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.

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.

Infantile scrotal hemangioma with transverse testicular ectopia.

Transverse testicular ectopia is a rare structural anomaly of abnormal testicular descent. We report a case of a 3-month-old boy with hemiscrotal infantile hemangioma and contralateral transverse testicular ectopia.

Verrucous venous malformation with thrombocytopenia in a neonate.

Verrucous venous malformations (VVM) are rare, congenital, slow-flow vascular anomalies that have been historically difficult to characterize due to clinical mimics and unclear histological evaluation. A life-threatening complication of VVMs is localized intravascular coagulation. Herein, we describe a male neonate who presented with a congenital VVM on the left lower extremity with associated severe thrombocytopenia. We discuss the multifaceted diagnostic approach used to identify this VVM, while highlighting the use of WT-1 as a negative predictive marker; we additionally outline novel treatment options and management beyond cutaneous involvement.

Primary Hepatic Neoplasms of Vascular Origin: Key Imaging Features and Differential Diagnoses With Radiology-Pathology Correlation.

OBJECTIVE: This article describes, illustrates, and correlates imaging and pathologic features of primary vascular mesenchymal neoplasms of the liver, which arise from the vascular endothelium and perivascular epithelioid cells. CONCLUSION: Familiarity with the spectrum of benign, malignant-potential and malignant vascular neoplasms, and nonneoplastic mimickers allows consideration in the differential diagnosis of enhancing hepatic masses. Understanding relevant pathologic features facilitates recognition of key imaging features, specifically dynamic contrast enhancement patterns on CT and MRI, which provide a useful classification system.

Atypical retiform hemangioendothelioma arising in a patient with Milroy disease: a case report and review of the literature.

Retiform hemangioendothelioma (RH) is a rare vascular neoplasm with a high rate of local recurrence and low metastatic potential. We describe an unusual case of RH in a 45-year-old patient with Milroy disease, with a prominent solid component diffusely involving a chronic lymphedematous leg. This case is consistent with the postulated relationship between lymphedema and vascular neoplasms developing as a result of local immune dysfunction, and highlights the need to closely monitor patients with Milroy disease for pathologic changes. Our case highlights a unique example of RH with atypical features. There are several noteworthy unusual clinical and histologic findings including diffuse involvement of an entire limb, solid component with cytologic atypia, D2-40 expression, and first-time-reported association with Milroy disease. Given the atypical histologic presentation of cytologic atypia, solid areas and atypical immunohistochemical profile with D2-40 positivity, this case could cause diagnostic difficulty, especially in the setting of such a broad clinical differential.

Composite hemangioendothelioma of the forehead and right eye; a case report.

BACKGROUND: Hemangioendothelioma includes several types of vascular neoplasms , as well as both benign and malignant growth. CASE PRESENTATION: This study evaluated a case of a 78-year-old female diagnosed with composite hemangioendothelioma (CHE). This patient had an 18-month history of painless inflammatory lesions and erythema on the left forehead and right upper eyelid. The clinical and pathologic characteristics of the CHE were evaluated in the present study. CONCLUSION: The evidence of the coexistence of variable components of the CHE in our study emphasized the importance of distinctive morphology and biology, and should be considered in the differential diagnosis of other vascular lesions.

Primary angiosarcoma of neck nodes with bony metastasis - A case report.

This is a case of a 30 year old female who presented in February 2012 with a large painless left neck swelling since 5 months. Her biopsy was done but the result was inconclusive. Later on after proper staging workup it was found to be bony metastatic disease. Her repeated biopsy showed spindle cell lesion. She was advised surgery, which was done revealing poorly differentiated angiosarcoma involving 27 out of 33 lymph nodes. Angiosarcoma is an uncommon malignant neoplasm characterized by rapidly proliferating, extensively infiltrating anaplastic cells derived from blood vessels and lining irregular blood-filled spaces. Cutaneous angiosarcoma of the scalp and face is the most common form of angiosarcoma. Angiosarcoma of the cervical lymph node without a primary scalp or oral cavity lesion is a very rare presentation and has been reported only once. Thus, this case reported with bony metastasis can be labeled as the first of its kind.

Cutaneous reactive angiomatosis associated with erythema ab igne.

We present the case of a 49-year-old man with an 18-month history of an intermittently pruritic red-brown reticulate eruption of the medial legs, with occasional blistering. There were areas of scale, crust and superficial erosions. The patient worked long overnight shifts standing in front of hot machinery wearing shorts, and spent time sitting in front of a heater at home. A clinical diagnosis of erythema ab igne (EAI) was made. This settled with heater avoidance and protection of the legs with long trousers, as well as a topical corticosteroid. Histology showed features of cutaneous reactive angiomatosis, a rare condition with various associations but only previously reported once in association with EAI.

An intraoral epitheloid hemangioendothelioma masquerading clinically as pyogenic granuloma.

Epitheloid hemangioendothelioma (EHE) is an uncommon angiocentric neoplasm of intermediate malignant potential. This tumor is frequently seen in the lungs and liver, but its presentation in the oral cavity is rare. In the oral cavity, gingival region is the common sites of occurrence. We report a rare case of epitheloid hemangioendothelioma in a 48-year-old male, presenting as a growth in the upper anterior gingiva of five months duration along with a review of its clinicopathological and immunohistochemical characteristics.

Two different treatment options for intramuscular plantar hemangioma: surgery versus percutaneous sclerotherapy.

Intramuscular hemangiomas are benign neoplasms usually seen in children and adolescents. They tend to occur in the deep fascia and muscle and more often in the lower extremity, although they are rarely encountered in the plantar musculature. Surgical excision, ultrasound- or fluoroscopic-guided percutaneous sclerotherapy, and angiographic embolization are all treatment options. Surgical excision is the most prevalent form of therapy, although this can be difficult in the hands and feet. For this reason, ultrasound- and fluoroscopic-guided percutaneous sclerotherapy is a useful treatment option for pedal intramuscular hemangioma. In the present report, we describe 2 cases of intramuscular hemangioma in children, 1 treated by excision and 1 by percutaneous sclerosis.

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.

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.

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.

Use of modified human hemangioma tissue cultures and human umbilical vein endothelial cell cultures to gain mechanistic insights into imiquimod treatment for infantile hemangioma.

Infantile hemangiomas (IH) are a common entity encountered by dermatologists, otolaryngologists, and other surgeons. Oral propranolol is a mainstay of treatment for IH and is well-tolerated, though propranolol-refractory IH and other drug-related adverse events are documented and can limit its usage. There are few in vitro testing systems for putative treatment agents. To address this, we modified a tissue culture system for human hemangioma treatment testing to evaluate the treatment impact of the immune modifier, imiquimod. Human umbilical vein endothelial cells (HUVEC) and hemangioma cultures were treated with several concentrations of imiquimod followed by MTT assays, reporter gene assays, PCR, ELISA, and Western blotting for IL-8, VEGF, Cyclin D1, and IFNα and immunohistochemistry for Cyclin D1 and Ki-67. HUVEC showed acute decreases in IL-8, VEGF, and Cyclin D1 promoter activity and increases in IFNα mRNA after imiquimod treatment. Hemangioma samples showed no change in Ki-67 or Cyclin D1 staining after treatment with imiquimod after 27 d, with significantly increased IL-8 and VEGF. From this preliminary analysis, we discerned that hemangioma tissues can be grown in tissue culture and used for drug treatment studies. We also conclude acute and chronic modulation of cell cycle, angiogenesis factors, and immunostimulatory conditions may be associated with imiquimod mechanisms of action in hemangioma involution.

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.