64-year-old woman • hot flashes, facial flushing, excessive sweating, and palpitations • daily headaches • history of hypertension • Dx?

► hot flashes, facial flushing, excessive sweating, and palpitations ► daily headaches ► history of hypertension.

Successful Long-Term Medical Management of Unresectable Insulinomas.

In this paper, we present two patients with unresectable insulinomas and a literature review. Patient 1: A 58-year-old woman was diagnosed at age 42, with an insulinoma in the pancreatic tail and hepatic metastasis. She underwent distal pancreatectomy, splenectomy, hepatic wedge resection, and chemoembolization, with resolution of her symptoms. By age 48, her symptoms returned, with new hepatic metastasis. She started long-acting octreotide, with subsequent resolution of her symptoms. She has since had an unremarkable clinical course. Patient 2: A 48-year-old female was diagnosed at age 37. Numerous imaging modalities and two exploratory surgeries did not localize a mass. A distal pancreatectomy did not resolve her symptoms. She tried several medications before her symptoms were finally controlled with low-dose prednisone. She has continued prednisone and diazoxide treatment for the past decade, which controls her symptoms, along with diet modification. In conclusion, while prednisone is not standard therapy, it can control symptoms in patients with unresectable insu-linoma. Providers should be aware of available and emerging medical options. Patients with unresectable insulinomas will likely have better long-term survival rates than those quoted in historical literature. Additional studies are needed to elucidate survival rate and the long-term efficacy of medical therapies.

Diarrhea as an Initial Presentation in Patients with Medullary Thyroid Cancer: Delaying the Diagnosis.

Tumoral secretion of various molecular factors, such as calcitonin (Ct), can cause diarrhea in patients with medullary thyroid cancer (MTC). We report 3 patients (age 26-38 years, serum Ct levels ranging from 2,890 to 52,894 ng/L) with chronic diarrhea, and the diagnosis of MTC was delayed. Diarrheal symptoms improved after thyroid surgery. Two patients with elevated Ct had no diarrhea. The link between tumor humoral secretion and diarrhea is not well established in patients with MTC. Diarrhea is more common in patients with metastatic disease and improves after resection of the tumor. Diarrhea may result from elevated circulating levels of Ct or other substances, such as prostaglandins or serotonin. Other proposed mechanisms include decreased absorption in the colon secondary to gastrointestinal motor disturbances. In conclusion, MTC should be considered when evaluating chronic diarrhea.

ARTIFACTS AFFECTING DUAL-ENERGY X-RAY ABSORPTIOMETRY MEASUREMENTS.

OBJECTIVE: To present 3 cases in which the presence of artifacts altered the dual-energy X-ray absorptiometry (DXA) measurements of bone mineral density (BMD) in the lumbar field. METHODS: Clinical presentation and literature review. RESULTS: The first patient was a 49-year-old premenopausal woman with a spinal cord stimulator implanted in her lumbar spine. The implant increased the measured BMD in the lumbar region from osteopenia to normal range. The second patient was a 56-year-old man who had a DXA scan after he was administered oral and intravenous iodine-based contrast earlier the same day. The oral contrast in the patient's colon created a dense artifact, which overlapped the lumbar spine and adjacent soft tissue. The effect almost changed his DXA diagnosis from osteoporosis to osteopenia. The third patient was a 64-year-old woman who had undergone laparoscopic adjustable gastric banding (LAGB). A part of the LAGB system adjacent to lumbar vertebra 3 was mistakenly included as part of the vertebrae by the DXA scanner, causing an increase in the measured BMD. CONCLUSION: There are numerous artifacts that can alter DXA BMD measurement. It is important for providers who interpret DXA scans to be familiar with the potential effects of artifacts and how to adjust for their presence, since erroneous measurements can lead to incorrect decisions regarding treatment and follow-up.

An Atypical HNF4A Mutation Which Does Not Conform to the Classic Presentation of HNF4A-MODY.

OBJECTIVE: To present the case of an atypical Hepatocyte Nuclear Factor 4 Alpha (HNF4A) mutation that is not consistent with the classically published presentation of HNF4A-Mature Onset Diabetes of the Young (MODY). METHODS: Clinical presentation and literature review. RESULTS: A 43-year-old nonobese man was referred to the endocrinology clinic for evaluation of elevated fasting blood glucose (FBG) measurements. Laboratory review revealed prediabetes and hypertriglyceridemia for the previous decade. Testing of autoantibodies for type 1 diabetes was negative. Genetic testing showed an autosomal dominant, heterozygous missense mutation (c.991C>T; p.Arg331Cys) in the HNF4A gene, which is correlated with HNF4A-MODY. Phenotypically, patients with an HNF4A-MODY tend to have early-onset diabetes, microvascular complications, low triglyceride levels, increased birth weight, fetal macrosomia, and less commonly neonatal hyperinsulinemic hypoglycemia. The patient did not demonstrate any of these features but instead presented with late-onset diabetes, an elevated triglyceride level, and a normal birth weight. CONCLUSION: Our patient likely represents an atypical variant of HNF4A-MODY with a milder clinical presentation. Patients with atypical, less-severe presentations of HNF4A-MODY may be largely undiagnosed or misdiagnosed, but identification is important due to implications for treatment, pregnancy, and screening of family members.