Extracorporeal irradiated tumor bone: A reconstruction option in diaphyseal Ewing��s sarcomas

Background:

Limb salvage in extremity tumors is now established as an oncologically safe option without compromising long-term survival. En bloc resection followed by extracorporeal radiation and reimplantation is a biological reconstruction option in diaphyseal Ewing’s sarcomas. We analyzed the results of 12 cases of diaphyseal Ewing’s sarcomas treated using this modality.

Materials and Methods:

Between March 2006 and March 2008, 12 patients with Ewing’s sarcoma underwent enbloc resection and reconstruction, with reimplantation of the sterilized tumor bone, after extracorporeal irradiation. There were eight males and four females, with a mean age of 14 years (range 2 to 22 years). The femur was the most common bone involved (n=8) followed by the tibia and the humerus (two cases each). All these patients were non-metastatic at presentation and received chemotherapy as per the existing hospital protocol. The mean length of the bone resected was 20 cm (range 11 to 25 cm). The specimen was irradiated with 50 Gy prior to reimplantation and stabilized with the host bone, using suitable internal fixation. Standard biplanar radiographs were assessed for evidence of union on the follow-up visits. The functional status was assessed using the Musculoskeletal Tumor Society Scoring system at the time of the last follow up. The mean follow up duration was 29 months (range 12 to 57 months).

Results:

Two patients (17%) had early infection with graft removal, hence are excluded from any analysis of union, however they are included when analysing complications such as infection. Rest 10 cases were analyzed for bony union at the osteotomy sites. Sixteen (84%) of the 19 osteotomy sites united primarily, without any intervention. Implant failure and non-union was seen at three diaphyseal osteotomy sites. The average time for union of all osteotomy sites was 7.2 months (range 3 to 13 months).The average time for union of the metaphyseal osteotomy sites was 5.9 months (range 3 to 12 months) and of diaphyseal osteotomy sites was 8.3 months (range 4 to 13 months). The mean Musculoskeletal Tumor Society Score was 27 (range 19 to 30) with a mean of 27. Nine of the ten patients with lower limb involvement were independent ambulators without additional aids. At the time of the last review, six patients were free of disease and six patients had died from the disease. There were two recurrences around the operative site. Both were associated with disseminated disease and in both the recurrences were in the soft tissue, away from the irradiated graft.

Conclusion:

Extracorporeal irradiation is a useful, convenient technique for limb salvage in diaphyseal Ewing’s sarcomas when there is reasonable residual bone stock. It is oncologically safe and has good functional results. A radiation dose of 50 Gy for sterilizing the bone ensures adequate tumor kill, while minimizing the deleterious effects on the biomechanical and biological properties of the bone. The use of appropriate implants for adequate internal fixation and supplementary bone grafting at the index surgery may help reduce the need for subsequent additional interventions to achieve union. The limitations of this procedure are that it is not applicable in tumor bones that are structurally weak and in bones with pathological fractures.     

A clinical evaluation of midface advancement using intraoral distractors in management of bone stock deficiencies

Background

Cleft lip and palate patients present large osseous defects of the alveolus and midface hypoplasia. Traditional orthognathic surgery has limitations of relapse and limited age group. Distraction osteogenesis (DO) as a modality for midface advancement is relatively new. Modular internal distractor (MID) offers the advantage of patient compliance and minimal discomfort. The purpose of this study was to evaluate the versatility of intraoral distractors in midface advancement.

Method

Nineteen patients with midface bone stock deficiency were selected for maxillary advancement. At the first surgery under general anaesthesia, after the osteotomy (Le Fort I/II), MID system was used. After distraction, evaluation was done for ease of the procedure, stability, and complications. Lateral cephalograms were evaluated at three stages: T1, pre-distraction; T2, post-distraction; and T3, one year post-distraction.

Results

Significant changes showed 13.3 mm mean midface advancement bone formation at the pterygomaxillary region. The results were stable even at one year follow-up.

Conclusion

Maxillary position improved in relation to the cranial base. This study showed that the MID was versatile in midface advancement in stability and patient compliance with minimal complications.     

Aggressive Approach in a Case of Cancer Cervix with Uremia

Carcinoma of cervix is the most common cancer in developing countries. Majority of them present in locally advanced stages. A 36-year-old lady presented with bleeding and white discharge per vagina since four months, vomiting and reduced urine output since two weeks. Patient had an exophytic cervical growth. Investigation revealed elevated serum creatinine. Patient received single fraction radiation and underwent percutaneous nephrostomy. At one month follow-up, serum creatinine returned to almost normal level. Patient underwent bilateral ante grade stenting and completed concurrent chemoradiotherapy. In selected subsets of patients, aggressive management offered longer palliation and good quality of life.     

Psychometric evaluation of the myasthenia gravis composite using Rasch analysis

Introduction: The MG Composite (MGC) is a validated outcome measure of the clinical manifestations of myasthenia gravis. Methods: We performed Rasch analyses of the MGC to investigate additional properties, including its unidimensionality and the appropriateness of the weights assigned to the response categories for the MGC items. Results: The fit statistics indicated that the 10 items belong together and can be summated for a total score. There was an overall absence of item order distortion between response categories. The Rasch model's expected category response values were compatible with item weights previously assigned. Conclusions: Our analysis suggests that: (1) the score can be summated to estimate an overall disease severity score; (2) the response options of the 10 items are not significantly distorted; and (3) the assigned weights of the response options are appropriate. Muscle Nerve 45: 820–825, 2012     

Expression of CD33, CD38, and HLA-DR on CD34+ human fetal liver progenitors with a high proliferative potential

High proliferative-potential colony-forming cells (HPP-CFC) have been identified in the bone marrow of mice and adult humans, and have been characterized as a compartment of primitive progenitors possibly including stem cells. In this report we describe the human fetal liver (FL) as a source of HPP-CFC. These FL HPP-CFC develop in clonal cultures in the presence of granulocyte-macrophage colony-stimulating factor (GM-CSF) and interleukin-3 (IL-3) within 3 to 4 weeks. The median frequency of HPP-CFC in FL tissues between 16 and 21 weeks of gestational age was 1 in 3,000 total FL cells. After 4 weeks of growth, FL HPP-CFC grew to a median colony size of 8.3 x 10(4) cells/colony. Using cell-sorting techniques FL HPP-CFC were shown to be predominantly contained in the CD34+ CD33+ CD38- fraction of FL cells. FL HPP-CFC were heterogeneous for HLA-DR expression, and no differences in proliferative capacities were observed between HLA-DR+ and HLA-DR- HPP- CFC. The CD34+ CD33-HLA-DR- CD38- population, previously suggested to contain stem cells, was observed to be very rare in the FL, representing approximately 1 in 1.7 x 10(5) light-density FL cells and containing almost no CFC. Therefore, it is possible that stem cells are contained in the CD33+ fraction of FL cells. Phenotypic characterization of CD34+ CD33+ CD38- lin -LDFL cells showed that these cells are also CD13+, predominantly Thy-1+, CD45RA-, CD45RO-, CD71-, and heterogenoeous for c-kit expression. These data suggest that FL HPP- CFC represent a heterogeneous compartment of primitive myeloid progenitors that may include stem cells.