Diffuse sclerosing variant of papillary thyroid carcinoma: outcomes of 33 cases

Introduction: Diffuse sclerosing variant of PTC (DSV-PTC) is an uncommon subtype of thyroid cancer. Although an aggressive behavior is often recognized, prognostic significance is still under debate. Objectives: To describe the clinicopathological features and outcomes of a series of DSV-PTC patients. Methods: Retrospective data collection regarding 33 patients diagnosed with DSV-PTC followed at the Endocrine Department of the Portuguese Institute of Oncology in Lisbon between 1981 and 2020. Results: 26 patients (78.8%) were females with a mean age at presentation of 29.4±11.7 years-old. Mean time of follow-up was 19.5±10.6 years (range 0.5 to 39). Histologically, bilateral tumors were present in 72.7% patients (n=24), thyroid capsular invasion was documented in 57.6% (n=19), 45.4% (n=15) had extrathyroidal extension (ETE), and 42.4% (n=14) had lymphovascular invasion. Most patients were staged pT3 (42.4%, n=14) and pN1 (81.8%, n=27). Median lymph nodes resected were 16. None of the patients showed distant metastases at presentation. All patients were treated at least once with 131I. During follow-up, 4 patients (14.8%), with persistent neck disease, were diagnosed with distant metastases, all of them in the lung. Two patients (1.8%) presented recurrent disease in the neck after being considered with no evidence of disease. At the last appointment, 18 patients (54.5%) were in remission, 4 (12.1%) had biochemical evidence of disease, 6 had structural disease, and for 5 patients disease status was considered as undetermined. There was no disease related mortality. Discussion/Conclusion: Our study confirms that DSV-PTC is diagnosed more often in young patients and exhibits a local extensive disease at presentation. On the other hand, even in the presence of distant metastases, no patient died during follow-up.

Assessment and management of metastatic neck disease

Tumour spread to the neck can occur from any primary sites that can harbour squamous cell carcinoma (SCC) in the upper aerodigestive tract (UADT) mucosa. This can also happen with other histological subtypes and from primary sites in the parotid and thyroid gland. Thus, the assessment of the neck should form an integral part of the clinical examination, imaging, and decision-making process prior to deciding treatment of the primary tumour. Given the propensity of these tumours to spread to the lymph nodes and given that the treatment algorithms used to manage lymph nodes in the neck are similar to those options available for the primary site, neck status often influences the choice of treatment. This chapter outlines the assessment and management of the metastatic neck node from an SCC of the UADT. To ensure uniformity in describing and communicating information about neck disease, the neck is divided into six levels, as recommended by the American Head and Neck Society and the American Academy of Otolaryngology–Head and Neck Surgery.

Molecular Pathways and Druggable Targets in Head and Neck Squamous Cell Carcinoma

Head and neck cancers are a heterogeneous group of neoplasms, affecting an ever increasing global population. Despite advances in diagnostic technology and surgical approaches to manage these conditions, survival rates have only marginally improved and this has occurred mainly in developed countries. Some improvements in survival, however, have been a result of new management and treatment approaches made possible because of our ever-increasing understanding of the molecular pathways triggered in head and neck oncogenesis, and the growing understanding of the abundant heterogeneity of this group of cancers. Some important pathways are common to other solid tumours, but their impact on reducing the burden of head and neck disease has been less than impressive. Other less known and little-explored pathways may hold the key to the development of potential druggable targets. The extensive work carried out over the last decade, mostly utilising next generation sequencing has opened up the development of many novel approaches to head and neck cancer treatment. This paper explores our current understanding of the molecular pathways of this group of tumours and outlines associated druggable targets which are deployed as therapeutic approaches in head and neck oncology with the ultimate aim of improving patient outcomes and controlling the personal and economic burden of head and neck cancer.

Prognostic Factors Associated with Successful Salvage Surgery in Recurrent Oral Cancer

Most patients with recurrent oral cancer are not eligible for salvage surgery. Among those who are candidates for surgical salvage, failure rates are high. Given the potential negative impact of salvage surgery on quality of life (QoL)—particularly in unsuccessful interventions—the decision to operate must be weighed carefully. However, the variables associated with successful surgical salvage in oral cancer have not been clearly established. In the present retrospective study, we sought to determine the factors associated with disease recurrence and successful salvage surgery. We evaluated the following parameters in patients (n = 261) treated for primary oral cancer at our institution from 2010 to 2017: age; T/N status; perineurial invasion; lymphovascular invasion; extranodal extension; and margin status. In total, 36 patients (33%) were considered eligible for salvage surgery. Four variables were significantly associated with suitability for salvage surgery: early primary T stage, no primary neck disease (N0), no positive margins in the primary resection, and no adjuvant radiotherapy following primary resection. The only variable significantly associated with improved salvage outcomes was negative margin status after the primary tumor resection, underscoring the importance of margin status on treatment outcomes. Additional studies are needed to identify other factors associated with successful salvage surgery in order to better stratify patients according to the likelihood of success, thus potentially avoiding the negative impact on QoL in patients who undergo unsuccessful surgery.

Outcomes with definitive local treatment to the primary site in non-nasopharyngeal head and neck squamous cell carcinoma patients with synchronous distant metastasis.

e18014 Background: Data on the efficacy of including definitive local therapy to the primary head and neck disease (PHN) for non-nasopharyngeal head and neck squamous cell carcinoma (HNSCC) patients with synchronous distant metastasis are lacking. Methods: In this single institution retrospective study, we evaluated the outcomes of patients treated from 2000-2020 at UPMC for non-nasopharyngeal HNSCC with synchronous distant metastasis whose therapy included definitive therapy to the PHN. We evaluated overall survival (OS), calculated as date of diagnosis to date of death and progression free survival (PFS), calculated as date of diagnosis to date of death or progression. Based on an initial univariate analysis, the potential significant predictors were evaluated further in the multiple covariates Cox model via stepwise procedures. The relative mortality rates were summarized with hazard ratio (HR), with HR > 1.0 corresponding to increased mortality. Results: A total of 40 patients met inclusion criteria. The median age was 61, primary sites included 52.5% oropharynx (48% HPV +), 40% larynx/hypopharynx, 7.5% oral cavity, and 85% had a solitary metastatic lesion, most commonly in the lung. Definitive treatment of the PHN was with surgery (55%) or chemoradiation (45%), and 45% also underwent local treatment for all distant disease. The median PFS was 8.6 months (95% CI, 6.4-11.6), and OS was 14.2 months (95% CI, 10.9-27.5). In the 28% of patients that received induction therapy, there was a two-fold increase in median OS to 27.5 vs. 13.7 months, p = 0.06. In the 33% of patients that received anti-PD-1 mAb immunotherapy (IO), the median OS was significantly increased to 41.7 months (95% CI, 8.7-NR) vs. 12.1 months (95% CI, 8.4-14.4), p = 0.01, with a numeric increase in PFS as well (11.3 vs. 8.2 months respectively, p = 0.07). Notably no difference in PFS or OS was seen with type of local therapy to the PHN, receipt of local treatment to all distant disease, by HPV status, or year of diagnosis. In multivariate analysis including induction and other variables significant in univariate analysis (age, number of metastatic sites), IO was independently associated with improved OS (HR 3.123 (No IO vs. IO) (95% CI, 1.198-8.137), p = 0.02), as was age and number of metastatic sites. In the patients that received IO started as part of induction the median PFS and OS were 19.5 and 45.5 months respectively. Conclusions: We observed impressive survival in select non-nasopharyngeal HNSCC patients with synchronous distant metastasis treated with definitive local therapy to the primary head and neck disease in addition to induction and/or IO, with IO independently associated with improved OS. To our knowledge this is the first evaluation of the efficacy of definitive local therapy and IO in this population. Prospective evaluation is warranted.

Van Neck Disease: A Rare Case

Hyperostosis of the ischiopubic synchondrosis, van Neck disease, is a rare skeletal abnormality in children. Asymmetric and delayed closure of this cartilaginous joint is considered as a normal phenomenon. The symptomatic presentation of this pathology often mimics other closely related conditions like stress fracture, infection, or tumor. We are presenting a case of van Neck disease in the nondominant limb of an eleven-year-old boy.

Painful ophthalmoplegia due to involvement of cavernous sinus region by malignant neoplasm: report of three cases

Context: Intracranial tumor spread is an infrequent and late manifestation of head and neck cancers. We report three cases of painful ophthalmoplegia due to larynx and parotid neoplastic involvement. Data disclosure was authorized by the patients through an Informed Consent Form. Case reports: A 47-year-old man presents right retro- orbital pain and progressive ophthalmoplegia 5 months after resection of laryngeal spinocellular carcinoma and local radiotherapy. A 44-year-old man, 9 months after excision of spinocellular carcinoma of the larynx and subsequent radiotherapy, presents severe pain and paralysis of the left CN VI. Imaging exams showed involvement of CS. A 67-year-old woman with a tumoral mass in the left preauricular region. Biopsy revealed adenocarcinoma of the parotid gland. After total parotidectomy, the supra-omohyoid cervical ganglion was removed. Patient received radiotherapy for 3 months. Then, she presented a frontal and right temporal headache, more intense in the retro-orbital region. After one month, she developed complete CS syndrome, with the right CN VI being the first to be affected. MRI revealed an irregular enhancement lesion in right CS after contrast administration. All patients died despite treatment. Conclusions: In patients with painful ophthalmoplegia, the most common hypotheses are diabetic neuropathy and Tolosa-Hunt syndrome. CS involvement may be the first evidence of a distant head and neck disease. Despite the poor prognosis, palliative care should be considered.