Infantile Hemangiomas and Vascular Anomalies.

Vascular anomalies represent a diverse group of disorders of abnormal vascular development or proliferation. Vascular anomalies are classified as vascular tumors and vascular malformations. Significant advances have been made in the understanding of the pathogenesis, natural history, and genetics of vascular anomalies, allowing for improvements in management including targeted molecular therapies. Infantile hemangiomas are the most common vascular tumor of childhood and follow a distinct natural history of proliferation and involution. Although benign, infantile hemangiomas can be associated with important complications. The use of beta-blockers has revolutionized the management of infantile hemangiomas. Other vascular tumors include pyogenic granulomas, congenital hemangiomas, and kaposiform hemangioendotheliomas, among others. Vascular malformations are categorized based on the type of involved vessel, including capillary malformations, venous malformations, lymphatic malformations, arteriovenous malformations, and mixed vascular malformations. Expert multidisciplinary management of vascular anomalies is critical to optimize outcomes in these patients. [Pediatr Ann. 2024;53(4):e129-e137.].

A Rare Case of Pulmonary Artery Sarcoma.

Pulmonary artery sarcoma (PAS) is a rare and aggressive mesenchymal tumor with an overall poor prognosis1-5. Due to similar clinical and radiologic findings, PAS is often misdiagnosed as a pulmonary embolism (PE) frequently leading to prolonged anticoagulation therapy, which delays the correct diagnosis 1-3. By presenting this clinical case our objective is to emphasize characteristic CT findings that favour a neoplastic origin of a pulmonary intravascular filling defect. PET-CT and MRI have also an important potential role in its diagnosis and therapeutical management.

[Diagnosis and treatment of intravenous leiomyomatosis].

Intravenous leiomyomatosis is a rare type of tumor that is histologically benign but biologically invasive. It originates from the smooth muscle of the uterine or the uterine vein. It can grow through the uterus and extend into the pelvic cavity, or grow along the veins without invading the wall of the venous vessel itself. The tumors are estrogen-dependent and can metastasize through the bloodstream. Thus, in addition to continuous growth, some tumors exhibit isolated growths in the venous system and heart chambers or show disseminated growth in the lungs, although distant metastasis to other regions usually do not occur. Currently, there is limited research on this disease, the majority of which are case reports, surgical experience summaries, and differentiation from ordinary gynecological myomas in terms of pathogenesis and radiological diagnostic experience. There are two main theories on the origin of the disease: uterine smooth muscle and smooth muscle of the uterine veins. Some studies have verified the role of estrogen, progesterone receptor-related pathways, and angiogenesis in the development of the disease. The clinical symptoms of this disease are varied, depending on the affected area. In the early stages, when the tumor only affects the pelvic cavity, patients show mild symptoms resulting from pelvic organ compression. When it progresses to the inferior vena cava and heart, patients show more complex symptoms resulting from venous return obstruction, cardiac obstruction, and hemodynamics appearing. Different institutions have proposed different disease staging and classification strategies for different clinical purposes. Some are based on the affected area of the lesion; others are based on the size of the tumor. Although surgery remains the main treatment for this disease, the specific surgical approach, adjuvant drug therapy, and prognosis still need further exploration.

A case report of anastomosing hemangioma of the ovary.

RATIONALE: Anastomosing hemangioma (AH) is a rare benign neoplastic vascular lesion that histologically resembles a well-differentiated angiosarcoma. AH commonly involves the urinary system and testes. However, these tumors can also involve the ovaries in some rare cases. This manuscript presents the case of a 28-year-old Chinese woman diagnosed with ovarian AH. PATIENT CONCERNS: The woman was admitted to the hospital with a 4-month history of a right ovarian mass discovered by ultrasound (US) after a spontaneous abortion. The US examination showed a 4 cm × 4 cm irregularly shaped mass with a rich blood supply. DIAGNOSES: AH of the right ovar. INTERVENTION: The patient underwent laparoscopic surgery to remove the mass. The postoperative pathological examination revealed that the mass contained capillaries arranged in a characteristic anastomotic or confluent pattern commonly seen in AHs. OUTCOMES: The mass was successfully removed. The follow-up examination at 7 months post-surgery showed that the patient recovered well, and no recurrence or metastasis was found. LESSONS: AH of the ovary is a rare benign vascular tumor. On imaging examinations, AHs appear as mostly well-defined, heterogeneous nodules with peripheral enhancement as other benign nodules. However, a definitive diagnosis can only be achieved through histopathological examination.

Contemporary diagnostic approach to atypical vascular lesion and angiosarcoma.

Vascular neoplasms account for a substantial fraction of cutaneous mesenchymal tumors, spanning from clinically indolent benign lesions to highly aggressive malignancies. These neoplasms present a distinctive challenge in terms of their diagnostic histopathology, both because of the breadth of their morphological manifestations and because of the significant histological overlap between different entities, even benign and malignant ones. The post-radiotherapy setting is particularly problematic diagnostically, insofar as radiation exposure predisposes not only to secondary angiosarcoma, but also to atypical vascular lesion, a largely benign proliferation of cutaneous blood vessels typically affecting the breast. To address these challenges, we explore the clinical, histological, and molecular features of malignant vascular neoplasia, including primary and secondary subtypes, through the comparative lens of atypical vascular lesion. In addition to highlighting the key morphological indicators of malignancy in superficial vasoformative tumors, we offer an approach that integrates clinical characteristics and molecular genetic profiling to facilitate accurate classification. With this current knowledge as our foundation, we also look ahead in an effort to frame some of the key unanswered questions regarding superficial vascular malignancies and their natural history, clinical management, and molecular underpinnings.

Gonadal vein leiomyosarcoma, from clinical practice to a literature review: surgical, oncological and histopathologic correlation.

BACKGROUND AND AIM: Vascular leiomyosarcomas are rare and generally originate from the muscular wall of the inferior vena cava. Leiomyosarcomas originating from the wall of the gonadal veins are rare and just about ten cases are described in literature. In the present paper, we have described a case of a LMS originating from the left gonadal vein. METHODS: A 44-year-old woman presented in March 2020 pain symptoms at the level of the left renal lodge. The subsequent CT and the biopsy confirmed the diagnosis of G2 grade LMS. The mass was then removed en bloc from the posterior and inferior pancreatic plane, from the aortic plane and from the retroperitoneal plane, post chemoteraphy. RESULTS: Pathologic report revealed a typical leiomyosarcoma, moderately differentiated G2 with minor dedifferentiated areas of pleomorphic leiomyosarcoma. CONCLUSIONS: The LMSs originating from gonadal veins represent an uncommon oncologic challenge. The radical en bloc excision represents the therapeutic gold standard.

Pulmonary artery sarcoma misdiagnosed as a pulmonary embolism: A case report.

Pulmonary artery sarcoma is a rare type of tumor and is easily misdiagnosed. We report a case of pulmonary artery sarcoma in a 26-year-old young man who presented with acute onset of dyspnea. Computed tomographic angiography of the chest had revealed a large filling defect within the main pulmonary artery (PA) that extended into both right and left PAs. Because pulmonary embolism was suspected, both anticoagulant and thrombolytic therapies were initiated. The patient responded poorly to these therapies, leading to the resection of the mass. After an uneventful postoperative course, the patient was discharged from the hospital on postoperative day ten. He was subsequently treated with chemotherapy and continued to show no evidence of disease. Multimodal therapy can provide prolonged survival.

Loss of NPM2 expression is a potential immunohistochemical marker for malignant peritoneal mesothelioma: a single-center study of 92 cases.

BACKGROUND: Malignant peritoneal mesothelioma (MPM) is a rare malignant tumor with a high mortality rate and extremely poor prognosis. In-depth pathological analysis is essential to assess tumor biological behaviors and explore potential therapeutic targets of MPM. Nucleoplasmin 2 (NPM2) is a molecular chaperone that binds histones and may play a key role in the development and progression of tumors. This study aimed to analyze the correlation between the expression level of NPM2 and the main clinicopathological characteristics and prognosis of MPM. METHODS: Ninety-two postoperative specimens from MPM patients following cytoreductive surgery were collected. Postoperative specimens were stained with immunohistochemistry. The expression level of NPM2 was quantitatively analyzed by QuPath-0.3.2 software. Univariate and multivariate analyses were conducted to investigate the correlation between NPM2 expression and other conventional clinicopathological characteristics. RESULTS: Among the 92 MPM patients, there were 47 males (48.9%) and 45 females (51.1%), with a median age of 56 (range: 24-73). There were 70 (76.0%) cases with loss of NPM2 protein expression, 11 (12.0%) cases with low expression, and 11 (12.0%) cases with high expression. Univariate analysis showed that NPM2 protein expression level (negative vs. low expression vs. high expression) was negatively correlated with the following three clinicopathological factors: completeness of cytoreduction (CC) score, vascular tumor emboli, and serious adverse events (SAEs) (all P < 0.05). Multivariate analysis showed that NPM2 protein expression level (negative vs. low expression vs. high expression) was independently negatively correlated with the following two clinicopathological factors: CC score [odds ratio (OR) = 0.317, 95% CI: 0.317-0.959, P = 0.042] and vascular tumor emboli (OR = 0.092, 95% CI = 0.011-0.770, P = 0.028). Survival analysis showed that loss of NPM2 protein expression (negative vs. positive) was associated with poor prognosis of MPM. CONCLUSIONS: Loss of NPM2 expression is a potential immunohistochemical marker for MPM.

Vascular Anomalies: Other Vascular Tumors.

Vascular tumors are classified into three categories by the International Society for the Study of Vascular Anomalies (ISSVA): benign, locally aggressive/borderline, and malignant. Many of these tumors are rare, cutaneous in nature, and present in childhood. The characterization and delineation of these distinct vascular tumors is an evolving area of clinical research. The diagnosis of these lesions relies on history and clinical presentation, location, histologic appearance, immunohistochemistry, and more recently, associated genetic mutations. This article provides a brief, yet comprehensive overview of all cutaneous vascular tumors currently recognized by the ISSVA, including presentation, diagnosis, and treatment.

How we approach localized vascular anomalies.

Vascular anomalies are a group of disorders divided into two distinct subtypes: vascular tumors and vascular malformations. Vascular tumors are proliferative in nature, while malformations are nonproliferative. Simple, localized vascular malformations refer to a group of malformations that are localized to a single area of involvement. These simple malformations include capillary, lymphatic, venous, and arteriovenous malformations. The pediatric hematologists and oncologists are becoming increasingly involved in the diagnosis and management of these disorders. This review presents four cases as a means to discuss the diagnosis, clinical and imaging features, and management strategies of simple, localized vascular malformations.

Epithelioid hemangioendothelioma - an unexpected diagnosis of a mediastinal tumor with extensive local thrombosis.

Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor and the mediastinal localization is amongst the most infrequent. We present the case of a 37-year-old woman with a history of resected left thyroid tumor that presented to our department for evaluation of a left supraclavicular palpable mass in close contact with local vascular structures, and with heterogeneous contrast enhancement as described by computed tomography (CT) and magnetic resonance imaging (MRI). Considering the history of the patient, the presumptive diagnosis of thyroid tumor recurrence was established, and the patient was referred to surgical department. During procedure, we encountered important bleeding from a ruptured jugular vein branch, which we assumed to be a newly formed tumor blood vessel. After surgery (48 hours postoperatively), the patient developed important local thrombosis that encompassed the left internal jugular vein, left subclavian vein and the left brachiocephalic trunk that partially subsided after anticoagulant therapy. The histological examination revealed the presence of a vascular tumor proliferation of epithelioid endothelial cells that was characteristic of an EHE confirmed later on the immunohistochemical studies as Yes-associated protein 1-transcription factor E3 (YAP1-TFE3) subtype. In addition to the case report, some relevant information from the scarce literature data about mediastinal EHE were reviewed here.

Presumptive metastatic leiomyosarcoma in a feedlot steer.

A 14-month-old feedlot steer was depressed and died while being examined. The gross post-mortem examination of the steer conducted at the feedlot identified numerous masses within the abdomen and thorax, including a large mass in the liver that eroded into the vena cava. Many masses in the lungs appeared to be the result of hematogenous distribution. Histologic examination of the masses confirmed the presence of neoplasia. Although the histologic appearance of the neoplasms was not typical of well-differentiated leiomyosarcoma, immunohistochemical staining supported that diagnosis. Leiomyosarcomas are rare among North American cattle. In this case, the primary neoplasm appears to have originated in the wall of the vena cava within the liver. Key clinical message: This report adds to the limited information on leiomyosarcomas in cattle, while highlighting both the challenges faced by veterinarians conducting post-mortem examinations on large animals in below freezing temperatures, as well as the current methods available to arrive at a diagnosis of a rare disease.

Léiomyosarcome métastatique présumé chez un bouvillon de parc d'engraissement. Un bouvillon de parc d'engraissement âgé de 14 mois était abattu et est mort pendant son examen. L'examen macroscopique du bouvillon réalisée au parc d'engraissement a identifié de nombreuses masses dans l'abdomen et le thorax, y compris une grosse masse dans le foie qui s'est étendue dans la veine cave. De nombreuses masses dans les poumons semblaient être le résultat d'une distribution hématogène. L'examen histologique des masses a confirmé la présence d'un néoplasme. Bien que l'aspect histologique du néoplasme ne soit pas typique d'un léiomyosarcome bien différencié, la coloration immunohistochimique a corroboré ce diagnostic. Les léiomyosarcomes sont rares chez les bovins nord-américains. Dans ce cas, le néoplasme primaire semble provenir de la paroi de la veine cave dans le foie.Message clinique clé :Ce rapport s'ajoute aux informations limitées sur les léiomyosarcomes chez les bovins, tout en soulignant à la fois les défis auxquels sont confrontés les vétérinaires effectuant des examens post-mortem sur de grands animaux à des températures inférieures au point de congélation, ainsi que les méthodes actuelles disponibles pour arriver à un diagnostic d'une maladie rare.(Traduit par Dr Serge Messier).

Epithelioid Vascular Lesions: The Differential Diagnosis and Approach in Cytology and Small Biopsies.

Vascular neoplasms are rare tumors with a multitude of clinical presentations and behavior, which make accurate identification and subclassification challenging on limited small biopsies. Within the spectrum of these lesions, the ones with epithelioid morphology, such as epithelioid hemangioendothelioma and epithelioid angiosarcoma, are particularly challenging given the morphologic overlap with nonvascular lesions and the limited cells due to hemodilution on sampling. Herein, we review the differential diagnosis of epithelioid vascular neoplasms, with a focus on the cytomorphology, differential diagnoses, and ancillary studies that pathologists should be aware of when evaluating small biopsies and aspirates, including novel translocations, and associated monoclonal immunohistochemistry antibodies, that can help in the diagnosis of some of these tumors. Awareness of these morphologic and ancillary study findings in these rare tumors will hopefully allow pathologists to recognize and render-specific diagnoses on limited samples of these challenging lesions.

Leiomyosarcoma arising from the ovarian vein as a gynecologic malignancy: Two case reports and a review of the literature.

Leiomyosarcoma arising from the ovarian vein has rarely been reported. Herein, we report two cases from a single institute. Given their direct connections to ovarian vessels, both leiomyosarcomas were initially suspected to be gynecological malignancies. In one case, leiomyosarcoma was discovered incidentally without any clinical symptoms; it had a close connection with the ovarian vein, was removed surgically, and the patient has survived for over 12 years. In another case, bowel obstruction caused by the tumor helped to identify metastatic leiomyosarcoma. Blood flow was supplied by the ovarian artery and grew into the lumen of the ovarian vein without invading adjacent organs. After surgical resection, the patient underwent 18 months of chemotherapy prior to palliative care. We propose that leiomyosarcoma arising from the ovarian vein should be treated as a gynecologic malignancy, especially if it develops in the lower abdomen.

Inferior vena cava leiomyosarcoma with liver metastasis at presentation in a young male: A challenging diagnostic quandary.

Leiomyosarcomas of vascular origin are very rare tumors, predominantly affecting the inferior vena cava (IVC). Although vascular leiomyosarcomas are slow-growing, their non-specific and late presentation results in delayed diagnosis which portends a very poor prognosis. Here we report a case of a 24-year-old man who presented with abdominal pain since 15 days and was found to have unresectable metastatic leiomyosarcoma of the inferior vena cava at initial diagnosis.

Histiocytic Sarcoma with an Unusual Clinical Manifestation Imitating Malignant Vascular Tumor: A Case Report.

BACKGROUND Histiocytic sarcoma is a rare malignant hematopoietic neoplasm with morphologic and immunohistochemical features of histiocytic differentiation, usually with unfavorable prognosis. Despite aggressive biological behavior, in subgroup of patients with localized disease, the prognosis can be very good. Few publications are available on localized cases of histiocytic sarcoma. These occur infrequently and continue to be a poorly-recognized morphological entity. CASE REPORT A 73-year old man treated for Parkinson syndrome presented with a tumor resistance on the dorsal surface of the left forearm. This lesion was clinically seen as an organized hematoma and was surgically resected. Histologically, the tumor was situated in the dermis and subcutis and it consisted of multiple neoplastic nodules. Vasoformative growth patterns with the vascular-like spaces containing erythrocytes and hemosiderin pigment presence simulated the morphology of angiosarcoma. Based on the immunohistochemical characteristics, we diagnosed the tumor as cutaneous histiocytic sarcoma. Genetic analysis revealed immunoglobulin heavy-chain gene rearrangement without any concomitant hematological malignancy. The patient demonstrated no systemic disease or impairment associated with diagnosed histiocytic sarcoma, and no recurrence has been found to date. CONCLUSIONS We report a case of primary cutaneous histiocytic sarcoma with an excellent outcome after surgical treatment only. Clinical data and histopathological and immunohistochemical evaluation were essential to rule out other malignant tumors in the differential diagnosis. Genetic analysis together with up-to-date knowledge and understanding of principles of tumorous transformations helped to diagnose this poorly-recognized entity with various clinical behaviors.

[Intravascular large B-cell lymphoma diagnosed by total hysterectomy].

Intravascular large B-cell lymphoma (IVLBCL) is a rare form of non-Hodgkin B-cell lymphoma which occurs mainly in capillaries and small blood vessels. Successful diagnosis of IVLBCL is challenging since it lacks tumor formation and presents various clinical manifestations. An 82-year-old Asian female patient presented to our emergency department with a history of general fatigue, weight loss, and fever for two weeks. The patient's random skin biopsy was negative, and her bone marrow biopsy revealed hemophagocytic syndrome with no obvious involvement of lymphoma cells. Gallium scintigraphy showed mild uptake in the uterus, pelvis, and spine. The repetitive bone marrow biopsy result and the endometrial cytology/biopsy were negative; however, the pelvic MRI was compatible with lymphoma, revealing lesions in the corpus uteri, pelvis, and vertebral body. After laparoscopic-assisted vaginal total hysterectomy and bilateral salpingo-oophorectomy, the diagnosis of the Asian variant of IVLBCL was made. Although total hysterectomy remains controversial for elderly patients with declining performance status, we could successfully diagnose the condition and initiate the treatment. The patient's general condition improved soon after starting rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone regimen on day 26, and she was discharged on day 45.