Extranodal NK/T-cell lymphoma, nasal type (ENKT), is a distinct clinicopathologic disease, common among East Asia and Latin America population. Clinically, it frequently occurs in middle-aged men. It predominantly occurs in the nasal or paranasal areas and less frequently in the skin. The main clinical features are nasal congestion, sore throat, dysphagia and epistaxis, due to a destructive mass involving the midline facial tissues. The pathogenesis of ENKT remains uncertain. It is thought that Epstein-Barr virus (EBV) may play a role in the development of this entity. Pathologically, lymphoma cells exhibit angioinvasion, angiodestruction and coagulative necrosis. It has unique characteristics including the expression of cytoplasmic CD3, CD56 and cytotoxic molecules such as TIA-1, and is positive for EBV in situ hybridization. The overall prognosis of this disease is poor because of frequent relapse or resistance to treatment. Although several studies have explored the treatment of ENKL in recent years, the optimal therapy has still not been found. Due to the highly aggressive features of tumors, every endeavor has been made to investigate factors associated with poor outcome. In this review, the recent advances on cause and pathogenesis, clinical manifestations and staging, pathologic characteristics, diagnosis and differential diagnosis, therapy and prognosis of ENKL are summarized.
Diagnosis, Differential ; Humans ; Lymphoma, T-Cell ; diagnosis ; etiology ; pathology ; therapy ; Neoplasm Staging ; Nose Neoplasms ; diagnosis ; etiology ; pathology ; therapy

OBJECTIVETo discuss the clinical characteristics, diagnosis and treatment of esthesioneuroblastoma (ENB).

METHODSThe clinical data of 7 patients with ENB were analyzed retrospectively, and the clinical characteristics, diagnosis, surgical approaches and prognosis of the disease were discussed.

RESULTSThe 7 patients received surgical treatment combined with radiotherapy and chemotherapy. Tumor relapse occurred within one year in two cases, which were treated with a second operation combined with radiotherapy, and one patient died and one survived with tumor after a one-year follow-up.

CONCLUSIONEarly diagnosis of ENB can be difficult. Transnasal-frontal approach with nasal endoscope is effective approach to ENB treatment, and the patients may benefit from postoperative radiotherapy and chemotherapy to improve the prognosis.

Adult ; Esthesioneuroblastoma, Olfactory ; diagnosis ; therapy ; Female ; Humans ; Male ; Middle Aged ; Nasal Cavity ; Nose Neoplasms ; diagnosis ; therapy ; Paranasal Sinus Neoplasms ; diagnosis ; therapy ; Retrospective Studies ; Young Adult

OBJECTIVETo discuss the diagnosis and treatment of the diseases originated in the olfactory cleft.

METHODSEight consecutive patients with the diagnosis of olfactory cleft diseases, encountered between December, 2003 and May, 2006, were included in this retrospective study. On the basis of case reports, the related anatomy, diagnosis and treatment of olfactory cleft diseases were discussed.

RESULTSFor the 8 patients with olfactory cleft diseases, the clinical and pathological diagnosis were as follows: adenocarcinoma, squamous cell carcinoma, inverted papilloma, glioma with cerebrospinal fluid (CSF) rhinorrhea, pyocyst of the superior turbinate, angiofibrosteoma, neurofibroma and hemangioma. All patients were treated by endoscopic surgery. Postoperative radiotherapy was given to two patients with malignant tumour.

CONCLUSIONSThe diseases in the olfactory cleft are not uncommon, which are often overlooked. In order to avoid misdiagnosis, (1) it is important to pay attention to this specific anatomic area; (2) the particularity of olfactory cleft diseases should be emphasized, especially in the cases of huge tumors. In that cases, the primary sites of olfactory cleft tumors can not be correctly judged preoperatively by CT scans, which can only be found during endoscopic surgery. For the treatment of olfactory cleft diseases, surgery under endoscope is the treatment of choice. If the tumor is malignant, postoperative radiotherapy should be added.

Adenocarcinoma ; diagnosis ; therapy ; Adolescent ; Adult ; Aged ; Female ; Humans ; Male ; Middle Aged ; Nasal Cavity ; pathology ; Neurofibroma ; diagnosis ; therapy ; Nose Neoplasms ; diagnosis ; therapy ; Retrospective Studies ; Smell ; Young Adult

OBJECTIVES: To investigate the clinical and pathological features of patients with unilateral sinonasal disease (USD). METHODS: A retrospective analysis was completed on 376 adult patients with USD from January 2015 to December 2016. Their presenting symptoms, nasal endoscope, CT scanning, and pathology were analyzed respectively. RESULTS: Among the 267 (71.01%) patients with inflammatory disease, there were 4 pathological types. And there were 8 pathological types in 60 (15.96%) patients with benign tumor. Of the 49 patients with malignant tumor, there were 15 pathological types which included squamous carcinoma, malignant melanoma, and lymphoma, as well as myoepithelial carcinoma and Mesodermal mesoderm. The onset age of inflammation group was younger than that of benign (<0.05) or malignant tumor groups (<0.05). The misdiagnosis rate was 8.33% in benign tumor (5/60), and 10.20% in malignant tumor (5/49). Nasal polyps was the most common misdiagnosis in the groups of benign and malignant tumor. CONCLUSIONS The pathology of adult patients with USD is complicated, and no specific clinical feature was found for distinguishing between benign and malignant lesions. The tumor took a quite proportion in adult patients with USD. Therefore, careful consideration should be taken before diagnosing patients with USD in order to reduce misdiagnosis rate.
Adult ; Carcinoma, Squamous Cell ; diagnosis ; pathology ; therapy ; Humans ; Melanoma ; diagnosis ; pathology ; therapy ; Nasal Cavity ; Nasal Polyps ; Nose Neoplasms ; diagnosis ; pathology ; therapy ; Retrospective Studies

OBJECTIVE: To evaluate the treatment and prognosis of sinonasal mucosal melanoma (SMM). METHOD: Clinicopathological data of SMM patients from January 1976 to December 2005 were analyzed retrospectively. Survival analysis was performed and Kaplan-Meier analysis was used to compare the effect of clinicopathological factors on survival using SPSS 18.0 software. A Cox model was applied for multivariate analysis. RESULT: The 3-year and 5-year overall survival (OS) rates of 68 cases of SMM were 36.1% and 29.4%, respectively. The 3-year and 5-year OS of patients who underwent surgery or biotherapy were significantly higher than that of patients who underwent other therapeutic regimens without surgery or without biotherapy, respectively. Multivariate analysis showed the patients with distant metastasis at first present or residual/recurrence had a worse prognosis than that without distant metastasis or residual/recurrence, respectively. Surgery and biotherapy were effective treatments for SMM. CONCLUSION SMM has a poor prognosis, especially in the patients with distant metastasis or residual/recurrence. Surgery or biotherapy may improve the prognosis of patients with SMM.
Adolescent ; Adult ; Aged ; Child ; Child, Preschool ; Female ; Humans ; Male ; Melanoma ; diagnosis ; therapy ; Middle Aged ; Nasal Mucosa ; Nose Neoplasms ; diagnosis ; therapy ; Prognosis ; Retrospective Studies ; Treatment Outcome ; Young Adult

OBJECTIVE: To explore the clinical feature, the reason of misdiagnosis and mistreatment, influential factor of prognosis in patients with extranodal NK/T cell lymphoma-nasal type. METHOD: A retrospective study was made on the clinical data of 34 patients with extranodal NK/T cell lymphoma-nasal type. Among them, 10 cases were staged I(E) intra-cavity, 15 cases were I(E) ex-cavity, 6 cases were II(E) and 3 cases were IV(E). Among them, 29 cases were in nasal cavity, 5 cases were outside nasal cavity; 14 cases were treated with single chemotherapy or radiation therapy, 20 cases were treated with radiation therapy add chemotherapy. RESULT: The total rate of misdiagnosis and mistreatment were 58.8% (20/34), 52.3% (18/34), respectively. The 5-year survival rate of the I(E) intra-cavity group were 60.0% (6/10), and those of I(E) ex-cavity group were 26.7% (4/15), and those of II(E) group and IV(E) group were 16.7% (1/6), 0% (0/3), respectively There was significant difference between 3 groups by statistical analysis (P < 0.01). The 5-year survival rate of I(E) ex-cavity group treated with single therapy were 0% (0/6), and those of I(E)-cavity group treated with combined therapy were 50% (1/2). CONCLUSION The early clinical manifestation of extranodal NK/T tell lymphoma-nasal type is atypical and which is hard to diagnose and treat. Diagnosis depends on pathologic biopsy and immunohistochemistry, there are many factors that influence the prognosis of this disease, in which the clinical stage is a major factor. It is crucial for diagnosing and treating early.
Adolescent ; Adult ; Aged ; Diagnostic Errors ; Female ; Humans ; Lymphoma, Extranodal NK-T-Cell ; diagnosis ; therapy ; Male ; Middle Aged ; Nose Neoplasms ; diagnosis ; therapy ; Retrospective Studies ; Survival Rate ; Young Adult

OBJECTIVE: To better understand the embryonal rhabdomyosarcoma of nose and make correct decision for diagnosis and treatment. METHOD: Thirteen patients with rhabdomyosarcoma in the nose were studied retrospectively and the related literature was reviewed. RESULT: Male predilection for the disease was present. One patient was in stage I, four patients were in stage II, seven patients were in stage III and one patients was in stage IV. Nine patients were operated plus chemotherapy and radiotherapy, one was operated together with radiotherapy, one was treated by radiotherapy and chemotherapy and the other two were treated by operation alone. The survival rate of two years was 69.2% (9/13), survival rate of 3 years was 30.8% (5/13). CONCLUSION The pathological growth form of embryonal rhabdomyosarcoma in nose is often convert. If the diagnosis are under suspicion, pathological or immunohistochemical examination should be taken in time. Early diagnosis and combined therapy will make contribution to better prognosis. Surgery combined with radiotherapy and chemotherapy can improve the survival rate.
Adolescent ; Adult ; Child ; Female ; Humans ; Male ; Neoplasm Staging ; Nose Neoplasms ; diagnosis ; therapy ; Prognosis ; Retrospective Studies ; Rhabdomyosarcoma, Embryonal ; diagnosis ; therapy ; Young Adult

Herein, we report a case of nasal natural killer T-cell lymphoma (NKTL) with intraocular involvement. A 57-year-old woman was referred due to a three-day history of photophobia and diplopia in the left eye. One-month previously, she was diagnosed with nasal NKTL of the right nasal cavity. Ophthalmic examination revealed conjunctival injection and ptosis. The left pupil was fully dilated and non-reactive to light. Ocular motion was restricted on left-upper gaze. Five days later, anterior uveitis developed and persisted despite topical steroid treatment. An orbital magnetic resonance imaging was without specific findings, however, ophthalmoplegia, vitreous opacity, and an iris mass were observed. A diagnostic anterior chamber aspiration was performed. Aqueous humor aspiration revealed 35% morphologically atypical lymphocytes. After an intravitreal triamcinolone injection, radiotherapy and chemotherapy were administered; this resolved the uveitis and iris mass. When refractory uveitis or orbital pseudotumor occurs in patients with nasal NKTL, ocular and orbital involvement of the NKTL should be considered.
Diagnosis, Differential ; Eye Neoplasms/diagnosis/*secondary/therapy ; Fatal Outcome ; Female ; Humans ; Lymphoma, T-Cell/*pathology ; Magnetic Resonance Imaging ; Middle Aged ; Natural Killer T-Cells/*pathology ; Nose Neoplasms/*pathology

OBJECTIVETo review the diagnosis, treatment and prognosis of olfactory neuroblastoma.

METHODSClinical data were retrospectively reviewed in 10 cases of olfactory neuroblastoma admitted from 1998 to 2002, including data of transmission electron microscopic (TEM) observation in 4 cases. According to Kadish's classification, 2 cases were in stage A, 4 in stage B and 4 in stage C. Three patients were treated with surgery alone, 7 with combined surgery and radiation.

RESULTAmong 10 cases, the overall 5-year survival rate was 60 %(6/10); 3 patients died from local recurrence, 1 lost follow-up. TEM demonstrated granules in the cytoplasm of 3 patients.

CONCLUSIONThe combined surgery and radiation can achieve excellent local control. Transmission electron microscope is important for its diagnosis.

Adult ; Aged ; Combined Modality Therapy ; Esthesioneuroblastoma, Olfactory ; diagnosis ; pathology ; radiotherapy ; surgery ; Female ; Humans ; Male ; Middle Aged ; Nasal Cavity ; Nose Neoplasms ; diagnosis ; pathology ; radiotherapy ; surgery ; Retrospective Studies

Metastatic renal cell carcinoma (RCC) in the nose and paranasal sinuses is very rare. We report an unusual case of metastatic RCC that presented as recurrent epistaxis ten years after curative nephrectomy. The purpose of this report is to draw the attention of clinicians to the possibility of metastatic RCC in patients with recurrent epistaxis and nasal mass. We also discuss treatment options and review the relevant literature.
Adult ; Carcinoma, Renal Cell ; diagnosis ; secondary ; therapy ; Chemoradiotherapy ; Diagnosis, Differential ; Epistaxis ; diagnosis ; therapy ; Humans ; Indoles ; therapeutic use ; Kidney Diseases ; diagnosis ; therapy ; Male ; Neoplasm Metastasis ; Nose ; pathology ; Nose Neoplasms ; diagnosis ; secondary ; therapy ; Positron-Emission Tomography ; Pyrroles ; therapeutic use ; Recurrence ; Tomography, X-Ray Computed