Angiosarcoma of the kidney with minute clear cell carcinomas: A case report

A case of renal angiosarcoma with minute clear cell carcinomas in a 61-year-old male is described. The tumor was clinically considered a renal cell carcinoma. The lesion, removed by nephrectomy, was an unencapsulated hemorrhagic mass measuring 8.0 cm in diameter. The tumor was histologically characterized by anastomosing vascular channels lined by cytologically atypical endothelial cells. The tumor cells reacted positively with CD31, factor-VIII related antigen, and CD34. A few minute clear cell carcinomas measuring less than 1 mm were observed in the periphery of the angiosarcoma. There was no transition between angiosarcoma and renal cell carcinoma. The patient died of a widespread disease 13 months after surgery. This tumor is considered a primary renal angiosarcoma with clear cell carcinomas and not a sarcomatoid carcinoma.     

Angiosarcoma of the breast: Report of a case and its findings of MRI

A 67-year-old woman with angiosarcoma of the left breast is presented. Physical findings showed a hard mass in the left breast with skin discoloration and erythema. Mammography showed a high density shadow in the mass without microcalcification and spicula. On ultrasonography, a hypoechoic mass with an ill-defined boundary was detected. On MRI, the tumor had low signal intensity on T1-weighted images, and higher signal intensity on T2-weighted images. MRI with Gd-DTPA images showed higher signal intensity on T1-weighted images with relatively lower intensity in the central area of the tumor. The artery supplying the tumor derived from the left inner thoracic artery and was visualized on three-dimensional dynamic MRI angiography. Initially misdiagnosed as inflammatory breast cancer, an arterial injection of CPA (100 mg) and 5-FU (500 mg) had been performed preoperatively. The definitive diagnosis of angiosarcoma was established by intraoperative frozen section examination. She underwent modified radical mastectomy and is now free of recurrence. This case emphasizes the difficulties in the clinical diagnosis of angiosarcoma of the breast.     

Angiosarcoma of the eyelid

Background: Angiosarcomas are uncommon malignant tumours of vascular endothelium. They frequently affect the face and scalp, but to our knowledge there are only three reports of eyelid involvement.
Methods/results: We report a case of angiosarcoma arising from the eyelid skin in a 79-year-old woman. Treatment was surgical with wide excision and staged reconstruction of the defect. Systemic oncological work-up for metastatic disease was negative. Adjuvant systemic chemotherapy and radiotherapy were considered but were not felt to be of proven benefit and were not used. The patient died seven months after presentation from a myocardial infarct.
Conclusion: Angiosarcoma should be considered in the differential diagnosis of unusual or atypical eyelid lesions. Management is wide surgical excision, although adjuvant systemic therapy should be considered in selected cases in consultation with an oncologist.     

肾移植患者术后并发原肾恶性肿瘤3例报告及文献复习

目的探讨肾移植术后患者并发原肾恶性肿瘤的临床特点及治疗方法。方法 1988年5月至2011年10月,在解放军第309医院器官移植中心接受同种异体肾移植手术的患者2016例,其中3例患者移植术后并发原肾恶性肿瘤,对这3例患者的临床资料进行回顾性分析,并结合文献进行复习。结果 3例患者中男性2例,女性1例,中位年龄43岁。肾移植术后采用钙调磷酸酶免疫抑制剂(CNI)+麦考酚吗乙酯(MMF)或硫唑嘌呤+泼尼松三联免疫抑制方案,术后1个月内移植肾功能均恢复正常。发生恶性肿瘤距肾移植术后时间分别14个月、16个月和54个月。首发症状分别为腰部疼痛1例、无痛肉眼血尿2例。3例患者均采用肾癌根治手术,病理结果分别为胚胎性横纹肌肉瘤并黏液性脂肪肉瘤1例、血管肉瘤1例和透明细胞癌1例。在保证移植肾功能的情况下均调整了免疫抑制剂用量。随访发现前2例患者发生远处转移,确诊半年内死亡,最后1例长期存活。结论肾移植患者术后原肾发生恶性肿瘤是一种严重的并发症,病死率极高。需要早期诊断,早期治疗。治疗上以肾癌根治术为主,术后保证移植肾功能正常的同时减少免疫抑制剂用量,同时辅以其他综合治疗。     

Pseudosarcoma - massive localized lymphoedema in morbidly obese - a rare entity: Case report

INTRODUCTIONMassive localized lymphoedema (MLL) first described in 1998 by Farshid and Weiss. Usually MLL present like huge pedunculated mass and appear like sarcoma hence called Pseudosarcoma. Morbid obesity is a growing epidemic in our society. Morbid obesity is usually associated with hypertension, Diabetes mellitus, dermatological complications like Acanthosis nigricans, skin tags, leg ulcers, edema, lymphoedema, plantar hyperkeratosis and massive localized lymphoedema (MLL) is one of the complications of morbid obesity.Pseudosarcoma is due to derangement of lymphatic channels secondary to excessive deposition of adipose tissue.PRESENTATION OF CASEWe report a patient afflicted with this unique disorder presented with huge mass arising from monspubis in morbidly obese individual with body mass index (BMI) 55.DISCUSSIONMassive localized lymphedema presenting like pseudosarcoma in morbidly obese individuals is rare. Awareness of this disease is essential to avoid misdiagnosis as soft tissue neoplasm. It is a term used to describe a benign over growth of lymhoproliferative tissue in morbidly obese patients. Because of its size patients have difficult to do daily activities. Histopathologically characterized by dilated lymphatic channels with fibrotic and edematous tissue, without evidence of malignancy. Patient seeks treatment only if there is huge swelling causing discomfort, complications like excoriation, wound break down occur. The treatment of choice is complete excision.CONCLUSIONSurgical treatment is effective if done along with bariatric surgery. Functional rehabilitation was achieved. No recurrence was observed within the follow up period of twenty months and BMI was reduced to 28.     

Different immunoreactivity of endothelial markers in well and poorly differentiated areas of angiosarcomas

This study evaluated the immunohistochemical staining of four endothelial cell markers in well differentiated and poorly differentiated areas of angiosarcomas. Formaldehyde-fixed, paraffin-embedded sections from eight angiosarcomas were studied using the antibodies anti-factor VIII-related antigen (FVIII-RA), Ulex europaeus I agglutinin, anti-CD34 (QBEND/10) and anti-CD31 (JC70). The immunostaining of the angiomatous (well differentiated) and solid (poorly differentiated) areas was separately analysed and specificity was evaluated in 20 non-vascular tumours. The antibody anti-CD31 and Ulex europaeus were the most sensitive markers staining well differentiated vasoformative structures and poorly differentiated solid areas. Anti-FVIII-RA and anti-CD34 did not stain undifferentiated malignant endothelial cells from solid areas. Ulex europaeus and anti-CD34 showed very low specificity; in contrast, none of the non-vascular tumours expressed CD31 or FVIII-RA. JC70 (anti-CD31) appears to be the most useful marker in elucidating the vascular nature of angiosarcomas. Is important to emphasize the lack of specificity of Ulex europaeus and the low sensitivity of anti-CD34 and anti-FVIII-RA for poorly differentiated lesions.     

Fine-needle aspiration biopsy of hepatic angiosarcoma: report of a case with immunocytochemical findings.

Hepatic angiosarcoma (Kupffer cell sarcoma) is a very rare but ominous malignancy. We report a case diagnosed by fine-needle aspiration biopsy (FNAB). The smear showed malignant spindle cells and a few rounded cells. The diagnosis was made on the cell block by the characteristic scaffolding arrangement of malignant cells along preexisting hepatocytes. This is the first report with immunocytochemical findings. The tumour cells stained positively for vimentin and negatively for keratin, factor VIII, Ulex europaeus agglutinin I (UEA-1), carcinoembryonic antigen (CEA), alpha-fetoprotein (AFP), and lysozyme. This case demonstrates the possibility of a definitive diagnosis by FNAB prior to death without inflicting serious complications.     

Infantile hepatic hemangioendothelioma with subsequent malignant degeneration

A 4 year old child was seen because of an enlarging epigastric mass and a rapidly falling hematocrit 31/2 years following steroid treatment for multiple hepatic hemangioendotheliomas. Technetium 99 m sulfur colloid liver scan and hepatic angiography confirmed the presence of a large, vascular mass involving the left lobe of the liver. A left hepatic lobectomy was performed and histopathology showed angiosarcoma. As children with hemangioendotheliomas of the liver begin to survive for longer periods of time, surveillance for the possible development of neoplastic disease is recommended.     

Cytokeratin-positive epithelioid angiosarcoma presenting in the tonsil: a diagnostic challenge

Primary oral cavity sarcomas are exceedingly rare and may pose a great diagnostic challenge. A 71-year-old woman without history of malignancy or radiation to the head and neck presented with an antibiotic-refractory diffuse painful swelling of the right tonsil necessitating tonsillectomy. Histologic evaluation revealed subtotal replacement of the right tonsil by a high-grade epithelioid neoplasm displaying extensive ulceration, necrosis, and primitive vasoformation. Immunohistochemistry showed strong/diffuse expression of pancytokeratin antibodies KL-1 and Lu5, cytokeratin 8, cytokeratin 18, cytokeratin 19, vimentin, CD31, ERG, and Freund leukemia integration site 1 (FLI-1). High-molecular-weight cytokeratins (cytokeratin 5, 34β12), cytokeratin 7, cytokeratin 13, and cytokeratin 20 were not expressed. Within months, the patient underwent surgical resection of multiple bleeding intraoral and gastrointestinal metastases. She is currently alive with disease 9 months from diagnosis. To our knowledge, this case represents the first well-documented primary epithelioid angiosarcoma of the tonsil. The strong cytokeratin expression in epithelioid angiosarcomas represents a diagnostic pitfall. Thus, awareness of this rare and highly aggressive neoplasm is necessary for distinguishing it from poorly differentiated and acantholytic squamous cell carcinoma and diffuse large cell lymphoma.     

Severe osteolysis and soft tissue mass around total hip arthroplasty: description of four cases and review of the literature with respect to clinico-radiographic and pathologic differential diagnosis

Periprosthetic osteolysis is a well known phenomenon caused by wear particle-induced bone resorption, particularly common and extensively reported in total hip arthroplasty. Its typical radiographic feature is a radiolucent area adjacent to an implant, sometimes associated with a soft tissue mass. Osteolytic changes may be caused by numerous other pathologic processes, including infection, metabolic disease, and neoplasia. Four cases of massive periprosthetic bone destruction associated with a large soft tissue mass around a failed total hip replacement are presented. In three cases, a diagnosis of periprosthetic osteolysis was correctly made and managed by revision surgery. However, in one case angiosarcoma of the ipsilateral hemipelvis went long unrecognized despite aggressive clinical course, requiring hind-quarter amputation and ultimately resulting in the patient's death. Periprosthetic malignancy in the form of either primary sarcoma or metastatic cancer is a very rare yet reported event in the setting of previous hip replacement, likely leading to catastrophic consequences when diagnosis is not established in a timely manner. The differential diagnosis of periprosthetic osteolysis should consider the entire spectrum of conditions that can present with radiolucent changes. Thorough review of patient's history and course of symptoms, along with careful evaluation of standard roentgenograms should be always performed and possibly integrated with imaging modalities such as CT, MRI, and bone scintigraphy in order to increase diagnostic accuracy. If uncertainty remains, biopsy should always be considered to rule out malignancy.