Clinical case of angiosarcoma of pericardium and pleura

Primary heart tumors are an extremely rare pathology, previously detected only by autopsy data and detected in 0.001%-0.03% of cases. Primary angiosarcomas of the pericardium are even more rare and are presented in the literature with isolated descriptions. The article describes a clinical case of epithelioid angiosarcoma of the pericardium and pleura of high malignancy in a 57-year-old man. The diagnosis was confirmed by immunohistochemical examination and positron emission tomography. For today, chemotherapeutic treatment of angiosarcomas localized in the chest cavity is a palliative treatment method. In this patient, the most effective scheme was the one containing anthracyclines (doxorubicin). Despite the ongoing therapy, the patient died 15 months after the diagnosis was made, with increasing signs of respiratory and heart failure. Thus, when patients, especially young and middle-aged men, show signs of hydropericardium without established etiological prerequisites, it is necessary to remember about the possibility of developing a primary malignant tumor of the heart and / or pericardium as the cause of this condition and include it in the standard diagnostic search.

Case Report: Postoperative Recurrence of Adrenal Epithelioid Angiosarcoma Achieved Complete Response by Combination Chemotherapy With Liposomal Doxorubicin and Paclitaxel

BackgroundPrimary adrenal epithelioid angiosarcoma is an extremely rare cancer with a poor prognosis. Because of the rarity of this disease, treatment options have not been well-studied.Case presentationA 51-year-old man was admitted to Zhejiang Cancer Hospital, diagnosed with a recurrence of adrenal epithelioid angiosarcoma. He had undergone a surgical resection seven months earlier. Combination chemotherapy with liposomal doxorubicin and paclitaxel was administered. After two cycles of chemotherapy, his pain was relieved. Computed tomography (CT) suggested that the soft tissue tumour lesions in the surgical area had disappeared, mediastinal and mediastinal-hilar lymph nodes were significantly reduced or had disappeared, and the patient had achieved a partial response (PR). CT after six cycles of chemotherapy indicated that the patient had achieved a complete response (CR).ConclusionCombination chemotherapy with liposomal doxorubicin and paclitaxel may be a preferred therapy for recurrent or advanced adrenal epithelioid angiosarcoma.

Pediatric Epithelioid Angiosarcoma of the Maxillary Sinus: Clinicopathologic, Immunophenotypic and Molecular Study of a Rare Entity

Introduction/Objective Angiosarcomas are aggressive neoplasms of the vascular endothelium and comprise approximately 2% of all soft tissue sarcomas. The occurrence in the sinonasal cavity is rare. A case of epithelioid angiosarcoma of the maxillary sinus in a 19-year-old patient is presented and its clinical presentation, histologic, immunohistochemical, and molecular features are discussed. Methods/Case Report A 19-year-old male evaluated at Rutgers University Hospital for 3 months of left-sided epistaxis. He reports left side nasal obstruction, facial pain, and epiphoria. Computed tomography revealed a heterogeneous soft tissue mass within the left maxillary sinus; with dehiscent segments extending into the premaxillary space, infratemporal fossa, and the left orbital floor. Bilateral rigid nasal endoscopy showed bowing of the left maxillary sinus medial wall, exposing a mucosalized mass. He underwent left maxillary antrostomy and left subtotal maxillectomy. Positron emission tomography scan showed no metastasis. The tumor was within the submucosa of the respiratory epithelium and focally demonstrated anastomosing vascular spaces lined by atypical endothelial cells. The neoplastic cells showed epithelioid morphology with increased nuclear size, prominent nucleoli, and atypical mitotic figures up to 22 mitoses per 10 high power fields. The tumor cells were strongly and diffusely positive for CD31, CD34, FLI1, ERG, vimentin, and cytokeratin AE1/AE3. The tumor was negative for HMB45, desmin, HHV8, CAMTA1, FOSB, and S-100 and retained the expression of INI-1. The proliferation index was 60-70%. A next generation sequencing disclosed stable tumors microsatellite status and a mutational burden of 1 mutation/Mb. Numerous variants of uncertain significance were identified including amplification of BRIP1, GNA13, and PRKAR1A as well as various point mutations including FLT4 G1296S and G71R, MET R359Q, ROS1 R442Q, DOT1L R757W, PDCD11 R1610L, TLL2 R849C, FANCD2 D400E, LEF1 D177V, PRDM1 A408P, FLT1 F989L, and MAP3K1 S939C. Results (if a Case Study enter NA) NA Conclusion Sinonasal angiosarcoma is incredibly rare with a poor overall prognosis. Few lesions have been reported in the pediatric population. Angiosarcoma may be confused with other types of malignant neoplasms when epithelioid morphology is present. The use of multiple vascular and epithelial markers with clinical correlation will be helpful in the diagnosis.

Epithelioid Angiosarcoma of the Tibia: A Case Report and Literature Review

Background：Epithelioid angiosarcoma (EA) is characterized by epithelioid-like neoplastic cells. In fact, there is little literature regarding EA of bone. Accordingly, this study presents an imaging analysis of a 66-year-old man who suffered from EA in his right tibia.Case presentation: A 66-year-old man developed right ankle pain four months prior to initial evaluation at our institute, which was progressively worsening for six days. All laboratory data were within the reference ranges. Plain radiographs and computed tomography (CT) revealed osteolytic lesions with multiple separations in the distal tibia, measuring 7.9 cm × 4.6 cm × 4.4 cm in size. The lytic lesions were ill-circumscribed and lacked marginal sclerosis. Punctate irregular calcifications and low-density areas were observed within lesion's areas. The cortical bone was irregularly thinned and was discontinuous. On magnetic resonance imaging (MRI), the lesions were heterogeneous. Immunohistochemically, the tumor cells expressed the vascular markers CD31, CD34, and factor VIII and revealed 5% positivity for Ki67. Finally, the patient was diagnosed with EA of bone.Conclusions: We reported a case of EA that occurred in the right tibia and summarized the imaging features of EA by reviewing the literature. Although pathological examination remains the gold standard for diagnosing EA, specific imaging features may assist in diagnosis.