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Dextroposition is a rare cardiac malformation defined as heart shift to right of midline. ECG findings vary with degree of displacement within the chest cavity. We report the second known case of dextroposition with accessory pathway (posteroseptal in our patient), presenting as pre-excited atrial tachycardia. Abnormal anatomy complicates pathway localization/ablation. (Level of Difficulty: Intermediate.)

Background:. Patient-reported outcomes regarding sexual health are lacking or have not been validated for transgender patients following vaginoplasty. The aim of this study is to further characterize the difference in sexual health, genital self-image, and the relationship between them for patients who were pre- and postvaginoplasty. Methods:. A community advisory board informed an anonymous online survey utilizing patient-reported outcomes. Pre- and postvaginoplasty respondents were recruited online. Survey measures included the Female Genital Self-Image Scale (FGSIS) and the Patient-Reported Outcomes Measurement Information System sexual health measures. Welch approximation t tests were performed for FGSIS and Patient-Reported Outcomes Measurement Information System questions, using Bonferroni correction. Results:. A total of 690 respondents prevaginoplasty (n = 525; 76%) and postvaginoplasty (n = 165; 24%) participated. The postoperative cohort, compared with the preoperative cohort, reported higher scores for orgasm (P = 0.0003), satisfaction (P = 0.001), and pleasure (P = 0.002). FGSIS total score was higher among postoperative respondents (79.4% ± 17.1%) than preoperative respondents (50.6% ± 15.1%) (P < 0.0001). Using Spearman rho, no significant correlation between FGSIS total score and any Patient-Reported Outcomes Measurement Information System subsectional measures was observed for the postoperative cohort, but a correlation (P

Objective: We report a 49-year-old woman who had minimal features of Cushing syndrome and an incidentally discovered adrenal adenoma. She was subsequently diagnosed with pituitary-dependent Cushing syndrome. Methods: Laboratory and imaging studies including serum cortisol, plasma adrenocorticotrophic hormone (ACTH), high dose dexamethasone test, corticotropin-releasing hormone test, computed tomography (CT) scan, and magnetic resonance imaging were performed. Results: A 49-year-old woman was admitted for urosepsis. An abdominal CT scan performed during the urosepsis workup showed a 2.7-cm right adrenal adenoma. She denied any abdominal striae or other symptoms. Physical examination showed normal vital signs, minimal facial fullness without central obesity, and striae. Laboratory results were as follows: 24-hour-urine cortisol 294 μg (reference 4.0-50.0), midnight serum cortisol 23.0 μg/dL (reference < 7.5), and plasma ACTH level 39 pg/mL (reference 5-27). A corticotropin-releasing hormone stimulation test showed >20% rise in serum cortisol and >35% rise in ACTH levels. A pituitary magnetic resonance image showed a 5 mm pituitary lesion. The patient underwent transsphenoidal pituitary surgery, which confirmed an ACTH-secreting lesion. Postoperatively, she required hydrocortisone replacement for the next 10 months. A follow-up adrenal CT performed 6 months later showed a decrease in the size of the adrenal adenoma (1.8 cm). Conclusion: This case highlights the importance of recognizing the coexistence of ACTH-dependent Cushing disease with an adrenal adenoma and partial ACTH dependency of the adrenal adenoma.

The placement of wireless mesh routers is a significant matter in enhancing the performance of a wireless mesh network. Therefore, it is essential that the mesh routers are optimally placed to guarantee strong connectivity and coverage for ensuring the best network accessibility. The importance of this issue has motivated the researchers to pursue more investigation on optimizing the networks’ performance. Various optimization algorithms have been developed in the literature to identify the trade-offs between network connectivity and client coverage. This work is aimed at implementing a novel application of Harris Hawk’s Optimization (HHO) algorithm to optimally place mesh routers in a wireless mesh network so as to improve connectivity and coverage. The HHO seeks optimal routers placement that leads to maximizing the connection between routers and increasing the number of covered clients. In this study, three network configurations are used with sizes of 40, 60, and 100 mesh routers. The simulation results are statistically analyzed and compared to other population-based algorithms such as Sine Cosine Algorithm (SCA), Gray Wolf’s Optimization (GWO), and Particle Swarm Optimization (PSO). Moreover, the performance of HHO is compared with the state-of-the-art to validate the effectiveness of the proposed approach. The simulation findings and statistical analysis confirm that the HHO outperforms the other algorithms in terms of network coverage and connectivity. The outcomes demonstrate that HHO can be considered an efficacious means to enhance the performance of wireless mesh networks (WMNs).

Objective: Diabetes insipidus (DI) can be classified into 2 types: central/neurogenic DI and nephrogenic DI. Most cases of central DI occur after brain surgery, trauma, tumor, or infection. Here we report a rare case of familial central DI due to a heterozygous AVP gene mutation. Methods: A case of familial neurogenic DI has been described with thorough clinical, laboratory, and genetic workup. PubMed and Google scholar databases were used for literature discussion. Results: A 22-year-old man presented with polyuria and polydipsia. He drank about 4 gallons of water everyday and urinated large volumes very frequently. His physical examination was unremarkable. After 2 hours of water-deprivation, his serum sodium level was 147 mmol/L, serum osmolality was 302 mOsm/kg with concurrent urine osmolality of 78 mOsm/kg, vasopressin level was

Objective: Severe hypertriglyceridemia carries increased health risks, including the development of pancreatitis. The objective of this study was to report on management of 2 cases with severe gestational hypertriglyceridemia. Cases: In case 1, a 33-year-old pregnant woman presented with serum triglyceride level of 14 000 mg/dL after discontinuing hypolipidemic medications. She was treated with Lovaza 12 g/day, and serum triglyceride remained near normal at level of less than 800 mg mg/dL until delivery. In case 2, a 28-year-old patient (29th week gestation) presented with acute pancreatitis and triglycerides >4000 mg/dL. She was treated with Gemfibrozil, Lovaza, insulin infusion, subcutaneous heparin, and escalated to plasmapheresis. She successfully delivered a baby at the week of 36th and her triglyceride level was 304 mg/dL after that. Discussion: Case 1 was treated with high-dose Lovaza and case 2 was treated with plasmapheresis successfully. Triglyceride levels were reduced to less than 500 mg/dL until delivery of healthy babies in both cases. Conclusion: Omega-3 fatty acids and plasmapheresis may be effective and safe to treat pregnant women with severe hypertriglyceridemia and pancreatitis.

Objectives: Renal papillary necrosis (RPN) occurring in primary hyperparathyroidism (PHPT) has not been reported. We present a 50-year-old woman who manifested RPN associated with hypercalciuria and normocalcemic PHPT. Methods: The diagnosis of RPN was based on imaging studies (ultrasound and computed tomography [CT] scan). PHPT was diagnosed with high parathyroid hormone (PTH) and high/normal serum calcium. Results: A 38-year-old woman was evaluated for hypercalcemia (serum calcium, 11.8 mg/dL; ionized calcium, 6.3 mg/dL; phosphorus, 1.8 mg/dL; intact PTH, 98 pg/mL; and 24-hour urine calcium, 543 mg). Renal ultrasound showed no nephrocalcinosis or nephrolithiasis. A parathyroid scan revealed a left parathyroid adenoma. The patient underwent parathyroidectomy, and she became normocalcemic with normal serum PTH levels postoperatively. One year later, she was diagnosed with a left-sided bronchial carcinoid tumor. Following surgery, a surveillance gallium68 positron emission tomography/CT scan performed 2 years later was negative for metastases. Twelve years later (aged 50 years), she presented for follow-up and reported no symptoms of hypercalcemia, fractures, nephrolithiasis, history of pyelonephritis, diabetes mellitus, analgesic drug use, or hypertension. Her serum calcium level was 9.1 mg/dL, PTH level was 82 pg/mL, 25-OH vitamin D level was 34 ng/mL, and 24-hour urine calcium level was 410 mg. However, renal ultrasound showed bilateral RPN that was confirmed by a CT scan. Conclusion: RPN may be associated with hypercalciuria and normocalcemic PHPT. Additional studies with a large number of patients are needed.

Objective: To evaluate a rare case of a postmenopausal woman with hirsutism and virilization due to Leydig cell tumors (LCTs) of both ovaries. Methods: In this challenging case, the diagnostic studies included the detection of total/free testosterone, hemoglobin, and estradiol levels; adrenal computed tomography; and pelvic magnetic resonance imaging. Results: A 61-year-old woman presented for the evaluation of hirsutism. Physical examination revealed normal vital signs and evidence of virilization. The baseline laboratory findings were hemoglobin level of 16.2 g/dL (reference, 12.0-15.5 g/dL), total testosterone level of 803 ng/dL (reference, 3-41 ng/dL), and free testosterone level of 20.2 pg/mL (reference, 0.0-4.2 pg/mL). Pelvic magnetic resonance imaging showed bilateral homogeneous ovarian enhancement. Based on the magnetic resonance imaging findings and clinical presentation, the patient was diagnosed with ovarian hyperthecosis and underwent laparoscopic bilateral oophorectomy. Pathology confirmed LCTs in both ovaries. Six months later, testosterone levels normalized, with significant improvement in hirsutism and virilization. Conclusion: Clinicians should be aware of androgen-secreting tumors, including rare bilateral LCTs in postmenopausal women presenting with progressing hirsutism and virilization. Marked hyperandrogenemia with total testosterone level of >150 ng/dL (5.2 nmol/L) or serum dehydroepiandrosterone sulfate level of >700 μg/dL (21.7 mmol/L) is typically found. It should be recognized that diffuse stromal Leydig cell hyperplasia and small LCTs may be missed on imaging, and in some cases only pathology can confirm the result.

ABSTRACT: Objective: Pheochromocytoma is diagnosed biochemically by demonstrating an excessive production of catecholamines and their metabolites in the blood and urine. However, these tests are at times fraught with false-positive results due to drug effects. We report here a patient with markedly elevated urinary metanephrines associated with the use of eletriptan for migraine treatment. Methods: A literature search was conducted using the PubMed and Google Scholar databases for eletriptan and false positive metanephrine elevation. Urine and plasma metanephrine tests were performed via liquid chromatography/tandem mass-spectrometry. Results: A 29-year-old man with migraine recently started on eletriptan was evaluated for a worsening headache. Initially his blood pressure was 220/160 mm Hg with a creatinine of 1.9 mg/dL. He was treated with intravenous nicardipine. His lab tests showed normal aldosterone/plasma renin activity ratio, midnight salivary cortisol, thyroid function, and urinary drug screen. A 24-hour urine metanephrine level at 2,494 μg (normal, 45 to 290 μg) and normetanephrine level at 1,341 μg (normal, 82 to 500 μg) for secondary hypertension work-up were markedly elevated. In contrast, plasma metanephrines were at 27 pg/mL (normal, 0 to 62 pg/mL) and normetanephrines were at 255 pg/mL (normal, 0 to 145 pg/mL) were only mildly elevated. Adrenal CT and gallium-68 positron emission tomography/computed tomography showed no abnormalities. Within 1 week of eletriptan discontinuation, his urine and plasma metanephrine and normetanephrine levels completely normalized as well as a reduction of blood pressure (130’s/80’s mm Hg). Conclusion: The discrepancy between plasma and urine studies in our patient suggests the possibility of false positive tests. It is possible that eletriptan may affect the urine assays, but the exact mechanism causing elevated urine metanephrines/normetanephrines is not clear. Abbreviations: CYP3A4 cytochrome P450 3A4 5-HT 5-hydroxytryptamine

ABSTRACT: Objective: To present a case of pyknodysostosis (PKND), a rare genetic cause of skeletal dysplasia that often goes undiagnosed even in patients with classic features. Methods: We report a case of PKND that went undiagnosed over many years despite classic features. We performed physical examination, imaging studies, and genetic testing on the patient. Results: A 21-year-old female presented to endocrinology to establish care. On evaluation, she was noted to have disproportionate short stature and a past medical history notable for bilateral blindness due to optic atrophy secondary to bone enlargement and thickening of the optic nerve canal before age 7 years. She also had a history of foot fractures occurring with ambulation. Her family history was significant for consanguineous parents and relatives with similar clinical features. Physical examination revealed a short, 128-cm tall female with open anterior and mastoid fontanels, mild frontal bossing and micrognathia, evidence of double rows of teeth, and digits of varied length in both hands and feet. Plain radiographs demonstrated diffuse sclerosis and marked cortical thickening of the pelvis, femurs, metacarpals, proximal phalanges, and metatarsals as well as decreased phalangeal length and acro-osteolysis of the hands and feet. Dual energy X-ray absorptiometry demonstrated increased bone mineral density (z scores +2.5 lumbar spine, +3.7 femoral neck, +4.5 total hip). Genetic testing revealed a exon 5-homozygous mutation in the cathepsin K (CTSK) gene consistent with PKND. Conclusion: Patients with PKND come to medical attention for a variety of reasons but often go undiagnosed even when presenting with classic features due to the rarity of the condition and the overlap with other skeletal dysplasias. Abbreviations: CTSK cathepsin K PKND pyknodysostosis

ABSTRACT: Objective: The galactose-alpha-1,3-galactose (alpha-gal) allergy, an IgE-mediated response to nonprimate meat, has a singular pathogenesis linked to tick bites and a delayed allergic presentation, which makes it especially cumbersome to diagnose and manage. As a large array of enteral medications contain ingredients derived from meat byproducts, this can affect the care of alpha-gal patients across multiple medical disciplines. Our objective is to present a patient with an alpha-gal allergy, which can complicate hypothyroidism treatment selection. Methods: In this case of difficulty tailoring thyroid replacement therapy secondary to the alpha-gal allergy, the diagnostic studies included IgE immunoassays for alpha-gal as well as thyroid function tests. Results: A 45-year-old woman with postoperative hypothyroidism and a history of an alpha-gal allergy (diagnosed after an anaphylactic reaction to beef) could not be immediately started on any common thyroid hormone replacement formulation because of our concern regarding the possible presence of nonprimate mammalian meat byproduct components in the thyroid hormone medication. After consulting allergy and immunology specialists and compounding pharmacists and contacting multiple drug companies in an effort to confirm the nature of the inactive ingredients in their thyroid hormone products, she was prescribed a plant-based compounded levothyroxine preparation with good clinical results. Conclusion: This case emphasizes the importance of recognizing various risk factors and common drugs which may be associated with the alpha-gal allergy. It is not known how to best tailor enteral medications for patients with an alpha-gal allergy. Further research and pharmaceutical attention to this allergy are needed.

ABSTRACT: Objective: Antiphospholipid syndrome (APS) can involve multiple organ systems but endocrine manifestations are rare. In most cases adrenal insufficiency (AI) is the first endocrine manifestation of APS. The prompt diagnosis of AI is critical as this disorder is a life-threatening disease that may lead to fatal outcomes if left untreated. We present a case of AI associated with APS the patient was diagnosed promptly and managed successfully. Methods: The diagnosis of APS was based on a combination of clinical features (deep venous thrombosis and pulmonary embolism) and laboratory findings (lupus anticoagulant, anticardiolipin antibody, anti-beta-2 glycoprotein-I antibody), without alternative diagnosis to explain the clinical findings. AI was diagnosed by low morning serum cortisol with elevated adrenocorticotropic hormone (ACTH) level as well as an ACTH stimulation test. Results: A 50-year-old male presented with deep venous thrombosis of the left extremity diagnosed by compressive ultrasound, and was subsequently diagnosed with a pulmonary embolism by computed tomography angiography and treated with heparin. Two days later, he developed hypotension and bilateral flank pain, and an abdominal computed tomography scan revealed bilateral adrenal hemorrhage. Laboratory results showed a serum cortisol of 3.3 mcg/dL (stress normal, 25 to 35 mcg/dL) and ACTH of 319 pg/mL (stress normal, 128 to 218 pg/mL), consistent with primary AI. Symptoms improved quickly with hydrocortisone therapy. The patient still required glucocorticoid therapy for at least 4 years thereafter. Conclusion: In all cases of adrenal hemorrhage and infarction with unknown etiology, screening with lupus anticoagulant and anticardiolipin antibodies is imperative. Recognition of this high-mortality condition allows for appropriate screening and confirmatory tests leading to a prompt diagnosis and timely management.

Background:. Abdominal-based autologous breast reconstruction remains a conflict between blood supply and donor site complication. Optimizing esthetics and minimizing recovery and postoperative pain add further complexity. We present a 2-stage technique of deep inferior epigastric artery perforator flap reconstruction to (1) reliably harvest single-vessel flaps while minimizing fat necrosis, (2) decrease abdominal wall morbidity, and (3) improve breast and donor site esthetics. Methods:. Female subjects presenting between August 2017 and January 2019 to the senior surgeon for abdominal-based breast reconstruction were included. After mastectomy, the subjects underwent subcutaneous placement of tissue expanders and in situ selection of a low, centrally located perforator based on preoperative computed tomographic angiography imaging through an infraumbilical “T” incision with ligation of all other perforators and superficial system. Subjects underwent tissue expander explant and flap transfer at a second stage. Results:. One hundred thirty-five subjects undergoing 215 free flaps met criteria. Mean age and body mass index were 52.1 years and 29.3 kg/m2, respectively. Seven perforator complications (3.3%) occurred with 2 (0.9%) total and 5 (2.3%) partial flap losses. There were 20 (14.8%) readmissions and 26 (19.3%) reoperations. Breast complications included arterial thrombosis (0.5%), venous congestion (1.9%), and fat necrosis (5.1%). The mastectomy skin flap necrosis rate decreased from 14.9% to 2.3% following staged reconstruction. Abdominal donor site complications included delayed healing (11.1%), seroma (5.9%), and hematoma (2.2%). Conclusions:. The 2-stage delayed deep inferior epigastric artery perforator flap technique represents a safe, efficacious modality to allow for reliable harvest of single-vessel flaps with low rates of fat necrosis while improving donor site esthetics and morbidity.

Purpose: Improvements in mental health and quality of life are well documented for transgender patients following vaginoplasty. Patient-reported outcomes (PROs) regarding sexual health are lacking in this intervention, or have not been validated in transgender patients. Methods: A community advisory board of transgender women informed an anonymous online survey utilizing PROs for those who were contemplative of (pre-) and post-vaginoplasty. They were recruited on online platforms not associated with an individual institution. Survey measures included the Female Genital Self-Image Scale (FGSIS) and Patient-Reported Outcomes Measurement Information System (PROMIS) sexual health measures. Welch approximation t-tests were performed for FGSIS and PROMIS questions using Bonferroni correction. Results: 687 transgender patients pre-vaginoplasty (n = 522, 76%) and post-vaginoplasty (n = 165, 24%) responded. In PROMIS measures, the postoperative cohort reported higher ability to orgasm (p = 0.0003), satisfaction with sex life (p = 0.001), pleasure in sex life (p = 0.002), use of lubricant (p < 0.0001), and scar interfere with sexual satisfaction (p < 0.0001). FGSIS total score was higher among postoperative patients (27.8 ± 6.0) than preoperative patients (17.8 ± 5.3) (p < 0.0001). Using Spearman’s rho, no significant correlation between FGSIS total score and any PROMIS sub-sectional measures was observed for either cohort. 193 patients (133 preoperative, 60 postoperative) reported their responses about sex being influenced by the Covid-19 crisis. Conclusion: Patients who are contemplating vaginoplasty have worse sexual health and genital self-image than those who have had vaginoplasty. FGSIS scores did not correlate with PROMIS sexual health measures when controlling for prior vaginoplasty, demonstrating that sexual health is multimodal for each individual patient.

ABSTRACT: 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.

ABSTRACT: Objective: Excess growth hormone (GH) secretion from a cystic sellar lesion is rare. Indeed, there have been few cases of hormone-secreting pituitary adenomas with a cystic component.Methods: We report a rare case of subclinical acromegaly that presented as a cystic sellar lesion on magnetic resonance imaging (MRI).Results: A 34-year-old Caucasian female presented with arthralgias, diaphoresis, paresthesias, cognitive slowing, headaches, presyncope, anxiety, and depression. She underwent evaluation by multiple providers without a diagnosis. Her physical examination was reportedly normal without evidence to suggest acromegaly. While she was undergoing workup for multiple sclerosis, a brain MRI scan revealed a cystic sellar lesion measuring approximately 1.6 × 0.9 cm approaching the optic chiasm. An insulin-like growth factor 1 level was incidentally screened months later and was elevated at 823 ng/mL (reference range is 69 to 227 ng/mL). A subsequent oral glucose tolerance test reported a growth hormone level of 7.5 ng/mL at its nadir (reference range is

Summary:. Vertical mandibular distraction results in translation of both proximal and distal segments. The force exerted on the condylar segment not only places unwanted force on the joint but also rotates the coronoid process into the cranial base. To prevent these sequelae, we investigate the use of a "check plate" on the condylar segment in an attempt to decrease force at the Temporomandibular joint (TMJ) and prevent unwanted rotation of the coronoid. Patients with hemifacial microsomia, seen at our Children's Hospital from 2012 to 2016 having undergone unilateral vertical mandibular distraction with placement of check plate were compared with a sample of those similarly having undergone distraction without use of the plate. Preoperative and postoperative cephalometric measures and 3-dimensional computed tomography imaging were analyzed. Three subjects were identified in each group. Age and Pruzansky-Kaban classification did not differ between groups. Vertical distance from the coronoid process perpendicular to the Frankfort Horizontal did not differ between groups (P < 0.07); however, postoperative distance significantly differed with the coronoid process rotating upward into the cranial base in subjects without a check plate (P < 0.005). Preoperative angle of the coronoid process based on the Frankfort Horizontal did not differ (P < 0.06); however, postoperative angle significantly changed, confirming upward rotation into the cranial base (P < 0.01). Total regenerate did not differ (P < 0.08). Vertical mandibular distraction results in undesirable upward rotation of the proximal segment into the cranial base and superior displacement of regenerate. This can be prevented with the use of a check plate.

ABSTRACT: Objective: There have been several case reports of hypercalcemia due to mutation in CYP24A1, which encodes an enzyme that controls the catabolism of 25-hydroxyvitamin D and 1,25-dihydroxyvitamin D to inactive forms. These patients develop nephrolithiasis, nephrocalcinosis, and eventually renal insufficiency. We describe a patient with hypercalcemia and nephrolithiasis due to CYP24A1 mutation with other co-existing factors for renal stone formation. Methods: A literature search was conducted using the PubMed and Google Scholar databases for CYP24A1 mutation and hypercalcemia. Publications were selected based the quality of the data and clinical relevance. Results: CYP24A1 mutation may lead to hypercalcemia, hypercalciuria, and renal stone formation; however, other risk factors for renal stone formation such as urinary citrate, sodium, oxalate, and uric acid levels need to be evaluated. Conclusions: This case highlights the need to evaluate for other renal stone risk factors in patients with CYP24A1 mutation.

ABSTRACT: Objective: The objective of this report is to describe a case of metastatic moderately differentiated neuroendocrine carcinoma (MDNC) of the larynx with markedly elevated serum calcitonin levels mimicking medullary thyroid carcinoma.Methods: We present the clinical, laboratory, and radiologic findings in a patient with an unusual clinical course of metastatic MDNC of the larynx, as well as a review of the relevant literature.Results: A 72-year-old male presented with an enlarging right neck mass. Positron emission tomography–computed tomography of the neck revealed enlarged right level III lymph nodes with compressive effect on the right internal jugular vein. Surgical resection of the lymph nodes was consistent with metastatic medullary carcinoma. Immunohistochemical staining was positive for cytokeratin, synaptophysin, chromogranin, and calcitonin, but negative for thyroid transcription factor-1 (TTF-1). No focus of medullary thyroid cancer or evidence of C-cell hyperplasia was identified after total thyroidectomy. Review of a prior vocal cord biopsy was consistent with neuroendocrine carcinoma; cells showed strong immunoreactivity for cytokeratin, synaptophysin, chromogranin, and calcitonin, and were negative for TTF-1, S100 protein, and cytokeratin 20. Based on these findings, the presentation was consistent with metastatic MDNC.Conclusion: The unique presentation emphasizes that identifying the origin of metastatic disease and understanding the immunohistochemical pattern is important in distinguishing between metastatic medullary thyroid cancer and MDNC of the larynx.Abbreviations: MDNC moderately differentiated neuroendocrine carcinoma; MTC medullary thyroid carcinoma; TTF-1 thyroid transcription factor-1