Unusual Cause of Ectopic Secretion of Adrenocorticotropic Hormone: Cushing Syndrome Attributable to Small Cell Prostate Cancer

ObjectiveTo report a rare cause of ectopic adrenocorticotropic hormone (ACTH) secretion leading to severe Cushing syndrome.MethodsWe describe the clinical presentation and management of a case of Cushing syndrome attributable to ectopic ACTH secretion from small cell cancer of the prostate.ResultsIn a 70-year-old man with hypertension and diabetes, congestive heart failure developed. He was found to have severe hypokalemia (serum potassium, 1.7 mEq/L) and a huge pelvic mass on a computed tomographic scan performed because of a complaint of urinary retention. Transurethral biopsy of the prostate showed features of small cell prostate cancer. Hormonal evaluation revealed a high urine free cortisol excretion of 6,214.5 μg/d (reference range, 36 to 137), confirming the diagnosis of Cushing syndrome. A serum ACTH level was elevated at 316 ng/dL (reference range, 10 to 52). An overnight highdose (8 mg orally) dexamethasone suppression test was positive (serum cortisol levels were 43.2 and 41 μg/dL before and after suppression, respectively), and magnetic resonance imaging of the pituitary gland disclosed no abnormalities. A prostate biopsy specimen showed small cell prostate cancer with positive staining for ACTH. The tumor was found to be unresectable, and the poor condition of the patient did not allow for bilateral adrenalectomy. He was treated with ketoconazole and metyrapone, which yielded good temporary control of his Cushing syndrome (24-hour urine free cortisol decreased to 55.2 μg/d). He received 1 cycle of chemotherapy (etoposide and cisplatin), but he died 6 months later as a result of sepsis.ConclusionSmall cell prostate cancer is a rare subtype that can be associated with ectopic secretion of ACTH and severe Cushing syndrome. With this subtype of prostate cancer, Cushing syndrome should be considered and appropriately managed. (Endocr Pract. 2010;16:249-254)     

Clinical Presentation and Long-Term Outcome of Patients With Ectopic Acth Syndrome Due to Bronchial Carcinoid Tumors: A One-Center Experience

Objective: To describe the diagnostic features and long-term outcome of patients with bronchial carcinoid tumors with ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS), treated in our department.Methods: We studied 10 cases with EAS and histologically confirmed bronchial carcinoid tumors, diagnosed from 1992 until 2006. Diagnosis was based upon blood, urine, radiologic, and interventional tests. Disease status at the time of the last follow-up was the primary outcome measure.Results: Clinical manifestations included Cushingoid features (100%), psychiatric symptoms (90%), hypertension (70%), diabetes/impaired glucose tolerance (40%), osteoporosis (10%), and hypokalemia (10%). The average time from the onset of symptoms until diagnosis was 14.2 ± 17.0 months. None of the patients exhibited a positive cortisol or ACTH response to corticotropin-releasing hormone (CRH) test, and none showed a positive gradient on bilateral inferior petrosal sinus sampling (BIPSS). All tumors were identified by computed tomography and by octreotide scintigraphy in 8 patients. All patients underwent surgical resection of the tumor, and 2 patients had adjuvant radiation therapy. The mean follow-up was 126.6 ± 63.3 months. At latest follow-up, 8 patients were in remission and 2 had recurrence of the EAS; both had a multifocal tumor. The 2 patients submitted to adjuvant radiation therapy were in remission at their latest follow-up, despite local invasion and lymph node metastases.Conclusion: CRH test and BIPSS are the most useful methods in diagnosing EAS. For localization, repeated imaging studies are necessary. Surgical treatment is effective in most cases. Adjunctive radiotherapy may be useful in patients with lymph node metastases. Patients with multifocal disease should be monitored for potential recurrence.Abbreviations: ACTH = adrenocotricotropic hormone BC = bronchial carcinoid BIPSS = bilateral inferior petrosal sinus sampling CD = Cushing disease CRH = corticotropin-releasing hormone CS = Cushing syndrome CT = computed tomography EAS = ectopic ACTH syndrome HDDST = high-dose dexamethasone suppression test HPA = hypothalamic-pituitary-adrenal IPS:P = inferior petrosal sinus to periphery ratio MRI = magnetic resonance imaging     

Production of Hormone B by Achlya heterosexualis

Sexual morphogenesis in Achlya, a filamentous water mold, is initiated by two hormones. Hormone A, or antheridiol, is a C-29 steroid. Four stereoisomers of antheridiol have been synthesized. The natural one (antheridiol 22S 23R) and its 7-deoxy 7-dihydro form, when added to an aerated culture of hermaphroditic Achlya heterosexualis, stimulated this mold to secrete twice as much hormone B as the untreated control. An unnatural stereoisomer (antheridiol 22R 23S) and fucosterol, however, did not stimulate strain 8-6 in the same way. Methods by which hormone B can be produced in sufficient yield for isolation and characterization are described.     

Ectopic Acromegaly due to A Pancreatic Neuroendocrine Tumor Producing Growth Hormone-Releasing Hormone

ObjectiveTo present a case of acromegaly due to ectopic growth hormone-releasing hormone (GHRH) secretion from a pancreatic neuroendocrine tumor in the context of multiple endocrine neoplasia type 1 (MEN 1).MethodsWe describe the clinical, imaging, and pathologic findings of the study patient.ResultsA 46 year old woman presented with clinical and biochemical findings diagnostic of acromegaly. Magnetic resonance imaging showed a 1.2-cm sellar mass. Following resection of the macroadenoma, serum insulinlike growth factor 1 (IGF-1) and growth hormone (GH) levels remained unchanged. Pathologic examination revealed adenomatous changes, including a nonsecretory focus and a prolactin immunopositive area (GH stain negative in both). Octreotide long-acting release was ineffective. Search for an ectopic tumor included normal octreoscan and abdominal computed tomography. GHRH was greater than 1000 pg/mL. Repeated abdominal computed tomography documented a 6.2-cm mass in the tail and body of the pancreas. Distal pancreatectomy revealed a pancreatic neuroendocrine tumor that stained positive for GHRH. Postoperatively, serum GHRH and IGF-1 normalized. Re-evaluation of the initial pituitary pathologic specimen revealed additional somatotroph hyperplasia of the adjacent, normal pituitary gland. Primary hyperparathyroidism was diagnosed, and multigland parathyroid hyperplasia was noted at surgery. Genetic testing was positive for a mutation in the MEN1 gene.ConclusionThis patient’s acromegaly was resistant to somatostatin analogue therapy, reflecting the negative octreoscan imaging. In addition, this case is novel because the patient presented with pituitary adenomatous changes, which were presumably associated with MEN 1 and/or possibly the elevated GHRH levels. (Endocr Pract. 2011; 17:79-84)     

Hyperparathyroid Crisis Due to Asymmetric Parathyroid Hyperplasia with A Massive Ectopic Parathyroid Gland

ObjectiveTo report a rare case of primary hyperparathyroidism presenting with hyper-parathyroid crisis due to parathyroid hyperplasia with ectopic glands.MethodsWe present the initial clinical manifestations, laboratory results, radiologic and surgical findings, and management in a patient who had hyper-parathyroid crisis. The pertinent literature and management options are also reviewed.ResultsA 60-year-old female presented with hyper-parathyroid crisis requiring preoperative stabilization with rehydration, diuresis, bisphosphonate therapy, and ultimately hemodialysis. Parathyroidectomy revealed asymmetric 4-gland hyperplasia, with a massive ectopic parathyroid gland in the tracheoesophageal groove extending into the mediastinum. Her postoperative course was complicated by hungry bone syndrome and hypocalcemia.ConclusionThis case illustrates the rare occurrence of hyper-parathyroid crises due to asymmetric parathyroid hyperplasia with a massive ectopic parathyroid gland. (Endocr Pract. 2014;20:e180-e182)     

Burning-Feet Syndrome: Case Due to Malabsorption and Responding to Riboflavine

A woman with the burning-feet syndrome was found on investigation to have malabsorption. The syndrome responded rapidly to intramuscular injections of 6 mg. of riboflavine daily. It is suggested that deficiency of this substance, due to malabsorption and aggravated by a defective diet and repeated pregnancies, was responsible for the syndrome in this case.     

Impaired Olfactory Associative Behavior of Honeybee Workers Due to Contamination of Imidacloprid in the Larval Stage

The residue of imidacloprid in the nectar and pollens of the plants is toxic not only to adult honeybees but also the larvae. Our understanding of the risk of imidacloprid to larvae of the honeybees is still in a very early stage. In this study, the capped-brood, pupation and eclosion rates of the honeybee larvae were recorded after treating them directly in the hive with different dosages of imidacloprid. The brood-capped rates of the larvae decreased significantly when the dosages increased from 24 to 8000 ng/larva. However, there were no significant effects of DMSO or 0.4 ng of imidacloprid per larva on the brood-capped, pupation and eclosion rates. Although the sublethal dosage of imidacloprid had no effect on the eclosion rate, we found that the olfactory associative behavior of the adult bees was impaired if they had been treated with 0.04 ng/larva imidacloprid in the larval stage. These results demonstrate that a sublethal dosage of imidacloprid given to the larvae affects the subsequent associative ability of the adult honeybee workers. Thus, a low dose of imidacloprid may affect the survival condition of the entire colony, even though the larvae survive to adulthood.