Primary pulmonary plasmacytoma: a case report introduction
© The Author(s). 2016
Received: 10 April 2016
Accepted: 13 July 2016
Published: 4 August 2016
Extramedullary plasmacytoma is a rare plasma cell neoplasm within soft tissue and without bone marrow involvement or other systemic characteristics of multiple myeloma. Primary pulmonary plasmacytoma is a rare type of extramedullary plasmacytoma.
A 48-year-old male with a tumor in the right middle ear was referred to our hospital. A routine chest X-ray was arranged and showed enlargement of the left lung hilum. His bilateral breathing sounded clear. A chest CT scan revealed a well-circumscribed mass. Pathological biopsy yielded a diagnosis of isolated pulmonary plasmacytoma.
This is the first presentation of primary pulmonary plasmacytoma with a solitary pulmonary nodule and no lymph node involvement.
Summary of the literature on the clinical treatment and prognosis of primary pulmonary plasmacytoma
Nozomi Niitsu, 2005 
A tumor in the right middle lobe
After completing three courses of the therapy, considerable diminution in the size
Montero C, 2009 
A tumor in the left main bronchus and enlarged subcarinal lymph nodes
Surgical and radiotherapy
Currently asymptomatic and has remained disease free during a follow-up of 10 years
Montero C, 2009 
A mass in the right upper lobe
A disease-free period of 15 years followed
Montero C, 2009 
A mass and reduced right upper lobe volume
Radiotherapeutic and adjuvant chemotherapy
Developed fever and signs of septic shock during the third cycle and died
Geetha Joseph, M.D. 
A right hilar mass
Right middle lobectomy
Sang-Heon Kim, 2012 
Infiltrative lesions in both lower lung fields
Near complete radiological resolution was observed after six cycles of treatment
Z. Mohammad Taheri, 2010 
Bilateral alveolar consolidation
After four monthly courses, the chest X-ray became normal
Shi-Ping Luh, 2007 
Right anterior mediastinal shadow with multiple pulmonary nodular lesions.
Surgical and chemotherapy
Symptoms improved after 2 months of treatment
Takahiro Horiuchi, MD, 1998 
Massive parenchymal infiltrate in the lower lobes
After four monthly courses, the chest X-ray became normal
Lenara Renó Arbex Coelho, 2015 
Ovoid opacity in the right hilar region
After 3 years, no suspect finding of disease recurrence/progression
James N. Wise, 2001 
A right hilar mass
A right thoracotomy with right upper lobectomy
Fifteen months postoperatively, without evidence of recurrence
A 48-year-old male with a tumor in the right middle ear was referred to our hospital. A routine chest X-ray was arranged and showed enlargement of the left lung hilum. His vital signs were as follows: blood pressure 117/75 mmHg, pulse 93/min, breathing 20/min, body temperature 36.3 °C. There was no systemic or superficial lymph node enlargement, sinus area tenderness, or swollen tonsils. The trachea was in the mid-line. The patient had a barrel chest. His bilateral breathing sounds are clear. Bronchoscopy revealed no obvious abnormal findings. The patient underwent a series of evaluations such as serum calcium, urine Bence-Jones protein, and plasma electrophoresis for M protein detection. However, all of the above tests were negative. A bone marrow biopsy revealed normal patterns of cell distribution. He had an approximately 10-year history of smoking. He had a tumor in the right tympanic cavity surgically removed with a postoperative pathological diagnosis of extramedullary plasmacytoma. He had no history of tuberculosis (TB). No family members had any similar clinical manifestations nor had any died of similar diseases.
A CT-guided needle aspiration biopsy specimen showed that, microscopically, plasma cells were situated in the fibrous tissue, and the cells were larger than normal with Russell bodies. Small blood vessels were also revealed (Fig. 1d); immunohistochemical kappa (+++) predominate, lambda (−), CD20 (−), CD79a (−), CD138 (+++), CD38 (+++). Pathological biopsy indicated isolated pulmonary plasmacytoma.
The patient was treated with chemotherapy alone. After 6 months, the chest X-ray became normal and the patient was free of symptoms. The patient remained disease free during a follow-up of 1.5 years. Now, he is still in follow-up.
Plasma cell neoplasms can be classified into the following types: multiple myeloma (bone marrow and other systemic involvements), solitary myeloma (bone plasmacytoma), extramedullary (soft tissue) plasmacytoma, and plasmablastic sarcoma .
EMP affects males three to four times more often than females, with an average age of 55. However, one third of patients with EMP are under 50 years old. The etiology of the disease is not well understood, but viral pathogenesis and chronic irritation are suggested to be contributing factors . It presents with non-specific symptoms, such as chronic cough, dyspnea, wheezing, huskiness, or hemoptysis. The presentation is closely related with lesion location. Violation of the trachea can result in a series of symptoms such as breathlessness and difficulty of breathing; bronchial tumor invasion may appear as blood in phlegm or hemoptysis. If there is any involvement of the pleura, the patient may experience symptoms such as chest pain and systemic symptoms are not obvious. In this case, the patient may not exhibit symptoms for a long time, have no obvious symptoms of cough, or coughing up phlegm or symptoms such as chest pain. The clinical staging of PPP was generally based on the Wilshaw method, divided into three stages as follows: stage I: the tumor is confined to the primary site; stage II: the tumor has invaded local lymph nodes; stage III: there are obvious widespread metastases. Therefore, in this case, the clinical staging should be classified as stage I.
Solitary extramedullary plasmacytoma of the lung is extremely rare; pulmonary involvement with multiple myeloma is more common.
To differentiate solitary EMP from multiple myeloma, bone marrow examination is required. The patient should have lower than 5 % plasma cells with no dyscrasia and a normal skeletal survey. Unlike multiple myeloma, EMPs may not have serum M protein or Bence-Jones light chains in the urine. After treatment, the review should detect the M protein level, and testing a bone marrow is essential for ruling out multiple myeloma.
PPP microscopically showed dense plasma cells and different levels of diffuse proliferation and infiltration. Mature and relative mature nuclei are round or ovoid, of the same size, often offset, with spoke-shaped chromatin and generally no nucleoli. Nuclear fission is rare and cytoplasm is abundant. These cells are primarily basophilic and the minority as eosinophilic. The nucleus is peripheral or one side is surrounded by perinuclear halos. Irregular immature plasma cells have a low nuclear membrane thickness, scattered chromatin n spoke-shaped arrangement, and clear dual-core nucleolus. Nuclear fission is common. Cells have less cytoplasm and are basophilic or eosinophilic. Immunohistochemical staining reveals single light chain expression, namely kappa lambda (−) or (+) or kappa lambda (+/−) predominate. Tumor cells were CD20 (−), CD79a (++), CD138 (++), and CD38 part (+) . These features are in accordance with the typical histopathological characteristics of PPP. The immune phenotype is highly specific for the diagnosis of plasmacytoma, especially the expression of CD138.
Image diagnosis and differential
CT diagnosis and differential diagnosis of PPP
Peripheral lung cancer
Nodular focal organizing pneumonia
Fine burr shadow
Short hard burrs
Long burrs, satellite lesions
Peripheral visible burrs
Relatively uniform density, occasionally empty
Most uneven, jagged hole in the inner wall
Calcification, hollow with smooth inner wall
Uneven density, pus cavity
Enhanced CT scan
Moderate uniform reinforcement
No or light enhancement
Uniform or delayed enhancement
Subpleural focal fatty infiltration
Adjacent pleural thickening
In this case, the presentation was isolated lung nodules, lobulated, rim visible, fine burr shadow, and pleural retraction and was initially misdiagnosed as peripheral lung cancer. However, that condition is often characterized by deep lobules, short hard burrs, accompanied by jagged cavity, and lesions containing stiff gas bronchial wall that may be truncated, and involvement of the pleura can blur the subpleural fat gap. An enhanced CT scan showed obvious strengthening at approximately 20~60 HU.
In this case, the lesion location in left lower lobe dorsal segment was characterized by isolated soft tissue nodular shadows, differentiating it from tuberculosis. Tuberculoma often presents with calcification, more satellite lesions around the nodule, hollow with smooth inner wall, and the enhanced scan is no or light improved. There are clinical case reports of PPP lesions with calcifications , so calcification does not exclude the possibility of PPP. Therefore, a clinical laboratory examination is necessary for the diagnosis of tuberculosis.
In this case, it showed an isolated lung lesion with a less clear boundary, which also differentiates it from nodular focal organizing pneumonia. The edge of focal organizing pneumonia can be clear or fuzzy, its lesions have uneven density, liquefied necrotic tissue forms hollows, and it presents with peripheral visible burrs. Enhanced scanning can show lesions uniform or peripheral enhancement with adjacent pleural thickening and pus cavity.
Treatment and prognosis
Primary pulmonary plasmacytoma consisting of a solitary lesion is usually treated by either surgical resection alone or resection followed by radiation therapy. But the previous three cases of diffuse pulmonary infiltration and one case of multiple lung nodules, as well one case of a tumor in each lung underwent combination chemotherapy including melphalan and prednisolone [5, 9, 10]. In this case, because of the small size of the lesion, we recommended chemotherapy to patients who previously had a history of surgery and in poor health.
We presented a case of PPP with an isolated lung nodule and no lymph node involvement. An isolated lung nodule showed superficial lobe, fine burr shadow, pleural retraction, and moderate uniform reinforcement; we should take into account the possibility of PPP. When the mass is small, not appropriate for operation, we can put chemotherapy as the preferred solution.
CT, computed tomography; EMP, extramedullary plasmacytoma; PPP, primary pulmonary plasmacytoma; TB, tuberculosis
The authors are grateful to the subjects who participated in this research. This work was supported by the Natural Science Foundation of China (grant number 81560285).
This work was supported by the Natural Science Foundation of China (grant number 81560285).
Availability of data and materials
The main materials of this study were the patient’s images. In order to avoid disclosure of patient information, we will not provide all of the data. But if you need to verify the data or other reasonable purposes, we will be glad to provide you all of the data.
SN provided the literature review and drafted the manuscript. D-CP has given the final approval of the version to be published. H-HG completed the clinical part of the manuscript. C-LY provided the imaging diagnosis, collaborated with the completion of the imaging part of this manuscript, and completed the clinical part of the manuscript. XN and H-JL provided the imaging diagnosis and collaborated with the completion of the imaging part of this manuscript. All authors read and approved the manuscript.
The authors declare that they have no competing interests.
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.
Ethics approval and consent to participate
The present study was approved by the ethics committee of The First Affiliated Hospital of Nanchang University. Written informed consent was obtained from the patient for participating in our study.
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