A Questionnaire Survey of Current Practice in the Management of Internal Mammary Lymph Nodes in Breast Cancer.

Nita S. NairBackground Radiotherapy (RT) is an important modality in the management of breast cancers (BC). Large randomized trials have suggested that prophylactic regional nodal irradiation inclusive of internal mammary lymph nodes (IMLN) reduces BC-related mortality. However, the adoption of IMLN-RT has been variable due to relative benefits and toxicity concerns. Methods A survey was emailed to radiation oncologists (ROs) across the country wherein they were asked about their practice regarding IMLN-RT in BC. Results We received 128 responses, which included radiation oncologists across both private institutions (PIs) and government institutions (GIs). Fifty-six (43.8%) routinely offer prophylactic(p) IMLN-RT and an additional 15 (11.71%) suggested they would have offered it in the absence of logistic constraints. Almost all, 121 (94.5%) radiate the IMLN in case of radiologically positive lymph nodes (LNs). Fifty-six ROs (43.8%) offered prophylactic IMLN-RT in node-negative disease. Among those who did not offer IMLN-RT, most (84.72%) felt the clinical evidence was equivocal. Of the 56 who offered pIMLN-RT, 34/56 (60.71%) offered to locally advanced tumors, 20/56 (35.71%) offered to all inner and central tumors (ICQT), 29/56 (51.78%) to > 4 axillary LN-positive and 9/56 (16.07%) to any axillary LN-positive. The majority, i.e., 36/56 (64.28%) radiated upper three intercostal spaces, 9 (16.07%) radiated upper five intercostal spaces, and 6 (10.9%) decided based on tumor location, while 5 (9%) irradiated one space below the involved space. Overall, simulation-based planning was undertaken in 99% of PIs as opposed to 89% of GIs ( p = 0.03). The majority of ROs, i.e., 92 (72.4%) preferred IMRT to IMLN-RT. In addition, the surgical approach to IMLN was practiced by surgeons at 18 (14%) centers, of which 13 (72.22%) operated the IMLN when radiologically evident. The IMLN dissection was preferentially performed for second and third intercostal spaces as suggested in 10 (55.55%) responses, while 8 (44.44%) performed thoracoscopic dissection of the IMLN chain. The distribution of prophylactic, definitive IMLN-RT, and IMLN dissection did not differ significantly between GI and PI ( p = NS). Conclusion pIMLN-RT is still not the standard protocol in most centers citing equivocal evidence in the literature. Logistics, though different in GIs and PIs, did not impact the decision of pIMLN-RT. Further efforts would be required to standardize practice in IMLN across India.

Primary Hyperparathyroidism in Young and Adolescents: Alike or Unlike Adult Hyperparathyroidism? - A Series from South India.

Background: Primary hyperparathyroidism (PHPT) is a common endocrine condition but rare in the pediatric and adolescent populations. The presentations can be unique, accounting for significant morbidity in the case of untimely detection. Aim: To study surgically treated pediatric PHPT retrospectively. Methods: Surgically treated children of PHPT up to 20 years of age between 2010 to 2022 were analyzed. All of them were operated on by an endocrine surgeon and team. Results: There was a total of 712 parathyroidectomies over 12 years, out of which there were 52 children (7.3%) had PHPT at less than 20 years of age. This group included 32 male children. The mean age was 16.1 years, including 7 cases of neonatal severe HPT. Multiple Endocrine Neoplasia type 1 was confirmed in 12 children. Presentations were more severe like bone pain (35.13%), renal stones (27.02%), incidental asymptomatic detection (18.9%), failure to thrive (10.8%), and pancreatitis (8.1%) as compared to adults. Mean serum calcium was 12.9 mg/dl (highest-14.1, N-8.8-10.8 mg/dl), mean parathormone levels were 386.91 pg/ml (N-10-65) and vitamin D levels ranged from 2.9-22.8 ng/ml. Localization was done with ultrasound and 99mTc- SESTAMIBI scans. Mean serum calcium levels in NSPHPT were 28.6 mg/dl (N-8.8-10.8 mg/dl). There were a total of 45 cases (6.32%) of PHPT less than 20 years of age, excluding the cases of NSPHPT. All children underwent parathyroidectomy, with 14 cases having an additional thymectomy, 2 cases with thyroidectomy, and a single case of hemithyroidectomy. The cure rate was 97.3%, while one baby with NSPHPT had persistent disease (postop PTH-110 pg/ml). The uniglandular disease was seen in 54.05% and the rest had a multiglandular disease. Adults accounted for 559/660 cases with 80% uniglandular disease. All cases had a postoperative histopathological confirmation with an average follow-up of 1 year. Conclusion: Childhood PHPT has a few features same as the adult population. Symptomatic presentations like adults, though pancreatitis and fatigue were more commonly seen as compared to bone pain. Calcium, phosphorus, and parathormone levels were comparable. Uniglandular involvement was seen just like the adult population. There are a few others that make them a distinct subtype like their symptoms of bone pain and being more common among boys. One-fourth of them had MEN1. Fewer cases in this age group make them unique.

Preservation of parathyroid glands during thyroid and neck surgery.

The parathyroid glands are situated in close proximity to the thyroid gland. They have an important endocrine function maintaining calcium and phosphate homeostasis in the body by the secretion of parathormone (PTH), which is responsible for this function. The parathyroid glands are commonly damaged during thyroid surgeries. This could lead to transient or permanent hypoparathyroidism in 30% of cases. Preservation of the parathyroid glands, is an important and integral part of thyroidectomy and other surgical interventions in the neck. The main principle underlying this is a thorough understanding of parathyroid anatomy in relation to the thyroid gland and other important structures in the area. There can also be significant variation in the anatomical location of the glands. Various techniques and methods have been described for parathyroid preservation. They include intraoperative identification utilizing indocyanine green (ICG) fluorescence, carbon nanoparticles, loupes, and microscopes. The techniques of surgery (meticulous capsular dissection), expertise, central compartment neck dissection, preoperative vitamin D deficiency, extent and type of thyroidectomy are the risk factors associated with damaged thyroids, inadvertent parathyroidectomy and subsequent hypoparathyroidism. Parathyroid Autotransplantation is a treatment option for inadvertent parathyroidectomy. Ultimately, the best way to assure normal parathyroid function is to preserve them in situ intraoperatively undamaged.

Potassium losing, aldosterone producing adrenocortical carcinoma: a rare presentation.

Adrenocortical carcinomas (ACCs) are rare malignancies with an incidence of one to two per million per year. Aldosterone-producing ACCs (APACs) are extremely rare with an incidence less than 1%. We describe a rare case of APAC, presenting with episodic lower-limb weakness and hypertension. Our patient was found to have serum aldosterone levels of 20.8 ng/dL (2.5-15.2) with persistent hypokalaemia and a 9.7×8.3×7.7 cm right adrenal mass, which was suspicious of malignancy on evaluation. He underwent a complete surgical resection which confirmed the diagnosis of ACC and normalised his aldosterone and potassium levels. He was then subjected to postoperative chemotherapy. Postoperative adjuvant chemotherapy with mitotane has a role in preventing recurrence.

Neonatal Severe Primary Hyperparathyroidism: A Series of Four Cases and their Long-term Management in India.

CONTEXT: Neonatal severe primary hyperparathyroidism (NSPHPT) is an extremely rare autosomal recessive disorder, requiring a high index of suspicion. Infants affected with this disorder present with severe life-threatening hypercalcemia early in life, requiring adequate preoperative medical management followed by surgery. AIMS: We report four newborns with NSPHPT who were managed over 10 years. SUBJECTS AND METHODS: Demography, clinical presentation, treatment, and follow-up data were retrospectively studied with descriptive analysis to highlight the utility of long-term medical management, surgery, and genetic testing reported in the literature. STATISTICAL ANALYSIS USED: Descriptive Analysis. RESULTS: We had three males and one female infant with a mean age of diagnosis at 28.7 days, calcium 29.2+/-2.8 mg/dL, and parathormone (PTH) 1963+/-270.4 pg/mL. All four infants presented with failure to thrive, hypotonia, and respiratory distress. All infants were treated medically followed by total parathyroidectomy plus transcervical thymectomy, with an additional hemithyroidectomy in one of them. Imaging was negative in all four cases. Three babies became hypocalcemic while the fourth infant had a drop in PTH and is on the tab. cinacalcet 30 mg/day. CaSR mutation was positive in three infants. CONCLUSIONS: Diagnosing NSPHPT needs expert clinical acumen. It requires emergency medical management to control calcium levels. The crisis may present later, necessitating parathyroidectomy in these cases once the child is fit for surgery. Surgery offers a cure for this unusual lethal hypercalcemia while the role of cinacalcet needs a special mention. Sound knowledge in endocrinology with parathyroid embryology and morphology is of paramount importance. Our case series might add a few insights into managing this unusual genetic disorder.

Prevalence and implications of thyroid related embryological remnants: A prospective study of 1118 total thyroidectomies.

CONTEXT: Modern thyroid surgery has undergone a paradigm shift from subtotal thyroidectomy to an extended total thyroidectomy (TT) even for benign disorders. This entails removal of all embryological remnants even in benign disorders. AIMS: To study the prevalence of various embryological remnants of the thyroid and surgical utility and implications in preventing complications. SETTINGS AND DESIGN: Retrospective study of total thyroidectomies done by a single endocrine surgeon by standardized technique. METHODS AND MATERIAL: A detailed search of all embryological rests including Pyramidal tract (PT), Tubercle of Zuckerkandl (TZ), and Thyro-thymic thyroid rests (TTR) were done in 1118 patients undergoing TT over 6 years. The cases with and without TTR were divided as Group A and B, respectively. Their prevalence and impact on parathyroid preservation and other clinical parameters were analysed. STATISTICAL ANALYSIS USED: Descriptive analyses. RESULTS: Out of the 1118 TT cases, TTR was seen in 230 (20.57%) cases, TZ in 598 (53.48%), cases and PT in 641 (57.33%) cases. Among group-A (n = 230), 213 had unilateral and 17 had bilateral TTR with 51 (22.17%) having retrosternal extension. Compressive symptoms, presence of TZ and PT were also significantly higher in group A. On follow up the incidence of temporary hypoparathyroidism was significantly higher in group-A, where as permanent hypoparathyroidism, temporary and permanent vocal cord palsy were comparable between the two study groups. CONCLUSIONS: Embryological remnants related to thyroid are not uncommonly encountered during total thyroidectomy. A thorough search and complete removal is crucial for the successful outcome of the procedure.