Pulmonary Epithelioid Hemangioendothelioma: A Case Report

A 45-year-old woman whose x-ray film revealed multiple widespreadbilateral small nodules was diagnosed as having pulmonary tuberculosisor metastatic pulmonary cancer. A conclusive histological diagnosisof pulmonary epithelioid hemangioendothelioma (PEH) was madefollowing an open-lung biopsy. PEH is a progressive diseasethat probably originates from an endothelial cell. However,there is still no effective therapeutic modality for PEH. Thepostoperative course of our patient has been uneventful withno evidence of tumor growth for four and a half years afterinitial x-ray detection of the lesions.     

Activity of Angiogenesis Inhibitors in Metastatic Epithelioid Hemangioendothelioma: A Case Report

This report describes a patient with metastatic epithelioid hemangioendothelioma treated with bevacizumab and nanoparticle albumin-bound paclitaxel.The treatment was well tolerated and led to the stabilization of an aggressive variant of the disease. This case report is the first one that describes the activity of the combination of chemotherapy and bevacizumab in epithelioid hemangioendothelioma.Literature describing the activity of bevacizumab and other agents(thalidomide,lenalidomide,and interferon) believed to possess anti-angiogenic activities is also reviewed.     

Malignant Intracranial Epithelioid Hemangioendothelioma Presumably Originating from the Lung: Case Report

OBJECTIVE AND IMPORTANCE: Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor that presents histological features and biological behavior of low-grade malignancy. The authors report a case of malignant intracranial EHE, in which surgical excisions and additional immuno-chemotherapy were ineffective. Emphasis is placed on the histological features of this rare tumor and its potential for malignancy. CLINICAL PRESENTATION: A 69-year old male presented with paresis of the right arm. Magnetic resonance imaging revealed intracranial multiple lesions in the right temporal and parietal and left frontal lobes. Chest radiography revealed a mass lesion occupying the lower lobe of the left lung that had been followed as old tuberculosis since it had not changed in size for as long as 5 years. INTERVENTION: We performed craniotomy and excised tumors. Histological examination demonstrated that the excised tumors had the features of EHE. Further intracranial recurrence after the excision of the tumors necessitated adjuvant treatment with interferon alpha-2b. However, the patient continued to deteriorate and died 3 months later. Postmortem study disclosed the tumor cells existing in the left lung, pleura, ribs, intercostal tissues and diaphragm. Other organs including liver, bone, and skin were free from tumor invasion, which indicated that the malignant EHE originated from the left lung and metastasized to the brain. CONCLUSION: To the authors knowledge, no case of malignant EHE that simultaneously involves the central nervous system and the lung has been previously reported. An extensive inspection for involvement of other organs is recommended after recognizing an intracranial EHE.     

Tentorial Schwannoma: A Case Report and Review of the Literature

Summary Introduction Schwannomas are most often found in association with the eighth cranial nerve, but may also arise from any other cranial nerve. They are rarely found in an intra-parenchymal location. Unusual locations for intracranial schwannomas have also been reported in association with neurofibromatosis. Clinical presentation A 23-year-old male without von Recklinghausen’s disease presented with intermittent dizziness and difficulty swallowing. Past medical history was significant for a motor vehicle accident (MVA) without loss of consciousness 6 months prior. Magnetic resonance imaging revealed a large tentorial-based tumor. At surgery the origin of the tumor was clearly the tentorium, and while the trigeminal nerve was displaced, it easily separated from the mass. There was no attachment to any other cranial nerve in the immediate vicinity and postoperative cranial nerve examination was unremarkable. Pathological review was consistent with schwannoma. Conclusion While there are few reported cases of tentorial-based schwannoma, these tumors have been noted in unusual locations within the intracranial vault, and clinicians should be aware of this diversity of origin.     

原发性肾脏上皮样血管内皮瘤1例报道及文献复习

[目的]探讨肾脏上皮样血管内皮瘤的诊断、鉴别诊断及治疗。[方法]结合文献对1例原发性肾脏上皮样血管内皮瘤的临床资料、病理特征、免疫表型及治疗预后进行分析。[结果]右肾下极肿物,光镜下肿瘤细胞排列成巢状、短梁状、部分区域灶性实性片状;肿瘤细胞呈圆形、多边形和较少的短梭形,瘤细胞胞浆丰富,粉红色,核呈空泡状,有一个不明显的核仁。肿瘤细胞形成小的细胞内管腔,表现为丰富的胞浆内空泡,偶尔含有红细胞,似原始血管管腔;部分区域几个短梭形细胞围成一个小腔隙,为发育较好的血管管腔。免疫表型:VIM、CD34、CD31血管内皮细胞弥漫阳性。随访11个月无复发。[结论]肾脏上皮样血管内皮瘤属罕见的低度恶性肿瘤,具有特征性的组织学结构,其形态学和免疫表型与发生于肾外者相同。治疗上应局部切除并随访。     

Multifocal Epithelioid Hemangioendothelioma Derived from the Spine Region: Case Report and Literature Review

Background

Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor with malignant biological behavior. It arises from endothelial cells, usually within soft tissues, and can occur in almost all locations.

Case Report

We report a unique case of a 25-year-old man who presented with sudden attacks of severe back pain followed by acute non-traumatic paraplegia. Emergency diagnostics revealed a pathologic fracture of the T7 vertebra with tumor tissue invasion of the spinal canal. Furthermore, multifocal metastases were found.

Results

To achieve en bloc resection, interdisciplinary surgical approaches were indicated. Despite multimodal therapy concepts, including radiotherapy and chemotherapy as well as endovascular embolization, the patient died within 8 weeks.

Conclusion

Prognosis of EHE is unpredictable and mainly determined by its location. The lesions are potentially aggressive; therefore, en bloc resection should be attempted whenever possible. However, as shown in the literature, only 15% of patients are suitable for total resection.Key words: Hemangioendothelioma, Vascular spinal tumor, Bone manifestation, Multifocal metastasis, Interdisciplinarity, Multimodal treatment     

骨上皮样血管内皮瘤2例并文献复习

目的:探讨2例骨上皮样血管内皮瘤（epithelioid hemangioendothelioma,EHE）的临床特点、治疗措施和预后因素。方法:回顾性分析2例原发于骨的EHE临床资料、诊断、治疗以及随访情况,并结合文献进行讨论。结果:2例骨EHE中男性和女性各1例,低度恶性的单发骨EHE患者经过局部病灶的放疗、TE方案联合恩度等综合治疗,目前仍存活;侵袭性多发病灶的骨EHE患者经过TE方案联合恩度系统治疗、局部TOMO等综合治疗,病情进展后死亡。结论:骨EHE的预后主要取决于肿瘤组织学级别和发生的部位;综合治疗可适当延长生存期,规范治疗尚无循证医学依据。     

三维适形放射治疗门脉癌栓的疗效观察

目的 探讨三维适形放射治疗原发性肝癌门脉癌栓的疗效.方法 31例门脉癌栓行三维适形放射治疗,观察患者治疗前后的临床症状和影像学变化来评价三维适形放射治疗的临床疗效.结果 22例门脉癌栓有缩小或消失.结论 三维适形放射治疗是一种有效的和安全的治疗门脉癌栓的方法,为临床治疗门脉癌栓提供了一个新的手段.     

Severe Acute Enteritis in a Multiple Myeloma Patient Receiving Bortezomib and Spinal Radiotherapy: Case Report

Abstract

Proteasome inhibitors have been reported to enhance radiosensitivity in vitro. A case of potential clinical interaction between bortezomib, a proteasome inhibitor, and spine radiation is reported. A woman undergoing palliative radiotherapy to the T12 -S2 spine with concurrent bortezomib developed unexpectedly severe, acute radiation enteritis requiring hospital admission. Clinicians are advised to consider the potential for interactions of bortezomib with radiotherapy when the two agents are used simultaneously in the clinic.     

A Case Report of Rare Vocal Cord Hemangioendothelioma in a Paediatric Larynx

Hemangioendothelioma (HEM) is a vascular tumour which is locally aggressive with a low-grade malignant potential. We present a rare case of HEM arising from the vocal cord of a 10-year-old female child. Video laryngoscopy clearly showed a large vocal cord polyp-like mass attached to middle one-third of left cord. Histopathological examination showed fragments of tissue with a neoplasm composed of spindle cells arranged diffusely admixed with numerous small vascular channels. These specific cells were positive for CD31, CD34, vimentin and ERG. Flexiblescopy and narrow band imaging were performed 3 weeks after surgery which showed no residual mass. Treatment of HEM is hampered by lack of proper guidelines and protocol. In this case of localized vocal cord lesion, a wide excision with regular follow up is considered ideal.     

Suprasellar Melanocytoma: A Case of Primary Radiotherapy and Review of the Literature

Meningeal melanocytoma is a rare benign neoplasm of the central nervous system. Approximately 50% of the tumors are located intracranially with a preference for posterior fossa. We report a new case of intracranial suprasellar melanocytoma in a 57-year-old male patient treated with primary high-dose conformal radiotherapy to 50.4Gy. Fourty-two months subsequent to treatment the tumor is well under control without neither deterioration of clinical symptoms nor progression of gross tumor volume on MR imaging. No late sequelae of treatment have been observed.A review of the literature is presented indicating that resection of melanocytoma is the treatment of first choice hampered by a relapse rate of approximately 30% depending on resection status. The role of adjuvant radiotherapy in patiens with complete resection of melanocytoma has not yet been defined. These patients carry a risk of relapse of approximately 15%, and adjuvant irradiation is currently not recommended. Primary high-dose radiotherapy has been shown to be effective in long-term control of the neoplasm in patients where no resection of the tumor could be accomplished. In case of incomplete resection of melanocytoma, data of the literature indicate that adjuvant radiotherapy may in fact increase long-term local control of the tumor. Requirements for optimal treatment results of radiotherapy are sufficiently high doses of, at least, 50Gy for intracranial lesions and three-dimensional treatment planning guaranting precise targeting of the tumor volume with only a low-risk of late sequalae to the surrounding tissues.     

Huge Osteoclastoma of Hyoid Bone: A Case Report

Giant cell tumors (GCT) of hyoid bone are very rare, and only few individual cases were reported in literature. We present such a case of GCT arising from left cornu of hyoid bone. The patient underwent trans-cervical complete excision of the tumour. Postoperatively, patient did well without recurrence or metastasis for 2 years follow up.     

食管癌术后颈部和纵隔淋巴结转移放疗的临床观察

目的　探讨食管癌术后颈部和纵隔淋巴结转移放疗的意义和效果。方法　回顾性分析 5 6例食管癌根治术后发生颈部、纵隔转移患者。放射治疗采用60 Co常规分割照射 ,大多数患者的放射剂量为 5 0～ 70Gy。结果　全组病人 1、2、3、5年存活率分别为41.1% ( 2 3 /5 6)、17.9% ( 10 /5 6)、7.1% ( 4 /5 6)、2 .1% ( 1/4 8)。结论　放疗对食管癌术后颈部和纵隔淋巴结转移的局部控制率有效 ,且可延长患者的生存时间。     

Ruptured Hepatic Epithelioid Angiomyolipoma: A Case Report and Literature Review

A 38-year-old male was admitted to our hospital due to upper abdominal pain. Computed tomography revealed a hepatic angiomyolipoma (AML; 10.5 × 9.5 × 7.0 cm in size), which had ruptured into the space between the liver and the diaphragm. Following transcatheter arterial embolization, surgical resection was performed. The tumor consisted of epithelioid cells (50–60%), mature fat (40–50%), and thickened-wall blood vessels. Considering the amount of epithelioid cells and their positivity for E-cadherin and β-catenin, the tumor was diagnosed as hepatic epithelioid AML. Cases of ruptured hepatic AML are rare. To the best of our knowledge, this is the sixth case reported in the English literature.Key words: Hepatic epithelioid angiomyolipoma, Rupture, Transcatheter arterial embolization, Surgical resection     

A Primary Epithelioid Angiosarcoma Arising in a Bilharzial Urinary Bladder: A Reappraisal and Case Report

Background: Angiosarcoma (AS) of the urinary bladder is a very rare and aggressive malignancy with a dismal outcome. Case report: Here, we report a primary epithelioid angiosarcoma (EAS) of the urinary bladder in a forty-nine-year-old male patient who presented with severe hematuria. Cystoscopic examination revealed hemorrhagic ulcerated bladder mucosa but no definite mass lesions. Intractable hematuria raised the initial clinical impression of idiopathic hemorrhagic cystitis. Analysis of the cystoscopic biopsy revealed features of old bilharzial cystitis, markedly atypical epithelioid endothelial cells arranged as primitive anastomosing vascular structures and expressing vascular markers. The diagnosis of EAS was established. The patient developed intractable severe hematuria, and a radical cystoprostatectomy was performed. The patient was started on chemotherapy but suddenly developed widespread distant metastasis (liver, lung, suprarenal glands, and lymph nodes) and succumbed to death two months after the surgery. Conclusion: To the best of these authors’ knowledge, we presented the first report of primary EAS arising in a bilharzial bladder. The relevant studies were discussed.     

Kaposiform Hemangioendothelioma： Case Report and Literature Review

Kaposiform hemangioendothelioma (KHE) is a rare locally ag- gressive vascular tumor that mainly occurs in children and early adolescents. It is often associated with the Kasabach-Meritt     

51例血管瘤放射治疗临床分析

目的　评价放射治疗在血管瘤临床治疗中的价值。方法　回顾性分析应用深部X线对 5 1例血管瘤患者实施放射治疗 ,少部分 (3 / 5 1例 ,占 5 9% )未控复发病例同时应用血管硬化剂鱼肝油酸钠。结果　本组血管瘤放疗治愈率、好转率分别为 86 3 % (4 4 /5 1)和 11 8% (6/ 5 1) ,总有效率 98 0 %。结论　放射治疗在血管瘤临床治疗方面占有重要的地位 ,方法简便易行 ,痛苦小 ,费用低廉 ,具有良好的临床价值     

Gemcitabine and Radiotherapy in the Treatment of Brain Metastases from Transitional Cell Carcinoma of the Bladder: A Case Report

Hematogenous metastases occur in over 50% of muscle-invasive transitional cell carcinomas (TCC) of the bladder. Despite treatment (mostly surgery and radiotherapy), patients with brain metastases have an especially poor prognosis (median survival 2–5 months), making palliative treatment an important consideration. We followed a 60-year-old man with multiple brain metastases who was ultimately treated with gemcitabine chemotherapy. He underwent a cystectomy in 1997 because of a T3a N0 M0 G3 TCC of the bladder. Two years later, he developed one brain metastasis and one lung metastasis. Both metastases were resected and adjuvant chemotherapy was planned. Before chemotherapy, the patient suffered from headaches and symptoms of hemiparesis. A magnetic resonance imaging (MRI) showed multiple brain metastases of up to 2cm, particularly in the brain stem. The patient underwent whole-brain radiotherapy with 30Gy, followed by four cycles of a 3-week gemcitabine (800mg/m² on days 1 and 8) schedule. Another MRI showed a nearly complete response after four cycles of chemotherapy, with only small residual tumors remaining in the brain stem. This impressive activity was accomplished without adverse side effects, suggesting that radiotherapy combined with gemcitabine monotherapy may be an excellent choice for palliative treatment of TCC of the bladder.     

Radiotherapy for Lowly Malignant Cranial Inflammatory Myofibroblastic Tumor Accompanied with Intracranial Invasion: Case Report and Literature Review

Inflammatory myofibroblastic tumor(IMT) is rare in clinical practice.As its treatment mainly involves surgery,radiotherapy alone is seldom reported in literature.Here we report a case of lowly malignant cranial IMT with intracranial invasion in a female patient. As surgery was not suitable,intensity modulated radiation therapy(IMRT) was administered.After radiotherapy,the cranial lesions tended to show efficacy.     

Hemangioendothelioma of the mandible in a newborn infant - diagnostic and treatment approach of a rare vascular tumor

Infantile hemangioendotheliomas are rare vascular tumors with predominant involvement of the soft tissues of the extremities. The occurence in the head and neck region and in particular intraoral occurence is extremely rare. The tumor is believed to be a neoplasm of borderline malignancy. We report a case of a 5-months old infant presenting with a hemangioendothelioma with extensive bony destruction of the mandible. Following surgical resection of the tumor with segmental mandibular resection primary mandibular reconstruction was performed using a rib graft being a useful compromise solution for primary mandibular reconstruction in young infants.