Volume 3, Issue 3 , Pages 143-146, September 2008
Extramedullary plasmacytoma of maxillary sinus in 10-year-old girl: A rare presentation
Article Outline
Summary
Extramedullary plasmacytomas are localized plasma cell neoplasms which arise within the soft tissues, by definition they cannot arise within bone. They represent less than 1% of all head and neck neoplasm. It commonly affect female in the sixth to seventh decade of life with no reported case presenting in first decade of life. The treatment of the condition is controversial with opinion split equally between surgery and radiotherapy. We present a case of extramedullary plasmacytoma of maxillary sinus in 10-year-old girl treated by excision of mass and post-operative radiotherapy.
Keywords: Plasmacytoma, Maxillary sinus
1. Case report
A 10-year-old female presented to the out-patient department of otolaryngology and head neck surgery with a complaint of swelling of right side of face for 6 months. She complained of slight pain in the region of the cheek for last 4 months. There was no history of nasal obstruction and epistaxis during the last 6 months. On examination, there was generalized swelling in the right maxillary region with slight proptosis and apparent ptosis on the same side (Fig. 1). Anterior rhinoscopy revealed gross narrowing of right nasal cavity with bulging of medial wall of right maxillary sinus. Diagnostic nasal endoscopy revealed no mass lesion in the nasal cavity and the nasal mucosa appeared normal. On palpation, the anterior maxillary wall on right side was firm and tender with apparent erosion of bone with hyposthesia of right cheek. The palate and alveolus was normal with no malocclusion or displacement of teeth.
Fig. 1.
Clinical photograph of the patient showing right maxillary swelling, slight proptosis and apparent ptosis.
On investigating the patient, the routine blood work-up was normal except slightly elevated ESR (45
mm). X-ray paranasal sinus occipitomental view revealed opacification of right maxillary sinus with rarefaction of maxillary sinus bone in few places. CT scan nose and paranasal sinus revealed a heterogeneous soft tissue mass filling the right maxillary sinus with erosion of anterior wall and roof and thinning of right orbit floor. The medial wall of the maxillary sinus, palate and alveolar process were fully intact (Fig. 2).
Fig. 2.
CT scan showing heterogeneous soft tissue mass filling the right maxillary sinus. The medial wall of the maxillary sinus was intact.
Based on the history, clinical examination and investigations, a provisional diagnosis of aggressive mass lesion of right maxillary sinus was made and the patient was prepared for excision of the mass with subsequent histopathological examination. Based on the CT finding and clinical examination, no attempts at antroscopic biopsy of the mass was done as the mass appeared to be malignant with erosions in the region of anterior wall of maxilla and upper part of canine fossa, to avoid seeding of the tract with malignant cells.
The patient was operated under general anesthesia with orotracheal intubation. Partial maxillectomy was performed via a lateral rhinotomy approach with Weber Ferguson extension with preservation of palate and alveolar process. The mass was well circumscribed (4cm×3.5cm) with extreme thinning of surrounding bone esp. the orbital plate and anterior wall of maxilla with extension into the infratemporal fossa (Fig. 3). A definite plane was present between the mass and the cheek skin allowing its preservation. A primary dacrocystorhinostomy was done on right side to prevent post-operative epiphora.
Fig. 3.
Photograph of the mass after removal.
The histopathological examination of the specimen revealed a diagnosis of extramedullary plasmacytoma of maxilla (Fig. 4, Fig. 5). Due to the unusual age of presentation, a further conformation by immunohistochemical staining of the specimen was done which revealed plasma cells expressing CD 138 with lambda light chain restriction thereby confirming the diagnosis. To rule out multiple myeloma, plasma electrophoresis and urinalysis for Bens-Johns protein was done which came out negative. Bone marrow examination was within normal limit.
Fig. 4.
Microphotograph showing feature of plasmacytoma (H & E stain; 10
×10).
Fig. 5.
Microphotograph showing features of plasmacytoma (H & E stain; 10
×40).
2. Discussion
Extramedullary plasmacytomas are localized plasma cell neoplasms which arise within the soft tissues, by definition they cannot arise within bone. They represent less than 1% of all head and neck neoplasms. 80% of extramedullary plasmacytomas occur in the head and neck and 10–20% of cases may present with multiple lesions with 75% of occurring in sinonasal/nasopharyngeal area, 12% in the oropharynx, and 8% in the larynx [1], [2]. Betsakis classified plasma cell neoplasms in head neck region as three disorders: extramedullary plasmacytoma, solitary plasmacytoma of bone and manifestations of multiple myeloma. They essentially represent a distinct manifestations of a disease continuum [1]. Clinical findings are critical to diagnosis, and distinguishing one disorder from other has significant implication for treatment and survival [2].
Extramedullary plasmacytoma affects men 3–4 times more often than women and typically occur in the sixth to seventh decade with over 95% of cases occurring in patients above 40 years of age [1], [3]. So a presentation in a girl of 10 years is indeed a rare presentation. After extensive research of literature we could not find any instance of extramedulary plasmacytoma presenting in first decade of life as was seen in our case. Most of the symptoms related to extramedullary plasmacytoma can be related to their specific location in the head and neck. However, most common symptom is presence of mass/swelling with additional symptoms of airway obstruction, epistaxis, localized pain and proptosis seen in association as was noted in our case [3], [4].
Physical examination usually reveals slightly raised submucosal swelling in cases with definite mass lesion. The mucosa is typically intact. Cervical lymph node metastasis is reported to occur in 12–26% of cases at initial presentation [3]. No definite mass can be seen either on anterior rhinoscopy or diagnostic endoscopy in our case and local examination revealed no regional lymphadenopathy.
Diagnosis usually requires judicious use of imaging modality but the first step towards diagnosis is usually a biopsy of the lesion in the presence of a definite mass lesion but deep biopsies must be taken since the tumor is submucosal and the mucosa may be thickened from an inflammatory reaction [2], [3]. Biopsy was not possible in our case as there was no mass in the nasal cavity and antroscopic biopsy was avoided to prevent seeding of probable malignant cells along the antroscopy tract. Radiographic imaging is important to evaluate the location and size of the lesion with CT scan helping in bone delineation and MRI helping in determining extension of soft tissue mass [3], [4].
Microscopically, extramedullary plasmacytomas appear as a monocellular proliferation of plasma cells set in a sparse matrix. Nuclear and cellular atypia may be minimal or prominent. Plasmacytic, plasmablastic, and anaplastic subtypes have been described, but Batsakis states that histological appearance is not a reliable indicator of biologic activity [1], [2].
Using immunohistochemical techniques, a monoclonal staining pattern demonstrating either one heavy chain class, one light chain type, or both can be demonstrated as was seen in our case. Plasmacytoma can be confused with other benign and malignant conditions like benign reactive plasmacytosis, undifferentiated carcinoma, non-Hodgkin's lymphoma, malignant melanoma or esthesioneuroblastoma. Therefore immunohistochemical staining helps in typing the neoplastic and monoclonal nature of cells [1], [2], [3].
FNAC is of limited use in diagnosis as it provides inadequate tissue for special staining and for complete histological examination. Therefore incisional or excisional biopsy, depending upon the size and location of the mass is necessary [2], [3].
In 20–30% of cases the extramedullary plasmacytoma may progress to multiple myeloma. Histopathologic examination cannot distinguish multiple myeloma from an extramedullary plasmacytoma, further evaluation is necessary to exclude the presence of systemic disease and confirm the diagnosis of extramedullary plasmacytoma. Recommend investigations are complete blood count with white blood cell count and platelet count, bone marrow biopsy, serum biochemistry including calcium, blood urea nitrogen, creatinine, uric acid, serum protein, serum and urine electophoresis, and a skeletal survey to rule out multiple myeloma [1], [2], [5]. Normal bone marrow examination, absence of lytic bone lesions on skeletal series, and low paraprotein levels are necessary to establish the diagnosis of extramedullary plasmacytoma as was seen in our case.
According to Batsakis, the natural history of extramedullary plasmacytoma may be characterized in the following five ways [1]:
The treatment of extramedullary plasmacytoma of the head and neck is controversial. Some authors advocate radiation therapy alone and others advocate surgery alone [2], [4], [5], [6]. Factors associated with poor prognosis on radiotherapy include the younger age, presence of bone destruction, large primary tumor, recurrence, and tumors located in the sphenoid, maxillary sinus, orbit, and larynx [1], [5]. Histological appearance and lymph node involvement are not reported to be of any prognostic significance [1], [3]. In our case, keeping in mind the age of the patient and detrimental effect of radiotherapy in pediatric age, size of the lesion and absence of preoperative biopsy findings, an excision biopsy of the lesion with curative intent was undertaken.
References
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- . Unusual presentations of extramedullary plasmacytoma in the head and neck. J. Laryngol. Otol. 1988;102:102–104
- . Maxillary sinus plasmacytoma. Isr. Med. Assoc. J. 2004;6:119–120
- Plasmacytoma—treatment results and conversion to myeloma. Cancer. 1992;69:1513–1517
- . Extramedullary plasamacytomas of the head and neck. Otolaryngol. Head Neck Surg. 1993;109:877–888
PII: S1871-4048(08)00010-5
doi:10.1016/j.pedex.2008.02.003
© 2008 Elsevier Ireland Ltd. All rights reserved.
Volume 3, Issue 3 , Pages 143-146, September 2008
