Unplanned Excision of Soft Tissue Sarcomas of the Extremities in a Low-to-Middle-Income Country.

BACKGROUND: Outcomes of unplanned excisions of extremity soft tissue sarcomas (STSE) range from poor to even superior compared with planned excisions in developed countries. However, little is known regarding outcomes in low-to-middle-income countries. This study aimed to determine whether definitively treated STSE patients with a previous unplanned excision have poorer oncologic outcomes compared with those with planned excisions. PATIENTS AND METHODS: Using the database of a single sarcoma practice, we reviewed 148 patients with STSE managed with definitive surgery-78 with previous unplanned excisions (UE) and 70 with planned excisions (PE). RESULTS: Median follow-up was 4.4 years. UE patients had more surgeries overall and plastic reconstructions (P < 0.001). On multivariate analysis, overall survival (OS), local recurrence-free survival (LRFS), and distant metastasis-free survival (DMFS) were not worse among UE patients compared with PE patients. Negative predictors for LRFS were high tumor grade (P = 0.031) and an R1 surgical margin (P < 0.001). High grade (P <0.001), local recurrence (P = 0.001), and planned excisions (P = 0.009) predicted poorer DMFS, while age over 65 years (P = 0.011) and distant metastasis predicted poorer OS (P < 0.001). CONCLUSIONS: We recommend systematic re-excision for patients with unplanned excisions. Our study shows that STSE patients with UE, when subjected to re-excision with appropriate surgical margins, can achieve oncologic results similar to those for PE patients. However, there is an associated increased number of surgeries and plastic reconstruction for UE patients. This underscores the need, especially in a resource-limited setting, for education and collaborative policies to raise awareness about STSE among patients and physicians.

A Comparison of Vascularized Free Fibular Flaps and Nonvascularized Fibular Grafts for Reconstruction of Long Bone Defects after Tumor Resection.

Background The use of the fibula autograft has been a reliable method in the reconstruction of long bone defects after tumor resection. The objective of this study was to report the outcomes fibular grafting in terms of graft union, functional score, complications, and oncologic outcome. Methods A retrospective review of patients with fibular grafting after tumor resection was done from January 1, 1993 to December 31, 2013. The primary outcome was graft union and the revised musculoskeletal tumor society scoring system (MSTS score). The secondary outcomes were oncologic outcomes, complications, and the factors associated with graft union. Results A total of 52 patients with a mean follow-up of 42 months (SD, 33; range, 12-132 months) were included. The overall union for all fibular grafts was 37 of 52. The use of vascularized free fibula flaps had a higher union rate compared with nonvascularized fibula grafts. The use of a vascularized free fibular flap was four times likely to unite (95% CI 1.1-12.8, p = 0.039) compared with nonvascularized fibular grafts. The mean MSTS score in 36 patients was 82.5 (SD, 12.9) at 35 months from surgery (SD, 30). A total of 39 complications were present in 29 patients. On final follow-up, 45 of 52 patients were alive, six patients had died of disease and one died of other causes. Conclusion A higher union rate was achieved using vascularized free fibular flaps compared with nonvascularized fibular grafts for long bone reconstruction after tumor resection. There was no difference in terms of MSTS score between the two types of grafts.

The relationship of the posterior interosseous nerve to the supinator muscle in the dorsal approach to the proximal radius: A descriptive and quantitative anatomic study of Filipino cadavers

@#<p style="text-align: justify;"><strong>BACKGROUND AND OBJECTIVE:</strong>The posterior interosseous nerve (PIN) is vulnerable to injury in the dorsal approach to the proximal radius. The goal of this study is to describe the quantitative relationship of the PIN to the supinator muscle in the context of anatomic landmarks. Knowledge of superficial landmarks related to the PIN would hopefully minimize iatrogenic injury to the posterior interosseous nerve.<br /><strong>METHODS:</strong> 12 cadavers (22 forearms) were dissected and analyzed. The length of the supinator muscle was determined. The oblique distances of the PIN entry and exit points to the proximal and distal borders of the supinator muscle as well as their perpendicular distances to the lateral epicondyle-Lister's tubercle (LE-LT) reference line were measured and recorded. The number of PIN branches inside the supinator substance was recorded. Mean and median values were determined and subjected to statistical analysis.<br /><strong>RESULTS:</strong> Mean supinator length was 5 centimeters. Ninety-one percent of the cadaveric forearms had PIN branches inside the supinator muscle substance. Twelve of the 22 forearms (55%) had 2 branches. The mean oblique distances of the PIN from the lateral epicondyle to the entry and exit points in the proximal and distal borders of the supinator muscle was 3.52 and 7.31 centimeters, respectively. The mean perpendicular distances of the PIN from LE-LT reference line to the entry and exit points in the proximal and distal borders of the supinator muscle was 1.13 and 1.26 centimeters, respectively. An imaginary danger-zone 4 centimeters wide overlying the LE-LT reference line depicts the possible area where the PIN and its branches may most likely be located.<br /><strong>CONCLUSION:</strong> The dorsal approach to the proximal radius may allow a safe exposure without causing iatrogenic injury to the posterior interosseous nerve through the use of superficial anatomic landmarks and reference lines in combination with mean measurements from our study.</p>

The Surgical Treatment and Outcome of Nonmetastatic Extremity Osteosarcoma with Pathological Fractures.

BACKGROUND: Recent studies have suggested that the presence of a pathological fracture does not impact on oncologic outcomes and the feasibility of limb salvage surgery (LSS) in appropriately selected patients when combined with neoadjuvant chemotherapy. These have largely been single institutional studies with limited numbers. The Eastern Asian Musculoskeletal Oncology Group reviewed the data from three large volume Asian orthopedic oncology centers to determine whether the presence of a pathologic fracture affected outcomes in osteosarcoma patients. METHODS: A retrospective review of the data was conducted. Ninety-five cases of nonmetastatic extremity osteosarcoma with a pathological fracture and 887 cases without fracture treated during the same period were compared. RESULTS: In the fracture group, the LSS rate was 62.1%, and the rate of amputation was 37.9%. In the nonfracture group, the LSS rate was 74.7%, and the amputation was 25.3%. In patients with a pathologic fracture, the rate of local recurrence for LSS and amputation groups was 8.5% and 2.8%, respectively. In this group, the 5-year survival in the LSS group was 66% as against. 46.8% in the amputation group. CONCLUSIONS: Our study suggests that surgically treated patients with pathologic fractures in osteosarcoma have adequate local control and do not have a poorer outcome compared to patients without a fracture. Though osteosarcoma with a pathologic fracture is not a contraindication for limb salvage, appropriate case selection is important when deciding local control options to ensure adequate oncologic clearance.

Synovial haemangioma of the knee: an under recognised condition.

BACKGROUND: Synovial haemangioma is a rare type of tumour for which only around 200 cases have been reported worldwide. It usually occurs in the female population during the second decade of life and most commonly affects the knee joint. Patients can complain of pain, recurrent knee swelling and limitation of motion. Since these lesions are uncommon and radilogical findings are nonspecific, physician awareness is low and diagnosis is often delayed, leading in turn to treatment delays and irreversible complications of the affected joint. MATERIALS AND METHODS: We report four cases of synovial haemangioma of the knee seen over a period of 20 years (1993-2013). Age at presentation ranged from six to 43 years (mean of 22.7 years) with an equal male-to-female ratio. Average duration of symptoms prior to treatment was three years--patients were often misdiagnosed and appropriate treatment was subsequently delayed. Radiographs showed moderate to severe degenerative changes. Magnetic resonance (MR) imaging revealed poorly defined intra-articular contrast-enhancing lesions, all of which were of the localised type. Three patients underwent open synovectomy and en bloc excision of the lesion; the fourth deferred surgery but continues to be monitored. Follow-up ranged from one to 11 years; all four patients are doing well, with no signs of symptom recurrence or progression. CONCLUSIONS: Synovial haemangioma is a rare but treatable condition. It should remain a differential for any patient with recurrent knee-joint symptoms.

Osteosarcoma in Asian Populations Over the Age of 40 Years: A Multicenter Study.

BACKGROUND: Elderly patients with osteosarcoma (OSA) are no longer uncommon; however, many questions remain regarding this population. We investigated the clinicopathological characteristics and prognostic factors of OSA in an Asian population over the age of 40 years. METHODS: This was a multi-national, multi-institutional study by the Eastern Asian Musculoskeletal Oncology Group (EAMOG). RESULTS: A total of 232 patients were enrolled (116 males and 116 females), with a median age of 50 years at diagnosis; 25 (10.8 %) patients exhibited initial metastasis. Median follow-up was 52 months for survivors. We observed 102 osteolytic and mixed radiographic findings for 173 lesions. Histological subtypes other than osteoblastic type were frequent. Radiation-associated OSA was seen in seven patients, with a 5-year overall survival (OS) of 16.7 %. No Paget's OSA was observed. High-grade spinopelvic OSA was seen in 29 (12.5 %) patients. The 5-year OS was 59.4 % in patients without initial metastasis and 45.2 % in patients with spinopelvic OSA. While surgery and initial metastasis were common prognostic factors for OS, chemotherapy was not. Histologic response to neoadjuvant chemotherapy was poor in 61 of 83 patients. CONCLUSION: This study revealed distinct clinicopathological features of OSA patients over 40 years of age compared with younger patients, such as the high incidence of axial tumors, common osteolytic and mixed radiographic findings, the high frequency of unusual histologic subtypes, and poor prognosis. Contrary to Western elderly patients with OSA, there was no Paget's OSA in this study, which may result in a lower incidence of secondary OSA. Prognostic factor analyses demonstrated chemotherapy did not influence OS.

Is curettage and high-speed burring sufficient treatment for aneurysmal bone cysts?

BACKGROUND: To decrease the recurrence rate after intralesional curettage for aneurysmal bone cysts, different adjuvant treatments have been recommended. Liquid nitrogen spray and argon beam coagulation have provided the lowest recurrence rates, but unlike the high-speed burr, these adjuvants are not always available in operating rooms. QUESTIONS/PURPOSES: We asked: (1) Is high-speed burring alone sufficient as an adjuvant to curettage with respect to recurrence rates? (2) What is the complication rate from this technique? (3) What are the risk factors for local recurrence? METHODS: A retrospective review of the database of the University Musculoskeletal Tumor Unit and the private files of the senior author (EHW) for a period of 19 years (1993-2011) was performed to identify all patients histologically diagnosed with primary aneurysmal bone cyst. During that period, patients with aneurysmal bone cysts were treated with intralesional curettage, burring, and bone grafting if the lesions showed an adequate cortical wall or a wall with thinned out portions which could be reconstructed with bone grafting. Based on those indications, we treated 54 patients for this condition. Of those, 18 were treated using approaches other than burring because they did not meet the defined indications, and an additional five patients were lost to followup before 2 years, leaving 31 patients for analysis, all of whom were followed up for at least 2 years (mean, 7 years; range, 2-18 years). RESULTS: Of these 31 patients, one had a recurrence (3.2%). Complications using this approach occurred in three patients (9.7%), and included growth plate deformity (1) and genu varus (2) secondary to collapse of the reconstructed condyle. With only one recurrence, we cannot answer what the risk factors might be for recurrence; however, the one patient with recurrence presented with a large lesion and a pathologic fracture. CONCLUSIONS: Curettage, burring, and bone grafting compare favorably in the literature with other approaches for aneurysmal bone cysts, such as cryotherapy and argon-beam coagulation. We conclude that high-speed burring alone as an adjuvant to intralesional curettage is a reasonable approach to achieving a low recurrence rate for aneurysmal bone cysts. LEVEL OF EVIDENCE: Level IV, therapeutic study. See the Instructions for Authors for a complete description of levels of evidence.

The prognostic value of pathologic fractures in patients with high-grade classic osteosarcoma

Pathologic fracture in osteosarcoma has traditionally been associated with a poor prognosis and is an immediate indication for amputation of the affected extremity. This concept has been questioned and remains an area of debate, especially because advances in osteosarcoma treatment have today resulted in better survival and limb salvage rates. It is the objectives of this paper to analyze the survival and limb salvage rate of osteosarcoma patients presenting with pathologic fractures and compare these with the population of osteosarcoma patients without pathologic fractures. Over a 15-year period (1993-2008), these were 84 patients presenting with non-metastatic high-grade classic osteosarcoma of the extremities (Enneking Stage IIB) who received complete treatment from the University of the Philippines-Musculoskeletal Tumor (UP-MuST) Unit and who had a follow up of at least 2 years. Ten of these 84 patients had pathologic fractures prior to surgery. Their overall survival was 50%, similar to the 50.38% overall survival of the entire group of 84 osteosarcoma patients. Limb salvage rate was 60%, similar to the 59% rate of patients without pathologic fractures. This study shows that contrary to traditional teaching, overall survival for this subset of osteosarcoma patients with pathologic fractures is acceptable at 50% and is equal to that of the entire group of osteosarcoma patients. At the same time, successful limb salvage surgery is possible despite the presence of a pathologic fracture, especially in patients with good response to chemotherapy.

The prognostic factors of recurrent GCT: a cooperative study by the Eastern Asian Musculoskeletal Oncology Group.

BACKGROUND: Giant-cell tumor (GCT) of bone is a common primary benign tumor with high local recurrence and potential distant metastasis or malignant transformation. We have investigated the clinical behavior of recurrent GCT of bone in the extremities. METHODS: We retrospectively reviewed 110 patients with recurrent GCTs of bone in the extremities treated by the Eastern Asian Musculoskeletal Oncology Group. The factors that affected the number of recurrences and distant metastasis were analyzed. RESULTS: The median interval between initial surgery and the first recurrence of GCT was 16 months (2-180 months). All patients received additional surgery for first recurrence. Twenty-five patients had a second recurrence and 6 patients had a third recurrence. The mean interval between the initial surgery and the first recurrence correlated with the eventual number of recurrences-14.1 months for the repeated recurrence groups (two and three recurrences) and 28.3 months for the single recurrence group (p = 0.016). Campanacci grade did not correlate with repeated recurrence (p = 0. 446). The venue of the initial surgery did not correlate with recurrence but did affect preservation of the adjacent joint (chi-squared test; p = 0.046). Campanacci grade II and III also correlated with sacrifice of the adjacent joint (p = 0.020). The incidence of lung metastasis and malignant transformation were 7.5% (8 out of 107 patients) and 2.7% (3 out of 110 patients), respectively. Repeat recurrence was associated with lung metastasis (p = 0.018). CONCLUSIONS: Early local recurrence of GCT is a risk factor for repeat recurrence. Repeat recurrence also correlates with lung metastasis. Recurettage with meticulous adjuvant treatment to completely preclude recurrent lesions is a reasonable method for preserving the adjacent joint. However, a continuous careful follow-up is mandatory.

Adductor myocutaneous flap coverage for hip and pelvic disarticulations of sarcomas with buttock contamination.

BACKGROUND: Hip disarticulation and hemipelvectomy are alternatives to limb-salvage procedures for patients with extensive tumors of the upper thigh and buttocks. In cases when neither the conventional posterior gluteus maximus flap nor the anterior quadriceps flap can be used because of the location of the tumor, a medial adductor myocutaneous flap may be an alternative. DESCRIPTION OF TECHNIQUE: The flap is outlined over the anteromedial thigh. The distal extent is at the level of the adductor hiatus. The common femoral vessels and nerve are traced, preserved, and protected. The adductor muscles then are divided above their insertions on the femur and preserved with the flap. En bloc removal of the tumor is performed by either hip disarticulation or hemipelvectomy. The long adductor myocutaneous flap is brought up laterally and proximally to close the wound. PATIENTS AND METHODS: We reviewed four patients who underwent a medial adductor myocutaneous flap after either hip disarticulation or hemipelvectomy. The medical records and radiographs were analyzed. These patients were followed up for at least a year or until death. RESULTS: Wide surgical margins were achieved in all four patients and the flap remained viable, with no skin necrosis or flap breakdown. The patients were able to sit on the flap, and one patient was able to wear a prosthesis. CONCLUSIONS: In patients undergoing hip disarticulation or hemipelvectomy where tumor infiltration or inadvertent contamination by previous surgery will not allow the traditional posterior gluteus maximus or anterior quadriceps flap, this unconventional medial adductor myocutaneous flap is a feasible, technically simple option. LEVEL OF EVIDENCE: Level IV therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.

Osteosarcoma in the preadolescent Filipino patient

Objective. Classic high-grade osteosarcoma is uncommon in preadolescents (less than or equal to 10 years of age). The possibilities of clinicopathologic differences from the typical adolescent osteosarcoma patient have been raised. We sought to compare the presentation, treatment and survival of this subgroup of patients with published rates in order to determine if there is a need to use a treatment regimen different from that for regular adolescent osteosarcoma patients. Methods. Records of the University of the Philippines-Musculoskeletal Tumor Unit (UP-MuST) over a 15-year period (1993-2008) were reviewed and data collected on patients 10 years and younger with biopsy-proven classic high-grade intramedullary osteosarcoma who underwent complete treatment by the Unit. Demographics and survival rates were then compared with published rates for preadolescent and regular adolescent osteosarcoma cases. Results. There were fourteen patients; (6M:8F; age: 4-10 years). The most common presentation was a painful mass in the distal femur (8); the tumors most commonly had osteoblastic histology (12). Treatment consisted of neoadjuvant chemotherapy, wide surgical excision through ablation (9) or limb-saving surgery (5), and postoperative chemotherapy. There was a good histologic response (over 90% tumor necrosis) in four patients. Seven patients are ANED (alive no evidence of disease) 25 to 186 months after diagnosis. Five-year survival estimate is 52%, compared to a dismal 5 to 10% 15 years ago. Conclusion. Clinicopathologic presentation, clinical course, and overall survival in this subgroup of patients are comparable with published results for both preadolescent and adolescent osteosarcoma patients. There is no need to alter the present treatment regimen for this group of young patients.

Case report: Primary squamous cell carcinoma of a tarsal bone.

We describe a rare case of primary squamous cell carcinoma of the cuneiform bone of the foot in a 57-year-old man. In the appendicular skeleton, epithelial carcinomas of bone are usually metastatic deposits, primary squamous cell carcinomas of bone being found more frequently in the skull. A review of the English literature revealed only two other reported cases of primary squamous cell carcinoma outside the skull--one in the ilium and one in the tibia. In our patient, extensive metastatic workup and monitoring during more than 2 years showed no primary focus, supporting the rare presentation of a primary squamous cell carcinoma of bone in the appendicular skeleton.

Cancer and the Philippine Cancer Control Program.

Cancer is the third leading cause of morbidity and mortality in the Philippines. Leading cancer sites/types are lung, breast, cervix, liver, colon and rectum, prostate, stomach, oral cavity, ovary and leukemia. There is at present a low cancer prevention consciousness and most cancer patients seek consultation only at advanced stages. Cancer survival rates are relatively low. The Philippine Cancer Control Program, begun in 1988, is an integrated approach utilizing primary, secondary and tertiary prevention in different regions of the country at both hospital and community levels. Six lead cancers (lung, breast, liver, cervix, oral cavity, colon and rectum) are discussed. Features peculiar to the Philippines are described; and their causation and prevention are discussed. A recent assessment revealed shortcomings in the Cancer Control Program and urgent recommendations were made to reverse the anticipated 'cancer epidemic'. There is also today in place a Community-based Cancer Care Network which seeks to develop a network of self-sufficient communities sharing responsibility for cancer care and control in the country.