Rare solitary neoplasm of the costa in an adult: a case report
© Shen et al.; licensee BioMed Central Ltd. 2014
Received: 1 January 2014
Accepted: 6 August 2014
Published: 25 September 2014
Regional solitary plasmacytoma of the costa is a rare disease and is characterized by only one or two isolated bone lesions. We report the case of a solitary plasmacytoma of the bone of the right chest wall in an adult. The patient underwent complete enbloc resection of the chest wall including ribs, muscle and parietal pleura. The patient is asymptomatic without any recurrence after two months of follow-up.
Primary malignant tumors of the bony chest wall are rare and localized solitary plasmacytoma of bone (SPB), characterized by only one or two isolated bone lesions with no evidence of disease dissemination, is a rare disease that accounts for about 5% of malignant plasma cell tumors. We report the case of SPB in the right chest wall.
Plasmacytoma involving the chest wall is a rare primary tumor classified as multiple myeloma (MM), solitary plasmacytoma, extramedullary plasmacytoma or plasma cell leukemia. More than 95% of these tumors are MMs. A solitary plasmacytoma in a rib usually shows destruction of the bone cortex with extension into the surrounding soft tissues. Plasmacytoma may be primary or secondary to the disseminated multiple myeloma and may arise from the osseous or non-osseous sites.
The ratio of male to female patientsis approximately 1.3:1 and the average age on presentation is 59.5 years with a range from 39 to 77 years. The most common symptoms are pain and spinal nerve compression. In our case, the patient (who was a 54-year-old male) had a symptom of constant pain in the chest on the right side with exacerbation upon coughing. With regard to radiological evaluation, chest radiographs are the most commonly performed imaging analysis to evaluate lumps, but may not always provide a conclusive diagnosis. In such cases, a spiral CT is the next tool of choice. Plasmacytoma is typically seen on a CT scan as well-defined, ‘punched-out’ lytic lesions with associated extrapleural soft tissue mass. In advanced plasmacytoma, there is often marked erosion, expansion and destruction of bone cortex, sometimes with thick ridging around the periphery, creating a ‘soap bubble’ appearance. There is also a word in our case. The patient’schest X-ray with lateral projection clearly showed a pulmonary lump and his contrast-enhanced CT revealed a well-defined hypodense soft tissue mass on the right side of the chest wall with invasion into the third rib. The differential diagnosis of costal plasmacytoma includes chondrosarcoma, lymphoma, metastases, osteosarcoma, fibrosarcoma, histiocytoma, chondroma, lipoma and bone infarction.
The purpose of the treatment of SPB is to prevent transformation into MM and recurrence. In the past, radiation therapy was used as the primary treatment for SPB because this tumor is sensitive to radiotherapy. Although there is no established relationship between dosage and response; 40 to 50 Gy radiation in 20 to 25 fractions has usually been recommended. Mendenhall et al. reported a 6% local failure rate in patients with solitary plasmacytoma treated with doses of 40 Gy or above, in contrast to a 31% failure rate for doses below 40 Gy. Aviles et al. discerned that most patients treated with adequate radiation therapy alone will develop MM within the first three years after diagnosis and treatment. When performing surgical resection, the possibility of structural instability and nerve injury should be considered for spinal lesions. For other lesions, variable surgical strategies can be applied according to the tumor size, extent, patient’s general condition or surgeon’s experience. One group reported that the primary methods for treating solitary plasmacytoma were surgery plus radiation therapy in 95 cases and surgery alone in 15 cases. They showed the lowest incidence of progressive diseases in patients with solitary plasmacytoma who were treated with surgery plus an adequate dose of radiation therapy. In our case, we had referred the patient firstly for a wide excision of a solitary rib tumor containing normal tissue without preoperative confirmation of diagnosis. The histopathologic diagnosis of SPB was confirmed, and a treatment plan with doses of 40 Gy radiation in 25 fractions was planned for protection against tumor recurrence.
In conclusion, there are very few reports in the literature depicting the natural history of SPB in an adult in regard to its presentation, pathology and treatment. Our case may help recognition of this rare disease in the thoracic surgery field, thus avoiding misdiagnosis and inadequate treatment.
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.
We greatly appreciate the assistance of the staff of the Department of Thoracic Surgery and Department of Pathology, West-China Hospital, Sichuan University, and thank them for their efforts.
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