SINONASAL TRACT

Sinonasal Tract Mucoepidermoid Carcinoma: A Clinicopathologic and Immunophenotypic Study of 19 Cases Combined with a Comprehensive Review of the Literature.

Wolfish EB, Nelson BL, Thompson LD.

Head Neck Pathol. 2011 Dec 20. [Epub ahead of print]

Primary sinonasal tract mucoepidermoid carcinomas (MEC) are uncommon tumors that are frequently misclassified, resulting in inappropriate clinical management. The design of this study is retrospective. Nineteen cases of MEC included 10 females and 9 males, aged 15-75 years (mean, 52.7 years); males, on average were younger by a decade than females (47.2 vs. 57.7 years). Patients presented most frequently with a mass, obstructive symptoms, pain, and/or epistaxis present for a mean of 12.6 months. The majority of tumors involved the nasal cavity alone (n = 10), maxillary sinus alone (n = 6), or a combination of the nasal cavity and paranasal sinuses (n = 3) with a mean size of 2.4 cm. Most patients presented at a low clinical stage (n = 15, Stage I & II), with only 4 patients presenting with Stage III disease. Histologically, the tumors were often invasive (bone or perineural invasion), with invasion into minor mucoserous glands. Surface involvement was common. The neoplastic cells were composed of a combination of squamoid cells, intermediate cells, and mucocytes. Cystic spaces were occasionally large, but the majoritywere focal to small. Pleomorphism was generally low grade. Necrosis (n = 5) and atypical mitotic figures (n = 6) were seen infrequently. Over half of the tumors were classified as low grade (n = 11), with intermediate (n = 4) and high grade (n = 4) comprising the remainder. Mucicarmine was positive in all cases tested. Immunohistochemical studies showed positive reactions for keratin, CK5/6, p63, CK7, EMA, and CEA in all cases tested, while bcl-2 and CD117 were rarely positive. GFAP, MSA, TTF-1, and S100 protein were non-reactive. p53 and Ki-67 were reactive to a variable degree. MEC need to be considered in the differential diagnosis of a number of sinonasal lesions, particularly adenocarcinoma and necrotizing sialometaplasia. The patients were separated into stage I (n = 9), stage II (n = 6), and stage III (n = 4), without any patients in stage IV at presentation. Surgery occasionally accompanied by radiation therapy (n = 2) was generally employed. Six patients developed a recurrence, with 5 patients dying with disease (mean, 2.4 years), while 14 patients are either alive (n = 9) or had died (n = 5) of unrelated causes (mean, 14.6 years). MEC probably arises from the minor mucoserous glands of the upper aerodigestive tract, usually presenting in patients in middle age with a mass. Most patients present with low stage disease (stage I and II), although invasive growth is common. Recurrences develop in about a third of patients, who experience a shorter survival (mean, 6.5 years). The following parameters, when present, suggest an increased incidence of recurrence or dying with disease: size ≥4.0 cm (P = 0.034), high mitotic count (P = 0.041), atypical mitoses (P = 0.007), mixed anatomic site (P = 0.032), development of recurrence (P = 0.041), high tumor grade (P = 0.007), and higher stage disease (P = 0.027).

PMID: 22183767

See full article (<1.2 MB .pdf)

Sinonasal-type hemangiopericytoma: a clinicopathologic and immunophenotypic analysis of 104 cases showing perivascular myoid differentiation.

Thompson LD, Miettinen M, Wenig BM.

Am J Surg Pathol. 2003 Jun;27(6):737-49.

Sinonasal-type hemangiopericytoma is an uncommon upper aerodigestive tract tumor of uncertain cellular differentiation. We report 104 cases of sinonasal-type hemangiopericytoma diagnosed between 1970 and 1995 from the files of the Armed Forces Institute of Pathology. There were 57 females and 47 males ranging in age from 5 to 86 years (mean 62.6 years). The most common clinical presentation was airway obstruction (n = 57) and/or epistaxis (n = 54), with symptoms averaging 10 months in duration. The tumors involved the nasal cavity alone (n = 47) or also a paranasal sinus (n = 26), were polypoid, and measured an average of 3.1 cm. Histologically, the tumors were submucosal and unencapsulated and showed a diffuse growth with fascicular (n = 37) to solid (n = 50) to focally whorled (n = 7) patterns. The tumor cells were uniform in appearance with minimal pleomorphism and had spindle-shaped (n = 82) to round/oval (n = 18) nuclei with vesicular to hyperchromatic chromatin and eosinophilic to amphophilic to clear-appearing cytoplasm with indistinct cell borders. Multinucleated (tumor) giant cells were identified in a minority of cases (n = 5). Mitotic figures were inconspicuous and necrosis was absent. The tumors were richly vascularized, including staghorn-appearing vessels that characteristically had prominent perivascular hyalinization (n = 92). An associated inflammatory cell infiltrate that included mast cells and eosinophils was noted in the majority of cases (n = 87). The immunohistochemical profile included reactivity with vimentin (98%), smooth muscle actin (92%), muscle specific actin (77%), factor XIIIa (78%), and laminin (52%). Surgery was the treatment of choice for all of the patients; adjunctive radiotherapy was given to four patients. Recurrences developed in 18 patients within 1-12 years from diagnosis. Ninety-seven patients were either alive (n = 51, mean 16.5 years) or dead (n = 46, mean 9.6 years) but free of disease. Four patients had disease at the last follow-up: three died with disease (mean 3.6 years) and one patient is alive with disease (28.3 years). Recurrent tumor (17.8%) can be managed by additional surgery. The majority of sinonasal-type hemangiopericytomas behave in a benign manner with excellent long-term prognosis (88% raw 5-year survival) following surgery alone. Sinonasal-type hemangiopericytomas have a characteristic light microscopic appearance with an immunophenotypic profile resembling that of glomus tumors.

PMID: 12766577

See full article (2 MB .pdf)

Olfactory neuroblastoma.

Thompson LD.

Head Neck Pathol. 2009 Sep;3(3):252-9. Epub 2009 Jul 16.

Few neoplasms are unique to the sinonasal tract, but sinonasal undifferentiated carcinoma and olfactory neuroblastoma are malignant tumors which require unique management. Due to the rarity of these tumors, practicing pathologists are not always aware of their distinctive clinical, radiographic, histologic, immunohistochemical, and molecular features. These cases are frequently submitted for consultation, further suggesting the diagnostic difficulties inherent to these tumors. Specifically, olfactory neuroblastoma is a neoplasm that can histologically mimic many tumors within the sinonasal tract, making recognition of this tumor important, as the management frequently requires a bicranial-facial surgical approach, a trephination procedure which can be quite technically difficult and challenging to achieve a good result. The management is therefore quite unique in comparison to other sinonasal tract malignancies, setting it apart diagnostically and managerially from other lesions.

PMID: 20596981

See full article (<1 MB .pdf)

Sinonasal tract and nasopharyngeal melanomas: a clinicopathologic study of 115 cases with a proposed staging system.

Thompson LD, Wieneke JA, Miettinen M.

Am J Surg Pathol. 2003 May;27(5):594-611.

Primary sinonasal tract mucosal malignant melanomas are uncommon tumors that are frequently misclassified, resulting in inappropriate clinical management. A total of 115 cases of sinonasal tract mucosal malignant melanoma included 59 females and 56 males, 13-93 years of age (mean 64.3 years). Patients presented most frequently with epistaxis (n = 52), mass (n = 42), and/or nasal obstruction (n = 34) present for a mean of 8.2 months. The majority of tumors involved the nasal cavity (n = 34), septum alone, or a combination of the nasal cavity and sinuses (n = 39) with a mean size of 2.4 cm. Histologically, the tumors were composed of a variety of cell types (epithelioid, spindled, undifferentiated), frequently arranged in a peritheliomatous distribution (n = 39). Immunohistochemical studies confirmed the diagnosis of sinonasal tract mucosal malignant melanomas with positive reactions for S-100 protein, tyrosinase, HMB-45, melan A, and microphthalmia transcription factor. Sinonasal tract mucosal malignant melanomas need to be considered in the differential diagnosis of most sinonasal malignancies, particularly carcinoma, lymphoma, sarcoma, and olfactory neuroblastoma. Surgery accompanied by radiation and/or chemotherapy was generally used. The majority of patients developed a recurrence (n = 79), with 75 patients dying with disseminated disease (mean 2.3 years), whereas 40 patients are either alive or had died of unrelated causes (mean 13.9 years). A TNM-type classification separated by anatomic site of involvement and metastatic disease is proposed to predict biologic behavior.

PMID: 12717245

See full article (2 MB .pdf)

Sinonasal carcinomas.

Thompson LD.

Curr Diag Pathol 2006 12, 40--53.

Malignant neoplasms of the sinonasal tract encompass a wide variety of epithelial, lymphoid and mesenchymal tumours. The separation and classification of epithelial or neuroepithelial tumours is sometimes challenging, especially when treatment and prognosis are different. Squamous cell carcinoma, keratinizing or non-keratinizing and, usually, the poorly differentiated type need to be separated from sinonasal undifferentiated carcinoma, lymphoepithelial carcinoma, neuroendocrine carcinoma and olfactory neuroblastoma. Whereas melanoma and lymphoma are also included in the broad differential, along with primitive neuroectodermal tumours and rhabdomyosarcomas, the focus of this commentary will be to present the major clinical, radiographical, histological, immunohistochemical, ultrastructural and molecular features which allow for separation of the principle mucosal epithelial neoplasms of the sinonasal tract.

PMID: n/a

See full article (2 MB .pdf)

Squamous cell carcinoma variants of the head and neck.

Thompson LDR.

Curr Diag Pathol 2003 9, 384--396.

Variants of squamous cellcarcinoma (SCC) frequently arise within the mucosa of the upper aerodigestive tract, accounting for up to15% of SCCs in these areas. The most common variants include verrucous, exophytic or papillary, spindle-cell (sarcomatoid), basaloid and adenosquamous carcinoma. Each of these variants has a unique histomorphologic appearance, which raises a number of different differential diagnostic considerations, with the attendant clinically relevant management decision.

Verrucous squamous cell carcinoma has a broad border of pushing infiltration of a non-dysplastic squamous epithelium, essentially devoid of mitotic figures, displaying hyperkeratosis on elongated rete pegs. Papillary and exophytic SCC have a papillary or exophytic architecture, but have malignant cytologic features within the epithelium. Spindle-cell (sarcomatoid) carcinoma is an SCC blended with a spindle-cell morphology, frequently mimicking other mesenchymal tumours. Epithelial markers are often negative. Basaloid SCC is a high-grade SCC variant with small cells arranged in a palisaded architecture, with hyperchromatic nuclei and only focal areas of squamous differentiation. A denosquamous carcinoma is a rare variant, which is a composite of adenocarcinoma and squamous cell carcinoma, often with areas of transition.The cytomorphologic features are described in detail in an attempt to allow the general surgical pathologist to separate these variants of SCC in order to achieve appropriate clinical management.

PMID: n/a

See full article (3 MB .pdf)

Basaloid squamous cell carcinoma of the sinonasal tract.

Wieneke JA, Thompson LD, Wenig BM.

Cancer. 1999 Feb 15;85(4):841-54.

BACKGROUND: Basaloid squamous cell carcinoma (BSCC) is a high grade, aggressive variant of squamous cell carcinoma with a predilection for the larynx, hypopharynx, tonsils, and base of the tongue. To the authors' knowledge, BSCC originating in the nasal cavity and paranasal sinuses rarely has been reported.

METHODS: Fourteen cases of BSCC involving the nasal cavity and paranasal sinuses were identified in the files of the Otolaryngic-Head and Neck Pathology Tumor Registry of the Armed Forces Institute of Pathology from 1975-1997. Clinical records and follow-up were available in all cases. Paraffin blocks were available for histochemical and immunohistochemical studies in all cases.

RESULTS: There were 7 females and 7 males, ages 32-86 years (median, 66.5 years; mean, 62 years). The patients presented primarily with a mass lesion and unilateral nasal obstruction. In nine patients the tumor was confined to the nasal cavity. In three patients the tumor involved the sinuses alone and in two patients the tumor involved the nasal cavity and paranasal sinuses. Histologically, the tumors were widely invasive with a variety of growth patterns, including lobular, solid, trabecular, cribriform, and fascicular. The neoplastic infiltrate included predominantly pleomorphic, basaloid-appearing cells with hyperchromatic nuclei, inconspicuous to prominent nucleoli, and a variable amount of eosinophilic to clear-appearing cytoplasm. Mitotic figures, including atypical forms, were readily apparent as was necrosis (individual cell and comedo-type). Foci of squamous differentiation were limited in extent but were found in all cases and included squamous whorls, individual cell keratinization, and intercellular bridges. Intraepithelial dysplasia, carcinoma in situ, or invasive squamous carcinoma was present in all cases. Other histologic features included intercellular stromal hyalinization and peripheral nuclear palisading. In two cases, neural-type rosettes were found. Immunoreactivity for a variety of epithelial markers including cytokeratin (AE1/AE3/LP34), CAM 5.2, 34betaE12, CK7, and epithelial membrane antigen was present in all cases. Variable reactivity was present with vimentin, actins (smooth muscle and muscle specific), neuron specific enolase, S-100 protein, glial fibrillary acidic protein, CK20, carcinoembryonic antigen, Leu7, and Ewing's marker. Chromogranin, synaptophysin, neurofibrillary protein, leukocyte common antigen, HMB-45, desmin, and Epstein-Barr virus latent membrane protein were absent. Surgical resection was the treatment of choice. Eight patients had recurrent or persistent tumor and metastatic disease occurred in five patients. At last follow-up, 7 patients (50%) had died of disease with a median survival of 12 months from the time of diagnosis and 3 patients were alive with disease over periods ranging from 8 months-5 years. Of the 4 remaining patients, 2 were alive without disease at 1 month and 5 years, respectively, 1 patient was lost to follow-up with no evidence of tumor at 3 years, and 1 patient had died of unrelated causes with no evidence of disease.

CONCLUSIONS: Sinonasal BSCC is a histologically distinct variant of squamous cell carcinoma with pathologic features and aggressive biologic behavior similar to BSCC localized to more common mucosal sites of the upper aerodigestive tract.

PMID: 10091761

See full article (2 MB .pdf)

Extracranial sinonasal tract meningiomas: a clinicopathologic study of 30 cases with a review of the literature.

Thompson LD, Gyure KA.

Am J Surg Pathol. 2000 May;24(5):640-50.

Extracranial meningiomas of the sinonasal tract are rare tumors. These tumors are frequently misclassified, resulting in inappropriate clinical management. To date, there has been no comprehensive study to evaluate the clinicopathologic aspects of meningioma in these anatomic sites. Thirty cases of sinonasal tract meningiomas diagnosed between 1970 and 1992 were retrieved from the files of the Otorhinolaryngic Registry of the AFIP. Histologic features were reviewed, immunohistochemical studies were performed, patient follow up was obtained, and the results were statistically analyzed. The patients included 15 females and 15 males, aged 13 to 88 years (mean, 47.6 yrs). Patients presented clinically with a mass, epistaxis, sinusitis, pain, visual changes, or nasal obstruction, dependent on the anatomic site of involvement. Symptoms were present for an average of 31.1 months. The tumors affected the nasal cavity (n = 14), nasopharynx (n = 3), frontal sinus (n = 2), sphenoid sinus (n = 2). or a combination of the nasal cavity and ethmoid, frontal, sphenoid, and/or maxillary sinuses (n = 9). The tumors ranged in size from 1.0 to 8.0 cm in greatest dimension (mean, 3.5 cm). Radiographic studies demonstrated a central nervous system connection in six cases. The tumors often eroded the bones of the sinuses (n = 18) and involved the surrounding soft tissues, the orbit, and occasionally the base of the skull. Histologically, the tumors demonstrated features similar to intracranial meningiomas. The majority were of the meningothelial type (n = 23), although there were three atypical meningiomas. Immunohistochemical studies confirmed the diagnosis of meningioma with positive reactions for epithelial membrane antigen (EMA) and vimentin (all tested). The differential diagnosis includes paraganglioma, carcinoma, melanoma, psammomatoid ossifying fibroma, and angiofibroma. Surgical excision was used in all patients. Three patients died with recurrent disease (mean, 1.2 yrs), one was alive with recurrent disease (25.6 years), and the remaining 24 patients were alive or had died of unrelated causes (mean, 13.9 yrs) at the time of last follow up (two patients were lost to follow up). Extracranial sinonasal tract meningiomas are rare tumors which need to be considered in the differential diagnosis of sinonasal tumors. A whorled growth pattern and psammoma bodies, combined with positive EMA and vimentin immunohistochemical reactions, can confirm the diagnosis of meningioma. The overall prognosis is good, without a difference in outcome between benign and atypical meningiomas.

PMID: 10800982

See full article (2 MB .pdf)

Sinonasal Tract Angiosarcoma: A Clinicopathologic and Immunophenotypic Study of 10 Cases with a Review of the Literature

Nelson BL, Thompson LDR

Head Neck Pathol 2007;1:1-12.

BACKGROUND: Primary sinonasal tract angiosarcoma are rare tumors that are frequently misclassified, resulting in inappropriate clinical management. There are only a few reported cases in the English literature.

MATERIALS AND METHODS: Ten patients with sinonasal tract angiosarcoma were retrospectively retrieved from the Otorhinolaryngic Registry of the Armed Forces Institute of Pathology.

RESULTS: Six males and four females, aged 13 to 81 years (mean, 46.7 years), presented with epistaxis and bloody discharge. Females were on average younger than their male counterparts (37.8 vs. 52.7 years, respectively). The tumors involved the nasal cavity alone (n = 8) or the maxillary sinus (n = 2), with a mean size of 4.3 cm; the average size was different between the genders: males: 2.8 cm; females: 6.4 cm. Histologically, all tumors had anastomosing vascular channels lined by remarkably atypical endothelial cells protruding into the lumen, neolumen formation, frequent atypical mitotic figures, necrosis, and hemorrhage. All cases tested (n = 6) demonstrated immunoreactivity with antibodies to Factor VIII-RA, CD34, CD31, and smooth muscle actin, while non-reactive with keratin and S-100 protein. The principle differential diagnosis includes granulation tissue, lobular capillary hemangioma (pyogenic granuloma), and Kaposi’s sarcoma. All patients had surgery followed by post-operative radiation (n = 4 patients). Follow-up was available in all patients: Six patients died with disease (mean, 28.8 months); two patients had died without evidence of disease (mean, 267 months); and two are alive with no evidence of disease at last follow-up (mean, 254 months).

CONCLUSIONS: Sinonasal tract angiosarcoma is a rare tumor, frequently presenting in middle-aged patients as a large mass usually involving the nasal cavity with characteristic histomorphologic and immunophenotypic features. Sinonasal tract angiosarcoma will often have a poor prognosis making appropriate separation from other conditions important.

PMID: DOI 10.1007/s12105-007-0017-2

See full article (<1 MB .pdf)

Primary ameloblastoma of the sinonasal tract: a clinicopathologic study of 24 cases.

Schafer DR, Thompson LD, Smith BC, Wenig BM.

Cancer. 1998 Feb 15;82(4):667-74.

BACKGROUND: Ameloblastomas are locally aggressive jaw tumors with a high propensity for recurrence and are believed to arise from the remnants of odontogenic epithelium. Extragnathic ameloblastomas are unusual and primary sinonasal tract origin is extraordinarily uncommon.

METHODS: Twenty-four cases of ameloblastoma confined to the sinonasal tract were retrieved from the Otorhinolaryngic-Head & Neck Pathology and Oral-Maxillofacial Pathology Tumor Registries of the Armed Forces Institute of Pathology between 1956 and 1996.

RESULTS: The patients included 5 females and 19 males with an age range of 43-81 years, with a mean age at presentation of 59.7 years. The patients presented with an enlarging mass in the maxillary sinus or nasal cavity (n = 24), sinusitis (n = 9), or epistaxis (n = 8). Unilateral opacification of the maxillary sinus (n = 12) was the most common radiographic finding. Histologically, the tumors exhibited the characteristic features of ameloblastoma, including peripherally palisaded columnar cells with reverse polarity. The majority of the tumors showed a plexiform growth pattern. Fifteen tumors demonstrated surface epithelial derivation. Surgical excision is the treatment of choice, ranging from conservative surgery (polypectomy) to more aggressive surgery (radical maxillectomy). Five patients experienced at least 1 recurrence, usually within 1 year of initial surgery. With follow-up intervals of up to 44 years (mean, 9.5 years), all 24 patients were alive without evidence of disease or had died of unrelated causes, without evidence of disease.

CONCLUSIONS: Primary ameloblastoma of the sinonasal tract is rare. In contrast to their gnathic counterparts, sinonasal tract tumors have a predilection for older age men. Therapy should be directed toward complete surgical resection to prevent local tumor recurrence.

PMID: 9477098

See full article (1 MB .pdf)

Primary chondrosarcoma of the head and neck in pediatric patients: a clinicopathologic study of 14 cases with a review of the literature.

Gadwal SR, Fanburg-Smith JC, Gannon FH, Thompson LD.

Cancer. 2000 May 1;88(9):2181-8.

BACKGROUND: Primary chondrosarcoma of the head and neck in the pediatric age group is rare. The literature contains several single cases and small series; however, to the authors' knowledge, there has been no previous comprehensive larger study to evaluate the clinicopathologic aspects of these tumors.

METHODS: Fourteen cases of chondrosarcoma of the head and neck from patients age 18 years or younger, diagnosed between 1970 and 1997, were retrieved from the Otorhinolaryngic-Head & Neck Tumor Registry of the Armed Forces Institute of Pathology. No secondary sarcomas (radiation-induced or arising in association with Maffucci syndrome or Ollier disease) were included. Clinical, radiographic, and histologic features were reviewed and patient follow-up obtained.

RESULTS: The patients included 6 girls and 8 boys ages 3-18 years (mean, 11.8 years). Patient symptoms (nasal stuffiness or discharge, sinusitis, headaches, or a mass lesion) were related to tumor location and were present for an average of 7.2 months. No genetic abnormalities were documented. The tumors most frequently involved the maxillary sinus (n=4), followed by the mandible (n=3), nasal cavity (n=2), and neck (n=2), with 1 each of the nasopharynx, orbit, and base of the skull. The tumors ranged in size from 2.0 to 15.0 cm (mean, 3.1 cm). All tumors were invasive and malignant as determined by radiology and/or histology. The tumors were Grade 1 (n=9), Grade 2 (n=1), or Grade 3 (mesenchymal, n=2; dedifferentiated n=2). All patients were treated by surgery, followed by radiation (n=5) and/or chemotherapy (n=2). Follow-up was available for 11 patients; all were alive (at a mean of 14.8 years), with only a single patient demonstrating evidence of residual/ recurrent tumor (at 16.6 years).

CONCLUSIONS: Primary head and neck chondrosarcoma in the pediatric population is typically low grade and occurs in the maxillary sinus or mandible. Despite the invasive and high grade nature of some of these tumors, there is an excellent long term prognosis for patients in this age group with tumors in these locations.

PMID: 9486586

See full article (<1 MB .pdf)

Primary osteosarcoma of the head and neck in pediatric patients: a clinicopathologic study of 22 cases with a review of the literature.

Gadwal SR, Gannon FH, Fanburg-Smith JC, Becoskie EM, Thompson LD.

Cancer. 2001 Feb 1;91(3):598-605.

BACKGROUND: Primary osteosarcomas of the head and neck in the pediatric age group, not associated with previous irradiation or a known syndrome, are rare. The literature contains several single cases and small study series; however, to the authors's knowledge, there has been no comprehensive large study to evaluate the clinicopathologic aspects of these tumors.

METHODS: Twenty-two cases of osteosarcomas of the head and neck in patients 18 years of age or younger, diagnosed between 1970 and 1997, were retrieved from the Otorhinolaryngic-Head & Neck Tumor Registry of the Armed Forces Institute of Pathology (AFIP). No secondary sarcomas (radiation-induced or those arising after chemotherapy) or those associated with known syndromes were included. Clinical, radiographic, and histologic features were reviewed, and patient follow-up was obtained.

RESULTS: The patients included 11 girls and 11 boys, 1-18 years of age (mean, 12.2 yrs). Patient symptoms related to tumor location were painless swelling, loss of teeth, headaches, or a mass lesion, present for an average of 5.9 months. No genetic abnormalities were documented. The tumors most frequently involved the mandible (n = 19), followed by the sphenoid sinus (n = 2) and the maxilla (n = 1). The tumors ranged in size from 1.1-10.0 cm (mean, 4.5 cm). All tumors were invasive and malignant by radiology and/or histology. The tumors were Grade 1 (n = 11), Grade 2 (n = 8), or Grade 3 (n = 3). All cases, except one chondroblastic osteosarcoma, were osteoblastic osteosarcomas. Thirteen patients underwent initial surgical resection with (n = 5) or without (n = 9) additional radiation and/or chemotherapy. The remaining 9 patients had an initial biopsy for diagnosis followed by surgery (n = 4) or surgery and radiation and/or chemotherapy (n = 5). Follow-up was available for 19 patients: 13 were alive at last follow-up with no evidence of disease (mean, 13.1 yrs); 1 was alive with disease (1.3 yrs); 3 had died without evidence of disease (mean, 23.2 yrs); and 2 had died of disease (mean, 7.8 yrs). The 3 patients with high-grade osteosarcoma were alive without disease (mean, 20.0 yrs).

CONCLUSIONS: Primary head and neck osteosarcomas in the pediatric population are typically low- to moderate-grade lesions in the mandible. Despite the invasive nature and high grade of a few of these tumors, there is an excellent overall long-term prognosis for patients in this age group with tumors in these locations.

PMID: 11169944

See full article (<1 MB .pdf)

Sinonasal tract eosinophilic angiocentric fibrosis. A report of three cases.

Thompson LD, Heffner DK.

Am J Clin Pathol. 2001 Feb;115(2):243-8.

Eosinophilic angiocentric fibrosis (EAF) is a rare submucosal fibrosis without a well-developed differential diagnosis. Three cases of sinonasal tract EAF were identified in 2 women and 1 man, aged 49, 64, and 28 years, respectively. The patients experienced a nasal cavity mass, maxillary pain, or nasal obstructive symptoms of long duration. The process involved the nasal septum (n = 2), nasal cavity (n = 1), and/or the maxillary sinus (n = 1). There was no evidence for Wegener granulomatosis, Churg-Strauss syndrome, Kimura disease, granuloma faciale, or erythema elevatum diutinum. Histologically, the lesions demonstrated a characteristic perivascular "onion-skin" fibrosis and a full spectrum of inflammatory cells, although eosinophils predominated. Necrosis and foreign body-type giant cells were not identified. Surgical excision was used for all patients, who are all alive but with disease at last follow-up. Sinonasal tract EAF is a unique fibroproliferative disorder that does not seem to have systemic associations with known diseases. The characteristic histomorphologic features permit accurate diagnosis.

PMID: 11211613

See full article (<1 MB .pdf)

Mesenchymal chondrosarcoma of the sinonasal tract: a clinicopathological study of 13 cases with a review of the literature.

Knott PD, Gannon FH, Thompson LD.

Laryngoscope. 2003 May;113(5):783-90.

OBJECTIVES/HYPOTHESIS: Mesenchymal chondrosarcoma of the sinonasal tract is a rare, malignant tumor of extraskeletal origin. Isolated cases have been reported in the English literature, with no large series evaluating the clinicopathological aspects of these tumors.

STUDY DESIGN: Retrospective review.

METHODS: Thirteen patients with sinonasal mesenchymal chondrosarcoma were retrieved from the Otorhinolaryngologic-Head and Neck Registry of the Armed Forces Institute of Pathology.

RESULTS: Nine women and 4 men (age range, 11 to 83 y; mean age, 38.8 y) presented with nasal obstruction (n = 8), epistaxis (n = 7), or mass effect (n = 4), or a combination of these. No patients reported prior head and neck irradiation. The maxillary sinus was the most common site of involvement (n = 9), followed by the ethmoid sinuses (n = 7) and the nasal cavity (n = 5). Tumors had an overall mean size of 5.1 cm. Microscopically, the tumors displayed a small, blue, round cell morphology appearance arranged in a hemangiopericytoma-like pattern with foci of cartilaginous matrix. All cases were managed by surgery with adjuvant radiation therapy (n = 4) and/or chemotherapy (n = 3). The overall mean survival was 12.1 years, although five of six patients who developed local recurrences died of disease (mean survival, 6.5 y). Six patients were alive and disease free (mean survival, 17.3 y), and two patients were lost to follow-up.

CONCLUSIONS: Mesenchymal chondrosarcoma of the sinonasal tract is an aggressive tumor with a predilection for young women. The pattern of growth and scarcity of cartilaginous matrix result in frequent misdiagnosis. Recurrence develops in approximately one-third of patients and seems to predict a poor prognosis. Aggressive, exenterative surgery combined with adjuvant therapy appears to yield the best clinical outcome.

PMID: 12792311

See full article (2 MB .pdf)

Nasal glial heterotopia: a clinicopathologic and immunophenotypic analysis of 10 cases with a review of the literature.

Penner CR, Thompson L.

Ann Diagn Pathol. 2003 Dec;7(6):354-9.

Nasal glial heterotopia (also known as "nasal glioma"), is a rare developmental abnormality seen in a wide age group but typically presenting at birth or in early childhood. Failure to recognize the entity is the principle difficulty in diagnosis. Ten cases of nasal glial heterotopic diagnosed between 1970 and 2000 were identified. Histologic and immunohistochemical features were evaluated and patient follow-up was obtained. The patients included five females and five males with a mean age at presentation of 8.6 years (range, birth to 44 years). Most patients presented clinically with a polypoid mass in the nasal cavity, although two patients had a mass on the nasal bridge. Symptoms were present for an average of 2 to 3 months. A connection to the central nervous system was identified in one case. Masses ranged in size from 1 to 7 cm in greatest dimension (mean, 2.4 cm). Histologically, the masses were composed of astrocytes (including gemistocytic type) and neuroglial fibers intermixed with a fibrovascular connective tissue stroma. Neurons and ependymal cells were noted in two cases. Focal calcifications and inflammatory cells were identified occasionally. Masson trichrome stains the collagen intensely blue, while the neural population stains magenta. Immunohistochemical reactivity with glial fibrillary acidic protein and S-100 protein will help to confirm the histologic diagnosis, while collagen type IV and laminin can highlight the reactive fibrosis. All cases were managed by surgery. All patients were alive without complications at last follow-up (mean, 26.8 years), except for the single fetus included in the study. Nasal glial heterotopia typically involves the nasal cavity and usually presents perinatally, although three patients presented in adulthood. The subtle glial component on routine microscopy can be accentuated with a trichrome stain or by immunoreactivity with glial fibrillary acidic protein and S-100 protein. Imaging studies must be performed before surgery to exclude an encephalocele, which requires different surgery. Complete surgical excision of nasal glial heterotopias is curative.

PMID: 15018118

See full article (<1 MB .pdf)

Sinonasal mucosal malignant melanoma: report of an unusual case mimicking schwannoma.

Kardon DE, Thompson LD.

Ann Diagn Pathol. 2000 Oct;4(5):303-7.

Primary mucosal melanoma of the sinonasal tract is a rare malignancy that has a more aggressive clinical course than its cutaneous counterpart. The histology of these lesions varies, with differing degrees of melanin production and an epithelioid or spindle-cell growth pattern. Cutaneous melanocytic lesions may differentiate in accordance with their neural crest derivation and express morphology similar to nerve sheath tumors. We believe the following case study reports the first instance of a mucosal melanoma with a Schwannian pattern of growth, arising from the nasal cavity of a 26-year-old man.

PMID: 11073336

See full article (<1 MB .pdf)

Malignant giant cell tumor of the sphenoid.

Chan J, Gannon FH, Thompson LD.

Ann Diagn Pathol. 2003 Apr;7(2):100-5.

Malignant giant cell tumors (MGCTs) of the sphenoid sinus are extremely rare neoplasms. They are challenging to diagnose and difficult to treat because of their skull base location. To the best of our knowledge, we report the first case of a primary MGCT of the sphenoid arising in a patient with Paget's disease. A 77-year-old man presented with epistaxis and a history of Paget's disease. There was normal cranial nerve function although radiographic images disclosed a large mass centered in the sphenoid sinus and extending into the ethmoid and maxillary sinuses. Excisional biopsy revealed a MGCT composed of a cellular stroma with increased mitotic activity and necrosis with giant cells present throughout. Additional therapy was declined and the patient died with disease 7 months later. Because of their rarity, no treatment guidelines exist for the management of MGCTs of the sphenoid. We discuss both the diagnostic and therapeutic considerations based on a review of the pertinent literature.

PMID: 12715335

See full article (<1 MB .pdf)

Replication of HIV-1 in dendritic cell-derived syncytia at the mucosal surface of the adenoid.

Frankel SS, Wenig BM, Burke AP, Mannan P, Thompson LDR, Abbondanzo SL, Nelson AM, Pope M, Steinman RM

Science 1996;272:115-117

Human immunodeficiency virus-type 1 (HIV-1) replicates actively in infected individuals, yet cells with intracellular depots of viral protein are observed only infrequently. Many cells expressing the HIV-1 Gag protein were detected at the surface of the nasopharyngeal tonsil or adenoid. This infected mucosal surface contained T cells and dendritic cells, two cell types that together support HIV-1 replication in culture. The infected cells were multinucleated syncytia and expressed the S100 and p55 dendritic cell markers. Eleven of the 13 specimens analyzed were from donors who did not have symptoms of acquired immunodeficiency syndrome (AIDS). The interaction of dendritic cells and T cells in mucosa may support HIV-1 replication, even in subclinical stages of infection.

PMID: 8600520

See full article (2.5 MB .pdf)

Allergic fungal sinusitis.

Thompson LD

Ear Nose Throat J. 2011 March;90(3):106-107.

FIRST PARAGRAPH: Allergic fungal sinusitis, also known as allergic mucin and eosinophilic fungal rhinosinusitis, is an allergic response in the sinonasal tract mucosa to aerosolized fungal allergens, amplified and perpetuated by eosinophils. The class II genes in the major histocompatibility complex are involved in antigen presentation and immune response (modulation), and an allergic reaction develops to inhaled fungal elements in immunocompetent people. Aspergillus species are the most common agents (widespread in soil, wood, and decomposing plant material), but Alternaria, Bipolaris, Curvularia, Exserohilum, and Phialophora species have also been reported.

PMID:  21412738

See full article (<1 MB .pdf)

Intestinal-type sinonasal adenocarcinoma.

Thompson LD

Ear Nose Throat J. 2010 Jan;89(1):16-8.

FIRST PARAGRAPH: Adenocarcinomas of the sinonasal tract can originate in the respiratory epithelium or the underlying mucoserous glands. Most (60%) arise from the mucoserous glands. These tumors are divided into two categories: salivary-gland-type and nonsalivary-gland-type adenocarcinomas (table). The latter are subdivided into two major categories: intestinal-type adenocarcinomas (ITACs) and nonintestinal-type adenocarcinomas.

PMID:  20155693

See full article (<1 MB .pdf)

Ewing sarcoma and primitive neuroectodermal tumor.

Thompson LDR.

Ear Nose Throat J. 2007 Feb;86(2):79-80.

FIRST PARAGRAPH: Ewing sarcoma (ES) and primitive neuroectodermal tumor (PNET) are closely related, high-grade, round-cell tumors with a neuroectodermal phenotype. These tumors are histologically considered on a morphologic spectrum, and they express similar genetic alterations. ES usually develops in bone and is more undifferentiated, while PNET tends to involve soft tissue and demonstrates more pronounced neuroendocrine features.

PMID: 17385610

See full article (<1 MB .pdf)

Rhinoscleroma.

Thompson LD

Ear Nose Throat J. 2002 Aug;81(8):506.

FIRST PARAGRAPH: Rhinoscleroma ("hard nose") is caused by Klebsiella rhinoscleromatis, a gram-negative encapsulated bacterium of low infectivity. The disease is uncommon in the United States; most cases are found in the Middle East (especially Egypt), in parts of Latin America, and in Eastern Europe. The disease process usually involves the nasal cavity and the nasopharynx, but it can also involve the larynx, trachea, bronchi, middle ear, and orbit.

PMID:  12199166

See full article (<1 MB .pdf)

Update on Nasopharyngeal Carcinoma.

Thompson LDR

Head Neck Pathol J 2007-02

PMID: n/a

See full article (<1 MB .pdf)

Nasopharyngeal carcinoma.

Thompson L.

Ear Nose Throat J.  2005 Jul;84(7):404-5.

FIRST PARAGRAPH: The most common type of nasopharyngeal tumor is a carcinoma. The etiology of nasopharyngeal carcinoma (NPC) is multifactorial; race, genetics, Epstein-Barr virus (EBV) infection, and the environment all play a role. NPC is rare in white populations, but it is one of the most common cancers among Chinese. EBV is almost always present in NPC, indicating that this virus plays an oncogenic role. The viral titer can be used to monitor therapy or possibly as a diagnostic tool in the evaluation of patients who present with a metastasis from an unknown primary. Exposure to environmental carcinogens, especially high levels of volatile nitrosamines (specifically, those in Cantonese-style salted fish), has been implicated in this complicated disorder; carcinogens related to smoking, formaldehyde exposure, and radiation have also been implicated.

PMID: 16813025

See full article (<1 MB .pdf)

Olfactory neuroblastoma.

Thompson L.

Ear Nose Throat J. 2006 Sep;85(9):569-70.

FIRST PARAGRAPH: Olfactory neuroblastoma (esthesioneuroblastoma) is an uncommon malignant neuroectodermal nasal tumor that accounts for approximately 5% of all malignant neoplasms. Olfactory neuroblastomas are thought to arise from the specialized sensory neuroepithelial (neuroectodermal) olfactory cells that are normally found in the upper part of the nasal cavity, usually including the cribriform plate of the ethmoid sinus. These tumors affect both sexes equally. A bimodal age distribution (the 2nd and 6th decades of life) has been documented, although patients of all ages can be affected. Patients present with nonspecific symptoms of nasal obstruction (70% of cases) and epistaxis (50%); less common symptoms include headache, pain, visual disturbances, and anosmia (<5%). Owing to the nonspecific nature of the presenting symptoms, patients often have a long history prior to diagnosis.

PMID: 17044420

See full article (<1 MB .pdf)

Sinonasal polyps.

Thompson LD

Ear Nose Throat J. 2007 Jun;86(6):322, 325.

FIRST PARAGRAPH: Sinonasal polyps are caused by a multitude of factors. The most common causes are repeated bouts of sinusitis, allergy, vasomotor rhinitis, infectious rhinosinusitis, and asthma. Less often, they occur in association with diabetes mellitus, cystic fibrosis, and aspirin intolerance. They form as a result of an influx of fluids into the schneiderian mucosal lamina propria. Occasionally, antral (maxillary) polyps expand and prolapse through sinus ostia to present intranasally or in the nasopharynx (antrochoanal polyps). Sinonasal polyps have no predisposition to age or sex. Polyps are uncommon in children, but when they do occur, as many as 30% are associated with cystic fibrosis.

PMID: 17703805

See full article (<1 MB .pdf)

Pharyngeal dermoids (“hairy polyps”) as accessory auricles.

Heffner DK, Thompson LDR, Schall D, Anderson V

Ann Otol Rhinol Laryngol 1996;105:819-824

The purpose of this study is to clarify the origin and nature of so-called hairy polyps or dermoids of the pharynx, which are often thought to be a variant of pharyngeal teratoma. For this purpose, a case is reported of a dermoid polyp involving the middle ear of an infant, the features of multiple examples of pharyngeal dermoid polyps and teratomas received for consultation by the Armed Forces Institute of Pathology are examined, and selected pertinent reports from the literature are reviewed. All three means are used to support the conclusion that these lesions are choristomatous developmental anomalies arising from the first branchial cleft area and that they essentially represent heterotopic accessory "ears" (auricles) without the growth potential of a teratoma.

PMID: 8865778

Nasal glial heterotopia.

Penner CR, Thompson LD.

Ear Nose Throat J.  2004 Feb;83(2):92-3.

FIRST PARAGRAPH: Nasal glial heterotopia (nasal glioma) is the term used to describe a mass made up of mature brain tissue that is isolated from the cranial cavity or spinal canal. Most of these rare, benign, congenital tumors are found in the nasal region, particularly at the bridge of the nose and in the nasal cavity. Nasal glial heterotopia is frequently diagnosed in newborns; a few cases have been found in adults.

PMID: 15008441

See full article (<1 MB .pdf)

Sinonasal tract glomangiopericytoma (hemangiopericytoma).

Thompson LD.

Ear Nose Throat J. 2004 Dec;83(12):807.

FIRST PARAGRAPH: A glomangiopericytoma (sinonasal-type hemangio-pericytoma) is a tumor believed to derive from perivascular modified smooth-muscle cells. Its origin is similar to that of a glomus tumor (not to be confused with glomus jugulare, which is a different neoplasm) but distinctly different from soft-tissue hemangiopericytoma. There is a very slight female preponderance, and the tumor’s peak incidence occurs during the seventh decade of life. Most affected patients experience nasal obstruction and epistaxis along with a wide array of other nonspecific findings that are generally present for less than 1 year. Glomangiopericytomas have a predilection for the nasal cavity and paranasal sinuses, where they grow as polypoid masses. Their average size is approximately 3 cm, and they are often mistaken clinically for inflammatory polyps.

PMID: 15724732

See full article (<1 MB .pdf)

College of American Pathologists (CAP)

Protocol for the Examination of Specimens from Patients with
Carcinomas of the of the Nasal Cavity and Paranasal Sinuses

Protocol applies to all invasive carcinomas of the nasal cavity and paranasal sinuses. Mucosal malignant melanoma is included. Lymphomas, neuroectodermal neoplasms, and sarcomas are not included.

Based on AJCC/UICC TNM, 7th edition
Protocol web posting date: October 2009
Protocol effective date: January 2010

See full article (<1 MB .pdf)