Anaplastic thyroid cancer (ATC) is a rare, but extremely aggressive, form of cancer with a high mortality rate. Differentiated thyroid cancer (DTC), on the other hand, including papillary and follicular subtypes, are relatively common and typically follows a more indolent course. Cases have been reported in which ATC transforms from DTC, and where DTC and ATC exist simultaneously. Given the low incidence of such cases, they have not been well studied, and the optimal treatment regimen has yet to be determined. We present a case of a 77-year-old woman who was initially presented with papillary thyroid cancer (PTC) with focal ATC. Five months after undergoing total thyroidectomy, she returned with a new right sided neck mass. Fine needle aspiration (FNA) with biopsies of the mass and lymph node at one level revealed a smear pattern consistent with ATC. However, lymph node biopsy taken from a different level revealed a smear pattern consistent with PTC. Mutation analysis was performed and results were positive for metastatic BRAF V600- mutant ATC. The patient was then started on dabrafenib/trametinib chemotherapy. Seven months later, she was tolerating treatment well. These unique clinical features including the initial presentation and the relatively favorable survival, that is more than double that of the median survival rate for ATC, suggests that those with synchronous PTC and ATC may have a more indolent course with better prognosis than those with ATC alone. It is also possible that the relatively longer survival in our patient is due to the use of the BRAF inhibitor, dabrafenib and the MEK inhibitor, trametinib in this case with concurrent ATC and PTC. While patients with both PTC and ATC have been documented to have mutations in the BRAF V600 gene, the objective of this report is to present the relatively favorable outcomes when a therapeutic regimen is guided by mutation analysis. Future research into advanced treatment options including targeted therapy and/or immunotherapy for both DTC and ATC is needed. Somatic mutation testing may also be helpful to identify oncogenic kinase abnormalities that will inform therapeutic decision making.
26 patients have had jejuno-ileal bypass for morbid obesity. 24 cm of proximal jejunum was anastomosed end-to-side to 24 cm of terminal ileum. Weight loss has been adequate, averaging 70% of ideal weight. Complications have mostly been transient and easily remediable on an out-patient basis. One patient was converted to gastric partitioning because of diarrhea. No other serious complications occurred. The authors recommend further trails with the 24/24 cm version of jejuno-ileal bypass for morbid obesity, complemented with cholecysto-jejunostomy to the proximal end of the excluded small bowel.
Abstract Background: Cribriform-Morular variant of Papillary Thyroid Cancer (PTC-CMV) is an exceedingly rare subtype of thyroid cancer that predominantly affects younger females. As the name implies, it is a papillary thyroid carcinoma with predominantly cribriform and morular pattern of carcinoma cells on cytopathology. While completion thyroidectomy is usually recommended for larger and higher-risk Papillary Thyroid Cancer (PTC), surveillance may be acceptable with PTC-CMV, which tends to be a less aggressive malignancy. Clinical Case: A 46-year-old Guyanese woman presented with a three week history of an enlarging right-sided neck mass associated with a globus sensation while swallowing food. She denied any history of radiation exposure. Her exam findings were positive for a tender, right-sided neck mass. CT neck without contrast revealed a 4.1 x 4.0 x 5.9cm heterogeneous mass within the right thyroid lobe causing mild tracheal deviation to the left. Ultrasound of thyroid gland showed a solid heterogeneous hypoechoic 4.22 x 2.39 x 2.46cm right lobe nodule with no microcalcifications, border irregularity or taller-than-wider morphology. Fine Needle Aspiration of the nodule came back as Atypia of Undetermined Significance. The patient then underwent a core needle biopsy. The resultant pathology was negative for thyroid carcinoma or medullary thyroid carcinoma but was suggestive of a bronchial cleft cyst versus bronchogenic cyst with atypical glandular proliferation. She subsequently underwent a right hemithyroidectomy which revealed a final pathological diagnosis of a 3.5cm PTC-CMV. Such pathology warranted the patient to undergo a colonoscopy which was negative for Familial Adenomatous Polyposis (FAP). Given her negative GI workup and non-contributory family history for colonic polyposis or carcinoma the decision was made to continue surveillance rather than performing completion thyroidectomy as the disease was presumed to be sporadic. Discussion: PTC-CMV accounts for 0.2% of all PTC. It is associated with FAP in more than 50% of cases but can also occur sporadically. This subtype of PTC generally follows a less aggressive course. Review of current literature revealed several case series of CMV-PTC patients. In the largest one, 32 cases were observed over a 19 year period and only two out of twelve patients with FAP-associated PTC-CMV initially treated with hemithyroidectomy developed recurrence to the contralateral lobe. Interestingly, none of the remaining patients with the sporadic type developed recurrence suggesting that completion thyroidectomy may not be mandatory in this group. It is, therefore, critical to identify these patients and screen them with a colonoscopy to avoid the potentially unnecessary resection of the contralateral lobe and the consequent need for thyroid hormone replacement.
Abstract The myriad of presentations associated with PHP are well established, however gout is not commonly associated with this disease. Additionally, it is unusual to see multiple threatening and potentially debilitating complications occur concurrently in one patient. An asymptomatic elderly male with hypertension presented to the PCP for the first time and was found on routine blood work to have a serum creatinine of 4.03 mg/dl, a serum calcium of 12.1mg/dl, and a PTH of 831.7ng/L. Subsequent Tc-99 Sestamibi scanning suggested that the source was both a single right inferior parathyroid adenoma and an ectopic mediastinal adenoma. At the initial encounter the patient’s hypercalcemia was treated with IV fluid resuscitation and calcitonin then subsequently cinacalcet. Renal ultrasound at that time showed normal sized kidneys with several cysts, and phosphate levels ranged from 2.0-3.9 mg/dl (range 2.5-4.5mg/dl. The patient’s serum calcium was controlled at 10.44mg/dl, and his renal function improved to a serum creatinine of 3.08mg/dl. Prior to discharge patient developed acute left knee pain, and was found to have an inflammatory arthritis, with urate crystals seen. The patient was diagnosed with an acute gout flare, which responded well to colchicine and was discharged. The patient eventually underwent parathyroidectomy, which showed a large 4cm left superior parathyroid as well as a large right superior parathyroid gland extending into the mediastinum. Pathology was consistent with parathyroid hyperplasia. After surgery, the patient developed hungry bone syndrome, with an admitting serum level calcium of 6.05mg/dl, serum magnesium of 2.00mg/dl, serum phosphorus of 2.8mg/dl, and serum potassium of 5.1mg/dl, with clinical features of tetany and weakness that resolved after two days with calcium and calcitriol administration. He again had an acute monoarticular arthritis prior to discharge that had both urate and calcium pyrophosphate crystals in the joint fluid and again responded well to colchicine and glucocorticoids. He was eventually discharged on vitamin D and calcium supplementation, with cinacalcet and colchicine. This case illustrates the multiple clinical teaching points that exist in primary hyperparathyroidism, including both types of presentation and potential complications. It also expresses the need to be vigilant of some rarer clinical features, such as potential hyperparathyroid crisis and multiple enlarged parathyroid glands. Physicians should also be wary of both gout and CPPD, as well as complications that occur post parathyroidectomy such as hungry bone syndrome.
Among several causes leading to adrenal insufficiency, opioid-induced adrenal insufficiency (OIAI) is an under-recognized condition that occurs secondary to prolonged exposure to opioids. OIAI can lead to a life-threatening adrenal crisis and may have serious adverse outcomes if left undetected or unmanaged.
Abstract Background Primary aldosteronism (PA) is one of the common causes of secondary hypertension. Primary hyperparathyroidism (PHPT) is a less common etiology of secondary hypertension. The concomitance of both PA and PHPT is very rare, with a possible bilateral positive feedback loop between the adrenal cortex and the parathyroid gland[1]. Clinical case: A 64-year-old female was referred to the endocrine clinic for secondary hypertension assessment. She was diagnosed with hypertension 30 years earlier, poorly controlled on three antihypertensive medications, nifedipine 90 mg daily, lisinopril 40 mg daily, and atenolol 50 mg daily. She started experiencing muscle cramps two years prior to the referral and was found to be hypokalemic on multiple occasions, with potassium ranging between 2.5 to 3 mmol/L. She also had multiple admissions to the emergency department for hypertensive urgencies within the last year. Family history was positive for poorly controlled hypertension in the patient's mother. Physical examination showed class I obesity with a BMI of 31, but otherwise unremarkable. Workup showed elevated aldosterone concentration of 38 ng/dl and suppressed renin concentration (<1 pg/ml) with an aldosterone/renin ratio of 38. Overnight dexamethasone suppression test was normal (cortisol of 1.5 mcg/dl, suppressed ACTH of 5.5 pg/ml, and appropriately elevated dexamethasone of 324 ng/dl). 24-hour urine metanephrine and normetanephrine levels were normal. Imaging showed bilateral adrenal adenomas of about 13-14 mm in widest diameter, with an average density of 10 Hounsfield units. Adrenal venous sampling revealed hyperfunctioning left adrenal with normal right adrenal. The left adrenal gland was removed laparoscopically, and blood pressure normalized postoperatively on the single antihypertensive agent (lisinopril). Interestingly, during the initial workup, the patient also had elevated calcium of 11 mg/dl and low phosphate at 2.5 mg/dl. In addition, parathyroid hormone (PTH) was elevated at 103 and 95 pg/ml on two occasions, with normal 25-OH vitamin D levels. 24-hoururine calcium was 240mg/dl, and creatinine in urine was 76.4 mg/dl. The calcium creatinine clearance ratio was 2.5%. Diagnosis of primary hyperparathyroidism was made. She had no renal stone, chronic kidney disease, or fractures, with a normal DEXA scan. There was no current indication for parathyroidectomy, and the patient continued to be followed up in the clinic. Clinical lesson: Recent evidence shows cross-talk between the adrenal cortex and parathyroid gland. Our patient may represent such a case, with concomitance of two rare endocrinopathies resulting in difficult-to-control hypertension [1,2]. Thus it is essential to investigate multiple etiologies of secondary hypertension. Reference: Brown JM, Vaidya A. Interactions between adrenal-regulatory and calcium-regulatory hormones in human health. Curr Opin Endocrinol Diabetes Obes . 2014;21(3): 193-201. doi: 10.1097/MED. 0000000000000062 Maniero C, Fassina A, Guzzardo V, et al. Primary hyperparathyroidism with concurrent primary aldosteronism. Hypertension. 2011 Sep;58(3): 341-6. doi: 10.1161/HYPERTENSIONAHA.111.173948. Epub 2011 Aug 8. PMID: 21825220. Presentation: No date and time listed
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