1
Introduction
Extramedullary plasmacytoma (EMP), multiple myeloma (MM), and solitary plasmacytoma of the bone (SPB) are each classified as distinct manifestations of a disease continuum with varying symptoms, treatment regimens, and prognostic implications. While the etiology for each of these neoplastic entities is due to a derangement of plasma cells, these B-cell neoplasms are indistinguishable histologically . EMP, a rare neoplasm, accounts for approximately 2–3% of all plasma cell tumors, SPB accounts for up to 4–6% of cases, and multiple myeloma for 91–94% . These tumors have a male predominance with a 4:1 male to female ratio, and typically present in the fifth or sixth decade of life . EMP of the head and neck are rare, accounting for < 1% of all head and neck neoplasms. Clinically, EMP can resemble more aggressive tumors, including squamous cell carcinoma, and can manifest in the oral cavity, nasal cavity, paranasal sinuses, cervical lymph nodes, salivary glands, thyroid gland, orbit, larynx, and skin of the head and neck region . EMP is highly responsive to radiotherapy, but can come to the attention of the skull base surgeon for intractable and/or recurrent sinonasal disease .
2
Materials and methods
This is a retrospective case study of a patient who presented with bilateral extramedullary plasmacytoma. The patient’s medical and surgical history, operative reports, and post-operative visits were evaluated. Institutional review board approval was not required.
2.1
Case patient
Our patient is a 44-year-old female with no significant past medical or surgical history. Two years prior to the patient’s definitive procedure, she presented to our clinic for the indication of recurrent epistaxis of the left nare. She had been referred to our institution after an episode of severe epistaxis requiring intranasal packing by an outside Otolaryngologist. The patient was noted at the time of nasal endoscopy to have a polypoid mass obstructing her left nare. A biopsy was performed in the office and interpreted as granulation tissue, with a significant number of foamy histiocytes noted in the specimen. Culture of the material grew pan sensitive Staphylococcus aureus . A contrast-enhanced computed tomography scan of the paranasal sinuses ( Fig. 1 ) showed multiple enhancing well-circumscribed lobulated masses in the anterior and central aspects of the left nasal cavity that appeared to be pedicled from the medially-displaced left inferior turbinate the largest of which was 18 mm in largest dimension. A smaller but similarly appearing mass was noted in the right anterior nasal cavity. There were no cervical lymph nodes meeting radiographic criteria for adenopathy. Approximately two weeks later the patient was brought to the operating room for biopsy and lesion debulking to provide a nasal airway. Surgical pathology was consistent with bilateral nasal plasmacytoma with immunohistochemical stains positive for Kappa light chain and IgD heavy chain. Lambda, IgG, IgM, IgA, CD3, and CD20 were all negative. The patient had an uneventful post-operative course and was immediately referred to a radiation oncologist. She further underwent bone marrow aspiration and biopsy that showed left shifted myeloid, randomly scattered plasma cells but no significant increase from normal, and no evidence of plasma cell dyscrasia. Post-operative laboratory results were significant for a hemoglobin of 11 g/dL due to iron deficiency anemia. Serum protein electrophoresis and serum immunofixation electrophoresis (SPEP/SIFE) showed a polyclonal pattern with slight elevation of IgA (441). Urine protein electrophoresis and urine immunofixation electrophoresis (UPEP/UIFE) were indeterminate due to insufficient sample, and was similarly indeterminate on repeat testing. Otherwise, the patient’s complete metabolic panel, IgD, IgG, IgM levels, free light chain (FLC) analysis, lactate dehydrogenase, and Beta 2 microglobulin were all within normal limits. One month later the patient began radiation therapy with planned dosing of 40–50 Gy and was subsequently monitored closely with endoscopic and radiologic evaluations, including combined positron emission tomography-computed tomography (PET-CT) scanning taken at a three-month interval following surgery, with plan for follow-up every three months. A PET-CT scan obtained eight months after completion of external beam radiation showed an interval increase in left nasal metabolism with an SUV increase from 8.2 to 10.4. Repeat endonasal biopsy was consistent with granulation. Six months later, repeat computed tomography imaging showed a bilobed enhancing nodular tissue medial to the nasal process of the maxilla without local erosion and measuring 2.4 × 7 × 14 (craniocaudad) mL. The decision was made to proceed to the operating room to obtain a biopsy for definitive pathologic diagnosis and possible resection.
2.2
Surgical method
The patient was brought to the operating room where she underwent bilateral nasal endoscopy and biopsy of the nasal lesion. Multiple biospies were taken at the onset of the procedure and sent for frozen section, these were consisted with recurrent plasmacytoma ( Figs. 2 and 3 ). Using image guided navigation, the mass was then sharply excised with wide margins via a medial maxillectomy with a Denker’s maxillotomy. The lacrimal sac was identified and sharply transected. Frozen section from the distal lacrimal sac margin was free of disease.
2
Materials and methods
This is a retrospective case study of a patient who presented with bilateral extramedullary plasmacytoma. The patient’s medical and surgical history, operative reports, and post-operative visits were evaluated. Institutional review board approval was not required.
2.1
Case patient
Our patient is a 44-year-old female with no significant past medical or surgical history. Two years prior to the patient’s definitive procedure, she presented to our clinic for the indication of recurrent epistaxis of the left nare. She had been referred to our institution after an episode of severe epistaxis requiring intranasal packing by an outside Otolaryngologist. The patient was noted at the time of nasal endoscopy to have a polypoid mass obstructing her left nare. A biopsy was performed in the office and interpreted as granulation tissue, with a significant number of foamy histiocytes noted in the specimen. Culture of the material grew pan sensitive Staphylococcus aureus . A contrast-enhanced computed tomography scan of the paranasal sinuses ( Fig. 1 ) showed multiple enhancing well-circumscribed lobulated masses in the anterior and central aspects of the left nasal cavity that appeared to be pedicled from the medially-displaced left inferior turbinate the largest of which was 18 mm in largest dimension. A smaller but similarly appearing mass was noted in the right anterior nasal cavity. There were no cervical lymph nodes meeting radiographic criteria for adenopathy. Approximately two weeks later the patient was brought to the operating room for biopsy and lesion debulking to provide a nasal airway. Surgical pathology was consistent with bilateral nasal plasmacytoma with immunohistochemical stains positive for Kappa light chain and IgD heavy chain. Lambda, IgG, IgM, IgA, CD3, and CD20 were all negative. The patient had an uneventful post-operative course and was immediately referred to a radiation oncologist. She further underwent bone marrow aspiration and biopsy that showed left shifted myeloid, randomly scattered plasma cells but no significant increase from normal, and no evidence of plasma cell dyscrasia. Post-operative laboratory results were significant for a hemoglobin of 11 g/dL due to iron deficiency anemia. Serum protein electrophoresis and serum immunofixation electrophoresis (SPEP/SIFE) showed a polyclonal pattern with slight elevation of IgA (441). Urine protein electrophoresis and urine immunofixation electrophoresis (UPEP/UIFE) were indeterminate due to insufficient sample, and was similarly indeterminate on repeat testing. Otherwise, the patient’s complete metabolic panel, IgD, IgG, IgM levels, free light chain (FLC) analysis, lactate dehydrogenase, and Beta 2 microglobulin were all within normal limits. One month later the patient began radiation therapy with planned dosing of 40–50 Gy and was subsequently monitored closely with endoscopic and radiologic evaluations, including combined positron emission tomography-computed tomography (PET-CT) scanning taken at a three-month interval following surgery, with plan for follow-up every three months. A PET-CT scan obtained eight months after completion of external beam radiation showed an interval increase in left nasal metabolism with an SUV increase from 8.2 to 10.4. Repeat endonasal biopsy was consistent with granulation. Six months later, repeat computed tomography imaging showed a bilobed enhancing nodular tissue medial to the nasal process of the maxilla without local erosion and measuring 2.4 × 7 × 14 (craniocaudad) mL. The decision was made to proceed to the operating room to obtain a biopsy for definitive pathologic diagnosis and possible resection.
