Pseudomyogenic hemangioendothelioma with bone and soft tissue involvement with favorable response to pamidronate: a case report and systematic review of the literature.

Pseudomyogenic hemangioendothelioma (PMH) can be a challenge for diagnosis and might be confused with other tumors, such as epithelioid sarcoma. Here we present a case and a systematic review of the literature to identify and discuss PMH treatment in primary bone involvement. A 25-year-old woman was referred for bone pain (10/10) in the left lower limb. Magnetic resonance imaging (MRI) showed multiple bone lesions (left femur, tibia, patella, ankle, and foot) with well-defined borders without signs of local aggressiveness. Positron Emission Tomography-Computed Tomography (PET-CT) showed multiple metabolic musculoskeletal lesions in the left lower limb. A CT scan-guided biopsy was performed. Histological and immunohistochemical findings confirmed the diagnosis of PMH. After treatment with intravenous pamidronate (90 mg/monthly), the patient had clinical improvement, mild pain 2/10 without the use of non-steroidal anti-inflammatory drugs or opiates. Follow-up was assessed by MRI and PET-CT. PET-CT showed metabolic resolution of most of the bone and muscular lesions and a significant improvement of the femoral lesion. MRI showed that the lesions in the left femur, tibia, and foot had a marked decrease in size without intravenous post-contrast enhancement and smaller lesions had disappeared. After a 3-year follow-up, PET-CT showed no metabolically active images. Literature review identified 31 records including 58 clinical cases of PMH with primary bone involvement and treatment description for qualitative analysis. Most lesions (69%) were treated by local excision or curettage. In addition, amputations were performed in a significant percentage of cases (20.7%). In the last years, mTOR inhibitors (n = 7) and anti-resorptive treatments (n = 4) were considered as alternative treatment options, especially in multifocal lesions.

Soft Tissue Sarcomas: Is Pre-operative Radiotherapy Associated With More Acute Wound Complications?

INTRODUCTION: Increased wound complication rates are attributed to the use of pre-operative radiotherapy. The purpose of this study is to evaluate the incidence of complications with or without pre-operative radiotherapy in our institution. METHODS: We retrospectively evaluated 48 adult patients with high-grade extremity soft tissue sarcoma. Twenty-two patients received pre-operative radiotherapy (group A) while 26 patients underwent initial surgery (group B). Complications were defined as major wound complications if they were severe enough to delay the delivery of adjuvant treatment (chemotherapy or radiotherapy) more than eight weeks after surgery or if their resolution required a new surgical intervention. RESULTS: Mean follow-up in group A and group B was 44.3 and 53.8 months, respectively. The incidence of complications of any grade in group A was 45.5% and 53.8% in group B (p: 0.566). Major wound complications in group A and group B occurred in 18% and 23% of the patients (p=0.630), respectively. All patients in group A completed local treatment - radiotherapy and surgery - in 66 days on average. In contrast, in group B post-operative radiotherapy was either delayed or suspended in four patients due to wound complications. This determined that 15.4% of the patients in group B did not receive the local treatment - surgery + radiotherapy - as planned. CONCLUSIONS: An increased risk of severe acute wound complications with the administration of pre-operative radiotherapy was not observed in patients with soft tissue sarcomas of the extremities. In addition, local treatment completion was not jeopardized with preoperative radiotherapy, as opposed to post-operative radiotherapy.