Stewart-Treves syndrome: a case report.

We present a case of chronic lymphoedema that progressed to Stewart-Treves syndrome in a 63-year-old woman with a previous modified radical mastectomy, associated lymph node dissection, chemotherapy and radiotherapy. While producing stabilisation of most cutaneous lesions initially, chemotherapeutic treatment of the angiosarcoma did not prevent subsequent metastasis and patient death. We urge vigilance and regular follow-up appointments for patients following a mastectomy with chronic lymphoedema to facilitate prevention or early treatment of this devastating syndrome.

Extended pelvic resections for recurrent or persistent uterine and cervical malignancies: an update on out of the box surgery.

OBJECTIVE: To update our report on the outcome of patients who underwent extended pelvic resection (EPR) for recurrent or persistent uterine and cervical malignancies. METHODS: We reviewed the records of all patients who underwent EPR between 6/2000 and 07/2011. EPR was defined as an en-bloc resection of a pelvic tumor with sidewall muscle, bone, major nerve, and/or major vascular structure. Complications up to 180 days post surgery were analyzed. Survivals were estimated using the Kaplan-Meier method. RESULTS: We identified 22 patients. Median age at the time of EPR was 58 years (range, 36-74). Median tumor diameter was 5.4 cm (range, 1.5-11.2). Primary tumor sites included: uterus, 13; cervix, 7; synchronous uterus/cervix, 1; and synchronous uterus/ovary, 1. The EPR structures were: muscle, 13; nerve, 10; bone, 8; vessel, 5. Complete gross resection with microscopically negative margins (R0 resection) was achieved in 17 patients (77%). There were no perioperative mortalities. Major postoperative complications occurred in 14 patients (64%). The two most common morbidities were pelvic abscesses and peripheral neuropathies. Median follow-up time was 28 months (range, 6-99). The 5-year overall survival (OS) for the entire cohort was 34% (95% CI, 13-57). For the 17 patients who had an R0 resection, the 5-year OS was 48% (95% CI, 19-73). In patients with positive pathologic margins (n=5), the 5-year OS was 0%. CONCLUSION: EPR was associated with prolonged survival when an R0 resection was achieved. The high rate of postoperative complications remains a hallmark of these procedures and properly selected patients should be extensively counseled preoperatively.

Extended pelvic resections for recurrent uterine and cervical cancer: out-of-the-box surgery.

Patients with recurrent uterine and cervical cancer have poor prognoses. The objective of this study was to analyze the outcomes of patients with recurrent uterine and cervical cancer who had undergone attempted curative resection of pelvic bone, sidewall muscle, major blood vessels, and/or nerves. We reviewed the records of all 14 patients with recurrent uterine and cervical cancer who had extended pelvic resections at our institution between June 2000 and November 2006. Primary sites of disease were the uterus (11 patients) and cervix (3 patients). Tumor histology was as follows: adenocarcinoma, seven; squamous cell carcinoma, three; leiomyosarcoma, three; and adenosarcoma, one. Previous treatment included hysterectomy, 11; pelvic radiation, 9; chemotherapy, 9; and total pelvic exenteration, 2. Extended pelvic resections included removal of pelvic sidewall muscle, five; bone, five; common and/or external iliac vessel, five; femoral nerve, two; lumbosacral nerve root, one; and obturator nerve, one. Other procedures included total pelvic exenteration, three; posterior exenteration, two; and anterior exenteration, one. Complete resection with negative margins was obtained in 11 (78%) of 14 patients. Seven patients (50%) received high-dose rate intraoperative radiation therapy. Reconstructive procedures included continent or incontinent urinary diversion, four; femoral-femoral arterial bypass, two; myocutaneous flap, two; and urinary ileal interposition, one. Median total operating time was 628 min (range, 345-935 min) and median estimated blood loss was 900 mL (range, 300-16,000 mL). Seven patients (50%) had one or more major complication(s), including pelvic abscess, three; colonic fistula, two; massive intraoperative hemorrhage, one; postoperative bladder perforation, one; thrombosed femoral-femoral graft, one; and disruption of appendicocutaneous urinary anastomosis, one. At a median follow-up of 26 months (range, 5-84 months), ten patients (71%) are alive and four patients (29%) have died of disease at 8, 13, 33, and 42 months postoperatively.

Acetabular development after bipolar hemiarthroplasty for osteosarcoma in children.

A retrospective analysis was performed of eight patients with an open triradiate cartilage, who underwent resection for osteosarcoma and reconstruction of the proximal femur with a hemiarthroplasty, in order to identify changes of acetabular development. An analysis of the centre-edge angle, teardrop-to-medial prosthesis distance, superior joint space, teardrop-to-superior prosthesis distance, degree of lateral translation, and arthritic changes, was performed on serial radiographs. The median age at the time of the initial surgery was 11 years (5 to 14). All patients developed progressive superior and lateral migration of the prosthetic femoral head. Following hemiarthroplasty in the immature acetabulum, the normal deepening and enlargement of the acetabulum is arrested. The degree of superior and lateral migration of the prosthetic head depends on the age at diagnosis and the length of follow-up.

Vesicant characteristics of oxaliplatin following antecubital extravasation.

Oxaliplatin is a novel class of platinum chermotherapeutic agent used in refractory adenocarcinoma. It has previously been regarded as a non-vesicant, and as such was considered safe to administer through peripheral veins. This report documents severe muscle and subcutaneous reaction with a single dose of oxaliplatin at the site of extravasation in a patient aged 58 years. Conventional therapeutic modalities were employed to reduce the effect of the soft tissue infiltrate. Despite that, significant muscle necrosis and fibrosis occurred. Surgery was deferred secondary to patient choice, and eventual extensive physical therapy restored function to the elbow joint. This case shows that oxaliplatin may not be an appropriate cytotoxic agent to be administered through a peripheral line and consideration must be made for central access when this drug is used. In addition, when extravasation does occur, the current report indicates that non-surgical management can be successful.

Intralesional resection of primary and metastatic sarcoma involving the spine: outcome analysis of 59 patients.

OBJECTIVE: Surgery plays an important role in achieving local tumor control and cure for primary and metastatic tumors of the spine. As has been established with regard to sarcomas at extraspinal sites, these goals may best be achieved by en bloc resection with negative histological margins. Unfortunately, sarcomas of the spine often present with tumor patterns that are amenable only to intralesional resection, if neurological preservation is a priority. This study is a retrospective analysis of the long-term outcomes of patients who had operations for sarcomas of the spine using modern surgical approaches, intralesional resections, and spinal instrumentation. METHODS: Between 1985 and 1997, 59 patients had spinal operations for sarcoma involving the extrasacral spine. Data regarding tumor histology, grade, surgical indications, patterns of spinal tumor involvement, and neurological and functional outcomes were reviewed at presentation and at tumor recurrence. RESULTS: Thirty-five patients underwent a single operation, and 24 patients required reoperation for locally recurrent tumors. At presentation, only nine patients (15%) had tumors that were amenable to marginal or wide resections. Functional outcomes after initial spinal surgery and after operations performed at first tumor recurrence showed that 95% of patients had maintained or regained ambulation. Intradural extension of tumor was seen in 5 of 12 patients who had three or more operations for locally recurrent disease. The median survival from first spine operation was 18 months, and the median event-free interval between the first and second spine operations was 13 months. CONCLUSION: Surgery for sarcoma of the spine is useful for maintaining or improving neurological and functional outcomes, but local tumor recurrences are common. Because of the anatomy of the tumor at presentation and concern for neurological preservation, few patients are candidates for marginal or wide resections.

Functional and oncological outcome of acetabular reconstruction for the treatment of metastatic disease.

BACKGROUND: Metastatic disease of the acetabulum can be painful and disabling. Operative intervention is indicated for patients who fail to respond adequately to nonoperative treatment. We evaluated the functional and oncological outcome of acetabular reconstruction after curettage for the treatment of refractory symptomatic acetabular metastases. METHODS: Fifty-five patients with metastatic disease of the acetabulum were treated with operative acetabular reconstruction combined with a total hip replacement. The most common primary tumor was carcinoma of the breast (eighteen patients), followed by carcinoma of the kidney (seven patients) and carcinoma of the prostate (seven patients). Forty (73 percent) of the patients presented with multiple skeletal metastases, and eighteen (33 percent) had associated visceral metastases. Twenty-eight (51 percent) had severe pain requiring continuous use of narcotics, twenty-four (44 percent) had moderate pain requiring periodic use of narcotics, and the remaining three (5 percent) had mild pain requiring use of non-narcotic analgesics. Eighteen (33 percent) of the patients could not walk, twenty-three (42 percent) needed a walker or crutches, twelve (22 percent) used a single cane, and two (4 percent) walked without assistive devices. Intralesional curettage of the tumor was performed in all of the patients. Fifty-four of the hips were reconstructed with a protrusio cup and one, with a hemipelvis endoprosthesis. Large defects were reinforced with cement and pin or screw fixation (the modified Harrington technique), which allowed transmission of weight-bearing forces to the remaining intact pelvis. Thirty-six acetabular reconstructions were performed with antegrade pins or cannulated screws; fifteen, with long retrograde screws; and four, with cement. RESULTS: The median period of survival was nine months. Patients with visceral metastases had a median period of survival of three months compared with twelve months for patients without visceral metastases (p < 0.001). Patients with breast cancer presented later in the disease process (p < 0.004) and lived longer than did those with other carcinomas (p < 0.004). Forty-five patients were evaluated three months after reconstruction. Thirty-four (76 percent) of them had relief of pain as determined by decreased use of narcotics. Nine of the eighteen patients who could not walk preoperatively regained the ability to walk. Fourteen of the seventeen patients who originally were able to walk in the community retained that ability. Thirty-three patients were available for evaluation at six months. Twenty-five (76 percent) still had relief of pain, and nineteen (58 percent) were able to walk and function in the community. Overall, fourteen (25 percent) of the fifty-five patients had moderate local progression of the disease, and five of these patients had failure of the fixation. Fourteen early complications developed in twelve (22 percent) of the patients. One patient (2 percent) died perioperatively. CONCLUSIONS: Patients who have acetabular metastases that are refractory to radiation and chemotherapy have a short life expectancy. The early, gratifying results of reconstruction validate the role of operative treatment as a short-term palliative procedure. Protrusio acetabular cups presumably compensate for deficiencies of the medial wall, while cement and pin fixation can be used effectively to reconstruct large defects in the acetabular column and dome. The low rate of fixation failure supports the biomechanical principles of the reconstruction. Generally, the reconstructions are sufficiently durable to exceed the life expectancy of the patients.

Low-grade fibrosarcoma with palisaded granulomalike bodies (giant rosettes): report of a case that metastasized.

"Hyalinizing spindle cell tumor with giant rosettes" is a tumor recently described by Lane et al. and thought by them possibly to represent a form of low-grade fibromyxoid sarcoma. Proof of a metastatic potential was lacking. We report an example of this tumor on the arm of a 28-year-old woman. The ultrastructural study of the tumor confirmed the fibroblastic nature of the lesion, which subsequently metastasized to the lung. Histologically, the giant rosettes simulated palisaded granulomas. Our findings warrant classifying the tumor as a sarcoma, and we suggest the designation low-grade fibrosarcoma with palisaded granulomalike bodies (giant rosettes).

The enigma of desmoid tumors.

OBJECTIVE: To analyze patients with recurrent extremity desmoids, in whom the surgical therapeutic option was either major amputation or observation. SUMMARY BACKGROUND DATA: The biology and natural history of desmoid tumors are an enigma. These tumors invade surrounding structures and recur locally but do not metastasize. The morbidity of treating these tumors in the context of their relatively benign biology is uncertain. METHODS: Between July 1982 and June 1998, the authors treated and prospectively followed 206 patients with extremity desmoid tumors. All patients underwent standardized surgical resection, the surgical goal always being complete resection with negative margins. When tumors recurred, they were evaluated for reresection. Amputation was considered when resection was not possible because of neurovascular or major bone involvement, or in the presence of a functionless, painful extremity. RESULTS: During this period, 22 patients had disease that was not resectable without amputation. This was out of a total of 115 patients with primary disease and 91 patients with recurrent disease. All recurrences were local; in no patient did metastasis develop. In this group of 22 patients with unresectable disease, 7 underwent amputation and 15 did not. These 15 patients were followed, alive with disease, having no surgical resection. Four patients received systemic treatment with tamoxifen and nonsteroidal antiinflammatories, three received systemic cytotoxic chemotherapy, and two received both tamoxifen and chemotherapy. Six patients received no systemic treatment. The range of follow-up was 25 to 92 months. In all patients, there was no or insignificant tumor progression; in three patients who underwent observation alone, there was some regression of tumor. During follow-up, no patient has required subsequent amputation, and no patient has died from disease. CONCLUSIONS: In desmoid tumors, aggressive attempts at achieving negative resection margins may result in unnecessary morbidity. Function- and structure-preserving procedures should be the primary goal. In select patients, whose only option is amputation, it may be prudent to observe them with their limb and tumor intact.

Relationship between magnitude of resection, complication, and prosthetic survival after prosthetic knee reconstructions for distal femoral tumors.

BACKGROUND AND OBJECTIVES: Limb-sparing surgery has become the preferred surgical treatment of malignant bone tumors. The objective of this study was to evaluate factors that influence the morbidity and outcome of prosthetic knee replacement after resection of malignant tumors of the distal femur. METHODS: Eighty-two patients who had a resection of malignant tumor of the distal femur and implantation of a segmental knee prosthesis (minimum follow-up, 2 years) were retrospectively reviewed. RESULTS: Twenty-nine patients (35%) underwent 32 prosthetic revisions, 6 from perioperative wound complications, 13 from aseptic loosening, and 13 from other complications. The 3-, 5-, and 10-year Kaplan-Meier prosthetic survival rates were 82%, 71%, and 50%, respectively. On univariate analysis, patients who had more than 40% resection of the distal femur (P = 0.010) and those who had all their vasti muscles resected (P = 0.011) had significantly worse prosthetic survivals than the others. On multivariate analysis, resection of more than 40% of the distal femur was a significant negative prognostic factor for prosthetic survival (P = 0.017). Aseptic loosening was the primary cause of late prosthetic failure. Differences in the magnitude of resection influenced prosthetic survivorship more than prosthetic design. CONCLUSIONS: In the distal femoral endoprosthetic replacement, higher short- and long-term complications were found after extensive resections. Aseptic loosening was the primary cause of prosthetic failure.

A rotating-hinge knee replacement for malignant tumors of the femur and tibia.

We evaluated the 2- to 7-year results of a rotating-hinge knee replacement after excision of malignant tumors of the knee joint. There were 25 distal femoral and 7 proximal tibial replacements. The 5-year prosthetic survival for distal femoral replacements was 88%, compared with 58% for proximal tibial replacements. Seven patients underwent prosthetic exchange: 1 for aseptic loosening, 2 for wound slough and perioperative infection, and 4 for articulating component failure. One patient underwent above-knee amputation owing to skin necrosis. The median functional scores at the latest follow-up were 27 by the International Society of Limb Salvage evaluation system and 80 by the Hospital for Special Surgery Knee Score system. This implant is a promising choice for joint reconstruction after excision of tumors at the knee joint.

Pathologic femoral fracture after periosteal excision and radiation for the treatment of soft tissue sarcoma.

BACKGROUND: Surgical resection and adjuvant radiation therapy are standard therapy for soft tissue sarcomas. When the tumor approximates bone, periosteal excision may be necessary. It was hypothesized that periosteal stripping and radiation therapy would increase the rate of pathologic fracture. METHODS: The soft tissue sarcoma data base at the Memorial Sloan-Kettering Cancer Center was used to identify a consecutive series of 205 patients who were treated over a 15-year period (1982-1997). All patients had a soft tissue sarcoma of the thigh, which was managed by limb-sparing surgery and radiation therapy. Patients who had bone invasion by tumor or bone resection were not included. RESULTS: Nine patients, including eight women and one man, developed a femoral fracture in an area of previous radiation and surgery. All nine patients had undergone periosteal excision. The risk of fracture, by Kaplan-Meier survivorship, was 29% at 5 years if the resection included periosteum (P < 0.0001). Cox multiple regression analysis showed that periosteal excision was the only independent prognostic factor for the entire set of 205 patients at risk. However, for the subset of 54 patients who had periosteal stripping, two factors were also found to be prognostically important: female gender (P=0.022) and chemotherapy (P=0.020). The risk of fracture was 47% and 45%, respectively. The treatment of the fractures was difficult. There were four nonunions and three delayed unions. CONCLUSIONS: Periosteal stripping and radiation therapy places the femur at high risk of pathologic fractures, especially for female patients and patients undergoing chemotherapy. When practical, the combination of periosteal stripping and radiation should be avoided.

Treatment of femoral fractures after irradiation.

Twelve fractures of the femur occurred after irradiation in 12 patients during the past 15 years. All 12 patients had excision of a soft tissue sarcoma of the thigh. The periosteum was stripped in every case. The fractures had a deceptively innocuous radiographic appearance, which showed minimal comminution, displacement, and shortening. Treatment of the fractures was difficult and demanding. At a mean followup of 37 months, bony union was achieved in only four patients, and in all four cases union was delayed beyond 12 months. Because fracture healing typically is delayed, a loadsharing device such as an intramedullary nail may be preferable in treating these fractures. Primary or delayed bone grafting may be necessary, for no fracture united without bone graft. For elderly patients with low supracondylar fractures, primary endoprosthetic replacement may effect quicker rehabilitation. The possibility of local recurrence of tumor should be ruled out before definitive surgical treatment of the fracture. Closed, antegrade nailing in the presence of recurrent tumor may contaminate the flap for a salvage procedure such as hemipelvectomy and may compromise chances for cure.

Deep venous thrombosis after orthopedic surgery in adult cancer patients.

BACKGROUND AND OBJECTIVES: Patients with cancer and patients undergoing major orthopedic procedures are two groups at risk of deep venous thrombosis (DVT). The objective was to determine the rate of venous thromboembolic disease in patients with a malignant neoplasm and major orthopaedic surgery of the lower limb. METHODS: The study included 169 patients. All patients were given knee-high intermittent pneumatic compression devices for prophylaxis. Postoperative surveillance for thrombosis was performed on all patients with venous duplex doppler ultrasonography. RESULTS: Proximal DVT occurred in 24 of 169 patients (14.2%). One patient (0.6%) developed a symptomatic, nonfatal pulmonary embolus (PE). The development of DVT was not associated with age, sex, type of surgery, type of neoplasm, location, or pathologic fracture. The addition of anticoagulant medication such as warfarin did not significantly reduce the rate of DVT in a subset of 54 patients. In three patients, the DVT occurred only in the contralateral limb, and in four patients, there were bilateral DVTs. CONCLUSIONS: When intermittent compression boots were used for prophylaxis in conjunction with ultrasound screening, the risk of proximal DVT was substantial (14.2%), but the rate of symptomatic PE was low (0.6%).

Prognostic factors for patients with sarcomas of the pelvic bones.

BACKGROUND: Treatment of malignant tumors of the pelvis represents one of the most difficult problems in musculoskeletal oncology. However, factors that influence the local and systemic control of the disease remain ill-defined. METHODS: One hundred and two patients with localized pelvic sarcomas who underwent a surgical excision of the tumors were analyzed. The tumor diagnosis was chondrosarcoma in 49 patients, osteosarcoma in 26 patients, Ewing's sarcoma in 20 patients, and other tumors in 7 patients. The tumor was located in the ilium in 65 patients, the pubis in 21 patients, the ischium in 8 patients, and the sacrum in 8 patients. Eighty-three patients underwent a limb-sparing surgery and 19 patients underwent hemipelvectomy. Prognostic factors for local recurrence, metastasis, and survival were analyzed. RESULTS: At last follow-up, 47 patients were disease free, 7 were alive with disease, and 48 had died. The 5-year survival rate was 55% (chondrosarcoma: 65%, osteosarcoma: 47%, and Ewing's sarcoma: 52%). Inadequate surgical margin emerged as the only independent adverse prognostic factor for local recurrence. For distant metastasis, surgical stage remained as an independent prognostic factor. Patients who underwent a hemipelvectomy and those who had an inadequate surgical margin had significantly poorer survivals. CONCLUSIONS: Pelvic sarcomas remain diseases with a poor prognosis. Independent prognostic factors are few; an adequate surgical margin is critical to prevent local recurrence, and the surgical stage is related to the risk of distant metastasis. Surgical margins and hemipelvectomy were predictors of survival, but the patients who underwent hemipelvectomy also tended to have the largest, most advanced tumors. Hemipelvectomy should be considered when there is sacral involvement.

Laparoscopically assisted radical sacrococcygectomy. A new operative approach to large sacrococcygeal chordomas.

Laparoscopic rectal mobilization and hypogastric arterial isolation were combined with a posterior sacrococcygectomy for the resection of a large sacrococcygeal chordoma in two patients. The laparoscopic procedure as described was uneventful in both cases. There was no postoperative morbidity associated with the laparoscopic procedure. The combination of laparoscopic pelvic dissection and radical posterior sacrococcygectomy is safe, effective, oncologically sound, and should be considered for all patients with a large proximal sacrococcygeal chordoma.

Quality of life as defined by orthopedic oncology patients.

BACKGROUND: Quality of life (QOL), not just survival, is central to outcomes analysis in musculoskeletal oncology. However, little information exists about the patients' definition of what constitutes QOL. METHODS: Self-administered outcomes questionnaires were given to 201 surgically treated patients with lower extremity tumors. Of these patients, 192 (137 with malignant tumors, 55 with benign tumors) provided a written definition of QOL. Their responses were independently collated and matched with clinical information. RESULTS: For most patients (153, or 80%) the definition of QOL encompassed several attributes. A consistent combination of four major attributes was used in the QOL definition by 44 (32%) of the malignant cases and 19 (35%) of the benign cases. Differences in responses between men and women were idiosyncratic and more common in the benign group. Good family relations and good health were equally important to men and women. Responses varied by patient age. Older patients valued self-sufficiency and freedom from pain, whereas younger patients emphasized happiness, trust in God or church, achieving goals and being successful, and love. Those whose surgery was less extensive cited good family relations, the ability to function physically and emotionally, and having a good job or work. CONCLUSION: The variation in patients' perspectives and definitions of quality of life must be taken into account when assessing QOL in musculoskeletal oncology patients. Patients often emphasize concerns that are not adequately addressed by current outcomes-measures in orthopedics and general oncology.

The management of pathologic fractures.

This article outlines the principles of pathologic fracture management and highlights the clinical features that predict impending fracture so that the nonsurgeon can recognize how to integrate orthopedic management into the overall treatment plan for patients with metastatic disease to bone.With the possible exception of carcinoma of the breast, there is little evidence that any treatment increases survival of patients with metastatic carcinoma. However, advances in chemotherapy, radiation therapy, and surgery have undoubtedly increased the quality of life in these patients and has kept them active for longer periods of time. Judicious surgical and physiatric management is needed to optimize care.