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Case report

Extramedullary plasmacytoma of the cricoid cartilage with solitary plasmacytoma of the rib☆ Grace C. Haser, BA a,⁎, Henry K. Su, BA a , Michael J. Pitman, MD b , Azita S. Khorsandi, MD c a b c

Thyroid Head and Neck Cancer (THANC) Foundation, Mount Sinai Beth Israel, 10 Union Square East, Suite 5B, New York, NY, USA Department of Otolaryngology–Head and Neck Surgery, New York Eye and Ear Infirmary, 310 East 14th Street, New York, NY, USA Department of Radiology, Mount Sinai Beth Israel, 10 Union Square East, New York, NY, USA

ARTI CLE I NFO

A BS TRACT

Article history:

Background: Extramedullary plasmacytoma is a rare plasma cell neoplasm of the soft

Received 12 February 2015

tissues characterized by the presence of a single, discrete lesion without evidence of systemic disease. Extramedullary plasmacytoma may disseminate into multiple myeloma, a systemic plasma cell disease. Methods: A rare case of extramedullary plasmacytoma of the cricoid cartilage with solitary plasmacytoma of the rib was reviewed. Results: The patient was found to have two discrete lesions; one of the cricoid cartilage and one of the lateral left fifth rib. Despite the presence of multiple tumors, the patient was not diagnosed with multiple myeloma as the bone marrow appeared normal. Conclusions: Due to the rarity of these neoplasms and the unusual localization of the extramedullary plasmacytoma tumor, a definitive diagnosis was difficult to make in this case. This case may be instructive for the differential diagnosis of laryngeal lesions. © 2015 Elsevier Inc. All rights reserved.

1.

Introduction

Plasma cell neoplasms can be classified into three major categories: multiple myeloma, extramedullary plasmacytoma, and solitary plasmacytoma of bone. These neoplasms represent approximately 1% of all malignancies of the head and neck region [1]. Multiple myeloma results from abnormal B cell proliferation in the bones and bone marrow and is characterized by the presence of multiple bony lesions and plasma cell infiltration of the bone marrow [2,3]. Multiple myeloma can damage multiple organ systems and lead to organ failure [3].

Extramedullary plasmacytomas are plasma cell tumors of the soft tissues and cartilage [4]; the most common sites of involvement are the aerodigestive tract and the oral cavity, although other sites have been reported [5]. Patients with extramedullary plasmacytomas do not have evidence of multiple myeloma, although they may develop multiple myeloma as the disease progresses [5]. Rather, these patients present with a single, discrete lesion of the soft tissue or cartilage and have bone marrow without significant plasma cell infiltration [2]. Similarly, solitary plasmacytoma of the bone is characterized by a single bony lesion without evidence of bone marrow infiltration [2]. These patients do not present



Conflicts of Interest: No conflicts of interest or financial disclosures. ⁎ Corresponding author at: Thyroid Head and Neck Cancer (THANC) Foundation, 10 Union Square East, Suite 5B, New York, NY 10003. Tel.: + 1 212 844 6441; fax: +1 212 844 8465. E-mail addresses: [email protected] (G.C. Haser), [email protected] (H.K. Su), [email protected] (M.J. Pitman), [email protected] (A.S. Khorsandi). http://dx.doi.org/10.1016/j.amjoto.2015.02.010 0196-0709/© 2015 Elsevier Inc. All rights reserved.

Please cite this article as: Haser GC, et al, Extramedullary plasmacytoma of the cricoid cartilage with solitary plasmacytoma of the rib, Am J Otolaryngol–Head and Neck Med and Surg (2015), http://dx.doi.org/10.1016/j.amjoto.2015.02.010

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AMER ICA N JOURNAL OF OT OLA RYNGOLOGY– H E A D A N D N E CK M EDI CI N E AN D S U RGE RY X X (2 0 1 5) XXX – XXX

with symptoms characteristic of multiple myeloma, such as hypercalcemia, anemia, or renal failure [5]. Extramedullary plasmacytoma of the cricoid cartilage is extremely rare [4], with fewer than 10 cases reported in the literature. Our case was particularly unusual because the patient was found to have an additional lesion in the left fifth rib, which was diagnosed as a solitary plasmacytoma of the bone. Despite the presence of multiple tumors, additional tests were non-diagnostic for systemic disease.

2.

Case history

A 72-year-old man presented to our voice clinic with a twoyear history of progressive hoarseness and stridor. He also complained of dyspnea on exertion. Flexible fiberoptic laryngoscopy revealed bilaterally mobile true vocal cords and evidence of subglottic stenosis. No masses or other lesions were noted. Initial computed tomography (CT) of the neck revealed an expansile lesion of the cricoid cartilage causing moderate glottic and subglottic narrowing (Fig. 1). The lesion did not extend to the mucosal surface or invade the thyroid gland or cartilage. Diffuse thickening and enhancement of the posterior commissure were noted. None of the lymph nodes identified in either side of the neck met the criteria for significant adenopathy. A core biopsy of the cricoid lesion was performed, and immunostains were performed. The biopsy revealed a few small foci of densely cellular infiltration by atypical plasma cells with perineural invasion and extension into the cricoid cartilage with accompanying necrosis. Immunohistochemical staining showed the presence of monoclonal plasma cells with kappa restriction, consistent CD138 expression, and scattered cytoplasmic IgD labeling, while IgG, IgA, and IgM were negative. All other immunostains were non-contributory. Following core biopsy, a full-body positron emission tomography-CT (PET/CT) study was obtained. PET/CT images demonstrated the previously identified glottis mass with maximum standardized uptake value (SUV) of 7.5 (Fig. 2). In addition, a fusiform expansile soft tissue lesion originating from the lateral left fifth rib, measuring 5 × 2 cm in axial cross-

Fig. 1 – Axial contrast-enhanced CT of the neck. CT, computerized tomography.

Fig. 2 – Axial 18F-FDG PET/CT of the neck. The area of hypermetabolism is associated with a calculated maximum standardized uptake value of 7.5. PET, positron emission tomography.

section with maximum SUV of 8.0, was identified (Fig. 3). No other osseous or soft tissue lesions were demonstrated, and there were no radiographically significant or hypermetabolic lymph nodes. Glottic defect secondary to prior core biopsy is evident on the PET/CT images of the neck. Subsequently, the patient was evaluated for multiple myeloma. Bone marrow biopsy revealed hypercellular marrow with tri-lineage hematopoiesis and mildly increased (5%–7%) plasma cells with kappa predominance. Blood tests did not reveal evidence of hypercalcemia, renal failure, or anemia. Serum immunoglobulin and β2 microglobulin levels were normal. Urine immunofixation showed a biclonal free kappa monoclonal protein. These results were not diagnostic for multiple myeloma. Given the imaging and pathologic findings, extramedullary plasmacytoma of the cricoid cartilage with solitary plasmacytoma of the rib is the most likely diagnosis. Extramedullary plasmacytomas and solitary plasmacytomas of the bone are treated with surgery and/or radiation therapy [5]. This patient was treated with two months of radiotherapy targeted to the two sites of disease involvement. He was followed up with PET/CT scans every six months and regular blood tests. A CT scan one year after presentation did not reveal any interval change in the volume of the residual soft tissue mass in the neck.

Fig. 3 – Axial 18F-FDG PET/CT of the chest. The area of hypermetabolism is associated with a calculated maximum standardized uptake value of 8.0. PET, positron emission tomography.

Please cite this article as: Haser GC, et al, Extramedullary plasmacytoma of the cricoid cartilage with solitary plasmacytoma of the rib, Am J Otolaryngol–Head and Neck Med and Surg (2015), http://dx.doi.org/10.1016/j.amjoto.2015.02.010

AMER IC AN JOURNAL OF OT OLA RYNGOLOGY– H E A D A N D NE CK M E D ICI N E AN D S U RGE RY X X (2 0 1 5) XXX – XXX

The patient has improved breathing and swallowing since completing radiotherapy. His hoarseness has also improved; however, he continues to experience some dyspnea upon exertion. He has not developed multiple myeloma at the time of publication.

3.

Discussion

Plasma cell neoplasms may be distinguished in several ways. Multiple myeloma, the most common plasma cell dyscrasia, results from abnormal proliferation of monoclonal immunoglobulins (IgG, IgA, and IgM) and/or light chains [4]. Rarely, excess proliferation may involve IgD, IgE, or heavy chains [4]. Multiple myeloma usually involves the bones, but it has been reported in the cricoid cartilage [3] and other soft tissues. Symptoms of multiple myeloma in the head and neck include bone pain and swelling [3]. Multiple myeloma can be distinguished from extramedullary plasmacytoma and solitary plasmacytoma of the bone by the presence of multiple, discrete areas of bone involvement, infiltration of plasma cells into the bone marrow, and the presence of monoclonal immunoglobulin in the blood [2]. Furthermore, multiple myeloma is associated with renal failure, anemia, and hypercalcemia [5]. Solitary plasmacytoma of the bone and extramedullary plasmacytoma are solitary plasma cell tumors occurring in the bone and soft tissues, respectively. Both may disseminate to multiple myeloma but are distinguished by the presence of a single radiologic lesion and normal bone marrow [2]. Extramedullary plasmacytoma is most commonly found in the nasopharynx, upper respiratory tract, gastrointestinal tract, and lymph nodes, while solitary plasmacytoma of the bone most often presents in the femur, spine, and pelvis [2]. To the best of our knowledge, fewer than 10 cases of extramedullary plasmacytoma arising in the cricoid cartilage have been reported [1]. In addition, only nine cases of multiple myeloma involvement of cricoid cartilage have been reported [6,7]. Other sites of extramedullary plasmacytoma involvement in the neck include the epiglottis, aryepiglottic folds, arytenoids, false vocal cords, ventricle, true vocal cords, and subglottis [1]. Differential considerations in this case include metastases, submucosal extension of squamous cell carcinoma, and other less common laryngeal malignancies, including soft-tissue sarcomas, minor salivary gland tumors, and primary nonHodgkin’s lymphoma of the larynx. Squamous cell carcinoma is the most common laryngeal malignancy [8]. It most commonly involves soft tissues, although it can invade other tissues, including cartilage in the neck. On CT imaging, neoplastic cartilage invasion is characterized by sclerosis, erosion and lysis of the cartilage [8]. Very advanced cases of cartilage invasion of squamous cell carcinoma may exhibit extralaryngeal spread [8]. However, in our case, no mucosal involvement was seen, as the tumor was limited to the cricoid cartilage in the neck. Chondrosarcoma and other cartilaginous tumors may arise from the cricoid cartilage, although rarely [9]. Chondrosarcomas show soft tissue stippled calcification, which is not seen in this case.

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Minor salivary gland neoplasms, most commonly adenoid cystic carcinoma and adenocarcinoma, may arise in the supraglottic and subglottic larynx [10]. In addition, primary manifestations of lymphoma can present within the larynx [11], although this is extremely rare. However, these malignancies were ruled out based on pathologic findings of the core biopsy, the presence of the rib lesion found on the PET/CT scan, and the findings of the subsequent bone marrow biopsy. Extramedullary plasmacytoma of the cricoid cartilage is an extremely rare tumor, and thus difficult to diagnose. The presence of the rib lesion in this patient without evidence of multiple myeloma makes this case particularly unusual. Confirming this patient’s diagnosis was essential both to rule out systemic disease and to address the patient’s airway concerns caused by the localization of the laryngeal lesion.

4.

Conclusion

We have presented a rare case of extramedullary plasmacytoma of the cricoid cartilage with solitary plasmacytoma of the left fifth rib. We hope this case may be instructive for the diagnosis and management of this unusual disease entity.

REFERENCES

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Please cite this article as: Haser GC, et al, Extramedullary plasmacytoma of the cricoid cartilage with solitary plasmacytoma of the rib, Am J Otolaryngol–Head and Neck Med and Surg (2015), http://dx.doi.org/10.1016/j.amjoto.2015.02.010

Extramedullary plasmacytoma of the cricoid cartilage with solitary plasmacytoma of the rib.

Extramedullary plasmacytoma is a rare plasma cell neoplasm of the soft tissues characterized by the presence of a single, discrete lesion without evid...
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