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A 20-year-old, sexually intact male Dutch Warmblood horse was presented with a 5-day history of difficulty moving and nonflexion of the right pelvic limb, followed by subcutaneous swelling and permanent lateral decubitus. Despite attempts at palliative care, the horse was euthanized after clinical worsening and lack of treatment response. The main gross finding was a soft, red-to-black, lobulated mass involving the right semitendinosus, semimembranosus, and biceps femoris muscles. Soft, red nodules were also seen in all pulmonary lobes and adrenal glands. Histology revealed neoplastic proliferation of malignant polygonal cells, occasionally arranged in irregular anastomosing vascular channels filled with erythrocytes. Neoplastic cells were immunoreactive for factor VIII–related antigen, confirming the blood vascular origin. A final diagnosis of epithelioid hemangiosarcoma was made. These findings reinforced the inclusion of hemangiosarcoma as a differential diagnosis of equine muscular lesions and highlighted the histological features of the epithelioid hemangiosarcoma subtype. [ABSTRACT FROM AUTHOR]

ABSTRACT: A 20-year-old, sexually intact male Dutch Warmblood horse was presented with a 5-day history of difficulty moving and non-flexion of the right pelvic limb, followed by subcutaneous swelling and permanent lateral decubitus. Despite attempts at palliative care, the horse was euthanized after clinical worsening and lack of treatment response. The main gross finding was a soft, red-to-black, lobulated mass involving the right semitendinosus, semimembranosus, and biceps femoris muscles. Soft, red nodules were also seen in all pulmonary lobes and adrenal glands. Histology revealed neoplastic proliferation of malignant polygonal cells, occasionally arranged in irregular anastomosing vascular channels filled with erythrocytes. Neoplastic cells were immunoreactive for factor VIII-related antigen, confirming the blood vascular origin. A final diagnosis of epithelioid hemangiosarcoma was made. These findings reinforced the inclusion of hemangiosarcoma as a differential diagnosis of equine muscular lesions and highlighted the histological features of the epithelioid hemangiosarcoma subtype.

Background: Intravenous lobular capillary hemangioma (IVLCH) of the neck is a kind of rare benign tumor of vein. Purpose: In this paper, we report two female patients who were hospitalized because of neck masses. Results: The tumors in the neck veins of our patients were white oval masses with pedicle, clearly defined and of different sizes. Their immunohistochemical staining results showed CD31 (+), CD34 (+), SMA (+), ERG (+). The pathological diagnosis was intravenous lobular capillary hemangioma. Conclusions: Due to the location, morphology and immunohistochemistry, This lesion needs to be distinguished from other intravascular lesions such as thrombus, hemangiosarcoma and papillary endothelial hyperplasia. [ABSTRACT FROM AUTHOR]

BackgroundThe en bloc resection of inferior vena cava (IVC) leiomyosarcoma often necessitates IVC reconstruction. The objective of this study is to examine outcomes after IVC reconstruction and determine optimal graft sizing.MethodsA retrospective review was conducted of all IVC reconstructions after IVC leiomyosarcoma resection at a single institution. Cross-sectional dimensions at the IVC resection margins were measured on preoperative imaging. The tumor location was based on the most superiorly involved region of the IVC and was classified as infrarenal, between hepatic and renal veins, or superior to the hepatic veins. Perioperative details and long-term outcomes including graft sizing, graft patency, morbidity, and mortality were recorded.ResultsBetween 2007 and 2017, 12 patients (6 females, mean age: 64.5 years, age range: 46-80 years) underwent IVC leiomyosarcoma resection and reconstruction. All reconstructions were performed with ringed polytetrafluoroethylene (PTFE); graft sizes ranged from 12 mm to 16 mm. The tumor location was exclusively infrarenal in seven patients, between the renal and hepatic veins in two patients, and involved multiple segments in three patients. Larger graft sizes were utilized in reconstructing more superior segments of the IVC. Grafts were typically undersized and based on the diameter of the superior resection margin with 12 mm grafts approximately correlating to a 20 mm diameter, 14 mm to 25 mm, and 16 mm to 30 mm. The average undersizing ratio was 0.6. At a mean follow-up time of 43 ± 27 months, radiographic graft patency was 92%, overall survival was 83%, and disease-free survival was 25%.ConclusionsAfter en bloc resection of IVC leiomyosarcoma, caval reconstruction with an undersized ringed PTFE has acceptable patency. Grafts sizes should be based on the IVC diameter superior to the tumor and undersizing by approximately 40% appears to be associated with acceptable patency rates. Further multiinstitutional studies should be performed to best determine the optimal treatment of this rarely encountered tumor.

Objective To explore the application value of endovascular embolization plus microneurosurgery for vascular rich tumors in children. Methods From July 2019 to July 2021, five children with giant intracranial vascular tumor undergoing combined surgery were recruited as research objects.And perioperative complications and outcomes were reviewed retrospectively. Results Among them, there were atypical teratoma/rhabdomyoma (AT/RT) in posterior fossa (WHO Grade Ⅳ, n=2), hairy cell astrocytoma in posterior fossa (WHO Grade Ⅰ, n=1), giant cell astrocytoma (WHO Grade Ⅰ) under ependymal membrane of temporal lobe (WHO Grade Ⅰ, n=1) and polymorphic yellow astrocytoma (WHO Grade Ⅱ, n=1). All patients underwent compound surgery.All of them remained conscious post-operation.Four of them could move their limbs and intracranial hemorrhage was absent on postoperative computed tomography (CT). During a follow-up period of 3 months, magnetic resonance imaging (MRI) reexamination showed no tumor recurrence or progression. Conclusion During microsurgery, preoperative occlusion of major tumor blood supply vessels may significantly minimize hemorrhage and maintain a distinct surgical field.And embolic material has the intraoperative function of positioning and navigation so that it ensures a complete resection of huge vascular rich tumors for retaining normal brain tissue as much as possible and protecting brain function.Compound surgery has an important application value for nervous system tumors. [ABSTRACT FROM AUTHOR]

BACKGROUND:Pediatric intracavernous sinus tumors are exceedingly rare and thus poorly characterized. Their neurosurgical management is challenging and diagnostic, and management guidelines are limited. OBJECTIVE:To report our institutional experience with the surgical resection of pediatric intracavernous sinus tumors. We also compare and contrast our results with the 14 cases of pediatric intracavernous sinus lesions in the current literature. METHODS:A retrospective descriptive analysis of consecutive pediatric patients (ages 0-18 yr) presenting to our institution with a diagnosis of an intracavernous sinus lesion was performed. From January 2012 to January 2017, 5 cases were identified. Eleven patients with secondary invasion of the cavernous sinus (2 meningiomas, 7 pituitary adenomas) or dermoid tumors involving the cavernous sinus (2) were not included in our review. RESULTS:Surgical resection via a frontotemporal orbitozygomatic approach was performed in all cases by a single senior neurosurgeon (M.L.). There were no perioperative or postoperative complications attributable to the surgery or approach. Four of 5 patients remained neurologically stable throughout the perioperative and postoperative period. The fifth patient had a complete resolution of their cranial neuropathies postoperatively. A pathological diagnosis that guided long-term management was obtained in all cases. CONCLUSION:Neurosurgical management of pediatric cavernous sinus lesions can be safely performed and critically guide future therapies. Surgeon familiarity with cavernous sinus and skull-base anatomy is critical to the successful management of these patients. The benefits of surgery should be balanced against the potential complications and need for a tissue diagnosis in children. The senior author had a significant experience with cavernous sinus approaches in adults prior to initiating use of the approach in the pediatric population.

IntroductionMany patients who undergo coronary artery bypass surgery have a prior history of cancer and potentially chest radiation which is a known risk factor for coronary atherosclerosis. Prior radiation increases fibrosis and can make the dissection of the left internal mammary artery (LIMA) more challenging.Case reportA 72-year-old woman with a history of stage IIA pT2N0M0 left breast intraductal carcinoma treated with lumpectomy, adjuvant chemotherapy and radiation therapy 11 years prior presented to the emergency room with a non-ST elevation myocardial infarction and was taken for cardiac catheterization followed by three-vessel coronary artery bypass grafting. The LIMA was found to be encased in scar tissue and was deemed unsuitable as a conduit, and a saphenous vein graft was bypassed to the left anterior descending artery in its place. Pathologic review of the LIMA showed nests of tumor cells infiltrating within dense fibrous tissue with areas of necrosis and calcifications consistent with recurrent breast cancer. Interestingly the patients original breast cancer was positive for estrogen receptors (ER) and progesterone receptors (PR) ER and PR and negative for HER2 and she had therefore been treated with 5 years of hormonal therapy. The recurrent cancer found in the LIMA dissection bed at the time of bypass surgery was ER, PR, and HER2 negative, suggesting hormonal therapy driven clonal selection of these metastatic tumor cells.Discussion and conclusionsScarring in the LIMA dissection bed in patients with a history of cancer and prior chest radiation should be carefully evaluated for the possibility of recurrent cancer. The gross appearance of tissue can be misleading and sending a biopsy for a formal frozen section histologic evaluation should be considered if there is any question of recurrent malignancy.

Malignant melanoma (MM) is traditionally known as the "great mime" of human pathology, as it is potentially capable of imitating the most disparate neoplasms. It is known that in addition to the more classic histotypes of MM, there are also rare forms, including angiomatoid MM. Similarly, it has been amply demonstrated in the literature that MM is capable of dedifferentiating, losing melanocytic lineage markers, constituting a diagnostic challenge for the pathologist. Although 5 cases of primary angiomatoid MM have been described in the literature, to the best of our knowledge, no cases of dedifferentiated melanoma with pseudo-angiomatoid aspects have ever been described. In this paper, we present a very rare case of partially dedifferentiated MM in which the most dedifferentiated component lost melanocytic lineage immunohistochemical markers and assumed a pseudo-angiomatous morphology. Given the rarity of the case, we carried out a literature review of similar cases described, trying to draw new future perspectives not only about this particular variant of MM but also about the widest field of dedifferentiation/undifferentiation of MM. [ABSTRACT FROM AUTHOR]

Herein, we describe the case of a 20-year-old woman who presented with dysphagia of 2 months’ duration associated with vomiting, moderate abdominal pain, decreased oral intake, and significant weight loss. During the past 3 years, the patient experienced intermittent mild abdominal pain with infrequent vomiting. Endoscopy at Jordan University Hospital showed a mass in the esophagus, and endoscopic biopsies were performed. The preliminary histopathological report excluded malignancy. Two days after endoscopy, the patient presented to the emergency department complaining of severely worsening pain and total dysphagia. The pain persisted despite intravenous paracetamol administration, which was concerning for esophageal perforation; therefore, an urgent surgical intervention was performed. The mass was removed surgically, along with a para-esophageal lymph node. The final histopathological results of the endoscopic and resected specimens supported the diagnosis of pseudomyogenic hemangioendothelioma (PMHE). This is the first case reporting esophageal involvement of PMHE.

Intravascular papillary endothelial hyperplasia (IPEH) is a rare benign non-neoplastic vascular lesion. A typical presentation consists of a subcutaneous nodule that may simulate other clinical entities. Presentation in the oral cavity is uncommon. It is thought to develop as an abnormal proliferative reaction of endothelial cells in a process of impaired thrombogenesis. When endothelial proliferation occurs, a differential diagnosis with a soft tissue sarcoma, in particular an angiosarcoma, should be performed. We report a case of a 68-year-old female patient who presented with a lesion on the upper lip of 3 months' duration. Surgical resection revealed an IPEH. 1 year later, the patient showed a local recurrence requiring excision with clear margins. Pathological and immunohistochemical features can help us distinguish these lesions from those requiring more aggressive treatment. The gold standard is surgical resection with clear margins. Accurate preoperative diagnosis is essential to avoid overtreatment. Emphasis should be placed on clinical, radiological and histological studies. [ABSTRACT FROM AUTHOR]

Diffuse hepatic hemangiomatosis (DHH) is an uncommon vascular lesion, though hemangiomas are the commonest benign tumors of the liver. The etiology is largely unknown to date; however, its association with giant cavernous hemangiomas (GCH) has been reported in the literature. We present herein, the case of a 37-year-old hypothyroid woman with abdominal fullness for 2 months. The contrast-enhanced computed tomography revealed multiple wellencapsulated lesions involving the liver lobes and was diagnosed as giant cavernous hemangiomas. Most of them, except the deep-seated ones, were enucleated. Histopathological examination highlighted the presence of GCH with irregular margin, replacement of hepatic parenchyma, and presence of multiple micro-hemangiomas suggesting the possibility of DHH further substantiated by retrospective radiological assessment. No extrahepatic vascular lesion was noted, and the post-operative recovery and follow-up were uneventful. Adult DHH is an uncommon entity. The diagnosis of DHH and its distinction from GCH are important from the management and prognostic point of view as recurrence, extrahepatic manifestations, features of consumption coagulopathy, and death from the complications are not uncommon.

Background: Masson's tumor, also known as IPEH (intravascular papillary endothelial hyperplasia), is a rare benign vascular process, comprising approximately 2% of vascular tumors of the skin and subcutaneous tissue. IPEH presenting as a neck mass is rare, with only 5 reports of anterior neck mass and 7 cases of lateral neck lesions, of which 1 was in an external jugular vein aneurysm. To the best of our knowledge, the localization of intravascular papillary endothelial hyperplasia in the supraclavicular region is rarely reported. Case Report: We describe our management of a non-traumatic and non-painful mass on the right supraclavicular region in 24-year-old woman. Ultrasound revealed a heterogeneously hypoechoic mass with intense vascularization. Magnetic resonance imaging (MRI) showed a formation with lobulated contours and closely related to muscular layers of serratus anterior muscle. The findings of ultrasound-guided biopsy (FNA) were inconclusive. Complete removal of the mass was performed. Histopathological examination showed a well-circumscribed lesion with numerous small papillary structures. The papillae had hyalinized hypocellular cores covered by flattened endothelium. Immunohistochemical analysis showed endothelial positivity for CD34 and CD31. These features are typical of IPEH. No recurrence had occurred at 12 months after surgery. Conclusions: The differential diagnosis of malignant tumors like angiosarcoma is important because the prognosis significantly differs. Surgical excision is the treatment of choice. In our experience, the size of the lesion and its critical landmarks have not influenced the outcomes of the surgical procedure in terms of potential functional damages. [ABSTRACT FROM AUTHOR]

Infantile hemangiomas (IHs) are the most common vascular tumors in infants, appearing in early infancy and ultimately regressing with time. Clinical presentation may vary, with a minority of lesions causing impairment of vital function (e.g., respiratory or visual obstruction), permanent scarring, and/or disfigurement. The pathogenesis of IH is complex and poorly understood. Risk factors implicated in their development include preterm birth and placental anomalies. IH presents a myriad of clinical challenges, including correct diagnosis and whether or not to pursue treatment. This article is a review of the current literature regarding pathogenesis, clinical presentation, treatment, and prognosis of IH. Birth Defects Research 109:809-815, 2017. © 2017 Wiley Periodicals, Inc.

Characteristic but rare vascular neoplasms in the adult liver composed of small vessels with an infiltrative border were collected from an international group of collaborators over a 5-year period (N=17). These tumors were termed hepatic small vessel neoplasm (HSVN), and the histologic differential diagnosis was angiosarcoma (AS). The average age of patients was 54years (range, 24-83years). HSVN was more common in men. The average size was 2.1cm (range, 0.2-5.5cm). Diagnosis was aided by immunohistochemical stains for vascular lineage (CD31, CD34, FLI-1), which were uniformly positive in HSVN. Immunohistochemical stains (p53, c-Myc, GLUT-1, and Ki-67) for possible malignant potential are suggestive of a benign/low-grade tumor. Capture-based next-generation sequencing (using an assay that targets the coding regions of more than 500 cancer genes) identified an activating hotspot GNAQ mutation in 2 of 3 (67%) tested samples, and one of these cases also had a hotspot mutation in PIK3CA. When compared with hepatic AS (n=10) and cavernous hemangioma (n=6), the Ki-67 proliferative index is the most helpful tool in excluding AS, which demonstrated a tumor cell proliferative index greater than 10% in all cases. Strong p53 and diffuse c-Myc staining was also significantly associated with AS but not with HSVN or cavernous hemangioma. There have been no cases with rupture/hemorrhage, disseminated intravascular coagulation, or Kasabach-Merritt syndrome. Thus far, there has been no metastasis or recurrence of HSVN, but complete resection and close clinical follow-up are recommended because the outcome remains unknown.

Introduction: Hemangiomas are benign tumors that are very common in the head and neck region. However, intravascular hemangiomas are very rare. Hemangiomas are classified as capillary, cavernous or mixed tumors according to the proliferating cells. Ultrasound, computed tomography, MR imaging and angiography are useful diagnostic tools and are generally required when planning surgical treatment. Definitive diagnosis is established by histopathological examination, differentiating hemangiomas from other vascular tumors or malignancies. Case Report: We present a rare case of capillary hemangioma protruding from the external jugular vein. In our patient, the tumor was totally removed under local anesthesia. No complications and no recurrence were observed in the following two years. Conclusion: Intravascular tumors can present as neck masses and a definitive diagnosis is made by histopathological examination. Imaging tools provide important information about anatomy, the extent of the tumor, and for surgical planning.

Kaposiform hemangioendothelioma is a rare neoplasm with intermediate malignant behavior, mainly affecting infants and children. Involvement head and neck is uncommon, and there are only four cases reported in the oral cavity and oropharynx. Microscopically, it is characterized by a vascular proliferation permeated by spindle-to-ovoid cells resembling Kaposi sarcoma. Immunohistochemically, the tumor is positive for CD31, CD34 and negative for D2-40. Herein we present a rare case of intraoral Kaposiform hemangioendothelioma in a 10-year-old boy. [ABSTRACT FROM AUTHOR]

Malignant melanoma (MM) is traditionally known as the “great mime” of human pathology, as it is potentially capable of imitating the most disparate neoplasms. It is known that in addition to the more classic histotypes of MM, there are also rare forms, including angiomatoid MM. Similarly, it has been amply demonstrated in the literature that MM is capable of dedifferentiating, losing melanocytic lineage markers, constituting a diagnostic challenge for the pathologist. Although 5 cases of primary angiomatoid MM have been described in the literature, to the best of our knowledge, no cases of dedifferentiated melanoma with pseudo-angiomatoid aspects have ever been described. In this paper, we present a very rare case of partially dedifferentiated MM in which the most dedifferentiated component lost melanocytic lineage immunohistochemical markers and assumed a pseudo-angiomatous morphology. Given the rarity of the case, we carried out a literature review of similar cases described, trying to draw new future perspectives not only about this particular variant of MM but also about the widest field of dedifferentiation/undifferentiation of MM.

Cavernous hemangiomas are benign neoplasms of endothelial cells. Although this neoplasm has the potential to develop in all parts of the body, it rarely develops in the axilla; in fact, there are only two case reports of axillary cavernous hemangiomas in the literature. Here, we describe a third case, which occurred in a 30-year-old Korean woman. The patient presented with a palpable mass in the left axilla that was initially thought to be either a phyllodes tumor or a lymphoma based on imaging studies. However, the results of an excisional biopsy led to a diagnosis of cavernous hemangioma. Although uncommon, a cavernous hemangioma can be encountered unexpectedly, presenting as a mass in axilla. Although usually curative, surgery may be challenging not only because of the rarity of the condition, but also because of inconsistent preoperative findings and the involvement of large vessels.

Symplastic hemangiomas (SH) are benign vascular lesions that show atypia in vascular smooth muscle and interstitial cells with sparing of endothelial cells. We present four cases of this rare tumor. The patients (two males; two females) ranged in age from 57 to 83 years (median 74); lesions were located on the leg (n = 3) and back (n = 1), and ranged from 6 to 8 mm. SH were well‐circumscribed and dermal‐based, often with an epidermal collarette (3/4). They were characterized by the presence of variably atypical, hyperchromatic/pleomorphic epithelioid to spindled cells within vascular walls (3/4) and/or perivascular stroma (4/4). Atypical multinucleated cells were present in three of four cases. The overall mitotic rate was low (0‐2 mitotic figures per 10 HPFs; mean 0.75 per 10 HPFs), but atypical mitotic figures were seen in two of four cases. Atypical cells were negative for SMA (0/2), desmin (0/2), AE1/3 (0/2), and CAM5.2 (0/1). ERG, CD31, and CD34 stains were positive in endothelial cells but negative in atypical cells (4/4). Lesional cells and vessels were negative for podoplanin (3/3). Symplastic hemangioma is a benign tumor with bizarre atypia that may be mistaken for malignancy. We present four cases of this rare entity to increase awareness of this tumor and discuss the differential diagnosis. [ABSTRACT FROM AUTHOR]

Intravascular large cell lymphoma (IVLCL) is a rare disease characterized by proliferation of malignant lymphocytes within the small blood vessel lumens. The association of IVLCL with autoimmune hemolytic anemia (AIHA) has been described in a single case report, but the true prevalence of this co-occurrence is not known because of declining autopsy rates. Here, we report a case of a 41-year-old woman who carried a diagnosis of AIHA for 2 years, with repeated hemolytic episodes that were initially well controlled with immunomodulatory treatment. At her last presentation, the patient developed rapidly progressive neurologic symptoms and leukoencephalopathy on MRI; she died 4 weeks later with a clinical impression of thrombotic microangiopathy, a known complication of AIHA. At autopsy, the brain showed widespread platelet thrombi and intraparenchymal hemorrhages characteristic of this disorder. In addition, there was evidence of a clinically unsuspected IVLCL, most likely of B-cell lineage. This case illustrates a potential association between IVLCL and AIHA, highlights the need for broad differential diagnosis in cases with atypical disease presentation or progression, and underlines the importance of autopsy in establishing the full cause of morbidity and mortality.

Abstract Lobular capillary hemangioma or pyogenic granuloma is a benign vascular tumor of the skin or mucous membranes. Most patients present a single lesion. It manifests clinically as an erythematous, friable, and fast-growing tumor. This report details a case with exuberant presentation in a patient with ankylosing spondylitis, using adalimumab. Factors triggering pyogenic granuloma are not well known. They may spontaneously regress, but most require treatment.

Hemangioendothelioma is a rare vascular tumor with involvement of the liver, brain, long bones, and lung. Among the 6 histological subtypes, epithelioid hemangioendothelioma (EHE) is the most aggressive. Its occurrence in the mediastinum is quite rare, and very few cases have been documented. The reported cases in the literature have described difficulties in the preoperative diagnosis due to the unusual histological appearance of the tumor. Immunohistochemistry remains the mainstay for a definitive diagnosis. Due to its low incidence, there is no standard treatment for mediastinal EHE, but curative resection is the preferred treatment option where possible, with chemotherapy used as an adjuvant treatment or in cases of widespread inoperable disease. The present case study describes an aggressive EHE occurring in an 18 year-old woman in the anterior mediastinum.

Infantile hemangiomas (IHs) are common neoplasms composed of proliferating endothelial-like cells. Despite the relative frequency of IH and the potential severity of complications, there are currently no uniform guidelines for treatment. Although propranolol has rapidly been adopted, there is significant uncertainty and divergence of opinion regarding safety monitoring, dose escalation, and its use in PHACE syndrome (PHACE = posterior fossa, hemangioma, arterial lesions, cardiac abnormalities, eye abnormalities; a cutaneous neurovascular syndrome characterized by large, segmental hemangiomas of the head and neck along with congenital anomalies of the brain, heart, eyes and/or chest wall). A consensus conference was held on December 9, 2011. The multidisciplinary team reviewed existing data on the pharmacologic properties of propranolol and all published reports pertaining to the use of propranolol in pediatric patients. Workgroups were assigned specific topics to propose protocols on the following subjects: contraindications, special populations, pretreatment evaluation, dose escalation, and monitoring. Consensus protocols were recorded during the meeting and refined after the meeting. When appropriate, protocol clarifications and revision were made and agreed upon by the group via teleconference. Because of the absence of high-quality clinical research data, evidence-based recommendations are not possible at present. However, the team agreed on a number of recommendations that arose from a review of existing evidence, including when to treat complicated IH; contraindications and pretreatment evaluation protocols; propranolol use in PHACE syndrome; formulation, target dose, and frequency of propranolol; initiation of propranolol in infants; cardiovascular monitoring; ongoing monitoring; and prevention of hypoglycemia. Where there was considerable controversy, the more conservative approach was selected. We acknowledge that the recommendations are conservative in nature and anticipate that they will be revised as more data are made available.

Retiform hemangioendothelioma (RH) is a rare intermediate (locally aggressive) vascular tumor that mostly affects the dermis of the trunk and limbs, but has never been reported in the inferior turbinate. A 10-year-old Chinese boy presented with recurrent epistaxis in his left nasal cavity and anemia for more than 2 years. Radiographic and electronic video laryngoscopic images showed an expansile mass in the left inferior turbinate. Endoscopic surgery and electrocautery were performed to resect the tumor beyond the macroscopic border. Histopathologically, the tissues were infiltrated by hyperplastic blood vessels arranged in a retiform pattern, and endothelial cells proliferate significantly in some areas. Immunohistochemistry showed a positive result for CD31, CD34, Fli-1, and ERG. No epistaxis, tumor recurrence, or metastasis was found on reexamination over 18 months after surgery., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Infantile hemangioma (IH) is a benign vascular tumor that occurs in 5% of newborns. The tumor follows a life cycle of rapid proliferation in infancy, followed by slow involution in childhood. This unique life cycle has attracted the interest of basic and clinical scientists alike as a paradigm for vasculogenesis, angiogenesis, and vascular regression. Unanswered questions persist about the genetic and molecular drivers of the proliferating and involuting phases. The beta blocker propranolol usually accelerates regression of problematic IHs, yet its mechanism of action on vascular proliferation and differentiation is unclear. Some IHs fail to respond to beta blockers and regrow after discontinuation. Side effects occur and long-term sequelae of propranolol treatment are unknown. This poses clinical challenges and raises novel questions about the mechanisms of vascular overgrowth in IH.

Abstract: Epithelioid hemangioma (EH) is a benign vascular tumor displaying diverse histomorphologies. Among these, one EH subtype comprises cellular sheets of atypical epithelioid cells, posing potential challenges in distinguishing it from malignant vascular lesions. In this case report, we present a cutaneous cellular EH that carries the rare GATA6::FOXO1 gene fusion, a recent discovery. Our aim is to provide an updated insight into the evolving knowledge of EHs while delving into the histologic and molecular characteristics of the primary differential diagnoses., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Papillary hemangioma is a novel variant of intravascular hemangioma. It is more common in adults and has a male predominance. Most tumors reported so far are solitary and cutaneous. Here we present a rare case of an intraosseous papillary hemangioma involving the frontal bone. Brain imaging in a 69-year-old man with a slowly enlarging swelling on the right frontal area following an accidental fall demonstrated a 4.5 cm × 1.7 cm × 4.2 cm mass originating from the right frontal bone, with a tiny defect on the orbital roof. A malignant process was favored, and the mass was removed. Histopathology revealed a vascular lesion showing intraosseous distribution with foci of extension into the fibrous connective tissue. There were areas of plump endothelial cells with intracytoplasmic hyaline globules arranged in papillary configuration. The lesional cells were immunoreactive with CD34. AE1/AE3, EMA, PR, D2-40, inhibin, and S100 stains were negative. Ki-67 was low. This is the first intraosseous and second noncutaneous papillary hemangioma. Clinically it differs from other cases by the presence of trauma as a preceding event. Since its prognosis is unknown such patients should be monitored for recurrence or malignant transformation., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Purpose: This study aimed to build and evaluate a deep learning (DL) model to predict vessels encapsulating tumor clusters (VETC) and prognosis preoperatively in patients with hepatocellular carcinoma (HCC)., Methods: 320 pathologically confirmed HCC patients (58 women and 262 men) from two hospitals were included in this retrospective study. Institution 1 (n = 219) and Institution 2 (n = 101) served as the training and external test cohorts, respectively. Tumors were evaluated three-dimensionally and regions of interest were segmented manually in the arterial, portal venous, and delayed phases (AP, PP, and DP). Three ResNet-34 DL models were developed, consisting of three models based on a single sequence. The fusion model was developed by inputting the prediction probability of the output from the three single-sequence models into logistic regression. The area under the receiver operating characteristic curve (AUC) was used to compare performance, and the Delong test was used to compare AUCs. Early recurrence (ER) was defined as recurrence within two years of surgery and early recurrence-free survival (ERFS) rate was evaluated by Kaplan-Meier survival analysis., Results: Among the 320 HCC patients, 227 were VETC- and 93 were VETC+ . In the external test cohort, the fusion model showed an AUC of 0.772, a sensitivity of 0.80, and a specificity of 0.61. The fusion model-based prediction of VETC high-risk and low-risk categories exhibits a significant difference in ERFS rates, akin to the outcomes observed in VETC + and VETC- confirmed through pathological analyses (p < 0.05)., Conclusions: A DL framework based on ResNet-34 has demonstrated potential in facilitating non-invasive prediction of VETC as well as patient prognosis., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Purpose: Vascular anomalies are classified as either vascular tumors or vascular malformations. Vascular malformations can be difficult to diagnose and treat in the pediatric population and can masquerade as malignant processes. Understanding the genetics behind vascular malformations can lead to identification of specific mutations which can be treated with targeted immunotherapy., Methods: Our case presents a pediatric patient with progressively enlarging vascular malformation despite multiple surgical resections and systemic medical treatments who underwent genetic evaluation and was found to have PIK3CA mutation., Results: After identification of PIK3CA mutation, our patient was successfully treated with the p110ɑ-specific inhibitor, alpelisib, with both shrinkage of malformation on follow-up imaging as well as gains in her developmental milestones., Conclusion: Progressive vascular malformations in the pediatric population can be hard to diagnose and treat and are thought to arise from somatic mutations. Our case highlights a patient with progressive malformation despite multiple surgical resections who was successfully treated with targeted immunotherapy after proper identification of genetic mutation., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Abstract: Epithelioid hemangioendothelioma (EHE) is a rare vascular malignant tumor that comprises less than 1% of all vascular tumors. Cutaneous involvement in EHE can occur either by spreading from underlying bone or rarely could be limited to the skin and mostly presents as solitary well-circumscribed mass to an ill-defined infiltrative lesion. We present a case of rapidly progressive and debilitating EHE presenting multiple vascular papules and nodules. Histopathology showed an ill-circumscribed nodular proliferation of epithelioid and spindled cells in the dermis that extended into the subcutaneous tissue. The tumor cells had moderate eosinophilic cytoplasm, vesicular chromatin, and prominent nucleoli. In addition, they showed evidence of lumen formation and intracytoplasmic vacuoles. Brisk mitosis was noted. On immunohistochemistry, the cells were strongly positive for CD31, CD34, and ERG (ETS [erythroblast transformation-specific]-related gene). MIB-1 labeling index was more than 75% in the highest proliferating areas. A high degree of clinical suspicion and immunopathological examination is recommended for early diagnosis of this rare condition before it becomes function or life-threatening., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Lobular capillary haemangioma (LCH), previously known as pyogenic granuloma, is a benign vascular tumour of the skin or mucosa. We report a patient with spontaneous eruption of LCH, a rare occurrence, which resolved probably due to reverse koebnerisation., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Neural invasion (NI) and vascular tumor thrombus (VT) are associated with poor prognosis in patients with colorectal cancer (CRC). In this study, we apply 16S rRNA amplicon sequencing to tumor tissues and adjacent normal tissues in patients with CRC to determine the microbial differences. A discovery cohort, including 30 patients with NI, 23 with VT, and 35 with double-negative CRC tissue, is utilized. Then, we analyze the relationship between the specific bacterial taxa and indicators of different dimensions in separate cohorts. In the discovery cohort, the diversity and composition of the gut microbiome distinctly differ between the tumor and nontumor tissues in the NI and VT groups. A high abundance of Cupriavidus is found to be related to a short survival time of NI CRC, while Herbaspirillum is a potential microbial biomarker predicting the prognosis of patients with CRC with NI or VT. Moreover, the abundance of Cupriavidus or Herbaspirillum is associated with some clinical patient characteristics and prognosis, respectively. In conclusion, this study is the first to comprehensively elaborate the differences in the gut microbiota of patients with CRC with different invasion statuses and to prove the relationship between some gut microbiota and clinical patient characteristics.

Background and Objectives: Kaposiform hemangioendothelioma (KHE) and tufted angioma (TA) are rare vascular tumors in children historically associated with significant morbidity and mortality. This study was conducted to determine first-line therapy in the absence of available prospective clinical trials., Methods: Patients from 17 institutions diagnosed with KHE/TA between 2005 and 2020 with more than 6 months of follow-up were included. Response rates to sirolimus and vincristine were compared at 3 and 6 months. Durability of response and response to other treatment modalities were also evaluated., Results: Of 159 unique KHE/TA subjects, Kasabach-Merritt phenomenon (KMP) was present in 64 (40.3%), and only two patients were deceased (1.3%). Over 60% (n = 96) demonstrated treatment response at 3 months, and more than 70% (n = 114) by 6 months (no significant difference across groups). The vincristine group had higher radiologic response at 3 months compared to sirolimus (72.7% vs. 20%, p = .03), but there were no differences between these groups at 6 months. There were no differences in rates of recurrent or progressive disease between vincristine and sirolimus., Conclusions: In this large, multicenter cohort of 159 patients with KHE/TA, rates of KMP were consistent with historical literature, but the mortality rate (1.3%) was much lower. Overall treatment response rates were high (>70%), and there was no significant difference in treatment response or durability of disease comparing sirolimus to vincristine. Our results support individualized treatment decision plans depending on clinical scenario and patient/physician preferences. Response criteria and response rates reported here will be useful for guiding future treatment protocols for vascular tumors., (© 2023 Wiley Periodicals LLC.)

Kasabach-Merritt Syndrome (KMS) is a rare clinical entity, found in 1% of angiomatous lesions, characterized by severe consumption coagulopathy/thrombocytopenia in association with highly aggressive neonatal vascular lesions. We describe a case of prenatally diagnosed vascular neoformation of the neck, revealed by obstetric ultrasound and then confirmed by fetal Magnetic Resonance, with subsequent onset of KMS at birth. The newborn was treated with Propanolol and Prednisone, without any benefit; Magnetic Resonance (MRI) at day 14 of life confirmed the hypervascularized lesion of about 6x7x6 cm on the right laterocervical site. Sirolimus therapy was then initiated leading to rapid improvement in thrombocytopenia and a volumetric reduction of the vascular lesion on MRI. After 12 months of treatment, MRI scan showed a further reduction in volume of the vascular lesion; also, the KMS-related thrombocytopenia was completely resolved: Sirolimus could represent the first line of treatment of vascular lesions in the neonatal age.

BACKGROUND: Hemangiomas at upper airway are a common disease in children, while in adults they are very infrequent. CLINICAL CASE: A 42-year-old HIV-negative man with HHV-8 negative/idiopathic multicentric Castleman disease. His main complaint began in 2016 with odynophagia, dysphagia and globus sensation in the hypopharynx. Physical examination revealed a violet tumor located at the right vallecula. Excisional biopsy reported a benign vascular tumor. Three days after the procedure, a new growth was observed by laryngoscopy. A second resection was performed and revealed an ulcerated capillary hemangioma. Two months later, the patient again referred globus sensation in the hypopharynx and another bigger violet tumor was found at the right vallecula. Resection was performed but due to airway complications, a tracheotomy was required. A month after this last procedure, the tumor reappeared again and was performed four episodes of sclerosis without improvement, by the tumor's relapsing nature, radiotherapy was the chosen treatment option. Twelve months after finishing the last radiotherapy session, the patient has not relapsed. CONCLUSIONS: Hemangiomas in the adult airway are an infrequent find, this topic is rarely mentioned in publications and radiotherapy is usually considered the last option for treating recurrences. [ABSTRACT FROM AUTHOR]

Epithelioid hemangioendotheliomas (EHE) are decidedly are form of hemangioendotheliomas with biphasic histopathological components. It is known to occur rarely in head and neck region. They are known to have aggressive behaviour with depictable recurrence rate and metastasis. Here we present two cases of epithelioidhemangioendothelioma with review of literature. [ABSTRACT FROM AUTHOR]

Background: Primary angiosarcoma (AS) of the central nervous system (PACNS) is an extremely rare malignancy. The meninges represent an uncommon site of origin of PACNS. This report describes a recurrent meningeal PACNS treated with surgery, radiotherapy, stereotactic radiosurgery, and paclitaxel at different stages of the disease. Case Description: A 36-year-old Asian male presented to our facility with a 4-month history of worsening headaches and complete right homonymous hemianopia. Neuroimaging revealed a left occipital lobe hematome with an underlying left tentorial tumor. After subtotal resection, neuropathological examination revealed features of a malignant endothelial cell AS. He received a course of adjuvant radiation therapy but experienced disease progression. He subsequently received additional stereotactic radiosurgery followed by weekly paclitaxel. Magnetic resonance imaging during the course of treatment revealed stable disease until patient died following another progression of his tumor. Conclusion: This case of a meningeal PACNS highlights the importance of considering this entity in the face of a malignant lesion presenting with intracranial hemorrhagic activity. Our observations suggest that the use of paclitaxel provided a modest clinical response in PACNS, highlighting the need to consider a combined approach structured mainly on surgery and radiotherapy. Stereotactic radiosurgery appears to be a promising treatment option. [ABSTRACT FROM AUTHOR]

Vascular Leiomyoma (VL) is a benign soft tissue tumor rarely observed in oral tissues. The aim of this paper is to present a new case of vascular leiomyoma of the lip (VLL), describing its clinical, microscopical, and immunohistochemical features, and review the literature on the tumor. A 27-year-old woman presented with a small painful lesion on lower lip mucosa. Excisional biopsy revealed a well-circumscribed, encapsulated mass formed by small and fusiform cells. Immunohistochemistry analysis revealed intense and diffuse expression of smooth muscle actin (SMA) within the tumor cells and CD34 immunoreactivity of the endothelial cells lining the vascular spaces, indicating the presence of blood vessels. A final diagnosis of VLL was established. No signs of recurrence were observed after five years of follow-up. Although VLL is a rare lesion, it must be considered in the differential diagnosis of nodular lesions on lower and upper lips. O Leiomioma Vascular é um tumor benigno de tecidos moles raramente observado nos tecidos da cavidade bucal. O objetivo desse artigo é apresentar um novo caso de um leiomioma vascular no lábio (LVL), descrevendo seus achados clínicos, microscópicos e imunoistoquímicos, e revisar a literatura sobre o tumor. Uma mulher de 27 anos de idade se apresentou com uma pequena lesão dolorosa na mucosa do lábio inferior. A biópsia excisional revelou um massa encapsulada e bem circunscrita, formada por células pequenas e fusiformes. A análise imunoistoquímica revelou expressão intensa e difusa de actina musculo liso (AML) nas células tumorais e imunorreatividade para CD34 em células endoteliais que revestem espaços vasculares, indicando a presença de vasos sanguíneos. O diagnóstico final de LVL foi estabelecido. Não foram observados sinais de recorrência após cinco anos de acompanhamento. Embora o LVL seja uma lesão rara, deve ser considerada no diagnóstico diferencial de lesões nodulares nos lábios inferior e superior. El leiomioma vascular (LV) es un tumor benigno de tejido blando que rara vez se observa en los tejidos orales. El objetivo de este trabajo es presentar un nuevo caso de leiomioma vascular del labio (VLL), describiendo sus características clínicas, microscópicas e inmunohistoquímicas, y revisar la literatura sobre el tumor. Una mujer de 27 años presentó una pequeña lesión dolorosa en la mucosa del labio inferior. La biopsia escisional reveló una masa encapsulada bien circunscrita formada por células pequeñas y fusiformes. El análisis de inmunohistoquímica reveló una expresión intensa y difusa de actina del músculo liso (AME) dentro de las células tumorales y la inmunorreactividad CD34 de las células endoteliales que recubren los espacios vasculares, lo que indica la presencia de vasos sanguíneos. Se estableció un diagnóstico final de VLL. No se observaron signos de recurrencia después de cinco años de seguimiento. Aunque VLL es una lesión rara, debe considerarse en el diagnóstico diferencial de las lesiones nodulares en los labios inferior y superior. [ABSTRACT FROM AUTHOR]

A 50-year-old man with a primary tricuspid valve intimal sarcoma and right ventricular and pulmonary arterial tumors with metastases to the chest wall and lung, confirmed on plain radiographs initially, presented with a cough. He underwent palliative complete removal of the cardiac tumor and partial removal of the right pulmonary arterial tumor with tricuspid valve replacement, to avoid sudden death caused by tumor embolization and to definitively diagnose the cardiac tumor. The cardiac tumor arose from the tricuspid valve leaflets. Nine months postoperatively, the patient died secondary to fatal progression of the pulmonary arterial and lung tumors. [ABSTRACT FROM AUTHOR]