Clinical and molecular characterisation of metastatic papillary thyroid cancer according to radioiodine therapy outcomes.

PURPOSE: Radioiodine (RAI) therapy remains the gold-standard approach for distant metastatic differentiated thyroid cancer (TC). The main objective of our work was to identify the clinical and molecular markers that may help to predict RAI avidity and RAI therapy response of metastatic lesions in a cohort of papillary thyroid cancer (PTC) patients. METHODS: We performed a retrospective analysis of 122 PTC patients submitted to RAI therapy due to distant metastatic disease. We also analysed, through next-generation sequencing, a custom panel of 78 genes and rearrangements, in a smaller cohort of 31 metastatic PTC, with complete follow-up, available RAI therapy data, and existing tumour sample at our centre. RESULTS: The most frequent outcome after RAI therapy was progression of disease in 59.0% of cases (n = 71), with median estimate progression-free survival of 30 months. RAI avidity was associated with PTC subtype, age and stimulated thyroglobulin at first RAI therapy for metastatic disease. The most frequently altered genes in the cohort of 31 PTC patients' primary tumours were RAS isoforms (54.8%) and TERT promoter (TERTp) (51.6%). The presence of BRAF p.V600E or RET/PTC alterations was associated with lower avidity (p = 0.012). TERTp mutations were not associated with avidity (p = 1.000) but portended a tendency for a higher rate of progression (p = 0.063); similar results were obtained when RAS and TERTp mutations coexisted (p = 1.000 and p = 0.073, respectively). CONCLUSIONS: Early identification of molecular markers in primary tumours may help to predict RAI therapy avidity, the response of metastatic lesions and to select the patients that may benefit the most from other systemic therapies.

Ectopic thyroid gland resembling a thyroglossal duct cyst.

Key Clinical Message: This rare diagnosis highlights the importance of anatomical and embryological knowledge in the differential diagnosis of neck masses. Abstract: A 41-year-old woman was referred to the head and neck surgery department because of a large midline neck mass at the level of the hyoid bone that was diagnostic for functioning thyroid tissue in a totally ectopic location.

Ectopic Cushing's Syndrome Secondary to Acinic Cell Carcinoma of the Parotid Gland: A Case Report.

We present a case report of a patient with a rare high-grade transformation of an acinic cell carcinoma (ACC) of the parotid gland, who developed Cushing's syndrome (CS) as a result of ectopic secretion of adrenocorticotropic hormone by the tumour. The hypercortisolism was successfully treated with metyrapone, and the ACC was treated with local radiotherapy and a combined six cycles of gemcitabine and cisplatin, having achieved a partial response to the tumour. A multidisciplinary approach and combined medical treatment with radiotherapy and were essential for disease control and CS management. ACC should be considered in the differential diagnosis of ectopic CS.

Antioxidant Potential of Tamarillo Fruits-Chemical and Infrared Spectroscopy Analysis.

Native to South America, tamarillo (Solanum betaceum Cav.) is a small tree cultivated as a fruit crop in several regions of the world. Known for its sweet and sour taste, tamarillo fruits are very nutritious due to the presence of health-beneficial components such as fiber, vitamins, and antioxidants. Despite its nutritional value, tamarillo remains poorly known in global markets. The present work aims to study the antioxidant activity of four genotypes of tamarillo. Several chemical assays were performed to assess the antioxidant components and antioxidant activity of aqueous ethanolic extracts from each genotype. Overall, the Mealhada genotype (a red cultivar) showed the most interesting results, displaying the highest amount of total phenolic, flavonoids, and anthocyanin contents, as well as higher antioxidant activity. To evaluate the composition of the extract, Fourier-transform infrared spectroscopy (FTIR) was used to characterize important components in aqueous ethanolic extracts of the fruits, having revealed the presence of high amounts of phenols (the main compounds responsible for antioxidant activity), as well as triterpenoids and polysaccharides. The present results highlight the potential nutraceutical importance of tamarillo fruits.

Target therapy for BRAF mutated anaplastic thyroid cancer: a clinical and molecular study.

OBJECTIVES: Anaplastic thyroid carcinoma (ATC) has a poor survival. The combination of Dabrafenib plus Trametinib (DT) had a significant impact in survival of BRAF p.V600E patients. However, durable responses may be compromised by resistance. We aim to present our experience with DT in BRAF positive ATC patients and compare the outcomes with usual therapy, and to study tumor molecular alterations in the DT group. METHODS: Patients treated between May 2018 and April 2022 in a tertiary referral center, assessed for BRAF status were included. Patients were divided in three groups: BRAF p.V600E treated with DT, BRAF wild type (WT) under multimodal therapy (MT), and BRAF WT under compassionate care (CC). Response was assessed monthly in the first 6 months and every 3 months afterwards, by RECIST 1.1. Overall survival (OS) and progression-free survival (PFS) were estimated with the Kaplan-Meier method and compared with the log-rank test. RESULTS: Twenty-seven ATC patients were included (DT = 9, MT = 8, and CC = 10). Median OS was 475 days for DT, 156 days for MT, and 39 days for CC (P < .001). At 12 months, only patients in the DT group were alive (71%). Median PFS was 270 days, in the DT group, compared with less than 32 days in BRAF WT (P < .001). No severe adverse events were reported. Molecular profiling showed that in one of the four clinical progressions, a pathogenic NRAS mutation was found. CONCLUSIONS: Our results show a significant real-world efficacy of Dabrafenib plus Trametinib in both survival and recurrence compared with standard treatment, with a good safety profile.

Entrectinib in the neoadjuvant setting of anaplastic thyroid cancer: a case report.

Background: Anaplastic thyroid carcinoma (ATC) is one of the most aggressive solid tumors. ATC is frequently diagnosed at advanced stages with unresectable disease and palliative care is often indicated. Recently, several patient-tailored therapies for ATC are emerging due to advances in molecular profiling of these tumors. Entrectinib is a potent oral selective inhibitor of neutrotrophic tropomyosin receptor kinase (NTRK), ROS1, and anaplastic lymphoma kinase fusions. The experience regarding ATC and other thyroid carcinomas, particularly in the neoadjuvant setting, is minimal. Case report: We present a case of a 51-year-old female patient presenting with a bulky mass of the left thyroid lobe measuring 100 × 108 × 80 mm that was considered surgically unresectable. While waiting for next-generation sequence (NGS) profiling, lenvatinib was initiated. There was an initial clinical and imagiologic response; however, progression occurred after 12 weeks, and at this time NGS identified an ETV6-NTRK3 fusion and entrectinib was started. After 12 weeks, tumor diameters reduced to a minimum of 68×60×49 mm, and the patient underwent total thyroidectomy plus central lymphadenectomy. Histological diagnosis confirmed an ATC (pT4a R2 N1a). Adjuvant radiotherapy (RT) (60 Grays) with weekly paclitaxel (45 mg/m2) was then administered followed by maintenance entrectinib 600 mg daily. Fluorodeoxyglucose positron emission tomography performed 3 months after completion of RT showed only non-specific uptake in the posterior wall of the hypopharynx and larynx, suggestive of inflammation. Conclusion: We report the first case of an ATC with a dramatic response to neoadjuvant therapy with entrectinib, which enabled surgical resection of an ab initio unresectable tumor.

Basal Cell Adenoma and Basal Cell Adenocarcinoma: a 50-year Experience From a Single Institution.

BACKGROUND: Basal cell adenoma (BCA) and adenocarcinoma (BCAd) are two of the least frequent salivary gland tumors. We describe the largest series of these neoplasms, spanning over a period of 50 years (1970-2020), diagnosed and treated in a single Institution. METHODS: Sixty-eight cases were identified. Clinical and pathological data were collected and correlated with outcome. RESULTS: Forty-one BCA and 27 BCAd were identified. BCA cases had almost pristine prognosis, with only a relapse in a tumor inadequately excised. Ten patients with BCAd developed metastases, and 14 died from the disease. The 2-year and 5-year survival was of 76% and 42%. CONCLUSIONS: The importance of adequate excision is reinforced in BCA, with no recurrences occurring when margins were negative. Contrary to previous reports, BCAd was not associated with a good prognosis. A better understanding of the genetics of these neoplasms may identify therapeutic options when dealing with inoperable or metastatic disease.

Mucocele of the appendix: what to expect.

Mucoceles of the appendix are rare and can have quite variable imaging and clinical presentations, sometimes mimicking an adnexal mass. The underlying cause can be neoplastic or non-neoplastic. The typical imaging appearance of a mucocele of the appendix is that of a cystic structure with a tubular morphology. This structure is defined by having a blind-ending and being contiguous with the cecum. Radiologists should be familiar with key anatomical landmarks and with the various imaging features of mucoceles of the appendix, in order to provide a meaningful differential diagnosis of a lesion in the right lower abdominal quadrant. In addition, a neoplastic mucocele can rupture, resulting in pseudomyxoma peritonei, which will change the prognosis dramatically. Therefore, prompt diagnostic imaging is crucial.

Mucoceles do apêndice são raras e podem ter uma apresentação clínica e imagiológica bastante variável, por vezes mimetizando patologia anexial. As causas subjacentes podem ser neoplásicas ou não neoplásicas. O aspecto de imagem típico de mucoceles do apêndice é o de uma estrutura de natureza cística com morfologia tubular. Esta estrutura deverá terminar "em fundo cego" e ser contígua com o ceco. Os radiologistas devem estar familiarizados com os pontos anatômicos de referência e com as diferentes características imagiológicas de mucoceles do apêndice, de modo a fornecer um adequado diagnóstico diferencial de uma lesão localizada no quadrante abdominal inferior direito. Para além disso, uma mucocele neoplásica pode sofrer ruptura, resultando em pseudomixoma peritoneal, o que altera drasticamente o prognóstico. Assim, o diagnóstico por imagem em tempo útil é crucial.

Mucocele of the appendix: what to expect / Mucocele do apêndice: o que esperar

Abstract Mucoceles of the appendix are rare and can have quite variable imaging and clinical presentations, sometimes mimicking an adnexal mass. The underlying cause can be neoplastic or non-neoplastic. The typical imaging appearance of a mucocele of the appendix is that of a cystic structure with a tubular morphology. This structure is defined by having a blind-ending and being contiguous with the cecum. Radiologists should be familiar with key anatomical landmarks and with the various imaging features of mucoceles of the appendix, in order to provide a meaningful differential diagnosis of a lesion in the right lower abdominal quadrant. In addition, a neoplastic mucocele can rupture, resulting in pseudomyxoma peritonei, which will change the prognosis dramatically. Therefore, prompt diagnostic imaging is crucial.

Resumo Mucoceles do apêndice são raras e podem ter uma apresentação clínica e imagiológica bastante variável, por vezes mimetizando patologia anexial. As causas subjacentes podem ser neoplásicas ou não neoplásicas. O aspecto de imagem típico de mucoceles do apêndice é o de uma estrutura de natureza cística com morfologia tubular. Esta estrutura deverá terminar "em fundo cego" e ser contígua com o ceco. Os radiologistas devem estar familiarizados com os pontos anatômicos de referência e com as diferentes características imagiológicas de mucoceles do apêndice, de modo a fornecer um adequado diagnóstico diferencial de uma lesão localizada no quadrante abdominal inferior direito. Para além disso, uma mucocele neoplásica pode sofrer ruptura, resultando em pseudomixoma peritoneal, o que altera drasticamente o prognóstico. Assim, o diagnóstico por imagem em tempo útil é crucial.

PIK3CA Gene Mutations in HNSCC: Systematic Review and Correlations with HPV Status and Patient Survival.

PIK3CA mutations are believed to contribute to the pathogenesis of human papillomavirus (HPV)-associated head and neck squamous cell carcinomas (HNSCC). This study aims to establish the frequency of PIK3CA mutations in a Portuguese HNSCC cohort and to determine their association with the HPV status and patient survival. A meta-analysis of scientific literature also revealed widely different mutation rates in cohorts from different world regions and a trend towards improved prognosis among patients with PIK3CA mutations. DNA samples were available from 95 patients diagnosed with HNSCC at the Portuguese Institute of Oncology in Lisbon between 2010 and 2019. HPV status was established based on viral DNA detected using real-time PCR. The evaluation of PIK3CA gene mutations was performed by real-time PCR for four mutations (H1047L; E542K, E545K, and E545D). Thirty-seven cases were found to harbour PIK3CA mutations (39%), with the E545D mutation (73%) more frequently detected. There were no significant associations between the mutational status and HPV status (74% WT and 68% MUT were HPV (+); p = 0.489) or overall survival (OS) (3-year OS: WT 54% and MUT 65%; p = 0.090). HPV status was the only factor significantly associated with both OS and disease-free survival (DFS), with HPV (+) patients having consistently better outcomes (3-year OS: HPV (+) 65% and HPV (-) 36%; p = 0.007; DFS HPV (+) 83% and HPV (-) 43%; p = 0.001). There was a statistically significant interaction effect between HPV status and PIK3CA mutation regarding DFS (Interaction test: p = 0.026). In HPV (+) patients, PIK3CA wild-type is associated with a significant 4.64 times increase in the hazard of recurrence or death (HR = 4.64; 95% CI 1.02-20.99; p = 0.047). Overall, PIK3CA gene mutations are present in a large number of patients and may help define patient subsets who can benefit from therapies targeting the PI3K pathway. The systematic assessment of PIK3CA gene mutations in HNSCC patients will require further methodological standardisation.

Diffuse leiomyomatosis: A rare cause of a diffusely enlarged uterus.

Diffuse uterine leiomyomatosis is a rare and benign condition which involves the development of innumerable poorly defined, confluent smooth muscle nodules that replace most of the uterine parenchyma. It results in a symmetrically enlarged uterus. The etiology of these benign tumors is not completely understood. Patients with leiomyomatosis usually present with menorrhagia or dysmenorrhea, abdominal pain and infertility and in most cases hormonal treatment fails to control the symptoms. In this manuscript, we present a case of a 36-year-old woman who underwent hysterectomy due to diffuse uterine leiomyomatosis with a review of the literature.

Dentinogenic ghost cell tumor - Case report of a rare entity.

INTRODUCTION: Dentinogenic ghost cell tumor (DGCT) is an entity with about 60 cases reported in the literature. It is a benign odontogenic tumor, despite being locally invasive and associated with a risk of local recurrence. PRESENTATION OF CASE: A 47-year-old woman presented with a 2-year-old expansive bone lesion. Radiologically, a multilocular mass was identified in the left superior maxilla, compatible with a tumor of odontogenic origin. She was submitted to an extended resection, and the histology was consistent with a DGCT. DISCUSSION: Central DGCT affects mainly male patients between the fourth and sixth decades, with a predilection for the posterior portion of the jaws. The symptoms are unspecific, and a vast percentage of patients is asymptomatic. Radiographically a unilocular feature is commonly found, unlike this case. The recommended treatment is extended local resection due to its high recurrence rate. CONCLUSION: Due to its rarity, knowledge of this entity is necessary for a better diagnostic and therapeutic guidance.

Mucinous cystadenocarcinoma of the oral tongue: malignant transformation from a mucinous cystadenoma?

Mucinous cystadenocarcinoma of minor salivary glands is an extremely rare entity that has only recently been described, with a few published cases in the English literature. A 42-year-old woman with a history of a surgically excised mucinous cystadenoma of the oral tongue, presented with a painful swelling in the oral tongue slowly growing for 1 month. On clinical examination, there was a firm, relatively well-circumscribed mass in the left posterior border of the mobile tongue. Subsequent MRI scan revealed a heterogeneous lesion composed of multiple cysts separated by contrast enhancing septa, in the posterior two-thirds of the left tongue. Imaging findings were similar to those of the previously resected mass, suggesting local relapse of the primary lesion. A complete surgical excision was performed and the histopathological examination revealed typical features of a low-grade mucinous cystadenocarcinoma of minor salivary glands.

Label-free quantitative mass spectrometry from formalin-fixed paraffin-embedded samples of nasopharyngeal carcinoma: Preliminary results from a non-endemic European cohort of patients.

AIM: Report our results of biomarker discovery in formalin-fixed paraffin-embedded (FFPE) nasopharyngeal carcinoma (NPC) via proteomic analysis. BACKGROUND: Nasopharyngeal carcinoma (NPC) is a rare cancer in Western countries. Proteomic analysis have already been reported as a useful tool to provide biomarkers. Formalin-fixed paraffin-embedded (FFPE) samples, despite largely underused, can provide invaluable information for biomarker research via proteomic analysis. METHODS: FFPE samples of NPC were submitted to protein extraction followed by FASP-digestion and label-free quantitative mass spectrometry (MS). Patients' received concurrent chemoradiation with or without adjuvant chemotherapy as per Intergroup 0099 trial. IMRT was delivered following the RTOG0615 specifications. Toxicity was scored using the CTCAE 4.03 tables. Survival was estimated using Kaplan-Meier curves. Log-rank was used to detect differences. KEGG ontology graphics were generated. RESULTS: 28 FFPE samples from NPC patients were used. Patients were: 79% male, 97% Caucasians, 86% WHO type 3, 40% T1, 10% T2, 25% T3, and 25% T4. With a median follow up of 37 months, local control was 83 (T1, 100% T2, T3 and T4), overall survival was 84%, and six patients developed distant metastases. All five patients that died were due to metastatic disease. Tumor samples contained a median of 75% of tumor material. We found Epstein-Barr (EBV) and Herpes simplex (HSV) viruses' related proteins significantly present in early-stage primary NPC (T1 and T2, p < 0.01). A pool of 10 proteins was statistically up-regulated in the metastatic group of patients (p < 0.01). Median survival from this M1 group was <1 year (p < 0.001). CONCLUSIONS: FFPE samples yielded adequate material for MS analysis. We found EBV and HSV related proteins on early-stage NPC, and proteomic profiling associated with distant metastases, potential candidates of disease biomarkers. Validation is needed.

Improved long-term results of intensity-modulated radiotherapy for a non-endemic European nasopharyngeal carcinoma cohort: single-center retrospective study.

PURPOSE: Report our matured outcomes of European nasopharyngeal carcinoma (NPC) treatment from a non-endemic region in the IMRT era. METHODS: We reviewed 109 consecutive patients with biopsy proven NPC treated between 2009 and 2013. All received IMRT as per RTOG 0615. Toxicity was scored accordingly to CTCAE 4.03. Platinum-based chemotherapy was delivered following the Intergroup 0099. RESULTS: Median age of 53 years; 97% Caucasian; 74% male; 72% WHO grade III; 43% T1; 14% T2; 18% T3, 25% T4; 17% N0; 17% N1; 39% N2; 27% N3. Compliance to adjuvant chemotherapy was 88%. With a median follow up of 56 months, the 4-year local control was 90.2% (88.6% for T1; 100% for T2; 85% for T3; and 91.7% for T4), the 4-year distant metastases-free survival was 86% and an overall survival rate was 77%. Local control and survival were better in G3 (p < 0.001 and p = 0.032, respectively). Xerostomia was the most frequent late toxicity in 55% (n = 60). Hypothyroidism requiring hormonal reposition occurred in 15.5% (n = 17). From the 36 deaths, 20 were due to distant metastases, 3 grade 5 toxicity, 2 from local progression, 5 non-cancer deaths and unknown cause in the remaining 6. On multivariable analysis, age (p = 0.017), local recurrence and distant metastases were associated with death (p < 0.001, both). CONCLUSION: Our matured data from the IMRT era showed a major improvement from our 3D cohort series reaching excellent local and regional control, even in T4. Local recurrences, despite few, and distant metastases were correlated with the risk of death.

Atypical mandibular metastasis as the first presentation of a colorectal cancer.

We describe a case of a 70-year-old man presenting with a mandibular metastasis as the first sign of a mucinous adenocarcinoma of the rectum. After 6 months of a protracted toothache, the patient presented with a palpable mandibular mass and trismus, precluding adequate clinical evaluation. A CT scan was performed, and imaging findings suggested an aggressive primary jawbone tumour, most likely an osteosarcoma. However, biopsy and further patient's management proved to be a metastasis. Metastasis to the oral cavity account for only 1%-3% of all malignant oral tumours, and the mandible is the most frequent site. Clinical presentation can be quite variable, and most often a primary malignancy is already known. Jawbone metastases are a sign of disseminated malignant neoplasms, with poor prognosis and usually an indication for palliative therapy.

Frequent and differential mutations of the CYLD gene in basal cell salivary neoplasms: linkage to tumor development and progression.

Basal cell salivary neoplasms display similar cyto-morphologic features and are classified into adenoma and adenocarcinoma based on the presence or absence of tumor invasion at diagnosis. These neoplasms also share considerable phenotypic resemblance and co-exist with certain dermal adnexal tumors harboring the CYLD gene mutations inferring common genetic association. We sequenced the CYLD gene in both basal cell adenomas and adenocarcinomas and correlated the findings with CYLD, NF-κB, and ß-catenin expression levels and clinicopathologic factors. Twenty mutations were identified and comprised of 3 synonymous and 17 non-synonymous (missense) types involving the coding exons of the CYLD gene. Mutations in exons 9-11 were identified in both adenomas and adenocarcinomas, while mutations in exons 12-20, encoding the USP domain, were exclusively found in carcinomas. Although no significant correlation between CYLD mutations and expression levels of CYLD, NF-κB, and ß-catenin or clinicopathologic parameters was found, basal cell adenocarcinomas with multiple mutations showed reduction in CYLD protein expression and pursued aggressive clinical behavior. Our study revealed high incidence and sequential CYLD mutations in both basal cell adenoma and adenocarcinoma supporting a single neoplastic continuum for their evolution and provides evidence for potential diagnostic and therapeutic utility.

Cutaneous Richter Syndrome Mimicking Primary Cutaneous CD4-Positive Small/Medium T-cell Lymphoma: Case Report and Review of the Literature.

Richter syndrome represents the transformation of chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma into an aggressive large B-cell lymphoma. Skin involvement is exceptionally rare, with <20 cases reported and its presence as the first presentation of CLL/small lymphocytic lymphoma, as an isolated skin lesion has never been described. Primary cutaneous CD4-positive small/medium T-cell lymphoma (CD4 PCSM-TCL) characteristically presents with a dense dermal infiltrate consisting primarily of small-/medium-sized pleomorphic T-cells and less than 30% large pleomorphic cells. A polymorphous reactive infiltrate composed of B-cells, histiocytes, plasma cells, and eosinophils is also found in differing proportions. Because of these morphological characteristics, the differential diagnosis includes not only other forms of cutaneous T-cell lymphomas but also B-cell lymphomas and reactive lymphoid infiltrates. We report a case of a cutaneous Richter syndrome as the initial manifestation of CLL, mimicking CD4 PCSM-TCL, in a 65-year-old apparently healthy asymptomatic man who presented with a solitary nodule in his arm. The objective of this study is to draw attention to this potential pitfall and describe this rare presentation.

Salivary Gland Neoplasms: Does Morphological Diversity Reflect Tumor Heterogeneity.

Salivary gland tumor classification encompasses a vast list of benign and malignant neoplasms. Their morphological diversity is recognized not only between different entities but also within individual tumors. Tumor categories as described by the World Health Organization reflect, in part, a true genetic heterogeneity (e.g., translocations involving CRTC1 and CRTC3-MAML2 genes in mucoepidermoid carcinoma and MYB-NFIB fusion in adenoid cystic carcinoma). Carcinoma ex pleomorphic adenoma shows diversity in its histological appearance, but recurrent rearrangements on PLAG1 and HMGA2 are common to its benign precursor. More recently, new categories have been defined, like secretory carcinoma with the t(12;15) (p13;q25) ETV6-NTRK3 translocation and clear-cell carcinoma with EWSR1-ATF1 fusion. Recent studies on cribriform adenocarcinoma of minor salivary gland origin and epithelial-myoepithelial carcinoma point to a correlation with their morphological features. All of these advances show that the search of a histogenetic and genetic basis for salivary gland tumors is helping to clarify morphological categories and unraveling new ones. Nevertheless, currently morphology is still the hallmark of tumor classification and the gold standard. The therapeutic options for advanced tumors remain very limited but the discovery of translocation-generated gene fusions and increased knowledge of the genomic information of salivary gland tumors is creating opportunities for the development of specific targeted therapies.

Ganglioneuroma of the retropharyngeal space in a patient with glottic cancer.

We describe the case of a 71-year-old man with a ganglioneuroma of the retropharyngeal space. The patient presented with a submucosal bulge of the left oropharyngeal wall during follow-up examination of a treated vocal cord carcinoma. CT and MRI revealed a non-specific, well-defined retropharyngeal soft tissue lesion. Positron emission tomography-CT did not show relevant metabolic activity, excluding the hypothesis of metastatic nodal disease. Surgical biopsy of the lesion was compatible with ganglioneuroma. Ganglioneuromas are well-differentiated tumours composed of mature sympathetic ganglion cells that account for approximately 1% of spinal and paraspinal tumours. Peripherally, ganglioneuromas are predominantly found in the posterior mediastinum and retroperitoneal space. Only nine ganglioneuromas of the retropharyngeal space have been reported in the English literature since the early 1980s.