Incidence and Overall Survival of Malignant Ameloblastoma

BackgroundMalignant ameloblastoma, comprising metastasizing ameloblastoma and ameloblastic carcinoma, represents 1.6–2.2% of all odontogenic tumors. Due to its rare nature, malignant ameloblastoma has only been reported in the literature in small case series or case reports. Using the Surveillance, Epidemiology and End-Results (SEER) database, we have performed a population-based study to determine the incidence rate and the absolute survival of malignant ameloblastoma.MethodUsing the International Classification of Diseases for Oncology (ICD-O) codes 9310/3 and 9270/3, data from the SEER database were used to calculate the incidence rate and absolute survival rate of population with malignant ameloblastoma.ResultsThe overall incidence rate of malignant ameloblastoma was 1.79 per 10 million person/year. The incidence rate was higher in males than females and also higher in black versus white population. The median overall survival was 17.6 years from the time of diagnosis and increasing age was associated with a statistically significant poorer survival.ConclusionsTo our best knowledge, we report the largest population-based series of malignant ameloblastoma. The incidence rate was 1.79 per 10 million person/year and the overall survival was 17.6 years.     

Giant Pancreatic Tumor With Clinical Characteristics of Insulinoma But Without Common Pathologic Features

ObjectiveTo report a case of a large pancreatic tumor that had clinical characteristics of an insulinoma without classic pathologic features.MethodsWe describe a 58-year-old woman who presented with a 3-month history of symptomatic hypoglycemic episodes, which were characterized by confusion. The laboratory, imaging, and pathologic findings are summarized, the current literature on giant insulinomas is reviewed, and the distinction between clinical and pathologic diagnosis of neuroendocrine tumors is discussed.ResultsThe biochemical diagnosis of insulinoma was established with concomitant low fasting blood glucose concentrations and inappropriately high insulin levels. An abdominal computed tomographic scan revealed a mass (10 by 11.7 by 9.7 cm) in the head and body of the pancreas, which was resected. Pathologic examination revealed a massive neuroendocrine tumor (13.5 by 11 by 8 cm) without immunohistochemical evidence of insulin expression. Nevertheless, tumor resection resulted in decreased blood insulin levels and resolution of the patient’s hypoglycemia.ConclusionAlthough more than 95% of insulinomas are smaller than 3 cm, this case is unique in that the extremely large pancreatic tumor had clinical characteristics of an insulinoma but did not have the classic pathologic findings. Because of the extensive pancreatic resection, the patient is dependent on both insulin and orally administered pancreatic enzymes but remained free of symptoms and disease recurrence at 1-year follow-up. (Endocr Pract. 2011;17:e12-e16)     

Metastatic Renal Cell Carcinoma to the Thyroid Gland

ObjectiveTo examine the presentation, diagnosis, and appropriate management of renal clear cell carcinoma metastasis to the thyroid gland.MethodsWe describe a clinical case of solitary thyroid metastasis from renal clear cell carcinoma and present a comprehensive review of the related English-language literature. Common patterns of presentation and generalized overall management recommendations are evaluated and summarized.ResultsEight years after nephrectomy for renal carcinoma at age 61 years, a man presented with a thyroid mass. Cytology and histopathologic surgical findings were consistent with a solitary metastasis most compatible with metastatic clear cell carcinoma from his previous renal carcinoma. After left thyroid lobectomy and isthmusectomy, the patient remains disease-free 5 years later. Although uncommon, nearly 150 cases of clinically recognized metastatic renal cell carcinoma to the thyroid have been reported in the English-language literature. Metastatic disease from the kidney to the thyroid gland can occur more than 20 years after nephrectomy with the average time interval being 7.5 years. Obtaining a full clinical history in any patient who presents with a thyroid nodule is essential to allow consideration of possible metastatic disease from previous primary tumor. Metastatic disease to the thyroid gland can be correctly diagnosed preoperatively. If metastatic renal cancer is limited to the thyroid gland only, prompt, appropriate surgical intervention can be curative.ConclusionMetastatic renal carcinoma to the thyroid should be considered in any patient presenting with a thyroid mass and a medical history of renal cell carcinoma. (Endocr Pract. 2008;14:1040-1046)     

Reconstruction with Modular Hemipelvic Endoprosthesis after Pelvic Tumor Resection: A Report of 50 Consecutive Cases

PurposeTo evaluate the effectiveness of reconstruction with a modular hemipelvic endoprosthesis after pelvic tumor resection.MethodsWe retrospectively studied 50 consecutive patients diagnosed with pelvic tumor from 2003 to 2013. All patients received limb-salvage surgery and reconstruction with modular hemipelvic endoprosthesis.ResultsPatients were followed for an average of 54 months. At the most recent follow-up, 32 patients were alive with an estimated three-year and five-year survival rate of 66.3% and 57.5% according to the Kaplan-Meier survival analysis. Eighteen patients died from the tumor, with a mean survival of 28 months, and 9 patients experienced local recurrence at an average of 19.6 months after surgery. Patients with marginal or intracapsular surgical margins had a significantly higher recurrence rate than those with wide margins (p=0.02). Metastasis occurred in 12 cases at an average of 16 months after surgery. The perioperative complication rate was 48.0%, and the most common complications were wound healing disturbance (28.0%) and deep infection (14.0%). The endoprosthetic complication rate was 16.0%, and breakage of the pubic connection plate was the most common complication. The mean Musculoskeletal Tumor Society score was 61.4%.ConclusionReconstruction with a modular hemipelvic endoprosthesis after pelvic tumor resection can improve function, with an acceptable complication rate.     

Activation of Wnt signalling reduces the population of cancer stem cells in ameloblastoma

ObjectivesThe treatment of ameloblastoma, an odontogenic epithelial tumour destroying jawbone, mainly depends on radical destructive resections. Other therapeutic options are limited by the characteristics of ameloblastoma, such as high recurrence rates and resistance to radiation and chemotherapy, which implies possible existence of cancer stem cells (CSCs) in ameloblastoma. Here, we identified a putative CSC population in immortalized and primary human ameloblastoma cells and examined possible therapeutic reagents to reduce the CSC population.MethodsWe investigated subpopulations of AM‐1 cell line and human ameloblastoma cells using immunocytochemistry and flow cytometry and the effects of Wnt signalling activators on the 2‐ and 3‐dimensional cultured ameloblastoma cells using molecular biological analyses.ResultAmong heterogenous ameloblastoma cells, small‐sized and round‐shaped cells were found to be proliferative and expressed a marker of dental epithelial stem cells, SRY‐box 2 (Sox2). Exogenous activation of Wnt signalling using glycogen synthase kinase 3β inhibitors, lithium chloride (LiCl) and valproic acid (VPA), increased the cell size and decreased proliferation of cells and expression of Sox2 in 2 dimensionally cultured AM‐1 and human primary ameloblastoma cells. Furthermore, the growth of 3 dimensionally cultured AM‐1 cells as suspended or embedded in gel was suppressed by treatment with Wnt signalling activators, VPA and CHIR99021, or antibodies to sclerostin, an antagonist of Wnt signalling.ConclusionWe suggest that Wnt signalling activators are potential drug candidates to suppress CSCs in ameloblastoma.     

Surgical resection as a predictor of cancer-specific survival by stage at diagnosis and cancer type,United States, 2006–2015

BackgroundWhen solid tumors are amenable to definitive resection, clinical outcomes are generally superior to when those tumors are inoperable. However, the population-level cancer survival benefit of eligibility for surgery by cancer stage has not yet been quantified.MethodsUsing Surveillance, Epidemiology and End Results data allowing us to identify patients who were deemed eligible for and received surgical resection, we examined the stage-specific association of surgical resection with 12-year cancer-specific survival. The 12-year endpoint was selected to maximize follow-up time and thereby minimize the influence of lead time bias.ResultsAcross a variety of solid tumor types, earlier stage at diagnosis allowed for surgical intervention at a much higher rate than later-stage diagnosis. At every stage, surgical intervention was associated with a substantially higher rate of 12-year cancer-specific survival, with absolute differences of up to 51% for stage I, 51% for stage II, and 44% for stage III cancer, and stage-specific mortality relative risks of 3.6, 2.4, and 1.7, respectively.ConclusionsDiagnosis of solid cancers in early stages often enables surgical resection, which reduces the risk of death from cancer. Receipt of surgical resection is an informative endpoint that is strongly associated with long-term cancer-specific survival at every stage.     

Diagnostic and Therapeutic Approach to Pituitary Incidentalomas

ObjectiveTo review data on epidemiology, differential diagnosis, clinical, laboratory, and imaging findings, natural history, and management of incidentally discovered pituitary lesions (pituitary incidentalomas).MethodsA nonsystematic review was conducted, including articles indexed in Index Medicus that contained reference to incidentally discovered pituitary masses (pituitary incidentalomas).ResultsBoth autopsy and sensitive neuroimaging studies (including magnetic resonance imaging) suggest that pituitary incidentalomas are common, affecting approximately 10% of the general population. Although typically small (less than 10 mm in greatest diameter) and clinically silent, some pituitary incidentalomas may be hormonally active or cause mass effects by compressing neighboring structures. In addition, a minority of these lesions may grow over time; hence, long-term follow-up is necessary. Therapeutic interventions, including dopamine agonist therapy (in the case of prolactin-secreting adenomas) or transsphenoidal resection, are indicated in the case of pituitary lesions that are hormonally active, cause mass effects, or increase in size.ConclusionPituitary incidentalomas are common and constitute a heterogeneous group with regard to pathologic features, clinical, laboratory, and imaging characteristics, natural history, and growth potential. Currently available evidence suggests that many hormonally nonfunctioning pituitary incidentalomas causing no mass effects can be safely managed by follow-up surveillance. Nonetheless, more data are needed for further elucidation of the natural history of these lesions and for improvement in accurate and noninvasive diagnosis and in prediction of growth potential of pituitary incidentalomas. Improved understanding of the pathogenesis of this heterogeneous group of lesions may also lead to the development of novel, noninvasive therapeutic agents, rationally designed to interact with well-characterized molecular targets. (Endocr Pract. 2004;10:438-444)     

Metastatic Struma Ovarii Treated With Total Thyroidectomy and Radioiodine Ablation

ObjectiveTo present a rare case of metastatic struma ovarii, review the related literature, and discuss the management.MethodsA case report of a patient with metastatic struma ovarii is presented. The treatment plan, postoperative care, and follow-up are discussed. We conducted a MEDLINE search of the English-language literature seeking additional cases of metastatic struma ovarii. In addition to the current case, the previous 40 cases of metastatic struma ovarii were reviewed and analyzed.ResultsThe mean age of the patients at presentation was 43 years. All patients underwent resection of the primary tumor. The most common sites of involvement for struma ovarii metastatic disease have been the peritoneum, mesentery, and omentum. After primary tumor resection, a wide range of additional treatments have been used, including chemotherapy, resection of metastatic disease, external beam radiation therapy, and radioiodine ablation.ConclusionIn cases of metastatic struma ovarii, we recommend total thyroidectomy in conjunction with radioiodine scanning and radioiodine ablation. Thyroglobulin levels should be followed as a tumor marker, and diagnostic radioiodine scans should be performed to screen for residual or recurrent disease. Although this treatment strategy is well established for thyroid cancer, long-term outcomes of this treatment for struma ovarii are still unknown. (Endocr Pract. 2009;15:167-173)     

Paraganglioma of the Head and Neck: A Review

ObjectiveTo review the epidemiology, presentation, diagnosis, and management of head and neck paragangliomas.MethodsA literature review of english language papers with focus on most current literature.ResultsParagangliomas (PGLs) are a group of neuroendocrine tumors that arise in the parasympathetic or sympathetic ganglia. Head and neck PGLs (HNPGLs) comprise 65% to 70% of all PGLs and account for 0.6% of all head and neck cancers. The majority of HNPGLs are benign, and 6% to 19% of all HNPGLs develop metastasis outside the tumor site and significantly compromise survival. PGLs can have a familial etiology with germline sequence variations in different susceptibility genes, with the gene encoding succinate dehydrogenase being the most common sequence variation, or they can arise from somatic sequence variations or fusion genes. Workup includes biochemical testing to rule out secretory components, although it is rare in HNPGLs. In addition, imaging modalities, such as computed tomography and magnetic resonance imaging, help in monitoring in surgical planning. Functional imaging with DOTATATE-positron emission tomography, 18F-fluorodeoxyglucose, or 18F-fluorohydroxyphenylalanine may be necessary to rule out sites of metastases. The management of HNPGLs is complex depending on pathology, location, and aggressiveness of the tumor. Treatment ranges from observation to resection to systemic treatment. Similarly, the prognosis ranges from a normal life expectancy to a 5-year survival of 11.8% in patients with distant metastasis.ConclusionOur review is a comprehensive summary of the incidence, mortality, pathogenesis, presentation, workup and management of HNPGLs.     

Genetics,Diagnosis, and Management of Medullary Thyroid Carcinoma and Pheochromocytoma/Paraganglioma

ObjectiveMedullary thyroid carcinoma (MTC) and pheochromocytoma/paraganglioma (PHEO/PGL) are rare neuroendocrine tumors. Because of the increased metastatic rates in certain genetic backgrounds, early diagnosis and treatment are essential to improved patient outcomes. Our objective was to summarize recent findings related to the genetics, diagnosis, and management of MTC and PHEO/PGL.MethodsA literature review was performed.ResultsMTC is primarily associated with mutations in the rearranged during transfection (RET) protooncogene. Determining the specific genetic mutation can guide patient management and screening. Early detection and appropriate surgical management of MTC is critical to prevent or limit metastatic spread, as treatment options for patients with metastatic disease are limited. PHEO/PGL also has a strong genetic component, with approximately 50% of cases linked to germline and somatic mutations in 15 genes. Although most PHEO/PGLs are benign, factors such as genetic background, size, tumor location, and high methoxytyramine levels are associated with higher rates of metastatic disease. The state-of-the-art diagnosis and localization of PHEO/PGLs is based on measurement of plasma metanephrines and methoxytyramine and functional imaging studies. For both PHEO/PGL and MTC, surgery is the only curative treatment. Treatment options for patients with metastatic disease are limited.ConclusionAs genetic testing becomes more widely available, the diagnosis of MTC and PHEO/PGL will be made earlier due to routine screening of at-risk patients. In addition, continued advances in basic science, diagnostic methods, and imaging techniques will improve understanding of the pathogenesis of these diseases and facilitate the introduction of novel treatment strategies for patients with metastatic disease. (Endocr Pract. 2014;20:176-187)     

Pheochromocytoma: A Cause of St-Segment Elevation Myocardial Infarction,Transient Left Ventricular Dysfunction,and Takotsubo Cardiomyopathy

ObjectiveTo report the case of a patient with a pheochromocytoma and apical left ventricular dysfunction that resolved after surgical resection of the pheochromocytoma, to review the effects of catecholamines on myocyte function and the concept that takotsubo cardiomyopathy (TC) is caused by excess catecholamines, and to illustrate the difficulty in the management of an acute coronary syndrome (ACS) during a hypertensive crisis attributable to a pheochromocytoma.MethodsWe present the clinical history, physical findings, laboratory results, and imaging studies in a 60-year-old man with an ACS, TC, and an incidentaloma later diagnosed to be a pheochromocytoma. The association with TC and the pertinent literature are reviewed.ResultsA 60-year-old man was suspected of having myocardial ischemia on the basis of symptoms of paroxysmal chest pain extending to the left shoulder, diaphoresis, ST-segment elevation on an electrocardiogram, and elevated serial levels of cardiac enzymes. Coronary angiography did not reveal substantial coronary artery obstruction but detected ballooning of the apical, anterior, and inferior cardiac walls, consistent with TC. He had a history of labile hypertension and palpitations of 3 months’ duration. An adrenal mass detected on a prior computed tomographic scan and increased 24-hour urine catecholamine levels were consistent with a pheochromocytoma. Treatment with phenoxybenzamine was initiated, and he underwent a right adrenalectomy, which confirmed that the tumor was a pheochromocytoma and dramatically improved the patient’s condition.ConclusionPheochromocytomas manifest with labile blood pressures and should be considered in the differential diagnosis of ACS. This case also supports the concept that TC is caused by excess catecholamines. (Endocr Pract. 2012;18:e77-e80)     

Adyuvant fractionated radiotherapy after resection of intracranial hemangiopericytoma

AimReview of literature and adjuvant treatment in Hemangiopericytoma after complete resection.BackgroundIntracranial hemangiopericytoma (HPC) is an uncommon malignant vascular tumor arising from mesenchymal cells with pericytic differentiation. Surgery remains the mainstay treatment, and adjuvant radiation therapy appears to be appropriate for patients with high grade tumors or incomplete resection. We present our experience and review of the literature.Materials and methodsWe describe two cases of intracranial hemangiopericytoma located in the frontal lobe of the CNS. Both patients underwent complete tumor resection followed by adjuvant fractionated radiotherapy and completed treatment without interruptions.ResultsA local recurrence was observed in one of these cases and fractionated stereotactic radiotherapy was performed. Both patients are alive and disease has been under control up to date.ConclusionThe treatment of choice for intracranial hemangiopericytoma is a complete surgical resection as long as possible. Adjuvant radiotherapy of HPC can result in increased tumor control and should be considered as an effective treatment for patients with high grade or demonstrated residual tumor in the postoperative period. Salvage treatment using limited-field fractionated radiotherapy for local recurrence treatment is considered an acceptable option.     

The clinical efficacy study of treatment to Chiari malformation type I with syringomyelia under the minimally invasive surgery of resection of Submeningeal cerebellar Tonsillar Herniation and reconstruction of Cisterna magna

PurposeDiscuss the clinical efficacy of treatment to Chiari malformation type I with syringomyelia under the minimally invasive surgery of resection of Submeningeal Cerebellar Tonsillar Herniation and reconstruction of Cisterna magna.Methods130 Chiari malformation type I with syringomyelia patients, divided into treatment group, literature group and control group, were collected to be treated under the monitoring of ultrasound in the surgery.Results6 months after operation, the lesions were decreased or disappeared, the symptoms were relieved obviously. According to MRI and Mimics 17.0 software, the volumes of Cisterna magna increased distinctly (P < 0.001), the proportions of brain in foramen magnum region were decreased (P < 0.001). Assessed by CCOS scale and Tator methods, the improvement rates of treatment group were 97.7% and 94.6%, the literature group and control group were 82.2% and 77.8%, respectively.ConclusionThe efficacy of Chiari malformation type I with syringomyelia under the minimally invasive surgery of resection of Submeningeal Cerebellar Tonsillar Herniation and reconstruction of Cisterna magna is remarkable, and the complications are fewer. This surgery emphasizes recovery of tonsil of cerebellum and reconstruction of Cisterna magna and the circulation path of cerebrospinal fluid, which is a safe and efficient treatment.     

Intercellular signaling between ameloblastoma and osteoblasts

Ameloblastoma is an odontogenic tumor located in the bone jaw with clinical characteristics of extensive bone resorption. It is a locally invasive tumor with a high recurrence rate despite adequate surgical removal. In bone disease, tumors and other cells including osteoblasts, osteoclasts, and osteocytes in the bone microenvironment contribute to the pathogenesis of tumor growth. However, the effect of osteoblasts on ameloblastoma cells is not well-understood, and there has been limited research on interactions between them.This study investigated interactions between ameloblastoma cells and osteoblasts using a human ameloblastoma cell line (AM-3 ameloblastoma cells) and a murine pre-osteoblast cell line (MC3T3-E1 cells). We treated each cell type with the conditioned medium by the other cell type. We analyzed the effect on cytokine production by MC3T3-E1 cells and the production of MMPs by AM-3 cells. Treatment with AM-3-conditioned medium induced inflammatory cytokine production of IL-6, MCP-1, and RANTES from MC3T3-E1 cells. The use of an IL-1 receptor antagonist suppressed the production of these inflammatory cytokines by MC3T3-E1 cells stimulated with AM-3-conditioned medium. The MC3T3-E1-conditioned medium triggered the expression of MMP-2 from AM-3 cells. Furthermore, we have shown that the proliferation and migration activity of AM-3 cells were accelerated by MC3T3-E1 conditioned media.In conclusion, these intercellular signalings between ameloblastoma cells and osteoblasts may play multiple roles in the pathogenesis of ameloblastoma.     

Pancreatoblastoma in children: Clinical management and literature review

PurposeThe aim of this study is to analyze the clinical and pathological features of pancreatoblastoma (PB) and to obtain better management for patients with relapsed or metastatic disease.MethodsFour cases treated in our institution and 59 cases reported previously in the literature from the PubMed biomedical database (2000–2020) were reviewed and analyzed.ResultsFour cases with PB presented with abdominal pain and palpable abdominal masses, with the tumor size ranging from 5.2 to 18 cm in diameter. The invasion of the splenic vein and superior mesenteric artery, duodenum, and lymph nodes were risk factors for PB. Three cases were treated with combination therapy and showed favorable outcomes, while one case was treated with chemotherapy alone due to tumor progression and died of the disease. Squamous corpuscles were revealed in the tumor samples and considered a defining component for histological diagnosis.ConclusionsMultidisciplinary diagnosis plays an important role in clinical management. The risk factors should be considered in the therapeutic stratification of PB before surgery.     

Change in Adrenal Mass Size as a Predictor of a Malignant Tumor

ObjectiveTo assess the value of adrenal mass absolute growth, growth rate, and percentage growth rate on serial imaging for distinguishing benign from malignant adrenal masses.MethodsWe retrospectively reviewed the Cleveland Clinic medical record data on 136 adrenalectomies or biopsies in 132 patients with 2 imaging studies performed more than 2 months apart (during 1997 to 2008).ResultsThere were 111 benign (81.6%) and 25 malignant (18.4%) adrenal masses. With use of receiver operating characteristic curve analysis, all 3 aforementioned growth measures showed similar levels of discrimination for the entire study group as well as for the subgroups with 3 to 12 months of follow-up (n = 75 masses) and noncontrast computed tomography Hounsfield units > 10 or not reported (n = 111 masses). After adjustment for other factors, the 3 growth measures remained statistically significant predictors of a malignant tumor. The absolute growth cutoff value of 0.8 cm had the highest sum of sensitivity and specificity of 72% and 81.1%, respectively. We could not identify an adrenal mass growth cutoff value to provide 100% sensitivity or specificity to confirm or exclude the presence of a malignant lesion. In 3 patients with metastatic lesions, no growth or a decrease in mass size during a period of 4 to 36 months was observed.ConclusionIn this study, the largest with surgical histopathology findings as the “gold standard” for diagnosis, change in adrenal mass size was a significant predictor of a malignant tumor. Nevertheless, we could not identify an adrenal mass growth cutoff value for reliable confirmation or exclusion of a malignant lesion. Change in adrenal mass size should be used in conjunction with other imaging and clinical characteristics when surgical resection is being considered. (Endocr Pract. 2010;16:577-587)     

Coexistence of tumor-induced osteomalacia and primary hyperparathyroidism

ObjectiveTo present an unusual case of coexisting tumor-induced osteomalacia (TIO) and primary hyperparathyroidism (PHPT).MethodsWe report the clinical features, imaging studies, and the results of laboratory investigations before and after surgical resection of both a soft-tissue tumor and a parathyroid adenoma.ResultsA 44-year-old woman was referred to the endocrinology department with a diagnosis of PHPT accompanied by unusually severe hypophosphatemia, despite having received treatment with cinacalcet. Debilitating muscle weakness and bone pain, severe phosphaturia and hypophosphatemia, inappropriately normal calcitriol, and elevated fibroblast growth factor-23 and intact parathyroid hormone levels raised the suspicion of coexisting TIO and PHPT. Imaging studies were negative, but histologic characteristics of a palpable subcutaneous mass from the patient’s thigh revealed a phosphaturic mesenchymal tumor. Complete remission after surgical removal of both the soft-tissue tumor and the parathyroid adenoma confirmed the diagnosis.ConclusionThe coexistence of TIO and PHPT has not been described before and can cause life-threatening hypophosphatemia. Diagnosis and localization of the tumor is of paramount importance since surgery is the treatment of choice for both TIO and PHPT. (Endocr Pract. 2011;17:e144-e148)     

Concomitant Secretion of Glucocorticoid,Androgens, and Mineralocorticoid by an Adrenocortical Carcinoma: Case Report and Review of Literature

ObjectiveTo present a case of concomitant secretion of cortisol, androgens, and 11-deoxycorticosterone (DOC) by an adrenocortical carcinoma and review the literature in an attempt to identify similar cases.MethodsThe patient’s medical history, physical examination, laboratory data, computed tomographic scan, and histopathologic results were analyzed and summarized in a case report, and an extensive review of the literature was performed.ResultsEndocrinologic data showed excess cortisol production, substantially elevated testosterone and androstenedione levels, and profoundly increased DOC in the setting of suppressed aldosterone. An abdominal computed tomographic scan showed a left adrenal tumor. A left adrenalectomy was performed, and the histopathologic diagnosis was stage II adrenocortical carcinoma. The review of the pertinent literature revealed the absence of any identical cases in the past.ConclusionOur patient presented with a rare case of cosecretion of cortisol, testosterone, androstenedione, and DOC by an adrenocortical carcinoma, resulting in a clinical picture consistent with Cushing’s syndrome, hyperandrogenism, and primary hypermineralocorticoidism. We recommend the routine performance of a DOC assay in the setting of mineralocorticoid excess in association with low plasma aldosterone levels. (Endocr Pract. 2007;13: 408-412)     

Bronchogenic Cyst Masquerading As an Adrenal Tumor: A Case of Mistaken Identity

ObjectiveTo describe a patient with a bronchogenic cyst that was erroneously diagnosed as an adrenal tumor and the surgical management strategy to address the operative challenges.MethodsWe summarize the clinical presentation, diagnostic workup, surgical management, and pathologic features of the study patient and review the pertinent literature.ResultsIn this report, we present the case of a 23-year-old woman who underwent retroperitoneoscopic exploration after imaging identified an enlarging left adrenal lesion. Preoperative biochemical testing confirmed that the mass was nonfunctional. No lesion was found after a thorough retroperitoneoscopic exploration under standard high insufflation pressure. Serendipitously, low-pressure inspection for hemostasis after failed exploration enabled discovery of an intradiaphragmatic mass that proved to be a bronchogenic cyst rather than an adrenal tumor. Not only was this a difficult operative dilemma, but it was also an unusual presentation for this tumor.ConclusionsDiscovery of a retroperitoneal or intradiaphragmatic bronchogenic cyst is a rare occurrence. The unusual location and tumor characteristics contributed to near surgical failure. The fortuitous surgical strategy of lowpressure inspection allowed visualization of the tumor for definitive resection. (Endocr Pract. 2012;18:e102-e105)     

Expression Analysis of Fibroblast Growth Factor-23, Matrix Extracellular Phosphoglycoprotein,Secreted Frizzled-Related Protein-4, and Fibroblast Growth Factor-7: Identification of Fibroblast Growth Factor-23 and Matrix Extracellular Phosphoglycoprotein as Major Factors Involved in Tumor-Induced Osteomalacia

ObjectiveTo evaluate the expression of various phosphaturic factors in the tumor of a patient with tumorinduced osteomalacia (TIO) and to analyze serum levels of fibroblast growth factor (FGF)-23 in TIO and healthy subjects.MethodsWe measured serum FGF-23 levels in 2 patients with TIO and 6 healthy volunteers. Expression of FGF-23, matrix extracellular phosphoglycoprotein (MEPE), FGF-7, and secreted frizzled-related protein-4 (sFRP-4) was analyzed in a hemangiopericytoma from a patient with TIO, in a hemangiopericytoma from a patient without TIO, and in various control cell lines.ResultsSerum FGF-23 levels were substantially higher in patients with TIO in comparison with those in healthy control subjects and normalized with successful resection of the tumor. Tissue expression analysis showed preferential expression of FGF-23 and MEPE in TIO related hemangiopericytoma, whereas FGF-7 and sFRP-4 were widely expressed in all studied cell lines and tissues.ConclusionThese results support the use of FGF-23 measurements for the diagnosis and follow-up of patients with TIO. In addition, the specific expression of FGF-23 and MEPE in the TIO-associated tumor suggests an important role of these two phosphatonins in the pathogenesis of TIO. Because of the limited number of patients in our report, further studies are needed to clarify the role of different phosphatonins in the development of the clinical features of TIO. (Endocr Pract. 2008;14:1108-1114)