Israel Medical Association Journal

Background: Dermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive, soft-tissue sarcoma. The treatment is surgical and includes wide local excision (WLE) or Mohs micrographic Surgery (MMS). There is no consensus regarding the preferred type of surgery.

Objectives: To compare the outcomes of the two types of surgery (WLE and MMS).

Methods: This retrospective cohort study was based on the medical records of 59 patients with DFSP treated at Sheba Medical Center (using the WLE method) or Assuta Medical Center (using the MMS method) between 1995 and 2018. The data included demographics, clinical presentations, imaging, types of wound closures, pathological margin status, surgical defect sizes, recurrences, and follow-up.

Results: Of the 59 included patients, 18 (30.5%) underwent WLE and 41 (69.5%) underwent MMS. The mean age at diagnosis was 40.1 ± 14.4 years. The male-to-female ratio was 1.5:1. The main tumor location was the trunk (50% for WLE and 41.5% for MMS). The main type of closure for both procedures was primary closure. In 72.2% of WLE and 78.8% of MMS cases, the margins were free. The difference between the final surgical defect and the original tumor size was statistically significantly smaller in patients who underwent MMS. The median duration of follow-up was 6.6 years. There was no significant difference in the rate of recurrence.

Conclusions: MMS enables better tissue preservation and results in a minor surgical defect compared to WLE, with no difference in tumor recurrence between the two methods.

Fever of unknown origin (FUO) is defined as the repeated occurrence of elevated body temperature above 38.3°C (101°F) lasting for at least 3 weeks with no clear diagnosis despite a thorough investigation of more than one-week duration. FUO cases could be categorized into three major etiologies: infectious, neoplastic, and systemic inflammatory. Despite novel diagnostic modalities, clinicians still encounter a significant number of unresolved FUO cases, accounting for as many as 50% of cases [1]. Prolonged futile FUO investigations may be a source of frustration for many clinicians [2]. We described a unique cause for FUO that shares the complexity of the diagnostic workup and emphasizes the importance of ¹⁸F-fluorodeoxyglucose positron-emission tomography/computed tomography (PET/CT) modality in the process of investigating FUO.

Histiocytic sarcoma (HS) is a rare hematopoietic malignancy originating from the monocyte/macrophage bone marrow lineage. HS can occur in isolation or in association with other hematological neoplasms such as non-Hodgkin lymphoma (NHL), myelodysplasia, or acute leukemia. Clinically, HS can affect lymph nodes, gastrointestinal tract, skin, bone marrow, and spleen as well as the central nervous system. Most cases of HS follow an aggressive clinical course, with most patients dying of progressive disease within one year of diagnosis

Background: Trabectedin is a marine-derived chemotherapy, which has received U.S. Food and Drug Administration approval for use in anthracycline-resistant advanced soft tissue sarcoma (STS), especially liposarcoma and leiomyosarcoma (L-sarcomas).

Objectives: To describe our 10 year real-life experience with trabectedin regarding safety and efficacy in a cohort of 86 patients.

Methods: In our study cohort, 46.51% were diagnosed with liposarcoma and 43.02% with leiomyosarcoma. A total of 703 cycles of trabectedin were given, with a median of five cycles per patient (range 1–59). Median overall survival was 13.5 months for the whole cohort, 11 months for liposarcoma patients (range 1–63), and 15 months for leiomyosarcoma patients (range 1–35).

Results: There was no statistically significant difference in progression free survival when stratified according to previous treatment lines given. Trabectedin exhibited a favorable safety profile, with only 22% requiring dose reductions. Grade 3 and higher toxicity was noted in 25% of the patients, mostly due to myelosuppression. There were no treatment-related deaths.

Conclusions: Trabectedin is a safe and effective drug for treating advanced STS. Our results reflect real-life data with patients receiving the drug as a third and even fourth line of treatment, or with a suboptimal performance status, yet achieving impressive clinical benefit rates and survival.

Background: Uterine carcinosarcoma (UCS) is a rare tumor with a poor prognosis. An elevated thrombocyte count and thrombocytosis were found to be associated with poor prognosis in several gynecological tumors. Data regarding an elevated thrombocyte count and thrombocytosis, particularly in UCS, are scarce.

Objectives: To assess the frequency of a preoperative elevated thrombocyte count and of thrombocytosis in UCS patients and their association with clinicopathological prognostic factors and survival.

Methods: The preoperative thrombocyte count of 29 consecutive verified USC patients diagnosed in our medical center from January 2000 to July 2015 was recorded, and clinicopathological data of these patients were abstracted from hospital files. 

Results: Thrombocytosis was found in two patients (6.8 %) and both died of the disease. An elevated thrombocyte count was found in nine patients (31.0%). The percentage of patients with the poor prognostic factors who had a preoperative elevated thrombocyte count was not statistically different from those without these risk factors. The cumulative survival of patients with an elevated count was 22.1 months and that of those without an elevated count was 31.1 months. This difference was statistically not significant (P = 0.85). There was also no difference between the groups regarding the progression free survival.

Conclusions: No association between an elevated thrombocyte count and prognosis was found. Larger studies are needed to clarify this issue.