This study aims to provide a comprehensive understanding of primary hepatic angiosarcoma, a rare and aggressive malignancy, focusing on its diagnostic challenges and unique imaging characteristics. The objective is to delineate the distinctive features of angiosarcoma through computed tomography and magnetic resonance imaging modalities, contributing to improved diagnostic precision and adding valuable insights to the scientific literature. We present the case of a 25-year-old male with primary hepatic angiosarcoma, emphasizing the challenges in distinguishing it from other vascular tumors.

Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor that can originate in various parenchymatous organs, soft tissue, and bone. Extrahepatic involvement is exceedingly rare. In this case, multifocal disease in the spleen and bone was present. Bone lesions showed a target appearance. Splenic lesions showed delayed enhancement of solid components with persistent rim enhancement. A bone biopsy with CAMTA1 staining confirmed the diagnosis. Teaching point: The presence of multifocal bone and splenic lesions can raise suspicion of EHE when other multifocal diseases are excluded.

We report a 48-year-old male patient with spinal epithelioid hemangioendothelioma in T3 and T4 who began with symptoms of paresthesia in the lower limbs and distal weakness of the right lower limb, back pain, increased limitation in walking, urinary incontinence, and constipation. A safe maximum resection was performed, finding residual disease during the PET/CT scan, so it was decided to treat with radiotherapy, and there was a good response to this treatment. A literature review of epithelioid hemangioendothelioma of the thoracic spine was done which showed a mean age of presentation of 41 years and a male-female ratio of 1:0.53. The main symptom was pain, which was present in 100% of the patients, and wide surgery was performed in 56.8% of the patients, intralesional surgery in 31.8%, and biopsy in 11.4%. A total of 46.6% of patients received radiation therapy, and only 6.6% received chemotherapy. The patients had an average follow-up of 38 months. We recommend that extension studies such as PET/CT scans be performed after surgical resection. This can serve as a follow-up with hemangioendothelioma epithelioma patients about metastatic disease or residual disease that will guide us in giving adjuvant treatments, such as radiotherapy or chemotherapy, for better control of the disease.

Epithelioid hemangioendothelioma (EHE) is an extremely rare vascular tumor, which can pose a diagnostic dilemma. It affects women more than men and is mainly found in the liver, lung, and bone. To date, there are no known predisposing factors. Limited data are available on the management of EHE at metastatic stages. The only optimal treatments to prevent metastatic dissemination are surgical resection and amputation in addition to radiotherapy at early stages. The oncologist in this rare entity plays an important role in the guided and standardized management of this disease, especially for advanced stages. In this article, we report the case of a 74-year-old patient admitted with swelling on the outer aspect of the right calf associated with pain and total functional impairment of the limb. The diagnosis favored a high-risk vascular tumor resembling EHE, confirmed by bone (tibia) and soft tissue biopsy. The patient underwent staging investigations, revealing diffuse metastases to the liver, bones, and lungs. The objective of this article is to advocate for oncological intervention in this entity, particularly in the advanced stages of the disease. Despite its rarity, the advancement of clinical trials and therapeutic recommendations remains crucial for optimal treatment.

BACKGROUND: Primary malignant hepatic vascular tumors with various malignant potentials include epithelioid hemangioendothelioma (EHE) and angiosarcoma (AS), which may overlap pathologically. This study aimed to compare the pathological findings of hepatic EHE with those of AS, in association with patient outcomes.
METHODS: Fifty-nine histologically confirmed patients with 34 EHE and 25 AS were admitted to a tertiary hospital from 2003 to 2020. Their EHE and AS pathological features were compared. Immunohistochemistry for CD31, ERG, CAMTA-1, TFE3, P53, and Ki-67 labeling was performed on paraffin-embedded blocks. Markers, along with histological findings, were analyzed for the purposes of diagnostic and prognostic significance by multivariate analysis.
RESULTS: CAMTA-1 was 91.2% positive in EHE, but negative in AS (p = < 0.001). AS was significantly correlated to an aberrant p53 expression, high Ki-67 labeling, and high mitotic activity, compared to EHE (all, p = < 0.001). EHE can be classified as low grade (LG) and high grade (HG) using the prognostic values of mitotic activity and ki-67 labeling (sensitivity = 1, specificity = 1). Low grade-EHE showed significantly better overall survival than high grade-EHE (p = 0.020).
CONCLUSIONS: Immunohistochemistry for CAMTA-1, P53, and Ki-67 labeling may help distinguish EHE and AS in histologically ambiguous cases, especially small biopsied tissue. Moreover, the combination of mitotic activity and Ki-67 labeling can be a prognostic factor for EHE with various clinical features.

Teaching point: When confronted with multifocal \"metastasis-like\" liver lesions without a known primary tumor, in particular in younger female patients, considering hepatic epithelioid hemangioendothelioma (HEHE) in the differential diagnosis can guide pathological examination and potentially avoid the need for multiple invasive biopsies.

A 5-year-old spayed female Breton dog was referred for a thyroid nodule. A total body CT scan evidenced multifocal hepatic nodules. Cytological liver samples were hemodiluted and non-diagnostic. Following a thyroidectomy, the histology was consistent with a follicular-compact thyroid carcinoma. On laparoscopy, most hepatic lobes had multifocal dark-red nodules that were biopsied for histology. Microscopically, the hepatic parenchyma in the nodules was substituted by blood channels lined by bland spindle cells but adjacent to epithelioid neoplastic cells, single or in clusters, embedded in a moderate amount of edematous collagen matrix. These cells had optically empty cytoplasmic space, occasionally containing erythrocytes (microlumina). Spindle and epithelioid cells expressed membranous-to-cytoplasmic CD31 and FVIII-RA consistent with endothelial origin. Based on morphology and immunolabelling, a hemangioendothelioma with epithelioid differentiation was diagnosed. Lesions in the liver were initially stable, showing progression with time. The dog was alive with no systemic clinical signs 36 months after laparoscopy.

Epithelioid hemangioendothelioma (EHE) is a rare low-grade malignant vascular tumor. It mainly occurs in the liver, lungs, bones, and other parts of the body. Reports of epithelioid hemangioendothelioma in the parotid gland are rare in both domestic and international literature. Here, we present a case report of a parotid gland epithelioid hemangioendothelioma, including its complete clinical course and imaging findings, to improve the diagnosis and treatment of this unusual disease.
UNASSIGNED: The patient, a 75-year-old female, presented with a swelling around the right ear for 2 months and pain for 20 days. Enhanced MRI of the parotid gland revealed a well-defined, round mass with homogeneous signal intensity. The mass showed low signal intensity on T1-weighted imaging, high signal intensity on T2-weighted imaging, nodular low signal intensity within, significant high signal intensity on DWI sequence, low signal intensity on ADC sequence, and heterogeneous enhancement in the arterial phase after intravenous injection of Gd-DTPA. Nodular non-enhancing low signal intensity was observed internally, and slight clearance was seen in the venous phase. The initial diagnosis before surgery was a benign lesion, but after histopathological and immunohistochemical examination, it was confirmed as epithelioid hemangioendothelioma.
UNASSIGNED: Complete tumor resection was performed.
UNASSIGNED: The patient experienced a favorable recovery, with meticulous follow-up conducted for up to 1 year revealing no signs of recurrence or metastasis. Continued patient surveillance is ongoing to substantiate and validate the long-term efficacy of the treatment.
UNASSIGNED: Due to the extreme rarity of parotid gland epithelioid hemangioendothelioma, it often leads to a high misdiagnosis rate. The most common misdiagnosis is salivary gland lymphoma, followed by epithelioid hemangiosarcoma. When the lesion is multifocal, fusiform, with internal necrosis, and shows punctate low signal intensity on T2-weighted imaging, significant enhancement in the arterial phase, particularly with more pronounced peripheral enhancement, and persistent enhancement in the venous and delayed phases, epithelioid hemangioendothelioma should be considered. However, the current clinical diagnosis of epithelioid hemangioendothelioma still primarily relies on immunohistochemical methods.

An 8-year-old boy initially thought to have a penile arteriovenous malformation was later diagnosed with a rare vascular sarcoma, epithelioid hemangioendothelioma (EHE). Despite challenges in diagnosis, he underwent supraselective angioembolization and partial penectomy for oncological clearance. EHE, a low-grade malignancy, requires prompt identification and treatment due to potential systemic involvement.

Epithelioid hemangioendothelioma is a very rare vascular neoplasm, which is often multifocal or metastatic at diagnosis. Most frequently arises in the liver, followed by the lung and bones. The authors present a case of a liver transplant recipient who developed a pattern of hepatic cholestasis associated with the appearance of a proliferative hepatic lesion with infiltrative growth. Histological examination and immunohistochemical study were compatible with the diagnosis of epithelioid hemangioendothelioma. Pulmonary micronodules were detected and lung metastases were hypothesized. Therefore, bronchoscopy was performed, which turned out to be normal, and cytology was negative for neoplastic cells. After a multidisciplinary discussion, liver re-transplantation was decided. After 8 years of follow-up, the patient is clinically stable, with no graft dysfunction, no neoplastic recurrence, and dimensional stability of the pulmonary micronodules. Patients with organ transplant have higher risk of developing carcinoma compared to the general population. The development of cancer is a multifactorial process and little is known about the etiology of epithelioid hemangioendothelioma. No standard treatment strategy has been defined yet, and the natural course of the disease is heterogenous and the individual prognosis unpredictable. Complete surgical resection is offered to patients with unifocal disease, and those with unresectable disease should be evaluated for orthotopic liver transplantation.
O hemangioendotelioma epitelióide é uma neoplasia vascular extremamente rara, muitas vezes multifocal ou metastática ao diagnóstico. O local mais frequente afetado é o fígado, seguido pelo pulmão e ossos. Os autores apresentam o caso de uma doente com antecedentes de transplante hepático que desenvolveu um padrão de colestase associado ao aparecimento de uma lesão hepática proliferativa e de crescimento infiltrativo. O exame histológico e o estudo imuno-histoquímico foram compatíveis com hemangioendotelioma epitelióide. Foram detetados micronódulos pulmonares, tendo sido colocada a hipótese de se tratarem de metástases pulmonares. Assim, foi realizada broncoscopia, que não revelou alterações, estando a citologia negativa para células neoplásicas. Após discussão multidisciplinar, foi decidido o retransplante hepático. Após 8 anos de seguimento, a doente encontra-se clinicamente estável, sem disfunção do enxerto, sem recidiva neoplásica e com estabilidade dimensional dos micronódulos pulmonares. Doentes submetidos a transplante têm maior risco de desenvolver neoplasias em comparação com a população geral. O desenvolvimento da neoplasia é um processo multifatorial, sendo a etiologia do hemangioendotelioma epitelióide ainda pouco compreendida. Não existe uma estratégia terapêutica standard, sendo o curso natural da doença heterogêneo e o prognóstico individual imprevisível. A ressecção cirúrgica é a primeira opção terapêutica nos doentes com doença unifocal, aqueles com doença irressecável devem ser avaliados para transplante hepático.