A 63-year-old female, a known diabetic on adequate therapy, presented to the ENT outpatient clinic with the primary complaints of left-sided nasal obstruction for the past three months. It was associated with left-sided nasal discharge for two weeks. It was mucoid, non-purulent, and non-blood stained. There was no antecedent history of any breathing difficulty or any visual complaints. There was no history of pain, cheek numbness, blood-stained nasal discharge, or loosening of teeth.
On clinical examination, there was no apparent distortion of the external osseocartilaginous nasal framework. Facial symmetry was maintained with no obliteration of the nasolabial groove. On anterior rhinoscopy, a red polypoidal mass was visualized occupying the entire left nasal cavity, pushing the nasal septum to the right, and reaching the floor of the cavity. It was insensitive to touch. On probing, the mass did not bleed and appeared to be attached to the lateral wall of the nasal cavity. There was no extension to the nasopharynx. All cranial nerves appeared intact. There was no obvious palpable cervical lymphadenopathy. Visual, dental, and systemic examinations were unremarkable.
After the initial blood workup, the patient underwent a contrast-enhanced computed tomography scan (CECT) of the nose, paranasal sinuses, orbit, and neck. It depicted a well-defined 3x3x2cm enhancing soft tissue density in the left nasal cavity and the anterior ethmoids, with some extension to the posterior ethmoids. The growth was abutting the medial wall of the left maxillary sinus and the orbit with no apparent bone erosion (Figure 1a). It obstructed the drainage of the left frontal recess (Figure 1b). A subsequent contrast-enhanced magnetic resonance imaging (MRI) showed a T2 intermediate intense irregularly enhancing lesion in the left ethmoid sinuses, with no intracranial extension (Figure 2).
Keeping in mind the age and the clinical presentation of the patient and the endoscopic appearance, the following diagnoses were broadly considered.
- Epithelial malignancy of the nasal cavity: Squamous cell carcinoma remains the first differential because it is more common. Squamous cell carcinoma comprises almost 50% of all sinonasal tumors in general (3).
- Adenocarcinoma was a possibility keeping in mind the age of the patient.
- Inverted papilloma of the nasal cavity: Also known as Schneiderian papilloma, it is a benign, locally aggressive tumor with malignant potential. Histopathology provides the mainstay of diagnosis, showing the invasion of the epithelium into the stroma.
- Fibrosarcoma, leiomyosarcomas, and schwannomas were considered due to the preliminary spindle cell histopathology.
A diagnostic nasal endoscopy under local anesthesia revealed the mass attached to the middle turbinate, with dilated vessels on the surface (Figure 3). An endoscopically guided biopsy was undertaken, which was reported as spindle cell morphology with nuclear atypia, possibly a sarcomatous lesion.
The case was discussed in the multidisciplinary tumor board meeting and due to the limited nature of the disease, a primary surgery, followed by adjuvant chemoradiotherapy was planned. The patient was offered endoscopic excision under general anesthesia. The red fleshy mass was visualized occupying the middle meatus on the left. Partial middle turbinectomy was done. Middle meatal antrostomy and anterior ethmoidectomy were done using a microdebrider, sparing as much of the nasal mucosa as possible. The tumor mass was seen to be arising from the ethmoid sinuses. Bleeding was encountered which was controlled with nasal packing and bipolar diathermy. The mass was removed and sent for histopathology. Routine postoperative care was administered.
The patient was discharged a day after the procedure with antibiotics and instructions on nasal douching. Post-operative histopathology showed spindle-shaped tumor cells arranged in fascicles with nuclear atypia and hyperchromatism. Blood vessels were found interspersed within the lesion (Figure 4a, 4b, and 4c). Immunohistochemistry was positive for nuclear TLE-1 and bcl-2 (Figure 5a and 5b) and was negative for EMA and CD34 (Figure 6a and 6b). Thus, melanoma and epithelial tumors were ruled out. A tissue diagnosis of monophasic synovial sarcoma was thus finalized. She was referred for adjuvant chemoradiotherapy. She received intensity-modulated radiotherapy of 66Gy in 33 fractions to the nasal cavity and the surrounding tissues along with palliative two agent chemotherapy with Doxorubicin and Ifosfamide throughout her treatment. She remains symptom-free on follow-up of two years.