Benign giant cell tumor of bone with osteosarcomatous transformation (”dedifferentiated” primary malignant GCT): report of two cases

 It is not uncommon for sarcomatous transformation of giant cell tumor (GCT) of bone to occur after radiation, but rarely does malignant transformation occur spontaneously, with less than 15 cases reported up to 1995. Only four of these cases have been documented in detail. We report two additional cases of GCT of bone spontaneously transforming or ”dedifferentiating” into osteosarcoma without radiation therapy. The first case is absolutely unique and most interesting in that the dedifferentiation process occurred in one of multiple GCT lung metastases 6 years after successful eradication of a primary tibial tumor. The right lung was resected due to development of a large tumor, and at pathologic examination, demonstrated several small nodules of conventional GCT and a much larger, 14-cm mass composed of a mixture of GCT and high-grade osteosarcoma. The second case involved a physician, who had a large tumor in the sacrum with vague symptoms for 8 years. Open biopsy revealed conventional, benign GCT of bone with a secondary aneurysmal bone cyst. Complete curettage 2 weeks later revealed, in addition to areas of conventional, benign GCT a second component of very high grade osteosarcoma. Both patients died less than 1.5 years from diagnosis. This report of osteosarcomatous transformation of a conventional GCT of bone strengthens the theory that there is a mesenchymal cell line in GCT that may spontaneously tansform to sarcoma.     

Aβ40 is a major form of β-amyloid in nonhuman primates

Because aged nonhuman primates show β-amyloid (Aβ) deposition in senile plaques and blood vessels similar to that seen in human aging and AD, we used C-terminal specific antibodies to Aβ₄₀ and Aβ₄₂ to investigate Aβ peptide length in the brains of 11 aged rhesus monkeys and a 59-year-old chimpanzee. In contrast to AD, where the earliest and most prominent form of Aβ in senile plaques is Aβ₄₂, in the monkey, Aβ₄₀-positive plaques predominated. The ratio of Aβ₄₎: Aβ₄₂-positive plaques averaged 2.08 in the monkey, as compared to a mean ratio of 0.37 in 68 human AD subjects (p < 0.001). Aβ₄₀ was also more prominent in the chimpanzee than in humans. Possible explanations for these findings include species differences in the cleavage of Aβ from the amyloid precursor protein or in the activity of a putative carboxy peptidase forming Aβ₄₀ from Aβ₄₂ in situ.     

A new histologic approach to the differentiation of enchondroma and chondrosarcoma of the bones. A clinicopathologic analysis of 51 cases

Fifty-one cases of central, hyalin cartilage tumors of the long and flat bones were analyzed. Although Grade 2 and 3 chondrosarcomas could be diagnosed on the basis of cytologic features alone, low-grade chondrosarcoma could not be adequately differentiated from pure, benign enchondroma(s) by cytology alone. The tumors can be distinguished by a new histologic approach based on tissue patterns. The crucial enchondroma patterns consist of multiple nodules of hyalin cartilage separated by normal marrow in conjunction with partial to complete encompassing plates of lamellar bone that conform to the irregular shapes of the cartilage nodules. The chondrosarcomatous patterns consist of a single confluent mass of cartilage, which commonly permeates the marrow, "trapping" host lamellar bone on all sides, and which forms bands of fibrosis between the confluent peripheral cartilage lobules. Other less common patterns included cartilagenous infiltration of the Haversian systems or marrow fat and/or the development of a soft tissue mass. A central secondary chondrosarcoma is defined as one that shows the combination of both the enchondromatous and chondrosarcomatous patterns. All 18 of the pure enchondromas diagnosed by the methods proposed in this article behaved with strict benignity (i.e., without evidence of recurrence or metastasis with an average follow-up period of 7.2 years). The 33 primary and secondary chondrosarcomas diagnosed using the described patterns behaved with the predicted frequency of recurrence, metastasis, and patient demise.     

Ischemic necrosis of the femoral head. Correlation of magnetic resonance imaging and histologic sections

Magnetic resonance (MR) images were correlated with matched histologic sections of a resected ischemic necrotic femoral head and neck. Preoperative radiographs had revealed Stage 3 ischemic necrosis. Preoperative MR images disclosed foci of abnormally low signal intensity alternating with normal-appearing foci of high signal intensity. Surgical resection of the femoral head and neck was followed by MR imaging and pathologic sectioning, fixation, and staining, enabling the individual sections to be compared with the postoperative MR slices. A subchondral focus of diminished signal intensity was shown to represent saponified fat and consisted of a transudate of proteinaceous material with probable calcifications. The subjacent region of normal, high signal intensity represented "mummified" fat. The next lower stratum of diminished signal intensity was composed of fibrous and vascular tissues and histiocytic infiltrates that had extensively or completely replaced the fatty marrow.     

Self-expanding oesophageal metal stents for the palliation of dysphagia due to extrinsic compression

The role of self-expanding metallic stents is well established in the palliation of oesophageal stenosis and dysphagia due to primary oesophageal malignancy. However, their role in palliation of dysphagia due to external compressive mediastinal malignancies is not well established. The purpose of this study was to assess the efficacy of self-expanding metallic stents in the palliation of dysphagia due to extrinsic oesophageal compression by mediastinal malignancy. Between January 1995 and January 1998, 21 patients with oesophageal compression due to malignant mediastinal tumours underwent oesophageal stent placement for palliation of dysphagia. Complete data were available in 17 patients (10 men and 7 women). The mean age was 63.5 years (range 46–89 years). A total of 19 stents were placed successfully. The dysphagia grade prior to and after oesophageal stent placement was assessed and the complications documented. Of the 17 patients, 16 reported an improvement in dysphagia. The mean dysphagia score improved from 3.1 prior to treatment to 1.3 after treatment. In 1 patient the stent slipped during placement and another stent was placed satisfactorily. Early complications (within 48 h) in the form of mild to moderate retrosternal chest pain occurred in 5 patients. This was treated symptomatically. Late complications (after 48 h) in the form of bolus impaction occurred in 2 patients. This was successfully treated with oesophagoscopy and removal of bolus. In 2 patients the stent was overgrown by tumour and in one of these an additional stent was placed. In 1 patient incomplete closure of a tracheo-oesophageal fistula was observed. There was no procedure- or stent-related mortality. The mean survival time of this group was 2.1 months. Self-expanding metallic stents can be safely and effectively used in the palliation of dysphagia due to external mediastinal malignancies. Received: 21 October 1998; Revised: 1 February 1999; Accepted: 4 February 1999     

Caustic ingestion in childhood: current treatment possibilities and their complications

In a review of 15 pediatric patients who had ingested caustic substances, the authors describe the diagnostic and therapeutic procedures to be followed as well as the complications that may occur with their use. The cases reported include 1 esophageal rupture caused by balloon dilatation and 1 recurrent stenosis treated with a silastic tutor.     

The "ultraperipheral" revascularization in arteriosclerotic obstruction of tibial vessels. Experimental study

In arteriosclerotic obstruction of the main tibial arteries, the tibial collateral vessels are usually patent, and about 70% of these arteries are potentially suitable for surgical revascularization. The present study aimed at investigating the practical feasibility of a selective revascularization procedure on these tibial muscular arteries (ultraperipheral revascularization). Six lower limbs amputated at thigh level for arteriosclerotic gangrene with complete obstruction of the main tibial arteries, were studied: the tibial collateral muscular vessels showed patency in 65% of cases. The authors propose a surgical technique for the revascularization of these peripheral vessels with the use of a vascular prosthesis. The "post-operative" angiographic studies showed that revascularization of these peripheral muscular arteries was possible.     

Confirmatory factor analysis and measurement invariance by gender,age and levels of psychological distress of the short TEMPS-A

Background

The Temperament Evaluation of Memphis, Pisa, Paris and San Diego – Autoquestionnaire (TEMPS-A) is a widely used self-reported tool aimed at measuring the affective temperaments that define the bipolar spectrum, with cyclothymic, depressive, irritable, hyperthymic, and anxious subscales. Confirmatory factor analysis (CFA) was rarely used to confirm the expected five-factor model. Measurement invariance was never tested.

Methods

Cross-sectional, survey design involving 649 Italian college students (males: 47%). The short 39-item TEMPS-A and the 12-item General Health Questionnaire (GHQ-12) were used as measures of the affective temperaments and of psychological distress, respectively. CFA was applied to the TEMPS-A. Measurement invariance by gender, age and levels of psychological distress on the GHQ-12 was calculated with the establishment of subsequent equivalence constraints in the model parameters across groups.

Results

The expected five-factor model had the best fit for all CFA indexes. Configural, metric and scalar invariance of the five-factor model of the TEMPS-A was proved across gender, age and levels of psychological distress of the participants. The hyperthymic temperament subscale has low or no links with the other affective temperament subscales, which were interrelated with medium to large effect sizes.

Limitations

College students might be not representative of the general population. No information on the clinical status of the students was available beyond self-report data.

Conclusion

The study proved the measurement invariance of the (short) TEMPS-A, which is a pre-requisite to compare groups or individuals in cross-sectional and longitudinal surveys. Generalizability cannot be assumed without replication of the findings in clinical samples.