Case Report:
A 70-year-old male presented to an otorhinolaryngology clinic by referral for evaluation of a right sinonasal passageway tumor. He had a medical history notable for retinoblastoma treated more than 60 years ago with periorbital radiotherapy and ultimately right eye enucleation. He was noted as being blind in the contralateral eye as well. Details regarding radiotherapy fields were requested from childhood treatment but were unfortunately not obtainable. Patient was likely treated with parallel opposed fields that would have included the sinonasal region. Additionally, he had a reported history of sarcoma on the back of the right leg and bladder cancer, for which detailed medical history records were not available upon request. The patient reported for the past year he had experienced progressively worsening right-sided nasal congestion and drainage. He also noted that his right eye prosthesis had begun to fit improperly. On physical exam, the patient had a soft tissue mass centered in the right nasal passage visualized by nasal telescope to involve the ethmoid and medial maxillary regions. Mild right-sided septal deflection was also noted. Cranial nerves III through XII were intact on exam.
Magnetic resonance imaging (MRI) demonstrated a large, T1 hypointense, heterogeneous T2 and enhancing soft tissue mass measuring approximately 6.1 x 3.1 x 4.1 cm centered within the right nasal cavity. The mass appeared to extend from the right piriform aperture to the ventral aspect of the right nasopharynx (Figure 1A,1B). A craniocaudal measurement demonstrated the mass extending 4.1 cm from the floor of the right nasal cavity to the floor of right anterior cranial fossa (Figure 1-C,D). Bony remodeling and thinning of adjacent osseous structures were present, with significant leftward bowing of the nasal septum and rightward bowing of the medial right maxillary sinus wall. Additionally, post-obstructive mucosal changes in the lateral recess of the maxillary sinus leading into the sphenoid sinuses were noted. The right sided nasal turbinates were completely obstructed with partial obstruction of right ethmoid air cells. Chronic obstruction of the right sphenoid sinus at the sphenoethmoidal recess and right frontal sinus were present. Imaging showed thinning of the medial floor of the right orbit with mild displacement of an atrophic right medial and right inferior rectus muscle without intracranial soft tissue mass extension.
The pathology report from biopsies taken in the office indicated a spindle cell neoplasm consistent with myofibroblastoma of low to intermediate grade. It was initially suspected the patient most likely had a radiation-induced sarcoma, which would require surgical treatment in the form of a partial or total right maxillectomy and ethmoidectomy. Additional resection of the alveolar and palatal bone was discussed, as well as the inferior and medial orbital walls which could limit future use of his prosthesis. If the patient’s lesion was determined to be non-malignant more conservative resection measures would be considered.
The patient underwent a maxillectomy and ethmoidectomy. During surgery, no evidence of tumor extension through the base of the maxilla, floor of the nose, or orbit was noted. Tumor was seen involving the inferior and medial orbital wall and was adherent but not invasive to the adjacent bone. It was also found that the tumor had adhered to the nasal septum, without apparent extension into the perichondrium or periosteum. Total ethmoidectomy with preservation of the base of the skull was achieved and thought to provide an adequate oncologic margin. A portion of the posterior turbinate and adjacent choana mucosa, along with a retained portion of the inferior medial maxillary wall, were also excised to provide an adequate posterior oncologic margin. Ultimately, a partial maxillectomy was achieved. The patient tolerated the procedure well and was discharged home the next day without perioperative complications.
The specimen received for histopathologic analysis consisted of a well-defined, ovoid, tan, mass having a soft and glistening surface, measuring (5.7 x 4.2 x 2.6 cm), with partial mucosal covering and attached bony fragments over the resection margins. Sectioning revealed the mass to have a tan-white, soft, smooth, homogenous cut surface with minute foci of hemorrhage. Histologic examination showed a well-circumscribed purely mesenchymal neoplasm that lacked a true capsule, demonstrating short to elongated spindle cells arranged in haphazard intersecting fascicles (Figure 2A). Bands of hyalinized, eosinophilic collagen were observed in the background. On higher magnifications, the tumor was mainly composed of bland spindle-shaped myofibroblasts with scattered larger epithelioid cells with mild to moderate nuclear pleomorphism. Focal areas of tumor degeneration and occasional mitotic figures were identified. These features can be contributed to reactive atypical changes in the tumor background. Diagnostic features of malignancy were not observed. The tumor was excised completely with negative surgical margins. Immunohistochemical studies showed strong diffuse reactivity to SMA and desmin in the tumor cells with focal reactivity of moderate intensity for CD34 (Figure 2B,2C,2D). Taken together, the findings are consistent with low to intermediate grade myofibroblastoma. The patient was subsequently discharged with close follow-up, without further intervention required. Written informed consent was obtained from the patient for their anonymized information to be published in this article.