Donald A. Hackbarth

Soft Tissue Tumors of the Foot and Ankle

Seven patients with malignant soft tissue tumors of the foot and ankle were retrospectively reviewed. Emphasis was placed on the value of various diagnostic modalities used preoperatively for staging and the adequacy of treatment rendered using current tumor surgery principles. The malignant tumors included synovial cell sarcoma (four), clear cell sarcoma (one), fibrosarcoma (one), and melanoma (one). Surgical management of the malignant lesions included intracapsular excision, marginal excision, wide excision, and radical amputation.

Extensile triradiate approach for complex acetabular reconstruction in total hip arthroplasty

The triradiate approach as described by Mears has been used for open reduction of complex dual-column acetabular fractures. Mears extended the anterior limb of this incision to the symphysis pubis by the ilioinguinal approach. From a consecutive series of 43 total hip revisions, this approach was used successfully in seven cases in which extensile exposure was necessary to revise complicated acetabular reconstructions for arthroplasty. The approach was used also to perform complex acetabular reconstruction after en bloc tumor resection. Three patients had severe intrapelvic component protrusio and anterior column deficiency where close proximity of the prosthesis and femoral neurovascular bundle were detected before surgery. Massive allograft and pelvic reconstructions were used to span large anterior column defects and to stabilize pelvic dehiscence. Complications included one hip dislocation and one posterior flap tip necrosis. Follow-up ranged from six to 34 months. No graft failure has been noted.

High-resolution bone scintigraphy of the adult wrist

Bony anatomic landmarks of the wrist (e.g., pisiform, hook of hamate, radioulnar joint, and styloid processes of the radius and ulna) were routinely identified in 28 adult patients examined for wrist pain. With the wrists prone and immobilized, bone scintigrams were obtained for 500,000 counts with an asymmetric (133 to 161 keV) Tc-99m energy window and either a converging (best choice) or straight-bore, high-resolution collimator. High-resolution scintigraphy precisely localized degenerative joint disease (nine patients), scaphoid fractures (five), pisiform fracture (one), lunate avascular necrosis (one), radioulnar arthritis (one), septic or inflammatory arthritis (six), ulnocarpal impingement (two), and reflex sympathetic dystrophy syndrome (two). Images obtained palm down with the wrist in ulnar deviation helped identify increased uptake within the scaphoid. Fracture and significant bone or joint disease were excluded in one patient.

Primary skeletal Ewing's sarcoma in down syndrome

Primary skeletal Ewings sarcoma that occurred in two teenage patients with Down syndrome are reported. Cytogenetic analysis of one of these tumors showed the 11;22 translocation characteristic of Ewings sarcoma as well as other complex karyotypic changes. The possible role of constitutional trisomy 21 in development of these sarcomas is discussed.

Multimodality management of metastatic patients with soft tissue sarcomas may prolong survival.

Objectives:Patients who develop metastatic disease from soft tissue sarcoma have a poor prognosis. The purpose of this study was to identify metastatic survival rates and identify prognostic variables that predict for these outcomes. Methods:Between 2000 and 2010, 182 patients with stage I to IV primary soft tissue sarcomas of the extremity and trunk were treated with multimodality treatment. Fifty-five patients developed or presented with metastasis. We retrospectively analyzed prognostic factors for metastatic survival. Metastatic survival between groups was compared with the log-rank test. Survival curves were estimated by Kaplan-Meier plots. Multivariate analysis was performed using the Cox proportional hazards model. Results:Median follow-up was 3.1 years. Median metastatic survival was 24.2 months. Median metastatic survival in those undergoing multimodality therapies was 40 versus 22 months in those receiving single modality treatments. In single predictor Cox models, age, stage, number of lung metastases, location of metastases, and primary disease were significant for metastatic survival. On multivariate analysis, number of pulmonary metastases, histology, stage, and location of primary disease predicted for metastatic survival. Patients who had pulmonary-only disease had improved metastatic survival versus those that had extrapulmonary with or without pulmonary metastatic disease (38 vs. 15 mo). Patients who had ⩽5 pulmonary metastasis had improved metastatic survival versus those that had >5 pulmonary lesions (55 vs. 22 mo). Conclusions:This analysis shows that >5 pulmonary metastasis, malignant fibrous histiocytoma histology, stage III disease, and proximal lower extremity sarcomas are associated with decreased metastatic survival. Moreover, aggressive multimodality management of metastatic disease may prolong metastatic survival.

Prognostic variables in patients with primary soft tissue sarcoma of the extremity and trunk treated with neoadjuvant radiotherapy or neoadjuvant sequential chemoradiotherapy

BackgroundNeoadjuvant radiotherapy (NRT) is an effective strategy to treat soft tissue sarcomas (STS). However, the role of neoadjuvant chemoradiotherapy (NCRT) remains to be determined.MethodsFrom May 1999 to July 2010, 112 patients with localized STS of the extremity and trunk who were treated with NRT or NCRT followed by surgery were retrospectively reviewed. Clinical outcomes including overall survival (OS), disease-free survival (DFS), and distant metastasis free survival (DMFS) were calculated using Kaplan-Meier survival analyses. Prognostic variables were determined by univariate (UVA) and multivariate analyses (MVA).ResultsMedian follow-up was 37 months. Median RT dose was 50 Gy. Forty-nine patients received NCRT. Overall limb-preservation rate was 99% and local control was 97%. The estimated 3-year OS, DFS, and DMFS were 86%, 68%, and 72%, respectively. Age was the only variable to predict for OS, DFS and DMFS on UVA. Age ≥ 70 predicted for poor OS, stage III disease predicted for poor DFS and DMFS, and the addition of chemotherapy predicted for improved DMFS on MVA.ConclusionsExcellent rates of local control and limb-preservation were observed in patients with primary STS treated with neoadjuvant therapy followed by surgery. Neoadjuvant sequential chemotherapy followed by radiotherapy may be considered for young patients with stage III STS.

Peroneal nerve compression by lateral gastrocnemius flap

Lateral gastrocnemius muscle flaps are reliable flaps routinely used to cover defects in the lower thigh, the knee, and the upper third of the tibia. Peroneal neuropathy following lateral gastrocnemius flap has been described previously and mostly attributed to intraoperative nerve injury. However, compression of the nerve by the flap itself has not been reported. A 56-year-old female developed right common peroneal nerve palsy 10 months after a lateral gastrocnemius flap rotation for knee prosthesis coverage. Surgical exploration found a common peroneal nerve neuroma under a compressive band formed by the lateral fascial edge of the rotated gastrocnemius muscle. The motor nerve of the flap was not denervated. When stimulated, muscle contracted and compressed the common peroneal nerve underneath. Excision of the lateral fascia and selective motor denervation were performed. Postoperatively, the patients symptoms improved. To prevent compressive common peroneal neuropathy from lateral gastrocnemius rotational flaps, we recommend fascial excision from the lateral aspect of the muscle and selective motor denervation.

The effect of radiation therapy on von Willebrand factor in patients with angiosarcoma

Radiation damages endothelial cells (EC) of malignant and normal tissues. Shortly after radiation injury platelet thrombi cause small vessel obstruction. Radiation doses of 20 Gy or higher release von Willebrand factor (VWF) from cultured human umbilical vein EC. Angiosarcoma (AS) is a vascular tumor with abnormal appearing EC which have detectable cytoplasmic VWF. We examined the effect of radiation therapy on VWF in three patients with AS. Each patients malignant tissue was demonstrated to have cytoplasmic VWF present. Venous blood samples were drawn immediately before and at weekly intervals during treatment. Patient 1 received 80 Gy over 6 weeks and had no alterations in the VWF. Patient 2 received 64 Gy over 6 weeks and was noted to have loss of the high molecular weight multimers of VWF without loss of FVIII:C, VW: RIST, or von Willebrand antigen activity (VW:Ag). Patient 3 was treated with 64.4 Gy over 9 weeks and had a disproportionate increase of the VW: Ag and VW: RIST to FVIII: C. This returned to normal after completion of therapy. These changes were minimal and might be explained by either limited local release or no increased release of VWF from the irradiated tissue. The minimally abnormal multimeric pattern in patient 2 may be due to the release of an abnormal VWF in normal amounts or small amounts of proteolysis. The alteration in the VW: RIST and VW: Ag to FVIII: C ratio can be explained by activated coagulation secondary to radiation injury.

Tumor Increase on MRI after Neoadjuvant Treatment is Associated withGreater Pathologic Necrosis and Poor Survival in Patients with Soft TissueSarcoma

Purpose: MRI is often used to evaluate sarcoma response to neoadjuvant treatment, however its role to predict for pathologic response and survival is unclear. Methods and materials: From 2003-2010, 116 patients with STS were treated with neoadjuvant therapy (NAT). 62 patients who had an MRI before and after radiotherapy were analyzed. Radiographic change was correlated with survival and necrosis and fibrosis on pathology. ROC curve analysis was used to assess change in volume that best predicted for pathological necrosis. Results: Median follow-up was 33 months. There was median tumor volume decrease of 15.08 cm3 after treatment. Increase in tumor size and volume was associated with greater necrosis (p<0.03, p=0.001, respectively) and less fibrosis (p<0.001) on pathology. High-grade tumors had more necrosis (p<0.001) and comprised the majority of patients with tumor increases following NAT (88%). Tumor increase of at least 66% predicted for ≥ 70% necrosis with 94% specificity. The 3-year OS was 65% vs. 93% in patients with a decrease in size and volume (p=0.004). In tumors with ≥ 70% necrosis, the 3-year OS was 38% vs. 91% if necrosis was <70% (p<0.001). Conclusions: MR-based tumor increase following NAT was associated with greater % necrosis and less fibrosis on pathology. This tumor increase was more likely high-grade and associated with worse survival.

Myoepithelioma of soft tissue: A cytological-pathological correlation with literature review

Myoepitheliomas of soft tissue are rare tumors with variable morphologic, immunohistochemical and molecular profiles and therefore are diagnostically challenging for pathologists. We report a case in a 60-year-old male with a painless slowly growing 3cm mass on left medial forefoot. Core biopsy of the mass showed a neoplastic proliferation of plasmacytoid tumor cells, consistent with myoepithelioma of soft tissue. Immunohistochemical stains demonstrated positivity of the tumor cells for cytokeratin AE1/AE3, CK18, S-100 protein and myosin heavy chain (SMMS-1), supporting the diagnosis. Fine-needle aspiration was performed intraoperatively before the resection of the mass. Air-dried Diff-Quik stained cytology slides showed singly scattered and loosely cohesive clusters of plasmacytoid and spindle cells with dense basophilic cytoplasm, distinct cytoplasmic borders, and round to oval mildly pleomorphic nuclei with smooth nuclear membrane. Scattered naked nuclei, binucleated tumor cells, as well as tumor cells with wispy elongated cytoplasm were also seen. Occasional clusters of cells were intimately associated with metachromatic fibrillary stromal material. Histologic examination of the resected tumor confirmed the diagnosis of myoepithelioma. Molecular studies showed absence of EWSR1 rearrangement. Cytological differential diagnosis, clinical, histological, immunohistochemical, and molecular features of soft tissue myoepitheliomas are discussed in this study.