Hereditary Medullary Thyroid Carcinoma: Genotype, Phenotype and Outcomes in a North Indian Cohort.

BACKGROUND: Aggressiveness of hereditary medullary thyroid carcinoma (hMTC) has been conventionally described to correlate with American Thyroid Association (ATA) risk groups based on RET mutations. Recent evidence increasingly contradicts this notion. We studied the RET genotype and its correlation with disease phenotype and survival outcomes in a cohort of hMTC patients. METHODS: In a retrospective cohort of 55 hMTC patients from 23 families treated at a north Indian tertiary care institute over 15-years, RET genotype was correlated with disease phenotype (clinical, biochemical, and pathological attributes) and outcomes in terms of biochemical cure (normalization of serum calcitonin), structural cure, overall survival (OS) and disease specific survival (DSS). RESULTS: Forty-nine patients had Multiple Endocrine Neoplasia (MEN)-type 2A syndrome, 02 had MEN-2B, and 4 had familial MTC. Two patients belonged to highest ATA risk, 41 to high-risk, and 12 to moderate risk categories. Age of the patients or stage of disease at presentation did not differ significantly between the ATA risk groups. Though the baseline serum calcitonin was significantly higher in highest risk category, the biochemical cure rates were not significantly different. At a median follow up of 48 months (Inter-quartile range 18-84, range 12-192) structural cure rates in ATA moderate and high risk groups were significantly higher than highest risk group (p = 0.04). No significant difference in OS between the three ATA groups of hMTC among the patients who underwent surgical treatment was observed (p = 0.098). CONCLUSIONS: The ATA moderate and high risk groups have better structural cure rates compared to ATA highest risk group. The biochemical cure and overall survival rates did not significantly differ between ATA risk-groups, and were impacted by the disease stage at presentation. The current ATA risk-groups do not reliably predict the outcomes in terms of biochemical cure and survival in hMTC patients.

Endoscopic versus Conventional Thyroid Surgery: A Comparison of Quality of Life, Cosmetic Outcomes and Overall Patient Satisfaction with Treatment.

BACKGROUND: Our aim was to investigate whether the surgical approach, i.e. endoscopic (ET) versus conventional/open thyroidectomy (ConT), contributes towards the improvement in quality of life (QoL), cosmetic outcomes and overall patient satisfaction with treatment. METHODS: We conducted a prospective study (October 2016-August 2018) consisting of patients undergoing hemithyroidectomy for euthyroid, non-malignant solitary thyroid nodules (STNs). Patients were divided into two groups: Group I (ET)-41 patients and Group II (ConT)-52 patients. ET was performed via bilateral-axillo breast approach (BABA). A thyroid disease-specific questionnaire "ThyPRO-39hin" was used to evaluate pre- and post-operative QoL. Visual analogue scale (VAS) was used for post-operative pain. Cosmetic satisfaction, paraesthesia and overall satisfaction with treatment were evaluated using Likert-type verbal response scales. Pre- and post-operative scores (>6 months) were compared, and p value < 0.05 was considered significant. RESULTS: Mean age (p = 0.26), gender distribution (p = 0.07), mean tumour size (p = 0.74) and preoperative scores of QoL were comparable between the two groups. Post-operatively scores of symptom domain (p = 0.03), tiredness (p = 0.03), impaired social life (p = 0.03), cosmetic complaints (p = < 0.001) and overall QoL (p = < 0.001) were significantly better in Group I. Also, post-operative pain perception at first follow-up visit (p = 0.001) was lower in patients undergoing ET/Group I, with higher scar satisfaction scores (p = < 0.001) and overall satisfaction with treatment (p = < 0.001). Post-operative paraesthesia perception was comparable (p = 0.06) amongst the two groups. CONCLUSION: In this cohort study, patients undergoing endoscopic thyroid surgery reported superior post-operative QoL, cosmetic and overall satisfaction with treatment as compared to conventional/open thyroidectomy.

Low-cost Fluorescein as an Alternative to Radio-colloid for Sentinel Lymph Node Biopsy-a Prospective Validation Study in Early Breast Cancer.

BACKGROUND: Sentinel lymph node biopsy (SLNB) using radio-pharmaceutical (RP) and a blue dye is gold standard for axillary staging in clinically node-negative early breast cancer. High costs and limited availability of RP and/or gamma probe are major deterrents in performing SLNB in developing countries. Fluorescence-guided SLNB can obviate the need for RP and gamma probe. Fluorescein is an inexpensive fluorescent lymphatic tracer. In this study, we compared SLN identification rate (SLN-IR) and false negative rates (FNR) of fluorescein-guided SLNB and radio-guided SLNB using 99mTc-Sulfur-colloid, in isolation, or in combination with methylene blue dye (MBD). METHODS: Sixty-five cN0 early and large operable breast cancer patients underwent validation SLNB using fluorescein (and blue LED light), 99mTc-Sulfur-colloid (and gamma probe) and MBD. Inj Fluorescein 4% was injected, 1 ml each peri-tumoral and sub-areolar five minutes before axillary incision. Axillary dissection was performed irrespective of SLNB histology. The SLN-IR and FNR with various tracers and their combinations were compared. RESULTS: The mean number of SLNs identified was 3.5 ± 1.8 (range 1-6). The SLN-IR using RP alone was 94%, fluorescein alone was 92%, and MBD alone was 82%. The SLN-IR using fluorescein plus MBD combination was 95.4%, compared to 97% using MBD plus RP combination. FNR was 6.3% using fluorescein plus MBD, as well as RP plus MBD combinations. CONCLUSIONS: SLN-IR of > 90% and SLN-FNR of < 10% using fluorescein plus MBD combination are in acceptable range, and are comparable to that of RP plus MBD combination. Fluorescein can replace RP for performing SLNB, in combination with MBD.

Prospective Randomized Trial of Use of In-House Prepared Low-Cost Radiopharmaceutical Versus Commercial Radiopharmaceutical for Sentinel Lymph Node Biopsy in Patients with Early Stage Invasive Breast Cancer.

BACKGROUND: The current standard-of-care for surgical staging of the axilla in clinically node-negative (N0) early breast cancers is sentinel lymph node biopsy (SLNB), which requires expensive radiopharmaceuticals for efficacious results. In-house produced low-cost radiopharmaceuticals may be the solution and have shown efficacy in earlier observational/pilot studies. We compared SLNB using in-house prepared radiopharmaceutical (99mTc-Antimony-colloid) versus commercially marketed radiopharmaceutical (99mTc-Sulphur-colloid) in this prospective randomized study. STUDY DESIGN: 78 clinically N0 early breast cancer patients (T1/2, N0 stages), undergoing primary surgery were prospectively randomized 1:1 into two groups; to receive SLNB using methylene blue, and either 99mTc-Antimony colloid (Group-1) or  99mTc-Sulphur colloid (Group-2). Completion axillary dissection was done in all (validation SLNB). SLNB indices were compared between the groups. RESULTS: The groups were comparable with regard to age, stage, tumour size, hormone receptors and HER2neu status. Cost of the in-house prepared 99mTc-antimony colloid was 16-times lesser compared to 99mTc-sulphur colloid. SLN identification rates (IR) in Groups 1 and 2 were 100 and 97.4% respectively, (p > 0.05). False negative rates (FNR) in Group 1 and 2 were 6.3% (1/16 patients) and 7.7% (1/13 patients), respectively, (p > 0.05). There were no major allergic reactions in either group. CONCLUSION: In this prospective randomized trial on early breast cancer patients, accuracy of SLNB was comparable using in-house prepared, 99mTc-antimony colloid and commercially marketed 99mTc-sulphur colloid as radiopharmaceutical, while 99mTc-antimony colloid was much cheaper than 99mTc-sulphur colloid.

Surgical Outcomes of Primary Versus Post-Neoadjuvant Chemotherapy Breast Conservation Surgery: A Comparative Study from a Developing Country.

INTRODUCTION: In India and other developing countries, breast conservation surgery (BCS) rates in breast cancer patients are low due to advanced disease at presentation and misconceptions about BCS outcomes. Many patients presenting with large or locally advanced breast cancers (LABC) can be offered post-neoadjuvant chemotherapy (NACT) BCS, safety of which is not as well established as that of primary BCS. This retrospective study compared pathological and surgical outcome parameters in patients undergoing primary and post-NACT BCS. METHODS: All non-metastatic breast cancer patients undergoing BCS during 2011-2015 with 1-year follow-up were included. Outcome parameters in form of margin infiltration, ipsilateral breast tumor recurrence (IBTR) rates and IBTR-free survival were compared between primary and post-NACT BCS patients groups. RESULTS: One hundred and twenty-nine patients underwent BCS; 95 underwent primary and 34 post-NACT BCS. Patients in both groups underwent similar multimodality treatment as per institutional protocols. Post-NACT patients more frequently required oncoplastic volume displacement or replacement surgery (p = 0.002). Re-excision of infiltrated margins was needed more frequently in primary BCS compared with post-NACT BCS group (14.4 vs. 8.8%; p = 0.40). IBTR (Mean follow-up = 30.7 months) was seen in 8.8% post-NACT patients compared with 2.1% primary BCS (p = 0.114). IBTR-free survival did not differ significantly between the groups in stage-wise comparison. CONCLUSION: Post-NACT BCS is safe even in large tumors and LABC, though many require oncoplastic procedures for satisfactory cosmesis. In a developing country where many patients present with large breast cancers or LABC, the benefits of BCS can be offered to a majority with the help of NACT, without compromising the chances of cure.

A Rare Complication of Left Open Adrenalectomy.

Chyle leakage after left open adrenalectomy is a quite rare complication. We encountered a young male patient who was operated for left adrenal pheochromocytoma, left laparoscopic adrenalectomy converted to open due to dense adhesions. Postoperatively patient developed chylous fluid drainage on day 2. He was managed conservatively with high protein and MCT oil diet. This case highlights the unusual complication of left adrenalectomy surgery and how it can be managed.

Laparoscopic Transperitoneal Adrenalectomy for Adrenal Tumors in Children: Technical Considerations and Surgical Experience.

Aims: This study aimed to review our surgical experience of laparoscopic adrenalectomy (LA) for adrenal masses in the pediatric age group. Materials and Methods: The electronic medical records of all patients younger than 18 years of age who underwent LA between 2016 and 2023 were retrospectively reviewed. Children with adrenal tumors localized to the site of origin without evidence of encasement of major vasculature or involvement of adjacent organs were considered for LA. Patients with diagnosis of malignant adrenal tumor on preoperative work-up underwent open adrenalectomy. Data were collected regarding demography, clinical presentation, hormonal workup, imaging, duration of surgery, transfusion requirement, conversion rate, postoperative recovery, duration of intravenous (i.v.) analgesia and hospital stay, pathology, complications, and status at follow-up. Results: Between 2016 and 2023, LA adrenalectomy was performed in 11 patients (6 boys and 5 girls) with a mean age of 46.3 (8-120) months. A functional tumor was detected in 6 (54.5%) children, manifesting clinically with Cushing's syndrome (3), virilization (1), feminization (1), or Conn's syndrome (1). Seven (63.6%) tumors originated from the right adrenal, and 4 (36.4%) from the left adrenal gland. The mean tumor weight and size was 49.1 (10-80) g and 5.6 (3-8) cm, respectively. Histopathology included adrenal adenoma (5), ganglioneuroma (3), ganglioneuroblastoma (1), myelolipoma (1), and intermediate adrenocortical tumor (1). The mean surgery duration was 186.6 (120-265) minutes. Intraoperative blood transfusion was required in 2 (18.2%) patients and none required conversion. Full feeds were resumed at 42.7 (24-60) hours, i.v. analgesia requirement was for 54.5 (36-72) hours and mean hospital stay was 5.1 (3-8) days. All patients were symptom-free with no recurrence at mean follow-up period of 50.6 (3-83) months. Conclusions: In children with well-circumscribed, localized, and noninvasive adrenal tumors, LA is feasible, effective, and safe with all advantages of minimally invasive surgery.

Comparison of indocyanine green angiography vs intraoperative parathyroid hormone in early prediction of risk of post-thyroidectomy hypocalcemia: a prospective cohort study.

Introduction: Indocyanine green (ICG) angiography is the 'real-time intraoperative imaging' technique used to reduce the chances of hypoparathyroidism in post-thyroidectomy patients. In our study, the authors predicted the risk of early post-thyroidectomy hypocalcemia by intraoperative evaluation of parathyroid gland perfusion by ICG angiography. Materials and methods: In patients who underwent total thyroidectomy, ICG angiography was done using the SPY PHI imaging system (Stryker). Post-thyroid specimen removal, scoring of parathyroids was done in spy contrast mode. All 4 or <4 visualized parathyroids were scored for vascularity with the highest score of 8. Serum ionized calcium was done 6 h postsurgery and on the morning and evening of postoperative days 1 and 2. Calcium supplements were given to only those who developed clinical or severe biochemical hypocalcemia. Results: Out of 60, postoperative hypocalcemia was noted in 41 patients. Total ICG score ≤5 was seen in 34 patients, out of which 28 developed postoperative hypocalcemia showing PPV 82.3% and diagnostic accuracy of 68.3% while iPTH (4.28 pmol/l) showed PPV 76.7 and diagnostic accuracy 70 %. In eight patients, none of the glands was scored as 2 (White) and all these patients developed hypocalcemia requiring calcium infusion. Conclusion: The absence of visualization of at least 1 well-perfused (score 2) gland on ICG angiography is highly predictive of hypocalcemia and the majority of patients with total ICG score ≤5 developed hypocalcemia in the immediate postoperative period. ICG is a good predictor of the absence of hypoparathyroidism after thyroidectomy and is comparable to iPTH in the prediction of post-thyroidectomy hypocalcemia.

What makes women receptive to breast self-examination, animation, or simulation? - a comparative study.

Background: Breast self-examination (BSE) plays an important role in the early diagnosis of breast cancer in India owing to the stigma attached to cancer. The authors compared the efficacies of animation video versus simulation techniques in BSE. Methods: Women with no previous history of conditions affecting the breasts were included in this prospective observational study and divided into an animation or simulation arm. The latter was further divided into three subgroups as per the simulation models used : the German (Delta Healthcare), British (Health Edco), and Indian (low-cost, validated) models used for teaching BSE. The hybrid animation video had a 9 min runtime with a lecture on BSE and a virtual character performing BSE. In both the arms, participants filled in a validated modified patient satisfaction questionnaire. Results: A total of 500 women participated. The mean age of the participants in the animation video arm was 20.21±3.88 years and 19.34±2.27, 22.94±9.6, and 18.97±1.31(20.41±5.99) years in the Indian, German, and British simulation models arm, respectively. The age difference between the two arms was statistically significant (P<0.05). Both animation video and simulation models were found to be useful by the participants. The participants' response to animation video being a better organized tool for learning BSE was statistically significant (90.48±7.98 vs. 84.02±15.09 P≤0.001) when compared to simulation models. The younger women (≤20 years) found these tools significantly more useful than those aged >20 years. Conclusions: All models had good efficiency and utility as learning tools for BSE. However, large studies in BSE set up with combination models are needed.

Validation of cost-effective model for breast self-examination.

The incidence of breast cancer is increasing in India; it predominantly affects women in their 30s and 40s. The disease burden is very high given the high incidence of triple-negative disease in a large portion of the population. Early detection can save lives and aid in breast conservation surgery. Breast self-examination (BSE) is a valid tool for early breast cancer detection. If performed with the help of a simulation model that resembles a given culture and tradition, it can result in good outcomes from screening programs. We designed and validated an Indian model for BSE and reported the feasibility of this model. Materials and methods: We designed an Indian model for the BSE based on the cultural mindset of Indian women. The design was finalized, and the model was constructed. It was then compared with preexisting international models and validated by in-depth interviews with validation experts from various fields involved in breast cancer management. Minor design revisions were made, followed by testing and re-testing. Finally, it was ready for public use. Results: The in-depth interview was conducted using a validated modified animation multimedia questionnaire. The majority of the validation experts had used stimulation models before, and all stated that it could help teach women about BSE, and it was comparable with other preexisting internationally validated models (91.33±4.98%). Conclusion: Using a breast model, women can learn to detect breast cancer as early as possible, and this can lead to good outcomes. We designed the model using easily available, cheap, and safe materials to keep it as realistic and useful as possible. The Indian BSE model can be used by Indian women to learn to detect breast lumps early. It is easily reproducible and cost-effective.

PHPT with Pancreatitis: Atypical Presentation of PHPT.

Background: Primary hyperparathyroidism (PHPT) is rarely associated with the occurrence of acute or chronic pancreatitis, requiring complex perioperative management. This study aimed to assess the prevalence and disease characteristics of pancreatitis in PHPT. Materials and Methods: This study is a clinicopathological analysis of the medical records of patients who were diagnosed with PHPT with pancreatitis between 1989 and 2021 in the Endocrine Surgery department, SGPGI, Lucknow. Results: Out of 548 PHPT cases, 44 (8.03%) were found to be associated with pancreatitis. The mean age was 33.57 years (15-65 years); 5 were ≤20 years, while 26 were ≤30 years of age. There were 27 males and 17 females. Twenty-one cases were of acute (11 acute, nine recurrent acute, one acute on chronic), whereas 23 were of chronic pancreatitis (six chronic calcific pancreatitis). The major clinical presentation of PHPT with pancreatitis was abdominal pain (65.91%). The mean number of attacks per patient in recurrent acute pancreatitis was two. Mean PTH levels were 68.19 pmol/L. The mean tumor size (in the largest dimension) was 2.79 ± 1.4 cm while the mean tumor weight was 4.91 g. Nephrolithiasis was associated with 25 cases. An association with multiple endocrine neoplasia type 1 syndrome was seen in one case. The final histopathological diagnosis was parathyroid carcinoma in two, hyperplasia in three, and parathyroid adenoma in 39 cases. Normocalcemia was seen in 27.2%, hypercalcemic crisis in 15.9%, and 25% of patients required semi-emergency parathyroidectomy. The outcome was favorable in all, as none had any further attacks of pancreatitis. Conclusion: In our study, the prevalence of pancreatitis in PHPT cases was 8.03%. The majority of patients were young. Normocalcemia was seen in 12 patients, so even if calcium levels are normal, PHPT should be suspected in young patients with pancreatitis. Parathyroidectomy resulted in the complete resolution of symptoms of pancreatitis in all 44 patients.