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Pathology Quiz Case 1: Diagnosis

Pathology Quiz Case 1: Diagnosis Diagnosis: Primary sinonasal inverted papilloma (SIP) of the ethmoid and sphenoid sinuses, with intracranial extension Sinonasal inverted papilloma is an uncommon benign tumor that arises from the schneiderian membrane, which consists of ciliated columnar epithelium of ectodermal origin in the sinonasal mucosa. An incidence of 0.2 to 0.7 cases per 100 000 population per year has been reported, representing 0.5% to 4% of all nasal tumors.1 Typically, SIPs are more prevalent among males and occur more frequently in the fifth and sixth decades of life. Approximately 80% of SIPs originate from the lateral wall of the nasal cavity, near the middle turbinate.1 There are 3 characteristic attributes of a SIP: (1) its tendency to recur; (2) its destructive or bone remodeling capacity; and (3) its propensity to be associated with malignant neoplasms, especially squamous cell carcinoma (SCC). According to a review of the literature, there may be a viral component in the pathogenesis of SIPs, as demonstrated by the detection of human papillomavirus DNA within SIPs by in situ hybridization.2 However, the virus inclusions have never been equivocally demonstrated by either light or electron microscopy.3 Human papillomavirus as an etiologic agent of SIP is still controversial. Other factors, such as bacterial infections, inflammation, allergies, tobacco, and occupational exposures, have been cited as possible causes, but the exact mechanisms of growth and malignant transformation of SIPs are still unknown.1 Intracranial extension by SIPs is extremely rare and is often associated with recurrent disease that has degenerated into malignancy. The most common sites of intracranial extension are the cribriform plate, fovea ethmoidalis, and orbit. Inadequate resection of SIPs adjacent to the anterior cranial fossa may result in recurrent disease with the potential for intracranial involvement.4 In 1999, Vural et al4 reviewed 21 cases of intracranial extension by SIPs. Nine of the patients involved had coexisting SCC, and 10 patients had recurrent SIPs. Only 2 cases were primary SIPs with intracranial extension, with the anterior and middle cranial fossa involved in one case and the orbit and optic nerve involved in the other.4 The clinical appearance of an SIP is usually described as a firm, nontranslucent, polypoid mass occupying the lateral portion of the nasal cavity. The symptoms and signs of SIPs are nonspecific and depend on the involved anatomical site. The common complaints are unilateral nasal obstruction, hyposmia, frontal headache, epistaxis, and rhinorrhea caused by mass effect. However, SIPs with intracranial extension may be asymptomatic on neurologic examination; therefore, imaging studies play a critical role in the evaluation.5 Radiographically, both magnetic resonance imaging and computed tomography give precise anatomical detail regarding tumor location, extension, and origin in the workup of an SIP. Magnetic resonance imaging is helpful in defining the extent of the lesion, in differentiating SIPs from retained or entrapped secretions, and in assessing intracranial or intraorbital tumor extension. An SIP may reveal a “convoluted cerebriform pattern” on T2-weighted or enhanced T1-weighted magnetic resonance images.1 The advantage of computed tomography lies in the evaluation of bony structure. Bony erosion and sclerosis are often found in SIPs; however, pressure erosion of bone may not be easily distinguished from osseous invasion associated with malignant transformation. Extensive bone destruction should always raise the possibility of SCC arising in SIPs.6 Histologically, SIPs are composed of epithelium growing endophytically into the stroma, with an intact basement that separates the epithelial proliferation from the underlying connective tissue (Figure 3). The epithelium is multilayered, usually 5 to 30 cells thick, and formed of squamous or ciliated, columnar cells. Cellular pleomorphism may be present but is focal and not associated with dyskeratosis or increased mitotic activity. Mitoses are not numerous and, if present at all, are seen primarily in the basal and parabasal epithelium. The other characteristic findings are infiltration by numerous inflammatory cells, especially neutrophils, in the stroma and the presence of intraepithelial microcysts containing macrophages, mucin, and cell debris (Figure 4). However, SIPs may be associated with varying degrees of atypia, dysplasia, and carcinoma in situ as well as SCC. View LargeDownload Figure 3. View LargeDownload Figure 4. The most important differential diagnosis is invasive SCC. An SCC may present as a synchronous or metachronous malignant neoplasm. Invasive SCC can be distinguished from ordinary SIPs by the presence of the following features: cellular pleomorphism, atypical mitoses, keratin pearls, and unequivocal invasion associated with an inflammatory desmoplastic stromal response.3 Immunohistochemical staining for the cell adhesion molecule CD44 may help distinguish between SIPs and invasive SCC. In the study by Ingle et al,7 SIPs expressed CD44, with strong membranous staining in 83% of cases and moderate or weak staining in the remaining 17%. In contrast, CD44 expression was entirely absent or only focally present in invasive SCC. The other differential diagnosis based on histopathologic features includes nasal polyps with squamous metaplasia, respiratory epithelial adenomatoid hamartoma, and inverted ductal papilloma (IDP) of minor salivary gland origin.3 In nasal polyps with squamous metaplasia, thickening and hyalinization of the basement membrane, prominent minor salivary glands, and a large number of inflammatory cells, especially eosinophils, are usually found. These features are absent in SIPs. Also, the epithelial lining of the minor salivary glands in nasal polyps is not multilayered. Respiratory epithelial adenomatoid hamartoma occurs primarily on the nasal septum and shows excess glands lined by respiratory epithelium with many interspersed mucous (goblet) cells, which are less frequently seen in SIPs. In contrast to SIPs arising from the surface epithelium and growing endophytically, IDPs arise from the excretory ducts of minor salivary glands. Therefore, IDPs will grow intraluminally and be confined by the duct.3 The criterion standard treatment of SIPs is complete surgical excision, which reduces the risk of recurrence and enables thorough histologic examination to detect any coexisting malignant transformation. The preferred conventional open surgical approaches are lateral rhinotomy and medial maxillectomy with meticulous removal of all mucosa in the involved paranasal sinuses.4 Recently, endoscopic resection of SIPs has become increasingly popular and offers several advantages, such as adequate illumination, lack of a facial incision, reduced scarring, and decreased facial swelling, nasal crusting, and bleeding. A meta-analysis of 32 studies comparing endoscopic and contemporary nonendoscopic techniques for SIP excision showed the recurrence rates of endoscopic and nonendoscopic procedures to be 15% and 20%, respectively.8 In 2000, Krouse9 reported a new staging system of SIPs for outcome comparisons among different treatment approaches. In cases of SIPs with intracranial extension or association with malignant transformation, the tumors are classified as Krouse stage T4. Craniofacial resection may be required for these lesions. Radiotherapy is usually reserved for cases that are associated with malignant transformation, incompletely excised lesions, locally aggressive tumors, and multiple recurrences.1 With regard to the prognosis of SIPs, recurrences typically appear within 2 to 3 years after surgery; however, in some instances, they are delayed for years. Malignant transformation may occur in 5% to 15% of cases and predominantly as SCC. The mean interval between the onset of SIP and the development of carcinoma has been shown to be 63 months (range, 6 months to 13 years).10 For SIPs with intracranial extension, the most important poor prognostic indicator is dural invasion.4,5 In our patient, the histologic examination did not demonstrate malignant degeneration after complete surgical removal via endoscopic assistance. No further postoperative treatment was arranged. He is currently healthy and has no evidence of recurrent disease 1 year after his initial treatment. In conclusion, SIPs are histologically benign lesions that may demonstrate aggressive behavior. An SIP with intracranial extension is a challenge in clinical management because of the possibility of malignant transformation. Complete surgical removal is necessary to avoid recurrence and to enable thorough histologic examination. Radiotherapy is usually reserved for cases associated with malignancy, incompletely excised lesions, and multiple recurrences. Patients with SIPs with intracranial extension require close endoscopic and radiographic follow-up because their lesions may recur and malignantly transform decades after the initial treatment. Return to Quiz Case. References 1. Anari S, Carrie S. Sinonasal inverted papilloma: narrative review. J Laryngol Otol. 2010;124(7):705-71520388243PubMedGoogle ScholarCrossref 2. Weber RS, Shillitoe EJ, Robbins KT, et al. Prevalence of human papillomavirus in inverted nasal papillomas. Arch Otolaryngol Head Neck Surg. 1988;114(1):23-262825730PubMedGoogle ScholarCrossref 3. Barnes L. Schneiderian papillomas and nonsalivary glandular neoplasms of the head and neck. Mod Pathol. 2002;15(3):279-29711904343PubMedGoogle ScholarCrossref 4. Vural E, Suen JY, Hanna E. Intracranial extension of inverted papilloma: an unusual and potentially fatal complication. Head Neck. 1999;21(8):703-70610562682PubMedGoogle ScholarCrossref 5. Visvanathan V, Wallace H, Chumas P, Makura ZG. An unusual presentation of inverted papilloma: case report and literature review [published online August 3, 2009]. J Laryngol Otol. 2010;124(1):101-10419646294PubMedGoogle ScholarCrossref 6. Lund VJ. Optimum management of inverted papilloma. J Laryngol Otol. 2000;114(3):194-19710829107PubMedGoogle ScholarCrossref 7. Ingle R, Jennings TA, Goodman ML, Pilch BZ, Bergman S, Ross JS. CD44 expression in sinonasal inverted papillomas and associated squamous cell carcinoma. Am J Clin Pathol. 1998;109(3):309-3149495203PubMedGoogle Scholar 8. Busquets JM, Hwang PH. Endoscopic resection of sinonasal inverted papilloma: a meta-analysis. Otolaryngol Head Neck Surg. 2006;134(3):476-48216500448PubMedGoogle ScholarCrossref 9. Krouse JH. Development of a staging system for inverted papilloma. Laryngoscope. 2000;110(6):965-96810852514PubMedGoogle ScholarCrossref 10. Lesperance MM, Esclamado RM. Squamous cell carcinoma arising in inverted papilloma. Laryngoscope. 1995;105(2):178-1838544600PubMedGoogle ScholarCrossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Otolaryngology - Head & Neck Surgery American Medical Association

Pathology Quiz Case 1: Diagnosis

Archives of Otolaryngology - Head & Neck Surgery , Volume 138 (6) – Jun 1, 2012
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Publisher
American Medical Association
Copyright
Copyright © 2012 American Medical Association. All Rights Reserved.
ISSN
0886-4470
eISSN
1538-361X
DOI
10.1001/archoto.2012.497b
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Abstract

Diagnosis: Primary sinonasal inverted papilloma (SIP) of the ethmoid and sphenoid sinuses, with intracranial extension Sinonasal inverted papilloma is an uncommon benign tumor that arises from the schneiderian membrane, which consists of ciliated columnar epithelium of ectodermal origin in the sinonasal mucosa. An incidence of 0.2 to 0.7 cases per 100 000 population per year has been reported, representing 0.5% to 4% of all nasal tumors.1 Typically, SIPs are more prevalent among males and occur more frequently in the fifth and sixth decades of life. Approximately 80% of SIPs originate from the lateral wall of the nasal cavity, near the middle turbinate.1 There are 3 characteristic attributes of a SIP: (1) its tendency to recur; (2) its destructive or bone remodeling capacity; and (3) its propensity to be associated with malignant neoplasms, especially squamous cell carcinoma (SCC). According to a review of the literature, there may be a viral component in the pathogenesis of SIPs, as demonstrated by the detection of human papillomavirus DNA within SIPs by in situ hybridization.2 However, the virus inclusions have never been equivocally demonstrated by either light or electron microscopy.3 Human papillomavirus as an etiologic agent of SIP is still controversial. Other factors, such as bacterial infections, inflammation, allergies, tobacco, and occupational exposures, have been cited as possible causes, but the exact mechanisms of growth and malignant transformation of SIPs are still unknown.1 Intracranial extension by SIPs is extremely rare and is often associated with recurrent disease that has degenerated into malignancy. The most common sites of intracranial extension are the cribriform plate, fovea ethmoidalis, and orbit. Inadequate resection of SIPs adjacent to the anterior cranial fossa may result in recurrent disease with the potential for intracranial involvement.4 In 1999, Vural et al4 reviewed 21 cases of intracranial extension by SIPs. Nine of the patients involved had coexisting SCC, and 10 patients had recurrent SIPs. Only 2 cases were primary SIPs with intracranial extension, with the anterior and middle cranial fossa involved in one case and the orbit and optic nerve involved in the other.4 The clinical appearance of an SIP is usually described as a firm, nontranslucent, polypoid mass occupying the lateral portion of the nasal cavity. The symptoms and signs of SIPs are nonspecific and depend on the involved anatomical site. The common complaints are unilateral nasal obstruction, hyposmia, frontal headache, epistaxis, and rhinorrhea caused by mass effect. However, SIPs with intracranial extension may be asymptomatic on neurologic examination; therefore, imaging studies play a critical role in the evaluation.5 Radiographically, both magnetic resonance imaging and computed tomography give precise anatomical detail regarding tumor location, extension, and origin in the workup of an SIP. Magnetic resonance imaging is helpful in defining the extent of the lesion, in differentiating SIPs from retained or entrapped secretions, and in assessing intracranial or intraorbital tumor extension. An SIP may reveal a “convoluted cerebriform pattern” on T2-weighted or enhanced T1-weighted magnetic resonance images.1 The advantage of computed tomography lies in the evaluation of bony structure. Bony erosion and sclerosis are often found in SIPs; however, pressure erosion of bone may not be easily distinguished from osseous invasion associated with malignant transformation. Extensive bone destruction should always raise the possibility of SCC arising in SIPs.6 Histologically, SIPs are composed of epithelium growing endophytically into the stroma, with an intact basement that separates the epithelial proliferation from the underlying connective tissue (Figure 3). The epithelium is multilayered, usually 5 to 30 cells thick, and formed of squamous or ciliated, columnar cells. Cellular pleomorphism may be present but is focal and not associated with dyskeratosis or increased mitotic activity. Mitoses are not numerous and, if present at all, are seen primarily in the basal and parabasal epithelium. The other characteristic findings are infiltration by numerous inflammatory cells, especially neutrophils, in the stroma and the presence of intraepithelial microcysts containing macrophages, mucin, and cell debris (Figure 4). However, SIPs may be associated with varying degrees of atypia, dysplasia, and carcinoma in situ as well as SCC. View LargeDownload Figure 3. View LargeDownload Figure 4. The most important differential diagnosis is invasive SCC. An SCC may present as a synchronous or metachronous malignant neoplasm. Invasive SCC can be distinguished from ordinary SIPs by the presence of the following features: cellular pleomorphism, atypical mitoses, keratin pearls, and unequivocal invasion associated with an inflammatory desmoplastic stromal response.3 Immunohistochemical staining for the cell adhesion molecule CD44 may help distinguish between SIPs and invasive SCC. In the study by Ingle et al,7 SIPs expressed CD44, with strong membranous staining in 83% of cases and moderate or weak staining in the remaining 17%. In contrast, CD44 expression was entirely absent or only focally present in invasive SCC. The other differential diagnosis based on histopathologic features includes nasal polyps with squamous metaplasia, respiratory epithelial adenomatoid hamartoma, and inverted ductal papilloma (IDP) of minor salivary gland origin.3 In nasal polyps with squamous metaplasia, thickening and hyalinization of the basement membrane, prominent minor salivary glands, and a large number of inflammatory cells, especially eosinophils, are usually found. These features are absent in SIPs. Also, the epithelial lining of the minor salivary glands in nasal polyps is not multilayered. Respiratory epithelial adenomatoid hamartoma occurs primarily on the nasal septum and shows excess glands lined by respiratory epithelium with many interspersed mucous (goblet) cells, which are less frequently seen in SIPs. In contrast to SIPs arising from the surface epithelium and growing endophytically, IDPs arise from the excretory ducts of minor salivary glands. Therefore, IDPs will grow intraluminally and be confined by the duct.3 The criterion standard treatment of SIPs is complete surgical excision, which reduces the risk of recurrence and enables thorough histologic examination to detect any coexisting malignant transformation. The preferred conventional open surgical approaches are lateral rhinotomy and medial maxillectomy with meticulous removal of all mucosa in the involved paranasal sinuses.4 Recently, endoscopic resection of SIPs has become increasingly popular and offers several advantages, such as adequate illumination, lack of a facial incision, reduced scarring, and decreased facial swelling, nasal crusting, and bleeding. A meta-analysis of 32 studies comparing endoscopic and contemporary nonendoscopic techniques for SIP excision showed the recurrence rates of endoscopic and nonendoscopic procedures to be 15% and 20%, respectively.8 In 2000, Krouse9 reported a new staging system of SIPs for outcome comparisons among different treatment approaches. In cases of SIPs with intracranial extension or association with malignant transformation, the tumors are classified as Krouse stage T4. Craniofacial resection may be required for these lesions. Radiotherapy is usually reserved for cases that are associated with malignant transformation, incompletely excised lesions, locally aggressive tumors, and multiple recurrences.1 With regard to the prognosis of SIPs, recurrences typically appear within 2 to 3 years after surgery; however, in some instances, they are delayed for years. Malignant transformation may occur in 5% to 15% of cases and predominantly as SCC. The mean interval between the onset of SIP and the development of carcinoma has been shown to be 63 months (range, 6 months to 13 years).10 For SIPs with intracranial extension, the most important poor prognostic indicator is dural invasion.4,5 In our patient, the histologic examination did not demonstrate malignant degeneration after complete surgical removal via endoscopic assistance. No further postoperative treatment was arranged. He is currently healthy and has no evidence of recurrent disease 1 year after his initial treatment. In conclusion, SIPs are histologically benign lesions that may demonstrate aggressive behavior. An SIP with intracranial extension is a challenge in clinical management because of the possibility of malignant transformation. Complete surgical removal is necessary to avoid recurrence and to enable thorough histologic examination. Radiotherapy is usually reserved for cases associated with malignancy, incompletely excised lesions, and multiple recurrences. Patients with SIPs with intracranial extension require close endoscopic and radiographic follow-up because their lesions may recur and malignantly transform decades after the initial treatment. Return to Quiz Case. References 1. Anari S, Carrie S. Sinonasal inverted papilloma: narrative review. J Laryngol Otol. 2010;124(7):705-71520388243PubMedGoogle ScholarCrossref 2. Weber RS, Shillitoe EJ, Robbins KT, et al. Prevalence of human papillomavirus in inverted nasal papillomas. Arch Otolaryngol Head Neck Surg. 1988;114(1):23-262825730PubMedGoogle ScholarCrossref 3. Barnes L. Schneiderian papillomas and nonsalivary glandular neoplasms of the head and neck. Mod Pathol. 2002;15(3):279-29711904343PubMedGoogle ScholarCrossref 4. Vural E, Suen JY, Hanna E. Intracranial extension of inverted papilloma: an unusual and potentially fatal complication. Head Neck. 1999;21(8):703-70610562682PubMedGoogle ScholarCrossref 5. Visvanathan V, Wallace H, Chumas P, Makura ZG. An unusual presentation of inverted papilloma: case report and literature review [published online August 3, 2009]. J Laryngol Otol. 2010;124(1):101-10419646294PubMedGoogle ScholarCrossref 6. Lund VJ. Optimum management of inverted papilloma. J Laryngol Otol. 2000;114(3):194-19710829107PubMedGoogle ScholarCrossref 7. Ingle R, Jennings TA, Goodman ML, Pilch BZ, Bergman S, Ross JS. CD44 expression in sinonasal inverted papillomas and associated squamous cell carcinoma. Am J Clin Pathol. 1998;109(3):309-3149495203PubMedGoogle Scholar 8. Busquets JM, Hwang PH. Endoscopic resection of sinonasal inverted papilloma: a meta-analysis. Otolaryngol Head Neck Surg. 2006;134(3):476-48216500448PubMedGoogle ScholarCrossref 9. Krouse JH. Development of a staging system for inverted papilloma. Laryngoscope. 2000;110(6):965-96810852514PubMedGoogle ScholarCrossref 10. Lesperance MM, Esclamado RM. Squamous cell carcinoma arising in inverted papilloma. Laryngoscope. 1995;105(2):178-1838544600PubMedGoogle ScholarCrossref

Journal

Archives of Otolaryngology - Head & Neck Surgery – American Medical Association

Published: Jun 1, 2012

Keywords: cancer,inverted papilloma,malignant transformation,ethmoid bone,sphenoid sinus,endoscopy,excision

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