|LETTER TO THE EDITOR
|Year : 2015 | Volume
| Issue : 3 | Page : 428-429
A rare case of extramedullary plasmacytoma arising from posterior mediastinum
D Gupta1, J Goyal2
1 Department of Medical Oncology, Bhagwan Mahaveer Cancer Hospital and Research Center, Jaipur, Rajasthan, India
2 Department of Radiation Oncology, Bhagwan Mahaveer Cancer Hospital and Research Center, Jaipur, Rajasthan, India
|Date of Web Publication||18-Feb-2016|
Department of Medical Oncology, Bhagwan Mahaveer Cancer Hospital and Research Center, Jaipur, Rajasthan
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Gupta D, Goyal J. A rare case of extramedullary plasmacytoma arising from posterior mediastinum. Indian J Cancer 2015;52:428-9
Extra medullary plasmacytoma (EMP) is a plasma cell tumor, which involves soft-tissues, without any signs of systemic involvement. It may originate in any site although most frequently it occurs in the upper respiratory tract and oral cavity., Rarely may it present with mediastinal mass lesion without any other organ involvement.
A 43-year-old male presented to us with a history of mediastinal mass for 2 years although patient was asymptomatic. Before presenting to us he had a history of cough and breathlessness 2 years back, for which chest X-ray was carried out elsewhere, which showed mediastinal widening with small heterogeneous patch in the left lower lung. He was advised contrast enhanced computed tomography (CECT) of chest for that which showed mediastinal mass. Subsequently, he underwent computed tomography guided fine needle aspiration cytology, which was inconclusive so repeat biopsy was performed, which also was inconclusive. His bronchoscopy also showed no mass lesion and bronchoalveolar lavage showed only chronic bronchitis. He received conservative treatment and was kept on follow-up. One year later repeat CECT scan was carried out, which showed mediastinal mass of the same size and location. His serum alpha fetoprotein and beta-human chorionic gonadotropin levels were normal. At current presentation, his latest CECT of the chest showed infiltrating mass in the middle and posterior mediastinum encasing trachea, carina and both main stem bronchi and esophagus without intraluminal extension. Bilateral lung fields were normal and there was no pleural effusion [Figure 1]a. This time, repeat biopsy was attempted in view of persistent mass lesion although patient had no symptoms. Repeat biopsy showed plasma cell tumor with CD 38/138 positivity and cytokeratin/CD20 negative. His beta2-microblobulin was 1.91 mg/L and serum protein electrophoresis showed an M band of 4.94 g/dL. His bone marrow aspiration and biopsy did not show any infiltration by plasma cells and he had no organ dysfunction required for multiple myeloma work-up. He was treated with external beam radiation therapy, 50 Gy in 25 fractions over 5 weeks. After 2 months of radiation, his CECT chest showed a significant reduction in mass lesion [Figure 1]b and his serum protein electrophoresis showed M spike of 1.12 g/dL. He has been kept on follow-up at a regular interval.
|Figure 1: (a) Computed tomography scan of thorax in sagittal section and coronal section; (b) showing mass lesion in posterior mediastinum predominantly encasing trachea and esophagus|
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EMP accounts for approximately 3% of plasma cell neoplasms and it occurs in the upper aero-digestive tract in 80% of patients, mostly in the nasal cavity, paranasal sinuses, larynx, tonsils. Additional characteristics are male predominance and advanced age, usually above 40 years, at presentation., Diagnostic criteria of EMP are tissue biopsy indicating monoclonal plasma cell infiltration, bone marrow plasma cells <5% of nucleated cells and absence of increased calcium, renal failure, anemia and multiple bone lesions features. Some patients may have serum M band, but usually it is of low quantity., Rarely EMP may present with primary mediastinal mass lesions with or without lymph nodes or lung lesions.,,,, Usually, these patients with mediastinal mass are having minimal symptoms of chest discomfort, cough or mild breathlessness as was in our case, but they may have compressing symptoms of superior vena cava syndrome, myasthenia gravis due to thymus involvement, pleural effusion  or may be diagnosed coexistent with multiple myeloma. EMP are highly radiosensitive with 80-20% local control rates and approximately 50-65% patients remain free of disease at 10 years., Surgery is usually not feasible in the head and neck area, but surgery may be considering for other sites like gastrointestinal sites. Considering the high cure rates of EMP with radiotherapy and lack of adequate data on chemotherapy, radiotherapy remains the standard of care and adjuvant chemotherapy should be reserved for non-responders or relapses to myeloma.
To conclude, EMP arising in mediastinum is a rare entity and radiotherapy remains the mainstay of therapy.
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