Mediastinal masses - the bad, the ugly and the unusual!

Background: Differential diagnosis of mediastinal masses is wide and management of individual cases can be challenging. In addition to common malignancies e.g. lymphomas and thymomas. Many other benign and malignant conditions can present with mediastinal masses. Patients and Methods: We describe five patients with a diagnosis of mediastinal mass. We wish to showcase the range of diagnosis possible in these situations. This is followed by a brief discussion on the general approach to such cases. Conclusion: A good history, detailed careful clinical examination, judicious use of imaging and investigations e.g. blood counts and tumour makers can give a vital clue to the diagnosis of mediastinal mass

Malignant peripheral nerve sheath tumors (MPNST) – Clinicopathological study and treatment outcome of twenty-four cases

BACKGROUND: Malignant peripheral nerve sheath tumor (MPNST) is biologically an aggressive tumor for which the treatment of choice is the surgery. We reviewed the clinical profile, diagnostic methods, treatment patterns, and outcome of twenty-four MPNST patients in this study. PATIENTS AND METHODS: A retrospective analysis of 24 MPNST patients, treated from 1994 to 2002, in the department of Surgical Oncology at All India Institute of Medical Sciences, New Delhi, was done. A combination of gross, histopathological and immunohistochemical findings, and proliferation markers (MIB1) were considered for diagnosis and grade of the MPNST. Survival analysis was done by the Kaplan-Meier method and differences were evaluated with the log-rank test. Multivariate analysis was carried out by using Cox's proportional hazards model by using SPSS (Version 9, Chicago, Illinois) software. RESULTS: MPNST constituted 12% of all soft tissue sarcomas, where 21% (5/24) of patients had associated Von Recklinghausen's disease (VRHD). A higher incidence of male preponderance and multifocal MPNST were noted in the present series. At a mean follow-up of 38 months, 13 (54 %) patients had relapse of disease and 5-year over all and disease free survival were 58% and 35% respectively. In univariate analysis, sex (p = 0.05), tumor depth (p < 0.03), and cellular differentiation (p < 0.002) were shown to be adverse prognostic factors for disease free survival and sex (p = 0.04), cellular differentiation (p < 0.0004), and tumor grade (p = 0.05) for overall survival. However, in multivariate analysis, cellular differentiation (p < 0.005) and tumor grade (p < 0.01) emerged as independent prognostic factors for both disease free and overall survival, respectively. Postoperative radiotherapy (RT) has shown a definite role in both disease free and overall survival in this study. CONCLUSION: MPNSTs constituted a significant proportion (12%) of soft tissue sarcoma in our medical center. Heterogeneous differentiation and multifocality of the tumor were few distinct features of MPNST. Sex and cellular differentiation were noticed as the new adverse prognostic factors and adjuvant radiotherapy has been proved to be a significant treatment tool in the current series

An unusual presentation of a malignant jejunal tumor and a different management strategy

BACKGROUND: Malignant small bowel tumors are very rare and leiomyosarcoma accounts for less than 15% of the cases. Management of these tumors is challenging in view of nonspecific symptoms, unusual presentation and high incidence of metastasis. In this case report, an unusual presentation of jejunal sarcoma and management of liver metastasis with radiofrequency ablation (RFA) is discussed. CASE PRESENTATION: A 45-year-old male presented with anemia and features of small bowel obstruction. Operative findings revealed a mass lesion in jejunum with intussusception of proximal loop. Resection of bowel mass was performed. Histopathological findings were suggestive of leiomyosarcoma. After 3-years of follow-up, the patient developed recurrence in infracolic omentum and a liver metastasis. The omental mass was resected and liver lesion was managed with radiofrequency ablation. CONCLUSION: Jejunal leiomyosarcoma is a rare variety of malignant small bowel tumor and a clinical presentation with intussusception is unusual. We suggest that an aggressive management approach using a combination of surgery and a newer technique like RFA can be attempted in patients with limited metastatic spread to liver to prolong the long-term survival in a subset of patients

Therapeutic radiological interventional procedures in hepatocellular carcinoma

Background: To improve the survival rate of patients with hepatocellular carcinoma (HCC) in whom surgery is not possible, various methods have been developed employing angiographic and percutaneous techniques. We analyzed our experience with various percutaneous therapeutic interventional techniques done for HCC in our center. Methods: Sixty-one patients with inoperable HCC (mean age 48.9 [SD 13.8] y; 47 men) were treated between January 1997 and December 2000 by transcatheter arterial chemoembolization (TACE) alone (22), TACE with percutaneous alcohol injection (PEI) (20), transcatheter arterial embolization (TAE) with steel coils and gel foam for gastrointestinal bleed (7), percutaneous radiofrequency ablation (1), percutaneous preoperative right portal vein embolization (3) and percutaneous preoperative tumor embolization to reduce blood loss at surgery (8). Results: In 42 patients treated by TACE and PEI and TACE alone, tumor necrosis was scored; over 50% necrosis was seen only after six and nine months in both treatment groups. The survival rates after six and nine months and the median survival were similar in the two groups. Of 7 cases treated with TAE with steel coils and gel foam, the gastrointestinal bleeding stopped in four; in the other three, bleeding did not stop completely although less transfusion was required. In the patient treated by radiofrequency ablation, follow-up contrast-enhanced CT did not show enhancing tumor mass. We noted left lobe enlargement after percutaneous preoperative right portal vein embolization, prior to right hepatectomy. Conclusion: In patients with HCC not amenable to surgical intervention, a variety of percutaneous therapeutic interventional techniques may be used

Hybrid SPECT-CT for characterizing isolated vertebral lesions observed by bone scintigraphy: comparison with planar scintigraphy, SPECT, and CT

PURPOSEWe aimed to assess the role of single photon emission computed tomography-computed tomography (SPECT-CT) for characterizing isolated vertebral lesions observed by bone scintigraphy compared to planar scintigraphy, SPECT, and CT, and to evaluate the impact of SPECT-CT on patient management. MATERIALS AND METHODSData from 99 patients (mean age, 52.4±18.9 years; females, 58.5%) with 108 isolated vertebral lesions visible on planar bone scintigraphy, who had undergone SPECT-CT of a selected volume, were retrospectively analyzed. Planar scintigraphy, SPECT, CT, and SPECT-CT images were independently evaluated in separate sessions to minimize recall bias. A scoring scale of 1 to 5 was used, with 1 being definitely metastatic, 2 most likely metastatic, 3 indeterminate, 4 most likely benign, and 5 definitely benign. Sensitivity, specificity, and predictive values were calculated; a score ≤3 was defined as metastatic. The areas under the receiver operating characteristic curve were calculated and compared. Clinical and imaging followup with or without histopathology were used as a reference standard. RESULTSAmong the 108 lesions, 49 were indeterminate on planar scintigraphy, 16 on SPECT, and one each on SPECT-CT and CT. SPECT-CT was superior to both planar scintigraphy (P < 0.001) and SPECT alone (P = 0.014), but not to CT (P = 0.302). CT was superior to planar scintigraphy (P < 0.001) but only slightly superior to SPECT (P = 0.063). SPECT-CT correctly characterized 96% of the indeterminate lesions observed by planar scintigraphy. SPECT-CT had an impact on the clinical management of 60.6% patients compared to planar scintigraphy and 18.1% compared to SPECT. CONCLUSIONSPECT-CT is better than planar scintigraphy and SPECT alone, but not CT alone, for characterizing equivocal vertebral lesions that are observed by bone scintigraphy, thus SPECT-CT can have a significant impact on patient management

Engraftment Syndrome: A Retrospective Analysis of the Experience at a Tertiary Care Institute

Engraftment syndrome (ES) is a clinical syndrome that occurs in the early neutrophil recovery phase following hematopoietic stem cell transplant (HSCT). Although also described for allogenic HSCT, it is basically diagnosed in the context of autologous HSCT. We retrospectively reviewed 171 consecutive HSCTs performed between January 2013 and January 2015 in our Bone Marrow Transplant (BMT) unit and analyzed all cases of noninfectious fever and strong clinical features suggestive of ES in the peri-engraftment period for up to 7 days. We observed the incidence of ES to be 12.3% (16/130) in the autologous and 4.8% (2/41) in the allogeneic cohort. Among plasma cell disorders, which constitute 50% of our study population, the incidence of ES was 19.7%. Among the ES cases of autologous transplants, 81.2% (13/16) patients satisfied the Maiolino criteria (MC) and 87.5% (14/16) patients the Spitzer diagnostic criteria (SC). A total of 68.7% (11/16) patients satisfied both MC and SC, and two patients (12.5%) did not satisfy either (MC− SC−). There was no significant difference in days of hospitalization and usage of supportive care between ES and non-ES patients, and there was no mortality due to ES. On univariate analysis, female patients (p < 0.013) and those with diagnosis of a plasma cell disorder (p < 0.03) had higher risk of ES. In conclusion, the incidence of ES in our study population is consistent with that of many others, but severity evaluation needs exploration in larger cohorts with pragmatically modified diagnostic criteria

Preliminary experience of fractionated stereotactic radiosurgery with extend system of Gamma Knife

Purpose: The purpose of this study is to present multisession stereotactic radiosurgery with initial experience using custom made extend system (ES) of Gamma Knife.Methods: The ES is comprised of a carbon fiber frame also called extend frame, vacuum head rest cushion, patient surveillance unit and a configurable front piece with dental impression tray. The extend frame is a rigid connection between patient's head and patient positioning system (PPS) of Gamma Knife. A dental impression of patient was created and attached to the frontal piece of extend system. The treatment setup involves positioning the patient within the extend frame using patient specific headrest cushion and front piece. The reference patient’s head position was recorded through measurements of repositioning check tool (RCT) apertures using a high precision digital probe before computed tomography (CT) scan. The RCT measurements taken before treatment were compared with recorded reference position to ensure appropriate patient treatment position. Volumetric magnetic resonance (MR) scan was co-registered with stereotactic CT scan on Leksell Gamma plan. Fused MR to CT images on Gamma Plan was utilized to delineate regions of interest and prepare a precise treatment plan. The presented study includes positional reproducibility check and dosimetric evaluation of ten patients treated with ES.Results: Forty-three fractions on ten patients with prescribed treatment format were delivered successfully. An average tumor volume of 11.26 cm3 (range, 340 mm3 to 59.12 cm3) was treated with ES. The mean tumor coverage of 91.91% (range, 90% to 95%) was able to achieve at 50% prescription isodose without compromising adjacent normal structure radiation dose tolerances. The mean inter-fraction positional variation of 0.69 mm influences an inherent strength of immobilization technique. Follow-up of seven patients at a median interval of 16 months (range, 9 months to 26 months) showed evidence of 100% radiographic control with improved clinical results.Conclusion: Conjugative clinical outcome shows the efficacy of fractionation in various clinical indications

Induction Therapy with Novel Agents and Autologous Stem Cell Transplant Overcomes the Adverse Impact of Renal Impairment in Multiple Myeloma

We investigated the impact of renal impairment (RI) on the outcome in multiple myeloma (MM) patients following induction and autologous stem cell transplantation (ASCT). Among 349 patients who received a first ASCT for MM, 86 (24.6%) had RI at diagnosis, defined as estimation of glomerular filtration rate (eGFR) <40 mL/min/1.73 m2 according to the modification of diet in renal disease (MDRD) formula. Post induction reversal of renal function occurred in 68 (79%) patients including complete renal response in 37.2%. Two hundred and fifty-one patients had received novel agents for induction; posttransplant complete response (CR) rates were 71.4% for patients with renal impairment (RI) versus 67.2% in those without RI, p = 0.23. The quality of stem cell collection and days to engraftment were similar except that patients with RI required higher transfusion numbers of packed red cells (p < 0.002) and platelets (p < 0.007). The median overall survival (OS) was 96 months (95% confidence interval [CI] 72.80–119.20) for patients with eGFR ≥40 mL/min, n = 195) versus 62 months (95% CI 28.7–95.3) for 56 patients with RI (eGFR <40 mL/min), p = 0.15. The 5-year OS was 64.6% versus 54.4%, respectively. The median progression-free survival (PFS) was 52 months (95% CI 36.3–67.7) for patients with eGFR ≥40 mL/min versus “not reached” for those with eGFR <40 mL/min p = 0.87; and the 5-year PFS was 48.1% versus 51%, respectively. We conclude that induction with novel agents results in reversal of renal dysfunction in the majority of patients. Consolidation with Hemopoietic Stem Cell Transplantation (HSCT) overcomes the adverse impact of RI on survival

Utility and potential of bedside ultrasound in palliative care

Bedside ultrasound is an important tool in modern palliative care practice. It can be utilized for rapid diagnostic evaluation or as an image guidance to perform invasive therapeutic procedures. With advent of portable ultrasound machines, it can also be used in community or home care settings, apart from palliative care wards. Major applications of bedside ultrasound include drainage of malignant pleural effusions and ascites, nerve blocks, venous access, evaluation of urinary obstruction, deep vein thrombosis and abscesses. Bedside ultrasound leads to better clinical decision-making as well as more accurate and faster invasive therapeutic procedures. It also enhances patient comfort and reduces cost burden. However, use of bedside ultrasound is still not widespread among palliative care givers, owing to initial cost, lack of basic training in ultrasound and apprehensions about its use. A team approach involving radiologists is important to develop integration of bedside ultrasound in palliative care