesions with central low attenuation (type II), diffuse metastatic muscle infiltration (type III), metastases presenting with multifocal intramuscular calcification (type IV), and metastases manifesting as intramuscular bleeding (type V) (7). Type I and II occurred most frequently [11]. Intramuscular sarcomas manifest typically as large masses with central liquefaction or necrosis, board enhancement (type II lesions), and peritumoral edema [10, 12–15]. However, solid masses with homogeneous enhancement (type I lesion) and diffuse muscle enlargement (type III lesion) can also occur [10, 13–16]. Intramuscular lymphoma can also manifest as diffuse muscle infiltration or intramuscular masses [17–19]. In most cases, however, muscle lymphoma presents as muscle enlargement [17]. Previously, it has been reported that muscle lymphoma was localized most frequently in the lower extremities [17–19]. Furthermore, according to the literature, multiple muscle compartments were affected in muscle lymphoma [18, 10]. Typically, sarcomas were also located in the muscles of the extremities [12, 13]. In contrast to intramuscular lymphoma and sarcoma, muscle metastases tend to occur more often in the iliopsoas, paravertebral, and gluteal musculature [11]. In our series, muscle plasmacytoma occurred in the trunk musculature predominantly. In addition, in most cases, IP was localized within one muscle. In conclusion, IP should be considered in the differential diagnosis of muscle tumors. It can manifest with two radiological patterns, namely intramuscular masses and diffuse muscle infiltration.

Skeletal Radiol (2014) 43:1561–1565 Conflict of interest There are no conflicts of interest to disclose.

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Intramuscular plasmacytoma.

In multiple myeloma, secondary infiltration of adjacent muscles from bone lesions is common. However, plasmacytoma directly arising within the skeleta...
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