The Pleural Origin of Retiform Hemangioendothelioma: An Unusual Origin of a Rare Diagnosis.

Introduction: Vascular malignancies are categorized into benign hemangiomas, malignant angiosarcomas, and tumors of intermediate malignancy. Retiform hemangioendothelioma (RH) is one of the rare diagnoses belonging to the class of intermediate malignancies that was initially diagnosed. Case Presentation: In this case report, we have reported a rare case of RH, a locally aggressive, vascular tumor of malignant potential presenting at an unusual site. The most observed locations of this soft tissue tumor are skin and subcutaneous tissues of extremities; however, this case of RH was observed in an unusual site called pleura, which is a rare occurrence related to this tumor. The presence of cell surface markers like CD31 and ERG of vascular and endothelial origin and the presence of hobnail appearance of endothelial channels on the biopsy profile supported the vascular origin of the tumor; the clinical features and imaging studies further confirmed the diagnosis of RH. Conclusion: In this case report, a rare case of RH with an unusual site of origin was highlighted. RH is a vascular neoplasm; commonly observed sites of this vascular tumor were skin and subcutaneous tissue of the extremities; however, in this case, the site was unusually different, which was the pleura of the left lung.

Challenges and Management of Capillary Intraosseous Hemangioma in the Mandibular Symphysis: A Case Report.

Capillary intraosseous hemangioma is a benign vascular neoplasm that affects bone tissue, yet its occurrence in the jaw bones has been seldom reported in the literature. We present a case of a capillary intraosseous hemangioma located in the mandibular symphysis of a 28-year-old male. Initially addressed by the patient's dentist as an infectious lesion of endodontic origin, the sudden worsening of the condition, marked by the development of a rapidly expanding exophytic mandibular lesion and tooth mobility, led to the consideration of various potential diagnoses. Subsequently, an incisional biopsy was performed, triggering multiple episodes of recurrent bleeding, leading to several visits to the emergency department, and prompting an urgent status upgrade for the patient. Upon the histological diagnosis of vascular neoplasm, the patient underwent the excision of the lesion, with a favorable and uneventful evolution, although with expected sequelae. As a result, a temporary prosthetic solution, comprising a Maryland Bridge, was implemented, with plans for guided bone regeneration and implant-supported fixed dental prostheses currently in progress. This case underscores the diagnostic and therapeutic challenges associated with this rare condition. Consequently, achieving the optimal outcome for the patient largely depends on a multidisciplinary approach, emphasizing the critical importance of thorough preoperative assessment, along with a well-devised treatment plan and rapid intervention.

Proposed diagnostic and prognostic markers of primary malignant hepatic vascular neoplasms.

INTRODUCTION: 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.

Dural composite hemangioendothelioma: The first intracranial case.

Background: Composite hemangioendothelioma (CHE) is a rare, locally aggressive neoplasm of intermediate malignant potential. It is composed of a mixture of vascular tumors with a predilection for the dermis and subcutis of the extremities. Case Description: In this report, we describe a 41-year-old man who presented with a 2-month history of headache, dizziness, and intermittent seizures. Magnetic resonance imaging showed a hemorrhagic, multilobulated, and dural-based mass with extension into the calvarium. The mass measured 10.3 × 4.8 × 4 cm along the interhemispheric fissure and encased the superior sagittal sinus. Excision was performed, and histopathologic examination revealed a heterogeneous mixture of vascular components consisting of epithelioid hemangioendothelioma, retiform hemangioendothelioma, and hemangioma. This is the first report of a primary intracranial CHE. Conclusion: The spectrum of mesenchymal neoplasms within the cranium expands to encompass CHE.

Myeloid sarcoma with RBM15::MRTFA (MKL1) mimicking vascular neoplasm.

Extramedullary involvement of acute myeloid leukemia (AML), aka myeloid sarcoma, is a rare phenomenon in acute megakaryoblastic leukemia with RBM15:: MRTFA(MKL1) fusion, which might mimic non-hematologic malignancies. A 7-month-old infant presented with leukocytosis, hepatosplenomegaly, multiple lymphadenopathies, and a solid mass in the right thigh. Initially, the patient was diagnosed with a malignant vascular tumor regarding the expression of vascular markers from the biopsy of the right thigh lesion that was performed after the inconclusive bone marrow biopsy. The second bone marrow biopsy, which was performed due to the partial response to sarcoma treatment, showed hypercellular bone marrow with CD34 and CD61-positive spindle cell infiltration and > 20% basophilic blasts with cytoplasmic blebs. RNA sequencing of soft tissue biopsy revealed the presence of RBM15::MRTFA(MKL1) fusion. Based on these findings, myeloid sarcoma/AML with RBM15::MRTFA(MKL1) fusion diagnosis was made. AML with RBM15::MRTFA(MKL1) fusion can initially present as extramedullary lesions and might cause misdiagnosis of non-hematologic malignancies.

Angiomatosis Occurring in an Uncommon Location and Age Group: A Case Report and Literature Review.

Angiomatosis is a rare benign vascular malformation. The lesion has a highly infiltrative nature and a high recurrence rate, making it easily misdiagnosed as malignancy. Therefore, diagnosis is best made based on a combination of clinical, radiological, and histological features. This case presentation is unique because aside from the fact that the lesion is rare, it was seen in an uncommon age and location. Occurrence in such location has not been documented, making this presentation the first of its kind. A 29-year-old male presented with a swelling on the right side of the back, reportedly present for the past six years. The swelling was painless, with a history of progressive increase in size. Examination at presentation revealed a fairly round mass located on the back, 5 cm below the right scapula. He underwent a wide local surgical excision with the material sent for histopathological evaluation. Based on the morphologic features, a definitive angiomatosis diagnosis was made. Our patient had complete surgical excision with histologically confirmed free margins and no recurrence after eight months of post-operative follow-up.

Cine magnetic resonance imaging in evaluating retroperitoneal leiomyosarcoma arising from the inferior vena cava.

Introduction: Leiomyosarcoma of the inferior vena cava is associated with poor prognosis. Complete resection is the only curative treatment. We present a patient with this disease in whom cine magnetic resonance imaging was valuable in selecting the surgical strategy and mitigating invasiveness. Case presentation: A 68-year-old woman presented with right-sided abdominal pain. Computed tomography revealed an 86 mm tumor in the right retroperitoneal space that extended into the inferior vena cava and reached superiorly to the right atrium. Percutaneous needle biopsy confirmed leiomyosarcoma. Cine magnetic resonance imaging demonstrated no adhesions between the tumor and the upper segment of inferior vena cava wall, nor with the right atrial wall, indicating resectability. Radical tumor resection was successfully performed without requiring thoracotomy. Conclusion: Cine magnetic resonance imaging appears to be useful in inferior vena cava leiomyosarcoma for evaluating adhesions between the tumor and vessel wall.

Corrigendum: Case Report: Surgical treatment of a primary giant epithelioid hemangioendothelioma of the spine with total en-bloc spondylectomy.

[This corrects the article DOI: 10.3389/fonc.2023.1109643.].

Sclerosing angiomatoid nodular transformation of the spleen in a child with anemia: a case report and review of the literature.

BACKGROUND: Sclerosing angiomatoid nodular transformation of the spleen is a relatively rare benign vascular lesion in both adult and pediatric age groups with unclear etiopathogenesis and variable clinical presentations. Many benign and also malignant splenic masses could mimic sclerosing angiomatoid nodular transformation, both clinically and radiologically. Herein, we report our experience with a case of sclerosing angiomatoid nodular transformation in a 3-year-old girl. CASE REPORT: A 3-year-old Iranian girl presented with abdominal pain, back pain, and constipation for 2 weeks. She was being followed up by a pediatrician due to her short stature and persistent anemia. Physical examination showed stable vital signs, short stature, pallor, and a puffy face. Laboratory evaluation showed normochromic normocytic anemia with a normal reticulocyte count, ferritin, and hemoglobin electrophoresis. Radiologic assessments revealed a hypoechoic lesion in the spleen with high vascularity, clinically suspected to be lymphoma. She was operated on, and after partial splenectomy, pathologic evaluation of the spleen showed a solitary, well-demarcated, and unencapsulated dark mass. Microscopic examination revealed micronodular appearance composed of irregular-shaped vascular spaces lined by plump endothelial cells and surrounded by concentric collagen fibers, features in keeping with sclerosing angiomatoid nodular transformation. The patient's anemia was resolved after surgery, and no clinical or radiologic deficits were noted during the 10-month follow-up visits. CONCLUSION: Although sclerosing angiomatoid nodular transformation is exceedingly rare in children, it should be considered a differential diagnosis in pediatric splenic neoplasms with concurrent hematologic manifestations, such as anemia.

Current concepts of vascular anomalies.

Vascular anomalies encompass a variety of malformations and tumors that can result in severe morbidity and mortality in both adults and children. Advances have been made in the classification and diagnosis of these anomalies, with the International Society for the Study of Vascular Anomalies establishing a widely recognized classification system. In recent years, notable progress has been made in genetic testing and imaging techniques, enhancing our ability to diagnose these conditions. The increasing sophistication of genetic testing has facilitated the identification of specific genetic mutations that help treatment decisions. Furthermore, imaging techniques such as magnetic resonance imaging and computed tomography have greatly improved our capacity to visualize and detect vascular abnormalities, enabling more accurate diagnoses. When considering reconstructive surgery for facial vascular anomalies, it is important to consider both functional and cosmetic results of the procedure. Therefore, a comprehensive multidisciplinary approach involving specialists from dermatology, radiology, and genetics is often required to ensure effective management of these conditions. Overall, the treatment approach for facial vascular anomalies depends on the type, size, location, and severity of the anomaly. A thorough evaluation by a team of specialists can determine the most appropriate and effective treatment plan.

Hepatic Small Vessel Neoplasm: A Case Report and Review of the Literature.

Hepatic small vessel neoplasm (HSVN) is a recently described vascular neoplasm of the liver. It demonstrates an infiltrative growth pattern and lacks cytologic atypia and mitotic activity. So far, no cases of metastasis or disease recurrence after excision have been reported in the literature. In this report, we present the case of a 31-year-old woman with a lesion in segments VII-VIII of the liver who was referred to our surgical department due to right lumbar pain. She underwent an atypical wedge hepatectomy (segments VII, VIII) and cholecystectomy. The histopathology of the resected specimen confirmed a 40mm HSVN. The patient did not receive any adjuvant therapy and is scheduled for follow-up with serial magnetic resonance imaging (MRI) scans over the next five years due to the unknown malignant potential of the tumor.

Hepatic small vessel neoplasm: not totally benign, not yet malignant.

Hepatic small vessel neoplasm (HSVN) is a rare vascular tumor with few reports in the literature. While imaging findings may show characteristic enhancement patterns, limited available literature may not reveal the full potential for image-based diagnosis. Histologically, HSVN mimics other entities, though certain morphologic and immunohistochemical findings provide clues for diagnosis. However, HSVN still provides diagnostic challenges, especially on core biopsies with limited material for morphologic and molecular evaluation. While current recommendations are surgical resection and close observation, the long-term course of the tumor is unknown. We report a case of HSVN in a liver with additional feature of organized lymphoid aggregates necessitating additional hematopathology consultation and workup to rule out concurrent entities.

[Multiple, eruptive epithelioid hemangiomas of the scalp]. / Multiple, eruptive epitheloide Hämangiome der Kopfhaut.

Epithelioid hemangioma is a benign vascular neoplasm with a characteristic histological and immunohistochemical pattern, characterized by a lymphocytic inflammatory infiltrate with admixed eosinophils and FOS­B expression. The correct diagnosis is of particular relevance, since malignant vascular tumors with differentiated epithelioid cells can also be considered in the differential diagnosis. We present a patient with multiple epithelioid hemangiomas of the scalp accompanied by severe pain and itching. The long history of multiple therapeutic attempts illustrates the limited success of currently available treatment options.

Kaposiform hemangioendothelioma of skull base with dura invasion in a pediatric patient: a case report.

Kaposiform hemangioendothelioma is an extremely rare vascular tumor which shows aggressive local growth. We present a case of rapid growing vascular skull tumor with dura invasion in a pediatric patient with neurofibromatosis type 1. A 14-year-old male complained of headache and dizziness for 1 month after minor head trauma. Brain magnetic resonance imaging (MRI) revealed a 5-cm-sized tumor in the left frontotemporal bone with internal hemorrhage and cystic changes. The gross total resection of tumor was done. At the 7-month follow-up, brain MRI revealed a recurrent skull tumor with intracranial dura mass. He underwent second surgery, and the pathologic diagnosis was suggestive of Kaposiform hemangioendothelioma. For this vascular proliferative tumor, mTOR inhibitor was treated for 6 months, and there was the recurred nodular-enhancing mass along the sphenoid ridge. After additional 2 months of medication, the following MRI revealed a decreased nodular-enhancing mass.

Patient with composite haemangioendothelioma containing angiosarcoma-like areas in the setting of congenital lymphoedema mimicking Stewart-Treves syndrome: a case report.

BACKGROUND: Composite haemangioendothelioma is a rare vascular neoplasm with indolent to intermediate malignant potential. Diagnosis of this disease relays on histopathological identification of at least two different morphologically distinctive vascular components in proper clinical settings. Exceedingly rare cases of this neoplasm can exhibit areas resembling high-grade angiosarcoma, which does not change the biological behaviour. Such lesions tend to occur in the setting of chronic lymphoedema and thus, can mimic Stewart-Treves syndrome, which has a much worse clinical outcome and prognosis. CASE PRESENTATION: We present a case of 49 years old male suffering from chronic lymphoedema of the left lower extremity who had developed a composite haemangioendothelioma with high grade angiosarcoma-like areas mimicking the Stewart-Treves syndrome. Given the multifocality of the disease, the only potentially curable surgical treatment would be hemipelvectomy, which was refused by the patient. The patient has been followed-up, with no signs of local progression of the remaining disease, nor a distant spread outside the involved extremity for two years. CONCLUSIONS: Composite haemangioendothelioma represents a rare malignant vascular tumour, with significantly more favourable biological behaviour than angiosarcoma, even in cases where angiosarcoma-like areas are present. For that reason, composite haemangioendothelioma can be easily misdiagnosed as true angiosarcoma. The rarity of this disease unfortunately hampers the development of clinical practice guidelines and the implementation of treatment recommendations. Most of the patients with localized tumour are treated by wide surgical resection, without neo- or adjuvant radiotherapy or chemotherapy. However, in the case of this diagnosis, the watch-and-wait approach is better than mutilating procedure, highlighting the necessity of establishing of the correct diagnosis.

Mandibular Arteriovenous Malformation (Vascular Lesion) in a 16-Year-Old Patient.

Intraosseous arteriovenous malformations in jaws are rare congenital vascular abnormalities that the dentist may encounter. A vascular lesion or disease should be suspected when there is unexplained bleeding from the oral cavity. Diagnostic imaging is a valuable tool in diagnosing and localizing vascular lesions. An understanding of some of the salient clinical and radiographic features of arteriovenous malformations in jaws aids the clinician in correctly diagnosing this condition and avoids iatrogenic injuries, such as hasty tooth extraction, which may potentially cause severe bleeding and possibly death. The dentist should acknowledge their expertise/limitation, and know when the need for referral arises.

Case report: Surgical treatment of a primary giant epithelioid hemangioendothelioma of the spine with total en-bloc spondylectomy.

Background: Epithelioid hemangioendothelioma (EHE) is an extremely uncommon malignant neoplasm that originates from vascular endothelial or pre-endothelial cells. In this report, we present the case of patient who was diagnosed with a primary giant EHE of the spine and underwent treatment with total en-bloc spondylectomy (TES). Case presentation: A 43-year-old male patient with a history of he presented to our hospital with chronic and progressive back pain. Physical examination revealed weakened sensation of acupuncture and touch on the left costal arch, while relatively normal neurological functions were preserved. Radiological examinations identified a giant destructive soft tissue lesion occupying the T8 vertebral region, with moderate destruction of the pedicle and lamina, as well as the 7th left rib. A preoperative biopsy of the 8th vertebra resulted in a diagnosis of epithelioid hemangioendothelioma(EHE). Postoperative immunohistochemical and pathological reports confirmed the presence of EHE in the left ribs and T8 ribs. The patient underwent resection of the 7th left rib and posterior pedicle screw fixation with 8 pairs of screws and a titanium mesh cage. Subsequently, thoracic en bloc spondylectomy was performed on the T8 vertebra. The patient did not receive radiation or chemotherapy following surgery. Over a period of 3 years, the patient remained free of disease and relapse. Conclusion: The use of transarterial embolization with spherical embolic agents (TES) has been demonstrated to be a safe, effective, and reliable treatment option for hepatic epithelioid hemangioendothelioma (EHE). Nevertheless, it is crucial to conduct long-term follow-up of this patient in order to assess their clinical outcome.

Primary Breast Angiosarcoma: Comparative Transcriptome Analysis.

Primary breast angiosarcoma, with de novo appearance and not associated with exposure to radiation or lymphedema, is a rare pathology representing less than 0.05% of the neoplasms related to this organ. The pathology is characterized by its aggressiveness, poor prognosis, and difficulties in its differential diagnosis. This article reports the case of a 55-year-old white woman with no family history of cancer, with a rapidly growing mass in the left mammary gland that ulcerates and bleeds. It is confirmed as primary breast angiosarcoma by immunostaining in the tumor tissue for CD31, CD34, and FLI-1. In addition, a sample of neoplastic and healthy tissues is collected from the patient for RNA sequencing; the results are contrasted with a tissue sample from a patient with Luminal A subtype of breast cancer, as well as data from other cases of angiosarcoma available in public databases. These findings revealed a genetic profile associated with the immune and inflammatory response in the patient's sample when compared to available angiosarcoma data; these molecular patterns are consistent with other recent studies. Due to the rarity of the disease, the studies carried out on each patient contribute to the expanding knowledge of the etiology and molecular pathways that are still partially known and continue to be the subject of research. Aside from a comparative transcriptome study, this article aims to provide an update on the state of knowledge about this disease.