Nasopharyngeal amyloidoma: report of three cases and review of the literature.

BACKGROUND: Nasopharyngeal amyloidoma is a rare, locally aggressive tumor that has been reported in the English literature in only 38 cases to date, most of which were in the form of case reports. The present study was aimed to summarize the characteristics of this rare tumor, with the goal of providing new insights for diagnosis and treatment. MATERIALS AND METHODS: We report three cases of nasopharyngeal amyloidoma diagnosed in our hospital following comprehensive medical examination and review the current literature on all cases of nasopharyngeal amyloidoma from PubMed. The journey of nasopharyngeal amyloidoma, including presentation, diagnostics, surgeries, and follow-up was outlined. RESULTS: None of the three patients had systemic amyloidosis. CT and nasal endoscopy showed irregular masses obstructing the nasopharyngeal cavity. Congo red staining confirmed the deposition of amyloid, and immunohistochemical analysis showed that the amyloid deposition was the AL light chain type. Through literature review, we found that nasopharyngeal amyloidoma most commonly occurred in individuals over the age of 40, patients usually had a good prognosis after complete tumor resection; however, there were still cases of recurrence, and unresected patients were at risk of progression to systemic amyloidosis. The efficacy of radiotherapy and chemotherapy was currently uncertain. CONCLUSION: Early clinical and pathological diagnosis is crucial, and surgical intervention is the primary treatment option for this disease. Although patients usually have a favorable prognosis, long-term monitoring is necessary to detect potential relapses and initiate timely intervention.

[Differential diagnosis to malignant neoplasia: Nasopharyngeal mass in a cat with nasal cryptococcosis in Germany]. / Differenzialdiagnose zur malignen Neoplasie: Nasopharyngeale Masse bei einer Katze mit nasaler Kryptokokkose in Deutschland.

A 2-year-old Norwegian Forest cat was presented for evaluation of bilateral purulent nasal discharge and stertorous breathing. A computed tomography (CT) scan of the head revealed an intranasal mass of the left nasal cavity extending behind the tube openings and completely obstructing the nasopharynx. Rhinoscopy confirmed a pinkish, shiny mass. CT scan showed both compartments of the right middle ear filled with abnormal soft tissue attenuating material. There was no change in the bony outline of the middle ear. In the endoscopic examination, after endoscopically assisted tympanocentesis, this material in the accessible dorsolateral compartment proved to be classic polypous tissue in addition to highly viscous glue-like secretions. A secondary otitis media due to a drainage disorder was suspected.Using an endoscopic-interventional approach through the nostril, the nasopharyngeal mass was removed for histopathological examination, in order to restore the nasal airway, and to allow tube drainage. In contrast to cats with classical malignant nasal cavity masses, the cat showed several attachment points of the mass and multiple undulating elevations bilaterally in the nasopharyngeal mucosa.Cytological and histopathological examination identified the mass as a fungal granuloma in the context of a cryptococcus infection only rarely observed in Germany. Molecular genetic analysis confirmed an infection with Cryptococcus neoformans var. grubii.A single intranasal and nasopharyngeal endoscopic debridement resulted in a significant improvement of the clinical signs and a complete healing of the right middle ear (including the tympanic membrane) within 14 days, but not in a complete cure of the disease. The cat was therefore treated with oral itraconazole solution for several weeks.The case report shows that nasal cryptococcosis can also affect cats in Germany. Rhinoscopy reveals a nasopharyngeal mass with multiple attachment points, which is unusual for a neoplasia. In addition to the recommended removal of the mass, oral administration of systemic antimycotics is strongly advised.

[Primary tuberculosis of the cavum: An unusual diagnosis]. / Tuberculose primitive du cavum : un diagnostic peu commun.

Primary tuberculosis of the cavum is a rare entity. It can occur at any age, especially between the second and ninth decade. We report the case of a 17-years-old patient with nasal obstruction and left laterocervical adenomegaly. A cervico-facial CT scan showed a suspicious looking tumor process of the nasopharynx. Histological analysis of the biopsies taken showed chronic granulomatous inflammation with necrosis and the absence of tuberculosis lesions in the usual sites, especially the lungs, led to the diagnosis of primary tuberculosis of the cavum. There was a good evolution on antituberculosis drugs. This unusual location can be a source of difficulties and delay in diagnosis, especially because of the clinical presentation, which suggests a nasopharyngeal tumour. In developing countries, where this disease remains relatively endemic, cross-sectional imaging techniques and histopathological analysis are of great interest for the management of patients.

Spontaneous Healing of the Crust Type of Nasopharyngeal Bursitis.

The nasopharyngeal bursa is an invaginated space that links the remnants of the notochord with the pharyngeal epithelium. Nasopharyngeal bursitis can occur in the midline of the upper portion of the nasopharynx if the notochord remnants and midline recess continue to persist. Nasopharyngeal bursitis, also known as Tornwaldt cyst, can be divided into cystic and crusting types. Crust types are relatively rare. Electrocauterization of the ulcerative lesion of the crust type is an effective and minimally invasive method. This report presents a case of crust-type nasopharyngeal bursitis that heals spontaneously without electrocauterization therapy.

Evaluation of the intranasal steroid treatment outcomes in adenoid tissue hypertrophy with or without allergic rhinitis.

PURPOSE: To compare the intranasal steroid (INS) treatment outcomes in patients with adenoid tissue hypertrophy (ATH) with or without allergic rhinitis (AR). MATERIALS AND METHODS: Medical records of 96 children diagnosed with ATH were retrospectively examined. The pediatric version of the Score for Allergic Rhinitis (SFAR) questionnaire was used to determine the AR status of the patients and classify them. The children were divided into two groups based on the questionnaire: Group 1, low probability of AR (SFAR<9); and Group 2, high probability of AR (SFAR≥9). Intranasal mometasone furoate (100 µg/mL) was used to treat ATH for at least 3 months. The severity of nasal obstruction and snoring was evaluated using the visual analog scale (VAS) score, the adenoid/choana (A/C) ratios before and after treatment were compared, and the rate of patient referral to surgery was recorded among groups. RESULTS: The change in the A/C ratio within the group between before and after treatment was significant (both P < 0.001). However, the reduction in the adenoid size was more significant in Group 1 than in Group 2 (P = 0.025). A significant improvement in the VAS scores was observed between before and after treatment in both groups (P < 0.001). Furthermore, the rate of surgical referral of Group 1 was significantly lower than that of Group 2 (P = 0.035). CONCLUSIONS: INS treatment was found more successful for reducing A/C ratio in ATH without AR. Related with this, when considering the INS treatment for ATH, AR status should be kept in mind for predicting the treatment success.

A Rare Nasopharyngeal Presentation of Amyloidosis.

Amyloidosis is a heterogeneous group of diseases characterized by the extracellular deposition of insoluble proteins whose pathogenesis is not yet fully understood. The deposition of amyloid proteins can be systemic or localized, idiopathic or related to systemic diseases, mostly multiple myeloma or chronic inflammatory diseases. Localized head and neck amyloidosis is a rare entity, mainly involving the larynx. Given the rarity of the disease and the absence of a lasting follow-up protocol, there is no standard treatment defined for localized amyloidosis. We report a rare case of localized nasopharyngeal amyloidosis, treated with complete transoral resection and confirmed by histological examination.

Primary Nasopharyngeal Tuberculosis: A Case Report Focused on Nasopharyngoscopic Features and CT Findings.

Primary nasopharyngeal tuberculosis, defined as an isolated tuberculosis infection of the nasopharynx without systemic or pulmonary disease, is rare, even in areas endemic for tuberculosis. It is challenging for ENT specialists to diagnose primary nasopharyngeal tuberculosis at an early stage. In this report, we describe a new case of primary nasopharyngeal tuberculosis, focusing on its nasopharyngoscopic features and radiological findings that can help the understanding and aid in accurate diagnosis of this unusual disease entity. Our experience suggests that although primary nasopharyngeal tuberculosis is a relatively rare disease, it must be included in the differential diagnosis of various nasopharyngeal lesions, particularly in patients with unusual nasopharyngoscopic and computed tomography findings.

Longitudinal investigation of nasopharyngeal pneumococcal carriage in early childhood: The PATCH birth cohort study.

Streptococcus pneumoniae is a common cause of infectious diseases such as pneumonia and sepsis. Its colonization is thought to be the first step in the development of invasive pneumococcal diseases. This study aimed to investigate pneumococcal colonization patterns in early childhood. A longitudinal birth cohort study was conducted for investigating nasopharyngeal colonized pneumococci at 1, 6, 12, 18, 24, and 36 months of age, particularly focusing on the serotype distribution and antimicrobial susceptibilities. Pneumococcal conjugate vaccine (PCV) effect on nasopharyngeal colonization was also assessed. During 2013-2017, 855 infants were enrolled and a total of 107 isolates were recovered from 95 infants during the first three years of life. In this period, the prevalence of pneumococcal colonization increased, with values ranging from 0.2% (2/834) at 1 month of age to 5.9% (19/323) at 36 months of age. The investigation of serotype revealed that 81.1% (73/90) belonged to the non-PCV13 serotypes-23A, 15A, 15C, and 15B. Moreover, PCV13 serotypes significantly decreased during 2014-2015, when routine PCV13 vaccination was initiated in Taiwan. PCV13 introduction may lead to the reduction in the rates of pneumococcal isolates resistant (R) to penicillin. Under conditional PCV13 vaccination, pneumococcal isolates primarily belonged to non-PCV13 serotypes. This non-PCV13 serotype replacement exhibited lower rates of penicillin R isolates, suggesting that PCV13 administration may reduce the antibiotic-nonsusceptible pneumococcal disease burden and antibiotic use.

Immunohistochemical and genetic analysis of respiratory epithelial adenomatoid hamartomas and seromucinous hamartomas: are they precursor lesions to sinonasal low-grade tubulopapillary adenocarcinomas?

Respiratory epithelial adenomatoid hamartoma (REAH) and seromucinous hamartoma (SH) are rare tumor-like lesions of the nasal cavity, paranasal sinuses, and nasopharynx. The pathogenesis of REAH/SH is still unclear. Neoplastic proliferation, chronic mechanical irritation, inflammation, or possible embryological tissue misplacement are speculated as possible mechanisms of their development. Low-grade tubulopapillary adenocarcinoma (LGTA) is a rare variant of nonsalivary, nonintestinal type sinonasal adenocarcinoma. The aim of this study was to evaluate the immunohistochemical and genetic profiles of 10 cases of REAH/SH, with serous, mucinous, and respiratory components evaluated separately and to compare these findings with the features of 9 cases of LGTA. All cases of REAH/SH and LGTA were analyzed immunohistochemically with a cocktail of mucin antigens (MUC1, MUC2, MUC4, MUC5AC, MUC6) and with epithelial (CK7, CK20, CDX2, SATB2) and myoepithelial markers (S100 protein, p63, SOX10). The next-generation sequencing assay was performed using FusionPlex Solid Tumor Kit (ArcherDx) in 10 cases of REAH/SH, and the EGFR-ZNF267 gene fusion was detected in 1 of them. Two female REAH/SH cases were assessed for the presence of clonality. Using the human androgen receptor assay, 1 case was proved to be clonal. The serous component of REAH/SH was positive for CK7/MUC1 and SOX10 similarly to LGTA. Although REAH/SH and LGTA are histopathologically and clinically separate entities, the overlap in their morphological and immunohistochemical profiles suggests that REAH/SH might be a precursor lesion of LGTA.

Blastic plasmacytoid dendritic cell neoplasm with unusual extracutaneous manifestation: Two case reports and literature review.

RATIONALE: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematologic malignancy. This disease almost always presents with cutaneous involvement. PATIENT CONCERNS: The 1st patient was a 16-year-old girl who presented with recurrent epistaxis. The 2nd patient was a 17-year-old female who presented with nasal obstruction and voice change for a month. DIAGNOSES: In the 1st patient, sinonasal computed tomography (CT) revealed a 2.9-cm sized, polypoid mass in the nasal cavity. In the 2nd patient, CT scans revealed a large enhancing nasopharyngeal mass involving adenoid and several small indeterminate lymph nodes at the neck. Cutaneous examination was unremarkable for either patient. Biopsy of these 2 masses and bone marrow biopsy were performed. Histologic diagnosis of the 2 cases was BPDCN. INTERVENTIONS: Both patients were treated with induction chemotherapy and received allogenic peripheral blood stem-cell transplant. OUTCOMES: No relapse was observed in the 2 patients for 14 and 11 months, respectively, after transplantation. Interestingly, they had no skin lesions at initial diagnosis or during the course of their illness. LESSONS: We 1st identified nasal cavity as an unusual site of BPDCN. BPDCN should be considered in differential diagnosis of blastic leukemia with an undifferentiated and ambiguous immunophenotype despite the absence of skin lesions.

Hairy polyps: A new case presentation and a pathogenetic hypothesis.

Hairy polyps are benign lesions found in the oropharynx or nasopharynx that are thought to be present at birth and can lead to upper airway obstruction in infants. Also known as naso-oropharyngeal choristoma, they are increasingly viewed as aggregates of bigeminal tissue, likely from the first or second branchial arches, found in aberrant locations. They are benign lesions that are usually successfully treated by surgical excision. Here we present a rare case of a hairy polyp originating in the eustachian tube of a 7-week-old male, discuss our management of the patient, and put forth a new hypothesis as to the origin of these lesions. Laryngoscope, 129:2398-2402, 2019.

Localised nasopharyngeal amyloidosis: the importance of postoperative follow-up.

Localised nasopharyngeal amyloidosis is rare. Findings on physical examination and invasive pattern on CT scan can be misleading as it can resemble nasopharyngeal carcinoma. A 64-year-old man presented with left aural fullness for 6 months. The physical examination showed straw-coloured fluid in the left middle ear and irregular reddish mass at the left side of the nasopharynx. The CT scan showed a lobulated heterogeneous mass at the left side of the nasopharynx involving the left Eustachian tube opening. Pathology report was amyloidosis, thus, surgery was done. After a year, there were new foci of amyloidosis at the right side of the nasopharynx, and a repeat surgery was performed. Two years later, the systemic amyloidosis with underlying IgG4-related disease was suspected due to multiple organ involvement. Surgery is the treatment for localised amyloidosis with compressive symptoms. Close follow-up is important after surgical excision due to its recurrence and progression to systemic amyloidosis.

Outcome After Radiation Therapy for Langerhans Cell Histiocytosis Is Dependent on Site of Involvement.

PURPOSE: To characterize the efficacy and safety of radiation therapy in a contemporary Langerhans cell histiocytosis (LCH) cohort and to explore whether there are sites at higher risk for local recurrence. PATIENTS AND METHODS: Between 1995 and 2015 we identified 39 consecutive LCH patients who were treated primarily with radiation therapy. Patients were staged by single/multisystem involvement and established risk organ criteria. In 46 irradiated lesions, clinical and radiologic responses were evaluated at multiple time points after radiation therapy. Patient demographics, treatment, and local failure were compared by site of lesion. RESULTS: Median age at radiation therapy was 35 years (range, 1.5-67 years). Twelve patients had multisystem involvement, and of those, 5 patients had disease in organs considered to be high risk. The following sites were irradiated: bone (31), brain (6), skin (3), lymph node (3), thyroid (2), and nasopharynx (1). Median dose was 11.4 Gy (range, 7.5-50.4 Gy). At a median follow-up of 45 months (range, 6-199 months), local recurrence or progression was noted in 5 of 46 lesions (11%). There were no local failures of the 31 bone lesions evaluated, whereas the 3-year freedom from local failure in the 15 non-bone lesions was 63% (95% confidence interval 32-83%; P=.0008). Local failures occurred in 2 of 3 skin lesions, in 2 of 6 brain lesions, and 1 of 3 lymph node lesions. Deaths were recorded in 5 of 39 patients (13%), all of whom were adults with multisystem disease. CONCLUSION: Radiation therapy is a safe and effective measure for providing local control of LCH involving the bone. Whereas bone lesions are well controlled with low doses of radiation, disease in other tissues, such as the skin and brain, may require higher doses of radiation or additional treatment modalities.