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.
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.
Objective: A rare disease. Background: von Hippel-Lindau (VHL) disease is an autosomal dominant familial syndrome with a multitude of benign and malignant multivisceral tumors and one-third of them harbor pheochromocytoma (PCC). We intend to present one such rare case and challenges in its surgical management. Case: A 38-year-old lady presented with hypertension and episodic headache. Biochemical evaluation was suggestive of hypercatecholaminism. Magnetic resonance imaging (MRI) showed the presence of a heterogeneous intensity lesion in the left suprarenal area suggestive of PCC. Multiple cysts were seen in both the kidneys as well as in the pancreatic head and tail. Imaging of the brain and spine showed a left cerebellar and spinal cord hemangioblastoma, respectively. I-131 metaiodobenzylguanidine (MIBG) showed high uptake in the left suprarenal region. After adequate preparation, she underwent left adrenalectomy. Histopathology was consistent with adrenal PCC and the patient was biochemically cured of hypercatecholaminism. The presence of PCC and other manifestations of VHL type 2A were evident in this patient. Conclusion:The presence of multiple renal and pancreatic cysts can camouflage adrenal tumors intraoperatively. Careful identification, dissection, and differentiation of PCC from these cysts are crucial. Multicentricity and multifocality of PCC are not uncommon in VHL and these patients need lifelong close follow-up for other tumors as well.
Background 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). Conclusions 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.
Background: Thyroid disease has myriad manifestations. Thyroid stimulating hormone (TSH) and a paired determination of TSH and freeT4 would add to find out the degree of dysfunction with overutilization of thyroid function tests (TFT’s) as the symptoms are being overseen in the recent days. The objectives of the present study were to validate the clinical symptom scoring index for thyroid disorders using TSH and FT4.Methods: The clinical symptom scoring index consisting of 20 parameters was given to 110 patients presenting to our outpatient department with new onset thyroid-related complaints from June2018 to Spetemer2018. They were subjected to TFT (TSH and free T4) and classified accordingly. Statistical analysis was done and receiver operator curves (ROC) plotted.Results: The mean TSH for the 3 groups (hypothyroid, hyperthyroid and euthyroid) were 13.65, 0.31 and 1.7 mIu/ml respectively (normal value: NV-0.25-5). Mean FT4 were 0.58, 5.01 and 1.28 ng/dl respectively (normal value 0.82-1.51). Most frequent findings in biochemical hyperthyroidism were easy tremors (100%), tiredness (90%), palpitations (93%) and weight loss (75.6%). The clinical symptom scoring index had a sensitivity of 85% and accuracy rate of 86.76% with area under the curve (AUC) 0.865 on ROC analysis for the detection of hyperthyroidism. It had a 100% sensitivity and AUC 0.65 for hypothyroidism.Conclusions: The symptom scoring index for thyroid disorders was found to have good specificity, negative predictive value (NPV) and accuracy rate when correlated with TSH and FT4 for the detection of hyperthyroidism. In the developing countries, these score indices can aid as auxiliary diagnostic tools, reducing the load on referral centers.
Thyroid cancer has become an epidemic due to easy availability of ultrasound of the neck, and in some countries, routine health checkup ultrasound of neck is routinely done and mandatory. Thyroid cancer detected incidentally and less than 1 cm may warrant only observation, whereas some cancers such as anaplastic thyroid cancer requires urgent intervention. Advances in the field of oncology have been revolutionized by the extensive study of tumor microenvironment (TME). The introduction of immune check point inhibitors resulted in a major shift in the understanding of differentiated thyroid cancer. Inflammation related to thyroid cancer involves various molecular patterns of cytokines and chemokines. They form the major targets for novel immunotherapies. Addition of discovery of newer tumor markers has significantly contributed to cancer management. Tumor immune escape is an important mechanism of oncogenesis. Innate immunity forms the major defense of the body to tumor cells. Polymorphonuclear leucocytes, macrophages, and lymphocytes form the defense that target tumor cells. The aim of this review is to comprehensively discuss the dynamic immune system, various oncogenic pathways and novel tumor antigens like cancer testis sperm associated antigen (SPAG9).
Aim: To present a case of posterior mediastinal goiter successfully delivered through the neck approach. Background: Posterior mediastinal goiters are rare, comprising less than 10% of intrathoracic goiters. They usually manifest with compressive symptoms and warrant definitive surgery to relieve symptoms. Majority require the combined cervicothoracic approach to deal with the goiter. Technique: We intend to discuss a case of retrosternal goiter extending predominantly into the posterior mediastinum, which has been purely delivered by cervical incision. Conclusion:The above technique describes successful and safe extraction of the retrosternal goiter extending primarily into the posterior mediastinum via the cervical approach. Clinical significance: The compressive symptoms were completely resolved after surgical management, thus avoiding a morbid procedure like thoracotomy and its associated complications.
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