Ashish Gulia

The outcome of the treatment of diaphyseal primary bone sarcoma by resection, irradiation and re-implantation of the host bone

We analysed the outcome of patients with primary non-metastatic diaphyseal sarcomas who had en bloc resection with preservation of the adjoining joints and reconstruction with re-implantation of sterilised tumour bone after extracorporeal radiation (50 Gy). Between March 2005 and September 2009, 32 patients (16 Ewings sarcoma and 16 osteogenic sarcoma) with a mean age of 15 years (2 to 35) underwent this procedure. The femur was the most common site in 17 patients, followed by the tibia in 11, humerus in three and ulna in one. The mean resected length of bone was 19 cm (10 to 26). A total of 31 patients were available at a mean follow-up of 34 months (12 to 74). The mean time to union for all osteotomy sites was 7.3 months (3 to 28): metaphyseal osteotomy sites united quicker than diaphyseal osteotomy sites (5.8 months (3 to 10) versus 9.5 months (4 to 28)). There were three local recurrences, all in soft-tissue away from irradiated graft. At the time of final follow-up, 19 patients were free of disease, one was alive with disease and 11 had died of disease. The mean Musculoskeletal Tumor Society Score for 29 patients evaluated at the last follow-up was 26 (9 to 30). Extracorporeal irradiation is an oncologically safe and inexpensive technique for limb salvage in diaphyseal sarcomas and has good functional results.

Outcomes after limb sparing resection in primary malignant pelvic tumors

AIM To evaluate morbidity, oncologic results and functional outcome in patients with malignant tumors of pelvis treated with limb sparing resection. METHODS Between March 2002 and November 2010, 106 cases of non metastatic malignant pelvic tumors were treated with limb sparing resections of pelvis. Diagnosis included chondrosarcoma (65), Ewings sarcoma (25), osteogenic sarcoma (10), synovial sarcoma (3) and malignant fibrous histiocytoma, high grade sarcoma, epitheloid hemangiothelioma (1 each). Three patients had intralesional surgery because of erroneous pre-operative diagnosis of benign tumor and were excluded from final analysis. Remaining 103 patients underwent limb sparing resections with intent to achieve tumor free margins. In 1 case, an intraoperative cardiac event lead to the surgery being abandoned. Reconstruction was done in 2 of 38 cases that did not include resection of acetabulum. For 64 resections involving acetabulum various reconstruction modalities were used. RESULTS Surgical margins were involved in 20 patients. Forty five patients had complications. 91 patients were available for follow up. Follow up of survivors ranged from 24 to 122 months (mean 55 months).Twenty one patients (23%) had local recurrence. Sixty patients are currently alive, 46 being continuously disease free. Overall survival was 67% at 5 years. Patients in whom acetabulum was retained had better function (mean MSTS score 27) compared to patients in whom acetabulum was resected (mean MSTS score 22). CONCLUSIONS Though complex and challenging, limb sparing surgery in non metastatic malignant tumors is oncologically safe and has better functional outcomes than after an amputation surgery.

Ulnar translocation after excision of a Campanacci grade-3 giant-cell tumour of the distal radius: AN EFFECTIVE METHOD OF RECONSTRUCTION

Between June 2005 and March 2008, 14 patients with a Campanacci grade-3 giant-cell tumour of the distal radius were treated by en bloc resection and reconstruction by ulnar translocation with arthrodesis of the wrist. The mean length of radius resected was 7.9 cm (5.5 to 15). All the patients were followed to bony union and 12 were available at a mean follow-up of 26 months (10 to 49). The mean time to union was four months (3 to 7) at the ulnocarpal junction and five months (3 to 8) at the ulnoradial junction. All except one patient had an excellent range of pronation and supination. The remaining patient developed a radio-ulnar synostosis. The mean Musculoskeletal Tumor Society score was 26 (87%, range 20 to 28). Three patients had a soft-tissue recurrence, but with no bony involvement. They underwent a further excision and are currently well and free from disease. Ulnar translocation provides a local vascularised bone graft to reconstruct the defect left after excision of the distal radius for giant cell tumour. It avoids the need for a microvascular procedure while retaining rotation of the forearm and good function of the hand.

Results of surgical resection in pelvic Ewing's sarcoma.

To evaluate the results of patients with non‐metastatic Ewings sarcoma of the pelvis treated with surgical resection as part of their multimodality treatment

Primary intraosseous myoepithelioma arising in the iliac bone and displaying trisomies of 11, 15, 17 with del (16q) and del (22q11)—A rare case report with review of literature

Mixed tumors are uncommonly observed in the musculoskeletal system, where they form a common spectrum with a myoepithelioma and a parachordoma. Herein, we present a rare case of a mixed tumor/myoepithelioma arising in the iliac bone of a young adult male who presented with swelling in his right hip. Radiological imaging disclosed a large, intraosseous, lytic, heterogenous mass with a soft tissue component. Biopsy and subsequent tumor resection showed an 18cm sized tumor involving the iliac bone and soft tissues and comprising polygonal and spindly cells, arranged in cords and aggregates, embedded in a myxohyaline stroma with osteochondroid differentiation. Tumor cells exhibited mild nuclear variation, rare mitotic figures, focal cytoplasmic clearing, and prominent squamous differentiation. On immunohistochemistry (IHC), tumor cells were diffusely positive for S100-P, EMA, CK5/6, p63, GFAP, calponin, and focally positive for CK/MNF116, but negative for Brachyury/T. Diagnosis of a myoepithelioma/mixed tumor was offered. Further, cytogenetic analysis revealed lack of EWSR1 gene rearrangement and showed clonal trisomies of 11, 15, 17 with del (16q) and del (22q11). The present case is a rare documentation of a myoepithelioma in the appendicular bones and the second such case identified in the iliac bone. IHC and cytogenetic findings supported a myoepithelial cell origin, and reinforced its relationship with a parachordoma and its distinction from mixed salivary gland tumors, a chordoma, and an extraskeletal myxoid chondrosarcoma that form its differential diagnoses.

Chemotherapy compliance in patients with osteosarcoma

Histological response (HR) to neoadjuvant‐chemotherapy (NACT) is considered as a robust prognostic marker in treated osteosarcomas. Chemotherapy compliance can affect both, dose intensity and density and may affect the final outcome in these cases. This vital aspect has been inadequately addressed and therefore merits further investigation.

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.

Melanotic neuroectodermal tumor of infancy in thigh of an infant—a rare case report with diagnostic implications

Melanotic neuroectodermal tumor of infancy (MNTI) is an uncommon tumor of neural crest origin, almost invariably identified in the head and neck region. It is a relatively benign tumor but can recur and rarely metastasize. Herein, a rare case of MNTI in the thigh of an infant is documented. Radiographic imaging showed a sclerotic and lytic lesion in the metadiaphyseal region of the right femur with a soft tissue component. The clinicoradiological impression was of a malignant round cell tumor and osteomyelitis. The patient’s urinary vanil mandelic acid (VMA) level was elevated. FNAC smears revealed a tumor with round to polygonal cells, focally containing black melanin pigment. Biopsy and tumor excision revealed a tumor with cells in cords and nests embedded in a fibrocollagenous stroma and showed bone and soft tissue infiltration. On immunohistochemistry, tumor cells displayed immunoreactivity to vimentin, cytokeratin (CK), CK7, CK19, and MIC2, and focal reactivity for synaptophysin and HMB45. Diagnosis of an MNTI was made. The patient underwent induction chemotherapy with extracorporeal radiotherapy, followed by a soft tissue excision that revealed residual tumor. Surgically, free marrow margins were accomplished, and the patient is currently being followed-up. To the best of our knowledge this case is the fifth documented case in the English literature of MNTI in the thigh. The diagnostic and therapeutic implications are discussed.

Revision of Broken Knee Megaprostheses: New Solution to Old Problems

BackgroundLow-cost indigenous megaprostheses used in the developing world are prone to mechanical failure but the frequency and causes are not well established.Questions/purposesWe retrospectively analyzed the causes of failure, particularly design, and suggest changes to reduce the breakage. We also report our experience with revision surgery.MethodsWe identified 28 breakages in 266 megaprosthetic knee arthroplasties performed between January2000 and December 2006. Twenty-six breakages were revised to another prosthesis. The complications were studied and the function was evaluated. Prostheses were studied for failure by the computer-aided design program SolidWorks® and Hyperworks® for finite element analysis (FEA). Design improvements were performed based on these results.ResultsIn 21 cases, the failure occurred at the stem-collar junction, the point of maximum stress predicted by FEA. Stainless steel implants were prone to failure. There was one early and one late infection. Three patients died of metastatic disease. The most difficult surgical step involved the removal of the well-cemented broken stem from the intramedullary canal. Musculoskeletal Tumor Society scores varied from 27 to 29 after revision. FEA revealed stress could be reduced by filleting the stem-collar junction and by two-piece stems.ConclusionsRevisions of broken total knee megaprostheses, though technically difficult, have allowed patients reasonable function. We recommend design analysis for custom prostheses to point to areas of weakness. Breakages can be reduced by using titanium stems and filleting the junction or by having two-piece inserted stems. Incorporating these changes has reduced the failures in our experience.Level of EvidenceLevel IV, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.

Functional and oncologic outcomes after excision of the total femur in primary bone tumors: Results with a low cost total femur prosthesis

Background: The extent of tumor may necessitate resection of the complete femur rarely to achieve adequate oncologic clearance in bone sarcomas. We present our experience with reconstruction in such cases using an indigenously manufactured, low-cost, total femoral prosthesis (TFP). We assessed the complications of the procedure, the oncologic and functional outcomes, and implant survival. Materials and Methods: Eight patients (four males and four females) with a mean age of 32 years, operated between December 2003 and June 2009, had a TFP implanted. The diagnosis included osteogenic sarcoma (5), Ewings sarcoma (1), and chondrosarcoma (2). Mean followup was 33 months (9–72 months) for all and 40 months (24–72 months) in survivors. They were evaluated by Musculoskeletal Tumor Society score, implant survival as well as patient survival. Results: There was one local recurrence and five of seven patients are currently alive at the time of last followup. The Musculoskeletal Tumor Society score for patients ranged from 21 to 25 with a mean of 24 (80%). The implant survival was 88% at 5 years with only one TFP needing removal because of infection. Conclusions: A TFP in appropriately indicated patients with malignant bone tumors is oncologically safe. A locally manufactured, cost-effective implant provided consistent and predictable results after excision of the total femur with good functional outcomes.