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Tumor-induced osteomalacia is a rare hypophosphatemic disease caused by unregulated production of fibroblast growth factor 23 by a tumor, thereby inducing renal phosphate wasting and inhibiting appropriate increase of calcitriol production. Symptoms of tumor-induced osteomalacia, including muscle weakness, bone pain, and pathologic fractures, are nonspecific and warrant further workup. We report the case of a 50-year-old African American female with no known psychiatric illness who was admitted after a failed suicide attempt provoked by severe bone pain. She had been treated for fibromyalgia and hypophosphatemic rickets at other facilities with no improvement. The findings of profound renal phosphate wasting initiated further evaluation, which revealed an elevated fibroblast growth factor 23 level and a right proximal fibular mesenchymal tumor on octreotide scintigraphy. Magnetic resonance imaging confirmed the findings of a solid intramuscular tumor corresponding to the octreotide avid lesion. After wide excision of the tumor, serum phosphate and parathyroid hormone levels began to normalize. This case highlights the importance of extensively investigating the cause of bone pain, weakness, and fatigue in patients without a family history of hypophosphatemia or bone disorders. The aforementioned symptoms may precede recurrent pathological fractures, and a thorough workup ensures that a diagnosis of tumor is not delayed or overlooked, as tumor resection confers a favorable prognosis and dramatic increase in the quality of life for patients.

99 Tc m -octreotide scintigraphy and serum eye muscle antibodies in evaluation of active thyroid-associated ophthalmopathy

Purpose: Diverse radionuclide imaging techniques are available for the diagnosis, staging, and follow-up of phaeochromocytoma and paraganglioma (PPGL). Beyond their ability to detect and localise the disease, these imaging approaches variably characterise these tumours at the cellular and molecular levels and can guide therapy. Here we present updated guidelines jointly approved by the EANM and SNMMI for assisting nuclear medicine practitioners in not only the selection and performance of currently available single-photon emission computed tomography and positron emission tomography procedures, but also the interpretation and reporting of the results. Methods: Guidelines from related fields and relevant literature have been considered in consultation with leading experts involved in the management of PPGL. The provided information should be applied according to local laws and regulations as well as the availability of various radiopharmaceuticals. Conclusion: Since the European Association of Nuclear Medicine 2012 guidelines, the excellent results obtained with gallium-68 (68Ga)-labelled somatostatin analogues (SSAs) in recent years have simplified the imaging approach for PPGL patients that can also be used for selecting patients for peptide receptor radionuclide therapy as a potential alternative or complement to the traditional theranostic approach with iodine-123 (123I)/iodine-131 (131I)-labelled meta-iodobenzylguanidine. Genomic characterisation of subgroups with differing risk of lesion development and subsequent metastatic spread is refining the use of molecular imaging in the personalised approach to hereditary PPGL patients for detection, staging, and follow-up surveillance.

Introduction Somatostatin-secreting neuroendocrine tumours may present with diabetes, cholelithiasis and steatorrhoea. In addition, hypoglycaemia has been associated with somatostatinomas. However, the mechanism of hypoglycaemia in patients with somatostatinomas has not been well characterized. Methods We describe two patients with recurrent neuroglycopenic episodes caused by somatostatin-secreting neuroendocrine tumours in the liver, detected by abdominal CTs and whole-body octreotide scintigraphy scans and confirmed by biopsy. Results Pancreatic islet hyperplasia and co-secretion of insulin (in addition to somatostatin) from tumour cells, respectively, have been characterized as completely distinct mechanisms of hypoglycaemia at both the functional and morphological levels in these two patients. Conclusions Hypoglycaemia may be caused by different mechanisms in patients with somatostatinomas.

Intracranial dural arteriovenous fistulae (DAVF) are rare vascular malformations. They are generally considered to be acquired lesions, often attributed to dural sinus thrombosis and intracranial venous hypertension. The authors encountered a case of DAVF associated with an octreotide-positive vestibular schwannoma. A 46-year-old female had symptoms of right ear congestion accompanied by pulsatile tinnitus and mild hearing loss. Magnetic resonance imaging (MRI) identified a lobulated mass centered at the cerebellopontine angle. Preoperatively, on cerebral angiography, there was an incidental discovery of a DAVF in the right posterior fossa. The decision was made to proceed with resection of the tumor in a staged fashion. Her latest follow-up MRI showed no evidence of recurrent tumor. This is the second reported case of DAVF associated with an intracranial schwannoma. Findings are discussed along with a thorough review of the literature. This case, combined with the data from the literature review, led us to believe that tumor-related angiogenesis might contribute to DAVF formation.

Ga-68 DOTATATE PET/MR imaging provides superior detection, localization, and characterization of pancreatic neuroendocrine tumors. It is excellent at detecting metastatic and recurrent disease and can detect therapy. Ga-68 DOTATATE has been shown to be superior to octreotide scintigraphy in the evaluation of these tumors.

Thyroid-stimulating hormone-secreting pituitary adenoma (TSHoma) is a rare benign endocrinological tumor which produces TSH in the pituitary gland. Herein, we presented a female patient having TSHoma with Graves' disease during and just after pregnancy that we found by indium-111 octreotide scintigraphy while investigating the patient for hyperthyroidism symptoms.

A 54-year-old woman underwent octreotide scintigraphy for evaluation of neuroendocrine tumor recurrence. The images demonstrated unusual uptake in gallbladder area in addition to physiologic uptake at other tissues. Whole-body planar and SPECT imaging were repeated after fatty meal ingestion at 28 hours in order to figure out whether this activity was physiologic or not. Since the unusual uptake in the gallbladder was still detected at these images, additional images were obtained 72 hours after radionuclide injection. The activity in the gallbladder disappeared at these images revealing the physiologic nature of this unusual accumulation.Elli dört yaşında bayan hastaya, nöroendokrin tümörün olası rekürrensinin saptanması amacıyla oktreotid sintigrafisi uygulandı. Görüntülerde, diğer dokulardaki fizyolojik tutulumlara ek olarak safra kesesi bölgesinde olağandışı tutulum gözlendi. Safra kesesindeki tutulumun fizyolojik olup olmadığının anlaşılabilmesi için 28. saatte yağlı yemek yenmesi sonrası tüm-vücut planar ve SPECT görüntüleri tekrarlandı. Safra kesesi bölgesindeki olağandışı aktivitenin bu görüntülerde de devam etmesi üzerine radyonüklidin enjeksiyonundan sonra 72. saatte görüntüler tekrarlandı. Bu görüntülerde safra kesesindeki aktivitenin kaybolması bu olağandışı tutulumun fizyolojik karakterde olduğunu gösterdi.

Tumors that cause osteomalacia are generally benign fibroblast growth factor-23 producing mesenchymal tumors which can be detected by octreoscan. A 45-year-old man underwent (99m)Tc-HYNIC-TOC scan to detect a possible culprit causing osteomalacia. The images showed abnormal activity in the left humerus and in the right foot. The lesion of the right foot was confirmed pathologically as phosphaturic mesenchymal tumor. After surgical removal of the tumor in the right foot, the patient's symptoms were promptly improved. However, the improvement was transient due to the unresected tumor in the left humerus.

Small bowel neuroendocrine tumors (NETs) are frequently characterized by a strong propensity to metastasize to the liver, mesentery, and peritoneum. However, only a few extra-abdominal metastatic sites have been reported in the published literature. The present paper implicates that primary small bowel NETs may unusually metastasize to the inguinal lymph nodes. Furthermore, we discuss the formidable diagnostic and therapeutic challenges associated with the metastatic NETs.

Ectopic ACTH-dependent Cushing syndrome-inducing small-cell neuroendocrine carcinoma of the cervix, undiagnosed by computed tomography and whole-body octreotide scintigraphy, was identified using F-18 FDG PET/CT imaging. This article is protected by copyright. All rights reserved.

Although somatostatin receptor positron emission tomography (PET)/CT is gaining increasing popularity and has shown its diagnostic superiority in several studies, (111)In-diethylenetriaminepentaacetic acid (DTPA)-octreotide is still the current standard for diagnosis of neuroendocrine tumours (NET). The aim of this study was to compare the costs for the two diagnostic tests and the respective consequential costs.From January 2009 to July 2009, 51 consecutive patients with enteropancreatic NET who underwent contrast-enhanced (68)Ga-DOTATOC PET/CT (n = 29) or (111)In-DTPA-octreotide (mean 3 whole-body scans plus 1.6 low-dose single photon emission computed tomography/CT; n = 22) were included. For cost analysis, direct costs (equipment) and variable costs (material, labour) per examination were calculated. Additionally required CT and/or MRI examinations within the staging process were assessed as consequential costs. An additional deterministic sensitivity analysis was performed.A (68)Ga-DOTATOC PET/CT examination yielded total costs (equipment, personnel and material costs) of 548 euro. On the other hand, an (111)In-DTPA-octreotide examination resulted in 827 euro total costs. Costs for equipment and material had a share of 460 euro/720 euro for (68)Ga-DOTATOC/(111)In-DTPA-octreotide and labour costs of 89 euro/106 euro. With (68)Ga-DOTATOC additional MRI had to be performed in 7% of the patients resulting in a mean of 20 euro for supplementary imaging per patient; 82% of patients with (111)In-DTPA-octreotide needed additional MRI and/or CT resulting in mean additional costs of 161 euro per patient.(68)Ga-DOTATOC PET/CT was considerably cheaper than (111)In-DTPA-octreotide with respect to both material and personnel costs. Furthermore, by using (68)Ga-DOTATOC PET/CT considerably fewer additional examinations were needed reducing the consequential costs significantly.

Formerly used concepts for phaeochromocytomas and paragangliomas have been challenged by recent discoveries that at least 24% of tumours are familial and thereby often multiple in various locations throughout the body. Furthermore, tumours are often malignant and perhaps more aggressive if associated with SDHB gene mutations. Some paragangliomas are clinically silent and may present only with dopamine hypersecretion. In the current era where CT and MRI are more commonly used, tumours are more often found as incidentalomas and MRI may be less specific for phaeochromocytoma and paraganglioma than previously thought. Because of unique tumour characteristics (e.g. the presence of cell membrane and intracellular vesicular norepinephrine transporters) these tumours were 'born' to be imaged by means of specific functional imaging approaches. Moreover, additional recent discoveries related to apoptosis, hypoxia, acidosis, anaerobic glycolysis and angiogenesis, often disturbed in tumour cells, open new options and challenges to specifically image phaeochromocytomas and paragangliomas and possibly link those results to their pathophysiology, genotypic alterations and metastatic potential. Functional imaging, especially represented by positron emission tomography (PET), offers an excellent approach by which tumour-specific processes can be detected, evaluated and seen in the context of tumour-specific behaviour and its genetic signature. In this review, we address the recent developments in new functional imaging modalities for phaeochromocytoma and paraganglioma and provide the reader with suggested imaging approaches in various phaeochromocytomas and paragangliomas of sympathetic origin. Current imaging algorithms of head and neck parasympathetic paragangliomas are not discussed. Finally, this review outlines some future perspectives of functional imaging of these tumours.

We report a 31-year-old woman with tumor-induced osteomalacia suffering from slowly progressive bilateral muscle weakness predominantly in the proximal muscles and multiple bone pains for the past 2 years. She was unable to walk or raise her arms above the shoulder. We suspected tumor-induced osteomalacia due to decreased serum phosphate and 1alpha, 25 (OH),-vitamin D3 levels, low percentage of tubular reabsorption of phosphate (%TRP), adult onset, and no family history of osteomalacia. Regular imaging examinations could not detect the location of the primary tumor: however, indium-111 octreotide scintigraphy detected the causative primary mesenchymal tumor in the right sole. Pain and muscle weakness improved promptly after tumor resection, and she was able to walk 6 d postoperatively. This is the first case report in Japan describing the detection of the primary tumor site by indium-111 octreotide scintigraphy.

Paragangliome werden den neuroendokrinen Tumoren zugeordnet und produzieren als solche gelegentlich Peptidhormone.Sehr selten kommen sie auch in der Nase und/oder den Nasennebenhohlen vor. Beschrieben wird der nach unserer Erkenntnis erste Fall eines rezidivierenden nasalen Paraganglioms, das nach zehnjahrigem endokrinologisch unauffalligem Krankheitsverlauf zu einem Cushing-Syndrom durch ektope ACTH-Produktion fuhrte. Nach chirurgischer Therapie kam es zu einer Normalisierung der ACTH- und Kortisolwerte.

Carcinoid tumors are rich in somatostatin receptors and show high uptake of radiotracer on octreotide scintigraphy. 68Ga-DOTATATE could be of great help at initial staging and during follow-up of these patients. We describe a patient with avid 68Ga-DOTATATE and poor F18-FDG uptake.

Paragangliomas are neuroendocrine tumors located primarily in the head and neck region, but they can also occur at other sites. Confirming the preoperative diagnosis and detecting synchronous tumors may be difficult in some patients. Octreotide is a somatostatin analog that, when coupled to a radioisotope, produces a scintigraphic image of tumors expressing somatostatin type 2 receptors. Paragangliomas, like many neuroendocrine tumors, have been found to have a high density of somatostatin type 2 receptors on the cell surface. This study compared the results of preoperative octreotide scintigraphy with the histopathology in 21 patients who underwent surgery for presumed head and neck paragangliomas. Octreotide scan findings were positive in 16 patients with paragangliomas and negative in 3 patients with other pathology. One false-positive and 1 false-negative result were obtained. Thus, this test had an accuracy of 90%, a sensitivity of 94%, and a specificity of 75%. Previously unidentified synchronous tumors were identified in 5 patients. On the basis of this series of patients, octreotide scintigraphy appears to be a reliable test to detect paragangliomas and may be quite helpful in preoperative planning.

European Annals of Otorhinolaryngology, Head and Neck Diseases - Vol. 132 - N° 2 - p. 113-114

Bronchial carcinoid tumor is the most frequent occult source of ectopic ACTH-dependent Cushing’s syndrome, but its initial localization may be very difficult, as well as its postoperative follow-up. We here present the case of a 21 -year-old man with Cushing’s syndrome and biochemical findings suggesting an ectopic source of ACTH (lack of inhibition of Cortisol after overnight 8-mg dexametha-sone supression test, and lack of response to h-CRH challenge). Chest CT-scan showed a node adjacent to the left lung hilium whose nature was confirmed by uptake of 111Indium-DTPA labelled octreotide scintigraphy. Surgical resection of the tumor consisted in an upper lobectomy of the left lung. Microscopic examination identified a typical carcinoid tumor. After surgery pituitary-adrenal function normalized and a second scintigraphy offered additional data on the absence of tumor remnants.

Both ectopic pancreatic tissue and malignant insulinoma are rare. We encountered the combination of these rare entities in a patient demonstrating hypoglycemic symptoms.A patient with episodic hypoglycemia, non-suppressed serum insulin levels, positive fasting test and a positive localization on both ultrasound and somatostatin receptor scintigraphy by (111)I-labelled octreotide, was operated and investigated.(111)I-labelled octreotide-guided surgery was performed and a tumor adjacent to the ligament of Treitz was extirpated. Follow-up was uneventful. Histopathology revealed a malignant insulinoma. The patient is currently under close surveillance.The rare combination of an insulinoma in ectopic pancreatic tissue, where the usual diagnostic endoscopic ultrasound revealed an extrapancreatic mass, is described. This was followed by a positive somatostatin receptor scintigraphy, enabling both radio-guided surgery and future treatment of developing recurrent disease or metastases.

Aim Various studies have been conducted for determining the most optimal method for the early diagnosis of local recurrent or distant metastatic thyroid cancers. The aim of this study was to evaluate the clinical utility of technetium-99m (Tc-99m)-labeled octreotide derivatives in the detection of recurrence or distant metastases in medullary thyroid cancer patients and to compare the lesions with those detected using 18F-fluorodeoxyglucose (18F-FDG)-PET and Tc-99m MIBI studies in the same patient group. Patients and methods Sixteen medullary thyroid cancer patients [two male and 14 female; mean age 52.0±14.1 years (range 13–72 years)] were included in this study. All patients underwent a whole-body scan 1 and 4 h after injection with octreotide derivatives and single photon emission computed tomography images were taken of the sites suspicious for metastasis. The lesions seen in Tc-99m HYNIC octreotide studies were compared with those seen in 18F-FDG-PET and Tc-99m MIBI studies. Results Among the Tc-99m-labeled octreotide scintigraphy studies, nine were evaluated as true positive (56.2%) and one was evaluated as false positive (6.2%); six were false negative (37.5%). In 16 patients, the total number of lesions seen on octreotide scintigraphy was 21. Thirteen of the 16 patients underwent 18F-FDG-PET imaging. Of the 13 patients studied, 10 showed true-positive (76.9%) and three showed false-negative (23.1%) results. The total number of lesions seen on 18F-FDG-PET was 23. The Tc-99m MIBI study yielded positive results in seven of 16 patients (43.7%) and negative results in nine patients (56.3%). The total number of lesions on Tc-99m MIBI was 12. Conclusion The Tc-99m-labeled somatostatin receptor scintigraphy analogs HYNIC-tyrosine octreotide and HYNIC-TATE are useful imaging alternatives in somatostatin receptor-expressing thyroid cancers. Radiolabeling using these analogs is easy and they are readily available for routine use.

A 44-year-old woman had a 13-year history of a small bulge of the left frontal region which had increased in size during the last year. At admission, an orange-sized, hard, fixed left frontal mass was found. Magnetic resonance imaging showed hyperostosis in the left frontal region which was causing a skull deformity and marked focal meningeal enhancement. A hyperostotic plaque meningioma was hypothesized. In-111 octreotide scintigraphy confirmed the diagnosis before surgery. In-111 octreotide scintigraphy allows biologic characterization of neoplasms in vivo.

Carcinoids represent 1–2% of the rectal cancer. We report the case of a woman with a rectal carcinoid and a hypogastric node metastasis. We propose a less invasive treatment of the metastase using In11-octreotide-scintigraphy and intraoperative gamma-probe detector.

Indium-111 octreotide scintigraphy is a useful investigation in the diagnosis and staging neuroendocrine or nonendocrine malignancies such as carcinoid, granulomas, small cell lung cancer, medullary thyroid carcinoma, and others. Tumors with large numbers of somatostatin receptors can be accurately imaged and this can be useful in guiding treatment. Although uncommon, false-positives studies have been reported and often the cause is unexplained but assumed to be a high number of somatostatin receptors in other pathology. We present a case with increased uptake resulting in a scan due to pulmonary fibrosis.

Primary pheochromocytomas of the heart are extremely rare tumors and difficult to diagnose. Iodine labeled metaiodobenzylguanidine (MIBG) is the first choice of the nuclear medicine modality in the evaluation of adrenal pheochromocytoma. However, the sensitivity of MIBG in the diagnosis of extraadrenal pheochromocytoma is less optimal. In this preliminary report, the efficacy of octreotide scintigraphy using Tc-99m labeled hydrazinonicotinyl-Tyr3-octreotide (HYNIC-TOC) in the evaluation of primary cardiac pheochromocytoma was assessed.

The worldwide 2009 estimates for the average annual per-capita effective radiation dose from medicine have doubled during the past 15 years. This has increased the concern for patients as radiation sources. The existing evidence indicates that the amount of radiation is small; but there are few empirical studies with results documenting the actual extent. In this study, we examined the radiation from 48 patients undergoing nuclear medical examination. 20 patients were examined with bone scintigraphy, 20 underwent MUGA, while the remaining 8 went through octreotide scintigraphy procedure. At 0.25 meters from the patient, the radiation ranged from 31±9 µSvh-1 for octreotide scintigraphy patients (111-In as agent), 69±13 µSvh-1 for bone scintigraphy (99mTc), to 92±26 µSvh-1 for the MUGA patients (99mTc). On the basis of these findings and others, one may consider current practices regarding waiting and using led shielding in areas where appropriate. Perhaps, and more important, these results could be used to improve patient and staff education. Better information material with more evidence will reduce undue anxiety.

Nous rapportons le cas d’un patient presentant une maladie de Paget osseuse associee a un diabete phosphate (phosphate :1,6 mg/dl (2.5-4.4 mg/dl), la clearance du phosphate est de 29 ml/’ (nl 85 %). Nous avons teste l’hypothese (comme recemment demontree lors de diabete phosphate associe aux tumeurs) d’une amelioration du diabete phosphate lors de l’administration d’analogue de la somatostatine. Le patient a beneficie d’une scintigraphie a l’octreotide marque a l’Indium-111 demontrant une captation dans l’os pagetique. Sous Lanreotide (40 mg IM), le patient a presente une amelioration des douleurs osseuses avec diminutions du taux de phosphatase alcaline. La clearance de phosphate et le taux de reabsorption tubulaire de phosphate ne sont pas modifies. Nous rapportons egalement les autres cas associant une maladie de Paget et un diabete phosphate.

An 81-year-old woman with an elevated serum gastrin level underwent an In-111 octreotide scan for evaluation of a possible gastrinoma. Planar and SPECT images acquired 24 and 48 hours after injection of 211 MBq (5.7 mCi) In-111 octreotide showed an apparent increase in tracer uptake in the left chest posteriorly. This was not uniform in size or appearance. Tracer uptake elsewhere appeared physiologic. A previous computed tomographic (CT) scan revealed eventration of the left hemidiaphragm. The accumulation of In-111 octreotide in the patient's left chest posteriorly represents displaced abdominal contents, including the spleen, stomach, and bowel, as can be seen on the CT scan, and is considered to be physiologic in this case. This is a potential false-positive cause of In-111 octreotide uptake in the left chest, and a chest radiograph or CT should be reviewed in all patients with such an appearance.

Medulloblastoma is a common cancer in children but not adults. The authors report the first case of adult medulloblastoma studied with somatostatin receptor scintigraphy (SRS). A 34-year-old man had a history of desmoplastic left cerebellar medulloblastoma that had been removed surgically. After surgery, findings of clinical follow-up and computed tomographic (CT) imaging were satisfactory. One month later, the patient had continuous epileptic seizures. CT scanning revealed nonspecific alterations after surgery in the left cerebellum. In-111 octreotide scintigraphy showed a left cerebellar lesion suggestive of residual tumor. In this case, conventional imaging did not detect the residual tumor that was clearly shown by SRS.

The increasingly popular use of somatostatin analogs in clinical practice for both diagnostic and therapeutic purposes prompted extensive investigations on somatostatin receptor (sst) expression in human tumors by autoradiography, nucleic acid analysis and, recently, immunohistochemistry (IHC). The currently employed radiotracer for scintigraphy (Octreoscan) is octreotide, a somatostatin analog having a high affinity for sst types 2, 3, and 5. In this study on 25 patients, we compared sst 2, 3, and 5 expression in surgical and biopsy specimens of lung tumors, as revealed by immunohistochemical and reverse transcriptase polymerase chain reaction (RT-PCR), with the octreoscan outcome (which was positive in 20/25 cases). By IHC, the tumors mainly expressed sst2 (17/25, 68%) at the cell membrane level, while sst 3 and 5 were detected in a fraction of cases (24% and 20%, respectively). Comparing RT-PCR and IHC data, a correlation was found in 83.3% of cases, while octreoscan findings and sst expression were correlated in 22/25 cases (88%). In addition, cytological and biopsy specimens expressed the same sst type found in the corresponding surgical sample, thus indicating that a cell membrane sst immunoreactivity in a biopsy reliably predicts the tumor-receptor profile before its resection. Finally, sst expression was not restricted to neuroendocrine lung tumors, but was also a feature of some non-neuroendocrine carcinomas, although to a lesser extent. The occasional expression of sst subtypes in intratumoral lymphocytes, endothelia and necrotic areas is an additional feature to be considered in the interpretation of Octreoscan findings, since the in vivo procedure does not allow to define the sst cellular distribution. IHC can therefore be usefully coupled to radionuclear investigations to better characterize the sst cellular location and subtype in lung tumors.