The role of bone scintigraphy in patients with Erdheim-Chester disease.

Erdheim-Chester disease (ECD) is a rare disorder that has been reported fewer than 60 times in the literature. Although clinical findings seem to be specific at first sight, histologic classification remains unclear. It has not been decided whether ECD is part of the spectrum of histiocytoses or whether it may be a lipid storage disorder or even a primary macrophage cell disorder, although it does show a distinct histologic pattern. However, the clinical appearance alone shows several typical features, rendering the diagnosis very probable if present. This article illustrates the importance of bone scanning in ECD, because the scintigraphic pattern of involved skeletal sites may in themselves lead to the diagnosis. Several differential diagnoses are considered. The importance of bone scintigraphy as an imaging method in patients with an unclear diagnosis is discussed, as exemplary in ECD, as is its role for the detection of sites of skeletal involvement in other diseases.

The value of somatostatin-receptor scintigraphy in newly diagnosed endocrine gastroenteropancreatic tumors.

BACKGROUND: Conventional imaging techniques do not routinely detect endocrine gastroenteropancreatic tumors preoperatively. The purpose of this study was to determine whether the new technique of somatostatin-receptor scintigraphy would improve the detection rate of these tumors before initial treatment. STUDY DESIGN: In a prospective study, 55 patients with a recent diagnosis of endocrine gastroenteropancreatic tumors (22 intestinal carcinoids, 17 gastrinomas, 10 nonfunctioning pancreatic tumors, and 6 insulinomas), were examined with somatostatin-receptor scintigraphy, computed tomography, and ultrasonography. Results of the three imaging modalities were compared with findings at surgical exploration. RESULTS: None of the insulinomas were localized by somatostatin-receptor scintigraphy, but 4 of 6 insulinomas were detected by computed tomography and ultrasonography. Of 17 gastrinomas, 9 were detected by somatostatin-receptor scintigraphy; computed tomography and ultrasonography localized only 7. Metastases from the gastrinoma were localized by somatostatin-receptor scintigraphy in all cases; computed tomography and ultrasonography detected metastases in only 6 of 9 patients. Nonfunctioning tumors could be localized by somatostatin-receptor scintigraphy, computed tomography, and ultrasonography in 4, 7, and 8 of 10 cases, respectively. Detection rate for corresponding metastases was the same for all three imaging techniques. Primary carcinoids were identified by somatostatin-receptor scintigraphy, ultrasonography, and computed tomography in 7, 8, and 11 of 22 cases, respectively. Extra-abdominal metastases were detected by somatostatin-receptor scintigraphy in only 7 of 19 patients. CONCLUSIONS: In patients with insulinomas, somatostatin-receptor scintigraphy is not indicated because none of the six tumors was imaged. This holds true for nonfunctional pancreatic endocrine tumors and their metastases because no advantage for somatostatin-receptor scintigraphy was found over computed tomography and ultrasonography. In contrast, somatostatin-receptor scintigraphy is superior to computed tomography and ultrasonography for determining the extent of the disease in patients with gastrinomas or carcinoids. The problem of detecting primary tumors in these patients is not solved by somatostatin-receptor scintigraphy.

[Somatostatin receptor scintigraphy in preoperative diagnosis of the site of endocrine gastrointestinal tumors]. / Die Somatostatinreceptor-Szintigraphie in der präoperativen Lokalisationsdiagnostik endokriner gastrointestinaler Tumoren.

To determine the value of somatostatin-receptor scintigraphy in the localization of various endocrine gastrointestinal tumors, we compared the results obtained with this new technique with the results obtained with computed tomography and sonography. We could not find an overall advantage of somatostatin-receptor scintigraphy as compared to computed tomography or sonography in the localization of intestinal carcinoids (n = 13), gastrinomas (n = 12), functionally non-active endocrine pancreatic tumors (n = 8) and various other endocrine pancreatic tumors (n = 4). In 2 patients with endocrine pancreatic tumors however, the tumors were localized preoperatively only by somatostatin-receptor scintigraphy. Somatostatin-receptor scintigraphy may occasionally be helpful in the localization of gastrointestinal endocrine tumors if these tumors are not localized by conventional imaging studies. Somatostatin-receptor scintigraphy does not solve the problem to localize small endocrine tumors.

[Scintigraphy of parathyroid adenomas with 99mTc-sestamibi in an endemic goiter area]. / Szintigraphische Darstellung von Adenomen der Nebenschilddrüse mit 99mTc-Sestamibi in einem Strumaendemiegebiet.

Recently 99mTc-Sestamibi (MIBI) has been introduced into parathyroid imaging. The purpose of this study was to evaluate the MIBI scan in an endemic goiter area. 25 patients with primary and 9 with secondary hyperparathyroidism (HPT) underwent a MIBI scan one day prior to surgical exploration of the neck. Cervicothoracic planar scintigraphy was performed 5, 15 and 120 min after i.v. injection of 444 MBq of 99mTc-Sestamibi. The MIBI scan correctly detected 20 of 25 adenomas in 25 patients with pHPT. In only 6 of 9 patients with parathyroid hyperplasia a focal uptake was found. The cause of one false-positive result in the control group was an increased MIBI uptake by a follicular adenoma of the thyroid. Parathyroid scintigraphy using 99mTc-Sestamibi as a single radiopharmaceutical is as sensitive in detecting and localizing parathyroid adenomas as the Tl/Tc-scintigraphy. Due to a high prevalence of thyroid adenomas in an endemic goiter area a higher rate of falsely positive results may be expected.

[Early recognition of autonomous thyroid tissue by a combination of quantitative thyroid pertechnetate scintigraphy with the free T4 equivalent (author's transl)]. / Früherkennung der thyreoidalen Autonomie durch Kombination von quantitativer Szintigrammauswertung mit einem Aquivalent des freien T4.

Autonomously functioning thyroid tissue (AFTT) of the circumscribed as well as the disseminated form was found with equal frequency and extent in eumetabolic patients under and over 40 years of age who live in an endemic goitre area. In an area of iodine deficiency this is the most frequent precondition for hyperthyroidism induced by iodine administration. A suspicion of AFTT can be raised in 80% of still euthyroid patients by a combined evaluation of an equivalent to the free T4 (FTE) and an equivalent to the clearance using a quantitative evaluation of the thyroid technetium scan (TcTU). The suppression test not only confirms the autonomy in a qualitative manner but also provides an estimate of the volume of autonomous tissue because the TcTU after suppression strictly correlates linearly with the volume of the AFTT. Since after iodine administration FTE increases proportionally to the volume of AFTT, the procedure can be of some prognostic value: those with autonomous tissue in excess of a "critical" volume will almost certainly develop hyperthyroidism following a certain minimum rate of iodine administration. A prospective study of patients under age 50 whose thyroids contained various amounts of AFTT has shown that prophylactic dietary iodine supplementation will not cause hyperthyroidism to develop provided the additional iodine intake does not exceed 100 micrograms per day.

[The diagnostic significance of immunoglobulin determination in chronic liver diseases. III. observations of progress (author's transl)]. / Die diagnostische Bedeutung der Immunglobulinbestimmung bei chronischen Lebererkrankungen. III. Verlaufsbeobachtungen

55 patients with chronic aggressive hepatitis and 20 with liver cirrhosis were observed over a 2-year period. Liver biopsy was performed several times but no immunosuppressive therapy was carried out. In this way a comparison was made between biochemically and histomorphologically demonstrable alterations in the process with the immunoglobulins. Significant agreement between the clinical biochemical and histomorphological activities and the behavior of immunoglobulin can only be concluded from increased activity of the process. But in histological deterioration, no correlation to the immunoglobulins was established, with the exception of IGM.

[The diagnostic significance of immunoglobulin determination in chronic liver diseases. II. Relationships between immunoglobulin changes and degree of activity (author's transl)]. / Die diagnostische Bedeutung der Immunglobulinbestimmung bei chronischen Lebererkrankungen. II. Beziehungen zwischen Immunglobulinveränderungen und Aktivitätsgrad

The relationship between the immunograms and aminotransferases, gamma-GT and AP were examined in 715 patients with hepatobiliary diseases closely confirmed by histomorphological criteria and a series of 60 normal subjects. The normal subjects showed positive relationships. These are largely lacking in acute and protracted courses and in chronic aggressive hepatitis. Only relationships of the individual immunoglobulins between themselves can be demonstrated in the aggressive chronic types. Positive relationships are present between immunograms and aminotransferases, gamma-GT and AP in the group with fatty livers, and so are other highly significant positive relationships in toxic hepatitis and toxic cirrhoses of the liver.

[The diagnostic significance of immunoglobulin determination in chronic liver diseases. I. Differential diagnostic accuracy of immunoglobulin changes and enzyme activities (author's transl)]. / Die diagnostische Bedeutung der Immunoglobulinbestimmung bei chronischen Lebererkrankungen. I. Differentialdiagnostische Treffsicherheit von Immunglobulinveränderungen und Enzymaktivitäten

715 patients with hepatobiliary diseases came for examination, who because of the morphological findings are classified in diagnostic groups. The immunoglobulin measurements of IGG, IGA, IGM were determined and assessed in combination with enzymatic investigations of GOT, GPT, AP and GGTP. Typical group-specific changes were only found in primary biliary liver diseases and toxic cirrhoses of the liver, all acute or chronic inflammatory liver diseases could not be separated and by inference from the final diagnosis showed great errors in classification. Data on the raised mean levels of immunoglobulins in the individual diagnostic groups were demonstrable, but the range limits were so widely scattered that their differential diagnostic valence is of no consequence. Immunoglobulins may appear to be of interest for the observation of the course of liver diseases, but they are unsuitable for diagnostic purposes.