Primary left atrial angiosarcoma mimicking pericarditis: A case report and review of the literature

A 45-year-old male presented with symptoms mimicking pericarditis, including rapidly worsening dyspnea and retrosternal chest pain. On imaging workup, an abnormal mass of 50x53 mm in size was detected at the left atrium, which partially obstructed blood flow through the mitral valve. PET/CT was done in searching for the probable site of origin but revealed no abnormal uptake lesions. The tumorectomy and excision of the posterior wall of the left atrium were then performed with curative intent. The postoperative histology of the tumor was in favor of a spindle cell sarcoma, originating from the left atrium, grade 2, which was confirmed as epithelioid angiosarcoma by immunohistochemistry. The patient denied adjuvant radiation, thus we treated him with six cycles of Paclitaxel monotherapy, which was completed six months ago. At present, he is doing well with no signs of recurrence on the imaging technique. This paper illustrates the rarity of cardiac angiosarcoma, its complex presentation, and a brief review of available treatment options for this devastating disease.

An Analysis of 20 Cases of Radiation-Associated Sarcoma, Including 4 Cases Treated by Carbon Ion Radiotherapy

Introduction Radiation-associated sarcoma (RAS) is one of the most life-threatening complications associated with the treatment of malignant neoplasms. Because all RAS patients have a history of radiotherapy, there have been no effective treatment options when RAS is not completely resected. Methods We retrospectively reviewed 20 RAS patients, including 4 unresectable cases treated by carbon ion radiotherapy (CIRT). Results The primary diseases targeted by radiotherapy included malignant lymphoma (n=4), cervical cancer (n=3), pharyngeal cancer (n=3), breast cancer (n=2), lung cancer (n=1), rectal cancer (n=1), maxillary cancer (n=1), synovial sarcoma (n=1), and benign neoplasms (n=4). The histological diagnoses of RAS included osteosarcoma (n=8), leiomyosarcoma (n=3), undifferentiated pleomorphic sarcoma (n=3), rhabdomyosarcoma (n=1), angiosarcoma (n=1), malignant peripheral nerve sheath tumor (n=1), spindle cell sarcoma NOS (n=1), and sarcoma not further specified (n=2). The median survival time from the diagnosis of RAS was 26 months. Eleven patients underwent surgery. Five of these patients achieved a continuous disease free status or showed no evidence disease. Four patients underwent CIRT. One of these patients with leiomyosarcoma achieved a continuous disease free status, and the other patient with osteosarcoma achieved a partial response. On the other hand, 2 patients experienced Grade 3 toxicities that required surgical treatment. Conclusion RAS originates from various types of diseases that are treated by radiotherapy and shows diverse pathological features. Complete resection achieves a good prognosis. CIRT can be an effective and feasible option for unresectable RAS.

Rare primary hepatic carcinosarcoma composed of hepatocellular carcinoma, cholangiocarcinoma, and sarcoma: a case report

Primary hepatic carcinosarcoma (HCS) is an extremely rare malignant tumor of the liver that contains carcinomatous and sarcomatous components. The diagnosis, treatment, and prognosis of HCS pose great challenges to clinicians. Herein, we present a case of HCS in a 67-year-old man with unique pathological manifestation. Preoperative magnetic resonance imaging showed a malignant lesion in the right liver and a small sub-focus in the left liver. Radical treatment was performed, including excision of the right posterior lobe of the liver, thrombectomy of the right posterior portal vein, and radiofrequency ablation of lesions in the left liver. The specimens were confirmed to be HCS by pathological examinations, which revealed a combination of poorly differentiated hepatocellular carcinoma, moderately differentiated cholangiocellular carcinoma, and spindle cell sarcoma. Transhepatic arterial chemotherapy and embolization was performed after surgery. Unfortunately, pulmonary metastasis occurred 1.5 months later, which meant a poor prognosis. In this report, we discuss the clinicopathological characteristics of this case and factors that affected surgical outcomes, which may add some ideas for the future diagnosis and treatment of HCS patients.

Dedifferentiated Central Chondrosarcoma: A Clinical, Histopathological, and Immunohistochemical Analysis of 57 Cases

Dedifferentiated central chondrosarcoma (DCCS) is a rare cartilage tumor with invasive biological behavior and a poor prognosis. To better understand the morphological characteristics of this type of tumor and its internal mechanism of dedifferentiation, we retrospectively analyzed 57 cases of DCCS. A total of 29 female and 28 male patients were included, ranging in age from 20 to 76 years, with a median age of 54 years. Fifty-seven cases of DCCS occurred in the pelvis (n = 29), femur (n = 17), scapula (n = 4), tibia (n = 2), humerus (n = 2), metatarsals (n = 1), fibula (n = 1), and radius (n = 1). Radiologically, DCCS had two different appearances on imaging, with an area showing calcifications of the cartilage forming the tumor juxtaposed to a lytic area with a highly aggressive, non-cartilaginous component. Histopathologically, the distinctive morphological features consisted of two kinds of defined components: a well-differentiated cartilaginous tumor and non-cartilaginous sarcoma. The cartilaginous components included grade 1 (n = 38; 66.7%) and grade 2 (n = 19; 33.3%) cartilage. The sarcoma components included those of osteosarcoma (n = 29; 50.9%), undifferentiated pleomorphic sarcoma (n = 20; 35.1%), rhabdomyosarcoma (n = 3; 5.2%), fibrosarcoma (n = 2; 3.5%), spindle cell sarcoma (n = 2; 3.5%) and angiosarcoma (n = 1; 1.8%). Immunohistochemistry showed that the expression of p53 and RB in the sarcoma components was significantly higher than that in the cartilaginous components, suggesting that these factors play roles in the dedifferentiation process of chondrosarcoma. DCCS is a highly malignant tumor with a poor prognosis. Except for the patients who were lost to follow-up, most of our patients died.

Spontaneous healing of malignant neoplasms. Saue g bru.ch (Deut. Med. Woch., 1922, no. 5)

Saue g bru.ch (Deut. Med. Woch., 1922, No. 5) observed 3 cases of complete spontaneous healing of neoplasms, the certainty of the malignant nature of which was precisely established. another, in a woman of 56 years, - advanced breast cancer, in a third, in a 22-year-old girl, - spindle cell sarcoma of the hip. The author also cites a number of similar cases from the literature. Such cases, apparently, speak for the possibility of conservative treatment of malignant neoplasms with such means as x-rays, Proteinkrpertherapie, etc.

Malignant spindle cell tumors of the posterior fossa in children: case series and review of management

OBJECTIVE The WHO Classification of Tumours of the Central Nervous System (2016) classifies nonmeningothelial malignant spindle cell tumors involving the extraaxial tissues of the posterior fossa as melanocytic tumors and malignant mesenchymal tumors (sarcomas). The objective of this study was to conduct a review of the literature pertaining to the management strategies of posterior fossa malignant spindle cell tumors in the pediatric population. METHODS The authors performed an institutional search of their pathology database for patients younger than 18 years of age who presented with posterior fossa malignant spindle cell tumors. A literature review was also performed using the PubMed database, with “posterior fossa” or “spindle cell tumors” or “Ewing sarcoma” or “high-grade” or “spindle cell sarcoma” or “leptomeningeal melanocytoma” as keywords. The database search was restricted to pediatric patients (age ≤ 18 years). Parameters reported from the literature review included patient age, tumor location, presenting symptoms, treatment modalities (resection, chemotherapy, and/or radiotherapy), leptomeningeal spread at or after the time of treatment, and follow-up length and resulting outcome. RESULTS The authors report 3 rare cases of posterior fossa malignant spindle cell tumors, including Ewing sarcoma in a 13-year-old male; high-grade spindle cell sarcoma, not otherwise specified in a 10-year-old male; and primary leptomeningeal melanocytoma in a 16-year-old female. All 3 patients underwent resection and radiotherapy and either chemotherapy or targeted immunotherapy. At the last follow-up, all patients were alive with either resolution or stable disease. CONCLUSIONS A review of these 3 cases and the existing literature support managing patients with intracranial malignant spindle cell tumors with multimodal therapy that can include a combination of resection, radiotherapy, and chemotherapy or immunotherapy to prolong progression-free and overall survival.