Unusual paranasal sinus tumors in two patients with common nasal complaints.

Abstract

Common nasal complaints are managed by both the otolaryngologist and the primary care physician. We describe the cases of two patients with nasal obstruction who were referred to us for evaluation--one with severe headache and the other with profuse epistaxis. Their histories prior to referral included long-term, common rhinologic complaints of low-grade headache and mild epistaxis. Neither patient had been referred to us until their symptoms had become severe. Our examination revealed that both patients had rare paranasal sinus pathology. One patient had a fibroxanthoma of the frontal sinus, and the other had extramedullary hematopoiesis of the maxillary sinus. Fibroxanthoma of the frontal sinus is rare, and extramedullary hematopoiesis of the maxillary sinus has not been previously reported. These two unique cases serve as a reminder that long-term common rhinologic complaints can occasionally be a sign of life-threatening pathology and require a full evaluation by an otolaryngologist.

Introduction

Inflammatory sinus disease is a far more common cause of chronic headache and epistaxis than are tumors of the paranasal sinuses. But when symptoms persist for an extended period, a complete examination is essential to investigate the possibility that one of these less common but more serious entities might be the cause. In this article, we describe two interesting cases of unusual paranasal sinus tumors that had been diagnosed at a very late stage. The delay in diagnosis had led to a worsening of symptoms and increased morbidity, including severe headaches and diplopia in one patient and recurrent epistaxis that required transfusions in the other. These two cases serve as a reminder that persistent rhinologic complaints can have life-threatening etiologies and require a complete evaluation by an otolaryngologist for a definitive diagnosis.

Case reports

Patient 1. A 44-year-old woman was referred to us for evaluation of a mass in her right frontal sinus. She had complained of right frontal headaches over the previous 15 years. The severity of these headaches had worsened during the 2 months prior to her referral, and she had also begun to experience double vision. She denied purulent rhinorrhea, allergies, or a history of nasal or orbital trauma.

In addition to the long-term headaches, her medical history was significant for breast cancer, and she had undergone a mastectomy 6 years earlier. The woman did not use tobacco or alcohol, and the only medication she took was ibuprofen for her headaches.

Physical examination revealed an obvious downward displacement of the right eye by a mass in the medial orbit. The mucosa of the nasal cavity was normal, and there was no evidence of any abnormal secretions.

Computed tomography (CT) revealed that a mass, outlined by a thin wall of bone, had arisen from the right frontal sinus (figure 1). The lesion's appearance was characteristic of a mucocele. Bony erosion was noted at the superomedial orbital wall and at the posterior table of the frontal sinus; because of this erosion, a neurosurgical consult was obtained. After a full evaluation, we developed a treatment plan that included a bicoronal flap surgical approach to the frontal sinus, an osteoplastic flap elevation, removal of the mucocele, and obliteration of the sinus.

During surgery, a rubbery yellow mass was elevated from the frontal sinus. The erosion into the orbit was obvious. After the mass was removed, the orbital contents were returned to their normal position. The tumor had been adherent to a large section of dura, which was resected and repaired by the neurosurgical service. The sinus was obliterated in the usual manner. During 12 months of followup, the patient was both symptom- and disease-free, and she experienced a complete resolution of her diplopia.

Histologic analysis revealed that the tumor contained a cellular proliferation of fibrohistiocytic elements with multinucleated giant cells and prominent xanthomatous features (figure 2). On staining, the lesion was positive for factor 13A and negative for CD34. The dendritic macrophages in the lesion were positive for S-100, but the proliferating tumor cells were negative. The specimen was also negative for cytokeratin. Based on these findings, we made a diagnosis of fibroxanthoma, which was confirmed by consultants at the Armed Forces Institute of Pathology.

Patient 2. A 71-year-old woman was referred to us for evaluation of a mass in her left nasal cavity. She had been experiencing recurrent epistaxis that had increased in both severity and frequency over the previous year and which had required two blood transfusions. She had undergone endoscopic sinus surgery for nasal polyposis 5 years earlier, but she was no longer being seen regularly by an otolaryngologist. Her history was also significant for myelofibrosis, which had been diagnosed 20 years earlier and which was considered to be well controlled by low-dose prednisone and folic acid.

Sinus endoscopy revealed that a soft polypoid mass had filled the left nasal cavity; it appeared to have arisen from the middle meatus. CT showed that the mass had filled the left maxillary sinus and extended into the nasal cavity (figure 3). There was no bony erosion or other mucosal disease. The patient was scheduled for an endoscopic biopsy and a possible sphenopalatine artery ligation.

During surgery, a soft, dark-purple mass was removed from the nasal cavity and sinus. Frozen-section analysis revealed a cellular proliferation, but the diagnosis was deferred until a histologic examination could be performed. Minimal bleeding was encountered during the procedure, and no nasal packing was necessary.

Histologically, the lesion was made up of portions of respiratory mucosa that exhibited chronic inflammation and fibrosis. A diverse population of hematopoietic cells was noted, including mature megakaryocytes admixed with normal erythroid and myeloid precursors (figure 4). Gomori methenamine silver staining was negative for fungal organisms.

A routine chest x-ray obtained prior to surgery had detected an ill-defined infiltrate that involved both lower lung lobes. This finding prompted a CT scan, which revealed a grossly enlarged spleen and bilateral paraspinal masses, but no parenchymal lung disease (figure 5). A CT-guided biopsy of the paraspinal lesions revealed extramedullary hematopoiesis that was indistinguishable from that in the nasal mass.

Discussion

Patient 1. Fibrous histiocytoma is a generic term used to describe both benign and malignant lesions that are made up of cells that have features of both fibroblasts and histiocytes. These lesions are usually found in the somatic soft tissues. Although they are rare in the head and neck, when they do occur there, the sinonasal cavity is one of the more common sites. Malignant fibrous histiocytoma, despite its rarity, has been well described in the maxillary and ethmoid sinuses. [1] One case has been documented in the frontal sinus. [2] Benign fibrous lesions are also uncommon in the sinuses, [3-5] Fibroxanthoma, a low-grade variant of benign fibrous histiocytoma, is characterized by a predominance of xanthomatous histiocytes. [6] To ourknowledge, only one case of fibroxanthoma of the frontal sinus has been documented in the literature.[7]

Regardless of the site, the treatment of fibroxanthomas consists of complete surgical excision. Radiation has little effect on fibroxanthomas and is not recommended following excision because these low-grade tumors are unlikely to recur. Prophylactic treatment of the lymph nodes is not necessary because the incidence of metastasis is low. [5]

Patient 2. Myelofibrosis is a myeloproliferative disorder in which the proliferation of fibroblasts in bone causes marrow depletion and anemia. The most common sites of extramedullary hematopoiesis associated with this disease are the spleen and liver. [8] Although they too are rare, extramedullary hematopoietic paraspinal masses are well recognized in the radiologic literature. Mounds of soft fleshy tumor surround the vertebrae, and extensions into the epidural space have been described. [9]

Extramedullary hematopoiesis of the head and neck is rare. The literature contains only five previous reports; one involved the thyroid, one involved the esophagus, and three involved the middle ear. [10-14] Surgical excision was sufficient treatment for all five patients. To our knowledge, ours is the first report of extramedullary hematopoiesis occurring in the paranasal sinuses.

References

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