Mesenchymal Neoplasms and Other Lesions
Eosinophilic Angiocentric Fibrosis
Definition
A rare benign cause of submucosal thickening and fibrosis within the upper respiratory tract.
Etiology
The etiology is unknown. It has been suggested that previous nasal surgery, some form of chronic inflammation, or autoimmune factors may be relevant, but this is largely supposition and, despite the marked eosinophilia, no allergic or parasitic cause has been shown.
Synonyms
First described in 1985, eosinophilic angiocentric fibrosis has been regarded as a possible submucosal variant of granuloma faciale.1,2
Incidence
This is an extremely rare condition with around 32 cases reported in the literature, although it is certain that many cases go undiagnosed.1–21
Site
This rare condition can affect any part of the upper respiratory tract but is most often seen in the nasal cavity and midfacial soft tissues.
Diagnostic Features
Clinical Features
The patient usually presents with slow onset of nasal obstruction, which can be unilateral or bilateral depending on the area affected. Facial pain and bleeding are not generally present. The septum and anterior columella are most often affected and the abnormal tissue may form a visible swelling. The process can then spread into the external nose and involve the superficial facial tissues so that a diffuse swelling is then seen on the external nose, cheeks, glabella, or frontal region. In some patients, the process spreads into the orbit, producing progressive proptosis and/or hypertelorism.
This condition can very rarely affect the subglottis (two cases) but multisite involvement has not been described. The orbit is also occasionally involved.22
Our clinical cohort comprises 7 cases (Table 8.1) comprising 2 men and 5 women, aged 46 to 68 years (mean 54 years). In 28 of the additional cases reported in the literature, there are 13 men and 15 women, age range 19 to 64 years (mean 45 years) (Table 8.2).
Imaging
The changes are nonspecific, a soft tissue infiltrative process that is homogeneous and not encapsulated or delineated (Figs. 8.1, 8.2, 8.3). The septum is most frequently affected and appears thickened, especially anteriorly.15 This swelling then extends to the lateral wall and beyond, although the cavities of the sinuses are generally spared in the early stages of the process. Bone destruction is not regarded as a common feature of this disease,7 but in several of our cases the lamina papyracea was eroded to involve the orbit.
Histological Features and Differential Diagnosis
Smooth submucosal mass(es) can be seen. Microscopically the tissue shows a marked eosinophilic vasculitis involving the submucosal capillaries and venules.3,4,6–9 Typically a concentric, perivascular fibrosis (“onion-skin” type) is seen.2 While eosinophils predominate, plasma cells and T lymphocytes are common in the early stages. However, true granulomas, giant cells, and necrosis are not generally seen.4,5,9 As other inflammatory cells progressively reduce in number, eosinophils increase further. There are some similarities to granuloma faciale but “onion-skin whorling” is not seen in granuloma faciale.
Due to its rarity, slow and indolent growth, and rather nonspecific findings of inflammation and fibrosis, this condition may not be recognized by the unsuspecting general pathologist and diagnosis is often delayed.
The differential diagnosis includes fibrosing forms of invasive fungal disease, midline granulomatous diseases such as Wegener’s granulomatosis and sarcoid, Kimura disease, angiolymphoid hyperplasia, and neurogenic and mesenchymal tumors.
Natural History
Although benign, this process can continue to spread insidiously and produce significant functional and cosmetic deformity. The infiltration of the orbit can eventually lead to visual problems due to the mass effect and corneal exposure. It has most often been diagnosed by accident when septal surgery has been undertaken for nasal obstruction.
Treatment
The only effective treatment is complete surgical excision. This can be difficult due to the extent of infiltration and the poorly defined nature of the process but should be done sooner rather than later. As this may produce significant cosmetic problems, surgeons may be tempted to be conservative but failure to completely excise the tissue will lead to repeated “recurrences” and multiple operations leading ultimately to problems as significant if not worse. Of the 32 cases reported in the literature, over half had residual disease or the outcome was not reported. In our 7 cases, 5 of whom have been under long-term follow-up, only 1 has residual disease and 4 are clear due to radical surgery.
An endoscopic approach may be possible in limited disease but generally an external procedure is required to adequately access the superficial facial tissues and/or orbit. In our cohort, external rhinoplasty,2 lateral rhinotomy,3 a coronal approach to the frontal region1 and a total rhinectomy1 have been employed.
Medical therapies have included nasal, oral, and intralesional steroids with some limited success, and hydroxychloroquine. Dapsone has been given because it has proved beneficial in granuloma faciale, again with little response in our patients.23,24 Radiotherapy is not recommended.
Outcome
Untreated, the process will continue inexorably although generally rather slowly over many years. It is not known to undergo malignant transformation.
References
1. Holmes DK, Panje WR. Intranasal granuloma faciale. Am J Otolaryngol 1983;4(3):184–186 2. Roberts PF, McCann BG. Eosinophilic angiocentric fibrosis of the upper respiratory tract: a mucosal variant of granuloma faciale? A report of three cases. Histopathology 1985;9(11):1217–1225 3. Fageeh NA, Mai KT, Odell PF. Eosinophilic angiocentric fibrosis of the subglottic region of the larynx and upper trachea. J Otolaryngol 1996;25(4):276–278 4. Altemani AM, Pilch BZ, Sakano E, Altemani JM. Eosinophilic angiocentric fibrosis of the nasal cavity. Mod Pathol 1997;10(4):391–393 5. Roberts PF, McCann BG. Eosinophilic angiocentric fibrosis of the upper respiratory tract: a postscript. Histopathology 1997;31(4):385–386 6. Matai V, Baer S, Barnes S, Boxer M. Eosinophilic angiocentric fibrosis. J Laryngol Otol 2000;114(7):563–564 7. Burns BV, Roberts PF, De Carpentier J, Zarod AP. Eosinophilic angiocentric fibrosis affecting the nasal cavity. A mucosal variant of the skin lesion granuloma faciale. J Laryngol Otol 2001;115(3):223–226 8. Loane J, Jaramillo M, Young HA, Kerr KM. Eosinophilic angiocentric fibrosis and Wegener’s granulomatosis: a case report and literature review. J Clin Pathol 2001;54(8):640–641 9. Thompson LD, Heffner DK. Sinonasal tract eosinophilic angiocentric fibrosis. A report of three cases. Am J Clin Pathol 2001;115(2):243–248 10. Pereira EM, Millas I, Reis-Filho JS, Maeda SA, Franco M. Eosinophilic angiocentric fibrosis of the sinonasal tract: report on the clinicopathologic features of a case and review of the literature. Head Neck 2002;24(3):307–311 11. Tabaee A, Zadeh MH, Proytcheva M, LaBruna A. Eosinophilic angiocentric fibrosis. J Laryngol Otol 2003; 117(5):410–413 12. Nguyen DB, Alex JC, Calhoun B. Eosinophilic angiocentric fibrosis in a patient with nasal obstruction. Ear Nose Throat J 2004;83(3):183–184, 186 13. Onder S, Sungur A. Eosinophilic angiocentric fibrosis: an unusual entity of the sinonasal tract. Arch Pathol Lab Med 2004;128(1):90–91 14. Narayan J, Douglas-Jones AG. Eosinophilic angiocentric fibrosis and granuloma faciale: analysis of cellular infiltrate and review of literature. Ann Otol Rhinol Laryngol 2005;114(1 Pt 1):35–42 15. Paun S, Lund VJ, Gallimore A. Nasal fibrosis: long-term follow up of four cases of eosinophilic angiocentric fibrosis. J Laryngol Otol 2005;119(2):119–124 16. Clauser L, Mandrioli S, Polito J, Marchetti E. Eosinophilic angiocentric fibrosis. J Craniofac Surg 2006;17(4):812–814 17. Watanabe N, Moriwaki K. Atypical eosinophilic angiocentric fibrosis on nasal septum. Auris Nasus Larynx 2006;33(3):355–358 18. Jain R, Robblee JV, O’Sullivan-Mejia E, et al. Sinonasal eosinophilic angiocentric fibrosis: a report of four cases and review of literature. Head Neck Pathol 2008;2(4):309–315 19. Kosarac O, Luna MA, Ro JY, Ayala AG. Eosinophilic angiocentric fibrosis of the sinonasal tract. Ann Diagn Pathol 2008;12(4):267–270 20. Fanlo P, Perez C, Ibanez J, Zubimendi K, Arbizu L, Bragado FG. Eosinophilic angiocentric fibrosis: an unusual entity producing saddle nose and nasal septal perforation. APMIS 2009;117(Suppl S127):100 21. Sunde J, Alexander KA, Reddy VV, Woodworth BA. Intranasal eosinophilic angiocentric fibrosis: a case report and review. Head Neck Pathol 2010;4(3):246–248 22. Leibovitch I, James CL, Wormald PJ, Selva D. Orbital eosinophilic angiocentric fibrosis case report and review of the literature. Ophthalmology 2006;113(1):148–152 23. Goldner R, Sina B. Granuloma faciale: the role of dapsone and prior irradiation on the cause of the disease. Cutis 1984;33(5):478–479, 482 24. van de Kerkhof PC. On the efficacy of dapsone in granuloma faciale. Acta Derm Venereol 1994;74(1):61–62Myxoma
Definition
Myxoma is a benign soft tissue tumor characterized by fibroblasts, myofibroblasts, and scattered vessels that are embedded in a hypovascular myxoid stroma. Usually myxomas contain only minimal amounts of thin collagen fibers within a mucus-rich stroma; where larger amounts of collagen exist they are sometimes referred to as fibromyxomas. Cardiac myxomas and intramuscular myxomas in the extremities are by far the most commonly encountered forms of these tumors (ICD-O 8840/0).
They are rare in the head and neck and comprise three different forms: (1) odontogenic myxoma; (2) soft tissue myxoma, which may occur within muscles; and (3) cutaneous or superficial angiomyxoma.
Etiology
There are no known etiological factors, but cutaneous myxomas may involve the head and neck and, at times, when they are multiple, particularly when they are in the eyelids or external ear, they may be associated with the rare Carney’s complex.1 Odontogenic myxomas, which may become large enough to involve the nasal cavity and paranasal sinuses, are not associated with the Carney’s complex or any other genetic lesions.
Incidence and Site
The intraosseous odontogenic myxoma represents over 60% of all noncutaneous myxomas affecting the head and neck, with approximately two-thirds of them involving the mandible and the remainder the maxilla.2 The most common site of these odontogenic myxomas in the maxilla is the posterior aspect of the alveolar region and they represent a high proportion of odontogenic tumors in children. The slowly growing mass tends to obliterate the maxillary sinus. The origin in the toothbearing areas of the gnathic bones, the presence of odontogenic epithelial structures, and the resemblance to dental papillae support the thesis that these tumors arise from odontogenic mesenchyme.3 (See Chapter 12.)
In contrast, true sinonasal myxomas, while being extremely rare in the literature, appear to arise in the anterior maxillary wall with no evidence of odontogenic origin.4
Diagnostic Features
Clinical Features
Heffner4 presented four cases from the tumor files of the Armed Forces Institute of Pathology, all being children between the ages of 1 and 12 years. The anterior maxillary wall appeared to be involved in all lesions, the main symptoms being cheek and facial swelling accompanied by pain. When these true sinonasal tumors are more advanced, symptoms of nasal block and epistaxis also occur.5 In contrast to these pediatric cases, rare cases of myxoma developing in the sphenoid sinus have been reported in adults.6,7
We have treated one case, a 14-month-old West Indian girl who presented with a large disfiguring mass in the right nasal cavity that was increasing in size in 1985 (Fig. 8.4). This was biopsied via a sublabial approach and, following histological confirmation as a myxoma, was excised via a lateral rhinotomy. The lesion appeared to arise within the maxilla and had breached the anterior and superior walls. She remained free of disease for the next 7 years of follow-up. This treatment predated the regular use of the midfacial degloving approach in our unit, and today this lesion would undoubtedly have been biopsied and excised endoscopically. With either approach, a facial incision would have been avoided in a young child.
Histological Features and Differential Diagnosis
Heffner found the tissue in all his sinonasal pediatric cases to be mostly soft and semigelatinous. The myxoid neoplasm contains a relatively uniform distribution of spindled and stellate cells and although the nuclei are slightly enlarged, they are not hypochromatic or anaplastic. Mitoses are rare. There are no cross-striations and no densely cellular areas. Additionally, others have commented on the wispy strands of thin collagen that appear to blend with the delicate cytoplasmic processes on the lesional cells. These sinonasal myxomas appeared more cellular than most myxomas and potentially could be confused with rhabdomyosarcoma in these young patients. However, recent advances in immunohistochemistry and our knowledge of rhabdomyosarcoma make this far less likely.
Treatment
The gelatinous and friable nature of these lesions is associated with infiltrative capacity into surrounding bone and makes complete removal by simple curettage unreliable. Despite the limited literature, they obviously have a propensity to recur and, while radical surgery is not indicated, they do need thorough local removal by endoscopic or open surgical approaches with at least a few millimeters of adjacent bone, cartilage, and mucosa. If this is not done, they may further invade the paranasal sinuses, skull base, and cranial cavity.4,8
As with many of the other benign but locally invasive neoplasms within the nose, sinuses, and skull base, the possibility of local recurrence, even years later, requires long-term follow-up, good patient education regarding the possibility of recurrence, and appropriate re-imaging as necessary.
References
1. Carney JA, Gordon H, Carpenter PC, Shenoy BV, Go VL. The complex of myxomas, spotty pigmentation, and endocrine overactivity. Medicine (Baltimore) 1985;64(4):270–283 2. Lo Muzio L, Nocini P, Favia G, Procaccini M, Mignogna MD. Odontogenic myxoma of the jaws: a clinical, radiologic, immunohistochemical, and ultrastructural study. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1996;82(4):426–433 3. Harrison JD. Odontogenic myxoma: ultrastructural and histochemical studies. J Clin Pathol 1973;26(8):570–582 4. Heffner DK. Sinonasal myxomas and fibromyxomas in children. Ear Nose Throat J 1993;72(5):365–368 5. Prasannan L, Warren L, Herzog CE, Lopez-Camarillo L, Frankel L, Goepfert H. Sinonasal myxoma: a pediatric case. J Pediatr Hematol Oncol 2005;27(2):90–92 6. Andrews T, Kountakis SE, Maillard AA. Myxomas of the head and neck. Am J Otolaryngol 2000;21(3):184–189 7. Sato H, Gyo K, Tomidokoro Y, Honda N. Myxoma of the sphenoidal sinus. Otolaryngol Head Neck Surg 2004;130(3):378–380 8. Fu Y, Perzin K. Non-epithelial tumors of the nasal cavity, paranasal sinuses and nasopharynx: a clinicopathologic study. VII. Myxomas. Cancer 1977;39:195–203Borderline and Low Malignancy–Potential Tumors of Soft Tissue
The sarcomas described in this book are rare in comparison with the many benign soft tissue tumors that can occur in the head and neck of both children and adults. The benign tumors and tumorlike lesions of the head and neck are often different from their soft tissue counterparts in other sites of the body. Some have an increased potential for recurrence, such as desmoid type fibromatosis, glomangiopericytoma and angiofibroma. They may even require adjuvant treatment to control the local disease, even when they are nonmetastasizing benign lesions. Atypical variants of these low-grade tumors and tumorlike lesions have at times in the past been confused with sarcomas and the names and classification of these lesions have changed considerably over recent years, which unfortunately can be a source of confusion in the literature. However, increasing evidence from ultrastructural studies, immunohistochemistry, and recently cytogenetics, is helping to clarify the continually evolving classification of these tumors when added to long-term knowledge of the clinical features and morphology. A good example is glomangiopericytoma (sinonasal hemangiopericytoma), which in the WHO classification is regarded as a soft tissue tumor of borderline malignant potential but which we have included with the vasoform tumors because of its relationship to the pericyte.
Desmoid-Type Fibromatosis
Definition
(ICD-O code 8821/1)
Desmoid-type fibromatosis is a locally aggressive lesion composed of bland cellular spindled cells of the myofibroblastic phenotype. It is comprised of intraabdominal, abdominal, and extra-abdominal types. It does not metastasize.
Synonyms
The terms infantile fibromatosis, juvenile desmoid-type fibromatosis, aggressive fibromatosis, desmoid tumor, extra-abdominal desmoid, and extra-abdominal fibromatosis have all been commonly used in the literature.
Incidence and Site
Approximately 15% of cases of desmoid-type fibromatosis occur in the head and neck, but it is rare in the sinonasal tract. All ages can be affected but it is more common in children, and the so-called infantile aggressive fibromatosis represents the childhood version of the desmoid tumors seen in adults.1–3 The lesions are common in patients with familial adenomatous polyposis, an autosomal inherited disease caused by a genetic mutation in the APC tumor suppressor gene.4,5
We have treated two patients, both children, aged 2 and 4 years. In the 2-year-old girl the condition affected the antrum and the ethmoid and impinged on the orbit. In the 4-year-old boy only the maxilla was involved.
Diagnostic Features
Clinical Features and Imaging
These relatively slow-growing lesions in the sinonasal tract have presented with nasal obstruction, epistaxis, facial swelling, facial pain, proptosis, loosening of teeth, nonhealing tooth sockets following extraction, and swelling of the hard palate.1–3,6 Both of our patients presented with a mass in the cheek and in one case orbital displacement.
On examination any visible lesions within the nasal cavity often have a glistening quality, but in contrast to simple nasal polyps they are usually rubbery and firm. They may measure up to several centimeters in size with displacement of both the lateral wall of the nose and nasal septum, intraorbital extension, and skull base erosion.4 Occasionally there may be multiple lesions, particularly when associated with Gardener’s syndrome.7 Imaging by CT usually shows a nonspecific mildly enhancing heterogeneous soft tissue mass.
Histological Features and Differential Diagnosis
On macroscopic examination fibromatosis is usually pinkish-white in color and firm and rubbery in consistency with infiltration into surrounding tissues. It usually comprises of fascicles of bland-looking spindle cells and collagen fibers, frequently arranged in a fairly uniform manner and with elongated blood vessels that tend to run parallel to each other. The spindle cells have a myofibroblastic appearance and the nuclei are usually ovoid with indistinct nucleoli. Normal mitosis may be present but atypical mitosis and necrosis are not features of fibromatosis. The matrix may contain keloidlike collagen and myxoid areas.
The main differential diagnosis in the sinonasal area is between variants of fibrosarcoma and malignant fibrous histiocytoma, and while in classical lesions immunohistochemistry may not be required, the myofibroblasts may be positive for actins and vimentin, but are usually negative for desmin, S100 protein, HMB 45, keratins, and CD 34. Beta-catenin is an important genetic immunohistochemistry marker showing nuclear reactivity in fibromatosis.
It is important to separate the fibromatosis in the head and neck region from a variety of benign and malignant lesions, since although it is locally aggressive and may be difficult to excise, it does not require the same level of treatment as more concerning lesions, such as the fibrosarcoma variants. Fortunately, β-catenin, while being specific to desmoid-type fibromatosis, is either negative or shows only cytoplasmic reactivity in all of the other tumors.
Treatment and Prognosis
Desmoid-type fibromatosis in the sinonasal area may be difficult to excise and can erode bone of the paranasal sinus and skull base area, but with modern approaches by midfacial degloving and craniofacial techniques, prognosis in these rare lesions should be excellent, and it is to be hoped that the quoted 20% recurrence rates from publications derived from the 1980s and 1990s can be further reduced by obtaining adequate surgical margins. As the lesion does not metastasize, total removal to prevent local recurrence is the primary aim. Both radiotherapy and chemotherapy have been advocated for aggressive and recurrent surgically nonresectable lesions. This has potential problems, particularly in the long term, for the children, given that this is a benign nonmetastasizing tumor.8–10
Wehl et al11 reported the results of liposomal doxorubicin in three children with progressive fibromatosis in the nasal cavity, oral cavity, infratemporal fossa, supraclavicular fossa, and abdomen. Tumor response was confirmed by MRI in all children without major short-term complications.
Both our young patients were treated successfully with surgery. The young boy treated in the early 1980s underwent a lateral rhinotomy; the little girl had a midfacial degloving for her extensive lesion.
Extrapleural Solitary Fibrous Tumors
Definition
(ICD-O code 8815/1)
Solitary fibrous tumors are tumors of CD34-positive fibroblasts. This mesenchymal spindle cell neoplasm was originally found in pleural tissue but subsequently numerous extrapleural sites have been described, including the upper respiratory tract.
Etiology, Incidence, and Site
There are no known etiological factors and the tumor is extremely rare. Hicks and Moe reported a nasal case arising from the anterior cranial fossa, estimated to be the fourteenth case in the English literature.12 Tumors have been reported affecting the nasal cavity, paranasal sinuses, and nasopharynx. We have seen two patients, one a man aged 37 years who underwent removal of a solitary lesion on the lateral nasal wall via a lateral rhinotomy in the late 1960s and was free of disease at the end of 5 years’ follow-up. The second, a 53-year-old man, recently underwent surgical removal via an endoscopic approach (Fig. 8.5).
Diagnostic Features
Clinical Features and Imaging
A wide range of ages and approximately equal numbers of males and females have been reported. Recorded patients present with the usual nonspecific symptoms of nasal obstruction and epistaxis and in those cases where the tumor can be visualized in the nose it is usually described as polypoid and firm. Substantial hemorrhage is a feature of note on biopsy. Approximately half the tumors recorded have infiltrated the ethmoid sinuses and extend posteriorly into the nasopharynx, but only a single reported case breached the anterior skull base. Imaging features have been nonspecific but obviously essential to accurately delineate the size of the lesion.
Histological Features, Immunohistochemical Features, and Differential Diagnosis
These tumors are usually well circumscribed but with a variable proliferation of cells with appearances ranging from plump to spindle. A nondescript growth pattern associated with “ropey” keloidal collagen bundles interlaced with thin-walled vascular spaces appears to be the most common element. Vascular spaces may be prominent, exhibiting a hemangiopericytoma-like pattern.
Immunohistochemical testing results in prominent staining for CD34 and variable reactivity for vimentin, and is generally negative for actin. Additionally, staining is usually negative for keratin, epithelial membrane antigen, S100 protein, factor 8-related antigen, carcinoembryonic antigen, desmin, HHF-35, and glial fibrillary acidic protein.
The differential diagnosis includes glomangiopericytoma, fibrous histiocytoma, leiomyoma, schwannoma, synovial sarcoma, and fibrous sarcoma.
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