Última actualización:
April 22, 2008
Años publicados: 2004
NORD gratefully acknowledges Andrew Martin, medical student, and Raja Mudad, MD, Associate Professor and Associate Section Chief of Medicine: Hematology-Oncology, Tulane Cancer Center; Director of Hematology-Oncology, Veterans Administration Medical Center, New Orleans, for assistance in the preparation of this report.
Sinonasal undifferentiated carcinoma (SNUC) is a rare cancer of the nasal cavity and/or paranasal sinuses. Initial symptoms range from bloody nose, runny nose, double vision, and bulging eye to chronic infections and nasal obstruction. It has been associated with several types of papilloma in the nasal cavity, which are benign, but can give rise to malignancy. Prior irradiation for other cancers has been associated with the development of SNUC in a number of cases, and has been associated with a genetic mutation known to be associated with cancer development. Most patients have not had prior irradiation, and no other causes have demonstrated to be significant, though some studies have found that woodworkers and nickel factory workers are generally more susceptible to sinonasal malignancy of all types.
Symptoms include bloody nose (epistaxis), runny nose (rhinorrhea), bulging eye (exopthalmos/proptosis), double vision (diplopia), nasal obstruction, and nasal infection.
The nasal cavity lies just behind the nostrils and continues backward to the nasopharynx, a space just behind the nasal cavity that is contiguous with the oropharynx (the space just behind the mouth and oral cavity). The paranasal sinuses are these: the maxillary sinus under the eyes, the ethmoid and sphenoid sinuses, which are above and behind the nasal cavity, and the frontal sinuses, which lie behind the space between the eyebrows. The nasal cavity and paranasal sinuses are lined with a thin layer of tissue called Schneiderian epithelium, which has cilia, which are tiny hairs whose movements aid in pushing dirt and other contaminants out of the nasal cavity.
Schneiderian epithelium gives rise to several types of papillomas, a type of benign tumor, several of which are known to give rise to cancers. The rare case of SNUC is known to arise in these papillomas. The precise localization of the Schneiderian epithelium to the nasal cavity and paranasal sinuses explains both the strict localization of this tumor, and the unique and troublesome combination of symptoms that make SNUC a difficult disease.
In the original scientific paper on SNUC, seven of eight patients were smokers, and the eighth had significant occupational exposure to known carcinogens in both coal mines and chrome plating factories (13). These patients came from a wide geographical distribution, which seemed to rule out environmental factors. Several later papers failed to confirm the data on smoking as a potential cause of SNUC. A large study from Wales, Canada and Norway has presented substantial evidence that nickel refinery workers are susceptible to all sinonasal cancers with incidences of individual pathologies proportional to those in the general population, as opposed to the established association of adenocarcinoma specifically with wood workers (59)(60). However, the majority of SNUC patients are not nickel workers, so other factors as yet undefined are at work in SNUC.
Retinoblastoma is a rare childhood tumor that arises in one of the layers of the eye. Treatment usually involves removal of the eye, and sometimes radiation therapy. There have been several cases of patients with retinoblastoma who have been treated with radiation and have gone on to develop SNUC many years later. In genetic studies done on several of these patients, mutations in a gene known as the retinoblastoma gene (on chromosome 13, and designated RB-1) have been found. This gene is known to stop the progression of healthy cells into cancer cells by controlling the cell division cycle, so a mutation increases the likelihood that cells will become cancerous. However, this, too, accounts for only a small number of SNUC patients.
Epstein Barr virus (EBV) is a human herpes virus with the known ability to cause cancer. In the nasopharynx, which is adjacent to the nasal cavity as discussed above, EBV is known to give rise to nasopharyngeal carcinoma (NPC), and in the sinonasal region it gives rise to PSNPC. It has been fairly well established that SNUC is not caused by EBV, but some controversy over this may still exist.
The bottom line is that the cause of SNUC is still undetermined.
There is no regional predilection for SNUC. It affects people in all countries more or less equally, based on available evidence. There is approximately a 2:1 male:female prevalence, and the average age from available published studies is 53 years old. The age range is 14 to 83 years old.
The diagnosis of SNUC requires two things: a history of a mass in the nasal cavity or the sinonasal region (see symptom section above), and tissue obtained via surgery or biopsy. Neither imaging studies nor laboratory studies are required, but sometimes SNUC does not manifest itself until imaging studies are performed for other sinus problems.
Treatment
The treatment of SNUC has no firmly established protocol. What has been established is that the disease tends to recur in the same area from which it arises, and that treatment must focus on eliminating the disease via all available treatment modalities. This means the ideal treatment is a coordinated effort by a team of medical oncologists, radiation oncologists, and surgeons who are specially trained to deal with cancers that occur in this very challenging anatomical region. Treatment, therefore, involves chemotherapy, radiation therapy and surgery in some combination. Follow-up treatment can involve dental prostheses, eye prostheses, and visits with dentists and ophthalmologists, in addition to regular follow-up with the original treating doctors for cancer follow-up. This follow-up consists of regular magnetic resonance imaging (MRI) and, if necessary, tissue biopsy.
Though research on cancer generally is ongoing, research on SNUC is virtually non-existent. The treatment paradigm for SNUC is drawn from successful treatment of other malignancies, and its rarity makes it unlikely ever to be studied in any great depth. In the near future, as the National Cancer Institute (NCI) builds an expanded database of information for all tumor types, there may be hope for as yet unconceived treatments.
Treatments that are highly specific to individual tumor types have resulted from just this sort of highly specific information about the types of receptors that appear on the surface of tumor cells. These receptors are used by the tumor cells to manipulate growth factors that are normally found within the body to their advantage. Three of these drugs are Herceptin, epidermal growth factor receptor blocker (EGFR blocker), and Gleevec. While these drugs were developed in the context of specific tumors (breast cancer and chronic myelogenous leukemia especially), recent discoveries have shown that other tumor types express these receptors, and thus may be susceptible to these drugs.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: [email protected]
For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com
JOURNAL ARTICLES
Lonardo F, Pass HI, Lucas DR: Immunohistochemistry Frequently Detects c-Kit Expression in Pulmonary Small Cell Carcinoma and May Help Select Clinical Subsets for a Novel Form of Chemotherapy. Applied Immunohistochemistry & Molecular Morphology 11(1): 51-55, 2003.
Haas I, Ganzer U: Does Sophisticated Diagnostic Workup on Neuroectodermal Tumors Have an Impact on the Treatment of Esthesioneuroblastoma? Onkologie 26: 261-267, 2003.
Rosenthal D, Barker JL, El-Naggar AK, Glisson BS, Kies MS, Diaz EM, Clayman GL, DeMonte F, Selek U, Morrison WM, Ang KK, Garden AS: Sinonasal Carcinoma with Neuroendocrine Differentiation: Patterns of failure according to histologic phenotype. International Journal of Radiation Oncology 57(2)suppl.: S248-S249, 2003.
Hsiao JR, Jin YT, Tsai ST, Shiau AL, Wu CL, Su WC: Constitutive Activation of STAT3 and Stat5 is Present with the Majority of Nasopharyngeal Carcinoma and Correlates with Better Prognosis. British Journal of Cancer 89: 344-349, 2003.
Jeng YM, Sung MT, Fang CL, Huang HY, Mao TL, Cheng W, Hsiao CH: Sinonasal Undifferentiated Carcinoma and Nasopharyngeal-Type Carcinoma. The American Journal of Surgical Pathology 26(3): 371-376, 2002.
Imola MJ, Schramm VL: Orbital Preservation in Surgical Management of Sinonasal Malignancy. Laryngoscope 112:1357-1365, 2002.
Cohen ZR, Marmor E, Fuller GN, DeMonte F: Misdiagnosis of Olfactory Neuroblastoma. Neurosurgical Focus 12(5): Article 3, 2002.
Barnes L: Schneiderian Papillomas and Nonsalivary Glandular Neoplasms of the Head and Neck. Modern Pathology 15(3): 279-297, 2002.
DeMonte F, Tabrizi P, Culpepper SA, Suki D, Soparkar CNS, Patrinely JR: Ophthalmalogic Outcome After Orbital Entry During Anterior and Anterolateral Skull Base Surgery. Journal of Neurosurgery 97: 851-856, 2002.
Franchi A, Moroni M, Massi D, Paglierani M, Santucci M: Sinonasal Undifferentiated Carcinoma, Nasopharyngeal-Type Undifferentiated Carcinoma, and Keratinizing and Nonkeratinizing Squamous Cell Carcinoma Express Different Cytokeratin Patterns. American Journal of Surgical Pathology 26(12):1597-1604, 2002.
Musy PY, Reibel JF, Levine PA: Sinonasal Undifferentiated Carcinoma: The Search for a Better Outcome. Laryngoscope 112: 1450-1455, 2002.
Masuda M, Suzui M, Yasumatu R, Nakashima T, Kuratomi Y, Azuma K, Tomita K, Komiyama S, Weinstein IB: Constitutive Activation of Signal Transducers and Activators of Transcription 3 Correlates with Cyclin D1 Overexpression and May Provide a Novel Prognostic Marker in Head and Neck Squamous Cell Carcinoma. Cancer Research 62: 3351-3355, 2002.
Mills SE: Neuroectodermal Neoplasms of the Head and Neck with Emphasis on Neuroendocrine Carcinomas. Modern Pathology 15(3): 264-278, 2002.
Maitra A, Baskin LB, Lee EL: Malignancies Arising in Schneiderian Papillomas: A report of 2 cases and review of the literature. Archives of Pathology and Laboratory Medicine 125: 1365-1367, 2001.
Cerilli LA, Holst VA, Brandwein MS, Stoler MH, Mills SE: Sinonasal Undifferentiated Carcinoma: Immunohistochemical profile and lack of EBV association. American Journal of Surgical Pathology 25(2): 156-163, 2001.
Sakamoto M, Nakamura K, Nishimura S: An Alternative Therapeutic Procedure for Sinonasal Undifferentiated Carcinoma. European Archives of Otolaryngology 258: 226-229, 2001.
Ghosh S, Weiss M, Streeter O, Sinha U, Commins D, Chen TC: Drop Metastasis from Sinonasal Undifferentiated Carcinoma. Spine 26(13): 1486-1491, 2001.
Cope JU, Tsokos M, Miller RW: Ewing Sarcoma and Sinonasal Neuroectodermal Tumors as Second Malignant Neoplasms after Retinoblastoma and Other Neoplasms. Medical and Pediatric Oncology 36: 290-294, 2001.
Diaz EM, Kies MS: Chemotherapy for Skull Base Cancers. Skull Base Tumor Surgery 34(6): 1079-1085, 2001.
Sharara N, Muller S, Olson J, Grist WJ, Grossniklaus HE: Sinonasal Undifferentiated Carcinoma With Orbital Invasion: Report of three cases. Ophthalmic Plastic and Reconstructive Surgery 17(4): 288-292, 2001.
Miyamoto RC, Gleich LL, Biddinger PW, Gluckman JL: Esthesioblastoma and Sinonasal Undifferentiated Carcinoma: Impact of histological grading and clinical staging on survival and prognosis. Laryngoscope 110: 1262-1265, 2000.
Shinokuma A, Hirakawa N, Tamiya S, Oda Y, Komiyama S, Tsuneyoshi M: Evaluation of Epstein-Barr Virus Infection in Sinonasal Small Round Cell Tumors. Journal of Cancer Research and Clinical Oncology 126:12-18, 2000.
Smith SR, Som P, Fahmy A, Lawson W, Sacks S, Brandwein M: A Clinicopathological Study of Sinonasal Neuroendocrine Carcinoma and Sinonasal Undifferentiated Carcinoma. Laryngoscope 110: 1617-1622, 2000.
Wick MR: Immunohistology of Neuroendocrine and Neuroectodermal Tumors. Seminars in Diagnostic Pathology 17(3): 194-203, 2000.
Gorelick J, Ross D, Marentette L, Blaivas M: Sinonasal Undifferentiated Carcinoma: A case series and review of the literature. Neurosurgery 47(3): 750-755, 2000.
Chu P, Wu E, Weiss LM: Cytokeratin 7 and Cytokeratin 20 Expression in Epithelial Neoplasms: A survey of 435 cases. Modern Pathology 13(9): 962-972, 2000.
Adelstein DJ: The Role of Chemotherapy for Skull Base Carcinomas and Sarcomas. Neurosurgery Clinics of North America 11(4): 681-691, 2000. (not cited)
Houston GD, Gillies E: Sinonasal Undifferentiated Carcinoma: A distinctive Clinicopathologic entity. Advances in Anatomic Pathology 6(6):317-323, 1999.
Tufano RP, Mokadam NA, Montone KT, Weinstein GS, Chalian AA, Wolf PF, Weber RS: Malignant Tumors of the Nose and Paranasal Sinuses: Hospital of the University of Pennsylvania experience 1990-1997. American Journal of Rhinology 13: 117-123, 1999.
Tune CE, Liavaag PG, Freeman JL, van den Brekel MWM, Shpitzer T, Kerrebijn JDF, Payne D, Irish JC, Ng R, Cheung RK, Dosch HM: Nasopharyngeal Brush Biopsies and Detection of Nasopharyngeal Cancer in a High-Risk Population. Journal of the National Cancer Institute 91(9): 796-800, 1999.
Lee MM, Vokes EE, Rosen A, Witt ME, Weichselbaum RR, Haraf DJ: Multimodality Therapy in Advanced Paranasal Sinus Carcinoma: Superior long-term results. Cancer Journal 5(4): 219, 5p, 1 chart, 5 graphs, 1999.
Svane-Knudsen V, Jorgensen KE, Hansen O, Lindgren A, Marker P: Cancer of the Nasal Cavity and Paranasal Sinuses: A series of 115 patients. Rhinology 36:12-14, 1998.
Kerrebijn JDF, Tietze L, Mock D, Freeman J: Sinonasal Undifferentiated Carcinoma. The Journal of Otolaryngology 27(1): 40-42, 1998.
Houston GD: Sinonasal Undifferentiated Carcinoma: Report of two cases and review of the literature. Oral Surgery Oral Medicine Oral Pathology 85(2): 185-188, 1998.
Ingle R, Jennings TA, Goodman ML, Pilch BZ, Bergman S, Ross JS: CD44 Expression in Sinonasal Inverted Papillomas and Associated Squamous Cell Carcinoma. Anatomic Pathology 109: 309-314, 1998.
Phillips CD, Futterer SF, Lipper MH, Levine PA: Sinonasal Undifferentiated Carcinoma: CT and MR imaging of an uncommon neoplasm of the nasal cavity. Radiology 202: 477-480, 1997.
Righi PD, Francis F, Aron BS, Weitzner S, Wilson KM, Gluckman J: Sinonasal Undifferentiated Carcinoma: A 10-year experience. American Journal of Otolaryngology 17(3): 167-171, 1996.
Devaney K, Wenig BM, Abbondanzo SL: Olfactory Neuroblastoma and Other Round Cell Lesions of the Sinonasal Region. Modern Pathology 9(6): 658-663, 1996.
McCary SW, Levine PA, Cantrell RW: Preservation of the Eye in the Treatment of Sinonasal Malignant Neoplasms With Orbital Involvement: A confirmation of the original treatise. Archives of Otolaryngology Head and Neck Surgery 122: 657-659, 1996.
Mills SE: Neuroendocrine Tumors of the Head and Neck: A Selected Review with Emphasis on Terminology. Endocrine Pathology 7(4): 329-343, 1996.
Spafford MF, Koeppe J, Pan Z, Archer PG, Meyers AD, Franklin WA: Correlation of Tumor Markers p53, bcl-2, CD34, CD44H, CD44v6 and Ki-67 with Survival and Metastasis in Laryngeal Squamous Cell Carcinoma. Archives of Otolaryngology Head and Neck Surgery 122: 627-632, 1996.
Xu XC, Lee JS, Lippman SM, Ro JY, Hong WK, Lotan R: Increased Expression of Cytokeratins CK8 and CK19 is Associated with Head and Neck Carcinogenesis. Cancer Epidemiology, Biomarkers & Prevention 4:871-876, 1995.
Miyaguchi M, Sakai S, Takashima H, Hosokawa H: Lymph Node and Distant Metastases in Patients with Sinonasal Carcinoma. The Journal of Laryngology and Otology 109: 304-307, 1995.
Pitman KT, Costantino PD, Lassen LF: Sinonasal Undifferentiated Carcinoma: Current trends in treatment. Skull Base Surgery 5(4): 269-272, 1995.
Pitman KT, Lassen LF: Pathologic Quiz Case 2. Archives of Otolaryngology Head and Neck Surgery 121:1201-1203, 1995.
McCary SW, Levine PA: Management of the Eye in the Treatment of Sinonasal Cancers. Otolaryngologic Clinics of North America 28(6): 1231-1238, 1995.
Leung SY, Yuen ST, Chung LP, Kwong WK, Wong MP, Chan SY: Epstein-Barr Virus is Present in a Wide Histological Spectrum of Sinonasal Carcinomas. The American Journal of Surgical Pathology 19(9): 994-1001, 1995.
Gallo, O, DiLollo S, Graziani P, Gallina E, Baroni G: Detection of Epstein-Barr virus Genome in Sinonasal Undifferentiated Carcinoma by Use of In Situ Hybridization. Otolaryngology-Head and Neck Surgery 112:659-664, 1995.
Nelson RS, Perlman EJ, Askin FB: Is Esthesioneuroblastoma a Peripheral Neuroectodermal Tumor? Human Pathology 26(6): 639-641, 1995.
Lopategui JR, Gaffey MJ, Frierson HF, Chan JKC, Mills SE, Chang KL, Chen YY, Weiss LM: Detection of Epstein-Barr Viral RNA in Sinonasal Undifferentiated Carcinoma from Western and Asian Patients. American Journal of Surgical Pathology 18(4): 391-398, 1994.
Ascaso FJ, Adiego MI, Garcia J, Royo J, Valles H, Palomar A, Ramon Y Cajal S: Sinonasal Undifferentiated Carcinoma Invading the Orbit. European Journal of Ophthalmology 4(4): 234-236, 1994.
Gallo O, Graziani P, Fini-Storchi O: Undifferentiated Carcinoma of the Nose and Paranasal Sinuses: An immunohistochemical and clinical study. ENT Journal 72(9): 588-595, 1993.
Kapadia SB, Barnes L, Pelzman K, Mirani N, Heffner DK, Bedetti C: Carcinoma Ex Oncocytic Schneiderian (Cylindrical Cell) Papilloma. American Journal of Otolaryngology 14(5):332-338, 1993.
Rosen A, Vokes EE, Scher N, Haraf D, Weichselbaum RR, Panje WR: Locoregionally Advanced Paranasal Sinus Carcinoma: Favorable survival with multimodality therapy. Archives of Otolaryngology Head and Neck Surgery 119: 743-746, 1993.
Deutsch BD, Levine PA, Stewart FM, Frierson HF, Cantrell RW: Sinonasal Undifferentiated Carcinoma: A ray of hope. Otolaryngology-Head and Neck Surgery 108: 697-700, 1993.
Kamel OW, Rouse RV, Warnke RA: Heterogeneity of Epithelial Marker Expression in Routinely Procesed, Poorly Differentiated Carcinomas. Archives of Pathology and Laboratory Medicine 115: 566-570, 1991.
Greger V, Schirmacher P, Bohl J, Bornemann A, Hürter T, Passarge E, Horsthemke B: Possible Involvement of the Retinoblastoma Gene in Undifferentiated Sinonasal Carcinoma. Cancer 66: 1954-1959, 1990.
Ward BE, Fechner RE, Mills SE: Carcinoma Arising in Oncocytic Schneiderian Papilloma. The American Journal of Surgical Pathology 14(4):364-369, 1990.
Schaafsma HE, Ramaekers FCS, van Muijen GNP, Lane EB, Leigh IM, Robben H, Huijsmans A, Ooms ECM, Ruiter DJ: Distribution of Cytokeratin Ploypeptides in Human Transitional Cell Carcinomas, with Special Emphasis on Changing Expression Patterns During Tumor Progression. American Journal of Pathology 136: 329-343, 1990.
Stewart MF, Lazarus HM, Levine PA, Stewart KA, Tabbara IA, Spaulding CA: High Dose Chemotherapy and Autologous Marrow Transplantation for Esthesioneuroblastoma ans Sinonasal Undifferentiated Carcinoma. American Journal of Clinical Oncology 12(3): 217-221, 1989.
Sunderman WF, Morgan LG, Andersen A, Ashley D, Forouhar FA: Histopathology of Sinonasal and Lung Cancers in Nickel Refinery Workers. Annals of Clinical and Laboratory Science 19(1)44-50, 1989.
Frierson HF, Ross GW, Stewart FM, Newman SA, Kelly MD: Unusual Small Cell Neoplasms Following Radiotherapy for Bilateral Retinoblastomas. The American Journal of Surgical Pathology 13(11): 947-954, 1989.
Mills SE, Fechner RE: “Undifferentiated” Neoplasms of the Sinonasal Region: Differential diagnosis based on clinical, light microscopic, immunohistochemical, and ultrastructural features. Seminars in Diagnostic Pathology 6(4): 316-328, 1989.
Levine PA, Frierson HF, Mills SE, Stewart FM, Fechner RE, Cantrell RW: Sinonasal Undifferentiated Carcinoma: A distinctive and highly aggressive neoplasm. Laryngoscope 97:905-908, 1987.
Frierson HF, Mills SE, Fechner RE, Taxy JB, Levine PA: Sinonasal Undifferentiated Carcinoma: An aggressive neoplasm derived from Schneiderian epithelium and distinct from Olfactory Neuroblastoma. The American Journal of Surgical Pathology 10(11):771-779, 1986.
NORD y la Fundación MedicAlert se han asociado en un nuevo programa para brindar protección a pacientes con enfermedades raras en situaciones de emergencia.
Aprende más https://rarediseases.org/patient-assistance-programs/medicalert-assistance-program/Asegurarse de que los pacientes y los cuidadores estén equipados con las herramientas que necesitan para vivir su mejor vida mientras manejan su condición rara es una parte vital de la misión de NORD.
Aprende más https://rarediseases.org/patient-assistance-programs/rare-disease-educational-support/Este programa de asistencia, primero en su tipo, está diseñado para los cuidadores de un niño o adulto diagnosticado con un trastorno raro.
Aprende más https://rarediseases.org/patient-assistance-programs/caregiver-respite/