Localized Angiosarcoma, Not One Disease: A Retrospective Single-Center Study on Prognosis Depending on the Primary Site and Etiology

Background. Angiosarcomas are rare and heterogeneous tumors with poor prognosis. The clinical subtypes are classified depending on the primary site and etiology. Methods. We conducted a retrospective, monocentric study of 136 patients with localized AS between May 1985 and November 2018. Overall survival (OS), local recurrence-free survival (LRFS), and metastasis-free survival (MFS) were estimated using the Kaplan–Meier method. To identify prognostic factors, univariate and multivariate analyses were performed based on Cox regressions. Results. The median age was 67 years (19–72.8 years). Primary sites were cutaneous (27.2%), breast (38.2%), and deep soft tissue (34.6%). The majority was primary angiosarcomas (55.9%) followed by postradiation (40.4%) and chronic lymphedema angiosarcomas (2.9%). Prognosis significantly differed depending on the primary site and etiology. Shortest median OS and MFS were observed in deep soft tissue angiosarcomas, whereas cutaneous angiosarcomas, angiosarcomas of the breast, and radiation-associated angiosarcomas displayed worse median LRFS. Univariate analyses showed better OS for tumor size <10 cm ( p = 0.009), negative surgical margins ( p = 0.021), and negative lymph node status ( p = 0.007). LRFS and MFS were longer for tumor size <10 cm ( p = 0.012 and p = 0.013). In multivariate analyses, age <70 years was the only independent positive prognostic factor for OS in all subgroups. For LRFS, secondary AS of the breast was a negative prognostic factor (HR: 2.35; p = 0.035). Conclusions. Different behaviors and prognoses depending on the primary site and etiology should be considered for the treatment of this heterogeneous disease. In cutaneous angiosarcomas of the head/neck and postradiation angiosarcomas of the breast, local recurrence seems to have a crucial impact on OS. Therefore, improved local therapies and local tumor staging may have to be implemented. However, in deep soft tissue angiosarcomas, distant recurrence seems to have a major influence on prognosis, which indicates a benefit of additional perioperative chemotherapy.

Sclerosing Epithelioid Fibrosarcoma of the Kidney: First Reported Case in a Young Child

Sclerosing epithelioid fibrosarcoma (SEF) is a rare variant of fibrosarcoma primarily arising in the deep soft tissue of the extremities and trunk. Despite having the morphologic appearance of a low-grade sarcoma, it generally has an aggressive clinical course with frequent local recurrences and distant metastases. It typically occurs in middle aged adults and is characterized by immunoexpression of MUC4 and recurrent gene fusions, most commonly EWSR1-CREB3L1. We report a primary renal SEF in a 4-year-old male. To our knowledge, this is the youngest patient reported with SEF and the second case of SEF in a pre-adolescent child. It is the eleventh reported case of primary renal SEF in the literature. While SEF arising in visceral organs is rare, the kidney is the most common primary site of any visceral organ. This case demonstrates SEF can occur in pre-adolescents, is an important consideration when evaluating sarcomas in young children, and should be considered in the differential diagnosis for primary renal tumors.

The safety of primary surgical excision of small deep indeterminate musculoskeletal soft tissue masses

Objective: To determine the suitability of primary excision of small indeterminate deep soft tissue masses presenting to a tertiary musculoskeletal oncology service. Methods and materials: Review of all patients referred to a specialist musculoskeletal oncology service over a 20-month period with a deep indeterminate soft tissue mass by non-contrast MRI criteria that was recommended for primary surgical excision due to relatively small size (<30 mm). Data collected included age, gender, site and maximal size of the lesion, and final histological diagnosis for excised lesions. Results: 85 patients were included, mean lesion size being 12 mm (range 5–29 mm). Primary surgical resection had been undertaken in 69 cases (81.2%) by the conclusion of data collection, 36 males and 33 females with mean age of 45.6 years (range 11–80 years). Of these, 11 cases (15.9%) were non-neoplastic, 53 (76.8%) were benign, 1 (1.4%) was intermediate grade, while 4 (5.8%) were malignant including 3 synovial sarcomas. Two of these were treated with re-excision of the tumour bed showing no residual disease, with no evidence of local recurrence at a mean of 10.7 months post-excision. Conclusion: Primary surgical excision of small deep soft tissue masses that are indeterminate by non-contrast MRI criteria is considered a safe procedure when undertaken in a specialist musculoskeletal oncology service, with only 4 of 69 cases (5.8%) being malignant. Advances in knowledge: Small indeterminate deep soft tissue masses can safely be treated with primary excision in the setting of a specialist musculoskeletal oncology service.

Septic emboli from pyomyositis

Pyomyositis commonly presents with fever, muscle pain and abscess formation involving deep soft-tissue compartments. Staphylococcus aureus is the main causative organism and diagnosis is usually established clinically, supported by imaging, but confirmation may be achieved by histopathological examination. Broad-spectrum antibiotic therapy and surgical debridement are the cornerstone of treatment. Its prognosis is good but, as in all soft-tissue infections, it depends on early intervention, directed antibiotics and, if indicated, prompt surgery. In this paper, we describe a case of pelvic pyomyositis complicated with bacteraemia and bilateral septic pulmonary emboli in a young man in Colombia.