Primary sclerosing fibroinflammatory pseudotumor of the maxillary sinus.
Inflammatory pseudotumor is a well known but poorly understood pathologic entity. It is associated with an unusual growth of fibrotic tissue admixed with varying amounts of inflammation that displaces and compresses normal anatomic structures, resulting in dysfunction. Pseudotumors have been.found in multiple locations--the orbit in particular--but to the best of our knowledge, none has previously been reported as an isolated sinus lesion. We describe a case of primary sclerosing fibroinflammatory pseudotumor of the maxillary sinus that manifested as recurrent unilateral maxillary sinusitis in a 47-year-old woman. The patient was managed with surgery and oral steroids with full resolution of her symptoms. We also review the presentation, diagnosis, and treatment of fibroinflammatory pseudotumors within the context of the current literature.
Fibrosclerosing disorders have been associated with many parts of the body, including the head and neck, thorax, and abdomen. Pseudotumors of the head and neck have historically and classically manifested as an inflammatory disease of the orbit, which represents the most well-characterized presentation in the literature. Histopathologically, these tumors produce a broad spectrum of findings, ranging from predominantly lymphoid tissue to highly fibrotic tissue. (1,2) In rare cases, orbital pseudotumors have been associated with other fibroinflammatory diseases, such as retroperitoneal fibrosis, sclerosing cholangitis, Riedel thyroiditis, mediastinal fibrosis, and fibrosis of the lung, parotid gland, and lacrimal glands. (1)
In this article, we present what we believe is the first reported case of an isolated fibrosclerotic pseudotumor of a sinus, which was seen in a patient with chronic rhinosinusitis.
A 47-year-old white woman with recurrent left maxillary sinusitis refractory to medical management (e.g, amoxicillin/clavulanate, prednisone, and fluticasone nasal spray) underwent a middle meatal antrostomy; histopathology was reported as nonspecific fibrosis. At about the same time, she underwent fine-needle aspiration of a left neck lymph node at another institution; the cytology identified nonspecific inflammatory changes and parotid tissue. Two months later, she presented to us with left cheek and periorbital pain that radiated to the ear, along with left nasal congestion, rhinorrhea, and sneezing.
Fiberoptic examination of the nose revealed wellhealed fibrotic maxillary and ethmoid sinuses. Computed tomography (CT) demonstrated left maxillary opacification and expansion (figure 1). The sinus was filled with hyperdense material with central hypodensity, and the walls were thickened. The left lamina papyracea was thinned medially, with bowing of material into the inferior orbit. However, orbital fat planes were maintained, and there was no frank invasion of the orbit. These findings suggested isolated maxillary sinus involvement.
The patient underwent an endoscopic left modified medial maxillectomy and left frontal sinusotomy. Intraoperatively, a granular and friable mass was seen filling the maxillary sinus. The mass was elevated away from the bone and removed circumferentially from the walls of the sinus. Frozen-section analysis during surgery confirmed the presence of a fibrotic process, and the final histopathologic examination confirmed the diagnosis of sclerosing fibroinflammatory pseudotumor (figure 2A). The patient's postoperative course was uncomplicated, and she was discharged home the same day.
Immunohistochemistry was positive for smoothmuscle actin (figure 2B), and negative for S-100, keratin, CD34, epithelial membrane antigen, progesterone receptor, and desmin. She was prescribed methylprednisolone.
At the 6-month follow-up, the patient was doing well. Magnetic resonance imaging at that time demonstrated evidence of a soft-tissue mass in the area of the left maxillary sinus without orbital involvement or bony invasion. This mass was believed to represent either residual pseudotumor or thickened mucosa. Endoscopic examination at approximately the same time revealed that her left maxillary sinus had contracted completely; there was evidence of osteoneogenesis but no evidence of recurrence. At 21 months of follow-up, the patient remained free of recurrence.
Inflammatory pseudotumor is a generic term used to describe a mass that displaces the surrounding anatomic structures and leads to organ dysfunction secondary to compressive growth. Fibroinflammatory disorders include heterogeneous clinical entities whose etiologies and pathogenesis remain unknown. (1-3) They can be subdivided into two types: sclerotic and nonsclerotic. The sclerotic type is locally more aggressive and less responsive to therapy. To the best of our knowledge, no case of isolated fibrosclerosing pseudotumor of a sinus has been previously reported in the literature.
Much of what is known about pseudotumors has been gleaned from the ophthalmology literature-particularly, from discussions of the more common orbital pseudotumor. This knowledge provides a foundation for understanding the presentation, diagnosis, and management of a sclerosing pseudotumor of the maxillary sinus.
The etiology of the sclerosing variant of fibroinflammatory disorders remains unclear. Some authors, including Jakobiec and Jones, (4) have noted that the nonsclerosing disease process begins with lymphocytic involvement that later evolves into dense collagen formation and fibrosis. According to this theory, nonsclerosing inflammatory conditions are subclassified into three subtypes: lymphoid, fibrotic, and mixed.
[FIGURE 1 OMITTED]
The sclerosing variant of pseudotumor is pathologically distinct, given its aggressive, locally destructive course that results in bone loss. Unlike the nonsclerosing type, the sclerosing form cannot be grouped into one of the three categories, despite the seemingly similar origins of the two types. Several authors have noted that sclerosing disease processes, including sclerosing orbital pseudotumor, are often associated with multifocal sclerotic disease processes. (1,3,5) Retroperitoneal fibrosis, sclerosing cholangitis, Riedel thyroiditis, and sclerosing mediastinitis are all associated with orbital pseudotumors, and they provide a basis for a unifying autoimmune or genetic cause. (1,3,5)
Our patient presented with fine-needle aspiration cytology results obtained at another institution at about the same time that she was diagnosed at our institution; these cytologic findings were consistent with inflammatory changes of the parotid gland, which were suggestive of a more diffuse inflammatory process.
Pseudotumors of the orbit often present unilaterally in adults; there is no predilection for either sex or any race. (1,2,5,6) The clinical presentation usually includes progressive proptosis, pain, limitation of extraocular movement, diplopia, and vision loss over a period of months. (1,2,5,6) Orbital pseudotumor may also be associated with increases in the erythrocyte sedimentation rate, white blood cell count, gamma globulin levels, and eosinophil level, as well as rheumatoid factor positivity and an elevated antinuclear antibody level.
[FIGURE 2 OMITTED]
In our case, the patient's white blood cell count was within normal limits. Her autoimmunity workup was pursued by her primary care physician, and at our last follow-up, she was autoimmunologically negative. Our patient had presented in typical fashion, with compressive and obstructive symptoms of the maxillary sinus that resulted in pain, congestion, and rhinorrhea. However, we found no evidence of frank orbital invasion or compression of the infraorbital nerve.
Pseudotumors can be treated either medically or surgically. The mainstay of medical therapy is an oral or intravenous steroid, with or without radiation therapy. (13,5,6) The nonsclerosing subtypes of orbital inflammation respond to steroids rapidly and dramatically, whereas the sclerosing variant that develops as a chronic, slowly progressive disease responds poorly. (2,5,6)
Our patient was initially treated with a course of oral prednisone and nasal steroids without experiencing any symptomatic relief. After we debulked her mass endoscopically, we noted an improvement in her clinical response to steroid administration. Some evidence suggests that the sclerosing variant is more likely to respond to oral steroids if the duration of the disease course has been brief. (2)
Brannan advocated the use of high-dose (2 mg/kg) oral prednisolone or intravenous methylprednisolone as initial therapy followed by radiotherapy and/or surgical debulking. (2) Adjunctive treatments with chemotherapeutic drugs (e.g., cyclophosphamide) and immune system modulators (e.g., rituximab and azathioprine) used in a limited fashion have been successful, and these regimens are emerging as possible treatment alternatives to steroid therapy. (2,6) However, because of the limited prevalence of the sclerosing variant, there is no specific guideline for its treatment.
Surgical excision or debulking of sclerotic lesions is another viable treatment alternative. (2,5) Mombaerts et al described a cohort of 23 patients who experienced complete cure following either total surgical excision (n = 9) or debulking (n = 14) of orbital pseudotumors, with or without adjunctive corticosteroid or radiation therapy. (5) Seven of these patients were successfully treated with surgery alone, and another 4 were successfully treated with surgery alone after a steroid trial had failed.
Our patient had residual fibrotic lesions refractory to steroid treatment, but she responded well to surgical excision with adjunctive steroid therapy. However, the need for complete excision remains uncertain. Jakobiec and Jones (4) and Jakobiec and Font (7) have noted that incomplete surgical excision may result in a worsening of symptoms caused by a reactivation of the immune system. However, that contention has been disputed by Brannan (2) and Mombaerts et al, (5) who have noted that a partial excision may provide significant symptomatic relief secondary to an inflammatory "burnout" phenomenon. Unfortunately, there is no standard of care at this time, and treatment is left to the discretion of the practitioner in each situation.
Regardless of treatment, the prognosis for patients with sclerosing inflammatory lesions is favorable, as most experience a complete resolution of symptoms. Our own experience was consistent with this finding. Practicing otolaryngologists must be cognizant of this disease process and its appropriate treatment possibilities. Furthermore, it is imperative that practitioners consider systemic inflammatory diseases when a patient presents with an isolated inflammatory pseudotumor, which may be a harbinger of more widespread disease.
In conclusion, as this case shows, sclerosing inflammatory pseudotumor can be localized to the maxillary sinus without orbital involvement. The traditional treatment of oral steroids for nonsclerosing pseudotumors may not be effective for patients with the fibrosclerotic variant. Surgical excision is a viable treatment alternative. Clinicians should be aware of the rare presentation of fibrosclerotic pseudotumor when exploring a mass lesion of an inflammatory nature in the maxillary sinus.
The authors acknowledge Clarence C. Whitcomb, MD, for his histopathologic review and comments.
(1.) Oguz KK, Kiratli H, Oguz O, et al. Multifocalfibrosclerosis: A new case report and review of the literature. Eur Radiol 2002;12(5):1134-8.
(2.) Brannan PA. A review ofsclerosingidiopathic orbital inflammation. Curr Opin Ophthalmo12007;18(5):402-4.
(3.) Dehner LP, Coffin CM. Idiopathic fibrosclerotic disorders and other inflammatory pseudotumors. Semin Diagn Pathol 1998; 15 (2): 16173.
(4.) Jakobiec FA, Jones IS. Orbital inflammations. In: Duane TD, ed. Clinical Ophthalmology. Hagerstown, Md.: Harper and Row; 1980.
(5.) Mombaerts I, Schlingemann RO, Goldschmeding R, et al. The surgical management of lacrimal gland pseudotumors. Ophthalmology 1996;103(10):1619-27.
(6.) Paris GL, Waltuch GF, Egbert PR. Treatment of refractory orbital pseudotumors with pulsed chemotherapy. Ophthal Plast Reconstr Surg 1990;6(2):96-101.
(7.) Jakobiec FA, Font FL. Noninfectious orbital inflammations. In: Spencer WH, ed. Ophthalmic Pathology: An Atlas and Textbook. 3rd ed. Philadelphia: W.B. Saunders; 1985-86:2777-95.
Richard J. Vivero, MD; Pooja H. Doshi, MD; Jean Anderson Eloy, MD; Carmen Gomez-Fernandez, MD; Roy R. Casiano, MD
From the Department of Otolaryngology-Head and Neck Surgery (Dr. Vivero and Dr. Casiano) and the Department of Pathology (Dr. Gomez- Fernandez), University of Miami M filer School of Medicine, Miami, Fla.; the Department of Radiology, Lennox Hill Hospital, New York City (Dr. Doshi); and the Department of Otolaryngology, University of Medicine and Dentistry of New Jersey, Newark (Dr. Eloy). The case described in this article occurred at the University of Miami.
Corresponding author: Richard J. Vivero, MD, Department of Otolaryngology-Head and Neck Surgery, UMHC Suite 4025, 1475 NW 12th Ave., Miami, FL 33136. Email: email@example.com
Previous presentation: The information in this article has been updated from its original presentation as a poster at the annual meeting of the American Academy of Otolaryngology-Head and Neck Surgery; Sept. 21-24, 2008; Chicago.
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|Title Annotation:||ORIGINAL ARTICLE|
|Author:||Vivero, Richard J.; Doshi, Pooja H.; Eloy, Jean Anderson; Gomez-Fernandez, Carmen; Casiano, Roy R.|
|Publication:||Ear, Nose and Throat Journal|
|Date:||Dec 1, 2011|
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