Multidisciplinary Management of Oligometastatic Soft Tissue Sarcoma.

Soft tissue sarcomas (STS) encompass a group of rare but heterogeneous diseases. Nevertheless, many patients, particularly those with oligometastatic disease can benefit from thoughtful multimodality evaluation and treatment regardless of the STS subtype. Here, we review surgical, interventional radiology, radiation, and chemotherapy approaches to maximize disease palliation and improve survival, including occasionally long-term disease-free survival. Surgical resection can include lung or other visceral, soft tissue and bone metastases with a goal of rendering the patient disease free. Staged resections can be appropriate, and serial resection of oligometastatic recurrent disease can be appropriate. Retrospective series suggest survival benefit from this approach, although selection bias may contribute. Interventional radiology techniques such as percutaneous thermal ablation (PTA) and arterial embolization can present nonoperative local approaches in patients who are not medically fit for surgery, surgery is too morbid, or patients who decline surgery. Similarly, radiation therapy can be delivered safely to areas that are inaccessible surgically or would result in excessive morbidity. Currently no randomized trials exist comparing interventional radiologic approaches or radiation therapy to surgery but retrospective reviews show relatively similar magnitude of benefit in terms of disease palliation and survival, although it is felt unlikely that these procedures will render a patient to long-term disease-free status. Chemotherapy has evolved recently with the addition of several new treatment options, briefly reviewed here. Importantly, if a patient sustains a good response to chemotherapy resulting in true oligometastatic disease, consideration of multimodality local therapy approaches can be considered in the appropriate patient.

Extradigital glomus tumor: a rare etiology for wrist soft tissue mass.

Glomus tumors are rare benign hamartomas arising from the neuromyoarterial glomus body, a highly specialized arteriovenous anastomosis responsible for thermoregulation. Although classically associated with a subungual location, less common extradigital glomus tumors can present a diagnostic challenge because of their rarity and nonspecific presentation. This case report adds to the literature of proven extradigital glomus tumors with documented pathologic and magnetic resonance imaging characteristics and describes their place in the differential for soft tissue mass of the wrist. Occasionally, a combination of imaging findings and clinical history as described may help suggest the diagnosis prospectively.

Clinical Activity of Pazopanib in Metastatic Extraosseous Ewing Sarcoma.

We report a response to pazopanib in a 69-year-old man with heavily pre-treated metastatic extraosseous Ewing sarcoma in addition to molecular profiling of his tumor. To our knowledge, this case is the earliest to demonstrate activity of an oral multi-targeted kinase inhibitor in Ewing sarcoma. This case provides rationale for adding a Ewing sarcoma arm to SARC024, a phase II study of regorafenib, another multi-targeted kinase inhibitor, in patients with liposarcoma, osteosarcoma and Ewing and Ewing-like sarcomas (NCT02048371). This national multi-institutional study is ongoing.

Atypical intradermal smooth muscle neoplasms (formerly cutaneous leiomyosarcomas): case series, immunohistochemical profile and review of the literature.

Atypical intradermal smooth muscle neoplasms (AISMN, formerly known as cutaneous leiomyosarcomas) are uncommon neoplasms, which seem to be remarkable for their excellent prognosis in contrast to their deeper counterparts. The rarity of AISMN has posed a challenge for characterizing the morphologic spectrum, immunohistochemical staining pattern, and behavior. In this study we evaluated the histologic and immunohistochemical features of 20 cases of AISMN. Clinical follow-up was available on 19 out of 20 patients and ranged from 1 to 124 months with an average of 35 months and a median of 20 months with a male predominance (male to female ratio was 2.3:1). Our data show a wide variation in differentiation and atypical features. Among these, the presence of mitotic figures is diagnostically valuable in rendering the final diagnosis. A broad panel of immunohistochemical stains revealed that smooth muscle actin and muscle specific actin, when used in combination, identified smooth muscle differentiation in 100% of the cases. With some caveats, CD34, S100, and CK 5/6 were helpful in ruling out other important cutaneous spindle cell neoplasms. Significantly, loss of phosphatase and tensin homolog (PTEN) staining was seen in the majority of our cases (80%), supporting a role for PTEN loss in the etiology of these lesions. Logistic regression analysis revealed that positive margin status was helpful for predicting recurrence (100% sensitivity and 94% specificity). We conclude that AISMN can have significant morphologic variation and overlap with other spindle cell neoplasms of the skin and that a limited panel of key immunohistochemical stains should be used to distinguish this lesion. The different surgical measures such as wide excision versus Mohs procedure showed a similar clinical outcome. Although the significance of frequent PTEN loss supports a molecular mechanism of tumor genesis, the diagnostic utility of the stain remains to be determined.

Intraoperative navigation for minimally invasive resection of periarticular and pelvic tumors.

The surgical approach to benign, metastatic, and some low-grade malignant tumors is often difficult due to their typically precarious locations. This article presents a series of cases where intraoperative stealth navigation was used to treat periarticular tumors. The use of paired point imaging with image fusion has made approaching tumors through an accurate and minimally invasive technique a viable option for the treatment of a subset of musculoskeletal tumors.

Intratendinous tophaceous gout imitating patellar tendonitis in an athletic man.

Patellar tendon-related pain is common in the athletic patient. When it occurs in skeletally mature patients participating in running, jumping, or kicking sports, the diagnosis of jumper's knee patellar tendonitis is usually made. If patellar tendon pain is associated with a mass, the differential diagnosis should be broadened to include crystalline arthropathy. This article presents a case of a highly athletic 45-year-old man with a history of gout, anterior knee pain, and an enlarging mass in the region of the patellar tendon. Conservative management failed, and an excisional biopsy found it to be an intra-tendinous gouty tophus. To our knowledge, only 1 report exists documenting a patellar tendon mass secondary to gout, and no case report exists documenting this problem in an athlete. The interplay between athletics and gout has not been well described. Despite the long-term protective nature of fitness, transient elevations in uric acid associated with athletic endeavors may contribute acutely to manifestations of gout in some athletes. Resultant intra- or extra-articular pathology may present as, and easily be mistaken for, a sports-related injury. Without appropriate medical management, tophaceous deposition may continue to occur and treatment of the resultant mass may require surgical intervention.