Epithelioid hemangioendothelioma is a rare vascular malignancy, and currently, there is no standard treatment regimen for this disease and existing treatment options have limited efficacy. In this case report, we present a patient with lung and lymph node metastases from prostate epithelioid hemangioendothelioma who achieved a significant partial response. This was accomplished through alternating nivolumab therapy with ipilimumab and liposomal doxorubicin, resulting in a progression-free-survival more than 6 months to date. The treatment was well-tolerated throughout. Our report suggests that dual immunotherapy alternating with anti-PD-1antibody plus doxorubicin may be a potential treatment modality for epithelioid hemangioendothelioma. However, larger sample studies are necessary to ascertain the effectiveness of this treatment strategy and it is essential to continue monitoring this patient to sustain progression-free survival and overall survival.

BACKGROUND: Epithelioid hemangioendothelioma (EHE) is a rare malignancy of vascular origin which can be primarily be seen in various tissues. EHE originating from the pleura is an even more uncommon subtype which may mimic mesothelioma and pleural carcinomatosis. The prognosis of pleural EHE is poor and there is no consensus on the optimal therapeutic approach.
METHODS: A 39-year-old middle-eastern female presented with progressive dyspnea and left shoulder discomfort. Chest computed tomography scan revealed a left side pleural effusion and pleural thickening. Pleuroscopy was done and biopsies were taken which were positive for CD31, CD34, CK, factor 8-R-antigen, and vimentin. Patient was diagnosed with pleural epithelioid hemangioendothelioma (PEHE) and chemotherapy was started and underwent extrapleural pneumonectomy 7 months later. Unfortunately, the patient passed away 10 months after diagnosis due to disease complications.
CONCLUSIONS: Once PEHE is suspected in histology it can be confirmed with immunohistochemistry. Chemotherapy, surgery or a combination of both is currently used as the treatment but the standard treatment remains a question.

BACKGROUND: Pulmonary epithelioid hemangioendothelioma is a rare malignant disease, and most cases are found as multiple lung nodules, rarely as a single nodule.
METHODS: Computed tomography( CT) in a 71-year-old man revealed a growing 3-mm lung nodule in the left S6 after rectal cancer operation. Wedge resection was performed. A pathological examination resulted in a diagnosis of pulmonary epithelioid hemangioendothelioma based on CD31 and CD34 positivity in immunohistochemistry.
CONCLUSIONS: When new nodules are noted on routine CT scans of other malignancies, it is essencial to make a pathological diagnosis, bearing in mind that pulmonary nodules can arise from a variety of causes.

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A physical examination of a 9-month-old female infant presenting with vomiting and diarrhea revealed tenderness in the right upper abdomen and heightened abdominal muscle tone. Abdominal ultrasonography identified an irregular hypoechoic area within the right lobe of the liver. While a subsequent enhanced CT examination disclosed a well-defined lesion exhibiting internal focal calcification and delayed heterogeneous enhancement. Subsequently, she underwent surgical resection, and postoperative pathology revealed areas of epithelioid hemangioendothelioma and cavernous hemangioma. Immunohistochemistry demonstrated positive expression of CD34, CD31, FLI-1, and F-VIII. The pathologic diagnosis was confirmed as composite hemangioendothelioma (CHE).

Epithelioid hemangioendothelioma (EHE) is an uncommon vascular tumor that can present in various organs, including the liver. Hepatic EHE (HEHE) may showcase with metastases at initial presentation, as patients have vague symptoms such as right upper quadrant pain leading to the risk of delayed diagnosis. There is no standard treatment. Fortunately, prognosis is good. This remains true for some patients with metastatic disease who are not being actively treated. In this report, we present a unique case of metastatic HEHE in a 65-year-old Caucasian male who has not received treatment and continues to remain in stable condition after his initial diagnosis three years ago.

Epithelioid emangioendothelioma (EHE) is a rare tumor originating from vascular endothelial cells, which belongs to malignant vascular tumors with low to moderate differentiation and potential metastatic ability and its incidence rate is less than one in a million. We herein report a rare case of EHE of pulmonary origin and a review of the literature concerning the clinical and pathological features of this disease. The patient underwent left upper lobectomy to completely extirpate the lesion by video-assisted thoracic surgery. Our findings suggest the difficulty of making a diagnosis before surgery and that more cases need to be reported in order to facilitate the preoperative diagnosis of such a rare tumor.

Primary adrenal angiosarcomas are exceedingly rare with a rapidly progressive clinical course and a poor outcome. Establishing the diagnosis can be challenging, and it is complicated by the fact that there are no characteristic clinical or imaging features that are pathognomonic for angiosarcoma. Histologically, they can overlap with other more commonly encountered adrenal tumors. Herein, we present an otherwise healthy 41-year-old woman diagnosed with a primary adrenal epithelioid angiosarcoma. We aim to expand the knowledge of the sparse literature existing on primary adrenal angiosarcomas to help better understand the diagnostic features, clinical behavior, and management of these rare tumors.

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Epithelioid and spindle cell sarcomas with NR1D1::MAML1/2 gene fusions are rare and emerging entities. Only six cases of NR1D1-rearranged mesenchymal tumors have previously been reported in the literature; they are often characterized by an epithelioid morphology, at least focal pseudogland formation, prominent cytoplasmic vacuoles, and focal to diffuse immunohistochemical expression of keratin. We herein report the first case of an NR1D1::MAML1 epithelioid and spindle cell sarcoma with dual immunohistochemical expression of ERG and FOSB, mimicking a pseudomyogenic hemangioendothelioma (PHE) on core biopsy. The sarcoma arose in the left forearm of a 64-year-old man. Initial biopsy showed a mesenchymal neoplasm composed of epithelioid and spindle cells dispersed in myxoid stroma with scattered stromal neutrophils. The morphologic features, combined with the dual immunohistochemical expression of ERG and FOSB, initially mimicked PHE, representing an important potential diagnostic pitfall. The patient subsequently underwent a radical resection, which showed a much more diffuse epithelioid appearance with nested architecture and pseudogland formation. Next-generation sequencing was performed on the resection specimen, which revealed an NR1D1::MAML1 gene fusion, confirming the final diagnosis. Given the fully malignant potential of this tumor, knowledge and recognition of this rare entity are essential to ensure proper management, prevent misdiagnosis, and further characterize the clinical course of this emerging entity. Comprehensive molecular testing can help to identify these rare tumors and exclude the possibility of epithelioid mimics, including PHE.