Syndromic Piere Robbin Sequence- A Rare Presentation in Association with Multiple Heart Defects and Type III Stickler Syndrome.

Pierre Robin Sequence (PRS), a rare congenital disorder, is a triad of micrognathia, glossoptosis, and tongue based airway obstruction (TBSO). It may occur as isolated anomaly (iPRS) or as a part of a syndrome (sPRS), like that seen in association with Stickler Syndrome. Approximately 20% of children with PRS have congenital heart diseases. To the best of our knowledge this case of a one-day old infant is the first one to be reported as having two heart defects; patent ductus arteriosus and patent foramen ovale in Pierre Robbin Sequence child.

Implication of stem cells and platelet rich plasma in otolaryngology, head and neck surgical procedures.

Human body has a set of unspecialized cells called as stem cells that have the ability to generate cells of specialized function. Volume of fractionated plasma extracted from autologous blood is termed as Platelet-rich plasma (PRP) that is rich in several growth factors. Both have shown effective results in the field of regenerative medicine. Physiologically, platelets are the first cells to concentrate at the site of tissue damage, therefore application of PRP in diverse surgical procedures enhances bone and soft tissue healing; this same phenomenon is currently being used in otology, head and neck flap surgery and yielding miraculous outcomes. The perspective role of stem cells in regenerative medicine is wrapped in its loosely arranged DNA with working genes; a similar concept is being worked upon in different ENT procedures with groundbreaking results. But still, the data is scarce and there is a dire need for clinical trials, and large population-level studies to further formulate the guidelines on basis of proven evidence.

High-riding innominate artery in the neck: A hazardous presentation during surgery.

Aberrant innominate artery lying high in the neck is a rare entity that can be encountered intraoperatively during midline neck surgeries such as thyroidectomy and tracheostomy. Surgeons should be mindful of this entity as injury to the artery can lead to life-threatening haemorrhage. We report a case of a 40 year old female in whom an aberrant innominate artery was identified high in the neck, while performing a total thyroidectomy.

Fibroepithelial Polyp: A Rare Presentation In Sinonasal Cavity.

A fibroepithelial polyp is a benign polypoidal lesion of mesodermal origin. It can present in any area of the body with an epithelial surface. These are relatively more common in skin and genitourinary tract and has been reported very rarely in head and neck region. We report here a rare presentation of fibroepithelial polyp in sinonasal cavity presenting with the symptoms of unilateral nasal obstruction. The patient underwent endoscopic surgical excision and recovered completely. In patients with unilateral sinonasal growing masses fibroepithelial polyp should be considered as one of the possible diagnoses. Sinonasal fibroepithelial polyps have good prognosis after being treated surgically.

Treatment Outcomes of Maxillary Sinus Squamous Cell Carcinoma at a Dedicated Cancer Institute: A Retrospective Study.

Introduction Squamous cell carcinoma arising at the maxillary sinus is a rare neoplasm, characterized by an aggressive growth pattern and glooming prognosis. The proximity of the maxillary sinus with complex anatomical structures such as the eye, skull base, infratemporal fossa, pterygomaxillary fossa, nasal cavities, and ethmoid sinuses makes the surgical treatment of tumors infiltrating into these structures very challenging. The study's objective was to investigate the prognostic factors of survival and maxillary sinus SCC treatment outcomes. Methods We did a retrospective analysis of patients treated for maxillary sinus SCC at our institution between 2004 -2018. The study included all the patients with histologically proven maxillary sinus SCC treated with curative intent. The medical record of 43 patients was reviewed and utilized for the analysis. The Kaplan-Meier curve calculated five-year overall survival. Results A total of 43 patients were analyzed. At the presentation time, the mean age was 54.56 years (SD ± 11.65). Smoking (n=13, 30.2%) was the common risk factor. 36 (83.7%) patients presented with stage IV disease. Surgery was performed in 16 (37.2%) patients, whereas 27 (62.8%) patients received radiation and chemotherapy. Treatment failure was seen in 35 (81.4%) patients with locoregional recurrence in 30 (85.7%) patients and distant metastases in 5 (14.3%) patients. The five years overall survival in our study was 22%. Loco-regional recurrence and distant metastasis were the significant factors impacting survival (p=0.01). Conclusion Maxillary sinus SCC is rare cancer that is more common in males and usually presents at an advanced stage with a poor outcome. These tumors have a higher rate of treatment failure with a poor prognosis. Locoregional recurrence and distant metastasis adversely impact the overall survival.

Mammary analogue secretory carcinoma of parotid gland: Literature review and experience of a dedicated cancer institution in Pakistan.

Mammary Analogue Secretory Carcinoma (MASC) is a rare pathology of the salivary gland, most commonly involving the parotid gland. The objective of this study was to identify the characteristic features of MASC and its treatment outcomes. A retrospective review of 12 patients with histological diagnosis of MASC, who were managed between 2010 to 2019, was carried out at the Shaukat Khanum Memorial Cancer Hospital & Research Centre, Lahore. Their mean age was 34±16 years. There were 9 (75%) male and 3 (25%) female patients. Painless slow growing swelling was the most common presenting symptom. All the patients had undergone surgical excision with or without neck dissection, followed by adjuvant treatment. Of these, six patients had T2 tumours, while four had cervical lymph node metastasis. The mean follow-up period was 23±21 months. Local recurrence was seen in one patient. MASC is considered a low-grade tumour with good prognosis which can be treated with curative intent of surgery followed by radiotherapy effectively.

Ethical Dilemmas in the Management of Head and Neck Cancers in the Era of the COVID-19 Pandemic.

Coronavirus disease 2019 (COVID-19) has emerged as an unforeseen challenge for head and neck cancer care providers. A similar challenge is also faced by other oncological fields, but the severity of this challenge is highest in otolaryngology because of the need for additional precautionary measures and curbs on the possibility of aerosol forming interventions related to the upper aerodigestive tract. In this narrative review, provision of ethical and consistent care on moral and professional grounds to head and neck cancer patients during the pandemic are discussed for professionals who provide head and neck oncology care.

Depth of Invasion as a Predictor of Cervical Nodal Metastasis of Oral Tongue Squamous Cell Carcinoma: Findings From a Tertiary Care Center in Pakistan.

Background and objective Cervical lymph node metastasis has a significant impact on the survival of patients with oral cavity tumors. The rate of occult neck node metastasis is reported to range from 20 to 40%. The depth of invasion (DOI) has been incorporated in the eighth edition of the American Joint Commission on Cancer (AJCC) staging manual and is an important predictor of cervical lymph node metastasis. In this study, we aimed to identify the occult neck node metastasis rate in early oral tongue squamous cell carcinoma (OTSCC) and correlate it with the DOI. Methods A retrospective review of all patients presenting to our facility with early-stage OTSCC was performed. Patients with tumor size of ≤4 cm and who underwent elective neck dissection at the time of surgery were included. The study outcomes were the rates of occult neck metastases in T1 and T2 OTSCC and their correlation with the DOI. Results There were 80 patients in total. Occult neck node metastases were seen in 29 (36.25%) patients. Patients with a DOI >5 mm were 1.41 times more likely to have occult neck node metastasis than those with a DOI ≤5 mm. Conclusion Occult neck node metastasis is significantly associated with the DOI. The risk of neck metastasis is higher in patients with a DOI >5 mm.

Differentiated Thyroid Carcinoma: Distant Metastasis as an Unusual Sole Initial Manifestation.

OBJECTIVE: The objective of this study was to identify the characteristic features of patients with distant metastasis as the only manifestation of well-differentiated thyroid cancers and to analyze the treatment outcomes. METHODS: A retrospective review of all patients with well-differentiated thyroid cancers and distant metastasis as the sole initial presentation was carried out. Data regarding age, gender, tumor histology, site, symptoms, and treatment outcomes were collected. RESULTS: There were 10 patients who presented with distant metastasis as the only presentation. The mean age was 56.1 years. Eight (80%) patients had osseous metastasis, one (10%) had pulmonary and one (10%) had both. Follicular thyroid carcinoma was more common and seen in six (60%) patients. Seven (77.8%) out of nine patients had demised within five years of initial presentation. CONCLUSION: Distant metastases without a neck lump as the initial presentation of well-differentiated thyroid cancers are extremely rare. No specific guidelines are available to manage such patients due to lack of relevant data in the literature.

Outcomes of Management of Sinonasal Malignancies at a Dedicated Cancer Institution: A Retrospective Study.

INTRODUCTION: Sinonasal malignancies (SNMs) are a rare and heterogeneous group of cancers with an incidence of 3%-5% of all head-and-neck tumours. The aim of the present study was to identify the characteristic features of SNMs and to analyze the treatment outcomes and the prognostic factors affecting it. MATERIALS AND METHODS: A retrospective review of patients with SNM treated at a dedicated cancer center of Pakistan between 2004 and 2018 was carried out. All patients with histologically proven diagnosis of malignancy arising from nasal cavity or paranasal sinuses and being treated with curative intent were included. The medical records of 184 cases were utilized for final analysis. Chi-square test was applied to identify significant differences. Five-year disease-specific survival (DSS) was calculated using Kaplan-Meier curve via log-rank test and comparison was made between squamous and nonsquamous histologies. P ≤0.05 was considered statistically significant. RESULTS: Squamous cell carcinoma was the most common histopathology (n = 62, 33.7%). 130 (70.7%) patients presented with stage IV disease. Nodal metastasis was seen in 24 (13%) patients. Treatment failure was seen in 96 (52.2%) patients and was significantly associated with tumour size and the overall stage. DSS of patients with squamous cell carcinoma was 29% as compared to 52% for patients with nonsquamous histologies (P = 0.001). DISCUSSION: SNM is a diverse group of cancers presenting in late stages. A higher rate of treatment failure associated significantly with stage of the disease was identified in our study.

The Efficacy of Bethesda System for Prediction of Thyroid Malignancies- A 9 Year Experience from a Tertiary Center.

INTRODUCTION: The best initial investigation for thyroid nodule is fine needle aspiration (FNA). Bethesda System is an international standardized system of reporting thyroid nodules and recommends subsequent management. Every institution should assess the risk of malignancy in each category to avoid unnecessary thyroid surgeries, with this aim we conducted a review at our center to calculate risk of malignancy in each category. MATERIALS AND METHODS: Retrospective 9-year (2009-2018) review of thyroid FNAs done at a tertiary care Centre. The FNA was stratified according to The Bethesda System. Histopathology reports of the operated cases were used to evaluate the cytology for diagnostic accuracy. RESULTS: There were 495 patients who underwent thyroidectomy. The mean age of the cohort was 42.51 +/- 13.2 years and 387 (78.2%) were females. The frequency of Bethesda categories I, II, III, IV, V, and VI were 9.1%, 55.6%, 16.4%, 6.5%, 9.3%, and 3.2% respectively. Malignancy rate in operated thyroid nodules were 37.8%, 8.4%, 33.3%, 50.0%, 89.1%, and 100% for Bethesda categories I to VI, respectively. The sensitivity, specificity, negative predictive value and positive predictive value and their 95% CIs were calculated as 81.30 (73.28 - 87.76%), 77.06 (72.12 - 81.51%), 91.64 (88.3 - 94.1%) and 57.14 (51.79 - 62.33%). The overall diagnostic accuracy was 78.22 (74.12 - 81.95%). CONCLUSIONS: All the Bethesda categories showed greater malignancy risks than other reported studies. Knowledge of local rates of malignancy is important to accurately predict the risk of malignancy even when reported with internationally accepted nomenclature like the Bethesda System.

Synchronous primary diffuse large B-cell lymphoma and papillary thyroid carcinoma: A case report highlighting "Lymphoma first approach".

Papillary thyroid carcinoma (PTC) is the most common type of all thyroid carcinomas and accounts for up to 90% of all thyroid malignancies. It is the most indolent form of the disease and has an excellent prognosis. On the other hand, Primary thyroid lymphoma (PTL) is a rare entity accounting for only about 1% to 5% of all thyroid malignancies and only 2% of extranodal lymphomas. Synchronous PTC and PTL is a very rare condition and only a few cases have been reported in the literature up till now. We report one such case in a 52-year-old lady who was referred to us with goiter and progressively increasing difficulty in breathing.

Prevalence of allergic fungal sinusitis among patients with nasal polyposis.

OBJECTIVE: To determine the frequency of allergic fungal sinusitis among patients with nasal polyps, and to compare Lund-Mackay scores of patients with and without allergic fungal sinusitis. METHODS: The cross-sectional longitudinal study was conducted at a tertiary healthcare centre in Karachi, Pakistan, from December 2016 to November 2018, and comprised patients with sinonasal polyposis undergoing surgery. The patients were categorised as having allergic fungal sinusitis when histopathology showed allergic mucin with fungal hyphae and culture was positive for fungal growth. Lund-Mackay scoring of each patient was noted and mean scores of allergic fungal sinusitis and non-allergic fungal sinusitis patients were compared. Data was analysed using SPSS 25. RESULTS: Of the 114 patients, 61(53.5%) were males. The overall mean age was 37.3±15.3 years. Of the total, 27(23.7%) patients had allergic fungal sinusitis. There was a significant relationship between asthma and allergic fungal sinusitis (p=0.03). The mean Lund-Mackay score was significantly higher for allergic fungal sinusitis patients (p<0.01). Recurrence was seen in 11(9.6%) cases. CONCLUSIONS: About one-fourth of patients with nasal polyps had allergic fungal sinusitis, and such patients showed significantly high mean Lund-Mackay score.

Level I Nodal Positivity as a Factor for Involvement of the Submandibular Gland in Oral Cavity Carcinoma: A Case Series Report.

Introduction The routine practice of neck dissection in the surgical management of oral carcinoma has evolved into a more functionally conservative approach. Over time, the rationale for removal of the submandibular gland has been questioned. Routine extirpation of the submandibular gland can aggravate the xerostomia experienced by many patients, significantly affecting their quality of life. Objective The objective of the present study was to determine the incidence of submandibular gland metastases in oral cavity carcinoma and to identify possible factors that may affect their involvement. Methods A total of 149 cases of oral carcinoma presenting at a private tertiary care hospital in Karachi, Pakistan, over the course of 1 year were reviewed retrospectively. Results Histopathological data showed that the submandibular gland was involved in 7 (4.7%) cases. Involvement of level I lymph nodes was found in all of the cases. Direct extension of primary tumor was noted in two cases when the primary tumor was in the floor of the mouth. Conclusion The results suggest that preservation of the submandibular gland during neck dissection for oral carcinoma can be practiced safely when there is no evidence of direct extension of the primary tumor toward the submandibular gland or when there is no clinical or radiological evidence of neck disease in level I. Presence of pathological lymph nodes in level I requires caution when contemplating preservation of the submandibular gland.

Bronchogenic Cyst in the Cervical Region: A Rare Entity - A Case Report and Review of the Literature.

Bronchogenic cysts originate from the tracheobronchial bud, which arises from the embryonic foregut. Congenital bronchogenic cysts in the cervical region, especially in the thyroid or perithyroidal area, are extremely rare. Moreover, distinguishing them from other cervical cystic lesions such as thyroglossal duct and branchial cleft cysts and metastatic cervical lymph nodes is difficult preoperatively. In this report, we discuss a case of a 41-year-old woman who presented to us with a history of anterior neck swelling for two weeks with occasional palpitations and bilateral flank pain. On workup, she was diagnosed as a case of multiple neuroendocrine neoplasm type 2A for which she underwent adrenalectomy first followed by total thyroidectomy with central neck dissection and parathyroidectomy. On the final histopathology specimen, an incidental bronchogenic cyst was diagnosed. A bronchogenic cyst is a rare entity, especially in the head and neck region, and can be confused with a metastatic lymph node. Diagnosis is made based on the histopathological examination, which requires surgical excision. The bronchogenic cyst should be considered in the differential diagnosis for midline and lateral neck masses.

Level I Nodal Positivity as a Factor for Involvement of the Submandibular Gland in Oral Cavity Carcinoma: A Case Series Report

Abstract Introduction The routine practice of neck dissection in the surgical management of oral carcinoma has evolved into a more functionally conservative approach. Over time, the rationale for removal of the submandibular gland has been questioned. Routine extirpation of the submandibular gland can aggravate the xerostomia experienced by many patients, significantly affecting their quality of life. Objective The objective of the present study was to determine the incidence of submandibular gland metastases in oral cavity carcinoma and to identify possible factors that may affect their involvement. Methods A total of 149 cases of oral carcinoma presenting at a private tertiary care hospital in Karachi, Pakistan, over the course of 1 year were reviewed retrospectively. Results Histopathological data showed that the submandibular gland was involved in 7 (4.7%) cases. Involvement of level I lymph nodes was found in all of the cases. Direct extension of primary tumor was noted in two cases when the primary tumor was in the floor of the mouth. Conclusion The results suggest that preservation of the submandibular gland during neck dissection for oral carcinoma can be practiced safely when there is no evidence of direct extension of the primary tumor toward the submandibular gland or when there is no clinical or radiological evidence of neck disease in level I. Presence of pathological lymph nodes in level I requires caution when contemplating preservation of the submandibular gland.

Prognostic outcomes of treatment naïve oral tongue squamous cell carcinoma (OTSCC): a comprehensive analysis of 14 years.

OBJECTIVES: To analyze the factors predicting survival outcomes in treatment naïve oral tongue squamous cell carcinoma (OTSCC). MATERIALS AND METHODS: A comprehensive review of 531 oral tongue carcinoma patients treated with upfront surgery followed by adjuvant radiotherapy or chemoradiotherapy was conducted from 2004-2018. RESULTS: The mean age of presentation was 53 years (11-86) with a male to female ratio of 1.3:1. The associated risk factors were smoking (21%), betel nut (16%), naswar (9%) and alcohol (1%). Most of the cases were either well (45.1%) or moderately (46.2%) differentiated. Surgery was performed in 164 patients alone while 368 were treated with surgery in combination with adjuvant modalities. Overall (OS) and disease free survival (DFS) were 66 and 71%, respectively, with a median follow up of 2.5 years. Cox regression analysis showed nodal positivity, increased depth of invasion (DOI) and higher lymph node ratio (LNR) as significant prognosticators impacting OS and DSS. CONCLUSION: Nodal volume, DOI and LNR are the most consistent predictors of poor outcome in OTSCC. Nodal positivity, depth of invasion > 5 mm and lymph node ratio > 0.04 adversely affect OS and DSS.

Role of Fine Needle Aspiration Cytology in the Diagnosis of Parotid Gland Tumors: Analysis of 193 Cases

Abstract Introduction Parotid gland tumors include a wide variety of inflammatory and neoplastic diseases. The majority of these tumors are benign (80%), which usually require superficial parotidectomy, while the incidence of malignant tumors is lower (20%), requiring more radical surgery with or without neck dissection. The diagnosis cannot be established on the basis of clinical history and simple physical examination and requires complementary diagnostic methods. Fine needle aspiration cytology (FNAC) guided by ultrasound is a widely used diagnostic tool to evaluate parotid swellings. Objective To determine the sensitivity, specificity, positive and negative predictive values and diagnostic accuracy of FNAC in the diagnosis of parotid gland tumors. Methods A retrospective chart review of 193 patients who underwent preoperative FNAC and parotidectomy at the Aga Khan University Hospital, Karachi, Pakistan, from the period of January 2000 to December 2015 was performed. Results Out of 193 patients undergoing parotidectomy, 110 (57%) were males and 83 (43%) were females, the mean age being 48.21 and 43.76 years old, respectively. The mean duration of the symptoms was 41.33 months, and the most common symptom was preauricular swelling present in all patients, followed by pain, which was present in 29 patients (15%) and facial nerve weakness in 6 patients (3.1%). Fine needle aspiration cytology was performed preoperatively and the results were compared with the final histopathology, which showed sensitivity of 88.9%, specificity of 97.9%, positive predictive value of 93%, negative predictive value of 96.7% and diagnostic accuracy of 95.8%. Conclusion Our results suggest that FNAC is relatively an accurate method for preoperative diagnosis of parotid swelling and can prove to be a valuable tool for preoperative counseling of the nature of the disease and prognosis.