An intramedullary capillary hemangioma of the spine with an underlying plasmocytoma.

BACKGROUND CONTEXT: In contrast to vertebral hemangiomas, which are very common within the general population, only 3% to 5% of patients with plasma cell dyscrasia show a single osteolytic bone lesion due to plasma cell infiltration without the evidence of generalized myeloma. The vast majority of these hemangiomas are completely asymptomatic and only discovered incidentally. In rare occasions, representing only 1% to 2% of the known lesions, a locally aggressive subtype can cause problems analogous to the ones triggered by a plasmocytoma, ranging from back pain to vertebral compression fractures to neurologic deficit, resulting from nerve root or spinal cord compression. Both entities are extensively discussed in the literature, but finding both lesions in one is rare if not described for the first time. PURPOSE: To advise colleagues that the differential diagnosis between benign and malignant vertebral tumors can be harder than expected and has to be definitely made to avoid severe consequences for the patient. PATIENT SAMPLE: A 46-year-old healthy man presented to the emergency department with an acute onset of thoracic back pain after a trivial incident. Although his medical history included no known diseases and no history of back pain, plain X-rays raised the clear suspicion of a fracture of T6 that was verified in computed tomography scans. OUTCOME MEASURES: Visual analog scale; neurologic status; tumor recurrence. METHODS: The case of the patient was evaluated retrospectively according to standard procedures, clinical outcome, and in review of the literature. RESULTS: Because there is still controversy about the best treatment (local radiation vs. operation vs. combination) of a solitary skeletal plasmocytoma, no gold standard has been established until now. Especially if a patient needs an emergency operation before all test results are obtained, each surgeon has to decide individually. CONCLUSIONS: Capillary hemangiomas can hide underlying plasmocytomas, which might demand totally different treatment strategies. Although our patient did not match the common criteria for a solitary plasmocytoma, one has to discuss whether a stand-alone decompression and biopsy would have been the emergency treatment of choice. Such a strategy would have reduced the risk of tumor spreading and would have made radiotherapy easier, whereas on the other hand requiring a secondary stabilization procedure later on.

Endobronchial lipomatous hamartoma: an incidental finding in a patient with atrial fibrillation-a case report.

Introduction. Lung hamartomas are the most common benign tumors of the lung. Typically, they are located in the peripheral lung, while an endobronchial localisation is rare. Case Presentation. We present a case with the rare diagnosis of an endobronchial hamartoma as incidental finding in a 69-year-old male, caucasian patient with atrial fibrillation. At first admission, the patient's exertional dyspnea was caused by atrial fibrillation. Relapse of exertional dyspnea in the absence of arrhythmia was due to postobstructive pneumonia caused by an endobronchial hamartoma. Conclusion. Endobronchial tumors such as endobronchial lipoma or hamartoma should be considered as potential causes of exertional dyspnea and thus as differential diagnosis of atrial fibrillation. Although endobronchial hamartomas are benign, resection is recommended to prevent postobstructive lung damage.

Diffusion-weighted echo-planar magnetic resonance imaging for the assessment of tumor cellularity in patients with soft-tissue sarcomas.

PURPOSE: To investigate the eligibility of diffusion-weighted imaging (DWI) for the evaluation of tumor cellularity in patients with soft-tissue sarcomas. MATERIALS AND METHODS: Thirty consecutive patients with a total of 31 histologically-proven soft-tissue sarcomas prospectively underwent magnetic resonance imaging (MRI) including DWI with echo-planar imaging (EPI) technique immediately before open biopsy (N = 1) or tumor resection (N = 30). Fourteen patients had no previous anticancer treatment, 16 had received neoadjuvant therapy. Tumor cellularity as determined from histological sections was compared with minimum apparent diffusion coefficient (ADC). RESULTS: Tumor cellularity correlated well with minimum ADC in a linear fashion, with a Pearson correlation coefficient of -0.88 (95% confidence interval [CI]: -0.75 to -0.96). This relationship was not influenced by prior anticancer treatment. There was only a tendency toward lower ADC in tumor with higher grading but no significant dependency (P = 0.08). CONCLUSION: DWI has proven useful for the assessment of tumor cellularity in soft-tissue sarcomas. In result, DWI may be used as a powerful noninvasive tool to monitor responses of cytotoxic treatment as reflected by changes in tumor cellularity.

Experimental treatment of pancreatic cancer with two novel histone deacetylase inhibitors.

AIM: To investigate in vitro and in vivo treatment with histone deacetylase inhibitors NVP-LAQ824 and NVP-LBH589 in pancreatic cancer. METHODS: Cell-growth inhibition by NVP-LAQ824 and NVP-LBH589 was studied in vitro in 8 human pancreatic cancer cell lines using the 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide (MTT) assay. In addition, the anti-tumoral effect of NVP-LBH589 was studied in a chimeric mouse model. Anti-tumoral activity of the drugs was assessed by immunoblotting for p21(WAF-1), acH4, cell cycle analysis, TUNEL assay, and immunohistochemistry for MIB-1. RESULTS: In vitro treatment with both compounds significantly suppressed the growth of all cancer cell lines and was associated with hyperacetylation of nucleosomal histone H4, increased expression of p21(WAF-1), cell cycle arrest at G2/M-checkpoint, and increased apoptosis. In vivo, NVP-LBH589 alone significantly reduced tumor mass and potentiated the efficacy of gemcitabine. Further analysis of the tumor specimens revealed slightly increased apoptosis and no significant reduction of cell proliferation. CONCLUSION: Our findings suggest that NVP-LBH589 and NVP-LAQ824 are active against human pancreatic cancer, although the precise mechanism of in vivo drug action is not yet completely understood. Therefore, further preclinical and clinical studies for the treatment of pancreatic cancer are recommended.

Infiltration of the pes anserinus complex by an extraarticular diffuse-type giant cell tumor (D-TGCT).

This report describes the case of a 26-year-old woman with a recurrent extraarticular diffuse-type tenosynovial giant cell tumor (D-TGCT) of the medial region of the knee affecting the pes anserinus and hamstring tendons. Presurgical MRI did not exclude infiltrative properties of the tumor. In the histological evaluation, the tumor showed an aggressive dispersion by infiltrating the collagenous tissue of the hamstring tendons. The treatment included a resection of the pes anserinus complex with distal semitendinosus and gracilis tendons. Regarding extraarticular D-TGCT a review of the literature showed a predominant affection of the medial region of the knee and thigh.

Inhibition of histone deacetylase for the treatment of biliary tract cancer: a new effective pharmacological approach.

AIM: To investigate in vitro and in vivo therapeutic effects of histone deacetylase inhibitors NVP-LAQ824 and NVP-LBH589 on biliary tract cancer. METHODS: Cell growth inhibition by NVP-LAQ824 and NVP-LBH589 was studied in vitro in 7 human biliary tract cancer cell lines by MTT assay. In addition, the anti-tumoral effect of NVP-LBH589 was studied in a chimeric mouse model. Anti-tumoral drug mechanism was assessed by immunoblotting for acH4 and p21( WAF-1/CIP-1), PARP assay, cell cycle analysis, TUNEL assay, and immunhistochemistry for MIB-1. RESULTS: In vitro treatment with both compounds significantly suppressed the growth of all cancer cell lines [mean IC(50) (3 d) 0.11 and 0.05 micromol/L, respectively], and was associated with hyperacetylation of nucleosomal histone H4, increased expression of p21( WAF-1/CIP-1), induction of apoptosis (PARP cleavage), and cell cycle arrest at G2/M checkpoint. After 28 d, NVP-LBH589 significantly reduced tumor mass by 66% (bile duct cancer) and 87% (gallbladder cancer) in vivo in comparison to placebo, and potentiated the efficacy of gemcitabine. Further analysis of the tumor specimens revealed increased apoptosis by TUNEL assay and reduced cell proliferation (MIB-1). CONCLUSION: Our findings suggest that NVP-LBH589 and NVP-LAQ824 are active against human biliary tract cancer in vitro. In addition, NVP-LBH589 demonstrated significant in vivo activity and potentiated the efficacy of gemcitabine. Therefore, further clinical evaluation of this new drug for the treatment of biliary tract cancer is recommended.

Achondrogenesis Type IA (Houston-Harris): a still-unresolved molecular phenotype.

Achondrogenesis type IA (Houston-Harris) is an extremely rare lethal chondrodysplasia with a characteristic severe disarrangement of endochondral ossification. The growth plate cartilage completely lacks columnar-zone formation and shows chondrocyte expansion due to intracellular vacuoles. This article on a new case of achondrogenesis type IA confirms these findings and demonstrates, on the ultrastructural level, the retention of fine fibrillar material within the rough endoplasmic reticulum (rER). Molecular analysis in the presented case of achondrogenesis type IA did not reveal mutations in the COL2A1 and SLC26A2 genes, which are known to cause achondrogenesis types IB and type II. Although the extracellular cartilage matrix was severely altered, all of the investigated matrix molecules (collagens, aggrecan, matrilins, cartilage oligomeric protein [COMP]) showed a normal distribution pattern. The only exception was type-X collagen, which was significantly reduced. Overall, our study suggests a disturbance in cartilage matrix assembly in the present case due to the retention of some sort of matrix component within the rER. Presumably, as a consequence of this event, processes of chondrocyte maturation and differentiation and endochondral bone formation are severely affected in this case of achondrogenesis type IA.

Where should implants be anchored in the humeral head?

To determine histomorphometric and bone strength distribution of the proximal humerus, analyses were done on 24 freshly harvested human cadaveric humeri. Median ages of 46 and 69 years were recorded respectively for the male group (n = 11; minimum, 34 years; maximum, 76 years) and the female group (n = 13; minimum, 46 years; maximum, 90 years). The humeral head was sliced into four equal horizontal levels (Levels 1-4). Five regions of interest were defined in each cutting plane: anterior, posterior, lateral, medial, and central. Histomorphometric analyses evaluated structural parameters (tissue volume to bone volume ratio, trabecular thickness), connectivity (number of nodes, node to node length), and trabecular orientation (mean bone length). The peak values of histomorphometric parameters and bone strength were identified for the cranial section and decreased caudally. The medial and dorsal aspects of the proximal humeral head were found to be the areas of highest bone strength. The trabecular network formed a pattern that connected the center of the gleaned cavity. The structural and connectivity parameters, bone strength, and trabecular orientation showed region- and level-related characteristics. Knowledge of distribution, microstructure, and quality of bone in the humeral head allows the remaining bone stock to be used effectively, even in elderly patients, with a minimally invasive approach and maximum mechanical stability.