Journal of Otology & RhinologyISSN: 2324-8785

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Case Report, J Otol Rhinol Vol: 3 Issue: 6

Persistent Plasma Cell Tumor of the Nasopharynx after Radiotherapy Treatment: A Case Report and Review of the Literature

Elena Pont Colomer1*, Miguel Mazón2, Juan Del Campo2 and Manuel Viel2
1Department of Otorhinolaryngology, Head and Neck surgery, Hospital San Francisco de Borja, Gandia, Valencia, Spain
2Department of Radiology, Hospital La Fe, Valencia, Spain
Corresponding author : Elena Pont Colomer
Arzobispo Mayoral 11-7 46002 Valencia
Tel: +34646699411
E-mail: [email protected]
Received: June 22, 2014 Accepted: August 23, 2014 Published: August 27, 2014
Citation: Colomer EP, Mazón M, Campo JD, Viel M (2014) Persistent Plasma Cell Tumor of the Nasopharynx after Radiotherapy Treatment: A Case Report and Review of the Literature. J Otol Rhinol 3:6. doi:10.4172/2324-8785.1000189

Abstract

Persistent Plasma Cell Tumor of the Nasopharynx after Radiotherapy Treatment: A Case Report and Review of the Literature

Extramedullary plasmacytoma of the head and neck region is a rare malignant tumor comprising approximately 3% of all plasma tumors. Approximately 80-90% involve the mucosa of the upper airway. These tumors are known to be very radiosensitive and therefore radiotherapy is considered the treatment of choice, surgery being limited to biopsy and to excision residual disease. A case of extramedullary plasmacytoma of the nasal cavity is reported wich did not respond to radiotherapy.

Keywords: Extramedullary plasmacytoma; Plasma cell tumor; Nasal tumor; Tumor persistent after radiation

Keywords

Extramedullary plasmacytoma; Plasma cell tumor; Nasal tumor; Tumor persistent after radiation

Introduction

Extramedullary plasmacytoma (EMP) is a plasmatic tumor characterized by monoclonal expansion of plasma cells that produce a single immunoglobulin molecule [1].
The EMP was first describing in 1905 by Schridde, but it was in 1952 when Ewing described a large series of cases [2]. The estimated global incidence of the disease is 1 case per 500.000 people.
It is important for the otolaryngologist to acquire knowledge of this disease since 80 to 90% of the EMP cases are found in the head and neck and they represent only 1% of all cancers in this area. These tumors mainly occur in the upper respiratory tract due to its rich lymphatic tissue, especially the submucosa of the nasal cavity, paranasal sinuses and nasopharynx [3].
To exclude multiple myeloma or plasmacytoma of the bone, a systemic work-up and follow-up of the patient are mandatory, including serum protein electrophoresis, urinalysis for the Bence- Jones protein, skeletal survey and bone marrow biopsy [4,5].
Diagnosis can only be made after histopathological studies and immunohistochemical methods. Regarding the treatment of extramedullary plasmacytoma in the head and neck, the choice of radiotherapy or surgery alone is controversial. The primary method of treatment, if lymph node are not affected, is radiation therapy. The role of surgery is generally limited to open biopsy for diagnosis or for debulking a large tumor [1,6].
We present a patient diagnosed with an Extramedullary Plamacytoma in the nasal cavity without clinically and imaging detectable reduction of the tumor size after radiation that underwent complete resection of the tumor by endonasal endoscopic surgery control.

Case Report

A seventy-one year old male patient presented a 2-year history of intermittent epistaxis with right side nasal obstruction. Nasopharyngeal examination revealed a smooth surface tumor obstructing the nasopharynx and introduced into right nasal cavity. The blood vessels were visible owing to their transparency and bleeding. The tumor seemed to be fixed to posterior wall of the nasopharynx and was very painful (Figure 1). There was any cervical lymphanedopathy clinically.
Figure 1: Endoscopic vision of the lesion from right and left (A,B) nasal cavity.
Non contrast computed tomography (CT) of the nose and paranasal sinuses revealed a soft tissue tumor projecting from right nasal cavity into nasopharynx (Figure 2). MRI showed a well-defined lesion, relatively homogeneous without areas of necrosis, cysts, calcifications or bleeding, within the right nasal cavity extending posteriorly into the nasopharynx that reduced significantly the airway.
Figure 2: Pre-radiation imaging. Coronal and axial non contrast CT (A,B) show a soft tissue polypoidal mass projecting from right nasal cavity into nasopharynx that significantly reduces tha airway.
The histological finding, following nasal endoscopy biopsy, was plasmacytoma CD138+, CD79+ with light Kappa chain. A systemic work-up to exclude multiple myeloma (MM) was performed. Results of serum electrophoresis and urine test were negative for myeloma component or Bence-Jones protein. Bone marrow needle biopsy and skeletal survey were negative.
The patient received irradiation of 40Gy during 27 sessions without clinically and imaging detectable reduction of tumor size. Due to these results, a new biopsy was performed at this time to confirm the diagnosis. Exhaustive control is performed for 2 years with analysis every 3 months and Magnetic Resonance every 6 months (Figure 3). Finally a surgical approach was decided by endonasal endoscopic control with complete resection and safety limits. The tumor was adhered to inferior turbinate, the floor nasal cavity, soft palate and posterior septum. It is suppose the adhesion was secondary to radiation. At 1 year follow-up, the patient was doing well with no signs of recurrences.
Figure 3: Postradiation imaging showing same tumor size. Axial MR T1 weighted image and T2 weighted image (A, B). Axial and sagittal post-contrast T1WI (C, D) show an homogenous enhancement of the mass.

Discussion

Plasma cells are mature immunocompetent cells derived from B lymphocytes, and they provide specific noncellular immunity within the immune system. They produce specific antibodies against antigens in different tissues. Large populations are seen in the mucosa of the nasal and paranasal sinuses. Plasmacytomas arises from a neoplastic proliferation of theses antibody producing plasma cells [7].
The most common location is the nasal cavity, sinuses and nasopharynx (75%). Other locations are oropharynx (12%), larynx (8%) and tongue, thyroid and minor salivary glands (5%) [4]. Men are predominantly affected, and these tumors are most commonly seen in the sixth to eighth decades, as in this case. Due to its presentation in the submucosa of the aereodigestive tracts, some authors have suggested that the aetiology of EMP may be related to chronic stimulation caused by inhaled irritants or viral infection [8]. In our case, the patient worked in a bank and has no past related infections. Other publications propose risk factors that cause chronic antigenic stimulation such as osteomielitis, cholecystitis, rheumatoid arthritis and bacterial flora [9].
EMP arising in the head and neck characteristically presents with localized disease and without clinical lymph node involvement. These observations are in agreement with most of the large reported series [10].
On gross examination, the masses are usually polypoid but can be sessile. They tend to appear granulomatous and red although some are yellow-gray, gray or dark brown. They are vascular and bleed easily and profusely. The most frequent clinical symptoms are obstruction of the respiratory tract due to the tumor growth, epistaxis, rhinorrhea, pain and swelling of the soft tissue of the neck [1,2].
Fine needle aspiration is non-diagnostic because of the limited tissue available for special staining and for complete histologic examination. Therefore, incisional or excisional biopsy, depending on the size and location of the mass is necessary.3,10 Deep biopsies must be taken as the tumor is submucosal and the mucosa may be thickened as the result of an inflammatory reaction. Microscopically it appears as mononuclear proliferation of plasmatic cells in a dispersed array. It can have atipia nuclear and cellular minimal or prominent. Plasma cells have a core of dense chromatin along the nuclear membrane with form of wheel of wagon. The cytoplasm is abundant, basophilic and appears a perinuclear halo corresponding to the Golgi apparatus.
Given this result pathology should do more tests to rule out systemic disease and confirm the diagnosis of extramedullary plasmacytoma. The study was performed with hemogram, myelogram, bone marrow biopsy, complete biochemical profile, calcium, serum proteins and plasma protein electrophoresis and urine, and x-ray to exclude lytic lesions [6,11].
The differential diagnostic evaluation often is very difficult because of the similarity of the histologic appearance of EMP to that of others tumors like lymphoma, undefferentiated carcinoma, esthesioneuroblasts [1].
The optimal management of EMP is controversial with therapeutic choices resting between radiotherapy or surgery. There have been no randomized controlled trials to define the best treatment, but there are several small retrospective case series that helped us to decide the best option to our patient.
Radiotherapy is accepted as the standard treatment for EMP of head and neck because of its high radiosensitivity. Published data supports high responses rates of 90-95% after radiotherapy and long-term local control rates of 90-100% [11]. The optimal dosage regimen for radiotherapy is a matter of debate. Various protocols have been described, with a total dose ranging from 3.000 to 8.000 rads over treatment periods of 3 and 6 weeks [1,12]. Other authors administer dose of radiotherapy depending on the tumour size (for EMP up to 5cm a dose of 40Gy in 20 fractions and for EMP bigger than 5cm a higher dose of up 50Gy in 25 fractions). Cervical nodes respond to the same sensitivity to radiotherapy. Since now surgery was recommended in cases of localized lesions that could be removed with minimal morbidity.
Publications described to date accepted a 50% of tumor reduction, only another publication and our actual case show no response to radiotherapy [13].
In general, EMP of the head and neck has a better prognosis than solitary bone plasmacytoma or MM. This may be due to early presentation, when the lesions are small and well localized and also probably due to easy accessibility of the tumor to examination [11].
The evolution of EMP to MM is the determinant factor for survival. Only 10% to 30% of cases progress to MM within 2 years, but the prognosis for these patients is poor. Several factors predictive of progression to MM have been reported, such as the size of the tumor at diagnosis, total serum protein level, and a monoclonal spike observed on serum protein electrophoresis. Because recurrence is usually within 24 months but may be as late as 15 years after treatment, long-term follow-up, including not only local control but also systemic observation via blood counts and immunoglobulin measurement, is necessary [1,14].
Although the follow-up is not sufficiently long to consider the complete remission of disease as definitive, the therapeutic strategy adapted to date, in this case, might be considered valid.

Conclusions

Extramedullary plasmacytoma is a rare tumor which predominantly occurs in the submucosa of the upper aerodigestive tract. The majority of the patients are elderly and present with low-grade tumours. These low-grade tumours should be treated with radiotherapy to the primary site with a dose equivalent to 35-45 Gy in 3 weeks. The dose will depend on volume of treatment and inclusion of critical structures but is well tolerated. Most publications indicate excellent results after radiotherapy. The clinical importance of this case lies in the lack of response to radiation, coinciding to the present with a unique similar case published in 2011. Since now surgery was recommended in cases of localized lesions that could be removed with minimal morbidity, we also indicate surgery when radio-resistant cases. Conversion to MM is significantly lower with EMP compared with solitary plasmacytoma of bone, this being its main prognosis factor.

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