Nasopharyngeal Papillary Adenocarcinoma Research Articles

Primary Nasopharyngeal Papillary Adenocarcinoma: Report of 2 Cases and Review of the Literature.

The primary nasopharyngeal papillary adenocarcinoma (NPPA) is extremely rare which accounts for less than 0.48% of all malignant neoplasms in the nasopharynx. The clinical features, diagnosis, and treatment of NPPA have not been well described. We present 2 patients with NPPA that were treated with total endoscopic resection and radiotherapy at Vietnam National Cancer Hospital. Through these cases and reviewing of the literature, we provide deeper understanding of NPPA to highlight clinical pathological characteristics and the optimal treatment strategy for patients with pathologically confirmed NPPA. Our 2 cases were successfully treated and remained disease-free 4 years after treatment. The NPPA was usually an indolent tumor with polypoid appearance and slow growth rate and has a good prognosis. Surgical excision, including endonasal endoscopic excision with or without adjuvant radiotherapy, was most effective with a localized and operable tumor.

Clinicopathological features of thyroid-like low-grade nasopharyngeal papillary adenocarcinoma

Objective: To investigate the clinicopathological features, immunophenotype and gene alterations of thyroid-like low-grade nasopharyngeal papillary adenocarcinoma (TL-LGNPPA). Methods: Fifteen case of TL-LGNPPA diagnosed at Zhejiang Cancer Hospital (5 cases) and the First Affiliated Hospital, Zhejiang University School of Medicine (10 cases) from November 2011 to August 2020 were collected. Clinical and pathological examinations, immunohistochemical staining and next-generation sequencing were performed. The clinicopathological and molecular characteristics were summarized, and relevant literature was reviewed. Results: Fifteen patients were identified and included. Their median age was 36 years (range, 20-60 years). The male-female ratio was 1.0∶1.1. The most common symptoms were epistaxis and nasal obstruction. The neoplasms were located on the roof of the nasopharynx or the posterior margin of the nasal septum. The pathological features included complex papillary and glandular structures mainly composed of single or pseudostratified cubic and columnar cells, with mild to moderate cytological atypia. In some cases, spindle cell features, nuclear grooves, ground glass nuclei, squamous metaplasia, or scattered psammoma bodies were identified. In addition, nuclear polar reversal cells, hobnail cells and micropapillary structures were found, but have not been reported in previous literature. Immunohistochemistry showed that the tumor cells were diffusely positive for TTF1, CK7, vimentin and CKpan; focally positive for p40, CK5/6 and p16; and negative for Tg, NapsinA, CK20, CDX2, S-100 and PAX8. The Ki-67 positive rates ranged from 1% to 20% and were≤10% in thirteen cases (13/15). EBER in situ hybridization was negative in all cases. DNA sequencing of 6 specimens was performed and all specimens were found harboring gene mutations (EWSR1, SMAD2, ROS1, JAK3, GRIN2A, ERRCC5, STAT3, and TET2), but no hot spot gene alterations were found. No MSI-H and MMR related gene changes were detected. All tumors showed low tumor mutation burden. All 15 patients underwent endoscopic surgery, and only 1 of them underwent radiotherapy postoperatively. All patients were recurrence free and alive at the end of follow-up periods (range: 23 to 129 months). Conclusions: TL-LGNPPA is a rare indolent tumor of the nasopharynx and exhibits a unique morphology and immunophenotype. Endoscopic resection is an effective treatment for TL-LGNPPA with excellent overall prognosis.

Thyroid-like low-grade nasopharyngeal papillary adenocarcinoma: a clinicopathological and molecular study of four cases.

Thyroid-like low-grade nasopharyngeal papillary adenocarcinoma (TLLGNPPA) is a rare nasopharyngeal carcinoma. To date, less than 60 cases of TLLGNPPA have been reported, and its clinical features and pathogenesis remain unclear. In this paper, four cases of TLLGNPPA were reported to clarify the clinicopathological and molecular features of this disease. Histopathological examination revealed that all tumors had papillary glandular arrangement, with a fibrovascular axis in the tumor stroma and focal nuclear groove. All tumors expressed pan-CK, CK7, and CK19, while TG and Pax-8 were negative, and the Ki-67 index was approximately 1-3%. The expression of TTF-1 was diffusely positive in two cases and focally positive in two cases. EBER was not expressed in four cases. Molecular testing was possible in three cases. No common driver event was noted, but unique, mutually exclusive molecular variants were found in each of the three tumors (FGFR4, PDK1, AXIN2, FOXL2, and PIK3C3), one also with copy number variants in MCL1 and STMN1. All four patients underwent surgical resection of the tumor and had no metastasis or recurrence from 7 to 60 months post-resection. Given the assertion that these tumors do not recur or metastasize in addition to their heterogeneous gene mutation spectrum, we propose that TLLGNPPA is a neoplasm with low malignant potential and should no longer to be referred to as an adenocarcinoma.

Biphasic thyroid-like low-grade nasopharyngeal papillary adenocarcinoma: A case report and literature review

Biphasic thyroid-like low-grade nasopharyngeal papillary adenocarcinoma: A case report and literature review

Genetic profile identification and clinicopathologic characteristics analysis of the thyroid-like low-grade nasopharyngeal papillary adenocarcinoma

Thyroid-like low-grade nasopharyngeal papillary adenocarcinoma (TL-LGNPPA) is an extremely rare malignancy bearing histomorphological similarities to papillary thyroid carcinoma with good prognosis. It’s important to distinguish TL-LGNPPA from other papillary tumors including nasopharyngeal papillary adenocarcinoma (NPPA), metastatic and ectopic papillary thyroid cancer, and metastasized adenocarcinomas, etc. To date, only 48 cases of TL-LGNPPA have been reported in the English literatures. Here, we reported the genomic characteristics of additional 4 cases and reviewed other reports to clarify the clinicopathological features of this tumor. In this study, 41 mutations were detected by whole-exome sequencing, but no typical driver mutations were found. Two sample with Copy Number Variations (CNV) were found (7 q22. 17 q12), of which the segment spanned the regions of RASA4, POLR2J2, SPDYE2, CCL3, CCL4, etc. Additionally, no MSI and HLA LOH were found. To our knowledge, we are the first to reveal the genetic underpinnings of this rare tumor. The clinicopathological features of TL-LGNPPA were characterized, shedding more light on the essential difference between TL-LGNPPA with other papillary tumors.

Low-Grade Nasopharyngeal Papillary Adenocarcinoma: A Review of 28 Patients in a Single Institution.

PurposeLow-grade nasopharyngeal papillary adenocarcinoma (LGNPPA) is a rare nasopharyngeal tumor. This study aimed to analyze the clinical and histopathological features of the disease, and to share our experience of its treatment.Patients and MethodsWe collected demographic data, clinical symptoms, tumor location, pathological features, immunohistochemical results, treatments, and outcomes of 28 patients with pathologically confirmed LGNPPA between 2009 and 2019.ResultsThe median age of the 28 patients was 41.5 years, with a female: male ratio of 1.5:1 (17 females, 11 males). The most common symptom was blood-stained rhinorrhea. The neoplasms were located on the roof of the nasopharynx (RON) in 13 patients, the posterior margin of the nasal septum (PMONP) in 12 patients, the lateral wall of the nasopharynx in one case, and both the RON and PMONP in two patients. Fourteen patients were diagnosed with thyroid-like LGNPPA. Immunohistochemically, the tumors were uniformly positive for cytokeratin 7, cytokeratin 8, vimentin, epithelial membrane antigen, and pan-cytokeratin, and negative for thyroglobulin. Twenty-three patients underwent pure endoscopic surgery, three patients underwent preoperative radiotherapy, and two patients underwent radiotherapy postoperatively. All patients were alive without evidence of lymphatic or distant metastases in the follow-up period (range: 7 to 121 months). Two patients (7%, 2/28) experienced disease recurrence.ConclusionLGNPPA is an indolent tumor with an excellent prognosis. Endonasal endoscopic excision was an effective treatment. It is important to distinguish thyroid-like LGNPPA from metastatic papillary thyroid carcinoma because these diseases have similar microscopic features but different prognoses.

Un cas rare d’adénocarcinome papillaire nasopharyngé thyroid-like de bas grade

Thyroid-like low-grade nasopharyngeal papillary adenocarcinoma is a rare histopathological entity. Patients usually complain of nasal obstruction and epistaxis. Diagnosis is confirmed on endonasal biopsy using immunohistochemical studies. Surgery is the treatment of choice and this pathology exhibits no metastasizes nor recurrence after treatment.

A Case of Thyroid-like Low Grade Nasopharyngeal Papillary Adenocarcinoma of the Posterior Nasal Septum

A Case of Thyroid-like Low Grade Nasopharyngeal Papillary Adenocarcinoma of the Posterior Nasal Septum

Low grade nasopharyngeal papillary adenocarcinoma case report from the Middle East

<p>Nasopharyngeal papillary adenocarcinoma variety is extremely rare type of nasopharyngeal carcinoma. Limited number of cases reported in the literature, with most cases being reported from west and south Asia. We report the first case in English literature from the middle east and south Africa. A young gentleman diagnosed with low-grade nasopharyngeal papillary adenocarcinoma with positive cervical lymph node. He received a combined approach with endoscopic resection of the lesion and concomitant Left neck dissection followed by planned chemoradiotherapy. Through this case report, we would like to share our treatment approach and explore the optimum therapeutic strategies for primary nasopharyngeal papillary adenocarcinomas.</p><p> </p>

A Case of Low Grade Thyroid Like Nasopharyngeal Papillary Adenocarcinoma in Posterior Nasal Septum

Nasopharyngeal papillary adenocarcinoma of low-grade malignancy (LGNPPA) is an extremely rare neoplasm. A thyroid-like LGNPPA morphologically resembles a papillary thyroid carcinoma; the lesion is usually a polypoid or pedunculated mass <1-3 cm in diameter, often in the posterosuperior wall of the nasopharynx. Here, we report a case of a thyroid-like LGNPPA in the left posterior septal wall. Surgical removal of the tumor was performed by transnasal endoscopic resection using laryngeal cutting forceps. The tumor was completely resected, bleeding was minimal, and the patient had no complaints of nasal pain, nasal congestion, or epistaxis. Further evaluation including immunohistochemistry confirmed a diagnosis of thyroid-like LGNPPA. No postoperative adjuvant treatment was administered. (J Clinical Otolaryngol 2019;30:70-73)

Thyroid-Like Low-Grade Nasopharyngeal Papillary Adenocarcinoma with Biphasic Histology

Thyroid-like low-grade nasopharyngeal papillary adenocarcinoma (TL-LGNPPA) is a rare primary adenocarcinoma of nasopharynx. The immunohistochemical pattern of this tumor is similar to that of papillary thyroid carcinoma making this neoplasm a challenging diagnosis. A case of TL-LGNPPA with biphasic morphology is presented. The removed tumor from the nasopharynx exhibited a polypoid appearance. Microscopically, it was composed of papillary structures admixed with a few solid areas of spindle cells. The papillae were lined by columnar epithelium. Both the epithelium and the spindle cells were strongly positive for TTF-1 and CK19 and negative for CK5/6, S-100, and thyroglobulin. Cellular atypia, necrosis, and high mitotic rate were absent. Ki67 was less than 2% in the neoplastic cells. No local recurrence or distant metastasis was reported after 12 months of follow-up. Caution should be taken to differentiate TL-LGPPA from other TTF-1-positive neoplasms, especially papillary thyroid carcinoma, as the prognosis for these two tumors is entirely different.

Thyroid-like low grade nasopharyngeal papillary adenocarcinoma in a 20 year old student: A case report and review of literature

Low grade nasopharyngeal papillary adenocarcinoma (LGNPA) is a rare variant of nasopharyngeal adenocarcinoma with an excellent prognosis and a histo-morphologic similarity with papillary thyroid carcinoma(PTC). It can be differentiated from PTC using ThyroidTranscriptionFactor-1 (TTF-1) and Thyroglobulin immunostains. We report a case of thyroid-like LGNPA in a 20 year old Nigerian student.Keywords: Papillary Adenocarcinoma, Nasopharynx, Thyroglobulin, Thyroid Transcription Factor-1.

Biphasic low-grade nasopharyngeal papillary adenocarcinoma: a case report and literature review

BackgroundLow-grade nasopharyngeal papillary adenocarcinoma (LGNPPA) is distinctly rare. We report a patient with a uniquely biphasic LGNPPA; additionally, we review similar tumors reported in the literature.Case presentationA 56-year-old man presented with an asymptomatic pedunculated tumor in the vault of the nasopharynx, at the junction of the nasal septum and the roof, which was discovered during screening for laryngeal cancer. To obtain a definitive diagnosis, the patient underwent endoscopic endonasal surgery under general anesthesia. Immunohistochemical analysis of the tumor revealed it to be an LGNPPA with a prominent spindle cell component.ConclusionTo our knowledge, this is the fourth reported LGNPPA exhibiting a spindle cell component and the second with a prominent pathological condition. The prognosis of LGNPPA is usually excellent. Therefore, it is important for clinicians to scrutinize the lesion’s pathology to avoid unnecessary, disfiguring surgery.

Update From The 4th Edition of the World Health Organization Classification of Head and Neck Tumours: Nasopharynx.

The current WHO classification system for tumors of the head and neck has made few changes from the previous edition with regards to tumors of the nasopharynx. The classification system is discussed here with particular attention to nasopharyngeal carcinoma, nasopharyngeal papillary adenocarcinoma, salivary gland anlage tumor, hairy polyp, juvenile angiofibroma, and other tumors.

Thyroid-like low-grade nasopharyngeal papillary adenocarcinoma with papillary carcinoma of thyroid: report of a case

Thyroid-like low-grade nasopharyngeal papillary adenocarcinoma with papillary carcinoma of thyroid: report of a case

Thyroid-like low-grade nasopharyngeal papillary adenocarcinoma: A case report.

Thyroid-like low-grade nasopharyngeal papillary adenocarcinoma (TL-LGNPPA) is a rare neoplasm characterized by morphological analogy to papillary thyroid carcinoma and abnormal expression of thyroid transcription factor-1 (TTF-1). We herein report a rare case of TL-LGNPPA with a review of its clinical, morphological and immunohistochemical characteristics. The patient was a 25-year-old Japanese woman complaining of a 2-year history of fever of unknown origin. There were no remarkable physical findings and the laboratory tests, including C-reactive protein levels, were normal. Laryngoscopy, magnetic resonance imaging and fluorodeoxyglucose-positron emission tomography identified a pedunculated mass at the roof of the nasopharynx. Histologically, the tumour exhibited papillary growth of cuboidal or columnar epithelium. Tubular architecture and a spindle cell component were also observed focally. Some tumour cells exhibited intranuclear cytoplasmic inclusions. Immunohistochemically, the neoplastic cells were positive for TTF-1, cytokeratin 7 and vimentin, but were negative for thyroglobulin. The Ki-67 labelling index (MIB-1 index) reached 5% in the most concentrated spot. The patient had neither local recurrence nor distant metastasis 3 years after removal of the tumour. In conclusion, TL-LGNPPA should be included it in the differential diagnosis of fever of unknown origin.

Spreading patterns, prognostic factors and treatment outcomes of nasopharyngeal papillary adenocarcinoma and salivary gland-type carcinomas.

In this study, we aim to analyse the different spreading patterns, prognostic factors and treatment outcomes of nasopharyngeal papillary adenocarcinoma (NPAC) and salivary gland-type carcinomas (NPCs). The current study report on a retrospective analysis of oncologic outcome of 76 pathologically confirmed consecutive cases of nasopharyngeal adenocarcinomas (NAC), including 31 NPAC, 33 adenoid cystic carcinomas (ACC) and 12 mucoepidermoid carcinomas (MEC). Overall survival rates (OS) and disease-free survival rates (DFS). In 12 patients with cranial nerve (CN) palsy, there were ACC (n = 9), NPAC (n = 2) and MEC (n = 1) (P = 0.016). CT-/MRI-detected CN involvements were found in 22 patients. Lymph node metastasis was observed in 25.8% of NPAC (n = 8), 12.1% of ACC (n = 4) and 8.3% of MEC (n = 1). Significant differences were observed in 5-year overall survival (OS) and disease-free survival (DFS) rates between patients with and without CT-/MRI-detected CN involvement (P = 0.002 and P = 0.002, respectively), and similar results were found between patients with and without lymph node metastasis (P = 0.002 and P = 0.018, respectively). In 37 patients with early-stage disease (stages I-II), significant differences were observed in 5-year OS and DFS rates between the surgical and non-surgical treated groups (P = 0.031 and P = 0.012, respectively). In 39 patients with advanced-stage disease (stages III-IV), significant or marginally differences were observed in DFS and OS between the chemoradiotherapy and non-chemoradiotherapy groups (P = 0.007 and P = 0.062, respectively). ACC has a higher CN invasion than NPAC and MEC, and NPAC has the highest rate of lymphatic metastases. CT-/MRI-detected CN involvements and lymph node metastasis indicate a negative impact on the prognosis. The outcome of surgical patients in our series is encouraging in early-stage NPAC and NPCs, and chemoradiotherapy may be the optimal treatment for the advanced-stage patients.

Clinicopathological features of low-grade nasopharyngeal papillary adenocarcinoma

To study the clinicopathological features, immunophenotype, differential diagnosis and prognosis of low-grade nasopharyngeal papillary adenocarcinoma (LGNPPA). The histopathological features and clinical and pathological data of nine cases of LGNPPA were retrospectively analyzed. Immunohistochemistry (Two-step EnVision methods) was used to evaluate the expression of CKpan, vimentin, CK7, CK19, TTF-1 and TG; in situ hybridization was used to detect Epstein-Barr virus mRNA (EBER); and flow-through hybridization was used to evaluate the presence of human papilloma virus (HPV). The mean age for the nine patients (eight males, one female) was 45.3 years (range 23 to 62 years). Microscopically the tumors were characterized by lobulated, papillary and glandular structures with patchy distribution of spindle cells. The papillary interstitial tissue was edematous, myxoid or hyalinized. The tumors were unencapsulated and infiltrated into the surrounding stroma. Four cases displayed transition between normal nasopharyngeal epithelium to neoplastic cells; and one case contained psammoma bodies. Five cases were strongly positive for CKpan, vimentin, CK7, CK19, TTF-1, and were focally positive for EMA and CD117. These five cases were all negative for TG, CK5/6, CK20, S-100 protein, p63, Calponin and SMA. In situ hybridization for EBER and flow-through hybridization for HPV were negative in all five cases. Follow-up data showed no post-operative recurrence of the LGNPPA. LGNPPA is a rare low-grade neoplasm with distinct morphological characteristics. Its diagnosis is primarily based on the site of lesions and the histological features. The diagnosis and differential diagnosis of LGNPPA could be aided by immunohistochemical staining. LGNPPA may originate from nasopharyngeal epithelium; and the prognosis is good with simple and complete resection.

Thyroid-like low-grade nasopharyngeal papillary adenocarcinoma: Case report and literature review

Thyroid-like low-grade nasopharyngeal papillary adenocarcinoma (TL-LGNPPA) is an extremely rare neoplasm characterized by morphological analogy to papillary thyroid carcinoma and abnormal expression of thyroid transcription factor-1 (TTF-1). Here we report a novel case of TL-LGNPPA with literature review. The patient was a 43-year-old woman complaining of nasal obstruction. Laryngoscopic study and computed tomography identified a pedunculated mass located on the posterior edge of the left nasal septum. Histologically, the tumor consisted of papillary growth of cuboidal or columnar epithelium. Tubular architecture and a spindle cell component were also observed focally. Some tumor cells exhibited intra-nuclear cytoplasmic inclusions. Immunohistochemically, the neoplastic cells were positive for pancytokeratin (AE1/AE3), CK7, CK19, TTF-1, vimentin and HBME1, but negative for thyroglobulin, Pax8 and CK5/6. Ki67-labeling index reached 5% in the most concentrated spot. Despite the morphological and immunohistochemical similarity to papillary thyroid carcinoma, no BRAF V600E mutation was detected by mutation-specific immunohistochemistry. The patient had neither local recurrence nor distant metastasis 19 months after removal of the tumor.

Positive immunostaining of thyroid transcription factor-1 in primary nasopharyngeal papillary adenocarcinoma

Department of Otolaryngology, Buddhist Tzu Chi General Hospital, Taipei Branch, New Taipei City, Taiwan Department of Pathology, National Taiwan University Hospital and National Taiwan University, College of Medicine, Taipei, Taiwan c Institute of Biomedical Engineering, College of Medicine and College of Engineering, National Taiwan University, Taipei, Taiwan Department of Otolaryngology, National Taiwan University Hospital and National Taiwan University, College of Medicine, Taipei, Taiwan Department of Otolaryngology, Buddhist Tzu Chi General Hospital, Taichung Branch, Taichung, Taiwan f School of Medicine, Tzu Chi University, Hualien, Taiwan