Large duodenal GIST with massive liver secondaries melting under Imatinib: a case report

Gastrointestinal stromal tumors(GIST) have become a well established entity and its taxonomy is no more ambiguous. Better understanding of the cell of origin and immunohistochmical markers have made this possible. Their treatment has been revolutionized with the advent of targeted molecular therapy, namely Imatinib mesylate. Herein we report a rare and interesting case of a thirty year old South Indian Lady with an extremely large Duodenal GIST with massive Liver secondaries. The phenomenon of metastatic GIST responding to Imatinib mesylate is not new. What is interesting in this case is the enormous tumor load at the time of presentation as exemplified by the cross sectional images. This kind of tumor response and patient survival deserves documentatio

Construct validation of the Amblyopia and Strabismus Questionnaire (A&SQ) by factor analysis

Background: The Amblyopia and Strabismus Questionnaire (A&SQ) was previously developed to assess quality of life (QoL) in amblyopia and/or strabismus patients. Here, factor analysis with Varimax rotation was employed to confirm that the questions of the A&SQ correlated to dimensions of quality of life (QoL) in such patients. Methods: Responses on the A&SQ from three groups were analyzed: healthy adults (controls) (n = 53), amblyopia and/or strabismus patients (n = 72), and a historic cohort of amblyopes born between 1962-1972 and occluded between 1968-1974 (n = 173). The correlations among the responses to the 26 A&SQ items were factor-analysed by Principal Component Analysis (PCA). As the development of the A&SQ was intuitive-deductive, it was expected that the pattern of correlation could be explained by the five a priori hypothesized dimensions: fear of losing the better eye, distance estimation, visual disorientation, diplopia, and social contact and cosmetic problems. Distribution of questions along the factors derived by PCA was examined by orthogonal Varimax rotation. Results: Data from 296 respondents were analyzed. PCA provided that six factors (cutoff point eigenvalue >1.0) accumulatively explained 70.5% of the variance. All A&SQ dimensions but one matched with four factors found by Varimax rotation (factor loadings >0.50), while two factors pertained to the fifth dimension. The six factors explained 33.7% (social contact and cosmetic problems); 10.3% (near distance estimation); 8.7% (diplopia); 7.2% (visual disorientation); 6.3% (fear of losing the better eye); and 4.3% (far distance estimation), together 70.48% of the item variance. Conclusion: The highly explained variance in the A&SQ scores by the factors found by the PCA confirmed the a priori hypothesized dimensions of this QoL instrument

Primary intra-abdominal malignant fibrous histiocytoma presenting as pyrexia of unknown origin – report of a case with review of literature

Primary intra-abdominal malignant mesenchymal tumours are very rare and there are not many cases of visceral malignant fibrous histiocytoma in the English literature. We report a new case of abdominal malignant fibrous histiocytoma presenting as abdominal pain and pyrexia of unknown origin in a 54 year old female followed by a brief review of literature. Presentation with pyrexia of unknown origin is extremely rare in this condition

A phase I trial of weekly gemcitabine and concurrent radiotherapy in patients with locally advanced pancreatic cancer

This study investigated the maximum-tolerated dose of gemcitabine based on the frequency of dose-limiting toxicities of weekly gemcitabine treatment with concurrent radiotherapy in patients with locally advanced pancreatic cancer. Fifteen patients with locally advanced pancreatic cancer that was histologically confirmed as adenocarcinoma were enrolled in this phase I trial of weekly gemcitabine (150–350 mg m−2) with concurrent radiotherapy (50.4 Gy in 28 fractions). Gemcitabine was administered weekly as an intravenous 30-min infusion before radiotherapy for 6 weeks. Three of six patients at the dose of 350 mg m−2 of gemicitabine demonstrated dose-limiting toxicities involving neutropenia/ leukocytopenia and elevated transaminase, while nine patients at doses of 150 mg m−2 and 250 mg m−2 did not demonstrate any sign of dose-limiting toxicity. Of all 15 enrolled patients, six patients (40.0%) showed a partial response. More than 50% reduction of serum carbohydrate antigen 19-9 level was observed in 13 (92.9%) of 14 patients who had pretreatment carbohydrate antigen 19-9 levels of 100 U ml−1 or greater. The maximum-tolerated dose of weekly gemcitabine with concurrent radiotherapy was 250 mg m−2, and this regimen may have substantial antitumour activity for patients with locally advanced pancreatic cancer. A phase II trial of weekly gemcitabine at the dose of 250 mg m−2 with concurrent radiation in patients with locally advanced pancreatic cancer is now underway

Primary temporal bone angiosarcoma: a case report.

We present a rare case of temporal bone angiosarcoma diagnosed in a 26-year-old female patient at 36 week of pregnancy. The patient was referred with a 2 months history of left otalgia and tinnitus with a tender swelling above the mastoid. Cranial imaging studies showed a 7 x 5 x 4 cm hypervascularized mass located in the left middle fossa with lysis of the temporal bone and extension to the subcutis. After the baby was delivered by caesarean section, the patient entered the oncology protocol. Selective embolization of the feeding vessels was followed by gross total surgical resection using a combined supra- and infra-tentorial approach. Pathological findings were those of a poorly differentiated, highly malignant sarcoma with a large epitheloid component and immunohistochemical evidence of endothelial differentiation (CD31, Factor VIII related antigen, CD34), consistent with an angiosarcoma with epitheloid features. No extra-cranial tumor was found after extensive staging. The patient received adjuvant radiotherapy followed by a course of chemotherapy consisting of 6 cycles of paclitaxel. At 15 months follow-up, she developed multiple distant metastasis to a left postauricular lymph node and to the lungs and ribs. The patient was given a second line chemotherapy using doxorubicine and ifosfamide. Despite an initial good response, she died with metastatic disease 26 months after diagnosis. We present a rare case of primary temporal bone angiosarcoma and report our experience with a multimode therapeutic approach combining surgery, radiotherapy and chemotherapy.Peer reviewe

Elevated reticulocyte count – a clue to the diagnosis of haemolytic-uraemic syndrome (HUS) associated with gemcitabine therapy for metastatic duodenal papillary carcinoma: a case report

In adults, the haemolytic-uraemic syndrome (HUS) is associated with probable causative factors in the minority of all cases. Cytotoxic drugs are one of these potential causative agents. Although metastatic cancer by itself is a recognized risk-factor for the development of HUS, therapy with mitomycin-C, with cis-platinum, and with bleomycin carries a significant, albeit extremely small, risk for the development of HUS, compared with all other cytotoxic drugs. Gemcitabine is a novel cytotoxic drug with promising activity against pancreatic adenocarcinoma. We are reporting on one patient with metastatic duodenal papillary carcinoma developing HUS while on weekly gemcitabine therapy. The presenting features in this patient were non-cardiac pulmonary oedema, renal failure, thrombocytopenia and haemolytic anaemia. The diagnosis of HUS was made on the day of admission of the patient to this institution. Upon aggressive therapy, including one single haemodialysis and five plasmaphereses, the patient recovered uneventfully, with modestly elevated creatinine-values as a remnant of the acute illness. Re-exposure to gemcitabine 6 months after the episode of HUS instituted for progressive carcinoma, thus far has not caused another episode of HUS. © 1999 Cancer Research Campaig

Intensive weekly chemotherapy is not effective in advanced pancreatic cancer patients: a report from the Italian Group for the Study of Digestive Tract Cancer (GISCAD)

Twenty-two patients, with locally advanced unresectable and/or metastatic pancreatic carcinoma, received weekly administration of cisplatin 40 mg m−2, 5-fluorouracil 500 mg m−2, epidoxorubicin 35 mg m−2, 6S stereoisomer of leucovorin 250 mg m−2 and glutathione 1.5 mg m−2, supported by a daily administration of lenograstim at a dose of 5 μg kg−1. Nineteen patients were men and three were women. Median age was 63 years (range 47–70). At study entry, pain was present in 15 out of 22 patients (68%) with a mean value of Scott–Huskisson scale of 27.6 ± 23.8, whereas a weight loss >10% was present in 15 patients. After eight weekly treatments, three partial responses were achieved for a response rate of 13% (95% CI 0–26%), five patients had stable disease and 14 progressed on therapy. Pain was present in 9 out of 22 patients (40%) with a mean value of Scott–Huskisson scale of 12.3 ± 18.4. Eight patients (36%) (three partial response and five stable disease) had a positive weight change. Toxicity was mild: WHO grade III or IV toxicity was recorded in terms of anaemia in 7 out of 188 cycles (3.7%), of neutropenia in 9 out of 188 cycles (4.7%) and of thrombocytopenia in 3 out of 188 cycles (1.5%). Median survival of all patients was 6 months. The outcome of this intensive chemotherapy regimen does not support its use in pancreatic cancer. © 1999 Cancer Research Campaig

A combination of gemcitabine and 5-fluorouracil in advanced pancreatic cancer, a report from the Italian Group for the Study of Digestive Tract Cancer (GISCAD)

In a randomized clinical trial, gemcitabine (GEM) was more effective than 5-fluorouracil (5-FU) in advanced pancreatic cancer patients. GEM and 5-FU have different mechanisms of action and their combination, from a theoretical point of view, could result in a higher activity. To test activity and feasibility of such a combination, a multi-institutional phase II study was initiated in November 1996 by the Italian Group for the study of Digestive Tract Cancer (GISCAD). Primary objectives of this study were to determine the activity in terms of response rate and clinical benefit, while the secondary objective was toxicity. According to the optimal two-stage phase II design, 54 patients were enrolled. Schedule was: GEM 1000 mg m(-2) intravenous (i.v.), and 5-FU 600 mg m(-2) bolus i.v. weekly for 3 weeks out of every 4. All the 54 patients were symptomatic (pain, weight loss, dyspepsia). A clinical benefit was obtained in 28 patients (51\%) (95\% confidence interval (CI) 38-64\%). Two patients achieved a partial response and 34 a stable disease. Median survival for all the patients was 7 months. Side-effects were mild: no gastrointestinal or haematological grade 3-4 toxicity (WHO) were recorded. We observed only six episodes of grade 2 (WHO) leukopenia and seven episodes of thrombocytopenia. Although the non-randomized design of this study suggests caution in the interpretation of these data, in consideration of the low incidence of toxicity and the favourable results obtained in terms of clinical benefit, it may be worthwhile to test more active schedules of 5-FU (continuous infusion) in combination with gemcitabine