Primary Intraosseous Malignant Peripheral Nerve Sheath Tumor of the Medial Cuneiform: A Case Report and Review of the Literature.

Peripheral nerve sheath tumors (benign and malignant) usually arise in the soft tissues and are unusual in bone. Intraosseous peripheral nerve sheath tumors are usually benign and constitute approximately 0.2% of all bone tumors. Intraosseous malignant peripheral nerve sheath tumors (MPNSTs) are uncommon and usually result from secondary invasion. Only a few cases of primary intraosseous MPNSTs have been reported in published studies, and these were localized mostly in the mandible (approximately 50%) or maxilla, spine, and, occasionally, in the appendicular skeleton. To the best of our knowledge, we report the first case of primary intraosseous MPNST involving a midtarsal bone (medial cuneiform). The patient was a 62-year-old female who presented with pain and tenderness but without swelling. Imaging revealed nonspecific findings, and the preoperative computed tomography-guided biopsy findings were consistent with MPNST. The patient was treated with neoadjuvant radiotherapy, followed by wide local excision and allograft reconstruction. At the final follow-up examination (24 months), the graft had been incorporated without evidence of local recurrence or distant disease. The patient with primary intraosseous MPNST of the medial cuneiform described in the present report presented with nonspecific clinical and radiologic findings. Thus, a high index of suspicion and histopathologic examination, including immunohistochemistry, are necessary for an accurate diagnosis.

Rupture of the ilio-psoas tendon after a total hip arthroplasty: an unusual cause of radio-lucency of the lesser trochanter simulating a malignancy.

Avulsion fracture or progressive radiolucency of lesser trochanter is considered a pathognomic finding in patients with malignancies. Although surgical release of the iliopsoas tendon may be required during a total hip arthroplasty (THA), there is no literature on spontaneous rupture of the ilio-psoas tendon after a THA causing significant functional impairment. We report here such a case, which developed progressive radiolucency of the lesser trochanter over six years after a THA, simulating a malignancy. The diagnosis was confirmed by MRI. Because of the chronic nature of the lesion, gross retraction of the tendon into the pelvis, and low demand of our patient, he was treated by physiotherapy and gait training. Injury to the ilio-psoas tendon can occur in various steps of the THA and extreme care should be taken to avoid this injury. Prevention during surgery is better, although there are no reports of repair in the THA setting. This condition should be considered in patients who present with progressive radioluceny of the lesser trochanter, especially in the setting of a hip/pelvic surgery. Awareness and earlier recognition of the signs and symptoms of this condition will aid in diagnosis and will direct appropriate management.

Squamous cell carcinoma of the foot.

BACKGROUND: Squamous cell carcinomas (SCC) of the foot are relatively common, but have been infrequently reported in the orthopaedic literature. MATERIALS AND METHODS: Twelve patients with SCC of the foot treated at a single institution from 1998 to 2005 were studied retrospectively with regard to risk factors for the disease, treatment, and functional and oncologic outcomes. The mean duration of postoperative followup was 43 (range, 24 to 105) months. RESULTS: Eight of the 12 patients had identifiable risk factors for SCC. Inadequate or inappropriate procedures had been previously performed in eight of the 12 cases, requiring more aggressive definitive treatment in at least four cases. Definitive operative treatment consisted of wide excision (4 patients), partial or complete toe amputation (4), partial foot amputation (3), and transtibial amputation (1). Two patients developed local recurrence of disease, and both ultimately required Syme amputations for local control. One patient with local recurrence died of metastatic disease and the other 11 patients are alive without evident disease. Musculoskeletal Tumor Society functional scores averaged 90 and were good or excellent in nine of the 11 surviving patients. CONCLUSION: Squamous cell carcinomas of the foot are likely underreported and frequently subject to inappropriate initial treatment. Most patients have identifiable risk factors for SCC that can aid in formulating an appropriate differential diagnosis. Despite frequent suboptimal initial treatment, most patients are candidates for complete or partial limb salvage, with generally good oncologic and functional outcomes expected.

Fungating soft-tissue sarcomas. Treatment implications and prognostic importance of malignant ulceration.

BACKGROUND: Several variables have been reported as being prognostic with regard to the outcomes of soft-tissue sarcomas. Although the tumors are subjectively ominous, no prior study has been performed to evaluate the treatment or prognosis of fungating soft-tissue sarcomas. METHODS: We performed a retrospective review of all soft-tissue sarcomas treated at our institution between 1989 and 2004 that had been followed for a minimum of two years or until the death of the patient. Our study group consisted of twenty-four patients with a primary high-grade fungating tumor, and our control group consisted of 146 consecutive patients with a primary high-grade non-fungating tumor. The study cohorts were compared with regard to disease presentation, treatment, and oncologic outcomes. RESULTS: There were no significant differences in tumor size, tumor depth, or histopathologic diagnoses between the cohorts, although the patients with a fungating tumor tended to be older (mean, sixty-five years compared with fifty-five years in the control group; p = 0.004) and have shorter postoperative follow-up (mean, thirty-eight months compared with sixty-five months in the control group; p = 0.03). The proportion of patients presenting with metastases was significantly greater in the group with a fungating tumor (33% compared with 9% in the control group; p = 0.003). Significantly more patients with a fungating tumor underwent amputation (35% compared with 12% in the control group; p = 0.01), while a greater proportion of control patients received radiation therapy (68% compared with 39% in the group with a fungating tumor; p = 0.02). There was no difference in the proportions of patients receiving chemotherapy or in the local recurrence rates between the two cohorts. The Kaplan-Meier five-year overall survival estimates were 20% in the group with a fungating tumor compared with 63% (p < 0.0001) in the control group. The Kaplan-Meier five-year disease-specific survival estimates for patients presenting with localized disease was 58% in the group with a fungating tumor and 74% in the control group (p = 0.05). Multivariate analysis demonstrated that disease stage, fungation, and a tumor size of > or = 10 cm were significant independent negative prognostic factors for disease-specific survival. CONCLUSIONS: Malignant tumor ulceration is an independent predictor of a poor prognosis for patients with a high-grade soft-tissue sarcoma. Despite the discouraging overall prognosis, aggressive multidisciplinary treatment can lead to long-term survival in an important subgroup of patients with fungating lesions.

Extraskeletal para-articular osteochondroma of the posterior knee.

Extraskeletal para-articular osteochondromas are unusual osteocartilaginous lesions that arise in the soft tissues adjacent to the joint with no bone or joint continuity. This diagnosis should be considered in patients with a well-circumscribed, extraskeletal, mineralized mass without any direct continuity with adjacent bone or joint. Although the knee is a common location for extraskeletal para-articular osteochondroma, it has not been described arising in the posterior aspect of the knee. This article presents a case of extraskeletal paraarticular osteochondroma posterior to the knee joint. Differentiation from other extraskeletal mineralized lesions, particularly extraskeletal sarcomas and synovial osteochondromatosis, is essential to avoid unnecessary aggressive surgical procedures as marginal excision is adequate for these lesions. Correlation of clinical and radiographic features with pathology is essential for diagnosis. The lesion in our patient was marginally excised, and the postoperative course was uneventful with no recurrence at 2-year follow-up.

Extended intralesional treatment versus resection of low-grade chondrosarcomas.

UNLABELLED: The need for segmental resection versus intralesional treatment of low-grade chondrosarcomas of the appendicular skeleton remains controversial. We hypothesized extended intralesional treatment would equally control malignant disease but with improved functional outcomes and decreased postoperative complications. We retrospectively reviewed 31 patients with 32 Grade I intracompartmental chondrosarcomas of the long bones of the appendicular skeleton treated with either resection (15 lesions) or extended intralesional curetting (17) at a minimum followup of 2 years (median, 55 months; range, 24-203 months). Lesions were larger and median followup was longer in the resection cohort. One local recurrence developed in each treatment cohort and neither transitioned to a higher grade of tumor. No patient had metastases develop or died of disease. The mean final Musculoskeletal Tumor Society functional scores were greater after extended intralesional versus resection treatment (29.5 versus 25.1). Complications were observed more frequently after resection and reconstruction (seven of 15) as compared with extended intralesional treatment (one of 17). Extended intralesional treatment of Grade I intracompartmental chondrosarcomas of the long bones of the appendicular skeleton therefore appears safe with improved functional scores and decreased complications versus segmental resection and reconstruction. LEVEL OF EVIDENCE: Level III, therapeutic study. See the Guidelines for Authors for a complete description of levels of evidence.

Consequences and prevention of inadvertent internal fixation of primary osseous sarcomas.

UNLABELLED: The evaluation and treatment of aggressive bone tumors continue to be diagnostic and therapeutic challenges for orthopaedic surgeons. Despite compelling data regarding the hazards of biopsy, incomplete preoperative evaluation, inappropriate biopsy techniques, and premature surgical interventions continue to compromise optimal treatment of primary bone sarcomas. We retrospectively identified eight patients who had internal fixation of a primary bone sarcoma before referral to an orthopaedic oncology service. Six of the eight patients subsequently underwent amputations and two patients underwent limb salvage for local disease control. Biopsy techniques from referring institutions were highly variable, with only two of seven rendering an accurate diagnosis. The average Musculoskeletal Tumor Society functional score was 10.6 and four of eight patients were disease-free and alive at a minimum followup of 8 months (mean, 26.9 months; range, 8-80 months). Implant violation of primary bone malignancies was associated with frequent high-level amputation for local disease control and low Musculoskeletal Tumor Society functional scores. Common errors in the initial evaluation and treatment included inadequate attention to patient history, incomplete radiographic evaluation, and improper biopsy and surgical techniques, which violated compartmental boundaries. LEVEL OF EVIDENCE: Level IV, prognostic study. See the Guidelines for Authors for a complete description of levels of evidence.

Proximal humerus reconstructions for tumors.

UNLABELLED: The optimal method for reconstructing the proximal humerus in patients with tumors is controversial. To determine functional outcomes and complication rates after different types of reconstructions, we reviewed a consecutive series of 49 patients who underwent proximal humerus resection and osteoarticular allograft (17 patients), allograft-prosthetic composite (16), or endoprosthetic (16) reconstruction. Operative indications included primary malignancies (24 patients), metastatic disease (19), and benign aggressive disease (six). Implant revision was more common after osteoarticular reconstruction (five of 17) than after allograft-prosthetic composite (one of 16) or endoprosthetic (zero of 16) reconstructions. At a minimum followup of 24 months (median, 98 months; range, 24-214 months) in surviving patients, Musculoskeletal Tumor Society functional scores averaged 79% for the allograft-prosthetic composite, 71% for the osteoarticular allograft, and 69% for the endoprosthetic reconstruction cohorts. Shoulder instability was associated with abductor mechanism compromise and was more common after endoprosthetic reconstruction. Allograft fractures occurred in 53% of patients receiving osteoarticular allografts. We recommend allograft-prosthetic composite reconstruction for younger patients with primary tumors of bone and endoprosthetic reconstruction for older patients with metastatic disease. Because of the unacceptable complication rate, we do not recommend osteoarticular allograft reconstruction for routine use in the proximal humerus. LEVEL OF EVIDENCE: Level III, therapeutic study. See the Guidelines for Authors for a complete description of levels of evidence.

Cystic presentation of Ewing's sarcoma with indolent clinico-radiologic behaviour.

Ewing's sarcoma is a high-grade malignant primary bone tumour with aggressive clinico-radiologic features. Rarely, it exhibits a benign pattern, but presentation as a solitary bone cyst in a long bone is rather unusual. The purpose of this paper is to document such a cystic presentation with relatively benign clinico-radiologic course and to review the pertinent literature. A 27-year-old male presented with a pathologic fracture through a radiologically benign appearing solitary bone cyst in the distal tibial metaphysis. The fracture healed on conservative treatment, but the patient presented again a year later with pain and difficulty with weight bearing. Curetting and bone grafting done elsewhere was suggestive of an Ewing's sarcoma, which was later confirmed by a second biopsy. He was treated by standard neoadjuvant chemotherapy followed by wide local excision and reconstruction with an intercalary allograft. At 2 years postoperative follow-up, the patient is without any evidence of local or distant recurrence. Awareness of this atypical presentation is important because it may help in an earlier diagnosis, avoid diagnostic confusion and thus may contribute to improved survival.

Soft tissue sarcomas of the foot and ankle: impact of unplanned excision, limb salvage, and multimodality therapy.

BACKGROUND: Foot and ankle sarcomas have historically been treated with amputation because of the difficulty in achieving local disease control and maintaining a functional foot. Potential opportunities for limb salvage may be further compromised by unplanned excisions. MATERIALS AND METHODS: We reviewed 52 consecutive patients with soft tissue sarcomas of the foot and ankle and analyzed the impact of planned versus unplanned initial excision, limb salvage, and multimodality therapy on treatment and outcomes. RESULTS: Unplanned excisions had been performed in 29 (55.8%) patients. Limb salvage was performed in 38 patients, with 14 requiring free soft tissue transfers. At an average followup of 99 (range, 24 to 216) months, the 5-year overall survival estimate was 76.3%. Although not statistically significant, we noted clinically relevant potential differences in local recurrence-free, disease-free, and oncologic survival between the planned and unplanned excision groups. Seven patients (13.5%) had a local recurrence, five of these following an unplanned excision. Functional scores averaged 83.2% for all patients which were similar between planned versus unplanned and amputation versus limb salvage groups. Significantly more patients with unplanned excisions required free flaps for limb salvage (p = 0.017) and received adjuvant radiotherapy (p = 0.0004). CONCLUSION: Unplanned surgery for soft tissue sarcomas of the foot and ankle often results in the need for more aggressive surgery and/or adjuvant radiotherapy and may impact oncologic outcomes, but does not necessarily portend worse functional outcomes. Multimodal therapy and judicious use of soft tissue flap reconstruction allows limb salvage in most patients with favorable outcomes.

Metastatic skeletal disease of the foot: case reports and literature review.

BACKGROUND: Metastatic disease of the skeleton occurs in at least 20% to 30% of patients with malignancy, but metastasis to the foot and hand (acrometastasis) is extremely rare (0.007% to 0.3%). Metastases to the feet are even rarer and have been reported in half to one-third the rate for hand metastases. Failure to recognize these lesions has led to delayed diagnosis and/or inappropriate treatment. The purpose of this report is to highlight the clinical and radiologic features that aid in the diagnosis and potential treatment of this condition along with a pertinent review of the literature. MATERIALS AND METHODS: In a retrospective review of 694 patients with histologically proven metastatic skeletal disease (January 1988 to January 2007), 14 cases of metastatic lesions to the foot were identified. RESULTS: The most frequent primary site was in the genito-urinary system in eight patients and the most common bones involved were the calcaneus and the talus in six patients each. All patients died after a mean survival of 14.8 (range, 1 to 54) months after diagnosis of metastases. CONCLUSION: Although metastatic disease of the foot is rare, it should be considered in the diagnosis of a painful foot, especially if suspicious radiographic changes are present in an older patient. The common primary sites are the genito-urinary, lungs, breast, and the colo-rectum. Treatment is usually palliative to reduce pain and maintain function.

Local recurrence of disease after unplanned excisions of high-grade soft tissue sarcomas.

UNLABELLED: Unplanned excisions of soft tissue sarcomas occur with alarming frequency and result in high rates of residual disease, potentially affecting patient prognosis. To determine if unplanned excisions and residual disease status at tumor bed excision increased local recurrence rates and predicted disease-specific patient survival, we retrospectively reviewed 203 consecutive patients with high-grade soft tissue sarcomas treated operatively and followed for at least 2 years (mean, 4.8 years) or until patient death. Among the 64 patients (32%) who had undergone previous unplanned excisions, six had gross residual disease and 40 of the remaining 58 (69%) had microscopic residual disease in the tumor bed. We observed subsequent local recurrence in nine of the 139 patients (6%) after planned excision compared with 22 patients (34%) after unplanned excision. More patients with unplanned excisions who underwent limb salvage procedures required flap coverage and/or skin grafting with their definitive resection (30% versus 5%). In the unplanned excision cohort, residual disease status at tumor bed excision predicted increased rates of local recurrence and decreased disease-specific survival. Unplanned excisions of high-grade soft tissue sarcomas resulted in increased rates of local recurrence but not disease-specific survival. Residual disease at reexcision predicted the likelihood of local recurrence. LEVEL OF EVIDENCE: Level II, prognostic study. See the Guidelines for Authors for a complete description of levels of evidence.

Benign multicystic peritoneal mesothelioma in a cesarean-section scar presenting as a fungating mass.

We report a case of a benign multicystic mesothelioma, which presented as a fungating mass through the anterior abdominal wall and arose in a cesarean-section scar without direct peritoneal involvement. A wide local excision was done and the diagnosis was confirmed by histopathology and immunohistochemistry. The postoperative course was uneventful and the patient is asymptomatic at 3 years' follow-up. Although a history of previous abdominal surgery has been reported in a patient with benign multicystic mesothelioma, to the best of our knowledge, there is no report of a benign multicystic mesothelioma arising in a cesarean-section scar or presentation as a fungating skin mass. This unusual presentation may point to a traumatic or inflammatory etiology, although seeding of the wound during the previous surgeries is a more likely postulate. A pertinent review of the literature on benign multicystic mesothelioma is also presented.

Pigmented villonodular bursitis/diffuse giant cell tumor of the pes anserine bursa: a report of two cases and review of literature.

Pigmented villonodular synovitis (PVNS) is a benign but potentially aggressive lesion, characterized by synovial villonodular proliferation with hemosiderin pigmentation and stromal infiltration of histiocytes and giant cells. This consists of a common family of lesions, including localized and diffuse forms of pigmented villonodular synovitis, giant cell tumor of the tendon sheath (nodular tenosynovitis) and the very rare cases of extra-articular pigmented villonodular synovitis arising from the bursa (pigmented villonodular bursitis or diffuse giant cell tumor of the tendon sheath). The purpose of this paper is to present two rare cases of pigmented villonodular bursitis arising from the pes anserinus bursa. The various differentials along with a review of literature of similar lesions are also being discussed. However, as with other lesions, clinicoradiographic features along with close histological correlation is essential for diagnosis.