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Fibrous dysplasia (FD), commonly known as Lichtenstein-Jaffe disease, is a benign fibro-osseous bone disease. Clinical symptoms often include bone pain, deformities, pathological fractures, or nerve compression. Fibrous dysplasia (FD) in the cranio-facial region presents major management concerns because to the risk of deformity, loss of function, and recurrence. The purpose of this study is to demonstrate a single institution's experience managing cranio-facial FD with zolendronic acid, as well as an extensive review of the available literature on the subject. This retrospective study was conducted in the Orthopedic Oncology unit of the Department of Surgical Oncology with a study duration between January 1, 2019, and January 1, 2022. There were seven patients with cranio-facial fibrous dysplasia in the current study. The effects of zolendronic acid were evaluated using clinical assessment, data, radiological findings, biochemical indicators. Furthermore, a comprehensive literature review was conducted in order to compile the current data of cranio-facial FD. The study included seven individuals (five men and two females), four with polyostotic FD and three with cranio-facial FD. The average follow-up duration was 18.75 months. The study found that all parameters improved: the mean VAS score increased from 7 to 1, mean serum calcium levels increased from 8.75 to 8.46 mg/dL, mean serum phosphorus levels increased from 4.46 to 4.17 mg/dL, and serum alkaline phosphatase levels increased from 152 to 93.25 IU/L.A comprehensive literature review was conducted using PubMed and Google Scholar with the following keywords: "Fibrous dysplasia," "Cranio-facial bones," "Bisphosphonate," and "Zolendronic acid." The search included 24 studies published between 2000 and 2022, incorporating all relevant studies available to date. Our study demonstrated the effectiveness of zolendronic acid in the treatment of cranio-facial fibrous dysplasia. Zolendronic acid offers potential as a feasible treatment options in treating cranio-facial FD, with possible advantages including alleviating symptoms, disease progression stabilisation, and morbidity reduction. However, multi-centre prospective randomised study with larger sample numbers and longer follow-up periods are needed in future.
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The incidence of synchronous second primary cancer (SSPC) with DIfferentiated Thyroid Cancers (DTC) is a very rare entity. Very few case reports and case series were published in the literature. To enumerate the clinicopathological data of patients with DTC with SSPC. This is a single-center retrospective study. All the patients diagnosed with DTC and SSPC from January 2016 to July 2023 were included in the study. The demographic, clinicopathological data, and survival data were collected from the institute's database. Twelve patients were found to have DTC with SSPC. All the patients had papillary thyroid cancer. Ten patients had head and neck squamous cell carcinoma, one patient had malignant phyllodes tumor, and another patient had endometrioid adenocarcinoma as the SSPC. Six patients were diagnosed during preoperative evaluation, one patient was diagnosed 3 months after the oral malignancy diagnosis, and five patients were diagnosed with PTC in cervical nodes after neck dissection. Complete thyroid removal was done in six patients and hemithyroidectomy in two patients. The median follow-up was 25.55 months. The median overall survival was 40.97 months. Head and neck SCC is the most common SSPC with DTC. Since these are rare scenarios multi-disciplinary panel discussion can help in deciding management.
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BACKGROUND: The aim of this study is to report the early outcomes of valvular heart surgeries performed via the right thoracotomy approach. While thoracotomy with femoro-femoral bypass is an established method for minimally invasive open-heart surgeries, thoracotomy with conventional cannulation is still being explored. In our center, we conducted 958 valvular heart surgery cases using the right anterolateral thoracotomy approach with central cannulation and data were analyzed. METHODS: This is a retrospective observational study based on prospectively collected data from patients who underwent valvular heart surgery at our center spanning from April 2013 to April 2023. The data encompass demographics, procedures, operative techniques, post-operative morbidity, mortality, and a 1-month follow-up. RESULTS: Our study revealed no procedure-related mortality. No patient required conversion to median sternotomy. Smooth cannulation and satisfactory exposure were achieved in all patients. The study encompassed a wide age range, from 14 to 68 years, with 618 female patients (64.5%) and 340 male patients (35.5%). The average cross-clamp time ranged from 38 to 90 min, the duration of cardio-pulmonary bypass ranged from 45 to 105 min, post-operative extubation ranged from 3 to 8 h, the average drain volume ranged from 100 to 350 ml, and the incision size ranged from 5 to 7 cm. CONCLUSIONS: Our data demonstrate that conventional cannulation via the right antero-lateral thoracotomy approach for valvular heart disease is a viable alternative to reduce the side effects associated with sternotomy and femoral cannulation. This procedure is safe, reproducible, and provides the same level of treatment quality.
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Toracotomia , Humanos , Toracotomia/métodos , Feminino , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Idoso , Adulto , Adolescente , Adulto Jovem , Doenças das Valvas Cardíacas/cirurgia , Cateterismo/métodosRESUMO
Lipoleiomyosarcoma is a malignant tumor of both mesenchymal and smooth muscle origin. Lipoleiomyosarcoma is a very rare type of retroperitoneal sarcoma, which by itself is a rare type of soft tissue sarcoma. Lipoleiomyosarcoma is a very rare subtype of liposarcoma, which is the most common type of retroperitoneal sarcoma. To our best review of available literature, this was the first case of lipoleiomyosarcoma presenting as a massive retroperitoneal mass of size 50 × 50 × 30 cm, managed with multi-visceral R0 resection. The computed tomography of the abdomen showed a massive fat density lesion with internal soft density lesions and internal calcifications. Multivisceral resection was planned and executed and achieved R0 resection. We suggest that lipoleiomyosarcoma should be included in differential diagnosis of retroperitoneal tumors, and multi-visceral R0 resection should be considered as an option for lipoleiomyosarcoma, as these tumors have very good prognosis with R0 resection.
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PURPOSE: Femur shaft fractures commonly occur due to high velocity trauma and most of them are fixed with nailing. Malrotation is common after fixation. A rotational malalignment more than 30° is a deformity which requires correction. Various techniques described for rotational deformities are: the 'cable techniques' for the determination of varus-valgus malalignment; the 'hyperextension test', 'radiographic recurvatum sign', 'tibial plateau sign', and 'meterstick technique' for length analysis; and the 'hip rotation test', 'lesser trochanter shape sign', 'cortical step sign', and 'diameter difference sign' for rotational analysis. We describe integration sign at the medial or lateral aspect of notch of femur in fixed internal or external rotation due to condensation of trabeculae. METHODS: This is a prospective observational study. Informed consent was taken, and this study was approved by institutional review board. C arm imaging study of 50 knees was done at our institution in which rotational profile of distal femur was analyzed In 3 different views -AP , Internal and external rotation views. The rotation views were taken sequentially and each observer was asked to identify the sign at its appearance and at rotation when it was best seen. All the observers were asked to draw the sign on linear line diagram of distal femur. The data was analysed statistically. RESULTS: Sign of integral (∫) for rotational deformity was seen at Mean external rotation of 22±1.71 with a range of 19 degree to 25 degree and Mean internal rotation of 15.78±1.21 with a range of 14 degree to 18 degree. CONCLUSION: The integration sign can be used as intraoperative C arm sign to understand the rotational deformity of distal fragment of femur. This helps the surgeon to understand the alignment and revise if needed intraoperatively. If combined with position of lesser trochanter, this will give alignment for both proximal as well as distal fragment of femur.
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Fraturas do Fêmur , Fêmur , Humanos , Fraturas do Fêmur/diagnóstico por imagem , Fraturas do Fêmur/cirurgia , Estudos Prospectivos , Rotação , Fêmur/diagnóstico por imagem , Fêmur/anormalidades , Mau Alinhamento Ósseo/diagnóstico por imagem , Feminino , Masculino , AdultoRESUMO
Head and neck cancer is a significant public health concern in India and globally, with substantial social and economic consequences for affected individuals and their families. The study aimed to assess the socioeconomic impact of head and neck cancer. This paper presents the results of a questionnaire-based study involving 178 head and neck cancer patients who were evaluated at least two years post-completion of their treatment. The questionnaire data collected data on various factors, including site of cancer, treatment modality, speech and diet impairment, changes in earning capacity, occupation, and salary. This was collected from all patients visiting our tertiary cancer care center outpatient department in Ahmedabad, India, between January 2023 and August 2023. The findings highlight the diverse and profound socioeconomic consequences of head and neck cancer. The findings emphasize the need for comprehensive support systems for affected individuals and their families.
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Introduction: Minimal Access Surgery (MAS) has shown better peri-operative outcomes with equivalent oncological outcomes in gastrointestinal and thoracic oncology. Open CRS (O-CRS) procedure accompanies inevitable and significant surgical morbidity in patients. The aim of the review article is to compare outcomes of M-CRS and HIPEC/EPIC with open procedure in peritoneal surface malignancies. Content: Comprehensive search of databases was done and total 2,807 articles were found (2793-PubMed and 14-Cochrane review). PRISMA flow chart was prepared and 14 articles were selected. Meta-analysis was performed according to PRISMA guidelines using random-effects model (DerSimonian Laird) and fixed effect model. Publication bias was tested with Funnel plot and Egger's regression test. Quality of studies was assessed by Newcastle-Ottawa scale. Summary and Outlook: Patients in both groups [total (732), M-CRS(319), O-CRS(413)] were similar in demographic characteristics. Peri-operative outcomes were significantly better in M-CRS group in terms of blood loss SMD=-2.379, p<0.001 (95â¯% CI -2.952 to -1.805), blood transfusion RR=0.598, p=0.011 (95â¯% CI 0.402 to 0.889), bowel recovery SMD=-0.843, p=0.01 (95â¯% CI -1.487 to -0.2), hospital stay SMD=-2.348, p<0.001 (95â¯% CI -3.178 to -1.519) and total morbidity RR=0.538, p<0.001 (95â¯% CI 0.395 to 0.731). Duration of surgery SMD=-0.0643 (95â¯% CI -0.993 to 0.865, p=0.892) and CC0 score RR=1.064 (95â¯% CI 0.992 to 1.140, p=0.083) had no significant difference. Limited studies which evaluated survival showed similar outcomes. This meta-analysis shows that M-CRS and HIPEC/EPIC is feasible and has better peri-operative outcomes compared to open procedure in patients with limited peritoneal carcinoma index (PCI) peritoneal surface malignancies. Survival outcomes were not calculated. Further studies are warranted in this regard.
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Introduction: Giant Cell Tumours (GCT) are benign tumours with aggressive potential that disrupt the local bony architecture, which can be especially problematic in peri-articular locations. Our aim was to assess the outcomes of patients with GCT of the distal ulna who were treated by resection without reconstruction. Methods: The study included 21 patients with distal ulna GCT that were treated with resection without reconstruction. There were 12 males and 9 females, with a mean age of 30.4years (range 14-45 years). The patients mean follow-up period was 4.4 years, with a two-year minimum follow-up. Results: Painful swelling was the presenting symtom in all cases. Nineteen patients had Campanacci grade 3 and two had Campanacci grade 2. The mean resected length of the distal ulna was 6.8 cm (range 4-10) cm. The Musculoskeletal Tumor Society score (MSTS) was 26.1. (range 22-28). Grip strength of the affected hand was reduced by 10.5% on average. (range 0%-16%). Two patients were having multi-centric disease on presentation and none of the cases had pathological fracture on presentation. One case had a local recurrence which was treated with surgery. Conclusion: Based on current study, GCT of the distal ulna, en bloc resection without reconstruction can be recommended as a valuable treatment option for Campanacci grades 2 and 3 tumours. Resection of the distal end of the ulna without reconstruction results in excellent functional outcomes, with forearm rotational movement and hand function preserved. According to review of literature this is the largest series of GCT Ulna and we recommend a multicentre and comparitive studies on this topic.
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Differentiated Thyroid carcinoma (DTC) with distant skeletal metastases is associated with a very poor prognosis and are unfortunately resistant to radioiodine therapy (RIT). Surgical removal of the metastases in such selected cases is a beneficial adjunct to RIT. We report two cases of DTC with sternal metastases whom we successfully managed with surgical resection of the sternal lesion with reconstruction of the chest wall defect using titanium mesh implant and myocutaneous flap.
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Head and neck osteosarcoma is an uncommon yet aggressive tumor which presents therapeutic challenges to get favourable results. Surgery remained the most effective treatment modality in this entity eventhough chemoradiotherapy have been tried in various studies for better outcome but still not yet becomes the standard in the management of these cases unlike in extremity osteosarcoma. We present our experience in the management of this uncommon yet lethal malignant tumor, i.e. head and neck osteosarcoma. To study the clinicopathological and prognostic features of Osteosarcoma in head and neck subsite. Retrospective study of patients diagnosed with head and neck osteosarcoma between 2003 and 2019. Total of 25 patients were included in our study. Mean age of our population is 27.5 years with slight male predominant. Mandible is the most commonly involved site. Multimodal treatment applied with surgical resection forms the main part in the management. Median DFS and OS were 16 and 36 months respectively with 5 year overall survival of 42%. Out of the various factors studied, absence of surgery, margin positivity are the principle features affecting the prognosis. Head and neck osteosarcoma is generally a jaw bone tumor commonly occurs in young adults with poor outcome. Since there is no universal guidelines to address this uncommon tumor, multiple studies have shown various results in the management. Till date, surgery remained the curative modality with mixed response on the role of chemotherapy and radiotherapy.
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Neoadjuvant chemoradiation followed by surgery has been the standard of care for locally advanced carcinoma esophagus. We present our experience and inference of various factors associated with the same treatment and the prognostic influence of the same. A retrospective analysis of a cohort of 132 squamous cell carcinoma esophagus patients post neoadjuvant chemoradiation operated with curative intent was carried out. The 2-year overall survival rate was 64.5%. A pathological complete response was achieved in 32.5% of patients and was the only factor that significantly determined overall survival (p = 0.048). Neoadjuvant chemoradiation before surgery for locally advanced squamous cell cancer of the esophagus remains the standard of care with a pathological complete response being a significant factor in predicting overall survival. More prospective randomized studies are necessary to analyze factors affecting and predicting a pathological complete response which would help organ preservation in patients with a complete response.
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BACKGROUND: Oral submucous fibrosis (OSF) is a pathological condition characterized by excessive tissue healing resulting from physical, chemical, or mechanical trauma. Notably, areca nut consumption significantly contributes to the development of oral fibrosis. The current definition of OSF, recognizing its potential for malignant transformation, necessitates a more comprehensive understanding of its pathophysiology and etiology. HIGHLIGHTS: Areca nut induces fibrotic pathways by upregulating inflammatory cytokines such as TGF-ß and expressing additional cytokines. Moreover, it triggers the conversion of fibroblasts to myofibroblasts, characterized by α-SMA and γSMA expression, resulting in accelerated collagen production. Arecoline, a component of areca nut, has been shown to elevate levels of reactive oxygen species, upregulate the expression of various cytokines, and activate specific signaling pathways (MEK, COX2, PI3K), all contributing to fibrosis. Therefore, we propose redefining OSF as "Areca nut-induced oral fibrosis" (AIOF) to align with current epistemology, emphasizing its distinctive association with areca nut consumption. The refined definition enhances our ability to develop targeted interventions, thus contributing to more effective prevention and treatment strategies for oral submucous fibrosis worldwide. CONCLUSION: Arecoline plays a crucial role as a mediator in fibrosis development, contributing to extracellular matrix accumulation in OSF. The re-evaluation of OSF as AIOF offers a more accurate representation of the condition. This nuanced perspective is essential for distinguishing AIOF from other forms of oral fibrosis and advancing our understanding of the disease's pathophysiology.
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Areca , Arecolina , Fibrose Oral Submucosa , Fibrose Oral Submucosa/patologia , Fibrose Oral Submucosa/etiologia , Fibrose Oral Submucosa/metabolismo , Humanos , Areca/efeitos adversos , Arecolina/efeitos adversos , Citocinas/metabolismo , Transdução de Sinais , Nozes/efeitos adversosRESUMO
Coronary artery disease (CAD) and peripheral vascular disease (PVD) often coexist and are prevalent due to population ageing, smoking, diabetes, unhealthy lifestyles, and the epidemic of obesity. In high-risk patients, it is critical to minimize the overall burden of surgery to avoid poor outcomes and morbidity. Here, we present a case of successful coronary artery bypass grafting (CABG) with thoraco-bifemoral bypass surgery for PVD via a left thoracotomy approach. Traditionally, median sternotomy is done for these kinds of surgeries. However, we preferred thoracotomy over sternotomy to avoid morbidity in old age. Our case suggests that combined CABG with thoraco-bifemoral bypass via thoracotomy approach is a reliable surgical option depending on the anatomy of the lesion.
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Background: Oral submucous fibrosis (OSF) is a persistent oral mucosal condition that carries an elevated risk of undergoing malignant transformation. Our objective was to elucidate the involvement of epithelial-to-mesenchymal transition (EMT) in OSF and its progression to malignancy by studying a panel of EMT markers, thereby understanding the molecular mechanisms. Methods: An immunohistochemical analysis was done to detect the presence of E-cadherin, N-cadherin, pan-cytokeratin (PanCK), vimentin, α-SMA (alpha-smooth muscle actin), and CD44 in a total of 100 tissue samples. These samples comprised 40 cases of OSF, 20 cases of oral squamous cell carcinoma associated with OSF (OSFSCC), and 40 cases of oral squamous cell carcinoma (OSCC). A whole transcriptomic analysis was performed on a group of seven matched samples encompassing NOM, OSF, OSFSCC, and OSCC. Results: We observed significantly decreased expression of E-cadherin and PanCK, while N-cadherin, vimentin, α-SMA, and CD44 showed significantly higher expression in OSFSCC and OSCC as compared to OSF, both at protein and RNA levels. CD44 expression was noticeably higher in OSFSCC (p < 0.001) than in OSCC. Conclusion: Downregulation of epithelial markers with concomitant upregulation of mesenchymal and stem cell markers suggests the potential role of EMT and stemness in accelerating the pathogenesis and malignant transformation of OSF. The high levels of CD44 expression seen in OSFSCC indicate a high propensity for aggressiveness and acquisition of stem-like characteristics by the cells undergoing EMT.
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Purpose: Lung cancer is one of the most common cancers in India. However, less than half receive treatment with a curative intent and very few undergo surgery amongst them. We present our surgical experience with non-small cell lung cancer. Methods: A retrospective analysis of a cohort of 92 non-small cell lung cancer patients operated with curative intent. Results: Less than 2% patients of lung cancer were operated on at our centre. Adenocarcinoma was the most common histological subtype. Right upper lobectomy was the most common surgery performed. Two- and 3-year overall survival was 74.3% and 70.6% respectively. Two- and 3- year disease-free survival was 65.4% and 60.8% respectively. Conclusion: The fraction of patients who are operated for lung cancer is very less. There is a definite missed window of opportunity. We have comparable survival to international data.
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Lip and oral cavity SCC account for 2nd highest incidence of cancers and 3rd most common cause of mortality from cancer in India. Reconstruction of defects of central arch invading cancers results in poor cosmetic and functional outcomes if free flaps are not used. 30 patients with Oral SCC in the age group 20-75 years requiring central arch segmental mandibulectomy were included. Reconstruction was done with pedicled bipaddled PMMC flap with 'AJ's orbicularis oris stitch' using Fiber wire. Patients were divided into 4 groups according to extent of lip and skin loss post excision of primary tumour. Patients were evaluated with subjective scores for drooling, oral competence and cosmesis. There were 4, 12, 9 and 5 patients in Group A, B, C and D respectively. Mean subjective scores using our technique for drooling, oral competence and cosmesis were 3.75/4,3.75/4 and 3.5/4 for group A, 3.45/4, 3.36/4 and 3.09/4 for group B, 2.8/4, 2.6/4 and 2.3/4 for group C defects and 2.5/4, 3/4 and 2.5/4 for group D defects respectively. Over all scores for all patients were 3.2/4, 3.14/4 and 2.84/4 for drooling, oral competence and cosmesis. This simple, quick and inexpensive technique of reconstruction of central mandibular arch defects can drastically improve cosmetic and functional outcomes in a resource restrained set up. However, long term results and comparison studies are required for standardisation of the technique.
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Malignant melanoma is an aggressive malignancy of melanocytes which is usually found on sun exposed areas of the body. A rare variant of this disease with no etiological association is the mucosal malignant melanoma found on all mucosal surfaces of the body including the oral cavity, respiratory mucosa and anorectal region. In the head and neck region, this disease is almost always diagnosed at an advanced stage and requires a very high index of suspicion for diagnosis. It is more commonly found in females than males.Indians are more prone to this disease as compared to Caucasians.Due to the obscure location within the oral and nasal cavity, it is clinically found at an advanced stage and requires surgical resection with adequate margins for complete eradication. This may be achieved either endoscopically in the nasal cavity or with wide local resection in the oral cavity. this in certain cases may not be feasible due to vicinity of vital structures. In such cases, adjuvant radiotherapy helps in the local control of disease. Histopathological evaluation of the specimen helps to determine aggressive biology of tumor with factors such as presence of ulceration, nodular morphology and perineural invasion being high risk features for development of local and regional recurrence. Supplementary Information: The online version contains supplementary material available at 10.1007/s12070-023-04001-y.
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Medullary thyroid carcinoma is a rare tumour that is anatomically located in the thyroid gland but is functionally a neuroendocrine tumour. It is usually a disease of older age group but manifests in a young patient in familial form. It is derived from parafollicular c cells and has a predilection for lymph node metastasis. It is associated with slow growth in thyroid gland with early nodal metastasis. Serum calcitonin is useful as a preoperative marker of disease burden and prognosis. In the preoperative period serum levels of calcitonin can guide regarding the need for compartment wise lymph node dissection and the possibility of distant metastasis. It is used as a tool of surveillance in the postoperative period. The levels of serum CEA and calcitonin and their doubling time is a useful guide in the detection of early recurrence or distant metastasis. Imaging modality useful for diagnosis is USG in a majority of patients. Thus, the initial diagnosis and preoperative assessment of medullary thyroid carcinoma is similar to other forms of thyroid cancer but further management of disease differs significantly form other forms of differentiated thyroid carcinoma or even anaplastic carcinoma. Prognosis however differs according to age, gender, presence or absence of lymph node metastasis at presentation, metastatic disease at presentation and levels of biochemical markers.
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Diagnostic dilemma, owing to the inconclusiveness of biopsy results, often leaves us with limited options to offer to the patients upfront, amongst the various armamentarium available. We hereby report a rare case of extramedullary plasmacytoma, whose diagnosis was established only on the final histopathology report with the aid of immunohistochemistry. A 50-year-old gentleman presented to our outpatient setup with computed tomography suggestive of a well-defined endobronchial mass occupying the left lower lobe bronchus. However, bronchoscopy and computed tomography-guided biopsies were inconclusive. After a routine metastatic workup, the patient underwent a left lower lobectomy following a provisional diagnosis of carcinoid on the frozen section. The final histopathology was solitary endobronchial plasmacytoma. Postoperative myeloma workup was within normal limits and the patient is doing well and disease free at 8 months of follow-up. This rare differential needs to be kept in mind while evaluating a case of well-defined endobronchial growth.
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Background: Adequate lymphadenectomy in middle- and lower-third esophagus cancer is still a matter of debate. This study aims to find out the extent of histopathological supracarinal lymph nodes positivity rate to establish an adequate lymph node dissection in esophageal squamous cell carcinoma cases operated up-front or after neoadjuvant chemotherapy (CT) + radiotherapy (RT) and its short-term oncological outcome. Materials and Methods: After approval from institutional board review, a retrospective study was conducted from April 2017 to September 2019. A total of 76 patients having mid- or lower-third carcinoma esophagus were operated at our institute for partial/total esophagectomy with extended two-field lymph node dissection were followed. Intraoperative nodal stations were harvested separately and lebeled individually according to the Japanese Esophageal Classification and sent for histopathological examination. Results: The patients had an average age of 52 years. Histologically all were squamous cell carcinoma (SCC). Forty-four patients received preoperative concurrent RT plus drug therapy, whereas 18 cases were operated up-front. Fourteen patients were operated after palliative treatment (CT/RT). The average total lymph node yield was 22 nodes (range 3-69). In 26 patients (34.2%), lymph nodes were positive (N+ disease). Supracarinal nodes were positive in 20 cases (26.31%). The average supracarinal lymph node yield was 10.33 nodes (range 2-32). Five patients (6.5%) had only supracarinal lymph nodes positive on histopathological examination. Seventeen patients had a complete pathological response rate (pCR). Conclusion: In cases of mid-third esophageal carcinoma, extended two fields with supracarinal lymphadenectomy is strongly recommended even after the patient has received neoadjuvant treatment, although the same for lower-third/gastroesophageal (GE) junction tumors should be considered.