Delayed metastatic pulmonary epithelioid hemangioendothelioma of the femoral vessels: case report and literature review.

Background: Epithelioid hemangioendothelioma (EHE) is a rare sarcoma of the blood vessels. We report a patient with vascular EHE with delayed pulmonary metastasis, of which there are no previously known case reports. Case Description: A 40-year-old female presents with a painful right groin mass and swelling in the lower extremity. A 3.8 cm soft tissue mass was identified in the femoral sheath with the abutment of the femoral artery on a computerized tomography (CT) scan. Surgical resection of the femoral vein was performed, and the final pathology confirmed a diagnosis of EHE. A second en-bloc resection of the femoral artery with bypass grafting was performed with clear surgical margins. The patient did well post-operatively with annual surveillance of the right groin as well as chest X-rays. However, the patient developed a metastatic Epithelioid hemangioendothelioma (EHE) to the right lower lobe, 12 years after the initial EHE treatment. She underwent a pulmonary resection with clear margins. The patient remains disease-free after one year. EHE is a rare soft tissue sarcoma with unpredictable clinical behavior. While most commonly presenting in the lung and liver they can also originate from any vascular system. Delayed pulmonary metastasis from vascular EHE has not been reported. Conclusions: Our case shows that indolent metastasis can occur in EHE, despite a prolonged disease-free interval. This case highlights the need for long-term surveillance with serial imaging of not only the primary site but pulmonary imaging beyond 5 years may be beneficial.

Successful surgical treatment of epithelioid hemangioendothelioma involving multiple liver lesions and bilateral lung nodules.

Epithelioid hemangioendothelioma (EHE) affects many organs, particularly lung and liver, and typically presents as multiple lesions. Treatment for EHE is not yet standardized, but surgery is appropriate when lesions are resectable. In our patient, radiography revealed multiple bilateral pulmonary nodules, and CT showed several liver tumors. The liver masses and those in the right lung were removed during the initial surgery; pathology of hepatic specimens confirmed the diagnosis of EHE. During the second operation, the left lung nodules were excised, and all were EHEs. Surgical removal of multiorgan multinodular EHE is a viable treatment option, especially for young patients.

Indications and Limitations of Sirolimus in the Treatment of Vascular Anomalies-Insights From a Retrospective Case Series.

Background: Despite recent developments, the role of sirolimus in the heterogeneous spectrum of vascular anomalies is yet to be defined, in terms of indication, dosage, and therapy duration, recognizing both its potential and limitations. Methods: We retrospectively analyzed 16 children with vascular anomalies treated with sirolimus in two pediatric centers between 2014 and 2020 [male: n = 7, the median age at diagnosis: 4.6 months (range, 0-281.4)]. In addition, repetitive volumetric analyses of the vascular anomalies were performed when possible (11 cases). Results: Ten patients were diagnosed with vascular malformations and 6 with vascular tumors. The mean therapy duration was 27.2 months (range, 3.5-65). The mean sirolimus level was 8.52 ng/ml (range, 5.38-12.88). All patients except one with central conducting lymphatic anomaly responded to sirolimus, with the most noticeable volume reduction in the first 4-6 months. Additional administration of vincristine was needed in five patients with kaposiform hemangioendothelioma and yielded a response, even in cases, refractory to sirolimus monotherapy. As a single agent, sirolimus led to impressive improvement in a patient with another vascular tumor-advanced epithelioid hemangioendothelioma. Complicated vascular malformations required long-term sirolimus therapy. Side effects of sirolimus included mucositis and laboratory abnormalities. No major infectious episodes were recorded. An infant with COVID-19, diagnosed while on sirolimus therapy, presented with a mild course. Conclusion: In the current series, we reported limitations of sirolimus as monotherapy, addressing the need to redefine its indications, and explore combination regimens and multimodal treatment strategies. Tools for objective evaluation of response trends over time could serve as a basis for the establishment of future therapeutic algorithms.

Primary epithelioid hemangioendothelioma of the penis: a case report and literature review.

Epithelioid hemangioendothelioma (EHE) is a rare vascular tumour with an intermediate behaviour between benign hemangioma and malignant angiosarcoma. There is scarce data on the penile EHE's management and its natural history, as our knowledge is based on few reported cases with a short follow-up period. We present a case report and conducted a literature review, including 17 cases. The relevance of this case report derives from the need for better clinical characterization of patients with penile EHE and the importance of defining the outcomes. We report the case of a 53-year-old male with a 1-year history of sleep-related painful erections. Imaging techniques showed a well-defined hypoechoic and hypervascular solid nodule on the dorsal aspect of the penis. It was surgically removed, and the histopathological study revealed a low-risk EHE of the penis. Follow-up magnetic resonance imaging (MRI) and computed tomography did not demonstrate local recurrence nor metastases. According to the literature review, most of the patients were in their fifth and sixth decades of life at the time of diagnosis and lesions were usually located in the glans. The most common clinical presentation was as a painful mass. Follow-up period ranged from 2 months to 5 years. Three patients showed systemic metastases, two of which died due to cancer. The conclusions from the literature review are limited by the reduced number of cases and the short follow-up. This case report highlights the importance of understanding the diagnosis and treatment of this type of rare non-squamous malignant tumours of the penis. Penile EHE is a malignant vascular tumour that is very rare in this location. The best treatment is local excision, with re-excision or intraoperative margins assessment. Occasionally, systemic chemotherapy and radiation therapy can be useful. There is consensus on the importance of very strict follow-up of these patients.

Epithelioid hemangioendothelioma of the spine: case report and review of the literature.

Epithelioid hemangioendothelioma (EHE) has been described as a rare vascular bone lesion with histological features between hemangioma and high-grade angiosarcoma. Spinal EHE is a quite rare disease with few case reports and series reported in the literature. The tumor cells are positive for vimentin, CD31and CD34, factor VIII related antigen, ERG, and FLI1. Radiological features are not specific; it may appear as an osteolytic lesion. It can present as a multifocal disease in 40% of cases. No clear correlation with age and sex; however, it is slightly more common in males. Focal neck or back pain is the most common presenting symptom. The natural history of spinal EHE is unpredictable, and currently, there is no standard of care for treatment. Treatment options include preoperative embolization, and surgical resection followed by radiotherapy and/or chemotherapy. A 34-year-old previously healthy male presented with mid-thoracic back pain. Magnetic resonance imaging (MRI) of the spine revealed a decrease in vertebral body height at T5 with an enhancing mass. He underwent T5 balloon kyphoplasty and needle vertebral body biopsy. Results of the biopsy samples were non-diagnostic. Approximately 3 months after surgery, the patient presented with unsteady gait. A subsequent MRI revealed progression of the T5 compression fracture with cord compression. The patient subsequently underwent T4-T6 bilateral posterior decompression for epidural tumor and T3-T7 posterior instrumentation with pedicle screws. Pathology of the lesion revealed EHE. The patient was started on local radiation therapy (RT). On follow-up, 3 months after the second surgery, the thoracic spinal pain had improved dramatically. Our review highlights the diagnosis, clinical presentation, and treatment of spinal EHE. Complete resection is associated with good outcome. Radiotherapy has been used in partially resected lesions. However, the role of radiotherapy as primary treatment is not yet defined. Further studies should develop a treatment algorithm for this rare tumour.

Other vascular tumors.

Vascular tumors are rare in children and adults. Classification of these tumors has been difficult, especially in the pediatric population, due to the rarity of these lesions, the unusual morphologic appearance, their diverse clinical behavior, and no independent stratification for pediatric tumors. In 2013, The World Health Organization updated the classification of soft tissue vascular tumors. Pediatric tumors were not independently stratified and the terminology was mostly left unchanged, but the intermediate category of tumors was divided into locally aggressive and rarely metastasizing. These tumors are treated with multimodality therapy and therefore need the guidance of an interdisciplinary team for best care.

Epithelioid hemangioendothelioma in parotid gland misdiagnosed as parotid gland cyst with hemorrhage in 1 case / 实用口腔医学杂志

The report presents a case of epithelioid hemangioendothelioma in parotid gland misdiagnosed as parotid gland cyst with hemor-rhage.Based on the literature review,clinical characteristics,diagnosis and treatment of the disease are discussed.