Progressive Pulmonary Metastases Research Articles

Gemcitabine Maintenance Therapy in Patients With Metastasized Soft Tissue Sarcomas.

BackgroundMetastasized soft-tissue sarcomas still pose a significant therapeutic challenge given the limited efficacy of currently available multimodal treatment strategies. Recent progress in molecular characterization of sarcoma subtypes has enabled successful personalized therapy approaches in a minority of selected patients with targetable mutations. However, in the majority of patients with refractory soft tissue sarcomas, long-term survival remains poor.MethodsWe report on three adult patients with various soft tissue sarcomas subjected to Gemcitabine maintenance therapy. Tumor entities included leiomyosarcoma of the pancreas (patient 1), undifferentiated pleomorphic sarcoma of the right femur (patient 2), and peri-aortic leiomyosarcoma (patient 3). Metastatic sites encompassed liver, lung, and bones. All patients received Gemcitabine maintenance therapy until disease progression following prior salvage chemotherapy with Docetaxel and Gemcitabine. Patients were treated outside of clinical trials. Response assessment was based on radiological imaging.ResultsIn response to salvage chemotherapy with Docetaxel and Gemcitabine, one patient exhibited a partial remission, and two patients showed stable disease. Patient 1 exhibited stable disease for 6 months during Gemcitabine maintenance therapy before suffering rapid progression of hepatic metastases. Patient 2 underwent 21 months of Gemcitabine maintenance therapy, which was discontinued after progressive pulmonary metastases were detected. Patient 3 is still being treated with Gemcitabine maintenance therapy. Remarkably, owing to significant chemotherapy-associated hematotoxicity, the dose of Gemcitabine dose was reduced by two-thirds. Nevertheless, stable disease with constant pulmonary metastases has been maintained in this patient for 14 months.ConclusionsGemcitabine maintenance therapy following prior Docetaxel and Gemcitabine chemotherapy is manageable and reveals potential benefits for patients with aggressive metastasized soft tissue sarcomas. Prospective trials evaluating Gemcitabine maintenance therapy are encouraged.

Ipilimumab and early signs of pulmonary toxicity in patients with metastastic melanoma: a prospective observational study.

Ipilimumab, an immune checkpoint inhibitor, is approved for treatment metastastic melanoma and is a promising agent against other malignancies. There is some preliminary evidence from case reports that ipilimumab treatment may be associated with pulmonary side effects. However, data from prospective studies on ipilimumab-related pulmonary toxicity are still scarce. Serial spirometries and measurements of CO-diffusion capacity (DLCO) in patients with metastatic melanoma before and during treatment with ipilimumab were performed. A reduction from baseline of forced vital capacity (FVC) of≥10%, or≥15% of DLCO was defined as clinically meaningful and indicative for pulmonary toxicity. Of 71 patients included in this study, a clinically meaningful lung function decline was registered in 6/65 (9%), 5/44 (11%), and 9/38 (24%) patients after 3, 6, and 9weeks of treatment initiation, respectively. Even after adjusting for age, concomitant melanoma treatment, progressive pulmonary metastases, and baseline pulmonary function values, mean±SD DLCO decreased significantly during follow-up (-4.3%±13.6% from baseline, p=0.033). Only 7% of patients reported respiratory symptoms. Clinically manifest ipilimumab-related pneumonitis was diagnosed only in one patient (1.4%). DLCO decline maybe an early indicator of subclinical pulmonary drug toxicity. Therefore, routine pulmonary function testing including DLCO measurement during treatment might help for risk stratification to screen for ipilimumab-related pneumonitis.

Surgical management of pelvic Ewing's sarcoma in children and adolescents.

The present study describes a novel surgical strategy used to treat immature pelvic Ewing's sarcoma (ES), one made possible owing to the intrinsic structure of the skeletally immature pelvis. A total of 12 children and adolescents with open triradiate cartilage received limb-salvage surgeries following a diagnosis of pelvic ES. In total, 3 patients with iliac lesions (2 lesions with extension into the sacrum) received surgical tumor excisions and allograft reconstructions. Another 8 patients with periacetabular lesions received trans-acetabular osteotomies and allograft reconstructions. No reconstruction was performed on 1 patient following excision of a pubic lesion. The median follow-up time was 39 months. At the time of the final follow-up appointment, 9 patients exhibited no evidence of disease, 2 patients had slow progressive pulmonary metastasis, 1 patient exhibited local recurrence of the disease and 1 patient had succumbed to the disease 24 months after surgery. Complications included issues with wound healing in 1 patient, a discrepancy in leg length in another and a screw loosening in a further patient. The patients had a mean Musculoskeletal Tumor Society score of 26 points and a mean International Society of Limb Salvage radiographic score of 90.1%. The proposed surgical strategy for children and adolescents with pelvic ES in the present study could provide a feasible solution for acetabulum salvage and local tumor control.

Giant-cell tumor of bone with pathological evidence of blood vessel invasion

Giant cell tumor of bone is a rare but aggressive benign tumor that arises at the end of long tubular bones. The tumor rarely metastasizes; however, we report a case in which a giant cell tumor of bone presented with progressive pulmonary metastases. There has been no clear pathologic evidence of the definitive cause or route of metastasis. In our case, the primary tumor site was located in the left femur with pathological evidence of blood vessel invasion. The histological and pathological features of this entity are discussed in this letter to the editor.

Clinical, therapeutic and prognostic features of osteosarcoma of the jaws – Experience of 36 cases

IntroductionOsteosarcoma of the jaws (OSJ) differs from osteosarcoma of other skeletal regions due to later development, a high mortality associated with the local disease, fewer incidences of metastases and its extreme rarity. In regard to clinical and pathological parameters as well as therapeutic approaches and prognosis, OSJ has not been specifically examined to date. In order to achieve a better understanding of this special malignancy, an evaluation of incidence, treatment and prognosis of patients with OSJ in our department over the past 38years was conducted. Materials and methodsA retrospective analysis of patients with OSJ between 1972 and 2010 was performed. Information regarding patient characteristics, site of the lesion, main presenting symptoms, latency of initial diagnosis, treatment, histology, local recurrence, development of metastatic disease, duration of follow up and survival was obtained. The data were compared to the literature. ResultsThirty-six patients (2–81years, mean: 33.9, standard deviation: 21.3) were diagnosed and treated for OSJ (maxillar:mandibular nearly 1:2). Initial symptoms were local swelling (81%) and pain (47%). The latency period between fist symptoms and clinical presentation was 3.7months (1–24). A radical resection alone was conducted in 15 patients. In nine patients, resection and radiotherapy was used. Resection with chemotherapy was the treatment of choice in seven patients. Five patients received a triple combination of resection, chemo- and radiation therapy. The osteoblastic subtype of osteosarcoma was most frequent (42%). In 15 cases (42%) local recurrences, in two cases (5%) metastasis were seen. Of these patients, 13 died within the observation period. One other patient (3%) died as a result of progressive pulmonary metastasis. A mean total survival rate of 61% could be seen whereas the highest survival rate (80%) was found in patients who were treated with neoadjuvant chemotherapy, radical resection and adjuvant radiation. Positive prognostic factors were a younger age and tumour-free resection margins. DiscussionOSJ is a highly lethal tumour entity. According to the data at hand, therapy should possibly include chemotherapy, radical resection and irradiation. Nevertheless, due to the rarity of OSJ, information remains limited and the treatment of choice should be within the focus of clinical multi-centre studies.

Nested Stromal Epithelial Tumor of the Liver—Liver Transplantation and Follow-Up

The nested stromal epithelial tumor (NSET) of the liver is a rare tumor entity which is being reported in young girls. In our 16-year-old female patient, we have performed a liver transplantation (LTX) for a non-metastasizing non-resectable liver tumor. The patient was tumor free in the follow-up. At 28months postoperatively, we detected lung metastases in the F18-FDG-PET/CT. The patient died 37months after LTX from progressive pulmonary metastases. LTX should not have been generally recommended for NSET. Further statements about the value of LTX for NSET will be possible only after evaluation of the course of the disease in a larger number of transplanted patients.

Parathyroid Carcinosarcoma: A Case Study

EDUCATIONAL OBJECTIVE:*At the conclusion of this presentation, the participants should be able to understand the diagnosis and treatment of parathyroid carcinoma, as well as gain awareness of parathyroid carcinosarcoma, an even more rare clinical entity. Objectives:*Parathyroid Carcinosarcoma was first described by Nacamuli et. al. in 2002. We present the second case of this rare disease. Methods/Study Design:* We present a case report of a patient with parathyroid carcinosarcoma and review the relevant literature. A 57 year old woman with long standing right sided vocal cord paralysis presented with a progressive 3 x 2 x 3 cm mass in the right neck. She had previously undergone a total thyroidectomy at an outside hospital, revealing benign pathology. Parathyroid hormone and calcium blood levels were within normal limits. Results:* The mass was surgically removed with negative surgical margins. Histopathology and immunohistochemistry was consistent with parathyroid carcinosarcoma. The patient received adjuvant chemoradiation therapy, and now, seven months post-operatively she has no evidence of local regional recurrence but has developed progressive pulmonary metastases. Conclusions:* This is only the second reported case of parathyroid carcinosarcoma. In our patient as well as the previously reported case, the disease has shown systemic progression despite aggressive surgical resection and adjuvant therapy.

Pulmonary Deposits of Anaplastic Oligoastrocytoma: Can It Be Prevented or Early Detected?

INTRODUCTION: Extracranial metastases of high-grade gliomas are traditionally thought to be a rare event, with a reported frequency of 0.44%. Recently published case reports have demonstrated distant metastases primarily from Glioblastoma Multiforme (WHO Grade-IV glioma) to regional lymph nodes, lungs, pleura, bone, liver; and rarely to skin, parotid and pancreas.(1,2) We describe a case of Anaplastic Oligoastrocytoma (WHO Grade-III Glioma) with rapidly progressive pulmonary metastasis after debulking of glial tumor.

Hypertrophic osteoarthropathy associated with endometrial carcinoma

The simultaneous development of digital clubbing, incapacitating arthralgias, and progressive pulmonary metastases was observed in a patient with endometrial carcinoma. Nuclide bone scan showed diffuse periosteal uptake along the tubular bones, the pattern which is typical of hypertrophic osteoarthropathy. Treatment with indomethacin rapidly relieved joint pain and swelling.