Primary pulmonary arterial sarcoma treated with endostar injection and radiotherapy.

We present a case of primary pulmonary arterial sarcoma (PPAS) treated with endostatin (endostar) injection and radiotherapy  discuss the diagnosis, clinical manifestations, and pathology of PPAS. The patient complained of cough with sputum, fever, and chest pain with hemoptysis. Numerous nodules were observed in the computed tomography (CT) scan. The patient was diagnosed with pulmonary embolism (PE) by computed tomography pulmonary angiography (CTPA). The pathology and immunohistochemistry results indicated soft tissue sarcomas, indicative of angiosarcoma. The nodules shrunk after 5 courses of endostatin and one course of radiotherapy, as determined in the CT scan. Primary pulmonary arterial sarcoma is clinically rare with nonspecific symptoms. Hence, it can be easily misdiagnosed as PE, and biopsy must be performed for confirmation. Current treatment methods, including surgery, are limited. Therefore, administration of endostatin injection combined with other therapies may be an alternative treatment methods.

Primary pulmonary arterial sarcoma treated with Endostar injection and radiotherapy.

A case of primary pulmonary arterial sarcoma (PPAS) treated with Endostar injection and radiotherapy and discuss the diagnosis, clinical characteristics, and pathology of PPAS. The patient complained of cough, sputum, fever, and chest pain with hemoptysis. Numerous nodules were seen in the computed tomography scan. The patient was diagnosed as pulmonary embolism (PE) by computed tomography pulmonary angiography. The pathology and immunohistochemistry results indicated soft tissue sarcomas, indicative of angiosarcoma. The nodules shrunk after 5 courses of endostatin and one course of radiotherapy, as seen by CT scan. Therefore, PPAS is clinically rare with nonspecific symptoms. Hence, it can be easily misdiagnosed as PE, biopsy for confirmation. Current treatment is limited and includes surgery. Hence, endostatin injection combined with other therapy may be an alternative treatment.

Intravascular involvement of cutaneous squamous cell carcinoma.

We present a case of a 72-year-old man who was found to have intravascular invasion during Mohs micrographic surgery (MMS) of cutaneous squamous cell carcinoma (cSCC) on the left side of the forehead. The patient was subsequently treated with adjuvant radiation without evidence of disease recurrence. We present a case review to discuss treatment options for high-risk features of cSCC with the aim to assist in treatment guidelines.

External beam radiation therapy to hepatocellular carcinoma involving inferior vena cava and/or right atrium: A meta-analysis and systemic review.

PURPOSE: Hepatocellular carcinoma (HCC) involving inferior vena cava (IVC) and/or right atrium (RA) is a very rare but serious disease. The objective of this meta-analysis was to assess efficacy and safety of external beam radiotherapy (EBRT) for HCC involving IVC and/or RA. MATERIALS AND METHODS: Systematic search of Pubmed, MEDLINE, EMBASE, and Cochrane library published was performed. Primary endpoints were 1-year overall survival (OS) rate and 2-year OS rates. Secondary endpoints were response rate, local control (LC) rate, and grade ≥3 toxicities. According to heterogeneity evaluated with Cochran Q test and I2 statistics, meta-analysis was performed using either random or fixed model. RESULTS: A total of 8 studies and 9 cohorts were included, encompassing 164 patients. Pooled 1- and 2-year OS rates were 53.6% (95% CI: 45.7-61.3%) and 36.9% (95% CI: 27.2-42.4%), respectively. Pooled response rate and LC rate were 59.2% (95% CI: 39.0-76.7%) and 83.8% (95% CI: 78.8-97.1%), respectively. Only one study reported 2 grade ≥3 toxicities, an esophageal rupture and a pulmonary embolism cases. The overall rate of possible grade ≥3 complications was 1.2% (2 of 164). CONCLUSIONS: EBRT is a feasible and safe option to palliate HCC with IVC and/or RA invasion.

Moyamoya Disease Associated with Tuberculum Sellae Meningioma and Cavernous Sinus Hemangioma.

BACKGROUND: Primary moyamoya disease associated with skull base tumors has been reported only rarely in the literature. Surgical treatment can be complicated due to the compensatory collateral circulation through meningeal and leptomeningeal anastomosis. A standard frontotemporal craniotomy may interrupt critical transdural anastomoses. CASE DESCRIPTION: We report a case of primary moyamoya disease coexisting with tuberculum sellae meningioma and left cavernous sinus hemangioma. Simultaneous management of tuberculum sellae meningioma and moyamoya disease was performed using a left modified pterional incision. Two separate bone windows were opened to protect the transdural anastomosis via the middle meningeal artery. The tuberculum sellae meningioma was successfully removed through a small frontal craniotomy, and encephaloduromyosynangiosis was used to treat moyamoya disease through a temporoparietal craniotomy. Finally, CyberKnife radiotherapy was used to treat the left cavernous sinus hemangioma at 6 weeks after the initial operation. The patient recovered well without complications. This is the first report of moyamoya disease associated with tuberculum sellae meningioma and cavernous sinus hemangioma. CONCLUSIONS: With careful bone flap design, moyamoya disease and skull base tumors can be treated simultaneously. Care should be taken to avoid interruption of critical dural-pial collaterals and injury to fragile moyamoya vessels.

Liver Transplantation After Transarterial Chemoembolization and Radiotherapy for Hepatocellular Carcinoma with Vascular Invasion.

PURPOSE: The study aims to analyze the oncologic outcomes of living donor liver transplantation (LDLT) after combined transarterial chemoembolization (TACE) and radiotherapy for hepatocellular carcinoma (HCC) with major vascular invasion. METHODS: We retrospectively reviewed 17 HCC patients with major vascular invasion who underwent LDLT after combined treatment modality between May 2007 and September 2014. The LDLT timing was determined by the surgeons depending on the disease status and liver function. The intrahepatic recurrence-free survival, disease-free survival (DFS), and overall survival (OS) rates were estimated from the date of the LDLT. RESULTS: The median follow-up period was 24.5 months (range, 6.4-66.0 months) after the LDLT. The interval between the combined treatment and the LDLT was a median of 5 months (range, 0.4-65.3 months). On the explanted liver, total necrosis was shown in five patients (29.4 %). The 1- and 3-year DFS rates were 70.6 and 57.8 %, respectively. The 1- and 3-year OS rates were 87.4 and 60.5 %, respectively. The major pattern of failure was distant metastasis (35.3 %), and intrahepatic recurrence occurred in three patients (17.6 %) who experienced distant metastasis. CONCLUSIONS: In the selected HCC patients with major vascular invasion, LDLT after combined TACE and radiotherapy showed acceptable oncologic outcomes.

CT-guided implantation of (125)I seeds (permanent brachytherapy) for metastatic tumors of the hepatic portal system: Effectiveness and safety in 13 patients.

PURPOSE: To retrospectively evaluate the efficacy and safety of CT-guided implantation of (125)I seeds (permanent brachytherapy) for metastatic tumors of the hepatic portal system (HPS). METHODS AND MATERIALS: Between January 2012 and January 2015, 13 patients with metastases measuring >3.0 cm in short-axis diameter, which remained in the HPS after conventional chemotherapy and/or transcatheter arterial chemoembolization, and for which an effective therapeutic dose from external beam radiotherapy could not be delivered, received CT-guided (125)I brachytherapy. Clinical data were studied retrospectively. In terms of metrological requirements, the minimum dose to 90% of the target volume (D90) was 90-160 Gy for (125)I seeds with activity of 2.96 × 10(7)Bq. CT-based evaluation after 2, 4, and 8 weeks, as well as 6 months after implantation enabled review of local control of tumors. RESULTS: All symptoms were improved after (125)I brachytherapy. The mean value for D90 for implantation of (125)I seeds was 136 Gy. Complete response (CR) + partial response (PR) was documented in 61.5%, 69.2%, and 84.6% of patients at 2 weeks, 4 weeks, and 6 months after implantation, respectively. Four of 13 patients had complete response, 7 cases had PR, 1 patient had stable disease, and 1 case had progressive disease. All metastatic foci were controlled by implantation. No serious complications were observed. CONCLUSION: CT-guided (125)I brachytherapy is a safe and effective treatment for metastatic tumors of the HPS and can achieve good local control in the short term as long as the radiation dose is sufficient. CT-guided (125)I brachytherapy carries few complications, is simple, safe, and a good complement to cancer treatment.

Clinical exuberance of classic Kaposi's sarcoma and response to radiotherapy

Abstract: Kaposi's sarcoma (KS) is a multicentric vascular neoplasm, with cutaneous and extracutaneous involvement. Different clinical and epidemiological variants have been identified. The classic form is manifested mainly in elderly men with indolent and long-term evolution, with lesions localized primarily in the lower extremities. We present two cases of classic Kaposi's sarcoma (CKS) in two female patients with extensive, exuberant skin involvement and rapid evolution, with good response to radiotherapy.

A rare case of intravascular epithelioid hemangioendothelioma of the cephalic vein treated with surgery and postoperative radiation therapy: a case report and review of the literature.

INTRODUCTION: Epithelioid hemangioendothelioma (EHE) is a rare endothelial tumor with an intermediate grade of malignancy. Few cases of primary vascular hemangioendothelioma have been described in the literature. Surgery is the treatment of choice, but radiation therapy and chemotherapy should also be considered in particular cases. CASE PRESENTATION: We present the case of a 44-year-old Caucasian woman affected by EHE of the cephalic vein, treated by complete surgical removal of the mass and postoperative local radiation therapy. At 5-year follow-up, our patient is alive, with no signs of local or distant relapse and with no late radiation-related effects. CONCLUSIONS: Postoperative radiotherapy may play a role in cases in which tumor margins are close or cannot be assessed or when high-risk features are present.

Effects of microbeam radiation therapy on normal and tumoral blood vessels.

Microbeam radiation therapy (MRT) is a new form of preclinical radiotherapy using quasi-parallel arrays of synchrotron X-ray microbeams. While the deposition of several hundred Grays in the microbeam paths, the normal brain tissues presents a high tolerance which is accompanied by the permanence of apparently normal vessels. Conversely, the efficiency of MRT on tumor growth control is thought to be related to a preferential damaging of tumor blood vessels. The high resistance of the healthy vascular network was demonstrated in different animal models by in vivo biphoton microscopy, magnetic resonance imaging, and histological studies. While a transient increase in permeability was shown, the structure of the vessels remained intact. The use of a chick chorioallantoic membrane at different stages of development showed that the damages induced by microbeams depend on vessel maturation. In vivo and ultrastructural observations showed negligible effects of microbeams on the mature vasculature at late stages of development; nevertheless a complete destruction of the immature capillary plexus was found in the microbeam paths. The use of MRT in rodent models revealed a preferential effect on tumor vessels. Although no major modification was observed in the vasculature of normal brain tissue, tumors showed a denudation of capillaries accompanied by transient increased permeability followed by reduced tumor perfusion and finally, a decrease in number of tumor vessels. Thus, MRT is a very promising treatment strategy with pronounced tumor control effects most likely based on the anti-vascular effects of MRT.

Clinical exuberance of classic Kaposi's sarcoma and response to radiotherapy.

Kaposi's sarcoma (KS) is a multicentric vascular neoplasm, with cutaneous and extracutaneous involvement. Different clinical and epidemiological variants have been identified. The classic form is manifested mainly in elderly men with indolent and long-term evolution, with lesions localized primarily in the lower extremities. We present two cases of classic Kaposi's sarcoma (CKS) in two female patients with extensive, exuberant skin involvement and rapid evolution, with good response to radiotherapy.

Histiocytic sarcoma of the cavernous sinus: case report and literature review.

Histiocytic sarcoma is a rare malignant neoplasm of hematopoietic origin composed of cells showing morphologic and immunophenotypic evidence of histiocytic differentiation. We describe the 2nd case of primary histiocytic sarcoma of the cavernous sinus/Meckel's cave, and the 8th case involving the CNS. A 61-year-old Caucasian man presented with numbness on the entire left side of his face, shooting pain in the left frontal region, and headaches. Imaging revealed an enhancing extra axial soft tissue mass located in the left cavernous sinus and left Meckel's cave. Diagnosis was established through open biopsy, after failed attempts via CT-guided trans-foramen ovale fine-needle aspiration biopsy and keyhole biopsy. The tumor was composed of large non-cohesive epithelioid cells invading nerves and ganglion cells. Tumor cells were immunopositive for CD68, CD163, and immunonegative for the anaplastic large cell lymphoma marker ALK-1 as well as other lymphoid, myeloid, and dendritic cell markers. Histiocytic sarcoma has strong potential for systemic spread; early diagnosis and treatment are important. Our patient was initially treated with radiation therapy but subsequently developed metastases.

Extraskeletal myxoid chondrosarcoma arising in the femoral vein: a case report.

Soft tissue tumors arising in deep veins of the extremities are uncommon, although a few cases of synovial sarcoma or leiomyosarcoma arising in the femoral vein have been documented. However, to the best of our knowledge, an extraskeletal myxoid chondrosarcoma (EMC) arising in the femoral vein has not been reported in the English literature. We report a case of EMC arising in the femoral vein of a 70-year-old man who presented with right leg edema and was diagnosed with a deep venous thrombosis (DVT) by computed tomography (CT). Magnetic resonance imaging (MRI) revealed a mass in the right proximal thigh that was diagnosed as myxomatous sarcoma by aspiration cytology, and anticoagulant therapy was initiated. The mass was surgically resected en bloc, including the femoral vein and surrounding soft tissue, and the femoral artery was preserved. The femoral vein was not reconstructed. The histologic diagnosis was an extraskeletal myxoid chondrosarcoma. The patient received postoperative local radiation treatment, with a total dose of 60 Gy, and is currently doing well with no evidence of local recurrence or metastasis at 8 months after surgery. In summary, this case report shows that EMC can arise in the femoral vein, and that reconstruction of the femoral vein is not always necessary during surgery for soft tissue tumors.

[Primary pulmonary artery sarcoma in 36-year-old women: 3-years follow-up after partial resection and radiotherapy]. / Pierwotny miesak tetnicy plucnej u 36-letniej pacjentki: obserwacja 3-letnia po czesciowej resekcji i radioterapii.

Intimal sarcoma of the heart and pulmonary artery is a very rare, malignant, primary tumour. The prognosis in patients with primary sarcoma of the pulmonary artery, including intimal sarcoma, is poor. We present the case and 3-years follow-up of 36-year-old woman who was successfully treated with surgical, partial resection of the tumour followed by radiotherapy.

Leiomyosarcoma of the inferior vena cava incidentally detected.

This report presents the case of a 78-year-old man affected by retroperitoneal tumor arising from the lower segment of the inferior vena cava. The patient underwent excision of the tumor and resection of the vena cava. Postoperative histopathologic examination revealed the diagnosis of leiomyosarcoma of the inferior vena cava, a rare tumor of mesenchymal origin.

(125)I brachytherapy seeds implantation for inoperable low-grade leiomyosarcoma of inferior vena cava.

A 60-year-old female presented with abdominal pain and tenderness of five-day duration. Contrast enhanced CT showed a mass of 9 × 6 × 5.5 cm in size with almost complete obliteration of the inferior vena cava and massive extension to the extravascular space. CT-guided biopsy demonstrated a low-grade leiomyosarcoma. The patient underwent (125)Iodine seeds implantation in two sessions, and another balloon cavoplasty. Abdominal pain and tenderness gradually improved and the patient continues to remain as disease free state for three years after the procedures.

Intravascular metastatic melanoma: a difficult diagnosis.

Melanoma is a common cancer with the potential for widespread metastasis; however intravascular metastasis is extremely rare. We report an unusual case of a patient with metastatic melanoma in whom (18) F-fluorodeoxyglucose positron emission tomography-computed tomography (FDG PET-CT) demonstrated an intravascular melanoma metastasis in the superior vena cava (SVC), successfully treated with external beam radiotherapy. To our knowledge, this is the first reported case where FDG PET-CT was used to make this diagnosis.

Neoadjuvant radiotherapy and reconstruction using autologous vein graft for the treatment of inferior vena cava leiomyosarcoma.

BACKGROUND AND OBJECTIVES: Inferior vena cava (IVC) leiomyosarcomas are rare and are a relatively small subset of retroperitoneal sarcomas. The current approach is resection and ligation or reconstruction of the IVC. This study was undertaken to analyze the outcomes associated with the use of neoadjuvant radiotherapy and IVC reconstruction in the treatment of IVC leiomyosarcoma. METHODS: A retrospective clinicopathological review of patients treated during a 10-year period. RESULTS: Four patients were treated with neoadjuvant radiotherapy, median 47.5 Gy, all underwent margin negative resection with 75% of the tumors being high grade and all patients requiring resection of adjacent organs. Reconstruction of the IVC was performed with an autologous superficial femoral vein graft. There were no mortalities and the morbidity rate was 50%. At a median follow up of 37 months; two patients had a patent IVC, no patients had a local recurrence, and one patient developed a distant metastases treated successfully with metastectomy. CONCLUSIONS: Neoadjuvant radiotherapy and resection of the IVC leiomyosarcoma resulted in 100% local control, and all patients are alive at median follow up of 37 months. IVC reconstruction with the superficial femoral vein is safe and associated with acceptable short and long term morbidity.

[Efficacy of first-line radiation for non-resectable carotid paraganglioma]. / Efficacité de la radiothérapie en première intention d'un paragangliome carotidien non résécable.

BACKGROUND: Carotid paragangliomas are rare. Surgical resection is the primary treatment. However, when surgery is impossible, radiation therapy is an alternative potentially enabling local control with less morbidity. We report a case of good local control of an unresectable paraganglioma after external beam radiation. CASE REPORT: A 72-year-old-woman, had a 4-year history of right facial palsy associated with pulsatile tinnitus, episodic ear bleeding and ipsilateral hearing loss. Physical examination revealed a retro-mandibular and sub-mastoid pulsatile mass. Magnetic resonance imaging showed a large carotid paraganglioma involving the temporal bone. Since surgical resection was impossible, our patient was given external beam radiation therapy at a dose of 60 Gy. At 12 months follow-up, local control was good without significant toxicity. CONCLUSION: External beam radiation therapy seems to be a good alternative therapy for local control of carotid paragangliomas if surgical resection is impossible.