Primary liposarcomas constitute less than 1% of all mediastinal tumors. The posterior mediastinum is the location in 95% of cases. Literature search did not show a case that encompassed three mediastinal compartments.
A 32-year old man was diagnosed with high grade primary mediastinal liposarcoma (80% myxoid plus 20% round cell), after presenting with superior vena cava syndrome. CT-scan revealed lobulated masses on the superior, anterior, and the right middle mediastinum measuring 7.8 x 9.0 x 7.5 cm and compressing the superior vena cava. He underwent surgical debulking and palliative 3 cycles doxorubicin-based chemotherapy, but later succumbed to pneumonia.

Human ; Male ; Adult ; Liposarcoma ; Mediastinum ; Vena Cava, Superior ; Myxoid ; Doxorubicin ; Pneumonia

Background: Follicular dendritic cell sarcoma (FDCS) accounts for about 0.4% of soft tissue sarcomas. Approximately onethird of cases occur in extranodal sites and about 28% of extranodal FDCS may metastasize. Intra-abdominal occurrence is rare and there is limited published data to guide oncologists on how to best treat this malignancy. Case Presentation: This is a case of a 33-year-old female who came in due to incidental finding of a left supraclavicular mass with 2-year history of early satiety. Neck node biopsy revealed a poorly differentiated malignant tumor with positive staining for CD21, CD23, vimentin and S100 consistent with FDCS. PET-CT revealed an intensely FDG-avid large mass in the left upper abdomen with signs of necrosis and mass effect. The patient was given three different chemotherapy regimens that included (1) gemcitabine/docetaxel, (2) single agent doxorubicin and (3) ifosfamide/etoposide, but she progressed on all these. Off-label use of bendamustine was then offered and after just the first cycle, the patient reportedly regained strength and was able to get up from wheelchair with noted interval decrease in size of the cervical mass. Unfortunately, the patient deteriorated and succumbed to infection and multiple pulmonary embolisms. Conclusion Intra-abdominal FDCS is a rare malignancy with heterogenous outcomes with no uniform treatment strategy at present. Molecular tumor board discussion and multi-disciplinary approach in extranodal FDCS is important in the diagnosis and management. Patients with multiple poor prognostic factors are at risk for tumor recurrence, metastasis, and death.
Dendritic Cell Sarcoma, Follicular ; Abdominal Neoplasms ; Drug Therapy ; Bendamustine Hydrochloride ; Prognosis

Background: Sarcomas, a heterogenous group of malignancies consisting of tumors, are rarely encountered in most general oncology clinics. As a result, most general oncologists have very little experience in dealing with these tumors and resulting in poorer treatment and survival outcomes. Having successful collaborative efforts in research, the Asian Sarcoma Consortium (ASC) pioneered a Sarcoma Preceptorship Program with its main goal of increasing awareness of the multidisciplinary approach in the management of sarcomas. Objective: The objective of this study is to evaluate the ASC Sarcoma Preceptorship Program using the first 2 levels of the Kirkpatrick Evaluation Model: Level 1) Reaction – through using satisfaction surveys; and Level 2) Knowledge – focusing on the amount of information gained from the activity evaluated by test-retest method. Methods: The 2-day preceptorship program held in Singapore utilized educational activities such as didactic lectures, multidisciplinary case discussions, and case presentations. The program was evaluated using: 1) pre-test and posttest; 2) satisfaction survey from ASC; and 3) satisfaction survey made by the sponsor, Novartis Pharma. Results: The preceptorship program enrolled 30 participants, most from the Asia-Pacific region. The overall results from the satisfaction surveys were generally optimistic, with results showing high levels of satisfaction with regard to the time allotment, scope of topics, and how helpful each session was. Test results showed that participants scored lower in post-test as compared to the pre-test. Computation of the Coefficient of Test reliability showed that although the pre-test was highly reliable, this was not true of the post-test. Conclusions Based on the Level 1 evaluation, the program was successful in terms of both participant and faculty satisfaction. However, certain areas for improvement were identified and recommendations were made in order to effect improvements for future iterations of this preceptorship.
soft tissue sarcoma ; clinical preceptorship

Background: Sarcomas, a heterogenous group of malignancies consisting of tumors, are rarely encountered in most general oncology clinics. As a result, most general oncologists have very little experience in dealing with these tumors and resulting in poorer treatment and survival outcomes. Having successful collaborative efforts in research, the Asian Sarcoma Consortium (ASC) pioneered a Sarcoma Preceptorship Program with its main goal of increasing awareness of the multidisciplinary approach in the management of sarcomas. Objective: The objective of this study is to evaluate the ASC Sarcoma Preceptorship Program using the first 2 levels of the Kirkpatrick Evaluation Model: Level 1) Reaction – through using satisfaction surveys; and Level 2) Knowledge – focusing on the amount of information gained from the activity evaluated by test-retest method. Methods: The 2-day preceptorship program held in Singapore utilized educational activities such as didactic lectures, multidisciplinary case discussions, and case presentations. The program was evaluated using: 1) pre-test and posttest; 2) satisfaction survey from ASC; and 3) satisfaction survey made by the sponsor, Novartis Pharma. Results: The preceptorship program enrolled 30 participants, most from the Asia-Pacific region. The overall results from the satisfaction surveys were generally optimistic, with results showing high levels of satisfaction with regard to the time allotment, scope of topics, and how helpful each session was. Test results showed that participants scored lower in post-test as compared to the pre-test. Computation of the Coefficient of Test reliability showed that although the pre-test was highly reliable, this was not true of the post-test. Conclusions Based on the Level 1 evaluation, the program was successful in terms of both participant and faculty satisfaction. However, certain areas for improvement were identified and recommendations were made in order to effect improvements for future iterations of this preceptorship.
Sarcoma

: Vulvar Rhabdomyosarcoma (VR) is a rare gynecological cancer primarily found in children. This case report discusses the diagnosis, treatment, and management complexities of a 19-year-old patient with a slow-growing vulvar lesion. : A 19-year-old female with obesity and non-alcoholic fatty liver disease presented with a left vulvar lesion measuring 11 x 7 x 7 cm that was noticed five months ago. Core needle biopsy of the lesion revealed findings consistent with rhabdomyosarcoma. A Positron Emission Tomography-Computed Tomography (PET-CT) scan showed a hypermetabolic 8.3 x 6.7 x 6.7 cm mass in the left vulvar area, extending to the vagina, rectal wall, and anal region along with enlarged left inguinal lymph nodes. : The patient was treated with the Intergroup Rhabdomyosarcoma Study-IV (IRS-IV) protocol for 16 weeks with vincristine, dactinomycin, and cyclophosphamide. Concurrent chemoradiotherapy was administered between weeks 9-14, followed by continuation chemotherapy until week 28. Interim PET-CT scan prior to concurrent chemoradiotherapy revealed a reduced mass size to 3.8 x 2.8 cm and resolved left inguinal lymphadenopathy. : Despite completing treatment, the patient reported persistent back pain and mobility issues three weeks later. A subsequent PET-CT scan showed hypermetabolic lesions at vertebral locations C6, T9, T12, and L1-L3, along with the left ischium and bilateral femoral shafts. Thoracic vertebrae biopsy confirmed rhabdomyoblasts. Patient underwent palliative radiotherapy and spinal stabilization then proceeded with second line therapy with 1 cycle of GemcitabineDocetaxel but showed progression of symptoms described as persistent bleeding (hematuria) and neutropenia. Further diagnostics done to the patient showed possible bone marrow involvement. Unfortunately, the patient expired owing to symptoms of cancer progression. The management of adult-onset VR presents a significant therapeutic challenge, largely attributable to the scarcity of clinical trials and tailored treatment regimens for this specific age group. Outcomes documented in existing literature for adult VR cases present with recurrence, disease progression, and mortality. The treatment landscape in adults is complicated by comorbidities which may influence both the therapeutic choices and outcomes. Given these intricate challenges, this case echoes the need for research efforts aimed at developing management protocols specifically designed for adults with VR.
rhabdomyosarcoma ; adolescent